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Sample records for juvenile localized scleroderma

  1. [Juvenile scleroderma].

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    de Mâcedo, Patrícia Andrade; Shinjo, Samuel Katsuyuki; Goldenstein-Schainberg, Cláudia

    2008-01-01

    Juvenile scleroderma is a rare childhood condition characterized by fibrosis of the skin and internal organs. Clinical manifestations of childhood scleroderma are different from adult disease and early recognition, correct classification and treatment can improve long-term outcome. This review explores the most recent actualizations on clinical manifestations, classification criteria, treatment options and prognosis of juvenile scleroderma. There are two main forms of the disease: localized scleroderma and systemic sclerosis. Localized scleroderma is the most common form in children and mostly restricted to the skin. Juvenile diffuse systemic sclerosis is related to visceral involvement and cardiac disease which is the main cause of death in these patients. The outcome of juvenile systemic sclerosis is better compared with the adult form. Treatment remains a medical challenge and the EULAR task force proposed an approach to juvenile scleroderma treatment based on expert's opinion and guidelines used for the treatment of adults. Larger studies on childhood scleroderma are warranted.

  2. Musculoskeletal MRI findings of juvenile localized scleroderma

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    Eutsler, Eric P. [Nemours Children' s Health System/Alfred I. duPont Hospital for Children, Wilmington, DE (United States); Washington University School of Medicine, Mallinckrodt Institute of Radiology, St. Louis, MO (United States); Horton, Daniel B. [Nemours Children' s Health System/Alfred I. duPont Hospital for Children, Division of Rheumatology, Department of Pediatrics, Wilmington, DE (United States); Rutgers Robert Wood Johnson Medical School, Department of Pediatrics, New Brunswick, NJ (United States); Epelman, Monica [Nemours Children' s Health System/Nemours Children' s Hospital, Department of Medical Imaging, Orlando, FL (United States); Finkel, Terri [Nemours Children' s Health System/Nemours Children' s Hospital, Department of Pediatrics, Orlando, FL (United States); Averill, Lauren W. [Nemours Children' s Health System/Alfred I. duPont Hospital for Children, Wilmington, DE (United States)

    2017-04-15

    Juvenile localized scleroderma comprises a group of autoimmune conditions often characterized clinically by an area of skin hardening. In addition to superficial changes in the skin and subcutaneous tissues, juvenile localized scleroderma may involve the deep soft tissues, bones and joints, possibly resulting in functional impairment and pain in addition to cosmetic changes. There is literature documenting the spectrum of findings for deep involvement of localized scleroderma (fascia, muscles, tendons, bones and joints) in adults, but there is limited literature for the condition in children. We aimed to document the spectrum of musculoskeletal magnetic resonance imaging (MRI) findings of both superficial and deep juvenile localized scleroderma involvement in children and to evaluate the utility of various MRI sequences for detecting those findings. Two radiologists retrospectively evaluated 20 MRI studies of the extremities in 14 children with juvenile localized scleroderma. Each imaging sequence was also given a subjective score of 0 (not useful), 1 (somewhat useful) or 2 (most useful for detecting the findings). Deep tissue involvement was detected in 65% of the imaged extremities. Fascial thickening and enhancement were seen in 50% of imaged extremities. Axial T1, axial T1 fat-suppressed (FS) contrast-enhanced and axial fluid-sensitive sequences were rated most useful. Fascial thickening and enhancement were the most commonly encountered deep tissue findings in extremity MRIs of children with juvenile localized scleroderma. Because abnormalities of the skin, subcutaneous tissues and fascia tend to run longitudinally in an affected limb, axial T1, axial fluid-sensitive and axial T1-FS contrast-enhanced sequences should be included in the imaging protocol. (orig.)

  3. Juvenile Scleroderma

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    Juvenile Scleroderma INTRODUCTION Every parent will experience a moment of panic when told their child has scleroderma. ... in all their family members as well. CONCLUSION Juvenile scleroderma can be unsettling for the child and ...

  4. Localized Scleroderma

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    ... the skin and cannot be removed easily by bleaching agents. Linear scleroderma is more common in children ... the scalp, it can cause varied degrees of hair loss. When involving the face, it can lead ...

  5. Successful combination treatment of a patient with progressive juvenile localized scleroderma (morphea) using imatinib, corticosteroids, and methotrexate.

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    Inamo, Yasuji; Ochiai, Toyoko

    2013-01-01

    We report a case of progressive juvenile localized scleroderma (JLS or morphea) treated with a combination of imatinib, corticosteroids, and methotrexate. This therapy halted the progressive skin thickening and the hand and finger joint deformity in the early stages of the disease. We conclude that imatinib used in addition to standard treatment with systemic corticosteroids and methotrexate may be of therapeutic benefit for individuals with JLS.

  6. SUBTYPES OF JUVENILE SYSTEMIC SCLERODERMA

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    M N Slarovoitova

    2001-01-01

    Full Text Available Aim: to allot clinical forms of juvenile systemic scleroderma (JSSD. Material and methods: investigation and dynamic observation of 60 patients aged 14-54 (mean age 25.1 ±7.2 with onset of disease in child's and adolescent’s ages from 1 to 16 years old ( in average 11. 4±3.8 year old and disease duration from 1 to 39 years (in average 13.1 ±7.9. Results: 55% of patients demonstrated JSSD subtype with focal cutaneous lesion of different localization. The possibility of overlap-syndrome development in JSSD patients with onset in adolescent age typical for SSD-rheumatoid arthritis, SSD-polymvositis should be underlined. Conclusion: knowledge of different clinical forms and courses of the disease, modern diagnostics and early beginning of differential JSSD treatment will enable us to improve the prognosis and disease outcome.

  7. Localized and systemic scleroderma.

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    Hawk, A; English, J C

    2001-03-01

    Scleroderma is a broad term encompassing both localized and systemic sclerosis. Localized scleroderma is a cutaneous limited fibrosis that manifests as plaque morphea, generalized morphea, linear scleroderma, and deep morphea. Systemic scleroderma (sclerosis) can manifest as either limited or diffuse disease. Limited systemic sclerosis is typically preceded by Raynaud's phenomenon, involves cutaneous sclerosis distal to the elbows, with gastrointestinal and pulmonary fibrosis, and anticentromere antibody positivity. Diffuse systemic scleroderma is characterized by simultaneous Raynaud's phenomenon, cutaneous skin involvement proximal to the elbow with gastrointestinal, pulmonary, renal and cardiac fibrosis, and positive serology for antitopoisomerase and anti-RNAP III antibodies. This article discusses the classification, epidemiology, pathogenesis, clinical manifestations, treatment, and prognosis of the scleroderma.

  8. Presentations and treatment of childhood scleroderma: localized scleroderma, eosinophilic fasciitis, systemic sclerosis, and graft-versus-host disease.

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    Hedrich, Christian Michael; Fiebig, Barbara; Hahn, Gabriele; Suttorp, Meinolf; Gahr, Manfred

    2011-07-01

    Juvenile scleroderma is a rare connective tissue disease that involves the skin and subcutaneous tissue. Among all presentations of juvenile scleroderma, localized scleroderma (JLSc) is the most frequent, followed by systemic disease (JSSc) and eosinophilic fasciitis (EF). In posttransplantation chronic graft-versus-host disease (GvHD), scleroderma-like skin involvement can occur. Systemic forms of juvenile scleroderma and GvHD can affect the internal organs, such as the lungs, the gastrointestinal tract, the heart, and kidneys and cause disability and severe, sometimes lethal, complications. Here, the authors give an overview of different presentations of juvenile scleroderma. They report their experience with the different forms and presentations of scleroderma, diagnostic workups, treatment, and outcome of all forms of childhood scleroderma in the context of the existing literature.

  9. Scleroderma

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    ... Limited scleroderma; CREST syndrome; Localized scleroderma; Morphea - linear; Raynaud's phenomenon - scleroderma ... blue or white in response to cold temperatures ( Raynaud phenomenon ) Stiffness and tightness of skin of fingers, hands, ...

  10. EXPERIENCE OF RITUXIMAB TREATMENT IN A PATIENT WITH JUVENILE SCLERODERMA

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    E. I. Alexeeva

    2012-01-01

    Full Text Available A clinical case of severe juvenile scleroderma is represented in this article. The patient had a high activity and aggressive course of disease, he was resistant to steroid, cyclophosphomide and methotrexate therapy in combination with drugs, improving blood circulation. The authors describe the successful usage of chimeric monoclonal antibody against the protein CD20 — Rituximab. By the 4th week of the treatment the signs of intoxication and local manifestations of the disease (density and area of scleroderma patches have diminished. By the 24th week the immunological markers of activity have become normal. Afore-mentioned clinical case demonstrates high efficacy of Rituximab in patient with severe course of juvenile scleroderma. By now the stage of clinical and laboratory remission has maintained for 52 weeks.

  11. Localized scleroderma: imaging features

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    Liu, P. (Dept. of Diagnostic Imaging, Hospital for Sick Children, Toronto, ON (Canada)); Uziel, Y. (Div. of Rheumatology, Hospital for Sick Children, Toronto, ON (Canada)); Chuang, S. (Dept. of Diagnostic Imaging, Hospital for Sick Children, Toronto, ON (Canada)); Silverman, E. (Div. of Rheumatology, Hospital for Sick Children, Toronto, ON (Canada)); Krafchik, B. (Div. of Dermatology, Dept. of Pediatrics, Hospital for Sick Children, Toronto, ON (Canada)); Laxer, R. (Div. of Rheumatology, Hospital for Sick Children, Toronto, ON (Canada))

    1994-06-01

    Localized scleroderma is distinct from the diffuse form of scleroderma and does not show Raynaud's phenomenon and visceral involvement. The imaging features in 23 patients ranging from 2 to 17 years of age (mean 11.1 years) were reviewed. Leg length discrepancy and muscle atrophy were the most common findings (five patients), with two patients also showing modelling deformity of the fibula. One patient with lower extremity involvement showed abnormal bone marrow signals on MR. Disabling joint contracture requiring orthopedic intervention was noted in one patient. In two patients with ''en coup de sabre'' facial deformity, CT and MR scans revealed intracranial calcifications and white matter abnormality in the ipsilateral frontal lobes, with one also showing migrational abnormality. In a third patient, CT revealed white matter abnormality in the ipsilateral parietal lobe. In one patient with progressive facial hemiatrophy, CT and MR scans showed the underlying hypoplastic left maxillary antrum and cheek. Imaging studies of areas of clinical concern revealed positive findings in half our patients. (orig.)

  12. Localized scleroderma: clinical spectrum and therapeutic update *

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    Careta,Mariana Figueiroa; Romiti, Ricardo

    2015-01-01

    Scleroderma is a rare connective tissue disease that is manifested by cutaneous sclerosis and variable systemic involvement. Two categories of scleroderma are known: systemic sclerosis, characterized by cutaneous sclerosis and visceral involvement, and localized scleroderma or morphea which classically presents benign and self-limited evolution and is confined to the skin and/or underlying tissues. Localized scleroderm...

  13. [Localized scleroderma (morphea) in childhood].

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    Weibel, L

    2012-02-01

    Localized scleroderma or morphea is a sclerosing connective tissue disease of the skin, which may affect underlying tissues such as subcutis, muscle and bone. Many patients show extracutaneous symptoms and antinuclear antibodies, however, secondary transformation into systemic sclerosis does not occur. Localized scleroderma usually begins in childhood with a wide variation in its clinical spectrum. The linear variant is the most common subtype in children, associated with a progressive course and increased risk of complications. The disease may progress over years and result in severe functional and cosmetic disability. The etiology of localized scleroderma remains unknown. A genetic background is suspected, while triggers such as trauma, vaccinations and infections may lead to secondary immunologic phenomena. Localized scleroderma often remains unrecognized for a long time, resulting in substantial delay in treatment. The combination of systemic corticosteroids and methotrexate has been established as first-line therapy for progressive (usually linear) disease, whereas phototherapy (UVA-1 or UVB-narrow band) is suitable for adolescents with superficial circumscribed subtypes.

  14. A Practical Approach to Juvenile Dermatomyositis and Juvenile Scleroderma.

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    McCann, Liza J; Pain, Clare E

    2016-02-01

    Juvenile dermatomyositis and juvenile scleroderma are rare multisystem autoimmune disorders. Although they share some pathognomonic hallmarks with adult onset myositis or scleroderma, there are significant differences in presentation, characteristics and associated features when the diseases present in childhood. In view of this, and the rarity of the conditions, it is important for care to be led by teams with expertise in pediatric rheumatology conditions. Prognosis has improved significantly in the West; likely due to early diagnosis and aggressive treatment with immunosuppressive medications. However, this trend is not replicated in the developing world. Early recognition of these diseases is crucial to achieve rapid and sustained remission and prevent disease or medication associated complications. This article aims to provide a practical overview for recognition, diagnosis and treatment of these conditions.

  15. Localized scleroderma: clinical spectrum and therapeutic update.

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    Careta, Mariana Figueiroa; Romiti, Ricardo

    2015-01-01

    Scleroderma is a rare connective tissue disease that is manifested by cutaneous sclerosis and variable systemic involvement. Two categories of scleroderma are known: systemic sclerosis, characterized by cutaneous sclerosis and visceral involvement, and localized scleroderma or morphea which classically presents benign and self-limited evolution and is confined to the skin and/or underlying tissues. Localized scleroderma is a rare disease of unknown etiology. Recent studies show that the localized form may affect internal organs and have variable morbidity. Treatment should be started very early, before complications occur due to the high morbidity of localized scleroderma. In this review, we report the most important aspects and particularities in the treatment of patients diagnosed with localized scleroderma.

  16. Localized severe scleroderma: a retrospective study of 26 pediatric patients.

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    Beltramelli, Matilde; Vercellesi, Paolo; Frasin, Adina; Gelmetti, Carlo; Corona, Fabrizia

    2010-01-01

    Juvenile localized scleroderma includes different conditions characterized by skin hardening with increased collagen deposition. Although juvenile localized scleroderma is considered a relatively benign disease, lesions may extend through the dermis, subcutaneous tissue, muscles, and the underlying bone, leading to significant functional and cosmetic deformities. Furthermore, extracutaneous manifestations are described. We retrospectively analyzed a cohort of 26 patients with severe Juvenile localized scleroderma with particular attention to clinical features, therapy, and long-term outcome. A subgroup of three patients has been further evaluated with infrared thermography. Our findings were consistent with the current literature for demographic, laboratory, and clinical characteristics at disease onset, but, with our patients, the prevalence of extracutaneous manifestations was higher, thus confirming the potential for severe juvenile localized scleroderma to affect organs other than the skin, without increased risk of development toward systemic sclerosis. Correlation between various treatments and clinical endpoint showed that systemic therapy lead to a better outcome: in particular, methotrexate appeared the most effective drug, capable in halting the progression of the disease and sometimes inducing its regression.

  17. Pediatric scleroderma: systemic or localized forms.

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    Torok, Kathryn S

    2012-04-01

    Pediatric scleroderma includes 2 major groups of clinical entities, systemic sclerosis (SSc) and localized scleroderma (LS). Although both share a common pathophysiology, their clinical manifestations differ. LS is typically confined to the skin and underlying subcutis, with up to a quarter of patients showing extracutaneous disease manifestations such as arthritis and uveitis. Vascular, cutaneous, gastrointestinal, pulmonary, and musculoskeletal involvement are most commonly seen in children with SSc. Treatment of both forms targets the active inflammatory stage and halts disease progression; however, progress needs to be made toward the development of more effective antifibrotic therapy to help reverse disease damage.

  18. Pathogenesis and treatment modalities of localized scleroderma.

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    Valančienė, Greta; Jasaitienė, Daiva; Valiukevičienė, Skaidra

    2010-01-01

    Localized scleroderma is a chronic inflammatory disease primarily of the dermis and subcutaneous fat that ultimately leads to a scar-like sclerosis of connective tissue. The disorder manifests as various plaques of different shape and size with signs of skin inflammation, sclerosis, and atrophy. This is a relatively rare inflammatory disease characterized by a chronic course, unknown etiology, and insufficiently clear pathogenesis. Many factors may influence its appearance: trauma, genetic factors, disorders of the immune system or hormone metabolism, viral infections, toxic substances or pharmaceutical agents, neurogenic factors, and Borrelia burgdorferi infection. Various therapeutic modalities are being used for the treatment of localized scleroderma. There is no precise treatment scheme for this disease. A majority of patients can be successfully treated with topical pharmaceutical agents and phototherapy, but some of them with progressive, disseminated, and causing disability localized scleroderma are in need of systemic treatment. The aim of this article is not only to dispute about the clinical and morphological characteristics of localized scleroderma, but also to present the newest generalized data about the possible origin, pathogenesis, and treatment modalities of this disease.

  19. [Nephrotic syndrome as the first manifestation of juvenile systemic scleroderma.

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    Couto, Saulo B; Sallum, Adriana M; Henriques, Luciana S; Malheiros, Denise M; Silva, Clovis A; Vaisbich, Maria H

    2014-10-22

    Renal involvement occurs in 1%-12% in juvenile systemic sclerosis (JSSc) patients, mainly with arterial hypertension, proteinuria and scleroderma renal crisis. We report herein a patient who presented nephrotic syndrome (NS) as the first manifestation of JSSc with focal segmental glomerulosclerosis (FSGS). A female patient presented steroid-sensitive NS at the age of 12 years. At 14 years, she had orbital and lower limbs edema, arterial hypertension, sclerodactyly and proximal skin sclerosis. Moderate capillary dilation and mild focal devascularization were observed in nailfold capillaroscopy, compatible with early stage of scleroderma (scleroderma pattern). Percutaneous renal biopsy guided by ultrasound revealed focal segmental glomerulosclerosis and direct immunofluorescence were negative. Therefore, she fullfilled the provisional classification criteria for JSSc. Patient was treated with oral 25-hydroxyvitamin D (800 IU/day), methotrexate (0.5mg/kg/week) and amlodipin (0.15 mg/kg). Prednisone (60 mg/m(2)/day) was administered for 4 consecutive weeks, followed by alternate-day (40mg/m(2)) for 2 consecutive months, with tapering for 4 months and then stopping this medication. Currently she is being treated with methotrexate 15 mg/week, without edema and proteinuria. In conclusion, we reported a rare case of NS with FSGS as the first manifestation of scleroderma. Therefore, renal biopsy is mandatory in JSSc patients with sustained proteinuria or NS.

  20. Systemic and localized scleroderma in children: current and future treatment options.

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    Rosenkranz, Margalit E; Agle, Lucila M A; Efthimiou, Petros; Lehman, Thomas J A

    2006-01-01

    Scleroderma is a group of rare and complex diseases with varied clinical manifestations. The most obvious manifestation of the diseases is skin hardening and sclerosis. Scleroderma can be divided into two main subgroups: systemic and localized. The systemic form, also known as systemic sclerosis, involves diffuse skin involvement and potentially severe visceral involvement. Localized scleroderma on the other hand is more common in children and usually confined to a specific region of the body with no internal organ involvement. The juvenile forms of systemic sclerosis and localized scleroderma are important conditions in children because of the clinical severity and substantial mortality of systemic scleroderma and the major growth defects associated with childhood-onset localized disease even if the active disease itself is self-limited. The pathogenic pathways of the various forms of scleroderma are only partially defined, but the main defect in scleroderma is abnormal collagen deposition leading to eventual fibrosis in the skin as well as multiple organ systems such as the heart and lungs in juvenile systemic sclerosis. Therapeutics are divided into three main subgroups for systemic sclerosis: antifibrotics, anti-inflammatories, and vasodilators. For localized disease, anti-inflammatories, vitamin D analogs, and UV irradiation have been investigated. However, the infrequency of scleroderma in the pediatric population plus the fact that this disease is very often self-limiting makes randomized controlled trials very difficult. It is for this reason that most data on treatment modalities for this disease have been extrapolated from studies in adult patients. There is no one therapy for systemic sclerosis or localized scleroderma that has proven to be very effective or significantly disease modifying. However, current therapeutic strategies must be initiated early in the disease course for maximum beneficial clinical effects. New interventions such as autologous

  1. Parry-Romberg Syndrome Associated with Localized Scleroderma

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    Jelena Maletic

    2010-06-01

    Full Text Available Parry-Romberg syndrome is a rare neurocutaneous disorder of unknown origin. It is characterized by progressive facial hemiatrophy and frequently overlaps with a condition known as linear scleroderma ‘en coup de sabre’. Neurological involvement is frequently described in these patients, including migraine, facial pain and epilepsy, which represent the commonest neurological conditions, sometimes associated with brain abnormalities ipsilaterally to the skin lesions. We present a case of Parry-Romberg syndrome with neurological involvement in a patient with diagnosed localized scleroderma (morphea.

  2. Update on the classification and treatment of localized scleroderma.

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    Bielsa Marsol, I

    2013-10-01

    Morphea or localized scleroderma is a distinctive inflammatory disease that leads to sclerosis of the skin and subcutaneous tissues. It comprises a number of subtypes differentiated according to their clinical presentation and the structure of the skin and underlying tissues involved in the fibrotic process. However, classification is difficult because the boundaries between the different types of morphea are blurred and different entities frequently overlap. The main subtypes are plaque morphea, linear scleroderma, generalized morphea, and pansclerotic morphea. With certain exceptions, the disorder does not have serious systemic repercussions, but it can cause considerable morbidity. In the case of lesions affecting the head, neurological and ocular complications may occur. There is no really effective and universal treatment so it is important to make a correct assessment of the extent and severity of the disease before deciding on a treatment approach.

  3. Localized and systemic forms of scleroderma in adults and children .

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    Atzeni, F; Bardoni, A; Cutolo, M; Hunzelmann, N; Krieg, T; Martini, G; Montecucco, C; Olski, T M; Secchi, M E; Valentini, G; Zulian, F; Sarzi-Puttini, P

    2006-01-01

    Systemic sclerosis (SSc) presents a great deal of variability in the extent and severity of skin and internal organ involvement. The diagnostic and prognostic significance of autoantibodies in SSc is undisputed and the patient's autoantibody profile represents a fundamental tool for clinicians. Scleroderma is a rare condition in children. Unlike adults, localized scleroderma is more frequent than the systemic sclerosis, nevertheless it represents a disabling condition. In both conditions, no validated outcome measures and proven effective treatment is available to date.Raynaud's phenomenon (RP) is one the most common and significant clinical symptoms of SSc and therefore in patients with RP a capillaroscopic analysis should be carried out as soon as possible. The actual and select advantage of the early nailfold videocapillaroscopic (NVC) analysis is to distinguish between the primary RP and the secondary RP and to allow the early detection of SSc.

  4. A Case Report of Successful Treatment of Recalcitrant Childhood Localized Scleroderma with Infliximab and Leflunomide

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    Ferguson, Ian D; Weiser, Peter; Torok, Kathryn S

    2015-01-01

    Herein we report successful treatment of an adolescent Caucasian female with severe progressive localized scleroderma (mixed subtype, including generalized morphea and linear scleroderma of the trunk/limb) using infliximab and leflunomide. The patient demonstrated improvement after the first 9 months of therapy based on her clinical examination, objective measures, and patient and parent global assessments. Infliximab is a potential treatment option for pediatric localized scleroderma patient...

  5. A Case Report of Successful Treatment of Recalcitrant Childhood Localized Scleroderma with Infliximab and Leflunomide.

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    Ferguson, Ian D; Weiser, Peter; Torok, Kathryn S

    2015-01-01

    Herein we report successful treatment of an adolescent Caucasian female with severe progressive localized scleroderma (mixed subtype, including generalized morphea and linear scleroderma of the trunk/limb) using infliximab and leflunomide. The patient demonstrated improvement after the first 9 months of therapy based on her clinical examination, objective measures, and patient and parent global assessments. Infliximab is a potential treatment option for pediatric localized scleroderma patients who have progression of disease or who are unable to tolerate the side effect profile of more standard systemic therapy. Larger longitudinal studies or case series are needed to confirm and further investigate infliximab's role in localized scleroderma.

  6. Treatment of morphea or localized scleroderma: review of the literature.

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    Vilela, Fernanda Aguiar Santos; Carneiro, Sueli; Ramos-e-Silva, Marcia

    2010-10-01

    Scleroderma is a disease that affects the microvasculature and the connective tissue. These alterations produce fibrosis and blood vessel occlusion. Its cause is still unknown, although the exaggerated synthesis of collagens I and IV, detected in skin and vessels, may be related to genetic, immunologic and, less frequently, exogenous factors as inhalation of silica and polyvinyl chloride. There is a localized and a systemic form, which affects both adults and children. The treatment of the localized form, also called morphea, is still controversial, and, in this article, the authors will discuss the main agents that were found to improve the lesions and symptoms.

  7. Localized scleroderma and its implications on quality of life

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    Nathalia Mebius

    2014-12-01

    Full Text Available Scleroderma is a disease of unknown etiology, which affects the connective tissue, is characterized histologically by a change in collagen synthesis with increased deposition, and dermal infiltration and thickening of the skin, asymmetric distribution without involvement of internal organs in this way (localized of the disease, but can lead, in some cases, important aesthetic and functional impairment of the patient. In our case, it is believed that the disease has already developed completely and now there is an overlap disease of old age, which makes their daily activities and independence.

  8. Evaluation of serum concentrations of the selected cytokines in patients with localized scleroderma

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    Jolanta Budzyńska-Włodarczyk

    2016-02-01

    Full Text Available Introduction : Localized scleroderma is an autoimmune disease primarily affecting the skin. The cause of disease remains unexplained although environmental factors are implicated, which are likely to be responsible for activation of the endothelium and subsequent inflammation leading to excessive synthesis of collagen and extracellular matrix components. Aim: To determine concentrations of interleukin (IL-27, transforming growth factor (TGF-β1, TGF-β2, IL-6, and sIL-6R in patients with localized scleroderma compared to controls and to assess the relations between their levels and laboratory markers. Material and methods: The study encompassed 17 females with localized scleroderma (aged 25–67. The control group consisted of 30 age-matched healthy women. The blood was sampled from the basilic vein. Serum levels of cytokines were determined using ELISA. Results : The TGF-β2 levels were found to be significantly lower in patients with localized scleroderma compared to controls. Concentrations of TGF-β1 were decreased in scleroderma patients when compared to controls but without statistical significance. There were no significant differences in serum IL-6, sIL-6R and IL-27 levels between patients and the control group; however, we found a significant positive correlation between the level of sIL-6 and ESR among subjects with localized scleroderma. Conclusions : The findings of decreased serum levels of TGF-β1 and TGF-β2 in patients with localized scleroderma demonstrate a possible association of these cytokines with pathogenesis of the disease. The results suggest also that sIL-6R is likely to be involved in inflammation in patients with localized scleroderma.

  9. Lichen sclerosus associated with localized scleroderma: dermoscopy contribution*

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    Nóbrega, Monisa Martins; Cabral, Fernanda; Corrêa, Mariana César; Barcaui, Carlos Baptista; Bressan, Aline Lopes; Gripp, Alexandre Carlos

    2016-01-01

    Lichen sclerosus is an uncommon inflammatory dermatosis with preferential involvement of the urogenital region. The extragenital involvement is uncommon and is characterized by small rounded macules or papules, pearly white in color. The coexistence of lichen sclerosus and scleroderma plaques in most cases with extragenital location has been reported in the literature. We report a case of lichen sclerosus associated with scleroderma in children, highlighting the importance of dermoscopy in diagnosis. PMID:27579757

  10. Use of Mycophenolate Mofetil in Patients with Severe Localized Scleroderma Resistant or Intolerant to Methotrexate

    NARCIS (Netherlands)

    Mertens, J.S.; Marsman, D.; Kerkhof, P.C.M. van de; Hoppenreijs, E.P.A.H.; Knaapen, H.K.A.; Radstake, T.R.D.J.; Jong, E.M.G.J. de; Seyger, M.M.B.

    2016-01-01

    To assess the efficacy and safety of mycophenolate mofetil (MMF) in patients with localized scleroderma (LoS) resistant or intolerant to previous treatment with methotrexate (MTX). A case series of patients with LoS treated with MMF. Outcome was assessed through clinical examination. Adverse events

  11. Use of mycophenolate mofetil in patients with severe localized scleroderma resistant or intolerant to methotrexate

    NARCIS (Netherlands)

    Mertens, Jorre S.; Marsman, Diane; van de Kerkhof, Peter C M; Hoppenreijs, Esther P A H; Knaapen, Hanneke K A; Radstake, Timothy R D; de Jong, Elke M G J; Seyger, Marieke M B

    2016-01-01

    To assess the efficacy and safety of mycophenolate mofetil (MMF) in patients with localized scleroderma (LoS) resistant or intolerant to previous treatment with methotrexate (MTX). A case series of patients with LoS treated with MMF. Outcome was assessed through clinical examination. Adverse events

  12. Localized and systemic scleroderma show different histological responses to methotrexate therapy.

    NARCIS (Netherlands)

    Seijger, M.M.B.; Hoogen, F.H.J. van den; Vlijmen-Willems, I.M.J.J. van; Kerkhof, P.C.M. van de; Jong, E.M.G.J. de

    2001-01-01

    Although morphoea (localized scleroderma) and systemic sclerosis are distinct disease entities, the skin lesions show identical histological characteristics and both diseases respond favourably to low-dose treatment with methotrexate (MTX). The aim of this study was to find out whether MTX treatment

  13. Scleroderma with Nodular Scleroderma

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    Chutika Srisuttiyakorn; Kobkul Aunhachoke

    2016-01-01

    Background: Nodular scleroderma is a rare variant of scleroderma which can occur in connection with systemic sclerosis or morphea. A biopsy from the lesion can demonstrate the scleroderma pattern, i.e., keloid pattern or mixed type. Treatment is challenging, and several treatments modalities have been reported with unsatisfactory results. Main Observations: We present a case of systemic sclerosis in a 50-year-old female who developed nodular scleroderma in the absence of deterioration of the ...

  14. 20-MHz B-mode ultrasound in monitoring the course of localized scleroderma (morphea).

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    Hoffmann, K; Gerbaulet, U; el-Gammal, S; Altmeyer, P

    1991-01-01

    Ultrasonographic methods have recently provided us with the means for objective and non-invasive monitoring of the dynamics of chronic skin diseases. We examined 34 patients with localized scleroderma (morphea) using a 20-MHz B-mode ultrasound scanner (DUB 20, Taberna pro Medicum, Lüneburg). In patients with plaque-type and linear band-type localized scleroderma intraindividual comparison of sclerotic skin with corresponding areas of healthy skin showed thickening of the corium. The increase in corium thickness was between 2% and 251%. The extent of the difference in corium thickness between sclerotic and healthy skin depended on the location-originally thin skin showed a greater degree of sclerosis. We also frequently found enhanced reflexes in the lower corium and hyperechoic, widened bands of connective tissue traversing the subcutaneous fatty tissue from the corium-subcutis border in the direction of the muscle fascia. 20 patients were examined several times in the course of one year. In nine patients we found ultrasonographic evidence of regression (decrease in thickness 26%) and in nine the ultrasound examination showed progression (increase in thickness 28%). 20-MHz B-mode ultrasound imaging is a suitable non-invasive method for monitoring the course and treatment of localized scleroderma. Its routine use is strongly recommended.

  15. [AWMF Guideline no. 013/066. Diagnosis and therapy of circumscribed scleroderma].

    Science.gov (United States)

    Kreuter, Alexander; Krieg, Thomas; Worm, Margitta; Wenzel, Jörg; Gambichler, Thilo; Kuhn, Annegret; Aberer, Elisabeth; Scharffetter-Kochanek, Karin; Hunzelmann, Nicolas

    2009-09-01

    Localized scleroderma is a rare autoimmune disease with primary affection of the skin, and occasional involvement of the fat tissue, muscle, fascia, and bone. Depending on the clinical subtype, the spectrum of skin lesions ranges from singular plaque lesions to severe generalized or linear subtypes which may lead to movement restrictions and permanent disability. This German S1-guideline proposes a classification of localized scleroderma that, considering the extent and depth of fibrosis, distinguishes limited, generalized, linear, and deep forms of localized scleroderma, together with its associated subtypes. The guideline includes a description of the pathogenesis, of differential diagnoses, and particular aspects of juvenile localized scleroderma, as well as recommendations for histopathologic, serologic, and biometric diagnostic procedures. Based on studies of topical and systemic treatments as well as phototherapy for localized scleroderma published in international literature, a treatment algorithm was developed which takes account of the different subtypes and the extent of disease.

  16. Scleroderma with Nodular Scleroderma

    Directory of Open Access Journals (Sweden)

    Chutika Srisuttiyakorn

    2016-11-01

    Full Text Available Background: Nodular scleroderma is a rare variant of scleroderma which can occur in connection with systemic sclerosis or morphea. A biopsy from the lesion can demonstrate the scleroderma pattern, i.e., keloid pattern or mixed type. Treatment is challenging, and several treatments modalities have been reported with unsatisfactory results. Main Observations: We present a case of systemic sclerosis in a 50-year-old female who developed nodular scleroderma in the absence of deterioration of the scleroderma condition. Although no additional treatment was given, the lesions remained stable without progression. Conclusions: Although this condition is rare, it has been reported sporadically, and clinicians should be able to recognize this variant in cases of scleroderma presenting with firm nodules or plaques.

  17. Localized scleroderma: clinical and epidemiological features with emphasis on adulthood- versus childhood-onset disease differences.

    Science.gov (United States)

    Lis-Święty, A; Skrzypek-Salamon, A; Ranosz-Janicka, I; Brzezińska-Wcisło, L

    2017-03-08

    Localized scleroderma is a rare inflammatory skin disorder that affects the skin and sometimes underlying subcutaneous tissue, muscles or bones. The disease has two modes of onset: juvenile- (JLS) and adult-onset (aLoS). Clinical features have impact on diagnostic and treatment recommendations, but no consensus on the disease management depending on the age at diagnosis was given. To identify these features which differentiate aLoS from JLS. A review of the literature was carried out using the MEDLINE to identify studies assessing demographics, subtype distribution, extracutaneous manifestations, comorbidities, delay at diagnosis and outcome in JLS and aLoS. Non-English articles, reviews, case reports, treatment trials that did not investigate long-term outcomes and studies with the fused data for children and adults were excluded. The analysed papers were published between June 1986 and December 2016. Fifty-five studies describing JLS or/and aLoS were included for analysis. Female: male ratio in aLoS was higher than in JLS. Adults presented with plaque and generalized subtype more often than paediatric patients. Linear subtype, musculoskeletal, neurologic and ophthalmologic involvement were more frequent, among children. aLoS was likely to be associated with increased prevalence of lichen sclerosus. There was significant delay in diagnosis in both groups of patients. Recurrences of disease were independent of its onset mode. aLoS patients had more poor quality of life scores than did JLS patients. As only a few studies or no studies assessing specified disease subtypes were identified, this limited the ability to compare the adult and paediatric patients with these subtypes. Despite more favourable course of aLoS, a thorough understanding of the broad spectrum of domains related to physical, mental, emotional and social functioning of patients seems to be important to the proper management of disease. LoS heterogeneity makes it important to develop universal

  18. Phototherapy in Scleroderma

    OpenAIRE

    Hassani, John; Feldman, Steven R.

    2016-01-01

    Systemic and localized scleroderma are difficult to manage diseases with no accepted gold standard of therapy to date. Phototherapeutic modalities for scleroderma show promise. A PubMed search of information on phototherapy for scleroderma was conducted. The information was classified into effects on pathogenesis and clinical outcomes. Studies on photopheresis were excluded. There were no randomized, double-blind, placebo-controlled studies, and only three controlled studies. The vast majorit...

  19. Imaging features in calcinosis circumscripta, a rare type of subcutaneous calcification in localized scleroderma

    Directory of Open Access Journals (Sweden)

    Pratiksha Yadav

    2013-01-01

    Full Text Available Calcinosis cutis circumscripta is a rare condition in which abnormal deposition of calcium seen in the dermis and subcutaneous tissue, it is associated with localized scleroderma. A 30-year-old female presented with an area of extensive calcification involving the right gluteal region, lateral aspect of right thigh and a small area on left thigh detected on radiograph with atrophy of subcutaneous tissue. Magnetic resonance imaging and computed tomography were done for further evaluation and the findings were of calcification and atrophy involving the skin and subcutaneous tissue.

  20. Esclerodermia localizada: Diagnósticos diferenciales Localized scleroderma: Differential diagnosis

    Directory of Open Access Journals (Sweden)

    MB Leroux

    2011-09-01

    Full Text Available La esclerodermia localizada constituye un desorden autoinmune órgano específico, que compromete sobre todo la piel. Se caracteriza por inflamación seguida de esclerosis e incluye distintas formas clínicas. La etiología de la esclerodermia localizada no ha sido establecida. El diagnóstico diferencial incluye cuadros esclerodermiformes, desencadenados por factores intrínsecos y extrínsecos que están siendo estudiados. Entre ellos se incluyen: exposición a radiaciones o tóxicos, consumo de medicamentos, infecciones y enfermedades de origen endocrino-metabólico, genético e inflamatorio. En primer lugar, se debe descartar la esclerodermia sistémica. En segundo lugar, se clasifican las entidades con predominio de esclerosis o atrofia. Por último, se incluyen algunas enfermedades en un cuadro comparativo.Localized scleroderma is an autoimmune organ specific disorder with an important skin compromise. It is an inflammatory process with several distinct clinical characteristics. The etiology of localized scleroderma has not been established yet. Differential diagnosis includes sclerodermiform onset unchained by intrinsic and extrinsic factors that are presently studied. Among them must be taking into account: exposure to radiation or toxic agents, therapeutic drugs, infections and diseases of endocrine, metabolic, genetic or inflammatory etiology. Firstly, it must be point out that systemic scleroderma must be ruled out. Secondly, disorders predominantly with sclerosis and atrophy must be classified and lastly, some other diseases are included in a comparative table.

  1. Systemic sclerosis and localized scleroderma--current concepts and novel targets for therapy.

    Science.gov (United States)

    Distler, Oliver; Cozzio, Antonio

    2016-01-01

    Systemic sclerosis (SSc) is a chronic autoimmune disease with a high morbidity and mortality. Skin and organ fibrosis are key manifestations of SSc, for which no generally accepted therapy is available. Thus, there is a high unmet need for novel anti-fibrotic therapeutic strategies in SSc. At the same time, important progress has been made in the identification and characterization of potential molecular targets in fibrotic diseases over the recent years. In this review, we have selected four targeted therapies, which are tested in clinical trials in SSc, for in depths discussion of their preclinical characterization. Soluble guanylate cyclase (sGC) stimulators such as riociguat might target both vascular remodeling and tissue fibrosis. Blockade of interleukin-6 might be particularly promising for early inflammatory stages of SSc. Inhibition of serotonin receptor 2b signaling links platelet activation to tissue fibrosis. Targeting simultaneously multiple key molecules with the multityrosine kinase-inhibitor nintedanib might be a promising approach in complex fibrotic diseases such as SSc, in which many partially independent pathways are activated. Herein, we also give a state of the art overview of the current classification, clinical presentation, diagnostic approach, and treatment options of localized scleroderma. Finally, we discuss whether the novel targeted therapies currently tested in SSc could be used for localized scleroderma.

  2. Mild Cognitive Impairment as a single sign of brain hemiatrophy in patient with Localized Scleroderma and Parry-Romberg Syndrome.

    Science.gov (United States)

    Klimiec, Elzbieta; Klimkowicz-Mrowiec, Aleksandra

    2016-01-01

    Neurologic involvement is well recognized in Systemic Scleroderma and increasingly reported in Localized Scleroderma. MRI brain abnormalities are often associated with symptoms such as seizures or headaches. In some cases they may be clinically silent. We describe a 23 years old female with head, trunk and limbs scleroderma who developed Parry-Romberg Syndrome. Brain MRI showed ipsilateral temporal lobe atrophy without any prominent neurologic symptoms. Neuropsychological examination revealed Mild Cognitive Impairment. During the 7 years of follow up we have noticed progression of face atrophy but no progression of brain atrophy. Cognitive functions have been stable. This case highlight that major MRI brain abnormalities in LS may occur with only subtle clinical manifestation such as Mild Cognitive Impairment.

  3. Localized and systemic scleroderma show different histological responses to methotrexate therapy.

    Science.gov (United States)

    Seyger, M M; van den Hoogen, F H; van Vlijmen-Willems, I M; van de Kerkhof, P C; de Jong, E M

    2001-04-01

    Although morphoea (localized scleroderma) and systemic sclerosis are distinct disease entities, the skin lesions show identical histological characteristics and both diseases respond favourably to low-dose treatment with methotrexate (MTX). The aim of this study was to find out whether MTX treatment induces different histological response patterns in these two diseases. In seven patients with morphoea and eight with systemic sclerosis, skin biopsies were taken before and after 24 weeks of treatment with low-dose MTX. In the centre and active margin of morphoea lesions, a significant reduction in tenascin staining was seen after 24 weeks of treatment, in contrast to systemic sclerosis. The numbers of mast cells decreased in the active margin of morphoea lesions, whereas in systemic sclerosis no significant change was seen after MTX therapy. Epidermal proliferation and staining of heparan sulphate proteoglycans showed no changes. Although skin lesions from both diseases respond clinically to treatment with MTX, systemic sclerosis shows no change in the immunohistochemical parameters investigated, whereas morphoea does. This difference in dynamic pattern suggests that the apparently similar lesions in localized and systemic sclerosis are not identical.

  4. Facial asymmetry: a case report of localized linear scleroderma patient with muscular strain and spasm.

    Science.gov (United States)

    Kim, Jae-Hyung; Lee, Suck-Chul; Kim, Chul-Hoon; Kim, Bok-Joo

    2015-12-01

    Facial asymmetry is found in patients with or without cosmetic facial alterations. Some patients have facial asymmetry that manifests underlying skeletal problems, while others have only limited soft-tissue facial asymmetry. Orthognathic surgery brings about a dermatic change, as soft tissue covers underlying bones. Limited soft-tissue asymmetry, meanwhile, is difficult to correct. The treatment modalities for the creation or restoration of an esthetically pleasing appearance were autogenous fat grafts, cartilage graft, and silicon injections. A young female patient had right-side facial asymmetry. The clinical assessment involved visual inspection of the face and palpation to differentiate soft tissue and bone. Although the extra-oral examination found facial asymmetry with skin atrophy, the radiographic findings revealed no mandibular atrophy or deviation. She was diagnosed as localized scleroderma with muscle spasm. In conclusion, facial asymmetry patients with skeletal asymmetry can be esthetically satisfied by orthognathic surgery; however, facial atrophy patients with skin or subdermal tissue contraction need treatment by cosmetic dermatological surgery and orthodontic correction.

  5. Pathogenesis and therapeutic approaches for improved topical treatment in localized scleroderma and systemic sclerosis.

    Science.gov (United States)

    Badea, I; Taylor, M; Rosenberg, A; Foldvari, M

    2009-03-01

    SSc is a chronic progressive disorder of unknown aetiology characterized by excess synthesis and deposition of collagen and other extracellular matrix components in a variety of tissues and organs. Localized scleroderma (LS) differs from SSc in that with LS only skin and occasionally subcutaneous tissues are involved. Although rarely life threatening, LS can be disfiguring and disabling and, consequently, can adversely affect quality of life. There is no known effective treatment for LS, and various options, including, as examples, corticosteroids and other immunomodulatory agents, ultraviolet radiation and vitamin D analogues, are of unproven efficacy. Clinical trials evaluating combination therapy such as corticosteroids with MTX or UVA1 exposure with psoralens have not been established as consistently effective. New immunomodulators such as tacrolimus and thalidomide are also being evaluated. A better understanding of the molecular and cellular mechanisms of LS has led to evaluation of new treatments that modulate profibrotic cytokines such as TGF-beta and IL-4, regulate assembly and deposition of extracellular matrix components, and restore Th1/Th2 immune balance by administering IL-12 or IFN-gamma. IFN-gamma acts by directly inhibiting collagen synthesis and by restoring immune balance. In this review, we evaluate current and future treatment options for LS and cutaneous involvement in SSc. Recent advances in therapy focus mainly on anti-fibrotic agents. Delivery of these drugs into the skin as the target tissue might be a key factor in developing more effective and safer therapy.

  6. Clinical and laboratory features of systemic sclerosis complicated with localized scleroderma.

    Science.gov (United States)

    Toki, Sayaka; Motegi, Sei-ichiro; Yamada, Kazuya; Uchiyama, Akihiko; Kanai, Sahori; Yamanaka, Masayoshi; Ishikawa, Osamu

    2015-03-01

    Localized scleroderma (LSc) primarily affects skin, whereas systemic sclerosis (SSc) affects skin and various internal organs. LSc and SSc are considered to be basically different diseases, and there is no transition between them. However, LSc and SSc have several common characteristics, including endothelial cell dysfunction, immune activation, and excess fibrosis of the skin, and there exist several SSc cases complicated with LSc during the course of SSc. Clinical and laboratory characteristics of SSc patients with LSc remain unclear. We investigated the clinical and laboratory features of 8 SSc patients with LSc among 220 SSc patients (3.6%). The types of LSc included plaque (5/8), guttate (2/8), and linear type (1/8). All cases were diagnosed as having SSc within 5 years before or after the appearance of LSc. In three cases of SSc with LSc (37.5%), LSc skin lesions preceded clinical symptoms of SSc. Young age, negative antinuclear antibody, and positive anti-RNA polymerase III antibody were significantly prevalent in SSc patients with LSc. The positivity of anticentromere antibody tended to be prevalent in SSc patients without LSc. No significant difference in the frequency of complications, such as interstitial lung disease, reflux esophagitis, and pulmonary artery hypertension, was observed. The awareness of these characteristic of SSc with LSc are essential to establish an early diagnosis and treatment.

  7. Scleroderma mimics.

    Science.gov (United States)

    Nashel, Jennifer; Steen, Virginia

    2012-02-01

    Scleroderma is a rare systemic autoimmune disease with multiple organ manifestations, including skin fibrosis. The groups of disorders classified as scleroderma mimics share the common thread of skin thickening but are otherwise quite incongruous in terms of underlying disease process and other organ involvement. This article reviews the clinical presentation, etiology, and treatment options available for scleroderma mimics, including morphea, scleredema, diabetic cheiroarthropathy, scleromyxedema, nephrogenic systemic fibrosis, and eosinophilic fasciitis. Through greater understanding of these diseases and the associated extradermal implications, we hope to facilitate recognition of scleroderma and its mimics.

  8. Risk Factors for Scleroderma

    Science.gov (United States)

    ... You are here: Home For Patients Risk Factors Risk Factors for Scleroderma The cause of scleroderma is ... what biological factors contribute to scleroderma pathogenesis. Genetic Risk Scleroderma does not tend to run in families ...

  9. Scleroderma Foundation

    Science.gov (United States)

    ... mail you a copy! News Researchers Have Identified Method to Slow Fibrosis in Scleroderma Video: Posture into ... org | Contact Us | Annual Report | 2015 Audited Financials | IRS Form 990 Disclaimer | Privacy | Share

  10. SCLERODERMA: A CASE REPORT*

    Directory of Open Access Journals (Sweden)

    Gülay Altan

    2015-04-01

    Full Text Available Scleroderma is an autoimmune connective tissue disorder which is characterized by fibrosis of visceral organs, skin and blood vessels. This condition can be localized or systemic. Its estimated prevalence is 250 cases in a million and it is more common in women than in men. Resorption of the mandibular angle and coronoid process can be observed in patients with scleroderma. Pressure of fibrous mucocutaneous tissues is thought to be the cause of the resorption. Decreased number of wrinkles due to sclerosis and distinct facial features because of the atrophy of ala nasi are among common clinical characteristics of this condition. The aim of this case report is to present a 40-year-old female patient with scleroderma who presented with signs of resorption at the angle of mandible, coronoid process, as well as widening of the periodontal space.

  11. What Is Scleroderma?

    Science.gov (United States)

    ... 11:39 Size: 10.9 MB November 2014 What Is Scleroderma? Fast Facts: An Easy-to-Read ... Scleroderma and Other Related Conditions For Your Information What Causes Scleroderma? The cause is unknown. You can’ ...

  12. Diagnosis and treatment of scleroderma.

    Science.gov (United States)

    Adnan, Zainal Arifin

    2008-04-01

    Scleroderma is a rare disease. Approximately 80% of patients are females, and one-half present before the age of 40. Some studies suggest a higher incidence and severity of disease in black females than in whites. Scleroderma affect approximately 20 new patients per million per year and has an estimated prevalence of approximately 250 patients per million in the United States, the synonyms from this disease including Progressive systemic sclerosis (PSS), or diffuse scleroderma. Scleroderma is a multisystem disorder characterized by skin thickening and vascular abnormalities. Causes of scleroderma remain mysterious. Immunologic abnormalities are suggested by the presence of characteristic autoantibodies such as ANA,anticentromere, and anti-Scl-70 antibodies. In addition to skin, the most commonly affected organs are lung and kidney. Three major diseases subsets are recognized based on the extent of skin disease. Limited disease is defined as skin fibrosis in distal extremities and some areas of face and neck. Limited diseases are also known as CREST syndrome. Diffuse disease includes patients with skin abnormalities extending to the proximal extremities (i.e., above the elbow or knee) and trunk. Localized disease manifests as patches (morphea) or bandlike (linear scleroderma) areas of skin thickening.

  13. [Systemic scleroderma].

    Science.gov (United States)

    Hunzelmann, N

    2013-04-01

    Systemic scleroderma is a chronic autoimmune disease affecting the skin, internal organs and the musculoskeletal system. The presence of Raynaud phenomenon, anti-nuclear antibodies and pathologic capillaroscopy are early signs of the disease. Limited cutaneous SSc, diffuse cutaneous SSc and SSc-overlap syndromes are the main clinical subtypes. Multidisciplinary care is mandatory. Follow-up examinations should be performed at least annually in order to recognize in a timely fashion treatable organ involvement such as pulmonary arterial hypertension. Besides symptomatic treatment of organ involvement, immunosuppressive therapy is indicated for a progressive inflammatory course.

  14. Autoimmune connective tissue disease: scleroderma.

    Science.gov (United States)

    Wilson, Helen; Vincent, Rachel

    Scleroderma is an umbrella term for a spectrum of rare and complex autoimmune connective tissue diseases, the cause and pathogenesis of which is only partially defined. Scleroderma can be divided into two main subgroups--systemic and localized--but the hallmark of both is skin fibrosis. As yet no drug has been found to be effective in reversing the disease process, however early intervention has been shown to give maximum benefit. Due to the chronic nature of the condition a multidisciplinary approach is essential and the nurse's input from an early stage is vital in supporting the patient to manage both their medical treatment and their activities of daily living.

  15. Scleroderma and Sexuality

    Science.gov (United States)

    Scleroderma and Sexuality INTRODUCTION If you or your partner have been diagnosed with scleroderma, you may be wondering how this will ... will continue to find satisfaction and enjoyment through sexuality. If you are single, you may wonder how ...

  16. Dental Care in Scleroderma

    Science.gov (United States)

    Dental Care in Scleroderma People living with scleroderma face unique challenges while trying to maintain their oral ... They are more likely to be affected by dental conditions such as small mouth, dry mouth, jaw ...

  17. Localized scleroderma: assessment of the therapeutic response to phototherapy Esclerodermia cutânea: avaliação da resposta terapêutica à fototerapia

    Directory of Open Access Journals (Sweden)

    Roberta Buense

    2012-02-01

    Full Text Available BACKGROUND: Scleroderma is a chronic autoimmune disease characterized by progressive connective tissue sclerosis and microcirculatory changes. Localized scleroderma is considered a limited disease. However, in some cases atrophic and deforming lesions may be observed that hinder the normal development. Literature reports indicate phototherapy as a therapeutic modality with favorable response in cutaneous forms of scleroderma. OBJECTIVES: This study had the purpose of assessing the phototherapy treatment for localized scleroderma. METHODS: Patients with localized scleroderma were selected for phototherapy treatment. They were classified according to the type of localized scleroderma and evolutive stage of the lesions. Clinical examination and skin ultrasound were used to demonstrate the results thus obtained. RESULTS: Some clinical improvement was observed after an average of 10 phototherapeutic sessions. All skin lesions were softer at clinical palpation with scores reduction upon pre and post treatment comparison. The ultrasound showed that most of the assessed lesions presented a decrease in dermal thickness, and only five maintained their previous measure. Treatment response was similar regardless of the type of phototherapeutic treatment employed. CONCLUSIONS: The proposed treatment was effective for all lesions, regardless of the phototherapeutic modality employed. The improvement was observed in all treated skin lesions and confirmed by clinical evaluation and skin ultrasound.FUNDAMENTOS: A esclerodermia é uma doença autoimune caracterizada pela esclerose progressiva do tecido conjuntivo e alterações da microcirculação. A forma cutânea é considerada uma doença autolimitada. No entanto, em alguns casos, ocorrem lesões atróficas, deformantes, que dificultam o desenvolvimento normal. Relatos da literatura apontam a fototerapia como uma modalidade terapêutica com resposta favorável nas formas cutâneas da esclerodermia. OBJETIVOS

  18. Comparison of the localization of tetrodotoxin between wild pufferfish Takifugu rubripes juveniles and hatchery-reared juveniles with tetrodotoxin administration.

    Science.gov (United States)

    Okita, Kogen; Takatani, Tomohiro; Nakayasu, Junichi; Yamazaki, Hideki; Sakiyama, Kazutaka; Ikeda, Koichi; Arakawa, Osamu; Sakakura, Yoshitaka

    2013-09-01

    To reveal the accumulation profile of tetrodotoxin (TTX) in pufferfish Takifugu rubripes juveniles, we compared the localization of TTX in various tissues among wild juveniles and hatchery-reared juveniles with or without TTX administration using immunohistochemical technique with anti-TTX monoclonal antibody. Immuno-positive reaction was observed in hepatic tissue, basal cell of skin and olfactory, olfactory epithelium, optic nerve and brain (optic tectum, cerebellum, medulla oblongata) of wild juveniles (body length: BL, 4.7-9.4 cm). TTX was detected in the same tissues as wild juveniles and epithelial cell layer of intestine of hatchery-reared juveniles (BL, 5.0-5.3 cm) to which TTX was orally administrated. No positive reaction was observed from the tissues of hatchery-reared juveniles without TTX administration. These results suggest that orally administrated TTX to the non-toxic cultured juveniles is accumulated in the same manner of wild juveniles. In addition, our study revealed that pufferfish accumulates TTX in the central nervous system.

  19. Systemic and localized scleroderma.

    Science.gov (United States)

    Chung, Lorinda; Lin, Jan; Furst, Daniel E; Fiorentino, David

    2006-01-01

    Sclerosing conditions of the skin are manifested by a full spectrum of presentations that includes skin-limited forms as well as those which can involve internal organs and result in death. At this point, we are just beginning to understand the mechanisms of tissue fibrosis, and it is likely that the fibrotic processes are a heterogeneous group of disorders in which perturbation of multiple molecular pathways, including vascular and immunologically mediated pathways, can lead to fibrosis. We now have some moderately effective therapies for vascular aspects of systemic sclerosis (eg, bosentan for pulmonary arterial hypertension, calcium-channel blockers for Raynaud's, or angiotensin-converting enzyme inhibitors for renal crisis). We also are beginning to find treatments interrupting the immunologic pathways that manifest as systemic sclerosis (eg, methotrexate for the skin or cyclophosphamide for the lungs). The basic process of fibrosis, however, awaits proven, effective therapy.

