WorldWideScience

Sample records for syndrome complicating adalimumab

  1. Adalimumab and Non-Arteritic Anterior Ischaemic Optic Neuropathy: A Case Report.

    Science.gov (United States)

    Kinard, Krista; Walsh, Jessica A; Penmetsa, Gopi K; Warner, Judith E A

    2014-01-01

    Sequential anterior ischaemic optic neuropathy was observed in a patient treated with a tumour necrosis factor α (TNF) inhibitor, adalimumab, for ankylosing spondylitis. He developed decreased visual acuity in the right eye after 17 months of treatment. Findings showed right optic disc oedema with haemorrhages and visual field defect. Adalimumab was discontinued and vision stabilised. After restarting adalimumab, he developed optic neuropathy in the left eye. Findings showed optic disc oedema, with haemorrhages and visual field changes in the left eye. Adalimumab may be associated with optic neuropathy; providers prescribing TNF inhibitors should be aware of optic neuropathy as a potential complication.

  2. Sinogenic intracranial complications

    DEFF Research Database (Denmark)

    Kofoed, Mikkel Seremet; Fisker, Niels; Christensen, Anne Estmann

    2018-01-01

    We present two 11-year-old girls with chronic recurrent multifocal osteomyelitis, treated with adalimumab. Both developed severe intracranial complications to sinusitis. Patient 1 had been treated with adalimumab for 15 months when she developed acute sinusitis complicated by an orbital abscess, ...

  3. Clinical response to adalimumab: relationship to anti-adalimumab antibodies and serum adalimumab concentrations in rheumatoid arthritis

    NARCIS (Netherlands)

    Bartelds, Geertje M.; Wijbrandts, Carla A.; Nurmohamed, Michael T.; Stapel, Steven; Lems, Willem F.; Aarden, Lucien; Dijkmans, Ben A. C.; Tak, Paul Peter; Wolbink, Gerrit Jan

    2007-01-01

    BACKGROUND: A substantial proportion of patients with rheumatoid arthritis (RA) do not respond, or lose initial response, to adalimumab treatment. One explanation for non-response is that patients develop anti-adalimumab antibodies. OBJECTIVES: To evaluate the incidence of formation of antibody

  4. Criptosporidiose em paciente com espondilite anquilosante usando adalimumabe Cryptosporidiosis in a patient with ankylosing spondylitis treated with adalimumab

    Directory of Open Access Journals (Sweden)

    Fernando Augusto Chiuchetta

    2010-06-01

    Full Text Available A criptosporidiose é uma doença parasitária causada pelo protozoário Cryptosporidium sp. Observou-se um aumento no número de diagnósticos realizados nos últimos vinte anos, principalmente em pacientes que apresentam imunodeficiências como a síndrome da imunodeficiência humana adquirida e as imunodeficiências induzidas como em pacientes transplantados e nos que necessitam realizar hemodiálise frequentemente. Relata-se o caso de um jovem com espondilite anquilosante que, usando adalimumabe, apresentou diarreia devido à criptosporidiose.Cryptosporidiosis is a parasitic disease caused by a protozoan called Cryptosporidium sp. An increased number of diagnoses were made in the last 20 years, especially in patients with immunodeficiency like the acquired human immunodeficiency syndrome and induced immunodeficiency, such as in transplant patients and those who need frequent hemodialysis, has been observed. We report the case of a young patient with ankylosing spondylitis treated with adalimumab who developed chronic diarrhea secondary to cryptosporidiosis

  5. The Experience of Successful Use of Adalimumab in a Patient With Juvenile Arthritis Associated With Enthesitis and Uveitis

    Directory of Open Access Journals (Sweden)

    Aleksandra M. Chomakhidze

    2017-01-01

    Full Text Available The article describes the case of successful use of human monoclonal antibodies to the tumor necrosis factor- adalimumab in a patient with a severe course of juvenile arthritis associated with enthesitis and uveitis resistant to non-steroidal anti-inflammatory drugs, methotrexate, glucocorticosteroids that have been applied topically and retrobulbarly. After the first adalimumab administration, the pain syndrome was stopped, the duration of morning stiffness was significantly reduced, the laboratory indices of the disease activity were normalized by the 4th week of treatment, uveitis activity was stopped, the inflammatory changes in the joints regressed and the phase of inactive disease was ascertained by the 12th week. The duration of remission of articular syndrome and uveitis during treatment with adalimumab was 26 months. Adverse reactions were not registered during therapy.

  6. Anti-adalimumab antibodies in juvenile idiopathic arthritis-related uveitis.

    Science.gov (United States)

    Leinonen, Sanna T; Aalto, Kristiina; Kotaniemi, Kaisu M; Kivelä, Tero T

    2017-01-01

    To evaluate the association of adalimumab trough levels and anti-adalimumab antibodies with activity of uveitis in juvenile idiopathic arthritis-related uveitis. This was a retrospective observational case series in a clinical setting at the Department of Ophthalmology, Helsinki University Hospital, Finland in 2014-2016. Thirty-one paediatric patients with chronic anterior juvenile idiopathic arthritis-related uveitis in 58 eyes and who had been on adalimumab ≥6 months were eligible for the study. Uveitis activity during adalimumab treatment, adalimumab trough levels and anti-adalimumab antibody levels were recorded. Anti-adalimumab antibody levels ≥12 AU /ml were detected in nine patients (29%). This level of anti-adalimumab antibodies was associated with a higher grade of uveitis (puveitis that was not in remission (p=0.001) and with lack of concomitant methotrexate therapy (p=0.043). In patients with anti-adalimumab antibody levels uveitis (p=0.86). Adalimumab treatment might be better guided by monitoring anti-adalimumab antibody formation in treating JIA-related uveitis.

  7. Anti tumor necrosis factor - alpha adalimumab for complex regional pain syndrome type 1 (CRPS-I): a case series.

    Science.gov (United States)

    Eisenberg, Elon; Sandler, Ifat; Treister, Roi; Suzan, Erica; Haddad, May

    2013-11-01

    Evidence suggests tumor necrosis factor-alpha (TNF-α) mediates, at least in part, symptoms and signs in complex regional pain syndrome (CRPS). Here, we present a case series of patients with CRPS type 1, in whom the response to the anti-TNF-α adalimumab was assessed. Ten patients with CRPS type 1 were recruited. Assessments were performed before treatment, at 1 week, and 1, 3, and 6 months following 3 biweekly subcutaneous injections (40 mg/0.8 mL) adalimumab (Humira(®) ) and included the followings: Pain intensity using a 0-10 cm visual analog scale; the Short Form of the McGill Pain Questionnaire; the Beck Depression Inventory; the SF-36 questionnaire and mechanical and thermal thresholds (Von frey hair and Thermal Sensory Analyzer, respectively). In addition to the description of individual patient responses, both intention to treat (ITT) and per-protocol (PP) analyses were performed for the entire group. Three subgroups of patients were identified (3 patients in each): "nonresponders", "partial responders", and "robust responders" in whom improvement in almost all parameters was noted. Both the ITT and PP analyses demonstrated only a trend toward improvement in mechanical pain thresholds following treatment (ITT χ² = 13.83, P = 0.008; PP χ² = 10.29, P = 0.036). These results suggest adalimumab, and possibly other anti-TNF-α, can be potentially useful in some (although not in all) patients with CRPS type 1. These preliminary results along with the growing body of evidence which points to the involvement of TNF-α in the pathogenesis of CRPS justify further studies in this area. © 2013 World Institute of Pain.

  8. Association of Trabecular Bone Score with Inflammation and Adiposity in Patients with Psoriasis: Effect of Adalimumab Therapy

    Directory of Open Access Journals (Sweden)

    José L. Hernández

    2016-01-01

    Full Text Available Studies on trabecular bone score (TBS in psoriasis are lacking. We aim to assess the association between TBS and inflammation, metabolic syndrome features, and serum adipokines in 29 nondiabetic patients with psoriasis without arthritis, before and after 6-month adalimumab therapy. For that purpose, adjusted partial correlations and stepwise multivariable linear regression analysis were performed. No correlation was found between TBS and disease severity. TBS was negatively associated with weight, BMI, waist perimeter, fat percentage, and systolic and diastolic blood pressure before and after adalimumab. After 6 months of therapy, a negative correlation between TBS and insulin resistance (p=0.02 and leptin (p=0.01 and a positive correlation with adiponectin were found (p=0.01. The best set of predictors for TBS values at baseline were female sex (p=0.015, age (p=0.05, and BMI (p=0.001. The best set of predictors for TBS following 6 months of biologic therapy were age (p=0.001, BMI (p<0.0001, and serum adiponectin levels (p=0.027. In conclusion, in nondiabetic patients with moderate-to-severe psoriasis, TBS correlates with metabolic syndrome features and inflammation. This association is still present after 6 months of adalimumab therapy. Moreover, serum adiponectin levels seem to be an independent variable related to TBS values, after adalimumab therapy.

  9. Effectiveness of infliximab after adalimumab failure in Crohn's disease.

    Science.gov (United States)

    Chaparro, María; Andreu, Montserrat; Barreiro-de Acosta, Manuel; García-Planella, Esther; Ricart, Elena; Domènech, Eugeni; Esteve, María; Merino, Olga; Nos, Pilar; Peñalva, Mireia; Gisbert, Javier P

    2012-10-07

    To evaluate the effectiveness of infliximab as a second-line therapy in Crohn's disease patients after adalimumab failure. A historical cohort study in a community-based gastroenterology practice evaluated Crohn's disease patients treated with infliximab (induction plus maintenance) after adalimumab failure. Patients were identified using a large Spanish database (ENEIDA). We included 15 Crohn's disease patients who received infliximab after adalimumab failure. Five patients discontinued adalimumab due to loss of response, 3 due to adverse events and 7 due to partial response. After infliximab therapy was started, all patients who had interrupted adalimumab due to loss of efficacy regained response. All patients who discontinued adalimumab due to adverse events responded to infliximab and maintained this response; one of these patients had an uneventful course on infliximab, but 2 developed adverse events. None of the 7 patients who interrupted adalimumab due to partial response reached remission with infliximab. Switching from adalimumab to infliximab may be useful in patients who develop adverse effects or loss of response, however, the benefit of infliximab in primary nonresponders was not established.

  10. Adalimumab Injection

    Science.gov (United States)

    ... the armpits, groin, and anal area) in adults, uveitis (swelling and inflammation of different areas of the ... certain lab tests before, during, and after your treatment to check your body's response to adalimumab.Do ...

  11. CROHN'S DISEASE: GENERAL CHARACTERISTICS AND TREATMENT WITH ADALIMUMABE BIOPHARMACEUTICAL

    Directory of Open Access Journals (Sweden)

    S. A. Marques

    2018-02-01

    Full Text Available Crohn's Disease (CD is a chronic inflammatory disease that affects the gastrointestinal system. The etiology is not fully understood, however, genetic, immunological, microbiological and environmental factors are related to its genesis. The most common manifestations of this disease are diarrhea, abdominal pain, ulcers and fistulas. In the absence of adequate treatment it can evolve into extra intestinal complications and is also an important risk factor for colon and rectal cancer. The treatment of the disease is palliative and there is no cure for the disease, being considered only the period of remission of symptoms, as a good prognosis. The biopharmaceutical, Adalimumabe, produced by recombinant DNA technology has demonstrated efficacy in the treatment of this disease, as it prevents the action of TNF-α, a cytokine involved in inflammation and is abundant in individuals with CD. Although Adalimumabe has good results, its use leads to side effects that can be mild or fatal, such as the activation of tuberculosis.

  12. Influence of adalimumab treatment on anastomotic strength, degree of inflammation, and collagen formation

    DEFF Research Database (Denmark)

    Ploug, Thomas; Andersen, Kristian; Hansen, Katrine

    2013-01-01

    Adalimumab is a TNF-α inhibitor, which has gained wide use in the treatment of inflammatory bowel diseases. The potential detrimental effect of TNF-α inhibitors on postoperative complications such as anastomotic leakage is unknown. The aim of this study was to investigate the effect of a single...

  13. Adalimumab treatment for severe recalcitrant chronic plaque psoriasis.

    LENUS (Irish Health Repository)

    Ryan, C

    2012-02-01

    AIM: To assess the efficacy and safety profile of adalimumab in patients with severe, recalcitrant chronic plaque psoriasis, and to assess short-term overlapping of other systemic treatment with adalimumab to prevent flaring of disease. METHODS: This was a retrospective study comprising 39 patients with chronic plaque psoriasis treated with adalimumab between October 2005 and January 2008. All had failed treatment with other systemic agents, including biological therapies in 59% of patients. Patients were started on adalimumab 40 mg weekly or fortnightly, as clinically indicated. Severity of psoriasis was assessed by the Psoriasis Area and Severity Index (PASI). Therapeutic response was assessed by 75% improvement on PASI (PASI 75). All adverse events were recorded. RESULTS: Results were analysed separately for those treated with adalimumab only and those on combination treatment. PASI 75 was achieved in 38% (8 of 21 patients at week 16), 62% (13 of 21 patients) at week 24, 69% (9 of 13 patients) at week 48% and 71% (5 of 7 patients) at week 72 in the adalimumab-only group, compared with 56% (5 of 9 patients) at week 16, 50% (4 of 8 patients) at week 24, 80% (4 of 5 patients) at week 48% and 67% (2 of 3 patients) at week 72 in the combined group. Of the 39 patients, 15 (38%) achieved a PASI of 0 at some point in their treatment. Adalimumab was well tolerated; 38% of patients experienced side-effects, which were generally mild. CONCLUSION: Adalimumab was effective in a group of patients with psoriasis refractory to other systemic therapies, including biological treatments, and was well tolerated.

  14. Efficacy of adalimumab in young children with juvenile idiopathic arthritis and chronic uveitis: a case series.

    Science.gov (United States)

    La Torre, Francesco; Cattalini, Marco; Teruzzi, Barbara; Meini, Antonella; Moramarco, Fulvio; Iannone, Florenzo

    2014-05-24

    Juvenile idiopathic arthritis is a relatively common chronic disease of childhood, and is associated with persistent morbidity and extra-articular complications, one of the most common being uveitis. The introduction of biologic therapies, particularly those blocking the inflammatory mediator tumor necrosis factor-α, provided a new treatment option for juvenile idiopathic arthritis patients who were refractory to standard therapy such as non-steroidal anti-inflammatory drugs, corticosteroids and/or methotrexate. The first case was a 2-year-old girl with juvenile idiopathic arthritis and uveitis who failed to respond to treatment with anti-inflammatories, low-dose corticosteroids and methotrexate, and had growth retardation. Adalimumab 24 mg/m2 every 2 weeks and prednisone 0.5 mg/kg/day were added to methotrexate therapy; steroid tapering and withdrawal started after 1 month. After 2 months the patient showed good control of articular and ocular manifestations, and she remained in remission for 1 year, receiving adalimumab and methotrexate with no side effects, and showing significant improvement in growth. Case 2 was a 9-year-old boy with an 8-year history of juvenile idiopathic arthritis and uveitis that initially responded to infliximab, but relapse occurred after 2 years off therapy. After switching to adalimumab, and adjusting doses of both adalimumab and methotrexate based on body surface area, the patient showed good response and corticosteroids were tapered and withdrawn after 6 months; the patient remained in remission taking adalimumab and methotrexate. The final case was a 5-year-old girl with juvenile idiopathic arthritis for whom adalimumab was added to methotrexate therapy after three flares of uveitis. The patient had two subsequent episodes of uveitis that responded well to local therapy, but was then free of both juvenile idiopathic arthritis and uveitis symptoms, allowing methotrexate and then adalimumab to be stopped; the patient remained in drug

  15. Treatment of pediatric uveitis with adalimumab: the MERSI experience.

    Science.gov (United States)

    Castiblanco, Claudia; Meese, Halea; Foster, C Stephen

    2016-04-01

    To evaluate adalimumab therapy in children with uveitis. The electronic health records of pediatric patients diagnosed with uveitis and treated with adalimumab therapy were reviewed retrospectively. Demographic information, site and degree of intraocular inflammation, visual acuity, underlying systemic disorders, duration of therapy, side effects, and ability to obtain steroid-free remission were recorded. A total of 17 patients were included, 16 patients with anterior uveitis and 1 with panuveitis; 14 patients had bilateral disease. Juvenile idiopathic arthritis had been diagnosed in 14 patients, sarcoidosis in 1 patient, and idiopathic etiology in 2 patients. Of the 17 patients, 13 (about 77%) achieved steroid-free remission, and 4 did not. Six patients flared after discontinuation of adalimumab, with evidence of inflammation noted 3-7 months later. Adalimumab therapy was of 12-64 months' duration (mean, 36 months). At the time of initiation, 14 patients were using other agents concomitantly with adalimumab; 3 patients were on adalimumab monotherapy. At 1 year's follow-up, 12 patients were using combination therapy, and 3 patients were on adalimumab monotherapy: 11 patients had no evidence of inflammation. Side effects included pain at site of injection in 3 patients, anemia in 1 patient, and depression in 1 patient. In our study cohort, adalimumab was effective in inducing steroid-free remission. It was well tolerated, especially in combination with other immunomodulatory agents. The dosing and the interval can be adjusted to further improve inflammation control. Copyright © 2016 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.

  16. Adalimumab for the treatment of uveitis.

    Science.gov (United States)

    LaMattina, Kara C; Goldstein, Debra A

    2017-03-01

    Adalimumab, an inhibitor of tumor necrosis factor-alpha (TNFα), is the only systemic non-corticosteroid agent which has been approved by the US Food and Drug Administration (FDA) for the treatment of non-infectious uveitis. Areas covered: The aim of this review is to summarize the research which demonstrated the effectiveness of adalimumab in the treatment of intraocular inflammation and helped to establish its side effect profile, ultimately leading to its FDA approval. Expert commentary: Adalimumab is a useful second-line agent in the treatment of non-infectious uveitis. While it is only approved in the United States for use in intermediate, posterior, and panuveitis in adults, I find it to be effective in off-label treatment of pediatric uveitis and scleritis as well.

  17. Lemierre's Syndrome Complicating Pregnancy

    Directory of Open Access Journals (Sweden)

    M. Thompson

    2007-01-01

    Full Text Available Lemierre's syndrome is an anaerobic suppurative thrombophlebitis involving the internal jugular vein secondary to oropharyngeal infection. There is only one previous case report in pregnancy which was complicated by premature delivery of an infant that suffered significant neurological damage. We present an atypical case diagnosed in the second trimester with a live birth at term. By reporting this case, we hope to increase the awareness of obstetricians to the possibility of Lemierre's syndrome when patients present with signs of unabating oropharyngeal infection and pulmonary symptoms.

  18. Adalimumab-induced acute interstitial lung disease in a patient with rheumatoid arthritis

    Directory of Open Access Journals (Sweden)

    Olivia Meira Dias

    2014-01-01

    Full Text Available The use of immunobiological agents for the treatment of autoimmune diseases is increasing in medical practice. Anti-TNF therapies have been increasingly used in refractory autoimmune diseases, especially rheumatoid arthritis, with promising results. However, the use of such therapies has been associated with an increased risk of developing other autoimmune diseases. In addition, the use of anti-TNF agents can cause pulmonary complications, such as reactivation of mycobacterial and fungal infections, as well as sarcoidosis and other interstitial lung diseases (ILDs. There is evidence of an association between ILD and the use of anti-TNF agents, etanercept and infliximab in particular. Adalimumab is the newest drug in this class, and some authors have suggested that its use might induce or exacerbate preexisting ILDs. In this study, we report the first case of acute ILD secondary to the use of adalimumab in Brazil, in a patient with rheumatoid arthritis and without a history of ILD.

  19. Demonstration of Functional Similarity of Proposed Biosimilar ABP 501 to Adalimumab.

    Science.gov (United States)

    Velayudhan, Jyoti; Chen, Yuh-Feng; Rohrbach, Amanda; Pastula, Christina; Maher, Gwen; Thomas, Heather; Brown, Ryan; Born, Teresa L

    2016-08-01

    Due to the complex molecular structure and proprietary manufacturing processes of monoclonal antibodies (mAbs), differences in structure and function may be expected during development of biosimilar mAbs. Important regulatory requirements for approval of biosimilar products involve comprehensive assessments of any potential differences between proposed biosimilars and reference mAbs, including differences in all known mechanisms of action, using sensitive and relevant methods. Any identified structural differences should not result in differences in biofunctional or clinical activity. A comprehensive assessment comparing the Amgen biosimilar candidate ABP 501 with FDA-licensed adalimumab (adalimumab [US]) and EU-authorized adalimumab (adalimumab [EU]) was conducted to demonstrate similarity in biofunctional activity. The functional similarity assessment included testing of binding kinetics to soluble tumor necrosis factor α (TNFα) and relative binding to transmembrane TNFα. The neutralization of TNFα-induced caspase activation, TNFα- and lymphotoxin-α (LTα)-induced chemokine production, and cytotoxicity was also tested. Binding to Fc-gamma receptors FcγRIa, FcγRIIa (131H), FcγRIIIa (158V and 158F), and neonatal Fc receptor (FcRn) was compared with the reference mAbs, as was antibody-dependent cell-mediated cytotoxicity and complement-dependent cytotoxicity. The data demonstrate that ABP 501 is similar to both adalimumab (US) and adalimumab (EU) with respect to evaluated biofunctional activities. Similarity in biofunctional activity is a critical component of the totality of evidence required for demonstration of biosimilarity. The functional similarity demonstrated for ABP 501 comprehensively assesses the known mechanisms of action of adalimumab, supporting the conclusion that ABP 501, adalimumab (US), and adalimumab (EU) are likely to be clinically similar.

  20. Adalimumab for treating childhood plaque psoriasis: a clinical trial evaluation.

    Science.gov (United States)

    Di Lernia, Vito

    2017-12-01

    Most systemic therapies have not been systematically investigated in moderate to severe childhood plaque psoriasis. Evidence on the efficacy and safety of systemic treatments is limited and therapeutic guidelines are lacking. Recently adalimumab, a fully human monoclonal antibody that binds tumor necrosis factor (TNF)- alpha, was investigated in childhood psoriasis. Adalimumab is licensed for many inflammatory conditions including chronic plaque psoriasis in adults. Areas covered: A randomized phase III study published provided favourable efficacy and safety data of adalimumab in childhood psoriasis. The active comparator was methotrexate. After 16 weeks of treatment, a PASI 75 score was achieved in 58% of patients within the adalimumab 0.8 mg/kg group compared with 32% of patients within the methotrexate group. Safety data gave no evidence of drug-related serious adverse events and no organ toxicity. This is the first randomised controlled study of either adalimumab or methotrexate in children and adolescents with psoriasis. Expert opinion: The aforementioned trial was the first to provide clinical data on adalimumab's efficacy and safety in the short term when treating children and adolescents with psoriasis. Through the use of an active comparator, this study has opened the way for the future assessment of systemic therapies in children and adolescent with this condition.

  1. Efficacy and safety of adalimumab in ankylosing spondylitis

    Directory of Open Access Journals (Sweden)

    Mounach A

    2014-08-01

    Full Text Available Aziza Mounach, Abdellah El MaghraouiRheumatology Department, Military Hospital Mohammed V, Rabat, MoroccoAbstract: Ankylosing spondylitis (AS is the most common and most severe subtype of spondyloarthritis. It also may be an outcome of any of the other spondyloarthritis subtypes. AS preferentially affects the sacroiliac joints and the tip of the column, with a tendency to later ankylosis. Peripheral joints, enthesis, and other extra-articular involvement may be observed. Tumor necrosis factor (TNF inhibitors are now well-established, effective drugs in the treatment of AS symptoms. Adalimumab, which is a fully human monoclonal antibody that binds to and neutralizes TNF, has demonstrated efficacy in treating AS symptoms, including axial involvement, peripheral arthritis, enthesitis, uveitis, gut involvement, and psoriasis. Furthermore, adalimumab has showed an overall acceptable safety profile. In this paper, we review the efficacy and safety profile of adalimumab in the treatment of AS, and discuss its differences from the other anti-TNF drugs reported in the literature.Keywords: ankylosing spondylitis, spondyloarthritis, adalimumab, tumor necrosis factor-α

  2. Retrospective Cohort Study of Effectiveness and Safety of Adalimumab Use in Children with Juvenile Idiopathic Arthritis in the Republic of Bashkortostan

    Directory of Open Access Journals (Sweden)

    V. A. Maliyevskiy

    2015-01-01

    Full Text Available Background: Methotrexate is the first-line drug for treating patients with juvenile idiopathic arthritis (JIA. If it is ineffective or intolerable, prescription of genetically engineered biopharmaceuticals is indicated. Objective: The study was aimed at assessing effectiveness and safety of genetically engineered biopharmaceutical adalimumab for treating children with JIA. Methods: A retrospective cohort study was conducted to analyze results of treating patients with JIA aged 2–17 years. Adalimumab would be prescribed biweekly in the dose of 24 mg/m2 (body surface subcutaneously (if body weight is under 30 kg or in the dose of 40 mg/m2 (if body weight is > 30 kg. Effectiveness and safety would be assessed after 4–12–24–48–96 weeks. Results: We analyzed treatment results of 17 patients (15 children with active joint syndrome, 2 — with active uveitis. All patients with active joint syndrome had been receiving adalimumab for 12 weeks, 12 patients — for 24 weeks, 8 — for 48 weeks, 5 — for 96 weeks. 30/50/70% improvement in terms of the ACRpedi criteria was observed in 15/11/4 children after 4 weeks, after 12 weeks — in 15/13/11 patients, after 48 weeks — in 7/6/6 patients. The status of inactive disease was established in 5 patients (33% after 12 weeks, after 24 weeks — in 9 children (75%, after 48 weeks — in 7 children (70%, after 96 weeks — in 4 (80% children. Active uveitis was terminated in all 5 patients with signs of eye damage in the treatment onset. 1 patient suffered from exacerbation of the disease after 48 weeks of therapy; the drug was withdrawn. Tubercular infection without local manifestations was established in 1 patient after 96 weeks (positive Mantoux test, papule — 10 mm. Adalimumab injection was terminated for the period of chemotherapy. Conclusion: Adalimumab has a sufficiently high effectiveness and safety for long-term (up to 2 years treatment of children with JIA. 

  3. Adalimumab treatment in patients with rheumatoid arthritis with renal insufficiency.

    Science.gov (United States)

    Sumida, Keiichi; Ubara, Yoshifumi; Suwabe, Tatsuya; Hayami, Noriko; Hiramatsu, Rikako; Hasegawa, Eiko; Yamanouchi, Masayuki; Hoshino, Junichi; Sawa, Naoki; Takaichi, Kenmei

    2013-03-01

    To elucidate the safety of adalimumab for rheumatoid arthritis (RA) patients with renal insufficiency, including those with end-stage renal disease undergoing hemodialysis. Sixty-five RA patients, including 2 patients undergoing hemodialysis, treated with adalimumab in our hospital from December 1, 2008 to June 30, 2011 were retrospectively analyzed. Renal function was evaluated by the estimated glomerular filtration rate (eGFR) calculated from the Cockcroft-Gault formula at the start and end of followup after adalimumab treatment. The proportion of the patients who discontinued or switched adalimumab treatment and the change of the eGFR were compared between patients with (n = 39) and without (n = 26) renal insufficiency, defined as an eGFR patients who discontinued or switched adalimumab treatment (51.3% versus 50.0%; P = 0.53). The mean ± SD changes of eGFR were from 41.6 ± 13.3 to 43.4 ± 17.9 ml/minute/1.73 m(2) in patients with renal insufficiency and from 83.6 ± 17.5 to 83.0 ± 16.8 ml/minute/1.73 m(2) in patients without renal insufficiency, and the differences in each group were not statistically significant (P = 0.92 and P = 0.78, respectively). No severe infections or other severe adverse events were observed in either group during adalimumab treatment. Our data indicate that adalimumab does not worsen renal function and has no serious adverse events even for RA patients with renal insufficiency, including those undergoing hemodialysis, and suggest that it could be a potential therapeutic option for them. Copyright © 2013 by the American College of Rheumatology.

  4. A Rare Side Effect due to TNF-Alpha Blocking Agent: Acute Pleuropericarditis with Adalimumab

    Directory of Open Access Journals (Sweden)

    Hakan Ozkan

    2013-01-01

    Full Text Available Tumor necrosis factor-alpha antagonism is an important treatment strategy in patients with rheumatoid arthritis, psoriatic arthritis, vasculitis, and ankylosing spondylitis. Adalimumab is one of the well-known tumor necrosis factor-alpha blocking agents. There are several side effects reported in patients with adalimumab therapy. Cardiac side effects of adalimumab are rare. Only a few cardiac side effects were reported. A 61-year-old man treated with adalimumab for the last 6 months due to psoriatic arthritis presented with typically acute pleuropericarditis. Chest X-ray and echocardiography demonstrated marked pericardial effusion. Patient was successfully evaluated for the etiology of acute pleuro-pericarditis. Every etiology was excluded except the usage of adalimumab. Adalimumab was discontinued, and patient was treated with 1200 mg of ibuprofen daily. Control chest X-ray and echocardiography after three weeks demonstrated complete resolution of both pleural and pericardial effusions. This case clearly demonstrated the acute onset of pericarditis with adalimumab usage. Acute pericarditis and pericardial effusion should be kept in mind in patients with adalimumab treatment.

  5. Adalimumab plus Methotrexate for Uveitis in Juvenile Idiopathic Arthritis.

    Science.gov (United States)

    Ramanan, Athimalaipet V; Dick, Andrew D; Jones, Ashley P; McKay, Andrew; Williamson, Paula R; Compeyrot-Lacassagne, Sandrine; Hardwick, Ben; Hickey, Helen; Hughes, Dyfrig; Woo, Patricia; Benton, Diana; Edelsten, Clive; Beresford, Michael W

    2017-04-27

    Adalimumab, a fully human anti-tumor necrosis factor α monoclonal antibody, is effective in the treatment of juvenile idiopathic arthritis (JIA). We tested the efficacy of adalimumab in the treatment of JIA-associated uveitis. In this multicenter, double-blind, randomized, placebo-controlled trial, we assessed the efficacy and safety of adalimumab in children and adolescents 2 years of age or older who had active JIA-associated uveitis. Patients who were taking a stable dose of methotrexate were randomly assigned in a 2:1 ratio to receive either adalimumab (at a dose of 20 mg or 40 mg, according to body weight) or placebo, administered subcutaneously every 2 weeks. Patients continued the trial regimen until treatment failure or until 18 months had elapsed. They were followed for up to 2 years after randomization. The primary end point was the time to treatment failure, defined according to a multicomponent intraocular inflammation score that was based on the Standardization of Uveitis Nomenclature criteria. The prespecified stopping criteria were met after the enrollment of 90 of 114 patients. We observed 16 treatment failures in 60 patients (27%) in the adalimumab group versus 18 treatment failures in 30 patients (60%) in the placebo group (hazard ratio, 0.25; 95% confidence interval [CI], 0.12 to 0.49; Ptreatment failure than placebo among children and adolescents with active JIA-associated uveitis who were taking a stable dose of methotrexate. Patients who received adalimumab had a much higher incidence of adverse events and serious adverse events than those who received placebo. (Funded by the NIHR Health Technology Assessment Programme and Arthritis Research UK; SYCAMORE EudraCT number, 2010-021141-41 .).

  6. Rare Complications of Cervical Spine Surgery: Horner's Syndrome.

    Science.gov (United States)

    Traynelis, Vincent C; Malone, Hani R; Smith, Zachary A; Hsu, Wellington K; Kanter, Adam S; Qureshi, Sheeraz A; Cho, Samuel K; Baird, Evan O; Isaacs, Robert E; Rahman, Ra'Kerry K; Polevaya, Galina; Smith, Justin S; Shaffrey, Christopher; Tortolani, P Justin; Stroh, D Alex; Arnold, Paul M; Fehlings, Michael G; Mroz, Thomas E; Riew, K Daniel

    2017-04-01

    A multicenter retrospective case series. Horner's syndrome is a known complication of anterior cervical spinal surgery, but it is rarely encountered in clinical practice. To better understand the incidence, risks, and neurologic outcomes associated with Horner's syndrome, a multicenter study was performed to review a large collective experience with this rare complication. We conducted a retrospective multicenter case series study involving 21 high-volume surgical centers from the AOSpine North America Clinical Research Network. Medical records for 17 625 patients who received subaxial cervical spine surgery from 2005 to 2011 were reviewed to identify occurrence of 21 predefined treatment complications. Descriptive statistics were provided for baseline patient characteristics. Paired t test was used to analyze changes in clinical outcomes at follow-up compared to preoperative status. In total, 8887 patients who underwent anterior cervical spine surgery at the participating institutions were screened. Postoperative Horner's syndrome was identified in 5 (0.06%) patients. All patients experienced the complication following anterior cervical discectomy and fusion. The sympathetic trunk appeared to be more vulnerable when operating on midcervical levels (C5, C6), and most patients experienced at least a partial recovery without further treatment. This collective experience suggests that Horner's syndrome is an exceedingly rare complication following anterior cervical spine surgery. Injury to the sympathetic trunk may be limited by maintaining a midline surgical trajectory when possible, and performing careful dissection and retraction of the longus colli muscle when lateral exposure is necessary, especially at caudal cervical levels.

  7. Assessing Analytical Similarity of Proposed Amgen Biosimilar ABP 501 to Adalimumab.

    Science.gov (United States)

    Liu, Jennifer; Eris, Tamer; Li, Cynthia; Cao, Shawn; Kuhns, Scott

    2016-08-01

    ABP 501 is being developed as a biosimilar to adalimumab. Comprehensive comparative analytical characterization studies have been conducted and completed. The objective of this study was to assess analytical similarity between ABP 501 and two adalimumab reference products (RPs), licensed by the United States Food and Drug Administration (adalimumab [US]) and authorized by the European Union (adalimumab [EU]), using state-of-the-art analytical methods. Comprehensive analytical characterization incorporating orthogonal analytical techniques was used to compare products. Physicochemical property comparisons comprised the primary structure related to amino acid sequence and post-translational modifications including glycans; higher-order structure; primary biological properties mediated by target and receptor binding; product-related substances and impurities; host-cell impurities; general properties of the finished drug product, including strength and formulation; subvisible and submicron particles and aggregates; and forced thermal degradation. ABP 501 had the same amino acid sequence and similar post-translational modification profiles compared with adalimumab RPs. Primary structure, higher-order structure, and biological activities were similar for the three products. Product-related size and charge variants and aggregate and particle levels were also similar. ABP 501 had very low residual host-cell protein and DNA. The finished ABP 501 drug product has the same strength with regard to protein concentration and fill volume as adalimumab RPs. ABP 501 and the RPs had a similar stability profile both in normal storage and thermal stress conditions. Based on the comprehensive analytical similarity assessment, ABP 501 was found to be similar to adalimumab with respect to physicochemical and biological properties.

  8. Complications of Cushing's syndrome: state of the art.

    Science.gov (United States)

    Pivonello, Rosario; Isidori, Andrea M; De Martino, Maria Cristina; Newell-Price, John; Biller, Beverly M K; Colao, Annamaria

    2016-07-01

    Cushing's syndrome is a serious endocrine disease caused by chronic, autonomous, and excessive secretion of cortisol. The syndrome is associated with increased mortality and impaired quality of life because of the occurrence of comorbidities. These clinical complications include metabolic syndrome, consisting of systemic arterial hypertension, visceral obesity, impairment of glucose metabolism, and dyslipidaemia; musculoskeletal disorders, such as myopathy, osteoporosis, and skeletal fractures; neuropsychiatric disorders, such as impairment of cognitive function, depression, or mania; impairment of reproductive and sexual function; and dermatological manifestations, mainly represented by acne, hirsutism, and alopecia. Hypertension in patients with Cushing's syndrome has a multifactorial pathogenesis and contributes to the increased risk for myocardial infarction, cardiac failure, or stroke, which are the most common causes of death; risks of these outcomes are exacerbated by a prothrombotic diathesis and hypokalaemia. Neuropsychiatric disorders can be responsible for suicide. Immune disorders are common; immunosuppression during active disease causes susceptibility to infections, possibly complicated by sepsis, an important cause of death, whereas immune rebound after disease remission can exacerbate underlying autoimmune diseases. Prompt treatment of cortisol excess and specific treatments of comorbidities are crucial to prevent serious clinical complications and reduce the mortality associated with Cushing's syndrome. Copyright © 2016 Elsevier Ltd. All rights reserved.

  9. [Refeeding syndrome in geriatric patients : A frequently overlooked complication].

    Science.gov (United States)

    Wirth, Rainer; Diekmann, Rebecca; Fleiter, Olga; Fricke, Leonhardt; Kreilkamp, Annika; Modreker, Mirja Katrin; Marburger, Christian; Nels, Stefan; Schaefer, Rolf; Willschrei, Heinz-Peter; Volkert, Dorothee

    2018-01-01

    The refeeding syndrome is a life-threatening complication that can occur after initiation of a nutrition therapy in malnourished patients. If the risk factors and pathophysiology are known, the refeeding syndrome can effectively be prevented and treated, if recognized early. A slow increase of food intake and the close monitoring of serum electrolyte levels play an important role. Because the refeeding syndrome is not well known and the symptoms may vary extremely, this complication is poorly recognized, especially against the background of geriatric multimorbidity. This overview is intended to increase the awareness of the refeeding syndrome in the risk group of geriatric patients.

  10. Long-term use of adalimumab in the treatment of rheumatic diseases

    Directory of Open Access Journals (Sweden)

    Charalampos Papagoras

    2009-05-01

    Full Text Available Charalampos Papagoras, Paraskevi V Voulgari, Alexandros A DrososRheumatology Clinic, Department of Internal Medicine, Medical School, University of Ioannina, Ioannina, GreeceAbstract: Adalimumab, a fully humanized monoclonal antibody against tumor necrosis factor-alpha (TNFα, has been evaluated in various randomized placebo-controlled trials in rheumatoid arthritis, ankylosing spondylitis, psoriatic arthritis and juvenile idiopathic arthritis. In the short time frame of these trials adalimumab has been shown to be effective in reducing disease activity, slowing radiographic disease progression and improving patients’ quality of life, while at the same time demonstrating an acceptable safety profile. Furthermore, release of adalimumab on the market, prospective observational studies, as well as open-label extensions of the original double-blind trials have provided experience and data about the long-term efficacy and safety of the drug. Initial effectiveness, in terms of reducing disease activity, is sustained, while in most cases patients treated with adalimumab experienced a slower radiographic progression and consequently less disability and improved health-related quality-of-life outcomes. Moreover, long-standing treatment of thousands of patients with adalimumab outside the controlled context of clinical trials was not related to new safety signals, with the most common adverse events being respiratory infections. The most common serious adverse events seem to be tuberculosis reactivation, while a putative association with malignant lymphoma development is not yet proven. Besides, both of these adverse reactions pertain to the whole TNFα blocker group. In conclusion, adalimumab is a safe and effective option for the treatment of patients with rheumatoid arthritis, ankylosing spondylitis, psoriatic arthritis and juvenile idiopathic arthritis. Keywords: adalimumab, tumor necrosis factor-alpha, rheumatoid arthritis, ankylosing spondylitis

  11. Lethality of patients with rheumatoid arthritis depending on adalimumab administration: imitation modeling

    Directory of Open Access Journals (Sweden)

    D V Goryachev

    2009-01-01

    Full Text Available Lethality of pts with rheumatoid arthritis (RA exceeds mortality values in general population. Possibility of disease modifying anti-rheumatic drugs (DMARD influence on RA pts lethality has been widely discussed lately in scientific works. Objective. To determine possible lethality diminishment in Russian population of RA pts with one of biological drugs TNFα antagonist adalimumab. Material and methods. Model construction is based on the fact of lethality dependence on pt functional state assessed by HAQ. Model simulating progression of functional disability in pts with RA visiting medical institutions of Russia was made (RAISER study. 3 model variants for imitation of consecutive change of DMARDs including adalimumab were done. First consecution assessed DMARD change in the next chain: adalimumab-methotrexate-sulfasalazine-leflunomide-azathioprine-cyclosporine-palliative therapy. Second consecution: adalimumab administration after failure of first 3 DMARDs. Third consecution considered only change of synthetic DMARDs without adalimumab inclusion. Model imitated participation of 3000 pts in every consecution. Prognosis horizon was 12 years. Age of pts and initial HAQ distribution were get from results of epidemiological RAISER study. Calculation was done on the base of elevation of standardized lethality level (SLL in population of RA pts in average from 135% to 300%. SLL values from 80 to 320% were used depending on functional disability degree with converting to Russian values of age-specific lethality coefficient for 1999. Results. Lethality in treatment consecutions including adalimumab was significantly lower. To the end of 12th year in group not using adalimumab, using it at once and using it after 376 DMARDs respectively 65,1%, 71,6% and 71,1% of pts were still alive. Conclusion. Significant decrease of lethality with adalimumab inclusion in consecution of DMARD change during treatment of RA pts was demonstrated with imitation modeling

  12. Cytotoxicity and genotoxicity of intravitreal adalimumab administration in rabbit retinal cells

    Directory of Open Access Journals (Sweden)

    Álcio Coutinho de Paula

    2015-04-01

    Full Text Available Purpose: To assess the cytotoxicity and genotoxicity of intravitreal adalimumab treatment in an animal experimental model using cytological and molecular techniques. Methods: Eighteen rabbits were randomly assigned to three groups: control, adalimumab treatment, and placebo. Cytotoxicity on retinal cells was evaluated using flow cytometry assays to determine the level of apoptosis and necrosis. Genotoxicity was evaluated by comet assays to assess DNA damage, and quantitative real-time polymerase chain reaction (qPCR was used to evaluate expression of apoptosis-inducing caspases (8 and 3. Results: No cytotoxicity or genotoxicity was observed in any of the two treatment groups (adalimumab and placebo following intravitreal administration compared with the control group. Flow cytometry analysis revealed that more than 90% of the cells were viable, and only a low proportion of retinal cells presented apoptotic (~10% or necrotic (<1% activity across all groups. Molecular damage was also low with a maximum of 6.4% DNA degradation observed in the comet assays. In addition, no increase in gene expression of apoptosis-inducing caspases was observed on retinal cells by qPCR in both the adalimumab and placebo groups compared with the control group. Conclusion: The use of adalimumab resulted in no detectable cytotoxicity or genotoxicity on retinal cells for up to 60 days upon administration. These results therefore indicate that adalimumab may be a safe option for intravitreal application to treat ocular inflammatory diseases in which TNF-α is involved.

  13. Profile of adalimumab and its potential in the treatment of uveitis

    Directory of Open Access Journals (Sweden)

    Balevic SJ

    2016-09-01

    Full Text Available Stephen J Balevic, C Egla Rabinovich Department of Pediatric Rheumatology, Duke University Medical Center, Durham, NC, USA Abstract: Uveitis refers to the presence of intraocular inflammation, and as a strict definition compromises the iris and ciliary body anteriorly and the choroid posteriorly (the uvea. Untreated, uveitis can lead to visual loss or blindness. The etiology of uveitis can include both infectious and noninfectious (usually immune-mediated causes, the latter of which are often mediated predominantly by Th1 CD4+ T-cells that secrete proinflammatory cytokines. Tumor necrosis factor-alpha (TNF-α is a proinflammatory cytokine involved in the pathogenesis of uveitis, which at high concentrations can cause excess inflammation and tissue damage. Adalimumab is a recombinant human IgG1 monoclonal antibody specific for human TNF-α. Historically, corticosteroids and methotrexate were used to treat uveitis; however, newer biologic agents such as adalimumab have revolutionized therapy for noninfectious uveitis. Adalimumab has shown efficacy in treating refractory uveitis in multiple settings, including idiopathic disease, juvenile idiopathic arthritis, sarcoidosis, Behçets disease, and uveitis secondary to spondyloarthropathies, among several other noninfectious uveitis conditions. In this paper, we will review the profile of adalimumab, the role of TNF-α in uveitis, discuss safety data, and summarize key articles evaluating the efficacy of adalimumab in treating uveitis secondary to the most commonly associated autoimmune diseases.Keywords: uveitis, autoimmune disease, adalimumab, TNF-α

  14. Development of sarcoidosis during adalimumab therapy for chronic plaque psoriasis.

    Science.gov (United States)

    Marcella, Stefanie; Welsh, Belinda; Foley, Peter

    2011-08-01

    A 38-year-old woman developed clinical, biochemical, radiological and histopathological evidence of cutaneous and pulmonary sarcoidosis 5 months after commencing adalimumab for chronic plaque psoriasis. Signs and symptoms resolved within 3 months of cessation of adalimumab. © 2010 The Authors. Australasian Journal of Dermatology © 2010 The Australasian College of Dermatologists.

  15. Grisel's syndrome: A rare complication following traditional uvulectomy

    African Journals Online (AJOL)

    A case is reported of an eight-month-old female patient who had traditional uvulectomy for sore throat complicated by Grisel's syndrome. She was admitted into the hospital one week after uvulectomy with Torticolis. Grisel's syndrome is a nontraumatic atlantoaxial subluxation, usually secondary of an infection or an ...

  16. Profile of adalimumab and its potential in the treatment of uveitis.

    Science.gov (United States)

    Balevic, Stephen J; Rabinovich, C Egla

    2016-01-01

    Uveitis refers to the presence of intraocular inflammation, and as a strict definition compromises the iris and ciliary body anteriorly and the choroid posteriorly (the uvea). Untreated, uveitis can lead to visual loss or blindness. The etiology of uveitis can include both infectious and noninfectious (usually immune-mediated) causes, the latter of which are often mediated predominantly by Th1 CD4 + T-cells that secrete proinflammatory cytokines. Tumor necrosis factor-alpha (TNF-α) is a proinflammatory cytokine involved in the pathogenesis of uveitis, which at high concentrations can cause excess inflammation and tissue damage. Adalimumab is a recombinant human IgG1 monoclonal antibody specific for human TNF-α. Historically, corticosteroids and methotrexate were used to treat uveitis; however, newer biologic agents such as adalimumab have revolutionized therapy for noninfectious uveitis. Adalimumab has shown efficacy in treating refractory uveitis in multiple settings, including idiopathic disease, juvenile idiopathic arthritis, sarcoidosis, Behçets disease, and uveitis secondary to spondyloarthropathies, among several other noninfectious uveitis conditions. In this paper, we will review the profile of adalimumab, the role of TNF-α in uveitis, discuss safety data, and summarize key articles evaluating the efficacy of adalimumab in treating uveitis secondary to the most commonly associated autoimmune diseases.

  17. Rare Complications of Cervical Spine Surgery: Horner’s Syndrome

    Science.gov (United States)

    Malone, Hani R.; Smith, Zachary A.; Hsu, Wellington K.; Kanter, Adam S.; Qureshi, Sheeraz A.; Cho, Samuel K.; Baird, Evan O.; Isaacs, Robert E.; Rahman, Ra’Kerry K.; Polevaya, Galina; Smith, Justin S.; Shaffrey, Christopher; Tortolani, P. Justin; Stroh, D. Alex; Arnold, Paul M.; Fehlings, Michael G.; Mroz, Thomas E.; Riew, K. Daniel

    2017-01-01

    Study Design: A multicenter retrospective case series. Objective: Horner’s syndrome is a known complication of anterior cervical spinal surgery, but it is rarely encountered in clinical practice. To better understand the incidence, risks, and neurologic outcomes associated with Horner’s syndrome, a multicenter study was performed to review a large collective experience with this rare complication. Methods: We conducted a retrospective multicenter case series study involving 21 high-volume surgical centers from the AOSpine North America Clinical Research Network. Medical records for 17 625 patients who received subaxial cervical spine surgery from 2005 to 2011 were reviewed to identify occurrence of 21 predefined treatment complications. Descriptive statistics were provided for baseline patient characteristics. Paired t test was used to analyze changes in clinical outcomes at follow-up compared to preoperative status. Results: In total, 8887 patients who underwent anterior cervical spine surgery at the participating institutions were screened. Postoperative Horner’s syndrome was identified in 5 (0.06%) patients. All patients experienced the complication following anterior cervical discectomy and fusion. The sympathetic trunk appeared to be more vulnerable when operating on midcervical levels (C5, C6), and most patients experienced at least a partial recovery without further treatment. Conclusions: This collective experience suggests that Horner’s syndrome is an exceedingly rare complication following anterior cervical spine surgery. Injury to the sympathetic trunk may be limited by maintaining a midline surgical trajectory when possible, and performing careful dissection and retraction of the longus colli muscle when lateral exposure is necessary, especially at caudal cervical levels. PMID:28451480

  18. Long-term treatment of rheumatoid arthritis with adalimumab

    Directory of Open Access Journals (Sweden)

    Murdaca G

    2013-05-01

    Full Text Available Giuseppe Murdaca, Francesca Spanò, Francesco PuppoDepartment of Internal Medicine, Clinical Immunology Unit, University of Genoa, Genoa, ItalyAbstract: Rheumatoid arthritis (RA is a chronic inflammatory disease that is associated with joint damage and progressive disability, an increased risk of morbidity related to comorbid conditions and substantial socioeconomic costs. Tumor necrosis factor-alpha (TNF-α is a proinflammatory cytokine known to have a central role in the initial host response to infection and in the pathogenesis of various immune-mediated diseases, such as RA, ankylosing spondylitis, psoriasis and/or psoriatic arthritis, Crohn’s disease, and systemic lupus erythematosus. Five TNF-α inhibitors are available for the clinical use: infliximab; adalimumab; etanercept; golimumab; and certolizumab pegol. Infliximab is a chimeric human/murine IgG1 monoclonal antibody (mAb; adalimumab, and golimumab are human mAbs; certolizumab pegol is composed of the fragment antigen-binding anti-binding domain of a humanized anti-TNF-α mAb, combined with polyethylene glycol to increase its half-life in the body; etanercept is a fusion protein that acts as a “decoy receptor” for TNF-α. In this paper, we will briefly review the current data on efficacy and safety of adalimumab in patients with RA, its potential beneficial effects upon comorbid conditions, such as endothelial dysfunction and accelerated atherosclerosis in RA, and the immunogenicity.Keywords: adalimumab, efficacy, safety, rheumatoid arthritis, VEGF, immunogenicity, infections

  19. Lupus-Negative Libman-Sacks Endocarditis Complicated by Catastrophic Antiphospholipid Syndrome.

    Science.gov (United States)

    Murtaza, Ghulam; Iskandar, Joy; Humphrey, Tara; Adhikari, Sujeen; Kuruvilla, Aneesh

    2017-04-01

    Libman-Sacks endocarditis is characterized by sterile and verrucous lesions that predominantly affect the aortic and mitral valves. In most cases, patients do not have significant valvular dysfunction. However, patients with significant valvular dysfunction may present with serious complications such as cardiac failure, arrhythmias, and thromboembolic events. Recently, association of Libman-Sacks endocarditis with antiphospholipid antibody syndrome (APS) has been made. APS is most commonly defined by venous and arterial thrombosis, recurrent pregnancy loss, and thrombocytopenia. While the syndrome can be a primary syndrome, it is usually secondary to systemic lupus erythematosus. Catastrophic antiphospholipid syndrome (CAPS) can be a life-threatening presentation of APS and can occur in 1% of patients with antiphospholipid syndrome. We present a very rare case of a young female patient with lupus-negative Libman-Sacks endocarditis complicated by CAPS.

  20. Compartment syndrome and popliteal vascular injury complicating unicompartmental knee arthroplasty

    NARCIS (Netherlands)

    Kort, Nanne Pieter; Van Raay, Jos J. J. A. M.; van Horn, Jim R.

    Popliteal vascular injury and the compartment syndrome of the leg are rare but important complications of knee arthroplasties. Early diagnosis and treatment are of paramount importance in preventing the devastating complications of these conditions. To our knowledge, these complications have not

  1. Arterial complications of vascular Ehlers-Danlos syndrome.

    Science.gov (United States)

    Eagleton, Matthew J

    2016-12-01

    Vascular Ehlers-Danlos syndrome (EDS) is a relatively rare genetic syndrome that occurs owing to disorders in the metabolism of fibrillary collagen. These defects affect the soft connective tissues resulting in abnormalities in the skin, joints, hollow organs, and blood vessels. Patients with these defects frequently present at a young age with spontaneous arterial complications involving the medium-sized arteries. Complications involving the hollow organs, such as spontaneous colonic perforation, are observed as well. Given the fragility of the soft tissue, open and endovascular intervention on patients with vascular EDS is fraught with high complication rates. A PubMed search was performed to identify manuscripts published related to vascular EDS. This search included more than 747 articles. These findings were cross-referenced using key terms, including endovascular, embolization, surgery, genetics, pathophysiology, connective tissue disorders, vascular complications, systematic review, type III collagen, and COL3A1. The references in key articles and review articles were evaluated for additional resources not identified in the PubMed search. Care must be taken to balance the risk of intervention vs the risk of continued observation. Life-threatening hemorrhage, however, mandates intervention. With careful, altered approaches to tissue handling, endovascular approaches may provide a safer option for managing the arterial complications observed in patients with vascular EDS. Additional hope may also be found in the use of pharmacologic agents that reduce the incidence and severity of the arterial complications. Copyright © 2016 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.

  2. Technology evaluation: adalimumab, Abbott laboratories.

    Science.gov (United States)

    Lorenz, Hanns M

    2002-04-01

    Adalimumab (D2E7), a human monoclonal antibody that binds to and neutralizes TNFa, is being developed by Abbott (formerly Knoll), under license from Cambridge Antibody Technology (CAT), for the potential treatment of inflammatory disorders such as rheumatoid arthritis (RA) and Crohn's disease. It is also being investigated for the potential treatment of coronary heart disease. Phase II studies for Crohn's disease and phase III for RA were ongoing throughout 2001. Limited data are only available for RA. In January 2002, it was reported that phase III trials of adalimumab for RA had been completed, but details have not been published in the primary literature so far. At this time CAT and Abbott expected to file for US approval in the second quarter of 2002 with a launch date anticipated for 2003. Phase III data are expected to be presented at the European League Against Rheumatism meeting in June 2002. In November 2000, Lehman Brothers predicted a US launch in June 2002 with peak US sales of $600 million in 2007 and a launch in non-US markets in 2003 with peak sales in these markets of $300 million in 2008. In December 2000, Merrill Lynch predicted regulatory clearance in the second half of 2003. The probability of adalimumab reaching the market is estimated to be 70%. In December 2000, Merrill Lynch predicted a 2003 launch, with estimated sales of pounds sterling 3.65 million in that year rising to pounds sterling 30.14 million in 2010. In March 2001, ABN AMRO predicted sales of $73 million in 2003 rising to $392 million in 2007.

  3. Concurrent immunomodulator therapy is associated with higher adalimumab trough levels during scheduled maintenance therapy.

    Science.gov (United States)

    Bond, Ashley; Dodd, Susanna; Fisher, Gareth; Skouras, Thomas; Subramanian, Sreedhar

    2017-02-01

    Combination therapy with infliximab and immunomodulators is superior to monotherapy, resulting in better outcomes and higher trough levels of infliximab. The role of concurrent immunomodulatory therapy on adalimumab trough levels has not been adequately investigated. We evaluated the impact of concomitant immunomodulation on adalimumab trough levels in patients on scheduled maintenance therapy. We conducted a prospective observational, cross-sectional study of all inflammatory bowel disease patients on maintenance therapy who had adalimumab trough levels measured between January 2013 and January 2016. Drug level and anti-drug antibody measurements were performed on sera using a solid phase assay. Pairwise comparison of means was used to compare trough levels in patients with and without concomitant immune modulator therapy. In total, 79 patients were included. Twenty-three patients (29.1%) were on weekly dosing whereas 56 (70.9%) were on alternate weeks. Median adalimumab trough levels were comparable in patients with and without clinical remission (6.8 μg/ml (IQR 5.6-8.1) versus 6.7 μg/ml (IQR 3.9-8.1), respectively. Patients with an elevated faecal calprotectin >250 μg/g had lower adalimumab trough levels (median 6.7, IQR 3.9-8) compared to patients with faecal calprotectin <250 μg/g (median 7.7, IQR 6.1-8.1) though this did not achieve statistical significance (p = .062). Median adalimumab trough levels among patients on concurrent immunomodulators was 7.2 μg/ml (IQR 5.7-8.1) compared to those not on concurrent immunomodulator, 6.1 μg/ml (IQR 2.7-7.7, p = .0297). Adalimumab trough levels were significantly higher in patients on concurrent immunomodulators during maintenance therapy. There was a trend towards a lower adalimumab trough level in patients with elevated calprotectin.

  4. Antiphospholipid antibody syndrome complicated by Grave's disease.

    Science.gov (United States)

    Takahashi, Ayumi; Tamura, Atsushi; Ishikawa, Osamu

    2002-12-01

    The report describes a woman with primary antiphospholipid antibody syndrome complicated with Grave's disease. Developing symptoms included a small cutaneous nodule on her finger and subsequently ecchymotic purpura on the cheeks, ears, buttocks and lower legs. Histological examinations showed thrombosed vessels in the dermis without or with hemorrhage, respectively. Laboratory investigation revealed positive lupus anticoagulant and immunogenic hyperthyroidism due to Grave's disease. There is a close relationship between the cutaneous manifestation of antiphospholipid antibody syndrome and the activities of Grave's disease and a possible link of antiphospholipid antibody syndrome with Grave's disease was suggested both by the etiology of the disease as well as the disease activity.

  5. Cushing's syndrome complicated by multiple opportunistic infections

    NARCIS (Netherlands)

    Bakker, R. C.; Gallas, P. R.; Romijn, J. A.; Wiersinga, W. M.

    1998-01-01

    The case history of a 56-year-old man is described who suffered from severe adrenocorticotrophic hormone (ACTH)-dependent Cushing's syndrome. The clinical course was complicated by simultaneous infections with Pneumocystis carinii, Staphylococcus aureus, Candida albicans, Aspergillus fumigatus and

  6. Targeting Tumor Necrosis Factor-α with Adalimumab: Effects on Endothelial Activation and Monocyte Adhesion.

    Directory of Open Access Journals (Sweden)

    Raghav Oberoi

    Full Text Available It is well known that atherosclerotic inflammatory vascular disease is critically driven by oxidized lipids and cytokines. In this regard, tumor necrosis factor (TNF-α is known as a crucial mediator of early pro-atherosclerotic events. Epidemiologic data suggest that blockade of TNF-α has beneficial effects on vascular outcomes in patients with rheumatoid arthritis, however, detailed mechanistic studies are still lacking. This study aims to elucidate effects of TNF-α blockade by adalimumab-which is approved for several inflammatory disorders-on endothelial activation and monocyte adhesion under pro-atherosclerotic conditions.Phorbol myristate acetate (PMA differentiated THP-1 macrophages were stimulated with oxidized low density lipoprotein and subsequent analysis of this conditioned media (oxLDL CM revealed a strong release of TNF-α. The TNF-α rich supernatant led to activation of human umbilical vein endothelial cells (HUVEC as shown by enhanced expression of major adhesion molecules, such as vascular cell adhesion molecule-1 (VCAM-1, intercellular adhesion molecule-1 (ICAM-1 and E-selectin which was suppressed by the TNF-α inhibitor adalimumab. Accordingly, adalimumab effectively prevented THP-1 monocyte adhesion to endothelial cells under static as well as under flow conditions. Furthermore, adalimumab suppressed endothelial leakage as shown by Evan's blue diffusion across a confluent endothelial monolayer. Of note, after intraperitoneal injection we detected abundant deposition of fluorophore-labelled adalimumab in atherosclerotic plaques of hypercholesterolemic mice.Our results show that adalimumab prevents major inflammatory effects of TNF-α on endothelial activation, endothelial monocyte adhesion, endothelial leakage and therefore extends the therapeutic options of adalimumab to limit vascular inflammation.

  7. Adalimumab for Treatment of Noninfectious Uveitis: Immunogenicity and Clinical Relevance of Measuring Serum Drug Levels and Antidrug Antibodies.

    Science.gov (United States)

    Cordero-Coma, Miguel; Calleja-Antolín, Sara; Garzo-García, Irene; Nuñez-Garnés, Ana M; Álvarez-Castro, Carolina; Franco-Benito, Manuel; Ruiz de Morales, Jose G

    2016-12-01

    To evaluate the rate of immunogenicity induced by adalimumab and its relationship with drug serum levels and clinical responses in patients with noninfectious uveitis. Prospective observational study. Consecutive patients from 1 referral center who initiated treatment with adalimumab for active noninfectious uveitis resistant to conventional therapy. All patients received 40 mg adalimumab every other week. Patients were evaluated clinically and immunologically before and after 4, 8, and 24 weeks of treatment. Clinical evaluation included assessment of changes in visual acuity, degree of inflammation in the anterior chamber and vitreous cavity, central macular thickness, and retinal angiographic leakage. Immunologic evaluation included assessment of serum trough adalimumab and antibodies against adalimumab (AAA) levels and class II HLA typing. Twenty-five patients were enrolled. Overall, 18 of 25 patients (72%) showed a favorable clinical response to adalimumab therapy. Eleven patients (44%) achieved a complete response and 7 (28%) achieved a partial response. However, 7 of 25 patients (28%) were considered nonresponders. Median trough adalimumab serum levels were higher in responders than in nonresponders (P uveitis outcome was observed only in patients with permanent AAA+, which correlated with undetectable adalimumab trough levels (P = 0.014). Treatment of noninfectious uveitis with adalimumab is associated with high rates of favorable clinical response. Overall, adalimumab trough levels were higher in responder patients. Development of permanent AAA was associated with undetectable trough adalimumab levels and worse uveitis outcome. Immunogenicity was more common in patients in whom uveitis was associated with a systemic disease and was not influenced by concomitant immunosuppressors. Copyright © 2016 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.

  8. Adalimumab for the treatment of moderate to severe hidradenitis suppurativa

    DEFF Research Database (Denmark)

    Kimball, A.B.; Kerdel, F.; Adams, D.

    2012-01-01

    Background: Hidradenitis suppurativa (HS) is a chronic, painful skin disease characterized by abscesses, nodules, and draining fistulas in the axilla and groin of young adults. Objective: To evaluate the efficacy and safety of adalimumab, an anti-tumor necrosis factor-αantibody, in patients......, respectively (EOW vs. placebo difference, 1.8% [CI,- 6.4% to 10.1%]; weekly vs. placebo difference, 3.9% [CI,- 5.2% to 13.0%]). Significantly greater improvements in patient-reported outcomes and pain were seen in the weekly dosing group than in the placebo group. A decrease in response was seen after...... the switch from weekly to EOW dosing in period 2. Limitations: Weeks 16 to 52 of the study were open-label. The study was not powered to assess the risk for known serious adverse effects of adalimumab, such as tuberculosis, other serious infections, and demyelinating disorders. Conclusion: Adalimumab dosed...

  9. IgG4 Production Against Adalimumab During Long Term Treatment of RA Patients

    NARCIS (Netherlands)

    van Schouwenburg, Pauline A.; Krieckaert, Charlotte L.; Nurmohamed, Michael; Hart, Margreet; Rispens, Theo; Aarden, Lucien; Wouters, Diana; Wolbink, Gerrit Jan

    2012-01-01

    A substantial part of rheumatoid arthritis (RA) patients is chronically treated with adalimumab. Some of these patients produce antibodies against adalimumab, which correlate with lower serum drug levels and reduced clinical response. Long term exposure to antigens may result in antigen specific

  10. Shrinking lung syndrome complicating pediatric systemic lupus erythematosus

    Energy Technology Data Exchange (ETDEWEB)

    Burns, Natalie S. [University of Washington Medical Center, Department of Radiology, Seattle, WA (United States); Stevens, Anne M. [Seattle Children' s Hospital, Division of Rheumatology, Department of Pediatrics, Seattle, WA (United States); Iyer, Ramesh S. [University of Washington School of Medicine, Seattle Children' s Hospital, Department of Radiology, Seattle, WA (United States)

    2014-10-15

    Systemic lupus erythematosis (SLE) can affect the lungs and pleura, usually manifesting with pleural effusions or diffuse parenchymal disease. A rare manifestation of SLE is shrinking lung syndrome, a severe restrictive respiratory disorder. While pleuropulmonary complications of pediatric SLE are common, shrinking lung syndrome is exceedingly rare in children. We present a case of a 13-year-old girl previously diagnosed with lupus, who developed severe dyspnea on exertion and restrictive pulmonary physiology. Her chest radiographs on presentation demonstrated low lung volumes, and CT showed neither pleural nor parenchymal disease. Fluoroscopy demonstrated poor diaphragmatic excursion. While shrinking lung syndrome is described and studied in adults, there is only sparse reference to shrinking lung syndrome in children. (orig.)

  11. Shrinking lung syndrome complicating pediatric systemic lupus erythematosus

    International Nuclear Information System (INIS)

    Burns, Natalie S.; Stevens, Anne M.; Iyer, Ramesh S.

    2014-01-01

    Systemic lupus erythematosis (SLE) can affect the lungs and pleura, usually manifesting with pleural effusions or diffuse parenchymal disease. A rare manifestation of SLE is shrinking lung syndrome, a severe restrictive respiratory disorder. While pleuropulmonary complications of pediatric SLE are common, shrinking lung syndrome is exceedingly rare in children. We present a case of a 13-year-old girl previously diagnosed with lupus, who developed severe dyspnea on exertion and restrictive pulmonary physiology. Her chest radiographs on presentation demonstrated low lung volumes, and CT showed neither pleural nor parenchymal disease. Fluoroscopy demonstrated poor diaphragmatic excursion. While shrinking lung syndrome is described and studied in adults, there is only sparse reference to shrinking lung syndrome in children. (orig.)

  12. Adalimumab Dose Tapering in Psoriasis: Predictive Factors for Maintenance of Complete Clearance.

    Science.gov (United States)

    Hansel, Katharina; Bianchi, Leonardo; Lanza, Francesco; Bini, Vittorio; Stingeni, Luca

    2017-03-10

    Psoriasis can be managed successfully with long-term biologics. Real-life clinical practice may require dose tapering as a therapeutic option to reduce the risk of drug-exposure and to increase cost-effectiveness. The responsiveness to extended intervals between adalimumab doses and the possible predictive factors of maintenance of complete clearance were studied in a retrospective 7-year single-centre analysis. Thirty patients who achieved complete clearance with adalimumab underwent dose tapering, progressively extending between-dose intervals (to 21-28 days). Sixty percent of subjects (group A) maintained complete clearance, whereas 40.0% (group B) relapsed and were switched back to the standard dosage to re-achieve complete clearance. Body mass index (BMI) and time to achieve Psoriasis Area Severity Index (PASI-100) with adalimumab standard treatment before dose tapering were significantly lower in group A than in group B (multi-variate Cox regression: p < 0.05, Kaplan-Meier analysis: p < 0.001, respectively). This study suggests that patients with lower BMI and shorter time to achieve PASI-100 with adalimumab standard dose were significantly more likely to be candidates for dose tapering.

  13. Efficacy of Adalimumab as a long term maintenance therapy in ulcerative colitis.

    LENUS (Irish Health Repository)

    McDermott, Edel

    2013-03-01

    Adalimumab is a recombinant human IgG1 monoclonal antibody to TNF-alpha. There are limited data with regard to its efficacy in ulcerative colitis. We report experience of adalimumab in ulcerative colitis in a single centre with a focus on the ability of this agent to maintain response and avoid colectomy in the medium to long-term.

  14. Clinical experience with adalimumab in a multicenter Swiss cohort of patients with Crohn's disease.

    Science.gov (United States)

    Nichita, Cristina; Stelle, Marc; Vavricka, Stephan; El-Wafa Ali, Abdou; Ballabeni, Pierluigi; de Saussure, Philippe; Straumann, Alex; Rogler, Gerhard; Michetti, Pierre

    2010-01-01

    Controlled clinical trials have demonstrated the efficacy and safety of adalimumab in patients with moderate-to-severe Crohn's disease (CD), but there is, however, only limited long-term experience with adalimumab in daily practice. To assess the long-term effectiveness and safety of adalimumab in a multicenter cohort of practice-based patients with moderate-to-severe CD. We retrospectively reviewed the charts of CD patients who received adalimumab over a 3-year period. Disease severity was scored using the Harvey-Bradshaw index (HBI). Remission was defined as an HBI of 3 points at evaluation compared to the baseline. Univariate logistic regression analysis was used to identify the predictive variables associated with response. The charts of 55 patients were reviewed; remission and response rates observed at weeks 4-6 were 52.7 and 83.6%, respectively. Remission was maintained at weeks 12, 24 and 52 in 89.6, 72.4 and 44.7% of patients, respectively. Remission and response rates were not influenced by smoking status, disease location or duration, the first month total dose, or previous infliximab therapy. The remission rate at weeks 4-6 was significantly higher in patients intolerant of infliximab as compared to those who lost response to this drug. Adalimumab was well tolerated overall. Adalimumab can be considered a suitable option in patients with moderate-to-severe CD, demonstrating sustained long-term effectiveness. Copyright (c) 2010 S. Karger AG, Basel.

  15. Unusual Complication of Superior Mesenteric Artery Syndrome: Spontaneous Upper Gastrointestinal Bleeding with Hypovolemic Shock

    Directory of Open Access Journals (Sweden)

    Kai-Hsiung Ko

    2009-01-01

    Full Text Available Superior mesenteric artery (SMA syndrome is an unusual form of duodenal obstruction. Complications of SMA syndrome may sometimes develop and are usually associated with marked gastric dilatation, although most complications can be corrected by supportive treatment. In this article, we report a case of severe SMA syndrome with hypovolemic shock in a 24-year-old man. Multidetector-row computed tomography with reconstructed images was performed to establish the diagnosis. Spontaneous gastrointestinal bleeding is an extremely uncommon complication of SMA syndrome, and emergent surgical intervention was unavoidable in our patient. To our knowledge, no other such case has been reported in the English-language literature.

  16. Observational study on the efficacy of adalimumab for the treatment of ulcerative colitis and predictors of outcome.

    Science.gov (United States)

    García-Bosch, Orlando; Gisbert, Javier P; Cañas-Ventura, Alex; Merino, Olga; Cabriada, José L; García-Sánchez, Valle; Gutiérrez, Ana; Nos, Pilar; Peñalva, Mireia; Hinojosa, Joaquin; García-Planella, Esther; Muñoz, Fernando; Calvet, Xavier; Panés, Julián

    2013-10-01

    Information on efficacy and predictors of response to adalimumab in ulcerative colitis (UC) clinical practice is limited. Assessment of response to adalimumab and its predictors in an observational cohort study. Retrospective cohort study based on data obtained from ENEIDA registry. All patients diagnosed with UC treated with adalimumab were included. Response to adalimumab was evaluated at weeks 12, 28, and 54 according to the partial Mayo score, and requirement of colectomy until end of follow-up. 48 patients with UC treated with adalimumab were included; 39 (81.3%) had previously received infliximab. Response rates at weeks 12, 28 and 54 were 70.8%, 43.2% and 35% respectively. Response to prior treatment with infliximab was the only predictive factor of response to adalimumab at week 12, which was obtained in 90% of infliximab remitters, 53.8% of responders and 33.3% of primary non-responders (p=0.01). Colectomy was required in 11 patients (22.9%), after a mean time of 205 days. The only clinical independent predictor of colectomy was non-response to adalimumab at week 12: colectomy rates were 5/34 (14.7%) in responders and 6/14 (42.9%) in non-responders (p=0.035), time free of colectomy was significantly reduced in non-responders (p=0.01). Adalimumab withdrawal due to adverse events occurred in 4.2% of patients. This study shows that adalimumab is an effective treatment in patients with UC. If used as a second anti-TNF, previous achievement of remission with the first anti-TNF predicts response, and failure to achieve response at week 12 predicts colectomy. Copyright © 2012 European Crohn's and Colitis Organisation. Published by Elsevier B.V. All rights reserved.

  17. Visual Loss Induced by Adalimumab Used for Plaque Psoriasis

    Directory of Open Access Journals (Sweden)

    Norman Saffra

    2017-03-01

    Full Text Available A 61-year-old Caucasian male with severe plaque psoriasis without joint involvement was initiated on adalimumab therapy. Shortly thereafter he presented to the emergency room with acute loss of vision in the right eye. A comprehensive systemic workup was instituted which included magnetic resonance imaging (MRI with and without gadolinium of the brain and orbits. MRI revealed findings that were consistent with CNS demyelination and retrobulbar optic neuritis. Immediate cessation of adalimumab was instituted without any other systemic therapy. Complete return of vision occurred within 6 weeks. No additional psoriatic or neurologic treatment was instituted, and the patient has remained stable now for 14 months.

