Cognitive, emotional and psychosocial functioning of girls treated with pharmacological puberty blockage for idiopathic central precocious puberty

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Slawomir Wojniusz

2016-07-01

Full Text Available Central precocious puberty (CPP develops due to premature activation of the hypothalamic-pituitary-gonadal (HPG axis, resulting in early pubertal changes and rapid bone maturation. CPP is associated with lower adult height and increased risk for development of psychological problems. Standard treatment of CPP is based on postponement of pubertal development by blockade of the HPG axis with gonadotropin releasing hormone analogs (GnRHa leading to abolition of gonadal sex hormones synthesis. Whereas the hormonal and auxological effects of GnRHa are well researched, there is a lack of knowledge whether GnRHa treatment influences psychological functioning of treated children, despite the fact that prevention of psychological problems is used as one of the main reasons for treatment initiation. In the present study we seek to address this issue by exploring differences in cognitive function, behavior, emotional reactivity, and psychosocial problems between GnRHa treated CPP girls and age-matched controls.Fifteen girls with idiopathic CPP; median age 10.4 years, treated with slow-release GnRHa (triptorelin acetate – Decapeptyl SR ® 11.25 and 15 age-matched controls, were assessed with a comprehensive test battery consisting of paper and pencil tests, computerized tasks, behavioral paradigms, heart rate variability, and questionnaires filled in by the children’s parents. Both groups showed very similar scores with regard to cognitive performance, behavioral and psychosocial problems. Compared to controls, treated girls displayed significantly higher emotional reactivity (p = 0.016; Cohen’s d = 1.04 on one of the two emotional reactivity task conditions. Unexpectedly, the CPP group showed significantly lower resting heart rates than the controls (p = 0.004; Cohen’s d = 1.03; lower heart rate was associated with longer treatment duration (r = - 0.582, p = 0.037. The results suggest that GnRHa treated CPP girls do not differ in their cognitive or

Precocious Puberty (For Parents)

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... spurt" start of menstruation (her period) acne "mature" body odor In boys, the signs of precocious puberty before ... growth — a growth "spurt" voice deepening acne "mature" body odor Many kids who show some of the early ...

MRI study of pituitary in girls with central precocious puberty

International Nuclear Information System (INIS)

Ye Zhiqiu; Guo Qinglu; Feng Changzheng; Wei Beiyang; Liu Yongxi; Zhang Yan

2008-01-01

Objective: To study the shape, size and signal intensity of pituitary gland in girls aged 3-10 year old with central precocious puberty. Methods: MRI data of pituitary glands in 40 girls aged 3-10 years old with central precocious puberty were selected. The shape, height and the appearances of pituitary glands were measured and observed on sagittal T 1 WI. Results: Quantitative data about size, shape and single intensity changes of pituitary glands in central precocious puberty were obtained in two groups, including girls aged from 3-5 and 5-10. The convex pituitary gland were 85.0% in former group. The height of pituitary gland were 6.1±0.2mm (former group) and 6.4± 0.4mm (latter one) respectively. The width of pituitary stalk was 1.93±0.50mm. The posterior pituitary gland demonstrated high signal intensity in all cases. Conclusion: Obvious changes of the size and shape of pituitary glands were found in central precocious puberty of girls aged from 3-10. The pituitary glands manifested physiologic hypertrophy with more convex in central precocious puberty girls than in normal ones. The changes on MRI could reflect the function of hypothalamus-pituitary-gonadal axis. It is of important value and significance in the diagnosis of central precocious puberty. (authors)

PRECOCIOUS PUBERTY DUE TO OVARIAN CYST – CASE REPORT

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Teresa M Guimarães

2017-01-01

Discussion/Conclusion: Most autonomous ovarian cysts regress spontaneously with regression of pubertal signs, as in the present case. Therapy with a GnRH agonist may become necessary in the case of transformation from precocious pseudopuberty to central precocious puberty after recurrences of the ovarian cysts or when there is significant loss of height potential.

Four generations in a family with neurofibromatosis 1: precocious puberty and optic nerve tumor (OPT).

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Gucev, Z; Krstevska-Konstantinova, M; Tasic, V; Jancevska, A; Kirovski, I; Pop-Jordanova, N

2010-01-01

Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder with varied clinical manifestations. The proband is a 6-year-old boy with signs of precocious puberty. His penis was 10 cm, testicles 8 ml, pubic hair P2-3, and the genital skin was hyperpigmented. Multiple cafe au lait spots well above 5 mm were noticeable on his skin, as well as hard subcutaneous nodules, mostly on his trunk. His intelligence and hearing are normal. He has no history of seizures. Laboratory analysis showed: LH LH 1.4 mIU/ml, FSH 6.2 mIU/ml, testosterone 183 ng/ml. Bone age was 9 years. LHRH stimulation was characteristic of true precocious puberty (LH 9.8 mIU/ml and FSH 8.9 mIU/ml after 30 minutes). The MRI of the brain showed a tumor of the suprasellar region with compression of the pituitary stalk. At present the boy is 6 years old and has been treated with triptoreline acetate for 3 months. The volume of the testicles has decreased to 7 ml and a slight loss of pubic hair was noted. In addition, his mother and his grandfather exhibited dermal masses, and focal cutaneous and subcutaneous growths. The great-grand father had had the same cutaneous changes and died at the age of 75 from unrelated causes. It has already been well documented that NF is associated with an increased risk of malignancy and precocious puberty. Hence, we emphasize the need for a close and regular clinical follow-up of the OPT, puberty and patterns of growth.

Central precocious puberty: evaluation by neuroimaging

International Nuclear Information System (INIS)

Kornreich, L.; Horev, G.; Blaser, S.; Daneman, D.; Kauli, R.; Grunebaum, M.

1995-01-01

To evaluate the incidence of abnormal intracranial findings in children with central precocious puberty, 62 children (51 girls, 11 boys) were examined by computerized tomography and/or magnetic resonance imaging (MRI) of the brain. Forty-four had normal examinations; 18 (11 girls, 7 boys) showed intracranial pathologies, including hamartoma of the tuber cinereum (8 cases), parenchymal loss (3 cases), hypothalamicchiasmatic lesions (2 cases), lesions of the corpus callosum (2 cases), suprasellar cyst (1 case), and pineal cyst and mesiotemporal sclerosis (1 case each). Based on the correlation between the clinical and the imaging results of this series, the authors recommend MRI as the imaging method of choice in the investigation of precocious puberty. (orig.)

Central precocious puberty: evaluation by neuroimaging

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Kornreich, L. [Dept. of Pediatric Imaging, Children`s Medical Center of Israel, Petah Tiqva (Israel)]|[Sackler Faculty of Medicine, Tel Aviv Univ. (Israel); Horev, G. [Dept. of Pediatric Imaging, Children`s Medical Center of Israel, Petah Tiqva (Israel)]|[Sackler Faculty of Medicine, Tel Aviv Univ. (Israel); Blaser, S. [Dept. of Radiology, Hospital for Sick Children, Toronto, ON (Canada); Daneman, D. [Div. of Endocrinology, Dept. of Pediatrics, Hospital for Sick Children, Toronto, ON (Canada); Kauli, R. [Sackler Faculty of Medicine, Tel Aviv Univ. (Israel)]|[Inst. of Pediatric and Adolescent Endocrinology, Children`s Medical Center of Israel, Petah Tiqva (Israel); Grunebaum, M. [Dept. of Pediatric Imaging, Children`s Medical Center of Israel, Petah Tiqva (Israel)]|[Sackler Faculty of Medicine, Tel Aviv Univ. (Israel)

1995-02-01

To evaluate the incidence of abnormal intracranial findings in children with central precocious puberty, 62 children (51 girls, 11 boys) were examined by computerized tomography and/or magnetic resonance imaging (MRI) of the brain. Forty-four had normal examinations; 18 (11 girls, 7 boys) showed intracranial pathologies, including hamartoma of the tuber cinereum (8 cases), parenchymal loss (3 cases), hypothalamicchiasmatic lesions (2 cases), lesions of the corpus callosum (2 cases), suprasellar cyst (1 case), and pineal cyst and mesiotemporal sclerosis (1 case each). Based on the correlation between the clinical and the imaging results of this series, the authors recommend MRI as the imaging method of choice in the investigation of precocious puberty. (orig.)

Asymmetry of the Vertebral Body and Pedicles in the True Transverse Plane in Adolescent Idiopathic Scoliosis: A CT-Based Study.

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

2017-01-01

Cross-sectional. To quantify the asymmetry of the vertebral bodies and pedicles in the true transverse plane in adolescent idiopathic scoliosis (AIS) and to compare this with normal anatomy. There is an ongoing debate about the existence and magnitude of the vertebral body and pedicle asymmetry in AIS and whether this is an expression of a primary growth disturbance, or secondary to asymmetrical loading. Vertebral body asymmetry, defined as left-right overlap of the vertebral endplates (ie, 100%: perfect symmetry, 0%: complete asymmetry) was evaluated in the true transverse plane on CT scans of 77 AIS patients and 32 non-scoliotic controls. Additionally, the pedicle width, length, and angle and the length of the ideal screw trajectory were calculated. Scoliotic vertebrae were on average more asymmetric than controls (thoracic: AIS 96.0% vs. controls 96.4%; p = .005, lumbar: 95.8% vs. 97.2%; p transverse pedicle angle was greater (12.3° vs. 5.7°; p transverse plane in AIS and no uniform relation between the axial rotation and vertebral asymmetry could be observed in these moderate to severe patients, suggesting that asymmetrical vertebral growth does not initiate rotation, but rather follows it as a secondary phenomenon. Level 4. Copyright © 2016 Scoliosis Research Society. Published by Elsevier Inc. All rights reserved.

Safety Extension Study Of Leuprolide Acetate (Lupron Depot) In The Treatment Of Central Precocious Puberty

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2014-01-08

Precocious; Leuprolide Acetate; Luteinizing Hormone (LH); Gonadotrophin-releasing Hormone Agonist (GnRHa); Tanner Staging; Depot Formulation; Suppression of LH; Central Precocious Puberty (CPP); Gonadotrophin-releasing Hormone (GnRH); Lupron; GnRH Analog; Pediatrics Central Precocious Puberty

Papular, profuse, and precocious keratosis pilaris.

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Castela, Emeline; Chiaverini, Christine; Boralevi, Franck; Hugues, Rosalind; Lacour, Jean Philippe

2012-01-01

Keratosis pilaris (KP) is a frequent and benign condition in children characterized by the presence of rough, follicular papules and varying degrees of erythema. Different variants have been described, including simple KP and red KP. Between September 2007 and October 2010, 11 children with profuse and precocious KP seen at the department of pediatric dermatology were included. They defined an underemphasized clinical variant of childhood KP: the papular, profuse, and precocious KP characterized by early age of onset (<18 mos), extensive involvement of the limbs and cheeks, and papular nature of lesions. No clinical association has been found. The main complication was episodes of folliculitis. Diagnosis was delayed for all patients. Treatment is difficult, but association between emollient and keratolytic agents can provide some help. © 2011 Wiley Periodicals, Inc.

Hypothalamic hamartoma associated with precocious puberty: Case report

International Nuclear Information System (INIS)

Bae, Won Kyong; Kim, Pyo Nuyn; Kim, Il Young; Lee, Byoung Ho; Lee, Kyeong Seok; Bae, Hack Gun; Yun, Il Gyu

1989-01-01

Hamartoma of the hypothalamic area is a well recognized cause of central precocious puberty. We report a case of histologically proven hypothalamic hamartoma in a 8 year old girl with precocious puberty. The CT showed an isodense, nonenhancing mass in suprasellar area, measuring 4.2 X 3.1 cm, which, to our knowledge, seems to be the largest one of the published cases. On MR imaging, the signal intensity of the mass was homogeneous and isointense relative to gray matter on T1-, and hyperintense on T2-weighted images. The clinical and radiologic findings of the published cases of hypothalamic hamartoma are reviewed

Hypothalamic hamartoma associated with precocious puberty: Case report

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Bae, Won Kyong; Kim, Pyo Nuyn; Kim, Il Young; Lee, Byoung Ho; Lee, Kyeong Seok; Bae, Hack Gun; Yun, Il Gyu [Soonchunhyang University, Chonan Hospital, Chonan (Korea, Republic of)

1989-12-15

Hamartoma of the hypothalamic area is a well recognized cause of central precocious puberty. We report a case of histologically proven hypothalamic hamartoma in a 8 year old girl with precocious puberty. The CT showed an isodense, nonenhancing mass in suprasellar area, measuring 4.2 X 3.1 cm, which, to our knowledge, seems to be the largest one of the published cases. On MR imaging, the signal intensity of the mass was homogeneous and isointense relative to gray matter on T1-, and hyperintense on T2-weighted images. The clinical and radiologic findings of the published cases of hypothalamic hamartoma are reviewed.

Premature pubarche before one year of age: distinguishing between mini-puberty variants and precocious puberty.

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Bourayou, Rafik; Giabicani, Eloïse; Pouillot, Monique; Brailly-Tabard, Sylvie; Brauner, Raja

2015-04-02

The aim of this study was to facilitate the distinction between the benign "mini-puberty of early infancy" and precocious puberty (PP). We compared 59 patients (21 boys and 38 girls) seen for pubic hair development before one year of age diagnosed as mini-puberty to 13 patients (2 boys) in whom pubertal development before one year revealed a PP. The boys with mini-puberty presented with pubic hair development and prepubertal testicular volume, with low plasma testosterone concentrations. Their gonadotropin responses to gonadotropin releasing hormone (GnRH) test showed predominant luteinising hormone increase in 9/13. The girls presented with pubic hair development that was accompanied by breast development in 47% of cases, with low plasma estradiol concentrations. Their gonadotropin responses showed predominant follicle-stimulating hormone increase in the 17 evaluated. The patients with PP had organic central PP (5 hypothalamic hamartoma) or idiopathic central PP (n=6), or peripheral PP (one ovarian tumor and one congenital adrenal hyperplasia). The diagnosis was challenging only in 3 girls with idiopathic central PP presenting with prepubertal plasma estradiol concentrations and responses to GnRH test. The diagnosis of PP was easily determined based on the clinical presentation and the pubertal concentrations of testosterone in boys or of estradiol in girls, as was the diagnosis of central or peripheral origin of PP based on gonadotropin response to the GnRH test. Once PP is excluded, these patients need careful follow-up and physician consultation is needed if clinical pubertal signs progress.

Serum levels of growth hormone binding protein in children with normal and precocious puberty

DEFF Research Database (Denmark)

Juul, A; Fisker, Sidse; Scheike, Thomas Harder

2000-01-01

To study the regulation of GHBP serum levels by gonadal steroids in normal and precocious puberty.......To study the regulation of GHBP serum levels by gonadal steroids in normal and precocious puberty....

Significance of determination of female sex hormones (E2, LH, FSH), insulin-like growth factor I (IGF-I) and leptin in girls with precocious puberty

International Nuclear Information System (INIS)

Huang Jianrong

2004-01-01

Objective: To investigate the diagnostic value of determination of serum levels of estradiol (E 2 ), luteinizing hormone (LH) and follicle stimulating hormone (FSH), insulin-like growth factor (IGF-I) and leptin in girls with idiopathic central precocious puberty (ICPP). Methods: Serum E 2 , LH, FSH, IGF-I (with chemiluminescence immunoassay) and leptin (with RIA) levels were determined in 35 girls with early development of breast as the sign of precocious puberty, of which, 15 was considered to be of the ICPP group and 20 of simple premature thelarche group (SPT). Criteria of diagnosis for ICPP were: peak LH value>12IU/L and LH/FSH>1 after GnRH stimulating test. Results: Serum E 2 , LH, FSH, IGF-I leptin levels in girls with ICPP were significantly higher than those in the girls with SPT (P 0.2 as the cut-off value for diagnosis of ICPP there would still be a positive rate of 86.6% suggesting that the diagnostic criteria might be set lower. Serum IGF-I levels were positively correlated to those of E 2 (r=0.47, P 2 and IGF-I. Conclusion: Determinations of serum E 2 , LH, FHS, IGF-I and leptin levels were helpful for the early diagnosis of ICPP. (author)

Increased risk of precocious puberty in internationally adopted children in Denmark

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Teilmann, G.; Pedersen, Carsten Bøcker; Skakkebæk, N.E.

2006-01-01

settings from birth to adoption, whereas most other countries are reported to take care of the children in orphanages before adoption. It can only be speculated whether a relation between preadoption living conditions and later risk of precocious puberty exists. Genetic factors play a key role...... identified through the unique Danish Civil Registration System and subsequently categorized as being Danish (N = 1,062,333), adopted (N = 10,997), immigrating with their family (N = 72,181), or being descendants of immigrants (N = 128,152). The incidence rate ratio of precocious puberty was estimated by log......-linear Poisson regression. All rate ratios were adjusted for age and its interaction with gender and calendar year. P values were based on likelihood ratio tests, and 95% confidence intervals were calculated by Wald's test. RESULTS: In the study period, 655 children developed precocious puberty during 5...

Central precocious puberty: from physiopathological mechanisms to treatment.

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Chirico, V; Lacquaniti, A; Salpietro, V; Buemi, M; Salpietro, C; Arrigo, T

2014-01-01

Puberty is a complex, coordinated biological process with multiple levels of regulations. The timing of puberty varies greatly in children and it is influenced by environmental, endocrine and genetic factors. Precocious puberty (PP) is an important issue, affecting between 1 in 5.000-10.000 children. The physiopathological mechanism is still unknown. From an etiological point of view, PP may be subdivided into gonadotropin-releasing hormone (GnRH) -dependent and independent causes. GnRH-dependent PP, often called central precocious puberty (CPP), is based on hypothalamic-pituitary-gonadal axis activation associated with progressive pubertal development, accelerated growth rate and advancement of skeletal age. Conversely, peripheral precocious puberty (PPP) is related to sex steroid exposure, independently of hypothalamic-–pituitary-–gonadal (HPG) axis activation. Kisspeptins play a central role in the modulation of GnRH secretion with peripheral factors that influence the timing of puberty, such as adipokines and endocrine disrupting chemicals. Moreover, PP could be related to genetic disorders, involving pivotal genes of the HPG axis. The standard test used to verify HPG activity is the gonadotropin response to administered GnRH analogs. We describe the physiopathological mechanisms of PP and its clinical implications, analysing diagnostic flow-chart and new potential biomarkers that could reveal PP. An update of the current literature was also carried out regarding the recent novelty for treatment.

