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Sample records for idiopathic clubfoot deformity

  1. Ponseti Casting Method in Idiopathic Congenital Clubfoot and Its Correlation with Radiographic Features Abstract

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    Taghi Baghdadi

    2017-05-01

    Full Text Available Background: The aim of this study was to evaluate the idiopathic congenital clubfoot deformity treated by Ponseti method to determine the different factors such as radiological investigations that may have relations with the risk of failure and recurrence in mid-term follow-up of the patients. Methods: Since 2006 to 2011, 226 feet from 149 patients with idiopathic congenital clubfoot were treated with weekly castings by Ponseti method. Anteroposterior and lateral foot radiographies were performed at the final follow-up visit and the data from clinical and radiological outcomes were analysed. Results: In our patients, 191(84.9% feet required percutaneous tenotomy. The successful correction rate was 92% indication no need for further surgical correction. No significant correlation was found between the remained deformity rate and the severity of the deformity and compliance of using the brace (P=0.108 and 0.207 respectively. The remained deformity rate had an inverse association with the beginning age of treatment (P=0.049. No significant correlation was found between the percutaneous tetonomy and passive dorsiflexion range (P=0.356. Conclusion: According to our results treatment with the Ponseti method resulted in poor or no correlation. The diagnosis of clubfoot is a clinical judgment; therefore, the outcome of the treatment must only be clinically evaluated. Although the Ponseti method can retrieve the normal shape of the foot, it fails to treat the bone deformities and eventually leads to remained radiologic deformity. Further studiesare suggested to define a different modification that can address the abnormal angles between the foot and ankle bones to minimize the risk of recurrence.

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

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    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.

  3. Design and development of a device to measure the deformities of clubfoot.

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    Khas, Kanwaljit S; Pandey, Pulak M; Ray, Alok R

    2015-03-01

    Clubfoot describes a range of foot abnormalities usually present at birth, in which the foot of a baby is twisted out of shape or position. In order to develop an effective treatment plan for clubfoot and/or assess the extent to which existing interventions are successful, medical practitioners need to be able to accurately measure the nature and extent of the deformity. This is typically performed using a goniometer. However, this device is only able to measure one dimension at a time. As such, a complete assessment of the condition of a foot can be extremely burdensome and time-consuming. This article describes a new device that can quickly and efficiently take several measurements on feet of various sizes and shapes. The use of this device was verified by measuring the deformities of real clubfeet. A silicone rubber clubfoot model was also used in this study to clearly illustrate the effectiveness with which the proposed device can measure the various deformities of clubfoot. It is envisaged that the use of this device will significantly reduce the time and effort orthopedists require to measure clubfoot deformities and develop and assess treatment plans. © IMechE 2015.

  4. Ponseti treatment in the management of clubfoot deformity - a continuing role for paediatric orthopaedic services in secondary care centres.

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    Docker, Charles E J; Lewthwaite, Simon; Kiely, Nigel T

    2007-07-01

    The Ponseti technique is a well-proven way of managing paediatric clubfoot deformity. We describe a management set-up which spreads the care between secondary and tertiary care with no loss of quality. In our audit of the first 2 years of Ponseti casting in the treatment of idiopathic congenital talipes equinovarus (CTEV, clubfoot) deformity, we identified 77 feet having been treated in 50 patients. Forty-nine feet were treated primarily in Oswestry, a tertiary referral centre for paediatric orthopaedic conditions, and 13 feet were treated in conjunction with the physiotherapy department at one of the region's district general hospitals (Leighton Hospital, Crewe, Cheshire). Similar good results and low requirement for surgical interventions other than Achilles tenotomy, which forms part of the Ponseti regimen, were found in both cohorts. This 'hub-and-spoke' approach would appear to be efficient in terms of resource utilisation. Additional benefits for patients and their carers include ease of access to services and reduced financial and transport burdens.

  5. Ponseti Treatment in the Management of Clubfoot Deformity – A Continuing Role for Paediatric Orthopaedic Services in Secondary Care Centres

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    Docker, Charles EJ; Lewthwaite, Simon; Kiely, Nigel T

    2007-01-01

    INTRODUCTION The Ponseti technique is a well-proven way of managing paediatric clubfoot deformity. We describe a management set-up which spreads the care between secondary and tertiary care with no loss of quality. PATIENTS AND METHODS In our audit of the first 2 years of Ponseti casting in the treatment of idiopathic congenital talipes equinovarus (CTEV, clubfoot) deformity, we identified 77 feet having been treated in 50 patients. Forty-nine feet were treated primarily in Oswestry, a tertiary referral centre for paediatric orthopaedic conditions, and 13 feet were treated in conjunction with the physiotherapy department at one of the region's district general hospitals (Leighton Hospital, Crewe, Cheshire). RESULTS Similar good results and low requirement for surgical interventions other than Achilles tenotomy, which forms part of the Ponseti regimen, were found in both cohorts. CONCLUSIONS This ‘hub-and-spoke’ approach would appear to be efficient in terms of resource utilisation. Additional benefits atients and their carers include ease of access to services and reduced financial and transport burdens. PMID:17688726

  6. Positive communication paradigm decreases early recurrence in clubfoot treatment.

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    Morin, Matthew L; Hoopes, Daniel M; Szalay, Elizabeth A

    2014-03-01

    The Ponseti method has become the treatment standard for idiopathic clubfoot. Deformity recurrence is most commonly attributed to premature abandonment of the requisite abduction orthosis. A study in 2009 from our center revealed a high rate of deformity recurrence in our patient population. It was surmised that the importance of bracing to maintain correction had not been adequately communicated to some families, especially Native Americans. As a result, the principal investigator developed a different communication protocol for parents of infants. All children treated for clubfoot at the University of New Mexico Carrie Tingley Hospital, Albuquerque, NM, from 2008 to 2010 were reviewed. They were compared with a historical control group from this institution, the subjects of the 2009 study, and were analyzed for the rate of recurrence and Pirani score improvement. Our study cohort comprised 69 infants (104 clubfeet), all of whom were treated with the new communication style. The recurrence rate for the new communication paradigm was 2.88% compared with 18.2% in the control group (Pgroup compared with 3.5 in the control group (P=0.001). Native American recurrence was zero in the treatment group and 41% in the control group (P=0.011). A positive, rather than a negative communication style, emphasis on the brace as the most important aspect of treatment, and a more culturally sensitive family education paradigm, resulted in a lower rate of deformity recurrence when treating children with clubfeet using the Ponseti method. Level III.

  7. Comparative Analysis between Podography and Radiography in the Management of Idiopathic Clubfeet by Ponseti Technique.

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    Trivedi, Vikas; Badhwar, Sumit; Dube, Abhay S

    2017-02-01

    Idiopathic clubfoot is one of the most common and oldest congenital foot anomalies. There are controversies regarding its optimum management protocol and methodologies to be employed for its functional outcome evaluation. This paper attempts to propose a simple, reasonable and easily reproducible technique of podography for clinical and functional evaluation of clubfoot treated by the popular Ponseti technique. To compare the Foot Bimalleolar (FBM) angle method (podography) and radiography with respect to management of idiopathic clubfoot by Ponseti's Technique and its functional evaluation. Sixty feet of 48 patients with idiopathic clubfoot deformity were assessed in terms of FBM by podography (foot print on paper and FBM angle drawing) and radiologically; before starting treatment, after 6 weeks and at 6 monthly intervals with a maximum follow up period of 4.8 years (Range 1.2 to 4.8 years). Mean age at start of treatment was 1.5 years (2 months to 2.5 years). Functional evaluation was done by Magone's scoring system. After treatment, 92 percent patients had good correction (FBM greater than 70 degrees) which correlated well with post treatment Magone's score of greater than 80 (good to excellent) in nearly 85 percent of cases. Radiologically, talocalcaneal angles in both the views improved in only 60 percent of cases. Radiological criteria show inconsistent correlation with functional outcome for feet treated by Ponseti's Technique. Podography (FBM angle analysis) is a very simple, objective, cost effective, radiation free, easily reproducible and highly reliable clinical criterion for the assessment of deformity correction in club foot by Ponseti's Technique with an excellent correlation with functional outcome.

  8. Gross Motor Skills in Children With Idiopathic Clubfoot and the Association Between Gross Motor Skills, Foot Involvement, Gait, and Foot Motion.

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    Lööf, Elin; Andriesse, Hanneke; André, Marie; Böhm, Stephanie; Iversen, Maura D; Broström, Eva W

    2017-02-24

    Little is known regarding gross motor skills (GMS) in children with idiopathic clubfoot (IC). This study describes GMS, specifically foot involvement and asymmetries, and analyses the association between GMS, gait, and foot status in children with IC. Gross motor tasks and gait were analyzed in children with IC and typically developed (TD) children. GMS were assessed using videotapes and the Clubfoot Assessment Protocol (CAP). The Gait Deviation Index (GDI) and GDI-Kinetic were calculated from gait analyses. Children were divided into bilateral, unilateral clubfoot, or TD groups. To analyze asymmetries, feet within each group were further classified into superior or inferior foot, depending on their CAP scores. Correlations identified associations between CAP and GDI, GDI-Kinetic, passive foot motion, and Dimeglio Classification Scores at birth in the clubfeet. In total, 75 children (mean age, 5 years) were enrolled (bilateral n=22, unilateral clubfoot n=25, TD=28). Children with clubfeet demonstrated significantly lower GMS, gait, and foot motion compared with TD children. One leg standing and hopping deviated in 84% and 91%, respectively, in at least one foot in children with clubfoot. Gross motor asymmetries were evident in both children with bilateral and unilateral involvement. In children with unilateral clubfoot, contralateral feet showed few deviations in GMS compared with TD; however, differences existed in gait and foot motion. The association between GMS and gait, foot motion, and initial foot status varied between poor and moderate. Gross motor deficits and asymmetries are present in children with both bilateral and unilateral IC. Development of GMS of the contralateral foot mirrors that of TD children, but modifies to the clubfoot in gait and foot motion. The weak association with gait, foot motion, and initial clubfoot severity indicates that gross motor measurements represent a different outcome entity in clubfoot treatment. We therefore, recommend

  9. Clubfoot Does Not Impair Gross Motor Development in 5-Year-Olds.

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    Zapata, Karina A; Karol, Lori A; Jeans, Kelly A; Jo, Chan-Hee

    2018-04-01

    To evaluate the gross motor development of 5-year-olds using the Peabody Developmental Motor Scales, 2nd Edition (PDMS-2), test after initial nonoperative management of clubfoot as infants. The PDMS-2 Stationary, Locomotion, and Object Manipulation subtests were assessed on 128 children with idiopathic clubfeet at the age of 5 years. Children were categorized by their initial clubfoot severity as greater than 13, unilateral or bilateral involvement, and required surgery. Children with treated clubfeet had average gross motor scores (99 Gross Motor Quotient) compared with age-matched normative scores. Children with more severe clubfeet required surgery significantly more than children with less severe scores (P < .01). Peabody scores were not significantly different according to initial clubfoot severity, unilateral versus bilateral involvement, and surgical versus nonsurgical outcomes. Clubfoot does not significantly impair gross motor development in 5-year-olds.

  10. What is a good result after clubfoot treatment? A Delphi-based consensus on success by regional clubfoot trainers from across Africa.

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    Tracey Smythe

    Full Text Available Congenital talipes equino-varus (CTEV, also known as clubfoot, is one of the most common congenital musculoskeletal malformations. Despite this, considerable variation exists in the measurement of deformity correction and outcome evaluation. This study aims to determine the criteria for successful clubfoot correction using the Ponseti technique in low resource settings through Africa.Using the Delphi method, 18 experienced clubfoot practitioners and trainers from ten countries in Africa ranked the importance of 22 criteria to define an 'acceptable or good clubfoot correction' at the end of bracing with the Ponseti technique. A 10cm visual analogue scale was used. They repeated the rating with the results of the mean scores and standard deviation of the first test provided. The consistency among trainers was determined with the intra-class correlation coefficient (ICC. From the original 22 criteria, ten criteria with a mean score >7 and SD 9 and SD<1.5.The consensus definition of a successfully treated clubfoot includes: (1 a plantigrade foot, (2 the ability to wear a normal shoe, (3 no pain, and (4 the parent is satisfied. Participants demonstrated good consistency in rating these final criteria (ICC 0.88; 0.74,0.97.The consistency of Ponseti technique trainers from Africa in rating criteria for a successful outcome of clubfoot management was good. The consensus definition includes basic physical assessment, footwear use, pain and parent satisfaction.

  11. Results of treatment of clubfoot by Ponseti′s technique in 40 cases : Pitfalls and problems in the Indian scenario

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    Bhaskar Atul

    2006-01-01

    Full Text Available Background : Though described long back, there has been a renewed interest towards Ponseti method of conservative treatment of clubfoot recently. Methods : Forty children with idiopathic clubfeet were treated by Ponseti technique. The median age at presentation was 9 days. Twenty-six children with bilateral and 12 children with unilateral clubfeet were graded by the Pirani method at the commencement of treatment and then at the final follow-up. Feet were graded as excellent if the Pirani score was zero, fair, if the sum of mid-foot and hind-foot score was one or less and poor, if the score was more than one. Thirty four children need a heel cord tenotomy and all children received conventional ankle-foot orthosis (AFO and foot-abduction orthosis (FAO to maintain correction. Results : Twenty-eight children had excellent correction, four had a fair outcome and eight cases had relapse in their deformity. Poor splint compliance and fitting along with incomplete correction of the deformity were identified as the chief causes leading to a poor result. Conclusion : A strict protocol and parent education can improve the outcome for all cases with the Ponseti technique. Key-words: Clubfoot; Congenital talipes equinovarus; Ponseti technique.

  12. Length of the casting phase in the treatment of clubfoot

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    Giesberts, R.B.; Steen, van der Marieke; Hekman, E.E.G.; Maathuis, Patrick; Besselaar, Arnold; Verkerke, G.J.

    2017-01-01

    INTRODUCTION Clubfoot is generally treated by the Ponseti method [1]. The deformity is reduced step-wise. Each interval starts with the manual manipulation of the deformed foot according to a strict protocol, after which a plaster cast is applied to the foot in (partially) corrected position. After

  13. History of clubfoot treatment, part I: From manipulation in antiquity to splint and plaster in Renaissance before tenotomy.

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    Hernigou, Philippe; Huys, Maxime; Pariat, Jacques; Jammal, Sibylle

    2017-08-01

    Idiopathic clubfoot is one of the most common problems in paediatric orthopaedics. The treatment is controversial and continues to be one of the challenges in paediatric orthopaedics. The aim of this review is to assess the different methods of clubfoot treatment used over the years in light of the documentation present in the literature and art paintings from the antiquity to the end of the 19th century. The aim of this paper is to review all treatment methods of the clubfoot over the years that were proposed to provide patients a functional, pain-free, normal-looking foot, with good mobility, without calluses, and requiring no special shoes. Hippocrates was the first to write references about treatment methods of clubfoot. After the Middle Ages and the Renaissance where patients were treated by barber-surgeons, quacks and charlatans, bonesetters, and trussmakers, there were more detailed studies on the disease, with the help of famous names in medicine such as Venel and Scarpa.

  14. ALGORITHM OF TREATMENT OF CONGENITAL CLUBFOOT IN CHILDREN OF YOUNGER AGE GROUP

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    Irina Yurievna Klychkova

    2013-03-01

    Full Text Available Congenital clubfoot requires complete correction before the period of verticalization. Conservative methods are priority in treatment. The most effective method is Ponseti method that allows correcting all the deformations even in severe clubfoot. However, if clubfoot is accompanied with abnormal anatomy of foot or significant changes in functional ability of muscles, operative treatment is inevitable. The extent of surgical intervention depends on the severity of detected abnormalities and is determined individually. The use of ultasound, radiographic, and CT investigations, according to indications, contributes to the diagnostics. The indication for surgery is the lack of effect of conservative treatment after 10-12 cast correction in children over the age of 4 months. Diagnostic criteria for deciding in favor of surgery are: availability of fibrous coalition, the deformation of the talar or the sphenoid bone, the anomaly in the point of attachment of the tendon of the tibialis anterior muscle, lack of the repositioning of navicular bone on the talar head. Orthosis supply and rehabilitation treatment depend on the extent of surgical intervention.

  15. Good results after Ponseti treatment for neglected congenital clubfoot in Ethiopia. A prospective study of 22 children (32 feet) from 2 to 10 years of age.

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    Ayana, Birhanu; Klungsøyr, Peter J

    2014-12-01

    Neglected clubfoot deformity is a major cause of disability in low-income countries. Most children with clubfoot have little access to treatment in these countries, and they are often inadequately treated. We evaluated the effectiveness of Ponseti's technique in neglected clubfoot in children in a rural setting in Ethiopia. A prospective study was conducted from June 2007 through July 2010. 22 consecutive children aged 2-10 years (32 feet) with neglected clubfoot were treated by the Ponseti method. The deformity was assessed using the Pirani scoring system. The average follow-up time was 3 years. A plantigrade functional foot was obtained in all patients by Ponseti casting and limited surgical intervention. 2 patients (4 feet) had recurrent deformity. They required re-manipulation and re-tenotomy of the Achilles tendon and 1 other patient required tibialis anterior transfer for dynamic supination deformity of the foot. This study shows that the Ponseti method with some additional surgery can be used successfully as the primary treatment in neglected clubfoot, and that it minimizes the need for extensive corrective surgery.

  16. Surgical treatment of neglected clubfoot using external fixator

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    Rodrigo Mota Pacheco Fernandes

    Full Text Available ABSTRACT The definition of neglected clubfoot (NC includes a variable range of complex deformities of the foot that are refractory to conventional treatments or are treated inappropriately. Several etiologies may be related to this. The Ilizarov method has become established as a tool for treating these deformities. It minimizes soft-tissue damage through gradual correction of the deformity, with a high success rate in relation to achieving a plantigrade foot, with low incidence of recurrence. The indications for treatment include severe rigid deformities (Dimeglio III and IV, or adverse skin conditions. Careful clinical and radiological examination is fundamental for proper planning and installation of the external fixator. The techniques used include selection of external fixation assemblies, which can be closed when there is a connection between the leg, hindfoot and forefoot. This closed assembly may or may not be constricted, according to whether hinges are provided or whether use of the natural anatomical hinges during correction of the deformity is envisaged. An open assembly makes it possible to add flexibility to the foot through histogenesis, while allowing closed corrections of greater precision later on. Hexapod fixators are an innovation with high potential for accuracy in correcting deformities. Procedures associated with external fixation include soft-tissue release and bone procedures. These procedures enable corrections that are more anatomical, for different degrees of severity and stiffness of deformity. It can be concluded from analyzing this case series that treatment of neglected clubfoot using an external fixator has a high rate of good and excellent results, with low frequency of complications.

  17. Metaphyseal fractures mimicking abuse during treatment for clubfoot

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    Grayev, A.M.; Boal, D.K.B.; Wallach, D.M.; Segal, L.S.

    2001-01-01

    Background. Metaphyseal injuries resembling the classic metaphyseal lesion (CML) of abuse may occur as the result of serial casting during treatment of clubfoot deformity. Mentioned in the orthopedic literature in 1972, this iatrogenic fracture has not been described in the radiologic literature nor has the similarity to injuries occurring with abuse been previously recognized. Objective. To describe the mechanism and radiographic appearance of metaphyseal injury observed during serial casting of clubfoot. Note similarities to the CML of abuse. Materials and methods. Eight children ranging in age from 1 to 4 months underwent casting for clubfoot. Five orthopedic surgeons from three different institutions performed the casting. Two patients had spina bifida and one, arthrogryposis. A complete skeletal survey was performed on one child who was abused; there was no suspicion of abuse in the remaining seven. Results. All children manifest injury with periosteal new bone. One child had clear evidence of abuse with 24 rib fractures. X-rays of lower extremities in short leg casts revealed bilateral tibial metaphyseal fractures. Four other children had metaphyseal fractures resembling the CML of abuse, and three developed an area of sclerosis within the metaphysis. Conclusion. In the setting of serial casting for equinovarus deformity, metaphyseal injury even the CML of abuse may be noted. Since inflicted injuries are almost always unobserved and explanations rarely offered, the fact that the CML occurs as a result of orthopedic manipulation may offer some further insight concerning the pathogenesis of this well-described abuse injury. (orig.)

  18. Metaphyseal fractures mimicking abuse during treatment for clubfoot

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    Grayev, A.M.; Boal, D.K.B. [Milton S. Hershey Medical Center, Penn State University College of Medicine, Hershey, PA (United States); Wallach, D.M.; Segal, L.S. [Milton S. Hershey Medical Center, Penn State University College of Medicine, Hershey, PA (United States)

    2001-08-01

    Background. Metaphyseal injuries resembling the classic metaphyseal lesion (CML) of abuse may occur as the result of serial casting during treatment of clubfoot deformity. Mentioned in the orthopedic literature in 1972, this iatrogenic fracture has not been described in the radiologic literature nor has the similarity to injuries occurring with abuse been previously recognized. Objective. To describe the mechanism and radiographic appearance of metaphyseal injury observed during serial casting of clubfoot. Note similarities to the CML of abuse. Materials and methods. Eight children ranging in age from 1 to 4 months underwent casting for clubfoot. Five orthopedic surgeons from three different institutions performed the casting. Two patients had spina bifida and one, arthrogryposis. A complete skeletal survey was performed on one child who was abused; there was no suspicion of abuse in the remaining seven. Results. All children manifest injury with periosteal new bone. One child had clear evidence of abuse with 24 rib fractures. X-rays of lower extremities in short leg casts revealed bilateral tibial metaphyseal fractures. Four other children had metaphyseal fractures resembling the CML of abuse, and three developed an area of sclerosis within the metaphysis. Conclusion. In the setting of serial casting for equinovarus deformity, metaphyseal injury even the CML of abuse may be noted. Since inflicted injuries are almost always unobserved and explanations rarely offered, the fact that the CML occurs as a result of orthopedic manipulation may offer some further insight concerning the pathogenesis of this well-described abuse injury. (orig.)

  19. Plantar Pressures After Nonoperative Treatment for Clubfoot: Intermediate Follow-up at Age 5 Years.

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    Jeans, Kelly A; Erdman, Ashley L; Karol, Lori A

    2017-01-01

    Worldwide, a nonoperative approach in the treatment of idiopathic clubfoot has been taken in an attempt to reduce the incidence of surgical outcomes. Although both the Ponseti casting (Ponseti) and the French physiotherapy (PT) methods have shown gait and pedobarograph differences at age 2 years, improved gait results have been reported by age 5 years. The purpose of this study was to assess plantar pressures in feet treated with the Ponseti versus the PT methods at this intermediate stage. Clubfoot patients treated nonoperatively (Ponseti or PT) underwent pedobarograph data collection at age 5 years. The foot was subdivided into the medial/lateral hindfoot, midfoot, and forefoot regions. Variables included Peak Pressure, Maximum Force, Contact Area%, Contact Time%, Pressure Time Integral, the hindfoot-forefoot angle, and displacement of the center of pressure (COP) line. Twenty controls were used for comparison. Pedobarograph data from 164 patients (238 feet; 122 Ponseti and 116 PT) showed no significant differences between the Ponseti and the PT feet, except the PT feet had a significantly less medial movement of the COP than the Ponseti feet (P=0.0379). Compared with controls, both groups had decreased plantar pressures in the hindfoot and first metatarsal regions, whereas the midfoot and lateral forefoot experienced significant increases compared with controls. This lateralization was also reflected in the hindfoot-forefoot angle and the COP. Feet that remain nonoperative and avoid surgical intervention are considered a good clinical result. However, pedobarograph results indicate mild residual deformity in these feet despite clinically successful outcomes. Level II-therapeutic.

  20. Adolescent Idiopathic Scoliosis: From Normal Spinal Anatomy To Three-Dimensional Deformity

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    Schlösser, T.P.C.

    2014-01-01

    Adolescent idiopathic scoliosis is a three-dimensional deformity of the spine and trunk that primarily affects previously healthy children. It is a classic orthopedic disorder. Despite many years of dedicated research into the etio-pathogenesis of idiopathic scoliosis, there is not one distinct

  1. Treatment of congenital club foot with Ponseti method

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    Agrawal R

    2005-01-01

    Full Text Available Introduction: Idiopathic congenital talipes equinovarus (Club Foot is a complex deformity that is difficult to correct. The goal of treatment is to reduce or eliminate its four components so that the patient has a functional, pain free, plantigrade foot, with good mobility and without calluses, and does not need to wear modified shoes. Material and Methods: We have treated 41 patients with 60 idiopathic clubfoot deformity using Ponseti method of management. The severity of foot deformity was assessed according to the grading system of Dimeglio et al. Results: The mean number of casts that were applied to obtain correction was six (range four to nine casts. Tenotomy was done in 58 feet. Fifty eight feet had good results.Two patients developed recurrence of the deformity due to non-compliance of the use of orthrotics. Conclusion: The Ponseti method is a safe and effective treatment for congenital idiopathic clubfoot and radically decreases the need for extensive corrective surgery. Non compliance with orthotics has been widely reported to be the main factor causing failure of the technique.

  2. Optimization of physical rehabilitation in congenital clubfoot

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    M.L. Golovakha

    2013-09-01

    Full Text Available The aim of the study was to improve the results of treatment of children with typical form of congenital clubfoot by optimizing of physical rehabilitation. The study included the following objectives: to make the algorithm work with the child, to justify the basis of physical rehabilitation, to study its effects, to develop a framework of implementation and optimization of the physical rehabilitation of children with congenital clubfoot. In the course of the study were 62 children involved with the typical form of congenital clubfoot: the main group (n = 42 and control group (n = 42. Age children from 4 years to 7 years. Physical rehabilitation was a logical continuation of treatment. Optimization analysis was performed by clinical examination, radiometric data and indicators of functional methods of research. Comparative analysis of the results of the physical rehabilitation of children with congenital clubfoot in both groups showed a trend more pronounced positive changes in children the main group in all respects.

  3. Repeatability of the Oxford Foot Model in children with foot deformity.

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    McCahill, Jennifer; Stebbins, Julie; Koning, Bart; Harlaar, Jaap; Theologis, Tim

    2018-03-01

    The Oxford Foot Model (OFM) is a multi-segment, kinematic model developed to assess foot motion. It has previously been assessed for repeatability in healthy populations. To determine the OFM's reliability for detecting foot deformity, it is important to know repeatability in pathological conditions. The aim of the study was to assess the repeatability of the OFM in children with foot deformity. Intra-tester repeatability was assessed for 45 children (15 typically developing, 15 hemiplegic, 15 clubfoot). Inter-tester repeatability was assessed in the clubfoot population. The mean absolute differences between testers (clubfoot) and sessions (clubfoot and hemiplegic) were calculated for each of 15 clinically relevant, kinematic variables and compared to typically developing children. Children with clubfoot showed a mean difference between visits of 2.9° and a mean difference between raters of 3.6° Mean absolute differences were within one degree for the intra and inter-rater reliability in 12/15 variables. Hindfoot rotation, forefoot/tibia abduction and forefoot supination were the most variable between testers. Overall the clubfoot data were less variable than the typically developing population. Children with hemiplegia demonstrated slightly higher differences between sessions (mean 4.1°), with the most reliable data in the sagittal plane, and largest differences in the transverse plane. The OFM was designed to measure different types of foot deformity. The results of this study show that it provides repeatable results in children with foot deformity. To be distinguished from measurement artifact, changes in foot kinematics as a result of intervention or natural progression over time must be greater than the repeatability reported here. Copyright © 2018 Elsevier B.V. All rights reserved.

  4. The Bangladesh clubfoot project: audit of 2-year outcomes of Ponseti treatment in 400 children.

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    Perveen, Roksana; Evans, Angela M; Ford-Powell, Vikki; Dietz, Frederick R; Barker, Simon; Wade, Paul W; Khan, Shariful I

    2014-01-01

    Congenital clubfoot deformity can cause significant disability, and if left untreated, may further impoverish those in developing countries, like Bangladesh. The Ponseti method has been strategically introduced in Bangladesh by a nongovernment organization, Walk For Life (WFL). WFL has provided free treatment for over 8000 Bangladeshi children with clubfeet, sustained by local ownership, and international support. This audit assesses the 2-year results in children for whom treatment began before the age of 3 years. The 10 largest WFL clinics, of the 24 across Bangladesh, were pragmatically accessed in this audit availing 1442 subjects meeting the study criteria, from which 400 children were randomly selected and examined. A specific assessment tool was developed and validated. Results for 400 cases were returned: 269 males, 131 females. Typical clubfeet comprised 79% of cases, and 55% were bilateral. A tenotomy rate of 79%, and brace use after 2 years of 85%, were notable findings. Functionally, most children could walk independently (99.0%), run (95.5%), squat (93.3%), and manage steps unassisted (93.0%). The ability to squat was the most indicative outcome measure, correlating with: less corrective casts, good and continued brace use, nonvarus heel position, good ankle range of motion, good Bangla clubfoot scores, and the ability to walk. Relapsing deformity was suspected with heel varus (18.0% left; 21.5% right). Parental satisfaction was very high, but cost of 3000 Taka ($US 38.48) was deemed unaffordable by 59%. The outcomes in young children after 2 years of Ponseti treatment for clubfoot deformity showed that 99% were able to walk independently. The assessment tool developed for this study avails ongoing monitoring. Without the patronage of WFL, most of these children would not have had access to treatment, and be unable to walk. Level II-lesser-quality prospective study.

  5. Heritability of clubfoot

    DEFF Research Database (Denmark)

    Engell, Vilhelm; Nielsen, Jan; Damborg, Frank

    2014-01-01

    INTRODUCTION: The aetiology of congenital clubfoot is unclear. Although studies on populations, families, and twins suggest a genetic component to the aetiology, other studies have identified environmental factors. The purpose of this study was to calculate heritability in order to determine...... based on a balance of goodness-of-fit and parsimony and to estimate heritability. RESULTS: We found an overall self-reported prevalence of congenital clubfoot of 0.0027 (95 % confidence interval 0.0022-0.0034). Fifty-five complete (both twins answered the question) twin pairs were identified...... representing 12 monozygotic, 22 same-sex dizygotic, 18 opposite-sex dizygotic, and 3 with unclassified zygosity. The model with only environmental factors (CE) was best fitting based on AIC, and the model with an additive genetic factor (ACE) came in second. Due to the small statistical power, we hypothesise...

  6. Walk for life - the National Clubfoot Project of Bangladesh: the four-year outcomes of 150 congenital clubfoot cases following Ponseti method

    Directory of Open Access Journals (Sweden)

    Angela Margaret Evans

    2016-11-01

    Full Text Available Abstract Background Congenital clubfoot deformity can cause significant disability, and if left untreated, may further impoverish those in developing countries, like Bangladesh. The Ponseti method has been strategically introduced in Bangladesh by a non-government organisation, Walk For Life (WFL. WFL has provided free treatment for over 17,500 Bangladeshi children with clubfeet since 2009, sustained by local ownership, and international support. This study assesses the 4-year results in children for whom treatment began before the age of 3 years. Methods A centrally located WFL clinic at Mymensingh Medical College Hospital (MMCH, representative of the larger WFL clinics, which treats >100 cases annually, was reviewed. In 2015, 99 of the 147 eligible subjects who had begun treatment in 2011 were available for follow up. Specific assessment tools enabled evaluation of parent satisfaction, gait function, and relapse cases. Results Results for 99/147 cases were returned after four years: 72 males, 27 females. Typical clubfeet comprised 98/99 of cases, and 55/99 were bilateral. The tenotomy rate was 80 %. Brace use after 3 months was 90 %, at 12 months was 65 %, and at 4 years post treatment was 40 %. Functionally, 98/99 of children could walk and run (99 %. Relapsing deformity was found in 13 %. Relapse severity varied: eight were flexible and partial, five were rigid. Half of the children lost to follow were due to changed phone numbers. While parents were very happy with their child’s feet (97 %, a materials cost of 3000 Taka ($US40 was deemed unaffordable by 60 %. Conclusions The 4-year outcomes after Ponseti treatment for clubfoot deformity, showed that 99 % of children available for follow up, were walking independently. The relapse rate was low. Parent satisfaction was high, but those whose children required further treatment were less satisfied.

  7. Medial to posterior release procedure after failure of functional treatment in clubfoot: a prospective study.

    Science.gov (United States)

    Bocahut, Nicolas; Simon, Anne-Laure; Mazda, Keyvan; Ilharreborde, Brice; Souchet, Philippe

    2016-04-01

    Two conservative techniques for clubfoot treatment are still being debated and depend upon the institution's expertise. For >40 years, the current institution has been a pioneer in the development of the physiotherapy method; however, some severe deformities remain resistant to this method which causes pain, sprains, and difficulties wearing shoes. Therefore, a surgical approach was developed simultaneously for the treatment of these residual or recurring clubfeet. The procedure reproduces the same chronological steps by performing forefoot derotation before correcting hindfoot equinus. The aim of the current study was to assess the results of this surgical technique. All clubfeet undergoing surgery between October 1995 and February 2009 were prospectively included. Initial severity was based on Dimeglio's classification and final outcomes on the International Clubfoot Study Group (ICFSG) outcome evaluation system. Last follow-up results were assessed by physical examination and radiographs. A total of 137 patients with severe clubfeet (mean Dimeglio score 12.0) underwent surgery. At the mean follow-up of 10.8 years, mean ICFSG score was 4.3 (range 0-23), and 12 % required revision surgery. The rate of undercorrection and overcorrection was low (17 pes-plano-valgus ft and 11 ft with undercorrection). Eight feet had a fixed deformity. Severe deformities are more resistant to conservative techniques even for institutions with large experience. These deformities require further treatment, including surgery if necessary. The medial to posterior soft-tissue release is a valuable technique with stable results. Level IV.

  8. Evaluation of cast creep occurring during simulated clubfoot correction.

    Science.gov (United States)

    Cohen, Tamara L; Altiok, Haluk; Wang, Mei; McGrady, Linda M; Krzak, Joseph; Graf, Adam; Tarima, Sergey; Smith, Peter A; Harris, Gerald F

    2013-08-01

    The Ponseti method is a widely accepted and highly successful conservative treatment of pediatric clubfoot involving weekly manipulations and cast applications. Qualitative assessments have indicated the potential success of the technique with cast materials other than standard plaster of Paris. However, guidelines for clubfoot correction based on the mechanical response of these materials have yet to be investigated. The current study sought to characterize and compare the ability of three standard cast materials to maintain the Ponseti-corrected foot position by evaluating cast creep response. A dynamic cast testing device, built to model clubfoot correction, was wrapped in plaster of Paris, semi-rigid fiberglass, and rigid fiberglass. Three-dimensional motion responses to two joint stiffnesses were recorded. Rotational creep displacement and linearity of the limb-cast composite were analyzed. Minimal change in position over time was found for all materials. Among cast materials, the rotational creep displacement was significantly different (p creep displacement occurred in the plaster of Paris (2.0°), then the semi-rigid fiberglass (1.0°), and then the rigid fiberglass (0.4°). Torque magnitude did not affect creep displacement response. Analysis of normalized rotation showed quasi-linear viscoelastic behavior. This study provided a mechanical evaluation of cast material performance as used for clubfoot correction. Creep displacement dependence on cast material and insensitivity to torque were discovered. This information may provide a quantitative and mechanical basis for future innovations for clubfoot care.

  9. [Locomotor development in infants with developmental dysplasia of the hip or idiopathic clubfoot undergoing orthopedic treatment. Prospective comparative study].

    Science.gov (United States)

    Masquijo, J J; Campos, L; Torres-Gómez, A; Allende, V

    2013-10-01

    Several disorders of early childhood, such as developmental dysplasia of the hip (DDH) and clubfoot, requires orthopedic treatment that limits active mobility of the lower extremities for a period of time. The aim of our study was to evaluate the impact on locomotor development of the orthopedic treatment in infants less than one year-old. The study included a prospective cohort of consecutive patients diagnosed with developmental dysplasia of the hip (Group A, 24 patients), and clubfoot (Group B, 32 patients) treated from January 2007 to June 2009. A third group (Group C) of 50 healthy children was used as control. The variables evaluated were: months with a brace, age to sit without support, age at the start of crawling, and age at the beginning of walking. The results obtained were analyzed. Comparisons between the three groups were performed using the Kruskal-Wallis test and Mann-Whitney test. We chose a value of P<.05 as level of statistical significance. The analysis of independent samples showed that the mean age at which the patients began to sit were similar: 6.12, 6.42 and 6.19 months, respectively (P=.249). The mean age for crawling was similar, although with a slight trend toward statistical significance: 8.84, 9.38 and 9.17 months, respectively (P=.08). The age at which they started walking was different between the three groups: 12.14, 13.21 and 12.41 months, respectively (P<.001). Orthopedic treatment of DDH and clubfoot in children less than one year-old slightly slows down the course of normal locomotor development. Copyright © 2012 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.

  10. Joint hyperlaxity prevents relapses in clubfeet treated by Ponseti method-preliminary results.

    Science.gov (United States)

    Cosma, Dan Ionuţ; Corbu, Andrei; Nistor, Dan Viorel; Todor, Adrian; Valeanu, Madalina; Morcuende, Jose; Man, Sorin

    2018-05-07

    The aim of the study was to evaluate the role of joint hyperlaxity (by Beighton score) as a protective factor for clubfoot relapse. Patients with idiopathic clubfoot treated with the Ponseti method between January 2004 and December 2012, without other congenital foot deformity, and not previously treated by open surgery were included in either the Relapse group (n = 23) if it was a clubfoot relapse or the Control group (n = 19) if no relapse was noted. Joint laxity was evaluated using the Beighton score at the latest follow-up against the Normal group (n = 22, children matched by sex and age without clubfoot deformity). We found a significantly higher joint laxity in the Control group (4.58, 95% confidence interval [CI]: 2.1-7.06) as compared to the Relapse (3.17, 95% CI: 1.53-4.81, p = 0.032) and Normal (3.14, 95% CI: 1.78-4.5, p = 0.03) groups. The univariate logistic regression showed a 5.28-times increase in the risk of relapse for a Beighton score lower than 4/9 points (odds ratio = 5.28; 95% CI = 1.29-21.5; p = 0.018). Joint hyperlaxity could be a protective factor for clubfoot relapse.

  11. CAUSES OF NON-OPTIMAL CONSERVATIVE TREATMENT OF CONGENITAL CLUBFOOT IN CHILDREN

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    V. M. Kenis

    2017-01-01

    Full Text Available Introduction. Ponseti method commonly accepted as the optimal approach to management of congenital clubfoot. Continuing with alternative methods should considered as malpractice. Aim: to assess causes of non-optimal treatment of congenital clubfoot in children.Materials and methods: Assessment group included 60 patients treated earlier in other clinics with non-optimal results. Control group included 60 patients treated in our clinic by Ponseti method. We used case history analysis and parents’ interviewing.Results. Family history of clubfoot and prenatal diagnosis positively influenced on the choice of Ponseti method. Primary consultancy of orthopedist and Internet search were main factors for choosing Ponseti method after birth. In contrast, the methods lead to non-optimal results chosen after maternity home and pediatricians.Conclusion. Main cause of non-optimal results of congenital clubfoot treatment is the lack of information regarding current approaches among non-orthopedic physicians, which emphasizes necessity of adequate informational support.

  12. An MRI volumetric study for leg muscles in congenital clubfoot.

    Science.gov (United States)

    Ippolito, Ernesto; Dragoni, Massimiliano; Antonicoli, Marco; Farsetti, Pasquale; Simonetti, Giovanni; Masala, Salvatore

    2012-10-01

    To investigate both volume and length of the three muscle compartments of the normal and the affected leg in unilateral congenital clubfoot. Volumetric magnetic resonance imaging (VMRI) of the anterior, lateral and postero-medial muscular compartments of both the normal and the clubfoot leg was obtained in three groups of seven patients each, whose mean age was, respectively, 4.8 months, 11.1 months and 4.7 years. At diagnosis, all the unilateral congenital clubfeet had a Pirani score ranging from 4.5 to 5.5 points, and all of them had been treated according to a strict Ponseti protocol. All the feet had percutaneous lengthening of the Achilles tendon. A mean difference in both volume and length was found between the three muscular compartments of the leg, with the muscles of the clubfoot side being thinner and shorter than those of the normal side. The distal tendon of the tibialis anterior, peroneus longus and triceps surae (Achilles tendon) were longer than normal on the clubfoot side. Our study shows that the three muscle compartments of the clubfoot leg are thinner and shorter than normal in the patients of the three groups. The difference in the musculature volume of the postero-medial compartment between the normal and the affected side increased nine-fold from age group 2 to 3, while the difference in length increased by 20 %, thus, showing that the muscles of the postero-medial compartment tend to grow in both thickness and length much less than the muscles of the other leg compartments.

  13. Evaluation of the stress level of children with idiopathic scoliosis in relation to the method of treatment and parameters of the deformity.

    Science.gov (United States)

    Leszczewska, Justyna; Czaprowski, Dariusz; Pawłowska, Paulina; Kolwicz, Aleksandra; Kotwicki, Tomasz

    2012-01-01

    Stress level due to existing body deformity as well as to the treatment with a corrective brace is one of factors influencing the quality of life of children with idiopathic scoliosis undergoing non-surgical management. The purpose of the study was to evaluate the stress level among children suffering from idiopathic scoliosis in relation to the method of treatment and the parameters of the deformity. Seventy-three patients with idiopathic scoliosis participated in the study. Fifty-two children were treated by means of physiotherapy, while 21 patients were treated with both Cheneau corrective brace and physiotherapy. To assess the stress level related to the deformity itself and to the method of treatment with corrective brace, the two Bad Sobernheim Stress Questionnaires (BSSQs) were applied, the BSSQ Deformity and the BSSQ Brace, respectively.

  14. Evaluation of the Stress Level of Children with Idiopathic Scoliosis in relation to the Method of Treatment and Parameters of the Deformity

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    Justyna Leszczewska

    2012-01-01

    Full Text Available Stress level due to existing body deformity as well as to the treatment with a corrective brace is one of factors influencing the quality of life of children with idiopathic scoliosis undergoing non-surgical management. The purpose of the study was to evaluate the stress level among children suffering from idiopathic scoliosis in relation to the method of treatment and the parameters of the deformity. Seventy-three patients with idiopathic scoliosis participated in the study. Fifty-two children were treated by means of physiotherapy, while 21 patients were treated with both Cheneau corrective brace and physiotherapy. To assess the stress level related to the deformity itself and to the method of treatment with corrective brace, the two Bad Sobernheim Stress Questionnaires (BSSQs were applied, the BSSQ Deformity and the BSSQ Brace, respectively.

  15. Modified Ponseti method of treatment for correction of neglected clubfoot in older children and adolescents--a preliminary report.

    Science.gov (United States)

    Bashi, Ramin Haj Zargar; Baghdadi, Taghi; Shirazi, Mehdi Ramezan; Abdi, Reza; Aslani, Hossein

    2016-03-01

    Congenital talipes equinovarus may be the most common congenital orthopedic condition requiring treatment. Nonoperative treatment including different methods is generally accepted as the first step in the deformity correction. Ignacio Ponseti introduced his nonsurgical approach to the treatment of clubfoot in the early 1940s. The method is reportedly successful in treating clubfoot in patients up to 9 years of age. However, whether age at the beginning of treatment affects the rate of effective correction and relapse is unknown. We have applied the Ponseti method successfully with some modifications for 11 patients with a mean age of 11.2 years (range, 6 to 19 years) with neglected and untreated clubbed feet. The mean follow-up was 15 months (12 to 36 months). Correction was achieved with a mean of nine casts (six to 13). Clinically, 17 out of 18 feet (94.4%) were considered to achieve a good result with no need for further surgery. The application of this method of treatment is very simple and also cheap in developing countries with limited financial and social resources for health service. To the best of the authors' knowledge, such a modified method as a correction method for clubfoot in older children and adolescents has not been applied previously for neglected clubfeet in older children in the literature.

  16. Evaluation of the walking pattern in clubfoot patients who received early intensive treatment

    DEFF Research Database (Denmark)

    Alkjaer, T; Pedersen, E N; Simonsen, E B

    2000-01-01

    joint in the clubfeet could possibly be owing to weaker plantar flexors. In conclusion, gait analysis can be an important tool when evaluating treatment for clubfoot. However, further investigation is needed to determine whether the higher hip and knee joint moments observed in subjects with clubfoot...... in the two groups. However, analysis revealed a smaller ankle joint moment and larger knee and hip joint moments in those with clubfoot. It was concluded that the larger knee and hip joint moments served as compensation for the smaller ankle moment. The reduced ankle moment and work developed about the ankle...

  17. Discrepancy in clinical versus radiological parameters describing deformity due to brace treatment for moderate idiopathic scoliosis

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    Szulc Andrzej

    2007-12-01

    Full Text Available Abstract Background The shape of the torso in patients with idiopathic scoliosis is considered to reflect the shape of the vertebral column, however the direct correlation between parameters describing clinical deformity and those characterizing radiological curvature was reported to be weak. It is not clear if the management proposed for scoliosis (physiotherapy, brace, surgery affects equally the shape of the axial skeleton and the surface of the body. The aim of the study was to compare clinical deformity of (1 idiopathic scoliosis girls being under brace treatment for radiological curves of 25 to 40 degrees and (2 non treated scoliotic girls matched for age and Cobb angle. Methods Cross-sectional study of 24 girls wearing the brace versus 26 girls without brace treatment, matched for age and Cobb angle. Hypothesis: Patients wearing the brace for more than 6 months, when comparing to patients without brace, may present different external morphology of the trunk, in spite of having similar Cobb angle. Material. Inclusion criteria: girls, idiopathic scoliosis, growing age (10–16 years, Cobb angle minimum 25°, maximum 40°. The braced group consisted of girls wearing a TLSO brace (Cheneau for more than 6 months with minimum of 16 hours per day. The non-braced group consisted of girls first seen for their spinal deformity, previously not treated. The groups presented similar curve pattern. Methods. Scoliometer exam: angle of trunk rotation at three levels of the spine: upper thoracic, main thoracic, lumbar or thoracolumbar. The maximal angle was noted at each level and the sum of three levels was calculated. Posterior trunk symmetry index (POTSI and Hump Sum were measured using surface topography. Results Cobb angle was 34.9° ± 4.8° in braced and 32.7° ± 4.9° in un-braced patients (difference not significant. The age was 14.1 ± 1.6 years in braced patients and 13.1 ± 1.9 years in un-braced group (p = 0.046. The value of angle of trunk

  18. Familial isolated clubfoot is associated with recurrent chromosome 17q23.1q23.2 microduplications containing TBX4.

    Science.gov (United States)

    Alvarado, David M; Aferol, Hyuliya; McCall, Kevin; Huang, Jason B; Techy, Matthew; Buchan, Jillian; Cady, Janet; Gonzales, Patrick R; Dobbs, Matthew B; Gurnett, Christina A

    2010-07-09

    Clubfoot is a common musculoskeletal birth defect for which few causative genes have been identified. To identify the genes responsible for isolated clubfoot, we screened for genomic copy-number variants with the Affymetrix Genome-wide Human SNP Array 6.0. A recurrent chromosome 17q23.1q23.2 microduplication was identified in 3 of 66 probands with familial isolated clubfoot. The chromosome 17q23.1q23.2 microduplication segregated with autosomal-dominant clubfoot in all three families but with reduced penetrance. Mild short stature was common and one female had developmental hip dysplasia. Subtle skeletal abnormalities consisted of broad and shortened metatarsals and calcanei, small distal tibial epiphyses, and thickened ischia. Several skeletal features were opposite to those described in the reciprocal chromosome 17q23.1q23.2 microdeletion syndrome associated with developmental delay and cardiac and limb abnormalities. Of note, during our study, we also identified a microdeletion at the locus in a sibling pair with isolated clubfoot. The chromosome 17q23.1q23.2 region contains the T-box transcription factor TBX4, a likely target of the bicoid-related transcription factor PITX1 previously implicated in clubfoot etiology. Our result suggests that this chromosome 17q23.1q23.2 microduplication is a relatively common cause of familial isolated clubfoot and provides strong evidence linking clubfoot etiology to abnormal early limb development. Copyright 2010 The American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.

  19. Unilateral pedicle screws asymmetric tethering: an innovative method to create idiopathic deformity

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    Zhang Xuesong

    2007-10-01

    Full Text Available Abstract Objective To evaluate the feasibility of the method that unilateral pedicle screws asymmetric tethering in concave side in combination with convex rib resection for creating idiopathic deformity. Summary of background data Various methods are performed to create idiopathic deformity. Among these methods, posterior asmmetric tethering of the spine shows satisfying result, but some drawbacks related to the current posterior asymmetric tether were still evident. Materials and methods Unilateral pedicle screws asymmetric tethering was performed to 14 female goats (age: 5–8 week-old, weight: 6–8 kg in concave side in combination with convex rib resection. Dorsoventral and lateral plain radiographs were taken of each thoracic spine in the frontal and sagittal planes right after the surgery and later every 4 weeks. Results All animals ambulated freely after surgery. For technical reasons, 2 goats were excluded (one animal died for anesthetic during the surgery, and one animal was lost for instrumental fail due to postoperative infection. Radiography showed that 11 goats exhibited scoliosis with convex toward to the right side, and as the curve increased with time, only 1 goat showed nonprogressive. The initial scoliosis generated in the progressors after the procedures measured 29.0° on average (range 23.0°–38.5° and increased to 43.0° on average (range 36.0°–58.0° over 8 to 10 weeks. The average progression of 14.0° was measured. The curvature immediately after tethering surgery (the initial Cobb angle did have a highly significant correlation with the final curvature (p Conclusion Unilateral pedicle screws asymmetric tethering is a practical method to create experimental scoliosis, especially for those who would like to study the correction of this deformity.

  20. Management of clubfoot by ponseti method: A prospective study

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    Manoj Kumar Saini

    2017-01-01

    CONCLUSION: The observations of the present study clearly showed that age at initial presentation, quality (mobility of foot and Pirani score at presentation directly affects final results. Ponseti technique for the treatment of clubfoot is a simple and effective which is suitable in the Indian subcontinent as it is economical.

  1. Talectomy for Equinovarus Deformity in Family Members with Hereditary Motor and Sensory Neuropathy Type I

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    Hristo Georgiev

    2014-01-01

    Full Text Available The treatment of severe rigid neurogenic clubfoot deformities still remains a challenging problem in modern paediatric orthopaedics. In those cases, in spite of being a palliative procedure, talectomy has been advocated for the correction of the deformity thus providing a stable plantigrade foot which allows pain-free walking with standard footwear. Herein, we present the results after talectomy in two patients (brother and sister affected by a hereditary motor and sensory neuropathy type I, with rigid severe pes equinovarus deformities.

  2. Surgical Site Infections in Pediatric Spine Surgery: Comparative Microbiology of Patients with Idiopathic and Nonidiopathic Etiologies of Spine Deformity.

    Science.gov (United States)

    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.

  3. Optimal management of idiopathic scoliosis in adolescence

    Science.gov (United States)

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

    2013-01-01

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

  4. Patient evaluation in idiopathic scoliosis: Radiographic assessment, trunk deformity and back asymmetry.

    Science.gov (United States)

    Rigo, Manuel

    2011-01-01

    Progressive adolescent idiopathic scoliosis (AIS) produces specific signs and symptoms, including trunk and spinal deformity and imbalance, impairment of breathing function, pain, progression during adult life, and psychological problems, as a whole resulting in an alteration of the health-related quality of life. A scoliosis-specific rehabilitation program attempts to prevent, improve, or minimize these signs and symptoms by using exercises and braces as the main tools in the rehabilitation treatment. Patient evaluation is an essential point in the decision-making process and determines the selection of the specific exercises and the specifications of the brace design. However, this article is not addressed to scoliosis management. In this present article, a complete definition and discussion of radiological aspects, such as the Cobb angle, axial rotation, curve pattern classifications, and sagittal configuration, follow a short description of the three-dimensional nature of AIS. The relationship between AIS and growth is also discussed. There is also a section dedicated to the assessment of trunk deformity and back asymmetry. Other important clinical aspects, such as pain and disability, changes in other regions of the body, muscular balance, breathing function, and health-related quality of life, are not discussed in this present article.

  5. [Which foot deformities should be radiologist be familiar with?

    Science.gov (United States)

    von Stillfried, E

    2018-05-01

    Most deformities of the foot are visible at birth and can be diagnosed without imaging. They can be divided into congenital flexible, congenital structural and acquired foot deformities. The most common congenital flexible foot deformity in children is the metatarsus adductus, which usually requires no long-term therapy. Regarding congenital structural deformities, such as the clubfoot and talus verticalis, plaster therapy should be started during the first week of life, so that by the end of the first year of life and the beginning of the verticalization, a pain-free resilient foot with normal function is present. Imaging is usually only necessary if a relapse arises. Coalitio of the tarsal bones is often visible only in the course of growth through the development of a rigid flatfoot and always requires imaging to confirm the diagnosis. This article is intended to give the radiologist an overview of the most important deformities and to inform about their course and therapy.

  6. Optimal management of idiopathic scoliosis in adolescence

    Directory of Open Access Journals (Sweden)

    Kotwicki T

    2013-07-01

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

  7. Evaluation of an E-Learning Course for Clubfoot Treatment in Tanzania: A Multicenter Study.

    Science.gov (United States)

    Vaca, Silvia D; Warstadt, Nicholus M; Ngayomela, Isidor H; Nungu, Rachel; Kowero, Emmanuel S; Srivastava, Sakti

    2018-01-01

    In total, 80% of clubfoot cases occur in low- and middle-income countries, where lack of clinical knowledge of the Ponseti method of treatment presents as a major barrier to treatment. This study aims to determine the effectiveness of an electronic learning course to teach clinicians in Tanzania Ponseti method theory. A total of 30 clinicians were recruited from clinics with high referral rates for clubfoot patients and invited to 1 of 3 training sites: Mbeya (n = 15), Zanzibar (n = 10), and Mwanza (n = 5). Baseline knowledge, measured through a pretest, was compared to performance on a posttest after e-learning course completion. Scores for Mbeya and Zanzibar participants improved from 44 ± 12.5 to 69.8 ± 16.5 ( P  e-learning course may be an effective method of disseminating Ponseti method theory in Tanzania. Successful implementation requires an understanding of the device availability and technology literacy of the users.

  8. Unilateral Congenital Knee and Hip Dislocation with Bilateral Clubfoot – A rare Packaging disorder

    Directory of Open Access Journals (Sweden)

    Mukesh Tiwari

    2013-04-01

    Full Text Available ntroduction: Reduced intrauterine space gives rise to ‘packaging disorder’ which may involve joint dislocations or contractures. We present an unique case where mutiple joints were dislocated involving left congenital knee dislocation (CDK, bilateral congenital hip dislocation (CDH and congenital talipes equino varus (CTEVdeformities. Case Report: A preterm baby boy born to mother with diagnosed oligohydramios presented with left CDK bilateral DDH and CTEV. The knee dislocation was treated first with gradual streaching and weekly above knee cast. At 7th week good flexion was achieved at both knees and abduction splint for DDH (using double diaper with ponseti cast for CTEV was done. At one year follow up all joints were reduced and maintained well with baby able to stand with support. Conclusion: Packaging disorders may present with multiple dislocations and deformities. Early intervention with serial casting and manipulation minimises disability and prevents ambulatory problems. In our case there was a good response to manipulation and serial casting. This differs from cases with inherent pathology like arthrogryposis where response to treatment is not so good. Keywords: Congenital genu recurvatum, Develpmental dysplasia hip, CTEV, Clubfoot, serial manipulation, packaging disorders

  9. Human upright spinopelvic alignment and the etio-pathogenesis of idiopathic scoliosis

    NARCIS (Netherlands)

    Janssen, M.M.A.

    2011-01-01

    Idiopathic scoliosis is a classic and intriguing orthopedic disorder in which the spine, usually during the pubertal growth spurt, collapses into a three-dimensional deformity without any known cause. Despite many anatomical similarities between the human spine and other spines in nature, idiopathic

  10. Photovoice and Clubfoot: Using a Participatory Research Method to Study Caregiver Adherence to the Ponseti Method in Perú.

    Science.gov (United States)

    Pletch, Alison; Morcuende, Jose; Barriga, Hersey; Segura, Jose; Salas, Alexandro

    2015-01-01

    The Ponseti Method of casting and bracing is the gold-standard treatment for congenital clubfoot in young children. Despite its many advantages, outcomes depend heavily on caregiver adherence to the treatment protocol. Our study explored the experience caregivers had with the Ponseti method using a photography-based participatory research method known as Photovoice. Five adult caregivers were recruited from families pursuing clubfoot treatment at the Children's Hospital in Lima, Perú, during June, 2013. Each was provided a digital camera and training and agreed to photograph their experiences caring for a child undergoing Ponseti Method clubfoot treatment. Participants held four to five weekly one-on-one meetings with the researcher to discuss their photos. They also attended a group meeting at the end of the study to view and discuss photos of other participants. Using photos collected at this meeting, participants identified themes that summarized their experiences with treatment and discussed ways to improve delivery of care in order to support caregiver adherence to treatment. These results were presented to clinicians in Lima who use the Ponseti Method. The Photovoice method allowed researchers and participants to study the experience caregivers have with the Ponseti Method, and results can be used to inform the design of patient-based care models.

  11. Health related quality of life and perception of deformity in patients with adolescent idiopathic scoliosis.

    Science.gov (United States)

    Çolak, Tuğba Kuru; Akgül, Turgut; Çolak, Ilker; Dereli, Elif Elçin; Chodza, Mehmet; Dikici, Fatih

    2017-01-01

    Quality of life and cosmethic appearance have gained importance as outcomes in AIS treatment. Improving aesthetic appearance and quality of life are defined as the primary aims of scoliosis treatment by health professionals. Studies that assess and compare the different treatment results in the field of quality of life and cosmethics are some what limited. A cross-sectional study was designed to compare quality of life and deformity perception in patients with adolescent idiopathic scoliosis (AIS) received conservative (exercise or exercise + brace) or surgical treatment. A total of 68 (58 females) patients aged 10-18 years with AIS received conservative (exercise or exercise+brace) or surgical treatment were invited to participate in the study. Quality of life (Scoliosis Research Society-23 (SRS-23)) and perception of deformity (Walter Reed Visual Assessment (WRVAS)) were assessed. Conservatively treated patients had significantly superior scores in function domain of SRS-23 than surgically treated patients (exercise/surgery, exercise+brace/surgery; p= 0.009, 0.004). Otherwise, surgically treated patients had significantly superior scores in self-image (p= 0.000, 0.000), and satisfaction with management (p= 0.001, 0.006) domains of SRS-23, and WRVAS (p= 0.000, 0.000) than conservative groups. In addition to radiographic assessments, quality of life, aesthetic perception, functionality, satisfaction with management, psycho-social status should carefully be taken into consideration by health professionals in the teratment of AIS.

  12. Fully automatic measurements of axial vertebral rotation for assessment of spinal deformity in idiopathic scoliosis

    International Nuclear Information System (INIS)

    Forsberg, Daniel; Andersson, Mats; Knutsson, Hans; Lundström, Claes; Vavruch, Ludvig; Tropp, Hans

    2013-01-01

    Reliable measurements of spinal deformities in idiopathic scoliosis are vital, since they are used for assessing the degree of scoliosis, deciding upon treatment and monitoring the progression of the disease. However, commonly used two dimensional methods (e.g. the Cobb angle) do not fully capture the three dimensional deformity at hand in scoliosis, of which axial vertebral rotation (AVR) is considered to be of great importance. There are manual methods for measuring the AVR, but they are often time-consuming and related with a high intra- and inter-observer variability. In this paper, we present a fully automatic method for estimating the AVR in images from computed tomography. The proposed method is evaluated on four scoliotic patients with 17 vertebrae each and compared with manual measurements performed by three observers using the standard method by Aaro–Dahlborn. The comparison shows that the difference in measured AVR between automatic and manual measurements are on the same level as the inter-observer difference. This is further supported by a high intraclass correlation coefficient (0.971–0.979), obtained when comparing the automatic measurements with the manual measurements of each observer. Hence, the provided results and the computational performance, only requiring approximately 10 to 15 s for processing an entire volume, demonstrate the potential clinical value of the proposed method. (paper)

  13. Relation between self-image score of SRS-22 with deformity measures in female adolescent idiopathic scoliosis patients.

