Diagnostic scope of computer tomography in hip disease - Paget's disease, femoral head necrosis, coxarthrosis, coxarthritis

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Nebel, G; Lingg, G; Reid, W

1982-10-01

The value of computer tomography was investigated in Paget's disease of the hip, femoral head necrosis (adult avascular and osteoradionecrosis), coxarthrosis and coxarthritis (bacterial and rheumatoid). The greatest value of CT is in the diagnosis of adult avascular necrosis of the femoral head and is valuable for localising the necrotic area in the axial plane. This is of value in planning surgical treatment (displacement osteotomy or endoprosthesis). In Paget's disease, coxarthrosis and coxarthritis, CT provides only additional or more precise information.

Diagnostic scope of computer tomography in hip disease - Paget's disease, femoral head necrosis, coxarthrosis, coxarthritis

International Nuclear Information System (INIS)

Nebel, G.; Lingg, G.; Reid, W.

1982-01-01

The value of computer tomography was investigated in Paget's disease of the hip, femoral head necrosis (adult avascular and osteoradionecrosis), coxarthrosis and coxarthritis (bacterial and rheumatoid). The greatest value of CT is in the diagnosis of adult avascular necrosis of the femoral head and is valuable for localising the necrotic area in the axial plane. This is of value in planning surgical treatment (displacement osteotomy or endoprosthesis). In Paget's disease, coxarthrosis and coxarthritis, CT provides only additional or more precise information. (orig.) [de

Protocol for stage 2 of the GaP study (genetic testing acceptability for Paget's disease of bone: A questionnaire study to investigate whether relatives of people with Paget's disease would accept genetic testing and preventive treatment if they were available

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McCallum Marilyn

2008-05-01

Full Text Available Abstract Background Paget's disease of bone (PDB disrupts normal bone architecture and causes pain, deformity, deafness, osteoarthritis, and fractures. Genetic factors play a role in PDB and genetic tests are now conducted for research purposes. It is thus timely to investigate the potential for a clinical programme of genetic testing and preventative treatment for people who have a family history of PDB. This study examines the beliefs of relatives of people with PDB. It focuses particularly on illness and treatment representations as predictors of the acceptability and uptake of potential clinical programmes. Illness representations are examined using Leventhal's Common Sense Self-Regulation Model while cognitions about treatment behaviours (acceptance of testing and treatment uptake are conceptualised within the Theory of Planned Behaviour. Methods/Design A postal questionnaire of non-affected relatives of people with Paget's disease. The sample will include relatives of Paget's patients with a family history of Paget's disease and relatives of Paget's patients without a family history of Paget's disease. The questionnaire will explore whether a range of factors relate to acceptability of a programme of genetic testing and preventive treatment in relatives of Paget's disease sufferers. The questionnaire will include several measures: illness representations (as measured by the Brief Illness Perceptions Questionnaire; treatment representations (as measured by Theory of Planned Behaviour-based question items, informed by a prior interview elicitation study; descriptive and demographic details; and questions exploring family environment and beliefs of other important people. Data will also be collected from family members who have been diagnosed with Paget's disease to describe the disease presentation and its distribution within a family. Discussion The answers to these measures will inform the feasibility of a programme of genetic testing and

Apocrine hidradenocarcinoma showing Paget's disease and mucinous metaplasia.

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Wahl, Carter E; Todd, Douglas H; Binder, Scott W; Cassarino, David S

2009-05-01

A 54-year-old man presented with a solitary, erythematous, rapidly growing 1-cm nodule on his scalp that had arisen over the previous 3 months. He had no history of skin cancer. An excisional biopsy of the lesion showed a fairly well-circumscribed but focally invasive tumor consisting of areas of typical-appearing clear cell hidradenoma as well as areas with mucinous goblet-type cells and cells with eosinophilic cytoplasm and decapitation-type secretion. There was marked cellular atypia, numerous atypical mitotic figures and focal necrosis. The tumor cells focally involved the overlying epidermis (Paget's disease). Large areas of mucin were identified throughout the lesion. The tumor cells stained with markers for cytokeratin 7 and focally for EMA and CEA, confirming ductal differentiation. The goblet cells and mucinous areas stained with mucicarmine and PASD. The patient was diagnosed with hidradenocarcinoma with mucinous differentiation. Associated Paget's disease has only rarely been reported, and mucinous metaplasia is a previously unreported feature in hidradenocarcinoma.

Cranial Paget's disease - clinical case of symptomatic secondary basilar impression

International Nuclear Information System (INIS)

Gagov, E.; Gabrovsky, N.; Gabrovsky, S.

2010-01-01

A clinical case of 52 years old woman with history of periodic headaches for many years. The headache became more intensive and constant during the last 4-6 months. Instability by walking and stagger occurred as well as weakness in all 4 extremities, difficult swallowing and speech changes. Bulbar, quadripyramidal and archicerebellar symptoms were in hand. Pagets disease was ascertained engaging the skull with secondary basilar impression and compression of the cerebellum and the brain-stem leading to the above described clinical signs. Decompressive median suboccipital craniectomy was performed with laminectomy of C1. Occipital squama was thickened and highly vascularized.. Secondary basilar impression could occur in cranial Pagets disease with clinical symptoms resulting from the compression of the cerebellum and the brain-stem

Sarcomatous Change in Polyostotic Paget's Disease-A Diagnostic ...

African Journals Online (AJOL)

This is a report of a 56-year-old man presented to highlight the clinical features and diagnostic difficulties of Paget's disease in our environment. The patient had a four year history of intractable body pains and at different times had pathological fracture, loss of vision, conductive hearing loss, frequent intermittent anaemia ...

The Paget Trial: A Multicenter, Observational Cohort Intervention Study for the Clinical Efficacy, Safety, and Immunological Response of Topical 5% Imiquimod Cream for Vulvar Paget Disease.

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van der Linden, Michelle; Meeuwis, Kim; van Hees, Colette; van Dorst, Eleonora; Bulten, Johan; Bosse, Tjalling; IntHout, Joanna; Boll, Dorry; Slangen, Brigitte; van Seters, Manon; van Beurden, Marc; van Poelgeest, Mariëtte; de Hullu, Joanne

2017-09-06

Vulvar Paget disease is a rare skin disorder, which is most common in postmenopausal Caucasian women. They usually present with an erythematous plaque that may show fine or typical "cake icing" scaling or ulceration that may cause itching, pain, irritation, or a burning sensation. Although most cases are noninvasive, vulvar Paget disease may be invasive or associated with an underlying vulvar or distant adenocarcinoma. The histological evidence of so-called "Paget cells" with abundant pale cytoplasm in the epithelium confirms the diagnosis. The origin of these Paget cells is still unclear. Treatment of choice is wide local excision with negative margins. Obtaining clear surgical margins is challenging and may lead to extensive and mutilating surgery. Even then, recurrence rates are high, ranging from 15% to 70%, which emphasizes the need for new treatment options. A number of case reports, retrospective case series, and one observational study have shown promising results using the topical immune response modifier imiquimod. This study aims to investigate the efficacy, safety, and immunological response in patients with noninvasive vulvar Paget disease using a standardized treatment schedule with 5% imiquimod cream. Topical 5% imiquimod cream might be an effective and safe treatment alternative for vulvar Paget disease. The Paget Trial is a multicenter observational cohort study including eight tertiary referral hospitals in the Netherlands. It is ethically approved by the Medical-Ethical Committee of Arnhem-Nijmegen and registered in the Central Committee on Research Involving Human Subjects (CCMO) Register by as NL51648.091.14. Twenty patients with (recurrent) noninvasive vulvar Paget disease will be treated with topical 5% imiquimod cream three times a week for 16 weeks. The primary efficacy outcome is the reduction in lesion size at 12 weeks after end of treatment. Secondary outcomes are safety, immunological response, and quality of life. Safety will be

Intraosseous lipomata in Paget's disease: an unusual CT appearance

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Robbie, M.J.; Pratsis, K.K.; Lockie, D.J.

1991-01-01

A case of multiple intraosseous lumbar vertebral lipomata is reported occurring in the prescence of Paget's disease. The pathology and radiology are correlated. An 84 year old man with Paget's disease presented after a cerebrovascular accident which caused him a mild right hemiparesis. Lumbar vertebral lesions were noted incidentally on abdominal radiographs and a CT scan was performed to define the abnormal areas. CT appearance favoured a slow growing non-invasive lesion. However, it failed to show the lower attenuation expected of a fatty lesion such as a lipoma. It is estimated that CT can be diagnostic if it does show fat level attenuation while Magnetic Resonance Imaging can define the lesion in the sagittal and coronal planes as well as provide the signal characteristics of fat (high signal on T1 and T2 weighted images) irrespective of its myxoid ground substance content

[Synthetic human calcitonin in Paget's disease of bone and osteoporosis (author's transl)].

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Nuti, R; Vattimo, A

1981-01-30

Synthetic human calcitonin was used in the treatment of 26 patients over a period of 1-14 months. 17 patients had Paget's disease of the bone, 6 postmenopausal osteoporosis and 3 Sudeck's syndrome. Subjective improvement (reduction of pain, improvement of mobility) was found in 15 patients with Paget's disease, in 4 females with postmenopausal osteoporosis and in all 3 patients with Sudeck's syndrome. Radiographic improvement of bone changes developed only very slowly. These results were confirmed by diminution of the exchangeable calcium pool indicating reduction of rates of osseous degradation. Calcitonin tolerance was acceptable. Transitory nausea and occasional vomiting occurred in 3 patients.

Doença de Paget com acometimento sacral: relato de caso Paget's disease with sacral involvement: a case report

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Fernanda Nogueira Holanda Ferreira Braga

2010-10-01

Full Text Available Os autores relatam o caso de um paciente do sexo masculino, 71 anos de idade, com diagnóstico de doença de Paget óssea sacral. Foi realizado estudo com radiografia, cintilografia, tomografia computadorizada e ressonância magnética, e o diagnóstico foi confirmado por análise histopatológica. O paciente evoluiu com boa resposta ao uso de ibandronato 150 mg, mensalmente, com redução significativa dos marcadores bioquímicos da doença.The authors report a case of a 71-year-old male patient diagnosed with Paget's disease of sacrum. Imaging study was performed with radiography, scintigraphy, computed tomography and magnetic resonance imaging, and the diagnosis was confirmed by biopsy. The patient progressed with a good response to monthly treatment with ibandronate 150 mg, presenting a significant reduction in biochemical markers of disease.

Paget's disease of bone resembling bone metastasis from gastric cancer.

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Shimoyama, Yasuyuki; Kusano, Motoyasu; Shimoda, Yoko; Ishihara, Shingo; Toyomasu, Yoshitaka; Ohno, Tetsuro; Mochiki, Erito; Sano, Takaaki; Hirato, Junko; Mori, Masatomo

2011-08-01

A 74-year-old man had an endoscopic type 0'-IIc tumor in the upper gastric body on the greater curvature and biopsy showed the tumor to be a well-differentiated adenocarcinoma (Group 5). He was referred to us for endoscopic submucosal dissection (ESD). Endoscopy revealed fold convergency, fold swelling, and fusion of the fold, indicating tumor invasion into the submucosa, which was outside the indications for ESD. In addition, there was an increase of serum bone-type alkaline phosphatase (ALP-III and ALP-IV) and urinary cross-linked N-terminal telopeptide of type I collagen (a bone metabolism marker), while (18)F-fluorodeoxyglucose positron emission tomography showed increased uptake in the left pelvis and Th10, suggesting bone metastases. We first diagnosed gastric cancer with bone metastases; however, the symptoms suggested pathological bone fracture and no bone pain. Therefore, a computed tomography-guided aspiration bone biopsy was performed to exclude the possibility of Paget's disease of bone. Biopsy specimens revealed no tumor and a mosaic pattern. No increased uptake of (18)F-FAMT (L-[3-(18)F] α-methyltyrosine) supported a diagnosis of no bone metastases from gastric cancer. We finally diagnosed gastric cancer accompanied by Paget's disease of bone and performed a laparoscopy-assisted proximal gastrectomy. The pathological diagnosis was U less 0-IIb, and U post 0-IIc ypT1a (M) N0H0P0M0 yp stage IA. In gastric cancer patients with suspected bone metastasis, we also need to consider Paget's disease of bone.

Measurement of regional calcium accretion in patients treated for Paget's disease and in paraplegics

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Bergmann, P.; Schoutens, A.; Paternot, J.; Heilporn, E.

1975-01-01

The measurement of regional bone accretion of calcium has proved its worth in the study of two localised bone disorders, Paget's disease and the bone complications of medullar lesions. Intraveinous injection of 47 Ca is followed during 6 days of external measurements with the human whole-body counter and daily determinations of the serum specific activity. Assuming that the decay slope of the extra-osseous activity is parallel to that which follows the serum activity between two and six days, the activity fixed on the skeleton is calculated by zones. The experiment covers 15 normal subjects, 10 patients with Paget's disease, 10 paraplegics and 28 case of miscellaneous ailments [fr

Functional bracing for delayed union of a femur fracture associated with Paget's disease of the bone in an Asian patient: a case report

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Fukuta Masashi

2010-05-01

Full Text Available Abstract Paget's disease of the bone is a common metabolic bone disease in most European countries, Australia, New Zealand, and North America. Conversely, this disease is rare in Scandinavia, Asia, and Africa. In Japan, it is extremely rare, with a prevalence of 0.15/100000. Paget's disease is a localized disorder of bone remodeling. Excessive bone resorption and abnormal bone formation result in biomechanically weakened bone and predispose patients to fracture. Delayed union and non-union of fractures have been reported in patients with Paget's disease. Therefore, open reduction and internal fixation of fractures has been recommended to prevent such complications. Here we report an unusual case of a 63-year-old Asian woman with delayed union of a femur fracture secondary to Paget's disease, which was treated successfully by functional bracing.

Brain metastasis from extramammary Paget's disease of the scrotum.

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Kim, In-Young; Yun, Suk-Jung; Lee, Ji-Shin; Jung, Shin; Jung, Tae-Young; Moon, Kyung-Sub; Jang, Woo-Youl

2014-04-01

We present to our knowledge the first patient with histopathologically proven brain metastasis from extramammary Paget's disease (EMPD) and discuss the effect of brain radiation therapy for this condition. A 68-year-old man presented to our hospital with headache and gait disturbance. Brain MRI showed multiple enhancing mass lesions, and two large cystic lesions in the left cerebellum. The patient had been diagnosed with scrotal Paget's disease 3 months previously but no further management had been performed due to his refusal. The patient underwent stereotactic aspiration and biopsy of the two large cystic lesions. A histopathological examination revealed that the tumor was a metastatic adenocarcinoma. Immunohistochemical staining revealed that the tumor cells were strongly positive for cytokeratin 7 and moderately positive for carcinoembryonic antigen and gross cystic disease fluid protein 15. These findings were similar to those of his scrotal skin lesions and were consistent with metastatic EMPD. The patient underwent brain radiation therapy with a total radiation dose of 30 Gy in 10 fractions. The patient improved neurologically so as to be self-ambulatory, and a mild improvement in the metastatic tumors was found on follow-up MRI. We had planned systemic chemotherapy, but the patient died of acute respiratory failure 2 months after radiation therapy. Copyright © 2013 Elsevier Ltd. All rights reserved.

Paget's disease of the thoracic rib with extension by multifocal contiguity: pathophysiologic paradox clarified by osseous SPECT / CT; Maladie de Paget du rachis dorsal avec extension costale plurifocale par contiguite: un paradoxe physiopathologique elucide par la TEMP/TDM osseuse

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Zwarthoed, C.; Girma, A.; Darcourt, J. [centre Antoine-Lacassagne, Service de medecine nucleaire, 06 - Nice (France); Paycha, F. [Hopital Louis-Mourier, service de medecine nucleaire, 92 - Colombes (France)

2010-07-01

Paget's disease of bone is defined by an acceleration of focused bone remodeling, bone hypertrophy and abnormal bone structure of mono, oligo or poly-ostotic distribution. The evolution of the disease is characterized by a progression of lesions in the bones already partially achieved, without extending bone to bone by contiguity. We report a case of Paget bone disease interesting the dorsal spine and extending in an atypical way to the adjoining ribs. This case illustrates an atypical radio- scintigraphy of Paget's disease with direct extension of the likely damage to bone on bone in the skeletal truncal bridge to know well. (N.C.)

Hypercalcaemia due to immobilization of a patient with Paget's disease of bone.

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Nathan, A. W.; Ludlam, H. A.; Wilson, D. W.; Dandona, P.

1982-01-01

A man with Paget's disease of bone was admitted with a fractured neck of femur. On admission he was normocalcaemic, but with immobilization he rapidly became hypercalcaemic, despite a normal diet and no medication. The hypercalcaemia responded to calcitonin. Although hypercalcaemia is often quoted as complicating the immobilization of such patients, well documented uncomplicated cases have rarely been reported.

[Treatment of Paget's disease with diphosphonate (disodium ethydronate)].

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Caniggia, A; Gennari, C; Guideri, R; Vattimo, A; Nardi, P

1976-01-07

16 patients suffering from Paget's disease were studied before, during and after 3 or 6 month treatment with disodium ethydronate (EHDP) per os. An appreciable improvement in pain symptomatology was noted and at times an evident improvement in audiometry; from the metabolic viewpoint there was a fall in serum alkaline phosphatase and urinary excretion of calcium and hydroxyproline. A study of radiocalcium kinetics demonstrated a reduction in the exchangeable calcium pool and the fractional turnover rate. Histological examination following needle biopsy of the iliac crest showed evident diminution in the active bone cell population (osteoclasts, osteoblasts) and, in certain cases, appearance of osteoid borders.

Treatment of extra-mammary Paget's disease with high energy electron beam

International Nuclear Information System (INIS)

Ogawa, Yasuhiro; Kimura, Syuji; Kumano, Machiko; Tsuboi, Yoshitaka; Gose, Kyuhei.

1979-01-01

10 patients with extra-mammary Paget's disease were treated with high energy electron beam. 3 were treated surgically before irradiation. They were irradiated with the dose from, 6,000 rad in 40 fractions in 53 days to 9,900 rad in 66 fractions in 90 days. 3 recurred within 2 years and 1 recurred after 4.8 years and 1 recurred after 5 years. The early recurrence was observed around the irradiated field, while the late recurrence was seen in the irradiated field. A wider area should be irradiated to control this invasive disease. (author)

Bone scintigraphy and radiology in untreated Paget's disease

International Nuclear Information System (INIS)

Vellenga, C.J.L.R.; Pauwels, E.K.J.; Bijvost, O.L.M.

1985-01-01

This study is based on 107 patients with untreated Paget's disease of bone. Scintigraphy was performed 3 hours after 20 mCi Tc-99m-Sn-EHDP, using a Toshiba 204 or 404 gamma camera. In each patient multiple spot films of the total skeleton were made and evaluated according to a six-point scale. Moreover, in 42 patients the ratio of uptake in a lesion over comparable normal bone was measured using an MDS Trinary computer. Radiographs were made only of affected parts and evaluated according to a three-point scale. In 59 (16%) of the 373 lesions found on the scintigam no radiographic abnormalities were found. These roentgen-negative lesions generally show rather low scintigraphic uptake and are mostly asymptomatic. There exists a clear correlation between scintigraphic uptake and radiological deformity: the scintigraphic scores 5 and 6 occur in only 24% of the ro-grade 1 lesions, but in 65% of the grade 3 lesions; after computer evaluation the mean ratio in grade 1 and 3 lesions is 3.0 and 6.5 resp. (p<0.01). There also appears to be a correlation between scint. uptake and symptomatology: typical Pagetic pain is present in 10% of scint. score 2 and 3 lesions, but in 40% of score 5 and 6 lesions; the mean computer measured scint. ratio is 4.0 for asymptomatic and 7.5 for painful lesions (p<0.001)

A case of Paget from a Northern Italy medieval necropolis.

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Rossetti, Chiara; Pasquinelli, Lidia; Verzeletti, Andrea; Armocida, Giuseppe; Licata, Marta; Fulcheri, Ezio

2018-03-01

An excavation conducted in 2002 by the Lombardy Archaeological Heritage in the St. Giulio Church (Cassano Magnago-Northern Italy) revealed an adult skeleton with an important alteration of the left femur. The femur, longer than the contralateral, exhibited an expansion of the full diaphysis; the surface was coarsened and porous. In cross section, it appeared that almost all the cortical bone had been converted into cancellous bone. Macroscopic and radiological alterations are suggestive of the monostotic form of Paget's disease, a chronic disorder, characterized by focal areas of excessive osteoclastic bone resorption followed by a secondary increase in osteoblastic bone formation. Differential diagnosis is complex for Paget's disease, requiring histological analysis, which revealed a thickened and disorganized trabecular pattern with thick cement lines. Such features, resembling to a mosaic pattern, are typical of Paget's disease. Copyright © 2017 Elsevier Inc. All rights reserved.

Invasive Paget Disease of the Vulva.

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Borghi, Chiara; Bogani, Giorgio; Ditto, Antonino; Martinelli, Fabio; Signorelli, Mauro; Chiappa, Valentina; Scaffa, Cono; Perotto, Stefania; Leone Roberti Maggiore, Umberto; Recalcati, Dario; Lorusso, Domenica; Raspagliesi, Francesco

2018-01-01

Extramammary Paget disease of the vulva (EPDV) is a rare occurrence with an indolent and relapsing course. Progression to invasion occurs in 4% to 19% of cases. The aim of this study is to report clinical-pathological features and outcomes of patients treated for invasive EPDV. Data of consecutive patients treated between 2000 and 2017 for invasive EPDV were reviewed. Among 79 patients with EPDV, 10 (12.7%) presented a microinvasive or invasive form at first diagnosis or during follow-up. All of them underwent upfront radical surgery; 7 (70%) received subsequent radiotherapy, chemotherapy, or both. The mortality rate was 40%. The recurrence rate after treatment for invasive forms was 60%, with a mean time to first recurrence of 20 (range, 5-36) months. Our study confirms that invasive EPDV remains a rare gynecological neoplasm with a poor prognosis. Multicentre trials or well-organized prospective data collection could improve the knowledge about the management of invasive EPDV.

Klinefelter's syndrome associated with breast carcinoma and Paget's disease of the nipple.

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Moshakis, V; Fordyce, M J; Griffiths, J D

1983-09-01

We describe a patient with Klinefelter's syndrome associated with multi-focal breast carcinoma and Paget's disease of the nipple. Reviewing the 16 previously documented cases in the world literature, it is apparent that patients with this syndrome have an increased incidence of breast carcinoma. There is no evidence to date to suggest that such tumour is morphologically or biologically different from breast cancer in females and normal men.

Extramammary Paget disease: review of patients seen in a non-melanoma skin cancer clinic.

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Pang, J; Assaad, D; Breen, D; Fialkov, J; Antonyshyn, O; Balogh, J; Tsao, M; Kamra, J; Czarnota, G; Barnes, E A

2010-10-01

Extramammary Paget disease (EMPD) is a rare skin disease commonly found in the anogenital region. In this study, we aimed to identify EMPD patients seen in the non-melanoma skin cancer clinic at Odette Cancer Centre and to describe the treatments delivered and outcomes achieved. From 2000 to 2009, 14 patients were seen. Initial treatment recommendations included imiquimod and surgical excision, although half the patients required more than one treatment modality, highlighting the difficulty of achieving complete eradication of this disease.

[Effects of the combined calcitonin and sodium etidronate therapy in Paget's disease of bone].

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Nuti, R; Turchetti, V; Righi, G; Vattimo, A

1982-03-03

The therapy of Paget's bone disease is essentially based on the use of calcitonin and diphosphonates: both drugs, if used in large doses for long periods, have shown themselves able to provoke particular side-effects. It was, therefore, decided to study the therapeutic efficacy of combined low-dosage treatment using synthetic salmon calcitonin and sodium-etidronate on a group of patients with Paget's osteodystrophy. A clear evident diminution in plasma alkaline phosphatase, hydroxyprolinuria and whole body retention (WBR) of MDP-Tc99m was observed, demonstrating a reduction of metabolic turnover in the bone. No changes in the bone mass (BMC), evaluated by bone mineral detector, were observed at the end of treatment. With this treatment the plateau effect was shown to be appreciably less than normally occurs when either calcitonin or sodium etidronate are used alone.

Bone scintigraphy and radiology in untreated Paget's disease

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Vellenga, C.J.L.R.; Pauwels, E.K.J.; Bijvost, O.L.M.

1985-05-01

This study is based on 107 patients with untreated Paget's disease of bone. Scintigraphy was performed 3 hours after 20 mCi Tc-99m-Sn-EHDP, using a Toshiba 204 or 404 gamma camera. In each patient multiple spot films of the total skeleton were made and evaluated according to a six-point scale. Moreover, in 42 patients the ratio of uptake in a lesion over comparable normal bone was measured using an MDS Trinary computer. Radiographs were made only of affected parts and evaluated according to a three-point scale. In 59 (16%) of the 373 lesions found on the scintigam no radiographic abnormalities were found. These roentgen-negative lesions generally show rather low scintigraphic uptake and are mostly asymptomatic. There exists a clear correlation between scintigraphic uptake and radiological deformity: the scintigraphic scores 5 and 6 occur in only 24% of the ro-grade 1 lesions, but in 65% of the grade 3 lesions; after computer evaluation the mean ratio in grade 1 and 3 lesions is 3.0 and 6.5 resp. (p<0.01). There also appears to be a correlation between scint. uptake and symptomatology: typical Pagetic pain is present in 10% of scint. score 2 and 3 lesions, but in 40% of score 5 and 6 lesions; the mean computer measured scint. ratio is 4.0 for asymptomatic and 7.5 for painful lesions (p<0.001).

Magnetic resonance imaging for extramammary Paget's disease: radiological and pathological correlations

International Nuclear Information System (INIS)

Akaike, Gensuke; Nozaki, Taiki; Matsusako, Masaki; Saida, Yukihisa; Matsui, Mizuko; Ohtake, Naoyuki; Eto, Hikaru; Suzuki, Koyu

2013-01-01

Extramammary Paget's disease (EMPD) is a rare cutaneous neoplasm that is thought to represent intraepithelial adenocarcinoma developing in an area rich in apocrine glands. Magnetic resonance imaging (MRI) findings for this disease are not well established. We report three cases of pathologically confirmed EMPD in which MRI was performed before surgery. The lesions were widespread in the epidermis and the dermis. Lesions were sharply well enhanced on gadolinium-enhanced T1-weighted imaging and appeared hyperintense on diffusion-weighted imaging in all cases. Areas with enhancement in depth corresponded well with the pathological lesion. In addition, different malignant legions were found on the same images from MRI in two cases, indicating potential associations with other malignancies. We describe the MRI findings and their pathological correlation. MRI could be useful for preoperative evaluation of disease spread and detection of associated malignancies. (orig.)

Effects of carbocalcitonin treatment on PAGET's disease assessed by quantitative bone scintigraphy

International Nuclear Information System (INIS)

Vattimo, A.; Di Maggio, G.; Burroni, L.

1988-01-01

Sixteen patients with PAGET's disease of bone underwent quantitative bone scintigraphy with 99m Tc-MDP before and after a long-term treatment with carbocalcitonin, a synthetic analogue of eel calcitonin. The radionuclide study consisted in the blood pool and bone uptake measurement and in the bone avidity calculation (bone uptake - blood pool ratio). In our patients, a significant decrease in bone uptake and bone avidity was found, whereas the blood pool remained statistically unchanged. This behaviour could be accounted for the shift of the compact bone to a lamellar pattern. (orig.) [de

Archaeological skeletons support a northwest European origin for Paget's disease of bone.

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Mays, Simon

2010-08-01

The strong genetic component in the etiology of Paget's disease of bone (PDB), together with marked geographic variation in its prevalence, with high frequencies in British populations, has led some to suggest that the disease originated in Britain and spread around the world in recent times by the migration and admixture of British populations. This study aims to investigate this hypothesis by studying the world geographic distribution of PDB cases identified in ancient skeletons excavated from archaeological sites. The methodology is a review of PDB cases described in the literature. There were 109 cases that met modern diagnostic criteria. All came from Western Europe, 94% from England. These data support the hypothesis that PDB originated in this geographic region.

Uptake of 18F-DCFPyL in Paget's Disease of Bone, an Important Potential Pitfall in Clinical Interpretation of PSMA PET Studies.

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Rowe, Steven P; Deville, Curtiland; Paller, Channing; Cho, Steve Y; Fishman, Elliot K; Pomper, Martin G; Ross, Ashley E; Gorin, Michael A

2015-12-01

Prostate-specific membrane antigen (PSMA)-targeted PET imaging is an emerging technique for evaluating patients with prostate cancer (PCa) in a variety of clinical contexts. As with any new imaging modality, there are interpretive pitfalls that are beginning to be recognized. In this image report, we describe the findings in a 63-year-old male with biochemically recurrent PCa after radical prostatectomy who was imaged with 18 F-DCFPyL, a small molecule inhibitor of PSMA. Diffuse radiotracer uptake was noted throughout the sacrum, corresponding to imaging findings on contrast-enhanced CT, bone scan, and pelvic MRI consistent with Paget's disease of bone. The uptake of 18 F-DCFPyL in Paget's disease is most likely due to hyperemia and increased radiotracer delivery. In light of the overlap in patients affected by PCa and Paget's, it is important for nuclear medicine physicians and radiologists interpreting PSMA PET/CT scans to be aware of the potential for this diagnostic pitfall. Correlation to findings on conventional imaging such as diagnostic CT and bone scan can help confirm the diagnosis.

Life-threatening hyperkalemia following zoledronic acid infusion for Paget's disease: a case report

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Naysmith Morag

2011-08-01

Full Text Available Abstract Introduction Zoledronic acid is a highly effective treatment in Paget's disease for persistent bone pain and prevention of further progression of the disease. The commonest electrolyte abnormality is hypocalcemia. To the best of our knowledge this is the first case of hyperkalemia secondary to zoledronic acid to be published in the world literature. The commonest arrhythmia related to zoledronic acid is atrial fibrillation. Case presentation We describe the case of an 80-year-old Caucasian man, with a history of ischemic heart disease, who had an in-hospital cardiac arrest related to hyperkalemia. Increasing potassium levels were noted following his first zoledronic acid infusion for symptomatic control of bone pain secondary to Paget's disease. Our patient suffered a cardiac arrest 10 days following the zoledronic acid infusion. Our patient's biochemistry and electrocardiogram output were monitored until his death 26 days after his cardiac arrest. Our patient developed paroxysmal atrial fibrillation in the post-resuscitation period and there was persistent hyperkalemia that required prolonged treatment with calcium resonium. All other possible causes of hyperkalemia were excluded. Conclusion In our patient's case persistent hyperkalemia and life-threatening arrhythmias were associated with use of zoledronic acid. These side effects have not been reported before and the causative mechanism is far from clear as there are no obvious systemic effects of zoledronic acid. The combination of zoledronic acid with predisposing factors such as structural heart disease might account for the clinical picture we witnessed. As a result, electrolyte monitoring should be adopted early in zoledronic acid use. Further studies are required to elucidate the underlying mechanism of hyperkalemia and identify the target group of patients where zoledronic acid can be safely administered. Great caution is advised in patients with underlying heart conditions.

Metachronous Paget's disease of the breast: case report.

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Gubitosi, A; Moccia, G; Malinconico, F A; Iside, G; Gilio, F; Cognetti, C; Foroni, F; Docimo, G; Ruggiero, R; Docimo, L; Agresti, M

2009-04-01

Paget breast disease is a kind of intraductal carcinoma that through an intracanalicular diffusion invades the basal epidermal layer, reaching the areola and nipple, producing a typical erythematous desquamative eczematous-like lesion. This neoplasia can remain undetected for a long time and inadequately treated as a dermatological affection. Synchronous or metachronous lesions are very uncommon. Surgical choice is conditioned by the presence of a tumor below the epidermal lesion, by its dimensions, and by the possible lymph node involvement. Surgical therapy can be radical or conservative. From our experience we think that lesion biopsy is always necessary to formulate a correct diagnosis and to schedule an appropriate therapeutic approach. In our case, a biopsy was performed first, then on the basis of the frozen section analysis a radical mastectomy with axillary third level lymph nodes dissection, because of the large dimensions of the lesion and the previous history of a methachronous lesion.

Prostate-specific antigen-positive extramammary Paget's disease--association with prostate cancer

DEFF Research Database (Denmark)

Hammer, Anne; Hager, Henrik; Steiniche, Torben

2008-01-01

Extramammary Paget's disease (EMPD) is a rare intraepidermal adenocarcinoma that primarily affects the anogenital region. Cases of EMPD reacting with PSA (prostate-specific antigen) have previously been associated with underlying prostate cancer. However, a recent case of EMPD in our department has...... led us to question the value of PSA as an indicator of underlying prostate cancer. Clinical and pathological data were obtained for 16 cases of EMPD. Formalin-fixed, paraffin-embedded tissue blocks from the primary skin lesions were investigated using PSA and other immunohistochemical markers. 5...... of the 16 cases of EMPD stained positive for PSA (2 women and 3 men). However, no reactivity was seen for the prostatic marker P501S. Three of the five patients had been diagnosed with internal malignant disease-two with prostate cancer, stage 1. Immunohistochemical investigations of the tumour specimens...

Multicentric primary extramammary Paget disease: a Toker cell disorder?

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Hashemi, Pantea; Kao, Grace F; Konia, Thomas; Kauffman, Lisa C; Tam, Christine C; Sina, Bahram

2014-07-01

Toker cells are epithelial clear cells found in the areolar and nipple areas of the breast, vulvar region, and other apocrine gland-bearing areas of the skin. Toker cells have been implicated in the pathogenesis of clear cell papulosis, cutaneous hamartoma with pagetoid cells, and rare cases of primary extramammary Paget disease (EMPD) but not in secondary EMPD with underlying adenocarcinoma. The pathogenesis of primary EMPD is not well defined. We report a case of multicentric primary EMPD with evidence of Toker cell proliferation and nonaggressive biologic behavior in a 63-year-old white man. A detailed description of the morphologic and biologic features of Toker cells and their possible carcinogenetic links also are discussed. Based on the observation and follow-up of our patient, we hypothesize that multicentric primary EMPD starts with Toker cell hyperplasia and can potentially evolve to carcinoma in the genital region.

Modern Trends in the Surgical Management of Paget's Disease.

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Wong, Stephanie M; Freedman, Rachel A; Stamell, Emily; Sagara, Yasuaki; Brock, Jane E; Desantis, Stephen D; Golshan, Mehra

2015-10-01

We examined the incidence and modern national trends in the management of Paget's disease (PD), including the use of breast-conserving surgery (BCS), mastectomy, axillary surgery, and receipt of radiotherapy. Using surveillance, epidemiology and end results (SEER) data, we identified 2631 patients diagnosed with PD during 2000-2011. Of these patients, 185 (7%) had PD of the nipple only, 953 (36.2%) had PD with ductal carcinoma in situ (PD-DCIS), and 1493 (56.7%) had PD with invasive ductal carcinoma (PD-IDC). Trends in age-adjusted incidence, primary surgery, sentinel lymph node biopsy (SLNB), and axillary lymph node dissection were examined. Multivariable logistic regression was used to evaluate factors associated with receipt of BCS and radiotherapy. A decrease in the age-adjusted incidence of PD occurred from 2000 to 2011 (-4.3% per year, p modern trends in local therapy suggest that BCS, SLNB, and adjuvant radiotherapy remain underutilized.

Effects of carbocalcitonin treatment on PAGET's disease assessed by quantitative bone scintigraphy

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Vattimo, A.; Di Maggio, G.; Burroni, L.

1988-06-01

Sixteen patients with PAGET's disease of bone underwent quantitative bone scintigraphy with /sup 99m/Tc-MDP before and after a long-term treatment with carbocalcitonin, a synthetic analogue of eel calcitonin. The radionuclide study consisted in the blood pool and bone uptake measurement and in the bone avidity calculation (bone uptake - blood pool ratio). In our patients, a significant decrease in bone uptake and bone avidity was found, whereas the blood pool remained statistically unchanged. This behaviour could be accounted for the shift of the compact bone to a lamellar pattern.

Radiological features of Paget disease of bone associated with VCP myopathy

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Farpour, Farzin [University of California, Department of Radiology, VA Long Beach Health Care, Irvine, CA (United States); Queens Hospital Center, Mount Sinai School of Medicine, New York, NY (United States); Tehranzadeh, Jamshid [University of California, Department of Radiology, VA Long Beach Health Care, Irvine, CA (United States); Donkervoort, Sandra; Vanjara, Pari [University of California, Division of Genetics and Metabolism, Department of Pediatrics, Irvine, CA (United States); Smith, Charles [University of Kentucky, Department of Neurology and Sanders-Brown Center on Aging, Lexington, KY (United States); Martin, Barbara [University of Kentucky, Lexington, KY (United States); Osann, Kathryn [University of California, Department of Medicine, Division of Hematology/Oncology, Irvine, CA (United States); Kimonis, Virginia E. [University of California, Division of Genetics and Metabolism, Department of Pediatrics, Irvine, CA (United States); UC Irvine Medical Center, Division of Genetics and Metabolism, Orange, CA (United States)

2012-03-15

Mutations in the Valosin-containing protein (VCP) gene cause a unique disorder characterized by classic Paget disease of bone (PDB), inclusion body myopathy, and frontotemporal dementia (IBMPFD). Our objective was to analyze the radiographic features of PDB associated with VCP mutations since there is a dearth of literature on the PDB component of VCP disease. Radiographic bone surveys were examined in 23 individuals with VCP mutation and compared with their unaffected relatives. Laboratory testing relevant for VCP disease was performed in all individuals. Of the 17 affected individuals with clinical manifestations of VCP disease, 16 of whom had myopathy, radiographic analysis revealed classic PDB in 11 individuals (65%). The mean age of diagnosis for myopathy was 43.8 years and for PDB was 38.1 years of age. Radiological evidence of PDB was seen in one individual (16%) amongst six clinically asymptomatic VCP mutation carriers. Alkaline phosphatase was a useful marker for diagnosing PDB in VCP disease. Radiographic findings of classic PDB are seen in 52% of individuals carrying VCP mutations at a significantly younger age than conventional PDB. Screening for PDB is warranted in at-risk individuals because of the benefit of early treatment with the new powerful bisphosphonates that hold the potential for prevention of disease. (orig.)

Dual photon absorptiometry in patients with Paget disease (bone mineral contents of lumbar spine and femoral neck)

International Nuclear Information System (INIS)

Talbot, J.N.; Piketty, C.; Kiffel, T.; Coutris, G.; Milhaud, G.

1988-01-01

The bone mineral contents (BMC) of the lumbar spine (84 cases) and of one femoral neck (53 cases) were measured by dual photon absorptiometry (DPA) in patients suffering from Paget's disease of bone. The BMC of the patients and of 53 controls were compared, taking into account the occurrence of a scintigraphic hyperfixation, in each patient. A hyperfixation of the technetium labelled diphosphonate in the lumbar spine, more frequent in males, was associated with an elevated mean BMC value. The difference versus controls was significant in men but not in women. A hyperfixation of the femoral neck was associated with an elevated mean BMC value in both sexes. BMC values greater than the mean BMC of controls + 2 SD were observed in 83 per cent of these male patients and 50 per cent of the females. In contrast, BMC values lower than in controls were observed in non-pagetic areas. DPA allows the quantification of these abnormalities which cannot be evidenced by bone scintigraphy even if alkaline phosphatases levels are assayed [fr

Quality of life and female sexual function after skinning vulvectomy with split-thickness skin graft in women with vulvar intraepithelial neoplasia or vulvar Paget disease.

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Lavoué, V; Lemarrec, A; Bertheuil, N; Henno, S; Mesbah, H; Watier, E; Levêque, J; Morcel, K

2013-12-01

Vulvar intraepithelial neoplasia (VIN) and vulvar Paget disease are managed with either vulvectomy, destructive treatments (laser, antimitotic drugs) or immunostimulants. All these options are associated with functional complications. The purpose of this study was to evaluate the surgical technique consisting of skinning vulvectomy with split-thickness skin graft, and its effect on overall quality of life and sexual function. A retrospective study was conducted on thirteen patients who underwent skinning vulvectomy with split-thickness skin graft between 1999 and 2009. Overall quality of life and sexual function were assessed with the Medical Outcome Study Short Form 36 (MOS SF-36) and Female Sexual Function Index (FSFI), respectively. The median age of patients was 54 (range: 33-77) years. Three patients had Paget disease and 10 patients had VIN lesions. The excision margins were clear in 46% of cases. The incidence of occult cancer was 31%. The mean follow-up period was 77 (±35) months. Four patients experienced a relapse of their intraepithelial disease. The mean disease-free survival was 58 (±44) months. There was no significant difference in MOS SF-36 scores between the study population and the general population. The patients assessed with the FSFI regained normal sexual function after the surgical procedure. Skinning vulvectomy with split-thickness skin graft is a feasible technique yielding good results in terms of quality of life and sexual function. It enables occult cancer to be diagnosed in patients with VIN or Paget disease. Copyright © 2013 Elsevier Ltd. All rights reserved.

Paget-Schroetter Syndrome in 52-Year-Old Male: An Interesting Case Report

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Nik Kosai

2017-04-01

Full Text Available Paget-Schroetter syndrome is a form of upper limb deep venous thrombosis and it is commonly seen in young individuals. Paget-Schroetter syndrome is a rare condition and diagnosis becomes more difficult if it occurs in old individuals. There is no clear consensus regarding the exact treatment of Paget's Schroetter syndrome. A high grade of suspicion with early diagnosis and treatment is needed. We here describe the case of Paget-Schroetter syndrome in a 52-year-old male and discuss the important clinical features and treatment modalities.

Radiological and clinical aspects of M. Paget

International Nuclear Information System (INIS)

Reid, W.

1983-01-01

Deforming bone dystrophy according to Paget is a disease of uncertain etiology and the affected age group is above 40. Earlier manifestations are rare. The most frequent site of the pathologic process is the pelvis. Further locations are the lumbar section of the vertebral column, the sacrum, extremities, skull, and the thoracal and cervical parts of the vertebral column. The sternum, clavides, hands, feet and ribs are rarely affected. There is no predisposition by sex, but more often the right half of the body seems to be attacked. Monostotic and polyostotic incidence are noted about equally often (Scully et al. 1981). As to the overall diagnostical mode of action, the following is suggested: If a bone has been attacked by M. Paget, skeletal scintiscanning of the total body with a bone-detecting tracer has to follow. If further skeletal sections turn out to be conspicuous, they have to be X-rayed. By proceeding in this manner, firstly, the diagnose can be confirmed and, secondly, the initial findings can be documented. Pharmaceutical therapy should be attempted if patients are severely affected and if there are special indications. (orig.) [de

Paget's disease of jaw bones as primary manifestation: A case report of a proper diagnosis made by general dentist.

Science.gov (United States)

Campolongo, Martin G; Cabras, Marco; Bava, Luca; Arduino, Paolo G; Carbone, Mario

2018-06-01

To present a case of early diagnosis mandibular Paget's disease of bone (PDB), recognised by a general dentist. PDB is responsible of rapid bone resorption and disorganised bone formation. The patient was a 72-year-old female patient complaining of dental malposition and blatant prognathism. Clinicians should consider PDB in differential diagnosis for an elderly patient undergoing unexplained alteration in face profile and occlusion. © 2018 John Wiley & Sons A/S and The Gerodontology Association. Published by John Wiley & Sons Ltd.

[The treatment of Paget's disease of bone with second-generation bisphosphonates via intravenous infusion].

Science.gov (United States)

Arboleya, L; Sánchez, J; Iglesias, G; Arranz, J L

1993-12-01

We compared the biochemical effects and safety of pamidronate (30 mg a day for 3 consecutive days) versus clodronate (300 mg a day for 3 consecutive days) via intravenous infusion in 14 patients with Paget's disease of bone (PDB). Both drugs induced a decrease in serum alkaline phosphatase levels as well as the elimination of hydroxyproline from urine, an effect most marked in the group treated with pamidronate. The response was maintained for 6 months after the infusion in the majority of the patients. No relevant side effects were found, except post-infusion febricula and in one patient, self-limiting thrombopenia 6 months after the infusion. We conclude that the intravenous infusion of either of the two drugs may constitute a safe and effective alternative for treatment of PDB with marked biochemical activity or resistant to conventional therapy.

Clinical presentation of juvenile Huntington disease

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Ruocco Heloísa H.

2006-01-01

Full Text Available OBJECTIVE: To describe the clinical presentation a group of patients with juvenile onset of Huntington disease. METHOD: All patients were interviewed following a structured clinical questioner. Patients were genotyped for the trinucleotide cytosine-adenine-guanine (CAG repeat in the Huntington Disease gene. High resolution brain MRI was performed in all patients. RESULTS: We identified 4 patients with juvenile onset of disease among 50 patients with Huntington disease followed prospectively in our Neurogenetics clinic. Age at onset varied from 3 to 13 years, there were 2 boys, and 3 patients had a paternal inheritance of the disease. Expanded Huntington disease allele sizes varied from 41 to 69 trinucleotide repeats. The early onset patients presented with rigidity, bradykinesia, dystonia, dysarthria, seizures and ataxia. MRI showed severe volume loss of caudate and putamen nuclei (p=0.001 and reduced cerebral and cerebellum volumes (p=0.01. CONCLUSION: 8% of Huntington disease patients seen in our clinic had juvenile onset of the disease. They did not present with typical chorea as seen in adult onset Huntington disease. There was a predominance of rigidity and bradykinesia. Two other important clinical features were seizures and ataxia, which related with the imaging findings of early cortical atrophy and cerebellum volume loss.

Computed tomography of Paget disease of the skull versus fibrous dysplasia

International Nuclear Information System (INIS)

Tehranzadeh, J.; Anavim, A.; Pribram, H.W.; Fung Ying; Donohue, M.

1998-01-01

Objective. Radiologists are often challenged to review CT examinations of the skull without pertinent clinical information or plain radiographs. Skull lesions of fibrous dysplasia (FD) may often be confused with Paget disease (PD). The purpose of this article is to evaluate radiographic similarities and to find the signs that can differentiate PD from FD of the skull on head CT and to describe the CT imaging features of PD and FD. Design and patients. CT scans of the skull in eight cases of PD, 18 cases of FD (13 cases of skull and facial bones, five cases of only facial bones) and 10 normals were studied retrospectively. Results. Ten features were found to be similar in PD and FD and 10 other features were found to be dissimilar. The frequency of the 10 differentiating features was evaluated to determine their reliability in distinguishing one disorder from the other. The differentiating features in order of significance include: (1) ''groundglass'' appearance, (2) symmetry, (3) involvement of the paranasal sinuses, (4) thickness of the cranial cortices, (5) involvement of the sphenoid bone, (6) orbital involvement, (7) nasal cavity involvement, (8) presence of a soft tissue mass, (9) maxillary involvement, and (10) the presence of cyst-like changes. Conclusion. These 10 signs improve the radiologist's skill in differentiating FD and PD. (orig.)

Clostridium difficile Colitis and Neutropenic Fever Associated with Docetaxel Chemotherapy in a Patient with Advanced Extramammary Paget's Disease.

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Nonomura, Yumi; Otsuka, Atsushi; Endo, Yuichiro; Fujisawa, Akihiro; Tanioka, Miki; Kabashima, Kenji; Miyachi, Yoshiki

2012-05-01

Extramammary Paget's disease is a rare cutaneous malignant neoplasm. Previous studies indicated the efficacy of docetaxel in advanced cases. The common side effects of docetaxel are usually tolerable and seldom life-threatening. We experienced a case of severe pseudomembranous colitis and neutropenic fever that developed just after the first cycle of docetaxel chemotherapy. To the best of our knowledge, there are few reports of pseudomembranous colitis associated with docetaxel administration for skin cancers. The patient showed complete resolution of her symptoms within 2 weeks with an oral metronidazole therapy. During the second and third cycles, the patient received docetaxel safely with lower doses. The present case indicated that pseudomembranous colitis should be included in the differential diagnosis when assessing patients who develop severe diarrhea during systemic chemotherapy with docetaxel.

Gas bubble disease monitoring and research of juvenile salmonids

International Nuclear Information System (INIS)

Maule, A.G.; Beeman, J.; Hans, K.M.; Mesa, M.G.; Haner, P.; Warren, J.J.

1997-10-01

This document describes the project activities 1996--1997 contract year. This report is composed of three chapters which contain data and analyses of the three main elements of the project: field research to determine the vertical distribution of migrating juvenile salmonids, monitoring of juvenile migrants at dams on the Snake and Columbia rivers, and laboratory experiments to describe the progression of gas bubble disease signs leading to mortality. The major findings described in this report are: A miniature pressure-sensitive radio transmitter was found to be accurate and precise and, after compensation for water temperature, can be used to determine the depth of tagged-fish to within 0.32 m of the true depth (Chapter 1). Preliminary data from very few fish suggest that depth protects migrating juvenile steelhead from total dissolved gas supersaturation (Chapter 1). As in 1995, few fish had any signs of gas bubble disease, but it appeared that prevalence and severity increased as fish migrated downstream and in response to changing gas supersaturation (Chapter 2). It appeared to gas bubble disease was not a threat to migrating juvenile salmonids when total dissolved gas supersaturation was < 120% (Chapter 2). Laboratory studies suggest that external examinations are appropriate for determining the severity of gas bubble disease in juvenile salmonids (Chapter 3). The authors developed a new method for examining gill arches for intravascular bubbles by clamping the ventral aorta to reduce bleeding when arches were removed (Chapter 3). Despite an outbreak of bacterial kidney disease in the experimental fish, the data indicate that gas bubble disease is a progressive trauma that can be monitored (Chapter 3)

Prevalence of Paget's disease of bone in Italy.

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Gennari, Luigi; Di Stefano, Marco; Merlotti, Daniela; Giordano, Nicola; Martini, Giuseppe; Tamone, Cristina; Zatteri, Roberto; De Lucchi, Roberto; Baldi, Carlo; Vattimo, Angelo; Capoccia, Silvia; Burroni, Luca; Geraci, Simone; De Paola, Vincenzo; Calabrò, Anna; Avanzati, Annalisa; Isaia, Giancarlo; Nuti, Ranuccio

2005-10-01

We examined the prevalence of PDB in Italy from radiological, scintigraphic, and biochemical surveys in two Italian towns. Prevalence rates varied from 0.7% to 2.4%, were higher in males than in females, and slightly differed between the two towns. Unlike previous studies in populations of British descent, no secular trend for a decreasing prevalence emerged. Clinical, radiological, and necropsy data from different countries suggested pronounced geographical variations in the prevalence of Paget's disease of bone (PDB). Despite the impact of the disease on the population, there are limited data on the prevalence of PDB in Italy. The objective of this study was to estimate the prevalence of PDB in the district of Siena (Central Italy) and Turin (Northern Italy) from radiological, biochemical, and scintigraphic surveys. We examined a sample of 1778 consecutive pelvic radiographs performed between 1999 and 2000 at the Hospital Radiology Unit in Siena and 6609 pelvic radiographs performed in 1986-1987, 1992-1993, and 1999-2002 from the Radiology Department of Molinette Hospital in Turin. In Siena, 7906 consecutive (99m)TC-MDP bone scans performed over a 4-year period (January 2000 to May 2004) were also screened for the presence of PDB, and the prevalence of elevated alkaline phosphatase (ALP) levels (>300 UI/liter) was estimated from 7449 computerized medical records over a 3-year period (January 2000 to February 2003). The finding of PDB on the pelvic radiograph and bone scan was based on standardized radiological criteria. At the end of the radiological surveys, 16/1778 pelvic PDB cases (8 males and 8 females) were observed in Siena and 41/6609 (27 males and 14 females) in Turin. The crude prevalence of the disease was 0.89% in Siena and 0.62% in Turin. Given that pelvic involvement is commonly described in 60-90% of PDB patients, the estimated overall prevalence of PDB ranged from 1.0% to 1.5% in Siena and from 0.7% to 1.0% in Turin. No decrease in the prevalence

The Effectiveness of Mapping Biopsy in Patients with Extramammary Paget's Disease

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Byung Jun Kim

2014-11-01

Full Text Available BackgroundExtramammary Paget's disease (EMPD is an intraepithelial carcinoma usually occurring on the skin or mucosa of the perineum. Clinically, it resembles eczema or dermatitis, and misdiagnosis and treatment delays are common. The treatment of choice for EMPD is a wide excision with adequate margins. Wide excision with intraoperative frozen biopsy and Mohs micrographic surgery are common methods; however, these are associated with a high recurrence rate and long operation time, respectively.MethodsBetween January 2010 and June 2013, 21 patients diagnosed with EMPD underwent mapping biopsy. Biopsy specimens were collected from at least 10 areas, 2 cm from the tumor margin. When the specimens were positive for malignancy, additional mapping biopsy was performed around the biopsy site of the positive result, and continued until no cancer cells were found. Based on the results, excision margins and reconstruction plans were established preoperatively.ResultsThe patients (18 male, 3 female had a mean age of 66.5 years (range, 50-82 years. Almost all cases involved in the perineal area, except one case of axillary involvement. Permanent biopsy revealed one case (4.8% of positive cancer cells on the resection margin, in which additional mapping biopsy and re-operation was performed. At the latest follow-up (mean, 27.4 months; range, 12-53 months, recurrence had not occurred.ConclusionsPreoperative mapping biopsy enables accurate resection margins and a preoperative reconstructing plan. Additionally, it reduces the operation time and risk of recurrence. Accordingly, it represents an effective alternative to Mohs micrographic surgery and wide excision with intraoperative frozen biopsy.

Juvenile Gaucher disease simulating osteomyelitis

International Nuclear Information System (INIS)

Miller, J.H.; Ortega, J.A.; Heisel, M.A.

1981-01-01

A case in which several imaging procedures suggested juvenile Gaucher disease in a child who presented with symptomatology of osteomyelitis is discussed. The 20-month girl was given a Technetium-99m radionuclide skeletal examination which revealed intense uptake of tracer agents along the shaft of the right femur. It was also found that the liver and spleen were dramatically Ga-67 avid. The bone pain symptomatology suggested an osteomyelitis of the femur, but skeletal scintigraphy with Tc-99m-labeled bone tracer demonstrated photopenic areas involving the femur, suggesting that the bone pain may have been due to marrow packed with Gaucher cells. This overexpansion of the marrow may lead to microfractures with remodeling seen radiographically as periosteal new bone and scintigraphically as increased periosteal deposition of tracer agent. The radiogallium study was useful to exclude an underlying osteomyelitis in the involved femurs. Although juvenile Gaucher disease is unusual, it should be considered in any child who presents with the constellation of hepatosplenomegaly and bone pain simulating osteomyelitis

Juvenile Gaucher disease simulating osteomyelitis

Energy Technology Data Exchange (ETDEWEB)

Miller, J.H.; Ortega, J.A.; Heisel, M.A.

