269 Refractory Chronic Urticaria Treated with Omalizumab

PubMed Central

Saenz, Martinez; Del Pozo, Ramírez; JJ, Lopez Tiro; Vera, Javier Gomez

2012-01-01

Background Chronic urticaria (CU) is a common disorder characterized by recurrent episodes of urticaria pruritic erythematous lesions, associated with angioedema1. It affects 0.1% of the population, it is estimated that approximately 15 to 25% of the population will have hives at some point in their lives.2 About 80% of UC patients are diagnosed as idiopathic chronic urticaria and that no cause is identified, 3 experiencing deterioration in their quality of life affecting your work, social relationships, schemes requiring multiple medications and doses higher than usual. This study proposes Omalizumab (anti-IgE humanized antibody) as a treatment for Refractory Chronic Urticaria (RCU) Object Demonstrate Omalizumab's effectiveness in the treatment of Refractory Chronic Urticaria. Methods A clinical study, was carried out to evaluate the effectiveness of the Omalizumab's treatment on RCU diagnosed patient, including male and female patients ages 12 to 50 diagnosed with RCU, with Scorad higher tan 30 points. We made a questionnaire to know about the patient's family background, skin symptoms beginning, administration of drugs such sistemic steroids, inmunosupresors, calceurine inhibitors, presence of inmunotherapy and age of start. Omalizumab was administered on doses according patient's weight and IgE levels, bimonthly or monthly according to treatment guides. Severeness level was calculated with scorad every 1 month, with IgE seric level measurement and life quality questionnaire. Results 5 patients diagnosed with RCU were included in the group of Omalizumab and 5 patients in the control group (placebo). All patients were female. A gradual decrease on the life quality score and in Score, with a significant P under 0.05 was observed on all patients treated with omalizumab compared with patient in the group with placebo. Conclusions Treatment with Omalizumab progressively decreases the severeness level on RCU, with a significant improvement on the patient's life

Prevalence of Inducible Urticaria in Patients with Chronic Spontaneous Urticaria: Associated Risk Factors.

PubMed

Sánchez, Jorge; Amaya, Emerson; Acevedo, Ana; Celis, Ana; Caraballo, Domingo; Cardona, Ricardo

Information on the prevalence of inducible urticaria (IU) in patients with chronic spontaneous urticaria (CSU) and the factors affecting this prevalence is scarce in the literature. To estimate the frequency of IU in patients with CSU and to explore possible factors associated with CSU. Patients older than 12 years diagnosed with CSU and a control group with no history of urticaria were recruited from 2 different cities. All patients were questioned about triggers associated with exacerbation of urticaria, and challenge tests were performed for symptomatic dermographism, pressure, cold, water, and exercise. Atopy to mites and self-reactivity to autologous serum were evaluated using skin tests. The study population comprised 245 patients with CSU and 127 controls. Of the patients with CSU, 186 (75.9%) reported a physical trigger, although only 89 (36.3%) had a positive challenge test result. The challenge tests showed that symptomatic dermographism was the most common type of IU, affecting 24.8% of the CSU group, followed by cold, which affected 13.4%. In the control group, 3.9% of patients were positive for symptomatic dermographism. People living in Medellín city had a higher frequency of symptomatic dermographism 28.5% (odds ratio, 2.1; 95% CI, 1-4.4; P = .03) and cold urticaria 16.5% (odds ratio, 3.3; 95% CI, 1.125-9.8; P = .02) than did people living in Bogotá (dermographism 14.4% and cold 5.2%). Atopy and self-reactivity were more frequent in patients with CSU than in the control group. Physical triggers must be verified by challenge tests to avoid unnecessary lifestyle restrictions. Environmental factors such as geographical characteristics could play a key role in the development of some types of IU, whereas atopy and self-reactivity are major risk factors for CSU. Copyright © 2016 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.

Chronic urticaria: aetiology, management and current and future treatment options.

PubMed

Kozel, Martina M A; Sabroe, Ruth A

2004-01-01

Chronic urticaria is a common condition that can be very disabling when severe. A cause for chronic idiopathic urticaria (CIU) is only infrequently identified. Potential causes include reactions to food and drugs, infections (rarely) and, apart from an increased incidence of thyroid disease, uncomplicated urticaria is not usually associated with underlying systemic disease or malignancy. About one-third of patients with CIU have circulating functional autoantibodies against the high affinity IgE receptor or against IgE, although it is not known why such antibodies are produced, or how the presence of such antibodies alters the course of the disease or response to treatment. There are only a few publications relating to childhood urticaria, but it is probably similar to the adult form, except that adult urticaria is more common. The diagnosis is based on patient history and it is vital to spend time documenting this in detail. Extensive laboratory tests are not required in the vast majority of patients. Chronic urticaria resolves spontaneously in 30-55% of patients within 5 years, but it can persist for many years. Treatment is aimed firstly at avoiding underlying causative or exacerbating factors. Histamine H1 receptor antagonists remain the mainstay of oral treatment for all forms of urticaria. The newer low-sedating antihistamines desloratadine, fexofenadine, levocetirizine and mizolastine should be tried first. Sedating antihistamines have more adverse effects but are useful if symptoms are causing sleep disturbance. Low-dose dopexin is effective and especially suitable for patients with associated depression. There is controversy as to whether the addition of an histamine H2 receptor antagonist or a leukotriene antagonist is helpful. For CIU, second-line agents include ciclosporin (cyclosporine) [which is effective in approximately 75% of patients], short courses of oral corticosteroids, intravenous immunoglobulins and plasmapheresis, although the last two were

Validity and responsiveness of the urticaria activity and impact measure (U-AIM), a new patient-reported tool.

PubMed

Maurer, Marcus; Mathias, Susan D; Crosby, Ross D; Rajput, Yamina; Zazzali, James L

2018-03-19

Chronic spontaneous urticaria (CSU), also known as chronic idiopathic urticaria (CIU), may produce hives, itch, and angioedema. The Urticaria Activity and Impact Measure (U-AIM) is a newly developed 9-item patient-reported measure designed for use in routine clinical practice to assess CSU activity and impact over the previous 7 days. To evaluate validity, responsiveness, and clinically meaningful change of the U-AIM. Data from a 24-week open-label single-arm period of a randomized, placebo-controlled study of omalizumab were used to assess the psychometric properties of U-AIM items for itch, hives, and angioedema. 206 patients (75% female, mean age 44.6 years) were enrolled. At baseline, U-AIM results included prevalent severe itch (55%) and >12 hives (67%), angioedema (15%), and bother by itch (84%), hives (84%), and angioedema (49%). Urticaria Patient Daily Diary (UPDD) mean weekly scores were 15.4 (itch severity), 16.8 (number of hives), and 32.2 (Urticaria Activity Score [UAS7]). At baseline, Weeks 12 and 24, U-AIM itch and hives items and UAS7 proxy scores (the sum of itch severity and number of hives over 7 days) demonstrated strong correlation coefficients with their corresponding measures from the UPDD (itch severity: 0.634-0.806; hives number: 0.735-0.843; UAS7 proxy: 0.724-0.852). Changes in U-AIM scores differentiated patients by their perspective of symptom improvement. Meaningful change thresholds were established for itch severity and number of hives scores (0.8-1.0 for both) and the UAS7 proxy score (10.5-12.5). The U-AIM is valid and responsive to change, and may help clinicians monitor CSU activity and track treatment effectiveness. Copyright © 2018. Published by Elsevier Inc.

Investigation of thiol-disulphide balance in patients with acute urticaria and chronic spontaneous urticaria.

PubMed

Akbas, Ayse; Kilinc, Fadime; Sener, Sertac; Aktaş, Akın; Baran, Pervin; Ergin, Merve

2017-09-01

Thiol-disulphide balance plays a major role in health and diseases. This balance may be disrupted by various diseases. We aimed to determine status of the effect of thiol-disulphide balance in urticaria. We aimed to investigate the thiol-disulphide balance in patients with acute urticaria (AUP) and chronic spontaneous urticaria (CSU). Study included 53 AUP and 47 healthy controls plus 57 patients with chronic spontaneous urticaria (CSUP) and 57 healthy controls. Levels of native thiols, disulphides and total thiols were evaluated in plasma using a new and automated spectrophotometric method. Ratios of disulphides/total thiols, disulphides/native thiols and native thiols/total thiols were calculated. For AU, there was no statistical difference compared to control group in levels of native thiols, disulphides and total thiols. For CSU, however, there was an increase in levels of native thiols, disulphides and total thiols and the ratio of thiol/disulphide in favour of disulphide. Thiol-disulphide balance was not affected by AU but shifted towards to disulphide in CSU indicating the presence of oxidative stress (OS).

Galactose-α-1,3-galactose and delayed anaphylaxis, angioedema, and urticaria in children.

PubMed

Kennedy, Joshua L; Stallings, Amy P; Platts-Mills, Thomas A E; Oliveira, Walter M; Workman, Lisa; James, Haley R; Tripathi, Anubha; Lane, Charles J; Matos, Luis; Heymann, Peter W; Commins, Scott P

2013-05-01

Despite a thorough history and comprehensive testing, many children who present with recurrent symptoms consistent with allergic reactions elude diagnosis. Recent research has identified a novel cause for "idiopathic" allergic reactions; immunoglobulin E (IgE) antibody specific for the carbohydrate galactose-α-1,3-galactose (α-Gal) has been associated with delayed urticaria and anaphylaxis that occurs 3 to 6 hours after eating beef, pork, or lamb. We sought to determine whether IgE antibody to α-Gal was present in sera of pediatric patients who reported idiopathic anaphylaxis or urticaria. Patients aged 4 to 17 were enrolled in an institutional review board-approved protocol at the University of Virginia and private practice allergy offices in Lynchburg, VA. Sera was obtained and analyzed by ImmunoCAP for total IgE and specific IgE to α-Gal, beef, pork, cat epithelium and dander, Fel d 1, dog dander, and milk. Forty-five pediatric patients were identified who had both clinical histories supporting delayed anaphylaxis or urticaria to mammalian meat and IgE antibody specific for α-Gal. In addition, most of these cases had a history of tick bites within the past year, which itched and persisted. A novel form of anaphylaxis and urticaria that occurs 3 to 6 hours after eating mammalian meat is not uncommon among children in our area. Identification of these cases may not be straightforward and diagnosis is best confirmed by specific testing, which should certainly be considered for children living in the area where the Lone Star tick is common.

[Physical urticaria].

PubMed

Fleischer, M; Grabbe, J

2004-04-01

The different types of physical urticaria are triggered by mechanical and thermal stimuli, as well as electromagnetic waves. Localized forms restricted to the skin and mucous membranes are most common, but generalized urticaria with variable extracutaneous manifestations can also occur. Physical urticaria is usually sporadic but may rarely have a familial form; it is often associated with chronic urticaria. In most instances, the short time interval between the physical stimulus and reaction points to a causal relationship, but in delayed types the exact diagnosis may be missed without provocation tests. The clinical implication of physical urticaria is demonstrated by investigations showing a greater degree of disability in affected patients as compared to other types of urticaria. There is still an incomplete understanding of the crucial pathophysiological aspects; most likely inflammatory reactions involving leukocytes, endothelial cells and nerves stimulated by various mediators play an important role in this form of urticaria.

[Management of psychologic factors in chronic urticaria. When and how?].

PubMed

Buffet, M

2003-05-01

Chronic idiopathic urticaria is a frequent disease witch treatment is often disappointing. Psychological factors seem to be frequently associated to it. In what cases consider psychological treatment? And according to what modalities? Review of the literature in search of articles in both French and English concerning psychological factors associated to chronic urticaria, either as responsible factors, or as aggravating factors, or as a consequence of the urticaria, with the study of the impact on the quality of life. We also studied articles analyzing various types of psychology-targeted treatments. We use a serie of keywords on following data banks: Medline (1970-2002), Embase, Pascal and Cochrane Library (period 1995-2002). Very few controlled studies were published.: Various studies are found reporting an association between stress, anxiety or depressive symptomatology and CIU, but none can assert a causality. Three controlled opened studies show significantly more anxiety and\\or depression in the chronic urticaria patients. Three studies analyze the psychopathological personalities of the patients with urticaria. Two studies focus specifically on the impact of the CIU on the quality of life. Various psychotropic drugs (mainly tricyclic antidepressants) have been tested, mostly because of their anti-H1 activity. There is no study on psychological support, psychotherapies, behavioral therapies, technique of biofeedback and group therapies. A particular attention is focused to hypnosis and relaxation techniques because of the improvement of the urticarial wheals reported in studies of cutaneous ability to react in subcutaneous injections of histamine. A complementary psychological treatment of patients suffering from CIU seems necessary, because of the high frequency of psychological symptoms. Published studies concern essentially the prescription of psychotropic drugs and the use of therapies with suggestion or relaxation under hypnosis. Prospective studies on

Pneumocystis jirovecii pneumonia induced by low-dose methotrexate in a patient with chronic urticaria.

PubMed

Wang, Sheng-Huei; Tang, Shih-En; Li, Yu-Huei; Wei, Kuang-Yu; Chang, Chan-Yuan

2017-01-01

Methotrexate has immunosuppressive effects and is administered for refractory chronic urticaria. We present a case of Pneumocystis jirovecii pneumonia in a patient with refractory chronic urticaria managed by low-dose weekly methotrexate treatment (total cumulative dose 195mg). Our study highlights the importance of providing prompt diagnosis and treatment of Pneumocystis jirovecii pneumonia in patients with chronic urticaria under methotrexate therapy.

Brain-derived neurotrophic factor is increased in serum and skin levels of patients with chronic spontaneous urticaria.

PubMed

Rössing, K; Novak, N; Mommert, S; Pfab, F; Gehring, M; Wedi, B; Kapp, A; Raap, U

2011-10-01

Chronic spontaneous urticaria is triggered by many direct and indirect aggravating factors including autoreactive/autoimmune mechanisms, infections, non-allergic and pseudoallergic intolerance reactions. However, the role of neuroimmune mechanisms in chronic spontaneous urticaria so far is unclear. Thus, we wanted to address the regulation of the neurotrophin brain-derived neurotrophic factor (BDNF) in serum and inflammatory skin of patients with chronic spontaneous urticaria in comparison to subjects with healthy skin. Fifty adult patients with chronic spontaneous urticaria and 23 skin-healthy subjects were studied. Chronic spontaneous urticaria was defined as recurrent weals for more than 6 weeks. Autologous serum skin test was performed in all patients with chronic spontaneous urticaria and BDNF serum levels were analysed by enzyme immunoassay in all subjects. Furthermore, skin biopsies were taken from weals of eight patients with chronic spontaneous urticaria as well as from healthy skin of eight controls to evaluate the expression of BDNF and its receptors including tyrosine kinase (trk) B and pan-neurotrophin receptor p75(NTR) by immunohistochemistry. BDNF serum levels were detectable in all subjects studied. However, BDNF levels were significantly higher in patients with chronic spontaneous urticaria compared to non-atopic skin-healthy controls (P<0.001). Furthermore, epidermal and dermal expression of BDNF and epidermal expression of p75(NTR) was significantly higher in patients with chronic spontaneous urticaria compared with controls (P<0.05-0.001). There was no difference with regard to the expression of trkB between chronic spontaneous urticaria and controls and no difference in BDNF serum levels between autologous serum skin test-positive (n=23) and -negative (n=27) patients with chronic spontaneous urticaria. This study shows that BDNF is increased in serum and diseased skin of patients with chronic spontaneous urticaria, suggesting a role for

Strongyloidiasis and chronic urticaria

PubMed Central

Corsini, A. C.

1982-01-01

Two patients with strongyloidiasis developed chronic urticaria. The cases provide support for inappropriate interactions of the effector mechanisms in helminthiasis resulting in urticaria in man. PMID:7111108

Chronic urticaria and autoimmunity: associations found in a large population study.

PubMed

Confino-Cohen, Ronit; Chodick, Gabriel; Shalev, Varda; Leshno, Moshe; Kimhi, Oded; Goldberg, Arnon

2012-05-01

Chronic urticaria (CU) is a common disease in which most cases were considered to be idiopathic. Recent evidence indicates that at least a subset of cases of chronic idiopathic urticaria are autoimmune in origin. We aimed to characterize the association between CU, autoimmune diseases, and autoimmune/inflammatory serologic markers in a large unselected population. Data on 12,778 patients given a diagnosis of CU by either allergy or dermatology specialists during 17 years in a large health maintenance organization in Israel were collected. For each patient, we collected information on diagnosis of major, well-defined autoimmune diseases and autoimmunity- and inflammatory-related serologic markers. Similar data were collected for a control group comprised of 10,714 patients who visited dermatologists, family physicians, or allergy specialists and had no indication of CU. Having CU was associated with an increased odds ratio for hypothyroidism, hyperthyroidism, and antithyroid antibodies. Female patients with CU had a significantly higher incidence of rheumatoid arthritis, Sjögren syndrome, celiac disease, type I diabetes mellitus, and systemic lupus erythematosus, mostly diagnosed during the 10 years after the diagnosis of CU. High mean platelet volume, positive rheumatoid factor, and antinuclear antibodies were all significantly more prevalent in patients with CU. A strong association was found between CU and major autoimmune diseases. A common pathogenic mechanism is implied by the high prevalence of autoantibodies and the existence of a chronic inflammatory process expressed by the high mean platelet volume. These findings have implications for the diagnosis, management, and prognosis of patients with CU. Copyright © 2012 American Academy of Allergy, Asthma & Immunology. Published by Mosby, Inc. All rights reserved.

Urticaria Multiforme

PubMed Central

Bernardo, Sebastian G.; Kovalerchik, Olga; Ahmad, Moneeb

2013-01-01

Urticaria multiforme is a benign cutaneous hypersensitivity reaction seen in pediatric patients that is characterized by the acute and transient onset of blanchable, annular, polycyclic, erythematous wheals with dusky, ecchymotic centers in association with acral edema. It is most commonly misdiagnosed as erythema multiforme, a serum-sickness-like reaction, or urticarial vasculitis. Since these three diagnoses represent distinct clinical entities with unique prognoses and management strategies, it is important that physicians distinguish urticaria multiforme from its clinical mimics in order to optimize patient care. By performing a thorough history and physical examination, the astute clinician can make the correct diagnosis and develop an appropriate, effective treatment plan while avoiding unnecessary biopsies and laboratory evaluations. The authors report a case of urticaria multiforme in a four-year-old girl in order to emphasize the distinctive morphological manifestations of this rare, albeit unique, disease seen in the pediatric population. PMID:23556035

Urticarial Reactions: Vascular Erythema, Urticaria, Vasculitis

PubMed Central

Sibbald, R. Gary

1987-01-01

Vascular erythemas, including urticaria and vasculitis, represent diagnostic and therapeutic challenges. A careful systemic approach to history and physical examination should be followed by appropriate investigations to rule out systemic disease. Chronic urticaria patients should be physically tested to identify cholinergic, dermagraphic, and cold-induced responses. Food diaries and careful drug history may be important to identify exacerbating factors in chronic urticaria. A skin biopsy is necessary to diagnose vasculitis. Therapy of any underlying cause is supplemented with H1 antihistamines in urticaria patients, while vasculitis requires a different anti-inflammatory approach. PMID:21263956

Association between chronic urticaria and self-reported penicillin allergy.

PubMed

Silverman, Susanna; Localio, Russell; Apter, Andrea J

2016-04-01

Penicillin allergy is the most commonly reported drug allergy and often presents with cutaneous symptoms. Other common diagnoses, such as chronic urticaria, may be falsely attributed to penicillin allergy. Because chronic urticaria is fairly common in the general population, evaluation of its prevalence in patients with self-reported penicillin allergy was of interest. Similarly, the prevalence of self-reported penicillin allergy in patients with chronic urticaria is not well known and also becomes interesting in light of the high prevalence of self-reported penicillin allergy in the general population. To determine the prevalence of self-reported penicillin allergy in patients with chronic urticaria and the prevalence of chronic urticaria in patients with self-reported penicillin allergy. This was a retrospective medical record review of 11,143 patients completed using the electronic health record of the University of Pennsylvania Allergy and Immunology clinic. The prevalence of self-reported penicillin allergy in patients with chronic urticaria was found to be approximately 3 times greater than in the general population. The prevalence of chronic urticaria in patients with self-reported penicillin allergy was also found to be approximately 3 times greater than in the population. This link between chronic urticaria and self-reported penicillin allergy highlights the need for clinicians to inquire about self-reported penicillin allergy in patients with chronic urticaria and to consider penicillin skin testing. Furthermore, patients who report penicillin allergy might actually have chronic urticaria, indicating the importance of inquiring about chronic urticaria symptoms in patients with self-reported penicillin allergy. Copyright © 2016 American College of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.

CHRONIC URTICARIA

PubMed Central

Sachdeva, Sandeep; Gupta, Vibhanshu; Amin, Syed Suhail; Tahseen, Mohd

2011-01-01

Chronic urticaria (CU) is a disturbing allergic condition of the skin. Although frequently benign, it may sometimes be a red flag sign of a serious internal disease. A multitude of etiologies have been implicated in the causation of CU, including physical, infective, vasculitic, psychological and idiopathic. An autoimmune basis of most of the ‘idiopathic’ forms is now hypothesized. Histamine released from mast cells is the major effector in pathogenesis and it is clinically characterized by wheals that have a tendency to recur. Laboratory investigations aimed at a specific etiology are not always conclusive, though may be suggestive of an underlying condition. A clinical search for associated systemic disease is strongly advocated under appropriate circumstances. The mainstay of treatment remains H1 antihistaminics. These may be combined with complementary pharmacopeia in the form of H2 blockers, doxepin, nifedipine and leukotriene inhibitors. More radical therapy in the form of immunoglobulins, plasmapheresis and cyclophosphamide may be required for recalcitrant cases. Autologous transfusion and alternative remedies like acupuncture have prospects for future. A stepwise management results in favorable outcomes. An update on CU based on our experience with patients at a tertiary care centre is presented. PMID:22345759

Prevalence and relevance of the positivity of skin prick testing in patients with chronic urticaria.

PubMed

Kulthanan, Kanokvalai; Jiamton, Sukhum; Rutnin, Ni-on; Insawang, Metavee; Pinkaew, Sumrauy

2008-06-01

Many patients with chronic urticaria (CU) worry that foods or other allergens are responsible for their urticaria. Skin prick testing (SPT) may be one of the investigations used to provide a clear illustration. The purpose of our study was to assess the prevalence of positivity of SPT to food allergens and aeroallergens and their relevance in patients with CU, in order to demonstrate the diagnostic value of SPT in CU. We retrospectively reviewed case record forms of patients with chronic ordinary urticaria who underwent SPT in the Urticaria Clinic, Siriraj Hospital, during the period 2000-2004. The studied allergens included 16 food allergens and 12 aeroallergens. Eighty-eight patients were enrolled. The prevalence of positive SPT among patients with CU was 47.7%. Patients who had personal histories of atopy had statistically significant positive SPT results compared with patients who had negative SPT. Of 88 patients, 26 patients (30%) gave positive results to food allergens, 36 patients (41%) gave positive results to aeroallergens and 20 patients (22.7%) gave positive results to both food and aeroallergens. One-third of the subjects (34.6%) who had positive SPT results to food allergens had clinical relevance of food allergy in some systems but only one patient had clinical relevance of food-induced urticaria. Half of the patients who had a history of aeroallergen sensitivity gave a positive SPT response for aeroallergens; however, there was no clinical relevance to their CU. Our study showed that the prevalence of positive SPT to food allergens and aeroallergens in patients with CU was common but had little clinical relevance to CU.

Dissociation between history and challenge in patients with physical urticaria.

PubMed

Komarow, Hirsh D; Arceo, Sarah; Young, Michael; Nelson, Celeste; Metcalfe, Dean D

2014-01-01

Physical urticaria is a subtype of chronic urticaria induced by a physical stimulus. To evaluate the consistency between a history of physical urticaria and results of challenge testing. Seventy-six subjects, ages 3 to 77 years old, were referred with the diagnosis of a physical urticaria and were evaluated by using challenge testing directed toward the presenting diagnosis, yet included other stimuli based on history. The majority of subjects were tested to 3 or more stimuli, thus 294 provocation tests were performed. Fifty-seven subjects were surveyed for the status of their physical urticaria at least 1 year after initial evaluation. Of the 76 subjects with a positive history of a physical urticaria, 38% (n = 29) were challenge negative to the presenting diagnosis. Eight subjects within the challenge negative group reacted positively to additional testing, thus 28% (n = 21) remained negative to all challenge testing, which allowed discontinuation of medications and avoidance behavior. A negative challenge result was less likely with subjects who presented with cold-induced urticaria (25%), delayed pressure urticaria (25%), and dermatographism (29%), yet more common with cholinergic (65%) and solar urticaria (67%). A 1-year follow-up survey of 57 subjects was consistent with initial results. Nineteen of this subgroup were rechallenged for the presenting diagnosis, and the outcome was unchanged in 17 subjects and, in 2 subjects the urticaria had resolved. The diagnosis by history of a physical urticaria should be verified by testing whenever possible and particularly if the condition is judged as severe and thus requires both significant life-style changes and pharmacologic intervention. Published by Elsevier Inc.

T cell activity in successful treatment of chronic urticaria with omalizumab

PubMed Central

2011-01-01

Omalizumab, a humanized monoclonal anti-IgE antibody has the potential to alter allergen processing. Recently, it has been postulated the assessment of PHA-stimulated adenosine triphosphate (ATP) activity as maker of CD4+ T cells activity in peripheral blood cells. We present the case report of a 35-year-old woman with a history of chronic idiopathic urticaria and angioedema of 8 years of development with poor response to treatment. The patient was partially controlled with cyclosporine at doses of 100 mg/12 h. However, she was still developing hives daily. Finally treatment with omalizumab was started at dose of 300 mg every 2 weeks. The patient experienced a decrease in urticarial lesions 2 days after starting therapy. We also evaluated the effects of omalizumab therapy on the activity of peripheral blood CD4+ T cells from the patient, in order to determine the potential modification of anti-IgE therapy on the process of antigen presentation-recognition. Activity of CD4+ cells by ATP release was clearly increased demonstrating an enlarged CD4 activity. Omalizumab may be useful in the treatment of severe chronic urticaria. ATP activity of peripheral blood CD4+ T cells might be a non-subjective method to assess Omalizumab activity. PMID:21791043

584 Frequency of Autoreactivity Demonstrated by Autologous Serum Skin Test in Patients with Chronic Urticaria in the Valley of Mexico During a Period of 8 Years

PubMed Central

Rojo Gutiérrez, María Isabel; Mellado-Abrego, Jaime; Gonzalez-Ibarra, Misael; Sandoval, Teresa; Bermejo, Mario-Alberto

2012-01-01

Background Urticaria is a skin disease characterized by rapid emergence of hives, accompanied or not with angioedema. Usually lasts less than 24 hours. Approximately 12 to 24 per cent of the population will have hives or angioedema at least once in their life. Some patients with chronic urticaria has been classified as autoimmune. The autologous serum skin test (ASST) has been used to show pro-inflammatory circulating endogenous factors and it is regarded as a test for autorreactividad. The autorreactividad does not define an autoimmune urticaria, but may be an indicator of the presence of auto- reactive antibodies with the capacity of to activate the mast cells however functional antibodies need to be confirmed through of release of basophils histamine test from basophils (BHRA) and its specificity immunoassay (Western Blot or ELISA)-confirmed. Objective: to evaluate the auto-reactivity by autologous serum skin test in patients with chronic urticaria idiopathic in a study of 8 years. Material and methods: we made 216 ASST and autologous plasma skin test (APST) in patients with chronic urticaria without specific cause identified, of any age, during the period of 2003 to 2011. Results Thirty five thousand patients were evaluated only 261 patients not identified the cause (0.6%) and we realized ASST, of these 190 (88%) were negative, and 26 (12%) were positive, 20 (76.9 %) were female and 6 (23.1%) male, the median of age for women was 30 years ago with medium of 28 and men average 29 years and median 20. Of the 26 patients one was positive for anti-thyroglobulin senior titles (1: 170) .dos with positive anti unclears and one with pANCA and cANCA positive of a total of 216 patients, 156 (72.2) had APST and 60 (27.8%) were positive, 46 (76.6%) women and 14 (23.3 %) men. Mc Neman concordance between tests P < 0.0001 and Kappa index gives us a highly significant concordance P < 0.0001. The correlation between ASST and APST by Sperman was high significance value of P < 0

The Correlation of Serums CCL11, CCL17, CCL26, and CCL27 and Disease Severity in Patients with Urticaria.

PubMed

Lu, Tao; Jiao, Xiaoyang; Si, Mengya; He, Ping; Zou, Jinbo; Zhang, Shuping; Zeng, Kang

2016-01-01

Chemokines may be involved in the pathogenesis of urticaria, but their correlation with disease severity as well as eruption type is unclear. The aim of this study was to explore the expression of chemokines in patients with urticaria. The association between disease severity and levels of chemokines was analysed. Serums CCL11, CCL17, CCL26, and CCL27, D-dimer, C-reactive protein, and total IgE were measured in 51 patients with urticaria and in 25 healthy control subjects. Serums CCL11, CCL17, CCL26, and CCL27 were significantly higher in patients with urticaria than in the healthy controls (P < 0.05). Serum CCL27 strongly correlated with urticarial disease severity. Serums CCL17, CCL26, and CCL27 significantly correlated with D-dimer, while innercorrelations were noted among the chemokines. Our findings reveal that chemokines participate in the pathogenesis of urticaria. Further study in larger cohort is needed to testify whether they could be the biomarkers for predicting the severity of urticaria.

Primary acquired cold urticaria.

PubMed

Lee, Chyh-Woei; Sheffer, Albert L

2003-01-01

Primary acquired cold urticaria (ACU) is the most common type of cold urticaria characterized by rapid onset of pruritic hives, swelling, and possible severe systemic reactions including hypotension and shock after cold exposure. Primary ACU is diagnosed by history of such symptoms, a positive immediate cold-contact stimulation test, and negative laboratory evaluation for underlying systemic disorders. Clinicians should be aware that patients with ACU may be susceptible to life-threatening systemic reactions especially during aquatic activities and that proper patient education is extremely important. This article reviews the clinical presentation, pathogenesis, diagnosis, and management of primary ACU.

[From psychosomatic disorder to autoimmune disease--50 years urticaria and Quincke edema].

PubMed

Wyss, M

1998-09-30

While the hereditary angioneurotic edema is a rare but serious disorder 10 to 25% of the population experience urticaria during a lifetime. Urticaria is for the patient a very impressive disease. He therefore has a great desire to know its cause. In acute urticaria this is usually possible since drugs or specific foods are the most common triggers. In chronic urticaria the search for a cause is much more difficult and successful only in 20 to 30% of cases. Over the past years it has been proven that about 30% of patients with chronic urticaria have antibodies against the high affinity Fc-receptor of mast cells. Thus a fraction of patients with chronic urticaria formerly often associated with psychosomatic illness suffer in fact from an autoimmune disorder.

Urticaria: "You're Probably Just Allergic to Something".

PubMed

Smallwood, Jordan

2016-11-01

Urticaria is a common symptom seen in pediatric patients, and it has multiple allergic and nonallergic causes. Unfortunately, it is far too common that when children present acutely for urticaria, they are told that it is an "allergy." This statement often leads to increased anxiety while the patient waits to be evaluated by an allergist/immunologist. This article discusses the frequency that allergic reactions are involved in urticaria and provides examples of potential nonallergic causes. Additionally, the article discusses approaches to treatment that may be appropriate to initiate in the pediatrician's office or acute setting. This article is intended to provide a broader understanding of urticaria and its management in the outpatient or emergency setting so that we are able to tell our patients more than"you're probably just allergic to something." [Pediatr Ann. 2016;45(11):e399-e402.]. Copyright 2016, SLACK Incorporated.

The use of a responder analysis to identify clinically meaningful differences in chronic urticaria patients following placebo- controlled treatment with rupatadine 10 and 20 mg.

PubMed

Giménez-Arnau, A; Izquierdo, I; Maurer, M

2009-09-01

According to the EAACI/GA(2)LEN/EDF guidelines for urticaria management, modern non-sedating H1-antihistamines are the first-line symptomatic treatment for chronic urticaria. Two previous randomized clinical trials demonstrated rupatadine efficacy and safety in chronic urticaria treatment. However, a responder analysis to identify clinically meaningful differences in patients with chronic urticaria has not yet been performed. This analysis includes the pooled data from two randomized, double-blind, placebo-controlled, multicentre studies in which chronic urticaria patients were treated with rupatadine at different doses. Responder rates were defined as the percentage of patients after 4 weeks of treatment who exhibited a reduction of symptoms by at least 50% or 75% as compared to baseline. The variables analysed were as follows: Mean Pruritus Score (MPS), Mean Number of Wheals (MNW), and Mean Urticaria Activity Score (UAS). A total of 538 patients were included. This responder analysis, using different response levels, shows that the efficacy of rupatadine 10 mg and 20 mg is significantly better as compared to placebo in the treatment of chronic urticaria patients. Notably, treatment with rupatadine 20 mg daily resulted in a higher percentage of patients with response of 75% symptom reduction or better than rupatadine 10 mg. Our results support the use of higher than standard doses of non sedating antihistamines in chronic urticaria. We strongly recommend performing and reporting responder analyses for established and new drugs used by patients with chronic urticaria.

Natural course of new-onset urticaria: Results of a 10-year follow-up, nationwide, population-based study.

PubMed

Eun, Sang Jun; Lee, Jin Yong; Kim, Do-Yeop; Yoon, Hyun-Sun

2018-06-23

Previous epidemiologic studies of the natural course of urticaria mainly focused on chronic spontaneous urticaria and were conducted at hospitals. The natural course of new-onset urticaria in the general population is unknown. Patients with new-onset urticaria were identified from the National Health Insurance Service-National Sample Cohort data. Patients who had at least one visit for urticaria in 2002 and 2003 were excluded and the study cohort consisted of 1,027,620 subjects with no history of urticaria. We analyzed cumulative incidences of urticaria, chronic urticaria, and chronic urticaria remission using the life table estimation method from 2004 to 2013. Their association with related factors was analyzed using the Cox proportional hazards analysis. From 2004 to 2013, a total of 49,129 patients with new-onset urticaria were identified. The 10-year cumulative incidence rate of urticaria for the general population was 4.9% and that of chronic urticaria among patients with new-onset urticaria was 7.8%. Remission rates of chronic urticaria were 52.6% at 1 year and 88.9% at 5 years. Age, sex, residential area, and autoimmune thyroid disease were significantly associated with urticaria or chronic urticaria, but not with chronic urticaria remission, after adjusting for covariates. Female individuals were more likely to have new-onset urticaria but less likely to develop chronic urticaria compared with male individuals. During the 10-year follow-up period, only a small proportion of patients with new-onset urticaria developed chronic urticaria. Remission was achieved in the majority of patients with chronic urticaria regardless of demographic characteristics or accompanying thyroid disease. Copyright © 2018 Japanese Society of Allergology. Production and hosting by Elsevier B.V. All rights reserved.

Late Onset of Acute Urticaria after Bee Stings.

PubMed

Asai, Yuko; Uhara, Hisashi; Miyazaki, Atsushi; Saiki, Minoru; Okuyama, Ryuhei

2016-01-01

Here we report the cases of five patients with a late onset of acute urticaria after a bee sting. The ages of the five Japanese patients ranged from 33 to 86 years (median: 61). All patients had no history of an allergic reaction to bee stings. The onset of urticaria was 6-14 days (median: 10) after a bee sting. Although four of the patients did not describe experiencing a bee sting at their presentation, the subsequent examination detected anti-bee-specific IgE antibodies. So, we think a history of a bee sting should thus be part of the medical interview sheet for patients with acute urticaria, and an examination of IgE for bees may help prevent a severe bee-related anaphylactic reaction in the future.

Serum eotaxin levels in patients with chronic spontaneous urticaria.

PubMed

Tedeschi, A; Asero, R; Lorini, M; Marzano, A V; Cugno, M

2012-10-01

Eotaxin is a potent agonist for CC chemokine receptor 3 that can attract eosinophils at sites of inflammation. Given the potential role of eosinophils in chronic spontaneous urticaria (CU), we measured serum eotaxin levels together with C-reactive protein in 100 CU patients who were characterized according to autologous serum skin test (ASST) and disease severity. Serum eotaxin concentration was significantly higher in CU patients (median 140.1 pg/ml, range 33.7-718.7 pg/ml) than in 45 healthy controls (median 108.9 pg/ml, range 45.5-409.4 pg/ml) (p = 0.032) Serum eotaxin concentration was not significantly different in ASST-positive and ASST-negative patients as well as in patients with different urticaria activity scores. However, eotaxin levels tended to be higher in patients with intense symptoms. In the 7 patients observed during CU exacerbation and during remission, eotaxin serum levels tended to decrease during remission, although statistical significance was not reached (median concentration decreased from 170.0 pg/ml to 123.8 pg/ml). CRP levels were not significantly different in CU patients and healthy subjects, although there was a trend towards higher levels in the former population. Furthermore, in the 7 patients observed during CU exacerbation and during remission, CRP levels decreased significantly during remission (median concentration dropped from 4.1 microg/ml to 0.7 microg/ml, p = 0.015). No significant correlation was found between eotaxin and CRP serum levels. These findings indicate that serum eotaxin levels are increased in CU patients, although they do not reflect strictly disease activity. A role for eotaxin in eosinophil attraction and activation in CU can be envisaged.

Diagnosis and Treatment of Urticaria and Angioedema: A Worldwide Perspective

PubMed Central

2012-01-01

Urticaria and angioedema are common clinical conditions representing a major concern for physicians and patients alike. The World Allergy Organization (WAO), recognizing the importance of these diseases, has contributed to previous guidelines for the diagnosis and management of urticaria. The Scientific and Clinical Issues Council of WAO proposed the development of this global Position Paper to further enhance the clinical management of these disorders through the participation of renowned experts from all WAO regions of the world. Sections on definition and classification, prevalence, etiology and pathogenesis, diagnosis, treatment, and prognosis are based on the best scientific evidence presently available. Additional sections devoted to urticaria and angioedema in children and pregnant women, quality of life and patient-reported outcomes, and physical urticarias have been incorporated into this document. It is expected that this article will supplement recent international guidelines with the contribution of an expert panel designated by the WAO, increasing awareness of the importance of urticaria and angioedema in medical practice and will become a useful source of information for optimum patient management worldwide. PMID:23282382

Epidemiology of urticaria in Poland - nationally representative survey results.

PubMed

Raciborski, Filip; Kłak, Anna; Czarnecka-Operacz, Magdalena; Jenerowicz, Dorota; Sybilski, Adam; Kuna, Piotr; Samoliński, Bolesław; Wg, Eup

2018-02-01

The prevalence of urticaria is 15-20%. Women are twice as likely to be affected. To present the epidemiology of urticaria and angioedema in Poland. A questionnaire-based survey was conducted on a representative group of 4,897 individuals, aged 15-74 years. A total of 11.2% of respondents reported at least one episode of urticaria symptoms in their life, and the frequency was higher in females (OR = 1.46; p < 0.001). Single episodes accounted for 34.8%, while repeated episodes accounted for 65.2%. Acute urticaria was observed in 10.6% (more frequent in females; OR = 1.41; p < 0.001), chronic in 0.6%. Median age of the first episode was 15 years. Angioedema was observed in 3.2%. The last episode of urticaria symptoms and/or angioedema occurred within the previous week in 0.7% of subjects, the last month in 1%, and the last year in 2.2%. 8.8% had experienced urticaria symptoms or angioedema over 1 year prior to the survey. The impact of urticaria symptoms on patients' daily activities was rated with 3.8 points on a scale of 0-10. In addition, chronic urticaria was rated 6.5, and acute urticaria 3.6. Angioedema and urticaria had a negative impact of 5.7 points. Urticaria and angioedema are serious health problems in Poland, although their prevalence is below the mean rate for the global population. Females are more likely to suffer from these diseases. People affected by urticaria most frequently report contact with plants and substances, insect bites/stings, and food as the provoking factors.

A neglected cause for chronic spontaneous urticaria in children: Helicobacter pylori.

PubMed

Akelma, A Z; Cizmeci, M N; Mete, E; Tufan, N; Bozkurt, B

2015-01-01

The aetiology of chronic urticaria is usually considered idiopathic. There is a paucity of research both on the prevalence of Helicobacter pylori infection in the aetiology of chronic spontaneous urticaria (CU) in children and also on which patients H. pylori should be investigated. All paediatric and adult patients who presented to the allergy outpatient clinic due to CU between January 2011 and July 2012 were included in this prospective, randomised study. Stool samples from all patients were examined for the H. pylori antigen. Paediatric and adult patients who had a positive stool test for the H. pylori antigen were reassessed following eradication therapy. Thirty-two children with CU and 35 adults with CU were enrolled in the study. Ten of the 32 (31.2%) children and 18 of the 35 (51.4%) adults were H. pylori positive (p=0.09). All children with positive-H. pylori were older than eight years of age. There was a significant positive correlation between age and the frequency of H. pylori infection (p<0.001; r=0.61). The presence of H. pylori was not significantly associated with the presence of GI (gastrointestinal) symptoms (p>0.05). Following H. pylori eradication, urticarial symptoms recovered in 15 of the adults (83.3%) and 10 of the paediatric (100%) patients (p=0.172). In the current study we found that H. pylori is common among children with CU, particularly after eight years of age. We suggest that CU patients with an unknown aetiology should be routinely screened for H. pylori even if they do not present with GI symptoms and that those with H. pylori-positive results may receive treatment. Copyright © 2013 SEICAP. Published by Elsevier Espana. All rights reserved.

Bilastine for the treatment of urticaria.

PubMed

Jáuregui, Ignacio; Ferrer, Marta; Bartra, Joan; del Cuvillo, Alfonso; Dávila, Ignacio; Montoro, Javier; Mullol, Joaquim; Sastre, Joaquín; Valero, Antonio

2013-08-01

Urticaria is a highly prevalent disease among people. First-choice treatment continues to be centred on the second-generation H1 antihistamines, including a wide group of drugs with a better therapeutic index (or risk:benefit ratio) than the classic ones, even in the high, off-label dosage occasionally required in chronic urticaria. Bilastine is a newly registered H1-antihistamine for treatment of allergic rhinoconjunctivitis and urticaria. With established antihistaminic and antiallergic properties, it is widely reviewed in the medical literature; however, to our knowledge, a specific review of bilastine's role in the treatment of urticaria was lacking. This article reviews the medical literature on the effectiveness and safety of bilastine in urticarial syndromes, either spontaneous or inducible, by means of a Medline search from 1990 to present, completed with some nonpublished data provided by the manufacturer. Once-daily treatment with bilastine 20 mg is effective in managing symptoms and improving patient's quality of life in chronic urticaria, with at least comparable efficacy to levocetirizine. As far as studies in healthy volunteers, clinical assays, and recent clinical experience can establish, bilastine's safety profile is adequate, appearing to be entirely free from cardiovascular effects, and not impairing psychomotor performance or actual driving, even at twice the therapeutic dose.

The protective effect of Lactobacillus and Bifidobacterium as the gut microbiota members against chronic urticaria.

PubMed

Rezazadeh, Akram; Shahabi, Shahram; Bagheri, Morteza; Nabizadeh, Edris; Jazani, Nima Hosseini

2018-06-01

Chronic Urticaria is a common disorder which is defined by recurrent occurrence of wheals and sometimes angioedema. It has a notable influence on the patients' quality of life. Regulation of the immune system is one of the important roles of the gut microbiota. The effect of dysbiosis considering some members of gut microbiota in patients with chronic urticaria has been demonstrated in our previous study. Comparing the frequency and bacterial load of Lactobacillus, Bifidobacterium, and Bacteroides between patients with chronic urticaria and healthy controls. 20 patients with chronic urticaria and 20 age and sex matched healthy individuals were included in the present study. Stool samples were analyzed for determining the frequency and bacterial load of Lactobacillus, Bifidobacterium, and Bacteroides genera. There were no significant differences among the frequencies of detectable Lactobacillus, Bifidobacterium, or Bacteroides in stool samples of patients with chronic urticaria and healthy controls. The relative amounts of Lactobacillus and Bifidobacterium were significantly higher in fecal samples from controls compared to patients with chronic urticaria (P = 0.038 and 0.039, respectively). It is the first study on the implication of Lactobacillus, Bifidobacterium, and Bacteroides genera as gut microbiota members in patients with chronic urticaria. Copyright © 2018 Elsevier B.V. All rights reserved.

Approaches to the diagnosis and management of patients with a history of nonsteroidal anti-inflammatory drug-related urticaria and angioedema.

PubMed

Kowalski, Marek L; Woessner, Katharine; Sanak, Marek

2015-08-01

Nonsteroidal anti-inflammatory drug (NSAID)-induced urticarial and angioedema reactions are among the most commonly encountered drug hypersensitivity reactions in clinical practice. Three major clinical phenotypes of NSAID-induced acute skin reactions manifesting with angioedema, urticaria, or both have been distinguished: NSAID-exacerbated cutaneous disease, nonsteroidal anti-inflammatory drug-induced urticaria/angioedema (NIUA), and single NSAID-induced urticaria and angioedema. In some patients clinical history alone might be sufficient to establish the diagnosis of a specific type of NSAID hypersensitivity, whereas in other cases oral provocation challenges are necessary to confirm the diagnosis. Moreover, classification of the type of cutaneous reaction is critical for proper management. For example, in patients with single NSAID-induced reactions, chemically nonrelated COX-1 inhibitors can be safely used. However, there is cross-reactivity between the NSAIDs in patients with NSAID-exacerbated cutaneous disease and NIUA, and thus only use of selective COX-2 inhibitors can replace the culprit drug if the chronic treatment is necessary, although aspirin desensitization will allow for chronic treatment with NSAIDs in some patients with NIUA. In this review we present a practical clinical approach to the patient with NSAID-induced urticaria and angioedema. Copyright © 2015 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.

[Acetylsalicylic acid and food additive intolerance in urticaria, bronchial asthma and rhinopathy].

PubMed

Wüthrich, B; Fabro, L

1981-09-26

Adverse reactions (urticaria, angio-edema, bronchoconstriction, purpura) to Aspirin (ASS) and food-and-drug additives such as the yellow dye tartrazine and the preservative benzoate are observed all over the world. Since the exact pathogenetic mechanisms of this condition is unknown, it is described as intolerance or pseudo-allergy and has been related to an imbalance of prostaglandin synthesis. Among 620 patients with urticaria, bronchial asthma or chronic rhinitis, oral provocation tests with ASS, tartrazine or benzoic acid revealed in 165 (26.6%) intolerance to ASS or additives. Frequency of intolerance to tartrazine varied between 6.1% in urticaria (n=308), 7.3% in asthma (n=96) and 14.5% in urticaria and asthma patients, while intolerance to benzoate varied from 2.5% in rhinitis (n=40) to 11.5% in asthma. More than two thirds of the intolerant patients were improved by an elimination diet and by the avoidance of "aspirin-like" drugs. More than one third of chronic urticaria patients became symptomfree. In Switzerland exact declaration of all food additives is urgently needed. Moreover, azo-dyes must no longer be used for colouring of drugs.

Chronic urticaria and irregular menstrual cycle: a case report of effective therapy with oral contraception.

PubMed

Kasperska-Zając, Alicja; Zamlynski, Jacek

2012-04-01

It has been reported that urticaria may be associated with some conditions characterized by sex hormone changes. The case presented concerns a 19-year-old patient who had severe symptoms of chronic urticaria for 3 years, concomitant with an irregular menstrual cycle and functional ovarian cysts. After a laparoscopic ovarian cystectomy the patient was treated with hormonal contraceptives. After 3 weeks the symptoms of urticaria withdrew. The patient continues to receive oral contraceptive treatment, has regular menstruation and shows no symptoms of urticaria. A diagnosis with an urticaria origin should take into consideration any changes in hormone balance as one of the possible reasons for the disease. In such cases, treatment with sex hormones may positively influence the course of the illness.

Hypersensitivity to lipid transfer protein is frequently associated with chronic urticaria.

PubMed

Asero, R

2011-02-01

Sparse clinical observations suggest a possible association between food allergy to lipid transfer protein (LTP) and chronic urticaria (CU). To investigate the possible association between LTP hypersensitivity and CU. History of CU, and/or of NSAID hypersensitivity was prospectively assessed in 75 consecutive LTP-allergic subjects (M/F 27/48; age 33.6 years); those with positive histories underwent an autologous serum skin test (ASST). 100 atopic subjects not sensitized to LTP and 100 subjects with chronic urticaria served as controls. 16/75 (21%) patients had a history of current or past CU. 7 (9%) had a history of NSAID-induced urticaria, and the ASST scored positive in 9/11 patients (82%). By comparison with atopic controls patients showed a significantly higher prevalence of CU (21% vs 6%; p < 0.01), a > 4 times more frequent history of NSAID hypersensitivity (9% vs 2%), and a higher prevalence of females (p< 0.05). In contrast, patients and controls with chronic urticaria showed a similar sex distribution, prevalence of positive ASST, and prevalence of NSAID hypersensitivity. An unidirectional association between LTP hypersensitivity and chronic urticaria seems to exist. The reasons for this are unclear although it is possible that CU makes mast cells more easily excitable by food allergens. Further, it has been shown that NSAIDs may up-regulate type 1 allergic responses to foods, possibly increasing permeability of the gut mucosa.

Open-label safety assessment of bilastine in elderly patients with allergic rhinoconjunctivitis and/or urticaria.

PubMed

Sologuren, Ander; Vinas, Rosa; Cordon, Esther; Riesgo, Susana E; Del Mar Fores, Maria; Senan, Maria Rosa; Fernandez, Sonia; Labeaga, Luis; Ruiz-Mijan, Manuel

2018-03-20

Bilastine is an H1-antihistamine approved for symptomatic treatment of patients with allergic rhinoconjunctivitis or urticaria. The safety profile of bilastine in clinical trials of allergic rhinoconjunctivitis or urticaria, assessed by type and frequency of adverse events (AE), was similar to that of placebo. As part of the risk management plan for bilastine, the safety profile of bilastine in the elderly was assessed. A prospective, multicenter, observational, open-label, 3-month follow-up study was performed to assess the safetyprofile of bilastine 20 mg in patients aged greater than or equal to 65 years with allergic rhinoconjunctivitis and/or urticaria. A total of 74 of 146 patients (50.7%) reported 129 treatment-emergent AEs (TEAE) during the study period. Theincidence of TEAEs was low, with monthly and quarterly rates of 0.29 (95% confidence intervals [CI], 0.229-0.367) and 0.88(95% CI, 0.688 -1.100), respectively. Monthly and quarterly incidence rates were 0.04 (95% CI, 0.016-0.082) and 0.12 (95%CI, 0.048-0.246), respectively, for related TEAEs (eight TEAEs in seven patients) and were 0.02 (95% CI, 0.003- 0.048) and0.05 (95% CI, 0.010-0.143), respectively, for serious TEAEs (five TEAES in three patients). All serious TEAEs were considered to be unrelated to bilastine. Bilastine 20 mg showed a favorable safety profile with a low incidence of TEAEs in patients aged greater than or equal to 65 years.The results were in accordance with the known safety profile of bilastine 20 mg and incidence of AEs reported in previous studies and described in the approved summary of product characteristics.

Improvement in the quality of life of patients with chronic spontaneous urticaria treated with omalizumab in real life.

PubMed

Larrea-Baca, Itzíar; Gurpegui-Resano, María

Evaluation of the impact on the quality of life (QOL) relating to health in patients with chronic urticaria (CSU) treated with omalizumab. Longitudinal descriptive observational study of quality of life based on18 patients with chronic urticaria ≥12 years treated with omalizumab. Changes in QOL examined at 1 month (T1), at 6 months (T6) and 12 months (T12), by: Visual Analogical Scale (UAS), specific urticaria QOL questionnaire (CU-Q2oL), general health questionnaire (SF-36) and activity of urticaria questionnaire (Score UAS7). The most disabling symptoms for patients were: pruritus (61.1%); decrease in health and physical activity (55.5%), wheals, swelling and nervousness (50%); depression and shame of marks (38.89%); side effects to drugs (33.33%); sleepiness and tiredness (27.78%); work (22.22%); "choosing clothes and pain" 16.67% and social relations (11.11%). Statistically significant differences were observed in T1, T6 and T12. In T1, UAS -4.72 (p=.001); CU-Q2oL -16.38 (p<.001); SF-36 15.44 (p=.006); UAS7 wheals -7.27 (p=.001), UAS7 pruritus -8.72 (p=.003). In T6, UAS -4.26 (p=.001); CU-Q2oL -18.94 (p<.001); SF-36 15.41 (p<.001); UAS7 wheals -7.41 (p=.001), UAS7 pruritus -7.11 (p=.001). In T12 UAS -9.61 (p=.004); CU-Q2oL -21.38 (p=.01); SF-36 17.53 (p=.04); UAS7 wheals -8.23 (p=.004), UAS7 pruritus -10.69 (p=.004). There was a very good response in T1, which was maintained in T6 and T12. Patients with chronic urticaria treated with omalizumab presented good results with a reduction of CUE activity and improvement of their QOL. Copyright © 2017 Elsevier España, S.L.U. All rights reserved.

[Diagnostic and Therapeutic Approach of Chronic Spontaneous Urticaria: Recommendations in Portugal].

PubMed

Costa, Célia; Gonçalo, Margarida

2016-11-01

Chronic spontaneous urticaria is a complex disorder, of unclear etiology, easily diagnosed although often difficult to treat. It has a significant impact on the patients' quality of life and results in high direct and indirect costs. The diagnosis of chronic spontaneous urticaria is mainly clinical and a limited number of tests is recommended for differential diagnosis and/or for the investigation/exclusion of possible causes. In addition to the complete blood count and C-reactive protein, and/or erythrocyte sedimentation rate, additional tests must be selected according to clinical criteria. The aim of therapy is the complete clinical control of chronic spontaneous urticaria. Evolution should be documented by weekly symptom scoring - Weekly Urticaria Activity Score (UAS7) -, as well as the assessment of quality of life. The therapeutic approach is based on second-generation H1 antihistamines (anti-H1) administered continuously in the approved doses (first line), and, in the absence of a clinical response, up to four times the daily-approved dose (second line). First generation H1 antihistamines are not recommended. Approximately 30% of patients are not controlled with second line therapy, and it is recommended to add a third line therapy. Of the two options, omalizumab and cyclosporine, only omalizumab is approved for chronic spontaneous urticaria and has a better safety profile, thus being preferably recommended. In Portugal there are no national-based recommendations applicable to clinical practice. The elaboration of these recommendations is justified by the need to standardize both the diagnosis and the treatment approach of patients with chronic spontaneous urticaria in Portugal, and for the referral of patients to specialized centers, in the most severe cases.

Comparison of monocyte gene expression among patients with neurocysticercosis-associated epilepsy, Idiopathic Epilepsy and idiopathic headaches in India.

PubMed

Prabhakaran, Vasudevan; Drevets, Douglas A; Ramajayam, Govindan; Manoj, Josephine J; Anderson, Michael P; Hanas, Jay S; Rajshekhar, Vedantam; Oommen, Anna; Carabin, Hélène

2017-06-01

Neurocysticercosis (NCC), a neglected tropical disease, inflicts substantial health and economic costs on people living in endemic areas such as India. Nevertheless, accurate diagnosis using brain imaging remains poorly accessible and too costly in endemic countries. The goal of this study was to test if blood monocyte gene expression could distinguish patients with NCC-associated epilepsy, from NCC-negative imaging lesion-free patients presenting with idiopathic epilepsy or idiopathic headaches. Patients aged 18 to 51 were recruited from the Department of Neurological Sciences, Christian Medical College and Hospital, Vellore, India, between January 2013 and October 2014. mRNA from CD14+ blood monocytes was isolated from 76 patients with NCC, 10 Recovered NCC (RNCC), 29 idiopathic epilepsy and 17 idiopathic headaches patients. A preliminary microarray analysis was performed on six NCC, six idiopathic epilepsy and four idiopathic headaches patients to identify genes differentially expressed in NCC-associated epilepsy compared with other groups. This analysis identified 1411 upregulated and 733 downregulated genes in patients with NCC compared to Idiopathic Epilepsy. Fifteen genes up-regulated in NCC patients compared with other groups were selected based on possible relevance to NCC, and analyzed by qPCR in all patients' samples. Differential gene expression among patients was assessed using linear regression models. qPCR analysis of 15 selected genes showed generally higher gene expression among NCC patients, followed by RNCC, idiopathic headaches and Idiopathic Epilepsy. Gene expression was also generally higher among NCC patients with single cyst granulomas, followed by mixed lesions and single calcifications. Expression of certain genes in blood monocytes can distinguish patients with NCC-related epilepsy from patients with active Idiopathic Epilepsy and idiopathic headaches. These findings are significant because they may lead to the development of new tools to

Chronic urticaria following acute hepatitis A.

PubMed

Griffin, Paul M; Kevat, Dev A S; McCarthy, James S; Woods, Marion L

2012-09-18

Urticaria has a documented association with the prodromal phases of hepatitis A, B and, although still contentious, likely hepatitis C. Despite the documented association there are few actual reported cases of urticaria occurring with hepatitis A infection and in all of the cases reported so far the urticaria preceded the diagnosis of hepatitis A and was acute rather than chronic. We describe a case of urticaria occurring following acute infection with hepatitis A, which persisted beyond 6 weeks and therefore was by definition chronic. Although chronic urticaria has been reported to be associated with other forms of viral hepatitis, to the best of our knowledge this has not been reported previously with hepatitis A.

Definition, aims, and implementation of GA(2) LEN Urticaria Centers of Reference and Excellence.

PubMed

Maurer, M; Metz, M; Bindslev-Jensen, C; Bousquet, J; Canonica, G W; Church, M K; Godse, K V; Grattan, C E; Hide, M; Kocatürk, E; Magerl, M; Makris, M; Meshkova, R; Saini, S S; Sussman, G; Toubi, E; Zhao, Z; Zuberbier, T; Gimenez-Arnau, A

2016-08-01

GA²LEN, the Global Allergy and Asthma European Network, has recently launched a program for the development, interaction, and accreditation of centers of reference and excellence in special areas of allergy embedded in its overall quality management of allergy centers of excellence. The first area chosen is urticaria. Urticaria is a common and debilitating condition and can be a challenge for both patients and treating physicians, especially when chronic. Centers of reference and excellence in urticaria (UCAREs) can help to improve the management of hard-to-treat conditions such as urticaria. Here, we describe the aims, the requirements and deliverables, the application process, and the audit and accreditation protocol for GA²LEN UCAREs. The main aims of GA²LEN UCAREs are to provide excellence in urticaria management, to increase the knowledge of urticaria by research and education, and to promote the awareness of urticaria by advocacy activities. To become a certified GA²LEN UCARE, urticaria centers have to apply and fulfill 32 requirements, defined by specific deliverables that are assessed during an audit visit. The GA²LEN UCARE program will result in a strong network of urticaria specialists, promote urticaria research, and harmonize and improve urticaria management globally. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Malassezia infection associated with chronic spontaneous urticaria without angioedema: a report on five cases.

PubMed

Zawar, Vijay; Pawar, Manoj; Kumavat, Shrikant

2018-06-01

Chronic spontaneous urticaria (CSU) is a challenging condition to treat and it significantly affects quality of life. Bacterial, viral, parasitic, and fungal infections have been associated with triggering and/or perpetuating urticaria in certain individuals. There is a paucity of literature on CSU associated with Malassezia infection. We present a case series of five patients with CSU without angioedema in whom we observed temporal association of Malassezia infection with CSU. The presence of Malassezia was confirmed by clinical examination, Wood's lamp, and KOH examination. The patients with CSU experienced improvement after specific antifungal therapy. Malassezia infection may be associated with recurrent and chronic urticaria in a certain group of susceptible patients and thus specific targeted therapy against it might result in complete remission of urticaria along with clearing of the infection.

EAACI/GA(2)LEN/EDF/WAO guideline: definition, classification and diagnosis of urticaria.

PubMed

Zuberbier, T; Asero, R; Bindslev-Jensen, C; Walter Canonica, G; Church, M K; Giménez-Arnau, A; Grattan, C E H; Kapp, A; Merk, H F; Rogala, B; Saini, S; Sánchez-Borges, M; Schmid-Grendelmeier, P; Schünemann, H; Staubach, P; Vena, G A; Wedi, B; Maurer, M

2009-10-01

This guideline, together with its sister guideline on the management of urticaria [Zuberbier T, Asero R, Bindslev-Jensen C, Canonica GW, Church MK, Giménez-Arnau AM et al. EAACI/GA(2)LEN/EDF/WAO Guideline: Management of urticaria. Allergy, 2009; 64:1427-1443] is the result of a consensus reached during a panel discussion at the 3rd International Consensus Meeting on Urticaria, Urticaria 2008, a joint initiative of the Dermatology Section of the European Academy of Allergology and Clinical Immunology (EAACI), the EU-funded network of excellence, the Global Allergy and Asthma European Network (GA(2)LEN), the European Dermatology Forum (EDF) and the World Allergy Organization (WAO). Urticaria is a frequent disease. The life-time prevalence for any subtype of urticaria is approximately 20%. Chronic spontaneous urticaria and other chronic forms of urticaria do not only cause a decrease in quality of life, but also affect performance at work and school and, as such, are members of the group of severe allergic diseases. This guideline covers the definition and classification of urticaria, taking into account the recent progress in identifying its causes, eliciting factors, and pathomechanisms. In addition, it outlines evidence-based diagnostic approaches for different subtypes of urticaria. The correct management of urticaria, which is of paramount importance for patients, is very complex and is consequently covered in a separate guideline developed during the same consensus meeting. This guideline was acknowledged and accepted by the European Union of Medical Specialists (UEMS).

Prevalence and Clinical Characteristics of Chronic Spontaneous Urticaria in Pediatric Patients.

PubMed

Balp, Maria-Magdalena; Weller, Karsten; Carboni, Veruska; Chirilov, Alexandra; Papavassilis, Charis; Severin, Thomas; Tian, Haijun; Zuberbier, Torsten; Maurer, Marcus

2018-04-21

Data on the prevalence and disease management of chronic urticaria (CU) and chronic spontaneous urticaria (CSU) in the pediatric population are scarce. The present study assessed the prevalence of CU and CSU, and disease management among pediatric patients (0-17 years). A physician-based online survey was conducted in 5 European countries (United Kingdom, Germany, Italy, France, and Spain) assessing the annual diagnosed prevalence, disease characteristics and treatment patterns in the target population. Results are based on physician responses and analyzed using descriptive statistics. Prevalence estimates were calculated based on the number of CU/CSU pediatric patients seen, treated and referred by the respondents and extrapolated to the total pediatric population from each country. Across 5 European countries, the one-year diagnosed prevalence of CU and CSU in pediatric patients was 1.38% (95% CI, 0.94-1.86) and 0.75% (95% CI, 0.44-1.08), respectively. Angioedema was reported in 6%-14% of patients. A large proportion of CSU pediatric patients (40%-60%) were treated with H1-antihistamines at approved dose and 16% to 51% received H1-antihistamines at higher doses. Approximately 1/3 of pediatric CSU patients remained uncontrolled with H1- antihistamines at approved/higher doses. Other prescribed treatments were oral corticosteroids (10% to 28%) and topical creams (15% to 26%). This study revealed a prevalence of CSU among pediatric population comparable to adults and also suggested an unmet need for approved treatments for inadequately-controlled pediatric CSU patients. It is truly of concern that harmful (oral steroids) or insufficient (topical creams) treatments were frequently used despite of better and guideline recommended alternatives. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

Bilastine: in allergic rhinitis and urticaria.

PubMed

Carter, Natalie J

2012-06-18

Bilastine is an orally administered, second-generation antihistamine used in the symptomatic treatment of seasonal or perennial allergic rhinoconjunctivitis and urticaria. In two well designed phase III trials, 14 days' treatment with bilastine was associated with a significantly lower area under the effect curve (AUEC) for the reflective total symptom score (TSS) than placebo in patients with symptomatic seasonal allergic rhinitis. Additionally, reflective nasal symptom scores were significantly lower in bilastine than placebo recipients in patients with a history of seasonal allergic rhinitis who were challenged with grass pollen allergen in a single-centre, phase II study. Neither bilastine nor cetirizine was effective in the treatment of perennial allergic rhinitis with regard to the mean AUEC for reflective TSS in another well designed phase III trial. However, results may have been altered by differences in some baseline characteristics and placebo responses between study countries. In another well designed phase III trial, compared with placebo, bilastine was associated with a significantly greater change from baseline to day 28 in the mean reflective daily urticaria symptom score in patients with chronic urticaria. There were no significant differences in primary endpoint results between bilastine and any of the active comparators used in these trials (i.e. cetirizine, levocetirizine and desloratadine). Bilastine was generally well tolerated, with a tolerability profile that was generally similar to that of the other second-generation antihistamines included in phase III clinical trials.

Contact urticaria from nickel and plastic additives (butylhydroxytoluene, oleylamide).

PubMed

Osmundsen, P E

1980-12-01

In two patients urticaria was elicited by contact with nickel-containing objects. Chamber-prick test with nickel sulfate 2.5% in pet. evoked a strong urticarial reaction in both patients. In one of the patients contact with plastic articles also provoked urticaria. A 20-min patch test with several articles of plastic (polyethylene and PVC) and with butylhydroxytoluene (BHT) 1% in ethanol elicited urticarial reactions. BHT is used as an antioxidant in plastic. Furthermore, open patch test with oleylamide (amide of oleic acid) 0.1% in ethanol elicited a strong urticarial reaction in 20 min. This chemical is used as a slipping agent in plastic.