  18. Fatal Lemierre's syndrome as a complication of chronic otitis media ...

    African Journals Online (AJOL)

    South African Journal of Child Health ... Background. Lemierre's syndrome is septic thrombophlebitis of the internal jugular vein, initiated by an infection of the head and neck region. This septic ... We discuss the case of a 14-year-old male with Lemierre's syndrome as a complication of chronic middle ear infection. Results.

  19. [Sudden death and cardiovascular complications in Marfan syndrome: impact of surgical intervention].

    Science.gov (United States)

    Ohtsubo, Satoshi; Itoh, Tsuyoshi

    2005-07-01

    Marfan syndrome is an autosomal dominant disorder of connective tissue characterized by abnormalities involving the skeletal, ocular, and cardiovascular systems. The cardiovascular complications of the syndrome lead to a reduced life expectancy for affected individuals if left untreated. Major cause of death include acute aortic dissection, aortic rupture, and sudden death, which resulted from congenital vascular fragility. Such life-threatening complications in Marfan syndrome can be managed effectively, by routine aortic imaging, beta-adrenergic blockade, and prophylactic replacement of the aortic root before the diameter exceeds 5.0 to 5.5 mm. Valve preserving aortic root reconstruction yielded improved postoperative quality of life compared with Bentall operation, by reducing late complications related to anticoagulants. It should be carried out before onset of aortic regurgitation for long-term native valve durability.

  20. A case of melanocytic cervical adenosquamous carcinoma complicated with Cushing's syndrome.

    Science.gov (United States)

    Chen, Y; Zhang, Y; Wang, L; Yang, X

    2017-01-01

    To date, cervical carcinoma complicated with Cushing's syndrome were all diagnosed as small cell carcinoma histo- logically, but not adenosquamous carcinoma. Here the authors present the diagnosis, management, and prognosis of a case of melanocytic cervical adenosquamous carcinoma complicated with Cushing's syndrome. A 28-year-old woman was admitted with the chief complaint of post-coital bleeding for one month. Gynecological examination revealed a nodular yellowish-pigmented vegetation (6x5 cm) on the cervix. Laboratory findings proved the diagnosis of Cushing's syndrome. Histopathological diagnosis showed the adenosquamous carcinoma with melanoma differentiation. Immunohistochemical stainings for melanoma A and anti- adrenocorticotropic hormone (ACTH) were positive in the majority of the tumor cells, which indicated that this melanocytic cervical carcinoma lesion was the source of ectopic ACTH production resulting in Cushing's syndrome. This is a unique case of a rare type of cervical carcinoma.

  1. Gluteal Compartment Syndrome following bariatric surgery: A rare but important complication

    Directory of Open Access Journals (Sweden)

    Bernadette Pereira

    2015-03-01

    Full Text Available Gluteal Compartment Syndrome is a rare condition caused by excessive pressure within the gluteal compartments which leads to a number of potentially serious sequelae including rhabdomyolysis, nerve damage, renal failure and death. As bariatric patients are heavy and during prolonged bariatric procedures lie in one position for extended periods of time, they are especially susceptible to developing this complication. It is therefore essential that bariatric surgeons are aware of this complication and how to minimise the chances of it occurring and how to diagnose it. We describe a case of Gluteal Compartment Syndrome in a patient following a gastric bypass and review the aetiology, pathophysiology, treatment and prevention of this complication.

  2. Hyper Ig E syndrome (Job syndrome, HIES) – radiological images of pulmonary complications on the basis of three cases

    International Nuclear Information System (INIS)

    Jończyk-Potoczna, Katarzyna; Szczawińska-Popłonyk, Aleksandra; Warzywoda, Małgorzata; Bręborowicz, Anna; Pawlak, Bogdan

    2012-01-01

    Hyperimmunoglobulinemia E syndrome (hyper-IgE syndrome, Job syndrome, HIES) is a complex immune deficiency with multiorgan clinical manifestations and diverse genetic background. The clinical triad of symptoms observed in approximately 75% of patients with HIES includes: recurrent abscesses of staphylococcal etiology, recurrent respiratory infections and elevated immunoglobulin E in serum. The paper discusses three cases of female patients presenting typical pulmonary complications of the hyper-Ig E syndrome. In the first case, the development of aspergilloma in a postinflamatory cyst was observed, in the other one, pneumonia with pleural effusion, and as a consequence of inflammatory infiltrations – fibrotic changes, giving rise to lobectomy, while in the last of these cases, the course of lung disease was complicated by formation of staphylococcal abscess. In one of the girls, bronchiectasis appeared at follow-up. Complications of pulmonary infections are the most common causes of death in hyper-Ig E syndrome. Late diagnosis significantly worsens the respiratory function and reduces the chance for normal development of a child. Introduction of comprehensive treatment, including prophylaxis, decreases the recurrences. Therefore, the important role is attributed to the radiologist in the multidisciplinary care of patients with this syndrome

  3. Metabolic complications in the small intestine syndrome

    International Nuclear Information System (INIS)

    Mora, Rafael; Orozco, Reynaldo

    2000-01-01

    Metabolic complications in the syndrome of small intestine is presented in a patient of masculine sex, 27 years old, who consulted for a square of inflammation gingival, migraine, fever, anorexia and adinamia for three days, followed by maculopapular-eritematose eruption for 8 days, coincident with the ampicillin ingestion, and later on severe abdominal pain and diarrhea

  4. Le syndrome de Budd-Chiari: une complication rare de la ...

    African Journals Online (AJOL)

    L'atteinte hépatique au cours de la sarcoïdose est une localisation fréquente, habituellement asymptomatique. La cholestase anictérique et l'hypertension portale représentent ses principales complications. Le syndrome de Budd-Chiari est une complication peu connue qui demeure exceptionnelle. Nous rapportons un ...

  5. Refeeding Syndrome: An Important Complication Following Obesity Surgery.

    Science.gov (United States)

    Chiappetta, Sonja; Stein, Jürgen

    2016-01-01

    Refeeding syndrome (RFS) is an important and well-known complication in malnourished patients, but the incidence of RFS after obesity surgery is unknown and the awareness of RFS in obese patients as a postsurgical complication must be raised. We present a case of RFS subsequent to biliopancreatic diversion in a morbidly obese patient. A 48-year-old female patient with a BMI of 41.5 kg/m2 was transferred to our hospital due to Wernicke's Encephalopathy in a global malabsorptive syndrome after biliopancreatic diversion. Parenteral nutrition, vitamin supplementation and high-dosed intravenous thiamine supplementation were initiated. After 14 days, the patient started to develop acute respiratory failure, and neurological functions were impaired. Blood values showed significant electrolyte disturbances. RFS was diagnosed and managed according to the NICE guidelines. After 14 days, phosphate levels had returned to normal range, and neurological symptoms were improved. Extreme weight loss following obesity surgery has been shown to be associated with undernutrition. These patients are at high risk for evolving RFS, even though they may still be obese. Awareness of RFS as a postsurgical complication, the identification of patients at risk as well as prevention and correct management should be routinely performed at every bariatric center. © 2016 The Author(s) Published by S. Karger GmbH, Freiburg.

  6. Refeeding Syndrome: An Important Complication Following Obesity Surgery

    Directory of Open Access Journals (Sweden)

    Sonja Chiappetta

    2016-01-01

    Full Text Available Background: Refeeding syndrome (RFS is an important and well-known complication in malnourished patients, but the incidence of RFS after obesity surgery is unknown and the awareness of RFS in obese patients as a postsurgical complication must be raised. We present a case of RFS subsequent to biliopancreatic diversion in a morbidly obese patient. Case Report: A 48-year-old female patient with a BMI of 41.5 kg/m2 was transferred to our hospital due to Wernicke‘s Encephalopathy in a global malabsorptive syndrome after biliopancreatic diversion. Parenteral nutrition, vitamin supplementation and high-dosed intravenous thiamine supplementation were initiated. After 14 days, the patient started to develop acute respiratory failure, and neurological functions were impaired. Blood values showed significant electrolyte disturbances. RFS was diagnosed and managed according to the NICE guidelines. After 14 days, phosphate levels had returned to normal range, and neurological symptoms were improved. Conclusion: Extreme weight loss following obesity surgery has been shown to be associated with undernutrition. These patients are at high risk for evolving RFS, even though they may still be obese. Awareness of RFS as a postsurgical complication, the identification of patients at risk as well as prevention and correct management should be routinely performed at every bariatric center.

  7. Superior sagittal sinus thrombosis: a rare complication of nephrotic syndrome.

    Directory of Open Access Journals (Sweden)

    Tullu M

    1999-10-01

    Full Text Available A two and half year-old-male child, known case of steroid responsive nephrotic syndrome presented with fever and vomiting of acute onset. He was diagnosed to have superior sagittal sinus thrombosis on a contrast computerised tomographic scan of brain. Recovery was complete without anticoagulant therapy. Superior sagittal sinus thrombosis is an extremely rare complication of nephrotic syndrome.

  8. Compartment syndrome, rhabdomyolysis and risk of acute renal failure as complications of the lithotomy position.

    NARCIS (Netherlands)

    Bocca, G.; Moorselaar, R.J.A. van; Feitz, W.F.J.; Staak, F.H.J.M. van der; Monnens, L.A.H.

    2002-01-01

    Compartment syndrome, rhabdomyolysis and the risk of acute renal failure are potential complications of the lithotomy position. A six-year-old girl is described who developed a compartment syndrome with rhabdomyolysis after prolonged surgery in the lithotomy position. This complication occurred

  9. EXPERIENCE OF TREATMENT OF JUVENILE POLYARTHRITIS WITH EYES LESION WITH ADALIMUMAB

    Directory of Open Access Journals (Sweden)

    A.O. Lisitsin

    2008-01-01

    Full Text Available A case report of early debut and severe course of juvenile poly arthritis with eyes lesion refractory to classic immuno suppressive agents is presented in this article. Successful application of biological agent adalimumab is described: acute inflammatory alterations in affected joints were stopped by 49 the week. Range of motions in affected joints was recovered. The remission of veitis, normalization of indicators of disease activity (ESR, concentration of creactive protein in blood plasma was registered by 89 th week.Key words: children, juvenile rheumatoid arthritis, rheumatoid uveitis, adalimumab.

  10. Switching from adalimumab to tofacitinib in the treatment of patients with rheumatoid arthritis.

    Science.gov (United States)

    Genovese, Mark C; van Vollenhoven, Ronald F; Wilkinson, Bethanie; Wang, Lisy; Zwillich, Samuel H; Gruben, David; Biswas, Pinaki; Riese, Richard; Takiya, Liza; Jones, Thomas V

    2016-06-23

    Tofacitinib is an oral Janus kinase inhibitor for the treatment of rheumatoid arthritis (RA). The aim of this study was to explore the safety and efficacy of open-label tofacitinib following blinded treatment with adalimumab or tofacitinib for moderate to severe RA. Analyses included patients treated with adalimumab 40 mg once every 2 weeks or tofacitinib 10 mg twice daily (BID) with background methotrexate (MTX) in a 12-month randomized study (NCT00853385), who subsequently received tofacitinib 10 mg BID (with/without background MTX) in an open-label extension (NCT00413699). Patients with treatment-related serious adverse events (AEs) and serious or recurrent infections in the index study were excluded from the extension study. Exposure-adjusted incidence rates of safety-related events were assessed in 3-month and 12-month periods in the year before and in the year after switching. Efficacy was assessed 3 months before, at the time of, and 3 months after switching. There were 233 (107 adalimumab to tofacitinib 10 mg BID, 126 blinded to open-label tofacitinib 10 mg BID) patients included in these analyses. Patients in both treatment sequences had similar incidence rates (per 100 patient-years) of discontinuation due to AEs, serious AEs, and serious infections in the year before and in the year after switching. Incidence rates of AEs were increased in the first 3 months after switching compared with the last 3 months before switching in both treatment groups. Switching from either blinded adalimumab or tofacitinib to open-label tofacitinib resulted in numerically higher incidence of responders for signs and symptoms of disease and improved physical function. Treatment can be directly switched from adalimumab to tofacitinib. A similar safety and efficacy profile was seen when patients received open-label tofacitinib after receiving either blinded adalimumab or tofacitinib. ClinicalTrials.gov Identifiers: NCT00853385 , registered 27 February 2009; NCT

  11. A Case of Acute Budd-Chiari Syndrome Complicating Primary Antiphospholipid Syndrome Presenting as Acute Abdomen and Responding to Tight Anticoagulant Therapy

    Directory of Open Access Journals (Sweden)

    Naofumi Chinen

    2016-01-01

    Full Text Available A 34-year-old woman with primary antiphospholipid syndrome was admitted to the Gastroenterology Department of our hospital with fever, acute abdomen, watery diarrhea, and extremely high levels of inflammatory parameters. She had a history of left lower limb deep vein thrombosis and pulmonary embolism and was taking warfarin potassium. Acute gastroenteritis was suspected and an antibiotic was administered, but symptoms progressed. Abdominal ultrasonography showed occlusion of the left hepatic vein and the middle hepatic vein and her D-dimer level was high. Accordingly, Budd-Chiari syndrome was diagnosed and high-dose intravenous infusion of heparin was initiated. Her abdominal symptoms improved and the levels of inflammatory parameters and D-dimer decreased rapidly. It is known that antiphospholipid syndrome can be complicated by Budd-Chiari syndrome that usually occurs as subacute or chronic onset, but acute onset is rare. It is difficult to diagnose acute Budd-Chiari syndrome complicating antiphospholipid syndrome and this complication generally has a poor outcome. However, the present case can get early diagnosis and successful treatment with tight anticoagulant therapy.

  12. Effect of Adalimumab on Refractory Arthritis in Juvenile Idiopathic Inflammatory Myopathy with Anti-MDA5 Autoantibody

    Directory of Open Access Journals (Sweden)

    Takako Miyamae

    2018-01-01

    Full Text Available A 10-year-old girl manifested persistent fever, skin rash, leg pain, fatigue, and joint pain. Based on muscle weakness, elevated muscle-derived enzymes, magnetic resonance imaging, and skin biopsy results, the diagnosis was juvenile idiopathic inflammatory myopathies (JIIM. Chest CT was normal; the anti-melanoma differentiation-associated protein-5 (anti-MDA5 autoantibody was positive. Initial manifestations subsided after prednisolone (PSL and methotrexate treatment. After the PSL dosage was decreased, the patient presented with metacarpophalangeal (MCP joint pain and swelling in both index fingers, synovial fluid, and signals on power Doppler ultrasound. The arthritis was refractory to cyclosporine and tacrolimus. Radiography showed progressive MCP joint space narrowing and joint erosion. Adalimumab was initiated 14 months after disease onset. There was a mildly increased matrix metalloproteinase-3 (MMP3 level, an erythrocyte sedimentation ratio (ESR, and a normal CRP level. Adalimumab resulted in decreased MCP joint pain and swelling. PSL was discontinued 10 months after adalimumab initiation; after 9 more months of adalimumab, there were no significant ultrasonography findings. MMP3 and ESR levels normalized during treatment. Radiography after 2 years of adalimumab showed further progressive MCP joint space narrowing restricting dorsiflexion. This report clarified that anti-MDA5-positive JIIM joint manifestations were due to active synovitis and that adalimumab is required for severe cases. Further experience is needed to determine the pathology, severity, and prognosis of this type of arthritis.

  13. Guillain-Barre syndrome complicating chikungunya virus infection.

    Science.gov (United States)

    Agarwal, Ayush; Vibha, Deepti; Srivastava, Achal Kumar; Shukla, Garima; Prasad, Kameshwar

    2017-06-01

    Chikungunya virus (CHIKV) is a mosquito-borne alphavirus which presents with symptoms of fever, rash, arthralgia, and occasional neurologic disease. While outbreaks have been earlier reported from India and other parts of the world, the recent outbreak in India witnessed more than 1000 cases. Various systemic and rarely neurological complications have been reported with CHIKV. We report two cases of Guillain-Barré syndrome (GBS) with CHIKV. GBS is a rare neurological complication which may occur after subsidence of fever and constitutional symptoms by several neurotropic viruses. We describe two cases of severe GBS which presented with rapidly progressive flaccid quadriparesis progressing to difficulty in swallowing and breathing. Both required mechanical ventilation and improved partly with plasmapharesis. The cases emphasize on (1) description of the rare complication in a setting of outbreak with CHIKV, (2) acute axonal as well as demyelinating neuropathy may occur with CHIKV, (3) accurate identification of this entity during outbreaks with dengue, both of which are vector borne and may present with similar complications.

  14. Real-Life Treatment Paradigms Show Adalimumab Is Cost-Effective for the Management of Ulcerative Colitis

    Directory of Open Access Journals (Sweden)

    Candace L. Beilman

    2016-01-01

    Full Text Available Background. Adalimumab is effective for the maintenance of remission in patients with moderate-to-severe ulcerative colitis (UC. Currently, biologic therapies are used in cases where patients fail conventional medical therapies. If biologic therapies are not available, patients often choose to remain in an unwell state rather than undergo colectomy. Objective. The aim of the study was to evaluate the cost-effectiveness of adalimumab in patients with UC where adalimumab was readily available compared to not available. Methods. A previously validated Markov model was used to simulate disease progression of patients with UC who are corticosteroid-dependent and/or did not respond to thiopurine therapy. Utility scores and transition probabilities between health states were determined by using data from randomized controlled trials and real-life observational studies. Costs were obtained from the Ontario Case Costing Initiative and the Alberta Health Schedule of Medical Benefits. Results. The incremental cost-effectiveness ratios for readily available adalimumab treatment of UC were $40,000 and $59,000 per quality-adjusted life year, compared with ongoing medical therapy in an unwell state, at 5-year and 10-year treatment time horizons, respectively. Conclusion. Considering real-life patient preferences to avoid colectomy, adalimumab is cost-effective according to a willingness-to-pay threshold of $80,000 for treatment of UC.

  15. Severe Postoperative Complications may be Related to Mesenteric Traction Syndrome during Open Esophagectomy.

    Science.gov (United States)

    Ambrus, R; Svendsen, L B; Secher, N H; Goetze, J P; Rünitz, K; Achiam, M P

    2017-09-01

    During abdominal surgery, traction of the mesenterium provokes mesenteric traction syndrome, including hypotension, tachycardia, and flushing, along with an increase in plasma prostacyclin (PGI 2 ). We evaluated whether postoperative complications are related to mesenteric traction syndrome during esophagectomy. Flushing, hemodynamic variables, and plasma 6-keto-PGF 1α were recorded during the abdominal part of open ( n = 25) and robotically assisted ( n = 25) esophagectomy. Postoperative complications were also registered, according to the Clavien-Dindo classification. Flushing appeared in 17 (open) and 5 (robotically assisted) surgical cases ( p = 0.001). Mean arterial pressure was stable during both types of surgeries, but infusion of vasopressors during the first hour of open surgery was related to development of widespread (Grade II) flushing ( p = 0.036). For patients who developed flushing, heart rate and plasma 6-keto-PGF 1α also increased ( p = 0.001 and p syndrome manifests more frequently during open than robotically assisted esophagectomy, and postoperative complications appear to be associated with severe mesenteric traction syndrome.

  16. A Rare Case of Morel-Lavallee Syndrome Complicating an Anterior Dislocation of Hip Joint.

    Science.gov (United States)

    Nekkanti, Supreeth; Vijay, C; Theja, Sujana; Shankar, R Ravi; Verma, Anubhav

    2016-01-01

    Hip dislocations are serious injuries as hip joint is an extremely stable joint. It requires a significant amount of force to produce such an injury. Anterior dislocations are uncommon. Potential complications of anterior hip dislocations are a neurovascular injury to femoral vessels or acetabular fractures. We report a rare late complication of Morel-Lavallee syndrome occurring 3 weeks after an anterior dislocation of the hip in a 43-year-old male. The patient presented to us with history. Morel-Lavallee syndrome is a rare complication. However if diagnosed early can be successfully treated with minimal burden to the patient. The authors recommend surgeons to have a high index of suspicion for this syndrome and a stringent follow-up examination of the patient.

  17. The role of levosimendan in acute heart failure complicating acute coronary syndrome

    DEFF Research Database (Denmark)

    Nieminen, Markku S; Buerke, M.; Cohen-Solal, A.

    2016-01-01

    Acute heart failure and/or cardiogenic shock are frequently triggered by ischemic coronary events. Yet, there is a paucity of randomized data on the management of patients with heart failure complicating acute coronary syndrome, as acute coronary syndrome and cardiogenic shock have frequently bee...

  18. EXPERIENCE OF THE ADALIMUMAB APPLICATION FOR THE PATIENT WITH EARLY DEBUT OF JUVENILE IDIOPATHIC ARTHRITIS AND UVEITIS

    Directory of Open Access Journals (Sweden)

    K. B. Isaeva

    2014-01-01

    Full Text Available The case of early debut and heavy course of juvenile idiopathic arthritis in the patient at the age of 1 year and 8 months, associated with uveitis refractory to the therapy by methotrexate and nonsteroid antiinflammatory preparations is presented. The given clinical example shows high therapeutic efficiency of the adalimumab. To the 8th week of treatment inflammatory changes in conjunctiva were stopped, to the 12th week the stage of inactive illness was registered, i.e. the patient had no inflammatory changes in joints, uveitis activity signs, increase of laboratory indicators of illness activity. Duration of remission of articulate syndrome and uveitis made 9 months.

  19. Successful Use of Adalimumab for Treating Pyoderma Gangrenosum with Ulcerative Colitis under Corticosteroid-tapering Conditions.

    Science.gov (United States)

    Sagami, Shintaro; Ueno, Yoshitaka; Tanaka, Shinji; Nagai, Kenta; Hayashi, Ryohei; Chayama, Kazuaki

    2015-01-01

    A 52-year-old woman with ulcerative colitis was admitted to our hospital for an ulcerative colitis flare-up under salazosulfapyridine therapy. The symptoms improved with high-dose corticosteroids. After prednisolone was tapered to 10 mg, the frequency of diarrhea increased. The diarrhea was accompanied by joint pain and a skin ulcer with abscess formation, which was diagnosed to be pyoderma gangrenosum. The patient was started on adalimumab. A positive response to the adalimumab therapy was observed after 2 weeks, during which time the ulcerative skin lesion healed completely, however, colonic mucosal healing was achieved at 2 months. Therefore, adalimumab appears to be an effective therapeutic option for patients with ulcerative colitis-associated pyoderma gangrenosum.

  20. Superior Mesenteric Artery Syndrome: An Infrequent Complication of Scoliosis Surgery

    Directory of Open Access Journals (Sweden)

    Metin Keskin

    2014-01-01

    Full Text Available Superior mesenteric artery syndrome is a rare condition that causes a proximal small intestinal obstruction due to contraction of the angle between the superior mesenteric artery and the aorta. Scoliosis surgery is one of the 15 reasons for superior mesenteric artery syndrome, which can present with acute or chronic manifestations. Although conservative treatment is usually possible, surgical treatment is required in certain cases that cannot be treated using conservative methods. In this paper, we describe a patient who developed superior mesenteric artery syndrome after scoliosis surgery and was treated with duodenojejunostomy due to failure and complications of conservative treatment.

  1. Long-term efficacy of adalimumab in the treatment of uveitis associated with juvenile idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    Kotaniemi K

    2011-10-01

    Full Text Available Kaisu Kotaniemi1,2, Hanna Säilä2, Hannu Kautiainen31Helsinki University Hospital, Helsinki, Finland; 2Orton Orthopaedic Hospital and Rehabilitation Unit, Helsinki, Finland; 3Unit of Primary Health Care, Kuopio University Hospital, Kuopio, FinlandBackground: The purpose of this study was to investigate the long-term effects of adalimumab, a tumor necrosis factor alpha antagonist, in the treatment of uveitis associated with juvenile idiopathic arthritis.Methods: Adalimumab was initiated in 94 patients with juvenile idiopathic arthritis to treat active arthritis and/or active associated uveitis. In 18 patients, therapy was discontinued after a short period because of inefficacy or side effects. The activity of uveitis (using Standardized Uveitis Nomenclature [SUN] criteria and clinical examination and arthritis (number of swollen or active joints was evaluated at the start and at end of the study.Results: At the end of the study, uveitis was under good clinical control in two thirds of 54 patients (31% did not need any local treatment and 35% used only 1–2 corticosteroid drops a day, and one third had active uveitis (at least three corticosteroid drops a day. According to SUN criteria, adalimumab treatment for uveitis showed improved activity (a two-fold decrease in uveitis activity in 28% of patients, with a moderate response in 16 patients, no change in a further 16 patients, and worsening activity (a two-fold increase in uveitis activity in 13% of patients. The overall proportion of patients with active arthritis decreased. At the beginning of the study, 69% of patients with uveitis had more than two active joints, and at the end of the study only 27% had active joint disease. In 27 patients with juvenile idiopathic arthritis without uveitis on adalimumab, the number of active joints decreased from 93% to 59%. Systemic corticosteroid treatment could be stopped in 22% of patients with uveitis and in 11% of those without uveitis. Most of the

  2. Adalimumab (TNFα Inhibitor Therapy Exacerbates IgA Glomerulonephritis Acute Renal Injury and Induces Lupus Autoantibodies in a Psoriasis Patient

    Directory of Open Access Journals (Sweden)

    S. S. Wei

    2013-01-01

    Full Text Available Adalimumab (Humira is a tumour necrosis factor α (TNFα inhibitor that is approved for the treatment of rheumatoid arthritis, psoriasis, psoriatic arthritis, Crohn's disease, ankylosing spondylitis, and juvenile idiopathic arthritis (Sullivan and Preda (2009, Klinkhoff (2004, and Medicare Australia. Use of TNFα inhibitors is associated with the induction of autoimmunity (systemic lupus erythematosus, vasculitis, and sarcoidosis or sarcoid-like granulomas (Ramos-Casals et al. (2010. We report a patient with extensive psoriasis presenting with renal failure and seropositive lupus markers without classical lupus nephritis after 18 months treatment with adalimumab. He has renal biopsy proven IgA nephritis instead. Renal biopsy is the key diagnostic tool in patients presenting with adalimumab induced nephritis and renal failure. He made a remarkable recovery after adalimumab cessation and steroid treatment. To our knowledge, this is a unique case of a psoriasis patient presenting with seropositive lupus markers without classical lupus nephritis renal failure and had renal biopsy proven IgA glomerulonephritis after receiving adalimumab.

  3. Cerebral tuberculoma in a patient receiving anti-TNF alpha (adalimumab) treatment.

    LENUS (Irish Health Repository)

    Lynch, Karen

    2010-10-01

    We report a case of a cerebral tuberculoma in a 60-year-old woman with rheumatoid arthritis while receiving the anti-tumor necrosis factor alpha monoclonal antibody, adalimumab (Humira), for active disease. MR brain imaging for dyspraxia revealed a left parietal ring-enhancing lesion, which on resection was shown to be a necrotizing granuloma. There were no associated pulmonary lesions, and the patient was systemically well. Sputum and urine cultures were negative for tuberculosis. The patient was treated with anti-tuberculous medications and made an excellent recovery. We consider this to be the first documented case of tuberculosis involving the central nervous system occurring in the setting of adalimumab treatment.

  4. Adalimumab for treatment of severe Behçet's uveitis: a retrospective long-term follow-up study.

    Science.gov (United States)

    Interlandi, Emanuela; Leccese, Pietro; Olivieri, Ignazio; Latanza, Loredana

    2014-01-01

    Behçet's disease (BD) is a chronic multisystem inflammatory disorder associated to uveitis that may represent a serious sight-threatening condition. The purpose of the present study is to assess the effectiveness of adalimumab as new strategic therapeutic approach in patients affected by severe Behçet's uveitis. Clinical data from twelve selected patients (22 eyes) were retrospectively analysed. All patients received 40 mg of adalimumab subcutaneously, once every 2 weeks, in addition to traditional immunosuppressive on-going therapy and eight of them were switched to adalimumab after failure of infliximab therapy. Primary outcome measures included ocular inflammatory activity, frequency of uveitis attacks and steroid-sparing effect. Secondary outcomes were changes of best-corrected visual acuity (BCVA), impact on traditional immunosuppressive therapy and occurrence of adalimumab-related side effects. Mean age of patients (11 males and 1 female) at the onset of disease was 24.34 years (±8.62 SD). Ocular involvement resulted bilateral in 83% of cases and mainly consisted in panuveitis (68% of eyes). After mean follow-up of 21 months (±9.63 SD) all patients but one (92%) achieved uveitis remission with BCVA improvement at least in one eye. Average uveitis attacks decreased from 2 to 0,42 during adalimumab (ptreatment of patients with severe and resistant Behçet's uveitis, providing an appropriate and long-term control of ocular inflammation.

  5. Effect of adalimumab on work productivity and indirect costs in moderate to severe Crohn’s disease: A meta-analysis

    Science.gov (United States)

    Binion, David G; Louis, Edouard; Oldenburg, Bas; Mulani, Parvez; Bensimon, Arielle G; Yang, Mei; Chao, Jingdong

    2011-01-01

    OBJECTIVE: To assess the effect of adalimumab on work productivity and indirect costs in patients with Crohn’s disease (CD) using a meta-analysis of clinical trials. METHODS: Study-level results were pooled from all clinical trials of adalimumab for moderate to severe CD in which work productivity outcomes were evaluated. Work Productivity and Activity Impairment Questionnaire outcomes (absenteeism, presenteeism and total work productivity impairment [TWPI]) were extracted from adalimumab trials. Meta-analyses were used to estimate pooled averages and 95% CIs of one-year accumulated reductions in work productivity impairment with adalimumab. Pooled averages were multiplied by the 2008 United States national average annual salary ($44,101) to estimate per-patient indirect cost savings during the year following adalimumab initiation. RESULTS: The four included trials (ACCESS, CARE, CHOICE and EXTEND) represented a total of 1202 employed adalimumab-treated patients at baseline. Each study followed patients for a minimum of 20 weeks. Pooled estimates (95% CIs) of one-year accumulated work productivity improvements were as follows: −9% (−10% to −7%) for absenteeism; −22% (−26% to −18%) for presenteeism; and −25% (−30% to −20%) for TWPI. Reductions in absenteeism and TWPI translated into per-patient indirect cost savings (95% CI) of $3,856 ($3,183 to $4,529) and $10,964 ($8,833 to $13,096), respectively. CONCLUSION: Adalimumab provided clinically meaningful improvements in work productivity among patients with moderate to severe CD, which may translate into substantial indirect cost savings from an employer’s perspective. PMID:21912760

  6. Systemic sarcoidosis with bone marrow involvement responding to therapy with adalimumab: a case report

    Directory of Open Access Journals (Sweden)

    Patel Supen R

    2009-07-01

    Full Text Available Abstract Introduction Sarcoidosis is an inflammatory disorder characterized by the presence of non-caseating granulomas in affected organs. The presence of CD4-positive T lymphocytes and macrophages in affected organs suggests an ongoing immune response. Systemic corticosteroids remain the mainstay of treatment, but therapy is often limited by adverse effects. This is the first report of the use of adalimumab (HUMIRA®, Abbott Laboratories, North Chicago, IL, USA, an anti-tumor necrosis factor monoclonal antibody, in a patient with systemic sarcoidosis with bone marrow involvement. Case presentation A 42-year-old African-American man with a medical history significant for hypertension and diabetes mellitus presented with anemia and thrombocytopenia of two months duration. The patient underwent physical examination, bone marrow aspiration and biopsy, chest X-ray, acid-fast bacilli stain, computed tomography with contrast, and additional laboratory tests. He was diagnosed with systemic sarcoidosis with splenomegaly and bone marrow involvement. Drug therapy included prednisone, which had to be discontinued owing to adverse effects, and adalimumab. Conclusion This is the first report describing the use of adalimumab in a patient with systemic sarcoidosis with bone marrow involvement. Tumor necrosis factor antagonism with adalimumab was efficacious and well-tolerated in this patient and may be considered as a treatment option for similar cases.

  7. Systemic sarcoidosis with bone marrow involvement responding to therapy with adalimumab: a case report.

    Science.gov (United States)

    Patel, Supen R

    2009-07-29

    Sarcoidosis is an inflammatory disorder characterized by the presence of non-caseating granulomas in affected organs. The presence of CD4-positive T lymphocytes and macrophages in affected organs suggests an ongoing immune response. Systemic corticosteroids remain the mainstay of treatment, but therapy is often limited by adverse effects. This is the first report of the use of adalimumab (HUMIRA((R)), Abbott Laboratories, North Chicago, IL, USA), an anti-tumor necrosis factor monoclonal antibody, in a patient with systemic sarcoidosis with bone marrow involvement. A 42-year-old African-American man with a medical history significant for hypertension and diabetes mellitus presented with anemia and thrombocytopenia of two months duration. The patient underwent physical examination, bone marrow aspiration and biopsy, chest X-ray, acid-fast bacilli stain, computed tomography with contrast, and additional laboratory tests. He was diagnosed with systemic sarcoidosis with splenomegaly and bone marrow involvement. Drug therapy included prednisone, which had to be discontinued owing to adverse effects, and adalimumab. This is the first report describing the use of adalimumab in a patient with systemic sarcoidosis with bone marrow involvement. Tumor necrosis factor antagonism with adalimumab was efficacious and well-tolerated in this patient and may be considered as a treatment option for similar cases.

  8. A novel method for the detection of antibodies to adalimumab in the presence of drug reveals "hidden" immunogenicity in rheumatoid arthritis patients

    NARCIS (Netherlands)

    van Schouwenburg, Pauline A.; Bartelds, Geertje M.; Hart, Margreet H.; Aarden, Lucien; Wolbink, Gerrit Jan; Wouters, Diana

    2010-01-01

    Production of anti drug antibodies (ADA) in adalimumab treated RA patients is associated with reduced serum adalimumab levels and less clinical response. However, most current assays to measure ADA are unable to detect ADA in complex with adalimumab. Thus, ADA is only measured if antibody production

  9. Formation of antibodies against infliximab and adalimumab strongly correlates with functional drug levels and clinical responses in rheumatoid arthritis

    DEFF Research Database (Denmark)

    Radstake, T R D J; Svenson, M; Eijsbouts, A M

    2008-01-01

    BACKGROUND: Tumour necrosis factor alpha (TNFalpha) neutralising antibody constructs are increasingly being used to treat rheumatoid arthritis (RA). OBJECTIVE: To determine potential differences in clinical responses, soluble drug levels and antibody formation between patients with RA receiving...... infliximab and adalimumab. METHODS: 69 patients with RA fulfilling the 1987 American College of Rheumatology criteria and about to start treatment with infliximab or adalimumab, were enrolled consecutively. All patients had active disease (28-joint count Disease Activity Score >3.2). Infliximab was given...... intravenously at 3 mg/kg at baseline and after 2, 6 and 14 weeks. Adalimumab was administered as 40 mg biweekly subcutaneously. Concomitant drug treatment was monitored and continued at constant dosage during the study. All serum samples were tested for infliximab/adalimumab levels and anti...

  10. A randomised, single-blind, single-dose, three-arm, parallel-group study in healthy subjects to demonstrate pharmacokinetic equivalence of ABP 501 and adalimumab.

    Science.gov (United States)

    Kaur, Primal; Chow, Vincent; Zhang, Nan; Moxness, Michael; Kaliyaperumal, Arunan; Markus, Richard

    2017-03-01

    To demonstrate pharmacokinetic (PK) similarity of biosimilar candidate ABP 501 relative to adalimumab reference product from the USA and European Union (EU) and evaluate safety, tolerability and immunogenicity of ABP 501. Randomised, single-blind, single-dose, three-arm, parallel-group study; healthy subjects were randomised to receive ABP 501 (n=67), adalimumab (USA) (n=69) or adalimumab (EU) (n=67) 40 mg subcutaneously. Primary end points were area under the serum concentration-time curve from time 0 extrapolated to infinity (AUC inf ) and the maximum observed concentration (C max ). Secondary end points included safety and immunogenicity. AUC inf and C max were similar across the three groups. Geometrical mean ratio (GMR) of AUC inf was 1.11 between ABP 501 and adalimumab (USA), and 1.04 between ABP 501 and adalimumab (EU). GMR of C max was 1.04 between ABP 501 and adalimumab (USA) and 0.96 between ABP 501 and adalimumab (EU). The 90% CIs for the GMRs of AUC inf and C max were within the prespecified standard PK equivalence criteria of 0.80 to 1.25. Treatment-related adverse events were mild to moderate and were reported for 35.8%, 24.6% and 41.8% of subjects in the ABP 501, adalimumab (USA) and adalimumab (EU) groups; incidence of antidrug antibodies (ADAbs) was similar among the study groups. Results of this study demonstrated PK similarity of ABP 501 with adalimumab (USA) and adalimumab (EU) after a single 40-mg subcutaneous injection. No new safety signals with ABP 501 were identified. The safety and tolerability of ABP 501 was similar to the reference products, and similar ADAb rates were observed across the three groups. EudraCT number 2012-000785-37; Results. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

  11. Two phase 3 trials of adalimumab for hidradenitis suppurativa

    DEFF Research Database (Denmark)

    Kimball, Alexa Boer; Okun, Martin; Williams, David A

    2016-01-01

    BACKGROUND Hidradenitis suppurativa is a painful, chronic inflammatory skin disease with few options for effective treatment. In a phase 2 trial, adalimumab, an antibody against tumor necrosis factor á, showed efficacy against hidradenitis suppurativa. METHODS PIONEER I and II were similarly desi...

  12. Fatal Lemierre’s syndrome as a complication of chronic otitis media with cholesteatoma

    Directory of Open Access Journals (Sweden)

    Tashneem Harris

    2016-12-01

    Full Text Available Background. Lemierre’s syndrome is septic thrombophlebitis of the internal jugular vein, initiated by an infection of the head and neck region. This septic thrombophlebitis gives rise to septic microemboli that can disseminate throughout the body to form septic infarctions and abscesses, with the most frequent site being pulmonary. Methods. We discuss the case of a 14-year-old male with Lemierre’s syndrome as a complication of chronic middle ear infection. Results. The patient developed septic shock and microemboli, and subsequently died. Conclusion. This case report illustrates that untreated chronic middle ear infection can lead to potentially fatal complications such as Lemierre’s syndrome, and emphasises the importance of timeous treatment of chronic middle ear pathology.

  13. Acute organophosphorus poisoning complicated by acute coronary syndrome.

    Science.gov (United States)

    Pankaj, Madhu; Krishna, Kavita

    2014-07-01

    We report a case of 30 year old alcoholic male admitted with vomiting, drowsiness, limb weakness and fasciculations after alleged history of consumption of 30 ml of chlorpyriphos insecticide. He had low serum cholinesterase levels. With standard treatment for organophosphorus poisoning (OPP), he improved gradually until day 5, when he developed neck and limb weakness and respiratory distress. This intermediate syndrome was treated with oximes, atropine and artificial ventilation. During treatment, his ECG showed fresh changes of ST elevation. High CPK & CPK-MB levels, septal hypokinesia on 2D echo suggested acute coronary syndrome. Coronary angiography was postponed due to his bedridden and obtunded status. The patient finally recovered fully by day 15 and was discharged. Acute coronary syndrome is a rare occurrence in OP poisoning. The present case thus emphasises the need for careful electrocardiographic and enzymatic monitoring of all patients of organophosphorus poisoning to prevent potential cardiac complication which can prove fatal.

  14. Long-term efficacy of adalimumab in the treatment of uveitis associated with juvenile idiopathic arthritis

    OpenAIRE

    Kotaniemi, Kaisu; S?il?, Hanna; Kautiainen, Hannu

    2011-01-01

    Kaisu Kotaniemi1,2, Hanna Säilä2, Hannu Kautiainen31Helsinki University Hospital, Helsinki, Finland; 2Orton Orthopaedic Hospital and Rehabilitation Unit, Helsinki, Finland; 3Unit of Primary Health Care, Kuopio University Hospital, Kuopio, FinlandBackground: The purpose of this study was to investigate the long-term effects of adalimumab, a tumor necrosis factor alpha antagonist, in the treatment of uveitis associated with juvenile idiopathic arthritis.Methods: Adalimumab was...

  15. Cost-effectiveness analysis of infliximab, adalimumab, golimumab and vedolizumab for moderate to severe ulcerative colitis in Spain.

    Science.gov (United States)

    Trigo-Vicente, Cristina; Gimeno-Ballester, Vicente; Montoiro-Allué, Raquel; López-Del Val, Alejandro

    2017-12-02

    Assess the efficiency of biologic treatment for moderate to severe ulcerative colitis (UC) which are indicated and financed for this pathology by Spain. A Markov model was constructed to simulate the progression in a cohort of patients with moderate to severe UC. The perspective chosen was National Health Service with an over 10 years of time horizon, with a discount rate of 3%, and established threshold of €30,000/quality-adjusted life-year (QALY). The comparison between infliximab versus adalimumab achieved an incremental cost-effectiveness ratio (ICER) of €45,582/QALY, with a 0.900 QALYs difference of efficacy and an incremental cost of €41,036. Golimumab versus adalimumab reached an ICER of €2,175,992/QALY, with a difference of 0.001 QALY in efficacy and a raising cost to €2,611. The comparison between vedolizumab with adalimumab achieved an ICER of €90,532/QALY, 0.930 QALYs of difference and an increasing cost of €84,218. The probabilistic sensitivity analysis shows that adalimumab would be cost-effective in the 65.2% of the simulations, infliximab in the 18.4%, golimumab in the 16.4% and vedulizumab for the 0%. Among all these drugs studied, adalimumab is the most cost-effective drug for the treatment of moderate to severe UC for a threshold of €30,000/QALY in Spain.

  16. A rare complication of Ramsey Hunt Syndrome: Sınus vein thrombosis

    Directory of Open Access Journals (Sweden)

    Ramiz Ahmedov

    2011-03-01

    Full Text Available Ramsay-Hunt Syndrome (RHS is a rare affection characterized by peripheral facial paralysis (PFP, skin eruption in the auricular canal and cochleovestibular symptoms. It is produced by varicella-zoster virus(VZV reactivation at the geniculate ganglia. In elderly and immunocompromised individuals, the virus may reactivate to produce shingles (zoster. After zoster resolves, many elderly patients experience postherpetic neuralgia. Uncommonly, VZV can spread to large cerebral arteries to cause a spectrum of large-vessel vascular damage, ranging from vasculopathy to vasculitis, with stroke. In immunocompromised individuals, especially those with cancer or acquired immunodeficiency syndrome, deeper tissue penetration of the virus may occur (as compared with immunocompetent individuals, with resultant myelitis, small-vessel vasculopathy, ventriculitis, and meningoencephalitis. The polymerase chain reaction (PCR analysis of cerebrospinal fluid remains the mainstay for diagnosing the neurologic complications of VZV during life. We report a case of Ramsay Hunt syndrome complicated with cerebral venous thrombosis. Patient received treatment with acyclovir and anticoagulation. Early treatment with acyclovir therapy and anticoagulation could improve the recovery rate of facial nerve palsy and sinus vein thrombosis

  17. SMART Syndrome: Case report of a rare complication after cerebral radiation therapy

    International Nuclear Information System (INIS)

    Truntzer, P.; Salze, P.; Monjour, A.; Gaultier, C.; Ahle, G.; Guillerme, F.; Boutenbat, G.; Atlani, D.; Stilhart, B

    2012-01-01

    The authors report a 71-year-old woman case who developed, 7 years after a cerebral radiation therapy for a parieto-occipital glioblastoma, a stroke-like migraine attacks after radiotherapy syndrome (SMART syndrome), a rare complication characterized by reversible neurologic deficits with migraine described after cerebral irradiation. Transient gyri-form reversible enhancement is found on MRI during crises. This case report allows discussing the clinical, iconographic presentation and the clinical outcome of this syndrome at the light of the literature publication. (authors)

  18. Dose Escalation and Healthcare Resource Use among Ulcerative Colitis Patients Treated with Adalimumab in English Hospitals: An Analysis of Real-World Data.

    Directory of Open Access Journals (Sweden)

    Christopher M Black

    Full Text Available To describe the real-world use of adalimumab for maintenance treatment of ulcerative colitis (UC and associated healthcare costs in English hospitals.Retrospective cohort study.Analysis of NHS Hospital Episode Statistics linked with pharmacy dispensing data in English hospitals.Adult UC patients receiving ≥240mg during adalimumab treatment induction, subsequently maintained on adalimumab.Frequency and pattern of adalimumab use and dose escalation during maintenance treatment and associated healthcare costs (prescriptions and hospital visits.191 UC patients completed adalimumab treatment induction. 83 (43.46% dose escalated during maintenance treatment by ≥100% (equivalent to weekly dosing (median time to dose escalation: 139 days. 56 patients (67.47% subsequently de-escalated by ≥50% (median time to dose de-escalation: 21 days. Mean all-cause healthcare costs for all patients ≤12 months of index were £13,892. Dose escalators incurred greater mean healthcare costs than non-escalators ≤12 months of index (£14,596 vs. £13,351. Prescriptions accounted for 96.49% of UC-related healthcare costs (£11,090 of £11,494 in all patients.Within the cohort, 43.46% of UC patients escalated their adalimumab dose by ≥100% and incurred greater costs than non-escalators. The apparent underestimation of adalimumab dose escalation in previous studies may have resulted in underestimated costs in healthcare systems.

  19. Buried bumper syndrome revisited: a rare but potentially fatal complication of PEG tube placement.

    Science.gov (United States)

    Biswas, Saptarshi; Dontukurthy, Sujana; Rosenzweig, Mathew G; Kothuru, Ravi; Abrol, Sunil

    2014-01-01

    Percutaneous endoscopic gastrostomy (PEG) has been used for providing enteral access to patients who require long-term enteral nutrition for years. Although generally considered safe, PEG tube placement can be associated with many immediate and delayed complications. Buried bumper syndrome (BBS) is one of the uncommon and late complications of percutaneous endoscopic gastrostomy (PEG) placement. It occurs when the internal bumper of the PEG tube erodes into the gastric wall and lodges itself between the gastric wall and skin. This can lead to a variety of additional complications such as wound infection, peritonitis, and necrotizing fasciitis. We present here a case of buried bumper syndrome which caused extensive necrosis of the anterior abdominal wall.

  20. Effect of the TNF-α inhibitor adalimumab in patients with recalcitrant sarcoidosis: a prospective observational study using FDG-PET

    DEFF Research Database (Denmark)

    Milman, Nils; Graudal, Niels; Loft, Annika

    2012-01-01

    -PET) in patients with recalcitrant sarcoidosis treated with adalimumab. Methods: Prospective 24-week observational study. Patients continued medication with steroids and antimetabolites and received adalimumab 40 mg subcutaneously every other week. Ten patients with biopsy-proven sarcoidosis (two men) were...

  1. Marfan syndrome and cardiovascular complications: results of a family investigation.

    Science.gov (United States)

    Sarr, Simon Antoine; Djibrilla, Siddikatou; Aw, Fatou; Bodian, Malick; Babaka, Kana; Ngaidé, Aliou Alassane; Dioum, Momar; Ba, Serigne Abdou

    2017-07-19

    Cardiovascular complications in Marfan syndrome (MFS) make all its seriousness. Taking as a basis the Ghent criteria, we conducted a family screening from an index case. The objective was to describe the clinical characteristics of MFS anomalies and to detect cardiovascular complications in our patients. Six subjects were evaluated. Patients had to be in the same uterine siblings of the index case or be a descendant. The objective was to search for MFS based on the diagnostic criteria of Ghent and, subsequently, detecting cardiovascular damage. The average age was 24 years. The examination revealed three cases of sudden death in a context of chest pain. Five subjects had systemic involvement with a score ≥ 7 that allowed to the diagnosis of MFS. Two patients had simultaneously ectopia lentis and myopia. In terms of cardiovascular damage, there were three cases of dilatation of the aortic root, two cases of aortic dissection of Stanford's type A with severe aortic regurgitation in one case and moderate in the other. There were three patients with moderate mitral regurgitation with a case by valve prolapse. The family screening is crucial in Marfan syndrome. It revealed serious cardiovascular complications including sudden death and aortic dissection.

  2. Spontaneous liver rupture in pregnancy complicating HELLP syndrome: case report

    Directory of Open Access Journals (Sweden)

    Sariyeh Golmahammadlou

    2014-10-01

    Conclusion: Spontaneous liver rupture associated with HELLP syndrome is a rare and life-threatening complication of pregnancy. Unruptured liver hematoma is also a rare condition during pregnancy with a very difficult diagnosis. Using clinical diagnostic tests such as CT scan or MRI would be helpful to improve clinical outcomes.

  3. Metabolic syndrome in an Hispanic population–cardiovascular complications

    Directory of Open Access Journals (Sweden)

    Pablo I. Altieri

    2011-11-01

    Full Text Available The metabolic syndrome (MetS is presently one of the main medical problems in developing countries. This syndrome was studied in Puerto Rico at the Cardiovascular Center of Puerto Rico and the Caribbean with emphasis on understanding the cardiovascular complications. The medical records of patients admitted between 1999 to 2005 were evaluated for three or more MetS diagnostic criteria. One hundred and seventy-three patients met the consensus criteria of metabolic syndrome (MetS. The mean age of those diagnosed with MetS was 60 years of age. Fifty-seven percent were males and 42 percent females. The mean body mass was 30 kg/m. The ejection fraction was found to be subnormal (49±8% and the end systolic dimension of the left atrium was increased ( 45±10 mm in comparison to a group of diabetic patients without MetS used for comparison. The incidence of atrial fibrillation was found to be 16% higher in the MetS group than in the comparison group. The number of cases of metabolic syndrome recorded within the Hispanic population of Puerto Rico showed a higher incidence of atrial fibrillation without ventricular tachycardia. This is thought to be as a result of the abnormal left ventricular and atrial function.

  4. Holmium laser use in the treatment of selected dry eye syndrome complications

    Science.gov (United States)

    Kecik, Dariusz; Kecik, Tadeusz; Kasprzak, Jan; Kecik, Mariusz

    1996-03-01

    The authors present initial results of treatment selected complications of dry eye syndrome with holmium laser. The lacrimal puncta obliteration and coagulation of the corneal ulcer surface were done.

  5. Treatment changes in patients with psoriasis on etanercept or adalimumab

    DEFF Research Database (Denmark)

    Lee, Erica B; Amin, Mina; Egeberg, Alexander

    2018-01-01

    PURPOSE: To determine the frequency of and reasons for treatment changes in patients with psoriasis. MATERIALS AND METHODS: A retrospective chart review of 140 patients with psoriasis treated with adalimumab or etanercept seen at Kaiser Permanente Los Angeles Medical Center between September 20, ...

  6. Microcephalic osteodysplastic primordial dwarfism type II (MOPD II) with multiple vascular complications misdiagnosed as Dubowitz syndrome

    OpenAIRE

    Dieks, Jana-Katharina; Baumer, Alessandra; Wilichowski, Ekkehard; Rauch, Anita; Sigler, Matthias

    2014-01-01

    To date, the genetic basis of Dubowitz syndrome (short stature, microcephaly, facial abnormalities, eczema) is unknown and vascular complications are not known to be associated with this syndrome. In microcephalic osteodysplastic primordial dwarfism type II (MOPD II; disproportionate short statue, microcephaly, facial abnormalities), however, cerebral aneurysms and other vascular abnormalities are frequent complications. MOPD II is a genetic disorder caused by mutations in the pericentrin (PC...

  7. Kocuria kristinae infection during adalimumab treatment.

    Science.gov (United States)

    Kolikonda, Murali K; Jayakumar, Priyanga; Sriramula, Srividya; Lippmann, Steven

    2017-03-01

    A common inhabitant of skin, the Kocuria kristinae of the Micrococcaceae family, has gained attention in recent years because it can induce pathology in humans. Reported is a Kocuria kristinae-caused abdominal abscess in a patient treated for rheumatoid arthritis with adalimumab. The tumor necrosis factor (TNF) inhibitor drugs are known to cause various bacterial, viral, and fungal infections. This is the first known case where an opportunistic infection with Kocuria has presented with an abdominal abscess in an immunocompromised individual who is on long term TNF inhibitors.

  8. The economics of adalimumab for ulcerative colitis.

    Science.gov (United States)

    Xie, Feng

    2015-06-01

    Ulcerative colitis is a chronic inflammatory disease, characterized by diffuse mucosal inflammation in the colon. Adalimumab, as a TNF-α blocker, offers a safe and efficacious treatment option for patients with moderate to severe ulcerative colitis and refractory or intolerant to conventional medications; however, its cost-effectiveness profile has not yet been well established. Future economic evaluations should choose appropriate comparators in the context of target-reimbursement decision making and focus on cost-effectiveness over a long time horizon.

  9. Acute Respiratory Distress Syndrome Complicating Strongyloides stercoralis Hyperinfection

    Directory of Open Access Journals (Sweden)

    Ming-Ju Tsai

    2011-03-01

    Full Text Available Strongyloidiasis is endemic in tropic and subtropic areas, but is currently seldom encountered in developed area like Taiwan. We present an elder man with acute respiratory distress syndrome complicating Strongyloides stercoralis hyperinfection. There was no significant clue initially for diagnosing this patient as having S. stercoralis hyperinfection. Neither peripheral eosinophilia nor significant hemoptysis was noted. Bronchoscopy played a critical role to define the unexpected cause of his progressive pulmonary infiltrates. The correct diagnosis was soon made by recognition of the worm in bronchioloalveolar lavage cytology, and specific treatment was initiated promptly. For a septic patient with progressive pulmonary infiltrates, bronchoscopic studies including cytology may be necessary for defining the cause. Hyperinfection strongyloidiasis should be considered as a cause of acute respiratory distress syndrome in immunocompromised patient, especially with the presence of chronic gastrointestinal symptoms.

  10. Successful treatment of ulcerative colitis complicated by Sweet's syndrome by corticosteroid therapy and leukocytapheresis.

    Science.gov (United States)

    Terai, Tomohiro; Sugimoto, Mitsushige; Osawa, Satoshi; Sugimoto, Ken; Furuta, Takahisa; Kanaoka, Shigeru; Ikuma, Mutsuhiro

    2011-06-01

    Ulcerative colitis is occasionally complicated by dermatological disorders presenting as extra-intestinal manifestations, including erythema nodosum and pyoderma gangrenosum. Sweet's syndrome is considered to be a rare cutaneous disease in patients with ulcerative colitis. To date, only 17 cases of Sweet's syndrome complicating ulcerative colitis have been reported in the English literature. Here, we report a case of a 41-year-old male who had been suffering from ulcerative colitis for 20 years. He was admitted to hospital with hematochezia, diarrhea and fever, and painful erythematous nodules on the face and arms. Histological examination of skin biopsies showed inflammatory cell infiltration composed mainly of neutrophils without evidence of necrotizing vasculitis, and the condition was diagnosed as Sweet's syndrome. The patient was treated with prednisolone and leukocytapheresis and the erythematous nodules on the skin, as well as the abdominal symptoms and endoscopic findings of ulcerative colitis, immediately improved. In this paper we report on this case and review the literature concerning ulcerative colitis and Sweet's syndrome.

  11. Tofacitinib or adalimumab versus placebo: patient-reported outcomes from a phase 3 study of active rheumatoid arthritis.

    Science.gov (United States)

    Strand, Vibeke; van Vollenhoven, Ronald F; Lee, Eun Bong; Fleischmann, Roy; Zwillich, Samuel H; Gruben, David; Koncz, Tamas; Wilkinson, Bethanie; Wallenstein, Gene

    2016-06-01

    To evaluate effects of tofacitinib or adalimumab on patient-reported outcomes (PROs) in patients with moderate to severe RA and inadequate responses to MTX. In this 12-month, phase 3, randomized controlled trial (ORAL Standard), patients (n = 717) receiving background MTX were randomized to tofacitinib 5 or 10 mg twice daily (BID), adalimumab 40 mg once every 2 weeks or placebo. PROs included HAQ-Disability Index, Patient Global Assessment of Arthritis, Patient Assessment of Arthritis Pain, health-related quality of life (Short Form-36 [SF-36]), fatigue (Functional Assessment of Chronic Illness Therapy-Fatigue) and sleep (Medical Outcomes Study-Sleep). At month 3, tofacitinib 10 mg BID treatment resulted in significant changes from baseline vs placebo across all PROs, sustained to month 12, with the highest number of patients reporting improvements ⩾minimum clinically important differences vs placebo (P tofacitinib 5 mg BID and adalimumab were similar and statistically significant vs placebo across most PROs, excluding SF-36 Mental Component Score and Social Functioning, Role Emotional, and Mental Health domains, with significantly more patients reporting improvements ⩾minimum clinically important differences. Numbers Needed to Treat were lowest for tofacitinib 10 mg BID and similar between tofacitinib 5 mg BID and adalimumab. Patients with moderate to severe RA and inadequate responses to MTX reported improvements across a broad range of PROs with tofacitinib 5 and 10 mg BID and adalimumab that were significantly superior to placebo. © The Author 2016. Published by Oxford University Press on behalf of the British Society for Rheumatology.

  12. Efficacy of adalimumab in patients with crohn's disease and failure to infliximab therapy: a clinical series Eficacia de Adalimumab en pacientes con enfermedad de Crohn y fracaso previo a la terapia con Infliximab: resultados de una serie clínica

    Directory of Open Access Journals (Sweden)

    Patricia Cordero-Ruiz

    2011-06-01

    Full Text Available Background: adalimumab, a human anti-TNF, is an effective induction and maintenance therapy for patients with moderate to severe Crohn's disease. It seems to be effective in patients with resistance to infliximab, too, though the experience is more limited. Aim: to evaluate the efficacy of adalimumab, in patients with Crohn's disease (CD and failure to previous treatment with infliximab. B twenty-five patients with CD and failure to previous treatment with infliximab were enrolled; they were treated with 160/80 (24 patients and 80/40 (1 patient induction doses. We analyze clinical response to treatment with adalimumab by the Crohn's disease Activity Index (CDAI and plasma concentration of C-reactive protein (CRP, steroid sparing and complete fistula closure at week 48. Results: eighteen out of twenty-five patients (72% achieved clinical remission (CDAI score Introducción: adalimumab, un anti-TNF humano, ha demostrado ser efectivo en la inducción y tratamiento de mantenimiento de la enfermedad de Crohn moderada-grave. Existe menos experiencia, pero este fármaco parece también eficaz en los pacientes con pérdida de respuesta o intolerancia al infliximab. Objetivo: evaluar la eficacia de adalimumab durante un año, en nuestra serie de pacientes con enfermedad de Crohn (EC y fracaso en el tratamiento previo con infliximab. Métodos: se incluyen 25 pacientes con enfermedad de Crohn y fracaso previo a la terapia con infliximab, que son tratados con adalimumab. Se utilizaron dosis de inducción de 160/80 mg en 24 pacientes y dosis de 80/40 en un paciente. Analizamos la respuesta clínica al tratamiento con adalimumab mediante el Índice de actividad de la enfermedad de Crohn (CDAI y las concentraciones plasmáticas de proteína C reactiva (PCR, el cese de la corticoterapia y el cierre completo de las fistulas en la semana 48. Resultados: dieciocho de veinticinco pacientes (72% alcanzan la remisión clínica (CDAI < 150 en la semana 24 y 15

  13. Five-year analysis from the ESPRIT 10-year postmarketing surveillance registry of adalimumab treatment for moderate to severe psoriasis.

    Science.gov (United States)

    Menter, Alan; Thaçi, Diamant; Papp, Kim A; Wu, Jashin J; Bereswill, Mareike; Teixeira, Henrique D; Rubant, Simone; Williams, David A

    2015-09-01

    ESPRIT is an ongoing, 10-year, observational registry, evaluating long-term safety and effectiveness of adalimumab treatment in routine clinical practice for patients with moderate to severe, chronic plaque psoriasis. Initial 5-year results are reported. Two populations were analyzed: the "all-treated" population received 1 or more adalimumab doses in registry, continuing adalimumab treatment from a current prescription or previous study participation, and included the "new-prescription" population initiating adalimumab 4 weeks or earlier preregistry entry. Data were collected from September 26, 2008, through November 30, 2013, for all-treated (n = 6059), which included new-prescription (n = 2580, 42.6%); median registry exposure was 765 and 677 days, respectively. In all-treated, rate (events per 100 patient-years of total adalimumab exposure [E/100PY]) of serious treatment-emergent adverse events (inside or outside of the registry) was 4.3 E/100PY, serious infection 1.0 E/100PY, malignancies 0.9 E/100PY (nonmelanoma skin cancers 0.6 E/100PY; melanomas <0.1 E/100PY). Standardized mortality ratio was 0.30 (95% confidence interval 0.19-0.44). Physician Global Assessment clear or minimal (effectiveness parameter) was achieved by 57.0% at 12 months and 64.7% at 60 months of treatment. Observational data are subject to outcome-reporting bias. No new safety signals were observed with adalimumab treatment during this initial 5-year registry review. Observed number of deaths was below expected. As-observed effectiveness remained stable through 60 months. Copyright © 2015 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.

  14. Changes in bone mineral density during long-term treatment with adalimumab in patients with rheumatoid arthritis: a cohort study

    NARCIS (Netherlands)

    Krieckaert, C.L.M.; Nurmohamed, M.T.; Wolbink, G.; Lems, W.F.

    2013-01-01

    Objective. To investigate the effect of long-term adalimumab treatment on BMD of the lumbar spine, total hip and hands in patients with RA. Methods. In 184 established RA patients treated with adalimumab for at least 1 year, BMD measurements of the total hip and lumbar spine were performed using

  15. Neurologic complications of cerebral angiography in childhood moyamoya syndrome

    International Nuclear Information System (INIS)

    Robertson, R.L.; Chavali, R.V.; Robson, C.D.; Barnes, P.D.; Burrows, P.E.; Eldredge, E.A.; Scott, R.M.

    1998-01-01

    Purpose. To determine the incidence of neurologic complications of cerebral angiography in children with moyamoya syndrome (MMS) as compared to children without MMS. Materials and methods. One-hundred-ninety consecutive cerebral angiograms obtained in 152 children were evaluated. Sixty of these angiograms were obtained in 40 children with MMS. Patients underwent neurologic evaluation prior to and after the procedure. For this study, a neurologic complication was defined as any new focal neurologic deficit or alteration in mental status occurring during the procedure or within the ensuing 24 hours. Results. There were 2 neurologic complications within 24 hours of angiography, one in the MMS group and one in the non-MMS group. One patient with MMS became mute following angiography. The symptom resolved within 12 hours. One patient without MMS being examined postoperatively for residual arteriovenous malformation developed intracranial hemorrhage requiring reexploration 12 hours after the angiogram. Using a two-tail Fisher's exact test, there was no significant statistical difference in the ischemic (P = 0.3) or hemorrhagic (P = 1.0) complication rates between the group of patients with MMS and the non-MMS groups. Conclusion. The risk of a neurologic complication from cerebral angiography in children with MMS is low and not statistically different from the risk in children with other cerebrovascular disorders. (orig.)

  16. Chronic calcific constrictive pericarditis complicating Churg-Strauss syndrome: first reported case.

    Science.gov (United States)

    Aboukhoudir, Falah; Pansieri, Michel; Rekik, Sofiene

    2014-10-01

    Churg-Strauss syndrome is a necrotizing systemic vasculitis characterized by extravascular granulomas and eosinophilic infiltrates of small vessels. Although cardiac complications are considered to be relatively common, no case of constrictive calcified pericarditis has ever been previously described in this setting. In this report, we present the case of a 46-year-old man with Churg-Strauss syndrome, in whom we were able to document the development of symptomatic calcific constrictive pericarditis during a 10-year period despite long-term corticosteroid therapy. Georg Thieme Verlag KG Stuttgart · New York.

  17. Premature Birth with Complicated Perinatal Course Delaying Diagnosis of Prader-Willi Syndrome

    Directory of Open Access Journals (Sweden)

    G. Ciana

    2011-01-01

    Full Text Available Prader-Willi syndrome in the newborn is essentially characterized by marked hypotonia, feeding difficulties, hypogonadism, and possible characteristic facial features. However, diagnosis at this age may be particularly difficult, and dysmorphic features may be subtle or absent. Prematurity can furthermore delay clinical features recognition and typical complications due to preterm birth may contribute to divert the diagnosis. We describe a preterm baby with a complicated perinatal course later diagnosed as PWS.

  18. Prediction of treatment response to adalimumab

    DEFF Research Database (Denmark)

    Krintel, S B; Dehlendorff, C; Hetland, M L

    2016-01-01

    At least 30% of patients with rheumatoid arthritis (RA) do not respond to biologic agents, which emphasizes the need of predictive biomarkers. We aimed to identify microRNAs (miRNAs) predictive of response to adalimumab in 180 treatment-naïve RA patients enrolled in the OPtimized treatment algori...... of low expression of miR-22 and high expression of miR-886.3p was associated with EULAR good response. Future studies to assess the utility of these miRNAs as predictive biomarkers are needed.The Pharmacogenomics Journal advance online publication, 5 May 2015; doi:10.1038/tpj.2015.30....

  19. Gastrointestinal Surgery and Related Complications in Patients with Ehlers-Danlos Syndrome

    DEFF Research Database (Denmark)

    Burcharth, Jakob; Rosenberg, Jacob

    2012-01-01

    Introduction: Ehlers-Danlos syndrome (EDS) is a rare inherited group of connective tissue diseases characterized by joint hypermobility, skin hyperextensibility and bruising tendency. Common features of patients with EDS include vascular and gastrointestinal perforations. The purpose...... of this systematic review is to address gastrointestinal diseases and the complications associated with surgical treatment of diseases relating to the gastrointestinal system in patients with EDS. Methods: PubMed search including the Medical Subject Heading (MeSH) terms 'Ehlers-Danlos Syndrome' and 'Gastrointestinal...... Diseases', and an Embase search including the Map Term to Subject Heading 'Ehlers-Danlos Syndrome' with 'AND' function of the keyword 'Gastrointestinal'. Results: The literature search resulted in inclusion of 53 articles after application of eligibility criteria. The primary results drawn from...

  20. Adalimumab impact on fatigue measured by FACIT-F: a study of rheumatoid arthritis patients in Saudi Arabia

    Directory of Open Access Journals (Sweden)

    Janoudi N

    2017-10-01

    Full Text Available Nahed Janoudi,1 Narges Omran,2 Waleed Hussain,3 Hanan Al-osaimi,4 Matouqa Baamer,5 Muhammad Irfanullah Siddiqui,6 Omar Fathaldin,7 Hani Almoallim1,4,8 1Department of Medicine, Dr. Soliman Fakeeh Hospital, Jeddah, 2Department of Medicine, Alnoor General Hospital, 3Department of Medicine, Heraa General Hospital, 4Alzaidi Chair of Research in Rheumatic Diseases, Umm Al-Qura University, Makkah, 5Department of Medicine, King Abdulaziz Hospital & Oncology Center, Jeddah, 6Department of Community Medicine and Public Health, Faculty of Medicine, Umm Al-Qura University, Makkah, 7Department of Medicine, Faculty of Medicine, King Abdulaziz University, Jeddah, 8Department of Medicine, Faculty of Medicine, Umm Al-Qura University, Makkah, Saudi Arabia Objective: The objective of the study was to assess the effectiveness of adalimumab as a treatment for fatigue in patients with rheumatoid arthritis (RA. Methods: Fatigue was monitored in patients with RA who were already on an adalimumab treatment regimen. Fatigue, general well-being, comorbidities, and disease activity were measured at baseline and ~8, 16, and 24 weeks, thereafter. Results: Significant reductions in fatigue scores and disease activity were observed from baseline to 6 months after. A predictive regression model of fatigue severity was proposed and was found to be significant, with RA disease activity as the most significant predictor of fatigue severity. Conclusion: This quasi-experimental study is a good starting point for research on the efficacy of adalimumab in treating fatigue in RA patients. The results here suggest that a randomized controlled trial assessing adalimumab as a treatment option for RA patients suffering from fatigue is warranted. Keywords: rheumatoid arthritis, fatigue, adalimumab, disease activity, Saudi Arabia, FACIT-F 

  1. Clinical similarity of biosimilar ABP 501 to adalimumab in the treatment of patients with moderate to severe plaque psoriasis: A randomized, double-blind, multicenter, phase III study.

    Science.gov (United States)

    Papp, Kim; Bachelez, Herve; Costanzo, Antonio; Foley, Peter; Gooderham, Melinda; Kaur, Primal; Narbutt, Joanna; Philipp, Sandra; Spelman, Lynda; Weglowska, Jolanta; Zhang, Nan; Strober, Bruce

    2017-06-01

    ABP 501 is a biosimilar of adalimumab. We sought to compare the efficacy and safety of ABP 501 with adalimumab. This 52-week, double-blind study randomized patients with moderate to severe psoriasis to ABP 501 or adalimumab. At week 16, those with 50% or more improvement in Psoriasis Area and Severity Index score from baseline on ABP 501 continued the same treatment, whereas adalimumab-treated patients were rerandomized to adalimumab or ABP 501. Clinical similarity in Psoriasis Area and Severity Index percent improvement from baseline to week 16 (primary end point) was established if the point estimate of treatment difference and its 2-sided 95% confidence interval between groups was within equivalence margin of ±15. Patients, including those undergoing a single transition at week 16, were evaluated for safety and immunogenicity. Psoriasis Area and Severity Index percent improvement at week 16 was 80.9 for ABP 501 and 83.1 for adalimumab (least-square mean difference -2.18 [95% confidence interval -7.39 to 3.02]). Adverse events (67.2% [117/174] vs 63.6% [110/173]) and antidrug antibody incidence (55.2% [96/174] vs 63.6% [110/173]) for ABP 501 vs adalimumab were similar. Safety, including immunogenicity, was similar among groups after single transition (week 20). The 52-week data are not reported here. ABP 501 was shown to be clinically similar to adalimumab. Safety and immunogenicity were not impacted immediately after single transition (adalimumab to ABP 501). Copyright © 2016 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.

  2. [Polycystic ovary syndrome: an example of obesity-related cardiovascular complication affecting young women].

    Science.gov (United States)

    Orio, Francesco; Cascella, Teresa; Giallauria, Francesco; Palomba, Stefano; De Lorenzo, Anna; Lucci, Rosa; Ambrosino, Elena; Lombardi, Gaetano; Colao, Annamaria; Vigorito, Carlo

    2006-03-01

    Polycystic ovary syndrome (PCOS) is a good example of obesity-related cardiovascular complication affecting young women. PCOS is not only considered a reproductive problem but rather represents a complex endocrine, multifaceted syndrome with important health implications. Several evidences suggest an increased cardiovascular risk of cardiovascular disease associated with this syndrome, characterized by an impairment of heart structure and function, endothelial dysfunction and lipid abnormalities. All these features, probably linked to insulin-resistance, are often present in obese PCOS patients. Cardiovascular abnormalities represent important long-term sequelae of PCOS that need further investigations.

  3. Adalimumab for maintenance therapy for one year in Crohn's disease: results of a Latin American single-center observational study.

    Science.gov (United States)

    Kotze, Paulo Gustavo; Abou-Rejaile, Vinícius Rezende; Uiema, Luciana Aparecida; Olandoski, Marcia; Sartor, Maria Cristina; Miranda, Eron Fábio; Kotze, Lorete Maria da Silva; Saad-Hossne, Rogério

    2014-01-01

    Adalimumab is a fully-human antibody that inhibits TNF alpha, with a significant efficacy for long-term maintenance of remission. Studies with this agent in Latin American Crohn's disease patients are scarce. The objective of this study was to outline clinical remission rates after 12 months of adalimumab therapy for Crohn's disease patients. Retrospective, single-center, observational study of a Brazilian case series of Crohn's disease patients under adalimumab therapy. Variables analyzed: demographic data, Montreal classification, concomitant medication, remission rates after 1, 4, 6 and 12 months. Remission was defined as Harvey-Bradshaw Index ≤ 4, and non-responder-imputation and last-observation-carried-forward analysis were used. The influence of infliximab on remission rates was analyzed by Fischer and Chi-square tests (Panalysis. After 12 months, remission on patients with previous infliximab occurred in 69.23% as compared to 94.59% in infliximab-naïve patients (P = 0.033). Adalimumab was effective in maintaining clinical remission after 12 months of therapy, with an adequate safety profile, and was also more effective in infliximab naïve patients.