A missense mutation in MKRN3 in a Danish girl with central precocious puberty and her brother with early puberty

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Känsäkoski, Johanna; Raivio, Taneli; Juul, Anders

2015-01-01

BACKGROUND: Idiopathic central precocious puberty (ICPP) results from the premature reactivation of the hypothalamic-pituitary-gonadal axis leading to development of secondary sexual characteristics prior to 8 y in girls or 9 y in boys. Since the initial discovery of mutations in the maternally...... hypothalamic complementary DNA (cDNA) was investigated by PCR. RESULTS: One paternally inherited rare variant, c.1034G>A (p.Arg345His), was identified in one girl with ICPP and in her brother with early puberty. The variant is predicted to be deleterious by three different in silico prediction programs....... Expression of MKRN3 was confirmed in adult human hypothalamus. CONCLUSION: Our results are in line with previous studies in which paternally inherited MKRN3 mutations have been found both in males and in females with ICPP or early puberty. Our report further expands the set of MKRN3 mutations identified...

Cellular and molecular basis of chronic constipation: taking the functional/idiopathic label out.

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Bassotti, Gabrio; Villanacci, Vincenzo; Creţoiu, Dragos; Creţoiu, Sanda Maria; Becheanu, Gabriel

2013-07-14

In recent years, the improvement of technology and the increase in knowledge have shifted several strongly held paradigms. This is particularly true in gastroenterology, and specifically in the field of the so-called "functional" or "idiopathic" disease, where conditions thought for decades to be based mainly on alterations of visceral perception or aberrant psychosomatic mechanisms have, in fact, be reconducted to an organic basis (or, at the very least, have shown one or more demonstrable abnormalities). This is particularly true, for instance, for irritable bowel syndrome, the prototype entity of "functional" gastrointestinal disorders, where low-grade inflammation of both mucosa and myenteric plexus has been repeatedly demonstrated. Thus, researchers have also investigated other functional/idiopathic gastrointestinal disorders, and found that some organic ground is present, such as abnormal neurotransmission and myenteric plexitis in esophageal achalasia and mucosal immune activation and mild eosinophilia in functional dyspepsia. Here we show evidence, based on our own and other authors' work, that chronic constipation has several abnormalities reconductable to alterations in the enteric nervous system, abnormalities mainly characterized by a constant decrease of enteric glial cells and interstitial cells of Cajal (and, sometimes, of enteric neurons). Thus, we feel that (at least some forms of) chronic constipation should no more be considered as a functional/idiopathic gastrointestinal disorder, but instead as a true enteric neuropathic abnormality.

Origins of knowledge: Insights from precocial species

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Elisabetta eVersace

2015-12-01

Full Text Available Behavioural responses are influenced by knowledge acquired during the lifetime of an individual and by predispositions transmitted across generations. Establishing the origin of knowledge and the role of the unlearned component is a challenging task, given that both learned and unlearned knowledge can orient perception, learning, and the encoding of environmental features since the first stages of life. Ethical and practical issues constrain the investigation of unlearned knowledge in altricial species, including human beings. On the contrary, precocial animals can be tested on a wide range of tasks and capabilities immediately after birth and in controlled rearing conditions. Insects and precocial avian species are very convenient models to dissect the knowledge systems that enable young individuals to cope with their environment in the absence of specific previous experience. We present the state of the art of research on the origins of knowledge that come from different models and disciplines. Insects have been mainly used to investigate unlearned sensory preferences and prepared learning mechanisms. The relative simplicity of the neural system and fast life cycle of insects make them ideal models to investigate the neural circuitry and evolutionary dynamics of unlearned traits. Among avian species, chicks of the domestic fowl have been the focus of many studies, and showed to possess unlearned knowledge in the sensory, physical, spatial, numerical and social domains. Solid evidence shows the existence of unlearned knowledge in different domains in several species, from sensory and social preferences to the left-right representation of the mental number line. We show how non-mammalian models of cognition, and in particular precocial species, can shed light into the adaptive value and evolutionary history of unlearned knowledge.

Origins of Knowledge: Insights from Precocial Species.

Science.gov (United States)

Versace, Elisabetta; Vallortigara, Giorgio

2015-01-01

Behavioral responses are influenced by knowledge acquired during the lifetime of an individual and by predispositions transmitted across generations. Establishing the origin of knowledge and the role of the unlearned component is a challenging task, given that both learned and unlearned knowledge can orient perception, learning, and the encoding of environmental features since the first stages of life. Ethical and practical issues constrain the investigation of unlearned knowledge in altricial species, including human beings. On the contrary, precocial animals can be tested on a wide range of tasks and capabilities immediately after birth and in controlled rearing conditions. Insects and precocial avian species are very convenient models to dissect the knowledge systems that enable young individuals to cope with their environment in the absence of specific previous experience. We present the state of the art of research on the origins of knowledge that comes from different models and disciplines. Insects have been mainly used to investigate unlearned sensory preferences and prepared learning mechanisms. The relative simplicity of the neural system and fast life cycle of insects make them ideal models to investigate the neural circuitry and evolutionary dynamics of unlearned traits. Among avian species, chicks of the domestic fowl have been the focus of many studies, and showed to possess unlearned knowledge in the sensory, physical, spatial, numerical and social domains. Solid evidence shows the existence of unlearned knowledge in different domains in several species, from sensory and social preferences to the left-right representation of the mental number line. We show how non-mammalian models of cognition, and in particular precocial species, can shed light into the adaptive value and evolutionary history of unlearned knowledge.

Central precocious puberty in girls

International Nuclear Information System (INIS)

Argyropoulou, M.; Malandry, F.; Perignon, F.; Brunelle, F.; Brauner, R.; Rappaport, R.

1990-01-01

The treatment of central precocious puberty (CPP) in girls depends on its etiology and evolution, the latter based on the degree of accelerated bone maturation. The goal of this paper is to assess the diagnostic value of MR imaging in studying CPP in girls. Thirty-four girls with CPP were studied with MR imaging. Pituitary gland height (PGH) was measured on a sagittal midline image and compared with normal measurements. Correlations among clinical presentation, PGH, estradiol levels, and LH/FSH ratio were evaluated

A case of late presentation of precocious puberty due to pituitary astrocytoma

OpenAIRE

Fahimeh Soheilipour; Hossein Ghalaenov; Seyyed-Hossein Samedanifard; Fatemeh Jesmi

2015-01-01

The importance of assessing precocious puberty, especially in boys, is not only due to the great complications it has for the affected patients, but also to the fatal underlying diseases. Therefore, children with central precocious puberty should first undergo neuroimaging. In this case study, we present a 9.5-year-old boy who was referred to Rasoul-e-Akram Medical Center with increased intracranial pressure, nausea/vomiting, and severe headache having begun three months earlier. ...

Bilateral vocal fold paralysis and dysphagia secondary to diffuse idiopathic skeletal hyperostosis.

Science.gov (United States)

Allensworth, Jordan J; O'Dell, Karla D; Schindler, Joshua S

2017-01-01

Diffuse idiopathic skeletal hyperostosis (DISH) is an idiopathic spinal disease common in the elderly and characterized by flowing ossification and osteophyte formation along the spinal column. Cervical hyperostosis is capable of producing dysphagia, stridor, and airway obstruction; however, there are no extant reports of true paralysis of bilateral vocal folds in patients fulfilling the criteria for DISH. We report a case of a 61-year-old man presenting with dysphagia and dyspnea. Flexible laryngoscopy revealed bilateral true vocal fold paralysis. Cervical radiograph showed flowing ossification of the anterior longitudinal ligament with preservation of intervertebral disc height. Tracheotomy and cervical osteophytectomy were performed, after which the patient showed improved swallowing and speaking ability and was decannulated without complication. In the case presented, cervical osteophytectomy dramatically reversed bilateral vocal fold paralysis and dysphagia secondary to hyperostosis, thus negating the need for prolonged tracheostomy and feeding tube dependence. © 2016 Wiley Periodicals, Inc. Head Neck 39: E1-E3, 2017. © 2016 Wiley Periodicals, Inc.

Factors related to the occurrence of precocious menarche in female primary school students in Makassar

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Renjani, Rizky Swastika; Mappaware, Nasrudin A.; Nontji, Werna; Marniati

2017-09-01

Precocious menarche currently affects 1 in 5000 girls and likely to occur as many as 10 times and possess greater risk to the emergence of cancer in women. The aim of this research is to determine the relationship of nutrition status and lipid profile with precocious menarche occurrence onstudents of primary school in Makassar city. The design of this research was cross sectional design. The samples werethe students of grades IV, V and VI who have already menarche occurrence as many as 20 studentscollected with purposive sampling. The data were analyzed with Mann Whitney U test. The results indicate that there are 17 students (85%) do not undergoprecocious menarche, normal nutrition status is 11 persons (55%), normal cholesterol levelis 12 persons (60%), and level of normal LDL = 19 persons (95%). While the respondents who undergo precocious menarche is 3 persons (15%), fat nutrition status is 9 persons (45%), cautious cholesterol level is 8 persons (40%) and cautious LDL level is 1 person (5%). Based on Mann Whitney U test, there is relationship between nutrition status and precocious menarche occurrence (p = 0.043) and there is correlation of total cholesterol and LDLcholesterollevelswith precocious menarche (p = 0.025) and (p = 0.017). The decrease of knowledge about health of reproduction can be promoted in school by making cooperation with nutritionist to give information to parents and students of primary schools about good and healthy food.

Precocious pseudopuberty due to juvenile granulosa cell tumor

International Nuclear Information System (INIS)

Saeed, G.A.; Farooq, N.

2003-01-01

A case of precocious puberty occurring in a young girl is presented. Vaginal bleeding and secondary sexual characteristics had occurred at 7 years of age associated with an abdominal mass. These findings were due to a functional juvenile granulosa cell tumor in the right ovary. Right adenectomy was performed. Histopathology was confirmatory. (author)

Salt tolerance of precocious-dwarf cashew rootstocks: physiological and growth indexes

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Carneiro Paulo Torres

2004-01-01

Full Text Available The cashew crop (Anacardium occiedentale L. is of great economic and social importance for Northeast Brazil, a region usually affected by water and soil salinity. The present study was conducted in a greenhouse to evaluate the effects of four salinity levels established through electrical conductivity of irrigation water (ECw: 0.7, 1.4, 2.1 and 2.8 dS m-1, at 25ºC, on growth and physiological indexes of five rootstocks of dwarf-precocious cashew varieties CCP06, CCP09, CCP1001, EMBRAPA50, and EMBRAPA51. Plant height, leaf area, dry weight of root, shoot and total; water content of leaves, root/shoot ratio, leaf area ratio, absolute and relative growth rates and rate of net assimilation were evaluated. The majority of the evaluated variables were found to be affected by ECw and the effects varied among clones; however, no significant interactive effects were observed for factors. The value of ECw = 1.39 dS m-1 was considered as a threshold tolerance for the precocious cashew rootstocks used in this study. The dwarf-precocious cashew is moderately sensible to soil salinity during the formation phase of rootstock. Clones EMBRAPA51 and EMBRAPA50 presented, respectively, the least and the best development indexes.

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

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

2010-09-01

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

Hormonal changes during GnRH analogue therapy in children with central precocious puberty

DEFF Research Database (Denmark)

Müller, J; Juul, A; Andersson, A M

2000-01-01

Gonadotropin releasing hormone analogues (GnRHa) have been used for treatment of central precocious puberty (CPP) for more than 15 years. They are generally considered safe although data on potential long-term side effects are scarce. However, GnRHa therapy has profound effects on both the hypoth......Gonadotropin releasing hormone analogues (GnRHa) have been used for treatment of central precocious puberty (CPP) for more than 15 years. They are generally considered safe although data on potential long-term side effects are scarce. However, GnRHa therapy has profound effects on both...

Qualitative assessment of precocious puberty-related user-created contents on YouTube

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Hyo-Kyoung Nam

2015-09-01

Full Text Available PurposeUser-created content (UCC has provided a considerable amount of medical information and become an important source. We aimed to evaluate the quality and scientific accuracy of precocious puberty-related UCC on YouTube.MethodsThe keywords "precocious puberty", "early puberty", "sexual precocity", and "precocity" were searched for on YouTube during June and July 2014. More than 1,500 UCC matched the keywords. According to the information provider, UCC was classified as medical, oriental, or commercial & others. We evaluated the quality and scientific accuracy of the information provided in UCC using the DISCERN instrument and information scores, respectively.ResultsWe selected 51 UCC, which were categorized into three types: medical (n=17, oriental (n=17, or commercial & others (n=17. The overall quality score for medical UCC (3.4 was significantly higher relative to those of oriental and commercial & others UCC (2.8 and 2.3, respectively (P<0.001. In the assessment of scientific accuracy, the mean information score for medical UCC (30.7 was significantly higher than those of oriental and commercial & others UCC (15.9 and 5.1, respectively (P<0.001. The mean duration of oriental UCC was the longest (P<0.001, however, it was viewed less frequently among them (P=0.086.ConclusionThe quality and accuracy of precocious puberty-related health information in UCC were variable and often unreliable. The overall quality of UCC regarding precocious puberty was moderate. Only medical UCC provided scientifically accurate information. As UCC becomes a popular source of health information, it is important to provide reliable, scientifically accurate information.

A case of late presentation of precocious puberty due to pituitary astrocytoma

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Fahimeh Soheilipour

2015-08-01

Full Text Available The importance of assessing precocious puberty, especially in boys, is not only due to the great complications it has for the affected patients, but also to the fatal underlying diseases. Therefore, children with central precocious puberty should first undergo neuroimaging. In this case study, we present a 9.5-year-old boy who was referred to Rasoul-e-Akram Medical Center with increased intracranial pressure, nausea/vomiting, and severe headache having begun three months earlier. The development of secondary sexual changes had started two years earlier, and had been neglected. His testes, penis, and pubic hair were at the fourth Tanner stage. He had elevated luteinizing and follicle stimulating hormones. Microscopic evaluation confirmed low-grade pilocytic astrocytoma WHO grade 1. Emergency brain surgery was conducted in which the brain was decompressed, and chemotherapy was started postoperatively. Two years after the surgery, he remains under chemotherapy, with obvious sexual maturation and a height of 154 cm. Training families and medical staff efficiently can help prevent the late diagnosis and treatment of precocious puberty and, as a result, help patients in their social life.

Virilizing adrenocortical carcinoma advancing to central precocious puberty after surgery.

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Kim, Min Sun; Yang, Eu Jeen; Cho, Dong Hyu; Hwang, Pyung Han; Lee, Dae-Yeol

2015-05-01

Adrenocortical carcinoma (ACC) in pediatric and adolescent patients is rare, and it is associated with various clinical symptoms. We introduce the case of an 8-year-old boy with ACC who presented with peripheral precocious puberty at his first visit. He displayed penis enlargement with pubic hair and facial acne. His serum adrenal androgen levels were elevated, and abdominal computed tomography revealed a right suprarenal mass. After complete surgical resection, the histological diagnosis was ACC. Two months after surgical removal of the mass, he subsequently developed central precocious puberty. He was treated with a gonadotropin-releasing hormone agonist to delay further pubertal progression. In patients with functioning ACC and surgical removal, clinical follow-up and hormonal marker examination for the secondary effects of excessive hormone secretion may be a useful option at least every 2 or 3 months after surgery.

Direct repair of spondylolysis presenting after correction of adolescent idiopathic scoliosis.

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Koptan, Wael M T; ElMiligui, Yasser H; ElSharkawi, Mohammad M

2011-02-01

Although spondylolysis is found in 6% of idiopathic scoliosis patients, very little was reported on management of pars defects in this group. These patients with painful spondylolysis are most eligible for direct repair of the defect rather than lumbosacral fusion in an attempt to save motion segments. The aim of this work was to analyze the clinical and radiologic outcome of pars repair in a group of adolescents who presented after surgical correction of their idiopathic scoliosis. A prospective nonrandomized study. Ten consecutive patients with spondylolysis presenting after an average of 3 months (range, 2-7 months) from correction of their idiopathic scoliosis with low back pain not responding to conservative therapy and interfering with everyday activities. The mean age at operation was 16 years (range, 14-19 years). Total blood loss, operative time, and hospital stay were recorded. Clinical outcome was assessed by the Oswestry Disability Index (ODI), visual analog scale (VAS), and Scoliosis Research Society (SRS)-22 questionnaire. Fusion of the pars interarticularis was assessed using plain, lateral, and oblique radiographs and a computed tomography (CT) scan. The surgical technique consisted of thorough debridement of the defect, impacting the gap created with a tricortical iliac crest graft, and rigid fixation by either pedicle screws and a V-shaped rod (five patients) or a cable-screw construct (five patients). Patients were followed up for an average of 4.5 years (range, 2-7 years). Nine patients had a good-to-excellent result, returned to normal everyday life, and participated in sports when desired. The mean ODI, VAS, and SRS total scores were 11 (range, 0-34), 1.1 (range, 0-2), and 92 (range, 61-108), respectively. Follow-up radiographs and CT scans revealed healing of all defects in nine cases, no signs of disc degeneration in any, and no implant-related complications. The results of direct repair of spondylolysis in idiopathic scoliosis patients were

Insulin sensitivity and lipid profiles in girls with central precocious puberty before and during gonadal suppression

DEFF Research Database (Denmark)

Sørensen, Kaspar; Mouritsen, Annette; Mogensen, Signe Sloth

2010-01-01

Early menarche is associated with increased risk of cardiovascular disease in adulthood. It is unknown whether metabolic risk factors are adversely affected in girls with central precocious puberty (CPP) already at time of diagnosis.......Early menarche is associated with increased risk of cardiovascular disease in adulthood. It is unknown whether metabolic risk factors are adversely affected in girls with central precocious puberty (CPP) already at time of diagnosis....

Idiopathic portal hypertension

International Nuclear Information System (INIS)

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

1996-01-01

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

Idiopathic anaphylaxis.

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Fenny, Nana; Grammer, Leslie C

2015-05-01

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

Precocious metamorphosis in the juvenile hormone-deficient mutant of the silkworm, Bombyx mori.

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Takaaki Daimon

Full Text Available Insect molting and metamorphosis are intricately governed by two hormones, ecdysteroids and juvenile hormones (JHs. JHs prevent precocious metamorphosis and allow the larva to undergo multiple rounds of molting until it attains the proper size for metamorphosis. In the silkworm, Bombyx mori, several "moltinism" mutations have been identified that exhibit variations in the number of larval molts; however, none of them have been characterized molecularly. Here we report the identification and characterization of the gene responsible for the dimolting (mod mutant that undergoes precocious metamorphosis with fewer larval-larval molts. We show that the mod mutation results in complete loss of JHs in the larval hemolymph and that the mutant phenotype can be rescued by topical application of a JH analog. We performed positional cloning of mod and found a null mutation in the cytochrome P450 gene CYP15C1 in the mod allele. We also demonstrated that CYP15C1 is specifically expressed in the corpus allatum, an endocrine organ that synthesizes and secretes JHs. Furthermore, a biochemical experiment showed that CYP15C1 epoxidizes farnesoic acid to JH acid in a highly stereospecific manner. Precocious metamorphosis of mod larvae was rescued when the wild-type allele of CYP15C1 was expressed in transgenic mod larvae using the GAL4/UAS system. Our data therefore reveal that CYP15C1 is the gene responsible for the mod mutation and is essential for JH biosynthesis. Remarkably, precocious larval-pupal transition in mod larvae does not occur in the first or second instar, suggesting that authentic epoxidized JHs are not essential in very young larvae of B. mori. Our identification of a JH-deficient mutant in this model insect will lead to a greater understanding of the molecular basis of the hormonal control of development and metamorphosis.