Rupatadine in Established Treatment Schemes Improves Chronic Spontaneous Urticaria Symptoms and Patients' Quality of Life: a Prospective, Non-interventional Trial.

PubMed

Metz, Martin; Weller, Karsten; Neumeister, Claudia; Izquierdo, Iñaki; Bödeker, Rolf-Hasso; Schwantes, Ulrich; Maurer, Marcus

2015-12-01

Chronic spontaneous urticaria (CSU) is a common and hard to treat condition associated with a substantial negative impact on patients' quality of life (QoL). Clinical studies have shown that rupatadine is effective and safe in the treatment of CSU, but data from routine clinical care are scarce. Therefore, we assessed the effectiveness and tolerability of rupatadine in established dosages on CSU activity and patients' QoL in a routine daily practice setting. This was an open, prospective, non-interventional study performed in 146 dermatological practices in Germany. CSU patients for whom treatment with rupatadine was indicated were eligible to participate. Key symptoms of urticaria activity and their impact on patients' QoL were assessed at the beginning and the end of treatment. Adverse events (AEs) and withdrawals, as well as the dosage regimens chosen, were documented. Patients and physicians were requested to rate effectiveness and tolerability of therapy at the final visit. All statistical analyses were descriptive. The majority of the 660 patients screened to be treated (median age 44 years, IQR = 31-59 years, n = 654) received rupatadine 10 mg tablets once (477 patients) or twice (105 patients) daily for a median time of 28 days. After treatment, 93.2% of the patients (606/650) reported a clear overall improvement of symptoms. Rupatadine significantly reduced the urticaria activity score (UAS7) as well as the frequency and severity of existing angioedema episodes. Similarly all domains of the urticaria-specific QoL questionnaire (CU-Q 2 oL) were markedly improved. The majority of physicians and patients rated rupatadine treatment as effective and well tolerated. There were 39 (5.9%) early treatment withdrawals, and 21 patients (3.2%) experienced AEs. Rupatadine when given according to the routine treating schemes improves symptoms and CU-Q 2 oL of CSU patients; the drug is also safe and well tolerated. Dr. R. Pfleger GmbH.

[Pharmacotherapy of urticaria--an analysis of the discrepancies between guidelines of expert bodies, registration documents and evidence for the effectiveness of drugs].

PubMed

Plichta, Danuta; Spiewak, Radosław

2013-01-01

The year 2012 has seen relevant changes in Polish pharmaceutical legislation and drug reimbursement, among others limiting the reimbursement solely to indications stated in the Summaries of Product Characteristics (SPCs). A discrepancy with expert recommendations became apparent. The aim of this study was to analyze discordances between up-to-date expert recommendations, the SPCs in force, and the evidence for the effectiveness of recommended drugs in urticaria. Guidelines for the treatment of urticaria issued by Polish and international expert bodies were analyzed, along with the SPCs. A systematic review of clinical trials of recommended drugs was carried out. Of drugs recommended by the experts, 203 were authorized in Poland for urticaria treatment, including 167 oral preparations of second-generation antihistamines (SGAH, 8 active substances), 29 oral preparations of first-generation antihistamines (6 substances), 4 preparations of systemic glucocorticosteroids (2), 2 topical glucocorticosteroid preparations (2) and one combined preparation of human immunoglobulin with histamine. Among products both recommended by experts and licensed for the treatment of urticaria in Poland, high or moderate-level of evidence of effectiveness was available for 7 active substances (bilastine, cetirizine, desloratadine, fexofenadine, loratadine, levocetirizine, rupatadine). Nevertheless, 39% of SGAH available in Poland (66 preparations of cetirizine, emedastine, levocetirizine, loratadine or fexofenadine) were registered exclusively for "chronic idiopathic urticaria" - a diagnosis inconsistent with the current state of medical knowledge. We conclude that there exist considerable discrepancies between expert recommendations for the pharmacotherapy of urticaria, the licensed use of drugs as defined in Summaries of Product Characteristics and scientific evidence for their effectiveness.

Basophil activation test with food additives in chronic urticaria patients.

PubMed

Kang, Min-Gyu; Song, Woo-Jung; Park, Han-Ki; Lim, Kyung-Hwan; Kim, Su-Jung; Lee, Suh-Young; Kim, Sae-Hoon; Cho, Sang-Heon; Min, Kyung-Up; Chang, Yoon-Seok

2014-01-01

The role of food additives in chronic urticaria (CU) is still under investigation. In this study, we aimed to explore the association between food additives and CU by using the basophil activation test (BAT). The BAT using 15 common food additives was performed for 15 patients with CU who had a history of recurrent urticarial aggravation following intake of various foods without a definite food-specific IgE. Of the 15 patients studied, two (13.3%) showed positive BAT results for one of the tested food additives. One patient responded to monosodium glutamate, showing 18.7% of CD203c-positive basophils. Another patient showed a positive BAT result to sodium benzoate. Both patients had clinical correlations with the agents, which were partly determined by elimination diets. The present study suggested that at least a small proportion of patients with CU had symptoms associated with food additives. The results may suggest the potential utility of the BAT to identity the role of food additives in CU.

Comparison of the efficacy and safety of bilastine 20 mg vs levocetirizine 5 mg for the treatment of chronic idiopathic urticaria: a multi-centre, double-blind, randomized, placebo-controlled study.

PubMed

Zuberbier, T; Oanta, A; Bogacka, E; Medina, I; Wesel, F; Uhl, P; Antépara, I; Jáuregui, I; Valiente, R

2010-04-01

Bilastine is a novel nonsedative H(1)-receptor antagonist, which may be used for the symptomatic treatment of chronic idiopathic urticaria (CU). The aim of this study was to compare the clinical efficacy and safety of bilastine 20 mg vs levocetirizine 5 mg and placebo in CU patients with moderate-to-severe symptoms. Overall 525 male and female subjects aged 18-70 years were randomized to receive bilastine 20 mg, levocetirizine 5 mg or placebo, once daily for 28 days, in double-blind manner, in 46 centres across Europe and Argentina. Patients rated symptoms of pruritus, number of wheals, and maximum size of wheals (on predefined scales) as reflective (over past 12 h) symptoms twice daily, for assessment of change from baseline in the total symptoms scores (TSS) over 28 days as the primary efficacy measure. Changes in reflective and instantaneous symptoms scores, Dermatology Life Quality Index (DLQI), and CU-associated discomfort and sleep disturbance were assessed as secondary outcomes. Safety was assessed according to adverse events, laboratory tests and electrocardiograms. Bilastine reduced patients' mean reflective and instantaneous TSS from baseline to a significantly greater degree than placebo (P < 0.001); from day 2 onwards of treatment. The DLQI, general discomfort, and sleep disruption were also improved significantly in bilastine-treated patients as compared with placebo-treated patients (P < 0.001 for all parameters). Comparison with levocetirizine indicated both treatments to be equally efficacious as well as equally safe and well tolerated as compared with placebo. Bilastine 20 mg is a novel effective and safe treatment option for the management of CU.

[Food urticaria in children. Review of 51 cases].

PubMed

Guillet, M H; Guillet, G

1993-10-01

Food origin seems to have a special place in the etiology of infantile urticaria, as it forms 62% of a series of 51 observations of urticaria in children, greater than drug etiology (22%), physical urticaria (8%) and contact urticaria (8%). Distinction between true and false food allergy, which is important for prognosis and evolution, puts true food allergy (55%) in the lead before false (7%). A reason for this, the atopic concept, was seen in 67% of the food urticarias, corresponding apparently to true food allergy. If in the majority of these cases the pathway of sensitizations was unknown, in very rare observations the sensitization pathway was reported as an inhalation or even a sensitization in utero. Finally, infantile urticaria, more serious because of the vital risks that may be associated with it, deserves a special comment from the etiological viewpoint: observation of urticaria (and shock) to peach in an infant of 4 months, to wheat flour at 5 months and egg at 6 months are a demonstration that between 4 and 6 months intolerance of cows milk proteins is not the only etiology of infantile urticaria. For prognosis, early detection of true food allergy in children gives hope of better results than in adults, with higher chances of regression of the sensitization.

Elevated blood histamine levels and mast cell degranulation in solar urticaria.

PubMed Central

Hawk, J L; Eady, R A; Challoner, A V; Kobza-Black, A; Keahey, T M; Greaves, M W

1980-01-01

1 Ultraviolet radiation (UVR)-induced wealing was studied in four patients with solar urticaria, whose measured action spectra were within the range 300 to 700 nm. 2 Elevated histamine levels were found in blood draining wealed skin in all four patients. 3 Histological and electron microscopial studies of the irradiated skin showed evidence of mast cell degranulation. 4 These findings demonstrate an association between histamine release from mast cells and wealing in solar urticaria, and should encourage evaluation of drugs which suppress histamine release in this disorder. Images Figure 2 PMID:7356907

Basophil Activation Test with Food Additives in Chronic Urticaria Patients

PubMed Central

Kang, Min-Gyu; Song, Woo-Jung; Park, Han-Ki; Lim, Kyung-Hwan; Kim, Su-Jung; Lee, Suh-Young; Kim, Sae-Hoon; Cho, Sang-Heon; Min, Kyung-Up

2014-01-01

The role of food additives in chronic urticaria (CU) is still under investigation. In this study, we aimed to explore the association between food additives and CU by using the basophil activation test (BAT). The BAT using 15 common food additives was performed for 15 patients with CU who had a history of recurrent urticarial aggravation following intake of various foods without a definite food-specific IgE. Of the 15 patients studied, two (13.3%) showed positive BAT results for one of the tested food additives. One patient responded to monosodium glutamate, showing 18.7% of CD203c-positive basophils. Another patient showed a positive BAT result to sodium benzoate. Both patients had clinical correlations with the agents, which were partly determined by elimination diets. The present study suggested that at least a small proportion of patients with CU had symptoms associated with food additives. The results may suggest the potential utility of the BAT to identity the role of food additives in CU. PMID:24527415

[RECOMMENDATION FOR EVALUATION AND TREATMENT OF CHRONIC URTICARIA - THE ISRAELI ASSOCIATION FOR ALLERGY AND CLINICAL IMMUNOLOGY].

PubMed

Levin Agmon, Nancy; Kessel, Aharon; Maoz Segal, Ramit; Rottem, Menachem; Tal, Yuval; Confino-Cohen, Ronit; Tobi, Elias

2017-06-01

Chronic urticaria is a disease manifested by a pruritic rash lasting longer than 6 weeks that may severely affect quality of life and daily function. Chronic urticaria can be further divided into chronic spontaneous urticaria which appears without a trigger and chronic inducible urticaria which evolves following distinct physical triggers. These two clinical manifestations could coexist in the same patient. The pathogenesis of chronic urticaria is not fully elucidated, although it is considered an autoimmune disease in at least 50% patients that produce auto- IgG antibodies targeted against the high affinity Fc receptor and to a lesser extent against IgE itself. Auto-antibodies associated with different autoimmune diseases can be detected such as those directed at thyroid proteins. Urticaria tends to spontaneously resolve in 50% of patients within the first year while others will suffer from it for a much longer period of time. The treatment of chronic urticaria has dramatically progressed in the last decade, enabling reduction of systemic corticosteroid use which has been the cornerstone of treatment in the past. The recommended treatment for chronic urticaria is currently based on a stepwise approach that enables achieving disease control with a reasonably good quality of life. The first step of the treatment ladder consists of selective, new generation, anti-H1 histamine blockers, which do not cross the blood brain barrier, starting from the recommended dose (first line) and increasing up to four-fold (second line). The third line of treatment is the addition of immune modulators such as leukotriene receptor blockers (Singulair), anti-IgE biological therapy (Xolair), or cyclosporine. In this review we present the updates and considerations arising during evaluation and treatment of chronic urticaria. The need for specific tests, immunologist/allergologist evaluation, as well as treatment modalities taking into consideration the large body of evidence that has

Brain MRI findings in patients with idiopathic hypersomnia.

PubMed

Trotti, Lynn Marie; Bliwise, Donald L

2017-06-01

Proper diagnosis of idiopathic hypersomnia necessitates the exclusion of neurologic or medical causes of sleepiness that better explain the clinical syndrome. However, there are no formal guidelines regarding the use of neuroimaging to identify such secondary causes of symptoms. We sought to characterize brain MRI findings in a series of patients with idiopathic hypersomnia. We reviewed medical records on a consecutive series of 61 patients diagnosed with idiopathic hypersomnia to determine the frequency and results of brain magnetic resonance imaging (MRI). One-third of patients had undergone brain MRI, with focal neurologic signs or symptoms being the most common indication for neuroimaging. Although seven patients had an identifiable finding on neuroimaging (e.g., chronic microvascular ischemic changes), clinical management was changed as a result of imaging in only three cases. In all three, the imaging finding was predated by clear clinical abnormalities. Neuroimaging may be a complementary part of an idiopathic hypersomnia evaluation, but the decision to pursue imaging should be made on a case-by-case basis. Copyright © 2017 Elsevier B.V. All rights reserved.

Clinical features and natural history of acquired cold urticaria in a tertiary referral hospital: a 10-year prospective study.

PubMed

Katsarou-Katsari, A; Makris, M; Lagogianni, E; Gregoriou, S; Theoharides, T; Kalogeromitros, D

2008-12-01

Acquired cold urticaria (ACU) represents a heterogeneous group of disorders that share a common clinical feature: the development of urticaria or angioedema after cold exposure. We present epidemiological and clinical data of subjects with ACU, natural progression and we examine possible parameters that could correlate with disease severity. During a 10-year period in all subjects with ACU, detailed record of personal history, laboratory testing, cold stimulation testing (CST), atopy assessment and disease severity took place. In a re-evaluation visit at the end of the surveillance period, ACU progression was assessed from patients in a subjective way. Four thousand one hundred fifty-seven individuals with chronic urticaria were referred, and 352 (198 males, 154 females, 8.47% of patients with chronic urticaria) presented definite symptoms of physical urticarias, while 95 individuals (49 males, 46 females, 27% of patients with physical urticarias) were detected with ACU. Sixty-two participants were included in study analysis. Thirty-two patients (51.6%) were female; the mean age was 41.5 +/- 15.6 years, while the mean age at disease onset was 32.5 +/- 15.6 years; half were < or = 30 years old at disease onset. The mean duration of surveillance was 9.0 +/- 6.9 years. During this time interval, 18 patients (29.0%) showed the same or even worse symptomatology, 26 patients reported some improvement (41.9%), while in 18 patients, symptoms resolved completely (29.0%); the mean time to resolution was 5.6 +/- 3.5 years. Disease severity was the only variable statistically significantly related to disease progression (P = 0.004). Cold urticaria is a chronic persistent disorder with occasional severe clinical manifestations.

Regression of urticaria pigmentosa in adult patients with systemic mastocytosis: correlation with clinical patterns of disease.

PubMed

Brockow, Knut; Scott, Linda M; Worobec, Alexandra S; Kirshenbaum, Arnold; Akin, Cem; Huber, Mary M; Metcalfe, Dean D

2002-06-01

To determine clinical correlates of urticaria pigmentosa (UP) regression in adult patients with systemic mastocytosis (SM). Cohort study of the natural history of mastocytosis. National Institutes of Health Clinical Center. In a study of adult patients referred to the National Institutes of Health after 1980 and observed for a minimum of 10 years, 12 of 106 adult patients experienced clearance or fading of UP. Data from each patient's history and results of physical examination, laboratory evaluation, and organ biopsy at presentation to the National Institutes of Health were compared with findings at the patient's most recent visit. In the patients in whom clearance of (n = 5) or a decrease in skin lesions (n = 7) was noted, UP had persisted from 4 to 34 years (median, 17 years). Older age was a prognostic feature for regression of UP. Despite improvement of UP, the 2 patients with SM with an associated hematologic disorder experienced a deterioration in clinical condition. In the 10 patients with indolent SM, severity and frequency of symptoms decreased as the UP regressed. However, bone marrow changes consistent with SM remained. Urticaria pigmentosa regresses in approximately 10% of the older patients who have SM. In patients with an associated hematologic disorder such as myelodysplasia, this regression may be accompanied by disease progression. In contrast, regression of UP in patients with indolent SM parallels a decrease in disease intensity, although bone marrow findings of indolent SM remain.

Dermatoscopic findings of urticaria pigmentosa*

PubMed Central

Miller, Marcela Duarte Benez; Nery, Natália Solon; Gripp, Alexandre Carlos; Maceira, Juan Piñeiro; do Nascimento, Gisele Moro

2013-01-01

Mastocytosis is a rare disease characterized by proliferation and accumulation of mast cells in various organs. The maculopapular cutaneus mastocytosis is divided into three subtypes: papular/plaque variant, urticaria pigmentosa and eruptive macular telangiectasia perstans. Dermoscopic may help to better characterize the different forms of cutaneus mastocytosis. We report a 55 year-old female with urticaria pigmentosa and its dermoscopy. PMID:24474113

Impact of chronic urticaria on quality of life and work in Japan: Results of a real-world study.

PubMed

Itakura, Asako; Tani, Yumiko; Kaneko, Naoko; Hide, Michihiro

2018-06-13

Little attention has been given to the burden of chronic urticaria (CU) in Japan compared with other skin diseases, such as atopic dermatitis (AD) and psoriasis. The primary objective of the RELEASE study was to evaluate the real-life quality-of-life impairment in CU patients in Japan. Data were collected from 1443 urticaria, 1668 AD and 435 psoriatic patients; 552 urticaria patients who presented urticaria symptoms for over 6 weeks were defined as CU. The mean Dermatology Life Quality Index (DLQI) total score was 4.8, 6.1 and 4.8 in CU, AD and psoriatic patients, respectively. Disease control of urticaria evaluated by the Urticaria Control Test (UCT) and DLQI exhibited a strong correlation with a Spearman's rank correlation coefficient of -0.7158. CU and AD patients had relatively higher scores in all Work Productivity and Activity Impairment - General Health subscales except for absenteeism. At the time of the survey, approximately 64% of CU patients reported UCT scores of <12 and demonstrated higher work productivity loss and activity impairment versus patients with UCT scores of ≥12. Patients with lower UCT scores also displayed a higher percentage of dissatisfaction with their health state and the treatment they received. Approximately 85% of patients with CU had visited dermatology clinics, and less than 20% had visited hospital, indicating existence of a highly burdened population outside specialized centers. These results highlight the unmet medical needs of CU patients, suggesting the need to increase awareness of CU burden among both physicians and patients and to pursue improved real-life patient care. © 2018 Novartis K.K. The Journal of Dermatology published by John Wiley & Sons Australia, Ltd on behalf of Japanese Dermatological Association.

[Argentine guidelines for urticaria and angioedema].

PubMed

Máspero, Jorge; Cabrera, Hugo; Ardusso, Ledit; De Gennaro, Mónica; Fernández Bussy, Ramón; Galimany, José; Galimberti, Daniel; Label, Marcelo; La Forgia, Marta; Medina, Iris; Neffen, Hugo; Troielli, Patricia

2014-01-01

This interdisciplinary paper summarizes the news in the diagnosis and treatment of chronic urticaria (CU), and provides concepts, definitions and evidence-based suggestions for its management. Urticaria occurs in at least 20% of the population at some point in their lives. Acute urticaria (less than 6 weeks' duration), differs from CU in its etiology, but the onset of this disease is always acute. CU may occur as spontaneous (SCU) or induced (ICU). The diagnosis is simple, although a careful evaluation is necessary for differential diagnosis. ICU's diagnosis is mainly clinical, even if provocation tests can be useful. Supplementary studies should be limited and based on the clinical suspicion. Treatment may be divided into three approaches: avoidance, elimination or treatment of the cause, and pharmacological treatment. Recently treatment has been modified with the use of second-generation antihistamines as first-line and increased doses of nonsedating H1 antihistamines, up to 4 times, as second line. Antihistamines are essential to treat CU; however, 40% of patients do not achieve good control despite increased doses and require additional treatment. The most recent evidence indicates a group of drugs to be used as third line in these cases, to improve quality of life and to limit toxicity from frequent or chronic use of systemic steroids. Only 3 drugs are recommended as third line: omalizumab, cyclosporin A or anti-leukotrienes.

Immunological Mechanisms Implicated in the Pathogenesis of Chronic Urticaria and Hashimoto Thyroiditis.

PubMed

Berghi, Nicolae Ovidiu

2017-08-01

Autoimmunity represents the attack of the immune system of an organism against its own cells and tissues. Autoimmune diseases may affect one organ (Hashimoto thyroiditis) or can be systemic (chronic urticaria). Many factors are implicated in the pathogenesis of autoimmunity (white cells, cytokines, chemokines). Hashimoto thyroiditis has been associated with chronic urticaria in the last 3 decades in a number of clinical studies. Anti-thyroid antibodies have been documented in a proportion ranging from 10% to 30% in chronic urticaria patients in different countries from 3 continents. Two of the factors involved in the mechanism of autoimmunity are present both in the pathophysiology of Hashimoto thyroiditis and chronic urticaria. According to recent studies, IL6 is implicated in the pathogenesis of both diseases. TregsCD4+CD25+Foxp3+ cells have also been implicated in the pathological mechanisms of these 2 entities. This review offers an explanation of the clinical and statistical association between these two diseases from the pathophysiological point of view.

Omalizumab substantially improves dermatology-related quality of life in patients with chronic spontaneous urticaria.

PubMed

Finlay, A Y; Kaplan, A P; Beck, L A; Antonova, E N; Balp, M-M; Zazzali, J; Khalil, S; Maurer, M

2017-10-01

Chronic spontaneous/idiopathic urticaria (CSU/CIU) has substantial detrimental effects on health-related quality of life (HRQoL) with an effect comparable to or worse than many other skin diseases. To assess the effect of omalizumab on CSU patients' HRQoL, measured by the Dermatology Life Quality Index (DLQI) in three phase III studies ASTERIA I, ASTERIA II and GLACIAL. A post hoc analysis examined changes in DLQI scores, distribution of patients across DLQI bands and the proportion reaching minimal clinically important difference (MCID) following omalizumab vs. placebo. Omalizumab 300 mg significantly improved total DLQI scores vs. placebo, with a mean decrease from baseline to week 12 of -10.3 vs. -6.1 (P < 0.0001) in ASTERIA I, -10.2 vs. -6.1 (P = 0.0004) in ASTERIA II and -9.7 vs. -5.1 (P < 0.0001) in GLACIAL. A significant shift from high disease impact on life at baseline towards less impact at week 12 was seen with omalizumab 300 mg vs. placebo (P < 0.001; all studies). The proportion of patients where change in mean total DLQI score from baseline to week 12 reached an MCID of ≥4 was 74.1%, 76.0% and 77.2% in ASTERIA I, II and GLACIAL, respectively (P < 0.01; all studies). Maximum duration of omalizumab treatment was 24 weeks. This additional analysis assessed the impact of CSU and benefit of treatment with omalizumab by exploring different facets of DLQI data by treatment arm at multiple assessment points. The original aspects of analysis included applying the concept of the recently validated score for the MCID of the DLQI, changes in DLQI domain scores and in the distribution of subjects based on validated total DLQI score bands. It showed consistently that omalizumab provides significant and clinically relevant improvements in many aspects of HRQoL that are important to patients with CSU. These results contribute to a better understanding of the impact of CSU and its treatment on patients and can support clinical decision-making in routine medical

Selecting optimal second-generation antihistamines for allergic rhinitis and urticaria in Asia.

PubMed

Recto, Marysia Tiongco; Gabriel, Ma Teresita; Kulthanan, Kanokvalai; Tantilipikorn, Pongsakorn; Aw, Derrick Chen-Wee; Lee, Tak Hong; Chwen, Ch'ng Chin; Mutusamy, Somasundran; Hao, Nguyen Trong; Quang, Vo Thanh; Canonica, Giorgio Walter

2017-01-01

Allergic diseases are on the rise in many parts of the world, including the Asia-Pacific (APAC) region. Second-generation antihistamines are the first-line treatment option in the management of allergic rhinitis and urticaria. International guidelines describe the management of these conditions; however, clinicians perceive the additional need to tailor treatment according to patient profiles. This study serves as a consensus of experts from several countries in APAC (Hong Kong, Malaysia, the Philippines, Singapore, Thailand, Vietnam), which aims to describe the unmet needs, practical considerations, challenges, and key decision factors when determining optimal second-generation antihistamines for patients with allergic rhinitis and/or urticaria. Specialists from allergology, dermatology, and otorhinolaryngology were surveyed on practical considerations and key decision points when treating patients with allergic rhinitis and/or urticaria. Clinicians felt the need for additional tools for diagnosis of these diseases and a single drug with all preferred features of an antihistamine. Challenges in treatment include lack of clinician and patient awareness and compliance, financial constraints, and treatment for special patient populations such as those with concomitant disease. Selection of optimal second-generation antihistamines depends on many factors, particularly drug safety and efficacy, impact on psychomotor abilities, and sedation. Country-specific considerations include drug availability and cost-effectiveness. Survey results reveal bilastine as a preferred choice due to its high efficacy and safety, suitability for special patient populations, and the lack of sedative effects. Compliance to the international guidelines is present among allergists, dermatologists and otorhinolaryngologists; however, this is lower amongst general practitioners (GPs). To increase awareness, allergy education programs targeted at GPs and patients may be beneficial. Updates to

Immunoreactivity to food antigens in patients with chronic urticaria.

PubMed

Rašković, Sanvila; Matić, Ivana Z; Dorđić, Marija; Damjanović, Ana; Kolundžija, Branka; Grozdanić-Stanisavljević, Nađa; Besu, Irina; Jovičić, Zikica; Bijelić, Borivoj; Janković, Ljiljana; Juranić, Zorica

2014-01-01

The goal of study was better understanding of complex immune mechanisms that can help to evaluate patients with chronic urticaria (CU), especially those with unknown etiology. The study involved 55 patients with CU. Control group consisted of up to 90 healthy persons. The presence and intensity of serum IgG, IgA, IgM and IgE antibodies to common food antigens: cow's milk proteins (CMP), gliadin and phytohemagglutinin were determined by ELISA. Determination of subpopulations of immunocompetent cells was performed by flow cytometry. Significantly enhanced IgE, but also IgA immunity to CMP was found in patients with CU in comparison to healthy controls: (p < 0.000004) and (p < 0.002), respectively. Notably, in 40 out of 55 CU patients, the increased levels of some type of immunoglobulin reactivity to CMP were found. Regarding gliadin, only the levels of serum IgE anti-gliadin antibodies were significantly enhanced in patients with CU (p < 0.04). Significantly enhanced percentage of CD89+ cells accompanied with significantly lower percentage of lymphocytes and significantly higher mean fluorescence intensity of CD26 expression on lymphocytes were found in patients with CU in comparison to healthy controls (p < 0.04), (p < 0.02) and (p < 0.003), respectively. Results of this study may help in better understanding the complex immune disturbances in patients with CU.

Urticaria induced by laser epilation: a clinical and histopathological study with extended follow-up in 36 patients.

PubMed

Landa, Nerea; Corrons, Natalia; Zabalza, Iñaki; Azpiazu, Jose L

2012-07-01

Laser epilation is the most common dermatologic light-based procedure in the world. We describe a unique side effect of the procedure: a delayed persistent urticarial rash. We conducted a retrospective study involving 13,284 patients who received laser epilation at our clinics from January 2006 through March 2010 with 755 nm alexandrite laser (MiniGentleLase, Gentlelase, and GentleMax, Candela). Using patient clinical data and photos that were recorded on a standard side-effect report chart, we identified patients with suspected urticaria. Those patients were then followed for a period that ranged from 12 to 63 months. Only patients who could be diagnosed, treated, and followed by the dermatologist at our clinics were included in the study. Patients diagnosed or treated by other physicians or nurses and those without clinical photos or insufficient follow-up data were not included. We identified 36 patients who developed a severe, itchy, persistent hive rash on the treated area 6-72 hours after treatment. Eruption occurred most often on the legs (31 cases), followed by the groin (11 cases), axillae (eight cases), forearms (one case), and upper lip (one case). The eruption consisted of a hive rash with multiple pruritic perifollicular papules and confluent plaques on the treated area. Most patients required oral corticosteroids to control the symptoms. Lesions resolved in 7-30 days. The urticaria occurred mostly after the first treatment (26 cases), and was recurrent in subsequent treatments. Pretreating with oral corticosteroids prevented or limited the eruption. Thirty-three of the 36 patients reported a history of allergic rhinitis or some other allergy. Skin biopsies on four patients showed edema and a deep, dense dermal infiltrate consistent with lymphocytes mixed with eosinophils in a perivascular and occasionally perifollicular pattern in the mid and lower dermis. Persistent urticaria is a rare side effect of laser epilation. Rupture of the hair follicle by

Safety and efficacy of cetirizine versus cetirizine plus ranitidine in chronic urticaria: Double-blind randomized placebo-controlled study.

PubMed

Guevara-Gutierrez, Elizabeth; Bonilla-Lopez, Sonia; Hernández-Arana, Socorro; Tlacuilo-Parra, Alberto

2015-01-01

First-line treatment for chronic urticaria is H1 non-sedating antihistamines. When these fail, guidelines recommend combination with H2 antihistamines. We conducted a randomized, double-blind, placebo-controlled trial. Thirty-two patients with chronic urticaria were included. Group A (16 subjects) treated with cetirizine plus ranitidine and Group B (16 subjects) with cetirizine plus placebo, both for 30 days. Efficacy measures were Urticaria Activity Score (UAS), Chronic Urticaria Quality of Life Questionnaire (CU-Q2oL) and time of symptom remission, safety measures were clinical and laboratory effects. Complete remission was obtained in ten patients (62.5%) from Group A and seven patients (44%) from Group B (p = 0.28). The UAS in Group A was 1.53 ± 2.09 versus Group B 2.06 ± 1.34 (p = 0.20). The CU-Q2oL in Group A was 12.93 ± 19.20 versus Group B 12.68 ± 10.30 (p = 0.20). At the end of treatment, 13 patients (81%) from Group A and 14 patients (87.5%) from Group B had some type of adverse effect (p = 1.0). Combination of cetirizine with ranitidine was not more effective than cetirizine alone in chronic urticaria. Both treatments resulted equally safe; however, our main limitation is the small sample size.

[Ketotifen treatment of chronic urticaria. An open study of therapeutically difficult courses].

PubMed

Taube, K M; Wozniak, K D; Lässig, W

1985-12-01

21 patients suffering from chronic urticaria were treated with Ketotifen (2 X 1 mg/d) for 4 weeks. The suppression of weals and pruritus, the side effects in comparison with antihistamines, as well as the effect after finishing therapy have been studied. Ketotifen has a good suppressing effect on weals and pruritus. The general tolerance of the preparation is good, as well. Thus Ketotifen may be regarded as an additional possibility concerning treatment of chronic urticaria.

Investigations of magnesium, histamine and immunoglobulins dynamics in acute urticaria.

PubMed

Mureşan, D; Oană, A; Nicolae, I; Alecu, M; Moşescu, L; Benea, V; Flueraş, M

1990-01-01

In 42 urticaria patients, magnesium, histamine and IgE were dosed. Magnesium, IgE and histamine variations were followed in urticaria evolution, during acute phase and clinical remission. We noticed magnesium, histamine, IgE values variations depending on disease evolution and applied therapeutic scheme. Therefore: At disease starting point, histamine presented 3.5 times higher values than the normal ones. The value decreases following a curve which tends to reach normal values during clinical remission. At disease starting point, magnesium presented values under the inferior limit of the normal, 0.5 m mol/L respectively, as a mean. The value increases towards the normal limit during clinical remission. Immunoglobulins E follow a similar curve to histamine one, presenting 1,250 U/L values at the starting point, that, under medication, influence decrease between normal limits (800 U/L), during clinical remission. Analyzing the variations of biochemical parameters, the authors emphasize magnesium substitution treatment in urticaria.

Updosing of Nonsedating Anti-histamines in Recalcitrant Chronic Urticaria

PubMed Central

Godse, Kiran; Bhattar, Prachi; Patil, Sharmila; Nadkarni, Nitin; Gautam, Manjyot

2016-01-01

Chronic urticaria (CU) is a persistent, debiliating condition that causes severe impairment on the quality of life (QoL) of patient by interrupting work productivity. Current guidelines recommend second-generation (nonsedating) anti-histamines for the treatment for all forms of urticaria. In patients who do not respond adequately to conventional doses of anti-histamines, it is recommended to increase the dose to up to four times to obtain control. But there are only few controlled studies that have assessed the efficacy and safety of nonsedating anti-histamines. Though sedating histamines are frequently used as an add-on therapy in severe cases, they have a negative impact on QoL by compromising sleep and performance. The use of other suggested therapeutic options (omalizumab, cyclosporine A, montelukast and dapsone) is also limited by paucity of data on their efficacy and adverse effect profile. Second-generation anti-histamines which are relatively safer require more proven data to support their judicious use to improve disease in patients with CU. PMID:27293247

Urticaria following varicella vaccine associated with gelatin allergy.

PubMed

Singer, S; Johnson, C E; Mohr, R; Holowecky, C

1999-01-28

An uncommon reaction to varicella vaccine has been urticaria. Based on two reports of urticaria believed to be due to gelatin in recipients of measles-mumps-rubella vaccine, we suspected gelatin as the cause of generalized urticaria in two children after varicella vaccination. Intradermal testing with gelatin yielded a wheal and flare reaction in both children. We conclude that children known to be allergic to gelatin should not receive Oka/Merck varicella vaccine (VARIVAX).

Sense of coherence as a protective factor in chronic urticaria

PubMed Central

Miniszewska, Joanna; Pietrzak, Anna; Zalewska-Janowska, Anna

2017-01-01

Introduction Chronic urticaria (CU) seems to be perceived as a psychodermatological disorder. Different psychological factors play an important role in CU triggering and course. One of them is a sense of coherence (SOC), which is believed to be a protective factor against anxiety and depression. Aim To investigate quality of life (QoL) in CU patients and to compare selected psychological parameters (anxiety, depression and sense of coherence) between CU individuals and the control group. Material and methods The study comprised 46 female patients with chronic urticaria and 33 healthy females as a control group. The following methods were employed: Urticaria Activity Score (UAS), Hospital Anxiety and Depression Scale (HADS), Sense of Coherence Questionnaire (SOC-29) and Dermatology Life Quality Index (DLQI). Results The CU patients presented a significantly higher anxiety level in comparison to the control group (z = 4.488; p < 0.001). There were no statistically significant differences regarding depression intensity and SOC. In both groups anxiety and depression negatively correlated with global SOC and all its components. Disease severity positively correlated with QoL (ρ = 0.46, p < 0.01) and negatively with global SOC (ρ = –0.33, p < 0.05). Conclusions Bearing in mind higher prevalence of anxiety and depressive symptoms in our CU group, it would be useful to perform screening of these aspects in all CU patients and subsequently develop respective psychological interventions, based on enhancement of personal resources. PMID:28507497

[Chronic urticaria in childhood : Rational diagnostics and treatment].

PubMed

Ott, H

2017-07-01

Chronic urticaria (CU) is defined by episodes of urticaria with or without angioedema, which recur daily or nearly daily over more than 6 weeks. Sudden manifestations of CU with or without known causes are termed chronic spontaneous urticaria, which is differentiated from chronic inducible urticaria. The differential diagnoses of CU in childhood range from self-limiting dermatoses to severe systemic diseases. Further targeted steps are taken to detect potential trigger factors or underlying illnesses only if suspicion arises on anamnestic grounds and CU is best treated in accordance with international guidelines. First-line therapy consists of non-sedating H 1 -antihistamines at approved or even higher doses. If symptoms persist, additional treatment with omalizumab, cyclosporine or montelukast can be initiated after careful individual consideration.

Food allergy preceded by contact urticaria due to the same food: involvement of epicutaneous sensitization in food allergy.

PubMed

Inomata, Naoko; Nagashima, Mayumi; Hakuta, Amiko; Aihara, Michiko

2015-01-01

There have recently been reports suggesting that sensitization to food allergens may occur outside the intestinal tract, especially through the skin. To clarify the role of epicutaneous sensitization in food allergy, we investigated the clinical characteristics of adult patients with food allergies preceded by contact urticaria due to the same foods. We investigated clinical characteristics of 15 patients (20-51 years of age; 5 men and 10 women), who had food allergies preceded by contact urticaria. Fourteen patients were contact urticaria due to the causative foods during occupationally cooking, whereas 1 patient during face pack. In the occupational group, causative foods included rice, wheat, fruits, vegetables, fish, shrimp and cuttlefish; in the fresh cucumber paste case the cause was cucumber. In the 15 patients, the causative foods were fresh, not processed, and were tolerated by most (9/15, 60%) after heating. Regarding to symptoms after ingestion of the causative foods, the most frequently induced symptoms was oral symptoms (14/15, 93.3%), followed by urticaria (4/15, 26.7%), abdominal symptoms (3/15, 20%). The duration between the start of jobs or face pack, and the onset of contact urticaria was from 1 month to 19 years (mean, 8.7 years). The duration between the onset of contact urticaria and the onset of food allergy was from a few weeks to 6 years (mean, 11 months). One sushi cook experienced severe anaphylactic shock after ingestion of fish. In the occupational group, 13 of 15 patients (86.7%) had atopic dermatitis or hand eczema, indicating that the impaired skin barrier might be a risk for food allergies induced by epicutaneous sensitization. Epicutaneous sensitization of foods could induce food allergy under occupational cooking and skin-care treatment with foods in adults. Copyright © 2014 Japanese Society of Allergology. Production and hosting by Elsevier B.V. All rights reserved.

[Therapy of chronic urticaria and recurrent Quincke edema].

PubMed

Kündig, T M

2001-05-01

A rational and differential therapy of chronic urticaria and relapsing angioedema must be based on a precise classification of the disease. The general management as well as the specific therapy will have to consider the various trigger factors involved, the differences in the response rates to antiallergic drugs and the different prognoses of the urticaria subforms. Therefore this article will first briefly describe the most frequent subforms of urticaria and relapsing angioedema, and then discuss their management and treatment.

Cold urticaria: inhibition of cold-induced histamine release by doxantrazole.

PubMed

Bentley-Phillips, C B; Eady, R A; Greaves, M W

1978-10-01

Thirteen patients with cold urticaria were studied to assess the effect of the systemic drug doxantrazole, which has actions resembling disodium cromoglycate, on cold evoked histamine release. The patients, all of whom developed an immediate local whealing response after cooling of the forearm, demonstrated release of histamine into venous blood draining that forearm. Following doxantrazole treatment, significant suppression of histamine release occurred. In some but not all patients this was accompanied by diminution of urtication in response to cooling. A double-blind study was carried out in 3 subjects, all of whom showed diminished cold-stimulated histamine release after doxantrazole. Two of these showed clinical improvement. Doxantrazole had no effect on erythema due to intradermal histamine, but did suppress the erythematous reaction to intradermal injection of compound 48/80. Our results suggest that doxantrazole or related anti-allergic agents might be useful in the treatment of cold urticaria.

EAACI/GA(2)LEN/EDF/WAO guideline: management of urticaria.

PubMed

Zuberbier, T; Asero, R; Bindslev-Jensen, C; Walter Canonica, G; Church, M K; Giménez-Arnau, A M; Grattan, C E H; Kapp, A; Maurer, M; Merk, H F; Rogala, B; Saini, S; Sánchez-Borges, M; Schmid-Grendelmeier, P; Schünemann, H; Staubach, P; Vena, G A; Wedi, B

2009-10-01

This guideline, together with its sister guideline on the classification of urticaria (Zuberbier T, Asero R, Bindslev-Jensen C, Canonica GW, Church MK, Giménez-Arnau AM et al. EAACI/GA(2)LEN/EDF/WAO Guideline: definition, classification and diagnosis of urticaria. Allergy 2009;64: 1417-1426), is the result of a consensus reached during a panel discussion at the Third International Consensus Meeting on Urticaria, Urticaria 2008, a joint initiative of the Dermatology Section of the European Academy of Allergology and Clinical Immunology (EAACI), the EU-funded network of excellence, the Global Allergy and Asthma European Network (GA(2)LEN), the European Dermatology Forum (EDF) and the World Allergy Organization (WAO). As members of the panel, the authors had prepared their suggestions regarding management of urticaria before the meeting. The draft of the guideline took into account all available evidence in the literature (including Medline and Embase searches and hand searches of abstracts at international allergy congresses in 2004-2008) and was based on the existing consensus reports of the first and the second symposia in 2000 and 2004. These suggestions were then discussed in detail among the panel members and with the over 200 international specialists of the meeting to achieve a consensus using a simple voting system where appropriate. Urticaria has a profound impact on the quality of life and effective treatment is, therefore, required. The recommended first line treatment is new generation, nonsedating H(1)-antihistamines. If standard dosing is not effective, increasing the dosage up to four-fold is recommended. For patients who do not respond to a four-fold increase in dosage of nonsedating H(1)-antihistamines, it is recommended that second-line therapies should be added to the antihistamine treatment. In the choice of second-line treatment, both their costs and risk/benefit profiles are most important to consider. Corticosteroids are not recommended for

Chronic urticaria in a health maintenance organization of Buenos Aires, Argentina - new data that increase global knowledge of this disease.

PubMed

Parisi, Claudio Alberto; Ritchie, Carla; Petriz, Natalia; Torres, Christian Morelo; Gimenez-Arnau, Ana

2018-01-01

In spite of the frequency of chronic urticaria, there are no epidemiological studies on its prevalence in Argentina. The objective of this study was to define the prevalence and epidemiological characteristics of chronic urticaria patients in Buenos Aires. The population studied were the members of the Italian Hospital Medical Care Program, a prepaid health maintenance organization located in the urban areas around the Autonomous City of Buenos Aires, Argentina. All patients with diagnosis of chronic urticaria members of the Italian Hospital Medical Care Program, and with at least 12 months of follow up were included in the study. All medical records obtained between January 1st, 2012 and December 31, 2014 were analyzed. The prevalence ratio for chronic urticaria per 100,000 population with 95% CI for December 31, 2014 was calculated. The prevalence rate for the entire population and then discriminated for adults and pediatric patients (less than 18 years old at diagnosis) was assessed. 158,926 members were analyzed. A total of 463 cases of chronic urticaria were identified on prevalence date (68 in pediatrics, 395 in adults), yielding a crude point prevalence ratio of 0.29% (CI 95% 0.26-0.31%). The observed prevalence of chronic urticaria in the adult population was 0.34 % (95% CI 0.31-0.38%), while in pediatrics it was 0.15 % (95% CI 0.11-0.20%). the main weakness is that the results were obtained from an HMO and therefore the possibility of selection bias. chronic urticaria is a global condition. Its prevalence in Buenos Aires is comparable with other countries.

High-dose anti-histamine use and risk factors in children with urticaria

PubMed Central

Uysal, Pınar; Avcil, Sibelnur; Erge, Duygu

2016-01-01

Aim The drugs of choice in the treatment of urticaria in children are H1-antihistamines. The aim of the study was to evaluate children with urticaria and define risk factors for requirement of high-dose H1-antihistamines in children with urticaria. Material and Methods The medical data of children who were diagnosed as having urticaria admitted to our outpatient clinic between January 2014 and January 2016 were searched. The medical histories, concomitant atopic diseases, parental atopy histories, medications, treatment responses, blood eosinophil and basophil counts, and serum total IgE levels were recorded. In addition, the urticaria activity score for seven days, autoimmune antibody tests, and skin prick test results were evaluated in children with chronic urticaria. Results The numbers of the children with acute and chronic urticaria were 138 and 92, respectively. The age of the children with chronic urticaria was higher than that of those with acute urticaria (p<0.0001). There was no difference between the two groups in terms of blood eosinophil and basophil counts, and serum total IgE levels (p>0.05). There was a negative correlation between blood eosinophil count and the UAS7 score in children with chronic urticaria (r=−0.276, p=0.011). Chronic urticaria and requirement of high dose H1-antihistamines were significant in children aged ≥10 years (p<0.001, p=0.015). High UAS7 score (OR: 1.09; CI 95%: [1.03–1.15]) and basopenia (OR: 6.77; CI 95%: [2.01–22.75]) were associated with the requirement of high-dose H1-AH in children with chronic urticaria. Conclusion The requirement of high-dose H1-antihistamines was higher with children’s increasing age. Disease severity and basopenia were risk factors for the requirement of high-dose H1-antihistamines. PMID:28123332

High-dose anti-histamine use and risk factors in children with urticaria.

PubMed

Uysal, Pınar; Avcil, Sibelnur; Erge, Duygu

2016-12-01

The drugs of choice in the treatment of urticaria in children are H1-antihistamines. The aim of the study was to evaluate children with urticaria and define risk factors for requirement of high-dose H1-antihistamines in children with urticaria. The medical data of children who were diagnosed as having urticaria admitted to our outpatient clinic between January 2014 and January 2016 were searched. The medical histories, concomitant atopic diseases, parental atopy histories, medications, treatment responses, blood eosinophil and basophil counts, and serum total IgE levels were recorded. In addition, the urticaria activity score for seven days, autoimmune antibody tests, and skin prick test results were evaluated in children with chronic urticaria. The numbers of the children with acute and chronic urticaria were 138 and 92, respectively. The age of the children with chronic urticaria was higher than that of those with acute urticaria (p<0.0001). There was no difference between the two groups in terms of blood eosinophil and basophil counts, and serum total IgE levels (p>0.05). There was a negative correlation between blood eosinophil count and the UAS7 score in children with chronic urticaria (r=-0.276, p=0.011). Chronic urticaria and requirement of high dose H1-antihistamines were significant in children aged ≥10 years (p<0.001, p=0.015). High UAS7 score (OR: 1.09; CI 95%: [1.03-1.15]) and basopenia (OR: 6.77; CI 95%: [2.01-22.75]) were associated with the requirement of high-dose H1-AH in children with chronic urticaria. The requirement of high-dose H1-antihistamines was higher with children's increasing age. Disease severity and basopenia were risk factors for the requirement of high-dose H1-antihistamines.

[Clinical features of idiopathic restless legs syndrome in Japanese patients].

PubMed

Kume, Akito; Kume, Hideaki

2010-06-01

Little is known about the diagnosis and management of restless legs syndrome (RLS) in Japanese neurology clinics. To validate the diagnostic criteria of the International RLS Study Group (IRLSSG) and the treatment algorithm of the Mayo Clinic in a Japanese neurology clinic setting and to clarify the features of Japanese patients with idiopathic RLS. Patients with RLS symptoms were examined by a neurologist and the assessment included neurological examination, tests for periodic limb movements (PLM) and dopaminergic response, and the clinical diagnosis was made according to IRLSSG diagnostic criteria. Patients diagnosed with idiopathic RLS were treated with dopaminergic agents and the efficacy was evaluated. The study subjects were 151 Japanese patients who presented with RLS symptoms. Idiopathic RLS was diagnosed in 113 patients, secondary RLS in 16 and RLS mimics in 22. The cause of RLS mimics was either myelopathy, radiculopathy or neuropathy in 11 patients. The mean age of patients with idiopathic RLS was 50.1 (SD 20.0) years, 63% were woman, 97% had daily RLS, 31% had family history (40% of the early-onset subgroup), 86% reported unpleasant sensations in the lower legs, 43% had PLM in the daytime suggested immobilization test, 81% suffered from insomnia, 49% had limitations of work and activities, 71% reported impaired mood, 27% had consulted physicians about their symptoms, 4% had been diagnosed with RLS, 73% improved after dopaminergic treatments, and 33% experienced complete remission. The clinical features of Japanese patients with idiopathic RLS were identical to those reported in western countries, which suggests that IRLSSG diagnostic criteria and Mayo Clinic treatment algorism are valid in Japanese neurology clinics. Both patients and physicians were not fully aware of RLS in this country. Neurological examination was important in excluding RLS mimics and making a diagnosis of RLS.

Chronic urticaria in a health maintenance organization of Buenos Aires, Argentina - new data that increase global knowledge of this disease*

PubMed Central

Parisi, Claudio Alberto; Ritchie, Carla; Petriz, Natalia; Torres, Christian Morelo; Gimenez-Arnau, Ana

2018-01-01

Background In spite of the frequency of chronic urticaria, there are no epidemiological studies on its prevalence in Argentina. Objective The objective of this study was to define the prevalence and epidemiological characteristics of chronic urticaria patients in Buenos Aires. Methods The population studied were the members of the Italian Hospital Medical Care Program, a prepaid health maintenance organization located in the urban areas around the Autonomous City of Buenos Aires, Argentina. All patients with diagnosis of chronic urticaria members of the Italian Hospital Medical Care Program, and with at least 12 months of follow up were included in the study. All medical records obtained between January 1st, 2012 and December 31, 2014 were analyzed. The prevalence ratio for chronic urticaria per 100,000 population with 95% CI for December 31, 2014 was calculated. The prevalence rate for the entire population and then discriminated for adults and pediatric patients (less than 18 years old at diagnosis) was assessed. Results 158,926 members were analyzed. A total of 463 cases of chronic urticaria were identified on prevalence date (68 in pediatrics, 395 in adults), yielding a crude point prevalence ratio of 0.29% (CI 95% 0.26-0.31%). The observed prevalence of chronic urticaria in the adult population was 0.34 % (95% CI 0.31-0.38%), while in pediatrics it was 0.15 % (95% CI 0.11-0.20%). Study limitations the main weakness is that the results were obtained from an HMO and therefore the possibility of selection bias. Conclusions chronic urticaria is a global condition. Its prevalence in Buenos Aires is comparable with other countries. PMID:29641702

Cold urticaria. Dissociation of cold-evoked histamine release and urticara following cold challenge.

PubMed

Keahey, T M; Greaves, M W

1980-02-01

Nine patients with acquired cold urticaria were studied to assess the effects of beta-adrenergic agents, xanthines, and corticosteroids on cold-evoked histamine release from skin in vivo. The patients, in all of whom an immediate urticarial response developed after cooling of the forearm, demonstrated release of histamine into the venous blood draining that forearm. Following treatment with aminophylline and albuterol in combination or prednisone alone, suppression of histamine release occurred in all but one patient. In some patients, this was accompanied by a subjective diminution in pruritus or buring, but there was no significant improvement in the ensuing edema or erythema. In one patient, total suppression of histamine release was achieved without any effect on whealing and erythema in response to cold challenge. Our results suggest that histamine is not central to the pathogenesis of vascular changes in acquired cold urticaria.

Orthodontic treatment in patient with idiopathic root resorption: a case report.

PubMed

Rey, Diego; Smit, Rosana Martínez; Gamboa, Liliana

2015-01-01

Multiple idiopathic external root resorption is a rare pathological condition usually detected as an incidental radiographic finding. External root resorption of permanent teeth is a multifactorial process related to several local and systemic factors. If an etiological factor cannot be identified for root resorption, the term "idiopathic" is applied. This report presents a case of multiple idiopathic apical root resorption. The condition was found in a young female patient seeking orthodontic treatment due to malocclusion. This kind of resorption starts apically and progresses coronally, causing a gradual shortening and rounding of the remaining root. Patients with this condition are not the ideal candidates for orthodontic treatment; however, the aim of this report is to describe an unusual case of idiopathic root resorption involving the entire dentition, and to present the orthodontic treatment of this patient. It describes the progress and completion of orthodontic therapy with satisfactory end results.

Real-World Characteristics and Treatment Patterns in Patients with Urticaria Initiating Omalizumab in the United States.

PubMed

Ke, Xuehua; Kavati, Abhishek; Wertz, Debra; Huang, Qing; Wang, Liya; Willey, Vincent J; Stephenson, Judith J; Ortiz, Benjamin; Paknis, Brandee; Bernstein, Jonathan A; Beck, Lisa A

2017-12-19

Omalizumab is indicated for the management of chronic idiopathic urticaria (CIU) in patients aged 12 years or older with persistent hives that are not adequately controlled by H1 antihistamines. While its safety and efficacy in CIU patients have been evaluated in multiple clinical trials, real-world use of omalizaumab in CIU has not been well characterized. To assess demographics, clinical characteristics, and treatment patterns of CIU patients who initiated omalizumab to better understand the usage of this agent in CIU management in the real world. This retrospective cohort study used medical and pharmacy claims data in the United States from the HealthCore Integrated Database to identify patients with CIU newly treated with omalizumab (≥ 4 omalizumab claims within 6 months of the initial claim) between March 21, 2014, and October 31, 2015 (study intake period). The index date was defined as the date of the first claim for omalizumab during the study intake period. Demographic and clinical characteristics were described for patients treated with omalizumab, as were treatment patterns associated with omalizumab and concomitant medications associated with CIU treatment. Descriptive and inferential statistics were reported. The Kaplan-Meier method was used to examine omalizumab treatment patterns. This study included 298 omalizumab-treated patients (mean [SD] age of 43.5 [13.64] years; 70.8% female); approximately 84% were seen by an allergist/immunologist. All patients had ≥ 12 months of continuous enrolment and a subset of 138 patients had ≥ 18 months of follow-up. For patients with ≥ 12 months of post-index follow-up, 12.1% (n = 36), 28.5% (n = 85), and 32.9% (n = 98) discontinued omalizumab within the 6-month, 12-month, and the entire post-index periods (mean 530 days), respectively; the mean number of days patients were continuously treated with omalizumab was 443.1 (95% CI = 425.0-461.3); the probabilities of continuous treatment (95% CI) were 0.879 (0

Significance of Myositis Autoantibody in Patients with Idiopathic Interstitial Lung Disease

PubMed Central

Song, Ju Sun; Hwang, Jiwon; Cha, Hoon-Suk; Jeong, Byeong-Ho; Suh, Gee Young; Chung, Man Pyo

2015-01-01

Purpose Some patients with interstitial lung disease (ILD) related to connective tissue disease (CTD) have a delayed diagnosis of the underlying CTD when the ILD is categorized as idiopathic. In this study, we evaluated the frequency of myositis autoantibodies in patients diagnosed with idiopathic ILD and investigated the clinical significance stemming from the presence of the antibodies. Materials and Methods A total 32 patients diagnosed with idiopathic ILD were enrolled in this study. We analyzed a panel of 11 myositis autoantibody specificities in the patients using a line blot immunoassay. Then, we divided them into myositis autoantibody-positive and -negative groups and compared the clinical features and laboratory data between the two groups. Results Of the 32 idiopathic ILD patients, 12 patients had myositis autoantibodies encompassing 9 specificities, except for anti-Mi-2 and anti-PM-Scl 100 (12/32, 38%). Anti-synthetase autoantibodies including Jo-1, EJ, OJ, PL-7, and PL-12 were present in 7 patients (7/32, 22%). The group with myositis autoantibodies presented more frequently with the symptom of mechanic's hand and showed abnormal pulmonary function test results with low forced vital capacity, diffusing capacity for carbon monoxide, total lung capacity, and high lactate dehydrogenase values in blood when compared with the group without myositis antibodies. Conclusion We strongly suggest that patients undergo an evaluation of myositis autoantibodies, if they are diagnosed with idiopathic ILD in the presence of clinical characteristics including mechanic's hand, arthralgia, and autoantibodies which are insufficient to make a diagnosis of a specific CTD category. PMID:25837172

Significance of myositis autoantibody in patients with idiopathic interstitial lung disease.

PubMed

Song, Ju Sun; Hwang, Jiwon; Cha, Hoon-Suk; Jeong, Byeong-Ho; Suh, Gee Young; Chung, Man Pyo; Kang, Eun-Suk

2015-05-01

Some patients with interstitial lung disease (ILD) related to connective tissue disease (CTD) have a delayed diagnosis of the underlying CTD when the ILD is categorized as idiopathic. In this study, we evaluated the frequency of myositis autoantibodies in patients diagnosed with idiopathic ILD and investigated the clinical significance stemming from the presence of the antibodies. A total 32 patients diagnosed with idiopathic ILD were enrolled in this study. We analyzed a panel of 11 myositis autoantibody specificities in the patients using a line blot immunoassay. Then, we divided them into myositis autoantibody-positive and -negative groups and compared the clinical features and laboratory data between the two groups. Of the 32 idiopathic ILD patients, 12 patients had myositis autoantibodies encompassing 9 specificities, except for anti-Mi-2 and anti-PM-Scl 100 (12/32, 38%). Anti-synthetase autoantibodies including Jo-1, EJ, OJ, PL-7, and PL-12 were present in 7 patients (7/32, 22%). The group with myositis autoantibodies presented more frequently with the symptom of mechanic's hand and showed abnormal pulmonary function test results with low forced vital capacity, diffusing capacity for carbon monoxide, total lung capacity, and high lactate dehydrogenase values in blood when compared with the group without myositis antibodies. We strongly suggest that patients undergo an evaluation of myositis autoantibodies, if they are diagnosed with idiopathic ILD in the presence of clinical characteristics including mechanic's hand, arthralgia, and autoantibodies which are insufficient to make a diagnosis of a specific CTD category.

Treatment of allergic rhinitis and urticaria: a review of the newest antihistamine drug bilastine.

PubMed

Wang, Xue Yan; Lim-Jurado, Margaret; Prepageran, Narayanan; Tantilipikorn, Pongsakorn; Wang, De Yun

2016-01-01

Allergic rhinitis and urticaria are common allergic diseases that may have a major negative impact on patients' quality of life. Bilastine, a novel new-generation antihistamine that is highly selective for the H1 histamine receptor, has a rapid onset and prolonged duration of action. This agent does not interact with the cytochrome P450 system and does not undergo significant metabolism in humans, suggesting that it has very low potential for drug-drug interactions, and does not require dose adjustment in renal impairment. As bilastine is not metabolized and is excreted largely unchanged, hepatic impairment is not expected to increase systemic exposure above the drug's safety margin. Bilastine has demonstrated similar efficacy to cetirizine and desloratadine in patients with seasonal allergic rhinitis and, in a Vienna Chamber study, a potentially longer duration of action than fexofenadine in patients with asymptomatic seasonal allergic rhinitis. It has also shown significant efficacy (similar to that of cetirizine) and safety in the long-term treatment of perennial allergic rhinitis. Bilastine showed similar efficacy to levocetirizine in patients with chronic spontaneous urticaria and can be safely used at doses of up to fourfold higher than standard dosage (80 mg once daily). The fourfold higher than standard dose is specified as an acceptable second-line treatment option for urticaria in international guidelines. Bilastine is generally well tolerated, both at standard and at supratherapeutic doses, appears to have less sedative potential than other second-generation antihistamines, and has no cardiotoxicity. Based on its pharmacokinetic properties, efficacy, and tolerability profile, bilastine will be valuable in the management of allergic rhinitis and urticaria.

Cholinergic urticaria with anaphylaxis: hazardous duty of a deployed US marine.

PubMed

Handfield, Kent S; Dolan, Christopher K; Kaplan, Michael

2015-04-01

Cholinergic urticaria (CU) is triggered by a rise in body temperature and can be complicated by bronchial hyperresponsiveness and anaphylaxis. It primarily affects young adults who actively engage in strenuous exercise, such as servicemen and servicewomen. If the patient reports a history of wheezing or difficulty breathing with urticaria, a water challenge test in a warm bath can be performed to confirm the presence of anaphylaxis. The test should be conducted in an environment in which the patient's airway can be secured and epinephrine can be administered if necessary. Nonsedating antihistamines commonly are used to treat CU, but few other treatments have been thoroughly evaluated for cases that are refractory to antihistamines. We present the case of a 27-year-old US Marine with CU and anaphylaxis confirmed by a water challenge test in a warm bath.

Idiopathic anaphylaxis.

PubMed

Fenny, Nana; Grammer, Leslie C

2015-05-01

Idiopathic anaphylaxis is a diagnosis of exclusion after other causes have been thoroughly evaluated and excluded. The pathogenesis of idiopathic anaphylaxis remains uncertain, although increased numbers of activated lymphocytes and circulating histamine-releasing factors have been implicated. Signs and symptoms of patients diagnosed with idiopathic anaphylaxis are indistinguishable from the manifestations of other forms of anaphylaxis. Treatment regimens are implemented based on the frequency and severity of patient symptoms and generally include the use of epinephrine autoinjectors, antihistamines, and steroids. The prognosis of idiopathic anaphylaxis is generally favorable with well-established treatment regimens and effective patient education. Copyright © 2015 Elsevier Inc. All rights reserved.

Electronic protocol of respiratory physical therapy in patients with idiopathic adolescent scoliosis.

PubMed

Cano, Danila Vieira Baldini; Malafaia, Osvaldo; Alves, Vera Lúcia dos Santos; Avanzi, Osmar; Pinto, José Simão de Paula

2011-01-01

To create a clinical database of respiratory function in patients with adolescent idiopathic scoliosis; computerize and store this clinical data through the use of a software; incorporate this electronic protocol to the SINPE© (Integrated Electronic Protocols System) and analyze a pilot project with interpretation of results. From the literature review a computerized data bank of clinical data of postural deviations was set up (master protocol). Upon completion of the master protocol a specific protocol of respiratory function in patients with adolescent idiopathic scoliosis was designed and a pilot project was conducted to collect and analyze data from ten patients. It was possible to create the master protocol of postural deviations and the specific protocol of respiratory function in patients with adolescent idiopathic scoliosis. The data collected in the pilot project was processed by the SINPE ANALYZER©, generating charts and statistics. The establishment of the clinical database of adolescent idiopathic scoliosis was possible. Computerization and storage of clinical data using the software were viable. The electronic protocol of adolescent idiopathic scoliosis could be incorporated into the SINPE© and its use in the pilot project was successful.

Ulcerative colitis masked by giant urticaria.

PubMed

Caroselli, C; Plocco, M; Pratticò, F; Bruno, C; Antonaglia, C; Rota, F; Curreli, I; Caroselli, A; Bruno, G

2007-01-01

The occurrence of giant urticaria and ulcerative colitis is very infrequent. A 23 year-old female reported the initial eruption of short-lived cutaneous itchy weals on her arms. Then lesions ran together and became confluent, extending to her legs, followed by undefined abdominal pain and a slight increase of body temperature. Exams showed hystologically confirmed ulcerative colitis, with perinuclear anti-neutrophil cytoplasmic antibody positivity. Ulcerative colitis therapy led not only to the remission of the colitic symptoms, but also to the prompt recovery of skin manifestations. Urticaria was the epiphenomenon of ulcerative colitis.

Treatment of allergic rhinitis and urticaria: a review of the newest antihistamine drug bilastine

PubMed Central

Wang, Xue Yan; Lim-Jurado, Margaret; Prepageran, Narayanan; Tantilipikorn, Pongsakorn; Wang, De Yun

2016-01-01

Allergic rhinitis and urticaria are common allergic diseases that may have a major negative impact on patients’ quality of life. Bilastine, a novel new-generation antihistamine that is highly selective for the H1 histamine receptor, has a rapid onset and prolonged duration of action. This agent does not interact with the cytochrome P450 system and does not undergo significant metabolism in humans, suggesting that it has very low potential for drug–drug interactions, and does not require dose adjustment in renal impairment. As bilastine is not metabolized and is excreted largely unchanged, hepatic impairment is not expected to increase systemic exposure above the drug’s safety margin. Bilastine has demonstrated similar efficacy to cetirizine and desloratadine in patients with seasonal allergic rhinitis and, in a Vienna Chamber study, a potentially longer duration of action than fexofenadine in patients with asymptomatic seasonal allergic rhinitis. It has also shown significant efficacy (similar to that of cetirizine) and safety in the long-term treatment of perennial allergic rhinitis. Bilastine showed similar efficacy to levocetirizine in patients with chronic spontaneous urticaria and can be safely used at doses of up to fourfold higher than standard dosage (80 mg once daily). The fourfold higher than standard dose is specified as an acceptable second-line treatment option for urticaria in international guidelines. Bilastine is generally well tolerated, both at standard and at supratherapeutic doses, appears to have less sedative potential than other second-generation antihistamines, and has no cardiotoxicity. Based on its pharmacokinetic properties, efficacy, and tolerability profile, bilastine will be valuable in the management of allergic rhinitis and urticaria. PMID:27110120

Idiopathic Ophthalmodynia and Idiopathic Rhinalgia: A Prospective Series of 16 New Cases.

PubMed

Pareja, Juan A; Montojo, Teresa; Guerrero, Ángel L; Álvarez, Mónica; Porta-Etessam, Jesús; Cuadrado, María L

2015-01-01

Idiopathic ophthalmodynia and idiopathic rhinalgia were described a few years ago. These conditions seem specific pain syndromes with a distinctive location in the eye or in the nose. We aimed to present a new prospective series in order to verify the consistency of these syndromes. We performed a descriptive study of all patients referred to our regional neurologic clinics from 2010 to 2014 because of facial pain exclusively felt in the eye or in the nose fulfilling the proposed diagnostic criteria for idiopathic ophthalmodynia and idiopathic rhinalgia. There were 9 patients with idiopathic ophthalmodynia and 7 patients with idiopathic rhinalgia, with a clear female preponderance, and a mean age at onset in the fifth decade. The pain was usually moderate and the temporal pattern was generally chronic. Only one patient reported accompaniments (hypersensitivity to the light and to the flow of air in the symptomatic eye). Preventive treatment with amitriptyline, pregabalin, or gabapentin was partially or totally effective. The clinical features of this new series parallels those of the original description, thus indicating that both idiopathic ophthalmodynia and idiopathic rhinalgia have clear-cut clinical pictures with excellent consistency both inter- and intra-individually. © 2015 American Headache Society.

Idiopathic ophthalmodynia and idiopathic rhinalgia: two topographic facial pain syndromes.