  4. Developments in the treatment of moderate to severe ulcerative colitis: focus on adalimumab

    Directory of Open Access Journals (Sweden)

    Freeman HJ

    2013-11-01

    Full Text Available Hugh J FreemanDepartment of Medicine (Gastroenterology, University of British Columbia, Vancouver, BC, Canada Abstract: In selected patients with moderate to severe active ulcerative colitis who have failed to respond or are poorly responsive to standard pharmacologic forms of treatment with corticosteroids and immunosuppressive agents, therapy with a biological agent may be considered. While infliximab is an established tumor necrosis factor blocker and has a longer history of clinical use, adalimumab is an alternative in the same class and may be employed as an initial biological agent, if indicated for treatment of the disease. Adalimumab may have special appeal to stable users able to self-inject in a home setting rather than a centralized infusion center. Short-term adverse effects have been limited, but long-term adverse events can be serious and remain less well defined. Recently, another agent, subcutaneous golimumab, has also been reported to induce and maintain clinical response and remission in clinical trials, but a large experience has not been accumulated to date in clinical practice. In the future, other biological agents with novel and distinct mechanisms of therapeutic action may become available. Keywords: ulcerative colitis, anti-tumor necrosis factor inhibitors, biological therapy, infliximab, adalimumab

  5. A double-blind placebo-controlled randomized trial of adalimumab in the treatment of hidradenitis suppurativa

    DEFF Research Database (Denmark)

    Miller, I; Lynggaard, C D; Lophaven, S

    2011-01-01

    BACKGROUND: Hidradenitis suppurativa (HS) has an impact on patients' quality of life. Treatment of HS is generally unsatisfactory, thus new treatments are needed. OBJECTIVES: To test the efficacy of adalimumab in HS. METHODS: This was a prospective, randomized, double-blinded, placebo-controlled,......BACKGROUND: Hidradenitis suppurativa (HS) has an impact on patients' quality of life. Treatment of HS is generally unsatisfactory, thus new treatments are needed. OBJECTIVES: To test the efficacy of adalimumab in HS. METHODS: This was a prospective, randomized, double-blinded, placebo......-controlled, two-centre clinical trial conducted in Denmark. Inclusion criteria were age above 18 years and a clinical diagnosis of moderate to severe HS defined as Hurley stage II or III for at least 6 months. The patients were randomized 1:2 (placebo/active). Actively treated patients received adalimumab 80 mg...... subcutaneously (s.c.) at baseline followed by 40 mg s.c. every other week for 12 weeks. Placebo-treated patients received identical-looking injections with no active ingredient. The medicine was dispensed in sequentially numbered computer-randomized containers. Participants, care givers and those assessing...

  6. Achievement of sustained deep remission with adalimumab in a patient with both refractory ulcerative colitis and seronegative erosive rheumatoid arthritis

    Directory of Open Access Journals (Sweden)

    G. Andrisani

    2013-05-01

    Full Text Available Inflammatory bowel disease (IBD is commonly associated with peripheral inflammatory arthritis, and it has been estimated that as many as 12% of IBD patients report these manifestations. However, rheumatoid arthritis (RA is rarely associated with ulcerative colitis (UC. Among all the biological agents available, nine have been currently approved for the treatment of RA. Conversely, only Infliximab and recently Adalimumab have been approved for UC. In particular, the efficacy of Adalimumab in UC has been demonstrated by both recent randomized controlled trials and real-life studies. Moreover, Adalimumab is a well-established treatment for RA. Herein, we describe a patient with RA and UC treated successfully with ADA.

  7. THE EXPERIENCE OF ADALIMUMAB USE IN A PATIENT WITH PAUTSIARTICULAR JUVENILE ARTHRITIS AND UVEITIS

    Directory of Open Access Journals (Sweden)

    E.I. Alexeeva

    2011-01-01

    Full Text Available The article presents the observation of early debut and the severe course of the juvenile polyarthritis involving the eyes, refractory to treatment by classical immunosuppressants. Successful use of the genetically engineered biological drug — adalimumab is described:  by the 4th week of therapy the acute inflammatory changes in affected joints were stopped, range of motion in them fully recovered; to the 8th week uveitis remission was registered, laboratory values of the disease activity were normalized: erythrocyte sedimentation rate, serum concentration of C-reactive protein. Key words: children, juvenile rheumatoid arthritis, rheumatoid uveitis, adalimumab. (Pediatric pharmacology. — 2011; 8 (6: 119–124.

  8. Neurologic complications of cerebral angiography in childhood moyamoya syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Robertson, R.L.; Chavali, R.V.; Robson, C.D.; Barnes, P.D.; Burrows, P.E. [Department of Radiology, Children`s Hospital Medical Center, Boston, MA (United States); Eldredge, E.A. [Department of Anesthesia, Children`s Hospital Medical Center and Harvard Medical School, Boston, MA (United States); Scott, R.M. [Department of Neurosurgery, Children`s Hospital Medical Center and Harvard Medical School, Boston, MA (United States)

    1998-11-01

    Purpose. To determine the incidence of neurologic complications of cerebral angiography in children with moyamoya syndrome (MMS) as compared to children without MMS. Materials and methods. One-hundred-ninety consecutive cerebral angiograms obtained in 152 children were evaluated. Sixty of these angiograms were obtained in 40 children with MMS. Patients underwent neurologic evaluation prior to and after the procedure. For this study, a neurologic complication was defined as any new focal neurologic deficit or alteration in mental status occurring during the procedure or within the ensuing 24 hours. Results. There were 2 neurologic complications within 24 hours of angiography, one in the MMS group and one in the non-MMS group. One patient with MMS became mute following angiography. The symptom resolved within 12 hours. One patient without MMS being examined postoperatively for residual arteriovenous malformation developed intracranial hemorrhage requiring reexploration 12 hours after the angiogram. Using a two-tail Fisher`s exact test, there was no significant statistical difference in the ischemic (P = 0.3) or hemorrhagic (P = 1.0) complication rates between the group of patients with MMS and the non-MMS groups. Conclusion. The risk of a neurologic complication from cerebral angiography in children with MMS is low and not statistically different from the risk in children with other cerebrovascular disorders. (orig.) With 8 tabs., 37 refs.

  9. AA amyloidosis complicating the hereditary periodic fever syndromes.

    Science.gov (United States)

    Lane, Thirusha; Loeffler, Jutta M; Rowczenio, Dorota M; Gilbertson, Janet A; Bybee, Alison; Russell, Tonia L; Gillmore, Julian D; Wechalekar, Ashutosh D; Hawkins, Philip N; Lachmann, Helen J

    2013-04-01

    AA amyloidosis is a life-threatening complication of the hereditary periodic fever syndromes (HPFS), which are otherwise often compatible with normal life expectancy. This study was undertaken to determine the characteristics, presentation, natural history, and response to treatment in 46 patients who had been referred for evaluation at the UK National Amyloidosis Centre. Disease activity was monitored by serial measurement of serum amyloid A. Renal function was assessed by measurement of serum creatinine and albumin levels, the estimated glomerular filtration rate, and proteinuria from 24-hour urine collections. The amyloid load was measured by serum amyloid P scintigraphy. Twenty-four patients had familial Mediterranean fever, 12 patients had tumor necrosis factor receptor-associated periodic syndrome, 6 patients had cryopyrin-associated periodic syndromes, and 4 patients had mevalonate kinase deficiency. The median age at onset of HPFS was 5 years; median age at presentation with AA amyloidosis was 38 years. Diagnosis of an HPFS had not been considered prior to presentation with AA amyloidosis in 23 patients (50%). Eleven patients (24%) had end-stage renal failure (ESRF) at presentation; of these, 3 had received transplants prior to referral. A further 13 patients developed ESRF over the followup period, with 10 undergoing renal transplantation. The median time to progression to ESRF from onset of AA amyloidosis was 3.3 years (interquartile range [IQR] 2-8), with a median time to transplant of 4 years (IQR 3-6). Eleven patients (24%) died. The median survival in the entire cohort was 19 years from diagnosis of AA amyloidosis. Of the 37 patients who were treated successfully, or in whom at least partial suppression of the underlying HPFS was achieved, 17 (46%) showed amyloid regression, 14 (38%) showed a stable amyloid load, and 2 (5%) showed increased amyloid deposition over the followup period. AA amyloidosis remains a challenging and serious late complication

  10. Long-term safety and efficacy of adalimumab for intestinal Behçet's disease in the open label study following a phase 3 clinical trial

    Directory of Open Access Journals (Sweden)

    Nagamu Inoue

    2017-07-01

    Full Text Available Background/Aims: Intestinal Behçet's disease (BD is an immune-mediated inflammatory disorder. We followed up the patients and evaluated safety profile and effectiveness of adalimumab for the treatment of intestinal BD through 100 weeks rolled over from the 52 week clinical trial (NCT01243671.Methods: Patients initiated adalimumab therapy at 160 mg at week 0, followed by 80 mg at week 2, followed by 40 mg every other week until the end of the study. Long-term safety and all adverse events (AEs were examined. The efficacy was assessed on the basis of marked improvement (MI and complete remission (CR using a composite efficacy index, which combined global gastrointestinal symptoms and endoscopic assessments.Results: Twenty patients were enrolled in this study; 15 patients received adalimumab treatment until study completion. The incidence of AEs through week 100 was 544.4 events/100 person-years, which was comparable to the incidence through week 52 (560.4 events/100 person-years. No unexpected trend was observed and adalimumab was well tolerated. At weeks 52 and 100, 60.0% and 40.0% of patients showed MI, respectively, and 20.0% and 15.0% of patients showed CR, respectively.Conclusions: This report demonstrates 2 years safety and effectiveness of adalimumab in intestinal BD patients. Patients with intestinal BD refractory to conventional treatment receiving up to 2 years of adalimumab treatment demonstrated safety outcomes consistent with the known profile of adalimumab, and the treatment led to sustained reduction of clinical and endoscopic disease activity.

  11. Severe Postoperative Complications may be Related to Mesenteric Traction Syndrome during Open Esophagectomy

    DEFF Research Database (Denmark)

    Ambrus, R; Svendsen, L B; Secher, N H

    2017-01-01

    . RESULTS: Flushing appeared in 17 (open) and 5 (robotically assisted) surgical cases ( p = 0.001). Mean arterial pressure was stable during both types of surgeries, but infusion of vasopressors during the first hour of open surgery was related to development of widespread (Grade II) flushing ( p = 0......BACKGROUND: During abdominal surgery, traction of the mesenterium provokes mesenteric traction syndrome, including hypotension, tachycardia, and flushing, along with an increase in plasma prostacyclin (PGI2). We evaluated whether postoperative complications are related to mesenteric traction...... syndrome during esophagectomy. METHODS: Flushing, hemodynamic variables, and plasma 6-keto-PGF1α were recorded during the abdominal part of open ( n = 25) and robotically assisted ( n = 25) esophagectomy. Postoperative complications were also registered, according to the Clavien-Dindo classification...

  12. Long-term use of adalimumab in the treatment of moderate to severe plaque psoriasis: a review of the literature

    Directory of Open Access Journals (Sweden)

    Angela Y Moore

    2010-04-01

    Full Text Available Angela Y Moore, Blakely S RichardsonArlington Center for Dermatology, Arlington, Texas, USAAbstract: Psoriasis is a chronic T-cell-mediated inflammatory disease that primarily affects the skin and joints. Patients with moderate to severe psoriasis constitute about 30% of the psoriasis population. Treatment of this group is challenging due to the long-term side effects, toxicities and inconvenience of conventional treatments such as phototherapy, methotrexate and cyclosporine. However, recent advances in our understanding of the pathogenesis of psoriasis have led to the popular use of biologics, which offer a safer, more convenient and effective targeted therapy. Adalimumab was originally approved for treating rheumatoid arthritis. Currently, adalimumab is also approved for treatment of adult patients with moderate to severe chronic plaque psoriasis who are candidates for systemic therapy or phototherapy or when other systemic therapies are medically less appropriate. Since the onset of the use of biologics, there have been concerns over safety and efficacy when used as long-term therapy. This paper reviews all publications, posters and abstracts reporting original data on the efficacy and/or safety of adalimumab in patients treated for chronic plaque psoriasis for more than 1 year.Keywords: psoriasis, adalimumab, biologics

  13. Adalimumab effect in a cohort of Libyan patients with rheumatic ...

    African Journals Online (AJOL)

    Four patients had psoriatic arthritis, mean age was 40 years, two were females and two were males. One patient had AOSD; she was a female aged 58 years. All RA patients were on prednisolone and/or one or two DMARD before starting adalimumab and failed to show a response. Fourteen AS patients were on one or two ...

  14. Preventing the aortic complications of Marfan syndrome: a case-example of translational genomic medicine

    Science.gov (United States)

    Li-Wan-Po, Alain; Loeys, Bart; Farndon, Peter; Latham, David; Bradley, Caroline

    2011-01-01

    The translational path from pharmacological insight to effective therapy can be a long one. We aim to describe the management of Marfan syndrome as a case-example of how pharmacological and genomic insights can contribute to improved therapy. We undertook a literature search for studies of Marfan syndrome, to identify milestones in description, understanding and therapy of the syndrome. From the studies retrieved we then weaved an evidence-based description of progress. Marfan syndrome shows considerable heterogeneity in clinical presentation. It relies on defined clinical criteria with confirmation based on FBN1 mutation testing. Surgical advances have prolonged life in Marfan syndrome. First-line prophylaxis of complications with β-adrenoceptor blockers became established on the basis that reduction of aortic pressure and heart rate would help. Over-activity of proteinases, first suggested in 1980, has since been confirmed by evidence of over-expression of matrix metalloproteinases (MMP), notably MMP-2 and MMP-9. The search for MMP inhibitors led to the evaluation of doxycycline, and both animal studies and small trials, provided early evidence that this widely used antimicrobial agent was useful. Identification of the importance of TGF-β led to evaluation of angiotensin II type I receptor (AT1R) blockers with highly promising results. Combination prophylactic therapy would appear rational. Pharmacological and genomic research has provided good evidence that therapy with losartan and doxycycline would prevent the aortic complications of Marfan syndrome. If on-going well designed trials confirm their efficacy, the outlook for Marfan syndrome patients would be improved considerably. PMID:21276043

  15. Treatment choices of paediatric rheumatologists for juvenile idiopathic arthritis : etanercept or adalimumab?

    NARCIS (Netherlands)

    Anink, Janneke; Otten, Marieke H; Gorter, Simone L; Prince, Femke H M; van Rossum, Marion A J; van den Berg, J Merlijn; van Pelt, Philomine A; Kamphuis, Sylvia; Brinkman, Danielle M C; Swen, Wijnand A A; Swart, Joost F; Wulffraat, Nico M; Dolman, Koert M; Koopman-Keemink, Yvonne; Hoppenreijs, Esther P A H; Armbrust, Wineke; ten Cate, Rebecca; van Suijlekom-Smit, Lisette W A

    OBJECTIVES: To evaluate differences in baseline characteristics between etanercept- and adalimumab-treated JIA patients and to reveal factors that influence the choice between these TNF inhibitors, which are considered equally effective in the recent ACR recommendations for JIA treatment. METHODS:

  16. Cellulitis as complication of nephrotic syndrome in a pediatric patient

    Science.gov (United States)

    Siregar, R. S.; Daulay, K. R.; Siregar, B.; Ramayani, O. R.; Eyanoer, P. C.

    2018-03-01

    Nephrotic syndrome is a chronic disease that may act as a risk for other major infection in skin, respiratory and urinary tract, while also increasingthe chance for other diseases, like peritonitis, meningitis, and cellulitis. Cellulitis is often caused by Streptococcus β-hemolytic, Staphylococcus aureus, and Escherichia coli. The clinical features of cellulitis marked with redness rash and well-defined borders, pain pressure and swelling. Hypoalbuminemia which occurs due to proteinuria occurred in this patient acts as a risk factor for cellulitis. It has been reported the case of cellulitis as one of the complications of the nephrotic syndrome in the pediatric patient. The treatment has been given to the patient such as antibiotics and supportive therapy and also planned albumin substitution.

  17. Generalized tetanus could be complicated with Guillain–Barré syndrome

    Directory of Open Access Journals (Sweden)

    Jae Hoon Lee

    2016-07-01

    Full Text Available A retrospective analysis of patients diagnosed with tetanus was conducted to evaluate the occurrence of Guillain–Barré syndrome (GBS. Two of 13 tetanus cases were complicated with GBS. Their symptoms and signs related to GBS improved markedly after a 5-day infusion of intravenous immunoglobulin. Physicians should keep in mind that GBS can be an important cause of muscle weakness in patients with tetanus.

  18. EXPERIENCE OF ADALIMUMAB ADMINISTRATION TO A PATIENT WITH JUVENILE ANKYLOSING SPONDYLOARTHRITIS AND CHRONIC VIRUS HEPATITIS B

    Directory of Open Access Journals (Sweden)

    E. A. Legostaeva

    2014-01-01

    Full Text Available The article presents a clinical case of high effectiveness of adalimumab — human monoclonal antibodies to tumor necrosis factor — in a patient with juvenile ankylosing spondyloarthritis refractory to therapy with classic immunosuppressive drugs and secondary to virus hepatitis B in the integrative phase. Joint pains and intensity of exudative alterations in interphalangeal and wrist joints attenuated as early as after as the first adalimumab injection; the authors achieved a 50% improvement in indices BASDAI (2.4 and ASDAI (1.1. Acute inflammatory alterations terminated, motion range of the affected joints completely recovered, general laboratory parameters of disease activity (erythrocyte sedimentation rate, C-reactive protein and white blood cell count normalized and inactive disease stage was registered after 6 months. Adverse events or virus hepatitis B exacerbations in the setting of adalimumab therapy were not observed. No osteochondral destruction progression was observed at computed and magnetic resonance imaging of joints 6 months after the therapy beginning. Emotional condition and quality of life of the child and his family improved considerably. 

  19. Frontal lobe syndrome from bilateral globus pallidus lesions a complication of Wernicke's encephalopathy

    OpenAIRE

    Arruda, Walter Oleschko

    1991-01-01

    A 38 year-old man developed the classical clinical picture of Wernicke's encephalopathy as a consequence of prolonged total parenteral nutrition. As a late complication he developed a frontal lobe syndrome. Bilateral globus pallidus lesions were observed in the CT-scan examination. Some aspects related to the cortical syndromes caused by subcortical lesions are discussed. Relata-se um caso de encefalopatia de Wernicke que ocorreu em paciente masculino de 38 anos, como complicação de alimen...

  20. Superior sagittal sinus thrombosis: a rare complication in a child with nephrotic syndrome

    International Nuclear Information System (INIS)

    Pirogovsky, A.; Adi, M.; Barzilai, N.; Dagan, A.; Sinai, L.; Sthoeger, D.; Tabachnik, E.

    2001-01-01

    A 2-year-old boy with new-onset nephrotic syndrome developed recurrent vomiting, apathy and papilloedema. Superior sagittal sinus thrombosis was diagnosed on cranial CT and MRI. He gradually recovered after treatment with heparin, fresh frozen plasma and warfarin with complete resolution of the thrombosis after 1 month. Superior sagittal sinus thrombosis is an extremely rare complication of nephrotic syndrome in children. Early diagnosis is essential for institution of anticoagulation therapy and a successful outcome. (orig.)

  1. Superior sagittal sinus thrombosis: a rare complication in a child with nephrotic syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Pirogovsky, A.; Adi, M.; Barzilai, N. [Dept. of Radiology, Kaplan Medical Center, Rehovot (Israel); Dagan, A.; Sinai, L.; Sthoeger, D. [Div. of Paediatrics, Kaplan Medical Center, Rehovot (Israel); Tabachnik, E. [Div. of Paediatrics, Kaplan Medical Center, Rehovot (Israel); Paediatric ICU, Kaplan Hospital, Rehovot (Israel)

    2001-10-01

    A 2-year-old boy with new-onset nephrotic syndrome developed recurrent vomiting, apathy and papilloedema. Superior sagittal sinus thrombosis was diagnosed on cranial CT and MRI. He gradually recovered after treatment with heparin, fresh frozen plasma and warfarin with complete resolution of the thrombosis after 1 month. Superior sagittal sinus thrombosis is an extremely rare complication of nephrotic syndrome in children. Early diagnosis is essential for institution of anticoagulation therapy and a successful outcome. (orig.)

  2. Phase III Randomized Study of SB5, an Adalimumab Biosimilar, Versus Reference Adalimumab in Patients With Moderate‐to‐Severe Rheumatoid Arthritis

    OpenAIRE

    Weinblatt, Michael E.; Baranauskaite, Asta; Niebrzydowski, Jaroslaw; Dokoupilova, Eva; Zielinska, Agnieszka; Jaworski, Janusz; Racewicz, Artur; Pileckyte, Margarita; Jedrychowicz‐Rosiak, Krystyna; Cheong, Soo Yeon; Ghil, Jeehoon; Sokolovic, S.; Mekic, M.; Prodanovic, N.; Gajic, B.

    2017-01-01

    Objective SB5 is a biosimilar agent for adalimumab (ADA). The aim of this study was to evaluate the efficacy, pharmacokinetics (PK), safety, and immunogenicity of SB5 in comparison with reference ADA in patients with rheumatoid arthritis (RA). Methods In this phase III, randomized, double‐blind, parallel‐group study, patients with moderately to severely active RA despite treatment with methotrexate were randomized 1:1 to receive SB5 or reference ADA at a dosage of 40 mg subcutaneously every o...

  3. A case of Lemierre's syndrome with septic shock and complicated parapneumonic effusions requiring intrapleural fibrinolysis

    Directory of Open Access Journals (Sweden)

    Daniel P. Croft

    2015-01-01

    Full Text Available Lemierre's syndrome is a septic thrombophlebitis of the internal jugular vein, which can lead to severe systemic illness. We report a case of an otherwise healthy 26-year-old man who suffered from pharyngitis followed by septic shock requiring intubation and vasopressor support from Fusobacterium necrophorum bacteremia. The septic emboli to his lungs caused complicated bilateral parapneumonic effusions, which recurred after initial drainage. He required bilateral chest tubes and intrapleural tPA to successfully drain his effusions. His fever curve and overall condition improved with the resolution of his effusions and after a 33-day hospitalization, he recovered without significant disability. The severity of his illness and difficult to manage complicated parapneumonic effusions were the unique facets of this case. Using an evidence-based approach of tPA and DNase for complicated parapneumonic effusions in Lemierre's syndrome can be safe and effective.

  4. Matrix metalloproteinase inhibitor therapy to prevent complications as well as therapy for Ehler-Danlos syndrome.

    Science.gov (United States)

    Sastry, P S R K

    2002-09-01

    Matrixmetalloproteinase inhibitors have been developed as anti-cancer agents. Their usage in pancreatic cancer and other such malignancies is under trial at present. An interesting undesired-effect of one of these agents is contracture of the hand. Ehler-Danlos syndrome is an inherited group of diseases with varying types. At present there is no known treatment or prevention for the complications associated with this inherited condition. Sometimes it is the adverse events of a drug, which provides an insight into its efficacy for another indication. It is hereby being hypothesized that the matrixmetalloproteinase inhibitors especially marimastat may be an effective drug for treatment of Ehler-Danlos syndrome and/or prevention of its major complications.

  5. Neuromyelitis optica in pregnancy complicated by posterior reversible encephalopathy syndrome, eclampsia and fetal death.

    Science.gov (United States)

    Igel, Catherine; Garretto, Diana; Robbins, Matthew S; Swerdlow, Michael; Judge, Nancy; Dayal, Ashlesha

    2015-03-01

    Neuromyelitis optica (NMO) is a demyelinating syndrome characterized by optic neuritis and acute myelitis with poor recovery and a progressive course. We report a poor outcome complicated by posterior reversible encephalopathy syndrome (PRES) and eclampsia and review available literature and current evidence for anticipation of adverse fetal and maternal effects. After a pregnancy complicated by multiple admissions for painful NMO exacerbations, a primiparous patient with seropositive NMO presented at 31 + 3/7 weeks with eclampsia, HELLP and subsequent fetal death. MRI confirmed PRES. NMO may be associated with eclampsia and leads to adverse maternal and fetal outcomes. Posited mechanisms include antibody-mediated placental damage and a heightened risk of eclampsia-associated PRES. Further characterization of the course of NMO and its relationship with pregnancy outcomes in larger series would be invaluable.

  6. HELLP Syndrome Complicated with Postpartum Subcapsular Ruptured Liver Hematoma and Purtscher-Like Retinopathy

    Directory of Open Access Journals (Sweden)

    Daniela Cernea

    2012-01-01

    Full Text Available Purtscher's retinopathy is usually associated with trauma, acute pancreatitis, vasculitis, lupus, and bone fractures. It was rarely described postpartum in patients with preeclampsia as well as associated with HELLP syndrome. We present a case of a multiparous patient aged 44 with severe preeclampsia and postpartum HELLP syndrome complicated with Purtscher-like retinopathy and large ruptured subcapsular liver hematoma that required emergency abdominal surgery after premature delivery of a dead fetus. Postsurgical outcome was favorable regarding both liver function and visual acuity.

  7. Adalimumab (Humira) restores clinical response in patients with secondary loss of efficacy from infliximab (Remicade) or etanercept (Enbrel): results from the STURE registry at Karolinska University Hospital.

    Science.gov (United States)

    Wick, M C; Ernestam, S; Lindblad, S; Bratt, J; Klareskog, L; van Vollenhoven, R F

    2005-01-01

    To determine whether the tumour necrosis factor-alpha (TNF-alpha) antagonist adalimumab (Humira) can be efficacious after secondary loss of efficacy (i.e. loss of clinical response in patients who had initially demonstrated clinical response) to infliximab (Remicade) or etanercept (Enbrel). We studied 36 patients from the Stockholm TNF-alpha follow-up registry (STURE) who received adalimumab after secondary loss of efficacy to infliximab (group A, n = 27) or etanercept (group B, n = 9), and 26 patients who were started on adalimumab as the first TNF-alpha antagonist (group C). In group A, the baseline disease activity score 28 (DAS28) at infliximab institution was 5.5+/-0.2. During infliximab treatment, the mean best DAS28 was 3.7+/-0.2 (p<0.001), but increased to 5.2+/-0.3 when infliximab was stopped. After 3 months on adalimumab, the mean DAS28 decreased to 4.5+/-0.3 (p<0.003), and then to 4.2+/-0.2 at 6 months (p<0.001). In group B, the baseline DAS28 at etanercept institution was 6.6+/-0.5. During etanercept treatment, the mean best DAS28 was 4.6+/-0.5 (p<0.01), but increased to 5.7+/-0.4 by the time etanercept was stopped. After 3 months on adalimumab, the mean DAS28 decreased to 4.8+/-0.3 (p<0.005), and to 4.1+/-0.2 at 6 months (p<0.001). In group C, the mean baseline DAS28 was 5.6+/-0.3. After 6 months of adalimumab therapy, the DAS28 decreased to 3.5+/-0.4 (p<0.001). ACR20 responses with adalimumab in groups A, B, and C were similar (70-78%). For patients with secondary loss of efficacy from infliximab or etanercept, switching to adalimumab can restore a good clinical response.

  8. The clinical value of pulmonary perfusion imaging complicated with pulmonary embolism in children of nephrotic syndrome

    International Nuclear Information System (INIS)

    Lin Jun; Chen Ning; Miao Weibing; Peng Jiequan; Jiang Zhihong; Wu Jing

    2001-01-01

    To investigate the clinical features of complicated with pulmonary embolism nephrotic syndrome in children. 99m Tc-MAA pulmonary perfusion imaging was performed on 30 nephrotic syndrome in children with elevated plasma D-dimer. Results shown that 14 of 30 patients were found to have pulmonary embolism (46.7%). Pulmonary perfusion imaging showed an involvement of 1 pulmonary segment in 3 cases, 2 segments in 2 cases and over 3 segments in other 9 cases. Among them, there were 7 segments involved in one case. After two weeks of heparin anti-coagulative therapy, most cases showed a recovery. The result of this study suggested that pulmonary embolism is a common complication of nephrotic syndrome. Pulmonary perfusion imaging is simple, effective and accurate method for the diagnosis of pulmonary embolism, and it also can help to assess the value of clinical therapy

  9. Surgical treatment of scoliosis in Marfan syndrome: outcomes and complications.

    Science.gov (United States)

    Qiao, Jun; Xu, Leilei; Liu, Zhen; Zhu, Feng; Qian, Bangping; Sun, Xu; Zhu, Zezhang; Qiu, Yong; Jiang, Qing

    2016-10-01

    To investigate surgical outcomes and complications of scoliosis associated with Marfan syndrome. Inclusion criteria were patients who were 10-20 years of age, had a diagnosis of Marfan syndrome by the Ghent nosology, had scoliosis and had undergone spinal fusion, and had at least 2 years of postoperative follow-up. The medical records of all patients were reviewed for age at the time of surgery, surgical procedures performed, instrumentation type, estimated blood loss (EBL) during surgery, operation time and complications related to surgery. Health-related quality-of-life measures (obtained with the SRS-22 Questionnaire before operation and at the last clinical follow-up) were also recorded. Patients were analyzed as two different groups, Group 1 and Group 2, according to the different approaches employed. Patients receiving combined anterior and posterior surgery were assigned to Group 1 and those who received posterior-only surgery to Group 2. Group 1 consisted of 30 patients (14 males, 16 females) with a mean age at surgery of 16.8 years (range: 10-20 years). Complications in Group 1 included two cases of instrumentation loosening with one removed, one case of instrumentation breakage and one case of chylothorax and hemothorax during video assisted thoracoscopic release. 66 patients (28 males, 38 females) with a mean age at surgery of years 16.4 years (range: 10-20 years) were included in Group 2. Complications in Group 2 included six cases of cerebro-spinal fluid leak, one case of deep wound infection secondary to cerebro-spinal fluid leak, one case of leg weakness and one case of pleural rupture cause by misplacement of pedicle screw. There is no difference of age at surgery, preoperative Cobb angles, and SRS-22 total scores (3.0 vs. 3.1) between the two groups (P > 0.05). Group 1 yielded larger correction rate than Group 2 for both thoracic (62.5 % vs. 56.2 %) and lumbar scoliosis (68.3 % vs. 62.7 %). Loss of correction was similar between the two

  10. Long-Term Safety and Effectiveness of Adalimumab for Moderate to Severe Psoriasis: Results from 7-Year Interim Analysis of the ESPRIT Registry.

    Science.gov (United States)

    Menter, Alan; Thaçi, Diamant; Wu, Jashin J; Abramovits, William; Kerdel, Francisco; Arikan, Dilek; Guo, Dianlin; Ganguli, Arijit; Bereswill, Mareike; Camez, Anne; Valdecantos, Wendell C

    2017-09-01

    ESPRIT (NCT00799877) is an ongoing 10-year international prospective observational registry evaluating the long-term safety and effectiveness of originator adalimumab in routine clinical practice for adult patients with chronic plaque psoriasis. Herein, we report the long-term safety, effectiveness, and patient-reported outcomes (PROs) following adalimumab treatment over the first 7 years of the ESPRIT registry. All treatment-emergent (All-TE) adverse events (AE) since the initial (first ever) dose of adalimumab were assessed. Physician Global Assessment (PGA) and PROs (PROs for US patients only) were evaluated during registry participation. As of 30 November 2015, 6051 patients in the ESPRIT registry were analyzed, representing 23,660.1 patient-years (PY) of overall adalimumab exposure. The incidence rates for All-TE serious AEs, serious infections, and malignancies were 4.4, 1.0, and 1.0 events per 100 PY (E/100PY), respectively. The standardized mortality ratio for TE deaths in the registry was 0.27 (95% CI 0.18-0.38). During the registry's first 7 years, PGA "clear" or "minimal" was achieved by >50% of patients at each annual visit, and among US patients, the mean improvement from baseline in different PROs was maintained. No new safety signals were identified during the first 7 years of the registry, and safety was consistent with the known safety profile of adalimumab. The number of TE deaths was below the expected rate. During the registry's first 7 years, most of the patients remained free of All-TE cardiovascular events, serious infections, and malignancy. As-observed effectiveness of adalimumab and improvements from baseline in PROs were maintained through 7 years of registry participation. Abbvie. ClinicalTrials.gov identifier, NCT00799877.

  11. Acute abdominal compartment syndrome complicating a colonoscopic perforation: a case report

    Directory of Open Access Journals (Sweden)

    Souadka Amine

    2012-02-01

    Full Text Available Abstract Introduction A perforation occurring during colonoscopy is an extremely rare complication that may be difficult to diagnose. It can be responsible for acute abdominal compartment syndrome, a potentially lethal complex pathological state in which an acute increase in intra-abdominal pressure may provoke the failure of several organ systems. Case presentation We report a case of acute abdominal compartment syndrome after perforation of the bowel during a colonoscopy in a 60-year-old North African man with rectal cancer, resulting in respiratory distress, cyanosis and cardiac arrest. Our patient was treated by needle decompression after the failure of cardiopulmonary resuscitation. An emergency laparotomy with anterior resection, including the perforated sigmoid colon, was then performed followed by immediate anastomosis. Our patient remains alive and free of disease three years later. Conclusion Acute abdominal compartment syndrome is a rare disease that may occasionally occur after a colonoscopic perforation. It should be kept in mind during colonoscopy, especially considering its simple salvage treatment.

  12. Os peroneum friction syndrome complicated by sesamoid fatigue fracture: a new radiological diagnosis? Case report and literature review.

    Science.gov (United States)

    Bashir, Waseem A; Lewis, Steve; Cullen, Nicholas; Connell, David A

    2009-02-01

    Injuries to the peroneal tendons are relatively common worldwide but tendon rupture without significant trauma is uncommon. Ankle mechanics can be seriously affected by disruption of one or both of the peroneal tendons although complete rupture can also remain asymptomatic. Accessory ossicles are sesamoid bones and are common findings in routine radiology of the foot and ankle. Although in the vast majority these "os" are normal variants of anatomy, they can lead to painful syndromes and suffer fractures and even undergo degenerative changes in response to overuse and trauma. Although similar syndromes have been discussed in the surgical literature, there is a lack of literature describing the use of modern imaging in the accurate diagnosis and its subsequent assistance towards appropriate management of os peroneum friction syndrome complicated by sesamoid fatigue syndrome. This article presents the plain film, sonographic and magnetic resonance imaging findings in a case of os peroneum friction syndrome complicated by a sesamoid fatigue fracture as well as reviewing the pertinent literature.

  13. Os peroneum friction syndrome complicated by sesamoid fatigue fracture: a new radiological diagnosis? Case report and literature review

    International Nuclear Information System (INIS)

    Bashir, Waseem A.; Connell, David A.; Lewis, Steve; Cullen, Nicholas

    2009-01-01

    Injuries to the peroneal tendons are relatively common worldwide but tendon rupture without significant trauma is uncommon. Ankle mechanics can be seriously affected by disruption of one or both of the peroneal tendons although complete rupture can also remain asymptomatic. Accessory ossicles are sesamoid bones and are common findings in routine radiology of the foot and ankle. Although in the vast majority these ''os'' are normal variants of anatomy, they can lead to painful syndromes and suffer fractures and even undergo degenerative changes in response to overuse and trauma. Although similar syndromes have been discussed in the surgical literature, there is a lack of literature describing the use of modern imaging in the accurate diagnosis and its subsequent assistance towards appropriate management of os peroneum friction syndrome complicated by sesamoid fatigue syndrome. This article presents the plain film, sonographic and magnetic resonance imaging findings in a case of os peroneum friction syndrome complicated by a sesamoid fatigue fracture as well as reviewing the pertinent literature. (orig.)

  14. Adalimumab for nail psoriasis: Efficacy and safety from the first 26 weeks of a phase 3, randomized, placebo-controlled trial.

    Science.gov (United States)

    Elewski, Boni E; Okun, Martin M; Papp, Kim; Baker, Christopher S; Crowley, Jeffrey J; Guillet, Gérard; Sundaram, Murali; Poulin, Yves; Gu, Yihua; Geng, Ziqian; Williams, David A; Rich, Phoebe A

    2018-01-01

    Previous clinical trials have not evaluated improvement in nail psoriasis as a primary end point. This phase 3 trial evaluated the safety and efficacy of adalimumab in patients with moderate-to-severe fingernail psoriasis and moderate-to-severe plaque psoriasis. Patients were randomized 1:1 to 40 mg adalimumab every other week or placebo. The primary efficacy end point was at least 75% improvement in total-fingernail modified Nail Psoriasis Severity Index (NAPSI75) response rate at week 26. Ranked secondary end point scores evaluated at week 26 were total-fingernail NAPSI and modified NAPSI, nail pain, Nail Psoriasis Physical Functioning Severity, Brigham Scalp Nail Inverse Palmo-Plantar Psoriasis Index, and Physician's Global Assessment (fingernail psoriasis). Of the 217 randomized patients (108 received placebo and 109 received adalimumab), 188 (86.6%) completed 26 weeks of treatment (period A) or escaped early to the open-label period. The study met the primary end point (response rate of 3.4% with placebo vs 46.6% with adalimumab [P psoriasis versus with placebo and no new safety risks were identified. Copyright © 2017 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.

  15. Clinical status, quality of life, and work productivity in Crohn's disease patients after one year of treatment with adalimumab.

    Science.gov (United States)

    Saro, Cristina; Ceballos, Daniel; Muñoz, Fernando; de la Coba, Cristóbal; Aguilar, María Dolores; Lázaro, Pablo; García-Sánchez, Valle; Hernández, Mariola; Barrio, Jesús; de Francisco, Ruth; Fernández, Luis I; Barreiro-de Acosta, Manuel

    2017-02-01

    Clinical trials have shown the efficacy of adalimumab in Crohn's disease, but the outcome in regular practice remains unknown. The aim of the study was to examine clinical status, quality of life, and work productivity of Crohn's disease patients receiving adalimumab for one year in the context of usual clinical practice. This was a prospective, observational study with a one-year follow-up. After baseline, Crohn's disease patients were evaluated at 1, 3, 6, 9, and 12 months after starting treatment with adalimumab. Outcome variables included: clinical status (measured with CDAI), quality of life (measured with EuroQoL-5D and IBDQ), and work productivity (measured with WPAI questionnaire). These outcome variables were compared using the Student's t test or Wilcoxon test for paired comparison data according to the data distribution. Statistical significance was set at two-sided p work productivity impact decreased from 53% to 24% (p quality of life and work productivity.

  16. Economy class syndrome: still a recurrent complication of long journeys.

    Science.gov (United States)

    Feltracco, Paolo; Barbieri, Stefania; Bertamini, Francesca; Michieletto, Elisa; Ori, Carlo

    2007-04-01

    Economy class syndrome is a rare but still unavoidable complication of long haul flights, particularly in patients who carry various intrinsic risk factors. The tendency to affect even asymptomatic young people and the greater risk to fragment and propagate to the pulmonary circulation are the main characteristics of deep vein thrombosis of long-flight travelers. We report the clinical history of eight patients admitted to intensive care unit for confirmed or highly suspected economy class syndrome. Seven of them developed the syndrome within 72 h from a long return flight, one suffered from pulmonary embolism after a 12-h car trip. Two out of eight patients died, one because of extremely severe hemodynamic impairment, the other as a consequence of multiple organ failure caused by a concomitant myocardial infarction. Deep vein thrombosis and pulmonary embolism represent one of the main medical problems of air travel and cause almost 20% of deaths in people with no medical history. Although economy class syndrome occurs mostly in elderly, even the healthy young population can be affected and, in fact, three out of eight patients of our series were under 50 years of age. All our patients but one carried a well recognized risk factor for deep vein thrombosis. Clinical symptoms of deep vein thrombosis can sometimes be aspecific and confusing, so that a certain proportion of post-travel deep vein thrombosis, evolving favorably and not giving rise to pulmonary embolism, might effectively remain undiagnosed. Economy class syndrome is still quite difficult to deal with and controversial in terms of preventive strategies.

  17. Switching from adalimumab to tofacitinib in the treatment of patients with rheumatoid arthritis

    NARCIS (Netherlands)

    Genovese, Mark C.; van Vollenhoven, Ronald F.; Wilkinson, Bethanie; Wang, Lisy; Zwillich, Samuel H.; Gruben, David; Biswas, Pinaki; Riese, Richard; Takiya, Liza; Jones, Thomas V.

    2016-01-01

    Tofacitinib is an oral Janus kinase inhibitor for the treatment of rheumatoid arthritis (RA). The aim of this study was to explore the safety and efficacy of open-label tofacitinib following blinded treatment with adalimumab or tofacitinib for moderate to severe RA. Analyses included patients

  18. Efficacy and safety of the biosimilar ABP 501 compared with adalimumab in patients with moderate to severe rheumatoid arthritis: a randomised, double-blind, phase III equivalence study.

    Science.gov (United States)

    Cohen, Stanley; Genovese, Mark C; Choy, Ernest; Perez-Ruiz, Fernando; Matsumoto, Alan; Pavelka, Karel; Pablos, Jose L; Rizzo, Warren; Hrycaj, Pawel; Zhang, Nan; Shergy, William; Kaur, Primal

    2017-10-01

    ABP 501 is a Food and Drug Administration-approved biosimilar to adalimumab; structural, functional and pharmacokinetic evaluations have shown that the two are highly similar. We report results from a phase III study comparing efficacy, safety and immunogenicity between ABP 501 and adalimumab. In this randomised, double-blind, active comparator-controlled, 26-week equivalence study, patients with moderate to severe active rheumatoid arthritis (RA) despite methotrexate were randomised (1:1) to ABP 501 or adalimumab (40 mg) every 2 weeks. Primary endpoint was risk ratio (RR) of ACR20 between groups at week 24. Primary hypothesis that the treatments were equivalent would be confirmed if the 90% CI for RR of ACR20 at week 24 fell between 0.738 and 1.355, demonstrating that ABP 501 is similar to adalimumab. Secondary endpoints included Disease Activity Score 28-joint count-C reactive protein (DAS28-CRP). Safety was assessed via adverse events (AEs) and laboratory evaluations. Antidrug antibodies were assessed to determine immunogenicity. A total of 526 patients were randomised (n=264, ABP 501; n=262 adalimumab) and 494 completed the study. ACR20 response at week 24 was 74.6% (ABP 501) and 72.4% (adalimumab). At week 24, the RR of ACR20 (90% CI) between groups was 1.039 (0.954, 1.133), confirming the primary hypothesis. Changes from baseline in DAS28-CRP, ACR50 and ACR70 were similar. There were no clinically meaningful differences in AEs and laboratory abnormalities. A total of 38.3% (ABP 501) and 38.2% (adalimumab) of patients tested positive for binding antidrug antibodies. Results from this study demonstrate that ABP 501 is similar to adalimumab in clinical efficacy, safety and immunogenicity in patients with moderate to severe RA. NCT01970475; Results. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  19. Usefulness of Adalimumab in the Treatment of Refractory Uveitis Associated with Juvenile Idiopathic Arthritis

    Science.gov (United States)

    García-De-Vicuña, Carmen; Díaz-Llopis, Manuel; Salom, David; Bou, Rosa; Díaz-Cascajosa, Jesus; Cordero-Coma, Miguel; Ortega, Gabriela; Ortego-Centeno, Norberto; Suarez-De-Figueroa, Marta; Cruz-Martínez, Juan; Fonollosa, Alex; Blanco, Ricardo; García-Aparicio, Ángel María; Benítez-Del-Castillo, Jose M.; Antón, Jordi

    2013-01-01

    Purpose. To assess the efficacy and safety of adalimumab in patients with juvenile idiopathic arthritis (JIA) and associated refractory uveitis. Design. Multicenter, prospective case series. Methods. Thirty-nine patients (mean [SD] age of 11.5 [7.9] years) with JIA-associated uveitis who were either not responsive to standard immunosuppressive therapy or intolerant to it were enrolled. Patients aged 13–17 years were treated with 40 mg of adalimumab every other week for 6 months and those aged 4–12 years received 24 mg/m2 body surface. Results. Inflammation of the anterior chamber (2.02 [1.16] versus 0.42 [0.62]) and of the posterior segment (2.38 [2.97] versus 0.35 [0.71] decreased significantly between baseline and the final visit (P treatment for JIA-associated refractory uveitis and may reduce steroid requirement. PMID:24489444

  20. Cerebellar Mutism Syndrome and Other Complications After Surgery in the Posterior Fossa in Adults

    DEFF Research Database (Denmark)

    Wibroe, Morten; Rochat, Per; Juhler, Marianne

    2018-01-01

    BACKGROUND: Cerebellar mutism syndrome (CMS) is rarely described in adults; however, data on self-assessed linguistic complications after posterior fossa surgery do not exist. METHODS: Through a prospective single-center study, data on 59 tumor operations in the posterior fossa were collected pre...

  1. Microcephalic osteodysplastic primordial dwarfism type II (MOPD II) with multiple vascular complications misdiagnosed as Dubowitz syndrome.

    Science.gov (United States)

    Dieks, Jana-Katharina; Baumer, Alessandra; Wilichowski, Ekkehard; Rauch, Anita; Sigler, Matthias

    2014-09-01

    To date, the genetic basis of Dubowitz syndrome (short stature, microcephaly, facial abnormalities, eczema) is unknown and vascular complications are not known to be associated with this syndrome. In microcephalic osteodysplastic primordial dwarfism type II (MOPD II; disproportionate short statue, microcephaly, facial abnormalities), however, cerebral aneurysms and other vascular abnormalities are frequent complications. MOPD II is a genetic disorder caused by mutations in the pericentrin (PCNT) gene (21q22). We report on a patient who came to our attention as a 22-year-old with subarachnoid bleeding due to a ruptured cranial aneurysm. Until then, the patient was thought and published to have Dubowitz syndrome; previously, he was treated with coronary bypass surgery for extensive coronary angiopathy. Consecutive genetic testing revealed MOPD II. After clinical stabilization, the patient was discharged to a specialized rehabilitation center where he died due to re-rupture of a cranial aneurysm. In patients with short stature-especially when clinical features are accompanied by vascular complications-MOPD II should be considered as a differential diagnosis leading to consecutive genetic testing. After detection of mutations in the PCNT gene, a full vascular status including cerebral imaging and cardiac evaluation needs to be determined in order to analyze vascular abnormalities and initiate prophylactic treatment.

  2. Os peroneum friction syndrome complicated by sesamoid fatigue fracture: a new radiological diagnosis? Case report and literature review

    Energy Technology Data Exchange (ETDEWEB)

    Bashir, Waseem A.; Connell, David A. [Royal National Orthopaedic Hospital NHS Trust, Department of Radiology, London, Middlesex (United Kingdom); Lewis, Steve [Craven Cottage, Fulham Football Club, London (United Kingdom); Cullen, Nicholas [The Royal National Orthopaedic Hospital NHS Trust, Department of Orthopaedics, London, Middlesex (United Kingdom)

    2009-02-15

    Injuries to the peroneal tendons are relatively common worldwide but tendon rupture without significant trauma is uncommon. Ankle mechanics can be seriously affected by disruption of one or both of the peroneal tendons although complete rupture can also remain asymptomatic. Accessory ossicles are sesamoid bones and are common findings in routine radiology of the foot and ankle. Although in the vast majority these ''os'' are normal variants of anatomy, they can lead to painful syndromes and suffer fractures and even undergo degenerative changes in response to overuse and trauma. Although similar syndromes have been discussed in the surgical literature, there is a lack of literature describing the use of modern imaging in the accurate diagnosis and its subsequent assistance towards appropriate management of os peroneum friction syndrome complicated by sesamoid fatigue syndrome. This article presents the plain film, sonographic and magnetic resonance imaging findings in a case of os peroneum friction syndrome complicated by a sesamoid fatigue fracture as well as reviewing the pertinent literature. (orig.)

  3. Metabolic complications in oncology

    International Nuclear Information System (INIS)

    Sycova-Mila, Z.

    2012-01-01

    Currently, a lot of space and time is devoted to the therapy of oncologic diseases itself. To reach the good therapy results, complex care of the oncologic patient is needed. Management of complications linked with the disease itself and management of complications emerged after administration of chemotherapy, radiotherapy or targeted therapy, plays a significant role. In addition to infectious, hematological, neurological, cardiac or other complications, metabolic complications are relatively extensive and serious. One of the most frequent metabolic complications in oncology is tumor lysis syndrome, hyperuricemia, hypercalcaemia and syndrome of inappropriate secretion of antidiuretic hormone. (author)

  4. ADALIMUMAB FOR MAINTENANCE THERAPY FOR ONE YEAR IN CROHN?S DISEASE: results of a Latin American single-center observational study

    Directory of Open Access Journals (Sweden)

    Paulo Gustavo KOTZE

    2014-03-01

    Full Text Available Context Adalimumab is a fully-human antibody that inhibits TNF alpha, with a significant efficacy for long-term maintenance of remission. Studies with this agent in Latin American Crohn’s disease patients are scarce. Objectives The objective of this study was to outline clinical remission rates after 12 months of adalimumab therapy for Crohn’s disease patients. Methods Retrospective, single-center, observational study of a Brazilian case series of Crohn’s disease patients under adalimumab therapy. Variables analyzed: demographic data, Montreal classification, concomitant medication, remission rates after 1, 4, 6 and 12 months. Remission was defined as Harvey-Bradshaw Index ≤4, and non-responder-imputation and last-observation-carried-forward analysis were used. The influence of infliximab on remission rates was analyzed by Fischer and Chi-square tests (P<0.05. Results Fifty patients, with median age of 35 years at therapy initiation, were included. Remission rates after 12 months of therapy were 54% under non-responder-imputation and 88% under last-observation-carried-forward analysis. After 12 months, remission on patients with previous infliximab occurred in 69.23% as compared to 94.59% in infliximab-naïve patients (P = 0.033. Conclusions Adalimumab was effective in maintaining clinical remission after 12 months of therapy, with an adequate safety profile, and was also more effective in infliximab naïve patients.

  5. Type IV neonatal Bartter syndrome complicated with congenital chloride diarrhea.

    Science.gov (United States)

    Sakallı, Hale; Bucak, Hakan İbrahim

    2012-01-01

    Pseudo-Bartter syndrome encompasses a heterogenous group of disorders similar to Bartter syndrome. Sometimes a few status may be nested, as in our case presented here. An 8-month-old boy was referred to our hospital with of intractable diarrhea, polyuria, persistent hypokalemia, abdominal distension and failure to thrive. He was born in the 34 6/7 gestational week (GW) to consanguineous parents. In the 30(th) GW polyhydramnios was verified by ultrasonography. The laboratory results showed hypokalemic-hypochloremic metabolic alkalosis, hyponatremia, and increased urinary loss of chloride, potassium and calcium. An audiogram test revealed complete sensorineural deafness. Ultrasonography revealed medullary nephrocalcinosis in both kidneys. Elevated plasma renin activity and aldosterone were found and a provisional diagnosis of type-IV neonatal Bartter syndrome was made. Treatment with indomethacin, spironolactone and additional intake of NaCl/KCl was initiated. Despite these therapies, the child's diarrhea persisted but serum potassium concentration normalized, and hypercalciuria and urine output reduced. After determining the high fecal chloride concentration, there was an immediate decompensation of the disease on indomethacin withdrawal, thus a diagnosis of type IV neonatal Bartter syndrome complicated with congenital chloride diarrhea was considered. Indomethacin, spironolactone and supplementary therapies with NaCl/KCl were continued, which resulted in the normalization of serum electrolytes as well as his physical development, but high contents of chloride in urine and faeces and nephrocalcinosis remains unchanged during 1-year follow-up. Because of the clinical and laboratory simulations between the various diseases that lead to hypokalemic-hypochloremic metabolic alkalosis, patients must be evaluated carefully.

  6. Concentrations of Adalimumab and Infliximab in Mothers and Newborns, and Effects on Infection

    DEFF Research Database (Denmark)

    Julsgaard, Mette; Christensen, Lisbet Ambrosius; Gibson, Peter R.

    2016-01-01

    these correlated with drug concentrations in mothers at birth and risk of infection during the first year of life.  Methods  We performed a prospective study of 80 pregnant women with inflammatory bowel diseases at tertiary hospitals in Denmark, Australia, and New Zealand from March 2012 through November 2014: 36......) and in mothers at time of birth (adalimumab, r = -0.80; infliximab, r = -0.80; P mother drug concentration at birth was 1.21 for adalimumab (95% confidence interval [CI], 0.94–1.49) and 1.97 for infliximab (95% CI, 1.50–2.43). The mean time to drug clearance...... risk for infection was 2.7 in infants whose mothers received the combination of an anti-TNF agent and thiopurine, compared with anti-TNF monotherapy (95% CI, 1.09–6.78; P =.02).  Conclusions  In a prospective study of infants born to mothers who received anti-TNF agents during pregnancy, we detected...

  7. Crohn's disease complicated by Epstein-Barr virus-driven haemophagocytic lymphohistiocytosis successfully treated with rituximab.

    Science.gov (United States)

    Thompson, Grace; Pepperell, Dominic; Lawrence, Ian; McGettigan, Benjamin David

    2017-02-22

    We report a case of Epstein-Barr virus (EBV)-driven haemophagocytic lymphohistiocytosis (HLH) in a man with Crohn's disease treated with 6-mercaptopurine and adalimumab therapy who was successfully treated with rituximab therapy alone. This is the first published case in an adult patient with EBV-driven HLH in the setting of thiopurine use and inflammatory bowel disease to be successfully treated with rituximab therapy alone. Here, we will discuss putative immunological mechanisms which may contribute to this potentially life-threatening complication. 2017 BMJ Publishing Group Ltd.

  8. Use of sequential diagnostic pain blocks in a patient of posttraumatic complex regional pain syndrome-not otherwise specified complicated by myofascial trigger points and thoracolumbar pain syndrome

    Directory of Open Access Journals (Sweden)

    Kailash Kothari

    2017-01-01

    Full Text Available We are presenting a case of posttraumatic lower limb Complex regional pain syndrome – Not otherwise specified (CRPS – NOS. As it was not treated in acute phase, the pain became chronic and got complicated by myofascial and thoracolumbar pain syndrome. This case posed us a diagnostic challenge. We used sequential diagnostic pain blocks to identify the pain generators and successfully treat the patient. We used diagnostic blocks step by step to identify and treat pain generators – T12,L1 and L2 Facet joints, Lumbar sympathetic block for CRPS NOS and Trigger point injection with dry needling for myofascial pain syndrome. This case highlights the facet that additional pain generators unrelated to original pain may complicate the presentation. Identifying these pain generators requires out of box thinking and high index of suspicion.

  9. The number needed to treat for adalimumab, etanercept, and infliximab based on ACR50 response in three randomized controlled trials on established rheumatoid arthritis

    DEFF Research Database (Denmark)

    Kristensen, L E; Christensen, R; Bliddal, H

    2011-01-01

    To compare the efficacy of adalimumab, etanercept, and infliximab in patients with established rheumatoid arthritis (RA) taking concomitant methotrexate (MTX) by calculating the number needed to treat (NNT) using three different methods.......To compare the efficacy of adalimumab, etanercept, and infliximab in patients with established rheumatoid arthritis (RA) taking concomitant methotrexate (MTX) by calculating the number needed to treat (NNT) using three different methods....

  10. The number needed to treat for adalimumab, etanercept, and infliximab based on ACR50 response in three randomized controlled trials on established rheumatoid arthritis

    DEFF Research Database (Denmark)

    Kristensen, L E; Christensen, R; Bliddal, H

    2007-01-01

    To compare the efficacy of adalimumab, etanercept, and infliximab in patients with established rheumatoid arthritis (RA) taking concomitant methotrexate (MTX) by calculating the number needed to treat (NNT) using three different methods.......To compare the efficacy of adalimumab, etanercept, and infliximab in patients with established rheumatoid arthritis (RA) taking concomitant methotrexate (MTX) by calculating the number needed to treat (NNT) using three different methods....

  11. Clinical similarity of the biosimilar ABP 501 compared with adalimumab after single transition: long-term results from a randomized controlled, double-blind, 52-week, phase III trial in patients with moderate-to-severe plaque psoriasis.

    Science.gov (United States)

    Papp, K; Bachelez, H; Costanzo, A; Foley, P; Gooderham, M; Kaur, P; Philipp, S; Spelman, L; Zhang, N; Strober, B

    2017-12-01

    ABP 501, a U.S.A. Food and Drug Administration- and European Medicines Agency-approved biosimilar, is highly similar to adalimumab in structure, function and pharmacokinetics. To demonstrate similarity in efficacy, safety and immunogenicity of ABP 501 vs. adalimumab for moderate-to-severe plaque psoriasis (clinical trial: NCT01970488). Patients were randomized (1 : 1) to receive ABP 501 or adalimumab 40 mg every 2 weeks for 16 weeks. At week 16, patients with ≥ 50% improvement from baseline in Psoriasis Area and Severity Index (PASI) score were eligible to continue to week 52. Patients receiving ABP 501 continued; adalimumab patients were rerandomized (1 : 1) to continue adalimumab or undergo a single transition to ABP 501. Key efficacy assessments included percentage PASI improvement from baseline, PASI responders and mean change in affected body surface area from baseline to weeks 16, 32 and 50. Safety was monitored via adverse events (AEs) and antidrug antibodies (ADAs) were assessed. A total of 308 patients were rerandomized at week 16 (ABP 501/ABP 501, n = 152; adalimumab/adalimumab, n = 79; adalimumab/ABP 501, n = 77). PASI percentage improvements from baseline were similar across groups for weeks 16, 32 and 50 (range: 85·8-88·2%), with no significant differences detected across groups in percentages of PASI 50, 75, 90 and 100 responders. Changes from baseline in percentage body surface area affected were similar across groups and time points. No new safety signals were detected. AEs were balanced between groups. Percentages of patients with binding and neutralizing ADAs were similar across treatments. ABP 501 and adalimumab have similar clinical efficacy, safety and immunogenicity profiles over 52 weeks, including after single transition, in this patient population. © 2017 British Association of Dermatologists.

  12. Compartment syndrome of the thigh complicating surgical treatment of ipsilateral femur and ankle fractures

    Science.gov (United States)

    Moore, M. R.; Garfin, S. R.; Hargens, A. R.

    1987-01-01

    A 26-year-old man presented with ipsilateral femur and ankle fractures. The patient was treated with interlocking nail of his femur fracture, followed by open reduction and internal fixation of his ankle fracture under tourniquet control. Postoperatively, the patient developed compartment syndrome of his thigh with elevated pressures, requiring decompressive fasciotomies. This case illustrates the possible complication of treating a femur fracture with intramedullary nailing and then immediately applying a tourniquet to treat an ipsilateral extremity fracture. Because of the complication with this patient, we feel the procedure should be staged, or a tourniquet should be avoided if possible.

  13. Similar Improvements in Patient-Reported Outcomes Among Rheumatoid Arthritis Patients Treated with Two Different Doses of Methotrexate in Combination with Adalimumab: Results From the MUSICA Trial.

    Science.gov (United States)

    Kaeley, Gurjit S; MacCarter, Daryl K; Goyal, Janak R; Liu, Shufang; Chen, Kun; Griffith, Jennifer; Kupper, Hartmut; Garg, Vishvas; Kalabic, Jasmina

    2018-06-01

    In patients with rheumatoid arthritis (RA), combination treatment with methotrexate (MTX) and adalimumab is more effective than MTX monotherapy. From the patients' perspective, the impact of reduced MTX doses upon initiating adalimumab is not known. The objective was to evaluate the effects of low and high MTX doses in combination with adalimumab initiation on patient-reported outcomes (PROs), in MTX-inadequate responders (MTX-IR) with moderate-to-severe RA. MUSICA was a randomized, double-blind, controlled trial evaluating the efficacy of 7.5 or 20 mg/week MTX, in combination with adalimumab for 24 weeks in MTX-IR RA patients receiving prior MTX ≥ 15 mg/week for ≥ 12 weeks. PROs were recorded at each visit, including physical function, health-related quality-of-life, work productivity, quality-of-sleep, satisfaction with treatment medication, sexual impairment due to RA, patient global assessment of disease activity (PGA), and patient pain. Last observation carried forward was used to account for missing values. At baseline, patients in both MTX dosage groups had similar demographics, disease characteristics, and PRO scores. Overall, initiation of adalimumab led to significant improvements from baseline in the PROs assessed for both MTX dosage groups. Improvements in presenteeism from baseline were strongly correlated with corresponding improvements in SF-36 (vitality), pain, and physical function. Physical and mental well-being had a good correlation with improvement in sleep. Overall, improvements in disease activity from baseline were correlated with improvements in several PROs. The addition of adalimumab to MTX in MTX-IR patients with moderate-to-severe RA led to improvements in physical function, quality-of-life, work productivity, quality of sleep, satisfaction with treatment medication, and sexual impairment due to RA, regardless of the concomitant MTX dosage. AbbVie. Clinicaltrials.gov identifier, NCT01185288.

  14. Hypothalamic-Pituitary-Adrenal Suppression and Iatrogenic Cushing's Syndrome as a Complication of Epidural Steroid Injections

    Directory of Open Access Journals (Sweden)

    Joyce Leary

    2013-01-01

    Full Text Available Epidural steroid injections are well accepted as a treatment for radicular back pain in appropriate candidates. While overall incidence of systemic side effects has not been well established, at least five biochemically proven cases of iatrogenic Cushing's Syndrome have been reported as complications of epidural steroid treatment. We present an additional case of iatrogenic Cushing's Syndrome and adrenal suppression in a middle-aged woman who received three epidural steroid injections over a four-month period. We review this case in the context of previous cases and discuss diagnostic and management issues.

  15. Efficacy and Safety of Adalimumab in Canadian Patients with Moderate to Severe Crohn’s Disease: Results of the Adalimumab in Canadian SubjeCts with ModErate to Severe Crohn’s DiseaSe (ACCESS Trial

    Directory of Open Access Journals (Sweden)

    Remo Panaccione

    2011-01-01

    Full Text Available OBJECTIVE: To evaluate open-label adalimumab therapy for clinical effectiveness, fistula healing, patient-reported outcomes and safety in Canadian patients with moderate to severe Crohn’s disease (CD who were either naive to or previously exposed to antitumour necrosis factor (anti-TNF therapy.

  16. Long QT syndrome and torsades de pointes complicating mitral valve replacement

    Directory of Open Access Journals (Sweden)

    Shegu Gilbert

    2016-09-01

    Full Text Available Severe QT interval prolongation >500 ms occurs in one quarter of cardiac surgical patients in the perioperative period while moderate prolongation occurs in most of them. Prolonged QT interval may be associated with torsades de pointes and lead to sudden cardiac death. Because of the high incidence of prolonged QT in cardiac surgery patients and its perioperative adverse outcomes, it is vital to identify it early and take necessary precautions. We report and discuss the catastrophic events and management of two patients with long QT syndrome complicating mitral valve replacement.

  17. Deterimental complication of meconium aspiration syndrome and their impact on outcome

    International Nuclear Information System (INIS)

    Shaikh, M.; Waheed, K.A.I.; Javaid, S.; Gul, R.

    2016-01-01

    Meconium aspiration syndrome (MAS) is respiratory distress in an infant born through meconium stained amniotic fluid (MSAF) whose signs cannot be otherwise explained. MAS is associated with different complications. Many studies have been conducted in developed world to find the outcome associated with MAS but data from developing countries is lacking. Present study was conducted to determine the impact of chemical pneumonitis, pulmonary hypertension (PHN) and air leak on outcome of new-borns with MAS. Methods: This cross sectional descriptive study was conducted in a tertiary care Neonatology unit. The babies diagnosed with MAS were included in the study. All patients were monitored for development of complications. Outcome in terms of mortality was recorded. Outcome was compared by chi-square test and p-value <0.05 is considered significant. Results: Seventy two babies were included in the study. Mean gestation was 37 ± 0.56 weeks and birth weight was 2.87 ± 0.49 kg. Male to female ratio was 1.57:1 and mean age of admission was 9.59 ± 5.27 hours. Chemical pneumonitis, PHN and chemical pneumonitis with PHN were observed in 23.6%, 20.8% and 26.4% neonates respectively. Mortality rate was 19.44%. The mortality was highest in babies having chemical pneumonitis with PHN (p-value=0.013) followed by chemical pneumonitis group (p-value=0.02) and PHN group (p-value=0.032). Conclusion: About three fourth babies with MAS developed one or more complications. Mortality is significantly increases with the development of complications so as more the complications more is the mortality. (author)

  18. Infectious Complications in Children With Acute Myeloid Leukemia and Down Syndrome: Analysis of the Prospective Multicenter Trial AML-BFM 2004.

    Science.gov (United States)

    Hassler, Angela; Bochennek, Konrad; Gilfert, Julia; Perner, Corinna; Schöning, Stefan; Creutzig, Ursula; Reinhardt, Dirk; Lehrnbecher, Thomas

    2016-06-01

    Children with acute myeloid leukemia (AML) and Down syndrome have high survival rates with intensity-reduced chemotherapeutic regimens, although the optimal balance between dose intensity and treatment toxicity has not been determined. We, therefore, characterized infectious complications in children with AML and Down syndrome treated according to AML-BFM 2004 study (ClinicalTrials.gov NCT00111345; amended 2006 for Down syndrome with reduced intensity). Data on infectious complications were gathered from the medical records in the hospital where the patient was treated. Infectious complications were categorized as fever without identifiable source (FUO), or as microbiologically or clinically documented infections. A total of 157 infections occurred in 61 patients (60.5% FUO, 9.6% and 29.9% clinically and microbiologically documented infections, respectively). Almost 90% of the pathogens isolated from the bloodstream were Gram-positive bacteria, and approximately half of them were viridans group streptococci. All seven microbiologically documented episodes of pneumonia were caused by viruses. Infection-related mortality was 4.9%, and all three patients died due to viral infection. Our data demonstrate that a reduced-intensity chemotherapeutic regimen in children with AML and Down syndrome is still associated with high morbidity. Although no patient died due to bacteria or fungi, viruses were responsible for all lethal events. Future studies, therefore, have to focus on the impact of viruses on morbidity and mortality of patients with AML and Down syndrome. © 2016 Wiley Periodicals, Inc.

  19. Wish to conceive and concerns to develop cardiovascular complications during pregnancy in patients with Turner syndrome

    NARCIS (Netherlands)

    van Hagen, Iris M.; Duijnhouwer, Anthonie L.; ten Kate-Booij, Marianne J.; Dykgraaf, Ramon H. M.; Duvekot, Johannes J.; Utens, Elisabeth M. W. J.; Roos-Hesselink, Jolien W.

    2017-01-01

    Turner syndrome (TS) is associated with subfertility and infertility. Nevertheless, an increasing number of women become pregnant through oocyte donation. The wish to conceive may be negatively influenced by the fear of cardiovascular complications. The aim was to investigate the wish to conceive

  20. Drug persistence and need for dose intensification to adalimumab therapy; the importance of therapeutic drug monitoring in inflammatory bowel diseases.

    Science.gov (United States)

    Gonczi, Lorant; Kurti, Zsuzsanna; Rutka, Mariann; Vegh, Zsuzsanna; Farkas, Klaudia; Lovasz, Barbara D; Golovics, Petra A; Gecse, Krisztina B; Szalay, Balazs; Molnar, Tamas; Lakatos, Peter L

    2017-08-08

    Therapeutic drug monitoring (TDM) aid therapeutic decision making in patients with inflammatory bowel disease (IBD) who lose response to anti-TNF therapy. Our aim was to evaluate the frequency and predictive factors of loss of response (LOR) to adalimumab using TDM in IBD patients. One hundred twelve IBD patients (with 214 TDM measurements, CD/UC 84/28, male/female 50/62, mean age CD/UC: 36/35 years) were enrolled in this consecutive cohort from two referral centres in Hungary. Demographic data were comprehensively collected and harmonized monitoring strategy was applied. Previous and current therapy, laboratory data and clinical activity were recorded at the time of TDM. Patients were evaluated either at the time of suspected LOR or during follow-up. TDM measurements were determined by commercial ELISA (LISA TRACKER, Theradiag, France). Among 112 IBD patients, LOR/drug persistence was 25.9%/74.1%. The cumulative ADA positivity (>10 ng/mL) and low TL (<5.0 μg/mL) was 12.1% and 17.8% after 1 year and 17.3% and 29.5% after 2 years of adalimumab therapy. Dose intensification was needed in 29.5% of the patients. Female gender and ADA positivity were associated with LOR (female gender: p < 0.001, OR:7.8 CI 95%: 2.5-24.3, ADA positivity: p = 0.007 OR:3.6 CI 95%: 1.4-9.5). ADA development, low TL and need for dose intensification were frequent during adalimumab therapy and support the selective use of TDM in IBD patients treated with adalimumab. ADA positivity and gender were predictors of LOR.

  1. [Prevention and treatment of the complications of polycystic ovarian syndrome--the significance of evidence-based, interdisciplinary management].

    Science.gov (United States)

    Gődény, Sándor; Csenteri, Orsolya Karola

    2015-12-13

    Polycystic ovary syndrome is the most common hormonal and metabolic disorder likely to affect women. The syndrome is often associated with obesity, hyperinsulinemia and adversely affects endocrine, metabolic, and cardiovascular health. The complex feature of the syndrome requires an interdisciplinary approach to treatment, where cooperation of paediatrician, internist, gynaecologist, endocrinologist, dermatologist, psychologist and oncologist is essential. The prevention and the treatment should be based on the best available evidence. This should include physical examination, laboratory tests for hormones, serum insulin, glucose, lipids, in addition patient's preferences should be considered, too. To maximise health gain of polycystic ovarian syndrome, adequate, effective, efficient and safe treatment is necessary. This article summarises the highest available evidence provided by meta-analyses and systematic reviews of the prevention of metabolic and cardiovascular complications of the syndrome, and discusses the relevant evidence published in the literature.

  2. Predictors of adalimumab drug survival in psoriasis differ by reason for discontinuation: long-term results from the Bio-CAPTURE registry.

    Science.gov (United States)

    van den Reek, J M P A; Tummers, M; Zweegers, J; Seyger, M M B; van Lümig, P P M; Driessen, R J B; van de Kerkhof, P C M; Kievit, W; de Jong, E M G J

    2015-03-01

    Drug survival is an indicator for treatment success; insight in predictors associated with drug survival is important. To analyse the long-term drug survival for adalimumab in patients with psoriasis treated in daily practice and (II) to identify predictors of prolonged drug survival for adalimumab split for different reasons of discontinuation. Data were extracted from a prospective psoriasis cohort and analysed using Kaplan-Meier survival curves split for reasons of discontinuation. Baseline predictors associated with longer drug survival were identified using multivariate Cox-regression analysis. One hundred and sixteen patients were included with a total of 208 patient-years. Overall drug survival was 76% after 1 year and 52% after 4.5 years. In patients who stopped due to ineffectiveness, longer drug survival was associated with the absence of specific comorbidities (P = 0.03). In patients who stopped due to side-effects, longer drug survival was associated with male gender (P = 0.02). Predictors of adalimumab drug survival in psoriasis differ by reason for discontinuation. Strong, specific predictors can lead to patient-tailored treatment. © 2014 European Academy of Dermatology and Venereology.

  3. Anaesthesia-related haemodynamic complications in Williams syndrome patients: a review of one institution's experience.

    Science.gov (United States)

    Olsen, M; Fahy, C J; Costi, D A; Kelly, A J; Burgoyne, L L

    2014-09-01

    Williams syndrome is a genetic disorder associated with cardiac pathology, including supravalvular aortic stenosis and coronary artery stenosis. Sudden cardiac death has been reported in the perioperative period and attributed to cardiovascular pathology. In this retrospective audit, case note and anaesthetic records were reviewed for all confirmed Williams syndrome patients who had received an anaesthetic in our institution between July 1974 and November 2009. There were a total of 108 anaesthetics administered in 29 patients. Twelve of the anaesthetics (11.1%) were associated with cardiac complications including cardiac arrest in two cases (1.85%). Of the two cardiac arrests, one patient died within the first 24 hours postanaesthetic and the other patient survived, giving an overall mortality of 0.9% (3.4%). We conclude that Williams syndrome confers a significant anaesthetic risk, which should be recognised and considered by clinicians planning procedures requiring general anaesthesia.

  4. Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) Complicated by Perforation of the Small Intestine and Cholecystitis.