An FSH and TSH pituitary adenoma, presenting with precocious puberty and central hyperthyroidism

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Guadalupe Vargas

2017-07-01

Full Text Available A 19-year-old woman with a history of isosexual precocious puberty and bilateral oophorectomy at age 10 years because of giant ovarian cysts, presents with headaches and mild symptoms and signs of hyperthyroidism. Hormonal evaluation revealed elevated FSH and LH levels in the postmenopausal range and free hyperthyroxinemia with an inappropriately normal TSH. Pituitary MRI showed a 2-cm macroadenoma with suprasellar extension. She underwent successful surgical resection of the pituitary tumor, which proved to be composed of two distinct populations of cells, each of them strongly immunoreactive for FSH and TSH, respectively. This mixed adenoma resulted in two different hormonal hypersecretion syndromes: the first one during childhood and consisting of central precocious puberty and ovarian hyperstimulation due to the excessive secretion of biologically active FSH and which was not investigated in detail and 10 years later, central hyperthyroidism due to inappropriate secretion of biologically active TSH. Although infrequent, two cases of isosexual central precocious puberty in girls due to biologically active FSH secreted by a pituitary adenoma have been previously reported in the literature. However, this is the first reported case of a mixed adenoma capable of secreting both, biologically active FSH and TSH.

Prospective assessment of pituitary size and shape on MR imaging after suppressive hormonal therapy in central precocious puberty

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Beek, J.T. van; Sharafuddin, M.J.A.; Kao, S.C.S. [Department of Radiology-JPP 3889, University of Iowa Hospitals and Clinics, 200 Hawkins Drive, Iowa City, IA 52246 (United States); Luisiri, A. [Cardinal Glennon Children' s Hospital, St. Louis, Missouri (United States); Garibaldi, L.R. [Children' s Hospital of New Jersey, Newark Beth Israel Medical Center, Newark, New Jersey (United States); St. Barnabas Medical Center, Livingston, New Jersey (United States)

2000-07-01

Objective. The diagnostic significance of an enlarged pituitary gland regarding both shape and size parameters on MR imaging has previously been demonstrated in children with central precocious puberty. This study was designed to assess changes in these parameters following successful suppressive therapy of central precocious puberty with the gonadotropin-releasing hormone (GnRH) analogue. Materials and methods. Twelve girls (mean age 7.3 years) with central precocious puberty were prospectively enrolled in our study protocol. Sagittal and coronal MR images of the pituitary region were obtained in all patients before treatment and after at least 6 months of GnRH analogue therapy (mean 18.0 months). Parameters measured included pituitary gland height, length, width, sagittal cross-sectional area, and volume. Results. All patients had excellent clinical response to treatment with arrest of secondary sexual development, normalization of serum estradiol levels, and complete obliteration of the LH response to diagnostic GnRH stimulation. No significant change occurred in any pituitary size or shape parameter following GnRH analogue therapy. Conclusion. Favorable clinical response to GnRH analogue therapy in central precocious puberty is not accompanied by significant a change in pituitary gland size and shape. (orig.)

Prospective assessment of pituitary size and shape on MR imaging after suppressive hormonal therapy in central precocious puberty

International Nuclear Information System (INIS)

Beek, J.T. van; Sharafuddin, M.J.A.; Kao, S.C.S.; Luisiri, A.; Garibaldi, L.R.

2000-01-01

Objective. The diagnostic significance of an enlarged pituitary gland regarding both shape and size parameters on MR imaging has previously been demonstrated in children with central precocious puberty. This study was designed to assess changes in these parameters following successful suppressive therapy of central precocious puberty with the gonadotropin-releasing hormone (GnRH) analogue. Materials and methods. Twelve girls (mean age 7.3 years) with central precocious puberty were prospectively enrolled in our study protocol. Sagittal and coronal MR images of the pituitary region were obtained in all patients before treatment and after at least 6 months of GnRH analogue therapy (mean 18.0 months). Parameters measured included pituitary gland height, length, width, sagittal cross-sectional area, and volume. Results. All patients had excellent clinical response to treatment with arrest of secondary sexual development, normalization of serum estradiol levels, and complete obliteration of the LH response to diagnostic GnRH stimulation. No significant change occurred in any pituitary size or shape parameter following GnRH analogue therapy. Conclusion. Favorable clinical response to GnRH analogue therapy in central precocious puberty is not accompanied by significant a change in pituitary gland size and shape. (orig.)

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

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

2015-01-01

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

Testes and brain gene expression in precocious male and adult maturing Atlantic salmon (Salmo salar

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Houeix Benoit

2010-03-01

Full Text Available Abstract Background The male Atlantic salmon generally matures in fresh water upon returning after one or several years at sea. Some fast-growing male parr develop an alternative life strategy where they sexually mature before migrating to the oceans. These so called 'precocious' parr or 'sneakers' can successfully fertilise adult female eggs and so perpetuate their line. We have used a custom-built cDNA microarray to investigate gene expression changes occurring in the salmon gonad and brain associated with precocious maturation. The microarray has been populated with genes selected specifically for involvement in sexual maturation (precocious and adult and in the parr-smolt transformation. Results Immature and mature parr collected from a hatchery-reared stock in January were significantly different in weight, length and condition factor. Changes in brain expression were small - never more than 2-fold on the microarray, and down-regulation of genes was much more pronounced than up-regulation. Significantly changing genes included isotocin, vasotocin, cathepsin D, anamorsin and apolipoprotein E. Much greater changes in expression were seen in the testes. Among those genes in the testis with the most significant changes in expression were anti-Mullerian hormone, collagen 1A, and zinc finger protein (Zic1, which were down-regulated in precocity and apolipoproteins E and C-1, lipoprotein lipase and anti-leukoproteinase precursor which were up-regulated in precocity. Expression changes of several genes were confirmed in individual fish by quantitative PCR and several genes (anti-Mullerian hormone, collagen 1A, beta-globin and guanine nucleotide binding protein (G protein beta polypeptide 2-like 1 (GNB2L1 were also examined in adult maturing testes. Down-regulation of anti-Mullerian hormone was judged to be greater than 160-fold for precocious males and greater than 230-fold for November adult testes in comparison to July testes by this method. For

Prostate specific antigen in boys with precocious puberty before and during gonadal suppression by GnRH agonist treatment

DEFF Research Database (Denmark)

Juul, A; Müller, J; Skakkebaek, N E

1997-01-01

antigen (PSA) is a marker of the androgen-dependent prostatic epithelial cell activity and it is used in the diagnosis and surveillance of adult patients with prostatic cancer. We have measured PSA concentrations in serum from boys with precocious puberty before and during gonadal suppression with Gn......RH agonists to evaluate the effect of normal and precocious puberty on PSA levels and to study the correlation between testosterone and PSA in boys....

Precocious puberty and large multicystic ovaries in young girls with primary hypothyroidism.

Science.gov (United States)

Sanjeevaiah, Aravind Raj; Sanjay, Subbarayappa; Deepak, Tejesweni; Sharada, Ardanareshwaran; Srikanta, Sri S

2007-10-01

To describe 2 cases of primary hypothyroidism, precocious puberty, large multicystic ovaries, possible diagnostic dilemma, unilateral oophorectomies, and subsequent response to levothyroxine replacement therapy. We present the clinical, biochemical, radiologic, and histopathologic findings in 2 patients with rare cases of Van Wyk-Grumbach syndrome and megaovaries, who underwent unilateral oophorectomy. Two patients, an 8-year-old girl and a 3-year-old girl (cases 1 and 2, respectively), were referred to our center. Both patients presented with precocious puberty and vaginal bleeding and had undergone unilateral oophorectomy before referral. In the first patient (case 1), the surgical intervention was a consequence of torsion of the left megaovary, necessitating emergency oophorectomy. Oophorectomy in the second patient (case 2) was a result of initial diagnostic confusion, inasmuch as a sexcord stromal tumor was suspected. A detailed history, physical examination, and laboratory results pointed toward primary hypothyroidism due to Hashimoto's thyroiditis and thyroid dysgenesis, respectively. Serial ultrasound studies of the abdomen and pelvis revealed large multicystic ovaries, with progressive enlargement (including regrowth from an apparent ovarian "postsurgical remnant"). Both patients responded dramatically after initiation of levothyroxine replacement therapy, with no further vaginal bleeding and reversal of megaovary to normal size (in case 1). In a highly selected minority of children with untreated primary hypothyroidism, there is development of precocious puberty and progressively enlarging multicystic ovaries. The precise endocrine, neuroanatomic, and neurophysiologic bases for this phenomenon are unclear. Nevertheless, the entire clinicopathologic picture, including giant ovaries, dramatically reverts to normal status with the restoration of a euthyroid state by means of simple levothyroxine replacement therapy.

Acquired versus innate prey capturing skills in super-precocial live-bearing fish

NARCIS (Netherlands)

Lankheet, Martin J.; Stoffers, Twan; Leeuwen, van Johan L.; Pollux, Bart J.A.

2016-01-01

Live-bearing fish start hunting for mobile prey within hours after birth, an example of extreme precociality. Because prenatal, in utero, development of this behaviour is constrained by the lack of free-swimming sensory-motor interactions, immediate success after birth depends on innate,

A missense mutation in MKRN3 in a Danish girl with central precocious puberty and her brother with early puberty.

Science.gov (United States)

Känsäkoski, Johanna; Raivio, Taneli; Juul, Anders; Tommiska, Johanna

2015-12-01

Idiopathic central precocious puberty (ICPP) results from the premature reactivation of the hypothalamic-pituitary-gonadal axis leading to development of secondary sexual characteristics prior to 8 y in girls or 9 y in boys. Since the initial discovery of mutations in the maternally imprinted MKRN3 gene in 2013, several case reports have described mutations in this gene in ICPP patients from different populations, highlighting the importance of MKRN3 as a regulator of pubertal onset. We screened 29 Danish girls with ICPP for mutations in MKRN3. Expression of MKRN3 in human hypothalamic complementary DNA (cDNA) was investigated by PCR. One paternally inherited rare variant, c.1034G>A (p.Arg345His), was identified in one girl with ICPP and in her brother with early puberty. The variant is predicted to be deleterious by three different in silico prediction programs. Expression of MKRN3 was confirmed in adult human hypothalamus. Our results are in line with previous studies in which paternally inherited MKRN3 mutations have been found both in males and in females with ICPP or early puberty. Our report further expands the set of MKRN3 mutations identified in ICPP patients across diverse populations, thus supporting the major regulatory function of MKRN3 in pubertal onset.

Molecular mechanism underlying juvenile hormone-mediated repression of precocious larval-adult metamorphosis.

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Kayukawa, Takumi; Jouraku, Akiya; Ito, Yuka; Shinoda, Tetsuro

2017-01-31

Juvenile hormone (JH) represses precocious metamorphosis of larval to pupal and adult transitions in holometabolous insects. The early JH-inducible gene Krüppel homolog 1 (Kr-h1) plays a key role in the repression of metamorphosis as a mediator of JH action. Previous studies demonstrated that Kr-h1 inhibits precocious larval-pupal transition in immature larva via direct transcriptional repression of the pupal specifier Broad-Complex (BR-C). JH was recently reported to repress the adult specifier gene Ecdysone-induced protein 93F (E93); however, its mechanism of action remains unclear. Here, we found that JH suppressed ecdysone-inducible E93 expression in the epidermis of the silkworm Bombyx mori and in a B. mori cell line. Reporter assays in the cell line revealed that the JH-dependent suppression was mediated by Kr-h1. Genome-wide ChIP-seq analysis identified a consensus Kr-h1 binding site (KBS, 14 bp) located in the E93 promoter region, and EMSA confirmed that Kr-h1 directly binds to the KBS. Moreover, we identified a C-terminal conserved domain in Kr-h1 essential for the transcriptional repression of E93 Based on these results, we propose a mechanism in which JH-inducible Kr-h1 directly binds to the KBS site upstream of the E93 locus to repress its transcription in a cell-autonomous manner, thereby preventing larva from bypassing the pupal stage and progressing to precocious adult development. These findings help to elucidate the molecular mechanisms regulating the metamorphic genetic network, including the functional significance of Kr-h1, BR-C, and E93 in holometabolous insect metamorphosis.

[Evolutionary perspective in precocious puberty].

Science.gov (United States)

Hochberg, Ze'ev

2014-10-01

Pubertal development is subject to substantial heritability, but much variation remains to be explained, including fast changes over the last 150 years, that cannot be explained by changes of gene frequency in the population. This article discusses the influence of environmental factors to adjust maturational tempo in the service of fitness goals. Utilizing evolutionary development thinking (evo-devo), the author examines adolescence as an evolutionary life-history stage in its developmental context. The transition from the preceding stage of juvenility entails adaptive plasticity in response to energy resources, social needs of adolescence and maturation toward youth and adulthood. Using Belsky's evolutionary theory of socialization, I show that familial psychosocial environment during the infancy-childhood and childhood-juvenility transitions foster a fast life-history and reproductive strategy rather than early maturation being just a risk factor for aggression and delinquency. The implications of the evo-devo framework for theory building, illuminates new directions in the understanding of precocious puberty other than a diagnosis of a disease.

A single sample GnRHa stimulation test in the diagnosis of precocious puberty

Science.gov (United States)

Gonadotropin-releasing hormone (GnRH) has been the standard test for diagnosing central precocious puberty. Because GnRH is no longer available, GnRH analogues (GnRHa) are now used. Random LH concentration, measured by the third-generation immunochemiluminometric assay, is a useful screening tool ...

Virilizing Adrenocortical Carcinoma Advancing to Central Precocious Puberty after Surgery

OpenAIRE

Kim, Min Sun; Yang, Eu Jeen; Cho, Dong Hyu; Hwang, Pyung Han; Lee, Dae-Yeol

2015-01-01

Adrenocortical carcinoma (ACC) in pediatric and adolescent patients is rare, and it is associated with various clinical symptoms. We introduce the case of an 8-year-old boy with ACC who presented with peripheral precocious puberty at his first visit. He displayed penis enlargement with pubic hair and facial acne. His serum adrenal androgen levels were elevated, and abdominal computed tomography revealed a right suprarenal mass. After complete surgical resection, the histological diagnosis was...

Adolescent Idiopathic Scoliosis

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Safak Ekinci

2014-06-01

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

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

Science.gov (United States)

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

2016-02-01

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

Serum IGF1 and insulin levels in girls with normal and precocious puberty

DEFF Research Database (Denmark)

Sørensen, Kaspar; Aksglaede, Lise; Petersen, Jørgen Holm

2012-01-01

IGF1 plays an important role in growth and metabolism during puberty. IGF1 levels are increased in girls with central precocious puberty (CPP). However, the relationship with insulin before and during gonadal suppression is unknown. In addition, the influence of the exon 3-deleted GH receptor gene...

Idiopathic chondrolysis - diagnostic difficulties

International Nuclear Information System (INIS)

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

1984-01-01

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

Data from: Acquired versus innate prey capturing skills in super-precocial live-bearing fish

NARCIS (Netherlands)

Lankheet, M.J.M.; Stoffers, Twan; Leeuwen, van J.L.; Pollux, B.J.A.

2016-01-01

Live-bearing fish start hunting for mobile prey within hours after birth, an example of extreme precociality. Because prenatal, in utero, development of this behaviour is constrained by the lack of free-swimming sensory-motor interactions, immediate success after birth depends on innate,

Spring Chinook Salmon Interactions Indices and Residual/Precocial Monitoring in the Upper Yakima Basin, Annual Report 1998

International Nuclear Information System (INIS)

James, Brenda B.; Pearsons, Todd N.; McMichael, Geoffrey A.

1999-01-01

Select ecological interactions and spring chinook salmon residual/precocial abundance were monitored in 1998 as part of the Yakima/Klickitat Fisheries Project's supplementation monitoring program. Monitoring these variables is part of an effort to help evaluate the factors that contribute to, or limit supplementation success. The ecological interactions that were monitored were prey consumption, competition for food, and competition for space. The abundance of spring chinook salmon life-history forms that have the potential to be influenced by supplementation and that have important ecological and genetic roles were monitored (residuals and precocials). Residual spring chinook salmon do not migrate to the ocean during the normal emigration period and continue to rear in freshwater. Precocials are those salmon that precocially mature in freshwater. The purpose of sampling during 1998 was to collect baseline data one year prior to the release of hatchery spring chinook salmon which occurred during the spring of 1999. All sampling that the authors report on here was conducted in upper Yakima River during summer and fall 1998. The stomach fullness of juvenile spring chinook salmon during the summer and fall averaged 12%. The food competition index suggested that mountain whitefish (0.59), rainbow trout (0.55), and redside shiner (0.55) were competing for food with spring chinook salmon. The space competition index suggested that rainbow trout (0.31) and redside shiner (0.39) were competing for space with spring chinook salmon but mountain whitefish (0.05) were not. Age-0 spring chinook salmon selected a fairly narrow range of microhabitat parameters in the summer and fall relative to what was available. Mean focal depths and velocities for age 0 spring chinook salmon during the summer were 0.5 m ± 0.2 m and 0.26 m/s ± 0.19 m/s, and during the fall 0.5 m ± 0.2 m and 0.24 m/s ± 0.18 m/s. Among potential competitors, age 1+ rainbow trout exhibited the greatest degree

Idiopathic Retroperitoneal Hematoma

African Journals Online (AJOL)

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

Intervention levels in a precocious detection program for breast cancer and evaluation of four participant units

International Nuclear Information System (INIS)

Carrera M, F.; Velazquez M, S.; Manzano M, F.J.; Sanchez S, J.