    Science.gov (United States)

    Wang, L; Wang, Y P; Yu, B; Zhang, J G; Shen, J X; Qiu, G X; Li, Y

    2014-11-01

    Adolescent idiopathic scoliosis (AIS) is a pathology which affects the individual's functioning in the widely understood physical, psychic, and social aspects. More attention should be paid to patients' perception of self-image when evaluating the spine deformity. The present retrospective study evaluated the associations between the deformity measures and self-image score as determined by the SRS-22 questionnaire in Chinese female AIS patients. The self-image score correlates significantly with deformity measures. The location of main curve apex and the number of curve could affect the self-image score. We retrospectively reviewed the records of 202 female patients, collected data on patient's age, body mass index, radiographic and physical measures and self-image score of SRS-22 questionnaire. According to the location of main curve apex and the number of curve, the patients were divided to different subgroups. Correlations between deformity measures and self-image score of different groups were evaluated by the Spearman correlation test. The self-image score correlated negatively with the main Cobb angle, apical vertebral translation (AVT), and razor hump height. There is no significant difference of self-image score between thoracic curve (TC) and thoracolumbar curve (TL/LC) subgroups. And the self-image scores of one-curve, two-curve and three-curve subgroups are similar. For Chinese female AIS patients in our study, self-image was found to correlate negatively with the main Cobb angle, AVT and razor hump height. And the location of scoliosis apex and the number of curve are not influencing factors of self-image perception. Level IV, retrospective study. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

  14. The effectiveness of core stabilization exercise in adolescent idiopathic scoliosis: A randomized controlled trial.

    Science.gov (United States)

    Gür, Gözde; Ayhan, Cigdem; Yakut, Yavuz

    2017-06-01

    Core stabilization training is used to improve postural balance in musculoskeletal problems. The purpose of this study was to investigate the effectiveness of stabilization training in adolescent idiopathic scoliosis. A randomized controlled trial, pretest-posttest design. In total, 25 subjects with adolescent idiopathic scoliosis were randomly divided into two groups: stabilization group ( n = 12) and control group ( n = 13). The stabilization group received core stabilization in addition to traditional rehabilitation, and the control group received traditional rehabilitation for 10 weeks. Assessment included Cobb's angle on radiograph, apical vertebral rotation in Adam's test, trunk asymmetry (Posterior Trunk Symmetry Index), cosmetic trunk deformity (Trunk Appearance Perception Scale), and quality of life (Scoliosis Research Society-22 questionnaire). Inter-group comparisons showed significantly greater improvements in the mean change in lumbar apical vertebral rotation degree and the pain domain of Scoliosis Research Society-22 in the stabilization group than those in the control group ( p adolescent idiopathic scoliosis. Clinical relevance Stabilization exercises are more effective in reducing rotation deformity and pain than traditional exercises in the conservative rehabilitation of adolescent idiopathic scoliosis. These improvements suggest that stabilization training should be added to rehabilitation programs in adolescent idiopathic scoliosis.

  15. Ethiopian Journal of Health Sciences - Vol 22, No 2 (2012)

    African Journals Online (AJOL)

    Treatment of Idiopathic Clubfoot by Ponseti Technique of Manipulation and Serial Plaster Casting and its Critical Evaluation · EMAIL FREE FULL TEXT EMAIL FREE FULL TEXT DOWNLOAD FULL TEXT DOWNLOAD FULL TEXT. Sharma Pulak, MKS Swamy ...

  16. Effects of Exercise on Spinal Deformities and Quality of Life in Patients with Adolescent Idiopathic Scoliosis

    Directory of Open Access Journals (Sweden)

    Shahnawaz Anwer

    2015-01-01

    Full Text Available Objectives. This systematic review was conducted to examine the effects of exercise on spinal deformities and quality of life in patients with adolescent idiopathic scoliosis (AIS. Data Sources. Electronic databases, including PubMed, CINAHL, Embase, Scopus, Cochrane Register of Controlled Trials, PEDro, and Web of Science, were searched for research articles published from the earliest available dates up to May 31, 2015, using the key words “exercise,” “postural correction,” “posture,” “postural curve,” “Cobb’s angle,” “quality of life,” and “spinal deformities,” combined with the Medical Subject Heading “scoliosis.” Study Selection. This systematic review was restricted to randomized and nonrandomized controlled trials on AIS published in English language. The quality of selected studies was assessed by the PEDro scale, the Cochrane Collaboration’s tool, and the Grading of Recommendations Assessment, Development, and Evaluation System (GRADE. Data Extraction. Descriptive data were collected from each study. The outcome measures of interest were Cobb angle, trunk rotation, thoracic kyphosis, lumbar kyphosis, vertebral rotation, and quality of life. Data Synthesis. A total of 30 studies were assessed for eligibility. Six of the 9 selected studies reached high methodological quality on the PEDro scale. Meta-analysis revealed moderate-quality evidence that exercise interventions reduce the Cobb angle, angle of trunk rotation, thoracic kyphosis, and lumbar lordosis and low-quality evidence that exercise interventions reduce average lateral deviation. Meta-analysis revealed moderate-quality evidence that exercise interventions improve the quality of life. Conclusions. A supervised exercise program was superior to controls in reducing spinal deformities and improving the quality of life in patients with AIS.

  17. ‘Fast cast’ and ‘needle Tenotomy’ protocols with the Ponseti method to improve clubfoot management in Bangladesh

    Directory of Open Access Journals (Sweden)

    Angela Evans

    2017-11-01

    Full Text Available Abstract Background The management of congenital talipes equino varus (clubfoot deformity has been transformed in the last 20 years as surgical correction has been replaced by the non-surgical Ponseti method. The Ponseti method, consists of corrective serial casting followed by maintenance bracing, and has been repeatedly demonstrated to give best results - regarded as the ‘gold standard’ treatment for paediatric clubfoot. Methods To develop the study protocol Level 2 evidence was used to modify the corrective casting phase of the Ponseti method in children aged up to 12 months. Using Level 4 evidence, the percutaneous Achilles tenotomy (PAT was performed using a 19-gauge needle instead of a scalpel blade, a technique found to reduce bleeding and scarring. Results A total of 123 children participated in this study; 88 male, 35 female. Both feet were affected in 67 cases, left only in 22 cases, right only in 34 cases. Typical clubfeet were found in 112/123 cases, six atypical, five syndromic. The average age at first cast was 51 days (13–240 days. The average number of casts applied was five (2–10 casts. The average number of days between the first cast and brace was 37.8 days (10–122 days, including 21 days in a post-PAT cast. Hence, average time of corrective casts was 17 days. Parents preferred the reduced casting time, and were less concerned about unseen skin wounds. PAT was performed in 103/123 cases, using the needle technique. All post tenotomy casts were in situ for three weeks. Minor complications occurred in seven cases - four cases had skin lesions, three cases disrupted casting phase. At another site, 452 PAT were performed using the needle technique. Conclusions The ‘fast cast’ protocol Ponseti casting was successfully used in infants aged less than 8 months. Extended manual manipulation of two minutes was the essential modification. Parents preferred the faster treatment phase, and ability to closer observe

  18. Return to sports after surgery to correct adolescent idiopathic scoliosis: a survey of the Spinal Deformity Study Group.

    Science.gov (United States)

    Lehman, Ronald A; Kang, Daniel G; Lenke, Lawrence G; Sucato, Daniel J; Bevevino, Adam J

    2015-05-01

    There are no guidelines for when surgeons should allow patients to return to sports and athletic activities after spinal fusion for adolescent idiopathic scoliosis (AIS). Current recommendations are based on anecdotal reports and a survey performed more than a decade ago in the era of first/second-generation posterior implants. To identify current recommendations for return to sports and athletic activities after surgery for AIS. Questionnaire-based survey. Adolescent idiopathic scoliosis after corrective surgery. Type and time to return to sports. A survey was administered to members of the Spinal Deformity Study Group. The survey consisted of surgeon demographic information, six clinical case scenarios, three different construct types (hooks, pedicle screws, hybrid), and questions regarding the influence of lowest instrumented vertebra (LIV) and postoperative physical therapy. Twenty-three surgeons completed the survey, and respondents were all experienced expert deformity surgeons. Pedicle screw instrumentation allows earlier return to noncontact and contact sports, with most patients allowed to return to running by 3 months, both noncontact and contact sports by 6 months, and collision sports by 12 months postoperatively. For all construct types, approximately 20% never allow return to collision sports, whereas all surgeons allow eventual return to contact and noncontact sports regardless of construct type. In addition to construct type, we found progressively distal LIV resulted in more surgeons never allowing return to collision sports, with 12% for selective thoracic fusion to T12/L1 versus 33% for posterior spinal fusion to L4. Most respondents also did not recommend formal postoperative physical therapy (78%). Of all surgeons surveyed, there was only one reported instrumentation failure/pullout without neurologic deficit after a patient went snowboarding 2 weeks postoperatively. Modern posterior instrumentation allows surgeons to recommend earlier return

  19. Adolescent idiopathic scoliosis: Indications and efficacy of nonoperative treatment

    Directory of Open Access Journals (Sweden)

    Federico Canavese

    2011-01-01

    Full Text Available The strategy for the treatment of idiopathic scoliosis depends essentially upon the magnitude and pattern of the deformity, and its potential for progression. Treatment options include observation, bracing and/or surgery. During the past decade, several studies have demonstrated that the natural history of adolescent idiopathic scoliosis can be positively affected by nonoperative treatment, especially bracing. Other forms of conservative treatment, such as chiropractic or osteopathic manipulation, acupuncture, exercise or other manual treatments, or diet and nutrition, have not yet been proven to be effective in controlling spinal deformity progression, and those with a natural history that is favorable at the completion of growth. Observation is appropriate treatment for small curves, curves that are at low risk of progression, and those with a natural history that is favorable at the completion of growth. Indications for brace treatment are a growing child presenting with a curve of 25°-40° or a curve less than 25° with documented progression. Curves of 20°-25° in patients with pronounced skeletal immaturity should also be treated. The purpose of this review is to provide information about conservative treatment of adolescent idiopathic scoliosis. Indications for conservative treatment, hours daily wear and complications of brace treatment as well as brace types are discussed.

  20. Ultrasonography and color Doppler in juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Laurell, Louise; Court-Payen, Michel; Nielsen, Susan

    2012-01-01

    The wrist region is one of the most complex joints of the human body. It is prone to deformity and functional impairment in juvenile idiopathic arthritis (JIA), and is difficult to examine clinically. The aim of this study was to evaluate the role of ultrasonography (US) with Doppler in diagnosis...

  1. Treatment of the idiopathic scoliosis with brace and physiotherapy.

    Science.gov (United States)

    Hundozi-Hysenaj, Hajrije; Dallku, Iliriana Boshnjaku; Murtezani, Ardiana; Rrecaj, Shkurte

    2009-01-01

    Scoliosis is a three-dimensional deformation of the spine with a lateral curvature or deviation greater than 10 degrees and associated with vertebral rotation. Many conservative treatments are available for adolescents with idiopathic scoliosis, but the evidence for their effectiveness is still questioned. The objective of this study was to define the effectiveness of braces and individual physiotherapy for the comprehensive treatment of idiopathic scoliosis in adolescents. A retrospective study of 57 children with idiopathic thoracic dextroscoliosis with the magnitude of the thoracic curve between 20 degrees-35 degrees, treated in Orthopedic and Physiatrist Clinic as well as National Ortho-prosthetic Center within University Clinical Center of Kosova in Prishtina, during the period of 2003-2006. Inclusion of kinesitherapy in the comprehensive management of idiopathic scoliosis varied in the improvement of the muscle strength (satisfied and moderate) in almost 80% of the children while the correction of the curve was small in approximately 42.1% of cases. For children with idiopathic scoliosis, who require braces, an exercise program helps chest mobility, muscle strength, proper breathing flexibility in the spine, correct posture and keeps muscles in tone so that the transition period after brace removal is easier.

  2. A new look at the etiopathogenesis ofadolescent idiopathic scoliosis

    Directory of Open Access Journals (Sweden)

    Maciej Brzęczek

    2016-06-01

    Full Text Available Adolescent idiopathic scoliosis is the most common form of spinal deformity in children. The aetiology of the condition has not been elucidated. Currently, the multifactorial theory seems to be the most probable. Certain authors propose that melatonin should be considered as a causative factor of adolescent idiopathic scoliosis. Their assumption is supported by a range of research studies conducted on animal models with removed pineal gland, which induced scoliosis. Melatonin has been proven to exert direct and indirect effects on the development of the skeletal system. The role of calmodulin or osteoprotegerin seems equally important. In patients with this condition, the levels of platelet calmodulin and calmodulin in the specimens of the paraspinal muscles on the convex side of the curve have been shown to rise. Osteoprotegerin, in turn, modifies osteoclastic and osteoblastic differentiation. These substances have a direct influence on the cellular calcium and phosphate metabolism and can be potentially responsible for spinal deformity in adolescents. The role of oestrogens is being investigated. Moreover, the role of growth factors or thrombospondins still remains obscure. Additionally, molecular tests have revealed a number of genes that can predispose to adolescent idiopathic scoliosis. It still needs to be determined which of the musculoskeletal disorders occur first in the development of scoliosis and which are secondary to the deformity. The identification of the aetiological factor and factors responsible for scoliosis progression determines the manner of treatment.

  3. Evidence for cognitive vestibular integration impairment in idiopathic scoliosis patients

    Directory of Open Access Journals (Sweden)

    Mercier Pierre

    2009-08-01

    Full Text Available Abstract Background Adolescent idiopathic scoliosis is characterized by a three-dimensional deviation of the vertebral column and its etiopathogenesis is unknown. Various factors cause idiopathic scoliosis, and among these a prominent role has been attributed to the vestibular system. While the deficits in sensorimotor transformations have been documented in idiopathic scoliosis patients, little attention has been devoted to their capacity to integrate vestibular information for cognitive processing for space perception. Seated idiopathic scoliosis patients and control subjects experienced rotations of different directions and amplitudes in the dark and produced saccades that would reproduce their perceived spatial characteristics of the rotations (vestibular condition. We also controlled for possible alteration of the oculomotor and vestibular systems by measuring the subject's accuracy in producing saccades towards memorized peripheral targets in absence of body rotation and the gain of their vestibulo-ocular reflex. Results Compared to healthy controls, the idiopathic scoliosis patients underestimated the amplitude of their rotations. Moreover, the results revealed that idiopathic scoliosis patients produced accurate saccades to memorized peripheral targets in absence of body rotation and that their vestibulo-ocular reflex gain did not differ from that of control participants. Conclusion Overall, results of the present study demonstrate that idiopathic scoliosis patients have an alteration in cognitive integration of vestibular signals. It is possible that severe spine deformity developed partly due to impaired vestibular information travelling from the cerebellum to the vestibular cortical network or alteration in the cortical mechanisms processing the vestibular signals.

  4. Upright, prone, and supine spinal morphology and alignment in adolescent idiopathic scoliosis

    NARCIS (Netherlands)

    Brink, Rob C; Colo, Dino; Schlösser, Tom P C; Vincken, Koen L; van Stralen, Marijn; Hui, Steve C N; Shi, Lin; Chu, Winnie C W; Cheng, Jack C Y; Castelein, RM

    2017-01-01

    BACKGROUND: Patients with adolescent idiopathic scoliosis (AIS) are usually investigated by serial imaging studies during the course of treatment, some imaging involves ionizing radiation, and the radiation doses are cumulative. Few studies have addressed the correlation of spinal deformity captured

  5. Comparison of CSF Distribution between Idiopathic Normal Pressure Hydrocephalus and Alzheimer Disease.

    Science.gov (United States)

    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

  6. The prevalence of predisposing deformity in osteoarthritic hip joints

    DEFF Research Database (Denmark)

    Klit, Jakob; Gosvig, Kasper; Jacobsen, Steffen

    2011-01-01

    relationship in both sexes with the clinical presentation. The study cohort which fulfilled these inclusion criteria consisted of 322 females (149 right hips and 173 left hips) and 162 males (77 right hips and 85 left hips) with osteoarthritis. We found an overall prevalence of predisposing hip deformities...... in females of 62.4% and in males of 78.9%. Minor and major deformities showed the same prevalence. Both sexes had a comparable prevalence of minor and major hip joint deformity, except for pistol grip deformity, which was more prevalent in men. We concluded that 'idiopathic osteoarthritis' is uncommon......, and that even minor predisposing deformities are associated with hip osteoarthritis....

  7. Segmental torso masses in adolescent idiopathic scoliosis.

    Science.gov (United States)

    Keenan, Bethany E; Izatt, Maree T; Askin, Geoffrey N; Labrom, Robert D; Pettet, Graeme J; Pearcy, Mark J; Adam, Clayton J

    2014-08-01

    Adolescent idiopathic scoliosis is the most common type of spinal deformity whose aetiology remains unclear. Studies suggest that gravitational forces in the standing position play an important role in scoliosis progression, therefore anthropometric data is required to develop biomechanical models of the deformity. Few studies have analysed the trunk by vertebral level and none have performed investigations of the scoliotic trunk. The aim of this study was to determine the centroid, thickness, volume and estimated mass, for sections of the scoliotic trunk. Existing low-dose CT scans were used to estimate vertebral level-by-level torso masses for 20 female adolescent idiopathic scoliosis patients. ImageJ processing software was used to analyse the CT images and enable estimation of the segmental torso mass corresponding to each vertebral level. The patients' mean age was 15.0 (SD 2.7) years with mean major Cobb angle of 52 (SD 5.9)° and mean patient weight of 58.2 (SD 11.6) kg. The magnitude of torso segment mass corresponding to each vertebral level increased by 150% from 0.6kg at T1 to 1.5kg at L5. Similarly, segmental thickness from T1-L5 increased inferiorly from a mean 18.5 (SD 2.2) mm at T1 to 32.8 (SD 3.4) mm at L5. The mean total trunk mass, as a percentage of total body mass, was 27.8 (SD 0.5) % which was close to values reported in previous literature. This study provides new anthropometric reference data on segmental (vertebral level-by-level) torso mass in adolescent idiopathic scoliosis patients, useful for biomechanical models of scoliosis progression and treatment. Copyright © 2014 Elsevier Ltd. All rights reserved.

  8. Effects of living environment on the postoperative Scoliosis Research Society-24 results in females with adolescent idiopathic scoliosis

    OpenAIRE

    Misterska, Ewa; G?owacki, Maciej; Panek, S?awomir; Igny?-O?Byrne, Anna; G?owacki, Jakub; Igny?, Iwona; Krauss, Hanna; Pi?tek, Jacek

    2012-01-01

    Summary Background There are many factors influencing postoperative health-related quality of life of adolescent idiopathic scoliosis patients, including the degree of the deformity, culture, differences in geography, rural versus urban living environments, and social factors. The objective of this study was to analyze the significance of geographic factors and their differences influencing the postoperative quality of life in females with adolescent idiopathic scoliosis residing in urban and...

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

    Science.gov (United States)

    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.

  10. Predictors of emotional functioning in youth after surgical correction of idiopathic scoliosis.

    Science.gov (United States)

    Zebracki, Kathy; Thawrani, Dinesh; Oswald, Timothy S; Anadio, Jennifer M; Sturm, Peter F

    2013-09-01

    Patients with idiopathic scoliosis, although otherwise healthy, often have significant concerns about their self-image and appearance. In a group of juveniles and adolescents, this can impact adjustment in school, functioning in peer groups, and general sense of well-being. There are limited data to help physicians reliably and precisely identify those who are at higher risk of poor emotional adjustment even after spine deformity correction. The purpose of this study was to determine the predictors of emotional maladjustment in juvenile and adolescent patients after surgical correction of idiopathic scoliosis. A total of 233 juveniles, mean age 11.26 ± 1.02 (range, 8 to 12) years and 909 adolescents, mean age 14.91 ± 1.61 (range, 13 to 21) years, who underwent surgical correction for idiopathic scoliosis and who were participating in a prospective longitudinal multicenter database, were enrolled in the study. Participants completed the Scoliosis Research Society-22 (SRS-22) questionnaire before surgery and 2 years postoperatively. Radiographs were used to measure Cobb angle and surface measurements were used to determine decompensation and trunk shift. Adolescents reported poorer mental health preoperatively (Pself-image (Pself-image (Padolescent group, mental health 2 years postoperatively was predicted by preoperative mental health (Pself-image 2 years postoperatively was predicted by preoperative mental health (Pself-image (PSelf-image and mental health are significantly improved after spine deformity correction in juveniles and adolescents with idiopathic scoliosis. However, consistent with normative development, adolescents are at higher risk for emotional maladjustment than juveniles. Surgical decision making in scoliosis correction should take the emotional status of the patient into consideration.

  11. Altered head orientation patterns in children with idiopathic scoliosis in conditions with sensory conflict

    NARCIS (Netherlands)

    Eijgelaar, P. N.; Wapstra, F. H.; Otten, E.; Veldhuizen, A. G.

    2014-01-01

    Idiopathic scoliosis (IS) is the most common spinal deformity in adolescents. Defective postural equilibrium may be a contributing factor. The information of the three sensory systems combined enables the formation of a central representation of head position and body posture. Comparison of head

  12. Clinical assessment of the efficacy of SpineCor brace in the correction of postural deformities in the course of idiopathic scoliosis.

    Science.gov (United States)

    Plewka, Barbara; Sibiński, Marcin; Synder, Marek; Witoński, Dariusz; Kołodziejczyk-Klimek, Katarzyna; Plewka, Michał

    2013-03-26

    The objective of the study was to perform a clinical, comparative assessment of the degree of postural deformities before and after the treatment of idiopathic scoliosis in patients treated with SpineCor brace compared to the control group. A group of 90 children with idiopathic scoliosis (including 74 girls) at the average age of 12.2 was subject to prospective observation. Average pre-treatment Cobb angle was 24.9° in the thoracic spine and 25.8° in the lumbar spine. The group actively treated with the SpineCor brace consisted of 45 children, while the control group consisted of the remaining 45 children with the natural course of the disease. Both groups did not differ significantly in terms of age, gender, height, body weight, Risser sign of skeletal maturity and baseline clinical and radiological parameters of scoliosis. Significant reduction of rib hump was observed upon 2-year SpineCor brace treatment (P=0.04) compared to the group treated by physiotherapy only (P=0.91). Similarly, improvement in lumbar prominence was observed in the actively treated group (P=0.009), with a trend towards worse results in the control group (P=0.07) In the group treated with the SpineCor brace, significant reduction in pectoral and hamstring muscle contractures as well as reduction in shoulder asymmetry and reduction in anterior and posterior vertical deviation were observed. Treatment using the SpineCor dynamic brace leads to a clinical improvement in posture, particularly to reduction in rib hump, lumbar prominence and muscular contractures.

  13. Influence of lumbar curvature and rotation on forward flexibility in idiopathic scoliosis

    Directory of Open Access Journals (Sweden)

    Feng-Chun Kao

    2014-04-01

    Full Text Available Background: Lumbar spine facet joints are arranged sagittally and mainly provide forward flexibility. Rotation of the lumbar vertebral body and coronal plane deformity may influence the function of lumbar forward flexibility. We hypothesize that the more advanced axial and coronal plane deformity could cause more limitation on forward flexibility in patients with idiopathic scoliosis. Methods: Between January 2011 and August 2011, 85 patients with adolescent idiopathic scoliosis were enrolled in this study. The proximal thoracic, major thoracic, thoracolumbar/lumbar (TL/L, and lumbar (L1/L5 curves were measured by Cobb's method. Lumbar apical rotation was graded using the Nash-Moe score. Lumbar forward flexibility was measured using the sit and reach (S and R test. Statistical analysis was performed using one-way analysis of variance (ANOVA, Spearman's and Pearson's correlation coefficients. Results: The mean age was 16.1 ± 2.84 years. The mean proximal thoracic, major thoracic, TL/L, and L1/L5 curves were 17.61° ± 8.92, 25.56° ± 11.61, 26.09° ± 8.6, and 15.10° ± 7.85, respectively. The mean S and R measurement was 25.56 ± 12.33 cm. The magnitude of the TL/L and L1/L5 curves was statistically positively related to vertebral rotation (rs = 0.580 and 0.649, respectively. The correlation between the S and R test and both the TL/L and L1/L5 curves was negative (rp = –0.371 and –0.595, respectively. Besides, the S and R test also demonstrated a significant negative relationship with vertebral rotation (rs = –0.768. Conclusion: In patients with idiopathic scoliosis, spinal deformity can diminish lumbar forward flexibility. Higher lumbar curvature and rotation lead to greater restriction of lumbar flexion.

  14. The Level of Self-Esteem and Sexual Functioning in Women with Idiopathic Scoliosis: A Preliminary Study

    Directory of Open Access Journals (Sweden)

    Jacek Durmała

    2015-08-01

    Full Text Available A person’s image, which is determined through physical appearance, considerably affects self-esteem developed from early childhood. Scoliosis causes multiple trunk deformations that can affect a person’s perception of the body. The aim of the study was to analyze the impact of scoliosis dimension and the degree of trunk deformation on the level of self-esteem and sexual functioning in women with idiopathic scoliosis. Thirty-six women diagnosed with idiopathic scoliosis were recruited to a prospective, double-blind, randomized controlled trial. The subjects were divided into two groups depending on the value of the Cobb angle. The level of self-esteem was determined by means of the Rosenberg Self-Esteem Scale (SES, whereas the sexual functioning was assessed via the Female Sexual Function Index (FSFI. The trunk deformations were specified with the Posterior Trunk Symmetry Index (POTSI. A statistically significant correlation was proved between the amount of points received in the Rosenberg scale evaluation and the POTSI index in Group A (R = −0.56, p = 0.04. Subjects with smaller deformations within the coronal plane had a higher level of self-confidence. The trunk asymmetries in the coronal plane may have a negative effect on women with scoliosis and their self-appraisal.

  15. The Level of Self-Esteem and Sexual Functioning in Women with Idiopathic Scoliosis: A Preliminary Study.

    Science.gov (United States)

    Durmała, Jacek; Blicharska, Irmina; Drosdzol-Cop, Agnieszka; Skrzypulec-Plinta, Violetta

    2015-08-12

    A person's image, which is determined through physical appearance, considerably affects self-esteem developed from early childhood. Scoliosis causes multiple trunk deformations that can affect a person's perception of the body. The aim of the study was to analyze the impact of scoliosis dimension and the degree of trunk deformation on the level of self-esteem and sexual functioning in women with idiopathic scoliosis. Thirty-six women diagnosed with idiopathic scoliosis were recruited to a prospective, double-blind, randomized controlled trial. The subjects were divided into two groups depending on the value of the Cobb angle. The level of self-esteem was determined by means of the Rosenberg Self-Esteem Scale (SES), whereas the sexual functioning was assessed via the Female Sexual Function Index (FSFI). The trunk deformations were specified with the Posterior Trunk Symmetry Index (POTSI). A statistically significant correlation was proved between the amount of points received in the Rosenberg scale evaluation and the POTSI index in Group A (R = -0.56, p = 0.04). Subjects with smaller deformations within the coronal plane had a higher level of self-confidence. The trunk asymmetries in the coronal plane may have a negative effect on women with scoliosis and their self-appraisal.

  16. Altered sensory-weighting mechanisms is observed in adolescents with idiopathic scoliosis

    Directory of Open Access Journals (Sweden)

    Allard Paul

    2006-10-01

    Full Text Available Abstract Background Scoliosis is the most common type of spinal deformity. In North American children, adolescent idiopathic scoliosis (AIS makes up about 90% of all cases of scoliosis. While its prevalence is about 2% to 3% in children aged between 10 to 16 years, girls are more at risk than boys for severe progression with a ratio of 3.6 to 1. The aim of the present study was to test the hypothesis that idiopathic scoliosis interferes with the mechanisms responsible for sensory-reweighting during balance control. Methods Eight scoliosis patients (seven female and one male; mean age: 16.4 years and nine healthy adolescents (average age 16.5 years participated in the experiment. Visual and ankle proprioceptive information was perturbed (eyes closed and/or tendon vibration suddenly and then returned to normal (eyes open and/or no tendon vibration. An AMTI force platform was used to compute centre of pressure root mean squared velocity and sway density curve. Results For the control condition (eyes open and no tendon vibration, adolescent idiopathic scoliosis patients had a greater centre of pressure root mean squared velocity (variability than control participants. Reintegration of ankle proprioception, when vision was either available or removed, led to an increased centre of pressure velocity variability for the adolescent idiopathic scoliosis patients whereas the control participants reduced their centre of pressure velocity variability. Moreover, in the absence of vision, adolescent idiopathic scoliosis exhibited an increased centre of pressure velocity variability when ankle proprioception was returned to normal (i.e. tendon vibration stopped. The analysis of the sway density plot suggests that adolescent idiopathic scoliosis patients, during sensory reintegration, do not scale appropriately their balance control commands. Conclusion Altogether, the present results demonstrate that idiopathic scoliosis adolescents have difficulty in

  17. Functional exercise capacity, lung function and chest wall deformity in patients with adolescent idiopathic scoliosis

    Directory of Open Access Journals (Sweden)

    Evandro Fornias Sperandio

    Full Text Available AbstractIntroduction The adolescent idiopathic scoliosis (AIS causes changes on the compliance of the chest. These changes may be associated with impaired lung function and reduced functional exercise capacity of these adolescents. We aimed to evaluate the correlation between functional exercise capacity, lung function and geometry of the chest at different stages of AIS.Materials and methods The study was carried out in a cross-sectional design which were evaluated 27 AIS patients at different stages of the disease. For chest wall evaluation, were created geometry angles/distances (A/D, which were quantified by Software Postural Assessment. The functional exercise capacity was assessed by a portable gas analyzer during the incremental shuttle walk test (ISWT. Besides that, manovacuometry and spirometry were also performed.Results Linear regressions showed that oxygen uptake (peak VO2 was correlated with distance travelled in the ISWT (R2 = 0.52, maximal respiratory pressures, cough peak flow (R2 = 0.59 and some thoracic deformity markers (D1, D2 and A6.Discussion We observed that the chest wall alterations, lung function and respiratory muscle strength are related to the functional exercise capacity and may impair the physical activity performance in AIS patients.Final considerations There is correlation between functional exercise capacity, lung function and geometry of the chest in AIS patients. Our results point to the possible impact of the AIS in the physical activities of these adolescents. Therefore, efforts to prevent the disease progression are extremely important.

  18. Temporomandibular Joint Idiopathic Condylar Resorptions – A CBCT based case-control study

    DEFF Research Database (Denmark)

    Kristensen, Kasper Dahl; Schmidt, Bjarke Hjalting; Stoustrup, Peter Bangsgaard

    Aim: To describe condylar deformation in the temporomandibular joint (TMJ) in subjects with idiopathic condylar resorptions (ICR) and compare these changes to a healthy control group. Materials and method: CBCT scans were analyzed using anatomical points on the skull to create a reference line....... Subsequently, anatomical structures were identified on the mandible to measure the condylar axial angle, the condylar neck angle as well as the condylar width, length, and height in 25 patients with ICR and 25 controls (50 joints in each group). Secondly, cross sections of the TMJs were evaluated for deformity...... %), cavitation defect (22 %), and flattering (22 %). In the control group condylar flattening was seen in 2 %. More than 70 % of the joints with deformity changes evaluated in the sagittal and the coronal plane respectively showed deformity changes along the entire condylar surface. Conclusion: The results...

  19. Tibial rotational osteotomy for idiopathic torsion. A comparison of the proximal and distal osteotomy levels.

    Science.gov (United States)

    Krengel, W F; Staheli, L T

    1992-10-01

    A retrospective analysis was done of 52 rotational tibial osteotomies (RTOs) performed on 35 patients with severe idiopathic tibial torsion. Thirty-nine osteotomies were performed at the proximal or midtibial level. Thirteen were performed at the distal tibial level with a technique previously described by one of the authors. Serious complications occurred in five (13%) of the proximal and in none of the distal RTOs. For severe and persisting idiopathic tibial torsion, the authors recommend correction by RTO at the distal level. Proximal level osteotomy is indicated only when a varus or valgus deformity required concurrent correction.

  20. Idiopathic portal hypertension

    International Nuclear Information System (INIS)

    Han, Tae Kyun; Ryu, Dae Sik; Kim, Heung Chul; Hur, Hun; Eom, Kyeung Tae; Namkung, Sook; Park, Man Soo; Hwang, Woo Chul; Lee, Kwan Seop

    1996-01-01

    To describe the radiologic findings of idiopathic portal hypertension and to find the points of differentiation between idiopathic portal hypertension and liver cirrhosis. Four portograms in five patients who for four years had suffered from pathologically confirmed idiopathic portal hypertension were retrospectively analyzed and compared with a portogram obtained from a control subject with liver cirrhosis. Portographic finding s of idiopathic portal hypertension were paucity of medium-sized portal branches, irregular and obtuse-angled division of peripheral branches, abrupt interruption and an avascular area beneath the liver margin. A portogram of idiopathic portal hypertension may be useful in differentiation this and liver cirrhosis

  1. Idiopathic anaphylaxis.

    Science.gov (United States)

    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.

  2. A meta-analysis identifies adolescent idiopathic scoliosis association with LBX1 locus in multiple ethnic groups

    DEFF Research Database (Denmark)

    Londono, Douglas; Kou, Ikuyo; Johnson, Todd A

    2014-01-01

    BACKGROUND: Adolescent idiopathic scoliosis (AIS) is a common rotational deformity of the spine that presents in children worldwide, yet its etiology is poorly understood. Recent genome-wide association studies (GWAS) have identified a few candidate risk loci. One locus near the chromosome 10q24....

  3. Ultrasonographic observation of the healing process in the gap after a Ponseti-type Achilles tenotomy for idiopathic congenital clubfoot at two-year follow-up.

    Science.gov (United States)

    Niki, Hisateru; Nakajima, Hiroshi; Hirano, Takaaki; Okada, Hirokazu; Beppu, Moroe

    2013-01-01

    Ponseti management usually requires Achilles tenotomy during the final stage of serial casting. However, we lack a good understanding of the sequential tendon healing process after tenotomy in the Ponseti bracing protocol. The purpose of this study was to clarify the ultrasonographic process of tendon healing in the gap for up to two years after Ponseti-type Achilles tenotomy in patients with clubfeet. We conducted an ultrasonographic study to clarify the sequential changes in gap healing for up to two years after tenotomy. The subjects were 23 patients with 33 clubfeet. Achilles tenotomy was performed at mean 10.4 (8-16) weeks after birth. Dynamic and static ultrasonography was performed before tenotomy and at 1, 2, 3, 4, 6, 8, and 12 weeks as well as at 4, 6, 12, 18, and 24 months after tenotomy. Continuity and gliding were noted within four weeks. The united portion continued to thicken for up to three months after tenotomy. Starting from the fourth month, the healed portion began to lose its thickness, and this process continued into the sixth month. At one year, the thickness of the tendon did not differ much from that of the tendon on the opposing foot. In cases where patients had clubfoot on both feet and underwent simultaneous tenotomies, measurement of the tendons could not be accurately compared. At two years after tenotomy, slight irregularity of the internal structure persisted when compared with the unaffected foot. In addition, clinical and X-ray findings were evaluated simultaneously, and no recurrence was confirmed. To our knowledge, our results are the first to describe the process of gap healing in the tendon after tenotomy up to and beyond two years, as recommended in the Ponseti bracing protocol. Level of evidence IV.

  4. Locomotor skills and balance strategies in adolescents idiopathic scoliosis.

    Science.gov (United States)

    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 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.

  5. [Perceived self-image in adolescent idiopathic scoliosis: an integrative review of the literature].

    Science.gov (United States)

    Carrasco, Maria Isabel Bonilla; Ruiz, Maria Carmen Solano

    2014-08-01

    To learn about the experiences of adolescents diagnosed with idiopathic scoliosis. Integrative review of the literature published within a specified time frame. For both sexes, the predominant clinical symptom of this condition appears to be the negative effect that the deformity exerts on perceived self-image. Quantitative studies used numerical scores to assess perceptions of body image but did not analyse emotional aspects. Patients treated surgically were found to have a better self-image than patients treated with a brace. Quality of life was improved by a reduction in the magnitude of the curve. Spinal deformity exerts a psychological effect on adolescent girls.

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

    Science.gov (United States)

    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.

  7. Perceived self-image in adolescent idiopathic scoliosis: an integrative review of the literature

    Directory of Open Access Journals (Sweden)

    Maria Isabel Bonilla Carrasco

    2014-08-01

    Full Text Available Objective: To learn about the experiences of adolescents diagnosed with idiopathic scoliosis. Method: Integrative review of the literature published within a specified time frame. Results: For both sexes, the predominant clinical symptom of this condition appears to be the negative effect that the deformity exerts on perceived self-image. Quantitative studies used numerical scores to assess perceptions of body image but did not analyse emotional aspects. Patients treated surgically were found to have a better self-image than patients treated with a brace. Quality of life was improved by a reduction in the magnitude of the curve. Conclusion: Spinal deformity exerts a psychological effect on adolescent girls.

  8. treatment of idiopathic clubfoot by ponseti technique of

    African Journals Online (AJOL)

    GB

    2012-07-02

    Jul 2, 2012 ... Around 80% of the cases occur in developing nations. ... health personnel can manage the cases effectively by manipulation and cast application. KEYWORDS: ..... J Bone Joint Surg Am, 1992; 74(3):448–454. 2. Ponseti IV.

  9. Idiopathic dilated cardiomyopathy: computerized anatomic study of relashionship between septal and free left ventricle wall.

    Science.gov (United States)

    Juliani, Paulo Sérgio; Costa, Eder França da; Correia, Aristides Tadeu; Monteiro, Rosangela; Jatene, Fabio Biscegli

    2014-01-01

    A feature of dilated cardiomyopathy is the deformation of ventricular cavity, which contributes to systolic dysfunction. Few studies have evaluated this deformation bearing in mind ventricular regions and segments of the ventricle, which could reveal important details of the remodeling process, supporting a better understanding of its role in functional impairment and the development of new therapeutic strategies. To evaluate if, in basal, equatorial and apical regions, increased internal transverse perimeter of left ventricle in idiopathic dilated cardiomyopathy occurs proportionally between the septal and non-septal segment. We performed an anatomical study with 28 adult hearts from human cadavers. One group consisted of 18 hearts with idiopathic dilated cardiomyopathy and another group with 10 normal hearts. After lamination and left ventricle digital image capture, in three different regions (base, equator and apex), the transversal internal perimeter of left ventricle was divided into two segments: septal and not septal. These segments were measured by proper software. It was established an index of proportionality between these segments, called septal and non-septal segment index. Then we determined whether this index was the same in both groups. Among patients with normal hearts and idiopathic dilated cardiomyopathy, the index of proportionality between the two segments (septal and non-septal) showed no significant difference in the three regions analyzed. The comparison results of the indices NSS/SS among normal and enlarged hearts were respectively: in base 1.99 versus 1.86 (P=0.46), in equator 2.22 versus 2.18 (P=0.79) and in apex 2.96 versus 3.56 (P=0.11). In the idiopathic dilated cardiomyopathy, the transversal dilatation of left ventricular internal perimeter occurs proportionally between the segments corresponding to the septum and free wall at the basal, equatorial and apical regions of this chamber.

  10. Small vertebral cross-sectional area and tall intervertebral disc in adolescent idiopathic scoliosis

    Energy Technology Data Exchange (ETDEWEB)

    Ponrartana, Skorn; Fisher, Carissa L.; Aggabao, Patricia C. [Keck School of Medicine, University of Southern California, Department of Radiology, Children' s Hospital Los Angeles, Los Angeles, CA (United States); Chavez, Thomas A. [Keck School of Medicine, University of Southern California, Department of Pediatrics, Children' s Hospital Los Angeles, Los Angeles, CA (United States); Broom, Alexander M.; Wren, Tishya A.L.; Skaggs, David L. [Keck School of Medicine, University of Southern California, Department of Orthopaedic Surgery, Children' s Hospital Los Angeles, Los Angeles, CA (United States); Gilsanz, Vicente [Keck School of Medicine, University of Southern California, Department of Radiology, Children' s Hospital Los Angeles, Los Angeles, CA (United States); Keck School of Medicine, University of Southern California, Department of Pediatrics, Children' s Hospital Los Angeles, Los Angeles, CA (United States); Keck School of Medicine, University of Southern California, Department of Orthopaedic Surgery, Children' s Hospital Los Angeles, Los Angeles, CA (United States)

    2016-09-15

    When compared to boys, girls have smaller vertebral cross-sectional area, which conveys a greater spinal flexibility, and a higher prevalence of adolescent idiopathic scoliosis. To test the hypothesis that small vertebral cross-sectional area and tall intervertebral disc height are structural characteristics of patients with adolescent idiopathic scoliosis. Using multiplanar imaging techniques, measures of vertebral cross-sectional area, vertebral height and intervertebral disc height in the lumbar spine were obtained in 35 pairs of girls and 11 pairs of boys with and without adolescent idiopathic scoliosis of the thoracic spine matched for age, height and weight. Compared to adolescents without spinal deformity, girls and boys with adolescent idiopathic scoliosis had, on average, 9.8% (6.68 ± 0.81 vs. 7.40 ± 0.99 cm{sup 2}; P = 0.0007) and 13.9% (8.22 ± 0.84 vs. 9.55 ± 1.61 cm{sup 2}; P = 0.009) smaller vertebral cross-sectional dimensions, respectively. Additionally, patients with adolescent idiopathic scoliosis had significantly greater values for intervertebral disc heights (9.06 ± 0.85 vs. 7.31 ± 0.62 mm and 9.09 ± 0.87 vs. 7.61 ± 1.00 mm for girls and boys respectively; both P ≤ 0.011). Multiple regression analysis indicated that the presence of scoliosis was negatively associated with vertebral cross-sectional area and positively with intervertebral disc height, independent of sex, age and body mass index. We provide new evidence that girls and boys with adolescent idiopathic scoliosis have significantly smaller vertebral cross-sectional area and taller intervertebral disc heights - two major structural determinants that influence trunk flexibility. With appropriate validation, these findings may have implications for the identification of children at the highest risk for developing scoliosis. (orig.)

  11. Small vertebral cross-sectional area and tall intervertebral disc in adolescent idiopathic scoliosis

    International Nuclear Information System (INIS)

    Ponrartana, Skorn; Fisher, Carissa L.; Aggabao, Patricia C.; Chavez, Thomas A.; Broom, Alexander M.; Wren, Tishya A.L.; Skaggs, David L.; Gilsanz, Vicente

    2016-01-01

    When compared to boys, girls have smaller vertebral cross-sectional area, which conveys a greater spinal flexibility, and a higher prevalence of adolescent idiopathic scoliosis. To test the hypothesis that small vertebral cross-sectional area and tall intervertebral disc height are structural characteristics of patients with adolescent idiopathic scoliosis. Using multiplanar imaging techniques, measures of vertebral cross-sectional area, vertebral height and intervertebral disc height in the lumbar spine were obtained in 35 pairs of girls and 11 pairs of boys with and without adolescent idiopathic scoliosis of the thoracic spine matched for age, height and weight. Compared to adolescents without spinal deformity, girls and boys with adolescent idiopathic scoliosis had, on average, 9.8% (6.68 ± 0.81 vs. 7.40 ± 0.99 cm 2 ; P = 0.0007) and 13.9% (8.22 ± 0.84 vs. 9.55 ± 1.61 cm 2 ; P = 0.009) smaller vertebral cross-sectional dimensions, respectively. Additionally, patients with adolescent idiopathic scoliosis had significantly greater values for intervertebral disc heights (9.06 ± 0.85 vs. 7.31 ± 0.62 mm and 9.09 ± 0.87 vs. 7.61 ± 1.00 mm for girls and boys respectively; both P ≤ 0.011). Multiple regression analysis indicated that the presence of scoliosis was negatively associated with vertebral cross-sectional area and positively with intervertebral disc height, independent of sex, age and body mass index. We provide new evidence that girls and boys with adolescent idiopathic scoliosis have significantly smaller vertebral cross-sectional area and taller intervertebral disc heights - two major structural determinants that influence trunk flexibility. With appropriate validation, these findings may have implications for the identification of children at the highest risk for developing scoliosis. (orig.)

  12. Three-dimensional vertebral wedging in mild and moderate adolescent idiopathic scoliosis.

    Directory of Open Access Journals (Sweden)

    Sophie-Anne Scherrer

    Full Text Available Vertebral wedging is associated with spinal deformity progression in adolescent idiopathic scoliosis. Reporting frontal and sagittal wedging separately could be misleading since these are projected values of a single three-dimensional deformation of the vertebral body. The objectives of this study were to determine if three-dimensional vertebral body wedging is present in mild scoliosis and if there are a preferential vertebral level, position and plane of deformation with increasing scoliotic severity.Twenty-seven adolescent idiopathic scoliotic girls with mild to moderate Cobb angles (10° to 50° participated in this study. All subjects had at least one set of bi-planar radiographs taken with the EOS® X-ray imaging system prior to any treatment. Subjects were divided into two groups, separating the mild (under 20° from the moderate (20° and over spinal scoliotic deformities. Wedging was calculated in three different geometric planes with respect to the smallest edge of the vertebral body.Factorial analyses of variance revealed a main effect for the scoliosis severity but no main effect of vertebral Levels (apex and each of the three vertebrae above and below it (F = 1.78, p = 0.101. Main effects of vertebral Positions (apex and above or below it (F = 4.20, p = 0.015 and wedging Planes (F = 34.36, p<0.001 were also noted. Post-hoc analysis demonstrated a greater wedging in the inferior group of vertebrae (3.6° than the superior group (2.9°, p = 0.019 and a significantly greater wedging (p≤0.03 along the sagittal plane (4.3°.Vertebral wedging was present in mild scoliosis and increased as the scoliosis progressed. The greater wedging of the inferior group of vertebrae could be important in estimating the most distal vertebral segment to be restrained by bracing or to be fused in surgery. Largest vertebral body wedging values obtained in the sagittal plane support the claim that scoliosis could be initiated

  13. Influence of implant rod curvature on sagittal correction of scoliosis deformity

    DEFF Research Database (Denmark)

    Salmingo, Remel A.; Tadano, Shigeru; Abe, Yuichiro

    2014-01-01

    of the implant rod’s angle of curvature during surgery and establish its influence on sagittal correction of scoliosis deformity. STUDY DESIGN: A retrospective analysis of the preoperative and postoperative implant rod geometry and angle of curvature was conducted. PATIENT SAMPLE: Twenty adolescent idiopathic......BACKGROUND CONTEXT: Deformation of in vivo–implanted rods could alter the scoliosis sagittal correction. To our knowledge, no previous authors have investigated the influence of implanted-rod deformation on the sagittal deformity correction during scoliosis surgery. PURPOSE: To analyze the changes...... scoliosis patients underwent surgery. Average age at the time of operation was 14 years. OUTCOME MEASURES: The preoperative and postoperative implant rod angle of curvature expressed in degrees was obtained for each patient. METHODS: Two implant rods were attached to the concave and convex side...

  14. Adolescent Idiopathic Scoliosis

    Directory of Open Access Journals (Sweden)

    Safak Ekinci

    2014-06-01

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

  15. Idiopathic chondrolysis - diagnostic difficulties

    International Nuclear Information System (INIS)

    Kozlowski, K.; Scougall, J.; Royal Alexandra Hospital for Children, Sydney

    1984-01-01

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

  16. Are we simplifying balance evaluation in adolescent idiopathic scoliosis?

    Science.gov (United States)

    Pasha, Saba; Baldwin, Keith

    2018-01-01

    Clinical evaluation of the postural balance in adolescent idiopathic scoliosis has been measured by sagittal vertical axis and frontal balance. The impact of the scoliotic deformity in three planes on balance has not been fully investigated. 47 right thoracic and left lumbar curves adolescent idiopathic scoliosis and 10 non-scoliotic controls were registered prospectively. 13 spinopelvic postural parameters were calculated from the 3-dimantional reconstructions of X-rays. 7 balance variables describing the position and sway of the center of pressure were recorded using a pressure mat. A regression analysis was used to predict sagittal vertical axis and frontal balance from the 7 balance variables. A canonical correlation analysis was performed between all the postural parameters and balance variables and the significant associations between the postural and balance variables were determined. sagittal vertical axis and frontal balance were not significantly associated with the position or sway of the center of pressure (p>0.05). Canonical correlation analysis showed significant associations between the postural variables in the 3 planes and center of pressure position (R 2 =0.81) and sway (R 2 =0.62), pbalance contributed to the postural balance in the cohort. The compensatory role of the pelvis and distal kyphosis in sagittal plane was underlined. Multidimensional analyses between the postural and balance variables showed the alignment of the thoracic, lumbar, and pelvis in the 3 planes, in addition to the global head-pelvic position impact on adolescent idiopathic scoliosis balance. Copyright © 2017 Elsevier Ltd. All rights reserved.

  17. Idiopathic Retroperitoneal Hematoma

    African Journals Online (AJOL)

    6. Stewart BT, McLaughlin SJ, Thompson GA. Spontaneous retroperitoneal haemorrhage:a general surgeon's perspective. Aust N. Z J Surg 1998;68:371-3. Monib, et al.: Idiopathic retroperitoneal hematoma. How to cite this article: Monib S, Ritchie A, Thabet E. Idiopathic retroperitoneal hematoma. J Surg Tech Case Report ...

  18. Management of Spinal Deformities and Evidence of Treatment Effectiveness

    Science.gov (United States)

    Bettany-Saltikov, Josette; Turnbull, Deborah; Ng, Shu Yan; Webb, Richard

    2017-01-01

    Introduction: The review evaluates the up-to-date evidence for the treatment of spinal deformities, including scoliosis and hyperkyphosis in adolescents and adults. Material and Methods: The PubMed database was searched for review articles, prospective controlled trials and randomized controlled trials related to the treatment of spinal deformities. Articles on syndromic scoliosis were excluded and so were the articles on hyperkyphosis of the spine with causes other than Scheuermann’s disease and osteoporosis. Articles on conservative and surgical treatments of idiopathic scoliosis, adult scoliosis and hyperkyphosis were also included. For retrospective papers, only studies with a follow up period exceeding 10 years were included. Results: The review showed that early-onset idiopathic scoliosis has a worse outcome than late-onset idiopathic scoliosis, which is rather benign. Patients with AIS function well as adults; they have no more health problems when compared to patients without scoliosis, other than a slight increase in back pain and aesthetic concern. Conservative treatment of adolescent idiopathic scoliosis (AIS) using physiotherapeutic scoliosis-specific exercises (PSSE), specifically PSSR and rigid bracing was supported by level I evidence. Yet to date, there is no high quality evidence (RCT`s) demonstrating that surgical treatment is superior to conservative treatment for the management of AIS. For adult scoliosis, there are only a few studies on the effectiveness of PSSEs and a conclusion cannot as yet be drawn. For hyperkyphosis, there is no high-quality evidence for physiotherapy, bracing or surgery for the treatment of adolescents and adults. However, bracing has been found to reduce thoracic hyperkyphosis, ranging from 55 to 80° in adolescents. In patients over the age of 60, bracing improves the balance score, and reduces spinal deformity and pain. Surgery is indicated in adolescents and adults in the presence of progression of kyphosis

  19. Genetics Home Reference: adolescent idiopathic scoliosis

    Science.gov (United States)

    ... Facebook Twitter Home Health Conditions Adolescent idiopathic scoliosis Adolescent idiopathic scoliosis Printable PDF Open All Close All ... Javascript to view the expand/collapse boxes. Description Adolescent idiopathic scoliosis is an abnormal curvature of the ...

  20. Prenatal diagnosis of congenital hallux varus deformity associated with pericentric inversion of chromosome 9.

    Science.gov (United States)

    Gürel, Sebahat Atar

    2015-04-01

    Congenital hallux varus is a rare deformity of the great toe characterized by adduction of the hallux and medial displacement of the first metatarsophalangeal joint. Prenatal diagnosis of congenital hallux varus is presented herein. A 32-year-old woman was referred to our unit due to significant deviation of the fetal right great toe at 22(+2) weeks of pregnancy. Ultrasound examination revealed a thick and short great toe, which was significantly angulated medially on the right side. Amniocentesis was performed and the result was reported as inv(9) (p11;q12). After delivery, the clinical examination confirmed the prenatal diagnosis. To our knowledge, this is the first reported prenatal diagnosis of an isolated congenital hallux varus. Congenital hallux varus can be diagnosed easily in the prenatal period by 2-D and 4-D ultrasonography. Prenatal karyotyping should be taken into consideration, especially in the presence of associated anomalies, such as polydactyly and clubfoot. © 2014 The Author. Journal of Obstetrics and Gynaecology Research © 2014 Japan Society of Obstetrics and Gynecology.

  1. Identification of apical vertebra for grading of idiopathic scoliosis using image processing.

    Science.gov (United States)

    Anitha, H; Prabhu, G K

    2012-02-01

    Scoliosis is a 3-D deformity of spinal column, characterized by both lateral curvature and vertebral rotation. The disease can be caused by congenital, developmental, or degenerative problems; but most cases of scoliosis actually have no known cause, and this is known as idiopathic scoliosis. Vertebral rotation has become increasingly prominent in the study of scoliosis and the most deformed vertebra is named as apical vertebra. Apical vertebral deformity demonstrates significance in both preoperative and postoperative assessment, providing better appreciation of the impact of bracing or surgical interventions. Precise measurement of apical vertebral rotation in terms of grading is most valuable for the determination of reference value in normal and pathological conditions for better understanding of scoliosis. Routine quantitative evaluation of vertebral rotation is difficult and error prone due to limitations of observer characteristic and specific imaging property. This paper proposes automatic identification of the apical vertebra and its parameter that depends on the objective criteria of measurement using active contour models. The proposed technique is more accurate and is a reliable measurement compared to manual and computer-assisted system.

  2. Idiopathic Chondrolysis of the Hip ( ICH: Report of three Cases

    Directory of Open Access Journals (Sweden)

    C Dechosilpa

    2014-11-01

    Full Text Available Idiopathic Chondrolysis of the Hip (ICH is a rare condition, occurring mostly in black female adolescence. It is characterized by the rapidly progressive destruction of articular cartilage in the hip joint resulting in premature degeneration and subsequent joint arthrosis. We report three cases of ICH: a 13-year old boy presented with left knee pain, an 11-year old girl with right hip pain and a 12-year old girl with right thigh pain. All of them had the same characteristic radiographic findings. The initial treatment was started conservatively. Surgical treatment was performed in one patient in order to confirm diagnosis and correct deformity.

  3. Current concepts and controversies on adolescent idiopathic scoliosis: Part I

    Directory of Open Access Journals (Sweden)

    Alok Sud

    2013-01-01

    Full Text Available Adolescent idiopathic scoliosis is the most common spinal deformity encountered by General Orthopaedic Surgeons. Etiology remains unclear and current research focuses on genetic factors that may influence scoliosis development and risk of progression. Delayed diagnosis can result in severe deformities which affect the coronal and sagittal planes, as well as the rib cage, waistline symmetry, and shoulder balance. Patient′s dissatisfaction in terms of physical appearance and mechanical back pain, as well as the risk for curve deterioration are usually the reasons for treatment. Conservative management involves mainly bracing with the aim to stop or slow down scoliosis progression during growth and if possible prevent the need for surgical treatment. This is mainly indicated in young compliant patients with a large amount of remaining growth and progressive curvatures. Scoliosis correction is indicated for severe or progressive curves which produce significant cosmetic deformity, muscular pain, and patient discontent. Posterior spinal arthrodesis with Harrington instrumentation and bone grafting was the first attempt to correct the coronal deformity and replace in situ fusion. This was associated with high pseudarthrosis rates, need for postoperative immobilization, and flattening of sagittal spinal contour. Segmental correction techniques were introduced along with the Luque rods, Harri-Luque, and Wisconsin systems. Correction in both coronal and sagittal planes was not satisfactory and high rates of nonunion persisted until Cotrel and Dubousset introduced the concept of global spinal derotation. Development of pedicle screws provided a powerful tool to correct three-dimensional vertebral deformity and opened a new era in the treatment of scoliosis.

  4. Current concepts and controversies on adolescent idiopathic scoliosis: Part I.

    Science.gov (United States)

    Sud, Alok; Tsirikos, Athanasios I

    2013-03-01

    Adolescent idiopathic scoliosis is the most common spinal deformity encountered by General Orthopaedic Surgeons. Etiology remains unclear and current research focuses on genetic factors that may influence scoliosis development and risk of progression. Delayed diagnosis can result in severe deformities which affect the coronal and sagittal planes, as well as the rib cage, waistline symmetry, and shoulder balance. Patient's dissatisfaction in terms of physical appearance and mechanical back pain, as well as the risk for curve deterioration are usually the reasons for treatment. Conservative management involves mainly bracing with the aim to stop or slow down scoliosis progression during growth and if possible prevent the need for surgical treatment. This is mainly indicated in young compliant patients with a large amount of remaining growth and progressive curvatures. Scoliosis correction is indicated for severe or progressive curves which produce significant cosmetic deformity, muscular pain, and patient discontent. Posterior spinal arthrodesis with Harrington instrumentation and bone grafting was the first attempt to correct the coronal deformity and replace in situ fusion. This was associated with high pseudarthrosis rates, need for postoperative immobilization, and flattening of sagittal spinal contour. Segmental correction techniques were introduced along with the Luque rods, Harri-Luque, and Wisconsin systems. Correction in both coronal and sagittal planes was not satisfactory and high rates of nonunion persisted until Cotrel and Dubousset introduced the concept of global spinal derotation. Development of pedicle screws provided a powerful tool to correct three-dimensional vertebral deformity and opened a new era in the treatment of scoliosis.

  5. Standardization of dynamic RX for preoperative planning in adolescent idiopathic scoliosis

    Directory of Open Access Journals (Sweden)

    Everton Quadros Fiebig

    2014-09-01

    Full Text Available OBJECTIVE: To compare X-rays usually performed in supine with lateralization with those in lateral decubitus with fulcrum at the apex of the primary curve caused by cushion, in order to monitor the achievement of improvement patterns of correction in preestablished deformities for the preoperative surgical planning. METHODS: Comparison of radiographic studies in the preoperative supine with lateralization and lateral decubitus with cushion performing fulcrum at the apex of the major curve in patients with adolescent idiopathic scoliosis. RESULTS: Curves varied in AP between 76° e 40° and were corrected in supine with lateralization to the average of 21° observing that when carried out with fulcrum with cushion in lateral decubitus the curves were corrected to 15° on average with higher discrepancy in values among the most rigid curves. CONCLUSIONS: It was verified that on flexible curves the cushions did not produce satisfactory corrections in primary curves. In more rigid curves and in collaborative patients, greater effectiveness on the correction of deformity in main curves was obtained with cushions producing local fulcrum for a better preoperative planning on correction of deformities.

  6. Foot related impairments and disability in juvenile idiopathic arthritis persist despite modern day treatment paradigms

    OpenAIRE

    Hendry, Gordon J; Gardner-Medwin, Janet; Watt, Gordon F; Woodburn, Jim; McColl, John H; Sturrock, Roger D

    2011-01-01

    Background: Foot problems such as synovitis, growth disturbance and deformity are considered common in juvenile idiopathic arthritis (JIA) and have been previously reported in over 90% of cases. The medical management of JIA appears to have improved recently however little is known about the impact of new regimes on localised joints such as in the foot. This pilot study aimed to investigate the prevalence of foot related impairments and disability, and survey the medical and podiatric managem...

  7. Functional Investigation of a Non-coding Variant Associated with Adolescent Idiopathic Scoliosis in Zebrafish: Elevated Expression of the Ladybird Homeobox Gene Causes Body Axis Deformation.