1981-10-01

A case in which several imaging procedures suggested juvenile Gaucher disease in a child who presented with symptomatology of osteomyelitis is discussed. The 20-month girl was given a Technetium-99m radionuclide skeletal examination which revealed intense uptake of tracer agents along the shaft of the right femur. It was also found that the liver and spleen were dramatically Ga-67 avid. The bone pain symptomatology suggested an osteomyelitis of the femur, but skeletal scintigraphy with Tc-99m-labeled bone tracer demonstrated photopenic areas involving the femur, suggesting that the bone pain may have been due to marrow packed with Gaucher cells. This overexpansion of the marrow may lead to microfractures with remodeling seen radiographically as periosteal new bone and scintigraphically as increased periosteal deposition of tracer agent. The radiogallium study was useful to exclude an underlying osteomyelitis in the involved femurs. Although juvenile Gaucher disease is unusual, it should be considered in any child who presents with the constellation of hepatosplenomegaly and bone pain simulating osteomyelitis.

TREATMENT FOR GASTROESOPHAGEAL REFLUX DISEASE AMONG CHILDREN, SUFFERING FROM JUVENILE ARTHRITIS

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T.M. Bzarova

2007-01-01

Full Text Available The article presents the evaluation results of esomeprazol efficacy in the complex therapy for gastroesophageal reflux disease among 152 children aged between 3 and 18, suffering from juvenile arthritis. The treatment scheme used in treatment induced the remission of gastro esophageal reflux disease among 45% of patients, conduced to considerable decrease of the esophagus affect intensity among 53% of patients, epithelized erosions of the mucous coat of esophagus among 30 of 32 children. The medication did not cause any clinically significant side responses among the cured children (even younger ones and may be applied to treat gastro esophageal reflux disease among the patients, suffering from juvenile arthritis.Key words: gastroesophageal reflux disease, children, treatment, reflux, esomeprazol, esophagitis, juvenile rheumatoid arthritis.

Invasive extramammary Paget's disease and the risk for secondary tumours in Europe.

Science.gov (United States)

van der Zwan, J M; Siesling, S; Blokx, W A M; Pierie, J P E N; Capocaccia, R

2012-03-01

The aim of this study was to determine the incidence and survival of Extramammary Paget's disease (EMPD) and to describe the possible increased risk of tumours after EMPD. All invasive cases diagnosed between 1990 and 2002 were selected from the RARECARE database. Incidence was expressed in European standardized rates. Relative survival was calculated for the period 1995-1999, with a follow-up until 31st December 2003. Standardized incidence ratios of second primary tumours were calculated to reveal possible increased risk after EMPD. European age standardized Incidence of EMPD within Europe is 0.6 per 1000,000 person years. Five-year relative survival for invasive EMPD was 91.2% (95%CI; 83.5-95.4), 8.6 percent of the EMPD patients developed other malignancies. The highest increased risk of developing a second primary tumour was found in the first year of follow-up (SIR:2.0 95%CI; 1.3-2.9), living in the South European region (SIR:2.3 95%CI; 1.5-3.5) or being female (SIR:1.5 95%CI; 1.1-1.9). Female genital organs displayed greatest increased risk of developing a second primary tumour after EMPD (SIR:15,1 95%CI; 0.38-84.23). Due to the increased risk of a second primary tumour after EMPD a thorough search for other tumours during their follow-up is recommended. Copyright © 2012 Elsevier Ltd. All rights reserved.

Development of a molecular test of Paget's disease of bone.

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Guay-Bélanger, Sabrina; Simonyan, David; Bureau, Alexandre; Gagnon, Edith; Albert, Caroline; Morissette, Jean; Siris, Ethel S; Orcel, Philippe; Brown, Jacques P; Michou, Laëtitia

2016-03-01

Depending on populations, 15 to 40% of patients have a familial form of Paget's disease of bone (PDB), which is transmitted in an autosomal-dominant mode of inheritance with incomplete penetrance. To date, only SQSTM1 gene mutations have been linked to the disease. Several single nucleotide polymorphisms (SNPs) have been associated with PDB in patient non-carriers of SQSTM1 mutations, but they have minor size effects. The current clinical practice guidelines still recommend to measure total serum alkaline phosphatase (sALP) for PDB screening. However, genetic or bone biomarkers alone may lack sensitivity to detect PDB. Thus, the objective of this study was to develop a molecular test of PDB, combining genetic and bone biomarkers, in order to detect PDB, which is frequently asymptomatic. We genotyped 35 SNPs previously associated with PDB in 305 patients, and 292 healthy controls. In addition, serum levels of 14 bone biomarkers were assayed in 51 patients and 151 healthy controls. Bivariate and multivariate logistic regression models with adjustment for age and sex were fitted to search for a combination of SNPs and/or bone biomarkers that could best detect PDB in patient non-carriers of SQSTM1 mutations. First, a combination of five genetic markers gave rise to the highest area under the ROC curve (AUC) with 95% confidence interval [95% CI] of 0.731 [0.688; 0.773], which allowed us to detect 81.5% of patients with PDB. Second, a combination of two bone biomarkers had an AUC of 0.822 [0.726; 0.918], and was present in 81.5% of patients with PDB. Then, the combination of the five genetic markers and the two bone biomarkers increased the AUC up to 0.892 [0.833; 0.951], and detected 88.5% of patients with PDB. These results suggested that an algorithm integrating first a screen for SQSTM1 gene mutations, followed by either a genetic markers combination or a combined genetic and biochemical markers test in patients non-carrier of any SQSTM1 mutation, may detect the PDB

Extramammary Paget's disease: extended subclinical growth detected using three-dimensional histology in routine paraffin procedure and course of the disease.

Science.gov (United States)

Boehringer, Alexandra; Leiter, Ulrike; Metzler, Gisela; Moehrle, Matthias; Garbe, Claus; Breuninger, Helmut

2011-10-01

Extramammary Paget's disease (EMPD) is a rare tumor of the skin characterized by extended irregular subclinical growth and high recurrence rates after surgery. To show that, using three-dimensional (3D) histology in surgical excision, the size of the safety margin can be individually determined and locally adjusted to subclinical growth and to evaluate the course of the disease in 33 patients. The clinical records of 33 patients with primary lesions of EMPD were prospectively documented, retrospectively confirmed, and analyzed. EMPD was found more frequently in women (54.5%). Dermal invasion was found in 21.2%, and regional lymph node metastases were present in one patient; 30.3% of the patients had secondary internal malignancies. In 25 cases examined using 3D histology, a mean surgical margin of 22 mm in asymmetric shape (range 5-65 mm) was achieved, with a recurrence rate of 28%. Surgery with conventional histologic examination had a higher recurrence rate of 62.5% using a mean surgical margin of 19 mm (range 7-40 mm). Subclinical spread of EMPD demonstrated using 3D histology with paraffin sectioning can be large. Three-dimensional histology allows individually defined safety margins to be locally adjusted to subclinical growth and gives low recurrence rates. The authors have indicated no significant interest with commercial supporters. © 2011 by the American Society for Dermatologic Surgery, Inc.

Clinical and genetic study of a juvenile-onset Huntington disease

Directory of Open Access Journals (Sweden)

HAO Ying

2012-06-01

Full Text Available Background Huntington's disease (HD is an autosomal dominant hereditary progressive neurodegenerative disorder with a distinct phenotype characterized by chorea, dementia, cognitive and affective impairment. There are selective neural cell loss and atrophy in the caudate and putamen. Dr. George Huntington firstly described the disease accurately and insightfully, which led to a widespread recognition of the inherited chorea that now bears his name. Huntington disease gene (IT15 locus on chromosome 4p16.3, and encompasses 67 exons with a trinucleotide repeat (CAG in the first exon. The CAG repeat length is highly polymorphic in the population and expanded on at least one chromosome of individuals with HD. Clinically, patient with HD are often onset in adulthood. Juvenile-onset HD is relatively rare. Adult-onset HD patients usually have a CAG expansion from 40 to 55 whereas those with juvenile-onset greater than 60 which are often inherited from the father. We investigated the clinical features of a juvenile-onset case with Huntington disease and dynamic mutation of his family. Methods The CAG repeats of IT15 gene were detected using polymerase chain reaction and capillary electrophoresis in 115 individuals with preliminary diagnosis as Huntington disease. The repeat numbers of some samples carried expanded or intermediate alleles were verified by the pMD18-T vector clone sequencing. Results Fragment analysis showed that one juvenile-onset case presenting with cognitive dysfunction and hypokinesis carried 15/68 CAG repeats of IT15. His father carried 17/37 and mother carried 15/17. Conclusion 1 The juvenile-onset case of HD presented with different clinical features compared with adult-onset cases. The typical signs of adult-onset cases include progressive chorea, rigidity and dementia. The most common sign of juvenile-onset Huntington disease is cognitive decline. 2 The dynamic mutation of IT15 gene expansion of the CAG repeats in the

Epidemiological study of Paget's disease of bone in a zone of the Province of Salamanca (spain)

International Nuclear Information System (INIS)

Mironon-Canelo, J.A.; Del Pino-Montes, J.; Vicente-Arroyo, M.; Saenz-Gonzalez, M.C.

1997-01-01

Bone Paget's disease is heterogeneously distributed and several foci of high prevalence have been reported in Spain. The aim of the present work was to determine the prevalence of the disease in a zone situated in the northwestern sector of the Province of Salamanca (Spain) using a cross sectional epidemiological study. Sample choice was based on a stratified sampling according to the residence, age and sex of the inhabitants of the zone. A sampling error of 5% and a confidence level of 95% were considered; these afforded a sample of 378 units. Final choice of the subjects was based on a random pathway method. Data collection was accomplished with a personal interview using a 31-item questionnaire and analytical screening (AP and GGT). The field work was carried out over a one-year period. The data were input onto a calculation sheet for analysis and epidemiological interpretation. Finally, clinico-radiological confirmation of the cases deemed positive in the screening was accomplished. The prevalence of PBD in the zone studied is 5.7% (95% CI: 4.5-6.9). The highest percentage of patients lies within the age group between 70-79 years; most of these patients were women. The mean residence time in the zone was 66 years. According to the findings, this geographic zone has a high prevalence of PBD

Nailfold capillaroscopy in juvenile rheumatic diseases: known measures, patterns and indications.

Science.gov (United States)

Gerhold, K; Becker, M O

2014-01-01

Nailfold capillaroscopy has become an established method in adults for the evaluation of structural abnormalities of the microcirculation associated with rheumatic disease. It is a cornerstone for the diagnostic work-up of patients with Raynaud's phenomenon and the early diagnosis of systemic sclerosis. However, this non-invasive examination may also be valuable in children and adolescents with rheumatic diseases. Based on the scarce data available, this review focuses on capillaroscopic findings in healthy children and adolescents as well as in children with juvenile systemic sclerosis, juvenile dermatomyositis, juvenile idiopathic arthritis, and Raynaud's phenomenon. In addition, it outlines the potential benefits and limitations of nailfold capillaroscopy for routine care in paediatric rheumatology.

Prognosis and management of extramammary Paget's disease and the association with secondary malignancies.

Science.gov (United States)

Pierie, Jean Pierre E N; Choudry, Umar; Muzikansky, Alona; Finkelstein, Dianne M; Ott, Mark J

2003-01-01

Extramammary Paget's disease (EMPD) is a rare clinical entity and can be associated with other malignancies. We analyzed our experience for prognosis and impact of therapy on outcomes. We conducted a retrospective review of all patients (n = 33) with EMPD treated at a tertiary care center from 1971 to 1998. Pathologic features of EMPD, concurrent secondary malignancies, and the effect of operations on recurrence were analyzed. Overall survival was compared with that of the general population. Male-to-female ratio was 4:29, and median age was 70 years. Median followup was 68 months, and no patient died from EMPD. The lesion was predominantly found on the vulva (76%). Patch-like, nonconfluent growth was present in 45% of patients, and no patient had pathologic lymph nodes. The most common signs and symptoms were irritation or pruritus (73%) and rash (61%). The presence of patches, invasive tumor growth, or a second malignancy were significantly associated with a higher recurrence rate. The type of operation, either local excision or hemivulvectomy, was not related to the time to recurrence. Complete gross resection was achieved in 94% of cases. Fifty-six percent of patients had microscopically positive margin and this correlated with a significantly higher recurrence rate (p = 0.002). The tumor recurred clinically in 14 of 33 patients (42%) after a median of 152 months (range 5 to 209 months). In those patients, between one and six reexcisions were performed. In 14 of 33 patients with EMPD (42%), 16 concurrent secondary malignancies were found. Overall survival rates for EMPD patients were similar to those of the general population. EMPD is an infrequently diagnosed disease that is preferably managed with complete local excision and reexcisions if needed. A thorough search for frequently occurring secondary malignancies might be beneficial to provide the best outcomes for these patients. Copyright 2003 by the American College of Surgeons

Biological therapy and development of neoplastic disease in patients with juvenile rheumatic disease: a systematic review

Directory of Open Access Journals (Sweden)

Vanessa Patricia L. Pereira

Full Text Available Abstract Juvenile rheumatic diseases affect the musculoskeletal system and begin before the age of 18. These conditions have varied, identifiable or unknown etiologies, but those of an autoimmune inflammatory nature have been associated with an increased risk of development of cancer, regardless of treatment. This study aims to assess, through a systematic review of the literature according to Prisma (Preferred Reporting Items for Systematic Reviews and Meta-Analyses quality criteria, the risk of cancer in patients with juvenile rheumatic disease, and its association with biological agents. The criteria described by the Strengthening the Reporting of Observational Studies in Epidemiology initiative were used in order to assess the methodological quality of those individual items selected in this study. We analyzed nine publications, from a total of 251 papers initially selected. There was an increase in cancer risk in the population with juvenile rheumatic disease versus the general population. Most specified cancers were of a lymphoproliferative nature. Seven studies did not specify the treatment or not defined an association between treatment and cancer risk. Only one study has suggested this association; in it, their authors observed high risk in patients diagnosed in the last 20 years, a period of the advent of new therapies. One study found an increased risk in a population not treated with biological agents, suggesting a disease in its natural course, and not an adverse effect of therapy. Studies have shown an increased risk of malignancy associated with juvenile rheumatic disease, and this may be related to disease activity and not specifically to the treatment with biological agents.

Intravenous injection of Ra-224 as a treatment in Morbus Paget

International Nuclear Information System (INIS)

Koch, W.

1976-01-01

Between 1956 and 1973, 21 patients with histologically verified Morbus Paget were treated at the author's hospital with low dosage 224 Ra. In none of these patients an immediate or late impairment of the hematogenic or parenchymal organs was observed. (MG) [de

Assessment of disease activity in juvenile idiopathic arthritis. The number and the size of joints matter

DEFF Research Database (Denmark)

Berntson, Lillemor; Wernroth, Lisa; Fasth, Anders

2007-01-01

Variables for assessment of disease activity of juvenile idiopathic arthritis (JIA) were studied, in order to develop a disease activity score for children with JIA.......Variables for assessment of disease activity of juvenile idiopathic arthritis (JIA) were studied, in order to develop a disease activity score for children with JIA....

Podoplanin expression in peritumoral keratinocytes predicts aggressive behavior in extramammary Paget's disease.

Science.gov (United States)

Cho, Zaigen; Konishi, Eiichi; Kanemaru, Mai; Isohisa, Taro; Arita, Takahiro; Kawai, Minako; Tsutsumi, Miho; Mizutani, Hiromi; Takenaka, Hideya; Ozawa, Toshiyuki; Tsuruta, Daisuke; Katoh, Norito; Asai, Jun

2017-07-01

Recent studies have demonstrated podoplanin expression in several tumors, which has been associated with lymph node metastasis and poor prognosis. Podoplanin expression in peritumoral cells such as cancer-associated fibroblasts also correlates with tumor progression in several cancers. However, podoplanin expression and its association with extramammary Paget's disease (EMPD) remain unclear. In this study, we examined whether the presence of podoplanin expression in tumor cells or peritumoral basal keratinocytes correlated with aggressive behavior in patients with EMPD and investigated the mechanisms of podoplanin-mediated tumor invasion in this disorder. Skin samples of 37 patients with EMPD were investigated by immunohistochemical analysis. The functions of podoplanin in keratinocytes were examined in vitro by RT-PCR and with invadopodia gelatin-degradation assays using HaCaT cells. Podoplanin was not identified in tumor cells in all cases. Podoplanin expression in peritumoral basal keratinocytes was observed in 25 patients (67.6%). In in situ EMPD, 50% of cases (9 in 18) exhibited podoplanin-positive keratinocytes, whereas 84.2% (16 in 19) demonstrated positive staining in invasive EMPD (P<0.05). Podoplanin expression in peritumoral keratinocytes was also associated with tumor thickness (P<0.005). By immunohistochemical analysis, podoplanin-positive peritumoral keratinocytes were found to be negative for E-cadherin, one of the major adhesion molecules of keratinocytes, which might contribute to tumor invasion into the dermis through a crack in the basal cell layer induced by down-regulation of cell adhesion therein. We further found that podoplanin-positive keratinocytes exhibited invadopodia, which are thought to function in the migration of cancer cells through tissue barriers, indicating that podoplanin-positive peritumoral basal keratinocytes might assist tumor invasion by degrading the extracellular matrix. The presence of podoplanin expression in

Juvenile rheumatoid arthritis

Science.gov (United States)

... joints. This form of JIA may turn into rheumatoid arthritis. It may involve 5 or more large and ... no known prevention for JIA. Alternative Names Juvenile rheumatoid arthritis (JRA); Juvenile chronic polyarthritis; Still disease; Juvenile spondyloarthritis ...

Juvenile Idiopathic Arthritis

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Kenan Barut

2017-04-01

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

Treatment of Cushing's disease in juveniles with intestinal pituitary irradiation

International Nuclear Information System (INIS)

Cassar, J.; Doyle, F.H.; Mashiter, K.; Joplin, G.F.

1979-01-01

Nine juvenile patients (five boys and four girls aged 10-18) with Cushing's disease were treated with pituitary implantation of 198 Au and/or 90 Y. No patient had any surgical complication from this procedure. At the latest assessment. 3 months to 17 years after operation, Cushing's disease was in remission in all the patients; the response time following operation was a few days to 3 months. Radiology of the pituitary fossa at time of pituitary implantation was normal in all patients and remains so. The final height in six patients is 149-172 cm (59-67.5 inches) and three patients who continue to grow have increased by 13.6 and 3 cm since implantation. Only one patient required full pituitary hormone replacement therapy, and he had been previously treated by external irradiation, and one other patient failed to complete puberty. In all the other seven sexual maturation is normal and one has fathered two children. It is concluded that pituitary implantation with interstitial irradiation is a satisfactory form of treatment for Cushing's disease in juveniles. (author)

Heart Disease, Hypertension, Gestational Diabetes Mellitus, and Preeclampsia/Eclampsia in Mothers With Juvenile Arthritis: A Nested Case-Control Study.

Science.gov (United States)

Feldman, Debbie E; Vinet, Évelyne; Bérard, Anick; Duffy, Ciarán; Hazel, Beth; Meshefedjian, Garbis; Sylvestre, Marie-Pierre; Bernatsky, Sasha

2017-02-01

To determine whether women with a history of juvenile arthritis are at higher risk for heart disease and hypertension and for developing adverse maternal outcomes: gestational diabetes mellitus, maternal hypertension, and preeclampsia/eclampsia. We designed a nested case-control study from a cohort of first-time mothers with prior physician billing codes suggesting juvenile arthritis, and a matched comparison group without juvenile arthritis. For the nested case-control design, we selected 3 controls for each case for the outcomes of heart disease (n = 403), prepregnancy hypertension (n = 66), gestational diabetes mellitus (n = 285), maternal hypertension (n = 561), and preeclampsia/eclampsia (n = 236). We used conditional logistic regression, adjusting for maternal age and education. Having juvenile arthritis was associated with heart disease (odds ratio [OR] 2.44 [95% confidence interval (95% CI) 1.15-5.15]) but not with gestational hypertension, diabetes mellitus, or preeclampsia/eclampsia. All 66 cases of prepregnancy hypertension had juvenile arthritis. Having prepregnancy hypertension was strongly associated with preeclampsia/eclampsia (OR 8.05 [95% CI 2.69-24.07]). Women with a history of juvenile arthritis had a higher risk of heart disease. This risk signals the potential importance of cardiac prevention strategies in juvenile arthritis. As this was a retrospective study, it was not possible to correct for some relevant potential confounders. Further studies should assess the impact of medications, disease severity, and other factors (e.g., obesity) on cardiac outcomes in juvenile arthritis. © 2016, American College of Rheumatology.

Analysis of Published Criteria for Clinically Inactive Disease in a Large Juvenile Dermatomyositis Cohort Shows That Skin Disease Is Underestimated

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Almeida, Beverley; Campanilho‐Marques, Raquel; Arnold, Katie; Pilkington, Clarissa A.; Wedderburn, Lucy R.; Armon, Kate; Briggs, Vanja; Ellis‐Gage, Joe; Roper, Holly; Watts, Joanna; Baildam, Eileen; Hanna, Louise; Lloyd, Olivia; McCann, Liza; Roberts, Ian; McGovern, Ann; Riley, Phil; Al‐Abadi, Eslam; Ryder, Clive; Scott, Janis; Southwood, Taunton; Thomas, Beverley; Amin, Tania; Burton, Deborah; Jackson, Gillian; Van Rooyen, Vanessa; Wood, Mark; Wyatt, Sue; Browne, Michael; Davidson, Joyce; Ferguson, Sue; Gardner‐Medwin, Janet; Martin, Neil; Waxman, Liz; Foster, Helen; Friswell, Mark; Jandial, Sharmila; Qiao, Lisa; Sen, Ethan; Smith, Eve; Stevenson, Vicky; Swift, Alison; Wade, Debbie; Watson, Stuart; Crate, Lindsay; Frost, Anna; Jordan, Mary; Mosley, Ellen; Satyapal, Rangaraj; Stretton, Elizabeth; Venning, Helen; Warrier, Kishore; Almeida, Beverley; Arnold, Katie; Beard, Laura; Brown, Virginia; Campanilho‐Marques, Raquel; Enayat, Elli; Glackin, Yvonne; Halkon, Elizabeth; Hasson, Nathan; Juggins, Audrey; Kassoumeri, Laura; Lunt, Sian; Maillard, Sue; Nistala, Kiran; Pilkington, Clarissa; Simou, Stephanie; Smith, Sally; Varsani, Hemlata; Wedderburn, Lucy; Murray, Kevin; Ioannou, John; Suffield, Linda; Al‐Obaidi, Muthana; Leach, Sam; Lee, Helen; Smith, Helen; Inness, Emma; Kendall, Eunice; Mayers, David; Wilkinson, Nick; Clinch, Jacqui; Pluess‐Hall, Helen

2015-01-01

Objective The Pediatric Rheumatology International Trials Organisation (PRINTO) recently published criteria for classification of patients with juvenile dermatomyositis (DM) as having clinically inactive disease. The criteria require that at least 3 of 4 conditions be met, i.e., creatine kinase level ≤150 units/liter, Childhood Myositis Assessment Scale score ≥48, Manual Muscle Testing in 8 muscles score ≥78, and physician's global assessment of overall disease activity (PGA) ≤0.2. The present study was undertaken to test these criteria in a UK cohort of patients with juvenile DM. Methods We assessed 1,114 patient visits for the 4 items in the PRINTO criteria for clinically inactive disease. Each visit was analyzed to determine whether skin disease was present. The Disease Activity Score (DAS) for juvenile DM was determined in 59 patients. Results At 307 of the 1,114 visits, clinically inactive disease was achieved based on the 3 muscle criteria (but with a PGA of >0.2); rash was present at 65.8% of these visits and nailfold capillary abnormalities at 35.2%. When PGA ≤0.2 was one of the 3 criteria that were met, the frequency of skin signs was significantly lower (rash in 23.1% and nailfold capillary abnormalities in 8.7%). If PGA was considered an essential criterion for clinically inactive disease (P‐CID), patients with active skin disease were less likely to be categorized as having clinically inactive disease (a median DAS skin score of 0 [of a possible maximum of 9] in visits where the PGA was ≤0.2, versus a median DAS skin score of 4 in patients meeting the 3 muscle criteria [with a PGA of >0.2]; P < 0.001). Use of the P‐CID led to improvements in the positive predictive value and the positive likelihood ratio (85.4% and 11.0, respectively, compared to 72.9% and 5.1 with the current criteria). Conclusion There was a high frequency of skin disease among patients with juvenile DM who did not meet the PGA criterion for inactive disease but met

Persistent association of nailfold capillaroscopy changes and skin involvement over thirty-six months with duration of untreated disease in patients with juvenile dermatomyositis.

Science.gov (United States)

Christen-Zaech, Stéphanie; Seshadri, Roopa; Sundberg, Joyce; Paller, Amy S; Pachman, Lauren M

2008-02-01

To determine the association of changes on nailfold capillaroscopy with clinical findings and genotype in children with juvenile dermatomyositis (DM), in order to identify potential differences in disease course over 36 months. At diagnosis of juvenile DM in 61 children prior to the initiation of treatment, tumor necrosis factor alpha (TNFalpha) -308 allele and DQA1*0501 status was determined, juvenile DM Disease Activity Scores (DAS) were obtained, and nailfold capillaroscopy was performed. The disease course was monitored for 36 months. Variations within and between patients were assessed by regression analysis. At diagnosis, shorter duration of untreated disease (P = 0.05) and a lower juvenile DM skin DAS (P = 0.035) were associated with a unicyclic disease course. Over 36 months, end-row loop (ERL) regeneration was associated with lower skin DAS (P nailfold capillaroscopy changes. The correlation of nailfold capillaroscopy results with cutaneous but not with musculoskeletal signs of juvenile DM over a 36-month period suggests that the cutaneous and muscle vasculopathies have different pathophysiologic mechanisms. These findings indicate that efforts to identify the optimal treatment of cutaneous features in juvenile DM require greater attention.

A Practical Approach to Juvenile Dermatomyositis and Juvenile Scleroderma.

Science.gov (United States)

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.

Radiographic abnormalities of the wrist in adult-onset still disease: Comparison with juvenile chronic arthritis and rheumatoid arthritis

International Nuclear Information System (INIS)

Bjorkengren, A.G.; Pathria, M.N.; Terkeltaub, R.; Esdaile, J.; Weisman, M.; Sartoris, D.J.; Resnick, D.

1987-01-01

Pericapitate involvement of the wrist has been described as characteristic of adult-onset Still disease, a relatively rare disorder that is often diagnosed by exclusion after extensive and frequently invasive tests. To evaluate the diagnostic value of carpal radiography in cases of adult-onset Still disease, a retrospective blinded analysis of 48 patients, 16 each with adult-onset Still disease, juvenile chronic arthritis, and rheumatoid arthritis, was performed. Pericapitate articular alterations without radiocarpal involvement were found to be frequent in the setting of adult-onset Still disease but distinctly unusual among patients with rheumatoid arthritis. In juvenile chronic arthritis, severe pericapitate involvement was frequent, but generally occurred in conjunction with radiocarpal joint abnormalities

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.

Intestinal absorption of /sup 47/Ca in elderly patients with osteoporosis, Paget's disease, and osteomalacia. Effects of calcitonin, oestrogen, and vitamin D2

Energy Technology Data Exchange (ETDEWEB)

Lender, M.; Verner, E.; Stankiewicz, H.; Menczel, J.

1977-01-01

The intestinal absorption of /sup 47/Ca was studied in elderly patients. A standard dose of 10 ..mu..Ci of /sup 47/Ca was given orally. The radioactivity was measured in the plasma, and expressed as percentage of the administered dose per litre plasma. As a control group served 12 patients aged 60--80 years, hospitalized for observation for various reasons, receiving no medical treatment and not suffering from any known metabolic bone diseases or other metabolic pathological conditions. Results of kinetic curves demonstrate in elderly patients a decreased absorption with maximum specific activity in plasma reached at 120 min, when compared to data from the literature referring to a group of young people with a mean age of 35 years. Oestrogen treatment, given as ethinyl oestradiol 10 ..mu..g once daily per os for 10 days proved to increase /sup 47/Ca absorption as was demonstrated in 2 patients with osteoporosis. The effect of calcitonin (160 MRC units given 45 min before the test) on calcium absorption, in 5 patients with Paget's disease or osteoporosis appears as biphasic: in the first hour depressing calcium absorption and then in the second and third hours increasing the absorption, suggesting a hyperparathyroid state secondary to the calcitonin effect. The vitamin D2 treatment proved to increase calcium absorption.

Antibodies in juvenile-onset myositis.

Science.gov (United States)

Tansley, Sarah L

2016-11-01

Juvenile-onset myositis is a highly heterogeneous disease. Myositis-specific and associated autoantibodies provide a potential means of subdividing patients into clinically homogenous subgroups. Given the increasing availability of autoantibody testing, this review explores the phenotypes associated with different autoantibodies in juvenile-onset myositis and the potential clinical utility of autoantibody testing. Autoantibodies can be identified in 60-70% of children with myositis and the recent discovery of novel myositis-associated autoantibodies in adult patients suggests this may increase in the near future. Detailed phenotype descriptions are now known for several autoantibodies commonly identified in juvenile-onset disease. Whilst there is insufficient evidence to recommend a differential treatment approach based on autoantibody status, it is becoming increasingly clear that some autoantibody subgroups are often treatment resistant and may benefit from a more aggressive approach. The validation of nonspecialised methods for myositis-specific autoantibody detection should lead to more widely available testing. In juvenile-onset disease, this will provide detailed prognostic information and in the future may also influence approach.

The challenge of juvenile Huntington disease: to test or not to test.

Science.gov (United States)

Koutsis, Georgios; Karadima, Georgia; Kladi, Athina; Panas, Marios

2013-03-12

In a cohort of patients with suspected juvenile-onset Huntington disease (HD), we compared HD expansion-positive and -negative cases in order to identify parameters that may allow differentiating between them and may act as a guide to clinicians contemplating genetic testing. We analyzed the clinical and genetic characteristics of 76 juvenile-onset patients referred consecutively for HD genetic testing over a 16-year period. In total, 24 patients were positive for the HD expansion (7.8% of our HD cohort). Mean age at onset of expanded cases was similar to unexpanded cases. All expanded cases had a family history of genetically confirmed HD compared to only 13.5% of unexpanded cases (p = 0.000). Clinical symptoms at onset or at presentation could not differentiate between expanded and unexpanded patients. Although criteria suggested by previous reports allowed statistical differentiation between the 2 groups, they were not sufficiently sensitive and specific to be used in clinical context and performed less satisfactorily than presence of a family history of HD alone. A diagnosis of juvenile HD should be primarily contemplated in symptomatic children with a family history of HD, although a proportion of these will test negative. With no family history of HD, juvenile HD is very unlikely and genetic testing should never delay searching for other causes. The specific nature of symptoms at onset or at presentation is of limited value in guiding the decision to test or not to test.

First case of juvenile granulosa cell tumor in an adult with Ollier disease.

NARCIS (Netherlands)

Rietveld, L.; Nieboer, T.E.; Kluivers, K.B.; Schreuder, H.W.B.; Bulten, J.; Massuger, L.F.A.G.

2009-01-01

Ollier's disease (OD) is a rare disorder associated with the presence of multiple enchondromas. Granulosa cell tumors are rare sex cord-stromal ovarian tumors. This is the first report of a patient in her fourth decade with a combination of OD and juvenile granulosa cell tumor.A 36-year-old woman

Sphingomyelin lipidosis (Niemann-Pick disease) in a juvenile raccoon (Procyon lotor).

Science.gov (United States)

Vapniarsky, N; Wenger, D A; Scheenstra, D; Mete, A

2013-01-01

A wild caught juvenile male raccoon with neurological disease was humanely destroyed due to poor prognosis. Necropsy examination revealed hepatomegaly, splenomegaly and multicentric lymphadenomegaly with diffuse hepatic pallor and pulmonary consolidation with pinpoint pale subpleural foci. Microscopically, there was marked pale cytoplasmic swelling of the central and peripheral neurons as well as the glial cells in the brain, accompanied by multiorgan infiltration by abundant foamy macrophages. Ultrastructural investigation revealed accumulation of concentrically arranged lamellar material within lysosomes of the affected neurons, macrophages and endothelial cells. Biochemical enzymatic analysis detected sphingomyelinase deficiency and lysosomal storage disease consistent with sphingomyelin lipidosis (Niemann-Pick disease [NPD]) was diagnosed. This is the first report of NPD in a raccoon. Copyright © 2013 Elsevier Ltd. All rights reserved.

Management of patients with chronic kidney disease

African Journals Online (AJOL)

management of the complications of CKD, e.g. renal anaemia, ... ARTICLE. Management of patients with chronic kidney disease. T Gerntholtz,1 FCP (SA); G Paget,2 ..... Telmisartan, ramipril, or both in patients at high risk for vascular events.

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

Science.gov (United States)

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

2016-01-01

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

How Is Paget's Disease of Bone Diagnosed?

Science.gov (United States)

... Paget’s disease. Sometimes the disease is found by accident when one of these tests is done for ... contains a higher-than-usual level of a chemical substance called serum alkaline ... that is in the process of healing. However, a SAP level greater than ...

Chromosome 16 microdeletion in a patient with juvenile neuronal ceroid lipofuscinosis (Batten disease)

NARCIS (Netherlands)

Taschner, P. E.; de Vos, N.; Thompson, A. D.; Callen, D. F.; Doggett, N.; Mole, S. E.; Dooley, T. P.; Barth, P. G.; Breuning, M. H.

1995-01-01

The gene that is involved in juvenile neuronal ceroid lipofuscinosis (JNCL), or Batten disease--CLN3--has been localized to 16p12, and the mutation shows a strong association with alleles of microsatellite markers D16S298, D16S299, and D16S288. Recently, haplotype analysis of a Batten patient from a

Validity and predictive ability of the juvenile arthritis disease activity score based on CRP versus ESR in a Nordic population-based setting

DEFF Research Database (Denmark)

Nordal, E B; Zak, M; Aalto, K

2012-01-01

To compare the juvenile arthritis disease activity score (JADAS) based on C reactive protein (CRP) (JADAS-CRP) with JADAS based on erythrocyte sedimentation rate (ESR) (JADAS-ESR) and to validate JADAS in a population-based setting.......To compare the juvenile arthritis disease activity score (JADAS) based on C reactive protein (CRP) (JADAS-CRP) with JADAS based on erythrocyte sedimentation rate (ESR) (JADAS-ESR) and to validate JADAS in a population-based setting....

Juvenile Angiofibroma: Evolution of Management

Science.gov (United States)

Nicolai, Piero; Schreiber, Alberto; Bolzoni Villaret, Andrea

2012-01-01

Juvenile angiofibroma is a rare benign lesion originating from the pterygopalatine fossa with distinctive epidemiologic features and growth patterns. The typical patient is an adolescent male with a clinical history of recurrent epistaxis and nasal obstruction. Although the use of nonsurgical therapies is described in the literature, surgery is currently considered the ideal treatment for juvenile angiofibroma. Refinement in preoperative embolization has provided significant reduction of complications and intraoperative bleeding with minimal risk of residual disease. During the last decade, an endoscopic technique has been extensively adopted as a valid alternative to external approaches in the management of small-intermediate size juvenile angiofibromas. Herein, we review the evolution in the management of juvenile angiofibroma with particular reference to recent advances in diagnosis and treatment. PMID:22164185

Juvenile Angiofibroma: Evolution of Management

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Piero Nicolai

2012-01-01

Full Text Available Juvenile angiofibroma is a rare benign lesion originating from the pterygopalatine fossa with distinctive epidemiologic features and growth patterns. The typical patient is an adolescent male with a clinical history of recurrent epistaxis and nasal obstruction. Although the use of nonsurgical therapies is described in the literature, surgery is currently considered the ideal treatment for juvenile angiofibroma. Refinement in preoperative embolization has provided significant reduction of complications and intraoperative bleeding with minimal risk of residual disease. During the last decade, an endoscopic technique has been extensively adopted as a valid alternative to external approaches in the management of small-intermediate size juvenile angiofibromas. Herein, we review the evolution in the management of juvenile angiofibroma with particular reference to recent advances in diagnosis and treatment.

Unbiased Stereologic Estimation of the Spatial Distribution of Paget’s Disease in the Human Temporal Bone

DEFF Research Database (Denmark)

Bloch, Sune Land; Sørensen, Mads Sølvsten

2014-01-01

remodeling around the inner ear space and to compare it with that of otosclerosis in a contemporary context of temporal bone dynamics. MATERIALS AND METHODS: From the temporal bone collection of Massachusetts Eye and Ear Infirmary, 15 of 29 temporal bones with Paget's disease were selected to obtain...... an independent sample. All volume distributions were obtained along the normal axis of capsular bone remodeling activity by the use of vector-based stereology. RESULTS: Pagetic bone remodeling was distributed centrifugally around the inner ear space at the individual and the general level. This pattern...

The Italian translation and adaptation of The Service Standards for Therapeutic Communities for Children and Young People 2nd Edition (by John O’Sullivan & Sarah Paget

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Simone Bruschetta

2014-09-01

Full Text Available This work presents the adaptation to the Italian context of the "Community of Communities standards" (by O'Sullivan & Paget. We have tried to remain as faithful as possible to the Community of Communities clinical sense, organizational and psychodynamic statements, while still trying to locate the huge experience behind the philosophy of the therapeutic community in the socio-political Italian context. In Italy, health work is heavily regulated within public institutions with their own competences that cover specific administrative services, different types of users and particular territorial areas. Especially in the health services to childhood, the institutional and legal powers that law administration attaches to families, public schools, social services of territorial competence and local health authorities, require an articulated taking charge, involving all these institutions, which is often added to the Juvenile Court of Justice, with its almost limitless powers of decree. For this reason, in chapter 5.4. of list (collaborating, we proposed two additional items (5.4.3 and 5.4.4, to 5.4.1 and 5.4.2, to better define the design mode of the clinical taking care by communities in Italian context and culture. These are the only items added to the text.Keywords: Therapeutic Community, Children, Yang People, Service Standards, Clinical Quality

Observations on total-body calcium in humans with bone disease

International Nuclear Information System (INIS)

Spinks, T.J.; Bewley, D.K.; Ranicar, A.S.O.; Joplin, G.F.; Evans, I.M.A.; Vlotides, J.; Paolillo, M.

1979-01-01

Total-body calcium was measured in-vivo by neutron activation in a number of patients suffering from metabolic abnormalities which affect the skeleton. In general, less than 2% of total calcium resides in tissue other than bone allowing calcium mass to be directly related to skeletal mass. The conditions studied were (i) Paget's disease, treated with synthetic human calcitonin, (ii) osteoporosis, treated variously with calcium and phosphate supplements and 1,25 hydroxycholecalciferol, and (iii) Cushing's disease treated by pituitary implant of 198 Au or 90 Y seeds. The neutron beam used in these studies was produced by bombarding a beryllium target with deuterons accelerated in a cyclotron. The mean neutron energy was 7.5 MeV and patients received a total dose of 1 rem in about 30 s, a bilateral irradiation being employed. Measurements were made at approximately yearly intervals, the maximum period of study being about four and a half years. The precision of the method was estimated to be +-3% (SE) and a correction was applied for changes in body weight. In most patients, total calcium remained stable. However, in the Paget's patients, there was an indication of a slow upward trend while the osteoporotics (both treated and untreated) showed on average no change. Most of the patients with Cushing's disease showed no recovery of skeletal mass. Absolute calibration indicated that mean total body calcium in the Paget's patients was close to a predicted normal while that for the osteoporotic and Cushing's patients was 20-25% below this. (author)

Founders lecture 2007. Metabolic bone disease: what has changed in 30 years?

Energy Technology Data Exchange (ETDEWEB)

Sundaram, Murali [Cleveland Clinic, Diagnostic Radiology, MSK, Cleveland, OH (United States)

2009-09-15

To provide an update on imaging of metabolic bone disease based on new developments, findings, and changing practices over the past 30 years. Literature review of osteoporosis, osteomalacia, renal osteodystrophy, Paget's disease, bisphosphonates, with an emphasis on imaging. Cited references and pertinent findings. Significant developments have occurred in the imaging of metabolic bone disease over the past 30 years. (orig.)

Juvenile Huntington's disease confirmed by genetic examination in twins Doença de Huntington juvenil confirmada por exame genético em gêmeas

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GILBERTO LEVY

1999-09-01

Full Text Available Early-onset Huntington's disease (HD occurs in approximately 10% of HD's cases. We report juvenile HD in phenotypically identical twins, evaluated by history, clinical and neurologic examination, mini-mental state examination, blood laboratory exams, cerebrospinal fluid examination, skull computed tomography, and genetic examination for HD. Patients had the akinetic-rigid variety (Westphal variant of the disease and paternal inheritance. The laboratory workup confirmed the clinical diagnosis of HD, which adds this report to the rare cases of HD in twins reported in the literature.Doença de Huntington (DH de início precoce ocorre em aproximadamente 10% dos casos de DH. Relatamos DH juvenil em gêmeas fenotipicamente idênticas, avaliadas por história, exames clínico e neurológico, mini-exame do estado mental, exames de sangue, exame do líquido cefalorraquidiano, tomografia computadorizada de crânio e exame genético para DH. As pacientes apresentavam a variedade rígido-acinética (variante de Westphal da doença e herança paterna. A avaliação laboratorial confirmou o diagnóstico clínico de DH, acrescentando-se este relato aos raros casos de DH em gêmeos relatados na literatura.

Temporary Efficacy of Pyrimethamine in Juvenile-Onset Tay-Sachs Disease Caused by 2 Unreported HEXA Mutations in the Indian Population.

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Udwadia-Hegde, Anaita; Hajirnis, Omkar

2017-01-01

Juvenile Tay-Sachs disease is rarer than other forms of Tay-Sachs disease and is usually seen in children between the age of 2 and 10 years. Pyrimethamine as a pharmacological chaperone was used to increase β-hexosaminidase A activity in this patient. We describe a patient with Tay-Sachs disease from the Indian population, a juvenile case who presented with developmental regression starting at the age of three, initially with motor followed by language regression. She is currently incapacitated with severe behavioral issues. This brief communication gives an insight into the efficacy of pharmacological chaperones. It also describes two unreported mutations in hexosaminidase A gene from the Indian population. After commencing Pyrimethamine, though initial benefits with increase in levels corresponded with briefly halting the motor regression, the observed increase was only transient and not associated with discernible beneficial neurological or psychiatric effects.

JUVENILE SCLERODERMA-what has changed in the meantime?

Science.gov (United States)

Adrovic, Amra; Sahin, Sezgin; Barut, Kenan; Kasapcopur, Ozgur

2018-04-22

Juvenile scleroderma is a rarely seen chronic connective tissue disorder characterized by stiffening of the skin. The frequency of the disease was reported as one per million. According to organ involvement, the disease is divided into two main forms: systemic and localized scleroderma. Since it is uncommon in children, many aspects of the disease remain discussable. With this review, we aimed to revise recent findings and new developments in this rare condition. Skin manifestations are most prominent feature of the systemic form, followed by musculoskeletal and vascular involvement. Cardiovascular, gastrointestinal and renal disorders are rare in childhood. Combination of disease modifying antirheumatic drugs (methotrexate, mycophenolate-mofetil, cyclosporine) and steroid reprents the first line therapy. Bosentan is used for cases with pulmonary hypertension and for extensive digital ulcerations. Biological treatment emerges as a useful treatment option in most severe form of the disease. Localized scleroderma is characterized with sclerodermatosis of the skin. Internal organ involvement is not expected. Classification of the local scleroderma is made according to the size and localization of the skin changes. There are few different therapeutical options but there is no specific therapy for the localized scleroderma. Many data regarding disease features and treatment options in juvenile scleroderma are based on studies among adults. There is a striking need for multicentric, prospective studies among children with juvenile scleroderma.Emerging biological agents and new treatment options are showing promising results. Anyhow, juvenile scleroderma remains a mystery with many aspects of the disease waiting to be solved. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

Temporary Efficacy of Pyrimethamine in Juvenile-Onset Tay-Sachs Disease Caused by 2 Unreported HEXA Mutations in the Indian Population

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Anaita Udwadia-Hegde MD, DCH, MRCPCH

2017-01-01

Full Text Available Background: Juvenile Tay-Sachs disease is rarer than other forms of Tay-Sachs disease and is usually seen in children between the age of 2 and 10 years. Pyrimethamine as a pharmacological chaperone was used to increase β-hexosaminidase A activity in this patient. Patient: We describe a patient with Tay-Sachs disease from the Indian population, a juvenile case who presented with developmental regression starting at the age of three, initially with motor followed by language regression. She is currently incapacitated with severe behavioral issues. Conclusion: This brief communication gives an insight into the efficacy of pharmacological chaperones. It also describes two unreported mutations in hexosaminidase A gene from the Indian population. After commencing Pyrimethamine, though initial benefits with increase in levels corresponded with briefly halting the motor regression, the observed increase was only transient and not associated with discernible beneficial neurological or psychiatric effects.

Juvenile generalized pustular psoriasis is a chronic recalcitrant disease: an analysis of 27 patients seen in a tertiary hospital in Johor, Malaysia.

Science.gov (United States)

Lau, Bi-Wen; Lim, Dee-Zhen; Capon, Francesca; Barker, Jonathan N; Choon, Siew-Eng

2017-04-01

Limited information exists regarding juvenile generalized pustular psoriasis (GPP). We aim to determine the clinical profile and outcome of Malaysians with juvenile GPP. Review of hospital case notes on patients with juvenile GPP. Twenty-seven patients with juvenile GPP were identified. Female to male ratio was 1.4:1. The median age at onset of GPP was 6.5 years. Ten patients had prior psoriasis with a median pre-pustular duration of 2.7 years. Onset of GPP was earlier in patients without prior psoriasis (5.1 years vs. 12.0 years, P = 0.002). Precipitating factors identified included stress, upper respiratory tract infection, systemic steroid use, vaccination, and pregnancy. A positive family history of psoriasis and GPP was present in six and one patient(s), respectively. Twenty-one patients had acute, five annular, and one localized variant of GPP. Arthritis was present in 22.2%. Fever, leukocytosis, and transaminitis were mainly seen in patients with acute GPP at 80.9, 72.2, and 11.1%, respectively. Among 20 patients screened, eight carry IL36RN variants and one has CARD14 mutation. IL36RN-positive patients have more severe disease characterized by early onset, low prevalence of prior plaque psoriasis, high prevalence of systemic inflammation, and need for continuous long-term systemic therapy. Acitretin and cyclosporine were effective in aborting acute GPP in 100% of 16 and 66.7% of six patients treated, respectively. However, relapses were common. Only three of the 17 patients whose initial acute GPP was controlled with systemic agents were successfully weaned off treatment. Juvenile GPP is a chronic recalcitrant disease. IL36RN-positive patients have more severe disease. © 2017 The International Society of Dermatology.

Analysis of the Juvenile Idiopathic Arthritis Immunization Schedule

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L. S. Namazova-Baranova

2016-01-01

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

Juvenile chronic arthritis into adulthood: a long-term follow-up study

DEFF Research Database (Denmark)

Zak, M; Pedersen, F K

2000-01-01

To evaluate a group of 65 adults with a history of or persistent juvenile chronic arthritis (JCA), on average, 26.4 yr after disease onset.......To evaluate a group of 65 adults with a history of or persistent juvenile chronic arthritis (JCA), on average, 26.4 yr after disease onset....

The treatment of juvenile/adult GM1-gangliosidosis with Miglustat may reverse disease progression.

Science.gov (United States)

Deodato, Federica; Procopio, Elena; Rampazzo, Angelica; Taurisano, Roberta; Donati, Maria Alice; Dionisi-Vici, Carlo; Caciotti, Anna; Morrone, Amelia; Scarpa, Maurizio

2017-10-01

Juvenile and adult GM1-gangliosidosis are invariably characterized by progressive neurological deterioration. To date only symptomatic therapies are available. We report for the first time the positive results of Miglustat (OGT 918, N-butyl-deoxynojirimycin) treatment on three Italian GM1-gangliosidosis patients. The first two patients had a juvenile form (enzyme activity ≤5%, GLB1 genotype p.R201H/c.1068 + 1G > T; p.R201H/p.I51N), while the third patient had an adult form (enzyme activity about 7%, p.T329A/p.R442Q). Treatment with Miglustat at the dose of 600 mg/day was started at the age of 10, 17 and 28 years; age at last evaluation was 21, 20 and 38 respectively. Response to treatment was evaluated using neurological examinations in all three patients every 4-6 months, the assessment of Movement Disorder-Childhood Rating Scale (MD-CRS) in the second patient, and the 6-Minute Walking Test (6-MWT) in the third patient. The baseline neurological status was severely impaired, with loss of autonomous ambulation and speech in the first two patients, and gait and language difficulties in the third patient. All three patients showed gradual improvement while being treated; both juvenile patients regained the ability to walk without assistance for few meters, and increased alertness and vocalization. The MD-CRS class score in the second patient decreased from 4 to 2. The third patient improved in movement and speech control, the distance covered during the 6-MWT increased from 338 to 475 m. These results suggest that Miglustat may help slow down or reverse the disease progression in juvenile/adult GM1-gangliosidosis.

Evaluation of the Role of Vibrational Spectroscopy in the Assessment of Vulval Disease

Science.gov (United States)

2017-08-03

Normal Vulval Skin; Lichen Sclerosus; High Grade Dysplasia - Usual Type ('VIN 2-3'); High Grade Dysplasia - Differentiated Type ('VIN 2-3'); Squamous Cell Carcinoma; Epithelial Hyperplasia Without Atypia; Atypia Not Otherwise Specified/ Low Grade Dysplasia ('VIN 1'); Pagets Disease of the Vulva

Long-term outcome in patients with juvenile dermatomyositis

DEFF Research Database (Denmark)

Mathiesen, P; Hegaard, H; Herlin, Troels

2012-01-01

To evaluate a group of 53 patients with juvenile dermatomyositis (JDM), on average 13.9 years after disease onset, in order to describe the long-term disease outcome and to identify disease-related parameters associated with poor disease outcome....

Juvenile polyposis syndrome

Science.gov (United States)

Hsiao, Yi-Han; Wei, Chin-Hung; Chang, Szu-Wen; Chang, Lung; Fu, Yu-Wei; Lee, Hung-Chang; Liu, Hsuan-Liang; Yeung, Chun-Yan

2016-01-01

Abstract Background: Juvenile polyposis syndrome, a rare disorder in children, is characterized with multiple hamartomatous polyps in alimentary tract. A variety of manifestations include bleeding, intussusception, or polyp prolapse. In this study, we present an 8-month-old male infant of juvenile polyposis syndrome initially presenting with chronic anemia. To the best of our knowledge, this is the youngest case reported in the literature. Methods: We report a rare case of an 8-month-old male infant who presented with chronic anemia and gastrointestinal bleeding initially. Panendoscopy and abdominal computed tomography showed multiple polyposis throughout the entire alimentary tract leading to intussusception. Technetium-99m-labeled red blood cell (RBC) bleeding scan revealed the possibility of gastrointestinal tract bleeding in the jejunum. Histopathological examination on biopsy samples showed Peutz-Jeghers syndrome was excluded, whereas the diagnosis of juvenile polyposis syndrome was established. Results: Enteroscopic polypectomy is the mainstay of the treatment. However, polyps recurred and occupied the majority of the gastrointestinal tract in 6 months. Supportive management was given. The patient expired for severe sepsis at the age of 18 months. Conclusion: Juvenile polyposis syndrome is an inherited disease, so it is not possible to prevent it. Concerning of its poor outcome and high mortality rate, it is important that we should increase awareness and education of the parents at its earliest stages. PMID:27631205

Clinical research of bone scan characteristics for metabolic bone diseases

International Nuclear Information System (INIS)

Zhu Ruisen; Luo Qiong; Lu Haikui; Chen Libo; Luo Quanyong

2009-01-01

Characteristic images of 99m Tc-MDP bone scintigraphy in patients with metabolic bone diseases (MBD) were analyzed and compared, in an attempt to improve the capability of differential diagnosis in this aspect. A total of 142 cases, clinically confirmed as (MBD), were categorized into six groups: hyperparathyroidism (117), renal osteodystrophy (4), Paget's disease (16), hypophosphatemic osteomalacia (2), Albers-Schonberg disease (2), and Brittle bone disease (1). They were diagnosed clinically or pathologically, and scanned with 99m Tc-MDP bone scintegraphy, from which the 142 MBD cases were classified into 4 types. The cases of Type I had increased amount of 99m Tc-MDP uptake in whole body bones, including hyperparathyroidism, Albers-Schonberg disease, brittle bone disease and renal osteodystrophy. The cases of Type II had high uptake of 99m Tc-MDP in local region of bones, including paget's disease, hypophosphatemic osteomalacia and hyperparathyroidism. A Type I case with pathological fracture or secondary osteopathy was classified as Type III. Type IV cases were in early stage of hyperparathyroidism, with normal bone scan image. Analysis of the characteristics of 99m Tc-MDP bone scintigraphic findings (locations, morphology and intensities) in patients with MBD may be helpful in the differential diagnosis of MBD, in association with the patient's history and X-ray data altogether. (authors)

Evolution of Juvenile Ankylosing Spondylitis

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Ye.V. Prohorov

2013-02-01

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.

PSYCHOSOCIAL PROFILE OF JUVENILE DIABETES

Science.gov (United States)

Dass, Jyoti; Dhavale, H.S.; Rathi, Anup

1999-01-01

A study of the complex relationships between the patient characteristics, family and environmental influences, physician's behaviour and the demands of the disease with its management in Juvenile Diabetics was taken up at a general hospital. 90 subjects were selected for the study and grouped into three. Group A consisted of 30 Juvenile Diabetics, Group B of 30 Adult Diabetics and Group C of 30 Normal healthy adolescents. The impact of the illness was measured on the Diabetes Impact Measurement Scale (DIMS), the behavioural deviations and the parental attitudes towards child rearing on the Fallstrom's Questionnaire (FQ) and the family environment on the Family Climate Scale (FCS). Psychiatric morbidity was assessed using DSM-IV criteria. Group A & B were compared on the DIMS and Group A & C on FQ & FCS. Adult diabetics had a greater impact of diabetes. Juvenile diabetics had significantly higher frequency of behavioural deviations as compared to controls. Also there was a higher number of responses on questions indicating an overprotecting attitude amongst parents of juvenile diabetics. There was an increased incidence of psychiatric morbidity in juvenile diabetics as compared to normal adolescents irrespective of the family environment. The results are discussed in relation to current literature. PMID:21430802

Multidisciplinary management of Paget-Schroetter syndrome. A case series of eight patients.

Science.gov (United States)

Rosa Salazar, Vladimir; Otálora Valderrama, Sonia Del Pilar; Hernández Contreras, María Encarnación; García Pérez, Bartolomé; Arroyo Tristán, Andrés Del Amor; García Méndez, María Del Mar

2015-08-01

Paget-Schroetter syndrome (PSS) in the context of upper extremity deep venous thrombosis (DVT) is an uncommon but potentially very serious condition affecting young, healthy adults, in which secondary post-thrombotic syndrome (PTS) can be a complication with major implications. The best treatment option remains controversial, with current guidelines recommending anticoagulation for at least 3 months. However, an incidence of PTS of approximately 50% after 6 months, 30% after 1 year and 25% after 2 years has been found using this therapeutic approach. Consequently, specialized units recommend local thrombolysis and early decompressive surgery. We describe a series of eight cases treated in this way. None of the patients showed signs of complications, and an early return to regular activities with no PTS was observed in 90% of cases. Copyright © 2014 SEPAR. Published by Elsevier Espana. All rights reserved.