  20. Unilateral Enophthalmos in Linear Scleroderma: A Case Report

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    Cho, So hyun; Hwang, Hee Young; Choi, Hye Young; Kim, Hyung Sik [Gachon University, Gil Hospital, Incheon (Korea, Republic of)

    2010-04-15

    Linear scleroderma is an uncommon subtype of localized scleroderma, which is characterized by a linear streak that crosses dermatomes and is associated with the tracking of fibrosis from the skin into deeper tissues, including muscle and fascia. A severe form of this condition sometimes causes growth atrophy of bone and supporting tissue in the affected area. Enophthalmos as a manifestation of linear scleroderma is very rare and occurs due to the replacement of orbital fat and muscle with collagen, which finally leads to atrophy of the affected orbit. This report introduces imaging findings of two cases of enophthalmos caused by linear scleroderma

  1. Paraneoplastic Scleroderma: Are There Any Clues?

    Science.gov (United States)

    Jedlickova, Hana; Durčanská, Veronika; Vašků, Vladimír

    2016-04-01

    localized scleroderma was seen in 3 patients and generalized localized scleroderma in one case. All patients had a histological picture consistent with scleroderma, negative ANA and ENA antibodies (Table 1, Figure 1). A 66-year-old woman presented with a 10 months history of sclerodermatous plaques on her neck, trunk, and upper and lower extremities. The skin on her breasts and cheeks was diffusely indurated. Examination showed thrombocytopenia, elevated transaminases, Cancer antigen 19-9 (Ca 19-9), thyroid stimulating hormone (TSH), and anti-thyroid peroxidase antibodies, dysmotility of the lower part of esophagus, hepatosplenomegaly, cholecystolithiasis, and benign polyps of colon. She was given prednisone 40 mg/day but did not return for follow up. After 6 months she was diagnosed with cholangiogenic carcinoma with metastatic disease and died shortly afterwards. A 74-year-old woman had localized scleroderma on the trunk for three years. She was treated with procaine penicillin for positive borrelia Immunoglobulin M (IgM) antibodies. Her condition worsened suddenly with confluent scleroderma plaques on her trunk, extremities, and genital region, and vasoneurosis on her lower extremities; she was started on prednisone 35 mg/day. Examination revealed endometrial cancer. The patient underwent a hysterectomy, adnexectomy, and radiotherapy with curative effect. Scleroderma patches softened with residual hyperpigmentation, and prednisone was stopped two years later. A 80-year-old man had a month-long history of diffuse thickening and toughening of the skin on the forearms and lower legs and scleroderma patches on the thighs and shins. Examination revealed prostate adenocarcinoma, and therapy with antiandrogen bicalutamide and prednisone 15 mg/day was started. Two years after the diagnosis he continues with bicalutamide treatment, prednisone 5 mg q.a.d. and has residual toughening of the skin on his lower legs. A 62-year-old woman with seronegative rheumatoid arthritis presented

  2. Conformal radiotherapy for locally advanced juvenile nasopharyngeal angio-fibroma

    Directory of Open Access Journals (Sweden)

    Supriya Mallick

    2015-01-01

    Full Text Available Purpose: To assess the efficacy of radiation in the treatment of juvenile nasopharyngeal angiofibroma (JNA. Materials and Methods: Data were retrieved for JNA treated with radiotherapy from 1987-2012. The demographics, treatment and outcome data were recorded in predesigned proforma. Results: Data of 32 patients were retrieved. Median age was 17 years (range: 12-33 years. All patients received radiation because of refractory, residual or unresectable locally advanced disease. All patients were planned with a three-dimensional conformal technique (3DCRT. The median radiation dose was 30 Gray (range: 30-45 Gray. Median follow-up was 129 months (range: 1-276 months. At the last follow-up, 13 patients were found to have a radiological complete response. Two patients progressed 38 and 43 months after completion of treatment and opted for alternative treatment. One patient developed squamous cell carcinoma of the nasal ale 15 years after radiation. Conclusion: Conformal radiotherapy shows promise as an alternative treatment approach for locally advanced JNA and confers long-term disease control with minimal toxicity.

  3. Diagnosis and treatment of mandibular osteomyelitis in localized scleroderma of the craniofacial region%颅面部局限性硬皮病伴发下颌骨骨髓炎的诊断与治疗

    Institute of Scientific and Technical Information of China (English)

    周超; 翦新春; 李宁; 毕青

    2015-01-01

    A case of localized scleroderma associated with mandibular osteomyelitis is reported.The formation mechanism of the case is a-nalysised according to clinical characteristics and literature data.%该文对1例局限性硬皮病合并下颌骨颌骨骨髓炎患者进行诊断与治疗,并结合病历资料与文献讨论其发病机制。

  4. Pregnancy issues in scleroderma.

    Science.gov (United States)

    Lidar, Merav; Langevitz, Pnina

    2012-05-01

    Systemic sclerosis is a systemic, inflammatory, autoimmune disease affecting the skin and viscera, manifesting pathologically with microvascular lesions, perivascular infiltration by mononuclear cells and increased deposition of extracellular collagen. The rarity of the disease as well as its propensity to appear in the early 1940s, explain the low frequency of concurrent scleroderma and pregnancy. However, the marked female excess, as well as the trend for increasing maternal age due to social change and assisted reproductive technologies, renders heightened significance to issues of fertility, pregnancy course and pregnancy outcomes. In the past, scleroderma patients were thought to be at high risk for poor fetal and maternal outcome, but more current retrospective studies show that despite an increased frequency of prematurity and small for gestational age infants, overall maternal and neonatal survival is good. Hence, at present, with close monitoring and appropriate therapy most scleroderma patients can sustain a successful pregnancy. Therapy with hydroxychloroquine and low dose steroids as well as judicious use of intravenous immunoglobulins is permitted. Renal crisis remains the most dreaded complication of a scleroderma pregnancy and necessitates prompt institution of ACE inhibitor therapy despite its potential teratogenicity. In order minimize the risk for renal crisis, pregnancies should be avoided in rapidly progressive diffuse disease as such patients are at a greater risk for developing serious cardiopulmonary and renal problems early in the disease. This review shall focus on the bi-directional effects of scleroderma on fertility and pregnancy as well as on the management of pregnancy and delivery in the scleroderma patient.

  5. A rare association of localized scleroderma type morphea, vitiligo, autoimmune hypothyroidism, pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis. Case report

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    Bonilla-Abadía Fabio

    2012-12-01

    Full Text Available Abstract Background The localized scleroderma (LS known as morphea, presents a variety of clinical manifestations that can include systemic involvement. Current classification schemes divide morphea into categories based solely on cutaneous morphology, without reference to systemic disease or autoimmune phenomena. This classification is likely incomplete. Autoimmune phenomena such as vitiligo and Hashimoto thyroiditis associated with LS have been reported in some cases suggesting an autoimmune basis. To our knowledge this is the first case of a morphea forming part of a multiple autoimmune syndrome (MAS and presenting simultaneously with autoimmune thrombocytopenic purpura and central nervous system vasculitis. Case presentation We report an uncommon case of a white 53 year old female patient with LS as part of a multiple autoimmune syndrome associated with pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis presenting a favorable response with thrombopoietin receptor agonists, pulses of methylprednisolone and cyclophosphamide. Conclusion Is likely that LS have an autoimmune origin and in this case becomes part of MAS, which consist on the presence of three or more well-defined autoimmune diseases in a single patient.

  6. Fractional carbon dioxide laser versus low-dose UVA-1 phototherapy for treatment of localized scleroderma: a clinical and immunohistochemical randomized controlled study.

    Science.gov (United States)

    Shalaby, S M; Bosseila, M; Fawzy, M M; Abdel Halim, D M; Sayed, S S; Allam, R S H M

    2016-11-01

    Morphea is a rare fibrosing skin disorder that occurs as a result of abnormal homogenized collagen synthesis. Fractional ablative laser resurfacing has been used effectively in scar treatment via abnormal collagen degradation and induction of healthy collagen synthesis. Therefore, fractional ablative laser can provide an effective modality in treatment of morphea. The study aimed at evaluating the efficacy of fractional carbon dioxide laser as a new modality for the treatment of localized scleroderma and to compare its results with the well-established method of UVA-1 phototherapy. Seventeen patients with plaque and linear morphea were included in this parallel intra-individual comparative randomized controlled clinical trial. Each with two comparable morphea lesions that were randomly assigned to either 30 sessions of low-dose (30 J/cm(2)) UVA-1 phototherapy (340-400 nm) or 3 sessions of fractional CO2 laser (10,600 nm-power 25 W). The response to therapy was then evaluated clinically and histopathologically via validated scoring systems. Immunohistochemical analysis of TGF-ß1 and MMP1 was done. Patient satisfaction was also assessed. Wilcoxon signed rank test for paired (matched) samples and Spearman rank correlation equation were used as indicated. Comparing the two groups, there was an obvious improvement with fractional CO2 laser that was superior to that of low-dose UVA-1 phototherapy. Statistically, there was a significant difference in the clinical scores (p = 0.001), collagen homogenization scores (p = 0.012), and patient satisfaction scores (p = 0.001). In conclusion, fractional carbon dioxide laser is a promising treatment modality for cases of localized morphea, with proved efficacy of this treatment on clinical and histopathological levels.

  7. Esclerodermia localizada na criança: aspectos clínicos, diagnósticos e terapêuticos Localized scleroderma in children: clinical, diagnostic and therapeutic aspects

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    Pedro C. Q. Zancanaro

    2009-04-01

    Full Text Available A esclerodermia localizada, ou morféia, acomete crianças em idade escolar e, em geral, é autolimitada, apesar de localmente desfiguradora. A literatura descreve inúmeros fatores etiopatogênicos, bem como modalidades de investigação e tratamento. Este artigo reúne os estudos mais recentes e discute sua aplicação clínica.Localized scleroderma or morphea affects school-aged children, is usually self-limited and a disfiguring condition. Several etiopathogenic factors, investigations and treatment options are described. This article reviews the recent literature and discusses its clinical applications.

  8. Malignancy in scleroderma patients from south west England: a population-based cohort study.

    LENUS (Irish Health Repository)

    Siau, Keith

    2010-01-08

    The pathophysiological relationship between scleroderma and malignancy remains poorly understood. Although some previous studies have demonstrated an increased malignancy risk in patients with scleroderma, others have been inconclusive. We aimed to determine if patients with scleroderma had an increased risk of malignancy compared to an age- and sex-matched local South West England population, and if there were any important differences between scleroderma patients with and without malignancy. Methods of this study are as follows. Notes were obtained on all local scleroderma patients (n = 68) locally, and those diagnosed with malignancy verified by contacting each patient\\'s general practitioner. Expected malignancy figures were obtained from age- and sex-stratified regional prevalence data provided by the South West Cancer Intelligence Service registry. Among the patients, 22.1% with scleroderma were identified with concurrent malignancy. Affected sites were of the breast (n = 5), haematological system (n = 5), skin (n = 4), and unknown primary (n = 1). Overall, malignancy risk was found to be increased in scleroderma (RR = 3.15, 95% CI 1.77-5.20, p = 0.01). In particular, this risk was the highest for haematological malignancies (RR = 18.5, 95% CI 6-43, p = 0.03), especially for non-Hodgkin\\'s lymphoma (RR = 25.8, 95% CI 5-75, p = 0.10). The majority of patients (86.7%) developed malignancy after the onset of scleroderma (mean = 6.9 years). Age of >70 and patients with limited scleroderma were significant risk factors for a patient with scleroderma to have a concurrent malignancy; however, no increased risk was found in patients with any particular pattern of organ involvement, cytotoxic usage or serology. To conclude, in this small patient cohort, we have found that scleroderma is associated with an increased risk of malignancy. This risk is statistically significant in patients with limited scleroderma. Patients who are elderly and those with limited disease

  9. Local Implementation of Drug Policy and Access to Treatment Services for Juveniles

    Science.gov (United States)

    Terry-McElrath, Yvonne M.; McBride, Duane C.

    2004-01-01

    Although there is a vigorous national debate regarding effective drug policy, such policies are implemented at the local level. Using a national sample of prosecutors, we examine reported typical processing for first-time juvenile marijuana, cocaine, or crack possession/sales offenders. The relationship between drug offense charge and adjudication…

  10. Scleroderma, Stress and CAM Utilization

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    Ka-Kit Hui

    2009-01-01

    Full Text Available Scleroderma is an autoimmune disease influenced by interplay among genetic and environmental factors, of which one is stress. Complementary and alternative medicine (CAM is frequently used to treat stress and those diseases in which stress has been implicated. Results are presented from a survey of patients with scleroderma. Respondents were a convenient sample of those attending a national conference in Las Vegas in 2002. Findings implicate stress in the onset, continuation and exacerbation of scleroderma. The implication is that CAM providers may be filling an important patient need in their provision of services that identify and treat stress and its related disorders.

  11. Scleroderma: a case report

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    Prachi Sankhe

    2015-06-01

    Full Text Available Scleroderma is systemic multi organ autoimmune disorder characterized by hardening of skin. Also known as systemic sclerosis. Estimated annual incidences of approximately 19 cases per million persons. The limited skin disease has a 10-year survival rate of 71%, whereas those with diffuse skin disease have a 10-year survival rate of just 21%. Risk is higher in women than men and peak in individuals aged 30-50 years. It has no definitive treatment. It may be limited or diffuse depending upon manifestations of symptoms or signs affecting internal organs especially lungs, heart, or kidney. We report a case of scleroderma with pulmonary hypertension and interstitial lung disease in our hospital who presented with tightening of skin, joint pain, dysphagia, and breathlessness. On examination skin appeared dark, shiny, and tight, with loss of hair, paraesthesia and digital ulceration. Patient also has history of Raynauds phenomenon. On investigation, Scl-70 and ANA (antinuclear antibodies by enzyme immunoassay came positive. HRCT thorax was suggestive of interstitial fibrosis and PFT revealed moderate restriction. On 2D echocardiography, mild pulmonary hypertension was present while barium swallow showed motility disorder involving oesophagus. On view of extensive systemic involvement like skin, respiratory system, gastrointestinal system and heart, we would like to present this rare disorder. [Int J Res Med Sci 2015; 3(3.000: 802-804

  12. Scleroderma en coup de sabre treated with polymethylmethacrylate - Case report*

    Science.gov (United States)

    Franco, Joanna Pimenta de Araujo; Serra, Márcio Soares; Lima, Ricardo Barbosa; D’Acri, Antônio Macedo; Martins, Carlos José

    2016-01-01

    The scleroderma en coup de sabre is a variant of localized scleroderma that occurs preferentially in children. The disease progresses with a proliferative and inflammatory phase and later atrophy and residual deformity, which are treated with surgical techniques such as injectable fillers, transplanted or autologous fat grafting and resection of the lesion. Among the most widely used fillers is hyaluronic acid. However, there are limitations that motivate the search for alternatives, such as polymethylmethacrylate, a permanent filler that is biocompatible, non-toxic, non-mutagenic and immunologically inert. In order to illustrate its application, a case of scleroderma en coup de sabre in a 17-year-old patient, who was treated with polymethylmethacrylate with excellent aesthetic results, is reported. PMID:27192521

  13. Scleroderma with crescentic glomerulonephritis: a case report

    OpenAIRE

    Ramaswami Arunachalam; Kandaswamy Thiraviam; Rajendran Tholappan; Jeyakrishnan Kizhake; Aung Hla; Iqbal Mohammaed; Jacob Chakko K; Zinna Haji; Kafeel Gazala

    2008-01-01

    Abstract Introduction Systemic sclerosis or scleroderma is an autoimmune rheumatic disease characterized by organ-based fibrosis. Renal involvement in scleroderma occurs mainly in the form of scleroderma renal crisis, affecting 5 to 10% of patients. It remains one of the most important and immediately life-threatening complications of scleroderma, but the prognosis improves considerably after treatment with angiotensin-converting enzyme inhibitors. Other renal pathologies can occur in sclerod...

  14. Antiphospholipid Antibodies and Systemic Scleroderma

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    Awa Oumar Touré

    2013-03-01

    Full Text Available Objective: Antiphospholipid antibodies (APLs could be associated with an increased risk of vascular pathologies in systemic scleroderma. The aim of our study was to search for APLs in patients affected by systemic scleroderma and to evaluate their involvement in the clinical manifestations of this disease. Materials and Methods: We conducted a cross-sectional descriptive study, from January 2009 until August 2010, with patients received at the Department of Dermatology (Dakar, Senegal. Blood samples were taken at the hematology laboratory and were analyzed for the presence of APLs. Results: Forty patients were recruited. Various types of either isolated or associated APLs were found in 23 patients, i.e. 57.5% of the study population. The most frequently encountered antibody was IgG anti-β2 GPI (37.5% of the patients, followed by anticardiolipins (17.5% and lupus anticoagulants (5%. No statistically significant association of positive antiphospholipid-related tests to any of the scleroderma complications could be demonstrated. Conclusion: A high proportion of patients showing association of systemic scleroderma and APLs suggests the presence of a morbid correlation between these 2 pathologies. It would be useful to follow a cohort of patients affected by systemic scleroderma in order to monitor vascular complications following confirmation of the presence of antiphospholipid syndrome.

  15. Local adaptation in adult feeding preference and juvenile performance in the generalist herbivore Idotea balthica.

    Science.gov (United States)

    Bell, Tina M; Sotka, Erik E

    2012-10-01

    Populations can respond to environmental heterogeneity by genetic adaptation to local conditions. Evidence for local adaptation in herbivores with relatively broad host breadth is scarce, either because generalists rarely locally adapt or because fewer studies have tested for local adaptation. The marine isopod Idotea balthica, a small (Fucus vesiculosus and consumed more of its water-soluble and lipophilic extracts relative to southern populations. In contrast, southern populations have a relatively higher preference for the green macroalga Ulva linza and sea grass Zostera marina. The rank of hosts in feeding assays exhibited by northern adults (Fucus = Ulva > Zostera) and southern adults (Ulva > Fucus > Zostera) closely mirrored ranking of juvenile growth rates, suggesting that preference and performance are strongly correlated across these macrophytes. Several of our assays included isopods that had parents reared under uniform laboratory conditions, indicating that geographic differences are genetically mediated and unlikely to reflect phenotypic plasticity or maternal effects. Local adaptation in host use traits may be common in broadly distributed, generalist herbivores in marine and terrestrial systems, and will manifest itself as local shifts in the preference ranking of hosts.

  16. Scleroderma with crescentic glomerulonephritis: a case report

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    Ramaswami Arunachalam

    2008-05-01

    Full Text Available Abstract Introduction Systemic sclerosis or scleroderma is an autoimmune rheumatic disease characterized by organ-based fibrosis. Renal involvement in scleroderma occurs mainly in the form of scleroderma renal crisis, affecting 5 to 10% of patients. It remains one of the most important and immediately life-threatening complications of scleroderma, but the prognosis improves considerably after treatment with angiotensin-converting enzyme inhibitors. Other renal pathologies can occur in scleroderma. These include scleroderma overlap syndromes with associated features of lupus nephritis, myeloperoxidase anti-neutrophil cytoplasmic antibodies (ANCA or proteinase 3 ANCA-associated glomerulonephritis, or crescentic glomerulonephritis. These alternative pathologies should be suspected in any individual patient with a differing clinical picture and the patient should be appropriately investigated. Crescentic glomerulonephritis occurs very rarely in scleroderma. This report describes a patient with scleroderma and crescentic glomerulonephritis. Case presentation A 52-year-old woman with a known history of scleroderma and hypertension on angiotensin-converting enzyme inhibitors was referred to the nephrologist because of a rapid decline in renal function. Kidney biopsy was performed which revealed immune complex type crescentic glomrulonephritis. Cytoplasmic-staining ANCA was negative. Despite immunosuppressive treatment the patient rapidly went into end-stage renal failure and is still on hemodialysis. Conclusion Scleroderma is a complex disease, and the best characterized renal involvement in scleroderma is scleroderma renal crisis. However, other renal pathologies can occur in scleroderma. These alternative pathologies should be suspected in any patient with a differing clinical picture and the patient should be appropriately investigated, as the clinical course and treatment are different from the more common scleroderma renal crisis.

  17. Scleroderma: nomenclature, etiology, pathogenesis, prognosis, and treatments: facts and controversies.

    Science.gov (United States)

    Fett, Nicole

    2013-01-01

    Scleroderma refers to a heterogeneous group of autoimmune fibrosing disorders. The nomenclature of scleroderma has changed dramatically in recent years, with morphea (localized scleroderma), limited cutaneous systemic sclerosis, diffuse cutaneous systemic sclerosis, and systemic sclerosis sine scleroderma encompassing the currently accepted disease subtypes. Major advances have been made in the molecular studies of morphea and systemic sclerosis; however, their etiologies and pathogenesis remain incompletely understood. Although morphea and systemic sclerosis demonstrate activation of similar inflammatory and fibrotic pathways, important differences in signaling pathways and gene signatures indicate they are likely biologically distinct processes. Morphea can cause significant morbidity but does not affect mortality, whereas systemic sclerosis has the highest disease-specific mortality of all autoimmune connective tissue diseases. Treatment recommendations for morphea and systemic sclerosis are based on limited data and largely expert opinions. Current collaborative efforts in morphea and systemic sclerosis research will hopefully lead to better understanding of the etiology and pathogenesis of these rare and varied diseases and improved treatment options.

  18. Tips for Living with Scleroderma

    Science.gov (United States)

    ... and stiffness, consult an occupational therapist to learn new ways to perform these tasks, allowing you to ... will provide greater protection against decay. Schedule regular dental check-ups and contact ... products, equipment or treatments. Scleroderma varies substantially from one patient ...

  19. Linear scleroderma after contusion and injection of mepivacaine hydrochloride.

    Science.gov (United States)

    Ueda, Takashi; Niiyama, Shiro; Amoh, Yasuyuki; Katsuoka, Kensei

    2010-05-15

    A 36-year-old woman initially was treated for a contusion by local injection of mepivacaine hydrochloride into the left dorsum of the foot. Approximately 3 months after the injury and injection, linear sclerotic plaques originating from the site of contusion and injection were recognized. These progressed in extent and severity over a period of 3 years, when she presented to our clinic. By biopsy, swelling of collagen fibers in the lower dermis was revealed and the condition was diagnosed as linear scleroderma. Our present case had multiple linear sclerotic plaques of the left lower extremity, the distribution of which was consistent with Blaschko lines. It was also revealed that the initial sclerotic plaque was at the site of the contusion and local mepivacaine hydrochloride injection. Our present case is interesting in that the findings suggest a correlation between linear scleroderma plaque occurrence and the contusion or injection of mepivacaine.

  20. A study of the localized humoral immune response to implicated microorganisms in juvenile periodontitis

    Energy Technology Data Exchange (ETDEWEB)

    Hall, E.R.

    1988-01-01

    A study was undertaken using an in vitro explant culture system to determine the presence of immunoglobulins (IgG, IgA, and IgM) in the supernatant fluids (SF) of disease gingival tissue explant cultures. Studies were also undertaken to determine if the de novo biosynthesis of {sup 14}C-immunoglobulins could be observed in the explant cultures of diseased tissues from juvenile periodontitis (JP) patients. Radiolabeled proteins were detected in the SF and immunodiffusion studies using goat antihuman gamma, alpha or mu chain serum revealed the presence of IgG and IgA but no IgM present in the SF of the JP gingival tissue explant cultures. Immunodiffusion studies using goat anti-human gamma chain serum with Staph protein A isolated IgG fractions of the SF, followed by autoradiography of the IgG precipitation lines demonstrated the biosynthesis of IgG by the JP gingival tissue explant cultures. The serological studies suggested that local immune response in JP was to a polymicrobic infection. The SF of JP showed significantly higher levels of antibody reactivity to B. intermedius, C. ochracea, E. nodatum and P. micros as compared to healthy tissues. The local antibody response to the microorganisms tested differed from that observed in the sera of the patients.

  1. [Digital ulcers in systemic scleroderma].

    Science.gov (United States)

    Belz, D; Hunzelmann, N; Moinzadeh, P

    2014-11-01

    Digital ulcers (DU's) are one of the main symptoms of systemic scleroderma and occur in approximately 60% of all scleroderma patients. Due to possible complications such as infections, gangrene or amputation, they require regular medical attention and a good wound treatment by doctors and nursing staff. A definition of DU's has not yet been established. In 2009 the European League Against Rheumatism (EULAR) published guidelines for the treatment of DU's. An improvement of the healing of active ulcers has been described with Iloprost. Bosentan significantly reduced the frequency of occurrence of new DU's. In some small studies PDE-5 inhibitors appear helpful. Further studies with other therapeutic approaches will follow in the next few years.

  2. Correlação clínica e ultra-sonográfica na esclerodermia localizada cutânea Clinical and ultrasonographic correlation in localized cutaneous scleroderma

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    Marcio Bouer

    2008-04-01

    Full Text Available OBJETIVO: Apresentar os aspectos ultra-sonográficos da esclerodermia localizada e relacioná-los com os aspectos clínicos. MATERIAIS E MÉTODOS: Foram analisadas 23 lesões de esclerodermia localizada em 21 pacientes. Foi utilizado equipamento Logiq 700 com transdutor linear de 6-14 MHz. Foram avaliados, pelo dermatologista, o estágio da doença (inflamatório ou atrófico, e pelo radiologista, a espessura e a ecogenicidade da derme nas regiões afetadas e sãs adjacentes. Foi feito acompanhamento de sete casos após tratamento. RESULTADOS: Todas as lesões apresentaram perda do padrão ultra-sonográfico normal da derme. Os casos de lesão clinicamente atrófica (52,2%; 12/23 corresponderam a redução da espessura e aumento da ecogenicidade da derme e os casos de lesão clinicamente inflamatória (47,8%; 11/23 corresponderam a aumento da espessura e redução da ecogenicidade da derme. Controles pós-tratamento mostraram alterações na espessura da derme. CONCLUSÃO: Os achados ultra-sonográficos nos permitem associar o aumento da espessura e a redução da ecogenicidade da derme com a fase inflamatória da doença, e a redução da espessura e o aumento da ecogenicidade da derme com a fase atrófica da doença. Notamos também que é possível quantificar a espessura da derme e usar essa informação no controle pós-tratamento associada à avaliação clínica.OBJECTIVE: To describe ultrasonographic findings of localized cutaneous scleroderma and correlating them with clinical findings. MATERIALS AND METHODS: Twenty-three lesions of localized cutaneous scleroderma in 21 patients were evaluated with a Logiq 700 equipment coupled with a 6-14 MHz linear transducer. The disease stage (athrophic or inflammatory was evaluated by a dermatologist, and the ultrasonographic findings (skin thickness and echogenicity for both the affected and adjacent healthy regions were evaluated by a radiologist. Seven of the cases underwent post

  3. [Scleroderma: an update on the pathogenesis and treatment].

    Science.gov (United States)

    Garza-Rodríguez, Verónica; Villarreal-Alarcón, Miguel Angel; Ocampo-Candiani, Jorge

    2013-01-01

    Scleroderma is a multiorganic disease characterized by inflammatory, vascular and sclerotic changes in skin and internal organs. It is considered as a tripartite disease, associated to autoimmune, fibroblast and endothelial defect, due to genetic, environmental and infectious factors. This disease can be classified in systemic and localized form. The Raynaud phenomenon occurs in 90% of the patients with the diagnosis. It explains the microcirculation involvement and the reduction in the number of capillaries. Malformation of nail bed capillaries is readily demonstrated by nail bed microscopy and has been shown to correlate both with disease severity and with degree of internal organ involvement. The MRSS-51 validates the skin involvement and has the main predictive value to determine the patient survival. MRSS-51 should not be considered as an activity disease parameter or used to validate the effectiveness of treatment. Nowadays, multiple treatment alternatives exist for scleroderma disease; however these treatments offer poor results for the cutaneous manifestations.

  4. Neurologic Involvement in Scleroderma en Coup de Sabre

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    Tiago Nardi Amaral

    2012-01-01

    Full Text Available Localized scleroderma is a rare disease, characterized by sclerotic lesions. A variety of presentations have been described, with different clinical characteristics and specific prognosis. In scleroderma en coup de sabre (LScs the atrophic lesion in frontoparietal area is the disease hallmark. Skin and subcutaneous are the mainly affected tissues, but case reports of muscle, cartilage, and bone involvement are frequent. These cases pose a difficult differential diagnosis with Parry-Romberg syndrome. Once considered an exclusive cutaneous disorder, the neurologic involvement present in LScs has been described in several case reports. Seizures are most frequently observed, but focal neurologic deficits, movement disorders, trigeminal neuralgia, and mimics of hemiplegic migraines have been reported. Computed tomography and magnetic resonance imaging have aided the characterization of central nervous system lesions, and cerebral angiograms have pointed to vasculitis as a part of disease pathogenesis. In this paper we describe the clinical and radiologic aspects of neurologic involvement in LScs.

  5. Monocyte differentiation in localized juvenile periodontitis is skewed toward the dendritic cell phenotype.

    Science.gov (United States)

    Barbour, Suzanne E; Ishihara, Yuichi; Fakher, Mohammed; Al-Darmaki, Salma; Caven, Timothy H; Shelburne, C P; Best, Al M; Schenkein, Harvey A; Tew, John G

    2002-06-01

    Several lines of evidence indicate that the monocytes of subjects with localized juvenile periodontitis (LJP) are functionally distinct from cells of age- and race-matched nonperiodontitis (NP) subjects. Among the abnormalities are the propensity to secrete large amounts of prostaglandin E(2) and the induction of immunoglobulin G2 (IgG2) antibodies. The experiments described here were performed to further characterize the LJP monocytes and to determine if these cells mature differently than NP monocytes. When adherent monocytes from LJP subjects were cultured in the presence of human serum, both macrophages and cells with the morphology of immature monocyte-derived dendritic cells (MDDC) were observed. Within 4 days the prevalence of the immature MDDC was approximately twofold higher in LJP cultures than in NP cultures. In addition to their dendritic morphology, these cells were CD11c(+) and CD14(-) or CD14(low) and stimulated potent autologous mixed leukocyte reactions, consistent with differentiation to the MDDC phenotype. Like LJP monocytes, cultures of MDDC generated with interleukin-4 and granulocyte-macrophage colony-stimulating factor selectively induced IgG2 in cultures of pokeweed mitogen-stimulated NP leukocytes. Together, these data suggest that the monocytes of LJP subjects have a propensity to differentiate into MDDC and that this differentiation may be related to the high levels of IgG2 that are observed in the sera of LJP subjects. As high levels of circulating IgG2 are correlated with less severe disease, the propensity of LJP monocytes to differentiate into MDDC may have important implications for both the host response against oral pathogens and the progression of LJP.

  6. Targeting miR-155 to Treat Experimental Scleroderma.

    Science.gov (United States)

    Yan, Qingran; Chen, Jie; Li, Wei; Bao, Chunde; Fu, Qiong

    2016-02-01

    Scleroderma is a refractory autoimmune skin fibrotic disorder. Alterations of microRNAs in lesional skin could be a new approach to treating the disease. Here, we found that expression of miR-155 was up regulated in lesional skin tissue from patients with either systemic or localized scleroderma, and correlated with fibrosis area. Then we demonstrated the potential of miR-155 as a therapeutic target in pre-clinical scleroderma models. MiR-155(-/-) mice were resistant to bleomycin induced skin fibrosis. Moreover, topical antagomiR-155 could effectively treat mice primed with subcutaneous bleomycin. In primary skin fibroblast, miR-155 silencing could inhibit collagen synthesis function, as well as signaling intensity of two pro-fibrotic pathways, Wnt/β-catenin and Akt, simultaneously. We further showed that miR-155 could regulate the two pathways via directly targeting casein kinase 1α (CK1α) and Src homology 2-containing inositol phosphatase-1 (SHIP-1), as previous reports. Mice with miR-155 knockout or topical antagomir-155 treatment showed inhibited Wnt/β-catenin and Akt signaling in skin upon bleomycin challenge. Together, our data suggest the potential of miR-155 silencing as a promising treatment for dermal fibrosis, especially in topical applications.

  7. Subcutaneous administration of polymerized type I collagen downregulates interleukin (IL)-17A, IL-22 and transforming growth factor-β1 expression, and increases Foxp3-expressing cells in localized scleroderma.

    Science.gov (United States)

    Furuzawa-Carballeda, J; Ortíz-Ávalos, M; Lima, G; Jurado-Santa Cruz, F; Llorente, L

    2012-08-01

    Localized scleroderma (LS) is a disfiguring inflammatory autoimmune disease of the skin and underlying tissue. As in systemic sclerosis, a key feature is the presence of T cells in inflammatory lesions. To evaluate the effect of polymerized type I collagen vs. methylprednisolone (MP) in LS, and to determine the influence of this polymerized collagen (PC) on CD4+ peripheral T cells expressing interleukin (IL)-4, IL-17A, interferon-γ and Forkhead box protein (Foxp)3, and on cells expressing transforming growth factor (TGF)-β1, IL-17A, IL-22 and Foxp3 in the skin. In total, 16 patients with LS were treated for 3 months with monthly subcutaneous intralesional injections of 0.1 mL MP (giving a total dose of 20 mg/mL each month) and 15 patients were treated, with weekly subcutaneous intralesional injections of PC, ranging from 0.2 mL (equivalent to 1.66 mg collagen) for a lesion of 50 mm in size, up to a maximum of 1.0 mL (8.3 mg collagen) for a lesion > 100 mm in size, and followed up for a further 6 months. Skin biopsies were obtained from lesions at baseline (before treatment) and 9 months later (6 months after treatment end). Tissue sections were evaluated by histology and immunohistochemistry (IL-17A, IL-22, TGF-β1 and Foxp3). CD4+ T-cell subsets were determined in peripheral blood by flow cytometry. Abnormal tissue architecture was seen in the biopsies taken from patients treated with MP, whereas the PC treatment restored normal skin architecture. PC downregulated pro-inflammatory/profibrotic cytokine expression in peripheral cells, and upregulated the number of regulatory T cells (Tregs) in skin. PC was safe and well tolerated. PC is not only an antifibrotic/fibrolytic agent but also an immunomodulator biodrug that restores the balance between T helper (Th)1, Th2, Th17 and Tregs, downregulates production of pro-inflammatory or profibrogenic cytokines (IL-17A, IL-22 and TGF-β1), and renews skin architecture, without adverse effects. © The Author(s). CED

  8. Esophagectomy in Scleroderma: Report of a Case

    Directory of Open Access Journals (Sweden)

    Erdal Yekeler

    2008-12-01

    Full Text Available Scleroderma is a generalized autoimmune disease with variable involvement of the skin and major organs (esophagus, lung, heart and kidney. Scleroderma is essentially a skin disease that frequently involves the digestive system. In scleroderma, the esophagus is the most frequently affected organ of the digestive system, and esophageal dysmotility, reflux and stricture may be observed in the advanced stage. Balloon dilatation and bougienage are generally sufficient in patients developing stricture, and the number of cases in whom resection is performed is very low. In a 20-year-old patient with difficulty in taking even liquid foods, tests revealed sclerodermal involvement of the distal end of the esophagus and stricture. Esophageal resection and gastric replacement were performed. Such systemic diseases as scleroderma, although rare, must be considered in the differential diagnosis of nonmalignant dysphagia, and resection must be borne in mind as a surgical option in cases of advanced stricture.

  9. Actinobacillus actinomycetemcomitans and localized juvenile periodontitis. Clinical, microbiologic and histologic studies.

    Science.gov (United States)

    Christersson, L A

    1993-01-01

    The present studies examined Actinobacillus actinomycetemcomitans and its role in localized juvenile periodontitis (LJP). The distribution of the bacteria was studied in healthy normals, patients with adult periodontitis, diabetics, and those with LJP. Over 95% of the LJP patients harbored A. actinomycetemcomitans, whereas only 17% of healthy subjects, 21% of adult periodontitis patients, and 5% of diabetics were positive. All members of a LJP family harboring the organism yielded isolates of the same biotype and serotype. The transmission of the bacteria was studied after transfer of the bacteria, with periodontal probes from infected to healthy gingival sites, within the oral cavity of LJP patients. Newly colonized gingival sites, 50% of those involved, became free of A. actinomycetemcomitans after only 3 weeks. A purposely forceful inoculation contributed to a more predictable colonization (89%), but only prolonged the colonization with one week. Treatment of LJP lesions with scaling and root planing resulted in minimal clinical and microbiological changes during a 16 week follow-up period. However, gingival curettage and modified Widman flap surgery suppressed A. actinomycetemcomitans in 75% and 89% of the sites, and resulted in resolution of periodontal pocket depth and gain in attachment level. Gingival tissue specimens, from 35 LJP sites, 3 control sites, and one monkey biopsy, were studied to verify the hypothesis of gingival infiltration of A. actinomycetemcomitans. Bacteria were identified immunohistologically with rabbit antisera serospecific to the three A. actinomycetemcomitans serotypes. Positive staining was observed in the tissue from all but one LJP patient. Twenty-eight (80%) lesions were positive for A. actinomycetemcomitans antigens in the gingival connective tissue, often with antigens located both between and within cells. The specimen from a culture positive control demonstrated no signs of invasion, similar to the monkey specimen

  10. My Approach to the Treatment of Scleroderma

    OpenAIRE

    Shah, Ami A.; Wigley, Fredrick M.

    2013-01-01

    Systemic sclerosis (scleroderma) is unique among the rheumatic diseases because it presents the challenge of managing a chronic multisystem autoimmune disease with a widespread obliterative vasculopathy of small arteries that is associated with varying degrees of tissue fibrosis. The hallmark of scleroderma is clinical heterogeneity with subsets that vary in the degree of disease expression, organ involvement, and ultimate prognosis. Thus the term “scleroderma” is used to describe patients th...

  11. ["Deep scleroderma" or diffuse fibromatous scleroderma. A case-report of uncertain nosologic classification (author's transl)].

    Science.gov (United States)

    Herreman, G; Huet, D; Mundler, B; Tamisier, J N; Blum, F; Wechsler, J; Ferme, I

    1982-05-20

    An unusual form of scleroderma, seen in one patient, is described. Clinical and histological examinations demonstrated no dermal changes. Both the subcutaneous tissue and the fascias were involved. This case can be distinguished from Shulman fasciitis, on both histological and biological grounds, as well as from scleroderma, since Raynaud phenomena, sclerodactylia, and visceral involvement were lacking.

  12. Knee contractures as the presenting manifestation of scleroderma.

    OpenAIRE

    Kane-Wanger, G; Ostrov, B E; FREUNDLICH, B.

    1992-01-01

    Musculoskeletal disorders may be the primary manifestations of scleroderma. The case is reported of a patient admitted for an evaluation of severe knee flexion contractures who was diagnosed as having scleroderma but lacked the typical presenting features of the disease. Radiographic, serological, and histological support for this diagnosis are presented. Scleroderma should be considered in the diagnostic evaluation of joint contractures.

  13. A cross-reactive idiotype in scleroderma.

    Science.gov (United States)

    Vázquez-Abad, D; Tian, L; Zanetti, M; Rothfield, N F

    1997-06-01

    Autoantibodies to centromere proteins (anti-CENPs) and to topoisomerase-I are highly specific for scleroderma. Unlike most autoantibodies in other diseases, these autoantibodies are mutually exclusive. We have analysed the idiotypes (Ids) expressed by anti-CENP-B, antitopoisomerase-I, and IgGs from 20 scleroderma patients. Rabbit anti-Ids were prepared to antitopoisomerase-I from two scleroderma patients, and to anti-CENP-B from four patients. These six anti-Ids were used to study the purified autoantibodies from 20 scleroderma patients: four antitopoisomerase-I, 10 anti-CENP-B, and six purified IgG from scleroderma patients who were negative for both autoantibodies. In addition, we studied sera from 40 normal autoantibody-negative controls, and sera and purified immunoglobulins from 17 systemic lupus erythematosus (SLE) patients containing high titres of anti-double-stranded DNA, and/or autoantibodies to extractable nuclear antigens (ENA). Using direct binding, and competitive inhibition ELISAs and immunoblots, we identified an Id present in the heavy chains of all the affinity-purified antitopoisomerase-I, and anti-CENP-B. Interestingly, this Id was also present in the immunoglobulins of the scleroderma patients who had neither of the two autoantibodies. By contrast, cross-reactive Id-EM was not found in the sera or immunoglobulins from 17 SLE patients, or in the sera from 40 normal subjects. Several samples from two patients showed that this cross-reactive Id-EM was stable over time. The scleroderma disease-specific autoantibodies may be identified through a common structural feature at the variable region of the heavy chain: cross-reactive Id-EM.

  14. Juvenile angiofibroma

    Science.gov (United States)

    Nasal tumor; Angiofibroma - juvenile; Benign nasal tumor; Juvenile nasal angiofibroma; JNA ... Juvenile angiofibroma is not very common. It is most often found in adolescent boys. The tumor contains ...

  15. Theory of hantavirus infection spread incorporating localized adult and itinerant juvenile mice

    Science.gov (United States)

    Kenkre, V. M.; Giuggioli, L.; Abramson, G.; Camelo-Neto, G.

    2007-02-01

    A generalized model of the spread of the Hantavirus in mice populations is presented on the basis of recent observational findings concerning the movement characteristics of the mice that carry the infection. The factual information behind the generalization is based on mark-recapture observations reported in Giuggioli et al. [Bull. Math. Biol. 67, 1135 (2005)] that have necessitated the introduction of home ranges in the simple model of Hantavirus spread presented by Abramson and Kenkre [Phys. Rev. E 66, 11912 (2002)]. The essential feature of the model presented here is the existence of adult mice that remain largely confined to locations near their home ranges, and itinerant juvenile mice that are not so confined, and, during their search for their own homes, move and infect both other juveniles and adults that they meet during their movement. The model is presented at three levels of description: mean field, kinetic and configuration. Results of calculations are shown explicitly from the mean field equations and the simulation rules, and are found to agree in some respects and to differ in others. The origin of the differences is shown to lie in spatial correlations. It is indicated how mark-recapture observations in the field may be employed to verify the applicability of the theory.

  16. Juvenile Arrests, 2007. Juvenile Justice Bulletin

    Science.gov (United States)

    Puzzanchera, Charles

    2009-01-01

    This Bulletin summarizes 2007 juvenile crime and arrest data reported by local law enforcement agencies across the country and cited in the FBI report, "Crime in the United States 2007." The Bulletin describes the extent and nature of juvenile crime that comes to the attention of the justice system. It serves as a baseline for comparison for…

  17. Current therapy of systemic sclerosis (scleroderma).

    Science.gov (United States)

    Müller-Ladner, U; Benning, K; Lang, B

    1993-04-01

    Treatment of systemic sclerosis (scleroderma) presents a challenge to both the patient and the physician. Established approaches include long-term physiotherapy, disease-modifying agents such as D-penicillamine, and treatment of organ involvement. These efforts are often unsatisfactory since the results are poor. However, recent advances include treatment of Raynaud's phenomenon (plasmapheresis, stanozolol, and prostacyclin analogues), scleroderma renal crisis (angiotensin-converting enzyme inhibitors), and gastric hypomotility (cisapride). This article covers the current approaches to the disease-modifying therapy including those related to the function of collagen-producing fibroblasts, vascular alterations, and the cellular and humoral immune system, as well as treatment of involved organs.

  18. Association of immunological cell profiles with specific clinical phenotypes of scleroderma disease.

    Science.gov (United States)

    López-Cacho, José Manuel; Gallardo, Soledad; Posada, Manuel; Aguerri, Miriam; Calzada, David; Mayayo, Teodoro; González-Rodríguez, María Luisa; Rabasco, Antonio María; Lahoz, Carlos; Cárdaba, Blanca

    2014-01-01

    This study aimed to search the correlation among immunological profiles and clinical phenotypes of scleroderma in well-characterized groups of scleroderma patients, comparing forty-nine scleroderma patients stratified according to specific clinical phenotypes with forty-nine healthy controls. Five immunological cell subpopulations (B, CD4(+) and CD8(+) T-cells, NK, and monocytes) and their respective stages of apoptosis and activation were analyzed by flow cytometry, in samples of peripheral blood mononuclear cells (PBMCs). Analyses of results were stratified according to disease stage, time since the diagnosis, and visceral damage (pulmonary fibrosis, pulmonary hypertension, and cardiac affliction) and by time of treatment with corticosteroids. An increase in the percentages of monocytes and a decrease in the B cells were mainly related to the disease progression. A general apoptosis decrease was found in all phenotypes studied, except in localized scleroderma. An increase of B and NK cells activation was found in patients diagnosed more than 10 years ago. Specific cell populations like monocytes, NK, and B cells were associated with the type of affected organ. This study shows how, in a heterogeneous disease, proper patient's stratification according to clinical phenotypes allows finding specific cellular profiles. Our data may lead to improvements in the knowledge of prognosis factors and to aid in the analysis of future specific therapies.

  19. Association of Immunological Cell Profiles with Specific Clinical Phenotypes of Scleroderma Disease

    Directory of Open Access Journals (Sweden)

    José Manuel López-Cacho

    2014-01-01

    Full Text Available This study aimed to search the correlation among immunological profiles and clinical phenotypes of scleroderma in well-characterized groups of scleroderma patients, comparing forty-nine scleroderma patients stratified according to specific clinical phenotypes with forty-nine healthy controls. Five immunological cell subpopulations (B, CD4+ and CD8+ T-cells, NK, and monocytes and their respective stages of apoptosis and activation were analyzed by flow cytometry, in samples of peripheral blood mononuclear cells (PBMCs. Analyses of results were stratified according to disease stage, time since the diagnosis, and visceral damage (pulmonary fibrosis, pulmonary hypertension, and cardiac affliction and by time of treatment with corticosteroids. An increase in the percentages of monocytes and a decrease in the B cells were mainly related to the disease progression. A general apoptosis decrease was found in all phenotypes studied, except in localized scleroderma. An increase of B and NK cells activation was found in patients diagnosed more than 10 years ago. Specific cell populations like monocytes, NK, and B cells were associated with the type of affected organ. This study shows how, in a heterogeneous disease, proper patient’s stratification according to clinical phenotypes allows finding specific cellular profiles. Our data may lead to improvements in the knowledge of prognosis factors and to aid in the analysis of future specific therapies.

  20. [Scleroderma related to specific immunotherapy. A report of a case].

    Science.gov (United States)

    Morfín Maciel, Blanca María; Castillo Morfín, Blanca María

    2009-01-01

    It has been described two main phenotypes of helper T cells. On activation, the immune system develops the most effective Th response. Whereas Th1 cells promote cell-mediate immunity against intracellular pathogens and an over expression could favor autoimmune diseases; Th2 cells develop humoral immunity against extracellular pathogens promoting allergic response. Normally, the two profiles coexist in the same individual with different grades of expression. Recently, it has been described a new subset: Th17, which is related to tissue injury in autoimmune diseases. Then, allergic and autoimmune diseases result from an unbalanced response of the immune system. Allergen-specific immunotherapy is the only curative treatment of a specific allergy, which leads to a life-long tolerance against allergens. There are no controlled studies about the effectiveness or risks associated with allergen-specific immunotherapy in patients with autoimmune disorders. On the other hand, scleroderma is an autoimmune chronic systemic disorder of unknown etiology characterized by excess collagen deposition in the skin and viscera, along with vascular injury. We report a girl with allergic asthma and with a second degree family history of systemic sclerosis who developed localized scleroderma during allergen specific immunotherapy. Because allergy vaccination alter the balance between effector and regulatory T-cell populations, which regulate immune tolerance, a positive family history of autoimmunity in first or second degree, could be a contraindication for allergen-specific immunotherapy.

  1. Drug compliance in patients with systemic scleroderma.

    Science.gov (United States)

    Hromadkova, Lucie; Soukup, Tomas; Cermakova, Eva; Vlcek, Jiri

    2012-11-01

    Although drug compliance is a crucial component of treatment effectiveness in chronic diseases, it has never been evaluated in patients with systemic scleroderma. Therefore, the aim of this descriptive study was to determine the drug compliance rate in systemic scleroderma patients and to identify risk factors for noncompliance in these patients. A cross-sectional observational study was conducted. All patients with systemic scleroderma (n = 41) who visited a rheumatic center and signed an informed consent form were included. Data were obtained during structured interviews with patients and from medical records. The Compliance Questionnaire Rheumatology (CQR) was used to determine patient compliance. The relationships between compliance rate and demographic and clinical characteristics were examined. The mean CQR score was 75 %. Based on a dichotomous rating, only 42 % of the patients achieved a satisfactory compliance rate (≥80 %). No relationships between various demographic and clinical characteristics and CQR score expressed as continuous or dichotomous variables were found. This study represents the first evaluation of drug compliance in patients with systemic scleroderma. Many noncompliant patients were identified, but no common risk factors for noncompliance were discovered. The reasons for noncompliance seem to depend on the personal features of the patients.

  2. Medical image of the week: scleroderma

    Directory of Open Access Journals (Sweden)

    Arteaga VA

    2015-04-01

    Full Text Available No abstract available. Article truncated at 150 words. A 56-year-old man presents with cough and dyspnea. Pertinent history is significant for scleroderma. A complete blood count and differential count were unremarkable. A chest radiograph was obtained (Figure 1. Based on overall imaging and clinical history, the chest x-ray findings are highly suggest interstitial lung disease likely related to scleroderma and a recommendation for high resolution chest CT was made. Progressive systemic sclerosis (scleroderma is an autoimmune connective tissue disease that affects 30-50 year old women more often than men and is characterized by the overproduction of collagen which can lead to fibrosis which includes the lungs, skin, and may also affect visceral organs (1. In the hands, vasculitis and Raynaud's phenomenon may lead to distal tapering (2. Although acro-osteolysis or distal tuft resorption can be seen in a wide variety of disorders, it may be present in up to 80% of patients with scleroderma. High-resolution chest CT is ...

  3. Periorbital scleroderma associated with heterochromia iridis.

    Science.gov (United States)

    Stone, R A; Scheie, H G

    1980-12-01

    Two patients had biopsy-proven linear scleroderma, associated with a pigmentary abnormality of the anterior segment of the eye. One patient had heterochromia iridis, atrophy of the upper eyelid, a typical coup de sabre lesion, and a markedly asymmetric pigmentary glaucoma. The second patient had bilateral scalp involvement, paresis of the extraocular muscles, blepharoptosis, and heterochromia iridis.

  4. My approach to the treatment of scleroderma.

    Science.gov (United States)

    Shah, Ami A; Wigley, Fredrick M

    2013-04-01

    Systemic sclerosis (scleroderma) is unique among the rheumatic diseases because it presents the challenge of managing a chronic multisystem autoimmune disease with a widespread obliterative vasculopathy of small arteries that is associated with varying degrees of tissue fibrosis. The hallmark of scleroderma is clinical heterogeneity with subsets that vary in the degree of disease expression, organ involvement, and ultimate prognosis. Thus, the term scleroderma is used to describe patients who have common manifestations that link them together, whereas a highly variable clinical course exists that spans from mild and subtle findings to aggressive, life-threatening multisystem disease. The physician needs to carefully characterize each patient to understand the specific manifestations and level of disease activity to decide appropriate treatment. This is particularly important in treating a patient with scleroderma because there is no treatment that has been proven to modify the overall disease course, although therapy that targets specific organ involvement early before irreversible damage occurs improves both quality of life and survival. This review describes our approach as defined by evidence, expert opinion, and our experience treating patients. Scleroderma is a multisystem disease with variable expression; thus, any treatment plan must be holistic, yet at the same time focus on the dominant organ disease. The goal of therapy is to improve quality of life by minimizing specific organ involvement and subsequent life-threatening disease. At the same time the many factors that alter daily function need to be addressed, including nutrition, pain, deconditioning, musculoskeletal disuse, comorbid conditions, and the emotional aspects of the disease, such as fear, depression, and the social withdrawal caused by disfigurement.

  5. 30例硬皮病临床治疗体会%The 30 Cases of Scleroderma Clinical Treatment Experience

    Institute of Scientific and Technical Information of China (English)

    庞金奎; 杨秀峰

    2015-01-01

    Objective To explore the clinical treatment of scleroderma. Methods To analysis the 30 patients’cases in scleroderma clinical which were chosen from January 2010 to March 2014. Results 30 cases scleroderma patients,13 cases were markedly effective,13 cases were effective,4 cases were invalid, the total efficiency was 86.66%. Conclusion To avoid cold and no smoke on localized scleroderma and systemic scleroderma patients. Steroidal anti-inflammatory drugs can reduce the autoimmune reaction,after the treatment,the symptoms can stop the development or ease.%目的:探讨硬皮病的临床治疗方案。方法选取2010年1月~2014年3月收治的硬皮病患者30例临床治疗方法资料进行分析。结果本组收治的硬皮病患者30例,显效13例,有效13例,无效4例,总有效率86.66%。结论对局限性硬皮病、系统性硬皮病患者,避免受凉,不能吸烟。甾体类抗炎药能减轻自身免疫反应,治疗后症状可停止发展或缓解。

  6. Dermatomyositis (Juvenile)

    Science.gov (United States)

    ... Am A Patient / Caregiver Diseases & Conditions Dermatomyositis (Juvenile) Dermatomyositis (Juvenile) Fast Facts Patients with JDM have varying ... What are common signs and symptoms of juvenile dermatomyositis? The most common signs and symptoms of JDM ...

  7. Retinoschisis (Juvenile)

    Science.gov (United States)

    ... here Home › Eye Conditions Listen Retinoschisis What is Juvenile Retinoschisis? Juvenile retinoschisis is an inherited disease diagnosed in childhood ... degeneration of the retina. What are the symptoms? Juvenile retinoschisis, also known as X-linked retinoschisis, occurs ...

  8. Localization of a gene for an autosomal recessive form of juvenile Parkinsonism to chromosome 6q25.2-27

    Energy Technology Data Exchange (ETDEWEB)

    Matsumine, Hiroto; Shimoda-Matsubayashi, Satoe; Nakagawa-Hattori, Yuko [Tokyo Metropolitan Ebara Hospital (Japan)] [and others

    1997-03-01

    An autosomal recessive form of juvenile Parkinsonism (AR-JP) (MIM 600116) is a levodopa-responsive Parkinsonism whose pathological finding is a highly selective degeneration of dopaminergic neurons in the zona compacta of the substantia nigra. By linkage analysis of diallelic polymorphism of the Mn-superoxide dismutase gene (SOD2), we found a family with AR-JP showing perfect segregation of the disease with the SOD2 locus. By extending the linkage analysis to 13 families with AR-JP, we discovered strong evidence for the localization of the AR-JP gene at chromosome 6q25.2-27, including the SOD2 locus, with the maximal cumulative pairwise LOD scores of 7.26 and 7.71 at D6S305 ({theta} = .03) and D6S253 ({theta} = .02), respectively. Observation of obligate recombination events, as well as multipoint linkage analysis, placed the AR-JP gene in a 17-cM interval between D6S437 and D6S264. Delineation of the AR-JP gene will be an important step toward our understanding of the molecular mechanism underlying selective degeneration of the nigral neurons. 38 refs., 4 figs., 1 tab.

  9. Advances on treatment of scleroderma%硬皮病的治疗及进展

    Institute of Scientific and Technical Information of China (English)

    曾抗; 赖宽; 陈平姣

    2013-01-01

    Systemic sclerosis (SSc) is and connective tissue disease characterized by fibrosis of skin and internal organs. Localized scleroderma only involve skin. The pathogenesis of thess diseases is not clear. Though new therapeutic methods keep coming out and adopted,no specific treatment is identified. Treatments and advances in therapeutic study of localized scleroderma and systemic sclerosis are reviewed in this paper.%硬皮病是一种以皮肤及各系统胶原纤维硬化为特征的结缔组织疾病,病因未明.根据疾病累及的范围可分为局限性硬皮病(localized scleroderma)和系统性硬皮病(systemic sclerosis,SSc).虽然目前尚缺乏特效疗法,但治疗手段在不断增加.本文将对目前的治疗方法及相关进展进行综述.

  10. Scleroderma associated with ANCA-associated vasculitis.

    Science.gov (United States)

    Rho, Young Hee; Choi, Seong Jae; Lee, Young Ho; Ji, Jong Dae; Song, Gwan Gyu

    2006-03-01

    We have recently reported on two cases of scleroderma patients with ANCA-associated vasculitis for the first time in Korea. In order to explore the nature of this disease combination, we pooled together all the previously known cases and statistically analyzed them. Out of the 50 selected cases, survival analysis was done for comparison of the scleroderma disease period and the clinical factors associated with ANCA-associated vasculitis (AAV). Kaplan-Meier analysis revealed that patients having anti-topoisomerase antibody (anti-Scl-70) and, probably, PR-3 ANCA are at a higher risk for developing AAV than patients without both anti-topoisomerase antibody and anti-centromere antibody (ACA), and patients with MPO-ANCA. Multivariate Cox regression analysis revealed having anti-topoisomerase antibody as a risk factor for developing AAV [OR 3.1 (95% CI 1.11-8.55), P=0.031]. We suggest that having anti-topoisomerase antibodies may play a role among scleroderma patients in developing AAV.

  11. A comparison between nailfold capillaroscopy patterns in adulthood in juvenile and adult-onset systemic sclerosis

    DEFF Research Database (Denmark)

    Ingegnoli, Francesca; Boracchi, Patrizia; Gualtierotti, Roberta

    2015-01-01

    OBJECTIVE: Qualitative capillaroscopy patterns in juvenile- and adult-onset systemic sclerosis (SSc) were studied in adulthood using data from the EULAR Scleroderma Trials and Research (EUSTAR) database. METHODS: Data collected between June 2004 and April 2013 were examined with focus...

  12. [Linear scleroderma in children (apropos of 27 cases)].

    Science.gov (United States)

    Larrègue, M; Ziegler, J E; Lauret, P; Bonafe, J; Lorette, G; Titi, A; Ramdenee, P; Bressieux, J M

    1986-01-01

    family. The same associations are found in frontoparietal "coup de sabre" scleroderma. The treatment of linear morphoea is not yet standardized. At the moment, the best and most regular results are obtained with systemic corticosteroids and local physiotherapy.

  13. Phenomenon of isomorphic provoking responses in cases of limited scleroderma

    Directory of Open Access Journals (Sweden)

    Talnikova Е.Е.

    2015-09-01

    Full Text Available The article presents the historical origin of the term "Koebner phenomenon". The literature data reflect the etiology, pathogenesis and epidemiology of isomorphic mechanisms provoking responses in lichen planus, psoriasis, scleroder-ma, syphilis. Variants of the Koebner phenomenon's classifications are given. The clinical cases of limited scleroderma after mechanical injury are described.