    Directory of Open Access Journals (Sweden)

    Long Guo

    2016-01-01

    Full Text Available Previously, we identified an adolescent idiopathic scoliosis susceptibility locus near human ladybird homeobox 1 (LBX1 and FLJ41350 by a genome-wide association study. Here, we characterized the associated non-coding variant and investigated the function of these genes. A chromosome conformation capture assay revealed that the genome region with the most significantly associated single nucleotide polymorphism (rs11190870 physically interacted with the promoter region of LBX1-FLJ41350. The promoter in the direction of LBX1, combined with a 590-bp region including rs11190870, had higher transcriptional activity with the risk allele than that with the non-risk allele in HEK 293T cells. The ubiquitous overexpression of human LBX1 or either of the zebrafish lbx genes (lbx1a, lbx1b, and lbx2, but not FLJ41350, in zebrafish embryos caused body curvature followed by death prior to vertebral column formation. Such body axis deformation was not observed in transcription activator-like effector nucleases mediated knockout zebrafish of lbx1b or lbx2. Mosaic expression of lbx1b driven by the GATA2 minimal promoter and the lbx1b enhancer in zebrafish significantly alleviated the embryonic lethal phenotype to allow observation of the later onset of the spinal curvature with or without vertebral malformation. Deformation of the embryonic body axis by lbx1b overexpression was associated with defects in convergent extension, which is a component of the main axis-elongation machinery in gastrulating embryos. In embryos overexpressing lbx1b, wnt5b, a ligand of the non-canonical Wnt/planar cell polarity (PCP pathway, was significantly downregulated. Injection of mRNA for wnt5b or RhoA, a key downstream effector of Wnt/PCP signaling, rescued the defective convergent extension phenotype and attenuated the lbx1b-induced curvature of the body axis. Thus, our study presents a novel pathological feature of LBX1 and its zebrafish homologs in body axis deformation at

  8. Juvenile Idiopathic Arthritis

    Directory of Open Access Journals (Sweden)

    Kenan Barut

    2017-04-01

    Full Text Available Juvenile idiopathic arthritis is the most common chronic rheumatic disease of unknown aetiology in childhood and predominantly presents with peripheral arthritis. The disease is divided into several subgroups, according to demographic characteristics, clinical features, treatment modalities and disease prognosis. Systemic juvenile idiopathic arthritis, which is one of the most frequent disease subtypes, is characterized by recurrent fever and rash. Oligoarticular juvenile idiopathic arthritis, common among young female patients, is usually accompanied by anti-nuclear antibodie positivity and anterior uveitis. Seropositive polyarticular juvenile idiopathic arthritis, an analogue of adult rheumatoid arthritis, is seen in less than 10% of paediatric patients. Seronegative polyarticular juvenile idiopathic arthritis, an entity more specific for childhood, appears with widespread large- and small-joint involvement. Enthesitis-related arthritis is a separate disease subtype, characterized by enthesitis and asymmetric lower-extremity arthritis. This disease subtype represents the childhood form of adult spondyloarthropathies, with human leukocyte antigen-B27 positivity and uveitis but commonly without axial skeleton involvement. Juvenile psoriatic arthritis is characterized by a psoriatic rash, accompanied by arthritis, nail pitting and dactylitis. Disease complications can vary from growth retardation and osteoporosis secondary to treatment and disease activity, to life-threatening macrophage activation syndrome with multi-organ insufficiency. With the advent of new therapeutics over the past 15 years, there has been a marked improvement in juvenile idiopathic arthritis treatment and long-term outcome, without any sequelae. The treatment of juvenile idiopathic arthritis patients involves teamwork, including an experienced paediatric rheumatologist, an ophthalmologist, an orthopaedist, a paediatric psychiatrist and a physiotherapist. The primary goals

  9. Knowledge about idiopathic scoliosis among students of physiotherapy.

    Science.gov (United States)

    Ciazynski, D; Czernicki, K; Durmala, J

    2008-01-01

    The aim of the study was to determine the level of basic knowledge about idiopathic scoliosis (IS) among students of physiotherapy. The study included 37 students of Medical University of Silesia (17F and 20M aged 22-25, mean 22.6), attending the 3(rd) year of a 1(st) degree of physiotherapy. All students had credits in kinesiotherapy, including methods of conservative treatment of IS. Students were examined using a questionnaire, comprising general knowledge of IS, questions related to sagittal plane correction, influence of various physical activities on IS and known methods of conservative treatment. 81 students considered IS as 3-D deformity. 62.2% of those questioned would diagnose IS when the Cobb angle reaches 10 degrees . All students agreed that the aetiology of IS remains unknown. 54.1% considered forcible extensory exercises of back as favourable in IS. Questioned students mostly preferred swimming (94.6%), yoga (73.0%) and martial arts (32.4%) as beneficial to IS. The methods of conservative treatment which were known best were: Lehnert-Schroth-Weiss (94.6%), Klapp (91.9%), Majoch (89.2%) and Dobosiewicz (78.4%). The conclusions indicate that the average level of knowledge of idiopathic scoliosis among students of physiotherapy is unsatisfactory, despite the education programme including the SOSORT guidelines. Education in the field of scoliosis should be comprehensive and meet contemporary guidelines and standards.

  10. Familial or Sporadic Idiopathic Scoliosis – classification based on artificial neural network and GAPDH and ACTB transcription profile

    Science.gov (United States)

    2013-01-01

    Background Importance of hereditary factors in the etiology of Idiopathic Scoliosis is widely accepted. In clinical practice some of the IS patients present with positive familial history of the deformity and some do not. Traditionally about 90% of patients have been considered as sporadic cases without familial recurrence. However the exact proportion of Familial and Sporadic Idiopathic Scoliosis is still unknown. Housekeeping genes encode proteins that are usually essential for the maintenance of basic cellular functions. ACTB and GAPDH are two housekeeping genes encoding respectively a cytoskeletal protein β-actin, and glyceraldehyde-3-phosphate dehydrogenase, an enzyme of glycolysis. Although their expression levels can fluctuate between different tissues and persons, human housekeeping genes seem to exhibit a preserved tissue-wide expression ranking order. It was hypothesized that expression ranking order of two representative housekeeping genes ACTB and GAPDH might be disturbed in the tissues of patients with Familial Idiopathic Scoliosis (with positive family history of idiopathic scoliosis) opposed to the patients with no family members affected (Sporadic Idiopathic Scoliosis). An artificial neural network (ANN) was developed that could serve to differentiate between familial and sporadic cases of idiopathic scoliosis based on the expression levels of ACTB and GAPDH in different tissues of scoliotic patients. The aim of the study was to investigate whether the expression levels of ACTB and GAPDH in different tissues of idiopathic scoliosis patients could be used as a source of data for specially developed artificial neural network in order to predict the positive family history of index patient. Results The comparison of developed models showed, that the most satisfactory classification accuracy was achieved for ANN model with 18 nodes in the first hidden layer and 16 nodes in the second hidden layer. The classification accuracy for positive Idiopathic

  11. Atypical idiopathic inflammatory demyelinating lesions

    DEFF Research Database (Denmark)

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

    2013-01-01

    Atypical lesions of a presumably idiopathic inflammatory demyelinating origin present quite variably and may pose diagnostic problems. The subsequent clinical course is also uncertain. We, therefore, wanted to clarify if atypical idiopathic inflammatory demyelinating lesions (AIIDLs) can be class......Atypical lesions of a presumably idiopathic inflammatory demyelinating origin present quite variably and may pose diagnostic problems. The subsequent clinical course is also uncertain. We, therefore, wanted to clarify if atypical idiopathic inflammatory demyelinating lesions (AIIDLs) can...... be classified according to previously suggested radiologic characteristics and how this classification relates to prognosis. Searching the databases of eight tertiary referral centres we identified 90 adult patients (61 women, 29 men; mean age 34 years) with ≥1 AIIDL. We collected their demographic, clinical...

  12. Evaluation and management of adolescent idiopathic scoliosis: a review.

    Science.gov (United States)

    Jada, Ajit; Mackel, Charles E; Hwang, Steven W; Samdani, Amer F; Stephen, James H; Bennett, James T; Baaj, Ali A

    2017-10-01

    Adolescent idiopathic scoliosis (AIS) is a 3D spinal deformity affecting children between the ages of 11 and 18, without an identifiable etiology. The authors here reviewed the available literature to provide spine surgeons with a summary and update on current management options. Smaller thoracic and thoracolumbar curves can be managed conservatively with observation or bracing, but corrective surgery may be indicated for rapidly growing or larger curves. The authors summarize the atypical features to look for in patients who may warrant further investigation with MRI during diagnosis and review the fundamental principles of the surgical management of AIS. Patients with AIS can be managed very well with a combination of conservative and surgical options. Outcomes for these children are excellent with sustained longer-term results.

  13. Natural evolution from idiopathic photosensitive occipital lobe epilepsy to idiopathic generalized epilepsy in an untreated young patient.

    Science.gov (United States)

    Bonini, Francesca; Egeo, Gabriella; Fattouch, Jinan; Fanella, Martina; Morano, Alessandra; Giallonardo, Anna Teresa; di Bonaventura, Carlo

    2014-04-01

    Idiopathic photosensitive occipital lobe epilepsy (IPOE) is an idiopathic localization-related epilepsy characterized by age-related onset, specific mode of precipitation, occipital photic-induced seizures--frequently consisting of visual symptoms--and good prognosis. This uncommon epilepsy, which usually starts in childhood or adolescence, has rarely been observed in families in which idiopathic generalized epilepsy also affects other members. We describe a nuclear family in which the proband showed electro-clinical features of idiopathic photosensitive occipital lobe epilepsy in childhood, which subsequently evolved into absences and a single generalized tonico-clonic seizure in early adolescence. His mother had features suggestive of juvenile myoclonic epilepsy. This case illustrates a continuum between focal and generalized entities in the spectrum of the so-called idiopathic (genetically determined) epileptic syndromes. Copyright © 2013 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  14. Idiopathic Intracranial Hypertension (Pseudotumor Cerebri)

    Science.gov (United States)

    ... cause is determined and is referred to as “secondary” intracranial hypertension. What are the risk factors for idiopathic intracranial ... clotting disorders, anemia and malnutrition. Can idiopathic intracranial ... to be “secondary” which affects males and females equally. The second ...

  15. The effect of surgeon experience on outcomes of surgery for adolescent idiopathic scoliosis.

    Science.gov (United States)

    Cahill, Patrick J; Pahys, Joshua M; Asghar, Jahangir; Yaszay, Burt; Marks, Michelle C; Bastrom, Tracey P; Lonner, Baron S; Shah, Suken A; Shufflebarger, Harry L; Newton, Peter O; Betz, Randal R; Samdani, Amer F

    2014-08-20

    Single-surgeon series investigating the learning curve involved in surgery for spinal deformity may be confounded by changes in technology and techniques. Our objective with this multicenter, prospective study was to present a cross-sectional analysis of the impact of surgeon experience on surgery for adolescent idiopathic scoliosis. All posterior-only surgical procedures for adolescent idiopathic scoliosis performed in the 2007 to 2008 academic year, with a minimum of two years of patient follow-up, were included. Two groups were created on the basis of surgeon experience: a young surgeons' group, which included patients of surgeons with less than five years of experience, and an experienced surgeons' group, which included patients of surgeons with five or more years of experience. Nine surgeons (four young and five experienced) operated on a total of one hundred and sixty-five patients with adolescent idiopathic scoliosis. The surgeons' experience ranged from less than one year to thirty-six years in practice. The two groups had similar preoperative curve-magnitude measurements, SRS-22 (Scoliosis Research Society-22) scores, and distribution by Lenke curve type. There were significant operative and postoperative differences. The young surgeons fused an average of 1.2 levels more than the experienced surgeons (p = 0.045). The mean intraoperative estimated blood loss (EBL) of the young surgeons' group was more than twice that of the experienced surgeons' group (2042 mL compared with 1013 mL; p self-image (p = 0.008), and function (p adolescent idiopathic scoliosis were significantly and positively correlated with surgeon experience. Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence. Copyright © 2014 by The Journal of Bone and Joint Surgery, Incorporated.

  16. Mutation of the planar cell polarity gene VANGL1 in adolescent idiopathic scoliosis

    DEFF Research Database (Denmark)

    Andersen, Malene Rask; Farooq, Muhammad; Koefoed, Karen

    2017-01-01

    STUDY DESIGN: Mutation analysis of a candidate disease gene in a cohort of patients with moderate to severe Adolescent idiopathic scoliosis (AIS). OBJECTIVE: To investigate if damaging mutations in the planar cell polarity gene VANGL1 could be identified in AIS patients. SUMMARY OF BACKGROUND DATA......: AIS is a spinal deformity which occurs in 1-3% of the population. The cause of AIS is often unknown, but genetic factors are important in the etiology. Rare variants in genes encoding regulators of WNT/planar cell polarity (PCP) signaling were recently identified in AIS patients. METHODS: We analyzed...

  17. Serial Casting for Infantile Idiopathic Scoliosis: Radiographic Outcomes and Factors Associated With Response to Treatment.

    Science.gov (United States)

    Iorio, Justin; Orlando, Giuseppe; Diefenbach, Chris; Gaughan, John P; Samdani, Amer F; Pahys, Joshua M; Betz, Randal R; Cahill, Patrick J

    Serial casting for early-onset scoliosis has been shown to improve curve deformity. Our goal was to define clinical and radiographic features that determine response to treatment. We retrospectively reviewed patients with idiopathic infantile scoliosis with a minimum of 2-year follow-up. Inclusion criteria were: progressive idiopathic infantile scoliosis and initial casting before 6 years of age. Two groups were analyzed and compared: group 1 (≥10-degree improvement in Cobb angle from baseline) and group 2 (no improvement). Twenty-one patients with an average Cobb angle of 48 degrees (range, 24 to 72 degrees) underwent initial casting at an average age of 2.1 years (range, 0.7 to 5.4 y). Average follow-up was 3.5 years (range, 2 to 6.9 y). Sex, age at initial casting, magnitude of spinal deformity, and curve flexibility (defined as change in Cobb angle from pretreatment to first in-cast radiograph) were not significantly different between groups (P>0.05). Group 1 had a significantly higher body mass index (BMI) than group 2 at the onset of treatment (17.6 vs. 14.8, Pcasted at less than 1.8 years of age had a Cobb angle casting does not confirm treatment success. Key aspects of treatment that may determine success include age of less than 1.8 years at initiation of casting and derotation of the spine to correct rib vertebral angle difference of <20 degrees. Level IV-Therapeutic.

  18. Understanding how axial loads on the spine influence segmental biomechanics for idiopathic scoliosis patients: A magnetic resonance imaging study.

    Science.gov (United States)

    Little, J P; Pearcy, M J; Izatt, M T; Boom, K; Labrom, R D; Askin, G N; Adam, C J

    2016-02-01

    Segmental biomechanics of the scoliotic spine are important since the overall spinal deformity is comprised of the cumulative coronal and axial rotations of individual joints. This study investigates the coronal plane segmental biomechanics for adolescent idiopathic scoliosis patients in response to physiologically relevant axial compression. Individual spinal joint compliance in the coronal plane was measured for a series of 15 idiopathic scoliosis patients using axially loaded magnetic resonance imaging. Each patient was first imaged in the supine position with no axial load, and then again following application of an axial compressive load. Coronal plane disc wedge angles in the unloaded and loaded configurations were measured. Joint moments exerted by the axial compressive load were used to derive estimates of individual joint compliance. The mean standing major Cobb angle for this patient series was 46°. Mean intra-observer measurement error for endplate inclination was 1.6°. Following loading, initially highly wedged discs demonstrated a smaller change in wedge angle, than less wedged discs for certain spinal levels (+2,+1,-2 relative to the apex, (pbiomechanical data on in vivo spinal biomechanics of the scoliotic spine, for analysis of deformity progression and surgical planning. Copyright © 2015 Elsevier Ltd. All rights reserved.

  19. Idiopathic ventricular tachycardia and fibrillation.

    Science.gov (United States)

    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.

  20. A multicenter study analyzing the relationship of a standardized radiographic scoring system of adolescent idiopathic scoliosis and the Scoliosis Research Society outcomes instrument.

    Science.gov (United States)

    Wilson, Philip L; Newton, Peter O; Wenger, Dennis R; Haher, Thomas; Merola, Andrew; Lenke, Larry; Lowe, Thomas; Clements, David; Betz, Randy

    2002-09-15

    A multicenter study examining the association between radiographic and outcomes measures in adolescent idiopathic scoliosis. To evaluate the association between an objective radiographic scoring system and patient quality of life measures as determined by the Scoliosis Research Society outcomes instrument. Although surgical correction of scoliosis has been reported to be positively correlated with patient outcomes, studies to date have been unable to demonstrate an association between radiographic measures of deformity and outcomes measures in patients with adolescent idiopathic scoliosis. A standardized radiographic deformity scoring system and the Scoliosis Research Society outcome tool were used prospectively in seven scoliosis centers to collect data on patients with adolescent idiopathic scoliosis. A total of 354 data points for 265 patients consisting of those with nonoperative or preoperative curves >or=10 degrees, as well as those with surgically treated curves, were analyzed. Correlation analysis was performed to identify significant relationships between any of the radiographic measures, the Harms Study Group radiographic deformity scores (total, sagittal, coronal), and the seven Scoliosis Research Society outcome domains (Total Pain, General Self-Image, General Function, Activity, Postoperative Self-Image, Postoperative Function, and Satisfaction) as well as Scoliosis Research Society outcomes instrument total scores. Radiographic measures that were identified as significantly correlated with Scoliosis Research Society outcome scores were then entered into a stepwise regression analysis. The coronal measures of thoracic curve and lumbar curve magnitude were found to be significantly correlated with the Total Pain, General Self-Image, and total Scoliosis Research Society scores (P Society domain and total scores. No radiographic measures taken after surgery were significantly correlated with the postoperative domains of the Scoliosis Research Society

  1. Use of the scoliosis research society outcomes instrument to evaluate patient outcome in untreated idiopathic scoliosis patients in Japan: part I: comparison with nonscoliosis group: preliminary/limited review in a Japanese population.

    Science.gov (United States)

    Watanabe, Kei; Hasegawa, Kazuhiro; Hirano, Toru; Uchiyama, Seiji; Endo, Naoto

    2005-05-15

    This preliminary study evaluates untreated Japanese patients with idiopathic scoliosis using the Scoliosis Research Society Outcomes Instrument (SRS-24). To determine the baseline patient outcome score using the SRS-24 for untreated Japanese scoliosis patients compared with a nonscoliosis group. The SRS instrument with 24 questions was developed to help evaluate patient-perceived outcomes of idiopathic scoliosis treatment. Evaluation of untreated Japanese idiopathic scoliosis patients using the SRS instrument has not been reported. Japanese idiopathic scoliosis patients (n = 141) (mean age, 13.6 years; range, 10-17 years) with a Cobb angle of more than 20 degrees who were not treated with a brace or surgery, were evaluated in comparison with a nonscoliosis group (healthy junior high school students; n = 72) using the SRS-24. The scoliosis group was categorized as mild deformity group with a major curve Cobb angle of less than 30 degrees, moderate deformity group with 30 degrees to 49 degrees, and severe deformity group with more than 50 degrees. The patients were evaluated using section 1 (15 questions) of the SRS-24, which was divided into four domains: total pain, general self-image, general function, and activity. Reliability, as determined by internal consistency, was validated using Cronbach's alpha for these domain scales. The severe deformity group had the lowest scores compared with the other deformity groups and the nonscoliosis group in pain (P self-image (P self-image of back appearance, were significantly lower in the scoliosis group than those in the nonscoliosis group. This tendency was more significant in the patients with greater curve magnitude. Scores for questions 14 and 15, evaluation of general self-image, in the scoliosis group were, however, higher than those in the nonscoliosis group. Internal consistency using Cronbach's alpha was 0.57 (pain), 0.27 (general self-image), -0.08 (general function), and 0.15 (overall level of activity

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

    Directory of Open Access Journals (Sweden)

    Стюарт Б. Гудмэн

    2014-03-01

    Full Text Available Joint replacement of the lower extremity in Juvenile Idiopathic Arthritis (JIA is becoming more commonly performed worldwide. These young adults experience severe pain and disability from end-stage arthritis, and require joint replacement of the hip or knee to alleviate pain, and restore ambulation and function. These procedures are very challenging from the anesthesia and surgical point of view, due to small overall proportions, numerous bony and other deformities and soft tissue contractures. Joint replacement operations for JIA are best performed by experienced teams, where pre-operative and peri-operative care, and post-operative rehabilitation can be optimized in a collaborative, patient-centered environment.

  3. Comparison of Srs-24 And Srs-22 Scores in Thirty Eight Adolescent Idiopathic Scoliosis Patients Who Had Undergone Surgical Correction

    OpenAIRE

    CYW Chan; LB Saw; MK Kwan

    2009-01-01

    Adolescent idiopathic scoliosis is a spinal deformity that affects patients’ self image and confidence. Surgery is offered when the curvature is greater than 50 degrees based on the likelihood of curvature progression. Outcome measures for scoliosis correction can be described in terms of radiological improvement or improvement of health related quality of life scores. The Scoliosis Research Society 22 (SRS-22) and Scoliosis Research Society 24 (SRS-24) questionnaires are widely accepted and ...

  4. Genetics Home Reference: idiopathic pulmonary fibrosis

    Science.gov (United States)

    ... these health problems has idiopathic pulmonary fibrosis . Other respiratory diseases, some of which are less serious, can cause similar signs and symptoms. In people with idiopathic pulmonary fibrosis , scarring of the lungs increases over time until the lungs can no longer ...

  5. Aetiology of idiopathic granulomatous mastitis.

    Science.gov (United States)

    Altintoprak, Fatih; Kivilcim, Taner; Ozkan, Orhan Veli

    2014-12-16

    Idiopathic granulomatous mastitis is a rare chronic inflammatory lesion of the breast that can clinically and radiographically mimic breast carcinoma. The most common clinical presentation is an unilateral, discrete breast mass, nipple retraction and even a sinus formation often associated with an inflammation of the overlying skin. The etiology of idiopathic granulomatous mastitis is still obscure. Its treatment remains controversial. The cause may be the autoimmune process, infection, a chemical reaction associated with oral contraceptive pills, or even lactation. Various factors, including hormonal imbalance, autoimmunity, unknown microbiological agents, smoking and α 1-antitrypsin deficiency have been suggested to play a role in disease aetiology. In this review, causing factors in the aetiology of idiopathic granulomatous mastitis are reviewed in detail.

  6. Do adolescent idiopathic scoliosis (AIS neglect proprioceptive information in sensory integration of postural control?

    Directory of Open Access Journals (Sweden)

    Christine Assaiante

    Full Text Available INTRODUCTION: It has been reported that AIS rely much more on ankle proprioception to control the amplitude of the balance control commands as compared to age-matched healthy adolescents. Our hypothesis was that AIS do not neglect proprioceptive information to control posture probably because of their vestibular deficits. We investigated the proprioceptive contribution to postural control in AIS which expresses spinal deformity during a crucial transitional period of ontogenesis. METHODS: 10 adolescents with idiopathic scoliosis (AIS with moderate spinal deformity (10° 35° and 10 control adolescents (CA had to maintain vertical stance while very slow oscillations in the frontal plane (below the detection threshold of the semicircular canal system were applied to the support with the eyes open and closed. Postural orientation and segmental stabilisation were analysed at head, shoulder, trunk and pelvis levels. RESULTS: Scoliosis did not affect vertical orientation control and segmental stabilization strategies. Vision improves postural control in both CA and AIS, which seem more dependent on visual cues than adults. CONCLUSIONS: AIS as CA were unable to control efficiently their postural orientation on the basis of the proprioceptive cues, the only sensory information available in the EC situation, whereas in the same condition healthy young adults present no difficulty to achieve the postural control. This suggests that AIS as CA transitory neglect proprioceptive information to control their posture. These results and previous studies suggest the existence of different afferent pathways for proprioceptive information subserving different parts in sensory integration of postural control. We conclude that the static proprioceptive system is not affected by the idiopathic scoliosis, while the dynamic proprioceptive system would be mainly affected.

  7. Do the SRS-22 self-image and mental health domain scores reflect the degree of asymmetry of the back in adolescent idiopathic scoliosis?

    OpenAIRE

    Cheshire, James; Gardner, Adrian; Berryman, Fiona; Pynsent, Paul

    2017-01-01

    Background Patient-reported outcomes are becoming increasingly recognised in the management of patients with adolescent idiopathic scoliosis (AIS). Integrated Shape Imaging System 2 (ISIS2) surface topography is a validated tool to assess AIS. Previous studies have failed to demonstrate strong correlations between AIS and patient-reported outcomes highlighting the need for additional objective surface parameters to define the deformities associated with AIS. The aim of this study was to exami...

  8. Idiopathic pulmonary fibrosis: treatment update.

    LENUS (Irish Health Repository)

    O'Connell, Oisin J

    2011-11-01

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

  9. Retrospective panoramic radiographic analysis for idiopathic osteosclerosis in Indians

    Directory of Open Access Journals (Sweden)

    Srikanth H Srivathsa

    2016-01-01

    Full Text Available Introduction: Idiopathic osteosclerosis is an area of increased radiodensity observed on panoramic radiographs. The prevalence of this entity is not known, especially in Indians. Aims and Objectives: To determine the prevalence and epidemiological characteristics of idiopathic osteosclerosis. Materials and Methods: Six hundred and forty panoramic radiographs were retrospectively analyzed for the presence of idiopathic osteosclerosis by a single trained oral radiologist. Statistical Analysis: The data obtained were analyzed using Microsoft Excel (Version 2007 for Windows. Results: Idiopathic osteosclerosis was identified in 32 individuals with a prevalence of 5%. There were 21 female (65.7% and 11 male (34.3% participants. There were 31 single, unilateral (96.85% osteosclerotic lesions and 1 (3.15% bilateral lesion. Right side localization was noted in 19 participants (57.57% and left side localization in 14 participants (42.42%. Conclusion: This study illustrates the prevalence of idiopathic osteosclerosis in Indians. Further, it depicts the characteristics of idiopathic osteosclerotic lesions.

  10. [Idiopathic facial paralysis in children].

    Science.gov (United States)

    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.

  11. The effectiveness of sustainable serial casting for clubfoot deformity ...

    African Journals Online (AJOL)

    burden is placed on its financial, medical and social services. The current gold standard .... Recruitment to the clinic was by word of mouth, via maternity nursing staff and media advertising on TV, radio and newspapers. Only 20 of the 25 ...

  12. Total fertilization failure and idiopathic subfertility

    Directory of Open Access Journals (Sweden)

    Goverde Angelique J

    2009-01-01

    Full Text Available Abstract Background To gain more insight in whether failure of intrauterine insemination (IUI treatment in patients with idiopathic subfertility could be related to diminished fertilization, the aim of this study is to compare the fertilization of an initial IVF procedure after six cycles of IUI and the fertilization of an initial IVF procedure without preceding IUI cycles in couples with idiopathic subfertility. Methods We performed a complimentary analysis of a randomized controlled trial, in which the number of total fertilization failure (TFF in the first IVF procedure after unsuccessful IUI was compared to those of IVF without preceding IUI in patients with idiopathic subfertility. These patients participated in a previous study that assessed the cost effectiveness of IUI versus IVF in idiopathic subfertility and were randomized to either IUI or IVF treatment. Results 45 patients underwent IVF after 6 cycles of unsuccessful IUI and 58 patients underwent IVF immediately without preceding IUI. In 7 patients the IVF treatment was cancelled before ovum pick. In the IVF after unsuccessful IUI group TFF was seen in 2 of the 39 patients (5% versus 7 of the 56 patients (13% in the immediate IVF group. After correction for confounding factors the TFF rate was not significantly different between the two groups (p = 0.08, OR 7.4; 95% CI: 0.5–14.9. Conclusion Our data showed that TFF and the fertilization rate in the first IVF treatment were not significantly different between couples with idiopathic subfertility undergoing IVF after failure of IUI versus those couples undergoing IVF immediately without prior IUI treatment. Apparently, impaired fertilization does not play a significant role in the success rate of IUI in patients with idiopathic subfertility.

  13. Value of the fetal plantar shape in prenatal diagnosis of talipes equinovarus.

    Science.gov (United States)

    Liao, Huifang; Cai, Ailu; Wang, Bing; Wang, Xiaoguang; Yan, Zhen; Li, Jingyu

    2012-07-01

    The purpose of this study was to evaluate the value of the fetal plantar shape in prenatal diagnosis of talipes equinovarus. A case-control study was conducted between September 2009 and February 2011. We measured the width and length of 249 feet (156 fetuses) included in this study and then calculated the width to length ratio. All of the fetuses were followed to obtain the pregnancy outcomes and confirm whether the deformity existed; then the bimalleolar angle of each foot with talipes equinovarus was measured. Independent samples t tests were performed to compare the foot width, length, and width to length ratio between normal and talipes equinovarus groups. We also assessed the correlation between the width to length ratio and bimalleolar angle in the talipes equinovarus cases with the Pearson correlation coefficient. Statistically significant differences were shown between the two groups (Pshape can provide valuable information for prenatal diagnosis of clubfoot. Compared with a normal foot, a clubfoot tends to be wider and shorter. A higher width to length ratio is associated with a smaller bimalleolar angle and indicates a more severe talipes equinovarus deformity.

  14. Drug therapy for chronic idiopathic axonal polyneuropathy

    NARCIS (Netherlands)

    Vrancken, A. F. J. E.; van Schaik, I. N.; Hughes, R. A. C.; Notermans, N. C.

    2004-01-01

    BACKGROUND: Chronic idiopathic axonal polyneuropathy is an insidiously progressive sensory or sensorimotor polyneuropathy that affects elderly people. Although severe disability or handicap does not occur, it reduces quality of life. OBJECTIVES: To assess whether drug therapy for chronic idiopathic

  15. Rare causes of scoliosis and spine deformity: experience and particular features

    Directory of Open Access Journals (Sweden)

    Pliarchopoulou Fani M

    2007-10-01

    Full Text Available Abstract Background Spine deformity can be idiopathic (more than 80% of cases, neuromuscular, congenital or neurofibromatosis-related. However, there are many disorders that may also be involved. We present our experience treating patients with scoliosis or other spine deformities related to rare clinical entities. Methods A retrospective study of the records of a school-screening study in North-West Greece was performed, covering a 10-year period (1992–2002. The records were searched for patients with deformities related to rare disorders. These patients were reviewed as regards to characteristics of underlying disorder and spine deformity, treatment and results, complications, intraoperative and anaesthesiologic difficulties particular to each case. Results In 13 cases, the spine deformity presented in relation to rare disorders. The underlying disorder was rare neurological disease in 2 cases (Rett syndrome, progressive hemidystonia, muscular disorders (facioscapulohumeral muscular dystrophy, arthrogryposis in 2 patients, osteogenesis imperfecta in 2 cases, Marfan syndrome, osteopetrosis tarda, spondyloepiphyseal dysplasia congenita, cleidocranial dysplasia and Noonan syndrome in 1 case each. In 2 cases scoliosis was related to other congenital anomalies (phocomelia, blindness. Nine of these patients were surgically treated. Surgery was avoided in 3 patients. Conclusion This study illustrates the fact that different disorders are related with curves with different characteristics, different accompanying problems and possible complications. Investigation and understanding of the underlying pathology is an essential part of the clinical evaluation and preoperative work-up, as clinical experience at any specific center is limited.

  16. Idiopathic Scoliosis from Psychopathological and Mind-Body Medicine Perspectives.

    Science.gov (United States)

    Talić, Goran; Ostojić, Ljerka; Bursać, Snježana Novaković; Nožica-Radulović, Tatjana; Stevanović-Papić, Đurđica

    2016-12-01

    Idiopathic scoliosis, defined as a three-dimensional spine and trunk deformity, which appears in otherwise healthy subjects, exhibits complex relations with various forms of personal well-being and psychopathology. Most research studies have documented a higher proportion of psychological disturbances (e.g., self-criticism, negative body image, low self-esteem) and mental disorders (e.g., anxiety and depressive disorders, personality disorders) among idiopathc scoliosis patients compared to healthy controls. In addition, there are some reports, although more systematic research is warranted, on the role of mental health and personality traits in relation to the adherence to conservative treatment. Given the increasing role of surgical treatment in the management of scoliosis, as well as several reports on negative psychological outcomes of such interventions, there is a growing need for ongoing screening and mental health care in this population. It seems this also holds true for non-operative treatments, particularly bracing therapy. One should keep in mind that these scoliosis-psychopathology relations are deduced from a limited number of empirical studies, usually conducted on small sample sizes, suggesting the need for further large-scale investigations, preferrably those with longitudinal research designs. Understanding the complex interplay between personality/psychopathology and spinal deformities within the framework of personalized mind-body medicine, should help clinicians tailor more individualized and specific treatments and predict therapeutic outcomes in this clinical population.

  17. [Juvenile idiopathic arthritis and oral health].

    Science.gov (United States)

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

    2016-05-04

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

  18. Role of the IL-6 Gene in the Etiopathogenesis of Idiopathic Scoliosis

    Directory of Open Access Journals (Sweden)

    Svetla Nikolova

    2015-01-01

    Full Text Available Scoliotic human nuclei pulposi can respond to exogenous proinflammatory stimuli by secreting increased amounts of interleukin-6 (IL-6. The G/C polymorphism of the promoter region of IL-6 gene influences levels and functional activity of the IL-6 protein. We conducted a case-control study of eighty patients with idiopathic scoliosis (IS and one hundred sixty healthy unrelated gender-matched controls trying to investigate the association between IS and the IL-6 promoter polymorphism at -174 position (rs1800795 G/C in Bulgarian population. Molecular detection of the IL-6 genotypes was performed by amplification followed by restriction technology. The statistical analysis was performed by Pearson’s chi-squared test. Our case-control study revealed a statistically significant association between the IL-6 (-174 G/C functional polymorphism and susceptibility to IS. In addition, a significant association between the IL-6 (-174 G/C polymorphism and curve severity was detected. IL-6 gene could be considered as susceptibility and modifying factor of idiopathic scoliosis. The identification of molecular markers with diagnostic and prognostic value could be useful for early detection of children at risk for the development of scoliosis and for prognosis of the risk for a rapid deformity progression. That would facilitate the therapy decisions and early stage treatment of the patient with the least invasive procedures.

  19. Adolescent Idiopathic Scoliosis

    Science.gov (United States)

    ... Radiation Exposure in Scoliosis Kyphosis Adolescent Back Pain Spondylolysis For Adolescents For Adults Common Questions & Glossary Resources ... Radiation Exposure in Scoliosis Kyphosis Adolescent Back Pain Spondylolysis For Adolescents For Adults Juvenile Idiopathic Scoliosis Diagnosed ...

  20. Quantitative electromyographic characteristics of idiopathic unilateral vocal fold paralysis.

    Science.gov (United States)

    Chang, Wei-Han; Fang, Tuan-Jen; Li, Hsueh-Yu; Jaw, Fu-Shan; Wong, Alice M K; Pei, Yu-Cheng

    2016-11-01

    Unilateral vocal fold paralysis with no preceding causes is diagnosed as idiopathic unilateral vocal fold paralysis. However, comprehensive guidelines for evaluating the defining characteristics of idiopathic unilateral vocal fold paralysis are still lacking. In the present study, we hypothesized that idiopathic unilateral vocal fold paralysis may have different clinical and neurologic characteristics from unilateral vocal fold paralysis caused by surgical trauma. Retrospective, case series study. Patients with unilateral vocal fold paralysis were evaluated using quantitative laryngeal electromyography, videolaryngostroboscopy, voice acoustic analysis, the Voice Outcome Survey, and the Short Form-36 Health Survey quality-of-life questionnaire. Patients with idiopathic and iatrogenic vocal fold paralysis were compared. A total of 124 patients were recruited. Of those, 17 with no definite identified causes after evaluation and follow-up were assigned to the idiopathic group. The remaining 107 patients with surgery-induced vocal fold paralysis were assigned to the iatrogenic group. Patients in the idiopathic group had higher recruitment of the thyroarytenoid-lateral cricoarytenoid muscle complex and better quality of life compared with the iatrogenic group. Idiopathic unilateral vocal fold paralysis has a distinct clinical presentation, with relatively minor denervation changes in the involved laryngeal muscles, and less impact on quality of life compared with iatrogenic vocal fold paralysis. 4. Laryngoscope, 126:E362-E368, 2016. © 2016 The American Laryngological, Rhinological and Otological Society, Inc.

  1. Syphilis mimicking idiopathic intracranial hypertension

    DEFF Research Database (Denmark)

    Yri, Hanne; Wegener, Marianne; Jensen, Rigmor

    2011-01-01

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

  2. Postoperative Perfection: Ceiling Effects and Lack of Discrimination With Both SRS-22 and -24 Outcomes Instruments in Patients With Adolescent Idiopathic Scoliosis.

    Science.gov (United States)

    Bastrom, Tracey P; Bartley, Carrie; Marks, Michelle C; Yaszay, Burt; Newton, Peter O

    2015-12-01

    Review of a prospective database registry. To compare the Scoliosis Research Society (SRS)-22 and SRS-24 outcomes instruments in terms of scores, rate of ceiling effects, and discriminant ability in patients with pre- and postoperative adolescent idiopathic scoliosis. Despite improvements noted with the SRS-22, the SRS-24 is still occasionally used prospectively and for comparisons with previous studies reporting SRS-24 scores. Previous work has demonstrated that postoperative scores from the 2 versions are not interchangeable. A multicenter prospective registry of patients who underwent surgical correction of adolescent idiopathic scoliosis was queried for preoperative and 2-year postoperative SRS-22 and SRS-24 scores. Scores were compared between versions and ceiling effects were identified. Groups of deformity severity were created to evaluate discriminant ability. 829 patients were identified. The SRS-22 scores for pain and general function were significantly greater than SRS-24 scores (P self-image (P self-image domain was able to discriminate between large (29°+) and small (≤11°) residual curves (P < 0.05). Scores obtained by the SRS-22 and the SRS-24 are not translatable despite shared domains. Whereas both versions demonstrated preoperative discriminant ability, postoperative discrimination of residual deformity is lacking in both. Patient-reported outcomes of treatment are crucial in advancing treatment, and improvement in the ability to assess subjective outcomes is essential. 3.

  3. Idiopathic Gingival Fibromatosis: Case Report and Its Management

    Directory of Open Access Journals (Sweden)

    Prashant P. Jaju

    2009-01-01

    Full Text Available Idiopathic gingival fibromatosis is a rare condition. We present a case of idiopathic gingival fibromatosis with its multidisciplinary approach of management. The clinical, radiographic, and histopathological features have been described in detail.

  4. Intraspinal anomalies in early-onset idiopathic scoliosis.

    Science.gov (United States)

    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.

  5. Idiopathic epileptic syndromes and cognition.

    Science.gov (United States)

    Hommet, Caroline; Sauerwein, Hannelore C; De Toffol, Bertrand; Lassonde, Maryse

    2006-01-01

    Epilepsy is frequently associated with cognitive impairments which result from various interacting factors. The present paper deals with the contribution of neuropsychology to the characterization of the type of epilepsy and the possible mechanisms underlying idiopathic epileptic syndromes. The non-lesional, so-called idiopathic epilepsies, constitute an interesting model for assessing the relationship between epileptiform EEG discharges and cognition. Among the idiopathic generalized epilepsies, disorders of social integration and personality have been frequently reported in juvenile myoclonic epilepsy (JME). Since similar disturbances are observed in frontal-lobe-lesioned patients, impairments in other frontal lobe functions (e.g. executive functions) might be expected in JME. This gives rise to speculation about the possible underlying pathophysiological mechanisms in JME. With regard to partial idiopathic epilepsies, benign childhood epilepsy with centrotemporal spikes (BCECTS) may provide a useful model for the study of the relationship between epileptiform EEG discharges in the peri-sylvian region and language functions. Furthermore, the description of mild cognitive dysfunctions in BCECTS, and their persistence into adulthood, can provide information about compensatory mechanisms and may allow for the generation of remedial strategies. Thus, 'lesional' neuropsychology has given way to 'dynamic' neuropsychology based on specific postulates. By using the cognitive profile to specify the mechanism underlying the behavioral disturbances observed in different types of epilepsy, neuropsychology may eventually contribute to a revision of the present classification of epileptic syndromes. In addition, the neuropsychological data may help predict the extent and limits of functional recovery and cerebral plasticity.

  6. Idiopathic granulomatous mastitis

    International Nuclear Information System (INIS)

    Ozturk, E.; Akin, M.; Can, Mehmet F.; Ozrehan, I.; Yagci, G.; Tufan, T.; Kurt, B.

    2009-01-01

    Objective was to discuss the clinical and radiological features and treatment approaches in 14 patients diagnosed with idiopathic granulomatous mastitis (GM). We retrospectively evaluated the clinical features, radiological findings and treatment approaches in 14 patients with idiopathic GM in the General Surgery Department, Gulhane School of Medicine, Ankara, Turkey between April 2000 and June 2006. The mean age of the patients was 34.5 years (range 27-41 years). The complaints at admission were a mass in the breast in 7 (50%) patients, an abscess and a mass in 6 (42.8%) and a skin fisculain one (7.2%). Granulomatous mastitis was unilateral in all subjects (on the right in 5 patients and on the left in 9). All of the patients underwent ultrasonographic evaluation. Mammography was performed in 8 and magnetic resonance imaging in 5 patients. Seven patients (50%) were suspected to have breast carcinoma according radiological findings. We performed the large excision in 11, incisional biopsy plus abscess drainage in one, and incisional biopsy plus abscess drainage plus medical treatment (prednisolone, methotrexate) in 2 patients. Due to the development of abscess after 9 months, drainage and large excision were performed in one patient who received medical treatment. Idiopathic GM is a disease that generally affects young women of reproductive age and may be mistaken for breast carcinoma in clinical and radiological evaluations. The gold standard for the diagnosis is histopathologic evaluation. (author)

  7. Angiopoietin-2 polymorphism in women with idiopathic recurrent miscarriage.

    Science.gov (United States)

    Pietrowski, Detlef; Tempfer, Clemens; Bettendorf, Hertha; Bürkle, Bernd; Nagele, Fritz; Unfried, Gertrud; Keck, Christoph

    2003-10-01

    To investigate the relationship between idiopathic recurrent miscarriage and a polymorphism of the gene encoding for angiopoietin-2 (ANGPT2), an autochthonous modulator of angiogenesis during pregnancy. Prospective case control study. Academic research institution. One hundred thirty-one women with a history of three or more consecutive pregnancy losses before 20 weeks' gestation, and 125 healthy, postmenopausal controls with at least two live births and no history of pregnancy loss. Peripheral venous puncture. Polymerase chain reaction and restriction fragment length polymorphism analysis were performed to identify the different ANGPT2 alleles. No association between mutant (mt) allele and the occurrence of idiopathic recurrent miscarriage was found. Between women with primary and secondary idiopathic recurrent miscarriage, no statistically significant differences with respect to allele frequencies were observed. This is the first report on the ANGPT2 gene polymorphism in women with idiopathic recurrent miscarriage, demonstrating that the investigated polymorphism is not associated with idiopathic recurrent miscarriage in a white population.

  8. Acute exacerbation of idiopathic pulmonary fibrosis triggered by Aspergillus empyema

    Directory of Open Access Journals (Sweden)

    Atsushi Suzuki

    Full Text Available 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. Keywords: Idiopathic pulmonary fibrosis, Acute exacerbation, AE-IPF, Triggered AE, Aspergillus infection

  9. Isotope scanning with /sup 99/Tcsup(m)-MDP of the spine and the costosternal junctions of patients with idiopathic scoliosis

    Energy Technology Data Exchange (ETDEWEB)

    Normelli, H.; Lewander, R.

    Bone scanning of the thoracolumbar spine and the anterior thorax was performed in 7 girls with recently diagnosed progressive thoracic idiopathic scoliosis. A reference group consisted of a group of 8 patients submitted to bone scanning for other reasons. In all 7 girls composing the scoliosis group the isotope uptake by the vertex vertebra, the 2 vertebrae above and the 2 below was homogeneous, with no areas of abnormally increased uptake. In 5 of these patients where quantitative studies were performed there was no significant difference in uptake between the vertex and the other 4 vertebrae. Nor did the 2 groups differ significantly as regards the left-right difference in uptake by the costosternal junctions. The results of this investigation confirm the observation in a preliminary study that there was no disturbance of spinal growth during the early stage of development of idiopathic thoracic scoliosis. However, the results did not support a tentative conclusion drawn on the basis of the preliminary study - namely, that the development of spinal deformity in idiopathic scoliosis might be ascribed to asymmetric longitudinal rib growth, reflected in asymmetric isotope uptake by the paired costosternal junctions. It is questionable however, whether scintigraphic scanning can provide an accurate procedure for quantitative measurement of skeletal growth of the ribs, especially in scoliotic patients. (orig.).

  10. Intestinal Volvulus in Idiopathic Steatorrhea

    Science.gov (United States)

    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

  11. Idiopathic epiretinal membrane

    NARCIS (Netherlands)

    Bu, Shao-Chong; Kuijer, Roelof; Li, Xiao-Rong; Hooymans, Johanna M M; Los, Leonoor I

    2014-01-01

    Background: Idiopathic epiretinal membrane (iERM) is a fibrocellular membrane that proliferates on the inner surface of the retina at the macular area. Membrane contraction is an important sight-threatening event and is due to fibrotic remodeling. Methods: Analysis of the current literature

  12. IDIOPATHIC SCOLIOSIS. /LECTURE, PART I. «PARADOXES»/

    Directory of Open Access Journals (Sweden)

    Михаил Георгиевич Дудин

    2013-03-01

    Full Text Available In the paper we discussed and analyzed the issues that confront practicing orthopedists with the most mysterious and at the same time the most studied vertebral column lesion in children and adolescents - idiopathic scoliosis. Nowadays a great amount of information on its various aspects has been already accumulated, but a practical output in the form of a system of effective treatment has not been yet found and (we can’t even speak about there is no speech at all about the prevention (prophylactic of the disease (scoliosis. On the basis of the own many year’s experience with this category of patients and the results of a comprehensive multi-faceted survey, the authors acquired the right to form their own point of view on the etiology and pathogenesis of the three-plane deformation in orthograde human (homo erectus. In this paper, the authors present their reflections on the history of the study of scoliosis, the terminology, statistical indicators and the existing views on its origins. Concerning argumentation on the own findings (conclusions and views on the disease the authors plan to tell in the following sections.

  13. Adults with idiopathic scoliosis improve disability after motor and cognitive rehabilitation: results of a randomised controlled trial.

    Science.gov (United States)

    Monticone, Marco; Ambrosini, Emilia; Cazzaniga, Daniele; Rocca, Barbara; Motta, Lorenzo; Cerri, Cesare; Brayda-Bruno, Marco; Lovi, Alessio

    2016-10-01

    To evaluate the effects of motor and cognitive rehabilitation on disability in adults with idiopathic scoliosis at lower risk of progression. 130 adults with idiopathic scoliosis (main curve rehabilitation programme consisting of active self-correction, task-oriented exercises and cognitive-behavioural therapy (experimental group, 65 subjects, mean age of 51.6, females 48) or general physiotherapy consisting of active and passive mobilizations, stretching, and strengthening exercises of the spinal muscles (control group, 65 subjects, mean age of 51.7, females 46). Before, at the end, and 12 months after treatment, each participant completed the Oswestry disability index (ODI) (primary outcome), the Tampa scale for kinesiophobia, the pain catastrophizing scale, a pain numerical rating scale, and the Scoliosis Research Society-22 Patient Questionnaire. Radiological (Cobb angle) and clinical deformity (angle of trunk rotation) changes were also investigated. A linear mixed model for repeated measures was used for each outcome. Significant effects of time, group, and time by group interaction were found for all outcome measures (P cognitive rehabilitation also led to improvements in dysfunctional thoughts, pain, and quality of life. Changes were maintained for at least 1 year.

  14. Polish adaptation of Bad Sobernheim Stress Questionnaire-Brace and Bad Sobernheim Stress Questionnaire-Deformity.

    Science.gov (United States)

    Misterska, Ewa; Głowacki, Maciej; Harasymczuk, Jerzy

    2009-12-01

    Bad Sobernheim Stress Questionnaire-Brace and Bad Sobernheim Stress Questionnaire-Deformity are relatively new tools aimed at facilitating the evaluation of long-term results of therapy in persons with idiopathic scoliosis undergoing conservative treatment. To use these tools properly in Poland, they must be translated into Polish and adapted to the Polish cultural settings. The process of cultural adaptation of the questionnaires was compliant with the guidelines of International Quality of Life Assessment (IQOLA) Project. In the first stage, two independent translators converted the originals into Polish. Stage two, consisted of a comparison of the originals and two translated versions. During that stage, the team of two translators and authors of the project identified differences in those translations and created a combination of the two. In the third stage, two independent translators, who were native speakers of German, translated the adjusted version of the Polish translation into the language of the original document. At the last stage, a commission composed of: specialists in orthopedics, translators, a statistician and a psychologist reviewed all translations and drafted a pre-final version of the questionnaires. Thirty-five adolescent girls with idiopathic scoliosis who were treated with Cheneau brace were subjected to the questionnaire assessment. All patients were treated in an out-patient setting by a specialist in orthopedics at the Chair and Clinic of Orthopedics and Traumatology. Median age of patients was 14.8 SD 1.5, median value of the Cobb's angle was 27.8 degrees SD 7.4. 48.6% of patients had thoracic scoliosis, 31.4% had thoracolumbar scoliosis, and 20% patients had lumbar scoliosis. Median results obtained by means of the Polish version of BSSQ-Brace and BSSQ-Deformity questionnaires were 17.9 SD 5.0 and 11.3 SD 4.7, respectively. Internal consistency of BSSQ-Brace and BSSQ-Deformity was at the level of 0.80 and 0.87, whereas the value of

  15. Ponseti's manipulation in neglected clubfoot in children more than 7 years of age: a prospective evaluation of 25 feet with long-term follow-up.

    Science.gov (United States)

    Khan, Shah Alam; Kumar, Ashok

    2010-09-01

    We wanted to evaluate the efficacy of Ponseti's technique in neglected clubfoot in children more than 7 years of age. The results of Ponseti's method were evaluated in 21 children (25 feet) with neglected club feet. Patients were evaluated using the Dimeglio scoring system. All patients underwent percutaneous tenotomy of the Achilles tendon. The mean age at the time of treatment was 8.9 years. The mean follow-up period was 4.7 years. The average Dimeglio score at the start of the treatment was 14.2 compared with an average score of 0.95 at the end of the treatment at 1-year follow-up. Eighteen feet (85.7%) had full correction. Recurrence was seen in six feet (24%). At 4-year follow-up, the average Dimeglio score for 19 feet was 0.18. We recommend that Ponseti's method should be the preferred initial treatment modality for neglected clubfeet.

  16. Bipolar sealer device reduces blood loss and transfusion requirements in posterior spinal fusion for adolescent idiopathic scoliosis.

    Science.gov (United States)

    Gordon, Zachary L; Son-Hing, Jochen P; Poe-Kochert, Connie; Thompson, George H

    2013-01-01

    Reducing perioperative blood loss and transfusion requirements is important in the operative treatment of idiopathic scoliosis. This can be achieved with special frames, cell saver systems, pharmacologic aspects, and other techniques. Recently there has been interest in bipolar sealer devices as an adjunct to traditional monopolar electrocautery. However, there is limited information on this device in pediatric spinal deformity surgery. We reviewed our experience with this device in a setting of a standard institutional operative carepath. Perioperative blood loss and transfusion requirements of 50 consecutive patients with adolescent idiopathic scoliosis undergoing a posterior spinal fusion and segmental spinal instrumentation and who had a bipolar sealer device used during their surgery was compared with a control group of the 50 preceding consecutive patients who did not. Anesthesia, surgical technique, use of intraoperative epsilon aminocaproic acid (Amicar), postoperative protocol, and indications for transfusions (hemoglobin≤7.0 g/dL) were identical in both groups. The preoperative demographics for the patients in both groups were statistically the same. The bipolar sealer group demonstrated a significant reduction in intraoperative estimated blood loss, total perioperative blood loss, volume of blood products transfused, and overall transfusion rate when compared with the control group. When subgroups consisting of only hybrid or all-pedicle screw constructs were considered individually, these findings remained consistent. There were no complications associated with the use of this device. Using the bipolar sealer device is a significant adjunct in decreasing perioperative blood loss and transfusion requirements in patients undergoing surgery for adolescent idiopathic scoliosis. Level III-retrospective comparative study.

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

    NARCIS (Netherlands)

    Veldhuizen, Albert Gerrit

    1985-01-01

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

  18. Influence of implant rod curvature on sagittal correction of scoliosis deformity.

    Science.gov (United States)

    Salmingo, Remel Alingalan; Tadano, Shigeru; Abe, Yuichiro; Ito, Manabu

    2014-08-01

    Deformation of in vivo-implanted rods could alter the scoliosis sagittal correction. To our knowledge, no previous authors have investigated the influence of implanted-rod deformation on the sagittal deformity correction during scoliosis surgery. To analyze the changes of the implant rod's angle of curvature during surgery and establish its influence on sagittal correction of scoliosis deformity. A retrospective analysis of the preoperative and postoperative implant rod geometry and angle of curvature was conducted. Twenty adolescent idiopathic scoliosis patients underwent surgery. Average age at the time of operation was 14 years. The preoperative and postoperative implant rod angle of curvature expressed in degrees was obtained for each patient. Two implant rods were attached to the concave and convex side of the spinal deformity. The preoperative implant rod geometry was measured before surgical implantation. The postoperative implant rod geometry after surgery was measured by computed tomography. The implant rod angle of curvature at the sagittal plane was obtained from the implant rod geometry. The angle of curvature between the implant rod extreme ends was measured before implantation and after surgery. The sagittal curvature between the corresponding spinal levels of healthy adolescents obtained by previous studies was compared with the implant rod angle of curvature to evaluate the sagittal curve correction. The difference between the postoperative implant rod angle of curvature and normal spine sagittal curvature of the corresponding instrumented level was used to evaluate over or under correction of the sagittal deformity. The implant rods at the concave side of deformity of all patients were significantly deformed after surgery. The average degree of rod deformation Δθ at the concave and convex sides was 15.8° and 1.6°, respectively. The average preoperative and postoperative implant rod angle of curvature at the concave side was 33.6° and 17.8

  19. Pathology of idiopathic non-cirrhotic portal hypertension.

    Science.gov (United States)

    Guido, Maria; Sarcognato, Samantha; Sacchi, Diana; Colloredo, Guido

    2018-04-12

    Idiopathic non-cirrhotic portal hypertension is an under-recognized vascular liver disease of unknown etiology, characterized by clinical signs of portal hypertension in the absence of cirrhosis. By definition, any disorder known to cause portal hypertension in the absence of cirrhosis and any cause of chronic liver disease must be excluded to make a diagnosis of idiopathic non-cirrhotic portal hypertension. However, the diagnosis is often difficult because the disease resembles cirrhosis and there is no gold standard test. Liver biopsy is an essential tool: it is able to exclude cirrhosis and other causes of portal hypertension and it allows the identification of the characteristic lesions. Nonetheless, the histological diagnosis of idiopathic non-cirrhotic portal hypertension is not always straightforward, in particular by needle biopsy samples, because there is no pathognomonic lesion, but rather a variety of vascular changes which are unevenly distributed, very subtle, and not all necessarily identified in a single specimen. Pathologists should be able to recognize several patterns of injury, involving portal/periportal areas as well as parenchymal structures.The histological features of idiopathic non-cirrhotic portal hypertension are described in this review, focusing on their interpretation in needle biopsy specimens.

  20. Patellofemoral morphometry in patients with idiopathic patellofemoral pain syndrome

    International Nuclear Information System (INIS)

    Mar Carrion Martin, Maria del; Ruiz Santiago, Fernando; Pozuelo Calvo, Rocio; Guzman Alvarez, Luis

    2010-01-01

    Purpose: To compare clinical and computed tomography (CT) measures in extension, 20 o and 30 o of flexion of symptomatic knees of patient with idiopathic patellofemoral pain syndrome with the contra lateral asymptomatic knee. Materials and methods: Knees of 52 consecutive patients with idiopathic patellofemoral pain were studied with CT. In 28 patients this condition was unilateral and asymptomatic knee was used as control; 76 knees were symptomatic. Results: In patients with idiopathic patellofemoral pain we found a greater Q angle and internal condylar facet width in symptomatic knees with regard to asymptomatic knees. Conclusion: Greater Q angle and medial condylar facet can lead to overpressure on the medial knee compartment during maneuvers that increase contact between patella and medial condylar facet, such as knee flexion and squatting, contributing to development of idiopathic patellofemoral pain.

  1. Idiopathic pulmonary fibrosis.

    Science.gov (United States)

    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.

  2. Transient long thoracic nerve injury during posterior spinal fusion for adolescent idiopathic scoliosis: A report of two cases

    Directory of Open Access Journals (Sweden)

    Athanasios I Tsirikos

    2013-01-01

    Full Text Available We present the transient long thoracic nerve (LTN injury during instrumented posterior spinal arthrodesis for idiopathic scoliosis. The suspected mechanism of injury, postoperative course and final outcome is discussed. The LTN is susceptible to injury due to its long and relatively superficial course across the thoracic wall through direct trauma or tension. Radical mastectomies with resection of axillary lymph nodes, first rib resection to treat thoracic outlet syndrome and cardiac surgery can be complicated with LTN injury. LTN injury producing scapular winging has not been reported in association with spinal deformity surgery. We reviewed the medical notes and spinal radiographs of two adolescent patients with idiopathic scoliosis who underwent posterior spinal arthrodesis and developed LTN neuropraxia. Scoliosis surgery was uneventful and intraoperative spinal cord monitoring was stable throughout the procedure. Postoperative neurological examination was otherwise normal, but both patients developed winging of the scapula at 4 and 6 days after spinal arthrodesis, which did not affect shoulder function. Both patients made a good recovery and the scapular winging resolved spontaneously 8 and 11 months following surgery with no residual morbidity. We believe that this LTN was due to positioning of our patients with their head flexed, tilted and rotated toward the contralateral side while the arm was abducted and extended. The use of heavy retractors may have also applied compression or tension to the nerve in one of our patients contributing to the development of neuropraxia. This is an important consideration during spinal deformity surgery to prevent potentially permanent injury to the nerve, which can produce severe shoulder dysfunction and persistent pain.

  3. Is idiopathic recurrent pancreatitis attributed to small stones?

    OpenAIRE

    Chow, Wai-Keung; Peng, Yen-Chun

    2013-01-01

    Idiopathic recurrent pancreatitis remains a clinical challenge. Intraductal ultrasonography in the management of idiopathic recurrent pancreatitis may be a new strategy for undetermined causes after initial diagnostic approaches, including endoscopic retrograde cholangio-pancreatography (ERCP). However, no definite cause after ERCP should be defined under optimal settings and with experienced technique.

  4. Chinese Adaptation of the Bad Sobernheim Stress Questionnaire for Patients With Adolescent Idiopathic Scoliosis Under Brace Treatment.

    Science.gov (United States)

    Xu, Ximing; Wang, Fei; Yang, Mingyuan; Huang, Qikai; Chang, Yifan; Wei, Xianzhao; Bai, Yushu; Li, Ming

    2015-08-01

    Bad Sobernheim Stress Questionnaire (BSSQ)-Deformity and BSSQ-Brace are the most widely used instruments for evaluating stress levels in adolescent idiopathic scoliosis (AIS) patients under brace treatment, and good reliability and validity have been demonstrated across different cultures. Great stress has been found among many adolescents, becoming a major concern for professionals. However, no previous research has addressed the cultural adaptations and psychometric testing of BSSQ-Deformity and BSSQ-Brace in China or the stress levels in AIS patients. The purposes of our study were to evaluate the cross-cultural adaptation and validation of the BSSQ-Deformity and BSSQ-Brace and to investigate stress levels in Chinese (AIS) patients under brace treatment.The original (German) versions of BSSQ-Deformity and BSSQ-Brace were cross-culturally translated according to international guidelines. Psychometric properties such as reliability and construct validity were tested. Eighty-six AIS patients were included in our study, and 50 patients paid a second visit 3 to 7 days later to test reproducibility. Cronbach α and the intraclass coefficient were determined to assess internal consistency and reproducibility. Scoliosis Research Society patient questionnaire-22 (SRS-22) was applied to evaluate construct validity.The mean BSSQ-Deformity and BSSQ-Brace scores were 15.3 and 13.4 points, respectively. Severe stress was observed in 12% of patients due to brace treatment. Item analysis demonstrated that each item was scored under a normal distribution with no redundancy. Psychometric analysis revealed excellent internal consistency (Cronbach α = 0.85 and 0.80, respectively) and reproducibility (intraclass correlation coefficient = 0.85 and 0.90, respectively) for BSSQ-Deformity and BSSQ-Brace. The correlation coefficients of BSSQ-Deformity, BSSQ-Brace and SRS-22 were 0.48 and 0.63, respectively.In conclusion, BSSQ-Deformity and BSSQ-Brace have been successfully

  5. PATTERN OF INHERITANCE OF IDIOPATHIC HYPERCALCIURIA IN TWO FAMILIES

    Directory of Open Access Journals (Sweden)

    A. Nickavar

    2006-09-01

    Full Text Available Idiopathic hypercalciuria is a leading cause of frequency-dysuria syndrome in childhood. Different modes of inheritance have been suggested in this disease. This article presents the occurrence of idiopathic hypercalciuria in all children of two families. In the first family, a 5.5 year old girl with a history of renal stones and dysuria due to hypercalciuria, had two involved brothers and one sister. In the second family, hypercalciuria and medullary nephrocalcinosis were detected in two siblings who were admitted for polyuria and dysuria. Idiopathic type of hypercalciuria was diagnosed in these two families by normal laboratory exams and exclusion of other causes of normocalcemic hypercalciuria. According to the involvement of all offsprings (both sexes in these two families, it is suggested that idiopathic hypercalciuria is an autosomal dominant disease with complete penetration.

  6. Patellofemoral morphometry in patients with idiopathic patellofemoral pain syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Mar Carrion Martin, Maria del [Department of Rehabilitation, Hospital of Traumatology (Ciudad Sanitaria Virgen de las Nieves), Carretera de Jaen SN, 18014 Granada (Spain); Ruiz Santiago, Fernando, E-mail: ferruizsan@terra.e [Department of Radiology, Hospital of Traumatology (Ciudad Sanitaria Virgen de las Nieves), Carretera de Jaen SN, 18014 Granada (Spain); Pozuelo Calvo, Rocio [Department of Rehabilitation, Hospital of Traumatology (Ciudad Sanitaria Virgen de las Nieves), Carretera de Jaen SN, 18014 Granada (Spain); Guzman Alvarez, Luis [Department of Radiology, Hospital of Traumatology (Ciudad Sanitaria Virgen de las Nieves), Carretera de Jaen SN, 18014 Granada (Spain)

    2010-07-15

    Purpose: To compare clinical and computed tomography (CT) measures in extension, 20{sup o} and 30{sup o} of flexion of symptomatic knees of patient with idiopathic patellofemoral pain syndrome with the contra lateral asymptomatic knee. Materials and methods: Knees of 52 consecutive patients with idiopathic patellofemoral pain were studied with CT. In 28 patients this condition was unilateral and asymptomatic knee was used as control; 76 knees were symptomatic. Results: In patients with idiopathic patellofemoral pain we found a greater Q angle and internal condylar facet width in symptomatic knees with regard to asymptomatic knees. Conclusion: Greater Q angle and medial condylar facet can lead to overpressure on the medial knee compartment during maneuvers that increase contact between patella and medial condylar facet, such as knee flexion and squatting, contributing to development of idiopathic patellofemoral pain.