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.

Naevoxanthoendothelioma (Synonym: Juvenile Xanthogranuloma

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F Handa

1978-01-01

Full Text Available A case of naevoxanthoendothelioma juvenile xanthogranuloma is reported with rare features like late onset of the disease, involvement of liver and diffuse cutaneous lesions including cafe au lait spots and pigmented naevus. Final diagnosis could be achieved only on histopathology report.

A Case of Juvenile Huntington Disease in a 6-Year-Old Boy

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Jun-Sang Sunwoo

2010-10-01

Full Text Available Huntington disease is a neurodegenerative disorder distinguished by the triad of dominant inheritance, choreoathetosis and dementia, usually with onset in the fourth and fifth decades. It is caused by an unstable cytosine-adenine-guanine (CAG trinucleotide repeat expansion in the gene IT15 in locus 4p16.3. Juvenile HD that constitutes about 3% to 10% of all patients is clinically different from adult-onset form and characterized by a larger number of CAG repeats typically exceeding 60. We report a case of a 6-year-old boy with myoclonic seizure and 140 CAG repeats confirmed by molecular genetic analysis.

X-linked juvenile retinoschisis: mutations at the retinoschisis and Norrie disease gene loci?

Science.gov (United States)

Hiraoka, M; Rossi, F; Trese, M T; Shastry, B S

2001-01-01

Juvenile retinoschisis (RS) and Norrie disease (ND) are X-linked recessive retinal disorders. Both disorders, in the majority of cases, are monogenic and are caused by mutations in the RS and ND genes, respectively. Here we report the identification of a family in which mutations in both the RS and ND genes are segregating with RS pathology. Although the mutations identified in this report were not functionally characterized with regard to their pathogenicity, it is likely that both of them are involved in RS pathology in the family analyzed. This suggests the complexity and digenic nature of monogenic human disorders in some cases. If this proves to be a widespread problem, it will complicate the strategies used to identify the genes involved in diseases and to develop methods for intervention.

Temporomandibular Joint Involvement in Association With Quality of Life, Disability, and High Disease Activity in Juvenile Idiopathic Arthritis.

Science.gov (United States)

Frid, Paula; Nordal, Ellen; Bovis, Francesca; Giancane, Gabriella; Larheim, Tore A; Rygg, Marite; Pires Marafon, Denise; De Angelis, Donato; Palmisani, Elena; Murray, Kevin J; Oliveira, Sheila; Simonini, Gabriele; Corona, Fabrizia; Davidson, Joyce; Foster, Helen; Steenks, Michel H; Flato, Berit; Zulian, Francesco; Baildam, Eileen; Saurenmann, Rotraud K; Lahdenne, Pekka; Ravelli, Angelo; Martini, Alberto; Pistorio, Angela; Ruperto, Nicolino

2017-05-01

To evaluate the demographic, disease activity, disability, and health-related quality of life (HRQOL) differences between children with juvenile idiopathic arthritis (JIA) and their healthy peers, and between children with JIA with and without clinical temporomandibular joint (TMJ) involvement and its determinants. This study is based on a cross-sectional cohort of 3,343 children with JIA and 3,409 healthy peers, enrolled in the Pediatric Rheumatology International Trials Organisation HRQOL study or in the methotrexate trial. Potential determinants of TMJ involvement included demographic, disease activity, disability, and HRQOL measures selected through univariate and multivariable logistic regression. Clinical TMJ involvement was observed in 387 of 3,343 children with JIA (11.6%). Children with TMJ involvement, compared to those without, more often had polyarticular disease course (95% versus 70%), higher Juvenile Arthritis Disease Activity Score (odds ratio [OR] 4.6), more disability, and lower HRQOL. Children with TMJ involvement experienced clearly more disability and lower HRQOL compared to their healthy peers. The multivariable analysis showed that cervical spine involvement (OR 4.6), disease duration >4.4 years (OR 2.8), and having more disability (Childhood Health Assessment Questionnaire Disability Index >0.625) (OR 1.6) were the most important determinants for TMJ involvement. Clinical TMJ involvement in JIA is associated with higher disease activity, higher disability, and impaired HRQOL. Our findings indicate the need for dedicated clinical and imaging evaluation of TMJ arthritis, especially in children with cervical spine involvement, polyarticular course, and longer disease duration. © 2016, American College of Rheumatology.

Juvenile Dermatomyositis in Pregnancy

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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.

[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.

The Diversity of Juvenile Sarcoidosis Symptoms

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O. Vougiouka

2012-01-01

Full Text Available We report a case of juvenile sarcoidosis, emphasizing the variety of clinical manifestations. The child had uveitis, which is among the most common manifestations of the disease. However, fever of unknown origin, glomerulonephritis and lymphadenopathy were also noticed, underscoring the diversity of the clinical spectrum of the disease.

A central role for TOR signalling in a yeast model for juvenile CLN3 disease

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Michael E. Bond

2015-11-01

Full Text Available Yeasts provide an excellent genetically tractable eukaryotic system for investigating the function of genes in their biological context, and are especially relevant for those conserved genes that cause disease. We study the role of btn1, the orthologue of a human gene that underlies an early onset neurodegenerative disease (juvenile CLN3 disease, neuronal ceroid lipofuscinosis (NCLs or Batten disease in the fission yeast Schizosaccharomyces pombe. A global screen for genetic interactions with btn1 highlighted a conserved key signalling hub in which multiple components functionally relate to this conserved disease gene. This signalling hub includes two major mitogen-activated protein kinase (MAPK cascades, and centers on the Tor kinase complexes TORC1 and TORC2. We confirmed that yeast cells modelling CLN3 disease exhibit features consistent with dysfunction in the TORC pathways, and showed that modulating TORC function leads to a comprehensive rescue of defects in this yeast disease model. The same pathways may be novel targets in the development of therapies for the NCLs and related diseases.

Ankle arthritis predicts polyarticular disease course and unfavourable outcome in children with juvenile idiopathic arthritis

DEFF Research Database (Denmark)

Esbjörnsson, Anna-Clara; Aalto, Kristiina; Broström, Eva W

2015-01-01

OBJECTIVES: To evaluate the occurrence, clinical characteristics and prognostic factors associated with ankle arthritis in children with juvenile idiopathic arthritis (JIA). METHODS: 440 children with JIA were followed for eight years in a prospective Nordic population-based cohort study. Data...... on remission was available for 427 of these children. Occurrence of clinically assessed ankle arthritis was analysed in relation to JIA category, clinical characteristics and remission data eight years after disease onset. RESULTS: In 440 children with JIA, 251 (57%) experienced ankle arthritis during...... the first eight years of disease. Ankle arthritis was least common in the persistent oligoarticular category (25%) and most common in children with extended oligoarticular (83%) and polyarticular RF-negative (85%) JIA. Children who developed ankle arthritis during the first year of disease were younger...

Juvenile Dermatomyositis in a Nigerian Girl: a Case Report ...

African Journals Online (AJOL)

Juvenile Dermatomyositis in a Nigerian Girl: a Case Report. MG Mustapha, MG Ashir, AA Mayun, Y Machoco, AB Ibrahim. Abstract. A case of Juvenile dermatomyositis (JDM) in a 10 year old Nigerian girl is herein reported to discuss some of the features of the disease and challenges in management of such a rare but ...

Distribution pattern of MRI abnormalities within the knee and wrist of juvenile idiopathic arthritis patients: signature of disease activity

NARCIS (Netherlands)

Nusman, Charlotte M.; Hemke, Robert; Schonenberg, Dieneke; Dolman, Koert M.; van Rossum, Marion A. J.; van den Berg, J. Merlijn; Kuijpers, Taco W.; Maas, Mario

2014-01-01

The aim of this study in clinically active juvenile idiopathic arthritis (JIA) was to assess the frequency and distribution pattern of synovitis as hallmark of disease and additional soft-tissue and bony abnormalities on MRI in the knee and wrist as two target joints. MRI datasets of 153 clinically

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 many blood ...

Retrocalcaneal bursitis in juvenile chronic arthritis.

OpenAIRE

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.

Cardiac juvenile xanthogranuloma in an infant presenting with pericardial effusion.

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Kobayashi, Daisuke; Delius, Ralph E; Debelenko, Larisa V; Aggarwal, Sanjeev

2013-01-01

Juvenile xanthogranuloma is a rare histiocytic disorder of childhood mainly affecting skin and rarely deep soft tissues and viscera. We report a 2-month-old infant who presented with respiratory distress secondary to a large pericardial effusion associated with an epicardial mass. Excisional biopsy was performed and the mass was diagnosed as juvenile xanthogranuloma. The child is well without evidence of disease 8 months following the excision. The corresponding literature on juvenile xanthogranuloma with cardiac manifestations is reviewed. © 2012 Wiley Periodicals, Inc.

Juvenile xanthogranuloma of the corneoscleral limbus.

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Yanoff, M; Perry, H D

1995-07-01

Juvenile xanthogranuloma is a rare and usually benign skin disease of unknown cause that occurs in infants and young children. We studied a case of juvenile xanthogranuloma of the corneoscleral limbus in a 17-year-old black boy, who presented with a 5-month history of a lump in the right eye. The lesion extended from the superior limbus, fanning out as it proceeded posteriorly for 6 mm with a width of 9 mm and a height of 2 to 3 mm. This yellow-orange mass was vascular and firmly fixed to the underlying tissue. The lesion was diagnosed as a dermoid and observed for 7 months without documented growth before an uneventful excisional biopsy was performed. The pathologic diagnosis showed the characteristic picture of juvenile xanthogranuloma with numerous Touton giant cells. Lipid stains provided further confirmation.

Bilateral coxofemoral degenerative joint disease in a juvenile male yellow-eyed penguin (Megadyptes antipodes).

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Buckle, Kelly N; Alley, Maurice R

2011-08-01

A juvenile, male, yellow-eyed penguin (Megadyptes antipodes) with abnormal stance and decreased mobility was captured, held in captivity for approximately 6 weeks, and euthanized due to continued clinical signs. Radiographically, there was bilateral degenerative joint disease with coxofemoral periarticular osteophyte formation. Grossly, the bird had bilaterally distended, thickened coxofemoral joints with increased laxity, and small, roughened and angular femoral heads. Histologically, the left femoral articular cartilage and subchondral bone were absent, and the remaining femoral head consisted of trabecular bone overlain by fibrin and granulation tissue. There was no gross or histological evidence of infection. The historic, gross, radiographic, and histopathologic findings were most consistent with bilateral aseptic femoral head degeneration resulting in degenerative joint disease. Although the chronicity of the lesions masked the initiating cause, the probable underlying causes of aseptic bilateral femoral head degeneration in a young animal are osteonecrosis and osteochondrosis of the femoral head. To our knowledge, this is the first reported case of bilateral coxofemoral degenerative joint disease in a penguin.

Genetics Home Reference: juvenile idiopathic arthritis

Science.gov (United States)

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

Dental and facial characteristics of patients with juvenile idiopathic arthritis Características dentárias e faciais de pacientes com artrite idiopática juvenil

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Cynthia Savioli

2004-01-01

Full Text Available OBJECTIVE: It has been shown that the temporomandibular joint is frequently affected by juvenile idiopathic arthritis, and this degenerative disease, which may occur during facial growth, results in severe mandibular dysfunction. However, there are no studies that correlate oral health (tooth decay and gingival diseases and temporomandibular joint dysfunction in patients with juvenile idiopathic arthritis. The aim of this study is to evaluate the oral and facial characteristics of the patients with juvenile idiopathic arthritis treated in a large teaching hospital. METHOD: Thirty-six patients with juvenile idiopathic arthritis (26 female and 10 male underwent a systematic clinical evaluation of their dental, oral, and facial structures (DMFT index, plaque and gingival bleeding index, dental relationship, facial profile, and Helkimo's index. The control group was composed of 13 healthy children. RESULTS: The mean age of the patients with juvenile idiopathic arthritis was 10.8 years; convex facial profile was present in 12 juvenile idiopathic arthritis patients, and class II molar relation was present in 12 (P = .032. The indexes of plaque and gingival bleeding were significant in juvenile idiopathic arthritis patients with a higher number of superior limbs joints involved (P = .055. Anterior open bite (5 and temporomandibular joint noise (8 were present in the juvenile idiopathic arthritis group. Of the group in this sample, 94% (P = .017 had temporomandibular joint dysfunction, 80% had decreased mandibular opening (P = 0.0002, and mandibular mobility was severely impaired in 33% (P = .015. CONCLUSION: This study confirms that patients with juvenile idiopathic arthritis a have a high incidence of mandibular dysfunction that can be attributed to the direct effect of the disease in the temporomandibular joint and b have a higher incidence of gingival disease that can be considered a secondary effect of juvenile idiopathic arthritis on oral health

Venous Thoracic Outlet Compression and the Paget-Schroetter Syndrome: A Review and Recommendations for Management

International Nuclear Information System (INIS)

Thompson, J. F.; Winterborn, R. J.; Bays, S.; White, H.; Kinsella, D. C.; Watkinson, A. F.

2011-01-01

Paget Schroetter syndrome, or effort thrombosis of the axillosubclavian venous system, is distinct from other forms of upper limb deep vein thrombosis. It occurs in younger patients and often is secondary to competitive sport, music, or strenuous occupation. If untreated, there is a higher incidence of disabling venous hypertension than was previously appreciated. Anticoagulation alone or in combination with thrombolysis leads to a high rate of rethrombosis. We have established a multidisciplinary protocol over 15 years, based on careful patient selection and a combination of lysis, decompressive surgery, and postoperative percutaneous venoplasty. During the past 10 years, a total of 232 decompression procedures have been performed. This article reviews the literature and presents the Exeter Protocol along with practical recommendations for management.

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

An integrated approach to consumer representation and involvement in a multicentre randomized controlled trial.

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Langston, Anne L; McCallum, Marilyn; Campbell, Marion K; Robertson, Clare; Ralston, Stuart H

2005-01-01

Although, consumer involvement in individual studies is often limited, their involvement in guiding health research is generally considered to be beneficial. This paper outlines our experiences of an integrated relationship between the organisers of a clinical trial and a consumer organisation. The PRISM trial is a UK multicentre, randomized controlled trial comparing treatment strategies for Paget's disease of the bone. The National Association for the Relief of Paget's Disease (NARPD) is the only UK support group for sufferers of Paget's disease and has worked closely with the PRISM team from the outset. NARPD involvement is integral to the conduct of the trial and specific roles have included: peer-review; trial steering committee membership; provision of advice to participants, and promotion of the trial amongst Paget's disease patients. The integrated relationship has yielded benefits to both the trial and the consumer organisation. The benefits for the trial have included: recruitment of participants via NARPD contacts; well-informed participants; unsolicited patient advocacy of the trial; and interested and pro-active collaborators. For the NARPD and Paget's disease sufferers, benefits have included: increased awareness of Paget's disease; increased access to relevant health research; increased awareness of the NARPD services; and wider transfer of diagnosis and management knowledge to/from health care professionals. Our experience has shown that an integrated approach between a trial team and a consumer organisation is worthwhile. Adoption of such an approach in other trials may yield significant improvements in recruitment and quality of participant information flow. There are, however, resource implications for both parties.

Imaging of juvenile spondyloarthritis. Part I: Classifications and radiographs

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Iwona Sudoł-Szopińska

2017-09-01

Full Text Available Juvenile spondyloarthropathies are manifested mainly by symptoms of peripheral arthritis and enthesitis. By contrast with adults, children rarely present with sacroiliitis and spondylitis. Imaging and laboratory tests allow early diagnosis and treatment. Conventional radiographs visualize late inflammatory lesions and post-inflammatory complications. Early diagnosis is possible with the use of ultrasonography and magnetic resonance imaging. The first part of the article presents classifications of juvenile spondyloarthropathies and discusses their radiographic presentation. Typical radiographic features of individual types of juvenile spondyloarthritis are listed (including ankylosing spondylitis, juvenile psoriatic arthritis, reactive arthritis and arthritis in the course of inflammatory bowel diseases. The second part will describe changes visible on ultrasonography and magnetic resonance imaging. In patients with juvenile spondyloarthropathies, these examinations are conducted to diagnose inflammatory lesions in peripheral joints, tendon sheaths, tendons and bursae. Moreover, magnetic resonance imaging also visualizes early inflammatory changes in the axial skeleton and subchondral bone marrow edema, which is considered an early sign of inflammation.

Exercise testing and fitness training in juvenile idiopathic arthritis

NARCIS (Netherlands)

Singh-Grewal, D.

2010-01-01

Juvenile Idiopathic Arthritis is the commonest rheumatic disease of childhood affecting 1:1000 children under the age of 16 years. Children with JIA have long been sidelined from physical activity due to active disease or irrational concerns that activity may in some way worsen disease. Children

Pain intensity in patients with juvenile idiopathic arthritis with respect to the level of their activity and disease acceptance

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Grażyna Cepuch

2014-11-01

Full Text Available Objectives: Being diagnosed with a chronic disease, such as juvenile idiopathic arthritis (JIA, may adversely affect the activity of a young person. Additionally, the pain associated with the disease may cause difficulty accepting it. The aim was to assess the intensity of pain as well as the degree of activity and acceptance of the disease in young people aged 14–18 years with JIA. Material and methods : The study group consisted of youth of both genders suffering from JIA aged 14–18 years during remission of the disease. The study included 50 people and used the following: numerical rating scale (NRS 0–10, Cantril ladder, activity scale of own authorship (grouped items of the questionnaire form subscales of: tiredness, physical activity, social and intellectual activity and Acceptance of Illness Scale (AIS. Statistical analysis included the following tests: Mann-Whitney U test, Wilcoxon’s, χ2 test, Fisher’s, and Spearman rank correlation. The statistical significance level was set as p value below 0.05. Results : The majority of the respondents observed currently do not feel pain or feel pain of low intensity. The young people are physically, psychologically and socially active. The majority of patients accept their illness. The patients who suffer from pain on the NRS scale within 4–7 points do not adapt to living with the disease as do patients without pain or experiencing pain of low intensity. There was a strong correlation between acceptance of the disease at the time of the study determined by the Cantril ladder and AIS. Conclusions : 1. The pain suffered by patients with juvenile idiopathic arthritis (JIA has undoubtedly a negative impact on their activity and acceptance of the disease. 2. It seems to be crucial to recognize pain as a significant determinant of acceptance of the disease.

Efecto de una sola dosis de ácido zoledrónico en un caso de enfermedad de Paget ósea

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Melina Sabán

2010-10-01

Full Text Available La enfermedad de Paget es un trastorno crónico del remodelado óseo, caracterizado por un aumento de la resorción ósea producido por osteoclastos atípicos, seguido por un incremento acelerado de la formación ósea, lo que resulta en la formación de hueso en mosaico desorganizado. Un excelente marcador bioquímico para orientar el diagnóstico y seguimiento es la fosfatasa alcalina (FAL. Se presenta el caso de un paciente de 90 años, de sexo masculino, con diagnóstico de enfermedad de Paget. Se inicia tratamiento con pamidronato vía oral con respuesta parcial, por lo que se rota a pamidronato endovenoso. Disminuyen el dolor y la concentración plasmática de FAL, persistiendo con centellograma óseo patológico. Luego de varios años de tratamiento, con adecuado aporte de calcio y vitamina D, comienza nuevamente con dolor y valores elevados de FAL. Se decide iniciar tratamiento con ácido zoledrónico endovenoso 4 mg, única aplicación, obteniéndose remisión clínica y bioquímica desde hace cuatro años y mejoría de la imagen centellográfica. Este informe refiere la buena respuesta, sostenida en el tiempo, al tratamiento con única dosis de ácido zoledrónico en un paciente que presentó resistencia al pamidronato.

Anakinra as first-line disease-modifying therapy in systemic juvenile idiopathic arthritis: report of forty-six patients from an international multicenter series

NARCIS (Netherlands)

Nigrovic, Peter A.; Mannion, Melissa; Prince, Femke H. M.; Zeft, Andrew; Rabinovich, C. Egla; van Rossum, Marion A. J.; Cortis, Elisabetta; Pardeo, Manuela; Miettunen, Paivi M.; Janow, Ginger; Birmingham, James; Eggebeen, Aaron; Janssen, Erin; Shulman, Andrew I.; Son, Mary Beth; Hong, Sandy; Jones, Karla; Ilowite, Norman T.; Cron, Randy Q.; Higgins, Gloria C.

2011-01-01

To examine the safety and efficacy of the interleukin-1 (IL-1) receptor antagonist anakinra as first-line therapy for systemic juvenile idiopathic arthritis (JIA). Patients with systemic JIA receiving anakinra as part of initial disease-modifying antirheumatic drug (DMARD) therapy were identified

Exclusively endoscopic approach for juvenile angiofibroma in an adult – a case report

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Wojciech Gawęcki

2010-09-01

Full Text Available Aim: To demonstrate clinical, radiological, and diagnostic pitfalls of juvenile angiofibroma (JA in an adult.Study design: Retrospective analysis of a case report.Results: Juvenile angiofibroma in adults is a rare entity with only two cases reported in the literature, confirming thelow prevalence of the disease. We present a case of juvenile angiofibroma in an adult that preoperatively wassuspected to be a malignant disease. Effective treatment included surgical excision via an exclusively endoscopicapproach.Conclusions: Symptoms of JA in an adult may mimic a malignant process. However, in the case of unilateral epistaxis,rhinorrhea and nasal obstruction in an adult JA should be considered in the differential diagnosis.

Overlap of disease susceptibility loci for rheumatoid arthritis and juvenile idiopathic arthritis

Science.gov (United States)

Hinks, Anne; Eyre, Steve; Ke, Xiayi; Barton, Anne; Martin, Paul; Flynn, Edward; Packham, Jon; Worthington, Jane; Thomson, Wendy

2010-01-01

Background Genome-wide association studies (GWAS) have been extremely successful in the search for susceptibility risk factors for complex genetic autoimmune diseases. As more studies are published, evidence is emerging of considerable overlap of loci between these diseases. In juvenile idiopathic arthritis (JIA), another complex genetic autoimmune disease, the strategy of using information from autoimmune disease GWAS or candidate gene studies to help in the search for novel JIA susceptibility loci has been successful, with confirmed association with two genes, PTPN22 and IL2RA. Rheumatoid arthritis (RA) is an autoimmune disease that shares similar clinical and pathological features with JIA and, therefore, recently identified confirmed RA susceptibility loci are also excellent JIA candidate loci. Objective To determine the overlap of disease susceptibility loci for RA and JIA. Methods Fifteen single nucleotide polymorphisms (SNPs) at nine RA-associated loci were genotyped in Caucasian patients with JIA (n=1054) and controls (n=3531) and tested for association with JIA. Allele and genotype frequencies were compared between cases and controls using the genetic analysis software, PLINK. Results Two JIA susceptibility loci were identified, one of which was a novel JIA association (STAT4) and the second confirmed previously published associations of the TRAF1/C5 locus with JIA. Weak evidence of association of JIA with three additional loci (Chr6q23, KIF5A and PRKCQ) was also obtained, which warrants further investigation. Conclusion All these loci are good candidates in view of the known pathogenesis of JIA, as genes within these regions (TRAF1, STAT4, TNFAIP3, PRKCQ) are known to be involved in T-cell receptor signalling or activation pathways. PMID:19674979

Atherosclerosis in Juvenile Idiopathic Arthritis

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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.

Negative public perceptions of juvenile diabetics: applying attribution theory to understand the public's stigmatizing views.

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Vishwanath, Arun

2014-01-01

Despite a rise in the incidence of juvenile diabetes globally, little research has focused on public perceptions regarding its patients. The need to evaluate whether the public holds stigmatizing views is pressing when one considers the relatively young age of the patients of the disease. The current study extends the attribution theoretic framework to evaluate public stigma regarding juvenile diabetes. The findings suggest that a large percentage of individuals misattribute the causes of the disease and believe it is relatively rare and that its patients are personally responsible for contracting it. Individuals often utilize pejorative terms describing juvenile diabetes as a disease afflicting children who are lazy, unhealthy, fat, obese, lacking exercise, and having eating disorders.

Calcitonin treatment in osteoectasia with hyperphosphatasia (juvenile Paget's disease): radiographic changes after treatment

Energy Technology Data Exchange (ETDEWEB)

Tueysuez, B. [Div. of Genetics and Teratology, Univ. of Istanbul (Turkey); Serencebey, Istanbul (Turkey); Mercimek, S.; Uenguer, S. [Dept. of Paediatrics, Univ. of Istanbul (Turkey); Deniz, M. [Dept. of Paediatrics, Haseki Hospital, Istanbul (Turkey)

1999-11-01

Osteoectasia with hyperphosphatasia is a rare skeletal disorder, characterised by demineralisation and expansion of tubular bones and elevated serum alkaline phosphatase. We present a girl diagnosed as having osteoectasia with hyperphosphatasia who had swelling of phalanges of both hands and motor retardation. She was treated with synthetic human calcitonin. Clinical and radiological findings showed remarkable improvement after 2 years' treatment. (orig.)

Assessment of Microvascular Abnormalities by Nailfold Capillaroscopy in Juvenile Dermatomyositis After Medium- to Long-Term Followup.

Science.gov (United States)

Barth, Zoltan; Witczak, Birgit N; Flatø, Berit; Koller, Akos; Sjaastad, Ivar; Sanner, Helga

2018-05-01

In juvenile dermatomyositis (DM), microvascular abnormalities, measured by nailfold capillaroscopy (NFC), are common early in the disease course. We aimed to compare the presence of NFC abnormalities in patients with medium- to long-term juvenile DM with that of controls, and to explore associations between NFC abnormalities and disease activity and other disease characteristics. Fifty-eight juvenile DM patients with a median disease duration of 16.8 (range 2-38) years were clinically examined and compared with matched controls. By NFC, we assessed nailfold capillary density (NCD), giant capillaries, scleroderma, and neovascular pattern (defined as scleroderma active or late pattern). NFC was analyzed with researchers blinded to patient/control identity and disease characteristics. We measured disease activity and damage by validated tools, and patients were categorized as having active or inactive disease according to the Paediatric Rheumatology International Trials Organisation criteria. Compared to controls, patients had decreased NCD (mean ± SD 6.4 ± 2.1/mm versus 7.6 ± 0.8/mm; P = 0.001) and showed more abnormality in all other NFC parameters; 36% of patients versus 4% of controls had NCD <6/mm (P < 0.001). Giant capillaries, scleroderma, and neovascular pattern were found in 9%, 84%, and 41% of patients, respectively. Patients with active disease (n = 30) presented more frequently with neovascular pattern than patients with inactive disease (n = 28) (P = 0.041). Decreased NCD and neovascular pattern were associated with higher levels of disease activity and impaired muscle function. After medium- to long-term followup, juvenile DM patients had decreased NCD and, often, neovascular pattern; both were associated with higher levels of disease activity and impaired muscle function. This suggests that NFC can be a biomarker for disease activity in longstanding juvenile DM too. © 2017, American College of Rheumatology.

New developments in juvenile systemic and localized scleroderma.

Science.gov (United States)

Foeldvari, Ivan

2013-11-01

Juvenile localized scleroderma (jLS) and juvenile systemic sclerosis (jSS) are both orphan diseases, with jLS around 10 times more frequent than jSS. In recent years the time gap between the appearance of symptoms and diagnosis has become significantly shorter. This review focuses on the new classifications of jSS and jLS, and on the developments and adaptations of the outcome measures for certain organ involvements whereby progress has been made regarding pediatric patients. Copyright © 2013 Elsevier Inc. All rights reserved.

Integrative Analysis of Disease Signatures Shows Inflammation Disrupts Juvenile Experience-Dependent Cortical Plasticity

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Smith, Milo R.; Burman, Poromendro

2016-01-01

Throughout childhood and adolescence, periods of heightened neuroplasticity are critical for the development of healthy brain function and behavior. Given the high prevalence of neurodevelopmental disorders, such as autism, identifying disruptors of developmental plasticity represents an essential step for developing strategies for prevention and intervention. Applying a novel computational approach that systematically assessed connections between 436 transcriptional signatures of disease and multiple signatures of neuroplasticity, we identified inflammation as a common pathological process central to a diverse set of diseases predicted to dysregulate plasticity signatures. We tested the hypothesis that inflammation disrupts developmental cortical plasticity in vivo using the mouse ocular dominance model of experience-dependent plasticity in primary visual cortex. We found that the administration of systemic lipopolysaccharide suppressed plasticity during juvenile critical period with accompanying transcriptional changes in a particular set of molecular regulators within primary visual cortex. These findings suggest that inflammation may have unrecognized adverse consequences on the postnatal developmental trajectory and indicate that treating inflammation may reduce the burden of neurodevelopmental disorders. PMID:28101530

Whole-body MRI of juvenile spondyloarthritis: protocols and pictorial review of characteristic patterns

Energy Technology Data Exchange (ETDEWEB)

Aquino, Michael R. [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States); Tse, Shirley M.L.; Rachlis, Alisa C. [Hospital for Sick Children, Department of Rheumatology, Toronto (Canada); Gupta, Sumeet; Stimec, Jennifer [Hospital for Sick Children, Department of Radiology, Toronto (Canada)

2015-05-01

Spondyloarthritides are a group of inflammatory rheumatological diseases that cause arthritis with a predilection for spinal or sacroiliac involvement in addition to a high association with HLA-B27. Juvenile spondyloarthritis is distinct from adult spondyloarthritis and manifests more frequently as peripheral arthritis and enthesitis. Consequently juvenile spondyloarthritis is often referred to as enthesitis-related arthritis (ERA) subtype under the juvenile idiopathic arthritis (JIA) classification criteria. The American College of Rheumatology Treatment Recommendations for JIA, including ERA, are based on the following clinical parameters: current treatment, disease activity and the presence of poor prognostic features. The MRI features of juvenile spondyloarthritis include marrow edema, peri-enthesal soft-tissue swelling and edema, synovitis and joint or bursal fluid. Marrow edema is nonspecific and can be seen with other pathologies as well as in healthy subjects, and this is an important pitfall to consider. With further longitudinal study and validation, however, whole-body MRI with dedicated images of the more commonly affected areas such as the spine, sacroiliac joints, hips, knees, ankles and feet can serve as a more objective tool compared to clinical exam for early detection and monitoring of disease activity and ultimately direct therapeutic management. (orig.)

The Disease of the Italian Poet Giacomo Leopardi (1798-1837): A Case of Juvenile Ankylosing Spondylitis in the 19th Century?

Science.gov (United States)

Sganzerla, Erik Pietro; Riva, Michele Augusto

2017-06-01

Some authors sustained that the pessimistic thought of the Italian writer and philosopher Giacomo Leopardi (1798-1837) may be attributed to his unhappy life, characterized by several health problems. His philosophical theories appear as the result of depressive and melancholic state, related to his precarious health conditions, so limiting their intrinsic values. Several authors formulated various hypotheses on the diseases that Leopardi suffered from and postulated different theories on the cause of his early death. This article assumed that Leopardi may have been affected by juvenile ankylosing spondylitis, conditioning spinal deformities, relapsing-remitting uveitis, urinary tract and bowel tract problems, and acute arthritis. Chest deformity, as a complication of juvenile ankylosing spondylitis, may have caused progressive cardiorespiratory failure, worsened by recurrent bronchial and pulmonary complications, until his death caused by acute right ventricular heart failure. The acknowledgment of a physical cause of Leopardi's disease contributes to reevaluate his "cosmic pessimism" as an original expression of his thought, so leading a general revaluation of the figure of one of the most important European thinkers of the 19th century.

Elevated tissue transglutaminase antibodies in juvenile idiopathic arthritis children: Relation to neutrophil-to-lymphocyte ratio and disease activity

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Rasha E. Gheith

2017-10-01

Full Text Available Background: Subclinical gut inflammation is described in juvenile idiopathic arthritis (JIA, so has joint involvement been related to celiac disease (CD. The well-known involvement of tissue transglutaminase (tTG in the pathogenesis of CD stimulated progress in the field of autoimmune diseases. Aim of the work: To screen JIA children for tTG antibodies and to detect its relation to the neutrophil-lymphocyte ratio (NLR and disease activity. Patients and methods: The study included 44 JIA children with 44 matched controls. All subjects had no GIT symptoms suggestive of CD. Disease activity was assessed using the juvenile arthritis disease activity score in 27 joints (JADAS-27. The tTG antibodies (IgA and IgG were assessed. Results: The patients mean age was 12.5 ± 2.8 years and disease duration 5.01 ± 2.9 years; Female:Male 3.4:1. The mean JADAS-27 score was 12.6 ± 2.04. tTG antibodies were positive in 43.2% of the patients compared to 18.2% control (p = 0.01. Antibodies positivity was comparable according to gender and subtypes. The NLR in JIA children (1.62 ± 0.58 was significantly higher than in control (1.3 ± 0.5 (p = 0.006. Those with positive tTG antibodies had a significantly reduced body mass index (p = 0.02 and increased NLR (p = 0.02 compared to those with negative tTG. Only NLR and JADAS-27 would significantly predict antibodies positivity (p = 0.037 and p = 0.04, respectively. Conclusion: Increased tTG antibodies are frequent in JIA children raising the possibility of an associated subclinical CD. Markedly reduced BMI and increased NLR could forecast the presence of these antibodies. In addition to the JADAS-27, the NLR is a simple test that could predict this association and could be a useful biomarker.

[Brownish coloration of the left nipple in a 60-year-old woman].

Science.gov (United States)

Blum, A; Hartmann, K W; Rütten, A

2010-01-01

Pigmented Paget disease of the nipple is a rare disease. We report on a 60-year-old woman in whom this diagnosis was made based on clinical findings, dermoscopy, histology and immunohistology. Circumscribed pigmentation of the nipple includes a wide spectrum of benign and malignant differential diagnostic considerations. Dermoscopy can enhance the clinical diagnosis. Biopsies should always be performed on unclear pigmentation of the nipple in both women and men. On histology, rare melanocytic tumors must be differentiated from pigmented Paget disease and pigmented Bowen disease. Pagetoid intraepithelial cells of a melanocytic tumor are positive for S100, HMB45 or melan A, but not for cytokeratins. Intraepithelial tumor cells of Paget disease are positive for cytokeratin 7. The clear cells in Bowen diseases are usually not positive for cytokeratin 7 and are also negative for all melanocytic markers.

Feeding frequency affects stress, innate immunity and disease resistance of juvenile blunt snout bream Megalobrama amblycephala.

Science.gov (United States)

Li, Xiang-Fei; Tian, Hong-Yan; Zhang, Ding-Dong; Jiang, Guang-Zhen; Liu, Wen-Bin

2014-05-01

This study aimed to evaluate the effects of feeding frequency on stress, innate immunity and disease resistance of juvenile blunt snout bream Megalobrama amblycephala (average weight: 9.92 ± 0.06 g). Fish were randomly assigned to one of six feeding frequencies (1, 2, 3, 4, 5 and 6 times/day) following the same ration size for 8 weeks. After the feeding trial, fish were challenged by Aeromonas hydrophila and cumulative mortality was recorded for the next 10 days. Daily gain index of fish fed 3-5 times/day was significantly higher than that of the other groups. High feeding frequencies induced significantly elevated plasma levels of both cortisol and lactate. Fish fed 3-4 times/day exhibited relatively low liver catalase and glutathione peroxidase activities as well as malondialdehyde contents, but obtained significantly higher reduced glutathione levels and post-challenged haemato-immunological parameters (include blood leukocyte and erythrocyte counts as well as plasma lysozyme, alternative complement, acid phosphatase and myeloperoxidase activities) compared with that of the other groups. After challenge, the lowest mortality was observed in fish fed 4 times/day. It was significantly lower than that of fish fed 1-3 times/day, but exhibited no statistical difference with that of the other groups. In conclusion, both low and high feeding frequencies could cause oxidative stress of juvenile M. amblycephala, as might consequently lead to the depressed immunity and reduced resistance to A. hydrophila infection. The optimal feeding frequency to enhance growth and boost immunity of this species at juvenile stage is 4 times/day. Copyright © 2014 Elsevier Ltd. All rights reserved.

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.

[Child-juvenile prostitution: a systematic literature review].

Science.gov (United States)

Ribeiro, Moneda Oliveira; Dias, Aretuzza de Fátima

2009-06-01

The purpose of this study was to understand how infant-juvenile prostitution is being explained by researchers through an extensive bibliographical survey on national and international scientific sources. It was possible to access 20 references in full text form, which were analyzed according to the Content Analysis method. The analysis consisted in answering how infant-juvenile prostitution is represented by the author in relation to the concepts, causes, effects and solutions described in the references. It was found that the authors approached the subject as a way of survival, resulting from an unequal society, adult-centered and male chauvinist causing mental and physical diseases in children.

Paget’s disease of the male breast: a case report

OpenAIRE

Akita, Masayuki; Kusunoki, Nobuya; Nakajima, Takahiro; Takase, Shiro; Maekawa, Yoko; Kajimoto, Kazuyoshi; Ohno, Masakazu

2015-01-01

The patient was a 91-year-old man with change in nipple appearance, itching and redness, and a palpable breast mass. At presentation, mammary Paget’s disease (PD) was clinically suspected. Skin biopsy was performed and showed epidermis invaded by Paget cells, characterized by hyperchromatic nuclei and abundant pale-staining cytoplasm. Computed tomography and mammary ultrasonography confirmed the absence of an underlying invasive carcinoma, and the patient underwent right mastectomy and sentin...

Survival, growth performance and immune capacity of the juvenile lined seahorse Hippocampus erectus fed with rifampicin-treated copepods.

Science.gov (United States)

Lin, Tingting; Wang, Changbo; Liu, Xin; Gao, Fen; Xiao, Dongxue; Zhang, Dong; Zhu, Xialian

2017-06-19

Gastrointestinal disease is one of the most serious diseases in cultured seahorse juveniles. Treatment with antimicrobials of live food (i.e. copepods and Artemia) that is used to feed the juveniles may be a promising measure to alleviate the occurrence of gastrointestinal disease. However, relevant investigations are rare. In the present study, we first investigated the antimicrobial efficacies on bacteria within copepods that were treated with 4 antimicrobials, including 3 antibiotics (i.e. enrofloxacin hydrochloride, oxytetracycline and rifampicin [RFP]) that are approved for use in aquaculture and 1 disinfectant (i.e. povidone iodine). We then assessed the effects of copepods treated with the antimicrobial that had the best antimicrobial efficacy on survival, growth performance and immune capacity of juvenile lined seahorses Hippocampus erectus. The results showed that RFP had the best antimicrobial efficacy on both Pseudoalteromonas spp. and Vibrio spp., 2 dominant bacteria with potential pathogenicity within the copepods; the proper concentration of RFP was 6 mg l-1. Moreover, H. erectus juveniles fed with RFP-treated copepods demonstrated an improved survivorship and immune capacity and had a lower abundance of pathogenic bacteria within their gastrointestinal tracts compared to juveniles fed with untreated copepods. These results suggest that treating live food with RFP is a potential measure for reducing the incidence of gastrointestinal disease in seahorse juveniles.

Mithramycin In Paget's Disease

African Journals Online (AJOL)

1974-06-29

Jun 29, 1974 ... Mithramycin (kindly supplied by courtesy of. Pfizer Pharmaceuticals), was administered intravenously in a dose of 15 pg/kg body mass over 4 hours in 1 litre of '5% dextrose water solution. This dose was given daily as a course for 5 days. Further courses were given only if the patient failed to improve on the ...

Depression Risk in Young Adults With Juvenile- and Adult-Onset Lupus: Twelve Years of Followup.

Science.gov (United States)

Knight, Andrea M; Trupin, Laura; Katz, Patricia; Yelin, Edward; Lawson, Erica F

2018-03-01

To compare major depression risk among young adults with juvenile-onset and adult-onset systemic lupus erythematosus (SLE), and to determine demographic and health-related predictors of depression. Young adults with SLE ages 18-45 years (n = 546) in the Lupus Outcomes Study completed annual telephone surveys from 2002-2015, including assessment of depression using the Center for Epidemiologic Studies Depression Scale (CES-D), and self-report measures of sociodemographics and health characteristics. Juvenile-onset SLE was defined as age adult-onset SLE. Older age, lower educational attainment, and physical function, higher disease activity, and a history of smoking were associated with an increased depression risk. Juvenile-onset SLE patients had a higher risk of major depression across all educational groups. Young adults with SLE, particularly those with juvenile-onset disease, are at high risk for major depression, which is associated with increased disease activity, poorer physical functioning, and lower educational attainment. Early depression intervention in young adults with SLE has the potential to improve both medical and psychosocial outcomes. © 2017, American College of Rheumatology.

Dietary supplementation of yeast (Saccharomyces cerevisiae) improves growth, stress tolerance, and disease resistance in juvenile Nile tilapia (Oreochromis niloticus)

DEFF Research Database (Denmark)

Abass, David Attim; Obirikorang, Kwasi Adu; Campion, Benjamin Betey

2018-01-01

resistance in juvenile (body mass ~ 21 g) Nile tilapia (Oreochromis niloticus). Fish were randomly distributed in groups of 20 into 12 1-m³ hapas and fed isoenergetic (~ 17 kJ g⁻¹ gross energy) and isonitrogenous (~ 300 g kg⁻¹ crude protein) diets at 3% of their bulk weight daily. Specific growth rates were...... as an additive in Nile tilapia diets has beneficial impacts on growth, stress tolerance, and disease resistance...

Neuropathological Comparison of Adult Onset and Juvenile Huntington's Disease with Cerebellar Atrophy: A Report of a Father and Son.

Science.gov (United States)

Latimer, Caitlin S; Flanagan, Margaret E; Cimino, Patrick J; Jayadev, Suman; Davis, Marie; Hoffer, Zachary S; Montine, Thomas J; Gonzalez-Cuyar, Luis F; Bird, Thomas D; Keene, C Dirk

2017-01-01

Huntington's disease (HD) is an autosomal dominant neurodegenerative disease caused by a trinucleotide (CAG) repeat expansion in huntingtin (HTT) on chromosome 4. Anticipation can cause longer repeat expansions in children of HD patients. Juvenile Huntington's disease (JHD), defined as HD arising before age 20, accounts for 5-10% of HD cases, with cases arising in the first decade accounting for approximately 1%. Clinically, JHD differs from the predominately choreiform adult onset Huntington's disease (AOHD) with variable presentations, including symptoms such as myoclonus, seizures, Parkinsonism, and cognitive decline. The neuropathologic changes of AOHD are well characterized, but there are fewer reports that describe the neuropathology of JHD. Here we report a case of a six-year-old boy with paternally-inherited JHD caused by 169 CAG trinucleotide repeats who presented at age four with developmental delay, dysarthria, and seizures before dying at age 6. The boy's clinical presentation and neuropathological findings are directly compared to those of his father, who presented with AOHD and 54 repeats. A full autopsy was performed for the JHD case and a brain-only autopsy was performed for the AOHD case. Histochemically- and immunohistochemically-stained slides were prepared from formalin-fixed, paraffin-embedded tissue sections. Both cases had neuropathology corresponding to Vonsattel grade 3. The boy also had cerebellar atrophy with huntingtin-positive inclusions in the cerebellum, findings not present in the father. Autopsies of father and son provide a unique opportunity to compare and contrast the neuropathologic findings of juvenile and adult onset HD while also providing the first immunohistochemical evidence of cerebellar involvement in JHD. Additionally this is the first known report to include findings from peripheral tissue in a case of JHD.

Dislipidemia em pacientes com dermatomiosite juvenil

OpenAIRE

Katia Tomie Kozu

2012-01-01

OBJETIVO: Avaliar a presença de dislipidemia em pacientes com dermatomiosite juvenil (DMJ) e seus possíveis fatores de risco. MÉTODO: 25 pacientes com DMJ foram comparados a 25 controles de acordo com dados demográficos, composição corporal, perfil lipídico, glicêmico, autoanticorpos e enzimas musculares. Foram avaliados os instrumentos de atividade da DMJ: Disease Activity Score (DAS), Childhood Myositis Assessment Scale (CMAS), Manual Muscle Testing (MMT), Myositis Disease Activity Assessme...

Nailfold capillaroscopy in children and adolescents with rheumatic diseases.

Science.gov (United States)

Piotto, Daniela Gerent Petry; Len, Cláudio Arnaldo; Hilário, Maria Odete Esteves; Terreri, Maria Teresa Ramos Ascensão

2012-10-01

To assess nailfold capillaroscopy in children and adolescents with autoimmune rheumatic diseases (juvenile idiopathic arthritis, systemic lupus erythematosus, juvenile dermatomyositis, scleroderma and mixed connective tissue disease) and relate it to clinical and laboratory findings and disease activity. Cross-sectional study assessing 147 patients by use of nailfold capillaroscopy as follows: 60 with juvenile idiopathic arthritis; 30 with systemic lupus erythematosus; 30 with juvenile dermatomyositis; 20 with localized scleroderma; four with systemic sclerosis; and three with mixed connective tissue disease. Clinical and laboratory tests and nailfold capillaroscopy were performed in all patients. The nailfold capillaroscopy was performed with an optical microscope (at 10- and 16-time magnifications) by the same observer. Most patients (76.2%) had normal nailfold capillaroscopy. The major changes in nailfold capillaroscopy, characterizing the scleroderma pattern, were observed in patients with juvenile dermatomyositis, systemic scleroderma and mixed connective tissue disease. There was no association between nailfold capillaroscopy and disease activity in patients with juvenile idiopathic arthritis, systemic lupus erythematosus and localized scleroderma. Disease activity and capillaroscopy were associated in patients with juvenile dermatomyositis. Nailfold capillaroscopy is a useful method to diagnose autoimmune rheumatic diseases and monitor disease activity.

Bacteriological study of juvenile periodontitis in China.

Science.gov (United States)

Han, N M; Xiao, X R; Zhang, L S; Ri, X Q; Zhang, J Z; Tong, Y H; Yang, M R; Xiao, Z R

1991-09-01

The predominant cultivable bacteria associated with juvenile periodontitis (JP) in China were studied for the first time. Subgingival plaque samples were taken on paper points from 23 diseased sites in 15 JP patients and from 7 healthy sites in 7 control subjects. Serially diluted plaque samples were plated on nonselective blood agar and on MGB agar, a selective medium for the isolation of Actinobacillus actinomycetemcomitans. Fifteen or more isolated colonies from each sample (in sequence without selection) were purified for identification. The results indicated that the microflora in healthy sulci of the 7 control subjects was significantly different from that in diseased sites of JP patients. The predominant species in healthy sulci were Streptococcus spp. and Capnocytophaga gingivalis. In JP patients, Eubacterium sp. was found in significantly higher frequency and proportion. Actinobacillus actinomycetemcomitans was not detected in any samples. It appears that this species is not associated with juvenile periodontitis in China.

Results of surgical treatment for juvenile myasthenia gravis.

Science.gov (United States)

Vázquez-Roque, F J; Hernández-Oliver, M O; Medrano Plana, Y; Castillo Vitlloch, A; Fuentes Herrera, L; Rivero-Valerón, D

2017-04-01

Radical or extended thymectomy is an effective treatment for myasthenia gravis in the adult population. There are few reports to demonstrate the effectiveness of this treatment in patients with juvenile myasthenia gravis. The main objective of this study was to show that extended transsternal thymectomy is a valid option for treating this disease in paediatric patients. Twenty-three patients with juvenile myasthenia gravis underwent this surgical treatment in the period between April 2003 and April 2014; mean age was 12.13 years and the sample was predominantly female. The main indication for surgery, in 22 patients, was the generalised form of the disease (Osserman stage II) together with no response to 6 months of medical treatment. The histological diagnosis was thymic hyperplasia in 22 patients and thymoma in one patient. There were no deaths and no major complications in the postoperative period. After a mean follow-up period of 58.87 months, 22 patients are taking no medication or need less medication to manage myasthenic symptoms. Extended (radical) transsternal thymectomy is a safe and effective surgical treatment for juvenile myasthenia gravis. Copyright © 2015 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.

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 ...

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

Science.gov (United States)

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

2018-03-01

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

Germline mutation of CBL is associated with moyamoya disease in a child with juvenile myelomonocytic leukemia and Noonan syndrome-like disorder.

Science.gov (United States)

Hyakuna, Nobuyuki; Muramatsu, Hideki; Higa, Takeshi; Chinen, Yasutsugu; Wang, Xinan; Kojima, Seiji

2015-03-01

Germline mutations in CBL have been identified in patients with Noonan syndrome-like phenotypes, while juvenile myelomonocytic leukemia (JMML) harbors duplication of a germline CBL, resulting in acquired isodisomy. The association between moyamoya disease and Noonan syndrome carrying a PTPN11 mutation has recently been reported. We present a patient with JMML who developed moyamoya disease and neovascular glaucoma. Our patient exhibited a Noonan syndrome-like phenotype. Genetic analysis revealed acquired isodisomy and a germline heterozygous mutation in CBL. This is a rare case of CBL mutation associated with moyamoya disease. Prolonged RAS pathway signaling may cause disruption of cerebrovascular development. © 2014 Wiley Periodicals, Inc.

What Is Juvenile Arthritis?

Science.gov (United States)

... Initiative Breadcrumb Home Health Topics English Español Juvenile Arthritis Basics In-Depth Download Download EPUB Download PDF What is it? Points To Remember About Juvenile Arthritis Juvenile arthritis is the term used to describe ...

The myositis autoantibody phenotypes of the juvenile idiopathic inflammatory myopathies.

Science.gov (United States)

Rider, Lisa G; Shah, Mona; Mamyrova, Gulnara; Huber, Adam M; Rice, Madeline Murguia; Targoff, Ira N; Miller, Frederick W

2013-07-01

The juvenile idiopathic inflammatory myopathies (JIIM) are systemic autoimmune diseases characterized by skeletal muscle weakness, characteristic rashes, and other systemic features. In follow-up to our study defining the major clinical subgroup phenotypes of JIIM, we compared demographics, clinical features, laboratory measures, and outcomes among myositis-specific autoantibody (MSA) subgroups, as well as with published data on adult idiopathic inflammatory myopathy patients enrolled in a separate natural history study. In the present study, of 430 patients enrolled in a nationwide registry study who had serum tested for myositis autoantibodies, 374 had either a single specific MSA (n = 253) or no identified MSA (n = 121) and were the subject of the present report. Following univariate analysis, we used random forest classification and exact logistic regression modeling to compare autoantibody subgroups. Anti-p155/140 autoantibodies were the most frequent subgroup, present in 32% of patients with juvenile dermatomyositis (JDM) or overlap myositis with JDM, followed by anti-MJ autoantibodies, which were seen in 20% of JIIM patients, primarily in JDM. Other MSAs, including anti-synthetase, anti-signal recognition particle (SRP), and anti-Mi-2, were present in only 10% of JIIM patients. Features that characterized the anti-p155/140 autoantibody subgroup included Gottron papules, malar rash, "shawl-sign" rash, photosensitivity, cuticular overgrowth, lowest creatine kinase (CK) levels, and a predominantly chronic illness course. The features that differed for patients with anti-MJ antibodies included muscle cramps, dysphonia, intermediate CK levels, a high frequency of hospitalization, and a monocyclic disease course. Patients with anti-synthetase antibodies had higher frequencies of interstitial lung disease, arthralgia, and "mechanic's hands," and had an older age at diagnosis. The anti-SRP group, which had exclusively juvenile polymyositis, was characterized by high

Fatores de risco associados à calcinose na dermatomiosite juvenil Risk factors associated with calcinosis of juvenile dermatomyositis

Directory of Open Access Journals (Sweden)

Adriana M. E. Sallum

2008-02-01

calcinosis in children and adolescents with juvenile dermatomyositis. METHODS: A review was carried out of the medical records of 54 patients with juvenile dermatomyositis. Data were collected on demographic characteristics, clinical features: muscle strength (stages I to V of the Medical Research Council scale, pulmonary involvement (restrictive pulmonary disease with presence or absence of anti-Jo1 antibodies, gastrointestinal problems (gastroesophageal reflux and/or heart disease (pericarditis and/or myocarditis; laboratory tests: elevated muscle enzyme levels in serum (creatine phosphokinase, aspartate aminotransferase, alanine aminotransferase and/or lactate dehydrogenase; and on the treatments given: corticoid therapy in isolation or associated with hydroxychloroquine and/or immunosuppressants. The patients were divided into two groups, depending on presence or absence of calcinosis and data were evaluated by both univariate and multivariate analyses. RESULTS: Calcinosis was identified in 23 (43% patients, and in six (26% patients it had emerged prior to diagnosis while in 17 (74% it was post diagnosis. The univariate analysis revealed that cardiac (p = 0.01 and pulmonary (p = 0.02 involvement and the need for one or more immunosuppressor (methotrexate, cyclosporine A and/or pulse therapy with intravenous cyclophosphamide to treat juvenile dermatomyositis (p = 0.03 were all associated with an increased incidence of calcinosis. The multivariate analysis then demonstrated that only cardiac involvement (OR = 15.56; 95%CI 1.59-152.2 and the use of one or more immunosuppressor (OR = 4.01; 95%CI 1.08-14.87 were independently associated with the presence of calcinosis. CONCLUSIONS: Calcinosis was a frequent development among these juvenile dermatomyositis cases, generally emerging as the disease progressed. Calcinosis was associated with the more severe cases that also had cardiac involvement and where immunosuppressors had to be included in the treatment.

Juvenile-specific cathepsin proteases in Fasciola spp.: their characteristics and vaccine efficacies.

Science.gov (United States)

Meemon, Krai; Sobhon, Prasert

2015-08-01

Fasciolosis, caused by Fasciola hepatica and Fasciola gigantica, is one of the most neglected tropical zoonotic diseases. One sustainable control strategy against these infections is the employment of vaccines that target proteins essential for parasites' invasion and nutrition acquiring processes. Cathepsin proteases are the most abundantly expressed proteins in Fasciola spp. that have been tested successfully as vaccines against fasciolosis in experimental as well as large animals because of their important roles in digestion of nutrients, invasion, and migration. Specifically, juvenile-specific cathepsin proteases are the more effective vaccines because they could block the invasion and migration of juvenile parasites whose immune evasion mechanism has not yet been fully developed. Moreover, because of high sequence similarity and identity of cathepsins from juveniles with those of adults, the vaccines can attack both the juvenile and adult stages. In this article, the characteristics and vaccine potentials of juvenile-specific cathepsins, i.e., cathepsins L and B, of Fasciola spp. were reviewed.