PubMed

Pareja, Juan A; Cuadrado, María L; Porta-Etessam, Jesús; Fernández-de-las-Peñas, César; Gili, Pablo; Caminero, Ana B; Cebrián, José L

2010-09-01

To describe 2 topographic facial pain conditions with the pain clearly localized in the eye (idiopathic ophthalmodynia) or in the nose (idiopathic rhinalgia), and to propose their distinction from persistent idiopathic facial pain. Persistent idiopathic facial pain, burning mouth syndrome, atypical odontalgia, and facial arthromyalgia are idiopathic facial pain syndromes that have been separated according to topographical criteria. Still, some other facial pain syndromes might have been veiled under the broad term of persistent idiopathic facial pain. Through a 10-year period we have studied all patients referred to our neurological clinic because of facial pain of unknown etiology that might deviate from all well-characterized facial pain syndromes. In a group of patients we have identified 2 consistent clinical pictures with pain precisely located either in the eye (n=11) or in the nose (n=7). Clinical features resembled those of other localized idiopathic facial syndromes, the key differences relying on the topographic distribution of the pain. Both idiopathic ophthalmodynia and idiopathic rhinalgia seem specific pain syndromes with a distinctive location, and may deserve a nosologic status just as other focal pain syndromes of the face. Whether all such focal syndromes are topographic variants of persistent idiopathic facial pain or independent disorders remains a controversial issue.

Delayed pressure urticaria: response to treatment with sulfasalazine in a case series of seventeen patients.

PubMed

Swerlick, Robert A; Puar, Neha

2015-01-01

We retrospectively identified 17 patients with delayed pressure urticaria (DPU), diagnosed by history and confirmed with provocative pressure testing. The average age in the cohort was 42.6 years with 10 women and seven men. The mean duration of disease before diagnosis was 19.7 months (range, 1-60 months). The diagnosis of DPU was not included in the differential diagnosis of referring physicians and was not a diagnostic consideration in any of seven biopsies obtained. None of the patients responded adequately to treatment with antihistamines, but all 17 responded transiently when treated with either oral or intramuscular steroids. Eleven patients experienced complete or near complete resolution of DPU with treatment with sulfasalazine (SZ). Four patients had a partial response while two were unable to continue therapy because of drug intolerance. SZ appears to be a low cost and effective treatment for DPU. © 2015 Wiley Periodicals, Inc.

Seroprevalence of Toxocariasis in Children with Urticaria: A Population-based Study.

PubMed

Matos Fialho, Paula Mayara; Correa, Carlos Roberto Silveira; Lescano, Susana Zevallos

2017-10-01

This study described the prevalence of IgG class antibodies against Toxocara spp. and their association with urticaria in 2- to 12-year-old children. This population-based cross-sectional study was conducted between May 2012 and September 2014. The study sample comprised 168 children. Blood samples were collected to verify the presence of toxocariasis by using ELISA to detect IgG antibodies. The guardians of the children were interviewed to characterize the presence or absence of other diseases, such as urticaria. The presence of urticaria was observed in 38% of participants. The seroprevalence of toxocariasis in this population was 16%. This study confirmed a positive association between urticaria and positive serology for Toxocara and a negative independent association with canine contact and the number of household residents. There are no previous reports in the literature of a population-based study that correlates the presence of urticaria with serology for toxocariasis. © The Author [2017]. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com

Risk of Idiopathic Dilated Cardiomyopathy in 29 000 Patients With Celiac Disease

PubMed Central

Emilsson, Louise; Andersson, Bert; Elfström, Peter; Green, Peter H.R.; Ludvigsson, Jonas F.

2012-01-01

Background Dilated cardiomyopathy (DCM) is a rare disease of largely unknown origin. Previous studies have suggested an increased prevalence of celiac disease (CD) in patients with DCM. These studies, however, were based on a maximum of 5 patients with both CD and DCM. In the present large Swedish population-based cohort study, we examined the risk of idiopathic DCM in patients with CD determined by small-intestinal histopathology. Methods and Results From 2006 to 2008, we collected duodenal/jejunal biopsy data on CD (equal to villous atrophy, Marsh stage 3, n=29 071 unique individuals) from (all) 28 pathology departments in Sweden. These individuals were compared with 144 429 reference individuals matched for age, sex, calendar year, and county. Data on DCM were obtained through the National Patient Register and confirmed by patient charts and echocardiography data. During follow-up, 17 patients with CD and 52 reference individuals developed idiopathic DCM. Thus, patients with CD were at an increased risk of idiopathic DCM (hazard ratio, 1.73; 95% confidence interval, 1.00 to 3.00), although the risk estimate failed to attain statistical significance (P=0.052). Conclusion This nationwide study found a moderately but not statistically significantly increased risk of idiopathic DCM in patients with biopsy-verified CD. (J Am Heart Assoc. 2012;1:e001594 doi: 10.1161/JAHA.112.001594.) PMID:23130142

Genetics Home Reference: vibratory urticaria

MedlinePlus

... exposing the skin to vibration, repetitive stretching, or friction results in allergy symptoms such as hives (urticaria), ... fragile connection can be more easily broken; vibration, friction, or stretching of the skin can disrupt the ...

Night-time sedating H1 -antihistamine increases daytime somnolence but not treatment efficacy in chronic spontaneous urticaria: a randomized controlled trial.

PubMed

Staevska, M; Gugutkova, M; Lazarova, C; Kralimarkova, T; Dimitrov, V; Zuberbier, T; Church, M K; Popov, T A

2014-07-01

Many physicians believe that the most effective way to treat chronic urticaria is to take a nonsedating second-generation H1 -antihistamine in the morning and a sedating first-generation H1 -antihistamine, usually hydroxyzine, at night to enhance sleep. But is this belief well founded? To test this belief by comparing the effectiveness and prevalence of unwanted sedative effects when treating patients with chronic spontaneous urticaria (CSU) with levocetirizine 15 mg daily plus hydroxyzine 50 mg at night (levocetirizine plus hydroxyzine) vs. levocetirizine 20 mg daily (levocetirizine monotherapy). In this randomized, double-blind, cross-over study, 24 patients with difficult-to-treat CSU took levocetirizine plus hydroxyzine or levocetirizine monotherapy for periods of 5 days each. At the end of each treatment period, assessments were made of quality of life (Chronic Urticaria Quality of Life Questionnaire, CU-Q2 oL), severity of urticaria symptoms (Urticaria Activity Score, UAS), sleep disturbance during the night and daytime somnolence. Both treatments significantly decreased UAS, night-time sleep disturbances and CU-Q2 oL scores (P < 0·001) without significant differences between the two. Compared with baseline, daytime somnolence was significantly reduced by levocetirizine monotherapy (P = 0·006) but not by levocetirizine plus hydroxyzine (P = 0·218). Direct comparison of the two treatment modalities in terms of daytime somnolence favoured levocetirizine monotherapy (P = 0·026). The widespread belief that sleep is aided by the addition of a sedating first-generation H1 -antihistamine, usually hydroxyzine, at night is not supported. These results are in line with the urticaria guidelines, which state that first-line treatment for urticaria should be new-generation, nonsedating H1 -antihistamines only. © 2014 The Authors. British Association of Dermatology published by John Wiley & Sons Ltd on behalf of British Association of Dermatologists.

Night-time sedating H1-antihistamine increases daytime somnolence but not treatment efficacy in chronic spontaneous urticaria: a randomized controlled trial

PubMed Central

Staevska, M; Gugutkova, M; Lazarova, C; Kralimarkova, T; Dimitrov, V; Zuberbier, T; Church, MK; Popov, TA

2014-01-01

Background Many physicians believe that the most effective way to treat chronic urticaria is to take a nonsedating second-generation H1-antihistamine in the morning and a sedating first-generation H1-antihistamine, usually hydroxyzine, at night to enhance sleep. But is this belief well founded? Objectives To test this belief by comparing the effectiveness and prevalence of unwanted sedative effects when treating patients with chronic spontaneous urticaria (CSU) with levocetirizine 15 mg daily plus hydroxyzine 50 mg at night (levocetirizine plus hydroxyzine) vs. levocetirizine 20 mg daily (levocetirizine monotherapy). Methods In this randomized, double-blind, cross-over study, 24 patients with difficult-to-treat CSU took levocetirizine plus hydroxyzine or levocetirizine monotherapy for periods of 5 days each. At the end of each treatment period, assessments were made of quality of life (Chronic Urticaria Quality of Life Questionnaire, CU-Q2oL), severity of urticaria symptoms (Urticaria Activity Score, UAS), sleep disturbance during the night and daytime somnolence. Results Both treatments significantly decreased UAS, night-time sleep disturbances and CU-Q2oL scores (P < 0·001) without significant differences between the two. Compared with baseline, daytime somnolence was significantly reduced by levocetirizine monotherapy (P = 0·006) but not by levocetirizine plus hydroxyzine (P = 0·218). Direct comparison of the two treatment modalities in terms of daytime somnolence favoured levocetirizine monotherapy (P = 0·026). Conclusions The widespread belief that sleep is aided by the addition of a sedating first-generation H1-antihistamine, usually hydroxyzine, at night is not supported. These results are in line with the urticaria guidelines, which state that first-line treatment for urticaria should be new-generation, nonsedating H1-antihistamines only. PMID:24472058

Contact urticaria from carboxymethylcellulose in white chalk.

PubMed

Moreau, Linda; Alomer, Ghanima; Dubé, Normand; Sasseville, Denis

2006-03-01

Carboxymethylcellulose (CMC) is widely used in consumer goods, foods, and medicaments as a binder, emulsifier, and viscosity enhancer. Cases of immediate and delayed allergic reactions to this anionic cellulose polymer have been reported. To report a case of contact urticaria from CMC in chalk, with possible cross-reaction to methyl hydroxyethylcellulose (MHEC). Patch tests with readings at 48 and 96 hours were performed with the North American Contact Dermatitis Group standard series and benzisothiazolinone. Open and prick tests with readings after 30 minutes were performed with two brands of chalk as well as with various petrolatum and aqueous dilutions of CMC, MHEC, oleic acid, and calcium carbonate. The patient developed strong urticarial reactions during open tests with both powdered chalks and had milder reactions to the open test with CMC 10% aqueous (aq) and to prick testing with CMC 0.1% aq. No reaction to MHEC or any of the other ingredients of the chalks was observed. No relevant delayed reaction was noted. CMC can cause contact urticaria. It remains unclear why our patient reacted more strongly to the chalk than to CMC itself. We speculate that the abrasive nature of the chalk enhances the cutaneous penetration of CMC or that calcium carbonate, the main ingredient of the chalk, acts as an adjuvant. It is also possible that CMC and MHEC cross-react and that our negative results with MHEC may be due to improper testing technique or concentrations.

Evaluation of autologous serum skin test and skin prick test reactivity to house dust mite in patients with chronic spontaneous urticaria.

PubMed

Song, Zhiqiang; Zhai, Zhifang; Zhong, Hua; Zhou, Ziyuan; Chen, WenChieh; Hao, Fei

2013-01-01

Chronic spontaneous urticaria (CSU) is a common skin disorder with etiology that is not well understood. In this study, we evaluated the prevalence of autologous serum skin test (ASST) and skin prick testing (SPT) to house dust mite (HDM) in 862 CSU cases in China. Clinical features, courses and treatment responses were also recorded. The prevalence of positive ASST was 46.3%, and patients aged 30-39 years had the highest positive rate (52.1%). Positive SPT to HDM was seen in 153 patients (17.7%) with the highest positive rate (34.2%) in patients aged 20 or less. Patients with positive ASST had higher urticaria activity scores (UAS) (4.18±0.65 vs. 3.67±0.53) but lower positive rates of HDM (24.6% vs. 37.6%), as compared with those with negative ASST (odds ratio (OR) 1.84, 95% CI 1.38-2.47). Patients could be categorized into four groups based on the results of ASST and SPT to HDM and patients with positive ASST and positive SPT to HDM had the highest disease activity scores, experienced higher frequencies of angioedema, diseases duration, and required higher dosage of loratadine every month, compared with other subgroups (P<0.0001). Patients with CSU showed varied responses of positive ASST and varied sensitivity to HDM, Patients with positive ASST and/or positive SPT had more disease activity compared with patients with negative ASST and/or negative SPT. Further classification can be made based on the result of SPT and ASST.

Evaluation of Autologous Serum Skin Test and Skin Prick Test Reactivity to House Dust Mite in Patients with Chronic Spontaneous Urticaria

PubMed Central

Song, Zhiqiang; Zhai, Zhifang; Zhong, Hua; Zhou, Ziyuan; Chen, WenChieh; Hao, Fei

2013-01-01

Background Chronic spontaneous urticaria (CSU) is a common skin disorder with etiology that is not well understood. Methods In this study, we evaluated the prevalence of autologous serum skin test (ASST) and skin prick testing (SPT) to house dust mite (HDM) in 862 CSU cases in China. Clinical features, courses and treatment responses were also recorded. Results The prevalence of positive ASST was 46.3%, and patients aged 30–39 years had the highest positive rate (52.1%). Positive SPT to HDM was seen in 153 patients (17.7%) with the highest positive rate (34.2%) in patients aged 20 or less. Patients with positive ASST had higher urticaria activity scores (UAS) (4.18±0.65 vs. 3.67±0.53) but lower positive rates of HDM (24.6% vs. 37.6%), as compared with those with negative ASST (odds ratio (OR) 1.84, 95% CI 1.38–2.47). Patients could be categorized into four groups based on the results of ASST and SPT to HDM and patients with positive ASST and positive SPT to HDM had the highest disease activity scores, experienced higher frequencies of angioedema, diseases duration, and required higher dosage of loratadine every month, compared with other subgroups (P<0.0001). Conclusions Patients with CSU showed varied responses of positive ASST and varied sensitivity to HDM, Patients with positive ASST and/or positive SPT had more disease activity compared with patients with negative ASST and/or negative SPT. Further classification can be made based on the result of SPT and ASST. PMID:23741306

[Preliminary approach to the mental component in dermatologic patients].

PubMed

Di Prima, T M; De Pasquale, R; Gilotta, S M; Cravotta, A

1989-04-01

The aim of this study was to approach on a psychical point of view 27 patients suffering from chronic idiopathic urticaria (14), pruritus sine materia (6), alopecia areata (3), pathomimia (4), in order to examine the possibility that psychic disorders could act as triggering or aggravating the dermatological affection. Psychical assessment was evaluated by colloquy and by the administration of some psychodiagnostic tests: EPI, MMPI, Zung. The role of psychogenic factors in skin diseases is emphasized and the results obtained from the use of antidepressant and minor tranquilizer drugs are discussed.

[Antihistamines for the treatment of urticaria in Mexico].

PubMed

Larenas-Linnemann, Désirée; Sánchez-Borges, Mario; Del Río-Navarro, Blanca Estela; Alonzo-Romero Pareyón, María De Lourdes; Maldonado-García, César Alfonso; Mendoza-López, Enrique; Ortega-Martell, José Antonio; Sienra-Monge, Juan José Luis; Medina-Ávalos, Miguel Alejandro; Rojo-Gutiérrez, María Isabel; Beirana-Palencia, Angélica María; Vargas-Correa, Jorge Bernardo; Báez-Loyola, Carlos; Ramírez-Segura, Ruth Ivonne Mireya; Guzmán-Perea, María Graciela

2015-01-01

There are four types of histamine receptors. Allergic symptoms, especially those in rhinoconjunctivitis and urticaria, are mainly caused by activation of histamine receptor 1 (H1). Consequently, oral H1-antihistamines form and integral part of the treatment of these diseases. Antihistamines are inverse agonists that stabilize the non-active configuration of the histamine receptor. First generation H1-antihistamines cause a variety of adverse effects via several mechanisms: sedation (accumulation in the central nervous system), dry mouth, urinary retention, weight gain (low selectivity: stimulation of serotonin/muscarinic/alpha-adrenergic receptors) and drug interactions (substrate of CYP450-3A4). Generally second generation H1-antihistamines have a better safety profile. New guidelines on allergic rhinitis and urticaria recommend second generation H1-antihistamines as first line drugs, with -if necessary- four-times updosing to obtain control in urticaria. The enhanced efficacy of quadruple doses in urticaria, while maintaining a good safety profile, has been shown for bilastine, desloratadine and levocetirizine (rupatadine). For ebastine and fexofenadine only the safety of quadruple doses has been shown till now. Extreme precaution should be taken with astemizol and terfenadine that never should be up-dosed, as high serum concentrations can cause potentially fatal ventricular tachycardia. First generation antihistamines are not recommended as first line treatment and updosing is not safe.

Idiopathic portal hypertension regarding thiopurine treatment in patients with inflammatory bowel disease.

PubMed

Suárez Ferrer, Cristina; Llop Herrera, Elba; Calvo Moya, Marta; Vera Mendoza, María Isabel; González Partida, Irene; González Lama, Yago; Matallana Royo, Virginia; Calleja Panero, José Luis; Abreu García, Luis

2016-02-01

The possibility of developing idiopathic portal hypertension has been described with thiopurine treatment despite compromises the prognosis of these patients, the fact its true prevalence is unknown. A cross-sectional study was conducted in a cohort of inflammatory bowel disease (IBD) patients followed at our unit, to determine the prevalence of diagnosis of idiopathic portal hypertension (IPH) and its relationship with thiopurine treatment. At the time of the analysis, 927/1,419 patients were under treatment with thiopurine drugs (65%). A total of 4 patients with IBD type Crohn's disease with idiopathic portal hypertension probably related to the thiopurine treatment were identified (incidence of 4.3 cases per 1,000). Seventy-five percent of patients started with signs or symptoms of portal hypertension. Only one patient was asymptomatic but the diagnosis of IPH because of isolated thrombocytopenia is suspected. However, note that all patients had thrombocytopenia previously. Abdominal ultrasound with fibroscan, hepatic vein catheterization and liver biopsy were performed on all of them as part of the etiology of portal hypertension. In the abdominal ultrasound, indirect portal hypertension data were observed in all patients (as splenomegaly) cirrhosis was also ruled out. The fibroscan data showed significant liver fibrosis (F2-F3). Idiopathic portal hypertension following thiopurine treatment in IBD patients is a rare occurrence, but it must be borne in mind in the differential diagnosis for early diagnosis, especially in patients undergoing thiopurine treatment over a long period. The presence of thrombocytopenia is often the only predictor of its development in the preclinical stage.

The definition, diagnostic testing, and management of chronic inducible urticarias - The EAACI/GA(2) LEN/EDF/UNEV consensus recommendations 2016 update and revision.

PubMed

Magerl, M; Altrichter, S; Borzova, E; Giménez-Arnau, A; Grattan, C E H; Lawlor, F; Mathelier-Fusade, P; Meshkova, R Y; Zuberbier, T; Metz, M; Maurer, M

2016-06-01

These recommendations for the definition, diagnosis and management of chronic inducible urticaria (CIndU) extend, revise and update our previous consensus report on physical urticarias and cholinergic urticaria (Allergy, 2009). The aim of these recommendations is to improve the diagnosis and management of patients with CIndU. Our recommendations acknowledge the latest changes in our understanding of CIndU, and the available therapeutic options, as well as the development of novel diagnostic tools. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Contact urticaria, allergic contact dermatitis, and photoallergic contact dermatitis from oxybenzone.

PubMed

Landers, Maeran; Law, Sandra; Storrs, Frances J

2003-03-01

There is little literature regarding conventional patch tests and photopatch tests to oxybenzone resulting in both immediate- and delayed-type hypersensitivity reactions. A patient was patch-tested and photopatch-tested to various sunscreen chemicals. Both immediate- and delayed-type hypersensitivity reactions were observed with oxybenzone. The positive patch tests were also photoaccentuated. Oxybenzone, a common sunscreen allergen, can result in both contact urticaria and delayed-type hypersensitivity on both conventional patch testing and photopatch testing. Allergic contact dermatitis to sunscreen chemicals has traditionally included contact urticaria, allergic contact dermatitis, and photoallergic contact dermatitis. Due to the recognition of p-aminobenzoic acid (PABA) and its esters as sensitizers, the presence of benzophenones in "PABA-free" sunscreens has become more prevalent, especially in sunscreens with a sun protection factor (SPF) greater than 8. In our patient, immediate- and delayed-type hypersensitivity reactions were seen to oxybenzone (2-hydroxy-4-methoxybenzophenone, 2-benzoyl-5-methoxyphenol, benzophenone-3, Eusolex 4360, Escalol 567, EUSORB 228, Spectra-Sorb UV-9, Uvinul M-40) upon conventional patch testing and photopatch testing.

Vocal cord paralysis: What matters between idiopathic and non-idiopathic cases?

PubMed

Özbal Koç, Ayça Eltaf; Türkoğlu, Seda Babakurban; Erol, Ozan; Erbek, Selim

2016-01-01

This study aims to evaluate the demographic and clinical characteristics of patients with idiopathic and non-idiopathic vocal cord paralysis (VCP). This retrospective cohort was performed on data extracted from medical files of 92 consecutive patients (43 males, 49 females; median age 52.1±23.1 years; min. 1 - max. 87) with VCP diagnosed in the otorhinolaryngology department between April 2012 and December 2015. Diagnoses associated with VCP, side of involvement (right, left or bilateral) and previous medical histories were noted and compared between patients with idiopathic and non-idiopathic VCP. Vocal cord paralysis occurred on the left side (n=56, 60.9%), right side (n=28, 30.4%) or bilaterally (n=8, 8.7%). A clinical entity related with VCP was identified in 63 patients (68.5%), while 29 (31.5%) patients had idiopathic VCP. Most common etiologies for VCP were thyroid surgery (n=32, 34.8%), cardiovascular surgery (n=9, 9.8%), lung cancer (n=6, 6.5%) and cardiac anomalies (n=4, 4.3%), respectively. Patients with idiopathic VCP were significantly older (p<0.001), while gender distribution (p=0.121) and side of involvement (p=0.340) did not differ between two groups. Vocal cord paralysis is a relatively common clinical entity with substantial rate of morbidity. Identification of the underlying etiology and awareness on the clinical characteristics are keystones for foreseeing complications and determining the appropriate therapeutic modality.

Pediatric Central Diabetes Insipidus: Brain Malformations Are Common and Few Patients Have Idiopathic Disease.

PubMed

Werny, David; Elfers, Clinton; Perez, Francisco A; Pihoker, Catherine; Roth, Christian L

2015-08-01

Pediatric cohorts of central diabetes insipidus (CDI) have shown varying prevalences for the different causes of CDI, including idiopathic. The objective of the study was to determine the causes of CDI at a pediatric tertiary care center and to characterize their clinical outcomes. All patients with CDI at Seattle Children's Hospital were identified and retrospectively analyzed. From 2000 to 2013, 147 patients with CDI were encountered (mean age 7 y at diagnosis, mean follow-up 6.2 y). The different causes of CDI were grouped, and age of diagnosis, anterior pituitary hormone deficiencies (APHDs), and presence of the posterior pituitary bright spot (PPBS) were analyzed. Patients with idiopathic CDI had infundibular thickening measured using a systematic method. Brain malformations caused 24% of CDI cases, and 12.2% were idiopathic. Four of 22 patients with initially idiopathic CDI were diagnosed with an underlying condition, none occurring later than 2.5 years from diagnosis. APHDs were as common in the brain malformation group as they were in the tumor/infiltrative group (72% vs 85%; P = .09). The PPBS was present in at least 13% of patients and in 19% of those with brain malformations. Patients with idiopathic CDI and stalk thickening on the initial magnetic resonance imaging were more likely to have an underlying diagnosis (40% vs 0%; P = .03). Brain malformations were a more common cause of pediatric CDI than previously reported. These patients have a high rate of APHDs, and many have persistence of the PPBS. Idiopathic CDI is an uncommon diagnosis, and none of our patients were diagnosed with Langerhans cell histiocytosis or germinoma for more than 3 years from CDI diagnosis. Providers can consider less frequent magnetic resonance imaging after this time point. A systematic method of infundibular measurement on the initial magnetic resonance imaging may predict an underlying germinoma or Langerhans cell histiocytosis.

Urticaria due to Urtica dioica in a neonate.

PubMed

Uslu, Sinan; Bulbul, Ali; Diler, Betul; Bas, Evrim Kiray; Nuhoglu, Asiye

2011-03-01

Urticaria is one of the most common dermatoses during the childhood, but it is very rare in the neonates. A 17-day-old infant with a generalized urticaria was admitted to our pediatric emergency unit. The mother of the infant reported having applied water boiled with stinging nettle (Urtica dioica) onto her nipples twice a day (before and after each breastfeeding) for 2 days in order to heal her nipple cracks. Serum total immunoglobulin E (IgE) and specific IgE levels for stinging nettle were high in the infant and the mother. The rashes began to regress within the first day of the hospitalization and disappeared completely on the second day without treatment. The skin prick test with the water boiled with stinging nettle was positive for the infant with significant induration, but not for the mother. Conclusion Reporting the first urticaria case in newborns due to stinging nettle, the authors suggest that breastfeeding mothers should always consult a physician before using skincare products.

The Clinical Characteristics of Patients with Chronic Idiopathic Anal Pain

PubMed Central

Mao, Weiming; Liao, Xiujun; Wu, Wenjing; Yu, Yanyan; Yang, Guangen

2017-01-01

Abstract The aim of this study was to investigate the clinical characteristics, treatment outcomes and psychological distress in patients with chronic idiopathic anal pain. The study was conducted on patients referred to Hangzhou Third Hospital for chronic anal pain from January, 2010 to December, 2014. Patient demographics, clinical history, anorectal physiology, and radiological imaging data were recorded for all patients. The treatment outcome was noted for patients treated and followed up for more than 6 month at the present unit. Ninety-six patients with mean age of 45.1 years (range, 17-82) were studied. Seventy-one patients (74.0%) had functional anorectal pain(FARP). The main complaints were dull, sharp, stabbing, or spasm pain. Among all patients, 34.3% reported that their pain radiated into other locations. Fifty-one patients (53.1%) had bowel dysfunction, while 28.1% patients had urinary dysfunction. The common factors associated with pain relief were day time, lying down and warm water baths; the factors that contributed to aggravated pain were night time, defecation or sitting. 92.7% (89/96) of patients reported symptoms of psychological disturbance. FARP patients exhibited increased depression than non-FARP patients(P<0.05). In addition, female patients were more likely to have depression than male patients (P<0.05). The overall pain treatment success rate was 55.2% (53/96). The pain treatment outcome was better in non-FARP patients than in FARP patients(χ2=3.85, P<0.05). Conclusively, chronic idiopathic anal pain is a complex clinical symptom, involving pelvic floor muscles, the nervous system, endocrine system, and the patients’ psychological conditions. Further research is needed to improve diagnosis and treatment for patients with chronic idiopathic anal pain. PMID:28730167

Psychologic management of brace therapy for patients with idiopathic scoliosis.

PubMed

Matsunaga, Shunji; Hayashi, Kyoji; Naruo, Tetsuro; Nozoe, Shin-ichi; Komiya, Setsuro

2005-03-01

A trial of brace therapy modified by a measured personality pattern of patients with idiopathic scoliosis was performed. To evaluate the effectiveness of performing personality tests for patients with idiopathic scoliosis who undergo brace therapy. Brace therapy has often been used for the treatment of scoliosis. However, emotional distress can result from this therapy. Few attempts have been made to reduce such stress. A test using the Maudsley Personality Inventory was performed on 145 adolescent females with idiopathic scoliosis, treated with brace therapy alone, before the start of brace therapy and 1 month after the start of brace therapy. On the basis of test results, the patients were rated as normal type and four abnormal types. Brace therapy was continued considering the personality pattern of patients. For all patients, changes in psychologic test results, compliance with braces wearing instructions, and correction of scoliosis were analyzed. Of the 134 patients rated as normal before the start of therapy, 108 patients were rated as abnormal pattern when tested 1 month after the start of therapy. After performing autogenic training for patients with E-N+ and E-N- personalities, and giving advice to school teachers to decrease the emotional stress for patients with E+N+ personality, 47 patients were finally rated as abnormal pattern. In total, 12 (8%) of the 145 patients dropped out. In dropouts, the average pretreatment deformity of 29 degrees (range: 21 degrees -37 degrees ) had increased to an average of 37 degrees (range, 31 degrees -48 degrees ). Psychologic tests may be useful and provide a means of modifying brace therapy tailored to the psychologic conditions of individual patients.

A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis.

PubMed

King, Talmadge E; Bradford, Williamson Z; Castro-Bernardini, Socorro; Fagan, Elizabeth A; Glaspole, Ian; Glassberg, Marilyn K; Gorina, Eduard; Hopkins, Peter M; Kardatzke, David; Lancaster, Lisa; Lederer, David J; Nathan, Steven D; Pereira, Carlos A; Sahn, Steven A; Sussman, Robert; Swigris, Jeffrey J; Noble, Paul W

2014-05-29

In two of three phase 3 trials, pirfenidone, an oral antifibrotic therapy, reduced disease progression, as measured by the decline in forced vital capacity (FVC) or vital capacity, in patients with idiopathic pulmonary fibrosis; in the third trial, this end point was not achieved. We sought to confirm the beneficial effect of pirfenidone on disease progression in such patients. In this phase 3 study, we randomly assigned 555 patients with idiopathic pulmonary fibrosis to receive either oral pirfenidone (2403 mg per day) or placebo for 52 weeks. The primary end point was the change in FVC or death at week 52. Secondary end points were the 6-minute walk distance, progression-free survival, dyspnea, and death from any cause or from idiopathic pulmonary fibrosis. In the pirfenidone group, as compared with the placebo group, there was a relative reduction of 47.9% in the proportion of patients who had an absolute decline of 10 percentage points or more in the percentage of the predicted FVC or who died; there was also a relative increase of 132.5% in the proportion of patients with no decline in FVC (P<0.001). Pirfenidone reduced the decline in the 6-minute walk distance (P=0.04) and improved progression-free survival (P<0.001). There was no significant between-group difference in dyspnea scores (P=0.16) or in rates of death from any cause (P=0.10) or from idiopathic pulmonary fibrosis (P=0.23). However, in a prespecified pooled analysis incorporating results from two previous phase 3 trials, the between-group difference favoring pirfenidone was significant for death from any cause (P=0.01) and from idiopathic pulmonary fibrosis (P=0.006). Gastrointestinal and skin-related adverse events were more common in the pirfenidone group than in the placebo group but rarely led to treatment discontinuation. Pirfenidone, as compared with placebo, reduced disease progression, as reflected by lung function, exercise tolerance, and progression-free survival, in patients with

A geriatric patient with diffuse idiopathic skeletal hyperostosis

PubMed Central

Karadag, Berrin; Cat, Huseyin; Aksoy, Selma; Ozulu, Banu; Ozturk, Ali Osman; Oguz, Sukru; Altuntas, Yuksel

2010-01-01

The most frequent health problems seen in senility are chronic and degenerative diseases. A 75-year-old male patient with the complaints of weight loss and difficulty in swallowing was admitted to our hospital from a nursing home. Upper system fiber-optic gastrointestinal endoscopy was performed and a mass at the junction of the hypopharynx and esophagus just below recessus piriformis obstructing almost the whole of the lumen and blocking the distal passage was detected. Computed tomography revealed marked narrowing secondary to osseous hypertrophy in the air column of the hypopharynx and proximal esophagus. Diffuse idiopathic skeletal hyperostosis or Forestier’s disease is an idiopathic disease characterized by the ossification of the anterior longitudinal ligament of vertebra and some of the extraspinal ligaments. In the present case we aim to discuss an elderly patient who suffered from dysphagia and weight loss and the diagnostic stages. PMID:20355249

State of the art of chronic spontaneous urticaria in Italy: a multicentre survey to evaluate physicians' and patients' perspectives.

PubMed

Rimoldi, Marco; Rossi, Oliviero; Rota, Nadia

2016-10-14

To assess the clinical status of chronic spontaneous urticaria (CSU) and understand treatment approaches in Italy through specialists who treat CSU (dermatologists and allergy specialists) and CSU patients' experience. Multicentre survey. Online structured questionnaires (one for physicians and one for patients). Physicians and patients with CSU in Italy. None. Physician and patient attitudes/experiences. Survey results from 160 allergy and 160 dermatology specialists show that specialists see a median of 40 (IQR 20-80) patients with CSU/year. While most specialists (56%) know the CSU guidelines, only 27% use them regularly (36% of allergy specialists vs 18% of dermatologists). This is reflected in treatment choices with differences between physicians who use guidelines regularly and those who do not: 91.6% vs 71.7% choose standard-dose, non-sedating antihistamines as first-line treatment; 85.9% vs 56.0% select up-dosing for second-line treatment and 65.3% vs 37.2% add leukotriene receptor antagonists or H 2 -antihistamines as third-line treatment. The diaries from 1385 patients highlight that, regardless of treatment regimen, 29.4% of currently treated patients are refractory to therapy. Specialists aim to resolve symptoms and only 7.8% report improving quality of life (QoL) as a priority. Only 16.6% of specialists are familiar with and use the Urticaria Activity Score while 46.9% do not know it. Overall, 537 patients with CSU were surveyed (median age 37 years, IQR 30-46; 44.3% men; median disease duration 5 years, IQR 3-20). Approximately 62% confirm that CSU negatively impacts their QoL. Patients also complain of difficulties in getting information and support: <5% of medical centres provide patient support services. In Italy, the gap between guideline-based care and QoL-related needs in CSU patients affects treatment satisfaction. This information could be used to improve the management of CSU in Italy. Published by the BMJ Publishing Group Limited. For

Sigmoid Sinus Diverticulum, Dehiscence, and Venous Sinus Stenosis: Potential Causes of Pulsatile Tinnitus in Patients with Idiopathic Intracranial Hypertension?

PubMed

Lansley, J A; Tucker, W; Eriksen, M R; Riordan-Eva, P; Connor, S E J

2017-09-01

Pulsatile tinnitus is experienced by most patients with idiopathic intracranial hypertension. The pathophysiology remains uncertain; however, transverse sinus stenosis and sigmoid sinus diverticulum/dehiscence have been proposed as potential etiologies. We aimed to determine whether the prevalence of transverse sinus stenosis and sigmoid sinus diverticulum/dehiscence was increased in patients with idiopathic intracranial hypertension and pulsatile tinnitus relative to those without pulsatile tinnitus and a control group. CT vascular studies of patients with idiopathic intracranial hypertension with pulsatile tinnitus ( n = 42), without pulsatile tinnitus ( n = 37), and controls ( n = 75) were independently reviewed for the presence of severe transverse sinus stenosis and sigmoid sinus diverticulum/dehiscence according to published criteria. The prevalence of transverse sinus stenosis and sigmoid sinus diverticulum/dehiscence in patients with idiopathic intracranial hypertension with pulsatile tinnitus was compared with that in the nonpulsatile tinnitus idiopathic intracranial hypertension group and the control group. Further comparisons included differing degrees of transverse sinus stenosis (50% and 75%), laterality of transverse sinus stenosis/sigmoid sinus diverticulum/dehiscence, and ipsilateral transverse sinus stenosis combined with sigmoid sinus diverticulum/dehiscence. Severe bilateral transverse sinus stenoses were more frequent in patients with idiopathic intracranial hypertension than in controls ( P < .001), but there was no significant association between transverse sinus stenosis and pulsatile tinnitus within the idiopathic intracranial hypertension group. Sigmoid sinus dehiscence (right- or left-sided) was also more common in patients with idiopathic intracranial hypertension compared with controls ( P = .01), but there was no significant association with pulsatile tinnitus within the idiopathic intracranial hypertension group. While our data

Cross-Cultural Adaptation of the Urticaria Control Test From German to Castilian Spanish.

PubMed

García-Díez, I; Curto-Barredo, L; Weller, K; Pujol, R M; Maurer, M; Giménez-Arnau, A M

2015-11-01

The clinical concept of urticaria embraces a heterogeneous group of conditions classified according to their clinical course as acute (lasting less than 6 weeks) or chronic (lasting 6 weeks or more). Chronic urticaria may be either spontaneous or induced. Few tools are available for monitoring the various clinical forms of this disease or for evaluating its impact on quality of life. The recently developed Urticaria Control Test to evaluate disease control is available in German, the original language, and American English. To culturally adapt the long and short versions of the Urticaria Control Test to Castilian Spanish to ensure equivalence between the translated items and those of the original version. To translate the Urticaria Control Test we followed the International Society for Pharmacoeconomics and Outcomes Research good practice guidelines, starting with forward translation and moving through back translation and cognitive debriefing steps. Three items were modified when the first Spanish version, translated from German, was discussed (cognitive debriefing). The revised translation was then translated back to German and sent to the Urticaria Control Test authors, who modified one item they considered had acquired a different focus through translation. A third Spanish version was then prepared and after minor proofreading changes was considered definitive. This study was the first step in making it possible to use the Urticaria Control Test questionnaire in Castilian Spanish. The next step will be to validate the translated questionnaire. Copyright © 2015 Elsevier España, S.L.U. and AEDV. All rights reserved.

Frequency and pattern of Chinese herbal medicine prescriptions for urticaria in Taiwan during 2009: analysis of the national health insurance database.

PubMed

Chien, Pei-Shan; Tseng, Yu-Fang; Hsu, Yao-Chin; Lai, Yu-Kai; Weng, Shih-Feng

2013-08-15

Large-scale pharmaco-epidemiological studies of Chinese herbal medicine (CHM) for treatment of urticaria are few, even though clinical trials showed some CHM are effective. The purpose of this study was to explore the frequencies and patterns of CHM prescriptions for urticaria by analysing the population-based CHM database in Taiwan. This study was linked to and processed through the complete traditional CHM database of the National Health Insurance Research Database in Taiwan during 2009. We calculated the frequencies and patterns of CHM prescriptions used for treatment of urticaria, of which the diagnosis was defined as the single ICD-9 Code of 708. Frequent itemset mining, as applied to data mining, was used to analyse co-prescription of CHM for patients with urticaria. There were 37,386 subjects who visited traditional Chinese Medicine clinics for urticaria in Taiwan during 2009 and received a total of 95,765 CHM prescriptions. Subjects between 18 and 35 years of age comprised the largest number of those treated (32.76%). In addition, women used CHM for urticaria more frequently than men (female:male = 1.94:1). There was an average of 5.54 items prescribed in the form of either individual Chinese herbs or a formula in a single CHM prescription for urticaria. Bai-Xian-Pi (Dictamnus dasycarpus Turcz) was the most commonly prescribed single Chinese herb while Xiao-Feng San was the most commonly prescribed Chinese herbal formula. The most commonly prescribed CHM drug combination was Xiao-Feng San plus Bai-Xian-Pi while the most commonly prescribed triple drug combination was Xiao-Feng San, Bai-Xian-Pi, and Di-Fu Zi (Kochia scoparia). In view of the popularity of CHM such as Xiao-Feng San prescribed for the wind-heat pattern of urticaria in this study, a large-scale, randomized clinical trial is warranted to research their efficacy and safety.

Autologous Serum Therapy in Chronic Urticaria

PubMed Central

Patil, Sharmila; Sharma, Nidhi; Godse, Kiran

2013-01-01

Autologous serum therapy is a promising therapy for treatment resistant urticaria. This is useful in developing countries as this is economical option. Minimum instruments like centrifuge, syringe and needles are required for the procedure. PMID:23723475

Role of bilastine in the management of allergic rhinitis and urticaria: an Asia-Pacific consensus statement

PubMed Central

Lee, Dennis Lip Yen; Abong, Jovilia; Siasoco, Bella; Chow, Steven KW; Leong, Jern-Lin; Singh, Harvinder; Kuljit, S; Campomanes, Benjamin

2016-01-01

The prevalence of allergic diseases is increasing globally, most particularly in middle- to low-income countries. This article examines the burden of allergic rhinitis and chronic urticaria in the Asia-Pacific region, unmet clinical needs, and the potential role of bilastine in the management of these conditions. An International Advisory Group meeting was convened in association with the Asian Pacific Society of Respirology Annual Congress in November 2014, followed by a literature review, and consensus-based outcomes from the meeting and literature review are described. Regional estimates of the prevalence of allergic rhinitis range from 10% to 50%, while little is known regarding the burden of urticaria in the Asia-Pacific region. A survey of allergy patients in the region identified fast, complete, and long-lasting symptom relief as the medication attributes most important to patients. International treatment guidelines for allergic rhinitis and urticaria advocate the first-line use of second-generation, no-sedating H1-antihistamines, such as bilastine, over their first-generation counterparts and a range of these agents are available to Asia-Pacific patients. The newer agents possess many of the properties of an "ideal" antihistamine (once daily administration, rapid and complete symptom relief, limited potential for drug-drug interactions, minimal side effects). The burgeoning prevalence of allergic diseases in the Asia-Pacific region and the uncontrolled symptoms that these patients experience demand a new antihistamine that offers the highest number of positive features according to the international guidelines. PMID:26844221

Role of bilastine in the management of allergic rhinitis and urticaria: an Asia-Pacific consensus statement.

PubMed

Mösges, Ralph; Lee, Dennis Lip Yen; Abong, Jovilia; Siasoco, Bella; Chow, Steven Kw; Leong, Jern-Lin; Singh, Harvinder; Kuljit, S; Campomanes, Benjamin

2016-01-01

The prevalence of allergic diseases is increasing globally, most particularly in middle- to low-income countries. This article examines the burden of allergic rhinitis and chronic urticaria in the Asia-Pacific region, unmet clinical needs, and the potential role of bilastine in the management of these conditions. An International Advisory Group meeting was convened in association with the Asian Pacific Society of Respirology Annual Congress in November 2014, followed by a literature review, and consensus-based outcomes from the meeting and literature review are described. Regional estimates of the prevalence of allergic rhinitis range from 10% to 50%, while little is known regarding the burden of urticaria in the Asia-Pacific region. A survey of allergy patients in the region identified fast, complete, and long-lasting symptom relief as the medication attributes most important to patients. International treatment guidelines for allergic rhinitis and urticaria advocate the first-line use of second-generation, no-sedating H1-antihistamines, such as bilastine, over their first-generation counterparts and a range of these agents are available to Asia-Pacific patients. The newer agents possess many of the properties of an "ideal" antihistamine (once daily administration, rapid and complete symptom relief, limited potential for drug-drug interactions, minimal side effects). The burgeoning prevalence of allergic diseases in the Asia-Pacific region and the uncontrolled symptoms that these patients experience demand a new antihistamine that offers the highest number of positive features according to the international guidelines.

Eighteen cases of wheat allergy and wheat-dependent exercise-induced urticaria/anaphylaxis sensitized by hydrolyzed wheat protein in soap.

PubMed

Kobayashi, Tomoko; Ito, Tomonobu; Kawakami, Hiroshi; Fuzishiro, Kanzan; Hirano, Hirofumi; Okubo, Yukari; Tsuboi, Ryoji

2015-08-01

Glupearl 19S, an acid-hydrolyzed wheat protein (HWP), is used widely in Japan as a moisturizing ingredient in facial soaps. Since 2010, there has been an increasing number of reports of contact urticaria and wheat allergy resulting from the use of products containing this substance. Sixty-one patients who had used HWP-containing facial soap visited our hospital. Thirty-five of these experienced urticaria or anaphylaxis after consuming wheat-containing food. Eighteen of the 35 patients tested positive to 0.01% Glupearl 19S solution. Wheat-specific IgE and serum gluten-specific IgE were higher in the patients with HWP allergy than in non-HWP allergy patients. Among the patients who tested positive to Glupearl 19S on the skin prick test, nine experienced HWP-wheat-dependent exercise-induced anaphylaxis, and four experienced food-dependent anaphylaxis. Moreover, four of these patients not only experienced food-dependent anaphylaxis but also a worsening of the symptoms during exercise. The clinical symptomology was so variable that the patients were classified into six groups. We found that patients with HWP allergy tended to manifest symptoms of both HWP-wheat-dependent exercise-induced anaphylaxis and contact urticaria. The etiology of hydrolyzed wheat protein allergy is unknown. Patients with a history of these symptoms need to be informed about the risk of consuming wheat-containing foods and the importance of excluding such items from their diet. © 2015 The International Society of Dermatology.

Perioperative Care of a Patient with Refractory Idiopathic Thrombocytopenic Purpura Undergoing Total Knee Arthroplasty

PubMed Central

Gudimetla, Veera; Stewart, Andrew; Luscombe, Karen L; Charalambous, Charalambos P

2012-01-01

Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disorder leading to low platelet count and an increased risk of bleeding. Major joint replacement surgery in a patient with ITP can be associated with severe postoperative bleeding. We present our experience of perioperative management in a patient with severe refractory chronic idiopathic thrombocytopenic purpura who successfully underwent a cemented total knee replacement. PMID:23269964

Refractory case of adrenergic urticaria successfully treated with clotiazepam.

PubMed

Kawakami, Yukari; Gokita, Mari; Fukunaga, Atsushi; Nishigori, Chikako

2015-06-01

Adrenergic urticaria (AU) is a rare type of stress-induced physical urticaria characterized by widespread pruritic urticarial papules. Diagnosis can be made by i.d. injection of adrenaline or noradrenaline, which produces the characteristic rash. Although the lesions of AU typically respond to beta-blockers such as propranolol, the therapeutic options for AU are limited. Here, we report a case of AU that was resistant to beta-blockers and successfully treated with clotiazepam. The clinical picture of AU resembles that of cholinergic urticaria (CU), however, positive noradrenaline test and negative acetylcholine skin test were useful for the differential diagnosis of AU and CU. Although his symptoms were resistant to several therapeutic methods including olopatadine (H1 antagonist), lafutidine (H2 antagonist) and propranolol, the severity and frequency of his attacks and his subjective symptoms were reduced by oral clotiazepam, an anxiolytic benzodiazepine. Dermatologists should be aware that anxiolytic benzodiazepines may be a therapeutic option in AU. © 2015 Japanese Dermatological Association.

Cosmetic components causing contact urticaria: a review and update.

PubMed

Verhulst, Lien; Goossens, An

2016-12-01

Immediate skin reactions are common in dermatological practice, but may often be overlooked. The main objective of this article is to provide an update of the literature concerning immediate-type reactions or contact urticaria/contact urticaria syndrome caused by cosmetic ingredients in terms of immediate clinical symptoms, positive reactions following open, scratch or, most often, prick testing, and sometimes the detection of specific IgE antibodies. To this end, a selective search in different medical literature databases was performed. This yielded a list of cosmetic ingredients causing immediate reactions, including hair dyes and bleaches, preservatives, fragrance and aroma chemicals, sunscreens, hair glues, plant-derived and animal-derived components, permanent makeup and tattoos, glycolic acid peel, lip plumper, and alcohols. Many of the reported cases, however, lack appropriate controls and detailed investigation. Contact urticaria may occur with or without systemic symptoms, which are sometimes life-threatening. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Lack of association of programmed cell death 1 gene (PDCD1) polymorphisms with susceptibility to chronic urticaria in patients with positive autologous serum skin test.

PubMed

Brzoza, Z; Grzeszczak, W; Trautsolt, W; Moczulski, D

2012-01-01

Autoimmune mechanisms play an important role in the pathophysiology of chronic urticaria (CU), and the autologous serum skin test (ASST) helps to identify patients with autoreactive CU. One of the factors involved in autoreactive mechanisms is the cell surface receptor programmed death-1 which is encoded by the programmed cell death 1 gene (PDCD1). To investigate whether PDCD1 polymorphisms influence susceptibility to CU. We enrolled 93 ASST-positive patients with CU and a control group consisting of 105 healthy volunteers. In all individuals, PD1.3 (7146 A/G; rs 11568821) and PD1.5 (7785 C/T; rs 2227981) polymorphisms were analyzed. No statistically significant differences were found between CU patients and controls for allele or genotype distribution. We also did not observe any association between PDCD1 genotypes and severity of urticaria or age of disease onset. PD1.3 and PD1.5 polymorphisms were not proven to be implicated in susceptibility to ASST-positive CU in the Polish population. A more comprehensive analysis of the 2q33-2q37 genomic region might reveal whether variants of 1 or more of the genes in this region are involved in susceptibility to CU.

Usefulness of the CD63 basophil activation test in detecting Anisakis hypersensitivity in patients with chronic urticaria: diagnosis and follow-up.

PubMed

Frezzolini, A; Cadoni, S; De Pità, O

2010-10-01

The basophil activation test (BAT) has been recently described as a useful in vitro tool for diagnosis of allergy to Anisakis species in patients with acute urticaria. To evaluate the relationship between sensitization to Anisakis simplex and chronic urticaria (CU), using flow cytometry analysis of in vitro BAT. Methods.  A. simplex sensitization was evaluated in patients with CU (n = 57) and in atopic (n = 22) and healthy controls (n = 20) by means of skin prick test (SPT), specific IgE and Anisakis-induced BAT using a triple-labelled strategy with anti-CD123, anti-human leucocyte antigen DR and anti-CD63 antibodies. During a follow-up period of 6 months in 10 patients with CU who accepted a fish-free dietary regimen, the diagnostic performance of the in vivo and in vitro methods was calculated, and changes in specific IgE and BAT were evaluated with respect to clinical response. A significant association between CU and A. simplex sensitization was found, with an overall prevalence of 75.4% in patients with CU (43/57) compared with 18% (4/22) and 10% (2/20) of the atopic and healthy controls, respectively (P < 0.0001). BAT (cut-off > 13%) had the highest sensitivity and specificity, with significantly better ability than specific IgE testing for the identification of A. simplex sensitization in patients with CU. During the 6-month follow-up, clinical improvement was seen in all patients, and specific IgE and BAT results decreased to normal values in 6/10 (60%) and 10/10 (100%) patients, respectively. BAT can be considered a reliable new in vitro method to evaluate A. simplex hypersensitivity in patients with CU, supplementing standardized procedures in both diagnosis and follow-up. © 2009 The Author(s). Journal compilation © 2009 British Association of Dermatologists.

Brain tissue water content in patients with idiopathic normal pressure hydrocephalus.

PubMed

Aygok, G; Marmarou, A; Fatouros, P; Young, H

2006-01-01

Relatively little is known regarding the water content of brain tissue in idiopathic normal-pressure hydrocephalus (NPH) patients. The objective of our study was to determine absolute water content non-invasively in hydrocephalic patients, particularly in the anterior and posterior ventricular horns and in the periventricular white matter. Ten patients who were diagnosed and treated for idiopathic NPH in our clinic were selected for study. Magnetic resonance imaging (MRI) techniques were used to obtain anatomical image slices for quantitative brain water measurements. Apparent diffusion coefficient measures were also extracted from regions of interest. To our knowledge, this is the first study to confirm that periventricular lucency seen on MRI represents increased water content in the extracellular space that is markedly elevated prior to shunting.

Impact of sildenafil on survival of patients with idiopathic pulmonary arterial hypertension.

PubMed

Zeng, Wei-Jie; Sun, Yun-Juan; Gu, Qing; Xiong, Chang-Ming; Li, Jian-Jun; He, Jian-Guo

2012-09-01

It has been reported that short-term sildenafil therapy is safe and effective for patients with pulmonary arterial hypertension. However, data regarding the impact of sildenafil on the survival of patients with idiopathic pulmonary arterial hypertension remain limited. The study was conducted on 77 patients with newly diagnosed idiopathic pulmonary arterial hypertension at Fu Wai Hospital between September 2005 and September 2009. Patients were divided into 2 groups: the sildenafil group and the conventional group. Nine patients treated with sildenafil were re-evaluated by right heart catheterization after 3 months. Our data demonstrated that the 6-minute walk distance, World Health Organization functional class, mixed venous oxygen saturation, and hemodynamics significantly improved after 3 months of sildenafil therapy (P < .05). The baseline characteristics of the sildenafil group were similar to those of the conventional group. The 1-, 2-, and 3-year survival rates in the sildenafil group were 88%, 72%, and 68% compared with 61%, 36%, and 27% in the conventional group (P < .001). The absence of sildenafil therapy, lower body mass index, and lower mixed venous oxygen saturation were found to be independent predictors of mortality. In conclusion, sildenafil therapy was found to be associated with improved survival in patients with idiopathic pulmonary arterial hypertension.

[Normal lung volumes in patients with idiopathic pulmonary fibrosis and emphysema].

PubMed

Casas, Juan Pablo; Abbona, Horacio; Robles, Adriana; López, Ana María

2008-01-01

Pulmonary function tests in idiopathic pulmonary fibrosis characteristically show a restrictive pattern, resulting from reduction of pulmonary compliance due to diffuse fibrosis. Conversely, an obstructive pattern with hyperinflation results in emphysema by loss of elastic recoil, expiratory collapse of the peripheral airways and air trapping. Previous reports suggest that when both diseases coexist, pulmonary volumes are compensated and a smaller than expected reduction or even normal lung volumes can be found. We report 4 male patients of 64, 60, 73 and 70 years, all with heavy cigarette smoking history and progressive breathlessness. Three of them had severe limitation in their quality of life. All four showed advanced lung interstitial involvement, at high resolution CT scan, fibrotic changes predominantly in the subpleural areas of lower lung fields and concomitant emphysema in the upper lobes. Emphysema and pulmonary fibrosis was confirmed by open lung biopsy in one patient. The four patients showed normal spirometry and lung volumes with severe compromise of gas exchange and poor exercise tolerance evaluated by 6 minute walk test. Severe pulmonary arterial hypertension was also confirmed in three patients. Normal lung volumes does not exclude diagnosis of idiopathic pulmonary fibrosis in patients with concomitant emphysema. The relatively preserved lung volumes may underestimate the severity of idiopathic pulmonary fibrosis and attenuate its effects on lung function parameters.

Isolated contact urticaria caused by immunoglobulin E-mediated fish allergy.

PubMed

Onesimo, Roberta; Giorgio, Valentina; Pill, Stefania; Monaco, Serena; Sopo, Stefano Miceli

2012-01-01

Fish is a common cause of food allergy. The reactions usually occur after its ingestion. In most immunoglobulin E-mediated reactions, the allergens are gastroresistant and heat-stable proteins of low molecularweight (parvalbumin). On the other hand, isolated contact urticaria following the handling of raw fish but without symptoms after its ingestion was found among cooks and professional fish handlers. In these cases, the fish allergens are gastrosensitive and thermolabile, as demonstrated by the decrease in the diameter of the wheal in the skin-prick test using cooked fish. To the best of our knowledge isolated fish contact urticaria in children has not been previously reported. We analyze the features of three pediatric cases of contact urticaria from cod (one of them was sensitized to parvalbumin), with tolerance after ingestion of this fish on oral food challenge.

Long-term efficacy of abatacept in pediatric patients with idiopathic uveitis: a case series.

PubMed

Marrani, Edoardo; Paganelli, Valeria; de Libero, Cinzia; Cimaz, Rolando; Simonini, Gabriele

2015-10-01

Non-infectious uveitis represents one of the most common causes of blindness, even at pediatric age; in particular, idiopathic chronic uveitis can pose significant difficulties during treatment, due to a partial response to TNF-α antagonists. To date, very few case series exist describing the treatment of idiopathic uveitis not adequately controlled by TNF-α antagonists. The aim of our study is to describe the role of abatacept in achieving remission in patients with idiopathic uveitis previously treated with TNF-α antagonists, and to assess how long abatacept efficacy is maintained during follow-up. The treatment's safety profile and tolerability were also specifically investigated. Three patients affected with chronic idiopathic uveitis, who have been treated with abatacept due to loss of efficacy of TNF-α antagonists, were reviewed. Details of the demographic and clinical characteristics were recorded, and a summary of the medical history was obtained. Patients were regularly reviewed in the ophthalmology and rheumatology clinics. Assessment of their ocular condition was characterized according to the Standardization of Uveitis Nomenclature (SUN) group. In our patients, abatacept was able to induce remission and to discontinue systemic corticosteroids after a mean of 30 weeks; the drug maintained its efficacy through a long follow-up period (42, 33, and 18 months respectively), with an excellent safety profile. Our small case series seems to suggest abatacept to be a promising therapy in children affected with chronic idiopathic uveitis not adequately controlled by TNF-α antagonists.

A case of recurrent toxocariasis presenting with urticaria.

PubMed

Kim, Min-Hye; Jung, Jae-Woo; Kwon, Jae-Woo; Kim, Tae-Whan; Kim, Sae-Hoon; Cho, Sang-Heon; Min, Kyung-Up; Kim, You-Young; Chang, Yoon-Seok

2010-10-01

Human toxocariasis is the most prevalent helminthiasis in Korea and other industrialized countries. The clinical features of toxocariasis are diverse, according to the involved organ. Typically, Toxocara spp. infection is easily treated with 400 mg albendazole twice a day for 5 days. However, we experienced a case of recurrent toxocariasis that was refractory to this standard therapy and presented with urticaria, an uncommon symptom in toxocariasis. A 35-year-old male visited our emergency room because of abdominal pain. He had recently consumed raw cow liver (3 weeks prior to presentation). Laboratory analyses revealed eosinophilia (1,612 cells/µL) and increased total IgE (3,060 IU/mL). Chest X-ray showed multiple lung nodules in both lungs, and computed tomography revealed multiple ground-glass opacities in both lungs and multiple tiny liver abscesses. Liver biopsy revealed an eosinophilic abscess. Enzyme-linked immunosorbent assay findings for Toxocara antigens were positive (optical density, 2.140), leading to a diagnosis of toxocariasis. We initiated a 5-day treatment with albendazole and prednisolone; however, 6 days after completing the treatment, the patient again experienced urticaria and severe itching that could not be controlled by antihistamines or hydrocortisone cream. A second bout of eosinophilia suggested recurring toxocariasis, for which we prescribed a second round of albendazole. Despite an initial improvement in his symptoms, the patient returned after 6 weeks complaining of abdominal pain for 6 hours, which was reminiscent of his first attack; he also exhibited eosinophilia. Accordingly, albendazole was administered once more for an additional 3 weeks, and his symptoms resolved.

A Case of Recurrent Toxocariasis Presenting With Urticaria

PubMed Central

Kim, Min-Hye; Jung, Jae-Woo; Kwon, Jae-Woo; Kim, Tae-Whan; Kim, Sae-Hoon; Cho, Sang-Heon; Min, Kyung-Up; Kim, You-Young

2010-01-01

Human toxocariasis is the most prevalent helminthiasis in Korea and other industrialized countries. The clinical features of toxocariasis are diverse, according to the involved organ. Typically, Toxocara spp. infection is easily treated with 400 mg albendazole twice a day for 5 days. However, we experienced a case of recurrent toxocariasis that was refractory to this standard therapy and presented with urticaria, an uncommon symptom in toxocariasis. A 35-year-old male visited our emergency room because of abdominal pain. He had recently consumed raw cow liver (3 weeks prior to presentation). Laboratory analyses revealed eosinophilia (1,612 cells/µL) and increased total IgE (3,060 IU/mL). Chest X-ray showed multiple lung nodules in both lungs, and computed tomography revealed multiple ground-glass opacities in both lungs and multiple tiny liver abscesses. Liver biopsy revealed an eosinophilic abscess. Enzyme-linked immunosorbent assay findings for Toxocara antigens were positive (optical density, 2.140), leading to a diagnosis of toxocariasis. We initiated a 5-day treatment with albendazole and prednisolone; however, 6 days after completing the treatment, the patient again experienced urticaria and severe itching that could not be controlled by antihistamines or hydrocortisone cream. A second bout of eosinophilia suggested recurring toxocariasis, for which we prescribed a second round of albendazole. Despite an initial improvement in his symptoms, the patient returned after 6 weeks complaining of abdominal pain for 6 hours, which was reminiscent of his first attack; he also exhibited eosinophilia. Accordingly, albendazole was administered once more for an additional 3 weeks, and his symptoms resolved. PMID:20885912

Management of chronic spontaneous urticaria in the elderly.

PubMed

Ventura, Maria Teresa; Cassano, Nicoletta; Romita, Paolo; Vestita, Michelangelo; Foti, Caterina; Vena, Gino Antonio

2015-04-01

The guidelines for the management of urticaria in adults and children have been revised and updated recently. However, there are few data in the literature concerning several aspects of this disease in the elderly (e.g., epidemiology, etiopathogenesis, clinical aspects, association with co-morbidities, efficacy and safety profiles of treatments, and management strategies). This is an obvious deficiency in the data, as this disease causes a deterioration in quality of life, affecting the quality of sleep, everyday life habits and activities, and inducing severe disability. Chronic spontaneous urticaria (CSU) can also be associated with internal, infectious, autoimmune, or neoplastic diseases. It is therefore necessary to pay particular attention to these clinical issues through appropriate clinical examinations. At the same time, the specific features of medications used to treat CSU in the elderly should be carefully evaluated, as its pharmacological treatment raises a number of problems related both to the clinical condition of the patient and to concomitant diseases, as well as to the polypharmacotherapy, which is common in older subjects and may cause safety problems because of the drug interactions. Non-sedating new-generation antihistamines are the mainstay treatment of CSU for the elderly. The efficacy and safety of alternative treatment options have not been assessed in the geriatric population with CSU; corticosteroids and cyclosporine (ciclosporin) should be used by this population with extreme caution. Similarly, there are no data regarding the actual safety profile of the new-generation antihistamines at higher doses than those recommended in elderly patients.

Bilastine: a new H1 -antihistamine with an optimal profile for updosing in urticaria.

PubMed

Church, M K; Labeaga, L

2017-09-01

This review set out to examine published papers detailing the efficacy of bilastine in skin models and urticaria to assess whether it meets the optimal profile for updosing in urticaria, that is, strong clinical efficacy and freedom from unwanted side effects, particularly sedation. Bilastine is a highly effective H 1 -antihistamine even when used at the basic dose of 20 mg daily. Its facilitated uptake after oral dosage gives it a rapid onset and long duration of action. In both wheal and flare studies and in urticaria updosing fourfold showed increased effectiveness. With respect to somnolence, bilastine is a substrate for P-glycoprotein, a membrane pump which prevents it crossing the blood-brain barrier. Consequently, bilastine is a practically 'non-sedating' H 1 -antihistamine. In conclusion, the excellent profile of bilastine in both efficacy and safety make it the ideal H 1 -antihistamine for updosing the daily dose fourfold in difficult-to-treat urticaria as recommended by the EAACI/GA 2 LEN/EDF/WAO guideline for the management of urticaria. © 2017 European Academy of Dermatology and Venereology.

Natural history of idiopathic diabetes insipidus.

PubMed

Richards, Gail E; Thomsett, Michael J; Boston, Bruce A; DiMeglio, Linda A; Shulman, Dorothy I; Draznin, Martin

2011-10-01

To determine what percentage of diabetes insipidus (DI) in childhood is idiopathic and to assess the natural history of idiopathic DI. We conducted a retrospective chart review of 105 patients with DI who were born or had DI diagnosed between 1980-1989 at 3 medical centers. A second cohort of 30 patients from 6 medical centers in whom idiopathic DI was diagnosed after 1990 was evaluated retrospectively for subsequent etiologic diagnoses and additional hypothalamic/pituitary deficiencies and prospectively for quality of life. In the first cohort, 11% of patients had idiopathic DI. In the second cohort, additional hypothalamic/pituitary hormone deficiencies developed in 33%, and 37% received an etiologic diagnosis for DI. Health-related quality of life for all the patients with idiopathic DI was comparable with the healthy reference population. Only a small percentage of patients with DI will remain idiopathic after first examination. Other hormone deficiencies will develop later in one-third of those patients, and slightly more than one-third of those patients will have an etiology for the DI diagnosed. Long-term surveillance is important because tumors have been diagnosed as long as 21 years after the onset of DI. Quality of life for these patients is as good as the reference population. Copyright © 2011 Mosby, Inc. All rights reserved.

Frequency and pattern of Chinese herbal medicine prescriptions for urticaria in Taiwan during 2009: analysis of the national health insurance database

PubMed Central

2013-01-01

Background Large-scale pharmaco-epidemiological studies of Chinese herbal medicine (CHM) for treatment of urticaria are few, even though clinical trials showed some CHM are effective. The purpose of this study was to explore the frequencies and patterns of CHM prescriptions for urticaria by analysing the population-based CHM database in Taiwan. Methods This study was linked to and processed through the complete traditional CHM database of the National Health Insurance Research Database in Taiwan during 2009. We calculated the frequencies and patterns of CHM prescriptions used for treatment of urticaria, of which the diagnosis was defined as the single ICD-9 Code of 708. Frequent itemset mining, as applied to data mining, was used to analyse co-prescription of CHM for patients with urticaria. Results There were 37,386 subjects who visited traditional Chinese Medicine clinics for urticaria in Taiwan during 2009 and received a total of 95,765 CHM prescriptions. Subjects between 18 and 35 years of age comprised the largest number of those treated (32.76%). In addition, women used CHM for urticaria more frequently than men (female:male = 1.94:1). There was an average of 5.54 items prescribed in the form of either individual Chinese herbs or a formula in a single CHM prescription for urticaria. Bai-Xian-Pi (Dictamnus dasycarpus Turcz) was the most commonly prescribed single Chinese herb while Xiao-Feng San was the most commonly prescribed Chinese herbal formula. The most commonly prescribed CHM drug combination was Xiao-Feng San plus Bai-Xian-Pi while the most commonly prescribed triple drug combination was Xiao-Feng San, Bai-Xian-Pi, and Di-Fu Zi (Kochia scoparia). Conclusions In view of the popularity of CHM such as Xiao-Feng San prescribed for the wind-heat pattern of urticaria in this study, a large-scale, randomized clinical trial is warranted to research their efficacy and safety. PMID:23947955

Immunosuppressive therapy for patients with Down syndrome and idiopathic aplastic anemia.