  7. Guidelines for the medical treatment of idiopathic pulmonary fibrosis.

    Science.gov (United States)

    Xaubet, Antoni; Molina-Molina, María; Acosta, Orlando; Bollo, Elena; Castillo, Diego; Fernández-Fabrellas, Estrella; Rodríguez-Portal, José Antonio; Valenzuela, Claudia; Ancochea, Julio

    2017-05-01

    Idiopathic pulmonary fibrosis is defined as chronic fibrosing interstitial pneumonia limited to the lung, with poor prognosis. The incidence has been rising in recent years probably due to improved diagnostic methods and increased life expectancy. In 2013, the SEPAR guidelines for the diagnosis and treatment for idiopathic pulmonary fibrosis were published. Since then, clinical trials and meta-analyses have shown strong scientific evidence for the use of pirfenidone and nintedanib in the treatment of idiopathic pulmonary fibrosis. In 2015, the international consensus of 2011 was updated and new therapeutic recommendations were established, prompting us to update our recommendation for the medical treatment of idiopathic pulmonary fibrosis accordingly. Diagnostic aspects and non-pharmacological treatment will not be discussed as no relevant developments have emerged since the 2013 guidelines. Copyright © 2017 SEPAR. Publicado por Elsevier España, S.L.U. All rights reserved.

  8. Family history of idiopathic REM behavior disorder

    DEFF Research Database (Denmark)

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

    2013-01-01

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

  9. Corrective Bracing for Severe Idiopathic Scoliosis in Adolescence: Influence of Brace on Trunk Morphology

    Directory of Open Access Journals (Sweden)

    Edyta Kinel

    2012-01-01

    Full Text Available Aim. The aim of the work was to study whether wearing a corrective brace by adolescent girls with severe idiopathic scoliosis can influence external shape of the trunk. Methods. Comparison of clinical deformity of two groups of girls matched for age and Cobb angle: group (1 of 23 girls, aged 14.9±1.3 years, Cobb angle 55.0°±6.8°, who refused surgical treatment and have been wearing Chêneau brace for more than 6 months, compared with group (2 of 22 girls, aged 14.1±1.8 years, Cobb angle 59.7°±14.6° never treated with corrective bracing. Clinical deformity was assessed with the Bunnell scoliometer (angle of trunk rotation ATR and surface topography (posterior trunk symmetry index POTSI and Hump Sum HS. Results. The ATR in the primary curvature was 11.9°±3.4° (5°–18° in group 1 versus 15.1°±5.6° (6°–25° in group 2 (P=0.027. The HS was 16.8°±3.8 versus 19.2°±4.6, respectively, P=0.07. The POTSI value did not differ between groups. Conclusion. Girls with Cobb angle above 45 degrees, who have been subjected to brace treatment, revealed smaller clinical deformity of their back comparing to nontreated girls having similar radiological curvatures.

  10. The Impact of Ocular Pressures, Material Properties and Geometry on Optic Nerve Head Deformation

    Science.gov (United States)

    Feola, Andrew J.; Myers, Jerry G.; Raykin, Julia; Nelson, Emily S.; Samuels, Brian C.; Ethier C. Ross

    2017-01-01

    Alteration in intracranial pressure (ICP) has been associated with various diseases that cause visual impairment, including glaucoma, idiopathic intracranial hypertension and Visual Impairment and Intracranial Pressure (VIIP) syndrome. However, how changes in ICP lead to vision loss is unclear, although it is hypothesized to involve deformations of the tissues in the optic nerve head (ONH). Recently, understanding the effect of ICP alterations on ocular tissues has become a major concern for NASA, where 42 of astronauts that partake in long duration space missions suffer from VIIP syndrome. Astronauts with VIIP syndrome suffer from visual impairment and changes in ocular anatomy that persist after returning to earth (1). It is hypothesized that the cephalad fluid shift that occurs upon entering microgravity increases ICP, which leads to an altered biomechanical environment in the posterior globe and optic nerve sheath, and subsequently VIIP syndrome. Our goal was to develop a finite element (FE) model to simulate the acute effects of elevated ICP on the posterior eye. Here, we simulated how inter-individual differences affect the deformation of ONH tissues. Further, we examined how several different geometries influenced deformations when exposed to elevated ICP.

  11. Computed tomography in the assessment of idiopathic spontaneous pneumothorax

    International Nuclear Information System (INIS)

    Kim, Sang Jin; Lee, Doo Yun; Kim, Hyung Jung

    1991-01-01

    It is well known that idiopathic spontaneous pneumothorax is caused by rupture of the subpleural bleb and presents difficulty in exact detection and localization of the bleb with plain chest X - ray alone. The authors performed chest CT scans for accurate diagnosis of bleb that would act as a guide for optimal management of idiopathic spontaneous pneumothorax patients in order to prevent recurrent pneumothorax. We could detect blebs in 93 % (26/28) of the patients with idiopathic spontaneous pneumothorax, and 68 % (19/ 28) of the patient had bilateral blebs. Sensitivity was 0.63, and false negative was 37% (37/100) of the blebs, and 51% (19/37) of these 37 false negative cases were ruptured blebs. Only 7 % (2/28) of the patients had a single bleb. The authors concluded that CT is a useful method of study for optimal management of idiopathic spontaneous pneumothorax patients

  12. Cardiopulmonary Exercise Testing in Patients with Idiopathic Scoliosis.

    Science.gov (United States)

    Shen, Jianxiong; Lin, Youxi; Luo, Jinmei; Xiao, Yi

    2016-10-05

    Scoliosis causes impairment of the respiratory and cardiovascular systems. Traditional pulmonary function tests only examine patients under static conditions. The aim of our study was to investigate the correlation between radiographic parameters and dynamic cardiopulmonary capacity in patients with idiopathic scoliosis. Forty patients with idiopathic scoliosis were included in this prospective study from January 2014 to February 2016. The patients underwent full radiographic assessment of deformity, pulmonary function testing, and cardiopulmonary bicycle ergometer testing. The impact of the severity of thoracic curvature and kyphosis on pulmonary function and physical capacity was investigated. Thirty-three female patients with a mean age of 15.5 years (range, 11 to 35 years) and coronal thoracic curvature of 49.4° (range, 24° to 76°) and 7 male subjects with a mean age of 15.9 years (range, 13 to 18 years) and coronal thoracic curvature of 47.1°(range, 22° to 80°) were included. No correlation was found between coronal thoracic curvature and pulmonary function test results in the female patients. Female patients with a thoracic curve of ≥60° had lower blood oxygen saturation at maximal exercise in the cardiopulmonary exercise test (p = 0.032). Female patients with a thoracic curve of ≥50° had a higher respiratory rate (p = 0.041) and ventilation volume per minute (p = 0.046) and lower breathing reserve at maximal exercise (p = 0.038). Thoracic kyphosis in female patients was positively correlated with pulmonary function, as shown by the forced expiratory volume in 1 second (r = 0.456, p = 0.01), forced vital capacity (r = 0.366, p = 0.043), vital capacity (r = 0.525, p = 0.006), and total lung capacity (r = 0.388, p = 0.031), as well as with tidal volume (r = 0.401, p = 0.025) in cardiopulmonary exercise testing. Female patients who engaged in regular exercise had better peak oxygen intake normalized by body weight (p rate (p = 0.020), and heart rate

  13. Idiopathic scrotal elephantiasis.

    Science.gov (United States)

    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.

  14. Understanding idiopathic intracranial hypertension

    DEFF Research Database (Denmark)

    Markey, Keira A; Mollan, Susan P; Jensen, Rigmor H

    2016-01-01

    Idiopathic intracranial hypertension is a disorder characterised by raised intracranial pressure that predominantly affects young, obese women. Pathogenesis has not been fully elucidated, but several causal factors have been proposed. Symptoms can include headaches, visual loss, pulsatile tinnitus...

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

    Science.gov (United States)

    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.

  16. Idiopathic gastric perforation in an asplenic infant | Olsen | African ...

    African Journals Online (AJOL)

    Introduction: The cause of idiopathic gastric perforations in neonates remains unknown. Perforations of the abdominal oesophagus, stomach and duodenum in infants and children are the rarest type of intestinal perforations. There are 21 reported cases of an idiopathic gastric rupture in nonneonates. Case Report: A ...

  17. IDIOPATHIC SC OLIOSIS. /LECTURE, PART I. «PARADOXES»/

    Directory of Open Access Journals (Sweden)

    Михаил Георгиевич Дудин

    2014-03-01

    Full Text Available In the paper we discussed and analyzed the issues that confront practicing orthopedists with the most mysterious and at the same time the most studied vertebral column lesion in children and adolescents - idiopathic scoliosis. Nowadays a great amount of information on its various aspects has been already accumulated, but a practical output in the form of a system of effective treatment has not been yet found and (we can’t even speak about there is no speech at all about the prevention (prophylactic of the disease (scoliosis. On the basis of the own many year’s experience with this category of patients and the results of a comprehensive multi-faceted survey, the authors acquired the right to form their own point of view on the etiology and pathogenesis of the three-plane deformation in orthograde human (homo erectus. In this paper, the authors present their reflections on the history of the study of scoliosis, the terminology, statistical indicators and the existing views on its origins. Concerning argumentation on the own findings (conclusions and views on the disease the authors plan to tell in the following sections.

  18. Idiopathic short stature

    Directory of Open Access Journals (Sweden)

    Vlaški Jovan

    2013-01-01

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

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

    DEFF Research Database (Denmark)

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

    2010-01-01

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

  20. Clinical and Polysomnographic Comparison between Narcolepsy without Cataplexy and Idiopathic Hypersomnia

    Directory of Open Access Journals (Sweden)

    Tae Won Kim

    2012-10-01

    Full Text Available Background and Objective The aim of this study is to compare the clinical, electrophysiological (Polysomnography, PSG; Multiple Sleep Latency Test, MSLT and biological data (HLA DQB1*0602 typing in idiopathic hypersomnia with narcolepsy without cataplexy. Methods 80 patients with narcolepsy without cataplexy and 71 patients with idiopathic hypersomnia without a long sleep time were recruited at the Sleep Center of St. Vincent’s Hospital. MSLT data and PSG findings from the time of their diagnosis were reviewed. HLA typing was performed. Results Results indicated that the idiopathic hypersomnia group showed a significant longer mean sleep latency in MSLT compared with the narcolepsy without cataplexy group. But there was no significant difference in the Epworth Sleepiness Scale (ESS scores between the two groups. Although HLA positivity of both groups was not statistically significant (p = 0.065, HLA positivity tended to be higher in the narcolepsy without cataplexy group than the idiopathic hypersomnia group. The number of awakenings was slightly higher in the idiopathic hypersomnia group, but there was no statistical significance. The number of spontaneous arousal and total arousal indices was not significantly different between the groups. For the PSG, the idiopathic hypersomnia group showed a significantly longer sleep latency than the narcolepsy without cataplexy group (p = 0.009. REM sleep latency (REML was significantly shorter in the narcolepsy without cataplexy group compared to the idiopathic hypersomnia group. The percentage of REM (SREM was significantly higher in the narcolepsy without cataplexy group, and the percentage of the wake time during sleep period (SWT was significantly lower in the narcolepsy without cataplexy group. Conclusions There were no significant differences of subjective sleep measures such as ESS, disturbed nocturnal sleep, number of naps, age of onset of hypnagogic hallucination, and age of onset of sleep

  1. Pulmonary function tests correlated with thoracic volumes in adolescent idiopathic scoliosis.

    Science.gov (United States)

    Ledonio, Charles Gerald T; Rosenstein, Benjamin E; Johnston, Charles E; Regelmann, Warren E; Nuckley, David J; Polly, David W

    2017-01-01

    Scoliosis deformity has been linked with deleterious changes in the thoracic cavity that affect pulmonary function. The causal relationship between spinal deformity and pulmonary function has yet to be fully defined. It has been hypothesized that deformity correction improves pulmonary function by restoring both respiratory muscle efficiency and increasing the space available to the lungs. This research aims to correlate pulmonary function and thoracic volume before and after scoliosis correction. Retrospective correlational analysis between thoracic volume modeling from plain x-rays and pulmonary function tests was conducted. Adolescent idiopathic scoliosis patients enrolled in a multicenter database were sorted by pre-operative Total Lung Capacities (TLC) % predicted values from their Pulmonary Function Tests (PFT). Ten patients with the best and ten patients with the worst TLC values were included. Modeled thoracic volume and TLC values were compared before and 2 years after surgery. Scoliosis correction resulted in an increase in the thoracic volume for patients with the worst initial TLCs (11.7%) and those with the best initial TLCs (12.5%). The adolescents with the most severe pulmonary restriction prior to surgery strongly correlated with post-operative change in total lung capacity and thoracic volume (r 2  = 0.839; p volume in this group was 373.1 cm 3 (11.7%) which correlated with a 21.2% improvement in TLC. Scoliosis correction in adolescents was found to increase thoracic volume and is strongly correlated with improved TLC in cases with severe restrictive pulmonary function, but no correlation was found in cases with normal pulmonary function. © 2016 Orthopaedic Research Society. Published by Wiley Periodicals, Inc. J Orthop Res 35:175-182, 2017. © 2016 Orthopaedic Research Society. Published by Wiley Periodicals, Inc.

  2. CORRECTIVE SURGERY IN CONGENITAL TALIPES EQUINOVARUS DEFORMITY: A CAMP APPROACH

    Directory of Open Access Journals (Sweden)

    Antony R.

    2015-09-01

    Full Text Available The study was intended to assess the results of soft tissue release and bony corrective surgery in patients of moderate to severe deformed rigid club foot (CTEV and neglected clubfoot (CTEV at free disabled surgical camps at Chhattisgarh state . MATERIAL AND METHODS : In our study 50 patients were included with 70% male and 30% female with 4 - 16 years of age grou p and 70% unilateral and 30% bilateral foot involvement. Patients were admitted and operated in different free disabled surgical camps at Chhattisgarh state over the period of 36 months (1 may 2004 to 30 th April 2007. Improvement in functional ability and locomotion of all operated patients were assessed by physical and clinical examination. RESULTS : All patients who were operated in our study showed significant improvement in functional ability and locomotion after surgery. All patients were maintaining f unctional ability at follow up duration of 12 months (1 year. 75% patients were walking normally, 10% cases were walking with internal rotation of leg and 5% cases were walking with midtarsal varus foot with AFO with medial bar support. CONCLUSION : Our st udy showed and established that excellent results can be obtained in congenital talipes equinovarus (CTEV patients by soft tissue release with bony corrective surgery. The team work of devoted surgeons, paramedical and rehabilitation staff in whole durati on of camps to achieve the goal. With an aim to help more number of CTEV cases by surgery, our team has started doing surgeries in small institutions, and organize charity camps to help poor patients and mankind even in small clinics

  3. Pulmonary tuberculosis in patients with idiopathic pulmonary fibrosis

    Energy Technology Data Exchange (ETDEWEB)

    Chung, Myung Jin; Goo, Jin Mo E-mail: jmgoo@plaza.snu.ac.kr; Im, Jung-Gi

    2004-11-01

    Objectives: Patients with idiopathic pulmonary fibrosis (IPF) have an increased risk of pulmonary tuberculosis. However, detecting pulmonary tuberculosis may be difficult due to the underlying fibrosis. The aim of this report is to describe the radiological and clinical findings of pulmonary tuberculosis in patients with idiopathic pulmonary fibrosis. Materials and methods: We reviewed 143 consecutive patients in whom IPF was diagnosed by either the histological or radio-clinical criteria. Among them, nine patients were histologically (n=2) or bacteriologically (n=7) confirmed to have active pulmonary tuberculosis. The location and patterns of pulmonary tuberculosis were examined on a thin section CT scan. Results: The most common thin section CT findings were subpleural nodules (n=6; mean diameter, 3.2 cm) and a lobar or segmental consolidation (n=3). The lesions were located most commonly in the right lower lobe (n=4). The incidence of tuberculosis in patients with idiopathic pulmonary fibrosis was more than five times higher than that of the general population. Conclusion: The atypical manifestation of pulmonary tuberculosis is common in patients with idiopathic pulmonary fibrosis, which may mimic lung cancer or bacterial pneumonia.

  4. Pulmonary tuberculosis in patients with idiopathic pulmonary fibrosis

    International Nuclear Information System (INIS)

    Chung, Myung Jin; Goo, Jin Mo; Im, Jung-Gi

    2004-01-01

    Objectives: Patients with idiopathic pulmonary fibrosis (IPF) have an increased risk of pulmonary tuberculosis. However, detecting pulmonary tuberculosis may be difficult due to the underlying fibrosis. The aim of this report is to describe the radiological and clinical findings of pulmonary tuberculosis in patients with idiopathic pulmonary fibrosis. Materials and methods: We reviewed 143 consecutive patients in whom IPF was diagnosed by either the histological or radio-clinical criteria. Among them, nine patients were histologically (n=2) or bacteriologically (n=7) confirmed to have active pulmonary tuberculosis. The location and patterns of pulmonary tuberculosis were examined on a thin section CT scan. Results: The most common thin section CT findings were subpleural nodules (n=6; mean diameter, 3.2 cm) and a lobar or segmental consolidation (n=3). The lesions were located most commonly in the right lower lobe (n=4). The incidence of tuberculosis in patients with idiopathic pulmonary fibrosis was more than five times higher than that of the general population. Conclusion: The atypical manifestation of pulmonary tuberculosis is common in patients with idiopathic pulmonary fibrosis, which may mimic lung cancer or bacterial pneumonia

  5. Hypercalcemia in idiopathic myelofibrosis

    DEFF Research Database (Denmark)

    Voss, A; Schmidt, K; Hasselbalch, H

    1992-01-01

    A case of idiopathic myelofibrosis (IMF) presenting with hypercalcemia and hypercalcitriolemia is reported. It is proposed that ectopic production of the active vitamin D metabolite related to ongoing clonal expansion in the bone marrow accounts for the hypercalcemic state. Consistently low level...

  6. Repressive coping and alexithymia in idiopathic environmental intolerance

    DEFF Research Database (Denmark)

    Skovbjerg, Sine; Zachariae, Robert; Rasmussen, Alice

    2010-01-01

    To examine if the non-expression of negative emotions (i.e., repressive coping) and differences in the ability to process and regulate emotions (i.e., alexithymia) is associated with idiopathic environmental intolerance (IEI).......To examine if the non-expression of negative emotions (i.e., repressive coping) and differences in the ability to process and regulate emotions (i.e., alexithymia) is associated with idiopathic environmental intolerance (IEI)....

  7. Sleepiness in Idiopathic REM Sleep Behavior Disorder and Parkinson Disease.

    Science.gov (United States)

    Arnulf, Isabelle; Neutel, Dulce; Herlin, Bastien; Golmard, Jean-Louis; Leu-Semenescu, Smaranda; Cochen de Cock, Valérie; Vidailhet, Marie

    2015-10-01

    To determine whether patients with idiopathic and symptomatic RBD were sleepier than controls, and if sleepiness in idiopathic RBD predicted earlier conversion to Parkinson disease. The Epworth Sleepiness Scale (ESS) and its determinants were compared at the time of a video-polysomnography for an RBD diagnosis in patients with idiopathic RBD, in patients with Parkinson disease, and in controls. Whether sleepiness at time of RBD diagnosis predicted an earlier conversion to neurodegenerative diseases was retrospectively analyzed in the followed-up patients. The 75 patients with idiopathic RBD were sleepier (ESS: 7.8 ± 4.6) at the time of RBD diagnosis than 74 age- and sex-matched controls (ESS: 5.0 ± 3.6, P sleep measures. Among the 69 patients with idiopathic RBD who were followed up for a median 3 years (1-15 years), 16 (23.2%) developed parkinsonism (n = 6), dementia (n = 6), dementia plus parkinsonism (n = 2), and multiple system atrophy (n = 2). An ESS greater than 8 at time of RBD diagnosis predicted a shorter time to phenoconversion to parkinsonism and dementia, from RBD onset, and from RBD diagnosis (when adjusted for age and time between RBD onset and diagnosis). Sleepiness is associated with idiopathic REM sleep behavior disorder and predicts more rapid conversion to parkinsonism and dementia, suggesting it is an early marker of neuronal loss in brainstem arousal systems. © 2015 Associated Professional Sleep Societies, LLC.

  8. Obesity is a significant susceptibility factor for idiopathic AA amyloidosis.

    Science.gov (United States)

    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.

  9. Juvenile idiopathic arthritis – an update on its diagnosis and ...

    African Journals Online (AJOL)

    2015-12-03

    Dec 3, 2015 ... Juvenile idiopathic arthritis (JIA) is the most common form of chronic arthritis in children and the most ... A swollen knee and uveitis in a young girl, for instance, is ..... Methotrexate for treating juvenile idiopathic arthritis.

  10. Allelic variation of the FRMD7 gene in congenital idiopathic nystagmus.

    Science.gov (United States)

    Self, James E; Shawkat, Fatima; Malpas, Crispin T; Thomas, N Simon; Harris, Christopher M; Hodgkins, Peter R; Chen, Xiaoli; Trump, Dorothy; Lotery, Andrew J

    2007-09-01

    To perform a genotype-phenotype correlation study in an X-linked congenital idiopathic nystagmus pedigree (pedigree 1) and to assess the allelic variance of the FRMD7 gene in congenital idiopathic nystagmus. Subjects from pedigree 1 underwent detailed clinical examination including nystagmology. Screening of FRMD7 was undertaken in pedigree 1 and in 37 other congenital idiopathic nystagmus probands and controls. Direct sequencing confirmed sequence changes. X-inactivation studies were performed in pedigree 1. The nystagmus phenotype was extremely variable in pedigree 1. We identified 2 FRMD7 mutations. However, 80% of X-linked families and 96% of simplex cases showed no mutations. X-inactivation studies demonstrated no clear causal link between skewing and variable penetrance. We confirm profound phenotypic variation in X-linked congenital idiopathic nystagmus pedigrees. We demonstrate that other congenital nystagmus genes exist besides FRMD7. We show that the role of X inactivation in variable penetrance is unclear in congenital idiopathic nystagmus. Clinical Relevance We demonstrate that phenotypic variation of nystagmus occurs in families with FRMD7 mutations. While FRMD7 mutations may be found in some cases of X-linked congenital idiopathic nystagmus, the diagnostic yield is low. X-inactivation assays are unhelpful as a test for carrier status for this disease.

  11. Exercises for adolescent idiopathic scoliosis.

    Science.gov (United States)

    Romano, Michele; Minozzi, Silvia; Bettany-Saltikov, Josette; Zaina, Fabio; Chockalingam, Nachiappan; Kotwicki, Tomasz; Maier-Hennes, Axel; Negrini, Stefano

    2012-08-15

    Adolescent idiopathic scoliosis (AIS) is a three-dimensional deformity of the spine . While AIS can progress during growth and cause a surface deformity, it is usually not symptomatic. However, in adulthood, if the final spinal curvature surpasses a certain critical threshold, the risk of health problems and curve progression is increased. The use of scoliosis-specific exercises (SSE) to reduce progression of AIS and postpone or avoid other more invasive treatments is controversial. To evaluate the efficacy of SSE in adolescent patients with AIS. The following databases (up to 30 March 2011) were searched with no language limitations: CENTRAL (The Cochrane Library 2011, issue 2), MEDLINE (from January 1966), EMBASE (from January 1980), CINHAL (from January 1982), SportDiscus (from January 1975), PsycInfo (from January 1887), PEDro (from January 1929). We screened reference lists of articles and also conducted an extensive handsearch of grey literature. Randomised controlled trials and prospective cohort studies with a control group comparing exercises with no treatment, other treatment, surgery, and different types of exercises. Two review authors independently selected studies, assessed risk of bias and extracted data. Two studies (154 participants) were included. There is low quality evidence from one randomised controlled study that exercises as an adjunctive to other conservative treatments increase the efficacy of these treatments (thoracic curve reduced: mean difference (MD) 9.00, (95% confidence interval (CI) 5.47 to 12.53); lumbar curve reduced:MD 8.00, (95% CI 5.08 to 10.92)). There is very low quality evidence from a prospective controlled cohort study that scoliosis-specific exercises structured within an exercise programme can reduce brace prescription (risk ratio (RR) 0.24, (95% CI 0.06 to1.04) as compared to usual physiotherapy (many different kinds of general exercises according to the preferences of the single therapists within different facilities

  12. Idiopathic Scoliosis

    Directory of Open Access Journals (Sweden)

    Jens Ivar Brox

    2014-07-01

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

  13. Use of the Universal Clamp in adolescent idiopathic scoliosis.

    Science.gov (United States)

    Sales de Gauzy, Jérôme; Jouve, Jean-Luc; Ilharreborde, Brice; Blondel, Benjamin; Accadbled, Franck; Mazda, Keyvan

    2014-07-01

    The Universal Clamp (UC) equipped with a soft sublaminar band is a relatively new thoracic anchor that can be used in hybrid constructs. A dedicated reduction tool that applies traction to the sublaminar band permits gentle translation of the thoracic curve to the pre-contoured fusion rods, which have been previously anchored distally by pedicle screws and proximally by hooks in a claw configuration. A literature review concerning the use of UC in adolescent idiopathic Scoliosis was performed. The results confirm the efficacy with the UC hybrid constructs, the good correction (71-66%) of the main thoracic curve and low loss of correction at 2-year follow-up (3-4%). The thoracic curve correction achieved and maintained in the AIS patients appears to be substantially higher than that reported for all hook and hook hybrid techniques. Regardless of the deformity correcting method employed with all-screw constructs, the Cobb angle correction achieved is equivalent to that achieved with UC hybrid devices. Among the present AIS patients who had normal values of thoracic kyphosis pre-operatively, there was almost no change in average thoracic kyphosis at the latest follow-up. However, among the patients with low pre-operative values of T5-T12 kyphosis, the average T5-T12 kyphosis improved. Hybrid construct with UC is safe, with reduced operative time and blood loss. While achieving deformity correction in the coronal and axial planes equivalent to the best-reported results of all screw or previous hybrid constructs, the UC hybrid technique appears to provide superior correction in the sagittal plane. The excellent outcome is maintained at the 2-year follow-up.

  14. Changes in plasma atrial natriuretic factor in patients with idiopathic atrial fibrillation

    International Nuclear Information System (INIS)

    Du Tongxin; Xia Xiaojie; Qu Wei; Wang Shukui; Sun Junjiang

    2002-01-01

    To observe the changes in plasma atrial natriuretic factor (AFN) in patients with idiopathic atrial fibrillation and investigate its mechanism, plasma ANF, platelet count and hematocrit were detected in 21 cases with transient idiopathic atrial fibrillation (group A, A1 representing attack, while A2 termination), 28 with persistent idiopathic atrial fibrillation (group B), 27 suffered from rheumatic heart disease with mitral stenosis and persistent atrial fibrillation (group C), 32 with transient supraventricular tachycardia (group D) and 20 normal controls (group E). It was found that the level of ANF was significantly higher in patients with attacking transient idiopathic atrial fibrillation than that in group A2, D and E (P 0.05), while there was significant difference in hematocrit in group A1 compared with group A2, D, E (P < 0.01). It suggested that ANF and hematocrit play an important role in the attack of idiopathic atrial fibrillation

  15. The clinical profile of idiopathic Parkinson's disease in a South ...

    African Journals Online (AJOL)

    The clinical profile of idiopathic Parkinson's disease in a South African hospital complex - the influence of ethnicity and gender. Marcelle Smith, Girish Modi. Abstract. Background Idiopathic Parkinson's Disease (IPD) has not been well studied in Black African populations. Data on the demographics, phenotype differences ...

  16. MUSCLE DISORDERS IN ADOLESCENT IDIOPATHIC SCOLIOSIS: LITERATURE REVIEW

    OpenAIRE

    Débora Pinheiro Lédio Alves; Barbara de Araújo

    2016-01-01

    ABSTRACT Scoliosis is characterized by three-dimensional changes of the spine and is estimated to be present in 4% of the population worldwide. The most common form is the adolescent idiopathic. The purpose of this study is to identify the major muscle abnormalities found in patients with adolescent idiopathic scoliosis through a literature review. We conducted an electronic search of the national databases PubMed, Lilacs, PEDro, and EMBASE using the keywords "scoliosis", "biomechanics", "exe...

  17. Association between adolescent idiopathic scoliosis prevalence and age at menarche in different geographic latitudes

    Directory of Open Access Journals (Sweden)

    Mihas Constantinos

    2006-05-01

    Full Text Available Abstract Background Age at menarche is considered a reliable prognostic factor for idiopathic scoliosis and varies in different geographic latitudes. Adolescent idiopathic scoliosis prevalence has also been reported to be different in various latitudes and demonstrates higher values in northern countries. A study on epidemiological reports from the literature was conducted to investigate a possible association between prevalence of adolescent idiopathic scoliosis and age at menarche among normal girls in various geographic latitudes. An attempt is also made to implicate a possible role of melatonin in the above association. Material-methods 20 peer-reviewed published papers reporting adolescent idiopathic scoliosis prevalence and 33 peer-reviewed papers reporting age at menarche in normal girls from most geographic areas of the northern hemisphere were retrieved from the literature. The geographic latitude of each centre where a particular study was originated was documented. The statistical analysis included regression of the adolescent idiopathic scoliosis prevalence and age at menarche by latitude. Results The regression of prevalence of adolescent idiopathic scoliosis and age at menarche by latitude is statistically significant (p Conclusion Late age at menarche is parallel with higher prevalence of adolescent idiopathic scoliosis. Pubarche appears later in girls that live in northern latitudes and thus prolongs the period of spine vulnerability while other pre-existing or aetiological factors are contributing to the development of adolescent idiopathic scoliosis. A possible role of geography in the pathogenesis of idiopathic scoliosis is discussed, as it appears that latitude which differentiates the sunlight influences melatonin secretion and modifies age at menarche, which is associated to the prevalence of idiopathic scoliosis.

  18. Comparing new treatments for idiopathic pulmonary fibrosis--a network meta-analysis.

    LENUS (Irish Health Repository)

    Loveman, Emma

    2015-01-01

    The treatment landscape for idiopathic pulmonary fibrosis, a devastating lung disease, is changing. To investigate the effectiveness of treatments for idiopathic pulmonary fibrosis we undertook a systematic review, network meta-analysis and indirect comparison.

  19. Idiopathic hemifacial spasm responsive to zonisamide: a case report.

    Science.gov (United States)

    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.

  20. Pulmonary function in children with idiopathic scoliosis

    Directory of Open Access Journals (Sweden)

    Tsiligiannis Theofanis

    2012-03-01

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

  1. Pediatric Idiopathic Intracranial Hypertension Presenting With Sensorineural Hearing Loss.

    Science.gov (United States)

    Reitsma, Sietze; Stokroos, Robert; Weber, Jacobiene W; van Tongeren, Joost

    2015-12-01

    To present the rare case of a young boy with idiopathic intracranial hypertension presenting with bilateral sensorineural hearing loss developing over several months. This was accompanied by headaches, otalgia, tinnitus, and vertigo. Furthermore, we aim to provide a concise review on this matter, as this report represents the second case in literature of pediatric idiopathic intracranial hypertension presenting with hearing loss. Workup of a 9-year-old boy with bilateral sensorineural hearing loss, including (among others) physical examination, audiometry, diagnostic imaging, and lumbar puncture. Physical examination including fundoscopy as well as imaging showed no abnormalities. At presentation, pure tone audiometry revealed bone conduction thresholds of about 30 dB HL in both ears. Two months later, this declined to about 35 dB HL in both ears. Lumbar puncture revealed an increased intracranial pressure. The boy was thus diagnosed with idiopathic intracranial hypertension. After the lumbar puncture, the otological complaints gradually resolved, and the hearing normalized (bone conduction thresholds of 0-5 dB HL). Although rare, sensorineural hearing loss in the pediatric population together with otalgia, tinnitus, and vertigo can be due to idiopathic intracranial hypertension and as such can be reversible. © The Author(s) 2015.

  2. Idiopathic intracranial hypertension in pediatric patients

    Directory of Open Access Journals (Sweden)

    Nada Jirásková

    2008-11-01

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

  3. Single photon emission computed tomography in children with idiopathic seizures

    International Nuclear Information System (INIS)

    Hara, Masafumi; Takahashi, Mutsumasa; Kojima, Akihiro; Shimomura, Osamu; Kinoshita, Rumi; Tomiguchi, Seiji; Taku, Keiichi; Miike, Teruhisa

    1991-01-01

    Single photon emission computed tomography (SPECT) with N-isoprophyl-p [ 123 I]-iodoamphetamine (IMP), X-ray computed tomography (X-CT), and magnetic resonance imaging (MRI) were performed in 20 children with idiopathic seizures. In children with idiopathic seizures, SPECT could detect the abnormal sites at the highest rate (45%) compared with CT (10%) and MRI (12%), but the abnormal sites on SPECT correlated poorly with the foci on electroencephalograph (EEG). Idiopathic epilepsy with hypoperfusion on SPECT was refractory to treatment and was frequently associated with mental and/or developmental retardation. Perfusion defects on SPECT scans probably affect the development and maturation of the brain in children. (author)

  4. Idiopathic central diabetes Insipidus.

    Science.gov (United States)

    Grace, Mary; Balachandran, Venu; Menon, Sooraj

    2011-10-01

    Idiopathic central diabetes insipidus (CDI) is a rare disorder characterized clinically by polyuria and polydipsia, and an abnormal urinary concentration without any identified etiology. We report a case of central diabetes insipidus in a 60-year-old lady in the absence of secondary causes like trauma, infection, and infiltrative disorders of brain.

  5. Venous Thromboembolism and Risk of Idiopathic Interstitial Pneumonia A Nationwide Study

    DEFF Research Database (Denmark)

    Sode, Birgitte Margareta; Dahl, Morten; Nielsen, Sune Fallgaard

    2010-01-01

    Rationale: Idiopathic interstitial pneumonia is characterized by pulmonary fibrosis and high mortality. Objectives: We examined the association between ever-diagnosed venous thromboembolism and risk of incident idiopathic interstitial pneumonia. Venous thromboembolism was taken as a proxy...... Danish registries. Measurements and Main Results: Age-standardized incidence rates per 10,000 person-years for idiopathic interstitial pneumonia were higher among those ever diagnosed with venous thromboembolism (1.8; n = 158,676), pulmonary embolism (2.8; n = 70,586), and deep venous thrombosis only (1.......2; n = 88,090), than among control subjects (0.8; n = 7,260,278). Multivariate-adjusted hazard ratios for idiopathic interstitial pneumonia were 1.8 (95% confidence interval [Cl], 1.7-1.9) in those ever diagnosed with venous thromboembolism, 2.4 (95% CI, 2.3-2.6) in those ever diagnosed with pulmonary...

  6. Idiopathic thoracic transdural intravertebral spinal cord herniation

    Directory of Open Access Journals (Sweden)

    Mazda K Turel

    2017-01-01

    Full Text Available Idiopathic spinal cord herniation is a rare and often missed cause of thoracic myelopathy. The clinical presentation and radiological appearance is inconsistent and commonly confused with a dorsal arachnoid cyst and often is a misdiagnosed entity. While ventral spinal cord herniation through a dural defect has been previously described, intravertebral herniation is a distinct entity and extremely rare. We present the case of a 70-year old man with idiopathic thoracic transdural intravertebral spinal cord herniation and discuss the clinico-radiological presentation, pathophysiology and operative management along with a review the literature of this unusual entity.

  7. Idiopathic facial pain related with dental implantation

    Directory of Open Access Journals (Sweden)

    Tae-Geon Kwon

    2016-06-01

    Full Text Available Chronic pain after dental implantation is rare but difficult issue for the implant practitioner. Patients with chronic pain who had been performed previous implant surgery or related surgical intervention sometimes accompany with psychological problem and difficult to adequately manage. According to the International Classification of Headache Disorders (ICHD 3rd eds, Cepalagia 2013, painful neuropathies and other facial pains are subdivided into the 12 subcategories; 13.1. Trigeminal neuralgia; 13.2 Glossopharyngeal neuralgia; 13.3 Nervus intermedius (facial nerve neuralgia; 13.4 Occipital neuralgia; 13.5 Optic neuritis; 13.6 Headache attributed to ischaemic ocular motor nerve palsy; 13.7 Tolosa-Hunt syndrome; 13.8 Paratrigeminal oculo-sympathetic (Raeder’s syndrome; 13.9 Recurrent painful ophthalmoplegic neuropathy; 13.10 Burning Mouth Syndrome (BMS; 13.11 Persistent Idiopathic Facial Pain (PIFP; 13.12 Central neuropathic pain. Chronic orofacial pain after dental implant surgery can be largely into the two main categories that can be frequently encountered in clinical basis ; 1 Neuropathic pain, 2 Idiopathic pain. If there is no direct evidence of the nerve injury related with the implant surgery, the clinician need to consider the central cause of pain instead of the peripheral cause of the pain. There might be several possibilities; 1 Anaesthesia dolorosa, 2 Central post-stroke pain, 3 Facial pain attributed to multiple sclerosis, 4 Persistent idiopathic facial pain (PIFP, 5 Burning mouth syndrome. In this presentation, Persistent idiopathic facial pain (PIFP, the disease entity that can be frequently encountered in the clinic would be discussed. Persistent idiopathic facial pain (PIFP can be defined as “persistent facial and/or oral pain, with varying presentations but recurring daily for more than 2 hours per day over more than 3 months, in the absence of clinical neurological deficit”. ‘Atypical’ pain is a diagnosis of

  8. Idiopathic megarectum in children.

    Science.gov (United States)

    Godbole, P P; Pinfield, A; Stringer, M D

    2001-02-01

    There is scant information about the management of idiopathic megarectum in childhood. Children with idiopathic megarectum referred to a single institution between 1994 and 1998 were identified prospectively. Those with Hirschsprung's disease or an anorectal malformation were excluded. The remaining patient group, 22 boys and 7 girls, had a median age of 8.0 years (range 3.5-14.0 y). Median duration of symptoms prior to referral was 2.0 years (range 0.4-11 y). Chronic soiling was the dominant complaint in 28/29 (97%) cases. 23 children had received regular stimulant laxatives for periods ranging from 1 month to 11 years, and 9 children had been treated with regular enemas. The degree of megarectum assessed by both abdominal palpation and plain radiography was: grade 1 (below umbilical level) n=6; grade 2 (at umbilical level) n=15; and grade 3 (above umbilical level) n=8. Hirschsprung's disease was specifically excluded by rectal biopsy in all cases and no patient had evidence of spinal dysraphism. Three boys with massive megarectums and intractable symptoms were treated by a staged Duhamel sigmoid pull-through with excellent functional results. Fifteen patients (52%) were treated by a single manual evacuation under general anaesthesia followed by a daily Bisacodyl 5-10 mg suppository. After a median follow-up of 16 months, 13 continue to respond well with a daily bowel action and no soiling (4 of the 13 have discontinued treatment and remain well). The remaining 11 patients (38%) have continued conventional treatment with oral laxatives but with limited success. Idiopathic megarectum is poorly described in children. It is more common in boys and is often resistant to laxative therapy alone. After appropriate preparation, treatment with stimulant suppositories can be effective. Surgery has a valuable role in selected patients with a massive megarectum.

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

    Directory of Open Access Journals (Sweden)

    Diego Rey

    2015-02-01

    Full Text Available 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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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 (Pthrombus (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.

  12. Nasal bacterial colonization in cases of idiopathic epistaxis in children.

    Science.gov (United States)

    Kamble, Payal; Saxena, Sonal; Kumar, Sunil

    2015-11-01

    To evaluate the role of nasal bacterial colonization in cases of idiopathic epistaxis in children. A descriptive, hospital based, observational study in our hospital was conducted on total 112 pediatric patients in the age group 4-16 years. Group A (control): 56 patients with no epistaxis; Group B (epistaxis): 56 patients with idiopathic epistaxis. A swab for microbiological evaluation was taken from the anterior nasal cavity of each child. A highly significant association between nasal colonization with pathological Staphylococcus aureus and idiopathic epistaxis was found. The presence of pathological S. aureus colonization in the anterior nasal cavity was also associated with statistically significant number of crusting and presence of dilated blood vessels on the anterior nasal septum of children in epistaxis group. Nasal bacterial colonization with S. aureus leads to a sequence of pathological events i.e. low grade inflammation, crusting and new vessel formation. This leads to irritation in nasal cavity resulting in digital trauma and subsequently epistaxis and thus it plays an important role in causing idiopathic epistaxis in children. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  13. Antioxidant therapy in idiopathic oligoasthenoteratozoospermia

    Directory of Open Access Journals (Sweden)

    Ahmad Majzoub

    2017-01-01

    Conclusion: Additional randomized controlled studies are required to confirm the efficacy and safety of antioxidant supplementation in the medical treatment of idiopathic male infertility as well as the dosage required to improve semen parameters, fertilization rates, and pregnancy outcomes in iOAT.

  14. A survey of foot problems in juvenile idiopathic arthritis.

    Science.gov (United States)

    Hendry, G; Gardner-Medwin, J; Watt, G F; Woodburn, J

    2008-12-01

    Evidence suggests that foot problems are common in juvenile idiopathic arthritis (JIA), with prevalence estimates over 90%. The aim of this survey was to describe foot-related impairment and disability associated with JIA and foot-care provision in patients managed under modern treatment paradigms, including disease-modifying anti-rheumatic drugs (DMARDs) and biologic therapies. The Juvenile Arthritis Foot Disability Index (JAFI), Child Health Assessment Questionnaire (CHAQ), and pain visual analogue scale (VAS) were recorded in 30 consecutive established JIA patients attending routine outpatient clinics. Foot deformity score, active/limited joint counts, walking speed, double-support time (s) (DS) and step length symmetry index % (SI) were also measured. Foot-care provision in the preceding 12 months was determined from medical records. Sixty-three per cent of children reported some foot impairment, with a median (range) JAFI subscale score of 1 (0-3); 53% reported foot-related activity limitation, with a JAFI subscale score of 1 (0-4); and 60% reported participation restriction, with a JAFI subscale score of 1 (0-3). Other reported variables were CHAQ 0.38 (0-2), VAS pain 22 (0-79), foot deformity 6 (0-20), active joints 0 (0-7), limited joints 0 (0-31), walking speed 1.09 m/s (0.84-1.38 m/s), DS 0.22 s (0.08-0.26 s) and SI +/-4.0% (+/-0.2-+/-31.0%). A total of 23/30 medical records were reviewed and 15/23 children had received DMARDS, 8/23 biologic agents and 20/23 multiple intra-articular corticosteroid injections. Ten children received specialist podiatry care comprising footwear advice, orthotic therapy and silicone digital splints together with intrinsic muscle strengthening exercises. Despite frequent use of DMARD/biologic therapy and specialist podiatry-led foot care, foot-related impairment and disability persists in some children with JIA.

  15. Idiopathic gingival fibromatosis

    Directory of Open Access Journals (Sweden)

    Sujata Rath

    2011-01-01

    This article presents a case report of a 14-year-old female patient with idiopathic gingival fibromatosis in the maxillary region with radiographic feature of congenitally missing maxillary permanent left lateral incisor, maxillary left and right permanent canine, mandibular right second premolar, all third molars along with overretained primary maxillary left lateral incisor and primary mandibular second molar. The treatment rendered in this patient comprised of surgical excision of the hyperplasia under general anesthesia.

  16. Epidemiology of idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    Ley B

    2013-11-01

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

  17. Milia-like idiopathic calcinosis cutis in a child with Down syndrome.

    Science.gov (United States)

    Kumar, Piyush; Savant, Sushil S; Nimisha, Esther; Das, Anupam; Debbarman, Panchami

    2016-05-15

    Idiopathic calcinosis cutis refers to progressive deposition of crystals of calcium phosphate in the skin and other areas of the body, in the absence of any inciting factor. Idiopathic calcinosis cutis may sometimes take the form of small, milia-like lesions. Most commonly, such milia like lesions are seen in the setting of Down syndrome. Herein, we report a 5-year-old girl with multiple asymptomatic discrete milia-like firm papules distributed over the face and extremities. A diagnosis of milia-like idiopathic calcinosis cutis associated with Down Syndrome was provisionally made and was confirmed by histopathology and karyotyping.

  18. 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).

    Science.gov (United States)

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

    2018-03-01

    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

  19. A new corrective technique for adolescent idiopathic scoliosis (Ucar′s convex rod rotation

    Directory of Open Access Journals (Sweden)

    Bekir Yavuz Ucar

    2014-01-01

    Full Text Available Study Design: Prospective single-center study. Objective: To analyze the efficacy and safety of a new technique of global vertebral correction with convex rod rotation performed on the patients with adolescent idiopathic scoliosis. Summary of Background Data: Surgical goal is to obtain an optimal curve correction in scoliosis surgery. There are various correction techniques. This report describes a new technique of global vertebral correction with convex rod rotation. Materials and Methods: A total of 12 consecutive patients with Lenke type I adolescent idiopathic scoliosis and managed by convex rod rotation technique between years 2012 and 2013 having more than 1 year follow-up were included. Mean age was 14.5 (range = 13-17 years years at the time of operation. The hospital charts were reviewed for demographic data. Measurements of curve magnitude and balance were made on 36-inch standing anteroposterior and lateral radiographs taken before surgery and at most recent follow up to assess deformity correction, spinal balance, and complications related to the instrumentation. Results: Preoperative coronal plane major curve of 62° (range = 50°-72° with flexibility of less than 30% was corrected to 11.5°(range = 10°-14° showing a 81% scoliosis correction at the final follow-up. Coronal imbalance was improved 72% at the most recent follow-up assessment. No complications were found. Conclusion: The new technique of global vertebral correction with Ucar′s convex rod rotation is an effective technique. This method is a vertebral rotation procedure from convex side and it allows to put screws easily to the concave side.

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

    Science.gov (United States)

    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.

  1. A Study of SPINK 1 Mutation and Other Clinical Correlates in Idiopathic Chronic Pancreatitis

    OpenAIRE

    Shiran Shetty; Venkatakrishnan Leelakrishnan; Krishnaveni; S Ramalingam; Seethalakshmi

    2016-01-01

    Chronic pancreatitis is labelled as idiopathic when no identifiable factors are found. The identifications of genetic mutations associated with pancreatitis have provided opportunities for identifying patients at risk for idiopathic pancreatitis. The aim of this study was to study the demographic, clinical profile and assess the prevalence of genetic mutation (SPINK 1) in idiopathic chronic pancreatitis.

  2. Idiopathic incapacitates in infancy and childhood

    International Nuclear Information System (INIS)

    Crankson, Stanley J.; Al-Rabeeh, Abdulla A.; Fischer, James D.; Al-Jaddan, Saud A.

    2003-01-01

    Idiopathic intussusception is an important cause of abdominal pain, bleeding per rectum and intestinal obstruction in infancy and childhood. The main aim of this study was to undertake a retrospective review of all chidren who presented with idiopathic intussusception over a 17-year period. The medical record of children who presented idiopathic intussusception from January 1984 to December 2000 at King Fahad National Guard Hospital ,Riyadh, Kingdom of Saudi Arabia were reviewed. The data obtained included age, sex clincal presentation, diagonstic investigations, mode of treatment, length of hospital stay and results. Thirty-three chidren (21 male, 12female) presented with 37 episodes of intussusception.Their mean age was 8.4 months (range 5 hours to 36 months ). Clinical features included rectal bleeding (81%), vomiting (78%), abdominal colic/pain (65%) and abdominal mass (62%) .All cases were ileocolic intussusception with no leading point. Barium enema was attempted in 36 cases with success in 20 (56%). Laparotomy was required in 16 csaes, manual reduction being successful in 11(30%) and 6 (16%) had bowel resection. At surgery, after attempted Barium reduction, 9(56%) cases had the intussusception already reduced to cecum. Seventy percent of the cases presented within 24 hours of onset of the symptoms.The 4 recurrences in 3 children had successful enema reduction. There was no mortality but 3 operative cases of this type required late surgery for adhesive intestinal obstruction including one requiring bowel resection. Idiopathic indussusception commonly presents as an ileo-colic type but is uncommon in our institution. The clinical features are classical, rectal bleeding being the most common. The majority presented within 24 hours of onset of symptoms and Barium enema reduction was sucessfull in 20 out of 36 cases in which it was attempted . Since most intussusception were already in the cecum at surgey after failed enema reduction could be considerd in stable

  3. Genetics Home Reference: idiopathic inflammatory myopathy

    Science.gov (United States)

    ... stumble while walking and find it difficult to grasp items. As in dermatomyositis and polymyositis, swallowing can ... and development? More about Mutations and Health Inheritance Pattern Most cases of idiopathic inflammatory myopathy are sporadic, ...

  4. Radiographic and MRI characteristics of lumbar disseminated idiopathic spinal hyperostosis and spondylosis deformans in dogs.

    Science.gov (United States)

    Togni, A; Kranenburg, H J C; Morgan, J P; Steffen, F

    2014-07-01

    To evaluate clinical signs, describe lesions and differences in the magnetic resonance imaging appearance of spinal new bone formations classified as disseminated idiopathic spinal hyperostosis and/or spondylosis deformans on radiographs and compare degeneration status of the intervertebral discs using the Pfirrmann scale. Retrospective analysis of 18 dogs presented with spinal disorders using information from radiographic and magnetic resonance imaging examinations. All dogs were found to be affected with both disseminated idiopathic spinal hyperostosis and spondylosis deformans. Neurological signs due to foraminal stenosis associated with disseminated idiopathic spinal hyperostosis were found in two dogs. Spondylosis deformans was associated with foraminal stenosis and/or disc protrusion in 15 cases. The Pfirrmann score on magnetic resonance imaging was significantly higher in spondylosis deformans compared with disseminated idiopathic spinal hyperostosis and signal intensity of new bone due to disseminated idiopathic spinal hyperostosis was significantly higher compared to spondylosis deformans. Differences between disseminated idiopathic spinal hyperostosis and spondylosis deformans found on magnetic resonance imaging contribute to an increased differentiation between the two entities. Clinically relevant lesions in association with disseminated idiopathic spinal hyperostosis were rare compared to those seen with spondylosis deformans. © 2014 British Small Animal Veterinary Association.

  5. Reliability and concurrent validity of postural asymmetry measurement in adolescent idiopathic scoliosis.

    Science.gov (United States)

    Prowse, Ashleigh; Aslaksen, Berit; Kierkegaard, Marie; Furness, James; Gerdhem, Paul; Abbott, Allan

    2017-01-18

    To investigate the reliability and concurrent validity of the Baseline ® Body Level/Scoliosis meter for adolescent idiopathic scoliosis postural assessment in three anatomical planes. This is an observational reliability and concurrent validity study of adolescent referrals to the Orthopaedic department for scoliosis screening at Karolinska University Hospital, Stockholm, Sweden between March-May 2012. A total of 31 adolescents with idiopathic scoliosis (13.6 ± 0.6 years old) of mild-moderate curvatures (25° ± 12°) were consecutively recruited. Measurement of cervical, thoracic and lumbar curvatures, pelvic and shoulder tilt, and axial thoracic rotation (ATR) were performed by two trained physiotherapists in one day. The intraclass correlation coefficient (ICC) was used to determine the inter-examiner reliability (ICC2,1) and the intra-rater reliability (ICC3,3) of the Baseline ® Body Level/Scoliosis meter. Spearman's correlation analyses were used to estimate concurrent validity between the Baseline ® Body Level/Scoliosis meter and Gold Standard Cobb angles from radiographs and the Orthopaedic Systems Inc. Scoliometer. There was excellent reliability between examiners for thoracic kyphosis (ICC2,1 = 0.94), ATR (ICC2,1 = 0.92) and lumbar lordosis (ICC2,1 = 0.79). There was adequate reliability between examiners for cervical lordosis (ICC2,1 = 0.51), however poor reliability for pelvic and shoulder tilt. Both devices were reproducible in the measurement of ATR when repeated by one examiner (ICC3,3 0.98-1.00). The device had a good correlation with the Scoliometer (rho = 0.78). When compared with Cobb angle from radiographs, there was a moderate correlation for ATR (rho = 0.627). The Baseline ® Body Level/Scoliosis meter provides reliable transverse and sagittal cervical, thoracic and lumbar measurements and valid transverse plan measurements of mild-moderate scoliosis deformity.

  6. Giant scrotal elephantiasis: an idiopathic case.

    Science.gov (United States)

    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.

  7. The role of the intervertebral disc in correction of scoliotic curves. A theoretical model of idiopathic scoliosis pathogenesis.

    Science.gov (United States)

    Grivas, T B; Vasiliadis, E S; Rodopoulos, G; Bardakos, N

    2008-01-01

    Wedging of the scoliotic inter-vertebral disc (IVD) was previously reported as a contributory factor for progression of idiopathic scoliotic (IS) curves. The present study introduces a theoretical model of IVD's role in IS pathogenesis and examines if, by reversing IVD wedging with conservative treatment (full- and night-time braces and exercises) or fusionless IS surgery with staples, we can correct the deformity of the immature spine. The proposed model implies the role of the diurnal variation and the asymmetric water distribution in the scoliotic IVD and the subsequent alteration of the mechanical environment of the adjacent vertebral growth plates. Modulation of the IVD by applying corrective forces on the scoliotic curve restores a close-to-normal force application on the vertebral growth plates through the Hueter-Volkmann principle and consequently prevents curve progression. The forces are now transmitted evenly to the growth plate and increase the rate of proliferation of chondrocytes at the corrected pressure side, the concave. Application of appropriately directed forces, ideally opposite to the apex of the deformity, likely leads to optimal correction. The wedging of the elastic IVD in the immature scoliotic spine could be reversed by application of corrective forces on it. Reversal of IVD wedging is thus amended into a "corrective", rather than "progressive", factor of the deformity. Through the proposed model, treatment of progressive IS with braces, exercises and fusionless surgery by anterior stapling could be effective.

  8. MRI of idiopathic orbital inflammation and lymphoid disease with lesions in extraocular muscle

    International Nuclear Information System (INIS)

    Matsuda, Chiharu; Kotake, Fumio; Kawanishi, Masayuki; Saito, Kazuhiro; Abe, Kimihiko

    2004-01-01

    Of the disorders accompanied by hypertrophy of the extraocular muscles, differentiating between idiopathic orbital inflammation and malignant lymphoma is difficult but important to treatment and prognosis. In this study using MRI, shape, signal intensity, and enhancement effects were compared between idiopathic orbital inflammation and lymphoproliferative lesions. The subjects were 27 patients (8 with idiopathic orbital inflammation, 1 with reactive lymphoid hyperplasia, 3 with atypical lymphoid hyperplasia, and 15 with malignant lymphoma) and 10 normal controls. The evaluation items were: thickness of extraocular muscles, number of extraocular muscles involved signal intensity of extraocular muscles, and enhancement effects on extraocular muscles. When compared to control subjects (p<0.05) the attachment portion of extraocular muscles were significantly thicker in the patients with idiopathic orbital inflammation, atypical lymphoid hyperplasia, or malignant lymphoma; the most marked hypertrophy was observed in patients with malignant lymphoma. The number of extraocular muscles involved was 1.5 (mean) in the patients with idiopathic orbital inflammation, 1 in the patient with reactive lymphoid hyperplasia, 1.7 (mean) in the patients with atypical lymphoid hyperplasia, and 5.1 (mean) in those with malignant lymphoma. The signal intensity ratio on T1W-images did not significantly differ between the patients and controls for all the disorders investigated. Signal intensity ratio on T2W-images significantly differed between patients with atypical lymphoid hyperplasia or malignant lymphoma and the controls (p<0.05) but not between patients with idiopathic orbital inflammation and controls. Signal intensity ratio after contrast enhancement differed significantly only between patients with idiopathic orbital inflammation and controls (p<0.05). (author)

  9. Idiopathic pulmonary fibrosis. A rare cause of scintigraphic ventilation-perfusion mismatch

    International Nuclear Information System (INIS)

    Pochis, W.T.; Krasnow, A.Z.; Collier, B.D.; Mewissen, M.W.; Almagro, U.A.; Hellman, R.S.; Isitman, A.T.

    1990-01-01

    A case of idiopathic pulmonary fibrosis with multiple areas of mismatch on ventilation-perfusion lung imaging in the absence of pulmonary embolism is presented. Idiopathic pulmonary fibrosis is one of the few nonembolic diseases producing a pulmonary ventilation-perfusion mismatch. In this condition, chest radiographs may not detect the full extent of disease, and xenon-133 ventilation imaging may be relatively insensitive to morbid changes in small airways. Thus, when examining patients with idiopathic pulmonary fibrosis, one should be aware that abnormal perfusion imaging patterns without matching ventilation abnormalities are not always due to embolism. In this setting, contrast pulmonary angiography is often needed for accurate differential diagnosis

  10. Idiopathic pulmonary fibrosis. A rare cause of scintigraphic ventilation-perfusion mismatch

    Energy Technology Data Exchange (ETDEWEB)

    Pochis, W.T.; Krasnow, A.Z.; Collier, B.D.; Mewissen, M.W.; Almagro, U.A.; Hellman, R.S.; Isitman, A.T. (Medical College of Wisconsin, Milwaukee (USA))

    1990-05-01

    A case of idiopathic pulmonary fibrosis with multiple areas of mismatch on ventilation-perfusion lung imaging in the absence of pulmonary embolism is presented. Idiopathic pulmonary fibrosis is one of the few nonembolic diseases producing a pulmonary ventilation-perfusion mismatch. In this condition, chest radiographs may not detect the full extent of disease, and xenon-133 ventilation imaging may be relatively insensitive to morbid changes in small airways. Thus, when examining patients with idiopathic pulmonary fibrosis, one should be aware that abnormal perfusion imaging patterns without matching ventilation abnormalities are not always due to embolism. In this setting, contrast pulmonary angiography is often needed for accurate differential diagnosis.

  11. A CASE OF IDIOPATHIC PULMONARY ARTERIAL HYPERTENSION IN MALE

    OpenAIRE

    Poongavanam Paranthaman; Ramani Bala Subra Manian; Thenrajan Balaji; Jayakrishnan Jayakumar; Govindaraj Ranjani

    2016-01-01

    Primary Pulmonary Hypertension is a rare disease occurring in 1-2 per million population. It is 2-4 times more common in female. Idiopathic or primary pulmonary hypertension is defined as a disorder with no identifiable cause in which resting mean pulmonary artery pressure in adults is above 25 mmHg and 30 mmHg with exercise. Idiopathic or primary pulmonary hypertension is diagnosed after ruling out all the possible secondary causes of pulmonary hypertension. We are presenting a ...

  12. Debate: idiopathic short stature should be treated with growth hormone.

    Science.gov (United States)

    Ambler, Geoffrey R; Fairchild, Jan; Wilkinson, Dominic J C

    2013-03-01

    In this paper we outline the case for and against the treatment of idiopathic short stature with growth hormone. Drs Ambler and Fairchild argue that many of those with 'idiopathic' short stature are not 'short, normal children' and will ultimately receive molecular diagnoses. They also argue that there is a subset of children who suffer negative psychosocial consequences of their stature for whom growth hormone therapy is effective. Growth hormone has a very good safety record and is likely to be as cost-effective in idiopathic short-stature as in some other conditions that are currently funded. Dr Wilkinson counters that short stature is not associated with physical or psychological illness, and that there is no evidence that growth hormone improves psychological or physical wellbeing. Moreover, growth hormone for idiopathic short stature represents a form of enhancement rather than treatment, and is not a fair use of resources. Socially mediated disadvantage should be treated by attention to prejudice and not by hormone treatment. © 2012 The Authors. Journal of Paediatrics and Child Health © 2012 Paediatrics and Child Health Division (Royal Australasian College of Physicians).