  14. Capillary regeneration in scleroderma: stem cell therapy reverses phenotype?

    Directory of Open Access Journals (Sweden)

    Jo N Fleming

    Full Text Available BACKGROUND: Scleroderma is an autoimmune disease with a characteristic vascular pathology. The vasculopathy associated with scleroderma is one of the major contributors to the clinical manifestations of the disease. METHODOLOGY/PRINCIPAL FINDINGS: We used immunohistochemical and mRNA in situ hybridization techniques to characterize this vasculopathy and showed with morphometry that scleroderma has true capillary rarefaction. We compared skin biopsies from 23 scleroderma patients and 24 normal controls and 7 scleroderma patients who had undergone high dose immunosuppressive therapy followed by autologous hematopoietic cell transplant. Along with the loss of capillaries there was a dramatic change in endothelial phenotype in the residual vessels. The molecules defining this phenotype are: vascular endothelial cadherin, a supposedly universal endothelial marker required for tube formation (lost in the scleroderma tissue, antiangiogenic interferon alpha (overexpressed in the scleroderma dermis and RGS5, a signaling molecule whose expression coincides with the end of branching morphogenesis during development and tumor angiogenesis (also overexpressed in scleroderma skin. Following high dose immunosuppressive therapy, patients experienced clinical improvement and 5 of the 7 patients with scleroderma had increased capillary counts. It was also observed in the same 5 patients, that the interferon alpha and vascular endothelial cadherin had returned to normal as other clinical signs in the skin regressed, and in all 7 patients, RGS5 had returned to normal. CONCLUSION/SIGNIFICANCE: These data provide the first objective evidence for loss of vessels in scleroderma and show that this phenomenon is reversible. Coordinate changes in expression of three molecules already implicated in angiogenesis or anti-angiogenesis suggest that control of expression of these three molecules may be the underlying mechanism for at least the vascular component of this disease

  15. Digital Ischemia in Scleroderma Spectrum of Diseases

    Directory of Open Access Journals (Sweden)

    Elena Schiopu

    2010-01-01

    Full Text Available Systemic Sclerosis (Scleroderma, SSc is a disease of unknown etiology characterized by widespread vasculopathy and extracellular matrix deposition leading to fibrosis and autoimmune processes. Digital ischemia (digital ulcers (DUs is the hallmark of SSc-related vasculopathy and is characterized by endothelial dysfunction leading to intimal proliferation and thrombosis. It happens frequently (30% of the patients each year and it is associated with significant morbidity. This paper summarizes the current information regarding pathogenesis, definitions, management, and exploratory therapies in DUs associated with SSc.

  16. Genetics of scleroderma: implications for personalized medicine?

    Directory of Open Access Journals (Sweden)

    Assassi Shervin

    2013-01-01

    Full Text Available Abstract Significant advances have been made in understanding the genetic basis of systemic sclerosis (scleroderma in recent years. Can these discoveries lead to individualized monitoring and treatment? Besides robustly replicated genetic susceptibility loci, several genes have been recently linked to various systemic sclerosis disease manifestations. Furthermore, inclusion of genetic studies in design and analysis of drug trials could lead to development of genetic biomarkers that predict treatment response. Future genetic studies in well-characterized systemic sclerosis cohorts paired with advanced analytic approaches can lead to development of genetic biomarkers for targeted diagnostic and therapeutic interventions in systemic sclerosis.

  17. Non-surgical periodontal management in scleroderma disease patients.

    Science.gov (United States)

    Laforgia, A; Corsalini, M; Stefanachi, G; Tafuri, S; Ballini, A; Pettini, F; Di Venere, D

    2016-01-01

    The aim of the present study is to investigate the periodontal status of people with scleroderma and their response to non-surgical treatment protocol aimed at controlling the evolution of the disease. The response to non-surgical periodontal treatment was tested on patients belonging to a scleroderma group and a control group: the data show an improvement of the periodontal conditions of all these patients in response to treatment. When compared on the same diagram, a slight remission of the periodontal disease was obtained in both scleroderma and healthy patients. This highlights the benefit to soft tissues produced by non-surgical periodontal treatment also in patients affected by systemic diseases.

  18. Redes (des)conexas de intervenção local na violência infanto-juvenil

    OpenAIRE

    Sebastião, João; Campos, Joana; Merlini, Sara; Chambino, Mafalda

    2013-01-01

    O objectivo deste estudo é apresentar os resultados de uma análise diagnóstica da atuação institucional relativamente às situações de violência infanto-juvenil em três territórios de um concelho da períferia de Lisboa(Portugal)

  19. Scleroderma Renal Crisis Precipitated by Steroid Treatment in ...

    African Journals Online (AJOL)

    Patients may present with features of more than one specific disease without ... Several cases of systemic sclerosis (SSc) and systemic lupus erythematosus ... She was diagnosed as SLE and scleroderma overlap syndrome based on clinical ...

  20. Bilateral coxitis in scleroderma-polymyositis overlap syndrome

    Science.gov (United States)

    Berrada, Khadija; Abourazzak, Fatima Ezzahra; Houssaini, Ghita Sqalli; Kadi, Nadira; Tahiri, Latifa; Amrani, Kawthar; Khammar, Zineb; Lahlou, Meriam; Berrady, Rhizlane; Rabhi, Samira; Tizniti, Siham; Bono, Wafaa; Harzy, Taoufik

    2014-01-01

    Joint manifestations in scleroderma (Scl) and polymyositis (PM) are dominated by inflammatory arthralgia. Arthritis is less common and preferentially affects the hands, wrists, knees, and ankles. Involvement of the hip has been rarely reported in the literature. We report a case of coxitis diagnosed in a patient suffering from scleroderma-polymyositis overlap syndrome successfully treated by ultrasound-guided infiltration of triamcinolone hexacetonide PMID:27708891

  1. Presentation of acute central retinal vein occlusion in scleroderma

    OpenAIRE

    Malik, Faisal; Al Habash, Ahmed

    2014-01-01

    Central retinal vein occlusion (CRVO) is a rare complication of scleroderma. Here we report a case of a 30-year-old man who was diagnosed to have scleroderma in the rheumatology and dermatology clinic. During treatment with systemic steroids and immunosuppressive therapy the patient developed a sudden decrease of vision in the right eye and was diagnosed to have right CRVO with macular edema on fundus examination. After three consecutive Intravitreal bevacizumab (IVB) injections for macular e...

  2. Intravital microscopy and capillaroscopically guided nail fold biopsy in scleroderma.

    OpenAIRE

    von Bierbrauer, A F; Mennel,H. D.; Schmidt, J A; von Wichert, P

    1996-01-01

    OBJECTIVES: To describe the frequency, extent, and nature of microvascular lesions in patients with scleroderma by means of capillaroscopy and capillaroscopically guided nail fold biopsy, and to determine the diagnostic value of the two methods and the pathophysiological significance of the lesions described. METHODS: A cohort study was made of 24 consecutive patients with scleroderma and 10 healthy controls, using standardised clinical, serological, capillaroscopic, and histological (nail fo...

  3. Scleroderma Renal Crisis: A Pathology Perspective

    Directory of Open Access Journals (Sweden)

    Ibrahim Batal

    2010-01-01

    Full Text Available Scleroderma renal crisis (SRC is an infrequent but serious complication of systemic sclerosis (SSc. It is associated with increased vascular permeability, activation of coagulation cascade, and renin secretion, which may lead to the acute renal failure typically associated with accelerated hypertension. The histologic picture of SRC is that of a thrombotic microangiopathy process with prominent small vessel involvement manifesting as myxoid intimal changes, thrombi, onion skin lesions, and/or fibrointimal sclerosis. Renal biopsies play an important role in confirming the clinical diagnosis, excluding overlapping/superimposed diseases that might lead to acute renal failure in SSc patients, helping to predict the clinical outcome and optimizing patient management. Kidney transplantation may be the only treatment option available for a subset of SRC patients who develop end-stage renal failure despite aggressive angiotensin-converting enzyme inhibitor therapy. However, the posttransplant outcome for SSc patients is currently suboptimal compared to the general renal transplant population.

  4. DIAGNOSIS OF PULMONARY HYPERTENSION IN SCLERODERMA SYSTEMATICA

    Directory of Open Access Journals (Sweden)

    A V Volkov

    2008-12-01

    Full Text Available Pulmonary hypertension (PH associated with scleroderma systematica (SDS is a menacing manifestation of this systemic disease of connective tissue, in which a rapid progression results in very poor outcomes. In SDS, PH is more frequently observed with the prolonged disease, circumscribed skin lesion, develops after a long benign course, and is one of the common causes of death. The early stage of PH can be identified by instrumental and not always accessible studies. The stage of clinical manifestations, which is frequently manifested only by dyspnea, requires a differential diagnosis from a wide range of conditions both caused by and concurrent with SDS. The need for differential diagnosis stems from the varying course and prognosis of the disease, as well as treatment policy.

  5. DIAGNOSIS OF PULMONARY HYPERTENSION IN SCLERODERMA SYSTEMATICA

    Directory of Open Access Journals (Sweden)

    A V Volkov

    2008-01-01

    Full Text Available Pulmonary hypertension (PH associated with scleroderma systematica (SDS is a menacing manifestation of this systemic disease of connective tissue, in which a rapid progression results in very poor outcomes. In SDS, PH is more frequently observed with the prolonged disease, circumscribed skin lesion, develops after a long benign course, and is one of the common causes of death. The early stage of PH can be identified by instrumental and not always accessible studies. The stage of clinical manifestations, which is frequently manifested only by dyspnea, requires a differential diagnosis from a wide range of conditions both caused by and concurrent with SDS. The need for differential diagnosis stems from the varying course and prognosis of the disease, as well as treatment policy.

  6. Novel Insights Into Causes of Scleroderma Offer Potential New Treatment Strategies

    Science.gov (United States)

    ... Into Causes of Scleroderma Offer Potential New Treatment Strategies Integrins, a large class of cell surface molecules, ... that targeting integrins could be a promising treatment strategy for scleroderma. The scientists were also curious about ...

  7. Lichen sclerosus et atrophicans, scleroderma en coup de sabre and Lyme borreliosis

    OpenAIRE

    Serena Bonin; Nicoletta Gubertini; Giusto Trevisan

    2011-01-01

    Lichen sclerosus et atrophicans (LSA) is a chronic, inflammatory skin disease of unknown etiology, characterized by atrophy. We report a case of LSA with frontoparietal distribution, mimicking scleroderma en coup de sabre, causing scarring alopecia. The case was associated with Borrelia infection. The lesion improved with 2 cycles of antibiotic therapy with ceftriaxone 2 gr /day i.v for 21 days associated with UVA-1 therapy and local and systemic vitamin E supply (400 mg 2x/day per os for 3 ...

  8. Juvenile Judge

    Institute of Scientific and Technical Information of China (English)

    1997-01-01

    SHANG Xiuyun was among the first sitting judges when the juvenile court was set up in Beijing 10 years ago. With enriched experience she has altered the way judges ask questions in court. She began the practice of inviting juvenile offenders, their parents, relatives, friends and teachers to the juvenile court to work hand in hand in dealing with cases: Facing their relatives and friends and hearing their heartfelt words, juvenile offenders would often be touched, thus bringing forth a positive attitude toward life.

  9. Juvenile Arthritis

    Science.gov (United States)

    Juvenile arthritis (JA) is arthritis that happens in children. It causes joint swelling, pain, stiffness, and loss of motion. It can affect any joint, but ... of JA that children get is juvenile idiopathic arthritis. There are several other forms of arthritis affecting ...

  10. Anticorpos antileucotoxina contra Actinobacillus actinomycetemcomitans em amostras de soro e saliva de pacientes com periodontite juvenil localizada Anti-leukotoxin antibodies against Actinobacillus actinomycetemcomitans in serum and saliva samples from patients with localized juvenile periodontitis

    Directory of Open Access Journals (Sweden)

    Roberto Issamu NAKAGAWA

    2001-03-01

    Full Text Available A leucotoxina de Actinobacillus actinomycetemcomitans é considerada seu principal fator de virulência com potencial de causar agressão às defesas do hospedeiro. No presente trabalho, foram analisados os níveis séricos e salivares de anticorpos antileucotoxina de A. actinomycetemcomitans em soros e salivas de pacientes com periodontite juvenil localizada (PJL e controles saudáveis. Adicionalmente, foi realizada a análise de complexo imune (CI nas amostras de saliva. Foram utilizados os métodos ELISA clássico com a leucotoxina obtida por gel filtração em Sephadex G-200 e ELISA de captura utilizando IgG de coelho anti-A. actinomycetemcomitans FDC Y4 leucotóxico adsorvido com uma cepa da mesma espécie, porém, não leucotóxica. Os resultados obtidos demonstraram níveis séricos de IgG significativamente mais elevados em pacientes com PJL em relação aos controles sadios, tanto por ELISA clássico como por ELISA de captura (p The leukotoxin produced by Actinobacillus actinomycetemcomitans is considered the major virulence factor with potential to cause damage to the host defenses. The present work analyzed the serumal and salivary levels of antibodies against the leukotoxin produced by A. Actinomycetemcomitans, in patients with Localized Juvenile Periodontitis (LJP and in healthy controls. Additionally, analysis of the immune complex (IC was carried out in saliva samples . The classic ELISA method, with leukotoxin obtained through Sephadex G-200 gel filtration, and the capture ELISA method, using rabbit anti-A. Actinomycetemcomitans (leucotoxic, FDC Y4, IgG adsorbed with a non-leukotoxic strain of A. actinomycetemcomitans, were used. The results obtained demonstrated significantly higher serumal levels of IgG in patients with LJP, when they were compared with the healthy controls, both for the classic and capture ELISA methods (p < 0.05. However, no significant differences were observed between the salivary levels of IgG, SIgA and IC

  11. Scleroderma of geriatric age and scleroderma-like paraneoplastic syndrome – description of two cases

    Directory of Open Access Journals (Sweden)

    Magdalena Marek

    2016-06-01

    Full Text Available Systemic sclerosis (Ssc is an autoimmune connective tissue disease of unknown origin, characterized by progressive fibrosis of the skin and internal organs. Immune reactions taking part in Ssc pathogenesis may contribute to cancer development; therefore patients with risk factors for this disease require observation for a neoplastic process. On the other hand, symptoms of Ssc may be a mask of various cancers. Differentiating between the idiopathic form of Ssc and scleroderma-like paraneoplastic syndrome often causes a lot of difficulties. The article presents two cases of Ssc at the beginning of the disease after 60 years of age. The first case was diagnosed as Ssc, whereas in the second case the defined diagnosis was scleroderma-like syndrome in the course of colorectal cancer. This paper presents an analysis of differential diagnostic procedures which were performed and led to the final diagnosis, mentions types of cancers co-occurring with Ssc and suggests a screening scheme for cancer development in patients with a diagnosis of Ssc.

  12. Scleroderma of geriatric age and scleroderma-like paraneoplastic syndrome - description of two cases.

    Science.gov (United States)

    Marek, Magdalena; Rudny, Robert

    2016-01-01

    Systemic sclerosis (Ssc) is an autoimmune connective tissue disease of unknown origin, characterized by progressive fibrosis of the skin and internal organs. Immune reactions taking part in Ssc pathogenesis may contribute to cancer development; therefore patients with risk factors for this disease require observation for a neoplastic process. On the other hand, symptoms of Ssc may be a mask of various cancers. Differentiating between the idiopathic form of Ssc and scleroderma-like paraneoplastic syndrome often causes a lot of difficulties. The article presents two cases of Ssc at the beginning of the disease after 60 years of age. The first case was diagnosed as Ssc, whereas in the second case the defined diagnosis was scleroderma-like syndrome in the course of colorectal cancer. This paper presents an analysis of differential diagnostic procedures which were performed and led to the final diagnosis, mentions types of cancers co-occurring with Ssc and suggests a screening scheme for cancer development in patients with a diagnosis of Ssc.

  13. Scleroderma of geriatric age and scleroderma-like paraneoplastic syndrome – description of two cases

    Science.gov (United States)

    Rudny, Robert

    2016-01-01

    Systemic sclerosis (Ssc) is an autoimmune connective tissue disease of unknown origin, characterized by progressive fibrosis of the skin and internal organs. Immune reactions taking part in Ssc pathogenesis may contribute to cancer development; therefore patients with risk factors for this disease require observation for a neoplastic process. On the other hand, symptoms of Ssc may be a mask of various cancers. Differentiating between the idiopathic form of Ssc and scleroderma-like paraneoplastic syndrome often causes a lot of difficulties. The article presents two cases of Ssc at the beginning of the disease after 60 years of age. The first case was diagnosed as Ssc, whereas in the second case the defined diagnosis was scleroderma-like syndrome in the course of colorectal cancer. This paper presents an analysis of differential diagnostic procedures which were performed and led to the final diagnosis, mentions types of cancers co-occurring with Ssc and suggests a screening scheme for cancer development in patients with a diagnosis of Ssc. PMID:27407287

  14. Cytokines as Therapeutic Targets for the Gastrointestinal Manifestations of Scleroderma

    Directory of Open Access Journals (Sweden)

    Jennifer M Raoul

    2004-01-01

    Full Text Available Systemic sclerosis (SSc, or scleroderma, is a connective tissue disorder characterized by progressive fibrosis of the skin and internal organs. It has significance for gastroenterologists because the gastrointestinal tract is involved in 90% of SSc patients, who often present with esophageal dysfunction. Though the exact pathogenesis of SSc is unknown, there is increasing evidence supporting an immune mechanism. Cytokines are the soluble mediators of immune activation, altered fibroblast proliferation and extracellular matrix accumulation in SSc and thereby provide important therapeutic targets. In the present review, the involvement of cytokines in SSc is discussed with particular emphasis on cytokines and growth factors that have been implicated in the disease process and likely play an important role in the gastrointestinal manifestations of scleroderma. The role of cytokines as therapeutic targets in scleroderma forms the basis of this timely review

  15. "Scleroderma linearis: hemiatrophia faciei progressiva (Parry-Romberg syndrom) without any changes in CNS and linear scleroderma "en coup de sabre" with CNS tumor

    OpenAIRE

    Brzezińska-Wcisło Ligia; Lis-Święty Anna; Bergler-Czop Beata

    2009-01-01

    Abstract Background Hemifacial atrophy (Parry-Romberg syndrome) is a relatively rare disease. The etiology of the disease is not clear. Some authors postulate its relation with limited scleroderma linearis. Linear scleroderma "en coup de sabre" is characterized by clinical presence of most commonly one-sided linear syndrome. In a number of patients, neurological affection is the medium of the disease. The treatment of both scleroderma varieties is similar to the treatment of limited systemic ...

  16. Scleroderma and sudden sensorineural hearing loss

    Directory of Open Access Journals (Sweden)

    Ravinder Verma

    2014-01-01

    Full Text Available Sudden sensorineural hearing loss (SNHL is an emergency in otolaryngological practice. The exact cause, in most of the cases cannot be found. A triad of vascular, viral, and membrane rupture are the known theories for the causation of sudden SNHL. Now various autoimmune disorders have also been suggested to play a role in the pathogenesis. Autoimmune inner ear disease in human being is an uncommon, but a distinct clinical entity. The diagnosis of the inner ear autoimmune disorder depends mainly on relatively distinct clinical course, immune laboratory tests and treatment response. The clinical picture usually consists of bilateral SNHL, bilateral reduced vestibular loss and symptoms of pressure with tinnitus and rarely tissue destruction of middle and external ear. Systemic scleroderma is an aggressive multi-system and multistage connective tissue disorder with high mortality. It is characterized by proliferative vascular lesion and marked deposition of collagen in various tissues and organs. Responses to immunosuppressive drugs are cited as evidence of an autoimmune process.

  17. Molecular subsets in the gene expression signatures of scleroderma skin.

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    Ausra Milano

    Full Text Available BACKGROUND: Scleroderma is a clinically heterogeneous disease with a complex phenotype. The disease is characterized by vascular dysfunction, tissue fibrosis, internal organ dysfunction, and immune dysfunction resulting in autoantibody production. METHODOLOGY AND FINDINGS: We analyzed the genome-wide patterns of gene expression with DNA microarrays in skin biopsies from distinct scleroderma subsets including 17 patients with systemic sclerosis (SSc with diffuse scleroderma (dSSc, 7 patients with SSc with limited scleroderma (lSSc, 3 patients with morphea, and 6 healthy controls. 61 skin biopsies were analyzed in a total of 75 microarray hybridizations. Analysis by hierarchical clustering demonstrates nearly identical patterns of gene expression in 17 out of 22 of the forearm and back skin pairs of SSc patients. Using this property of the gene expression, we selected a set of 'intrinsic' genes and analyzed the inherent data-driven groupings. Distinct patterns of gene expression separate patients with dSSc from those with lSSc and both are easily distinguished from normal controls. Our data show three distinct patient groups among the patients with dSSc and two groups among patients with lSSc. Each group can be distinguished by unique gene expression signatures indicative of proliferating cells, immune infiltrates and a fibrotic program. The intrinsic groups are statistically significant (p<0.001 and each has been mapped to clinical covariates of modified Rodnan skin score, interstitial lung disease, gastrointestinal involvement, digital ulcers, Raynaud's phenomenon and disease duration. We report a 177-gene signature that is associated with severity of skin disease in dSSc. CONCLUSIONS AND SIGNIFICANCE: Genome-wide gene expression profiling of skin biopsies demonstrates that the heterogeneity in scleroderma can be measured quantitatively with DNA microarrays. The diversity in gene expression demonstrates multiple distinct gene expression programs

  18. Juvenile Prostitution.

    Science.gov (United States)

    Csapo, Marg

    1986-01-01

    Recent research and Canadian government committee reports concerning juvenile prostitution are reviewed. Proposals are made in the realms of law and social policy; and existing programs are described. (DB)

  19. Juvenile Prostitution.

    Science.gov (United States)

    Csapo, Marg

    1986-01-01

    Recent research and Canadian government committee reports concerning juvenile prostitution are reviewed. Proposals are made in the realms of law and social policy; and existing programs are described. (DB)

  20. Juvenile myasthenia

    Directory of Open Access Journals (Sweden)

    Knežević-Pogančev Marija

    2011-01-01

    Full Text Available Introduction. Juvenile myasthenia is a chronic autoimmune neuromuscular disease characterized by varying degrees of fluctuating, painless muscle weakness and rapid fatigue of any muscles under voluntary control. Juvenile myasthenia is a form of myasthenia appearing in adolescent age, representing 10% to 15% of all cases of myasthenia gravis. Juvenile myasthenia is presented by a defect in the transmission of nerve impulses to muscles, resulting from a breakdown in the normal communication between nerves and muscles. In myasthenia, antibodies produced by the body’s own immune system block, alter, or destroy the receptors for acetylcholine. Juvenile myasthenia is neither directly inherited nor is it contagious. Signs and Symptoms. The first noticeable symptoms may be eye muscle weakness, difficulty in swallowing, or slurred speech. Juvenile myasthenia usually affects muscles innervated by the cranial nerves (face, lips, tongue, neck and throat, but it can affect any muscle group. Symptoms vary in type and severity with typical periods of exacerbation interspersed with periods of remission. When the muscles necessary for breathing are affected, a patient is said to be in a myasthenic crisis, which is a life-threatening situation. Disease Outcome and Treatment. Juvenile myasthenia produces sporadic but progressive weakness and abnormal fatigability of striated (skeletal muscles, exacerbated by exercise and repeated movement, but improved by rest and anticholinesterase drugs. Juvenile myasthenia follows an unpredictable course of recurring exacerbations and periodic remissions. With current therapies, however, most cases of juvenile myasthenia are not as serious as the name implies. Although there is no known cure, drug treatment has improved prognosis and allows patients to lead relatively normal lives, except during exacerbations.

  1. Partially Evoked Epithelial-Mesenchymal Transition (EMT Is Associated with Increased TGFβ Signaling within Lesional Scleroderma Skin.

    Directory of Open Access Journals (Sweden)

    Joanna Nikitorowicz-Buniak

    Full Text Available The origin of myofibroblasts in fibrotic conditions remains unknown and in systemic sclerosis (SSc it has been proposed that activation of local fibroblasts, trans-differentiation of perivascular or vascular cells, recruitment of fibrocyte progenitors, or epithelial to mesenchymal transition (EMT could be contributing. Data from our laboratory indicate that the epidermis in scleroderma is activated with the keratinocytes exhibiting a phenotype normally associated with tissue repair, including phosphorylation profiles indicative of TGFβ signaling. Since TGFβ is a known inducer of EMT, we investigated if there is evidence of this process in the SSc epidermis. In order to validate antibodies and primers, EMT was modeled in HaCaT cells cultured in the presence of TGFβ1. Skin sections were stained with phosho-SMAD2/3, as well as with epithelial and mesenchymal markers. Moreover, mRNA levels of transcription factors associated with EMT were studied in epidermal blister sheets. We observed critical changes in the scleroderma epidermis; showing significantly increased nuclear translocation of phosphorylated Smad2/3, consistent with active TGFβ signaling in SSc keratinocytes. While profound EMT could be induced in keratinocytes in vitro with the appearance of SNAI1/2 and FSP-1, and an accompanying loss of E-cadherin, in the scleroderma skin active TGFβ signaling was accompanied by only partial EMT-like changes characterised by induction of SNAI1 alone and with no loss of E-cadherin. Together, our findings support a model of altered differentiation and TGFβ dependent activation of scleroderma epithelial cells leading to a partially evoked EMT like process in the fibrotic skin.

  2. Exploring Sources of Emotional Distress among People Living with Scleroderma: A Focus Group Study.

    Directory of Open Access Journals (Sweden)

    Stephanie T Gumuchian

    Full Text Available Systemic sclerosis, or scleroderma, is a chronic and rare connective tissue disease with negative physical and psychological implications. Sources of emotional distress and the impact they have on the lives of people with scleroderma are not well understood.To gain an in-depth understanding of the emotional experiences and sources of emotional distress for women and men living with scleroderma through focus group discussions.Three semi-structured focus group discussions were conducted (two in English, one in French with a total of 22 people with scleroderma recruited through the Scleroderma Society of Ontario in Hamilton, Ontario and a scleroderma clinic in Montreal, Canada. Interviews were recorded, transcribed, and then coded for emerging themes using thematic inductive analysis.Core themes representing sources of emotional distress were identified, including: (a facing a new reality; (b the daily struggle of living with scleroderma; (c handling work, employment and general financial burden; (d changing family roles; (e social interactions; and (f navigating the health care system. Collectively, these themes refer to the stressful journey of living with scleroderma including the obstacles faced and the emotional experiences beginning prior to receiving a diagnosis and continuing throughout the participants' lives.Scleroderma was portrayed as being an unpredictable and overwhelming disease, resulting in many individuals experiencing multiple sources of emotional distress. Interventions and supportive resources need to be developed to help individuals with scleroderma and people close to them manage and cope with the emotional aspects of the disease.

  3. Juvenile idiopathic arthritis

    Science.gov (United States)

    Juvenile rheumatoid arthritis (JRA); Juvenile chronic polyarthritis; Still disease; Juvenile spondyloarthritis ... The cause of juvenile idiopathic arthritis (JIA) is not known. It ... illness . This means the body attacks and destroys healthy body ...

  4. Juvenile Polyposis Syndrome

    Science.gov (United States)

    ... Types of Cancer > Juvenile Polyposis Syndrome Request Permissions Juvenile Polyposis Syndrome Approved by the Cancer.Net Editorial Board , 12/2015 What is juvenile polyposis syndrome? Juvenile polyposis syndrome (JPS) is a ...

  5. A Survey of TCM Studies on Systemic Scleroderma

    Institute of Scientific and Technical Information of China (English)

    Hu Dongliu; Chen Dacan; Xuan Guowei

    2006-01-01

    @@ Systemic scleroderma (SSc) is a progressive dermatosis with symmetric skin sclerosis and ischemia of the fingers or toes, accompanied with lesions of the joints, muscles and many internal organs, and it may clinically result in functional disablement due to sclerosis, rigidity and atrophy of the skin1. The following is a survey of the basic and clinical studies of TCM on SSc.

  6. Patient-Reported Outcome Measures in Systemic Sclerosis (Scleroderma).

    Science.gov (United States)

    Pellar, Russell E; Tingey, Theresa M; Pope, Janet Elizabeth

    2016-05-01

    Scleroderma (systemic sclerosis) is a rare autoimmune connective tissue disease that can damage multiple organs and reduce quality of life. Patient-reported outcome measures capture the patient's perspective. Some measures are specific to systemic sclerosis and others are general. Patient-reported outcomes in systemic sclerosis are important to aid in understanding the impact of systemic sclerosis on patients.

  7. Clinical experience with pirfenidone in five patients with scleroderma-related interstitial lung disease.

    Science.gov (United States)

    Miura, Yukiko; Saito, Takefumi; Fujita, Kazutaka; Tsunoda, Yoshiya; Tanaka, Toru; Takoi, Hiroyuki; Yatagai, Yohei; Rin, Shigen; Sekine, Akimasa; Hayashihara, Kenji; Nei, Takahito; Azuma, Arata

    2014-10-20

    Interstitial lung disease is the most common complication and cause of death among patients with scleroderma. Scleroderma-related interstitial lung disease has usually been treated with cyclophosphamide; however, its effect was evaluated to be modest and long-term administration of this drug is associated with adverse effects. Herein, we report our clinical experience of administering pirfenidone, which is an antifibrotic agent, in five patients with scleroderma-related interstitial lung disease. All patients demonstrated an increase in vital capacity.

  8. ANCA-associated vasculitis in scleroderma: a case series of fourteen patients

    OpenAIRE

    Liang, Kimberly P.; Michet, Clement J

    2011-01-01

    Antimyeloperoxidase (MPO), perinuclear antineutrophil cytoplasmic antibodies (pANCA), and/or clinically evident vasculitis in patients with scleroderma have been reported only rarely. The clinical significance and prognosis of ANCA-associated vasculitis in systemic sclerosis is uncertain. To report a case and identify the clinical characteristics of scleroderma patients with ANCA-associated vasculitis. Patients with both vasculitis and scleroderma occurring between 1976 to 2006 were identifie...

  9. Juvenile Spondyloarthritis

    Science.gov (United States)

    Gmuca, Sabrina; Weiss, Pamela F.

    2015-01-01

    Purpose of review To provide a comprehensive update of the pathogenesis, diagnostic imaging, treatments, and disease activity measurements of juvenile spondyloarthritis (JSpA). Recent findings Genetic and microbiome studies have provided new information regarding possible pathogenesis of JSpA. Recent work suggests that children with JSpA have decreased thresholds for pain in comparison to healthy children. Additionally, pain on physical examination and abnormalities on ultrasound of the entheses are not well correlated. Treatment guidelines for juvenile arthritis, including JSpA, were published by the American College of Rheumatology and are based on active joint count and presence of sacroiliitis. Recent studies have established the efficacy of tumor necrosis factor inhibitors in the symptomatic treatment of axial disease, though their efficacy for halting progression of structural damage is less clear. Newly developed disease activity measures for JSpA include the Juvenile Arthritis Disease Activity Score and the JSpA Disease Activity index. In comparison to other categories of juvenile arthritis, children with JSpA are less likely to attain and sustain inactive disease. Summary Further microbiome and genetic research may help elucidate JSpA pathogenesis. More randomized therapeutic trials are needed and the advent of new composite disease activity measurement tools will hopefully allow for the design of these greatly needed trials. PMID:26002028

  10. Mobilizing Communities To Prevent Juvenile Crime. Juvenile Justice Bulletin.

    Science.gov (United States)

    Bownes, Donna; Ingersoll, Sarah

    Through Title V Incentive Grants for Local Delinquency Prevention Programs (Community Prevention Grants), the Office of Juvenile Justice and Delinquency Prevention (OJJDP) allocated $20 million in fiscal year 1997 to states to complement law enforcement and justice system efforts by helping local communities foster strong families and nurture…

  11. Effects of replacing fishmeal with wastes derived from local fisheries on the growth of juvenile tilapia, Oreochromis niloticus

    Directory of Open Access Journals (Sweden)

    Chanagun Chitmanat

    2009-01-01

    Full Text Available A feeding trial was conducted to investigate the effects of partially and totally replacing fishmeal with by-product derived from local fisheries on growth performances of tilapia (Oreochromis niloticus. Tilapia fingerlings (average initialweight 0.38±0.05 g were fed with 5 different diet formulas composing of fish meal protein replacement levels of 0%, 25%,50%, 75, and 100%. Tilapia were raised in 80 x 80 x 80 cm3 hapa in an earthen pond for 8 weeks. Each treatment contained3 replications. No feeding trial was applied as negative control. The result showed that fish by-product powder could substitute for fishmeal on a crude protein basis at a level of not more than 25%. As a result, feed cost can only be cut down approximately 3 US cents/kg. Specific growth rate, weight gain, survival rate and feed conversion ratio were not significantly different between the fish fed with the 75% and 100% fishmeal containing diets (P>0.05. The outcome would beapplied to reduce the solid wastes from fish processing and partially replace the imported fishmeal. This can also be used as guideline for farmers in small communities to produce their own tilapia feed.

  12. Increased Levels of Type I and III Collagen and Hyaluronan in Scleroderma Skin

    DEFF Research Database (Denmark)

    Søndergaard, Klaus; Heickendorff, Lene; L, Risteli

    1997-01-01

    The aminoterminal propeptide of type III procollagen (PIIINP) and the carboxyterminal propeptide of type I procollagen (PICP) and hyaluronan (HA) were measured in plasma and suction blister fluid from 13 systemic sclerosis patients and 11 healthy volunteers. Suction blisters and skin biopsies were...... from the transition zone between normal skin and scleroderma, and uninvolved abdominal skin of patients. The median value of suction blister PIIINP from the transition zone was 38% higher than suction blister PIIINP from uninvolved skin. PIIINP was localized to the dermis by immunohistochemical...... techniques. PICP and HA levels in blisters from the transition zone were 87% and 53%, respectively, above the levels measured in uninvolved skin. Furthermore, PICP and HA blister levels from the transition zone were 67% and 63%, respectively, higher than the levels measured in healthy volunteers. In plasma...

  13. Lichen sclerosus et atrophicans, scleroderma en coup de sabre and Lyme borreliosis.

    Science.gov (United States)

    Gubertini, Nicoletta; Bonin, Serena; Trevisan, Giusto

    2011-08-03

    Lichen sclerosus et atrophicans (LSA) is a chronic, inflammatory skin disease of unknown etiology, characterized by atrophy. We report a case of LSA with frontoparietal distribution, mimicking scleroderma en coup de sabre, causing scarring alopecia. The case was associated with Borrelia infection. The lesion improved with 2 cycles of antibiotic therapy with ceftriaxone 2 gr/day i.v for 21 days associated with UVA-1 therapy and local and systemic vitamin E supply (400 mg 2x/day per os for 3 months). This case stresses the importance of identifying clinical manifestations associated with Lyme disease and the use of tissue PCR to detect borrelial DNA in patients with these lesions, but characterized by negative serology for Borrelia.

  14. Increased Levels of Type I and III Collagen and Hyaluronan in Scleroderma Skin

    DEFF Research Database (Denmark)

    Søndergaard, Klaus; Heickendorff, Lene; L, Risteli

    1997-01-01

    The aminoterminal propeptide of type III procollagen (PIIINP) and the carboxyterminal propeptide of type I procollagen (PICP) and hyaluronan (HA) were measured in plasma and suction blister fluid from 13 systemic sclerosis patients and 11 healthy volunteers. Suction blisters and skin biopsies were...... from the transition zone between normal skin and scleroderma, and uninvolved abdominal skin of patients. The median value of suction blister PIIINP from the transition zone was 38% higher than suction blister PIIINP from uninvolved skin. PIIINP was localized to the dermis by immunohistochemical...... techniques. PICP and HA levels in blisters from the transition zone were 87% and 53%, respectively, above the levels measured in uninvolved skin. Furthermore, PICP and HA blister levels from the transition zone were 67% and 63%, respectively, higher than the levels measured in healthy volunteers. In plasma...

  15. Lichen sclerosus et atrophicans, scleroderma en coup de sabre and Lyme borreliosis

    Directory of Open Access Journals (Sweden)

    Serena Bonin

    2011-09-01

    Full Text Available Lichen sclerosus et atrophicans (LSA is a chronic, inflammatory skin disease of unknown etiology, characterized by atrophy. We report a case of LSA with frontoparietal distribution, mimicking scleroderma en coup de sabre, causing scarring alopecia. The case was associated with Borrelia infection. The lesion improved with 2 cycles of antibiotic therapy with ceftriaxone 2 gr /day i.v for 21 days associated with UVA-1 therapy and local and systemic vitamin E supply (400 mg 2x/day per os for 3 months. This case stresses the importance of identifying clinical manifestations associated with Lyme disease and the use of tissue PCR to detect borrelial DNA in patients with these lesions, but characterized by negative serology for Borrelia.

  16. Pesticide-induced scleroderma and early intensive immunosuppressive treatment.

    Science.gov (United States)

    Sozeri, Betul; Gulez, Nesrin; Aksu, Guzide; Kutukculer, Necil; Akalın, Taner; Kandiloglu, Gulsen

    2012-01-01

    The authors report 2 children with generalized cutaneous sclerosis exposed to pesticides containing malathion and diniconazole. Treatment with immunosuppressives resulted in partial improvement in the cutaneous signs, particularly over the face, trunk, and proximal limbs. The considerable exposure to chemicals related with the initiation of symptoms and absence of organ involvement suggested a diagnosis of chemically induced scleroderma-like disorder. Although autoantibodies were negative, previously reported relevant associations of anti-kinetochore and anti-topoisomerase function of active ingredients-diniconazole and phosphorodithioate-and solvents of these pesticides are also discussed. Careful follow-up for systemic involvement is warranted, since these agents may have triggered systemic scleroderma in these patients. Elimination of chemical exposure of children is stressed.

  17. Juvenile Justice in Milwaukee

    Science.gov (United States)

    Williams, Gary L.; Greer, Lanetta

    2010-01-01

    Historically, there have been several attempts made to address issues surrounding juvenile delinquency. The Wisconsin Legislature outlines the objectives of the juvenile justice system in the Juvenile Justice Code in s. 939.01, ?to promote a juvenile justice system capable of dealing with the problem of juvenile delinquency, a system which will…

  18. A case of cutaneous scleroderma with primary sclerosing cholangitis

    Directory of Open Access Journals (Sweden)

    H P Nandeesh

    2014-01-01

    Full Text Available Sclerosing cholangitis comprises of a spectrum of cholestatic conditions that are characterized by patchy fibrosis, inflammation and destruction of intra hepatic and extrahepatic ducts. We report a case of a 42 year old woman who presented with darkening of skin with yellowish discolouration of the eyes. Clinical examination revealed icterus, taut skin with hepatosplenomegaly. Liver function tests showed a cholestatic picture. Skin biopsy showed features of cutaneous scleroderma. MRCP and Liver biopsy was suggestive of sclerosing cholangitis.

  19. Use of the international systemic scleroderma activity index

    Directory of Open Access Journals (Sweden)

    Maya Nikolayevna Starovoitova

    2013-01-01

    Full Text Available Up to now, it is difficult to determine systemic scleroderma (SSD activity because of the lack of validated tools to estimate changes in the pathological process. Attempts have been made to develop unified activity assessing methods for many years. The indices proposed by the European SSD Group are most popular today. This paper gives the results of using this index in a cohort of Russian patients.

  20. Predicting Disease Progression in Scleroderma with Skin and Blood Biomarkers

    Science.gov (United States)

    2016-12-01

    98) Prescribed by ANSI Std . Z39.18 SCLERODERMA RESEARCH PROGRAM  Predicting Disease Progression in Scleroderma with Skin and Blood Biomarkers Proposal...NW, Calgary, AB T2N 4N1, Canada. 20Division of Rheumatology, University of Minnesota, 420 Delaware Street SE, Minneapolis, MN 55455, USA. 21Instituto...844, 1720 2nd Ave S. Birmingham AL 35294-3408. USA qUniversity of Minnesota. Division of Rheumatic/Autoimmune Diseases. MCC 108, 420 Delaware St SE

  1. 硬皮病证治%Differentiating and Treating Scleroderma

    Institute of Scientific and Technical Information of China (English)

    周宝宽

    2011-01-01

    目的:总结周宝宽主任医师辨证论治硬皮病经验.方法:以医案形式阐述硬皮病的病因病机、证型、治法、方药.结果:硬皮病的病因病机为肺、牌、肾功能失调,营卫失和,腠理不密,风、寒、湿之邪乘虚内袭,正气为邪所阻,气血凝涩,经络阻隔,痹塞不通;或情志内伤,气血失和,运行受阻,复感外邪,阻滞经络,气滞血瘀.常见证型为寒湿阻滞、脾肾阳虚、血瘀经脉.结论:辨证论治硬皮病有较好疗效.%Objective:To summarize the experiences of Chief Physician Zhou Baokuan on the treatment of scleroderma based on syndrome differentiation. Methods; The etiology, pathogenesis, types, therapeutic methods and prescriptions of scleroderma were expounded by the way of medical cases. Result: The etiology and pathogenesis of scleroderma was imbalance of lung, spleen and kidney, disharmony of nutritive and defensive phases, and interspaces of skin and muscles being loose, combined with invasion of pathogenic wind, cold and dampness. And then vital Qi is restrained by the evil, Qi and blood were stagnated, meridians were blocked, or internal injury by emotions leads to disharmony of Qi and blood, and then circulation was restrained, combined with the invasion of exogenous evil, and the meridians were blocked, leading to Qi stagnation and blood stasis. The common types were blockage of damp cold, Yang deficiency of spleen and kidney, and blood stasis in vessels. Conclusion; The treatment of scleroderma based on syndrome differentiation is effective.

  2. Targeting the Nociceptin/Orphanin FQ Receptor for Scleroderma Therapy

    Science.gov (United States)

    2015-12-01

    the United States and accounts for an estimated $400 million in total annual medical costs. There is no treatment that stops or cures scleroderma...the project made an impact or are likely to make an impact on the base of knowledge, theory , and research in the principal disciplinary field(s) of the...that were involved with the project. Partner organizations may have provided financial or in-kind support, supplied facilities or equipment

  3. Juvenile xanthogranuloma.

    Science.gov (United States)

    Singh, R; Ghazali, W

    1992-05-01

    Juvenile xanthogranuloma is a benign cutaneous growth presenting as papules or nodules. It is characterized by an intradermal collection of lipid-laden macrophages and varying degrees of fibroblastic proliferation. We have recently observed two patients with xanthogranulomas: one was found to have a papular type and the second patient had multiple nodular growths. We present these cases, which should be considered in the differential diagnosis of skin nodules.

  4. Esclerose sistêmica e sarcoidose Scleroderma and sarcoidosis

    Directory of Open Access Journals (Sweden)

    Fernanda Guidolin

    2005-10-01

    Full Text Available Os autores descrevem o caso de uma paciente com esclerose sistêmica (ES - forma limitada - com comprometimento pulmonar tipo fibrose intersticial. Após sete anos sem acompanhamento, foram identificados gânglios mediastinais e esplenomegalia. A biópsia de linfonodos mostrou granuloma não caseoso sugestivo de sarcoidose. Estamos mostrando, neste caso, a associação de ES e sarcoidose, para chamar a atenção para esse fato e enfatizar que a sarcoidose deve ser lembrada no diagnóstico diferencial das complicações pulmonares da esclerodermia.The authors describe the case of a patient with limited scleroderma and interstitial lung disease. Follow-up was lost for seven years, when patient returned presenting nodular mediastinal enlargement and splenomegaly. Lymph node biopsy showed granulomatous lesions without caseum suggestive of sarcoidosis. This case is being presented to remind the association of scleroderma and sarcoidosis as a possible differential diagnosis of scleroderma pulmonary complications.

  5. Discovery or Extinction of New Scleroderma Species in Amazonia?

    Science.gov (United States)

    Baseia, Iuri G.; Silva, Bianca D. B.; Ishikawa, Noemia K.; Soares, João V. C.; França, Isadora F.; Ushijima, Shuji; Maekawa, Nitaro

    2016-01-01

    The Amazon Forest is a hotspot of biodiversity harboring an unknown number of undescribed taxa. Inventory studies are urgent, mainly in the areas most endangered by human activities such as extensive dam construction, where species could be in risk of extinction before being described and named. In 2015, intensive studies performed in a few locations in the Brazilian Amazon rainforest revealed three new species of the genus Scleroderma: S. anomalosporum, S. camassuense and S. duckei. The two first species were located in one of the many areas flooded by construction of hydroelectric dams throughout the Amazon; and the third in the Reserva Florestal Adolpho Ducke, a protected reverse by the INPA. The species were identified through morphology and molecular analyses of barcoding sequences (Internal Transcribed Spacer nrDNA). Scleroderma anomalosporum is characterized mainly by the smooth spores under LM in mature basidiomata (under SEM with small, unevenly distributed granules, a characteristic not observed in other species of the genus), the large size of the basidiomata, up to 120 mm diameter, and the stelliform dehiscence; S. camassuense mainly by the irregular to stellate dehiscence, the subreticulated spores and the bright sulfur-yellow colour, and Scleroderma duckei mainly by the verrucose exoperidium, stelliform dehiscence, and verrucose spores. Description, illustration and affinities with other species of the genus are provided. PMID:28002414

  6. Discovery or Extinction of New Scleroderma Species in Amazonia?

    Science.gov (United States)

    Baseia, Iuri G; Silva, Bianca D B; Ishikawa, Noemia K; Soares, João V C; França, Isadora F; Ushijima, Shuji; Maekawa, Nitaro; Martín, María P

    2016-01-01

    The Amazon Forest is a hotspot of biodiversity harboring an unknown number of undescribed taxa. Inventory studies are urgent, mainly in the areas most endangered by human activities such as extensive dam construction, where species could be in risk of extinction before being described and named. In 2015, intensive studies performed in a few locations in the Brazilian Amazon rainforest revealed three new species of the genus Scleroderma: S. anomalosporum, S. camassuense and S. duckei. The two first species were located in one of the many areas flooded by construction of hydroelectric dams throughout the Amazon; and the third in the Reserva Florestal Adolpho Ducke, a protected reverse by the INPA. The species were identified through morphology and molecular analyses of barcoding sequences (Internal Transcribed Spacer nrDNA). Scleroderma anomalosporum is characterized mainly by the smooth spores under LM in mature basidiomata (under SEM with small, unevenly distributed granules, a characteristic not observed in other species of the genus), the large size of the basidiomata, up to 120 mm diameter, and the stelliform dehiscence; S. camassuense mainly by the irregular to stellate dehiscence, the subreticulated spores and the bright sulfur-yellow colour, and Scleroderma duckei mainly by the verrucose exoperidium, stelliform dehiscence, and verrucose spores. Description, illustration and affinities with other species of the genus are provided.

  7. U3 snoRNP associates with fibrillarin a component of the scleroderma clumpy nucleolar domain

    DEFF Research Database (Denmark)

    Herrera-Esparza, Rafael; Kruse, Lars; von Essen, Marina

    2002-01-01

    Serum from patients with scleroderma recognizes the clumpy autoantigen. The present studies addressed the issue as to whether the clumpy nucleolar autoantigen recognized by scleroderma serum is fibrillarin-U3 snoRNP. Clones encoding for clumpy autoantigen were immunodetected from a lambdagt11 HeLa...

  8. Soluble intercellular adhesion molecule-1 (sICAM-1) and soluble interleukin-2 receptors (sIL-2R) in scleroderma skin

    DEFF Research Database (Denmark)

    Søndergaard, Klaus; Deleuran, Mette; Heickendorff, Lene

    1998-01-01

    In order to investigate whether soluble intercellular adhesion molecule-1 (sICAM-1) and soluble interleukin-2 receptors (sIL-2R) were present in scleroderma skin, and to compare their levels to concentrations measured in plasma and clinical parameters, we examined suction blister fluid and plasma...... from 13 patients with systemic sclerosis and 11 healthy volunteers. Suction blisters and biopsies were from the transition zone between normal skin and scleroderma, and uninvolved abdominal skin. The levels of sICAM-1 and sIL-2R were significantly increased in both plasma and suction blister fluid from...... systemic sclerosis patients compared with healthy volunteers. ICAM-1 was localized to vessels and perivascular mononuclear infiltrates by immunohistochemical methods. IL-2R was expressed by CD3-positive cells. The elevated levels of sICAM-1 and sIL-2R in suction blister fluid point towards activation...

  9. Successful Treatment of Hemifacial Myokymia and Dystonia Associated to Linear Scleroderma “En Coup de Sabre” with Repeated Botox Injections

    Directory of Open Access Journals (Sweden)

    Carlos A. Cañas

    2012-01-01

    Full Text Available Linear scleroderma “en coup de sabre” (LSCS is a form of localized scleroderma presents as band-like sclerotic lesions of the frontoparietal area. It has been reported in association with diverse neurological manifestations like seizures, migraine, neuromyotonia, dystonia and abnormalities in MRI and CT studies as cerebral atrophy, white matter lesions, intraparenchymal calcification, meningeocortical alterations, and skull atrophy. We describe a patient with LSCS associated with two abnormal movements: permanent myokimia of right masseter muscle and recurrent spasmodic retraction of right cigomatic and depressor labii inferioris muscles. He was initially treated with methotrexate and steroids without response, so later on he underwent repeated Botox injections with remarkable improvement.

  10. Soluble intercellular adhesion molecule-1 (sICAM-1) and soluble interleukin-2 receptors (sIL-2R) in scleroderma skin

    DEFF Research Database (Denmark)

    Søndergaard, Klaus; Deleuran, Mette; Heickendorff, Lene;

    1998-01-01

    In order to investigate whether soluble intercellular adhesion molecule-1 (sICAM-1) and soluble interleukin-2 receptors (sIL-2R) were present in scleroderma skin, and to compare their levels to concentrations measured in plasma and clinical parameters, we examined suction blister fluid and plasma...... from 13 patients with systemic sclerosis and 11 healthy volunteers. Suction blisters and biopsies were from the transition zone between normal skin and scleroderma, and uninvolved abdominal skin. The levels of sICAM-1 and sIL-2R were significantly increased in both plasma and suction blister fluid from...... systemic sclerosis patients compared with healthy volunteers. ICAM-1 was localized to vessels and perivascular mononuclear infiltrates by immunohistochemical methods. IL-2R was expressed by CD3-positive cells. The elevated levels of sICAM-1 and sIL-2R in suction blister fluid point towards activation...

  11. Dermatomiositis juvenil

    OpenAIRE

    Goldaracena, Pablo; Pérez, Federico

    2008-01-01

    La dermatomiositis juvenil (DMJ) es una enfermedad multi sistémica de etiología desconocida, caracterizada por una vasculitis que ocasiona una inflamación no supurativa del músculo estriado y lesiones cutáneas distintivas. La cobertura de los criterios de Bohan y Peter establece el diagnóstico: exantema patognomónico junto a debilidad muscular proximal simétrica, elevación sérica de enzimas musculares, s...

  12. Phase 4 paroxysmal AV block in a patient with scleroderma.

    Science.gov (United States)

    Butschek, Ross; Powell, Brian D; Littmann, Laszlo

    2013-01-01

    A 72-year-old man with limited cutaneous systemic scleroderma was hospitalized for two episodes of witnessed syncope. The baseline 12-lead electrocardiogram was normal but on telemetry there were numerous episodes of paroxysmal AV block with asystolic periods of up to 7.5 s duration. Analysis of the rhythm strips revealed phase 4 intra-His bundle block characterized by critical P-P intervals that triggered the AV block, and a narrow range of junctional escape to subsequent P wave intervals that were required to release the AV block. A dual chamber pacemaker was implanted. Copyright © 2013 Elsevier Inc. All rights reserved.

  13. Management of Raynaud Phenomenon and Digital Ulcers in Scleroderma.

    Science.gov (United States)

    Cappelli, Laura; Wigley, Fredrick M

    2015-08-01

    Raynaud phenomenon (RP) and associated digital ischemia can be among the most vexing clinical problems for patients with systemic sclerosis (scleroderma). Understanding the treatment approach to RP and associated ischemia and how to prevent digital ulcers is important for clinicians caring for these patients. This article reviews the management of RP and digital ischemic ulcers. The magnitude of the problem and pathophysiology of RP are first discussed, with an emphasis on recent advances in understanding of the disease process. Options for the practical pharmacologic and nonpharmacologic interventions for RP and digital ischemic ulcers are detailed.

  14. COMPLEX THERAPY FOR VASCULAR DISORDERS IN PATIENTS WITH SCLERODERMA SYSTEMATICA

    Directory of Open Access Journals (Sweden)

    M N Starovoitova

    2009-06-01

    Full Text Available Scleroderma systematica (SDS is a disease in which vascular diseases underlie the pathogenesis and presented by diverse clinical manifestations. Raynaud's syndrome and digital ulceration are the most common symptom of the diseases, which influences the quality of life in patients and requires continuous drug therapy. The paper discusses the recent European guidelines for the management of this manifestation of SDS. The proposed recommendations cannot unfortunately be realized in full measure now due to the unavailability of some drugs. The authors give their clinical experience with therapy for the vascular manifestations of SDS.

  15. COMPLEX THERAPY FOR VASCULAR DISORDERS IN PATIENTS WITH SCLERODERMA SYSTEMATICA

    Directory of Open Access Journals (Sweden)

    M N Starovoitova

    2009-01-01

    Full Text Available Scleroderma systematica (SDS is a disease in which vascular diseases underlie the pathogenesis and presented by diverse clinical manifestations. Raynaud's syndrome and digital ulceration are the most common symptom of the diseases, which influences the quality of life in patients and requires continuous drug therapy. The paper discusses the recent European guidelines for the management of this manifestation of SDS. The proposed recommendations cannot unfortunately be realized in full measure now due to the unavailability of some drugs. The authors give their clinical experience with therapy for the vascular manifestations of SDS.

  16. ANCA-associated vasculitis in scleroderma: a case series of fourteen patients

    Directory of Open Access Journals (Sweden)

    Kimberly P. Liang

    2011-01-01

    Full Text Available Antimyeloperoxidase (MPO, perinuclear antineutrophil cytoplasmic antibodies (pANCA, and/or clinically evident vasculitis in patients with scleroderma have been reported only rarely. The clinical significance and prognosis of ANCA-associated vasculitis in systemic sclerosis is uncertain. To report a case and identify the clinical characteristics of scleroderma patients with ANCA-associated vasculitis. Patients with both vasculitis and scleroderma occurring between 1976 to 2006 were identified using an electronic diagnostic index. These diagnoses were confirmed by retrospective review of complete medical records. Clinical features and outcomes recorded included age at vasculitis diagnosis, connective tissue disease (CTD features, type of scleroderma (limited or diffuse; ANCA serology, vasculitic organ system manifestations; and death. Fourteen cases of scleroderma patients with ANCA-associated and/or small vessel vasculitis were identified. The majority (71% were female, with mean age at vasculitis diagnosis 53 years. Seven patients (50% had overlap CTD features, and the majority (79% had limited variant of scleroderma. All of the 10 patients tested were MPO and pANCA positive. Seven patients (50% had glomerulonephritis, 11 (79% pulmonary involvement including 3 with pulmonary-renal syndrome, 6 skin purpura, and 5 mononeuritis multiplex and/or peripheral neuropathy. Six patients (43% died during followup to 2008. The presence of pANCA-associated small vessel vasculitis is a rarely reported complication of scleroderma. It occurs most commonly in women with limited scleroderma and most commonly includes pulmonary and/or renal involvement, including severe organ-threatening manifestations and death. Further studies are needed to clarify the role and clinical impact of ANCA in scleroderma patients with and without vasculitis.

  17. Gadd45b is an epigenetic regulator of juvenile social behavior and alters local pro-inflammatory cytokine production in the rodent amygdala.

    Science.gov (United States)

    Kigar, Stacey L; Chang, Liza; Auger, Anthony P

    2015-05-01

    Precise regulation of the epigenome during perinatal development is critical to the formation of species-typical behavior later in life. Recent data suggests that Gadd45b facilitates active DNA demethylation by recruiting proteins involved in base excision repair (BER), which will catalyze substitution of 5-methyl-cytosine (5mC) for an unmodified cytosine. While a role for Gadd45b has been implicated in both hippocampal and amygdalar learning tasks, to the best of our knowledge, no study has been done investigating the involvement of Gadd45b in neurodevelopmental programming of social behavior. To address this, we used a targeted siRNA delivery approach to transiently knock down Gadd45b expression in the neonatal rat amygdala. We chose to examine social behavior in the juvenile period, as social deficits associated with neurodevelopmental disorders tend to emerge in humans at an equivalent age. We find that neonatal Gadd45b knock-down results in altered juvenile social behavior and reduced expression of several genes implicated in psychiatric disorders, including methyl-CpG-binding protein 2 (MeCP2), Reelin, and brain derived neurotrophic factor (BDNF). We furthermore report a novel role for Gadd45b in the programmed expression of α2-adrenoceptor (Adra2a). Consistent with Gadd45b's role in the periphery, we also observed changes in the expression of pro-inflammatory cytokines interleukin-6 (Il-6) and interleukin-1beta (Il-1beta) in the amygdala, which could potentially mediate or exacerbate effects of Gadd45b knockdown on the organization of social behavior. These data suggest a prominent role for Gadd45b in the epigenetic programming of complex juvenile social interactions, and may provide insight into the etiology of juvenile behavioral disorders such as ADHD, autism, and/or schizophrenia. Copyright © 2015 Elsevier Inc. All rights reserved.