    Science.gov (United States)

    Ohnuki, Yoichi; Moriya, Yusuke; Yutani, Sachiko; Mizuma, Atsushi; Nakayama, Taira; Ohnuki, Yuko; Uda, Shuji; Inomoto, Chie; Yamamoto, Soichiro; Nakamura, Naoya; Takizawa, Shunya

    2018-03-01

    We report a case of eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss syndrome) complicated by perforation of the small intestine and necrotizing cholecystitis. A 69-year-old man with a history of bronchial asthma was admitted with mononeuritis multiplex. The laboratory findings included remarkable eosinophilia. He was treated with corticosteroids and his laboratory indices showed improvement; however, his functional deficits remained. His neuropathy gradually improved after the addition of intravenous immunoglobulin (IVIG). He was subsequently treated with oral prednisolone (40 mg/day) as maintenance therapy. Within a month after finishing IVIG, he developed perforation of the small intestine and necrotizing cholecystitis. Intestinal perforation has often been reported as a gastrointestinal complication of EGPA. In contrast, cholecystitis is a rare complication. We report this case because the manifestation of more than one complication is extremely rare. Gastrointestinal symptoms may be a complication of EGPA itself and/or immunosuppressive treatment.

  5. Successful adalimumab treatment of a psoriasis vulgaris patient with hemodialysis for renal failure: A case report and a review of the previous reports on biologic treatments for psoriasis patients with hemodialysis for renal failure.

    Science.gov (United States)

    Kusakari, Yoshiyuki; Yamasaki, Kenshi; Takahashi, Toshiya; Tsuchiyama, Kenichiro; Shimada-Omori, Ryoko; Nasu-Tamabuchi, Mei; Aiba, Setsuya

    2015-07-01

    The efficacy and safety of biologic treatments have been established in patients with moderate to severe psoriasis, but there are few reports on biologic therapy for patients with psoriasis complicated by end-stage renal failure on hemodialysis (HD). In this report, we demonstrated the efficacy and safety of adalimumab for patients with severe psoriasis on HD. A 46-year-old Japanese man with a 14-year history of psoriasis was referred to our clinic in September 2009. He had developed hypertension and renal failure during a 7-year history of cyclosporin treatment. With the infliximab treatment, he achieved 75% improvement of the Psoriasis Area and Severity Index (PASI) score within 3 months from the PASI of 42.3 before the treatment. However, his renal failure gradually deteriorated, and HD was initiated at 1 year after the introduction of infliximab. Because of hydration during the i.v. injection of infliximab, he developed pulmonary edema with every infliximab treatment after starting HD. We switched to ustekinumab treatment, but his psoriasis was not improved. Then, we switched to adalimumab and achieved a PASI-100 response within 2 months. The patient received adalimumab treatment for more than a year without any adverse effects. In addition to our case, five articles reported cases of psoriasis patients with renal failure on HD who were treated with biologics. The psoriatic lesions were improved by biologics in these cases, and no severe adverse effects on the renal function were reported. Thus, biologics are a reasonable treatment option for patients with severe psoriasis with renal failure on HD. © 2015 Japanese Dermatological Association.

  6. Vascular Ehlers-Danlos Syndrome With a Novel Missense COL3A1 Mutation Present With Pulmonary Complications and Iliac Arterial Dissection.

    Science.gov (United States)

    Gu, Guangchao; Yang, Hang; Cui, Lijia; Fu, Yuanyuan; Li, Fangda; Zhou, Zhou; Zheng, Yuehong

    2018-02-01

    Vascular Ehlers-Danlos syndrome (vEDS) is a life-threatening connective tissue disorder due to its high tendency of arterial and organ rupture. Pulmonary complications in vEDS are rare. We present a young male patient with vEDS who developed severe pulmonary complications and severe rupture of the iliac artery at different stages of his life. Vascular Ehlers-Danlos syndrome was diagnosed based on clinical manifestations and confirmed by the identification of COL3A1 gene mutation. Due to high bleeding tendency and weak cardiopulmonary capacity, conservative treatment was taken for him. To our knowledge, this is the first report of vEDS case in which the patient developed both pulmonary complications and dissection of large arteries. Our report emphasizes the importance of considering vEDS when an adolescent develops unexplained pulmonary cysts with fragility of lung tissues. Genetic counseling and close monitoring should be performed for earlier diagnosis and prevention of severe complications of large arteries. The typical presentations of vEDS were also discussed by means of a review of case reports on vEDS with pulmonary complications.

  7. [A postpartum woman with toxic shock syndrome: group A streptococcal infection, a much feared postpartum complication.

    NARCIS (Netherlands)

    Abbink, K.; Kortekaas, J.C.; Buise, M.P.; Dokter, J.; Kuppens, S.M.; Hasaart, T.H.M.

    2016-01-01

    BACKGROUND: The development of toxic shock syndrome (TSS) after an invasive group A streptococcal (GAS) infection in the postpartum period is a much feared complication. The mortality rate of TSS with necrotizing fasciitis is 30 to 50%. CASE DESCRIPTION: We present the case of a woman with atypical

  8. Adalimumab for the treatment of Crohn’s disease

    Directory of Open Access Journals (Sweden)

    Andrea Cassinotti

    2008-09-01

    Full Text Available Andrea Cassinotti, Sandro Ardizzone, Gabriele Bianchi PorroDepartment of Clinical Sciences, Chair of Gastroenterology, “Luigi Sacco” University Hospital, Milan, ItalyIntroduction: Crohn’s disease (CD is a chronic inflammatory bowel disease characterized by a relapsing-remitting course with trans-mural inflammation of potentially any section of the digestive tract. Adalimumab (ADA is a subcutaneously administered, recombinant, fully human, IgG1 monoclonal antibody that binds with high affinity and specificity to human TNF-alpha, thus modulating its biologic functions and its proinflammatory effects.Aims: To review the available data on ADA in CD for biological properties, efficacy, and safety.Methods: Electronic searches were conducted using the Pubmed and SCOPUS databases from the earliest records to April 2008. The search terms used were “adalimumab”, “anti-TNF”, “TNF-alpha”, “biologicals”, “inflammatory bowel disease”, and “Crohn’s disease”. Reference lists of all relevant articles were searched for further studies.Results: Available studies suggest that ADA has the potential to induce and maintain clinical response and remission in moderate-severe CD, both in anti-TNF-naïve patients and in subjects who lost their response and/or became intolerant to infliximab (IFX. ADA seems also effective in maintaining corticosteroid-free remission and obtaining complete fistula closure (although no specific randomized trials are available. No concomitant immunosuppressors seem to be necessary. Side effects appear similar to IFX, while site-injection reactions are frequent and specific. Data on immunogenicity and its clinical impact are uncertain.Conclusions: ADA appears to be effective in inducing and maintain clinical remission in CD, including patients not manageable with IFX. Successive clinical practice and further on going trials will confirm a positive role for ADA as a new anti-TNF treatment in CD. The impact on

  9. Blockade of Tumor Necrosis Factor-Alpha: A Role for Adalimumab in Neovascular Age-Related Macular Degeneration Refractory to Anti-Angiogenesis Therapy

    Directory of Open Access Journals (Sweden)

    Beatriz Fernández-Vega

    2016-03-01

    Full Text Available Aims: To report a case of wet age-related macular degeneration (wet-AMD refractory to intravitreal anti-vascular endothelial growth factor (anti-VEGF therapy in a patient who showed visual and anatomical improvement and stabilization after starting a subcutaneous treatment course with adalimumab, an anti-tumor necrosis factor-alpha (TNF-α drug, for concomitant Crohn's disease. Methods: Observational case report of a female patient. Ophthalmological evaluation was performed by slit lamp and ophthalmoscopy (posterior pole and anterior segment. Best-corrected visual acuity (BCVA was determined, and imaging was performed by fluorescein angiography, indocyanine green angiography, and optical coherence tomography (OCT. Intravitreal therapies used and treatment with anti-TNF-α were recorded. Results: A 64-year-old woman with wet-AMD was treated with fourteen intravitreal injections of ranibizumab (0.5 mg for a period of 40 months with intervals of 1-6 months. She initially showed a good visual and anatomical response to periodic anti-VEGF treatment but during check visits, anatomical and functional responses deteriorated. At the 40-month follow-up, the patient had developed Crohn's disease, and her rheumatologist started treatment with adalimumab (40 mg subcutaneously every 2 weeks. During the 25 months of treatment with adalimumab, the patient did not require any additional intravitreal anti-VEGF treatments because her BCVA, clinical, and OCT findings improved and remained stable. Conclusions: We described a case of a patient with wet-AMD refractory to anti-VEGF therapy, which clinically benefited from subcutaneous adalimumab therapy. Treatment with subcutaneous anti-TNF-α in combination with anti-VEGF therapy avoids the high cost and risks related to multiple intravitreal anti-VEGF injections with good functional and anatomic outcomes.

  10. Conservative management of severe serotonin syndrome with coma, myoclonus, and crossed-extensor reflex complicated by hepatic encephalopathy.

    Science.gov (United States)

    Ramachandran, Vignesh; Ding, Belicia; George, Rollin; Novakovic, Matthew

    2018-01-01

    Serotonin syndrome (SS) is an underrecognized and potentially fatal disorder that occurs secondary to combinational use or overdose of a single serotonergic medication. The presentation may be complicated by hepatic encephalopathy in cirrhotic patients, which may also affect metabolism of these serotonergic agents. The authors report a rare case of severe SS complicated by hepatic encephalopathy secondary to cirrhosis in a 52-year-old woman after an increase in her home dosage of fluoxetine and addition of other psychiatric medications.

  11. Adalimumabe no tratamento da artrite reumatoide: uma revisão sistemática e metanálise de ensaios clínicos randomizados

    Directory of Open Access Journals (Sweden)

    Marina Amaral de Ávila Machado

    2013-10-01

    Full Text Available Desde a descoberta do papel do fator de necrose tumoral no processo fisiopatológico da artrite reumatoide, cinco medicamentos bloqueadores dessa citocina têm sido empregados como opção terapêutica. Para avaliar a eficácia e a segurança do adalimumabe no tratamento da artrite reumatoide foi conduzida uma revisão sistemática com metanálise de ensaios clínicos controlados e randomizados. Foi realizada busca de estudos relevantes nas bases de dados Medline (via PubMed e LILACS em junho de 2011. A seleção dos estudos, coleta e análise de dados foram realizadas de forma pareada e independente por dois revisores e por um terceiro revisor em casos de discordância. A metanálise foi conduzida no software Review Manager® 5.1 usando o modelo de efeitos aleatórios. Onze artigos referentes ao adalimumabe foram incluídos e contemplaram nove estudos com 3461 pacientes. Dez estudos mostraram baixo risco de viés quanto ao cegamento dos participantes e pessoal e cegamento de avaliação de resultados. Os pacientes que receberam tratamento da associação de adalimumabe e metotrexato apresentam melhores resultados de eficácia e menor progressão radiográfica quando comparados ao grupo placebo + metotrexato em 24 a 104 semanas. Os pacientes que utilizaram adalimumabe em monoterapia apresentaram melhores resultados de eficácia em relação ao placebo em 24 e 26 semanas. Os resultados das metanálises de eventos adversos não foram estatisticamente significantes, exceto para reações no local de aplicação, na qual favoreceu o grupo controle. A eficácia do adalimumabe foi demonstrada em monoterapia e associado a algum MMCD, porém as evidências para o uso combinado são mais robustas.

  12. Bouveret's syndrome complicated by distal gallstone ileus after laser lithotropsy using Holmium: YAG laser

    Directory of Open Access Journals (Sweden)

    Rodgers John B

    2002-06-01

    Full Text Available Abstract Background Bouveret's syndrome is an unusual presentation of duodenal obstruction caused by the passage of a large gallstone through a cholecystoduodenal fistula. Endoscopic therapy has been used as first-line treatment, especially in patients with high surgical risk. Case presentation We report a 67-year-old woman who underwent an endoscopic attempt to fragment and retrieve a duodenal stone using a Holmium: Yttrium-Aluminum-Garnet Laser (Ho:YAG which resulted in small bowel obstruction. The patient successfully underwent enterolithotomy without cholecystectomy or closure of the fistula. Conclusion We conclude that, distal gallstone obstruction, due to migration of partially fragmented stones, can occur as a possible complication of laser lithotripsy treatment of Bouveret's syndrome and might require urgent enterolithotomy.

  13. Extent and consequences of antibody formation against adalimumab in patients with psoriasis: one-year follow-up

    NARCIS (Netherlands)

    Menting, S.P.; Lumig, P.P.M. van; Vries, A.C. de; Reek, J.M.P.A. van den; Kleij, D. van der; Jong, E.M.G.J. de; Spuls, P.I.; Lecluse, L.L.

    2014-01-01

    IMPORTANCE: In a previously reported cohort of 29 patients with plaque-type psoriasis followed up for 24 weeks, clinically relevant antidrug antibody (ADA) to adalimumab was frequently found. Long-term data were lacking. We now present the extension of this study: 80 patients followed up for 1 year.

  14. Extent and Consequences of Antibody Formation Against Adalimumab in Patients With Psoriasis One-Year Follow-up

    NARCIS (Netherlands)

    Menting, Stef P.; van Lümig, Paula P. M.; de Vries, Anna-Christa Q.; van den Reek, Juul M. P. A.; van der Kleij, Desiree; de Jong, Elke M. G. J.; Spuls, Phyllis I.; Lecluse, Lidian L. A.

    2014-01-01

    IMPORTANCE In a previously reported cohort of 29 patients with plaque-type psoriasis followed up for 24 weeks, clinically relevant antidrug antibody (ADA) to adalimumab was frequently found. Long-term data were lacking. We now present the extension of this study: 80 patients followed up for 1 year.

  15. Analysis of the complications in interventional treatment for Budd-Chiari syndrome

    International Nuclear Information System (INIS)

    Niu Zhike; Lv Guanghua; Du Fei; Guo Yanxia; Guan Lan

    2010-01-01

    Objective: To discuss the causes and the prevention measures of the complications occurred after interventional therapy for different type of Budd-Chiari syndrome (BCS). Methods: Based on the type of BCS, the corresponding interventional management was adopted in 204 patients with BCS. The interventional procedures included PTA and stent placement of inferior vena cava (IVC), percutaneous transhepatic recanalization and dilation (PTRD) of hepatic vein, percutaneous trans jugular or trans inferior vena cava recanalization, dilation and stent placement of hepatic vein and trans jugular intrahepatic portal-systemic stenting shunt (TIPSS). Results: The successful rate of interventional therapy was 95.5% (21 / 22) for type Ia, 81.8% (9/11) for type Ib, 97.3% (109/112) for type IIa, 92.9% (13/14) for type IIb, 88.9% (8/9) for type IIIa, 100% (2/2) type IIIb, 92% (23/25) for type IVa and 88.9% (8/9) for type IVb BCS. The main complications occurred during or after the operation included acute cardiac insufficiency (n=2), pulmonary arterial embolization (n=4), disseminated intravascular coagulation (n=1), extravasation of contrast medium (n=3), arrhythmia (n=2), and cardiac tamponade (n=1). Conclusion: Interventional therapy is simple, safe and effective for the treatment of BCS, but its indications should be strictly considered and all kinds of effective prevention measures should be taken to avoid or to reduce the possible complications. (authors)

  16. Jugular thrombophlebitis complicating bacterial pharyngitis (Lemierre's syndrome)

    International Nuclear Information System (INIS)

    Sena, S.De; Rosenfeld, D.L.; Santos, S.; Keller, I.

    1996-01-01

    Lemierre's syndrome is a rare syndrome caused by Fusobacterium necrophorum, a gram-negative anaerobic organism that normally inhabits the oropharynx. The syndrome follows primary oropharyngeal infection and affects previously healthy adolescents in a characteristic manner with fatal results if left untreated. The authors present two cases seen at their institution and discuss the clinical and radiologic features of the syndrome, along with considerations for patient management. (orig.). With 5 figs

  17. Complications and challenges associated with polycystic ovary syndrome: current perspectives.

    Science.gov (United States)

    Palomba, Stefano; Santagni, Susanna; Falbo, Angela; La Sala, Giovanni Battista

    2015-01-01

    Polycystic ovary syndrome (PCOS) represents the most common endocrine dysfunction in fertile women and it is considered a heterogeneous and multifaceted disorder, with multiple reproductive and metabolic phenotypes which differently affect the early- and long-term syndrome's risks. Women with PCOS present an adverse reproductive profile, including a high risk of pregnancy-induced hypertension, preeclampsia, and gestational diabetes mellitus. Patients with PCOS present not only a higher prevalence of classic cardiovascular risk factors, such as hypertension, dyslipidemia, and type-2 diabetes mellitus, but also of nonclassic cardiovascular risk factors, including mood disorders, such as depression and anxiety. Moreover, at the moment, clinical data on cardiovascular morbidity and mortality in women with PCOS are controversial. Finally, women with PCOS show an increased risk of endometrial cancer compared to non-PCOS healthy women, particularly during premenopausal period. Currently, we are unable to clarify if the increased PCOS early- and long-term risks are totally due to PCOS per se or mostly due to obesity, in particular visceral obesity, that characterized the majority of PCOS patients. In any case, the main endocrine and gynecological scientific societies agree to consider women with PCOS at increased risk of obstetric, cardiometabolic, oncology, and psychological complications throughout life, and it is recommended that these women be accurately assessed with periodic follow-up.

  18. Tofacitinib or adalimumab versus placebo: patient-reported outcomes from a phase 3 study of active rheumatoid arthritis

    NARCIS (Netherlands)

    Strand, Vibeke; van Vollenhoven, Ronald F.; Lee, Eun Bong; Fleischmann, Roy; Zwillich, Samuel H.; Gruben, David; Koncz, Tamas; Wilkinson, Bethanie; Wallenstein, Gene

    2016-01-01

    To evaluate effects of tofacitinib or adalimumab on patient-reported outcomes (PROs) in patients with moderate to severe RA and inadequate responses to MTX. In this 12-month, phase 3, randomized controlled trial (ORAL Standard), patients (n = 717) receiving background MTX were randomized to

  19. MRI examination of West syndrome complicated by periventricular leukomalacia (PVL)

    International Nuclear Information System (INIS)

    Fukuda, Kuniaki; Yamashita, Akiko; Endo, Shoichi; Gouda, Tomoko

    2001-01-01

    The magnetic resonance imaging (MRI) findings of 19 low-birth-weight infants with periventricular leukomalacia (PVL) and cerebral palsy, 6 with West syndrome as a complication (group III) and 13 without it (group II), were compared with those of normal 13 low-birth-weight infants without PVL or cerebral palsy (group I) to investigate the pathogenetic mechanism of West syndrome. All of the subjects were born between 1989 and 1997, and there were no significant differences between the three groups in birth weight or age at the time of the examination. Although the gestation period was significantly longer in the group III (infants with West syndrome) than in group I (control infants), there was no significant difference was seen between group I and group II (infants with PVL and cerebral palsy). PVL was diagnosed by MRI, and the diagnostic standards included periventricular high signal intensity on T2-weighted images, decreased cerebral white matter, and deformity or enlargement of the lateral ventricles. The area of periventricular high signal intensity was significantly larger in group II and group III than in group I, and the occipital lobe white matter was significantly thinner in the former than the latter. The lateral ventricles were significantly larger in group II than in the other two groups. The area of the corpus callosum and the thickness of the splenium and trunk of the corpus callosum were significantly smaller in group II and group III than in group I, but the corpus callosum was significantly shorter in group III than in the other two groups. Findings in the brain stem showed a significantly smaller pons and medulla oblongata in group III than in group I, but there were no significant differences between group II and group I. The results of this study indicate that lower brainstem lesions as well as cerebral lesions are associated with the development of West syndrome. (K.H.)

  20. MRI examination of West syndrome complicated by periventricular leukomalacia (PVL)

    Energy Technology Data Exchange (ETDEWEB)

    Fukuda, Kuniaki; Yamashita, Akiko; Endo, Shoichi [National Kagawa Children' s Hospital, Zentuji (Japan); Gouda, Tomoko

    2001-10-01

    The magnetic resonance imaging (MRI) findings of 19 low-birth-weight infants with periventricular leukomalacia (PVL) and cerebral palsy, 6 with West syndrome as a complication (group III) and 13 without it (group II), were compared with those of normal 13 low-birth-weight infants without PVL or cerebral palsy (group I) to investigate the pathogenetic mechanism of West syndrome. All of the subjects were born between 1989 and 1997, and there were no significant differences between the three groups in birth weight or age at the time of the examination. Although the gestation period was significantly longer in the group III (infants with West syndrome) than in group I (control infants), there was no significant difference was seen between group I and group II (infants with PVL and cerebral palsy). PVL was diagnosed by MRI, and the diagnostic standards included periventricular high signal intensity on T2-weighted images, decreased cerebral white matter, and deformity or enlargement of the lateral ventricles. The area of periventricular high signal intensity was significantly larger in group II and group III than in group I, and the occipital lobe white matter was significantly thinner in the former than the latter. The lateral ventricles were significantly larger in group II than in the other two groups. The area of the corpus callosum and the thickness of the splenium and trunk of the corpus callosum were significantly smaller in group II and group III than in group I, but the corpus callosum was significantly shorter in group III than in the other two groups. Findings in the brain stem showed a significantly smaller pons and medulla oblongata in group III than in group I, but there were no significant differences between group II and group I. The results of this study indicate that lower brainstem lesions as well as cerebral lesions are associated with the development of West syndrome. (K.H.)

  1. Pilot study of the safety and effect of adalimumab on pain, physical function, and musculoskeletal disease in mucopolysaccharidosis types I and II

    Directory of Open Access Journals (Sweden)

    Lynda E. Polgreen

    2017-03-01

    Full Text Available Mucopolysaccharidosis I and II are lysosomal storage disorders that, despite treatment with hematopoietic cell transplantation (HCT and/or enzyme replacement therapy (ERT, continue to cause significant skeletal abnormalities leading to pain, stiffness, physical dysfunction, and short stature. Tumor necrosis factor – alpha (TNF-α is elevated in individuals with MPS I and II and associated with pain and physical dysfunction. Therefore, we evaluated the safety and effects of the TNF-α inhibitor adalimumab in patients with MPS I and II in a 32-week, randomized, double blind, placebo-controlled, crossover study of adalimumab at a dose of 20 mg (weight 15–5° in seven of eight joints in Subject #1 and five of eight joints in Subject #2 (range 7.0° to 52.8°. There was no change in the PPQ, 6MWT, or hand dynamometer. Data from this small pilot study suggest that treatment with adalimumab is safe, tolerable, and may improve ROM, physical function, and possibly pain, in children with MPS I or II. However, additional clinical trials are needed before this therapy should be recommended as part of clinical care.

  2. Genetic polymorphism in ATG16L1 gene influences the response to adalimumab in Crohn's disease patients

    NARCIS (Netherlands)

    Koder, Silvo; Repnik, Katja; Ferkolj, Ivan; Pernat, Cvetka; Skok, Pavel; Weersma, Rinse K.; Potocnik, Uros

    2015-01-01

    Aim: To see if SNPs could help predict response to biological therapy using adalimumab (ADA) in Crohn's disease (CD). Materials & methods: IBDQ index and CRP levels were used to monitor therapy response. We genotyped 31 CD-associated genes in 102 Slovenian CD patients. Results: The strongest

  3. Treatment of infectious complications of acquired immunodeficiency syndrome.

    Science.gov (United States)

    Furio, M M; Wordell, C J

    1985-01-01

    The infectious complications of the acquired immunodeficiency syndrome (AIDS) are discussed, and the conventional and nonconventional therapies used for these infections are reviewed. The infections most commonly encountered in patients with AIDS are Pneumocystis carinii pneumonia (58%), Candida esophagitis (31%), toxoplasmosis (21%), cytomegalovirus infections (15%), and herpes-simplex virus infections (12%). Pneumocystis carinii pneumonia is the most common life-threatening process in these patients. Trimethoprim-sulfamethoxazole (TMP-SMZ) is considered the drug of choice for its treatment. Oral candidiasis often indicates the progression to AIDS in the high-risk populations of homosexual or bisexual men, intravenous drug abusers, and individuals with hemophilia. Nystatin suspension is commonly used to treat oral candidiasis, while Candida esophagitis demands systemic therapy with ketoconazole. Toxoplasmosis most commonly manifests itself in patients with AIDS as a cerebral mass lesion. The recommended therapy includes sulfadiazine and pyrimethamine. AIDS patients frequently experience protozoal invasion of the intestinal tract with Giardia lamblia, Isospora belli, and Cryptosporidium muris. Various drugs have been tried for these infections, including quinacrine hydrochloride, metronidazole, TMP-SMZ, and spiramycin. Cytomegalovirus (CMV) infections commonly involve the lungs, gastrointestinal tract, eyes, brain, and nervous system. Attempts to treat these disseminated CMV infections with antiviral agents, including acyclovir, have not been successful. However, acyclovir has been found beneficial in the treatment of herpes-simplex virus infections. Multiple infectious complications may occur in patients with AIDS as a result of the cellular-immune deficiency associated with this disease. Until more research is done with AIDS patients, therapy must be based on the data available from the treatment of these infections in immunosuppressed patients without AIDS.

  4. Budd-Chiari syndrome complicating hydatid liver disease

    International Nuclear Information System (INIS)

    Robotti, G.C.; Meister, F.; Schroeder, R.; Bern Univ.; Bern Univ.

    1985-01-01

    In two female patients a diagnosis of Budd-Chiari syndrome secondary to hepatic echinococcosis was established by CT. One patient developed acute Budd-Chiari syndrome secondary to E. granulosus lesions of the liver. The second patient presented with a picture of chronic Budd-Chiari syndrome secondary to alveolar echinococcosis. CT findings of Budd-Chiari syndrome included ascites, low density areas in the liver parenchyma, hypertrophy of the caudate lobe, non visualisation of the hepatic veins, occlusion of the retrohepatic inferior vena cava and enlarged retroperitoneal veins. (orig.) [de

  5. Ex vivo inhibited cytokine profiling may explain inferior treatment response to golimumab after adalimumab failure in rheumatoid arthritis

    NARCIS (Netherlands)

    Tweehuysen, L; Schraa, K.; Netea, M.G.; Hoogen, F.H.J. van den; Joosten, L.A.B.; Broeder, A.A. den

    2018-01-01

    OBJECTIVES: Clinical data suggest that the response of rheumatoid arthritis patients to treatment with golimumab is much lower among those who switched from adalimumab than among those who switched from etanercept. To elucidate the mechanism behind this difference in response to sequential biologic

  6. Ectopic ACTH syndrome complicated by multiple opportunistic infections treated with percutaneous ablation of the adrenal glands.

    Science.gov (United States)

    Chan, Chrystal; Roberts, James Mark

    2017-11-14

    Ectopic adrenocorticotropic hormone (ACTH)-related Cushing's syndrome can lead to multiple complications including severe immunosuppression. If the ACTH-secreting tumour cannot be found, definitive treatment is surgical adrenalectomy, typically followed by glucocorticoid replacement. Here, we present a case of fulminant respiratory failure secondary to coinfection with Pneumocystis jirovecii and cytomegalovirus in a patient with ectopic ACTH-dependent Cushing's syndrome with occult primary. Due to significant deconditioning, she was unable to undergo definitive adrenalectomy and instead underwent percutaneous microwave ablation of the adrenal glands. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  7. Severe and acute complications of biologics in psoriasis.

    Science.gov (United States)

    Oussedik, Elias; Patel, Nupur U; Cash, Devin R; Gupta, Angela S; Feldman, Steven R

    2017-12-01

    Biologic therapies have revolutionized the approach to immune-mediated diseases such as psoriasis. Due to their favorable safety profiles and excellent efficacy, biologic agents are considered the gold standard for moderate-to-severe psoriasis. The aim of this paper is to saliently review the severe and acute complications of the Food and Drug Administration (FDA) approved biologic agents for psoriasis. Reviewed agents include tumor necrosis factor alpha inhibitors (etanercept, infliximab, and adalimumab), interleukin 12/23 inhibitors (ustekinumab), and interleukin 17 (IL-17) inhibitors (secukinumab and ixekizumab). While malignancies, serious infections, and major adverse cardiovascular events have been reported, their association with biologic therapy are not hypothesized as causal. However, IL-17 inhibitors appear to cause exacerbations and new cases of inflammatory bowel disease. While more long-term studies are warranted in understanding the biologic's long-term side effect profile, short-term studies have confirmed that the biologics are some of the safest treatment options for psoriasis. Nevertheless, certain populations yield higher risk to acute complications with the biologics than others - physicians must use their judgement and vigilance when monitoring and treating patients undergoing therapy with biological agents.

  8. [Epidural pharmacotherapy in the treatment of the pain syndrome in osteochondrosis complicated by intervertebral discs protrusion of lumbar part of the vertebral column].

    Science.gov (United States)

    Khizhniak, M V; Priĭmak, E V

    2013-03-01

    Experience of application of intralaminar epidural pharmacotherapy for the treatment of the pain syndrome in the patients, suffering osteochondrosis of lumbar portion of vertebral column, complicated by intervertebral discs protrusion, was summarized. The method introduction permits to escape the operative intervention performance for complicated osteochondrosis.

  9. Systemic sarcoidosis with bone marrow involvement responding to therapy with adalimumab: a case report

    OpenAIRE

    Patel, Supen R

    2009-01-01

    Abstract Introduction Sarcoidosis is an inflammatory disorder characterized by the presence of non-caseating granulomas in affected organs. The presence of CD4-positive T lymphocytes and macrophages in affected organs suggests an ongoing immune response. Systemic corticosteroids remain the mainstay of treatment, but therapy is often limited by adverse effects. This is the first report of the use of adalimumab (HUMIRA®, Abbott Laboratories, North Chicago, IL, USA), an anti-tumor necrosis facto...

  10. Tocilizumab for uncontrollable systemic inflammatory response syndrome complicating adult-onset Still disease

    Science.gov (United States)

    Masui-Ito, Asami; Okamoto, Ryuji; Ikejiri, Kaoru; Fujimoto, Mika; Tanimura, Muneyoshi; Nakamori, Shiro; Murata, Tomohiro; Ishikawa, Eiji; Yamada, Norikazu; Imai, Hiroshi; Ito, Masaaki

    2017-01-01

    Abstract Rationale: Adult-onset Still disease (AOSD) is a rare systemic inflammatory disease of unknown etiology characterized by evanescent salmon-pink rash, fever spikes, arthralgia, and lymphadenopathy. AOSD usually has a good prognosis, but it can sometimes be fatal, especially when it is complicated by systemic inflammatory response syndrome (SIRS) and multiple organ failure. Patient concerns: A previously healthy 26-year-old woman was referred to our hospital for persistent high fever and mild systemic edema. Five days later, the patient presented with dyspnea, hypotension, and anuria. Anasarca developed with massive pleural effusion, ascites, and systemic edema, resulting in an increase of 47 kg in body weight. Diagnoses: The patient was diagnosed as AOSD after infection, malignancy, hematologic disorders, and other autoimmune diseases were excluded. Interventions: We administered tocilizumab, an IL-6 receptor inhibitor, intravenously in addition to cyclosporine, prednisolone, plasma exchange, and continuous hemodiafiltration. Outcomes: The patient's systemic condition improved. After stabilization by all medications, the patient was managed and responded to tocilizumab alone. To the best of our knowledge, this was the first case of severe SIRS complicating AOSD that was successfully treated with an anti- IL-6 receptor antibody. Lessons: SIRS should not be overlooked in a patient with steroid-resistant AOSD and edema. Inhibitors of the IL-6 receptor can be used safely and effectively to control AOSD complicated with severe SIRS. PMID:28723802

  11. Antibodies Against Infliximab Are Associated with De Novo Development of Antibodies to Adalimumab and Therapeutic Failure in Infliximab-to-Adalimumab Switchers with IBD

    DEFF Research Database (Denmark)

    Frederiksen, Madeline Therese; Ainsworth, Mark Andrew; Brynskov, Jørn

    2014-01-01

    BACKGROUND: A notable proportion of patients with inflammatory bowel disease (IBD) are switched from infliximab (IFX) to adalimumab (ADL). We investigated if immunogenicity of IFX influenced immunogenicity and clinical outcomes of later ADL therapy. METHODS: Single-center cohort study including all...... patients with IBD assessed for antibodies (Abs) against IFX or ADL. RESULTS: Anti-IFX Abs were evaluated in 187 patients treated with IFX as first line anti-TNF agent. Approximately, half (49%) were positive. Detected anti-IFX Abs had functional capacity as judged by a median IFX concentration below limit...... of detection (interquartile range, 0.0-0.0 μg/mL) versus 3.8 μg/mL (IQR, 1.3-7.9) in anti-IFX Ab-negative patients, P tested positive. Patients with previous anti-IFX Ab development were...

  12. Efficacy and safety of the adalimumab biosimilar Exemptia as induction therapy in moderate-to-severe ulcerative colitis

    Directory of Open Access Journals (Sweden)

    Vandana Midha

    2018-01-01

    Full Text Available Background/Aims: Data on the efficacy and safety of the adalimumab biosimilar Exemptia are limited. Methods: Patients with moderate-to-severe active steroid-refractory ulcerative colitis (UC treated at Dayanand Medical College and Hospital, India were offered cyclosporine A, biologicals or biosimilars, or surgery. A retrospective analysis was conducted on patients who were treated with the adalimumab biosimilar, Exemptia. These patients were administered an induction dosing schedule of 160 mg Exemptia at week 0, 80 mg at week 2, and then 40 mg every other week from week 4 to 8. The clinical response and remission were assessed at week 8 using Mayo score. Results: A total of 29 patients (62.1% male; mean age, 34.9 ± 9.7 years with moderate-to-severe steroid-refractory active UC (mean disease duration, 6.3±5.1 years; pancolitis in 9 patients [31.1%]; left-sided colitis in 20 patients [68.9%] were treated with the Exemptia induction dosing schedule. The mean Mayo score at presentation was 8.2±1.4. At week 8, clinical response was observed in 7 patients (24.1%, whereas clinical remission was observed only in 1 patient (3.5%. Among the non-responders (n=21, 4 patients required colectomy, 1 died, 1 was lost to follow-up, 10 were offered fecal microbiota transplant, 3 were administered infliximab, and 2 patients were administered cyclosporine and tacrolimus, respectively. Four patients (13.8% developed extrapulmonary tuberculosis. Conclusions: The adalimumab biosimilar Exemptia has limited efficacy for the attainment of clinical response and remission in moderate-to-severe steroid-refractory UC, with a significant risk of acquisition or reactivation of tuberculosis in developing countries such as India.

  13. Cavernous sinus thrombosis syndrome and brainstem involvement in patient with leptospirosis: Two rare complications of leptospirosis

    Directory of Open Access Journals (Sweden)

    Shahriyar Alian

    2014-01-01

    Full Text Available Leptospirosis is a bacterial disease that is caused by pathogenic spirochetes of the genus Leptospira. It can affect humans and animals. In humans, it can lead to a wide spectrum of symptoms. It is known as the most common zoonosis in the world. The typical presentation of the disease is an acute biphasic febrile illness with or without jaundice. Less common clinical manifestations may result from involvement of different human body systems. In many places, this disease may be under-diagnosed, especially when associated with neurological complications. Moreover, without treatment, leptospirosis can lead to organ damages, and even death. Neurological complications are uncommon and are reported in a few cases. Cavernous sinus thrombosis syndrome and brainstem involvement are rare complications of leptospirosis and are associated with a high mortality risk. To our knowledge, no such cases have been reported in the literature.

  14. [Approaches to the treatment of patients with climacteric disorders complicated with menopausal metabolic syndrome with cholestasis].

    Science.gov (United States)

    Gavrilova, N P; Seliverstov, P V; Tatarova, N A; Radchenko, V G

    2014-01-01

    Development of the individual comprehensive program of follow treatment of patients with climacteric disorders complicated MMS (menopausal metabolic syndrome) with cholestasis; on the basis of application of low-dose hormone replacement therapy in combination with ursodeoxycholic acid, to improve the quality of life. We observed 101 woman with climacteric syndrome, obesity and cholestasis; conducted a comprehensive clinical and laboratory examination, ultrasound of the hepatobiliary system, measurement modified menopausal index (MMI), the measurement of the quality of life on questionnaire SF-36 before treatment and after 6 and 12 months. Positive and statistically significant changes in lipid spectrum, the activity of transaminases, bilirubin and its fractions, improvement of MMI and quality of life, the indices of coagulation remained virtually unchanged. Low-dose hormone replacement therapies in combination with ursodeoxycholic acid are highly effective drugs for the treatment of menopausal syndrome, which normalize lipid profile of patients and the performance of the hepatobiliary system.

  15. Mucosal complications of modified osteo-odonto keratoprosthesis in chronic Stevens-Johnson syndrome.

    Science.gov (United States)

    Basu, Sayan; Pillai, Vinay Sukumara; Sangwan, Virender S

    2013-11-01

    To describe clinical outcomes of complications afflicting the autologous oral mucous membrane graft after modified osteo-odonto keratoprosthesis surgery in chronic Stevens-Johnson syndrome (SJS). Prospective case series. This study included 30 eyes of 30 patients with SJS-induced dry keratinized ocular surfaces; the patients underwent various stages of this procedure between August 2009 and February 2012. Mucosal complications were classified as either necrosis or overgrowth. Mucosal necrosis was managed according to a predesigned algorithm based on timing (pre- and postimplantation) and location (central or peripheral) of necrosis. Cases with mucosal overgrowth underwent mucosal debulking and trimming. Mucosal necrosis developed in 15 (50%) eyes and overgrowth in 4 (13.3%) eyes. Preimplantation necrosis (n = 7) was initially managed conservatively, but 2 eyes required free labial-mucous membrane grafting for persistent corneal exposure. Free labial-mucous membrane grafting was performed in all cases of postimplantation necrosis (n = 10), but 8 eyes required additional tarsal pedicle flaps (n = 6, for peripheral necrosis) or through-the-lid revisions (n = 2, for central necrosis). Debulking and trimming effectively managed all cases of mucosal overgrowth, but 3 eyes required repeat procedures. At 24.1 ± 6.5 months postimplantation, the keratoprosthesis was retained in all eyes, and the probability of maintaining 20/60 or better vision was similar in eyes with or without mucosal necrosis (86 ± 8.8% vs 80 ± 10.3%). Mucosal complications, especially necrosis, occurred commonly following modified osteo-odonto keratoprosthesis surgery in dry keratinized post-SJS eyes. The algorithm-based management approach described in this study was successful in treating these complications, retaining the prosthesis and preserving useful vision. Copyright © 2013 Elsevier Inc. All rights reserved.

  16. Rituximab for childhood-onset, complicated, frequently relapsing nephrotic syndrome or steroid-dependent nephrotic syndrome: a multicentre, double-blind, randomised, placebo-controlled trial.

    Science.gov (United States)

    Iijima, Kazumoto; Sako, Mayumi; Nozu, Kandai; Mori, Rintaro; Tuchida, Nao; Kamei, Koichi; Miura, Kenichiro; Aya, Kunihiko; Nakanishi, Koichi; Ohtomo, Yoshiyuki; Takahashi, Shori; Tanaka, Ryojiro; Kaito, Hiroshi; Nakamura, Hidefumi; Ishikura, Kenji; Ito, Shuichi; Ohashi, Yasuo

    2014-10-04

    Rituximab could be an effective treatment for childhood-onset, complicated, frequently relapsing nephrotic syndrome (FRNS) and steroid-dependent nephrotic syndrome (SDNS). We investigated the efficacy and safety of rituximab in patients with high disease activity. We did a multicentre, double-blind, randomised, placebo-controlled trial at nine centres in Japan. We screened patients aged 2 years or older experiencing a relapse of FRNS or SDNS, which had originally been diagnosed as nephrotic syndrome when aged 1-18 years. Patients with complicated FRNS or SDNS who met all other criteria were eligible for inclusion after remission of the relapse at screening. We used a computer-generated sequence to randomly assign patients (1:1) to receive rituximab (375 mg/m(2)) or placebo once weekly for 4 weeks, with age, institution, treatment history, and the intervals between the previous three relapses as adjustment factors. Patients, guardians, caregivers, physicians, and individuals assessing outcomes were masked to assignments. All patients received standard steroid treatment for the relapse at screening and stopped taking immunosuppressive agents by 169 days after randomisation. Patients were followed up for 1 year. The primary endpoint was the relapse-free period. Safety endpoints were frequency and severity of adverse events. Patients who received their assigned intervention were included in analyses. This trial is registered with the University Hospital Medical Information Network clinical trials registry, number UMIN000001405. Patients were centrally registered between Nov 13, 2008, and May 19, 2010. Of 52 patients who underwent randomisation, 48 received the assigned intervention (24 were given rituximab and 24 placebo). The median relapse-free period was significantly longer in the rituximab group (267 days, 95% CI 223-374) than in the placebo group (101 days, 70-155; hazard ratio: 0·27, 0·14-0·53; p<0·0001). Ten patients (42%) in the rituximab group and six (25

  17. Abdominal complications in black and Indian children with nephrotic ...

    African Journals Online (AJOL)

    Abdominal complications were detected and investigated in 19 (10%) of 191 children with nephrotic syndrome who experienced 35 episodes of these complications. Fourteen children were Indian with steroid-responsive nephrotic syndrome, and 5 were black, of whom 4 had membranous nephropathy and 1 focal ...

  18. [Clinical analysis of pregnancy and postpartum cardiovascular complications in Marfan syndrome].

    Science.gov (United States)

    Xie, Jin-sheng; Li, Bin; Sun, Yan-qing; Guan, Xin-liang; Xu, Bao-wei; Qiao, Zhi-yu

    2007-11-01

    To investigate the management of pregnancy and cardiovascular complications in women with Marfan syndrome (MFS). From October 1994 to September 2006, 30 patients with MFS undergoing cardiovascular surgery were studied retrospectively. In the labor of 46 offsprings given birth by 30 women, 5 cases (11%) were performed elective cesarean section because of the existence of aortic complication, and 12 (26%) were diagnosed as MFS. The gestation in two patients was terminated due to deterioration of aortic abnormalities during their third trimester, and they received surgical treatment with Bentall procedure. Two developed acute aortic dissection during labor and post delivery respectively. With the manipulation of anticoagulation peripartum, one who had the implantation with mechanical prosthesis went through pregnancy and delivery uneventfully. The average duration between delivery and cardiovascular surgery was (15 +/- 9) years. Vaginal delivery can be done safely in patients with the MFS who do not have or have mild cardiovascular system abnormalities, aortic dissection, or other important cardiac abnormalities, cesarean section should be the preferred method of delivery. Women with MFS are at increased risk for dissection and congestive heart failure during pregnancy and should be counseled before pregnancy about these risks, as well as the inheritance of the condition.

  19. PRES (posterior reversible encephalopathy syndrome), a rare complication of tacrolimus therapy.

    LENUS (Irish Health Repository)

    Hodnett, P

    2009-11-01

    With increasing numbers of solid organ and hematopoietic stem cell transplantations being performed, there have been significant increases in the use of immunosuppressive agents such as cyclosporine and tacrolimus. Posterior reversible encephalopathy syndrome (PRES) is a serious complication of immunosuppressive therapy use following solid organ or stem cell transplants. Clinical findings including headache, mental status changes, focal neurological deficits, and\\/or visual disturbances. Associated with these are characteristic imaging features of subcortical white matter lesions on computed tomography (CT) or magnetic resonance imaging (MRI). The changes in the subcortical white matter are secondary to potentially reversible vasogenic edema, although conversion to irreversible cytotoxic edema has been described. These imaging findings predominate in the territory of the posterior cerebral artery. Many studies have shown that the neurotoxicity associated with tacrolimus may occur at therapeutic levels. In most cases of PRES, the symptom complex is reversible by reducing the dosage or withholding the drug for a few days. While PRES is an uncommon complication, it is associated with significant morbidity and mortality if it is not expeditiously recognized. MRI represents the most sensitive imaging technique for recognizing PRES. This report highlights the value of MRI in prompt recognition of this entity, which offers the best chance of avoiding long-term sequelae.

  20. Pre- and perinatal complications in relation to Tourette syndrome and co-occurring obsessive-compulsive disorder and attention-deficit/hyperactivity disorder

    NARCIS (Netherlands)

    Abdulkadir, M. (Mohamed); J.A. Tischfield (Jay); King, R.A. (Robert A.); T.V. Fernandez (Thomas); Brown, L.W. (Lawrence W.); Cheon, K.-A. (Keun-Ah); Coffey, B.J. (Barbara J.); S.F. de Bruijn (S.); Elzerman, L. (Lonneke); Garcia-Delgar, B. (Blanca); D.L. Gilbert (D.); S.F.J. Le Grice; Hagstrøm, J. (Julie); T. Hedderly (Tammy); Heyman, I. (Isobel); Hong, H.J. (Hyun Ju); J. Huyser (Jochanan); Ibanez-Gomez, L. (Laura); Kim, Y.K. (Young Key); Kim, Y.-S. (Young-Shin); Koh, Y.-J. (Yun-Joo); Kook, S. (Sodahm); Kuperman, S. (Samuel); Lamerz, A. (Andreas); Leventhal, B. (Bennett); Ludolph, A.G. (Andrea G.); Madruga-Garrido, M. (Marcos); A. Maras (Athanasios); Messchendorp, M.D. (Marieke D.); Mir, P. (Pablo); Morer, A. (Astrid); A. Münchau (Alexander); Murphy, T.L. (Tara L.); Openneer, T.J.C. (Thaïra J.C.); Plessen, K.J. (Kerstin J.); S. Rath (Santosh); V. Rœssner (Veit); Fründt, O. (Odette); Shin, E.-Y. (Eun-Young); D.A. Sival (Deborah); Song, D.-H. (Dong-Ho); Song, J. (Jungeun); Stolte, A.-M. (Anne-Marie); Tübing, J. (Jennifer); van den Ban, E. (Els); F. Visscher (Frank); Wanderer, S. (Sina); Woods, M. (Martin); Zinner, S.H. (Samuel H.); M.W. State (Matthew); M.L. Heiman (Mark); P.J. Hoekstra (Pieter); A. Dietrich (Andrea)

    2016-01-01

    textabstractPre- and perinatal complications have been implicated in the onset and clinical expression of Tourette syndrome albeit with considerable inconsistencies across studies. Also, little is known about their role in co-occurring obsessive-compulsive disorder (OCD) and

  1. Efficacy and safety of tofacitinib monotherapy, tofacitinib with methotrexate, and adalimumab with methotrexate in patients with rheumatoid arthritis (ORAL Strategy): a phase 3b/4, double-blind, head-to-head, randomised controlled trial.

    Science.gov (United States)

    Fleischmann, Roy; Mysler, Eduardo; Hall, Stephen; Kivitz, Alan J; Moots, Robert J; Luo, Zhen; DeMasi, Ryan; Soma, Koshika; Zhang, Richard; Takiya, Liza; Tatulych, Svitlana; Mojcik, Christopher; Krishnaswami, Sriram; Menon, Sujatha; Smolen, Josef S

    2017-07-29

    Tofacitinib is an oral Janus kinase inhibitor for the treatment of rheumatoid arthritis. The Oral Rheumatoid Arthritis triaL (ORAL) Strategy aimed to assess the comparative efficacy of tofacitinib monotherapy, tofacitinib plus methotrexate, and adalimumab plus methotrexate for the treatment of rheumatoid arthritis in patients with a previous inadequate response to methotrexate. ORAL Strategy was a 1 year, double-blind, phase 3b/4, head-to-head, non-inferiority, randomised controlled trial in patients aged 18 years or older with active rheumatoid arthritis despite methotrexate therapy. Patients were randomly assigned (1:1:1) to receive oral tofacitinib (5 mg twice daily) monotherapy, oral tofacitinib (5 mg twice daily) plus methotrexate, or subcutaneous adalimumab (40 mg every other week) plus methotrexate at 194 centres in 25 countries. Eligible patients received live zoster vaccine at investigators' discretion. The primary endpoint was the proportion of patients who attained an American College of Rheumatology response of at least 50% (ACR50) at month 6 in the full analysis set (patients who were randomly assigned to a group and received at least one dose of the study treatment). Non-inferiority between groups was shown if the lower bound of the 98·34% CI of the difference between comparators was larger than -13·0%. This trial is registered with ClinicalTrials.gov, number NCT02187055. 1146 patients received treatment (384 had tofacitinib monotherapy; 376 had tofacitinib and methotrexate; and 386 had adalimumab and methotrexate). At 6 months, ACR50 response was attained in 147 (38%) of 384 patients with tofacitinib monotherapy, 173 (46%) of 376 patients with tofacitinib and methotrexate, and 169 (44%) of 386 patients with adalimumab and methotrexate. Non-inferiority was declared for tofacitinib and methotrexate versus adalimumab and methotrexate (difference 2% [98·34% CI -6 to 11]) but not for tofacitinib monotherapy versus either adalimumab and methotrexate (-6

  2. Rapid alleviation of signs and symptoms of rheumatoid arthritis with intravenous or subcutaneous administration of adalimumab in combination with methotrexate.

    NARCIS (Netherlands)

    Rau, R.; Simianer, S.; Riel, P.L.C.M. van; Putte, L.B.A. van de; Kruger, K.; Schattenkirchner, M.; Allaart, C.F.; Breedveld, F.C.; Kempeni, J.; Beck, K.; Kupper, H.

    2004-01-01

    OBJECTIVE: This randomized, placebo-controlled, double-blind, Phase 1 study assessed the magnitude, onset, and duration of response with intravenous (i.v.) and subcutaneous (s.c.) adalimumab (Humira, Abbott Laboratories) combined with methotrexate (MTX) in patients with active rheumatoid arthritis

  3. Complicações Imediatas de 3.555 aplicações de agentes anti-TNFα Immediate complications of 3,555 injections of anti-TNFα

    Directory of Open Access Journals (Sweden)

    Júlio César Bertacini de Moraes

    2010-04-01

    Full Text Available OBJETIVO: Avaliar as complicações imediatas da aplicação de agentes anti-TNFα no Centro de Dispensação de Medicação deAlto Custo do HC-FMUSP. PACIENTES E MÉTODOS: Foram incluídos todos os pacientes que receberam agentes anti-TNFα entre agosto/2007 e março/2009.As complicações imediatas (até 1 hora após o término da aplicação foram classificadas em leves (cefaleia, rash, tontura, prurido, náuseas, moderadas (febre, urticária, palpitação, dor torácica, dispneia, variação da pressão arterial de 20 a 40 mmHg ou graves (febre com calafrios, dispneia com sibilância, variação da pressão arterial > 40 mmHg. RESULTADOS: Foram avaliados 242 pacientes: 94 (39% com artrite reumatoide, 64 (26% com espondilite anquilosante, 32 (13% com artrite psoriásica, 26 (11% com artrite idiopática juvenil e 27 (11% com outros diagnósticos. O número total de aplicações foi de 3.555, sendo 992 (28% de adalimumabe, 1.546 (43% de etanercepte e 1.017 (29% de infliximabe. Complicações imediatas foram observadas em 39/242 (16% pacientes. As complicações ocorreram em 45/3.555 (1,2% aplicações. Estas foram mais frequentes com infliximabe comparado com adalimumabe (3,7% vs. 0,5%, P OBJECTIVE: To evaluate the immediate complications of anti-TNFα drugs at the "Center for Dispensation of High Cost Medications" of HC-FMUSP. PATIENTS AND METHODS: All patients who received anti-TNFα agents between August 2007 and March 2009 were included in this study. Immediate complications (up to 1 hour after the injection were classified as mild (headache, rash, dizziness, itching, nausea, moderate (fever, urticaria, palpitation, chest pain, dyspnea, blood pressure variations between 20 and 40 mmHg, or severe (fever with chills, dyspnea with wheezing, variations in blood pressure > 40 mmHg. RESULTS: Two hundred and forty-two patients were evaluated: 94 (39% with rheumatoid arthritis, 64 (26% with ankylosing spondylitis, 32 (13% with psoriatic arthritis

  4. Two different nervous system complications of mycoplasma pneumoniae

    OpenAIRE

    Faruk İncecik; M. Özlem Hergüner; Şakir Altunbaşak

    2010-01-01

    Mycoplasma pneumoniae is an important agent of the lower and upper respiratory system infections. It may resultin some complications and clinical conditions other than infections. Meningoencephalitis, encephalitis, transversemyelitis, acute disseminated encephalomyelitis, cerebral infarction and Gullian-Barre syndrome are major neurological complications.We present here two cases with meningoencephalitis and Gullian-Barre syndrome secondary to Mycoplasma pneumoniaeinfection.

  5. Two different nervous system complications of mycoplasma pneumoniae

    Directory of Open Access Journals (Sweden)

    Faruk İncecik

    2010-06-01

    Full Text Available Mycoplasma pneumoniae is an important agent of the lower and upper respiratory system infections. It may resultin some complications and clinical conditions other than infections. Meningoencephalitis, encephalitis, transversemyelitis, acute disseminated encephalomyelitis, cerebral infarction and Gullian-Barre syndrome are major neurological complications.We present here two cases with meningoencephalitis and Gullian-Barre syndrome secondary to Mycoplasma pneumoniaeinfection.

  6. Disease activity, physical function, and radiographic progression after longterm therapy with adalimumab plus methotrexate: 5-year results of PREMIER

    NARCIS (Netherlands)

    van der Heijde, Désirée; Breedveld, Ferdinand C.; Kavanaugh, Arthur; Keystone, Edward C.; Landewé, Robert; Patra, Kaushik; Pangan, Aileen L.

    2010-01-01

    To evaluate the efficacy and safety of initial combination treatment with adalimumab (ADA) and methotrexate (MTX) versus monotherapy with ADA or MTX during an open-label extension of PREMIER. Patients with early rheumatoid arthritis (RA) received blinded ADA plus MTX, ADA alone, or MTX alone for 2

  7. Pre- and perinatal complications in relation to Tourette syndrome and co-occurring obsessive-compulsive disorder and attention-deficit/hyperactivity disorder

    NARCIS (Netherlands)

    Abdulkadir, Mohamed; Tischfield, Jay A.; King, Robert A.; Fernandez, Thomas V.; Brown, Lawrence W.; Cheon, Keun-Ah; Coffey, Barbara J.; de Bruijn, Sebastian F. T. M.; Elzerman, Lonneke; Garcia-Delgar, Blanca; Gilbert, Donald L.; Grice, Dorothy E.; Hagstrom, Julie; Hedderly, Tammy; Heyman, Isobel; Hong, Hyun Ju; Huyser, Chaim; Ibanez-Gomez, Laura; Kim, Young Key; Kim, Young-Shin; Koh, Yun-Joo; Kook, Sodahm; Kuperman, Samuel; Lamerz, Andreas; Leventhal, Bennett; Ludolph, Andrea G.; Madruga-Garrido, Marcos; Maras, Athanasios; Messchendorp, Marieke D.; Mir, Pablo; Morer, Astrid; Munchau, Alexander; Murphy, Tara L.; Openneer, Thaira J. C.; Plessen, Kerstin J.; Rath, Judith J. G.; Roessner, Veit; Frundt, Odette; Shin, Eun-Young; Sival, Deborah A.; Song, Dong-Ho; Song, Jungeun; Stolte, Anne-Marie; Tubing, Jennifer; van den Ban, Els; Visscher, Frank; Wanderer, Sina; Woods, Martin; Zinner, Samuel H.; State, Matthew W.; Heiman, Gary A.; Hoekstra, Pieter J.; Dietrich, Andrea

    2016-01-01

    Pre- and perinatal complications have been implicated in the onset and clinical expression of Tourette syndrome albeit with considerable inconsistencies across studies. Also, little is known about their role in co-occurring obsessive-compulsive disorder (OCD) and attention-deficit/hyperactivity

  8. Prenatal management and perinatal outcome in giant placental chorioangioma complicated with hydrops fetalis, fetal anemia and maternal mirror syndrome

    Directory of Open Access Journals (Sweden)

    García-Díaz Lutgardo

    2012-07-01

    Full Text Available Abstract Background Giant placental chorioangiomas have been associated with a number of severe fetal complications and high perinatal mortality. Case presentation We report a case of giant chorioangioma with fetal hydrops, additionally complicated by severe anemia, mild cardiomegaly with hyperdinamic heart circulation and maternal mirror syndrome. Intrauterine blood transfusion and amniodrainage was performed at 29 weeks. Worsening of the fetal and maternal condition prompted us to proceed with delivery at 29 + 5 weeks. The newborn died 3 hours later due to pulmonary hypoplasia and hemodynamic failure. Maternal course was favourable, mirror syndrome resolved in the second day and the patient was discharged four days following delivery. Conclusions In the case described here, fetal condition got worse despite of the anemia correction and amniodrainage. Our outcome raises the issue whether additional intrauterine clinical intervention, as intersticial laser, should have been performed to stop further deterioration of the fetal condition when progressive severe hydrops develops.

  9. An indirect comparison and cost per responder analysis of adalimumab, methotrexate and apremilast in the treatment of methotrexate-naïve patients with psoriatic arthritis.

    Science.gov (United States)

    Betts, Keith A; Griffith, Jenny; Friedman, Alan; Zhou, Zheng-Yi; Signorovitch, James E; Ganguli, Arijit

    2016-01-01

    Apremilast was recently approved for the treatment of active psoriatic arthritis (PsA). However, no studies compare apremilast with methotrexate or biologic therapies, so its relative comparative efficacy remains unknown. This study compared the response rates and incremental costs per responder associated with methotrexate, apremilast, and biologics for the treatment of active PsA. A systematic literature review was performed to identify phase 3 randomized controlled clinical trials of approved biologics, methotrexate, and apremilast in the methotrexate-naïve PsA population. Using Bayesian methods, a network meta-analysis was conducted to indirectly compare rates of achieving a ≥20% improvement in American College of Rheumatology component scores (ACR20). The number needed to treat (NNT) and the incremental costs per ACR20 responder (2014 US$) relative to placebo were estimated for each of the therapies. Three trials (MIPA for methotrexate, PALACE-4 for apremilast, and ADEPT for adalimumab) met all inclusion criteria. The NNTs relative to placebo were 2.63 for adalimumab, 6.69 for apremilast, and 8.31 for methotrexate. Among methotrexate-naïve PsA patients, the 16 week incremental costs per ACR20 responder were $3622 for methotrexate, $26,316 for adalimumab, and $45,808 for apremilast. The incremental costs per ACR20 responder were $222,488 for apremilast vs. methotrexate. Among methotrexate-naive PsA patients, adalimumab was found to have the lowest NNT for one additional ACR20 response and methotrexate was found to have the lowest incremental costs per ACR20 responder. There was no statistical evidence of greater efficacy for apremilast vs. methotrexate. A head-to-head trial between apremilast and methotrexate is recommended to confirm this finding.

  10. Late-onset cytomegalovirus infection complicated by Guillain-Barre syndrome in a kidney transplant recipient: case report and review of the literature.

    Science.gov (United States)

    Shaban, E; Gohh, R; Knoll, B M

    2016-04-01

    Cytomegalovirus (CMV) infection remains a common infection after solid-organ transplantation. In the general population CMV disease is associated with Guillain-Barre syndrome (GBS), an autoimmune disease leading to an acute peripheral neuropathy, in 1 of 1000 cases. Interestingly, GBS is a rarely observed complication in solid-organ transplant recipients, possibly related to maintenance immunosuppression. We describe a case of CMV infection complicated by GBS in a kidney transplant recipient and review the literature.

  11. Lung-Protective Ventilation With Low Tidal Volumes and the Occurrence of Pulmonary Complications in Patients Without Acute Respiratory Distress Syndrome: A Systematic Review and Individual Patient Data Analysis.

    Science.gov (United States)

    Neto, Ary Serpa; Simonis, Fabienne D; Barbas, Carmen S V; Biehl, Michelle; Determann, Rogier M; Elmer, Jonathan; Friedman, Gilberto; Gajic, Ognjen; Goldstein, Joshua N; Linko, Rita; Pinheiro de Oliveira, Roselaine; Sundar, Sugantha; Talmor, Daniel; Wolthuis, Esther K; Gama de Abreu, Marcelo; Pelosi, Paolo; Schultz, Marcus J

    2015-10-01

    Protective mechanical ventilation with low tidal volumes is standard of care for patients with acute respiratory distress syndrome. The aim of this individual patient data analysis was to determine the association between tidal volume and the occurrence of pulmonary complications in ICU patients without acute respiratory distress syndrome and the association between occurrence of pulmonary complications and outcome in these patients. Individual patient data analysis. ICU patients not fulfilling the consensus criteria for acute respiratory distress syndrome at the onset of ventilation. Mechanical ventilation with low tidal volume. The primary endpoint was development of a composite of acute respiratory distress syndrome and pneumonia during hospital stay. Based on the tertiles of tidal volume size in the first 2 days of ventilation, patients were assigned to a "low tidal volume group" (tidal volumes ≤ 7 mL/kg predicted body weight), an "intermediate tidal volume group" (> 7 and volume group" (≥ 10 mL/kg predicted body weight). Seven investigations (2,184 patients) were included. Acute respiratory distress syndrome or pneumonia occurred in 23% of patients in the low tidal volume group, in 28% of patients in the intermediate tidal volume group, and in 31% of the patients in the high tidal volume group (adjusted odds ratio [low vs high tidal volume group], 0.72; 95% CI, 0.52-0.98; p = 0.042). Occurrence of pulmonary complications was associated with a lower number of ICU-free and hospital-free days and alive at day 28 (10.0 ± 10.9 vs 13.8 ± 11.6 d; p volumes is associated with a lower risk of development of pulmonary complications in patients without acute respiratory distress syndrome.

  12. Intravitreal Adalimumab in Active Noninfectious Uveitis: A Pilot Study.

    Science.gov (United States)

    Hamam, Rola N; Barikian, Anita W; Antonios, Rafic S; Abdulaal, Marwan R; Alameddine, Ramzi M; El Mollayess, Georges; Mansour, Ahmad M

    2016-06-01

    To evaluate the short-term efficacy of intravitreal adalimumab (IVA) for the treatment of eyes with active noninfectious uveitis. Consecutive eyes with active noninfectious uveitis were injected with IVA at 0, 2, then every 4 weeks for total of 26 weeks. Six out of 7 patients (12 of 13 eyes) completed 26 weeks of treatment. One patient (1 eye) failed treatment. Seven out of 12 eyes had improvement of ≥2 ETDRS lines. Three out of three eyes had resolution of anterior chamber cells. And 9 of 10 eyes with vitreous haze had zero haze at 26 weeks. Five out of 8 eyes with macular edema had complete resolution. Median fluorescein angiography score improved from 14 to 4 on last follow-up. IVA was effective in controlling the inflammation, decreasing the macular edema, and improving the best corrected visual acuity in the majority of eyes in this series.

  13. Otitis complicated by Jacod's syndrome with unusal facial nerve involvement: Case report and review of literature.

    Science.gov (United States)

    Abdulkadir, Kocer; Buket, Sanlisoy; Dilek, Agircan; Munevver, Okay; Ayse, Aralasmak

    2015-04-01

    Otitis media is a well-known condition and its infra-temporal and intracranial complications are extremely rare because of the widespread usage of antibiotic treatment. We report a case of 63-year-old female with complaints of right-sided facial pain and diplopia. She had a history of acute otitis media before 4 months of admission to our neurology unit. Neurological examination showed that total ophthalmoplegia with ptosis, mydriasis, decreased vision and loss of pupil reflex on the right side. In addition, there was involvement of 5th and 7th cranial nerves. Neurological and radiological follow-up examinations demonstrated Jacod's Syndrome with unusual facial nerve damage and infection in aetiology. Sinusitis is the most common aetiology, but there are a few cases reported Jacod's Syndrome originating from otitis media.

  14. Adalimumab dose escalation and dose de-escalation success rate and predictors in a large national cohort of Crohn's patients

    NARCIS (Netherlands)

    Baert, Filip; Glorieus, Elien; Reenaers, Cathérine; D'Haens, Geert; Peeters, Harald; Franchimont, Dennis; Dewit, Olivier; Caenepeel, Philippe; Louis, Edouard; van Assche, Gert; D'Heygere, F.; George, C.; van Hootegem, P.; Ilegems, S.; Fontaine, F.; Colard, A.; Schoofs, N.; Belaiche, J.; Louis, E.; Reenaers, C.; van Kemseke, C.; Coche, J. C.; Dewit, O.; Rahier, J. F.; de Reuck, M.; Baert, F.; Decaestecker, J.; de Wulf, D.; Amininejad, L.; Franchimont, D.; van Gossum, A.; Du Ville, L.; Hendrickx, K.; Lepoutre, L.; Vandervoort, J.; van der Spek, P.; Sprengers, D.; van de Mierop, F.; Potvin, P.; Bontems, P.; Moreels, T.; van Outryve, M.; Mana, F.; de Looze, D.; de Vos, M.; Peeters, H.; Ferrante, M.; Rutgeerts, P.; van Assche, G.; Vermeire, S.

    2013-01-01

    Adalimumab is efficacious in inducing and maintaining remission in Crohn's disease but dose escalation is needed in 30-40% after 1 year. Attempts for dose de-escalation have not been studied. This study aimed to assess the need for, predictors, and outcome of dose escalation and de-escalation in a

  15. Is there a difference between the STOP-BANG and the Berlin Obstructive Sleep Apnoea Syndrome questionnaires for determining respiratory complications during the perioperative period?

    Science.gov (United States)

    Gokay, Pervin; Tastan, Sevinc; Orhan, Mehmet Emin

    2016-05-01

    This study aimed to compare the efficiency of the STOP-BANG and Berlin Obstructive Sleep Apnoea Syndrome questionnaires for evaluating potential respiratory complications during the perioperative period. Questionnaires that are used to determine obstructive sleep apnoea risk are not widely used for surgical patients. Among the questionnaires that are commonly used for obstructive sleep apnoea screening, it remains unclear whether the STOP-BANG or Berlin Obstructive Sleep Apnoea Syndrome questionnaire is more effective in terms of ease of use, usage period and diagnosis of surgical patients with obstructive sleep apnoea risk. This study was designed as a descriptive and prospective study. The study included 126 patients over 18 years of age who were American Society of Anesthesiologists classification class I-II and underwent laparoscopic cholecystectomy. To determine the potential obstructive sleep apnoea syndrome risk, the STOP-BANG and Berlin questionnaires were administered. Respiratory complications were then observed during the perioperative period. During intubation and extubation, we observed statistically significant differences in difficult intubation, difficult facemask ventilation and desaturation frequency between the high- and low-risk groups for obstructive sleep apnoea syndrome, as determined by the STOP-BANG questionnaire. During extubation, statistically significant differences in coughing, breath-holding and desaturation frequency were observed between the high-risk and low-risk groups, according to the Berlin questionnaire. In the post-anaesthesia care unit, both questionnaires found statistically significant differences between the low- and high-risk groups. Obstructive sleep apnoea syndrome screening questionnaires administered during the preoperative period are useful for predicting perioperative respiratory complications. It may be most useful to administer the STOP-BANG questionnaire as the initial evaluation. Questionnaires may be used to

  16. Increased serum levels of hyaluronic acid in pregnancies complicated by preeclampsia or hemolysis, elevated liver enzymes, and low platelets syndrome.

    Science.gov (United States)

    Osmers, R G; Schütz, E; Diedrich, F; Wehry, B; Krauss, T; Oellerich, M; Kuhn, W

    1998-02-01

    Fifteen percent of patients who later have hemolysis, elevated liver enzymes, and low platelets syndrome develop initially have nonspecific symptoms. Early diagnosis could ensure adequate obstetric management; however, prognostic biochemical tests are lacking. We hypothesized that elevated hyaluronic acid serum levels might be an early indicator of hemolysis, elevated liver enzymes, and low platelets syndrome because it is known to be a sensitive marker of liver cell function. Hyaluronic acid in serum was measured in patients with normal pregnancies (n = 109) and in those patients with pregnancies complicated by preeclampsia (n = 14) or hemolysis, elevated liver enzymes, and low platelets syndrome (n = 11). A significant increase in hyaluronic acid serum concentrations was observed in patients with hemolysis, elevated liver enzymes, and low platelets syndrome or with preeclampsia (p hyaluronic acid serum levels in hemolysis, elevated liver enzymes, and low platelets syndrome correlated with the clinical severity of the individual course of disease as measured by intensive care unit time (r = 0.72; p hyaluronic acid in preeclampsia and hemolysis, elevated liver enzymes, and low platelets syndrome are significantly elevated and might play an important diagnostic and prognostic role in patients with hemolysis, elevated liver enzymes, and low platelets syndrome.

  17. Noonan Syndrome Complicated by Primary Pulmonary Lymphangiectasia.

    Science.gov (United States)

    Ford, Jaclyn Jo; Trotter, Carol W

    2015-01-01

    Noonan syndrome is a genetic disorder that has several features common to other conditions, making diagnosis a challenge. This column summarizes the case of a neonate with an atypical presentation of Noonan syndrome involving a fatal type of lymphangiectasia resulting in persistent pleural effusions. Radiographic features of this condition are presented along with the complexities of diagnosis and treatment.

  18. Obstetric complications in women with IVF conceived pregnancies and polycystic ovarian syndrome

    Directory of Open Access Journals (Sweden)

    Sunita R Tandulwadkar

    2014-01-01

    Full Text Available Polycystic ovarian syndrome (PCOS is often accompanied by infertility that necessitates ovulation induction using clomiphene citrate, gonadotropins or even in vitro fertilization (IVF. These treatment methods are known to increase the incidence of multiple pregnancies as well as some negative consequences, including a rise in the risk for gestational diabetes mellitus, pre-eclampsia, etc., Furthermore, pregnancies established after IVF carry an increased risk for maternal complications. However, the increased risk of developing adverse obstetric complications has been suggested to occur independently of obesity as well as in populations without assisted reproductive techniques. Many studies have been performed to study the effect of PCOS on pregnancy and the effect of pregnancy on PCOS. The hormonal milieu that is exaggerated in PCOS women is quite well understood at the biochemical and genetic levels. The maternal and neonatal outcomes of PCOS women who have undergone in vitro fertilization-embryo transfer (IVF-ET have not been widely studied till date. This review aims to evaluate the current evidence regarding adverse obstetric outcomes of PCOS women undergoing IVF-ET. The rationale of this review is to study whether the adverse obstetric outcomes are increased in PCOS women in general, or particularly in those PCOS women who are undergoing IVF-ET. It is also important to analyze via a literature review whether the increased adverse outcomes are due to infertility in general or PCOS per se. An attempt has been made to give evidence regarding preventive strategies for obstetric complications in PCOS women who have undergone IVF-ET.

  19. Characterization of vascular complications in experimental model of fructose-induced metabolic syndrome.

    Science.gov (United States)

    El-Bassossy, Hany M; Dsokey, Nora; Fahmy, Ahmed

    2014-12-01

    Vascular dysfunction is an important complication associated with metabolic syndrome (MS). Here we fully characterized vascular complications in a rat model of fructose-induced MS. MS was induced by adding fructose (10%) to drinking water to male Wistar rats of 6 weeks age. Blood pressure (BP) and isolated aorta responses phenylephrine (PE), KCl, acetylcholine (ACh), and sodium nitroprusside (SNP) were recorded after 6, 9, and 12 weeks of fructose administration. In addition, serum levels of glucose, insulin, uric acid, tumor necrosis factor α (TNFα), lipids, advanced glycation end products (AGEs), and arginase activity were determined. Furthermore, aortic reactive oxygen species (ROS) generation, hemeoxygenase-1 expression, and collagen deposition were examined. Fructose administration resulted in a significant hyperinslinemia after 6 weeks which continued for 12 weeks. It was also associated with a significant increase in BP after 6 weeks which was stable for 12 weeks. Aorta isolated from MS animals showed exaggerated contractility to PE and KCl and impaired relaxation to ACh compared with control after 6 weeks which were clearer at 12 weeks of fructose administration. In addition, MS animals showed significant increases in serum levels of lipids, uric acid, AGEs, TNFα, and arginase enzyme activity after 12 weeks of fructose administration. Furthermore, aortae isolated from MS animals were characterized by increased ROS generation and collagen deposition. In conclusion, adding fructose (10%) to drinking water produces a model of MS with vascular complications after 12 weeks that are characterized by insulin resistance, hypertension, disturbed vascular reactivity and structure, hyperuricemia, dyslipidemia, and low-grade inflammation.

  20. Cost per remission and cost per response with infliximab, adalimumab, and golimumab for the treatment of moderately-to-severely active ulcerative colitis.