  13. Physiotherapeutic scoliosis-specific exercises for adolescents with idiopathic scoliosis.

    Science.gov (United States)

    Bettany-Saltikov, J; Parent, E; Romano, M; Villagrasa, M; Negrini, S

    2014-02-01

    The use of exercises for the treatment of Adolescents with Idiopathic Scoliosis is controversial. Whilst exercises are routinely used in a number of central and southern European countries, most centres in the rest of the world (mainly in Anglo-Saxon countries), do not advocate its use. One of the reasons for this is that many health care professionals are usually not conversant with the differences between generalised physiotherapy exercises and physiotherapeutic scoliosis-specific exercises (PSSE): while the former are generic exercises usually consisting of low-impact stretching and strengthening activities like yoga, Pilates and the Alexander technique, PSSE consist of a program of curve-specific exercise protocols which are individually adapted to a patients' curve site, magnitude and clinical characteristics. PSSEs are performed with the therapeutic aim of reducing the deformity and preventing its progression. It also aims to stabilise the improvements achieved with the ultimate goal of limiting the need for corrective braces or the necessity of surgery. This paper introduces the different 'Schools' and approaches of PSSE currently practiced (Scientific Exercise Approach to Scoliosis - SEAS, Schroth, Barcelona Scoliosis Physical Therapy School - BSPTS, Dobomed, Side Shift, Functional Individual Therapy of Scoliosis - FITS and Lyon) and discusses their commonalities and differences.

  14. MUSCLE DISORDERS IN ADOLESCENT IDIOPATHIC SCOLIOSIS: LITERATURE REVIEW

    Directory of Open Access Journals (Sweden)

    Débora Pinheiro Lédio Alves

    2016-03-01

    Full Text Available ABSTRACT Scoliosis is characterized by three-dimensional changes of the spine and is estimated to be present in 4% of the population worldwide. The most common form is the adolescent idiopathic. The purpose of this study is to identify the major muscle abnormalities found in patients with adolescent idiopathic scoliosis through a literature review. We conducted an electronic search of the national databases PubMed, Lilacs, PEDro, and EMBASE using the keywords "scoliosis", "biomechanics", "exercise", "physical therapy specialty", "idiopathic", and "muscles", from January 2003 to April 2015. The most relevant articles in English, Portuguese and Spanish were selected by title and abstract. It was also performed a manual search of the references of the selected articles. From a total of 4,319 articles, 11 were selected. We conclude that individuals with AIS have changes in the paraspinal muscles, with a difference in activation between the concave and convex sides, suggesting an increase in EMG activity on the convex side, although there is still no consensus among the authors.

  15. Idiopathic unilateral vocal-fold paralysis in the adult.

    Science.gov (United States)

    Rubin, F; Villeneuve, A; Alciato, L; Slaïm, L; Bonfils, P; Laccourreye, O

    2018-02-02

    To analyze the characteristics of adult idiopathic unilateral vocal-fold paralysis. Retrospective study of diagnostic problems, clinical data and recovery in an inception cohort of 100 adult patients with idiopathic unilateral vocal-fold paralysis (Group A) and comparison with a cohort of 211 patients with isolated non-idiopathic non-traumatic unilateral vocal-fold paralysis (Group B). Diagnostic problems were noted in 24% of cases in Group A: eight patients with concomitant common upper aerodigestive tract infection, five patients with a concomitant condition liable to induce immunodepression and 11 patients in whom a malignant tumor occurred along the path of the ipsilateral vagus and inferior laryngeal nerves or in the ipsilateral paralyzed larynx. There was no recovery of vocal-fold motion beyond 51 months after onset of paralysis. The 5-year actuarial estimate for recovery differed significantly (Pvocal-fold paralysis. In non-traumatic vocal-fold paralysis in adult patients, without recovery of vocal-fold motion, a minimum three years' regular follow-up is recommended. Copyright © 2018 Elsevier Masson SAS. All rights reserved.

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

    Science.gov (United States)

    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

  17. Alteration of placental haemostatic mechanisms in idiopathic intrauterine growth restriction

    Directory of Open Access Journals (Sweden)

    Jaime Eduardo Bernal Villegas

    2012-08-01

    Full Text Available Intrauterine growth restriction is a complication of pregnancy with a high probability of perinatal morbidity and mortality. It appears tobe caused by abnormal development of placental vasculature. Haemostatic processes are important for the development of the placenta,and an imbalance between procoagulant and anticoagulant factors has been associated with risk of intrauterine growth restriction.Objective. To evaluate coagulation abnormalities in placenta of pregnancies complicated with idiopathic intrauterine growth restriction.Materials and methods. Five placentas from pregnancies with idiopathic intrauterine growth restriction were compared to 19 controls.We performed gross and histological examination of the placenta. Analysis was made of both mRNA expression by real-time PCRand protein by ELISA of tissue factor and thrombomodulin in placental tissue. Results. Results based on histological evaluation wereconsistent with an increased prothrombotic state in placentas from pregnancies with idiopathic intrauterine growth restriction, andthrombosis of chorionic vessels was the most important finding. The study showed an increased expression of tissue factor protein(p=0.0411 and an increase in the ratio of tissue factor/thrombomodulin mRNA (p=0.0411 and protein (p=0.0215 in placentas frompregnancies with idiopathic intrauterine growth restriction. There were no statistically significant differences neither between cases andcontrols in the mRNA levels of tissue factor or thrombomodulin nor at the protein level of thrombomodulin. Conclusion. Evidence ofalteration of local haemostatic mechanisms at the level of the placenta, including abnormal expression of tissue factor and tissue factor/thrombomodulin ratio, in pregnancies that occur with idiopathic intrauterine growth restriction is presented.

  18. Idiopathic pulmonary fibrosis: evolving concepts.

    Science.gov (United States)

    Ryu, Jay H; Moua, Teng; Daniels, Craig E; Hartman, Thomas E; Yi, Eunhee S; Utz, James P; Limper, Andrew H

    2014-08-01

    Idiopathic pulmonary fibrosis (IPF) occurs predominantly in middle-aged and older adults and accounts for 20% to 30% of interstitial lung diseases. It is usually progressive, resulting in respiratory failure and death. Diagnostic criteria for IPF have evolved over the years, and IPF is currently defined as a disease characterized by the histopathologic pattern of usual interstitial pneumonia occurring in the absence of an identifiable cause of lung injury. Understanding of the pathogenesis of IPF has shifted away from chronic inflammation and toward dysregulated fibroproliferative repair in response to alveolar epithelial injury. Idiopathic pulmonary fibrosis is likely a heterogeneous disorder caused by various interactions between genetic components and environmental exposures. High-resolution computed tomography can be diagnostic in the presence of typical findings such as bilateral reticular opacities associated with traction bronchiectasis/bronchiolectasis in a predominantly basal and subpleural distribution, along with subpleural honeycombing. In other circumstances, a surgical lung biopsy may be needed. The clinical course of IPF can be unpredictable and may be punctuated by acute deteriorations (acute exacerbation). Although progress continues in unraveling the mechanisms of IPF, effective therapy has remained elusive. Thus, clinicians and patients need to reach informed decisions regarding management options including lung transplant. The findings in this review were based on a literature search of PubMed using the search terms idiopathic pulmonary fibrosis and usual interstitial pneumonia, limited to human studies in the English language published from January 1, 2000, through December 31, 2013, and supplemented by key references published before the year 2000. Copyright © 2014 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved.

  19. Idiopathic intracranial hypertension

    DEFF Research Database (Denmark)

    Yri, Hanne M; Jensen, Rigmor H

    2015-01-01

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

  20. Genetics Home Reference: juvenile idiopathic arthritis

    Science.gov (United States)

    ... disease to fight microbial invaders and facilitate tissue repair. Normally, the body stops the inflammatory response after healing is complete to prevent damage to its own cells and tissues. In people with juvenile idiopathic arthritis , the inflammatory ...

  1. Idiopathic retroperitoneal fibrosis involving a unilateral renal sinus: A case report and literature review

    International Nuclear Information System (INIS)

    Lee, Seul Bi; Yoon, Jung Hee; Kim, Seung Ho; Lee, Ye Daum; Kim, Suk Jung; Lim, Yun Jung; Jung, Hyun Kyung; Lee, Jin Soo

    2016-01-01

    Idiopathic retroperitoneal fibrosis (RPF) is a rare disease entity and its etiology is uncertain. We report two similar cases which showed an uncommon presentation of idiopathic RPF. A 66-year-old woman and an 80-year-old man presented with incidental findings of left renal pelvic mass-like lesions. Computed tomography revealed a soft tissue density mass replacing the left renal pelvis, which was suspicious for renal pelvic cancer, and the diagnosis of idiopathic RPF was surgically confirmed. To the best of our knowledge, a few cases of idiopathic RPF presenting with features of a localized unilateral renal pelvic mass mimicking renal pelvic cancer have been reported

  2. Idiopathic retroperitoneal fibrosis involving a unilateral renal sinus: A case report and literature review

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Seul Bi; Yoon, Jung Hee; Kim, Seung Ho; Lee, Ye Daum; Kim, Suk Jung; Lim, Yun Jung; Jung, Hyun Kyung; Lee, Jin Soo [Dept. of Radiology, Haeundae Paik Hospital, Inje University College of Medicine, Busan (Korea, Republic of)

    2016-06-15

    Idiopathic retroperitoneal fibrosis (RPF) is a rare disease entity and its etiology is uncertain. We report two similar cases which showed an uncommon presentation of idiopathic RPF. A 66-year-old woman and an 80-year-old man presented with incidental findings of left renal pelvic mass-like lesions. Computed tomography revealed a soft tissue density mass replacing the left renal pelvis, which was suspicious for renal pelvic cancer, and the diagnosis of idiopathic RPF was surgically confirmed. To the best of our knowledge, a few cases of idiopathic RPF presenting with features of a localized unilateral renal pelvic mass mimicking renal pelvic cancer have been reported.

  3. Interventional Radiologic Treatment for Idiopathic Portal Hypertension

    International Nuclear Information System (INIS)

    Hirota, Shozo; Ichikawa, Satoshi; Matsumoto, Shinichi; Motohara, Tomofumi; Fukuda, Tetsuya; Yoshikawa, Takeshi

    1999-01-01

    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: In 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

  4. Neurodevelopment in preschool idiopathic toe-walkers.

    Science.gov (United States)

    Martín-Casas, P; Ballestero-Pérez, R; Meneses-Monroy, A; Beneit-Montesinos, J V; Atín-Arratibel, M A; Portellano-Pérez, J A

    2017-09-01

    Idiopathic toe walking, a differential diagnosis for neurological and orthopaedic disorders, has been associated with neurodevelopmental alterations. Neurodevelopmental assessment at early ages using specific tests may improve management and follow-up of these patients. The aim of our study is to analyse the neurodevelopmental characteristics of preschool idiopathic toe-walkers (ITW) by comparing them to a control group. Our descriptive cross-sectional study compared possible risk factors, neurodevelopmental characteristics, and scores on the Child Neuropsychological Maturity Questionnaire (CUMANIN) between a group of 56 ITWs aged 3 to 6 and a control group including 40 children. The proportion of males was significantly higher in the ITW group (P=.008). The percentage of patients with a family history (P=.000) and biological risk factors during the perinatal period (P=.032) was also higher in this group. According to the parents' reports, motor coordination in ITWs was significantly poorer (59%; P=.009). ITWs scored significantly lower on CUMANIN subscales of psychomotricity (=0,001) and memory (P=.001), as well as in verbal development (P=.000), non-verbal development (P=.026), and overall development (P=.004). Foot preference was less marked in the ITW group (P=.047). The neurodevelopmental characteristics of our sample suggest that idiopathic toe walking is a marker of neurodevelopmental impairment. However, further studies are necessary to confirm these findings. Copyright © 2016 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.

  5. Fahr's syndrome - Idiopathic Bilateral Striopallidodentate Calcinosis ...

    African Journals Online (AJOL)

    Log in or Register to get access to full text downloads. ... Fehr's disease, also known as Idiopathic Calcification of the Basal Ganglia (ICBG) or ... ferrocalcinosis (and many others), is a rare sporadic or familial neurological disorder whose ...

  6. Idiopathic noncirrhotic portal hypertension: current perspectives.

    Science.gov (United States)

    Riggio, Oliviero; Gioia, Stefania; Pentassuglio, Ilaria; Nicoletti, Valeria; Valente, Michele; d'Amati, Giulia

    2016-01-01

    The term idiopathic noncirrhotic portal hypertension (INCPH) has been recently proposed to replace terms, such as hepatoportal sclerosis, idiopathic portal hypertension, incomplete septal cirrhosis, and nodular regenerative hyperplasia, used to describe patients with a hepatic presinusoidal cause of portal hypertension of unknown etiology, characterized by features of portal hypertension (esophageal varices, nonmalignant ascites, porto-venous collaterals), splenomegaly, patent portal, and hepatic veins and no clinical and histological signs of cirrhosis. Physicians should learn to look for this condition in a number of clinical settings, including cryptogenic cirrhosis, a disease known to be associated with INCPH, drug administration, and even chronic alterations in liver function tests. Once INCPH is clinically suspected, liver histology becomes mandatory for the correct diagnosis. However, pathologists should be familiar with the histological features of INCPH, especially in cases in which histology is not only requested to exclude liver cirrhosis.

  7. Idiopathic granulomatous mastitis masquerading as carcinoma of the breast: a case report and review of the literature

    OpenAIRE

    Tuli, Richard; O'Hara, Brian J; Hines, Janet; Rosenberg, Anne L

    2007-01-01

    Background Idiopathic granulomatous mastitis is an uncommon, benign entity with a diagnosis of exclusion. The typical clinical presentation of idiopathic granulomatous mastitis often mimics infection or malignancy. As a result, histopathological confirmation of idiopathic granulomatous mastitis combined with exclusion of infection, malignancy and other causes of granulomatous disease is absolutely necessary. Case Presentation We present a case of a young woman with idiopathic granulomatous ma...

  8. Pirfenidone treatment in idiopathic pulmonary fibrosis

    DEFF Research Database (Denmark)

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

    2016-01-01

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

  9. Gain of function Nanu1.7 mutations in idiopathic small fiber neuropathy.

    NARCIS (Netherlands)

    Faber, C.G.; Hoeijmakers, J.G.; Ahn, H.S.; Cheng, X.; Han, C.; Choi, J.S.; Estacion, M.; Lauria, G.; Vanhoutte, E.K.; Gerrits, M.M.; Dib-Hajj, S.; Drenth, J.P.H.; Waxman, S.G.; Merkies, I.S.

    2012-01-01

    OBJECTIVE: Small nerve fiber neuropathy (SFN) often occurs without apparent cause, but no systematic genetic studies have been performed in patients with idiopathic SFN (I-SFN). We sought to identify a genetic basis for I-SFN by screening patients with biopsy-confirmed idiopathic SFN for mutations

  10. Psychological aspects of idiopathic scoliosis: the specificity of the mother-daughter relationship

    Directory of Open Access Journals (Sweden)

    Galina V. Pyatakova

    2016-12-01

    Conclusion: General and specific characteristics of the mother-daughter relationship in families of adolescent girls with idiopathic scoliosis and families of healthy adolescent girls were revealed. In the context of complex surgical treatment, preventive measures are necessary to address psychological difficulties in adolescent patients with idiopathic scoliosis.

  11. EFFICACY OF ETANERCEPT IN TREATMENT OF VARIOUS TYPES OF JUVENILE IDIOPATHIC ARTHRITIS

    Directory of Open Access Journals (Sweden)

    O. Yu. Konopel'ko

    2013-01-01

    Full Text Available Aim: to assess efficacy and safety of etanercept in treatment of various types of juvenile idiopathic arthritis in children under conditions of real clinical practice. Patients and methods: 52 children were included into the study, among them 16 were with systemic and 36 with juvenile idiopathic arthritis without extra-articular involvement. Results: etanercept treatment was the most efficient in patients with systemic juvenile idiopathic arthritis without extra-articular involvement. In 6 and 12 months of the treatment 50 and 70% improvement according to the ACRpedi criteria were established in 31/36 (86% and 28/36 (78% of the patients, respectively. In 24 months in 5 (29% of 17 children remained in the study remission stage of the diseases was confirmed. Conclusions: etanercept treatment was not associated with significant unfavorable effects, which allows to recommend this drug for treatment of juvenile idiopathic arthritis without extra-articular involvent and resistant to standard anti-rheumatic therapy.

  12. Asynchronous neuro-osseous growth in adolescent idiopathic scoliosis - MRI-based research

    International Nuclear Information System (INIS)

    Chu, Winnie C.W.; Rasalkar, Darshana D.; Cheng, Jack C.Y.

    2011-01-01

    Adolescent idiopathic scoliosis (AIS) is a common worldwide problem and has been treated for many decades; however, there still remain uncertain areas about this disorder. Its involvement and impact on different parts of the human body remain underestimated due to lack of technology in imaging for objective assessment in the past. The advances in imaging technique and image analysis technology have provided a novel approach for the understanding of the phenotypic presentation of neuro-osseous changes in AIS patients as compared with normal controls. This review is the summary of morphological assessment of the skeletal and nervous systems in girls with AIS based on MRI. Girls with AIS are found to have morphological differences in multiple areas including the vertebral column, spinal cord, skull and brain when compared with age- and sex-matched normal controls. Taken together, the abnormalities in the skeletal system and nervous system of AIS are likely to be inter-related and reflect a systemic process of asynchronous neuro-osseous growth. The current knowledge about the anatomical changes in AIS has important implications with respect to the understanding of fundamental pathomechanical processes involved in the evolution of the scoliotic deformity. (orig.)

  13. Asynchronous neuro-osseous growth in adolescent idiopathic scoliosis - MRI-based research

    Energy Technology Data Exchange (ETDEWEB)

    Chu, Winnie C.W.; Rasalkar, Darshana D. [The Chinese University of Hong Kong, Department of Diagnostic Radiology and Organ Imaging, Hong Kong, SAR (China); Cheng, Jack C.Y. [The Chinese University of Hong Kong, Department of Orthopaedics and Traumatology, Hong Kong, SAR (China)

    2011-09-15

    Adolescent idiopathic scoliosis (AIS) is a common worldwide problem and has been treated for many decades; however, there still remain uncertain areas about this disorder. Its involvement and impact on different parts of the human body remain underestimated due to lack of technology in imaging for objective assessment in the past. The advances in imaging technique and image analysis technology have provided a novel approach for the understanding of the phenotypic presentation of neuro-osseous changes in AIS patients as compared with normal controls. This review is the summary of morphological assessment of the skeletal and nervous systems in girls with AIS based on MRI. Girls with AIS are found to have morphological differences in multiple areas including the vertebral column, spinal cord, skull and brain when compared with age- and sex-matched normal controls. Taken together, the abnormalities in the skeletal system and nervous system of AIS are likely to be inter-related and reflect a systemic process of asynchronous neuro-osseous growth. The current knowledge about the anatomical changes in AIS has important implications with respect to the understanding of fundamental pathomechanical processes involved in the evolution of the scoliotic deformity. (orig.)

  14. A novel deformation mechanism for superplastic deformation

    Energy Technology Data Exchange (ETDEWEB)

    Muto, H.; Sakai, M. (Toyohashi Univ. of Technology (Japan). Dept. of Materials Science)

    1999-01-01

    Uniaxial compressive creep tests with strain value up to -0.1 for a [beta]-spodumene glass ceramic are conducted at 1060 C. From the observation of microstructural changes between before and after the creep deformations, it is shown that the grain-boundary sliding takes place via cooperative movement of groups of grains rather than individual grains under the large-scale-deformation. The deformation process and the surface technique used in this work are not only applicable to explain the deformation and flow of two-phase ceramics but also the superplastic deformation. (orig.) 12 refs.

  15. Bone marrow stroma in idiopathic myelofibrosis and other haematological diseases. An immunohistochemical study

    DEFF Research Database (Denmark)

    Lisse, I; Hasselbalch, H; Junker, P

    1991-01-01

    Bone marrow stroma was investigated immunohistochemically in 31 patients with haematological diseases, mainly idiopathic myelofibrosis (n = 8) and related chronic myeloproliferative disorders (n = 14). The bone marrow from patients with idiopathic myelofibrosis and some CML patients showed marked...

  16. Atherosclerosis in Juvenile Idiopathic Arthritis

    Directory of Open Access Journals (Sweden)

    Ewa Jednacz

    2012-01-01

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

  17. Auditory verbal memory and psychosocial symptoms are related in children with idiopathic epilepsy.

    Science.gov (United States)

    Schaffer, Yael; Ben Zeev, Bruria; Cohen, Roni; Shuper, Avinoam; Geva, Ronny

    2015-07-01

    Idiopathic epilepsies are considered to have relatively good prognoses and normal or near normal developmental outcomes. Nevertheless, accumulating studies demonstrate memory and psychosocial deficits in this population, and the prevalence, severity and relationships between these domains are still not well defined. We aimed to assess memory, psychosocial function, and the relationships between these two domains among children with idiopathic epilepsy syndromes using an extended neuropsychological battery and psychosocial questionnaires. Cognitive abilities, neuropsychological performance, and socioemotional behavior of 33 early adolescent children, diagnosed with idiopathic epilepsy, ages 9-14years, were assessed and compared with 27 age- and education-matched healthy controls. Compared to controls, patients with stabilized idiopathic epilepsy exhibited higher risks for short-term memory deficits (auditory verbal and visual) (pmemory deficits (plong-term memory deficits (pmemory deficits was related to severity of psychosocial symptoms among the children with epilepsy but not in the healthy controls. Results suggest that deficient auditory verbal memory may be compromising psychosocial functioning in children with idiopathic epilepsy, possibly underscoring that cognitive variables, such as auditory verbal memory, should be assessed and treated in this population to prevent secondary symptoms. Copyright © 2015 Elsevier Inc. All rights reserved.

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

    Science.gov (United States)

    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.

  19. A case of idiopathic omental hemorrhage

    Directory of Open Access Journals (Sweden)

    Toshimitsu Hosotani

    2016-05-01

    Full Text Available With the exception of trauma, intraperitoneal hemorrhage in young women is caused by the high frequency of ectopic pregnancy and ovarian bleeding. Here, we describe a case of idiopathic omental hemorrhage, which is a rare cause of intraperitoneal hemorrhage. Intraperitoneal hemorrhage was suspected in a 38-year-old Japanese woman based on contrast-enhanced computed tomography. Her last menstrual period was 23 days prior, and ovarian bleeding was considered based on bloody ascites revealed by culdocentesis. She underwent emergency surgery for hypovolemic shock. Although both ovaries were of normal size and no abnormal findings were observed, we performed a partial omentectomy because multiple clots were attached only to the greater omentum. Postoperatively, no rebleeding occurred, and she was discharged 11 days after the surgery. Because she did not have a clear history of trauma and underlying disease, idiopathic omental hemorrhage was diagnosed.

  20. Idiopathic burning mouth syndrome: a common treatment-refractory somatoform condition responsive to ECT.

    Science.gov (United States)

    McGirr, Alexander; Davis, Lindsay; Vila-Rodriguez, Fidel

    2014-04-30

    Somatic symptom disorders are common causes of disability and suffering, and can pose significant management challenges. Idiopathic burning mouth syndrome is a challenging somatic symptom disorder with relatively high prevalence, particularly among post-menopausal women. Here, we present the case of a woman with severe treatment refractory idiopathic burning mouth syndrome and comorbid major depressive disorder, who was successfully treated with bitemporal electroconvulsive therapy. This case highlights the potential effectiveness of electroconvulsive therapy in idiopathic burning mouth syndrome when other treatment options have been exhausted. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  1. Sleep, stress, neurocognitive profile and healthrelated quality of life in adolescents with idiopathic musculoskeletal pain

    Directory of Open Access Journals (Sweden)

    Juliana Molina

    2012-10-01

    Full Text Available OBJECTIVES: The aims of this study were to measure levels of sleep, stress, and depression, as well as health-related quality of life, and to assess the neurocognitive profiles in a sample of adolescents with idiopathic musculoskeletal pain. METHODS: Nineteen adolescents with idiopathic musculoskeletal pain and 20 age-matched healthy control subjects were evaluated regarding their levels of sleep and stress, as well as quality of life, and underwent neurocognitive testing. RESULTS: The sample groups consisted predominantly of females (84%, and the socioeconomic status did not differ between the two groups. In addition, the occurrence of depressive symptoms was similar between the two groups; specifically, 26% of the idiopathic musculoskeletal pain patients and 30% of the control subjects had scores indicative of depression. Teenagers in the group with idiopathic musculoskeletal pain reported poorer quality of life and sleep scores than those in the control group. Regarding stress, patients had worse scores than the control group; whereas 79% of the adolescents with idiopathic musculoskeletal pain met the criteria for a diagnosis of stress, only 35% of the adolescents in the control group met the criteria. In both groups, we observed scores that classified adolescents as being in the resistance phase (intermediate and exhaustion phase (pathological of distress. However, the idiopathic musculoskeletal pain group more frequently reported symptomatic complaints of physical and emotional distress. The neurocognitive assessment showed no significant impairments in either group. CONCLUSION: Adolescents with idiopathic musculoskeletal pain did not exhibit cognitive impairments. However, adolescents with idiopathic musculoskeletal pain did experience intermediate to advanced psychological distress and lower health-related quality of life, which may increase their risk of cognitive dysfunction in the future.

  2. The role of serial casting in early-onset scoliosis (EOS).

    Science.gov (United States)

    Baulesh, David M; Huh, Jeannie; Judkins, Timothy; Garg, Sumeet; Miller, Nancy H; Erickson, Mark A

    2012-01-01

    Serial casting has demonstrated efficacy for idiopathic early-onset scoliosis (EOS). Results of casting in nonidiopathic (syndromic and congenital) EOS patients have not previously been well described. A total of 53 patients underwent serial casting for EOS from 2005 to 2010 at a single institution. Deformity was classified as idiopathic or nonidiopathic. Diagnosis, time in cast, number of casts, use of bracing, complications, and outcomes were recorded. Radiographic measures included Cobb angle and thoracic height (T1-T12). Thoracic height velocity was calculated and compared with established norms. A total of 36 patients, 19 idiopathic and 17 nonidiopathic (14 syndromic, 3 congenital), completed cast treatment and had >6-month follow-up and were therefore included. Of those, 17% (6/36) experienced resolution of their deformity, 53% (19/26) are currently in braces, and 31% (11/36) had undergone surgery. Surgery occurred on average at age 5.6 years and was delayed by an average of 2.1 years from time of first cast. A 19% complication was observed. There was no statistical difference in the rate of resolution of deformity between idiopathic (5/19) and nonidiopathic (1/17) patients (P=0.182), although there exists a trend toward greater curve correction in idiopathic patients. Surgery occurred in fewer patients (2/19) in the idiopathic group compared with the nonidiopathic group (9/17) (P=0.006). Significant improvements in Cobb angle was observed in the idiopathic group (12.2 degrees) during casting (P=0.003). Nonidiopathic patients did not maintain the correction gained during casting at the time of final follow-up. T1-T12 height increased across all study patients regardless of etiology during the period of casting at similar velocity to established norms of 1.4 cm/y for this age group. Serial casting offers modest deformity correction in idiopathic deformities compared with nonidiopathic deformities. Thoracic height growth continued throughout the casting period

  3. Exercise therapy in juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Takken, T.; van Brussel, M.; Engelbert, R. H. H.; van der Net, J.; Kuis, W.; Helders, P. J. M.

    2008-01-01

    Exercise therapy is considered an important component of the treatment of arthritis. The efficacy of exercise therapy has been reviewed in adults with rheumatoid arthritis but not in children with juvenile idiopathic arthritis (JIA). To assess the effects of exercise therapy on functional ability,

  4. Protocol for the Foot in Juvenile Idiopathic Arthritis trial (FiJIA: a randomised controlled trial of an integrated foot care programme for foot problems in JIA

    Directory of Open Access Journals (Sweden)

    Hendry Gordon J

    2009-06-01

    Full Text Available Abstract Background Foot and ankle problems are a common but relatively neglected manifestation of juvenile idiopathic arthritis. Studies of medical and non-medical interventions have shown that clinical outcome measures can be improved. However existing data has been drawn from small non-randomised clinical studies of single interventions that appear to under-represent the adult population suffering from juvenile idiopathic arthritis. To date, no evidence of combined therapies or integrated care for juvenile idiopathic arthritis patients with foot and ankle problems exists. Methods/design An exploratory phase II non-pharmacological randomised controlled trial where patients including young children, adolescents and adults with juvenile idiopathic arthritis and associated foot/ankle problems will be randomised to receive integrated podiatric care via a new foot care programme, or to receive standard podiatry care. Sixty patients (30 in each arm including children, adolescents and adults diagnosed with juvenile idiopathic arthritis who satisfy the inclusion and exclusion criteria will be recruited from 2 outpatient centres of paediatric and adult rheumatology respectively. Participants will be randomised by process of minimisation using the Minim software package. The primary outcome measure is the foot related impairment measured by the Juvenile Arthritis Disability Index questionnaire's impairment domain at 6 and 12 months, with secondary outcomes including disease activity score, foot deformity score, active/limited foot joint counts, spatio-temporal and plantar-pressure gait parameters, health related quality of life and semi-quantitative ultrasonography score for inflammatory foot lesions. The new foot care programme will comprise rapid assessment and investigation, targeted treatment, with detailed outcome assessment and follow-up at minimum intervals of 3 months. Data will be collected at baseline, 6 months and 12 months from baseline

  5. Effects of living environment on the postoperative Scoliosis Research Society-24 results in females with adolescent idiopathic scoliosis.

    Science.gov (United States)

    Misterska, Ewa; Głowacki, Maciej; Panek, Sławomir; Ignyś-O'Byrne, Anna; Głowacki, Jakub; Ignyś, Iwona; Krauss, Hanna; Piątek, Jacek

    2012-08-01

    There are many factors influencing postoperative health-related quality of life of adolescent idiopathic scoliosis patients, including the degree of the deformity, culture, differences in geography, rural versus urban living environments, and social factors. The objective of this study was to analyze the significance of geographic factors and their differences influencing the postoperative quality of life in females with adolescent idiopathic scoliosis residing in urban and rural environments, by use of the Polish version of the SRS-24 questionnaire. Forty urban and 20 rural postoperative patients with adolescent scoliosis with a minimum 2-year follow-up period after surgery were included in the study. The process of cross-cultural adaptation was performed according to the IQOLA Project. General results of the Polish SRS-24 equalled 4.1 (SD 0.5) and 4.0 (SD.0.5) in the rural and urban groups of patients, respectively. The 2 groups do not differ in incidence of floor and ceiling effects. The Cronbach's alpha values are excellent for the general result of SRS-24 in urban and rural groups (0.85 and 0.85, respectively). The sub-groups differed significantly in the self-image after surgery domain (p=0.048). Patients from the rural group scored higher in the self-image after surgery domain but reported higher pain levels when compared to urban patients. The associations between SRS-24 results and radiographic parameters in the rural group of patients were strong, compared with moderate relations reported in the urban group.

  6. CELSR2 is a candidate susceptibility gene in idiopathic scoliosis

    DEFF Research Database (Denmark)

    Einarsdottir, Elisabet; Grauers, Anna; Wang, Jingwen

    2017-01-01

    and the US. No association was found to variants in CELSR1 or CELSR3. Our findings suggest a rare variant in CELSR2 as causative for idiopathic scoliosis in a family with dominant segregation and further highlight common variation in CELSR2 in general susceptibility to idiopathic scoliosis in the Swedish......-Danish population. Both variants are located in the highly conserved GAIN protein domain, which is necessary for the auto-proteolysis of CELSR2, suggesting its functional importance....

  7. Acute Idiopathic Scrotal Edema

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    Micheál Breen

    2013-01-01

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

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

    Science.gov (United States)

    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.

  9. Familial steroid-sensitive idiopathic nephrotic syndrome: seven cases from three families in China

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    Yonghui Xia

    2013-05-01

    Full Text Available OBJECTIVES: Familial steroid-sensitive idiopathic nephrotic syndrome is rare, and only approximately 3% of patients have affected siblings. METHODS: Herein, we report seven cases of patients with steroid-sensitive idiopathic nephrotic syndrome from three Chinese families. Mutational screening of the Nphs2 gene was performed in all the patients. RESULTS: All seven of the familial steroid-sensitive idiopathic nephrotic syndrome cases in our sample exhibited minimal change disease, and one case also presented with mesangial proliferative glomerulonephritis, according to the renal pathology. No significant was associations were found between Nphs2 gene mutations and the onset of proteinuria and nephrotic syndrome in these familial cases. CONCLUSIONS: The presence of minimal change disease is important, but it is not an unusual finding in patients with familial steroid-sensitive idiopathic nephrotic syndrome, which appears to be clinically benign and genetically distinct from other types of nephrosis.

  10. Glymphatic MRI in idiopathic normal pressure hydrocephalus.

    Science.gov (United States)

    Ringstad, Geir; Vatnehol, Svein Are Sirirud; Eide, Per Kristian

    2017-10-01

    The glymphatic system has in previous studies been shown as fundamental to clearance of waste metabolites from the brain interstitial space, and is proposed to be instrumental in normal ageing and brain pathology such as Alzheimer's disease and brain trauma. Assessment of glymphatic function using magnetic resonance imaging with intrathecal contrast agent as a cerebrospinal fluid tracer has so far been limited to rodents. We aimed to image cerebrospinal fluid flow characteristics and glymphatic function in humans, and applied the methodology in a prospective study of 15 idiopathic normal pressure hydrocephalus patients (mean age 71.3 ± 8.1 years, three female and 12 male) and eight reference subjects (mean age 41.1 + 13.0 years, six female and two male) with suspected cerebrospinal fluid leakage (seven) and intracranial cyst (one). The imaging protocol included T1-weighted magnetic resonance imaging with equal sequence parameters before and at multiple time points through 24 h after intrathecal injection of the contrast agent gadobutrol at the lumbar level. All study subjects were kept in the supine position between examinations during the first day. Gadobutrol enhancement was measured at all imaging time points from regions of interest placed at predefined locations in brain parenchyma, the subarachnoid and intraventricular space, and inside the sagittal sinus. Parameters demonstrating gadobutrol enhancement and clearance in different locations were compared between idiopathic normal pressure hydrocephalus and reference subjects. A characteristic flow pattern in idiopathic normal hydrocephalus was ventricular reflux of gadobutrol from the subarachnoid space followed by transependymal gadobutrol migration. At the brain surfaces, gadobutrol propagated antegradely along large leptomeningeal arteries in all study subjects, and preceded glymphatic enhancement in adjacent brain tissue, indicating a pivotal role of intracranial pulsations for glymphatic function. In

  11. Idiopathic noncirrhotic portal hypertension in dogs: 33 cases (1982-1998).

    Science.gov (United States)

    Bunch, S E; Johnson, S E; Cullen, J M

    2001-02-01

    To describe clinical signs, diagnostic findings, and outcome in dogs with idiopathic intrahepatic portal hypertension. Retrospective study. 33 dogs. Medical records of dogs with portal hypertension of intra-abdominal origin were reviewed. Dogs with intra-abdominal portal hypertension of vascular causes or with hepatic histopathologic changes consistent with severe diffuse hepatobiliary disease were excluded. History and results of physical examination, clinicopathologic tests, diagnostic imaging studies, histologic examination, and treatment were summarized. Outcome was determined in 26 dogs. Dogs were referred most often because of ascites, intermittent vomiting or diarrhea, and polydipsia of several months' duration. Microcytosis, high serum alkaline phosphatase and alanine transaminase activities, hepatic dysfunction, urine specific gravity anastomoses were the major findings of diagnostic imaging. Hepatic histopathologic changes were consistent with idiopathic noncirrhotic portal hypertension and were indistinguishable from those of dogs with surgically created portocaval anastomosis. Outcome was determined for 19 dogs released from hospital; 13 dogs remained healthy with mostly palliative treatment for periods of 5 months to 9 years. The clinical signs, clinicopathologic test results, portal pressure, and gross appearance of the liver of dogs with idiopathic noncirrhotic portal hypertension may be identical to those of dogs with cirrhosis; therefore liver biopsy is crucial. Because the prognosis for idiopathic noncirrhotic portal hypertension is generally favorable, owners of affected dogs should be discouraged from choosing euthanasia.

  12. TREATMENT APPROACH FOR JUVENILE IDIOPATHIC ARTHRITIS-RELATED UVEITIS: 2012 UPDATE

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    Francesco Zulian

    2012-01-01

    Full Text Available Chronic anterior uveitis is the most common extra-articular complication of juvenile idiopathic arthritis. It is more frequent in the early onset forms with a higher prevalence in the oligoarticular (40% than in other juvenile idiopathic arthritis subtypes (5–14%. The risk for severe visual impairment is still high due to the development of sight-threatening complications (synechiae, band keratopathy, cataract, glaucoma, cystoid macular oedema. Treatment is not standardized and requires a complex decision-making process, involving a close collaboration between paediatric ophthalmologist and rheumatologist. Topical therapy alone is often inadequate to control ocular inflammation and bulbar injections are too invasive to perform in children therefore immunosuppressive treatment is often advocated. Low dose methotrexate is the second-line agent mostly used although no controlled studies comparing effects of early to late methotrexate treatment have been reported. Mycophenolate mofetil is effective in controlling inflammation in methotrexate -refractory patients. Its efficacy, however, seems to be more relevant in intermediate or posterior uveitis, than in juvenile idiopathic arthritis uveitis and scleritis. Anti-TNFα agents, namely infliximab and adalimimab showed effectiveness in open-label studies but no wide controlled trials have been reported so far. Adalimimab is as effective as infliximab but has an easier way of administration and a better drug tolerance. Abatacept should be used in anti-TNF refractory patients with juvenile idiopathic arthritis uveitis.

  13. Idiopathic Gingival Fibromatosis Rehabilitation: A Case Report with Two-Year Followup

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    Mahesh Jayachandran

    2013-01-01

    Full Text Available Gingival enlargements are quite common and may be either inflammatory, noninflammatory, or a combination of both. Gingival hyperplasia is a bizarre condition causing esthetic, functional, psychological, and masticatory disturbances of the oral cavity. Causes of gingival enlargement can be due to plaque accumulation, due to poor oral hygiene, inadequate nutrition, or systemic hormonal stimulation (Bakaeen and Scully, 1998. It can occur as an isolated disease or as part of a syndrome or chromosomal abnormality. A progressive fibrous enlargement of the gingiva is a facet of idiopathic fibrous hyperplasia of the gingiva (Carranza and Hogan, 2002; Gorlin et al., 1976. It is described variously as fibromatosis gingivae, gingivostomatitis, hereditary gingival fibromatosis, idiopathic fibromatosis, familial elephantiasis, and diffuse fibroma. We present a case of idiopathic gingival fibromatosis with its multidisciplinary approach of management.

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

    Science.gov (United States)

    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 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 corroborate previous studies demonstrating increased prevalence of sigmoid sinus diverticulum

  15. Treatment of idiopathic FSGS with adrenocorticotropic hormone gel.

    Science.gov (United States)

    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.

  16. The role of intrinsic spinal mechanisms in the pathogenesis of adolescent idiopathic scoliosis

    NARCIS (Netherlands)

    Kouwenhoven, J.W.M.

    2007-01-01

    Despite numerous years of dedicated research into the origin of idiopathic scoliosis, the pathogenesis of this classic orthopaedic disorder has so far remained elusive. A striking feature of idiopathic scoliosis is the fact that it does not occur in vertebrates other than humans, despite many

  17. [Biologic therapy in idiopathic inflammatory myopathy].

    Science.gov (United States)

    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.

  18. Idiopathic Harlequin syndrome – case report

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    Marcelina Grochowiec

    2015-09-01

    Full Text Available Introduction. Harlequin syndrome is a very rare neurological condition characterized by redness and excessive sweating of one half of the face in response to exercise and emotions. In most cases this disorder is not life-threatening. Objective. To present diagnostic difficulties of Harlequin syndrome in dermatological practice. Case report. We present a case of a 30-year-old man with redness and excessive sweating of the right half of the face as a result of exercise that was observed during the diagnosis of chronic urticaria at the Department of Dermatology. The patient was examined ophthalmologically and neurologically, had a CT scan of the head, and the Minor test performed. Idiopathic Harlequin syndrome was diagnosed based on case history and workup results. Conclusions . Harlequin syndrome occurs most often in the form of an idiopathic condition, but neurologic and ophthalmologic assessment should be performed since some diseases, such as brainstem infarction and schwannoma of the upper chest, may initially appear as Harlequin syndrome.

  19. Time course of recovery of idiopathic vocal fold paralysis.

    Science.gov (United States)

    Husain, Solomon; Sadoughi, Babak; Mor, Niv; Levin, Ariana M; Sulica, Lucian

    2018-01-01

    To clarify the time course of recovery in patients with idiopathic vocal fold paralysis. Retrospective chart review. Medical records for all patients with idiopathic vocal fold paralysis over a 10-year period were reviewed to obtain demographic and clinical information, including onset of disease and recovery of vocal function. Stroboscopic exams of patients who recovered voice were reviewed blindly to assess return of vocal fold motion. Thirty-eight of 55 patients (69%) recovered vocal function. Time course of recovery could be assessed in 34 patients who did not undergo injection augmentation. The mean time to recovery was 152.8 ± 109.3 days (left, 179.8 ± 111.3 days; right, 105.3 ± 93.7 days; P = .088). Two-thirds of patients recovered within 6 months. Probability of recovery declined over time. Five of 22 patients who recovered voice had return of vocal fold motion; 17 did not. The mean time to recovery did not differ between these groups (return of motion, 127.4 ± 132.3 days; no return of motion, 160.1 ± 105.1 days; P = .290). Sixty-nine percent of patients with idiopathic vocal fold paralysis recovered vocal function, two-thirds doing so within 6 months of onset. Age, gender, laterality, use of injection augmentation did not influence recovery rate. Declining probability of recovery over time leads us to consider framework surgery after 6 months in patients with idiopathic paralysis. 4. Laryngoscope, 128:148-152, 2018. © 2017 The American Laryngological, Rhinological and Otological Society, Inc.

  20. Bilateral versus single lung transplant for idiopathic pulmonary fibrosis.

    Science.gov (United States)

    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.

  1. Total spine and posterior fossa MRI screening in adolescent idiopathic scoliosis (177 cases

    Directory of Open Access Journals (Sweden)

    MR Etemadifar

    2005-05-01

    Full Text Available Background: MRI screening for idiopathic scoliosis is controversial. Considering our clinical experiences, the results of MRI in all patients with idiopathic scoliosis were evaluated. Methods: In a prospective clinical study, all neurologically normal patients with idiopathic scoliosis screened by posterior fossa and total spine MRI. Results: After excluding 9 patients for mild neurological findings, in other 177 patients (132 female, 45 male, the average age and curve angle was 15±2 years and 59±17º (30 to 135º, respectively. Convexity was to right in 146 and to left in 31 cases. MRI was positive in 12 cases (6.8%. In 5 cases (2.8%, neurosurgical intervention was necessary prior to scoliosis surgery. There was no relation between age, sex, presence of pain or curve angle and positive MRI findings (P>0.05. Left convexity was significantly related to positive MRI findings (P=0.013. In males with left convex curves, the probability of positive MRI findings was 8.8 folds other patients. Conclusion: Considering our results and other reported articles, it seems that routine MRI screening of all patients presenting as idiopathic scoliosis is necessary for detection of underlying pathologies. Key words: Idiopathic Scoliosis, MRI, Spine Syrinx, Chiari

  2. Growth factors in idiopathic pulmonary fibrosis: relative roles

    Directory of Open Access Journals (Sweden)

    Allen Jeremy T

    2001-11-01

    Full Text Available Abstract Treatment of idiopathic pulmonary fibrosis patients has evolved very slowly; the fundamental approach of corticosteroids alone or in combination with other immunosuppressive agents has had little impact on long-term survival. The continued use of corticosteroids is justified because of the lack of a more effective alternative. Current research indicates that the mechanisms driving idiopathic pulmonary fibrosis reflect abnormal, dysregulated wound healing within the lung, involving increased activity and possibly exaggerated responses by a spectrum of profibrogenic growth factors. An understanding of the roles of these growth factors, and the way in which they modulate events at cellular level, could lead to more targeted therapeutic strategies, improving patients' quality of life and survival.

  3. Update on diagnosis and treatment of idiopathic pulmonary fibrosis

    Science.gov (United States)

    Baddini-Martinez, José; Baldi, Bruno Guedes; da Costa, Cláudia Henrique; Jezler, Sérgio; Lima, Mariana Silva; Rufino, Rogério

    2015-01-01

    Idiopathic pulmonary fibrosis is a type of chronic fibrosing interstitial pneumonia, of unknown etiology, which is associated with a progressive decrease in pulmonary function and with high mortality rates. Interest in and knowledge of this disorder have grown substantially in recent years. In this review article, we broadly discuss distinct aspects related to the diagnosis and treatment of idiopathic pulmonary fibrosis. We list the current diagnostic criteria and describe the therapeutic approaches currently available, symptomatic treatments, the action of new drugs that are effective in slowing the decline in pulmonary function, and indications for lung transplantation. PMID:26578138

  4. Update on diagnosis and treatment of idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    José Baddini-Martinez

    2015-10-01

    Full Text Available Idiopathic pulmonary fibrosis is a type of chronic fibrosing interstitial pneumonia, of unknown etiology, which is associated with a progressive decrease in pulmonary function and with high mortality rates. Interest in and knowledge of this disorder have grown substantially in recent years. In this review article, we broadly discuss distinct aspects related to the diagnosis and treatment of idiopathic pulmonary fibrosis. We list the current diagnostic criteria and describe the therapeutic approaches currently available, symptomatic treatments, the action of new drugs that are effective in slowing the decline in pulmonary function, and indications for lung transplantation.

  5. A two-dimensional deformable phantom for quantitatively verifying deformation algorithms

    Energy Technology Data Exchange (ETDEWEB)

    Kirby, Neil; Chuang, Cynthia; Pouliot, Jean [Department of Radiation Oncology, University of California San Francisco, San Francisco, California 94143-1708 (United States)

    2011-08-15

    Purpose: The incorporation of deformable image registration into the treatment planning process is rapidly advancing. For this reason, the methods used to verify the underlying deformation algorithms must evolve equally fast. This manuscript proposes a two-dimensional deformable phantom, which can objectively verify the accuracy of deformation algorithms, as the next step for improving these techniques. Methods: The phantom represents a single plane of the anatomy for a head and neck patient. Inflation of a balloon catheter inside the phantom simulates tumor growth. CT and camera images of the phantom are acquired before and after its deformation. Nonradiopaque markers reside on the surface of the deformable anatomy and are visible through an acrylic plate, which enables an optical camera to measure their positions; thus, establishing the ground-truth deformation. This measured deformation is directly compared to the predictions of deformation algorithms, using several similarity metrics. The ratio of the number of points with more than a 3 mm deformation error over the number that are deformed by more than 3 mm is used for an error metric to evaluate algorithm accuracy. Results: An optical method of characterizing deformation has been successfully demonstrated. For the tests of this method, the balloon catheter deforms 32 out of the 54 surface markers by more than 3 mm. Different deformation errors result from the different similarity metrics. The most accurate deformation predictions had an error of 75%. Conclusions: The results presented here demonstrate the utility of the phantom for objectively verifying deformation algorithms and determining which is the most accurate. They also indicate that the phantom would benefit from more electron density heterogeneity. The reduction of the deformable anatomy to a two-dimensional system allows for the use of nonradiopaque markers, which do not influence deformation algorithms. This is the fundamental advantage of this

  6. Idiopathic granulomatous mastitis: case report and review of the literature.

    Science.gov (United States)

    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.

  7. Idiopathic Chronic Parotitis: Imaging Findings and Sialendoscopic Response.

    Science.gov (United States)

    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.

  8. Lung cancer in patients with idiopathic pulmonary fibrosis: frequency and CT findings

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Hak Jong; Im, Jung Gi; Ahn, Joong Mo; Yeon, Kyung Mo [College of Medicine, Seoul National University, Seoul (Korea, Republic of)

    1994-12-15

    The incidence of lung cancer in patients with idiopathic pulmonary fibrosis(lPF) is higher than that of general population. To evaluate the frequency and CT findings of lung cancer associated with idiopathic pulmonary fibrosis, we analyzed 19 patients with lung cancer associated with idiopathic pulmonary fibrosis. We analyzed retrospectively 19 patients with histologically confirmed lung cancer out of 208 patients diagnosed as IPF either by CT and clinical findings(n=188) or histologically(n=20). All 19 patients were male, aged 40-85 years (mean 66 years). Scanning techniques were conventional CT in 12 patients, HRCT in 1 patient and both conventional CT and HRCT in 6 patients. We analyzed the CT patterns of lung cancer and IPF, locations of the tumor and histologic types of lung cancer. The incidence of lung cancer in patients with idiopathic pulmonary fibrosis was 9.1%(19/208). In 11 of 19 patients, CT findings of lung cancer were ill-defined consolidation-like mass. Lung cancer was located mainly in lower lobes(right lower lobe; 10/19, left lower lobe; 5/19) and at the periphery(12/19). Histologically, squamous cell carcinoma was the most common cell type (11/19). The incidence of lung cancer in patients with idiopathic pulmonary fibrosis was much higher than that of general population. Typical CT findings of lung cancer were predominantly ill-defined consolidation like mass at the peripheral lung portion which is the location where the most advanced fibrosis occur.

  9. Lung cancer in patients with idiopathic pulmonary fibrosis: frequency and CT findings

    International Nuclear Information System (INIS)

    Lee, Hak Jong; Im, Jung Gi; Ahn, Joong Mo; Yeon, Kyung Mo

    1994-01-01

    The incidence of lung cancer in patients with idiopathic pulmonary fibrosis(lPF) is higher than that of general population. To evaluate the frequency and CT findings of lung cancer associated with idiopathic pulmonary fibrosis, we analyzed 19 patients with lung cancer associated with idiopathic pulmonary fibrosis. We analyzed retrospectively 19 patients with histologically confirmed lung cancer out of 208 patients diagnosed as IPF either by CT and clinical findings(n=188) or histologically(n=20). All 19 patients were male, aged 40-85 years (mean 66 years). Scanning techniques were conventional CT in 12 patients, HRCT in 1 patient and both conventional CT and HRCT in 6 patients. We analyzed the CT patterns of lung cancer and IPF, locations of the tumor and histologic types of lung cancer. The incidence of lung cancer in patients with idiopathic pulmonary fibrosis was 9.1%(19/208). In 11 of 19 patients, CT findings of lung cancer were ill-defined consolidation-like mass. Lung cancer was located mainly in lower lobes(right lower lobe; 10/19, left lower lobe; 5/19) and at the periphery(12/19). Histologically, squamous cell carcinoma was the most common cell type (11/19). The incidence of lung cancer in patients with idiopathic pulmonary fibrosis was much higher than that of general population. Typical CT findings of lung cancer were predominantly ill-defined consolidation like mass at the peripheral lung portion which is the location where the most advanced fibrosis occur

  10. Bone mineral density of girls with idiopathic scoliosis: a comparative study

    Directory of Open Access Journals (Sweden)

    Ameri E

    2012-11-01

    Full Text Available Background: Several studies have suggested higher incidence of osteoporosis in patients with idiopathic scoliosis in comparison with the normal population. The aim of this study was to assess the prevalence of low bone mass among adolescent girls with idiopathic scoliosis.Methods: In this cross-sectional study performed in shafa Hospital in Tehran, Iran during 2011-2012, we recruited fifty-seven 12- to-20-year old girls with idiopathic scoliosis and compared them with 100 age-matched healthy girls. The patients had no other diseases including neuromuscular disorders, congenital vertebral anomalies or a history of spinal surgery. Bone mineral densities (BMD of the hip and spine were evaluated and compared in all 157 participants using dual X-ray absorptiometry (DXA. Standard BMD (sBMD was also calculated at the lumbar spine. Results: Analysis of the data revealed that hip BMD was significantly (P=0.004 lower in patients with idiopathic scoliosis versus the controls. Moreover, BMD and sBMD of the Spine were also significantly lower in the patients (respectively, P=0.030 and P=0.030. Curve location had no effect on the values of hip BMD, spine BMD or spine sBMD (respectively, P=0.061 and P=0.274 and P=0.208.Finally, with more severe curves a lower bone mass was detected for sBMD and spine BMD (respectively, P=0.017 and P=0.016, but it was not significant for hip BMD (P=0.069.Conclusion: Adolescent girls with idiopathic scoliosis had lower bone mass compared with their healthy peers. The lower bone mass was correlated with the severity of the curve but not its location.

  11. Idiopath=ic Granulomatous Lobular Mastitis Masquerading as a Breast Tumor: A Case Report

    OpenAIRE

    Raman R, Thulasi; Manimaran, D

    2016-01-01

    Introduction Idiopathic granulomatous lobular mastitis (IGLM) is an inflammatory disease of the breast with an obscure etiology. It occurs mainly in women of reproductive age, and the lesion mimics carcinoma of the breast both clinically and radiologically Case Presentation We present the case of a 29-year-old female who visited our hospital in Kancheepuram, Tamil Nadu, with a 4 ? 3 cm lump in the upper outer quadrant of her left breast. The clinical and radiological findings were indicative ...

  12. Idiopathic multiple aneurysm of external carotid artery

    Directory of Open Access Journals (Sweden)

    Saravanan Balachandran

    2014-01-01

    Full Text Available Aneurysms of external carotid artery are rare. Treatments for these are undertaken for the prevention of complications like hemorrhage or rupture, and embolism. We present a 71-year-old male with idiopathic multiple aneurysm for the past 34 years on conservative management and regular follow up for the past 4 years. This case was discussed for the rarity of idiopathic multiple aneurysm of the external carotid artery and the need for individualized treatment protocol to be followed as in this case, only watchful observation considering the age and patient compliance. In this world of evolving surgical techniques and newer treatment modalities, conservative treatment still has a role to play. Primary care physicians at the community level have a major role in following these patients and referring them as and when the need arises.

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

    LENUS (Irish Health Repository)

    2012-02-01

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

  14. The classification, natural history and radiological/histological appearance of idiopathic pulmonary fibrosis and the other idiopathic interstitial pneumonias

    Directory of Open Access Journals (Sweden)

    G. Raghu

    2008-12-01

    Full Text Available The idiopathic interstitial pneumonias (IIPs are a heterogeneous group of rare interstitial lung diseases (ILDs or diffuse parenchymal lung diseases, which, as their name implies, are of unknown aetiology. The past 10 yrs have seen important advances in the classification of the IIPs into idiopathic pulmonary fibrosis (IPF and its corresponding histopathological pattern of usual interstitial pneumonia (UIP, plus six non-IPF IIP subtypes. The present article will look at the current classification of IIPs, arising from the Consensus Statement of the American Thoracic Society and European Respiratory Society, and discusses the importance of differential diagnosis of IPF from the non-IPF IIP subtypes, especially nonspecific interstitial pneumonia. Diagnosis of IIPs is a dynamic process involving close collaboration between pulmonologists, radiologists and pathologists. Increasingly accurate diagnosis of IPF has been made possible by the use of high-resolution computed tomography (HRCT and refinements in surgical lung biopsy. In IPF, a lung HRCT will typically reveal irregular reticular opacities, traction bronchiestasis and, most importantly, peripheral honeycombing. In contrast, histological examination shows evidence of UIP manifesting as typically subpleural and paraseptal established fibrosis, often with honeycomb changes, associated with mild chronic inflammation and varying numbers of fibroblastic foci in continuity with the edges of areas of established fibrosis. Despite these advances, obtaining a consistent and uniform diagnosis of idiopathic interstitial pneumonias is difficult, with studies showing significant disagreement in the diagnosis of interstitial lung diseases between academic centres of expertise and community-based clinicians. Greater interaction between academic and community clinicians, together with improved education, is needed to bridge this gap.

  15. Treatment of Refractory Idiopathic Supraorbital Neuralgia Using Percutaneous Pulsed Radiofrequency.

    Science.gov (United States)

    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.

  16. Familial Idiopathic Cranial Neuropathy in a Chinese Family.

    Science.gov (United States)

    Zhang, Li; Liang, Jianfeng; Yu, Yanbing

    Cranial neuropathy is usually idiopathic and familial cases are uncommon. We describe a family with 5 members with cranial neuropathy over 3 generations. All affected patients were women, indicating an X-linked dominant or an autosomal dominant mode of inheritance. Our cases and a review of the literature suggest that familial idiopathic cranial neuropathy is a rare condition which may be related to autosomal dominant vascular disorders (e.g. vascular tortuosity, sclerosis, elongation or extension), small posterior cranial fossas, anatomical variations of the posterior circulation, hypersensitivity of cranial nerves and other abnormalities. Moreover, microvascular decompression is the treatment of choice because vascular compression is the main factor in the pathogenesis. To the best of our knowledge, this is the first report of familial cranial neuropathy in China.

  17. Idiopathic brain herniation. A report of two paediatric cases.

    Science.gov (United States)

    Koc, Gonca; Doganay, Selim; Bayram, Ayse Kacar; Gorkem, Sureyya Burcu; Dogan, Mehmet Sait; Per, Huseyin; Coskun, Abdulhakim

    2014-10-01

    SUMMARY - 'Idiopathic' herniation of the brain is a rare entity previously reported in 13 cases. It may be incidentally encountered in neuroimaging studies acquired for various clinical indications. We herein describe two cases of idiopathic brain herniation that were incidentally diagnosed. A 12-year-old boy presented with a six-month history of daytime sleepiness and sudden spells of sleep. Herniation of the left inferior temporal gyrus was revealed in MRI acquired with the suspicion of epilepsy. His overnight polysomnogram and multiple sleep latency tests were compatible with the diagnosis of narcolepsy. The other case, a two-year-old girl, was transferred from an outside hospital due to partial seizures with the fever. Herniation of the precuneal gyrus was encountered in MRI acquired after controlling her seizures with the initiation of phenytoin. The brain herniations of both patients were considered to be inconsistent with their medical conditions, so that they were symptom-free with only medical treatment for following three and six months, respectively. This is a rare presentation of idiopathic brain herniation as an incidental finding that accompanied narcolepsy and epilepsy. Awareness of this entity would avoid excessive surgical and medical treatments.

  18. Idiopathic pulmonary fibrosis misdiagnosed as sputum-negative pulmonary tuberculosis.

    Science.gov (United States)

    Isah, Muhammad Danasabe; Abbas, Aminu; Abba, Abdullahi A; Umar, Mohammed

    2016-01-01

    Idiopathic pulmonary fibrosis (IPF), also known as cryptogenic fibrosing alveolitis, is one of a spectrum of idiopathic interstitial pneumonia. IPF is an increasingly common condition which poses many diagnostic and therapeutic challenges leading to misdiagnosis and mismanagement. We presented a 55-year-old male textile trader who was initially managed as sputum-negative pulmonary tuberculosis before histology report. He presented to our clinic with Breathlessness and cough of 3 years and 2.5 years, respectively. He had commenced anti-tuberculosis two months before presentation without significant relief. General Physical examination and vital signs were essentially normal. SPO2 was 96% on room air. Chest Examination revealed end-inspiratory bi-basal velcro-like crackles. Other systemic examinations were normal. Radiological examination by way of chest X- ray and chest CT showed features suggestive of IPF. The patient also had open Lung biopsy for histology and spirometry which demonstrated restrictive ventilatory function pattern. A diagnosis of Interstitial lung disease probably Idiopathic Pulmonary Fibrosis was entertained. He was commenced on Tab prednisolone, Tab Rabeprazole, with minimal improvement. IPF have often been misdiagnosed and treated as pulmonary tuberculosis with unfavorable outcome.

  19. Phenotypic spectrum of probable and genetically-confirmed idiopathic basal ganglia calcification.

    Science.gov (United States)

    Nicolas, Gaël; Pottier, Cyril; Charbonnier, Camille; Guyant-Maréchal, Lucie; Le Ber, Isabelle; Pariente, Jérémie; Labauge, Pierre; Ayrignac, Xavier; Defebvre, Luc; Maltête, David; Martinaud, Olivier; Lefaucheur, Romain; Guillin, Olivier; Wallon, David; Chaumette, Boris; Rondepierre, Philippe; Derache, Nathalie; Fromager, Guillaume; Schaeffer, Stéphane; Krystkowiak, Pierre; Verny, Christophe; Jurici, Snejana; Sauvée, Mathilde; Vérin, Marc; Lebouvier, Thibaud; Rouaud, Olivier; Thauvin-Robinet, Christel; Rousseau, Stéphane; Rovelet-Lecrux, Anne; Frebourg, Thierry; Campion, Dominique; Hannequin, Didier

    2013-11-01

    Idiopathic basal ganglia calcification is characterized by mineral deposits in the brain, an autosomal dominant pattern of inheritance in most cases and genetic heterogeneity. The first causal genes, SLC20A2 and PDGFRB, have recently been reported. Diagnosing idiopathic basal ganglia calcification necessitates the exclusion of other causes, including calcification related to normal ageing, for which no normative data exist. Our objectives were to diagnose accurately and then describe the clinical and radiological characteristics of idiopathic basal ganglia calcification. First, calcifications were evaluated using a visual rating scale on the computerized tomography scans of 600 consecutively hospitalized unselected controls. We determined an age-specific threshold in these control computerized tomography scans as the value of the 99th percentile of the total calcification score within three age categories: 60 years. To study the phenotype of the disease, patients with basal ganglia calcification were recruited from several medical centres. Calcifications that rated below the age-specific threshold using the same scale were excluded, as were patients with differential diagnoses of idiopathic basal ganglia calcification, after an extensive aetiological assessment. Sanger sequencing of SLC20A2 and PDGFRB was performed. In total, 72 patients were diagnosed with idiopathic basal ganglia calcification, 25 of whom bore a mutation in either SLC20A2 (two families, four sporadic cases) or PDGFRB (one family, two sporadic cases). Five mutations were novel. Seventy-one per cent of the patients with idiopathic basal ganglia calcification were symptomatic (mean age of clinical onset: 39 ± 20 years; mean age at last evaluation: 55 ± 19 years). Among them, the most frequent signs were: cognitive impairment (58.8%), psychiatric symptoms (56.9%) and movement disorders (54.9%). Few clinical differences appeared between SLC20A2 and PDGFRB mutation carriers. Radiological analysis

  20. Idiopathic fibrosing pancreatitis: A cause of obstructive jaundice in childhood

    International Nuclear Information System (INIS)

    Atkinson, G.O. Jr.; Wyly, J.B.; Gay, B.B. Jr.; Ball, T.I.; Winn, K.J.