Juvenile idiopathic arthritis-associated uveitis

OpenAIRE

Clarke, Sarah; Sen, Ethan; Ramanan, Athimalaipet

2016-01-01

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

Safety and efficacy of meningococcal c vaccination in juvenile idiopathic arthritis

NARCIS (Netherlands)

Zonneveld-Huijssoon, Evelien; Ronaghy, Arash; van Rossum, Marion A. J.; Rijkers, Ger T.; van der Klis, Fiona R. M.; Sanders, Elisabeth A. M.; Vermeer-de Bondt, Patricia E.; Hoes, Arno W.; van der Net, Jan Jaap; Engels, Carla; Kuis, Wietse; Prakken, Berent J.; van Tol, Maarten J. D.; Wulffraat, Nico M.

2007-01-01

To determine whether vaccinations aggravate the course of autoimmune diseases such as juvenile idiopathic arthritis (JIA) and whether the immune response to vaccinations may be hampered by immunosuppressive therapy for the underlying disease. In this multicenter cohort study, 234 patients with JIA

Safety of measles, mumps and rubella vaccination in juvenile idiopathic arthritis

NARCIS (Netherlands)

Heijstek, Marloes W; Pileggi, Gecilmara C S; Zonneveld-Huijssoon, Evelien; Armbrust, Wineke; Hoppenreijs, Esther P A H; Uiterwaal, Cuno S P M; Kuis, Wietse; Wulffraat, Nico M

2007-01-01

Objective: To assess the effect of measles, mumps and rubella (MMR) vaccination on disease activity in children with juvenile idiopathic arthritis (JIA). Methods: A retrospective observational multicentre cohort study was performed in 314 patients with JIA, born between 1989 and 1996. Disease

Juvenile Firesetting.

Science.gov (United States)

Peters, Brittany; Freeman, Bradley

2016-01-01

Juvenile firesetting is a significant cause of morbidity and mortality in the United States. Male gender, substance use, history of maltreatment, interest in fire, and psychiatric illness are commonly reported risk factors. Interventions that have been shown to be effective in juveniles who set fires include cognitive behavior therapy and educational interventions, whereas satiation has not been shown to be an effective intervention. Forensic assessments can assist the legal community in adjudicating youth with effective interventions. Future studies should focus on consistent assessment and outcome measures to create more evidence for directing evaluation and treatment of juvenile firesetters. Copyright © 2016 Elsevier Inc. All rights reserved.

Exercise training reverses skeletal muscle atrophy in an experimental model of VCP disease.

Directory of Open Access Journals (Sweden)

Angèle Nalbandian

Full Text Available The therapeutic effects of exercise resistance and endurance training in the alleviation of muscle hypertrophy/atrophy should be considered in the management of patients with advanced neuromuscular diseases. Patients with progressive neuromuscular diseases often experience muscle weakness, which negatively impact independence and quality of life levels. Mutations in the valosin containing protein (VCP gene lead to Inclusion body myopathy associated with Paget's disease of bone and frontotemporal dementia (IBMPFD and more recently affect 2% of amyotrophic lateral sclerosis (ALS-diagnosed cases.The present investigation was undertaken to examine the effects of uphill and downhill exercise training on muscle histopathology and the autophagy cascade in an experimental VCP mouse model carrying the R155H mutation. Progressive uphill exercise in VCP(R155H/+ mice revealed significant improvement in muscle strength and performance by grip strength and Rotarod analyses when compared to the sedentary mice. In contrast, mice exercised to run downhill did not show any significant improvement. Histologically, the uphill exercised VCP(R155H/+ mice displayed an improvement in muscle atrophy, and decreased expression levels of ubiquitin, P62/SQSTM1, LC3I/II, and TDP-43 autophagy markers, suggesting an alleviation of disease-induced myopathy phenotypes. There was also an improvement in the Paget-like phenotype.Collectively, our data highlights that uphill exercise training in VCP(R155H/+ mice did not have any detrimental value to the function of muscle, and may offer effective therapeutic options for patients with VCP-associated diseases.

Non-HLA gene polymorphisms in juvenile idiopathic arthritis

DEFF Research Database (Denmark)

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

2017-01-01

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

Imaging The Complications Of Paget's Disease Of Bones In A ...

African Journals Online (AJOL)

The skull and chest radiographs, CT scan and echocardiography with their findings are highlighted so as to increase awareness and index of suspicion in physicians to this relatively rare bone disease. Classical features were illustrated on plain skull radiograph and CT Scan. Echocardiography revealed left ventricular ...

Juvenile Animal Testing: Assessing Need and Use in the Drug Product Label.

Science.gov (United States)

Baldrick, Paul

2018-01-01

Juvenile animal testing has become an established part of drug development to support safe clinical use in the human pediatric population and for eventual drug product label use. A review of European Paediatric Investigation Plan decisions showed that from 2007 to mid-2017, 229 drugs had juvenile animal work requested, almost exclusively incorporating general toxicology study designs, in rat (57.5%), dog (8%), mouse (4.5%), monkey (4%), pig (2%), sheep (1%), rabbit (1%), hamster (0.5%), and species not specified (21.5%). A range of therapeutic areas were found, but the most common areas were infectious diseases (15%), endocrinology (13.5%), oncology (13%), neurology (11%), and cardiovascular diseases (10%). Examination of major clinical indications within these therapeutic areas showed some level of consistency in the species of choice for testing and the pediatric age that required support. Examination of juvenile animal study findings presented in product labels raises questions around how useful the data are to allow prescribing the drug to a child. It is hopeful that the new ICH S11 guideline "Nonclinical Safety Testing in Support of Development of Pediatric Medicines" currently in preparation will aid drug developers in clarifying the need for juvenile animal studies as well as in promoting a move away from toxicology studies with a conventional design. This would permit more focused testing to examine identified areas of toxicity or safety concerns and clarify the presentation/interpretation of juvenile animal study findings for proper risk assessment by a drug prescriber.

Infectious Hematopoietic Necrosis Virus Transmission and Disease among Juvenile Chinook Salmon Exposed in Culture Compared to Environmentally Relevant Conditions

Directory of Open Access Journals (Sweden)

J. Scott Foott

2006-02-01

Full Text Available The dynamics of IHNV infection and disease were followed in a juvenile Chinook salmon population both during hatchery rearing and for two weeks post-release. Cumulative weekly mortality increased from 0.03%–3.5% as the prevalence of viral infection increased from 2%–22% over the same four-week period. The majority of the infected salmon was asymptomatic. Salmon demonstrating clinical signs of infection shed 1000 pfu mL-1 of virus into the water during a 1 min observation period and had a mean concentration of 106 pfu mL-1 in their mucus. The high virus concentration detected in mucus suggests that it could act as an avenue of transmission in high density situations where dominance behavior results in nipping. Infected smolts that had migrated 295 km down river were collected at least two weeks after their release. The majority of the virus positive smolts was asymptomatic. A series of transmission experiments was conducted using oral application of the virus to simulate nipping, brief low dose waterborne challenges, and cohabitation with different ratios of infected to naïve fish. These studies showed that asymptomatic infections will occur when a salmon is exposed for as little as 1 min to >102 pfu mL-1, yet progression to clinical disease is infrequent unless the challenge dose is >104 pfu mL-1. Asymptomatic infections were detected up to 39 d post-challenge. No virus was detected by tissue culture in natural Chinook juveniles cohabitated with experimentally IHNV-infected hatchery Chinook at ratios of 1:1, 1:10, and 1:20 for either 5 min or 24 h. Horizontal transmission of the Sacramento River strain of IHNV from infected juvenile hatchery fish to wild cohorts would appear to be a low ecological risk. The study results demonstrate key differences between IHNV infections as present in a hatchery and the natural environment. These differences should be considered during risk assessments of the impact of IHNV infections on wild salmon and

Angiofibroma juvenil nasofaríngeo Juvenile nasopharyngeal angiofibroma

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Juan Gualberto Lescaille Torres

2012-06-01

Full Text Available Se presenta el caso de un angiofibroma juvenil nasofaríngeo, en un paciente del consultorio médico No. 9, que pertenece al Policlínico Integral Docente "Carlos Manuel Portuondo" de Marianao. Se reconoce la importancia de realizar una historia clínica detallada, así como un minucioso examen físico, por el médico de familia y el otorrinolaringólogo del área de salud, que incluyó la rinoscopia posterior, para poder llegar al diagnóstico de esa patología, y realizar la extirpación precoz del angiofibroma, mediante el proceder quirúrgico. Se concluyó que el diagnóstico clínico se correspondió con el histopatológico, y que la conducta quirúrgica temprana es resolutiva en la afección.It is presented a case of juvenile nasopharyngeal angiofibroma in a patient with this pathology, from the clicial practice No. 9, in Comprehensive Teaching Polyclinic "Carlos Manuel Portuondo" in Marianao. It recognizes the importance of a thorough clinical history and careful physical examination by the family physician and the otolaryngologist in this health area, including a posterior rhinoscopy, to diagnose this disease and to achieve early removal of the angiofibroma, by a surgical procedure. It was concluded that the clinical diagnosis corresponded to the histopathological diagnosis, and that early surgical treatment is resolute in this condition.

Brief Report: Association of Myositis Autoantibodies, Clinical Features, and Environmental Exposures at Illness Onset With Disease Course in Juvenile Myositis.

Science.gov (United States)

Habers, G Esther A; Huber, Adam M; Mamyrova, Gulnara; Targoff, Ira N; O'Hanlon, Terrance P; Adams, Sharon; Pandey, Janardan P; Boonacker, Chantal; van Brussel, Marco; Miller, Frederick W; van Royen-Kerkhof, Annet; Rider, Lisa G

2016-03-01

To identify early factors associated with disease course in patients with juvenile idiopathic inflammatory myopathies (IIMs). Univariable and multivariable multinomial logistic regression analyses were performed in a large juvenile IIM registry (n = 365) and included demographic characteristics, early clinical features, serum muscle enzyme levels, myositis autoantibodies, environmental exposures, and immunogenetic polymorphisms. Multivariable associations with chronic or polycyclic courses compared to a monocyclic course included myositis-specific autoantibodies (multinomial odds ratio [OR] 4.2 and 2.8, respectively), myositis-associated autoantibodies (multinomial OR 4.8 and 3.5), and a documented infection within 6 months of illness onset (multinomial OR 2.5 and 4.7). A higher overall clinical symptom score at diagnosis was associated with chronic or monocyclic courses compared to a polycyclic course. Furthermore, severe illness onset was associated with a chronic course compared to monocyclic or polycyclic courses (multinomial OR 2.1 and 2.6, respectively), while anti-p155/140 autoantibodies were associated with chronic or polycyclic courses compared to a monocyclic course (multinomial OR 3.9 and 2.3, respectively). Additional univariable associations of a chronic course compared to a monocyclic course included photosensitivity, V-sign or shawl sign rashes, and cuticular overgrowth (OR 2.2-3.2). The mean ultraviolet index and highest ultraviolet index in the month before diagnosis were associated with a chronic course compared to a polycyclic course in boys (OR 1.5 and 1.3), while residing in the Northwest was less frequently associated with a chronic course (OR 0.2). Our findings indicate that myositis autoantibodies, in particular anti-p155/140, and a number of early clinical features and environmental exposures are associated with a chronic course in patients with juvenile IIM. These findings suggest that early factors, which are associated with poorer

Comparação entre o Disease Activity Score-28 e o Juvenile Arthritis Disease Activity Score na artrite idiopática juvenil

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Renata Campos Capela

2015-02-01

Full Text Available Introdução A avaliação de atividade da artrite reumatoide e da artrite idiopática juvenil é feita por meio de instrumentos distintos, respectivamente pelo DAS-28 e pelo JADAS. Objetivo Comparar o DAS-28 e o JADAS com a pontuação de 71, 27 e 10 articulações, na artrite idiopática juvenil. Método Foram avaliadas 178 visitas em oito pacientes com artrite idiopática juvenil, participantes de um ensaio clínico controlado de fase III, testando eficácia e segurança do abatacepte. Pontuaram-se as articulações ativas e limitadas, a avaliação global pelo médico e pelos pais em escala analógica visual de 0-10 cm e a velocidade de hemossedimentação convertida em escala de 0-10, em todas as visitas. A comparação entre os índices de atividade entre diferentes observações foi por Anova ou modelo ajustado Gama. As observações pareadas entre o DAS-28 e o JADAS 71, 27 e 10, respectivamente, foram analisadas por meio de regressão linear. Resultados Houve diferença significativa entre as medidas individuais, exceto a VHS, nos primeiros quatro meses de tratamento com biológico, quando cinco entre os oito pacientes atingiram a resposta ACR-Pedi 30, com melhora. Os índices DAS-28, JADAS 71, 27 e 10 também apresentaram diferença relevante durante o período de observação. O ajustamento por meio de regressão linear entre o DAS-28 e o JADAS resultou em fórmulas matemáticas para conversão: [DAS-28 = 0,0709 (JADAS 71 + 1,267] (R2 = 0,49; [DAS-28 = 0,084 (JADAS 27 + 1,7404] (R2 = 0,47 e [DAS-28 = 0,1129 (JADAS-10 + 1,5748] (R2 = 0,50. Conclusão A conversão da pontuação do DAS-28 e do JADAS 71, 27 e 10 por esse modelo matemático permitiria a aplicação equivalente de ambos em adolescentes com artrite.

Cytokine profiles in peripheral blood and whole blood cell cultures associated with aggressive periodontitis, juvenile idiopathic arthritis, and rheumatoid arthritis

DEFF Research Database (Denmark)

Poulsen, Anne Havemose; Sørensen, Lars Korsbaek; Stoltze, Kaj

2005-01-01

Cytokines play a key role in the pathogenesis of inflammatory diseases. An obvious question is whether patients with aggressive periodontitis, juvenile idiopathic arthritis, or rheumatoid arthritis share blood cytokine profiles distinguishing them from individuals free of disease.......Cytokines play a key role in the pathogenesis of inflammatory diseases. An obvious question is whether patients with aggressive periodontitis, juvenile idiopathic arthritis, or rheumatoid arthritis share blood cytokine profiles distinguishing them from individuals free of disease....

Uveitis and Juvenile Psoriatic Arthritis or Psoriasis.

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Salek, Sherveen S; Pradeep, Archana; Guly, Catherine; Ramanan, Athimalaipet V; Rosenbaum, James T

2018-01-01

To describe the phenotype of the uveitis that accompanies juvenile psoriatic arthritis or psoriasis. Observational case series. Setting: Two university-based referral clinics: 1 in England, 1 in the United States. Five children with uveitis and psoriatic arthritis and 1 with uveitis and psoriasis Observational Procedure: Retrospective chart review. Demographics of subjects such as age and sex; description of ocular and joint disease; surgical and other complications; medical treatment. Five of the 6 children in this series had the onset of disease at or before age 6 (P = .0008 compared to expected age of onset for psoriatic arthritis in childhood). All children in this series had an inadequate response to topical corticosteroids. Most of the children were treated with systemic corticosteroids for many months, yet all of them went on to require methotrexate. Therapy with systemic methotrexate did not suffice, as all the patients also required some form of biologic therapy. Five of 6 had surgeries such as vitrectomy, cataract extraction, or a procedure for glaucoma control. The observations suggest that the uveitis that accompanies juvenile psoriatic arthritis might be a distinct disease that is particularly severe when its onset affects children aged 6 years or younger. Copyright © 2017 Elsevier Inc. All rights reserved.

Juvenile Court Statistics - 1972.

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Office of Youth Development (DHEW), Washington, DC.

This report is a statistical study of juvenile court cases in 1972. The data demonstrates how the court is frequently utilized in dealing with juvenile delinquency by the police as well as by other community agencies and parents. Excluded from this report are the ordinary traffic cases handled by juvenile court. The data indicate that: (1) in…

Juvenile Court Statistics, 1974.

Science.gov (United States)

Corbett, Jacqueline; Vereb, Thomas S.

This report presents information on juvenile court processing of youth in the U.S. during 1974. It is based on data gathered under the National Juvenile Court Statistical Reporting System. Findings can be summarized as follows: (1) 1,252,700 juvenile delinquency cases, excluding traffic offenses, were handled by courts in the U.S. in 1974; (2) the…

Familial juvenile hyperuricemic nephropathy : report on a new mutation and a pregnancy

NARCIS (Netherlands)

Lhotta, Karl; Gehringer, A; Jennings, P; Kronenberg, F.; Brezinka, C; Andersone, I; Strazdins, V

BACKGROUND: Familial juvenile hyperuricemic nephropathy (FJHN) is a rare autosomal dominant disease caused by mutations in the uromodulin gene (UMOD) and leading to gout, tubulointerstitial nephropathy and end-stage renal disease. CASE REPORTS AND RESULTS: A Latvian family suffering from FJHN is

Advances in the treatment of polyarticular juvenile idiopathic arthritis

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Webb, Kate; Wedderburn, Lucy R.

2015-01-01

Purpose of review To review recent advances in the management strategies of polyarticular course juvenile idiopathic arthritis (JIA) and identify unanswered questions and avenues for further research. Recent findings There is evidence for an early, aggressive, treat-to-target approach for polyarticular JIA. Clinical disease activity criteria have been recently defined and validated, including criteria for inactive disease and the juvenile arthritis disease activity score (JADAS). There is a need for evidence-based, defined disease targets and biomarkers for prediction of response, including targets for remission induction, and guidelines on drug withdrawal. Recent treatment consensus plans and guidelines are discussed and compared, including the 2015 NHS England clinical policy statement, the 2014 Childhood Arthritis and Rheumatology Research Alliance (CARRA) treatment plans and the 2011 American College of Rheumatology (ACR) guidelines. Evidence for new agents such as tocilizumab, rituximab, golimumab, ustekinumab, certolizumab and tofacitinib is promising: the recent clinical trials are summarized here. Stratification of individual patient treatment remains a goal, and predictive biomarkers have been shown to predict success in the withdrawal of methotrexate therapy. Summary There are promising advances in the treatment approaches, disease activity criteria, clinical guidelines, pharmaceutical choices and individually stratified therapy choices for polyarticular JIA. PMID:26147756

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

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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.

Juvenile Justice in Mexico

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Martha Frías Armenta

2014-08-01

Full Text Available The first tribunal in Mexico was established in the central state of San Luis Potosi in 1926. The Law Regarding Social Prevention and Juvenile Delinquency for the Federal District and Mexican territories was promulgated in 1928. In 2005, Article 18 of the Mexican Constitution was modified to establish a comprehensive system (“Sistema Integral de justicia” in Spanish of justice for juveniles between 12 and 18 years old who had committed a crime punishable under criminal law. Its objective was to guarantee juveniles all the due process rights established for adults, in addition to the special ones recognized for minors. The constitutional reform also provides a framework that includes special tribunals as well as alternative justice options for juveniles. With these reforms, institutionalization of minors was to be considered an extreme measure applicable only to felonies and to juveniles older than 14. In 2006, all states within the Mexican federation enacted the “Law of justice for adolescents”. This system, at both the federal and state levels, formalizes a new global paradigm with regard to the triangular relationship between children, the State and the Law. It recognizes that children are also bearers of the inherent human rights recognized for all individuals, instead of simply objects in need of protection. However, despite formally aligning Mexican juvenile justice law with the Convention on the Rights of the Child (CRC, issues of actual substantive rights remained and new ones have appeared. For example, juveniles younger than 14 who have not committed a felony are released from institutions without any rehabilitation or treatment options, and alternative forms of justice were included without evaluating their possibilities of application or their conditions for success. In addition, the economic status of most juvenile detainees continues to be one of the most important determining factors in the administration of justice

How common is clinically inactive disease in a prospective cohort of patients with juvenile idiopathic arthritis? The importance of definition.

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Shoop-Worrall, Stephanie J W; Verstappen, Suzanne M M; Baildam, Eileen; Chieng, Alice; Davidson, Joyce; Foster, Helen; Ioannou, Yiannis; McErlane, Flora; Wedderburn, Lucy R; Thomson, Wendy; Hyrich, Kimme L

2017-08-01

Many criteria for clinically inactive disease (CID) and minimal disease activity (MDA) have been proposed for juvenile idiopathic arthritis (JIA). It is not known to what degree each of these criteria overlap within a single patient cohort. This study aimed to compare the frequency of MDA and CID across different criteria in a cohort of children with JIA at 1 year following presentation. The Childhood Arthritis Prospective Study recruits children at initial presentation to paediatric or adolescent rheumatology in seven UK centres. Children recruited between October 2001 and December 2013 were included. The proportions of children with CID and MDA at 1 year were calculated using four investigator-defined and eight published composite criteria. Missing data were accounted for using multiple imputation under different assumptions. In a cohort of 1415 children and adolescents, 67% patients had no active joints at 1 year. Between 48% and 61% achieved MDA and between 25% and 38% achieved CID using published criteria. Overlap between criteria varied. Of 922 patients in MDA by either the original composite criteria, Juvenile Arthritis Disease Activity Score (JADAS) or clinical JADAS cut-offs, 68% were classified as in MDA by all 3 criteria. Similarly, 44% of 633 children with CID defined by either Wallace's preliminary criteria or the JADAS cut-off were in CID according to both criteria. In a large JIA prospective inception cohort, a majority of patients have evidence of persistent disease activity after 1 year. Published criteria to capture MDA and CID do not always identify the same groups of patients. This has significant implications when defining and applying treat-to-target strategies. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

A case report: a young waiter with Paget-Schroetter syndrome.

Science.gov (United States)

Drakos, Nicholas; Gausche-Hill, Marianne

2013-03-01

Paget-Schroetter syndrome (PSS) is a rare presentation of primary axillary subclavian vein thrombosis that classically occurs in young men with a degree of underlying thoracic outlet syndrome after a period of upper extremity exertion. The primary complication of PSS is post-thrombotic syndrome, a result of chronic venous hypertension. To educate Emergency Physicians on this condition to potentiate timely diagnosis and appropriate disposition. A 29-year-old right-handed restaurant waiter presented with 3 days of non-painful, gradual-onset right upper extremity swelling with normal vital signs. The patient's history was otherwise notable for subjective fevers and a right forearm abrasion. Upon examination, the right upper extremity was neurovascularly intact and remarkable for uniform edema and erythema extending distally from the level of the mid-humerus. The primary differential diagnoses were deep venous thrombosis (DVT) vs. soft tissue infection. Venous phase contrast computed tomography did not reveal evidence of underlying soft tissue infection and was inconclusive regarding a DVT. Ultrasound demonstrated a right subclavian vein DVT. The patient was admitted and underwent thrombolysis, venolysis, and first rib resection and initiation of warfarin. PSS is a rare presentation of upper-extremity DVT occurring classically in patients without commonly recognized pro-thrombotic risk factors. PSS carries the potential of significant morbidity in the form of post-thrombotic syndrome and pulmonary embolism. Current literature suggests that optimal outcomes are achieved when treatment is initiated within 6 weeks of onset. The treatment paradigm calls for thrombolysis and, frequently, a first rib resection. Copyright © 2013 Elsevier Inc. All rights reserved.

EFFICACY OF RECURRENT RITUXIMAB TREATMENT IN PATIENT WITH SEVERE REFRACTORY SYSTEMIC JUVENILE RHEUMATOID ARTHRITIS

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E.I. Alexeeva

2011-01-01

Full Text Available The article contains clinical case description of a severe systemic juvenile rheumatoid arthritis, that was refractory to classic immunosuppressant therapy. The disease was characterized by such extraarticular manifestations as fever, lymphadenopathy,  hepatosplenomegaly, polyserositis, generalized joint involvement and high activity in lab tests. As a result of severe clinical course of the disease, patients develop bilateral aseptic bone necrosis in coxofemoral joints and coxarthrosis. Against the background of glucocorticosteroid treatment the patient has developed hormone-dependency and hormone resistance. Inclusion into the treatment of anti-CD20 monoclonal antibodies (rituximab has stopped systemic manifestations of the disease, inflammation in the joints, normalized lab activity rates. The positive therapeutic effect allowed to perform surgery due to bilateral coxarthrosis. These results show that rituximab is highly effective in children with systemic juvenile rheumatoid arthritis, that is resistant to classic immunosupressants and glucocorticoides. Key words: children, systemic juvenile rheumatoid arthritis, rituximab, recurrent treatment, prosthetics, hip joint. (Voprosy sovremennoi pediatrii — Current Pediatrics. — 2011; 10 (5: 157–163.

Criteria for evaluating response and outcome in clinical trials for children with juvenile myelomonocytic leukemia.

LENUS (Irish Health Repository)

Niemeyer, Charlotte M

2015-01-01

Juvenile myelomonocytic leukemia is a rare myeloproliferative disease in young children. While hematopoietic stem cell transplantation remains the only curative therapeutic option for most patients, children with juvenile myelomonocytic leukemia increasingly receive novel agents in phase I-II clinical trials as pre-transplant therapy or therapy for relapse after transplantation. However, response criteria or definitions of outcome for standardized evaluation of treatment effect in patients with juvenile myelomonocytic leukemia are currently lacking. Here we propose criteria to evaluate the response to the non-transplant therapy and definitions of remission status after hematopoietic stem cell transplantation. For the evaluation of non-transplant therapy, we defined 6 clinical variables (white blood cell count, platelet count, hematopoietic precursors and blasts in peripheral blood, bone marrow blast percentage, spleen size and extramedullary disease) and 3 genetic variables (cytogenetic, molecular and chimerism response) which serve to describe the heterogeneous picture of response to therapy in each individual case. It is hoped that these criteria will facilitate the comparison of results between clinical trials in juvenile myelomonocytic leukemia.

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

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E. I. Alexeeva

2013-01-01

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

Nasal juvenile angiofibroma: Current perspectives with emphasis on management.

Science.gov (United States)

López, Fernando; Triantafyllou, Asterios; Snyderman, Carl H; Hunt, Jennifer L; Suárez, Carlos; Lund, Valerie J; Strojan, Primož; Saba, Nabil F; Nixon, Iain J; Devaney, Kenneth O; Alobid, Isam; Bernal-Sprekelsen, Manuel; Hanna, Ehab Y; Rinaldo, Alessandra; Ferlito, Alfio

2017-05-01

Juvenile angiofibroma is an uncommon, benign, locally aggressive vascular tumor. It is found almost exclusively in young men. Common presenting symptoms include nasal obstruction and epistaxis. More advanced tumors may present with facial swelling and visual or neurological disturbances. The evaluation of patients with juvenile angiofibroma relies on diagnostic imaging. Preoperative biopsy is not recommended. The mainstay of treatment is resection combined with preoperative embolization. Endoscopic surgery is the approach of choice in early stages, whereas, in advanced stages, open or endoscopic approaches are feasible in expert hands. Postoperative radiotherapy (RT) or stereotactic radiosurgery seem valuable in long-term control of juvenile angiofibroma, particularly those that extend to anatomically critical areas unsuitable for complete resection. Chemotherapy and hormone therapy are ineffective. The purpose of the present review was to update current aspects of knowledge related to this rare and challenging disease. © 2017 Wiley Periodicals, Inc. Head Neck 39: 1033-1045, 2017. © 2017 Wiley Periodicals, Inc.

Dynamic contrast-enhanced magnetic resonance imaging of the wrist in children with juvenile idiopathic arthritis

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Nusman, Charlotte M. [Emma Children' s Hospital, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Academic Medical Center, Amsterdam (Netherlands); Academic Medical Center, Department of Radiology, Amsterdam (Netherlands); Lavini, Cristina; Hemke, Robert; Caan, Matthan W.A.; Maas, Mario [Academic Medical Center, Department of Radiology, Amsterdam (Netherlands); Schonenberg-Meinema, Dieneke; Berg, J.M. van den; Kuijpers, Taco W. [Emma Children' s Hospital, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Academic Medical Center, Amsterdam (Netherlands); Dolman, Koert M. [Sint Lucas Andreas Hospital, Department of Pediatrics, Amsterdam (Netherlands); Reade Institute location Jan van Breemen, Department of Pediatric Rheumatology, Amsterdam (Netherlands); Rossum, Marion A.J. van [Reade Institute location Jan van Breemen, Department of Pediatric Rheumatology, Amsterdam (Netherlands); Emma Children' s Hospital, Department of Pediatrics, Academic Medical Center, Amsterdam (Netherlands)

2017-02-15

Dynamic contrast-enhanced MRI provides information on the heterogeneity of the synovium, the primary target of disease in children with juvenile idiopathic arthritis (JIA). To evaluate the feasibility of dynamic contrast-enhanced MRI in the wrist of children with JIA using conventional descriptive measures and time-intensity-curve shape analysis. To explore the association between enhancement characteristics and clinical disease status. Thirty-two children with JIA and wrist involvement underwent dynamic contrast-enhanced MRI with movement-registration and were classified using validated criteria as clinically active (n = 27) or inactive (n = 5). Outcome measures included descriptive parameters and the classification into time-intensity-curve shapes, which represent the patterns of signal intensity change over time. Differences in dynamic contrast-enhanced MRI outcome measures between clinically active and clinically inactive disease were analyzed and correlation with the Juvenile Arthritis Disease Activity Score was determined. Comprehensive evaluation of disease status was technically feasible and the quality of the dynamic dataset was improved by movement registration. The conventional descriptive measure maximum enhancement differed significantly between clinically active and inactive disease (P = 0.019), whereas time-intensity-curve shape analysis showed no differences. Juvenile Arthritis Disease Activity Score correlated moderately with enhancing volume (P = 0.484). Dynamic contrast-enhanced MRI is a promising biomarker for evaluating disease status in children with JIA and wrist involvement. Conventional descriptive dynamic contrast-enhanced MRI measures are better associated with clinically active disease than time-intensity-curve shape analysis. (orig.)

Acute Respiratory Failure in 3 Children With Juvenile Myelomonocytic Leukemia

DEFF Research Database (Denmark)

Gustafsson, Britt; Hellebostad, Marit; Ifversen, Marianne

2011-01-01

Juvenile myelomonocytic leukemia is a rare hematopoietic stem cell disease in children with features of both myelodysplasia and myeloproliferation. Extramedullary involvement has been reported and pulmonary involvement secondary to leukemic infiltration is an initial manifestation, which may result...

Uveíte na artrite idiopática juvenil Uveitis in juvenile idiopathic arthritis

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Adriana M. Roberto

2002-02-01

üente na população de pacientes com AIJ associada com uveíte (60% do que naqueles sem uveíte (12% (pObjective: to evaluate the frequency of chronic anterior uveitis in patients with juvenile idiopathic arthritis and its association with the presence of antinuclear antibodies. Patients and methods: we retrospectively studied 72 patients with juvenile idiopathic arthritis. All of them were submitted to slit-lamp examination of the anterior chamber at diagnosis. Both antinuclear antibodies and rheumatoid factor were determined. Patients with positive results for antinuclear antibodies were evaluated every three months and those with negative results were assessed every six months.Results: forty patients were male (55.5% and 36 were Caucasoid (50%. The mean age at the onset of juvenile idiopathic arthritis was 6.4 years (range = 1 to 14 years and the mean age at the beginning of the study was 10.4 years (1 to 19 years. According to the type of disease at onset, 32 were pauciarticular (44.4% (17 boys and 15 girls, 30 were polyarticular (41.6% (17 boys and 13 girls and 10 were systemic (14% (6 boys and 4 girls. We observed chronic anterior uveitis in five patients (6.5% (mean age = 11.4 years. Among them, four (80% had pauciarticular juvenile idiopathic arthritis at disease onset (three girls with type I juvenile idiopathic arthritis and positive antinuclear antibodies and one boy with type I juvenile idiopathic arthritis and negative antinuclear antibodies and one girl with polyarticular juvenile idiopathic arthritis (negative antinuclear antibodies and rheumatoid factor. In this group, the mean age at the onset of juvenile idiopathic arthritis was 5.1 years and the mean age of uveitis onset was 9 years. Antinuclear antibodies were positive in 3/5 patients (60% with uveitis. Antinuclear antibodies were positive in 12% of the patients without uveitis (n = 67. Among the patients with uveitis, three had only one flare and the other two had four flares with cataract. The

Juvenile giant fibroadenoma

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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.

Giant juvenile fibroadenoma: a case and review of novel modalities in treatment.

Science.gov (United States)

Sosin, Michael; Feldman, Elizabeth

2012-01-01

A giant juvenile fibroadenoma is defined as a fibroadenoma greater than 5 centimeters in size occurring in the pediatric population. It frequently affects adolescents. Rapid growth of the mass may result in breast asymmetry and deformity. Varying techniques in surgical extirpation have been described in order to optimize aesthetics and minimize distortion. The advent of new methods to remove benign breast disease is in its infancy stages. Many practitioners are unaware of the novel options that are emerging in the treatment of fibroadenoma. We describe an excision of a 12 centimeter giant juvenile fibroadenoma and adjacent juvenile fibroadenoma using a strategically atypical incision that resulted in excellent cosmesis and contour of the breast without subsequent reconstruction. Multiple modalities of removing a fibroadenoma are described with a review of the associated risks, benefits, and long term implications as well as a discussion on the indication for reconstructive surgery in patients with giant juvenile fibroadenoma.

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

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O. Yu. Konopel'ko

2013-01-01

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

EXPERIENCE OF TREATMENT OF JUVENILE POLYARTHRITIS WITH EYES LESION WITH ADALIMUMAB

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A.O. Lisitsin

2008-01-01

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

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

Science.gov (United States)

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

2015-01-01

Machrophage activation syndrome (MAS) is a rare and potentially fatal disease, commonly associated with chronic rheumatic diseases, mainly juvenile idiopathic arthritis. It is included in the group of secondary forms of haemophagocytic syndrome, and other causes are lymphoproliferative diseases and infections. Its most important clinical and laboratorial manifestations are non-remitting fever, splenomegaly, bleeding, impairment of liver function, cytopenias, hypoalbuminemia, hypertriglyceridemia, hypofibrinogenemia and hyperferritinemia. The treatment needs to be started quickly, and the majority of cases have a good response with corticosteroids and cyclosporine. The Epstein-Barr virus is described as a possible trigger for many cases of MAS, especially in these patients in treatment with tumor necrosis factor (TNF) blockers. In these refractory cases, etoposide (VP16) should be administered, associated with corticosteroids and cyclosporine. Our objective is to describe a rare case of MAS probably due to EBV infection in a subject with systemic-onset juvenile idiopathic arthritis, which achieved complete remission of the disease after therapy guided by 2004-HLH protocol. Copyright © 2014 Elsevier Editora Ltda. All rights reserved.

Juvenile localized scleroderma with port wine stain: coincidental or possible common pathogenetic association.

Science.gov (United States)

Kacar, Seval Dogruk; Ozuguz, Pinar; Polat, Serap; Kacar, Emre; Polat, Onur; Tokyol, Cigdem

2015-01-01

Port wine stain and juvenile localized scleroderma are two different dermatoses usually encountered in pediatric age group. Up to now, there are reports of morphea patients initially diagnosed and treated as port wine stain. Coexistence of both diseases is not found yet. We herein present a case of juvenile localized scleroderma on the left side of trunk, with congenital port wine stain located on the ipsilateral face at V1-V2 distribution.

Impact of Juvenile Idiopathic Arthritis Associated Uveitis in Early Adulthood

NARCIS (Netherlands)

Haasnoot, AJW; Vernie, Lenneke A; Rothova, Aniki; V D Doe, Patricia; Los, Leonoor I; Schalij-Delfos, Nicoline E; de Boer, Joke H

2016-01-01

BACKGROUND: Typically juvenile idiopathic arthritis (JIA)-associated uveitis (further referred as 'JIA-uveitis') has its onset in childhood, but some patients suffer its, sometimes visual threatening, complications or ongoing disease activity in adulthood. The objective of this study was to analyze

Impact of Juvenile Idiopathic Arthritis Associated Uveitis in Early Adulthood

NARCIS (Netherlands)

Haasnoot, Anne-Mieke J. W.; Vernie, Lenneke A.; Rothova, Aniki; van der Doe, Patricia; Los, Leonoor I.; Schalij-Delfos, Nicoline E.; de Boer, Joke H.

2016-01-01

Background Typically juvenile idiopathic arthritis (JIA)-associated uveitis (further referred as 'JIA-uveitis') has its onset in childhood, but some patients suffer its, sometimes visual threatening, complications or ongoing disease activity in adulthood. The objective of this study was to analyze

Impact of juvenile idiopathic arthritis associated uveitis in early adulthood

NARCIS (Netherlands)

Haasnoot, A.-M.J.W. (Anne-Mieke J. W.); Vernie, L.A. (Lenneke A.); A. Rothová (Aniki); Doe, P.V.D. (Patricia V. D.); L.I. Los (Leonoor I.); N.E. Schalij-Delfos (Nicoline); J.H. de Boer (Joke)

2016-01-01

textabstractBackground: Typically juvenile idiopathic arthritis (JIA)-associated uveitis (further referred as 'JIA-uveitis') has its onset in childhood, but some patients suffer its, sometimes visual threatening, complications or ongoing disease activity in adulthood. The objective of this study was

Results of radiotherapy in case of juvenile xanthogranuloma of the iris

International Nuclear Information System (INIS)

Mueller, R.P.; Juenemann, G.

1983-01-01

The juvenile xanthogranuloma is a rare, benign, cutaneous disease occurring in infants and young children which can be situated in about 10% of the cases on the eye and preferentially on the iris. The reason for consulting an ophthalmologist is mostly a recurrent unilateral hyphemia which is typical for this disease. The diagnosis of the juvenile xanthogranuloma has to be established clinically, based on the characteristic alterations of the iris - heterochromia, vascularized tumor or diffuse thickening of the iris - and the possibly increased intraocular pressure. In order to avoid the deleting consequences of an uncontrollable secondary glaucoma, radiotherapy with a total dose of 300 to 400 cGy combined with a mild local steroid treatment has to be undertaken immediately, if possible. Taking into consideration the indications of literature, the value of a low-dose radiotherapy for this disease shall be demonstrated by five own cases in all of which the concerned eye could be healed and preserved. (orig.) [de

The Evolution of Juvenile Schönlein-Henoch Purpura

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O.E. Chernyshova

2016-10-01

Full Text Available Background. Hemorrhagic vasculitis, or Schönlein-Henoch purpura (SHP, is the most common type of systemic vasculitis in childhood, and peculiarities of the further evolution of the pathological process in adult patients remain unexplored. Objective: to study the evolution of juvenile SHP, comparing the nature of lesions of the skin, joints, heart and kidneys in patients in childhood and adulthood. Material and methods. The study included 92 patients (61 men and 31 women with the average age of 27 years, and the average age of disease onset — 11 years. I degree of the activity of the pathological process is determined in 40 % of cases, II — in 35 %, III — in 25 %. Seropositivity by hyperimmunoglobulinemia A occurred in 27 % of cases, by the presence of rheumatoid factor — in 21 %. At the time of the survey, cutaneous syndrome was diagnosed in 55 % of patients, joint one — in 45 %, kidney one — in 71 %. Renal biopsy was performed in 15 cases. Results. The cutaneous, cutaneous-joint-abdominal and cutaneous-abdominal-renal forms of the disease, lesions of the skin, gastrointestinal tract, wrist, ankle and knee joints become rarer in the process of juvenile SHP evolution, but exceptionally renal variant of the pathological process, changes of skeletal muscles, liver, spleen and the heart are revealed more often, chronic kidney disease with the renal failure progression is developed in 12 % of patients (in 17 % of cases of nephropathy, sacroiliitis, spondylopathy, tendovaginitis, enthesopathies, epiphyseal osteoporosis and meniscitis of the knee joints are arisen, II, III, VI and IV morphological classes of Henoch glomerulonephritis are formed in a ratio of 8 : 4 : 2 : 1 with tubulointerstitial component in all cases, and lymphohistiocytic infiltration of the vascular wall is the unfavorable sign for the prognosis of the disease. Conclusions. In cases of transition of juvenile SHP into the chronic adult form, the disease often obtain

Psoralea corylifolia L. Attenuates Nonalcoholic Steatohepatitis in Juvenile Mouse

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Lishan Zhou

2017-11-01

Full Text Available Psoralea corylifolia L. (PC is a traditional Chinese herb used to treat yang deficiency of the spleen and kidney in pediatric disease. Recent studies have shown its liver protection and anti-oxidative effects. The aim of this study was to explore the effect and mechanism of PC on nonalcoholic steatohepatitis in juvenile mice. The juvenile mouse model of nonalcoholic fatty liver disease/nonalcoholic steatohepatitis (NAFLD/NASH was established by being fed a high-fat diet in maternal-offspring manner. PC granules were prepared and the quality was assessed. The main components were identified by high performance liquid chromatography. Then, different dosages of PC were administered for 6 weeks. Homeostatic model assessment of insulin resistance, plasma liver enzymes, hepatic morphology, hepatic superoxide anion, and triglyceride/total cholesterol levels were examined. The changes of nuclear factor-κB (NF-κB activity phosphatidylinositol 3 kinase (PI3K/protein kinase B (Akt and protein kinase C-α (PKC-α/nicotinamide-adenine dinucleotide phosphate (NADPH oxidase signaling pathways in hepatic tissues were also determined. Our data demonstrated that PC significantly improved liver dysfunction, liver triglyceride/total cholesterol accumulation and insulin resistance in juvenile NAFLD/NASH mice. PC also alleviated hepatic steatosis, inflammatory cell infiltration, and fibroplasia in the portal area. Additionally, PC inhibited the activation of NF-κB and the mRNA expression of inflammatory factors while enhancing PI3K/Akt signaling in hepatic tissues. PC could also reduce hepatic superoxide anion levels, and NADPH oxidase activity as well as p47phox protein expression and PKCα activation in hepatic tissues. The results suggest that PC is effective in the treatment of NASH in juvenile mice. The mechanism may be related to the attenuation of hepatic oxidative stress through the PKC-α/NADPH oxidase signaling pathway.

Self-control with urinalysis in juvenile diabetes.

Science.gov (United States)

Ludvigsson, J; Svensson, P G

1979-11-01

Urinary glucose excretion reflects the blood glucose levels and is therefore recommended and used as a relevant and practical method for self-control in juvenile diabetes. The purpose of this study was to estimate the attitudes of diabetic children and their parents towards such daily urinalysis. In 1975 69 juvenile diabetics 6--18 years old and their parents were studied and three years later another 69 patients were added. Standardized interviews and questionnaires were used. Only 3 out of 138 patients refused to test their urine regularly and to write down their results in the diary. The results indicate that a great majority of the patients and the parents easily accept the self-testing method and regard it as a valuable tool in the management of the disease. Almost nobody experienced the urine tests as a psychological problem.

Ceroid lipofuscinosis in the border collie dog: retinal lesions in an animal model of juvenile Batten disease.

Science.gov (United States)

Taylor, R M; Farrow, B R

1992-02-15

Ceroid lipofuscinosis, an inherited disorder of lipopigment accumulation, was identified in a group of Border Collie dogs. The dogs developed mental, motor, and visual signs between age 15 and 22 months and progressed rapidly to severe neurological disease. The principal signs were blindness and gait and behavioural abnormalities with progressive dementia. Lipopigment accumulation was severe in neurones and glial cells of the central nervous system and was present in some visceral cells. Inclusions with variable ultrastructure were common in all cells of the retina, but the pigment accumulation did not damage the retinal architecture. The cytoplasmic inclusions were granular, sudanophilic, eosinophilic, and autofluorescent. Ultrastructural morphology varied, but fingerprint and curvilinear patterns predominated. The retinal lesions in the Border Collies were similar to those in English Setters with ceroid lipofuscinosis, but were much less severe than in juvenile human ceroid lipofuscinosis. This disorder bears a close resemblance to ceroid lipofuscinosis in English Setters and is another useful model for Batten's disease.

Genetic architecture distinguishes systemic juvenile idiopathic arthritis from otherforms of juvenile idiopathic arthritis: clinical and therapeutic implications

OpenAIRE

Ombrello, Michael J.; Arthur, Victoria L.; Remmers, Elaine F.; Hinks, Anne; Tachmazidou, Ioanna; Grom, Alexei A.; Foell, Dirk; Martini, Alberto; Gattorno, Marco; Ozen, Seza; Prahalad, Sampath; Zeft, Andrew S.; Bohnsack, John F.; Ilowite, Norman T.; Mellins, Elizabeth D.

2016-01-01

Objectives: Juvenile idiopathic arthritis (JIA) is a heterogeneous group of conditions unified by the presence of chronic childhood arthritis without an identifiable cause. Systemic JIA (sJIA) is a rare form of JIA characterized by systemic inflammation. sJIA is distinguished from other forms of JIA by unique clinical features and treatment responses that are similar to autoinflammatory diseases. However approximately half of children with sJIA develop destructive, longstanding arthritis that...

Ghrelin levels in patients with juvenile idiopathic arthritis: relation to anti-tumor necrosis factor treatment and disease activity.

Science.gov (United States)

Karagiozoglou-Lampoudi, Thomais; Trachana, Maria; Agakidis, Charalampos; Pratsidou-Gertsi, Polyxeni; Taparkou, Anna; Lampoudi, Sotiria; Kanakoudi-Tsakalidou, Florentia

2011-10-01

Studies in adults with rheumatoid arthritis reported low serum ghrelin that increased following anti-tumor necrosis factor (TNF) infusion. Data on juvenile idiopathic arthritis (JIA) are lacking. The aim of this pilot study was to explore serum ghrelin levels in patients with JIA and the possible association with anti-TNF treatment, disease activity, and nutritional status. Fifty-two patients with JIA (14/52 on anti-TNF treatment) were studied. Juvenile idiopathic arthritis was inactive in 3 of 14 anti-TNF-treated patients and in 11 of 38 non-anti-TNF-treated patients. The nutritional status, energy intake/requirements, appetite, and fasting serum ghrelin levels were assessed. Ghrelin control values were obtained from 50 individuals with minor illness matched for age, sex, and body mass index. Ghrelin levels in patients with JIA were significantly lower than in controls (P ghrelin levels were comparable to control values only in 3 patients with anti-TNF-induced remission. Ghrelin in non-anti-TNF-treated patients in remission was low. Multiple regression analysis showed that disease activity (P = .002, CI = -84.16 to -20.01) and anti-TNF treatment (P = .003, CI = -82.51 to -18.33) were significant independent predictors of ghrelin after adjusting for other potential confounders. Ghrelin did not correlate with nutritional status, energy balance, and appetite. Serum ghrelin is low in patients with JIA and is restored to values similar to those in controls following anti-TNF-induced remission. Our study provides evidence that TNF blockade is independently associated with serum ghrelin, which possibly contributes to anti-TNF-induced remission. These preliminary results could form the basis for future research. Copyright © 2011 Elsevier Inc. All rights reserved.

Acute respiratory failure in 3 children with juvenile myelomonocytic leukemia

DEFF Research Database (Denmark)

Gustafsson, Britt; Hellebostad, Marit; Ifversen, Marianne

2011-01-01

Juvenile myelomonocytic leukemia is a rare hematopoietic stem cell disease in children with features of both myelodysplasia and myeloproliferation. Extramedullary involvement has been reported and pulmonary involvement secondary to leukemic infiltration is an initial manifestation, which may resu...... in acute respiratory failure....

Juvenile localized scleroderma with port wine stain: Coincidental or possible common pathogenetic association

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Seval Dogruk Kacar

2015-01-01

Full Text Available Port wine stain and juvenile localized scleroderma are two different dermatoses usually encountered in pediatric age group. Up to now, there are reports of morphea patients initially diagnosed and treated as port wine stain. Coexistence of both diseases is not found yet. We herein present a case of juvenile localized scleroderma on the left side of trunk, with congenital port wine stain located on the ipsilateral face at V1-V2 distribution.

The frequency of pulmonary hypertension in patients with juvenile scleroderma

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Amra Adrovic

2015-08-01

Full Text Available Juvenile scleroderma (JS represents a rarely seen group of connective tissue diseases with multiple organ involvement. Cardiac involvement in JSS is well known and, although rare in children, it may be an important cause of mortality and morbidity. Therefore, an early determination of cardio-vascular and pulmonary involvement is of the most relevance to reduce the mortality in patients with juvenile scleroderma. The aim of the study was to explore the non-invasive methods (Doppler echocardiography, pulmonary function tests, Forced vital capacity (FVC and Carbon monoxide diffusion capacity (DLCO in the assessment of the cardiopulmonary involvement in patients with JS. The assessment of pulmonary arterial pressure (PAP and risk factors for pulmonary arterial hypertension (PAH were made by the measurement of maximum tricuspid insufficiency (TI, end-diastolic pulmonary insufficiency (PI, ratio of acceleration time (AT to ejection time (ET (AT/ET, right atrial pressure (RAP and contraction of vena cava inferior during inspiration. Thirty-five patients with confirmed JS were included in the study. The mean age of onset of the disease was 9.57 years (median 10 years, range 2-18 years. The mean disease duration and follow-up time was 2 years (median 1 year, range 0.5-8 years and 3.57 years (median 2 years, range 0.5-14.5 years, respectively.The values of all the analyzed parameters including TI, PI, AT/ET, PAP, FVC and DLCO were found to be within normal ranges in all the patients tested, confirming an uncommonness of cardiopulmonary involvement in patients with juvenile scleroderma.

The frequency of pulmonary hypertension in patients with juvenile scleroderma.

Science.gov (United States)

Adrovic, Amra; Oztunc, Funda; Barut, Kenan; Koka, Aida; Gojak, Refet; Sahin, Sezgin; Demir, Tuncalp; Kasapcopur, Ozgur

2015-08-22

Juvenile scleroderma (JS) represents a rarely seen group of connective tissue diseases with multiple organ involvement. Cardiac involvement in JSS is well known and, although rare in children, it may be an important cause of mortality and morbidity. Therefore, an early determination of cardio-vascular and pulmonary involvement is of the most relevance to reduce the mortality in patients with juvenile scleroderma. The aim of the study was to explore the non-invasive methods (Doppler echocardiography, pulmonary function tests), Forced vital capacity (FVC) and Carbon monoxide diffusion capacity (DLCO) in the assessment of the cardiopulmonary involvement in patients with JS. The assessment of pulmonary arterial pressure (PAP) and risk factors for pulmonary arterial hypertension (PAH) were made by the measurement of maximum tricuspid insufficiency (TI), end-diastolic pulmonary insufficiency (PI), ratio of acceleration time (AT) to ejection time (ET) (AT/ET), right atrial pressure (RAP) and contraction of vena cava inferior during inspiration. Thirty-five patients with confirmed JS were included in the study. The mean age of onset of the disease was 9.57 years (median 10 years, range 2-18 years). The mean disease duration and follow-up time was 2 years (median 1 year, range 0.5-8 years) and 3.57 years (median 2 years, range 0.5-14.5 years), respectively.The values of all the analyzed parameters including TI, PI, AT/ET, PAP, FVC and DLCO were found to be within normal ranges in all the patients tested, confirming an uncommonness of cardiopulmonary involvement in patients with juvenile scleroderma.

Tics as an initial manifestation of juvenile Huntington's disease: case report and literature review.

Science.gov (United States)

Cui, Shi-Shuang; Ren, Ru-Jing; Wang, Ying; Wang, Gang; Chen, Sheng-Di

2017-08-08

Huntington's disease (HD) is an autosomal dominant disorder, typically characterized by chorea due to a trinucleotide repeat expansion in the HTT gene, although the clinical manifestations of patients with juvenile HD (JHD) are atypical. A 17-year-old boy with initial presentation of tics attended our clinic and his DNA analysis demonstrated mutation in the HTT gene (49 CAG repeats). After treatment, his symptoms improved. Furthermore, we performed literature review through searching the databases and summarized clinical features in 33 JHD patients. The most prevalent symptoms are ataxia, and two cases reported that tics as initial and prominent manifestation in JHD. Among them, 88% patients carried CAG repeats beyond 60 and most of them have family history. This case here illustrates the variable range of clinical symptoms of JHD and the necessity of testing for the HD mutation in young patients with tics with symptoms unable to be explained by Tourette's syndrome (TS).

Ultrasonographic examination in juvenile idiopathic arthritis is better than clinical examination for identification of intraarticular disease

DEFF Research Database (Denmark)

Nielsen, Hans Erik; Strandberg, Charlotte; Andersen, Steen

2013-01-01

The diagnosis of juvenile idiopathic arthritis (JIA) is formally based on clinical examination, but ultrasound (US) examination is used increasingly. Our purpose was to compare US and clinical examination in the assessment of synovitis in JIA.......The diagnosis of juvenile idiopathic arthritis (JIA) is formally based on clinical examination, but ultrasound (US) examination is used increasingly. Our purpose was to compare US and clinical examination in the assessment of synovitis in JIA....

Clostridium difficile Colitis and Neutropenic Fever Associated with Docetaxel Chemotherapy in a Patient with Advanced Extramammary Paget’s Disease

OpenAIRE

Nonomura, Yumi; Otsuka, Atsushi; Endo, Yuichiro; Fujisawa, Akihiro; Tanioka, Miki; Kabashima, Kenji; Miyachi, Yoshiki

2012-01-01

Extramammary Paget's disease is a rare cutaneous malignant neoplasm. Previous studies indicated the efficacy of docetaxel in advanced cases. The common side effects of docetaxel are usually tolerable and seldom life-threatening. We experienced a case of severe pseudomembranous colitis and neutropenic fever that developed just after the first cycle of docetaxel chemotherapy. To the best of our knowledge, there are few reports of pseudomembranous colitis associated with docetaxel administration...

Juvenile hyperthyroidism: an experience.

Science.gov (United States)

Bhadada, S; Bhansali, A; Velayutham, P; Masoodi, S R

2006-04-01

To analyze the clinical profile of juvenile hyperthyroidism at presentation, their treatment outcome; predictors of remission and relapse. Retrospective analysis of medical records of 56 patients with juvenile hyperthyroidism seen over a period of 16 years. A cohort of 38 females and 18 males with mean (+/-SD) age of 14.9 +/- 3.4 years (range 3 to 18 years) was analyzed. Majority of patients was in the age group of 12-16 years. Common symptoms observed at presentation were weight loss (82.1%), excessive sweating (78.6%), heat intolerance (76.8%), increased appetite (73.2%) and diarrhea in 48.2%. In addition, accelerated linear growth was observed in 7.1% of patients. Goiter was present in 98.2% of children; 94.5% of which was diffuse and 4.8% was multinodular. The mean ((+/-SD) T3 was 4.8 +/- 3.4 ng/mL (N, 0.6-1.6), T4 was 218 +/- 98 ng/mL (N, 60-155) and TSH was 0.44 +/- 0.36 (N, 0.5-5.5 microIU/mL). TMA positivity seen in 36.9% of patients. All patients were treated with carbimazole; subsequently 4 patients required thyroidectomy and one required radioactive iodine ablation. Mean (+/-SD) duration of follow-up in our patients was 4.9 +/- 3 years, ranging between 1.6 to 16 years and mean (+/-SD) duration of treatment was 34.4 +/- 22.6 months (range 12 to 120 months). Mean (+/-SD) duration to achieve euthyroidism was 5.2 +/- 4.7 months, ranging between 1-33 months. On intention to treat analysis, remission with carbimazole was achieved in 47.6%, remaining patients failed to achieve remission with drug treatment. Graves disease is the commonest cause of juvenile hyperthyroidism. Carbimazole is safe, effective, cheap, and easily available form of therapy. It is occasionally associated with serious side effects but requires prolonged follow up.