PubMed

Suzuki, Kyogo; Muramatsu, Hideki; Okuno, Yusuke; Narita, Atsushi; Hama, Asahito; Takahashi, Yoshiyuki; Yoshida, Makoto; Horikoshi, Yasuo; Watanabe, Ken-Ichiro; Kudo, Kazuko; Kojima, Seiji

2016-07-01

Idiopathic aplastic anemia (AA) is a rare hematological complication of Down syndrome (DS). The safety and efficacy of immunosuppressive therapy (IST) in individuals with DS remain unknown. We used a standard regimen of IST, comprising antithymocyte globulin and cyclosporine A, to treat three children with DS and idiopathic acquired AA. Two patients achieved a hematological (complete or partial) response and became transfusion independent at the final follow-up. The third patient failed to respond to IST and underwent bone marrow transplantation from a human leukocyte antigen (HLA)-mismatched unrelated donor. None of the patients experienced severe or unexpected adverse events during IST. Our experience suggests that IST is a safe and reasonable treatment, even in individuals with DS who suffer from AA and lack an HLA-matched sibling donor.

Effects of standing on cerebrovascular resistance in patients with idiopathic orthostatic intolerance

NASA Technical Reports Server (NTRS)

Jacob, G.; Atkinson, D.; Jordan, J.; Shannon, J. R.; Furlan, R.; Black, B. K.; Robertson, D.

1999-01-01

PURPOSE: Patients with idiopathic orthostatic intolerance often have debilitating symptoms on standing that are suggestive of cerebral hypoperfusion despite the absence of orthostatic hypotension. SUBJECTS AND METHODS: We evaluated the effects of graded head-up tilt on cerebral blood flow as determined by transcranial Doppler measurements in 10 patients with idiopathic orthostatic intolerance (nine women, one man, 22 to 47 years) and nine age- and sex-matched control subjects. RESULTS: In patients, mean (+/- SD) arterial pressure at 0 degrees head-up tilt was 90 +/- 11 mm Hg and was well maintained at all tilt angles (90 +/- 11 mm Hg at 75 degrees). In controls, mean arterial pressure was 85 +/- 7 mm Hg at 0 degrees and 82 +/- 11 mm Hg at 75 degrees head-up tilt. There was a substantial decrease in peak velocity with increasing tilt angle in patients (28% +/- 10%) but not in controls (10% +/- 10% at 75 degrees, P <0.001). Similarly, mean velocity decreased 26% +/- 13% in patients and 12% +/- 11% in controls (P = 0.01). With increasing head-up tilt, patients had a significantly greater increase in regional cerebrovascular resistance than controls. CONCLUSIONS: In patients with idiopathic orthostatic intolerance, peak and mean middle cerebral artery blood flow velocity decreased in response to head-up tilt despite well sustained arterial blood pressure. These observations indicate that in this group of patients, regulation of cerebrovascular tone may be impaired and might therefore be a target for therapeutic interventions.

The prevalence of intraspinal anomalies in infantile and juvenile patients with "presumed idiopathic" scoliosis: a MRI-based analysis of 504 patients.

PubMed

Zhang, Wen; Sha, Shifu; Xu, Leilei; Liu, Zhen; Qiu, Yong; Zhu, Zezhang

2016-04-27

Though several studies have reported the incidence of intraspinal neural axis abnormalities in infantile and juvenile "presumed idiopathic" scoliosis, there has been a varying prevalence ranging from 11.1 to 26.0% based on a limited sample size. Therefore, such inconclusive findings have resulted in some questions on the MRI-associated role in the management of these patients. We aimed to investigate the prevalence and distribution of intraspinal anomalies in the infantile and juvenile patients with "presumed idiopathic" scoliosis and to explore the radiographic and clinical indicators with large sample size. A total of 504 infantile and juvenile patients diagnosed with "presumed idiopathic" scoliosis were examined for potentially-existing neural axis abnormalities by MRI. Patients were grouped into two cohorts according to the presence of neural axis abnormalities. Radiographic parameters including curve magnitude, curve pattern, location of apex, degree of thoracic kyphosis, and span of curve were recorded and compared between the two groups. The prevalence of the neural abnormalities between the infantile-age group and juvenile-age group was also compared. The student t test was used to evaluate the differences of continuous variables and the chi-square test was used to evaluate the difference of categorical variables. Fisher exact test was applied to detect the difference of the rate of intraspinal anomalies between the "infantile idiopathic scoliosis" and "juvenile idiopathic scoliosis" group. Involving the spinal cord, 94 patients (18.7%) were found to have a neural abnormality: Arnold-Chiari malformation alone in 43 patients, Arnold-Chiari malformation combined with syringomyelia in 18 patients, isolated syringomyelia in 13 patients, diastematomyelia in six patients, tethered cord combined with diastematomyelia in six patients, tethered cord alone in four patients, and other uncommon intraspinal abnormalities in the remaining four patients. Totally Arnold

Prevalence of dyslipidemia among Iranian patients with idiopathic tinnitus.

PubMed

M-Shirazi, Minoo; Farhadi, Mohammad; Jalessi, Maryam; Kamrava, Seyyed-Kamran; Behzadi, Ashkan Heshmatzade; Arami, Behin

2011-07-01

Tinnitus is a sense of sound perception in absence of an external source which can affect life quality. Different conditions may lead to tinnitus including metabolic disorders such as dyslipidemia. The aim of this study was to investigate the prevalence of dyslipidemia among Iranian patients with idiopathic tinnitus. This was a cross-sectional study in which prevalence of dyslipidemia in fasting state and its subclasses were assessed in 1043 tinnitus patients aged 12-90 years who referred to Rasool Akram Hospital, Tehran, Iran, 2006-2009. Data was summarized by SPSS software version 17 and one sample t-test and Chi-Square test were applied to analyze the results. P less than 0.05 were considered significant. The most prevalent type of dyslipidemia was hypercholesterolemia with the frequency of 14.4% followed by low HDL-C with the frequency of 12.8%. Mean of total cholesterol, HDL-C, LDL-C and triglyceride levels in all patients were not greater than general population. Based on the results of the present study, there might be no need to check the serum lipid profile in tinnitus patients. We recommend further studies to assess both fasting and postprandial serum lipid profile in patients with idiopathic tinnitus. Simultaneous investigation of their dietary intake is also suggested.

Immunologic contact urticaria--the human touch.

PubMed

Wang, Christina Y; Maibach, Howard I

2013-06-01

To review immunologic contact urticaria (ICU) in the occupational and environmental context, and describe its continued relevance in light of the ever-increasing onslaught of new chemicals and products, as well as new technology placing novel interactions, such as nanoparticles, within reach of the population at home and work. Publications were searched via PubMed, using key words: Occupational, immunologic, contact urticaria, nanoparticle. ICU remains an important diagnosis to make and treat because it has widespread health and social morbidity, including job and income loss, persistent life-long allergies, and progression from self-limiting skin eruptions to multi-systemic, sometimes life-threatening, illnesses. There is no short supply of known ICU causing allergens, but it is equally important to be ever vigilant in recognizing, and even adding to, items in the constantly expanding list of novel allergenic agents provided to us by the advances of modern chemistry and technology, and by the changing social structure and lifestyle dynamics.

Increased mean platelet volume in patients with idiopathic subjective tinnitus.

PubMed

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

2016-11-01

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

Management of adult patients with persistent idiopathic thrombocytopenic purpura following splenectomy: a systematic review.

PubMed

Vesely, Sara K; Perdue, Jedidiah J; Rizvi, Mujahid A; Terrell, Deirdra R; George, James N

2004-01-20

Treatment of chronic refractory idiopathic thrombocytopenic purpura is a dilemma because many patients have minimal symptoms, response to treatment is uncertain, and treatments may have serious adverse effects. To determine the effectiveness of treatments for adult patients with idiopathic thrombocytopenic purpura who have not responded to splenectomy. English-language reports from 1966 through 2003 that were retrieved from MEDLINE and Reference Update and bibliographies of retrieved articles. Articles reporting 5 or more total patients were reviewed to select eligible patients. Patients were eligible for inclusion if they were more than 16 years of age, had idiopathic thrombocytopenic purpura for more than 3 months, had a previous splenectomy, and had a platelet count less than 50 x 10(9) cells/L. Patients were assessed for platelet count response, bleeding complications, duration of follow-up, and death. Complete remission was defined as a normal platelet count with no treatment for more than 3 months and for the duration of follow-up. 90 articles with 656 patients treated with 22 therapies met selection criteria. Azathioprine, cyclophosphamide, and rituximab had the most reported complete responses, but they were reported in only 41 to 109 patients. Reported complete response rates ranged from 17% to 27%, but 36% to 42% of patients had no response with these 3 treatments. Most reports described only platelet count responses; bleeding outcomes were reported in only 63 patients (10%). Only 111 (17%) of the 656 eligible patients had pretreatment platelet counts of less than 10 x 10(9) cells/L. No treatment method was reported in more than 20 patients. Evidence for the effectiveness of any treatment for patients with idiopathic thrombocytopenic purpura and persistent severe thrombocytopenia after splenectomy is minimal. Potentially effective treatments must be evaluated by randomized, controlled trials to determine both benefit and safety.

Febrile urticaria in a family: uncommon manifestation of a common disease.

PubMed

Sharma, Vishal; Singhal, Mayank; Sharma, Alka; Kumar, Vivek

2012-12-15

Cutaneous manifestations are uncommon with malaria. These include urticaria, purpura fulminans, and petechial rash. We report on a series of three patients from a single family who had an urticarial rash with fever that was subsequently diagnosed to be caused by malaria. Urticarial rash has been previously reported with both falciparum and vivax malaria infections. Although the exact pathogenesis is not clear urticarial rash might be related with IgE mediated mast cell degranulation.

Influence of proprioceptive insoles on spinal curvature in patients with slight idiopathic scoliosis.

PubMed

Noll, Christine; Steitz, Vanessa; Daentzer, Dorothea

2017-01-01

Proprioceptive insoles are known to influence the functions of posture and gait by modulations of the sensory structures at the sole of the foot. Literature has shown that they could improve the position of the upper-body in patients with postural complaints of the musculoskeletal system. The aim of this study was to evaluate the influence of proprioceptive insoles on the spinal curvature in patients with slight idiopathic scoliosis. Eighteen patients were included in this prospective, single-centre, randomized study. All patients needed to have a relevant growth potential and suffered from a slight idiopathic scoliosis. Two groups were used, where group 1 performed physiotherapy twice a week, whereas group 2 was additionally supplied with proprioceptive insoles. Patients underwent three-dimensional rasterstereography for back-shape analysis. Furthermore, a conventional x-ray imaging of the spine was performed at the beginning and 1 year later to document the curvatures. There was no statistical difference in the Cobb angles, and in almost all parameters of the rasterstereography, there was no statistically significant change between and within both groups. According to the results of this study, there was no evidence of any statistical significant effect of proprioceptive insoles on spinal curvature in patients with slight idiopathic scoliosis.

Baseline factors predictive of patient satisfaction with sacral neuromodulation for idiopathic fecal incontinence.

PubMed

Duelund-Jakobsen, Jakob; van Wunnik, Bart; Buntzen, Steen; Lundby, Lilli; Laurberg, Søren; Baeten, Cor

2014-07-01

Sacral neuromodulation (SNM) is an established treatment for fecal incontinence (FI). A recent study from our group found that the relationship between patient satisfaction and clinical outcome is complex and does not match the traditional used success criteria. Therefore, the ability to predict patient satisfaction must be given priority. The aim of the present study is to identify baseline factors predictive of patient satisfaction, with SNM, for idiopathic FI. We analyzed data from patients treated with SNM for idiopathic FI in Aarhus, Denmark, and Maastricht, The Netherlands. A questionnaire considering self-reported satisfaction was mailed to these patients and compared to baseline characteristics. Logistic regression was used to determine the predictive value of baseline demographic and diagnostic variables. In total, 131 patients were included in the analysis. Patient satisfaction with the current treatment result was reported in 75 patients. Fifty-six patients were dissatisfied with SNM treatment, after median 46 months (range 11-122) with permanent implantation. Pudendal nerve terminal motor latency (PNTML) was the solely identified predictor for long-term patient satisfaction. A subgroup univariate-logistic regression analysis showed that PNTML ≤ 2.3 ms at the side of lead implantation was a statistically significant predictor for patient satisfaction (odds ratio (OR) 2.3, 95% confidence interval (CI) 1.01-5.24, p = 0.048). Baseline PNTML measurement may be predictive of long-term satisfaction with SNM therapy for idiopathic FI. Further studies are needed to confirm this result.

An overview of the novel H1-antihistamine bilastine in allergic rhinitis and urticaria.

PubMed

Jáuregui, Ignacio; García-Lirio, Eduardo; Soriano, Ana María; Gamboa, Pedro M; Antépara, Ignacio

2012-01-01

Currently available second-generation H1-antihistamines include a wide group of drugs with a better therapeutic index (or risk-benefit ratio) than the classic antihistamines, although their properties and safety profiles may differ. Bilastine is a newly registered H1-antihistamine for the oral treatment of allergic rhinitis and urticaria, with established antihistaminic and antiallergic properties. Clinical studies in allergic rhinitis and chronic urticaria show that once-daily treatment with bilastine 20 mg is effective in managing symptoms and improving patient's quality of life, with at least comparable efficacy to other nonsedative H1-antihistamines. As far as studies in healthy volunteers, clinical assays and clinical experience can establish, bilastine's safety profile is satisfactory, since it lacks anticholinergic effects, does not impair psychomotor performance or actual driving, and appears to be entirely free from cardiovascular effects.

Quality of life in patients with an idiopathic rapid eye movement sleep behaviour disorder in Korea.

PubMed

Kim, Keun Tae; Motamedi, Gholam K; Cho, Yong Won

2017-08-01

There have been few quality of life studies in patients with idiopathic rapid eye movement sleep behaviour disorder. We compared the quality of life in idiopathic rapid eye movement sleep behaviour disorder patients to healthy controls, patients with hypertension, type 2 diabetes mellitus without complication and idiopathic restless legs syndrome. Sixty patients with idiopathic rapid eye movement sleep behaviour disorder (24 female; mean age: 61.43 ± 8.99) were enrolled retrospectively. The diagnosis was established based on sleep history, overnight polysomnography, neurological examination and Mini-Mental State Examination to exclude secondary rapid eye movement sleep behavior disorder. All subjects completed questionnaires, including the Short Form 36-item Health Survey for quality of life. The total quality of life score in idiopathic rapid eye movement sleep behaviour disorder (70.63 ± 20.83) was lower than in the healthy control group (83.38 ± 7.96) but higher than in the hypertension (60.55 ± 24.82), diabetes mellitus (62.42 ± 19.37) and restless legs syndrome (61.77 ± 19.25) groups. The total score of idiopathic rapid eye movement sleep behaviour disorder patients had a negative correlation with the Pittsburg Sleep Quality Index (r = -0.498, P < 0.001), Insomnia Severity Index (r = -0.645, P < 0.001) and the Beck Depression Inventory-2 (r = -0.694, P < 0.001). Multiple regression showed a negative correlation between the Short Form 36-item Health Survey score and the Insomnia Severity Index (β = -1.100, P = 0.001) and Beck Depression Inventory-2 (β = -1.038, P < 0.001). idiopathic rapid eye movement sleep behaviour disorder had a significant negative impact on quality of life, although this effect was less than that of other chronic disorders. This negative effect might be related to a depressive mood associated with the disease. © 2016 European Sleep Research Society.

Biochemical analysis of tunica vaginalis fluid in patients with or without idiopathic hydroceles.

PubMed

Madlala, T S; Rencken, R K; Bornman, M S; Reif, S; Joubert, H F; Van der Merwe, C A

1994-10-01

To establish the differences, if any, between the biochemical composition of idiopathic hydrocele fluid and the fluid normally present in the tunica vaginalis. Aspiration and sclerotherapy of 37 idiopathic hydroceles from patients who presented to this urology clinic were performed. The biochemical content of the fluid was compared with that of the tunica vaginalis fluid from a small group of controls (n = 8), taken from patients undergoing orchidectomy for carcinoma of the prostate. Differences in several measurements were recorded; in particular, there were significantly higher concentrations of calcium, albumin, total protein and creatine-kinase in the hydrocele group. The levels of potassium, aspartate transaminase, alanine transaminase and alkaline phosphatase were significantly lower in the hydrocele group. Whether these differences have a role in the causation of an idiopathic hydrocele is, at this stage, speculative. A similar study on a larger scale would probably be more conclusive.

Prevalence of dyslipidemia among Iranian patients with idiopathic tinnitus

PubMed Central

M-Shirazi, Minoo; Farhadi, Mohammad; Jalessi, Maryam; Kamrava, Seyyed-Kamran; Behzadi, Ashkan Heshmatzade; Arami, Behin

2011-01-01

BACKGROUND: Tinnitus is a sense of sound perception in absence of an external source which can affect life quality. Different conditions may lead to tinnitus including metabolic disorders such as dyslipidemia. The aim of this study was to investigate the prevalence of dyslipidemia among Iranian patients with idiopathic tinnitus. METHODS: This was a cross-sectional study in which prevalence of dyslipidemia in fasting state and its subclasses were assessed in 1043 tinnitus patients aged 12-90 years who referred to Rasool Akram Hospital, Tehran, Iran, 2006-2009. Data was summarized by SPSS software version 17 and one sample t-test and Chi-Square test were applied to analyze the results. P less than 0.05 were considered significant. RESULTS: The most prevalent type of dyslipidemia was hypercholesterolemia with the frequency of 14.4% followed by low HDL-C with the frequency of 12.8%. Mean of total cholesterol, HDL-C, LDL-C and triglyceride levels in all patients were not greater than general population. CONCLUSIONS: Based on the results of the present study, there might be no need to check the serum lipid profile in tinnitus patients. We recommend further studies to assess both fasting and postprandial serum lipid profile in patients with idiopathic tinnitus. Simultaneous investigation of their dietary intake is also suggested. PMID:22279456

Idiopathic Hypersomnia: A Study of 77 Cases

PubMed Central

Anderson, Kirstie N.; Pilsworth, Samantha; Sharples, Linda D.; Smith, Ian E.; Shneerson, John M.

2007-01-01

Study Objectives: To review the clinical and polysomnographic characteristics of idiopathic hypersomnia as well as the long-term response to treatment. Setting: The Respiratory Support and Sleep Centre at Papworth Hospital, Cambridge, UK. Patients and Design: A large database of more than 6000 patients with sleep disorders was reviewed. A retrospective study of the clinical and polysomnographic characteristics of 77 patients with idiopathic hypersomnia was performed. Comparison with a similar group of patients with narcolepsy was performed. The response to drug treatment was assessed in 61 patients over a mean follow-up of 3.8 years. Measurements and Results: Idiopathic hypersomnia was 60% as prevalent as narcolepsy. Comparison with a similar group of patients with narcolepsy showed that those with idiopathic hypersomnia were more likely to have prolonged unrefreshing daytime naps, a positive family history, increased slow-wave sleep, and a longer sleep latency on the Multiple Sleep Latency Test. The results of the Multiple Sleep Latency Test were not helpful in predicting disease severity or treatment response. The clinical features were heterogeneous and of variable severity. The majority of patients with idiopathic hypersomnia had symptoms that remained stable over many years, but 11% had spontaneous remission, which was never seen in narcolepsy. Two thirds of patients with idiopathic hypersomnolence had a sustained improvement in daytime somnolence with medication, although a third needed high doses or combinations of drugs. Conclusions: Idiopathic hypersomnolence has characteristic clinical and polysomnographic features but the prolonged latency on the Multiple Sleep Latency Test raises doubt about the validity of this test within the current diagnostic criteria. The disease often responds well to treatment and a substantial minority of patients appear to spontaneously improve. Citation: Anderson KN; Pilsworth S; Sharples LD; Smith IE; Shneerson JM. Idiopathic

Infrared radiation increases skin damage induced by other wavelengths in solar urticaria.

PubMed

de Gálvez, María Victoria; Aguilera, José; Sánchez-Roldán, Cristina; Herrera-Ceballos, Enrique

2016-09-01

Photodermatoses are typically investigated by analyzing the individual or combined effects of ultraviolet A (UVA), ultraviolet B (UVB), and visible light using light sources that simulate portions of the solar spectrum. Infrared radiation (IRR), however, accounts for 53% of incident solar radiation, but its effects are not taken into account in standard phototest protocols. The aim was to analyze the effects of IRR, alone and combined with UVA and visible light on solar urticaria lesions, with a distinction between infrared A (IRA) and infrared B (IRB). We performed standard phototests with UVA and visible light in four patients with solar urticaria and also tested the effects after blocking IRB with a water filter. To analyze the direct effect of IRR, we performed phototests with IRA and IRB. Initial standard phototests that were all positive found the induction of erythema and whealing, while when IRR was blocked from the UVA and visible light sources, three of the patients developed no lesions, while the fourth developed a very small wheal. These results suggest that IRR has the potential to produce and exacerbate lesions caused by other types of radiation. Consideration of these effects during phototesting could help prevent diagnostic errors. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

[Insight into the training of patients with idiopathic inflammatory myopathy].

PubMed

Váncsa, Andrea

2016-09-01

Using current recommended treatment, a majority of patients with idiopathic inflammatory myopathy develop muscle impairment and poor health. Beneficial effects of exercise have been reported on muscle performance, aerobic capacity and health in chronic polymyositis and dermatomyositis, as well as in active disease and inclusion body myositis to some extent. Importantly, randomized controlled trials indicate that improved health and decreased clinical disease activity could be mediated through increased aerobic capacity. Recently, reports seeking pathomechanisms of the underlying effects of exercise on skeletal muscle indicate increased aerobic capacity (i.e. increased mitochondrial capacity and capillary density, reduced lactate levels), activation of genes of aerobic phenotype and muscle growth programs and down regulation of genes related to inflammation. Exercise contributes to both systemic and within-muscle adaptations demonstrating that it is fundamental for improving muscle performance and health in patients with idiopathic inflammatory myopathy. There is a need for randomized controlled trials to study the effects of exercise in patients with active disease and inclusion body myositis. Orv. Hetil., 2016, 157(39), 1557-1562.

Prediction of the Efficacy of Antihistamines in Chronic Spontaneous Urticaria Based on Initial Suppression of the Histamine- Induced Wheal.

PubMed

Sánchez, J; Zakzuk, J; Cardona, R

2016-01-01

Antihistamines are the first line of treatment for chronic spontaneous urticaria. However, there is no effective method to predict whether an antihistamine will have a beneficial clinical effect or not. To assess whether the change in histamine-induced wheal and flare measurements 24 hours after administration of antihistamine can predict the efficacy of treatment. We performed a multicenter, triple-blind, randomized study. Patients received a daily oral dose of cetirizine, fexofenadine, bilastine, desloratadine, or ebastine over 8 weeks. After 4 weeks, a higher dose of antihistamine was administered to patients who did not experience a clinical response. A histamine skin prick test was carried out at baseline and 24 hours after the first dose of antihistamine. Disease severity (Urticaria Activity Score [UAS]), response to the histamine skin prick test, and impact on the patient's quality of life (Dermatology Life Quality Index [DLQI]) were determined every 2 weeks. The study population comprised 150 patients (30 per group) and 30 controls. Twenty-four hours after administration of antihistamine, inhibition of the histamine wheal by >75% was significantly associated with better UAS and DLQI scores. The safety and efficacy of the 5 antihistamines were similar. After updosing, rates of disease control (DLQI score <5) increased from 58.7% to 76.7%. Measurement of the histamine-induced wheal can predict which patients will have a strong clinical response to antihistamines but has limited utility for identifying nonresponders. The clinical significance of these data could be relevant in the search for new urticaria treatment regimens.

9 CFR 311.22 - Hogs affected with urticaria, tinea tonsurans, demodex follicurlorum, or erythema.

Code of Federal Regulations, 2010 CFR

2010-01-01

... 9 Animals and Animal Products 2 2010-01-01 2010-01-01 false Hogs affected with urticaria, tinea tonsurans, demodex follicurlorum, or erythema. 311.22 Section 311.22 Animals and Animal Products FOOD SAFETY... OTHERWISE ADULTERATED CARCASSES AND PARTS § 311.22 Hogs affected with urticaria, tinea tonsurans, demodex...

Acupuncture and a gluten-free diet relieve urticaria and eczema in a case of undiagnosed dermatitis herpetiformis and atypical or extraintestinal celiac disease: a case report.

PubMed

Ohlsen, Bahia A

2011-12-01

The purpose of this case report is to describe the use of acupuncture and a gluten-free diet (GFD) for urticaria and severe eczema in a patient with undiagnosed dermatitis herpetiformis and atypical or extraintestinal celiac disease. A 48-year-old woman presented with intense urticaria, eczema, worsening heartburn, chronic constipation, headaches, and an intense feeling of heat for 4 months. Results of punch biopsies of the skin lesions and laboratory tests were inconclusive. After the acupuncture sessions reported here ended, human leukocyte antigen blood typing revealed celiac disease and dermatitis herpetiformis-associated human leukocyte antigen DQ-8. Results of an endoscopy and colonoscopy were negative. The patient received 3 acupuncture treatments a week for 12 weeks. The patient's symptoms began in March 2008. She began using topical and oral steroids and felt that her symptoms were not responding. Acupuncture began in July 2008. At the end of the first 12 treatments, during which she was using topical and oral steroids, the urticaria and constipation resolved completely; and she had temporary relief from the heartburn. It is thought that the urticaria and constipation resolved because of the acupuncture as that was the only change. At the end of the second 12 treatments, during which time she had started Optifast, a GFD, the heartburn, headache, and eczema resolved. At the end of the third 12 treatments, all her symptoms remained resolved. Steroid treatment was discontinued after the first 12 treatments. Acupuncture and diet changes appeared to provide relief from the urticaria and eczema of dermatitis herpetiformis beyond that obtained by traditional treatment of a GFD alone.

Outcome of facial physiotherapy in patients with prolonged idiopathic facial palsy.

PubMed

Watson, G J; Glover, S; Allen, S; Irving, R M

2015-04-01

This study investigated whether patients who remain symptomatic more than a year following idiopathic facial paralysis gain benefit from tailored facial physiotherapy. A two-year retrospective review was conducted of all symptomatic patients. Data collected included: age, gender, duration of symptoms, Sunnybrook facial grading system scores pre-treatment and at last visit, and duration of treatment. The study comprised 22 patients (with a mean age of 50.5 years (range, 22-75 years)) who had been symptomatic for more than a year following idiopathic facial paralysis. The mean duration of symptoms was 45 months (range, 12-240 months). The mean duration of follow up was 10.4 months (range, 2-36 months). Prior to treatment, the mean Sunnybrook facial grading system score was 59 (standard deviation = 3.5); this had increased to 83 (standard deviation = 2.7) at the last visit, with an average improvement in score of 23 (standard deviation = 2.9). This increase was significant (p < 0.001). Tailored facial therapy can improve facial grading scores in patients who remain symptomatic for prolonged periods.

Periprosthetic Tissue Loss in Patients With Idiopathic Vitreous Inflammation After the Boston Keratoprosthesis.

PubMed

Grassi, Christina M; Cruzat, Andrea; Taniguchi, Elise V; Crnej, Alja; Colby, Kathryn A; Dohlman, Claes H; Chodosh, James

2015-11-01

Idiopathic vitritis is a poorly understood complication after Boston keratoprosthesis surgery with unclear etiology. We sought to determine whether an association exists between periprosthetic corneal tissue loss and the development of idiopathic vitritis in keratoprosthesis recipients. Thirteen Boston type I keratoprosthesis recipient eyes with a history of idiopathic vitritis and 34 type I keratoprosthesis recipient eyes with no history of idiopathic vitritis underwent anterior segment optical coherence tomography (AS-OCT) at a median time postoperatively of 2.4 years versus 1.9 years (range, 0.5-14.2 vs. 0.1-13.6 years), respectively. Areas of corneal graft tissue loss ("gaps") around the keratoprosthesis stem were identified and analyzed by 2 masked observers. The difference in the presence, number, and size of gaps was compared between cases and controls. A periprosthetic gap was identified more commonly in idiopathic vitritis cases than in controls on AS-OCT (11/13, 86% vs. 11/34, 33.3%, P < 0.001). The number of gaps between cases and controls was also significantly different (2.6 ± 1.6 vs. 0.5 ± 0.8, P < 0.001), but not the estimated gap area (0.056 ± 0.049 mm² vs. 0.039 ± 0.025 mm², P = 0.22). A significantly higher proportion of keratoprosthesis recipient eyes with idiopathic vitritis had corneal tissue loss around the keratoprosthesis stem than did controls. Tissue loss could serve as an entry point for debris or bacterial components, triggering idiopathic vitritis. Our study underscores the utility of AS-OCT imaging in the postoperative management of keratoprosthesis patients.

High prevalence of myocardial monoclonal antimyosin antibody uptake in patients with chronic idiopathic dilated cardiomyopathy

DOE Office of Scientific and Technical Information (OSTI.GOV)

Obrador, D.; Ballester, M.; Carrio, I.

1989-05-01

Monoclonal antimyosin antibody studies were undertaken to assess the presence of myocardial uptake in patients with chronic idiopathic dilated cardiomyopathy. Three groups were studied: 17 patients with chronic (greater than 12 months) idiopathic dilated cardiomyopathy, 12 patients with a large, poorly contracting left ventricle not due to dilated cardiomyopathy (control patients) and 8 normal individuals. The patients in the cardiomyopathy and control groups showed a similar degree of clinical and functional impairment. Imaging was undertaken 48 h after antimyosin injection. The heart/lung ratio of antimyosin uptake was used to assess the results. The mean ratio in the cardiomyopathy group wasmore » 1.83 +/- 0.36 (range 1.40 to 2.80), a value significantly higher than that obtained in the control patients without cardiomyopathy (mean 1.46 +/- 0.04, range 1.38 to 1.50) or normal subjects (mean 1.46 +/- 0.13, range 1.31 to 1.6) (p less than 0.01). No difference in the ratio was noted between the normal subjects and control patients. Abnormal antimyosin uptake was seen in 12 (70%) of the 17 patients with cardiomyopathy and in only 1 (8%) of the 12 control patients. Positive monoclonal antimyosin antibody studies are highly prevalent in chronic idiopathic dilated cardiomyopathy.« less

Determinants of 6-minute walk distance in patients with idiopathic pulmonary fibrosis undergoing lung transplant evaluation.

PubMed

Porteous, Mary K; Rivera-Lebron, Belinda N; Kreider, Maryl; Lee, James; Kawut, Steven M

2016-03-01

Little is known about the physiologic determinants of 6-minute walk distance in idiopathic pulmonary fibrosis. We investigated the demographic, pulmonary function, echocardiographic, and hemodynamic determinants of 6-minute walk distance in patients with idiopathic pulmonary fibrosis evaluated for lung transplantation. We performed a cross-sectional analysis of 130 patients with idiopathic pulmonary fibrosis who completed a lung transplantation evaluation at the Hospital of the University of Pennsylvania between 2005 and 2010. Multivariable linear regression analysis was used to generate an explanatory model for 6-minute walk distance. After adjustment for age, sex, race, height, and weight, the presence of right ventricular dilation was associated with a decrease of 50.9 m (95% confidence interval [CI], 8.4-93.3) in 6-minute walk distance ([Formula: see text]). For each 200-mL reduction in forced vital capacity, the walk distance decreased by 15.0 m (95% CI, 9.0-21.1; [Formula: see text]). For every increase of 1 Wood unit in pulmonary vascular resistance, the walk distance decreased by 17.3 m (95% CI, 5.1-29.5; [Formula: see text]). Six-minute walk distance in idiopathic pulmonary fibrosis depends in part on circulatory impairment and the degree of restrictive lung disease. Future trials that target right ventricular morphology, pulmonary vascular resistance, and forced vital capacity may potentially improve exercise capacity in patients with idiopathic pulmonary fibrosis.

Impact of Glycemic Control on Morbidity and Mortality in Adult Idiopathic Scoliosis Patients Undergoing Spinal Fusion.

PubMed

Shin, John I; Phan, Kevin; Kothari, Parth; Kim, Jun S; Guzman, Javier Z; Cho, Samuel K

2017-08-01

This is a retrospective analysis of administrative database. To elucidate the effect of glycemic control on surgical outcomes of middle-aged and elderly idiopathic scoliosis patients undergoing spinal fusion surgery. Diabetes mellitus (DM) is a condition thought to adversely affect outcomes of spine surgery. However, no study has stratified glycemic control levels and their impact on outcome for idiopathic scoliosis patients receiving a spinal fusion surgery. Previous studies may have reported higher than true rates of complications for controlled diabetic patients, who are the majority of diabetic patients. The Nationwide Inpatient Sample was queried from years 2002 to 2011. We extracted idiopathic scoliosis patients older than 45 years of age that received spinal fusion and analyzed complications and outcomes variables among 3 cohorts: nondiabetic patients, controlled diabetics, and uncontrolled diabetics. Multivariate analyses were used to assess whether glycemic control was a risk factor for adverse postoperative outcomes. Controlled diabetics had significantly increased rates of acute renal failure (ARF), while uncontrolled diabetics had significantly increased rates of acute postoperative hemorrhage. In multivariate analyses controlling for patient factors and comorbidities, controlled DM was found to be an independent predictor of ARF [odds ratio (OR), 1.863; 95% confidence interval (CI), 1.346-2.579; P=0.0002), and uncontrolled DM was found to be a significant risk factor for acute postoperative hemorrhage (OR, 2.182; 95% CI, 1.192-3.997; P=0.0115), ARF (OR, 4.839; 95% CI, 1.748-13.392; P=0.0024), deep vein thrombosis (OR, 5.825; 95% CI, 1.329-25.522, P=0.0194) and in-patient mortality (OR, 8.889; 95% CI, 1.001-78.945; P=0.0499). Controlled DM was found to be a risk factor for ARF in adult idiopathic scoliosis patients undergoing spinal fusion surgery, while uncontrolled DM was shown to be a risk factor for postoperative hemorrhage, ARF, deep vein

Varicella Zoster Virus Necrotizing Retinitis in Two Patients with Idiopathic CD4 Lymphocytopenia.

PubMed

Gupta, Meenakashi; Jardeleza, Maria Stephanie R; Kim, Ivana; Durand, Marlene L; Kim, Leo; Lobo, Ann-Marie

2016-10-01

Progressive outer retinal necrosis (PORN) associated with varicella zoster virus (VZV) is usually diagnosed in HIV positive or immunosuppressed patients. We report two cases of immunocompetent patients with necrotizing viral retinitis found to have idiopathic CD4 lymphocytopenia. Clinical presentation, examination, imaging, and laboratory testing of two patients with VZV retinitis are presented. An HIV negative patient with history of herpes zoster presented with rapid loss of vision and examination consistent with PORN. PCR testing confirmed VZV. Lymphocytopenia was noted with a CD4 count of 25/mm(3). A second HIV negative patient presented with blurred vision and lid swelling and was found to have peripheral VZV retinitis confirmed by PCR. Laboratory workup revealed lymphocytopenia with a CD4 count of 133/mm(3). VZV necrotizing retinitis classic for PORN can occur in HIV negative patients. Idiopathic CD4 lymphocytopenia should be considered healthy patients who develop ocular infections seen in the immunocompromised.

Psychological Evaluations of Patients Operated for Idiopathic Scoliosis by the Harrington Method.

ERIC Educational Resources Information Center

Orvomaa, E.

1998-01-01

A study of 204 patients operated on for idiopathic scoliosis by the Harrington method between 1970 and 1975 found that patients were content with their lives, tended to form families later in life, and had fewer sexual relationships. The patients felt their illness had mostly influenced their participation in work and in physical activities.…

Idiopathic hypersomnia: a study of 77 cases.

PubMed

Anderson, Kirstie N; Pilsworth, Samantha; Sharples, Linda D; Smith, Ian E; Shneerson, John M

2007-10-01

To review the clinical and polysomnographic characteristics of idiopathic hypersomnia as well as the long-term response to treatment. The Respiratory Support and Sleep Centre at Papworth Hospital, Cambridge, UK. A large database of more than 6000 patients with sleep disorders was reviewed. A retrospective study of the clinical and polysomnographic characteristics of 77 patients with idiopathic hypersomnia was performed. Comparison with a similar group of patients with narcolepsy was performed. The response to drug treatment was assessed in 61 patients over a mean follow-up of 3.8 years. Idiopathic hypersomnia was 60% as prevalent as narcolepsy. Comparison with a similar group of patients with narcolepsy showed that those with idiopathic hypersomnia were more likely to have prolonged unrefreshing daytime naps, a positive family history, increased slow-wave sleep, and a longer sleep latency on the Multiple Sleep Latency Test. The results of the Multiple Sleep Latency Test were not helpful in predicting disease severity or treatment response. The clinical features were heterogeneous and of variable severity. The majority of patients with idiopathic hypersomnia had symptoms that remained stable over many years, but 11% had spontaneous remission, which was never seen in narcolepsy. Two thirds of patients with idiopathic hypersomnolence had a sustained improvement in daytime somnolence with medication, although a third needed high doses or combinations of drugs. Idiopathic hypersomnolence has characteristic clinical and polysomnographic features but the prolonged latency on the Multiple Sleep Latency Test raises doubt about the validity of this test within the current diagnostic criteria. The disease often responds well to treatment and a substantial minority of patients appear to spontaneously improve.

[Effect on chronic urticaria and serum IL-4 and IgE in the patients treated with moving cupping therapy and autohemotherapy with acupoint inection].

PubMed

Zhang, Wan-Rong; Lang, Na

2014-12-01

To explore the clinical efficacy and effect mechanism on chronic urticaria treated with moving cupping therapy combined with autohemotherapy with acupaint injection for desensitization at acupoints. One hundred and four patients of chronic urticaria were randomized into a comprehensive therapy group and a medication group, 52 cases in each one. In the comprehensive therapy group, the moving cupping therapy along the governor vessel and bladder meridian of foot-taiyang was applied combined with autohemotherapy with acupaint injection for desensitization at acupoints. The self-venous blood was injected at bilateral Quchi (LI 11) and Zusanli (ST 36), 1 mL at each acupoint, once every 3 days. In the medication group, cetirizine tablets, 10 mg were prescribed for oral administration, once every day, and the compound dexamethasone acetate cream was used externally, once to twice a day. The clinical efficacy: was observed in 30 days of treatment in the two groups. The changes of serum interleukin 4 (IL-4) and immunoglobulin E (Ig E) before and after treatment were observed. The recurrence rate was compared between the two groups in 3 months after treatment. The cured and markedly effective rate was 90.4% (47/52) in the comprehensive therapy group, which was higher obviously than 78.8% (41/52, P < 0.05) in the medication group. The levels of serum IL-4 and IgE were reduced in the patients of the two groups, indicating the significant difference in comparison before and after treatment (all P < 0.01) and the reducing degree in the comprehensive therapy group was much more significant than that in the medicine group (both P < 0.01). The recurrence rate was 19. 1% (9/47) in the comprehensive therapy group in the 3-month follow-up after treatment, and apparently lower than 51.2% (21/41, P < 0.01). The moving cupping therapy combined with autohemotherapy with acupaint injection for desensitization at acupoints achieves the better efficacy on chronic urticaria compared with the

Aspirin Desensitization in Patients With Coronary Artery Disease: Results of the Multicenter ADAPTED Registry (Aspirin Desensitization in Patients With Coronary Artery Disease).

PubMed

Rossini, Roberta; Iorio, Annamaria; Pozzi, Roberto; Bianco, Matteo; Musumeci, Giuseppe; Leonardi, Sergio; Lettieri, Corrado; Bossi, Irene; Colombo, Paola; Rigattieri, Stefano; Dossena, Cinzia; Anzuini, Angelo; Capodanno, Davide; Senni, Michele; Angiolillo, Dominick J

2017-02-01

There are limited data on aspirin (ASA) desensitization for patients with coronary artery disease. The aim of the present study was to assess the safety and efficacy of a standard rapid desensitization protocol in patients with ASA sensitivity undergoing coronary angiography. This is a prospective, multicenter, observational study including 7 Italian centers including patients with a history of ASA sensitivity undergoing coronary angiography with intent to undergo percutaneous coronary intervention. A total of 330 patients with history of ASA sensitivity with known/suspected stable coronary artery disease or presenting with an acute coronary syndrome, including ST-segment-elevation myocardial infarction were enrolled. Adverse effects to aspirin included urticaria (n=177, 53.6%), angioedema (n=69, 20.9%), asthma (n=65, 19.7%), and anaphylactic reaction (n=19, 5.8%). Among patients with urticaria/angioedema, 13 patients (3.9%) had a history of idiopathic chronic urticaria. All patients underwent a rapid ASA (5.5 hours) desensitization procedure. The desensitization procedure was performed before cardiac catheterization in all patients, except for those (n=78, 23.6%) presenting with ST-segment-elevation myocardial infarction who underwent the desensitization after primary percutaneous coronary intervention. Percutaneous coronary intervention was performed in 235 patients (71%) of the overall study population. The desensitization procedure was successful in 315 patients (95.4%) and in all patients with a history of anaphylactic reaction. Among the 15 patients (4.6%) who did not successfully respond to the desensitization protocol, adverse reactions were minor and responded to treatment with corticosteroids and antihistamines. Among patients with successful in-hospital ASA desensitization, 253 patients (80.3%) continued ASA for at least 12 months. Discontinuation of ASA in the 62 patients (19.7%) who had responded to the desensitization protocol was because of medical

Allergy and parasites reevaluated: wide-scale induction of chronic urticaria by the ubiquitous fish-nematode Anisakis simplex in an endemic region.

PubMed

Daschner, A; Vega de la Osada, F; Pascual, C Y

2005-01-01

The ubiquitous fish-nematode Anisakis simplex produces acute urticaria or angioedema in the course of gastro-allergic anisakiasis. We studied the relationship between this nematode and chronic urticaria (CU), as well as the clinical usefulness of measuring specific IgG4 in A. simplex-sensitized patients with CU. First, the prevalence of sensitization to A. simplex was estimated in 135 consecutive CU patients and the result was compared with known data about sensitization in a healthy population. Then, clinical response to a 2-month diet without fish was analyzed in 76 CU patients. The improvement rate in patients with and without sensitization to A. simplex was compared. Finally, the improvement rate, other clinical data and specific immunoglobulins in sensitized patients with and without detectable specific IgG4 were compared. a) The A. simplex sensitization rate in CU patients was 52.6 % compared with a known prevalence of between 16 and 20 % in our region. b) Of 65 sensitized patients, 52 experienced clinical improvement after the diet compared with only three of 11 patients without sensitization to A. simplex (p = 0.001). c) Of 43 patients with detectable specific IgG4, 38 showed clinical improvement compared with only 14 of 22 patients without detectable IgG4 (p = 0.02). Eight of nine patients with previous fish-associated cutaneous symptoms had detectable specific IgG4 compared with 15 of 32 patients who reported no previous fish-associated symptoms or acute urticaria (p = 0.03). Our results indicate that A. simplex is a possibly widespread etiologic agent able to induce CU. This parasite model constitutes the first report that associates an infectious agent with CU on a large scale. The detection of IgG4 antibodies reflects a previous acute parasitic infection and a temporary diet without fish improves symptoms in most patients with detectable specific IgG4.

A cohort study of the relationship between anger and chronic spontaneous urticaria.

PubMed

Altınöz, Ali Ercan; Taşkıntuna, Nilgün; Altınöz, Sengül Tosun; Ceran, Selvi

2014-09-01

Anger plays a major role in psychodermatological diseases. Researchers have reported that anger and other psychological factors play a role in the etiology of chronic urticaria. This study aimed to examine symptoms of anger, anger-related behavioral patterns, thoughts associated with anger, situations that cause anger and experiences of interpersonal anger in patients with chronic spontaneous urticaria (CSU). The authors hypothesized that patients with CSU react to more situations with anger and experience more anger symptoms as compared to alopecia areata (AA) patients and healthy controls. The cohort study population consisted of literate adult patients aged <65 years that were diagnosed with CSU at the outpatient dermatology clinics of Başkent and Gazi University, Ankara, Turkey, between September 2011 and October 2012. The first control group included individuals without any physical or mental disorders and the second one included literate adult patients diagnosed with AA. The patients and controls were matched according to age, gender, and level of education. A sociodemographic data form, and the Hospital Anxiety and Depression Scale and Multi-Dimensional Anger Inventory were administered to the participants. Data were analyzed using SPSS v.17.0 for Windows. The primary outcome was to determine whether there was a relationship between anger and CSU. The CSU group consisted of 30 participants; AA group consisted of 30 participants; and the healthy group consisted of 39 participants. Anxiety and depression scores in the CSU group were significantly higher than those in the healthy control group. Symptoms of anger, situations that cause anger, and thoughts associated with anger were significantly more common in the CSU group compared to AA group and healthy group. More of the CSU patients were observed to respond with excessive anger to most situations, to have high levels of anxiety anger and passive aggressive interpersonal relationships.

Conservative treatment of idiopathic spontaneous pneumoperitoneum in a bedridden patient: a case report.

PubMed

Tanaka, Ryo; Kameyama, Hitoshi; Nagahashi, Masayuki; Kanda, Tatsuo; Ichikawa, Hiroshi; Hanyu, Takaaki; Ishikawa, Takashi; Kobayashi, Takashi; Sakata, Jun; Kosugi, Shin-Ichi; Wakai, Toshifumi

2015-01-01

Idiopathic spontaneous pneumoperitoneum is a rare condition that is characterized by intraperitoneal gas for which no clear etiology has been identified. We report here a case of idiopathic spontaneous pneumoperitoneum, which was successfully managed by conservative treatment. A 77-year-old woman who was bedridden with speech disability as a sequela of brain hemorrhage presented at our hospital with a 1-day history of abdominal distention. On physical examination, she had stable vital signs and slight epigastric tenderness on deep palpation without any other signs of peritonitis. A chest radiograph and computed tomography showed that a large amount of free gas extended into the upper abdominal cavity. Esophagogastroduodenoscopy revealed no perforation of the upper gastrointestinal tract. The patient was diagnosed with idiopathic spontaneous pneumoperitoneum, and conservative treatment was selected. The abdominal distension rapidly disappeared, and the patient resumed oral intake on the 5th hospital day without deterioration of symptoms. Knowledge of this rare disease and accurate diagnosis with findings of clinical imaging might contribute towards refraining from unnecessary laparotomy.

A case of severe refractory chronic urticaria: a novel method for evaluation and treatment.

PubMed

Otto, Hans F; Calabria, Christopher W

2009-01-01

With cholinergic urticaria (ChU), the ultimate diagnosis often depends on the demonstration of characteristic urticaria by appropriate provocation. Several treatment options may be helpful but traditional options (antihistamines, leukotriene inhibitors, and immunosuppressives) may be exhausted by the refractory ChU patient. Here, we describe such a case. Demonstration of immediate hypersensitivity to autologous sweat skin testing (ASwST) may provide a rationale for use of omalizumab (Xolair, Genentech Novartis, South San Francisco, CA). Patients with severe ChU may have difficulty producing sufficient quantities of sweat for ASwST given that the very effort that produces the sample exacerbates ChU. Generation of sweat by iontophoresis with pilocarpine nitrate can be performed at many large medical centers. The procedure is simple, safe, and produces varying amounts of sweat depending on the individual. This sweat can then be used for ASwST. Our patient had a positive ASwST with appropriate positive and negative controls. Our testing methods were validated by negative ASwST, saline control, and positive histamine control in a nonatopic, nonurticarial control patient. By the patient's second injection of omalizumab, her quality of life score was significantly improved, as were her daily medication scores and exercise tolerance. We describe the first case of a patient with severe refractory ChU who had a positive ASwST by a novel collection method who has been successfully treated with omalizumab. We present a novel tool for the evaluation and demonstration of sweat-specific IgE in ChU patients who are unable to provide sweat by more traditional means.

Risk factors for poor visual outcome in patients with idiopathic intracranial hypertension.

PubMed

Wall, Michael; Falardeau, Julie; Fletcher, William A; Granadier, Robert J; Lam, Byron L; Longmuir, Reid A; Patel, Anil D; Bruce, Beau B; He, Hua; McDermott, Michael P

2015-09-01

Determine potential risk factors for progressive visual field loss in the Idiopathic Intracranial Hypertension Treatment Trial, a randomized placebo-controlled trial of acetazolamide in patients with idiopathic intracranial hypertension and mild visual loss concurrently receiving a low sodium, weight reduction diet. Logistic regression and classification tree analyses were used to evaluate potential risk factors for protocol-defined treatment failure (>2 dB perimetric mean deviation [PMD] change in patients with baseline PMD -2 to -3.5 dB or >3 dB PMD change with baseline PMD -3.5 to -7 dB). Seven participants (6 on diet plus placebo) met criteria for treatment failure. The odds ratio for patients with grades III to V papilledema vs those with grades I and II was 8.66 (95% confidence interval [CI] 1.65-∞, p = 0.025). A 1-unit decrease in the number of letters correct on the ETDRS (Early Treatment Diabetic Retinopathy Study) chart at baseline was associated with an increase in the odds of treatment failure by a factor of 1.16 (95% CI 1.04-1.30, p = 0.005). Compared with female participants, the odds ratio for male participants was 26.21 (95% CI 1.61-433.00, p = 0.02). The odds of treatment failure were 10.59 times higher (95% CI 1.63-116.83, p = 0.010) for patients with >30 transient visual obscurations per month vs those with ≤30 per month. Male patients, those with high-grade papilledema, and those with decreased visual acuity at baseline were more likely to experience treatment failure. All but one of these patients were treated with diet alone. These patients should be monitored closely and be considered for aggressive treatment of their idiopathic intracranial hypertension. © 2015 American Academy of Neurology.

Genetic variability of prostaglandin E2 receptor subtype EP4 gene in aspirin-intolerant chronic urticaria.

PubMed

Palikhe, Nami Shrestha; Sin, Hye Jung; Kim, Seung Hyun; Sin, Hyun Jung; Hwang, Eui Kyung; Ye, Young Min; Park, Hae-Sim

2012-08-01

Prostaglandin E2 receptor subtype EP4 (PTGER4) is one of the four subtypes of receptors for prostaglandin E2 (PGE2). Overproduction of cysteinyl leukotriene in mast cells may be related with suppression of PGE2 in patients with aspirin hypersensitivity. Considering the association of PTGER4 in mast cells, urticaria- and aspirin-related disease, we hypothesized the genetic variability of PTGER4 may be associated with aspirin-intolerant chronic urticaria (AICU). The case-control study was performed in 141 with AICU, 153 with aspirin-tolerant chronic urticaria (ATCU) and 174 with normal controls (NCs). PTGER4 promoter single-nucleotide polymorphism was genotyped using a primer extension method with the SNAPshot ddNTP primer extension kit. The functional variability of PTGER4 promoter polymorphism was carried out by dual-luciferase system and electrophoretic mobility shift assay (EMSA) in human mast cells (HMC-1). Furthermore, the effect of aspirin was performed for PTGER4 mRNA expression using real-time PCR, and PGE2 production was checked in HMC-1 cells using ELISA. AICU patients carrying GG genotype at -1254 G>A showed significantly higher frequency compared with NC (P=0.032). Similarly, the minor allele frequency, G allele was significantly higher in AICU compared with NC (P=0.031). In vitro functional study demonstrated that the -1254 G allele had lower luciferase activity (P<0.001) in HMC-1 cells. EMSA finding showed that PTGER4 -1254 G produced a specific band. Significantly decreased PTGER4 expression (P=0.008) and PGE2 production by aspirin exposure was confirmed in in vitro HMC cell line model (P=0.001). The PTGER4 -1254 G allele demonstrated a higher frequency in AICU patients and lower promoter activity with decreased expression of PTGER4 and contributes to the development of AICU.

Efficacy of high-dose intravenous immunoglobulins in two patients with idiopathic recurrent pericarditis refractory to previous immunosuppressive treatment.

PubMed

Tona, Francesco; Bellotto, Fabio; Laveder, Francesco; Meneghin, Alessia; Sinagra, Gianfranco; Marcolongo, Renzo

2003-01-01

Although idiopathic acute pericarditis is usually a self-limiting disease, in many patients it may recur over a period of months or years. Even if some evidence seems to suggest the possible role of a deranged immune reactivity in the pathogenesis of idiopathic recurrent pericarditis, the etiology of the disease is still unknown. Furthermore, while some trial data confirm the usefulness of colchicine, its medical treatment is not yet clearly established. We here report the clinical history of 2 patients with idiopathic recurrent pericarditis resistant to prednisone, colchicine and other immunosuppressive drugs, who have been successfully treated with high-dose intravenous immunoglobulins.

Unipedal Diagnostic Lymphangiography Followed by Sequential CT Examinations in Patients With Idiopathic Chyluria: A Retrospective Study.

PubMed

Dong, Jian; Xin, Jianfeng; Shen, Wenbin; Chen, Xiaobai; Wen, Tingguo; Zhang, Chunyan; Wang, Rengui

2018-04-01

The objective of our study was to investigate the clinical value of diagnostic lymphangiography followed by sequential CT examinations in patients with idiopathic chyluria. Thirty-six patients with idiopathic chyluria underwent unipedal diagnostic lymphangiography and then underwent sequential CT examinations. The examinations were reviewed separately by two radiologists. Abnormal distribution of contrast medium, lymphourinary leakages, and retrograde flow were noted, and the range and distribution of lymphatic vessel lesions were recorded. The stage of idiopathic chyluria based on CT findings and the stage based on clinical findings were compared. Therapeutic management and follow-up were recorded. Statistical analyses were performed. Compared with CT studies performed after lymphangiography, diagnostic lymphangiography showed a unique capability to depict lymphourinary leakages in three patients. Lymphourinary fistulas and abnormal dilated lymphatic vessels were found in and around kidney in all patients. CT depicted retrograde flow of lymph fluid in 47.2% of patients. The consistency in staging chyluria based on CT findings and clinical findings was fair (κ = 0.455). Twenty-nine patients underwent conservative therapy, and seven underwent surgery. Surgical therapy was superior to conservative management (no recurrence, 85.7% of patients who underwent surgery vs 62.1% of patients who underwent conservative therapy; p = 0.025). From assessing the drainage of contrast medium on unipedal diagnostic lymphangiography and the redistribution of contrast medium on sequential CT examinations, it is possible to detect the existence of lymphourinary fistulas, the precise location of lymphatic anomalies, the distribution of collateral lymphatic vessels, and hydrodynamic pressure abnormality in the lymph circulation in patients with idiopathic chyluria. CT staging of chyluria provides additional information that can be used to guide therapeutic management.

Perspective: Update on Idiopathic Intracranial Hypertension

PubMed Central

Bruce, Beau B.; Biousse, Valérie; Newman, Nancy J.

2011-01-01

Purpose Provide an update on various features of idiopathic intracranial hypertension. Design Perspective. Methods Selected articles on the epidemiology, clinical and imaging features, natural history, pathophysiology, and treatment of idiopathic intracranial hypertension were reviewed and interpreted in the context of the authors’ clinical and research experience. Results Idiopathic intracranial hypertension is primarily a disease of obese women of childbearing age, but it can affect patients of any weight, sex, and age. Although a relatively rare disorder, idiopathic intracranial hypertension’s associated costs in the U.S. entail hundreds of millions of dollars. Even following treatment, headaches are frequently persistent and may require the continued involvement of a neurologist. Quality of life reductions and depression are common among idiopathic intracranial hypertension patients. However, visual dysfunction, especially visual field abnormalities, represents the major morbidity of this disorder, and serial automated perimetry remains the primary mode of patient monitoring. Patients who are men, black, very obese, or anemic are at higher risk of visual loss. Vitamin A metabolism, adipose tissue as an actively secreting endocrine tissue, and cerebral venous abnormalities are areas of active study regarding idiopathic intracranial hypertension’s pathophysiology. Treatment studies show that lumbar puncture is a valuable treatment (in addition to its crucial diagnostic role) and that weight management is critical. However, open questions remain regarding the efficacy of acetazolamide, CSF diversion procedures, and cerebral venous stenting. Conclusions Many questions remain unanswered about idiopathic intracranial hypertension. Ongoing studies, especially an ongoing NIH-funded clinical trial of acetazolamide, should provide more insight into this important, yet poorly understood syndrome of isolated intracranial hypertension. PMID:21696699

Molecular analysis of HLA-DPB1 alleles in idiopathic systemic sclerosis patients and uranium miners with systemic sclerosis.

PubMed

Rihs, H P; Conrad, K; Mehlhorn, J; May-Taube, K; Welticke, B; Frank, K H; Baur, X

1996-03-01

According to clinical mainifestation and autoantibody pattern [anti-Scl-70, anti-centromere antibodies (ACAs)], systemic sclerosis is a connective tissue disease with heterogenous subgroups. PCR-sequence-specific-oligonucleotide typing was used to study the genetic association of HLA-DPB1 alleles in 54 patients with idiopathic systemic sclerosis, 26 uranium miners with systemic sclerosis and 70 unrelated healthy control subjects. Systemic sclerosis patients with and without former employment in mines were divided into two subgroups according to their scleroderma-typical autoantibody specificities--anti-Scl-70 positive and ACA positive--and third subgroup comprising the rest. Statistical analysis revealed a significantly increased frequency of DPB1*1301(p=0.0001, corrected p=0.011) in idiopathic anti-Scl-70-positive systemic sclerosis cases when compared with unexposed controls. In the same group, we observed an enhanced frequency of DPB1*0601 and *1701 alleles. Since these three alleles carry the information for a glutamic acid residue in position 69 of DPB1, we tested the association of this residue with anti-Scl-70 expression. A strong association between anti-Scl-70 positivity in idiopathic systemic sclerosis patients and amino acid residue 69 of DPB1 was observed when compared with anti-Scl-70-negative idiopathic systemic sclerosis patients (p=0.0009) or unrelated controls (p=0.0007). ACA expression was not associated with the presence of any DPB1 allele tested. The data show that anti-Scl-70 expression in idiopathic systemic sclerosis patients is linked with DPB1*1301 whereas anti-Scl-70-positive miners do not show such a DPB1 association. Futhermore, the data indicate that glutamate 69 of DPB1 might be involved in the susceptibility to idiopathic anti-Scl-70 expression.

Occupational IgE-mediated asthma, rhinoconjunctivitis, and contact urticaria caused by Easter lily (Lilium longiflorum) and tulip.

PubMed

Piirilä, P; Kanerva, L; Alanko, K; Estlander, T; Keskinen, H; Pajari-Backas, M; Tuppurainen, M

1999-03-01

We report on IgE-mediated asthma, rhinoconjunctivitis, and contact urticaria to two Liliaceae plants, tulip and Easter lily (Lilium longiflorum), diagnosed in a floral shop worker. Occupational asthma was diagnosed according to patient history, PEF monitoring, and a work-simulating provocation test. Flower-specific IgE was studied, and RAST inhibition tests were performed. Skin prick testing showed positive reactions to tulip, Easter lily, and chrysanthemum. Total IgE was 180 kU/I, and specific IgE to tulip was 2.6 and to Easter lily 6.5 kU/I. In the RAST-inhibition test, no cross-reactivity was found. Occupational asthma was diagnosed by peak flow monitoring at work and at home, as well as specific inhalation challenge with Easter lily, with an immediate 18% reduction in PEF. In addition, contact urticaria and conjunctivitis were diagnosed. After a 9-year follow-up without exposure to lilies, the skin prick tests to L. longiflorum and tulip were still positive, but the specific IgE had disappeared. A case of IgE-mediated occupational asthma, rhinoconjunctivitis, and contact urticaria caused by L. longiflorum and tulip is presented. RAST inhibition tests indicated concomitant sensitization to the two Liliaceae plants.

The Natural History of Idiopathic Scoliosis During Growth: A Meta-Analysis.

PubMed

Di Felice, Francesca; Zaina, Fabio; Donzelli, Sabrina; Negrini, Stefano

2018-05-01

The aim of the study was to provide a meta-analysis of current literature concerning the natural history of idiopathic scoliosis during growth. A comprehensive search of Medline, Embase, And Scopus databases was conducted up to November 2016. Eligible works were prospective or retrospective studies that enrolled patients with infantile idiopathic scoliosis, juvenile idiopathic scoliosis, or adolescent idiopathic scoliosis, followed up without any treatment from the time of detection. A meta-analysis for proportion was performed. The following studies were grouped per diagnosis: infantile idiopathic scoliosis, juvenile idiopathic scoliosis, and adolescent idiopathic scoliosis. Of the 1797 citations screened, we assessed 61 full-text articles and included 13 of these (2301 participants). Three studies included infantile idiopathic scoliosis patients (347 participants), five studies included a mixed population of juvenile idiopathic scoliosis and adolescent idiopathic scoliosis (1330 participants), and five studies included adolescent idiopathic scoliosis patients only (624 participants). The random pooled estimated progression rate was 49% (95% confidence interval = 1%-97%) for infantile idiopathic scoliosis, 49% in a mixed group of patients affected by juvenile idiopathic scoliosis or adolescent idiopathic scoliosis (95% confidence interval = 19%-79%), and 42% in adolescent idiopathic scoliosis (95% confidence interval = 11%-73%). During growth, idiopathic scoliosis tends to progress in a high percentage of cases. The progression rate varies according to the age at diagnosis, with infantile scoliosis being the most unpredictable. There are many confounders, such as age, Risser sign and baseline Cobb angles that were not consistent among studies, making the data very heterogeneous.

Triggers, risk factors and clinico-pathological features of urticaria in dogs - a prospective observational study of 24 cases.

PubMed

Rostaher, Ana; Hofer-Inteeworn, Natalie; Kümmerle-Fraune, Claudia; Fischer, Nina Maria; Favrot, Claude

2017-02-01

Urticaria and anaphylaxis are frequently encountered in veterinary practice, but little is known about the causes and relative frequencies of these reactions. This study was designed to improve current knowledge on the triggers, risk factors and clinico-pathological features of urticaria. Twenty four dogs with signs of urticaria with or without anaphylaxis. The study included dogs with cutaneous immediate-type hypersensitivity reactions. The cases were grouped by clinical severity into either an urticaria or an anaphylaxis group. All treatments and diagnostic tests (haematology, biochemical profile, allergy investigation) were recorded. A causality algorithm for urticaria and anaphylaxis (ALUA) was designed to determine the probability of the identified triggers and cofactors. Disease incidence, breed, age and gender predispositions were evaluated statistically. Sixteen of 24 urticaria cases were associated with anaphylaxis whilst 8 of 24 were confined to the skin. The annual hospital incidence was 0.12%. Females seemed to be over-represented (2.4:1) and most of the dog breeds were pure breed (22 of 24), with Rhodesian ridgeback, boxer, beagle, Jack Russell terrier, French bulldog and Vizslas over-represented. In addition to skin lesions, the most frequently and severely affected organ systems were the gastrointestinal and cardiovascular systems. The predominant blood abnormalities were elevated lipase and alanine aminotransferase values. Insects, food and drugs were the most commonly identified triggers. To the best of our knowledge, this is the first study describing the trigger factors and clinico-pathological features of dogs with urticaria in veterinary medicine. Insects, food and drugs were the most frequently detected triggers. © 2016 ESVD and ACVD.

Posttraumatic stress disorder (PTSD) and the dermatology patient.

PubMed

Gupta, Madhulika A; Jarosz, Patricia; Gupta, Aditya K

Dermatologic symptoms can be associated with posttraumatic stress disorder (PTSD) in several situations: (1) as features of some core PTSD symptoms, such as intrusion symptoms manifesting as cutaneous sensory flashbacks, as autonomic arousal manifesting as night sweats and idiopathic urticaria, and as dissociation manifesting as numbness and dermatitis artefacta; (2) the cutaneous psychosomatic effects of emotional and physical neglect and sexual abuse (eg, infantile eczema, cutaneous self-injury, and body-focused repetitive behaviors such as trichotillomania and skin picking disorder) and eating disorders, which can have dermatologic effects; (3) the direct effect of physical or sexual abuse or catastrophic life events (eg, earthquakes) on the skin; and (4) as a result of significant alterations in hypothalamic-pituitary-adrenal and sympatho-adrenal medullary axes, which can affect neuroendocrine and immune functions, and can lead to exacerbations of stress-reactive inflammatory dermatoses such as psoriasis, chronic urticaria, and atopic dermatitis. Elevated levels of inflammatory biomarkers and impaired epidermal barrier function have been reported in situations involving sustained psychologic stress and sleep deprivation. Some PTSD patients show hypothalamic-pituitary-adrenal axis hyporesponsiveness and higher circulating T lymphocytes, which can exacerbate immune-mediated dermatologic disorders. PTSD should be considered an underlying factor in the chronic, recurrent, or treatment-resistant stress-reactive dermatoses and in patients with self-induced dermatoses. Copyright © 2017 Elsevier Inc. All rights reserved.

Efficacy and safety of bilastine in Japanese patients with chronic spontaneous urticaria: A multicenter, randomized, double-blind, placebo-controlled, parallel-group phase II/III study.