    1988-01-01

    Idiopathic fibrosing pancreatitis is a chronic process of unknown etiology characterized by extensive infiltration of the pancreatic parenchyma by fibrous tissue. This disease process is uncommon in the pediatric patient and is consequently rarely considered in the differential diagnosis of abdominal pain and jaundice in the child. The sonographic demonstration of a dilated biliary tree and common bile duct compressed by an enlarged pancreas may be the first suggestion of this entity. Two patients with idiopathic fibrosing pancreatitis and obstructive jaundice are reported with a review of the clinical, radiographic, and pathologic findings. (orig.)

  1. Idiopathic fibrosing pancreatitis: A cause of obstructive jaundice in childhood

    Energy Technology Data Exchange (ETDEWEB)

    Atkinson, G.O. Jr.; Wyly, J.B.; Gay, B.B. Jr.; Ball, T.I.; Winn, K.J.

    1988-01-01

    Idiopathic fibrosing pancreatitis is a chronic process of unknown etiology characterized by extensive infiltration of the pancreatic parenchyma by fibrous tissue. This disease process is uncommon in the pediatric patient and is consequently rarely considered in the differential diagnosis of abdominal pain and jaundice in the child. The sonographic demonstration of a dilated biliary tree and common bile duct compressed by an enlarged pancreas may be the first suggestion of this entity. Two patients with idiopathic fibrosing pancreatitis and obstructive jaundice are reported with a review of the clinical, radiographic, and pathologic findings.

  2. Proposed outcome measures for prospective clinical trials in juvenile idiopathic arthritis-associated uveitis

    DEFF Research Database (Denmark)

    Heiligenhaus, Arnd; Foeldvari, Ivan; Edelsten, Clive

    2012-01-01

    To develop a set of core outcome measures for use in randomized controlled trials (RCTs) and longitudinal observational studies in juvenile idiopathic arthritis (JIA)-associated uveitis.......To develop a set of core outcome measures for use in randomized controlled trials (RCTs) and longitudinal observational studies in juvenile idiopathic arthritis (JIA)-associated uveitis....

  3. Idiopathic Hypoparathyroidism Mimicking Ankylosing Spondylitis: A Case Report

    Directory of Open Access Journals (Sweden)

    Ozge Illeez Memetoglu

    2016-01-01

    Full Text Available Idiopathic hypoparathyroidism, inadequate secretion of parathyroid hormone of unknown etiology, may mimic ankylosing spondylitis both clinically and radiologically. Spinal complaints may be the first sign of any endocrinological disorder.

  4. Idiopathic non-cirrhotic portal hypertension: a review

    NARCIS (Netherlands)

    Schouten, Jeoffrey N. L.; Verheij, Joanne; Seijo, Susana

    2015-01-01

    Idiopathic non-cirrhotic portal hypertension (INCPH) is a rare disease characterized of intrahepatic portal hypertension in the absence of cirrhosis or other causes of liver disease and splanchnic venous thrombosis. The etiology of INCPH can be classified in five categories: 1) immunological

  5. Diffuse idiopathic skeletal hyperostosis in ancient clergymen.

    NARCIS (Netherlands)

    Verlaan, J.J.; Oner, F.C.; Maat, G.J.

    2007-01-01

    Diffuse idiopathic skeletal hyperostosis (DISH) is a common but often unrecognized systemic disorder observed mainly in the elderly. DISH is diagnosed when the anterior longitudinal ligament of the spine is ossified on at least four contiguous spinal levels or when multiple peripheral enthesopathies

  6. Diffuse idiopathic skeletal hyperostosis in ancient clergymen

    NARCIS (Netherlands)

    Verlaan, J.J.; Oner, F.C.; Maat, G.R.J.

    2007-01-01

    Diffuse idiopathic skeletal hyperostosis (DISH) is a common but often unrecognized systemic disorder observed mainly in the elderly. DISH is diagnosed when the anterior longitudinal ligament of the spine is ossified on at least four contiguous spinal levels or when multiple peripheral

  7. Drug therapy for chronic idiopathic axonal polyneuropathy

    NARCIS (Netherlands)

    Warendorf, Janna; Vrancken, Alexander F.J.E.; van Schaik, Ivo N.; Hughes, Richard A.C.; Notermans, Nicolette C.

    2017-01-01

    Background: Chronic idiopathic axonal polyneuropathy (CIAP) is an insidiously progressive sensory or sensorimotor polyneuropathy that affects elderly people. Although severe disability or handicap does not occur, CIAP reduces quality of life. CIAP is diagnosed in 10% to 25% of people referred for

  8. Drug therapy for chronic idiopathic axonal polyneuropathy

    NARCIS (Netherlands)

    Warendorf, Janna; Vrancken, Alexander F. J. E.; van Schaik, Ivo N.; Hughes, Richard A. C.; Notermans, Nicolette C.

    2017-01-01

    Chronic idiopathic axonal polyneuropathy (CIAP) is an insidiously progressive sensory or sensorimotor polyneuropathy that affects elderly people. Although severe disability or handicap does not occur, CIAP reduces quality of life. CIAP is diagnosed in 10% to 25% of people referred for evaluation of

  9. Acute Lymphoblastic Leukaemia presenting as Juvenile Idiopathic ...

    African Journals Online (AJOL)

    Background: Acute Lymphoblastic Leukaemia in children commonly presents with osteo articular manifestations that may mimic Juvenile Idiopathic Arthritis. This may create considerable diagnostic difficulty and lead to delay in commencing appropriate treatment. Case: An eight year old boy who presented with multiple ...

  10. A case of acute exacerbation of idiopathic pulmonary fibrosis after proton beam therapy for non-small cell lung cancer

    International Nuclear Information System (INIS)

    Nagano, Tatsuya; Kotani, Yoshikazu; Fujii, Osamu

    2012-01-01

    There have been no reports describing acute exacerbations of idiopathic pulmonary fibrosis after particle radiotherapy for non-small cell lung cancer. The present study describes the case of a 76-year-old Japanese man with squamous cell carcinoma of the lung that relapsed in the left upper lobe 1 year after right upper lobectomy. He had been treated with oral prednisolone 20 mg/day every 2 days for idiopathic pulmonary fibrosis, and the relapsed lung cancer was treated by proton beam therapy, which was expected to cause the least adverse effects on the idiopathic pulmonary fibrosis. Fifteen days after the initiation of proton beam therapy, the idiopathic pulmonary fibrosis exacerbated, centered on the left upper lobe, for which intensive steroid therapy was given. About 3 months later, the acute exacerbation of idiopathic pulmonary fibrosis had improved, and the relapsed lung cancer became undetectable. Clinicians should be aware that an acute exacerbation of idiopathic pulmonary fibrosis may occur even in proton beam therapy, although proton beam therapy appears to be an effective treatment option for patients with idiopathic pulmonary fibrosis. (author)

  11. Impact of juvenile idiopathic arthritis on schooling

    Directory of Open Access Journals (Sweden)

    Bouaddi Ilham

    2013-01-01

    Full Text Available Abstract Background Juvenile idiopathic arthritis (JIA is the most common arthropathy of childhood. Different diseases affect school attendance to varying degrees. The aim of this study was to assess the impact of juvenile idiopathic arthritis (JIA on Moroccan children’s schooling. Methods Thirty-three children with JIA were included in this study, having been previously diagnosed according to the classification criteria of the International League of Associations for Rheumatology (ILAR. Seventy-four healthy children were recruited to serve as controls. Data was obtained for all children on their school level, educational performance, and attendance. The rate of absenteeism due to health complications was noted. Results All healthy children were able to attend school (p Conclusions Our study suggested that the schooling of children with JIA was negatively impacted due to the disorder. More studies, with a larger sample of children, are needed to confirm our findings.

  12. Analysis of the Juvenile Idiopathic Arthritis Immunization Schedule

    Directory of Open Access Journals (Sweden)

    L. S. Namazova-Baranova

    2016-01-01

    Full Text Available Background: The connection between vaccination and autoimmune diseases (and rheumatic pathology in particular is still a subject of discussions. When discussing the possibility of vaccinating rheumatic patients we should take into account the ultra high dangers that infectious diseases pose for such patients, including those that can be prevented by vaccination. We should also take into account the experience of using various vaccine types in rheumatic patients, which illustrates of their high safety profile.Objective: Our aim was to study the immunization schedule in children with juvenile idiopathic arthritis.Methods: The evaluation of vaccine history and other anamnestic data in juvenile idiopathic arthritis patients was based on individual medical records (individual child’s card/preventive vaccination certificate, as well as questionnaires filled by mothers.Results: It has been determined that a significant proportion of children with vaccination schedule deviations are juvenile idiopathic arthritis patients. Almost one in four children with a confirmed rheumatic diagnosis has not been immunized against the major vaccine-preventable diseases. In one non-vaccinated group, there was a case of juvenile arthritis onset after recovering from measles. A small number of patient mothers connects the manifestation of rheumatic diseases with vaccination.Conclusion: Violations of vaccination status in JIA patients require corrections according to the results of clinical studies and the recommendations of international experts.

  13. The effect of myofascial release (MFR) on an adult with idiopathic scoliosis.

    Science.gov (United States)

    LeBauer, Aaron; Brtalik, Robert; Stowe, Katherine

    2008-10-01

    The lack of evidence of conservative treatment has led to an interest in exploring myofascial release (MFR) as an effective means of controlling spinal curvature progression in adolescents with idiopathic scoliosis. The purpose of this case study is to measure the effects of MFR as a manual therapy technique in the treatment of idiopathic scoliosis. One 18-year-old female subject underwent 6 weeks of MFR treatment consisting of two sessions each week for 60min. Pain, pulmonary function, and quality of life were measured. Six goniometric measurements were taken encompassing trunk flexion, extension, and rotation. The subject improved with pain levels, trunk rotation, posture, quality of life, and pulmonary function. The results suggest further investigation is needed using MFR, as an effective manual therapy treatment for idiopathic scoliosis.

  14. Percutaneous micro-balloon compression for treatment of high risk idiopathic trigeminal neuralgia

    International Nuclear Information System (INIS)

    Zou Jianjun; Ma Yi; Wang Bin; Li Yanfeng; Huang Haitao; Li Fuyong

    2008-01-01

    Objective: To evaluate the clinical effectiveness and complications of percutaneous micro- balloon compression (PMC) of trigeminal ganglion for high risk idiopathic trigeminal neuralgia. Methods: To analyze retrospectively the clinical data of 3053 cases of idiopathic trigeminal nemalgia, of which 804 cases were in high risk, who underwent PMC from Jan. 2001 to Dec. 2007 in our department. Results: 833 procedures were performed on these 804 patients. The immediate effective rate was 97.3%; with recurrence rate of 6.8%, ipsilateral paresthesia incidence 3.7%; and no keratohelcosis with approximately 2/3 masticator, muscles weakness and diplopia 0.2%. Mean follow-up time was 36 months. Conclusions: PMC procedure is very effective for idiopathic trigeminal neuralgia especially in high risk patients, and especially prefer for the pain involved the first branch neuralgia. (authors)

  15. Idiopathic Noncirrhotic Portal Hypertension: An Appraisal

    Science.gov (United States)

    Lee, Hwajeong; Rehman, Aseeb Ur; Fiel, M. Isabel

    2016-01-01

    Idiopathic noncirrhotic portal hypertension is a poorly defined clinical condition of unknown etiology. Patients present with signs and symptoms of portal hypertension without evidence of cirrhosis. The disease course appears to be indolent and benign with an overall better outcome than cirrhosis, as long as the complications of portal hypertension are properly managed. This condition has been recognized in different parts of the world in diverse ethnic groups with variable risk factors, resulting in numerous terminologies and lack of standardized diagnostic criteria. Therefore, although the diagnosis of idiopathic noncirrhotic portal hypertension requires clinical exclusion of other conditions that can cause portal hypertension and histopathologic confirmation, this entity is under-recognized clinically as well as pathologically. Recent studies have demonstrated that variable histopathologic entities with different terms likely represent a histologic spectrum of a single entity of which obliterative portal venopathy might be an underlying pathogenesis. This perception calls for standardization of the nomenclature and formulation of widely accepted diagnostic criteria, which will facilitate easier recognition of this disorder and will highlight awareness of this entity. PMID:26563701

  16. A case of idiopathic portalhypertension

    International Nuclear Information System (INIS)

    Serizawa, Ken; Yajima, Yoshiaki; Onodera, Hiroyoshi; Hirata, Toru; Sugawara, Hiroshi

    1982-01-01

    A 40-year-old man was referred to our clinic for esophageal varices. Histological examination of the liver biopsy samples revealed no sign of liver cirrhosis. Celiac angiography and ultrasound showed no obstruction of portal vein. A diagnosis of idiopathic portalhypertension was established. Splenomegaly and collateral circulation from spleen to left retroperitoneum were shown on CT scan and confirmed by surgical operation. CT scan following operation showed no collateral circulation. (author)

  17. Juvenile idiopathic arthritis-associated uveitis

    OpenAIRE

    Clarke, Sarah; Sen, Ethan; Ramanan, Athimalaipet

    2016-01-01

    Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease of childhood, with JIA-associated uveitis its most common extra-articular manifestation. JIA-associated uveitis is a potentially sight-threatening condition and thus carries a considerable risk of morbidity. The aetiology of the condition is autoimmune in nature with the predominant involvement of CD4(+) T cells. However, the underlying pathogenic mechanisms remain unclear, particularly regarding interplay between geneti...

  18. Syphilis mimicking idiopathic intracranial hypertension

    DEFF Research Database (Denmark)

    Yri, Hanne; Wegener, Marianne; Jensen, Rigmor

    2011-01-01

    Idiopathic intracranial hypertension (IIH) is a condition of yet unknown aetiology affecting predominantly obese females of childbearing age. IIH is a diagnosis of exclusion as raised cerebrospinal fluid pressure may occur secondary to numerous other medical conditions. An atypical phenotype or a...... antibiotic treatment, signs and symptoms of elevated intracranial pressure resolved completely. Syphilis is a rare, but very important, differential diagnosis that in this case was clinically indistinguishable from IIH....

  19. Idiopathic retroperitoneal fibrosis associated with Hashimoto's thyroiditis in a patient with a single functioning kidney

    Directory of Open Access Journals (Sweden)

    Byung Sun Kim

    2013-12-01

    Full Text Available Retroperitoneal fibrosis (RPF is a rare disease characterized by the presence of fibroinflammatory tissue around the abdominal aorta and ureteral entrapment in most cases. Idiopathic RPF is frequently reported in association with autoimmune diseases; however, there have been few reports of idiopathic RPF associated with Hashimoto's thyroiditis. Here, we report a case of idiopathic RPF with Hashimoto's thyroiditis in a patient with a single functioning kidney, which was successfully treated by corticosteroid therapy and transient intraureteral stent insertion with a double-J catheter.

  20. Non-Autoimmune Subclinical and Overt Hypothyroidism in Idiopathic Steroid-resistant Nephrotic Syndrome in Children.

    Science.gov (United States)

    Marimuthu, Vidhya; Krishnamurthy, Sriram; Rajappa, Medha

    2017-11-15

    To evaluate the frequency of non-autoimmune subclinical and overt hypothyroidism in children with idiopathic steroid-resistant nephrotic syndrome (SRNS). This cross-sectional study recruited 30 children (age 1-18 y) with idiopathic SRNS; and 30 healthy controls. Serum T3, T4 and TSH were performed in cases as well as controls. Anti-thyroid peroxidase and anti-thyroglobulin antibody tests were performed in all cases. Non-autoimmune subclinical or overt hypothyroidism was detected in 10 out of 30 children with idiopathic SRNS; 2 had overt hypothyroidism, while 8 patients had subclinical hypothyroidism. Children with SRNS had a mean (SD) TSH value 4.55 (4.64) mIU/L that was higher as compared to controls (1.88 (1.04) mIU/L) (Phypothyroidism (2 cases) and grade III subclinical hypothyroidism (1 case) were subsequently started on levothyroxine therapy. The prevalence of subclinical and overt hypothyroidism seems to be high in idiopathic SRNS, with almost one-third of children having overt or subclinical non-autoimmune hypothyroidism.

  1. Effect of acid suppression therapy on gastroesophageal reflux and cough in idiopathic pulmonary fibrosis: an intervention study.

    Science.gov (United States)

    Kilduff, Claire E; Counter, Melanie J; Thomas, Gareth A; Harrison, Nicholas K; Hope-Gill, Benjamin D

    2014-01-01

    Chronic cough affects more than 70 percent of patients with Idiopathic Pulmonary Fibrosis and causes significant morbidity. Gastroesophageal reflux is the cause of some cases of chronic cough; and also has a postulated role in the aetiology of Idiopathic Pulmonary Fibrosis. A high prevalence of acid; and more recently non-acid, reflux has been observed in Idiopathic Pulmonary Fibrosis cohorts. Therefore, gastroesophageal reflux may be implicated in the pathogenesis of cough in Idiopathic Pulmonary Fibrosis. Eighteen subjects with Idiopathic Pulmonary Fibrosis underwent 24-hour oesophageal impedance and cough count monitoring after the careful exclusion of causes of chronic cough other than gastroesophageal reflux. All 18 were then treated with high dose acid suppression therapies. Fourteen subjects underwent repeat 24-hour oesophageal impedance and cough count monitoring after eight weeks. Total reflux and acid reflux frequencies were within the normal range in the majority of this cohort. The frequencies of non-acid and proximal reflux events were above the normal range. Following high dose acid suppression therapy there was a significant decrease in the number of acid reflux events (p = 0.02), but an increase in the number of non-acid reflux events (p = 0.01). There was no change in cough frequency (p = 0.70). This study confirms that non-acid reflux is prevalent; and that proximal oesophageal reflux occurs in the majority, of subjects with Idiopathic Pulmonary Fibrosis. It is the first study to investigate the effect of acid suppression therapy on gastroesophageal reflux and cough in patients with Idiopathic Pulmonary Fibrosis. The observation that cough frequency does not improve despite verifiable reductions in oesophageal acid exposure challenges the role of acid reflux in Idiopathic Pulmonary Fibrosis associated cough. The finding that non-acid reflux is increased following the use of acid suppression therapies cautions against the widespread use

  2. Idiopathic Thrombocytopenic Purpura Misdiagnosed as Hereditary Angioedema

    DEFF Research Database (Denmark)

    Andersen, Michelle Fog; Bygum, Anette

    2015-01-01

    however not always have to be caused by angioedema but can relate to other concomitant disorders. In this report we are focusing on misdiagnosis in a patient with known hereditary angioedema, whose bleeding episode caused by idiopathic thrombocytopenic purpura was mistaken for an acute attack...

  3. Histological characteristics of diffuse idiopathic skeletal hyperostosis

    NARCIS (Netherlands)

    Kuperus, JS; Westerveld, L Anneloes; Rutges, Joost A; Alblas, Jacqueline; van Rijen, Mattie H; Bleys, Ronald L A W; Oner, F Cumhur; Verlaan, JJ

    Diffuse idiopathic skeletal hyperostosis (DISH) is a predominantly radiographic diagnosis and histological knowledge of DISH is limited. The aim of this study was to describe the histological characteristics of DISH in the spinal column and to study the relation between DISH and intervertebral disc

  4. High-dose acetylcysteine in idiopathic pulmonary fibrosis

    NARCIS (Netherlands)

    Demedts, Maurits; Behr, Juergen; Buhl, Roland; Costabel, Ulrich; Dekhuijzen, Richard; Jansen, Henk M.; MacNee, William; Thomeer, Michiel; Wallaert, Benoit; Laurent, François; Nicholson, Andrew G.; Verbeken, Eric K.; Verschakelen, Johny; Flower, Christopher D. R.; Capron, Frédérique; Petruzzelli, Stefano; de Vuyst, Paul; van den Bosch, Jules M. M.; Rodriguez-Becerra, Eulogio; Corvasce, Giuseppina; Lankhorst, Ida; Sardina, Marco; Montanari, Mauro

    2005-01-01

    BACKGROUND Idiopathic pulmonary fibrosis is a chronic progressive disorder with a poor prognosis. METHODS We conducted a double-blind, randomized, placebo-controlled multicenter study that assessed the effectiveness over one year of a high oral dose of acetylcysteine (600 mg three times daily) added

  5. Natural history of idiopathic abducens nerve paresis in a young adult.

    Science.gov (United States)

    Hussaindeen, Jameel Rizwana; Mani, Revathy; Rakshit, Archayeeta; Ramasubramanian, Srikanth; Vittal Praveen, Smitha

    2016-01-01

    The natural history of idiopathic abducens nerve paresis and the role of conservative management such as vision training during the recovery process is not well documented in the literature to the best of our knowledge. This case report presents the natural recovery process of idiopathic abducens nerve paresis in a young adult and the role of vision therapy in the recovery process. Copyright © 2016 Spanish General Council of Optometry. Published by Elsevier España, S.L.U. All rights reserved.

  6. Comparison of Srs-24 And Srs-22 Scores in Thirty Eight Adolescent Idiopathic Scoliosis Patients Who Had Undergone Surgical Correction

    Directory of Open Access Journals (Sweden)

    CYW Chan

    2009-05-01

    Full Text Available Adolescent idiopathic scoliosis is a spinal deformity that affects patients’ self image and confidence. Surgery is offered when the curvature is greater than 50 degrees based on the likelihood of curvature progression. Outcome measures for scoliosis correction can be described in terms of radiological improvement or improvement of health related quality of life scores. The Scoliosis Research Society 22 (SRS-22 and Scoliosis Research Society 24 (SRS-24 questionnaires are widely accepted and used to characterize clinical results. Therefore, this prospective study of 38 patients aims to investigate how the SRS-24 and SRS-22 questionnaires compare to each other in terms of scoring when the same group of patients is evaluated. The SRS-22 questionnaire tends to give an inflated value in the overall score, pain and self image domain compared to the SRS-24 questionnaire.

  7. The diffuse interstitial lung disease - with emphasis in the idiopathic interstitial pneumonias

    International Nuclear Information System (INIS)

    Bustillo P, Jose G; Pacheco, Pedro M; Matiz, Carlos; Ojeda, Paulina; Carrillo B, Jorge A.

    2003-01-01

    The term diffuse interstitial lung disease, it refers to those diseases that commit the interstice basically, the space between the membrane basal epithelial and endothelial, although the damage can also commit the outlying air spaces and the vessels; the supplement is centered in the diffuse interstitial lung illness of unknown cause; well-known as idiopathic interstitial pneumonias, making emphasis in the more frequents, the pulmonary fibrosis idiopathic or cryptogenic fibrosant alveolitis

  8. Idiopathic trigeminal neuropathy in a poodle

    Directory of Open Access Journals (Sweden)

    Carlos Eduardo Aparicio

    2010-12-01

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

  9. Contemporary Management of Idiopathic Laryngotracheal Stenosis.

    Science.gov (United States)

    Donahoe, Laura; Keshavjee, Shaf

    2018-05-01

    Idiopathic laryngotracheal stenosis is a rare but well-described indication for subglottic tracheal resection. Initially described by Pearson in 1975, the 1-stage subglottic tracheal resection with reconstruction of the airway ensures preservation of the recurrent laryngeal nerves while resulting in an effective and durable repair of the stenosis. Copyright © 2018 Elsevier Inc. All rights reserved.

  10. Expression Signatures of Long Noncoding RNAs in Adolescent Idiopathic Scoliosis

    Directory of Open Access Journals (Sweden)

    Xiao-Yang Liu

    2015-01-01

    Full Text Available Purpose. Adolescent idiopathic scoliosis (AIS, the most common pediatric spinal deformity, is considered a complex genetic disease. Causing genes and pathogenesis of AIS are still unclear. This study was designed to identify differentially expressed long noncoding RNAs (lncRNAs involving the pathogenesis of AIS. Methods. We first performed comprehensive screening of lncRNA and mRNA in AIS patients and healthy children using Agilent human lncRNA + mRNA Array V3.0 microarray. LncRNAs expression in different AIS patients was further evaluated using quantitative PCR. Results. A total of 139 lncRNAs and 546 mRNAs were differentially expressed between AIS patients and healthy control. GO and Pathway analysis showed that these mRNAs might be involved in bone mineralization, neuromuscular junction, skeletal system morphogenesis, nucleotide and nucleic acid metabolism, and regulation of signal pathway. Four lncRNAs (ENST00000440778.1, ENST00000602322.1, ENST00000414894.1, and TCONS_00028768 were differentially expressed between different patients when grouped according to age, height, classification, severity of scoliosis, and Risser grade. Conclusions. This study demonstrates the abnormal expression of lncRNAs and mRNAs in AIS, and the expression of some lncRNAs was related to clinical features. This study is helpful for further understanding of lncRNAs in pathogenesis, treatment, and prognosis of AIS.

  11. Clinical characteristics and outcomes of familial and idiopathic ...

    African Journals Online (AJOL)

    Clinical characteristics and outcomes of familial and idiopathic dilated cardiomyopathy in Cape Town: A comparative study of 120 cases followed up over 14 years. NBA Ntusi, M Badri, F Gumedze, A Wonkam, BM Mayosi ...

  12. Diffuse idiopathic skeletal hyperostosis: case report and literature ...

    African Journals Online (AJOL)

    Diffuse idiopathic skeletal hyperostosis: case report and literature review. ... NSAIDs, local applications and physiotherapy, might also prove to be useful in patients with ... He had no history of peripheral joint involvement, fevers, cough, bowel ...

  13. Successful treatment of idiopathic pulmonary capillaritis with intravenous cyclophosphamide.

    LENUS (Irish Health Repository)

    Flanagan, Frances

    2013-03-01

    Idiopathic pulmonary hemosiderosis (IPH), a subtype of diffuse alveolar hemorrhage is a rare condition, first described by Virchow in 1864. Historically, it manifests in children in the first decade of life with the combination of hemoptysis, iron deficiency anemia, and alveolar infiltrates on chest radiograph. More recently, diffuse alveolar hemorrhage has been classified by the absence or presence of pulmonary capillaritis (PC), the latter carrying a potential for a poorer outcome. While systemic corticosteroids remain the first line treatment option, other immune modulators have been trailed including hydroxychloroquine, azathioprine, 6-mercaptopurine, and cyclophosphamide with varying results. Our case demonstrates for the first time, the successful use of intravenous cyclophosphamide in the management of chronic idiopathic PC.

  14. MRI findings in acute idiopathic transverse myelopathy in children

    International Nuclear Information System (INIS)

    Andronikou, Savvas; Albuquerque-Jonathan, Glenda; Hewlett, Richard; Wilmshurst, Jo

    2003-01-01

    To describe the clinical and MRI findings in three children with acute idiopathic myelopathy (AIM). Retrospective review of the clinical presentation, MRI findings and outcome of three patients diagnosed with acute idiopathic transverse myelitis. Of note was the swift onset of symptoms in all patients, without any preceding illness or history of vaccination in two of the patients, and the rapid resolution of symptoms on steroid therapy in all the patients. MRI showed T2-weighted hyperintensity and patchy enhancement with gadolinium, but the extensive cord involvement did not correlate with the severity of presentation or outcome. Our findings do not support that MRI evidence alone of diffuse myelopathy is a predictor of poor outcome in childhood AIM. (orig.)

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

    Science.gov (United States)

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

    2001-05-01

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

  16. Idiopathic intracranial hypertension and transverse sinus stenoses

    DEFF Research Database (Denmark)

    Skyrman, Simon; Fytagoridis, Anders; Andresen, Morten

    2013-01-01

    An 18-year-old woman was diagnosed with idiopathic intracranial hypertension (IIH) and bilateral transverse sinus stenoses (TSS), after presenting with papilledema and decreased visual acuity. Lumbar puncture revealed an opening pressure of >60 cm H2O. MRI showed bilateral TSS believed to be asso...

  17. Biological agents in polyarticular juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Amarilyo, Gil; Tarp, Simon; Foeldvari, Ivan

    2016-01-01

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

  18. Clinical balance tests, proprioceptive system and adolescent idiopathic scoliosis.

    Science.gov (United States)

    Le Berre, Morgane; Guyot, Marc-Alexandre; Agnani, Olivier; Bourdeauducq, Isabelle; Versyp, Marie-Christine; Donze, Cécile; Thévenon, André; Catanzariti, Jean-Francois

    2017-06-01

    Adolescent idiopathic scoliosis (AIS) is a three-dimensional deformity of the spinal column of unknown etiology. Multiple factors could be involved, including neurosensory pathways and, potentially, an elective disorder of dynamic proprioception. The purpose of this study was to determine whether routine balance tests could be used to demonstrate an elective alteration of dynamic proprioception in AIS. This was a multicentre case-control study based on prospectively collected clinical data, in three hospitals pediatric, with spine consultation, from January 2013 through April 2015. From an original population of 547 adolescents, inclusion and non-inclusion criteria indentified 114 adolescents with right thoracic AIS (mean age 14.5 ± 1.9 years, Cobb angle 35.7 ± 15.3°) and 81 matched adolescents without scoliosis (mean age 14.1 ± 1.9 years). Participants performed three routine clinical balance tests to assess the static and dynamic proprioception: the Fukuda-Utenberger stepping test (angle of rotation in degrees and distance of displacement in cm) to assess dynamic balance; the sharpened Romberg test and the unipedal stance test (eyes closed) to assess static balance. There was no significant difference between AIS subjects and controls for the static tests, but there was a significant difference for the dynamic test for both measures: distance of displacement (p tests can be performed in routine practice. Their validity as a biomarker for screening and monitoring purposes should be assessed.

  19. Detection of sex hormone in serum and semen of patients with idiopathic oligospermia and its significance

    International Nuclear Information System (INIS)

    Wang Guohong; Xu Ruiji; Zhang Zhongshu; Wang Youji

    2005-01-01

    The objective of this study was to detect testosterone(T), free testosterone (FT) and sex hormone-binding globulin(SHBG) in serum and semen of patients with idiopathic oligospermia, and further analyze the relationship between T, FT, SHBG and idiopathic oligospermia. Blood and semen samples were collected from males of a normal control group and an idiopathic oligospermia group at 8:00-10:00 am. The sperm density in semen was detected by routine semen analysis, while T, FT, SHBG in serum and semen were detected by RIA. There were no significant differences in serum concentrations of T, FT, SHBG between normal control group [(30.03±13.07)nmol/L,(97.50±46.96)pmol/L, (40.37±16.73)nmol/L, respectively] and idiopathic oligspermia group [(28.11±11.54) nmol/L, (94.88±42.04) pmol/L, (41.61± 18.86)noml/LJ(all P>0. 05). There were significant differences in semsn concentrations of FT and SHBG between normal control group[(2.01±0.32) pmol/L, (0.17±0.21)nmol/L] and idiopathic oligspermia group [ (0.52±0.44) pmol/L, (0.22±0.15) nmol/LJ (P 0.05). Therefore, measurement of semsn FT, SHBG concentration could early reflect the function of tesis, which is useful for early diagnosis and treatment of idiopathic oligospermia. (authors)

  20. Deformation-specific and deformation-invariant visual object recognition: pose vs identity recognition of people and deforming objects

    Directory of Open Access Journals (Sweden)

    Tristan J Webb

    2014-04-01

    Full Text Available When we see a human sitting down, standing up, or walking, we can recognise one of these poses independently of the individual, or we can recognise the individual person, independently of the pose. The same issues arise for deforming objects. For example, if we see a flag deformed by the wind, either blowing out or hanging languidly, we can usually recognise the flag, independently of its deformation; or we can recognise the deformation independently of the identity of the flag. We hypothesize that these types of recognition can be implemented by the primate visual system using temporo-spatial continuity as objects transform as a learning principle. In particular, we hypothesize that pose or deformation can be learned under conditions in which large numbers of different people are successively seen in the same pose, or objects in the same deformation. We also hypothesize that person-specific representations that are independent of pose, and object-specific representations that are independent of deformation and view, could be built, when individual people or objects are observed successively transforming from one pose or deformation and view to another. These hypotheses were tested in a simulation of the ventral visual system, VisNet, that uses temporal continuity, implemented in a synaptic learning rule with a short-term memory trace of previous neuronal activity, to learn invariant representations. It was found that depending on the statistics of the visual input, either pose-specific or deformation-specific representations could be built that were invariant with respect to individual and view; or that identity-specific representations could be built that were invariant with respect to pose or deformation and view. We propose that this is how pose-specific and pose-invariant, and deformation-specific and deformation-invariant, perceptual representations are built in the brain.

  1. Do the SRS-22 self-image and mental health domain scores reflect the degree of asymmetry of the back in adolescent idiopathic scoliosis?

    Science.gov (United States)

    Cheshire, James; Gardner, Adrian; Berryman, Fiona; Pynsent, Paul

    2017-01-01

    Patient-reported outcomes are becoming increasingly recognised in the management of patients with adolescent idiopathic scoliosis (AIS). Integrated Shape Imaging System 2 (ISIS2) surface topography is a validated tool to assess AIS. Previous studies have failed to demonstrate strong correlations between AIS and patient-reported outcomes highlighting the need for additional objective surface parameters to define the deformities associated with AIS. The aim of this study was to examine whether the Scoliosis Research Society-22 (SRS-22) outcome questionnaire reflects the degree of measurable external asymmetry of the back in AIS and thus is a measure of patient outcome for external appearance. A total of 102 pre-operative AIS patients were identified retrospectively. Objective parameters were measured using ISIS2 surface topography. The associations between these parameters and the self-image and mental health domains of the SRS-22 questionnaire were investigated using correlation coefficients. All correlations between the parameters of asymmetry and SRS-22 self-image score were of weak strength. Similarly, all correlations between the parameters of asymmetry and SRS-22 mental health score were of weak strength. The SRS-22 mental health and self-image domains correlate poorly with external measures of deformity. This demonstrates that the assessment of mental health and self-image by the SRS-22 has little to do with external torso shape. Whilst the SRS-22 assesses the patient as a whole, it provides little information about objective measures of deformity over which a surgeon has control.

  2. GENETIC PREDICTORS OF IDIOPATHIC SICK SINUS SYNDROME

    Directory of Open Access Journals (Sweden)

    A. A. Chernova

    2012-01-01

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

  3. Towards a new taxonomy of idiopathic orofacial pain.

    Science.gov (United States)

    Woda, Alain; Tubert-Jeannin, Stéphanie; Bouhassira, Didier; Attal, Nadine; Fleiter, Bernard; Goulet, Jean-Paul; Gremeau-Richard, Christelle; Navez, Marie Louise; Picard, Pascale; Pionchon, Paul; Albuisson, Eliane

    2005-08-01

    There is no current consensus on the taxonomy of the different forms of idiopathic orofacial pain (stomatodynia, atypical odontalgia, atypical facial pain, facial arthromyalgia), which are sometimes considered as separate entities and sometimes grouped together. In the present prospective multicentric study, we used a systematic approach to help to place these different painful syndromes in the general classification of chronic facial pain. This multicenter study was carried out on 245 consecutive patients presenting with chronic facial pain (>4 months duration). Each patient was seen by two experts who proposed a diagnosis, administered a 111-item questionnaire and filled out a standardized 68-item examination form. Statistical processing included univariate analysis and several forms of multidimensional analysis. Migraines (n=37), tension-type headache (n=26), post-traumatic neuralgia (n=20) and trigeminal neuralgia (n=13) tended to cluster independently. When signs and symptoms describing topographic features were not included in the list of variables, the idiopathic orofacial pain patients tended to cluster in a single group. Inside this large cluster, only stomatodynia (n=42) emerged as a distinct homogenous subgroup. In contrast, facial arthromyalgia (n=46) and an entity formed with atypical facial pain (n=25) and atypical odontalgia (n=13) could only be individualised by variables reflecting topographical characteristics. These data provide grounds for an evidence-based classification of idiopathic facial pain entities and indicate that the current sub-classification of these syndromes relies primarily on the topography of the symptoms.

  4. Increased brain water self-diffusion in patients with idiopathic intracranial hypertension

    DEFF Research Database (Denmark)

    Gideon, P; Sørensen, P S; Thomsen, C

    1995-01-01

    PURPOSE: To investigate changes in brain water diffusion in patients with idiopathic intracranial hypertension. METHODS: A motion-compensated MR pulse sequence was used to create diffusion maps of the apparent diffusion coefficient (ADC) in 12 patients fulfilling conventional diagnostic criteria...... for idiopathic intracranial hypertension and in 12 healthy volunteers. RESULTS: A significantly larger ADC was found within subcortical white matter in the patient group (mean, 1.16 x 10(-9) m2/s) than in the control group (mean, 0.75 x 10(-9) m2/s), whereas no significant differences were found within cortical...

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

    Directory of Open Access Journals (Sweden)

    Moore Peter

    2010-02-01

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

  6. Brief Report: Sensorimotor Gating in Idiopathic Autism and Autism Associated with Fragile X Syndrome

    Science.gov (United States)

    Yuhas, Jennifer; Cordeiro, Lisa; Tassone, Flora; Ballinger, Elizabeth; Schneider, Andrea; Long, James M.; Ornitz, Edward M.; Hessl, David

    2011-01-01

    Prepulse inhibition (PPI) may useful for exploring the proposed shared neurobiology between idiopathic autism and autism caused by FXS. We compared PPI in four groups: typically developing controls (n = 18), FXS and autism (FXS+A; n = 15), FXS without autism spectrum disorder (FXS-A; n = 17), and idiopathic autism (IA; n = 15). Relative to…

  7. Genome-wide association studies of adolescent idiopathic scoliosis suggest candidate susceptibility genes

    Science.gov (United States)

    Sharma, Swarkar; Gao, Xiaochong; Londono, Douglas; Devroy, Shonn E.; Mauldin, Kristen N.; Frankel, Jessica T.; Brandon, January M.; Zhang, Dongping; Li, Quan-Zhen; Dobbs, Matthew B.; Gurnett, Christina A.; Grant, Struan F.A.; Hakonarson, Hakon; Dormans, John P.; Herring, John A.; Gordon, Derek; Wise, Carol A.

    2011-01-01

    Adolescent idiopathic scoliosis (AIS) is an unexplained and common spinal deformity seen in otherwise healthy children. Its pathophysiology is poorly understood despite intensive investigation. Although genetic underpinnings are clear, replicated susceptibility loci that could provide insight into etiology have not been forthcoming. To address these issues, we performed genome-wide association studies (GWAS) of ∼327 000 single nucleotide polymorphisms (SNPs) in 419 AIS families. We found strongest evidence of association with chromosome 3p26.3 SNPs in the proximity of the CHL1 gene (P protein related to Robo3. Mutations in the Robo3 protein cause horizontal gaze palsy with progressive scoliosis (HGPPS), a rare disease marked by severe scoliosis. Other top associations in our GWAS were with SNPs in the DSCAM gene encoding an axon guidance protein in the same structural class with Chl1 and Robo3. We additionally found AIS associations with loci in CNTNAP2, supporting a previous study linking this gene with AIS. Cntnap2 is also of functional interest, as it interacts directly with L1 and Robo class proteins and participates in axon pathfinding. Our results suggest the relevance of axon guidance pathways in AIS susceptibility, although these findings require further study, particularly given the apparent genetic heterogeneity in this disease. PMID:21216876

  8. Epithelial to mesenchymal transition-related proteins ZEB1, β-catenin, and β-tubulin-III in idiopathic pulmonary fibrosis.

    Science.gov (United States)

    Chilosi, Marco; Caliò, Anna; Rossi, Andrea; Gilioli, Eliana; Pedica, Federica; Montagna, Licia; Pedron, Serena; Confalonieri, Marco; Doglioni, Claudio; Ziesche, Rolf; Grubinger, Markus; Mikulits, Wolfgang; Poletti, Venerino

    2017-01-01

    Epithelial to mesenchymal transition has been suggested as a relevant contributor to pulmonary fibrosis, but how and where this complex process is triggered in idiopathic pulmonary fibrosis is not fully understood. Beta-tubulin-III (Tubβ3), ZEB1, and β-catenin are partially under the negative control of miR-200, a family of micro-RNAs playing a major role in epithelial to mesenchymal transition, that are reduced in experimental lung fibrosis and idiopathic pulmonary fibrosis. We wonder whether in situ expression of these proteins is increased in idiopathic pulmonary fibrosis, to better understand the significance of miR-200 feedback loop and epithelial to mesenchymal transition. We investigated the immunohistochemical and immunofluorescent expression and precise location of ZEB1, Tubβ3, and β-catenin in tissue samples from 34 idiopathic pulmonary fibrosis cases and 21 controls (5 normal lungs and 16 other interstitial lung diseases). In 100% idiopathic pulmonary fibrosis samples, the three proteins were concurrently expressed in fibroblastic foci, as well in damaged epithelial cells overlying these lesions and in pericytes within neo-angiogenesis areas. These results were also confirmed by immunofluorescence assay. In controls the abnormal expression of the three proteins was absent or limited. This is the first study that relates concurrent expression of Tubβ3, ZEB1, and β-catenin to abnormal epithelial and myofibroblast differentiation in idiopathic pulmonary fibrosis, providing indirect but robust evidence of miR-200 deregulation and epithelial to mesenchymal transition activation in idiopathic pulmonary fibrosis. The abnormal expression and localization of these proteins in bronchiolar fibro-proliferative lesions are unique for idiopathic pulmonary fibrosis, and might represent a disease-specific marker in challenging lung biopsies.

  9. Clinical and nutritional outcomes in children with idiopathic superior mesenteric artery syndrome.

    Science.gov (United States)

    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 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 nutritional status in pediatric patients with

  10. Precision Medicine: The New Frontier in Idiopathic Pulmonary Fibrosis.

    Science.gov (United States)

    Brownell, Robert; Kaminski, Naftali; Woodruff, Prescott G; Bradford, Williamson Z; Richeldi, Luca; Martinez, Fernando J; Collard, Harold R

    2016-06-01

    Precision medicine is defined by the National Institute of Health's Precision Medicine Initiative Working Group as an approach to disease treatment that takes into account individual variability in genes, environment, and lifestyle. There has been increased interest in applying the concept of precision medicine to idiopathic pulmonary fibrosis, in particular to search for genetic and molecular biomarker-based profiles (so called endotypes) that identify mechanistically distinct disease subgroups. The relevance of precision medicine to idiopathic pulmonary fibrosis is yet to be established, but we believe that it holds great promise to provide targeted and highly effective therapies to patients. In this manuscript, we describe the field's nascent efforts in genetic/molecular endotype identification and how environmental and behavioral subgroups may also be relevant to disease management.

  11. An unusual case of idiopathic multiple invasive cervical resorption

    Directory of Open Access Journals (Sweden)

    Sathya Prakash Reddy Kesary

    2014-01-01

    Full Text Available Invasive cervical resorption is a rare form of root resorption, characterized by destruction of the cervical region of teeth resulting from the action of tooth resorbing cells. Being an asymptomatic condition, it is often discovered on routine radiographic examination. This multifactorial disease process can most commonly occur as a sequel to orthodontic treatment, dental trauma, bleaching procedures, and less commonly, as an outcome of segmental orthognathic surgery, periodontal root planning, tetracycline conditioning of the root canal, bruxism, transplantation of tooth, guided tissue regeneration, cementoenamel disjunction. In the absence of these predisposing factors, it can be labeled as ′idiopathic multiple cervical resorption′. This article describes the case of a medically fit Indian male, who displayed idiopathic invasive cervical resorption in multiple teeth.

  12. The efficacy of flutamide, an antiandrogen in idiopathic hirsutism

    Directory of Open Access Journals (Sweden)

    Somani V

    1998-01-01

    Full Text Available The efficacy of flutamide, an antiandrogen in idiopathic hirsutism was studied. The long term effects of. treatment with low doses of flutamide on clinical and hormonal parameters were investigated. Nine patients with idiopathic hirsutism were studied basally and during treatment with 125mg flutamide thrice daily for a period of 9 months. Safety parameters were assessed throughout the study. Hirsutism was graded by Ferriman and Gallwey score and hormones were evaluated basally and later quarterly. After three months of therapy, flutamide had caused a significant alleviation of hirsutism and this continued during the subsequent months. No clinical significant side effects were observed during the period of the study. Biochemical and hormonal parameters remained unchanged after 9 months of flutamide.

  13. Idiopathic granulomatous mastitis: in search of a therapeutic paradigm.

    Science.gov (United States)

    Wilson, Jason P; Massoll, Nicole; Marshall, Julia; Foss, Robin M; Copeland, Edward M; Grobmyer, Stephen R

    2007-08-01

    Idiopathic granulomatous mastitis, also known as idiopathic granulomatous lobular mastitis, is a benign breast lesion that represents both a diagnostic and therapeutic dilemma. We report two cases of granulomatous mastitis recently evaluated and managed at our institution. To better understand this rare disease, we analyzed treatment outcomes in reported cases of granulomatous mastitis. One hundred sixteen cases were subsequently analyzed. Primary management strategies included observation (n = 9), steroids (n = 29), partial mastectomy (n = 75), and mastectomy (n = 3). Success rates with each treatment were observation, 56 per cent; steroids, 42 per cent; partial mastectomy, 79 per cent; and mastectomy, 100 per cent. Based on this analysis, we propose a clinically useful algorithm for both workup and management of these challenging cases.

  14. Idiopathic Pulmonary Calcification and Ossification in an Elderly ...

    African Journals Online (AJOL)

    Histology of tissue from autopsy showed intraparenchymal pulmonary calcification and ossification with marrow elements. Idiopathic pulmonary calcification and ossification is rare. At autopsy, she was also found to have had bilateral subarachnoid haemorrhage (SAH), a diagnosis missed during clinical evaluation.

  15. The value of myocardial perfusion imaging in differentiating between idiopathic dilated cardiomyopathy from the ischemic form

    International Nuclear Information System (INIS)

    Fad, A.; Emami, F.; Eftekhari, M.; Saghari, M.; Fallahi, B.; Beiki, D.; Tkavar, A.

    2004-01-01

    Introduction: differentiating between ischemic cardiomyopathy and idiopathic dilated cardiomyopathy is important as coronary revascularization can improve prognosis in the ischemic subgroup. Due to inherent problems of coronary angiography in patients with depressed ejection fraction introducing a noninvasive tool to diagnose those who will benefit from angiography seems to be rewarding. We examined usefulness of myocardial perfusion scan in this group of patients. Materials and methods: study was performed on 64 patients (62 male and 2 female) aged 57.1 ± 6.7 y (mean ± SD) all with dilation of the left ventricular cavity and ejection fraction less than 40 % by echocardiography. Myocardial perfusion scan was performed in stress and rest phases. All the patients had coronary angiography which was used as the gold standard test. On each set of images, heart was arbitrary divided into 17 segments and perfusion abnormality in each segment was scored by a 5 grade scoring system (0-4). Summed stress Score was used as the scan criteria to differentiate dilated ischemic from idiopathic cardiomyopathy. Scores more than 17 were considered ischemic, and less than that, idiopathic. Results were compared with angiography. Results: from total 40 cases of ischemic cardiomyopathy (proved by angiography) 39 were correctly diagnosed by scan and only one case was mis categorized as idiopathic dilated cardiomyopathy . All 24 cases of idiopathic dilated cardiomyopathy were correctly diagnosed by scintigraphy. Sensitivity, specificity, positive predictive value, and negative predictive value of myocardial perfusion imaging for discrimination between ischemic and idiopathic dilated cardiomyopathy were 97.5 %, 100 %, 100 %, and 96 % respectively. Conclusion: Considering excellent accuracy of myocardial perfusion scan with scoring system in discrimination of ischemic dilated cardiomyopathy from idiopathic cardiomyopathy, this noninvasive test could be considered the main diagnostic test

  16. Development of additional pituitary hormone deficiencies in pediatric patients originally diagnosed with idiopathic isolated GH deficiency

    NARCIS (Netherlands)

    W.F. Blum (Werner); C.L. Deal (Cheri Lynn); A.G. Zimmermann (Alan); E.P. Shavrikova (Elena); C.J. Child (Christopher); C.A. Quigley (Charmian); S.L.S. Drop (Stenvert); G. Cutler (Gordon); R.G. Rosenfeld (Ron)

    2014-01-01

    textabstractObjective: We assessed the characteristics of children initially diagnosed with idiopathic isolated GH deficiency (IGHD) who later developed additional (multiple) pituitary hormone deficiencies (MPHD). Design: Data were analyzed for 5805 pediatric patients with idiopathic IGHD, who were

  17. An On-Chip RBC Deformability Checker Significantly Improves Velocity-Deformation Correlation

    Directory of Open Access Journals (Sweden)

    Chia-Hung Dylan Tsai

    2016-10-01

    Full Text Available An on-chip deformability checker is proposed to improve the velocity–deformation correlation for red blood cell (RBC evaluation. RBC deformability has been found related to human diseases, and can be evaluated based on RBC velocity through a microfluidic constriction as in conventional approaches. The correlation between transit velocity and amount of deformation provides statistical information of RBC deformability. However, such correlations are usually only moderate, or even weak, in practical evaluations due to limited range of RBC deformation. To solve this issue, we implemented three constrictions of different width in the proposed checker, so that three different deformation regions can be applied to RBCs. By considering cell responses from the three regions as a whole, we practically extend the range of cell deformation in the evaluation, and could resolve the issue about the limited range of RBC deformation. RBCs from five volunteer subjects were tested using the proposed checker. The results show that the correlation between cell deformation and transit velocity is significantly improved by the proposed deformability checker. The absolute values of the correlation coefficients are increased from an average of 0.54 to 0.92. The effects of cell size, shape and orientation to the evaluation are discussed according to the experimental results. The proposed checker is expected to be useful for RBC evaluation in medical practices.

  18. High-dose steroid therapy for idiopathic optic perineuritis: a case series

    Directory of Open Access Journals (Sweden)

    Mimura Tatsuya

    2010-12-01

    Full Text Available Abstract Introduction It has been reported that the prognosis of optic perineuritis may be poor when initiation of treatment is delayed. Here we report the successful treatment of three patients with idiopathic optic perineuritis, including two in whom initiation of therapy was delayed. Case presentation Three Japanese patients (two women aged 73 and 66 years, and one man aged 27 years presented with loss of vision (for five months, several months, and two months respectively and pain on eye movement in the third case only, and were diagnosed as having idiopathic optic perineuritis. Fat-suppressed T2-weighted magnetic resonance images showed high signal intensity areas around the affected optic nerves, suggesting the presence of optic perineuritis. Two patients received steroid pulse therapy and the third was given high-dose steroid therapy. The visual acuity improved in all three cases. Conclusion High-dose steroid therapy may be effective for idiopathic perineuritis in patients without optic nerve atrophy, even if initial treatment (including moderate-dose steroids has failed.

  19. CLINICAL CASE OF TOCILIZUMAB THERAPY IN A PATIENT WITH SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS

    Directory of Open Access Journals (Sweden)

    E. I. Alexeeva

    2013-01-01

    Full Text Available The article presents a case of successful application of a monoclonal antibodies drug to interleukin 6 receptors (tocilizumab at severe systemic juvenile idiopathic arthritis with the development of secondary hemophagocytic syndrome. Tocilizumab treatment secured a decrease in clinical and laboratory parameters of the disease activity, life quality improvement, systemic juvenile idiopathic arthritis and hemophagocytic syndrome remission and allowed avoiding the per os prescription of glucocorticoids.

  20. Unusual Presentation Of Idiopathic Retroperitoneal Fibrosis: Case ...

    African Journals Online (AJOL)

    Idiopathic retroperitoneal fibrosis (IRF) is an uncommon entity described as progressive proliferation of connective tissues leading to a fibrous plaque-like lesions that encases the aorta and inferior vena cava inferior to the level of the renal arteries. Mass forming retroperitoneal fibrosis is rare. We present a rare case of a ...

  1. Idiopathic granulomatous lobular mastitis.

    Science.gov (United States)

    Pereira, Frederick A; Mudgil, Adarsh V; Macias, Edgar S; Karsif, Karen

    2012-02-01

    Idiopathic granulomatous lobular mastitis (IGLM) is a rare breast condition with prominent skin findings. It is typically seen in young parous women. Painful breast masses, draining sinuses, scarring, and breast atrophy are the main clinical manifestations. IGLM can resemble a variety of other inflammatory and neoplastic processes of the breast. It is thought to result from obstruction and rupture of breast lobules. Extravasated breast secretions then induce an inflammatory reaction. Corynebacteria have also been implicated in the pathogenesis. Treatment is surgical, but systemic corticosteroids, methotrexate, and antibiotics also play a role. © 2012 The International Society of Dermatology.

  2. Detection of equine herpesvirus in horses with idiopathic keratoconjunctivitis and comparison of three sampling techniques.

    Science.gov (United States)

    Hollingsworth, Steven R; Pusterla, Nicola; Kass, Philip H; Good, Kathryn L; Brault, Stephanie A; Maggs, David J

    2015-09-01

    To determine the role of equine herpesvirus (EHV) in idiopathic keratoconjunctivitis in horses and to determine whether sample collection method affects detection of EHV DNA by quantitative polymerase chain reaction (qPCR). Twelve horses with idiopathic keratoconjunctivitis and six horses without signs of ophthalmic disease. Conjunctival swabs, corneal scrapings, and conjunctival biopsies were collected from 18 horses: 12 clinical cases with idiopathic keratoconjunctivitis and six euthanized controls. In horses with both eyes involved, the samples were taken from the eye judged to be more severely affected. Samples were tested with qPCR for EHV-1, EHV-2, EHV-4, and EHV-5 DNA. Quantity of EHV DNA and viral replicative activity were compared between the two populations and among the different sampling techniques; relative sensitivities of the sampling techniques were determined. Prevalence of EHV DNA as assessed by qPCR did not differ significantly between control horses and those with idiopathic keratoconjunctivitis. Sampling by conjunctival swab was more likely to yield viral DNA as assessed by qPCR than was conjunctival biopsy. EHV-1 and EHV-4 DNA were not detected in either normal or IKC-affected horses; EHV-2 DNA was detected in two of 12 affected horses but not in normal horses. EHV-5 DNA was commonly found in ophthalmically normal horses and horses with idiopathic keratoconjunctivitis. Because EHV-5 DNA was commonly found in control horses and in horses with idiopathic keratoconjunctivitis, qPCR was not useful for the etiological diagnosis of equine keratoconjunctivitis. Conjunctival swabs were significantly better at obtaining viral DNA samples than conjunctival biopsy in horses in which EHV-5 DNA was found. © 2015 American College of Veterinary Ophthalmologists.

  3. Idiopathic intracranial hypertension with altered consciousness in a ...

    African Journals Online (AJOL)

    Idiopathic intracranial hypertension (IIH) is a clinical condition of increased intracranial pressure (ICP) without an obvious underlying pathological brain lesion. It is usually characterized by headache, neck pain, vomiting, visual disturbances, papilledema, cranial nerve palsy or a combination of these signs and symptoms.

  4. Löffler's endomyocarditis in the idiopathic hypereosinophilic syndrome

    NARCIS (Netherlands)

    Corssmit, E. P.; Trip, M. D.; Durrer, J. D.

    1999-01-01

    The idiopathic hypereosinophilic syndrome (HES) is a leukoproliferative disorder characterized by sustained eosinophilia (> 1.5 x 10(9)/l) and (multi-)organ dysfunction caused by infiltration of eosinophils. Especially the heart is frequently affected. In this report, we describe 2 patients with HES

  5. Treatment of Resistant Idiopathic Hiccups with Pulse Radio ...

    African Journals Online (AJOL)

    2017-09-14

    Sep 14, 2017 ... Our case suggests that administration of gabapentin after pulsed radiofrequency might be effective for the treatment of persistent hiccups. KEYWORDS: Hiccups, persistent, pulse radio frequency. Treatment of Resistant Idiopathic Hiccups with Pulse Radio Frequency on Phrenic Nerve and Gabapentin: A ...

  6. Multiplex assessment of serum cytokine and chemokine levels in idiopathic morphea and vitamin K1-induced morphea.

    Science.gov (United States)

    Cox, Lori Ann; Webster, Guy F; Piera-Velazquez, Sonsoles; Jimenez, Sergio A

    2017-05-01

    The levels of 63 cytokines, chemokines, and growth factors were measured in the serum of four patients with idiopathic morphea and of one patient with vitamin K 1 -induced morphea employing a multiplex assay to identify the role of inflammatory/immunologic events in their pathogenesis. Full-thickness skin biopsies of affected skin were analyzed by histopathology. Luminex assays for 63 cytokines, chemokines, and growth factors were performed in the sera from four patients with idiopathic morphea and in two different samples of serum obtained in two separate occasions from one patient with vitamin K 1 -induced morphea. The serum values of numerous inflammatory cytokines and growth factors including IL-2, IL-4, IL-6, and IFNβ were markedly increased in the serum of patients with idiopathic morphea, whereas, these values were normal in the serum of the patient with vitamin K 1 -induced morphea. In contrast, serum eotaxin levels were greater than threefold higher in the patient with vitamin K 1 -induced morphea compared to patients with idiopathic morphea. The results demonstrated remarkable increases in the levels of numerous cytokines and chemokines in the serum samples of all patients with idiopathic morphea indicative of a prominent role of inflammatory/immunologic events in its pathogenesis. The results also showed statistically significant differences between idiopathic morphea and vitamin K 1 -induced morphea suggesting that their development involves different pathogenetic mechanisms.

  7. Cortical thickness in children receiving intensive therapy for idiopathic apraxia of speech.

    Science.gov (United States)

    Kadis, Darren S; Goshulak, Debra; Namasivayam, Aravind; Pukonen, Margit; Kroll, Robert; De Nil, Luc F; Pang, Elizabeth W; Lerch, Jason P

    2014-03-01

    Children with idiopathic apraxia experience difficulties planning the movements necessary for intelligible speech. There is increasing evidence that targeted early interventions, such as Prompts for Restructuring Oral Muscular Phonetic Targets (PROMPT), can be effective in treating these disorders. In this study, we investigate possible cortical thickness correlates of idiopathic apraxia of speech in childhood, and changes associated with participation in an 8-week block of PROMPT therapy. We found that children with idiopathic apraxia (n = 11), aged 3-6 years, had significantly thicker left supramarginal gyri than a group of typically-developing age-matched controls (n = 11), t(20) = 2.84, p ≤ 0.05. Over the course of therapy, the children with apraxia (n = 9) experienced significant thinning of the left posterior superior temporal gyrus (canonical Wernicke's area), t(8) = 2.42, p ≤ 0.05. This is the first study to demonstrate experience-dependent structural plasticity in children receiving therapy for speech sound disorders.

  8. A systematic approach for the diagnosis and treatment of idiopathic peptic ulcers

    Science.gov (United States)

    Chung, Chen-Shuan; Chiang, Tsung-Hsien; Lee, Yi-Chia

    2015-01-01

    An idiopathic peptic ulcer is defined as an ulcer with unknown cause or an ulcer that appears to arise spontaneously. The first step in treatment is to exclude common possible causes, including Helicobacter pylori infection, infection with other pathogens, ulcerogenic drugs, and uncommon diseases with upper gastrointestinal manifestations. When all known causes are excluded, a diagnosis of idiopathic peptic ulcer can be made. A patient whose peptic ulcer is idiopathic may have a higher risk for complicated ulcer disease, a poorer response to gastric acid suppressants, and a higher recurrence rate after treatment. Risk factors associated with this disease may include genetic predisposition, older age, chronic mesenteric ischemia, smoking, concomitant diseases, a higher American Society of Anesthesiologists score, and higher stress. Therefore, the diagnosis and management of emerging disease should systematically explore all known causes and treat underlying disease, while including regular endoscopic surveillance to confirm ulcer healing and the use of proton-pump inhibitors on a case-by-case basis. PMID:26354049

  9. A sigmoidal fit for pressure-volume curves of idiopathic pulmonary fibrosis patients on mechanical ventilation: clinical implications

    Directory of Open Access Journals (Sweden)

    Juliana C. Ferreira

    2011-01-01

    Full Text Available OBJECTIVE: Respiratory pressure-volume curves fitted to exponential equations have been used to assess disease severity and prognosis in spontaneously breathing patients with idiopathic pulmonary fibrosis. Sigmoidal equations have been used to fit pressure-volume curves for mechanically ventilated patients but not for idiopathic pulmonary fibrosis patients. We compared a sigmoidal model and an exponential model to fit pressure-volume curves from mechanically ventilated patients with idiopathic pulmonary fibrosis. METHODS: Six idiopathic pulmonary fibrosis patients and five controls underwent inflation pressure-volume curves using the constant-flow technique during general anesthesia prior to open lung biopsy or thymectomy. We identified the lower and upper inflection points and fit the curves with an exponential equation, V = A-B.e-k.P, and a sigmoid equation, V = a+b/(1+e-(P-c/d. RESULTS: The mean lower inflection point for idiopathic pulmonary fibrosis patients was significantly higher (10.5 ± 5.7 cm H2O than that of controls (3.6 ± 2.4 cm H2O. The sigmoidal equation fit the pressure-volume curves of the fibrotic and control patients well, but the exponential equation fit the data well only when points below 50% of the inspiratory capacity were excluded. CONCLUSION: The elevated lower inflection point and the sigmoidal shape of the pressure-volume curves suggest that respiratory system compliance is decreased close to end-expiratory lung volume in idiopathic pulmonary fibrosis patients under general anesthesia and mechanical ventilation. The sigmoidal fit was superior to the exponential fit for inflation pressure-volume curves of anesthetized patients with idiopathic pulmonary fibrosis and could be useful for guiding mechanical ventilation during general anesthesia in this condition.