  18. "Scleroderma linearis: hemiatrophia faciei progressiva (Parry-Romberg syndrom without any changes in CNS and linear scleroderma "en coup de sabre" with CNS tumor

    Directory of Open Access Journals (Sweden)

    Brzezińska-Wcisło Ligia

    2009-07-01

    Full Text Available Abstract Background Hemifacial atrophy (Parry-Romberg syndrome is a relatively rare disease. The etiology of the disease is not clear. Some authors postulate its relation with limited scleroderma linearis. Linear scleroderma "en coup de sabre" is characterized by clinical presence of most commonly one-sided linear syndrome. In a number of patients, neurological affection is the medium of the disease. The treatment of both scleroderma varieties is similar to the treatment of limited systemic sclerosis. Case presentation We present two cases of a disease: a case of a 49-year-old woman with a typical image of hemifacial atrophy, without any changes of the nervous system and a case of a 33-year-old patient with an "en coup de sabre" scleroderma and with CNS tumor. Conclusion We described typical cases of a rare diseases, hemifacial atrophy and "en coup de sabre" scleroderma. In the patient diagnosed with Parry-Romberg syndrome, with Borrelia burgdoferi infection and with minor neurological symptoms, despite a four-year case history, there was a lack of proper diagnosis and treatment. In the second patient only skin changes without any neurological symptoms could be observed and only a precise neurological diagnosis revealed the presence of CNS tumor.

  19. New directions for patient-centred care in scleroderma: the Scleroderma Patient-centred Intervention Network (SPIN)

    Science.gov (United States)

    Thombs, Brett D.; Jewett, Lisa R.; Assassi, Shervin; Baron, Murray; Bartlett, Susan J.; Costa Maia, Angela; El-Baalbaki, Ghassan; Furst, Daniel E.; Gottesman, Karen; Haythornthwaite, Jennifer A.; Hudson, Marie; Ann Impens, PhD; Korner, Annett; Leite, Catarina; Mayes, Maureen D.; Malcarne, Vanessa L.; Motivala, Sarosh J.; Mouthon, Luc; Nielson, Warren R.; Plante, Diane; Poiraudeau, Serge; Poole, Janet L.; Pope, Janet; Sauve, Maureen; Steele, Russell J.; Suarez-Almazor, Maria E.; Taillefer, Suzanne; van den Ende, Cornelia H.; Erin Arthurs, BSc; Bassel, Marielle; Delisle, Vanessa; Milette, Katherine; Leavens, Allison; Razykov, Ilya; Khanna, Dinesh

    2014-01-01

    Systemic sclerosis (SSc), or scleroderma, is a chronic multisystem autoimmune disorder characterised by thickening and fibrosis of the skin and by the involvement of internal organs such as the lungs, kidneys, gastrointestinal tract, and heart. Because there is no cure, feasibly-implemented and easily accessible evidence-based interventions to improve health-related quality of life (HRQoL) are needed. Due to a lack of evidence, however, specific recommendations have not been made regarding non-pharmacological interventions (e.g. behavioural/psychological, educational, physical/occupational therapy) to improve HRQoL in SSc. The Scleroderma Patient-centred Intervention Network (SPIN) was recently organised to address this gap. SPIN is comprised of patient representatives, clinicians, and researchers from Canada, the USA, and Europe. The goal of SPIN, as described in this article, is to develop, test, and disseminate a set of accessible interventions designed to complement standard care in order to improve HRQoL outcomes in SSc. PMID:22244687

  20. Emergency Surgery in a Patient with Scleroderma - Anaesthetic Challenges: A Case Report

    Directory of Open Access Journals (Sweden)

    Teena Bansal

    2013-06-01

    Full Text Available Scleroderma (progressive systemic sclerosis is a multisystem disease involving the skin, airway, musculoskeletal, gastrointestinal, pulmonary, renal and cardiac systems that can pose a significant challenge for the anaesthetist. The multisystem involvement of scleroderma can impact on every aspect of anaesthetic care especially airway management. There are no specific contraindications to the use of any type of anaesthesia, although the selection must be guided by identification of organ dysfunction. The anaesthetist must be aware of the organs involved, the severity of the disease and the associated anaesthetic considerations and potential risks in order to safely & skilfully manage the patient with scleroderma. We hereby present a case report of a patient with scleroderma for emergency orbital decompression because of orbital cellulitis.

  1. INTERSTITIAL LUNG-DISEASE AND MYOSITIS IN A PATIENT WITH SIMULTANEOUSLY OCCURRING SARCOIDOSIS AND SCLERODERMA

    NARCIS (Netherlands)

    GROEN, H; POSTMA, DS; KALLENBERG, CGM

    1993-01-01

    A patient initially presented with sarcoidosis in combination with myositis of sarcoid origin and Raynaud's phenomenon. During the course of his disease, he additionally developed scleroderma. Bronchoalveolar lavage, performed because of increase of interstitial markings in the presence of enlarged

  2. Lack of increased expression of cell surface markers for circulating fibrocyte progenitors in limited scleroderma.

    Science.gov (United States)

    Russo, R; Medbury, H; Guiffre, A; Englert, H; Manolios, N

    2007-07-01

    The aetiology and pathogenesis of scleroderma is incompletely understood. Recently, a cell called the fibrocyte has been shown to be derived from circulating monocytes with the ability to produce collagen. The aim of this study was to evaluate differences in the cell surface characteristics of circulating fibrocyte progenitors (monocytes) in patients with limited scleroderma compared to controls. A case-control study was performed in eight patients with limited scleroderma, which were matched with eight controls. Three-colour flow cytometry was used to assess the relative expression of cell surface markers. Statistical analysis then compared the relative expression between the two groups. In this preliminary study, there were no significant differences in the expression of circulating monocyte surface molecules involved with cell transformation, function, or migration presumed to give rise to fibrocytes, in a population of patients with limited scleroderma. Various explanations for the results are discussed.

  3. Mortality and causes of death of 344 Danish patients with systemic sclerosis (scleroderma)

    DEFF Research Database (Denmark)

    Jacobsen, Søren; Halberg, P; Ullman, S

    1998-01-01

    To determine survival, mortality and causes of death in Danish patients with systemic sclerosis (scleroderma), and to analyse how these parameters are influenced by demographic variables and the extent of skin involvement.......To determine survival, mortality and causes of death in Danish patients with systemic sclerosis (scleroderma), and to analyse how these parameters are influenced by demographic variables and the extent of skin involvement....

  4. Autoantibodies against nuclear, nucleolar, and mitochondrial antigens in systemic sclerosis (scleroderma).

    Science.gov (United States)

    Reimer, G

    1990-02-01

    One of the most characteristic serologic features of systemic sclerosis (scleroderma) is the occurrence of autoantibodies against nuclear and most notably against nucleolar antigens. This humoral autoimmune response is one of best studied immunologic phenomena in scleroderma. Detailed molecular information on the structure and function, as well as on reactive epitopes of autoantigens targeted by specific serum antibodies, has been revealed by clinical, immunologic, and biochemic studies in several laboratories. Autoantigens such as DNA topoisomerase I (Scl-70), centromere proteins, RNA polymerase I, U3 RNP-associated fibrillarin, PM-Scl, and 7-2 RNP antigens were shown to be specific targets of scleroderma patients and were observed to have clinical correlates within the scleroderma disease spectrum. Therefore, autoantibodies in scleroderma are not only valuable diagnostic tools but also prognosticators of the disease. Although autoantibodies in scleroderma do not appear to play a pathogenetic role in the disease process, the knowledge of the structure and function of their reactive antigens may help in answering questions concerning the etiology of the disease.

  5. Lung transplantation in patients with scleroderma: case series, review of the literature, and criteria for transplantation.

    Science.gov (United States)

    Shitrit, David; Amital, Anat; Peled, Nir; Raviv, Yael; Medalion, Benjamin; Saute, Milton; Kramer, Mordechai R

    2009-01-01

    The use of lung transplantation (LTX) to treat respiratory failure because of scleroderma is controversial. We present our experience, review the current literature, and suggest specific criteria for LTX in scleroderma. Of the 174 patients who underwent LTX at our center, seven (4%) had scleroderma-associated respiratory failure. A MEDLINE search of the English literature was performed for studies of LTX in patients with scleroderma between 1986 and 2006. A Kaplan-Meier survival curve was calculated over the time of the studies. The MEDLINE search yielded one large review and four small case series. The small case series were included in the review. The review and our series yield a total of 54 patients. Mean patient age was 47.1 yr; 59.3% were female. Pre-operative lung data were available for 24 patients: 22 (92%) had pulmonary fibrosis and 17 (71%) had pulmonary hypertension. Most patients (69%) underwent single-lung transplantation. Mean forced expiratory volume at one s after LTX was 67% (range 56-87%). There was no difference in infection and rejection rates between the patients with scleroderma and other LTX recipients. The two- and five-yr survival rates were 72% and 55%, respectively. LTX is a valid option in well-selected patients with scleroderma and pulmonary fibrosis, yielding good pulmonary function and acceptable morbidity and mortality.

  6. A survey of dentists' knowledge and attitudes with respect to the treatment of scleroderma patients.

    Science.gov (United States)

    Leader, David; Papas, Athena; Finkelman, Matthew

    2014-06-01

    Scleroderma or systemic sclerosis causes dry mouth, a major risk factor for tooth decay, and shrinks the mouth opening, complicating care. A 2011 survey determined that 28% of systemic sclerosis patients have difficulty finding dentists prepared to treat them, and 63% do not recommend their current dentist to other systemic sclerosis patients. We use a survey to gauge dentists' knowledge and attitudes regarding the care of scleroderma patients. We conducted an Internet-based survey of all 4465 members of the Massachusetts Dental Society to determine their knowledge and attitudes of treating systemic sclerosis patients. Data were analyzed using SPSS and Qualtrics research suite. Surveys were accessed by 351 dentists and completed by 269. Responses were primarily from Eastern Massachusetts (80%), but represented the Boston area less than expected. Most dentists believed they have an ethical responsibility to treat patients who have scleroderma (93%). More than half of dentists believed that in not knowing about systemic sclerosis they might harm a patient (51%). If contacted by a patient who has scleroderma, 50% of dentists would gather information on the disease or the patient's condition. Dentists who felt prepared (71%) were more likely to correctly answer questions related to diagnosis and classification of scleroderma than those who felt unprepared (P = 0.004, Mann-Whitney U test). Results indicate the potential value of creating a health communication effort targeting oral health providers to improve scleroderma patient satisfaction and access to care.

  7. What Is Juvenile Arthritis?

    Science.gov (United States)

    ... Analgesics for Osteoarthritis (Report from AHRQ) Joint Replacement Surgery: Health Information Basics for You and Your Family NIH Pediatric Rheumatology Clinic Health Information Juvenile Arthritis Find a Clinical Trial Journal Articles Juvenile Arthritis PDF Version Size: 123 KB ...

  8. Juvenile Delinquency: An Introduction

    Science.gov (United States)

    Smith, Carolyn A.

    2008-01-01

    Juvenile Delinquency is a term which is often inaccurately used. This article clarifies definitions, looks at prevalence, and explores the relationship between juvenile delinquency and mental health. Throughout, differences between males and females are explored. (Contains 1 table.)

  9. Parenting and juvenile delinquency

    NARCIS (Netherlands)

    Hoeve, Machteld

    2008-01-01

    Juvenile delinquency is a noteworthy problem. This thesis addressed the association between parenting and juvenile delinquency by analyzing the concepts of parenting adopted in family research in relation to criminological concepts and measures of delinquent behavior. Four studies were conducted.

  10. Aggressive juvenile mandibular fibromatosis.

    Science.gov (United States)

    Ivanov, Georgi P; Atanasov, Dimitar T; Anavi, Beniamin L

    2013-01-01

    Aggressive juvenile fibromatosis of the jawbones is a rare tumor presenting as infiltrative mass with unpredictable evolution. We report herein a 17-year-old student with a 6-month history of radiologically proven resorption of a part of the mandible, lingual displacement of tooth 34 and malocclusion. Alveolar ridge resorption and three dark-brown foci in the bone were seen after the tooth was extracted. Histological study showed the tumor tissue to have a bundle-like structure; immunohistochemically it was positive for vimentin, smooth muscle actin, beta-catenin, Ki-67 (5%), and negative for desmin and cytokeratin 34bE12. The golden standard in the diagnostics of desmoid fibromatoses is the nuclear or membrane expression of beta-catenin, which is found in 90% of the cases. Differential diagnosis include mandibular fibroma, well-differentiated fibrosarcoma, fibrosing histiocytoma, and infiltration from adjacent soft-tissue tumor. Aggressive juvenile fibromatosis should be managed by radical excision. Local recurrences are not rare, but metastases do not develop. In rare cases this type of fibromatosis has been known to regress spontaneously. Aggressive fibromatosis is a diagnostic challenge, since it remains in the grey zone between benign and malignant lesions of the oral cavity.

  11. Juvenile polyposis syndrome

    NARCIS (Netherlands)

    L.A.A. Brosens; D. Langeveld; W.A. van Hattem; F.M. Giardiello; G.J.A. Offerhaus

    2011-01-01

    Juvenile polyposis syndrome is a rare autosomal dominant syndrome characterized by multiple distinct juvenile polyps in the gastrointestinal tract and an increased risk of colorectal cancer. The cumulative life-time risk of colorectal cancer is 39% and the relative risk is 34. Juvenile polyps have a

  12. Localization of a locus for juvenile myoclonic epilepsy on chromosome 6p11-21.2 and evidence for genetic heterogeneity

    Energy Technology Data Exchange (ETDEWEB)

    Liu, A.W.; Delgado-Escueta, A.V. [Univ. of California, Los Angeles, CA (United States)]|[West Los Angeles VA Medical Center, CA (United States); Alonso, V.M.E. [Instituto Nacional de Neurologia Y Neurocirugia, Mexico City (Mexico)

    1994-09-01

    Juvenile myoclonic epilepsy (JME) is a common form of primary idiopathic generalized epilepsy characterized by myoclonias, tonic-clonic or clonic tonic-clonic convulsions and absences. Ictal electroencephalograms (EEGs) show high amplitude multispikes folowed by slow waves and interictal EEGs manifest 3.5-6 Hz diffuse multispike wave complexes. JME affected about 7-10% of patients with epilepsies and its onset peaks between 13-15 years of age. We recently mapped a JME locus on chromosome 6p21.1-6p11 by linkage analysis of one relatively large JME family from Los Angeles and Belize. Assuming autosomal dominant inheritance with 70% penetrance, pairwise analyses tightly linked JME to D6S257 (Z = 3.67), D6S428 (Z = 3.08) and D6S272 (Z = 3.56) at {theta} = 0, m = f. Recombination and multipoints linkage analysis also suggested a locus is between markers D6S257 and D6S272. We then screened three relatively larger Mexican JME pedigrees with D6S257, D6S272, D6S282, TNF, D6S276, D6S273, D6S105 and F13A1 on chromosome 6p. Assuming autosomal dominant inheritance with incomplete penetrance, linkage to chromosome 6p DNA markers are excluded. Our findings underline the genetic heterogeneity of juvenile myoclonic epilepsy.

  13. Prospects for using mycophenolate mofetil in diffuse scleroderma

    Directory of Open Access Journals (Sweden)

    L P Ananyeva

    2009-01-01

    Full Text Available The paper considers the immunosuppressive properties of micophenolate mofetil (MMF and results of its use in graft rejection and rheumatic diseases. It also provides evidence suggesting the suppression of overfibrosis with the use of MMF, as well as the results of the experiments strongly confirming its antifibrotic properties. The author analyzes the results of clinical trials of MMF used in lung interstitial injury in patients with diffuse scleroderma, notes not only improvement or stabilization of lung functional tests, but also the optimization of prognosis (as evidenced by retrospective observations. Of interest are the experimental data demonstrating the suppression of pulmonary hypertension due to the antiproliferative and anti-inflammatory effects of MMF, which are indicative of the potential abilities of the agent to treat pulmonary hypertension.

  14. Juveniles on trial.

    Science.gov (United States)

    Quinn, Kathleen M

    2002-10-01

    This article describes common forensic evaluations requested of juvenile court mental health evaluators. There has been a legal shift toward criminalization of juvenile court, with a greater emphasis on rights, abandonment of the rehabilitative model, and greater movement of adolescents into the adult criminal court. A resulting shift has been the redefinition of juvenile court forensic evaluations toward the specificity of adult forensic work. The challenge for evaluators is to refine their knowledge of the forensic standards and bring knowledge of development, assessment, and diagnosis in juveniles and interview techniques appropriate to juveniles to improve the evaluation and forensic reports.

  15. Successful Treatment with Bosentan of Lower Extremity Ulcers in a Scleroderma Patient

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    Alix Naert

    2013-01-01

    Full Text Available Digital ulcers are a well-known problem in patients with systemic sclerosis. Lower extremity ulcers are less prevalent but are also a challenging and underestimated complication of the disease causing important pain and morbidity. Bosentan, an oral dual endothelin receptor antagonist, has been shown to be effective in preventing digital ulcers in patients with systemic sclerosis. A few recent observations showed the efficacy of bosentan for accelerating the healing of nondigital ulcers in scleroderma patients. This report deals with a 48-year-old patient with systemic sclerosis who developed painful ulcers on the left ankle and hallux. The ulcers were refractory to a combination of vasodilator therapy with a calcium antagonist and several courses of intravenous prostanoids, low molecular weight heparin, aspirin, simvastatin, and intensive local treatment. Bosentan treatment showed spectacular healing of the ulcers already after 4 months of therapy. This case supports the previous few observations of accelerating wound healing of lower extremity ulcers in systemic sclerosis patients with bosentan treatment.

  16. Juvenile polyposis syndrome

    Institute of Scientific and Technical Information of China (English)

    Lodewijk AA Brosens; Danielle Langeveld; W Arnout van Hattem; Francis M Giardiello; G Johan A Offerhaus

    2011-01-01

    Juvenile polyposis syndrome is a rare autosomal dominant syndrome characterized by multiple distinct juvenile polyps in the gastrointestinal tract and an increased risk of colorectal cancer.The cumulative life-time risk of colorectal cancer is 39% and the relative risk is 34.Juvenile polyps have a distinctive histology characterized by an abundance of edematous lamina propria with inflammatory cells and cystically dilated glands lined by cuboidal to columnar epithelium with reactive changes.Clinically, juvenile polyposis syndrome is defined by the presence of 5 or more juvenile polyps in the colorectum,juvenile polyps throughout the gastrointestinal tract or any number of juvenile polyps and a positive family history of juvenile polyposis.In about 50%-60% of patients diagnosed with juvenile polyposis syndrome a germline mutation in the SMAD4 or BMPR1A gene is found.Both genes play a role in the BMP/TGF-beta signalling pathway.It has been suggested that cancer in juvenile polyposis may develop through the so-alled "landscaper mechanism" where an abnormal stromal environment leads to neoplastic transformation of the adjacent epithelium and in the end invasive carcinoma.Recognition of this rare disorder is important for patients and their families with regard to treatment,follow-up and screening of at risk individuals.Each clinician confronted with the diagnosis of a juvenile polyp should therefore consider the possibility of juvenile polyposis syndrome.In addition, juvenile polyposis syndrome provides a unique model to study colorectal cancer pathogenesis in general and gives insight in the molecular genetic basis of cancer. This review discusses clinical manifestations, genetics, pathogenesis and management of juvenile polyposis syndrome.

  17. Management of cutaneous discomfort in patients with scleroderma: a clinical trial

    Directory of Open Access Journals (Sweden)

    M. Muratore

    2013-12-01

    Full Text Available Systemic sclerosis (scleroderma is a disease of unknown cause, the hallmark of which is induration of the skin. This bad condition of the skin influences negatively the quality of life of patients with scleroderma. The aim of the study was to verify the efficacy of two formulations, specifically designed to wash, moisturize and soothe the scleroderma skin. An independent, randomized, double blind, controlled trial was conducted in the Department of Rheumatology of “A. Galateo” Hospital in San Cesario di Lecce. Forty-six women affected by scleroderma, and treated with Iloprost every month, were divided into two groups: group 1 followed a specific treatment with cleansing formulation only, group 2 followed a combined treatment with the cleansing solution and the moisturizing solution. In addition, a third group was evaluated: 14 women, who did not undergo intravenous Iloprost therapy, were treated simultaneously with the cleansing formulation and the moisturizing formulation. The three treatments lasted for 4 weeks. Reduction in trans epidermal water loss (TEWL, increase in moisturization of the stratum corneum, reduction in Skin Score and improvement in quality of life were assessed. Very significant improvement in quality of life occurred in each group. Group 2 obtained very significant improvement in hydration and reduction in skin score and TEWL. The study showed that the daily use of both formulations proved to be effective in washing, hydrating and soothing the skin of patients with scleroderma, especially in association with Iloprost therapy.

  18. Understanding coping strategies among people living with scleroderma: a focus group study.

    Science.gov (United States)

    Gumuchian, Stephanie T; Peláez, Sandra; Delisle, Vanessa C; Carrier, Marie-Eve; Jewett, Lisa R; El-Baalbaki, Ghassan; Fortune, Catherine; Hudson, Marie; Körner, Annett; Kwakkenbos, Linda; Bartlett, Susan J; Thombs, Brett D

    2017-08-17

    Systemic sclerosis or scleroderma is a chronic, rare connective tissue disease with negative physical and psychological implications. Coping strategies used by scleroderma patients have not been studied in-depth. The objective of the present study was to gain a greater understanding of the coping strategies employed by people living with scleroderma. Three semi-structured focus group discussions were conducted with a total of 22 people with scleroderma. Interviews were recorded, transcribed, and analyzed using content analysis. Coping strategies discussed were analyzed through Lazarus and Folkman's theoretical model of coping, including: (1) problem-focused, (2) emotion-focused, and (3) meaning-focused coping. Participants reported using a combination of problem-focused (e.g., professional help; seeking disease-related information), emotion-focused (e.g., social support; adaptive distraction techniques), and meaning-focused coping strategies (e.g., benefit finding; goal reappraisal) to help them to cope with and manage their disease. However, many patients reported having difficulty in accessing support services. Scleroderma patients use similar coping strategies as patients with more common diseases, but they may not have access to the same level of support services. Accessible interventions, including self-management programs, aimed at improving problem- and emotion-focused coping are needed. Further, increased access to support groups may provide patients with opportunities to obtain social support and enhance coping.

  19. Crecimiento somático y relación ARN/ADN en estadios juveniles de Eucinostomus argenteus (Pisces: Gerreidae en dos localidades del Caribe de Venezuela Somatic growth and RNA/DNA rate of Eucinostomus argenteus (Pisces: Gerreidae juveniles stages at two localities of the Venezuelan

    Directory of Open Access Journals (Sweden)

    Ana Teresa Herrera-Reveles

    2012-03-01

    Full Text Available Con la finalidad de evaluar la asociación de índices de crecimiento en estadios tempranos de peces marinos, se estimó la tasa de crecimiento somático y las condiciones fisiológicas de Eucinostomus argenteus en dos zonas del nor-oriente venezolano: Bahía de Mochima y Golfo de Cariaco. La edad y el crecimiento fueron estimados basados en análisis de otolitos sagitta. Las condiciones fisiológicas fueron evaluadas por medio de las concentraciones de proteínas y la relación ARN/ADN, empleando técnicas espectofotométricas y fluorométricas sobre tejido muscular. Las relaciones entre tallas con la edad y el diámetro de los otolitos resultaron positivas, significativas y ajustadas a un modelo de regresión lineal. Los valores de la tasa de crecimiento reciente oscilaron entre 0.178 y 0.418mm día-1, la tasa de crecimiento retrocalculado varió entre 0.295 y 0.393mm día-1, y la tasa ARN/ADN osciló entre 1.65 y 6.97. No se registraron diferencias entre las zonas de estudio, sin embargo se reportaron diferencias entre localidades. A pesar de no encontrarse correlación entre la tasa de crecimiento y la relación ARN/ADN, los valores reportados sugieren crecimiento positivo de los individuos silvestres en las localidades evaluadas. No obstante, ciertas localidades mostraron valores que indican pobres condiciones nutricionales, pudiendo afectarse a futuro otras tasas vitales.Somatic growth and RNA/DNA rate of Eucinostomus argenteus (Pisces: Gerreidae juveniles stages at two localities of the Venezuelan Caribbean. In order to evaluate the association among growth indices of marine fishes at early life stages, the somatic growth rate and physiological conditions of Eucinostomus argenteus were estimated at two Venezuelan North-East zones: Mochima Bay and Cariaco Gulf. The age and somatic growth rate were estimated based on daily growth increments in sagitta otoliths. The physiological conditions were evaluated with proteins concentrations and RNA

  20. Effects of connective tissue growth factor and collagen type Ⅰ scleroderma

    Institute of Scientific and Technical Information of China (English)

    Xiaoning Yan; Jie Feng; Bingjun Shi

    2007-01-01

    Objective: To investigate the effects of connective tissue growth factor(CTGF) and collagen type Ⅰ(COL-I) on the pathogenesis of scleroderma and explore the relationship between the level of COL-I and CTGF. Methods: 12 mice model of scleroderma was established by the injection of Bleomycin. The level of CTGF and COL-I were detected by immunohistochemical method. The relationship was analyzed between CTGF and COL-I level. As control group, 12 healthy mice were selected. Results: The levels of CTGF and COL-I in sclerotic models were higher than in normal controls (P < 0.05). It was found that there was a correlation between the level of CTGF and COL-I. Conclusion: CTGF and COL-I played an important role in the hardening process of the skin lesions of the mice model, which may be involved in the pathogenesis of scleroderma.

  1. Evaluation of clinical symptoms of patients with scleroderma according to the onset time1

    Directory of Open Access Journals (Sweden)

    Khalvat A

    2000-07-01

    Full Text Available Systemic sclerosis is a generalized disorder of connective tissue, in which the pattern of disease extent, progression and outcome is heterogenous. To determine clinical features, disease extent and progression, we studied our patients in two phases of disease; early (the first 3 years and late phases (after 6 years of disease. 19 patients had diffuse cutaneous and 34 patients had limited cutaneous scleroderma. In patients with diffuse cutaneous scleroderma, disease progression has occurred mostly in the early phase of disease, but in patients with limited cutaneous scleroderma, disease progression was slow and incidious, so disease progression has occurred mostly in the late phase of the disease, thus raynaud's phenomenon, telangiectasia, hyperpigmentation and esophagitis were observed more in the late phase of the disease (statistically significant. In comparison of two groups, early and extensive organ involvement was observed in patients with diffuse cutaneous sclerodema.

  2. Evaluation of clinical symptoms of patients with scleroderma according to the onset time

    Directory of Open Access Journals (Sweden)

    Gharibduost F

    2001-07-01

    Full Text Available Systemic sclerosis is a generalized disorder of connective tissue, in which the pattern of disease extent, progression and outcome is heterogenous. To determine clinical features, disease extent and progression, we studied our patients in two phases of disease; early (the first 3 years and late phases (after 6 years of disease. 19 patients had diffuse cutaneous and 34 patients had limited cutaneous scleroderma. In patients with diffuse cutaneous scleroderma, disease progression has occurred mostly in the early phase of disease, but in patients with limited cutaneous scleroderma, disease progression was slow and incidious, so disease progression has occurred mostly in the late phase of the disease, thus raynaud's phenomenon, telangiectasia, hyperpigmentation and esophagitis were observed more in the late phase of the disease (statistically significant. In comparison of two groups, early and extensive organ involvement was observed in patients with diffuse cutaneous sclerodema.

  3. Hippocampal atrophy and developmental regression as first sign of linear scleroderma "en coup de sabre".

    Science.gov (United States)

    Verhelst, Helene E; Beele, Hilde; Joos, Rik; Vanneuville, Benedicte; Van Coster, Rudy N

    2008-11-01

    An 8-year-old girl with linear scleroderma "en coup de sabre" is reported who, at preschool age, presented with intractable simple partial seizures more than 1 year before skin lesions were first noticed. MRI revealed hippocampal atrophy, controlaterally to the seizures and ipsilaterally to the skin lesions. In the following months, a mental and motor regression was noticed. Cerebral CT scan showed multiple foci of calcifications in the affected hemisphere. In previously reported patients the skin lesions preceded the neurological signs. To the best of our knowledge, hippocampal atrophy was not earlier reported as presenting symptom of linear scleroderma. Linear scleroderma should be included in the differential diagnosis in patients with unilateral hippocampal atrophy even when the typical skin lesions are not present.

  4. An unusual cause of acute kidney injury due to oxalate nephropathy in systemic scleroderma.

    Science.gov (United States)

    Mascio, Heather M; Joya, Christie A; Plasse, Richard A; Baker, Thomas P; Flessner, Michael F; Nee, Robert

    2015-08-01

    Oxalate nephropathy is an uncommon cause of acute kidney injury. Far rarer is its association with scleroderma, with only one other published case report in the literature. We report a case of a 75-year-old African-American female with a history of systemic scleroderma manifested by chronic pseudo-obstruction and small intestinal bacterial overgrowth (SIBO) treated with rifaximin, who presented with acute kidney injury with normal blood pressure. A renal biopsy demonstrated extensive acute tubular injury with numerous intratubular birefringent crystals, consistent with oxalate nephropathy. We hypothesize that her recent treatment with rifaximin for SIBO and decreased intestinal transit time in pseudo-obstruction may have significantly increased intestinal oxalate absorption, leading to acute kidney injury. Oxalate nephropathy should be considered in the differential diagnosis of acute kidney injury in scleroderma with normotension, and subsequent evaluation should be focused on bowel function to include alterations in gut flora due to antibiotic administration.

  5. Digital ulcers in a cohort of 333 scleroderma patients

    Directory of Open Access Journals (Sweden)

    A. Ruffatti

    2011-09-01

    Full Text Available Digital ulcers (DU at the hands are one of the more frequent and severe complications in systemic sclerosis. Data on their prevalence and distribution in the different subsets of disease are variable in the literature. We studied the frequency of DU in a cohort of 333 scleroderma patients followed in the last 10 years in our Unit. DU have been recorded in 133 patients (39,9%, more frequently in males, in patients with cutaneous diffuse form of disease and in patients with anti-Scl70 ANA specificity. Complications of DU have been observed in 12,3% of cases. Surgery of the hands has been required in 8,7% of patients. The more effective treatment of DU are i.v. prostanoids, performed usually in day hospital, with high costs for the National Health Service. Recently the efficacy of bosentan, an oral receptor antagonist of endothelin, has been demonstrated, thus opening new perspectives in the treatment of DU in systemic sclerosis.

  6. NEW CLASSIFICATION CRITERIA FOR SCLERODERMA SYSTEMATICA (a lecture

    Directory of Open Access Journals (Sweden)

    Lidia Petrovna Ananyeva

    2013-01-01

    Full Text Available In 1980, the American College of Rheumatology proposed the first criteria as tentative for the diagnosis of scleroderma systematica (SDS. These criteria were aimed at revealing the comprehensive picture of mainly the diffuse form of the disease. They could not timely make a diagnosis in the low manifestive forms of the disease or virtually reveal its early stages that were most therapeutically promising. The review traces the evolution of approaches to diagnosing SDS and considers the novel classification criteria elaborated to select patients for epidemiological surveys and clinical trials. They include 8 parameters, each having scores. Skin thickening on both hands above the metacarpophalangeal articulations was highest (9 scores. The clinical variants of skin thickening on the fingers as its swelling (2 scores or sclerodactyly (4 scores are estimated from the maximum scores, as digital ischemia (sores, 2 scores; cicatricles, 3 scores. Teleangiectasias and characteristic capillaroscopic changes, as well as leading lung injury (pulmonary hypertension and/or interstitial lung disease are rated as 2 scores each. Three scores are added to the total amount when Raynaud's syndrome is present and SDS-specific (anti-Scl-70, anti-centromere, and anti- RNA polymerase III autoantibodies are detected. The patients gaining a total of 9 scores or more are classified as having definite SDS. Testing the novel criteria on a validation sample of 405 people showed that their sensitivity and specificity were 91 and 92%, respectively.

  7. Children in Custody: Public Juvenile Facilities, 1985. Bureau of Justice Statistics Bulletin.

    Science.gov (United States)

    Sickmund, Melissa; Baunach, Phyllis Jo

    A total of 1,040 publicly operated state and local juvenile detention, correction, and shelter facilities held 49,322 juvenile residents on February 1, 1985, an increase of 1% from the previous year. About 93% of the juveniles were accused of, or had been convicted for, acts which would be criminal offenses if committed by adults. Most of the rest…

  8. Juvenile giant fibroadenoma

    Directory of Open Access Journals (Sweden)

    Vipul Yagnik

    2011-07-01

    Full Text Available Fibroadenomas are benign solid tumor associated with aberration of normal lobular development. Juvenile giant fibroadenoma is usually single and >5 cm in size /or >500 gms in weight. Important differential diagnoses are: phyllodes tumor and juvenile gigantomastia. Simple excision is the treatment of choice.

  9. Renewing Juvenile Justice

    Science.gov (United States)

    Macallair, Daniel; Males, Mike; Enty, Dinky Manek; Vinakor, Natasha

    2011-01-01

    The Center on Juvenile and Criminal Justice (CJCJ) was commissioned by Sierra Health Foundation to critically examine California's juvenile justice system and consider the potential role of foundations in promoting systemic reform. The information gathered by CJCJ researchers for this report suggests that foundations can perform a key leadership…

  10. Philanthropist in Juvenile Reformatory

    Institute of Scientific and Technical Information of China (English)

    HAN NIU

    2007-01-01

    @@ On the afternoon of February 1, 2007, Chen Guangbiao, a noted philanthropist, found himself in the Jiangsu Provincial Juvenile Reformatory in Jurong City for a ceremony to donate two buses, 100 computers, and 100 desks and 100 chairs for the juvenile offenders to use in their study.

  11. Parenting and juvenile delinquency

    NARCIS (Netherlands)

    Hoeve, Machteld

    2008-01-01

    Juvenile delinquency is a noteworthy problem. This thesis addressed the association between parenting and juvenile delinquency by analyzing the concepts of parenting adopted in family research in relation to criminological concepts and measures of delinquent behavior. Four studies were conducted. Th

  12. Juvenile Confinement in Context

    Science.gov (United States)

    Mendel, Richard A.

    2012-01-01

    For more than a century, the predominant strategy for the treatment and punishment of serious and sometimes not-so-serious juvenile offenders in the United States has been placement into large juvenile corrections institutions, alternatively known as training schools, reformatories, or youth corrections centers. America's heavy reliance on…

  13. Parenting and juvenile delinquency

    NARCIS (Netherlands)

    Hoeve, Machteld

    2008-01-01

    Juvenile delinquency is a noteworthy problem. This thesis addressed the association between parenting and juvenile delinquency by analyzing the concepts of parenting adopted in family research in relation to criminological concepts and measures of delinquent behavior. Four studies were conducted. Th

  14. Juvenile mammary papillomatosis; Papilomatosis juvenil mamaria

    Energy Technology Data Exchange (ETDEWEB)

    Alvarez, M.; Jimenez, A. V. [Hospital Reina Sofia. Cordoba (Spain)

    2001-07-01

    Juvenile mammary papillomatosis is a benign proliferative disease of young patients, generally under 30 years of age. The most frequent clinical presentation is the existence of an elastic and mobile lymph node of the breast. Anatomopathologically, it is characterized because it presents ductal epithelial hyperplasia, sometimes with marked atypia, and there are numerous cysts having different sizes among the findings. It has been associated with an increase in the incidence of breast cancer, both in the patient herself as well as her family. We review the literature on the subject and present the mammographic and ultrasonographic findings of a 22 year old woman diagnosed of juvenile mammary papillomatosis. (Author) 12 refs.

  15. Bronchoalveolar lavage and response to cyclophosphamide in scleroderma alveolitis.

    Science.gov (United States)

    Colaci, M; Sebastiani, M; Giuggioli, D; Manfredi, A; Spagnolo, P; Luppi, F; Richeldi, L; Ferri, Clodoveo

    2010-03-01

    Systemic sclerosis (SSc) is characterized by abnormal fibrosis of the skin and internal organs, particularly the lungs. Recent reports have revealed a lack of correlation between bronchoalveolar lavage (BAL) variations and response to cyclophosphamide (CYC) in patients with scleroderma-related alveolitis. Our study aimed to evaluate whether the normalization of BAL cellularity correlates with long-term response to CYC. We retrospectively studied 26 consecutive SSc patients with alveolitis diagnosed by BAL and treated with CYC therapy (cumulative dosage 26.5 +/- 11.7 g; 21.1 +/- 8.9 months of treatment). We evaluated high-resolution computed tomography (HRCT), forced vital capacity (FVC), and carbon monoxide diffusing capacity (DLCO) variations before and after CYC. Radiological and functional parameters were re-evaluated in 23 patients after 1-year follow-up. BAL cellularity normalized after CYC therapy in 12/26 (46.2%) patients (group 1), while it remained abnormal in 14/26 (53.8%) (group 2). FVC and DLCO of group 1 slightly increased after CYC (p = 0.014 and p = 0.07, respectively) and remained stable at follow-up, whereas in group 2 they did not change after CYC and at follow-up (p = not significant). Moreover, at the end of CYC, FVC and/or DLCO showed a clinical improvement/stabilization in all patients of group 1 versus 8/14 of group 2, while at the re-evaluation 1 year after completing CYC, 2/11 patients of group 1 worsened versus 5/12 of group 2. HRCT progression was observed in 1/11 of group 1 and 8/12 of group 2 (p = 0.009). BAL fluid normalization after CYC therapy correlated with long-term response to treatment, contrary to what is observed in individuals with persistent alveolitis.

  16. Scleroderma and CREST syndrome: a case report in dentistry.

    Science.gov (United States)

    Lauritano, D; Bussolati, A; Baldoni, M; Leonida, A

    2011-09-01

    CREST syndrome is part of the heterogeneous scleroderma group of autoimmune diseases that cause thickening, hardening and tightening of the connective tissue in different parts of the body, and it may lead to complex disorders. CREST syndrome is characterized by the coexistence of calcinosis, Raynaud's phenomenon, esophageal hypomotility, sclerodactily and telangectasia. A 72-year-old caucasian woman is referred to the S. Gerardo Hospital of Monza, with a chief complaint of oral pain and difficulties in deglutition and eating, associated with denture instability and difficulties to fit it. She had been previously diagnosed with Raynaud's phenomenon, and afterwards with CREST syndrome. Extra-oral examination underlined taut, thickened and rigid skin, pallid-red irregular maculae all over the face, telangiectasias and acrocyanosis. Intra-oral examination showed no alteration of the mucosa, but we can observe tongue rigidity and some speckled red alternating with white spots on the hard palate and in the vestibule. We undermitted the patient the dental treatment of Sjogren's syndrome. The management of the Sjogren's syndrome is symptomatic and empirical, and involves the use of saliva secretion stimulators, salivary substitutes and coadjuvants. Dental treatment and prophylaxis are important to prevent the consequences of xerostomia, such as rampant caries, based on the administration of topical fluoride in toothpastes and rinses, and supplemented by fluoride gels and varnishes. Instruction and reinforcement of oral hygiene, along with frequent dental assessment and management by the dentist are essential measures to preserve the oral health of those affected with CREST syndrome in progression to SS, complicated with Sjogren's syndrome.

  17. [Juvenile monomelic amyotrophy: Hirayama disease].

    Science.gov (United States)

    Drozdowski, W; Baniukiewicz, E; Lewonowska, M

    1998-01-01

    We present three patients with unilateral upper limb weakness (with muscular atrophy)-two of them with distal and one with proximal localization. The disease onset was between 18th end 35-th year of life; the disease course was biphasic (i.e. progressive within first 1 to 3 years, and stabilized during following 4-24 years). The laboratory investigations permitted to diagnose juvenile monomelic amyotrophy, an entity that is very rare outside Japan. Electromyography revealed neurogenic involvement with spinal features also in clinically unaffected muscles. We suggest that these results may support the hypothesis of this disease being a benign variant of spinal muscular atrophy.

  18. Iloprost suppresses connective tissue growth factor production in fibroblasts and in the skin of scleroderma patients

    Science.gov (United States)

    Stratton, Richard; Shiwen, Xu; Martini, Giorgia; Holmes, Alan; Leask, Andrew; Haberberger, Thomas; Martin, George R.; Black, Carol M.; Abraham, David

    2001-01-01

    Patients with scleroderma receiving Iloprost as a treatment for severe Raynaud’s phenomenon report a reduction in skin tightness, suggesting that this drug inhibits skin fibrosis. Connective tissue growth factor (CTGF), a recently described profibrotic cytokine, acts downstream and in concert with TGF-β to stimulate the fibrotic process and is involved in the fibrosis seen in scleroderma. Here we show that Iloprost, acting by elevation of cAMP, blocks the induction of CTGF and the increase in collagen synthesis in fibroblasts exposed to TGF-β. The potency of Iloprost with respect to suppression of CTGF far exceeds that of other prostanoid receptor agonists, suggesting that its effect is mediated by the prostacyclin receptor IP. By sampling dermal interstitial fluid using a suction blister device, we show that CTGF levels are greatly elevated in the dermis of scleroderma patients compared with healthy controls and that Iloprost infusion causes a marked decrease in dermal CTGF levels. These studies suggest that Iloprost could be reducing the level of a key profibrotic cytokine in scleroderma patients and that endogenous production of eicosanoids may limit the fibrotic response to TGF-β. PMID:11457877

  19. Efficacy of anorectal biofeedback in scleroderma patients with fecal incontinence: a case-control study.

    Science.gov (United States)

    Collins, Josephine; Mazor, Yoav; Jones, Michael; Kellow, John; Malcolm, Allison

    2016-12-01

    To determine whether anorectal biofeedback therapy can improve the symptoms of fecal incontinence (FI) in patients with scleroderma when compared to patients with functional FI, and also whether there is any effect on anorectal physiology or quality of life (QOL). FI in patients with scleroderma is highly prevalent and is associated with significant loss of QOL. Biofeedback has been proven to be an effective treatment for functional FI, but there are no data to support its use in scleroderma. 13 consecutive female patients (median age 59, IQR 47-65 years) with scleroderma, and 26 age- and parity-matched female patients with functional FI (disease controls, 2:1), underwent biofeedback therapy for management of FI. Fecal incontinence severity index (FISI), anorectal physiology, feeling of control and QOL were collected before and after 6 weeks of biofeedback therapy, with additional scoring repeated at 6-month follow-up. After biofeedback treatment FISI, feeling of control and QOL significantly improved in both groups (p biofeedback therapy to the same extent as that achieved in patients with functional FI. There are significant improvements in symptoms, physiology and QOL. Biofeedback is an effective, low-risk treatment option in this patient group.

  20. A prospective study of plasma exchange in the treatment of diffuse scleroderma.

    Institute of Scientific and Technical Information of China (English)

    1995-01-01

    A prospective randomized study to compare the efficacy between plasma exchange (PE) plus Dpenicillamine (13 cases) (group Ⅰ)and D-penicillamine alone (16 cases) (group Ⅱ) in the treatment of diffuse scleroderma was carried out. Total skin score, the distance between finger and palm the distance between

  1. Site-Specific Differentiation of Fibroblasts in Normal and Scleroderma Skin

    Science.gov (United States)

    2009-06-01

    noncoding RNA expression in scleroderma ● Construction of skin tissue microarrays ● Use of skin diversity TMA to interrogate site-specific gene...disrupted during disease. Tissue microarray: genomics and proteo- mics in the context of skin architecture Gene and tiling microarrays are useful for

  2. local

    Directory of Open Access Journals (Sweden)

    Abílio Amiguinho

    2005-01-01

    Full Text Available The process of socio-educational territorialisation in rural contexts is the topic of this text. The theme corresponds to a challenge to address it having as main axis of discussion either the problem of social exclusion or that of local development. The reasons to locate the discussion in this last field of analysis are discussed in the first part of the text. Theoretical and political reasons are there articulated because the question is about projects whose intentions and practices call for the political both in the theoretical debate and in the choices that anticipate intervention. From research conducted for several years, I use contributions that aim at discuss and enlighten how school can be a potential locus of local development. Its identification and recognition as local institution (either because of those that work and live in it or because of those that act in the surrounding context are crucial steps to progressively constitute school as a partner for development. The promotion of the local values and roots, the reconstruction of socio-personal and local identities, the production of sociabilities and the equation and solution of shared problems were the dimensions of a socio-educative intervention, markedly globalising. This scenario, as it is argued, was also, intentionally, one of transformation and of deliberate change of school and of the administration of the educative territoires.

  3. Cryopyrin-Associated Autoinflammatory Syndromes (CAPS) - Juvenile

    Science.gov (United States)

    ... Cryopyrin-Associated Autoinflammatory Syndrome (CAPS) (Juvenile) Dermatomyositis (Juvenile) Familial Mediterranean Fever (Juvenile) Fibromyalgia Giant Cell Arteritis Glucocorticoid-induced Osteoperosis ...

  4. Juvenil idiopatisk arthritis

    DEFF Research Database (Denmark)

    Herlin, Troels

    2002-01-01

    The new classification of juvenile idiopathic arthritis (JIA) is described in this review. Clinical characteristics divide JIA in to subtypes: systemic, oligoarticular (persistent and extended type), RF-positive and--negative polyarticular, enthesitis-related arthritis and psoriatic arthritis...

  5. Juvenile Rockfish Recruitment Cruise

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — In 1983, the groundfish analysis project began a series of yearly cruises designed to assess the annual abundance of juvenile rockfish along the central California...

  6. Juvenile Idiopathic Arthritis

    Science.gov (United States)

    ... Physical Therapy Regular Exercise en español Artritis idiopática juvenil It may begin with a swollen knuckle, a ... may suddenly appear and disappear, developing in one area and then another. High fevers that tend to ...

  7. Juvenile Sex Offenders.

    Science.gov (United States)

    Ryan, Eileen P; Otonichar, Joseph M

    2016-07-01

    Sexual offending by juveniles accounts for a sizable percentage of sexual offenses, especially against young children. In this article, recent research on female juvenile sex offenders (JSOs), risk factors for offending in juveniles, treatment, and the ways in which these youth may differ from general delinquents will be reviewed. Most JSOs do not go on to develop paraphilic disorders or to commit sex offenses during adulthood, and as a group, they are more similar to nonsexual offending juvenile delinquents than to adult sex offenders. Recent research has elucidated some differences between youth who commit sex offenses and general delinquents in the areas of atypical sexual interests, the use of pornography, and early sexual victimization during childhood.

  8. Juvenile Spondyloarthritis Treatment Recommendations

    OpenAIRE

    Tse, Shirley; Burgos-Vargas, Ruben; Colbert, Robert A

    2012-01-01

    No specific recommendations for the treatment of juvenile spondyloarthritis have been established. Important differences exist in how spondyloarthritis begins and progresses in children and adults, supporting the need for pediatric-specific recommendations. Recently published recommendations for the treatment of juvenile arthritis consider children with sacroiliitis in a separate group, and allow for more accelerated institution of a TNF inhibitor depending on disease activity and prognostic ...

  9. Parenting and juvenile delinquency

    OpenAIRE

    Hoeve, Machteld

    2008-01-01

    Juvenile delinquency is a noteworthy problem. This thesis addressed the association between parenting and juvenile delinquency by analyzing the concepts of parenting adopted in family research in relation to criminological concepts and measures of delinquent behavior. Four studies were conducted. The first study addressed a meta-analysis on parenting characteristics and styles in relation to delinquency. In this meta-analysis, previous manuscripts were systematically analyzed, computing mean ...

  10. "The City of Colors": a good practical experience of youth integration in the local context of a marginal neighborhood in the city of Alicante / “La Ciudad de los Colores”: una experiencia de buena práctica de integración juvenil en el marco local de un barrio marginal en la ciudad de Alicante

    Directory of Open Access Journals (Sweden)

    Virgilio Francisco Candela Sevila

    2015-10-01

    Full Text Available This article explains the birth, evolution and subsequent project management of a juvenile intervention in the Northern of the city of Alicante, born in 2007 and remains in effect within a specific Municipal Comprehensive Plan. A project managed by a local social organization nonprofit (Danae Cultural Association and is formed by a group of 80 people, including students, parents or guardians, monitors and coordinators. The program is developed with the official support of the City Council of Alicante and the Generalitat Valenciana in a neighborhood context degraded and economically disadvantaged (North Zone of the city which hosts a significant number of immigrants that in some neighborhoods, exceeds 45% and live more than 120 nationalities. It is a very socially recognized project and has been awarded by municipal and regional institutions.

  11. Scleroderma and pulmonary hypertension complicating two pregnancies: use of neuraxial anesthesia, general anesthesia, epoprostenol and a multidisciplinary approach for cesarean delivery.

    Science.gov (United States)

    Moaveni, D; Cohn, J; Brodt, J; Hoctor, K; Ranasinghe, J

    2015-11-01

    Literature regarding the anesthetic care of patients with scleroderma during labor and delivery is limited to remote case reports. No recent publications provide information on the anesthetic management of patients with coexisting pulmonary hypertension. This report describes the anesthetic and multidisciplinary management of two pregnant patients with concomitant scleroderma and pulmonary hypertension undergoing cesarean delivery; one with neuraxial anesthesia and one with general anesthesia. Considerations for neuraxial and general anesthesia in patients with concurrent scleroderma and pulmonary hypertension are discussed.

  12. β-thymosins and interstitial lung disease: study of a scleroderma cohort with a one-year follow-up

    Directory of Open Access Journals (Sweden)

    Messana Irene

    2011-02-01

    Full Text Available Abstract Background β-thymosins play roles in cytoskeleton rearrangement, angiogenesis, fibrosis and reparative process, thus suggesting a possible involvement in the pathogenesis of systemic sclerosis. The aim of the study was to investigate the presence of thymosins β4, β4 sulfoxide, and β10 in bronchoalveolar lavage fluid of scleroderma patients with interstitial lung disease and the relation of these factors with pulmonary functional and radiological parameters. Methods β-thymosins concentrations were determined by Reverse Phase-High Performance Liquid Chromatography-Electrospray-Mass Spectrometry in the bronchoalveolar lavage fluid of 46 scleroderma patients with lung involvement and of 15 controls. Results Thymosin β4, β4 sulfoxide, and β10 were detectable in bronchoalveolar lavage fluid of patients and controls. Thymosin β4 levels were significantly higher in scleroderma patients than in controls. In addition, analyzing the progression of scleroderma lung disease at one-year follow-up, we have found that higher thymosin β4 levels seem to have a protective role against lung tissue damage. Thymosin β4 sulfoxide levels were higher in the smokers and in the scleroderma patients with alveolitis. Conclusions We describe for the first time β-thymosins in bronchoalveolar lavage fluid and their possible involvement in the pathogenesis of scleroderma lung disease. Thymosin β4 seems to have a protective role against lung tissue damage, while its oxidation product mirrors an alveolar inflammatory status.

  13. β-thymosins and interstitial lung disease: study of a scleroderma cohort with a one-year follow-up

    Science.gov (United States)

    2011-01-01

    Background β-thymosins play roles in cytoskeleton rearrangement, angiogenesis, fibrosis and reparative process, thus suggesting a possible involvement in the pathogenesis of systemic sclerosis. The aim of the study was to investigate the presence of thymosins β4, β4 sulfoxide, and β10 in bronchoalveolar lavage fluid of scleroderma patients with interstitial lung disease and the relation of these factors with pulmonary functional and radiological parameters. Methods β-thymosins concentrations were determined by Reverse Phase-High Performance Liquid Chromatography-Electrospray-Mass Spectrometry in the bronchoalveolar lavage fluid of 46 scleroderma patients with lung involvement and of 15 controls. Results Thymosin β4, β4 sulfoxide, and β10 were detectable in bronchoalveolar lavage fluid of patients and controls. Thymosin β4 levels were significantly higher in scleroderma patients than in controls. In addition, analyzing the progression of scleroderma lung disease at one-year follow-up, we have found that higher thymosin β4 levels seem to have a protective role against lung tissue damage. Thymosin β4 sulfoxide levels were higher in the smokers and in the scleroderma patients with alveolitis. Conclusions We describe for the first time β-thymosins in bronchoalveolar lavage fluid and their possible involvement in the pathogenesis of scleroderma lung disease. Thymosin β4 seems to have a protective role against lung tissue damage, while its oxidation product mirrors an alveolar inflammatory status. PMID:21314931

  14. Hyponatremia in a patient with scleroderma renal crisis: a potential role of activated renin-angiotensin system

    Directory of Open Access Journals (Sweden)

    Fukasawa Hirotaka

    2012-06-01

    Full Text Available Abstract Background Scleroderma renal crisis is an important complication of scleroderma (systemic sclerosis that is associated with significant morbidity and mortality. On the other hand, hyponatremia has never been reported in patients with scleroderma renal crisis. Case presentation A 66-year-old man with scleroderma was admitted to our hospital for an evaluation of renal dysfunction and extreme hypertension. The laboratory evaluation revealed remarkably high plasma renin activity in association with microangiopathic hemolytic anemia, and the anti-RNA polymerase III antibody assessment was positive. The patient was diagnosed with scleroderma renal crisis and was started treatment with enalapril maleate, an angiotensin-converting enzyme inhibitor. During hospitalization, the patient developed symptomatic hyponatremia three times and each laboratory analysis revealed improperly high levels of antidiuretic hormone without signs of extracellular fluid volume depletion as well as remarkably high plasma renin activities and angiotensin levels. However, hyponatremia has not been demonstrated to occur as a result of combined therapy with candesartan cilexetil, an angiotensin II receptor blocker, and aliskiren fumarate, a direct renin inhibitor. The plasma renin activities and angiotensin levels were normalized and the renal function was maintained after treatment. Conclusions To our best knowledge, this is the first documented case of scleroderma renal crisis complicated with hyponatremia. This report also suggests that the activated renin-angiotensin system may play a role in the development of hyponatremia and that hyponatremia should be taken into consideration as a rare but possible complication associated with screloderma renal crisis.

  15. Vocational Teachers' Role in Serving Juvenile Offenders.

    Science.gov (United States)

    Meers, Gary D.

    1983-01-01

    Educators need to understand the juvenile justice system to understand what juvenile offenders go through while completing their sentences. This article reviews cases and juvenile charge classifications, and presents a model for alternative sentencing options for juveniles. (JOW)

  16. Trunk asymmetry in juveniles

    Directory of Open Access Journals (Sweden)

    Triantafyllopoulos Georgios

    2008-09-01

    Full Text Available Abstract Background Trunk asymmetry (TA is a common phenomenon in children, but its incidence in juveniles is not known. The present cross sectional study reports TA in normal juveniles and provides data which describe the evolution of TA from early childhood to adolescence. Materials and methods The scoliometer readings in both standing and sitting forward bending position (FBP of 3301 children, (1645 boys, and 1656 girls aged from 3 to 9 years old were studied. TA was quantified by measuring angle of trunk rotation (ATR and children were categorized as symmetric (ATR = 0°, mild asymmetric (ATR 1° – 6° and severely asymmetric (ATR ≥ 7°. The difference of TA between standing and sitting FBP as well as differences between boys and girls in frequency of TA were also calculated. The scoliometer readings were analyzed by age to reveal at which age the juvenile pattern of TA changes into the adolescent one. Results 74.2% of boys and 77% of girls were symmetric (ATR = 0° in the thoracic region in standing FBP, while 82.7% of boys and 84.1% of girls were symmetric in the thoracic region in sitting FBP. Juvenile girls are more symmetric than boys but severe TA was found almost the same between the two genders. A significant reduction in the frequency of mild TA from standing into sitting FBP, in all the examined regions in both boys and girls was found, but in severe TA this reduction is very small. Analysing scoliometer readings by age it appears that significant TA changes take place between 8–9 years of age for boys and between 6–7 and 8–9 years for girls. TA in boys is changing into the adolescent pattern at a later age than in girls. Conclusion Juveniles were found more symmetric than adolescents, who were studied previously in a different study. Furthermore, juvenile girls were found more symmetric than boys. Juvenile TA pattern seems to be in accordance with the higher incidence of juvenile idiopathic scoliosis in boys. Furthermore

  17. Juvenile Incarceration and Health.

    Science.gov (United States)

    Barnert, Elizabeth S; Perry, Raymond; Morris, Robert E

    2016-03-01

    Addressing the health status and needs of incarcerated youth represents an issue at the nexus of juvenile justice reform and health care reform. Incarcerated youth face disproportionately higher morbidity and higher mortality compared to the general adolescent population. Dental health, reproductive health, and mental health needs are particularly high, likely as a result of lower access to care, engagement in high-risk behaviors, and underlying health disparities. Violence exposure and injury also contribute to the health disparities seen in this population. Further, juvenile incarceration itself is an important determinant of health. Juvenile incarceration likely correlates with worse health and social functioning across the life course. Correctional health care facilities allow time for providers to address the unmet physical and mental health needs seen in this population. Yet substantial challenges to care delivery in detention facilities exist and quality of care in detention facilities varies widely. Community-based pediatricians can serve a vital role in ensuring continuity of care in the postdetention period and linking youth to services that can potentially prevent juvenile offending. Pediatricians who succeed in understanding and addressing the underlying social contexts of their patients' lives can have tremendous impact in improving the life trajectories of these vulnerable youth. Opportunities exist in clinical care, research, medical education, policy, and advocacy for pediatricians to lead change and improve the health status of youth involved in the juvenile justice system.

  18. Infection of SARS-CoV on juvenile and adult Brandt's vole Microtus brandtii

    Institute of Scientific and Technical Information of China (English)

    GAO Hong; PENG Jingpian; DENG Wei; SHI Dazhao; BAO Linlin; WANG Dehua; ZHANG Binglin; QIN Chuan; ZHANG Zhibin

    2005-01-01

    We studied the infectious effect of SARS-CoV virus on juvenile and adult Brandt's Vole (Microtus brandtii) by nasal cavity spraying method (CCID50 is 105.7). SARS virus caused serious deaths in adults. The death adults demonstrated hemorrhage from mouth, nasal cavity and intestine, hemorrhageious interstitial pneumonia and gore in liver, spleen and kidney. The survival adults demonstrated local hemorrhagic spot in lung and emphysema, but the other organs showed no pathological abnormality. SARS virus caused no deaths in juveniles, but locomotion of infected juveniles became slower. In the early stage, there was local pneumonia in lung and SARS viruses were isolated from the pathological tissue. Only one control juvenile lived and the infected juvenile showed local pneumonia in lung. The results demonstrated that SARS-CoV infected Brandt's vole seriously and adults were more susceptive to SARS-CoV than juveniles. The Brandt's vole may be a potential animal model for SARS research.