Cartilage oligomeric matrix protein in patients with juvenile idiopathic arthritis: relation to growth and disease activity

DEFF Research Database (Denmark)

Bjørnhart, Birgitte; Juul, Anders; Nielsen, Susan

2009-01-01

OBJECTIVE: Cartilage oligomeric matrix protein (COMP) has been identified as a prognostic marker of progressive joint destruction in rheumatoid arthritis. In this population based study we evaluated associations between plasma concentrations of COMP, disease activity, and growth velocity...... in patients with recent-onset juvenile idiopathic arthritis (JIA). COMP levels in JIA and healthy children were compared with those in healthy adults. Plasma levels of insulin-like growth factor I (IGF-1), which has been associated with COMP expression and growth velocity, were studied in parallel. METHODS......: 87 patients with JIA entered the study, including oligoarticular JIA (n = 34), enthesitis-related arthritis (n = 8), polyarticular rheumatoid factor (RF)-positive JIA (n = 2), polyarticular RF-negative JIA (n = 27), systemic JIA (n = 6), and undifferentiated JIA (n = 10). Plasma levels of COMP were...

Impact of juvenile idiopathic arthritis on schooling

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Bouaddi Ilham

2013-01-01

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

Juvenile angiofibromer

DEFF Research Database (Denmark)

Thuesen, Anne Daugaard; Jakobsen, John; Nepper-Rasmussen, Jørgen

2005-01-01

Juvenile angiofibroma is a rare, benign, rich vascular tumor, and approximately one new case is diagnosed in Denmark each year. It sits in the foramen sphenopalatinum and occurs in boys from 14 to 25 years of age. The most frequent initial symptoms are nasal obstruction and epistaxis. Through...... the years, the treatment of juvenile angiofibroma has included many methods, including surgical excision, electrocoagulation, interstitial or external radiation therapy, cryosurgery, hormone administration and chemotherapy. Radiation, chemotherapy and surgery have proven to be the most effective treatments...

Early-Life Stress Triggers Juvenile Zebra Finches to Switch Social Learning Strategies.

Science.gov (United States)

Farine, Damien R; Spencer, Karen A; Boogert, Neeltje J

2015-08-17

Stress during early life can cause disease and cognitive impairment in humans and non-humans alike. However, stress and other environmental factors can also program developmental pathways. We investigate whether differential exposure to developmental stress can drive divergent social learning strategies between siblings. In many species, juveniles acquire essential foraging skills by copying others: they can copy peers (horizontal social learning), learn from their parents (vertical social learning), or learn from other adults (oblique social learning). However, whether juveniles' learning strategies are condition dependent largely remains a mystery. We found that juvenile zebra finches living in flocks socially learned novel foraging skills exclusively from adults. By experimentally manipulating developmental stress, we further show that social learning targets are phenotypically plastic. While control juveniles learned foraging skills from their parents, their siblings, exposed as nestlings to experimentally elevated stress hormone levels, learned exclusively from unrelated adults. Thus, early-life conditions triggered individuals to switch strategies from vertical to oblique social learning. This switch could arise from stress-induced differences in developmental rate, cognitive and physical state, or the use of stress as an environmental cue. Acquisition of alternative social learning strategies may impact juveniles' fit to their environment and ultimately change their developmental trajectories. Copyright © 2015 The Authors. Published by Elsevier Ltd.. All rights reserved.

Marcadores de inmunorrespuesta en la periodontitis juvenil

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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

Parenting and juvenile delinquency

NARCIS (Netherlands)

Hoeve, M.

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.

21 CFR 862.1400 - Hydroxyproline test system.

Science.gov (United States)

2010-04-01

... diagnosis and treatment of various collagen (connective tissue) diseases, bone disease such as Paget's disease, and endocrine disorders such as hyperparathyroidism and hyperthyroidism. (b) Classification...

Juvenile delinquency and correctional treatment in Britain

OpenAIRE

堀尾, 良弘; ホリオ, ヨシヒロ; Yoshihiro, Horio

2006-01-01

Japanese modernistic culture is influenced not a little from Britain. In looking at the Juvenile Law and the history of correctional treatment in Britain, understanding of today's juvenile delinquency and treatment deepen. Moreover, the background and issue of juvenile delinquency in Britain are also discussed. As a feature of the juvenile delinquency in Britain, the common field with Japan and the field peculiar to Britain became clear in each. It is common to the world that the juvenile del...

Juvenile prison in parallel legislation

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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.

Estudo comparativo de testes diagnósticos para olho seco entre crianças saudáveis e portadoras de artrite reumatóide juvenil Comparative study of diagnostic tests for dry eye disease between healthy and juvenile rheumatoid arthritis children

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Jayter Silva de Paula

2004-10-01

clinically and underwent tests for keratoconjunctivitis sicca: Schirmer's 1, tear film break-up time and rose bengal staining tests. RESULTS: Six children with juvenile rheumatoid arthritis presented one or more symptoms of keratoconjunctivitis sicca (40% and five of them (83.3% presented meibomitis or other signs of this disease. In group 2, no child presented symptoms or signs of keratoconjunctivitis sicca. Mean Schirmer test did not differ between group 1 and 2 (p=0.156. However, the mean tear film break-up time was significantly reduced in group 1 (p=0.0005 and the mean rose Bengal staining score in group 1 was significantly greater than in group 2 (p=0.0038. Five of the fifteen children of group 1 (33% have two or more abnormal tests and were diagnosed as having definite keratoconjunctivitis sicca, while four children (26% were labeled with probable keratoconjunctivitis sicca. No child of group 2 had more than one positive test. CONCLUSIONS: Signs and symptoms of keratoconjunctivitis sicca appear to be a common ocular finding in juvenile rheumatoid arthritis children. Although only tear film break-up time and rose bengal staining score were significantly different in these groups, there was a trend toward worsening of the other dry eye tests in juvenile rheumatoid arthritis children.

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...

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…

Juvenile morphology in baleen whale phylogeny.

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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.

[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.

Larval, pre-juvenile and juvenile development of Diapterus peruvianus (Perciformes: Gerreidae

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Sylvia Patricia Adelheid Jiménez Rosenberg

2003-06-01

Full Text Available The development of Diapterus peruvianus (Sauvage 1879 is based on 60 larvae collected in superficial tows made in Bahía Concepción, and on 16 prejuvenile and juvenile organisms collected in Bahía de La Paz, B. C. S., México, using a standard plankton net and a rectangular epibenthonic net, respectively. Larvae of D. peruvianus show three large blotches on the dorsum of the gut that can fuse together and give the appearance of one large continuous blotch. There are two to three pre-anal pigments and 16 post-anal pigments in the ventral midline; cephalic pigments are present from the postflexion stage, as well as a serrated preoperculum. The prejuvenile and juvenile organisms are distinguished by their body depth, the analfin formula, the serrated preoperculum and the base pigments in the dorsal and anal fins.El desarrollo de Diapterus peruvianus se analizó con base en 60 larvas recolectadas en Bahía Concepción y 16 pre-juveniles y juveniles recolectados en la Ensenada de La Paz, B. C. S. México, usando respectivamente, una red estándar de plancton en arrastres superficiales y una red epibentónica para arrastres de plancton. Las larvas presentan desde la pre-flexión tres manchas alargadas sobre la superficie dorsal de la masa visceral, que pueden unirse y dar apariencia de pigmentación continua, observándose hasta 16 pigmentos post-anales en la línea media ventral y de dos a tres pigmentos pre-anales; la pigmentación cefálica así como la forma aserrada del pre-opérculo característica del género, aparecen a partir de la post-flexión. Los organismos pre-juveniles y juveniles se distinguen por la profundidad del cuerpo, la fórmula de la aleta anal, la fina forma aserrada del pre-opérculo y la pigmentación en la base de las aletas dorsal y anal.

Juvenile Residential Facility Census, 2010: Selected Findings. Juvenile Offenders and Victims: National Report Series. Bulletin NCJ 241134

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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…

Vaccination of free-living juvenile wild rabbits (Oryctolagus cuniculus) against myxomatosis improved their survival.

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Guitton, Jean-Sébastien; Devillard, Sébastien; Guénézan, Michel; Fouchet, David; Pontier, Dominique; Marchandeau, Stéphane

2008-04-17

For several decades, the populations of the European wild rabbit (Oryctolagus cuniculus) have declined, which is partly due to myxomatosis. Vaccination against this disease is expected to contribute to restoration of rabbit populations but the actual impact of myxomatosis is not well known and vaccination might have some negative effects. We analyzed the capture-mark-recapture data obtained in a 4-year field experiment (1991-1994) in a park near Paris, France wherein 300 out of 565 seronegative juvenile rabbits were vaccinated at first capture against myxomatosis with the nontransmissible Dervaximyxo SG33 vaccine. After accounting for weight at first capture, age-class (juvenile/adult), "trap-happiness" and season (spring/autumn) of the capture event, vaccinated rabbits had 1.8-fold greater odds of surviving than the unvaccinated rabbits. The average summer survival risk for vaccinated juveniles was 0.63 (+/-0.08 S.E.) whereas it was 0.48 (+/-0.08 S.E.) for unvaccinated juvenile rabbits.

Imaging of juvenile spondyloarthritis. Part II: Ultrasonography and magnetic resonance imaging

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Iwona Sudoł-Szopińska

2017-09-01

Full Text Available Juvenile spondyloarthropathies are mainly manifested by symptoms of peripheral arthritis and enthesitis. Early involvement of sacroiliac joints and spine is exceptionally rare in children; this usually happens in adulthood. Conventional radiographs visualize late inflammatory lesions. Early diagnosis is possible with the use of ultrasonography and magnetic resonance imaging. The first part of the article presented classifications and radiographic presentation of juvenile spondyloarthropathies. This part discusses changes seen on ultrasonography and magnetic resonance imaging. In patients with juvenile spondyloarthropathies, these examinations are conducted to diagnose inflammatory lesions in peripheral joints, tendon sheaths, tendons and bursae. Moreover, magnetic resonance also shows subchondral bone marrow edema, which is considered an early sign of inflammation. Ultrasonography and magnetic resonance imaging do not show specific lesions for any rheumatic disease. Nevertheless, they are conducted for early diagnosis, treatment monitoring and identifying complications. This article presents a spectrum of inflammatory changes and discusses the diagnostic value of ultrasonography and magnetic resonance imaging.

APPRAISAL OF NIMESULIDE EFFICIENCY, TOLERANCE AND SAFETY AMONG CHILDREN WITH JUVENILE ARTHRITIS

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E.I. Alexeeva

2007-01-01

Full Text Available The article analyzes nimesulide application experience among children with juvenile arthritides against the insufficient efficiency of a therapy by other non steroid anti-inflammatory medications. The researchers show that nimesulide is an efficient non steroid anti-inflammatory medication for the patients, suffering from oligo- and limited poly arthritis of the I–II activity degree. Nimesulide also provided for the reliable regression of the clinical and laboratory activity manifestations of a disease and, what is more important, among most patients without applications of the local glucocorticosteroidbassisted therapy. Nimesulide is characterized by good tolerance and low toxicity, which is along with the permit to use it among children aged 2 and over, allows one to consider this drug as one of the medications to choose for the treatment of the inflammatory joint diseases among children.Key words: children, juvenile arthritis, nimesulide.

Juvenile Confinement in Context

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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…

[Periodontal disease in pediatric rheumatic diseases].

Science.gov (United States)

Fabri, Gisele M C; Savioli, Cynthia; Siqueira, José T; Campos, Lucia M; Bonfá, Eloisa; Silva, Clovis A

2014-01-01

Gingivitis and periodontitis are immunoinflammatory periodontal diseases characterized by chronic localized infections usually associated with insidious inflammation This narrative review discusses periodontal diseases and mechanisms influencing the immune response and autoimmunity in pediatric rheumatic diseases (PRD), particularly juvenile idiopathic arthritis (JIA), childhood-onset systemic lupus erythematosus (C-SLE) and juvenile dermatomyositis (JDM). Gingivitis was more frequently observed in these diseases compared to health controls, whereas periodontitis was a rare finding. In JIA patients, gingivitis and periodontitis were related to mechanical factors, chronic arthritis with functional disability, dysregulation of the immunoinflammatory response, diet and drugs, mainly corticosteroids and cyclosporine. In C-SLE, gingivitis was associated with longer disease period, high doses of corticosteroids, B-cell hyperactivation and immunoglobulin G elevation. There are scarce data on periodontal diseases in JDM population, and a unique gingival pattern, characterized by gingival erythema, capillary dilation and bush-loop formation, was observed in active patients. In conclusion, gingivitis was the most common periodontal disease in PRD. The observed association with disease activity reinforces the need for future studies to determine if resolution of this complication will influence disease course or severity. Copyright © 2014 Elsevier Editora Ltda. All rights reserved.

EVALUATION OF THICKNESS OF INTIMA-MEDIA COMPLEX OF COMMON CAROTID ARTERIES IN CHILDREN WITH JUVENILE ARTHRITIS AND SYSTEMIC LUPUS ERYTHEMATOSUS

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A.B. Sugak

2010-01-01

Full Text Available Rheumatic diseases in adults are associated with accelerated atherosclerosis, and its early signs can be stated by the thickening of intima-media complex of common carotid arteries (CCA. This symptom is detected during ultrasound examination in 49% of children with systemic lupus erythematosus, in 24% of patients with juvenile rheumatoid arthritis and in 13% of children with juvenile spondylarthritis. Besides, 36% of children with systemic lupus erythematosus and 17% — with systemic type of juvenile rheumatoid arthritis had structure changes of CCA wall. A dependence of these disorders on cholesterol and glucose levels in blood serum, overweight and Cushing syndrome, age, duration and activity of a disease, levels of ESR, C-reactive protein and white blood cells was not showed. Authors detected a correlation between the thickness of intima-media complex of CCA and hemostasis parameters.Key words: children, juvenile arthritis, systemic lupus erythematosus, intima-media complex, ultrasound diagnostics.(Voprosy sovremennoi pediatrii — Current Pediatrics. 2010;9(2:64-69

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

OpenAIRE

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

2014-01-01

Background Juvenile idiopathic arthritis is a relatively common chronic disease of childhood, and is associated with persistent morbidity and extra-articular complications, one of the most common being uveitis. The introduction of biologic therapies, particularly those blocking the inflammatory mediator tumor necrosis factor-α, provided a new treatment option for juvenile idiopathic arthritis patients who were refractory to standard therapy such as non-steroidal anti-inflammatory drugs, corti...

Bone scintigraphy in systemic and metabolic diseases

International Nuclear Information System (INIS)

Engels, H.J.; Schmidt, H.A.E.

1984-01-01

Bone scintigraphy is a very sensitive method to identify pathological processes affecting the bone. Its specificity is, however, considerably lower than its sensitivity, particularly in systemic diseases. We therefore investigated the possibilities of differential diagnosis based on typical sites or patterns of distribution. The Paget syndrome with characteristic manifestation in the pelvic region, including crutch-shaped accumulation in the proximal femur, may be diagnosed by scintigraphy alone. If these typical sites are absent, however, differential diagnosis is difficult. Differential diagnosis for multiple myeloma, fibrous dysplasia, enchondromatosis, hyperparathyroidism, osteopathies, osteomalacia, inflammatory rheumatic diseases is also required and should be based on further examinations, taking into consideration the history, clinical signs and course. In this connexion scintigraphy is relevant both for early assessment and documentation of the spread of pathological processes and for the follow-up. (orig.) [de

Extramammary Paget’s Disease Versus Lichen Sclerosus

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Pappova T.

2016-12-01

Full Text Available Burning, itching and dyspareunia are typical symptoms of many genital diseases. These subjective complaints can be misdiagnosed because of different clinical presentations. We present a case report of a postmenopausal woman treated for genital warts over a period of three years followed by the development of leukoplastic lesions in the whitish area clinical classified as Lichen sclerosus (LS. Histology of this lesion revealed carcinoma in situ. After radical surgical removal, vulvar Paget’s disease was histologically verified. LS and extramammary Paget's disease (EMPD belong to a group of uncommon dermatoses which mainly affect the skin of the genitals in postmenopausal women. Ulceration, erosions and leukoplastic lesions can signalize the development of squamous cell carcinoma in association of lichen sclerosus, on the other hand, they can be the sign of EMPD after a long period of time using different topical agents. The importance of reaching the correct diagnosis is essential and can influence current patient investigations and invasive or non-invasive treatment.

Serum Cytokine Profiles in Children with Crohn’s Disease

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Ekaterina Vasilyeva

2016-01-01

Full Text Available Crohn’s disease (CD is a chronic inflammatory bowel disease that can be diagnosed at any age. There are two major patient groups based on diagnosis of this disease, before or after the age of 20 (juvenile/adolescent or adult, with disease progression in adults usually milder than in juvenile CD patients. Immune mechanisms have been suggested to play an important role in CD pathogenesis, with cytokines governing the development of the immune response. Upregulation of inflammatory cytokines in serum of juvenile and adult CD patients has been documented; still little is known about age-dependent differences in serum cytokine profiles of CD patients. We applied multiplex technology to analyze serum levels of 12 cytokines in juveniles and adults. We show that during the acute stage of the disease all CD patients have high serum levels of CXCL10, which remains upregulated during remission. Increased serum levels of TNF-α and IL-6 during the acute stage was characteristic of juvenile CD patients, whereas adult CD patients had upregulated levels of GM-CSF and IFN-γ. Taken together, these results demonstrate age-dependent differences in cytokine profiles, which may affect the pathogenesis of CD in patients at different ages of disease onset.

Effect of dietary carbohydrate on non-specific immune response, hepatic antioxidative abilities and disease resistance of juvenile golden pompano (Trachinotus ovatus).

Science.gov (United States)

Zhou, Chuanpeng; Ge, Xianping; Lin, Heizhao; Niu, Jin

2014-12-01

The present study was conducted to investigate the effects of dietary carbohydrate (CHO) levels on non-specific immune responses, hepatic antioxidative status and disease resistance of juvenile golden pompano. Fish were fed six isonitrogenous and isoenergetic diets containing various CHO levels for 8 weeks. After the feeding trial, fish were challenged by Vibrio harveyi and survival rate was recorded for the next 12 days. Plasma total protein and albumin content, respiratory burst activity, alkaline phosphatase, slightly increased with dietary starch level from 0% to 16.8%, but significantly decreased at dietary starch levels of 16.8%-28%. Plasma lysozyme, complement 3 and complement 4 levels increased with increasing dietary carbohydrate up to 11.2% and then declined (P 0.05) with those of the other treatments. After challenge, fish fed 11.2% and 16.8% dietary CHO showed higher survival rate than that of fish in 0% CHO group (P 0.05). The results of this study suggest that ingestion of 11.2-16.8% dietary CHO can enhance the non-specific immune responses, increase the hepatic antioxidant abilities, and improve resistance to V. harveyi infection of juvenile golden pompano. Copyright © 2014 Elsevier Ltd. All rights reserved.

Facts a New Patient Needs to Know about Paget's Disease of Bone

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... important, so some doctors recommend that children and siblings of a person with Paget’s disease be tested ... most up-to-date (accurate) information available. Occasionally, new information on medication is released. For updates and ...

Juvenile polyposis syndrome

OpenAIRE

Hsiao, Yi-Han; Wei, Chin-Hung; Chang, Szu-Wen; Chang, Lung; Fu, Yu-Wei; Lee, Hung-Chang; Liu, Hsuan-Liang; Yeung, Chun-Yan

2016-01-01

Abstract Background: Juvenile polyposis syndrome, a rare disorder in children, is characterized with multiple hamartomatous polyps in alimentary tract. A variety of manifestations include bleeding, intussusception, or polyp prolapse. In this study, we present an 8-month-old male infant of juvenile polyposis syndrome initially presenting with chronic anemia. To the best of our knowledge, this is the youngest case reported in the literature. Methods: We report a rare case of an 8-month-old male...

ECHOGRAPHY POTENTIAL IN DIAGNOSTICS OF THE KNEE JOINT IMPAIRMENT IN THE EVENT OF THE JUVENILE ARTHRITIDES

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N.V. Osipova

2008-01-01

Full Text Available The juvenile rheumatoid arthritis (JRA is one of the most widely spread rheumatic diseases among children, which is characterized by the steady progressive run, rapidly leading to the disability. Among numerous JRA manifestations, one of the leading is the joint syndrome with children mostly suffering from the knee joint impairment. With JRA, the initial changes in the joints affect the joint tissues and articular cartilage, while the bone changes evolve at the late stages of the disease. As a result, the echography plays an important role in the early arthritides diagnostics if compared to the x ray study and computerized tomography. To identify the typical features of the knee joint impairment, 97 children with JRA and 16 children with the juvenile spondyl arthritis (JSA underwent the high pitched linear transducer assisted echography. The overwhelming majority of patients showed the changes in the hyaline cartilage. The hyperechoic enlargements in the depth of the cartilage were typical of JRA, while the cartilage thinning was typical of JSA. About a half of the patients showed the effusion. Over a third of the patients showed the changes in the cortical layer of the articular bone surface.Key words: juvenile rheumatoid arthritis, juvenile spondyl arthritis, diagnostics, knee joint echography, children.

Function of the parotid gland in juvenile recurrent parotitis: a case series.

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Xie, Li-song; Pu, Yi-ping; Zheng, Ling-yan; Yu, Chuang-qi; Wang, Zhi-jun; Shi, Huan

2016-04-01

Our aim was to find out how the parotid gland functions in 44 patients with juvenile recurrent parotitis, and to assess the value of measuring the serum amylase activity. Clinical and personal details were recorded, and all patients had their serum amylase activity measured together with sialography during the chronic phase. The function of the gland was classified by sialographic images. The chi square test and Spearman's rank correlation coefficient were used in the statistical analyses. There was a significant association between the degree of glandular function and serum amylase activity (p=0.014). The patients with unilateral and bilateral disease differed significantly in their degree of glandular function (p=0.020), those with bilateral disease having poorer function. There were no significant correlations between other clinical variables and glandular function. Serum amylase activity is an important diagnostic variable in juvenile recurrent parotitis, and poor parotid function reflects the severity of the disease. Copyright © 2016 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

Risk factors for aggressive recurrent respiratory papillomatosis in adults and juveniles.

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Turid Omland

Full Text Available In this cohort study we examined whether gender, age at onset, observation time or human papillomavirus (HPV genotype are risk factors for an aggressive clinical course in Recurrent Respiratory Papillomatosis (RRP. Clinical data from patient records comprised gender, age at onset, date of first endolaryngeal procedure with biopsy, date of last follow-up, total number of endolaryngeal procedures, and complications during the observation period. Disease was defined as juvenile (JoRRP or adult onset (AoRRP according to whether the disease was acquired before or after the age of 18. Aggressive disease was defined as distal spread, tracheostomy, four surgical operations annually or >10 surgeries in total. DNA was extracted from formalin-fixed paraffin-embedded tissue. HPV genotyping was performed by quantitative PCR assay identifying 15 HPV genotypes. The study included 224 patients. The majority were males (141/174 in AoRRPs and 31/50 in JoRRPs; p = 0.005. The median follow-up from initial diagnosis was 12.0 years (IQR 3.7-32.9 for JoRRPs and 4.0 years (IQR 0.8-11.7 for AoRRPs. The disease was more aggressive in juveniles than adults (p10 years (OR = 13.41, 95% CI [5.46, 32.99[, p10 years were risk factors for an aggressive disease course in AoRRPs.

Chest Abnormalities in Juvenile-Onset Mixed Connective Tissue Disease: Assessment with High-Resolution Computed Tomography and Pulmonary Function Tests

International Nuclear Information System (INIS)

Aaloekken, T.M.; Mynarek, G.; Kolbenstvedt, A.; Lilleby, V.; Foerre, Oe.; Soeyseth, V.; Pripp, A.H.; Johansen, B.

2009-01-01

Background: Mixed connective tissue disease (MCTD) is associated with several chest manifestations. Only a few studies have focused on chest manifestations in juvenile-onset MCTD (jMCTD), and the true prevalence of pulmonary abnormalities on high-resolution computed tomography (HRCT) in these patients is unknown. Purpose: To investigate the occurrence of pulmonary abnormalities in jMCTD with particular reference to interstitial lung disease (ILD), and to evaluate a possible association between pulmonary findings and disease-related variables. Material and Methods: Twenty-four childhood-onset MCTD patients with median disease duration of 10.5 years (range 1-21 years) were investigated in a cross-sectional study by means of HRCT, pulmonary function tests (PFT), and clinical assessment. Results: Discrete ILD was identified in six patients (25%). Median extent of ILD was 2.0%, and all except one of the patients had very mild disease in which 5% or less of the parenchyma was affected. The CT features of fibrosis were mainly microcystic and fine intralobular. The most frequently abnormal PFT was carbon monoxide uptake from the lung, which was abnormal in 33% of the patients. PFT and disease duration were not significantly associated with HRCT findings of ILD. Conclusion: The prevalence of ILD in childhood-onset MCTD patients was lower than previously believed. In most of the patients with ILD, the findings were subtle and without clinical correlation. The results suggest a low extent of ILD in childhood-onset MCTD, even after long-term disease duration

Social and psychological aspects of criminal juvenile justice in the world practice (Anglo-Saxon model of juvenile justice

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D.S. Oshevsky

2013-10-01

Full Text Available The article is the final part of the review of existing foreign models of juvenile criminal justice system. We analyze the principles of juvenile justice in the criminal trial: protective orientation, personalization and social richness of the trial, the emphasis on educational influences. We present the foreign experience of incorporating social, psychological and clinical special knowledge into specialized justice concerning juvenile offenders. We analyze modern trends in the development of juvenile justice in the United States and Canada. We present material related to methods of risk assessment of re-offending among adolescents. We highlight approaches to complex long-term follow-up of juvenile offenders in Anglo-Saxon juvenile justice. We describe some aspects of the probation service using the method of case management. In the context of the accepted “National Strategy for Action for the Benefit of Children for 2012-2017”, the prospects for the development of specialized criminal justice for young offenders in the Russian Federation are discussed

Acupuntura em adolescentes com fibromialgia juvenil Acupuntura en adolescentes con fibromialgia juvenil Acupuncture in adolescents with juvenile fibromyalgia

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Marialda Höfling P. Dias

2012-01-01

Full Text Available 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. Durante o estudo, os pacientes puderam usar analgésicos, amitriptilina e foram orientados a praticar atividade física aeróbica. Os resultados antes e após acupuntura foram comparados pelo teste não paramétrico de Wilcoxon. RESULTADOS: Dos 38 pacientes com fibromialgia juvenil acompanhados em oito anos consecutivos, 13 tinham todas as informações nos prontuários e nas fichas de acupuntura e foram avaliados. Destes 13, sete obtiveram melhora nos três parâmetros analisados (número de pontos dolorosos, EVA de dor e IM. As medianas do número de pontos dolorosos e da EVA de dor foram significativamente maiores antes do tratamento quando comparados ao final do tratamento com as sessões de acupuntura [14 (11-18 versus 10 (0-15, p=0,005; 6 (2-10 versus 3 (0-10, p=0,045; respectivamente]. Em contraste, a mediana do IM foi significativamente menor antes do tratamento [3,4 (2,49-4,39 versus 4,2 (2,71-5,99, p=0,02]. Nenhum dos pacientes com fibromialgia juvenil apresentou eventos adversos associados à acupuntura. CONCLUSÕES: Acupuntura é uma modalidade de Medicina Tradicional Chinesa que pode ser utilizada nos pacientes pediátricos com fibromialgia. Futuros estudos controlados serão necessários.OBJETIVO: Describir el uso de acupuntura en adolescentes con fibromialgia juvenil. MÉTODOS: Estudio retrospectivo realizado en pacientes con fibromialgia juvenil (criterios del Colegio Americano de Reumatología sometidos a al menos 11 sesiones

Synovial and inflammatory diseases in childhood: role of new imaging modalities in the assessment of patients with juvenile idiopathic arthritis

International Nuclear Information System (INIS)

Damasio, Maria Beatrice; Malattia, Clara; Martini, Alberto; Toma, Paolo

2010-01-01

Juvenile idiopathic arthritis (JIA) represents a group of heterogeneous diseases characterized by a chronic inflammatory process primarily targeting the synovial membrane. A persistent synovitis is associated with an increased risk of osteocartilaginous damage. With the advent of effective structure-modifying treatment for JIA, it may be possible to significantly reduce or even completely prevent structural damage and associated functional disability. The trend towards early suppression of inflammation, in order to prevent erosive disease, shifts the emphasis away from conventional radiographic detectable structural damage to the slightest traces of early joint damage, and drives the need for alternative imaging techniques more sensitive in detecting early signs of disease activity and damage. In this regard MRI and US are playing an increasing role in the evaluation of arthritic joints. This article will review the key aspects of the current status and recent important advances of imaging techniques available to investigate the child with rheumatic disease, briefly discussing conventional radiography, and particularly focusing on MRI and US. In this era of advancing imaging technology, knowledge of the relative values of available imaging techniques is necessary to optimize the management of children with JIA. (orig.)

Yellow Fever Vaccine in Patients With Rheumatic Diseases

Science.gov (United States)

2018-04-05

Systemic Lupus; Rheumatoid Arthritis; Spondyloarthritis; Inflammatory Myopathy; Systemic Sclerosis; Mixed Connective Tissue Disease; Takayasu Arteritis; Granulomatosis With Polyangiitis; Sjogren's Syndrome; Juvenile Idiopathic Arthritis; Juvenile Dermatomyositis

Non-HLA gene polymorphisms in juvenile idiopathic arthritis: associations with disease outcome.

Science.gov (United States)

Alberdi-Saugstrup, M; Enevold, C; Zak, M; Nielsen, S; Nordal, E; Berntson, L; Fasth, A; Rygg, M; Müller, K

2017-09-01

To test the hypothesis that non-HLA single-nucleotide polymorphisms (SNPs) associated with the risk of juvenile idiopathic arthritis (JIA) are risk factors for an unfavourable disease outcome at long-term follow-up. The Nordic JIA cohort is a prospective multicentre study cohort of patients from the Nordic countries. In all, 193 patients met the inclusion criteria of having an 8 year follow-up assessment and available DNA sample. Seventeen SNPs met the inclusion criteria of having significant associations with JIA in at least two previous independent study cohorts. Clinical endpoints were disease remission, actively inflamed joints and joints with limitation of motion (LOM), articular or extra-articular damage, and history of uveitis. Evidence of associations between genotypes and endpoints were found for STAT4, ADAD1-IL2-IL21, PTPN2, and VTCN1 (p = 0.003-0.05). STAT4_rs7574865 TT was associated with the presence of actively inflamed joints [odds ratio (OR) 20.6, 95% confidence interval (CI) 2.2-> 100, p = 0.003] and extra-articular damage (OR 7.9, 95% CI 1-56.6, p = 0.057). ADAD1_rs17388568 AA was associated with a lower risk of having joints with LOM (OR 0.1, 95% CI 0-0.55, p = 0.016). PTPN2_rs1893217 CC was associated with a lower risk of having joints with LOM (OR 0.2, 95% CI 0-0.99, p = 0.026), while VTCN1_rs2358820 GA was associated with uveitis (OR 3.5, 95% CI 1-12.1, p = 0.029). This exploratory study, using a prospectively followed JIA cohort, found significant associations between long-term outcome and SNPs, all previously associated with development of JIA and involved in immune regulation and signal transduction in immune cells.

Síndrome da ativação do macrófago em paciente com artrite idiopática juvenil poliarticular Macrophage activation syndrome in a patient with polyarticular juvenile idiopathic arthritis

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Acir Rachid

2004-10-01

Full Text Available A linfohistiocitose hemofagocítica caracteriza-se por ativação e proliferação excessiva de linfócitos e macrófagos. Quando associada à artrite idiopática juvenil é também conhecida por "síndrome de ativação do macrófago", sendo uma complicação potencialmente fatal desta doença. Apresentamos o caso de uma mulher de 26 anos portadora de artrite idiopática juvenil (poliartrite, fator reumatóide negativo, com diagnóstico aos 13 anos, em uso de antiinflamatórios não esteroidais (diclofenaco, nimesulide. Admitida com quadro de resposta inflamatória sistêmica, febre, linfonodomegalia, esplenomegalia, anemia, trombocitopenia, hipofibrinogenemia, hiperferritinemia, hipertrigliceridemia e achados de hematofagocitose na medula óssea. Os autores discutem aspectos relacionados com a patogênese, diagnóstico e tratamento desta doença pouco conhecida.Hemophagocytic lymphohistiocytosis is characterized by massive lymphocyte and macrophage activation and proliferation. When observed in association with juvenile idiopathic arthritis it is also called "macrophage activation syndrome" being a potentially lethal complication of this disease. We report the case of a 26 years old woman with juvenile idiopathic arthritis (polyarthritis, rheumatoid factor negative since 13 years old, receiving nonsteroidal anti-inflammatory drugs (diclofenac, nimesulide. She was admitted with systemic inflammatory response, fever, lymph node enlargement, splenomegaly, anemia, thrombocytopenia, hypofibrinogenemia, hyperferritinemia, hypertriglyceridemia and bone marrow hemophagocytosis. Aspects related to pathogenesis, diagnosis and treatment of this little known disease are discussed.

Modulation of growth performance, immunological responses and disease resistance of juvenile Nile tilapia (Oreochromis niloticus (Linnaeus, 1758 by supplementing dietary inosine monophosphate

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Md. Abdul Kader

2018-05-01

Full Text Available This study was investigated to examine supplemental effects of dietary inosine monophosphate (IMP on growth performance, feed utilization, biochemical, hematological and immunological parameters of juvenile Nile tilapia Oreochromis niloticus. Disease resistance to experimental infection with Streptococcus agalactiae was also assessed. A semi-purified basal diet was supplemented with 0 (IMP0, Control, 1 (IMP1, 2 (IMP2, 4 (IMP4 and 8 (IMP8 g purified IMP kg−1 diet to formulate five experimental diets. Each diet was randomly allocated to triplicate groups of fish (0.59 g for 60 days. The results indicated that supplementation of IMP significantly (P  0.05. Among whole body proximate composition and somatic parameters, condition factor was significantly influenced by dietary supplementation of IMP. A wide variation in hematological parameters were observed and dietary supplementation increased the hematocrit content (P  0.05. Total serum protein (TSP, lysozyme activity (LA, superoxide dismutase activity (SOD and bactericidal activity (BA tended to increase with the supplementation of dietary IMP. TSP and SOD were significantly improved with ≥4 g kg−1 supplementation, while LA with 8 g kg−1 and BA with ≥1 g kg−1 supplementations. IMP supplemented groups showed higher (P > 0.05 cumulative survival compared to that of supplementation free control group. IMP supplemented diet groups also showed significantly higher BA in the post challenge test. Based on the overall performances, the results of the current study indicated that the inclusion of IMP in Nile tilapia diet can improve growth performance, feed utilization, haematological and immunological parameters; and disease resistance of juvenile Nile tilapia. Keywords: Nucleotides, Inosine monophosphate, Growth, Immunity, Disease resistance, Nile tilapia, Streptococcus agalactiae

Clinical Case of Tocilizumab Use in a Patient with Systemic Juvenile Idiopathic Arthritis

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Y. M. Spivakovskiy

2015-01-01

Full Text Available The article presents a case of using genetically engineered biopharmaceutical tocilizumab in a child with systemic juvenile idiopathic arthritis. On the initial stage, the treatment was characterized by resistance to high doses of glucocorticoids and cytostatic drugs. Successful termination of visceral and articular manifestations of systemic juvenile idiopathic arthritis and normalization of laboratory indicators of disease activity in the setting of use of interleukin 6 receptor blocker were described. We observed stable improvement of the child’s condition during a year-long follow-up in the setting of the selected anti-inflammatory therapy pattern. 

Juvenile dispersal in Calomys venustus (Muridae: Sigmodontinae)

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Priotto, José; Steinmann, Andrea; Provensal, Cecilia; Polop, Jaime

2004-05-01

Both spacing behaviour and dispersal movement are viewed as hierarchical processes in which the effects may be expressed at spatial scale. This research was carried out to examine the hypothesis that the presence of parents promotes the dispersal of juveniles from their natal nest and their father or mother home-range, in Calomys venustus.The study was carried out in four 0.25 ha fences (two controls and two experimentals), in a natural pasture. This study had two periods: Father Removal (FR) (August and December 1997; year one) and Mother Removal (MR) (August 1998 and January 1999; year two). For the FR treatment fathers were removed after juveniles were born, but in the MR treatment mothers were removed after the juveniles were weaned. The effect of parents on the dispersal distance of juveniles was analysed with respect to their natal nest and their mother and father home-range. Dispersal distance from the nest of C. venustus was independent of either male or female parent. Juveniles were more dispersing in relation to the centre of activity of their mothers than to that of their fathers, and females were more dispersing than males. Female juveniles overlap their home-range with their parents less than male juveniles do. The differences observed between female and male juveniles would be related to their different sexual maturation times, as well as to the female territoriality.

Essential habitat for sardine juveniles in Iberian waters

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Sílvia Rodríguez-Climent

2017-09-01

Full Text Available In a period when the Iberian sardine stock abundance is at its historical minimum, knowledge of the sardine juvenile’s distribution is crucial for the development of fishery management strategies. Generalized additive models were used to relate juvenile sardine presence with geographical variables and spawning grounds (egg abundance and to model juvenile abundance with the concurrent environmental conditions. Three core areas of juvenile distribution were identified: the Northern Portuguese shelf (centred off Aveiro, the coastal region in the vicinity of the Tagus estuary, and the eastern Gulf of Cadiz. Spatial differences in the relationship between juvenile presence and egg abundances suggest that essential juvenile habitat might partially differ from the prevailing spawning grounds. Models also depicted significant relationships between juvenile abundance, temperature and geographical variables in combination with salinity in the west and with zooplankton in the south. Results indicate that the sardine juvenile distribution along the Iberian Peninsula waters are an outcome of a combination of dynamic processes occurring early in life, such as egg and larva retention, reduced mortality and favourable feeding grounds for both larvae and juveniles.

[Asu(1,7)E-CT, an analog of eel calcitonin. A comparative study in man with reference to other synthetic calcitonins].

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Caniggia, A; Nuti, R; Vattimo, A; Galli, M; Turchetti, V; Franci, B; Martorelli, T; Righi, G

1983-04-28

(Asu) E-CT is a deaminodicarba-analog of the synthetic eel-calcitonin (E-CT) that shows specific activity and the potency reasonably high in comparison with that of the most potent natural hormone. The structure of its molecule indicates that the disulphide bond in calcitonins is not essential for the biological activity but only for the maintenance of the specific conformation by forming an intramolecular bridge. The instability of calcitonins should mainly be attributed to the presence of the disulfide bond and (Asu)E-CT proved to be more stable "in vitro" than native calcitonins. The more prolonged hypocalcemic effect of E-CT and its aminosuberic analog (Asu)E-CT has been accounted for to a greater stability of and persistence at the receptor site. (Asu) E-CT has been largely studied in Japan on experimental animals and successfully used in the treatment of hypercalcemia in man. On the contrary investigations on human administration of this analog are very scarce. The present paper reports studies carried out in normal subjects and Paget's disease patients to investigate the effects of (Asu)E-CT in man in comparison with the effects of synthetic human calcitonin (H-CT) and synthetic salmon calcitonin (S-CT). Two different experimental procedures have been used: 1) rapid intravenous injection of (Asu)E-CT (80 MRC. U.) or respectively of H-CT and S-CT (100 MRC. U.) in 15 subjects (7 normals and 8 with Paget's disease); 2) slow 7 days continuous subcutaneous infusion of similar daily amounts of (Asu)E-CT, H-CT and S-CT administered by a microjet pump device in 21 subjects (7 normals and 14 with Paget's disease). The intravenous administration of (Asu)E-CT induced a rapid and persistent decrease in total plasma calcium, ionized calcium and plasma phosphate that was more evident in Paget's disease patients than in normal subjects. No clearly cut differences have been observed with the hypocalcemic and hypophosphatemic effect of H-CT and S-CT administered

Juvenile offenders: competence to stand trial.

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Soulier, Matthew

2012-12-01

This article details the legal background and assists the reader in the preparation and practical conduct of evaluations regarding juvenile adjudicative competency. The material is presented to be useful as a guide to direct questions of competency and covers aspects of evaluation that include: legal standard for competency to stand trial, developmental immaturity, current practice in juvenile competency to stand trial, forensic evaluation of juvenile competency to stand trial, organizing the evaluation, collateral sources of information, psychiatric evaluation of juvenile adjudicative competency, assessment of mental disorder and intellectual disability, assessment of developmental status, assessment of functional abilities for adjudicative competence, and reaching the forensic opinion. Copyright © 2012 Elsevier Inc. All rights reserved.

[Morphea or juvenile localised scleroderma: Case report].

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Strickler, Alexis; Gallo, Silvanna; Jaramillo, Pedro; de Toro, Gonzalo

2016-01-01

Morphea or juvenile localised scleroderma (JLS) is an autoimmune, inflammatory, chronic, slowly progressive connective tissue disease of unknown cause that preferably affects skin and underlying tissues. To report a case of Juvenil Localised scleroderma in an 8-year old girl, contributing to an early diagnosis and treatment. The case is presented of an 8 year-old girl who presented with indurated hypopigmented plaques, of linear distribution in the right upper extremity of two years onset, together with papery texture hyperpigmented indurated plaques with whitish areas of thinned skin in right lower extremity, and leg and ankle swelling. The clinical features and diagnostic tests, including histology were compatible with linear and pansclerotic JLS. She started with immunosuppressive therapy, physiotherapy, and occupational therapy. We report a case of linear and pansclerotic ELJ type, in which there was a 2 year delay in diagnosis, however the response to treatment was positive as expected. Copyright © 2016 Sociedad Chilena de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.

Physiotherapy in pauciarticular juvenile idiopathic arthritis. Case study.

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Zuk, Beata; Kaczor, Zofia; Zuk-Drążyk, Berenika; Księżopolska-Orłowska, Krystyna

2014-01-01

Juvenile idiopathic arthritis (JIA) is the most common arthropathy of childhood and adolescence. This term encompasses a group of chronic systemic inflammatory diseases of the connective tissue which cause arthritis in patients under 16 years of age lasting at least 6 weeks. The authors presented the characteristic features of physiotherapy based on functional examination results on the basis of two cases of girls with pauciarticular JIA treated according to an established pharmacological regimen. Physiotherapy should be introduced at an early stage of the disease. Kinesiotherapy preceded by history-taking and a functional examination of the patient, has to focus on both primary and secondary joint lesions.

EFFECTIVENESS OF INFLIXIMAB IN PATIENTS WITH JUVENILE ANKYLOSING SPONDYLARTHRITIS

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A.L. Kozlova

2009-01-01

Full Text Available The objective of open-labeled study was estimation of effectiveness and safety of infliximab — monoclonal antibodies to tumor necrosis factor (TNF - in treatment of 48 patients with juvenile ankylosing spondylarthritis. Duration of observation was 6 weeks — 1,5 years. Anticytokine treatment was administrated on the ground of therapy with immunosuppressive agents in 96% of patients. Infliximab was administrated in median dose 7,4 ± 3,7 mg/kg of body weight by standard scheme (0–2–6 — week and further every 8 weeks intravenously. Results of a trial showed that infliximab has evident anti-inflammatory effect. Treatment with infliximab provided development of remission of articular syndrome, decreasing and normalization of laboratory indices of activity of disease, the rate of disability, and increasing of quality of life in 84% of patients. Effect of this medication was registered after first infusion and continued during all period of follow up. Adverse effects included transfusion reactions: fever, head ache, nausea/vomiting in 10% of patients, allergic arthritis — in 2% of patients. Thus, treatment of infliximab is pathogenetically grounded, effective and safe in patients with juvenile ankylosing spondylarthritis.Key words: children, juvenile ankylosing spondylarthritis, infliximab, treatment.(Voprosy sovremennoi pediatrii — Current Pediatrics. 2009;8(2:20-26

Sexuality education groups in juvenile detention.

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Farrow, J A; Schroeder, E

1984-01-01

Several major studies have described the magnitude and character of adolescent sexual activity and sexual knowledge related to contraception and sexually transmitted diseases (Diepold & Young, 1979; Hass, 1979; Sorenson, 1973; Zelnick & Kantner, 1980). Few systematic studies have been conducted, however, which analyze the attitudes toward sexuality and contraception of delinquent adolescents who are generally school dropouts and who may engage in socially unacceptable behaviors such as running away, drug abuse, and prostitution. Delinquent youths, especially delinquent girls, have been characterized as being more sexually active and less sexually knowledgeable than their nondelinquent peers (Gibbon, 1981; Mannarino & Marsh, 1978). Despite the assumed high-risk nature of this delinquent population, few juvenile detention facilities have offered systematically evaluated coeducational sex education programs. One barrier to implementation of such programs in juvenile detention centers is the lack of a treatment or program orientation of most staff, and/or staff denial of adolescent sexuality in general, an attitude which suppresses the development of healthier sexual values and often promotes pathologic sexual interaction within institutions (Shore & Gochros, 1981). A recent survey of adolescent sexuality (Diepold, 1979) points out that teenagers' feelings about their "sexual selves" impacts greatly upon their general self-image. Low self-esteem is more frequently found among delinquents than nondelinquents (Jones & Swain, 1977; Lund & Salury, 1980), and treatment for delinquent girls often focuses on increasing self-esteem and developing assertiveness skills based on feelings of self-worth (DeLange, Lanahan, & Barton, 1981; NiCarthy, 1981). Two studies carried out with juvenile detainees from a large urban center confirmed that sexual activity among delinquent adolescents is significantly greater than that of the general adolescent population, and that the delinquents

Parenting and juvenile delinquency

OpenAIRE

Hoeve, M.

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 ...

Epidemiology and treatment of extramammary Paget disease in the Netherlands.

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Siesling, S; Elferink, M A G; van Dijck, J A A M; Pierie, J P E N; Blokx, W A M

2007-10-01

To determine the incidence of EMPD and to describe its epidemiology, treatment, survival and the risk of developing other malignancies. All cases of EMPD, diagnosed between 1989 and 2001, were selected from the Netherlands Cancer Registry. In total, 178 cases of invasive and 48 cases of in situ EMPD had been registered. The overall relative 5-year survival for invasive tumours was 72%. Most patients with invasive as well as in situ cancer underwent surgery. Other malignancies were found in 32% of patients with invasive EMPD and 35% of patients with in situ EMPD. Patients had an increased risk of developing a second primary cancer (standardized incidence ratio: 1.7; 95% confidence interval 1.2-2.4). The most frequent localizations of the other cancers were the colorectum, the prostate, the breast and the extragenital skin. For EMPD, which is a rare disease in the Netherlands, there are no clear diagnostic and treatment guidelines. The prognosis is fairly good. A thorough search for other tumours is recommended for these patients.

Juvenile Prostitution.

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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)

A rare case of juvenile dermatomyositis and review of literature

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Anjali T Bharani

2017-01-01

Full Text Available Idiopathic inflammatory myopathies are rare group of systemic connective tissue diseases. The hallmark of these disorders is symmetrical chronic inflammation and weakness of proximal muscles. Juvenile dermatomyositis (JDM is the most common inflammatory myositis in children. We describe a rare case of JDM in a 4-year-old female child who presented with characteristic cutaneous rash and proximal muscle weakness.

Periodontal aspects of the juvenile form of paracoccidioidomycosis

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MIGLIARI Dante A.

1998-01-01

Full Text Available Three cases of the juvenile form of paracoccidioidomycosis are reported. Emphasis has been given to the oral manifestations, particularly the periodontal involvement. The main periodontal findings were: generalized and progressive alveolar bone destruction leading to gingival recession with exposure of the tooth roots, and spontaneous tooth losses. The gingival mucosa was predominantly smooth, erithematous and slightly swollen. These aspects, although rare, may be the earliest signs of the disease and sometimes its only manifestation.

Imaging in juvenile idiopathic arthritis with a focus on ultrasonography

DEFF Research Database (Denmark)

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

2013-01-01

Early therapeutic intervention and use of new highly efficacious treatments have improved the outcome in many patients with juvenile idiopathic arthritis (JIA), but have also led to the need for more precise methods to evaluate disease activity. In adult rheumatology, numerous studies have...... US studies have been conducted. Sonographic assessment of disease activity has, however, been proven to be more informative than clinical examination and is also readily available at points of care. This review summarises the literature on imaging in JIA, focusing on US and the important role...

Methotrexate therapy may prevent the onset of uveitis in juvenile idiopathic arthritis.

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Papadopoulou, Charalampia; Kostik, Mikhail; Böhm, Marek; Nieto-Gonzalez, Juan Carlos; Gonzalez-Fernandez, Maria Isabel; Pistorio, Angela; Martini, Alberto; Ravelli, Angelo

2013-09-01

To evaluate whether early treatment with methotrexate (MTX) prevents the onset of uveitis in children with juvenile idiopathic arthritis. The clinical charts of all consecutive patients seen between January 2002 and February 2011 who had a disease duration uveitis had occurred. A total of 254 patients with a median disease duration of 0.3 year were included. Eighty-six patients (33.9%) were treated with MTX, whereas 168 patients (66.1%) did not receive MTX. During the 2-year follow-up, 211 patients (83.1%) did not develop uveitis, whereas 43 patients (16.9%) had uveitis a median of 1.0 year after the first visit. The frequency of uveitis was lower in MTX-treated than in MTX-untreated patients (10.5% vs 20.2%, respectively, P = .049). Survival analysis confirmed that patients treated with MTX had a lower probability of developing uveitis. Early MTX therapy may prevent the onset of uveitis in children with juvenile idiopathic arthritis. Because our study may be affected by confounding by indication, the potential of MTX to reduce the incidence of ocular disease should be investigated in a randomized controlled trial. Copyright © 2013 Mosby, Inc. All rights reserved.

COMPUTED TOMOGRAPHIC ANATOMY AND CHARACTERISTICS OF RESPIRATORY ASPERGILLOSIS IN JUVENILE WHOOPING CRANES.

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Schwarz, Tobias; Kelley, Cristin; Pinkerton, Marie E; Hartup, Barry K

2016-01-01

Respiratory diseases are a leading cause of morbidity and mortality in captivity reared, endangered whooping cranes (Grus americana). Objectives of this retrospective, case series, cross-sectional study were to describe computed tomography (CT) respiratory anatomy in a juvenile whooping crane without respiratory disease, compare CT characteristics with gross pathologic characteristics in a group of juvenile whooping cranes with respiratory aspergillosis, and test associations between the number of CT tracheal bends and bird sex and age. A total of 10 juvenile whooping cranes (one control, nine affected) were included. Seven affected cranes had CT characteristics of unilateral extrapulmonary bronchial occlusion or wall thickening, and seven cranes had luminal occlusion of the intrapulmonary primary or secondary bronchi. Air sac membrane thickening was observed in three cranes in the cranial and caudal thoracic air sacs, and air sac diverticulum opacification was observed in four cranes. Necropsy lesions consisted of severe, subacute to chronic, focally extensive granulomatous pathology of the trachea, primary bronchi, lungs, or air sacs. No false positive CT scan results were documented. Seven instances of false negative CT scan results occurred; six of these consisted of subtle, mild air sacculitis including membrane opacification or thickening, or the presence of small plaques found at necropsy. The number of CT tracheal bends was associated with bird age but not sex. Findings supported the use of CT as a diagnostic test for avian species with respiratory disease and tracheal coiling or elongated tracheae where endoscopic evaluation is impractical. © 2015 The Authors. Veterinary Radiology & Ultrasound published by Wiley Periodicals, Inc. on behalf of American College of Veterinary Radiology.

Extramammary Paget ’s disease Of Glans Penis: A Rare Case Report

LENUS (Irish Health Repository)

Inder, M S

2018-06-01

We present the case of an 83-year-old man with Extramammary Paget’s disease (EMPD) of the penis. He underwent a total penectomy and histopathology confirms the association of underlying invasive high grade urothelial carcinoma. Penile EMPD is rare and can be misinterpreted for benign skin conditions. A high index of suspicion is required for correct diagnosis and appropriate treatment.

Relationship of external fish condition to pathogen prevalence and out-migration survival in juvenile steelhead

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Hostetter, N.J.; Evans, A.F.; Roby, D.D.; Collis, K.; Hawbecker, M.; Sandford, B.P.; Thompson, D.E.; Loge, F.J.

2011-01-01

Understanding how the external condition of juvenile salmonids is associated with internal measures of health and subsequent out-migration survival can be valuable for population monitoring programs. This study investigated the use of a rapid, nonlethal, external examination to assess the condition of run-of-the-river juvenile steelhead Oncorhynchus mykiss migrating from the Snake River to the Pacific Ocean. We compared the external condition (e.g., body injuries, descaling, external signs of disease, fin damage, and ectoparasite infestations) with (1) the internal condition of a steelhead as measured by the presence of selected pathogens detected by histopathology and polymerase chain reaction analysis and (2) out-migration survival through the Snake and Columbia rivers as determined by passive integrated transponder (PIT) tag technology. The results from steelhead captured and euthanized (n = 222) at Lower Monumental Dam on the lower Snake River in 2008 indicated that external condition was significantly correlated with selected measures of internal condition. The odds of testing positive for a pathogen were 39.2, 24.3, and 5.6 times greater for steelhead with severe or moderate external signs of disease or more than 20% descaling, respectively. Capture-recapture models of 22,451 PIT-tagged steelhead released at Lower Monumental Dam in 2007-2009 indicated that external condition was significantly correlated with juvenile survival. The odds of outmigration survival for steelhead with moderate or severe external signs of disease, more than 20% descaling, or severe fin damage were 5.7, 4.9, 1.6, and 1.3 times lower, respectively, than those for steelhead without these external conditions. This study effectively demonstrated that specific measures of external condition were associated with both the internal condition and out-migration survival of juvenile steelhead. ?? American Fisheries Society 2011.