PubMed

Hide, Michihiro; Yagami, Akiko; Togawa, Michinori; Saito, Akihiro; Furue, Masutaka

2017-04-01

Bilastine, a novel non-sedating second-generation H 1 -antihistamine, has been widely used in the treatment of allergic rhinoconjunctivitis and urticaria with a recommended dose of 20 mg once daily in most European countries since 2010. We evaluated its efficacy and safety in Japanese patients with chronic spontaneous urticaria (CSU). We conducted a multicenter, randomized, double-blind, placebo-controlled phase II/III study (trial registration No. JapicCTI-142574). Patients (age, 18-74 years) were randomly assigned to receive bilastine 20 mg, 10 mg or placebo once daily for 2 weeks. The primary efficacy endpoint was the change from baseline (Day -3 to 0) in total symptom score (TSS) at 2 weeks (Day 8-14), consisting of the itch and rash scores. A total of 304 patients were randomly allocated to bilastine 20 mg (101 patients), bilastine 10 mg (100 patients), and placebo (103 patients). The changes in TSS at 2 weeks were significantly decreased by bilastine 20 mg than did placebo (p < 0.001), demonstrating the superiority of bilastine 20 mg. Bilastine 10 mg also showed a significant difference from placebo (p < 0.001). The TSS changes for the bilastine showed significant improvement from Day 1, and were maintained during the treatment period. The Dermatology Life Quality Index scores were also improved in bilastine than in placebo. The bilastine treatments were safe and well tolerated. Two-week treatment with bilastine (20 or 10 mg) once daily was effective and tolerable in Japanese patients with CSU, demonstrating an early onset of action. Copyright © 2016 Japanese Society of Allergology. Production and hosting by Elsevier B.V. All rights reserved.

[Acute blue urticaria following subcutaneous injection of patent blue dye].

PubMed

Hamelin, A; Vial-Dupuy, A; Lebrun-Vignes, B; Francès, C; Soria, A; Barete, S

2015-11-01

Patent blue (PB) is a lymphatic vessel dye commonly used in France for sentinel lymph node detection in breast cancer, and less frequently in melanoma, and which may induce hypersensitivity reactions. We report a case of acute blue urticaria occurring within minutes of PB injection. Ten minutes after PB injection for sentinel lymph node detection during breast cancer surgery, a 49-year-old woman developed generalised acute blue urticaria and eyelid angioedema without bronchospasm or haemodynamic disturbance, but requiring discontinuation of surgery. Skin testing using PB and the anaesthetics given were run 6 weeks after the episode and confirmed PB allergy. PB was formally contra-indicated. Immediate hypersensitivity reactions to PB have been reported for between 0.24 and 2.2% of procedures. Such reactions are on occasion severe, chiefly involving anaphylactic shock. Two mechanisms are probably associated: non-specific histamine release and/or an IgE-mediated mechanism. Skin tests are helpful in confirming the diagnosis of PB allergy. Blue acute urticaria is one of the clinical manifestations of immediate hypersensitivity reactions to patent blue dye. Skin tests must be performed 6 weeks after the reaction in order to confirm the diagnosis and formally contra-indicate this substance. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

The Prevalence and Characteristics of Primary Headache and Dream-Enacting Behaviour in Japanese Patients with Narcolepsy or Idiopathic Hypersomnia: A Multi-Centre Cross-Sectional Study.

PubMed

Suzuki, Keisuke; Miyamoto, Masayuki; Miyamoto, Tomoyuki; Inoue, Yuichi; Matsui, Kentaro; Nishida, Shingo; Hayashida, Kenichi; Usui, Akira; Ueki, Yoichiro; Nakamura, Masaki; Murata, Momoyo; Numao, Ayaka; Watanabe, Yuji; Suzuki, Shiho; Hirata, Koichi

2015-01-01

Because the prevalence and characteristics of primary headache have yet to be thoroughly studied in patients with hypersomnia disorders, including narcolepsy and idiopathic hypersomnia, we examined these parameters in the Japanese population. In a multicentre cross-sectional survey, among 576 consecutive outpatients with sleep disorders, 68 narcolepsy patients and 35 idiopathic hypersomnia patients were included. Additionally, 61 healthy control subjects participated. Semi-structured headache questionnaires were administered to all participants. The patients with narcolepsy (52.9%) and idiopathic hypersomnia (77.1%) more frequently experienced headache than the healthy controls (24.6%; p<0.0001). The prevalence rates were 23.5%, 41.2% and 4.9% for migraine (p<0.0001) and 16.2%, 23.5% and 14.8% (p = 0.58) for tension-type headache among the narcolepsy patients, the idiopathic hypersomnia patients and the control subjects, respectively. Those who experienced migraine more frequently experienced excessive daytime sleepiness, defined as an Epworth Sleepiness Scale score of ≥10, than those who did not experience headache among the patients with narcolepsy (93.8% vs. 65.6%, p = 0.040) and idiopathic hypersomnia (86.7% vs. 37.5%, p = 0.026). Dream-enacting behaviour (DEB), as evaluated by the rapid eye movement sleep disorders questionnaire, was more frequently observed in the narcolepsy patients than in the idiopathic hypersomnia patients and the control subjects. An increased DEB frequency was observed in the narcolepsy patients with migraines compared to those without headache. Migraines were frequently observed in patients with narcolepsy and idiopathic hypersomnia. DEB is a characteristic of narcolepsy patients. Further studies are required to assess the factors that contribute to migraines in narcolepsy and idiopathic hypersomnia patients.

Systemic photoprotection in solar urticaria with α-melanocyte-stimulating hormone analogue [Nle4-D-Phe7]-α-MSH.

PubMed

Haylett, A K; Nie, Z; Brownrigg, M; Taylor, R; Rhodes, L E

2011-02-01

Solar urticaria is a rare photosensitivity disorder demonstrating a range of action spectra, which can inflict a very large impact on life quality despite available treatments. Melanin broadly reduces skin penetration by ultraviolet-visible wavelengths, thus increased melanization may protect in solar urticaria. To examine quantitatively for impact of the potent α-melanocyte stimulating hormone analogue afamelanotide ([Nle(4)-D-Phe(7)]-α-MSH, Scenesse(®); Clinuvel Pharmaceuticals Ltd, Melbourne, Vic., Australia) on the solar urticaria response and skin melanization. Five patients with solar urticaria received a single dose of 16 mg subcutaneous afamelanotide implant in winter time. Melanin density was assessed spectrophotometrically from day 0 to day 60. Detailed monochromated light testing to geometric dose series (increment ) of wavelengths 300-600 nm was performed at 0, 30 and 60 days, with assessment of weal and flare area and minimum urticarial dose (MUD). Data were analysed by repeated-measures anova. Mean melanin density increased by day 7, peaked at day 15 and remained elevated at day 60 (P=0·03, 0·01, 0·02 vs. baseline, respectively). Baseline phototesting revealed action spectra of 320-400 (n=1), 320-500 (n=2), 300-600 (n=1) and 370-500 nm (n=1), and on afamelanotide mean rises in MUD of 1-12 and 1-3 dose increments were seen at the individual wavelengths tested, at 30 and 60 days, respectively. A significant fall in weal area occurred across responding wavelengths from 300 to 600 nm at 60 days postimplant (P=0·049 vs. baseline), accompanied by greater than twofold overall increase in MUD (P=0·058 vs. baseline). Melanization following afamelanotide is accompanied by reduction in solar urticaria response across a broad spectrum of wavelengths. Further study is warranted to assess clinical benefit under ambient conditions in summer. © 2011 The Authors. BJD © 2011 British Association of Dermatologists.

The contribution of the SPINK1 c.194+2T>C mutation to the clinical course of idiopathic chronic pancreatitis in Chinese patients.

PubMed

Sun, Chang; Liao, Zhuan; Jiang, Lili; Yang, Fu; Xue, Geng; Zhou, Qi; Chen, Ruiwen; Sun, Shuhan; Li, Zhaoshen

2013-01-01

Recent data suggest that the serine protease inhibitor Kazal type 1 (SPINK1) gene mutation is associated with idiopathic chronic pancreatitis. However, few studies have focused on the serine protease inhibitor Kazal type 1 c.194+2T>C mutation. Therefore, our goal was to study the prevalence and impact of serine protease inhibitor Kazal type 1 mutations on the clinical profile of idiopathic chronic pancreatitis patients in China. A retrospective-cohort study of 118 Chinese patients with idiopathic chronic pancreatitis was performed, and genetic tests were carried out to detect SPINK1 mutations. Subjects without pancreatitis were used as controls. In total, 118 idiopathic chronic pancreatitis patients and 100 control subjects were evaluated. The serine protease inhibitor Kazal type 1 c.194+2T>C variant was present in 44.9% of patients with idiopathic chronic pancreatitis. The frequency of diabetes in idiopathic chronic pancreatitis patients with the serine protease inhibitor Kazal type 1 c.194+2T>C mutation (39.6%) was higher than that of patients without the mutation (9.2%). The time to occurrence of diabetes mellitus after idiopathic chronic pancreatitis symptom onset is significantly influenced by the c.194+2T>C mutation (p<0.001). In addition, the mean age of diabetes onset in patients with the serine protease inhibitor Kazal type 1 c.194+2T>C mutation (38.33 ± 9.50) was significantly younger than that of patients without this mutation (49.67 ± 6.74). The presence of the serine protease inhibitor Kazal type 1 c.194+2T>C mutation seems to be associated with idiopathic chronic pancreatitis and could predispose individuals to pancreatic diabetes onset at an earlier age. Copyright © 2012 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

Intraspinal anomalies in early-onset idiopathic scoliosis.

PubMed

Pereira, E A C; Oxenham, M; Lam, K S

2017-06-01

In the United Kingdom, lower incidences of intraspinal abnormalities in patients with early onset idiopathic scoliosis have been observed than in studies in other countries. We aimed to determine the rates of these abnormalities in United Kingdom patients diagnosed with idiopathic scoliosis before the age of 11 years. This retrospective study of patients attending an urban scoliosis clinic identified 71 patients satisfying a criteria of: clinical diagnosis of idiopathic scoliosis; age of onset ten years and 11 months or less; MRI screening for intraspinal abnormalities. United Kingdom census data combined with patient referral data was used to calculate incidence. Mean age at diagnosis was six years with 39 right-sided and 32 left-sided curves. Four patients (5.6%) were found to have intraspinal abnormalities on MRI. These consisted of: two combined Arnold-Chiari type 1 malformations with syrinx; one syrinx with a low lying conus; and one isolated syrinx. Overall annual incidence of early onset idiopathic scoliosis was one out of 182 000 (0.0006%). This study reports the lowest rates to date of intraspinal anomalies in patients with early onset idiopathic scoliosis, adding to knowledge regarding current incidences of these abnormalities as well as any geographical variation in the nature of the disease. Cite this article: Bone Joint J 2017;99-B:829-33. ©2017 The British Editorial Society of Bone & Joint Surgery.

Intrathecal morphine for postoperative analgesia in patients with idiopathic scoliosis undergoing posterior spinal fusion.

PubMed

Tripi, Paul A; Poe-Kochert, Connie; Potzman, Jennifer; Son-Hing, Jochen P; Thompson, George H

2008-09-15

A retrospective study of postoperative pain management with intrathecal morphine. Identify the dosing regimen of intrathecal morphine that safely and effectively provides postoperative analgesia with minimal complications in patients with idiopathic scoliosis undergoing posterior spinal fusion (PSF) and segmental spinal instrumentation (SSI). Postoperative pain after surgery for idiopathic scoliosis is a concern. Intrathecal morphine has been used to decrease pain. However, the most appropriate dose has not been determined. We retrospectively analyzed 407 consecutive patients with idiopathic scoliosis who underwent PSF and SSI at our institution from 1992 through 2006. Patients were divided into 3 groups based on the intrathecal morphine dose: no dose (n = 68); moderate dose of 9 to 19 microg/kg, mean 14 microg/kg (n = 293); and high dose of 20 microg/kg or greater, mean 24 microg/kg (n = 46). Data included demographics, Wong-Baker visual analog scale postoperative pain scores, postoperative intravenous morphine requirements, time to first rescue dose of intravenous morphine, and postoperative complications of pruritus, nausea/vomiting, respiratory depression, and pediatric intensive care unit (PICU) admission. The demographics of the 3 study groups showed no statistical differences. The mean Wong-Baker visual analog scale pain score in the post anesthesia care unit was 5.2, 0.5, and 0.2, and the mean time to first morphine rescue was 6.6, 16.7, and 22.9 hours, respectively. In the first 48 postoperative hours, respiratory depression occurred in 1 (1.5%), 8 (2.7%), and 7 (15.2%) patients, whereas PICU admission occurred in 0 (0%), 6 (2%), and 8 (17.4%) patients, respectively. The majority of PICU admissions were the result of respiratory depression. Frequency of pruritus and nausea/vomiting was similar in all 3 groups. Intrathecal morphine in the moderate dose range of 9 to 19 microg/kg (mean 14 microg/kg), provides safe and effective postoperative analgesia in the

Juvenile idiopathic arthritis.

PubMed

Boros, Christina; Whitehead, Ben

2010-09-01

Juvenile idiopathic arthritis is the most common rheumatic disease in childhood, occurring in approximately 1:500 children. Despite a recent expansion in treatment options and improvement of outcomes, significant morbidity still occurs. This article outlines the clinical manifestations, assessment, detection of complications, treatment options and monitoring requirements, with the aid of guidelines recently published by The Royal Australian College of General Practitioners, which provide practical support for general practitioners to ensure best practice care and to prevent lifelong disability in patients with juvenile idiopathic arthritis. General practice plays an important role in the early detection, initial management and ongoing monitoring of children with juvenile idiopathic arthritis. Early detection involves understanding the classification framework for subtypes of juvenile idiopathic arthritis, and being aware of the clinical manifestations and how to look for them, through history, examination and appropriate investigation. The major extra-articular manifestations of juvenile idiopathic arthritis are uveitis and growth disturbance. Treatment options include nonsteroidal anti-inflammatory drugs, methotrexate, biologic agents, and corticosteroids. Management using a multidisciplinary approach can prevent long term sequelae. Unfortunately, approximately 50% of children will have active disease as adults.

Satisfactory patient-based outcomes after surgical treatment for idiopathic clubfoot: includes surgeon's individualized technique.

PubMed

Mahan, Susan T; Spencer, Samantha A; Kasser, James R

2014-09-01

Treatment of idiopathic clubfoot has shifted towards Ponseti technique, but previously surgical management was standard. Outcomes of surgery have varied, with many authors reporting discouraging results. Our purpose was to evaluate a single surgeon's series of children with idiopathic clubfoot treated with a la carte posteromedial and lateral releases using the Pediatric Outcomes Data Collection Instrument (PODCI) with a minimum of 2-year follow-up. A total of 148 patients with idiopathic clubfoot treated surgically by a single surgeon over 15 years were identified, and mailed PODCI questionnaires. Fifty percent of the patients were located and responded, resulting in 74 complete questionnaires. Median age at surgery was 10 months (range, 5.3 to 84.7 mo), male sex 53/74 (71.6%), bilateral surgery 31/74 (41.9%), and average follow-up of 9.7 years. PODCI responses were compared with previously published normal healthy controls using t test for each separate category. Included in the methods is the individual surgeon's operative technique. In PODCIs where a parent reports for their child or adolescent, there was no difference between our data and the healthy controls in any of the 5 categories. In PODCI where an adolescent self-reports, there was no difference in 4 of 5 categories; significant difference was only found between our data (mean = 95.2; SD = 7.427) and normal controls (mean = 86.3; SD = 12.5) in Happiness Scale (P = 0.0031). In this group of idiopathic clubfoot patients, treated with judicious posteromedial release by a single surgeon, primarily when surgery was treatment of choice for clubfoot, patient-based outcomes are not different from their normal healthy peers through childhood and adolescence. While Ponseti treatment has since become the treatment of choice for clubfoot, surgical treatment, in some hands, has led to satisfactory results. Level III.

The Prevalence and Characteristics of Primary Headache and Dream-Enacting Behaviour in Japanese Patients with Narcolepsy or Idiopathic Hypersomnia: A Multi-Centre Cross-Sectional Study

PubMed Central

Suzuki, Keisuke; Miyamoto, Masayuki; Miyamoto, Tomoyuki; Inoue, Yuichi; Matsui, Kentaro; Nishida, Shingo; Hayashida, Kenichi; Usui, Akira; Ueki, Yoichiro; Nakamura, Masaki; Murata, Momoyo; Numao, Ayaka; Watanabe, Yuji; Suzuki, Shiho; Hirata, Koichi

2015-01-01

Background Because the prevalence and characteristics of primary headache have yet to be thoroughly studied in patients with hypersomnia disorders, including narcolepsy and idiopathic hypersomnia, we examined these parameters in the Japanese population. Methods In a multicentre cross-sectional survey, among 576 consecutive outpatients with sleep disorders, 68 narcolepsy patients and 35 idiopathic hypersomnia patients were included. Additionally, 61 healthy control subjects participated. Semi-structured headache questionnaires were administered to all participants. Results The patients with narcolepsy (52.9%) and idiopathic hypersomnia (77.1%) more frequently experienced headache than the healthy controls (24.6%; p<0.0001). The prevalence rates were 23.5%, 41.2% and 4.9% for migraine (p<0.0001) and 16.2%, 23.5% and 14.8% (p = 0.58) for tension-type headache among the narcolepsy patients, the idiopathic hypersomnia patients and the control subjects, respectively. Those who experienced migraine more frequently experienced excessive daytime sleepiness, defined as an Epworth Sleepiness Scale score of ≥10, than those who did not experience headache among the patients with narcolepsy (93.8% vs. 65.6%, p = 0.040) and idiopathic hypersomnia (86.7% vs. 37.5%, p = 0.026). Dream-enacting behaviour (DEB), as evaluated by the rapid eye movement sleep disorders questionnaire, was more frequently observed in the narcolepsy patients than in the idiopathic hypersomnia patients and the control subjects. An increased DEB frequency was observed in the narcolepsy patients with migraines compared to those without headache. Conclusions Migraines were frequently observed in patients with narcolepsy and idiopathic hypersomnia. DEB is a characteristic of narcolepsy patients. Further studies are required to assess the factors that contribute to migraines in narcolepsy and idiopathic hypersomnia patients. PMID:26418536

[Idiopathic facial paralysis in children].

PubMed

Achour, I; Chakroun, A; Ayedi, S; Ben Rhaiem, Z; Mnejja, M; Charfeddine, I; Hammami, B; Ghorbel, A

2015-05-01

Idiopathic facial palsy is the most common cause of facial nerve palsy in children. Controversy exists regarding treatment options. The objectives of this study were to review the epidemiological and clinical characteristics as well as the outcome of idiopathic facial palsy in children to suggest appropriate treatment. A retrospective study was conducted on children with a diagnosis of idiopathic facial palsy from 2007 to 2012. A total of 37 cases (13 males, 24 females) with a mean age of 13.9 years were included in this analysis. The mean duration between onset of Bell's palsy and consultation was 3 days. Of these patients, 78.3% had moderately severe (grade IV) or severe paralysis (grade V on the House and Brackmann grading). Twenty-seven patients were treated in an outpatient context, three patients were hospitalized, and seven patients were treated as outpatients and subsequently hospitalized. All patients received corticosteroids. Eight of them also received antiviral treatment. The complete recovery rate was 94.6% (35/37). The duration of complete recovery was 7.4 weeks. Children with idiopathic facial palsy have a very good prognosis. The complete recovery rate exceeds 90%. However, controversy exists regarding treatment options. High-quality studies have been conducted on adult populations. Medical treatment based on corticosteroids alone or combined with antiviral treatment is certainly effective in improving facial function outcomes in adults. In children, the recommendation for prescription of steroids and antiviral drugs based on adult treatment appears to be justified. Randomized controlled trials in the pediatric population are recommended to define a strategy for management of idiopathic facial paralysis. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

The distribution of immunomodulatory cells in the lungs of patients with idiopathic pulmonary fibrosis

PubMed Central

Nuovo, Gerard J.; Hagood, James S.; Magro, Cynthia M.; Chin, Nena; Kapil, Rubina; Davis, Luke; Marsh, Clay B.; Folcik, Virginia A.

2011-01-01

We have characterized the immune system involvement in the disease processes of idiopathic pulmonary fibrosis in novel ways. To do so, we analyzed lung tissue from 21 cases of idiopathic pulmonary fibrosis and 21 (non-fibrotic, non-cancerous) controls for immune cell and inflammation-related markers. The immunohistochemical analysis of the tissue was grouped by patterns of severity in disease pathology. There were significantly greater numbers of CD68+ and CD80+ cells, and significantly fewer CD3+, CD4+, and CD45RO+ cells in areas of relatively (histologically) normal lung in biopsies from idiopathic pulmonary fibrosis patients compared to controls. In zones of active disease, characterized by epithelial cell regeneration and fibrosis, there were significantly more cells expressing CD4, CD8, CD20, CD68, CD80, CCR6, S100, IL-17, tumor necrosis factor-α, and retinoic acid-related orphan receptors compared to histologically normal lung areas from idiopathic pulmonary fibrosis patients. Inflammation was implicated in these active regions by the cells that expressed retinoid orphan receptor-α, -β, and -γ, CCR6, and IL-17. The regenerating epithelial cells predominantly expressed these pro-inflammatory molecules, as evidenced by co-expression analyses with epithelial cytokeratins. Macrophages in pseudo-alveoli and CD3+ T cells in the fibrotic interstitium also expressed IL-17. Co-expression of IL-17 with retinoid orphan receptors, and epithelial cytoskeletal proteins, CD68, and CD3 in epithelial cells, macrophages, and T-cells, respectively, confirmed the production of IL-17 by these cell types. There was little staining for Foxp3, CD56, or CD34 in any idiopathic pulmonary fibrosis lung regions. The fibrotic regions had fewer immune cells overall. In summary, our study shows participation of innate and adaptive mononuclear cells in active-disease regions of idiopathic pulmonary fibrosis lung, where the regenerating epithelial cells appear to propagate inflammation

Idiopathic granulomatous mastitis: an institutional experience

PubMed Central

Prasad, Seetharam; Jaiprakash, Padmapriya; Dave, Aniket; Pai, Deepti

2017-01-01

Objective To study idiopathic granulomatous mastitis with respect to its various clinical features, etiologic factors, treatment modalities and complications. Material and methods Retrospective study of all patients who were diagnosed with idiopathic granulomatous mastitis from 1st January 2006 to 31st December 2014 at Kasturba Hospital, Manipal, India (a tertiary care referral centre). The research was performed according to the World Medical Association Declaration of Helsinki. Informed consent was taken from the patient before invasive procedures including surgery. Data was analysed using the Statistical Package for Social Sciences version 16.0 wherever appropriate. Results 73 patients diagnosed with idiopathic granulomatous mastitis during the time period were included. One patient was a male (1.37%), rest were all females (98.63%). The mean age of presentation was 32.67 years (range 23 to 66 years). 70 patients (95.89%) were parous females. Average duration since last childbirth was 4.6 years (range: 3 months to 33 years). 8 patients (10.95%) were lactating. History of oral contraceptive pill use was present in 40 patients (54.79%). The right breast was affected in 44 patients (60.27%), and the left breast in 29 patients (39.73%). None of the patients had bilateral disease. The most common symptom was a painless lump (61.64%). Rest of the patients (38.36%) presented with features of a breast abscess. 19 out of 39 FNACs done (48.72%) were positive for granulomatous mastitis. 59 were primarily managed surgically (lumpectomy/wide excision-33, incision & drainage-26). One patient was treated primarily with prednisolone. 13 patients did not receive specific treatment, and were only kept on regular follow-up. Patients managed with lumpectomy/wide excision had the least rate of complications & recurrence (18.18%). Conclusion Patients with idiopathic granulomatous mastitis can present with a wide variety of symptoms which mimic other more common conditions. Surgical

Autologous serum skin test as an indicator of chronic autoimmune urticaria in a tertiary care hospital in South India.

PubMed

Vikramkumar, Adaikalampillai Ganapathy; Kuruvila, Sheela; Ganguly, Satyaki

2014-12-01

Autologous serum skin test (ASST) is a simple in-vivo clinical test for the detection of basophil histamine releasing activity and to diagnose chronic autoimmune urticaria (CAU) among chronic spontaneous urticaria (CSU) patients. Diagnosing these patients is also important as they may need high doses of antihistamines and systemic corticosteroids during acute exacerbations. The aim of this study is to study the prevalence of CAU among cases of CSU by using ASST. This was a cross-sectional study done among 48 patients presenting with CSU. Detailed history, physical examination and routine investigations were recorded for all patients. ASST was done on all the 48 patients. Of the 48 patients included in the study, 20 patients (41.6%) were ASST positive, while the remaining 28 (58%) were ASST negative. The median duration of disease in both ASST positive and negative patients was 1 year. ASST positivity was higher (66.6%) among patients with a history of round shaped weals, though not statistically significant. ASST positivity was seen in 5 (71.4%) out of seven patients with systemic involvement, which was again not statistically significant. Our study did not show any significant difference between patients with and without antibodies regarding mean age and sex distribution, clinical morphology of individual weals, duration, severity, systemic symptoms, angioedema, atopy, and association with other autoimmune conditions.

Comparison of endogenous and radiolabeled bile acid excretion in patients with idiopathic chronic diarrhea

DOE Office of Scientific and Technical Information (OSTI.GOV)

Schiller, L.R.; Bilhartz, L.E.; Santa Ana, C.A.

Fecal recovery of radioactivity after ingestion of a bolus of radiolabeled bile acid is abnormally high in most patients with idiopathic chronic diarrhea. To evaluate the significance of this malabsorption, concurrent fecal excretion of both exogenous radiolabeled bile acid and endogenous (unlabeled) bile acid were measured in patients with idiopathic chronic diarrhea. Subjects received a 2.5-microCi oral dose of taurocholic acid labeled with 14C in the 24th position of the steroid moiety. Endogenous bile acid excretion was measured by a hydroxysteroid dehydrogenase assay on a concurrent 72-h stool collection. Both radiolabeled and endogenous bile acid excretion were abnormally high inmore » most patients with chronic diarrhea compared with normal subjects, even when equivoluminous diarrhea was induced in normal subjects by ingestion of osmotically active solutions. The correlation between radiolabeled and endogenous bile acid excretion was good. However, neither radiolabeled nor endogenous bile acid excretion was as abnormal as is typically seen in patients with ileal resection, and none of these diarrhea patients responded to treatment with cholestyramine with stool weights less than 200 g. These results suggest (a) that this radiolabeled bile acid excretion test accurately reflects excess endogenous bile acid excretion; (b) that excess endogenous bile acid excretion is not caused by diarrhea per se; (c) that spontaneously occurring idiopathic chronic diarrhea is often associated with increased endogenous bile acid excretion; and (d) that bile acid malabsorption is not likely to be the primary cause of diarrhea in most of these patients.« less

Up-dosing with bilastine results in improved effectiveness in cold contact urticaria

PubMed Central

Krause, K; Spohr, A; Zuberbier, T; Church, M K; Maurer, M

2013-01-01

Background Cold contact urticaria (CCU) is characterized by itchy wheal and flare responses due to the release of histamine and other pro-inflammatory mediators after exposure to cold. The treatment of choice is nonsedating antihistamines, dosages of which may be increased up to fourfold if standard doses are ineffective. Here, we assess the effects of a standard 20 mg dose and up-dosing to 40 and 80 mg of bilastine in reducing the symptoms of CCU and inflammatory mediator release following cold challenge. Methods Twenty patients with CCU were included in this randomized, crossover, double-blind, placebo-controlled 12-week study. They received placebo, 20, 40 or 80 mg of bilastine daily each for 7 days with 14-day washout periods. The primary readout was change in critical temperature thresholds (CTT). Secondary readouts were changes in pruritus, levels of histamine IL-6, IL-8 and TNF-α collected by skin microdialysis and safety and tolerability of bilastine. Results Bilastine 20 mg was highly effective (P < 0.0001) in reducing CTT. Up-dosing to 80 mg significantly (P < 0.04) increased its effectiveness. At this dose, 19 of 20 (95%) patients responded to treatment, with 12 of 20 (60%) becoming symptom free. Only one patient was refractory to treatment. Microdialysis levels of histamine, IL-6 and IL-8 assessed 1–3 h after cold challenge were significantly (P < 0.05) decreased following up-dosing with 80 mg bilastine. Bilastine treat-ment was well tolerated without evidence of increased sedation with dose escala-tion. Conclusions Bilastine was effective in reducing the symptoms of patients with CCU. Increased efficacy of bilastine with fourfold up-dosing was without sedation and supports urticaria treatment guidelines. PMID:23742030

Up-dosing with bilastine results in improved effectiveness in cold contact urticaria.

PubMed

Krause, K; Spohr, A; Zuberbier, T; Church, M K; Maurer, M

2013-07-01

Cold contact urticaria (CCU) is characterized by itchy wheal and flare responses due to the release of histamine and other pro-inflammatory mediators after exposure to cold. The treatment of choice is nonsedating antihistamines, dosages of which may be increased up to fourfold if standard doses are ineffective. Here, we assess the effects of a standard 20 mg dose and up-dosing to 40 and 80 mg of bilastine in reducing the symptoms of CCU and inflammatory mediator release following cold challenge. Twenty patients with CCU were included in this randomized, crossover, double-blind, placebo-controlled 12-week study. They received placebo, 20, 40 or 80 mg of bilastine daily each for 7 days with 14-day washout periods. The primary readout was change in critical temperature thresholds (CTT). Secondary readouts were changes in pruritus, levels of histamine IL-6, IL-8 and TNF-α collected by skin microdialysis and safety and tolerability of bilastine. Bilastine 20 mg was highly effective (P < 0.0001) in reducing CTT. Up-dosing to 80 mg significantly (P < 0.04) increased its effectiveness. At this dose, 19 of 20 (95%) patients responded to treatment, with 12 of 20 (60%) becoming symptom free. Only one patient was refractory to treatment. Microdialysis levels of histamine, IL-6 and IL-8 assessed 1-3 h after cold challenge were significantly (P < 0.05) decreased following up-dosing with 80 mg bilastine. Bilastine treatment was well tolerated without evidence of increased sedation with dose escalation. Bilastine was effective in reducing the symptoms of patients with CCU. Increased efficacy of bilastine with fourfold up-dosing was without sedation and supports urticaria treatment guidelines. © 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Effect of 4-aminopyridine on gravity dependence and neural integrator function in patients with idiopathic downbeat nystagmus.

PubMed

Sander, T; Sprenger, A; Marti, S; Naumann, T; Straumann, D; Helmchen, C

2011-04-01

Downbeat nystagmus (DBN) is a frequent sign in patients with cerebellar degeneration. It consists of an upward drift of the eye that does not depend on vertical head position (spontaneous drift, SD), a gravity-dependent component (GD), and a gaze-evoked drift reflecting gaze-holding impairment (deficient neural integrator function). The potassium-channel blocker 4-aminopyridine (4-AP) is reported to reduce DBN in patients with cerebellar atrophy but with little or no effect in patients with idiopathic DBN. We prospectively studied the effect of 4-AP on all three components in a large (n = 24) group of the clinically frequent idiopathic DBN. DBN was reduced by 22-31% when the head was off the head erect position. In contrast, there was no effect on vertical gaze-evoked drift. This indicates the therapeutic efficacy of 4-AP not only in patients with cerebellar atrophy but also in idiopathic DBN patients. This beneficial effect, which might be missed when gravity-dependent head positions are not tested, was not related to an improvement of gaze-holding deficit. We suggest it may be related to the restored inhibition of the overacting otolith-ocular reflex.

Idiopathic Inflammatory Myopathies

PubMed Central

Dimachkie, Mazen M.; Barohn, Richard J.

2012-01-01

The idiopathic inflammatory myopathies are a group of rare disorders including polymyositis (PM), dermatomyositis (DM), and autoimmune necrotizing myopathies (NMs). The idiopathic inflammatory myopathies share many similarities. They present acutely, subacutely, or chronically with marked proximal and symmetric muscle weakness, except for associated distal and asymmetric weakness in inclusion body myositis. The idiopathic inflammatory myopathies also share a variable degree of creatine kinase (CK) elevation and a nonspecifically abnormal electromyogram demonstrating an irritative myopathy. The muscle pathology demonstrates inflammatory exudates of variable distribution within the muscle fascicle. Despite these similarities, the idiopathic inflammatory myopathies are a heterogeneous group. The overlap syndrome (OS) refers to the association of PM, DM, or NM with connective tissue disease, such as scleroderma or systemic lupus erythematosus. In addition to elevated antinuclear antibodies (ANA), patients with OS may be weaker in the proximal arms than the legs mimicking the pattern seen in some muscular dystrophies. In this review, we focus on DM, PM, and NM and examine current and promising therapies. PMID:23117947

Acute Idiopathic Scrotal Edema: Systematic Literature Review.

PubMed

Santi, Maristella; Lava, Sebastiano A G; Simonetti, Giacomo D; Bianchetti, Mario G; Milani, Gregorio P

2018-06-01

 Existing information on acute idiopathic scrotal edema relies on small case series and textbooks.  We searched reports with no date limits on acute idiopathic scrotal edema.  Thirty-seven studies were included. Sixteen case series addressed the prevalence of acute idiopathic scrotal edema among males with acute scrotum: among 3,403 cases, the diagnosis of acute idiopathic scrotal edema was made in 413 cases (12%). Twenty-four reports addressed history, findings, management, and course of acute idiopathic scrotal edema in 311 patients. The patients mostly ranged in age from 5 to 8 years, presented with acute scrotal redness and swelling, associated or not with mild pain. Ninety percent or more of the cases developed in patients without atopic diathesis and were not preceded by inguinoscrotal surgery, acute febrile illnesses, or trauma. They were afebrile; in good general condition; and presented without pruritus, nausea or vomiting, or abdominal pain. The lesions were bilateral in two-thirds and unilateral in one-third of the cases. The condition resolved spontaneously within 2 to 3 days without sequelae. Approximately 10% of the cases experienced a recurrence.  Acute idiopathic scrotal edema is a self-limiting condition that accounts for ≥ 10% of cases of acute scrotum in children and adolescents. Georg Thieme Verlag KG Stuttgart · New York.

Serum Levels of 25-hydroxyvitamin D in Chronic Urticaria and its Association with Disease Activity: A Case Control Study.

PubMed

Rather, Shagufta; Keen, Abid; Sajad, Peerzada

2018-01-01

To evaluate the relationship between vitamin D levels and chronic spontaneous urticaria (CSU) and compare with healthy age and sex matched controls. This was a hospital-based cross-sectional study conducted over a period of 1 year, in which 110 patients with CSU were recruited along with an equal number of sex and age-matched healthy controls. For each patient, urticaria activity score (UAS) was calculated and autologous serum skin test (ASST) was performed. Plasma 25-hydroxyvitamin D [25-(OH)D] was analyzed by chemiluminescence method. A deficiency in vitamin D was defined as serum 25-(OH)D concentrations <30 ng/mL. The statistical analysis was carried out by using appropriate statistical tests. The mean serum 25-(OH)D levels of CSU patients was 19.6 ± 6.9 ng/mL, whereas in control group, the mean level was 38.5 ± 6.7, the difference being statistically significant ( P < 0.001). A significant negative correlation was found between vitamin D levels and UAS. ( P < 0.001). The number of patients with ASST positivity was 44 (40%). The patients with CSU had reduced levels of vitamin D when compared to healthy controls. Furthermore, there was a significant negative correlation between the levels of serum vitamin D and severity of CSU.

IgG abnormality in narcolepsy and idiopathic hypersomnia.

PubMed

Tanaka, Susumu; Honda, Makoto

2010-03-05

A close association between narcolepsy and the Human Leukocyte Antigen (HLA)-DQB1*0602 allele suggests the involvement of the immune system, or possibly an autoimmune process. We investigated serum IgG levels in narcolepsy. We measured the serum total IgG levels in 159 Japanese narcolepsy-cataplexy patients positive for the HLA-DQB1*0602 allele, 28 idiopathic hypersomnia patients with long sleep time, and 123 healthy controls (the HLA-DQB1*0602 allele present in 45 subjects). The serum levels of each IgG subclass were subsequently measured. The distribution of serum IgG was significantly different among healthy controls negative for the HLA-DQB1*0602 allele (11.66+/-3.55 mg/ml), healthy controls positive for the HLA-DQB1*0602 allele (11.45+/-3.43), narcolepsy patients (9.67+/-3.38), and idiopathic hypersomnia patients (13.81+/-3.80). None of the following clinical variables, age, disease duration, Epworth Sleepiness Scale, smoking habit and BMI at the time of blood sampling, were associated with IgG levels in narcolepsy or idiopathic hypersomnia. Furthermore we found the decrease in IgG1 and IgG2 levels, stable expression of IgG3, and the increase in the proportion of IgG4 in narcolepsy patients with abnormally low IgG levels. The increase in the proportion of IgG4 levels was also found in narcolepsy patients with normal serum total IgG levels. Idiopathic hypersomnia patients showed a different pattern of IgG subclass distribution with high IgG3 and IgG4 level, low IgG2 level, and IgG1/IgG2 imbalance. Our study is the first to determine IgG abnormalities in narcolepsy and idiopathic hypersomnia by measuring the serum IgG levels in a large number of hypersomnia patients. The observed IgG abnormalities indicate humoral immune alterations in narcolepsy and idiopathic hypersomnia. Different IgG profiles suggest immunological differences between narcolepsy and idiopathic hypersomnia.

Nonsurgical Management of Adolescent Idiopathic Scoliosis.

PubMed

Gomez, Jaime A; Hresko, M Timothy; Glotzbecker, Michael P

2016-08-01

Pediatric patient visits for spinal deformity are common. Most of these visits are for nonsurgical management of scoliosis, with approximately 600,000 visits for adolescent idiopathic scoliosis (AIS) annually. Appropriate management of scoliotic curves that do not meet surgical indication parameters is essential. Renewed enthusiasm for nonsurgical management of AIS (eg, bracing, physical therapy) exists in part because of the results of the Bracing in Adolescent Idiopathic Scoliosis Trial, which is the only randomized controlled trial available on the use of bracing for AIS. Bracing is appropriate for idiopathic curves between 20° and 40°, with successful control of these curves reported in >70% of patients. Patient adherence to the prescribed duration of wear is essential to maximize the effectiveness of the brace. The choice of brace type must be individualized according to the deformity and the patient's personality as well as the practice setting and brace availability.

Pre- and postoperative photographs and surgical outcomes in patients with Lenke type 1 adolescent idiopathic scoliosis.

PubMed

Albayrak, Akif; Buyuk, Abdul Fettah; Ucpunar, Hanifi; Balioglu, Mehmet Bulent; Kargin, Deniz; Kaygusuz, Mehmet Akif

2015-04-01

Clinical study with pre- and postoperative back photographs and postoperative Scoliosis Research Society-22 survey in patients who had undergone surgery for idiopathic scoliosis. To determine the effect of showing patients their photographs before and after surgery for idiopathic scoliosis on postoperative patient satisfaction. After scoliosis surgery, patient satisfaction may be limited because the patients cannot directly see their back and may forget the preoperative appearance. In 60 patients who had undergone surgery for correction of idiopathic scoliosis (Lenke type 1), pre- and postoperative photographs were taken from the anterior, posterior, and right and left lateral views. After surgery, patients in group 1 (30 patients) were shown the preoperative and most recent follow-up photographs, and patients in group 2 (30 patients) had routine evaluation but were not shown their photographs. All patients completed the Scoliosis Research Society-22 survey. Patients in both groups had similar age, sex, distribution of Lenke type and Risser sign, follow-up, and pre- and postoperative Cobb angles and balance (coronal and sagittal). A significant difference was observed between the groups for survey question 10 (which was about self-image), question 18 (which was about function and activity), and question 21 (which was about satisfaction) (P ≤ 0.05). There were no differences between patients in groups 1 and 2 in Scoliosis Research Society-22 domain or total scores. By showing patients the pre- and postoperative clinical photographs, patient satisfaction may be greater, as measured with some SRS-22 scores. This method may enable clinicians to positively change the patients' self-image perception after surgery for correction of scoliosis. 4.

Chronic Spontaneous Urticaria: Pathogenesis and Treatment Considerations.

PubMed

Kaplan, Allen P

2017-11-01

The treatment of chronic spontaneous urticaria begins with antihistamines; however, the dose required typically exceeds that recommended for allergic rhinitis. Second-generation, relatively non-sedating H₁-receptor blockers are typically employed up to 4 times a day. First-generation antihistamines, such as hydroxyzine or diphenhydramine (Atarax or Benadryl), were employed similarly in the past. Should high-dose antihistamines fail to control symptoms (at least 50%), omalizumab at 300 mg/month is the next step. This is effective in 70% of antihistamine-refractory patients. H₂-receptor blockers and leukotriene antagonists are no longer recommended; they add little and the literature does not support significant efficacy. For those patients who are unresponsive to both antihistamines and omalizumab, cyclosporine is recommended next. This is similarly effective in 65%-70% of patients; however, care is needed regarding possible side-effects on blood pressure and renal function. Corticosteroids should not be employed chronically due to cumulative toxicity that is dose and time dependent. Brief courses of steroid e.g., 3-10 days can be employed for severe exacerbations, but should be an infrequent occurrence. Finally, other agents, such as dapsone or sulfasalazine, can be tried for those patients unresponsive to antihistamines, omalizumab, and cyclosporine. Copyright © 2017 The Korean Academy of Asthma, Allergy and Clinical Immunology · The Korean Academy of Pediatric Allergy and Respiratory Disease.

Stent-Graft Placement for Femoral Artery Pseudoaneurysm in a Patient with Idiopathic Multiple Arterial Aneurysmal Disease

DOE Office of Scientific and Technical Information (OSTI.GOV)

Koo, Bon Kwon; Choi, Donghoon; Kwon, Kihwan

2002-12-15

A 34-year-old man presenting with a left inguinal mass was diagnosed as having idiopathic multiple aneurysmal disease and pseudoaneurysm of a left superficial femoral artery. A stent-graft was successfully deployed percutaneously at the left superficial femoral artery pseudoaneurysm. Less invasive treatment should be used to avoid vascular complications in patients with idiopathic multiple aneurysmal disease, especially in those who have a progressive course or a history of prior complications after surgical repair.

Idiopathic Hypersomnia: Clinical Features and Response to Treatment

PubMed Central

Ali, Mohsin; Auger, R. Robert; Slocumb, Nancy L.; Morgenthaler, Timothy I.

2009-01-01

Objective: A recent American Academy of Sleep Medicine publication identified a need for research regarding idiopathic hypersomnia. We describe various clinical and polysomnographic features of patients with idiopathic hypersomnia, with an emphasis on response to pharmacotherapy. Methods: A retrospective review of our database initially identified 997 patients, utilizing “idiopathic hypersomnia,” “hypersomnia NOS,” and “primary hypersomnia” as keywords. The charts of eligible patients were examined in detail, and data were abstracted and analyzed. Response to treatment was graded utilizing an internally developed scale. Results: Eighty-five patients were ultimately identified (65% female). Median (interquartile range) ages of onset and diagnosis were 19.6 (15.5) and 33.7 (15.5), respectively. During a median follow-up duration of 2.4 (4.7) years, 65% of patients demonstrated a “complete response” to pharmacotherapy as assessed by the authors' grading schema. Methylphenidate was most commonly used as a first-line agent prior to December 1998, but subsequently, modafinil became the most common first drug. At the last recorded follow-up visit, 92% of patients were on monotherapy, with greater representation of methylphenidate versus modafinil (51% vs. 32%). Among these patients, methylphenidate produced a higher percentage of “complete” or “partial” responses than modafinil, although statistical significance was not reached (38/40 [ 95%] vs 22/25 [88%], respectively, p = 0.291). Conclusions: The majority of patients with idiopathic hypersomnia respond well to treatment. Methylphenidate is chosen more often than modafinil as final monotherapy in the treatment of idiopathic hypersomnia, despite the fact that it is less commonly used initially. Further prospective comparisons of medications should be explored. Citation: Ali M; Auger RR; Slocumb NL; Morgenthaler TI. Idiopathic hypersomnia: clinical features and response to treatment. J Clin Sleep

Effect of Emphysema Extent on Serial Lung Function in Patients with Idiopathic Pulmonary Fibrosis.

PubMed

Cottin, Vincent; Hansell, David M; Sverzellati, Nicola; Weycker, Derek; Antoniou, Katerina M; Atwood, Mark; Oster, Gerry; Kirchgaessler, Klaus-Uwe; Collard, Harold R; Wells, Athol U

2017-11-01

Patients with idiopathic pulmonary fibrosis and emphysema may have artificially preserved lung volumes. In this post hoc analysis, we investigated the relationship between baseline emphysema and fibrosis extents, as well as pulmonary function changes, over 48 weeks. Data were pooled from two phase III, randomized, double-blind, placebo-controlled trials of IFN-γ-1b in idiopathic pulmonary fibrosis (GIPF-001 [NCT00047645] and GIPF-007 [NCT00075998]). Patients with Week 48 data, baseline high-resolution computed tomographic images, and FEV 1 /FVC ratios less than 0.8 or greater than 0.9 (<0.7 or >0.9 in GIPF-007), as well as randomly selected patients with ratios of 0.8-0.9 and 0.7-0.8, were included. Changes from baseline in pulmonary function at Week 48 were analyzed by emphysema extent. The relationship between emphysema and fibrosis extents and change in pulmonary function was assessed using multivariate linear regression. Emphysema was identified in 38% of patients. A negative correlation was observed between fibrosis and emphysema extents (r = -0.232; P < 0.001). In quartile analysis, patients with the greatest emphysema extent (28 to 65%) showed the smallest FVC decline, with a difference of 3.32% at Week 48 versus patients with no emphysema (P = 0.047). In multivariate analyses, emphysema extent greater than or equal to 15% was associated with significantly reduced FVC decline over 48 weeks versus no emphysema or emphysema less than 15%. No such association was observed for diffusing capacity of the lung for carbon monoxide or composite physiologic index. FVC measurements may not be appropriate for monitoring disease progression in patients with idiopathic pulmonary fibrosis and emphysema extent greater than or equal to 15%.

Determinants of hand dermatitis, urticaria and loss of skin barrier function in professional cleaners in New Zealand.

PubMed

Douwes, Jeroen; Slater, Tania; Shanthakumar, Mathangi; McLean, Dave; Firestone, Ridvan Tua; Judd, Lissa; Pearce, Neil

2017-04-01

This study assessed the risk of dermatitis, urticaria and loss of skin barrier function in 425 cleaners and 281 reference workers (retail workers and bus drivers). Symptoms, atopy and skin barrier function were assessed by questionnaire, skin prick tests, and measurement of transepidermal water loss. Cleaners had an increased risk of current (past 3 months) hand/arm dermatitis (14.8% vs. 10.0%; OR = 1.9, p < 0.05) and urticaria (11% vs. 5.3%; OR = 2.4, p < 0.05) and were more likely to have dermatitis as adults (17.6% vs. 11.4%; OR = 1.8, p < 0.05). The risk of atopy was not increased, but associations with symptoms were more pronounced in atopics. Transepidermal water loss was significantly higher in cleaners. Wet-work was a significant risk factor for dermatitis and hand washing and drying significantly reduced the risk of urticaria. In conclusion, cleaners have an increased risk of hand/arm dermatitis, urticaria and loss of skin barrier function.

Prevalence of the HLA-DQB1*0602 allele in narcolepsy and idiopathic hypersomnia patients seen at a sleep disorders outpatient unit in São Paulo.

PubMed

Coelho, Fernando Morgadinho Santos; Pradella-Hallinan, Márcia; Predazzoli Neto, Mario; Bittencourt, Lia Rita Azeredo; Tufik, Sérgio

2009-03-01

Narcolepsy (with and without cataplexy) and idiopathic hypersomnia, are disorders with common features but with different HLA-DQB1*0602 allele prevalence. The present study describes the prevalence of HLA-DQB1*0602 allele in narcoleptics with and without cataplexy and in patients with idiopathic hypersomnia. Subjects comprised 68 patients who were diagnosed for narcolepsy or idiopathic hypersomnia and 23 healthy controls according to the International Classification of Sleep Disorders-2. Subjects comprised 43 patients with narcolepsy and cataplexy, 11 patients with narcolepsy but without cataplexy, 14 patients with idiopathic hypersomnia and 23 healthy controls. Genotyping of HLA-DQB1*0602 allele was performed for all subjects. The prevalence of the HLA-DQB1*0602 allele was increased in idiopathic hypersomnia and in narcoleptic patients with and without cataplexy when compared to healthy subjects (p = 0.04; p = 0.03 and p < 0.0001, respectively). This finding is in accordance with those of previous studies. The gold standard exam of narcolepsy with cataplexy is Hypocretin-1 dosage, but in patients without cataplexy and idiopathic hypersomnia, there are no specific diagnostic lab findings. The presence of the HLA-DQB1* 0602 allele may be important for the differential diagnosis of situations that resemble those sleep disorders such as secondary changes in sleep structure due to drugs' consumption.

Postinfluenza Vaccination Idiopathic Thrombocytopenic Purpura in Three Elderly Patients

PubMed Central

Nagasaki, Joji; Manabe, Masahiro; Ido, Kentaro; Ichihara, Hiroyoshi; Aoyama, Yasutaka; Ohta, Tadanobu; Furukawa, Yoshio; Mugitani, Atsuko

2016-01-01

The etiologies of secondary idiopathic thrombocytopenic purpura (ITP) include infection, autoimmune disease, and immunodeficiency. We report the cases of three elderly patients who developed ITP after receiving influenza vaccinations. The platelet count of an 81-year-old woman fell to 27,000/μL after she received an influenza vaccination. A 75-year-old woman developed thrombocytopenia (5,000 platelets/μL) after receiving an influenza vaccination. An 87-year-old woman whose laboratory test values included a platelet count of 2,000/μL experienced genital bleeding after receiving an influenza vaccination. After Helicobacter pylori (HP) eradication or corticosteroid treatment, all of the patients' platelet counts increased. Influenza vaccination is an underlying etiology of ITP in elderly patients. HP eradication or corticosteroid treatment is effective for these patients. Clinicians should be aware of the association between ITP and influenza vaccinations. PMID:26998369

An electrocardiographic scoring system for distinguishing right ventricular outflow tract arrhythmias in patients with arrhythmogenic right ventricular cardiomyopathy from idiopathic ventricular tachycardia.

PubMed

Hoffmayer, Kurt S; Bhave, Prashant D; Marcus, Gregory M; James, Cynthia A; Tichnell, Crystal; Chopra, Nagesh; Moxey, Laura; Krahn, Andrew D; Dixit, Sanjay; Stevenson, William; Calkins, Hugh; Badhwar, Nitish; Gerstenfeld, Edward P; Scheinman, Melvin M

2013-04-01

Ventricular arrhythmias in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) and idiopathic ventricular tachycardia (VT) can share a left bundle branch block/inferior axis morphology. We previously reported electrocardiogram characteristics during outflow tract ventricular arrhythmias that helped distinguish VT related to ARVD/C from idiopathic VT. To prospectively validate these criteria. We created a risk score by using a derivation cohort. Two experienced electrophysiologists blinded to the diagnosis prospectively scored patients with VT/premature ventricular contractions (PVCs) with left bundle branch block/inferior axis pattern in a validation cohort of 37 ARVD/C tracings and 49 idiopathic VT tracings. All patients with ARVD/C had their diagnosis confirmed based on the revised task force criteria. Patients with idiopathic VT were selected based on structurally normal hearts with documented right ventricular outflow tract VT successfully treated with ablation. The scoring system provides 3 points for sinus rhythm anterior T-wave inversions in leads V1-V3 and during ventricular arrhythmia: 2 points for QRS duration in lead I≥120 ms, 2 points for QRS notching, and 1 point for precordial transition at lead V5 or later. A score of 5 or greater was able to correctly distinguish ARVD/C from idiopathic VT 93% of the time, with a sensitivity of 84%, specificity of 100%, positive predictive value of 100%, and negative predictive value of 91%. We describe a simple scoring algorithm that uses 12-lead electrocardiogram characteristics to effectively distinguish right ventricular outflow tract arrhythmias originating from patients with ARVD/C versus patients with idiopathic VT. Copyright © 2013 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.

The EAACI/GA²LEN/EDF/WAO Guideline for the Definition, Classification, Diagnosis and Management of Urticaria. The 2017 Revision and Update.

PubMed

Zuberbier, T; Aberer, W; Asero, R; Abdul Latiff, A H; Baker, D; Ballmer-Weber, B; Bernstein, J A; Bindslev-Jensen, C; Brzoza, Z; Buense Bedrikow, R; Canonica, G W; Church, M K; Craig, T; Danilycheva, I V; Dressler, C; Ensina, L F; Giménez-Arnau, A; Godse, K; Gonçalo, M; Grattan, C; Hebert, J; Hide, M; Kaplan, A; Kapp, A; Katelaris, C H; Kocatürk, E; Kulthanan, K; Larenas-Linnemann, D; Leslie, T A; Magerl, M; Mathelier-Fusade, P; Meshkova, R Y; Metz, M; Nast, A; Nettis, E; Oude-Elberink, H; Rosumeck, S; Saini, S S; Sánchez-Borges, M; Schmid-Grendelmeier, P; Staubach, P; Sussman, G; Toubi, E; Vena, G A; Vestergaard, C; Wedi, B; Werner, R N; Zhao, Z; Maurer, M

2018-01-15

This evidence and consensus-based guideline was developed following the methods recommended by Cochrane and the Grading of Recommendations Assessment, Development and Evaluation (GRADE) working group. The conference was held on December 1st, 2016. It is a joint initiative of the Dermatology Section of the European Academy of Allergology and Clinical Immunology (EAACI), the EU-founded network of excellence, the Global Allergy and Asthma European Network (GA²LEN), the European Dermatology Forum (EDF), and the World Allergy Organization (WAO) with the participation of 48 delegates of 42 national and international societies. This guideline was acknowledged and accepted by the European Union of Medical Specialists (UEMS). Urticaria is a frequent, mast cell-driven disease, presenting with wheals, angioedema, or both. The lifetime prevalence for acute urticaria is approximately 20%. Chronic spontaneous urticaria and other chronic forms of urticaria are disabling, impair quality of life, and affect performance at work and school. This guideline covers the definition and classification of urticaria, taking into account the recent progress in identifying its causes, eliciting factors and pathomechanisms. In addition, it outlines evidence-based diagnostic and therapeutic approaches for the different subtypes of urticaria. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

CLINICAL CHARACTERISTICS OF IDIOPATHIC FOVEOMACULAR RETINOSCHISIS.

PubMed

Maruko, Ichiro; Morizane, Yuki; Kimura, Shuhei; Shiode, Yusuke; Hosokawa, Mio; Sekiryu, Tetsuju; Iida, Tomohiro; Shiraga, Fumio

2016-08-01

To describe the clinical features of idiopathic foveomacular retinoschisis not in association with myopia, glaucoma, optic disk pit, or juvenile retinoschisis. Retrospective observational case series. Five eyes of five patients with idiopathic foveomacular retinoschisis were included. The patients were 2 men and 3 women (average age, 75.2 years; range, 71-78 years). The average spherical equivalent was +2.40 diopters (range, +0.88 to +5.75 diopters), and the average axial length was 22.0 mm (range, 21.1-23.1 mm). All patients had retinoschisis from the macula to the optic disk in the affected eye. No patients had retinoschisis in the fellow eye. The average best-corrected visual acuity was 20/44 (68 Early Treatment Diabetic Retinopathy Study letter score). Idiopathic foveomacular retinoschisis is not inherited or associated with myopia, vitreomacular traction syndrome, optic pit, or glaucoma but is associated with older age, unilaterality, hyperopia with short axial length, complete posterior vitreous detachment, and weak leakage from the optic disk on fluorescein angiography.

Migraine headache in patients with idiopathic intracranial hypertension.

PubMed

Sina, Farzad; Razmeh, Saeed; Habibzadeh, Neda; Zavari, Arefeh; Nabovvati, Mona

2017-08-29

Migraine is a neurological disorder that afflicts many people in the world and can cause severe disability during the attacks. The pathophysiology of migraine is complex and not fully understood. It seems that migraine is common in idiopathic intracranial hypertension (IIH). However, the association between migraine headache and IIH is still unclear. The present study was conducted to assess the prevalence of migraine headache and associated factors in IIH patients. In this cross-sectional study, a total of 68 patients diagnosed with IIH underwent a medical history interview and a neurological examination. The diagnosis of migraine was based on the four diagnostic criteria of the International Classification of Headache Disorders 3rd edition. Forty-five patients (63.2%) met the diagnostic criteria of migraine headache. There was no significant difference between patients with and without migraine headache in respect of their age, gender, body mass. This study revealed high prevalence of migraine headache in IIH patients; appropriate treatment can reduce their headache and prevent unnecessary treatments for IIH.

Migraine headache in patients with idiopathic intracranial hypertension

PubMed Central

Sina, Farzad; Razmeh, Saeed; Habibzadeh, Neda; Zavari, Arefeh; Nabovvati, Mona

2017-01-01

Migraine is a neurological disorder that afflicts many people in the world and can cause severe disability during the attacks. The pathophysiology of migraine is complex and not fully understood. It seems that migraine is common in idiopathic intracranial hypertension (IIH). However, the association between migraine headache and IIH is still unclear. The present study was conducted to assess the prevalence of migraine headache and associated factors in IIH patients. In this cross-sectional study, a total of 68 patients diagnosed with IIH underwent a medical history interview and a neurological examination. The diagnosis of migraine was based on the four diagnostic criteria of the International Classification of Headache Disorders 3rd edition. Forty-five patients (63.2%) met the diagnostic criteria of migraine headache. There was no significant difference between patients with and without migraine headache in respect of their age, gender, body mass. This study revealed high prevalence of migraine headache in IIH patients; appropriate treatment can reduce their headache and prevent unnecessary treatments for IIH. PMID:29071043

Body image in patients with adolescent idiopathic scoliosis: validation of the Body Image Disturbance Questionnaire--Scoliosis Version.

PubMed

Auerbach, Joshua D; Lonner, Baron S; Crerand, Canice E; Shah, Suken A; Flynn, John M; Bastrom, Tracey; Penn, Phedra; Ahn, Jennifer; Toombs, Courtney; Bharucha, Neil; Bowe, Whitney P; Newton, Peter O

2014-04-16

Appearance concerns in individuals with adolescent idiopathic scoliosis can result in impairment in daily functioning, or body image disturbance. The Body Image Disturbance Questionnaire (BIDQ) is a self-reported, seven-question instrument that measures body image disturbance in general populations; no studies have specifically examined body image disturbance in those with adolescent idiopathic scoliosis. This study aimed to validate a modified version of the BIDQ in a population with adolescent idiopathic scoliosis and to establish discriminant validity by comparing responses of operatively and nonoperatively treated patients with those of normal controls. In the first phase, a multicenter study of forty-nine patients (mean age, fourteen years; thirty-seven female) with adolescent idiopathic scoliosis was performed to validate the BIDQ-Scoliosis version (BIDQ-S). Participants completed the BIDQ-S, Scoliosis Research Society (SRS)-22, Children's Depression Index (CDI), and Body Esteem Scale for Adolescents and Adults (BESAA) questionnaires. Descriptive statistics and Pearson correlation coefficients were calculated. In the second phase, ninety-eight patients with adolescent idiopathic scoliosis (mean age, 15.7 years; seventy-five female) matched by age and sex with ninety-eight healthy adolescents were enrolled into a single-center study to evaluate the discriminant validity of the BIDQ-S. Subjects completed the BIDQ-S and a demographic form before treatment. Independent-sample t tests and Pearson correlation coefficients were calculated. The BIDQ-S was internally consistent (Cronbach alpha = 0.82), and corrected item total correlations ranged from 0.47 to 0.67. The BIDQ-S was significantly correlated with each domain of the SRS-22 and the total score (r = -0.50 to -0.72, p ≤ 0.001), with the CDI (r = 0.31, p = 0.03), and with the BESAA (r = 0.60, p < 0.001). BIDQ-S scores differed significantly between patients (1.50) and controls (1.06, p < 0.005), establishing

Randomized controlled trial of foot reflexology for patients with symptomatic idiopathic detrusor overactivity.

PubMed

Mak, Ho-Leung Jimmy; Cheon, Willy Cecilia; Wong, To; Liu, Yu Sun John; Tong, Wai Mei Anny

2007-06-01

The aim of this study was to examine whether foot reflexology has beneficial effects on patients with idiopathic detrusor overactivity. One hundred and nine women with symptomatic idiopathic detrusor overactivity were randomized into either foot reflexology treatment group or nonspecific foot massage control group. The primary outcome measure was the change in the diurnal micturition frequency. There was significant change in the number of daytime frequency in the reflexology group when compared with the massage group (-1.90 vs -0.55, p = 0.029). There was also a decrease in the 24-h micturition frequency in both groups, but the change was not statistically significant (-2.80 vs -1.04 p = 0.055). In the reflexology group, more patients believed to have received "true" reflexology (88.9 vs 67.4%, p = 0.012). This reflects the difficulty of blinding in trials of reflexology. Larger scale studies with a better-designed control group and an improved blinding are required to examine if reflexology is effective in improving patients' overall outcome.

Obesity is a significant susceptibility factor for idiopathic AA amyloidosis.

PubMed

Blank, Norbert; Hegenbart, Ute; Dietrich, Sascha; Brune, Maik; Beimler, Jörg; Röcken, Christoph; Müller-Tidow, Carsten; Lorenz, Hanns-Martin; Schönland, Stefan O

2018-03-01

To investigate obesity as susceptibility factor in patients with idiopathic AA amyloidosis. Clinical, biochemical and genetic data were obtained from 146 patients with AA amyloidosis. Control groups comprised 40 patients with long-standing inflammatory diseases without AA amyloidosis and 56 controls without any inflammatory disease. Patients with AA amyloidosis had either familial Mediterranean fever (FMF) or long-standing rheumatic diseases as underlying inflammatory disease (n = 111, median age 46 years). However, in a significant proportion of patients with AA amyloidosis no primary disease was identified (idiopathic AA; n = 37, median age 60 years). Patients with idiopathic AA amyloidosis were more obese and older than patients with AA amyloidosis secondary to FMF or rheumatic diseases. Serum leptin levels correlated with the body mass index (BMI) in all types of AA amyloidosis. Elevated leptin levels of more than 30 µg/l were detected in 18% of FMF/rheumatic + AA amyloidosis and in 40% of patients with idiopathic AA amyloidosis (p = .018). Finally, the SAA1 polymorphism was confirmed as a susceptibility factor for AA amyloidosis irrespective of the type of the disease. Obesity, age and the SAA1 polymorphism are susceptibility factors for idiopathic AA amyloidosis. Recent advances in treatment of FMF and rheumatic disorders will decrease the incidence of AA amyloidosis due to these diseases. Idiopathic AA, however, might be an emerging problem in the ageing and increasingly obese population.

Sensory Alterations in Patients with Isolated Idiopathic Dystonia: An Exploratory Quantitative Sensory Testing Analysis.

PubMed

Paracka, Lejla; Wegner, Florian; Blahak, Christian; Abdallat, Mahmoud; Saryyeva, Assel; Dressler, Dirk; Karst, Matthias; Krauss, Joachim K

2017-01-01

Abnormalities in the somatosensory system are increasingly being recognized in patients with dystonia. The aim of this study was to investigate whether sensory abnormalities are confined to the dystonic body segments or whether there is a wider involvement in patients with idiopathic dystonia. For this purpose, we recruited 20 patients, 8 had generalized, 5 had segmental dystonia with upper extremity involvement, and 7 had cervical dystonia. In total, there were 13 patients with upper extremity involvement. We used Quantitative Sensory Testing (QST) at the back of the hand in all patients and at the shoulder in patients with cervical dystonia. The main finding on the hand QST was impaired cold detection threshold (CDT), dynamic mechanical allodynia (DMA), and thermal sensory limen (TSL). The alterations were present on both hands, but more pronounced on the side more affected with dystonia. Patients with cervical dystonia showed a reduced CDT and hot detection threshold (HDT), enhanced TSL and DMA at the back of the hand, whereas the shoulder QST only revealed increased cold pain threshold and DMA. In summary, QST clearly shows distinct sensory abnormalities in patients with idiopathic dystonia, which may also manifest in body regions without evident dystonia. Further studies with larger groups of dystonia patients are needed to prove the consistency of these findings.

Family history and its association to curve size and treatment in 1,463 patients with idiopathic scoliosis.

PubMed

Grauers, Anna; Danielsson, Aina; Karlsson, Magnus; Ohlin, Acke; Gerdhem, Paul

2013-11-01

To study family history in relation to curve severity, gender, age at diagnosis and treatment in idiopathic scoliosis. A self-assessment questionnaire on family history of scoliosis was administered to 1,463 untreated, brace or surgically treated idiopathic scoliosis patients. Out of the 1,463 patients, 51 % had one or more relatives with scoliosis. There was no significant difference between females and males, nor between juvenile and adolescent study participants in this respect (p = 0.939 and 0.110, respectively). There was a significant difference in maximum curve size between patients with one or more relatives with scoliosis (median 35°, interquartile range 25) and patients without any relative with scoliosis (median 32°, interquartile range 23) (p = 0.022). When stratifying patients according to treatment (observation, brace treatment or surgery), we found that it was more common to have a relative with scoliosis among the treated patients (p = 0.011). The OR for being treated was 1.32 (95% CI 1.06-1.64) when the patient had a relative with scoliosis, compared to not having. Larger curve sizes were found in patients with a family history of scoliosis than in the ones without. No relation between family history and gender or between family history and age at onset of idiopathic scoliosis was found. Although the presence of a family history of scoliosis may not be a strong prognostic risk factor, it indicates that these patients are at higher risk of developing a more severe curve.

Intensive care unit versus hospital floor: a comparative study of postoperative management of patients with adolescent idiopathic scoliosis.