  19. Estudio de la migración y de la actividad metabólica de los polimofonucleares neutrófilos en pacientes con periodontitis juvenil localizada Migration and metabolic activity of polymorphonuclear neutrophils in patients with localized juvenile periodontitis

    Directory of Open Access Journals (Sweden)

    Pablo Javier Patiño Grajales

    1993-03-01

    -family: Arial">Localized Juvenile periodontitis (LJP is characterized by a rapid loss of connective tissue insertion and of alveolar bone around the first molars and incisors of the permanent teeth. This research was carried out in order to establish a relationship between in vitro behavior of PMN and the different clinical types of the disease. Twenty-one patients were studied; they Included incipient, moderate and advanced LJP. Before any kind of dental treatment was applied PMN were isolated from each patient to determine their chemotaxis under agarose and their metabolic activity (luminoidependent- chemiluminiscence, LDCL. A group of healthy control individuals was similarly studied.

     

    Patients with advanced LJP had significantly lower chemotaxis (p=O.O23; on the other hand, LDCL of phorbol-myristate-acetate stimulated PMN was normalin the three groups as compared with controls; PMN stimulation with opsonized zimosan revealed significantly decreased metabolic activity in patients with incipient and advanced disease (p=O.O12. These findings indicate that patients with LJP behave as a heterogeneous group regarding PMN function. In order to define PMN response in each type of this disease It Is necessary to study the relationship between genetic components, environment and clinical phenotypes of the disease.

  20. Bronchogenic Carcinoma in a Scleroderma Patient with Multiple Metastases: One Case Report

    Institute of Scientific and Technical Information of China (English)

    Pradipta Guha; hivesh Shanker Sahai; Debasis Sarkar; Partha Sardar; Anup Singh; Biplab Mandal; Bidyut Kumar Das; Sanjoy Kumar Chatterjee

    2010-01-01

    @@ Introduction The association between pulmonary interstitial fibrosis and the development of bronchogenic carcinoma in a patient with scleroderma has been reported rarely[1]. It is hypothesized that intense epithelial proliferation that is accompanied by the .brotic process increases the occurrence of carcinomatous changes[2]. We report the case of a pa-tient who presented with 3-year history of Raynaud's phenomenon, gradual tightening of the skin which was ignored by the patient and her family members, and a 2-week history of severe respiratory dis-tress with left shoulder and upper back pain followed by the develop-ment of paraparesis. After a series of examinations, the patient was diagnosed with scleroderma and simultaneously with bronchogenic carcinoma and multiple distant metastases.

  1. Scleroderma renal crisis in a case of mixed connective tissue disease

    Directory of Open Access Journals (Sweden)

    Mukul Vij

    2014-01-01

    Full Text Available Mixed connective tissue disease (MCTD is an overlap syndrome first defined in 1972 by Sharp et al. In this original study, the portrait emerged of a connective tissue disorder sharing features of systemic lupus erythematosus, systemic sclerosis (scleroderma and polymyositis. Scleroderma renal crisis (SRC is an extremely infrequent but serious complication that can occur in MCTD. The histologic picture of SRC is that of a thrombotic micro-angiopathic process. Renal biopsy plays an important role in confirming the clinical diagnosis, excluding overlapping/superimposed diseases that might lead to acute renal failure in MCTD patients, helping to predict the clinical outcome and optimizing patient management. We herewith report a rare case of SRC in a patient with MCTD and review the relevant literature.

  2. Extending juvenility in grasses

    Energy Technology Data Exchange (ETDEWEB)

    Kaeppler, Shawn; de Leon Gatti, Natalia; Foerster, Jillian

    2017-04-11

    The present invention relates to compositions and methods for modulating the juvenile to adult developmental growth transition in plants, such as grasses (e.g. maize). In particular, the invention provides methods for enhancing agronomic properties in plants by modulating expression of GRMZM2G362718, GRMZM2G096016, or homologs thereof. Modulation of expression of one or more additional genes which affect juvenile to adult developmental growth transition such as Glossy15 or Cg1, in conjunction with such modulation of expression is also contemplated. Nucleic acid constructs for down-regulation of GRMZM2G362718 and/or GRMZM2G096016 are also contemplated, as are transgenic plants and products produced there from, that demonstrate altered, such as extended juvenile growth, and display associated phenotypes such as enhanced yield, improved digestibility, and increased disease resistance. Plants described herein may be used, for example, as improved forage or feed crops or in biofuel production.

  3. Unilateral acquired Brown's syndrome in systemic scleroderma: An unusual cause for diplopia.

    Science.gov (United States)

    Pawar, Neelam; Ravindran, Meenakshi; Ramakrishnan, Renagappa; Maheshwari, Devendra; Trivedi, Bhakti

    2015-11-01

    Brown's syndrome can be congenital or acquired with multiple causes. It has been described as a ocular complication in various rheumatic and nonrheumatic diseases. We describe a case of 27-year-old female patient with 5 years old history of systemic scleroderma who developed vertical diplopia, a left head tilt, and restriction of left eye on elevation in adduction. The patient responded to systemic steroids with resolution of diplopia.

  4. Recent Developments in the Classification, Evaluation, Pathophysiology, and Management of Scleroderma Renal Crisis.

    Science.gov (United States)

    Ghossein, Cybele; Varga, John; Fenves, Andrew Z

    2016-01-01

    Scleroderma renal crisis (SRC) is an uncommon complication of systemic sclerosis. Despite the advent of angiotensin-converting inhibitor therapy, SRC remains a life-threatening complication. Recent studies have contributed to a better understanding of SRC, but much remains unknown regarding its pathophysiology, risk factors, and optimal management. Genetic studies provide evidence that immune dysregulation might be a contributing factor, providing hope that further research in this direction might illuminate pathogenesis and provide novel predictors for this complication.

  5. DERMATOMIOSITIS JUVENIL Y EMBARAZO

    OpenAIRE

    Evans M,Gregorio; Poulsen R,Ronald; Blanco R,Romiely; Luna V,Viviana

    2002-01-01

    La dermatomiositis juvenil es un desorden inflamatorio crónico multisistémico del tejido conectivo. Tiene una incidencia de 2-3/100.000/año. Con la disminución en la mortalidad experimentada en los últimos decenios, la atención está cifrada en la morbilidad a largo plazo y en las alteraciones funcionales. Con un tratamiento agresivo los niños con dermatomiositis juvenil generalmente tienen un futuro promisorio, sin incapacidad o con incapacidad mínima. La mortalidad actualmente se estima cerc...

  6. Juvenile idiopatiske inflammatoriske myopatier

    Directory of Open Access Journals (Sweden)

    Helga Sanner

    2009-10-01

    Full Text Available Juvenile idiopathic inflammatory myopathies (JIIM is a group of rare autoimmune systemic diseases in children and adolescents, characterized by chronic skeletal muscle inflammation. Unlike in adults, dermatomyositis (JDM is by far the most common of the idiopathic inflammatory myopathies in children and adolescents. The hallmark of JDM is calcinosis, lipodystrophy and vasculitis, findings that differs the juvenile form of dermatomyosits from the adult form. JDM is still diagnosed and classified by Bohan and Peter’s criteria from 1975. There are limited data on long time outcome of this disease

  7. Antitopoisomerase I monoclonal autoantibodies from scleroderma patients and tight skin mouse interact with similar epitopes.

    Science.gov (United States)

    Muryoi, T; Kasturi, K N; Kafina, M J; Cram, D S; Harrison, L C; Sasaki, T; Bona, C A

    1992-04-01

    We have generated for the first time monoclonal antibodies (mAbs) specific for topoisomerase I (topo I) from scleroderma patients, and tight skin mice which develop a scleroderma-like syndrome. The epitope specificity of these antibodies has been determined using a series of fusion proteins containing contiguous portions of topo I polypeptide. Western blot analysis demonstrated that both human and mouse mAbs bound strongly to fusion protein C encompassing the NH2-terminal portion of the enzyme, and weakly to fusion proteins F and G containing regions close to the COOH-terminal end of the molecule. This crossreactivity is related to a tripeptide sequence homology in F, G, and C fusion proteins. It is interesting that a pentapeptide sequence homologous to that in fusion protein C was identified in the UL70 protein of cytomegalovirus, suggesting that activation of autoreactive B cell clones by molecular mimicry is possible. Both human and mouse mAbs exhibiting the same antigen specificity, also share an interspecies cross-reactive idiotope. These data suggest that B cell clones producing antitopo autoantibodies present in human and mouse repertoire are conserved during phylogeny, and are activated during the development of scleroderma disease.

  8. Linear scleroderma as a rare cause of enophthalmos: a case report

    Directory of Open Access Journals (Sweden)

    Fernando Bertie S

    2007-12-01

    Full Text Available Abstract Introduction Enophthalmos is an important physical sign which can be easily missed. Case presentation A 64-year old female presented with painless and progressive shrinking of her right eye. Visual acuity was 6/6 in both eyes. The main clinical findings included exophthalmometry readings of 14 mm in the right eye and 22 mm in the left eye and a linear scar on her right forehead. This scar is a feature of linear scleroderma and called "en coup de sabre". She was referred to a dermatologist for further assessment. Conclusion Enophthalmos is defined as the relative recession of the globe into the bony orbit and if measuring greater than 2 mm can give a noticeable cosmetic deformity. Scleroderma is a systemic or localised disease. Linear scleroderma has the following features-localised fibrosis of the skin, blood vessels, subcutaneous fat, muscle and sometimes bone. Histology shows an inflammatory and a sclerotic phase. Ophthalmic effects include enophthalmos, lash loss, lid induration or tightening and periorbital oedema.

  9. Oral cyclophosphamide improves pulmonary function in scleroderma patients with fibrosing alveolitis: experience in one centre.

    Science.gov (United States)

    Beretta, Lorenzo; Caronni, Monica; Raimondi, Massimo; Ponti, Alessandra; Viscuso, Tiziana; Origgi, Laura; Scorza, Raffaella

    2007-02-01

    Lung involvement constitutes nowadays the major cause of morbidity and mortality in scleroderma patients. Pulmonary fibrosis in systemic sclerosis (SSc) is thought to be the consequence of interstitial inflammation. Early diagnosis and treatment of active alveolitis is essential to prevent the deterioration of pulmonary function, improving outcome in SSc patients. The aim of the study was to investigate the effect of 1-year treatment with oral cyclophosphamide (CYC) on the evolution of interstitial lung disease in scleroderma patients with a diagnosis of active alveolitis. An open-label one-arm monocenteric study was conducted on 33 scleroderma patients with active alveolitis--defined as the presence of areas of 'ground-glass attenuation' on high-resolution computed tomography and a recent deterioration in lung function-treated with oral CYC 2 mg kg-1 day-1 for 1 year and medium-low dose steroids (prednisone 25 mg for 3 months and then tapered to 5 mg/day). Results showed that diffusing capacity for carbon monoxide (DLco) values remained stable after 6 months of treatment and significantly increased after 12 months (2.06+/-1.38, 2.21+/-1.62 and 2.39+/-1.64 mmol/min/kPa, at baseline/6/12 months, respectively; palveolitis, with beneficial effects lasting up to 1 year after interruption. The higher efficacy in those patients with an early pulmonary disease stage and a lower radiological grade underlies the importance of an early diagnosis and intervention.

  10. Juvenile Battens Disease.

    Science.gov (United States)

    Gayton, Romayne

    1987-01-01

    Ten children diagnosed with juvenile Battens disease were tested over a three-year period in general intelligence, memory, listening and speech, motor skills, and general learning. Results showed that the patients followed a predetermined pattern but that the time span for development of memory, communication, and behavior problems varied greatly.…

  11. Juvenile Victimization and Delinquency.

    Science.gov (United States)

    Esbensen, Finn-Aage; Huizinga, David

    1991-01-01

    Demographic characteristics of juvenile victims of crime and a potential relationship between victimization and self-reported delinquency are examined for 877 adolescents from a large midwestern city. Lifetime victimization rates (LVRs) are higher for those involved in delinquency, and LVRs rise with age and higher levels of delinquent behavior.…

  12. Juvenile Battens Disease.

    Science.gov (United States)

    Gayton, Romayne

    1987-01-01

    Ten children diagnosed with juvenile Battens disease were tested over a three-year period in general intelligence, memory, listening and speech, motor skills, and general learning. Results showed that the patients followed a predetermined pattern but that the time span for development of memory, communication, and behavior problems varied greatly.…

  13. Juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Prakken, Berent; Albani, Salvatore; Martini, Alberto

    2011-01-01

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

  14. Juvenile Polyps in Denmark From 1995 to 2014

    DEFF Research Database (Denmark)

    Jelsig, Anne Marie; Ousager, Lilian Bomme; Brusgaard, Klaus;

    2016-01-01

    BACKGROUND: Juvenile polyps in the large bowel are rare but the most common type of polyp in children. The prevalence and incidence are unknown, and few studies exist on the occurrence in adults. They are considered not to harbor any malignant potential unless they are part of the hereditary...... juvenile polyposis syndrome. OBJECTIVE: We aimed to study the demographics of juvenile polyps in Denmark in a 20-year period from 1995 to 2015 in both adults and children. This is the first report on the occurrence, anatomic localization, and reoccurrence of these polyps in a whole population. DESIGN: Data...... from all of the patients who had been diagnosed with 1 or more juvenile polyp from January 1, 1995, until December 31, 2014, were obtained. SETTINGS: The study was conducted based on patients registered in the nationwide pathological register in Denmark, the Danish Pathology Data Bank. PATIENTS: We...

  15. Late Onset Juvenile Xanthogranuloma

    Directory of Open Access Journals (Sweden)

    Punithwavathy K

    1999-01-01

    Full Text Available A 19 year old female was seen with multiple skin coloured and hyperpigmented macules, discrete as well as grouped papules and nodules of varying sizes distributed over the face, neck, extensor and flexor aspects of both upper and lower extremities including joints. The trunk was spared. Some of the lesions showed features of spontaneous regression. Investigations confirmed the diagnosis of juvenile xanthogranuloma. Lesions regressed satisfactorily with liquid nitrogen cryotherapy.

  16. Juvenile Incarceration and Health

    OpenAIRE

    Barnert, ES; R Perry; Morris, RE

    2015-01-01

    © 2015. Addressing the health status and needs of incarcerated youth represents an issue at the nexus of juvenile justice reform and health care reform. Incarcerated youth face disproportionately higher morbidity and higher mortality compared to the general adolescent population. Dental health, reproductive health, and mental health needs are particularly high, likely as a result of lower access to care, engagement in high-risk behaviors, and underlying health disparities. Violence exposure a...

  17. Juvenile Ultracool Dwarfs

    CERN Document Server

    Rice, Emily L; Cruz, Kelle; Barman, Travis; Looper, Dagny; Malo, Lison; Mamajek, Eric E; Metchev, Stanimir; Shkolnik, Evgenya L

    2011-01-01

    Juvenile ultracool dwarfs are late spectral type objects (later than ~M6) with ages between 10 Myr and several 100 Myr. Their age-related properties lie intermediate between very low mass objects in nearby star-forming regions (ages 1-5 Myr) and field stars and brown dwarfs that are members of the disk population (ages 1-5 Gyr). Kinematic associations of nearby young stars with ages from ~10-100 Myr provide sources for juvenile ultracool dwarfs. The lowest mass confirmed members of these groups are late-M dwarfs. Several apparently young L dwarfs and a few T dwarfs are known, but they have not been kinematically associated with any groups. Normalizing the field IMF to the high mass population of these groups suggests that more low mass (mainly late-M and possibly L dwarf) members have yet to be found. The lowest mass members of these groups, along with low mass companions to known young stars, provide benchmark objects with which spectroscopic age indicators for juvenile ultracool dwarfs can be calibrated and...

  18. Facial asymmetry: a case report of localized linear scleroderma patient with muscular strain and spasm

    OpenAIRE

    Kim, Jae-Hyung; Lee, Suck-Chul; Kim, Chul-Hoon; Kim, Bok-Joo

    2015-01-01

    Facial asymmetry is found in patients with or without cosmetic facial alterations. Some patients have facial asymmetry that manifests underlying skeletal problems, while others have only limited soft-tissue facial asymmetry. Orthognathic surgery brings about a dermatic change, as soft tissue covers underlying bones. Limited soft-tissue asymmetry, meanwhile, is difficult to correct. The treatment modalities for the creation or restoration of an esthetically pleasing appearance were autogenous ...

  19. Probable role of Brain Natriuretic Peptide (BNP in lung hypertension secondary to scleroderma

    Directory of Open Access Journals (Sweden)

    P. Faggioli

    2013-05-01

    Full Text Available BACKGROUND Scleroderma, when complicated with pulmonary hypertension (PHT, presents a worse prognosis; recently treatment with new drugs seems to offer good perspectives, especially in early diagnosis and treatment. The standard approach for diagnosing PHT consists in measurement of the pulmonary artery pressure (PAP by means of echodoppler. AIM OF INVESTIGATION Aim of this work is evaluating the significance of the NT-proBNP parameter, matched to echodoppler, in diagnosing scleroderma PHT. MATERIALS AND METHODS Sixty (60 patients, who came to observation for progressive systemic sclerosis underwent echodoppler in order to measure the PAP (normal values up to 30 mmHg. NT-proBNP was determined on serum sample using ECLIA method by Modular E170 (Roche Diagnostics; manufacturer reference values for age and gender were used. Forty-three (43 patients underwent a further NT-proBNP sampling 5 days later in order to assess parameter stability. RESULTS PHT and non- PHT patients showed statistically different (p < 0,001 medians (126 vs 69 pg/ml. No pathologic values of NT-proBNP were measured in the group with PAP < 30 mmHg, while 27% of cases who had PAP between 30 and 40 showed pathologic concentrations. The positivity ratio increases to 57% in patients showing PAP > 40 mmHg. No relevant correlation (r = 0,2 was found between PAP and NT-proBNP. Mean average between the two sampling groups was 31%. CONCLUSIONS In scleroderma patients, combination of NT-proBNP and PAP seems to improve the diagnosis of pulmonary hypertension, especially in presence of borderline pulmonary pressure values. We therefore propose the biochemical observation of NT-proBNP when PAP is > 30 mmHg and in monitoring the evolution of the pathology.

  20. Scleroderma and pulmonary hypertension Esclerodermia e hipertensão pulmonar

    Directory of Open Access Journals (Sweden)

    Karen A. Fagan

    2003-10-01

    Full Text Available Patients with scleroderma are at increased risk for the development of pulmonary hypertension, and the development of unexplained dyspnea or an isolated decrease in diffusing capacity should prompt evaluation. Echocardiography is often helpful in this situation, with further testing being performed as indicated. Because the prognosis of untreated pulmonary hypertension occurring in the setting of scleroderma is generally quite poor, vigilance is required on the part of physicians following this "at risk" group of patients. The past decade has seen important advances in the treatment of pulmonary arterial hypertension, including intravenous epoprostenol, oral bosentan and subcutaneously infused treprostinil. As new therapies are developed for the treatment of pulmonary arterial hypertension, it is essential that patients with scleroderma-related disease are included in clinical trials.Pacientes com esclerodermia têm risco aumentado para desenvolver hipertensão pulmonar. O aparecimento de dispnéia e/ou a diminuição da capacidade de difusão devem levar à suspeita imediata dessa complicação. A ecodopplercardiografia é importante para o diagnóstico e o seguimento desses casos. Os casos não tratados de hipertensão pulmonar em esclerodermia têm mau prognóstico, daí a necessidade em manter sob vigilância estes pacientes. Na última década surgiram avanços para o tratamento da hipertensão arterial pulmonar, incluindo os medicamentos epoprostenol EV, bosentan VO e treprostinil SC. À medida que novas terapias vão sendo desenvolvidas, torna-se necessário a realização de estudos clínicos de maior validade.

  1. Genetics Home Reference: juvenile idiopathic arthritis

    Science.gov (United States)

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

  2. Genetics Home Reference: juvenile primary osteoporosis

    Science.gov (United States)

    ... Home Health Conditions juvenile primary osteoporosis juvenile primary osteoporosis Enable Javascript to view the expand/collapse boxes. ... PDF Open All Close All Description Juvenile primary osteoporosis is a skeletal disorder characterized by thinning of ...

  3. Genetics Home Reference: juvenile myoclonic epilepsy

    Science.gov (United States)

    ... Home Health Conditions juvenile myoclonic epilepsy juvenile myoclonic epilepsy Enable Javascript to view the expand/collapse boxes. ... PDF Open All Close All Description Juvenile myoclonic epilepsy is a condition characterized by recurrent seizures (epilepsy). ...

  4. Collaboration between Correctional and Public School Systems Serving Juvenile Offenders: A Case Study.

    Science.gov (United States)

    Hellriegel, Kimberly L.; Yates, James R.

    1999-01-01

    This case study examined the relationship between an educational agency and a human service agency in providing services to juvenile offenders in a county-operated correctional facility as they transition to the local public school system. It urges juvenile justice and public school systems to work together to effectively meet the needs of this…

  5. Comprehensive Strategy for Serious, Violent, and Chronic Juvenile Offenders: Program Summary.

    Science.gov (United States)

    Office of Juvenile Justice and Delinquent Prevention (Dept. of Justice), Washington, DC.

    This document presents a comprehensive strategy for dealing with serious, violent, and chronic juvenile offenders developed by the United States Office of Juvenile Justice and Delinquency Prevention. It notes that the program described can be implemented at the state, county, or local level. The introduction presents statistics on violent…

  6. Collaboration between Correctional and Public School Systems Serving Juvenile Offenders: A Case Study.

    Science.gov (United States)

    Hellriegel, Kimberly L.; Yates, James R.

    1999-01-01

    This case study examined the relationship between an educational agency and a human service agency in providing services to juvenile offenders in a county-operated correctional facility as they transition to the local public school system. It urges juvenile justice and public school systems to work together to effectively meet the needs of this…

  7. Antinuclear antibodies in scleroderma, mixed connective tissue disease and "primary" Raynaud's phenomenon.

    Science.gov (United States)

    Cruz, M; Mejia, G; Lavalle, C; Cortes, J J; Reyes, P A

    1988-03-01

    The diversity of antibodies in patients with scleroderma, mixed connective tissue disease or "primary" Raynaud's phenomenon could be used as a laboratory aid in the clinical diagnosis. In serum samples of 75 patients we screened for antinuclear antibodies (HEp 2 cells), anti DNA, soluble nucleoprotein and extractable nuclear antigens (Sm, rRNP, U1-nRNP, SSA/Ro, SSB/La and Scl-70). Distinctive antinuclear antibodies pattern was identified in each group of patients. This immunologic profile is valuable for clinical diagnosis and the preferential association of certain autoantibodies with some diseases and not with others, suggest an antigen-driven stimulus for its production.

  8. 中国硬皮马勃属研究%Studies of Scleroderma from China

    Institute of Scientific and Technical Information of China (English)

    李建宗

    2003-01-01

    报道中国硬皮马勃属Scleroderma11种:S. cepa,S. areolatum,S. verrucosum,S. floridanum,S. texense,S. polyrhizum,S. sinnamariense,S. bovista,S. paradoxum,S. dicyosporum,S. citrinum. 其中2个新记录种:佛罗里达硬皮马勃 S. floridanum,黄硬皮马勃,S. sinnamariense,对这两个种进行了特征描述.

  9. Highly infectious symbiont dominates initial uptake in coral juveniles.

    Science.gov (United States)

    Abrego, David; VAN Oppen, Madeleine J H; Willis, Bette L

    2009-08-01

    The majority of reef-building corals acquire their obligate algal symbionts (Symbiodinium) from the environment. However, factors shaping the initial establishment of coral-algal symbioses, including parental effects, local environmental conditions and local availability of symbionts, are not well understood. This study monitored the uptake and maintenance of Symbiodinium in juveniles of two common corals, Acropora tenuis and Acropora millepora, that were reciprocally explanted between sites where adult colonies host different types of Symbiodinium. We found that coral juveniles were rapidly dominated by type D Symbiodinium, even though this type is not found in adult colonies (including the parental colonies) in four out of the five study populations. Furthermore, type D Symbiodinium was found in less than one-third of a wide range of coral species (n > 50) sampled at the two main study sites, suggesting that its dominance in the acroporid juveniles is not because it is the most abundant local endosymbiotic type. Moreover, dominance by type D was observed irrespective of the light intensity to which juveniles were exposed in a field study. In summary, despite its relatively low abundance in coral assemblages at the study sites and irrespective of the surrounding light environment, type D Symbiodinium is the main symbiont type initially acquired by juveniles of A. millepora and A. tenuis. We conclude that during early ontogeny in these corals, there are few barriers to the uptake of Symbiodinium types which differ from those found in parental colonies, resulting in dominance by a highly infectious and potentially opportunistic symbiont.

  10. Juvenile Justice Bulletin: Aftercare Services. Juvenile Justice Practices Series.

    Science.gov (United States)

    Gies, Steve V.

    This bulletin examines aftercare services that provide youth with comprehensive health, education, family, and vocational services upon their release from the juvenile justice system. Aftercare can be defined as reintegrative services that prepare out-of-home placed juveniles for reentry into the community by reestablishing the necessary…

  11. Miastenia gravis juvenil Juvenile myasthenia gravis

    Directory of Open Access Journals (Sweden)

    Oscar Papazian

    2009-01-01

    Full Text Available La miastenia gravis juvenil (MGJ es un trastorno crónico auto inmune en el cual existen anticuerpos séricos que al unirse a los receptores de acetilcolin nicotínicos de la membrana muscular de la placa motora alteran la transmisión neuromuscular. El resultado es fatiga muscular precoz con progresión a la parálisis durante estados de contracción muscular iterativos (movimientos o sostenidos (posturas y más raramente parálisis permanente durante el reposo. Los músculos inervados por los nervios craneales, especialmente los extraoculares y elevadores de los párpados, tienen más tendencia a la debilidad muscular persistente que los inervados por otros pares craneales y las extremidades. Las formas clínicas de presentación son generalizadas, oculares y respiratorias. El diagnóstico se sospecha mediante la anamnesia, la fatiga anormal se comprueba mediante el examen físico y la estimulación eléctrica iterativa del nervio que inerva al músculo afectado pero no paralizado. Se corrobora mediante la administración de inhibidores de la acetilcolin esterasa (IACE que al aumentar la cantidad de acetilcolin en la hendidura sináptica, corrigen la fatiga o la debilidad muscular transitoriamente. Se hace el diagnóstico de certeza mediante la demostración sérica de anticuerpos contra los receptores de acetilcolin (ACRA. El tratamiento es a largo plazo sintomático con IACE y etiopatogénico con inmunosupresores, plasmaféresis, gamma globulina endovenosa y timectomía. El curso es crónico. La remisión espontánea o después de tratamiento sintomático o etiopatogénico ocurre entre 1-10 años respectivamente. La mortalidad es prácticamente nula aun durantes las crisis miastenias gracias a la educación de padres, pacientes y público en general sobre el tema, al desarrollo del sistema de respuesta rápida de auxilio domiciliario y las unidades de cuidados intensivos y el empleo de la ventilación asistida profiláctica, plasmaféresis y

  12. Juvenile Dermatomyositis in Pregnancy

    Directory of Open Access Journals (Sweden)

    Anthony Emeka Madu

    2013-01-01

    Full Text Available Juvenile dermatomyositis has variable clinical presentations both in and outside of pregnancy. A literature review indicated that optimal maternal and fetal outcomes can be anticipated when the pregnancy is undertaken while the disease is in remission. Poorer outcomes are associated with flare-up of the disease in early pregnancy compared with exacerbation in the second or third trimester, when fetal prognosis is usually good. We present a case of JDM in pregnancy with disease exacerbation late in pregnancy and review of the relevant literature.

  13. Juvenile dermatomyositis in pregnancy.

    Science.gov (United States)

    Madu, Anthony Emeka; Omih, Edwin; Baguley, Elaine; Lindow, Stephen W

    2013-01-01

    Juvenile dermatomyositis has variable clinical presentations both in and outside of pregnancy. A literature review indicated that optimal maternal and fetal outcomes can be anticipated when the pregnancy is undertaken while the disease is in remission. Poorer outcomes are associated with flare-up of the disease in early pregnancy compared with exacerbation in the second or third trimester, when fetal prognosis is usually good. We present a case of JDM in pregnancy with disease exacerbation late in pregnancy and review of the relevant literature.

  14. Juvenile hyaline fibromatosis.

    Science.gov (United States)

    Larralde, M; Santos-Muñoz, A; Calb, I; Magariños, C

    2001-01-01

    Juvenile hyaline fibromatosis (JHF) is a rare autosomal recessive disease with onset in infancy or early childhood. It is characterized by papulonodular skin lesions, soft tissue masses, gingival hypertrophy, and flexion contractures of the large joints. The light and electron microscopic features are very distinctive. Here we report an 8-month-old boy with characteristic stiffness of the knees and elbows and pink confluent papules on the paranasal folds, and periauricular and perianal regions. He also had hard nodules all over the scalp and around the mouth, and severe gingival hypertrophy. Histologic and ultrastructural features were typical of JHF. Clinical features, pathology, and physiology are discussed.

  15. Juvenile Justice in Rural America.

    Science.gov (United States)

    Jankovic, Joanne, Ed.; And Others

    Producing a much-needed organized body of literature about rural juvenile justice, 14 papers (largely from the 1979 National Symposium on Rural Justice) are organized to identify current issues, identify forces causing changes in current systems, review programs responding to rural juvenile justice problems, and provide planning models to aid…

  16. Causes and risk factors for death in systemic sclerosis : a study from the EULAR Scleroderma Trials and Research (EUSTAR) database

    NARCIS (Netherlands)

    Tyndall, Anthony J.; Bannert, Bettina; Vonk, Madelon; Airo, Paolo; Cozzi, Franco; Carreira, Patricia E.; Bancel, Dominique Farge; Allanore, Yannick; Mueller-Ladner, Ulf; Distler, Oliver; Iannone, Florenzo; Pellerito, Raffaele; Pileckyte, Margarita; Miniati, Irene; Ananieva, Lidia; Gurman, Alexandra Balbir; Damjanov, Nemanja; Mueller, Adelheid; Valentini, Gabriele; Riemekasten, Gabriela; Tikly, Mohammed; Hummers, Laura; Henriques, Maria J. S.; Caramaschi, Paola; Scheja, Agneta; Rozman, Blaz; Ton, Evelien; Kumanovics, Gabor; Coleiro, Bernard; Feierl, Eva; Szucs, Gabriella; Von Muehlen, Carlos Alberto; Riccieri, Valeria; Novak, Srdan; Chizzolini, Carlo; Kotulska, Anna; Denton, Christopher; Coelho, Paulo C.; Koetter, Ina; Simsek, Ismail; de la Pena Lefebvre, Paloma Garcia; Hachulla, Eric; Seibold, James R.; Rednic, Simona; Stork, Jiri; Morovic-Vergles, Jadranka; Walker, Ulrich A.

    2010-01-01

    Objectives To determine the causes and predictors of mortality in systemic sclerosis (SSc). Methods Patients with SSc (n=5860) fulfilling the American College of Rheumatology criteria and prospectively followed in the EULAR Scleroderma Trials and Research (EUSTAR) cohort were analysed. EUSTAR centre

  17. Role of sodium silicate in induction of scleroderma-related autoantibodies in brown Norway rats through oral and subcutaneous administration.

    Science.gov (United States)

    Al-Mogairen, Sultan M

    2011-05-01

    Silica hazard is a growing occupational problem and has been reported to be associated with scleroderma via case reports and occupational studies. The aim of this study is to demonstrate whether oral or subcutaneous silicate exposure can induce an autoimmunity and scleroderma susceptibility in immunosensitive rats. Sodium silicate in a dose of 3 mg in 0.2 ml NS was administered through oral and subcutaneous routes to 20 brown Norway rats. Autoantibodies including ANA, anti-RNP, anti-SCL70 and anti-centromere were measured and compared with pre- and post-challenge serum samples. Serum ANA and anti-RNP were high in significant number of rats (P < 0.05) of only the subcutaneous silicate group. There is an increase in the number of positive readings of autoantibodies at 14th week in comparison with the number of positive readings of autoantibodies at 7th week but P values were not significant. It may be concluded that silicate might induce autoimmunity and scleroderma and it seems to be that the longer the duration of exposure the greater the risk. This is probably the first experimental animal study demonstrating the induction of scleroderma-related autoantibodies after challenge with silicate.

  18. Inhibition of collagen production in scleroderma fibroblast cultures by a connective tissue glycoprotein extracted from normal dermis

    Energy Technology Data Exchange (ETDEWEB)

    Maquart, F.X.; Bellon, G.; Cornillet-Stoupy, J.; Randoux, A.; Triller, R.; Kalis, B.; Borel, J.P.

    1985-08-01

    It was shown in a previous paper that a connective tissue glycoprotein (CTGP) extracted from normal rabbit dermis was able to inhibit total protein and collagen syntheses by normal dermis fibroblast cultures. In the present study, the effects of CTGP on scleroderma fibroblasts were investigated. (/sup 14/C)Proline incorporation into total proteins of the supernatant was not significantly different from that found in controls. By contrast, the amount of collagen, expressed as percentage of total secreted protein, was far higher in scleroderma cultures than in normal ones (14.4% +/- 6.0% vs 4.6% +/- 0.9%). Addition of CTGP to the medium induced a concentration-dependent inhibition of (/sup 14/C)proline incorporation into proteins from both control and scleroderma cells. In control cultures, no significant decrease of the percentage of collagen was observed, but over 60 micrograms/ml, both cytotoxic effects and inhibition of protein synthesis occurred. In scleroderma cultures, the inhibition was twice as effective on collagen as on noncollagen protein synthesis. The inhibition of collagen secretion was not related either to changes in collagen hydroxylation or to the intracellular catabolism of newly synthesized procollagen.

  19. Successful pregnancy with scleroderma renal disease and pulmonary hypertension in a patient using angiotensin converting enzyme inhibitors.

    OpenAIRE

    Baethge, B A; Wolf, R E

    1989-01-01

    A patient with scleroderma renal disease and pulmonary hypertension who had a successful pregnancy with the use of angiotensin converting enzyme inhibitors is presented. The routine use of these inhibitors during pregnancy is not recommended, however, owing to the reported potential risks to the fetus.

  20. Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials And Research group database

    OpenAIRE

    Walker, U.; A. Tyndall; Czirják, L; Denton, C.; Farge-Bancel, D.; Kowal-Bielecka, O.; Müller-Ladner, U; Bocelli-Tyndall, C.; Matucci-Cerinic, M; Ribi, Camillo; Chizzolini, Carlo; EUSTAR Group

    2007-01-01

    Systemic sclerosis (SSc) is a multisystem autoimmune disease, which is classified into a diffuse cutaneous (dcSSc) and a limited cutaneous (lcSSc) subset according to the skin involvement. In order to better understand the vascular, immunological and fibrotic processes of SSc and to guide its treatment, the EULAR Scleroderma Trials And Research (EUSTAR) group was formed in June 2004.

  1. Effects and safety of rituximab in systemic sclerosis : An analysis from the European Scleroderma Trial and Research (EUSTAR) group

    NARCIS (Netherlands)

    Jordan, Suzana; Distler, Jörg H W; Maurer, Britta; Huscher, Dörte; Van Laar, Jacob M.; Allanore, Yannick; Distler, Oliver; Kvien, Tore K.; Airo, Paolo; Sancho, Juan José Alegre; Ananjeva, Lidia; Ancuta, Codrina Michaela; Aringer, Martin; Balbir-Gurman, Alexandra; Cantatore, Francesco Paolo; Caramaschi, Paola; Chatelus, Emmanuel; Codullo, Veronica; Farge-Bancel, Dominique; Foti, Rosario; Gabrielli, Armando; Henes, Jörg; Herrgott, Ilka; Iannone, Florenzo; Ingegnoli, Francesca; Loyo, Esthela; Matucci-Cerinić, Marco; Mohamed, Walid Ahmed Abdel Atty; Müller-Ladner, Ulf; Palm, Øyvind; Popa, Sergiu; Riemekasten, Gabriela; Rednic, Simona; Rosato, Edoardo; Saracco, Marta; Scheja, Agneta; Smith, Vanessa; Mihai, Carina; Szucs, Gabriela; Tomšić, Matija; Valentini, Gabriele; Walker, Ulrich A.; Westhovens, Rene; Yavuz, Sule Kurhan; Zenone, Thierry

    2015-01-01

    Objectives: To assess the effects of Rituximab (RTX) on skin and lung fibrosis in patients with systemic sclerosis (SSc) belonging to the European Scleroderma Trial and Research (EUSTAR) cohort and using a nested case-control design. Methods: Inclusion criteria were fulfilment of American College of

  2. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database.

    NARCIS (Netherlands)

    Tyndall, A.J.; Bannert, B.; Vonk, M.C.; Airo, P.; Cozzi, F.; Carreira, P.E.; Bancel, D.F.; Allanore, Y.; Muller-Ladner, U.; Distler, O.; Iannone, F.; Pellerito, R.; Pileckyte, M.; Miniati, I.; Ananieva, L.; Gurman, A.B.; Damjanov, N.; Mueller, A.; Valentini, G.; Riemekasten, G.; Tikly, M.; Hummers, L.; Henriques, M.J.; Caramaschi, P.; Scheja, A.; Rozman, B.; Ton, E.; Kumanovics, G.; Coleiro, B.; Feierl, E.; Szucs, G.; Muhlen, C.A. Von; Riccieri, V.; Novak, S.; Chizzolini, C.; Kotulska, A.; Denton, C.; Coelho, P.C.; Kotter, I.; Simsek, I.; Pena Lefebvre, P.G. de la; Hachulla, E.; Seibold, J.R.; Rednic, S.; Stork, J.; Morovic-Vergles, J.; Walker, U.A.

    2010-01-01

    OBJECTIVES: To determine the causes and predictors of mortality in systemic sclerosis (SSc). METHODS: Patients with SSc (n=5860) fulfilling the American College of Rheumatology criteria and prospectively followed in the EULAR Scleroderma Trials and Research (EUSTAR) cohort were analysed. EUSTAR

  3. Effects and safety of rituximab in systemic sclerosis : An analysis from the European Scleroderma Trial and Research (EUSTAR) group

    NARCIS (Netherlands)

    Jordan, Suzana; Distler, Jörg H W; Maurer, Britta; Huscher, Dörte; Van Laar, Jacob M.; Allanore, Yannick; Distler, Oliver; Kvien, Tore K.; Airo, Paolo; Sancho, Juan José Alegre; Ananjeva, Lidia; Ancuta, Codrina Michaela; Aringer, Martin; Balbir-Gurman, Alexandra; Cantatore, Francesco Paolo; Caramaschi, Paola; Chatelus, Emmanuel; Codullo, Veronica; Farge-Bancel, Dominique; Foti, Rosario; Gabrielli, Armando; Henes, Jörg; Herrgott, Ilka; Iannone, Florenzo; Ingegnoli, Francesca; Loyo, Esthela; Matucci-Cerinić, Marco; Mohamed, Walid Ahmed Abdel Atty; Müller-Ladner, Ulf; Palm, Øyvind; Popa, Sergiu; Riemekasten, Gabriela; Rednic, Simona; Rosato, Edoardo; Saracco, Marta; Scheja, Agneta; Smith, Vanessa; Mihai, Carina; Szucs, Gabriela; Tomšić, Matija; Valentini, Gabriele; Walker, Ulrich A.; Westhovens, Rene; Yavuz, Sule Kurhan; Zenone, Thierry

    2015-01-01

    Objectives: To assess the effects of Rituximab (RTX) on skin and lung fibrosis in patients with systemic sclerosis (SSc) belonging to the European Scleroderma Trial and Research (EUSTAR) cohort and using a nested case-control design. Methods: Inclusion criteria were fulfilment of American College of

  4. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database.

    NARCIS (Netherlands)

    Tyndall, A.J.; Bannert, B.; Vonk, M.C.; Airo, P.; Cozzi, F.; Carreira, P.E.; Bancel, D.F.; Allanore, Y.; Muller-Ladner, U.; Distler, O.; Iannone, F.; Pellerito, R.; Pileckyte, M.; Miniati, I.; Ananieva, L.; Gurman, A.B.; Damjanov, N.; Mueller, A.; Valentini, G.; Riemekasten, G.; Tikly, M.; Hummers, L.; Henriques, M.J.; Caramaschi, P.; Scheja, A.; Rozman, B.; Ton, E.; Kumanovics, G.; Coleiro, B.; Feierl, E.; Szucs, G.; Muhlen, C.A. Von; Riccieri, V.; Novak, S.; Chizzolini, C.; Kotulska, A.; Denton, C.; Coelho, P.C.; Kotter, I.; Simsek, I.; Pena Lefebvre, P.G. de la; Hachulla, E.; Seibold, J.R.; Rednic, S.; Stork, J.; Morovic-Vergles, J.; Walker, U.A.

    2010-01-01

    OBJECTIVES: To determine the causes and predictors of mortality in systemic sclerosis (SSc). METHODS: Patients with SSc (n=5860) fulfilling the American College of Rheumatology criteria and prospectively followed in the EULAR Scleroderma Trials and Research (EUSTAR) cohort were analysed. EUSTAR cent

  5. The role of ultrasound imaging in the evaluation of peripheral nerve in systemic sclerosis (scleroderma)

    Energy Technology Data Exchange (ETDEWEB)

    Tagliafico, Alberto, E-mail: atagliafico@sirm.org [Department of Radiology, University of Genova, Genova (Italy); Panico, Nicoletta [Division of Immunology, Department of Internal Medicine, University of Genoa, Genoa (Italy); Resmini, Eugenia [Department of Endocrinological and Medical Sciences (DiSEM), Center of Excellence for Biomedical Research, University of Genova, Genova (Italy); Derchi, Lorenzo E. [Department of Radiology, University of Genova, Genova (Italy); Ghio, Massimo [Division of Immunology, Department of Internal Medicine, University of Genoa, Genoa (Italy); Martinoli, Carlo [Department of Radiology, University of Genova, Genova (Italy)

    2011-03-15

    Background: Patients affected by scleroderma may complain of sensory disturbances especially in the hands. Purpose: To study the imaging features of upper limb nerves in patients affected by scleroderma (SSc). Materials and method: Twenty-five patients affected only by SSc were prospectively evaluated with high-resolution US and magnetic resonance (MRI) or computer tomography (CT) when necessary (2 patients). Median and ulnar nerves were evaluated bilaterally. Nerve conduction studies were performed in the symptomatic patients (n = 10). Results of imaging studies were correlated with disease duration, autoimmunity and immunosuppression. Nerves of SSc patients were compared with a control group of 90 patients matched for age and body mass index. Results: The prevalence of sensory disturbances revealed by clinical examination was 40%. In symptomatic SSc patients (n = 10) US evaluation revealed nerve abnormalities in 70% of cases (n = 7/10). n = 2 had a carpal tunnel syndrome. n = 5 had cubital tunnel syndrome. In two of them CT and MR were necessary to identify the compressed nerve at the level of the elbow due to the presence of calcifications. There was no association between the presence of an entrapment neuropathy and disease duration, autoantibodies and immunosuppression. Conclusion: Ultrasound, CT and MR may detect nerve abnormalities in 70% of SSc patients complaining of neurologic disturbances in the hands. The results of imaging studies support the hypothesis of a vascular dependent neuropathy in SSc.

  6. Ten years EULAR Scleroderma Research and Trials (EUSTAR): what has been achieved?

    Science.gov (United States)

    Müller-Ladner, Ulf; Tyndall, Alan; Czirjak, Laszlo; Denton, Christopher; Matucci-Cerinic, Marco

    2014-02-01

    Ten years ago a group of researchers interested in and dedicated to the field of systemic sclerosis started a unique experiment called European League Against Rheumatism (EULAR) Scleroderma Trials And Research (EUSTAR) that was designed to establish a large-scale database in a rare disease to facilitate the exact characterisation of this debilitating disease, spread the knowledge even beyond the borders of Europe and stimulate innovative research targeting the major problems of the affected patients. This EUSTAR experiment, with all its facets, including the creation of a large-scale database, the initiation of more than 40 investigator-driven clinical and basic science projects, the teaching of more than 400 young clinicians in the field of systemic sclerosis and the realisation of multicenter EU grants, which were all facilitated by an initial research grant from EULAR, is outlined and commented upon by the members of the steering committee in this viewpoint article on behalf of the now more than 150 contributing centres and the international systemic sclerosis patients' association, the Federation of European Scleroderma Associations.

  7. [Diffuse infiltrative lung disease in scleroderma. Analysis of radio-clinical and functional semiology].

    Science.gov (United States)

    El Khattabi, W; Afif, H; Moussali, N; Aichane, A; Abdelouafi, A; Bouayad, Z

    2013-06-01

    Scleroderma (SD) is a systemic disease that predominantly affects the skin. Diffuse infiltrative lung disease (DILD) is rare and occurs most often in the course of the disease. We analyzed seven cases of DILO of SD recorded between 2003 and 2010 among 196 PID (3.6%). Functional signs were limited to respiratory dyspnea, it was associated to dysphagia in six cases, dry syndrome in five cases and Raynaud's phenomenon in four cases. Clinical examination found crackles in the bases of the thorax in all cases and specific cutaneous signs in six cases. The chest radiograph showed that interstitial disease predominates at the lung bases in all cases with a large aspect of the pulmonary arteries in two cases. The chest CT scan confirmed the predominance of basal and peripheral damage with signs of fibrosis in six cases. The pulmonary function objectified a severe restrictive ventilatory defect in all cases. Bronchoscopy showed a normal macroscopic appearance in all cases, the broncho-alveolar lavage was predominated by neutrophilic formula in four cases. SCL 70 antibodies were positive in four cases. All patients were treated by steroids with improvement of dyspnea and stabilization of radiographs. A patient had died in an array of acute respiratory failure and one patient was lost to follow-up. DILD in scleroderma is rare and seldom reveals the disease, it affects the patient's prognosis especially when associated with arterial pulmonary hypertension. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

  8. Juvenile arthritis and uveitis.

    Science.gov (United States)

    Kanski, J J

    1990-01-01

    The association between juvenile arthritis and uveitis is reviewed. Some children with the HLA-B27 related spondyloarthropathies develop anterior uveitis. About 20% of patients with juvenile rheumatoid arthritis (JRA) who are negative for IgM rheumatoid factor develop a frequently bilateral, nongranulomatous chronic anterior uveitis. Risk factors for uveitis in JRA patients are: female gender, pauciarticular onset of arthritis, presence of circulating antinuclear antibodies, and the antigens HLA-DW5 and HLA-DPw2. Uveitis is rare after seven years or more have elapsed from the onset of arthritis. The visual prognosis in patients with uveitis is good in 25% and fair in 50%. The remaining 25% develop visual impairment from complicated cataract and/or secondary inflammatory glaucoma. The potential benefit of cytotoxic agents in the treatment of intractable uveitis is outweighed by the risk of serious side effects. The management of secondary inflammatory glaucoma is unsatisfactory, but the results of treatment of complicated cataracts by lensectomy-vitrectomy are good.

  9. Juvenile justice mental health services.

    Science.gov (United States)

    Thomas, Christopher R; Penn, Joseph V

    2002-10-01

    As the second century of partnership begins, child psychiatry and juvenile justice face continuing challenges in meeting the mental health needs of delinquents. The modern juvenile justice system is marked by a significantly higher volume of cases, with increasingly complicated multiproblem youths and families with comorbid medical, psychiatric, substance abuse disorders, multiple family and psychosocial adversities, and shrinking community resources and alternatives to confinement. The family court is faced with shrinking financial resources to support court-ordered placement and treatment programs in efforts to treat and rehabilitate youths. The recognition of high rates of mental disorders for incarcerated youth has prompted several recommendations for improvement and calls for reform [56,57]. In their 2000 annual report, the Coalition for Juvenile Justice advocated increased access to mental health services that provide a continuum of care tailored to the specific problems of incarcerated youth [58]. The specific recommendations of the report for mental health providers include the need for wraparound services, improved planning and coordination between agencies, and further research. The Department of Justice, Office of Juvenile Justice and Delinquency Prevention has set three priorities in dealing with the mental health needs of delinquents: further research on the prevalence of mental illness among juvenile offenders, development of mental health screening assessment protocols, and improved mental health services [59]. Other programs have called for earlier detection and diversion of troubled youth from juvenile justice to mental health systems [31,56]. Most recently, many juvenile and family courts have developed innovative programs to address specific problems such as truancy or substance use and diversionary or alternative sentencing programs to deal with first-time or nonviolent delinquents. All youths who come in contact with the juvenile justice system

  10. Cranial juvenile psammomatoid ossifying fibroma: case report.

    Science.gov (United States)

    Barrena López, Cristina; Bollar Zabala, Alicia; Úrculo Bareño, Enrique

    2016-03-01

    Juvenile psammomatoid ossifying fibroma (JPOF) is a fibroosseous tumor that arises in the craniofacial bones in young people. This lesion usually originates in the jaw, orbit, and ethmoid complex but can also be associated with the skull base and calvaria. Diagnosis must be made based on observing typical radiological and histopathological features. Although JPOF is a rare pathological entity, neurosurgeons must consider this odontogenic lesion in the differential diagnosis of skull masses given the lesion's aggressive behavior and locally invasive growth. Treatment must be gross-total resection. In the following article, the authors present a case of cranial JPOF and discuss various aspects of this entity.

  11. Miranda Rights: Implications for Juveniles with Disabilities

    Science.gov (United States)

    Katsiyannis, Antonis; Barrett, David E.; Losinski, Mickey L.

    2011-01-01

    Juvenile delinquency in the United States has been a persistent concern for decades. Consequently, because more juveniles have been referred to juvenile court and the arrest rate of preteen offenders has increased to almost three times that of older youth, the persistent and often controversial issue of the capacity of juvenile offenders to waive…

  12. Juvenile prison in parallel legislation

    Directory of Open Access Journals (Sweden)

    Lutovac Mitar

    2016-01-01

    Full Text Available The need for punishment of juveniles occurred from the time when there was no clear line separating them from the adult criminal population. At the same time, the evolution of the juvenile punishment is not in itself involve substantial changes to their criminal status. On the contrary, the status of minors in society did not show serious differences regarding the status of young adults, as well as the adult elderly. On the other hand, on the ground of their punishment is recorded deviations that go in the direction of application of mild corporal punishment. Closing the minor was performed in a physically separate parts of the general penal institutions with the use of a lower degree of restrictions while serving juvenile prison. Due to the different treatment of minors during the evolution of their criminal status leads to their different treatment in comparative law. That is why we are witnessing the existence of numerous differences in the juvenile punishment in some countries in the world. On the European continent there is a wide range of different legal solutions when it comes to punishing juveniles. There are considerable differences in the procedure pronouncing juvenile prison and in particular penal treatment of juveniles in penitentiary institutions. For these reasons, the author has decided to show the basic statutory provisions in the part that relates to the issue of punishment of minors in the legislation of individual countries.

  13. Update on juvenile myasthenia gravis.

    Science.gov (United States)

    Liew, Wendy K M; Kang, Peter B

    2013-12-01

    Juvenile myasthenia gravis is a relatively rare autoimmune neuromuscular disorder. The pathophysiology of juvenile myasthenia gravis is similar to that of adult myasthenia gravis, though there remain important differences regarding presentation and therapeutic options. We review the pathophysiology, clinical presentation, and treatment options for juvenile myasthenia gravis. Randomized clinical studies of myasthenia gravis have been carried out primarily in adult populations. As juvenile myasthenia gravis is rare, it has been difficult to collect prospective randomized controlled data to evaluate treatment outcomes and efficacy. A recent retrospective series suggests that, as in adult myasthenia gravis, thymectomy is a viable therapeutic option for selected cases of generalized juvenile myasthenia gravis. This is corroborated by the clinical experience of the authors in a referral center with a cohort of patients affected by juvenile myasthenia gravis over a number of years. Recent studies illustrate that some, but not all, adult research on myasthenia gravis is applicable to children and adolescents with juvenile myasthenia gravis. Adult research can inform pediatric studies, but should not be regarded as a substitute for dedicated research in those populations.

  14. Juvenile chronic arthritis.

    Science.gov (United States)

    Southwood, T R; Woo, P

    1995-05-01

    The nomenclature and classification criteria for arthritis in children should be dealt with initially as separate issues, although they are undoubtedly intertwined. The classification criteria should aim to delineate homogeneous patient populations, yet should be flexible enough to incorporate advances in disease knowledge. It should be recognized that arriving at an international consensus for classification criteria will merely provide a set of operational definitions to facilitate research, and not a set of diagnostic criteria. Indeed the only point to obtaining consensus is to begin a process of systematic ongoing review of the criteria. The labels attached to any of these diseases should facilitate accurate communication. In view of the heterogeneous nature of childhood arthritis, consideration should be given to using a broad umbrella term such as juvenile or childhood arthritis only for communicating with the lay public. Medical nomenclature should be formulated to reflect accurately homogeneous subgroups of arthritis, and should not artificially proscribe a relationship between paediatric and adult disease.

  15. [Juvenile idiopathic arthritis].

    Science.gov (United States)

    Herlin, Troels

    2002-08-19

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

  16. Juvenile hyaline fibromatosis

    Directory of Open Access Journals (Sweden)

    Jayashree Krishnamurthy

    2011-01-01

    Full Text Available Juvenile hyaline fibromatosis is a rare, autosomal-recessive disease characterized by papular and nodular skin lesions, gingival hyperplasia, joint contractures and bone involvement in variable degrees. It is a connective tissue disorder with aberrant synthesis of glycosaminoglycans by fibroblasts. We report a 5-year-old female born of first-degree consanguineous marriage who presented with multiple, recurrent, painless, variable-sized nodules. Fine needle aspiration cytology smears and the subsequent histopathological examination from the nodules showed benign spindle cells in a Periodic acid Schiff-positive myxoid background. The disease has a relentlessly progressive course, with most patients surviving only up to the 4 th decade. As of now, there is no specific treatment for this disorder. Genetic counseling is essential to explain to parents about a 25% chance of having a diseased baby in any pregnancy. With the gene being mapped recently, techniques for antenatal diagnosis are likely to be established.

  17. [JUVENILE DERMATOMYOSITIS AND CALCINOSIS].

    Science.gov (United States)

    Zhvania, M

    2015-01-01

    Juvenile Dermatomiositis (JD) is autoimmune disease that progresses with time; JD's main differentiated syndromes are rash on the skin, poor function of muscles, and often developing invalidism. If the health practitioners manage to diagnose the JD on an early stage and prescribe the adequate treatment the disease will not progress aggressively. This approach is tangible for practical rheumatology and pediatric. The article aims to present the reasons of the development of the JD and calcinosis. The study based on the description of the patients with JD. There are distinguished the main symptoms of the disease in children: frequent and acute developments of muscles calcinosis, occasionally with diffuse character followed with hypotrophy of the muscles, contractures and invalidism. One of the patient cases that describe the article is the thirteen-year boy with JD indicating repeated sequence of the disease, with diffusive calcinosis, cellulitis followed with secondary infection and impaired vision.

  18. Juvenile idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    Krupa H Bhatt

    2014-01-01

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

  19. Juvenile homosexual homicide.

    Science.gov (United States)

    Myers, Wade C; Chan, Heng Choon Oliver

    2012-01-01

    Limited information exists on juvenile homosexual homicide (JHH), that is, youths who perpetrate sexual homicides against same-sex victims. Only a handful of cases from the United States and internationally have been described in the literature. This study, the first of its kind, examines the epidemiology, victimology, victim-offender relationship, and weapon-use patterns in JHH offenders using a large U.S. database on homicide spanning three decades. The data for this study were derived from the Federal Bureau of Investigation's Supplementary Homicide Reports (SHRs) for the years 1976 through 2005. A total of 93 cases of JHH were identified. On average, three of these crimes occurred annually in the U.S., and there was a marked decline in its incidence over the study period. Ninety-five percent were male offender-male victim cases and 5% were female offender-female victim cases. JHH offenders were over-represented amongst all juvenile sexual murderers, similar to their adult counterparts. The majority of these boys were aged 16 or 17 and killed adult victims. They were significantly more likely to kill adult victims than other age groups, to be friends or acquaintances of the victims, and to use contact/edged weapons or firearms. Most offenders killed same-race victims, although Black offenders were significantly more likely than White offenders to kill interracially. A case report is provided to illustrate JHH. Further research is needed to promote our understanding of the pathogenesis, etiology, and associated risk factors for this aberrant form of murder by children.