Juvenile rheumatoid arthritis

International Nuclear Information System (INIS)

Naz, S.; Mushtaq, A.; Bari, A.; Maqsud, A.; Khan, M. Z.; Ahmad, T. M.; Saira Rehman

2013-01-01

Objective: To determine the spectrum of clinical presentation, laboratory parameters and drug therapy in patients with Juvenile Rheumatoid Arthritis (JRA). Study Design: Case series. Place and Duration of Study: The Children's Hospital and The Institute of Child Health, Lahore, from October 2008 to October 2011. Methodology: All patients who fulfilled the American College of Rheumatology criteria for JRA were enrolled. Their clinical features, investigations done and treatment received for JRA were noted. Statistical analysis of data was done on SPSS version 16.0 for obtaining descriptive statistics. Results: Out of 185 patients, 50.3% (n = 93) were females; 54% (n = 100) were between 10 - 15 years of age. Polyarthritis was found in 71.9% (n = 133) followed by oligoarthritis (22.7%, n = 42) and systemic onset disease (5.4%, n = 10). Morning stiffness (78%) and fever (68%) were the most common clinical presentations. All patients with systemic onset disease had fever (n = 10) followed by skin rash, hepatosplenomegaly and lymphadenopathy. Uveitis was found in 2 patients, and both belonged to the oligoarticular group. Rheumatoid factor was found in 10.27% (n = 19) of all patients. All patients were given non-steroidal anti-inflammatory drugs (NSAIDs). Disease modifying agents (methotrexate) were given to 43.8% (n = 81). Steroids were used in 61% (n = 113) of patients either with NSAIDs alone or NSAIDs plus methotrexate. Conclusion: Disease profile of JRA at the study centre showed that polyarthritis is the commonest type. Recognition of subtypes will help in planning the management of these patients. (author)

Experience of the Tocilizumab Application in Systemic Onset Juvenile Arthritis

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A. V. Krasnopol’skaja

2015-01-01

Full Text Available The article provides information on the unfavourable course of systemic onset juvenile arthritis, resistant to immunosuppressive therapy with methotrexate in combination with cyclosporine, and pulse therapy with methylprednisolone and methotrexate. We describe the successful use of genetically engineered biological drug tocilizumab in the patient with systemic onset juvenile arthritis. After the first injection, pain was already significantly reduced; after the second, fever was relieved and non-steroid anti-inflammatory drugs were cancelled; after the third, lymphadenopathy and splenomegaly disappeared and the child’s functional activity improved significantly. After 12 months of treatment, an inactive phase of the disease was achieved, the joints’ kinetics (with the exception of the right hip were almost entirely restored and the patient’s quality of life had significantly improved. At the same time, metabolic disorders and changes in the cardiovascular system were reversed. This example demonstrated the high effectiveness of interleukin-6 antagonist tocilizumab in systemic arthritis, which allowed arresting joint affection as well as extra-articular manifestations of the disease, providing normal puberty, the restoration of growth and sexual development.

Imágenes juveniles, medios y nuevos escenarios

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Oscar Aguilera Ruiz

2015-01-01

Full Text Available Este artículo nace del análisis de los discursos radiales producidos exclusivamente para jóvenes en Santiago de Chile. Aborda además las categorías comprensivas de la vida juvenil, la vida juvenil de los años 90, el imaginario juvenil des-simbolizado, hacia una comprensión de lo juvenil, dinámica social propuesta por los medios y estrategias comunicacionales.

Hyperphosphatasia with massive osteoectasia: a 45-year follow-up

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McCarthy, E.F. [Division of Surgical Pathology, Department of Pathology and Orthopaedic Surgery, Baltimore, MD (United States); Sack, G.H. [Department of Internal Medicine, Baltimore, MD (United States)

2007-06-15

Hyperphosphatasia is a heterogeneous group of disorders characterized by a generalized skeletal disease and increased alkaline phosphatase. Increased bone remodeling secondary to increased osteoclastic activity appears to be the underlying feature of these disorders. These disorders include juvenile Paget's disease, expansile skeletal hyperphosphatasia, hyperostosis generalisata with striations, and Camurati-Engelmann's disease, type II. The genetic mutations for a number of these disorders have been identified. We present a patient with congenital hyperphosphatasia whose clinical and radiographic features were somewhat different from these other well-defined syndromes. The patient was followed for 45 years until his death of at age 49. The patient had massive osteoectasia with dense striations involving the entire shaft of his long bones. His spine, pelvis, short tubular bones, and calvarium were also involved. He suffered hearing loss and optic atrophy, but he kept his teeth throughout his life. He was tall with a marfanoid habitus, and he had hypogonadism and hypothyroidism. There was no evidence of mental retardation, and other laboratory studies where within normal limits. This case, as well as other manifestations of hyperphosphatasia, attests to the complexity of the bone remodeling system. (orig.)

Disturbance of the muscoloskeletal system in juvenile ankylosing spondylitis and disease developed in the adulthood (involvement of spine and sacroiliac joints

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Yehudina Ye.D.

2018-03-01

Full Text Available Background. Two forms of ankylosing spondylitis (AS are distinguished: juvenile and adult, depending on debut age of the disease. The diagnosis of juvenile AS (JAS is one of the most urgent problems in a pediatric rheumatology. The peculiarities of AS course that onsets in childhood and adulthood are manifested by differences in the nature of a spinal column disturbance. At the same time, the evolution of JAS in adulthood remains unexplored. The goals and objectives of research: to study clinical and X-ray symptoms of spondylopathy and sacroiliitis course, to assess their characteristics in the disease that onset in childhood and adulthood. Material and methods. 217 patients with AS (193 men and 24 women with an average age of 38 years were examined. The fast-progressing course of the disease was detected in 21% of cases, moderate and high degree of activity – in 79% of cases, the ІІ-ІІІ stage in 82%, and polyarthritis – in 65%. JAS was detected in 16% of cases (all boys, among them the third stage occurred twice more likely than among the other patients. Results. The clinical and radiologic signs of spondylopathy and sacroiliitis are observed in 95% and 97% of the total number of AS cases, respectively, among all patients with JAS lumbago was detected 4,3 times more frequently, sciatic muscles hypotrophy – 7,8 times, "the string symptom" - 2,9 times", the calcification of the spinal cord - 2,3 times, whereas the prevalence of spinal column injury, the severity of cervico-spondylopathy and sacroiliitis among patients with the disease debut in the adulthood is significantly greater, and the involvement in the process of the lumbar and thoracic spine are detected correspondingly twice as often and by 19%, occurrence of dorsalgia is 4 times as often, the limitation of body lateral bendover by 59%, while there are ambiguous dispersion-correlation links with extraarticular (systemic manifestations of the disease, and the high prevalence of

Subpopulations Within Juvenile Psoriatic Arthritis: A Review of the Literature

Directory of Open Access Journals (Sweden)

Matthew L. Stoll

2006-01-01

Full Text Available The presentation of juvenile psoriatic arthritis (JPsA has long been recognized to be clinically heterogeneous. As the definition of JPsA expanded to accommodate atypical manifestations of psoriasis in young children, studies began to reflect an increasingly clear biphasic distribution of age of onset, with peaks in the first few years of life and again in early adolescence. These two subpopulations differ in gender ratio, pattern of joint involvement, laboratory findings and potentially response to therapy. Intriguingly, a similar distribution of age of onset has been observed in juvenile rheumatoid arthritis (JRA, and correlates with patterns of HLA association. While a secure classification of subpopulations within JPsA awaits improved pathophysiologic understanding, future research must consider the possibility that different disease mechanisms may be operative in distinct subsets of patients with this disorder.

What Is Paget's Disease of Bone?

Science.gov (United States)

... Site Map Accessibility Privacy FOIA Disclaimer Glossary FAQs Digital Strategy Open Source Data Public Data Listing Download Adobe Reader Disclaimer Glossary FAQs Digital Strategy Open Source Data Public Data Listing Download Adobe ...

Pain and Paget's Disease of Bone

Science.gov (United States)

... usually in tablet form. Although there are many brand names for these drugs, they can be purchased ... mission of NIH ORBD~NRC is to expand awareness and enhance knowledge and understanding of the prevention, ...

Vegetative propagation of mature and juvenile northern red oak

Science.gov (United States)

James J. Zaczek; K. C. Steiner; C. W., Jr. Heuser

1993-01-01

Rooting trials were established to evaluate rooting success of cuttings from mature and juvenile, grafted and ungrafted northern red oak (NRO). Buds from 4 mature NRO ortets and juvenile seedlings were grafted onto juvenile and mature rootstock. Cuttings were collected from the grafts and from juvenile and mature shoots developed in situ and...

[New insights of myositis-specific and -associated autoantibodies in juvenile and adult type myositis].

Science.gov (United States)

Váncsa, Andrea; Dankó, Katalin

2016-07-01

Myositis, which means inflammation of the muscles, is a general term used for inflammatory myopathies. Myositis is a rare idiopathic autoimmune disease. It is believed that environmental factors such as virus, bacteria, parasites, direct injuries, drugs side effect can trigger the immune system of genetically susceptible individuals to act against muscle tissues. There are several types of myositis with the same systemic symptoms such as muscle weakness, fatigue, muscle pain and inflammation. These include dermatomyositis, juvenile dermatomyositis, inclusion-body myositis, polymyositis, orbital myositis and myositis ossificans. Juvenile and adult dermatomyositis are chronic, immune-mediated inflammatory myopathies characterized by progressive proximal muscle weakness and typical skin symptoms. The aim of the authors was to compare the symptoms, laboratory and serological findings and disease course in children and adult patients with idiopathic inflammatory myopathy. Early diagnosis and aggressive immunosuppressive treatment improve the mortality of these patients. Myositis-specific autoantibodies have predictive and prognostic values regarding the associated overlap disease, response to treatment and disease course. The authors intend to lighten the clinical and pathogenetic significance of the new target autoantigens. Orv. Hetil., 2016, 157(29), 1179-1184.

Interictal mood and personality disorders in temporal lobe epilepsy and juvenile myoclonic epilepsy.

Science.gov (United States)

Perini, G I; Tosin, C; Carraro, C; Bernasconi, G; Canevini, M P; Canger, R; Pellegrini, A; Testa, G

1996-01-01

BACKGROUND: Mood disorders have been described as the commonest psychiatric disorders in patients with temporal lobe epilepsy. Secondary depression in temporal lobe epilepsy could be interpreted either as an adjustment reaction to a chronic disease or as a limbic dysfunction. To clarify this issue, a controlled study of psychiatric disorders was conducted in different forms of epileptic and non-epileptic chronic conditions. METHODS: Twenty outpatients with temporal lobe epilepsy, 18 outpatients with juvenile myoclonic epilepsy--a primary generalised seizure disorder--20 matched type I diabetic patients, and 20 matched normal controls were assessed by a structured interview (SADS) and by self rating scales (Beck depression inventory (BDI) and the state and trait anxiety scales STAIX1 and STAIX2). RESULTS: Sixteen (80%) patients with temporal lobe epilepsy fulfilled the criteria for a psychiatric diagnosis at the SADS interview with a significantly higher frequency than patients with juvenile myoclonic epilepsy (22%) and diabetic patients (10%) (P personality or anxiety disorder. Patients with temporal lobe epilepsy scored significantly higher on BDI, STAIX1, and STAIX2 than the three control groups (P personality disorders, often in comorbidity, than patients with juvenile myoclonic epilepsy and diabetic patients suggesting that these psychiatric disorders are not an adjustment reaction to a chronic disease but rather reflect a limbic dysfunction. PMID:8971108

REFORMATIONS IN ZIMBABWE'S JUVENILE JUSTICE SYSTEM

African Journals Online (AJOL)

Mugumbate

1996-05-23

May 23, 1996 ... The article is based on a desk review of existing literature on juvenile crime in the country. ... that Zimbabwe's juvenile justice system is transforming from being ... recommendations include expanding the Pre-trial Diversion ...

Estimativa da herdabilidade do diabetes juvenil em uma população brasileira An estimate of the inheritability of juvenile diabetes in a Brazilian population

Directory of Open Access Journals (Sweden)

Calógeras A. de A. Barbosa

1984-10-01

Full Text Available A herdabilidade da suscetibilidade do diabetes juvenil em uma população brasileira é estimada baseando-se em informações da prevalência da doença na população em geral e entre os pais de afetados. A estimativa de 90%, então obtida, sugere uma alta participação de fatores genéticos na determinação da moléstia. Exemplifica-se a aplicação desse resultado no cálculo de risco de recorrência do diabetes em familiares de indivíduos afetados.The inheritability of liability to juvenile diabetes in a Brazilian population is estimated from parent-offspring correlation. The high value of the estimate, 0.9, suggests that genetic factors are the most important causative agents of the disease in this population.

Validation and clinical significance of the Childhood Myositis Assessment Scale for assessment of muscle function in the juvenile idiopathic inflammatory myopathies.

Science.gov (United States)

Huber, Adam M; Feldman, Brian M; Rennebohm, Robert M; Hicks, Jeanne E; Lindsley, Carol B; Perez, Maria D; Zemel, Lawrence S; Wallace, Carol A; Ballinger, Susan H; Passo, Murray H; Reed, Ann M; Summers, Ronald M; White, Patience H; Katona, Ildy M; Miller, Frederick W; Lachenbruch, Peter A; Rider, Lisa G

2004-05-01

To examine the measurement characteristics of the Childhood Myositis Assessment Scale (CMAS) in children with juvenile idiopathic inflammatory myopathy (juvenile IIM), and to obtain preliminary data on the clinical significance of CMAS scores. One hundred eight children with juvenile IIM were evaluated on 2 occasions, 7-9 months apart, using various measures of physical function, strength, and disease activity. Interrater reliability, construct validity, and responsiveness of the CMAS were examined. The minimum clinically important difference (MID) and CMAS scores corresponding to various degrees of physical disability were estimated. The intraclass correlation coefficient for 26 patients assessed by 2 examiners was 0.89, indicating very good interrater reliability. The CMAS score correlated highly with the Childhood Health Assessment Questionnaire (C-HAQ) score and with findings on manual muscle testing (MMT) (r(s) = -0.73 and 0.73, respectively) and moderately with physician-assessed global disease activity and skin activity, parent-assessed global disease severity, and muscle magnetic resonance imaging (r(s) = -0.44 to -0.61), thereby demonstrating good construct validity. The standardized response mean was 0.81 (95% confidence interval 0.53, 1.09) in patients with at least 0.8 cm improvement on a 10-cm visual analog scale for physician-assessed global disease activity, indicating strong responsiveness. In bivariate regression models predicting physician-assessed global disease activity, MMT remained significant in models containing the CMAS (P = 0.03) while the C-HAQ did not (P = 0.4). Estimates of the MID ranged from 1.5 to 3.0 points on a 0-52-point scale. CMAS scores corresponding to no, mild, mild-to-moderate, and moderate physical disability, respectively, were 48, 45, 39, and 30. The CMAS exhibits good reliability, construct validity, and responsiveness, and is therefore a valid instrument for the assessment of physical function, muscle strength, and

SPATACSIN mutations cause autosomal recessive juvenile amyotrophic lateral sclerosis.

Science.gov (United States)

Orlacchio, Antonio; Babalini, Carla; Borreca, Antonella; Patrono, Clarice; Massa, Roberto; Basaran, Sarenur; Munhoz, Renato P; Rogaeva, Ekaterina A; St George-Hyslop, Peter H; Bernardi, Giorgio; Kawarai, Toshitaka

2010-02-01

The mutation of the spatacsin gene is the single most common cause of autosomal recessive hereditary spastic paraplegia with thin corpus callosum. Common clinical, pathological and genetic features between amyotrophic lateral sclerosis and hereditary spastic paraplegia motivated us to investigate 25 families with autosomal recessive juvenile amyotrophic lateral sclerosis and long-term survival for mutations in the spatascin gene. The inclusion criterion was a diagnosis of clinically definite amyotrophic lateral sclerosis according to the revised El Escorial criteria. The exclusion criterion was a diagnosis of hereditary spastic paraplegia with thin corpus callosum in line with an established protocol. Additional pathological and genetic evaluations were also performed. Surprisingly, 12 sequence alterations in the spatacsin gene (one of which is novel, IVS30 + 1 G > A) were identified in 10 unrelated pedigrees with autosomal recessive juvenile amyotrophic lateral sclerosis and long-term survival. The countries of origin of these families were Italy, Brazil, Canada, Japan and Turkey. The variants seemed to be pathogenic since they co-segregated with the disease in all pedigrees, were absent in controls and were associated with amyotrophic lateral sclerosis neuropathology in one member of one of these families for whom central nervous system tissue was available. Our study indicates that mutations in the spatascin gene could cause a much wider spectrum of clinical features than previously recognized, including autosomal recessive juvenile amyotrophic lateral sclerosis.

Contrast-enhanced MRI compared with the physical examination in the evaluation of disease activity in juvenile idiopathic arthritis

International Nuclear Information System (INIS)

Hemke, Robert; Maas, Mario; Veenendaal, Mira van; Kuijpers, Taco W.; Dolman, Koert M.; Rossum, Marion A.J. van; Berg, J.M. van den

2014-01-01

To assess the value of magnetic resonance imaging (MRI) in discriminating between active and inactive juvenile idiopathic arthritis (JIA) patients and to compare physical examination outcomes with MRI outcomes in the assessment of disease status in JIA patients. Consecutive JIA patients with knee involvement were prospectively studied using an open-bore MRI. Imaging findings from 146 JIA patients were analysed (59.6 % female; mean age, 12.9 years). Patients were classified as clinically active or inactive. MRI features were evaluated using the JAMRIS system, comprising validated scores for synovial hypertrophy, bone marrow oedema, cartilage lesions and bone erosions. Inter-reader reliability was good for all MRI features (intra-class correlation coefficient [ICC] = 0.87-0.94). No differences were found between the two groups regarding MRI scores of bone marrow oedema, cartilage lesions or bone erosions. Synovial hypertrophy scores differed significantly between groups (P = 0.016). Nonetheless, synovial hypertrophy was also present in 14 JIA patients (35.9 %) with clinically inactive disease. Of JIA patients considered clinically active, 48.6 % showed no signs of MRI-based synovitis. MRI can discriminate between clinically active and inactive JIA patients. However, physical examination is neither very sensitive nor specific in evaluating JIA disease activity compared with MRI. Subclinical synovitis was present in >35 % of presumed clinically inactive patients. (orig.)

[Juvenile sterile granulomatous dermatitis and lymphadenitis in the dog].

Science.gov (United States)

Weingart, C; Eule, C; Welle, M; Kohn, B

2011-04-01

Juvenile sterile granulomatous dermatitis and lymphadenitis is a rare immune-mediated skin disease in young dogs. History, signalment, diagnostics, treatment, and outcome in 10 dogs are described. The age ranged from 8 - 36 weeks. The lymph nodes were enlarged in all dogs, especially the mandibular and prescapular lymph nodes. Systemic signs including fever were present in 8 dogs. Seven dogs suffered from blepharitis and painful edema of the muzzle with hemorrhagic discharge, pustules and papules. Cytology of pustules and lymph node aspirates revealed a pyogranulomatous inflammation. In 7 cases the diagnosis of juvenile sterile granulomatous dermatitis and lymphadenitis was confirmed by histology. Nine dogs were treated with prednisolone (0.5 - 1.25 mg/kg BID), H2-receptor antagonists and analgetics; all dogs were treated with antibiotics. Four dogs were treated with eye ointment containing antibiotics and glucocorticoids. The prednisolone dosage was tapered over 3 - 8 weeks. One dog had a relapse.

Adolescent neglect, juvenile delinquency and the risk of recidivism.

Science.gov (United States)

Ryan, Joseph P; Williams, Abigail B; Courtney, Mark E

2013-03-01

Victims of child abuse and neglect are at an increased risk of involvement with the juvenile justice and adult correctional systems. Yet, little is known about the continuation and trajectories of offending beyond initial contact with law enforcement. Neglect likely plays a critical role in continued offending as parental monitoring, parental rejection and family relationships are instrumental in explaining juvenile conduct problems. This study sought to determine whether neglect is associated with recidivism for moderate and high risk juvenile offenders in Washington State. Statewide risk assessments and administrative records for child welfare, juvenile justice, and adult corrections were analyzed. The sample was diverse (24 % female, 13 % African American, 8 % Hispanic, 5 % Native American) and included all moderate and high risk juvenile offenders screened by juvenile probation between 2004 and 2007 (n = 19,833). Official records from child protection were used to identify juvenile offenders with a history of child neglect and to identify juvenile offenders with an ongoing case of neglect. Event history models were developed to estimate the risk of subsequent offending. Adolescents with an ongoing case neglect were significantly more likely to continue offending as compared with youth with no official history of neglect. These findings remain even after controlling for a wide range of family, peer, academic, mental health, and substance abuse covariates. Interrupting trajectories of offending is a primary focus of juvenile justice. The findings of the current study indicate that ongoing dependency issues play a critical role in explaining the outcomes achieved for adolescents in juvenile justice settings. The implications for improved collaboration between child welfare and juvenile justice are discussed.

Beneficial Effects of Rhodotorula sp. C11 on Growth and Disease Resistance of Juvenile Japanese Spiky Sea Cucumber Apostichopus japonicus.

Science.gov (United States)

Yang, ZhiPing; Sun, JianMing; Xu, Zhe

2015-06-01

The purpose of this study was to evaluate the effects of dietary administration of the live yeast, Rhodotorula sp. C11, on growth and disease resistance against Vibrio splendidus infection in juvenile Japanese spiky sea cucumber Apostichopus japonicus. Sea cucumbers were fed diets containing Rhodotorula sp. C11 at 0 (control), 10⁴, 10⁵, and 10⁶ CFU/g of feed for 45 d. There were three replicate tanks per dietary treatment. The specific growth rates were higher in all sea cucumbers treated with Rhodotorula sp. C11 than in the controls. Following a challenge with V. splendidus NB13, the cumulative prevalence and mortality of sea cucumbers fed diets supplemented with Rhodotorula sp. C11 were lower than in animals fed the basal diet. In sea cucumbers fed diets supplemented with Rhodotorula sp. C11 for 42 d, the only viable yeast found in the intestine was Rhodotorula sp. C11, which had counts of 1.58-1.98 × 10⁴CFU/g. No yeast was isolated from the intestine of animals fed the basal diet. For the colonization study, 20 sea cucumbers from each dietary treatment were removed to separate tanks and fed the control diet from day 16 to day 46. The viable yeast (Rhodotorula sp. C11) counts in the intestine decreased to 60-80 CFU/g by day 37. Moreover, as demonstrated by denaturing gradient gel electrophoresis, Rhodotorula sp. C11 colonization of the intestine could be detected until day 46. The differences in culture and PCR-denaturing gradient gel electrophoresis may be due to differences in the sensitivity of both methods. The present result showed that Rhodotorula sp. C11 was able to successfully colonize the intestine of juvenile Japanese spiky sea cucumbers by dietary supplementation, which improved its growth and disease resistance.

Do juveniles bully more than young offenders?

Science.gov (United States)

Ireland, Jane L

2002-04-01

This study compares bullying behaviour among juvenile and young offenders and incorporates two different methods to measure bullying. Ninety-five male juvenile and 196 male young offenders completed two questionnaires, one that measured bullying directly and one that measured behaviours indicative of "being bullied" or of "bullying others". Juveniles perceived a higher extent of bullying than young offenders. Juveniles reported significantly more physical, psychological or verbal and overall direct forms of bullying behaviour than young offenders. A number of differences were found between juveniles and young offenders with regard to the types of prisoners likely to become victims, who they would advise a victim to speak to and how bullying could be prevented. The results are discussed in relation to developmental theories of aggression and how bullying behaviour can be defined and measured among prisoners. Copyright 2002 The Association for Professionals in Services for Adolescents. Published by Elsevier Science Ltd. All rights reserved.

The role of heme oxygenase-1 in systemic-onset juvenile idiopathic arthritis.

Science.gov (United States)

Takahashi, Akitaka; Mori, Masaaki; Naruto, Takuya; Nakajima, Shoko; Miyamae, Takako; Imagawa, Tomoyuki; Yokota, Shumpei

2009-01-01

We have determined the serum levels of heme oxygenase-1 (HO-1) in 56 patients with systemic-onset juvenile idiopathic arthritis (s-JIA) and compared these with serum HO-1 levels in healthy controls and patients with other pediatric rheumatic diseases. Serum HO-1 levels were measured by the sandwich enzyme-linked immunosorbent assay. The mean serum HO-1 level in s-JIA patients during the active phase was 123.6 +/- 13.83 ng/ml, which was significantly higher than that in patients with polyarticular juvenile idiopathic arthritis (p-JIA), Kawasaki disease, systemic lupus erythematosus or mixed connective tissue disease (P < 0.0005). The serum levels of HO-1, cytokines and cytokine receptors in patients with s-JIA were also assessed at both the active and inactive phases. The serum HO-1 level in patients with s-JIA in the active phase was found to be significantly greater than that in patients with the disease in the inactive phase (P < 0.0001). An assessment of the relationships between serum HO-1 levels and other laboratory parameters or cytokines in patients with s-JIA did not reveal any strong correlations. These results suggest that the serum level of HO-1 may be a useful marker for the differential diagnosis of s-JIA. Further study will be necessary to elucidate the mechanism of HO-1 production and to clarify the role of HO-1 in the disease process.

Safety and Efficacy of Anti-Pandemic H1N1 Vaccination in Rheumatic Diseases

Science.gov (United States)

2010-06-25

Rheumatoid Arthritis; Spondyloarthritis; Systemic Lupus Erythematosus (SLE); Dermatomyositis (DM); DMixed Connective Tissue Disease; Systemic Vasculitis; Systemic Sclerosis (SSc); Sjögren's Syndrome; Antiphospholipid Syndrome; Juvenile Idiopathic Arthritis; Juvenile SLE; Juvenile DM

An unusual presentation of juvenile lupus nephritis

Directory of Open Access Journals (Sweden)

Malleshwar Bottu

2016-01-01

Full Text Available The incidence of juvenile lupus varies widely ranging between 4 and 250 per 100,000 population. Most common organ involvement in juvenile lupus is kidney. Neurological, cutaneous and hematological involvements are also involved. Skeletal muscle involvement in the form of myositis is rare. Myositis as presenting manifestation in juvenile lupus is also unusual. Herein, we report one such case wherein myositis preceded the onset of lupus nephritis

Laser capture microdissection of gonads from juvenile zebrafish

DEFF Research Database (Denmark)

Jørgensen, Anne; Nielsen, John; Morthorst, Jane Ebsen

2009-01-01

was adjusted and optimised to isolate juvenile zebrafish gonads. Results: The juvenile zebrafish gonad is not morphologically distinguishable when using dehydrated cryosections on membrane slides and a specific staining method is necessary to identify the gonads. The protocol setup in this study allows......Background: Investigating gonadal gene expression is important in attempting to elucidate the molecular mechanism of sex determination and differentiation in the model species zebrafish. However, the small size of juvenile zebrafish and correspondingly their gonads complicates this type...... of investigation. Furthermore, the lack of a genetic sex marker in juvenile zebrafish prevents pooling gonads from several individuals. The aim of this study was to establish a method to isolate the gonads from individual juvenile zebrafish allowing future investigations of gonadal gene expression during sex...

Australian bat lyssavirus infection in a captive juvenile black flying fox.

OpenAIRE

Field, H.; McCall, B.; Barrett, J.

1999-01-01

The newly emerging Australian bat lyssavirus causes rabieslike disease in bats and humans. A captive juvenile black flying fox exhibited progressive neurologic signs, including sudden aggression, vocalization, dysphagia, and paresis over 9 days and then died. At necropsy, lyssavirus infection was diagnosed by fluorescent antibody test, immunoperoxidase staining, polymerase chain reaction, and virus isolation. Eight human contacts received postexposure vaccination.

Disease: H00274 [KEGG MEDICUS

Lifescience Database Archive (English)

Full Text Available odontitis. Inherited metabolic disease CTSC; cathepsin C...oving dipeptides from the amino termini of its substrates. The disease is characterized by palmoplantar keratoderma and juvenile peri

Síndrome de ativação macrofágica em pacientes com artrite idiopática juvenil Macrophage activation syndrome in patients with juvenile idiopathic arthritis

Directory of Open Access Journals (Sweden)

Rogério do Prado

2004-10-01

Full Text Available A síndrome de ativação macrofágica (SAM é uma complicação rara das doenças reumáticas crônicas, particularmente a artrite idiopática juvenil (AIJ de início sistêmico. Este processo pode ser desencadeado por agentes infecciosos virais e bacterianos, neoplásicos, drogas antiinflamatórias não esteroidais ou drogas modificadoras da doença, mudanças abruptas das medicações e doenças reumáticas. O quadro clínico inicia-se com irritação do sistema nervoso central, acompanhado de falências hepática e renal, além de pancitopenia. Relatamos três casos de pacientes com AIJ do nosso serviço que desenvolveram SAM com descrição das características clínicas, evolutivas e de tratamento.The macrophage activation syndrome (MAS is an uncommon complication of chronic rheumatic diseases, specially systemic onset juvenile idiopathic arthritis (JIA. It can be triggered by infectious (viral or bacterial or malignant diseases, non-steroidal anti-inflammatory or disease modified anti-rheumatic drugs, changes in the therapy and rheumatic diseases. The clinical features present at the onset are related mainly with central nervous system involvement, hepatic and renal failure and pancytopenia. We describe the clinical, evolutive features and treatment of three patients with JIA that developed MAS.

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

Directory of Open Access Journals (Sweden)

E.I. Alexeeva

2011-01-01

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

Tissue and plasma enzyme activities in juvenile green iguanas.

Science.gov (United States)

Wagner, R A; Wetzel, R

1999-02-01

To determine activities of intracellular enzymes in 8 major organs in juvenile green iguanas and to compare tissue and plasma activities. 6 green iguanas iguanas, but high values may not always indicate overt muscle disease. The AMS activity may be specific for the pancreas, but the wide range of plasma activity would likely limit its diagnostic usefulness. Activities of AST and LDH may reflect tissue damage or inflammation, but probably do not reflect damage to specific tissues or organs.

Law & psychiatry: punishing juveniles who kill.

Science.gov (United States)

Appelbaum, Paul S

2012-10-01

Punishment of juvenile murderers forces policy makers to weigh the developmental immaturity of adolescents against the heinousness of their crimes. The U.S. Supreme Court has progressively limited the severity of punishments that can be imposed on juveniles, holding that their impulsivity, susceptibility to peer pressure, and more fluid character render them less culpable for their actions. Having eliminated the death penalty as a punishment, the Court recently struck down mandatory life sentences without prospect of parole. The decision is interesting for its emphasis on rehabilitation, opening the door to further restrictions on punitive sentences for juveniles-and perhaps for adults too.

A simple method of screening for metabolic bone disease

International Nuclear Information System (INIS)

Broughton, R.B.K.; Evans, W.D.

1982-01-01

The purpose of this investigation was to find a simple method -to be used as an adjunct to the conventional bone scintigram- that could differentiate between decreased bone metabolism or mass, i.e., osteoporosis -normal bone- and the group of conditions of increased bone metabolism or mass namely, osteomalacia, renal osteodystrophy, hyperparathyroidism and Paget's disease. The Fogelman's method using the bone to soft tissue ratios from region of interest analysis at 4 hours post injection, was adopted. An initial experience in measuring a value for the count rate density in lumbar vertebrae at 1 hr post injection during conventional bone scintigraphy appears to give a clear indication of the overall rate of bone metabolism. The advantage over whole body retention methods is that the scan performed at the end of the metabolic study will reveal localized bone disease that may otherwise not be anticipated

[Early diagnosis, variety of outcomes and prognosis of juvenile schizophrenia with over-value disorders of the "metaphysical intoxication" type onset (results of a late follow-up study)].

Science.gov (United States)

Tsutsul'kovskaia, M Ia; Izvol'skiĭ, S A; Bol'zho, A G; Kopeĭko, G I

1986-01-01

On the basis of follow-up findings about juvenile schizophrenia first expressed in superworship manifestations according to the type of metaphysical intoxication (98 observations), the authors established that most frequently these cases were characterized by a torpid course of the disease in the framework of slowly progressing juvenile schizophrenia. There was a high rate of favourable outcomes at the level of "clinical recovery" (in 42.5%). Favourable prognostic signs were as follows: the complete nature of the clinical picture of the syndrome, its similarity to pubertal crisis manifestations, the absence in its structure of other positive disturbances and a number of specific features of the premorbid picture. The authors determined the pattern of correlation between the typological characteristics of the state of juvenile metaphysical intoxication and the outcome of the disease, peculiarities of manifestations of this phenomenon at the initial stage of psychotic forms of juvenile schizophrenia, as well as distinctive features of superworship formations according to the type of juvenile metaphysical intoxication observed in the framework of pubertal decompensation in schizoid psychopathies.

Calcitonin Salmon Injection

Science.gov (United States)

Calcitonin salmon injection is used to treat osteoporosis in postmenopausal women. Osteoporosis is a disease that causes bones to weaken and break more easily. Calcitonin salmon injection is also used to treat Paget's disease ...

Development of consensus treatment plans for juvenile localized scleroderma: a roadmap toward comparative effectiveness studies in juvenile localized scleroderma.

Science.gov (United States)

Li, Suzanne C; Torok, Kathryn S; Pope, Elena; Dedeoglu, Fatma; Hong, Sandy; Jacobe, Heidi T; Rabinovich, C Egla; Laxer, Ronald M; Higgins, Gloria C; Ferguson, Polly J; Lasky, Andrew; Baszis, Kevin; Becker, Mara; Campillo, Sarah; Cartwright, Victoria; Cidon, Michael; Inman, Christi J; Jerath, Rita; O'Neil, Kathleen M; Vora, Sheetal; Zeft, Andrew; Wallace, Carol A; Ilowite, Norman T; Fuhlbrigge, Robert C

2012-08-01

Juvenile localized scleroderma (LS) is a chronic inflammatory skin disorder associated with substantial morbidity and disability. Although a wide range of therapeutic strategies has been reported in the literature, a lack of agreement on treatment specifics and accepted methods for clinical assessment has made it difficult to compare approaches and identify optimal therapy. Our objective was to develop standardized treatment plans, clinical assessments, and response criteria for active, moderate to high severity juvenile LS. A core group of pediatric rheumatologists, dermatologists, and a lay advisor was engaged by the Childhood Arthritis and Rheumatology Research Alliance (CARRA) to develop standardized treatment plans and assessment parameters for juvenile LS using consensus methods/nominal group techniques. Recommendations were validated in 2 face-to-face conferences with a larger group of practitioners with expertise in juvenile LS and with the full membership of CARRA, which encompasses the majority of pediatric rheumatologists in the US and Canada. Consensus was achieved on standardized treatment plans that reflect the prevailing treatment practices of CARRA members. Standardized clinical assessment methods and provisional treatment response criteria were also developed. Greater than 90% of pediatric rheumatologists responding to a survey (66% of CARRA membership) affirmed the final recommendations and agreed to utilize these consensus plans to treat patients with juvenile LS. Using consensus methodology, we have developed standardized treatment plans and assessment methods for juvenile LS. The high level of support among pediatric rheumatologists will support future comparative effectiveness studies and enable the development of evidence-based guidelines for the treatment of juvenile LS. Copyright © 2012 by the American College of Rheumatology.

The semantic sphere of juvenile offenders

Directory of Open Access Journals (Sweden)

Oshevsky D.S.

2017-01-01

Full Text Available The article presents the results of a preliminary empirical study aimed to identify features of the semantic sphere of adolescents who have committed illegal, including aggressive acts. The study included 50 male juveniles aged of 16 - 17 years. The first group consisted of adolescents convicted of aggressive and violent crimes; the second – of property socially dangerous acts (SDA. It is shown that evaluation of such adolescents is generally categorical and polar, the semantic field is subdifferentiable, less hierarchic, and has not enough realistic structure of meanings. Developed structure of motives and meanings is the basis of voluntary regulation of socially significant behavior. Thus, assessing the semantic sphere of juvenile offenders we can highlight its characteristics as risk factors of unlawful behavior, as well as the resource side, that will contribute to addressing issues of prevention and correction of unlawful behavior. Key words: juvenile offenders, semantic field of juvenile offenders, unlawful behavior.

Sex differences in metabolic and adipose tissue responses to juvenile-onset obesity in sheep.

Science.gov (United States)

Bloor, Ian D; Sébert, Sylvain P; Saroha, Vivek; Gardner, David S; Keisler, Duane H; Budge, Helen; Symonds, Michael E; Mahajan, Ravi P

2013-10-01

Sex is a major factor determining adipose tissue distribution and the subsequent adverse effects of obesity-related disease including type 2 diabetes. The role of gender on juvenile obesity and the accompanying metabolic and inflammatory responses is not well established. Using an ovine model of juvenile onset obesity induced by reduced physical activity, we examined the effect of gender on metabolic, circulatory, and related inflammatory and energy-sensing profiles of the major adipose tissue depots. Despite a similar increase in fat mass with obesity between genders, males demonstrated a higher storage capacity of lipids within perirenal-abdominal adipocytes and exhibited raised insulin. In contrast, obese females became hypercortisolemic, a response that was positively correlated with central fat mass. Analysis of gene expression in perirenal-abdominal adipose tissue demonstrated the stimulation of inflammatory markers in males, but not females, with obesity. Obese females displayed increased expression of genes involved in the glucocorticoid axis and energy sensing in perirenal-abdominal, but not omental, adipose tissue, indicating a depot-specific mechanism that may be protective from the adverse effects of metabolic dysfunction and inflammation. In conclusion, young males are at a greater risk than females to the onset of comorbidities associated with juvenile-onset obesity. These sex-specific differences in cortisol and adipose tissue could explain the earlier onset of the metabolic-related diseases in males compared with females after obesity.

Differential heritability of adult and juvenile antisocial traits.

Science.gov (United States)

Lyons, M J; True, W R; Eisen, S A; Goldberg, J; Meyer, J M; Faraone, S V; Eaves, L J; Tsuang, M T

1995-11-01

Studies of adult antisocial behavior or criminality usually find genetic factors to be more important than the family environment, whereas studies of delinquency find the family environment to be more important. We compared DSM-III-R antisocial personality disorder symptoms before vs after the age of 15 years within a sample of twins, rather than comparing across studies. We administered the Diagnostic Interview Schedule Version III-revised by telephone to 3226 pairs of male twins from the Vietnam Era Twin Registry. Biometrical modeling was applied to each symptom of antisocial personality disorder and summary measures of juvenile and adult symptoms. Five juvenile symptoms were significantly heritable, and five were significantly influenced by the shared environment. Eight adult symptoms were significantly heritable, and one was significantly influenced by the shared environment. The shared environment explained about six times more variance in juvenile anti-social traits than in adult traits. Shared environmental influences on adult antisocial traits overlapped entirely with those on juvenile traits. Additive genetic factors explained about six times more variance in adult vs juvenile traits. The juvenile genetic determinants overlapped completely with genetic influences on adult traits. The unique environment (plus measurement error) explained the largest proportion of variance in both juvenile and adult antisocial traits. Characteristics of the shared or family environment that promote antisocial behavior during childhood and early adolescence also promote later antisocial behavior, but to a much lesser extent. Genetic causal factors are much more prominent for adult than for juvenile antisocial traits.

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…

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...

Disease introduction is associated with a phase transition in bighorn sheep demographics

Science.gov (United States)

Manlove, Kezia; Cassirer, E. Frances; Cross, Paul C.; Plowright, Raina K.; Hudson, Peter J.

2016-01-01

Ecological theory suggests that pathogens are capable of regulating or limiting host population dynamics, and this relationship has been empirically established in several settings. However, although studies of childhood diseases were integral to the development of disease ecology, few studies show population limitation by a disease affecting juveniles. Here, we present empirical evidence that disease in lambs constrains population growth in bighorn sheep (Ovis canadensis) based on 45 years of population-level and 18 years of individual-level monitoring across 12 populations. While populations generally increased (λ = 1.11) prior to disease introduction, most of these same populations experienced an abrupt change in trajectory at the time of disease invasion, usually followed by stagnant-to-declining growth rates (λ = 0.98) over the next 20 years. Disease-induced juvenile mortality imposed strong constraints on population growth that were not observed prior to disease introduction, even as adult survival returned to pre-invasion levels. Simulations suggested that models including persistent disease-induced mortality in juveniles qualitatively matched observed population trajectories, whereas models that only incorporated all-age disease events did not. We use these results to argue that pathogen persistence may pose a lasting, but under-recognized, threat to host populations, particularly in cases where clinical disease manifests primarily in juveniles.

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...

Osteogenic sarcoma of the skull. A clinicopathologic study of 19 patients

International Nuclear Information System (INIS)

Huvos, A.G.; Sundaresan, N.; Bretsky, S.S.; Butler, A.

1985-01-01

The authors studied 19 patients with well documented osteogenic sarcomas arising in the skull, which represent 1.6% of all osteogenic sarcomas registered during a 60-year period (1921-1981). Ten sarcomas were primary, de novo tumors. Nine others developed secondary osteogenic sarcomas; among these, six arose as a complication of Paget's disease, two followed irradiation, and one was associated with pre-existent fibrous dysplasia. The sarcomas arose in equal proportion in both sexes with the men being much older (mean age, 44 years) as compared to the women (mean age, 31 years). Patients with de novo osteogenic sarcomas were considerably younger than those with secondary lesions. Osteoblastic osteogenic sarcoma was by far the most common histologic variant in both the primary and the Paget's sarcomas. None of the patients with Paget's sarcoma lived longer than 1 year; the median survival here was 4 months. Patients with de novo osteogenic sarcomas fared much better and there are four long-term survivors (longer than 3 years) who are currently disease-free

MRI Findings of Juvenile Xanthogranuloma of the Spinal Cord: A Case Report

International Nuclear Information System (INIS)

Kim, Se Young; Park, Hee Jin; Lee, So Yeon; Chung, Eun Chul; Park, Hae Won; Kook, Shin Ho; Rho, Myung Ho; Goo, Ji Hye

2013-01-01

Juvenile xanthogranuloma (JXG) is a proliferative histiocytic disorder experienced during childhood and adolescents. JXG commonly presents as a solitary cutaneous lesion. Despite the term 'juvenile', development of the disease during adulthood is possible, although spinal JXG is extremely rare in adults. We describe a 67-year-old female patient who presented with an intradural-extramedullary (IDEM) tumor of the spinal cord. Magnetic resonance imaging (MRI) findings indicative of JXG of the spinal cord were seen, which was then confirmed pathologically. A lumbar spinal MRI with contrast enhancement showed an oval-shaped, well-defined IDEM tumor at the L1 level. This tumor had mixed signal intensity on the T1-weighted image and high signal intensity on the T2-weighted image. Central homogenous enhancement was observed after contrast administration.

"Scared Straight" and other juvenile awareness programs for preventing juvenile delinquency.

Science.gov (United States)

Petrosino, A; Turpin-Petrosino, C; Buehler, J

2002-01-01

'Scared Straight' and other programmes involve organised visits to prison by juvenile delinquents or children at risk for criminal behavior. programmes are designed to deter participants from future offending through first-hand observation of prison life and interaction with adult inmates. These programmes remain in use world-wide despite studies and reviews questioning their effectiveness. To assess the effects of programmes comprising organised visits to prisons by juvenile delinquents (officially adjudicated or convicted by a juvenile court) or pre-delinquents (children in trouble but not officially adjudicated as delinquents), aimed at deterring them from criminal activity. Handsearching by the first author in identifying randomised field trials 1945-1993 relevant to criminology was augmented by structured searches of 16 electronic data bases, including the Campbell SPECTR database of trials and the Cochrane CCTR. Experts in the field were consulted and relevant citations were followed up. Studies that tested the effects of any program involving the organised visits of juvenile delinquents or children at-risk for delinquency to penal institutions were included. Studies that included overlapping samples of juvenile and young adults (e.g. ages 14-20) were included. We only considered studies that randomly or quasi-randomly (i.e. alternation) assigned participants to conditions. Each study had to have a no-treatment control condition with at least one outcome measure of "post-visit" criminal behavior. We report narratively on the nine eligible trials. We conducted one meta-analysis of post-intervention offending rates using official data. Information from other sources (e.g. self-report) was either missing from some studies or critical information was omitted (e.g. standard deviations). We examined the immediate post-treatment effects (i.e. "first-effects") by computing Odds Ratios (OR) for data on proportions of each group re-offending, and assumed both fixed and

Multimodality approach in the diagnosis and management of bilateral giant juvenile breast fibroadenoma.

Science.gov (United States)

Rafeek, Noora; Rangasami, Rajeswaran; Dhanraj, Kamakshi; Joseph, Santhosh

2016-10-06

Juvenile giant fibroadenoma is a very rare breast disease affecting young girls of premenarche and adolescent ages. It is a benign fibroepithelial tumour characterised by stromal and epithelial proliferation that causes rapidly growing breast mass. Bilateral symmetrical involvement is extremely rare. In this article, we describe this entity in a girl aged 13 years who presented with bilateral gigantically enlarged breasts. Ultrasonography and MRI showed large, multilobulated masses involving both breasts entirely. Endovascular embolisation of bilateral internal mammary arteries and lateral thoracic arteries supplying the masses was performed prior to surgery to reduce their vascularity. The patient subsequently underwent excision of bilateral breast masses and reduction mammoplasty. Histopathologically, bilateral breast masses were confirmed to be juvenile fibroadenomas. 2016 BMJ Publishing Group Ltd.

Growth Abnormalities in Children with Type 1 Diabetes, Juvenile Chronic Arthritis, and Asthma

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Cosimo Giannini

2014-01-01

Full Text Available Children and adolescents with chronic diseases are commonly affected by a variable degree of growth failure, leading to an impaired final height. Of note, the peculiar onset during childhood and adolescence of some chronic diseases, such as type 1 diabetes, juvenile idiopathic arthritis, and asthma, underlines the relevant role of healthcare planners and providers in detecting and preventing growth abnormalities in these high risk populations. In this review article, the most relevant common and disease-specific mechanisms by which these major chronic diseases affect growth in youth are analyzed. In addition, the available and potential targeting strategies to restore the physiological, hormonal, and inflammatory pattern are described.

Preventing Juvenile Delinquency

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Carolina dos Reis

2016-04-01

Full Text Available This article aims to problematize discourses about protection and care that have surrounded compulsory hospitalization by evidencing its use as a control and punishment mechanism that increases the social vulnerability of young drug users. For such, we analyze lawsuits involving juveniles who were consigned to psychiatric institutions for drug addiction treatment as a protection measure in the state of Rio Grande do Sul, in Brazil. The analysis of the materials has evidenced discourses that have circumscribed young drug users and constructed this population as potentially dangerous subjects as well as a population category at risk. In this sense, we point out how compulsory hospitalization has emerged out of the lawsuits as a tool for prevention of juvenile delinquency.

Attitudes towards self-control with urinalysis in juvenile diabetes.

Science.gov (United States)

Ludvigsson, J; Svensson, P G

1980-01-01

Urinary glucose excretion reflects the blood glucose levels and is therefore recommended and used as a relevant and practical method for self-control in juvenile diabetes. The purpose of this study was to estimate the attitudes of of diabetic children and their parents towards such daily urinalysis. In 1975 69 juvenile diabetics 6-18 years old and their parents were studied and three years later another 69 patients were added. Still a year later 31 of the children were studied again. Standardized interviews, questionnaires and a special attitude test were used. The results indicate that a great majority of the patients and the parents accept the self-testing method and regard it as a valuable tool in the management of the disease. Almost nobody experienced the urine tests as a psychological problem. As urinalysis has become established as a self-evident part of the treatment, the attitudes have become even more positive among a growing number of patients. Parallel to this feeling of usefulness the patients are honest and the urine tests thus give reliable information.

The World of Juvenile Justice According to the Numbers

Science.gov (United States)

Rozalski, Michael; Deignan, Marilyn; Engel, Suzanne

2008-01-01

Intended to be an instructive, yet sobering, introduction to the complex and disturbing nature of the juvenile justice system, this article details the "numbers," including selected percentages, ratios, and dollar amounts, that are relevant to developing a better understanding of the juvenile justice system. General statistics about juvenile and…

Contraception for adolescents with chronic rheumatic diseases

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Benito Lourenço

Full Text Available ABSTRACT Contraception is an important issue and should be a matter of concern in every medical visit of adolescent and young patients with chronic rheumatic diseases. This narrative review discusses contraception methods in adolescents with juvenile systemic lupus erythematosus (JSLE, antiphospholipid syndrome (APS, juvenile idiopathic arthritis (JIA and juvenile dermatomyositis (JDM. Barrier methods are safe and their use should be encouraged for all adolescents with chronic rheumatic diseases. Combined oral contraceptives (COC are strictly prohibited for JSLE and APS patients with positive antiphospholipid antibodies. Reversible long-acting contraception can be encouraged and offered routinely to the JSLE adolescent patient and other rheumatic diseases. Progestin-only pills are safe in the majority of rheumatic diseases, although the main concern related to its use by adolescents is poor adherence due to menstrual irregularity. Depot medroxyprogesterone acetate injections every three months is a highly effective contraception strategy, although its long-term use is associated with decreased bone mineral density. COC or other combined hormonal contraceptive may be options for JIA and JDM patients. Oral levonorgestrel should be considered as an emergency contraception method for all adolescents with chronic rheumatic diseases, including patients with contraindication to COC.

HIV testing among non-incarcerated substance-abusing juvenile offenders.

Science.gov (United States)

Tolou-Shams, Marina; Conrad, Selby; Louis, Alaina; Shuford, Sarah Hart; Brown, Larry K

2015-11-01

Juvenile offenders are a subgroup of adolescents at particular risk for HIV/STI infection. Although HIV prevalence among these youth is low (justice system, which is known to have an extremely high rate of HIV infection. US constitutional mandates provide HIV/STI testing for incarcerated juveniles, but close to 80% of juvenile arrestees are never detained. Moreover, although they engage in similar HIV risk behaviors as those detained, they have limited access to available HIV/STI testing services. Thus, our study examined rates of lifetime HIV testing among a pilot sample of 60 court-involved, substance-using juveniles monitored in the community to explore rates of testing and the reasons related to lifetime testing among a high-risk, yet understudied US juvenile population.

Iris juvenile xanthogranuloma in an infant - spontaneous hyphema and secondary glaucoma

OpenAIRE

Pantalon, Anca; Ștefănache, Tudor; Danciu, Mihai; Zurac, Sabina; Chiseliță, Dorin

2017-01-01

Juvenile xanthogranuloma (JXG) is a benign histiocytic skin disorder mainly encountered during infancy and childhood. Although with multiple potential localizations, less than 1% of the cases exhibit ocular manifestations. Some of these might lead to serious complications, specifically, secondary glaucoma that can result in severe and blinding eye disease. The aim of the present case report was to demonstrate typical clinical features, emphasize the difficulties attributed when managing these...

Overt and occult vidian canal involvement in juvenile angiofibroma and its possible impact on recurrence.

Science.gov (United States)

Thakar, Alok; Hota, Ashutosh; Bhalla, Ashu Seth; Gupta, Siddharth Datta; Sarkar, Chitra; Kumar, Rakesh

2016-04-01

Postexcision residual disease in the vidian canal is speculated to contribute to recurrence in juvenile angiofibroma. We composed a prospective cohort of 16 consecutive patients with juvenile angiofibroma (stages IIA-IIIB). The presurgical vidian canal assessment was done by contrast-enhanced CT (1.2 mm collimation). At surgery after complete tumor excision, the vidian canal tissue was sampled for histology. Postexcision drilling of the vidian canal was done in 8 of 15 patients to remove microscopic residual disease, with a 24 to 48 month follow-up period. Presurgical radiology indicated ipsilateral vidian canal enlargement (≥3 mm)/destruction in 13 of 16 patients. Radiologically occult involvement was documented only by histology in another 1 of 16 patients. Postexcision sampling of the vidian canal noted microscopic residual tumor in 3 of 15 patients. No recurrences were noted in 8 cases (0 of 8) with postexcision drilling of the vidian canal and 2 recurrences in 7 cases (2 of 7) with no drilling (p = .20). Vidian canal involvement in juvenile angiofibroma is almost universal (14 of 16) and may be occult to CT evaluation. The site may harbor microscopic residual tumor after seemingly complete excision. Surgical attention toward it may reduce recurrences. © 2015 Wiley Periodicals, Inc. Head Neck 38: E421-425, 2016. © 2015 Wiley Periodicals, Inc.

A Giant Juvenile Nasopharyngeal Angiofibroma

Science.gov (United States)

Yüce, Salim; Uysal, İsmail Önder; Doğan, Mansur; Polat, Kerem; Şalk, İsmail; Müderris, Suphi

2012-01-01

Juvenile nasopharyngeal angiofibroma (JNA) are locally growing highly vascular tumours. They are treated primarily by surgical excision ranging from open approach to endoscopic approach. We presented a 20-year-old male with a giant nasopharyngeal juvenile angiofibroma obliterating the pterygopalatine fossa bilaterally, invasing the sphenoid bone and extending to the left nasal passage. His complaints were epistaxis and nasal obstruction. After embolization, the patient was treated surgically with endoscopic approach and discharged as cured without any complication. PMID:23714961

Combination of Juvenile Papillomatosis, Juvenile Fibroadenoma and Intraductal Carcinoma of the Breast in a 15-Year-old Girl.

Science.gov (United States)

Sedloev, Theophil; Bassarova, Assia; Angelov, Kostadin; Vasileva, Mariela; Asenov, Yavor

2015-09-01

The association of juvenile papillomatosis with carcinoma is proven, but very rare, as there exist several reported cases. A 15-year-old girl with no family history of breast cancer presented with two masses in the left breast. The excisional biopsy on both lumps revealed juvenile fibroadenoma and juvenile papillomatosis epithelial proliferation multiple cystic expanded channels. In some of these channels, cytological features of intraductal carcinoma were observed. We performed a full immunohistochemical examination of the juvenile papillomatosis. The patient refused any further surgical or adjuvant treatment. There are no signs of recurrence in the 15 year follow-up. This case is a diagnostic and therapeutic challenge, taking into account the patient's age and the controversial treatment recommendations. Good collaboration between surgeons and pathologists is essential for an accurate diagnostic process and aims to avoid under- or overtreatment. Copyright© 2015 International Institute of Anticancer Research (Dr. John G. Delinassios), All rights reserved.

Challenges and prospects of the juvenile justice administration in ...

African Journals Online (AJOL)

Juvenile justice administration in Nigeria is weak and has been given very little priority, despite Nigeria being signatory to the major international instruments relevant to the administration of juvenile justice. This is attributable to the history of the penal system of Nigeria, with laws guiding juvenile justice administration having ...

[Juvenile idiopathic arthritis and oral health].

Science.gov (United States)

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

2016-05-04

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

QUALITY OF LIFE IN CHILDREN WITH JUVENILE RHEUMATOID ARTHRITIS TREATED WITH INFLIXIMAB

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R.V. Denisova

2008-01-01

Full Text Available Juvenile rheumatoid arthritis (JRA is chronic disease, leading to early incapacitating injury in patients. Treatment of JRA with new expensive biological agents allows obtaining long term remission of disease and improving its prognosis. Estimation of quality of life is one of the main effectiveness criteria of treatment. A quality of life in children who were 2–4 years old treated with infliximab was estimated. 43 patients with different types of JRA were examined. A quality of life was estimated with the help of questionnaire PEDSQL generic core scale, PEDSQL rheumatology module. Index of functional disability was estimated by childhood health assessment questionnaire (CHAQ. Significant increase of quality of life rates and decrease of index of functional disability was registered in 6 weeks of therapy with infliximab. The rates of quality of life in patients with JRA treated with infliximab were significantly equal to that in healthy children in the same age in 6, 12 and 24 months of treatment. Thus, treatment with infliximab significantly increases quality of life in children in 2–4 years old with JRA and their families, decreases negative influence of disease on child's living, improves physical activity and emotional state of patients, and allows improving contact between patients and healthy children in the same age.Key words: children, juvenile rheumatoid arthritis, quality of life, infliximab.