    Science.gov (United States)

    Toor, Kabirraaj; Druyts, Eric; Jansen, Jeroen P; Thorlund, Kristian

    2015-06-01

    To determine the short-term costs per sustained remission and sustained response of three tumor necrosis factor inhibitors (infliximab, adalimumab, and golimumab) in comparison to conventional therapy for the treatment of moderately-to-severely active ulcerative colitis. A probabilistic Markov model was developed. This included an 8-week induction period, and 22 subsequent 2-week cycles (up to 1 year). The model included three disease states: remission, response, and relapse. Costs were from a Canadian public payer perspective. Estimates for the additional cost per 1 year of sustained remission and sustained response were obtained. Golimumab 100 mg provided the lowest cost per additional remission ($935) and cost per additional response ($701) compared with conventional therapy. Golimumab 50 mg yielded slightly higher costs than golimumab 100 mg. Infliximab was associated with the largest additional number of estimated remissions and responses, but also higher cost at $1975 per remission and $1311 per response. Adalimumab was associated with the largest cost per remission ($7430) and cost per response ($2361). The cost per additional remission and cost per additional response associated with infliximab vs golimumab 100 mg was $14,659 and $4753, respectively. The results suggest that the additional cost of 1 full year of remission and response are lowest with golimumab 100 mg, followed by golimumab 50 mg. Although infliximab has the highest efficacy, it did not exhibit the lowest cost per additional remission or response. Adalimumab produced the highest cost per additional remission and response.

  1. Severe neurological complication following adjustable gastric banding.

    Science.gov (United States)

    Martines, G; Musa, N; Aquilino, F; Capuano, P

    2018-01-01

    In the last years with the increase of bariatric surgery, first of all as a result of new indications, a rise in the incidence of nutrient-related complications has been observed. Currently little is known about the impact of post-bariatric malnutrition and neurological complications. Wernicke's encephalopathy is a severe neurological syndrome which occurs as a result of thiamine deficiency. Wernicke-Korsakoff syndrome must be considered a serious neurological complication of bariatric surgery with significant morbidity and mortality, with rapidly progressing neurological symptoms, and must be treated immediately. We report the case of a 35 years-old male patient, affected by morbid obesity, anxious-depressive syndrome and alcohol use disorder, who after adjustable gastric banding implanted in another hospital developed a severe malnutrition and neurological syndrome. The patient showed poor adherence to the follow-up and to the dietary indications and after all, we needed to place a PEG for enteral nutrition in order to resolve the malnutrition condition and the neurological syndrome. Our experience emphasizes that preoperative selection and assessment of a patient's nutritional status according to guidelines, is required to identify potential problems, and that bariatric surgeons or physicians caring for patient who have undergone bariatric surgery should be familiar with the constellation of nutritional and neurological disorder that may occur after surgery. We want to remark the importance of preoperative selection of the patients, the follow-up and the cooperation between patient and physician in order to obtain the best result and avoid severe complications.

  2. Emerging and Underrecognized Complications of Illicit Drug Use

    OpenAIRE

    Wurcel, Alysse G.; Merchant, Elisabeth A.; Clark, Roger P.; Stone, David R.

    2015-01-01

    This comprehensive review of infectious and noninfectious syndromes associated with illicit drug use focuses on underrecognized complications of common illicit drugs and constellations of syndromes associated with emerging illicit drugs.

  3. A case of possible Kounis syndrome as a complication of scombroid syndrome

    Directory of Open Access Journals (Sweden)

    Stefano Rusconi

    2017-11-01

    Full Text Available Kounis syndrome is defined as the concurrence of acute coronary syndromes such as coronary spasm or acute myocardial infarction with conditions associated with activation of inflammatory mediators such histamine, arachidonic acid and various cytokines and chemokines. Recently, a variety of unusual etiologies have been reported, including scombroid syndrome. We present a case of a woman without previous history of cardiac diseases or cardiovascular risk factors, who presented to emergency department after the onset of flushing, asthenia, palpitations, burning sensation in the mouth having just eaten tuna. The electrocardiogram revealed a sinus tachycardia with diffuse ST segment depression. After therapy, in a short time symptoms recovered and a second electrocardiogram no longer showed any ST changes. These electrocardiographic changes observed in our case were probably due to transitory coronary vasospasm as described in type I variant of Kounis syndrome.

  4. Clinical correlates of complicated grief among individuals with acute coronary syndromes

    Directory of Open Access Journals (Sweden)

    Pini S

    2015-10-01

    Full Text Available Stefano Pini,1 Camilla Gesi,1 Marianna Abelli,1 Alessandra Cardini,1 Lisa Lari,1 Francesca Felice,2 Rossella Di Stefano,2 Gianfranco Mazzotta,3 Francesco Bovenzi,4 Daniele Bertoli,5 Lucia Borelli,4 Paola Michi,1 Claudia Oligeri,3 Alberto Balbarini,2 Vijaya Manicavasagar6 1Department of Clinical and Experimental Medicine, Psychiatry Sector, University of Pisa, Pisa, 2Department of Surgical, Medical, Molecular and Critical Area Pathology, University of Pisa, Pisa, 3Unit of Cardiology, Ospedale Sant’Andrea, La Spezia, 4Cardio-Respiratory Department, Ospedale Campo di Marte, Lucca, 5Unit of Cardiology, Ospedale San Bartolomeo, Sarzana (SP, Italy; 6Black Dog Institute, University of New South Wales, Sydney, NSW, Australia Objective: The study aimed at exploring bereavement and complicated grief (CG symptoms among subjects without a history of coronary heart disease (CHD at the time of a first acute coronary syndrome (ACS and to evaluate the relationship of CG symptoms and ACS. Method: Overall, 149 subjects with ACS (namely, acute myocardial infarct with or without ST-segment elevation or unstable angina, with no previous history of CHD, admitted to three cardiac intensive care units were included and evaluated by the Structured Clinical Interview for Complicated Grief (SCI-CG, Hamilton Depression Rating Scale, Hamilton Anxiety Rating Scale, and the 36-item Short-Form Health Survey (MOS-SF-36. Results: Of the total sample of 149 subjects with ACS, 118 (79.2% met criteria for DSM-5 persistent complex bereavement disorder. Among these, subjects who lost a partner, child, or sibling were older (P=0.008, less likely to be working (P=0.032, and more likely to be suffering from hypertension (P=0.021, returned higher scores on the SCI-CG (P=0.001 and developed the index ACS more frequently between 12 and 48 months after the death than those who lost a parent or another relative (P≤0.0001. The occurrence of ACS 12–48 months (P=0.019 after the loss was

  5. Complications and challenges associated with polycystic ovary syndrome: current perspectives

    Directory of Open Access Journals (Sweden)

    Palomba S

    2015-07-01

    Full Text Available Stefano Palomba,1 Susanna Santagni,1 Angela Falbo,1 Giovanni Battista La Sala1,21Unit of Obstetrics and Gynecology, Arcispedale Santa Maria Nuova-Scientific Institute of Treatment and Care (IRCCS, Reggio Emilia, 2Department of Obstetrics and Gynecology, University of Modena and Reggio Emilia, Modena, ItalyAbstract: Polycystic ovary syndrome (PCOS represents the most common endocrine dysfunction in fertile women and it is considered a heterogeneous and multifaceted disorder, with multiple reproductive and metabolic phenotypes which differently affect the early- and long-term syndrome’s risks. Women with PCOS present an adverse reproductive profile, including a high risk of pregnancy-induced hypertension, preeclampsia, and gestational diabetes mellitus. Patients with PCOS present not only a higher prevalence of classic cardiovascular risk factors, such as hypertension, dyslipidemia, and type-2 diabetes mellitus, but also of nonclassic cardiovascular risk factors, including mood disorders, such as depression and anxiety. Moreover, at the moment, clinical data on cardiovascular morbidity and mortality in women with PCOS are controversial. Finally, women with PCOS show an increased risk of endometrial cancer compared to non-PCOS healthy women, particularly during premenopausal period. Currently, we are unable to clarify if the increased PCOS early- and long-term risks are totally due to PCOS per se or mostly due to obesity, in particular visceral obesity, that characterized the majority of PCOS patients. In any case, the main endocrine and gynecological scientific societies agree to consider women with PCOS at increased risk of obstetric, cardiometabolic, oncology, and psychological complications throughout life, and it is recommended that these women be accurately assessed with periodic follow-up.Keywords: cardiovascular disease, infertility, polycystic ovary syndrome, PCOS, pregnancy

  6. Tofacitinib or Adalimumab versus Placebo for Psoriatic Arthritis.

    Science.gov (United States)

    Mease, Philip; Hall, Stephen; FitzGerald, Oliver; van der Heijde, Désirée; Merola, Joseph F; Avila-Zapata, Francisco; Cieślak, Dorota; Graham, Daniela; Wang, Cunshan; Menon, Sujatha; Hendrikx, Thijs; Kanik, Keith S

    2017-10-19

    Tofacitinib is an oral Janus kinase inhibitor that is under investigation for the treatment of psoriatic arthritis. We evaluated tofacitinib in patients with active psoriatic arthritis who previously had an inadequate response to conventional synthetic disease-modifying antirheumatic drugs (DMARDs). In this 12-month, double-blind, active-controlled and placebo-controlled, phase 3 trial, we randomly assigned patients in a 2:2:2:1:1 ratio to receive one of the following regimens: tofacitinib at a 5-mg dose taken orally twice daily (107 patients), tofacitinib at a 10-mg dose taken orally twice daily (104), adalimumab at a 40-mg dose administered subcutaneously once every 2 weeks (106), placebo with a blinded switch to the 5-mg tofacitinib dose at 3 months (52), or placebo with a blinded switch to the 10-mg tofacitinib dose at 3 months (53). Placebo groups were pooled for analyses up to month 3. Primary end points were the proportion of patients who had an American College of Rheumatology 20 (ACR20) response (≥20% improvement from baseline in the number of tender and swollen joints and at least three of five other important domains) at month 3 and the change from baseline in the Health Assessment Questionnaire-Disability Index (HAQ-DI) score (scores range from 0 to 3, with higher scores indicating greater disability) at month 3. ACR20 response rates at month 3 were 50% in the 5-mg tofacitinib group and 61% in the 10-mg tofacitinib group, as compared with 33% in the placebo group (P=0.01 for the comparison of the 5-mg dose with placebo; Ptofacitinib group and -0.40 in the 10-mg tofacitinib group, as compared with -0.18 in the placebo group (P=0.006 for the comparison of the 5-mg dose with placebo; Ptofacitinib group, 71% in the 10-mg tofacitinib group, 72% in the adalimumab group, 69% in the placebo group that switched to the 5-mg tofacitinib dose, and 64% in the placebo group that switched to the 10-mg tofacitinib dose. There were four cases of cancer, three serious

  7. Review of the refeeding syndrome.

    Science.gov (United States)

    Kraft, Michael D; Btaiche, Imad F; Sacks, Gordon S

    2005-12-01

    Refeeding syndrome describes a constellation of metabolic disturbances that occur as a result of reinstitution of nutrition to patients who are starved or severely malnourished. Patients can develop fluid and electrolyte disorders, especially hypophosphatemia, along with neurologic, pulmonary, cardiac, neuromuscular, and hematologic complications. We reviewed literature on refeeding syndrome and the associated electrolyte abnormalities, fluid disturbances, and associated complications. In addition to assessing scientific literature, we also considered clinical experience and judgment in developing recommendations for prevention and treatment of refeeding syndrome. The most important steps are to identify patients at risk for developing refeeding syndrome, institute nutrition support cautiously, and correct and supplement electrolyte and vitamin deficiencies to avoid refeeding syndrome. We provide suggestions for the prevention of refeeding syndrome and suggestions for treatment of electrolyte disturbances and complications in patients who develop refeeding syndrome, according to evidence in the literature, the pathophysiology of refeeding syndrome, and clinical experience and judgment.

  8. Cauda equina syndrome complicating ankylosing spondylitis: use of electromyography and computerised tomography in diagnosis.

    Science.gov (United States)

    Young, A; Dixon, A; Getty, J; Renton, P; Vacher, H

    1981-06-01

    A case of the cauda equina syndrome complicating ankylosing spondylitis (AS) is described. An unusual feature of this case was the relapsing and remitting nature of the condition, but there is sufficient evidence to explain the clinical picture on the basis of a recurrent intraspinal inflammatory process. The clinical and radiological features are similar to those of a further 28 reported in the literature. An electromyogram (EMG) proved important in defining the extent of neurological involvement. Computerised tomography (CT) showed marked laminar erosion and no bony exit foramen encroachment. We believe that the clinical diagnosis of this condition can be adequately confirmed with plain radiology, EMG, and CT scan.

  9. Efficacy of metformin on pregnancy complications in women with polycystic ovary syndrome: a meta-analysis.

    Science.gov (United States)

    Feng, Li; Lin, Xiao-Fang; Wan, Zhi-Hua; Hu, Dan; Du, Yu-Kai

    2015-01-01

    To evaluate the efficacy of metformin administration throughout pregnancy on pregnancy-related complications in women with polycystic ovary syndrome (PCOS). MEDLINE and ScienceDirect were searched to retrieve relevant trials. The endpoint was the incidence of complications of pregnancy, gestational diabetes mellitus (GDM), pre-eclampsia (PE), miscarriage and premature birth included. Five studies with 502 PCOS patients with metformin administration throughout pregnancy and 427 controls who used metformin just to get conception were included in our meta-analysis. In study group, a significantly lower change of emerging miscarriage and premature birth was observed, the pooled relative risk (RR) was 0.32 (95% confidence interval (CI): 0.19-0.56) for miscarriage and 0.40 (95%CI: 0.18-0.91) for premature birth. No significant difference was demonstrated in emerging GDM and PE. Metformin therapy throughout pregnancy can reduce the RR of miscarriage and premature birth incidence in PCOS patients with no serious side effects.

  10. Hepatorenal syndrome

    Science.gov (United States)

    ... 2016:chap 153. Nevah MI, Fallon MB. Hepatic encephalopathy, hepatorenal syndrome, hepatopulmonary syndrome, and other systemic complications of liver disease. In: Feldman M, Friedman LS, Brandt LJ, ...

  11. Thoracic air-leakage syndrome in allogeneic stem cell transplant recipients as a late complication of chronic graft-versus-host disease: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Park, Jae Wook; Kim, Song Soo; Jo, Daeg Yeon; Yun, Hwan Jung; Lee, Hyo Jin; Kim, Jin Hwan [Chungnam National University Hospital, Chungnam National University School of Medicine, Daejeon (Korea, Republic of)

    2016-08-15

    Air-leakage syndrome associated with graft-versus-host disease (GVHD) is a rare complication, but it is also reported as an independent predictor of a worse survival rate after stem cell transplantation. We report two cases of air-leakage syndrome associated with GVHD after allogeneic stem cell transplantation in acute leukemia patients who presented with spontaneous pneumomediastinum and subcutaneous emphysema, and finally death due to respiratory failure seven to eight months later.

  12. Prevalence of polycystic ovary syndrome and its associated complications in Iranian women: A meta-analysis.

    Science.gov (United States)

    Jalilian, Anahita; Kiani, Faezeh; Sayehmiri, Fatemeh; Sayehmiri, Kourosh; Khodaee, Zahra; Akbari, Malihe

    2015-10-01

    Polycystic ovary syndrome (PCOS) is the most common endocrine disorder in women of reproductive age and is the most common cause of infertility due to anovulation. There is no single criterion for the diagnosis of this syndrome. The purpose of this study was to investigate the prevalence of PCOS and its associated complications in Iranian women using meta-analysis method. Prevalence of PCOS was investigated from the SID, Goggle scholar, PubMed, Magiran, Irandoc, and Iranmedex, and weighting of each study was calculated according to sample size and prevalence of the binomial distribution. Data were analyzed using a random-effects model meta-analysis (Random effects model) and the software R and Stata Version 11.2. 30 studies conducted between the years 2006 to 2011 were entered into meta-analysis. The total sample size was 19, 226 women aged between 10-45 years. The prevalence of PCOS based on National institute of child health and human disease of the U.S was, 6.8% (95 % CI: 4.11-8.5), based on Rotterdam was 19.5% (95 % CI: 2.24-8.14), and based on ultrasound was 4.41% (95% CI: 5.68-4.14). Also, the prevalence of hirsutism was estimated to be 13%, acne 26%, androgenic alopecia 9%, menstrual disorders 28%, overweight 21%, obesity 19%, and infertility 8%. The prevalence of PCOS in Iran is not high. However, given the risk of complications such as heart disease - cardiovascular and infertility, prevention of PCOS is important; we suggest that health officials must submit plans for the community in this respect.

  13. Lyme neuroborreliosis in a patient treated with TNF-alpha inhibitor.

    Science.gov (United States)

    Merkac, Maja Ivartnik; Tomazic, Janez; Strle, Franc

    2015-12-01

    A 57-year-old woman, receiving TNF-alpha inhibitor adalimumab for psoriasis, presented with early Lyme neuroborreliosis (Bannwarth's syndrome). Discontinuation of adalimumab and 14-day therapy with ceftriaxone resulted in a smooth course and favorable outcome of Lyme borreliosis. This is the first report on Lyme neuroborreliosis in a patient treated with TNF-alpha inhibitor.

  14. Venous thromboembolism and arterial complications.

    Science.gov (United States)

    Prandoni, Paolo; Piovella, Chiara; Pesavento, Raffaele

    2012-04-01

    An increasing body of evidence suggests the likelihood of a link between venous and arterial thrombosis. The two vascular complications share several risk factors, such as age, obesity, smoking, diabetes mellitus, blood hypertension, hypertriglyceridemia, and metabolic syndrome. Moreover, there are many examples of conditions accounting for both venous and arterial thrombosis, such as the antiphospholipid antibody syndrome, hyperhomocysteinemia, malignancies, infections, and the use of hormonal treatment. Finally, several recent studies have consistently shown that patients with venous thromboembolism are at a higher risk of arterial thrombotic complications than matched control individuals. We, therefore, speculate the two vascular complications are simultaneously triggered by biological stimuli responsible for activating coagulation and inflammatory pathways in both the arterial and the venous system. Future studies are needed to clarify the nature of this association, to assess its extent, and to evaluate its implications for clinical practice. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  15. [Tubulointerstitial nephritis with uveitis (TINU) syndrome. A relatively rare rheumatological differential diagnosis with unexplained uveitis].

    Science.gov (United States)

    Häusler, U; Guminski, B; Helmchen, U; Kisters, K; Heinz, C; Braun, J

    2013-05-01

    The tubulo-interstitial nephritis and uveitis (TINU) syndrome, first described in 1975, is a rare disease most probably of autoimmune origin that is characterized by unilateral or bilateral uveitis and tubulointerstitial nephritis. Most patients are adolescents and it is sometimes associated with other autoimmune diseases, such as spondyloarthritis, rheumatoid arthritis and hyperthyroidosis. This article reports the case of a 43-year-old female patient who presented with refractory recurrent bilateral uveitis despite therapy with high doses of corticosteroids in combination with cyclosporin. When the patient was referred to this hospital for rheumatological examination after almost 1 year of therapy, mild renal insufficiency and proteinuria were found. The kidney biopsy revealed interstitial nephritis, partly crescent-shaped and partly chronic. A diagnosis of TINU syndrome was made and treatment with adalimumab in combination with methotrexate was started. The favorable clinical outcome indicated that tumor necrosis factor (TNF) alpha may play an important role in the pathogenesis of TINU syndrome.

  16. The hypertension of Cushing's syndrome: controversies in the pathophysiology and focus on cardiovascular complications

    Science.gov (United States)

    Isidori, Andrea M.; Graziadio, Chiara; Paragliola, Rosa Maria; Cozzolino, Alessia; Ambrogio, Alberto G.; Colao, Annamaria; Corsello, Salvatore M.; Pivonello, Rosario

    2015-01-01

    Cushing's syndrome is associated with increased mortality, mainly due to cardiovascular complications, which are sustained by the common development of systemic arterial hypertension and metabolic syndrome, which partially persist after the disease remission. Cardiovascular diseases and hypertension associated with endogenous hypercortisolism reveal underexplored peculiarities. The use of exogenous corticosteroids also impacts on hypertension and cardiovascular system, especially after prolonged treatment. The mechanisms involved in the development of hypertension differ, whether glucocorticoid excess is acute or chronic, and the source endogenous or exogenous, introducing inconsistencies among published studies. The pleiotropic effects of glucocorticoids and the overlap of the several regulatory mechanisms controlling blood pressure suggest that a rigorous comparison of in-vivo and in-vitro studies is necessary to draw reliable conclusions. This review, developed during the first ‘Altogether to Beat Cushing's syndrome’ workshop held in Capri in 2012, evaluates the most important peculiarities of hypertension associated with CS, with a particular focus on its pathophysiology. A critical appraisal of most significant animal and human studies is compared with a systematic review of the few available clinical trials. A special attention is dedicated to the description of the clinical features and cardiovascular damage secondary to glucocorticoid excess. On the basis of the consensus reached during the workshop, a pathophysiology-oriented therapeutic algorithm has been developed and it could serve as a first attempt to rationalize the treatment of hypertension in Cushing's syndrome. PMID:25415766

  17. Intraabdominal Compartment Syndrome Complicating Transurethral Resection of Bladder Tumor

    Directory of Open Access Journals (Sweden)

    Sachin Narain

    2012-01-01

    Full Text Available Abdominal compartment syndrome can result from many different causes. We present a case where this dangerous syndrome occurred in the operating room during a transurethral resection of a bladder tumor. It was initially recognized by an elevation in the peak inspiratory pressure. We report the typical physiologic changes that occur with this syndrome and its treatment options.

  18. Anterior pituitary lobe atrophy as late complication of hemorrhagic fever with renal syndrome

    Directory of Open Access Journals (Sweden)

    Jovanović Dragan

    2009-01-01

    Full Text Available Introduction. Hemorrhagic fever with renal syndrome (HFRS is acute infective multisystemic disease followed by febrility, hemorrhages and acute renal insufficiency. Bleeding in the anterior pituitary lobe leading to tissue necrosis occurs in acute stage of severe clinical forms of HFRS, while atrophy of the anterior pituitary lobe with diminution of the gland function occurs after recovery stage. Case report. We presented a patient with the development of chronic renal insufficiency and hypopituitarism as complication that had been diagnosed six years after Hantavirus infection. Magnetic resonance of the pituitary gland revealed atrophy and empty sella turcica. Conclusion. Regarding frequency of this viral infection and its endemic character in some parts of our country partial and/or complete loss of pituitary function should be considered during the late stage of HFRS.

  19. Prevalence of polycystic ovary syndrome and its associated complications in Iranian women: A meta-analysis

    Directory of Open Access Journals (Sweden)

    Anahita Jalilian

    2015-10-01

    Full Text Available Background: Polycystic ovary syndrome (PCOS is the most common endocrine disorder in women of reproductive age and is the most common cause of infertility due to anovulation. There is no single criterion for the diagnosis of this syndrome. Objective: The purpose of this study was to investigate the prevalence of PCOS and its associated complications in Iranian women using meta-analysis method. Materials and Methods: Prevalence of PCOS was investigated from the SID, Goggle scholar, PubMed, Magiran, Irandoc, and Iranmedex, and weighting of each study was calculated according to sample size and prevalence of the binomial distribution. Data were analyzed using a random-effects model meta-analysis (Random effects model and the software R and Stata Version 11.2. Results: 30 studies conducted between the years 2006 to 2011 were entered into meta-analysis. The total sample size was 19, 226 women aged between 10-45 years. The prevalence of PCOS based on National institute of child health and human disease of the U.S was, 6.8% (95 % CI: 4.11–8.5, based on Rotterdam was 19.5% (95 % CI: 2.24-8.14, and based on ultrasound was 4.41% (95% CI: 5.68-4.14. Also, the prevalence of hirsutism was estimated to be 13%, acne 26%, androgenic alopecia 9%, menstrual disorders 28%, overweight 21%, obesity 19%, and infertility 8%. Conclusion: The prevalence of PCOS in Iran is not high. However, given the risk of complications such as heart disease - cardiovascular and infertility, prevention of PCOS is important; we suggest that health officials must submit plans for the community in this respect.

  20. Evans syndrome with non-ST segment elevation myocardial infarction complicated by hemopericardium

    Directory of Open Access Journals (Sweden)

    Filiz Kizilirmak

    2016-09-01

    Full Text Available Evans syndrome (ES is a rare hematological disease characterized by autoimmune hemolytic anemia, immune thrombocytopenia, and/or neutropenia, all of which may be seen simultaneously or subsequently. Thrombotic events in ES are uncommon. Furthermore, non-ST segment-elevation myocardial infarction (NSTEMI during ES is a very rare condition. Here, we describe a case of a 69-year-old female patient presenting with NSTEMI and ES. Revascularization via percutaneous coronary intervention (PCI was scheduled and performed. Hemopericardium and cardiac tamponade occurred 5 h after PCI, and urgent pericardiocentesis was performed. Follow-up was uneventful, and the patient was safely discharged. Early recognition and appropriate management of NSTEMI is crucial to prevent morbidity and mortality. Coexistence of NSTEMI and ES, which is associated with increased bleeding risk, is a challenging scenario and these patients should be closely monitored in order to achieve early recognition and treatment of complications.

  1. Anesthetic Implications of Emergent Cesarean Section in a Parturient with Marfan Syndrome Complicated by Ascending Aortic Aneurysm and Heart Failure

    Directory of Open Access Journals (Sweden)

    Young Sung Kim

    2014-07-01

    Full Text Available Cardiovascular comorbidities to the Marfan syndrome may induce hemodynamic instability especially in the parturients during labor or delivery. For anesthesiologists, it is challenging to maintain hemodynamic stability during Cesarean section in those patients with Marfan syndrome. Remifentanil is an ultra-short-acting opioid with rapid onset and offset of action which provides cardiovascular stability during surgery. Together with remifentanil, the use of a laryngeal mask airway can reduce the risk of hypertensive response followed by tracheal intubation. We describe the successful administration of remifentanil and application of laryngeal mask airway for emergent Cesarean section performed under general anesthesia in a patient with Marfan syndrome complicated by ascending aortic aneurysm and heart failure. The use of remifentanil (loading dose of 1 μg/kg for 1 min, 2 min before induction; thereafter continuous infusion dose of 0.1 μg/kg/min was useful to maintain hemodynamic stability of the parturient throughout the surgery without neonatal respiratory depression. Keywords: Ascending Aortic Aneurysm; Cesarean section; Laryngeal mask airway; Marfan syndrome; Remifentanil

  2. The imaging of coeliac disease and its complications

    International Nuclear Information System (INIS)

    Buckley, O.; Brien, J.O.; Ward, E.; Doody, O.; Govender, P.; Torreggiani, W.C.

    2008-01-01

    Coeliac disease is a malabsorption syndrome in which dietary gluten damages the small bowel mucosa. Gluten contains gliadin, the primary toxic component that is primarily found in wheat, barley and rye products. The initial diagnosis of coeliac disease is usually made by endoscopic biopsy of the jejunum although sometimes imaging features can suggest the diagnosis. Once a diagnosis is made, patients need to be diet compliant and monitored for potential complications. Many complications are more common when dietary compliance is poor. Complications include intussusception (usually intermittent), ulcerative jejunitis, osteomalacia, cavitating lymph node syndrome and an increased risk of malignancies such as lymphoma, adenocarcinoma and squamous cell carcinoma. Radiological evaluation is central in the evaluation of these complications. Imaging may assist both in the diagnosis and staging of complications as well as enabling radiological guided percutaneous biopsy for complications of coeliac disease such as lymphoma. As coeliac disease is a relatively common disorder, it is likely that most radiologists will encounter the disease and its potential complications. The aim of this review article is to discuss and illustrate the role of modern radiology in evaluating the many presentations of this complex disease

  3. The imaging of coeliac disease and its complications

    Energy Technology Data Exchange (ETDEWEB)

    Buckley, O.; Brien, J.O.; Ward, E.; Doody, O.; Govender, P. [Adelaide and Meath Incorporating the National Children' s Hospital, Dublin (Ireland); Torreggiani, W.C. [Adelaide and Meath Incorporating the National Children' s Hospital, Dublin (Ireland)], E-mail: William.torreggiani@amnch.ie

    2008-03-15

    Coeliac disease is a malabsorption syndrome in which dietary gluten damages the small bowel mucosa. Gluten contains gliadin, the primary toxic component that is primarily found in wheat, barley and rye products. The initial diagnosis of coeliac disease is usually made by endoscopic biopsy of the jejunum although sometimes imaging features can suggest the diagnosis. Once a diagnosis is made, patients need to be diet compliant and monitored for potential complications. Many complications are more common when dietary compliance is poor. Complications include intussusception (usually intermittent), ulcerative jejunitis, osteomalacia, cavitating lymph node syndrome and an increased risk of malignancies such as lymphoma, adenocarcinoma and squamous cell carcinoma. Radiological evaluation is central in the evaluation of these complications. Imaging may assist both in the diagnosis and staging of complications as well as enabling radiological guided percutaneous biopsy for complications of coeliac disease such as lymphoma. As coeliac disease is a relatively common disorder, it is likely that most radiologists will encounter the disease and its potential complications. The aim of this review article is to discuss and illustrate the role of modern radiology in evaluating the many presentations of this complex disease.

  4. Unusual complication and successful high-dose chemotherapy ...

    African Journals Online (AJOL)

    ... treated with high-dose chemotherapy in our institution, complicated by unusual bilateral renal vein tumour thrombi and tumour lysis syndrome. We believe this unique case highlights the need for early recognition of current and potential complications on staging computed tomography imaging, as well as successful use of ...

  5. Introduction of a glycosylation site in the constant region decreases the aggregation of adalimumab Fab.

    Science.gov (United States)

    Nakamura, Hitomi; Oda-Ueda, Naoko; Ueda, Tadashi; Ohkuri, Takatoshi

    2018-06-18

    The production of therapeutic monoclonal antibodies is costly; therefore, antigen-binding fragments (Fabs) can be used instead. However, their tendency toward aggregation can reduce the half-life in the plasma and the therapeutic effectiveness. To examine the effect of glycosylation on the properties of the Fab of a therapeutic antibody, an N-glycosylation site was introduced at position 178 of the H-chain constant region of adalimumab Fab through site-directed mutagenesis of L178 N (H:L178 N Fab), and then H:L178 N Fab was expressed in Pichia pastoris. SDS-PAGE analysis with treatment of N-glycosidase F or periodic acid-Schiff reagent showed that H:L178 N Fab contained a relatively low glycan level. Moreover, the H:L178 N mutation did not decrease the binding activity and thermal stability of Fab, and H:L178 N Fab was more resistant to protease digestion than wild-type Fab. The aggregation of Fab induced by pH-shift stress was measured by monitoring the optical density at 350 nm. Although the wild-type Fab showed a large increase in optical density with an increase of protein concentration, no such increase of turbidity during aggregation was found in H:L178 N Fab. These results demonstrated that glycosylation at position 178 of the H-chain constant region of adalimumab Fab can prevent protein aggregation, and therefore serve as a potentially effective platform for drug development. Copyright © 2018. Published by Elsevier Inc.

  6. A novel engineered interchain disulfide bond in the constant region enhances the thermostability of adalimumab Fab.

    Science.gov (United States)

    Nakamura, Hitomi; Oda-Ueda, Naoko; Ueda, Tadashi; Ohkuri, Takatoshi

    2018-01-01

    We constructed a system for expressing the Fab of the therapeutic human monoclonal antibody adalimumab at a yield of 20 mg/L in the methylotrophic yeast Pichia pastoris. To examine the contribution of interchain disulfide bonds to conformational stability, we prepared adalimumab Fab from which the interchain disulfide bond at the C-terminal region at both the CH 1 and CL domains was deleted by substitution of Cys with Ala (Fab ΔSS ). DSC measurements showed that the Tm values of Fab ΔSS were approximately 5 °C lower than those of wild-type Fab, suggesting that the interchain disulfide bond contributes to conformational thermostability. Using computer simulations, we designed a novel interchain disulfide bond outside the C-terminal region to increase the stability of Fab ΔSS . The resulting Fab (mutSS Fab ΔSS ) had the mutations H:V177C and L:Q160C in Fab ΔSS , confirming the formation of the disulfide bond between CH 1 and CL. The thermostability of mutSS Fab ΔSS was approximately 5 °C higher than that of Fab ΔSS . Therefore, the introduction of the designed interchain disulfide bond enhanced the thermostability of Fab ΔSS and mitigated the destabilization caused by partial reduction of the interchain disulfide bond at the C-terminal region, which occurs in site-specific modification such as PEGylation. Copyright © 2017 Elsevier Inc. All rights reserved.

  7. [Morel-Lavallée syndrome and post-traumatic nodular fat necrosis: Two post-traumatic complications mimicking cellulitis].

    Science.gov (United States)

    Moulin, C; Barthélémy, I; Emering, C; D'Incan, M

    Dermal and subcutaneous inflammation following direct trauma is initially evocative of soft-tissue infection. However, two differential diagnoses must be considered: Morel-Lavallée syndrome and post-traumatic nodular fat necrosis. Case 1: a 51-year-old woman fell off her motorbike and had dermabrasions on her right and left tibial ridges that rapidly developed into dermo-hypodermitis of the entire limb. There was no improvement after 3 weeks of antibiotics. The patient was apyretic. She had a soft, non-inflammatory tumefaction on the inner aspect of her left knee. Ultrasound revealed subcutaneous collection in both legs. The surgeons confirmed a diagnosis of Morel-Lavallée syndrome and drained the two collections. Progress was good and the patient healed without major consequences. Case 2: following a fall on her stairs, a 40-year-old woman presented dermabrasions and haematomas on her left leg. Antibiotic therapy failed to prevent the progression of dermo-hypodermitis. The patient remained apyretic and there was no inflammatory syndrome. A CT scan showed thickening of a subcutaneous fat and fluid collection, resulting in diagnosis of post-traumatic nodular fat necrosis. Management was surgical and the outcome was good. These two cases show two post-traumatic cutaneous complications: Morel-Lavallée syndrome and post-traumatic nodular fat necrosis. Morel-Lavallée syndrome occurs after tangential trauma next to richly vascularized tissue. Post-traumatic nodular fat necrosis is defined as necrosis of adipocytes. In both cases, diagnosis is confirmed by imagery (Ultrasonography, tomography). Our two case reports show that inflammatory presentation of both Morel-Lavallée syndrome and post-traumatic nodular fat necrosis can lead to diagnostic and therapeutic errors while a surgical procedure is necessary since tissue necrosis can occur. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  8. Neurological complications of Zika virus infection.

    Science.gov (United States)

    Carod-Artal, Francisco Javier

    2018-04-26

    Zika virus (ZIKV) disease is a vector-borne infectious disease transmitted by Aedes mosquitoes. Recently, ZIKV has caused outbreaks in most American countries. Areas covered: Publications about neurological complications of ZIKV infection retrieved from pubmed searchers were reviewed, and reference lists and relevant articles from review articles were also examined. Vertical/intrauterine transmission leads to congenital infection and causes microcephaly and congenital ZIKV syndrome. ZIKV preferentially infects human neural progenitor cells and triggers cell apoptosis. ZIKV RNA has been identified in foetal brain tissue and brains of microcephalic infants who died; amniotic fluid and placentas of pregnant mothers; and umbilical cord, cerebro-spinal fluid and meninges of newborns. The increase in the number of Guillain-Barre syndrome (GBS) cases during the ZIKV outbreak in the Americas provides epidemiological evidence for the link between ZIKV infection and GBS. Less frequently reported ZIKV neurological complications include encephalitis/meningoencephalitis, acute disseminated encephalomyelitis, myelitis, cerebrovascular complications (ischemic infarction; vasculopathy), seizures and encephalopathy, sensory polyneuropathy and sensory neuronopathy. Analysis of GBS incidence could serve as an epidemiological 'marker' or sentinel for ZIKV disease and other neurological complications associated to ZIKV. Expert commentary: An expanding spectrum of neurological complications associated with ZIKV infection is being recognised.

  9. Subcutaneous Injection of Adalimumab Trial compared with Control (SCIATiC): a randomised controlled trial of adalimumab injection compared with placebo for patients receiving physiotherapy treatment for sciatica.

    Science.gov (United States)

    Williams, Nefyn H; Jenkins, Alison; Goulden, Nia; Hoare, Zoe; Hughes, Dyfrig A; Wood, Eifiona; Foster, Nadine E; Walsh, David A; Carnes, Dawn; Sparkes, Valerie; Hay, Elaine M; Isaacs, John; Konstantinou, Kika; Morrissey, Dylan; Karppinen, Jaro; Genevay, Stephane; Wilkinson, Clare

    2017-10-01

    Biological treatments such as adalimumab (Humira ® ; AbbVie Ltd, Maidenhead, UK) are antibodies targeting tumour necrosis factor alpha, released from ruptured intervertebral discs, which might be useful in sciatica. Recent systematic reviews concluded that they might be effective, but that a definitive randomised controlled trial was needed. Usual care in the NHS typically includes a physiotherapy intervention. To test whether or not injections of adalimumab plus physiotherapy are more clinically effective and cost-effective than injections of saline plus physiotherapy for patients with sciatica. Pragmatic, parallel-group, randomised controlled trial with blinded participants and clinicians, and an outcome assessment and statistical analysis with concurrent economic evaluation and internal pilot. Participants were referred from primary care and musculoskeletal services to outpatient physiotherapy clinics. Adults with persistent symptoms of sciatica of 1-6 months' duration and with moderate to high levels of disability. Eligibility was assessed by research physiotherapists according to clinical criteria for diagnosing sciatica. After a second eligibility check, trial participants were randomised to receive two doses of adalimumab (80 mg and then 40 mg 2 weeks later) or saline injections. Both groups were referred for a course of physiotherapy. Outcomes were measured at the start, and after 6 weeks' and 6 months' follow-up. The main outcome measure was the Oswestry Disability Index (ODI). Other outcomes: leg pain version of the Roland-Morris Disability Questionnaire, Sciatica Bothersomeness Index, EuroQol-5 Dimensions, 5-level version, Hospital Anxiety and Depression Scale, resource use, risk of persistent disabling pain, pain trajectory based on a single question, Pain Self-Efficacy Questionnaire, Tampa Scale of Kinesiophobia and adverse effects. To detect an effect size of 0.4 with 90% power, a 5% significance level for a two-tailed t -test and 80% retention

  10. Four Cases of Atopic Dermatitis Complicated by Sjogren's Syndrome: Link between Dry Skin and Autoimmune Anhidrosis

    Directory of Open Access Journals (Sweden)

    Shun Kitaba

    2011-01-01

    Full Text Available We report four adult cases of atopic dermatitis (AD complicated by Sjogren's syndrome (SS. The patients fulfilled diagnostic criteria for AD and SS. All cases showed persistent itchy dry skin and eczematous lesions complicated by sicca symptoms including dry eyes and dry mouth with moderate joint pain. One case manifested annular erythema and another manifested widespread discoid erythema. To investigate the underlying cause of dry skin in these cases, sweating function was evaluated using a quantitative sudomotor axon reflex test (QSART in which the axon reflex is stimulated by acetylcholine iontophoresis. The sweating latency time was significantly prolonged in eczematous skin of AD and AD/SS compared to normal controls. Axon reflex (AXR sweat volume was also significantly reduced in AD (normal and eczematous skin and AD/SS (normal and eczema compared to normal control. In contrast, the direct sweat volume of lesional or non-lesional AD skin induced by direct stimulation with acetylcholine was only slightly reduced compared to that in normal controls, but not in SS and lesional skin of AD/SS patients. These results suggest that the impaired sweat response in AD is attributable to an abnormal sudomotor axon reflex, which is accelerated and modulated when complicated by SS resulting in dry skin in the present cases.

  11. Total bile acids in the maternal and fetal compartment in relation to placental ABCG2 expression in preeclamptic pregnancies complicated by HELLP syndrome

    NARCIS (Netherlands)

    Jebbink, Jiska; Veenboer, Geertruda; Boussata, Souad; Keijser, Remco; Kremer, Andreas E.; Elferink, Ronald Oude; van der Post, Joris; Afink, Gijs; Ris-Stalpers, Carrie

    2015-01-01

    To investigate total bile acid (TBA) levels in maternal (MB) and umbilical cord blood (UCB) in normotensive, preeclamptic (PE), and PE pregnancies complicated by hemolysis elevated liver enzymes and low platelets (HELLP) syndrome in the context of ABCG2 placental gene expression levels, a recently

  12. Pulmonary complications in neurosurgical patients

    Directory of Open Access Journals (Sweden)

    Randeep Guleria

    2012-01-01

    Full Text Available Pulmonary complications are a major cause of morbidity and mortality in neurosurgical patients. The common pulmonary complications in neurosurgical patients include pneumonia, postoperative atelectasis, respiratory failure, pulmonary embolism, and neurogenic pulmonary edema. Postoperative lung expansion strategies have been shown to be useful in prevention of the postoperative complications in surgical patients. Low tidal volume ventilation should be used in patients who develop acute respiratory distress syndrome. An antibiotic use policy should be put in practice depending on the local patterns of antimicrobial resistance in the hospital. Thromboprophylactic strategies should be used in nonambulatory patients. Meticulous attention should be paid to infection control with a special emphasis on hand-washing practices. Prevention and timely management of these complications can help to decrease the morbidity and mortality associated with pulmonary complications.

  13. Post dengue neurological complication

    Directory of Open Access Journals (Sweden)

    Hizlinda Tohid

    2015-12-01

    Full Text Available Dengue infection is highly endemic in many tropical countries including Malaysia. However, neurological complications arising from dengue infection is not common; Gullain–Barre syndrome (GBS is one of these infrequent complications. In this paper, we have reported a case in which a 39-year-old woman presented with a neurological complication of dengue infection without typical symptoms and signs of dengue fever. She had a history of acute gastroenteritis (AGE followed by an upper respiratory tract infection (URTI weeks prior to her presentation rendering GBS secondary to the post viral URTI and AGE as the most likely diagnosis. Presence of thrombocytopenia was the only clue for dengue in this case.

  14. Investigation of an outbreak of bloody diarrhea complicated with hemolytic uremic syndrome

    Directory of Open Access Journals (Sweden)

    Otar Chokoshvili

    2014-12-01

    Full Text Available In July–August 2009, eight patients with bloody diarrhea complicated by hemolytic uremic syndrome (HUS were admitted to hospitals in Tbilisi, Georgia. We started active surveillance in two regions for bloody diarrhea and post-diarrheal HUS. Of 25 case-patients who developed HUS, including the initial 8 cases, half were ⩾15 years old, 67% were female and seven (28% died. No common exposures were identified. Among 20 HUS case-patients tested, Shiga toxin was detected in the stools of 2 patients (one with elevated serum IgG titers to several Escherichia coli serogroups, including O111 and O104. Among 56 persons with only bloody diarrhea, we isolated Shiga toxin-producing E. coli (STEC O104:H4 from 2 and Shigella from 10; 2 had serologic evidence of E. coli O26 infection. These cases may indicate a previously unrecognized burden of HUS in Georgia. We recommend national reporting of HUS and improving STEC detection capacity.

  15. Protein-Losing Enteropathy as a Complication of the Ketogenic Diet

    OpenAIRE

    Ahn, Won Kee; Park, Soyoung; Kim, Heung Dong

    2017-01-01

    The ketogenic diet is an effective treatment for the patients with intractable epilepsy, however, the diet therapy can sometimes be discontinued by complications. Protein?losing enteropathy is a rarely reported serious complication of the ketogenic diet. We present a 16-month-old Down syndrome baby with protein-losing enteropathy during the ketogenic diet as a treatment for West syndrome. He suffered from diarrhea, general edema and hypoalbuminemia which were not controlled by conservative ca...

  16. COMPARATIVE EVALUATION OF THE EFFICACY AND SAFETY OF TREATMENT USING ADALIMUMAB IN COMBINATION WITH METHOTREXATE AND METHOTREXATE MONOTHERAPY IN CHILDREN WITH POLYARTICULAR JUVENILE IDIOPATHIC ARTHRITIS IN COMBINATION WITH UVEITIS

    Directory of Open Access Journals (Sweden)

    V. А. Kel’tsev

    2014-01-01

    Full Text Available Juvenile idiopathic arthritis (JIA is the most common rheumatic disease in children and it is characterized by a primary lesion of the joints, organs and tissues with the formation of multiple organ failure of varying severity. The article describes the results of studying the efficacy and safety of adalimumab in combination with methotrexate (n = 26 and methotrexate monotherapy (n = 17 when treating the patients with polyarticular JIA and uveitis refractory to the basic immunosuppressive therapy. It was shown that the combination therapy induced the remission of arthritis in children with JIA in a shorter period of time. After 1 year, the disease remission was recorded in 42% of children in the treatment group and in 18% of children in the comparison group, the uveitis remission — in 26 (54% of 48 eyes and 2 (7% of 28 eyes with signs of lesions, respectively. It should be noted that adalimumab in combination with methotrexate was well tolerated and no serious adverse effects were recorded. Thus, the introduction of adalimumab in the treatment regimen of children with JIA and uveitis refractory to the basic immunosuppressive therapy allowed for the rapid disease remission while preserving the effect in a significant number of patients during the following year.

  17. Sjogren′s Syndrome: A Review

    Directory of Open Access Journals (Sweden)

    Rani Somani

    2011-01-01

    Full Text Available Sjogren′s syndrome, also known as "Mikulicz disease" or "Sicca syndrome" is a systemic autoimmune disease in which immune cells attack and destroy the exocrine glands that produce tears and saliva. It can exist by itself (primary Sjogren syndrome or develop in association with another disorder such as rheumatoid arthritis, systemic sclerosis, primary biliary cirrhosis or Hashimoto thyroiditis (associated Sjogren syndrome. Hallmarks are the dry mouth and dry eyes known as the Sicca syndrome. Sjogren syndrome affects t million to 4 million people in the United States- Most are over 40 years old at the time of diagnosis. As there is no known cure for Sjogren syndrome, treatment focuses on relieving symptoms and preventing complications. The most serious complication associated with primary Sjogren syndrome is the development of a lymphoproliferative disease. primarily non-Hodgkin lymphoma.

  18. What Is Respiratory Distress Syndrome?

    Science.gov (United States)

    ... Home / Respiratory Distress Syndrome Respiratory Distress Syndrome Also known as What Is Respiratory ... This condition is called apnea (AP-ne-ah). Respiratory Distress Syndrome Complications Depending on the severity of ...

  19. Adipose tissue mitochondrial dysfunction triggers a lipodystrophic syndrome with insulin resistance, hepatosteatosis, and cardiovascular complications.

    Science.gov (United States)

    Vernochet, Cecile; Damilano, Federico; Mourier, Arnaud; Bezy, Olivier; Mori, Marcelo A; Smyth, Graham; Rosenzweig, Anthony; Larsson, Nils-Göran; Kahn, C Ronald

    2014-10-01

    Mitochondrial dysfunction in adipose tissue occurs in obesity, type 2 diabetes, and some forms of lipodystrophy, but whether this dysfunction contributes to or is the result of these disorders is unknown. To investigate the physiological consequences of severe mitochondrial impairment in adipose tissue, we generated mice deficient in mitochondrial transcription factor A (TFAM) in adipocytes by using mice carrying adiponectin-Cre and TFAM floxed alleles. These adiponectin TFAM-knockout (adipo-TFAM-KO) mice had a 75-81% reduction in TFAM in the subcutaneous and intra-abdominal white adipose tissue (WAT) and interscapular brown adipose tissue (BAT), causing decreased expression and enzymatic activity of proteins in complexes I, III, and IV of the electron transport chain (ETC). This mitochondrial dysfunction led to adipocyte death and inflammation in WAT and a whitening of BAT. As a result, adipo-TFAM-KO mice were resistant to weight gain, but exhibited insulin resistance on both normal chow and high-fat diets. These lipodystrophic mice also developed hypertension, cardiac hypertrophy, and cardiac dysfunction. Thus, isolated mitochondrial dysfunction in adipose tissue can lead a syndrome of lipodystrophy with metabolic syndrome and cardiovascular complications. © FASEB.

  20. Unusual Complications of Quinalphos Poisoning

    Directory of Open Access Journals (Sweden)

    Stalin Viswanathan

    2013-01-01

    Full Text Available This 40-year-old man was treated for suicidal quinalphos 25%EC consumption. He developed intermediate syndrome with normal response to repetitive nerve stimulation, pancreatitis with high enzyme elevations, and normal computed tomography and excreted black, brown, and orange urine sequentially over the first nine days of hospitalization. The last complication has not been previously reported with any organophosphate compound. He finally succumbed to complication of ventilator associated pneumonia related septic shock and ventricular tachycardia.

  1. Rituximab for nephrotic syndrome in children.

    Science.gov (United States)

    Iijima, Kazumoto; Sako, Mayumi; Nozu, Kandai

    2017-04-01

    Idiopathic nephrotic syndrome is the most common chronic glomerular disease in children. At least 20 % of children with this syndrome show frequent relapses and/or steroid dependence during or after immunosuppressive therapies, a condition defined as complicated frequently relapsing/steroid-dependent nephrotic syndrome (FRNS/SDNS). Approximately 1-3 % of children with idiopathic nephrotic syndrome are resistant to steroids and all immunosuppressive agents, a condition defined as refractory steroid-resistant nephrotic syndrome (SRNS); these SRNS children have a high risk of end-stage renal failure. Rituximab, a chimeric anti-CD20 monoclonal antibody, has been shown to be effective for patients with complicated FRNS/SDNS and refractory SRNS. This review describes the recent results of rituximab treatment applied to pediatric nephrotic syndrome, as well as those of our recent study, a multicenter, double-blind, randomized, placebo-controlled trial of rituximab for childhood-onset complicated FRNS/SDNS (RCRNS01). The overall efficacy and safety of rituximab for this disease are discussed.

  2. [Anemic syndrome frequency in complicated obstetrical patients].

    Science.gov (United States)

    Martínez, Maria Guadalupe Veloz; Erasto, Luis Cruz; Maxines, Claudia García; Rodríguez, María Antonia Basavilvazo; Valencia, Marcelino Hernández

    2008-09-01

    The prevalence of anemia varies from country to country and there is not a trustworthy record. To determine the frequency of anemia in obstetric patients and the association among healthy pregnancy and aggregate complications. Was carried out as transversal, observational and comparative study. Obstetrical patients entered and responded in the period of a year, were formed a group with normal pregnancy and another with complicated pregnancy, with a total sample of 194 patients. In the statistical analysis was employed Student t test for independent groups, with value if p anemia was found in 22.4%. Hematological stage from group with normal pregnancy was mild anemia in 16.9% and anemia moderated in 4.1% of the cases. The anemia degrees in the group with associated illness and pregnancy were mild anemia in 19.2% and moderated anemia in 4.2%. Not any case was found with severe anemia. The statistical analysis showed difference significant among both groups p preeclampsia severe (22.6%), type 2 diabetes (13.9%), gestational diabetes (12.2%) and the remainder with other complications that include to the hypertiroidism, rheumatoid arthritis, lupus, asthma and vein deep thrombosis. Frequency of anemia in this study was greater upon informing in the international literature. The obstetrical complication more frequently relates to diverse anemia degrees were the hypertensive stage during pregnancy. The anemia is presented with greater frequency in pregnancy patients with others associated illness.

  3. Compartment syndrome in a labrador retriever

    International Nuclear Information System (INIS)

    Williams, J.; Bailey, M.Q.; Schertel, E.R.; Valentine, A.

    1994-01-01

    Compartment syndrome is an elevation of interstitial pressure in a closed osseofascial compartment that results in microvascular compromise. This report documents a clinical syndrome in the crus of a fourteen-month-old intact male Labrador Retriever which was consistent with trauma-induced compartment syndrome. A six month history of recurring trauma or complications resulted in the need for referral. Survey radiography and ultrasonography aided in the diagnosis, but the definitive answer was provided by femoral angiography. The patient was successfully treated and was discharged with normal limb function. One year later, there were no complications observed. Compartment syndrome is not uncommon in humans, and is routinely considered in certain blunt and most penetrating traumas. However, few reports of this complication in animals are found

  4. Sinusoidal Obstruction Syndrome during Treatment for Wilms' Tumor: A Life-threatening Complication.

    Science.gov (United States)

    Totadri, Sidharth; Trehan, Amita; Bansal, Deepak; Jain, Richa

    2017-01-01

    Survival rates exceed 90% in Wilms' tumor (WT). Actinomycin-D (ACT-D) which is indispensable in the management of WT is associated with the development of sinusoidal obstruction syndrome (SOS), a potentially fatal complication. The aim is to study the presentation, management, and outcome of SOS complicating ACT-D administration in WT. Retrospective file review conducted in a Pediatric Hematology-Oncology unit. Patients diagnosed and treated for WT from January 2012 to December 2015 were analyzed. SOS was diagnosed clinically, based on McDonalds criteria, requiring two of the following: jaundice, hepatomegaly and/or right upper quadrant pain, weight gain with or without ascites. Of 104 patients treated, SOS occurred in 5 (4.8%). Age: 6 months to 5 years, 3 were girls. Tumor involved left kidney in 3, right in 1 and a horseshoe kidney in 1. Histopathology was consistent with WT in 4 and clear cell sarcoma kidney in 1. One had pulmonary metastases. Three developed SOS preoperatively and two during adjuvant chemotherapy. None received radiotherapy. Clinical manifestations comprised of jaundice, hepatomegaly, ascites/weight gain, respiratory distress, hypotension, and encephalopathy. Laboratory findings included thrombocytopenia, elevated serum transaminases, and coagulopathy. Treatment included fluid restriction, broad spectrum antibiotics, and transfusional support. Two children received N-acetyl cysteine infusion. Defibrotide was administered to two patients. Four recovered and one succumbed to multi-organ failure. Two patients were safely re-challenged with 50% doses of ACT-D. SOS is a clinical diagnosis. Systematic supportive care can enable complete recovery. Under close monitoring, re-challenge of ACT-D can be performed in gradually escalating doses.

  5. Pediatric Miller Fisher Syndrome Complicating an Epstein-Barr Virus Infection.

    Science.gov (United States)

    Communal, Céline; Filleron, Anne; Baron-Joly, Sandrine; Salet, Randa; Tran, Tu-Anh

    2016-10-01

    Miller Fisher syndrome, a variant of Guillain-Barré syndrome, is an acute inflammatory demyelinating polyradiculoneuropathy that may occur weeks after a bacterial or viral infection. Campylobacter jejuni and Haemophilus influenzae are frequently reported etiological agents. We describe a boy with Miller Fisher syndrome following Epstein-002DBarr virus primary infectious mononucleosis. He presented with bilateral dysfunction of several cranial nerves and hyporeflexia of the limbs but without ataxia. Miller Fisher syndrome was confirmed by the presence of anti-GQ1b antibodies in a blood sample. Epstein-Barr virus was identified by polymerase chain reaction and serology. Epstein-Barr virus should be considered as a Miller Fisher syndrome's causative agent. The physiopathology of this condition may involve cross-reactive T-cells against Epstein-Barr virus antigens and gangliosides. Copyright © 2016 Elsevier Inc. All rights reserved.

  6. Compartment Syndrome as a Result of Systemic Capillary Leak Syndrome

    Directory of Open Access Journals (Sweden)

    Kwadwo Kyeremanteng

    2016-01-01

    Full Text Available Objective. To describe a single case of Systemic Capillary Leak Syndrome (SCLS with a rare complication of compartment syndrome. Patient. Our patient is a 57-year-old male, referred to our hospital due to polycythemia (hemoglobin (Hgb of 220 g/L, hypotension, acute renal failure, and bilateral calf pain. Measurements and Main Results. The patient required bilateral forearm, thigh, and calf fasciotomies during his ICU stay and continuous renal replacement therapy was instituted following onset of acute renal failure and oliguria. Ongoing hemodynamic (Norepinephrine and Milrinone infusion and respiratory (ventilator support in the ICU was provided until resolution of intravascular fluid extravasation. Conclusions. SCLS is an extremely rare disorder characterized by unexplained episodic capillary hyperpermeability, which causes shift of volume and protein from the intravascular space to the interstitial space. Patients present with significant hypotension, hemoconcentration, hypovolemia, and oliguria. Severe edema results from leakage of fluid and proteins into tissue. The most important part of treatment is maintaining stable hemodynamics, ruling out other causes of shock and diligent monitoring for complications. Awareness of the clinical syndrome with the rare complication of compartment syndrome may help guide investigations and diagnoses of these critically ill patients.

  7. Clinical and radiographic outcome of a treat-to-target strategy using methotrexate and intra-articular glucocorticoids with or without adalimumab induction

    DEFF Research Database (Denmark)

    Hørslev-Petersen, K; Hetland, M L; Ørnbjerg, L M

    2015-01-01

    OBJECTIVES: To study clinical and radiographic outcomes after withdrawing 1 year's adalimumab induction therapy for early rheumatoid arthritis (eRA) added to a methotrexate and intra-articular triamcinolone hexacetonide treat-to-target strategy (NCT00660647). METHODS: Disease-modifying antirheuma......OBJECTIVES: To study clinical and radiographic outcomes after withdrawing 1 year's adalimumab induction therapy for early rheumatoid arthritis (eRA) added to a methotrexate and intra-articular triamcinolone hexacetonide treat-to-target strategy (NCT00660647). METHODS: Disease.......12). Erosive progression (Δerosion score (ES)/year) was year 1:0.57/0.06 (p=0.02); year 2:0.38/0.05 (p=0.005). Proportion of patients without erosive progression (ΔES≤0) was year 1: 59%/76% (p=0.03); year 2:64%/79% (p=0.04). CONCLUSIONS: An aggressive triamcinolone and synthetic DMARD treat-to-target strategy...... was (re)initiated in 12/12 patients and cumulative triamcinolone dose was 160/120 mg (p=0.15). The treatment target (disease activity score, 4 variables, C-reactive protein (DAS28CRP) ≤3.2 or DAS28>3.2 without swollen joints) was achieved at all visits in ≥85% of patients in year 2; remission rates were...

  8. Comparative effectiveness and survival of infliximab, adalimumab, and etanercept for rheumatoid arthritis patients in the Hellenic Registry of Biologics: Low rates of remission and 5-year drug survival.

    Science.gov (United States)

    Flouri, Irini; Markatseli, Theodora E; Voulgari, Paraskevi V; Boki, Kyriaki A; Papadopoulos, Ioannis; Settas, Loukas; Zisopoulos, Dimitrios; Skopouli, Fotini N; Iliopoulos, Alexios; Bertsias, George K; Geborek, Pierre; Drosos, Alexandros A; Boumpas, Dimitrios T; Sidiropoulos, Prodromos

    2014-02-01

    To compare effectiveness, drug survival, and safety between infliximab, adalimumab, and etanercept, in a nationwide cohort of rheumatoid arthritis (RA) patients. This study is a prospective cohort study of 1208 active RA patients. Effectiveness, drug survival, and serious adverse events during entire follow-up (median 2.9 years) were monitored. EULAR and CDAI responses were comparable between the three agents (EULAR good/moderate responses at 12 months ranged 76-79%). At 12 months, 15-23% achieved remission. For adalimumab and etanercept, adjusted hazard rate (HR) for EULAR/ACR remission (reference: infliximab) was 2.7 and 2.1 (95% confidence interval was 1.7-4.1 and 1.3-3.4, respectively); males (HR 1.6; 1.1-2.4), use of glucocorticoids (HR 2.0; 1.3-3.0), and swollen joint count >7 (HR 0.36; 0.24-0.55) were independent predictors. Five-year drug survival was 31%, 43%, and 49% for infliximab, adalimumab, and etanercept, respectively (p = 0.010). Infliximab was associated with significantly more withdrawals due to adverse events. Disease activity, CRP, and use of glucocorticoids predicted efficacy-related drug survival; age, use of methotrexate, and prior DMARDs failures predicted safety-related survival. Risk for serious infections was lower with adalimumab (odds ratio [OR] 0.62; 0.38-1.00) or etanercept (OR 0.39; 0.21-0.72) than infliximab, independent of the effects of age (OR 1.65; 1.37-2.00 per 10 years), tender joint count >10 (OR 1.86; 1.21-2.86), and glucocorticoids >35mg/week (OR 1.83; 1.12-2.99). Response rates were comparable among anti-TNF agents. Overall, 5-year drug survival was below 50%, with infliximab demonstrating increased safety-related discontinuations. Remission rates are low in clinical practice. Strategies to increase effectiveness and long-term survival of anti-TNF agents in RA are needed. Copyright © 2014 Elsevier Inc. All rights reserved.

  9. Ulnar nerve entrapment complicating radial head excision

    Directory of Open Access Journals (Sweden)

    Kevin Parfait Bienvenu Bouhelo-Pam

    Full Text Available Introduction: Several mechanisms are involved in ischemia or mechanical compression of ulnar nerve at the elbow. Presentation of case: We hereby present the case of a road accident victim, who received a radial head excision for an isolated fracture of the radial head and complicated by onset of cubital tunnel syndrome. This outcome could be the consequence of an iatrogenic valgus of the elbow due to excision of the radial head. Hitherto the surgical treatment of choice it is gradually been abandoned due to development of radial head implant arthroplasty. However, this management option is still being performed in some rural centers with low resources. Discussion: The radial head plays an important role in the stability of the elbow and his iatrogenic deformity can be complicated by cubital tunnel syndrome. Conclusion: An ulnar nerve release was performed with favorable outcome. Keywords: Cubital tunnel syndrome, Peripheral nerve palsy, Radial head excision, Elbow valgus

  10. Prader-Willi Syndrome

    Science.gov (United States)

    ... syndrome can be helpful in genetic counseling. Complications Obesity-related complications In addition to having constant hunger, ... with this disorder are unable to have children. Osteoporosis. Osteoporosis causes bones to become weak and brittle, ...

  11. [Late respiratory function complications following burns].

    Science.gov (United States)

    Ernesto, S; Marduel, Y; Freymond, N; Pacheco, Y; Devouassoux, G

    2008-03-01

    Twenty five per cent of thermal injuries are associated with secondary respiratory events linked to several mechanisms. In the acute phase of the accident oedema of the airways, the fume inhalation syndrome and ARDS are the most common causes responsible for death in 60% of cases. Late respiratory complications are little known and neglected. They comprise obstructive ventilatory defects due to the inhalation syndrome and restrictive defects secondary to ARDS or to dermal injury. We report the case of a female patient, extensively burnt 2 years previously, admitted to hospital with severe acute respiratory failure complicating COPD. The presence of both restrictive and obstructive defects led to the suggestion of alternative underlying mechanisms such as the pulmonary consequences of ARDS and extensive dermal scars. The latter were responsible for an armour like thickening of the skin of the thorax compatible with the restrictive defect. These functional abnormalities and the potential severity of acute respiratory failure are indications for regular pulmonary follow-up of patients with severe circumferential scarring of the thorax who are at high risk for respiratory complications.

  12. Pheochromocytoma presenting as an acute coronary syndrome complicated by acute heart failure: The challenge of a great mimic.

    Science.gov (United States)

    Sanna, Giuseppe Damiano; Talanas, Giuseppe; Fiore, Giuseppina; Canu, Antonella; Terrosu, Pierfranco

    2016-10-01

    Pheochromocytoma is a rare neuroendocrine tumor with a highly variable clinical presentation. The serious and potentially lethal cardiovascular complications of these tumors are related to the effects of secreted catecholamines. We describe a case of a 50-year-old woman urgently admitted to our hospital because of symptoms and clinical and instrumental findings consistent with an acute coronary syndrome complicated by acute heart failure. Urgent coronary angiography showed normal coronary arteries. During her hospital stay, the recurrence of episodes characterized by a sudden increase in blood pressure, cold sweating, and nausea allowed us to hypothesize a pheochromocytoma. The diagnosis was confirmed by elevated levels of urinary catecholamines and by the finding of a left adrenal mass on magnetic resonance imaging. The patient underwent left adrenalectomy. Therefore, the initial diagnosis was critically reappraised and reviewed as a cardiac manifestation of a pheochromocytoma during catecholaminergic crisis.

  13. Complications of Diabetes and Their Implications for Service Providers.

    Science.gov (United States)

    Ponchillia, S. V.

    1993-01-01

    This article presents information on the complications of both Type I and Type II diabetes and the implications for the rehabilitation of persons with diabetes and visual impairment. Topics covered include retinopathy, cataracts, glaucoma, peripheral neuropathy, carpal tunnel syndrome, diabetic hand syndrome, neuropathy of the autonomic nervous…

  14. Open-label study of etanercept treatment in patients with moderate-to-severe plaque psoriasis who lost a satisfactory response to adalimumab.

    Science.gov (United States)

    Bagel, J; Tyring, S; Rice, K C; Collier, D H; Kricorian, G; Chung, J; Iles, J; Stolshek, B S; Kaliyaperumal, A; Papp, K A

    2017-08-01

    Some patients with plaque psoriasis experience secondary failure of tumour necrosis factor inhibitor therapy. To evaluate efficacy, safety and patient-reported outcomes (PROs) with etanercept in patients with secondary adalimumab failure. This phase IV open-label single-arm estimation study (NCT01543204) enrolled patients on adalimumab who had achieved static Physician's Global Assessment (sPGA) score 0/1 (clear/almost clear). Patients subsequently lost response, defined as sPGA ≥ 3 or loss of 50% improvement in Psoriasis Area and Severity Index (PASI 50). At baseline, patients had involved body surface area ≥ 10%, sPGA ≥ 3 and PASI ≥ 10. Antiadalimumab antibodies (ADAs) were measured at screening. Patients received etanercept 50 mg twice weekly for 12 weeks, followed by 50 mg weekly. The primary end point was sPGA 0/1 at week 12 (intention-to-treat analysis; no hypothesis tested). Additional outcomes included rates of sPGA 0/1, PASI responses, safety, PROs of itch, pain and flaking, Dermatology Life Quality Index, treatment satisfaction and Work Productivity and Activity Impairment questionnaire. Sixty-four patients enrolled; 67% had ADAs. sPGA 0/1 rates at week 12 were 39·7% [95% confidence interval (CI) 27·6-52·8; primary end point] and 45% (95% CI 29·3-61·5) for patients positive for ADAs and 35% (95% CI 15·4-59·2) for patients negative for ADAs. PASI 75 response rates at week 12 were 47·5% (95% CI 31·5-63·9) for patients who were positive for ADAs and 50% (95% CI 27·2-72·8) for patients negative for ADAs. No new safety signals were observed. PROs of itch, pain and flaking consistently improved at week 12 and were maintained through week 24. Patients with psoriasis who experienced secondary failure of adalimumab achieved satisfactory response to etanercept regardless of ADA status. © 2016 The Authors. British Journal of Dermatology published by John Wiley & Sons Ltd on behalf of British Association of Dermatologists.

  15. Imaging of postpartum complications

    International Nuclear Information System (INIS)

    Rooholamini, S.A.; Au, A.H.; Hansen, G.C.; Kioumehr, F.; Yaghmai, I.

    1991-01-01

    The purpose of this paper is to evaluate the efficacy of various imaging modalities in the detection of postpartum complications. Nineteen cases of postpartum complications, studied radiologically, form the basis of this exhibit. Plain abdominal radiography, US, CT, and MR imaging were performed alone or in combination. The abnormalities detected included uterine rupture, intramural uterine hematoma, hematomas of the broad ligaments and the abdominal rectus muscles, ovarian vein thrombosis, HELLP syndrome, pyometrium, retained placenta and blood clots, periappendiceal abscess, superior sagittal sinus thrombosis, and cerebral edema. Representative cases will be illustrated; and the effective value of each technique will be stressed

  16. Extracorporeal Membrane Oxygenation for Complicated Scrub Typhus

    Directory of Open Access Journals (Sweden)

    Eun Sun Kim

    Full Text Available Scrub typhus is a mite-borne infectious disease caused by Orientia tsutsugamushi . Although early diagnosis and appropriate antibiotic therapy improve the prognosis for the majority of patients, life-threatening complications are not uncommon. Here, we present a case of successful veno-veno-type extracorporeal membrane oxygenation for scrub typhus-induced complications, including acute respiratory distress syndrome, myocarditis and multi-organ dysfunction. To our knowledge, this is the first case report of successful extracorporeal membrane oxygenation in complicated scrub typhus in Korea.

  17. Ophthalmologic complications of systemic disease.

    Science.gov (United States)

    Klig, Jean E

    2008-02-01

    The human eye, as an organ, can offer critical clues to the presence of systemic disease. This article discusses the various ophthalmologic manifestations of systemic disease that can be evident on examination by an emergency department provider, as well as some findings that can be discerned with specialty consultation. The following topics are reviewed with respect to potential ocular signs and complications: syphilis, herpes zoster, Lyme disease, acquired immunodeficiency syndrome, Reiter's syndrome, Kawasaki's disease, temporal arteritis, endocarditis, hypertension, and diabetes mellitus. Indications for emergent ophthalmologic consultation are also emphasized.

  18. Algorithms to identify colonic ischemia, complications of constipation and irritable bowel syndrome in medical claims data: development and validation.

    Science.gov (United States)

    Sands, Bruce E; Duh, Mei-Sheng; Cali, Clorinda; Ajene, Anuli; Bohn, Rhonda L; Miller, David; Cole, J Alexander; Cook, Suzanne F; Walker, Alexander M

    2006-01-01

    A challenge in the use of insurance claims databases for epidemiologic research is accurate identification and verification of medical conditions. This report describes the development and validation of claims-based algorithms to identify colonic ischemia, hospitalized complications of constipation, and irritable bowel syndrome (IBS). From the research claims databases of a large healthcare company, we selected at random 120 potential cases of IBS and 59 potential cases each of colonic ischemia and hospitalized complications of constipation. We sought the written medical records and were able to abstract 107, 57, and 51 records, respectively. We established a 'true' case status for each subject by applying standard clinical criteria to the available chart data. Comparing the insurance claims histories to the assigned case status, we iteratively developed, tested, and refined claims-based algorithms that would capture the diagnoses obtained from the medical records. We set goals of high specificity for colonic ischemia and hospitalized complications of constipation, and high sensitivity for IBS. The resulting algorithms substantially improved on the accuracy achievable from a naïve acceptance of the diagnostic codes attached to insurance claims. The specificities for colonic ischemia and serious complications of constipation were 87.2 and 92.7%, respectively, and the sensitivity for IBS was 98.9%. U.S. commercial insurance claims data appear to be usable for the study of colonic ischemia, IBS, and serious complications of constipation. (c) 2005 John Wiley & Sons, Ltd.

  19. Sinusoidal Obstruction Syndrome during Treatment for Wilms' Tumor: A Life-threatening Complication

    Science.gov (United States)

    Totadri, Sidharth; Trehan, Amita; Bansal, Deepak; Jain, Richa

    2017-01-01

    Context: Survival rates exceed 90% in Wilms' tumor (WT). Actinomycin-D (ACT-D) which is indispensable in the management of WT is associated with the development of sinusoidal obstruction syndrome (SOS), a potentially fatal complication. Aims: The aim is to study the presentation, management, and outcome of SOS complicating ACT-D administration in WT. Settings and Design: Retrospective file review conducted in a Pediatric Hematology-Oncology unit. Materials and Methods: Patients diagnosed and treated for WT from January 2012 to December 2015 were analyzed. SOS was diagnosed clinically, based on McDonalds criteria, requiring two of the following: jaundice, hepatomegaly and/or right upper quadrant pain, weight gain with or without ascites. Results: Of 104 patients treated, SOS occurred in 5 (4.8%). Age: 6 months to 5 years, 3 were girls. Tumor involved left kidney in 3, right in 1 and a horseshoe kidney in 1. Histopathology was consistent with WT in 4 and clear cell sarcoma kidney in 1. One had pulmonary metastases. Three developed SOS preoperatively and two during adjuvant chemotherapy. None received radiotherapy. Clinical manifestations comprised of jaundice, hepatomegaly, ascites/weight gain, respiratory distress, hypotension, and encephalopathy. Laboratory findings included thrombocytopenia, elevated serum transaminases, and coagulopathy. Treatment included fluid restriction, broad spectrum antibiotics, and transfusional support. Two children received N-acetyl cysteine infusion. Defibrotide was administered to two patients. Four recovered and one succumbed to multi-organ failure. Two patients were safely re-challenged with 50% doses of ACT-D. Conclusions: SOS is a clinical diagnosis. Systematic supportive care can enable complete recovery. Under close monitoring, re-challenge of ACT-D can be performed in gradually escalating doses. PMID:29333010

  20. Complications and challenges associated with polycystic ovary syndrome: current perspectives

    Science.gov (United States)

    Palomba, Stefano; Santagni, Susanna; Falbo, Angela; La Sala, Giovanni Battista

    2015-01-01

    Polycystic ovary syndrome (PCOS) represents the most common endocrine dysfunction in fertile women and it is considered a heterogeneous and multifaceted disorder, with multiple reproductive and metabolic phenotypes which differently affect the early- and long-term syndrome’s risks. Women with PCOS present an adverse reproductive profile, including a high risk of pregnancy-induced hypertension, preeclampsia, and gestational diabetes mellitus. Patients with PCOS present not only a higher prevalence of classic cardiovascular risk factors, such as hypertension, dyslipidemia, and type-2 diabetes mellitus, but also of nonclassic cardiovascular risk factors, including mood disorders, such as depression and anxiety. Moreover, at the moment, clinical data on cardiovascular morbidity and mortality in women with PCOS are controversial. Finally, women with PCOS show an increased risk of endometrial cancer compared to non-PCOS healthy women, particularly during premenopausal period. Currently, we are unable to clarify if the increased PCOS early- and long-term risks are totally due to PCOS per se or mostly due to obesity, in particular visceral obesity, that characterized the majority of PCOS patients. In any case, the main endocrine and gynecological scientific societies agree to consider women with PCOS at increased risk of obstetric, cardiometabolic, oncology, and psychological complications throughout life, and it is recommended that these women be accurately assessed with periodic follow-up. PMID:26261426

  1. Occam paradox? A variation of tapia syndrome and an unreported complication of guidewire-assisted pedicle screw insertion.