1998-01-01

It is presented the basis to make a cost benefit analysis for a breast cancer precocious detection program and consequently the keys for its optimization from the radiological point of view. Taking this as a reference it is made an exhaustive quality control to four mammographic unities which were participating or they were candidates to participate in a breast cancer precocious detection program. Also it is presented its results. It is followed the protocol for quality control in mammography in Spain obtaining values for the measurement of twelve interesting parameters. It should be maintained the standard breast dose about 1 mGy/ image. It should be available a 24 x 30 cm portacassete and considering the utilization of a single projection by breast. (Author)

Sex hormone binding globulin, free estradiol index, and lipid profiles in girls with precocious puberty

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Hyun-Wook Chae

2013-06-01

Full Text Available PurposeSex hormone-binding globulin (SHBG modulates the availability of biologically active free sex hormones. The regulatory role of SHBG might be important in the relationship between hormone levels and the modification of lipid profiles in girls with precocious puberty. However, few studies have evaluated the relationship of SHBG, free estradiol index (FEI, and lipid levels in these girls.MethodsOne hundred and nine girls less than 8 years of age with pubertal development were enrolled. FEI was calculated with SHBG and estradiol (E2. We analyzed SHBG between peak luteinizing hormone (LH≥5 (IU/L (group 1 and LH<5 (IU/L (group 2 through a gonadotropin releasing hormone stimulation test.ResultsBody mass index (BMI standard deviation score (SDS was higher in group 2 than in group 1 (P=0.004. Serum SHBG levels did not differ and FEI was not higher in group 1 (P=0.122. Serum cholesterol, HDL, and LDL did not differ; however, triglyceride levels were higher in group 2 (P=0.023. SHBG was negatively correlated with bone age advancement, BMI, BMI SDS, and FEI, and was positively correlated with HDL. However, SHBG was not correlated with E2 or peak LH.ConclusionSerum SHBG itself might not be associated with precocious puberty in girls, but it might be related to BMI and lipid profiles. Further studies are needed to reveal the relationship between sex hormone and obesity in girls with precocious puberty.

Genetics Home Reference: adolescent idiopathic scoliosis

Science.gov (United States)

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

STUDY OF CLINICAL PROFILE OF PATIENTS PRESENTING WITH SEXUAL PRECOCITY TO A TERTIARY CARE HOSPITAL

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Binoy Kumar Mohanty

2016-07-01

Full Text Available BACKGROUND Precocious puberty is a common paediatric endocrine disorder seen in clinical practice. OBJECTIVE To study the various aetiologies and clinical presentations of patients presenting with sexual precocity to a tertiary care hospital. DESIGN Cross sectional study. MATERIAL AND METHODS We collected and analysed the clinical data including hormonal status of 24 consecutive patients who presented to our department from January 2014 to December 2015 for evaluation of sexual precocity. RESULTS Most of the patients presenting to us had evidence of precocious puberty (n=16, followed by premature adrenarche (n=5 and premature thelarche (n=3 respectively. The females outnumbered males in our study (68.75% of total cases. Females presenting with central precocious puberty had no appreciable cause (idiopathic in majority (85.72% of cases. While, males presenting with central precocious puberty had an organic cause (60% in majority of cases. CONCLUSIONS Precocious puberty is more common among females as compared to males. Organic lesion must be ruled out in all patients presenting with central precocious puberty especially in males.

Leptin and adiponectin levels in girls with central precocious puberty before and during GnRH agonist treatment

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Jae Won Yoo

2016-12-01

Full Text Available PurposeThe effects of gonadotropin-releasing hormone agonist (GnRHa treatment on the energy metabolism in girls with central precocious puberty (CPP are controversial. We focused the changes and related factors of serum levels of leptin and adiponectin in girls with CPP before and during GnRHa treatment.MethodsThirty girls with idiopathic CPP were enrolled in the study. Their auxological data and fasting blood were collected at the baseline and after six months of GnRHa treatment.ResultsAfter treatment, height (P<0.001, weight (P<0.001, and serum leptin levels (P=0.033 were significantly increased, whereas body mass index (BMI, homeostasis model of assessment-insulin resistance, serum adiponectin levels, and adiponectin/leptin ratio exhibited no significant changes. A Pearson correlation analysis showed that height, weight, BMI, and their standard deviation scores (SDSs, but not basal LH, FSH, and estradiol, were significantly correlated with serum leptin levels before and after GnRHa treatment. After a multiple linear regression analysis, only BMI was associated with serum leptin levels. Moreover, leptin SDSs adjusted for BMI were not significantly different before and after GnRHa. The Δ leptin levels (r2=0.207, P=0.012, but not with Δ leptin SDS (r2=0.019, P=0.556, during GnRHa treatment were positively correlated with Δ BMI.ConclusionThese results suggest that GnRHa treatment in girls with CPP does not affect serum levels of leptin and adiponectin and insulin resistance. Serum leptin levels were depend on the changes in BMI during GnRHa treatment.

Insulin sensitivity and lipid profiles in girls with central precocious puberty before and during gonadal suppression

DEFF Research Database (Denmark)

Sørensen, Kaspar; Mouritsen, Annette; Mogensen, Signe Sloth

2010-01-01

Early menarche is associated with increased risk of cardiovascular disease in adulthood. It is unknown whether metabolic risk factors are adversely affected in girls with central precocious puberty (CPP) already at time of diagnosis....

Serum insulin-like growth factor I (IGF-I) and IGF-binding protein 3 levels are increased in central precocious puberty

DEFF Research Database (Denmark)

Juul, A; Scheike, Thomas Harder; Nielsen, C T

1995-01-01

Central precocious puberty (CPP) is characterized by early activation of the pituitary-gonadal axis, which leads to increased growth velocity and development of secondary sexual characteristics. It is generally believed that gonadal sex steroids stimulate pulsatile GH secretion, which, in turn......, stimulates insulin-like growth factor I (IGF-I) and IGF-binding protein 3 (IGFBP-3) production. However, little is known about GH, IGF-I, and IGFBP-3 serum levels in children with precocious puberty. Treatment of CPP by GnRH agonists has become the treatment of choice. However, the effect of long term...

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

Assessment of High Rates of Precocious Male Maturation in a Spring Chinook Salmon Supplementation Hatchery Program, Annual Report 2002-2003.

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Larsen, Donald; Beckman, Brian; Cooper, Kathleen

2003-08-01

The Yakima River Spring Chinook Salmon Supplementation Project in Washington State is currently one of the most ambitious efforts to enhance a natural salmon population in the United States. Over the past five years we have conducted research to characterize the developmental physiology of naturally- and hatchery-reared wild progeny spring chinook salmon (Oncorhynchus tshawytscha) in the Yakima River basin. Fish were sampled at the main hatchery in Cle Elum, at remote acclimation sites and, during smolt migration, at downstream dams. Throughout these studies the maturational state of all fish was characterized using combinations of visual and histological analysis of testes, gonadosomatic index (GSI), and measurement of plasma 11-ketotestosterone (11-KT). We established that a plasma 11-KT threshold of 0.8 ng/ml could be used to designate male fish as either immature or precociously maturing approximately 8 months prior to final maturation (1-2 months prior to release as 'smolts'). Our analyses revealed that 37-49% of the hatchery-reared males from this program undergo precocious maturation at 2 years of age and a proportion of these fish appear to residualize in the upper Yakima River basin throughout the summer. An unnaturally high incidence of precocious male maturation may result in loss of potential returning anadromous adults, skewing of female: male sex ratios, ecological, and genetic impacts on wild populations and other native species. Precocious male maturation is significantly influenced by growth rate at specific times of year and future studies will be conducted to alter maturation rates through seasonal growth rate manipulations.

Atypical idiopathic inflammatory demyelinating lesions

DEFF Research Database (Denmark)

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

2013-01-01

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

Psychosexual development, maternalism, nonpromiscuity, and body image in 15 females with precocious puberty.

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Money, J; Walker, P A

1971-03-01

Fifteen females with a history of idiopathic sexual precocity were selected without known sampling bias. They had been followed for as long as 18 years. They showed various behavioral characteristics as a group, but were also individually unique. If the IQ permitted, they benefited socially from school acceleration. Left to their own devices, the majority preferred friends nearer to them in physique age rather than chronologic age. Play interests, though influenced by the age of playmates, showed no features unique to the precocious onset of hormonal puberty. About half of the girls had occasional moody or depressed spells and wanted to be left alone. Maternalistic interests were strongly represented; only one girl was a tomboy. Masturbation and sexual play in childhood were rarely confirmed, and in no instances were totally contrary to family or community mores. No consistent progression of erotic dream content was discerned. Dreams of having a baby were rare, but antedated intercourse dreams, which were also rarely reported and did not include sensations of climax. Whereas the youngest age of having a serious boyfriend was 8 years, and the youngest age of intercourse, 11, the majority of girls did not report romantic and sexual involvements before the middle teenage years or later. In the three instances of marriage, the youngest was at age 21. Motherhood has so far been achieved by only one patient. She delivered her first child at age 11. The visible appearance of early sexual development and early advanced statural growth created a problem in childhood human relationships for most of the girls, regardless of what they said, and regardless of their skill or ineptitude in handling it. They all benefited even from minimal counseling, as did their parents. Early appearance of physical sexual development does not automatically lead to premature engagement in erotic activity or promiscuous sexual behavior. Such activity and behavior require appropriate experience

Hypothalamic hamartoma with precocious puberty: a case report.

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Amin, M S; Kader, M A; Huq, F I; Khan, N A

2012-07-01

Hypothalamic hamartoma (HH) is one of the most important causes of central precocious puberty in male children. Hamartomas are malformations composed of ectopic gonadotropic hormone (GnRH) neurons which secrete pulsatile gonadotropin releasing hormone. They are generally observed in children under 3 years. A case of 11/3 year-old male child presented with premature development of secondary sexual characters i.e., growth of pubic and axillary hair, enlargement of penis and acne over the face for the last 5 months. On physical examination, his height was 1.02 m and his weight 18kg, enlarged penile length of which 58mm; testicles were enlarged in size right one measuring 32X25mm and the left 30X23mm. His hematological and other biochemical investigations revealed no abnormality. Plain radiographic examination revealed radiological bone age of about 8-9 years. Endocrinological findings were as follows: Follicle stimulating hormone (FSH): 1.5mIU/ml, Luteinizing hormone (LH): 9.1mIU/ml, Testosterone: 701ng/dl (Testosterone level less than 30ng/dl in prepubertal age). Thyroid function tests were normal. Patient showed no adrenal pathology on ultrasound and his testicular parenchyma was homogeneous echotexture with the size of 30X22X16mm on the right (volume 5.4ml) and 30X20X15mm on the left (volume 4.6ml). With above physical & endocrinological findings and age of the child, it was suspected as a case of central precocious puberty. Subsequently MR imaging of the brain done & showed an oval non-enhancing pedunculated hypothalamic mass arising from the tubercinereum that was iso to hypointense to brain parenchyma on T1 - and intermediate signal on T2-weighted images, 20X10X10mm in diameter, extending into suprasellar cistern. During follow up after 06 months of starting conservative medication with gonadotropin-releasing hormone (GnRH) analog (Leuprolide acetate), his progression of puberty has been arrested and the testosterone level 18ng/dl, which is normal for his age.

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

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

2007-06-01

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

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

Science.gov (United States)

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

2014-04-01

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

Treatment of central precocious puberty by subcutaneous injections of leuprorelin 3-month depot (11.25 mg).

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Carel, Jean-Claude; Lahlou, Najiba; Jaramillo, Orlando; Montauban, Vincent; Teinturier, Cécile; Colle, Michel; Lucas, Christel; Chaussain, Jean Louis

2002-09-01

Depot GnRH agonists are widely used for the treatment of precocious puberty. Leuprorelin 3-month depot is currently used in adults but has not been evaluated in children. We evaluated the efficacy of this new formulation (11.25 mg every 3 months), for the suppression of gonadotropic activation and pubertal signs in children with central precocious puberty. We included 44 children (40 girls) with early-onset pubertal development in a 6-month open trial. The inclusion criteria were clinical pubertal development before the age of 8 (girls) or 10 (boys), advanced bone age, enlarged uterus (>36 mm), testosterone more than 1.7 nmol/liter (boys), and pubertal response of LH to GnRH (peak >5 IU/liter). The principal criterion for efficacy assessment, GnRH-stimulated LH peak less than 3 IU/liter, was met in 81 of 85 (95%) of the tests performed at months 3 and 6. The remaining four values were slightly above the threshold. The levels of sex steroids were also significantly reduced and clinical pubertal development was arrested. Plasma leuprorelin levels, measured every 30 d, were essentially stable after d 60. Local intolerance was noted after 10 of 86 injections (12%), and was mild in four cases, moderate in five cases, and severe in one. Among these 10 events, 4 consisted in local pain at injection's site. In conclusion, leuprorelin 3-month depot efficiently inhibits the gonadotropic axis in 95% of children with central precocious puberty studied for a 6-month period. This regimen allows the reduction of the number of yearly injections from 12 to 4.

Precocious scaling in antiproton-proton scattering at low energies

International Nuclear Information System (INIS)

Ion, D.B.; Petrascu, C.; Topor Pop, V.; Popa, V.

1993-08-01

The scaling of the diffraction peak in antiproton-proton scattering has been investigated from nera threshold up to 3 GeV/c laboratory momenta. It was shown that the scaling of the differential cross sections are evidentiated with a surprising accuracy not only at high energies, but also at very low ones (e.g. p LAB = 0.1 - 0.5 GeV/c), beyond the resonance and exotic resonance regions. This precocious scaling strongly suggests that the s-channel helicity conservation (SCHC) can be a peculiar property that should be tested in antiproton-proton interaction not only at high energies but also at low energy even below p LAB = 1 GeV/c. (author). 36 refs, 9 figs

GnRH-dependent precocious puberty manifested at the age of 14 months in a girl with 47,XXX karyotype.

Science.gov (United States)

Skordis, Nicos; Ferrari, Eleana; Antoniadou, Aria; Phylactou, Leonidas A; Fanis, Pavlos; Neocleous, Vassos

2017-07-01

This case report describes a 47,XXX girl who presented very early, at the age of 14 months, with signs of sexual precocity (breast and pubic hair development, menarche) and was finally diagnosed with GnRH dependent precocious puberty with no evidence of underlying central nervous system pathology. Molecular testing did not identify any genetic defect in any of the genes tested (KISS1, KISS1R, DLK1 and the intronless MKRN3). Though previous studies have shown a link between karyotype 47,XXX and precocious puberty, this is the youngest patient reported so far. Treatment with GnRH analog was commenced and proved to be effective, indicating a successful suppression of the hypothalamic-pituitary-ovarian axis.

Precocious Degenerative Arthropathy And Bluish Patches On Ears : Ochronosis And Alkaptonuria

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Mahajan Vikram K

2004-01-01

Full Text Available Alkaptonuria is a rare, autosomal recessive disorder of phenylalanin/tyrosine metabolism due to congenital deficiency of the enzyme homogentisic acid oxidase. The diagnosis is clinical and the triad of homogentisic aciduria, ochronosis and precocious degenerative arthritis is characteristic. Its diagnosis in infancy and early therapeutic intervention help delaying its complications. These patients may remain undiagnosed until the darkening of urine soaked diapers is noticed or the early degenerative arthropathy develops. This paper describes two cases of alkaptonuria presenting late in life; one of them had associated hyperthyroidism.

Final height after gonadotrophin releasing hormone agonist treatment for central precocious puberty : The Dutch experience

NARCIS (Netherlands)

Mul, D; Oostdijk, W; Otten, BJ; Rouwe, C; Jansen, M; Delemarre-van de Waal, HA; Waelkens, JJJ; Drop, SLS

Final height (FH) data of 96 children (87 girls) treated with GnRH agonist for central precocious puberty were studied. In girls mean FH exceeded initial height prediction by 7.4 (5.7) cm (p <0.001); FH was significantly lower than target height, but still in the genetic target range. When treatment

Idiopathic Intracranial Hypertension (Pseudotumor Cerebri)

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

Precocious germination and its regulation in embryos of triticale caryopses

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Stanisław Weidner

2014-01-01

Full Text Available Triticale var. Lasko embryos, isolated from grain gathered at milk ripeness, the beginning of wax ripeness and at full ripeness, were allowed to germinate for 48 h on agar with glucose. The highest incorporation of tritiated adenosine into polyribosomal RNA during germination was found in the ribosome fractions from embryos of grain gathered at full ripeness, lower incorporation was in preparations from embryos of milk ripe grain and the lowest in preparations from embryos of wax ripe grain. Different tendencies were observed in respect to the synthesis of ribosomal proteins. The highest incorporation of 14C-amino acids into ribosomal proteins was found in preparations of ribosome fractions from embryos of milk ripe grain, lower in preparations of embryos from fully ripe grain, the lowest in preparations of embryos from wax ripe grain. ABA (10-4 M completely inhibited the external symptoms of germination of immature embryos, while its inhibition of the synthesis of polyribosomal RNA and ribosomal proteins was greater the more mature the embryos that were germinated. The greatest stimulation of precocious germination by exogenous BA and GA3 was demonstrated in the least mature embryos isolated from milk ripe grain. Under the influence of both stimulators, an increase of the proportion of polyribosomes in the total ribosome fraction occurred in this sample, as did a rise in the intensity of ribosomal protein synthesis. The incorporation of 3H-adenosine into polyribosomal RNA, however, was lower than in the control sample. The results obtained suggest that the regulation of precocious germination of triticale embryos by phyto-hormones is not directly related to transcription.

Trabalho precoce: realidade social e desafio de política pública Precocious labor: a social reality and a challenge to public policy

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Sonia Rocha

2003-01-01

Full Text Available This article presents the basic characteristics of precocious labor in Brazil in the nineties. Since the age individuals start working does not seem to be an important determinant of future income, we examined its related aspects, such as school attendance and hard work incidence of. As precocious labor still involves large contingents of children in Brazil under quite different working conditions, it is essential to distinguish the most critical situations so as to support a social policy design targeted at a specific clientele.

Maternal-fetal communication of circadian phase in a precocious rodent, the spiny mouse

International Nuclear Information System (INIS)

Weaver, D.R.; Reppert, S.M.