  20. Do juvenile Amphiprion ocellaris (Pisces

    DEFF Research Database (Denmark)

    Brolund, Thea Marie; Nielsen, Lis Engdahl; Arvedlund, Michael

    2003-01-01

    . This is contrary to the settling mechanisms of the damselfish D. aruanus and D. reticulatus, and of the temperate herring Clupea harengus. Hence the results emphasize the variation of sensory abilities and behaviours in fish larvae and juveniles. It is not an area prone for generalizations.......Juvenile anemonefish Amphiprion ocellaris were tested in two behavioural laboratory set-ups for their ability to visually or chemically recognize conspecifics. Individuals of two other species of anemonefish, A. clarkii and Dascyllus aruanus, were also used as test specimens for recognition....... The results indicate that juvenile A. ocellaris recognize conspecifics visually rather than by olfaction. This is contrary to their finding mechanism of their host anemone. However, the results also indicate that the juvenile A ocellaris are neither attracted nor deterred by the presence of conspecifics...

  1. Bilateral, independent juvenile nasopharyngeal angiofibroma

    DEFF Research Database (Denmark)

    Mørkenborg, Marie-Louise; Frendø, M; Stavngaard, T;

    2015-01-01

    BACKGROUND: Juvenile nasopharyngeal angiofibroma is a benign, vascular tumour that primarily occurs in adolescent males. Despite its benign nature, aggressive growth patterns can cause potential life-threatening complications. Juvenile nasopharyngeal angiofibroma is normally unilateral, originating...... from the sphenopalatine artery, but bilateral symptoms can occur if a large tumour extends to the contralateral side of the nasopharynx. This paper presents the first reported case of true bilateral extensive juvenile nasopharyngeal angiofibroma involving clinically challenging pre-surgical planning...... embolisation. Radical removal performed as one-step, computer-assisted functional endoscopic sinus surgery was performed. The follow-up period was uncomplicated. CONCLUSION: This case illustrates the importance of suspecting bilateral juvenile nasopharyngeal angiofibroma in patients presenting with bilateral...

  2. Editor's Shelf: International Juvenile Titles.

    Science.gov (United States)

    Mitchell-Powell, Brenda

    1994-01-01

    Provides an annotated list of international juvenile picture books and notes those that emphasize text over pictures. The 49 titles present international perspectives for educators, librarians, and parents seeking materials with alternative cultural content. The majority are folk tales. (SLD)

  3. Envenomation by a juvenile Malayan pit viper (Agkistrodon rhodostoma).

    Science.gov (United States)

    Vest, D K; Kardong, K V

    1980-05-01

    Following an accidental bite inflicted by a juvenile Malayan pit viper (Agkistrodon rhodostoma), the progress of envenomation was carefully monitored and subsequent laboratory work performed to determine the amount and quality of venom injected. Even a very small amount of venom from this species is capable of inducing noticeable local symptoms including edema, subcutaneous bleeding, and throbbing. Constitutional symptoms were present but minimal. The extent of inflammation present at any given time following envenomation was found to be a more accurate diagnostic signal than the speed with which it developed. This case is similar to that of bites by juveniles of other species of viperines and crotalines and may serve to aid in the diagnosis and treatment of bites by juvenile specimens of serpents in these families.

  4. Juvenile psammomatoid ossifying fibroma: An unusual case report

    Directory of Open Access Journals (Sweden)

    Nidhi Yadav

    2013-01-01

    Full Text Available Ossifying fibromas are well-demarcated benign fibro-osseous tumors of craniofacial skeleton most often in the jaws. It has two types, conventional and juvenile ossifying fibroma (JOF. JOF is considered a separate entity from ossifying fibroma due to its locally aggressive behavior and tendency to occur at a young age. Two subtypes of JOF, i.e., juvenile psammomatoid ossifying fibroma (JPOF and juvenile trabecular ossifying fibroma have been identified on the basis of histology 70% of the JPOF occur in the paranasal sinuses, 20% in the maxilla and only 10% in the mandible. Here, we report a case of JPOF in a 14-year-old girl causing an asymptomatic expansile swelling in the right mandibular posterior region, which is a rare site for this tumor.

  5. Microcirculatory disorders in scleroderma systematica: an association with vascular wall stiffness

    Directory of Open Access Journals (Sweden)

    Ulyana Yuryevna Ruzhentsova

    2013-01-01

    Full Text Available Objective: to study the specific features of regulation of peripheral vascular tone and their association with the endothelial structure and function of large vessels in patients with scleroderma systematica (SDS. Subjects and methods. The investigation enrolled 25 patients with SDS (mean age, 47±2.6 years; mean disease duration, 8.3+1.7 years and 15 apparently healthy individuals matched for age and gender. Comprehensive examination involved laboratory and instrumental studies, laser Doppler study to evaluate endothelium-dependent and endothelium-independent vasodilation, as well as applanation tonometry calculating the pulse wave velocity and augmentation index. Results. All the patients were found to have impaired peripheral vascular responsiveness as compared to the controls. The examination established a relationship between the magnitude of endothelium-dependent vasodilation and the stiffness index of large vessels. There was no association between endothelium-independent vasodilation and vascular elasticity parameters.

  6. Applying the ACR/EULAR Systemic Sclerosis Classification Criteria to the Spanish Scleroderma Registry Cohort.

    Science.gov (United States)

    Sáez-Comet, Luis; Simeón-Aznar, Carmen Pilar; Pérez-Conesa, Mercedes; Vallejo-Rodríguez, Carmen; Tolosa-Vilella, Carles; Iniesta-Arandia, Nerea; Colunga-Argüelles, Dolores; Egurbide-Arberas, Maria Victoria; Ortego-Centeno, Norberto; Vargas-Hitos, José Antonio; Freire-Dapena, Mayka; Rubio-Rivas, Manuel; Ríos-Blanco, Juan José; Trapiella-Martínez, Luis; Fonollosa-Pla, Vicent

    2015-12-01

    To compare American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria for systemic sclerosis (SSc) with previous American Rheumatology Association (ARA) criteria. This was a cross-sectional multicenter study comparing sensitivity of both criteria in the cutaneous subsets in the Spanish scleroderma registry (RESCLE) cohort. In 1222 patients with SSc, the most prevalent items were Raynaud phenomenon (95%), skin thickening (91%), and abnormal capillaroscopy (89%). ARA criteria classified as SSc 63.5% of all patients, and 63%, 100%, 11.2%, and 0% in the limited, diffuse, sine, and pre-SSc subsets, respectively. ACR/EULAR criteria classified 87.5% of all patients and 98.5%, 100%, 41.8%, and 15.9% in the same subsets, respectively. ACR/EULAR criteria are more sensitive than ARA criteria, especially in limited, sine, and pre-SSc subsets.

  7. Bilateral linear scleroderma "en coup de sabre" associated with facial atrophy and neurological complications

    Directory of Open Access Journals (Sweden)

    Altmeyer Peter

    2001-12-01

    Full Text Available Abstract Background Linear scleroderma "en coup de sabre" (LSCS usually affects one side of the face and head in the frontoparietal area with band-like indurated skin lesions. The disease may be associated with facial hemiatrophy. Various ophthalmological and neurological abnormalities have been observed in patients with LSCS. We describe an unusual case of LSC. Case presentation A 23 year old woman presented bilateral LSCS and facial atrophy. The patient had epileptic seizures as well as oculomotor and facial nerve palsy on the left side which also had pronounced skin involvement. Clinical features of different stages of the disease are presented. Conclusions The findings of the presented patient with bilateral LSCS and facial atrophy provide further evidence for a neurological etiology of the disease and may also indicate that classic progressive facial hemiatrophy (Parry-Romberg syndrome and LSCS actually represent different spectra of the same disease.

  8. Specificity and sensitivity of esophageal motor abnormality in systemic sclerosis (scleroderma) and related diseases: a cineradiographic study.

    Science.gov (United States)

    Campbell, W L; Schultz, J C

    1986-01-01

    Using cineradiography, we studied esophageal peristalsis in patients with suspected systemic sclerosis and related diseases, and in patients without known esophageal disease who had been referred for upper gastrointestinal series. Patterns of peristaltic abnormality were identified, and specificity and sensitivity values calculated. Esophageal aperistalsis was 100% specific, and consistently weak peristalsis after all, swallows was 88% specific for systemic sclerosis. Specificity decreased to 70% in patients over age 60. Abnormal peristalsis after some, but not all, swallows had only 52% specificity. Consistently abnormal peristalsis (absent or diminished) was 67% sensitive for scleroderma. Although a high sensitivity value (87%) for cineradiography can be obtained by considering any peristaltic abnormality as a sign of scleroderma, this is achieved at the price of an undesirably high number of false positives (specificity 40%).

  9. Anti-neutrophil cytoplasmic antibody negative crescentic paucimmune glomerulonephritis in a case of scleroderma with systemic lupus erythematosus overlap

    Directory of Open Access Journals (Sweden)

    Rohit Tewari

    2016-01-01

    Full Text Available Renal Involvement in scleroderma is a known problem and the manifestations are well described. Renal involvement in systemic lupus erythematosus (SLE is also well known. However, in scleroderma and SLE overlap syndrome, the renal findings may vary being a combination of features of immune complex mediated glomerulonephritis as well as thrombotic microangiopathy. We report a case in which the renal manifestation in such a situation was of a focal necrotising pauci-immune glomerulonephritis with crescents, anti-neutrophil cytoplasmic antibody negative. To the best of our knowledge, such manifestations have not been described before. Renal dysfunction in a normotensive setting in such a case should direct one towards evaluation for other causes and should prompt a kidney biopsy. This would be valuable in delineating the pathological process in the kidney and would help in guiding therapy.

  10. Fixed Implant Supported Rehabilitation of Partially Edentulous Posterior Maxilla in a Patient With Systemic Scleroderma: A Case Report.

    Science.gov (United States)

    Baptist, Benjamin A

    2016-02-01

    Systemic Scleroderma (SSc) is an autoimmune disease that affects connective tissue, resulting in hardening skin, reduced vascular perfusion, gingival fibrosis, enlarged periodontal ligament, xerostomia, and trigeminal neuralgia. Secondary effects, including reduced oral opening and reduced manual dexterity may exacerbate the primary effects. Severe bone loss and premature tooth loss are common eventualities of SSc. Removable prosthetics can be a tedious option for these patients as the progression of the disease often leads to the impossibility of obtaining minimal standards of care, including stability, retention, and hygienic maintainability. Implant treatment of patients with Systemic Scleroderma is poorly documented, and common medications used to treat SSc have been considered relative contraindications to the prescription of dental implant therapy. This report describes 1 case after 2 years in function, where dental implants were successfully utilized to offer definitive fixed rehabilitation to a patient with SSc.

  11. Gemcitabine-induced hemolytic uremic syndrome mimicking scleroderma renal crisis presenting with Raynaud's phenomenon, positive antinuclear antibodies and hypertensive emergency.

    Science.gov (United States)

    Yamada, Yuichiro; Suzuki, Keisuke; Nobata, Hironobu; Kawai, Hirohisa; Wakamatsu, Ryo; Miura, Naoto; Banno, Shogo; Imai, Hirokazu

    2014-01-01

    A 58-year-old woman who received gemcitabine for advanced gallbladder cancer developed an impaired renal function, thrombocytopenia, Raynaud's phenomenon, digital ischemic changes, a high antinuclear antibody titer and hypertensive emergency that mimicked a scleroderma renal crisis. A kidney biopsy specimen demonstrated onion-skin lesions in the arterioles and small arteries along with ischemic changes in the glomeruli, compatible with a diagnosis of hypertensive emergency (malignant hypertension). The intravenous administration of a calcium channel blocker, the oral administration of an angiotensin-converting enzyme inhibitor and angiotensin II receptor blocker and the transfusion of fresh frozen plasma were effective for treating the thrombocytopenia and progressive kidney dysfunction. Gemcitabine induces hemolytic uremic syndrome with accelerated hypertension and Raynaud's phenomenon, mimicking scleroderma renal crisis.

  12. JUVENILE RHEUMATOID ARTHRITIS

    Directory of Open Access Journals (Sweden)

    I N Sartika

    2012-11-01

    Full Text Available Juvenile rheumatoid arthritis (JRA is the most common rheumatic condition in children. JRA is defined as persistent arthritis in 1 or more joints for at least 6 weeks, with the onset before age 16 years. The etiology of JRA is unknown. Antigen activated CD4+ T cell stimulate monocytes, macrophages, and synovial fibroblasts to produce the cytokines Interleukin-1 (IL-1, IL-6, and tumor necrosis factor ? (TNF-? and to secrete matrix metalloproteinases, which lead to chronic inflammation due to infiltration of inflammatory cell, angiogenesis, destruction of cartilage and bone with pannus formation. The 3 major subtypes of JRA are based on the symptoms at disease onset and are designated systemic onset, pauciarticular onset, and polyarticular onset. For all patients, the goals of therapy are to decrease chronic joint pain and suppress the inflammatory process. Poor prognostic have been observed in patients with polyarticular onset, rheumatoid factor, persistent morning stiffness, tenosynovitis, involvement of the small joints, rapid appearance of erosions, active late onset childhood, subcutaneous nodules, or antinuclear antibody.

  13. Cadherin-11 Regulation of Fibrosis through Modulation of Epithelial-to-Mesenchymal Transition: Implications for Pulmonary Fibrosis in Scleroderma

    Science.gov (United States)

    2016-05-01

    12 Approved Through 8/31/2015) Page 9 Figure 13. Soluble Cad-11 ELISA detects human (blue) and mouse (red) Cad-11 Figure 14. Serum cadherin...Determine the circulating levels of cadherin-11 in scleroderma patients with interstitial lung disease . In the past year a lot of effort has been...lung disease . At the outset of these experiments, there was not a commercial Cad-11 ELISA. Therefore, we developed an ELISA and optimize our

  14. Juveniles Are More Resistant to Warming than Adults in 4 Species of Antarctic Marine Invertebrates.

    Science.gov (United States)

    Peck, Lloyd S; Souster, Terri; Clark, Melody S

    2013-01-01

    Juvenile stages are often thought to be less resistant to thermal challenges than adults, yet few studies make direct comparisons using the same methods between different life history stages. We tested the resilience of juvenile stages compared to adults in 4 species of Antarctic marine invertebrate over 3 different rates of experimental warming. The species used represent 3 phyla and 4 classes, and were the soft-shelled clam Laternula elliptica, the sea cucumber Cucumaria georgiana, the sea urchin Sterechinus neumayeri, and the seastar Odontaster validus. All four species are widely distributed, locally abundant to very abundant and are amongst the most important in the ecosystem for their roles. At the slowest rate of warming used (1°C 3 days(-1)) juveniles survived to higher temperatures than adults in all species studied. At the intermediate rate (1°C day(-1)) juveniles performed better in 3 of the 4 species, with no difference in the 4(th), and at the fastest rate of warming (1°C h(-1)) L. elliptica adults survived to higher temperatures than juveniles, but in C. georgiana juveniles survived to higher temperatures than adults and there were no differences in the other species. Oxygen limitation may explain the better performance of juveniles at the slower rates of warming, whereas the loss of difference between juveniles and adults at the fastest rate of warming suggests another mechanism sets the temperature limit here.

  15. Assessing body image in patients with systemic sclerosis (scleroderma): validation of the adapted Satisfaction with Appearance Scale.

    Science.gov (United States)

    Heinberg, Leslie J; Kudel, Ian; White, Barbara; Kwan, Amy; Medley, Keya; Wigley, Fredrick; Haythornthwaite, Jennifer

    2007-03-01

    People with scleroderma often experience disfiguring appearance-related changes in socially visible and interpersonally salient areas. Although disfigurement can lead to body image dissatisfaction, this phenomenon has not been well investigated due to the lack of a disfigurement-specific measure. The Satisfaction With Appearance (SWAP) scale, previously developed in burn survivors, was adapted and administered to 254 participants with scleroderma to evaluate its psychometric integrity and its validity for use in a different medical population that experiences changes in appearance. Principal component analysis revealed two factors - Subjective Dissatisfaction and Perceived Social Impact - rather than the four found in burn victims. Excellent estimates of internal consistency and temporal stability and strong evidence for the reliability of the two-factor solution were found. The resulting factor structure in a scleroderma population suggests that differing medical conditions may create alternate constellations of BID, reflects the need for body image researchers to assess psychometrics across medical populations and may have clinical implications for BID interventions.

  16. A prospective study of the use of botulinum toxin injections in the treatment of Raynaud's syndrome associated with scleroderma.

    Science.gov (United States)

    Uppal, L; Dhaliwal, K; Butler, P E

    2014-10-01

    Raynaud's syndrome contributes to the pain, paraesthesia, ulceration, and gangrene of scleroderma. Botulinum toxin has been shown to improve digital perfusion in patients with Raynaud's. This is the first study to objectively assess hand function following this treatment in patients with scleroderma. Twenty patients were treated with 100 units of botulinum toxin injected into the hand. An assessment of hand function and symptoms was performed prior to injection and then 8-12 weeks later. The outcomes assessed were change in pain, appearance, cold intolerance, pinch and power grip, ranges of movement, and Disabilities of the Arm, Shoulder and Hand (DASH) score. In total, 80% of patients reported an overall improvement in their symptoms, reduction in pain, and improved DASH score and 65% reported improvement in cold intolerance. Overall, 90% showed an improvement in pinch grip and 65% an improvement in power grip. Objective parameters were statistically significantly improved; however, subjective outcomes only showed a trend. We have found botulinum toxin to be an effective treatment for Raynaud's syndrome secondary to scleroderma. © The Author(s) 2013.

  17. Juveniles tried as adults: the age of the juvenile matters.

    Science.gov (United States)

    Semple, Jaclyn K; Woody, William Douglas

    2011-08-01

    Serious juvenile crimes require evaluation of a child as a criminal defendant in adult court. In such cases, it is crucial to understand jurors' attitudes, biases, and ability to follow legal instructions and maintain fairness. 308 undergraduate psychology students served as mock jurors, were randomly separated into four groups, and each group read the same realistic summary of a trial with the defendant's age presented as 13, 15, 17, or 21 years. Participants were asked to render guilty or not guilty verdicts and, if guilty, to suggest sentences. Chi-squared analysis indicated 13- and 15-year-old defendants were convicted less often than 17- and 21-year-old defendants, showing that jurors distinguished between juvenile defendants of different ages, but not minors and adults as defined by law. Additional analysis showed that age did not affect sentencing recommendations. Decision processes jurors use for juveniles tried as adults are discussed.

  18. Forensic aspects of juvenile violence.

    Science.gov (United States)

    Haller, L H

    2000-10-01

    The juvenile justice system was created because it was recognized that youthful offenders needed to be managed differently from adults. They were to receive habilitation services instead of punishment. It is now more than a century since the creation of the first juvenile court. After 67 years, the US Supreme Court, in Kent v United States stated that the model was not working because juveniles in the criminal justice system received no treatment and they had no rights. Because the issue that had been appealed was the lack of rights (not lack of treatment), the Court mandated that juveniles, like adults, be given certain rights. The following year, in In re Gault, the Court expanded these rights. Subsequent Supreme Court cases have dealt with these kinds of issues--that is, whether juvenile offenders are entitled to the same rights as adults and subject to the same penalties. The Supreme Court has never heard a "right to treatment" case, which is the other part of the juvenile court system. Cases have been brought in lower courts (e.g., Nelson v. Heyne, 1972) alleging inadequate treatment services, but no national impact has resulted. Thus, in general, children in the juvenile court system do not have an enforceable right to treatment and can obtain only what services are available in their jurisdictions. The services often are woefully inadequate. Sentencing a youth to probation, with the requirement that he or she participate in counseling or mental health treatment, is meaningless if services are not available. Community-based, model programs that provide effective treatment do exist. They are, as yet, the rare exception rather than the norm and, therefore, are not available to most youthful offenders. Incarcerated juveniles, obviously, cannot avail themselves of community programs. Litigation to give these youth the same rights as adults in penal institutions is not the answer because incarcerated adults don't have a right to treatment, only a right to be free

  19. The Heterogeneity of Juvenile Myositis

    Science.gov (United States)

    Rider, Lisa G.

    2007-01-01

    Juvenile myositis is a heterogeneous group of systemic autoimmune diseases, in which clinical and serologic subgroups result in subsets of patients with distinct clinical manifestations, disease courses, immunogenetic associations, responses to therapy, and prognoses. A newly identified autoantibody of unknown specificity, anti-p155, is myositis-associated and seen in up to 20 – 30% of juvenile and adult DM patients. HLA DRB1*0301 and its linked allele DQA1*0501 have been identified as the major immunogenetic risk factor for juvenile and adult DM in both European- and African- American patients, and DQA1*0301 is an additional risk factor in European American patients. Several DQA1 alleles also are protective for juvenile DM. Environmental risk factors are poorly understood, but growing evidence suggests a role for infectious agents and ultraviolet radiation. The current therapy of juvenile DM consists of corticosteroids and other immunosuppressive agents, with the adjunctive treatment of cutaneous manifestations and rehabilitation. Therapeutic trials of biologic agents, including anti-TNFα and anti-CD20, may aid in developing promising new therapies for these disorders. PMID:17317616

  20. THE MAIN RESULTS OF COOPERATION BETWEEN THE V.A. NASONOVA RESEARCH INSTITUTE OF RHEUMATOLOGY AND RESEARCH CENTERS OF EUROPE WITHIN THE EUSTAR (EULAR SCLERODERMA TRIALS AND RESEARCH GROUP IN SYSTEMIC SCLEROSIS

    Directory of Open Access Journals (Sweden)

    L. P. Ananyeva

    2014-01-01

    Full Text Available The major goals of the EUSTAR (EULAR Scleroderma Trials And Research group are to coordinate and centralize systemic sclerosis (SS researches in Europe, to reach a consensus in the standards of evidence-based medicine for patient management, to enhance treatment efficacy, and to improve quality of life in patients and prognosis. Under the aegis of the EUSTAR, more than 100 centers have combined their efforts to solve basic research problems, to study the clinical aspects of the disease, and to conduct clinical trials. The major task of their activities has been to follow-up and timely treat SS patients examined according to a standard protocol, and to accumulate information in the unified European database that includes more than 10,000 patients now. The V.A. Nasonova Research Institute of Rheumatology has formed a cohort of 220 patients with SS, of whom 90–100 persons are annually followed up.The main joint projects implemented have shown the present-day course of SS, studied the causes of death, and characteristics of patient subgroups (juvenile scleroderma, SS in the elderly, damage of the lung, heart, gastrointestinal tract, etc.. New diagnostic criteria for SS and an algorithm for its very early, preclinical diagnosis have been proposed. An analysis of the first 3656 patents with SS has revealed the major clinical characteristics of its diffuse and limited forms at the present stage. Pooled patient data have been used in international multicenter projects whose results are given in joint publications. The results of EUSTAR projects with the participation of the V.A. Nasonova Research Institute of Rheumatology are covered in 28 articles. Analysis of the results of EULAR/EUSTAR researches gives grounds to hold that the early diagnosis and timely detection of visceral injuries and the use of current therapy standards may assist in improving the quality of life of patients as well as in reducing the severity of SS and its

  1. Juvenile Correctional Institutions Library Services: A Bibliography.

    Science.gov (United States)

    McAlister, Annette M.

    This bibliography lists citations for 14 articles, books, and reports concerned with library services in juvenile correctional institutions. A second section lists 21 additional materials on adult correctional libraries which also contain information relevant to the juvenile library. (KP)

  2. The Correlation between endothelin-1 antibody plasma concentrations in patients with scleroderma and different manifestations of the disease

    Directory of Open Access Journals (Sweden)

    Aghaei M

    2011-03-01

    Full Text Available "n Normal 0 false false false EN-US X-NONE AR-SA MicrosoftInternetExplorer4 st1":*{behavior:url(#ieooui } /* Style Definitions */ table.MsoNormalTable {mso-style-name:"Table Normal"; mso-tstyle-rowband-size:0; mso-tstyle-colband-size:0; mso-style-noshow:yes; mso-style-priority:99; mso-style-qformat:yes; mso-style-parent:""; mso-padding-alt:0cm 5.4pt 0cm 5.4pt; mso-para-margin:0cm; mso-para-margin-bottom:.0001pt; mso-pagination:widow-orphan; font-size:11.0pt; font-family:"Calibri","sans-serif"; mso-ascii-font-family:Calibri; mso-ascii-theme-font:minor-latin; mso-fareast-font-family:"Times New Roman"; mso-fareast-theme-font:minor-fareast; mso-hansi-font-family:Calibri; mso-hansi-theme-font:minor-latin; mso-bidi-font-family:Arial; mso-bidi-theme-font:minor-bidi;} Background: Systemic scleroderma (SSc is a generalized connective tissue disorder of unknown origin which most notably is characterized by skin thickening and organ damage. Endothelin-1 (ET-1 antibody plays a role in skin fibrosis. The aim of this study was to determine the prevalence and correlation of different manifestations of SSc with ET-1 plasma levels."n"nMethods: This cross-sectional analytical study was conducted on 95 patients (91 women and four men with scleroderma in 2006. The patients had been referred to the Rheumatology Clinic of Shariati Hospital in Tehran, Iran. The demographic data and signs and symptoms were entered in a questionnaire and endothelin-1 concentrations were measured. "n"nResults: The mean age of the patients was 38±12.29 years. Diffuse cutaneous SSc (dcSSc was diagnosed in 52 and limited cutaneous SSc (lcSSc in 43 patients. Raynaud's phenomenon (91% was the most common manifestation in the patients. The relationship between the resorption of terminal phalanges due to fibrosis with the plasma concentration of Endothelin-1 was statistically significant (p=0.001. Pitting ulcers had significant relationships with endothelin-1 concentrations too (p<0

  3. The thickness of the A1 pulleys reflects the disability of hand mobility in scleroderma. A pilot study using high-frequency ultrasound

    Energy Technology Data Exchange (ETDEWEB)

    Tagliafico, Alberto, E-mail: atagliafico@sirm.org [Department of Radiology, National Institute for Cancer Research, Genova (Italy); Panico, Nicoletta [Division of Immunology, Department of Internal Medicine, University of Genova, Genova (Italy); Serafini, Giovanni [Department of Radiology, Santa Corona Hospital, Pietra Ligure (Italy); Ghio, Massimo [Division of Immunology, Department of Internal Medicine, University of Genova, Genova (Italy); Martinoli, Carlo [Department of Radiology, University of Genova, Genova (Italy)

    2011-02-15

    Background: Hand involvement in scleroderma is a serious concern. Clinical tests to asses hand dysfunction are based on the experience of the clinician. Objective: To asses if utrasonographic (US) measurement of A1 pulley thickness may be used as an indicator of hand mobility in scleroderma. Materials and methods: Institutional review board approval and patient informed consent was obtained. Twenty-eight patients affected suffering from scleroderma and 40 healthy controls were prospectively evaluated by two blinded radiologists with US, with a transducer operating at 17 MHz. A1 pulley thickness was measured and correlated with the Hand Mobility in Scleroderma Test (HAMIS) and disease duration. Results: The thickness of the A1 pulley was greater in sclerodermic patients than in controls (p < 0.05). Intra and inter-observer agreement were better for ultrasound (0.94;0.88) than for HAMIS tests (0.71;0.70). A good correlation between pulley thickness, hand mobility and disease duration was found (r = 0.78, p < 0.018; r = 0.54, p < 0.05). Conclusion: A1 pulley thickness measured on ultrasound correlates with hand mobility and disease duration. Ultrasound is an useful tool to evaluate hand disability in scleroderma.

  4. Juvenile technologies in foreign publications

    Directory of Open Access Journals (Sweden)

    Shpagina E.M.

    2012-09-01

    Full Text Available The article provides the review of foreign publications, concerning the juvenile technologies used in France, Canada, Germany and Switzerland. The paper presents legal, social and psychotherapeutic aspects of juvenile judiciary in foreign countries. The authors paid special attention to the complexity of approaches to young children and teenagers who found themselves in complicated life circumstances or got into trouble with the law. The article gives examples of using the following techniques: cognitive-behavioral intervention, mediation, family therapy (including family background and family history, relations theory, narrative practices, utilization of «emotional intelligence» resources.

  5. Evolution of Juvenile Ankylosing Spondylitis

    Directory of Open Access Journals (Sweden)

    Ye.V. Prohorov

    2013-03-01

    Full Text Available Evolution of juvenile ankylosing spondylitis tend to follow a more frequent involvement in the pathological process of elbow and ankle joints, development of enthesiopathies, changes of intraarticular meniscal horns, forming of Baker’s cysts, cartilage flaps and systemic osteoporosis, and total value of all these signs 13 times exceeds thereof in patients with with the debut of disease in adulthood, but for juvenile ankylosing spondylitis vertebral lesion is less common. Age dimorphism of the use of certain groups of drugs and physiotherapy facilities is observed.

  6. On the Prevention of Juvenile Crime

    Science.gov (United States)

    Lelekov, V. A.; Kosheleva, E. V.

    2008-01-01

    Crimes committed by juveniles are among the most urgent social problems. Juvenile crime is as prevalent as crime itself is, and it has not been solved completely in any society and cannot be solved through law enforcement measures alone. In this article, the authors discuss the dynamics and structure of juvenile crime in Russia and present data…

  7. School-Related Characteristics of Male Juveniles.

    Science.gov (United States)

    Sapp, Gary L.; Abbott, Gypsy A.

    School-related characteristics of 256 male juveniles under the jurisdiction of a Family Court system were examined by perusing court records and conducting individual interviews with the juveniles. Results indicated that most juveniles last attended eighth grade, more than 81% had failed at least once, and more than half had fought frequently at…

  8. Intensive Reading Instruction in Juvenile Correctional Settings

    Science.gov (United States)

    Williams, Jacob L.; Wexler, Jade; Roberts, Greg; Carpenter, Clint

    2011-01-01

    Despite 60 years of evidence linking juvenile illiteracy and delinquency, practitioners and policymakers have been painfully slow in the implementation of evidence-based reading interventions for incarcerated juveniles. We will present the Texas Juvenile Justice Tiered Instructional Model, an evidence-based reading program model created…

  9. Sex Differences in Attributions of Juvenile Delinquency.

    Science.gov (United States)

    Sagatun, Inger J.

    This paper is an application of attribution theory to the processing of juvenile delinquents in an attempt to understand the differential treatment of female and male offenders within the juvenile justice system. The paper explores the attributions of juvenile delinquency both by male and female minors, by male and female parents, and by male and…

  10. Do Juveniles Bully More than Young Offenders?

    Science.gov (United States)

    Ireland, Jane L.

    2002-01-01

    Study compares bullying behavior among juvenile and young offenders. Ninety-five male juvenile and 196 male young offenders completed two questionnaires, measuring bullying directly and behaviors indicative of "being bullied" or of "bullying others". Juveniles perceived a higher extent of bullying and reported significantly…

  11. The Juvenile Court: Changes and Challenges.

    Science.gov (United States)

    Feld, Barry C.

    2000-01-01

    Explores the changes in the juvenile court system, in particular, the juvenile waiver and sentencing laws, as it transformed from a social welfare agency into a type of criminal court system for young offenders. Addresses whether states should create an integrated juvenile and criminal justice system. (CMK)

  12. Reforming Our Expectations about Juvenile Justice

    Science.gov (United States)

    Rodriguez, Pamela F.; Baille, Daphne M.

    2010-01-01

    Typing the term "juvenile justice reform" into a Google[TM] search will result in 60 pages of entries. But what is meant by juvenile justice reform? What does it look like? How will one know when it is achieved? This article defines juvenile justice reform, discusses the principles of effective reform, and describes the practice of juvenile…

  13. Juvenile papillomatosis of the breast in young male: a case report.

    Science.gov (United States)

    Sanguinetti, A; Fioriti, L; Brugia, M; Roila, F; Farabi, R; Sidoni, A; Avenia, N

    2011-01-01

    Juvenile papillomatosis of the breast ("Swiss cheese disease'') is a benign localized proliferative condition of the breast which occurs almost exclusively in young adult women. Patients with this lesion often have a family history of breast carcinoma, but rarely carcinoma may coexist with the lesion at the time of diagnosis. We present a case of a young male with juvenile papillomatosis of the breast. The pathology and clinical management of this rare lesion is discussed.

  14. Antifibrotic effects of crocetin in scleroderma fibroblasts and in bleomycin-induced sclerotic mice

    Directory of Open Access Journals (Sweden)

    Yinghua Song

    2013-10-01

    Full Text Available OBJECTIVE: To investigate the antifibrotic effects of crocetin in scleroderma fibroblasts and in sclerotic mice. METHODS: Skin fibroblasts that were isolated from three systemic scleroderma (SSc patients and three healthy subjects were treated with crocetin (0.1, 1 or 10 μM. Cell proliferation was measured with an MTT assay. Alpha-smooth muscle actin was detected via an immunohistochemical method. Alpha 1 (I procollagen (COL1A1, alpha 1 (III procollagen (COL3A1, matrix metalloproteinase (MMP-1 and tissue inhibitor of matrix metalloproteinase (TIMP-1 mRNA levels were measured using real-time PCR. SSc mice were established by the subcutaneous injection of bleomycin. Crocetin (50 mg/kg/d was injected intraperitoneally for 14 days. Dermal thickness and lung fibrosis were assessed with Masson's trichrome staining. Plasma ET-1 was detected with an enzyme-linked immunosorbent assay (ELISA. Skin and lung ET-1 and COL1A1 mRNA levels were measured via real-time PCR. RESULTS: Crocetin inhibited the proliferation of SSc and normal fibroblasts, an effect that increased with crocetin concentration and incubation time. Crocetin decreased the expression of α-SMA and the levels of mRNA for COL1A1, COL3A1 and matrix metalloproteinase-1, while crocetin increased TIMP-1 mRNA levels in both SSc and normal fibroblasts. Skin and lung fibrosis was induced, and the levels of ET-1 in the plasma, skin and lungs were elevated in bleomycin-injected mice. Crocetin alleviated the thickening of the dermis and lung fibrosis; decreased COL1A1 mRNA levels in the skin and lung; and simultaneously decreased ET-1 concentrations in the plasma and ET-1 mRNA levels in the skin and lungs of the bleomycin-induced sclerotic mice, especially during the early phase (weeks 1-3. CONCLUSION: Crocetin inhibits cell proliferation, differentiation and collagen production in SSc fibroblasts. Crocetin alleviates skin and lung fibrosis in a bleomycin-induced SSc mouse model, in part due to a

  15. Lower extremity lipedema, upper extremity lipodystrophy and severe calcinosis complicating juvenile dermatomyositis.

    Science.gov (United States)

    Pavlov-Dolijanovic, Slavica R; Vujasinovic Stupar, Nada Z; Gavrilov, Nikola; Seric, Srdjan

    2014-11-01

    Juvenile dermatomyositis (JDM) is a rare but complex and potentially life-threatening autoimmune disease of childhood. Significant proportions of patients have residual weakness, muscle atrophy, joint contractures, and calcinosis. Recently, new clinical findings, such as lipodystrophy accompanied with increased fat deposition in certain areas, have been reported. So far, it is not known whether the redistribution of body fat may be the type of lipedema of lower extremity. We describe a 39-year-old woman who was diagnosed with JDM at the age of 7. Later she developed symmetrical lipodystrophy of upper extremities and symmetrical lipedema of lower extremities (making 2 and 58.3 % of total body fat mass, respectively), with multiple calcified nodules in the subcutaneous tissues. These nodules gradually increased in size despite therapy. Capillaroscopy findings showed scleroderma-like abnormalities. ANA and anti-U1RNP antibodies were positive. Similar cases with simultaneous occurrence of the lipedema of lower extremities, lipodystrophy of upper extremities, and severe calcinosis complicating JDM have not been published so far. We showed that the calcinosis and lipodystrophy were associated with short duration of active disease. Also, we display case that raises the question whether it is possible overlapping autoimmune diseases revealed during follow-up.

  16. Juvenile rheumatoid arthritis: therapeutic perspectives.

    Science.gov (United States)

    Chikanza, Ian C

    2002-01-01

    Juvenile rheumatoid arthritis (JRA) is the most common childhood chronic systemic autoimmune inflammatory disease. The therapeutic approach to JRA has, to date, been casual and based on extensions of clinical experiences gained in the management of adult rheumatoid arthritis (RA). The physiology of inflammation has been systemically studied and this has led to the identification of specific therapeutic targets and the development of novel approaches to the management of JRA. The classical treatments of the disease such as methotrexate, sodium aurothiomalate and sulfasalazine, are not always effective in controlling RA and JRA. This has necessitated the development of novel agents for treating RA, most of which are biological in nature and are targeted at specific sites of the inflammatory cascades. These biological therapeutic strategies in RA have proved successful and are being applied in the management of JRA. These developments have been facilitated by the advances in molecular biology which have heralded the advent of biodrugs (recombinant proteins) and gene therapy, in which specific genes can be introduced locally to enhance in vivo gene expression or suppress gene(s) of interest with a view to down-regulating inflammation. Some of these biodrugs, such as anti-tumor necrosis factor alpha (anti-TNFalpha), monoclonal antibodies (infliximab, adalimumab), TNF soluble receptor constructs (etanercept) and interleukin-1 receptor antagonist (IL-1Ra) have been tested and shown to be effective in RA. Etanercept has now been licensed for JRA. Clinical trials of infliximab in JRA are planned. Studies show that the clinical effects are transient, necessitating repeated treatments and the risk of vaccination effects. Anti-inflammatory cytokines such as IL-4, IL-10, transforming growth factor-beta and interferon-beta (IFN-beta) are undergoing clinical trials. Many of these agents have to be administered parenterally and production costs are very high; thus, there is a need

  17. Juvenile xanthogranuloma of the proximal nail fold.

    Science.gov (United States)

    Piraccini, Bianca Maria; Fanti, Pier Alessandro; Iorizzo, Matilde; Tosti, Antonella

    2003-01-01

    An 18-month-old Caucasian boy presented with a firm 0.5 mm nodule, pink-red in color, with a yellow hue and some telangiectases on the surface, localized on the right thumbnail. The nodule involved all of the proximal nail fold and covered the proximal third of the nail. Pathology showed a dense dermal infiltrate of histiocytes, some of which had foamy nuclei, and multinucleated Touton giant cells. The lesion progressively decreased in size and had completely disappeared after 3 years. Periodic follow-up was important not only to monitor evolution of the juvenile xanthogranuloma, but also to avoid excessive growth of the lesion with possible definitive nail matrix damage.

  18. Cytokines in juvenile rheumatoid arthritis (JRA).

    Science.gov (United States)

    Mangge, H; Schauenstein, K

    1998-06-01

    Juvenile rheumatoid arthritis (JRA), unlike rheumatoid arthritis of adulthood (RA), is a heterogenous disease comprising at least five subtypes that differ in clinical course and prognosis, and require different therapeutical approaches. As compared to RA, the production of local and systemic cytokines in JRA have not yet been as extensively investigated. In this article we review the available literature on cytokine expression in serum and synovial fluid in all five different subtypes of JRA. Even though the data are still fragmentary, the evidence so far suggests that the determination of serum cytokines yields relevant information as to clinical subtype and inflammatory activity of the disease. Furthermore, the cytokine data suggest that the pathogenesis of JRA may even by more heterogenous than defined by the clinical subtypes. Finally, future directions of research in this area are proposed, and-based on the latest results-arguments for (anti)cytokine therapies in JRA are critically discussed.

  19. Juvenile Justice and Substance Use

    Science.gov (United States)

    Chassin, Laurie

    2008-01-01

    Laurie Chassin focuses on the elevated prevalence of substance use disorders among young offenders in the juvenile justice system and on efforts by the justice system to provide treatment for these disorders. She emphasizes the importance of diagnosing and treating these disorders, which are linked both with continued offending and with a broad…

  20. Genetics in juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Albers, Heleen Marion

    2015-01-01

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

  1. Juvenile Courts. Creation and development

    Directory of Open Access Journals (Sweden)

    Montserrat GONZÁLEZ FERNÁNDEZ

    2013-11-01

    Full Text Available This paper studies the creation of Juvenile or Children's Courts in Spain, analysing their reasons and aims, as well as the ethical and political connotations present on their way of acting. Their history and the one of the institutions that complement them is built from the legislation, writings and ideas of their promoters.

  2. Juvenile Diabetes and Rehabilitation Counseling.

    Science.gov (United States)

    Stone, J. Blair; Gregg, Charles H.

    1981-01-01

    Severe complications of diabetes are more likely to occur with the juvenile diabetic and problems of psychosocial adjustment are recurring and difficult. Implications for the rehabilitation counselor are discussed in terms of employment considerations, the effects of complications, genetic counseling, and cooperation with other professionals.…

  3. Case Report: Juvenile Tophaceous Gout

    Directory of Open Access Journals (Sweden)

    Seyma Gunes

    2016-01-01

    Full Text Available Gout is a metabolic disease that manifests as recurrent arthritis. Its incidance increases with age. Clinical findings include recurrent acute arthritis, tophus at joints and tissues, uricacid stones and gouty nephropathy. Tophi is a late period complication of arthritis. In this casereport we presented  a patient with early-onset juvenile tophaceous gout.

  4. Do juvenile Amphiprion ocellaris (Pisces

    DEFF Research Database (Denmark)

    Brolund, Thea Marie; Nielsen, Lis Engdahl; Arvedlund, Michael

    2003-01-01

    . This is contrary to the settling mechanisms of the damselfish D. aruanus and D. reticulatus, and of the temperate herring Clupea harengus. Hence the results emphasize the variation of sensory abilities and behaviours in fish larvae and juveniles. It is not an area prone for generalizations....

  5. [Sex-linked juvenile retinoschisis].

    Science.gov (United States)

    François, P; Turut, P; Soltysik, C; Hache, J C

    1976-02-01

    About 13 observations of sexe linked juvenile retinoschisis, the authors describe the ophthalmoscopic, fluorographic and functional aspects of the disease whose caracteristics are:--its sexe linked recessive heredity; --its clinical characterestics associating: a microcystic macular degeneration, peripheral retinal lesions, vitreous body alterations, --an electroretinogram of the negative type.

  6. Effects of bosentan on collagen type I synthesis on in vitro culture of scleroderma skin fibroblasts

    Directory of Open Access Journals (Sweden)

    S. Soldano

    2011-01-01

    Full Text Available The present study evaluated the effects of a non-selective endothelin (ETA/B receptors antagonist, on collagen type I (COLI synthesis on in vitro culture of scleroderma (SSc skin fibroblasts (Fb. Fb were obtained from skin biopsies of 6 female SSc patients (mean age 64. 1±6 years, after informed consent and Ethical Committee Approval. Cells were treated with endothelin-I [ET-I, 100nM] for 24 and 48 hrs, pre-treated for I hr with ETA/B receptors antagonist [10nM] alone or followed by ET-I for 24 and 48 hrs. Untreated Fb were used as controls. Immunocytochemistry and western blot analysis were performed to evaluate COLI synthesis. ET-I increased COLI synthesis both at 24 and 48 hrs when compared to controls. ETA/B receptor antagonost blocks the increased COLI synthesis ET-I-mediated both at 24 and 48 hrs vs. ET-I. Results showed that ET-I receptors blockage by ETA/B receptors antagonist might prevent the excessive synthesis of COLI, supporting its positive action in the management of skin fibrosis.

  7. Clinicopathologic assessment of arrhythmias in a case of scleroderma heart disease with sudden death.

    Science.gov (United States)

    Marinato, P G; Thiene, G; Menghetti, L; Buja, G F; Nava, A; Cecchetto, A; Rossi, L

    1981-01-01

    Electrophysiologic and histopathologic correlation has been carried out in a patient with scleroderma heart disease, affected by syncopal seizures, who died of recorded ventricular fibrillation. The electrophysiological investigation disclosed dysfunction of sinoatrial conduction, revealed by sinoatrial blocks and by an abnormal return cycle pattern after premature atrial beats. Atrial effective and functional refractory periods were increased and an unusual 'pseudo-Wenckebach' phenomenon between artificial stimulus and atrium was observed during atrial pacing. Intra-AV nodal conduction time was at normal upper limits and Wenckebach-type AV block was obtained on pacing the atrium at 100 beats/min. HV conduction was moderately prolonged in the presence of left anterior hemiblock. The histopathologic substrates of these electrophysiologic disturbances were fibrosis of the sinus node, disrupted internodal pathways and atrio-AV nodal connections, and left bundle branch atrophy. As far as fatal tachyarrhythmia is concerned, myofibrillar degeneration may have contributed to its pathogenesis. It is suggested that both lesions of the ordinary myocardium and specialized conduction system account for the electrical instability of sclerodermic patients.

  8. The Assessment of Health-Related Quality of Life in Scleroderma-Interstitial Lung Disease

    Directory of Open Access Journals (Sweden)

    Shahrzad M Lari

    2014-05-01

    Full Text Available Introduction: Pulmonary involvement is the most common cause of mortality and disability in patients with systemic sclerosis and it significantly affects the quality of life in these patients. Therefore, early diagnosis and treatment of pulmonary involvement seems necessary in patients with SSc. In this study, we aimed to assess the health-related quality of life (HRQoL in patients with Scleroderma-Interstitial Lung Disease (SSc-ILD and its relationship with pulmonary function parameters. Materials and Methods: Considering the inclusion and exclusion criteria, 25 patients with SSc-ILD were enrolled in this cross-sectional study from April 2012 to June 2013. Full tests of lung function, including body plethysmography and diffusing capacity of the lungs for carbon monoxide (DLCO, 6-minute walk distance (6MWD, and pulse oximetry were performed. The HRQoL was assessed using St. George’s and CAT questionnaires; also, dyspnea was evaluated for all the patients, using modified medical research council (MMRC scale. Afterwards, the relationship between the total scores of HRQoL questionnaires and the severity of lung disease was analyzed, based on the recorded variables. Results: The mean age of the patients was 40.36±9.50 years and the mean duration of the disease was 7.16±4.50 years. A statistically significant inverse correlation was observed between 6MWD (r=-0.50, P=0.01, DLCO (r=-0.67, P

  9. Analyzing HLA-G polymorphisms in children from women with scleroderma.

    Science.gov (United States)

    Picard, Christophe; Di Cristofaro, Julie; Azzouz, Doua F; Kanaan, Sami B; Roudier, Jean; Lambert, Nathalie C

    2013-04-01

    Embryos during pregnancy and organs during transplantation, express high levels of soluble HLA-G (sHLA-G) molecules for successful implantation and protection against maternal immune cells or recipient's cells. We and others have shown that women with scleroderma (SSc) carry cells/DNA arising from pregnancy, so-called fetal microchimerism (Mc) more often and in higher quantities than healthy women decades after delivery. We hypothesized that high levels of fetal Mc were the consequence of a fetus with a high sHLA-G profile, therefore that children from women with SSc would have this profile more often than children from healthy women. High sHLA-G secretor profile is influenced by at least two variations in the HLA-G 3' untranslated region (UTR): a 14 bp deletion in exon 8 and the presence of cysteine (C) in position +3142 and by one variation in the 5' Upstream Regulatory Region (URR) at position -725. By a previously developed three-step multiplex PCR SNaPshot method, we evaluated 16 HLA-G polymorphisms in DNA samples from the first-born children of 39 women with SSc and 32 healthy women. Contrary to expectations, children from women with SSc did not have a high sHLA-G profile, but rather the opposite. We discuss possible reasons for this result and future orientations for HLA-G studies in SSc.

  10. Distinguishing between limited systemic scleroderma-associated pseudo-obstruction and peritoneal dissemination.

    Science.gov (United States)

    Saigusa, Susumu; Inoue, Yasuhiro; Ohi, Masaki; Imaoka, Hiroki; Uratani, Ryo; Kobayashi, Minako; Kusunoki, Masato

    2015-12-01

    A 78-year-old woman receiving treatment for limited systemic scleroderma (SSc) underwent high anterior resection and partial liver resections for rectosigmoid colon cancer with multiple liver metastases. A year after surgery, an abdominal computed tomography (CT) demonstrated suspicion for peritoneal dissemination with an increase in ascites, and (18)F-fluorodeoxy glucose-positron emission tomography-CT was suggestive of carcinomatosis. We began to decompress the small intestine and administer octreotide. However, the intestinal obstruction did not improve. Although intestinal pseudo-obstruction caused by limited SSc was considered as a differential diagnosis, we performed an exploratory laparotomy because the possibility of peritoneal dissemination-associated obstruction could not be excluded. We observed a moderate amount of serous ascites and dilatation of the small intestine that was white in color, hard, and with limited contractility. There was no evidence of peritoneal dissemination nor of mechanical obstruction. Our experience thus shows the difficulty of distinguishing SSc-associated intestinal pseudo-obstruction from peritoneal dissemination.

  11. Gammadelta receptor bearing T cells in scleroderma: enhanced interaction with vascular endothelial cells in vitro.

    Science.gov (United States)

    Kahaleh, M B; Fan, P S; Otsuka, T

    1999-05-01

    In view of the documented perivascular mononuclear cell infiltration in the involved organs in scleroderma (SSc) and the reported accumulation of gammadelta-T cells in SSc skin and lung, we evaluated gammadelta-T cell interaction with endothelial cells (EC) in vitro. gammadelta- and alphabeta-T cells were isolated from BPMN of SSc patients with early diffuse disease and of matched control subjects by an immunomagnetic method after stimulation with mycobacterium lysate and interleukin-2 for 2 weeks. Lymphocyte adhesion, proliferation, and cytotoxicity to EC were investigated. SSc gammadelta-T cells adhered to cultured EC and proliferated at higher rates than control cells. Furthermore, significant EC cytotoxicity by SSc gammadelta was seen. The cytotoxicity was blocked by addition of anti-gammadelta-TCR antibody and by anti-granzyme A antibody but not by anti-MHC class I and II antibodies. Expression of granzyme A mRNA was seen in five/five SSc gammadelta-T cells and in one/five control cells. alphabeta-T cells from both SSc and control subjects were significantly less interactive with EC than gammadelta-T cells. The data demonstrate EC recognition by SSc gammadelta-T cells and propose gammadelta-T cells as a possible effector cell type in the immune pathogenesis of SSc.

  12. Parasitization by Scleroderma guani influences protein expression in Tenebrio molitor pupae.

    Science.gov (United States)

    Zhu, Jia-Ying; Wu, Guo-Xing; Ze, Sang-Zi; Stanley, David W; Yang, Bin

    2014-07-01

    Ectoparasitoid wasps deposit their eggs onto the surface and inject venom into their hosts. Venoms are chemically complex and they exert substantial impact on hosts, including permanent or temporary paralysis and developmental arrest. These visible venom effects are due to changes in expression of genes encoding physiologically relevant proteins. While the influence of parasitization on gene expression in several lepidopterans has been reported, the molecular details of parasitoid/beetle relationships remain mostly unknown. This shortcoming led us to pose the hypothesis that envenomation by the ectoparasitic ant-like bethylid wasp Scleroderma guani leads to changes in protein expression in the yellow mealworm beetle Tenebrio molitor. We tested our hypothesis by comparing the proteomes of non-parasitized and parasitized host pupae using iTRAQ-based proteomics. We identified 41 proteins that were differentially expressed (32↑- and 9↓-regulated) in parasitized pupae. We assigned these proteins to functional categories, including immunity, stress and detoxification, energy metabolism, development, cytoskeleton, signaling and others. We recorded parallel changes in mRNA levels and protein abundance in 14 selected proteins following parasitization. Our findings support our hypothesis by documenting changes in protein expression in parasitized hosts.

  13. Parasitization by Scleroderma guani influences expression of superoxide dismutase genes in Tenebrio molitor.

    Science.gov (United States)

    Zhu, Jia-Ying; Ze, Sang-Zi; Stanley, David W; Yang, Bin

    2014-09-01

    Superoxide dismutase (SOD) is an antioxidant enzyme involved in detoxifying reactive oxygen species. In this study, we identified genes encoding the extracellular and intracellular copper-zinc SODs (ecCuZnSOD and icCuZnSOD) and a manganese SOD (MnSOD) in the yellow mealworm beetle, Tenebrio molitor. The cDNAs for ecCuZnSOD, icCuZnSOD, and MnSOD, respectively, encode 24.55, 15.81, and 23.14 kDa polypeptides, which possess structural features typical of other insect SODs. They showed 20-94% identity to other known SOD sequences from Bombyx mori, Musca domestica, Nasonia vitripennis, Pediculus humanus corporis, and Tribolium castaneum. Expression of these genes was analyzed in selected tissues and developmental stages, and following exposure to Escherichia coli and parasitization by Scleroderma guani. We recorded expression of all three SODs in cuticle, fat body, and hemocytes and in the major developmental stages. Relatively higher expressions were detected in late-instar larvae and pupae, compared to other developmental stages. Transcriptional levels were upregulated following bacterial infection. Analysis of pupae parasitized by S. guani revealed that expression of T. molitor SOD genes was significantly induced following parasitization. We infer that these genes act in immune response and in host-parasitoid interactions.

  14. Quantitative nanohistological investigation of scleroderma: an atomic force microscopy-based approach to disease characterization

    Science.gov (United States)

    Strange, Adam P; Aguayo, Sebastian; Ahmed, Tarek; Mordan, Nicola; Stratton, Richard; Porter, Stephen R; Parekh, Susan; Bozec, Laurent

    2017-01-01

    Scleroderma (or systemic sclerosis, SSc) is a disease caused by excess crosslinking of collagen. The skin stiffens and becomes painful, while internally, organ function can be compromised by the less elastic collagen. Diagnosis of SSc is often only possible in advanced cases by which treatment time is limited. A more detailed analysis of SSc may provide better future treatment options and information of disease progression. Recently, the histological stain picrosirius red showing collagen register has been combined with atomic force microscopy (AFM) to study SSc. Skin from healthy individuals and SSc patients was biopsied, stained and studied using AFM. By investigating the crosslinking of collagen at a smaller hierarchical stage, the effects of SSc were more pronounced. Changes in morphology and Young’s elastic modulus were observed and quantified; giving rise to a novel technique, we have termed “quantitative nanohistology”. An increase in nanoscale stiffness in the collagen for SSc compared with healthy individuals was seen by a significant increase in the Young’s modulus profile for the collagen. These markers of stiffer collagen in SSc are similar to the symptoms experienced by patients, giving additional hope that in the future, nanohistology using AFM can be readily applied as a clinical tool, providing detailed information of the state of collagen. PMID:28138238

  15. Juvenile Crime, Juvenile Justice. Panel on Juvenile Crime: Prevention, Treatment, and Control.

    Science.gov (United States)

    McCord, Joan, Ed.; Widom, Cathy Spatz, Ed.; Crowell, Nancy A., Ed.

    This book discusses patterns and trends in crimes committed by children and adolescents, analyzing youth crime as a subset of general crime and studying the impact of race and gender. It evaluates different approaches to forecasting future crime rates. Data come from a national panel that examined what is known about juvenile crime and its…

  16. Endothelial injury in a transforming growth factor β-dependent mouse model of scleroderma induces pulmonary arterial hypertension.

    Science.gov (United States)

    Derrett-Smith, Emma C; Dooley, Audrey; Gilbane, Adrian J; Trinder, Sarah L; Khan, Korsa; Baliga, Reshma; Holmes, Alan M; Hobbs, Adrian J; Abraham, David; Denton, Christopher P

    2013-11-01

    To delineate the constitutive pulmonary vascular phenotype of the TβRIIΔk-fib mouse model of scleroderma, and to selectively induce pulmonary endothelial cell injury using vascular endothelial growth factor (VEGF) inhibition to develop a model with features characteristic of pulmonary arterial hypertension (PAH). The TβRIIΔk-fib mouse strain expresses a kinase-deficient transforming growth factor β (TGFβ) receptor type II driven by a fibroblast-specific promoter, leading to ligand-dependent up-regulation of TGFβ signaling, and replicates key fibrotic features of scleroderma. Structural, biochemical, and functional assessments of pulmonary vessels, including in vivo hemodynamic studies, were performed before and following VEGF inhibition, which induced pulmonary endothelial cell apoptosis. These assessments included biochemical analysis of the TGFβ and VEGF signaling axes in tissue sections and explanted smooth muscle cells. In the TβRIIΔk-fib mouse strain, a constitutive pulmonary vasculopathy with medial thickening, a perivascular proliferating chronic inflammatory cell infiltrate, and mildly elevated pulmonary artery pressure resembled the well-described chronic hypoxia model of pulmonary hypertension. Following administration of SU5416, the pulmonary vascular phenotype was more florid, with pulmonary arteriolar luminal obliteration by apoptosis-resistant proliferating endothelial cells. These changes resulted in right ventricular hypertrophy, confirming hemodynamically significant PAH. Altered expression of TGFβ and VEGF ligand and receptor was consistent with a scleroderma phenotype. In this study, we replicated key features of systemic sclerosis-related PAH in a mouse model. Our results suggest that pulmonary endothelial cell injury in a genetically susceptible mouse strain triggers this complication and support the underlying role of functional interplay between TGFβ and VEGF, which provides insight into the pathogenesis of this disease. Copyright

  17. Coupled dynamics of territorial damselfishes and juvenile corals on the reef crest

    Science.gov (United States)

    Casey, J. M.; Choat, J. H.; Connolly, S. R.