PubMed

Shan, Le-Qun; Skaggs, David L; Lee, Christopher; Kissinger, Catherine; Myung, Karen S

2013-04-03

Patients undergoing posterior spinal instrumentation and fusion surgery for adolescent idiopathic scoliosis were admitted to the intensive care unit until two years ago, at which time we changed our protocol to admit these patients to the general hospital floor following a brief stay in a postanesthesia care unit. This study compared postoperative management on a hospital floor with that in the intensive care unit for patients with adolescent idiopathic scoliosis undergoing posterior spinal fusion. A retrospective review of 124 consecutive patients with adolescent idiopathic scoliosis treated with spinal fusion from August 2007 to August 2010 was performed. Inclusion criteria were a diagnosis of adolescent idiopathic scoliosis and posterior spinal instrumentation and fusion surgery. Of 124 patients, sixty-six were managed postoperatively in the intensive care unit and fifty-eight, on the hospital floor. The mean age at the time of surgery was fourteen years. A mean of eleven vertebral levels (range, six to fifteen levels) were fused. No significant difference between the groups was found with respect to the mean age at the time of surgery, mean weight, mean preoperative and postoperative Cobb angles, and mean number of levels fused (p ≥ 0.12). However, the use of analgesic and antianxiety medication, number of postoperative blood tests, days of hospital stay, and number of physical therapy sessions were significantly decreased in the floor group compared with the intensive care unit group (p ≤ 0.05). No patient from the floor group had to be admitted to the intensive care unit. The mean charge was $33,121 for the floor group and $39,252 for the intensive care unit group (p < 0.001). Initial postoperative management of patients with adolescent idiopathic scoliosis following a posterior spinal instrumentation and fusion surgery on a general hospital floor, rather than in an intensive care unit, was associated with a shorter hospital stay, fewer blood tests, less

Dietary intake and nutritional deficiencies in patients with diabetic or idiopathic gastroparesis.

PubMed

Parkman, Henry P; Yates, Katherine P; Hasler, William L; Nguyan, Linda; Pasricha, Pankaj J; Snape, William J; Farrugia, Gianrico; Calles, Jorge; Koch, Kenneth L; Abell, Thomas L; McCallum, Richard W; Petito, Dorothy; Parrish, Carol Rees; Duffy, Frank; Lee, Linda; Unalp-Arida, Aynur; Tonascia, James; Hamilton, Frank

2011-08-01

Gastroparesis can lead to food aversion, poor oral intake, and subsequent malnutrition. We characterized dietary intake and nutritional deficiencies in patients with diabetic and idiopathic gastroparesis. Patients with gastroparesis on oral intake (N = 305) were enrolled in the National Institute of Diabetes and Digestive and Kidney Diseases Gastroparesis Registry and completed diet questionnaires at 7 centers. Medical history, gastroparesis symptoms, answers to the Block Food Frequency Questionnaire, and gastric emptying scintigraphy results were analyzed. Caloric intake averaged 1168 ± 801 kcal/day, amounting to 58% ± 39% of daily total energy requirements (TER). A total of 194 patients (64%) reported caloric-deficient diets, defined as <60% of estimated TER. Only 5 patients (2%) followed a diet suggested for patients with gastroparesis. Deficiencies were present in several vitamins and minerals; patients with idiopathic disorders were more likely to have diets with estimated deficiencies in vitamins A, B(6), C, K, iron, potassium, and zinc than diabetic patients. Only one-third of patients were taking multivitamin supplements. More severe symptoms (bloating and constipation) were characteristic of patients who reported an energy-deficient diet. Overall, 32% of patients had nutritional consultation after the onset of gastroparesis; consultation was more likely among patients with longer duration of symptoms and more hospitalizations and patients with diabetes. Multivariable logistic regression analysis indicated that nutritional consultation increased the chances that daily TER were met (odds ratio, 1.51; P = .08). Many patients with gastroparesis have diets deficient in calories, vitamins, and minerals. Nutritional consultation is obtained infrequently but is suggested for dietary therapy and to address nutritional deficiencies. Copyright © 2011 AGA Institute. Published by Elsevier Inc. All rights reserved.

Idiopathic hypersomnia: clinical features and response to treatment.

PubMed

Ali, Mohsin; Auger, R Robert; Slocumb, Nancy L; Morgenthaler, Timothy I

2009-12-15

A recent American Academy of Sleep Medicine publication identified a need for research regarding idiopathic hypersomnia. We describe various clinical and polysomnographic features of patients with idiopathic hypersomnia, with an emphasis on response to pharmacotherapy. A retrospective review of our database initially identified 997 patients, utilizing "idiopathic hypersomnia", "hypersomnia NOS", and "primary hypersomnia" as keywords. The charts of eligible patients were examined in detail, and data were abstracted and analyzed. Response to treatment was graded utilizing an internally developed scale. Eighty-five patients were ultimately identified (65% female). Median (interquartile range) ages of onset and diagnosis were 19.6 (15.5) and 33.7 (15.5), respectively. During a median follow-up duration of 2.4 (4.7) years, 65% of patients demonstrated a "complete response" to pharmacotherapy as assessed by the authors' grading schema. Methylphenidate was most commonly used as a first-line agent prior to December 1998, but subsequently, modafinil became the most common first drug. At the last recorded follow-up visit, 92% of patients were on monotherapy, with greater representation of methylphenidate versus modafinil (51% vs. 32%). Among these patients, methylphenidate produced a higher percentage of "complete" or "partial" responses than modafinil, although statistical significance was not reached (38/40 [95%] vs. 22/25 [88%], respectively, p = 0.291). The majority of patients with idiopathic hypersomnia respond well to treatment. Methylphenidate is chosen more often than modafinil as final monotherapy in the treatment of idiopathic hypersomnia, despite the fact that it is less commonly used initially. Further prospective comparisons of medications should be explored.

Idiopathic intracranial hypertension and sickle cell disease: two case reports.

PubMed

Segal, Laura; Discepola, Marino

2005-12-01

Two patients with sickle cell disease presented with headaches and visual disturbances, typical complaints of this disorder. However, prompt diagnosis of idiopathic intracranial hypertension and initiation of medical therapy lead to improved symptoms and restored vision. Ophthalmologists should consider sickle cell disease to be an independent risk factor for idiopathic intracranial hypertension when a patient is being assessed for visual disturbances. Although a rare condition, idiopathic intracranial hypertension has several key signs useful in establishing a diagnosis. It is critical to recognize the warning signs and symptoms to prevent devastating ophthalmologic complications. We report the first cases of idiopathic intracranial hypertension in patients with the novel Quebec-Chori beta-chain variant of sickle cell disease.

The significance of hypersensitivity to autologous sweat and serum in cholinergic urticaria: cholinergic urticaria may have different subtypes.

PubMed

Kim, Jung Eun; Jung, Kwan Ho; Cho, Hyun Hee; Kang, Hoon; Park, Young Min; Park, Hyun Jeong; Lee, Jun Young

2015-07-01

The pathogenesis of cholinergic urticaria (ChU) has been unclear except for the involvement of acetylcholine. Attempts to classify ChU according to etiology have rarely been performed. To evaluate the significance of responsiveness to autologous sweat and serum in ChU in relation to their clinical characteristics. This study involved 18 patients diagnosed with ChU between January 2010 and April 2011 in the Catholic Medical Center-St. Paul's Hospital. History taking included symptom duration, association with atopy, decreased sweat secretions, seasonal variation, and response to treatment. Intradermal autologous serum skin test (ASST) and autologous sweat skin test (ASwST) and basophil histamine release test with sweat were done. Sweat hypersensitivity was proven by a positive ASwST and basophil histamine release test in only 37.5% of patients with ChU, and in none of the healthy controls. The weal size of ASwST correlated with percentage basophil histamine release. A positive response to autologous serum was displayed by 38.9% of patients, whereas 10% of healthy controls showed a positive ASST response. Intriguingly, patients with a positive ASwST had a negative ASST, and vice versa. Despite this, there was no difference in the clinical characteristics between positive ASST and positive ASwST groups. The frequency of hypersensitivity to autologous sweat and serum was significantly higher in patients with ChU, compared with healthy controls. This suggests that autoimmunity to an unknown serum factor as well as sweat hypersensitivity may be involved in the pathogenesis of ChU. © 2014 The International Society of Dermatology.

Risk factors for work-related eczema and urticaria among vocational students of agriculture.

PubMed

Śpiewak, Radosław; Góra-Florek, Anna; Horoch, Andrzej; Jarosz, Mirosław J; Doryńska, Agnieszka; Golec, Marcin; Dutkiewicz, Jacek

2017-12-23

Farmers are at high risk of occupational skin diseases which may start already during vocational training. This study was aimed at identification of risk factors for work-related skin diseases among vocational students of agriculture. The study involved 440 students (245 males, 195 females aged 17-21 years) in 11 vocational schools which were at least 100 km from each other. The protocol included a physician-managed questionnaire and medical examination, skin prick tests, patch tests, total IgE and Phadiatop. Logistic regression model was used for the identification of relevant risk factors. Work-related dermatoses were diagnosed in 29 study participants (6.6%, 95%CI: 4.3-8.9%): eczema in 22, urticaria in 14, and co-existence of both in 7 students. Significant risk factors for work-related eczema were: history of respiratory allergy (OR=10.10; p<0.001), history of eczema (itchy rash) provoked by wet work and detergents before entering the school (OR=5.85; p<0.001), as well as history of contact dermatitis to metals, rubber or cosmetics prior to inscription (OR=2.84; p=0.016), and family history of any skin disease (OR=2.99; p=0.013). Significant risk factors for work-related urticaria were: history of allergic rhinitis and asthma prior to inscription (OR=7.29; p=0.006), positive skin prick tests to work place allergens (OR=4.65; p=0.002) and to environmental allergens (OR=3.79; p=0.009), and positive Phadiatop test (OR=3.61; p=0.013). Work-related skin diseases are common among vocational students of agriculture. Atopy, past history of asthma, allergic rhinitis, and eczema (either atopic, allergic or irritant) are relevant risk factors for work-related eczema and urticaria in young farmers, along with family history of any skin disease. Positive skin prick tests seem relevant, especially in the case of urticaria. Asking simple, aimed questions during health checks while enrolling students into agricultural schools would suffice to identify students at risk for work

Intestinal Volvulus in Idiopathic Steatorrhea

PubMed Central

Warner, H. A.; Kinnear, D. G.; Cameron, D. G.

1963-01-01

Volvulus of the intestine has recently been observed in three patients with idiopathic steatorrhea in relapse. Two patients gave a history of intermittent abdominal pain, distension and obstipation. Radiographic studies during these attacks revealed obstruction at the level of the sigmoid colon. Reduction under proctoscopic control was achieved in one instance, spontaneous resolution occurring in the other. The third patient presented as a surgical emergency and underwent operative reduction of a small intestinal volvulus. Persistence of diarrhea and weight loss postoperatively led to further investigation and a diagnosis of idiopathic steatorrhea. In all cases, treatment resulted in clinical remission with a coincident disappearance of obstructive intestinal symptoms. The pathogenesis of volvulus in sprue is poorly understood. Atonicity and dilatation of the bowel and stretching of the mesentery likely represent important factors. The symptoms of recurrent abdominal pain and distension in idiopathic steatorrhea necessitate an increased awareness of intestinal volvulus as a complication of this disease. ImagesFig. 1Fig. 2Fig. 3Figs. 4 and 5Fig. 6 PMID:13998948

Do all patients with idiopathic pulmonary fibrosis warrant a trial of therapeutic intervention? A pro-con perspective.

PubMed

Moodley, Yuben; Corte, Tamera; Richeldi, Luca; King, Talmadge E

2015-04-01

Idiopathic pulmonary fibrosis (IPF) is an incurable condition that is characterized by progressive pulmonary fibrosis, architectural distortion of the lung and loss of gas exchange units. Until recently, there was no effective treatment for this condition. However, there were two landmark trials published earlier this year, which have changed the management of this condition. Pirfenidone (Assessment of Pirfenidone to Confirm Efficacy and Safety in Idiopathic Pulmonary Fibrosis trial) and nintedanib (Efficacy and Safety of Nintedanib in Idiopathic Pulmonary Fibrosis-1 and -2 trials) have both demonstrated positive outcomes in patients with IPF. In this perspective, we critically discuss the role of these agents in IPF and in the broader pulmonary fibrosis population. © 2015 Asian Pacific Society of Respirology.

Serum Levels of 25-hydroxyvitamin D in Chronic Urticaria and its Association with Disease Activity: A Case Control Study

PubMed Central

Rather, Shagufta; Keen, Abid; Sajad, Peerzada

2018-01-01

Aim: To evaluate the relationship between vitamin D levels and chronic spontaneous urticaria (CSU) and compare with healthy age and sex matched controls. Material and Methods: This was a hospital-based cross-sectional study conducted over a period of 1 year, in which 110 patients with CSU were recruited along with an equal number of sex and age-matched healthy controls. For each patient, urticaria activity score (UAS) was calculated and autologous serum skin test (ASST) was performed. Plasma 25-hydroxyvitamin D [25-(OH)D] was analyzed by chemiluminescence method. A deficiency in vitamin D was defined as serum 25-(OH)D concentrations <30 ng/mL. The statistical analysis was carried out by using appropriate statistical tests. Results: The mean serum 25-(OH)D levels of CSU patients was 19.6 ± 6.9 ng/mL, whereas in control group, the mean level was 38.5 ± 6.7, the difference being statistically significant (P < 0.001). A significant negative correlation was found between vitamin D levels and UAS. (P < 0.001). The number of patients with ASST positivity was 44 (40%). Conclusion: The patients with CSU had reduced levels of vitamin D when compared to healthy controls. Furthermore, there was a significant negative correlation between the levels of serum vitamin D and severity of CSU. PMID:29854636

[Idiopathic sudden deafness: a report of 96 patients].

PubMed

Gabanou, F; Bera, G; Vincent, C

2012-01-01

Evaluation of the management of idiopathic sudden deafness indicating the usefulness of biological assessments and the pronostic factors of hearing recovery. This is a retrospective study of 96 patients with idiopathic sudden deafness referred to a tertiary centre between 2005 and 2009 treated with corticosteroids intravenously at a daily dose of 1 mg/kg. Mean tonal thresholds were assessed (PTA = [500 Hz + 1000 Hz + 2000 Hz + 4000 Hz]/4). Each audiogram was classified as five classes according to its frequency profile. The hearing recovery is significant between D0-D5 and D5-M1 for the frequencies 0.5, 1 and 2 kHz. For 4 kHz, the recovery is significant between 0 and J5. There is no statistically significant correlation between the presence of associated signs (tinnitus, vertigo) and hearing recovery. Hearing recovery according to the five types of audiograms has the same evolution in the follow-up time but with audiograms type E (cophosis or subcophosis) often associated with an hyporeactivity at the videonystagmography. The presence of cardiovascular disease is a predictor of poor hearing recovery. The usefullness of systematic extensive blood tests is low. In sudden deafness, the maximum hearing recovery takes place in the month following the onset of symptoms. The predictors of poor hearing recovery are an initial mean threshold > 70 dB, the existence of an associated cardiovascular disease.

Effectiveness of Chêneau brace treatment for idiopathic scoliosis: prospective study in 79 patients followed to skeletal maturity

PubMed Central

2011-01-01

Background Progressive idiopathic scoliosis can negatively influence the development and functioning of 2-3% of adolescents, with health consequences and economic costs, placing the disease in the centre of interest of the developmental medicine. The aim of this study was to evaluate the effectiveness of Chêneau brace in the management of idiopathic scoliosis. Methods A prospective observational study according to SOSORT and SRS recommendations comprised 79 patients (58 girls and 21 boys) with progressive idiopathic scoliosis, treated with Chêneau brace and physiotherapy, with initial Cobb angle between 20 and 45 degrees, no previous brace treatment, Risser 4 or more at the final evaluation and minimum one year follow-up after weaning the brace. Achieving 50° of Cobb angle was considered surgical recommendation. Results At follow-up 20 patients (25.3%) improved, 18 patients (22.8%) were stable, 31 patients (39.2%) progressed below 50 degrees and 10 patients (12.7%) progressed beyond 50 degrees (2 of these 10 patients progressed beyond 60 degrees). Progression concerned the younger and less skeletally mature patients. Conclusion Conservative treatment with Chêneau orthosis and physiotherapy was effective in halting scoliosis progression in 48.1% of patients. The results of this study suggest that bracing is effective in reducing the incidence of surgery in comparison with natural history. PMID:21266084

Three-year outcome of shunted idiopathic NPH patients.

PubMed

Aygok, G; Marmarou, A; Young, H F

2005-01-01

The incidence of idiopathic normal pressure hydrocephalus (iNPH) has increased as a result of improved longevity. This report describes the 3-year outcome of shunted iNPH patients compared to three-month outcome after shunting. Patients (n = 50) (Age 70.4 +/- 8.9) admitted to our service were diagnosed and treated according to a fixed protocol for management of iNPH and after shunting were followed at least three times per year in clinic. The outcome of 50 patients was graded according to the level of improvement in symptoms as Excellent/Good, Partial or None in each category of Gait, Incontinence and Dementia. If we lump favorable (excellent, good, partial recovery) vs poor recovery (none), we found from 3 months to 3 years, a moderate decline in gait performance (91% to 75%), a retention of memory improvement (80%-80%) and an improvement in incontinence occurred over time (70%-82.5%). With proper diagnosis and management of iNPH, shunting of patients is associated with a favorable risk/benefit ratio that is reasonably long lasting.

[Abnormal growth of spine in patients with adolescent idiopathic thoracic scoliosis].

PubMed

Bao, Hongda; Liu, Zhen; Qiu, Yong; Zhu, Feng; Zhu, Zezhang; Zhang, Wen

2014-05-01

To investigate if the growth patterns of the spine and pelvis are consistent in adolescent idiopathic scoliosis (AIS) patients with single thoracic curves. Forty-eight thoracic adolescent idiopathic scoliosis (T-AIS) female patients and 48 healthy age-matched adolescents were recruited consecutively between December 2011 and October 2012. Radiographic parameters including height of spine (HOS), length of spine (LOS), height of thoracic spine (HOT), length of thoracic spine (LOT), height of pelvis (HOP), width of pelvis (WOP) and width of thorax (WOT) were measured on the long-cassette posteroanterior standing radiographs. In addition, ratios including HOS/HOP, LOS/HOP, HOT/HOP, LOT/HOP, LOS/LOT, WOT/WOP were also calculated. Independent t-test was performed to compare the radiographic parameters and ratios between the two groups. Compared to the age-matched healthy adolescents, T-AIS patients had a significantly higher LOS and LOT (t = -2.364 and -1.495, P = 0.020 and 0.043) and smaller HOS and HOT (t = 2.060 and 3.359, P = 0.042 and 0.001). Yet, all of HOP, WOP and WOT showed no significant difference between T-AIS patients and healthy adolescents. Similarly, LOS/HOP and LOT/HOP were significantly higher in T-AIS patients as may be expected with an average LOS/HOP of 2.26 ± 0.14 in normal controls.In addition, LOS/LOT in normal controls had a trend of increase with age which was different from the stable LOS/LOT in T-AIS patients, indicating an increased growth of thoracic vertebra compared to lumbar vertebra. Compared to the age-matched healthy adolescents, T-AIS patients have an abnormal growth characteristics with longer spine. The growth of pelvis and thorax show no significant differences between T-AIS patients and healthy adolescents.

Non-surgical periodontal therapy improves serum levels of C-reactive protein and edematous states in female patients with idiopathic edema.

PubMed

Joseph, Rosamma; Narayan, Vivek; Krishnan, Rajaratnam; Melemadathil, Sreelatha

2011-02-01

The relationship between periodontal disease and systemic disease is revealing new and exciting associations. Idiopathic edema presents a clinical syndrome with obscure pathophysiology. The present study investigates whether non-surgical periodontal therapy is beneficial in patients who are not responding to conventional treatment of idiopathic edema. Thirty patients with idiopathic edema were allocated to intervention and control groups. All the subjects were assessed for systemic (body weight, body mass index, visual scale of edema, serum C-reactive protein, and serum albumin) and periodontal (plaque index, calculus index, and gingival index) parameters. Non-surgical periodontal therapy, including oral hygiene instructions, scaling and root planing, and systemic antibiotic therapy, was provided in the intervention group. Both groups were reevaluated after 4 weeks. Both groups were comparable at baseline. All parameters, except serum albumin, showed significant improvement after periodontal therapy. The control group showed further worsening of these parameters. This study shows that sources for systemic inflammation, such as periodontal disease, could affect the pathogenesis of idiopathic edema. Successful elimination of such covert sources of inflammation leads to a clinical benefit in patients who are distressed by this condition.

Treatment of idiopathic pulmonary fibrosis with losartan: a pilot project.

PubMed

Couluris, Marisa; Kinder, Brent W; Xu, Ping; Gross-King, Margaret; Krischer, Jeffrey; Panos, Ralph J

2012-10-01

Idiopathic pulmonary fibrosis is a progressive interstitial lung disease with no current effective therapies. Treatment has focused on antifibrotic agents to stop proliferation of fibroblasts and collagen deposition in the lung. We present the first clinical trial data on the use of losartan, an antifibrotic agent, to treat idiopathic pulmonary fibrosis. The primary objective was to evaluate the effect of losartan on progression of idiopathic pulmonary fibrosis measured by the change in percentage of predicted forced vital capacity (%FVC) after 12 months. Secondary outcomes included the change in forced expiratory volume at 1 second, diffusing capacity of carbon monoxide, 6-minute walk test distance, and baseline/transition dyspnea index. Patients with idiopathic pulmonary fibrosis and a baseline %FVC of ≥50 % were treated with losartan 50 mg by mouth daily for 12 months. Pulmonary function testing, 6-minute walk, and breathlessness indices were measured every 3 months. Twenty participants with idiopathic pulmonary fibrosis were enrolled and 17 patients were evaluable for response. Twelve patients had a stable or improved %FVC at study month 12. Similar findings were observed in secondary end-point measures, including 58, 71, and 65 % of patients with stable or improved forced expiratory volume at 1 second, diffusing capacity for carbon monoxide, and 6-minute walk test distance, respectively. No treatment-related adverse events that resulted in early study discontinuation were reported. Losartan stabilized lung function in patients with idiopathic pulmonary fibrosis over 12 months. Losartan is a promising agent for the treatment of idiopathic pulmonary fibrosis and has a low toxicity profile.

Chronic urticaria in adults: state-of-the-art in the new millennium*

PubMed Central

Criado, Paulo Ricardo; Criado, Roberta Facchini Jardim; Maruta, Celina Wakisaka; dos Reis, Vitor Manoel Silva

2015-01-01

Chronic urticaria has been explored in several investigative aspects in the new millennium, either as to its pathogenesis, its stand as an autoimmune or auto-reactive disease, the correlation with HLA-linked genetic factors, especially with class II or its interrelation with the coagulation and fibrinolysis systems. New second-generation antihistamines, which act as good symptomatic drugs, emerged and were commercialized over the last decade. Old and new drugs that may interfere with the pathophysiology of the disease, such as cyclosporine and omalizumab have been developed and used as treatments. The purpose of this article is to describe the current state of knowledge on aspects of chronic urticaria such as, pathophysiology, diagnosis and the current therapeutic approach proposed in the literature. PMID:25672302

Urinary metabolites of histamine and leukotrienes before and after placebo-controlled challenge with ASA and food additives in chronic urticaria patients.

PubMed

Di Lorenzo, G; Pacor, M L; Vignola, A M; Profita, M; Esposito-Pellitteri, M; Biasi, D; Corrocher, R; Caruso, C

2002-12-01

The recovery of mediator metabolites from urine has the potential to provide a rapid, safe, and easily available index of release of mediators. We aimed to determine urinary metabolites of both histamine and leukotrienes (LTs) in patients affected by chronic urticaria (CU). Twenty patients with CU were studied. They were selected on the basis of double-blind placebo-controlled challenge (DBPC) with acetyl salicylic acid (ASA) and food additives. Ten patients (group B) were negative to both challenges. Ten patients (group C) presented urticaria and/or the appearance of angioedema during or 24 h after challenge, with reactions to ASA (five patients) or food additives (five patients). We recruited 15 healthy volunteers as controls (group A). During a second challenge, groups B and C were challenged double-blind with a single dose of ASA, or a specific food additive, or placebo. The healthy group was challenged only with a placebo (talc capsule). Patients in groups B and C were challenged twice: with placebo (as groups B1 and C1) and with ASA (groups B2 and C2) or food additives (C2). Four samples of urine were collected; one during the night before the specific or sham challenge (baseline), and three at 2, 6 and 24 h after the challenge. Urinary methylhistamine (N-MH) and LTE4 were analyzed and normalized for urinary creatinine. For urinary N-MH at baseline, there was a significant difference only between group A and groups B1, B2, C1 and C2 (A vs. B1, P < 0.0001; A vs. B2, P < 0.0001; A vs. C1, P < 0.0001; A vs. C2, P < 0.0001). We detected a significant variation in urinary methylhistamine excretion only in group C2 after 2 h, 6 h and 24 h (P < 0.0001). However, no variations were observed in N-MH excretion rate in the other groups (A, B1, C1) after challenge with placebo, and in B2 after challenge with ASA 20 mg. For urinary LTE4 at baseline no differences were found between the mean values for the different groups. After specific challenge, only C2 patients showed

The EAACI/GA(2) LEN/EDF/WAO Guideline for the definition, classification, diagnosis, and management of urticaria: the 2013 revision and update.

PubMed

Zuberbier, T; Aberer, W; Asero, R; Bindslev-Jensen, C; Brzoza, Z; Canonica, G W; Church, M K; Ensina, L F; Giménez-Arnau, A; Godse, K; Gonçalo, M; Grattan, C; Hebert, J; Hide, M; Kaplan, A; Kapp, A; Abdul Latiff, A H; Mathelier-Fusade, P; Metz, M; Nast, A; Saini, S S; Sánchez-Borges, M; Schmid-Grendelmeier, P; Simons, F E R; Staubach, P; Sussman, G; Toubi, E; Vena, G A; Wedi, B; Zhu, X J; Maurer, M

2014-07-01

This guideline is the result of a systematic literature review using the 'Grading of Recommendations Assessment, Development and Evaluation' (GRADE) methodology and a structured consensus conference held on 28 and 29 November 2012, in Berlin. It is a joint initiative of the Dermatology Section of the European Academy of Allergy and Clinical Immunology (EAACI), the EU-funded network of excellence, the Global Allergy and Asthma European Network (GA(2) LEN), the European Dermatology Forum (EDF), and the World Allergy Organization (WAO) with the participation of delegates of 21 national and international societies. Urticaria is a frequent, mast cell-driven disease, presenting with wheals, angioedema, or both. The life-time prevalence for acute urticaria is approximately 20%. Chronic spontaneous urticaria and other chronic forms of urticaria do not only cause a decrease in quality of life, but also affect performance at work and school and, as such, are members of the group of severe allergic diseases. This guideline covers the definition and classification of urticaria, taking into account the recent progress in identifying its causes, eliciting factors and pathomechanisms. In addition, it outlines evidence-based diagnostic and therapeutic approaches for the different subtypes of urticaria. This guideline was acknowledged and accepted by the European Union of Medical Specialists (UEMS). © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Idiopathic ventricular tachycardia and fibrillation.

PubMed

Belhassen, B; Viskin, S

1993-06-01

Important data have recently been added to our understanding of sustained ventricular tachyarrhythmias occurring in the absence of demonstrable heart disease. Idiopathic ventricular tachycardia (VT) is usually of monomorphic configuration and can be classified according to its site of origin as either right monomorphic (70% of all idiopathic VTs) or left monomorphic VT. Several physiopathological types of monomorphic VT can be presently individualized, according to their mode of presentation, their relationship to adrenergic stress, or their response to various drugs. The long-term prognosis is usually good. Idiopathic polymorphic VT is a much rarer type of arrhythmia with a less favorable prognosis. Idiopathic ventricular fibrillation may represent an underestimated cause of sudden cardiac death in ostensibly healty patients. A high incidence of inducibility of sustained polymorphic VT with programmed ventricular stimulation has been found by our group, but not by others. Long-term prognosis on Class IA antiarrhythmic medications that are highly effective at electrophysiologic study appears excellent.

High-resolution manometry classifications for idiopathic achalasia in patients with Chagas' disease esophagopathy.

PubMed

Vicentine, Fernando P P; Herbella, Fernando A M; Allaix, Marco E; Silva, Luciana C; Patti, Marco G

2014-02-01

Idiopathic achalasia (IA) and Chagas' disease esophagopathy (CDE) share several similarities. The comparison between IA and CDE is important to evaluate whether treatment options and their results can be accepted universally. High-resolution manometry (HRM) has proved a better diagnostic tool compared to conventional manometry. This study aims to evaluate HRM classifications for idiopathic achalasia in patients with CDE. We studied 98 patients: 52 patients with CDE (52 % females, mean age, 57 ± 14 years) and 46 patients with IA (54 % females; mean age 48 ± 19 years). All patients underwent a HRM and barium esophagogram. The Chicago classification was distributed in IA as Chicago I, 35 %; Chicago II, 63 %; and Chicago III, 2 %, and in CDE as Chicago I, 52 %; Chicago II, 48 %; and Chicago III, 0 % (p = 0.1, 0.1, and 0.5, respectively). All patients had the classic Rochester type. CDE patients had more pronounced degrees of esophageal dilatation (p < 0.002). The degree of esophageal dilatation did not correlate with Chicago classification (p = 0.08). In nine (9 %) patients, the HRM pattern changed during the test from Chicago I to II. Our results show that (a) HRM classifications for IA can be applied in patients with CDE and (b) HRM classifications did not correlate with the degree of esophageal dilatation. HRM classifications may reflect esophageal repletion and pressurization instead of muscular contraction. The correlation between manometric findings and treatment outcomes for CDE needs to be answered in the near future.

Incidental (malignancy) and coincidental (idiopathic polydactylous longitudinal erythronychia) conditions in patients with segmental neurofibromatosis.

PubMed

Cohen, Philip R

2013-04-01

Segmental neurofibromatosis (SNF) is an uncommon presentation of neurofibromatosis type 1 (NF-1). Although patients with SNF are at a lower risk for developing NF-l-associated complications, the estimated occurrence of related malignancies may be approaching the frequency observed in patients with NF-1. Idiopathic polydactylous longitudinal erythronychia also may be associated with SNF, though the frequency of this association remains to be determined.

Assessment of mental health of carers according to patient stage of idiopathic Parkinson's disease.

PubMed

Olgun Yazar, Hülya; Yazar, Tamer; Yancar Demir, Esra; Cankaya, Soner; Enginyurt, Özgür

2018-05-30

In this study the aim was to collect data to assess the mental health of carers for patients with diagnosis of idiopathic Parkinson's disease (IPD) according to disease stage and to examine precautions to reduce the patient and disease load on carers. The study included 144 patients with staging according to modified Hoehn and Yahr criteria and 144 patient relatives who provided care support for patients every day, for some or all of the day, and who were over the age of 18 years and accepted participation in the research. Our prospective and cross-sectional study performed detailed neurological examination of patients, and after completing the 'Personal Information Form' with the interviewer every patient, with idiopathic Parkinson's disease (IPD) according to 'UK Brain Bank' diagnostic criteria, had the 'Unified Parkinson's Disease Rating Scale (UPDRS)' and 'Modified Hoehn and Yahr scale (HYS)' applied. Carers first completed the 'Personal Information Form' and then had the 'Short Symptom Inventory (SSI)' applied. As the stage of disease increased, the points for all sub-scales of the Short Symptom Inventory increased. With the parallel increase in disease scores and UPDRS stage scores, the points obtained by carers on the SSI sub-scales increased. This data shows that with progressing disease stage, the load on the carer increases and mental health begins to be disrupted.

Quantification of topographic changes in the surface of back of young patients monitored for idiopathic scoliosis: correlation with radiographic variables

NASA Astrophysics Data System (ADS)

Pino-Almero, Laura; Mínguez-Rey, María Fe; Sentamans-Segarra, Salvador; Salvador-Palmer, María Rosario; Anda, Rosa María Cibrián-Ortiz de; La O, Javier López-de

2016-11-01

Idiopathic scoliosis requires a close follow-up while the patient is skeletally immature to detect early progression. Patients who are monitored by radiographs are exposed to high doses of ionizing radiation. The purpose of this study is to evaluate if an optic noninvasive method of back surface topography based on structured light would be clinically useful in the follow-up of young patients with idiopathic scoliosis. This could reduce the number of radiographs made on these children. Thirty-one patients with idiopathic scoliosis were submitted twice to radiograph and our topographic method at intervals of 6 months to 1 year. Three topographical variables were applied horizontal plane deformity index (DHOPI), posterior trunk symmetry index (POTSI), and columnar profile (PC). A statistically significant correlation was found between variations of Cobb angle with DHOPI (r=0.720, p<0.01) and POTSI (r=0.753, p<0.01) during the monitoring period. Hence, this topographic method could be useful in clinical practice as an objective adjuvant tool in routine follow-up of scoliosis.

Long-term safety and effectiveness of lubiprostone, a chloride channel (ClC-2) activator, in patients with chronic idiopathic constipation.

PubMed

Lembo, Anthony J; Johanson, John F; Parkman, Henry P; Rao, Satish S; Miner, Philip B; Ueno, Ryuji

2011-09-01

Lubiprostone helps relieve constipation in short-term 4-week studies. There are limited data on long-term pharmacological treatment with lubiprostone for chronic idiopathic constipation. To examine the long-term safety and effectiveness of lubiprostone in patients with chronic idiopathic constipation. In this prospective, multicenter, open-labeled trial, 248 patients aged ≥18 years with chronic idiopathic constipation were directed to take lubiprostone 24 mcg BID as needed for 48 weeks. Patients were allowed to decrease the dose in response to the perceived severity of constipation and need for relief. Hematology and chemistry profiles and assessment of constipation symptoms and its severity were performed at all visits. Adverse events (AEs) were recorded. Of the 248 patients who entered the trial, 127 (51%) completed the trial. A dose reduction was observed in 17% of the patients, resulting in an average study medication exposure across the study of approximately 1.7 capsules (or approximately 40.8 mcg) per day. The most common treatment-related AEs were nausea (19.8%), diarrhea (9.7%), abdominal distension (6.9%), headache (6.9%), and abdominal pain (5.2%). No deaths were reported and of the 16 reported serious AEs, one was considered possibly treatment related. Average changes in serum electrolytes were not clinically relevant at any time point during the study. On average, lubiprostone significantly (p < 0.0001) reduced patient-reported constipation severity, abdominal bloating, and abdominal discomfort across 48 weeks when compared to baseline. During this 48-week open-label study, lubiprostone was well tolerated. Bowel symptoms consistently improved over 48 weeks in adult patients with chronic idiopathic constipation.

Relation between adolescent idiopathic scoliosis and morphologic somatotypes.

PubMed

LeBlanc, R; Labelle, H; Rivard, C H; Poitras, B

1997-11-01

A prospective and controlled comparative study. To verify the difference in morphologic appearance between a group of adolescents with progressive adolescent idiopathic scoliosis and a control group of normal adolescents. In a previous retrospective study, the possibility of a relation between progressive adolescent idiopathic scoliosis and specific morphotypes was demonstrated. Fifty-two adolescent girls with progressive adolescent idiopathic scoliosis were compared with an age-matched control group of 62 unaffected girls using a classification technique based on morphologic somatotypes. Morphotypes were evaluated with standardized pre-established criteria based on Sheldon's technique. Patients with progressive adolescent idiopathic scoliosis showed significantly less mesomorphism (mean value of 0.88 +/- 0.51) than control girls (mean value of 1.72 +/- 0.52). Adolescent girls with progressive adolescent idiopathic scoliosis have a morphologic somatotype that is different from the normal adolescent population. Subjects with progressive adolescent idiopathic scoliosis are significantly less mesomorphic than control girls. This observation may be of value as a predictive factor for early identification of subjects with adolescent idiopathic scoliosis at greater risk of progression.

[Efficacy and safety of astemizole in the treatment of allergic rhinitis and urticaria: a systematic review with meta-analysis].

PubMed

Gutiérrez Castrellón, Pedro; Ramírez Mayans, Jaime; López Pérez, Gerardo

2005-01-01

To compare astemizole with other first or second generation antihistaminics in the treatment of allergic rhinitis or urticaria. Systematic revision of clinical, controlled and randomized tests. 36 controlled, randomized, clinical, double or simple blind tests were made in 6,446 patients; 4,513 of them were assigned to astemizole and 1,933 to other first or second generation antihistaminics. Analyzed outcomes: Rate of global success; global scoring improvement of rhinitis; ocular, nasal or pharingeal pruritus; watering; nasal obstruction; sneeze; urticaria; cutaneous response to histamine; time to get a satisfactory improvent; frequency of sedation and headache. In connection to global success there were significant differences in favor of astemizole (OR 6.72, CI95% 5.36 to 8.41, p 0.0001); alike global scoring improvement of rhinitis (SMD -0.82, CI95% -1.70 to 0.06, p 0.04); rhinorrhea (SMD of -0.70, CI95% -1.47 to -0.03, p 0.02); nasal, pharingeal or ocular pruritus (SMD -0.64, CI95% -1.63 to 0.35, p 0.03); urticaria (SMD of -3.53, CI95% -4.11 to -2.94, p 0.0001), and reduction of cutaneous response to histamine (SMD -2.02, CI95% -2.47 to -1.57, p 0.0001). Differences for watering, nasal obstruction or sneeze were not observed. Finally, the safety area was analyzed considering the existence of sedation and headache; it was demonstrated less sedation (OR 0.23, CI95% 0.18 to 0.30, p 0.0001) and less headache (OR 0.58, CI95% 0.40 to 0.85, p 0.005) in the group treated with astemizole. The available evidence shows a therapeutic superiority of astemizole versus other antihistaminics in relation to the percentage of global success and the global scoring improvement of rhinitis, rhinorrhea, pruritus in general, urticaria symptoms and of the reduction of response to histamine, with a smaller frequency of headache or sedation.

Lung Ultrasonography in Patients With Idiopathic Pulmonary Fibrosis: Evaluation of a Simplified Protocol With High-Resolution Computed Tomographic Correlation.

PubMed

Vassalou, Evangelia E; Raissaki, Maria; Magkanas, Eleftherios; Antoniou, Katerina M; Karantanas, Apostolos H

2018-03-01

To compare a simplified ultrasonographic (US) protocol in 2 patient positions with the same-positioned comprehensive US assessments and high-resolution computed tomographic (CT) findings in patients with idiopathic pulmonary fibrosis. Twenty-five consecutive patients with idiopathic pulmonary fibrosis were prospectively enrolled and examined in 2 sessions. During session 1, patients were examined with a US protocol including 56 lung intercostal spaces in supine/sitting (supine/sitting comprehensive protocol) and lateral decubitus (decubitus comprehensive protocol) positions. During session 2, patients were evaluated with a 16-intercostal space US protocol in sitting (sitting simplified protocol) and left/right decubitus (decubitus simplified protocol) positions. The 16 intercostal spaces were chosen according to the prevalence of idiopathic pulmonary fibrosis-related changes on high-resolution CT. The sum of B-lines counted in each intercostal space formed the US scores for all 4 US protocols: supine/sitting and decubitus comprehensive US scores and sitting and decubitus simplified US scores. High-resolution CT-related Warrick scores (J Rheumatol 1991; 18:1520-1528) were compared to US scores. The duration of each protocol was recorded. A significant correlation was found between all US scores and Warrick scores and between simplified and corresponding comprehensive scores (P < .0001). Decubitus simplified US scores showed a slightly higher correlation with Warrick scores compared to sitting simplified US scores. Mean durations of decubitus and sitting simplified protocols were 4.76 and 6.20 minutes, respectively (P < .005). Simplified 16-intercostal space protocols correlated with comprehensive protocols and high-resolution CT findings in patients with idiopathic pulmonary fibrosis. The 16-intercostal space simplified protocol in the lateral decubitus position correlated better with high-resolution CT findings and was less time-consuming compared to the

Chronic Non-infectious Uveitis in Patients with Juvenile Idiopathic Arthritis.

PubMed

Kolomeyer, Anton M; Tu, Yufei; Miserocchi, Elisabetta; Ranjan, Mangala; Davidow, Amy; Chu, David S

2016-08-01

To describe clinical findings and analyze treatment evolution of chronic, non-infectious uveitis in patients with juvenile idiopathic arthritis (JIA). A total of 82 patients (147 eyes) with JIA-related uveitis treated for ≥2 months were included (78% females; 79% bilateral uveitis; 74% anterior uveitis). Outcome measures were visual acuity (VA), inflammation control, side-effects, and surgical procedures. Mean ± SD age at diagnosis was 4.9 ± 3.8 years; mean ± SD follow-up time was 8.7 ± 7.8 years. Mean VA did not significantly change throughout the study. Three (2%) eyes resulted in no light perception (NLP) vision. Thirty (37%) patients underwent 69 procedures. In total, 41 (50%) patients achieved inflammation control. TNF-α inhibitors were significantly associated with inflammation control. Seven (8.5%) patients stopped treatment due to side-effects. JIA is a cause of significant ocular morbidity. TNF-α inhibitor use was associated with inflammation control. Prospective, randomized, double blind clinical trials in this regard are warranted.

Interventional Radiologic Treatment for Idiopathic Portal Hypertension

DOE Office of Scientific and Technical Information (OSTI.GOV)

Hirota, Shozo; Ichikawa, Satoshi; Matsumoto, Shinichi

1999-07-15

Purpose: To evaluate the usefulness of interventional radiological treatment for idiopathic portal hypertension. Methods: Between 1995 and 1998, we performed an interventional radiological treatment in five patients with idiopathic portal hypertension, four of whom had refused surgery and one of whom had undergone surgery. Three patients with gastroesophageal varices (GEV) were treated by partial splenic embolization (PSE), one patient with esophageal varices (EV) and massive ascites by transjugular intrahepatic portosytemic shunt (TIPS) and PSE, and one patient with GEV by percutaneous transhepatic obliteration (PTO). Midterm results were analyzed in terms of the effect on esophageal and/or gastric varices. Results: Inmore » one woman with severe GEV who underwent three sessions of PSE, there was endoscopic confirmation that the GEV had disappeared. In one man his EV shrunk markedly after two sessions of PSE. In two patients slight reduction of the EV was obtained with one application of PSE combined with endoscopic variceal ligation therapy. PTO for GV in one patient resulted in good control of the varices. All patients have survived for 16-42 months since the first interventional treatment, and varices are well controlled. Conclusion: Interventional radiological treatment is effective for patients with idiopathic portal hypertension, whether or not they have undergone surgery.« less

Successful treatment with levothyroxine for idiopathic hypersomnia patients with subclinical hypothyroidism.

PubMed

Shinno, Hideto; Inami, Yasushi; Inagaki, Takuji; Kawamukai, Tetsuya; Utani, Etsuko; Nakamura, Yu; Horiguchi, Jun

2009-01-01

Our objective was to discuss the effect of levothyroxine on excessive daytime sleepiness (EDS) and a prolonged nocturnal sleep at patients with idiopathic hypersomnia who presented with subclinical hypothyroidism. We present two patients with hypersomnia who complained of EDS and a prolonged nocturnal sleep time. Sleep architecture and subjective daytime sleepiness were estimated by polysomnography (PSG) and Epworth Sleepiness Scale (ESS), respectively. Diagnoses were made using the International Classification of Sleep Disorders, 2nd Edition criteria for idiopathic hypersomnia with long sleep time. PSG demonstrated a short sleep latency, a prolonged total sleep time and normal proportions of all non-rapid eye movement (REM) and REM sleep stages. Nocturnal PSG excluded other causes of EDS. No medical, neurological and mental disorders were present. Their laboratory data indicated mildly elevated thyrotropin, despite free thyroxine (T4) and triiodothyronine (T3) estimates within their reference ranges, which is a characteristic of latent hypothyroidism. Levothyroxine (25 microg/day) was administrated orally. After treatment with levothyroxine for 8 weeks, the mean daily sleep times decreased. EDS was also improved, and a significant decrease in the ESS score was observed. Levothyroxine was effective for their hypersomnia and well tolerated. It should be noted that hypersomnia may be associated with subclinical hypothyroidism, although few abnormalities in physical and neurological examinations are present.

Fluid Distribution Pattern in Adult-Onset Congenital, Idiopathic, and Secondary Normal-Pressure Hydrocephalus: Implications for Clinical Care.

PubMed

Yamada, Shigeki; Ishikawa, Masatsune; Yamamoto, Kazuo

2017-01-01

In spite of growing evidence of idiopathic normal-pressure hydrocephalus (NPH), a viewpoint about clinical care for idiopathic NPH is still controversial. A continuous divergence of viewpoints might be due to confusing classifications of idiopathic and adult-onset congenital NPH. To elucidate the classification of NPH, we propose that adult-onset congenital NPH should be explicitly distinguished from idiopathic and secondary NPH. On the basis of conventional CT scan or MRI, idiopathic NPH was defined as narrow sulci at the high convexity in concurrent with enlargement of the ventricles, basal cistern and Sylvian fissure, whereas adult-onset congenital NPH was defined as huge ventricles without high-convexity tightness. We compared clinical characteristics and cerebrospinal fluid distribution among 85 patients diagnosed with idiopathic NPH, 17 patients with secondary NPH, and 7 patients with adult-onset congenital NPH. All patients underwent 3-T MRI examinations and tap-tests. The volumes of ventricles and subarachnoid spaces were measured using a 3D workstation based on T2-weighted 3D sequences. The mean intracranial volume for the patients with adult-onset congenital NPH was almost 100 mL larger than the volumes for patients with idiopathic and secondary NPH. Compared with the patients with idiopathic or secondary NPH, patients with adult-onset congenital NPH exhibited larger ventricles but normal sized subarachnoid spaces. The mean volume ratio of the high-convexity subarachnoid space was significantly less in idiopathic NPH than in adult-onset congenital NPH, whereas the mean volume ratio of the basal cistern and Sylvian fissure in idiopathic NPH was >2 times larger than that in adult-onset congenital NPH. The symptoms of gait disturbance, cognitive impairment, and urinary incontinence in patients with adult-onset congenital NPH tended to progress more slowly compared to their progress in patients with idiopathic NPH. Cerebrospinal fluid distributions and

Vitamin D in Atopic Dermatitis, Chronic Urticaria and Allergic Contact Dermatitis

PubMed Central

Quirk, Shannon K; Rainwater, Ellecia; Shure, Anna K; Agrawal, Devendra K

2016-01-01

Summary Vitamin D influences allergen-induced pathways in the innate and adaptive immune system, and its potential immunomodulatory role in allergic skin disorders has been explored. This comprehensive review article provides an overview of the role of vitamin D in three common dermatologic conditions: atopic dermatitis (AD), chronic urticaria, and allergic contact dermatitis (ACD). Whereas the literature regarding vitamin D and AD has resulted in mixed findings, several studies have described an inverse relationship between vitamin D levels and AD severity, and improvement in AD with vitamin D supplementation. Similarly, several studies report an inverse relationship between vitamin D levels and severity of chronic urticaria. Although current research in humans remains limited, an increased likelihood of ACD has been demonstrated in vitamin D-deficient mice. Additional well-designed clinical trials will be necessary to determine whether vitamin D supplementation should be recommended for prevention or adjuvant treatment of these common dermatologic conditions. PMID:27014952

An Evaluation of Factors Associated With Pathogenic PRSS1, SPINK1, CTFR, and/or CTRC Genetic Variants in Patients With Idiopathic Pancreatitis.

PubMed

Jalaly, Niloofar Y; Moran, Robert A; Fargahi, Farshid; Khashab, Mouen A; Kamal, Ayesha; Lennon, Anne Marie; Walsh, Christi; Makary, Martin A; Whitcomb, David C; Yadav, Dhiraj; Cebotaru, Liudmila; Singh, Vikesh K

2017-08-01

We evaluated factors associated with pathogenic genetic variants in patients with idiopathic pancreatitis. Genetic testing (PRSS1, CFTR, SPINK1, and CTRC) was performed in all eligible patients with idiopathic pancreatitis between 2010 to 2015. Patients were classified into the following groups based on a review of medical records: (1) acute recurrent idiopathic pancreatitis (ARIP) with or without underlying chronic pancreatitis; (2) idiopathic chronic pancreatitis (ICP) without a history of ARP; (3) an unexplained first episode of acute pancreatitis (AP)<35 years of age; and (4) family history of pancreatitis. Logistic regression analysis was used to determine the factors associated with pathogenic genetic variants. Among 197 ARIP and/or ICP patients evaluated from 2010 to 2015, 134 underwent genetic testing. A total of 88 pathogenic genetic variants were found in 64 (47.8%) patients. Pathogenic genetic variants were identified in 58, 63, and 27% of patients with ARIP, an unexplained first episode of AP <35 years of age, and ICP without ARP, respectively. ARIP (OR: 18.12; 95% CI: 2.16-151.87; P=0.008) and an unexplained first episode of AP<35 years of age (OR: 2.46; 95% CI: 1.18-5.15; P=0.017), but not ICP, were independently associated with pathogenic genetic variants in the adjusted analysis. Pathogenic genetic variants are most likely to be identified in patients with ARIP and an unexplained first episode of AP<35 years of age. Genetic testing in these patient populations may delineate an etiology and prevent unnecessary diagnostic testing and procedures.

Update on the Treatment of Uveitis in Patients with Juvenile Idiopathic Arthritis: A Review.

PubMed

Asproudis, Ioannis; Katsanos, Andreas; Kozeis, Nikolaos; Tantou, Alexandra; Konstas, Anastasios G

2017-12-01

Chronic uveitis is a common extra-articular manifestation of juvenile idiopathic arthritis. The classic clinical picture is one of chronic anterior uveitis, which usually remains asymptomatic until ocular complications arise. The risk of uveitis is increased in girls with an early onset of oligoarthritis and positive antinuclear antibodies. Even though the inflammation in patients with juvenile idiopathic arthritis is initially limited in the anterior part of the eye, chronic active inflammation may eventually cause significant damage to the posterior pole. Complications may include band keratopathy, cataract, secondary glaucoma, posterior synechiae, cystoid macular edema, and hypotony. The cooperation of ophthalmologists with rheumatologists may help define the best treatment plan. The ophthalmic therapeutic regimen includes topical corticosteroids and mydriatics, while in severe cases immunosuppressive and biological agents are introduced. Surgical management of complications might be needed.

Idiopathic Hypersomnia with and without Long Sleep Time: A Controlled Series of 75 Patients

PubMed Central

Vernet, Cyrille; Arnulf, Isabelle

2009-01-01

Objective: To characterize the clinical, psychological, and sleep pattern of idiopathic hypersomnia with and without long sleep time, and provide normative values for 24-hour polysomnography. Setting: University Hospital Design: Controlled, prospective cohort Participants: 75 consecutive patients (aged 34 ± 12 y) with idiopathic hypersomnia and 30 healthy matched controls. Intervention: Patients and controls underwent during 48 hours a face-to face interview, questionnaires, human leukocyte antigen genotype, a night polysomnography and multiple sleep latency test (MSLT), followed by 24-h ad libitum sleep monitoring. Results: Hypersomniacs had more fatigue, higher anxiety and depression scores, and more frequent hypnagogic hallucinations (24%), sleep paralysis (28%), sleep drunkenness (36%), and unrefreshing naps (46%) than controls. They were more frequently evening types. DQB1*0602 genotype was similarly found in hypersomniacs (24.2%) and controls (19.2%). Hypersomniacs had more frequent slow wave sleep after 06:00 than controls. During 24-h polysomnography, the 95% confidence interval for total sleep time was 493–558 min in controls, versus 672–718 min in hypersomniacs. There were 40 hypersomniacs with and 35 hypersomniacs without long ( > 600 min) sleep time. The hypersomniacs with long sleep time were younger (29 ± 10 vs 40 ± 13 y, P = 0.0002), slimmer (body mass index: 26 ± 5 vs 23 ± 4 kg/m2; P = 0.005), and had lower Horne-Ostberg scores and higher sleep efficiencies than those without long sleep time. MSLT latencies were normal ( > 8 min) in 71% hypersomniacs with long sleep time. Conclusions: Hypersomnia, especially with long sleep time, is frequently associated with evening chronotype and young age. It is inadequately diagnosed using MSLT. Citation: Vernet C; Arnulf I. Idiopathic Hypersomnia with and without Long Sleep Time: A Controlled Series of 75 Patients. SLEEP 2009;32(6):753-759. PMID:19544751

Characteristics of Ocular Pain Complaints in Patients With Idiopathic Dry Eye Symptoms.

PubMed

Kalangara, Jerry P; Galor, Anat; Levitt, Roy C; Covington, Derek B; McManus, Katherine T; Sarantopoulos, Constantine D; Felix, Elizabeth R

2017-05-01

The purpose of this study was to examine the severity and quality of ocular pain complaints in patients with dry eye symptoms. Subjects with clinically relevant dry eye symptoms (dryness, discomfort, tearing) of unknown origin seen in the Miami Veterans Affairs eye clinic were administered questionnaires for dry eye symptoms and ocular pain and underwent a standardized ocular examination. Qualities and severity ratings of ocular pain in subjects with idiopathic dry eye were compared with similar measures from published data in other chronic pain populations. The study sample consisted of 154 subjects, of which 91% were men and ranged in age from 27 to 89 (mean age=61). Fifty-three percent of participants reported an average ocular pain of at least moderate intensity (numerical rating scale≥4), with specific characteristics (i.e., "burning" spontaneous pain) reported at frequencies comparable to prevalent chronic neuropathic pain syndromes as reported in the literature. Significant correlations were found between ocular pain metrics and dry eye symptom severity scores (r=0.57-0.66). Dry eye signs, however, did not generally correlate with ocular pain severity. A significant proportion of subjects with idiopathic dry eye symptoms reported moderate or greater ocular pain intensity, with most endorsing descriptors commonly used by patients with nonocular neuropathic pain conditions. Identifying subgroups of dry eye patients based on the presence and characteristics of ocular pain complaints may improve dry eye subclassification and better individualize treatment strategies.

A procedure to detect abnormal sensorimotor control in adolescents with idiopathic scoliosis.

PubMed

Pialasse, Jean-Philippe; Mercier, Pierre; Descarreaux, Martin; Simoneau, Martin

2017-09-01

This work identifies, among adolescents with idiopathic scoliosis, those demonstrating impaired sensorimotor control through a classification procedure comparing the amplitude of their vestibular-evoked postural responses. The sensorimotor control of healthy adolescents (n=17) and adolescents with idiopathic scoliosis (n=52) with either mild (Cobb angle≥15° and ≤30°) or severe (Cobb angle >30°) spine deformation was assessed through galvanic vestibular stimulation. A classification procedure sorted out adolescents with idiopathic scoliosis whether the amplitude of their vestibular-evoked postural response was dissimilar or similar to controls. Compared to controls, galvanic vestibular stimulation evoked larger postural response in adolescents with idiopathic scoliosis. Nonetheless, the classification procedure revealed that only 42.5% of all patients showed impaired sensorimotor control. Consequently, identifying patients with sensorimotor control impairment would allow to apply personalized treatments, help clinicians to establish prognosis and hopefully improve the condition of patients with adolescent idiopathic scoliosis. Copyright © 2017 Elsevier B.V. All rights reserved.

Influence of Pulmonary Hypertension on Patients With Idiopathic Pulmonary Fibrosis Awaiting Lung Transplantation.

PubMed

Hayes, Don; Black, Sylvester M; Tobias, Joseph D; Kirkby, Stephen; Mansour, Heidi M; Whitson, Bryan A

2016-01-01

The influence of varying levels of pulmonary hypertension (PH) on survival in idiopathic pulmonary fibrosis is not well defined. The United Network for Organ Sharing database was queried from 2005 to 2013 to identify first-time lung transplant candidates listed for lung transplantation who were tracked from waitlist entry date until death or censoring to determine the influence of PH on patients with advanced lung disease. Using data for right heart catheterization measurements, mild PH was defined as mean pulmonary artery pressure of 25 mm Hg or more, and severe as 35 mm Hg or more. Of 6,657 idiopathic pulmonary fibrosis patients, 6,651 were used for univariate analysis, 6,126 for Kaplan-Meier survival function, 6,013 for multivariate Cox models, and 5,186 (mild PH) and 2,014 (severe PH) for propensity score matching, respectively. Univariate Cox proportional hazards analysis found significant differences in survival for mild PH (hazard ratio [HR] 1.689, 95% confidence interval [CI]: 1.434 to 1.988, p < 0.001) and severe PH (HR 2.068, 95% CI: 1.715 to 2.493, p < 0.001). Further assessment by multivariate Cox models identified significant risk for death for mild PH (HR 1.433, 95% CI: 1.203 to 1.706, p < 0.001) and severe PH (HR 1.597, 95% CI: 1.308 to 1.949, p < 0.001). Propensity score matching confirmed the risk for death for mild PH (HR 1.530, 95% CI: 1.189 to 1.969, p = 0.001) and severe PH (HR 2.103, 95% CI: 1.436 to 3.078, p < 0.001). The manifestation of PH, even with mild severity, is associated with significantly increased risk for death among patients with idiopathic pulmonary fibrosis awaiting lung transplantation, so referral should be considered early in the disease course. Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Pathogenesis, Newly Recognized Etiologies, and Management of Idiopathic Anaphylaxis

PubMed Central

Kuhlen, James L.; Virkud, Yamini V.

2018-01-01

Idiopathic anaphylaxis (IA) is a life-threatening allergic disease and the most common diagnosis given to patients following an anaphylactic event. The inability of the healthcare provider and the patient to identify the trigger for anaphylaxis makes standard allergen avoidance measures ineffectual. IA is diagnosed after other causes of anaphylaxis have been excluded. Mast cell activation syndromes (MCAS), mastocytosis, IgE to galactose-alpha-1,3-galactose (α-gal), and certain medications have recently been recognized as causes of anaphylaxis that were previously labeled idiopathic. This review will describe the epidemiology and proposed theories of pathogenesis for IA, its diagnostic approach, its clinical management, and examine newly recognized disorders that were previously labeled as idiopathic anaphylaxis. PMID:25725228

Evaluation of patients with symptomatic dermographism.

PubMed

Taşkapan, O; Harmanyeri, Y

2006-01-01

Dermographism or 'simple' dermographism, which has been noted in about 1.5% to 5% of healthy individuals, is regarded as a normal physiological phenomenon. However, in symptomatic dermographism (SD), even light pressure or rubbing from clothes may provoke widespread weal-and-flare reactions with itching and burning. It is one of the dermatologic diseases that negatively impacts quality of life. We aimed to reveal triggering factors and/or associated clinical conditions in patients with SD. Forty patients (28 women and 12 men) with SD whose ages varied between 7 and 65 (30.3 +/- 10.7) participated in the study. Patients having chronic idiopathic urticaria, other types of physical urticaria and cutaneous/systemic mastocytosis were excluded. The diagnosis of SD was confirmed by stroking the skin of the back with a tongue blade. This procedure elicited within minutes a linear weal with a flare and severe itching. In light of the history, physical examination, skin prick test (SPT) with aeroallergens and laboratory results, a possible relationship of certain triggering factors and/or associated diseases (psychic factors, atopy, thyroid diseases, diabetes, menopause, infectious, systemic or malignant diseases, history of scabies, and history of drug reaction) to SD were investigated. The duration of SD varied between 10 days and 10 years (mean: 20.8 months). Psychic factors were found to play the initial triggering role in 12 patients (30%). Five patients (12.50%) were atopic, and they had SPT reactivity to house dust mites. Three female patients (7.50%) defined that their complaints began following drug-induced urticarial rash. Two patients (5%) had hyperthyroidism, and one of the patients with SD of 1-month duration had type-II diabetes mellitus. A female patient defined that her complaints began during the onset of menopausal period. SD was observed with scabies in one patient; and after poststreptococcal glomerulonephritis in a 7-year-old girl. No autoimmune

Diagnosis, pathogenesis, and treatment of chronic spontaneous urticaria.

PubMed

Kaplan, Allen P

2018-05-01

Chronic Spontaneous Urticaria (CSU) is an endogenous disorder that is strongly associated with autoimmunity, particularly with immunoglobulin G (IgG) antibody to the alpha subunit of the IgE receptor seen in 35-40% of patients. Basophils and cutaneous mast cells can be activated and lead to a late-phase-like perivascular infiltration about small venules and hive formation. Review of current literature. Antibody to thyroid antigens are seen in 25% of patients; a small fraction of these may be clinically hypothyroid (Hashimoto's Thyroiditis). Forty percent of patients have angioedema, but not laryngeal edema. Therapy typically begins with second-generation antihistamines (H1 receptor blockers) up to four times a day. The failure rate is substantial, and estimates vary from 25% to 50%. The drug of choice for antihistamine resistant cases is omalizumab, at 300 mg/month, which is effective in 70% of patients. H-2-antagonists and leucotriene antagonists are no longer recommended because the literature does not support additional efficacy beyond blockage of H-1 receptors. For patients unresponsive to antihistamines and omalizumab, cyclosporine is recommended next. This is similarly effective in 65-70% of patients; however, assessment of blood pressure and renal function need to be followed every 4-6 weeks. Corticosteroid should not be employed chronically; however, a brief course of 3-10 days can be used acutely for severe exacerbations. Other agents, such as dapsone, sulfasalazine, or hydroxychloroquin, can be tried when the aforementioned medications fail, but the results are unpredictable because they have not been shown to have efficacy beyond the placebo effect (25-30%), and have not been studied in patients for whom the aforementioned approach i.e. antihistamines, omalizumab, and cyclosporine has failed. High dose antihistamines, omalizumab and cyclosporine (in that order) are effective and recommended for therapy of CUS, an inflammatory skin disorder associated

Idiopathic scrotal elephantiasis.

PubMed

Hornberger, Brad J; Elmore, James M; Roehrborn, Claus G

2005-02-01

Scrotal lymphedema (scrotal elephantiasis) is a condition that has historically been described in areas endemic to filariasis. We present a unique case of a 22-year-old man with idiopathic lymphedema isolated to the scrotum. After acquired causes of lymphedema were ruled out, the patient was treated with scrotectomy and scrotal reconstruction.

Occurrence of idiopathic pulmonary fibrosis during immunosuppressive treatment: a case report.

PubMed

Cerri, Stefania; Sgalla, Giacomo; Richeldi, Luca; Luppi, Fabrizio

2016-05-25

Immunosuppressive therapy has been-until the recent release of new guidelines on diagnosis and management-the recommended treatment for idiopathic pulmonary fibrosis. However, its efficacy in patients with idiopathic pulmonary fibrosis has always been a matter of debate. We report the occurrence of idiopathic pulmonary fibrosis in a white man receiving chronic immunosuppressive treatment following a heart transplant. This case report suggests that the immune mechanisms targeted by azathioprine and cyclosporine do not play a role in the pathogenesis of idiopathic pulmonary fibrosis.

[Bilateral tuberculous mastitis nulliparous patient, initially treated as idiopathic granulomatous mastitis].

PubMed

Ruiz-Moreno, José Luis; Peña-Santos, Genaro

2012-03-01

The breast infection by M tuberculosis is rare, when it occurs, clinical and histologically confused with other forms of granulomatous inflammation, making it essential to use other diagnostic methods also may be negative. We report a patient with fimica mastitis that originally was treated as idiopathic granulomatous mastitis with apparent satisfactory clinical response. However, frequent relapses forced to look for other etiologies. Fortunately, the PCR showed the cause and was managed with specific treatment with disappearance of the disease. Clinical suspicion should be in mind when faced with a case like ours.

Locomotor skills and balance strategies in adolescents idiopathic scoliosis.

PubMed

Mallau, Sophie; Bollini, Gérard; Jouve, Jean-Luc; Assaiante, Christine

2007-01-01

Locomotor balance control assessment was performed to study the effect of idiopathic scoliosis on head-trunk coordination in 17 patients with adolescent idiopathic scoliosis (AIS) and 16 control subjects. The aim of this study was to explore the functional effects of structural spinal deformations like idiopathic scoliosis on the balance strategies used during locomotion. Up to now, the repercussion of the idiopathic scoliosis on head-trunk coordination and balance strategies during locomotion is relatively unknown. Seventeen patients with AIS (mean age 14 years 3 months, 10 degrees < Cobb angle > 30 degrees) and 16 control subjects (mean age 14 years 1 month) were tested during various locomotor tasks: walking on the ground, walking on a line, and walking on a beam. Balance control was examined in terms of rotation about the vertical axis (yaw) and on a frontal plane (roll). Kinematics of foot, pelvis, trunk, shoulder, and head rotations were measured with an automatic optical TV image processor in order to calculate angular dispersions and segmental stabilizations. Decreasing the walking speed is the main adaptive strategy used in response to balance problems in control subjects as well as patients with AIS. However, patients with AIS performed walking tasks more slowly than normal subjects (around 15%). Moreover, the pelvic stabilization is preserved, despite the structural changes affecting the spine. Lastly, the biomechanical defect resulting from idiopathic scoliosis mainly affects the yaw head stabilization during locomotion. Patients with AIS show substantial similarities with control subjects in adaptive strategies relative to locomotor velocity as well as balance control based on segmental stabilization. In contrast, the loss of the yaw head stabilization strategies, mainly based on the use of vestibular information, probably reflects the presence of vestibular deficits in the patients with AIS.

Bayesian comparative effectiveness study of four consensus treatment plans for initial management of systemic juvenile idiopathic arthritis: FiRst-Line Options for Systemic juvenile idiopathic arthritis Treatment (FROST).