1987-01-01

The development of circadian rhythms was examined in a precocious rodent species, the spiny mouse. Spiny mouse pups born and reared in constant darkness expressed robust circadian rhythms in locomotor activity as early as day 5 of live. Free-running activity rhythms of pups born and reared in constant darkness were coordinated with the dam on the day of birth. Postnatal maternal influences on pup rhythmicity are minimal in this species, as pups fostered on the day of birth to dams whose circadian phases were opposite to the pups' original dams were coordinated with their original dams on the day of birth. Studies using 2-deoxy-D-[1- 14 C]-glucose authoradiography showed that there were synchronous (coordinated) rhythms in metabolic activity in the maternal and fetal suprachiasmatic nuclei, directly demonstrating prenatal coordination of maternal and fetal rhythmicity. Maternal-fetal coordination of circadian phase was not the result of direct entrainment of the fetuses to the environmental light-dark cycle. These results demonstrate that there is prenatal communication of circadian phase in this precocious species, without demonstrable postnatal maternal influences on pup circadian rhythmicity. Spiny mice therefore represent an important animal model in which circadian rhythms in the postnatal period can be used to precisely assess prenatal influences on circadian phase

Temporal dynamics of milk composition of the precocial caviomorph Octodon degus (Rodentia: Octodontidae Dinámica temporal de la composición de la leche del caviomorfo precocial Octodon degus (Rodentia: Octodentidae

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CLAUDIO VELOSO

2005-06-01

Full Text Available During lactation, both the nutritional and energetic requirements of suckling change gradually. These changes normally are accompanied by modifications in chemical composition of the milk. We investigated the temporal course of milk composition during lactation in a precocial caviomorph rodent, the "degu" (Octodon degus under laboratory condition. Female degus were kept in laboratory during gestation and lactation and fed with commercial food pellets. Milk was collected at three stages of lactation: early (days 5-8, n = 12, middle (days 15-21, n = 7 and late (days 26-40, n = 6, and analyzed for protein, carbohydrates, lipids, ash, total solids and energy. On average, carbohydrates decreased from 3.1 ± 0.3 % (early to 1.1 ± 0.3 % (late during lactation; lipids, protein, ash, total solids and energy remained about the same. Lipids, the main component of the milk, were 17.3 % and protein remained near 4.4 %. Over lactation, total energy concentration of milk remained near 4.0 kJ mL-1. The maintenance of milk composition during lactation may be related to the initially high energetic and nutritional requirements associated with a precocial reproductive modeDurante la lactancia, tanto los requerimientos energéticos como nutricionales de las crías cambian gradualmente. Estos cambios normalmente van acompañados por modificaciones en la composición química de la leche. Se investigaron los cambios temporales de la composición de la leche durante la lactancia en el roedor caviomorfo precocial "degu" (Octodon degus bajo condiciones de laboratorio. Las hembras de degu fueron mantenidas en el laboratorio durante preñez y lactancia, y fueron alimentadas con alimento comercial de conejo. La leche fue colectada en tres estados de lactancia: temprana (días 5-8, n = 12, media (días 15-21, n = 7 y tardía (días 26-40, n = 6, y analizadas para proteínas, hidratos de carbono, lípidos, ceniza, sólidos totales y energía. En promedio, los hidratos de

Idiopathic ventricular tachycardia and fibrillation.

Science.gov (United States)

Belhassen, B; Viskin, S

1993-06-01

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

Stephen C. Woods: a precocious scientist.

Science.gov (United States)

Smith, Gerard P

2011-04-18

To investigate the early scientific development of Steve Woods, I reviewed his research during the first decade after he received his doctoral degree in 1970. The main parts of his research program were conditioned insulin secretion and hypoglycemia, Pavlovian conditioning of insulin secretion before a scheduled access to food, and basal insulin as a negative-feedback signal from fat mass to the brain. These topics were pursued with experimental ingenuity; the resulting publications were interesting, clear, and rhetorically effective. Although the theoretical framework for his experiments with insulin was homeostatic, by the end of the decade he suggested that classic negative-feedback homeostasis needed to be revised to include learning acquired by lifestyle. Thus, Woods functioned as a mature scientist from the beginning of his research-he was very precocious. This precocity also characterized his teaching and mentoring as recalled by two of his students during that time, Joseph Vasselli and Paul Kulkosky. The most unusual and exemplary aspect of his precocity is that the outstanding performance of his first decade was maintained during the subsequent 30years. Copyright © 2011 Elsevier Inc. All rights reserved.

Adult height in girls with central precocious puberty treated with gonadotropin-releasing hormone agonist with or without growth hormone

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Mo Kyung Jung

2014-12-01

Full Text Available PurposeThere is controversy surrounding the growth outcomes of treatment with gonadotropin-releasing hormone agonist (GnRHa in central precocious puberty (CPP. We analyzed height preservation after treatment with GnRHa with and without growth hormone (GH in girls with CPP.MethodsWe reviewed the medical records of 82 girls with idiopathic CPP who had been treated with GnRHa at Severance Children's Hospital from 2004 to 2014. We assessed the changes in height standard deviation score (SDS for bone age (BA, and compared adult height (AH with midparental height (MPH and predicted adult height (PAH during treatment in groups received GnRHa alone (n=59 or GnRHa plus GH (n=23.ResultsIn the GnRHa alone group, the height SDS for BA was increased during treatment. AH (160.4±4.23 cm was significantly higher than the initial PAH (156.6±3.96 cm (P<0.001, and it was similar to the MPH (159.9±3.52 cm. In the GnRHa plus GH group, the height SDS for BA was also increased during treatment. AH (159.3±5.33 cm was also higher than the initial PAH (154.6±2.55 cm (P<0.001, which was similar to the MPH (158.1±3.31 cm. Height gain was slightly higher than that in the GnRHa alone group, however it statistically showed no significant correlation with GH treatment.ConclusionIn CPP girls treated with GnRHa, the height SDS for BA was increased, and the AH was higher than the initial PAH. Combined GH treatment showed a limited increase in height gain.

Laterization of epileptiform discharges in patients with epilepsy and precocious destructive brain insults

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Teixeira Ricardo A.

2004-01-01

Full Text Available Unilateral destructive brain lesions of early development can result in compensatory thickening of the ipsilateral cranial vault. The aim of this study was to determine the frequency of these bone changes among patients with epilepsy and precocious destructive lesions, and whether a relationship exists between these changes and epileptiform discharges lateralization. Fifty-one patients had their ictal / interictal scalp EEG and skull thickness symmetry on MRI analyzed. Patients were divided into three main groups according to the topographic distribution of the lesion on the MRI: hemispheric (H (n=9; main arterial territory (AT (n=25; arterial borderzone (Bdz (n=17. The EEG background activity was abnormal in 26 patients and were more frequent among patients of group H (p= 0.044. Thickening of the skull was more frequent among patients of group H (p= 0.004. Five patients (9.8% showed discordant lateralization between epileptiform discharges and structural lesion (four of them with an abnormal background, and only two of them with skull changes. In one of these patients, ictal SPECT provided strong evidence for scalp EEG false lateralization. The findings suggest that compensatory skull thickening in patients with precocious destructive brain insults are more frequent among patients with unilateral and large lesions. However, EEG lateralization discordance among these patients seems to be more related to EEG background abnormalities and extent of cerebral damage than to skull changes.

Final height in central precocious puberty after long term treatment with a slow release GnRH agonist

NARCIS (Netherlands)

Oostdijk, W; Rikken, B; Schreuder, S; Otten, Barto; Odink, R; Rouwe, C; Jansen, M; Gerver, WJ; Waelkens, J; Drop, S

1996-01-01

Objective-To study the resumption of puberty and the final height achieved in children with central precocious puberty (CPP) treated with the GnRH agonist triptorelin. Patients-31 girls and five boys with CPP who were treated with triptorelin 3.75 mg intramuscularly every four weeks. Girls were

Genetics Home Reference: idiopathic pulmonary fibrosis

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

Performance measurement of PSF modeling reconstruction (True X) on Siemens Biograph TruePoint TrueV PET/CT.

Science.gov (United States)

Lee, Young Sub; Kim, Jin Su; Kim, Kyeong Min; Kang, Joo Hyun; Lim, Sang Moo; Kim, Hee-Joung

2014-05-01

The Siemens Biograph TruePoint TrueV (B-TPTV) positron emission tomography (PET) scanner performs 3D PET reconstruction using a system matrix with point spread function (PSF) modeling (called the True X reconstruction). PET resolution was dramatically improved with the True X method. In this study, we assessed the spatial resolution and image quality on a B-TPTV PET scanner. In addition, we assessed the feasibility of animal imaging with a B-TPTV PET and compared it with a microPET R4 scanner. Spatial resolution was measured at center and at 8 cm offset from the center in transverse plane with warm background activity. True X, ordered subset expectation maximization (OSEM) without PSF modeling, and filtered back-projection (FBP) reconstruction methods were used. Percent contrast (% contrast) and percent background variability (% BV) were assessed according to NEMA NU2-2007. The recovery coefficient (RC), non-uniformity, spill-over ratio (SOR), and PET imaging of the Micro Deluxe Phantom were assessed to compare image quality of B-TPTV PET with that of the microPET R4. When True X reconstruction was used, spatial resolution was RC with True X reconstruction was higher than that with the FBP method and the OSEM without PSF modeling method on the microPET R4. The non-uniformity with True X reconstruction was higher than that with FBP and OSEM without PSF modeling on microPET R4. SOR with True X reconstruction was better than that with FBP or OSEM without PSF modeling on the microPET R4. This study assessed the performance of the True X reconstruction. Spatial resolution with True X reconstruction was improved by 45 % and its % contrast was significantly improved compared to those with the conventional OSEM without PSF modeling reconstruction algorithm. The noise level was higher than that with the other reconstruction algorithm. Therefore, True X reconstruction should be used with caution when quantifying PET data.

Adrenocorticotropin-dependent precocious puberty of testicular origin in a boy with X-linked adrenal hypoplasia congenita due to a novel mutation in the DAX1 gene.

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Domenice, S; Latronico, A C; Brito, V N; Arnhold, I J; Kok, F; Mendonca, B B

2001-09-01

Primary adrenal insufficiency is a rare condition in pediatric age, and its association with precocious sexual development is very uncommon. We report a 2-yr-old Brazilian boy with DAX1 gene mutation whose first clinical manifestation was isosexual gonadotropin-independent precocious puberty. He presented with pubic hair, enlarged penis and testes, and advanced bone age. T levels were elevated, whereas basal and GnRH-stimulated LH levels were compatible with a prepubertal pattern. Chronic GnRH agonist therapy did not reduce T levels, supporting the diagnosis of gonadotropin-independent precocious puberty. Testotoxicosis was ruled out after normal sequencing of exon 11 of the LH receptor gene. At age 3 yr he developed clinical and hormonal features of severe primary adrenal insufficiency. The entire coding region of the DAX1 gene was analyzed through direct sequencing. A nucleotide G insertion between nucleotides 430 and 431 in exon 1, resulting in a novel frameshift mutation and a premature stop codon at position 71 of DAX-1, was identified. Surprisingly, steroid replacement therapy induced a clear decrease in testicular size and T levels to the prepubertal range. These findings suggest that chronic excessive ACTH levels resulting from adrenal insufficiency may stimulate Leydig cells and lead to gonadotropin-independent precocious puberty in some boys with DAX1 gene mutations.

Influence of GnRH analogue on body mass index in girls with precocious puberty: a prospective study

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Heshmat Moaieri

2014-06-01

Conclusion: Gonadotropin-releasing hormone analog (GnRHa therapy in central precocious puberty (CPP is safe for BMI and increasing of BMI is not significant, long- term follow-up study is required to elucidate whether GnRHa treatment affects adult obesity. Using growth hormone concomitantly, the effect on increasing height is significant.

Energetics of growth in semi-precocial shorebird chicks in a warm environment : The African black oystercatcher, Haematopus moquini

NARCIS (Netherlands)

Tjorve, Kathleen M. C.; Underhill, Les G.; Visser, G. Henk

2007-01-01

We studied prefledging growth, energy expenditure and time budgets of African Black Oystercatcher, Haematopus moquini, chicks on Robben Island, Western Cape, South Africa. The aim of the study was to investigate the effect of parental feeding on the growth and energetics of semi-precocial shorebird

Aetiology of idiopathic granulomatous mastitis.

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

2014-12-16

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

Advanced bone age as an indicator facilitates the diagnosis of precocious puberty

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Yue-Qin Xu

Full Text Available Abstract Objective: Diagnosis of central precocious puberty has always been challenging in clinical practice. As an important method in the diagnosis of central precocious puberty, luteinizing hormone-releasing hormone stimulation test is complex and time-consuming. In many cases, clinical traits are inconsistent with luteinizing hormone-releasing hormone stimulation test results, therefore not reliable for diagnosis. In this study, the authors intended to find an indicator that predicts the results of the luteinizing hormone-releasing hormone stimulation test among subjects with early pubertal signs. Methods: Cases of 382 girls with early breast development before 8 years old and luteinizing hormone-releasing hormone stimulation test before 9 years old were included and underwent follow-up tests. Patients with peak luteinizing hormone level ≥5 IU/L were considered positive in the luteinizing hormone-releasing hormone stimulation test. Anthropometric data, body mass index, bone age evaluation, blood hormones levels of luteinizing hormone, estradiol, follicle-stimulating hormone, and uterine and ovarian volumes were analyzed. Results: Subjects with positive results in the initial test demonstrated early bone maturation, accelerated growth, and elevated basal blood luteinizing hormone, estradiol, and follicle-stimulating hormone levels, when compared with subjects with negative results in the initial test. Subjects with positive results in the follow-up test presented a more advanced bone age and more accelerated linear growth, when compared with subjects with negative results in the follow-up test. Conclusions: According to the statistical analysis, advanced bone age is the most effective predictor of the result of luteinizing hormone-releasing hormone stimulation test.

The Diagnostic Value of Pelvic Ultrasound in Girls with Central Precocious Puberty.

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Lee, Sang Heon; Joo, Eun Young; Lee, Ji-Eun; Jun, Yong-Hoon; Kim, Mi-Young

2016-01-01

The gonadotropin-releasing hormone (GnRH) stimulation test is the gold standard for differentiating central precocious puberty (CPP) from exaggerated thelarche (ET). Because of this test's limitations, previous studies have clarified the clinical and laboratory factors that predict CPP. The present study investigated the early diagnostic significance of pelvic ultrasound in girls with CPP. The GnRH stimulation test and pelvic ultrasound were performed between March 2007 and February 2015 in 192 girls (aged values in pelvic ultrasound for differentiating between CPP and ET. Pelvic ultrasound should be combined with clinical and laboratory tests to maximize its diagnostic value for CPP.

Idiopathic pulmonary fibrosis: treatment update.

LENUS (Irish Health Repository)

O'Connell, Oisin J

2011-11-01

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

RNAi-mediated knockdown of SPOOK reduces ecdysteroid titers and causes precocious metamorphosis in the desert locust Schistocerca gregaria.

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Sugahara, Ryohei; Tanaka, Seiji; Shiotsuki, Takahiro

2017-09-01

The Halloween gene SPOOK (SPO) is involved in the production of the active metabolite of ecdysteroid, 20-hydroxyecdysone (20E), in insects. A previous study showed that RNAi-mediated knockdown of SPO in Schistocerca gregaria last instar nymphs markedly reduced the hemolymph 20E titer, but did not affect metamorphosis. In the present study, the effects of SPO interference on development were re-examined in this locust. Injections of SPO double-stranded RNA (dsSPO) into nymphs at mid and late instars significantly delayed nymphal development and interfered with molting. The 20E levels of dsSPO-treated nymphs were generally low, with a delayed, small peak, suggesting that disturbance of the 20E levels caused the above developmental abnormalities. A small proportion of the dsSPO-injected nymphs metamorphosed precociously, producing adults and adultoids. Precocious adults were characterized by small body size, short wings with abbreviated venation, and normal reproductive activity. Fourth instar nymphs that precociously metamorphosed at the following instar exhibited temporal expression patterns of ecdysone-induced protein 93F and the juvenile hormone (JH) early-inducible gene Krüppel homolog 1 similar to those observed at the last instar in normal nymphs. Adultoids displayed mating behavior and adultoid females developed eggs, but never laid eggs. JH injection around the expected time of the 20E peak in the dsSPO-injected nymphs completely inhibited the appearance of adultoids, suggesting that appearance of adultoids might be due to a reduced titer of JH rather than of 20E. These results suggest that SPO plays an important role in controlling morphogenesis, metamorphosis, and reproduction in S. gregaria. Copyright © 2017 Elsevier Inc. All rights reserved.

A boy with Prader-Willi syndrome: unmasking precocious puberty during growth hormone replacement therapy.

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Ludwig, Natasha G; Radaeli, Rafael F; Silva, Mariana M X; Romero, Camila M; Carrilho, Alexandre J F; Bessa, Danielle; Macedo, Delanie B; Oliveira, Maria L; Latronico, Ana Claudia; Mazzuco, Tânia L

2016-01-01

Prader-Willi syndrome (PWS) is a genetic disorder frequently characterized by obesity, growth hormone deficiency, genital abnormalities, and hypogonadotropic hypogonadism. Incomplete or delayed pubertal development as well as premature adrenarche are usually found in PWS, whereas central precocious puberty (CPP) is very rare. This study aimed to report the clinical and biochemical follow-up of a PWS boy with CPP and to discuss the management of pubertal growth. By the age of 6, he had obesity, short stature, and many clinical criteria of PWS diagnosis, which was confirmed by DNA methylation test. Therapy with recombinant human growth hormone (rhGH) replacement (0.15 IU/kg/day) was started. Later, he presented psychomotor agitation, aggressive behavior, and increased testicular volume. Laboratory analyses were consistent with the diagnosis of CPP (gonadorelin-stimulated LH peak 15.8 IU/L, testosterone 54.7 ng/dL). The patient was then treated with gonadotropin-releasing hormone analog (GnRHa). Hypothalamic dysfunctions have been implicated in hormonal disturbances related to pubertal development, but no morphologic abnormalities were detected in the present case. Additional methylation analysis (MS-MLPA) of the chromosome 15q11 locus confirmed PWS diagnosis. We presented the fifth case of CPP in a genetically-confirmed PWS male. Combined therapy with GnRHa and rhGH may be beneficial in this rare condition of precocious pubertal development in PWS.

Retrospective panoramic radiographic analysis for idiopathic osteosclerosis in Indians

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Srikanth H Srivathsa

2016-01-01

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

[Idiopathic facial paralysis in children].

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

2015-05-01

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

Adolescent Violent Victimization and Precocious Union Formation.

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C Kuhl, Danielle; Warner, David F; Wilczak, Andrew

2012-11-01

This article bridges scholarship in criminology and family sociology by extending arguments about "precocious exits" from adolescence to consider early union formation as a salient outcome of violent victimization for youths. Research indicates that early union formation is associated with several negative outcomes; yet the absence of attention to union formation as a consequence of violent victimization is noteworthy. We address this gap by drawing on life course theory and data from the National Longitudinal Study of Adolescent Health (Add Health) to examine the effect of violent victimization ("street" violence) on the timing of first co-residential union formation-differentiating between marriage and cohabitation-in young adulthood. Estimates from Cox proportional hazard models show that adolescent victims of street violence experience higher rates of first union formation, especially marriage, early in the transition to adulthood; however, this effect declines with age, as such unions become more normative. Importantly, the effect of violent victimization on first union timing is robust to controls for nonviolent delinquency, substance abuse, and violent perpetration. We conclude by discussing directions for future research on the association between violent victimization and coresidential unions with an eye toward the implications of such early union formation for desistance.

Total fertilization failure and idiopathic subfertility

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Goverde Angelique J

2009-01-01

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

Drug therapy for chronic idiopathic axonal polyneuropathy

NARCIS (Netherlands)

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

2004-01-01

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

Serum insulin-like growth factor I (IGF-I) and IGF-binding protein 3 levels are increased in central precocious puberty

DEFF Research Database (Denmark)

Juul, A; Scheike, Thomas Harder; Nielsen, C T

1995-01-01

between IGF-I and IGFBP-3 (i.e. free biologically active IGF-I) declined concomitantly with a decrease in growth velocity. Serum levels of IGF-I and IGFBP-3 (expressed as the SD score for bone age), but not those of estradiol, correlated with height velocity before and during treatment (r = 0.34; P ...Central precocious puberty (CPP) is characterized by early activation of the pituitary-gonadal axis, which leads to increased growth velocity and development of secondary sexual characteristics. It is generally believed that gonadal sex steroids stimulate pulsatile GH secretion, which, in turn......, stimulates insulin-like growth factor I (IGF-I) and IGF-binding protein 3 (IGFBP-3) production. However, little is known about GH, IGF-I, and IGFBP-3 serum levels in children with precocious puberty. Treatment of CPP by GnRH agonists has become the treatment of choice. However, the effect of long term...