    Science.gov (United States)

    Emohare, Osa; Peterson, Erik; Slinkard, Nathaniel; Janus, Seth; Morgan, Robert

    2013-10-01

    Study Design Case report. Clinical Question The clinical aim is to report on a previously unknown association between guidewire-assisted pedicle screw insertion and neuropraxia of the recurrent laryngeal nerve (RLN), and how this may overlap with the signs of Tapia syndrome; we also report our approach to the clinical management of this patient. Methods A 17-year-old male patient with idiopathic scoliosis experienced Tapia syndrome after posterior instrumentation and arthrodesis at the level of T1-L1. After extubation, the patient had a hoarse voice and difficulty in swallowing. Imaging showed a breach in the cortex of the anterior body of T1 corresponding to the RLN on the right. Results Otolaryngological examination noted right vocal fold immobility, decreased sensation of the endolarynx, and pooling of secretions on flexible laryngoscopy that indicated right-sided cranial nerve X injury and left-sided tongue deviation. Aspiration during a modified barium swallow prompted insertion of a percutaneous endoscopic gastrostomy tube before the patient was sent home. On postoperative day 20, a barium swallow demonstrated reduced aspiration, and the patient reported complete resolution of symptoms. The feeding tube was removed, and the patient resumed a normal diet 1 month later. Tapia syndrome, or persistent unilateral laryngeal and hypoglossal paralysis, is an uncommon neuropraxia, which has previously not been observed in association with a breached vertebral body at T1 along the course of the RLN. Conclusion Tapia syndrome should be a differential diagnostic consideration whenever these symptoms persist postoperatively and spine surgeons should be aware of this as a potential complication of guidewires in spinal instrumentation.

  2. Chondroectodermal dysplasia: a rare syndrome.

    Directory of Open Access Journals (Sweden)

    Dana Tahririan

    2014-06-01

    Full Text Available Chondroectodermal dysplasia (Ellis-Van Creveld syndrome is a rare autosomal recessive congenital abnormality. This syndrome is characterized by a spectrum of clinical findings, among which chondrodystrophy, polydactyly, ectodermal dysplasia, and congenital cardiac anomalies are the most common. It is imperative to not overlook the cardiac complications in patients with this syndrome during dental procedures. The case presented here, although quite rare, was detected under normal conditions and can be alarming for dental care providers. Clinical reports outline the classical and unusual oral and dental manifestations, which help health care providers diagnose chondroectodermal dysplasia, and refer patients with this syndrome to appropriate health care professionals to receive treatment to prevent further cardiac complications and bone deformities.

  3. Adalimumab for the treatment of refractory noninfectious paediatric uveitis.

    Science.gov (United States)

    Muñoz-Gallego, Alicia; Barral, Estefanía; Enríquez, Eugenia; Tejada, Pilar; Barceló, Ana; de Inocencio, Jaime

    2017-06-01

    To report the experience of our center with the use of adalimumab (ADA) for the treatment of severe refractory noninfectious paediatric uveitis. The study is a retrospective case series of all paediatric patients with refractory uveitis who were treated with ADA at the Paediatric Uveitis Unit of our center from 2008 to 2015. We present 12 patients (6 Juvenile idiopathic arthritis-associated uveitis, 4 idiopathic panuveitis, 1 early-onset sarcoidosis-associated panuveitis, and 1 intermediate uveitis), with uveitis in 19/24 eyes. Once ADA therapy was started, all the patients presented improved activity according to Standardization of Uveitis Nomenclature (SUN) criteria. Nine out of the 12 patients had structural damage before ADA could be started: cataract (n = 4), glaucoma (n = 2), cystic macular edema (n = 1), exudative retinal detachment (n = 1), and optic disk edema (n = 5). Visual acuity improved or maintained stable in 17/19 affected eyes, and only 2 eyes decreased its visual acuity because of structural damage, which was already present before ADA therapy. In our experience, ADA presents a good safety profile and is efficacious in the treatment of paediatric patients with different forms of refractory noninfectious uveitis.

  4. Uveitis Events During Adalimumab, Etanercept, and Methotrexate Therapy in Juvenile Idiopathic Arthritis: Data From the Biologics in Pediatric Rheumatology Registry.

    Science.gov (United States)

    Foeldvari, Ivan; Becker, Ingrid; Horneff, Gerd

    2015-11-01

    Uveitis is a major extraarticular quality of life-restricting manifestation of juvenile idiopathic arthritis (JIA). The aim of the study is to describe the occurrence of uveitis in JIA patients receiving tumor necrosis factor inhibitors or methotrexate (MTX). Patients' characteristics, treatment, and the reported first occurrence of uveitis as an adverse event were searched in the Biologics in Pediatric Rheumatology Registry. The rates per exposed patients, exposure time, and time until event were calculated. Uveitis was reported as an adverse event in 75 of 3,467 patients; 51 of 2,844 patients were receiving MTX, 37 of 1,700 patients were receiving etanercept, and 13 of 364 patients were receiving adalimumab. Patients with uveitis were younger (mean ± SD age 4.6 ± 4.2 versus 7.4 ± 4.5 years; P uveitis diagnosis before starting treatment more often had a uveitis event (n = 28, 8.4%; OR 8.5, P uveitis event occurred: 11 while taking MTX (3.2 per 1,000 patient-years), 2 while taking etanercept monotherapy (1.9 per 1,000 patient-years), and 3 while taking etanercept and MTX combination (0.9 per 1,000 patient-years). A new uveitis event occurred early in the disease course after a median disease duration of 1.5 years (interquartile range [IQR] 1.3-3.8) while taking etanercept and 1.8 years (IQR 1.8-2.1) for the MTX cohort. A recurrent uveitis event was reported after a disease duration of 7.6 years (IQR 4.3-10.0) in the etanercept cohort and 4.8 years (IQR 1.0-5.8) in the MTX cohort. Univariate analysis showed that MTX, but not etanercept or adalimumab, led to a lower rate of uveitis. Patients with a history of uveitis had higher risks for uveitis events while taking both etanercept and adalimumab. Methotrexate turned out to be protective. Few patients developed a first uveitis event while taking etanercept, while the rate is comparable to that with MTX. Uveitis may not be attributed to be an adverse drug reaction to etanercept. © 2015, American

  5. Adalimumab, etanercept and ustekinumab for treating plaque psoriasis in children and young people: systematic review and economic evaluation.

    Science.gov (United States)

    Duarte, Ana; Mebrahtu, Teumzghi; Goncalves, Pedro Saramago; Harden, Melissa; Murphy, Ruth; Palmer, Stephen; Woolacott, Nerys; Rodgers, Mark; Rothery, Claire

    2017-11-01

    Psoriasis is a chronic inflammatory disease that predominantly affects the skin. Adalimumab (HUMIRA ® , AbbVie, Maidenhead, UK), etanercept (Enbrel ® , Pfizer, New York, NY, USA) and ustekinumab (STELARA ® , Janssen Biotech, Inc., Titusville, NJ, USA) are the three biological treatments currently licensed for psoriasis in children. To determine the clinical effectiveness and cost-effectiveness of adalimumab, etanercept and ustekinumab within their respective licensed indications for the treatment of plaque psoriasis in children and young people. Searches of the literature and regulatory sources, contact with European psoriasis registries, company submissions and clinical study reports from manufacturers, and previous National Institute for Health and Care Excellence (NICE) technology appraisal documentation. Included studies were summarised and subjected to detailed critical appraisal. A network meta-analysis incorporating adult data was developed to connect the effectiveness data in children and young people and populate a de novo decision-analytic model. The model estimated the cost-effectiveness of adalimumab, etanercept and ustekinumab compared with each other and with either methotrexate or best supportive care (BSC), depending on the position of the intervention in the management pathway. Of the 2386 non-duplicate records identified, nine studies (one randomised controlled trial for each drug plus six observational studies) were included in the review of clinical effectiveness and safety. Etanercept and ustekinumab resulted in significantly greater improvements in psoriasis symptoms than placebo at 12 weeks' follow-up. The magnitude and persistence of the effects beyond 12 weeks is less certain. Adalimumab resulted in significantly greater improvements in psoriasis symptoms than methotrexate for some but not all measures at 16 weeks. Quality-of-life benefits were inconsistent across different measures. There was limited evidence of excess short

  6. Lemierre's syndrome

    DEFF Research Database (Denmark)

    Johannesen, Katrine M; Bodtger, Uffe

    2016-01-01

    This is a systematic review of cases with Lemierre's syndrome (LS) in the past 5 years. LS is characterized by sepsis often evolving after a sore throat or tonsillitis and then complicated by various septic emboli and thrombosis of the internal jugular vein. Symptoms include sepsis, pain, and/or ...... LS in this day and age appears to be low, however the syndrome is difficult to recognize, and still requires the full attention of the clinician.......This is a systematic review of cases with Lemierre's syndrome (LS) in the past 5 years. LS is characterized by sepsis often evolving after a sore throat or tonsillitis and then complicated by various septic emboli and thrombosis of the internal jugular vein. Symptoms include sepsis, pain, and....../or swelling in the throat or neck, as well as respiratory symptoms. Laboratory findings show elevated infectious parameters and radiological findings show thrombosis of the internal jugular vein and emboli in the lungs or other organs. The syndrome is often associated with an infection with Fusobacterium...

  7. Diagnostic Overlap between Fanconi Anemia and the Cohesinopathies: Roberts Syndrome and Warsaw Breakage Syndrome

    Directory of Open Access Journals (Sweden)

    Petra van der Lelij

    2010-01-01

    Full Text Available Fanconi anemia (FA is a recessively inherited disease characterized by multiple symptoms including growth retardation, skeletal abnormalities, and bone marrow failure. The FA diagnosis is complicated due to the fact that the clinical manifestations are both diverse and variable. A chromosomal breakage test using a DNA cross-linking agent, in which cells from an FA patient typically exhibit an extraordinarily sensitive response, has been considered the gold standard for the ultimate diagnosis of FA. In the majority of FA patients the test results are unambiguous, although in some cases the presence of hematopoietic mosaicism may complicate interpretation of the data. However, some diagnostic overlap with other syndromes has previously been noted in cases with Nijmegen breakage syndrome. Here we present results showing that misdiagnosis may also occur with patients suffering from two of the three currently known cohesinopathies, that is, Roberts syndrome (RBS and Warsaw breakage syndrome (WABS. This complication may be avoided by scoring metaphase chromosomes—in addition to chromosomal breakage—for spontaneously occurring premature centromere division, which is characteristic for RBS and WABS, but not for FA.

  8. Refeeding syndrome: a clinical review.

    Science.gov (United States)

    Ormerod, Clare; Farrer, Kirstine; Harper, Lindsay; Lal, Simon

    2010-12-01

    Refeeding syndrome can result in a wide variety of complications and may be life threatening. Although well described in hospital practice, refeeding syndrome is often under-recognized and inadequately treated.

  9. Economic impact of adalimumab treatment in Japanese patients with rheumatoid arthritis from the adalimumab non-interventional trial for up-verified effects and utility (ANOUVEAU) study.

    Science.gov (United States)

    Tanaka, Yoshiya; Yamazaki, Kiyotaka; Nakajima, Ryo; Komatsu, Shuichi; Igarashi, Ataru; Tango, Toshiro; Takeuchi, Tsutomu

    2018-01-01

    The objective of this study is to evaluate the economic impact of adalimumab (ADA) on Japanese rheumatoid arthritis (RA) patients. ANOUVEAU was a 48-week multicenter, prospective, observational, single-cohort study. Work-related outcomes including absenteeism, presenteeism, overall work impairment (OWI), and activity impairment (AI) were evaluated using the RA-related work productivity and activity impairment (WPAI/RA). The amount of productivity loss was estimated via multiplication of absenteeism, presenteeism and OWI by the national average occupational wage for paid worker (PW) and part time worker (PTW), and via multiplication of AI by the estimated wage for domestic work for home maker (HM). In this analysis, 1196 patients were included. At week 48, measures of productivity loss due to absenteeism, presenteeism, OWI, and AI were significantly improved by administrating ADA to RA patients in all employment types (PW, PTW, and HM), compared to baseline (p < .01). Productivity loss of Japanese society by RA disease was estimated to be $9.80 billion. The annual decrease in productivity loss through ADA administration to Japanese RA patients was estimated to be $3.76 billion. The socioeconomic burden of RA is high, but ADA treatment may reduce productivity loss related to RA.

  10. The obstetric antiphospholipid syndrome

    NARCIS (Netherlands)

    Derksen, R. H. W. M.; de Grootb, Ph. G.

    The association of persistent presence of circulating antiphospholipid antibodies and thromboembolic events, (recurrent) pregnancy loss or both is termed antiphospholipid syndrome. Pregnancies in women with the syndrome should be regarded as at high-risk for complications. Optimal management

  11. The Marfan syndrome genetics

    Directory of Open Access Journals (Sweden)

    Galina Pungerčič

    2005-05-01

    Full Text Available Background: The Marfan syndrome is an autosomal dominant heritable disorder of connective tissue. It is caused by mutations in the fibrillin-1 gene encoding glycoprotein fibrillin-1, a component of microfibrils of extracellular matrix. Patients with Marfan syndrome show wide spectra of clinical signs, primarily on skeletal, cardiovascular and ocular organ systems. Cardiovascular complications (especially aortic aneurysm and aortic dissection are the most common cause of mortality of Marfan syndrome patients. Discovering genotype-phenotype correlations is complicated because of the large number of mutations reported as well as clinical heterogeneity among individuals with the same mutation. Despite the progress in the knowledge of the molecular nature of Marfan syndrome the diagnosis is still based mainly on the clinical features in the different body systems.Conclusions: Early identification of patient with Marfan syndrome is of considerable importance because of appropriate treatment that can greatly improve life expectancy. Unfortunately, despite the improvement of diagnostic methods, medical and surgical therapy, the mortality due to undiagnosed Marfan syndrome is still high. The present article reviews the molecular genetic studies of Marfan syndrome since the discovery of the mutations in the fibrillin-1 gene.

  12. Spontaneous Splenic Rupture in Vascular Ehlers-Danlos Syndrome.

    Science.gov (United States)

    Batagini, Nayara Cioffi; Gornik, Heather; Kirksey, Lee

    2015-01-01

    Vascular Ehlers-Danlos Syndrome (VEDS) is a rare autosomal dominant collagen vascular disorder. Different from other Ehler-Danlos Syndrome subtypes, VEDS has poor prognosis due to severe fragility of connective tissues and association with life-threatening vascular and gastrointestinal complications. Spontaneous splenic rupture is a rare but hazardous complication related to this syndrome. To date, only 2 cases have been reported in the literature. Here we present another case of this uncommon complication, occurring in a 54-year-old woman in clinical follow-up for VEDS who presented with sudden onset of abdominal pain and hypotension. © The Author(s) 2015.

  13. Oral manifestations associated with systemic complications of prune belly syndrome.

    Science.gov (United States)

    Pessoa, Larissa; Galvão, Virgilio

    2013-01-01

    Prune belly syndrome (PBS) is a rare congenital malformation of unknown etiology characterized by congenital abnormalities including abdominal wall flaccidity, urinary tract alterations, and bilateral cryptorchidism. The incidence of the syndrome is between 1/35000 and 1/50000 live births and there is little information about the oral findings. The present case describes the oral manifestations of a 15-year-old boy diagnosed with PBS. The findings include enamel hypoplasia associated with generalized hypocalcemic dental lines. In the radiographic exam, pronounced demineralization of the trabecular bone of the jaws, loss of lamina dura in all the teeth, and discontinuity of the mandibular cortical bone were observed. Prune belly syndrome is a rare disease, whose clinical dental aspects are not pathognomonic of the syndrome. The comprehension of the systemic mechanism of PBS and its comorbidities enable an understanding of the systemic etiologic factors associated with oral manifestations. Copyright © 2013 Elsevier Inc. All rights reserved.

  14. Hamman’s syndrome in diabetic ketoacidosis

    Directory of Open Access Journals (Sweden)

    Alexandra Rose Pain

    2017-11-01

    Full Text Available Hamman’s syndrome (spontaneous subcutaneous emphysema and pneumomediastinum is a rare complication of diabetic ketoacidosis (DKA, with a multifactorial etiology. Awareness of this syndrome is important: it is likely underdiagnosed as the main symptom of shortness of breath is often attributed to Kussmaul’s breathing and the findings on chest radiograph can be subtle and easily missed. It is also important to be aware of and consider Boerhaave’s syndrome as a differential diagnosis, a more serious condition with a 40% mortality rate when diagnosis is delayed. We present a case of pneumomediastinum, pneumopericardium, epidural emphysema and subcutaneous emphysema complicating DKA in an eighteen-year-old patient. We hope that increasing awareness of Hamman’s syndrome, and how to distinguish it from Boerhaave’s syndrome, will lead to better recognition and management of these syndromes in patients with diabetic ketoacidosis.

  15. Severe acute respiratory syndrome (SARS)

    Science.gov (United States)

    SARS; Respiratory failure - SARS ... Complications may include: Respiratory failure Liver failure Heart failure ... 366. McIntosh K, Perlman S. Coronaviruses, including severe acute respiratory syndrome (SARS) and Middle East respiratory syndrome (MERS). ...

  16. Neurological Complications in Child with Chronic Renal Failure

    Directory of Open Access Journals (Sweden)

    Faruk Incecik

    2003-08-01

    Full Text Available Congenital uremic encephalopathy, progressive dialysis encephalopathy, Wernicke encefalopathy, headache, seizures because of dialysis, disequilibrium syndrome, cerebral hemorrhage and uremic neuropathy are the neurologic complications seen in child with chronic renal failure. Here it is aimed to discuss these complications with literature, and to emphasize the importance of evaluation of patients with these aspects. [Archives Medical Review Journal 2003; 12(4.000: 406-412

  17. Tetrabenazine-induced oculogyric crisis – a rare complication in the treatment of Gilles de la Tourette syndrome

    Directory of Open Access Journals (Sweden)

    Janik P

    2016-02-01

    Full Text Available Piotr Janik,1 Monika Figura1,2 1Department of Neurology, Anna Gostynska Wolski Hospital, 2Department of Neurology, Faculty of Health Sciences, Medical University of Warsaw, Warsaw, Poland Abstract: Tetrabenazine is used in the treatment of chorea, tardive dyskinesia, tics, and dystonia. It rarely causes acute eyeball dystonia and the description of this complication in Gilles de la Tourette syndrome is limited. We provide a description of an acute oculogyric crisis caused by tetrabenazine in a patient with severe tics. The patient had never developed acute dystonic reactions, although he was previously exposed to numerous dopamine receptor-blocking agents. After 8 days of therapy with tetrabenazine at a dose of 62.5 mg daily, the patient developed involuntary movement of the eyeballs. Withdrawal of tetrabenazine caused resolution of all symptoms after a week. The purpose of this description is to draw attention to the potential of tetrabenazine to induce acute oculogyric crisis as well as the difficulty of differentiating drug-induced dystonia from dystonic tics in patients with Gilles de la Tourette syndrome. Keywords: acute dyskinesia, dystonic tics, eyeball dystonia, drug-induced dystonia, tic disorder, tetrabenazine-induced side effects

  18. Indicators of complicated mild TBI predict MMPI-2 scores after 23 years.

    Science.gov (United States)

    Hessen, Erik; Nestvold, Knut

    2009-03-01

    Research suggests that post-concussive syndrome may become persistent after mild traumatic brain injury (mTBI). The aim of this study was to investigate determinants of subjective complaints, characteristic for post-concussive syndrome, 23 years after mTBI. The study was a follow-up after a prospective head injury study at a general hospital in Norway. Ninety-seven patients were assessed with the Minnesota Multiphasic Personality Inventory-2 (MMPI-2) 23 years after sustaining primarily mTBI. A good overall outcome was found with scores close to the normative mean, average length of education and normal employment rate. However, the patients that sustained complicated mTBI showed somewhat more pathological scores, well-matched with mild post-concussive syndrome. The most important predictors of poor outcome were a combination of post-traumatic amnesia >30 minutes and EEG pathology within 24 hours after TBI. No influence of pre- and post-injury risk factors on current MMPI-2 profiles was found. The results are in line with previous research findings and support the notion of potentially differential impact of uncomplicated vs. complicated mTBI. The findings suggest that complicated mTBI may cause subtle chronic symptoms typical of post-concussive syndrome.

  19. Abdominal CT findings of delayed postoperative complications

    Energy Technology Data Exchange (ETDEWEB)

    Zissin, R.; Osadchy, A. [Sapir Medical Center, Dept. of Diagnostic Imaging, Kfar Saba (Israel)]. E-mail: zisinrivka@clalit.org.il; Gayer, G. [Assaf Harofe Medical Center, Dept. of Diagnostic Imaging, Zrifin (Israel)

    2007-10-15

    Despite progress in surgical techniques and modern medical treatment, postoperative complications occur not infrequently and vary according to type of surgery, clinical setting, and time elapsed since surgery. In general, they can be divided into early and delayed complications. Delayed postoperative complications can be classified as specific and nonspecific. The common nonspecific delayed complications are incisional hernia and postoperative bowel obstruction. Bowel obstruction can be further categorized as obstruction related to benign or neoplastic etiology, the latter occurring in oncology patients in whom the primary surgery was related to an underlying abdominal neoplasm. Gossypiboma is another, fortunately rare, postoperative complication. Specific complications appear after specific operations and include the following: Splenosis - following splenectomy. Retained gallstones and spilled gallstones - following cholecystectomy, mainly laparoscopic. Dropped appendicolith and stump appendicitis - following appendectomy, mainly laparoscopic. Obturation obstruction by a bezoar - following gastric surgery. Afferent loop syndrome (ALS) - following Bilroth II gastrectomy. (author)

  20. Complicated Crohn's-like colitis, associated with Hermansky-Pudlak syndrome, treated with Infliximab: a case report and brief review of the literature

    Directory of Open Access Journals (Sweden)

    Kouklakis George

    2007-12-01

    Full Text Available Abstract Introduction Hermansky-Pudlak syndrome (HPS is a rare autosomal recessive inherited disorder consisting of a triad of albinism, increased bleeding tendency secondary to platelet dysfunction, and systemic complications associated with ceroid depositions within the reticuloendothelial system. HPS has been associated with gastrointestinal (GI complications related to chronic granulomatous colitis with pathologic features suggestive of Crohn's disease. This colitis can be severe and has been reported to be poorly responsive to medical therapies including antibiotics, corticosteroids, sulfasalazine, mesalamine and azathioprine. Case presentation We report a patient with HPS which was complicated by inflammatory bowel disease with clinical and pathologic features of Crohn's disease, refractory to antibiotics, corticosteroids and azathioprine. A trial of infliximab was attempted and repeated infusions produced a complete response. Conclusion The occurrence of ileitis and perianal lesions and also the histopathological findings in our case suggest that HPS and Crohn's disease may truly be associated. Given this similarity and the failure of the standard medical therapy of corticosteroids and azathioprine, our patient received infliximab with marked clinical improvement.

  1. Ehlers-Danlos syndrome type IV

    Directory of Open Access Journals (Sweden)

    Germain Dominique P

    2007-07-01

    Full Text Available Abstract Ehlers-Danlos syndrome type IV, the vascular type of Ehlers-Danlos syndromes (EDS, is an inherited connective tissue disorder defined by characteristic facial features (acrogeria in most patients, translucent skin with highly visible subcutaneous vessels on the trunk and lower back, easy bruising, and severe arterial, digestive and uterine complications, which are rarely, if at all, observed in the other forms of EDS. The estimated prevalence for all EDS varies between 1/10,000 and 1/25,000, EDS type IV representing approximately 5 to 10% of cases. The vascular complications may affect all anatomical areas, with a tendency toward arteries of large and medium diameter. Dissections of the vertebral arteries and the carotids in their extra- and intra-cranial segments (carotid-cavernous fistulae are typical. There is a high risk of recurrent colonic perforations. Pregnancy increases the likelihood of a uterine or vascular rupture. EDS type IV is inherited as an autosomal dominant trait that is caused by mutations in the COL3A1 gene coding for type III procollagen. Diagnosis is based on clinical signs, non-invasive imaging, and the identification of a mutation of the COL3A1 gene. In childhood, coagulation disorders and Silverman's syndrome are the main differential diagnoses; in adulthood, the differential diagnosis includes other Ehlers-Danlos syndromes, Marfan syndrome and Loeys-Dietz syndrome. Prenatal diagnosis can be considered in families where the mutation is known. Choriocentesis or amniocentesis, however, may entail risk for the pregnant woman. In the absence of specific treatment for EDS type IV, medical intervention should be focused on symptomatic treatment and prophylactic measures. Arterial, digestive or uterine complications require immediate hospitalisation, observation in an intensive care unit. Invasive imaging techniques are contraindicated. Conservative approach is usually recommended when caring for a vascular

  2. Ehlers-Danlos syndrome type IV

    Science.gov (United States)

    Germain, Dominique P

    2007-01-01

    Ehlers-Danlos syndrome type IV, the vascular type of Ehlers-Danlos syndromes (EDS), is an inherited connective tissue disorder defined by characteristic facial features (acrogeria) in most patients, translucent skin with highly visible subcutaneous vessels on the trunk and lower back, easy bruising, and severe arterial, digestive and uterine complications, which are rarely, if at all, observed in the other forms of EDS. The estimated prevalence for all EDS varies between 1/10,000 and 1/25,000, EDS type IV representing approximately 5 to 10% of cases. The vascular complications may affect all anatomical areas, with a tendency toward arteries of large and medium diameter. Dissections of the vertebral arteries and the carotids in their extra- and intra-cranial segments (carotid-cavernous fistulae) are typical. There is a high risk of recurrent colonic perforations. Pregnancy increases the likelihood of a uterine or vascular rupture. EDS type IV is inherited as an autosomal dominant trait that is caused by mutations in the COL3A1 gene coding for type III procollagen. Diagnosis is based on clinical signs, non-invasive imaging, and the identification of a mutation of the COL3A1 gene. In childhood, coagulation disorders and Silverman's syndrome are the main differential diagnoses; in adulthood, the differential diagnosis includes other Ehlers-Danlos syndromes, Marfan syndrome and Loeys-Dietz syndrome. Prenatal diagnosis can be considered in families where the mutation is known. Choriocentesis or amniocentesis, however, may entail risk for the pregnant woman. In the absence of specific treatment for EDS type IV, medical intervention should be focused on symptomatic treatment and prophylactic measures. Arterial, digestive or uterine complications require immediate hospitalisation, observation in an intensive care unit. Invasive imaging techniques are contraindicated. Conservative approach is usually recommended when caring for a vascular complication in a patient suffering

  3. Admission Glycaemia and Acute Insulin Resistance in Heart Failure Complicating Acute Coronary Syndrome.

    Science.gov (United States)

    Lazzeri, Chiara; Valente, Serafina; Chiostri, Marco; D'Alfonso, Maria Grazia; Spini, Valentina; Angelotti, Paola; Gensini, Gian Franco

    2015-11-01

    Few data are so far available on the relation between increased glucose values and insulin resistance and mortality at short-term in patients with acute heart failure (AHF). The present investigation, performed in 409 consecutive patients with AHF complicating acute coronary syndrome (ACS), was aimed at assessing the prognostic role of admission glycaemia and acute insulin resistance (as indicated by the Homeostatic Model Assessment - HOMA index) for death during Intensive Cardiac Care (ICCU) stay. Admission glucose tertiles were considered. In our series, diabetic patients accounted for the 33%. Patients in the third glucose tertiles exhibited the lowest LVEF (both on admission and at discharge), a higher use of mechanical ventilation, intra-aortic balloon pump and inotropic drugs and the highest in-ICCU mortality rate. In the overall population, hyperglycaemic patients (both diabetic and non diabetic) were 227 (227/409, 55.5%). Admission glycaemia was an independent predictor of in-ICCU mortality, together with admission LVEF and eGFR, while acute insulin resistance (as indicated by HOMA-index) was not associated with early death. The presence of admission hyperglycaemia in non-diabetic patients was independently associated with in-ICCU death while hyperglycaemia in diabetic patients was not. According to our results, hyperglycaemia is a common finding in patients with ACS complicated by AHF and it is an independent predictor of early death. Non-diabetic patients with hyperglycaemia are the subgroup with the highest risk of early death. Copyright © 2015 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.

  4. Vascular complications after the treatment of carcinoma of the cervix

    International Nuclear Information System (INIS)

    Cormier, J.M.; Laurian, C.

    1980-01-01

    Vascular complications after this treatment are rare. Artery stenosis or occlusions, more often iliac, are well recognized complications of postoperative radiotherapy. These segmental lesions, sometimes, are pathological lesions similar to arteriosclerotic changes. These lesions may be corrected later on after the treatment of carcinoma of the cervix. The venous complications often present later and tall within the context of post thrombotic syndrome. Conservative treatment is indicated alone. A well recognized complication, lymphedema is usually mild; a superimposed iliofemoral thrombophlebitis often may be suspected [fr

  5. A treat-to-target strategy with methotrexate and intra-articular triamcinolone with or without adalimumab effectively reduces MRI synovitis, osteitis and tenosynovitis and halts structural damage progression in early rheumatoid arthritis

    DEFF Research Database (Denmark)

    Axelsen, Mette Bjørndal; Eshed, Iris; Hørslev-Petersen, Kim

    2014-01-01

    To investigate whether a treat-to-target strategy with methotrexate and intra-articular glucocorticosteroid injections suppresses MRI inflammation and halts structural damage progression in patients with early rheumatoid arthritis (ERA), and whether adalimumab provides an additional effect....

  6. Pericardial tamponade complicated by interventional management for Budd-Chiari syndrome: clinical analysis and treatment

    International Nuclear Information System (INIS)

    Zhang Luxi; Zu Maoheng; Wu Jinping; Xu Hao; Jiao Xudong; Chen Zhengkan

    2011-01-01

    Objective: To discuss the cases and treatment of pericardial tamponade (PT) occurred in the interventional management for Budd-Chiari syndrome (BCS). Methods: During the period from 1990 to 2006, interventional treatment was performed in 812 patients with BCS. Pericardial tamponade occurred in nine patients during the period of interventional treatment. The clinical data, including angiographic findings, clinical symptoms, management and outcomes, of the nine patients were retrospectively analyzed. The possible causes of pericardial tamponade were discussed. Results: Of the nine patients occurring pericardial tamponade, successful treatment was obtained in eight and death occurred in one. The lesions of BCS in the nine cases included inferior vena cava obstruction type (n=7), hepatic venous obstruction type (n=1) and mixed type (n=1). Pericardial tamponade was caused by mistakenly puncturing into pericardium (n=5), mistakenly puncturing together with laceration of pericardium by balloon (n=3), and breaking of pericardium by displaced stent (n=1). Conventional pericardicentesis was employed in one case, surgery was carried out in three cases, and infra-xiphoid catheterization and drainage using Seldinger technique was performed in two cases. Conservative treatment was adopted in one case and aspiration through the wrongly inserted catheter was tried in one case. In the remaining one case, aspiration through the wrongly inserted catheter together with infra-xiphoid catheterization and drainage by using Seldinger technique was carried out. Conclusion: The pericardial tamponade is an severe complication occurred in the interventional management for Budd-Chiari syndrome, although it is rarely seen. Preoperative prevention, prompt detection and rational treatment are the keys avoid serious consequences. (authors)

  7. Lemierre syndrome: two cases and a review.

    Science.gov (United States)

    Syed, Mohammed Iqbal; Baring, David; Addidle, Michael; Murray, Craig; Adams, Calum

    2007-09-01

    Lemierre syndrome is usually caused by an acute oropharyngeal infection in previously healthy young adults, resulting in thrombophlebitis of the internal jugular vein, leading to metastatic septic embolization and bacteraemia. The usual organism is Fusobacterium necrophorum. Lemierre syndrome, not so long ago labeled as the "forgotten disease," is on the rise. Today with increasing antibiotic-resistant organisms, and decreasing awareness of the syndrome, subsequent re-emergence of this "forgotten disease" is becoming more common in clinical settings. Lemierre syndrome has significant morbidity. Cranial nerve complications associated with the condition have been increasingly diagnosed in the last few years. Looking back at literature on Lemierre syndrome, there have been review articles in medical and microbiology journals but rarely in otolaryngology journals. By presenting our cases we demonstrate the diverse presentations and severity of the illness. A review of the literature and a case report on two cases seen in our institution in the last year are presented. Each of these had varied presentations and neurologic complications-one developed 9th to 12th cranial nerve palsies and Horner syndrome, which have not been described in previous literature, and the other developed polyneuropathy and a frontal lobe infarct among other multisystem complications. Diagnosis of Lemierre syndrome is not always straightforward as clinical features are variable and blood cultures are often negative. Awareness of the syndrome and a high degree of suspicion are needed.

  8. Classic Bartter syndrome complicated with profound growth hormone deficiency: a case report.

    Science.gov (United States)

    Adachi, Masanori; Tajima, Toshihiro; Muroya, Koji; Asakura, Yumi

    2013-12-30

    Classic Bartter syndrome is a salt-wasting tubulopathy caused by mutations in the CLCNKB (chloride channel Kb) gene. Although growth hormone deficiency has been suggested as a cause for persistent growth failure in patients with classic Bartter syndrome, in our opinion the diagnoses of growth hormone deficiency has been unconvincing in some reports. Moreover, Gitelman syndrome seems to have been confused with Bartter syndrome in some cases in the literature. In the present work, we describe a new case with CLCNKB gene mutations and review the reported cases of classic Bartter syndrome associated with growth hormone deficiency. Our patient was a Japanese boy diagnosed as having classic Bartter syndrome at eight months of age. The diagnosis of Bartter syndrome was confirmed by CLCNKB gene analysis, which revealed compound heterozygous mutations with deletion of exons 1 to 3 (derived from his mother) and ΔL130 (derived from his father). His medical therapy consisted of potassium (K), sodium chloride, spironolactone, and anti-inflammatory agents; this regime was started at eight months of age. Our patient was very short (131.1cm, -4.9 standard deviation) at 14.3 years and showed profoundly impaired growth hormone responses to pharmacological stimulants: 0.15μg/L to insulin-induced hypoglycemia and 0.39μg/L to arginine. His growth response to growth hormone therapy was excellent. The present case strengthens the association between classic Bartter syndrome and growth hormone deficiency. We propose that growth hormone status should be considered while treating children with classic Bartter syndrome.

  9. Lung-Protective Ventilation With Low Tidal Volumes and the Occurrence of Pulmonary Complications in Patients Without Acute Respiratory Distress Syndrome: A Systematic Review and Individual Patient Data Analysis

    NARCIS (Netherlands)

    Neto, Ary Serpa; Simonis, Fabienne D.; Barbas, Carmen S. V.; Biehl, Michelle; Determann, Rogier M.; Elmer, Jonathan; Friedman, Gilberto; Gajic, Ognjen; Goldstein, Joshua N.; Linko, Rita; Pinheiro de Oliveira, Roselaine; Sundar, Sugantha; Talmor, Daniel; Wolthuis, Esther K.; Gama de Abreu, Marcelo; Pelosi, Paolo; Schultz, Marcus J.

    2015-01-01

    Protective mechanical ventilation with low tidal volumes is standard of care for patients with acute respiratory distress syndrome. The aim of this individual patient data analysis was to determine the association between tidal volume and the occurrence of pulmonary complications in ICU patients

  10. Pre- and perinatal complications in relation to Tourette syndrome and co-occurring obsessive-compulsive disorder and attention-deficit/hyperactivity disorder

    Science.gov (United States)

    Abdulkadir, Mohamed; Tischfield, Jay A.; King, Robert A.; Fernandez, Thomas V.; Brown, Lawrence W.; Cheon, Keun-Ah; Coffey, Barbara J.; de Bruijn, Sebastian F. T. M.; Elzerman, Lonneke; Garcia-Delgar, Blanca; Gilbert, Donald L.; Grice, Dorothy E.; Hagstrøm, Julie; Hedderly, Tammy; Heyman, Isobel; Hong, Hyun Ju; Huyser, Chaim; Ibanez-Gomez, Laura; Kim, Young Key; Kim, Young-Shin; Koh, Yun-Joo; Kook, Sodahm; Kuperman, Samuel; Lamerz, Andreas; Leventhal, Bennett; Ludolph, Andrea G.; Madruga-Garrido, Marcos; Maras, Athanasios; Messchendorp, Marieke D.; Mir, Pablo; Morer, Astrid; Münchau, Alexander; Murphy, Tara L.; Openneer, Thaïra J. C.; Plessen, Kerstin J.; Rath, Judith J. G.; Roessner, Veit; Fründt, Odette; Shin, Eun-Young; Sival, Deborah A.; Song, Dong-Ho; Song, Jungeun; Stolte, Anne-Marie; Tübing, Jennifer; van den Ban, Els; Visscher, Frank; Wanderer, Sina; Woods, Martin; Zinner, Samuel H.; State, Matthew W.; Heiman, Gary A.; Hoekstra, Pieter J.; Dietrich, Andrea

    2016-01-01

    Pre- and perinatal complications have been implicated in the onset and clinical expression of Tourette syndrome albeit with considerable inconsistencies across studies. Also, little is known about their role in co-occurring obsessive-compulsive disorder (OCD) and attention–deficit/hyperactivity disorder (ADHD) in individuals with a tic disorder. Therefore, we aimed to investigate the role of pre- and perinatal complications in relation to the presence and symptom severity of chronic tic disorder and co-occurring OCD and ADHD using data of 1,113 participants from the Tourette International Collaborative Genetics study. This study included 586 participants with a chronic tic disorder and 527 unaffected family controls. We controlled for age and sex differences by creating propensity score matched subsamples for both case-control and within-case analyses. We found that premature birth (OR=1.72) and morning sickness requiring medical attention (OR=2.57) were associated with the presence of a chronic tic disorder. Also, the total number of pre- and perinatal complications was higher in those with a tic disorder (OR=1.07). Furthermore, neonatal complications were related to the presence (OR=1.46) and severity (b=2.27) of co-occurring OCD and also to ADHD severity (b=1.09). Delivery complications were only related to co-occurring OCD (OR=1.49). We conclude that early exposure to adverse situations during pregnancy is related to the presence of chronic tic disorders. Exposure at a later stage, at birth or during the first weeks of life, appears to be associated with co-occurring OCD and ADHD. PMID:27494079

  11. Churg-Strauss syndrome masquerading as an acute coronary syndrome.

    Science.gov (United States)

    Triantafyllis, Andreas S; Sakadakis, Eleftherios A; Papafilippaki, Argyro; Katsimbri, Pelagia; Panou, Fotios; Anastasiou-Nana, Maria; Lekakis, Ioannis

    2015-02-01

    Churg-Strauss Syndrome (CSS) is a rare vasculitis with multiorgan involvement. Cardiac manifestations are common causing serious complications. We report a case of CSS masquerading as a non-ST elevation myocardial infarction with heart failure. CSS should be considered in the differential diagnosis of an acute coronary syndrome(ACS)with normal coronary arteries when history of asthma, peripheral eosinophilia and multisystemic involvement is present.

  12. Chronic Disseminated Candidiasis Complicated by Immune Reconstitution Inflammatory Syndrome in Child with Acute Lymphoblastic Leukemia

    Directory of Open Access Journals (Sweden)

    Olga Zając-Spychała

    2016-01-01

    Full Text Available Hepatosplenic candidiasis also known as chronic disseminated candidiasis is a rare manifestation of invasive fungal infection typically observed in patients with acute leukemia in prolonged, deep neutropenia. Immune reconstitution inflammatory syndrome (IRIS is an inflammatory disorder triggered by rapid resolution of neutropenia. Diagnosis and treatment of IRIS are still challenging due to a variety of clinical symptoms, lack of certain diagnostic criteria, and no standards of treatment. The diagnosis of IRIS is even more difficult in patients with hematological malignancies complicated by “probable” invasive fungal infection, when fungal pathogen is still uncertain. We report a case of probable hepatic candidiasis in 4-year-old boy with acute lymphoblastic leukemia. Despite proper antifungal therapy, there was no clinical and radiological improvement, so diagnosis of Candida-related IRIS was made and corticosteroid therapy was added to antifungal treatment achieving prompt resolution of infection symptoms.

  13. Computed tomography findings of postoperative complications in lung transplantation

    International Nuclear Information System (INIS)

    Hochhegger, Bruno; Irion, Klaus Loureiro; Marchiori, Edson; Bello, Rodrigo; Moreira, Jose; Camargo, Jose Jesus; Universidade Federal do Rio de Janeiro

    2009-01-01

    Due to the increasing number and improved survival of lung transplant recipients, radiologists should be aware of the imaging features of the postoperative complications that can occur in such patients. The early treatment of complications is important for the long-term survival of lung transplant recipients. Frequently, HRCT plays a central role in the investigation of such complications. Early recognition of the signs of complications allows treatment to be initiated earlier, which improves survival. The aim of this pictorial review was to demonstrate the CT scan appearance of pulmonary complications such as reperfusion edema, acute rejection, infection, pulmonary thromboembolism, chronic rejection, bronchiolitis obliterans syndrome, cryptogenic organizing pneumonia, post transplant lymphoproliferative disorder, bronchial dehiscence and bronchial stenosis. (author)

  14. Effective intravenous immunoglobulin therapy for Churg-Strauss syndrome (allergic granulomatous angiitis complicated by neuropathy of the eighth cranial nerve: a case report

    Directory of Open Access Journals (Sweden)

    Ozaki Yoshio

    2012-09-01

    Full Text Available Abstract Introduction We report the case of a patient with Churg-Strauss syndrome with eighth cranial nerve palsy. Vestibulocochlear nerve palsy is extremely rare in Churg-Strauss syndrome. To the best of our knowledge, only one case of complicated neuropathy of the eighth cranial nerve has been described in a previous report presenting an aggregate calculation, but no differentiation between polyarteritis nodosa and Churg-Strauss syndrome was made. High-dose immunoglobulin was administered to our patient, and her neuropathy of the eighth cranial nerve showed improvement. Case presentation At the age of 46, a Japanese woman developed Churg-Strauss syndrome that later became stable with low-dose prednisolone treatment. At the age of 52, she developed sudden difficulty of hearing in her left ear, persistent severe rotary vertigo, and mononeuritis multiplex. At admission, bilateral perceptive deafness of about 80dB and eosinophilia of 4123/μL in peripheral blood were found. A diagnosis of cranial neuropathy of the eighth cranial nerve associated with exacerbated Churg-Strauss syndrome was made. Although high doses of steroid therapy alleviated the inflammatory symptoms and markers, the vertigo and bilateral hearing loss remained. Addition of a high-dose immunoglobulin finally resulted in marked alleviation of the symptoms associated with neuropathy of the eighth cranial nerve. Conclusions A high dose of immunoglobulin therapy shows favorable effects in neuropathy of the eighth cranial nerve, but no reports regarding its efficacy in cranial neuropathy have been published.

  15. Acute pancreatitis : a newly recognised potential complication of canine babesiosis

    Directory of Open Access Journals (Sweden)

    A.J. Möhr

    2000-07-01

    Full Text Available This retrospective study describes 4 cases of canine babesiosis with histologically confirmed acute pancreatitis. In addition, 16 dogs with babesiosis are reported with serum amylase (>3500 U/l and/or lipase (>650 U/l activity elevations of a magnitude that would support a diagnosis of probable acute pancreatitis, although extra-pancreatic sources of the enzymes could not be excluded in these cases. Median time of pancreatitis diagnosis was 2.5 days post-admission, with primarily young (median age 3 years, sexually intact dogs affected. The development of pancreatitis was unrelated to the degree of anaemia at time of admission. In addition to pancreatitis, 80 % of cases suffered from other babesial complications, namely icterus (13, acute respiratory distress syndrome (6, immune-mediated haemolytic anaemia (6, renal failure (3, haemoconcentration (2 and cerebral syndrome (2. Acute respiratory distress syndrome, renal failure and cerebral syndrome were associated with a poor prognosis, with 4 of the 5 dogs included in the overall 26 % mortality rate having at least 1 of these complications. Haemolytic anaemia with ischaemia-reperfusion injury to the pancreas is proposed as a possible primary pathophysiological mechanism in babesial pancreatitis. Hypotensive shock, immune-mediated haemolytic anaemia, haemoconcentration and possibly altered lipid metabolism in babesiosis may also be involved. The previously postulated pro-inflammatory cytokine milieu of complicated babesiosis may underlie the progression, if not the primary initiation, of pancreatic pathology. Acute pancreatitis may represent the previously reported 'gut' form of babesiosis.

  16. Cardiorenal Syndrome in Acute Heart Failure Syndromes

    Directory of Open Access Journals (Sweden)

    Mohammad Sarraf

    2011-01-01

    Full Text Available Impaired cardiac function leads to activation of the neurohumoral axis, sodium and water retention, congestion and ultimately impaired kidney function. This sequence of events has been termed the Cardiorenal Syndrome. This is different from the increase in cardiovascular complications which occur with primary kidney disease, that is, the so-called Renocardiac Syndrome. The present review discusses the pathogenesis of the Cardiorenal Syndrome followed by the benefits and potential deleterious effects of pharmacological agents that have been used in this setting. The agents discussed are diuretics, aquaretics, natriuretic peptides, vasodilators, inotropes and adenosine α1 receptor antagonists. The potential role of ultrafiltration is also briefly discussed.

  17. Thrombophilia and pregnancy complications: cause or association?

    NARCIS (Netherlands)

    Middeldorp, S.

    2007-01-01

    Both acquired and inherited thrombophilia is associated with an increased risk of pregnancy failure (i.e. sporadic and recurrent miscarriage, late fetal loss), as well as hypertensive pregnancy complications such as pre-eclampsia and HELLP syndrome. The question of whether this relationship can be

  18. Hypophosphatemia is a common complication in severely disabled individuals with neurological disorders and is caused by infection, refeeding and Fanconi syndrome.

    Science.gov (United States)

    Saito, Yoshiaki; Aoki, Yusuke; Takeshita, Eri; Saito, Takashi; Sugai, Kenji; Komaki, Hirofumi; Nakagawa, Eiji; Ishiyama, Akihiko; Takanoha, Satoko; Wada, Satoru; Sasaki, Masayuki

    2014-11-01

    To describe the characteristics of hypophosphatemia in severely disabled individuals with neurological disorders and to identify its causative factors. We retrospectively reviewed clinical data from 82 individuals with motor skills classified as sitting, rollover or bedridden. Age, gender and body mass index were compared in individuals with (n=19) and without (n=63) a history of hypophosphatemia (serum phosphate levels refeeding syndrome (n=4) and Fanconi syndrome (n=3), but was unidentifiable in one episode. Significant elevations in C-reactive protein levels and reductions in sodium levels were observed during hypophosphatemia episodes. Hypophosphatemia is a common complication in severely disabled individuals with frequent bacterial infections, refeeding following malnutrition and valproate administration for epilepsy treatment. Because severe hypophosphatemia is life threatening, serum phosphate levels should be closely monitored in this population. Copyright © 2013 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  19. Surgical complications following cochlear implantation in adults based on a proposed reporting consensus

    DEFF Research Database (Denmark)

    Jeppesen, Jonas; Faber, Christian Emil

    2013-01-01

    Abstract Conclusion: The rate of severe complications was low and cochlear implantation is a relatively safe procedure. Standardization is crucial when reporting on cochlear implant complications to ensure comparability between studies. A consensus on the reporting of complications proposed by a ...... occurred following one implantation (0.3%). Transient chorda tympani syndrome (30.8%), vertigo/dizziness (29.5%) and tinnitus (4.9%) were the most frequent minor complications....

  20. [Study on the relation between Pi-deficiency pattern and metabolic syndrome in patients with polycystic ovarian syndrome].

    Science.gov (United States)

    Wang, Xing-Juan; Jin, Hua-Liang; Liu, Ying

    2010-11-01

    To evaluate the relation between Pi-deficiency syndrome (PDS) pattern and metabolic syndrome (MS) in patients with polycystic ovarian syndrome (PCOS), for exploring their internal pathologic mechanism. Among the 102 PCOS patients, 22 complicated with MS (PCOS-MS) and 80 not complicated with MS (PCOS-NMS), the Chinese medicine syndrome pattern was differentiated as PDS in 50 patients and non-PDS in 52. The clinical data, in terms of fasting blood glucose (FBG), fasting insulin (FINS), waistline, body weight (BW), stature, blood pressure (BP), etc. was collected and compared and the relation between data was analyzed. Levels of FINS and homeostasis model of assessment for insulin resistence index (HOMA-IR), in PCOS-MS patients were significantly higher than those in PCOS-NMS patients, also higher in patients of PDS pattern than those of non-PDS pattern (P 0.05). PCOS patients of PDS pattern are the high-risk population of MS, which might be related with the insulin resistance. So, early treatment of PCOS, especially on patients of PDS pattern, is of important significance for preventing the complication, as MS, of the disease.

  1. Clinical features and respiratory complications in Myhre syndrome

    NARCIS (Netherlands)

    McGowan, Ruth; Gulati, Ramkumar; McHenry, Pamela; Cooke, Alexander; Butler, Sandra; Keng, Wee Teik; Murday, Victoria; Whiteford, Margo; Dikkers, Frederik G.; Sikkema-Raddatz, Brigit; van Essen, Ton; Tolmie, John

    2011-01-01

    We describe the clinical characteristics of 4 singleton cases, 3 males and 1 female, with Myhre Syndrome (OMIM 139210), who were born to non-consanguineous parents. Three cases had no family history of similarly affected individuals but 1 male's mother had short stature, some facial features

  2. A randomised controlled trial of the clinical effectiveness, safety and cost-effectiveness of adalimumab in combination with methotrexate for the treatment of juvenile idiopathic arthritis associated uveitis (SYCAMORE Trial).

    Science.gov (United States)

    Ramanan, Athimalaipet V; Dick, Andrew D; Benton, Diana; Compeyrot-Lacassagne, Sandrine; Dawoud, Dalia; Hardwick, Ben; Hickey, Helen; Hughes, Dyfrig; Jones, Ashley; Woo, Patricia; Edelsten, Clive; Beresford, Michael W

    2014-01-09

    Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children. Children with JIA are at risk of inflammation of the uvea in the eye (uveitis). Overall, 20% to 25% of paediatric uveitis is associated with JIA. Major risk factors for development of uveitis in JIA are oligoarticular pattern of arthritis, an age at onset of arthritis of less than seven years of age, and antinuclear antibody positivity. In the initial stages of mild to moderate inflammation the uveitis is asymptomatic. This has led to current practice of screening all children with JIA for uveitis. Approximately 12% to 38% of patients with JIA develop uveitis in seven years following onset of arthritis. In 30% to 50% of children with JIA-associated uveitis structural complications are present at diagnosis. Furthermore about 50% to 75% of those with severe uveitis will eventually develop visual impairment secondary to ocular complications such as cataract and glaucoma. Defining the severity of inflammation and structural complications in uveitis patients is now possible following Standardised Uveitis Nomenclature (SUN) guidelines, and modified to incorporate the consensus of end point and outcome criteria into the design of randomised trials. Despite current screening and therapeutic options (pre-biologics) 10% to 15% of children with JIA-associated uveitis may develop bilateral visual impairment and certified legally blind. To date, there remains no controlled trial evidence of benefits of biologic therapy. This study will randomise 154 patients aged 2 to 18 years with active JIA-associated uveitis (despite methotrexate (MTX) treatment for at least 12 weeks). All participants will be treated for 18 months, with follow up of 3 years from randomisation (continuing on MTX throughout). All participants will receive a stable dose of MTX and in addition either adalimumab (20 mg/0.8 ml for patientstreatment of juvenile idiopathic arthritis associated uveitis. ISRCTN10065623.

  3. Pre- and perinatal complications in relation to Tourette syndrome and co-occurring obsessive-compulsive disorder and attention-deficit/hyperactivity disorder.

    Science.gov (United States)

    Abdulkadir, Mohamed; Tischfield, Jay A; King, Robert A; Fernandez, Thomas V; Brown, Lawrence W; Cheon, Keun-Ah; Coffey, Barbara J; de Bruijn, Sebastian F T M; Elzerman, Lonneke; Garcia-Delgar, Blanca; Gilbert, Donald L; Grice, Dorothy E; Hagstrøm, Julie; Hedderly, Tammy; Heyman, Isobel; Hong, Hyun Ju; Huyser, Chaim; Ibanez-Gomez, Laura; Kim, Young Key; Kim, Young-Shin; Koh, Yun-Joo; Kook, Sodahm; Kuperman, Samuel; Lamerz, Andreas; Leventhal, Bennett; Ludolph, Andrea G; Madruga-Garrido, Marcos; Maras, Athanasios; Messchendorp, Marieke D; Mir, Pablo; Morer, Astrid; Münchau, Alexander; Murphy, Tara L; Openneer, Thaïra J C; Plessen, Kerstin J; Rath, Judith J G; Roessner, Veit; Fründt, Odette; Shin, Eun-Young; Sival, Deborah A; Song, Dong-Ho; Song, Jungeun; Stolte, Anne-Marie; Tübing, Jennifer; van den Ban, Els; Visscher, Frank; Wanderer, Sina; Woods, Martin; Zinner, Samuel H; State, Matthew W; Heiman, Gary A; Hoekstra, Pieter J; Dietrich, Andrea

    2016-11-01

    Pre- and perinatal complications have been implicated in the onset and clinical expression of Tourette syndrome albeit with considerable inconsistencies across studies. Also, little is known about their role in co-occurring obsessive-compulsive disorder (OCD) and attention-deficit/hyperactivity disorder (ADHD) in individuals with a tic disorder. Therefore, we aimed to investigate the role of pre- and perinatal complications in relation to the presence and symptom severity of chronic tic disorder and co-occurring OCD and ADHD using data of 1113 participants from the Tourette International Collaborative Genetics study. This study included 586 participants with a chronic tic disorder and 527 unaffected family controls. We controlled for age and sex differences by creating propensity score matched subsamples for both case-control and within-case analyses. We found that premature birth (OR = 1.72) and morning sickness requiring medical attention (OR = 2.57) were associated with the presence of a chronic tic disorder. Also, the total number of pre- and perinatal complications was higher in those with a tic disorder (OR = 1.07). Furthermore, neonatal complications were related to the presence (OR = 1.46) and severity (b = 2.27) of co-occurring OCD and also to ADHD severity (b = 1.09). Delivery complications were only related to co-occurring OCD (OR = 1.49). We conclude that early exposure to adverse situations during pregnancy is related to the presence of chronic tic disorders. Exposure at a later stage, at birth or during the first weeks of life, appears to be associated with co-occurring OCD and ADHD. Copyright © 2016 Elsevier Ltd. All rights reserved.

  4. Complications and Monitoring – Guidelines on Parenteral Nutrition, Chapter 11

    Directory of Open Access Journals (Sweden)

    Working group for developing the guidelines for parenteral nutrition of The German Association for Nutritional Medicine

    2009-11-01

    Full Text Available Compared to enteral or hypocaloric oral nutrition, the use of PN (parenteral nutrition is not associated with increased mortality, overall frequency of complications, or longer length of hospital stay (LOS. The risk of PN complications (e.g. refeeding-syndrome, hyperglycaemia, bone demineralisation, catheter infections can be minimised by carefully monitoring patients and the use of nutrition support teams particularly during long-term PN. Occuring complications are e.g. the refeeding-syndrome in patients suffering from severe malnutrition with the initiation of refeeding or metabolic, hypertriglyceridemia, hyperglycaemia, osteomalacia and osteoporosis, and hepatic complications including fatty liver, non-alcoholic fatty liver disease, cholestasis, cholecystitis, and cholelithiasis. Efficient monitoring in all types of PN can result in reduced PN-associated complications and reduced costs. Water and electrolyte balance, blood sugar, and cardiovascular function should regularly be monitored during PN. Regular checks of serum electrolytes and triglycerides as well as additional monitoring measures are necessary in patients with altered renal function, electrolyte-free substrate intake, lipid infusions, and in intensive care patients. The metabolic monitoring of patients under long-term PN should be carried out according to standardised procedures. Monitoring metabolic determinants of bone metabolism is particularly important in patients receiving long-term PN. Markers of intermediary, electrolyte and trace element metabolism require regular checks.

  5. A Giant Hepatic Hemangioma Complicated by Kasabach-Merritt Syndrome: Findings of Tc-99m RBC Scintigraphy and SPECT Including a Total Body Blood Pool Imaging Study

    Energy Technology Data Exchange (ETDEWEB)

    Sohn, Myung Hee; Jeong, Hwan Jeong; Lim, Seok Tae; Kim, Dong Wook; Yim, Chang Yeol [Chonbuk National University Medical School, Jeonju (Korea, Republic of)

    2009-02-15

    Kasabach-Merritt syndrome (KMS) consists of thrombocytopenia, microangiopathic hemolytic anemia, and localized consumption coagulopathy that develops within vascular hemangioma. This syndrome may also be associated with occult hemangiomas located at various sites. Tc-99m RBC scintigraphy and SPECT have proven to be reliable for confirming or excluding hemangioma. Total body blood pool imaging study during the scintigraphy also provides a means of screening for occult lesions. The authors report the case of a 29-year-old man who presented with a giant hepatic hemangioma complicated by KMS, and underwent Tc-99m RBC scintigraphy and SPECT including a total body blood pool imaging study.

  6. CT study in primary low spinal fluid pressure syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Hoshino, Moritoshi; Okayama, Kenji; Kubo, Hiromasa; Watanabe, Hiromi; Endou, Riuko (Ohmiya Red Cross Hospital, Yono, Saitama (Japan))

    1991-02-01

    CT findings in primary low spinal fluid pressure syndrome were studied on the basis of 3 cases. Case 1 was a 43-year-old male with a complicated bilateral isodense subdural hematoma (SDH). Case 2 was a 45-year-old female with a complicated bilateral high dense SDH. Case 3 was a 36-year-old female discharged without any complications after spinal fluid pressure normalized. Slight downward displacement of the brain under low spinal fluid pressure was shown as the narrowing of a Sylvian fissures and infratentorial cisterns on CT. On the other hand, in this syndrome with a complicated bilateral isodense SDH, in addition to this finding, CT revealed distortion and narrowing of body lateral ventricles, which might be differential findings from this syndrome without complicated SDH. Under low spinal fluid pressure, bridging veins are more stretched by a downward displacement of the brain. And consequently they were easily injured and SDH was developed. (author).

  7. Cardiac complications and immunophenotypic profile of infectious mononucleosis syndrome in children.

    Science.gov (United States)

    Papadopoulou-Legbelou, Kyriaki; Papadopoulou-Alataki, Efimia; Fleva, Alexandra; Spanou, Sofia; Pavlitou, Aikaterini; Varlamis, George

    2012-03-01

    To investigate cardiac complications in infectious mononucleosis patients and to associate them with biochemical and immunological parameters, as well as with spleen ultrasound findings. Cross-sectional study with follow-up. Tertiary care pediatric unit, in the city of Thessaloniki, Greece. Twenty-five children (15 boys, aged 1-11.6 years) suffering from infectious mononucleosis were studied during the acute phase and after 3-6 months. Cardiac evaluation comprised of electrocardiogram, echocardiogram, and measurement of creatine phosphokinase, creatine phosphokinase cardiac isoenzyme, and troponin levels. Biochemical and immunological tests included serum transaminases, serum amylase, CD3+/CD8+ T-lymphocytes subpopulation and CD4+/CD8+ T-lymphocytes ratio. During acute phase, all children had splenomegaly and normal serum amylase values. 17 patients had elevated serum transaminases. Percentages of CD3+/CD8+ T-lymphocytes subpopulation were elevated and CD4+/CD8+ ratio was decreased in all patients. Echocardiography revealed mild pericardial effusion in 13 patients (10/21 with Epstein-Barr infection, 3/4 with cytomegalovirus infection), but none presented with myocarditis. Four out of these 13 patients also had markedly elevated liver enzymes, 10/13 had significant splenomegaly and 12/13 presented very low CD4+/CD8+ T-lymphocytes ratio. Pericardial effusion demonstrated a statistically significant association solely with very low CD4+/CD8+ T-lymphocytes ratio (mononucleosis syndrome, asymptomatic pericardial effusion could be associated with very low CD4+/CD8+ ratio (<0.5). Further studies would extend and confirm such an association.

  8. Vascular manifestations of syndromic aortopathies: role of current and emerging imaging techniques

    International Nuclear Information System (INIS)

    Westerland, O.; Frigiola, A.; Robert, L.; Shaw, A.; Blakeway, L.; Katsanos, K.; Kiesewetter, C.; Chung, N.; Karunanithy, N.

    2015-01-01

    Patients with connective tissue diseases such as Marfan's syndrome, Loeys–Dietz syndrome, and vascular Ehlers–Danlos syndrome comprise a small but important group of patients who present early with acute aortic syndrome comprising aneurysmal dilation, rupture, or aortic dissection. Cardiovascular pathologies are an important yet treatable cause of morbidity and mortality in these patients. Imaging plays an important role in initial diagnosis, surveillance, and identification of complications. Furthermore, these patients are prone to developing complications in other vascular territories. Effective screening and surveillance will allow early diagnosis and elective treatment thus reducing the morbidity and mortality associated with presentation with acute complications. In this article, we will provide an overview of the role of magnetic resonance and computed tomography angiography in the management of syndromic aortopathies.

  9. Clinical review: Bleeding - a notable complication of treatment in patients with acute coronary syndromes: incidence, predictors, classification, impact on prognosis, and management

    Science.gov (United States)

    2013-01-01

    This article focuses on the incidence, predictors, classification, impact on prognosis, and management of bleeding associated with the treatment of acute coronary syndrome. The issue of bleeding complications is related to the continual improvement of ischemic heart disease treatment, which involves mainly (a) the widespread use of coronary angiography, (b) developments in percutaneous coronary interventions, and (c) the introduction of new antithrombotics. Bleeding has become an important health and economic problem and has an incidence of 2.0% to 17%. Bleeding significantly influences both the short- and long-term prognoses. If a group of patients at higher risk of bleeding complications can be identified according to known risk factors and a risk scoring system can be developed, we may focus more on preventive measures that should help us to reduce the incidence of bleeding. PMID:24093465

  10. Iatrogenic Cushing's syndrome caused by intranasal steroid use.

    Science.gov (United States)

    Dursun, Fatma; Kirmizibekmez, Heves

    2017-01-01

    Cushing's syndrome (CS) is common after oral steroid use and has also been reported following topical or inhaled use, but it is extremely uncommon after intranasal administration. This is the case of a 6-year-old child who developed Cushing's syndrome after intranasal application of dexamethasone sodium phosphate for a period of 6 months. Pediatricians and other clinical practitioners should be aware that high-dose and long-term nasal steroid administration may cause iatrogenic Cushing's syndrome characterized by complications of glucocorticoid excess as well as serious and even life-threatening complications of adrenal insufficiency.

  11. Magnetic resonance imaging of the claude syndrome

    International Nuclear Information System (INIS)

    Toshima, Hiroko; Miyajima, Masayuki; Kinoshita, Masanobu; Shimojou, Sadatomo; Miyahara, Tadashi

    1988-01-01

    A case of the Claude syndrome complicated by Parinaud's syndrome is reported. A 68-year-old male suddenly developed a diplopia and ataxic gait on June 16, 1985. On admission, he exhibited a right oculomotor palsy, an upward-gaze palsy, and left cerebellar ataxia. A brain CT was negative, but MRI performed on June 19 showed a low-intensity area in the left red nucleus of the midbrain on inversion recovery. The patient was thought to have a Claude syndrome due to an ischemic cerebral infarct. Since the first report, by Claude in 1912 of a case, with an unilateral oculomotor palsy and contralateral cerebellar ataxia, the lesions responsible for which were attributed to the red nucleus, many cases have been reported in the literature as the Claude syndrome or as a red-nucleus syndrome. In Japan 8 cases of the Claude syndrome have thus far been reported, but only one report refers to the CT findings of this syndrome. This is the first report to describe the MRI findings of a case of the Claude syndrome complicated with Parinaud's syndrome; a red-nucleus lesion of the homolateral midbrain has been clearly demonstrated. (author)

  12. Magnetic resonance imaging of the claude syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Toshima, Hiroko; Miyajima, Masayuki; Kinoshita, Masanobu; Shimojou, Sadatomo; Miyahara, Tadashi

    1988-04-01

    A case of the Claude syndrome complicated by Parinaud's syndrome is reported. A 68-year-old male suddenly developed a diplopia and ataxic gait on June 16, 1985. On admission, he exhibited a right oculomotor palsy, an upward-gaze palsy, and left cerebellar ataxia. A brain CT was negative, but MRI performed on June 19 showed a low-intensity area in the left red nucleus of the midbrain on inversion recovery. The patient was thought to have a Claude syndrome due to an ischemic cerebral infarct. Since the first report, by Claude in 1912 of a case, with an unilateral oculomotor palsy and contralateral cerebellar ataxia, the lesions responsible for which were attributed to the red nucleus, many cases have been reported in the literature as the Claude syndrome or as a red-nucleus syndrome. In Japan 8 cases of the Claude syndrome have thus far been reported, but only one report refers to the CT findings of this syndrome. This is the first report to describe the MRI findings of a case of the Claude syndrome complicated with Parinaud's syndrome; a red-nucleus lesion of the homolateral midbrain has been clearly demonstrated.

  13. Iliotibial band syndrome following hip arthroscopy: An unreported complication

    Directory of Open Access Journals (Sweden)

    Roberto Seijas

    2016-01-01

    Conclusions: This is a newly described observation within followup of hip arthroscopy. These findings may help orthopedic surgeons when planning rehabilitation after hip arthroscopy, including stretching exercises to prevent this syndrome.

  14. Fusobacterium necrophorum Pharyngitis Complicated by Lemierre’s Syndrome

    Directory of Open Access Journals (Sweden)

    Antonio Faraone

    2016-01-01

    Full Text Available We report the case of an 18-year-old woman who was referred to our outpatient clinic because of a 2-week history of sore throat, high fever, and neck tenderness unresponsive to a 7-day amoxicillin/clavulanic acid course. Infectious mononucleosis was initially suspected, but an extremely high value of procalcitonin and clinical deterioration suggested a bacterial sepsis, prompting the patient admission to our internal medicine ward. Blood cultures were positive for Fusobacterium necrophorum. CT scan detected a parapharyngeal abscess, a right internal jugular vein thrombosis, and multiple bilateral lung abscesses, suggesting the diagnosis of Lemierre’s syndrome. The patient was treated with a 2-week course of metronidazole and meropenem with a gradual clinical recovery. She was thereafter discharged home with metronidazole and amoxicillin/clavulanic acid for 14 days and a 3-month course of enoxaparin, experiencing an uneventful recovery. The present case highlights the importance of taking into consideration the Lemierre’s syndrome whenever a pharyngotonsillitis has a severe and unusual course.

  15. Nicolau syndrome following intramuscular benzathine penicillin

    Directory of Open Access Journals (Sweden)

    De Sousa R

    2008-01-01

    Full Text Available Nicolau syndrome (NS is a rare complication of an intramuscular injection characterized by severe pain, skin discoloration, and varying levels of tissue necrosis. The case outcomes vary from atrophic ulcers and severe pain to sepsis and limb amputation. We describe a case of a seven-year-old boy with diagnosis of NS after intramuscular benzathine penicillin injection to the ventrolateral aspect of the left thigh. Characteristic violaceous discoloration of skin and immediate injection site pain identified it as a case of NS. The case was complicated by rapid progression of compartment syndrome of the lower limb, proceeding to acute renal failure and death. Associated compartment syndrome can be postulated as a poor prognostic factor for NS.

  16. Neurological complications of dengue fever: Experience from a tertiary center of north India

    Directory of Open Access Journals (Sweden)

    Rajesh Verma

    2011-01-01

    Full Text Available Introduction: Dengue, an acute viral disease transmitted by Aedes mosquitoes, is highly endemic in many tropical and subtropical areas of the world. Neurological complications of dengue infection have been observed more frequently in the recent past and some studies highlighted varied neurological complications arising in the course of dengue illness. In this retrospective study, we report various neurological complications observed during the last 2 years in patients of dengue fever. Materials and Methods: The patients presenting with neurological complications with positive serology (IgM antibody for dengue infection were consecutively recruited from the Department of Neurology/Medicine from a tertiary center of Lucknow, India. These patients were subjected to a detailed clinical evaluation, laboratory assessment including blood count, hematocrit, coagulation parameters, biochemical assays, serology for dengue fever, enzyme-linked immunosorbent assay for human immunodeficiency virus and other relevant investigations. Results: Twenty-six patients with neurological complications associated with confirmed dengue infection were observed during the last 2 years. Eighteen of these patients were male. Of the 26 patients, 10 patients were suffering from brachial neuritis, four patients had encephalopathy, three patients were consistent with the diagnosis of Guillain Barre syndrome, three patients had hypokalemic paralysis associated with dengue fever and two patients had acute viral myositis. Opsoclonus-myoclonus syndrome was diagnosed in two patients, myelitis in one patient and acute disseminated encephalo-myelitis also in one patient. Conclusion: Dengue fever was associated with widespread neurological complications. Brachial neuritis and opsoclonus-myoclonus syndrome were observed for the first time in this study.

  17. [Clinical effect of high-frequency oscillatory ventilation combined with pulmonary surfactant in treatment of neonatal severe meconium aspiration syndrome complicated by pulmonary hemorrhage].

    Science.gov (United States)

    Huang, Jing; Lin, Xin-Zhu; Zheng, Zhi

    2016-11-01

    To study the clinical effect and safety of high-frequency oscillatory ventilation (HFOV) combined with pulmonary surfactant (PS) in the treatment of neonatal severe meconium aspiration syndrome (MAS) complicated by neonatal pulmonary hemorrhage (NPH). A total of 48 children with severe MAS complicated by NPH were enrolled, and a retrospective analysis was performed for the clinical effects of HFOV+PS (trial group, 25 children) and HFOV alone (control group, 23 children). The blood gas parameters, oxygenation index (OI), PaO 2 /FiO 2 (P/F) value, duration of pulmonary hemorrhage, ventilation time, length of hospital stay, incidence of complications, and outcome were compared between the two groups. At 6, 12, 24, and 48 hours after treatment, the trial group had significantly better PaO 2 , OI, and P/F value than the control group (Phemorrhage (P0.05). HFOV combined with PS can better improve oxygenation function and shorten the duration of NPH and ventilation time. Meanwhile, it does not increase the incidence of adverse events. Therefore, it is a safe and effective therapy.