    2015-03-01

    Territories of grazing fishes in the family Pomacentridae have been documented to cover a substantial proportion of shallow, exposed coral reef fronts, and these fishes can have profound effects on benthic community composition, including the recruitment and post-settlement survival of scleractinian corals. However, current studies of territorial grazer effects on corals have focused on back-reef habitats. Territorial damselfishes occur in distinct behavioural guilds ranging from indeterminate territorial grazers with thin algal turfs and low rates of territorial aggression to intensive territorial grazers with thick turfs and high rates of aggression. To determine the impact of territorial grazers on the establishment of juvenile corals, we surveyed the reef crest habitat of Lizard Island, Great Barrier Reef, using fixed transects to assess the effects of indeterminate and intensive territorial grazers on juvenile coral abundance and taxonomic composition. In addition, the turnover of territorial pomacentrids was monitored as well as the effects of turnover on juvenile coral assemblages. Intensive territorial grazers were associated with a significantly lower juvenile coral abundance (34 % decrease), but neither intensive nor indeterminate grazer territories impacted juvenile coral taxonomic composition. Over the course of 1 yr, there was a high rate of territorial turnover (39.7 %). Turnover from control plots to intensive damselfish territories was accompanied by a 44 % decrease in juvenile corals; conversely, turnover from intensive damselfish territories to control plots coincided with a 48 % increase in juvenile corals. These findings reveal two main conclusions. Firstly, the association between damselfish territories and the abundance and spatial turnover of juvenile corals strongly implies that territorial grazers have a negative effect on juvenile coral populations. Secondly, the unexpectedly high temporal turnover of damselfish territories indicates that

  18. 8 CFR 236.3 - Detention and release of juveniles.

    Science.gov (United States)

    2010-01-01

    ... 8 Aliens and Nationality 1 2010-01-01 2010-01-01 false Detention and release of juveniles. 236.3... Aliens Prior to Order of Removal § 236.3 Detention and release of juveniles. (a) Juveniles. A juvenile is defined as an alien under the age of 18 years. (b) Release. Juveniles for whom bond has been posted,...

  19. Marcadores de inmunorrespuesta en la periodontitis juvenil

    Directory of Open Access Journals (Sweden)

    Amparo Pérez Borrego,

    2002-12-01

    Full Text Available La periodontitis juvenil es una enfermedad del periodonto propia de adolescentes y adultos jóvenes, afecta principalmente los primeros molares y los incisivos y se caracteriza por la pérdida severa del hueso alveolar alrededor de dientes permanentes sin correspondencia entre la rapidez y severidad de la destrucción con los factores locales. En la causa de la enfermedad se citan factores genéticos, infecciosos e inmunológicos. Estudiamos algunos marcadores de inmunorrespuesta en 6 adolescentes que acudieron a nuestro servicio con el diagnóstico de periodontitis juvenil, además de su valoración clínica y radiológica. Ambos sexos se afectaron por igual, la movilidad dentaria y el sangramiento al sondeo fueron los hallazgos clínicos más relevantes y el índice de higiene bucal fue adecuado en todos los casos. No encontramos homogeneidad en las alteraciones inmunológicas, pero todos los pacientes estuvieron afectados en más de un marcador. Predominaron las alteraciones funcionales de linfocitos T en los estudios celulares. La hipogammaglobulinemia y la IgM elevada fueron las alteraciones más frecuentes en la inmunidad de anticuerpos. Se señala la dificultad que aún existe para explicar la patogenia de la enfermedad basándose solamente en un único factor de riesgo, así como la importancia de la valoración individual de cada enfermo.Juvenile periodontitis is a disease of the periodontium inherent to adolescents and young adults, affecting mainly the first molars and incisives and characterized by the severe loss of the alveolar bone sorrounding the permanent teeth with no correspondance between the celerity and severity of the destruction and the local factors. Genetic, infectious and immunological factors are considereed as the causes of the disease. Some immunoresponse markers were studied in 6 adolescents that were seen at our service with the diagnosis of juvenile periodontitis in addition to their clinical and radiological

  20. Uveitis in juvenile chronic arthritis.

    Science.gov (United States)

    Kanski, J J

    1990-01-01

    About 20% of patients with juvenile chronic arthritis develop uveitis which is frequently bilateral. Risk factors for uveitis are: female gender, pauciarticular onset of arthritis, presence of circulating antinuclear antibodies, and the antigens HLA-DW5 and HLA-DPw2. The visual prognosis in patients with uveitis is good in 25% and fair in 50%. The remaining 25% develop cataract and/or glaucoma. The management of glaucoma is unsatisfactory, but the results of cataract surgery by lensectomy are good.

  1. Juvenile morphology in baleen whale phylogeny.

    Science.gov (United States)

    Tsai, Cheng-Hsiu; Fordyce, R Ewan

    2014-09-01

    Phylogenetic reconstructions are sensitive to the influence of ontogeny on morphology. Here, we use foetal/neonatal specimens of known species of living baleen whales (Cetacea: Mysticeti) to show how juvenile morphology of extant species affects phylogenetic placement of the species. In one clade (sei whale, Balaenopteridae), the juvenile is distant from the usual phylogenetic position of adults, but in the other clade (pygmy right whale, Cetotheriidae), the juvenile is close to the adult. Different heterochronic processes at work in the studied species have different influences on juvenile morphology and on phylogenetic placement. This study helps to understand the relationship between evolutionary processes and phylogenetic patterns in baleen whale evolution and, more in general, between phylogeny and ontogeny; likewise, this study provides a proxy how to interpret the phylogeny when fossils that are immature individuals are included. Juvenile individuals in the peramorphic acceleration clades would produce misleading phylogenies, whereas juvenile individuals in the paedomorphic neoteny clades should still provide reliable phylogenetic signals.

  2. Juvenile morphology in baleen whale phylogeny

    Science.gov (United States)

    Tsai, Cheng-Hsiu; Fordyce, R. Ewan

    2014-09-01

    Phylogenetic reconstructions are sensitive to the influence of ontogeny on morphology. Here, we use foetal/neonatal specimens of known species of living baleen whales (Cetacea: Mysticeti) to show how juvenile morphology of extant species affects phylogenetic placement of the species. In one clade (sei whale, Balaenopteridae), the juvenile is distant from the usual phylogenetic position of adults, but in the other clade (pygmy right whale, Cetotheriidae), the juvenile is close to the adult. Different heterochronic processes at work in the studied species have different influences on juvenile morphology and on phylogenetic placement. This study helps to understand the relationship between evolutionary processes and phylogenetic patterns in baleen whale evolution and, more in general, between phylogeny and ontogeny; likewise, this study provides a proxy how to interpret the phylogeny when fossils that are immature individuals are included. Juvenile individuals in the peramorphic acceleration clades would produce misleading phylogenies, whereas juvenile individuals in the paedomorphic neoteny clades should still provide reliable phylogenetic signals.

  3. Identification of Candidate Genes in Scleroderma-Related Pulmonary Arterial Hypertension

    Science.gov (United States)

    Grigoryev, DN; Mathai, SC; Fisher, MR; Girgis, RE; Zaiman, AL; Housten-Harris, T; Cheadle, C; Gao, L; Hummers, LK; Champion, HC; Garcia, JGN; Wigley, FM; Tuder, RM; Barnes, KC; Hassoun, PM

    2008-01-01

    We hypothesize that pulmonary arterial hypertension (PAH)-associated genes identified by expression profiling of peripheral blood mononuclear cells (PBMCs) from patients with idiopathic pulmonary arterial hypertension (IPAH) can also be identified in PBMCs from scleroderma patients with PAH (PAH-SSc). Gene expression profiles of PBMCs collected from IPAH (n=9), PAH-SSc (n=10) patients and healthy controls (n=5) were generated using HG_U133A_2.0 GeneChips and processed by RMA/GCOS_1.4/SAM_1.21 data analysis pipeline. Disease severity in consecutive patients was assessed by functional status and hemodynamic measurements. The expression profiles were analyzed using PAH severity-stratification, and identified candidate genes were validated with real time PCR (rtPCR). Transcriptomics of PBMCs from IPAH patients was highly comparable with that of PMBCs from PAH-SSc patients. The PBMC gene expression patterns significantly correlate with right atrium pressure (RA) and cardiac index (CI), known predictors of survival in PAH. Array stratification by RA and CI identified 364 PAH-associated candidate genes. Gene ontology analysis revealed significant (Zscore > 1.96) alterations in angiogenesis genes according to PAH severity: MMP9 and VEGF were significantly upregulated in mild as compared to severe PAH and healthy controls, as confirmed by rtPCR. These data demonstrate that PBMCs from patients with PAH-SSc carry distinct transcriptional expression. Furthermore, our findings suggest an association between angiogenesis-related gene expression and severity of PAH in PAH-SSc patients. Deciphering the role of genes involved in vascular remodeling and PAH development may reveal new treatment targets for this devastating disorder. PMID:18355767

  4. High alveolar concentration of nitric oxide is associated with alveolitis in scleroderma.

    Science.gov (United States)

    Tiev, Kiet Phong; Hua-Huy, Thong; Rivière, Sébastien; Le-Dong, Nhat-Nam; Febvre, Michel; Cabane, Jean; Dinh-Xuan, Anh Tuan

    2013-01-15

    Alveolar concentration of nitric oxide (C(A)NO) is a non invasive prognostic marker of systemic sclerosis (SSc) lung disease. There is, however, as yet no direct evidence showing concomitant increase of C(A)NO and the presence of inflammatory cells in alveoli. We have therefore measured C(A)NO and performed broncho-alveolar lavage (BAL) in SSc patients. Exhaled NO was measured, by the means of two different models, the two-compartment model (2CM) and the trumpet model with axial diffusion (TMAD), in 22 SSc patients and compared with 15 healthy controls. BAL was performed in all SSc patients. Alveolitis was defined as lymphocytes >14%, polymorphonuclears >4%, or eosinophils >3% on cell count in BAL fluid. Comparisons of C(A)NO levels were made between SSc patients with, and without, alveolitis. Levels of C(A)NO were significantly higher in SSc patients as compared with controls (palveolitis as compared with SSc patients without alveolitis (8.4ppb; 1st and 3rd interquartile range: 6.0-10.5 vs 3.3ppb; 2.2-3.5; p=0.004 for 2CM and 5.4ppb; 3.2-9.2 vs 3.2ppb; 1.4-3.3, p=0.02 for TMAD), while bronchial airway output of NO (J'awNO, p=0.19), and fractional exhaled NO (F(E)NO, p=0.12) were comparable. C(A)NO was consistently high in SSc patients with alveolitis irrespective of the methods chosen (TMAD or 2CM). Our findings showed that increased C(A)NO was associated with alveolitis in patients with SSc. We submit that C(A)NO could be used as a reliable non-invasive surrogate biomarker of alveolitis in scleroderma lung disease. Copyright © 2012 Elsevier Inc. All rights reserved.

  5. Quantitative nanohistological investigation of scleroderma: an atomic force microscopy-based approach to disease characterization

    Directory of Open Access Journals (Sweden)

    Strange AP

    2017-01-01

    Full Text Available Adam P Strange,1 Sebastian Aguayo,1 Tarek Ahmed,1 Nicola Mordan,1 Richard Stratton,2 Stephen R Porter,3 Susan Parekh,4 Laurent Bozec1 1Department of Biomaterials and Tissue Engineering, UCL Eastman Dental Institute, 2Centre for Rheumatology and Connective Tissue Diseases, Royal Free Hospital, UCL Medical School, 3UCL Eastman Dental Institute, 4Department of Pediatrics, UCL Eastman Dental Institute, London, UK Abstract: Scleroderma (or systemic sclerosis, SSc is a disease caused by excess crosslinking of collagen. The skin stiffens and becomes painful, while internally, organ function can be compromised by the less elastic collagen. Diagnosis of SSc is often only possible in advanced cases by which treatment time is limited. A more detailed analysis of SSc may provide better future treatment options and information of disease progression. Recently, the histological stain picrosirius red showing collagen register has been combined with atomic force microscopy (AFM to study SSc. Skin from healthy individuals and SSc patients was biopsied, stained and studied using AFM. By investigating the crosslinking of collagen at a smaller hierarchical stage, the effects of SSc were more pronounced. Changes in morphology and Young’s elastic modulus were observed and quantified; giving rise to a novel technique, we have termed “quantitative nanohistology”. An increase in nanoscale stiffness in the collagen for SSc compared with healthy individuals was seen by a significant increase in the Young’s modulus profile for the collagen. These markers of stiffer collagen in SSc are similar to the symptoms experienced by patients, giving additional hope that in the future, nanohistology using AFM can be readily applied as a clinical tool, providing detailed information of the state of collagen. Keywords: rheumatology, adjunct diagnosis, picrosirius red, collagen, nanohistology

  6. Valuation of scleroderma and psoriatic arthritis health states by the general public

    Directory of Open Access Journals (Sweden)

    Hays Ron D

    2010-10-01

    Full Text Available Abstract Objective Psoriatic arthritis (PsA and scleroderma (SSc are chronic rheumatic disorders with detrimental effects on health-related quality of life. Our objective was to assess health values (utilities from the general public for health states common to people with PsA and SSc for economic evaluations. Methods Adult subjects from the general population in a Midwestern city (N = 218 completed the SF-12 Health Survey and computer-assisted 0-100 rating scale (RS, time trade-off (TTO, range: 0.0-1.0 and standard gamble (SG, range: 0.0-1.0 utility assessments for several hypothetical PsA and SSc health states. Results Subjects included 135 (62% females, 143 (66% Caucasians, and 62 (28% African-Americans. The mean (SD scores for the SF-12 Physical Component Summary scale were 52.9 (8.3 and for the SF-12 Mental Component Summary scale were 49.0 (9.1, close to population norms. The mean RS, TTO, and SG scores for PsA health states varied with severity, ranging from 20.2 to 63.7 (14.4-20.3 for the RS 0.29 to 0.78 (0.24-0.31 for the TTO, and 0.48 to 0.82 (0.24-0.34 for the SG. The mean RS, TTO, and SG scores for SSc health states were 25.3-69.7 (15.2-16.3 for the RS, 0.36-0.80 (0.25-0.31 for the TTO, and 0.50-0.81 (0.26-0.32 for the SG, depending on disease severity. Conclusion Health utilities for PsA and SSc health states as assessed from the general public reflect the severity of the diseases. These descriptive findings could have implications regarding comparative effectiveness research for tests and treatments for PsA and SSc.

  7. Algorithm for the Diagnosis of Scleroderma. Early Systemic Sclerosis: Definitions and diagnostic criteria

    Directory of Open Access Journals (Sweden)

    Domenico Galasso

    2013-04-01

    Full Text Available Introduction: The term scleroderma derives from the Greek words skleros, which means hard, and derma, which means skin. It refers to an acquired systemic inflammatory disease of the connective tissue –also known as systemic sclerosis (SSc– characterized by excessive collagen deposition in the skin and the internal organs that results in fibrosis. The typical vascular lesion in SSc leads to narrowing of the vessel lumen, intimal thickening, medial hypotrophy, and adventitial fibrosis of small muscular vessels, collagen deposition in the other matrix components of interstice, and the Raynaud phenomenon secondary to these widespread microvascular abnormalities. All these characteristics lead to a connective tissue re-modeling. Discussion: Several clinical studies utilize the American Rheumatology Association’s 1980 classification. However, these diagnostic criteria are unsatisfactory because they fail to take into consideration part of the disease spectrum. Early-phase SSc is characterized by the Raynaud phenomenon (in 90% of all patients, sclerodactyly, and positivity for SSc-specific autoantibodies (antinuclear antibodies, anti-topoisomerase I antibodies, anti-RNA polymerase I and III antibodies, anti-centromere antibodies, anti-fibrillarin antibodies, anti-PM-SCL antibodies.. It is necessary to reduce delays in the diagnosis of SSc. Conclusions: Patients with red-flag positivity (Raynaud phenomenon and digital edema require Phase I SSc screening, which consists in capillaroscopic assessment of possible microvascular abnormalities. The work-up will then focus on inflammatory indices, renal function tests, and internal organ involvement (echocardiography, high-resolution computed tomography of the chest, diffusing capacity of the lungs for carbon monoxide.

  8. Transcriptomic immune response of Tenebrio molitor pupae to parasitization by Scleroderma guani.

    Directory of Open Access Journals (Sweden)

    Jia-Ying Zhu

    Full Text Available BACKGROUND: Host and parasitoid interaction is one of the most fascinating relationships of insects, which is currently receiving an increasing interest. Understanding the mechanisms evolved by the parasitoids to evade or suppress the host immune system is important for dissecting this interaction, while it was still poorly known. In order to gain insight into the immune response of Tenebrio molitor to parasitization by Scleroderma guani, the transcriptome of T. molitor pupae was sequenced with focus on immune-related gene, and the non-parasitized and parasitized T. molitor pupae were analyzed by digital gene expression (DGE analysis with special emphasis on parasitoid-induced immune-related genes using Illumina sequencing. METHODOLOGY/PRINCIPAL FINDINGS: In a single run, 264,698 raw reads were obtained. De novo assembly generated 71,514 unigenes with mean length of 424 bp. Of those unigenes, 37,373 (52.26% showed similarity to the known proteins in the NCBI nr database. Via analysis of the transcriptome data in depth, 430 unigenes related to immunity were identified. DGE analysis revealed that parasitization by S. guani had considerable impacts on the transcriptome profile of T. molitor pupae, as indicated by the significant up- or down-regulation of 3,431 parasitism-responsive transcripts. The expression of a total of 74 unigenes involved in immune response of T. molitor was significantly altered after parasitization. CONCLUSIONS/SIGNIFICANCE: obtained T. molitor transcriptome, in addition to establishing a fundamental resource for further research on functional genomics, has allowed the discovery of a large group of immune genes that might provide a meaningful framework to better understand the immune response in this species and other beetles. The DGE profiling data provides comprehensive T. molitor immune gene expression information at the transcriptional level following parasitization, and sheds valuable light on the molecular

  9. Juvenile psammomatoid ossifying fibroma. Case report

    OpenAIRE

    Vahtsevanos, Konstantinos; Persephone XIROU; Giorgos BALLIS; Tsekos, Antonis; Ntomouchtsis, Aris; Alexandros VALASIDIS; Doxa MAGGOUDI

    2012-01-01

    Ossifying fibroma (OS) represents a slow growing, benign neoplasm that belongs to the greater group of fibro-osseous lesions. Based on its histological features, ossifying fibroma is divided into: a) juvenile trabecular OS and b) juvenile psammomatoid OS which affects mainly the paranasal sinuses of children and teenagers aging from 5 to 15 years.A rare case of juvenile psammomatoid ossifying fibroma in a 30 year old male patient located in the left mandibular ramus is presented. Treatment pl...

  10. Study of pulmonary function injury caused by system scleroderma%系统性硬化症致肺功能损害的探讨

    Institute of Scientific and Technical Information of China (English)

    张生山; 竺红

    2000-01-01

    Objectives Study changing of pulmonary function injury caused by scleroderma system. Methods Test 30 patients pulmonary function with system scleroderma using U. S. A Sensormedics 6200 Automate Body Plethysmograph Results There are 28 patients with system scleroderma that have the changes of pulmonary function. It is the change of small airway function, the increase of airway resistance, and there are negative correlation. Conclusions It is mainly the change of small airway function and the increase of airway resistance for change of pulmonary function caused by system scleroderma.%目的 研究系统性硬化症导致肺功能损害的变化。方法 采用美国Sensormedics 6200型人体体积描记仪检测30例系统性硬化症患者的肺功能。结果  28例系统性硬化症患者有肺功能改变,主要表现为小气道功能改变,气道阻力增高,且两者存在负相关性(P<0.05)。结论 系统性硬化症引起肺功能改变以小气道功能改变及气道阻力增高为主。

  11. The short-term effect of gloving in combination with Traditional Thai Massage, heat, and stretching exercise to improve hand mobility in scleroderma patients

    Directory of Open Access Journals (Sweden)

    Kunavut Vannajak

    2014-01-01

    Full Text Available Background: Systemic sclerosis (SSc is a chronic, multisystem connective tissue disorder characterized by autoimmune activation, microvascular endothelium damage, and excessive collagen proliferation. The most affected hand presents claw hand deformity and microvascular disease. Deformed hands can cause functional disability and decrease the quality of life. A daily home program can improve mobility of scleroderma patients. Objective: We sought to determine the effect of a daily home exercise program on hand mobility among scleroderma patients. Materials and Methods: This was a randomized control trial. Twenty-eight participants were divided into two groups, both of which received the same daily home treatment: Group 1 with gloves (n = 14 and Group 2 without gloves (n = 14. The 2-week daily home program combined traditional Thai massage (TTM with stretching exercises and heat. Hand mobility was assessed using hand mobility in scleroderma (HAMIS. The study was conducted in patients who were already on vasodilator drugs. Results: Both groups showed a significant improvement in hand mobility after 2 weeks of daily home exercise program (P < 0.05. Wearing the glove, however, resulted in better thumb mobility. Conclusions: A daily home exercise program improved hand mobility among patients with scleroderma and wearing gloves may improve thumb mobility.

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

    NARCIS (Netherlands)

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

    2006-01-01

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

  13. Justicia juvenil restaurativa como respuesta alternativa

    OpenAIRE

    Mariño Rojas, Cielo

    2016-01-01

    El artículo explora las posibilidades de la justicia juvenil restaurativa como respuesta alternativa en los sistemas de justicia juvenil en la región. Si bien la justicia restaurativa no aparece explícitamente en los instrumentos internacionales sobre justicia penal juvenil, estos dan la oportunidad para que aquella se desarrolle dentro de los sistemas de justicia juvenil. Inicialmente se aborda su evolución histórica para establecer el origen de sus principales características. A continuació...

  14. Long-term clinical stabilization of scleroderma patients treated with a chronic and intensive IV iloprost regimen.

    Science.gov (United States)

    Foti, Rosario; Visalli, Elisa; Amato, Giorgio; Benenati, Alessia; Converso, Giovanni; Farina, Alberto; Bellofiore, Salvatore; Mulè, Massimiliano; Di Gangi, Marcella

    2017-02-01

    Intravenous iloprost is a first-line option for the treatment of scleroderma-related digital vasculopathy, and some studies have suggested its favourable role on disease progression. The aim of our study is to evaluate the disease progression, specifically in terms of cardiopulmonary function, in a group of consecutive patients chronically treated with intravenous iloprost. Our retrospective study enrolled 68 scleroderma patients (68 F, 54.4 ± 12.3 years) treated with iloprost for 7.1 ± 2.9 years, with a schedule of 5-6 consecutive daily infusions per month (6 h/day, 0.5-2.0 ng/kg/min). In all patients, modified Rodnan skin score (4.7 ± 5.3 vs. 3.7 ± 5.3, p iloprost administration seems to stabilize and potentially improve the long-term development of disease in SSc patients, as suggested by stabilization or significant improvement of cardiopulmonary parameters and vasculopathy.

  15. [Long-term effects of sildenafil in a patient with scleroderma-associated pulmonary hypertension and Raynaud's syndrome].

    Science.gov (United States)

    Rosenkranz, S; Caglayan, E; Diet, F; Karasch, T; Weihrauch, J; Wassermann, K; Erdmann, E

    2004-08-13

    A 65-year-old woman was admitted because of dyspnea at rest and peripheral edema due to scleroderma-associated pulmonary fibrosis and hypertension, as well as Raynaud's phenomenon. She had a marked restrictive ventilatory disorder with severe impairment of diffusion capacity. Right heart catheterization demonstrated a mean pulmonary artery pressure of 50 mmHg. She was able to walk only 220 m. All usual methods of treatment failed to give satisfactory results so that sildenafil (phospherodiesterase type-5 |PDE-5| inhibitor; Viagra ((R)) was given, even though it is not licensed for this indications ("off-label", as a therapeutic attempt. This achieved definite reduction in pulmonary arterial pressure and significantly improved the clinical symptoms. In particular, it drastically reduced the level of atrial natriuretic peptide, an important prognostic marker in right heart failure. Sildenafil also significantly raised peripheral perfusion and the signs of Raynaud's syndrome. PDE-5 inhibitors are efficacious in scleroderma-associated pulmonary hypertension and may also provide a new option in the treatment of Raynaud's disease.

  16. Juvenile hyaline fibromatosis. Radiological diagnosis. Fibromatosis hialina juvenil. Diagnostico radiologico

    Energy Technology Data Exchange (ETDEWEB)

    Fuentes, R.; Sar, V.; Cabrera, J.J.; Diaz, L.; Hernandez, B.; Valeron, P.; Baez, O.; Rodriguez, M.

    1993-10-01

    Juvenile hyaline fibromatosis (JHF) is a rare disorder of unknown etiology, very few cases of which have been reported in the literature. It presents similarities to other fibromatosys, but has its particular radiological features which differentiate it from them. The clinical findings consist of several, slow growing, subcutaneous nodules, flexion contractures of the joints which can lead to disability, gingival hypertrophy and muscular atrophy. The suspected radiological diagnosis is confirmed by electron microscopy study of the nodules, although light microscopy can also reveal suggestive images. Author (9 refs.)

  17. Juvenile ossifying fibroma: Psammamatoid variant

    Directory of Open Access Journals (Sweden)

    Shivani Aggarwal

    2012-01-01

    Full Text Available Juvenile ossifying fibroma is a rare fibro-osseous lesion containing variable amount of calcified masses, which resembles bone or cementum within a fibrocellular connective tissue stroma. It has variable clinical behavior, highly aggressive in nature including invasion and destruction of adjacent anatomic structures with a strong tendency to recur. We reported a 28-year-old female patient with a growth in the upper left vestibule region extending from canine to molar region with clinical, histopathological, and radiological features are presented. Surgical management was done, and regular follow-up was advised.

  18. Juvenile Competency to Stand Trial.

    Science.gov (United States)

    Stepanyan, Sofia T; Sidhu, Shawn S; Bath, Eraka

    2016-01-01

    Competency to stand trial is interpreted as a protected due process right for all defendants and is defined as a defendant's fundamental knowledge and understanding of the criminal charges being filed, roles and procedures within the courtroom, and a general ability to work with the defense counsel. Questions of competency are most often raised by the judge, defense, or the prosecution, and competency evaluations are most often completed by psychiatrists or psychologists with forensic training or work experience. Mental illness, intellectual disability, developmental disorders, and developmental immaturity are the 4 main factors considered in most juvenile competency evaluations.

  19. Glucocorticoids in juvenile idiopathic arthritis.

    Science.gov (United States)

    Malattia, Clara; Martini, Alberto

    2014-05-01

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

  20. Imaging of juvenile idiopathic arthritis

    Energy Technology Data Exchange (ETDEWEB)

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

    2006-08-15

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

  1. THE STUDY OF FEATURES OF GUILT OF JUVENILE OFFENDERS IN THE CONTEXT OF JUVENILE JUSTICE

    Directory of Open Access Journals (Sweden)

    Natalija Vladimirovna Galkina

    2015-08-01

    Full Text Available The article is devoted to the results of empirical studies of the experiences of guilt of juvenile offenders in the context of juvenile justice where a minor appears as the subject of legal relations. Restorative approach of juvenile justice is based on an admission of guilt to the victim. In connection with it, the research of features of the guilt of minors who have committed an offence and the conditions for the development of the subjectivity will enhance understanding of the possibilities of restorative juvenile justice system in the prevention of juvenile delinquency.Thus, the results of empirical research presented in the article are important for determining of the psychological bases of realization of rehabilitation programs in the context of juvenile justice. In particular, the results are important for the organization and conduct of psychological work to overcome the psychological barriers in the behavior of juveniles having inherently maladaptive guilt and destructive psychological defense mechanisms.

  2. Changes in Juvenile Justice in China.

    Science.gov (United States)

    Wong, Dennis S. W.

    2001-01-01

    Discusses rising juvenile and youth crime in China, highlighting the essence of Chinese Marxist criminological thought and changing conceptions of delinquency from the postrevolutionary period to the present; examining official responses to delinquency and the recent development of juvenile justice; and suggesting that current delinquency control…

  3. Juvenile Anorexia Nervosa: Family Therapy's Natural Niche

    Science.gov (United States)

    Fishman, H. Charles

    2006-01-01

    Juvenile Anorexia Nervosa (AN) is a severe problem both in terms of presenting symptomatology and its tendency toward chronicity. Researchers have consistently shown that family-based approaches are superior to individual approaches for the treatment of juvenile AN. This article addresses the capacity deficit of trained family therapists to treat…

  4. Sexually dimorphic body plumage in juvenile crossbills

    NARCIS (Netherlands)

    Edelaar, P; Phillips, RE; Knops, P

    2005-01-01

    Sexual dimorphism in color and pattern of contour feathers is rare in juvenile songbirds. We describe how captive-bred juvenile males of Scottish Crossbill (Loxia scotica) and nominate Red Crossbill (L. curvirostra curvirostra) can be differentiated from females prior to prebasic molt by an unstreak

  5. Juveniles' Motivations for Remaining in Prostitution

    Science.gov (United States)

    Hwang, Shu-Ling; Bedford, Olwen

    2004-01-01

    Qualitative data from in-depth interviews were collected in 1990-1991, 1992, and 2000 with 49 prostituted juveniles remanded to two rehabilitation centers in Taiwan. These data are analyzed to explore Taiwanese prostituted juveniles' feelings about themselves and their work, their motivations for remaining in prostitution, and their difficulties…

  6. Alternative sanctions for juveniles in the Netherlands

    NARCIS (Netherlands)

    Laan, P.H. van der

    1993-01-01

    In the Netherlands alternative sanctions for juveniles have become very popular. In less than ten years, the alternative sanction has surpassed the fine as the most frequently imposed penal sanction for juveniles. As a result of this popularity, some net widening has occured. In general, alternativl

  7. Psychiatric Disorder in a Juvenile Assessment Center

    Science.gov (United States)

    McReynolds, Larkin S.; Wasserman, Gail A.; DeComo, Robert E.; John, Reni; Keating, Joseph M.; Nolen, Scott

    2008-01-01

    Juvenile assessment centers (JACs) were developed to address service fragmentation and promote the sharing of information among agencies providing services to youth involved with the juvenile justice system. To date, there are no reports that describe the diagnostic profiles of the youth served by such centers. The authors hypothesize that the…

  8. Moral Development of Solo Juvenile Sex Offenders

    Science.gov (United States)

    Van Vugt, Eveline; Stams, Geert Jan; Dekovic, Maja; Brugman, Daan; Rutten, Esther; Hendriks, Jan

    2008-01-01

    This study compared the moral development of solo juvenile male sex offenders (n = 20) and juvenile male non-offenders (n = 76), aged 13-19 years, from lower socioeconomic and educational backgrounds. The Moral Orientation Measure (MOM) was used to assess punishment- and victim-based moral orientation in sexual and non-sexual situations. Moral…

  9. Juveniles' Motivations for Remaining in Prostitution

    Science.gov (United States)

    Hwang, Shu-Ling; Bedford, Olwen

    2004-01-01

    Qualitative data from in-depth interviews were collected in 1990-1991, 1992, and 2000 with 49 prostituted juveniles remanded to two rehabilitation centers in Taiwan. These data are analyzed to explore Taiwanese prostituted juveniles' feelings about themselves and their work, their motivations for remaining in prostitution, and their difficulties…

  10. Ethnic disparities in Dutch juvenile justice

    NARCIS (Netherlands)

    Komen, M.; van Schooten, E.

    2009-01-01

    In the Netherlands, ethnic minority boys are heavily overrepresented in prisons and secure judicial institutions for juveniles. In a sample of 324 juveniles of both native Dutch and ethnic minority origin who have come into contact with the Dutch criminal justice authorities, we compared the number

  11. Group sexual offending by juvenile females

    NARCIS (Netherlands)

    Wijkman, M.; Weerman, F.; Bijleveld, C.; Hendriks, J.

    2015-01-01

    This study examined all group sexual offending cases in the Netherlands between 1995 and 2009 (n = 26) in which at least one juvenile female offender (n = 35) had been adjudicated. Information from court files showed that the majority of juvenile female group sexual offenders have (inter)personal pr

  12. A social work study on juvenile delinquency

    Directory of Open Access Journals (Sweden)

    Mohammad Reza Iravani

    2012-08-01

    Full Text Available We present an empirical study to study the effects of different factors on juvenile delinquency. The investigation distributes 100 questionnaires among people who are involved with crime and analyzes their feedbacks. There are five hypotheses in our survey and we look to see whether family conditions, religion, economical conditions, media and physical and psychological characteristics play important role on juvenile delinquency in Iranian society. The results shows that while family conditions, physical and psychological characteristics play important role on juvenile delinquency, other factors do not statistically have any impact on juvenile delinquency. The study suggests that a better family condition could help reduce juvenile delinquency and people could guide their children through better consultations.

  13. Family transitions and juvenile delinquency.

    Science.gov (United States)

    Schroeder, Ryan D; Osgood, Aurea K; Oghia, Michael J

    2010-01-01

    There is a large body of research that shows children from non-intact homes show higher rates of juvenile delinquency than children from intact homes, partially due to weaker parental control and supervision in non-intact homes. What has not been adequately addressed in the research is the influence of changes in family structure among individual adolescents over time on delinquent offending. Using the first and third waves of the National Youth Study, we assess the effect of family structure changes on changes in delinquent offending between waves through the intermediate process of changes in family time and parental attachment. Although prior research has documented adolescents in broken homes are more delinquent than youth in intact homes, the process of family dissolution is not associated with concurrent increases in offending. In contrast, family formation through marriage or cohabitation is associated with simultaneous increases in offending. Changes in family time and parental attachment account for a portion of the family formation effect on delinquency, and prior parental attachment and juvenile offending significantly condition the effect of family formation on offending.

  14. 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.

  15. Frequência elevada de calcinose em dermatomiosite juvenil: estudo de fatores de risco High frequency of calcinosis in juvenile dermatomyositis: a risk factor study

    Directory of Open Access Journals (Sweden)

    Gleice Clemente

    2012-08-01

    divided into two groups as follows: those who developed calcinosis (up to the sixth month of follow-up and after six months of follow-up and those who did not develop calcinosis. Twentyseven patients underwent two nailfold capillaroscopies (NFC, which were considered altered when the scleroderma pattern was found. RESULTS: The mean age of symptom onset of the 34 patients was 6.5 years, the time until diagnosis was 1.2 years, and 70% were females. Half of the patients had a monocyclic disease course, and only 14.7% had severe vasculitis. Almost 90% of the patients undergoing NFC showed a change on the first assessment, 74% showed a change on the second assessment, and the mean interval between both assessments was 1.6 year. Calcinosis was evidenced in 16 (47.1% patients. No association was observed between the variables analyzed and the development of calcinosis. CONCLUSION: No risk factors for calcinosis were identified in this study, although that complication was found in half of the patients with juvenile dermatomyositis studied.

  16. Juvenile recurrent parotitis in children: diagnosis and treatment using sialography.

    Science.gov (United States)

    Narsimha Rao, Vanga V; Putta Buddi, Jai Shankar Homberhali; Kurthukoti, Ameet J

    2014-01-01

    Juvenile recurrent parotitis (JRP) is a nonobstructive, nonsuppurative parotid inflammation in young children. Causative factors are many such as allergy, infection, local autoimmune manifestations, and genetic inheritance have been suggested, but none have been proved. Parotid sialography is a hallmark in the diagnosis of JRP but newer modalities such as ultrasonography, computed tomography and magnetic resonance imaging-sialography are noninvasive investigative techniques. Recurrent attacks are often managed conservatively. Here we report a case of a 5-year-old child with JRP. Sailography can be used as both diagnostic and therapeutic modality.

  17. Increased frequency of HLA-DPw2 in pauciarticular onset juvenile chronic arthritis

    DEFF Research Database (Denmark)

    Ødum, Niels; Morling, Niels; Friis, J;

    1986-01-01

    Thirty-six unrelated Danish patients with pauciarticular Juvenile Chronic Arthritis (PJCA) and 120 controls were typed for HLA-DPw1-w6 and the local specificity CDPHEI with bulk-expanded Primed Lymphocyte Typing (PLT) cells. The frequency of HLA-DPw2 was 52.8% in PJCA patients and 16.7% in contro...

  18. Periodontal and hematological characteristics associated with aggressive periodontitis, juvenile idiopathic arthritis, and rheumatoid arthritis

    DEFF Research Database (Denmark)

    Poulsen, Anne Havemose; Westergaard, Jytte; Stoltze, Kaj

    2006-01-01

    Periodontitis shares several clinical and pathogenic characteristics with chronic arthritis, and there is some degree of coexistence. The aims of this study were to elucidate whether patients with localized aggressive periodontitis (LAgP), generalized aggressive periodontitis (GAgP), juvenile...

  19. 76 FR 2135 - Office of Juvenile Justice and Delinquency Prevention Proposed Plan for Fiscal Year 2011

    Science.gov (United States)

    2011-01-12

    ... mentoring programs that provide general guidance and support; promote personal and social responsibility... period. OJJDP posts on its Web site ( http://www.ojjdp.gov ) solicitations of grant or cooperative...) communities,\\1\\ and other localities retain primary responsibility for administering juvenile justice and...

  20. Profile of Incarcerated Juveniles: Comparison of Male and Female Offenders

    Science.gov (United States)

    Martin, Don; Martin, Magy; Dell, Rex; Davis, Candice; Guerrieri, Karen

    2008-01-01

    Effective methods of identifying potential juvenile offenders are critical when developing prevention programs within both state and national juvenile justice systems. The characteristics of juvenile offenders in a large juvenile justice system are examined in this study. Participants live in a Midwestern city with a high rate of crime as…

  1. 8 CFR 1236.3 - Detention and release of juveniles.

    Science.gov (United States)

    2010-01-01

    ... 8 Aliens and Nationality 1 2010-01-01 2010-01-01 false Detention and release of juveniles. 1236.3... ORDERED REMOVED Detention of Aliens Prior to Order of Removal § 1236.3 Detention and release of juveniles. (a) Juveniles. A juvenile is defined as an alien under the age of 18 years. (b) Release....

  2. Habitat associations of juvenile versus adult butterflyfishes

    Science.gov (United States)

    Pratchett, M. S.; Berumen, M. L.; Marnane, M. J.; Eagle, J. V.; Pratchett, D. J.

    2008-09-01

    Many coral reef fishes exhibit distinct ontogenetic shifts in habitat use while some species settle directly in adult habitats, but there is not any general explanation to account for these differences in settlement strategies among coral reef fishes. This study compared distribution patterns and habitat associations of juvenile (young of the year) butterflyfishes to those of adult conspecifics. Three species, Chaetodon auriga, Chaetodon melannotus, and Chaetodon vagabundus, all of which have limited reliance on coral for food, exhibited marked differences in habitat association of juvenile versus adult individuals. Juveniles of these species were consistently found in shallow-water habitats, whereas adult conspecifics were widely distributed throughout a range of habitats. Juveniles of seven other species ( Chaetodon aureofasciatus, Chaetodon baronessa, Chaetodon citrinellus, Chaetodon lunulatus, Chaetodon plebeius, Chaetodon rainfordi, and Chaetodon trifascialis), all of which feed predominantly on live corals, settled directly into habitat occupied by adult conspecifics. Butterflyfishes with strong reliance on corals appear to be constrained to settle in habitats that provide access to essential prey resources, precluding their use of distinct juvenile habitats. More generalist butterflyfishes, however, appear to utilize distinct juvenile habitats and exhibit marked differences in the distribution of juveniles versus adults.

  3. "Paul Klee and the hypothesis of morphic resonance". Adjustment to illness and life history of patients with progressive systemic scleroderma.

    Science.gov (United States)

    Morscher, C

    1994-06-01

    Within the context of a Psychoanalytic Psychosomatic Medicine the biography of Paul Klee is analysed, who suffered during his last years from progressive systemic scleroderma. Amongst the pervading and characteristic motives of his life and his work, the motives of the dragon-like demon and that of the Dead are introduced. Both are shown to relate to his illness as well as to a highly significant narcissistic idealized picture of his grandmother on the mother's side. Finally, a general biological hypothesis--Sheldrake's hypothesis of morphogenetic fields and morphic resonance--is laid out for the domain of Psychosomatic Medicine. According to this hypothesis, primary process characteristics such as condensation and displacement are also seen to be valid in the somatic domain without being restricted only to conversion events.

  4. Juvenile psammomatoid ossifying fibroma. Case report

    Directory of Open Access Journals (Sweden)

    Konstantinos VAHTSEVANOS

    2012-08-01

    Full Text Available Ossifying fibroma (OS represents a slow growing, benign neoplasm that belongs to the greater group of fibro-osseous lesions. Based on its histological features, ossifying fibroma is divided into: a juvenile trabecular OS and b juvenile psammomatoid OS which affects mainly the paranasal sinuses of children and teenagers aging from 5 to 15 years.A rare case of juvenile psammomatoid ossifying fibroma in a 30 year old male patient located in the left mandibular ramus is presented. Treatment plan included radical surgical excision of the lesion and restoration with autologous osteochondral graft from the 6th rib of the ipsilateral side.

  5. Juvenile eye growth, when completed?

    DEFF Research Database (Denmark)

    Fledelius, Hans C; Christensen, Anders S; Fledelius, Christian

    2014-01-01

    PURPOSE: To test Sorsby's classical statement of axial eye growth as completed at the age of 13 years, with a view also to differentiating between basic eye growth and juvenile elongation associated with eventual refractive change towards myopia. METHODS: (i) A total of 160 healthy eyes close...... was preferred for conventional ultrasound oculometry due to its extreme repeatability of measuring values, thus making it well fitted for evaluating very small differences. In particular, this had bearing for the decelerating end phase of growth in the longitudinal investigation. RESULTS: Sorby's statement...... about age 13 as general limit found support from the cross-sectional data, which suggested stable emmetropic eye size from about 11-12 years, with an average apparently outgrown male emmetropic value of 23.5 mm versus females' 22.9 mm. The longitudinal data, however, showed emmetropic growth also beyond...

  6. Juvenile Huntington disease in Argentina.

    Science.gov (United States)

    Gatto, Emilia Mabel; Parisi, Virginia; Etcheverry, José Luis; Sanguinetti, Ana; Cordi, Lorena; Binelli, Adrian; Persi, Gabriel; Squitieri, Ferdinando

    2016-01-01

    We analyzed demographic, clinical and genetic characteristics of juvenile Huntington disease (JHD) and it frequency in an Argentinean cohort. Age at onset was defined as the age at which behavioral, cognitive, psychiatric or motor abnormalities suggestive of JHD were first reported. Clinical and genetic data were similar to other international series, however, in this context we identified the highest JHD frequency reported so far (19.72%; 14/71). Age at onset of JHD is challenging and still under discussion. Our findings reinforce the hypothesis that clinical manifestations, other than the typical movement disorder, may anticipate age at onset of even many years. Analyses of JHD cohorts are required to explore it frequency in populations with different backgrounds to avoid an underestimation of this rare phenotype. Moreover, data from selected populations may open new pathways in therapeutic approaches and may explain new potential correlations between HD presentations and environmental or biological factors.

  7. [Physiotherapy for juvenile idiopathic arthritis].

    Science.gov (United States)

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

    2012-07-01

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

  8. A Rare Case of Juvenile Systemic Lupus Erythematosus with Disseminated Histoplasmosis

    Science.gov (United States)

    Sarkar, Piyabi; Basu, Keya; Mallick (Sinha), Mamata Guha

    2016-01-01

    Histoplasmosis is a systemic fungal infection caused by dimorphic fungus, Histoplasma capsulatum. Immunocompetent individuals usually have self-limiting or localized disease whereas immunocompromised individuals develop disseminated disease. The occurrence of progressive disseminated histoplasmosis in juvenile systemic lupus erythematosus is extremely rare with only one reported case in literature showing such association. Therefore, we report a case of severe opportunistic fungal infection caused by Histoplasma in a 13-year-old girl who was diagnosed with juvenile lupus erythematosus, subsequently developed septic shock and died of the disease despite of aggressive antifungal therapy. PMID:27904204

  9. Juvenile prison: Remarks on the specific characteristics of regular sentencing

    OpenAIRE

    Miladinović-Stefanović, Dušica

    2015-01-01

    The system of the juvenile criminal law in the Republic of Serbia includes different mechanisms of social response to juvenile delinquency, including corrective orders, corrective measures and juvenile prison. This paper deals with the issue of determining a relevant sentence for juvenile offenders in trial proceedings. The legislator has provided a number of guidelines for these proceedings: the specific range of the juvenile prison sentence, the purpose of punishment, the degree of maturity...

  10. Parenting Styles and Family Communication as Correlates of Juvenile Delinquency

    OpenAIRE

    1997-01-01

    The goal of this study was to examine parenting styles and family communication as correlates of juvenile delinquency. A review of the literature was completed in the areas of parenting styles, family communication, and juvenile delinquency. The literature that was reviewed for this study was examined mainly from juvenile perceptions. This study was approached from a general systems theory perspective. A sample of juveniles (N = 78) from Weber County, Utah, involved in the juvenile justice...

  11. Combined pre-injection wrist and ankle MRI protocol and steroid joint injections in juvenile idiopathic arthritis

    Energy Technology Data Exchange (ETDEWEB)

    Kan, J.H. [Texas Children' s Hospital, Department of Radiology, Houston, TX (United States); Graham, T.B. [Monroe Carell Jr. Children' s Hospital at Vanderbilt, Department of Pediatric Rheumatology, Nashville, TN (United States)

    2011-10-15

    Precise localization of affected compartments of the wrist and ankle in children with an established diagnosis of juvenile idiopathic arthritis (JIA) is clinically challenging. The purpose of this paper is to describe our experience utilizing a pre-injection MRI protocol of the wrist and ankle for localizing disease activity followed by fluoroscopically guided joint injections in children with JIA. (orig.)

  12. Are antipredator behaviours of hatchery Salmo salar juveniles similar to wild juveniles?

    Science.gov (United States)

    Salvanes, A G V

    2017-01-27

    This study explores how antipredator behaviour of juvenile Atlantic salmon Salmo salar developed during conventional hatchery rearing of eggs from wild brood stock, compared with the behaviour of wild-caught juveniles from the same population. Juveniles aged 1+ years were tested in two unfamiliar environments; in one S. salar were presented with simulated predator attacks and in the other they were given the opportunity to explore an open-field arena. No difference was found in their spontaneous escape responses or ventilation rate (reflex responses) after simulated predator attacks. Hatchery-reared juveniles were more risk-prone in their behaviours than wild-caught individuals. Hatchery juveniles stayed less time in association with shelter. In the open-field arena, hatchery juveniles were more active than wild juveniles. Hatchery juveniles were also immobile for less time and spent a shorter amount of time than wild juveniles in the fringe of the open-field arena. Salmo salar size had no effect on the observed behaviour. Overall, this study provides empirical evidence that one generation of hatchery rearing does not change reflex responses associated with threats, whereas antipredator behaviour, typically associated with prior experience, was less developed in hatchery-reared than in wild individuals.

  13. Conceptualizing juvenile prostitution as child maltreatment: findings from the National Juvenile Prostitution Study.

    Science.gov (United States)

    Mitchell, Kimberly J; Finkelhor, David; Wolak, Janis

    2010-02-01

    Two studies were conducted to identify the incidence (Study 1) and characteristics (Study 2) of juvenile prostitution cases known to law enforcement agencies in the United States. Study 1 revealed a national estimate of 1,450 arrests or detentions (95% confidence interval [CI]: 1,287-1,614) in cases involving juvenile prostitution during a 1-year period. In Study 2, exploratory data were collected from a subsample of 138 cases from police records in 2005. The cases are broadly categorized into three main types: (a) third-party exploiters, (b) solo prostitution, and (c) conventional child sexual abuse (CSA) with payment. Cases were classified into three initial categories based on police orientation toward the juvenile: (a) juveniles as victims (53%), (b) juveniles as delinquents (31%), and (c) juvenile as both victims and delinquents (16%). When examining the status of the juveniles by case type, the authors found that all the juveniles in CSA with payment cases were treated as victims, 66% in third-party exploiters cases, and 11% in solo cases. Findings indicate law enforcement responses to juvenile prostitution are influential in determining whether such youth are viewed as victims of commercial sexual exploitation or as delinquents.

  14. MHC-compatible bone marrow stromal/stem cells trigger fibrosis by activating host T cells in a scleroderma mouse model.

    Science.gov (United States)

    Ogawa, Yoko; Morikawa, Satoru; Okano, Hideyuki; Mabuchi, Yo; Suzuki, Sadafumi; Yaguchi, Tomonori; Sato, Yukio; Mukai, Shin; Yaguchi, Saori; Inaba, Takaaki; Okamoto, Shinichiro; Kawakami, Yutaka; Tsubota, Kazuo; Matsuzaki, Yumi; Shimmura, Shigeto

    2016-01-26

    Fibrosis of organs is observed in systemic autoimmune disease. Using a scleroderma mouse, we show that transplantation of MHC compatible, minor antigen mismatched bone marrow stromal/stem cells (BMSCs) play a role in the pathogenesis of fibrosis. Removal of donor BMSCs rescued mice from disease. Freshly isolated PDGFRα(+) Sca-1(+) BMSCs expressed MHC class II following transplantation and activated host T cells. A decrease in FOXP3(+) CD25(+) Treg population was observed. T cells proliferated and secreted IL-6 when stimulated with mismatched BMSCs in vitro. Donor T cells were not involved in fibrosis because transplanting T cell-deficient RAG2 knock out mice bone marrow still caused disease. Once initially triggered by mismatched BMSCs, the autoimmune phenotype was not donor BMSC dependent as the phenotype was observed after effector T cells were adoptively transferred into naïve syngeneic mice. Our data suggest that minor antigen mismatched BMSCs trigger systemic fibrosis in this autoimmune scleroderma model.

  15. Cyclophosphamide-refractory scleroderma-associated interstitial lung disease: remarkable clinical and radiological response to a single course of rituximab combined with high-dose corticosteroids.

    LENUS (Irish Health Repository)

    Haroon, Muhammad

    2011-10-01

    We would like to report our experience of using rituximab in cyclophosphamide refractory, rapidly progressive interstitial lung disease (ILD) in a patient with limited scleroderma. A 40-year-old man presented with 10-week history of inflammatory polyarthritis, which responded to a short course of oral corticosteroids. However, 3 weeks later, he developed new onset of exertional dyspnoea. High-resolution CT of the thorax was suggestive of early ILD. Surgical lung biopsy showed features of fibrotic non-specific interstitial pneumonia. He was diagnosed with scleroderma on the basis of: presence of anticentromere antibodies, Raynaud\\'s phenomenon, pulmonary fibrosis, digital oedema and hypomotility along with a dilated oesophagus. He was treated aggressively with pulse doses of corticosteroids and cyclophosphamide; however, his ILD continued to deteriorate. At this stage, he received rituximab (two pulses of 1 g each), which led to a gradual clinical improvement. Now, 12 months since his rituximab infusion, he walks 2 miles daily without any exertional dyspnoea.

  16. Juvenile myoclonic epilepsy Epilepsia mioclônica juvenil

    Directory of Open Access Journals (Sweden)

    Isabel Alfradique

    2007-12-01

    Full Text Available Juvenile myoclonus epilepsy (JME is a common epileptic syndrome, the etiology of which is genetically determined. Its onset occurs from 6 through 22 years of age, and affected patients present with myoclonic jerks, often associated with generalized tonic-clonic seizures - the most common association - and absence seizures. JME is non-progressive, and there are no abnormalities on clinical examination or intellectual deficits. Psychiatric disorders may coexist. Generalized polyspike-and-waves are the most characteristic electroencephalographic pattern. Usual neuroimaging studies show no abnormalities. Atypical presentations should be entertained, as they are likely to induce misdiagnosis. Prevention of precipitating factors and therapy with valproic acid (VPA are able to control seizures in the great majority of patients. Whenever VPA is judged to be inappropriate, other antiepileptic drugs such as lamotrigine may be considered. Treatment should not be withdrawn, otherwise recurrences are frequent.A epilepsia mioclônica juvenil é uma síndrome epiléptica comum, cuja etiologia é fundamentada na genética. Inicia-se entre 6 e 22 anos e os indivíduos apresentam mioclonias, que podem ser acompanhadas por crises tônico-clônicas generalizadas - associação mais comum - e crises de ausência. A doença não é progressiva, e não há alterações detectáveis no exame físico ou déficits intelectuais. Distúrbios psiquiátricos podem coexistir. Polipontas-ondas lentas generalizadas constituem o padrão eletrencefalográfico ictal típico. Não há anormalidades em exames de imagem convencionais. Apresentações atípicas devem ser consideradas, pois predispõem a erros de diagnóstico. A prevenção de fatores desencadeantes e o uso de ácido valpróico (VPA controlam as crises epilépticas na grande maioria dos casos. Quando o VPA é inapropriado, outras drogas como a lamotrigina podem ser utilizadas. O tratamento não deve ser interrompido

  17. Juvenile idiopathic arthritis and the temporomandibular joint ...

    African Journals Online (AJOL)

    ... resonance imaging findings of temporomandibular joint inflammation among juvenile ... The mean total MRI score was significantly higher in patients with active ... Clinical signs of TMJ arthritis can be used as filter for MRI examination TMJ is ...

  18. Bilateral giant juvenile fibroadenoma of breasts

    Directory of Open Access Journals (Sweden)

    Mukhopadhyay Madhumita

    2009-01-01

    Full Text Available An 11-year-old girl with rapidly enlarging bilateral breast lumps is reported. It was diagnosed as a case of juvenile fibroadenoma following fine needle aspiration cytology and confirmed on histopathological examination of the excised specimens.

  19. Genetics Home Reference: juvenile Paget disease

    Science.gov (United States)

    ... Information & Resources MedlinePlus (1 link) Health Topic: Bone Diseases Genetic and Rare Diseases Information Center (1 link) Juvenile ... on PubMed Daroszewska A, Ralston SH. Mechanisms of disease: genetics of Paget's disease of bone and related disorders. ...

  20. Genetics Home Reference: juvenile hyaline fibromatosis

    Science.gov (United States)

    ... Antaya RJ, Cajaiba MM, Madri J, Lopez MA, Ramirez MC, Martignetti JA, Reyes-Múgica M. Juvenile hyaline ... 103. Citation on PubMed Dowling O, Difeo A, Ramirez MC, Tukel T, Narla G, Bonafe L, Kayserili ...

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

    African Journals Online (AJOL)

    Acute Lymphoblastic Leukaemia presenting as Juvenile Idiopathic Arthritis in a Nigerian boy. ... lead to delay in commencing appropriate treatment. ... of two months duration, had an elevated Rheumatoid factor and X-ray findings suggestive of ...

  2. Screening Incarcerated Juveniles Using the MAYSI-2.

    Science.gov (United States)

    Gilbert, Amy L; Grande, Todd L; Hallman, Janelle; Underwood, Lee A

    2015-01-01

    The high prevalence of mental health disorders among incarcerated juveniles is a matter of national and global concern. Juvenile justice personnel need accurate screening measures that identify youth requiring immediate mental health services. The purpose of this study was threefold: (a) to examine the utility of the Massachusetts Youth Screening Instrument, Version 2 (MAYSI-2) in identifying juveniles with mental health concerns in a large sample of juveniles (N = 4,009), (b) to provide data regarding rates of identified mental health needs in incarcerated youth, and (c) to provide descriptive comparisons to other studies using the MAYSI-2. Mean scores of subscales were compared with the MAYSI-2 normative samples and other recent studies. Results indicated that this population has a high occurrence of mental health symptoms and there is high variability in the severity of the symptoms. In addition, a multivariate analysis of variance test found significant differences in mental health problems across ethnic groups.

  3. SAB Juvenile Reef Fish (2002-2015)

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — Trawls were made during the summer months in shallow seagrass beds to monitor the number and species of juvenile snapper using the grass as a nursery.

  4. AFSC/ABL: Juvenile rockfish habitat utilization

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — Juvenile rockfish were observed amongst coral, sponge, cobble, and gravel habitats. Rockfish utilized coral habitats more than any other, while gravel was the least...

  5. Juvenile Delinquency in Romania: The Indirect Result of the Transition Process

    Directory of Open Access Journals (Sweden)

    Andrea Fabian

    2010-11-01

    Full Text Available The general purpose of the research is to present the evolution of juvenile delinquency in Romania after 1989, with some of its social and psychological aspects. We use a comparative perspective: the general, Romanian framework will be compared with local situation occurred in Cluj county.The presentation is based on an extensive study of 420 delinquent juveniles, whose data on family situation, education, age, etc were analyzed and the youngsters were also tested with psychological tests. Looking at the data of the psychological tests (Nowicki & Strickland's Internal-External Control Scale for Children, McGuire & Priestley's Testing Your Reaction, Zuckerman-Kuhlman's Personality Questionnaire and Tucker's Inmate Dilemma Test and the descriptors of the social situation of juvenile delinquents, it looks like personality factors are associated with low school performance and school drop-out, as important determinants of delinquency. An integrative model was tested, to analyze the social and cognitive determinants of delinquency.

  6. Spatial distribution of juvenile and adult female Tanner crabs (Chionoecetes bairdi) in a glacial fjord ecosystem: Implications for recruitment processes

    Science.gov (United States)

    Nielsen, J.K.; Taggart, S.J.; Shirley, T.C.; Mondragon, J.

    2007-01-01

    A systematic pot survey in Glacier Bay, Alaska, was conducted to characterize the spatial distribution of juvenile and adult female Tanner crabs, and their association with depth and temperature. The information was used to infer important recruitment processes for Tanner crabs in glaciated ecosystems. High-catch areas for juvenile and adult female Tanner crabs were identified using local autocorrelation statistics. Spatial segregation by size class corresponded to features in the glacial landscape: high-catch areas for juveniles were located at the distal ends of two narrow glacial fjords, and high-catch areas for adults were located in the open waters of the central Bay. Juvenile female Tanner crabs were found at nearly all sampled depths (15-439 m) and temperatures (4-8??C), but the biggest catches were at depths crabs. ?? 2007 International Council for the Exploration of the Sea. Published by Oxford Journals. All rights reserved.