Adolescent Idiopathic Scoliosis

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

Quantitative electromyographic characteristics of idiopathic unilateral vocal fold paralysis.

Science.gov (United States)

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

2016-11-01

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

Aneurisma idiopático de artéria radial: relato de caso Idiopathic radial artery aneurysm: case report

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Luiz Ernani Meira Jr.

2011-12-01

Full Text Available Os aneurismas da artéria radial são extremamente raros. Em sua maioria, consistem de pseudoaneurismas pós-traumáticos. Os aneurismas da artéria radial verdadeiros podem ser idiopáticos, congênitos, pós-estenóticos ou associados a patologias, tais como vasculites e doenças do tecido conjuntivo. Foi relatado um caso de aneurisma idiopático de artéria radial em uma criança de três anos, que, após completa investigação diagnóstica complementar, foi submetida à ressecção cirúrgica.Radial artery aneurysms are extremely rare. Post-traumatic pseudoaneurysms are the vast majority. True radial artery aneurysms can be idiopathic, congenital, poststenotic, or associated with some pathologies, such as vasculitis and conjunctive tissue diseases. We report a case of an idiopathic aneurysm of the radial artery in a three-year-old child who was submitted to surgical resection after a complete diagnostic approach.

Concordance Rates of Adolescent Idiopathic Scoliosis in a Danish Twin Population

DEFF Research Database (Denmark)

Simony, Ane; Carreon, Leah Y; Højmark, Karen

2016-01-01

STUDY DESIGN: Clinical, radiological and genetic determination of zygosity of twin pairs from the Danish Twin Registry who self-reported having Adolescent Idiopathic Scoliosis (AIS). OBJECTIVE: To establish concordance rates of AIS. SUMMARY OF BACKGROUND DATA: The aetiology of and the true mode...... reported. METHODS: All 46,418 twins registered in the Danish Twin Registry born from 1931 to 1982 were sent a survey, which included questions about scoliosis. The survey was returned by 34,944 individuals (75.3%) representing 23,204 pairs. From this study, 548 individuals representing 274 complete twin...... pairs where at least one twin self-reported having scoliosis were invited to a clinical and radiological examination. Zygosity was established by genetic testing. RESULTS: 182 individuals (33.2%) of the original cohort agreed to participate, 128 of whom had scoliosis by self-report. There were 91 twin...

Syphilis mimicking idiopathic intracranial hypertension

DEFF Research Database (Denmark)

Yri, Hanne; Wegener, Marianne; Jensen, Rigmor

2011-01-01

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

Idiopathic Gingival Fibromatosis: Case Report and Its Management

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Prashant P. Jaju

2009-01-01

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

Adolescent Violent Victimization and Precocious Union Formation*

Science.gov (United States)

C. Kuhl, Danielle; Warner, David F.; Wilczak, Andrew

2013-01-01

This article bridges scholarship in criminology and family sociology by extending arguments about “precocious exits” from adolescence to consider early union formation as a salient outcome of violent victimization for youths. Research indicates that early union formation is associated with several negative outcomes; yet the absence of attention to union formation as a consequence of violent victimization is noteworthy. We address this gap by drawing on life course theory and data from the National Longitudinal Study of Adolescent Health (Add Health) to examine the effect of violent victimization (“street” violence) on the timing of first co-residential union formation—differentiating between marriage and cohabitation—in young adulthood. Estimates from Cox proportional hazard models show that adolescent victims of street violence experience higher rates of first union formation, especially marriage, early in the transition to adulthood; however, this effect declines with age, as such unions become more normative. Importantly, the effect of violent victimization on first union timing is robust to controls for nonviolent delinquency, substance abuse, and violent perpetration. We conclude by discussing directions for future research on the association between violent victimization and coresidential unions with an eye toward the implications of such early union formation for desistance. PMID:24431471

Intraspinal anomalies in early-onset idiopathic scoliosis.

Science.gov (United States)

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

2017-06-01

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

Idiopathic epileptic syndromes and cognition.

Science.gov (United States)

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

2006-01-01

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

Idiopathic granulomatous mastitis

International Nuclear Information System (INIS)

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

2009-01-01

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

Angiopoietin-2 polymorphism in women with idiopathic recurrent miscarriage.

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

2003-10-01

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

Acute exacerbation of idiopathic pulmonary fibrosis triggered by Aspergillus empyema

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Atsushi Suzuki

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

Boy with central precocious puberty probably due to a peripheral cause.

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Sahana, Pranab Kumar; Gopal Sankar, Krishna Shankar; Sengupta, Nilanjan; Chattopadhyay, Kaustubh

2016-06-02

A 6½ years Indian boy was brought by his parents, who were anxious about the excessive increase in the size of the boy's phallus, from the age of 2 years. On physical examination, the child had a penis length greater than the 97th centile for age, a sexual maturity rating of gonads at stage 2 and pubic hair at stage 3, with height in the high normal range (90-97th centile). The bone age was 12 years. Laboratory evaluation showed pubertal levels of testosterone and pubertal gonadotropin response to stimulation, confirming central precocious puberty (CPP). Incidentally, the hormonal profile also suggested congenital adrenal hyperplasia (CAH). This case report depicts a case of CPP probably caused by CAH in boys, which is rare. 2016 BMJ Publishing Group Ltd.

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

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Yamada, S; Ishikawa, M; Yamamoto, K

2016-07-01

CSF volumes in the basal cistern and Sylvian fissure are increased in both idiopathic normal pressure hydrocephalus and Alzheimer disease, though the differences in these volumes in idiopathic normal pressure hydrocephalus and Alzheimer disease have not been well-described. Using CSF segmentation and volume quantification, we compared the distribution of CSF in idiopathic normal pressure hydrocephalus and Alzheimer disease. CSF volumes were extracted from T2-weighted 3D spin-echo sequences on 3T MR imaging and quantified semi-automatically. We compared the volumes and ratios of the ventricles and subarachnoid spaces after classification in 30 patients diagnosed with idiopathic normal pressure hydrocephalus, 10 with concurrent idiopathic normal pressure hydrocephalus and Alzheimer disease, 18 with Alzheimer disease, and 26 control subjects 60 years of age or older. Brain to ventricle ratios at the anterior and posterior commissure levels and 3D volumetric convexity cistern to ventricle ratios were useful indices for the differential diagnosis of idiopathic normal pressure hydrocephalus or idiopathic normal pressure hydrocephalus with Alzheimer disease from Alzheimer disease, similar to the z-Evans index and callosal angle. The most distinctive characteristics of the CSF distribution in idiopathic normal pressure hydrocephalus were small convexity subarachnoid spaces and the large volume of the basal cistern and Sylvian fissure. The distribution of the subarachnoid spaces in the idiopathic normal pressure hydrocephalus with Alzheimer disease group was the most deformed among these 3 groups, though the mean ventricular volume of the idiopathic normal pressure hydrocephalus with Alzheimer disease group was intermediate between that of the idiopathic normal pressure hydrocephalus and Alzheimer disease groups. The z-axial expansion of the lateral ventricle and compression of the brain just above the ventricle were the common findings in the parameters for differentiating

Intestinal Volvulus in Idiopathic Steatorrhea

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

1963-01-01

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

Idiopathic epiretinal membrane

NARCIS (Netherlands)

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

2014-01-01

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

Experience with the once-yearly histrelin (GnRHa subcutaneous implant in the treatment of central precocious puberty

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Katherine A Lewis

2008-12-01

Full Text Available Katherine A Lewis, Erica A EugsterDepartment of Pediatrics, Indiana University School of Medicine, Riley Hospital for Children, Indianapolis, USAAbstract: In 2007, a hydrogel histrelin implant was approved for the treatment of children with central precocious puberty (CPP. Children with CPP commonly have reduced height potential due to premature closure of the epiphyseal growth plates from exposure to sex steroids. Gonadotropin-releasing hormone analog (GnRHa treatment halts puberty and allows for improvement of adult height. A hydrogel implant delivery system utilizing the potent GnRHa, histrelin, was first developed for use in men with prostate cancer. A once yearly histrelin subcutaneous implant was subsequently developed for the treatment of children with CPP. Studies to date have demonstrated safety, tolerability, and effectiveness of this treatment option in patients treated up to 2 years. The most common adverse effects of the implant relate to implant site pain or bruising. Cost of this treatment seems comparable to somewhat higher than the commonly used GnRHa treatment option, depot leuprolide. While long term studies are needed to establish continued efficacy and safety beyond 2 years of treatment, the histrelin implant appears to be an attractive option for GnRHa treatment in patients with CPP.Keywords: central precocious puberty, histrelin, implant, gonadotropin-releasing-hormone analogs

Idiopathic scoliosis; a biomechanical and functional anatomical study.

NARCIS (Netherlands)

Veldhuizen, Albert Gerrit

1985-01-01

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

Pathology of idiopathic non-cirrhotic portal hypertension.

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

2018-04-12

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

Patellofemoral morphometry in patients with idiopathic patellofemoral pain syndrome

International Nuclear Information System (INIS)

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

2010-01-01

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

Idiopathic pulmonary fibrosis.

Science.gov (United States)

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

2017-02-23

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

MR imaging of the pituitary gland in central precocious puberty

International Nuclear Information System (INIS)

Kao, S.C.S.; Cook, J.S.; Hansen, J.R.; Simonson, T.M.

1992-01-01

Cranial magnetic resonance imaging was performed in 17 children with central precocious puberty (CPP) and 19 aged-matched controls to compare the appearance of the pituitary gland. Gland size was measured on T1-weighted sagittal and coronal images. The gland was graded according to the concavity or convexity of the upper surface, and the signal intensity of the gland was assessed visually. The mean pituitary volume in 13 CPP children without hypothalamic tumor (292.6 mm 3 ) was significantly greater than that in normal controls (181.35 mm 3 ). The mean volume for the four CPP children with hypothalamic tumor was smaller (145.0 mm 3 ). Compared to controls, the upper pituitary surface in CPP patients appeared convex in a higher proportion. The anterior pituitary was isointense to pons in all patients and controls. Although the posterior pituitary bright spot was present in 14 controls and 11 CPP patients, none with hypothalamic tumor showed it. (orig.)

MR imaging of the pituitary gland in central precocious puberty

Energy Technology Data Exchange (ETDEWEB)

Kao, S.C.S. (Dept. of Radiology, Univ. of Iowa Coll. of Medicine, Iowa City, IA (United States)); Cook, J.S. (Dept. of Pediatrics, Univ. of Iowa Coll. of Medicine, Iowa City, IA (United States)); Hansen, J.R. (Dept. of Pediatrics, Univ. of Iowa Coll. of Medicine, Iowa City, IA (United States)); Simonson, T.M. (Dept. of Radiology, Univ. of Iowa Coll. of Medicine, Iowa City, IA (United States))

1992-11-01

Cranial magnetic resonance imaging was performed in 17 children with central precocious puberty (CPP) and 19 aged-matched controls to compare the appearance of the pituitary gland. Gland size was measured on T1-weighted sagittal and coronal images. The gland was graded according to the concavity or convexity of the upper surface, and the signal intensity of the gland was assessed visually. The mean pituitary volume in 13 CPP children without hypothalamic tumor (292.6 mm[sup 3]) was significantly greater than that in normal controls (181.35 mm[sup 3]). The mean volume for the four CPP children with hypothalamic tumor was smaller (145.0 mm[sup 3]). Compared to controls, the upper pituitary surface in CPP patients appeared convex in a higher proportion. The anterior pituitary was isointense to pons in all patients and controls. Although the posterior pituitary bright spot was present in 14 controls and 11 CPP patients, none with hypothalamic tumor showed it. (orig.)

Evidence for cognitive vestibular integration impairment in idiopathic scoliosis patients

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Mercier Pierre

2009-08-01

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

Activity cycles and foraging behaviors of free-ranging sidewinder rattlesnakes (Crotalus cerastes): the ontogeny of hunting in a precocial vertebrate.

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Clark, Rulon W; Dorr, Scott W; Whitford, Malachi D; Freymiller, Grace A; Putman, Breanna J

2016-06-01

Predators often employ a complex series of behaviors to overcome antipredator defenses and effectively capture prey. Although hunting behaviors can improve with age and experience, many precocial species are necessarily effective predators from birth. Additionally, many predators experience innate ontogenetic shifts in predatory strategies as they grow, allowing them to adapt to prey more appropriate for their increased size and energetic needs. Understanding how the relative roles of innate age-specific adaptation and learning have evolved requires information on how predation behavior develops in situ, in free-ranging predators. However, most of the research on the ontogeny of predation behavior is based on laboratory studies of captive animals, largely due to the difficulty of following newborn individuals in nature. Here, we take advantage of the unique tracks left by juveniles of a precocial viperid, the sidewinder rattlesnake (Crotalus cerastes), which we used to follow free-ranging snakes in the field. We recorded details of their ambush hunting behavior, and compared the behaviors of these juveniles to adult snakes that we monitored in the field via radio telemetry. Although juvenile and adult behaviors were similar in most respects, we did find that adults chose more effective ambush sites, which may be due to their increased experience. We also found that juveniles (but typically not adults) perform periodic tail undulations while in ambush, and that juveniles displayed slightly different activity cycles. Both of these latter differences are likely the result of age-specific adaptations for juveniles' greater reliance on lizards versus small mammals as prey. We also compared the general predatory behavior of sidewinders to that of other species in the genus Crotalus. These findings will provide important baseline field information for more detailed empirical research on the ontogeny of predation behavior in precocial vertebrates. Copyright © 2016

Optimal management of idiopathic scoliosis in adolescence

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

2013-01-01

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

Mutational Analysis of TAC3 and TACR3 Genes in Patients with Idiopathic Central Pubertal Disorders

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Tusset, Cintia; Noel, Sekoni D.; Trarbach, Ericka B.; Silveira, Letícia F. G.; Jorge, Alexander A. L.; Brito, Vinicius N.; Cukier, Priscila; Seminara, Stephanie B.; de Mendonça, Berenice B.; Kaiser, Ursula B.; Latronico, Ana Claudia

2013-01-01

Aim To investigate the presence of variants in the TAC3 and TACR3 genes, which encode NKB and its receptor (NK3R), respectively, in a large cohort of patients with idiopathic central pubertal disorders. Patients and Methods Two hundred and thirty seven patients were studied: 114 with central precocious puberty (CPP), 73 with normosmic isolated hypogonadotropic hypogonadism (IHH) and 50 with constitutional delay of growth and puberty (CDGP). The control group consisted of 150 Brazilian individuals with normal pubertal development. Genomic DNA was extracted from peripheral blood and the entire coding region of both TAC3 and TACR3 genes were amplified and automatically sequenced. Results We identified one variant (p.A63P) in NKB and four variants, p.G18D, p.L58L (c.172C>T), p.W275* and p.A449S in NK3R, which were absent in the control group. The p.A63P variant was identified in a girl with CPP, and p.A449S in a girl with CDGP. The known p.G18D, p.L58L and p.W275* variants were identified in three unrelated males with normosmic IHH. Conclusion Rare variants in the TAC3 and TACR3 genes were identified in patients with central pubertal disorders. Loss-of-function variants of TACR3 were associated with the normosmic IHH phenotype. PMID:23329188

Is idiopathic recurrent pancreatitis attributed to small stones?

OpenAIRE

Chow, Wai-Keung; Peng, Yen-Chun

2013-01-01

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

Treatment of the idiopathic scoliosis with brace and physiotherapy.

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

2009-01-01

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

Economic impact of low dose polyethylene glycol 3350 plus electrolytes compared with lactulose in the management of idiopathic constipation in the UK.

Science.gov (United States)

Christie, Angela H; Culbert, Pearl; Guest, Julian F

2002-01-01

To estimate the economic impact of using low dose polyethyene glycol 3350 (PEG 3350) plus electrolytes (PEG+E) compared with lactulose in the treatment of idiopathic constipation in ambulant patients. DESIGN AND PERSPECTIVE: This was a decision analytic modelling study performed from the perspective of the UK's National Health Service (NHS). The clinical outcomes from a previously reported single-blind, randomised, multicentre trial were used as the clinical basis for the analysis. These data were combined with resource utilisation estimates derived from a panel of six general practitioners (GPs) and four nurses enabling a decision model to be constructed depicting the management of idiopathic constipation with either PEG+E or lactulose over 3 months. The model was used to estimate the expected 3-monthly NHS cost of using either laxative to manage idiopathic constipation. The expected 3-monthly NHS cost of using PEG+E or lactulose to manage idiopathic constipation was estimated to be 85 pound sterling and 96 pound sterling per patient, respectively (1999/2000 values). However, significantly more patients were successfully treated with PEG+E than lactulose (53% versus 24%; p PEG+E- and lactulose-treated patients, accounting for 56% (2.9 visits) and 73% (4.4 visits), respectively, of the expected NHS cost per patient at 3 months. Among PEG+E-treated patients, the acquisition cost of PEG+E was the secondary cost driver, accounting for 30% of the expected NHS cost per patient at 3 months, whereas the acquisition cost of lactulose accounted for only 11% of the expected NHS cost per lactulose-treated patient. District nurse domiciliary visits accounted for 4% and thyroid function tests for 2%. The costs of switched laxatives, concomitant laxatives, and gastroenterologist and colorectal surgeon visits collectively accounted for up to 9% of the total. The true cost of managing idiopathic constipation is impacted on by a broad range of resources and not only laxative

PATTERN OF INHERITANCE OF IDIOPATHIC HYPERCALCIURIA IN TWO FAMILIES

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A. Nickavar

2006-09-01

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

Patellofemoral morphometry in patients with idiopathic patellofemoral pain syndrome

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

2010-07-15

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

Guidelines for the medical treatment of idiopathic pulmonary fibrosis.

Science.gov (United States)

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

2017-05-01

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

Family history of idiopathic REM behavior disorder

DEFF Research Database (Denmark)

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

2013-01-01

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

Luteinizing hormone-releasing hormone analogue (Buserelin) treatment for central precocious puberty: a multi-centre trial.