  18. Dress syndrome with sepsis, acute respiratory distress syndrome and pneumomediastinum

    Directory of Open Access Journals (Sweden)

    Prabhas Prasun Giri

    2011-01-01

    Full Text Available Drug rash with eosinophilia and systemic symptoms (DRESS syndrome reflects a serious hypersensitivity reaction to drugs, and is characterized by skin rash, fever, lymph node enlargement, and internal organ involvement. So far, numerous drugs such as sulfonamides, phenobarbital, sulfasalazine, carbamazepine, and phenytoin have been reported to cause DRESS syndrome. We report a case of a 10-year-old girl who developed clinical manifestations of fever, rash, lymphadenopathy, hypereosinophilia, and visceral involvement (hepatitis and pneumonitis after taking phenobarbital for seizures, with subsequent development of sepsis, acute respiratory distress syndrome (ARDS and spontaneous air leak syndrome (pnemothorax and pneumomediastinum. She was put on steroids and various antibiotics and was ventilated, but ultimately succumbed to sepsis and pulmonary complications.

  19. [Contribution to Ehlers-Danlos syndrome (author's transl)].

    Science.gov (United States)

    Fuxa, G; Brandt, H P

    1975-02-01

    A case of Ehler-Danlos syndrome with the rare complication of ablatio retinae without myopia is described. The alterations of the eye be Ehlers-Danlos syndrome, which are available in literature are demonstrated.

  20. Cerebral venous thrombosis and secondary polycythemia in a case of nephrotic syndrome.

    Science.gov (United States)

    Nagaraju, Shankar Prasad; Bairy, Manohar; Attur, Ravindra Prabhu; Sambhaji, Charudutt Jayant

    2016-03-01

    Cerebral venous thrombosis (CVT) and polycythemia are considered as rare and life threatening complications of nephrotic syndrome. We report an unusual combination of both these complications in a case of nephrotic syndrome due to minimal change disease that was treated successfully. There was prompt and complete remission of nephrotic syndrome with steroid therapy, concurrent with complete resolution of polycythemia and CVT.

  1. Neurological complications following bariatric surgery

    Directory of Open Access Journals (Sweden)

    Yara Dadalti Fragoso

    2012-09-01

    Full Text Available OBJECTIVE: It was to report on Brazilian cases of neurological complications from bariatric surgery. The literature on the subject is scarce. METHOD: Cases attended by neurologists in eight different Brazilian cities were collected and described in the present study. RESULTS: Twenty-six cases were collected in this study. Axonal polyneuropathy was the most frequent neurological complication, but cases of central demyelination, Wernicke syndrome, optical neuritis, radiculits, meralgia paresthetica and compressive neuropathies were also identified. Twenty-one patients (80% had partial or no recovery from the neurological signs and symptoms. CONCLUSION: Bariatric surgery, a procedure that is continuously increasing in popularity, is not free of potential neurological complications that should be clearly presented to the individual undergoing this type of surgery. Although a clear cause-effect relation cannot be established for the present cases, the cumulative literature on the subject makes it important to warn the patient of the potential risks of this procedure.

  2. Prevention of infectious diseases in patients with Good syndrome.

    Science.gov (United States)

    Multani, Ashrit; Gomez, Carlos A; Montoya, José G

    2018-08-01

    Good syndrome is a profoundly immunocompromising condition with heterogeneous immune deficits characterized by the presence of thymoma, low-to-absent B-lymphocyte counts, hypogammaglobulinemia, and impaired cell-mediated immunity. Opportunistic infectious diseases associated with Good syndrome represent a diagnostic and therapeutic challenge, given their protean clinical manifestations. Although these infectious complications have been reviewed in prior publications, recommendations regarding their prevention have been lacking. Good syndrome usually occurs in adult patients between the ages of 40 and 70 years. Immunologically, it is characterized by low or absent peripheral blood B lymphocytes, hypogammaglobulinemia, and variable defects in cell-mediated immunity including low CD4 T counts, inverted CD4:CD8 T-lymphocyte ratio, and reduced T-lymphocyte mitogen proliferative responses. Patients with Good syndrome are susceptible to a variety of infectious diseases, of which the most common are recurrent bacterial sinopulmonary infections, mucocutaneous candidiasis, and CMV tissue-invasive disease. Preventive guidelines including targeted antimicrobial prophylaxis and vaccination strategies can mitigate infectious complications in patients with Good syndrome. Immunological deficits and infectious complications in Good syndrome have been described for over 60 years. Further research is needed to elucidate its exact pathogenesis and define the mechanistic relationship between thymoma and hypogammaglobulinemia. However, tailored prophylactic strategies can be recommended for patients with Good syndrome.

  3. Prevention and Management of Refeeding Syndrome

    OpenAIRE

    Andika Indrarespati; Kaka Renaldi

    2016-01-01

    Refeeding Syndrome is a syndrome which occurs as a result of food administration in poorly nourished individuals. In this syndrome, there are wide range of biochemical alterations, clinical manifestations, and complications, starting from mild (asymptomatic) to severe (death). This syndrome was initially proposed in 1950s; however, there is still no agreement for its clear definition, causing clinicians to be less aware and tend to overlook this condition. Clinical manifestations which usuall...

  4. Recognizing and preventing refeeding syndrome.

    Science.gov (United States)

    Adkins, Susan M

    2009-01-01

    Refeeding syndrome is an uncommon but potentially fatal phenomenon that can occur in patients receiving parenteral, enteral, or oral feedings after a period of sustained malnutrition or starvation. This syndrome is characterized by hypophosphatemia, hypokalemia, and hypomagnesemia. The purpose of this article was to bring an acute awareness of refeeding syndrome to the critical care nurse. The recognition, pathogenesis, clinical manifestations, potential life threatening complications, and treatment are presented.

  5. An observational study of complications in chickenpox with special reference to unusual complications in an apex infectious disease hospital, Kolkata, India

    Directory of Open Access Journals (Sweden)

    A K Kole

    2013-01-01

    Full Text Available Background: Chickenpox can cause serious complications and even death in persons without any risk factors. Aims: To observe the different complications with special reference to unusual complications of chickenpox and their outcomes. Materials and Methods: The present study was a prospective observational study where 300 patients suffering from chickenpox were evaluated with special reference to unusual complications and outcomes. Results: The usual complications of chickenpox commonly observed were acute hepatitis in 30 (10% and cerebellar ataxia in 22 patients (7.3%, whereas common unusual complications were acute pancreatitis in 45 (15%, hemorrhagic rash in 10 (3.3%, Guillain-Barrι syndrome in 4 (1.3%, disseminated intravascular coagulation in 4 (1.3%, necrotizing fasciitis in 4 (1.3%, and acute renal failure in 3 patients (1%. It had been observed that most of these unusual complications occurred in patients without any risk factor. A total of 18 patients (6% died in this study and of them 12 patients (4% died due to unusual complications. Conclusions: Compulsory childhood varicella vaccination including vaccination of risk groups and susceptible individuals are all essential to reduce the incidence of chickenpox, associated complications, and subsequent death.

  6. Broken Heart Syndrome – An intra operative complication

    Directory of Open Access Journals (Sweden)

    Zara Wani

    2018-03-01

    Full Text Available We report a case of Broken Heart Syndrome in a 56 year old Postmenopausal woman suffered while undergoing simple biopsy procedure for vocal cord polyp that lead to physical, mental and financial burden both for the patient as well as the doctors. A team of cardiologists based on clinical and echocardiographic findings made the diagnosis of this case.

  7. Can femoral dialysis catheter insertion cause a life threatening complication?

    Directory of Open Access Journals (Sweden)

    Nurkay Katrancıoğlu

    2014-09-01

    Full Text Available Venous catheter (VC insertion may be necessary for the patients with renal failure facing vascular access problem. Femoral VCs are commonly used for their lower complication rates especially in emergency clinics. The incidence of bleeding associated with VC is reported 0.5-1.6%, however, life threatening hemorrhage and complications requiring surgical intervention are very rare. In this manuscript, we aimed to present a case with hemolytic uremic syndrome complicated with retroperitoneal hematoma after femoral VC insertion. J Clin Exp Invest 2014; 5 (3: 472-474

  8. Wolff-Parkinson-White syndrome in infants.

    Science.gov (United States)

    Hermosura, Tisha; Bradshaw, Wanda T

    2010-01-01

    Wolff-Parkinson-White (WPW) syndrome is a ventricular preexcitation that presents as supraventricular tachycardia. Health care professionals can attain optimal results in caring for infants with WPW syndrome by understanding both its pathophysiology and proper management to prevent and treat complications associated with it. This article reviews the prevalence, pathophysiology, clinical manifestations, diagnostic modalities, assessment, and management of WPW syndrome.

  9. Cerebral venous thrombosis and secondary polycythemia in a case of nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Shankar Prasad Nagaraju

    2016-01-01

    Full Text Available Cerebral venous thrombosis (CVT and polycythemia are considered as rare and life threatening complications of nephrotic syndrome. We report an unusual combination of both these complications in a case of nephrotic syndrome due to minimal change disease that was treated successfully. There was prompt and complete remission of nephrotic syndrome with steroid therapy, concurrent with complete resolution of polycythemia and CVT.

  10. Measles and its Complications | Wesley | South African Medical ...

    African Journals Online (AJOL)

    The radiological features that accompany measles are reviewed. Changes due to viral infection may be complicated by those secondary to bacterial superinfection. The syndrome of relapsing pulmonary infection after measles is discussed, and studies on the pathogenesis are presented. S. Afr. Med. J., 48, 1001 (1974) ...

  11. Two cases of Budd-Chiari syndrome complicated by hepatocellular carcinoma. Hemodynamic study with computed tomography and venacavography

    Energy Technology Data Exchange (ETDEWEB)

    Takayasu, Kenichi; Moriyama, Noriyuki; Suzuki, Masao [National Cancer Center, Tokyo (Japan). Hospital

    1982-12-01

    Two patients with Budd-Chiari syndrome complicated by hepatocellular carcinoma in whom CT and inferior venacavography were carried out are described. The imaging study with CT disclosed: 1) calcified inferior vena cava (IVC) at the level of the diaphragm and lack of enhancement of the IVC and hepatic veins after contrast enhancement, 2) markedly dilated inferior right hepatic vein, 3) marked compensatory enlargement of the caudate lobe, 4) icile-like thrombus extending caudally in the IVC and dilatation of that portion of the IVC, and 5) enlarged and dilated azygos, hemiazygos and anterior longitudinal venous trunk of the internal vertebral venous plexus, and with cavography, 1) the inferior right hepatic vein as a venous outlet to maintain the liver functions, and 2) occlusion of the right and left hepatic vein openings and a communicating vein between the left hepatic and caudate lobe vein.

  12. Pregnancy outcome in a woman with prune belly syndrome.

    Science.gov (United States)

    Hillman, R Tyler; Garabedian, Matthew James; Wallerstein, Robert J

    2012-11-30

    Prune belly syndrome is a rare congenital syndrome that primarily affects male fetuses. Affected men are universally infertile; however, there is a paucity of information published on the reproductive potential of affected women. Pregnancy outcomes in affected women have not been described in the literature. We describe the case of pregnancy in an affected woman. Her pregnancy progressed without complication. Her fetus had no stigmata of the syndrome. Her labour and delivery were, however, complicated by a prolonged second stage of labour and need for vacuum-assisted vaginal delivery.

  13. [Pulmonary complications of malaria: An update].

    Science.gov (United States)

    Cabezón Estévanez, Itxasne; Górgolas Hernández-Mora, Miguel

    2016-04-15

    Malaria is the most important parasitic disease worldwide, being a public health challenge in more than 90 countries. The incidence of pulmonary manifestations has increased in recent years. Acute respiratory distress syndrome is the most severe form within the pulmonary complications of malaria, with high mortality despite proper management. This syndrome manifests with sudden dyspnoea, cough and refractory hypoxaemia. Patients should be admitted to intensive care units and treated with parenteral antimalarial drug treatment and ventilatory and haemodynamic support without delay. Therefore, dyspnoea in patients with malaria should alert clinicians, as the development of respiratory distress is a poor prognostic factor. Copyright © 2016 Elsevier España, S.L.U. All rights reserved.

  14. [Anesthetic management for surgery of giant coronary aneurysms complicated with Churg-Strauss syndrome].

    Science.gov (United States)

    Kido, Koji; Tokuda, Rui; Suzuki, Tomofumi; Hanashiro, Ako; Kobashigawa, Teruyo; Mayama, Takashi; Kamikawa, Michie

    2014-04-01

    Few cases of Churg-Strauss syndrome (CSS) complicated by giant coronary aneurysms (CAs)have been reported thus far. We report a case of CSS in a 60-year-old man who underwent surgery for giant CAs, and was managed with anesthetics. The patient developed acute myocardial infarction, and was diagnosed with giant CAs in the right coronary artery (RCA, 11 cm) and circumflex artery (3 cm). The CA in RCA was communicating with the right ventricle. He had a history of pericardiectomy for pericarditis caused by the CSS and developed thrombocytopenia due to consumptive coagulopathy within the CAs. An operation, including ligation and excision of the CAs, and coronary artery bypass grafting was performed under general anesthesia and cardiopulmonary bypass. There was massive hemorrhage followed by hemodynamic instability while detaching the tight pericardial adhesion and fragile surface of the CAs. Massive transfusion was required along with inotropes administration and intraaortic balloon support. In this case, determination of the appropriate surgical timing was difficult because symptoms of the CSS became worse followed by rapid enlargement of the CAs, myocardial infarction, and thrombocytopenia. Steroids were administered for treating CSS, and the blood transfusion was sufficient. However, it was difficult to control the hemorrhage and maintain hemodynamic stability.

  15. [Refeeding syndrome : Pathophysiology, risk factors, prevention, and treatment].

    Science.gov (United States)

    Wirth, R; Diekmann, R; Janssen, G; Fleiter, O; Fricke, L; Kreilkamp, A; Modreker, M K; Marburger, C; Nels, S; Pourhassan, M; Schaefer, R; Willschrei, H-P; Volkert, D

    2018-04-01

    Refeeding syndrome is a life-threatening complication that may occur after initiation of nutritional therapy in malnourished patients, as well as after periods of fasting and hunger. Refeeding syndrome can be effectively prevented and treated if its risk factors and pathophysiology are known. The initial measurement of thiamine level and serum electrolytes, including phosphate and magnesium, their supplementation if necessary, and a slow increase in nutritional intake along with close monitoring of serum electrolytes play an important role. Since refeeding syndrome is not well known and the symptoms can be extremely heterogeneous, this complication is poorly recognized, especially against the background of severe disease and multimorbidity. This overview aims to summarize the current knowledge and increase awareness about refeeding syndrome.

  16. Identification of a Monoclonal Antibody That Attenuates Antiphospholipid Syndrome-Related Pregnancy Complications and Thrombosis

    Science.gov (United States)

    Mineo, Chieko; Lanier, Lane; Jung, Eunjeong; Sengupta, Samarpita; Ulrich, Victoria; Sacharidou, Anastasia; Tarango, Cristina; Osunbunmi, Olutoye; Shen, Yu-Min; Salmon, Jane E.; Brekken, Rolf A.; Huang, Xianming; Shaul, Philip W.

    2016-01-01

    In the antiphospholipid syndrome (APS), patients produce antiphospholipid antibodies (aPL) that promote thrombosis and adverse pregnancy outcomes. Current therapy with anticoagulation is only partially effective and associated with multiple complications. We previously discovered that aPL recognition of cell surface β2-glycoprotein I (β2-GPI) initiates apolipoprotein E receptor 2 (apoER2)-dependent signaling in endothelial cells and in placental trophoblasts that ultimately promotes thrombosis and fetal loss, respectively. Here we sought to identify a monoclonal antibody (mAb) to β2-GPI that negates aPL-induced processes in cell culture and APS disease endpoints in mice. In a screen measuring endothelial NO synthase (eNOS) activity in cultured endothelial cells, we found that whereas aPL inhibit eNOS, the mAb 1N11 does not, and instead 1N11 prevents aPL action. Coimmunoprecipitation studies revealed that 1N11 decreases pathogenic antibody binding to β2-GPI, and it blocks aPL-induced complex formation between β2-GPI and apoER2. 1N11 also prevents aPL antagonism of endothelial cell migration, and in mice it reverses the impairment in reendothelialization caused by aPL, which underlies the non-thrombotic vascular occlusion provoked by disease-causing antibodies. In addition, aPL inhibition of trophoblast proliferation and migration is negated by 1N11, and the more than 6-fold increase in fetal resorption caused by aPL in pregnant mice is prevented by 1N11. Furthermore, the promotion of thrombosis by aPL is negated by 1N11. Thus, 1N11 has been identified as an mAb that attenuates APS-related pregnancy complications and thrombosis in mice. 1N11 may provide an efficacious, mechanism-based therapy to combat the often devastating conditions suffered by APS patients. PMID:27463336

  17. Cochlear implantation in Pendred syndrome and non-syndromic enlarged vestibular aqueduct – clinical challenges, surgical results, and complications

    DEFF Research Database (Denmark)

    Mey, Kristianna; Bille, Michael; Cayé-Thomasen, Per

    2016-01-01

    , and post-operative complications were retrieved. RESULTS: Complications occurred in 36% of implantations; 5% hereof major complications. Gushing/oozing from the cochleostoma occurred in 10% of implantations and was related to transient, but not prolonged post-operative vertigo. CONCLUSION: Intra......-operative risks of gushing/oozing and post-operative vertigo are the primary clinical issues in PS/NSEVA patients regarding CI. Nonetheless, the surgical success rate is high and the major complication rate is low; similar to studies of unselected series of CI recipients....

  18. Frequency of neonatal complications after premature delivery

    Directory of Open Access Journals (Sweden)

    Gordana Grgić

    2013-04-01

    Full Text Available Introduction: Preterm delivery is the delivery before 37 weeks of gestation are completed. The incidence of preterm birth ranges from 5 to 15%. Aims of the study were to determine the average body weight, Apgar score after one and five minutes, and the frequency of the most common complications in preterminfants.Methods: The study involved a total of 631 newborns, of whom 331 were born prematurely Aims of this study were to (24th-37th gestational weeks-experimental group, while 300 infants were born in time (37-42 weeks of gestation-control group.Results: Average body weight of prematurely born infants was 2382 grams, while the average Apgar score in this group after the fi rst minute was 7.32 and 7.79 after the fifth minute. The incidence of respiratory distress syndrome was 50%, intracranial hemorrhage, 28.1% and 4.8% of sepsis. Respiratory distresssyndrome was more common in infants born before 32 weeks of gestation. Mortality of premature infants is present in 9.1% and is higher than that of infants born at term.Conclusions: Birth body weight and Apgar scores was lower in preterm infants. Respiratory distress syndrome is the most common fetal complication of prematurity. Intracranial hemorrhage is the second most common complication of prematurity. Mortality of premature infants is higher than the mortality of infants born at term birth.

  19. Neurologic Complications of Transplantation.

    Science.gov (United States)

    Dhar, Rajat

    2018-02-01

    Neurologic disturbances including encephalopathy, seizures, and focal deficits complicate the course 10-30% of patients undergoing organ or stem cell transplantation. While much or this morbidity is multifactorial and often associated with extra-cerebral dysfunction (e.g., graft dysfunction, metabolic derangements), immunosuppressive drugs also contribute significantly. This can either be through direct toxicity (e.g., posterior reversible encephalopathy syndrome from calcineurin inhibitors such as tacrolimus in the acute postoperative period) or by facilitating opportunistic infections in the months after transplantation. Other neurologic syndromes such as akinetic mutism and osmotic demyelination may also occur. While much of this neurologic dysfunction may be reversible if related to metabolic factors or drug toxicity (and the etiology is recognized and reversed), cases of multifocal cerebral infarction, hemorrhage, or infection may have poor outcomes. As transplant patients survive longer, delayed infections (such as progressive multifocal leukoencephalopathy) and post-transplant malignancies are increasingly reported.

  20. Malignancy in Noonan syndrome and related disorders.

    Science.gov (United States)

    Smpokou, P; Zand, D J; Rosenbaum, K N; Summar, M L

    2015-12-01

    Noonan syndrome (NS) and related disorders, such as NS with multiple lentigines (formerly called LEOPARD syndrome), cardiofaciocutaneous syndrome, and Costello syndrome, constitute an important group of developmental malformation syndromes with variable clinical and molecular features. Their underlying pathophysiologic mechanism involves dysregulation of the Ras/mitogen-activated protein kinase signaling pathway, an essential mediator of developmental and growth processes in the prenatal and postnatal setting. Malignant tumor development is an important complication encountered in other RASopathies, such as neurofibromatosis type 1, but the neoplastic risks and incidence of malignant tumors are less clearly defined in NS and related disorders of the Noonan spectrum. Malignant tumor development remains an important complication variably seen in the RASopathies and, thus, a clear understanding of the underlying risks is essential for appropriate clinical care in this patient population. This review discusses previously published reports of malignancies in individuals with RASopathies of the Noonan spectrum. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  1. Ehlers–Danlos Syndrome in an Adult Woman: A Hidden Syndrome

    Directory of Open Access Journals (Sweden)

    Diana Spinelli

    2014-05-01

    Full Text Available Ehlers–Danlos syndrome is a rare disease and a diagnostic challenge. This case report serves to remind the clinician that it is important to identify all affected patients in order to prevent complications.

  2. [Peripubertal ovarian cyst torsion as an early complication of undiagnosed polycystic ovarian syndrome].

    Science.gov (United States)

    Ságodi, László; Schmidt, Ildikó; Vámosi, Ildikó; Barkai, László

    2013-01-20

    The aim of the authors is to present two cases which raise the possibility of an association between polycystic ovarian syndrome/hyperandrogenism and ovarian cyst torsion in peripubertal girls. Androgen excess may cause more frequently ovarian cyst formation in premenarcheal or young adolescents with undiagnosed polycystic ovarian syndrome than in adults. The authors recommend that polycystic ovarian syndrome as well as late onset congenital adrenal hyperplasia should be considered in peripubertal adolescents with ovarian cyst torsion. In case polycystic ovarian syndrome is confirmed, adequate management according to age and pubertal development of the patients should be commenced.

  3. Management of liver complications in haemoglobinopathies

    Directory of Open Access Journals (Sweden)

    Pierre Brissot

    2014-12-01

    Full Text Available Liver complications in haemoglobinopathies (thalassaemia and sickle cell disease are due to several factors, dominated (beside chronic viral infections, not considered here by chronic iron overload, biliary obstruction and venous thrombosis. Whereas the latter two factors can cause acute hepatic syndromes, all three mechanisms - when becoming chronic- can produce fibrosis and cirrhosis and even, in thalassaemia, hepatocellular carcinoma. These chronic hepatic complications are an indirect consequence of the significant improvement in life expectancy due to the overall amelioration of disease management. The diagnostic approach has benefited from non invasive (biochemical and imaging approaches which have considerably reduced the indication of liver biopsy. The therapeutic management involves relatively efficient curative medical, endoscopic or surgical methods, but should rest primarily on preventive measures focused on the haematological causative factors but also on hepatic co-morbidities. This chapter will focus on hepatic complications in thalassaemia and sickle cell disease (SCD, without considering the complications related to virus B or C infections which will be described in another chapter.

  4. Complications of cranioplasty after decompressive craniectomy

    Directory of Open Access Journals (Sweden)

    Maša Glišović

    2015-04-01

    Full Text Available Cranioplasty is a surgical repair of a defect or deformity of a skull with the use of autologous bone or synthetic materials.[4] It usually follows decompressive craniectomy, which is a commonly practiced neurosurgical intervention in patients with raised intracranial pressure unresponsive to other forms of treatment.[1] There are many conditions that may lead to intracranial hypertension, and the goal is to avoid brain necrosis caused by compartment pressure syndrome.[2] Consequently, the extensive use of decompressive craniectomy directly results in more cranioplasties, which sometimes present with unwanted complications.[5] Generally, the occurence of cranioplasty complications is between 16% and 34%.[3] Because of the many indications for craniectomy based on clinical data that speak in its favour, if will probably remain a relatively common neurosurgical intervention also in the future. The frequency of decompressive craniectomy and consequently of cranioplasty requires awareness of the many potential postoperative complications and understanding of its evolution. This article is a review of pathophysiological mechanisms after decompressive craniectomy and cranioplasty, of its complications and factors that potentially contribute to their occurence.

  5. A CASE OF METABOLIC SYNDROME

    OpenAIRE

    Khoo Ee Ming; Rabia Khatoon

    2006-01-01

    This case report illustrates a 40-year-old woman who presented with chest discomfort that was subsequently diagnosed to have metabolic syndrome. Metabolic syndrome is a common condition associated with increased cardiovascular morbidity and mortality. As primary care providers, we should be detect this condition early, intervene and prevent appropriately before complications occur.

  6. Obstetric antiphospholipid syndrome.

    Science.gov (United States)

    Esteve-Valverde, E; Ferrer-Oliveras, R; Alijotas-Reig, J

    2016-04-01

    Obstetric antiphospholipid syndrome is an acquired autoimmune disorder that is associated with various obstetric complications and, in the absence of prior history of thrombosis, with the presence of antiphospholipid antibodies directed against other phospholipids, proteins called cofactors or PL-cofactor complexes. Although the obstetric complications have been related to the procoagulant properties of antiphospholipid antibodies, pathological studies of human placenta have shown the proinflammatory capacity of antiphospholipid antibodies via the complement system and proinflammatory cytokines. There is no general agreement on which antiphospholipid antibodies profile (laboratory) confers the greatest obstetric risk, but the best candidates are categories I and IIa. Combined treatment with low doses of aspirin and heparin achieves good obstetric and maternal outcomes. In this study, we also review the therapeutic possibilities in refractory cases, although the likelihood of progressing to other autoimmune diseases is low. We briefly comment on incomplete obstetric antiphospholipid syndrome, also known as antiphospholipid antibody-mediated pregnancy morbidity syndrome. Copyright © 2015 Elsevier España, S.L.U. y Sociedad Española de Medicina Interna (SEMI). All rights reserved.

  7. Extranodal marginal zone B cell lymphoma: An unexpected complication in children with Sjögren's syndrome.

    Science.gov (United States)

    Collado, Paz; Kelada, Aml; Cámara, Maria; Zeft, Andrew; Flagg, Aron

    2017-03-08

    Sjögren's syndrome (SS) is a systemic autoimmune disease characterized by the infiltration of lymphocytes into exocrine glands, resulting in the typical sicca symptoms. Unlike adults, primary SS is a very rare condition in childhood, and the risk of malignancy in juvenile SS (JSS) has not been defined. We report the detection of extranodal marginal zone B-cell lymphoma (EMZL) occurring in two children with SS. Fine needle aspiration of the salivary glands (SG) showed nonspecific findings that led to delayed diagnosis of SS. The diagnosis of B-cell lymphoma associated with JSS was based on morphologic and immunohistochemical staining done during the biopsy. To highlight awareness of EMZL as a timely and appropriate update of an unusual complication in children with SS. Copyright © 2017 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  8. [Infectious complications in patients undergoing a heart transplant].

    Science.gov (United States)

    Bouza, E; Muñoz, P

    1995-01-01

    Infectious complications are, with rejection, the main cause of morbidity and mortality in heart transplantation recipients. Adequate management of these patients requires an adequate knowledge of risk factors, of most common infectious syndromes and of relevant microorganism, as long as of their resistance pattern. Among the first group, we may mention lower respiratory tract infections, mediastinitis and meningitis, and among the microorganisms, Citomegalovirus, Aspergillus and Toxoplasma gondii. The impressive development of diagnostical techniques and of prophylactic and therapeutical possibilities suggest the convenience of a multidisciplinar approach to these complications.

  9. A Rare Manifestation of Uveitis-glaucoma-hyphema Syndrome.

    Science.gov (United States)

    Sousa, David Cordeiro; Leal, Inês; Faria, Mun Yueh; Pinto, Luís Abegão

    2016-01-01

    To report a case of a patient who developed uveitis-glaucoma-hyphema (UGH) syndrome after an uneventful cataract surgery and to discuss risk factors, diagnostic challenges, management options, and clinical implications. Uveitis-glaucoma-hyphema syndrome is a rare but potentially serious cataract surgery complication. Clinical manifestations include increased intraocular pressure (IOP), anterior chamber inflammation, and recurrent hyphema or microhyphema. Uveitis-glaucoma-hyphema Plus syndrome also includes accompanying vitreous hemorrhage. Although classically associated with rigid anterior chamber intraocular lenses (lOLs), cases of malpositioning and subluxated posterior chamber lOLs have also been described as possible triggers. We report a case of a 70-year-old Caucasian man who developed UGH Plus syndrome after an uneventful cataract surgery with an lOL implanted in the capsular bag. During postoperative follow-up, persistent intraocular inflammation, increased IOP, hyphema, and vitreous hemorrhage were consistent with this diagnosis. Slit-lamp examination demonstrated progressive localized iris atrophy, compatible with chafing of the posterior iris by the IOL haptic as the trigger for UGH syndrome. A pars plana vitrectomy was performed and a retropupillary intraocular lens was implanted. No further complications occurred during follow-up. Given the increasing prevalence of single-piece lOLs implanted in the capsular bag, it is important to recognize UGH syndrome as a rare but potentially serious complication. How to cite this article: Sousa DC, Leal I, Faria MY, Pinto LA. A Rare Manifestation of Uveitis-glaucoma-hyphema Syndrome. J Curr Glaucoma Pract 2016;10(2):76-78.

  10. Organic brain syndrome

    Science.gov (United States)

    ... DRUG AND ALCOHOL-RELATED CONDITIONS Alcohol withdrawal state Intoxication from drug or alcohol use Wernicke-Korsakoff syndrome ( ... Late-stage syphilis Complications of cancer can also lead to neurocognitive disorder. Other conditions that may mimic ...

  11. Unusual paraneoplastic syndromes of breast carcinoma: a combination of cerebellar degeneration and Lambert-Eaton Myasthenic Syndrome.

    LENUS (Irish Health Repository)

    Romics, L

    2011-06-01

    Paraneoplastic neurological disorders are rare complications of breast carcinoma. Lambert-Eaton Myasthenic Syndrome (LEMS) is most commonly associated with small cell lung cancer. However, a combination of LEMS and subacute cerebellar degeneration as paraneoplastic syndromes is extremely rare, and has never been described in association with breast cancer.

  12. Overview of complications secondary to tongue and lip piercings.

    Science.gov (United States)

    Maheu-Robert, Léo-François; Andrian, Elisoa; Grenier, Daniel

    2009-07-01

    In recent years, intraoral and perioral piercings have grown in popularity among teenagers and young adults. This is of concern to dental and medical professionals because of the risks and complications for oral, dental and general health. The risks and complications associated with tongue and lip piercings range from abnormal tooth wear and cracked tooth syndrome to gingival recession and systemic infections. In this report, we provide an overview of possible problems associated with oral piercings that may be encountered by dentists.

  13. Antiphospholipid syndrome and kidney disease.

    Science.gov (United States)

    Bienaimé, Frank; Legendre, Christophe; Terzi, Fabiola; Canaud, Guillaume

    2017-01-01

    The antiphospholipid syndrome is a common autoimmune disease caused by pathogenic antiphospholipid antibodies, leading to recurrent thrombosis and/or obstetrical complications. Importantly for nephrologists, antiphospholipid antibodies are associated with various renal manifestations including large renal vessel thrombosis, renal artery stenosis, and a constellation of intrarenal lesions that has been termed antiphospholipid nephropathy. This last condition associates various degrees of acute thrombotic microangiopathy, proliferative and fibrotic lesions of the intrarenal vessels, and ischemic modifications of the renal parenchyma. The course of the disease can range from indolent nephropathy to devastating acute renal failure. The pejorative impact of antiphospholipid antibody-related renal complication is well established in the context of systemic lupus erythematous or after renal transplantation. In contrast, the exact significance of isolated antiphospholipid nephropathy remains uncertain. The evidence to guide management of the renal complications of antiphospholipid syndrome is limited. However, the recent recognition of the heterogeneous molecular mechanisms underlying the progression of intrarenal vascular lesions in antiphospholipid syndrome have opened promising tracks for patient monitoring and targeted therapeutic intervention. Copyright © 2016 International Society of Nephrology. Published by Elsevier Inc. All rights reserved.

  14. [Retrospective analysis of risk factors in 900 patients with ischemic cerebral stroke of wind-phlegm collateral obstruction syndrome and qi deficiency blood stasis syndrome in Wuhan District].

    Science.gov (United States)

    Qiu, Xin; Wang, Kai-xin; Chen, Guo-hua

    2011-11-01

    To analyze the correlation between risk factors and ischemic cerebral stroke of wind-phlegm collateral obstruction syndrome and qi deficiency blood stasis syndrome. Totally 900 patients of the two syndrome types were recruited. Risk factors correlated to ischemic cerebral stroke such as gender, age, time of onset, site of infarction, tongue proper, tongue fur, pulse picture, hypertension, diabetes, past stroke history, hyperlipidemia, hematocrit, smoking, drinking, genetic factor, blood type, complications were analyzed using Chi-square test and non-conditional Logistic regression analysis. Statistical significance existed between the two syndrome types in age (X2 = 8.2392, P = 0.0413), hyperlipidemia (X2 = 4.8386, P = 0.0278), tongue proper (X2 = 7.9470, P = 0.0048), and tongue fur (X2 = 4.3298, P = 0.0375). Statistical significance existed between the two syndrome types in hyperlipidemia, tongue proper, and tongue fur, and their OR value was 0.699 (P = 0.0282), 0.332 (P =0.0071), and 0.667 (P = 0.0382) respectively. The OR value of the past stroke history was 3.226 (P = 0.0314), that of complications 0.203 (P = 0.0705), and that of anterior circulation infarction 0.214 (P = 0.0098). Among different ages groups, the constituent ratio of qi deficiency blood stasis syndrome was obviously higher than that of wind-phlegm collateral obstruction syndrome. Besides, patients of qi deficiency blood stasis syndrome were liable to suffer from hyperlipidemia, anterior circulation infarction, and complications. The age, blood lipid levels, site of infarction, complications are closely correlated with Chinese syndrome types of ischemic cerebral stroke, which can provide objective indices for typing ischemic cerebral stroke.

  15. Cushing's syndrome in pregnancy.

    Science.gov (United States)

    Nassi, Rossella; Ladu, Cristina; Vezzosi, Chiara; Mannelli, Massimo

    2015-02-01

    Cushing's syndrome is a rare condition in the general population and is even less common during pregnancy with only a few cases reported in literature. The diagnosis of Cushing's syndrome may be difficult during pregnancy because the typical features of the disorder and pregnancy may overlap. However, Cushing's syndrome results in increased fetal and maternal complications, and diagnosis and treatment are critical. This report describes a case of 26-year-old female at the 19th week of pregnancy with symptoms and signs of hypercortisolism, where ACTH-independent Cushing's syndrome was diagnosed and treated by robotic laparoscopic adrenalectomy at the 21th week of gestation.

  16. Gradenigo's syndrome--surgical management in a child.

    Science.gov (United States)

    Humayun, Hassan Nabeel; Akhtar, Shabbir; Ahmed, Shakeel

    2011-04-01

    Otits media is a common problem. Some of its complications that were seen frequently in the preantibiotic era are rare today. We report a case of an 8 year boy who presented with earache, retro-orbital pain and diplopia secondary to a sixth nerve palsy--Gradenigo's syndrome. In this syndrome infection from the middle ear spreads medially to the petrous apex of the temporal bone. Work-up includes CT scan of the temporal bones. Timely management with intravenous antibiotics (+ surgery) is needed to prevent intra-cranial complications.

  17. An unusual complication after craniofacial surgery for Apert syndrome

    Directory of Open Access Journals (Sweden)

    Abhay A Lune

    2014-01-01

    A case of Apert syndrome who had undergone craniofacial surgery elsewhere 4 years back presented to us with purulent discharge near the lateral orbital margin of right orbit, watering and redness of the right eye. He had telltale signs of this syndrome in the form of skull deformities such as brachycephaly, frontal bony prominence, mid-face hypoplasia, proptosis and syndactyly of both hands and feet. There was a surgical scar of previous craniofacial surgery over the bi-coronal region. He had a discharging granuloma over the lateral orbital margin and the adjacent lower eyelid had developed cicatricial ectropion. X-ray and computed tomography scan orbit confirmed the clinical suspicion of osteomyelitis of the underlying zygomatic bone at the site of miniplate and screw fixation of the earlier surgery. He was treated with excision of granuloma and extraction of loose screw and infected miniplate while ectropion was corrected by rotation advancement of temporal skin flap.

  18. [Economic impact of etanercept and adalimumab biosimilars on hospitals scale covered by PharmAlp'Ain, a hospitals grouping of orders for health products].

    Science.gov (United States)

    Berreur, B; Guerin, F; Christophe, B; Limido, G; Paubel, P

    2018-01-01

    To evaluate the economic impact of future prescriptions of etanercept and adalimumab biosimilars at the territorial scale covered by PharmAlp'Ain, a hospitals grouping of orders for health products. Determination of the number and status of patients (naive or in continuation of treatment) from the National Database "Datamart de Consommation Inter-Régimes" of health insurance, concerned by a dispensation in a pharmacy of etanercept or adalimumab in 2015. Calculation of potential savings in case of biosimilar requirements according to 3 hypotheses: 63% (rate observed in a previous study) of initiations are treated with biosimiliaries and the others by princeps (H 1 ); all initiations under biosimilars and continuation therapy with the princeps (H 2 ) or all patients are treated with biosimilars (H 3 ). The annual savings are estimated at 237,000 € with the H 1 hypothesis. In the case of H 2 , the expected savings would be 376,200 € per year. In the case of H 3 , savings for the community could reach almost 1,282,800 € per year. The arrival of biosimilars allows significant savings for medicines market. According to the French recommendations in 2016, the expected savings are between the H 1 and H 2 hypothesis. The rate of penetration of biosimilars depends on many factors such as the involvement of health professionals, patient adherence, or health authority recommendations. Copyright © 2017 Académie Nationale de Pharmacie. Published by Elsevier Masson SAS. All rights reserved.

  19. Neonatal bartter syndrome

    International Nuclear Information System (INIS)

    Parkash, J.; Salat, S. M.; Khan, I.A.

    2006-01-01

    A pre-term baby girl was born following a pregnancy complicated by severe polyhydramnios at a gestational age of 36 weeks. She was initially suffering from respiratory distress consistent with idiopathic respiratory distress syndrome, and altered electrolyte imbalance with hyponatremia, hypokalemia and hypochloremic metabolic alkalosis. However, during the third week of life when she had dehydration along with significant electrolyte imbalance, Bartter's syndrome was considered which was supported by findings of high renin and aldosterone levels. Treatment was done by correction of electrolytes and dehydration along with indomethacin. The drug was well tolerated. The infant showed correction of electrolyte imbalance. The features of this case suggest an extreme form of Bartter's syndrome presenting from the early days of life. The syndrome is reported because of it's rarity and alerts pediatricians to the antenatal and neonatal variant of Bartter's syndrome. (author)

  20. Fahr Syndrome Unknown Complication: Overactive Bladder

    Directory of Open Access Journals (Sweden)

    Devrim Tuglu

    2014-01-01

    Full Text Available A 38-year-old male patient was admitted to our outpatient department because of frequency and urgency incontinence. During evaluation it was detected that the patient was suffering from frequency which was progressive for one year, feeling of incontinence, and urgency incontinence. There was no urologic pathology detected in patient’s medical and family history. Neurologic consultation was requested due to his history of boredom, reluctance to do business, balance disorders, and recession for about 3 years. Brain computerized tomography (CT scan revealed that amorphous calcifications were detected in the bilaterally centrum semiovale, basal ganglia, capsula interna, thalami, mesencephalon, pons and bulbus, and the bilateral cerebellar hemispheres. We have detected spontaneous neurogenic detrusor overactivity without sphincter dyssynergia after evaluating the voiding diary, cystometry, and pressure flow study. We consider the detrusor overactivity which occurred one year after the start of the neurological symptoms as the suprapontine inhibition and damage in the axonal pathways in the Fahr syndrome.

  1. Complications of allogeneic hematopoietic stem cell transplantation.

    Science.gov (United States)

    Arnaout, Karim; Patel, Nihar; Jain, Maneesh; El-Amm, Joelle; Amro, Farah; Tabbara, Imad A

    2014-08-01

    Infection, graft-versus-host disease (GVHD), and to a lesser extent sinusoidal obstructive syndrome (SOS) represent the major causes of morbidity and mortality in patients undergoing allogeneic hematopoietic stem cell transplantation (AHSCT). During the last decade, progress in prevention and treatment of these complications led to improvement in the outcome of these patients. Despite the fact that nonmyeloablative regimens have been increasingly used in elderly patients and in patients with co-morbidities, the nonrelapse related mortality remains a challenge and long-term follow-up is required. The objective of this manuscript is to provide an updated concise review of the complications of AHSCT and of the available treatment interventions.

  2. Acute respiratory failure following ovarian hyperstimulation syndrome

    Directory of Open Access Journals (Sweden)

    Antonello Nicolini

    2013-03-01

    Full Text Available Ovarian hyperstimulation syndrome is a serious and potentially life-threatening physiological complication that may be encountered in patients who undergo controlled ovarian hyperstimulation cycles. The syndrome is typically associated with regimes of exogenous gonadotropins, but it can be seen, albeit rarely, when clomiphene is administered during the induction phase. Although this syndrome is widely described in scientific literature and is well known by obstetricians, the knowledge of this pathological and potentially life-threatening condition is generally less than satisfactory among physicians. The dramatic increase in therapeutic strategies to treat infertility has pushed this condition into the realm of acute care therapy. The potential complications of this syndrome, including pulmonary involvement, should be considered and identified so as to allow a more appropriate diagnosis and management. We describe a case of a woman with an extremely severe (Stage 6 ovarian hyperstimulation syndrome who presented ascites, bilateral pleural effusion and severe respiratory failure treated with non-invasive ventilation. The patient was admitted to the intensive care unit because of severe respiratory failure, ascites, and bilateral pleural effusion due to ovarian hyperstimulation syndrome. Treatment included non-invasive ventilation and three thoracentesis procedures, plus the administration of albumin, colloid solutions and high-dose furosemid. Severe form of ovarian hyperstimulation syndrome is observed in 0.5-5% of the women treated, and intensive care may be required for management of thromboembolic complications, renal failure and severe respiratory failure. Pulmonary intensive care may involve thoracentesis, oxygen supplementation and, in more severe cases, assisted ventilation. To our knowledge, there have been only two studies in English language medical literature that describe severe respiratory failure treated with non

  3. Protein-Losing Enteropathy as a Complication of the Ketogenic Diet.

    Science.gov (United States)

    Ahn, Won Kee; Park, Soyoung; Kim, Heung Dong

    2017-07-01

    The ketogenic diet is an effective treatment for the patients with intractable epilepsy, however, the diet therapy can sometimes be discontinued by complications. Protein-losing enteropathy is a rarely reported serious complication of the ketogenic diet. We present a 16-month-old Down syndrome baby with protein-losing enteropathy during the ketogenic diet as a treatment for West syndrome. He suffered from diarrhea, general edema and hypoalbuminemia which were not controlled by conservative care for over 1 month. Esophagogastroduodenoscopy and stool alpha-1 antitrypsin indicated protein-losing enteropathy. Related symptoms were relieved after cessation of the ketogenic diet. Unexplained hypoalbuminemia combined with edema and diarrhea during ketogenic suggests the possibility of protein-losing enteropathy, and proper evaluation is recommended in order to expeditiously detect it and to act accordingly. © Copyright: Yonsei University College of Medicine 2017.

  4. Low starting dosage of infliximab with possible escalating dosage in psoriatic arthritis gives the same treatment results as standard dosage of adalimumab or etanercept: results from the nationwide Icelandic ICEBIO registry

    Directory of Open Access Journals (Sweden)

    Gudbjornsson B

    2018-05-01

    Full Text Available Bjorn Gudbjornsson,1,2 Arni Jon Geirsson,3,4 Niels Steen Krogh5 1Centre for Rheumatology Research, University Hospital, Reykjavik, Iceland; 2Faculty of Medicine, University of Iceland, Reykjavik, Iceland; 3Department of Rheumatology, University Hospital, Reykjavik, Iceland; 4Laeknasetrid - Medical Clinic, University of Iceland, Reykjavik, Iceland; 5Zitelab Aps, Copenhagen, Denmark Objective: To explore differences in response to a low dosage regimen of infliximab with an escalating dosage in comparison to a standard dosage of etanercept and adalimumab in patients with psoriatic arthritis (PsA. Methods: Biologically naïve PsA patients who were beginning anti-TNF-α therapy were selected from the ICEBIO registry. Demographics and clinical differences were compared in four treatment groups: infliximab <4 mg/kg; infliximab >4 mg/kg; etanercept or adalimumab at baseline and on follow-up (6 and 12 months, last visit. The Kruskal–Wallis rank sum test was used for comparison of the groups and the Wilcoxon test to compare the two infliximab dosage regimens. Results: One hundred and eighty-five patients (61% female were identified; 84 patients received infliximab, 66 etanercept, and 35 adalimumab. A total of 19% of the patients treated with infliximab escalated their dosage ≥4 mg/kg. No significant differences were observed at baseline in respect to visual analog scale (VAS pain, VAS fatigue, Health Assessment Questionnaire, C-reactive protein (CRP, numbers of swollen or tender joints, or Disease Activity Score (DAS 28-CRP values. A similar treatment response was observed in all four treatment groups on follow-up. Conclusion: In respect to treatment effects, a low dosage of infliximab with possible escalating dosage is acceptable for the majority of PsA patients who are in need of biological treatment. Keywords: psoriatic arthritis, outcome, biological treatment, routine care, clinical nationwide registry

  5. Effectiveness of Psychotherapy-Based Interventions for Complicated Grief: A Systematic Review

    Directory of Open Access Journals (Sweden)

    Ozge Enez

    2017-12-01

    Full Text Available Grief is a normal, dynamic and multidimensional process, which relates to the individuality and uniqueness of reactions to loss. However, complicated grief is a syndrome where normal grief is unusually prolonged because of complications in the natural healing process. Approximately one third of grieving individuals develop complicated grief symptoms. The individuals suffering from complicated grief tend to report clinical complaints that refer to anxiety, depression, psychical symptoms and life-threatening behaviours. The aims of this study were: to identify which psychother-apy-based interventions were designed for the treatment of complicated grief; and to make an infer-ence about the effect of these interventions. [Psikiyatride Guncel Yaklasimlar - Current Approaches in Psychiatry 2017; 9(4.000: 441-463

  6. Nicolau Syndrome after Intramuscular Benzathine Penicillin Injection

    Directory of Open Access Journals (Sweden)

    Morteza Noaparast

    2014-11-01

    Full Text Available A 3-year-old boy was admitted to the emergency department with right lower limb pain, edema, and livedoid discoloration that occurred immediately after intramuscular injection of benzathine penicillin. The patient was diagnosed with Nicolau syndrome, a rare complication of intramuscular injection presumed to be related to the inadvertent intravascular injection. It was first reported following intramuscular injection of bismuth salt, but it can occur as a complication of various other drugs. Fasciotomy was carried out due to the resultant compartment syndrome and medical therapy with heparin, corticosteroid, and pentoxifyllin was initiated.

  7. Case report

    African Journals Online (AJOL)

    abp

    2014-02-07

    Feb 7, 2014 ... 1. Possible macrophage activation syndrome following initiation of adalimumab in a ... A 26-yearold woman with classical features of AOSD developed persistent fever, severe .... de la lymphopénie NK et de l'IL-18 sérique.

  8. Calcineurin-inhibitor pain syndrome.

    Science.gov (United States)

    Prommer, Eric

    2012-07-01

    There has been increased recognition of calcineurin, a phosphoprotein serine/threonine phosphatase enzyme, in the regulation of many physiologic systems. Calcineurin mediates activation of lymphocytes, which play a role in immune response. Widely distributed in the central nervous system, calcinuerin also plays an important role in sensory neural function, via its role in the regulation of newly discovered 2-pore potassium channels, which greatly influence neuronal resting membrane potentials. Calcinuerin inhibition is the mechanism of action of immunomodulatory drugs such as cyclosporine and tacrolimus, which are widely used in transplantation medicine to prevent rejection. While important for immunosuppression, the use of calcineurin inhibitors has been associated with the development of a new pain syndrome called the calcineurin pain syndrome, which appears to be an untoward complication of the interruption of the physiologic function of calcineurin. This is a narrative review focusing on the epidemiology, pathophysiology, characterization of a newly recognized pain syndrome associated with the use of calcineurin inhibitors. The use of immunosuppressants however is associated with several well-known toxicities to which the calcineurin pain syndrome can be added. The development of this syndrome most likely involves altered nociceptive processing due to the effect of calcineurin inhibition on neuronal firing, as well as effects of calcineurin on vascular tone. The most striking aspect of the treatment of this syndrome is the response to calcium channel blockers, which suggest that the effects of calcineurin inhibition on vascular tone play an important role in the development of the calcineurin pain syndrome. The calcineurin syndrome is a newly recognized complication associated with the use of calcineurin inhibitors. There is no standard therapy at this time but anecdotal reports suggest the effectiveness of calcium channel blockers.

  9. Pulmonary complications of AIDS: radiologic features

    International Nuclear Information System (INIS)

    Cohen, B.A.; Pomeranz, S.; Rabinowitz, J.G.; Rosen, M.J.; Train, J.S.; Norton, K.I.; Mendelson, D.S.

    1984-01-01

    Fifty-two patients with pulmonary complications of acquired immunodeficiency syndrome (AIDS) were studied over a 3-year period. The vast majority of the patients were homosexual; however, a significant number were intravenous drug abusers. Thirteen different organisms were noted, of which Pneumocystis carinii was by far the most common. Five patients had neoplasia. Most patients had initial abnormal chest films; however, eight patients subsequently shown to have Pneumocystis carinii pneumonia had normal chest films. A significant overlap in chest radiographic findings was noted among patients with different or multiple organisms. Lung biopsy should be an early consideration for all patients with a clinical history consistent with the pulmonary complications of AIDS. Of the 52 patients, 41 had died by the time this report was completed

  10. Ehlers-Danlos' Syndrom

    DEFF Research Database (Denmark)

    Leganger, Julie; Søborg, Marie-Louise Kulas; Farholt, Stense

    2016-01-01

    Ehlers-Danlos syndrome (EDS) comprises a group of diseases characterized by connective tissue fragility. The clinical symptoms primarily involve the skin, joints, blood vessels and internal organs. Diagnosing EDS is complicated because of the clinical variability, imprecise diagnostic criteria...

  11. Imaging spectrum of central nervous system complications of hematopoietic stem cell and solid organ transplantation

    Energy Technology Data Exchange (ETDEWEB)

    Server, Andres [Oslo University Hospital-Rikshospitalet, Section of Neuroradiology, Department of Radiology and Nuclear Medicine, Oslo (Norway); Bargallo, Nuria [Universitat de Barcelona, Section of Neuroradiology, Department of Radiology, Hospital Clinic, Barcelona (Spain); Institut d' investigacions Biomediques August Pi i Sunyer (IDIBARS), Resonance Magnetic Image Core Facility, Barcelona (Spain); Floeisand, Yngvar [Oslo University Hospital-Rikshospitalet, Department of Hematology, Oslo (Norway); Sponheim, Jon [Oslo University Hospital-Rikshospitalet, Section of Gastroenterology, Department of Transplantation Medicine, Oslo (Norway); Graus, Francesc [Universitat de Barcelona, Department of Neurology, Hospital Clinic, Barcelona (Spain); Institut d' investigacions Biomediques August Pi i Sunyer (IDIBARS), Neuroimmunology Program, Barcelona (Spain); Hald, John K. [Oslo University Hospital-Rikshospitalet, Section of Neuroradiology, Department of Radiology and Nuclear Medicine, Oslo (Norway); University of Oslo, Faculty of Medicine, Oslo (Norway)

    2017-02-15

    Neurologic complications are common after hematopoietic stem cell transplantation (HSCT) and solid organ transplantation (SOT) and affect 30-60% of transplant recipients. The aim of this article is to provide a practical imaging approach based on the timeline and etiology of CNS abnormalities, and neurologic complications related to transplantation of specific organs. The lesions will be classified based upon the interval from HSCT procedure: pre-engraftment period <30 days, early post-engraftment period 30-100 days, late post-engraftment period >100 days, and the interval from SOT procedure: postoperative phase 1-4 weeks, early posttransplant syndromes 1-6 months, late posttransplant syndromes >6 months. Further differentiation will be based on etiology: infections, drug toxicity, metabolic derangements, cerebrovascular complications, and posttransplantation malignancies. In addition, differentiation will be based on complications specific to the type of transplantation: allogeneic and autologous hematopoietic stem cells (HSC), heart, lung, kidney, pancreas, and liver. Thus, in this article we emphasize the strategic role of neuroradiology in the diagnosis and response to treatment by utilizing a methodical approach in the work up of patients with neurologic complications after transplantation. (orig.)

  12. Imaging spectrum of central nervous system complications of hematopoietic stem cell and solid organ transplantation

    International Nuclear Information System (INIS)

    Server, Andres; Bargallo, Nuria; Floeisand, Yngvar; Sponheim, Jon; Graus, Francesc; Hald, John K.

    2017-01-01

    Neurologic complications are common after hematopoietic stem cell transplantation (HSCT) and solid organ transplantation (SOT) and affect 30-60% of transplant recipients. The aim of this article is to provide a practical imaging approach based on the timeline and etiology of CNS abnormalities, and neurologic complications related to transplantation of specific organs. The lesions will be classified based upon the interval from HSCT procedure: pre-engraftment period <30 days, early post-engraftment period 30-100 days, late post-engraftment period >100 days, and the interval from SOT procedure: postoperative phase 1-4 weeks, early posttransplant syndromes 1-6 months, late posttransplant syndromes >6 months. Further differentiation will be based on etiology: infections, drug toxicity, metabolic derangements, cerebrovascular complications, and posttransplantation malignancies. In addition, differentiation will be based on complications specific to the type of transplantation: allogeneic and autologous hematopoietic stem cells (HSC), heart, lung, kidney, pancreas, and liver. Thus, in this article we emphasize the strategic role of neuroradiology in the diagnosis and response to treatment by utilizing a methodical approach in the work up of patients with neurologic complications after transplantation. (orig.)

  13. Rehabilitation in Guillian Barre syndrome.

    Science.gov (United States)

    Khan, Fary

    2004-12-01

    Guillian Barre syndrome (GBS) is the most common form of neuromuscular paralysis. It mostly affects young people and can cause long-term residual disability. This article outlines the rehabilitation treatment for patients recovering from GBS. Recovery from GBS can be prolonged. Early rehabilitation intervention ensures medical stability, appropriate treatment and preventive measures to minimise long term complications. Specific problems include deep venous thrombosis prevention, complications of immobility, dysautonomia, de-afferent pain syndromes, muscle pain and fatigue. Longer-term issues include psychosocial adjustment, return to work and driving, and resumption of the role within the family and community. Effective communication between the GP and rehabilitation physicians is imperative for improved functional outcomes and successful social reintegration.

  14. Det hepatopulmonale syndrome

    DEFF Research Database (Denmark)

    Eibye, Simone; Christensen, Erik

    2016-01-01

    The hepatopulmonary syndrome (HPS) - a complication of liver disease - seems to be underdiagnosed, probably because of decreased awareness. HPS consists of the triade liver disease, intrapulmonary vascular dilatation and as a consequence arterial hypoxaemia. No medical therapy has proven effective...

  15. Scrub typhus complicated by acute respiratory distress syndrome and multiorgan failure; an unrecognized alarming entity in central India: A report of two cases

    Directory of Open Access Journals (Sweden)

    Amrish Saxena

    2014-01-01

    Full Text Available Scrub typhus is an acute infectious illness, distributed throughout the Asia Pacific rim. In India, it has been reported from northern, eastern, and southern India. However, cases of scrub typhus have not been well-documented from Vidarbha, an eastern region of Maharashtra state in central India. We report two cases of complicated scrub typhus from Vidarbha region. These cases admitted in unconscious state with 8-10 days history of fever, body ache, cough, and progressive breathlessness. The diagnosis in both cases was based on presence of eschar, a positive Weil-Felix test, and a positive rapid diagnostic test (immunochromatographic assay. Both cases were complicated by acute respiratory distress syndrome (ARDS and multiorgan failure. Both of them presented in their 2 nd week of illness and died during the hospital course in spite of intensive supportive care. The main cause of mortality was delayed referral leading to delay in diagnosis and treatment.

  16. Effects of blood glucose, blood lipids and blood pressure control on recovery of patients with gastric cancer complicated with metabolic syndrome after radical gastrectomy.

    Science.gov (United States)

    Sun, Li; Zhou, Pingping; Hua, Qingli; Jin, Changming; Guo, Chunling; Song, Bing

    2018-06-01

    This study aimed to investigate the effects of blood glucose, blood lipids and blood pressure control on recovery of patients with gastric cancer complicated with metabolic syndrome (MS) after radical gastrectomy. A total of 150 patients with gastric cancer, who were treated in Daqing Longnan Hospital from November, 2015 to May, 2017, were enrolled in this study. The patients were divided into the MS group (80 cases) and non-MS group (70 cases). Patients in the MS group were given corresponding drugs to control blood pressure, blood lipids and blood glucose, while patients in the non-MS group were not treated with those drugs. Patients in the MS group were divided into the normal and abnormal groups according to the levels of blood glucose, blood lipids and blood pressure. Moreover, occurrences of complications were compared between the normal and abnormal groups. Before surgery, blood glucose, blood lipids and blood pressure in the MS group were significantly higher than those in the non-MS group (pblood glucose, blood lipids and blood pressure of the MS group decreased significantly compared to those before operation (pblood glucose, 2 h postprandial blood glucose, glycosylated hemoglobin, total triglycerides (TGs), LDL, mean blood pressure and BMI (pblood glucose, blood lipids and blood pressure in patients with gastric cancer complicated with MS after radical gastrectomy can reduce the incidence of postoperative complications and promote postoperative recovery.

  17. Fatal Lemierre's syndrome as a complication of chronic otitis media ...

    African Journals Online (AJOL)

    a high index of suspicion should be maintained, as it remains a ... developed Lemierre's syndrome secondary to untreated chronic otitis ... This septic thrombophlebitis gives rise to septic microemboli that can disseminate throughout the body ...

  18. False diagnosis of type 1 diabetes mellitus and its complications in Wolfram syndrome--is it the reason for the low number of reported cases of this abnormality?

    Science.gov (United States)

    Homa, Katarzyna; Stefański, Adam; Zmysłowska, Agnieszka; Molęda, Piotr; Bryśkiewicz, Marta Ewa; Majkowska, Liliana

    2014-01-01

    Wolfram syndrome (WS), also known as DIDMOAD (Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy and Deafness), is a rare autosomal recessive syndrome (1/770,000 in the United Kingdom), characterised by juvenile onset of diabetes mellitus, optic nerve atrophy, diabetes insipidus, sensorineural deafness, renal tract and neurological abnormalities, and primary gonadal atrophy. WS is caused mainly by biallelic mutations in the WFS1 gene, which encodes wolframin. Wide tissue distribution of wolframin and many mutations in the wolframin gene resulting in Wolfram syndrome may contribute to different phenotypes and the unusual combinations of clinical features. We describe a female patient with Wolfram syndrome diagnosed at the age of 25, with a previous false diagnosis of type 1 diabetes mellitus and misdiagnosed diabetic complications. The patient was found to be a compound heterozygote for two novel mutations in exon 8 of WFS1 gene: a 2-bp deletion AT at nt 1539 leading to a frameshift (Y513fs) and a single-base substitution 1174C > T resulting in a stop codon (Q392X). A detailed analysis of the patient's medical history and a review of the literature suggest that many cases of Wolfram syndrome may remain undiagnosed due to misdiagnosis as type 1 diabetes mellitus and incorrect interpretation of clinical symptoms of neurodegenerative abnormalities, especially in their early stages.

  19. Thrombophilia and Pregnancy Complications

    Directory of Open Access Journals (Sweden)

    Louise E. Simcox

    2015-11-01

    Full Text Available There is a paucity of strong evidence associated with adverse pregnancy outcomes and thrombophilia in pregnancy. These problems include both early (recurrent miscarriage and late placental vascular-mediated problems (fetal loss, pre-eclampsia, placental abruption and intra-uterine growth restriction. Due to poor quality case-control and cohort study designs, there is often an increase in the relative risk of these complications associated with thrombophilia, particularly recurrent early pregnancy loss, late fetal loss and pre-eclampsia, but the absolute risk remains very small. It appears that low-molecular weight heparin has other benefits on the placental vascular system besides its anticoagulant properties. Its use is in the context of antiphospholipid syndrome and recurrent pregnancy loss and also in women with implantation failure to improve live birth rates. There is currently no role for low-molecular weight heparin to prevent late placental-mediated complications in patients with inherited thrombophilia and this may be due to small patient numbers in the studies involved in summarising the evidence. There is potential for low-molecular weight heparin to improve pregnancy outcomes in women with prior severe vascular complications of pregnancy such as early-onset intra-uterine growth restriction and pre-eclampsia but further high quality randomised controlled trials are required to answer this question.

  20. Cardiovascular complications of obesity in adolescents.

    Science.gov (United States)

    Orio, F; Palomba, S; Cascella, T; Savastano, S; Lombardi, G; Colao, A

    2007-01-01

    Obesity is an increasingly important worldwide health problem, representing the major risk factor for coronary heart disease. The increase in the prevalence of obesity, particularly among younger age groups, is likely to have long-term implications for cardiovascular disease (CVD) in the years to come, especially at a young age. Obesity plays a central role in the insulin resistance (IR) syndrome and increases the risk of atherosclerotic CVD. The present review will examine the relationships among cardiovascular risk (CVR) factors during the childhood-adolescence-adulthood transition. In fact, the relation between obesity, in particular visceral obesity and CVD, appears to develop at a relatively young age. The foremost physical consequence of obesity is atherosclerotic CVD, and an intriguing example of obesity-related cardiovascular complications affecting young women is the polycystic ovary syndrome (PCOS).

  1. Complications of Central Venous Totally Implantable Access Port: Internal Jugular Versus Subclavian Access

    Directory of Open Access Journals (Sweden)

    Pil Young Jung

    Full Text Available Background: Totally implantable access port (TIAP provides reliable, long term vascular access with minimal risk of infection and allows patients normal physical activity. With wide use of ports, new complications have been encountered. We analyzed TIAP related complications and evaluated the outcomes of two different percutaneous routes of access to superior vena cava. Methods: All 172 patients who underwent port insertion with internal jugular approach (Group 1, n = 92 and subclavian approach (Group 2, n = 79 between August 2011 and May 2013 in a single center were analyzed, retrospectively. Medical records were analyzed to compare the outcomes and the occurrence of port related complications between two different percutaneous routes of access to superior vena cava. Results: Median follow-up for TIAP was 278 days (range, 1-1868. Twenty four complications were occurred (14.0%, including pneumothorax (n = 1, 0.6%, migration/malposition (n = 4, 2.3%, pinch-off syndrome (n = 4, 2.3%, malfunction (n = 2, 1.1%, infection (n = 8, 4.7%, and venous thrombosis (n = 5, 2.9%. The overall incidence was 8.7% and 20.3% in each group (p = 0.030. Mechanical complications except infectious and thrombotic complications were more often occurred in group 2 (p = 0.033. The mechanical complication free probability is significantly higher in group 1 (p = 0.040. Conclusions: We suggest that the jugular access should be chosen in patients who need long term catheterization because of high incidence of mechanical complication, such as pinch-off syndrome.

  2. Cochlear implants in Waardenburg syndrome.

    Science.gov (United States)

    Cullen, Robert D; Zdanski, Carlton; Roush, Patricia; Brown, Carolyn; Teagle, Holly; Pillsbury, Harold C; Buchman, Craig

    2006-07-01

    Waardenburg syndrome is an autosomal-dominant syndrome characterized by dystopia canthorum, hyperplasia of the eyebrows, heterochromia irides, a white forelock, and sensorineural hearing loss in 20% to 55% of patients. This patient population accounts for approximately 2% of congenitally deaf children. The purpose of this retrospective case review was to describe the outcomes for those children with Waardenburg syndrome who have undergone cochlear implantation. Pediatric cochlear implant recipients with documented evidence of Waardenburg syndrome underwent retrospective case review. All patients received their cochlear implants at the study institution followed by outpatient auditory habilitation. Charts were reviewed for etiology and duration of deafness, age at time of cochlear implantation, perioperative complications, duration of use, and performance outcomes. Results of standard tests batteries for speech perception and production administered as a part of the patients' auditory habilitation were reviewed. Seven patients with Waardenburg syndrome and cochlear implants were identified. The average age at implantation was 37 months (range, 18-64 months) and the average duration of use was 69 months (range, 12-143 months). All of these patients are active users of their devices and perform very well after implantation. There were no major complications in this small group of patients. Children with congenital sensorineural hearing loss without other comorbidities (e.g., developmental delay, inner ear malformations) perform well when they receive cochlear implantation and auditory habilitation. Patients with Waardenburg syndrome can be expected to have above-average performance after cochlear implantation.

  3. Does anemia-polycythemia complicating twin-twin transfusion syndrome affect outcome after fetoscopic laser surgery?

    Science.gov (United States)

    Donepudi, R; Papanna, R; Snowise, S; Johnson, A; Bebbington, M; Moise, K J

    2016-03-01

    Twin anemia-polycythemia sequence (TAPS) can occur as a unique disease or as a complication of twin-twin transfusion syndrome (TTTS). Middle cerebral artery (MCA) Doppler studies are not currently part of the routine evaluation of monochorionic twins since they are not used in the Quintero staging system. As such, the true incidence of TAPS is unknown. We aimed to compare the characteristics and outcomes of twin pregnancies with TTTS complicated by spontaneous anemia-polycythemia vs those with TTTS alone. This was a secondary analysis of data collected prospectively from a cohort of 156 consecutive patients undergoing fetoscopic laser surgery for TTTS, between October 2011 and August 2014. TAPS was defined as discordance in the preoperative MCA peak systolic velocity (PSV), with one twin fetus having MCA-PSV ≤ 1.0 multiples of the median (MoM) and the other having MCA-PSV ≥ 1.5 MoM. Maternal demographics as well as preoperative, operative and postoperative variables were analyzed. Included in the final analysis were 133 patients with complete records: 11 cases with TTTS with anemia-polycythemia and 122 cases with TTTS alone. There was no difference in maternal body mass index, gestational age (GA) at procedure, rate of preterm prelabor rupture of membranes or GA at delivery between the two groups. Patients with TTTS and anemia-polycythemia were more likely to be older (P = 0.03) and parous (P = 0.04) and had a significantly lower number of placental anastomoses (P = 0.01). The dual live-birth rate was similar for both groups (P = 0.76). Cases of TTTS with anemia-polycythemia were more likely to be found in parous and older women and were characterized by fewer vascular anastomoses. TTTS with anemia-polycythemia was not associated with worse perinatal outcome after laser therapy. Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd. Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd.

  4. Imaging of Budd-Chiari syndrome

    International Nuclear Information System (INIS)

    Buckley, O.; O' Brien, J.; Snow, A.; Stunell, H.; Torreggiani, W.C.; Lyburn, I.; Munk, P.L.

    2007-01-01

    Budd-Chiari syndrome occurs when venous outflow from the liver is obstructed. The obstruction may occur at any point from the hepatic venules to the left atrium. The syndrome most often occurs in patients with underlying thrombotic disorders such as polycythemia rubra vera, paroxysmal nocturnal hemoglobinuria and pregnancy. It may also occur secondary to a variety of tumours, chronic inflammatory diseases and infections. Imaging plays an important role both in establishing the diagnosis of Budd-Chiari syndrome as well as evaluating for underlying causes and complications such as portal hypertension. In this review article, we discuss the role of modern imaging in the evaluation of Budd-Chiari syndrome. (orig.)

  5. Imaging of Budd-Chiari syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Buckley, O.; O' Brien, J.; Snow, A.; Stunell, H.; Torreggiani, W.C. [Adelaide and Meath Hospital, Incorporating the National Children' s Hospital (AMNCH), Department of Radiology, Dublin 24 (Ireland); Lyburn, I. [Cheltenham Hospital, Department of Radiology, Cheltenham (United Kingdom); Munk, P.L. [Vancouver General Hospital, Department of Radiology, Vancouver (Canada)

    2007-08-15

    Budd-Chiari syndrome occurs when venous outflow from the liver is obstructed. The obstruction may occur at any point from the hepatic venules to the left atrium. The syndrome most often occurs in patients with underlying thrombotic disorders such as polycythemia rubra vera, paroxysmal nocturnal hemoglobinuria and pregnancy. It may also occur secondary to a variety of tumours, chronic inflammatory diseases and infections. Imaging plays an important role both in establishing the diagnosis of Budd-Chiari syndrome as well as evaluating for underlying causes and complications such as portal hypertension. In this review article, we discuss the role of modern imaging in the evaluation of Budd-Chiari syndrome. (orig.)

  6. A Rare Case of Acute Coronary Syndrome in a Patient With Turner Syndrome.

    Science.gov (United States)

    Kemaloglu, Tugba; Ozer, Nihat; Fikri Yapici, Mehmet

    2016-05-01

    In Turner syndrome, cardiovascular complications are the most important causes of early mortality. Congenital cardiovascular abnormalities are found in approximately one third of Turner syndrome patients. Developments in diagnosis and treatment have decreased the rate of mortality related to these abnormalities. In recent years, many papers have mentioned that coronary artery disease developing at early ages in patients with Turner syndrome causes sudden deaths. The patient, a 27-year-old female was admitted to the emergency room with chest pain at rest. She was diagnosed with Turner Syndrome in her teenage years due to amenorrhea. Patients with ECG changes and cardiac enzyme elevations were treated with acute coronary syndrome. The young woman with Turner Syndrome have several risk factors for early Coronary Artery Disease development. In such cases, dramatic results like sudden death or heart attack at an early age may occur in cases of insufficient follow-up and treatment.

  7. Non Piluitary Surgery in a patient with Acromegaly complicated by ...

    African Journals Online (AJOL)

    The anaesthetic management of patients with acromegaly, a rare clinical syndrome, for urgent non pituitary surgery has little documentation in the literature. We report such a case further complicated by dilated cardiomyopathy. The anaethetic challenges in the face of limited resources are highlighted. Keywords: ...

  8. Plasmapheresis for Preventing Complication of Hypertriglyceridemia: A Case Report and Review of Literature.

    Science.gov (United States)

    Costantini, Nicoletta; Mameli, Antonella; Marongiu, Francesco

    2016-01-01

    Severe hypertriglyceridemia is a common indication for the need of plasma exchange in treatment of hypertriglyceridemic-induced pancreatitis when normal therapies fail to garner a response. Application of plasmapheresis to prevent complication of hypertriglyceridemia is limited because of its cost and availability. We present a case of a 44-year-old man with metabolic syndrome and a medical history of secondary polycythemia in obesity hypoventilation syndrome, whose laboratory tests revealed a triglycerides value of 3965 mg/dL. To prevent the complication of pancreatitis due to hypertriglyceridemia, we performed plasma exchange 3 times when conventional treatments did not sufficiently reduce the high level of triglycerides. A review of the current available literature was therefore conducted to provide an overview of the present data on apheretic treatment for patients with severe hypertriglyceridemia. Several case reports and case series have used plasmapheresis in acute treatment of hypertriglyceridemia pancreatitis related. In our case, the choice of plasmapheresis was applied in prevention of possible complications of hypertriglyceridemia.

  9. Volvulus as a complication of chronic intestinal pseudo-obstruction syndrome

    NARCIS (Netherlands)

    C.T. de Betue (Carlijn); D. Boersma (Doeke); M.W. Oomen (Matthijs W.); M.A. Benninga (Marc); J.R. de Jong (Justin)

    2011-01-01

    textabstractChronic intestinal pseudo-obstruction syndrome (CIPS) is a severe motility disorder of the gastrointestinal tract that presents with continuous or recurrent symptoms and signs of intestinal obstruction without evidence of a structural lesion occluding the intestinal lumen. Mechanical

  10. Volvulus as a complication of chronic intestinal pseudo-obstruction syndrome

    NARCIS (Netherlands)

    de Betue, Carlijn T.; Boersma, Doeke; Oomen, Matthijs W.; Benninga, Marc A.; de Jong, Justin R.

    2011-01-01

    Chronic intestinal pseudo-obstruction syndrome (CIPS) is a severe motility disorder of the gastrointestinal tract that presents with continuous or recurrent symptoms and signs of intestinal obstruction without evidence of a structural lesion occluding the intestinal lumen. Mechanical obstruction

  11. [Refeeding syndrome].