PubMed

Nigrovic, Peter A; Beukelman, Timothy; Tomlinson, George; Feldman, Brian M; Schanberg, Laura E; Kimura, Yukiko

2018-06-01

Systemic juvenile idiopathic arthritis is a rare febrile arthritis of childhood characterized by a potentially severe course, including prolonged glucocorticoid exposure, growth failure, destructive arthritis, and life-threatening macrophage activation syndrome. Early cytokine-blocking biologic therapy may improve long-term outcomes, although some systemic juvenile idiopathic arthritis patients respond well to non-biologic treatment, leaving optimal management undefined. Consequently, treatment of new-onset systemic juvenile idiopathic arthritis by expert clinicians varies widely. To describe a pragmatic, observational comparative effectiveness study that takes advantage of diversity in the management of a rare disease: FiRst-Line Options for Systemic juvenile idiopathic arthritis Treatment (FROST), comparing non-biologic and biologic consensus treatment plans for new-onset systemic juvenile idiopathic arthritis within the 60-center Childhood Arthritis and Rheumatology Research Alliance Registry (CARRA). FiRst-Line Options for Systemic juvenile idiopathic arthritis Treatment (FROST) is a multicenter, prospective, non-randomized study that compares four Childhood Arthritis and Rheumatology Research Alliance (CARRA) consensus treatment plans for new-onset systemic juvenile idiopathic arthritis: (1) glucocorticoids alone, (2) methotrexate, (3) interleukin-1 blockade, and (4) interleukin-6 blockade. Patients consenting to participation in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry are started on one of four Consensus Treatment Plans at the discretion of the treating physician. The outcome of primary interest is clinically inactive disease off glucocorticoids at 9 months, comparing non-biologic (Consensus Treatment Plans 1 + 2) versus biologic (Consensus Treatment Plans 3 + 4) strategies. Bayesian analytic methods will be employed to evaluate response rates, using propensity scoring to balance treatment groups for potential

Ghrelin plasma levels in patients with idiopathic short stature.

PubMed

Iñiguez, Germán; Román, Rossana; Youlton, Ronald; Cassorla, Fernando; Mericq, Verónica

2011-02-01

Novel molecular insights have suggested that ghrelin may be involved in the pathogenesis of some forms of short stature. Recently, growth hormone secretagogue receptor (GHSR) mutations that segregate with short stature have been reported. To study plasma ghrelin levels in prepubertal patients with idiopathic short stature (ISS). Fasting total plasma ghrelin levels (radioimmunoassay) in 41 prepubertal patients with ISS (18 females, age 7.9 ± 0.5 years) compared with 42 age- and sex-matched controls (27 females, age 8.0 ± 0.3 years) with normal height. In a subset of 28 patients, the ghrelin receptor was sequenced. ISS patients exhibited a higher level of ghrelin (1,458 ± 137 vs. 935 ± 55 pg/ml, p < 0.01) and similar IGF-I levels (-0.66 ± 1.29 vs. -0.32 ± 0.78 SDS) compared to controls. Ten patients with ISS had ghrelin levels greater than +2 SDS compared to controls. These patients did not differ in height, BMI or IGF-I SDS compared to ISS patients with ghrelin levels within the normal range. Molecular analysis of GHSR did not show any mutations, but showed some polymorphisms. These results suggest that in ISS patients, short stature does not appear to be frequently caused by abnormalities in ghrelin signaling. Copyright © 2010 S. Karger AG, Basel.

Female patients' and parents' assessment of deformity- and brace-related stress in the conservative treatment of adolescent idiopathic scoliosis.

PubMed

Misterska, Ewa; Glowacki, Maciej; Latuszewska, Joanna

2012-06-15

A cross-sectional analysis of parents' and patients' perceptions of deformity- and brace-related stress regarding conservative treatment of adolescent idiopathic scoliosis. The purpose of this study was to determine the agreement between patients' and parents' assessments of emotional stress and to compare these assessments with radiographical measurements of spinal deformity. Conservative treatment in patients with scoliosis may cause emotional stress. To our knowledge, no group has ever reported patient and parental estimation of stress related to wearing a brace and spinal deformity in girls with adolescent idiopathic scoliosis. Sixty-three pairs of parents and girls with adolescent idiopathic scoliosis treated with a Cheneau brace were separately asked to complete the Bad Sobberheim Stress Questionnaire-Deformity and the Bad Sobberheim Stress Questionnaire-Brace. The age range of the patients was from 10 to 17 years. Patients were assessed at a mean of 14.12 (SD, 10.99) months after the start of the conservative treatment. Patients thought that a moderate level of stress was connected with conservative treatment; however, the stress level, related to perceived trunk deformation, was low. From the parents' perspective, patients experienced a moderate level of stress during conservative treatment and related to spinal deformity. The study groups differ in their perception of stress levels due to body disfigurement but not during the conservative treatment. Parent-patient stress-level disparities were not related to body mass index, age of the patient, brace application, and radiographical measurements of spinal deformity. Patients and parents perceive the emotional stress related to brace treatment in the same way; however, parents overestimate the assessment of stress levels related to body deformity. From the perspective of patients and parents, brace wearing increased the level of stress induced by the deformity alone. Complete assessment of conservative

Serial Derotational Casting in Idiopathic and Non-Idiopathic Progressive Early-Onset Scoliosis.

PubMed

Gussous, Yazeed M; Tarima, Sergey; Zhao, Shi; Khan, Safdar; Caudill, Angela; Sturm, Peter; Hammerberg, Kim W

2015-05-01

Serial derotational casting has been used as a definitive treatment or as delaying strategy in progressive idiopathic (IS) and non-idiopathic (NIS) early-onset scoliosis (EOS). Retrospective chart and radiographic review of patients who underwent serial casting for progressive EOS between 2005 and 2012 at a single institution. A total of 74 consecutive patients entered serial cast treatment. Twenty-eight were currently being casted, 30 completed cast treatment and were converted to thoracolumbosacral orthosis (TLSO), 9 were treated surgically, 6 were lost to follow-up, and 1 had no further treatment. The researchers diagnosed IS in 41 patients; 33 had NIS. At presentation the IS group had an average Cobb angle (CA) of 49° and a rib vertebral angle difference (RVAD) of 37°. The NIS group had a CA of 51° (p = .69) and RVAD of 37° (p = .94). In patients currently being casted, 19 IS patients had a decreased CA, from 47° to 27°. The 9 NIS patients had a decreased CA, from 62° to 57° (p = .0002). Cobb angle improvement was significantly better in IS (p = .0005). In the TLSO group the 17 IS patients had a decreased average CA, from 46° to 18°, after serial casting and the 13 NIS patients decreased CA from 42° to 32°. Patients with IS had better improvement in CA than the NIS group (p < .001). At last follow-up, this was reduced to 11° in the IS group and maintained at 32° in the NIS. In the IS group, 5 of 41 patients were converted to growth constructs, and 4 of 26 in the NIS group. Casting initiated before age 2 years yielded better curve correction for IS (p < .01) compared with NIS. Progressive idiopathic scoliosis patients had better curve correction with casting than NIS patients. Casting in IS patients before age 24 months yielded better curve correction. Patients who required surgery had a higher age and Cobb angle at presentation than those who transitioned to a TLSO. The surgical group was observed for a similar duration of time and there was no

Giant scrotal elephantiasis: an idiopathic case.

PubMed

Dianzani, C; Gaspardini, F; Persichetti, P; Brunetti, B; Pizzuti, A; Margiotti, K; Degener, A M

2010-01-01

Scrotal elephantiasis is very rare disease in industrialized countries, where it is mainly due to surgery, irradiation or malignancies. It can be defined as idiopathic only when the possible congenital, infectious and compressive causes are excluded. We report a case of massive scrotal lymphoedema in an adult Caucasian patient, in Italy. He presented an extremely voluminous scrotal mass measuring 50 x 47 x 13 cm (weight 18 kg), which extended below his knees, invalidating all his daily activities. The patient was hospitalized in order to undergo to surgical treatment. Although genetic causes were searched and the possible role of infectious agents and compressive factors was evaluated, no etiology was ascertained. Histopathologic examination showed non-specific chronic inflammation, confirming the diagnosis of idiopathic elephantiasis. One year after surgical treatment, the patient is healthy without recurrence signs.

Short-term Clinical Results of Manipulation Under Ultrasound-Guided Brachial Plexus Block in Patients with Idiopathic Frozen Shoulder and Diabetic Secondary Frozen Shoulder.

PubMed

Ando, Akira; Hamada, Junichiro; Hagiwara, Yoshihiro; Sekiguchi, Takuya; Koide, Masashi; Itoi, Eiji

2018-01-01

This study examined the effectiveness of manipulation under ultrasound-guided brachial plexus block in patients with recalcitrant idiopathic frozen shoulder and diabetic secondary frozen shoulder (diabetic frozen shoulder). Forty-four idiopathic frozen shoulders and 10 diabetic frozen shoulders with failed conservative treatment for at least 3 months were included in this study. The manipulation was performed under ultrasound-guided brachial plexus block and visual analogue scale, range of motion, and Constant scores were measured before manipulation and at the last follow-up examination. No major complications were observed during the procedure. Sufficient improvement was not obtained in two patients during the procedure and to avoid complications, the procedure was discontinued and subsequently arthroscopic capsular release was performed. Visual analogue scale, range of motion towards all directions, and Constant scores were significantly improved after the manipulation in both the idiopathic frozen shoulder and diabetic frozen shoulder groups, however the diabetic group showed inferior results compared with those of the idiopathic group. This manipulation was effective and shortened the duration of symptoms in most of the idiopathic and diabetic frozen shoulders without major complications during the procedure. Diabetic frozen shoulder showed inferior clinical results and difficulty in recovery in range of motion, which indicated that diabetic frozen shoulder should be discussed as a different entity.

Contact urticaria due to the common stinging nettle (Urtica dioica)--histological, ultrastructural and pharmacological studies.

PubMed

Oliver, F; Amon, E U; Breathnach, A; Francis, D M; Sarathchandra, P; Black, A K; Greaves, M W

1991-01-01

A frequent cause of contact urticaria is skin exposure to the common stinging nettle (Urtica dioica). The urticaria is accompanied by a stinging sensation lasting longer than 12 h. Little is known of the cellular and molecular mechanism of stinging-nettle urticaria. After preliminary pharmacological analysis of pro-inflammatory activity in nettle stings, the cellular response of mononuclear cells, polymorphonuclear cells and mast cells was examined in six people 5 min and 12 h after nettle contact. Only mast cell numbers were significantly increased at 12 h. Ultrastructurally, some mast cells showed evidence of degranulation at 5 min and 12 h. At 12 h mast cells were closely associated with dermal dendritic cells and lymphocytes suggesting a functional unit. The mean histamine and serotonin contents of a nettle hair were found to be 6.1 ng and 33.25 pg, respectively. Nettle-sting extracts did not demonstrate histamine release from dispersed rat mast cells in vitro. These results suggest that part of the immediate reaction to nettle stings is due to histamine introduced by the nettle. However, the persistence of the stinging sensation might suggest the presence of substances in nettle fluid directly toxic to nerves or capable of secondary release of other mediators.

Characteristics of ocular pain complaints in patients with idiopathic dry eye symptoms

PubMed Central

Kalangara, Jerry P.; Galor, Anat; Levitt, Roy C.; Covington, Derek B.; McManus, Katherine T.; Sarantopoulos, Constantine D.; Felix, Elizabeth R.

2016-01-01

Objective The purpose of this study was to examine the severity and quality of ocular pain complaints in patients with dry eye symptoms. Methods Subjects with clinically-relevant dry eye symptoms (dryness, discomfort, tearing) of unknown origin seen in the Miami Veterans Affairs eye clinic were administered questionnaires for dry eye symptoms and ocular pain and underwent a standardized ocular examination. Qualities and severity ratings of ocular pain in subjects with idiopathic dry eye were compared to similar measures from published data in other chronic pain populations. Results The study sample consisted of 154 subjects, of which 91% were male and ranged in age from 27 to 89 (mean age = 61). Fifty-three percent of participants reported an average ocular pain of at least moderate intensity (numerical rating scale (NRS) ≥ 4), with specific characteristics (i.e., “burning” spontaneous pain) reported at frequencies comparable to prevalent chronic neuropathic pain syndromes as reported in the literature. Significant correlations were found between ocular pain metrics and dry eye symptom severity scores (r=0.57 to 0.66). Dry eye signs, however, did not generally correlate with ocular pain severity. Conclusions A significant proportion of subjects with idiopathic dry eye symptoms reported moderate or greater ocular pain intensity, with the majority endorsing descriptors commonly used by patients with non-ocular neuropathic pain conditions. Identifying sub-groups of dry eye patients based on the presence and characteristics of ocular pain complaints may improve dry eye sub classification and better individualize treatment strategies. PMID:26925537

Idiopathic hemifacial spasm responsive to zonisamide: a case report.

PubMed

Siniscalchi, Antonio; Gallelli, Luca; Palleria, Caterina; De Sarro, Giovambattista

2009-01-01

We describe a patient with idiopathic hemifacial spasm (HFS) that was responsive to zonisamide treatment. A 65-year-old woman presented with a 4-year history of left-sided HFS developing gradually, starting from the upper facial muscles. After several analyses, the diagnosis of idiopathic HFS was made, and the clonazepam treatment (0.5 mg every 8 hours) was started, without a complete remission of symptoms. Therefore, zonisamide (150 mg twice a day for a 6-week period) was added, with a complete resolution. The rechallenge with zonisamide after its dechallenge confirmed its effectiveness. During follow-up, the patient remained symptom-free, with no adverse drug reactions. We suggest that zonisamide could represent a useful therapeutic option in the treatment of idiopathic HFS.

Treatment of idiopathic FSGS with adrenocorticotropic hormone gel.

PubMed

Hogan, Jonathan; Bomback, Andrew S; Mehta, Kshama; Canetta, Pietro A; Rao, Maya K; Appel, Gerald B; Radhakrishnan, Jai; Lafayette, Richard A

2013-12-01

Adrenocorticotropic hormone (ACTH) has shown efficacy as primary and secondary therapy for nephrotic syndrome due to membranous nephropathy. The data on using ACTH to treat idiopathic FSGS are limited. This report describes our experience using ACTH for nephrotic syndrome due to idiopathic FSGS in the United States. Twenty-four patients with nephrotic syndrome from idiopathic FSGS were treated with ACTH gel at two academic medical centers between 2009 and 2012, either as part of investigator-initiated pilot studies (n=16) or by prescription for treatment-resistant FSGS (n=8). The primary outcome was remission of proteinuria. The median dose of ACTH was 80 units injected subcutaneously twice weekly. Treatment durations were not uniform. Twenty-two patients had received immunosuppression (mean, 2.2 medications) before ACTH therapy. Six patients had steroid-dependent and 15 had steroid-resistant FSGS. At the time of ACTH initiation, the median serum creatinine (interquartile range) was 2.0 (1.1-2.7) mg/dl, estimated GFR was 36 (28-78) ml/min per 1.73 m(2), and urine protein-to-creatinine ratio was 4595 (2200-8020) mg/g. At the end of ACTH therapy, 7 of 24 patients (29%) experienced remission (n=2 complete remissions, n=5 partial remissions). All remitters had steroid-resistant (n=5) or steroid-dependent (n=2) FSGS. Two responders relapsed during the follow-up period (mean ± SD, 70±31 weeks). Adverse events occurred in 21 of 24 patients, including one episode of new-onset diabetes that resolved after stopping ACTH and two episodes of AKI. Response to ACTH treatment among steroid-resistant or steroid-dependent patients with FSGS is low, but ACTH gel may be a viable treatment option for some patients with resistant nephrotic syndrome due to idiopathic FSGS. Further research is necessary to determine which patients will respond to therapy.

[Biologic therapy in idiopathic inflammatory myopathy].

PubMed

Selva-O'Callaghan, Albert; Ramos Casals, Manel; Grau Junyent, Josep M

2014-09-15

The aim of this article is to study the evidence-based knowledge related to the use of biological therapies in patients diagnosed with idiopathic inflammatory myopathy (dermatomyositis, polymyositis and inclusion body myositis). In this review the leading published studies related to the use of biological therapy in patients with myositis are analysed; mainly those with high methodological standards, that means randomized and controlled studies. Methodological drawbacks due to the rarity and heterogeneity of these complex diseases are also addressed. Up to now is not possible to ascertain the biologics as a recommended therapy in patients with myositis, at least based in the current evidence-based knowledge, although it can not be neglected as a therapeutic option in some clinical situations, taking into account the scarce of effective treatments in those patients, especially in refractory myositis. Future studies probably will help to better define the role of biological therapies in patients with idiopathic inflammatory myopathy. Copyright © 2013 Elsevier España, S.L.U. All rights reserved.

Patient-reported outcomes in idiopathic pulmonary fibrosis research.

PubMed

Swigris, Jeffrey J; Fairclough, Diane

2012-08-01

Patient-reported outcomes (PROs) include questionnaires or surveys that ask patients for their perceptions about things like symptoms they are experiencing or quality of life. For incurable, morbid, life-shortening conditions like idiopathic pulmonary fibrosis (IPF), PROs are particularly germane: They elucidate for clinicians and researchers what it is like for patients to live with such a disease, and they may detect important treatment effects not captured by other metrics (eg, pulmonary physiology). However, a relative paucity of research on PROs in IPF has left significant knowledge gaps in this area and contributed to the timidity investigators have about using PROs as prominent outcomes in IPF drug trials. Additional research on existing instruments is needed to establish or bolster their basic psychometric properties in IPF. When PROs are used as end points in therapeutic trials, analyzing PRO response data can be challenging, but these challenges can be overcome with a transparent, thoughtful, and sophisticated statistical approach. In this article, we discuss some of the basics of PRO assessment, existing knowledge gaps in IPF-related PRO research, and the potential usefulness of using PROs in IPF trials and conclude by offering specific recommendations for an approach to analyzing repeated-measures PRO data from IPF trials.

MR venography in idiopathic intracranial hypertension: unappreciated and misunderstood

PubMed Central

Higgins, J; Gillard, J; Owler, B; Harkness, K; Pickard, J

2004-01-01

Background: Venous sinus disease must be excluded before diagnosing idiopathic intracranial hypertension but is found only rarely in typical cases. Magnetic resonance venography (MRV) is the technique of choice for investigating this, and provides images that are diagnostic and easy to interpret. However, recent work using more invasive techniques has documented pressure gradients and stenoses in the lateral venous sinuses in many cases of idiopathic intracranial hypertension. Objective: To examine the reason for this discrepancy and to establish whether there are characteristic appearances on MRV in idiopathic intracranial hypertension that are routinely overlooked in clinical practice. Methods: MRVs from 20 patients with idiopathic intracranial hypertension were reviewed, unblinded, by two neuroradiologists, and their appearances rated for focal narrowings and signal gaps. A control group of 40 asymptomatic volunteers, matched for age and sex with the patient group, was recruited prospectively for MRV, and their scans rated in the same way. Results: The lateral sinuses presented a range of appearances with quite different distributions in the two groups (p<0.001). Bilateral lateral sinus flow gaps were seen in 13 of 20 patients with idiopathic intracranial hypertension and in none of 40 controls. Conclusions: A historical failure to use normal healthy controls to establish the boundaries between imaging artefact, normal anatomical variant, and disease means that the pathological significance of the different appearances of the lateral sinuses on MRV has not so far been appreciated. PMID:15026510

Aspirin enhances the induction of type I allergic symptoms when combined with food and exercise in patients with food-dependent exercise-induced anaphylaxis.

PubMed

Harada, S; Horikawa, T; Ashida, M; Kamo, T; Nishioka, E; Ichihashi, M

2001-08-01

We examined the effect of aspirin as a substitute for exercise in inducing urticaria/anaphylaxis in three patients with food-dependent exercise-induced anaphylaxis (FDEIA). Two of the patients had specific IgE antibodies to wheat and the other had antibodies to shrimp. Administration of aspirin before ingestion of food allergens induced urticaria in one patient and urticaria and hypotension in another, while aspirin alone or food alone elicited no response. The third patient developed urticaria only when he took all three items, i.e. aspirin, food and additional exercise, whereas provocation with any one or or two of these did not induce any symptoms. These findings suggest that aspirin upregulates type I allergic responses to food in patients with FDEIA, and further shows that aspirin synergizes with exercise to provoke symptoms of FDEIA. This is the first report of a synergistic effect of aspirin in inducing urticaria/anaphylaxis, which was confirmed using challenge tests in patients with FDEIA.

Bilateral versus single lung transplant for idiopathic pulmonary fibrosis.

PubMed

Lehmann, Sven; Uhlemann, Madlen; Leontyev, Sergey; Seeburger, Joerg; Garbade, Jens; Merk, Denis R; Bittner, Hartmuth B; Mohr, Friedrich W

2014-10-01

It is unknown if uni- or bilateral lung transplant is best for treatment of usual idiopathic pulmonary fibrosis. We reviewed our single-center experience comparing both treatments. Between 2002 and 2011, one hundred thirty-eight patients at our institution underwent a lung transplant. Of these, 58 patients presented with idiopathic pulmonary fibrosis (56.9%) and were the focus of this study. Thirty-nine patients received a single lung transplant and 19 patients a bilateral sequential lung transplant. The mean patient age was 54 ± 10 years, and 69% were male. The intraoperative course was uneventful, save for 7 patients who needed extracorporeal membrane oxygenation support. Three patients had respiratory failure before the lung transplant that required mechanical ventilation and was supported by extracorporeal membrane oxygenation. Elevated pulmonary artery pressure > 40 mm Hg was identified as an independent predictor of early mortality by uni- and multivariate analysis (P = .01; OR 9.7). Using a Cox regression analysis, postoperative extracorporeal membrane oxyge-nation therapy (P = .01; OR 10.2) and the need for > 10 red blood cell concentrate during the first 72 hours after lung transplant (P = .01; OR 5.6) were independent predictors of long-term survival. Actuarial survival at 1 and 5 years was 65.6% and 55.3%, with no significant between-group differences (70.6% and 54.3%). Lung transplant is a safe and curative treatment for idiopathic pulmonary fibrosis. According to our results, unilateral lung transplant for idiopathic pulmonary fibrosis is an alternative to bilateral lung transplant and may affect the allocation process.

Association of spinal deformity and pelvic tilt with gait asymmetry in adolescent idiopathic scoliosis patients: Investigation of ground reaction force.

PubMed

Park, Yang Sun; Lim, Young Tae; Koh, Kyung; Kim, Jong Moon; Kwon, Hyun Joon; Yang, Ji Seung; Shim, Jae Kun

2016-07-01

Adolescent idiopathic scoliosis is a prevalent orthopedic problem in children ages 10 to 16years. Although genetic, physiological and biomechanical factors are considered to contribute to the onset and progression of adolescent idiopathic scoliosis, the underlying mechanisms are not yet clear. The purpose of this study was to investigate the association between spinal deformity and inter-leg ground reaction force asymmetry during walking in adolescent idiopathic scoliosis patients. Fourteen patients (3 males and 11 females) participated in this study. Maximum Cobb's angle, adjusted Cobb's angle, and pelvic tilt were calculated from X-ray images. Asymmetry indices between legs were also calculated from ground reaction force magnitude and time variables from their preferred speed walking. Pearson coefficients of correlation were used to investigate associations of asymmetry indices with angle variables. Asymmetry indices of ground reaction force magnitudes positively correlated with adjusted Cobb's angle and maximum Cobb's angle mainly during the peak of braking phase, average of braking phase, while asymmetry indices of ground reaction force time variables showed no significant correlation with adjusted or maximum Cobb's angle. In contrast, asymmetry indices of ground reaction force time variables positively correlated with pelvic tilt during stance phase. We concluded that the spinal deformity of adolescent idiopathic scoliosis patients estimated using the maximum and adjusted Cobb's angles is generally associated with greater asymmetry of ground reaction force magnitudes in walking, while the pelvic tilt is associated with the greater asymmetry of ground reaction force time variables. Copyright © 2016 Elsevier Ltd. All rights reserved.

Selection of patients with idiopathic normal-pressure hydrocephalus for shunt placement: a single-institution experience.

PubMed

Anile, Carmelo; De Bonis, Pasquale; Albanese, Alessio; Di Chirico, Alessandro; Mangiola, Annunziato; Petrella, Gianpaolo; Santini, Pietro

2010-07-01

The ability to predict outcome after shunt placement in patients with idiopathic normal-pressure hydrocephalus (NPH) represents a challenge. To date, no single diagnostic tool or combination of tools has proved capable of reliably predicting whether the condition of a patient with suspected NPH will improve after a shunting procedure. In this paper, the authors report their experience with 120 patients with the goal of identifying CSF hydrodynamics criteria capable of selecting patients with idiopathic NPH. Specifically, they focused on the comparison between CSF-outflow resistance (R-out) and intracranial elastance (IE). Between January 1977 and December 2005, 120 patients in whom idiopathic NPH had been diagnosed (on the basis of clinical findings and imaging) underwent CSF hydrodynamics evaluation based on an intraventricular infusion test. Ninety-six patients underwent CSF shunt placement: 32 between 1977 and 1989 (Group I) on the basis of purely clinical and radiological criteria; 44 between 1990 and 2002 (Group II) on the basis of the same criteria as Group I and because they had an IE slope > 0.25; and 20 between 2003 and 2005 (Group III) on the basis of the same criteria as Group II but with an IE slope > or = 0.30. Outcomes were evaluated by means of both Stein-Langfitt and Larsson scores. Patients' conditions were considered improved when there was a stable decrease (at 6- and 12-month follow-up) of at least 1 point in the Stein-Langfitt score and 2 points in the Larsson score. Group I: while no statistically significant difference in mean R-out value between improved and unimproved cases was observed, a clear-cut IE slope value of 0.25 differentiated very sharply between unimproved and improved cases. Group II: R-out values in the 2 unimproved cases were 20 and 47 mm Hg/ml/min, respectively. The mean IE slope in the improved cases was 0.56 (range 0.30-1.4), while the IE slopes in the 2 unimproved cases were 0.26 and 0.27. Group III: the mean IE slope was

Lung Cancer in Patients with Severe Idiopathic Pulmonary Fibrosis: Critical Aspects.

PubMed

Bargagli, Elena; Bonti, Viola; Ferrari, Katia; Rosi, Elisabetta; Bindi, Alessandra; Bartolucci, Maurizio; Chiara, Moroni; Voltolini, Luca

2017-01-01

Idiopathic pulmonary fibrosis (IPF) is a rare interstitial lung disease limited to the lung with an undefined etiopathogenesis and a very short life expectancy (less than 5 years). IPF susceptibility has been associated with several genetic and environmental risk factors and the prognosis is conditioned by comorbidities such as gastro-esophageal reflux, depression, venous thromboembolism, pulmonary hypertension and lung cancer. At 5 years follow-up, 15% of IPF patients develop lung cancer, which can significantly reduce their survival. Because diagnostic or therapeutic procedures such as surgical, radiation or pharmacological treatments may induce acute exacerbations and increase mortality, the management of lung cancer in IPF patients is a very difficult task. This study discusses advantages and disadvantages of lung cancer treatments in patients with severe IPF, highlighting several controversial aspects on this topic, including potential nintedanib treatment. Copyright© 2017, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.

Idiopathic diaphragmatic paralysis: Bell's palsy of the diaphragm?

PubMed

Crausman, Robert S; Summerhill, Eleanor M; McCool, F Dennis

2009-01-01

Idiopathic diaphragm paralysis is probably more common and responsible for more morbidity than generally appreciated. Bell's palsy, or idiopathic paralysis of the seventh cranial nerve, may be seen as an analogous condition. The roles of zoster sine herpete and herpes simplex have increasingly been recognized in Bell's palsy, and there are some data to suggest that antiviral therapy is a useful adjunct to steroid therapy. Thus, we postulated that antiviral therapy might have a positive impact on the course of acute idiopathic diaphragm paralysis which is likely related to viral infection. Three consecutive patients with subacute onset of symptomatic idiopathic hemidiaphragm paralysis were empirically treated with valacyclovir, 1,000 mg twice daily for 1 week. Prior to therapy, diaphragmatic function was assessed via pulmonary function testing and two-dimensional B-mode ultrasound, with testing repeated 1 month later. Diaphragmatic function pre- and post-treatment was compared to that of a historical control group of 16 untreated patients. All three subjects demonstrated ultrasound recovery of diaphragm function 4-6 weeks following treatment with valacyclovir. This recovery was accompanied by improvements in maximum inspiratory pressure (PI(max)) and vital capacity (VC). In contrast, in the untreated cohort, diaphragm recovery occurred in only 11 subjects, taking an average of 14.9 +/- 6.1 months (mean +/- SD). The results of this small, preliminary study suggest that antiviral therapy with valacyclovir may be helpful in the treatment of idiopathic diaphragm paralysis induced by a viral infection.

State of the art of chronic spontaneous urticaria in Italy: a multicentre survey to evaluate physicians’ and patients’ perspectives

PubMed Central

Rimoldi, Marco; Rossi, Oliviero; Rota, Nadia

2016-01-01

Objective To assess the clinical status of chronic spontaneous urticaria (CSU) and understand treatment approaches in Italy through specialists who treat CSU (dermatologists and allergy specialists) and CSU patients' experience. Design Multicentre survey. Setting Online structured questionnaires (one for physicians and one for patients). Participants Physicians and patients with CSU in Italy. Interventions None. Primary/secondary outcomes Physician and patient attitudes/experiences. Results Survey results from 160 allergy and 160 dermatology specialists show that specialists see a median of 40 (IQR 20–80) patients with CSU/year. While most specialists (56%) know the CSU guidelines, only 27% use them regularly (36% of allergy specialists vs 18% of dermatologists). This is reflected in treatment choices with differences between physicians who use guidelines regularly and those who do not: 91.6% vs 71.7% choose standard-dose, non-sedating antihistamines as first-line treatment; 85.9% vs 56.0% select up-dosing for second-line treatment and 65.3% vs 37.2% add leukotriene receptor antagonists or H2-antihistamines as third-line treatment. The diaries from 1385 patients highlight that, regardless of treatment regimen, 29.4% of currently treated patients are refractory to therapy. Specialists aim to resolve symptoms and only 7.8% report improving quality of life (QoL) as a priority. Only 16.6% of specialists are familiar with and use the Urticaria Activity Score while 46.9% do not know it. Overall, 537 patients with CSU were surveyed (median age 37 years, IQR 30–46; 44.3% men; median disease duration 5 years, IQR 3–20). Approximately 62% confirm that CSU negatively impacts their QoL. Patients also complain of difficulties in getting information and support: <5% of medical centres provide patient support services. Conclusions In Italy, the gap between guideline-based care and QoL-related needs in CSU patients affects treatment satisfaction. This information could

Patients' experience of adolescent idiopathic scoliosis surgery: a phenomenological analysis.

PubMed

Honeyman, Cheryl; Davison, Jean

2016-09-12

Background Adolescent idiopathic scoliosis is a three-dimensional curvature of the spine of unknown cause that occurs in often otherwise fit young people. A complex surgical procedure is required for the most severe curves. Quantitative literature suggests scoliosis surgery improves patients' lives, while qualitative literature focuses on patients' concerns rather than their experience. Aims To explore how adolescents interpret their perioperative experience. Method Six participants, aged 15-18, were interviewed and transcripts were analysed. Findings Four themes were identified: shock, fears and worries; parental interaction; coping; and motivation and positivity. Conclusion Participants were reluctant to share concerns, however those they shared related more to fear of the unknown and lack of control than specific issues such as pain. Participants depended on their parents, especially their mothers, during the perioperative period, and they recognised their parents' stress. Participants coped well, were motivated and had a positive outlook.

Evidence for human leukocyte antigen-related susceptibility in idiopathic childhood ischemic stroke.

PubMed

Zou, Li-Ping; Guo, Yu-Hong; Fang, Fang; Jin, Hong; Wu, Hu-Sheng; Mix, Eilhard

2002-01-01

Stroke in children is a relatively uncommon condition and frequently associated with other diseases like cardiopathies, sickle cell disease and chronic smoking. In contrast to stroke in adults, it is rarely caused by atherosclerosis, hypertension or diabetes mellitus. Childhood stroke of unknown causes is called idiopathic stroke. The etiology of idiopathic stroke is unknown. However, several so-called idiopathic diseases develop on the basis of a genetic predisposition. As an approach to investigate this possibility in idiopathic childhood ischemic stroke, we studied the relationship between clinical and immunogenetic features in this disease. We demonstrate that the gene frequencies and relative risk of HLA-B51 were markedly increased in our patients compared with controls (p < 0.001). Thirteen of seventeen HLA-B51-positive patients had had a preceding respiratory infection, which was a higher proportion than in the control group (p < 0.05). In the patient group, the alleles HLA-DRB1*0802, -DRAI*0401 and -DQBI*0402 were also significantly increased, defining the haplotype DRB1*0802-DRA1*0401-DQB1*0402 as a high-risk haplotype for idiopathic childhood ischemic stroke. Transient viral or bacterial infections, which involve vasculitis and vascular occlusion in the brain, can trigger idiopathic childhood ischemic stroke on the basis of an genetic predisposition. Copyright 2002 S. Karger AG, Basel

Sodium oxybate for idiopathic REM sleep behavior disorder: a report on two patients.

PubMed

Moghadam, Keivan Kaveh; Pizza, Fabio; Primavera, Alberto; Ferri, Raffaele; Plazzi, Giuseppe

2017-04-01

REM-sleep behavior disorder (RBD) therapy is based on small to medium-sized case series, as no large controlled clinical trials have been performed. The most used and widely recognized effective drugs are clonazepam and melatonin, with anecdotal reports on the potential benefit of other drug classes. We report on two patients suffering from idiopathic RBD presenting with almost nightly complex and violent episodes, refractory to conventional drugs. Both patients, after informed consent, were treated off-label with sodium oxybate in add-on therapy. We followed up the patients in order to assess treatment efficacy by means of clinical interview, visual analog scales (VAS) for frequency and severity, Clinical Global Impression (CGI) improvement scale and efficacy index, video-polysomnography and at-home actigraphy. Sodium oxybate intake was well tolerated and effective in reducing the number and intensity of RBD episodes; patients reported no new traumatic episodes. Results were confirmed by bed-partner reports, VAS, CGI improvement scale and efficacy index, and at-home actigraphic monitoring, the latter showing a trend of improvement in nocturnal sleep quality and reduction in motor activity, compared to the baseline. Nevertheless, video-polysomnography did not show a clear beneficial effect on sleep-related electromyographic parameters. Our cases suggest that sodium oxybate can be an effective add-on option for the treatment of idiopathic RBD refractory to conventional therapies. The lack of improvement of polysomnographic parameters suggests caution in considering only polysomnographic data as endpoints in the assessment of the efficacy of therapies for RBD, and that long-term home-based assessment seems a promising tool. Copyright © 2016 Elsevier B.V. All rights reserved.

Acute exacerbation of idiopathic pulmonary fibrosis triggered by Aspergillus empyema.

PubMed

Suzuki, Atsushi; Kimura, Tomoki; Kataoka, Kensuke; Matsuda, Toshiaki; Yokoyama, Toshiki; Mori, Yuta; Kondoh, Yasuhiro

2018-01-01

Acute exacerbation (AE) is a severe and life-threatening complication of idiopathic pulmonary fibrosis (IPF). In 2016, the definition and diagnostic criteria for AE-IPF were updated by an international working group. The new definition includes any acute, clinically significant respiratory deterioration (both idiopathic and triggered events) characterized by evidence of new widespread alveolar abnormality in patients with IPF. There are no currently proven beneficial management strategies for idiopathic and triggered AE-IPF. This is the first report describing AE-IPF triggered by Aspergillus empyema, which was improved by a combination of corticosteroid, systemic antifungal therapy, local antifungal therapy, and additional pharmacological therapies. Future research may reveal optimal strategies for both idiopathic and triggered AE-IPF.

Optimal management of idiopathic scoliosis in adolescence

PubMed Central

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

2013-01-01

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

Thrombospondin Type-1 Domain-Containing 7A in Idiopathic Membranous Nephropathy

PubMed Central

Meyer-Schwesinger, Catherine; Seitz-Polski, Barbara; Ma, Hong; Zahner, Gunther; Dolla, Guillaume; Hoxha, Elion; Helmchen, Udo; Dabert-Gay, Anne-Sophie; Debayle, Delphine; Merchant, Michael; Klein, Jon; Salant, David J.; Stahl, Rolf A.K.; Lambeau, Gérard

2014-01-01

BACKGROUND Idiopathic membranous nephropathy is an autoimmune disease. In approximately 70% of patients, it is associated with autoantibodies against the phospholipase A2 receptor 1 (PLA2R1). Antigenic targets in the remaining patients are unknown. METHODS Using Western blotting, we screened serum samples from patients with idiopathic membranous nephropathy, patients with other glomerular diseases, and healthy controls for antibodies against human native glomerular proteins. We partially purified a putative new antigen, identified this protein by means of mass spectrometry of digested peptides, and validated the results by analysis of recombinant protein expression, immunoprecipitation, and immunohistochemical analysis. RESULTS Serum samples from 6 of 44 patients in a European cohort and 9 of 110 patients in a Boston cohort with anti-PLA2R1–negative idiopathic membranous nephropathy recognized a glomerular protein that was 250 kD in size. None of the serum samples from the 74 patients with idiopathic membranous nephropathy who were sero-positive for anti-PLA2R1 antibodies, from the 76 patients with other glomerular diseases, and from the 44 healthy controls reacted against this antigen. Although this newly identified antigen is clearly different from PLA2R1, it shares some biochemical features, such as N-glycosylation, membranous location, and reactivity with serum only under nonreducing conditions. Mass spectrometry identified this antigen as thrombospondin type-1 domain-containing 7A (THSD7A). All reactive serum samples recognized recombinant THSD7A and immunoprecipitated THSD7A from glomerular lysates. Moreover, immunohistochemical analyses of biopsy samples from patients revealed localization of THSD7A to podocytes, and IgG eluted from one of these samples was specific for THSD7A. CONCLUSIONS In our cohort, 15 of 154 patients with idiopathic membranous nephropathy had circulating autoantibodies to THSD7A but not to PLA2R1, a finding that suggests a distinct

Enlarged Dural Sac in Idiopathic Bronchiectasis Implicates Heritable Connective Tissue Gene Variants

PubMed Central

Birchard, Katherine R.; Lowe, Jared R.; Patrone, Michael V.

2016-01-01

Rationale: Patients with idiopathic bronchiectasis are predominantly female and have an asthenic body morphotype and frequent nontuberculous mycobacterial respiratory infections. They also demonstrate phenotypic features (scoliosis, pectus deformity, mitral valve prolapse) that are commonly seen in individuals with heritable connective tissue disorders. Objectives: To determine whether lumbar dural sac size is increased in patients with idiopathic bronchiectasis as compared with control subjects, and to assess whether dural sac size is correlated with phenotypic characteristics seen in individuals with heritable connective tissue disorders. Methods: Two readers blinded to diagnosis measured anterior–posterior and transverse dural sac diameter using L1–L5 magnetic resonance images of 71 patients with idiopathic bronchiectasis, 72 control subjects without lung disease, 29 patients with cystic fibrosis, and 24 patients with Marfan syndrome. We compared groups by pairwise analysis of means, using Tukey’s method to adjust for multiple comparisons. Dural sac diameter association with phenotypic and clinical features was also tested. Measurements and Main Results: The L1–L5 (average) anterior–posterior dural sac diameter of the idiopathic bronchiectasis group was larger than those of the control group (P < 0.001) and the cystic fibrosis group (P = 0.002). There was a strong correlation between increased dural sac size and the presence of pulmonary nontuberculous mycobacterial infection (P = 0.007) and long fingers (P = 0.003). A trend toward larger dural sac diameter was seen in those with scoliosis (P = 0.130) and those with a family history of idiopathic bronchiectasis (P = 0.149). Conclusions: Individuals with idiopathic bronchiectasis have an enlarged dural sac diameter, which is associated with pulmonary nontuberculous mycobacterial infection, long fingers, and family history of idiopathic bronchiectasis. These findings support our

Genetics Home Reference: idiopathic pulmonary fibrosis

MedlinePlus

... structures at the ends of chromosomes known as telomeres . It is not well understood how defects in ... Collard HR, Wolters PJ, Garcia CK. Effect of telomere length on survival in patients with idiopathic pulmonary ...

Survival of Idiopathic Pulmonary Arterial Hypertension Patients in the Modern Era in Australia and New Zealand.

PubMed

Strange, Geoff; Lau, Edmund M; Giannoulatou, Eleni; Corrigan, Carolyn; Kotlyar, Eugene; Kermeen, Fiona; Williams, Trevor; Celermajer, David S; Dwyer, Nathan; Whitford, Helen; Wrobel, Jeremy P; Feenstra, John; Lavender, Melanie; Whyte, Kenneth; Collins, Nicholas; Steele, Peter; Proudman, Susanna; Thakkar, Vivek; Keating, Dominic; Keogh, Anne

2017-09-20

Epidemiology and treatment strategies continue to evolve in pulmonary arterial hypertension (PAH). We sought to define the characteristics and survival of patients with idiopathic, heritable and drug-induced PAH in the current management era. Consecutive cases of idiopathic, heritable and drug-induced PAH were prospectively enrolled into an Australian and New Zealand Registry. Between January 2012 and December 2016, a total of 220 incident cases were enrolled (mean age 57.2±18.7years, female 69.5%) and followed for a median duration of 26 months (IQR17-39). Co-morbidities were common such as obesity (34.1%), systemic hypertension (30.5%), coronary artery disease (16.4%) and diabetes mellitus (19.5%). Initial combination therapy was used in 54 patients (dual, n=50; triple, n=4). Estimated survival rates at 1-year, 2-years and 3-years were 95.6% (CI 92.8-98.5%), 87.3% (CI 82.5-92.4%) and 77.0% (CI 70.3-84.3%), respectively. Multivariate analysis showed that male sex and lower 6-minute distance at diagnosis independently predicted worse survival, whereas obesity was associated with improved survival. Co-morbidities other than obesity did not impact survival. Initial dual oral combination therapy was associated with a trend towards better survival compared with initial oral monotherapy (adjusted HR=0.27, CI 0.06-1.18, p=0.082) CONCLUSIONS: The epidemiology and survival of patients with idiopathic PAH in Australia and New Zealand are similar to contemporary registries reported in Europe and North America. Male sex and poorer exercise capacity are predictive of mortality whereas obesity appears to exert a protective effect. Despite current therapies, PAH remains a life-threatening disease associated with significant early mortality. Copyright © 2017 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). All rights reserved.

Treatment of Idiopathic FSGS with Adrenocorticotropic Hormone Gel

PubMed Central

Bomback, Andrew S.; Mehta, Kshama; Canetta, Pietro A.; Rao, Maya K.; Appel, Gerald B.; Radhakrishnan, Jai; Lafayette, Richard A.

2013-01-01

Summary Background and objectives Adrenocorticotropic hormone (ACTH) has shown efficacy as primary and secondary therapy for nephrotic syndrome due to membranous nephropathy. The data on using ACTH to treat idiopathic FSGS are limited. This report describes our experience using ACTH for nephrotic syndrome due to idiopathic FSGS in the United States. Design, setting, participants, & measurements Twenty-four patients with nephrotic syndrome from idiopathic FSGS were treated with ACTH gel at two academic medical centers between 2009 and 2012, either as part of investigator-initiated pilot studies (n=16) or by prescription for treatment-resistant FSGS (n=8). The primary outcome was remission of proteinuria. The median dose of ACTH was 80 units injected subcutaneously twice weekly. Treatment durations were not uniform. Results Twenty-two patients had received immunosuppression (mean, 2.2 medications) before ACTH therapy. Six patients had steroid-dependent and 15 had steroid-resistant FSGS. At the time of ACTH initiation, the median serum creatinine (interquartile range) was 2.0 (1.1–2.7) mg/dl, estimated GFR was 36 (28–78) ml/min per 1.73 m2, and urine protein-to-creatinine ratio was 4595 (2200–8020) mg/g. At the end of ACTH therapy, 7 of 24 patients (29%) experienced remission (n=2 complete remissions, n=5 partial remissions). All remitters had steroid-resistant (n=5) or steroid-dependent (n=2) FSGS. Two responders relapsed during the follow-up period (mean ± SD, 70±31 weeks). Adverse events occurred in 21 of 24 patients, including one episode of new-onset diabetes that resolved after stopping ACTH and two episodes of AKI. Conclusions Response to ACTH treatment among steroid-resistant or steroid-dependent patients with FSGS is low, but ACTH gel may be a viable treatment option for some patients with resistant nephrotic syndrome due to idiopathic FSGS. Further research is necessary to determine which patients will respond to therapy. PMID:24009220

Computed tomographic features of idiopathic fibrosing interstitial pneumonia: comparison with pulmonary fibrosis related to collagen vascular disease.

PubMed

Hwang, Jeong-Hwa; Misumi, Shigeki; Sahin, Hakan; Brown, Kevin K; Newell, John D; Lynch, David A

2009-01-01

To compare the computed tomographic (CT) features of idiopathic fibrosing interstitial pneumonia with those of pulmonary fibrosis related to collagen vascular disease (CVD). We reviewed the CT scans of 177 patients with diffuse interstitial pulmonary fibrosis, of which 97 had idiopathic fibrosing interstitial pneumonia and 80 had CVD. The CT images were systematically scored for the presence and extent of pulmonary and extrapulmonary abnormalities. Computed tomographic diagnosis of usual interstitial pneumonia (UIP) or nonspecific interstitial pneumonia (NSIP) was assigned. A CT pattern of UIP was identified in 59 (60.8%) of patients with idiopathic fibrosing interstitial pneumonia compared with 15 (18.7%) of those patients with CVD; conversely, the CT diagnosis of NSIP was made in 51 (64%) of patients with CVD compared with 36 (37%) of patients with idiopathic disease (P < 0.01). In 113 patients who had lung biopsy, the CT diagnoses of UIP and NSIP were concordant with the histologic diagnoses in 36 of 50 patients and 34 of 41 patients, respectively. Pleural effusions, esophageal dilation, and pericardial abnormalities were more frequent in patients with CVD than in patients with idiopathic fibrosing interstitial pneumonia. Compared with patients with CVD, those patients with an idiopathic fibrosing interstitial pneumonia showed a higher prevalence of a UIP pattern and lower prevalence of an NSIP pattern as determined by CT. Identification of coexisting extrapulmonary abnormalities on CT can support a diagnosis of CVD.

Metabolomic analysis of urine with Nuclear Magnetic Resonance spectroscopy in patients with idiopathic sudden sensorineural hearing loss: A preliminary study.

PubMed

Carta, Filippo; Lussu, Milena; Bandino, Fabrizio; Noto, Antonio; Peppi, Marcello; Chuchueva, Natalia; Atzori, Luigi; Fanos, Vassilios; Puxeddu, Roberto

2017-08-01

Idiopathic sudden sensorineural hearing loss is a frequent emergency, with unknown aetiology and usually treated with empiric therapy. Steroids represent the only validated treatment but prognosis is unpredictable and the possibility to select the patients who will not respond to steroids could avoid unnecessary treatments. Metabolomic profiling of the biofluids target the analysis of the final product of genic expression and enzymatic activity, defining the biochemical phenotype of a whole biologic system. We studied the metabolomics of the urine of a cohort of patients with idiopathic sudden sensorineural hearing loss, correlating the metabolic profiles with the clinical outcomes. Metabolomic profiling of urine samples was performed by 1 H Nuclear Magnetic Resonance spectroscopy in combination with multivariate statistical approaches. 26 patients were included in the study: 5 healthy controls, 13 patients who did not recover after treatment at 6 months while the remaining 8 patients recovered from the hearing loss. The orthogonal partial least square-discriminant analysis score plot showed a significant separation between the two groups, responders and non-responders after steroid therapy, R 2 Y of 0.83, Q 2 of 0.38 and p value <0.05. The resulting metabolic profiles were characterized by higher levels of urinary B-Alanine, 3-hydroxybutyrate and Trimethylamine N-oxide, and lower levels of Citrate and Creatinine in patients with worst outcome. Idiopathic sudden sensorineural hearing loss is a specific disease with unclear systemic changes, but our data suggest that there are different types of this disorder or patients predisposed to effective action of steroids allowing the recover after treatment. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

[Vitrectomy for idiopathic and secondary preretinal macular membrane].

PubMed

Oficjalska-Młyńczak, Jolanta; Jamrozy-Witkowska, Agnieszka

2004-01-01

To evaluate the results of pars plana vitrectomy and membrane stripping for idiopathic and secondary preretinal macular membrane (PMM). Twenty one consecutive subjects (21 eyes) ranging in age from 40 to 78 (mean 66.9) with PMM underwent vitrectomy and membrane peeling. 17 cases had membranes that were considered idiopathic, and 4 cases were associated with other disorders: 3 occurred after successful retinal reattachment surgery, 1--after laserotherapy in the course of diabetic retinopathy. Visual acuity (VA), Amsler grid, and postoperative complications were assessed. The follow-up was 1 to 22 months, mean 5.7. Visual acuity improved postoperatively in 15 eyes (71.4%), at least two lines on the Snellen chart in 8 eyes (38.1%), entirely in patients with idiopathic PMM. It remained unchanged in 3 eyes (14.3%) and deteriorated in 3 eyes (14.3%). Eyes with transparent membrane showed greater visual improvement than opaque ones. The preoperative Amsler test was positive in 15 patients (71.4%), postoperatively--in 4 cases (19%). 2 idiopathic cases with VA of 0.7 showed postoperatively VA of 1.0. Complications included retinal detachment in 2 eyes (1 in idiopathic and 1 in secondary PMM), and development of nuclear sclerotic cataract in 2 eyes. At 6 months of follow-up, a residual membrane formation in 1 cases appeared. Macular pseudohole was observed in 1 eye with no impact on visual results. 1. Vitrectomy with membrane peeling for preretinal macular membrane provides improvement in visual acuity and reduces metamorphopsia 2. Thin, cellophane-like appearance of the membrane gives a better prognosis of visual function improvement.

[Identifying clinical risk factors in recurrent idiopathic deep venous thrombosis].

PubMed

Del Río Solá, M Lourdes; González Fajardo, José Antonio; Vaquero Puerta, Carlos

2016-03-18

Oral anticoagulant therapy for more than 6 months in patients with an episode of idiopathic thromboembolic disease is controversial. The objective was to determine predictive clinical signs that identify patients at increased risk of thromboembolic recurrence after stopping anticoagulant therapy for 6 months after an episode of idiopathic deep vein thrombosis (DVT). A prospective study which included 306 consecutive patients with a first episode of idiopathic DVT from June 2012 to June 2014. Predictor variables of recurrent thromboembolic disease and episodes of recurrence during follow-up of the patients (28.42 months) were collected. We performed a multivariate analysis to analyze possible predictors (P<.20) and an analysis of Kaplan-Meier to establish mean recurrence-free survival. We identified 91 episodes of residual vein thrombosis on follow-up of the patients (37.5% men and 20.3% women) (OR 1.84; 95% CI 1.25-2.71). In the Cox regression analysis stratified by gender, variables showed significant presence of hyperechoic thrombus (P=.001) in males, and persistence of residual thrombus in women (P=.046). The mean recurrence-free survival was shorter in both groups. The presence of echogenic thrombus in men and the existence of residual DVT in women were 2 clinical signs associated with increased risk of thromboembolic recurrence after stopping anticoagulant therapy for 6 months after an episode of idiopathic DVT in our study. Copyright © 2015 Elsevier España, S.L.U. All rights reserved.

Treatment of Refractory Idiopathic Supraorbital Neuralgia Using Percutaneous Pulsed Radiofrequency.

PubMed

Luo, Fang; Lu, Jingjing; Ji, Nan

2018-02-26

No ideal therapeutic method currently exists for refractory idiopathic supraorbital neuralgia patients who do not respond to conservative therapy, including medications and nerve blocks. Pulsed radiofrequency is a neuromodulation technique that does not produce sequelae of nerve damage after treatment. However, the efficacy of percutaneous pulsed radiofrequency for the treatment of refractory idiopathic supraorbital neuralgia is still not clear. The purpose of our study was to evaluate the efficacy and safety of pulsed radiofrequency treatment of the supraorbital nerve for refractory supraorbital neuralgia patients. We prospectively investigated the long-term effects of ultrasound-guided percutaneous pulsed radiofrequency in the treatment of 22 refractory idiopathic supraorbital neuralgia patients. A reduction in the verbal pain numeric rating scale score of more than 50% was used as the standard of effectiveness. The effectiveness rates at different time points within 2 years were calculated. After a single pulsed radiofrequency treatment, the effectiveness rate at 1 and 3 months was 77%, and the rates at 6 months, 1 year, and 2 years were 73%, 64%, and 50%, respectively. Except for a small portion of patients (23%) who experienced mild upper eyelid ecchymosis that gradually disappeared after approximately 2 weeks, no obvious complications were observed. In conclusion, the results of our study demonstrate that for patients with refractory idiopathic supraorbital neuralgia, percutaneous pulsed radiofrequency may be an effective and safe treatment choice. © 2018 World Institute of Pain.

Anterior hypopituitarism is rare and autoimmune disease is common in adults with idiopathic central diabetes insipidus.

PubMed

Hannon, M J; Orr, C; Moran, C; Behan, L A; Agha, A; Ball, S G; Thompson, C J

2012-05-01

Central diabetes insipidus is a rare clinical condition with a heterogenous aetiology. Up to 40% of cases are classified as idiopathic, although many of these are thought to have an autoimmune basis. Published data have suggested that anterior hypopituitarism is common in childhood-onset idiopathic diabetes insipidus. We aimed to assess the incidence of anterior hypopituitarism in a cohort of adult patients with idiopathic diabetes insipidus. We performed a retrospective review of the databases of two pituitary investigation units. This identified 39 patients with idiopathic diabetes insipidus. All had undergone magnetic resonance imaging scanning and dynamic pituitary testing (either insulin tolerance testing or GHRH/arginine and short synacthen testing) to assess anterior pituitary function. One patient had partial growth hormone deficiency; no other anterior pituitary hormonal deficits were found. Thirty-three percent had at least one autoimmune disease in addition to central diabetes insipidus. Our data suggest that anterior hypopituitarism is rare in adult idiopathic diabetes insipidus. Routine screening of these patients for anterior hypopituitarism may not, therefore, be indicated. The significant prevalence of autoimmune disease in this cohort supports the hypothesis that idiopathic diabetes insipidus may have an autoimmune aetiology. © 2012 Blackwell Publishing Ltd.

Idiopathic bile acid malabsorption: qualitative and quantitative clinical features and response to cholestyramine.

PubMed

Sinha, L; Liston, R; Testa, H J; Moriarty, K J

1998-09-01

Idiopathic bile acid malabsorption is a poorly recognized cause of chronic diarrhoea. The SeHCAT (75Selenium HomotauroCholic Acid Test) can accurately diagnose this condition. To identify patients with idiopathic bile acid malabsorption, to describe their clinical features, both qualitatively and quantitatively, and to assess the response to cholestyramine. Idiopathic bile acid malabsorption was considered in all patients complaining of chronic diarrhoea. They were included in the study if their SeHCATs were positive (< 15% retention) and secondary causes of bile acid malabsorption were excluded. The response to therapy with cholestyramine was assessed. Nine patients were diagnosed with idiopathic bile acid malabsorption (median SeHCAT retention 8%, range 3-12.6). Their median daily faecal weight was 285 g (range 85-676) and median faecal fat output was 17 mmol/24 h (range 8.3-38.8). Six patients had an immediate response to cholestyramine. There was a marked reduction in stool frequency (median stool frequency pre-treatment 5/day vs. 2/day post-treatment, P = 0.03). Five patients had large volume diarrhoea (faecal weight > 200 g/day) and three had steatorrhoea. Idiopathic bile acid malabsorption, once suspected, especially by documenting true 'large volume' watery diarrhoea or steatorrhoea, is easily diagnosed and response to therapy is often very good. There is often a previous history of gastrointestinal infection and this condition should be considered in patients with chronic diarrhoea of undetermined origin, especially before they are labelled as having irritable bowel syndrome.

Idiopathic Chronic Parotitis: Imaging Findings and Sialendoscopic Response.

PubMed

Heineman, Thomas E; Kacker, Ashutosh; Kutler, David I

2015-01-01

The purpose of this study was to correlate imaging and sialendoscopic findings to therapeutic response in patients with idiopathic chronic parotitis. We retrospectively reviewed 122 consecutive sialendoscopies performed in an academic medical center by two surgeons between 2008 and 2013. Forty-one (34%) and 54 (44%) patients were excluded on the basis of having parotid or submandibular sialolith, respectively. Nineteen cases were included in the study with idiopathic chronic parotitis. There was a median follow-up of 5 months. Computed tomography (CT) imaging had a sensitivity and specificity of 80.0 and 71.4%, respectively, for predicting abnormal findings on sialendoscopy, while magnetic resonance imaging (MRI) had 100% accuracy in a small set of cases. In glands with noticeable pathology present on preoperative imaging or sialendoscopy, 11 out of 12 glands (92%) treated experienced symptomatic improvement, while 3 out of 7 glands (43%) without pathology on imaging or endoscopy experienced symptomatic improvement (p = 0.038). Sialendoscopy for the treatment of idiopathic chronic parotid disease can improve pain and swelling with a higher frequency of success in patients with abnormalities noted on endoscopy. CT and MRI have a moderate degree of accuracy in predicting which patients will benefit from therapeutic sialendoscopy. © 2015 S. Karger AG, Basel.

Cross-cultural comparison of the Scoliosis Research Society Outcomes Instrument between American and Japanese idiopathic scoliosis patients: are there differences?

PubMed

Watanabe, Kei; Lenke, Lawrence G; Bridwell, Keith H; Hasegawa, Kazuhiro; Hirano, Toru; Endo, Naoto; Cheh, Gene; Kim, Yongjung J; Hensley, Marsha; Stobbs, Georgia; Koester, Linda

2007-11-15

A comparative study. To report a preliminary evaluation of the Scoliosis Research Society Outcomes Instrument (SRS-24) and determine whether differences in baseline scores exist between American and Japanese patients with idiopathic scoliosis. Because the SRS outcomes instrument was primarily introduced for the American population, baseline scores in the Japanese population might differ from the American population. A comparative study using the SRS instrument between American and Japanese patients with idiopathic scoliosis has not been reported. Two comparable groups of 100 idiopathic scoliosis patients before spinal fusion were separated into American (A) and Japanese (J). There were no statistically significant differences between the groups for gender (A: 9 men/91 women vs. J: 13 men/87 women), age (A: 15.0 +/- 2.4 vs. J: 14.9 +/- 3.8), main curve location (A: 77 thoracic/23 lumbar, J: 76 thoracic/24 lumbar), main curve Cobb angle (A: 50.5 +/- 5.2 vs. J: 51.1 +/- 8.7), and thoracic kyphosis (A: 20.9 +/- 14.3 vs. J: 19.9 +/- 12.1) (P > 0.05, for all comparisons). Patients were evaluated using the first section of the SRS-24 which was divided into 4 domains: total pain, general self-image, general function, and activity. SRS-24 scores were statistical compared in individual domains and questions using the Mann-Whitney U test. American patients had significantly lower scores in pain (P < 0.0001, A: 3.7 +/- 0.8 vs. J: 4.3 +/- 0.4), function (P < 0.01, A: 3.9 +/- 0.6 vs. J: 4.2 +/- 0.5), and activity (P < 0.0001, A: 4.5 +/- 0.8 vs. J: 4.9 +/- 0.3) domains compared with Japanese patients. Japanese patients had significantly lower scores in the self-image (P < 0.0001, A: 4.0 +/- 0.7 vs. J: 3.5 +/- 0.5) domain. With regard to individual questions, there were significant differences in the scores between the 2 groups for all questions except 5 and 13 (P < 0.05, for all comparisons). SRS-24 scores in the Japanese idiopathic scoliosis population differed from that of the

From bad to worse: when lung cancer complicates idiopathic pulmonary fibrosis.

PubMed

Strock, Stephen B; Alder, Jonathan K; Kass, Daniel J

2018-04-01

Patients with idiopathic pulmonary fibrosis have a significantly increased risk for the development of lung cancer. The morbidity and mortality of this disease combination are substantial, and, unfortunately, there are currently few data to help guide clinicians in its diagnosis and treatment. In a recent issue of this journal, Hwang et al presented one of the first studies to evaluate lung cancer in patients with idiopathic pulmonary fibrosis at the molecular level. They demonstrate variants in regulators of the cell cycle, which are known to be important in malignant transformation and may also be important in the pathogenesis of idiopathic pulmonary fibrosis. Further understanding of the pathogenic overlap between lung cancer and idiopathic pulmonary fibrosis could help point the direction to specific diagnostic modalities and targeted treatment of both conditions in the future. Copyright © 2017 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd. Copyright © 2017 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.

Do adolescents with idiopathic scoliosis have body schema disorders? A cross-sectional study.

PubMed

Picelli, Alessandro; Negrini, Stefano; Zenorini, Andrea; Iosa, Marco; Paolucci, Stefano; Smania, Nicola

2016-01-01

To date etiology of adolescent idiopathic scoliosis appears complex and still remains unclear. A distorted body schema has been proposed to be a part of a sequence of pathological events in the development of adolescent idiopathic scoliosis. To investigate the awareness of trunk misalignment in adolescents with idiopathic scoliosis. Information about 44 adolescents with idiopathic scoliosis was collected as follows: age; sex; handedness; family history of scoliosis; back pain; sport practice; shoulder and waist line symmetry; leg length; dorsal kyphosis; back hump; rehabilitation; scoliotic curve; Risser sign. We evaluated awareness of trunk misalignment with a graphic table displaying pictures of progressively increasing scoliotic curves. Patients were asked to indicate which picture corresponded to their perceived own spinal alignment. Patients with thoracolumbar scoliosis overestimated their actual thoracic spine curve. Patients with thoracic-thoracolumbar scoliosis underestimated their actual thoracolumbar spine curve and overestimated their actual lumbar spine curve. Scoliotic curve > 15°, double curve, younger age, back pain, family history of scoliosis and lower Risser score related with a misperception of trunk alignment. Our results support the hypothesis that adolescents with idiopathic scoliosis have an altered corporeal awareness of their trunk alignment.

Studies of the prevalence and significance of radiolabeled bile acid malabsorption in a group of patients with idiopathic chronic diarrhea

DOE Office of Scientific and Technical Information (OSTI.GOV)

Schiller, L.R.; Hogan, R.B.; Morawski, S.G.

1987-01-01

We studied radiolabeled fecal bile acid excretion in 11 normal subjects and 17 patients with idiopathic chronic diarrhea for three major purposes: to establish normal values for this test in the presence of increased stool volumes (induced in normal subjects by ingestion of poorly absorbable solutions); to test for bile acid malabsorption in the patients and to correlate this with an independent test of ileal function, the Schilling test; and to compare the results of the bile acid excretion test with the subsequent effect of a bile acid binding agent (cholestyramine) on stool weight. In normal subjects fecal excretion ofmore » the radiolabel was increased with increasing stool volumes. As a group, patients with idiopathic chronic diarrhea excreted radiolabeled bile acid more rapidly than normal subjects with induced diarrhea (t1/2 56 +/- 8 vs. 236 +/- 60 h, respectively, p less than 0.005). There was a statistically significant positive correlation between t1/2 of radiolabeled bile acid and Schilling test results in these patients. Although 14 of 17 patients absorbed labeled taurocholic acid less well than any of the normal subjects with comparable volumes of induced diarrhea, cholestyramine had no statistically significant effect on stool weight in the patient group, and in none of the patients was stool weight reduced to within the normal range. In summary, most patients with idiopathic chronic diarrhea have bile acid malabsorption (as measured by fecal excretion of labeled bile acid), but they do not respond to cholestyramine therapy with a significant reduction in stool weight. Although the significance of these findings was not clearly established, the most likely interpretation is that bile acid malabsorption is a manifestation of an underlying intestinal motility or absorptive defect rather than the primary cause of diarrhea.« less

[Adolescent idiopathic scoliosis].

PubMed

2016-12-01

Adolescent idiopathic scoliosis is a 3D spinal deformity in frontal, sagittal and axial planes, with high relevance in the pediatric population especially in adolescents and females between 10 years of age and the end of growth spurt and skeletal maturity. The radiographic manifestation is a curve greater than 10° measured by Cobb method associated with vertebral rotation. "Idiopathic" diagnosis has to be done after neuroanatomical anomalies of the posterior cerebral fosa and spinal canal have been ruled out. The physical finding of a thoracic or lumbar hump is the clinical manifestation of vertebral rotation seen in a forward bending test (Adam's Test). It is recommended that all curves with a magnitude greater than 20° have to be controlled and treated by a spinal surgeon being observation, bracing and surgery the different treatment options based on the extent, progression of deformity and basically the clinical condition of the patient. Sociedad Argentina de Pediatría.

Idiopathic pulmonary fibrosis.

PubMed

Xaubet, Antoni; Ancochea, Julio; Molina-Molina, María

2017-02-23

Idiopathic pulmonary fibrosis is a fibrosing interstitial pneumonia associated with the radiological and/or histological pattern of usual interstitial pneumonia. Its aetiology is unknown, but probably comprises the action of endogenous and exogenous micro-environmental factors in subjects with genetic predisposition. Its diagnosis is based on the presence of characteristic findings of high-resolution computed tomography scans and pulmonary biopsies in absence of interstitial lung diseases of other aetiologies. Its clinical evolution is variable, although the mean survival rate is 2-5 years as of its clinical presentation. Patients with idiopathic pulmonary fibrosis may present complications and comorbidities which modify the disease's clinical course and prognosis. In the mild-moderate disease, the treatment consists of the administration of anti-fibrotic drugs. In severe disease, the best therapeutic option is pulmonary transplantation. In this paper we review the diagnostic and therapeutic aspects of the disease. Copyright © 2016 Elsevier España, S.L.U. All rights reserved.