Science.gov (United States)

Werther, G A; Warne, G L; Ennis, G; Gold, H; Silink, M; Cowell, C T; Quigley, C; Howard, N; Antony, G; Byrne, G C

1990-02-01

A multi-centre open trial of Buserelin, a luteinizing hormone-releasing hormone (LHRH) analogue, was conducted in 13 children with central precocious puberty. Eleven children (eight girls and three boys), aged 3.4-10.2 years at commencement, completed the required 12 month period of treatment. Initially all patients received the drug by intranasal spray in a dose of 1200 micrograms/day, but by the end of the 12 month period two were having daily subcutaneous injections and three were receiving an increased dose intranasally. The first month of treatment was associated in one boy with increased aggression and masturbation, and in the girls with an increase in the prevalence of vaginal bleeding. Thereafter, however, both behavioural abnormalities and menstruation were suppressed. Median bone age increased significantly during the study, but without any significant change in the ratio of height age to bone age. The median predicted adult height for the group therefore did not alter significantly over the twelve months of the study. Buserelin treatment caused a reduction in the peak luteinizing hormone and follicle-stimulating hormone (FSH) responses to LHRH, mostly to prepubertal levels, and also suppressed basal FSH. In the first weeks of treatment, the girls' serum oestradiol levels rose significantly and then fell to prepubertal or early pubertal levels. A similar pattern was seen for serum testosterone levels. Serum somatomedin-C levels, however, showed little fluctuation over the course of the study. Buserelin treatment was safe and well accepted, and offers the promise of improved linear growth potential in precocious puberty.

Computed tomography in the assessment of idiopathic spontaneous pneumothorax

International Nuclear Information System (INIS)

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

1991-01-01

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

Idiopathic scrotal elephantiasis.

Science.gov (United States)

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

2005-02-01

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

Understanding idiopathic intracranial hypertension

DEFF Research Database (Denmark)

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

2016-01-01

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

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

Science.gov (United States)

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

2009-01-01

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

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

African Journals Online (AJOL)

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

True Detective

OpenAIRE

Gajhede, Andreas; Westmark, [No Value; Bantoulidis, Georgios

2016-01-01

This paper examines the HBO’s television-series True Detective, a 2014 crime drama, attempting to point out the central elements of detective-fiction, to be found in its narrative. True Detective narrative is a heavy text with a number of references from various well-known, non-adjacent texts within the field of weird fiction, horror fiction and detective fiction. Therefore, the case study of this research will focus on narratological and literary analysis, in order to discover the predominan...

Optimal management of idiopathic scoliosis in adolescence

Directory of Open Access Journals (Sweden)

Kotwicki T

2013-07-01

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

Precocious development of self-awareness in dolphins.

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Rachel Morrison

Full Text Available Mirror-self recognition (MSR is a behavioral indicator of self-awareness in young children and only a few other species, including the great apes, dolphins, elephants and magpies. The emergence of self-awareness in children typically occurs during the second year and has been correlated with sensorimotor development and growing social and self-awareness. Comparative studies of MSR in chimpanzees report that the onset of this ability occurs between 2 years 4 months and 3 years 9 months of age. Studies of wild and captive bottlenose dolphins (Tursiops truncatus have reported precocious sensorimotor and social awareness during the first weeks of life, but no comparative MSR research has been conducted with this species. We exposed two young bottlenose dolphins to an underwater mirror and analyzed video recordings of their behavioral responses over a 3-year period. Here we report that both dolphins exhibited MSR, indicated by self-directed behavior at the mirror, at ages earlier than generally reported for children and at ages much earlier than reported for chimpanzees. The early onset of MSR in young dolphins occurs in parallel with their advanced sensorimotor development, complex and reciprocal social interactions, and growing social awareness. Both dolphins passed subsequent mark tests at ages comparable with children. Thus, our findings indicate that dolphins exhibit self-awareness at a mirror at a younger age than previously reported for children or other species tested.

Idiopathic short stature

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Vlaški Jovan

2013-01-01

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

Perceived health status in self-reported adolescent idiopathic scoliosis

DEFF Research Database (Denmark)

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

2010-01-01

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

Clinical and Polysomnographic Comparison between Narcolepsy without Cataplexy and Idiopathic Hypersomnia

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Tae Won Kim

2012-10-01

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

Very true operators on MTL-algebras

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Wang Jun Tao

2016-01-01

Full Text Available The main goal of this paper is to investigate very true MTL-algebras and prove the completeness of the very true MTL-logic. In this paper, the concept of very true operators on MTL-algebras is introduced and some related properties are investigated. Also, conditions for an MTL-algebra to be an MV-algebra and a Gödel algebra are given via this operator. Moreover, very true filters on very true MTL-algebras are studied. In particular, subdirectly irreducible very true MTL-algebras are characterized and an analogous of representation theorem for very true MTL-algebras is proved. Then, the left and right stabilizers of very true MTL-algebras are introduced and some related properties are given. As applications of stabilizer of very true MTL-algebras, we produce a basis for a topology on very true MTL-algebras and show that the generated topology by this basis is Baire, connected, locally connected and separable. Finally, the corresponding logic very true MTL-logic is constructed and the soundness and completeness of this logic are proved based on very true MTL-algebras.

Leuprolide Acetate 1-Month Depot for Central Precocious Puberty: Hormonal Suppression and Recovery

Directory of Open Access Journals (Sweden)

Neely EKirk

2011-01-01

Full Text Available Methods. This prospective US multicenter trial of leuprolide acetate 1-month depot (7.5–15 mg for central precocious puberty utilized an open-label treatment period, long-term follow-up, and adult callback. Forty-nine females Results. Subjects were treated for years. Mean peak GnRH-stimulated LH and FSH were prepubertal after the first dose and remained suppressed throughout treatment. During treatment, mean estradiol decreased to the limit of detection and mean testosterone decreased but remained above prepubertal norms. During posttreatment follow-up ( years, all patients achieved a pubertal hormonal response within 1 year and menses were reported in all females ≥12 years old. No impairment of reproductive function was observed at adulthood (mean age: 24.8 years.

Pulmonary tuberculosis in patients with idiopathic pulmonary fibrosis

Energy Technology Data Exchange (ETDEWEB)

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

2004-11-01

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

Pulmonary tuberculosis in patients with idiopathic pulmonary fibrosis

International Nuclear Information System (INIS)

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

2004-01-01

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

Hypercalcemia in idiopathic myelofibrosis

DEFF Research Database (Denmark)

Voss, A; Schmidt, K; Hasselbalch, H

1992-01-01

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

Repressive coping and alexithymia in idiopathic environmental intolerance

DEFF Research Database (Denmark)

Skovbjerg, Sine; Zachariae, Robert; Rasmussen, Alice

2010-01-01

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

Sleepiness in Idiopathic REM Sleep Behavior Disorder and Parkinson Disease.

Science.gov (United States)

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

2015-10-01

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

Obesity is a significant susceptibility factor for idiopathic AA amyloidosis.

Science.gov (United States)

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

2018-03-01

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

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

African Journals Online (AJOL)

2015-12-03

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

Allelic variation of the FRMD7 gene in congenital idiopathic nystagmus.

Science.gov (United States)

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

2007-09-01

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

Psychological assessment of mothers and their daughters at the time of diagnosis of precocious puberty.

Science.gov (United States)

Schoelwer, Melissa J; Donahue, Kelly L; Bryk, Kristina; Didrick, Paula; Berenbaum, Sheri A; Eugster, Erica A

2015-01-01

Concerns about psychological distress are often used to justify treatment of girls with precocious puberty, but there is little evidence to support these concerns. The extent to which psychological problems are associated with central precocious puberty (CPP) compared with other forms of early puberty in girls has likewise not been established. Girls presenting with untreated CPP, premature adrenarche (PA) or early normal puberty (ENP) were recruited from our pediatric endocrine clinic along with their mothers. Child psychological adjustment was assessed by child self-report and parent report. Parent self-reported personality, anxiety, and depression were also assessed. Differences between groups were explored using one-way ANOVA and Dunnett's T3 test. Sixty-two subjects (aged 7.5 ± 1.4 years, range 4.8-10.5) were enrolled, of whom 19 had CPP, 22 had PA, and 21 had ENP. Girls with ENP were significantly older (8.9 ± .9 years) than girls with CPP (6.9 ± 1.1 years, p psychological adjustment. However, mothers of girls with PA scored significantly higher than mothers of girls with ENP on one measure of depression (p = .04) and stress (p = .01). While mothers of girls with PA report increased psychological distress on some measures, no differences in psychological adjustment were found at baseline amongst the girls themselves. Whether these results will change as puberty progresses in the PA and ENP groups or with treatment of CPP is unknown. Long-term prospective studies are needed in order to further investigate psychological correlates of early puberty in girls.

Idiopathic Scoliosis

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Jens Ivar Brox

2014-07-01

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

Multicenter clinical trial of leuprolide acetate depot (Luphere depot 3.75 mg for efficacy and safety in girls with central precocious puberty

Directory of Open Access Journals (Sweden)

You Jin Kim

2013-12-01

Full Text Available PurposeWe evaluated the efficacy, safety and psychological aspect of monthly administrations of the gonadotropin-releasing hormone agonists (GnRHa, leuprolide acetate depot (Luphere depot 3.75 mg, in patients with precocious puberty.MethodsA total of 54 girls with central precocious puberty were administered with leuprolide acetate (Luphere depot 3.75 mg every four weeks over 24 weeks. We evaluated the percentage of children exhibiting a suppressed luteinizing hormone (LH response to GnRH (LH peak≤3 IU/L, peak LH/follicle stimulating hormone (FSH ratio of GnRH stimulation test less than 1, change in bone age/chronologic age ratio, change in the Tanner stage and change in eating habit and psychological aspect.Results(1 The percentage of children exhibiting a suppressed LH response to GnRH, defined as an LH peak≤3 IU/L at 24 weeks was 96.3 % (52/54. (2 The percentage of children exhibiting peak LH/FSH ratio<1 at 24 weeks of the study was 94.4 % (51/54. (3 The ratio of bone age and chronological age significantly declined from 1.27±0.07 to 1.24±0.01 after the 6 months of the study. (4 The mean Tanner stage manifested a significant change 2.3±0.48 at baseline, down to 1.70±0.61 at 24 weeks. (5 Based on the questionnaires, the score for eating habits showed a significant change from the baseline 34.0±6.8 to 31.3±6.8. (6 The psychological assessment did not exhibit a significant difference except with scores for sociability, problem behavior total score and other problems.ConclusionThe leuprolide 3.75 mg (Luphere depot is useful and safety for treating children with central precocious puberty.

RNAi-mediated silencing of vitellogenin gene function turns honeybee ( Apis mellifera) workers into extremely precocious foragers

Science.gov (United States)

Marco Antonio, David Santos; Guidugli-Lazzarini, Karina Rosa; Do Nascimento, Adriana Mendes; Simões, Zilá Luz Paulino; Hartfelder, Klaus

2008-10-01

The switch from within-hive activities to foraging behavior is a major transition in the life cycle of a honeybee ( Apis mellifera) worker. A prominent regulatory role in this switch has long been attributed to juvenile hormone (JH), but recent evidence also points to the yolk precursor protein vitellogenin as a major player in behavioral development. In the present study, we injected vitellogenin double-stranded RNA (dsVg) into newly emerged worker bees of Africanized genetic origin and introduced them together with controls into observation hives to record flight behavior. RNA interference-mediated silencing of vitellogenin gene function shifted the onset of long-duration flights (>10 min) to earlier in life (by 3 4 days) when compared with sham and untreated control bees. In fact, dsVg bees were observed conducting such flights extremely precociously, when only 3 days old. Short-duration flights (<10 min), which bees usually perform for orientation and cleaning, were not affected. Additionally, we found that the JH titer in dsVg bees collected after 7 days was not significantly different from the controls. The finding that depletion of the vitellogenin titer can drive young bees to become extremely precocious foragers could imply that vitellogenin is the primary switch signal. At this young age, downregulation of vitellogenin gene activity apparently had little effect on the JH titer. As this unexpected finding stands in contrast with previous results on the vitellogenin/JH interaction at a later age, when bees normally become foragers, we propose a three-step sequence in the constellation of physiological parameters underlying behavioral development.

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

International Nuclear Information System (INIS)

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

2002-01-01

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

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

African Journals Online (AJOL)

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

MUSCLE DISORDERS IN ADOLESCENT IDIOPATHIC SCOLIOSIS: LITERATURE REVIEW

OpenAIRE

Débora Pinheiro Lédio Alves; Barbara de Araújo

2016-01-01

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

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

Directory of Open Access Journals (Sweden)

Mihas Constantinos

2006-05-01

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

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

LENUS (Irish Health Repository)

Loveman, Emma

2015-01-01

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

Pulmonary epithelial permeability in normal subjects and patients with idiopathic interstitial pneumonia

International Nuclear Information System (INIS)

Anazawa, Yoshiki; Isawa, Toyoharu; Teshima, Takeo; Miki, Makoto; Motomiya, Masakichi

1991-01-01

99m Tc-DTPA is a low molecular weight substance, which is believed to pass through the pulmonary epithelium when it is inhaled as an aerosol. We performed 99m Tc-DTPA inhalation studies in 10 nonsmoking normal subjects and 10 patients with biopsy proven idiopathic interstitial pneumonia prior to therapy. 99m Tc-DTPA aerosol was inhaled for 3 min with the subject in the supine position and radioactivity was measured anteriorly with a gamma camera and recorded on computer. Measurements were performed for 3 min with the subject inhaling aerosol and for the subsequent 30 min with the subject in the same position. Time activity curves from the five regions of interest (ROIs) including the entire left lung, the entire right lung, and the upper, middle and lower third of the right lung were separately fitted to a single exponential function for the initial 7 min following cessation of inhalation, and the respective clearance half life (t1/2) in min was calculated. Lung function data, arterial blood gas tensions and blood chemistry were also obtained for comparison with the t1/2 values. The t1/2 values were significantly smaller in all ROIs in patients with idiopathic interstitial pneumonia than in normal subjects, indicating an increased pulmonary epithelial permeability in these patients. There was no relationship between t1/2 and %DL co , %DL co /V A , PaO 2 , or LDH. Although the true pathophysiologic significance of t1/2 measured using 99m Tc-DTPA aerosol is still not known, we consider that this measurement may be an important indicator of nonrespiratory lung function, in particular the degree of alveolar epithelial damage. (author)

Idiopathic hemifacial spasm responsive to zonisamide: a case report.

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

2009-01-01

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

Pulmonary function in children with idiopathic scoliosis

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Tsiligiannis Theofanis

2012-03-01

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

Pediatric Idiopathic Intracranial Hypertension Presenting With Sensorineural Hearing Loss.

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

2015-12-01

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

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

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Allard Paul

2006-10-01

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

Idiopathic intracranial hypertension in pediatric patients

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Nada Jirásková

2008-11-01

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

Single photon emission computed tomography in children with idiopathic seizures

International Nuclear Information System (INIS)

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

1991-01-01

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

Idiopathic central diabetes Insipidus.

Science.gov (United States)

Grace, Mary; Balachandran, Venu; Menon, Sooraj

2011-10-01

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

Venous Thromboembolism and Risk of Idiopathic Interstitial Pneumonia A Nationwide Study

DEFF Research Database (Denmark)

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

2010-01-01

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

Acquirement of true stress-strain curve using true fracture strain obtained by tensile test and FE analysis

International Nuclear Information System (INIS)

Lee, Kyoung Yoon; Kim, Tae Hyung; Lee, Hyung Yil

2009-01-01

In this work, we predict a true fracture strain using load-displacement curves from tensile test and Finite Element Analysis (FEA), and suggest a method for acquiring true Stress-Strain (SS) curves by predicted fracture strain. We first derived the true SS curve up to necking point from load-displacement curve. As the beginning, the posterior necking part of true SS curve is linearly extrapolated with the slope at necking point. The whole SS curve is then adopted for FE simulation of tensile test. The Bridgman factor or suitable plate correction factors are applied to pre and post FEA. In the load-true strain curve from FEA, the true fracture strain is determined as the matching point to test fracture load. The determined true strain is validated by comparing with test fracture strain. Finally, we complete the true SS curve by combining the prior necking part and linear part, the latter of which connects necking and predicted fracture points.

Acquirement of true stress-strain curve using true fracture strain obtained by tensile test and FE analysis

Energy Technology Data Exchange (ETDEWEB)

Lee, Kyoung Yoon; Kim, Tae Hyung; Lee, Hyung Yil [Sogang University, Seoul (Korea, Republic of)

2009-07-01

In this work, we predict a true fracture strain using load-displacement curves from tensile test and Finite Element Analysis (FEA), and suggest a method for acquiring true Stress-Strain (SS) curves by predicted fracture strain. We first derived the true SS curve up to necking point from load-displacement curve. As the beginning, the posterior necking part of true SS curve is linearly extrapolated with the slope at necking point. The whole SS curve is then adopted for FE simulation of tensile test. The Bridgman factor or suitable plate correction factors are applied to pre and post FEA. In the load-true strain curve from FEA, the true fracture strain is determined as the matching point to test fracture load. The determined true strain is validated by comparing with test fracture strain. Finally, we complete the true SS curve by combining the prior necking part and linear part, the latter of which connects necking and predicted fracture points.

Acquirement of True Stress-strain Curve Using True Fracture Strain Obtained by Tensile Test and FE Analysis

Energy Technology Data Exchange (ETDEWEB)

Lee, Kyoung Yoon; Lee, Hyung Yil [Sogang University, Seoul (Korea, Republic of); Kim, Tae Hyung [Korea Atomic Energy Research Institute, Daejeon (Korea, Republic of)

2009-10-15

In this work, we predict a true fracture strain using load-displacement curves from tensile test and finite element analysis (FEA), and suggest a method for acquiring true stress-strain (SS) curves by predicted fracture strain. We first derived the true SS curve up to necking point from load-displacement curve. As the beginning, the posterior necking part of true SS curve is linearly extrapolated with the slope at necking point. The whole SS curve is then adopted for FE simulation of tensile test. The Bridgman factor or suitable plate correction factors are applied to pre and post FEA. In the load-true strain curve from FEA, the true fracture strain is determined as the matching point to test fracture load. The determined true strain is validated by comparing with test fracture strain. Finally, we complete the true SS curve by combining the prior necking part and linear part, the latter of which connects necking and predicted fracture points.

Aromatase inhibitors in men: effects and therapeutic options

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de Jong Frank H

2011-06-01

Full Text Available Abstract Aromatase inhibitors effectively delay epiphysial maturation in boys and improve testosterone levels in adult men Therefore, aromatase inhibitors may be used to increase adult height in boys with gonadotropin-independent precocious puberty, idiopathic short stature and constitutional delay of puberty. Long-term efficacy and safety of the use of aromatase inhibitors has not yet been established in males, however, and their routine use is therefore not yet recommended.

Idiopathic thoracic transdural intravertebral spinal cord herniation

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Mazda K Turel

2017-01-01

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

Idiopathic facial pain related with dental implantation

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Tae-Geon Kwon

2016-06-01

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

Idiopathic megarectum in children.

Science.gov (United States)

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

2001-02-01

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

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

NARCIS (Netherlands)

Schlösser, T.P.C.