Radiological imaging in pediatric rheumatic diseases

International Nuclear Information System (INIS)

Matuszewska, Genowefa; Zaniewicz-Kaniewska, Katarzyna; Włodkowska-Korytkowska, Monika; Smorawińska, Patrycja; Saied, Fadhil; Kunisz, Wojciech; Sudoł-Szopińska, Iwona

2014-01-01

Radiological imaging plays a fundamental role in the diagnosis and monitoring of rheumatic diseases. The basic method of imaging is a classic X-ray picture, which for many years has been used as a single method for the recognition and evaluation of the effects of disease management. In today’s modern day treatment of rheumatic diseases, ultrasonography and magnetic resonance are more commonly performed for early detection of inflammatory changes in the region of soft tissue, subchondral bone and bone marrow. In spite of their usefulness and fundamental role in the diagnosis, X-ray still remains an essential tool in the diagnosis of rheumatoid arthritis in children and is complementary to today’s methods of imaging diagnostics. In clinical practice, X-ray imaging is still an important examination performed not only to recognize the disorders, but also to provide a differential diagnosis. It helps estimate disease progression and is used to monitor the effects of treatment and the development of possible complications. Differential diagnosis of rheumatic diseases is performed on the basis of localization and type of radiographic changes. The surrounding periarticular soft tissues, bone structures, joint space, with special attention to articular bone surfaces and epiphyses, are analyzed. The aim of this work is to describe characteristic inflammatory changes present on X-ray imaging typical for the most commonly diagnosed rheumatic diseases in children, such as juvenile idiopathic arthritis, systemic lupus erythematosus, systemic scleroderma, mixed connective tissue disease, juvenile dermatomyositis, juvenile spondyloarthropathy and systemic vascular disease

Two unusual causes of peripatellar nonmetastatic positive bone scans in patients with malignancies: case reports

International Nuclear Information System (INIS)

Turner, J.W.; Syed, I.B.; Spencer, R.P.

1976-01-01

Bone scans performed with /sup 99m/Tc-polyphosphate in two patients with epidermoid carcinoma of the lung each showed activity in one knee area. In the first case, radiographs of the area revealed Pellegrini-Stieda calcification over the internal femoral condyle. Uptake corresponded to this process, rather than to a lesion deep in the bone. In the second case, the activity accumulation in the right tibia was the only abnormality noted in the scan. An open biopsy revealed Paget's disease, and no radiographic evidence of Paget's disease was found in any other bone. These cases again illustrate that localized accumulation of a bone-scanning agent, in patients with known primary tumors, does not necessarily denote malignancy in bone

Increased Fas and Bcl-2 Expression on Peripheral Blood T and B Lymphocytes from Juvenile-Onset Systemic Lupus Erythematosus, but not from Juvenile Rheumatoid Arthritis and Juvenile Dermatomyositis

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Bernadete L. Liphaus

2006-01-01

Full Text Available Defective regulation of apoptosis may play a role in the development of autoimmune diseases. Fas and Bcl-2 proteins are involved in the control of apoptosis. The aims of this study were to determine the expression of Fas antigen and Bcl-2 protein on peripheral blood T and B lymphocytes from patients with juvenile-onset systemic lupus erythematosus (JSLE, juvenile rheumatoid arthritis (JRA and juvenile dermatomyositis (JDM. Thirty-eight patients with JSLE, 19 patients with JRA, 10 patients with JDM and 25 healthy controls entered the study. Freshly isolated peripheral blood mononuclear cells (PBMC were stained for lymphocyte markers CD3, CD4, CD8, CD19 and for Fas and Bcl-2 molecules. Expressions were measured by three-color flow cytometry. Statistical analysis was performed using Kruskal–Wallis test. Percentages of freshly isolated T lymphocytes positively stained for Fas protein from JSLE patients were significantly increased compared to healthy controls, patients with JRA and patients with JDM. Percentages of B lymphocytes positive for Fas from JSLE patients were higher than healthy controls and JRA patients. In addition, Fas expression on T cells from patients with JRA was increased compared to JDM patients. Otherwise, Fas expression on T and B cells from JRA and JDM patients were similar to healthy controls. MFI of Bcl-2 positive T lymphocytes from JSLE patients were significantly increased compared to healthy controls and JRA patients. MFI of Bcl-2 protein on B lymphocytes from JSLE patients was similar to healthy controls and patients with JRA and JDM. Bcl-2 expression did not differ between JRA and JDM patients and healthy controls. In conclusion, increased expression of Fas and Bcl-2 proteins observed in circulating T and B lymphocytes from patients with JSLE, but not from patients with JRA and JDM, suggests that abnormalities of apoptosis may be related to the pathogenesis of JSLE and probably are not a result of chronic inflammation.

A new mass mortality of juvenile Protoceratops and size-segregated aggregation behaviour in juvenile non-avian dinosaurs.

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David W E Hone

Full Text Available BACKGROUND: Monodominant bonebeds are a relatively common occurrence for non-avian dinosaurs, and have been used to infer associative, and potentially genuinely social, behavior. Previously known assemblages are characterized as either mixed size-classes (juvenile and adult-sized specimens together or single size-classes of individuals (only juveniles or only adult-sized individuals within the assemblage. In the latter case, it is generally unknown if these kinds of size-segregated aggregations characterize only a particular size stage or represent aggregations that happened at all size stages. Ceratopsians ("horned dinosaurs" are known from both types of assemblages. METHODS/PRINCIPAL FINDINGS: Here we describe a new specimen of the ceratopsian dinosaur Protoceratops andrewsi, Granger and Gregory 1923 from Mongolia representing an aggregation of four mid-sized juvenile animals. In conjunction with existing specimens of groups of P. andrewsi that includes size-clustered aggregations of young juveniles and adult-sized specimens, this new material provides evidence for some degree of size-clustered aggregation behaviour in Protoceratops throughout ontogeny. This continuity of size-segregated (and presumably age-clustered aggregation is previously undocumented in non-avian dinosaurs. CONCLUSIONS: The juvenile group fills a key gap in the available information on aggregations in younger ceratopsians. Although we support the general hypothesis that many non-avian dinosaurs were gregarious and even social animals, we caution that evidence for sociality has been overstated and advocate a more conservative interpretation of some data of 'sociality' in dinosaurs.

A new mass mortality of juvenile Protoceratops and size-segregated aggregation behaviour in juvenile non-avian dinosaurs.

Science.gov (United States)

Hone, David W E; Farke, Andrew A; Watabe, Mahito; Shigeru, Suzuki; Tsogtbaatar, Khishigjav

2014-01-01

Monodominant bonebeds are a relatively common occurrence for non-avian dinosaurs, and have been used to infer associative, and potentially genuinely social, behavior. Previously known assemblages are characterized as either mixed size-classes (juvenile and adult-sized specimens together) or single size-classes of individuals (only juveniles or only adult-sized individuals within the assemblage). In the latter case, it is generally unknown if these kinds of size-segregated aggregations characterize only a particular size stage or represent aggregations that happened at all size stages. Ceratopsians ("horned dinosaurs") are known from both types of assemblages. Here we describe a new specimen of the ceratopsian dinosaur Protoceratops andrewsi, Granger and Gregory 1923 from Mongolia representing an aggregation of four mid-sized juvenile animals. In conjunction with existing specimens of groups of P. andrewsi that includes size-clustered aggregations of young juveniles and adult-sized specimens, this new material provides evidence for some degree of size-clustered aggregation behaviour in Protoceratops throughout ontogeny. This continuity of size-segregated (and presumably age-clustered) aggregation is previously undocumented in non-avian dinosaurs. The juvenile group fills a key gap in the available information on aggregations in younger ceratopsians. Although we support the general hypothesis that many non-avian dinosaurs were gregarious and even social animals, we caution that evidence for sociality has been overstated and advocate a more conservative interpretation of some data of 'sociality' in dinosaurs.

Association of neopterin as a marker of immune system activation and juvenile rheumatoid arthritis activity

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Mones M. Abu Shady

2015-08-01

Full Text Available OBJECTIVE: To evaluate neopterin plasma concentrations in patients with active juvenile idiopathic arthritis (JIA and correlate them with disease activity.METHODS: Sixty patients diagnosed as active JIA, as well as another 60 apparently healthy age- and gender-matched children as controls, were recruited from the Pediatrics Allergy and Immunology Clinic, Ain Shams University. Disease activity was assessed by the Juvenile Arthritis Disease Activity Score 27 (JADAS-27. Laboratory investigations were performed for all patients, including determination of hemoglobin concentration (Hgb, erythrocyte sedimentation rate (ESR, and C-reactive protein. Serum concentrations of tumor necrosis factor-alpha (TNF-a, interleukin-6 (IL-6, monocyte chemoattractant protein-1 (MCP-1, and neopterin were measured.RESULTS: Significant differences were found between JIA patients and controls with regard to the mean levels of Hgb, ESR, TNF-a, IL-6, and MCP-1 (p 0.05. Multiple linear regression analysis showed that JADAS- 27 and ESR were the main variables associated with serum neopterin in JIA patients (p < 0.05.CONCLUSION: The elevation of plasma neopterin concentrations in early JIA patients may indicate stimulation of immune response. Serum neopterin can be used as a sensitive marker for assaying background inflammation and disease activity score in JIA patients.

JUVENILE RHEUMATOID ARTHRITIS

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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.

Sports Participation and Juvenile Delinquency: A Meta-Analytic Review.

Science.gov (United States)

Spruit, Anouk; van Vugt, Eveline; van der Put, Claudia; van der Stouwe, Trudy; Stams, Geert-Jan

2016-04-01

Participation in sports activities is very popular among adolescents, and is frequently encouraged among youth. Many psychosocial health benefits in youth are attributed to sports participation, but to what extent this positive influence holds for juvenile delinquency is still not clear on both the theoretical and empirical level. There is much controversy on whether sports participation should be perceived as a protective or a risk factor for the development of juvenile delinquency. A multilevel meta-analysis of 51 published and unpublished studies, with 48 independent samples containing 431 effect sizes and N = 132,366 adolescents, was conducted to examine the relationship between sports participation and juvenile delinquency and possible moderating factors of this association. The results showed that there is no overall significant association between sports participation and juvenile delinquency, indicating that adolescent athletes are neither more nor less delinquent than non-athletes. Some study, sample and sports characteristics significantly moderated the relationship between sports participation and juvenile delinquency. However, this moderating influence was modest. Implications for theory and practice concerning the use of sports to prevent juvenile delinquency are discussed.

Moral development of solo juvenile sex offenders

NARCIS (Netherlands)

van Vugt, E.; Stams, G.J.; Dekovic, M.; Brugman, D.; Rutten, E.; Hendriks, J.

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

Cardiovascular assessment of asymptomatic patients with juvenile-onset localized and systemic scleroderma: 10 years prospective observation.

Science.gov (United States)

Borowiec, A; Dabrowski, R; Wozniak, J; Jasek, S; Chwyczko, T; Kowalik, I; Musiej-Nowakowska, E; Szwed, H

2012-02-01

The aim of the present study was non-invasive evaluation of the cardiovascular system in asymptomatic young adult patients with juvenile localized scleroderma (JLS) and juvenile systemic sclerosis (JSS). A group of 34 consecutive children with scleroderma were prospectively observed in the study. The control group (CG) consisted of 20 healthy subjects. In each subject 12-lead electrocardiographic, echocardiographic, ECG Holter, and ambulatory blood pressure monitoring examinations were performed at the baseline visit and after 10 years. Additionally, B-type natriuretic peptide (BNP) concentrations were measured after 10 years. Examinations were performed in 13 patients with JLS and 15 with JSS at the final visit. Two children had died (one from each group). Four patients were alive but refused the final visit. After 10 years, a higher prevalence of ventricular extrasystoles (p = 0.01) and an elevated pulmonary arterial pressure (JLS: p = 0.04, JSS: p = 0.03) were observed in both groups, but in comparison with the controls there was no significant difference at the final visit. In JLS patients more cases of left ventricle diastolic dysfunction, hypertension, and sinus tachycardia were diagnosed at the final visit (p ≤ 0.05). More atrioventricular block episodes in both groups of scleroderma patients were observed. Over the 10 years, arterial hypertension was diagnosed in three patients from the JLS group and in two with JSS. There were no significant differences in BNP concentrations at the final visit. The results of the present study show that juvenile scleroderma seems to be more benign than adult-onset disease. This observational study shows subclinical, not severe, cardiac abnormalities in adult patients with juvenile-onset disease.

Alteration of fecal microbiota profiles in juvenile idiopathic arthritis. Associations with HLA-B27 allele and disease status.

Directory of Open Access Journals (Sweden)

Monica Di Paola

2016-10-01

Full Text Available Alteration of gut microbiota is involved in several chronic inflammatory and autoimmune diseases, including rheumatoid arthritis, and gut microbial pro-arthritogenic profiles have been hypothesized. Intestinal inflammation may be involved in spondyloarthropathies and in a subset of patients affected by Juvenile Idiopathic Arthritis (JIA, the most common chronic rheumatic disease of childhood. We compared the fecal microbiota composition of JIA patients with healthy subjects (HS, evaluating differences in microbial profiles between sub-categories of JIA, such as enthesitis-related arthritis (JIA-ERA, in which inflammation of entheses occurs, and polyarticular JIA, non-enthesitis related arthritis (JIA-nERA. Through taxon-level analysis, we discovered alteration of fecal microbiota components that could be involved in subclinical gut inflammation, and promotion of joint inflammation. We observed abundance in Ruminococcaceae in both JIA categories, reduction in Clostridiaceae and Peptostreptococcaceae in JIA-ERA, and increase in Veillonellaceae in JIA-nERA, respectively compared with HS. Among the more relevant genera, we found an increase in Clostridium cluster XIVb, involved in colitis and arthritis, in JIA-ERA patients compared with HS, and a trend of decrease in Faecalibacterium, known for anti-inflammatory properties, in JIA-nERA compared with JIA-ERA and HS. Differential abundant taxa identified JIA patients for the HLA-B27 allele, including Bilophila, Clostridium cluster XIVb, Oscillibacter and Parvimonas. Prediction analysis of metabolic functions showed that JIA-ERA metagenome was differentially enriched in bacterial functions related to cell motility and chemotaxis, suggesting selection of potential virulence traits. We also discovered differential microbial profiles and intra-group variability among active disease and remission, suggesting instability of microbial ecosystem in autoimmune diseases with respect to healthy status. Similarly

Avaliação de provas de fase aguda em crianças e adolescentes com artrite idiopática juvenil e sua correlação com atividade da doença Acute phase reactants evaluation in children and adolescents with juvenile idiopathic arthritis and its correlation with disease activity

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Aline Alencar M. F. Nicácio

2009-06-01

Full Text Available OBJETIVO:Analisar a relação entre as provas de fase aguda e a atividade clínica da artrite idiopática juvenil e avaliar a concordância entre velocidade de hemossedimentação e proteína C reativa (VHS e PCR na fase aguda da doença. MÉTODOS: Foi realizado estudo retrospectivo tipo coorte a partir da análise de prontuários de 30 crianças e adolescentes que preenchiam os critérios diagnósticos para artrite idiopática juvenil, estavam em atendimento em ambulatório de Reumatologia Pediátrica e haviam realizado as provas de fase aguda (VHS e PCR. RESULTADOS: Dos 30 pacientes, 21 (70% eram do sexo feminino e 19 (63,3% apresentavam o subtipo oligoarticular da doença. A média de idade de início dos sintomas foi 65,6 meses, a idade de diagnóstico de 85,3 e o tempo de evolução, 57,2 meses. As provas de fase aguda mostraram associação positiva com a atividade de doença. A anemia não teve relação com a atividade de doença. A concordância entre as duas provas de fase aguda foi superior a 80%. CONCLUSÕES: As provas de fase aguda mantêm relação positiva com a atividade da doença e o seu uso concomitante aumenta a especificidade.OBJECTIVE:To analyze the relationship between the acute phase reactants and the disease activity of Juvenile Idiopathic Arthritis (JIA and to evaluate the agreement between erythrocyte sedimentation rate and C-reactive protein during the acute phase of the disease. METHODS: a cohort retrospective study has been conducted based on the analysis of 30 children and adolescents who fulfilled the diagnostic criteria of JIA. All of them were in current follow-up at the pediatric rheumatology outpatient clinic and had acute phase reactants blood tests performed. RESULTS: Studied population comprised 30 patients: 21 (70% of them were females and 19 (63.3% presented oligoarticular subtype. The mean age at disease onset was 65.6 months; the age at diagnosis was 85.3 months and the follow-up had 57.2 months of

Bone scintigraphy and metabolic disorders

International Nuclear Information System (INIS)

Mari', C.; Catafau, A.; Carrio', I.

1999-01-01

The paper discusses the main clinical value of bone scan in metabolic bone disease: its detection of focal conditions or focal complications of such generalized disease, its most common use of being the detection of fractures in osteoporosis, pseudofractures in osteomalacia and the evaluation of Paget's disease

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...

Adenoviral infection in a collection of juvenile inland bearded dragons (Pogona vitticeps).

Science.gov (United States)

Doneley, R J T; Buckle, K N; Hulse, L

2014-01-01

Juvenile inland bearded dragons (Pogona vitticeps) from a breeding collection in south-east Queensland were presented at age 6-10 weeks with neurological signs, poor growth and occasional deaths. Histopathological examination revealed that six of eight lizards had multifocal non-suppurative hepatitis associated with 5-10 μm diameter, smudgy, basophilic, hyaline intranuclear inclusion bodies that marginated the nuclear chromatin. These histological lesions were considered consistent with adenoviral hepatitis. Infection with adenovirus was confirmed positive in one of the eight dragons by PCR for adenoviral DNA. DNA was extracted from formalin-fixed paraffin-embedded pooled tissues of the juvenile inland bearded dragons and tested using a nested-PCR protocol with primers specific for identification of adenovirus. Sequencing of the one PCR-positive dragon showed 95% nucleotide sequence alignment with agamid atadenovirus 1. Further investigation involved testing the breeding population, including the parents of the affected juveniles. Blood and cloacal samples were collected from the adult population, DNA was extracted and tested by PCR for adenovirus. There was a high percentage of positive results from the samples collected from the breeding population. This is the first reported group outbreak of adenoviral disease in bearded dragons in Australia. © 2014 Australian Veterinary Association.

Common Marmosets: A Potential Translational Animal Model of Juvenile Depression

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Nicole Leite Galvão-Coelho

2017-09-01

Full Text Available Major depression is a psychiatric disorder with high prevalence in the general population, with increasing expression in adolescence, about 14% in young people. Frequently, it presents as a chronic condition, showing no remission even after several pharmacological treatments and persisting in adult life. Therefore, distinct protocols and animal models have been developed to increase the understanding of this disease or search for new therapies. To this end, this study investigated the effects of chronic social isolation and the potential antidepressant action of nortriptyline in juvenile Callithrix jacchus males and females by monitoring fecal cortisol, body weight, and behavioral parameters and searching for biomarkers and a protocol for inducing depression. The purpose was to validate this species and protocol as a translational model of juvenile depression, addressing all domain criteria of validation: etiologic, face, functional, predictive, inter-relational, evolutionary, and population. In both sexes and both protocols (IDS and DPT, we observed a significant reduction in cortisol levels in the last phase of social isolation, concomitant with increases in autogrooming, stereotyped and anxiety behaviors, and the presence of anhedonia. The alterations induced by chronic social isolation are characteristic of the depressive state in non-human primates and/or in humans, and were reversed in large part by treatment with an antidepressant drug (nortriptyline. Therefore, these results indicate C. jacchus as a potential translational model of juvenile depression by addressing all criteria of validation.

Orofacial pain, jaw function, and temporomandibular disorders in adult women with a history of juvenile chronic arthritis or persistent juvenile chronic arthritis

DEFF Research Database (Denmark)

Bakke, M.; Zak, M.; Jensen, B.L.

2001-01-01

Orofacial pain, jaw function, temporomandibular disorders, adult women persistent juvenil chronic arthritis......Orofacial pain, jaw function, temporomandibular disorders, adult women persistent juvenil chronic arthritis...

Quantitative MR characterization of disease activity in the knee in children with juvenile idiopathic arthritis: a longitudinal pilot study

International Nuclear Information System (INIS)

Workie, Dagnachew W.; Graham, T.B.; Laor, Tal; Racadio, Judy M.; Rajagopal, Akila; O'Brien, Kendall J.; Bommer, Wendy A.; Shire, Norah J.; Dardzinski, Bernard J.

2007-01-01

The development of a quantifiable and noninvasive method of monitoring disease activity and response to therapy is vital for arthritis management. The purpose of this study was to investigate the utility of quantitative dynamic contrast-enhanced MRI (DCE-MRI) based on pharmacokinetic (PK) modeling to evaluate disease activity in the knee and correlate the results with the clinical assessment in children with juvenile idiopathic arthritis (JIA). A group of 17 children with JIA underwent longitudinal clinical and laboratory assessment and DCE-MRI of the knee at enrollment, 3 months, and 12 months. A PK model was employed using MRI signal enhancement data to give three parameters, K trans ' (min -1 ), k ep (min -1 ), and V p ' and to calculate synovial volume. The PK parameters, synovial volumes, and clinical and laboratory assessments in most children were significantly decreased (P < 0.05) at 12 months when compared to the enrollment values. There was excellent correlation between the PK and synovial volume and the clinical and laboratory assessments. Differences in MR and clinical parameter values in individual subjects illustrate persistent synovitis when in clinical remission. A decrease in PK parameter values obtained from DCE-MRI in children with JIA likely reflects diminution of disease activity. This technique may be used as an objective follow-up measure of therapeutic efficacy in patients with JIA. MR imaging can detect persistent synovitis in patients considered to be in clinical remission. (orig.)

Juvenile Obesity, Physical Activity, and Lifestyle Changes.

Science.gov (United States)

Bar-Or, Oded

2000-01-01

Because many obese children become obese adults, the recent rapid increase in juvenile obesity poses a major public health challenge. Enhanced physical activity is a cornerstone in a multidisciplinary approach to preventing and treating juvenile obesity. Giving exercise recommendations focused for obese youth is critical. Cutting down on sedentary…

Preliminary evidence of altered biomechanics in adolescents with juvenile fibromyalgia.

Science.gov (United States)

Sil, Soumitri; Thomas, Staci; DiCesare, Christopher; Strotman, Daniel; Ting, Tracy V; Myer, Gregory; Kashikar-Zuck, Susmita

2015-01-01

Juvenile fibromyalgia (FM) is characterized by chronic musculoskeletal pain and marked reduction in physical activity. Despite recommendations for exercise to manage juvenile FM pain, exercise adherence is poor. Because of pain and activity avoidance, adolescents with juvenile FM are at risk for altered joint mechanics that may make them susceptible to increased pain and reduced tolerance for exercise. The primary aim of this study was to assess functional deficits in patients with juvenile FM compared to healthy controls using objective biomechanical assessment. Female adolescent patients with juvenile FM (n = 17) and healthy controls (n = 14) completed biomechanical assessments, including gait analysis and tests of lower extremity strength (isokinetic knee extension/flexion and hip abduction) and functional performance (drop vertical jump test) along with self-reported measures of disability (Functional Disability Inventory), pain intensity, depressive symptoms (Children's Depression Inventory), and fear of movement (Tampa Scale of Kinesiophobia). Patients with juvenile FM demonstrated mild deficiencies in walking gait and functional performance (P < 0.05 for both) and significantly lower left knee extension and flexion strength (18-22% deficit) and bilateral hip abduction strength (34-38%) compared with healthy controls (P < 0.008 for all). Patients with juvenile FM reported significantly higher functional disability, pain intensity, depressive symptoms, and fear of movement relative to controls (P < 0.01 for all). This study showed that adolescents with juvenile FM exhibited objective alterations in biomechanics and self-reported fear of movement that may have reinforced their activity avoidance. Interventions for juvenile FM should include a focus on correcting functional deficits and instilling greater confidence in adolescents with juvenile FM to engage in exercise to improve functional outcomes. Copyright © 2015 by the American College of Rheumatology.

Examination of the influence of juvenile Atlantic salmon on the feeding mode of juvenile steelhead in Lake Ontario tributaries

Science.gov (United States)

Johnson, James H.; Waldt, Emily M.

2014-01-01

We examined diets of 1204 allopatric and sympatric juvenile Atlantic salmon (Salmo salar) and steelhead (Oncorhynchus mykiss) in three tributaries of Lake Ontario. The diet composition of both species consisted primarily of ephemeropterans, trichopterans, and chironomids, although juvenile steelhead consumed more terrestrial invertebrates, especially at the sympatric sites. Subyearlings of both species consumed small prey (i.e. chironomids) whereas large prey (i.e. perlids) made up a higher percentage of the diet of yearlings. The diet of juvenile steelhead at the allopatric sites was more closely associated with the composition of the benthos than with the drift, but was about equally associated with the benthos and drift at the sympatric sites. The diet of both subyearling and yearling Atlantic salmon was more closely associated with the benthos than the drift at the sympatric sites. The evidence suggests that juvenile steelhead may subtly alter their feeding behavior in sympatry with Atlantic salmon. This behavioral adaptation may reduce competitive interactions between these species.

THERAPEUTIC CAPABILITIES OF ETHANERCEPT IN TREATMENT OF SYSTEMIC JUVENILE RHEUMATOID ARTHRITIS

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E.I. Alexeeva

2011-01-01

Full Text Available The article presents a clinical case of severe systemic juvenile rheumatoid arthritis, refractory to traditional immunosuppressive treatment and with insufficient efficacy of chimeric homogenous anti-TNF-a antibodies. The patient received 2 courses of anti-Blymphocyte’s- CD20-antibodies that helped to arrest all systemic manifestations of the disease. Articular syndrome was arrested only with the help of soluble receptors to TNF a — ethanercept, given in following dose — 0.4 mg per kg of body mass. The patient has been on treatment with ethanercept for 24 weeks. Joint tenderness and exudation were diminished already after the first 4 weeks of treatment, also there was a dramatic increase in joint motion range. After 6 months of therapy we have managed to improve the patients and his family quality of life.Key words: children, juvenile rheumatoid arthritis, ethanercept, treatment.(Voprosy sovremennoi pediatrii — Current Pediatrics. 2011; 10 (3: 141–149

Juvenile idiopathic arthritis-associated uveitis.

Science.gov (United States)

Clarke, Sarah L N; Sen, Ethan S; Ramanan, Athimalaipet V

2016-04-27

Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease of childhood, with JIA-associated uveitis its most common extra-articular manifestation. JIA-associated uveitis is a potentially sight-threatening condition and thus carries a considerable risk of morbidity. The aetiology of the condition is autoimmune in nature with the predominant involvement of CD4(+) T cells. However, the underlying pathogenic mechanisms remain unclear, particularly regarding interplay between genetic and environmental factors. JIA-associated uveitis comes in several forms, but the most common presentation is of the chronic anterior uveitis type. This condition is usually asymptomatic and thus screening for JIA-associated uveitis in at-risk patients is paramount. Early detection and treatment aims to stop inflammation and prevent the development of complications leading to visual loss, which can occur due to both active disease and burden of disease treatment. Visually disabling complications of JIA-associated uveitis include cataracts, glaucoma, band keratopathy and macular oedema. There is a growing body of evidence for the early introduction of systemic immunosuppressive therapies in order to reduce topical and systemic glucocorticoid use. This includes more traditional treatments, such as methotrexate, as well as newer biological therapies. This review highlights the epidemiology of JIA-associated uveitis, the underlying pathogenesis and how affected patients may present. The current guidelines and criteria for screening, diagnosis and monitoring are discussed along with approaches to management.

Intelligence Score Profiles of Female Juvenile Offenders

Science.gov (United States)

Werner, Shelby Spare; Hart, Kathleen J.; Ficke, Susan L.

2016-01-01

Previous studies have found that male juvenile offenders typically obtain low scores on measures of intelligence, often with a pattern of higher scores on measures of nonverbal relative to verbal tasks. The research on the intelligence performance of female juvenile offenders is limited. This study explored the Wechsler Intelligence Scale for…

Penalty responsibility of juveniles in the Republic of Srpska

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Grbić-Pavlović Nikolina

2011-01-01

Full Text Available The youngest members of organized society, more intensive than ever enter the circle of those whose behavior is deviant. Juvenile delinquency is a social problem, which recently experienced an expansion in all modern countries, including Bosnia and Herzegovina and the Republic of Srpska. Considering the fact that juvenile delinquency includes lighter criminal conducts, such as, for example misdemeanors, in this paper a position of juveniles when they are a perpetrators of misdemeanors will be analyzed. Also, the paper will statistically show the number of misdemeanors in the field of public peace and order that juveniles conducted in the Republic of Srpska in the period 2004-2009.

Seasonal dynamics of Posthodiplostomum cuticola (Digenea, Diplostomatidae) metacercariae and parasite-enhanced growth of juvenile host fish

Czech Academy of Sciences Publication Activity Database

Ondračková, Markéta; Reichard, Martin; Jurajda, Pavel; Gelnar, M.

2004-01-01

Roč. 93, č. 2 (2004), s. 131-136 ISSN 0932-0113 R&D Projects: GA ČR GA524/02/0924 Institutional research plan: CEZ:AV0Z6093917 Keywords : black-spot disease * juvenile fish Subject RIV: EG - Zoology Impact factor: 1.060, year: 2004

Delincuencia y responsabilidad penal juvenil en Colombia

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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

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

Science.gov (United States)

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.

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...... idiopathic arthritis (JIA), and rheumatoid arthritis (RA) share periodontal and hematological characteristics distinguishing them from individuals free of diseases....

Optical Biopsy of Human Skin in Conjunction With Laser Treatment

Science.gov (United States)

2017-02-08

Malignant Melanoma; Merkel Cell Carcinoma; Basal Cell Carcinoma; Squamous Cell Carcinoma; Atypical Nevi; Congenital Nevi; Seborrheic Keratosis; Paget's Disease; Dermatofibroma; Kaposi's Sarcoma; Port Wine Stain; Hemangioma; Tattoos; Scleroderma; Burns

Yolo Bypass Juvenile Salmon Utilization Study 2016—Summary of acoustically tagged juvenile salmon and study fish release, Sacramento River, California

Science.gov (United States)

Liedtke, Theresa L.; Hurst, William R.

2017-09-12

The Yolo Bypass is a flood control bypass in Sacramento Valley, California. Flood plain habitats may be used for juvenile salmon rearing, however, the potential value of such habitats can be difficult to evaluate because of the intermittent nature of inundation events. The Yolo Bypass Juvenile Salmon Utilization Study (YBUS) used acoustic telemetry to evaluate the movements and survival of juvenile salmon adjacent to and within the Yolo Bypass during the winter of 2016. This report presents numbers, size data, and release data (times, dates, and locations) for the 1,197 acoustically tagged juvenile salmon released for the YBUS from February 21 to March 18, 2016. Detailed descriptions of the surgical implantation of transmitters are also presented. These data are presented to support the collaborative, interagency analysis and reporting of the study findings.

IL-6 blockade in systemic juvenile idiopathic arthritis - achievement of inactive disease and remission (data from the German AID-registry).

Science.gov (United States)

Bielak, M; Husmann, E; Weyandt, N; Haas, J-P; Hügle, B; Horneff, G; Neudorf, U; Lutz, T; Lilienthal, E; Kallinich, T; Tenbrock, K; Berendes, R; Niehues, T; Wittkowski, H; Weißbarth-Riedel, E; Heubner, G; Oommen, P; Klotsche, J; Foell, Dirk; Lainka, E

2018-04-05

Systemic juvenile idiopathic arthritis (sJIA) is a complex disease with an autoinflammatory component of unknown etiology related to the innate immune system. A major role in the pathogenesis has been ascribed to proinflammatory cytokines like interleukin-6 (IL-6), and effective drugs inhibiting their signaling are being developed. This study evaluates sJIA patients treated with the IL-6 inhibitor tocilizumab (TCZ) concerning clinical response rate, disease course and adverse effects in a real-life clinical setting. In 2009 a clinical and research consortium was established, including an online registry for autoinflammatory diseases (AID) ( https://aid-register.de ). Data for this retrospective TCZ study were documented by 13 centers. From 7/2009 to 4/2014, 200 patients with sJIA were recorded in the AID-registry. Out of these, 46 (19 m, 27 f, age 1-18 years) received therapy with TCZ. Long term treatment (median 23 months) has been documented in 24/46 patients who were evaluated according to Wallace criteria (active disease 6/24, inactive disease 5/24, remission 13/24 cases). Under observation co-medication were used in 40/46 cases. Adverse events were reported in 11/46 patients. The clinical response rate (no clinical manifestation, no increased inflammation parameters) within the first 12 weeks of treatment was calculated to be 35%. Out of 200 sJIA children reported in the German AID-registry, 46 were treated with TCZ, showing a clinical response rate of 35% during the first 12 weeks, and inactive disease and/or remission under medication in 75% after one year. Adverse events were seen in 24% and severe adverse events in 4%. The AID-Registry is funded by the BMBF (01GM08104, 01GM1112D, 01GM1512D).

Prevalence of traumatic brain injury in juvenile offenders: a meta-analysis.

Science.gov (United States)

Farrer, Thomas J; Frost, R Brock; Hedges, Dawson W

2013-01-01

Studies of traumatic brain injury (TBI) among adult populations demonstrate that such injuries can lead to aggressive behaviors. Related findings suggest that incarcerated individuals have high rates of brain injuries. Such studies suggest that traumatic brain injury may be related to the etiology and recidivism of criminal behavior. Relatively few studies have examined the prevalence of TBI using a delinquent juvenile sample. In order to assess the relationship between TBI and juvenile offender status, the current study used meta-analytic techniques to examine the odds of having a TBI among juvenile offenders. Across 9 studies, we found that approximately 30% of juvenile offenders have sustained a previous brain injury. Across 5 studies that used a control group, a calculated summary odds ratio of 3.37 suggests that juvenile offenders are significantly more likely to have a TBI compared to controls. Results suggest that the rate of TBIs within the juvenile offender population is significant and that there may be a relationship between TBIs and juvenile criminal behavior.

Treatment of juvenile idiopathic arthritis-associated uveitis: challenges and update.

Science.gov (United States)

Rabinovich, C Egla

2011-09-01

To update the current understanding of the risk factors for poor outcomes in juvenile idiopathic arthritis-related uveitis. In addition, current therapies, both traditional and biological, are reviewed. Male sex, independent of age or antinuclear antibody status, is associated with increased ocular morbidity. Having anterior chamber inflammation on first exam increases the risk of developing vision-threatening eye complications. Presence of one complication increases the risk of developing another. Risk of cataract development associated with topical glucocorticoid use is better defined. Longer duration of remission on therapy has been found to decrease the risk of disease flare after discontinuation of methotrexate. Recent studies of both nonbiological and biological therapies for arthritis-related uveitis are discussed. With a better understanding of risk factors associated with the ocular morbidity of uveitis associated with juvenile idiopathic arthritis, aggressive therapies can be targeted for improved visual outcomes. Alternative treatments to avoid long-term corticosteroid use include the use of antimetabolites and biological therapies. More prospective comparator studies and/or use of multicenter databases are needed to better understand best treatments.

Juvenile polyposis syndrome: An unusual case report of anemia and gastrointestinal bleeding in young infant.

Science.gov (United States)

Hsiao, Yi-Han; Wei, Chin-Hung; Chang, Szu-Wen; Chang, Lung; Fu, Yu-Wei; Lee, Hung-Chang; Liu, Hsuan-Liang; Yeung, Chun-Yan

2016-09-01

Juvenile polyposis syndrome, a rare disorder in children, is characterized with multiple hamartomatous polyps in alimentary tract. A variety of manifestations include bleeding, intussusception, or polyp prolapse. In this study, we present an 8-month-old male infant of juvenile polyposis syndrome initially presenting with chronic anemia. To the best of our knowledge, this is the youngest case reported in the literature. We report a rare case of an 8-month-old male infant who presented with chronic anemia and gastrointestinal bleeding initially. Panendoscopy and abdominal computed tomography showed multiple polyposis throughout the entire alimentary tract leading to intussusception. Technetium-99m-labeled red blood cell (RBC) bleeding scan revealed the possibility of gastrointestinal tract bleeding in the jejunum. Histopathological examination on biopsy samples showed Peutz-Jeghers syndrome was excluded, whereas the diagnosis of juvenile polyposis syndrome was established. Enteroscopic polypectomy is the mainstay of the treatment. However, polyps recurred and occupied the majority of the gastrointestinal tract in 6 months. Supportive management was given. The patient expired for severe sepsis at the age of 18 months. Juvenile polyposis syndrome is an inherited disease, so it is not possible to prevent it. Concerning of its poor outcome and high mortality rate, it is important that we should increase awareness and education of the parents at its earliest stages.

Inherited Retinal Degenerative Disease Registry

Science.gov (United States)

2017-09-13

Eye Diseases Hereditary; Retinal Disease; Achromatopsia; Bardet-Biedl Syndrome; Bassen-Kornzweig Syndrome; Batten Disease; Best Disease; Choroidal Dystrophy; Choroideremia; Cone Dystrophy; Cone-Rod Dystrophy; Congenital Stationary Night Blindness; Enhanced S-Cone Syndrome; Fundus Albipunctatus; Goldmann-Favre Syndrome; Gyrate Atrophy; Juvenile Macular Degeneration; Kearns-Sayre Syndrome; Leber Congenital Amaurosis; Refsum Syndrome; Retinitis Pigmentosa; Retinitis Punctata Albescens; Retinoschisis; Rod-Cone Dystrophy; Rod Dystrophy; Rod Monochromacy; Stargardt Disease; Usher Syndrome

Miastenia gravis juvenil Juvenile myasthenia gravis

OpenAIRE

Oscar Papazian; Israel Alfonso; Nayle Araguez

2009-01-01

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 nerv...

Modelling population effects of juvenile offshore fish displacement towards adult habitat

DEFF Research Database (Denmark)

van de Wolfshaar, K.E.; Tulp, I.; Wennhage, H.

2015-01-01

consequences on population dynamics through changes in resource use and competition. To explore this, a conceptual stage-structured model was developed with 3 stages and 2 resources and allowing a move of large juveniles from the shallow to the deep habitat. Large juveniles compete with small juveniles...... in shallow waters and with adults in deeper waters. Alternative stable states occur, with one state dominated by small juvenile biomass and the other dominated by adult biomass. The model results show for both states that while large juvenile biomass responds to a change in time spent in the deep habitat...

Spatial dynamics of juvenile anchovy in the Bay of Biscay

KAUST Repository

Boyra, Guillermo

2016-07-08

In autumn 2009, the implementation of two successive acoustic surveys targeting juvenile anchovy (Engraulis encrasicolus) in the Bay of Biscay allowed us to monitor the changes in the spatial distribution and aggregation patterns of juveniles of this species during 45 days under fairly stable meteorological conditions. Juvenile anchovy changed its biological condition and behavior in a different manner in two distinct areas. In the Spanish sector, the juveniles migrated 20 nautical miles (n.mi.) towards the coast, but they remained on the shelf and near the surface during the whole surveyed period. As the advance towards the shelf break progressed, their area of distribution decreased, their density increased and the juveniles spread in fewer but heavier shoals. In the French sector, the juveniles also migrated from slope waters towards the coast at a similar velocity, but they crossed the shelf break into the continental shelf, where they increased their mean depth significantly until gradually adopting the typical nyctemeral migrations of adult anchovy. The mean length of the juveniles that adopted the nyctemeral migrations was significantly higher than that of the juveniles remaining at the surface, suggesting that body size is relevant to accomplish this change. Besides, the stronger temperature gradients between the shelf and oceanic waters in the Spanish sector, favored by a narrow shelf, may have acted as a barrier influencing the distinct observed spatial patterns in the two areas. © 2016 John Wiley & Sons Ltd

Spatial dynamics of juvenile anchovy in the Bay of Biscay

KAUST Repository

Boyra, Guillermo; Peñ a, Marian; Cotano, Unai; Irigoien, Xabier; Rubio, Anna; Nogueira, Enrique

2016-01-01

In autumn 2009, the implementation of two successive acoustic surveys targeting juvenile anchovy (Engraulis encrasicolus) in the Bay of Biscay allowed us to monitor the changes in the spatial distribution and aggregation patterns of juveniles of this species during 45 days under fairly stable meteorological conditions. Juvenile anchovy changed its biological condition and behavior in a different manner in two distinct areas. In the Spanish sector, the juveniles migrated 20 nautical miles (n.mi.) towards the coast, but they remained on the shelf and near the surface during the whole surveyed period. As the advance towards the shelf break progressed, their area of distribution decreased, their density increased and the juveniles spread in fewer but heavier shoals. In the French sector, the juveniles also migrated from slope waters towards the coast at a similar velocity, but they crossed the shelf break into the continental shelf, where they increased their mean depth significantly until gradually adopting the typical nyctemeral migrations of adult anchovy. The mean length of the juveniles that adopted the nyctemeral migrations was significantly higher than that of the juveniles remaining at the surface, suggesting that body size is relevant to accomplish this change. Besides, the stronger temperature gradients between the shelf and oceanic waters in the Spanish sector, favored by a narrow shelf, may have acted as a barrier influencing the distinct observed spatial patterns in the two areas. © 2016 John Wiley & Sons Ltd

Using the Juvenile Justice Poster. Teaching Strategy.

Science.gov (United States)

Update on Law-Related Education, 2000

2000-01-01

Presents a lesson that can help students review and summarize what they have learned about the juvenile justice system. Explains that the students discuss how the juvenile justice system can be improved and conduct a survey on how it might be changed in the future. Provides a copy of the survey and directions. (CMK)

Reformations in Zimbabwe's juvenile justice system | Ruparanganda ...

African Journals Online (AJOL)

Children in conflict with the law are often stigmatized and shunned by society as they are perceived as a threat to society. Historically, Zimbabwe's juvenile justice system has been retributive and focused on punishing the juvenile offender. As a result, it has been criticised from a number of viewpoints, including the need to ...

Juvenile Delinquency and Teenage Pregnancy: A Comparison of Ecological Risk Profiles among Midwestern White and Black Female Juvenile Offenders

Science.gov (United States)

Khurana, Atika; Cooksey, Elizabeth C.; Gavazzi, Stephen M.

2011-01-01

The authors examined ecological risk factors associated with teen pregnancy with a sample of 1,190 court-involved female juvenile offenders between 11 and 18 years of age. Data were obtained from five Midwestern juvenile county courts using a recently developed youth risk assessment instrument called the global risk assessment device (GRAD). In…

83 CHALLENGES AND PROSPECTS OF THE JUVENILE JUSTICE ...

African Journals Online (AJOL)

Fr. Ikenga

Juvenile justice administration in Nigeria is weak and has been given very .... The Nigerian criminal justice system, of which the juvenile justice system is an integral part, ... as instruments of security and justice but as weapons of oppression8.

Bilateral, independent juvenile nasopharyngeal angiofibroma: case report.

Science.gov (United States)

Mørkenborg, M-L; Frendø, M; Stavngaard, T; Von Buchwald, C

2015-10-01

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 and surgical strategy. A 21-year-old male presented with increasing bilateral nasal obstruction and discharge. Examination revealed tumours bilaterally and imaging demonstrated non-contiguous tumours. Pre-operative angiography showed strictly ipsilateral vascular supplies requiring bilateral embolisation. Radical removal performed as one-step, computer-assisted functional endoscopic sinus surgery was performed. The follow-up period was uncomplicated. This case illustrates the importance of suspecting bilateral juvenile nasopharyngeal angiofibroma in patients presenting with bilateral symptoms. Our management, including successful pre-operative planning, enabled one-step total removal of both tumours and rapid patient recovery.

Radium rentention and dosimetry in juvenile beagles

International Nuclear Information System (INIS)

Lloyd, R.D.; Jones, C.W.; Bruenger, F.W.; Atherton, D.R.; Mays, C.W.

1983-01-01

Retention of administered 226 Ra was substantially greater in beagles injected as 3-month-old juveniles than as 1.4-year-old adults, but the measured 222 Rn/ 226 Ra ratio in bone was significantly less in juveniles for about the first 600 days after injection. An equation that describes the total-body biological retention R in beagles injected with 226 Ra at 3 months of age at any time t (in days) after injection during the first 6.6 years is R = 0.331e/sup -0.206t/ + 0.245e/sup -0.00374t/ + 0.424e/sup -0.000114t/. The rate constant of the final term in the equation for juveniles is similar to that for young adults, suggesting that this component reflects the net turnover rate in the slowly remodeling component of adult bone. Compared to young adult beagles, animals injected as juveniles had a greater fraction of their retained 226 Ra in parts of the skeleton containing much cortical bone, such as paws, and a smaller fraction in those parts containing much trabecular bone

Race and the fragility of the legal distinction between juveniles and adults.

Directory of Open Access Journals (Sweden)

Aneeta Rattan

Full Text Available Legal precedent establishes juvenile offenders as inherently less culpable than adult offenders and thus protects juveniles from the most severe of punishments. But how fragile might these protections be? In the present study, simply bringing to mind a Black (vs. White juvenile offender led participants to view juveniles in general as significantly more similar to adults in their inherent culpability and to express more support for severe sentencing. Indeed, these differences in participants' perceptions of this foundational legal precedent distinguishing between juveniles and adults accounted for their greater support for severe punishment. These results highlight the fragility of protections for juveniles when race is in play. Furthermore, we suggest that this fragility may have broad implications for how juveniles are seen and treated in the criminal justice system.

Criminal history and future offending of juveniles convicted of the possession of child pornography.

Science.gov (United States)

Aebi, Marcel; Plattner, Belinda; Ernest, Melanie; Kaszynski, Katie; Bessler, Cornelia

2014-08-01

Most child pornography is distributed online. It is estimated that 3% to 15% of child pornography consumers are juveniles. The present study analyzed a consecutive sample of 54 male juveniles convicted of the possession of child pornography. Demographic characteristics, criminal history, and subsequent offending were assessed from criminal files and official reports. Juvenile possessors of child pornography were compared to three different groups of juveniles: Juvenile possessors of other illegal pornography (n = 42), juveniles who committed a sexual contact offense against a child (n = 64), and juveniles who committed a sexual contact offense against a peer or adult (n = 104). Juvenile possessors of child pornography were found to have downloaded the illegal material more frequently and over a longer time period than juvenile possessors of other illegal pornography. Furthermore, juvenile possessors of child pornography differed from juveniles who had committed a sexual contact offense in terms of demographics and showed fewer previous and subsequent offending than juveniles who sexually offended against a peer or adult. We conclude that juvenile possessors of child pornography need a specific target intervention focusing on dysfunctional Internet use and sexually deviant arousal. © The Author(s) 2013.

Consenso em reumatologia pediátrica: parte I - definição dos critérios de doença inativa e remissão em artrite idiopática juvenil/artrite reumatóide juvenil Consensus in pediatric rheumatology: part I - criteria definition of inactive disease and remission in juvenile idiopathic arthritis / juvenile rheumatoid arthritis

Directory of Open Access Journals (Sweden)

Claudia Machado

2005-02-01

Full Text Available Não há critérios universalmente aceitos para a remissão clínica em artrite idiopática juvenil/artrite reumatóide juvenil (AIJ/ARJ. OBJETIVO: formar consenso sobre estes critérios. MÉTODOS: foi utilizado um inquérito pelo método Delphi para reunir os critérios vigentes e utilizados por especialistas em reumatologia pediátrica (RP no mundo todo. A análise dos resultados constituiu a base para uma Consensus Conference utilizando a nominal group technique (NGT para alcançar o consenso nas questões não resolvidas após a análise dos questionários deste inquérito. Cento e trinta RP de 34 países responderam ao inquérito e 20 RP de nove países elegeram os critérios durante dois dias, em processo de discussão estruturada, para formar consenso pela NGT. RESULTADOS: os critérios de doença inativa deveriam incluir: 1 nenhuma articulação com artrite em atividade; 2 ausência de febre, rash, serosite, esplenomegalia ou linfadenopatia generalizada atribuída à AIJ/ARJ; 3 ausência de uveíte em atividade; 4 VHS ou PCR negativas (se ambos forem testados, ambos devem ser normais; 5 a avaliação global pelo médico deve indicar o melhor escore possível, indicando doença inativa. CONCLUSÕES: de acordo com o voto de consenso, seis meses contínuos de doença inativa são necessários para se considerar um paciente em estado de remissão com medicação; 12 meses contínuos de doença inativa e sem medicação são necessários para considerar um paciente em estado de remissão sem medicação. O critério para remissão sem medicação deve prever com acurácia de 95% a probabilidade inferior a 20% de recaída em cinco anos.Validated and widely accepted criteria for clinical remission in JIA/JRA do not currently exist. OBJECTIVE: To achieve consensus in this matter. METHODS: The Delphi consensus-formation approach was used to gather the criteria in use by pediatric rheumatologists (PR worldwide. Results from the questionnaires

Stridor: an unusual presentation of juvenile nasopharyngeal angiofibroma.

Science.gov (United States)

Singh, Hitendra Prakash; Kumar, Sunil; Vashishtha, Madhukar; Agarwal, Satya Prakash

2014-04-07

Nasopharyngeal angiofibroma is a rare and benign disease, which is mainly found in adolescent male subjects. It is usually diagnosed on clinical grounds on the basis of its presenting symptoms of nasal obstruction, nasal mass and most importantly unprovoked recurrent moderate to severe epistaxis. Imaging studies are only needed to confirm the diagnosis and formulate the management plan. A case of juvenile nasopharyngeal angiofibroma is presented here, which presented to us with severe respiratory distress and stridor. Urgent tracheostomy had to be performed before definitive management could be started. Definitive treatment was excision through modified transpalatal with sublabial route, which resulted in successful outcome.

IL-1 inhibition in systemic juvenile idiopathic arthritis

Directory of Open Access Journals (Sweden)

Gabriella Giancane

2016-12-01

Full Text Available Systemic juvenile idiopathic arthritis (sJIA is the form of childhood arthritis whose treatment is most challenging. The demonstration of the prominent involvement of interleukin (IL-1 in disease pathogenesis has provided the rationale for the treatment with biologic medications that antagonize this cytokine. The three IL-1 blockers that have been tested so far (anakinra, canakinumab and rilonacept have all been proven effective and safe, although only canakinumab is currently approved for use in sJIA. The studies on IL-1 inhibition in sJIA published in the past few years suggest that children with fewer affected joints, higher neutrophil count, younger age at disease onset, shorter disease duration, or, possibly, higher ferritin level may respond better to anti-IL-1 treatment. In addition, it has been postulated that use of IL-1 blockade as first-line therapy may take advantage of a window of opportunity, in which disease pathophysiology can be altered to prevent the occurrence of chronic arthritis. In this review, we analyze the published literature on IL-1 inhibitors in sJIA and discuss the rationale underlying the use of these medications, the results of therapeutic studies, and the controversial issues.

Growth of juvenile shrimp Metapenaeus monoceros fed with squid and mussel

Digital Repository Service at National Institute of Oceanography (India)

Achuthankutty, C.T.; Nair, S.R.S.; Krishnakumari, L.

Small juveniles of both sexes and females of large juveniles of Metapenaeus monoceros attained faster growth with squid diet. Males of large juveniles registered better growth with mussel diet. No significant difference was observed in moult weights...

Clinicopathologic evaluation of mammary Paget′s disease

Directory of Open Access Journals (Sweden)

Meibodi Naser

2008-01-01

Full Text Available Mammary and extramammary Paget′s diseases are rare neoplasms of epidermis and mucosal epithelium. Due to their nonspecific and variable clinical view, they have differential diagnosis with eczema, melanoma, Bowen′s disease, etc. To the best of our knowledge, no such study has been performed in Iran regarding the prevalence, clinical aspects, underlying disease and pathological characteristics of these two diseases. In this study, we have evaluated the clinical and histopathological aspects of this disorder. Materials and Methods: In this retrospective study, all Paget′s biopsied samples referred to the Pathology Department of Imam-Reza hospital, Mashhad, since 1984 till 2004 were evaluated. Collected data were analyzed by descriptive statistical methods. Results: Among 98925 specimens, there were 29 cases of Paget′s disease. All cases were married women suffering from mammary Paget. The mean age was 53 ± 11 years. Left and right breast involvement was observed in 17 and 12 cases, all unilateral. The most common clinical view was ulcerated (27% and then erythematosus exudative plaques. More than 50% of patients were symptomatic. Most common symptoms were itching, pain and burning. The exclusive underlying pathological diagnosis was ductal carcinoma (55%. Discussion: In most cases, the clinical view of mammary Paget′s disease was helpful. Unilateral ulcerated plaque was the most common clinical sign. Majority of the accompanying pathology was ductal carcinoma. We had no cases of extramammary Paget′s disease in our study.

[Benign cutaneous lymphadenosis (Bäfverstedt's disease) of the nipple (author's transl)].

Science.gov (United States)

Kindermann, G; Sebicke, E

1977-12-01

Benign circumscribed cutaneous lymphadenosis of the areola of the nipple (Bäfverstedt's disease) occurs in children and adults. We observed 11 cases: 3 men, 3 children and 6 women. The areola undergoes rapid swelling and becomes red. At times the nipple itself is included. Unilateral enlargment and distortion of the nipple area becomes apparent. Sometimes this is associated with a tumor-like infiltration of the retro-mammillary tissue. In Contradistinction to Pagets disease of the nipple and to a mammillary adenoma there is no eczema and no ulceration. The clinical features and the typical pruritic course establish the diagnosis. Healing is spontaneous, but protracted. The aetiology is probably of an inflammatory nature. Treatment with short courses of antibiotics and anti-inflammatory agents resulted in a faster reduction of the cutaneous signs and corroborated the clinical diagnosis. We consider surgical treatment as unnecessary because circumscript benign lymphadenosis of the nipple area has a harmless course.

Bone scintigraphy and metabolic disorders

Energy Technology Data Exchange (ETDEWEB)

Mari' , C.; Catafau, A.; Carrio' , I. [Hospital de Sant Pau, Barcelone (Spain). Serv. of Nuclear Medicine

1999-09-01

The paper discusses the main clinical value of bone scan in metabolic bone disease: its detection of focal conditions or focal complications of such generalized disease, its most common use of being the detection of fractures in osteoporosis, pseudo fractures in osteomalacia and the evaluation of Paget's disease.

Preoperative embolization for juvenile nasopharyngeal angiofibroma. Usefulness of IADSA and problems of external carotid ligation

Energy Technology Data Exchange (ETDEWEB)

Saitoh, Ryuichi; Nakagawa, Nobuo; Kohrogi, Yukinori; Izunaga, Hiroshi; Bussaka, Hiromasa; Miyawaki, Masayuki; Takahashi, Mutsumasa; Ikawa, Tsutomu; Ishikawa, Takashi (Kumamoto Univ. (Japan). School of Medicine)

1984-10-01

Three male cases of juvenile nasopharyngeal angiofibroma are presented. Satisfactory results were obtained by performing preoperative embolization combined with intraarterial digital subtraction angiography (IADSA) in two of them. The other case had undergone external carotid ligation. Usefulness of IADSA in performing preoperative embolization for this disease and problems of external carotid ligation are discussed.

Iris juvenile xanthogranuloma in an infant - spontaneous hyphema and secondary glaucoma.