  10. Acute surgical management in idiopathic intracranial hypertension.

    LENUS (Irish Health Repository)

    Zakaria, Zaitun

    2012-01-01

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

  11. Idiopathic thrombocytopenic purpura

    Directory of Open Access Journals (Sweden)

    L Kayal

    2014-01-01

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

  12. Candidate gene analysis and exome sequencing confirm LBX1 as a susceptibility gene for idiopathic scoliosis

    DEFF Research Database (Denmark)

    Grauers, Anna; Wang, Jingwen; Einarsdottir, Elisabet

    2015-01-01

    samples from 100 surgically treated idiopathic scoliosis patients. Novel or rare missense, nonsense, or splice site variants were selected for individual genotyping in the 1,739 cases and 1,812 controls. In addition, the 5'UTR, noncoding exon and promoter regions of LBX1, not covered by exome sequencing...... by exome sequencing after filtration and an initial genotyping validation. However, we could not verify any association to idiopathic scoliosis in the large cohort of 1,739 cases and 1,812 controls. We did not find any variants in the 5'UTR, noncoding exon and promoter regions of LBX1. CONCLUSIONS: Here...... that are significantly associated with idiopathic scoliosis in Asian and Caucasian populations, rs11190870 close to the LBX1 gene being the most replicated finding. PURPOSE: The aim of the present study was to investigate the genetics of idiopathic scoliosis in a Scandinavian cohort by performing a candidate gene study...

  13. q-deformed Weinberg-Salam model and q-deformed Maxwell equations

    International Nuclear Information System (INIS)

    Alavi, S.A.; Sarbishaei, M.; Mokhtari, A.

    2000-01-01

    We study the q-deformation of the gauge part of the Weinberg-Salam model and show that the q-deformed theory involves new interactions. We then obtain q-deformed Maxwell equations from which magnetic monopoles appear naturally. (author)

  14. Non-HLA gene polymorphisms in juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Alberdi-Saugstrup, M.; Enevold, C.; Zak, M.

    2017-01-01

    Objective: To test the hypothesis that non-HLA single-nucleotide polymorphisms (SNPs) associated with the risk of juvenile idiopathic arthritis (JIA) are risk factors for an unfavourable disease outcome at long-term follow-up. Methods: The Nordic JIA cohort is a prospective multicentre study cohort...

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

    Science.gov (United States)

    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 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.

  16. Graves' disease and idiopathic intracranial hypertension

    OpenAIRE

    Manish Gutch; Annesh Bhattacharjee; Sukriti Kumar; Durgesh Pushkar

    2017-01-01

    Idiopathic intracranial hypertension (IIH) is a central nervous system disorder characterized by raised intracranial pressure with normal cerebrospinal fluid composition and absence of any structural anomaly on neuroimaging. Among all endocrine disorders associated with the development of IIH, the association of hyperthyroidism and IIH is very rare with few cases reported till date. Thyroid disturbances have a unique association with IIH. Hypo- and hyper-thyroidism have been reported in assoc...

  17. Bell's palsy before Bell: Evert Jan Thomassen à Thuessink and idiopathic peripheral facial paralysis.

    Science.gov (United States)

    van de Graaf, R C; IJpma, F F A; Nicolai, J-P A; Werker, P M N

    2009-11-01

    Bell's palsy is the eponym for idiopathic peripheral facial paralysis. It is named after Sir Charles Bell (1774-1842), who, in the first half of the nineteenth century, discovered the function of the facial nerve and attracted the attention of the medical world to facial paralysis. Our knowledge of this condition before Bell's landmark publications is very limited and is based on just a few documents. In 1804 and 1805, Evert Jan Thomassen à Thuessink (1762-1832) published what appears to be the first known extensive study on idiopathic peripheral facial paralysis. His description of this condition was quite accurate. He located several other early descriptions and concluded from this literature that, previously, the condition had usually been confused with other afflictions (such as 'spasmus cynicus', central facial paralysis and trigeminal neuralgia). According to Thomassen à Thuessink, idiopathic peripheral facial paralysis and trigeminal neuralgia were related, being different expressions of the same condition. Thomassen à Thuessink believed that idiopathic peripheral facial paralysis was caused by 'rheumatism' or exposure to cold. Many aetiological theories have since been proposed. Despite this, the cold hypothesis persists even today.

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

    Directory of Open Access Journals (Sweden)

    V.I. Petrov

    2010-06-01

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

  19. Assessment of cardiac neuronal function with iodine-123 MIBG scintigraphy in children with idiopathic dilated cardiomyopathy

    International Nuclear Information System (INIS)

    Maunoury, Ch.; Sebahoun, St.; Hallaj, I.; Barritault, L.; Acar, Ph.; Sidi, D.; Kachaner, J.; Agostini, D.; Bouvard, G.

    2000-01-01

    The I-123 MIBG cardiac scintigraphy can assess norepinephrine uptake. It has been showed that cardiac adrenergic neuronal function was impaired in adults with dilated cardiomyopathy. The aim of this prospective study was to assess cardiac neuronal function in children with idiopathic dilated cardiomyopathy (DCM) and to compare cardiac uptake of I-123 MIBG with left ventricular ejection fraction (LVEF). We studied 26 consecutive patients with idiopathic DCM, aged 44 ± 50 months, and 12 controls, aged 49 ±65 months. A planar scintigraphy was performed in all children 4 hours after intravenous injection of 20 to 75 MBq of I-123 MIBG. A static anterior view was acquired for 10 minutes. Cardiac uptake of I-123 MIBG was expressed as the heart to mediastinum count ratio (HMR). Equilibrium radionuclide angiography was performed following a standard protocol. Cardiac uptake of I-123 MIBG was significantly decreased in patients with idiopathic DCM when compared with cardiac uptake in controls (172±34% vs 277±14%, P<0.0001. There was a good correlation between RCM and LVEF in patients with idiopathic DCM (y = 2.5 x +113.3, r = 0.80, P < 0.0001). In conclusion, cardiac neuronal function was impaired in children with idiopathic DCM and related to impairment of left ventricular function. (author)

  20. Idiopathic hypertrophic subaortic stenosis. I

    International Nuclear Information System (INIS)

    Kvam, G.

    1980-01-01

    Biplane left ventricular cineangiographies in 4 patients with typical obstructive idiopathic hypertrophic subaortic stenosis (IHSS) and in control patients with normal left ventricles were analysed. In the protruding hypertrophic muscular interventricular septum of IHSS a markedly reduced shortening occurs in either direction during the systolic contraction. It does not bend towards the right ventricle. It is suggested that the septum of IHSS acts as a suspender during the systolic contraction, thereby accounting for the fast stroke volume ejection and the high ejection fraction of IHSS. (Auth.)

  1. Prevalence of idiopathic normal-pressure hydrocephalus in the elderly population of a Japanese rural community

    International Nuclear Information System (INIS)

    Hiraoka, Kotaro; Meguro, Kenichi; Mori, Etsuro

    2008-01-01

    The prevalence of idiopathic normal-pressure hydrocephalus (NPH) in a community was investigated by retrospective analysis of data from a previous community-based study of 170 randomly selected elderly residents aged 65 years or older. Magnetic resonance (MR) images of the subjects were reviewed for the specific structural features of idiopathic NPH, i.e. ventricular enlargement and narrow cerebrospinal fluid (CSF) space at high convexity and high midline areas. The clinical features of idiopathic NPH, gait disturbance, urinary incontinence, and cognitive impairment, were evaluated on the basis of records of the subjects' neurological examinations, a health questionnaire, the Mini-Mental State Examination, and Clinical Dementia Rating. Thirteen of the 170 subjects showed lateral ventricular enlargement greater than 0.3 on Evans' index. Five subjects (2.9%) demonstrated both ventricular enlargement and narrow CSF space at the high convexity/midline. All five subjects with these MR imaging signs had cognitive impairment, one had gait disturbance, and one had urinary incontinence. The present study found 2.9% of community-dwelling elderly subjects showed radiological and clinical features consistent with idiopathic NPH. (author)

  2. Idiopathic granulomatous mastitis | Rubin | SA Journal of Radiology

    African Journals Online (AJOL)

    The rare condition of idiopathic granulomatous mastitis (IGM) is presented here, unusually, in a 54-year-old woman. IGM mimics breast carcinoma and further differentials include tuberculosis and fungal infections of the breast together with other chronic granulomatous conditions. Of note is its characteristic ultrasound ...

  3. Idiopathic Oesophageal Dysmotility Disorder: Stridor Secondary to Megaesophagus

    Directory of Open Access Journals (Sweden)

    B. G. Natesh

    2013-01-01

    Full Text Available We present an interesting case of an elderly lady who presented with stridor caused by megaesophagus secondary to an acquired idiopathic dysmotility disorder. We discuss the aetiology and management of megaesophagus secondary to this condition and how it differs from megaesophagus secondary to achalasia.

  4. Perioperative Management of Pregnant Women With Idiopathic Pulmonary Arterial Hypertension: An Observational Case Series Study From China.

    Science.gov (United States)

    Zhang, Jinglan; Lu, Jiakai; Zhou, Xiaorui; Xu, Xuefeng; Ye, Qing; Ou, Qitan; Li, Yanna; Huang, Jiapeng

    2018-03-07

    The mortality of pregnant women with idiopathic pulmonary arterial hypertension (PAH) is very high. There are limited data on the management of idiopathic PAH during pregnancy. The authors aimed to examine systematically the characteristics of parturient women with idiopathic PAH, to explore the adverse effects of idiopathic PAH on pregnancy outcomes, and to report the multidisciplinary perioperative management strategy from the largest comprehensive cardiac hospital in China. Observational case series study. Tertiary referral acute care hospital in Beijing, China. The cases of 17 consecutive pregnant idiopathic PAH patients undergoing abortion or parturition at Anzhen Hospital were reviewed retrospectively. Preoperative characteristics, anesthesia method, intensive care management, PAH-specific therapy, and maternal and neonatal outcomes were analyzed in this case series study. Maternal and neonatal outcomes were the main measures. The mean ages of the 17 parturient women with idiopathic PAH were 28.3 ± 5.4 years, and the mean systolic pulmonary arterial pressure was 97.9 ± 18.6 mmHg. Fifteen patients (88.2%) received PAH-specific therapy before delivery, including sildenafil, iloprost, and treprostinil. All except 1 parturient received epidural anesthesia for surgery due to an emergency Caesarean section. Three patients experienced pulmonary hypertension crisis that necessitated conversion to general anesthesia. Ten parturients underwent Caesarean delivery at a median gestational age of 31 weeks. Three patients developed acute pulmonary hypertensive crisis intraoperatively. Two patients underwent cardiopulmonary resuscitation and extracorporeal membrane oxygenation support. The maternal mortality was 17.6% (3/17). Of the 10 delivered neonates, 9 (90.0%) survived. The maternal mortality of the idiopathic PAH parturient was high in this case series from China. The authors applied epidural anesthesia, early management with multidisciplinary approaches, PAH

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

    Science.gov (United States)

    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

  6. A three-generation family with idiopathic facial palsy suggesting an autosomal dominant inheritance with high penetrance

    DEFF Research Database (Denmark)

    Larsen, Christian Grønhøj; Gyldenløve, Mette; Jønch, Aia Elise

    2015-01-01

    Idiopathic facial palsy (IFP), also known as Bell's palsy, is a common neurologic disorder, but recurrent and familial forms are rare. This case series presents a three-generation family with idiopathic facial palsy. The mode of inheritance of IFP has previously been suggested as autosomal dominant...

  7. Fracture-associated and idiopathic subchondral vertebral lesions: a magnetic resonance study in autopsy specimens with histologic correlation

    International Nuclear Information System (INIS)

    Peters, C.A.; Berg, B.C. vande; Lecouvet, F.E.; Malghem, J.; Galand, C.

    2009-01-01

    The aim of this study was to describe and compare the magnetic resonance (MR) and histological appearance of subchondral vertebral lesions that are idiopathic or that develop with vertebral fractures. T1- and T2-weighted spin-echo images and radiographs were obtained in 81 cadaveric spine specimens. All subchondral vertebral lesions that were considered to be idiopathic or associated with vertebral end plate fractures were selected. Lesions due to growth disturbance were excluded. Radiographs and MR images were analyzed in consensus by two radiologists, and sampled specimens were analyzed by a pathologist. Eleven idiopathic and ten fracture-associated vertebral lesions were available. On T1-weighted images, all lesion signal intensity was low and homogeneous. On T2-weighted images, all idiopathic lesions showed a heterogeneous signal with a central low or intermediate signal component and a peripheral high or intermediate component. All but one fracture-related lesions showed a homogeneous intermediate to high signal intensity. Histological analysis of idiopathic lesions showed a central acellular fibrous connective tissue in all cases surrounded by loose connective tissue in nine cases. Herniated disk material and cartilage metaplasia were found in one lesion only. Fracture-associated lesions contained herniated disk material, necrotic tissue, and loose connective tissue with a peripheral component of loose fibrovascular connective tissue in four cases only. MR and histological appearance of idiopathic and fracture-associated subchondral vertebral lesions differ, suggesting that they might have a different origin. (orig.)

  8. A Case Study on Idiopathic Orbital Pseudotumor: Surgery and ...

    African Journals Online (AJOL)

    one year revealed a gradual improvement in the vision of all the patients. The VA of the right eye for all the patients ... Hunt syndrome, pituitary histiocytosis, idiopathic meningitis ... computer tomography (CT) and magnetic resonance imaging ...

  9. Idiopathic intracranial hypertension, hormones, and 11β-hydroxysteroid dehydrogenases

    DEFF Research Database (Denmark)

    Markey, Keira A; Uldall, Maria; Botfield, Hannah

    2016-01-01

    Idiopathic intracranial hypertension (IIH) results in raised intracranial pressure (ICP) leading to papilledema, visual dysfunction, and headaches. Obese females of reproductive age are predominantly affected, but the underlying pathological mechanisms behind IIH remain unknown. This review provi...

  10. Positron emission tomography suggests that the rate of progression of idiopathic parkinsonism is slow

    International Nuclear Information System (INIS)

    Bhatt, M.H.; Snow, B.J.; Martin, W.R.; Pate, B.D.; Ruth, T.J.; Calne, D.B.

    1991-01-01

    The authors performed sequential positron emission tomography scans with 6-[18F]fluoro-L-dopa in 9 patients with idiopathic parkinsonism and 7 age-matched normal control subjects to compare changes in the nigrostriatal dopaminergic pathway over time. The mean interval between the scans was 3.3 years for the group with idiopathic parkinsonism and 3.9 years for the control subjects. The scans were analyzed by calculating the ratio of striatal to background radioactivity. Both groups showed statistically significant reductions of striatal uptake over the interval. The rate of decrease was almost identical in each group (p = 0.6). They infer that the usual rate of loss of integrity of the dopaminergic nigrostriatal pathway in patients with idiopathic parkinsonism is slow and the rate of change between the two groups was comparable

  11. Discrete potentials guided radiofrequency ablation for idiopathic outflow tract ventricular arrhythmias.

    Science.gov (United States)

    Liu, Enzhao; Xu, Gang; Liu, Tong; Ye, Lan; Zhang, Qitong; Zhao, Yanshu; Li, Guangping

    2015-03-01

    Discrete potentials (DPs) have been recorded and targeted as the site of ablation of the outflow tract arrhythmias. The aim of the present study was to investigate the significance of DPs with respect to mapping and ablation for idiopathic outflow tract premature ventricular contractions (PVCs) or ventricular tachycardias (VTs). Seventeen consecutive patients with idiopathic right or left ventricular outflow tract PVCs/VTs who underwent radiofrequency catheter ablation were included. Intracardiac electrograms during the mapping and ablation were analysed. During sinus rhythm, sharp high-frequency DPs that displayed double or multiple components were recorded following or buried in the local ventricular electrograms in all of the 17 patients, peak amplitude 0.51 ± 0.21 mV. The same potential was recorded prior to the local ventricular potential of the PVCs/VTs. Spontaneous reversal of the relationship of the DPs to the local ventricular electrogram during the arrhythmias was noted. The DPs were related to a region of low voltage showed by intracardiac high-density contact mapping. At the sites with DPs, lower unipolar and bipolar ventricular voltage of sinus beats were noted compared with the adjacent regions without DPs (unipolar: 6.1 ± 1.8 vs. 8.3 ± 2.3 mV, P Discrete potentials were not present in seven controls. Discrete potentials and related low-voltage regions were common in idiopathic outflow tract ventricular arrhythmias. Discrete potential- and substrate-guided ablation strategy will help to reduce the recurrence of idiopathic outflow tract arrhythmias. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2014. For permissions please email: journals.permissions@oup.com.

  12. Introversion, the prevalent trait of adolescents with idiopathic scoliosis: an observational study

    Directory of Open Access Journals (Sweden)

    Elisabetta D’Agata

    2017-11-01

    Full Text Available Abstract Background A large number of studies about adolescents with idiopathic scoliosis focus on health-related quality of life (HRQOL. However, only a few articles aim at evaluating the personality of these patients. Therefore, the purpose of the present research is to assess the personality traits of adolescents with idiopathic scoliosis and their relationship with HRQOL. Our hypothesis is that adolescents with idiopathic scoliosis present the principal personality trait of introversion, defined as self-reliance and inhibition in social relationships. Methods This was a cross-sectional study. The examined group consisted of 43 patients (only 4 boys, mean age = 14.3 (SD = 2.23. On the day of the visit, HRQOL tools (Scoliosis Research Society-22 Questionnaire (SRS-22 and Trunk Appearance Perception Scale (TAPS and a personality test (16 Personality Factors-Adolescent Personality Questionnaire (16PF-APQ were completed; in addition, a posterior-anterior radiography was performed. Correlations among demographic and medical data and HRQOL and personality tests were assessed. Results Results for SRS-22 were as follows: Function 4.5 (SD = .4, Pain 4.3 (SD = .5, Self-image 3.6 (SD = .7, Mental Health 3.8. (SD = .7, and Subtotal 4.2 (SD = .7. Mean TAPS was 3.5 (SD = .6. In personality, the lowest values were assessed for Extroversion (M = 29.4, SD = 24.7 and Self-reliance (M = 71, SD = 25.3. Independence was negatively related to Self-image (r = −.51, Mental Health (r = −.54, and Subtotal SRS-22 (r = −.60 (p < .01. Conclusions Adolescents with idiopathic scoliosis presented a common style of personality, characterized by social inhibition (introversion, preference for staying alone, and being self-sufficient (self-reliance. Specific programs in promoting social abilities may help adolescent patients with idiopathic scoliosis in finding a way to express themselves and to become more sociable. Correlational studies

  13. MRI evaluation of multifidus muscles in adolescent idiopathic scoliosis

    International Nuclear Information System (INIS)

    Chan Yu-Leung; King, A.D.; Griffith, J.F.; Metreweli, C.; Cheng, J.C.Y.; Guo Xia

    1999-01-01

    Background. The role of the multifidus muscles in the initiation and progression of curve in adolescent idiopathic scoliosis is not fully understood and controversy exists as to the side of the abnormality. Objective. To evaluate on MRI the multifidus muscles at the apex of the major curve in adolescent idiopathic scoliosis to ascertain if the multifidus muscles on the convex or concave side are abnormal and the relationship to curve severity. Materials and methods. Forty-six patients with adolescent idiopathic scoliosis, separated into two groups, were studied using a 1.5-T MR scanner with the synergy spine coil, employing a modified STIR (short tau inversion recovery) axial sequence obtained at the apex of the major scoliotic curve. Results. No hyperintense signal change was demonstrated in the convex side multifidus muscles in any patient. In group I, 16 of 18 patients with severe or rapidly progressive curve showed increase in signal intensity in the multifidus muscle on the concave side of the apex of the curve. In group II, of the 15 patients with mild curve (Cobb angle 10-30 ), 4 had increased signal intensity in the multifidus muscle on the concave side; of the 13 with more severe curve (Cobb angle greater than 30 ), 10 had increase in multifidus signal intensity on the concave side. Conclusions. The concave-side multifidus muscle at the apex of a scoliotic curve was morphologically abnormal. A significant association between abnormal signal change and curve severity was also established. (orig.)

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

    NARCIS (Netherlands)

    Engelbert, Raoul; Gorter, Jan Willem; Uiterwaal, Cuno; van de Putte, Elise; Helders, Paul

    2011-01-01

    Idiopathic Toe Walking (ITW) is present in children older than 3 years of age still walking on their toes without signs of neurological, orthopaedic or psychiatric diseases. ITW has been estimated to occur in 7% to 24% of the childhood population. To study associations between Idiopathic Toe Walking

  15. The pattern of juvenile idiopathic arthritis; a retrospective Egyptian ...

    African Journals Online (AJOL)

    Ehab

    subtype specially at rural areas which differed from Western and Gulf countries pattern. ... version 18) and the following statistical measures ..... the results of Abdwani et al27 at 2015 (an Omani ... Ravelli A, Martini A. Juvenile idiopathic arthritis.

  16. Clubfoot

    Science.gov (United States)

    ... Departments & specialties Preparing for your appointment Self-management Advertisement Mayo Clinic does not endorse companies or products. ... a Job Site Map About This Site Twitter Facebook Google YouTube Pinterest Mayo Clinic is a not- ...

  17. An Update on Idiopathic Intracranial Hypertension

    OpenAIRE

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

    2010-01-01

    Idiopathic intracranial hypertension (IIH) is a condition of unknown etiology often encountered in neurologic practice. It produces non-localizing symptoms and signs of raised intracranial pressure and, when left untreated, can result in severe irreversible visual loss. It most commonly occurs in obese women of childbearing age, but it can also occur in children, men, non-obese adults, and older adults. While it is frequently associated with obesity, it can be associated with other conditions...

  18. Case of twin pregnancy complicated by idiopathic thrombocytopenic ...

    African Journals Online (AJOL)

    2016-05-09

    May 9, 2016 ... Idiopathic thrombocytopenic purpura (ITP) is an acquired thrombocytopenia without other clear cause of thrombocytopenia. It is not common in a singleton pregnancy and less common in twin pregnancy. We report a 33‑year‑old ITP pluripara whose first pregnancy was uneventful. She carried twin ...

  19. Progressive multifocal leucoencephalopathy in a patient with idiopathic CD4+ lymphocytopenia.

    LENUS (Irish Health Repository)

    Moloney, F

    2012-03-01

    Progressive multifocal leucoencephalopathy (PML) is an opportunistic, demyelinating neurological disease caused by reactivation of the JC polyomavirus. PML occurs almost exclusively in immunosuppressed individuals, with only isolated case reports of PML occurring in patients without apparent immunosuppression. Idiopathic CD4+ lymohocytopenia (ICL) is a syndrome defined by the Centre for Disease Control and Prevention as a CD4+ count <300 cells\\/uL or <20% of total T cell count on >1 occasion, with no evidence of human immunodeficiency virus (HIV) infection, and the absence of other known immunodeficiency or therapy associated with lymphocytopenia. We describe a case of PML occurring in a patient with idiopathic CD4+ lymphocytopenia.

  20. Comparison of plasma, liver, and skeletal muscle carnitine concentrations in cats with idiopathic hepatic lipidosis and in healthy cats.

    Science.gov (United States)

    Jacobs, G; Cornelius, L; Keene, B; Rakich, P; Shug, A

    1990-09-01

    Concentrations of total, free, and esterified carnitine were determined in plasma, liver, and skeletal muscle from cats with idiopathic hepatic lipidosis and compared with values from healthy cats. The mean concentrations of plasma, liver, and skeletal muscle total carnitine; plasma and skeletal muscle free carnitine; and plasma and liver esterified carnitine were greater (P less than 0.05) in cats with idiopathic hepatic lipidosis than in control cats. The mean for the ratio of free/total carnitine in plasma and liver was lower (P less than 0.05) in cats with idiopathic hepatic lipidosis than in control cats. These data suggest that carnitine deficiency does not contribute to the pathogenesis of feline idiopathic hepatic lipidosis.

  1. [A case of cutaneous extramedullary hematopoiesis associated with idiopathic myelofibrosis].

    Science.gov (United States)

    Corella, F; Barnadas, M A; Bordes, R; Curell, R; Espinosa, I; Vergara, C; Alomar, A

    2008-05-01

    Cutaneous extramedullary hematopoiesis is a rare manifestation of chronic myeloproliferative processes, mainly chronic idiopathic myelofibrosis. In adults, it manifests as macules, papules, nodules, and ulcers on the trunk. The lesions usually appear soon after diagnosis and the possibility of a relationship between splenectomy and the appearance of extramedullary foci of hematopoiesis is still debated. Diagnosis is based on histopathology showing an infiltrate with different combinations of myeloid and erythroid cell precursors and megakaryocytes. Symptomatic treatment is provided alongside treatment of the underlying disease. We report a new case associated with chronic idiopathic myelofibrosis in which foci of cutaneous extramedullary hematopoiesis were observed 9 years after initial diagnosis. The lesions were progressive and the patient went on to develop acute myeloid leukemia.

  2. Deformation twinning in a creep-deformed nanolaminate structure

    International Nuclear Information System (INIS)

    Hsiung, Luke L

    2010-01-01

    The underlying mechanism of deformation twinning occurring in a TiAl-(γ)/Ti 3 Al-(α 2 ) nanolaminate creep deformed at elevated temperatures has been studied. Since the multiplication and propagation of lattice dislocations in both γ and α 2 thin lamellae are very limited, the total flow of lattice dislocations becomes insufficient to accommodate the accumulated creep strains. Consequently, the movement of interfacial dislocations along the laminate interfaces, i.e., interface sliding, becomes an alternative deformation mode of the nanolaminate structure. Pile-ups of interfacial dislocations occur when interfacial ledges and impinged lattice dislocations act as obstacles to impede the movement of interfacial dislocations. Deformation twinning can accordingly take place to relieve a stress concentration resulting from the pile-up of interfacial dislocations. An interface-controlled twinning mechanism driven by the pile-up and dissociation of interfacial dislocations is accordingly proposed.

  3. Deformation twinning in a creep-deformed nanolaminate structure

    Science.gov (United States)

    Hsiung, Luke L.

    2010-10-01

    The underlying mechanism of deformation twinning occurring in a TiAl-(γ)/Ti3Al-(α2) nanolaminate creep deformed at elevated temperatures has been studied. Since the multiplication and propagation of lattice dislocations in both γ and α2 thin lamellae are very limited, the total flow of lattice dislocations becomes insufficient to accommodate the accumulated creep strains. Consequently, the movement of interfacial dislocations along the laminate interfaces, i.e., interface sliding, becomes an alternative deformation mode of the nanolaminate structure. Pile-ups of interfacial dislocations occur when interfacial ledges and impinged lattice dislocations act as obstacles to impede the movement of interfacial dislocations. Deformation twinning can accordingly take place to relieve a stress concentration resulting from the pile-up of interfacial dislocations. An interface-controlled twinning mechanism driven by the pile-up and dissociation of interfacial dislocations is accordingly proposed.

  4. Assessment of disease activity in juvenile idiopathic arthritis. The number and the size of joints matter

    DEFF Research Database (Denmark)

    Berntson, Lillemor; Wernroth, Lisa; Fasth, Anders

    2007-01-01

    Variables for assessment of disease activity of juvenile idiopathic arthritis (JIA) were studied, in order to develop a disease activity score for children with JIA.......Variables for assessment of disease activity of juvenile idiopathic arthritis (JIA) were studied, in order to develop a disease activity score for children with JIA....

  5. Outcomes of recalcitrant idiopathic epistaxis in children: Septoplasty as a surgical treatment.

    Science.gov (United States)

    Levi, Jessica M; McKee-Cole, Katherine M; Barth, Patrick C; Brody, Robert M; Reilly, James S

    2016-12-01

    Our objective was to measure short- and long-term outcomes of children presenting with recalcitrant idiopathic epistaxis. The study was an 11-year (2000-2011) retrospective chart review of children evaluated and treated for epistaxis. A retrospective review of patients with diagnostic International Classification of Diseases, Ninth Revision code 784.7 (epistaxis) and 21.5/21.88 (septoplasty) was completed reviewing age at presentation, type of surgery, and number of bleeding events prior to and after surgery. In our cohort, almost 100% of children with idiopathic recurrent epistaxis responded to topical treatments. About 0.2% were refractory (20/9239), and 90% of those (18/20) resolved with either a traditional septoplasty (14/20) or modified septoplasty (6/20) without cartilage excision, with a mean follow-up of 35 months. One of the patients who did not show resolution was found to have Von Willebrand disease, which likely contributed to this outcome. Septoplasty surgery, with or without cartilage removal, appears beneficial for refractory idiopathic epistaxis in children. 4 Laryngoscope, 126:2833-2837, 2016. © 2016 The American Laryngological, Rhinological and Otological Society, Inc.

  6. Genetic architecture distinguishes systemic juvenile idiopathic arthritis from otherforms of juvenile idiopathic arthritis: clinical and therapeutic implications

    OpenAIRE

    Ombrello, Michael J.; Arthur, Victoria L.; Remmers, Elaine F.; Hinks, Anne; Tachmazidou, Ioanna; Grom, Alexei A.; Foell, Dirk; Martini, Alberto; Gattorno, Marco; Ozen, Seza; Prahalad, Sampath; Zeft, Andrew S.; Bohnsack, John F.; Ilowite, Norman T.; Mellins, Elizabeth D.

    2016-01-01

    Objectives: Juvenile idiopathic arthritis (JIA) is a heterogeneous group of conditions unified by the presence of chronic childhood arthritis without an identifiable cause. Systemic JIA (sJIA) is a rare form of JIA characterized by systemic inflammation. sJIA is distinguished from other forms of JIA by unique clinical features and treatment responses that are similar to autoinflammatory diseases. However approximately half of children with sJIA develop destructive, longstanding arthritis that...

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

    Science.gov (United States)

    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.

  8. Structural white matter abnormalities in patients with idiopathic dystonia

    NARCIS (Netherlands)

    Bonilha, Leonardo; de Vries, Paulien M.; Vincent, Diana J.; Rorden, Chris; Morgan, Paul S.; Hurd, Mark W.; Besenski, Nada; Bergmann, Kenneth J.; Hinson, Vanessa K.

    2007-01-01

    We investigated whether structural white matter abnormalities, in the form of disruption of axonal coherence and integrity as measured with diffusion tensor imaging (DTI), constitute an underlying pathological mechanism of idiopathic dystonia (ID), independent of genotype status. We studied seven

  9. Evaluation of classification systems for nonspecific idiopathic orbital inflammation

    NARCIS (Netherlands)

    Bijlsma, Ward R.; van 't Hullenaar, Fleur C.; Mourits, Maarten P.; Kalmann, Rachel

    2012-01-01

    To systematically analyze existing classification systems for idiopathic orbital inflammation (IOI) and propose and test a new best practice classification system. A systematic literature search was conducted to find all studies that described and applied a classification system for IOI.

  10. Prognosis in canine idiopathic immune-mediated haemolytic anaemia

    NARCIS (Netherlands)

    Piek, C.J.

    2011-01-01

    Canine idiopathic immune-mediated haemolytic anaemia (iIMHA) is one of the most frequently occurring immune-mediated diseases in dogs. A gel-based Coombs' test was shown to perform equally well as a classical Coombs' test. Since the gel-based Coombs' test can be commercially produced and is easy and

  11. The Brazilian version of the SRS-22r questionnaire for idiopathic scoliosis

    Directory of Open Access Journals (Sweden)

    Paula M. F. Camarini

    2013-10-01

    Full Text Available BACKGROUND: The SRS-22r questionnaire is a well-accepted instrument used to measure health-related quality of life in patients with idiopathic scoliosis. No validated tool exists in Brazil for idiopathic scoliosis, and the use of the SRS-22r in non-English Laguage contries requires its transcultural adaptation. OBJECTIVE: The objective of this study was to culturally adapt the translated Brazilian version of the SRS-22r questionnaire and to determine its reliability using statistical tests for internal consistency and test-retest reliability. METHOD: The transcultural adaptation process was carried out according to the recommendations of the American Academy of Orthopedic Surgeons. The pre-final version was administered to 44 patients with idiopathic scoliosis. The mean age of the participants was 18.93 years and the mean curve magnitude was 54.6°. A subgroup of 30 volunteers completed the questionnaire a second time one week later to determine the scale's reproducibility. Internal consistency was determined using Cronbach's alpha coefficient, and the test-retest reliability was determined using the Intraclass Correlation Coefficient (ICC. RESULTS: No floor effects were observed using the Brazilian version of the SRS-22r. Ceiling effects were observed in the Pain and Satisfaction with Management domains. The internal consistency values were very good for 3 domains and good for 2 domains. The ICC values were excellent for all domains. CONCLUSIONS: The high values of internal consistency and ICC reproducibility suggest that this version of the questionnaire can be used in Brazilian patients with idiopathic scoliosis.

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

    Science.gov (United States)

    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.

  13. Vertebral derotation in adolescent idiopathic scoliosis causes hypokyphosis of the thoracic spine

    Directory of Open Access Journals (Sweden)

    Watanabe Kota

    2012-06-01

    Full Text Available Abstract Background The purpose of this study was to test the hypothesis that direct vertebral derotation by pedicle screws (PS causes hypokyphosis of the thoracic spine in adolescent idiopathic scoliosis (AIS patients, using computer simulation. Methods Twenty AIS patients with Lenke type 1 or 2 who underwent posterior correction surgeries using PS were included in this study. Simulated corrections of each patient’s scoliosis, as determined by the preoperative CT scan data, were performed on segmented 3D models of the whole spine. Two types of simulated extreme correction were performed: 1 complete coronal correction only (C method and 2 complete coronal correction with complete derotation of vertebral bodies (C + D method. The kyphosis angle (T5-T12 and vertebral rotation angle at the apex were measured before and after the simulated corrections. Results The mean kyphosis angle after the C + D method was significantly smaller than that after the C method (2.7 ± 10.0° vs. 15.0 ± 7.1°, p  Conclusions In the 3D simulation study, kyphosis was reduced after complete correction of the coronal and rotational deformity, but it was maintained after the coronal-only correction. These results proved the hypothesis that the vertebral derotation obtained by PS causes hypokyphosis of the thoracic spine.

  14. A case of idiopathic interstitial pneumonia

    OpenAIRE

    豊見山, 寛; 兼島, 洋; 下地, 克佳; 金城, 勇徳; 中富, 昌夫; 小張, 一峰; 松井, 克明; Tomiyama, Hiroshi; Kaneshima, Hiroshi; Shimoji, Katsuyoshi; Kinjo, Yutoku; Nakatomi, Masao; Kobari, Kazumine; Matsui, Katsuaki; 琉球大学医学部第一内科

    1982-01-01

    A case of idiopathic interstitial pneumonia was reported. A 53-year-old man was admitted to our hospital with his complaint of shortness of breath. On physical examination clubbing of the fingers was noticed and velcro rales was heard on the bilateral lower back. On laboratory data no remarkable finding was revealed. Chest roentgenogram showed diffuse reticulonodular shadow and small ring shadow of the bilateral lower lung fields. On pulmonary function test decline of VC and DLco was revealed...

  15. Idiopathic fibrosing pancreatitis: a rare cause of chronic obstructive jaundice in childhood

    International Nuclear Information System (INIS)

    Park, Hye Seong; Kim, Hyun Sook; Kim, Hack Hee; Kim, Ok Hwa; Kim, Choon Yul; Bahk, Yong Whee

    1992-01-01

    We report a 14-months-old infant who had obstructive jaundice caused by idiopathic fibrosing pancreatitis. Ultrasonography and abdominal computed tomography showed dilatation of the intrahepatic ducts, common bile duct, and the pancreatic duct. Diffuse swelling of the pancreas was also noted on CT. At laparotomy, the head portion of the pancreas revealed a stony hard consistency, and proliferation of fibrotic tissue was confirmed pathologically. Idiopathic fibrosing pancreatitis is a very rare disease entity in childhood, but should be considered in the differential diagnosis of obstructive jaundice in children who demonstrate bile duct and pancreatic duct dilatation and/or diffuse pancreas swelling

  16. Idiopathic Scrotal Hematoma in Neonate: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Bioku Muftau Jimoh

    2014-01-01

    Full Text Available Neonatal scrotal hematoma is a rare genitourinary emergency. Some cases have underlining aetiologic factors such as testicular torsion, adrenal hemorrhage, or birth trauma, and others are idiopathic. Previously, immediate scrotal exploration was considered imperative for diagnosis and treatment. With good imaging techniques, some patients are managed nonoperatively. We report a case of idiopathic scrotal hematoma in a neonate. He was managed conservatively with clinical and radiological follow-up. There was complete resolution of hematoma within two months, thus, avoiding unnecessary exploration.

  17. Handwriting difficulties in juvenile idiopathic arthritis: a pilot study

    NARCIS (Netherlands)

    Haberfehlner, Helga; Visser, Bart; Daffertshofer, Andreas; van Rossum, Marion Aj; Roorda, Leo D.; van der Leeden, Marike; Dekker, Joost; Hoeksma, Agnes F.

    2011-01-01

    The aim of the present study was to describe handwriting difficulties of primary school children with juvenile idiopathic arthritis (JIA), and to investigate possible correlations with hand function and writing performance. In a cross-sectional approach, 15 children with JIA and reported handwriting

  18. Disrupted thalamic prefrontal pathways in patients with idiopathic dystonia

    NARCIS (Netherlands)

    Bonilha, Leonardo; de Vries, Paulien M.; Hurd, Mark W.; Rorden, Chris; Morgan, Paul S.; Besenski, Nada; Bergmann, Kenneth J.; Hinson, Vanessa K.

    There are quantifiable abnormalities in water diffusion properties of the white matter in thalamic and prefrontal areas in patients with idiopathic dystonia (ID). However, it is unclear which pathways are disrupted in these patients. Using probabilistic tractography of high resolution DTI, we

  19. Fahr’s syndrome and idiopathic hypoparathyroidism: A case report

    Directory of Open Access Journals (Sweden)

    Marinković Dejan M.

    2017-01-01

    Full Text Available Introduction. Fahr´s syndrome is a rare, slowly progressive, neurodegenerative disorder, characterised by extensive, bilateral, and symmetrical basal ganglia calcification. It is associated with neuropsychiatric manifestations and gradually progressive cognitive impairment. Fahr's syndrome is the secondary form of brain calcification that is caused by various metabolic, infectious, or degenerative diseases. Case report. We presented a middle-aged male with Fahr's syndrome due to primary idiopathic hypoparathyroidism. Clinical diagnosis was based on signs and symptoms of hypocalcemia, progressive neuropsychiatric illnesses, laboratory evidence of hypoparathyroidism, and radiological signs of calcifications in the basal ganglia. The patient improved after only a few days of intravenous rehydration and calcium substitution, followed by oral supplemental calcitriol. Conclusion. Timely recognition of idiopathic and iatrogenic hypoparathyroidism allows appropriate treatment that can prevent the development and clinical manifestations of Fahr´s syndrome and potentially slow its progression.

  20. Idiopathic interstitial pneumonias. From classification to diagnostic work-up

    International Nuclear Information System (INIS)

    Mueller-Mang, C.; Bankier, A.; Stiebellehner, L.; Schmid, K.

    2007-01-01

    Idiopathic interstitial pneumonias (IIP) comprise seven entities with distinct histologic patterns. In their idiopathic form IIP are rare diseases. They are, nevertheless, considered prototypes of the much more common secondary interstitial pneumonias. The advent of high-resolution computed tomography (HRCT) has had a profound impact on the imaging of IIP, because the detailed delineation of the lung anatomy allows a close correlation between the histologic patterns of IIP and the CT features. On the basis of CT morphology and in the correct clinical context, the radiologist can achieve an accurate diagnosis in many cases. However, due to overlap between the various entities, complementary lung biopsy is recommended in virtually all cases. This article reviews the CT pattern of IIP and offers relevant clinical and histological information for the purpose of enabling the radiologist to understand and participate in the multidisciplinary concept of IIP. (orig.) [de

  1. Analysis of scapular muscle EMG activity in patients with idiopathic neck pain: a systematic review.

    Science.gov (United States)

    Castelein, Birgit; Cools, Ann; Bostyn, Emma; Delemarre, Jolien; Lemahieu, Trees; Cagnie, Barbara

    2015-04-01

    It is proposed that altered scapular muscle function can contribute to abnormal loading of the cervical spine. However, it is not clear if patients with idiopathic neck pain show altered activity of the scapular muscles. The aim of this paper was to systematically review the literature regarding the differences or similarities in scapular muscle activity, measured by electromyography ( = EMG), between patients with chronic idiopathic neck pain compared to pain-free controls. Case-control (neck pain/healthy) studies investigating scapular muscle EMG activity (amplitude, timing and fatigue parameters) were searched in Pubmed and Web of Science. 25 articles were included in the systematic review. During rest and activities below shoulder height, no clear differences in mean Upper Trapezius ( = UT) EMG activity exist between patients with idiopathic neck pain and a healthy control group. During overhead activities, no conclusion for scapular EMG amplitude can be drawn as a large variation of results were reported. Adaptation strategies during overhead tasks are not the same between studies. Only one study investigated timing of the scapular muscles and found a delayed onset and shorter duration of the SA during elevation in patients with idiopathic neck pain. For scapular muscle fatigue, no definite conclusions can be made as a wide variation and conflicting results are reported. Further high quality EMG research on scapular muscles (broader than the UT) is necessary to understand/draw conclusions on how scapular muscles react in the presence of idiopathic neck pain. Copyright © 2015 Elsevier Ltd. All rights reserved.

  2. Idiopathic granulomatous hypophysitis: clinical and imaging features

    Energy Technology Data Exchange (ETDEWEB)

    Vasile, M. [Service de Radiologie, Hopital Saint-Antoine, 75 - Paris (France); Marsot-Dupuch, K. [Service de Radiologie, Hopital Saint-Antoine, 75 - Paris (France); Kujas, M. [Service d`Histologie Embryologie Cytogenetique, Hopital Pitie-Salpetriere, 75 - Paris (France); Brunereau, L. [Service de Radiologie, Hopital Saint-Antoine, 75 - Paris (France); Bouchard, P. [Service d`Histologie Embryologie Cytogenetique, Hopital Pitie-Salpetriere, 75 - Paris (France); Comoy, J. [Service de Neurochirurgie, Hopital Kremlin Bicetre, 94 (France); Tubiana, J.M. [Service de Radiologie, Hopital Saint-Antoine, 75 - Paris (France)

    1997-01-01

    Idiopathic pituitary granuloma is a rare disorder similar to lymphocytic adenohypophysitis. Few cases have been reported. We report a new histologically case proven with MRI. The patterns of clinical and radiological presentation and the management of this disorder are discussed. MRI findings suggestive of this condition include an intensely enhancing pituitary mass, associated with dural enhancement. Steroid therapy may be suggested avoiding unnecessary surgery. (orig.)

  3. Adolescent idiopathic scoliosis treated with posteromedial translation: radiologic evaluation with a 3D low-dose system.

    Science.gov (United States)

    Ilharreborde, Brice; Sebag, Guy; Skalli, Wafa; Mazda, Keyvan

    2013-11-01

    Computed tomography can be used for three-dimensional (3D) evaluation of adolescent idiopathic scoliosis (AIS) patients, but at the expense of high radiation exposure, and with the limitation of being performed in the supine position. These drawbacks can now be avoided with low-dose stereoradiography, even in routine clinical use. The purpose of this study was to determine the 3D postoperative correction of AIS patients treated by posteromedial translation. Forty-nine consecutive patients operated for AIS (Lenke 1-4) using posteromedial translation were included. Corrections were evaluated preoperatively, postoperatively and after at least 2 years using the EOS imaging system. 3D angles were measured in the plane of maximum deformity. Mean number of levels fused and operative time were 13.5 ± 1 and 215 ± 25 min, respectively. Main thoracic, proximal thoracic, and lumbar curves corrections averaged 64.4 ± 18, 31 ± 10 and 69 ± 20 %, respectively. Mean T4-T12 kyphosis increased 18.8° ± 9° in the subgroup of hypokyphotic patients. Mean apical vertebral rotation reduction was 48.3 ± 20 %. Trunk height gain averaged 27.8 ± 14 mm. There was no pseudarthrosis or significant loss of correction in any plane during follow-up. Two patients (4 %) developed asymptomatic proximal junctional kyphosis, despite having normal thoracic kyphosis. Their sagittal balance was shifted posteriorly by 36 and 47 mm, respectively, by the operation, but revision surgery was not performed. Low-dose stereoradiography provided 3D reconstructions of the fused and unfused spine in routine clinical use. Postoperative 3D analysis showed that posteromedial translation enhanced sagittal balance correction, without sacrificing frontal or axial correction of the deformity.

  4. Transcranial magnetic stimulation in the semi-quantitative, pre-operative assessment of patients undergoing spinal deformity surgery.

    Science.gov (United States)

    Glasby, Michael A; Tsirikos, Athanasios I; Henderson, Lindsay; Horsburgh, Gillian; Jordan, Brian; Michaelson, Ciara; Adams, Christopher I; Garrido, Enrique

    2017-08-01

    To compare measurements of motor evoked potential latency stimulated either magnetically (mMEP) or electrically (eMEP) and central motor conduction time (CMCT) made pre-operatively in conscious patients using transcranial and intra-operatively using electrical cortical stimulation before and after successful instrumentation for the treatment of adolescent idiopathic scoliosis. A group initially of 51 patients with adolescent idiopathic scoliosis aged 12-19 years was evaluated pre-operatively in the outpatients' department with transcranial magnetic stimulation. The neurophysiological data were then compared statistically with intra-operative responses elicited by transcranial electrical stimulation both before and after successful surgical intervention. MEPs were measured as the cortically evoked compound action potentials of Abductor hallucis. Minimum F-waves were measured using conventional nerve conduction methods and the lower motor neuron conduction time was calculated and this was subtracted from MEP latency to give CMCT. Pre-operative testing was well tolerated in our paediatric/adolescent patients. No neurological injury occurred in any patient in this series. There was no significant difference in the values of mMEP and eMEP latencies seen pre-operatively in conscious patients and intra-operatively in patients under anaesthetic. The calculated quantities mCMCT and eCMCT showed the same statistical correlations as the quantities mMEP and eMEP latency. The congruency of mMEP and eMEP and of mCMCT and eCMCT suggests that these measurements may be used comparatively and semi-quantitatively for the comparison of pre-, intra-, and post-operative spinal cord function in spinal deformity surgery.

  5. Identification of a novel idiopathic epilepsy locus in Belgian Shepherd dogs

    DEFF Research Database (Denmark)

    Seppälä, Eija H.; Koskinen, Lotta L.E.; Gulløv, Christina Hedal

    2012-01-01

    Epilepsy is the most common neurological disorder in dogs, with an incidence ranging from 0.5% to up to 20% in particular breeds. Canine epilepsy can be etiologically defined as idiopathic or symptomatic. Epileptic seizures may be classified as focal with or without secondary generalization...... collected 159 cases and 148 controls and confirmed the presence of epilepsy through epilepsy questionnaires and clinical examinations. The MRI was normal while interictal EEG revealed abnormalities and variable foci in the clinically examined affected dogs. A genome-wide association study using Affymetrix...... mutation. It would establish the affected breed as a novel therapeutic model, help to develop a DNA test for breeding purposes and introduce a novel candidate gene for human idiopathic epilepsies....

  6. Effects of the use of growth hormone in children and adolescents with juvenile idiopathic arthritis: a systematic review

    Directory of Open Access Journals (Sweden)

    Renan Bazuco Frittoli

    Full Text Available Abstract Introduction: Children with juvenile idiopathic arthritis (JIA often have impaired growth and short stature. There is evidence that the therapeutic use of growth hormone (GH is useful and safe in these patients. Objective: To analyze the effects of GH use in patients with JIA. Method: A systematic review of the literature over the last 18 years in Medline and Embase databases. The criteria were analyzed independently by the researchers. We used the following keywords: "growth hormone", "arthritis, juvenile", "arthritis, rheumatoid", "child" and "adolescent". Results: Among the 192 identified articles, 20 corresponded to the inclusion criteria. Seventeen longitudinal studies and 3 case reports were found. Most studies analyzed observed increased growth, muscle mass and bone mass using GH. Adverse effects observed were glucose intolerance, diabetes, bone deformities, osteonecrosis, reactivation of the disease and low final height. Conclusion: The majority of studies reported positive effects after the therapeutic use of GH, but some variability in response to treatment was observed. The combination of growth hormone with other drugs seems to be a good option.

  7. MRI evaluation of multifidus muscles in adolescent idiopathic scoliosis

    Energy Technology Data Exchange (ETDEWEB)

    Chan Yu-Leung; King, A.D.; Griffith, J.F.; Metreweli, C. [Dept. of Diagnostic Radiology and Organ Imaging, Chinese University of Hong Kong, Prince of Wales Hospital, Shatin (Hong Kong); Cheng, J.C.Y.; Guo Xia [Orthopaedics and Traumatology, Chinese University of Hong Kong, Prince of Wales Hospital, Shatin (Hong Kong)

    1999-05-01

    Background. The role of the multifidus muscles in the initiation and progression of curve in adolescent idiopathic scoliosis is not fully understood and controversy exists as to the side of the abnormality. Objective. To evaluate on MRI the multifidus muscles at the apex of the major curve in adolescent idiopathic scoliosis to ascertain if the multifidus muscles on the convex or concave side are abnormal and the relationship to curve severity. Materials and methods. Forty-six patients with adolescent idiopathic scoliosis, separated into two groups, were studied using a 1.5-T MR scanner with the synergy spine coil, employing a modified STIR (short tau inversion recovery) axial sequence obtained at the apex of the major scoliotic curve. Results. No hyperintense signal change was demonstrated in the convex side multifidus muscles in any patient. In group I, 16 of 18 patients with severe or rapidly progressive curve showed increase in signal intensity in the multifidus muscle on the concave side of the apex of the curve. In group II, of the 15 patients with mild curve (Cobb angle 10-30 ), 4 had increased signal intensity in the multifidus muscle on the concave side; of the 13 with more severe curve (Cobb angle greater than 30 ), 10 had increase in multifidus signal intensity on the concave side. Conclusions. The concave-side multifidus muscle at the apex of a scoliotic curve was morphologically abnormal. A significant association between abnormal signal change and curve severity was also established. (orig.) With 2 figs., 3 tabs., 25 refs.

  8. Omalizumab for the Treatment of Chronic Idiopathic Urticaria: Systematic Review of the Literature.

    Science.gov (United States)

    Tonacci, Alessandro; Billeci, Lucia; Pioggia, Giovanni; Navarra, Michele; Gangemi, Sebastiano

    2017-04-01

    Omalizumab is recombinant humanized monoclonal antibody to immunoglobulin E. Guidelines for the treatment of chronic idiopathic urticaria (also known as chronic spontaneous urticaria) recommend the use of omalizumab as third-line therapy in addition to high doses of histamine receptor type 1 (H 1 ) antihistamines when they are unsuccessful as first- and second-line therapy. We performed a systematic review of the literature to identify studies that evaluated the efficacy of omalizumab for the treatment of chronic idiopathic urticaria, in both controlled and real-world settings, to assess its potential role as a preferred therapy. The PubMed, ScienceDirect, LILACS (Latin American and Caribbean Health Sciences Literature), and Google Scholar databases were searched between January 1, 2000, and November 21, 2016. The search was limited to articles published in peer-reviewed journals in the English language, and 29 studies were included in this review. Omalizumab 300 mg administered every 4 weeks appears to be the most effective and safe dosage, with a rapid response time, for the treatment of chronic idiopathic urticaria, with few minor adverse effects, and appears to be safe in the offspring of pregnant patients who received the drug. However, as published studies of omalizumab are sparse, future studies are warranted. When findings are confirmed in larger studies, due to its efficacy, safety, and increased benefit/cost ratio, omalizumab could become the preferred method of treatment for chronic idiopathic urticaria in patients unresponsive to H 1 antihistamines. © 2017 Pharmacotherapy Publications, Inc.

  9. TOPOGRAPHIC CHANGES OF THE MACULA AFTER CLOSURE OF IDIOPATHIC MACULAR HOLE.

    Science.gov (United States)

    Pak, Kang Yeun; Park, Keun Heung; Kim, Kyong Ho; Park, Sung Who; Byon, Ik Soo; Kim, Hyun Woong; Chung, In Young; Lee, Joo Eun; Lee, Sang Joon; Lee, Ji Eun

    2017-04-01

    To investigate retinal displacement in the macula after surgical closure of idiopathic macular hole and to identify factors correlated with displacement. This retrospective multicenter study included 73 eyes of 73 patients having idiopathic macular hole. A custom program was developed to compare the position of the retinal vessels in the macula between preoperative and postoperative photographs. En face images of a 6 mm × 6 mm optical coherence tomography volume scans were registered to calculate the scale. A grid comprising 16 sectors in 2 rings (inner; 2-4 mm and outer; 4-6 mm) was superimposed. The displacement of the retinal vessels was measured as a vector value by comparing the location of the retinal vessels in each sector. The correlation between displacement and various clinical parameters was analyzed. The average displacement was 57.2 μm at an angle of -3.3° (nasal and slightly inferior). Displacement was larger in the inner ring (79.2 μm) than in the outer ring (35.3 μm, P macula was displaced centripetally, nasally, and slightly inferiorly after surgical closure of idiopathic macular hole. Hole closure, contraction of the nerve fiber layer, and gravity are the suggested mechanisms of macular displacement caused by internal limiting membrane peeling.

  10. Exercise therapy in juvenile idiopathic arthritis: a Cochrane Review

    NARCIS (Netherlands)

    Takken, T.; van Brussel, M.; Engelbert, R. H. H.; van der Net, J.; Kuis, W.; Helders, P. J. M.

    2008-01-01

    Exercise therapy is considered an important component of the treatment of arthritis. The efficacy of exercise therapy has been reviewed in adults with rheumatoid arthritis but not in children with juvenile idiopathic arthritis (JIA). To assess the effects of exercise therapy on functional ability,

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

    DEFF Research Database (Denmark)

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

    2015-01-01

    Introduction: Since 2008 the non-surgical treatment of adolescent idiopathic scoliosis (AIS) in the southern part of Denmark, went from full-time bracing with Boston brace, to Providence night-time bracing. Methods: Since 2008, skeletally immature patients diagnosed with AIS and a primary curve w...

  12. Relationship between thoracic hypokyphosis, lumbar lordosis and sagittal pelvic parameters in adolescent idiopathic scoliosis.

    Science.gov (United States)

    Clément, Jean-Luc; Geoffray, Anne; Yagoubi, Fatima; Chau, Edouard; Solla, Federico; Oborocianu, Ioana; Rampal, Virginie

    2013-11-01

    Sagittal spine and pelvic alignment of adolescent idiopathic scoliosis (AIS) is poorly described in the literature. It generally reports the sagittal alignment with regard to the type of curve and never correlated to the thoracic kyphosis. The objective of this study is to investigate the relationship between thoracic kyphosis, lumbar lordosis and sagittal pelvic parameters in thoracic AIS. Spinal and pelvic sagittal parameters were evaluated on lateral radiographs of 86 patients with thoracic AIS; patients were separated into hypokyphosis group (n = 42) and normokyphosis group (n = 44). Results were statistically analyzed. The lumbar lordosis was lower in the hypokyphosis group, due to the low proximal lordosis. The thoracic kyphosis was not correlated with any pelvic parameters but with the proximal lordosis. The pelvic incidence was correlated with sacral slope, pelvic tilt, lumbar lordosis and highly correlated with distal lumbar lordosis in the two groups. There was a significant linear regression between thoracic kyphosis and proximal lordosis and between pelvic incidence and distal lordosis. We can consider that the proximal part of the lordosis depends on the thoracic kyphosis and the distal part depends on the pelvic incidence. The hypokyphosis in AIS is independent of the pelvic parameters and could be described as a structural parameter, characteristic of the scoliotic deformity.

  13. Effects of Low-frequency Current Sacral Dermatome Stimulation on Idiopathic Slow Transit Constipation.

    Science.gov (United States)

    Kim, Jin-Seop; Yi, Seung-Ju

    2014-06-01

    [Purpose] This study aimed to determine whether low-frequency current therapy can be used to reduce the symptoms of idiopathic slow transit constipation (ISTC). [Subjects] Fifteen patients (ten male and five female) with idiopathic slow transit constipation were enrolled in the present study. [Results] Bowel movements per day, bowel movements per week, and constipation assessment scale scores significantly improved after low-frequency current simulation of S2-S3. [Conclusion] Our results show that stimulation with low-frequency current of the sacral dermatomes may offer therapeutic benefits for a subject of patients with ISTC.

  14. Diagnostic value of optical coherence tomography for intracranial pressure in idiopathic intracranial hypertension

    DEFF Research Database (Denmark)

    Skau, M; Yri, H; Sander, B

    2013-01-01

    BACKGROUND: Idiopathic intracranial hypertension (IIH) is a condition of raised intracranial pressure (ICP) in the absence of space-occupying lesions or other known etiology. It primarily affects young obese females, and potentially causes permanent visual loss due to papilledema and secondary...... optic atrophy. The aim of this study was to evaluate the diagnostic value of optical coherence tomography (OCT) as a marker for CSF opening pressure in patients with idiopathic intracranial hypertension (IIH). METHODS: We conducted a case-control study of 20 newly diagnosed, 21 long-term IIH patients...

  15. Is nucleon deformed?

    International Nuclear Information System (INIS)

    Abbas, Afsar

    1992-01-01

    The surprising answer to this question Is nucleon deformed? is : Yes. The evidence comes from a study of the quark model of the single nucleon and when it is found in a nucleus. It turns out that many of the long standing problems of the Naive Quark Model are taken care of if the nucleon is assumed to be deformed. Only one value of the parameter P D ∼1/4 (which specifies deformation) fits g A (the axial vector coupling constant) for all the semileptonic decay of baryons, the F/D ratio, the pion-nucleon-delta coupling constant fsub(πNΔ), the double delta coupling constant 1 fsub(πΔΔ), the Ml transition moment μΔN and g 1 p the spin structure function of proton 2 . All this gives strong hint that both neutron and proton are deformed. It is important to look for further signatures of this deformation. When this deformed nucleon finds itself in a nuclear medium its deformation decreases. So much that in a heavy nucleus the nucleons are actually spherical. We look into the Gamow-Teller strengths, magnetic moments and magnetic transition strengths in nuclei to study this property. (author). 15 refs

  16. Wind turbines and idiopathic symptoms

    DEFF Research Database (Denmark)

    Blanes-Vidal, Victoria; Schwartz, Joel

    2016-01-01

    Whether or not wind turbines pose a risk to human health is a matter of heated debate. Personal reactions to other environmental exposures occurring in the same settings as wind turbines may be responsible of the reported symptoms. However, these have not been accounted for in previous studies. We...... investigated whether there is an association between residential proximity to wind turbines and idiopathic symptoms, after controlling for personal reactions to other environmental co-exposures. We assessed wind turbine exposures in 454 residences as the distance to the closest wind turbine (Dw) and number...... of wind turbines

  17. Toxocara Seroprevalence in Patients with Idiopathic Parkinson's Disease: Chance Association or Coincidence?

    Directory of Open Access Journals (Sweden)

    Tuncay Çelik

    2013-01-01

    Full Text Available Most cases of idiopathic Parkinson disease (IPD are believed to be due to a combination of genetic and environmental factors. The purpose of this study is to investigate the relationship between toxocariasis and Parkinson disease (PD. Patients were selected from people who were admitted to the Movement Disorders Branch, Neurology Department of Elazığ University Faculty of Medicine Elazığ, Turkey. We studied specific IgG antibodies against Toxocara canis (T. canis in 50 patients with idiopathic Parkinson and 50 healthy volunteers. We investigated the clinical history of three patients infected with T. canis. We also studied specific IgG antibodies against Toxoplasma gondii in these groups. Antibodies anti-Toxocara canis were found in 3 idiopathic PD (6% (P=0.121 and antibody titer was not found in control. A patient had history of the presence of dog in current dog ownership. We did not detect any statistically significant association between T. canis and IPD. But, we believe that further comprehensive studies are required for understanding whether there is a causal relation between toxocariasis and PD. We didn’t find possible association between Toxoplasma gondii and IPD (P=0.617.