Idiopathic toe walking.

PubMed

Oetgen, Matthew E; Peden, Sean

2012-05-01

Toe walking is a bilateral gait abnormality in which a normal heel strike is absent and most weight bearing occurs through the forefoot. This abnormality may not be pathologic in patients aged <2 years, but it is a common reason for referral to an orthopaedic surgeon. Toe walking can be caused by several neurologic and developmental abnormalities and may be the first sign of a global developmental problem. Cases that lack a definitive etiology are categorized as idiopathic. A detailed history, with careful documentation of the developmental history, and a thorough physical examination are required in the child with a primary report of toe walking. Treatment is based on age and the severity of the abnormality. Management includes observation, stretching, casting, bracing, chemodenervation, and surgical lengthening of the gastrocnemius-soleus complex and/or Achilles tendon. An understanding of idiopathic toe walking as well as treatment options and their outcomes can help the physician individualize treatment to achieve optimal results.

Utilizing a Homecare Platform for Remote Monitoring of Patients with Idiopathic Pulmonary Fibrosis.

PubMed

Panagopoulos, Christos; Malli, Foteini; Menychtas, Andreas; Smyrli, Efstathia-Petrina; Georgountzou, Aikaterini; Daniil, Zoe; Gourgoulianis, Konstantinos I; Tsanakas, Panayiotis; Maglogiannis, Ilias

2017-01-01

Homecare and home telemonitoring are a focal point of emerging healthcare schemes, with proven benefits for both patients, caregivers and providers, including reduction of healthcare costs and improved patients' quality of life, especially in the case of chronic disease management. Studies have evaluated solutions for remote monitoring of chronic patients based on technologies that allow daily symptom and vital signs monitoring, tailored to the needs of specific diseases. In this work, we present an affordable home telemonitoring system for patients with idiopathic pulmonary fibrosis (IPF), based on an application for mobile devices and Bluetooth-enabled sensors for pulse oximetry and blood pressure measurements. Besides monitoring of vital signs, the system incorporates communication via videoconferencing and emergency response, with support from a helpdesk service. A pilot study was conducted, in order to verify the proposed solution's feasibility. The results support the utilization of the system for effective monitoring of patients with IPF.

Outcome of patients presenting with idiopathic facial nerve paralysis (Bell's palsy) in a tertiary centre--a five year experience.

PubMed

Tang, I P; Lee, S C; Shashinder, S; Raman, R

2009-06-01

This is a retrospective study. The objective of this study is to review the factors influencing the outcome of treatment for the patients presented with idiopathic facial nerve paralysis. The demographic data, clinical presentation and management of 84 patients with idiopathic facial nerve paralysis (Bell's palsy) were collected from the medical record office, reviewed and analyzed from 2000 to 2005. Thirty-four (72.3%) out of 47 patients who were treated with oral prednisolone alone, fully recovered from Bell's palsy meanwhile 36 (97%) out of 37 patients who were treated with combination of oral prednisolone and acyclovir fully recovered. The difference was statistically significant. 42 (93.3%) out of 45 patients who presented within three days to our clinic, fully recovered while 28 (71.8%) out of 39 patients presented later then three days had full recovery from Bell's palsy. The difference was statistically significant. The outcome of full recovery is better with the patients treated with combined acyclovir and prednisolone compared with prednisolone alone. The patients who were treated after three days of clinical presentation, who were more than 50 years of age, who had concurrent chronic medical illness and facial nerve paralysis HB Grade IV to VI during initial presentation have reduced chance of full recovery of facial nerve paralysis.

Study of Autophagy and Microangiopathy in Sural Nerves of Patients with Chronic Idiopathic Axonal Polyneuropathy

PubMed Central

Samuelsson, Kristin; Osman, Ayman A. M.; Angeria, Maria; Risling, Mårten; Mohseni, Simin; Press, Rayomand

2016-01-01

Twenty-five percent of polyneuropathies are idiopathic. Microangiopathy has been suggested to be a possible pathogenic cause of chronic idiopathic axonal polyneuropathy (CIAP). Dysfunction of the autophagy pathway has been implicated as a marker of neurodegeneration in the central nervous system, but the autophagy process is not explored in the peripheral nervous system. In the current study, we examined the presence of microangiopathy and autophagy-related structures in sural nerve biopsies of 10 patients with CIAP, 11 controls with inflammatory neuropathy and 10 controls without sensory polyneuropathy. We did not find any significant difference in endoneurial microangiopathic markers in patients with CIAP compared to normal controls, though we did find a correlation between basal lamina area thickness and age. Unexpectedly, we found a significantly larger basal lamina area thickness in patients with vasculitic neuropathy. Furthermore, we found a significantly higher density of endoneurial autophagy-related structures, particularly in patients with CIAP but also in patients with inflammatory neuropathy, compared to normal controls. It is unclear if the alteration in the autophagy pathway is a consequence or a cause of the neuropathy. Our results do not support the hypothesis that CIAP is primarily caused by a microangiopathic process in endoneurial blood vessels in peripheral nerves. The significantly higher density of autophagy structures in sural nerves obtained from patients with CIAP and inflammatory neuropathy vs. controls indicates the involvement of this pathway in neuropathy, particularly in CIAP, since the increase in density of autophagy-related structures was more pronounced in patients with CIAP than those with inflammatory neuropathy. To our knowledge this is the first report investigating signs of autophagy process in peripheral nerves in patients with CIAP and inflammatory neuropathy. PMID:27662650

Contact Urticaria to Nickel: A Series of 11 Patients Who Were Prick Test Positive and Patch Test Negative to Nickel Sulfate 2.5% and 5.0.

PubMed

Saluja, Sandeep S; Davis, Crystal L; Chong, Tracie A; Powell, Douglas L

2016-01-01

Nickel is the most common allergen found by patch testing; however, not all cases of nickel allergy are type 4 (delayed) allergies. Contact urticaria (CU) to nickel (immediate reaction) has been reported; however, few seem to evaluate it as per a recent published survey of American Contact Dermatitis Society members. The aim of the study was to present a series of patients who had clinical histories suggestive of nickel allergy and yet were patch test negative but prick test positive to nickel, thus demonstrating CU. We reviewed the charts of 11 patients who were patch test negative but prick test positive. All 11 patients demonstrated evidence of CU by prick testing (or closed chamber test in 1). None were patch test positive to nickel 2.5% or 5.0%. Four patients' histories mentioned reactions to various jewelry/earrings within minutes, whereas 2 histories mentioned reacting within a few hours. These histories are consistent with CU. Others (except 1 patient) recalled reacting to jewelry/earrings but did not recall a time frame. Our series suggests that CU to nickel may be far more common than anticipated and should be evaluated with prick testing when patients' history suggests nickel allergy and yet they are patch test negative.

The coeruleus/subcoeruleus complex in idiopathic rapid eye movement sleep behaviour disorder.

PubMed

Ehrminger, Mickael; Latimier, Alice; Pyatigorskaya, Nadya; Garcia-Lorenzo, Daniel; Leu-Semenescu, Smaranda; Vidailhet, Marie; Lehericy, Stéphane; Arnulf, Isabelle

2016-04-01

Idiopathic rapid eye movement sleep behaviour disorder is characterized by nocturnal violence, increased muscle tone during rapid eye movement sleep and the lack of any other neurological disease. However, idiopathic rapid eye movement sleep behaviour disorder can precede parkinsonism and dementia by several years. Using 3 T magnetic resonance imaging and neuromelanin-sensitive sequences, we previously found that the signal intensity was reduced in the locus coeruleus/subcoeruleus area of patients with Parkinson's disease and rapid eye movement sleep behaviour disorder. Here, we studied the integrity of the locus coeruleus/subcoeruleus complex with neuromelanin-sensitive imaging in 21 patients with idiopathic rapid eye movement sleep behaviour disorder and compared the results with those from 21 age- and gender-matched healthy volunteers. All subjects underwent a clinical examination, motor, cognitive, autonomous, psychological, olfactory and colour vision tests, and rapid eye movement sleep characterization using video-polysomnography and 3 T magnetic resonance imaging. The patients more frequently had preclinical markers of alpha-synucleinopathies, including constipation, olfactory deficits, orthostatic hypotension, and subtle motor impairment. Using neuromelanin-sensitive imaging, reduced signal intensity was identified in the locus coeruleus/subcoeruleus complex of the patients with idiopathic rapid eye movement sleep behaviour. The mean sensitivity of the visual analyses of the signal performed by neuroradiologists who were blind to the clinical diagnoses was 82.5%, and the specificity was 81% for the identification of idiopathic rapid eye movement sleep behaviour. The results confirm that this complex is affected in idiopathic rapid eye movement sleep behaviour (to the same degree as it is affected in Parkinson's disease). Neuromelanin-sensitive imaging provides an early marker of non-dopaminergic alpha-synucleinopathy that can be detected on an individual

Optimizing quality of life in patients with idiopathic pulmonary fibrosis

PubMed Central

van Manen, Mirjam J. G.; Geelhoed, J. J. Miranda.; Tak, Nelleke C.; Wijsenbeek, Marlies S.

2017-01-01

Idiopathic pulmonary fibrosis (IPF) is a devastating, progressive and ultimately fatal lung disease. The combination of poor prognosis, uncertainty of disease course and severe symptom burden heavily impacts patients’ and their families’ quality of life. Though new antifibrotic drugs have been shown to decrease disease progression, the effect on health-related quality of life (HRQOL) has not been convincingly demonstrated. In a relentless disease such as IPF, striving to optimize HRQOL should complement the endeavour to prolong life. Unfortunately, there is a paucity of interventions improving symptoms and functionality for patients with IPF, and research focusing on symptom improvement, and assessing and optimizing HRQOL, is limited. This review summarizes the most recent insights into measuring and improving quality of life for patients with IPF, and discusses challenges in the management of this devastating disease. Moreover, we postulate a new model for continuous care in IPF – ‘the ABCDE of IPF care’: Assessing patients’ needs; Backing patients by giving information and support; delivering Comfort care by focusing on treating symptoms and taking into account Comorbidities; striving to prolong life by Disease modification; helping and preparing patients and their caregivers for the eventual End-of-life events that are likely to occur. PMID:28134007

Surgical Site Infections in Pediatric Spine Surgery: Comparative Microbiology of Patients with Idiopathic and Nonidiopathic Etiologies of Spine Deformity.

PubMed

Maesani, Matthieu; Doit, Catherine; Lorrot, Mathie; Vitoux, Christine; Hilly, Julie; Michelet, Daphné; Vidal, Christophe; Julien-Marsollier, Florence; Ilharreborde, Brice; Mazda, Keyvan; Bonacorsi, Stéphane; Dahmani, Souhayl

2016-01-01

Surgical site infections (SSIs) are a concern in pediatric spine surgery with unusually high rates for a clean surgery and especially for patients with deformity of nonidiopathic etiology. Microbiologic differences between etiologies of spine deformities have been poorly investigated. We reviewed all cases of SSI in spinal surgery between 2007 and 2011. Characteristics of cases and of bacteria according to the etiology of the spine disease were investigated. Of 496 surgeries, we identified 51 SSIs (10.3%) in 49 patients. Staphylococcus aureus was the most frequent pathogen whatever the etiology (n = 31, 61% of infection cases). The second most frequent pathogens vary according to the etiology of the spine deformity. It was Gram-negative bacilli (GNB) in nonidiopathic cases (n = 19, 45% of cases) and anaerobe in idiopathic cases (n = 8, 38% of cases), particularly Gram-positive anaerobic cocci (n = 5, 24% of cases). Infection rate was 6.8% in cases with idiopathic spine disease (n = 21) and 15.9% in cases with nonidiopathic spine disease (n = 30). Nonidiopathic cases were more frequently male with lower weight. American Society of Anesthesiologists score was more often greater than 2, they had more frequently sacral implants and postoperative intensive care unit stay. GNB were significantly associated with a nonidiopathic etiology, low weight, younger age and sacral fusion. SSIs were polymicrobial in 31% of cases with a mean of 1.4 species per infection cases. S. aureus is the first cause of SSI in pediatric spine surgery. However, Gram-positive anaerobic cocci should be taken into account in idiopathic patients and GNB in nonidiopathic patients when considering antibiotic prophylaxis and curative treatment.

Subjective symptoms in idiopathic hypersomnia: beyond excessive sleepiness.

PubMed

Vernet, Cyrille; Leu-Semenescu, Smaranda; Buzare, Marie-Annick; Arnulf, Isabelle

2010-12-01

Patients with idiopathic hypersomnia never feel fully alert despite a normal or long sleep night. The spectrum of the symptoms is insufficiently studied. We interviewed 62 consecutive patients with idiopathic hypersomnia (with a mean sleep latency lower than 8 min or a sleep time longer than 11 h) and 50 healthy controls using a questionnaire on sleep, awakening, sleepiness, alertness and cognitive, psychological and functional problems during daily life conditions. Patients slept 3 h more on weekends, holidays and in the sleep unit than on working days. In the morning, the patients needed somebody to wake them, or to be stressed, while routine, light, alarm clocks and motivation were inefficient. Three-quarters of the patients did not feel refreshed after short naps. During the daytime, their alertness was modulated by the same external conditions as controls, but they felt more sedated in darkness, in a quiet environment, when listening to music or conversation. Being hyperactive helped them more than controls to resist sleepiness. They were more frequently evening-type and more alert in the evening than in the morning. The patients were able to focus only for 1 h (versus 4 h in the controls). They complained of attention and memory deficit. Half of them had problems regulating their body temperature and were near-sighted. Mental fatigability, dependence on other people for awakening them, and a reduced benefit from usually alerting conditions (except being hyperactive or stressed) seem to be more specific of the daily problems of patients with idiopathic hypersomnia than daytime sleepiness. © 2010 European Sleep Research Society.

Critical appraisal of the unmet needs in the treatment of chronic spontaneous urticaria with omalizumab: an Italian perspective.

PubMed

Asero, Riccardo; Canonica, Giorgio W; Cristaudo, Antonio; Fierro, Maria T; Girolomoni, Giampiero; Marzano, Angelo V; Nettis, Eustachio; Pepe, Patrizia; Pigatto, Paolo; Rossi, Oliviero

2017-12-01

The humanized anti-IgE antibody omalizumab has been available for patients with chronic spontaneous urticaria (CSU) in Italy since 2015. This review summarizes the unresolved issues and unmet therapeutic needs associated with omalizumab and discusses practical recommendations for its use in the management of CSU. Although modern second-generation H1-antihistamines are the standard of care for patients with CSU, adjunctive treatments (including omalizumab) may be required for effective control of symptoms in many patients. Evidence from clinical trials and experience from daily clinical practice suggest that the use of omalizumab in patients with CSU who have inadequate response to H1-antihistamines remains challenging. Based on current international guidelines, omalizumab labelling information and our experience in clinical practice, we provide treatment recommendations regarding the use of omalizumab in patients with CSU. These include: optimal treatment duration, the use of concomitant antihistamine therapy, the definition and management of disease relapse after treatment, and the management of patients with late or no response to treatment.

Juvenile Idiopathic Arthritis

MedlinePlus

... Is Juvenile Idiopathic Arthritis the same as Juvenile Rheumatoid Arthritis? Yes, Juvenile Idiopathic Arthritis (JIA) is a new ... of chronic inflammatory diseases that affect children. Juvenile Rheumatoid Arthritis (JRA) is the older term that was used ...

Idiopathic Intracranial Hypertension (Pseudotumor Cerebri)

MedlinePlus

... Asked Questions Español Condiciones Chinese Conditions Idiopathic Intracranial Hypertension (Pseudotumor Cerebri) En Español Read in Chinese What is idiopathic intracranial hypertension? Idiopathic intracranial hypertension (IIH) is a disorder that ...

Clinical characteristics in patients with asymmetric idiopathic pulmonary fibrosis.

PubMed

Callahan, Sean J; Xia, Meng; Murray, Susan; Flaherty, Kevin R

2016-10-01

A group of patients with idiopathic pulmonary fibrosis (IPF) presents with disease affecting one lung markedly more than the other. At this time, it is unclear how this population differs from those who present with more symmetric disease. We sought to explain the characteristics of the asymmetric group and how their disease progresses. In this retrospective case-control study we accessed an interstitial lung disease (ILD) database and identified 14 asymmetric IPF cases via high-resolution computed tomography (HRCT) scoring of each lung lobe's disease severity. We identified 28 symmetric IPF controls from the same database using the same methods, and compared the clinical features of each group. Patients with asymmetric disease exhibited similar demographics as those in the general IPF population; they were predominantly male (64%), elderly (69 years old), and used tobacco (57%). We found a trend toward significantly increased all-cause mortality in the case population two years following diagnosis (p = 0.089). Pulmonary function tests were significantly lower in the case group at the time of diagnosis, then both groups experienced gradual decline. We found no statistically significant differences in number of IPF exacerbations (cases 43%, controls 39%, p = 0.824) and gastro-esophageal reflux (both groups 50%). Patients with asymmetric IPF resemble patients in the general IPF population but may have a lower overall survival rate. Further systemic factors may be studied to identify reasons for disease asymmetry and clinical decline in this population. Published by Elsevier Ltd.

The Melanopsin-Mediated Pupillary Light Response Is Not Changed in Patients with Newly Diagnosed Idiopathic Intracranial Hypertension.

PubMed

Ba-Ali, Shakoor; Jensen, Rigmor Højland; Larsen, Line Sofie; Lund-Andersen, Henrik; Hamann, Steffen

2018-04-01

Previously, it has been reported that melanopsin-mediated pupillary light response (PLR), measured with pupillometry, is reduced in patients with idiopathic intracranial hypertension (IIH), indicating the clinical utility of the tool in the diagnosis of IIH. In the current study, the authors aimed to measure the PLR in 13 treatment-naive patients with new-onset IIH and 13 healthy controls. In contrast to the previous report, which was based on patients with longstanding IIH ( n = 13), the authors found no significant difference in the melanopsin-mediated PLR ( p = 0.48).

Quantitative nailfold video capillaroscopy in patients with idiopathic inflammatory myopathy.

PubMed

Mercer, Louise K; Moore, Tonia L; Chinoy, Hector; Murray, Andrea K; Vail, Andy; Cooper, Robert G; Herrick, Ariane L

2010-09-01

To quantify nailfold capillary density and dimensions in patients with idiopathic inflammatory myopathy (IIM) and compare them with those in healthy controls; to look for associations with microvascular disease in IIM; and to determine whether nailfold capillary density and dimensions change over time. Nailfold video microscopy (x300 magnification) was performed on 24 patients with IIM and 35 healthy controls. Capillary density and dimensions (total width and apical width) were quantified. Patients were clinically assessed and disease activity recorded using the Myositis Disease Activity Assessment Tool. Disease severity and physical function were assessed using the myositis damage index and Stanford HAQ, respectively. Findings were analysed using linear and logistic regression, adjusted for age and sex. In a subgroup of 16 patients with IIM and 27 controls, the process was repeated 6-12 months later and the results were analysed using Student's t-test. Capillary density was lower and dimensions were higher in patients with IIM compared with healthy controls (P < 0.001 for all). Anti-Jo-1 antibody was associated with reduced capillary density. In the longitudinal cohort, the mean change in capillary density was -1.4 in patients vs -0.4 in controls (P = 0.07). Mean change in capillary dimensions did not differ between patients and controls, but some patients demonstrated pronounced changes in capillary morphology over time. Reduced capillary density and increased dimensions in patients with IIM can be quantified using nailfold capillaroscopy, suggesting that nailfold capillaroscopy may be useful as an outcome measure of microvascular disease in studies of IIM.

An Open-Label Study of the Long-Term Safety of Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis (RECAP).

PubMed

Costabel, Ulrich; Albera, Carlo; Lancaster, Lisa H; Lin, Chin-Yu; Hormel, Philip; Hulter, Henry N; Noble, Paul W

2017-01-01

RECAP (NCT00662038) was an open-label extension study in patients with idiopathic pulmonary fibrosis (IPF) who completed either the Assessment of Pirfenidone to Confirm Efficacy and Safety in Idiopathic Pulmonary Fibrosis (ASCEND) 016 phase 3 trial or the Clinical Studies Assessing Pirfenidone in Idiopathic Pulmonary Fibrosis: Research of Efficacy and Safety Outcomes (CAPACITY) 004/006 phase 3 trials. To obtain long-term safety data for pirfenidone in patients with IPF in RECAP. Of the 1,334 patients who participated in the phase 3 trials, 1,058 entered RECAP. The final analysis from enrollment (September 2008) to June 2015 is presented. Mean (SD) and median (range) pirfenidone exposures in RECAP were 122 (98) weeks and 88 (>0 to 349) weeks, respectively, with a mean daily dose of 2,091.1 mg. Cumulative total exposure was 2,482 patient exposure years (PEY). The treatment-emergent adverse event (TEAE) rate was 701.9 per 100 PEY. The serious TEAE rate was 53.5 per 100 PEY, with the most common serious TEAE being IPF (11.1 per 100 PEY). Of the 231 deaths (9.3 per 100 PEY), the most common cause was IPF (5.4 per 100 PEY). The treatment discontinuation rate due to a TEAE was 17.9 per 100 PEY; discontinuations were due to IPF (7.2 per 100 PEY), pneumonia, respiratory failure, acute respiratory failure, rash (0.5 per 100 PEY each), and nausea (0.4 per 100 PEY). For patients from CAPACITY 004/006 who entered RECAP, the mean change in percent predicted forced vital capacity from RECAP baseline at 180 weeks was -9.6%. Median on-treatment survival from the first pirfenidone dose in RECAP was 77.2 months. RECAP provides long-term follow-up and safety data for pirfenidone that were consistent with the known profile, with no new safety signals observed. © 2017 The Author(s) Published by S. Karger AG, Basel.

Lung cancer in patients with idiopathic pulmonary fibrosis.

PubMed

Karampitsakos, Theodoros; Tzilas, Vasilios; Tringidou, Rodoula; Steiropoulos, Paschalis; Aidinis, Vasilis; Papiris, Spyros A; Bouros, Demosthenes; Tzouvelekis, Argyris

2017-08-01

Idiopathic pulmonary fibrosis (IPF) is a chronic fibrotic lung disease of unknown etiology. With a gradually increasing worldwide prevalence and a mortality rate exceeding that of many cancers, IPF diagnosis and management are critically important and require a comprehensive multidisciplinary approach. This approach also involves assessment of comorbid conditions, such as lung cancer, that exerts a dramatic impact on disease survival. Emerging evidence suggests that progressive lung scarring in the context of IPF represents a risk factor for lung carcinogenesis. Both disease entities present with major similarities in terms of pathogenetic pathways, as well as potential causative factors, such as smoking and viral infections. Besides disease pathogenesis, anti-cancer agents, including nintedanib, have been successfully applied in the treatment of patients with IPF while an oncologic approach with a cocktail of several pleiotropic anti-fibrotic agents is currently in the therapeutic pipeline of IPF. Nevertheless, epidemiologic association between IPF and lung cancer does not prove causality. Currently there is significant lack of knowledge supporting a direct association between lung fibrosis and cancer reflecting to disappointing therapeutic algorithms. An optimal therapeutic strategy for patients with both IPF and lung cancer represents an amenable need. This review article synthesizes the current state of knowledge regarding pathogenetic commonalities between IPF and lung cancer and focuses on clinical and therapeutic data that involve both disease entities. Copyright © 2017. Published by Elsevier Ltd.

Patient-controlled Intermittent Epidural Bolus Versus Epidural Infusion for Posterior Spinal Fusion After Adolescent Idiopathic Scoliosis: Prospective, Randomized, Double-blinded Study.

PubMed

Erdogan, Mehmet Ali; Ozgul, Ulku; Ucar, Muharrem; Korkmaz, Mehmet Fatih; Aydogan, Mustafa Said; Ozkan, Ahmet Selim; Colak, Cemil; Durmus, Mahmut

2017-06-15

A prospective, randomized, double-blinded study. The aim of this study was to compare the efficacy and side effects of patient-controlled intermittent bolus epidural analgesia (PCIEA) and patient-controlled continuous epidural analgesia (PCCEA) for postoperative pain control in adolescent idiopathic scoliosis. Epidural analgesia is an accepted efficacious and safe procedure for postoperative pain management in scoliosis surgery. However, the PCIEA has not been adequately investigated for postoperative pain control in adolescent idiopathic scoliosis. Forty-seven patients, 8 to 18 years of age, who were undergoing posterior spinal fusion for idiopathic scoliosis were randomized to either the PCIEA or PCCEA group. An epidural catheter was inserted by a surgeon under direct visualization. The PCIEA group received 0.2 mg/mL of morphine, 0.25 mL/kg of morphine bolus, additional doses of 0.25 mL/kg morphine with a 1-hour lockout given by patient-controlled demand, and no infusion. The PCCEA group received the following: 0.2 mg/mL morphine, an initial morphine loading set at 0.1 mL/kg, followed by a 0.05 mL/kg/h continuous infusion of morphine, and a 0.025 mL/kg bolus dose of morphine. There was a 30-minute lockout interval. The primary outcome was morphine usage. The secondary outcomes were pain score, postoperative nausea and vomiting, and pruritus. Cumulative morphine consumption was lower in the PCIEA group than in the PCCEA group. Both methods provided effective pain control. There were no differences in pain scores between the groups. Postoperative nausea, vomiting, and pruritus were lower in the PCIEA group. The two epidural analgesia techniques studied are both safe and effective methods for postoperative pain control after posterior spinal fusion in idiopathic scoliosis. Nausea, vomiting and pruritus were considerably higher in the PCCEA group. Concerns regarding side effects associated with epidural opioids can be avoided by an intermittent bolus

Gastric mucin expression in Helicobacter pylori-related, nonsteroidal anti-inflammatory drug-related and idiopathic ulcers

PubMed Central

Boltin, Doron; Halpern, Marisa; Levi, Zohar; Vilkin, Alex; Morgenstern, Sara; Ho, Samuel B; Niv, Yaron

2012-01-01

AIM: To determine the pattern of secreted mucin expression in Helicobacter pylori (H. pylori)-related, nonsteroidal anti-inflammatory drug (NSAID)-related and idiopathic gastric ulcers. METHODS: We randomly selected 92 patients with H. pylori-associated (n = 30), NSAID-associated (n = 18), combined H. pylori and NSAID-associated gastric ulcers (n = 24), and patients with idiopathic gastric ulcers (n = 20). Immunohistochemistry for T-cell CD4/CD8, and for mucin 5AC (MUC5AC) and mucin 6 (MUC6), was performed on sections of the mucosa from the ulcer margin. Inflammation score was assessed according to the Sydney system. RESULTS: MUC5AC was expressed on the surface epithelium (98.9%) and neck glands (98.9%) with minimal expression in the deep glands (6.5%). MUC6 was strongly expressed in the deep glands (97.8%), variable in the neck glands (19.6%) and absent in the surface epithelium (0%). The pattern of mucin expression in idiopathic ulcer margins was not different from the expression in ulcers associated with H. pylori, NSAIDs, or combined H. pylori and NSAIDs. CD4/CD8 ratio was higher in H. pylori-positive patients (P = 0.009). Idiopathic ulcers are associated with hospitalized patients and have higher bleeding and mortality rates. CONCLUSION: Idiopathic ulcers have a unique clinical profile. Gastric mucin expression in idiopathic gastric ulcers is unchanged compared with H. pylori and/or NSAID-associated ulcers. PMID:22969235

High frequency of TTTY2-like gene-related deletions in patients with idiopathic oligozoospermia and azoospermia.

PubMed

Yapijakis, C; Serefoglou, Z; Papadimitriou, K; Makrinou, E

2015-06-01

Genes located on Y chromosome and expressed in testis are likely to be involved in spermatogenesis. TTTY2 is a Y-linked multicopy gene family of unknown function that includes TTTY2L2A and TTTY2L12A at Yq11 and Yp11 loci respectively. Using PCR amplification, we screened for TTTY2L2A- and TTTY2L12A-associated deletions, in 94 Greek men with fertility problems. Patients were divided into three groups as following: group A (n = 28) included men with idiopathic moderate oligozoospermia, group B (n = 34) with idiopathic severe oligozoospermia and azoospermia, and group C (n = 32) with oligo- and azoospermia of various known etiologies. No deletions were detected in group C patients and 50 fertile controls. However, two patients from group A had deletions in TTTY2L2A (7.1%) and six in TTTY2L12A (21.4%), whereas from group B, four patients had deletions in TTTY2L2A (11.8%) and 10 in TTTY2L12A (29.4%). In addition, five patients from both groups A and B (8%) appeared to have deletions in both studied TTTY2 genes, although these are located very far apart. These results indicate that the TTTY2 gene family may play a significant role in spermatogenesis and suggest a possible mechanism of nonhomologous recombinational events that may cause genomic instability and ultimately lead to male infertility. © 2014 Blackwell Verlag GmbH.

9 CFR 311.22 - Hogs affected with urticaria, tinea tonsurans, demodex follicurlorum, or erythema.

Code of Federal Regulations, 2012 CFR

2012-01-01

... folliculorum, or erythema may be passed for human food after detaching and condemning the affected skin, if the... 9 Animals and Animal Products 2 2012-01-01 2012-01-01 false Hogs affected with urticaria, tinea tonsurans, demodex follicurlorum, or erythema. 311.22 Section 311.22 Animals and Animal Products FOOD SAFETY...

9 CFR 311.22 - Hogs affected with urticaria, tinea tonsurans, demodex follicurlorum, or erythema.

Code of Federal Regulations, 2011 CFR

2011-01-01

... folliculorum, or erythema may be passed for human food after detaching and condemning the affected skin, if the... 9 Animals and Animal Products 2 2011-01-01 2011-01-01 false Hogs affected with urticaria, tinea tonsurans, demodex follicurlorum, or erythema. 311.22 Section 311.22 Animals and Animal Products FOOD SAFETY...

9 CFR 311.22 - Hogs affected with urticaria, tinea tonsurans, demodex follicurlorum, or erythema.

Code of Federal Regulations, 2014 CFR

2014-01-01

... folliculorum, or erythema may be passed for human food after detaching and condemning the affected skin, if the... 9 Animals and Animal Products 2 2014-01-01 2014-01-01 false Hogs affected with urticaria, tinea tonsurans, demodex follicurlorum, or erythema. 311.22 Section 311.22 Animals and Animal Products FOOD SAFETY...

9 CFR 311.22 - Hogs affected with urticaria, tinea tonsurans, demodex follicurlorum, or erythema.

Code of Federal Regulations, 2013 CFR

2013-01-01

... folliculorum, or erythema may be passed for human food after detaching and condemning the affected skin, if the... 9 Animals and Animal Products 2 2013-01-01 2013-01-01 false Hogs affected with urticaria, tinea tonsurans, demodex follicurlorum, or erythema. 311.22 Section 311.22 Animals and Animal Products FOOD SAFETY...

Circulating soluble LIGHT/TNFSF14 is increased and associated with IL-8 concentration in chronic spontaneous urticaria.

PubMed

Kasperska-Zając, Alicja; Damasiewicz-Bodzek, Aleksandra; Grzanka, Ryszard; Skrzypulec-Frankel, Agnieszka; Bieniek, Katarzyna; Sikora-Żydek, Agnieszka; Jochem, Jerzy

2018-01-01

LIGHT (homologous to lymphotoxins, exhibiting inducible expression, and competing with herpes simplex virus (HSV) glycoprotein D for herpes virus entry mediator (HVEM), a receptor expressed by T lymphocytes) has been involved in various autoimmune and inflammatory disorders. LIGHT induces the expression of interleukin-8 (IL-8), which is up-regulated in chronic spontaneous urticaria (CSU). To determine circulating soluble LIGHT concentration and its relationship with IL-8 concentration in patients with CSU. Concentrations of LIGHT, IL-8, and C-reactive protein (CRP) were determined in plasma or serum of CSU patients by an enzyme-linked immunosorbent assay. LIGHT plasma concentration was significantly higher in moderate-severe CSU patients as compared with the healthy subjects, but not with mild CSU patients. There were significant correlations between increased LIGHT and IL-8 concentrations, but not with increased CRP in CSU patients. Enhanced plasma concentrations of soluble LIGHT and its association with IL-8 concentration suggest the role of LIGHT in systemic inflammatory activation in CSU patients. We hypothesize that LIGHT-mediated immune-inflammatory response plays a role in severe phenotypes of the disease.

Clinical and nutritional outcomes in children with idiopathic superior mesenteric artery syndrome.

PubMed

Shiu, Jr-Rung; Chao, Hsun-Chin; Luo, Chih-Cheng; Lai, Ming-Wei; Kong, Man-Shan; Chen, Shih-Yen; Chen, Chien-Chang; Wang, Chao-Jan

2010-08-01

There are no available data for outcomes in children's idiopathic superior mesenteric artery syndrome (SMAS) strictly treated conservatively. The aim of the study was to evaluate clinical and nutritional outcome in children with idiopathic SMAS. A 1-year prospective observation study of effects of treatment and outcome was performed in 27 children (8 boys, 19 girls) with idiopathic SMAS who underwent an upper gastrointestinal (UGI) series, ultrasound measurement of the aortomesenteric angle, treatment, clinical assessment, growth evaluation, and regular clinical visits for more than 12 months. Mean age of the patients was 11.77 +/- 2.15 years. The major clinical complaints were postprandial pain or fullness (88.9%), vomiting (55.6%), and early satiety (51.9%). Eight patients (29.6%) had weight loss. The UGI series revealed typical features of SMAS. The aortomesenteric angle on ultrasound was 10 degrees to 19 degrees. The height of most patients (92.6%) was above the 10th percentile, whereas 15 (55.6%) patients weighed below the 10th percentile. Six patients underwent surgical intervention (3 for obstruction and 3 for persistent anorexia with weight loss), and their clinical symptoms and weight status improved steadily during the follow-up months. Among the 21 patients not subject to surgical intervention, 11 (52.4%) experienced a reduction of symptoms >50% after 3 months of treatment, and weight-for-age percentile increased significantly after 6 months of treatment. Overall, a significant increase in the weight-for-age status was seen in the patients with surgical treatment or with medication only after 6 and 12 months of treatment. An aortomesenteric angle <20 degrees is a constant phenomenon in children with idiopathic SMAS. A duodenojejunostomy can effectively relieve the obstructive symptoms, such as anorexia, and improve nutritional status, whereas long-term medical treatment may aid in relieving the clinical symptoms, promoting appetite, and improving

Echocardiography-based spectrum of severe tricuspid regurgitation: the frequency of apparently idiopathic tricuspid regurgitation.

PubMed

Mutlak, Diab; Lessick, Jonathan; Reisner, Shimon A; Aronson, Doron; Dabbah, Salim; Agmon, Yoram

2007-04-01

The cause of tricuspid valve (TV) regurgitation (TR) occasionally remains unclear. The objectives of our study were to define the causal spectrum of severe TR diagnosed by echocardiography at a tertiary medical center and to assess the relative frequency and determine the clinical and echocardiographic characteristics of TR without an apparent cause (idiopathic TR). Consecutive patients with severe TR were identified by the echocardiography laboratory computerized database. The echocardiographic reports of all patients were reviewed and the causes of TR were determined. The echocardiographic studies and medical charts were reviewed in patients without an obvious cause of TR. Of 242 consecutive patients diagnosed with severe TR, organic TV disease was evident in 23 patients (9.5%) and significant pulmonary hypertension (estimated pulmonary artery systolic pressure > 50 mm Hg) in an additional 157 patients (64.9%). After further excluding patients with various confounding factors, possibly associated with occult organic TV disease or significant pulmonary hypertension, 23 patients (9.5%) had severe TR without an apparent cause. Of these, TV coaptation appeared relatively intact, allowing adequate estimation of pulmonary artery pressure, in 15 patients (6.2% of all patients with severe TR; idiopathic TR group). Patients with idiopathic TR were older (76 +/- 10 years), with a high frequency of atrial fibrillation (93%), and prominent TV annular dilatation. After excluding multiple potential causes of TR, severe TR is occasionally idiopathic. Annular dilatation (secondary to aging, atrial fibrillation, or other causes) is the likely mechanism of TR in these patients.

Expression of Mas-related gene X2 on mast cells is upregulated in the skin of patients with severe chronic urticaria.

PubMed

Fujisawa, Daisuke; Kashiwakura, Jun-Ichi; Kita, Hirohito; Kikukawa, Yusuke; Fujitani, Yasushi; Sasaki-Sakamoto, Tomomi; Kuroda, Kazumichi; Nunomura, Satoshi; Hayama, Koremasa; Terui, Tadashi; Ra, Chisei; Okayama, Yoshimichi

2014-09-01

Wheal reactions to intradermally injected neuropeptides, such as substance P (SP) and vasoactive intestinal peptide, are significantly larger and longer lasting in patients with chronic urticaria (CU) than in nonatopic control (NC) subjects. Mas-related gene X2 (MrgX2) has been identified as a receptor for basic neuropeptides, such as SP and vasoactive intestinal peptide. Mast cell (MC) responsiveness to eosinophil mediators contributes to the late-phase reaction of allergy. We sought to compare the frequency of MrgX2 expression in skin MCs from patients with CU and NC subjects and to identify the receptor for basic eosinophil granule proteins on human skin MCs. MrgX2 expression was investigated by using immunofluorescence in skin tissues from NC subjects and patients with severe CU and on skin-derived cultured MCs. MrgX2 expression in human MCs was reduced by using a lentiviral small hairpin RNA silencing technique. Ca(2+) influx was measured in CHO cells transfected with MrgX2 in response to eosinophil granule proteins. Histamine and prostaglandin D2 levels were measured by using enzyme immunoassays. The number of MrgX2(+) skin MCs and the percentage of MrgX2(+) MCs in all MCs in patients with CU were significantly greater than those in NC subjects. Eosinophil infiltration in urticarial lesions was observed in 7 of 9 patients with CU. SP, major basic protein, and eosinophil peroxidase, but not eosinophil-derived neurotoxin, induced histamine release from human skin MCs through MrgX2. MrgX2 might be a new target molecule for the treatment of wheal reactions in patients with severe CU. Copyright © 2014 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.

Idiopathic hypersomnia.

PubMed

Billiard, Michel; Sonka, Karel

2016-10-01

Idiopathic hypersomnia continues to evolve from the concept of "sleep drunkenness" introduced by Bedrich Roth in Prague in 1956 and the description of idiopathic hypersomnia with two forms, polysymptomatic and monosymptomatic, by the same Bedrich Roth in 1976. The diagnostic criteria of idiopathic hypersomnia have varied with the successive revisions of the International classifications of sleep disorders, including the recent 3rd edition. No epidemiological studies have been conducted so far. Disease onset occurs most often during adolescence or young adulthood. A familial background is often present but rigorous studies are still lacking. The key manifestation is hypersomnolence. It is often accompanied by sleep of long duration and debilitating sleep inertia. Polysomnography (PSG) followed by a multiple sleep latency test (MSLT) is mandatory, as well as a 24 h PSG or a 2-wk actigraphy in association with a sleep log to ensure a total 24-h sleep time longer than or equal to 66O minutes, when the mean sleep latency on the MSLT is longer than 8 min. Yet, MSLT is neither sensitive nor specific and the polysomnographic diagnostic criteria require continuous readjustment and biologic markers are still lacking. Idiopathic hypersomnia is most often a chronic condition though spontaneous remission may occur. The condition is disabling, sometimes even more so than narcolepsy type 1 or 2. Based on neurochemical, genetic and immunological analyses as well as on exploration of the homeostatic and circadian processes of sleep, various pathophysiological hypotheses have been proposed. Differential diagnosis involves a number of diseases and it is not yet clear whether idiopathic hypersomnia and narcolepsy type 2 are not the same condition. Until now, the treatment of idiopathic hypersomnia has mirrored that of the sleepiness of narcolepsy type 1 or 2. The first randomized, double-blind, placebo-controlled trials of modafinil have just been published, as well as a double

Clinical characteristics of 138 Chinese female patients with idiopathic hypogonadotropic hypogonadism

PubMed Central

Tang, Rui-yi; Ma, Miao; Lin, Shou-qing; Zhang, Yi-wen; Wang, Ya-ping

2017-01-01

Objective To evaluate the clinical features of Chinese women with idiopathic hypogonadotropic hypogonadism (IHH). Methods We retrospectively reviewed the clinical characteristics, laboratory and imaging findings, therapeutic management and fertility outcomes of 138 women with IHH. All patients had been treated and followed up at an academic medical centre during 1990–2016. Results Among the 138 patients, 82 patients (59.4%) were diagnosed with normosmic IHH and 56 patients (40.6%) were diagnosed with Kallmann syndrome (KS). The patients with IHH experienced occasional menses (4.3%), spontaneous thelarche (45.7%) or spontaneous pubarche (50.7%). Women with thelarche had a higher percentage of pubarche (P < 0.001) and higher gonadotropin concentrations (P < 0.01). Olfactory bulb/sulci abnormalities were found during the magnetic resonance imaging (MRI) of all patients with KS. Most patients with IHH had osteopenia and low bone age. Among the 16 women who received gonadotropin-releasing hormone treatment, ovulation induction or assisted reproductive technology, the clinical pregnancy rate was 81.3% and the live birth rate was 68.8%. Conclusions The present study revealed that the phenotypic spectrum of women with IHH is broader than typical primary amenorrhoea with no secondary sexual development, including occasional menses, spontaneous thelarche or pubarche. MRI of the olfactory system can facilitate the diagnosis of KS. Pregnancy can be achieved after receiving appropriate treatment. PMID:29018155

Outcome of Radiofrequency Catheter Ablation as a non-pharmacological therapy for idiopathic Ventricular Tachycardia.

PubMed

Samore, Naseer Ahmed; Imran Majeed, Syed Muhammad; Kayani, Azhar Mahmud; Bhalli, Muhammad Asif; Shabbir, Muhammad

2009-09-01

To determine the outcome of Radiofrequency Catheter Ablation (RFCA) as a non-pharmacological curative therapy for idiopathic Ventricular Tachycardia (VT) and to identify procedure-related complications. Descriptive study. The Armed Forces Institute of Cardiology and National Institute of Heart Diseases, Rawalpindi, from February 2001 to October 2008. Ninety eight consecutive patients with idiopathic VT, resistant to drug therapy, who underwent Electrophysiology Studies (EPS) radiofrequency catheter ablation were enrolled. Clinical and electrophysiological variables were recorded and a descriptive analysis was done. Out of the 98 patients, 79 were males (80.6%). The mean age was 33.29+11.93 years. Modes of presentation were sustained VT, Repetitive Monomorphic VT (RMVT), Non-sustained VT (NSVT) and Ventricular Premature Beats (VPBs). Right Ventricular Outflow Tract (RVOT) VT was found in 37 patients, 37 had Idiopathic Left Ventricular Tachycardia (ILVT), 20 had Left Ventricular Outflow Tract (LVOT) VT, and Inflow Right Ventricular Tachycardia (IRVT) was found in 7 patients. Other sites of origin of VT were infrequent. Eight patients had dual morphologies of VT. Atrioventricular Nodal Re-entry Tachycardia (AVNRT) was found in 8 patients. RFCA was successful in abolishing inducible VT in 88 patients. One patient developed complete AV block requiring a permanent pacemaker. Results of this study confirm a high degree of success and safety of radiofrequency catheter ablation as curative therapy for idiopathic ventricular tachycardia.

Case Report of a Patient With Idiopathic Hypersomnia and a Family History of Malignant Hyperthermia Undergoing General Anesthesia: An Overview of the Anesthetic Considerations.

PubMed

Aflaki, Sena; Hu, Sally; Kamel, Rami A; Chung, Frances; Singh, Mandeep

2017-05-01

The pathophysiologic underpinnings of idiopathic hypersomnia and its interactions with anesthetic medications remain poorly understood. There is a scarcity of literature describing this patient population in the surgical setting. This case report outlines the anesthetic considerations and management plan for a 55-year-old female patient with a known history of idiopathic hypersomnia undergoing an elective shoulder arthroscopy in the ambulatory setting. In addition, this case offers a unique set of considerations and conflicts related to the patient having a family history of malignant hyperthermia. A combined technique of general and regional anesthesia was used. Anesthesia was maintained with total intravenous anesthesia via the use of propofol and remifentanil. The depth of anesthesia was monitored with entropy. There were no perioperative complications.

Impact of a disease-management program on symptom burden and health-related quality of life in patients with idiopathic pulmonary fibrosis and their care partners.

PubMed

Lindell, Kathleen Oare; Olshansky, Ellen; Song, Mi-Kyung; Zullo, Thomas G; Gibson, Kevin F; Kaminski, Naftali; Hoffman, Leslie A

2010-01-01

Patients were recruited from the Dorothy P. and Richard P. Simmons Center for Interstitial Lung Disease, located within the University of Pittsburgh Medical Center. Idiopathic pulmonary fibrosis results in scarring of the lung and respiratory failure, and has a median survival of 3 to 5 years from the time of diagnosis. The purpose of this study was to determine whether patients with idiopathic pulmonary fibrosis and their care partners could be more optimally managed by a disease-management intervention entitled "Program to Reduce Idiopathic Pulmonary Fibrosis Symptoms and Improve Management," which nurses delivered using the format of a support group. We hypothesized that participation would improve perceptions of health-related quality of life (HRQoL) and decrease symptom burden. Subjects were 42 participants randomized to an experimental (10 patient/care partner dyads) or control (11 patient/care partner dyads) group. Experimental group participants attended the 6-week program, and controls received usual care. Before and after the program, all participants completed questionnaires designed to assess symptom burden and HRQoL. Patients and care partners in the intervention group were also interviewed in their home to elicit information on their experience after participating in the Program to Reduce Idiopathic Pulmonary Fibrosis Symptoms and Improve Management. After the intervention, experimental group patients rated their HRQoL less positively (P = .038) and tended to report more anxiety (P = .077) compared with controls. Care partners rated their stress at a lower level (P = .018) compared with controls. Course evaluations were uniformly positive. Post-study qualitative interviews with experimental group participants suggested benefits not exemplified by these scores. Patient participants felt less isolated, were able to put their disease into perspective, and valued participating in research and helping others. Further exploration of the impact of disease

Impact of a disease-management program on symptom burden and health-related quality of life in patients with idiopathic pulmonary fibrosis and their care partners

PubMed Central

Lindell, Kathleen Oare; Olshansky, Ellen; Song, Mi-Kyung; Zullo, Thomas G.; Gibson, Kevin F.; Kaminski, Naftali; Hoffman, Leslie A.

2012-01-01

BACKGROUND Patients were recruited from the Dorothy P. and Richard P. Simmons Center for Interstitial Lung Disease, located within the University of Pittsburgh Medical Center. Idiopathic pulmonary fibrosis results in scarring of the lung and respiratory failure, and has a median survival of 3 to 5 years from the time of diagnosis. The purpose of this study was to determine whether patients with idiopathic pulmonary fibrosis and their care partners could be more optimally managed by a disease-management intervention entitled “Program to Reduce Idiopathic Pulmonary Fibrosis Symptoms and Improve Management,” which nurses delivered using the format of a support group. We hypothesized that participation would improve perceptions of health-related quality of life (HRQoL) and decrease symptom burden. METHODS Subjects were 42 participants randomized to an experimental (10 patient/care partner dyads) or control (11 patient/care partner dyads) group. Experimental group participants attended the 6-week program, and controls received usual care. Before and after the program, all participants completed questionnaires designed to assess symptom burden and HRQoL. Patients and care partners in the intervention group were also interviewed in their home to elicit information on their experience after participating in the Program to Reduce Idiopathic Pulmonary Fibrosis Symptoms and Improve Management. RESULTS After the intervention, experimental group patients rated their HRQoL less positively (P = .038) and tended to report more anxiety (P = .077) compared with controls. Care partners rated their stress at a lower level (P = .018) compared with controls. Course evaluations were uniformly positive. Post-study qualitative interviews with experimental group participants suggested benefits not exemplified by these scores. Patient participants felt less isolated, were able to put their disease into perspective, and valued participating in research and helping others. CONCLUSION

One-year safety and efficacy study of bilastine treatment in Japanese patients with chronic spontaneous urticaria or pruritus associated with skin diseases.

PubMed

Yagami, Akiko; Furue, Masutaka; Togawa, Michinori; Saito, Akihiro; Hide, Michihiro

2017-04-01

A number of second-generation non-sedating antihistamines are used in clinical practices over the world. However, long-term safety and efficacy have not been proved high level evidence based medicine. We have performed an open-label, multicenter, phase III study to evaluate the long-term safety and efficacy of bilastine, a novel non-sedating H 1 -antihistamine for patients with chronic spontaneous urticaria (CSU) or pruritus associated with skin diseases (trial registration no. JapicCTI-142528). Patients aged 18-74 years were treated with bilastine 20 mg once daily for up to 52 weeks. Safety and tolerability were assessed on the basis of adverse events (AE), bilastine-related AE, laboratory tests and vital signs. Efficacy was assessed based on rash score, itch score, overall improvement and quality of life. One hundred and ninety-eight patients enrolled, 122 of whom (61.6%) completed the 52-week treatment period. AE were reported in 64.5% and bilastine-related AE in 2.5% of patients throughout the 52-week treatment period. All AE were mild to moderate in severity. AE associated with the nervous system occurred in 10 patients (5.1%) including seven patients (3.6%) with headache. Somnolence reported in two of these patients (1.0%) was related to bilastine. All efficacy variables improved during treatment with bilastine. In conclusion, long-term treatment with bilastine 20 mg once daily for 52 weeks is safe and well tolerated in Japanese patients with CSU or pruritus associated with skin diseases. Bilastine improved disease symptoms of both conditions early in treatment, and the efficacy was maintained throughout the treatment. © 2016 The Authors. The Journal of Dermatology published by John Wiley & Sons Australia, Ltd on behalf of Japanese Dermatological Association.

Computational modeling of venous sinus stenosis in idiopathic intracranial hypertension

PubMed Central

Levitt, Michael R; McGah, Patrick M; Moon, Karam; Albuquerque, Felipe C; McDougall, Cameron G; Kalani, M Yashar S; Kim, Louis J; Aliseda, Alberto

2016-01-01

Background and Purpose Idiopathic intracranial hypertension has been associated with dural venous sinus stenosis in some patients, but the hemodynamic environment of the dural venous sinuses has not been quantitatively described. Here, we present the first such computational fluid dynamics model using patient-specific blood pressure measurements. Materials and Methods Six patients with idiopathic intracranial hypertension and at least one stenosis or atresia at the transverse-sigmoid sinus junction underwent MRV followed by cerebral venography and manometry throughout the dural venous sinuses. Patient-specific computational fluid dynamics models were created using MRV anatomy, with venous pressure measurements as boundary conditions. Blood flow and wall shear stress were calculated for each patient. Results Computational models of dural venous sinuses were successfully reconstructed in all six patients with patient-specific boundary conditions. Three patients demonstrated a pathologic pressure gradient (≥ 8 mm Hg) across four dural venous sinus stenoses. Small sample size precludes statistical comparisons, but average overall flow throughout the dural venous sinuses of patients with pathologic pressure gradients was higher than in those without (1041.00 ± 506.52 vs. 358.00 ± 190.95 mL/min). Wall shear stress was also higher across stenoses in patients with pathologic pressure gradients (37.66 ± 48.39 vs 7.02 ± 13.60 Pa). Conclusion The hemodynamic environment of the dural venous sinuses can be computationally modeled using patient-specific anatomy and physiological measurements in patients with idiopathic intracranial hypertension. There was substantially higher blood flow and wall shear stress in patients with pathological pressure gradients. PMID:27197986

Flow void of cerebrospinal fluid in idiopathic normal pressure hydrocephalus of the elderly: can it predict outcome after shunting?

PubMed

Krauss, J K; Regel, J P; Vach, W; Jüngling, F D; Droste, D W; Wakhloo, A K

1997-01-01

We investigate the predictive value of cerebrospinal fluid (CSF) flow void on outcome after shunting in a prospective series of patients with idiopathic normal pressure hydrocephalus (NPH). The degree and extension of CSF flow void were examined on T2-weighted magnetic resonance imaging scans of 37 elderly patients with idiopathic NPH who underwent subsequent shunting. The degree of flow void was assessed in comparison with the signal of large cerebral arteries. The extension was evaluated via the calculation of sum scores for the occurrence of flow void in different locations of the ventricular system. Those parameters were not considered in the decision to perform shunting. CSF flow void in the aqueduct and the adjacent third and fourth ventricles of the 37 patients with idiopathic NPH was compared with that of 37 age-matched control patients. CSF flow void scores in patients with idiopathic NPH were investigated for correlations between postoperative outcome scores and ventricular width indices. No difference was found between the occurrence of aqueductal CSF flow void in patients with idiopathic NPH and the control group. A significant difference, however, was noted for the extension of the CSF flow void, which was greater in the NPH group. Postoperative improvement was found in 33 of 37 patients with idiopathic NPH at a mean follow-up of 15.6 months. Only small, statistically not significant correlations were found between CSF flow void and postoperative outcome. Flow void sum scores, however, correlated significantly with ventricular width indices. The degree and extension of CSF flow void on T2-weighted magnetic resonance imaging scans have little predictive value for outcome after shunting in patients with idiopathic NPH. The greater extension of the CSF flow void in patients with NPH is most likely related to increased ventricular width. It is not useful to consider CSF flow void findings on conventional magnetic resonance imaging scans in making the

Eliminating the use of allogeneic blood products in adolescent idiopathic scoliosis surgery.

PubMed

Berney, Mark J; Dawson, Peter H; Phillips, Margaret; Lui, Darren F; Connolly, Paul

2015-07-01

The aim of this study was to compare transfusion requirements in patients before and after the introduction of tranexamic acid as standard in patients undergoing spinal surgery for idiopathic scoliosis in a national orthopaedic hospital. A retrospective chart review of 56 idiopathic scoliosis patients who underwent posterior spinal instrumentation and fusion between 2009 and 2013 at our institution. Preoperative, intraoperative, and postoperative data were measured. Patients who received tranexamic acid as standard (n = 31) showed a trend towards a decrease in transfusion requirements compared with those who received no tranexamic acid (n = 25). These patients had a statistically significant decrease in operative time (223 vs 188 min, p = 0.005), and estimated intraoperative blood loss was reduced by nearly 50% in the tranexamic acid group. They also had an associated reduced decrease in haemoglobin between preoperative and postoperative levels (4 vs 5 g/dL, p = 0.01). Since February 2012, no patient has required intraoperative or postoperative allogeneic blood product transfusion in this hospital. The routine use of antifibrinolytic medications in patients undergoing surgery for adolescent idiopathic scoliosis has effectively eliminated the need for allogeneic blood products.

Lack of evidence for central sensitization in idiopathic, non-traumatic neck pain: a systematic review.

PubMed

Malfliet, Annaleen; Kregel, Jeroen; Cagnie, Barbara; Kuipers, Mandy; Dolphens, Mieke; Roussel, Nathalie; Meeus, Mira; Danneels, Lieven; Bramer, Wichor M; Nijs, Jo

2015-01-01

Chronic neck pain is a common problem with a poorly understood pathophysiology. Often no underlying structural pathology can be found and radiological imaging findings are more related to age than to a patient's symptoms. Besides its common occurrence, chronic idiopathic neck pain is also very disabling with almost 50% of all neck pain patients showing moderate disability at long-term follow-up. Central sensitization (CS) is defined as "an amplification of neural signaling within the central nervous system that elicits pain hypersensitivity," "increased responsiveness of nociceptive neurons in the central nervous system to their normal or subthreshold afferent input," or "an augmentation of responsiveness of central neurons to input from unimodal and polymodal receptors." There is increasing evidence for involvement of CS in many chronic pain conditions. Within the area of chronic idiopathic neck pain, there is consistent evidence for the presence and clinical importance of CS in patients with traumatic neck pain, or whiplash-associated disorders. However, the majority of chronic idiopathic neck pain patients are unrelated to a traumatic injury, and hence are termed chronic idiopathic non-traumatic neck pain. When comparing whiplash with idiopathic non-traumatic neck pain, indications for different underlying mechanisms are found. The goal of this article was to review the existing scientific literature on the role of CS in patients with chronic idiopathic non-traumatic neck pain. Systematic review. All selected studies were case control studies. A systematic search of existing, relevant literature was performed via the electronic databases Medline, Embase, Web of Science, Cinahl, PubMed, and Google Scholar. All titles and abstracts were checked to identify relevant articles. An article was considered eligible if it met following inclusion criteria: (1) participants had to be human adults (> 18 years) diagnosed with idiopathic non-traumatic chronic (present for at

Comparison of CSF Distribution between Idiopathic Normal Pressure Hydrocephalus and Alzheimer Disease.

PubMed

Yamada, S; Ishikawa, M; Yamamoto, K

2016-07-01

CSF volumes in the basal cistern and Sylvian fissure are increased in both idiopathic normal pressure hydrocephalus and Alzheimer disease, though the differences in these volumes in idiopathic normal pressure hydrocephalus and Alzheimer disease have not been well-described. Using CSF segmentation and volume quantification, we compared the distribution of CSF in idiopathic normal pressure hydrocephalus and Alzheimer disease. CSF volumes were extracted from T2-weighted 3D spin-echo sequences on 3T MR imaging and quantified semi-automatically. We compared the volumes and ratios of the ventricles and subarachnoid spaces after classification in 30 patients diagnosed with idiopathic normal pressure hydrocephalus, 10 with concurrent idiopathic normal pressure hydrocephalus and Alzheimer disease, 18 with Alzheimer disease, and 26 control subjects 60 years of age or older. Brain to ventricle ratios at the anterior and posterior commissure levels and 3D volumetric convexity cistern to ventricle ratios were useful indices for the differential diagnosis of idiopathic normal pressure hydrocephalus or idiopathic normal pressure hydrocephalus with Alzheimer disease from Alzheimer disease, similar to the z-Evans index and callosal angle. The most distinctive characteristics of the CSF distribution in idiopathic normal pressure hydrocephalus were small convexity subarachnoid spaces and the large volume of the basal cistern and Sylvian fissure. The distribution of the subarachnoid spaces in the idiopathic normal pressure hydrocephalus with Alzheimer disease group was the most deformed among these 3 groups, though the mean ventricular volume of the idiopathic normal pressure hydrocephalus with Alzheimer disease group was intermediate between that of the idiopathic normal pressure hydrocephalus and Alzheimer disease groups. The z-axial expansion of the lateral ventricle and compression of the brain just above the ventricle were the common findings in the parameters for differentiating

3-(Bromomethyl)-2-chloro-4-(methylsulfonyl)- benzoic acid: a new cause of sensitiser induced occupational asthma, rhinitis and urticaria.

PubMed

Suojalehto, Hille; Karvala, Kirsi; Ahonen, Saana; Ylinen, Katriina; Airaksinen, Liisa; Suuronen, Katri; Suomela, Sari; Lindström, Irmeli

2018-04-01

3-(Bromomethyl)-2-chloro-4-(methylsulfonyl)-benzoic acid (BCMBA) has not previously been identified as a respiratory sensitiser. We detected two cases who presented respiratory and urticaria symptoms related to BCMBA and had positive skin prick tests to the agent. Subsequently, we conducted outbreak investigations at the BCMBA-producing factory and performed clinical examinations to confirm occupational diseases. The outbreak investigations included observations of work processes, assessment of exposure, a medical survey with a questionnaire and skin prick tests with 0.5% BCMBA water solution on 85 exposed workers and 9 unexposed workers. We used specific inhalation or nasal challenge and open skin application test to investigate BCMBA-related occupational asthma, rhinitis and contact urticaria. We identified nine workers with respiratory and/or skin symptoms and positive skin prick tests to BCMBA in a chemical factory. A survey among chemical factory workers indicated a BCMBA-related sensitisation rate of 8% among all exposed workers; the rate was highest (25%) among production workers in the production hall. Sensitisation was detected only in workers with the estimated highest exposure levels. Six cases of occupational asthma, rhinitis and/or contact urticaria caused by BCMBA were confirmed with challenge tests. Asthma-provoking doses in specific inhalation challenges were very low (0.03% or 0.3% BCMBA in lactose). We identified a new low molecular weight agent causing occupational asthma, rhinitis and contact urticaria. A typical clinical picture of allergic diseases and positive skin prick tests suggest underlying IgE-mediated disease mechanisms. Stringent exposure control measures are needed in order to prevent BCMBA-related diseases. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Idiopathic granulomatous mastitis: case report and review of the literature.

PubMed

Imoto, S; Kitaya, T; Kodama, T; Hasebe, T; Mukai, K

1997-08-01

We report a case of idiopathic granulomatous mastitis in a 35-year-old Japanese woman, who came to our hospital complaining of a tender mass in her right breast. Because the results of initial aspiration cytology were considered highly suspicious for carcinoma, modified radical mastectomy was performed. However, the final histological diagnosis was granulomatous lobular mastitis with no evidence of malignancy. Idiopathic granulomatous mastitis is a rare inflammatory breast disease of unknown etiology. Since the clinical manifestations are similar to those of mammary carcinoma, this condition has been misdiagnosed as carcinoma and treated as such. A review of the literature revealed that idiopathic granulomatous mastitis has tended to occur in young patients with a history of childbirth or oral contraceptive usage. Clinical or imaging diagnosis has often been difficult. Complete resection or corticosteroid therapy can be recommended as the optimal treatment. Since 38% of patients experience recurrence, long-term follow-up is indicated.

Dynamic patient counseling: a novel concept in idiopathic pulmonary fibrosis.

PubMed

Brown, A Whitney; Shlobin, Oksana A; Weir, Nargues; Albano, Maria C; Ahmad, Shahzad; Smith, Mary; Leslie, Kevin; Nathan, Steven D

2012-10-01

The characteristics of long-term survivors with idiopathic pulmonary fibrosis (IPF) have never been fully elucidated. We sought to illustrate the attenuated mortality and describe the characteristics of patients with IPF who survived at least 5 years beyond their initial presentation. Patients with IPF evaluated between 1997 and 2006 were identified through the clinic database. Patients who survived beyond 5 years from the time of their evaluation were compared with those who died or underwent lung transplantation within 5 years. Survival analyses were performed from the time of initial evaluation and contingent on annualized survival thereafter. Eighty-seven patients who survived at least 5 years formed the comparator group to whom other patients were contrasted. These patients had a higher BMI, FVC % predicted, FEV1 % predicted, total lung capacity % predicted, and diffusing capacity of lung for carbon monoxide % predicted, but a lower FEV1/FVC ratio and lower mean pulmonary artery pressures. More than one-half of these patients had moderate or severe disease at the time of presentation. Our annualized contingent survival analyses revealed a progressively increasing median survival dependent on the duration of the disease. Although we were able to demonstrate differences in our 5-year survivors, rather than being a distinct group, these patients appear to exist within a continuum of improving survival dependent on prior disease duration. This progressively improving time-dependent prognosis mandates the serial reevaluation of an individual patient’s projected outcomes. The implementation of dynamic counseling is an important concept in more accurately predicting life expectancy for patients with IPF who are frequently haunted by the prospects of a dismal survival.

The prognostic impact of dynamic ventricular dyssynchrony in patients with idiopathic dilated cardiomyopathy and narrow QRS.

PubMed

D'Andrea, Antonello; Mele, Donato; Nistri, Stefano; Riegler, Lucia; Galderisi, Maurizio; Agricola, Eustachio; Losi, Maria Angela; Ballo, Piercarlo; Mondillo, Sergio; Badano, Luigi P

2013-02-01

Asynchronous myocardial contraction adversely influences left ventricular (LV) function and is therefore associated with a poor prognosis in heart failure. Exercise-induced change in ventricular dyssynchrony may be an important determinant of dynamic changes in cardiac output and mitral regurgitation. A prospective, longitudinal study was designed with pre-defined dyssynchrony index and outcome variables to test the hypothesis that dynamic dyssynchrony is associated with worse long-term event-free survival in patients with dilated cardiomyopathy (DCM) and 'narrow' QRS complex. One-hundred eighty patients (62 ± 8 years; 110 males) with NYHA class II-III, idiopathic DCM, ejection fraction ≤35%, and QRS duration <120 ms were selected. All the patients underwent standard Doppler echo, colour tissue velocity imaging (DTI), and supine bicycle exercise stress echocardiography. Cardiac synchronicity was defined, at rest and at peak exercise, as DTI velocity opposing-wall delay (significant if ≥65 ms). Outcome was defined as freedom from death, heart transplantation, or LV-assist device implantation, over a median follow-up of 48 months, and a Cox proportional hazards model was used for survival analysis. At baseline examination, DCM patients showed a reduced LV ejection fraction (31 + 4%). A significant electromechanical delay in 58 patients (32%). At the peak of physical exercise, a significant electromechanical delay was detected in 103 patients (57%). There were 41 events during the follow-up (23%): 28 cardiac deaths, 8 heart transplantations, and 5 LV-assist device implantations over 4 years. When adjusted for confounding baseline variables, LV end-diastolic volume, restrictive mitral flow pattern, severity of mitral regurgitation, and the presence of exercise-induced intraventricular dyssynchrony were the only independent determinants of an adverse outcome. In patients with idiopathic DCM and narrow QRS, the increase in echocardiographic dyssynchrony during