Science.gov (United States)

Pantalon, Anca; Ștefănache, Tudor; Danciu, Mihai; Zurac, Sabina; Chiseliță, Dorin

2017-01-01

Juvenile xanthogranuloma (JXG) is a benign histiocytic skin disorder mainly encountered during infancy and childhood. Although with multiple potential localizations, less than 1% of the cases exhibit ocular manifestations. Some of these might lead to serious complications, specifically, secondary glaucoma that can result in severe and blinding eye disease. The aim of the present case report was to demonstrate typical clinical features, emphasize the difficulties attributed when managing these patients and literature review. We present the case of 4 months old female baby with spontaneous hyphema and secondary unilateral glaucoma due to ocular JXG. The natural history and treatment of the condition were extremely difficult to handle due to multiple opinions in histopathology related to other severe conditions that resembled with the lesions detected in this case: myelomonocytic leukemia and Langerhans cell histiocytosis. Although a minority of patients with JXG have ocular involvement, recognition of this condition is important because a treatment delay can lead to serious complications, such as glaucoma and spontaneous hyphema, as in our case. A thorough differential diagnosis represents the key to a proper management plan in these patients, both on short and long term. "Triple disease" defined as JXG plus neurofibromatosis type 1 (NF-1) and juvenile chronic myelogenous leukemia (JCML) has been reported, but it was not confirmed in our patient.

Experience with conservative rehabilitation in patients with juvenile chronic arthritis

Directory of Open Access Journals (Sweden)

T. A. Shelepina

2016-01-01

Full Text Available Objective: to estimate a need for conservative rehabilitation treatment in patients with juvenile chronic arthritis (JCA.Material and methods. Data on the principles and procedures of rehabilitation treatment were analyzed in patients with JCA on the basis of 25- year experience. The need for these packages of measures in 1999, 2008, and 2014 was compared. Standard procedures for joints at different sites were described. According to the degree of joint functions, there were rehabilitation treatment packages: corrective, mobilization, and general health-improving.Results and discussion. All patients with juvenile arthritis need rehabilitation (physical, psychological, and social. Comparison of the total number of patients who had received rehabilitation treatment in 1999, 2008, and 2014 showed a small trend towards its reduction. This is due to the smaller number of patients with dysfunctions and to the larger number of those without movement disorders who had received adequate treatment in early periods of the disease. The high percentage of patients having limited joint functions needs a mobilization package. Analysis of the data available in the literature and the authors' experience may lead to the conclusion that all patients with JCA need exercise therapy. The latter is a major procedure for physical rehabilitation and should be included in the standards for adjuvant treatment during basic medical therapy. Emphasis is laid on the importance of the early initiation of treatment to prevent incapacitating deformity at early stages of the disease.

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.

OPTIMIZATION OF PHARMACEUTICAL CARE FOR JUVENILE RHEUMATOID ARTHRITIS AT THE REGIONAL LEVEL

OpenAIRE

A. A. Skripko; L. N. Geller; G. G. Radnaev; Т. V. Hadanova

2017-01-01

Clinical practice and content analysis of scientifi c literature indicate that for treatment of the immune-infl amatory disease called juvenile rheumatoid arthritis (JRA), the use of only basic medicines (BM) is not suffi cient.The aim of the study is the organization of pharmacotherapy of JRA patients by genetically engineered biological preparations (GEBP), affecting the immune processes of the body directly.Materials and methods: the theory of marketing, content analysis, the ABC-analysis,...

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.

Clinical and Biochemical Characteristics of Children with Juvenile Idiopathic Arthritis

International Nuclear Information System (INIS)

Ahmed, S.; Ali, S. R.; Ishaque, S.

2014-01-01

Objective: To determine the clinical and biochemical characteristics of children with Juvenile Idiopathic Arthritis (JIA) at a tertiary care centre in Karachi, Pakistan. Study Design: A descriptive study. Place and Duration of Study: Paediatric Rheumatology Clinic of The Aga Khan University Hospital (AKUH), Karachi, from January 2008 to December 2011. Methodology: Clinical and laboratory profile and outcome of children less than 15 years of age attending the Paediatric Rheumatology Clinic of the Aga Khan University, Karachi with the diagnosis of Juvenile Idiopathic Arthritis according to International League against Rheumatism were studied. These children were classified into different types of JIA; their clinical and laboratory characteristics, response to therapy and outcome was evaluated. Results: Sixty eight patients satisfying the criteria of International League against Rheumatism (ILAR) for Juvenile Idiopathic Arthritis were enrolled during the study period of four consecutive years, their age ranged from 9 months to 15 years. Mean age at onset was 6.45 +- 4.03 years while mean age at diagnosis was 7.60 +- 3.93 years. Polyarticular was the most predominant subtype with 37 (54%) patients, out of these, 9 (24%) were rheumatoid factor positive. An almost equal gender predisposition was observed. Fever and arthritis were the most common presenting symptoms, with only 2 patients presenting with uveitis. Conclusion: The clinico-biochemical characteristics of JIA at the study centre showed a pattern distinct with early onset of disease, high frequency of polyarticular type and a higher rheumatoid factor (QRA) and ANA positivity in girls. (author)

Larval and juvenile Pacific herring Clupea pallasii are not susceptible to infectious hematopoietic necrosis under laboratory conditions

Science.gov (United States)

Hart, L.M.; Traxler, G.S.; Garver, K.A.; Richard, J.; Gregg, J.L.; Grady, C.A.; Kurath, G.; Hershberger, P.K.

2011-01-01

Infectious hematopoietic necrosis (IHN) leads to periodic epidemics among certain wild and farmed fish species of the Northeast (NE) Pacific. The source of the IHN virus (IHNV) that initiates these outbreaks remains unknown; however, a leading hypothesis involves viral persistence in marine host species such as Pacific herring Clupea pallasii. Under laboratory conditions we exposed specific pathogen-free (SPF) larval and juvenile Pacific herring to 103 to 104 plaque-forming units (pfu) of IHNV ml–1 by waterborne immersion. Cumulative mortalities among exposed groups were not significantly different from those of negative control groups. After waterborne exposure, IHNV was transiently recovered from the tissues of larvae but absent in tissues of juveniles. Additionally, no evidence of viral shedding was detected in the tank water containing exposed juveniles. After intraperitoneal (IP) injection of IHNV in juvenile herring with 103 pfu, IHNV was recovered from the tissues of sub-sampled individuals for only the first 5 d post-exposure. The lack of susceptibility to overt disease and transient levels of IHNV in the tissues of exposed fish indicate that Pacific herring do not likely serve a major epizootiological role in perpetuation of IHNV among free-ranging sockeye salmon Oncorhynchus nerka and farmed Atlantic salmon Salmo salar in the NE Pacific.

28 CFR 0.94 - Office of Juvenile Justice and Delinquency Prevention.

Science.gov (United States)

2010-07-01

... Delinquency Prevention. 0.94 Section 0.94 Judicial Administration DEPARTMENT OF JUSTICE ORGANIZATION OF THE... Delinquency Prevention. The Office of Juvenile Justice and Delinquency Prevention is headed by an...., relating to juvenile delinquency, the improvement of juvenile justice systems and missing children. ...

Value of the region of interest technique in the scintigraphic diagnosis of primary bone tumors

International Nuclear Information System (INIS)

Buell, U.; Keyl, W.; Meister, P.; Pfeifer, J.P.; Hartel, P.; Muenchen Univ.

1981-01-01

Employing ROI-technique, a ratio Q was obtained from relating accumulation of 99 sup(m)Tc-MDP at the site of the bone lesion (n = 150) with that of contralateral non-involved osseous areas. Values of Q were correlated with histologic tumor diagnosis, its dignity and frequency. Values of Q of greater than 3.0 were found in 95% of all sarcomas, in 100% of the osteosarcomas but in only 3.8% of all benign bone tumors. Values ranging from 1.0 to 1.2 were exclusively measured in benign tumors (e.g., in 52% of juvenile bone cysts and in 67% of non-ossifing fibromas). Since the threshold - separating benign from malignant lesions - at Q = 3.0 was blurred by tumorlike lesions, metastases and especially by Paget's disease, this method does not precisely predict dignity. However, this method may complement radiographic evaluation with low values supporting the diagnosis of a benign lesion. The combined findings of radiography and these rations gained by nuclear imaging may help determine the pathway of a patient through further diagnosis and treatment. (orig.) [de

Parental Low Self-Control, Family Environments, and Juvenile Delinquency.

Science.gov (United States)

Meldrum, Ryan C; Connolly, George M; Flexon, Jamie; Guerette, Rob T

2016-10-01

Research consistently finds that low self-control is significantly correlated with delinquency. Only recently, however, have researchers started to examine associations between parental low self-control, family environments, and child antisocial behavior. Adding to this emerging area of research, the current study examines associations between parental low self-control, aspects of the family environment, and officially recoded juvenile delinquency among a sample (N = 101) of juveniles processed through a juvenile justice assessment facility located in the Southeastern United States. Furthermore, it considers whether aspects of family environments, particularly family cohesion, family conflict, and parental efficacy, mediate the influence of parental low self-control on delinquency. The results of a series of analyses indicate that parental low self-control is correlated with various aspects of family environments and juvenile delinquency, and that the association between parental low self-control and juvenile delinquency is mediated by family environments. Supplementary analyses also suggest that the association between parental low self-control and the family environment may be reciprocal. © The Author(s) 2015.

Temperament of juvenile delinquents with history of substance abuse.

Science.gov (United States)

Chang, Hsueh-Ling; Chen, Sue-Huei; Huang, Chien

2007-01-01

The etiological factors and interrelations of juvenile delinquents, with psychiatric morbidity and substance abuse have been continuously debated. Cloninger's Tridimensional Theory of Temperament has been reported to predict patterns of substance abuse and comorbidity. In the current study, we aimed to examine the usability of the theory in predicting juvenile delinquency and substance abuse. Sixty consecutive and newly incarcerated male delinquents with history of substance abuse were recruited from a juvenile correctional facility in northwestern Taiwan from January 2002 through December 2003. All subjects were assessed of their temperament, behavioral problems, and psychiatric disorders on an individual base. The juvenile delinquent subjects with childhood history of attention deficit and hyperactivity disorder (ADHD) were significantly younger, consumed less betel nuts, and had more siblings with history of drug abuse. Consistent with the results of Cloninger's studies, novelty seeking positively correlated to the amount of substance abuse, while harm avoidance inversely correlated in juvenile delinquents. Endemic trend of choice of substance abuse needs to be taken into consideration in future research projects.

Prevention and Firesetting: Juvenile Justice and Intervention Strategies.

Science.gov (United States)

Slavkin, Michael L.

2003-01-01

Examines the literature on preventing firesetting behavior in preadolescents and adolescents, suggesting the need for policies and programs designed to help juveniles by providing community support and stability. Alternatives to juvenile justice interventions include making changes in the home environment, acquiring a greater sense of self, and…

Neurofibromatosis Type 1 Diagnosed in a Child Based on Multiple Juvenile Xanthogranulomas and Juvenile Myelomonocytic Leukemia

DEFF Research Database (Denmark)

Jans, Sune R R; Schomerus, Eckhard; Bygum, Anette

2015-01-01

An association between juvenile xanthogranuloma (JXG), neurofibromatosis type 1 (NF1), and juvenile myelomonocytic leukemia (JMML) has been described in the literature but has only been documented in approximately 20 cases. We diagnosed a patient with NF1 at 25 months of age, before any cutaneous...... with chemotherapy and allogenic bone marrow transplantation. With increased awareness, patients with JXG and NF1 who develop symptoms possibly related to JMML, such as paleness, skin bleeding, cough, unexplained fever, and hepatosplenomegaly, should be further evaluated. We also emphasize that multiple JXG lesions...

Skeletal diseases. Diagnostic clinical radiology and differential diagnostics. 2. rev. and enl. ed.

International Nuclear Information System (INIS)

Freyschmidt, J.

1997-01-01

The book focuses on the diagnostic evaluation of idiopathic diseases of the skeleton and bone joints, also including the fundamental healing processes of bone fractures, particularly of stress-induced and pathologic fractures. Ample space has been given to the description and imaging of the course of diseases under treatment by up-to-date therapies, as e.g. for ostitis deformans Paget's disease, or skeletal metastases. This second edition of the book incorporates the progress achieved over the last five years in skeletal diagnostics. The advances in this field have been resulting from basic research work, for instance in molecular biology, or from a variety of completed studies relating to clinical medicine, laboratory chemistry, histopathology and radiology of skeletal diseases, and from experience obtained with the diagnostic radiology methods and techniques, with the potentials and constraints of magnetic resonance imaging (MRI) today being more critically assessed than five years ago. MRI is a modality currently meeting with interest in the context of search for additional diagnostic information, new definition of complete pictures of diseases, or false or overinterpretation of diagnostic findings. (orig./MG). 431 figs [de

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...

Psychotic Symptomatology in a Juvenile Court Clinic Population

Science.gov (United States)

Lewis, Dorothy Otnow; And Others

1973-01-01

This report indicating an unexpectedly high incidence of psychotic symptomatology in a population of cases referred to the Juvenile Court Psychiatric Clinic of the Second District of Connecticut, manifests the necessity for juvenile court systems to be made aware of the possibility of psychosis in our delinquent populations. (CS)

Disease course and long-term outcome of juvenile localized scleroderma: Experience from a single pediatric rheumatology Centre and literature review.

Science.gov (United States)

Martini, Giorgia; Fadanelli, Gloria; Agazzi, Anna; Vittadello, Fabio; Meneghel, Alessandra; Zulian, Francesco

2018-05-03

Juvenile Localized Scleroderma (JLS) is a rare disorder that may cause severe aesthetic sequelae and functional disability. To date, data on natural history and long-term outcome are discordant and difficult to compare due to the heterogeneity of clinical subtypes, treatments and methods to evaluate activity and outcome in previous studies. A retrospective and cross-sectional study including 133 patients followed between January 1991 and December 2016 was conducted at our Pediatric Rheumatology Centre. Disease course was drawn by retrospective analysis of patients' clinical features, treatment, disease course and outcome at the last evaluation. Disease activity and severity of tissue damage were assessed by using parameters derived from the Localized Scleroderma Cutaneous Assessment Tool (LoSCAT) and thermography. Most patients achieved complete remission, as only 12.5%, all with the linear subtype, had still active disease after over 10 years of follow-up. At least one disease relapse occurred in 22.2% of patients and first flare was observed 20 months after first treatment discontinuation. Mild tissue damage was observed in more than half of patients, in 25.4% was moderate and in 23.0% severe; 19.8% presented a functional limitation. The entity of skin and subcutaneous fat loss established at the early stages of the disease as 27.8% of patients with shorter disease duration had severe damage and the rates remained constant in patients with longer follow-up. The delay in start of systemic treatment was associated with longer disease activity and higher relapse rate. Patients with linear scleroderma (LS), pansclerotic morphea (PM) and mixed subtype (MS) presented more severe aesthetic and functional damage but did not differ from other subtypes as for rate of complete remission. JLS in some patients can be a very aggressive disease with persistent activity after >10 years and/or several disease relapses. As tissue damage establishes early in disease course a

Effects of Prior Experience on Shelter-Seeking Behavior of Juvenile American Lobsters.

Science.gov (United States)

Bayer, Skylar R; Bianchi, Katherine M; Atema, Jelle; Jacobs, Molly W

2017-04-01

Shelter-seeking behaviors are vital for survival for a range of juvenile benthic organisms. These behaviors may be innate or they may be affected by prior experience. After hatching, American lobsters Homarus americanus likely first come into contact with shelter during the late postlarval (decapodid) stage, known as stage IV. After the subsequent molt to the first juvenile stage (stage V), they are entirely benthic and are thought to be highly cryptic. We hypothesized that postlarval (stage IV) experience with shelter would carry over into the first juvenile stage (stage V) and reduce the time needed for juveniles to locate and enter shelters (sheltering). We found some evidence of a carryover effect, but not the one we predicted: stage V juveniles with postlarval shelter experience took significantly longer to initiate sheltering. We also hypothesized that stage V juveniles would demonstrate learning by relocating shelters more quickly with immediate prior experience. Our findings were mixed. In a maze, juveniles with immediate prior experience were faster to regain visual contact with shelter, suggesting that they had learned the location of the shelter. In contrast, there was no significant effect of immediate prior experience on time to initiate sheltering in an open arena, or in the maze after juveniles had regained visual contact. We conclude that very young (stage V) juvenile lobsters modify their shelter-seeking behavior based on prior experiences across several timescales. Ecologically relevant variation in habitat exposure among postlarval and early juvenile lobsters may influence successful recruitment in this culturally and commercially important fishery species.

Experimental Transmission of Frog Virus 3-Like Ranavirus in Juvenile Chelonians at Two Temperatures.

Science.gov (United States)

Allender, Matthew C; Barthel, Ashley C; Rayl, Jeremy M; Terio, Karen A

2018-06-07

The pathogenicity of frog virus 3 (FV3)-like ranavirus varies in adult chelonian species at different environmental temperatures, but differences in pathogenicity at different temperatures has yet to be determined in juveniles. Our objective was to determine the susceptibility to FV3-like ranavirus in four species of juvenile chelonians: red-eared sliders (RES; Trachemys scripta elegans), Mississippi map turtles ( Graptemys pseudogeographica kohnii), false map turtles (FMT; Graptemys pseudogeographica), and eastern river cooters ( Pseudemys concinna concinna) at two environmental temperatures. Two simultaneous trials ( n=8 treatment and n=4 controls of each species) were conducted in separate temperature-controlled rooms with animals maintained at 22 C or 27 C. All of the inoculated animals of each species at each temperature died, but no mortality was observed in control animals. Median survival times varied between 8 d and 11 d, based on species and temperature, with RES in the 27 C trial surviving the shortest time and the FMT in the 22 C trial surviving the longest. Combining all species, turtles in the 27 C trial survived for fewer days than those housed at 22 C, despite all turtles in both trials having similar viral copies detected in postmortem tissues. Lesions in inoculated turtles resembled those noted in natural and experimental FV3-like ranavirus infections and included vasculitis, thrombosis, hemorrhage in multiple organs, renal tubular necrosis, and hepatic necrosis. Myositis was not present in any juvenile, infected turtles in this study. This study confirmed that juvenile chelonians have a high susceptibility to ranaviral disease.

The Education of Juveniles in Detention: Policy Considerations and Infrastructure Development

Science.gov (United States)

Geib, Catherine Foley; Chapman, John F.; D'Amaddio, Amy H.; Grigorenko, Elena L.

2011-01-01

This article presents a discussion of the state of affairs pertaining to educating juvenile justice-involved youth. It summarizes general observations regarding the schooling of juveniles in pre-trial and post-trial incarceration settings, as well as, juveniles on probation or in community settings. The article selectively presents relevant…

Juvenile myelomonocytic leukemia presenting as bilateral breast masses

Energy Technology Data Exchange (ETDEWEB)

Edison, Michele N.; Letter, Haley P. [Florida Hospital, Department of Radiology, Orlando, FL (United States); University of Central Florida, College of Medicine, Orlando, FL (United States); O' Dell, M.C. [University of Central Florida, College of Medicine, Orlando, FL (United States); Children' s Hospital of Philadelphia, Pediatric Radiology, Philadelphia, PA (United States); Scherer, Kurt; Williams, Jennifer L. [Florida Hospital, Department of Radiology, Orlando, FL (United States); University of Central Florida, College of Medicine, Orlando, FL (United States); Florida State University, College of Medicine, Tallahassee, FL (United States)

2017-01-15

An 8-year-old girl presented with bilateral breast masses and was subsequently diagnosed with juvenile myelomonocytic leukemia. Juvenile myelomonocytic leukemia is a rare myelodysplastic syndrome that typically presents in boys younger than 3 years of age with splenomegaly, lymphadenopathy and skin findings. Bilateral breast masses in a child are rare and, as such, present a diagnostic dilemma due to the relative paucity of cases in the literature. We present a case of granulocytic sarcoma of the breasts in a patient with juvenile myelomonocytic leukemia. The authors hope that increased reporting and research regarding pediatric breast masses will help create awareness for such cases. (orig.)

Clinical research of juvenile hyperthyroidism treatment with radioiodine

International Nuclear Information System (INIS)

Qiu Ling; Zhang Chunying; Chen Yue

2001-01-01

Objective: To evaluate the effects and side effects in the radioiodine management of juvenile hyperthyroidism. Methods: 80 patients with poor effects using anti-thyroid drug were assigned to receive 131 I therapy. The follows of therapy outcome were assessed 1, 3, 6 and 12 months after the start of treatment. One follows up per 1-3 years. Results: Among 80 patients followed by 6 months, clinical response was excellent in 65 patients (81%), good in 15 (19%). There were 12 patients with hypothyroidism followed 4 years, and with no other side effects. Conclusion: The good therapeutic effect was obtained in radioiodine treatment for juvenile hyperthyroidism. Radioiodine was effective to juvenile hyperthyroidism

Juvenile myelomonocytic leukemia presenting as bilateral breast masses

International Nuclear Information System (INIS)

Edison, Michele N.; Letter, Haley P.; O'Dell, M.C.; Scherer, Kurt; Williams, Jennifer L.

2017-01-01

An 8-year-old girl presented with bilateral breast masses and was subsequently diagnosed with juvenile myelomonocytic leukemia. Juvenile myelomonocytic leukemia is a rare myelodysplastic syndrome that typically presents in boys younger than 3 years of age with splenomegaly, lymphadenopathy and skin findings. Bilateral breast masses in a child are rare and, as such, present a diagnostic dilemma due to the relative paucity of cases in the literature. We present a case of granulocytic sarcoma of the breasts in a patient with juvenile myelomonocytic leukemia. The authors hope that increased reporting and research regarding pediatric breast masses will help create awareness for such cases. (orig.)

Capilaroscopia periungueal em crianças e adolescentes com doenças reumáticas Nailfold capillaroscopy in children and adolescents with rheumatic diseases

Directory of Open Access Journals (Sweden)

Daniela Gerent Petry Piotto

2012-10-01

Full Text Available OBJETIVO: Avaliar a capilaroscopia periungueal de crianças e adolescentes com doenças reumáticas autoimunes (artrite idiopática juvenil, lúpus eritematoso sistêmico, dermatomiosite juvenil, esclerodermia e doença mista do tecido conjuntivo e relacioná-la com comprometimentos clínico e laboratorial e atividade de doença. MÉTODOS: Estudo transversal no qual foram avaliados 147 pacientes por meio de capilaroscopia periungueal: 60 com artrite idiopática juvenil, 30 com lúpus eritematoso sistêmico, 30 com dermatomiosite juvenil, 20 com esclerodermia localizada, quatro com esclerodermia sistêmica e três com doença mista do tecido conjuntivo. Exames clínico, laboratorial e de capilaroscopia periungueal foram realizados em todos os pacientes. A capilaroscopia periungueal foi realizada com microscópio óptico com aumentos de 10 e 16 vezes pelo mesmo observador. RESULTADOS: A maioria dos pacientes avaliados (76,2% apresentou capilaroscopia periungueal normal. As maiores alterações na capilaroscopia periungueal foram observadas nos pacientes com dermatomiosite juvenil, esclerodermia sistêmica e doença mista do tecido conjuntivo, e caracterizaram o padrão escleroderma. Não houve associação entre capilaroscopia periungueal e atividade de doença nos pacientes com artrite idiopática juvenil, lúpus eritematoso sistêmico e esclerodermia localizada. Houve associação entre atividade da doença e alterações capilaroscópicas nos pacientes com dermatomiosite juvenil. CONCLUSÃO: A capilaroscopia periungueal é um método útil para o diagnóstico das doenças reumáticas autoimunes e para o acompanhamento de atividade de doença.OBJECTIVE: To assess nailfold capillaroscopy in children and adolescents with autoimmune rheumatic diseases (juvenile idiopathic arthritis, systemic lupus erythematosus, juvenile dermatomyositis, scleroderma and mixed connective tissue disease and relate it to clinical and laboratory findings and disease

A social work study on family related issues influencing juvenile delinquency

Directory of Open Access Journals (Sweden)

Mohammad Reza Iravani

2013-07-01

Full Text Available We present a study to investigate the impacts of various factors on juvenile delinquency. The study distributes 400 questionnaires among young people aged 19-26 who are involved with some sort of crime and analyzes their feedbacks. There are three hypotheses including family income, tendency to spirituality and religion and educational backgrounds of families and juvenile delinquency among these people. The results show that while family income and religious as well as spirituality characteristics play important role on juvenile delinquency, educational background of the parents do not statistically have any influence on juvenile delinquency. The study recommends that a better family condition could contribute society to reduce juvenile delinquency and people could guide their children through better consultations.

Catch rate of juveniles Ethamatosa fimbriata , Sardinella maderensis ...

African Journals Online (AJOL)

We collected data on the quantity of juvenile fish and the daily duration of fishing trips in four landing sites over a two-week period Bernoulli random variables and properties of uniform distribution were used to analyze the data. Catch rates of juveniles Ethamatosa fimbriata, Sardinella maderensis, and Brachydeuterus ...

Extinguishing All Hope: Life-without-Parole for Juveniles

Science.gov (United States)

Butler, Frank

2010-01-01

Sentencing juveniles to life-without-parole (JLWOP) is a practice fraught with ethical dilemmas. Through in-depth interviews with 11 men living sentences of JLWOP, their narratives of their backgrounds and experiences as juveniles were studied. Common themes were identified, and 3 general categories of cases emerged from the narratives. Ethical…

Setting a minimum age for juvenile justice jurisdiction in California.

Science.gov (United States)

S Barnert, Elizabeth; S Abrams, Laura; Maxson, Cheryl; Gase, Lauren; Soung, Patricia; Carroll, Paul; Bath, Eraka

2017-03-13

Purpose Despite the existence of minimum age laws for juvenile justice jurisdiction in 18 US states, California has no explicit law that protects children (i.e. youth less than 12 years old) from being processed in the juvenile justice system. In the absence of a minimum age law, California lags behind other states and international practice and standards. The paper aims to discuss these issues. Design/methodology/approach In this policy brief, academics across the University of California campuses examine current evidence, theory, and policy related to the minimum age of juvenile justice jurisdiction. Findings Existing evidence suggests that children lack the cognitive maturity to comprehend or benefit from formal juvenile justice processing, and diverting children from the system altogether is likely to be more beneficial for the child and for public safety. Research limitations/implications Based on current evidence and theory, the authors argue that minimum age legislation that protects children from contact with the juvenile justice system and treats them as children in need of services and support, rather than as delinquents or criminals, is an important policy goal for California and for other national and international jurisdictions lacking a minimum age law. Originality/value California has no law specifying a minimum age for juvenile justice jurisdiction, meaning that young children of any age can be processed in the juvenile justice system. This policy brief provides a rationale for a minimum age law in California and other states and jurisdictions without one.

The myosin chaperone UNC45B is involved in lens development and autosomal dominant juvenile cataract

DEFF Research Database (Denmark)

Hansen, Lars; Comyn, Sophie; Mang, Yuan

2014-01-01

Genome-wide linkage analysis, followed by targeted deep sequencing, in a Danish multigeneration family with juvenile cataract revealed a region of chromosome 17 co-segregating with the disease trait. Affected individuals were heterozygous for two potentially protein-disrupting alleles in this reg...... advance online publication, 19 February 2014; doi:10.1038/ejhg.2014.21....

The Juvenile Transition: A Developmental Switch Point in Human Life History

Science.gov (United States)

Del Giudice, Marco; Angeleri, Romina; Manera, Valeria

2009-01-01

This paper presents a new perspective on the transition from early to middle childhood (i.e., human juvenility), investigated in an integrative evolutionary framework. Juvenility is a crucial life history stage, when social learning and interaction with peers become central developmental functions; here it is argued that the "juvenile transition"…

Nocardia brasiliensis infection mimicking juvenile idiopathic arthritis in a 4-year-old girl.

Science.gov (United States)

Kapur, Nitin; Adib, Navid; Grimwood, Keith

2013-11-01

Nocardia are ubiquitous environmental saprophytes that cause pneumonia and disseminated disease in immunocompromised patients. They can also cause localized cutaneous and soft tissue infections in healthy people after direct percutaneous inoculation. Nocardia arthritis is rare in both forms of the disease. Here we present the first published case of a child with septic arthritis caused by N brasiliensis. Importantly, this otherwise well 4-year-old girl had no known history of trauma but presented with transient cutaneous lesions and a 6-week history of arthritis involving the right fourth digit proximal interphalangeal joint without accompanying fever or raised systemic inflammatory markers. She received a diagnosis of juvenile idiopathic arthritis and underwent antiinflammatory and immunosuppressant therapy. After 2 months she developed frank septic arthritis, which necessitated a surgical joint washout, from which an intraoperative swab grew N brasiliensis. The patient received 6 months of high-dose trimethoprim-sulfamethoxazole and remains well more than 4 years after treatment. This unusual case highlights the importance of considering an indolent infection from slow-growing organisms, including Nocardia, when diagnosing the oligoarthritis subtype of juvenile idiopathic arthritis. This is especially relevant when a single joint is involved and response to antiinflammatory therapy is suboptimal because antiinflammatory agents may mask evolving signs of infection.

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.

75 FR 39025 - Determination That ACTONEL (Risendronate Sodium) Tablets, 75 Milligrams, and ACTONEL WITH CALCIUM...

Science.gov (United States)

2010-07-07

... Patent Term Restoration Act of 1984 (Public Law 98-417) (the 1984 amendments), which authorized the... Paget's disease in men and women. In a separate citizen petition dated January 20, 2010 (FDA-2010-P...

Sexual dimorphism and plumage characteristics of juvenile Cape ...

African Journals Online (AJOL)

Juveniles, or birds in their first year, were genetically sexed. Standardised photographs were taken of plucked juvenile breast feathers and analysed using Adobe Photoshop. Pixel counts were taken to analyse the streak coverage of a single feather. A scale from 0 to 3 was used to score streak intensity of the entire breast.

Pattern of juvenile periodontitis in Lagos University Teaching ...

African Journals Online (AJOL)

Objective: The aim of the survey was to study the pattern of juvenile periodontitis patients that presented at the Lagos University Teaching Hospital Dental Centre from November 1999 to March 2004. Methods: Through review of case files of patients, twenty six juvenile periodontitis patients, within the age range of 18 and 30 ...

Giant Bilateral Juvenile Fibroadenoma of the Breast in Prepubescent Girl.

Science.gov (United States)

Khan, Salma; Khan, Momna; Rafique, Sadia

2015-10-01

Juvenile fibroadenoma accounts for 4% of the total fibroadenomas. Giant juvenile fibroadenoma is found in only 0.5% of all fibroadenomas. The authors report a 10-year girl presenting with progressive enlargement of both breasts for one year. Based on clinical findings and Fine Needle Aspiration Cytology (FNAC), a diagnosis of bilateral giant juvenile fibroadenomas of breast was made. She underwent bilateral lumpectomy with breast conservation and made uneventful postoperative recovery.

Reassessing the Anatomic Origin of the Juvenile Nasopharyngeal Angiofibroma.

Science.gov (United States)

McKnight, Colin D; Parmar, Hemant A; Watcharotone, Kuanwong; Mukherji, Suresh K

A modern imaging review is necessary to further define the anatomic origin of the juvenile nasopharyngeal angiofibroma. After institutional review board approval, a search from January 1998 to January 2013 yielded 33 male patients (aged 10-23 years) with pathologically proven juvenile nasopharyngeal angiofibroma lesions, as well as pretreatment computed tomography/magnetic resonance imaging. Juvenile nasopharyngeal angiofibroma involvement was assessed in the following regions: sphenopalatine foramen, pterygopalatine fossa, vidian canal, nasopharynx, nasal cavity, sphenoid sinus, choana, pterygomaxillary fissure/masticator space, orbit, and sphenoid bone. The choana and nasopharynx were involved in all 33 patients. In contrast, only 22 lesions involved the pterygopalatine fossa, 24 lesions involved the sphenopalatine foramen, and 28 lesions involved the vidian canal. Our results suggest that the juvenile nasopharyngeal angiofibroma origin is in the region of the choana and nasopharynx rather than the sphenopalatine foramen or pterygopalatine fossa.

Coronary artery abnormalities in children with systemic-onset juvenile idiopathic arthritis.

Science.gov (United States)

Lefèvre-Utile, Alain; Galeotti, Caroline; Koné-Paut, Isabelle

2014-05-01

Still's disease (Systemic-onset Juvenile Idiopathic Arthritis: SoJIA) is characterised by high-spiking daily fevers, arthritis and evanescent rashes. Diagnosis of Still's disease is often challenging. Infectious diseases and other inflammatory conditions, especially in young children, Kawasaki disease may look similar. Clinicians often rely on echocardiographic evidence of coronary artery abnormalities to differentiate between Kawasaki disease and Still's disease. Coronary artery dilation would typically favour the diagnosis of Kawasaki disease. We present four children with Still's disease and coronary artery abnormalities who were initially misdiagnosed as Kawasaki disease. The first patient had pericarditis and an irregular wall of the left coronary artery, without dilation on echocardiography. The second patient had a left coronary artery dilatation and a pericarditis. The third patient had thickened left coronary artery walls, and the fourth patient had a hyperechogenicity of the left and right coronary arteries. They received IVIG without success. The diagnosis of Still's disease was made secondary with evidence of persistent arthritis. All but one patient finally needed biologic treatments. Coronary abnormalities may be observed during various febrile conditions and do not exclude the diagnosis of Still's disease. Copyright © 2013 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

Crocodylus acutus (American Crocodile). Long distance juvenile movement

Science.gov (United States)

Crespo, Rafael; Beauchamp, Jeffrey S.; Mazzotti, Frank; Cherkiss, Michael S.

2015-01-01

Crocodylus acutus (American Crocodile) is the most widely distributed New World crocodilian species with its range extending from Peru in the south to the southern tip of peninsular Florida in the north. Crocodylus acutus occupies primarily coastal brackish water habitat, however it also occurs in freshwater to hypersaline habitats (Thorbjarnarson 2010. In Crocodiles. Status Survey and Conservation Action Plan. [Third Edition], American Crocodile Crocodylus acutus, pp. 46–53 S.C. Manolis and C. Stevenson. Crocodile Specialist Group, Darwin). There is limited literature on long distance movements of juvenile crocodilians worldwide and no literature on juvenile crocodiles in Florida. However, adult C. acutus in Florida have been documented to make seasonal movements of 5–15 km from preferred foraging habitat to nesting beaches (Mazzotti 1983. The Ecology of Crocodylus acutus in Florida. PhD Dissertation. The Pennsylvania State University, University Park, Pennsylvania. 161pp), and one adult was documented making a 35 km trip from her nest site to preferred foraging habitat (Cherkiss et. al. 2006. Herpetol. Rev. 38:72–73). Rodda (1984. Herpetologica 40:444–451) reported on juvenile C. acutus movement in Gatun Lake, Panama, and found that juveniles stayed within 1 km of their nest site for the first month. Movements of juvenile Crocodylus porosus (Saltwater Crocodile) in a river system in Northern Australia showed a maximum movement of 38.9 km from a known nest site, with the majority of the crocodiles staying within 15.6 km downstream to 6.8 km upstream (Webb and Messel 1978. Aust. Wildlife Res. 5:263–283). Juvenile movement of Crocodylus niloticus (Nile Crocodile) in Lake Ngezi, Zimbabwe showed crocodiles restricted their movements from 1.0 km up to 4.5 km through the wet and dry seasons (Hutton 1989. Am. Zool. 29:1033–1049). Long distance movements of alligators were recorded for sizes ranging from 28 cm to 361 cm in a coastal refuge in Louisiana, where

Musculoskeletal MRI findings of juvenile localized scleroderma

Energy Technology Data Exchange (ETDEWEB)

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.)

Musculoskeletal MRI findings of juvenile localized scleroderma

International Nuclear Information System (INIS)

Eutsler, Eric P.; Horton, Daniel B.; Epelman, Monica; Finkel, Terri; Averill, Lauren W.

2017-01-01

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.)

Musculoskeletal MRI findings of juvenile localized scleroderma.

Science.gov (United States)

Eutsler, Eric P; Horton, Daniel B; Epelman, Monica; Finkel, Terri; Averill, Lauren W

2017-04-01

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.

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).

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

Science.gov (United States)

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

2014-05-24

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

Change in Family Structure and Rates of Violent Juvenile Delinquency

OpenAIRE

Fry, Jeannie A

2010-01-01

This paper addresses the question: Have the changes in family structure in the U.S. become a catalyst for juvenile delinquency? For this research, I use existing statistics for my three independent variables: divorce rates, rate of working mothers with children under age 18, percent female-headed households. My dependent variable, juvenile violent crime rates, is measured using data from the Office of Juvenile Justice and Delinquency Prevention. My control variables consist of the followin...

Penicillium oxalicum reduces the number of cysts and juveniles of potato cyst nematodes.

Science.gov (United States)

Martinez-Beringola, M L; Salto, T; Vázquez, G; Larena, I; Melgarejo, P; De Cal, A

2013-07-01

To test the biocontrol potential of Penicillium oxalicum, a biocontrol agent against fungal diseases and against the potato cyst nematodes (PCNs), Globodera pallida and Globodera rostochiensis. We tested the effect of P. oxalicum on the nematode cysts under laboratory conditions or in soil microcosms. A reduction in the rate of G. pallida juveniles hatching by P. oxalicum was observed when root diffusates from the 'Monalisa' and the 'Désirée' potato cultivar were used (98·6 and 74·1% reduction, respectively). However, the rate of G. pallida juveniles hatching was not significantly reduced when root diffusates from the 'San Pedro' tomato cultivar were used. Penicillium oxalicum also significantly reduced the ability of the G. rostochiensis juveniles to hatch (30·9% reduction) when root diffusates of the 'Désirée' potato cultivars were used. Penicillium oxalicum treatment of the soil significantly reduced the number of G. pallida cysts that were recovered from the soil of each pot that contained the 'Désirée' potato cultivar. Our results show that P. oxalicum is a potential biocontrol inoculant for protecting potato crops against PCNs. Penicillium oxalicum has potential to be used in order to reduce PCNs. Journal of Applied Microbiology © 2013 The Society for Applied Microbiology.

Setting a minimum age for juvenile justice jurisdiction in California

Science.gov (United States)

Barnert, Elizabeth S.; Abrams, Laura S.; Maxson, Cheryl; Gase, Lauren; Soung, Patricia; Carroll, Paul; Bath, Eraka

2018-01-01

Purpose Despite the existence of minimum age laws for juvenile justice jurisdiction in 18 US states, California has no explicit law that protects children (i.e. youth less than 12 years old) from being processed in the juvenile justice system. In the absence of a minimum age law, California lags behind other states and international practice and standards. The paper aims to discuss these issues. Design/methodology/approach In this policy brief, academics across the University of California campuses examine current evidence, theory, and policy related to the minimum age of juvenile justice jurisdiction. Findings Existing evidence suggests that children lack the cognitive maturity to comprehend or benefit from formal juvenile justice processing, and diverting children from the system altogether is likely to be more beneficial for the child and for public safety. Research limitations/implications Based on current evidence and theory, the authors argue that minimum age legislation that protects children from contact with the juvenile justice system and treats them as children in need of services and support, rather than as delinquents or criminals, is an important policy goal for California and for other national and international jurisdictions lacking a minimum age law. Originality/value California has no law specifying a minimum age for juvenile justice jurisdiction, meaning that young children of any age can be processed in the juvenile justice system. This policy brief provides a rationale for a minimum age law in California and other states and jurisdictions without one. Paper type Conceptual paper PMID:28299968

Juvenile Myasthenia Gravis in Korea: Subgroup Analysis According to Sex and Onset Age.

Science.gov (United States)

Lee, Ha Neul; Kang, Hoon-Chul; Lee, Joon Soo; Kim, Heung Dong; Shin, Ha Young; Kim, Seung Min; Sunwoo, Il Nam; Lee, Young-Mock

2016-12-01

Juvenile myasthenia gravis presents before 18 years of age with different characteristics according to racial background and pubertal development. The authors aimed to determine the clinical characteristics of children and adolescents of Korean ethnicity with myasthenia gravis, and evaluate the presentation and clinical outcomes according to the sex and onset age of the patients. The authors recruited 88 Korean juvenile myasthenia gravis patients between September 2005 and August 2015. Worse clinical severity from presentation, more aggressive treatment strategies, and worse final treatment outcomes were noted in girls with postpubertal onset than in the other patients. The symptoms were milder (pure ocular presentation in 96.6% [85/88]) and the disease course was more benign (94.3% [83/88]) in this study than in the literature. The homogenous racial background might have contributed to these results. These findings highlight the influence of pubertal development and the need for timely and appropriate active treatment, including thymectomy, to improve prognosis. © The Author(s) 2016.

Marine nurseries and effective juvenile habitats: concepts and applications.

NARCIS (Netherlands)

Dahlgren, C.P.; Kellison, G.T.; Adams, A.J.; Gillanders, B.M.; Kendall, M.S.; Layman, C.A.; Ley, J.A.; Nagelkerken, I.; Serafy, J.E.

2006-01-01

Much recent attention has been focused on juvenile fish and invertebrate habitat use, particularly defining and identifying marine nurseries. The most significant advancement in this area has been the development of a standardized framework for assessing the relative importance of juvenile habitats

Sialendoscopic Approach in Management of Juvenile Recurrent Parotitis.

Science.gov (United States)

Singh, P P; Goyal, M; Goyal, A

2017-12-01

To assess the role of sialendoscopy as a diagnostic and therapeutic modality in juvenile recurrent parotitis. Juvenile recurrent parotitis (JRP) is the second most frequent salivary gland disease in childhood and is characterized by recurrent non suppurative and non obstructive parotid inflammation. These attacks influence the quality of life and can even lead to gland destruction, and there are no definitive treatment to avoid them. Sialendoscopic dilatation is emerging as the new treatment modality in this aspect.  retrospective study. Department of Otorhinolaryngology in tertiary care hospital. 17 cases of juvenile recurrent parotitis (i.e. children of age group 3-11 years presenting with complaints of recurrent parotid region swelling and pain, sometimes associated with fever) were included in the study during October 2012-September 2015. All cases underwent sialendoscopy under general anaesthesia. Diagnostic (classifying the ductal lesion) and interventional sialendoscopic procedure (dilatation with instillation of steroid) were carried out in single sitting. Follow up was done for a minimum of 6 months (range 6-36 months). 17 patients with mean age of 5.6 years and gender distribution of 47:53 (boys:girls) underwent sialendoscopy for JRP. 8 patients presented with unilateral parotitis and 9 with bilateral. The mean number of attacks in previous 1 year were 9.2. Average time for procedure was 20 min. All cases had ductal stenosis and ductal mucosa was pale in 15 cases on endoscopy. 1 patient underwent repeat endoscopy after 2 years. 50% had complete resolution of symptoms and 6 patients had one mild (swelling not associated with fever which subsided on its own) attack after treatment. Follow up period ranged from 6 months to 3 years. No complications were observed. Sialendoscopy has emerged as a viable option for assessment and treatment of JRP. Dilatation of the parotid duct and steroid instillation has significantly reduced the morbidity of this

75 FR 70293 - Meeting of the Federal Advisory Committee on Juvenile Justice

Science.gov (United States)

2010-11-17

... DEPARTMENT OF JUSTICE Office of Justice Programs [OJP (OJJDP) Docket No. 1532] Meeting of the Federal Advisory Committee on Juvenile Justice AGENCY: Office of Juvenile Justice and Delinquency Prevention, Office of Justice Programs, Justice. ACTION: Notice of Meeting. SUMMARY: The Office of Juvenile...

Juvenile delinquency in Russia: Ccriminal justice, trends, key issues

Directory of Open Access Journals (Sweden)

Kovačević Milica

2015-01-01

Full Text Available In the literature, as well as in international statistical surveys, we can rarely find more detailed information on juvenile delinquency in the Russian Federation, as well as on the criminal reaction towards juvenile offenders. Due to the turbulent conditions and great social turmoil which took place in the last few decades in this country, there should be a greater interest in the problem of juvenile delinquency. For Serbia, the experience of the Russian Federation could be especially important if one bears in mind that our country is still going through a transition, population stratification and through economic crisis, and also that Serbia and the Russian Federation share some cultural and religious similarities. Therefore, the aim of this paper is to present, in the summary way, the basic features of juvenile delinquency and the criminal justice system, and thereby build a basis for future research and comparison.

Aerobic training in persons who have recovered from juvenile dermatomyositis

DEFF Research Database (Denmark)

Riisager, M; Mathiesen, P R; Vissing, J

2013-01-01

A recent study has shown that 36 persons who had recovered from juvenile dermatomyositis (JDM) have on average an 18% decrease in maximal oxygen uptake. The objective of this study was to investigate the effect of a 12-week aerobic training program in this group, and assess whether aerobic training...... can normalize aerobic capacity to the expected level for age and gender. The patients participating in the study, one male and nine females (16-42years of age), were in remission from JDM, defined as no clinical or biochemical evidence of disease activity and no medical treatment for 1year...

Radioangiography in diagnosis of juvenile angiofibroma

International Nuclear Information System (INIS)

Kalantarov, K.D.; Anyutin, R.G.; Ashikhmina, I.G.

1979-01-01

To study the blood supply of juvenile angiofibroma of the nasopharynx 10 patients were examined (2 of them were examined twice). Four patients with the deseases of the nose and the nasopharynx of a nonvascular nature were in the control group. The authors used sup(99m)Tc for radioangiography. The investigations were made on the gamma-chamber ''Nuclear-Chicago'' connected to the computer ''Cina-200''. Ten patients with juvenile angiofibroma of the nasopharynx showed rapid accumulation of the radioactive substance at the site of the tumour exceeding its concentration in the carotic arteries by 4-10 times. In unilateral localization of angiofibroma, its blood supply was provided mainly through the external carotic artery at the affection site or evenly through the both external carotic arteries. The data on dynamic distribution of the radioactive substance, scintiphotograms and clinical observations allow a distinct differentiation between juvenile angiofibroma of the nasopharynx the adenoid tissue or any other tumour to determine the angiofibroma size as well as to follow the results of surgical and radiation treatment

Effects of Astragalus polysaccharides (APS) and chitooligosaccharides (COS) on growth, immune response and disease resistance of juvenile largemouth bass, Micropterus salmoides.

Science.gov (United States)

Lin, Shi-Mei; Jiang, Yu; Chen, Yong-Jun; Luo, Li; Doolgindachbaporn, Sompong; Yuangsoi, Bundit

2017-11-01

The effects of oral administration of Astragalus polysaccharides (APS) and chitooligosaccharides (COS), single or combined, on the growth performance, immunity and disease resistance of M. salmoides were investigated. Largemouth bass juvenile were divided into 4 groups and each group was fed with diets supplemented with or without immunostimulant for 8 weeks. After 8 weeks of feeding trial, five fish per tank were sampled for immunity determination, ten fish per tank were challenged by A. hydrophila. The results showed that the largemouth bass fed with two immunostimulants alone or in combination significantly enhanced the final weight and specific growth rate (SGR), decreased feed conversion ratio (FCR) (P APS. In addition, both COS and APS upregulated respiratory burst activity (RBA), phagocytic activity (PA), lysozyme activity and superoxide dismutase (SOD) activity. Meanwhile, COS also exhibited a increase in total leukocyte count, nitric oxide (NO) content and inducible nitric oxide synthase (iNOS) activity compared to the control. When challenged with A. hydrophila, the mortality of groups fed with COS and/or APS was lower than the control (P APS and COS had a synergistic effect on lysozme activity, iNOS activity, NO content and disease resistance of fish (P < 0.05). Copyright © 2017 Elsevier Ltd. All rights reserved.

From juvenile hyperuricaemia to dysfunctional uromodulin: an ongoing metamorphosis.

Science.gov (United States)

Venkat-Raman, Gopalakrishnan; Gast, Christine; Marinaki, Anthony; Fairbanks, Lynnette

2016-11-01

Familial juvenile hyperuricaemic nephropathy (FJHN) is a diagnosis that is easily missed. It has taken a long time to clarify the pathophysiology and prevalence of this disease entity which has been shown to be genetically identical to medullary cystic kidney disease (MCKD) type II. The initial suspicion that uric acid was the noxious agent has been replaced by the recognition that a mutant uromodulin (UMOD) is the real culprit-although the exact mechanisms of pathogenicity remain uncertain. The mutation has been traced to the UMOD gene in chromosome 16. The disease is characterised by the classic triad of autosomal dominant inheritance, progressive renal failure beginning in the third to fifth decade of life and gout. Phenotypically similar but genotypically distinct entities have been described over the last 10 years, making a clinical diagnosis difficult. These include mutations in the renin, hepatocyte nuclear factor 1-β and mucin 1 genes. UMOD-associated kidney disease has been proposed as a logical diagnostic label to replace FJHN, but given all these other mutations, an over-arching diagnostic term of 'autosomal dominant tubulointerstitial kidney disease' (ADTKD) has been recently adopted. Allopurinol has been suggested as a therapeutic agent, but unfortunately this was based on non-randomised uncontrolled trials with small patient numbers.

Genetics Home Reference: Sandhoff disease

Science.gov (United States)

... Cooper A, Ferrie CD. Juvenile Sandhoff disease--nine new cases and a review of the literature. J Inherit Metab Dis. 2004;27(2):241-9. Review. Citation on PubMed Tay SK, Low PS, Ong HT, Loke KY. Sandhoff disease--a case report of 3 siblings and a review of potential therapies. Ann Acad ...

Social skills training for juvenile delinquents : post-treatment changes

NARCIS (Netherlands)

van der Stouwe, Trudy; Asscher, Jessica J.; Hoeve, Machteld; van der Laan, Peter H.; Stams, Geert Jan J M

2016-01-01

Objectives: To examine the post-treatment effectiveness of an outpatient, individual social skills training for juvenile delinquents in the Netherlands and to conduct moderator tests for age, gender, ethnicity, and risk of reoffending. Methods: The sample consisted of juveniles who received Tools4U,

Social Skills Training for Juvenile Delinquents: Post-Treatment Changes

NARCIS (Netherlands)

van der Stouwe, Trudy; Asscher, J.J.; Stams, G.J.J.M.; Hoeve, M.; van der Laan, Peter H.

2016-01-01

Objectives: To examine the post-treatment effectiveness of an outpatient, individual social skills training for juvenile delinquents in the Netherlands and to conduct moderator tests for age, gender, ethnicity, and risk of reoffending. Methods: The sample consisted of juveniles who received Tools4U,

Social skills training for juvenile delinquents : Post-treatment changes

NARCIS (Netherlands)

van der Stouwe, T.; Asscher, J.J.; Hoeve, M.; van der Laan, P.H.; Stams, G.J.J.M.

2016-01-01

Objectives To examine the post-treatment effectiveness of an outpatient, individual social skills training for juvenile delinquents in the Netherlands and to conduct moderator tests for age, gender, ethnicity, and risk of reoffending. Methods The sample consisted of juveniles who received Tools4U, a

Male Breast Cancer

Science.gov (United States)

... types of breast cancer that can occur in men include Paget's disease of the nipple and inflammatory breast cancer. Inherited genes that increase breast cancer risk Some men inherit abnormal (mutated) genes from their parents that ...

Tracking from the tropics reveals behaviour of juvenile songbirds on their first spring migration.

Directory of Open Access Journals (Sweden)

Emily A McKinnon

Full Text Available Juvenile songbirds on spring migration travel from tropical wintering sites to temperate breeding destinations thousands of kilometres away with no prior experience to guide them. We provide a first glimpse at the migration timing, routes, and stopover behaviour of juvenile wood thrushes (Hylocichla mustelina on their inaugural spring migration by using miniaturized archival geolocators to track them from Central America to the U.S. and Canada. We found significant differences between the timing of juvenile migration and that of more experienced adults: juveniles not only departed later from tropical wintering sites relative to adults, they also became progressively later as they moved northward. The increasing delay was driven by more frequent short stops by juveniles along their migration route, particularly in the U.S. as they got closer to breeding sites. Surprisingly, juveniles were just as likely as adults to cross the Gulf of Mexico, an open-water crossing of 800-1000 km, and migration route at the Gulf was not significantly different for juveniles relative to adults. To determine if the later departure of juveniles was related to poor body condition in winter relative to adults, we examined percent lean body mass, fat scores, and pectoral muscle scores of juvenile versus adult birds at a wintering site in Belize. We found no age-related differences in body condition. Later migration timing of juveniles relative to adults could be an adaptive strategy (as opposed to condition-dependent to avoid the high costs of fast migration and competition for breeding territories with experienced and larger adults. We did find significant differences in wing size between adults and juveniles, which could contribute to lower flight efficiency of juveniles and thus slower overall migration speed. We provide the first step toward understanding the "black box" of juvenile songbird migration by documenting their migration timing and en route performance.

Martial arts participation and externalizing behavior in juveniles : A meta-analytic review

NARCIS (Netherlands)

Gubbels, J.; van der Stouwe, T.; Spruit, A.; Stams, G.J.J.M.

2016-01-01

Martial arts are very popular among juveniles all over the world, but the relation between martial arts and externalizing behavior in juveniles remains unclear. The current multilevel meta-analysis of 12 studies, including 94 effect sizes and N = 5949 juveniles, was conducted to examine the relation

Juvenile nasopharyngeal angiofibroma staging: An overview.

Science.gov (United States)

Alshaikh, Nada Ali; Eleftheriadou, Anna

2015-06-01

Staging of tumors is very important in treatment and surgical decision making, as well as in predicting disease recurrence and prognosis. This review focuses on the different available classifications of juvenile nasopharyngeal angiofibroma (JNA) and their impact on the evaluation, management, and prognosis of JNA. The literature was reviewed, and publications on JNA staging were examined. Our MEDLINE search of the entire English-language literature found no review article on the current available staging systems for JNA. In this article, we review the common JNA classification systems that have been published, and we discuss some of their advantages and disadvantages. The most commonly used staging systems for JNA are the Radkowski and the Andrews-Fisch staging systems. However, some newer staging systems that are based on advances in technology and surgical approaches-the Onerci, INCan, and UPMC systems-have shown promising utility, and they will probably gain popularity in the future.

CORRELATION BETWEEN FAMILY COMMUNICATION PATTERNS AND JUVENILE DELINQUENCY IN JUNIOR HIGH SCHOOL

Directory of Open Access Journals (Sweden)

Zurriyatun Thoyibah

2017-08-01

Full Text Available Background: Adolescents who are in transition period have high risk behavior of juvenile delinquency. Communication between parents and adolescents effectively and openly could help adolescents to avoid delinquency behavior. Objective: This study aims to examine the relationship between family communication patterns and juvenile delinquency in Junior High School. Methods: This research employed a cross-sectional design with correlation description approach. There were 243 students selected using simple random sampling from the 7th and 8th grade students of Junior High School. A questionnaire of juvenile delinquency and family communication pattern were used in this study. Data were analyzed using Chi Square test. Result: The research showed that the majority juvenile delinquency category was low (65% and the majority of communication pattern was in functional category (73.3%. There was a significant relationship between family communication pattern and juvenile delinquency (p<0.05. Conclusion: Communication pattern within family have significant association with juvenile delinquency.