  18. Chiropractic manipulation in Adolescent Idiopathic Scoliosis: a pilot study

    Directory of Open Access Journals (Sweden)

    Stoline Michael R

    2006-08-01

    Full Text Available Abstract Background Adolescent idiopathic scoliosis (AIS remains the most common deforming orthopedic condition in children. Increasingly, both adults and children are seeking complementary and alternative therapy, including chiropractic treatment, for a wide variety of health concerns. The scientific evidence supporting the use chiropractic intervention is inadequate. The purpose of this study was to conduct a pilot study and explore issues of safety, patient recruitment and compliance, treatment standardization, sham treatment refinement, inter-professional cooperation, quality assurance, and outcome measure selection. Methods Six patients participated in this 6-month study, 5 of whom were female. One female was braced. The mean age of these patients was 14 years, and the mean Cobb angle was 22.2 degrees. The study design was a randomized controlled clinical trial with two independent and blinded observers. Three patients were treated by standard medical care (observation or brace treatment, two were treated with standard medical care plus chiropractic manipulation, and one was treated with standard medical care plus sham manipulation. The primary outcome measure was Cobb, and the psychosocial measure was Scoliosis Quality of Life Index. Results Orthopedic surgeons and chiropractors were easily recruited and worked cooperatively throughout the trial. Patient recruitment and compliance was good. Chiropractic treatments were safely employed, and research protocols were successful. Conclusion Overall, our pilot study showed the viability for a larger randomized trial. This pilot confirms the strength of existing protocols with amendments for use in a full randomized controlled trial. Trial registration This trial has been assigned an international standard randomized controlled trial number by Current Controlled Trials, Ltd. http://www.controlled-trials.com/isrctn/. The number is ISRCTN41221647.

  19. Idiopathic Intracranial Hypertension – Pathophysiology Based on Case Series

    Directory of Open Access Journals (Sweden)

    Ljubisavljević Srdjan

    2016-09-01

    Full Text Available According to the definition, idiopathic intracranial hypertension (IIH is a pathological state characterized by an increase in intracranial pressure; however, there are no obvious intracranial pathological processes. The pathophysiology of this disorder is not clear, although there are many reports related to it.

  20. The aetiology of idiopathic scoliosis : biomechanical and neuromuscular factors

    NARCIS (Netherlands)

    Veldhuizen, AG; Webb, PJ

    The aetiology of adolescent idiopathic scoliosis (AIS) remains an enigma. In the literature there are two opinions: one believes a deviating growth pattern is responsible for the condition - patients with AIS lend to be growing faster/be taller - while the other opinion assumes that the growth

  1. Exercise testing and fitness training in juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Singh-Grewal, D.

    2010-01-01

    Juvenile Idiopathic Arthritis is the commonest rheumatic disease of childhood affecting 1:1000 children under the age of 16 years. Children with JIA have long been sidelined from physical activity due to active disease or irrational concerns that activity may in some way worsen disease. Children

  2. Treatment of Juvenile Idiopathic Arthritis-Associated Uveitis

    OpenAIRE

    Oray, Merih; Tu?al-Tutkun, ?lknur

    2016-01-01

    Pediatric uveitis may be a serious health problem because of the lifetime burden of vision loss due to severe complications if the problem is not adequately treated. Juvenile idiopathic arthritis (JIA)-associated uveitis is characterized by insidious onset and potentially blinding chronic anterior uveitis. Periodic ophthalmologic screening is of utmost importance for early diagnosis of uveitis. Early diagnosis and proper immunomodulatory treatment are essential for good visual progno...

  3. Correlation of Seminal Plasma Total Antioxidant Capacity and Malondialdehyde Levels With Sperm Parameters in Men With Idiopathic Infertility

    Directory of Open Access Journals (Sweden)

    Fazeli

    2016-01-01

    Full Text Available Background Oxidative stress is the result of an imbalance between the production and scavenging of reactive oxygen species (ROS. Recently, oxidative stress has been introduced as a major cause of male infertility. Objectives The aim of the present study was to determine the correlation between total antioxidant capacity (TAC and malondialdehyde (MDA as markers of oxidative stress in relation to idiopathic male infertility and sperm parameters. Patients and Methods This case control study was conducted using 35 men with idiopathic infertility and 34 men with proven fertility. Seminal plasma TAC and MDA were measured by ferric reducing ability of plasma (FRAP and thiobarbituric acid (TBA reaction methods, respectively. Results Seminal TAC levels were significantly lower and seminal MDA levels were significantly higher in men with idiopathic infertility than in fertile men (P < 0.0001 and P = 0.004, respectively. A positive correlation was shown between sperm motility, sperm morphology, and TAC levels in men with idiopathic infertility (P = 0.002 and P = 0.002, respectively. In addition, there was a correlation between sperm motility and TAC levels in fertile men (P = 0.005. There was no correlation between sperm count and TAC levels in either men with idiopathic infertility or in fertile men. Negative correlations were observed between MDA levels and sperm motility, morphology, and sperm count only in men with idiopathic infertility (P = 0.003, P = 0.001, and P = 0.006, respectively. Conclusions Our results show that oxidative stress could play an important role in male infertility as well as in sperm motility and sperm morphology.

  4. Adolescent idiopathic scoliosis (AIS, environment, exposome and epigenetics: a molecular perspective of postnatal normal spinal growth and the etiopathogenesis of AIS with consideration of a network approach and possible implications for medical therapy

    Directory of Open Access Journals (Sweden)

    Burwell R Geoffrey

    2011-12-01

    Full Text Available Abstract Genetic factors are believed to play an important role in the etiology of adolescent idiopathic scoliosis (AIS. Discordant findings for monozygotic (MZ twins with AIS show that environmental factors including different intrauterine environments are important in etiology, but what these environmental factors may be is unknown. Recent evidence for common chronic non-communicable diseases suggests epigenetic differences may underlie MZ twin discordance, and be the link between environmental factors and phenotypic differences. DNA methylation is one important epigenetic mechanism operating at the interface between genome and environment to regulate phenotypic plasticity with a complex regulation across the genome during the first decade of life. The word exposome refers to the totality of environmental exposures from conception onwards, comprising factors in external and internal environments. The word exposome is used here also in relation to physiologic and etiopathogenetic factors that affect normal spinal growth and may induce the deformity of AIS. In normal postnatal spinal growth we propose a new term and concept, physiologic growth-plate exposome for the normal processes particularly of the internal environments that may have epigenetic effects on growth plates of vertebrae. In AIS, we propose a new term and concept pathophysiologic scoliogenic exposome for the abnormal processes in molecular pathways particularly of the internal environment currently expressed as etiopathogenetic hypotheses; these are suggested to have deforming effects on the growth plates of vertebrae at cell, tissue, structure and/or organ levels that are considered to be epigenetic. New research is required for chromatin modifications including DNA methylation in AIS subjects and vertebral growth plates excised at surgery. In addition, consideration is needed for a possible network approach to etiopathogenesis by constructing AIS diseasomes. These approaches may

  5. Idiopathic Transverse Myelitis and Neuromyelitis Optica: Clinical Profiles, Pathophysiology and Therapeutic Choices

    Science.gov (United States)

    Awad, Amer; Stüve, Olaf

    2011-01-01

    Transverse myelitis is a focal inflammatory disorder of the spinal cord which may arise due to different etiologies. Transverse myelitis may be idiopathic or related/secondary to other diseases including infections, connective tissue disorders and other autoimmune diseases. It may be also associated with optic neuritis (neuromyelitis optica), which may precede transverse myelitis. In this manuscript we review the pathophysiology of different types of transverse myelitis and neuromyelitis optica and discuss diagnostic criteria for idiopathic transverse myelitis and risk of development of multiple sclerosis after an episode of transverse myelitis. We also discuss treatment options including corticosteroids, immunosuppressives and monoclonal antibodies, plasma exchange and intravenous immunoglobulins. PMID:22379456

  6. Anti-TNF therapy for juvenile idiopathic arthritis-related uveitis

    Science.gov (United States)

    Semeraro, Francesco; Arcidiacono, Barbara; Nascimbeni, Giuseppe; Angi, Martina; Parolini, Barbara; Costagliola, Ciro

    2014-01-01

    Juvenile idiopathic arthritis-related uveitis is the most common type of uveitis in childhood and one of the main causes of visual impairment in children. The introduction of biological treatment has widened the range of therapeutic options for children with uveitis refractory to standard nonbiologic immunosuppressants. Data from clinical trials suggest that both adalimumab and infliximab have demonstrated effectiveness and safety in open-label studies, although no large, randomized, controlled trials have been reported so far. The role of etanercept in treating juvenile idiopathic arthritis-related uveitis is not yet well defined. In our experience, anti-tumor necrosis factor therapy has been shown to be more effective than steroids and/or methotrexate in treating uveitis. Up to now, tumor necrosis factor blocking compounds have been reserved for the treatment of the most severe cases of refractory uveitis, and larger prospective clinical trials are required in order to better assess the safety of these new compounds. PMID:24711694

  7. Anti-TNF therapy for juvenile idiopathic arthritis-related uveitis.

    Science.gov (United States)

    Semeraro, Francesco; Arcidiacono, Barbara; Nascimbeni, Giuseppe; Angi, Martina; Parolini, Barbara; Costagliola, Ciro

    2014-01-01

    Juvenile idiopathic arthritis-related uveitis is the most common type of uveitis in childhood and one of the main causes of visual impairment in children. The introduction of biological treatment has widened the range of therapeutic options for children with uveitis refractory to standard nonbiologic immunosuppressants. Data from clinical trials suggest that both adalimumab and infliximab have demonstrated effectiveness and safety in open-label studies, although no large, randomized, controlled trials have been reported so far. The role of etanercept in treating juvenile idiopathic arthritis-related uveitis is not yet well defined. In our experience, anti-tumor necrosis factor therapy has been shown to be more effective than steroids and/or methotrexate in treating uveitis. Up to now, tumor necrosis factor blocking compounds have been reserved for the treatment of the most severe cases of refractory uveitis, and larger prospective clinical trials are required in order to better assess the safety of these new compounds.

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

    Science.gov (United States)

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

    2017-01-01

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

  9. Adult-onset photosensitivity: clinical significance and epilepsy syndromes including idiopathic (possibly genetic) photosensitive occipital epilepsy.

    Science.gov (United States)

    Koutroumanidis, Michalis; Tsirka, Vasiliki; Panayiotopoulos, Chrysostomos

    2015-09-01

    To evaluate the clinical associations of adult-onset photosensitivity, we studied the clinical and EEG data of patients who were referred due to a possible first seizure and who had a photoparoxysmal response on their EEG. Patients with clinical evidence of photosensitivity before the age of 20 were excluded. Of a total of 30 patients, four had acute symptomatic seizures, two had vasovagal syncope, and 24 were diagnosed with epilepsy. Nine of the 24 patients had idiopathic (genetic) generalized epilepsies and predominantly generalized photoparoxysmal response, but also rare photically-induced seizures, while 15 had exclusively, or almost exclusively, reflex photically-induced occipital seizures with frequent secondary generalization and posterior photoparoxysmal response. Other important differences included a significantly older age at seizure onset and paucity of spontaneous interictal epileptic discharges in patients with photically-induced occipital seizures; only a quarter of these had occasional occipital spikes, in contrast to the idiopathic (genetic) generalized epilepsy patients with typically generalized epileptic discharges. On the other hand, both groups shared a positive family history of epilepsy, common seizure threshold modulators (such as tiredness and sleep deprivation), normal neurological examination and MRI, a generally benign course, and good response to valproic acid. We demonstrated that photosensitivity can first occur in adult life and manifest, either as idiopathic (possibly genetic) photosensitive occipital epilepsy with secondary generalization or as an EEG, and less often, a clinical/EEG feature of idiopathic (genetic) generalized epilepsies. Identification of idiopathic photosensitive occipital epilepsy fills a diagnostic gap in adult first-seizure epileptology and is clinically important because of its good response to antiepileptic drug treatment and fair prognosis.

  10. Idiopathic focal epilepsies: the "lost tribe".

    Science.gov (United States)

    Pal, Deb K; Ferrie, Colin; Addis, Laura; Akiyama, Tomoyuki; Capovilla, Giuseppe; Caraballo, Roberto; de Saint-Martin, Anne; Fejerman, Natalio; Guerrini, Renzo; Hamandi, Khalid; Helbig, Ingo; Ioannides, Andreas A; Kobayashi, Katsuhiro; Lal, Dennis; Lesca, Gaetan; Muhle, Hiltrud; Neubauer, Bernd A; Pisano, Tiziana; Rudolf, Gabrielle; Seegmuller, Caroline; Shibata, Takashi; Smith, Anna; Striano, Pasquale; Strug, Lisa J; Szepetowski, Pierre; Valeta, Thalia; Yoshinaga, Harumi; Koutroumanidis, Michalis

    2016-09-01

    The term idiopathic focal epilepsies of childhood (IFE) is not formally recognised by the ILAE in its 2010 revision (Berg et al., 2010), nor are its members and boundaries precisely delineated. The IFEs are amongst the most commonly encountered epilepsy syndromes affecting children. They are fascinating disorders that hold many "treats" for both clinicians and researchers. For example, the IFEs pose many of the most interesting questions central to epileptology: how are functional brain networks involved in the manifestation of epilepsy? What are the shared mechanisms of comorbidity between epilepsy and neurodevelopmental disorders? How do focal EEG discharges impact cognitive functioning? What explains the age-related expression of these syndromes? Why are EEG discharges and seizures so tightly locked to slow-wave sleep? In the last few decades, the clinical symptomatology and the respective courses of many IFEs have been described, although they are still not widely appreciated beyond the specialist community. Most neurologists would recognise the core syndromes of IFE to comprise: benign epilepsy of childhood with centro-temporal spikes or Rolandic epilepsy (BECTS/RE); Panayiotopoulos syndrome; and the idiopathic occipital epilepsies (Gastaut and photosensitive types). The Landau-Kleffner syndrome and the related (idiopathic) epilepsy with continuous spikes and waves in sleep (CSWS or ESES) are also often included, both as a consequence of the shared morphology of the interictal discharges and their potential evolution from core syndromes, for example, CSWS from BECTS. Atypical benign focal epilepsy of childhood also has shared electro-clinical features warranting inclusion. In addition, a number of less well-defined syndromes of IFE have been proposed, including benign childhood seizures with affective symptoms, benign childhood epilepsy with parietal spikes, benign childhood seizures with frontal or midline spikes, and benign focal seizures of adolescence. The

  11. Idiopathic urolithiasis in Tunisian children: A report of 134 cases

    Directory of Open Access Journals (Sweden)

    A Alaya

    2013-01-01

    Full Text Available We evaluated the metabolic and the nutritional aspects of 134 urolithiasis children in order to outline the characteristics of idiopathic urolithiasis in children. This prospective study group of 134 children (56 females, 78 males with renal calculi was evaluated. The age range of the patients was six months to 16 years. A dietary survey was performed on every child. All patients were investigated with respect to stone localization and serum and urine risk factors. Statistical analysis of data was carried out using software SPSS 11.0 for Windows. Hypercalciuria was the most common risk factor detected in this group (28.3%. A decrease of water intake was noted in all age groups, especially in the rural area (549.6 mL/day vs. 1150.6 mL/day, and there was an increase in animal protein intake in 17 cases (mean: 1.9 g/kg. In addition, increased intake of starchy foods and food with high oxalate content (sorgum were detected in the ten to 16 years age group (51% of our study. Calcium oxalate monohydrate represents the principal component of idiopathic stone (58.2%, which is more frequent in children (68% than in infants (51.7% (P <0.02. The major etiology of idiopathic urolithiasis highlights the influence of dietary habit in stone formers in our country. The increased occurrence of calcium oxalate stones in school age children confirms the change in the etiology of urolithiasis according to age.

  12. Analysis of T Cell Subsets in Adult Primary/Idiopathic Minimal Change Disease: A Pilot Study

    Directory of Open Access Journals (Sweden)

    Francisco Salcido-Ochoa

    2017-01-01

    Full Text Available Aim. To characterise infiltrating T cells in kidneys and circulating lymphocyte subsets of adult patients with primary/idiopathic minimal change disease. Methods. In a cohort of 9 adult patients with primary/idiopathic minimal change recruited consecutively at disease onset, we characterized (1 infiltrating immune cells in the kidneys using immunohistochemistry and (2 circulating lymphocyte subsets using flow cytometry. As an exploratory analysis, association of the numbers and percentages of both kidney-infiltrating immune cells and the circulating lymphocyte subsets with kidney outcomes including deterioration of kidney function and proteinuria, as well as time to complete clinical remission up to 48 months of follow-up, was investigated. Results. In the recruited patients with primary/idiopathic minimal change disease, we observed (a a dominance of infiltrating T helper 17 cells and cytotoxic cells, comprising cytotoxic T cells and natural killer cells, over Foxp3+ Treg cells in the renal interstitium; (b an increase in the circulating total CD8+ T cells in peripheral blood; and (c an association of some of these parameters with kidney function and proteinuria. Conclusions. In primary/idiopathic minimal change disease, a relative numerical dominance of effector over regulatory T cells can be observed in kidney tissue and peripheral blood. However, larger confirmatory studies are necessary.

  13. Lung microbiome and disease progression in idiopathic pulmonary fibrosis: an analysis of the COMET study.

    Science.gov (United States)

    Han, MeiLan K; Zhou, Yueren; Murray, Susan; Tayob, Nabihah; Noth, Imre; Lama, Vibha N; Moore, Bethany B; White, Eric S; Flaherty, Kevin R; Huffnagle, Gary B; Martinez, Fernando J

    2014-07-01

    The role of the lung microbiome in the pathogenesis of idiopathic pulmonary fibrosis is unknown. We investigated whether unique microbial signatures were associated with progression of idiopathic pulmonary fibrosis. Patients (aged 35-80 years) with idiopathic pulmonary fibrosis within 4 years of diagnosis from the Correlating Outcomes with biochemical Markers to Estimate Time-progression (COMET) in idiopathic pulmonary fibrosis study were followed up for a maximum of 80 weeks. Progression-free survival was defined as time to death, acute exacerbation, lung transplant, or decrease in forced vital capacity (FVC) of 10% or greater or decrease in diffusion capacity of the lung (DLCO) of 15% or greater. DNA was isolated from 55 samples of bronchoscopic alveolar lavage. 454 pyrosequencing was used to assign operational taxonomic units (OTUs) to bacteria based on a 3% sequence divergence. Adjusted Cox models were used to identify OTUs that were significantly associated with progression-free survival at a pidiopathic pulmonary fibrosis is associated with the presence of specific members within the Staphylococcus and Streptococcus genera. Additional research will be needed to identify the specific bacterial species and to ascertain whether this is a causal association. National Institutes of Health. Copyright © 2014 Elsevier Ltd. All rights reserved.

  14. Eisenmenger syndrome and idiopathic pulmonary arterial hypertension: do parenchymal lung changes reflect aetiology?

    International Nuclear Information System (INIS)

    Griffin, N.; Allen, D.; Wort, J.; Rubens, M.; Padley, S.

    2007-01-01

    Aim: To document the pulmonary vascular changes on thin-section computed tomography (CT) in patients with Eisenmenger syndrome and idiopathic pulmonary arterial hypertension, and to determine whether there is any correlation with pulmonary arterial pressures or the aetiology of pulmonary hypertension. Material and methods: From the National Pulmonary Hypertension Database, we identified eight patients with idiopathic pulmonary arterial hypertension and 20 patients with Eisenmenger syndrome (secondary to a ventriculoseptal defect) who had also undergone contrast-enhanced thin-section CT. CT studies were reviewed for the presence of centrilobular nodules, mosaicism, neovascularity, and bronchial artery hypertrophy. Haemodynamic data were also reviewed. Results: Centrilobular nodules, mosaicism, and neovascularity were seen in both patient groups (p > 0.05). A significantly higher number of enlarged bronchial arteries were seen in patients with Eisenmenger syndrome. There was no correlation with pulmonary arterial pressures. Conclusion: Patients with idiopathic pulmonary arterial hypertension and Eisenmenger syndrome demonstrated similar pulmonary vascular changes on CT. These changes did not predict the underlying cause of pulmonary hypertension or its severity

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

    Science.gov (United States)

    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.

  16. Eisenmenger syndrome and idiopathic pulmonary arterial hypertension: do parenchymal lung changes reflect aetiology?

    Energy Technology Data Exchange (ETDEWEB)

    Griffin, N. [Royal Brompton and Harefield NHS Trust, London (United Kingdom)]. E-mail: nyreegriffin@hotmail.com; Allen, D. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Wort, J. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Rubens, M. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Padley, S. [Royal Brompton and Harefield NHS Trust, London (United Kingdom)

    2007-06-15

    Aim: To document the pulmonary vascular changes on thin-section computed tomography (CT) in patients with Eisenmenger syndrome and idiopathic pulmonary arterial hypertension, and to determine whether there is any correlation with pulmonary arterial pressures or the aetiology of pulmonary hypertension. Material and methods: From the National Pulmonary Hypertension Database, we identified eight patients with idiopathic pulmonary arterial hypertension and 20 patients with Eisenmenger syndrome (secondary to a ventriculoseptal defect) who had also undergone contrast-enhanced thin-section CT. CT studies were reviewed for the presence of centrilobular nodules, mosaicism, neovascularity, and bronchial artery hypertrophy. Haemodynamic data were also reviewed. Results: Centrilobular nodules, mosaicism, and neovascularity were seen in both patient groups (p > 0.05). A significantly higher number of enlarged bronchial arteries were seen in patients with Eisenmenger syndrome. There was no correlation with pulmonary arterial pressures. Conclusion: Patients with idiopathic pulmonary arterial hypertension and Eisenmenger syndrome demonstrated similar pulmonary vascular changes on CT. These changes did not predict the underlying cause of pulmonary hypertension or its severity.

  17. The Role of Diffusion-Weighted MRI in Differentiation of Idiopathic Acute Transverse Myelitis and Acute Spinal Cord Infarction

    International Nuclear Information System (INIS)

    Kim, Yeo Goon; Lee, Joon Woo; Kim, Jae Hyoung; Kang, Heung Sik; Park, Kyung Seok

    2011-01-01

    To compare the diffusion characteristics of idiopathic acute transverse myelitis (ATM) and acute spinal cord infarction (SCI). Diffusion-weighted images (DWI) and an apparent diffusion coefficient (ADC) map were prospectively obtained from patients diagnosed with myelopathy between February 2006 and April 2009. Inclusion criteria included 1) the presence of an intramedullary T2-high signal intensity and 2) a final diagnosis of idiopathic ATM or SCI established by one neurologist. In total, 13 patients (M : F = 8 : 5; mean age, 39.5 years; range, 29-50 years) with idiopathic ATM and seven patients (M : F = 2 : 5; mean age, 58 years; range, 48-75 years) with SCI were included in this study. Two radiologists evaluated the DWIs and ADC map in consensus. The extent of the cord signal change was also evaluated on T2-weighted sagittal images. Among the 16 patients with ATM, 14 patients showed iso-signal on an ADC map, but one case showed restricted diffusion and another showed increased diffusion on the ADC map. Among the seven patients with SCI, five patients showed restricted diffusion. Idiopathic ATM usually does not demonstrate restricted diffusion, which can be a clue to differentiate it from SCI. However, idiopathic ATM with larger segment involvement can show focal diffusion restriction.

  18. Idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    Noble Paul W

    2008-03-01

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

  19. [Idiopathic scrotal elephantiasis. A case report].

    Science.gov (United States)

    López-Caballero, Ignacio; Sánchez-Ruvalcaba, Itzel; Sánchez-Martinez, Luis Carlos; Hernández-Ordoñez, Octavio; Gómez-Lara, Miguel; Flores-Carrillo, Víctor

    2014-01-01

    Penoscrotal lymphedema (scrotal elephantiasis) is a condition that has been described in areas in which filariasis is endemic. This paper presents a case of a 45-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 penoscrotal reconstruction. Currently, the patient is receiving follow-up care without evidence of recurrence. Penoscrotal lymphedema may cause symptoms of weakness, immobility and emotional disturbance. Surgery procedure provides a satisfactory cosmetical and functional outcome.

  20. Body composition in children with juvenile idiopathic arthritis: effect ...

    African Journals Online (AJOL)

    Introduction: the aim of this study was to evaluate the relationship between macronutrient intake, body composition (lean body mass and fat mass) and bone mineral content in Moroccan children with juvenile idiopathic arthritis (JIA). Methods: a cross-sectional study, conducted between May 2010 and June 2011, covering ...

  1. Hyperoxaluria in idiopathic calcium nephrolithiasis--what are the limits?

    DEFF Research Database (Denmark)

    Osther, P J

    1999-01-01

    OBJECTIVE: The object of this study was to investigate the role for measurement of 24-h renal oxalate excretion in the evaluation of idiopathic calcium stone formers. MATERIALS AND METHODS: Renal excretion rates of oxalate and creatinine were measured in 24-h urines in 46 consecutive male recurrent...

  2. Idiopathic sclerosing encapsulating peritonitis (or abdominal cocoon).

    Science.gov (United States)

    Serafimidis, Costas; Katsarolis, Ioannis; Vernadakis, Spyros; Rallis, George; Giannopoulos, George; Legakis, Nikolaos; Peros, George

    2006-02-13

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

  3. Risk Factors for Neurodegeneration in Idiopathic REM sleep Behavior Disorder: A Multicenter Study

    Science.gov (United States)

    Postuma, RB; Iranzo, A; Hogl, B; Arnulf, I; Ferini-Strambi, L; Manni, R; Miyamoto, T.; Oertel, W; Dauvilliers, Y; Ju, Y; Puligheddu, M; Sonka, K; Pelletier, A; Santamaria, J; Frauscher, B; Leu-Semenescu, S; Zucconi, M; Terzaghi, M; Miyamoto, M.; Unger, MM; Carlander, B; Fantini, ML; Montplaisir, JY

    2018-01-01

    Objective To assess whether risk factors for Parkinson’s disease and Dementia with Lewy bodies increase rate of defined neurodegenerative disease in idiopathic REM sleep behavior disorder Methods 12 centers administered a detailed questionnaire assessing risk factors for neurodegenerative synucleinopathy to patients with idiopathic REM sleep behavior disorder. Variables included demographics, lifestyle factors, pesticide exposures, occupation, co-morbid conditions, medication use, family history, and autonomic/motor symptoms. After 4-years follow-up, patients were assessed for dementia or parkinsonism. Disease risk was assessed with Kaplan-Meier analysis, and epidemiologic variables were compared between convertors and those still idiopathic using logistic regression. Results Of 305 patients, follow-up information was available for 279, of whom 93 (33.3%) developed defined neurodegenerative disease. Disease risk was 25% at 3 years, and 41% after 5 years. Patients who converted were older (difference=4.5 years, pconversion. Although occupation was similar between groups, those who converted had a lower likelihood of pesticide exposure (occupational insecticide=2.3% vs. 9.0%). Convertors were more likely to report family history of dementia (OR=2.09), without significant differences in Parkinson’s disease or sleep disorders. Medication exposures and medical history were similar between groups. Autonomic and motor symptoms were more common among those who converted. Risk factors for primary dementia and parkinsonism were generally similar, except for a notably higher clonazepam use in dementia convertors (OR=2.6). Interpretation Patients with idiopathic RBD are at very high risk of neurodegenerative synucleinopathy. Risk factor profiles between convertors and non-convertors have both important commonalities and differences. PMID:25767079

  4. Manual therapy as a conservative treatment for adolescent idiopathic scoliosis: a systematic review.

    Science.gov (United States)

    Romano, Michele; Negrini, Stefano

    2008-01-22

    The treatment of adolescent idiopathic scoliosis is contingent upon many variables. Simple observation is enough for less serious curvatures, but for very serious cases surgical intervention could be proposed. Between these there is a wide range of different treatments. Manual therapy is commonly used: the aim of this paper is to verify the data existing in the literature on the efficacy of this approach. A systematic review of the scientific literature published internationally has been performed. We have included in the term manual therapy all the manipulative and generally passive techniques performed by an external operator. In a more specific meaning, osteopathic, chiropractic and massage techniques have been considered as manipulative therapeutic methods. We performed our systematic research in Medline, Embase, Cinhal, Cochrane Library, Pedro with the following terms: idiopathic scoliosis combined with chiropractic; manipulation; mobilization; manual therapy; massage; osteopathy; and therapeutic manipulation. The criteria for inclusion were as follows: Any kind of research; diagnosis of adolescent idiopathic scoliosis; patients treated exclusively by one of the procedures established as a standard for this review (chiropractic manipulation, osteopathic techniques, massage); and outcome in Cobb degrees. We founded 145 texts, but only three papers were relevant to our study. However, no one of the three satisfied all the required inclusion criteria because they were characterized by a combination of manual techniques and other therapeutic approaches. The lack of any kind of serious scientific data does not allow us to draw any conclusion on the efficacy of manual therapy as an efficacious technique for the treatment of Adolescent idiopathic scoliosis.

  5. Plastic deformation

    NARCIS (Netherlands)

    Sitter, de L.U.

    1937-01-01

    § 1. Plastic deformation of solid matter under high confining pressures has been insufficiently studied. Jeffreys 1) devotes a few paragraphs to deformation of solid matter as a preface to his chapter on the isostasy problem. He distinguishes two properties of solid matter with regard to its

  6. MANAGEMENT OF CHRONIC IDIOPATHIC THROMBOTIC THROMBOCYTOPENIC PURPURA WITH SPLENECTOMY

    Directory of Open Access Journals (Sweden)

    Mojca Modic

    2002-07-01

    Full Text Available Background. Treatment of thrombotic thrombocytopenic purpura (TTP with plasma exchange (PE has reduced mortality rates from 90% in untreated patients to less than 20%. Despite primary effective PE, relapses occur in up to 40% of patients. Beside PE, second line treatment options are corticosteroids, vincristine, defibrotide, prostacycline. We describe two patients with relapsing TTP treated successfully with splenectomy.Patients and methods. Case 1. A 20–year female was diagnosed in October 2000 with idiopathic chronic relapsing TTP. First episode of PE plus methylprednisolone treatment led to complet remission. Subsequent relapses occured 3, 6 and 8 months after first TTP episode. Every relaps was effectivelly treated with PE and methylprednisolone. Because of more and more frequent TTP relapses laparoscopic splenectomy was performed. Currently, 7 months after splenectomy she is in complet TTP remission.Case 2. A 18–year female, was diagnosed in January 1997 with idiopathic TTP. After PE and methylprednisolone complete remission was established. Relaps occured after nearly three years. Later on, TTP got chronic course with neurological clinical symptoms and plateled drops as soon as PE treatment was withdrawn. Besides PE and methylprednisolone she received also defibrotide. Because of chronic, PE dependant TTP, laparoscopic splenectomy was performed. Today, 15 months after splenectomy she is in complete TTP remission.Conclusions. In idiopathic relapsing and chronic TTP, PE treatment only is usually not sufficient for durable remission. Second line TTP treatment drugs have limited efficacy. Based on our experience in patients with relapsing and/or chronic TTP, laparoscopic splenectomy should be considered as a first treatment option, especialy in junger patients.

  7. Transcranial motor evoked potential waveform changes in corrective fusion for adolescent idiopathic scoliosis.

    Science.gov (United States)

    Kobayashi, Kazuyoshi; Imagama, Shiro; Ito, Zenya; Ando, Kei; Hida, Tetsuro; Ito, Kenyu; Tsushima, Mikito; Ishikawa, Yoshimoto; Matsumoto, Akiyuki; Nishida, Yoshihiro; Ishiguro, Naoki

    2017-01-01

    OBJECTIVE Corrective surgery for spinal deformities can lead to neurological complications. Several reports have described spinal cord monitoring in surgery for spinal deformity, but only a few have included patients younger than 20 years with adolescent idiopathic scoliosis (AIS). The goal of this study was to evaluate the characteristics of cases with intraoperative transcranial motor evoked potential (Tc-MEP) waveform deterioration during posterior corrective fusion for AIS. METHODS A prospective database was reviewed, comprising 68 patients with AIS who were treated with posterior corrective fusion in a prospective database. A total of 864 muscles in the lower extremities were chosen for monitoring, and acceptable baseline responses were obtained from 819 muscles (95%). Intraoperative Tc-MEP waveform deterioration was defined as a decrease in intraoperative amplitude of ≥ 70% of the control waveform. Age, Cobb angle, flexibility, operative time, estimated blood loss (EBL), intraoperative body temperature, blood pressure, number of levels fused, and correction rate were examined in patients with and without waveform deterioration. RESULTS The patients (3 males and 65 females) had an average age of 14.4 years (range 11-19 years). The mean Cobb angles before and after surgery were 52.9° and 11.9°, respectively, giving a correction rate of 77.4%. Fourteen patients (20%) exhibited an intraoperative waveform change, and these occurred during incision (14%), after screw fixation (7%), during the rotation maneuver (64%), during placement of the second rod after the rotation maneuver (7%), and after intervertebral compression (7%). Most waveform changes recovered after decreased correction or rest. No patient had a motor deficit postoperatively. In multivariate analysis, EBL (OR 1.001, p = 0.085) and number of levels fused (OR 1.535, p = 0.045) were associated with waveform deterioration. CONCLUSIONS Waveform deterioration commonly occurred during rotation maneuvers

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

    Science.gov (United States)

    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.

  9. Pulmonary function vascular index predicts prognosis in idiopathic interstitial pneumonia

    NARCIS (Netherlands)

    Corte, Tamera J.; Wort, Stephen J.; MacDonald, Peter S.; Edey, Anthony; Hansell, David M.; Renzoni, Elisabetta; Maher, Toby M.; Nicholson, Andrew G.; Bandula, Steven; Bresser, Paul; Wells, Athol U.

    2012-01-01

    Background and objective: Pulmonary hypertension (PH) is associated with increased mortality in fibrotic idiopathic interstitial pneumonia (IIP). We hypothesize that baseline KCO (diffusing capacity of carbon monoxide/alveolar volume) and 6-month decline in KCO reflect PH, thus predicting mortality

  10. Impact of Juvenile Idiopathic Arthritis Associated Uveitis in Early Adulthood

    NARCIS (Netherlands)

    Haasnoot, AJW; Vernie, Lenneke A; Rothova, Aniki; V D Doe, Patricia; Los, Leonoor I; Schalij-Delfos, Nicoline E; de Boer, Joke H

    2016-01-01

    BACKGROUND: Typically juvenile idiopathic arthritis (JIA)-associated uveitis (further referred as 'JIA-uveitis') has its onset in childhood, but some patients suffer its, sometimes visual threatening, complications or ongoing disease activity in adulthood. The objective of this study was to analyze

  11. Impact of Juvenile Idiopathic Arthritis Associated Uveitis in Early Adulthood

    NARCIS (Netherlands)

    Haasnoot, Anne-Mieke J. W.; Vernie, Lenneke A.; Rothova, Aniki; van der Doe, Patricia; Los, Leonoor I.; Schalij-Delfos, Nicoline E.; de Boer, Joke H.

    2016-01-01

    Background Typically juvenile idiopathic arthritis (JIA)-associated uveitis (further referred as 'JIA-uveitis') has its onset in childhood, but some patients suffer its, sometimes visual threatening, complications or ongoing disease activity in adulthood. The objective of this study was to analyze

  12. Impact of juvenile idiopathic arthritis associated uveitis in early adulthood

    NARCIS (Netherlands)

    Haasnoot, A.-M.J.W. (Anne-Mieke J. W.); Vernie, L.A. (Lenneke A.); A. Rothová (Aniki); Doe, P.V.D. (Patricia V. D.); L.I. Los (Leonoor I.); N.E. Schalij-Delfos (Nicoline); J.H. de Boer (Joke)

    2016-01-01

    textabstractBackground: Typically juvenile idiopathic arthritis (JIA)-associated uveitis (further referred as 'JIA-uveitis') has its onset in childhood, but some patients suffer its, sometimes visual threatening, complications or ongoing disease activity in adulthood. The objective of this study was

  13. Unravelling the incidence and etiology of chronic idiopathic axonal polyneuropathy

    NARCIS (Netherlands)

    Visser, N.A.

    2016-01-01

    Chronic idiopathic axonal polyneuropathy (CIAP) is a sensory or sensorimotor polyneuropathy that has a slowly progressive course without severe disability. CIAP is diagnosed in a significant proportion of patients with polyneuropathy, but precise figures on the incidence of polyneuropathy and CIAP

  14. International Veterinary Epilepsy Task Force's current understanding of idiopathic epilepsy of genetic or suspected genetic origin in purebred dogs

    DEFF Research Database (Denmark)

    Hülsmeyer, Velia-Isabel; Fischer, Andrea; Mandigers, Paul J. J.

    2015-01-01

    Canine idiopathic epilepsy is a common neurological disease affecting both purebred and crossbred dogs. Various breed-specific cohort, epidemiological and genetic studies have been conducted to date, which all improved our knowledge and general understanding of canine idiopathic epilepsy, and in ...

  15. Clinical Case of Tocilizumab Use in a Patient with Systemic Juvenile Idiopathic Arthritis

    Directory of Open Access Journals (Sweden)

    Y. M. Spivakovskiy

    2015-01-01

    Full Text Available The article presents a case of using genetically engineered biopharmaceutical tocilizumab in a child with systemic juvenile idiopathic arthritis. On the initial stage, the treatment was characterized by resistance to high doses of glucocorticoids and cytostatic drugs. Successful termination of visceral and articular manifestations of systemic juvenile idiopathic arthritis and normalization of laboratory indicators of disease activity in the setting of use of interleukin 6 receptor blocker were described. We observed stable improvement of the child’s condition during a year-long follow-up in the setting of the selected anti-inflammatory therapy pattern. 

  16. Deformation compensation in dynamic tomography; Compensation de deformations en tomographie dynamique

    Energy Technology Data Exchange (ETDEWEB)

    Desbat, L. [Universite Joseph Fourier, UMR CNRS 5525, 38 - Grenoble (France); Roux, S. [Universite Joseph Fourier, TIMC-IMAG, In3S, Faculte de Medecine, 38 - Grenoble (France)]|[CEA Grenoble, Lab. d' Electronique et de Technologie de l' Informatique (LETI), 38 (France); Grangeat, P. [CEA Grenoble, Lab. d' Electronique et de Technologie de l' Informatique (LETI), 38 (France)

    2005-07-01

    This work is a contribution to the compensation of motion in tomography. New classes of deformation are proposed, that compensates analytically by an algorithm of a F.B.P. type reconstruction. This work makes a generalisation of the known results for affine deformations, in parallel geometry and fan-beam, to deformation classes of infinite dimension able to include strong non linearities. (N.C.)

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

    Science.gov (United States)

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

    2014-05-24

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

  18. IDIOPATHIC LARYN GEAL STENOSIS - A VERY RARE CASE

    OpenAIRE

    Sudip Kumar; Ruma; Rajesh; Arvind Kumar; Subhendu

    2014-01-01

    A 35 year old lady presented in the Out Patient Department with cough , dyspnea and gradual hoarseness for last 5 years. After proper history taking and thorough clinical examination , diagnosis of Laryngeal Stenosis was made. Subsequently by excluding important causes of Laryngeal Stenosis like trauma , chronic infection , tuberculosis and other granulomatous diseases , the diagnosis of Idiopathic Laryngeal Stenosis was established KEYWORDS ...

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

    Science.gov (United States)

    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.

  20. Idiopathic hypereosinophilic syndrome involving the liver: CT features vs. peripheral eosinophilia

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Kyung Sook; Lee, Moon Gyu; Won, Young Chul; Lee, Eun Hye; Noh, Han Na; Ha, Hyun Kwon; Kim, Pyo Nyun; Auh, Yong Ho [Ulsan Univ. College of Medicine, Seoul (Korea, Republic of)

    1997-10-01

    To correlate CT features with peripheral eosinophilia in patients with idiopathic hypereosinophilic syndrome involving the liver. During the last three years, features of liver involvement in nine of 20 patients with idiopathic hypereosinophilic syndrome were evaluated on CT. The shape and distribution of intrahepatic low densities and the presence of hepatomegaly and/or splenomegaly were reviewed on CT, and the percentage of eosinophils in peripheral blood was also determined. In seven cases, interval change in hepatic lesion and the percentage of eosinophils were reviewed on follow-up examination. On initial CT, varying low-density patterns were seen in the liver in all cases; hepatomegaly was seen in four cases, and hepatosplenomegaly in two. The percentage of eosinophils was 89% in a case with diffuse patch low densities in the liver, 65-85% in three cases with numerous nodular low density lesions, 12-29% in four cases with multiple (below ten) nodular or small geographic hypodense lesions, and 24% in a case with a single nodular hypodense lesion. On follow-up CT, seven patients showed a decrease in the percentage of eosinophils, and in six, improved intrahepatic low densities were seen. On CT, intrahepatic low densities were seen in patients with idiopathic hypereosinophilic syndrome, and these were distributed more extensively when peripheral eosinophilia was more severe. With improvement in peripheral eosinophilia, the low densities also improved.

  1. Idiopathic hypereosinophilic syndrome involving the liver: CT features vs. peripheral eosinophilia

    International Nuclear Information System (INIS)

    Kim, Kyung Sook; Lee, Moon Gyu; Won, Young Chul; Lee, Eun Hye; Noh, Han Na; Ha, Hyun Kwon; Kim, Pyo Nyun; Auh, Yong Ho

    1997-01-01

    To correlate CT features with peripheral eosinophilia in patients with idiopathic hypereosinophilic syndrome involving the liver. During the last three years, features of liver involvement in nine of 20 patients with idiopathic hypereosinophilic syndrome were evaluated on CT. The shape and distribution of intrahepatic low densities and the presence of hepatomegaly and/or splenomegaly were reviewed on CT, and the percentage of eosinophils in peripheral blood was also determined. In seven cases, interval change in hepatic lesion and the percentage of eosinophils were reviewed on follow-up examination. On initial CT, varying low-density patterns were seen in the liver in all cases; hepatomegaly was seen in four cases, and hepatosplenomegaly in two. The percentage of eosinophils was 89% in a case with diffuse patch low densities in the liver, 65-85% in three cases with numerous nodular low density lesions, 12-29% in four cases with multiple (below ten) nodular or small geographic hypodense lesions, and 24% in a case with a single nodular hypodense lesion. On follow-up CT, seven patients showed a decrease in the percentage of eosinophils, and in six, improved intrahepatic low densities were seen. On CT, intrahepatic low densities were seen in patients with idiopathic hypereosinophilic syndrome, and these were distributed more extensively when peripheral eosinophilia was more severe. With improvement in peripheral eosinophilia, the low densities also improved

  2. Idiopathic global unclassified ectrodactyly – A case report | Udigwe ...

    African Journals Online (AJOL)

    Congenital digital limb anomalies are not very common and are usually associated with congenital syndromes. They present enormous challenges to the managing team due to the intricacies involved while the parents go through a lot of anxiety. The case presented here is an isolated idiopathic form of digital anomalies ...

  3. Idiopathic central precocious puberty in a Nigerian boy | Ojukwu ...

    African Journals Online (AJOL)

    A case of idiopathic central precocious puberty, a rare condition is reported in a 21/2 year-old Nigerian boy. He presented with progressive genital growth, a growth spurt, advanced skeletal maturation, and inappropriately high serum concentrations of pituitary and gonadal sex homones for his age. There was no family ...

  4. Consanguineous marriage and increased risk of idiopathic congenital talipes equinovarus: a case-control study in a rural area.

    Science.gov (United States)

    Sahin, Orcun; Yildirim, Cengiz; Akgun, Rahmi C; Haberal, Bahtiyar; Yazici, Ayse C; Tuncay, Ismail C

    2013-01-01

    The purpose of this study is to evaluate if there is any relationship between consanguineous marriages and idiopathic congenital talipes equinovarus (CTEV). A case-control study on CTEV screening was conducted in a rural eastern city of Turkey between 2009 and 2011 and a total of 28 cases (infants with idiopathic CTEV) and 575 controls (healthy infants) were recruited. Sociodemographic status of the infants, including gestational age and birth weights, maternal characteristics and, if any, the degree of consanguinity, were recorded. As an inclusion criterion, only singleton, full-term, live births were accepted. A backward stepwise logistic regression model was used to evaluate the relationship between idiopathic CTEV and parental consanguinity. Unadjusted and adjusted odds ratios (OR) with 95% confidence interval (CI) were calculated. Among maternal and infant characteristics, significant risk factors for idiopathic CTEV in the regression analysis were work status (employed), consanguineous marriage, sex (male), and gestational age (>42 wk). Babies born to first-cousin parents had >4 times the risk of idiopathic CTEV [OR, 4.138, (95% CI, 1.484, 11.538)] and the risk for those born to distant relatives was 2.9 times higher [OR, 2.941, (95% CI, 1.070, 8.087)] than for children of unrelated parents. Consanguineous marriage was significantly associated with an increased risk of idiopathic CTEV. This association remained significant even after adjusting for potential confounding variables. To obtain more accurate results, a population-based screening study with an increased number of cases and controls should be performed in future studies. Case-control study investigating the effect of a patient characteristic on the outcome of disease (level-III).

  5. Manual Therapy in the Treatment of Idiopathic Scoliosis. Analysis of Current Knowledge.

    Science.gov (United States)

    Czaprowski, Dariusz

    2016-10-28

    Apart from the recommended specific physiotherapy, the treatment of idiopathic scoliosis (IS) also incorporates non-specific manual therapy (NMT). The aim of this paper is to assess the efficacy of NMT (manual therapy, chiropractic, osteopathy) used in the treatment of children and adolescents with IS. The study analysed systematic reviews (Analysis 1) and other recent scientific publications (Analysis 2). Analysis 1 encompassed papers on the use of NMT in patients with IS. Works concerning specific physiotherapy (SP) or bracing (B) and other types of scoliosis were excluded from the analysis. Inclusion criteria for Analysis 2 were: treatment with NMT; subjects aged 10-18 years with IS. The following types of papers were excluded: works analysing NMT combined with SP or B, reports concerning adult pa tients, analyses of single cases and publications included in Analysis 1. Analysis 1: six systematic reviews contained 6 papers on the efficacy of NMT in the treatment of IS. The results of these studies are contradictory, ranging from Cobb angle reduction to no treatment effects whatsoever. The papers analysed are characterised by poor methodological quality: small group sizes, incomplete descriptions of the study groups, no follow-up and no control groups. Analysis 2: in total, 217 papers were found. None of them met the criteria set for the analysis. 1. Few papers verifying the efficacy of manual therapy, chiropractic and osteopathy in the treatment of idiopathic scoliosis have been published to date. 2. The majority are experimental studies with poor methodology or observational case studies. 3. At present, the efficacy of non-specific manual therapy in the treatment of patients with idiopathic scoliosis cannot be reliably evaluated. 4. It is necessary to conduct further research based on appropriate methods (prospective, rando mi s ed, controlled studies) in order to reliably assess the usefulness of non-specific manual therapy in the treatment of idiopathic

  6. Ball tip method for thoracic pedicle screw placement in patients with adolescent idiopathic scoliosis

    International Nuclear Information System (INIS)

    Watanabe, Kota; Matsumoto, Morio; Iizuka, Shingo

    2008-01-01

    The purpose of this study was to evaluate the efficacy of ball tip method for thoracic pedicle screw placements in idiopathic scoliosis patients. 24 patients with adolescent idiopathic scoliosis were included in this study. Conventional method was performed in 12 patients. Ball tip method was performed in 12 patients. Accuracy of the pedicle screw placement was evaluated based on the postoperative CT. In the ball tip method, a probe which was consisted of ball tip with flexible shaft was used. After removing of cortical bone at a starting point, the probe was inserted manually or sometimes with gently tapping by hammer. During the maneuver, the probe will gradually progress into cancellous bone in the pedicle, without perforating cortical bone in the pedicle. Following expansion of the hole by a rigid gear shift probe, screw was placed in the pedicle. 65.1% of screws were located within pedicle in the conventional group and 86.5% in the ball tip group. 5.3% of screws were located out of pedicle within 2 mm in the conventional group and 8.2% in the ball tip group. 15.8% of screws were located out of pedicle beyond 2 mm and 1.8% in the ball tip group. The ball tip method enhanced the accuracy of thoracic pedicle screw placements in adolescent idiopathic scoliosis patients. The ball tip method may be effective for accurate pedicle screw placement in patients with adolescent idiopathic scoliosis. (author)

  7. Idiopathic thrombocytopenic purpura during pregnancy

    Directory of Open Access Journals (Sweden)

    Tânia Regina Padovani

    2012-04-01

    Full Text Available ABSTRACT This essay is based on a medical case of idiopathic thrombocytopenic purpura (ITP during pregnancy. The cause of ITP is unknown, who suffer from this disorder, generate antibodies that destroy thrombocytes from their blood. ITP affects women of childbearing age and is associated to maternal and fetal complications. The management of a pregnant patient is difficult and requires the combined care of an obstetrician, a hematologist, and a neonatologist. The main therapeutic options for ITP in pregnant women include glucocorticoids and intravenous immunoglobulin. Splenectomy may be (performed in refractory cases. There is no concerning the management and treatment of pregnant women.

  8. Surgical repair of idiopathic scrotal elephantiasis.

    Science.gov (United States)

    Zacharakis, Evangelos; Dudderidge, Tim; Zacharakis, Emmanouil; Ioannidis, Evangelos

    2008-02-01

    Scrotal lymphedema (scrotal elephantiasis) is uncommon outside of filariasis endemic regions. We present a case of a 65-year-old with idiopathic lymphedema of the scrotum and functional impairment of the penis. The patient underwent surgical excision of the edematous subcutaneous tissues and plastic reconstruction of his penis and scrotum. Three years later, the patient showed no signs of local recurrence, had complete restoration of urinary and sexual function and was extremely satisfied with the result. Surgical management was an effective strategy in the management of scrotal lymphedema in this case.

  9. Potential novel targets: Protease-activated receptors in idiopathic pulmonary fibrosis

    NARCIS (Netherlands)

    Lin, C.

    2015-01-01

    Idiopathic pulmonary fibrosis (IPF) is the most devastating diffuse fibrosing lung disease of unknown etiology. IPF patients suffer from severe breathlessness caused by decreasing lung compliance eventually leading to respiratory failure and death. The prognosis of IPF is devastating: there is only

  10. Lysyl oxidases regulate fibrillar collagen remodelling in idiopathic pulmonary fibrosis

    NARCIS (Netherlands)

    Tjin, Gavin; White, Eric S; Faiz, Alen; Sicard, Delphine; Tschumperlin, Daniel J; Mahar, Annabelle; Kable, Eleanor P W; Burgess, Janette K

    2017-01-01

    Idiopathic pulmonary fibrosis (IPF) is a progressive scarring disease of the lung with feweffective therapeutic options. Structural remodelling of the extracellular matrix [i.e. collagen cross-linkingmediated by the lysyl oxidase (LO) family of enzymes (LOX, LOXL1-4)] might contribute to disease

  11. The adolescent experience in Juvenile Idiopathic Arthritis: A narrative approach

    NARCIS (Netherlands)

    Fuchs, C.E.

    2013-01-01

    This dissertation focused on the self-experience of adolescents with juvenile idiopathic arthritis (JIA) or chronic fatigue syndrome (CFS). Although the etiology and nosology of JIA and CFS are fundamentally different, some commonalities in the emotional experience of adolescents dealing with these

  12. Intra-observer reproducibility and interobserver reliability of the radiographic parameters in the Spinal Deformity Study Group's AIS Radiographic Measurement Manual.

    Science.gov (United States)

    Dang, Natasha Radhika; Moreau, Marc J; Hill, Douglas L; Mahood, James K; Raso, James

    2005-05-01

    Retrospective cross-sectional assessment of the reproducibility and reliability of radiographic parameters. To measure the intra-examiner and interexaminer reproducibility and reliability of salient radiographic features. The management and treatment of adolescent idiopathic scoliosis (AIS) depends on accurate and reproducible radiographic measurements of the deformity. Ten sets of radiographs were randomly selected from a sample of patients with AIS, with initial curves between 20 degrees and 45 degrees. Fourteen measures of the deformity were measured from posteroanterior and lateral radiographs by 2 examiners, and were repeated 5 times at intervals of 3-5 days. Intra-examiner and interexaminer differences were examined. The parameters include measures of curve size, spinal imbalance, sagittal kyphosis and alignment, maximum apical vertebral rotation, T1 tilt, spondylolysis/spondylolisthesis, and skeletal age. Intra-examiner reproducibility was generally excellent for parameters measured from the posteroanterior radiographs but only fair to good for parameters from the lateral radiographs, in which some landmarks were not clearly visible. Of the 13 parameters observed, 7 had excellent interobserver reliability. The measurements from the lateral radiograph were less reproducible and reliable and, thus, may not add value to the assessment of AIS. Taking additional measures encourages a systematic and comprehensive assessment of spinal radiographs.

  13. Idiopathic omental hemorrhage: A case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Jiro Kimura

    2016-01-01

    Conclusion: Idiopathic omental hemorrhage is a rare cause of an acute abdomen, which may develop after eating. Omentectomy is preferred to ligation or transcatheter arterial embolization to rule out an underlying malignancy or aneurysm.

  14. [Qilin Pills combined with clomiphene for idiopathic oligoasthenozoospermia].

    Science.gov (United States)

    Yang, Ming-gen; Zheng, Gou-da; Xu, Zhen-qiang; Lin, Hai-li; Zhuang, Zhi-ming; Zhang, Chao-xian

    2015-06-01

    To observe the therapeutic effect of Qilin Pills combined with clomiphene on idiopathic oligoasthenospermia. We randomly assigned 300 patients with idiopathic oligoasthenospermia to a trial (n = 156) and a control group (n = 144) to be treated with Qilin Pills (6 g, tid) combined with clomiphene (50 mg, qd) and clomiphene alone (50 mg, qd), respectively, both for a course of 12 weeks. Before and after 4, 8, and 12 weeks of medication, we determined sperm concentration, the percentages of grade a and grade a + b sperm, sperm motility, and the levels of follicle-stimulating hormone (FSH), luteinizing hormone (LH), and testosterone (T), followed by evaluation of the clinical efficacy of Qilin Pills with the pregnancy rate in the patients' spouses as the secondaty therapeutic indexes. Compared with the baseline, both groups of patients showed remarkably improved semen parameters and hormone levels after treatment (all P 0.05). No obvious adverse reactions were observed. Qilin Pills combined with clomiphene can evidently improve the seminal quality and hormone level of oligoasthenospermia patients with no obvious adverse events. However, its long-term efficacy and tolerance deserve further clinical investigation.

  15. Stellate ganglion block for persistent idiopathic facial pain

    Directory of Open Access Journals (Sweden)

    Poonam Patel

    2016-01-01

    Full Text Available Persistent idiopathic facial pain is a facial pain disorder without any identifiable cause. A patient has persistent facial pain without any objective sign on clinical examination or investigations. There are associated psychological problems such as depression and anxiety. This condition is poorly responsive to therapy with anticonvulsants or analgesics. Stellate ganglion block interrupts the sympathetic supply to head, neck, and upper extremities. This block can be used to alleviate pain of sympathetic origin in head and neck region as well as upper extremities. We report a case of a middle-aged female with persistent idiopathic facial pain on the right side of face with no response to analgesics and anticonvulsants. Her pain was provoked by exposure to cold weather or wind. Assuming a sympathetic component to her pain, we did a right-sided stellate ganglion block for her with local anesthetic and steroid. The patient had significant pain relief (>80% after the block. This indicates that the sympathetic nervous system plays a major role in initiation and perpetuation of this pain condition. Stellate ganglion block can be done early in such patients both as a diagnostic and therapeutic modality.

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

    OpenAIRE

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

    2014-01-01

    Background Juvenile idiopathic arthritis is a relatively common chronic disease of childhood, and is associated with persistent morbidity and extra-articular complications, one of the most common being uveitis. The introduction of biologic therapies, particularly those blocking the inflammatory mediator tumor necrosis factor-α, provided a new treatment option for juvenile idiopathic arthritis patients who were refractory to standard therapy such as non-steroidal anti-inflammatory drugs, corti...

  17. Objective and subjective analysis of women's voice with idiopathic Parkinson's disease

    Directory of Open Access Journals (Sweden)

    Riviana Rodrigues das Graças

    2012-07-01

    Full Text Available OBJECTIVE: To compare the voice quality of women with idiopathic Parkinson's disease and those without it. METHODS: An evaluation was performed including 19 female patients diagnosed with idiopathic Parkinson's disease, with an average age of 66 years, and 27 women with an average of 67 years-old in the Control Group. The assessment was performed by computed acoustic analysis and perceptual evaluation. RESULTS: Parkinson's disease patients presented moderate rough and unstable voice quality. The parameters of grade, roughness, and instability had higher scores in Parkinson's disease patients with statistically significant differences. Acoustic measures of Jitter and period perturbation quotient (PPQ significantly differ between groups. CONCLUSIONS: Parkinson's disease female individuals showed more vocal alterations compared to the Control Group, when both perceptual and acoustic evaluations were analyzed.

  18. Bilateral idiopathic optic perineuritis with severe vision loss: A case report

    Directory of Open Access Journals (Sweden)

    Wee-Min Teh

    2015-07-01

    Full Text Available Optic perineuritis is an orbital inflammatory disorder that is either idiopathic or secondary to other conditions such as infection or systemic inflammatory disorders. This condition is very similar to demyelinating optic neuritis, but certain features of the history and magnetic resonance imaging findings are characteristic for and aid in the diagnosis of optic perineuritis. Vision loss varies greatly, from minimal clouding of vision up to only light perception. We report a case of a 44-year-old female with idiopathic bilateral optic perineuritis with vision loss of up to no light perception in both eyes. Radio imaging studies were typical of optic perineuritis and she was started on systemic corticosteroids. She responded very well to steroid therapy and achieved nearly complete visual recovery. There had been no relapse despite cessation of therapy.

  19. q-Deformed KP Hierarchy and q-Deformed Constrained KP Hierarchy

    OpenAIRE

    He, Jingsong; Li, Yinghua; Cheng, Yi

    2006-01-01

    Using the determinant representation of gauge transformation operator, we have shown that the general form of $au$ function of the $q$-KP hierarchy is a $q$-deformed generalized Wronskian, which includes the $q$-deformed Wronskian as a special case. On the basis of these, we study the $q$-deformed constrained KP ($q$-cKP) hierarchy, i.e. $l$-constraints of $q$-KP hierarchy. Similar to the ordinary constrained KP (cKP) hierarchy, a large class of solutions of $q$-cKP hierarchy can be represent...

  20. Comparison of low density and high density pedicle screw instrumentation in Lenke 1 adolescent idiopathic scoliosis.

    Science.gov (United States)

    Shen, Mingkui; Jiang, Honghui; Luo, Ming; Wang, Wengang; Li, Ning; Wang, Lulu; Xia, Lei

    2017-08-02

    The correlation between implant density and deformity correction has not yet led to a precise conclusion in adolescent idiopathic scoliosis (AIS). The aim of this study was to evaluate the effects of low density (LD) and high density (HD) pedicle screw instrumentation in terms of the clinical, radiological and Scoliosis Research Society (SRS)-22 outcomes in Lenke 1 AIS. We retrospectively reviewed 62 consecutive Lenke 1 AIS patients who underwent posterior spinal arthrodesis using all-pedicle screw instrumentation with a minimum follow-up of 24 months. The implant density was defined as the number of screws per spinal level fused. Patients were then divided into two groups according to the average implant density for the entire study. The LD group (n = 28) had fewer than 1.61 screws per level, while the HD group (n = 34) had more than 1.61 screws per level. The radiographs were analysed preoperatively, postoperatively and at final follow-up. The perioperative and SRS-22 outcomes were also assessed. Independent sample t tests were used between the two groups. Comparisons between the two groups showed no significant differences in the correction of the main thoracic curve and thoracic kyphosis, blood transfusion, hospital stay, and SRS-22 scores. Compared with the HD group, there was a decreased operating time (278.4 vs. 331.0 min, p = 0.004) and decreased blood loss (823.6 vs. 1010.9 ml, p = 0.048), pedicle screws needed (15.1 vs. 19.6, p density and high density pedicle screw instrumentation achieved satisfactory deformity correction in Lenke 1 AIS patients. However, the operating time and blood loss were reduced, and the implant costs were decreased with the use of low screw density constructs.