  7. Association of VDR ApaI and TaqI Gene Polymorphisms with the Risk of Scleroderma and Behçet's Disease.

    Science.gov (United States)

    Kamal, Asmaa; Gamal, Sherif M; Elgengehy, Fatema T; Alkemary, Alkhateeb K; Siam, Ibrahem

    2016-08-01

    Vitamin D deficiency and vitamin D receptor (VDR) gene polymorphisms have been reported in autoimmune diseases. However, their role in Behçet's disease (BD) and scleroderma remains inconclusive. Our aim was to evaluate vitamin D receptor (ApaI, TaqI) gene polymorphisms in relation to Behçet's disease and scleroderma in Egyptians. The study was conducted on 54 patients with BD, 30 scleroderma patients, and 60 healthy control subjects. VDR (ApaI, TaqI) gene polymorphisms were investigated using polymerase chain reaction-restriction fragment length polymorphism technique. The "a" allele of ApaI (A/a) polymorphism was significantly associated with increased BD risk (OR = 2.09, 95% CI = 1.18-3.71, p = 0.011), while the TaqI "tt" genotype was significantly lower in BD patients as compared to control subjects (OR = 0.35, 95% CI = 0.13-0.9, p = 0.026). Carriage of "aT" VDR haplotype was significantly associated with higher BD risk (OR = 2.28, 95% = 1.14-4.56, p = 0.022). In conclusion, our findings suggest that VDR gene polymorphisms have a significant association with BD in Egyptian patients.

  8. An Empirical Evaluation of Juvenile Awareness Programs in the United States: Can Juveniles Be "Scared Straight"?

    Science.gov (United States)

    Klenowski, Paul M.; Bell, Keith J.; Dodson, Kimberly D.

    2010-01-01

    Juvenile awareness programs like Scared Straight became popular crime prevention strategies during the 1970s. Juvenile offenders and at-risk youth who participate in these programs are taken to prisons where inmates use confrontational methods to recount stories about violence, sex, and abuse perpetrated by fellow inmates while living a life…

  9. Juvenile fibromyalgia: Guidance for management.

    Science.gov (United States)

    Yokota, Shumpei; Kikuchi, Masako; Miyamae, Takako

    2013-08-01

    Juvenile fibromyalgia (JFM) is a disease in which patients complain of acute and chronic severe pain, an overt primary cause for which cannot be found or surmised. Although patients with JFM mainly complain of systemic pain or allodynia in the medical interview and physical examination, the concept of the disease is the total sum of painful illness, chronic fatigue, hypothermia and many other autonomic symptoms and signs. Many issues are interacting including individual traits (personality, temperament, sensitivity, memory of pain; age: early adolescence), individual states (self-esteem, anxiety, developmental level), and external stressors (parent especially mother, school environment). JFM is diagnosed on the combination of disease history, physical examination to determine the 18 tender points and allodynia, pain from gently touching their hair, and negative results of blood tests (inflammatory markers, thyroid function, myogenic enzymes). The goals of treatment are the following: restoration of function and relief of pain. Psychological support is advocated. Although the exact number of patients with JFM is still to be elucidated, it seems to be growing because pediatric rheumatologists in Japan encounter children with a wide variety of musculoskeletal pains. This guideline describes how to diagnose JFM in children and how to treat them appropriately.

  10. Academic Achievement Among Juvenile Detainees.

    Science.gov (United States)

    Grigorenko, Elena L; Macomber, Donna; Hart, Lesley; Naples, Adam; Chapman, John; Geib, Catherine F; Chart, Hilary; Tan, Mei; Wolhendler, Baruch; Wagner, Richard

    2015-01-01

    The literature has long pointed to heightened frequencies of learning disabilities (LD) within the population of law offenders; however, a systematic appraisal of these observations, careful estimation of these frequencies, and investigation of their correlates and causes have been lacking. Here we present data collected from all youth (1,337 unique admissions, mean age 14.81, 20.3% females) placed in detention in Connecticut (January 1, 2010-July 1, 2011). All youth completed a computerized educational screener designed to test a range of performance in reading (word and text levels) and mathematics. A subsample (n = 410) received the Wide Range Achievement Test, in addition to the educational screener. Quantitative (scale-based) and qualitative (grade-equivalence-based) indicators were then analyzed for both assessments. Results established the range of LD in this sample from 13% to 40%, averaging 24.9%. This work provides a systematic exploration of the type and severity of word and text reading and mathematics skill deficiencies among juvenile detainees and builds the foundation for subsequent efforts that may link these deficiencies to both more formal, structured, and variable definitions and classifications of LD, and to other types of disabilities (e.g., intellectual disability) and developmental disorders (e.g., ADHD) that need to be conducted in future research.

  11. Joint Injection/Aspiration

    Science.gov (United States)

    ... Pharyngitis, Adenitis Syndrome (Juvenile) Polymyalgia Rheumatica Psoriatic Arthritis Raynaud's Phenomenon Reactive Arthritis Rheumatoid Arthritis Scleroderma Sjogren's Syndrome Spinal Stenosis Spondyloarthritis Systemic Lupus Erythematosus (Juvenile) Takayasu's ...

  12. Carpal Tunnel Syndrome

    Science.gov (United States)

    ... Pharyngitis, Adenitis Syndrome (Juvenile) Polymyalgia Rheumatica Psoriatic Arthritis Raynaud's Phenomenon Reactive Arthritis Rheumatoid Arthritis Scleroderma Sjogren's Syndrome Spinal Stenosis Spondyloarthritis Systemic Lupus Erythematosus (Juvenile) Takayasu's ...

  13. Giant Cell Arteritis

    Science.gov (United States)

    ... Pharyngitis, Adenitis Syndrome (Juvenile) Polymyalgia Rheumatica Psoriatic Arthritis Raynaud's Phenomenon Reactive Arthritis Rheumatoid Arthritis Scleroderma Sjogren's Syndrome Spinal Stenosis Spondyloarthritis Systemic Lupus Erythematosus (Juvenile) Takayasu's ...

  14. Find a Rheumatologist

    Science.gov (United States)

    ... Pharyngitis, Adenitis Syndrome (Juvenile) Polymyalgia Rheumatica Psoriatic Arthritis Raynaud's Phenomenon Reactive Arthritis Rheumatoid Arthritis Scleroderma Sjogren's Syndrome Spinal Stenosis Spondyloarthritis Systemic Lupus Erythematosus (Juvenile) Takayasu's ...

  15. Granulomatosis with Polyangiitis (Wegener's)

    Science.gov (United States)

    ... Pharyngitis, Adenitis Syndrome (Juvenile) Polymyalgia Rheumatica Psoriatic Arthritis Raynaud's Phenomenon Reactive Arthritis Rheumatoid Arthritis Scleroderma Sjogren's Syndrome Spinal Stenosis Spondyloarthritis Systemic Lupus Erythematosus (Juvenile) Takayasu's ...

  16. Spinal Stenosis

    Science.gov (United States)

    ... Pharyngitis, Adenitis Syndrome (Juvenile) Polymyalgia Rheumatica Psoriatic Arthritis Raynaud's Phenomenon Reactive Arthritis Rheumatoid Arthritis Scleroderma Sjogren's Syndrome Spinal Stenosis Spondyloarthritis Systemic Lupus Erythematosus (Juvenile) Takayasu's ...

  17. Antinuclear Antibodies (ANA)

    Science.gov (United States)

    ... Pharyngitis, Adenitis Syndrome (Juvenile) Polymyalgia Rheumatica Psoriatic Arthritis Raynaud's Phenomenon Reactive Arthritis Rheumatoid Arthritis Scleroderma Sjogren's Syndrome Spinal Stenosis Spondyloarthritis Systemic Lupus Erythematosus (Juvenile) Takayasu's ...

  18. What Is a Rheumatologist?

    Science.gov (United States)

    ... Pharyngitis, Adenitis Syndrome (Juvenile) Polymyalgia Rheumatica Psoriatic Arthritis Raynaud's Phenomenon Reactive Arthritis Rheumatoid Arthritis Scleroderma Sjogren's Syndrome Spinal Stenosis Spondyloarthritis Systemic Lupus Erythematosus (Juvenile) Takayasu's ...

  19. Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome (PFAPA)

    Science.gov (United States)

    ... Pharyngitis, Adenitis Syndrome (Juvenile) Polymyalgia Rheumatica Psoriatic Arthritis Raynaud's Phenomenon Reactive Arthritis Rheumatoid Arthritis Scleroderma Sjogren's Syndrome Spinal Stenosis Spondyloarthritis Systemic Lupus Erythematosus (Juvenile) Takayasu's ...

  20. Sjögren's Syndrome

    Science.gov (United States)

    ... Pharyngitis, Adenitis Syndrome (Juvenile) Polymyalgia Rheumatica Psoriatic Arthritis Raynaud's Phenomenon Reactive Arthritis Rheumatoid Arthritis Scleroderma Sjogren's Syndrome Spinal Stenosis Spondyloarthritis Systemic Lupus Erythematosus (Juvenile) Takayasu's ...

  1. NSAIDs (Nonsteroidal Anti-inflammatory Drugs)

    Science.gov (United States)

    ... Pharyngitis, Adenitis Syndrome (Juvenile) Polymyalgia Rheumatica Psoriatic Arthritis Raynaud's Phenomenon Reactive Arthritis Rheumatoid Arthritis Scleroderma Sjogren's Syndrome Spinal Stenosis Spondyloarthritis Systemic Lupus Erythematosus (Juvenile) Takayasu's ...

  2. HCV and Rheumatic Disease

    Science.gov (United States)

    ... Pharyngitis, Adenitis Syndrome (Juvenile) Polymyalgia Rheumatica Psoriatic Arthritis Raynaud's Phenomenon Reactive Arthritis Rheumatoid Arthritis Scleroderma Sjogren's Syndrome Spinal Stenosis Spondyloarthritis Systemic Lupus Erythematosus (Juvenile) Takayasu's ...

  3. Metabolic Myopathies

    Science.gov (United States)

    ... Pharyngitis, Adenitis Syndrome (Juvenile) Polymyalgia Rheumatica Psoriatic Arthritis Raynaud's Phenomenon Reactive Arthritis Rheumatoid Arthritis Scleroderma Sjogren's Syndrome Spinal Stenosis Spondyloarthritis Systemic Lupus Erythematosus (Juvenile) Takayasu's ...

  4. Polymyalgia Rheumatica

    Science.gov (United States)

    ... Pharyngitis, Adenitis Syndrome (Juvenile) Polymyalgia Rheumatica Psoriatic Arthritis Raynaud's Phenomenon Reactive Arthritis Rheumatoid Arthritis Scleroderma Sjogren's Syndrome Spinal Stenosis Spondyloarthritis Systemic Lupus Erythematosus (Juvenile) Takayasu's ...

  5. American College of Rheumatology

    Science.gov (United States)

    ... Pharyngitis, Adenitis Syndrome (Juvenile) Polymyalgia Rheumatica Psoriatic Arthritis Raynaud's Phenomenon Reactive Arthritis Rheumatoid Arthritis Scleroderma Sjogren's Syndrome Spinal Stenosis Spondyloarthritis Systemic Lupus Erythematosus (Juvenile) Takayasu's ...

  6. Pendidikan Agama Islam Sebagai Pencegah Juvenile Delinquency

    Directory of Open Access Journals (Sweden)

    Yuli Choirul Umah

    2016-09-01

    Full Text Available The challenges of Islamic education in the era of globalization are getting stronger now. It’s visible clearly changes happening so fast. The rapid of globalization is not only affect for adults, but also children, adolescents. A problem that often arises in the community revolves around the problems of Juvenile (teenagers, education and social community. Because adolescence is known as self-identity searching, so teens that can fulfill their role will have a positive impact, such as children understand their responsibilities better, and if they cannot, then there will emerge the exact opposite behavior that occurs an aberration or delinquency (juvenile delinquency. The existence of juvenile delinquency at this time also affect increasing in crime or criminal behavior in community. Juvenile delinquency can destroy moral values, the noble values ​​of religion, and the various aspects of the subject matter contained therein. Understanding, deepening, and adherence to the teachings of religion, especially Islamic education is required by the juvenile. Because Islamic education is a systematic effort by educators and adults to students both physical and spiritual by Islamic law to led the formation of personality according to the standard of Islam. Because in fact the children or adolescents who commit delinquency or crime mostly less understand the norms of Islam, perhaps they are negligent in fulfill the commandments of religion.

  7. PENDIDIKAN AGAMA ISLAM SEBAGAI PENCEGAH JUVENILE DELINQUENCY

    Directory of Open Access Journals (Sweden)

    Yuli Choirul Umah

    2016-09-01

    Full Text Available The challenges of Islamic education in the era of globalization are getting stronger now. It’s visible clearly changes happening so fast. The rapid of globalization is not only affect for adults, but also children, adolescents. A problem that often arises in the community revolves around the problems of Juvenile (teenagers, education and social community. Because adolescence is known as self-identity searching, so teens that can fulfill their role will have a positive impact, such as children understand their responsibilities better, and if they cannot, then there will emerge the exact opposite behavior that occurs an aberration or delinquency (juvenile delinquency. The existence of juvenile delinquency at this time also affect increasing in crime or criminal behavior in community. Juvenile delinquency can destroy moral values, the noble values of religion, and the various aspects of the subject matter contained therein. Understanding, deepening, and adherence to the teachings of religion, especially Islamic education is required by the juvenile. Because Islamic education is a systematic effort by educators and adults to students both physical and spiritual by Islamic law to led the formation of personality according to the standard of Islam. Because in fact the children or adolescents who commit delinquency or crime mostly less understand the norms of Islam, perhaps they are negligent in fulfill the commandments of religion.

  8. Psychiatric and Medical Health Care Policies in Juvenile Detention Facilities

    Science.gov (United States)

    Pajer, Kathleen A.; Kelleher, Kelly; Gupta, Ravindra A.; Rolls, Jennifer; Gardner, William

    2007-01-01

    A study aims to examine the existing health care policies in U.S. juvenile detention centres. The results conclude that juvenile detention facilities have many shortfalls in providing care for adolescents, particularly mental health care.

  9. AFSC/ABL: Juvenile rockfish DNA species identification

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — Many pelagic juvenile rockfish (Sebastes) were collected in juvenile salmonid surveys in the Gulf of Alaska (GOA) from 1998 to 2002. Often species identification of...

  10. A case report of juvenile Huntington disease

    Directory of Open Access Journals (Sweden)

    Anita Choudhary

    2017-09-01

    Full Text Available Huntington disease (HD is a progressive neurodegenerative disorder, characterized by autosomal dominant inheritance, movement disorder, dementia, and behavioural disturbances. It is caused by a mutation in IT15 gene on chromosome 4p16.3, which leads to unstable CAG trinucleotide repeat expansion. The onset of juvenile HD occurs before the 2nd decade of life and comprises approximately 10% of total HD patients. Juvenile HD differs in symptomatology and is usually transmitted from paternal side with genetic anticipation phenomenon. Magnetic resonance imaging (MRI of the brain shows specific changes of early affection of caudate nucleus and putamen. Multidisciplinary approach with symptomatic treatment of specific symptoms is the current available management. Gene editing and gene silencing treatment are under trial. Hereby, we introduce a case of an 8-year-old boy, who presented with typical symptoms of juvenile HD, positive family history with genetic anticipation phenomenon and characteristic MRI findings.

  11. Corporal and capital punishment of juveniles.

    Science.gov (United States)

    Frazier, H C

    1990-01-01

    There is a previously unobserved connection between corporal punishment of public school children and capital punishment of juveniles. Both are barometers of acceptable levels of violent punishment and their elimination is a hallmark of a maturing and decent society. Within a majority of the eighteen states where school authorities most frequently strike children are housed 25 of the nation's 28 juvenile death row inmates. On average, the homicide rates of these jurisdictions are two and a half times greater than those that have abolished both state-sanctioned corporal and capital punishment or limit death sentences to those age eighteen and older at the time of their crime(s). Most of the eighteen state abolitions of corporal punishment occurred in the 1980's. The US Supreme Court has ruled both corporal and capital punishment of juveniles constitutional. Additional state legislative abolition of both is anticipated in the 1990s.

  12. Delincuencia y responsabilidad penal juvenil en Colombia

    Directory of Open Access Journals (Sweden)

    Cristina Montalvo Velásquez

    2011-01-01

    Full Text Available ResumenEl término «delincuencia juvenil» fue acuñado en Inglaterra en el año 1815, “Se entiende por delincuencia juvenil el conjunto de delitos, contravenciones o comportamientos socialmente reprochables, que cometen las personas consideradas como jóvenes por la ley”1 . Cada Estado está sujeto a su propio sistema jurídico, para algunos es delincuente juvenil el adolescente que comete acciones sancionadas por la ley sin importar su gravedad, otros Estados sólo consideran como delincuente juvenil al joven que comete un acto delictivo grave.El fenómeno de la delincuencia juvenil es algo que se inscribe en los espacios de una sociedad en la cual su estructura material, y su formación social consecuente, se halla en una profunda crisis. Que jóvenes conformen bandas de delincuencia organizada nos está indicando que son el resultado de la misma criminalidad general que se ha apoderado de la sociedad en la perspectiva de lograr sobrevivir materialmente. El capitalismo no es sólo acumulación de riqueza sino concentración de la misma en muy pocas manos; y todo el sistema institucional y legal tiende a favorecer ese fenómeno porque éste constituye la supra estructura del modo de producción capitalista. Así como los adultos se organizan para delinquir, lo hacen los niños y los jóvenes a partir de una edad en la cual pueden percibir que la sociedad no es sana y no tienen porvenir humano en ella. Abandonados y sujetos a la violencia que engendra el sistema, ellos simplemente responden en una manifestación de reflejos condicionados que sostienen la sobrevivencia en forma instintiva; “los niños no saben de normas legales sino de formas de sobrevivir a semejante situación; el instinto de sobrevivencia no tiene edades ni la normatividad puede incidir en él”.Palabras ClavesDelincuencia juvenil, Jóvenes, Criminalidad, Familia, Factores, Acto delictivo, Responsabilidad Penal.AbstractThe term “juvenile delinquency” was coined in

  13. Immunization Coverage Among Juvenile Justice Detainees.

    Science.gov (United States)

    Gaskin, Gregory L; Glanz, Jason M; Binswanger, Ingrid A; Anoshiravani, Arash

    2015-07-01

    This study sought to (1) quantify the baseline immunization coverage of adolescents entering the juvenile justice system and (2) assess the effect of detention-based care on immunization coverage in youth. A cross-sectional retrospective chart review was performed of 279 adolescents detained at a large juvenile detention facility. Only 3% of adolescents had received all study immunizations prior to detention. Before detention, immunization coverage was significantly lower than that for the general adolescent population for all vaccines except the first doses of hepatitis A and varicella-zoster virus vaccines. Subsequent to detention, most individual immunization coverage levels increased and were significantly higher than in the general adolescent population. The routine administration of immunizations in the juvenile justice setting can help detained youth achieve levels of immunization coverage similar to their nondetained peers.

  14. Effect of TBT on Ruditapes decussatus juveniles.

    Science.gov (United States)

    Coelho, M R; Langston, W J; Bebianno, M J

    2006-06-01

    The effects of sublethal concentrations of tributyltin (TBT) on growth of juvenile clams Ruditapes decussatus were determined during exposure to TBT concentrations of 50, 100 and 250 ng l(-1) (as Sn) for a period up to two years. Length and weight of clams increased continuously in all treatments throughout the experimental period, and, overall, rates were not significantly influenced by TBT exposure, although final length and weight were inversely related to increasing TBT concentration. Juvenile R. decussatus therefore appear to be less sensitive to TBT than larval stages. Some juveniles exposed to TBT developed abnormal shell growth, laterally, changing the typical flattened shape of clams into a more "rounded" form. This characteristic was more visible in the anterior margins of valves than posteriorly, and mainly observed in clams exposed to TBT at 50 ng l(-1) (as Sn).

  15. Juvenile Fibromyalgia: A Multidisciplinary Approach to Treatment.

    Science.gov (United States)

    Tesher, Melissa S

    2015-06-01

    A 14-year-old boy presented with months of severe widespread musculoskeletal pain. He was profoundly fatigued and unable to attend school. Laboratory evaluation, including complete blood count, comprehensive metabolic panel, inflammatory markers, and thyroid function, was unrevealing. Physical examination was also normal except for multiple tender points. The patient was diagnosed with juvenile primary fibromyalgia syndrome and referred for multidisciplinary treatment including physical therapy, exercise, and counseling, and his daily functioning gradually improves. Juvenile fibromyalgia is a complex syndrome that often severely limits patients' activities and can impede normal adolescent development. Effective treatment requires an understanding of the biologic, psychologic, and social factors contributing to the perpetuation of chronic pain. The author reviews the diagnostic criteria, pathophysiology, and treatment of juvenile fibromyalgia. Medications, particularly antidepressants and anticonvulsants, can be useful adjuncts to therapy. However, multimodal pain management including intensive physical therapy, exercise, counseling, and sleep hygiene is most effective in treating fibromyalgia.

  16. A juvenile subfossil crocodylian from Anjohibe Cave, Northwestern Madagascar

    Directory of Open Access Journals (Sweden)

    Joshua C. Mathews

    2016-09-01

    Full Text Available Madagascar’s subfossil record preserves a diverse community of animals including elephant birds, pygmy hippopotamus, giant lemurs, turtles, crocodiles, bats, rodents, and carnivorans. These fossil accumulations give us a window into the island’s past from 80,000 years ago to a mere few hundred years ago, recording the extinction of some groups and the persistence of others. The crocodylian subfossil record is limited to two taxa, Voay robustus and Crocodylus niloticus, found at sites distributed throughout the island. V. robustus is extinct while C. niloticus is still found on the island today, but whether these two species overlapped temporally, or if Voay was driven to extinction by competing with Crocodylus remains unknown. While their size and presumed behavior was similar to each other, nearly nothing is known about the growth and development of Voay, as the overwhelming majority of fossil specimens represent mature adult individuals. Here we describe a nearly complete juvenile crocodylian specimen from Anjohibe Cave, northwestern Madagascar. The specimen is referred to Crocodylus based on the presence of caviconchal recesses on the medial wall of the maxillae, and to C. niloticus based on the presence of an oval shaped internal choana, lack of rostral ornamentation and a long narrow snout. However, as there are currently no described juvenile specimens of Voay robustus, it is important to recognize that some of the defining characteristics of that genus may have changed through ontogeny. Elements include a nearly complete skull and many postcranial elements (cervical, thoracic, sacral, and caudal vertebrae, pectoral elements, pelvic elements, forelimb and hindlimb elements, osteoderms. Crocodylus niloticus currently inhabits Madagascar but is locally extinct from this particular region; radiometric dating indicates an age of ∼460–310 years before present (BP. This specimen clearly represents a juvenile based on the extremely small

  17. A juvenile subfossil crocodylian from Anjohibe Cave, Northwestern Madagascar

    Science.gov (United States)

    Samonds, Karen E.

    2016-01-01

    Madagascar’s subfossil record preserves a diverse community of animals including elephant birds, pygmy hippopotamus, giant lemurs, turtles, crocodiles, bats, rodents, and carnivorans. These fossil accumulations give us a window into the island’s past from 80,000 years ago to a mere few hundred years ago, recording the extinction of some groups and the persistence of others. The crocodylian subfossil record is limited to two taxa, Voay robustus and Crocodylus niloticus, found at sites distributed throughout the island. V. robustus is extinct while C. niloticus is still found on the island today, but whether these two species overlapped temporally, or if Voay was driven to extinction by competing with Crocodylus remains unknown. While their size and presumed behavior was similar to each other, nearly nothing is known about the growth and development of Voay, as the overwhelming majority of fossil specimens represent mature adult individuals. Here we describe a nearly complete juvenile crocodylian specimen from Anjohibe Cave, northwestern Madagascar. The specimen is referred to Crocodylus based on the presence of caviconchal recesses on the medial wall of the maxillae, and to C. niloticus based on the presence of an oval shaped internal choana, lack of rostral ornamentation and a long narrow snout. However, as there are currently no described juvenile specimens of Voay robustus, it is important to recognize that some of the defining characteristics of that genus may have changed through ontogeny. Elements include a nearly complete skull and many postcranial elements (cervical, thoracic, sacral, and caudal vertebrae, pectoral elements, pelvic elements, forelimb and hindlimb elements, osteoderms). Crocodylus niloticus currently inhabits Madagascar but is locally extinct from this particular region; radiometric dating indicates an age of ∼460–310 years before present (BP). This specimen clearly represents a juvenile based on the extremely small size and open

  18. Bacterial acquisition in juveniles of several broadcast spawning coral species.

    Directory of Open Access Journals (Sweden)

    Koty H Sharp

    Full Text Available Coral animals harbor diverse microorganisms in their tissues, including archaea, bacteria, viruses, and zooxanthellae. The extent to which coral-bacterial associations are specific and the mechanisms for their maintenance across generations in the environment are unknown. The high diversity of bacteria in adult coral colonies has made it challenging to identify species-specific patterns. Localization of bacteria in gametes and larvae of corals presents an opportunity for determining when bacterial-coral associations are initiated and whether they are dynamic throughout early development. This study focuses on the early onset of bacterial associations in the mass spawning corals Montastraea annularis, M. franksi, M. faveolata, Acropora palmata, A. cervicornis, Diploria strigosa, and A. humilis. The presence of bacteria and timing of bacterial colonization was evaluated in gametes, swimming planulae, and newly settled polyps by fluorescence in situ hybridization (FISH using general eubacterial probes and laser-scanning confocal microscopy. The coral species investigated in this study do not appear to transmit bacteria via their gametes, and bacteria are not detectable in or on the corals until after settlement and metamorphosis. This study suggests that mass-spawning corals do not acquire, or are not colonized by, detectable numbers of bacteria until after larval settlement and development of the juvenile polyp. This timing lays the groundwork for developing and testing new hypotheses regarding general regulatory mechanisms that control bacterial colonization and infection of corals, and how interactions among bacteria and juvenile polyps influence the structure of bacterial assemblages in corals.

  19. Bacterial acquisition in juveniles of several broadcast spawning coral species.

    Science.gov (United States)

    Sharp, Koty H; Ritchie, Kim B; Schupp, Peter J; Ritson-Williams, Raphael; Paul, Valerie J

    2010-05-28

    Coral animals harbor diverse microorganisms in their tissues, including archaea, bacteria, viruses, and zooxanthellae. The extent to which coral-bacterial associations are specific and the mechanisms for their maintenance across generations in the environment are unknown. The high diversity of bacteria in adult coral colonies has made it challenging to identify species-specific patterns. Localization of bacteria in gametes and larvae of corals presents an opportunity for determining when bacterial-coral associations are initiated and whether they are dynamic throughout early development. This study focuses on the early onset of bacterial associations in the mass spawning corals Montastraea annularis, M. franksi, M. faveolata, Acropora palmata, A. cervicornis, Diploria strigosa, and A. humilis. The presence of bacteria and timing of bacterial colonization was evaluated in gametes, swimming planulae, and newly settled polyps by fluorescence in situ hybridization (FISH) using general eubacterial probes and laser-scanning confocal microscopy. The coral species investigated in this study do not appear to transmit bacteria via their gametes, and bacteria are not detectable in or on the corals until after settlement and metamorphosis. This study suggests that mass-spawning corals do not acquire, or are not colonized by, detectable numbers of bacteria until after larval settlement and development of the juvenile polyp. This timing lays the groundwork for developing and testing new hypotheses regarding general regulatory mechanisms that control bacterial colonization and infection of corals, and how interactions among bacteria and juvenile polyps influence the structure of bacterial assemblages in corals.

  20. Juvenile age estimation from facial images.

    Science.gov (United States)

    Ferguson, Eilidh; Wilkinson, Caroline

    2017-01-01

    Age determination from images can be of vital importance, particularly in cases involving suspected child sexual abuse (CSA). It is imperative to determine if an individual depicted in such an image is indeed a child, with a more concise age often sought, as this may affect the severity of offender sentencing. The aims of this study were to establish the accuracy of visual age estimation of the juvenile face in children aged between 0 and 16years and to determine if varying levels of exposure to children affected an individual's ability to assess age from the face. An online questionnaire consisting of 30 juvenile face images was created using SurveyMonkey®. The overall results suggested poor accuracy for visual age estimation of juvenile faces. The age, sex, occupation and number of children of the participants did not affect the ability to estimate age from facial images. Similarly, the sex and age of the juvenile faces did not appear to affect the accuracy of age estimation. When specific age groups are considered, sex may have an influence on age estimation, with female faces being aged more accurately in the younger age groups and male faces more accurate after the age of 11years, however this is based on a small sample. This study suggests that the accuracy of juvenile age estimation from the face alone is poor using simple visual assessment of images. Further research is required to determine exactly how age is assessed from a facial image, if there are indicators, or features in particular that lead to over- or under-estimation of juvenile age.

  1. Juvenile dermatomyositis in a Nigerian girl

    Science.gov (United States)

    Adelowo, Olufemi; Nwankwo, Madu; Olaosebikan, Hakeem

    2014-01-01

    Juvenile dermatomyositis is an autoimmune connective tissue disease occurring in children less than 16 years old. It is part of a heterogeneous group of muscle diseases called idiopathic Iiflammatory myopathies. It had previously been reported in black Africans resident in UK. However, there is no documented case reported from Africa. The index sign of heliotrope rashes is often difficult to visualise in the black skin. An 11-year-old Nigerian girl presenting with clinical, laboratory and histopathological features of juvenile dermatomyositis is presented here. It is hoped that this case will heighten the index of suspicion of this condition among medical practitioners in Africa. PMID:24706700

  2. Posttraumatic stress among youths in juvenile detention.

    Science.gov (United States)

    Falk, Derek; Thompson, Sanna J; Sanford, Julia

    2014-01-01

    An estimated 1.8 million juveniles were arrested in the United States for delinquency in 2009. Previous studies indicate high rates of exposure to traumatic events and posttraumatic stress (PTS) symptoms for these youths. This study examined PTS in a sample of 170 youths in juvenile detention. The results of this study reveal higher rates of PTS symptoms (21%) compared to national rates (6%). The data also suggest youths suffering from more PTS symptoms also report higher depression, anxiety, anger, family relationship worries, thought problems, and attention problems. These factors provide a direction for continued practice targeting these youths.

  3. [HLA antigens in juvenile rheumatoid arthritis].

    Science.gov (United States)

    Rumba, I V; Sochnev, A M; Kukaĭne, E M; Burshteĭn, A M; Benevolenskaia, L I

    1990-01-01

    Antigens of I class HLA system (locus A and B) were investigated in 67 patients of Latvian nationality suffering from juvenile rheumatoid arthritis (JRA). Associations of HLA antigens with juvenile rheumatoid arthritis partially coincided with the ones revealed earlier. Typing established an increased incidence of antigen B27 (p less than 0.01) and gaplotype A2, B40 (p less than 0.01). Antigen B15 possessed a protective action with respect to JRA. Interlocus combinations demonstrated a closer association with the disease than a single antigen. The authors also revealed markers of various clinico-anatomical variants of JRA.

  4. Metabolic responses to hypoglycemia in juvenile diabetics

    DEFF Research Database (Denmark)

    Hilsted, J; Madsbad, S; Krarup, T;

    1980-01-01

    Glucagon and metabolic responses to insulin-induced hypoglycemia were studied in seven juvenile diabetics, age 31 +/- 2 years (mean and S.E.M.), duration of diabetes 17 +/- 3 years, with diabetic autonomic neuropathy (decreased beat-to-beat variation in heart rate during hyperventilation and...... in both patient groups. Metabolic responses to hypoglycemia were also similar in the two patient groups. In conclusion, diabetic autonomic neuropathy has no effect on glucagon and metabolic responses to hypoglycemia in juvenile, insulin-treated diabetics....

  5. Juvenil Pityriasis Rubra Pilaris: A Case Report

    Directory of Open Access Journals (Sweden)

    M. Emin YANIK et al.

    2010-05-01

    Full Text Available Juvenile pityriasis rubra pilaris (PRP is an uncommon skin disease characterized by follicularkeratotic papules, erythemato-squamous plaques and palmoplantar keratoderma. Etyology isunknown. A 8 years-old boy presented with a 15 days history of scaly patches and plaques withfollicular papules involving his scalp and face. However he had palmoplantar keratoderma onhis hands and feet. Based upon clinical and histopatological findings, he was diagnosed asJuvenile PRP. Acitretin was initiated for therapy. We presented our case because of its rarity.

  6. Acupuntura em adolescentes com fibromialgia juvenil

    OpenAIRE

    Dias,Marialda Höfling P.; Amaral,Elisabete; PAI, Hong Jin; Daniela Terumi Y. Tsai; LOTITO, Ana Paola N; Leone,Claudio; Silva, Clovis Artur

    2012-01-01

    OBJETIVO: Descrever a utilização da acupuntura em adolescentes com fibromialgia juvenil. MÉTODOS: Estudo retrospectivo realizado em pacientes com fibromialgia juvenil (critérios do Colégio Americano de Reumatologia) submetidos a, pelo menos, 11 sessões semanais de acupuntura. As avaliações antes e após acupuntura incluíram dados demográficos, características da dor musculoesquelética, número de pontos dolorosos (NPD), escala visual analógica (EVA) de dor, algiometria e índice miálgico (IM). D...

  7. Resiliency Scales for Children and Adolescents: Profiles of Juvenile Offenders

    Science.gov (United States)

    Mowder, Melissa H.; Cummings, Jack A.; McKinney, Robert

    2010-01-01

    An exploratory study of resiliency profiles of male and female juvenile offenders committed to a juvenile correctional facility was conducted. The goal of the present study was to examine juvenile offenders' positive characteristics (e.g., adaptability, optimism, self-efficacy, tolerance of differences). To assess positive characteristics and…

  8. Programa Shortstop: A Culturally Focused Juvenile Intervention for Hispanic Youth

    Science.gov (United States)

    Cervantes, Richard C.; Ruan, Karen; Duenas, Norma

    2004-01-01

    Culturally sensitive juvenile delinquency and substance abuse interventions are relatively limited and unavailable to many first-time Hispanic juvenile offenders. The purpose of this study was to test the effectiveness of a culturally focused juvenile and substance abuse intervention program for first time Hispanic youth offenders. The intent of…

  9. Increased Cyclooxygenase-2 Expression in Juvenile Polyposis Syndrome

    NARCIS (Netherlands)

    W.A. van Hattem; L.A.A. Brosens; S.Y. Marks; A.N.A. Milne; S. van Eeden; C.A. Iacobuzio-Donahue; A. Ristimäki; F.M. Giardiello; G.J.A. Offerhaus

    2009-01-01

    Background & Aims: Gastrointestinal juvenile polyps may occur in juvenile polyposis syndrome (JPS) or sporadically. JPS is an autosomal-dominant condition caused by a germline defect in SMAD4 or BMPR1A in 50% to 60% of cases, and is characterized by multiple juvenile polyps, predominantly in the col

  10. Chronic Juvenile Delinquency and the "Suppression Effect": An Exploratory Study.

    Science.gov (United States)

    Fraser, Mark; Norman, Michael

    1988-01-01

    Notes that fear of apprehension and punishment have been reported to suppress juvenile crime. Discusses suppression effect in regard to the correlates of chronic juvenile delinquency and exploratory evidence that youth who commit large volume of crime do not fear sanctions imposed by juvenile court any more than youth who commit only one offense…

  11. The challenges of the first migration: movement and behaviour of juvenile vs. adult white storks with insights regarding juvenile mortality.

    Science.gov (United States)

    Rotics, Shay; Kaatz, Michael; Resheff, Yehezkel S; Turjeman, Sondra Feldman; Zurell, Damaris; Sapir, Nir; Eggers, Ute; Flack, Andrea; Fiedler, Wolfgang; Jeltsch, Florian; Wikelski, Martin; Nathan, Ran

    2016-07-01

    Migration conveys an immense challenge, especially for juvenile birds coping with enduring and risky journeys shortly after fledging. Accordingly, juveniles exhibit considerably lower survival rates compared to adults, particularly during migration. Juvenile white storks (Ciconia ciconia), which are known to rely on adults during their first fall migration presumably for navigational purposes, also display much lower annual survival than adults. Using detailed GPS and body acceleration data, we examined the patterns and potential causes of age-related differences in fall migration properties of white storks by comparing first-year juveniles and adults. We compared juvenile and adult parameters of movement, behaviour and energy expenditure (estimated from overall dynamic body acceleration) and placed this in the context of the juveniles' lower survival rate. Juveniles used flapping flight vs. soaring flight 23% more than adults and were estimated to expend 14% more energy during flight. Juveniles did not compensate for their higher flight costs by increased refuelling or resting during migration. When juveniles and adults migrated together in the same flock, the juvenile flew mostly behind the adult and was left behind when they separated. Juveniles showed greater improvement in flight efficiency throughout migration compared to adults which appears crucial because juveniles exhibiting higher flight costs suffered increased mortality. Our findings demonstrate the conflict between the juveniles' inferior flight skills and their urge to keep up with mixed adult-juvenile flocks. We suggest that increased flight costs are an important proximate cause of juvenile mortality in white storks and likely in other soaring migrants and that natural selection is operating on juvenile variation in flight efficiency.

  12. Criminal Profiles of Violent Juvenile Sex and Violent Juvenile Non-Sex Offenders: An Explorative Longitudinal Study

    Science.gov (United States)

    van Wijk, Anton Ph.; Mali, Bas R. F.; Bullens, Ruud A. R.; Vermeiren, Robert R.

    2007-01-01

    Few studies have longitudinally investigated the criminal profiles of violent juvenile sex and violent juvenile non-sex offenders. To make up for this lack, this study used police records of juveniles to determine the nature of the criminal profiles of violent sex offenders (n = 226) and violent non-sex offenders (n = 4,130). All offenders…

  13. Juvenile Residential Facility Census, 2010: Selected Findings. Juvenile Offenders and Victims: National Report Series. Bulletin NCJ 241134

    Science.gov (United States)

    Hockenberry, Sarah; Sickmund, Melissa; Sladky, Anthony

    2013-01-01

    This bulletin is part of the "Juvenile Offenders and Victims National Report Series." The "National Report" offers a comprehensive statistical overview of the problems of juvenile crime, violence, and victimization and the response of the juvenile justice system. During each interim year, the bulletins in the "National…

  14. Freqüência de alterações hepáticas em pacientes com esclerodermia Frequency of hepatic abnormalities in patients with scleroderma

    Directory of Open Access Journals (Sweden)

    Ariene Paixão

    2007-10-01

    Full Text Available OBJETIVO: avaliar a freqüência das doenças hepáticas em pacientes com esclerodermia e, secundariamente, estudar a freqüência de infecção pelos vírus B e C da hepatite nesses pacientes, assim como a freqüência de auto-anticorpos séricos. MATERIAL E MÉTODOS: estudaram-se pacientes com diagnóstico de esclerodermia, localizada ou sistêmica, acompanhados no Ambulatório de Reumatologia do Hospital Santa Izabel. Como grupo de comparação, foram estudados pacientes com diagnóstico de acne vulgar. RESULTADOS: dos 65 pacientes com diagnóstico de esclerodermia incluídos nesse trabalho, 35% apresentaram a gama-glutamiltransferase (gama-GT alterada, 30% tiveram a fosfatase alcalina aumentada e 17,1%, a alaninoaminotransferase (ALT acima dos valores de referência. A ALT apresentou-se mais alterada nos pacientes do que nos controles. Apenas um indivíduo dos 41 testados apresentou positividade para o anticorpo antimitocôndria enquanto 19% tinham anticorpo antimúsculo liso, não se observando diferença estatística na positividade desses anticorpos entre os dois grupos. Um paciente apresentou o HBsAg positivo e outro foi positivo para o anticorpo anti-HCV. Nenhum paciente apresentou manifestações clínicas de doença hepática. CONCLUSÕES: no presente estudo, embora as alterações de enzimas hepáticas em pacientes com esclerodermia não tenham sido incomuns, não se observou nenhum caso com manifestações clínicas de doença hepática.INTRODUCTION: to determine the frequency of hepatic disease in patients with scleroderma and, secondarily, to investigate the frequency of hepatitis B and C virus infection and determine the frequency of autoantibodies in this disease. MATERIAL AND METHODS: patients with scleroderma followed at Hospital Santa Izabel were included in the study and patients with acne vulgaris served as a comparison group. RESULTS: considering the 65 scleroderma patients, 35% had elevated gamma

  15. Juvenile penalty or leniency: Sentencing of juveniles in the criminal justice system.

    Science.gov (United States)

    Jordan, Kareem L; McNeal, Brittani A

    2016-08-01

    The purpose of this study is to examine the impact of being juvenile on sentencing in the criminal justice system. More specifically, youth transferred to criminal court are compared to adults in terms of likelihood of incarceration, jail length, and prison length. In this study, 2 national data sets are merged. The juvenile sample includes 3,381 convicted offenders, and the adult sample is comprised of 6,529 convicted offenders. The final sample is 9,910 offenders across 36 U.S. counties. The key independent variable is juvenile status, and the dependent variables are incarceration, jail length, and prison length. Because of the multilevel nature of the data, hierarchical linear modeling is used across all models. Juveniles are punished less severely in the jail incarceration decision. However, when youth are actually sentenced to incarceration (either jail or prison), they are given longer confinement time than adults. (PsycINFO Database Record

  16. The Relationship between Serum Pro‐Brain Natriuretic Peptide (Pro‐BNP Levels and Pulmonary Arterial Hypertension (PAH in Patients with Limited Scleroderma

    Directory of Open Access Journals (Sweden)

    Seyedeh Zahra Mirfeizi

    2014-08-01

    Full Text Available Introduction: Pulmonary arterial hypertension (PAH is a late progressive sclerodermarelated complication, which can lead to right heart failure and cor pulmonale. Given that cardiac catheterization is a diagnostic method of choice for PAH, and considering the high risks of this method, the purpose of this study was to evaluate the relationship between serum Pro‐Brain natriuretic peptide (Pro‐BNP Levels and PAH in patients with limited scleroderma. Materials and Methods: In this cross sectional study , during June 2011‐ Dec 2013, referring patients to two major educational hospitals, Mashhad‐ Iran, with scleroderma, who were afflicted with the disease for at least two years (or more, were enrolled in the study if they met the inclusion and exclusion criteria. All the patients underwent echocardiography to determine the pulmonary artery pressure (PAP. Afterwards, the subjects were referred to a lung center for performing body plethysmography, carbon monoxide diffusing capacity (DLCO, and 6‐ minute walk test (6MWT. Pro‐BNP Serum level was determined using flourescent immune assay method. Results: The present study included 20 patients (18 female subjects with the mean age of 43.28±9.56 yrs, and the mean pro‐BNP level of 138 pg/ml. The logarithmic correlation between PAP values, Forced Vital Capacity /DLCO ratio, and pro‐BNP level, which was measured using Pearson's correlation coefficient, showed a significant association among these variables( respectively, r=0.76, P0.001; r=0.677, P=0.011. Moreover, the DLCO decrease was associated with increasing pro‐BNP level, though this relationship was not significant. Conclusion: This study showed that there was a significant relationship between the serum levels of pro‐BNP marker and increased PAP in the echocardiography, DLCO reduction, and FVC/DLCO increase. In fact, this serum marker can be used in patients with systemic scleroderma (SSc to evaluate the status of PAH.

  17. Scleroderma therapy:a review of recent advances and perspective%硬皮病的治疗现状与展望

    Institute of Scientific and Technical Information of China (English)

    陈卫; 杨蓉娅

    2015-01-01

    系统性硬皮病(systemic sclerosis,SSc)是一种慢性多系统的结缔组织疾病,其发病机制复杂。目前,尚无一种特效的治疗方法能检测硬皮病的发展,常需要多个临床科室对患者进行综合治疗。近年来,大量的临床研究揭示硬皮病的发病机制涉及多种细胞和分子因素,这就为未来产生新的治疗方法提供了可能。该文主要对目前的治疗方法和最新进展进行综述。这些治疗方法根据他们不同的作用机制,如免疫调节、抗纤维化及血管扩张剂等进行分类,同时对每种治疗方法进行分析讨论。%Scleroderma (systemic sclerosis, SSc) is a chronic, multisystem connective tissue disease with a complex pathogenesis. Till now there is no sole therapy with optimal beneficial effects on all aspects of the disease process. It usually requires interdisciplinary approach in individual patient management. Recently, Clinical research to unravel the pathogenesis of scleroderma has implicated multiple cellular and molecular factors that are potential targets for future therapy. The objective of this article is to provide an overview of the available treatments and recent advances in the therapy of Scleroderma. These therapies have been classified into categories according to their mode of action-immunomodulators, antifibrotic or vasodilators and each will be discussed individually.

  18. Analysis of 10 Years Based on Scleroderma Literature which Published in PUBMED%10年间基于PUBMED发表硬皮病文献的分析

    Institute of Scientific and Technical Information of China (English)

    贾红侠; 韩苏闽; 吴振君; 翟萌

    2015-01-01

    目的:分析10年间基于PUBMED发表硬皮病文献,为临床提供研究资料.方法:采用美国国立医学图书馆网站的PUBMED数据库自行检索,用PUBMEDPRO和GOPUBMED进行对照分析.结果:检索2003年PUBMED中硬皮病文献发文量,从年度486篇(占比7.4%)到2012年的780篇(占比12.0%),呈逐年递增趋势,总检出文献6 538篇.结论:采用主题词和自由词相结合的方法,从数量和质量两方面揭示文献的规律,国外发达国家比较重视硬皮病研究.%Objective: To analyze the scleroderma literatures among 10 years of PUBMED,to provide clinical research data. Methods:The U.S.national library of medicine website PUBMED database retrieval were used,the PUBMEDPRO and GOPUBMED were contrasted and analyzed.Results:In 2003 PUBMED document retrieval from the annual number of scleroderma were 486 pieces for 7.4% to 2012 of 780 pieces for 12%,showed an increasing trend year by year,the total out 6538 pieces of literature.Conclusion:The method of using subject headings and text words combining literature,reveals the law from two aspects of quantity and quality,compared to the scleroderma research in foreign countries.

  19. Ethnic monitoring and social control: Descriptions from juveniles in juvenile care institutions

    OpenAIRE

    Basic, Goran

    2014-01-01

    Previous research has emphasized the institutional racism in total institutions. Researchers have highlighted the importance of narratives but have not focused on narratives about ethnic monitoring and social control. This article tries to fill this gap by analysing stories related to descriptions of ethnic monitoring and social control as told by juveniles of non-Swedish ethnicity in Swedish juvenile care institutions. A juvenile’s ethnicity was highlighted by drawing attention to the staff’...

  20. Secondary Osteoporosis in Patients with Juvenile Idiopathic Arthritis

    Directory of Open Access Journals (Sweden)

    Kristyna Brabnikova Maresova

    2011-01-01

    Full Text Available Bone disease in patients with juvenile idiopathic arthritis (JIA is associated with focal (joint erosion and juxtaarticular osteopenia and systemic bone loss (generalized osteopenia or reduction of bone mass density. Pathophysiology of bone loss is multifactorial and involves particularly proinflammatory cytokines and deleterious effects of glucocorticoid therapy. Clinical studies in patients with JIA indicate excessive activation of osteoclastogenesis and reduction of bone formation. Reduction of physical activity, muscle atrophy caused by high disease activity, and compulsory restriction in movements are also associated with bone loss. In patients with JIA, the disease can be complicated by growth cartilage involvement and systemic or local growth retardation. In the absence of preventive measures, fragility fractures can occur even at an early age.

  1. Juvenile nasopharyngeal angiofibroma: Clinical diagnosis and treatment experience

    Directory of Open Access Journals (Sweden)

    Sladoje Radmila

    2002-01-01

    Full Text Available Juvenile nasopharyngeal angiofibroma is an infrequent epi-pharyngeal tumor necessitating particular diagnostic and therapeutic procedures in comparison to other benign epipharyngeal tumors due to its expansive growth tendency. Our retrospective study is aimed at presenting clinical casuistry of the tumor in order to evaluate modern diagnostic and therapeutic possibilities. The study included 13 male patients, aged 13-24 years, who were hospitalized, diagnostically assessed and surgically treated at the Institute of Otorhinolaryngology and Maxillofacial Surgery, Clinical Centre of Serbia over the period 1990 - June 2001. The following parameters were analyzed: sex, age groups, preoperative symptoms of the disease, diagnostic methods, embolization, local tumor spreading, number and time of tumor relapses and surgical approach.

  2. A national survey of substance abuse treatment for juvenile offenders.

    Science.gov (United States)

    Young, Douglas W; Dembo, Richard; Henderson, Craig E

    2007-04-01

    Despite consensus about the value of substance abuse treatment for delinquent youth, information about its prevalence and availability is inadequate and inconsistent. This article presents findings about treatment and other correctional service provision from a national survey of directors of 141 juvenile institutional and community corrections (CC) facilities. Educational/General Educational Development programming and drug and alcohol education were the most prevalent types of correctional and substance abuse services. Other common services included physical health services and mental health assessment, provided to about 60% of youth across facilities, and mental health counseling, life and communication skills, and anger management, provided to about half of the youth. Substance abuse treatment, as with most other services, were more prevalent in large, state-funded residential facilities (where 66% provided treatment) than in local detention centers (20%) and CC facilities (56%). More detailed data showed that the number of youth attending treatment in all types of facilities on any given day was very low.

  3. 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...

  4. Mineral Oil Aspiration Related Juvenile Idiopathic Arthritis

    OpenAIRE

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

    2015-01-01

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

  5. Retrocalcaneal bursitis in juvenile chronic arthritis.

    OpenAIRE

    Goldenstein-Schainberg, C; Homsi, C; Rodrigues Pereira, R M; W. Cossermelli

    1992-01-01

    Retrocalcaneal bursitis has been described in various adult rheumatic diseases and septic bursitis unrelated to previous bursal disease has been reported in children. The case is reported here of a girl with juvenile chronic arthritis who developed non-septic retrocalcaneal bursitis; the diagnosis was suggested by a combination of clinical and radiographic studies and was confirmed by ultrasonography.

  6. Family Disruption and Delinquency. Juvenile Justice Bulletin.

    Science.gov (United States)

    Thornberry, Terence P.; Smith, Carolyn A.; Rivera, Craig; Huizinga, David; Stouthamer-Loeber, Magda

    At study sites in Rochester (New York), Denver (Colorado) and Pittsburgh (Pennsylvania), three research teams studying the impact of family disruption on juvenile delinquency have interviewed approximately 4,000 participants at regular intervals for a decade, recording their lives in detail. Findings to date indicate that preventing delinquency…

  7. Childhood ovarian juvenile granulosa cell tumour

    African Journals Online (AJOL)

    Prof Ezechukwu

    2012-05-12

    May 12, 2012 ... years old of age. We describe a case ... Juvenile granulosa cell tumour a subtype of ovarian stro- mal cell ... A more serious estrogen effects can occur in various end ... usually behave in a benign manner despite having histo-.

  8. Antineutrophil cytoplasmic antibodies in juvenile chronic arthritis

    NARCIS (Netherlands)

    Mulder, L; Horst, G; Limburg, P; deGraeffMeeder, ER; Kuis, W; Kallenberg, C

    1997-01-01

    Objective, To evaluate the diagnostic significance of antineutrophil cytoplasmic antibodies (ANCA) by assessing the prevalence of ANCA in juvenile chronic arthritis (JCA) (n = 93) of either oligoarticular, polyarticular, or systemic onset. To investigate the prevalence of ANCA in other diseases of c

  9. Biologisk terapi ved juvenil idiopatisk artritis

    DEFF Research Database (Denmark)

    Herlin, Troels

    2008-01-01

    In recent years the treatment of juvenile idiopathic arthritis (JIA) has undergone marked changes. There is substantial evidence that inhibitors of tumor necrosis factor alpha (TNFalpha) like etanercept, infliximab and adalimumab show significant efficacy when standard therapy fails, and long-ter...

  10. Smerte og smertemestring ved juvenil idiopatisk artritis

    DEFF Research Database (Denmark)

    Herlin, Troels; Thastum, Mikael

    2008-01-01

    Pain is one of the primary symptoms of juvenile idiopathic arthritis (JIA). JIA patients have reduced pain tolerance and pain threshold compared to healthy controls. In children with JIA the greater use of coping strategies such as problem-solving, positive self-statements and distraction consist...

  11. Increasing Incidence of Juvenile Thyrotoxicosis in Denmark

    DEFF Research Database (Denmark)

    Kjær, R. H.; Andersen, M. S.; Hansen, D.

    2015-01-01

    . Additional data were collected on children diagnosed with GD in 2008-2012. Results: In total, 237 patients with juvenile thyrotoxicosis (JT) were identified. The overall IR in 1998-2012 was 1.58/100,000 person-years and has increased significantly from 0.79/100,000 person-years in 1982-1988 (p

  12. Predictors of juveniles' noncompliance with probation requirements.

    Science.gov (United States)

    NeMoyer, Amanda; Goldstein, Naomi E S; McKitten, Rhonda L; Prelic, Ana; Ebbecke, Jenna; Foster, Erika; Burkard, Casey

    2014-12-01

    Probation is the most common disposition for adjudicated youth, but little is known about which specific requirements are commonly imposed on juveniles, the requirements with which juveniles most often fail to comply, and how certain youth characteristics and/or imposed requirements might relate to probation noncompliance. An investigation of 120 archived files of youth represented by an urban public defender's office identified 29 probation requirements imposed on youth and 18 requirements with which youth commonly failed to comply. Results revealed that 52% of youth failed to comply with at least one probation requirement; prior probation noncompliance and race were both significantly associated with noncompliance in the examined probation disposition. In addition, the probability of probation noncompliance was significantly higher when youth received either of two substance-related probation requirements: drug tests or drug and alcohol counseling. Such results may prompt further investigation of juvenile probation-related predictors, identify areas of need for clinical service provision to foster successful completion of probation requirements, and help identify areas of potential biases among juvenile court personnel.

  13. Retrocalcaneal bursitis in juvenile chronic arthritis.

    Science.gov (United States)

    Goldenstein-Schainberg, C; Homsi, C; Rodrigues Pereira, R M; Cossermelli, W

    1992-01-01

    Retrocalcaneal bursitis has been described in various adult rheumatic diseases and septic bursitis unrelated to previous bursal disease has been reported in children. The case is reported here of a girl with juvenile chronic arthritis who developed non-septic retrocalcaneal bursitis; the diagnosis was suggested by a combination of clinical and radiographic studies and was confirmed by ultrasonography. Images PMID:1444631

  14. The human microbiome and juvenile idiopathic arthritis

    NARCIS (Netherlands)

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

    2016-01-01

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

  15. Alteracioness cognitivas en familias con Parkinson juvenil

    Directory of Open Access Journals (Sweden)

    Francisco Javier Lopera Restrepo

    2005-03-01

    Full Text Available El Grupo de Neurociencias de la Universidad de Antioquia reportó por primera vez en Colombia cuatro familias afecatas por la Enfermedad de Parkinson Familiar Juvenil portadoras de la mutación G736A en el gen Parkin.

  16. Metamorphosis: How Missouri Rehabilitates Juvenile Offenders

    Science.gov (United States)

    Dubin, Jennifer

    2012-01-01

    Juveniles convicted of serious offenses usually end up in large correctional facilities that focus on punishment--not rehabilitation. The state of Missouri, however, has found a better way to help end the cycle of crime: by creating a network of small facilities that provide therapy and educational opportunities, it has dramatically reduced…

  17. The human microbiome and juvenile idiopathic arthritis

    NARCIS (Netherlands)

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

    2016-01-01

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

  18. Autoimmune hepatitis and juvenile systemic lupus erythematosus

    NARCIS (Netherlands)

    Deen, M. E. J.; Porta, G.; Fiorot, F. J.; Campos, L. M. A.; Sallum, A. M. E.; Silva, C. A. A.

    2009-01-01

    Juvenile systemic lupus erythematosus (JSLE) and autoimmune hepatitis (AIH) are both autoimmune disorders that are rare in children and have a widespread clinical manifestation. A few case reports have shown a JSLE-AIH associated disorder. To our knowledge, this is the first study that simultaneousl

  19. JUVENILE RHEUMATOID ARTHRITIS (TERMINOLOGICALAND CLASSIFICATION ASPECTS

    Directory of Open Access Journals (Sweden)

    N N Kuzmina

    2000-01-01

    Full Text Available Basing on the data of home and foreign literature and on the long-term experience of pediatric rheumatologists, terminologic and classification aspects of Juvenile rheumatoid arthritis (JRA are presented. Approaches to developing of diagnostic and classification of JRA criteria in future are described.

  20. A massive sinonasal psammomatoid variant of juvenile ossifying fibroma: Report of a rare entity

    OpenAIRE

    VR Guttikonda; Taneeru, S; Gaddipati, R; Madala, J

    2013-01-01

    Juvenile ossifying fibroma (JOF) is an uncommon, benign, bone-forming neoplasm with an aggressive local growth that is distinguished from other fibro-osseous lesions primarily by its age of onset, clinical presentation and aggressive behaviour. JOF is considered as a variant of the ossifying fibroma (OF) and the former includes psammomatoid JOF (PsJOF) and Trabecular JOF (TrJOF). Both variants involve the craniofacial bones with the trabecular variant being more common in the jaws and the psa...