    Science.gov (United States)

    Ševela, Stanislav; Novák, František; Kazda, Antonín; Brodská, Helena

    Despite being known more than 60 years, refeeding syndrome (RS) still bears many uncertainties. For example, its definition is not clear and definite, and the attitude to it varies from the complete neglect to over-prevention.The term "refeeding syndrome" refers to electrolyte and metabolic changes occurring in malnourished patients after the readministration of nutrition. These changes concern especially to phosphates and ions. Potassium, magnesium, naturism and fluids balance are involved. The changes lead to cell energetic metabolism and electric potential disturbances, with related clinical symptoms.Fully developed refeeding syndrome is quite rare; nevertheless it can be fatal for the patient. However, even its development can lead to many complications increasing the patient's morbidity and the length of stay in the hospital. Yet the refeeding syndrome is more or less predictable and if kept in mind also preventable.The aim of this article is to get the reader to know more about this metabolic phenomenon and possible attitudes towards it.

  12. Multipl Pregnancies and Their Complications

    Directory of Open Access Journals (Sweden)

    Volkan Turan

    2011-03-01

    Full Text Available Objective: To investigate the complications observed in multipl pregnancies which are increasing in day by day. Material and method: We reviewed 173 multiple pregnancies that were followed up in the Department of Obstetric and Gynecology in Ege University during one year period and determined the preterm delivery ratio, discordance between fetuses and investigated the complications which occurred during pregnancy. Results: 148 twin, 24 triplet and 1 quadriplet pregnancies had been followed in a year. While 56 of twin pregnancies and 4 of the triplet pregnancies occurred spontaneously, others conceived with medical treatment or with assisted reproductive technology. Cerclage was performed in 11 pregnancies. Preeclampsia, gestational diabetes mellitus and cholestasis were observed in 12,26 and 8 patients respectively. Twin-to-twin transfusion syndrome was present in 4 patients and one patient had acardiac-acephalic twin pregnancy. Four patients had emergency cesarean section due to ablatio placenta. While 54 patients were hospitalized for one week to twelve weeks because of preterm labour 36 women had preterm premature rupture of the membranes. Only 38 patients had any problem during pregnancy. Discussion: Developments in assisted reproductive technology have been increasing the number of multiple gestations and their complications. The complications due to preterm labor, increased requirement of Neonatal Intensive Care Units and hospital payments are all burden on the families as well as on the social insurance companies.

  13. E valuation and clinical significance of serum C-reactive protein and homocysteine level in obstructive sleep apnea/hypopnea syndrome complicated with coronary heart disease patients

    International Nuclear Information System (INIS)

    Su Yingrui; Zha Jinshun; Xu Chaoxiang; Chen Xiaoyang; Wang Yaoguo; Du Xinqing

    2011-01-01

    Objective: To explore the relationship between homocysteine (HCY) and C-reactive protein (CRP) in obstructive sleep apnea/hypopnea syndrome (OSAHS) patients and OSAHS patients complicated with coronary heart disease by detecting the scrum level of HCY and CRP on the mechanism of OSAHS complicated with coronary heart disease. Methods: Ninety-one patients were divided into three groups, 30 patients as control group, 36 patients as OSAHS group, and 25 patients as OSAHS complicated with CHD group. Serum HCY level was detected through chemiluminescence. Serum CRP level was detected through radioimmunity. The serum level of HCY and CRP was compared among these groups. OSAHS patients were divided into mild OSAHS subgroup, moderate OSAHS subgroup and severe OSAHS subgroup. The morbidity rate of CHD and the serum level of HCY and CRP were compared among these subgroups. Meanwhile the parameters of polysomnogram such as activity apnea-hypopnea index (AHI) and blood oxygen saturation (SaO 2 ) were compared between OSAHS group and OSAHS complicated with coronary heart disease group. Results: (1) There was significant difference among the serum level of HCY and CRP of control group, OSAHS group and OSAHS complicated with CHD group (F HCY =15.80, F CRP =19.21, P all HCY =4.74, t CRP =5.14, P all HCY =7.31, t CRP =8.17, P all 2 =6.96, χ 2 =4.18, P HCY =16.38, F CRP =12.97, P all 2 of OSAHS group and OSAHS complicated with CHD group (t AHI =5.46, percentage of SaO 2 2 : t=4.68, average lowest SaO 2 : t=3.65, longest duration of disordered breathing events: t=4.73, P all<0.01 ). Conclusion: The serum level of HCY and CRP rose because of hypoxia in OSAHS patients,and might play an important role in the mechanism of OSAHS complicated with CHD. (authors)

  14. Frey's syndrome - unusually long delayed clinical onset ...

    African Journals Online (AJOL)

    Frey's syndrome is a complication of parotidectomy that is thought to occur as a result of aberrant regeneration of the postganglionic parasympathetic nerve fibres supplying the parotid gland to severed ostganglionic sympathetic fibres which innervate the sweat glands of the face. Frey's syndrome is difficult to treat but is a ...

  15. [Bilateral "crocodile tears syndrome" associated with Melkersson-Rosenthal syndrome--case report].

    Science.gov (United States)

    Owecki, Michał K; Kapelusiak-Pielok, Magdalena; Kowal, Piotr; Kozubski, Wojciech

    2006-01-01

    We present a rare case of bilateral crocodile tears syndrome (CTS) in the course of Melkersson-Rosenthal syndrome. Melkersson-Rosenthal syndrome is characterised by a triad of recurrent orofacial swelling, relapsing facial paralysis, and fissured tongue. The classic triad is infrequent and oligosymptomatic variants are seen more frequently. CTS is a rare complication of facial nerve paralysis characterised by inappropriate lacrimation on the side of the palsy in response to salivary stimuli. It results from aberrant reinnervation of the lacrimal gland by salivary parasympathetic fibres. The therapeutic approach for an acute bout of Melkersson-Rosenthal syndrome consists mainly of steroid administration. CTS management is composed of anticholinergic drugs and surgical procedures. Botulin toxin injection into the lacrimal gland is the most modern therapeutic option. In the case presented CTS developed in a 50-year-old man after 5 incidents of facial palsy due to Melkersson-Rosenthal syndrome. The case deserves attention due to the rarity of the observed symptoms and signs.

  16. Ichthyosis prematurity syndrome: a well-defined congenital ichthyosis subtype

    DEFF Research Database (Denmark)

    Bygum, Anette; Westermark, Per; Brandrup, Flemming

    2008-01-01

    Ichthyosis prematurity syndrome is a rare syndrome characterized by the clinical triad of premature birth, thick caseous desquamating epidermis, and neonatal asphyxia. We describe two siblings with ichthyosis prematurity syndrome. The index patient was born at gestational week 34. Immediately aft...... in the stratum corneum and stratum granulosum. Diagnosing this syndrome is important to reassure parents, obstetricians, and pediatricians about its benign course after complications in the perinatal period....

  17. Complications associated with prone positioning in elective spinal surgery.

    Science.gov (United States)

    DePasse, J Mason; Palumbo, Mark A; Haque, Maahir; Eberson, Craig P; Daniels, Alan H

    2015-04-18

    Complications associated with prone surgical positioning during elective spine surgery have the potential to cause serious patient morbidity. Although many of these complications remain uncommon, the range of possible morbidities is wide and includes multiple organ systems. Perioperative visual loss (POVL) is a well described, but uncommon complication that may occur due to ischemia to the optic nerve, retina, or cerebral cortex. Closed-angle glaucoma and amaurosis have been reported as additional etiologies for vision loss following spinal surgery. Peripheral nerve injuries, such as those caused by prolonged traction to the brachial plexus, are more commonly encountered postoperative events. Myocutaneous complications including pressure ulcers and compartment syndrome may also occur after prone positioning, albeit rarely. Other uncommon positioning complications such as tongue swelling resulting in airway compromise, femoral artery ischemia, and avascular necrosis of the femoral head have also been reported. Many of these are well-understood and largely avoidable through thoughtful attention to detail. Other complications, such as POVL, remain incompletely understood and thus more difficult to predict or prevent. Here, the current literature on the complications of prone positioning for spine surgery is reviewed to increase awareness of the spectrum of potential complications and to inform spine surgeons of strategies to minimize the risk of prone patient morbidity.

  18. Recent developments in our understanding of the antiphospholipid syndrome

    NARCIS (Netherlands)

    de Groot, P. G.; Meijers, J. C. M.; Urbanus, R. T.

    2012-01-01

    The antiphospholipid syndrome is an autoimmune disease that manifests clinically as recurrent thrombotic complications or foetal losses and serologically with elevated levels of antiphospholipid antibodies in the plasmas of these patients. The term 'antiphospholipid syndrome' is confusing, because

  19. Severe Renal Hemorrhage in a Pregnant Woman Complicated with Antiphospholipid Syndrome: A Case Report

    Directory of Open Access Journals (Sweden)

    Shohei Kawaguchi

    2011-01-01

    Full Text Available Antiphospholipid syndrome is a systemic autoimmune disease with thrombotic tendency. Consensus guidelines for pregnancy with antiphospholipid syndrome recommend low-dose aspirin combined with unfractionated or low-molecular-weight heparin because antiphospholipid syndrome causes habitual abortion. We report a 36-year-old pregnant woman diagnosed with antiphospholipid syndrome receiving anticoagulation treatment. The patient developed left abdominal pain and gross hematuria at week 20 of pregnancy. An initial diagnosis of left ureteral calculus was made. Subsequently abdominal-pelvic computed tomography was required for diagnosis because of the appearance of severe contralateral pain. Computed tomography revealed serious renal hemorrhage, and ureteral stent placement and pain control by patient-controlled analgesia were required. After treatment, continuance of pregnancy was possible and vaginal delivery was performed safely. This is the first case report of serious renal hemorrhage in a pregnant woman with antiphospholipid syndrome receiving anticoagulation treatment and is an instructive case for urological and obstetrical practitioners.

  20. A successfully treated case of hypopituitarism complicated with hyperosmolar hyperglycaemic state and rhabdomyolysis.

    Science.gov (United States)

    Wang, Yi-Chen; Gao, Li-Chao; Xu, Hui; Qu, Xiao-Zhang; Wang, Ye; Lou, Xiao-Qian; Guo, Hui

    2015-05-01

    This study aimed to report a rare case of hypopituitarism complicated with hyperosmolar hyperglycaemic state and rhabdomyolysis. Hypopituitarism is a clinical syndrome in which there is a deficiency in hormone production by the pituitary gland. It often leads to hypoglycaemia, but in this case the patient was complicated with hyperosmolar hyperglycaemic state. The patient received prompt medical treatment, which effectively prevented the occurrence of possible acute kidney failure and other complications. This is a complicated and rare case. Our report provides some indications for the timely diagnosis and the standardised treatments for a patient who has hypopituitarism complicated with hyperosmolar hyperglycaemic state and rhabdomyolysis. © The Author(s) 2015 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  1. Drug treatment of metabolic syndrome.

    Science.gov (United States)

    Altabas, Velimir

    2013-08-01

    The metabolic syndrome is a constellation of risk factors for cardiovascular diseases including: abdominal obesity, a decreased ability to metabolize glucose (increased blood glucose levels and/or presence of insulin resistance), dyslipidemia, and hypertension. Patients who have developed this syndrome have been shown to be at an increased risk of developing cardiovascular disease and/or type 2 diabetes. Genetic factors and the environment both are important in the development of the metabolic syndrome, influencing all single components of this syndrome. The goals of therapy are to treat the underlying cause of the syndrome, to reduce morbidity, and to prevent complications, including premature death. Lifestyle modification is the preferred first-step treatment of the metabolic syndrome. There is no single effective drug treatment affecting all components of the syndrome equally known yet. However, each component of metabolic syndrome has independent goals to be achieved, so miscellaneous types of drugs are used in the treatment of this syndrome, including weight losing drugs, antidiabetics, antihypertensives, antilipemic and anticlothing drugs etc. This article provides a brief insight into contemporary drug treatment of components the metabolic syndrome.

  2. Cervical ankylosis following Grisel's syndrome in a 14-year-old boy with infectious mononucleosis

    International Nuclear Information System (INIS)

    Haidar, Salwa; Armstrong, Derek; Drake, James

    2005-01-01

    Non-traumatic atlanto-axial subluxation (Grisel's syndrome) is an uncommon complication of neck space infection or otolaryngologic procedures. It most frequently affects children, although it may occur in adults. We present a 14-year-old boy with Grisel's syndrome as a complication of infectious mononucleosis proceeding to cervical ankylosis. (orig.)

  3. ROHHAD Syndrome: The Girl who Forgets to Breathe.

    Science.gov (United States)

    Sanklecha, Mukesh; Sundaresan, Suba; Udani, Vrajesh

    2016-04-01

    ROHHAD syndrome is an exceedingly rare cause of central hypoventilation. A 7-year-old girl with ROHHAD syndrome who had central hypoventilation, rapid weight gain, multiple cardiac arrests and hyperprolactinemia. She required prolonged and repeated ventilation, and finally died due to complications of ventilation. ROHHAD Syndrome should be suspected in any child who presents with obesity, behavioral changes or autonomic instability following a neural crest tumor.

  4. Oral Complications in Hematopoietic Stem Cell Recipients: The Role of Inflammation

    Directory of Open Access Journals (Sweden)

    T. M. Haverman

    2014-01-01

    Full Text Available Hematopoietic stem cell transplantation (HSCT is widely used as a potentially curative treatment for patients with various hematological malignancies, bone marrow failure syndromes, and congenital immune deficiencies. The prevalence of oral complications in both autologous and allogeneic HSCT recipients remains high, despite advances in transplant medicine and in supportive care. Frequently encountered oral complications include mucositis, infections, oral dryness, taste changes, and graft versus host disease in allogeneic HSCT. Oral complications are associated with substantial morbidity and in some cases with increased mortality and may significantly affect quality of life, even many years after HSCT. Inflammatory processes are key in the pathobiology of most oral complications in HSCT recipients. This review article will discuss frequently encountered oral complications associated with HSCT focusing on the inflammatory pathways and inflammatory mediators involved in their pathogenesis.

  5. [Nephrourologic pathology in girls with Turner syndrome].

    Science.gov (United States)

    Di Pinto, Diana; Balestracci, Alejandro; Dujovne, Noelia; de Palma, Isabel; Adragna, Marta; Delgado, Norma

    2010-08-01

    Nephrourologic malformations in Turner syndrome are frequent, its diagnosis and follow-up is important in order to diminish the morbidity of this disease. The aim of this retrospective study was to analyze the nephrourologic pathology in 72 girls with Turner syndrome followed between 1989 and 2008 at Garrahan Hospital. The prevalence of nephrourologic involvement was 33% (24 patients). The most frequent findings were urinary system malformations, isolated (10 pacientes, 42%) or associated with renal malformations (9 patients, 37%); 5 patients (21%) had only renal malformations. Fifty percent of patients developed complications (8 urinary tract infection, 2 proteinuria and 2 arterial hypertension); however, none progressed to chronic renal failure. The prevalence of nephrourologic involvement was 33% and a half of these girls developed complications, our findings show the need of routine nephrological follow-up of girls with Turner syndrome and nephrourologic malformations.

  6. Plasma MicroRNA Profiles in Patients with Early Rheumatoid Arthritis Responding to Adalimumab plus Methotrexate vs Methotrexate Alone

    DEFF Research Database (Denmark)

    Sode, Jacob; Krintel, Sophine B; Carlsen, Anting Liu

    2018-01-01

    OBJECTIVE: The aim was to identify plasma (i.e., cell-free) microRNA (miRNA) predicting antitumor necrosis and/or methotrexate (MTX) treatment response in patients enrolled in an investigator-initiated, prospective, double-blinded, placebo-controlled trial (The OPERA study, NCT00660647). METHODS......: We included 180 disease-modifying antirheumatic drug-naive patients with early rheumatoid arthritis (RA) randomized to adalimumab (ADA; n = 89) or placebo (n = 91) in combination with MTX. Plasma samples before and 3 months after treatment initiation were analyzed for 91 specific mi...... multivariate miRNA models were able to predict response to ADA treatment after 3 and 12 months, with 63% and 82% area under the ROC curves, respectively. CONCLUSION: We identified miR-27a-3p as a potential predictive biomarker of ACR/EULAR remission in patients with early RA treated with ADA in combination...

  7. Tolerance of the central nervous system to photon irradiation. Endocrine complications

    Energy Technology Data Exchange (ETDEWEB)

    Wigg, D.R.; Murray, R.M.L.; Koschel, K. (Royal Adelaide Hospital (Australia))

    1982-01-01

    Dose-response isoeffect equations have been determined for hypothalamic pituitary insufficiency following cranial irradiation. Of particular importance is the occurrence of complications at doses substantially less than those commonly used for the treatment of central nervous system tumors. Such complications may be severe and potentially life threatening. These complications occur when a small midline 'target' volume containing the pituitary gland, infundibulum and adjacent inferior hypothalamic structures is irradiated. Direct pituitary irradiation is unlikely to be a factor, at least in some cases. The possible role of incidental hypothalamic irradiation in the control of acromegaly and pituitary dependent Cushing's syndrome is discussed.

  8. Pinch-off syndrome: transection of implantable central venous access device

    OpenAIRE

    Sugimoto, Takuya; Nagata, Hiroshi; Hayashi, Ken; Kano, Nobuyasu

    2012-01-01

    As the population of people with cancer increases so does the number of patients who take chemotherapy. Majority of them are administered parentally continuously. Implantable central venous catheter device is a good choice for those patients; however, severe complication would occur concerning the devices. Pinch-off syndrome is one of the most severe complications. The authors report a severe case of pinch-off syndrome. The patient with the implantable central venous device could not take che...

  9. Frey's syndrome - unusually long delayed clinical onset post ...

    African Journals Online (AJOL)

    cqq1a

    2010-04-07

    Apr 7, 2010 ... Frey's syndrome is a complication of parotidectomy that is thought to occur as a result of aberrant regeneration of the postganglionic parasympathetic nerve fibres supplying the parotid gland to severed ostganglionic sympathetic fibres which innervate the sweat glands of the face. Frey's syndrome is.

  10. Gradenigo syndrome, a rare complication of the otitis media: a case report

    International Nuclear Information System (INIS)

    Aribal, S.; Incedayo, M.; Sivrioglu, A.

    2012-01-01

    Full text: 5-year-old child was admitted to our hospital's pediatrics service with the symptoms of acute otitis media. After 10 days antibiotherapy, he was readmitted with headache, diplopia and right sided retroauricular pain. In accordance with these symptomatologies, cranial MRI was performed to demonstrate the pathology and to make a further evaluation. According to clinical and MRI findings he was accepted as Gradenigo Syndrome and treated surgically. We just aimed to present the Gradenigo Syndrome's MRI findings in this case report

  11. Modern tactics of observation the postpartum purulent-septic complications

    Directory of Open Access Journals (Sweden)

    O. M. Slinko

    2014-04-01

    Full Text Available Introduction. Postpartum purulent-septic diseases remain an urgent problem of modern obstetrics and are one of the important causes of maternal mortality. The presence of lethal proceeds from septic complications in the postpartum period is above all due to late diagnosis of this complication, delayed acceptance decision on the need and extent of surgery, inadequate capacity of syndromic and intensive care. The aim of research. The clinical analysis of different methods of diagnosis and treatment of patients with postpartum purulent-septic complications, obtained during the period from January 2011 to June 2013, based on work of gynecological department of Zaporozhye Regional Clinical Hospital. Materials and methods. During the period from January 2011 to June 2013 in the gynecological department of Zaporozhye Regional Clinical Hospital specialized medical care of 30 patients with purulent-septic complications in the postpartum period have been provided. 5 women in labor (16.67% were transferred from the maternity homes of Zaporozhye city and the rest (83.33% from different regions. Results. All patients admitted to gynecology department of Zaporozhye Regional Clinical Hospital were conducted massive antibiotic, syndromic therapy. 11 additional patients had an operation vacuum aspiration contents of the uterus, and 5 mothers had absolute indications for surgery: hysterectomy with uterine tube (2 patients diagnosed - septic condition, 3 cases - failure scar on the uterus after cesarean. Conclusion. 1. Delivery by cesarean section surgery significantly increases the risk of developing pyo-septic complications. Prompt delivery should be made according to strict indications, using modern sutures , intraoperative blood loss objective assessment (if necessary with timely appointment of replacement therapy and anti-anemic , with mandatory drainage of the abdominal cavity with repeated cesarean operations . 2. Using the entire set of clinical

  12. A Summation Analysis of Compliance and Complications of Compression Hosiery for Patients with Chronic Venous Disease or Post-thrombotic Syndrome.

    Science.gov (United States)

    Kankam, Hadyn K N; Lim, Chung S; Fiorentino, Francesca; Davies, Alun H; Gohel, Manj S

    2018-03-01

    Compression stockings are commonly prescribed for patients with a range of venous disorders, but are difficult to don and uncomfortable to wear. This study aimed to investigate compliance and complications of compression stockings in patients with chronic venous disease (CVD) and post-thrombotic syndrome (PTS). A literature search of the following databases was carried out: MEDLINE (via PubMed), EMBASE (via OvidSP, 1974 to present), and CINAHL (via EBSCOhost). Studies evaluating the use of compression stockings in patients with CVD (CEAP C2-C5) or for the prevention or treatment of PTS were included. After scrutinising full text articles, compliance with compression and associated complications were assessed. Compliance rates were compared based on study type and degree of compression. Good compliance was defined as patients wearing compression stockings for >50% of the time. From an initial search result of 4303 articles, 58 clinical studies (37 randomised trials and 21 prospective studies) were selected. A total of 10,245 limbs were included, with compression ranging from 15 to 40 mmHg (not stated in 12 studies) and a median follow-up of 12 months (range 1-60 months). In 19 cohorts, compliance was not assessed and in a further nine, compliance was poorly specified. Overall, good compliance with compression was reported for 5371 out of 8104 (66.2%) patients. The mean compliance, weighted by study size, appeared to be greater for compression ≤25 mmHg (77%) versus > 25 mmHg (65%) and greater in the randomised studies (74%) than in prospective observational studies (64%). Complications of stockings were not mentioned in 43 out of 62 cohorts reviewed. Where complications were considered, skin irritation was a common event. In published trials, good compliance with compression is reported in around two thirds of patients, with inferior compliance in those given higher degrees of compression. Further studies are required to identify predictors of non

  13. Shielded battery syndrome: a new hardware complication of deep brain stimulation.

    Science.gov (United States)

    Chelvarajah, Ramesh; Lumsden, Daniel; Kaminska, Margaret; Samuel, Michael; Hulse, Natasha; Selway, Richard P; Lin, Jean-Pierre; Ashkan, Keyoumars

    2012-01-01

    Deep brain stimulation hardware is constantly advancing. The last few years have seen the introduction of rechargeable cell technology into the implanted pulse generator design, allowing for longer battery life and fewer replacement operations. The Medtronic® system requires an additional pocket adaptor when revising a non-rechargeable battery such as their Kinetra® to their rechargeable Activa® RC. This additional hardware item can, if it migrates superficially, become an impediment to the recharging of the battery and negate the intended technological advance. To report the emergence of the 'shielded battery syndrome', which has not been previously described. We reviewed our deep brain stimulation database to identify cases of recharging difficulties reported by patients with Activa RC implanted pulse generators. Two cases of shielded battery syndrome were identified. The first required surgery to reposition the adaptor to the deep aspect of the subcutaneous pocket. In the second case, it was possible to perform external manual manipulation to restore the adaptor to its original position deep to the battery. We describe strategies to minimise the occurrence of the shielded battery syndrome and advise vigilance in all patients who experience difficulty with recharging after replacement surgery of this type for the implanted pulse generator. Copyright © 2012 S. Karger AG, Basel.

  14. Noonan syndrome - a new survey.

    Science.gov (United States)

    Tafazoli, Alireza; Eshraghi, Peyman; Koleti, Zahra Kamel; Abbaszadegan, Mohammadreza

    2017-02-01

    Noonan syndrome (NS) is an autosomal dominant disorder with vast heterogeneity in clinical and genetic features. Various symptoms have been reported for this abnormality such as short stature, unusual facial characteristics, congenital heart abnormalities, developmental complications, and an elevated tumor incidence rate. Noonan syndrome shares clinical features with other rare conditions, including LEOPARD syndrome, cardio-facio-cutaneous syndrome, Noonan-like syndrome with loose anagen hair, and Costello syndrome. Germline mutations in the RAS-MAPK (mitogen-activated protein kinase) signal transduction pathway are responsible for NS and other related disorders. Noonan syndrome diagnosis is primarily based on clinical features, but molecular testing should be performed to confirm it in patients. Due to the high number of genes associated with NS and other RASopathy disorders, next-generation sequencing is the best choice for diagnostic testing. Patients with NS also have higher risk for leukemia and specific solid tumors. Age-specific guidelines for the management of NS are available.

  15. A child of Williams-Beuren syndrome for inguinal hernia repair: Perioperative management concerns

    Directory of Open Access Journals (Sweden)

    Sangeeta Deka

    2016-01-01

    Full Text Available Williams-Beuren syndrome, commonly known as Williams syndrome (WS, is a multi-organ disorder. The principal anomalies of the syndrome are developmental delay, unusual craniofacial dysmorphic features, and cardiovascular anomalies such as valvular or supravalvular aortic stenosis, pulmonary artery stenosis, and coronary insufficiency. Sudden cardiac death during minor procedures even in the absence of gross cardiovascular pathology is the most dreaded complication in these patients. A 7-year-old child with WS was posted for left-sided inguinal hernia repair under general anesthesia. Our article describes the uneventful perioperative course of the patient and highlights the concerns and complications that may be an integral part with the syndrome.

  16. Controversies concerning the antiphospholipid syndrome in obstetrics.

    Science.gov (United States)

    Camarena Cabrera, Dulce María Albertina; Rodriguez-Jaimes, Claudia; Acevedo-Gallegos, Sandra; Gallardo-Gaona, Juan Manuel; Velazquez-Torres, Berenice; Ramírez-Calvo, José Antonio

    Antiphospholipid antibody syndrome is a non-inflammatory autoimmune disease characterized by recurrent thrombotic events and/or obstetric complications associated with the presence of circulating antiphospholipid antibodies (anticardiolipin antibodies, anti-β 2 glycoprotein-i antibodies, and/or lupus anticoagulant. Antiphospholipid antibodies are a heterogeneous group of autoantibodies associated with recurrent miscarriage, stillbirth, fetal growth restriction and premature birth. The diversity of the features of the proposed placental antiphospholipid antibodies fingerprint suggests that several disease processes may occur in the placentae of women with antiphospholipid antibody syndrome in the form of immune responses: inflammatory events, complement activation, angiogenic imbalance and, less commonly, thrombosis and infarction. Because of the disparity between clinical and laboratory criteria, and the impact on perinatal outcome in patients starting treatment, we reviewed the aspects of antiphospholipid antibody syndrome related to obstetric complications and seronegative antiphospholipid antibody syndrome, and their treatment in obstetrics. Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  17. Cervical sympathetic chain schwannoma masquerading as a carotid body tumour with a postoperative complication of first-bite syndrome.

    LENUS (Irish Health Repository)

    Casserly, Paula

    2012-01-31

    Carotid body tumours (CBT) are the most common tumours at the carotid bifurcation. Widening of the bifurcation is usually demonstrated on conventional angiography. This sign may also be produced by a schwannoma of the cervical sympathetic plexus. A 45-year-old patient presented with a neck mass. Investigations included contrast-enhanced CT, MRI and magnetic resonance arteriography with contrast enhancement. Radiologically, the mass was considered to be a CBT due to vascular enhancement and splaying of the internal and external carotid arteries. Intraoperatively, it was determined to be a cervical sympathetic chain schwannoma (CSCS). The patient had a postoperative complication of first-bite syndrome (FBS).Although rare, CSCS should be considered in the differential diagnosis for tumours at the carotid bifurcation. Damage to the sympathetic innervation to the parotid gland can result in severe postoperative pain characterised by FBS and should be considered in all patients undergoing surgery involving the parapharyngeal space.

  18. Does vitamin D affects components of the metabolic syndrome?

    OpenAIRE

    Sevil Karahan Yılmaz; Aylin Ayaz

    2015-01-01

    Metabolic syndrome is a major public health problem which has become increasingly common worlwide with cardiometabolic complications and have high morbidity and mortality. In addition to some genetical features, environmental factors such sedentary lifestyle, improper eating habits constitutes a risk factor for metabolic syndrome. Important components of the metabolic syndrome are dyslipidemia (low HDL levels, high triglycerides level), hyperglycemia, elevated blood...

  19. An update on renal involvement in hemophagocytic syndrome (macrophage activation syndrome).

    Science.gov (United States)

    Esmaili, Haydarali; Mostafidi, Elmira; Mehramuz, Bahareh; Ardalan, Mohammadreza; Mohajel-Shoja, Mohammadali

    2016-01-01

    Hemophagocytic syndrome (HPS) is mainly characterized by massive infiltration of bone marrow by activated macrophages and often presents with pancytopenia. Thrombotic microangiopathy (TMA) is also present with thrombocytopenia and renal involvement. Both conditions could coexist with each other and complicate the condition. Directory of Open Access Journals (DOAJ), EMBASE, Google Scholar, PubMed, EBSCO, and Web of Science with keywords relevant to; Hemophagocytic syndrome, macrophage activation syndrome, interferon-gamma and thrombotic microangiopathy, have been searched. Viral infection, rheumatologic disease and malignancies are the main underlying causes for secondary HPS. calcineurin inhibitors and viral infections are also the main underlying causes of TMA in transplant recipients. In this review, we discussed a 39-year-old male who presented with pancytopenia and renal allograft dysfunction. With the diagnosis of HPS induced TMA his renal condition and pancytopenia improved after receiving intravenous immunoglobulin (IVIG) and plasmapheresis therapy. HPS is an increasingly recognized disorder in the realm of different medical specialties. Renal involvement complicates the clinical picture of the disease, and this condition even is more complex in renal transplant recipients. We should consider the possibility of HPS in any renal transplant recipient with pancytopenia and allograft dysfunction. The combination of HPS with TMA future increases the complexity of the situation.

  20. Broken Heart Syndrome – An intra operative complication

    African Journals Online (AJOL)

    Zara Wani

    2017-03-09

    Mar 9, 2017 ... diomyopathy, Stress Cardiomyopathy1 is an acute reversible left ventricular dysfunction in the absence ... acute coronary syndrome with charac- teristics features of typical chest pain, pulmonary edema, ... Beta-blockers5 and intensive care is the prime mode of treatment, which provides good recovery and ...

  1. Obstructive sleep apnoea syndrome in children

    Directory of Open Access Journals (Sweden)

    Anna Włodarska

    2016-09-01

    Full Text Available Sleep-related breathing disorders in children are a clinical problem which is more and more often diagnosed by doctors nowadays. They can be the basis for diagnosing obstructive sleep apnoea syndrome that causes a number of complications: lowering the quality of life, behavioural problems, complications involving cardiovascular system. The incidence of obstructive sleep apnoea syndrome in the paediatric population is estimated to be at the level of 2%. The symptoms of obstructive sleep apnoea syndrome can be divided into daytime and night ones. Night symptoms in children include: snoring, apnoea, breathing with open mouth (both during the day and at night, dry tongue and mouth during sleep, agitated sleep in unnatural positions. Among daytime symptoms of obstructive sleep apnoea syndrome there are: irritability, aggressiveness, hyperactivity, attention deficit disorder, delayed development and growth pattern (mainly failure to thrive, learning problems, morning headaches. Parents often do not connect the night and daytime symptoms with the possible development of obstructive sleep apnoea syndrome in their children. The main predisposing factor of obstructive sleep apnoea syndrome in children is adenotonsillar hypertrophy. Effective and in most cases preferred treatment for the management of obstructive sleep apnoea syndrome in children is adenotonsillectomy. Polysomnography and polygraphy are diagnostic tools helpful in the study of sleep-related disorders. The objective of this study was to systematise the knowledge on the epidemiology, aetiology, clinical image and prevention of obstructive sleep apnoea syndrome in children.

  2. Johnson syndrome in a Nigerian woman with chronic lymphocytic ...

    African Journals Online (AJOL)

    Stevens-Johnson syndrome is an adverse muco-cutaneous complication arising from a number of conditions which include the administration of some drugs. A female Nigerian with chronic lymphocytic leukaemia, (Binet stage C) who developed Stevens-Johnson syndrome following commencement of allopurinol is ...

  3. The Study of Pulmonary Complication of Neonatal Mechanical Ventilation in NICU

    Directory of Open Access Journals (Sweden)

    M.K. Sabzeie

    2016-01-01

    Full Text Available Introduction & Objective: The main indication of mechanical ventilation is in the treatment of neonates with respiratory failure. With the increased use of mechanical ventilation, its complications have increased too. The aim of this study was to evaluate the prevalence of complications and short-term improvement in infants undergoing mechanical ventilation in the neonatal intensive care unit (NICU. Materials & Methods: In this prospective-analytic study, all infants requiring mechanical ventilation and admitted in the neonatal intensive care unit of Fatemiyeh and Be’sat hospitals, have been evaluated for one year (2012. Their data included: neonatal age, sex, gestational age, birth weight, weight at admission, diagnosis, length of hospitalization, disease outcome (improvement-died, need for mechanical ventilation, complications and culture results (blood, endotracheal tube, urine, CSF insert in check list. The data were analysed by SPSS and c2 statistical test. Results: In this study, a total of 114 infants hospitalized in intensive care unit and needed mechanical ventilation was studied of whom 72 were male and 42 were female. The mean of gestational age in the admitted neonates was 32.9 ± 0.85 weeks. The majority of neonates (80.70% were undergoing mechanical ventilation with respiratory distress syndrome (RDS. 67% of neonates were suffering from complications of mechanical ventilation. The prevalent complication was seen in the neonates was narrowing or obstruction of the endotracheal tube (52.63%. 47.37% of infants died and respiratory distress syndrome was the common cause of death in these neonates (46.29%. In our study, there was significant relationship between resuscitation at birth (P=0.002, time required for mechanical ventilation (P=0.0000 and Apgar score (P=0.0000 and complications of mechanical ventilation. Conclusions: The results show that the high prevalence of pulmonary complications is associated with mechanical

  4. Predictors of clinical complications in patients with spinomedullary injury

    Directory of Open Access Journals (Sweden)

    Dionei Freitas De Morais

    2014-01-01

    Full Text Available Objective: To analyze individuals with spinal cord injury who developed secondary clinical complications, and the variables that can influence the prognosis. Methods: A prospective study of 321 patients with spinal cord injury. The variables were collected: age, sex, cause of the accident, anatomical distribution, neurological status, associated injuries, in-hospital complications, and mortality only in patients who developed complications. Results: A total of 72 patients were analyzed (85% male with a mean age of 44.72±19.19 years. The individuals with spinal cord injury who developed clinical complications were mostly male, over 50 years of age, and the main cause was accidental falls. These patients had longer hospitalization times and a higher risk of progressing to death. Pneumonia was the main clinical complication. With regard to the variables that can influence the prognosis of these patients, it was observed that spinal cord injury to the cervical segment with syndromic quadriplegia, and neurological status ASIA-A, have a higher risk of developing pneumonia, the most common complication, as well as increased mortality. Conclusion: Clinical complications secondary to spinal cord injury are influenced by demographic factors, as well as characteristics of the injury contributing to an increase in mortality.

  5. Larsen syndrome

    Directory of Open Access Journals (Sweden)

    Mohammed Mahbubul Islam

    2016-08-01

    Full Text Available Larsen syndrome is a rare inherited disorder characterized by congenital dislocation of multiple joints along with other anomalies of heart, face, hands and bones. Larsen syndrome was first described in 1950 by Larsen, Schottstaedt and Bost. In the present report, we describe a 10 year old girl who presented with mid facial hypoplasia with depressed nasal bridge, high arched palate, bilateral talipes equinovarus and high arched feet. On examination, she had short stature (HAZ -3.5 SD with hyperextension of knee joint, fixed flexion of elbow joint. Awareness of this condition and associated complications may help in management and follow up of these patients. 

  6. Surgical outcomes of laparoscopic adrenalectomy for patients with Cushing's and subclinical Cushing's syndrome: a single center experience.

    Science.gov (United States)

    Miyazato, Minoru; Ishidoya, Shigeto; Satoh, Fumitoshi; Morimoto, Ryo; Kaiho, Yasuhiro; Yamada, Shigeyuki; Ito, Akihiro; Nakagawa, Haruo; Ito, Sadayoshi; Arai, Yoichi

    2011-12-01

    We retrospectively examined the outcome of patients who underwent laparoscopic adrenalectomy for Cushing's/subclinical Cushing's syndrome in our single institute. Between 1994 and 2008, a total of 114 patients (29 males and 85 females, median age 54 years) with adrenal Cushing's/subclinical Cushing's syndrome were studied. We compared the outcome of patients who underwent laparoscopic adrenalectomy between intraperitoneal and retroperitoneal approaches. Surgical complications were graded according to the Clavien grading system. We also examined the long-term results of subclinical Cushing's syndrome after laparoscopic adrenalectomy. Laparoscopic surgical outcome did not differ significantly between patients with Cushing's syndrome and those with subclinical Cushing's syndrome. Patients who underwent laparoscopic intraperitoneal adrenalectomy had longer operative time than those who received retroperitoneal adrenalectomy (188.2 min vs. 160.9 min). However, operative blood loss and surgical complications were similar between both approaches. There were no complications of Clavien grade III or higher in either intraperitoneal or retroperitoneal approach. We confirmed the improvement of hypertension and glucose tolerance in patients with subclinical Cushing's syndrome after laparoscopic adrenalectomy. Laparoscopic adrenalectomy for adrenal Cushing's/subclinical Cushing's syndrome is safe and feasible in either intraperitoneal or retroperitoneal approach. The use of the Clavien grading system for reporting complications in the laparoscopic adrenalectomy is encouraged for a valuable quality assessment.

  7. Evans Syndrome Complicated by Intratubular Hemoglobin Cast Nephropathy

    Directory of Open Access Journals (Sweden)

    Iván González

    2017-01-01

    Full Text Available Evans syndrome (ES is a rare autoimmune disorder whose exact pathophysiology is unknown. It is characterized by the simultaneous or subsequent development of autoimmune hemolytic anemia (AIHA and immune thrombocytopenia (ITP. Intravascular hemolysis, with hemoglobinemia, is known to produce acute kidney injury; however, the development of intratubular hemoglobin casts (hemoglobin cast nephropathy in the setting of acute hemolysis is uncommon. Likewise, the association of ES and acute renal failure is equally uncommon. We present a case of a 7-year-old girl with ES who developed acute kidney injury in the setting of intravascular hemolysis and had widespread intratubular hemoglobin casts.

  8. Nutrition in clinical practice-the refeeding syndrome: illustrative cases and guidelines for prevention and treatment.

    Science.gov (United States)

    Stanga, Z; Brunner, A; Leuenberger, M; Grimble, R F; Shenkin, A; Allison, S P; Lobo, D N

    2008-06-01

    The refeeding syndrome is a potentially lethal complication of refeeding in patients who are severely malnourished from whatever cause. Too rapid refeeding, particularly with carbohydrate may precipitate a number of metabolic and pathophysiological complications, which may adversely affect the cardiac, respiratory, haematological, hepatic and neuromuscular systems leading to clinical complications and even death. We aimed to review the development of the refeeding syndrome in a variety of situations and, from this and the literature, devise guidelines to prevent and treat the condition. We report seven cases illustrating different aspects of the refeeding syndrome and the measures used to treat it. The specific complications encountered, their physiological mechanisms, identification of patients at risk, and prevention and treatment are discussed. Each case developed one or more of the features of the refeeding syndrome including deficiencies and low plasma levels of potassium, phosphate, magnesium and thiamine combined with salt and water retention. These responded to specific interventions. In most cases, these abnormalities could have been anticipated and prevented. The main features of the refeeding syndrome are described with a protocol to anticipate, prevent and treat the condition in adults.

  9. Apical ballooning syndrome complicated by acute severe mitral regurgitation with left ventricular outflow obstruction – Case report

    Directory of Open Access Journals (Sweden)

    Celermajer David S

    2007-02-01

    Full Text Available Abstract Background Apical ballooning syndrome (or Takotsubo cardiomyopathy is a syndrome of transient left ventricular apical ballooning. Although first described in Japanese patients, it is now well reported in the Caucasian population. The syndrome mimicks an acute myocardial infarction but is characterised by the absence of obstructive coronary disease. We describe a serious and poorly understood complication of Takotsubo cardiomyopathy. Case Presentation We present the case of a 65 year-old lady referred to us from a rural hospital where she was treated with thrombolytic therapy for a presumed acute anterior myocardial infarction. Four hours after thrombolysis she developed acute pulmonary oedema and a new systolic murmur. It was presumed she had acute mitral regurgitation secondary to a ruptured papillary muscle, ischaemic dysfunction or an acute ventricular septal defect. Echocardiogram revealed severe mitral regurgitation, left ventricular apical ballooning, and systolic anterior motion of the mitral valve with significant left ventricular outflow tract gradient (60–70 mmHg. Coronary angiography revealed no obstructive coronary lesions. She had an intra-aortic balloon pump inserted with no improvement in her parlous haemodynamic state. We elected to replace her mitral valve to correct the outflow tract gradient and mitral regurgitation. Intra-operatively the mitral valve was mildly myxomatous but there were no structural abnormalities. She had a mechanical mitral valve replacement with a 29 mm St Jude valve. Post-operatively, her left ventricular outflow obstruction resolved and ventricular function returned to normal over the subsequent 10 days. She recovered well. Conclusion This case represents a serious and poorly understood association of Takotsubo cardiomyopathy with acute pulmonary oedema, severe mitral regurgitaton and systolic anterior motion of the mitral valve with significant left ventricular outflow tract obstruction. The

  10. Combined Pulmonary Fibrosis and Emphysema Syndrome

    Science.gov (United States)

    Rounds, Sharon I. S.

    2012-01-01

    There is increasing clinical, radiologic, and pathologic recognition of the coexistence of emphysema and pulmonary fibrosis in the same patient, resulting in a clinical syndrome known as combined pulmonary fibrosis and emphysema (CPFE) that is characterized by dyspnea, upper-lobe emphysema, lower-lobe fibrosis, and abnormalities of gas exchange. This syndrome frequently is complicated by pulmonary hypertension, acute lung injury, and lung cancer. The CPFE syndrome typically occurs in male smokers, and the mortality associated with this condition, especially if pulmonary hypertension is present, is significant. In this review, we explore the current state of the literature and discuss etiologic factors and clinical characteristics of the CPFE syndrome. PMID:22215830

  11. Marfan Syndrome: A Report of Two Cases and Review

    Directory of Open Access Journals (Sweden)

    K Vinay Kumar Reddy

    2011-01-01

    Full Text Available Marfan syndrome is an autosomal dominant disorder of the connective tissues, resulting in abnormalities of the musculoskeletal system, cardiovascular system and eyes. It has a prevalence of 1 in 100,000 population and occurs in all ethnic groups. It may be familial or due to new mutation (30%, in the fibrillin gene on arm of chromosome 15. It is estimated that one person in every 3000 to 5000 has Marfan′s syndrome may have cardiovascular abnormalities and may be complicated by infective endocarditis. About 90% of Marfan patients will develop cardiac complications.

  12. Features of Polycystic Ovary Syndrome in adolescence.

    Science.gov (United States)

    Tsikouras, P; Spyros, L; Manav, B; Zervoudis, S; Poiana, C; Nikolaos, T; Petros, P; Dimitraki, M; Koukouli, C; Galazios, G; von Tempelhoff, G F

    2015-01-01

    To elucidate the prepubertal risk factors associated with the development of Polycystic Ovary Syndrome (PCOS) and determine the special clinical manifestations of the syndrome in this transitional time of a woman's life. To propose therapeutic targets and regimens, not only to prevent the long-term complications of the syndrome, but also to improve the self-esteem of a young girl who matures into womanhood. A systematic review of literature was performed through electronic database searches (Pubmed, Medline and Embase). Studies published in English-language, peer-reviewed journals from 1996 to 2013 were included. The selected studies focused on the risk factors, the unique features and treatment options of the PCOS in puberty. The pathogenesis of the PCOS was hypothesized to be based on interactions between genetic and certain environmental factors. The diagnosis was usually difficult in young girls. The syndrome was related to a greater risk of future infertility, type II diabetes mellitus, the metabolic syndrome and cardiovascular disease. Early treatment was crucial to prevent the long-term complications of the syndrome, especially infertility and cardiovascular disease. The recognition of the early signs of PCOS during or even before adolescence is of great importance. It is essential to establish the correct diagnosis for PCOS and rule out other causes of androgen excess in young women with hyperandrogenism. The type of treatment applied should be considered on an individual basis. PCOS = Polycystic Ovary Syndrome.

  13. Prevention and Management of Refeeding Syndrome

    Directory of Open Access Journals (Sweden)

    Andika Indrarespati

    2016-12-01

    Full Text Available Refeeding Syndrome is a syndrome which occurs as a result of food administration in poorly nourished individuals. In this syndrome, there are wide range of biochemical alterations, clinical manifestations, and complications, starting from mild (asymptomatic to severe (death. This syndrome was initially proposed in 1950s; however, there is still no agreement for its clear definition, causing clinicians to be less aware and tend to overlook this condition. Clinical manifestations which usually appear include electrolyte imbalances (hypophosphatemia, hypokalemia, hyponatremia, hypomagnesemia and vitamin B1 deficiency. The main principle in management of refeeding syndrome is prevention, where clinicians need to identify this condition in the early stage in high risk individuals, supervision during refeeding, and administration of appropriate diet.

  14. [Anesthetic Management of a Patient Complicated with Marfan Syndrome and Suffering from Stanford Type A Aortic Dissection during Pregnancy].

    Science.gov (United States)

    Uozaki, Nako; Mizuno, Kaori; Shiraishi, Yoshito; Doi, Matsuyuki; Sato, Shigehito

    2015-04-01

    We report a case of a 36-year-old woman at 34 weeks of gestation complicated with Marfan syndrome who underwent Bentall type aortic replacement surgery due to Stanford type A aortic dissection after undergoing caesarean section. Since this patient exhibited severe hypotension before coming to the operating room, it was very difficult to determine whether the cardiac surgery or caesarean section should be performed first. In this case, the caesarean section was performed first, followed by Bentall's surgery. Although intra-aortic balloon pumping and percutaneous cardiopulmonary support were required after weaning from the cardiopulmonary bypass, she was discharged on post-operative day (POD) 40 and the baby was discharged on POD 60, without signs of cerebral palsy. Unfortunately, this patient died on POD 57, due to heart failure. We discuss how to determine the priority of surgeries for patients who require emergency surgery for cardiovascular disease during pregnancy.

  15. Classification of Tattoo Complications in a Hospital Material of 493 Adverse Events

    DEFF Research Database (Denmark)

    Serup, Jørgen; Sepehri, Mitra; Hutton Carlsen, Katrina

    2016-01-01

    agglomeration; 53 (11%) had bacterial infections; 46 (9%) were psycho-social complications; 144 (30%) belonged to several specific diagnostic entities, including photosensitivity, pain syndrome, and lymphopathy. We found no cases of cutaneous or other malignancies. Sarcoidosis was primarily seen in black...

  16. Visceroptosis and the Ehlers-Danlos Syndrome.

    Science.gov (United States)

    Kucera, Stephen; Sullivan, Stephen N

    2017-11-08

    The case of a patient with visceroptosis and Ehlers-Danlos syndrome hypermobility type (RDS-HT) is reported here. The literature on this unusual but probably under-recognized complication is reviewed.

  17. Vitreolensectomy in Marfan's syndrome.

    Science.gov (United States)

    Hubbard, A D; Charteris, D G; Cooling, R J

    1998-01-01

    To evaluate the effectiveness of pars plana vitreolensectomy in the management of subluxed lenses associated with Marfan's syndrome and to assess the need for intraoperative retinal photocoagulation to prevent post-operative retinal detachment. A retrospective review was carried out of 40 eyes of patients with Marfan's syndrome who underwent pars plana vitreolensectomy for subluxed lenses. All patients demonstrated stable or improved visual acuity following surgery with a low incidence of complications. Pars plana vitreolensectomy is a safe and effective treatment for subluxed lenses in patients with Marfan's syndrome. It appears that intraoperative prophylactic laser treatment need only be applied to areas of lattice degeneration to limit the incidence of post-operative retinal detachment.

  18. Sonographic monitoring of complications and anomalies in twin gestations

    International Nuclear Information System (INIS)

    Coleman, B.G.; Grumbach, K.; Arger, P.H.; Mintz, M.C.; Arenson, R.L.; Mennuti, M.T.; Gabbe, S.G.

    1986-01-01

    One hundred sixty-eight twin gestations were evaluated to assess the role of US in the diagnosis of obstetric complications and anomalies. US revealed the following complications: symptomatic polyhydramnios (eight cases), myomas (seven), placenta previa (three), and abruption (five). Fetal anomalies included twin-twin transfusion syndrome (two), acardiac anomaly (two), chromosomal abnormality (two), anencephaly (one), hydrocephaly (one), and conjoined twins (two). In 26 gestations intrauterine fetal demise or neonatal death involved one or both twins for a total of 42 deaths, constituting a mortality of 12.5%. No maternal deaths occurred. The impact of sonographic monitoring on the obstetric management of twin gestations is emphasized

  19. Adult Prader-Willi Syndrome: An Update on Management

    OpenAIRE

    Ho-Ming, Luk

    2016-01-01

    With the advancement of medical care, the survival of most patients with syndromal genetic disease is greatly improved. In this case report, we have reported an adult Prader-Willi syndrome patient who is being diagnosed at the age of 33. The clinical features and their associated complications during adulthood have been reviewed.

  20. Twin-twin transfusion syndrome: etiology, severity and rational management

    NARCIS (Netherlands)

    van Gemert, M. J.; Umur, A.; Tijssen, J. G.; Ross, M. G.

    2001-01-01

    The twin-twin transfusion syndrome is a serious complication of monochorionic twin pregnancies. Partly as a result of an inadequate understanding of the pathophysiology of the syndrome, there is a lack of consensus in clinical management. We sought to review the available information on the etiology

  1. Antiphospholipid Syndrome during pregnancy: the state of the art

    Science.gov (United States)

    Di Prima, Fosca A. F.; Valenti, Oriana; Hyseni, Entela; Giorgio, Elsa; Faraci, Marianna; Renda, Eliana; De Domenico, Roberta; Monte, Santo

    2011-01-01

    Obstetric complications are the hallmark of antiphospholipid syndrome. Recurrent miscarriage, early delivery, oligohydramnios, prematurity, intrauterine growth restriction, fetal distress, fetal or neonatal thrombosis, pre-eclampsia/eclampsia, HELLP syndrome, arterial or venous thrombosis and placental insufficiency are the most severe APS-related complication for pregnant women. Antiphospholipid antibodies promote activation of endothelial cells, monocytes and platelets, causing an overproduction of tissue factor and thromboxane A2. Complement activation might have a central pathogenetic role. These factors, associated with the typical changes in the hemostatic system during normal pregnancy, result in a hypercoagulable state. This is responsible of thrombosis that is presumed to provoke many of the pregnancy complications associated with APS. Obstetric care is based on combined medical-obstetric high-risk management and treatment with the association between aspirin and heparin. This review aims to deter- mine the current state of the art of APS by investigating the knowledge achievements of recent years, to provide the most appropriate diagnostic and therapeutic management for pregnant women suffering from this syndrome. PMID:22439075

  2. Systematic review and meta-analysis of complications and mortality of veno-venous extracorporeal membrane oxygenation for refractory acute respiratory distress syndrome.

    Science.gov (United States)

    Vaquer, Sergi; de Haro, Candelaria; Peruga, Paula; Oliva, Joan Carles; Artigas, Antonio

    2017-12-01

    Veno-venous extracorporeal membrane oxygenation (ECMO) for refractory acute respiratory distress syndrome (ARDS) is a rapidly expanding technique. We performed a systematic review and meta-analysis of the most recent literature to analyse complications and hospital mortality associated with this technique. Using the PRISMA guidelines for systematic reviews and meta-analysis, MEDLINE and EMBASE were systematically searched for studies reporting complications and hospital mortality of adult patients receiving veno-venous ECMO for severe and refractory ARDS. Studies were screened for low bias risk and assessed for study size effect. Meta-analytic pooled estimation of study variables was performed using a weighted random effects model for study size. Models with potential moderators were explored using random effects meta-regression. Twelve studies fulfilled inclusion criteria, representing a population of 1042 patients with refractory ARDS. Pooled mortality at hospital discharge was 37.7% (CI 95% = 31.8-44.1; I 2  = 74.2%). Adjusted mortality including one imputable missing study was 39.3% (CI 95% = 33.1-45.9). Meta-regression model combining patient age, year of study realization, mechanical ventilation (MV) days and prone positioning before veno-venous ECMO was associated with hospital mortality (p < 0.001; R 2  = 0.80). Patient age (b = 0.053; p = 0.01) and maximum cannula size during treatment (b = -0.075; p = 0.008) were also independently associated with mortality. Studies reporting H1N1 patients presented inferior hospital mortality (24.8 vs 40.6%; p = 0.027). Complication rate was 40.2% (CI 95% = 25.8-56.5), being bleeding the most frequent 29.3% (CI 95% = 20.8-39.6). Mortality due to complications was 6.9% (CI 95% = 4.1-11.2). Mechanical complications were present in 10.9% of cases (CI 95% = 4.7-23.5), being oxygenator failure the most prevalent (12.8%; CI 95% = 7.1-21.7). Despite initial severity, significant portion of patients

  3. DETEKSI DAN MANAJEMEN REFEEDING SYNDROME

    Directory of Open Access Journals (Sweden)

    Darmadi Darmadi

    2012-09-01

    Full Text Available AbstrakRefeeding syndrome (RFS dideskripsikan sebagai perubahan biokimiawi, manifestasi klinis dan komplikasi sebagai konsekuensi pemberian nutrisi pada pasien kurang gizi. Refeeding syndrome ini menyebabkan dampak buruk dan kematian. Sindroma ini lebih sering terjadi pada kelompok risiko. Refeeding syndrome merupakan suatu sindroma yang sering tak terdiagnosis oleh karena itu perlu peningkatan pengetahuan dan kesadaran dari tenaga medis untuk mengurangi morbiditas dan mortalitas dari RFS. Karena alasan tersebut ditulis tinjauan pustaka ini yang akan membahas mengenai patofisiologi, faktor risiko, manifestasi klinis, dan manajemennya.Kata kunci : refeeding syndrome, kurang gizi, hipofosfatemia, dukungan nutrisiAbstractRefeeding syndrome (RFS describel as biochemical changes, clinical manifestations, and complications that can occur as a consequence of feeding a malnourished individual. RFS can result in serious harm and death. RFS more commonly occurs in at-risk populations. Increased awareness amongst healthcare professionals is likely to reduce morbidity and mortality. This review examines the pathophysiology, risk factors, clinical manifestations, and management of RFS.Key word : refeeding syndrome, malnourished, hypophosphatemia, nutritional support

  4. Radiation therapy for the prevention of postoperative and traumatic complications

    Energy Technology Data Exchange (ETDEWEB)

    Kishkovskij, A.N.; DudareV, A.L. (Voenno-Meditsinskaya Akademiya, Leningrad (USSR))

    1983-05-01

    An analysis of the results of radiation therapy of 587 patients with postoperative and traumatic complications has shown that special ..gamma..-therapy used at early time following trauma or surgical intervention, with the first clinical signs of an incipient inflammatory process (the so-called ''anticipating'' irradiation), makes it possible to avoid the development of serious postoperative, post-traumatic complications: wound suppuration, fistulas, secondary parotitis, postamputation pain syndrome, ''needle'' osteomyelitis, keloid cicatrix, skin graft rejection, etc. In the author opinion, this promising trend in radiotherapy of nontumorous diseases is worth a wider using in clinical practice.

  5. [Effect of hyperandrogenism on obstetric complications of singleton pregnancy from in vitro fertilization in women with polycystic ovary syndrome].

    Science.gov (United States)

    Wei, D M; Zhang, Z Z; Wang, Z; Li, P; Wang, J F; Liu, Y J; Zhang, J T; Shi, Y H

    2018-01-25

    Objective: To compare the difference in risks of obstetric complications of singleton pregnancy between women with hyperandrogenic polycystic ovary syndrome (PCOS) and women with normoandrogenic PCOS. Methods: Prospective cohort study. This study was a secondary analysis of data collected during a multicenter randomized controlled clinical trial. Women who got clinical singleton pregnancy were grouped according to whether they were diagnosed with hyperandrogenism at baseline. There were 118 women with hyperandrogenism and 366 women without hyperandrogenism. The incidences of obstetric complications and birth weight were compared between the two groups. Results: Women with hyperandrogenic PCOS had a significantly higher risk of preterm delivery than women with normoandrogenic PCOS [12.7% (15/118) versus 3.6% (13/366); OR= 3.94, 95% CI: 1.82-8.56]. After adjustment of age, duration of infertility, body mass index, and fresh or frozen embryo transfer group, hyperandrogenism was still associated with an increased risk of preterm delivery ( OR= 3.67, 95% CI: 1.67-8.07). Compared with women with normoandrogenic PCOS, women with hyperandrogenic PCOS had similar risks of pregnancy loss, gestational diabetes mellitus, pre-eclampsia, placenta previa, and postpartum hemorrhage (all P> 0.05). Birth weight as well as the risks of being small for gestational age and large for gestational age were also comparable between the two groups (all P> 0.05). Conclusion: In women with PCOS and singleton pregnancy, those with preconceptional hyperandrogenism have a higher risk of preterm delivery than those without hyperandrogenism.

  6. Polycystic ovary syndrome: update on diagnosis and treatment.

    Science.gov (United States)

    Setji, Tracy L; Brown, Ann J

    2014-10-01

    Polycystic ovary syndrome is now a well-recognized condition affecting 6%-25% of reproductive-aged women, depending on the definition. Over the past 3 decades, research has launched it from relative medical obscurity to a condition increasingly recognized as common in internal medicine practices. It affects multiple systems, and requires a comprehensive perspective on health care for effective treatment. Metabolic derangements and associated complications include insulin resistance and diabetes, hyperlipidemia, hypertension, fatty liver, metabolic syndrome, and sleep apnea. Reproductive complications include oligo-/amenorrhea, sub-fertility, endometrial hyperplasia, and cancer. Associated psychosocial concerns include depression and disordered eating. Additionally, cosmetic issues include hirsutism, androgenic alopecia, and acne. This review organizes this multi-system approach around the mnemonic "MY PCOS" and discusses evaluation and treatment options for the reproductive, cosmetic, and metabolic complications of this condition. Copyright © 2014 Elsevier Inc. All rights reserved.

  7. Fatal Complications of Aesthetic Techniques: The Gluteal Region.

    Science.gov (United States)

    Rapkiewicz, Amy V; Kenerson, Katherine; Hutchins, Kenneth D; Garavan, Fintan; Lew, Emma O; Shuman, Mark J

    2018-02-21

    Cosmetic procedures are common and utilize many techniques to obtain aesthetically good outcomes for patient satisfaction with acceptable safety standards. Cosmetic procedures that involve the gluteal region are becoming increasingly popular as various procedures can target the gluteal region such as liposuction, tumescent liposuction, cosmetic filler injections, autologous fat transfer, depot drug delivery, and implants. Complications of cosmetic gluteal procedures can be localized or systemic with systemic complications being responsible for most deaths. These reported systemic complications include sepsis, thromboembolism, fat embolism with or without fat embolism syndrome, macroscopic fat embolism, anesthesia-related and blood volume abnormalities. We herein report 10 deaths due to elective gluteal cosmetic procedures. Autologous fat transfer (fat grafting, lipoinjection) following liposuction resulted in 8 of 10 fatal outcomes of the gluteal aesthetic procedures. A comprehensive discussion of gluteal anatomy, gluteal contouring procedures, and the approach to such cases is presented along with the autopsy findings of the reported cases. © 2018 American Academy of Forensic Sciences.

  8. Adult Prader-Willi Syndrome: An Update on Management

    Directory of Open Access Journals (Sweden)

    Luk Ho-Ming

    2016-01-01

    Full Text Available With the advancement of medical care, the survival of most patients with syndromal genetic disease is greatly improved. In this case report, we have reported an adult Prader-Willi syndrome patient who is being diagnosed at the age of 33. The clinical features and their associated complications during adulthood have been reviewed.

  9. Hypertensive Cerebral Hemorrhage in a Patient with Turner Syndrome Caused by Deletion in the Short Arm of the X Chromosome.

    Science.gov (United States)

    Hori, Yusuke S; Ohkura, Takahiro; Ebisudani, Yuki; Umakoshi, Michiari; Ishi, Masato; Oda, Kazunori; Aoi, Mizuho; Inoue, Takushi; Furujo, Mahoko; Tanaka, Hiroyuki; Fukuhara, Toru

    2018-01-01

    Turner syndrome is a chromosomal disorder usually caused by complete deletion of an X chromosome, with deletion in the short arm of the X chromosome being a rare cause of the condition. Patients with Turner syndrome commonly develop hypertension, and associated vascular complications such as aortic dissection or cerebral hemorrhage have been reported. Cerebral hemorrhage in Turner syndrome is a rare complication, and only a few reports have been published. In these reports, all patients have XO karyotypes or a mosaic type as the cause of Turner syndrome, while no other Turner syndrome types have been documented. In this report, we present for the first time a patient with Turner syndrome caused by deletion in the short arm of the X chromosome who experienced hypertensive hemorrhage as a late complication. © 2017 S. Karger AG, Basel.

  10. Gorlin-Goltz syndrome: A rare case report.

    Science.gov (United States)

    Pol, Chetan A; Ghige, Suvarna K; Kalaskar, Ritesh R; Gosavi, Suchitra R

    2013-10-01

    Gorlin-Goltz syndrome is an uncommon autosomal dominant inherited disorder characterized by numerous basal cell carcinomas, odontogenic keratocysts (OKCs) and musculoskeletal malformations. A rare case of this syndrome observed in a 13-year-old male patient is presented in which multiple OKCs were causing disfigurement of the lower jaw as well as displacement and malocclusion of the teeth. Early diagnosis and treatment of this syndrome is important to reduce the severity of complications including cutaneous and cerebral malignancy and oromaxillofacial deformation and destruction due to jaw cysts.

  11. Lowe syndrome

    Directory of Open Access Journals (Sweden)

    Loi Mario

    2006-05-01

    Full Text Available Abstract Lowe syndrome (the oculocerebrorenal syndrome of Lowe, OCRL is a multisystem disorder characterised by anomalies affecting the eye, the nervous system and the kidney. It is a uncommon, panethnic, X-linked disease, with estimated prevalence in the general population of approximately 1 in 500,000. Bilateral cataract and severe hypotonia are present at birth. In the subsequent weeks or months, a proximal renal tubulopathy (Fanconi-type becomes evident and the ocular picture may be complicated by glaucoma and cheloids. Psychomotor retardation is evident in childhood, while behavioural problems prevail and renal complications arise in adolescence. The mutation of the gene OCRL1 localized at Xq26.1, coding for the enzyme phosphatidylinositol (4,5 bisphosphate 5 phosphatase, PtdIns (4,5P2, in the trans-Golgi network is responsible for the disease. Both enzymatic and molecular testing are available for confirmation of the diagnosis and for prenatal detection of the disease. The treatment includes: cataract extraction, glaucoma control, physical and speech therapy, use of drugs to address behavioural problems, and correction of the tubular acidosis and the bone disease with the use of bicarbonate, phosphate, potassium and water. Life span rarely exceeds 40 years.

  12. [Epidemiology, diagnostics, and treatment of complications after neuroinfections: chronic fatigue syndrome].

    Science.gov (United States)

    Verner, O M; Murashko, N K

    2012-01-01

    Epidemiology information which testify to prevalence syndrome of chronic ustalostti (SV) is resulted in the article, and from some data this diagnosis is covered at more than 20% patients which carried neyroinfection. SV meets more frequent only in age 40-59, thus for women a disease is marked in 4 times more frequent, than for men. Today etiology of disease remains unknown, but the value of genetic, immunological factors, pathogens, neurogenic violations and features of feed is examined. Possibility of infectious etiology SV causes considerable interest of researchers, but at first this syndrome was examined as a sharp viral infection, where the most reliable exciter is consider the virus of Epshteyna-barr. Using of intravenous introduction of globulin for SV carries experimental character and grounded on a hypothesis about immunological or infectious etiology of this disease.

  13. The acquired hyperostosis syndrome

    International Nuclear Information System (INIS)

    Dihlmann, W.; Hering, L.; Bargon, G.W.

    1988-01-01

    Sterno-costo-clavicular hyperostosis (SCCH) is the most common manifestation of a syndrome, consisting of increased bone metabolism, mostly new bone formation and heterotopic ossification of fibrous tissue, which we have characterised as the acquired hyperostosis syndrome. In part I we discuss the terminology, radiological appearances, scintigraphy, clinical and laboratory findings, bacteriology, histology, nosology, complications, treatment and differential diagnosis of SCCH. Chronic recurrent multifocal osteomyelitis (CRMO) is regarded as a phaenotype of SCCH, depending on the age. CRMO occurs in children, adolescents and young adults, SCCH predominantly in middleaged and elderly adults. (orig.) [de

  14. How to Diagnose and Classify Tattoo Complications in the Clinic

    DEFF Research Database (Denmark)

    Serup, Jørgen

    2017-01-01

    Tattoo complications represent a broad spectrum of clinical entities and disease mechanisms. Infections are known, but chronic inflammatory reactions have hitherto been inconsistently reported and given many interpretations and terms. A clinical classification system of distinct patterns with emp...... is reported to the 11th revision of the WHO diagnosis classification used as international standard.......Tattoo complications represent a broad spectrum of clinical entities and disease mechanisms. Infections are known, but chronic inflammatory reactions have hitherto been inconsistently reported and given many interpretations and terms. A clinical classification system of distinct patterns...... with sarcoidosis affecting other organs. Tattoo complications include a large group of less frequent but nevertheless specific entities, i.e. irritant and toxic local events, photosensitivity, urticaria, eczematous rash due to soluble allergen, neurosensitivity and pain syndrome, lymphopathies, pigment diffusion...

  15. Posterior reversible encephalopathy syndrome: An atypical postpartum complication

    Directory of Open Access Journals (Sweden)

    Debashish Paul

    2016-01-01

    Full Text Available Posterior reversible encephalopathy syndrome (PRES is presented by headache, altered mental status, blurring of vision, vomiting and seizure in conjunction with radiological finding of posterior cerebral white matter edema. Data suggest that most cases occur in young middle-aged with marked female preponderance, hypertension being the most common cause. In this case, it was diagnosed in a normotensive patient in the postnatal period that underwent cesarean section. The initial symptoms had misled toward a diagnosis of postdural puncture headache. Symptomatic treatment was started immediately in the ICU. This is an interesting case as the patient was a normotensive one without any other contributory factors and there was unanticipated delay in diagnosing the case until the time we could get a magnetic resonance imaging report.

  16. Benign prostatic hyperplasia: clinical treatment can complicate cataract surgery

    Directory of Open Access Journals (Sweden)

    Fernando Facio

    2010-10-01

    Full Text Available PURPOSE: To investigate the effects of alpha-1 adrenergic receptor antagonists for the treatment of benign prostatic hyperplasia (BPH regarding potential risks of complications in the setting of cataract surgery. AIM: To address recommendations, optimal control therapy, voiding symptoms and safety within the setting of cataract surgery. MATERIALS AND METHODS: A comprehensive literature review was performed using MEDLINE with MeSH terms and keywords "benign prostatic hyperplasia", "intraoperative floppy iris syndrome", "adrenergic alpha-antagonist" and "cataract surgery". In addition, reference lists from identified publications were reviewed to identify reports and studies of interest from 2001 to 2009. RESULTS: The first report of intraoperative floppy iris syndrome (IFIS was observed during cataract surgery in patients taking systemic alpha-1 AR antagonists in 2005. It has been most commonly seen related to use of tamsulosin. Changes of medication and washout periods of up to 2 weeks have been attempted to reduce the risk of complications in the setting of cataract surgery. CONCLUSION: Patients under clinical treatment for BPH should be informed about potential risks of this drug class so that it can be discuss with their healthcare providers, in particular urologist and ophthalmologist, prior to cataract surgery.

  17. Noonan syndrome – a new survey

    Science.gov (United States)

    Tafazoli, Alireza; Eshraghi, Peyman; Koleti, Zahra Kamel

    2016-01-01

    Noonan syndrome (NS) is an autosomal dominant disorder with vast heterogeneity in clinical and genetic features. Various symptoms have been reported for this abnormality such as short stature, unusual facial characteristics, congenital heart abnormalities, developmental complications, and an elevated tumor incidence rate. Noonan syndrome shares clinical features with other rare conditions, including LEOPARD syndrome, cardio-facio-cutaneous syndrome, Noonan-like syndrome with loose anagen hair, and Costello syndrome. Germline mutations in the RAS-MAPK (mitogen-activated protein kinase) signal transduction pathway are responsible for NS and other related disorders. Noonan syndrome diagnosis is primarily based on clinical features, but molecular testing should be performed to confirm it in patients. Due to the high number of genes associated with NS and other RASopathy disorders, next-generation sequencing is the best choice for diagnostic testing. Patients with NS also have higher risk for leukemia and specific solid tumors. Age-specific guidelines for the management of NS are available. PMID:28144274

  18. Complications of central venous stenosis due to permanent central venous catheters in children on hemodialysis.

    Science.gov (United States)

    Rinat, Choni; Ben-Shalom, Efrat; Becker-Cohen, Rachel; Feinstein, Sofia; Frishberg, Yaacov

    2014-11-01

    Central venous catheters are frequently used as access for hemodialysis (HD) in children. One of the known complications is central venous stenosis. Although this complication is not rare, it is often asymptomatic and therefore unacknowledged. Superior vena cava (SVC) stenosis is obviously suspected in the presence of upper body edema, but several other signs and symptoms are often unrecognized as being part of this syndrome. We describe four patients with various manifestations of central venous stenosis and SVC syndrome. These sometimes life- or organ-threatening conditions include obstructive sleep apnea, unresolving stridor, increased intracranial pressure, increased intraocular pressure, right-sided pleural effusion, protein-losing enteropathy and lymphadenopathy. The temporal relationship of these complications associated with the use of central venous catheters and documentation of venous stenosis, together with their resolution after alleviation of high venous pressure, points to a causal role. We suggest pathophysiological mechanisms for the formation of each of these complications. In patients with occlusion of the SVC, various unexpected clinical entities can be caused by high central venous pressure. As often the etiology is not obvious, a high index of suspicion is needed as in some cases prompt alleviation of the high pressure is mandatory.

  19. Nasogastric Tube Syndrome: Why Is It Important in the Intensive Care Unit?

    Directory of Open Access Journals (Sweden)

    Taehyun Kim

    2015-08-01

    Full Text Available Although the nasogastric tube (NGT is widely used in critically ill patients, most intensivists do not give much thought to it or its possible complications. NGT syndrome is a rare but fatal complication characterized by throat pain and vocal cord paralysis in the presence of NGT. Recently, we experienced a case of NGT syndrome developed in an 86-year-old female twelve days after NGT insertion. We immediately removed the NGT and secured the airway by tracheostomy. She was treated successfully with an intravenous antibiotic, steroid and proton pump inhibitor and the syndrome did not recur after reinsertion of the NGT.

  20. Aggressive Fluid Resuscitation in Severe Pediatric Hyperglycemic Hyperosmolar Syndrome: A Case Report

    Directory of Open Access Journals (Sweden)

    Sharara-Chami Rana

    2010-03-01

    Full Text Available Objective. This report describes a severe case of hyperglycemic hyperosmolar syndrome complicated by rhabdomyolysis, acute kidney injury, hyperthermia, and hypovolemic shock, with management centred upon fluid administration. Design. Case report. Setting. Pediatric intensive care unit in university teaching hospital. Patients. 12 years old adolescent female presenting with hyperglycemic hyperosmolar syndrome with a new diagnosis of type 2 diabetes mellitus. Intervention. Aggressive fluid resuscitation and insulin. Main results. The patient had a good outcome, discharged home on hospital day 6. Conclusions. Hyperglycemic hyperosmolar syndrome is associated with a number of complications. Management strategies are undefined, given the rarity of its presentation, and further studies are warranted.