2014-01-01

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

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

NARCIS (Netherlands)

Janssen, M.M.A.

2011-01-01

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

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

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Diego Rey

2015-02-01

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

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

Science.gov (United States)

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

2015-01-01

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

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

Science.gov (United States)

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

2016-03-18

Oral anticoagulant therapy for more than 6 months in patients with an episode of idiopathic thromboembolic disease is controversial. The objective was to determine predictive clinical signs that identify patients at increased risk of thromboembolic recurrence after stopping anticoagulant therapy for 6 months after an episode of idiopathic deep vein thrombosis (DVT). A prospective study which included 306 consecutive patients with a first episode of idiopathic DVT from June 2012 to June 2014. Predictor variables of recurrent thromboembolic disease and episodes of recurrence during follow-up of the patients (28.42 months) were collected. We performed a multivariate analysis to analyze possible predictors (Pthrombus (P=.001) in males, and persistence of residual thrombus in women (P=.046). The mean recurrence-free survival was shorter in both groups. The presence of echogenic thrombus in men and the existence of residual DVT in women were 2 clinical signs associated with increased risk of thromboembolic recurrence after stopping anticoagulant therapy for 6 months after an episode of idiopathic DVT in our study. Copyright © 2015 Elsevier España, S.L.U. All rights reserved.

Nasal bacterial colonization in cases of idiopathic epistaxis in children.

Science.gov (United States)

Kamble, Payal; Saxena, Sonal; Kumar, Sunil

2015-11-01

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

Antioxidant therapy in idiopathic oligoasthenoteratozoospermia

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Ahmad Majzoub

2017-01-01

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

Prostate specific antigen in boys with precocious puberty before and during gonadal suppression by GnRH agonist treatment

DEFF Research Database (Denmark)

Juul, A; Müller, J; Skakkebaek, N E

1997-01-01

antigen (PSA) is a marker of the androgen-dependent prostatic epithelial cell activity and it is used in the diagnosis and surveillance of adult patients with prostatic cancer. We have measured PSA concentrations in serum from boys with precocious puberty before and during gonadal suppression with Gn......In healthy boys, the pituitary-gonadal axis exhibits diurnal variation in early puberty. Serum testosterone levels are higher during the night and low or immeasurable during the day. These fluctuating levels of circulating androgens in early pubertal boys are difficult to monitor. Prostate specific...

Idiopathic gingival fibromatosis

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Sujata Rath

2011-01-01

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

[Diagnostic value of baseline serum luteinizing hormone level for central precocious puberty in girls].

Science.gov (United States)

Ou-Yang, Li-Xue; Yang, Fan

2017-07-01

To evaluate the diagnostic value of baseline serum luteinizing hormone (LH) level for central precocious puberty (CPP) in girls. A total of 279 girls with precocious puberty were subjected to assessment of growth and development, bone age determination, baseline LH test, and follicle-stimulating hormone (FSH) test, gonadotropin-releasing hormone stimulation test, and other related examinations. Of the 279 patients, 175 were diagnosed with CPP and 104 with premature thelarche (PT). The receiver operating characteristic (ROC) curve was used to evaluate the diagnostic value of baseline LH and FSH levels and their peak levels for CPP, and the correlation between the baseline LH level and the peak LH level was analyzed. The CPP group had significantly higher bone age, baseline LH and FSH levels, peak LH and FSH levels, and ratio of peak LH level to peak FSH level than the PT group (Pbaseline LH level and peak LH level had good diagnostic values for CPP. Among the three bone age subgroups in the CPP group (7.0-9.0 years, 9.0-11.0 years, and >11.0 years), baseline LH level showed the best diagnostic value in the >11.0 years subgroup, with the largest area under the ROC curve. At a baseline LH level of 0.45 IU/L, the Youden index reached the peak value, and the sensitivity and specificity were 66.7% and 80% respectively, for the diagnosis of CPP. At a peak LH level of 9.935 IU/L, the Youden index reached the peak value, and the sensitivity and specificity were 74.8% and 100% respectively, for the diagnosis of CPP. The baseline LH level was positively correlated with the peak LH level (r=0.440, PBaseline LH level can be used as an primary screening index for the diagnosis of CPP. It has a certain diagnostic value for CPP at different bone ages, and may be used as a monitoring index during the treatment and follow-uP.

Epidemiology of idiopathic pulmonary fibrosis

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Ley B

2013-11-01

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

Longitudinal follow-up of bone density and body composition in children with precocious or early puberty before, during and after cessation of GnRH agonist therapy

NARCIS (Netherlands)

I.M. van der Sluis (Inge); A.M. Boot (Annemieke); E.P. Krenning (Eric); S.L.S. Drop (Stenvert); S.M.P.F. de Muinck Keizer-Schrama (Sabine)

2002-01-01

textabstractWe studied bone mineral density (BMD), bone metabolism, and body composition in 47 children with central precocious puberty (n = 36) or early puberty (n = 11) before, during, and after cessation of GnRH agonist. Bone density and body composition were measured with dual

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

Science.gov (United States)

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

2016-05-15

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

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

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

Science.gov (United States)

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

2018-04-01

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

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

OpenAIRE

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

2016-01-01

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

Idiopathic incapacitates in infancy and childhood

International Nuclear Information System (INIS)

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

2003-01-01

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

[Primary glucocorticoid resistance syndrome presenting as pseudo-precocious puberty and galactorrhea].

Science.gov (United States)

Xiang, Shu-lin; He, Li-ping; Ran, Xing-wu; Tian, Hao-ming; Li, Xiu-jun; Liang, Jin-zhong

2008-09-01

Primary glucocorticoid resistance syndrome (PGRS) is a rare condition characterized by hypercortisolism without Cushing's syndrome. This report describes a 7-year-old boy of PGRS with pseudo-precocious puberty and galactorrhea as the main manifestation. His height was 135 cm and body weight was 31 kg. Pigmentation could be seen in the skin, mammary areola and penis. He had hirsutism, low hair line, coarse voice, Tanner stage 3 pubic hair, penis in adult form, accelerated linear growth, and advanced bone age (13 yr.), but normal (for age) testes. Furthermore, he had mammoplasia and galactorrhea. There were no features of glucocorticoid (GC) excess. Hepatic function was impaired (ALT 1426 IU/L, AST 611 IU/L) with no definite causes. Serum cortisol concentration was 1294 nmol/L, 777 nmol/L, 199.3 nmol/L at 8:00, 16:00 and 24:00 respectively. Plasma adrenocorticotropic hormone (ACTH) was normal or a little higher (43.9-80 ng/L). Urinary-free cortisol (UFC) was normal (55.5-62.4 microg/24 h). Serum estradiol (E2), progesterone (P), testosterone (T), luteinizing hormone (LH) and follicle-stimulating hormone (FSH) were normal. Serum dehydroepiandrosterone sulfate (DHEAS, 60 microg/dL) and serum prolactin (PRL, 58.7-183.9 ng/mL) level were high, urinary dehydroepiandrosterone (DHEA) level was also elevated (0.96-3.2 mg/mL). Gonadotrophin hormone-releasing hormone (GnRH) stimulation test was negative. Serum cortisol responded normally to insulin-induced hypoglycemia. However, serum cortisol and plasma ACTH concentration was suppressed to more than 50% by 0.5 mg dexamethasone (DEX). The diagnosis of PGRS was made. TREATMENT AND FOLLOW-UP: The patient received a treatment of 0.75-1.0 mg/d DEX. Because of galactorrhea, bromocriptine was given by 1.25-3.75 mg/d. After 24 months follow-up, the pigmentation was relieved and galactorrhea disappeared. No advanced development of the external genitalia and breast was found. The acceleration of the bone age was also slowed down. But

Paraneoplastic precocious puberty and excessively rapid somatic growth associated with pediatric malignant hepatic tumor: 1 report of 2 cases

Energy Technology Data Exchange (ETDEWEB)

Kim, Ok Hwa; Bahk, Yong Whee [Cathalic University College of Medicine, Seoul (Korea, Republic of)

1990-04-15

Sexual precocity in a 28-months-old boy and markedly accelerated skeletal growth with large body in a 5 years and 5 months-old-girl are reported. The former resulted from human chorionic gonadotropin-producing hepatoblastoma and the latter from cerebral gigantism associated with hepatoma. These two different disorders are discussed on the common basis of rare association of malignant hepatic tumors with precocious sexual and / or somatic growth. The clinical manifestations, chemical abnormalities, and the radiologic findings are presented with a brief review of the literature.

Paraneoplastic precocious puberty and excessively rapid somatic growth associated with pediatric malignant hepatic tumor: 1 report of 2 cases

International Nuclear Information System (INIS)

Kim, Ok Hwa; Bahk, Yong Whee

1990-01-01

Sexual precocity in a 28-months-old boy and markedly accelerated skeletal growth with large body in a 5 years and 5 months-old-girl are reported. The former resulted from human chorionic gonadotropin-producing hepatoblastoma and the latter from cerebral gigantism associated with hepatoma. These two different disorders are discussed on the common basis of rare association of malignant hepatic tumors with precocious sexual and / or somatic growth. The clinical manifestations, chemical abnormalities, and the radiologic findings are presented with a brief review of the literature

Genetics Home Reference: idiopathic inflammatory myopathy

Science.gov (United States)

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

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

Science.gov (United States)

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

2014-07-01

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

Epidemiological studies in idiopathic pulmonary fibrosis: pitfalls in methodologies and data interpretation

Directory of Open Access Journals (Sweden)

Antonella Caminati

2015-09-01

Full Text Available Data on incidence, prevalence and mortality of idiopathic pulmonary fibrosis (IPF are sparse and vary across studies. The true incidence and prevalence of the disease are unknown. In general, the overall prevalence and incidence reported in European and Asian countries are lower than those reported in American studies. In recent years, the epidemiological approach to IPF has been difficult for many reasons. First, the diagnostic criteria of the disease have changed over time. Secondly, the coding system used for IPF in administrative databases, the most common data source used to study this aspect of the disease, has been modified in the past few years. Finally, the study design, the methodology and the population selected in each of the studies are very different. All these aspects make comparisons among studies very difficult or impossible. In this review, we list the main issues that might arise when comparing different studies and that should be taken into consideration when describing the state of epidemiological knowledge concerning this pathology.

Giant scrotal elephantiasis: an idiopathic case.

Science.gov (United States)

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

2010-01-01

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

Increasing abscisic acid levels by immunomodulation in barley grains induces precocious maturation without changing grain composition.

Science.gov (United States)

Staroske, Nicole; Conrad, Udo; Kumlehn, Jochen; Hensel, Götz; Radchuk, Ruslana; Erban, Alexander; Kopka, Joachim; Weschke, Winfriede; Weber, Hans

2016-04-01

Abscisic acid (ABA) accumulates in seeds during the transition to the seed filling phase. ABA triggers seed maturation, storage activity, and stress signalling and tolerance. Immunomodulation was used to alter the ABA status in barley grains, with the resulting transgenic caryopses responding to the anti-ABA antibody gene expression with increased accumulation of ABA. Calculation of free versus antibody-bound ABA reveals large excess of free ABA, increasing signficantly in caryopses from 10 days after fertilization. Metabolite and transcript profiling in anti-ABA grains expose triggered and enhanced ABA-functions such as transcriptional up-regulation of sucrose-to-starch metabolism, storage protein synthesis and ABA-related signal transduction. Thus, enhanced ABA during transition phases induces precocious maturation but negatively interferes with growth and development. Anti-ABA grains display broad constitutive gene induction related to biotic and abiotic stresses. Most of these genes are ABA- and/or stress-inducible, including alcohol and aldehyde dehydrogenases, peroxidases, chaperones, glutathione-S-transferase, drought- and salt-inducible proteins. Conclusively, ABA immunomodulation results in precocious ABA accumulation that generates an integrated response of stress and maturation. Repression of ABA signalling, occurring in anti-ABA grains, potentially antagonizes effects caused by overshooting production. Finally, mature grain weight and composition are unchanged in anti-ABA plants, although germination is somewhat delayed. This indicates that anti-ABA caryopses induce specific mechanisms to desensitize ABA signalling efficiently, which finally yields mature grains with nearly unchanged dry weight and composition. Such compensation implicates the enormous physiological and metabolic flexibilities of barley grains to adjust effects of unnaturally high ABA amounts in order to ensure and maintain proper grain development. © The Author 2016. Published by

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

International Nuclear Information System (INIS)

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

2004-01-01

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

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

International Nuclear Information System (INIS)

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

1990-01-01

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

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

Energy Technology Data Exchange (ETDEWEB)

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

1990-05-01

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

A CASE OF IDIOPATHIC PULMONARY ARTERIAL HYPERTENSION IN MALE

OpenAIRE

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

2016-01-01

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

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

Science.gov (United States)

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

2013-03-01

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

Locomotor skills and balance strategies in adolescents idiopathic scoliosis.

Science.gov (United States)

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

2007-01-01

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

MUSCLE DISORDERS IN ADOLESCENT IDIOPATHIC SCOLIOSIS: LITERATURE REVIEW

Directory of Open Access Journals (Sweden)

Débora Pinheiro Lédio Alves

2016-03-01

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

Idiopathic unilateral vocal-fold paralysis in the adult.

Science.gov (United States)

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

2018-02-02

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

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

Science.gov (United States)

Yamada, Shigeki; Ishikawa, Masatsune; Yamamoto, Kazuo

2017-01-01

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

True bursal pigmented villonodular synovitis

International Nuclear Information System (INIS)

Abdelwahab, Ibrahim Fikry; Kenan, Samuel; Steiner, German C.; Abdul-Quader, Mohammed

2002-01-01

We describe two cases of pigmented villonodular synovitis affecting true bursae. This study was also designed to discuss the term ''pigmented villonodular bursitis'', not confined to true synovial bursae, sometimes creating misunderstanding. (orig.)

Alteration of placental haemostatic mechanisms in idiopathic intrauterine growth restriction

Directory of Open Access Journals (Sweden)

Jaime Eduardo Bernal Villegas

2012-08-01

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

Idiopathic pulmonary fibrosis: evolving concepts.

Science.gov (United States)

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

2014-08-01

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

Idiopathic intracranial hypertension

DEFF Research Database (Denmark)

Yri, Hanne M; Jensen, Rigmor H

2015-01-01

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

Cup Blocks the Precocious Activation of the Orb Autoregulatory Loop

Science.gov (United States)

Wong, Li Chin; Schedl, Paul

2011-01-01

Translational regulation of localized mRNAs is essential for patterning and axes determination in many organisms. In the Drosophila ovary, the germline-specific Orb protein mediates the translational activation of a variety of mRNAs localized in the oocyte. One of the Orb target mRNAs is orb itself, and this autoregulatory activity ensures that Orb proteins specifically accumulate in the developing oocyte. Orb is an RNA-binding protein and is a member of the cytoplasmic polyadenylation element binding (CPEB) protein family. We report here that Cup forms a complex in vivo with Orb. We also show that cup negatively regulates orb and is required to block the precocious activation of the orb positive autoregulatory loop. In cup mutant ovaries, high levels of Orb accumulate in the nurse cells, leading to what appears to be a failure in oocyte specification as a number of oocyte markers inappropriately accumulate in nurse cells. In addition, while orb mRNA is mislocalized and destabilized, a longer poly(A) tail is maintained than in wild type ovaries. Analysis of Orb phosphoisoforms reveals that loss of cup leads to the accumulation of hyperphosphorylated Orb, suggesting that an important function of cup in orb-dependent mRNA localization pathways is to impede Orb activation. PMID:22164257

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 ...

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

International Nuclear Information System (INIS)

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

2016-01-01

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

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

Energy Technology Data Exchange (ETDEWEB)

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

2016-06-15

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

Interventional Radiologic Treatment for Idiopathic Portal Hypertension

International Nuclear Information System (INIS)

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

1999-01-01

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

Neurodevelopment in preschool idiopathic toe-walkers.

Science.gov (United States)

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

2017-09-01

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

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

African Journals Online (AJOL)

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

True bursal pigmented villonodular synovitis

Energy Technology Data Exchange (ETDEWEB)

Abdelwahab, Ibrahim Fikry [Department of Radiology, New York Methodist Hospital, Affiliated with New York Hospital-Cornell Medical Center, Brooklyn, NY (United States); Kenan, Samuel [Department of Orthopedics, New York University Medical Center, NY (United States); Steiner, German C. [Department of Pathology, Hospital for Joint Diseases/Orthopedic Institute, New York, NY (United States); Abdul-Quader, Mohammed [Department of Radiology, New York Presbyterian Hospital, Columbia University, New York, NY (United States)

2002-06-01

We describe two cases of pigmented villonodular synovitis affecting true bursae. This study was also designed to discuss the term ''pigmented villonodular bursitis'', not confined to true synovial bursae, sometimes creating misunderstanding. (orig.)

Idiopathic noncirrhotic portal hypertension: current perspectives.

Science.gov (United States)

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

2016-01-01

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

Material Properties Test to Determine Ultimate Strain and True Stress-True Strain Curves for High Yield Steels

Energy Technology Data Exchange (ETDEWEB)

K.R. Arpin; T.F. Trimble

2003-04-01

This testing was undertaken to develop material true stress-true strain curves for elastic-plastic material behavior for use in performing transient analysis. Based on the conclusions of this test, the true stress-true strain curves derived herein are valid for use in elastic-plastic finite element analysis for structures fabricated from these materials. In addition, for the materials tested herein, the ultimate strain values are greater than those values cited as the limits for the elastic-plastic strain acceptance criteria for transient analysis.

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

OpenAIRE

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

2007-01-01

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

Pirfenidone treatment in idiopathic pulmonary fibrosis

DEFF Research Database (Denmark)

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

2016-01-01

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

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

NARCIS (Netherlands)

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

2012-01-01

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

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

Directory of Open Access Journals (Sweden)

Galina V. Pyatakova

2016-12-01

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

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

Directory of Open Access Journals (Sweden)

O. Yu. Konopel'ko

2013-01-01

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

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

DEFF Research Database (Denmark)

Lisse, I; Hasselbalch, H; Junker, P

1991-01-01

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

Atherosclerosis in Juvenile Idiopathic Arthritis

Directory of Open Access Journals (Sweden)

Ewa Jednacz

2012-01-01

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

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

Science.gov (United States)

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

2015-07-01

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

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

Science.gov (United States)

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

2016-01-01

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

Spring Chinook Salmon Interactions Indices and Residual/Precocious Male Monitoring in the Upper Yakima Basin; Yakima/Klickitat Fisheries Project Monitoring and Evaluation, 2004-2005 Annual Report.

Energy Technology Data Exchange (ETDEWEB)

Pearsons, Todd N.; Johnson, Christopher L. (Washington Department of Fish and Wildlife, Olympia, WA); James, Brenda B. (Cascade Aquatics, Ellensburg, WA)

2005-05-01

This report examines some of the factors that can influence the success of supplementation, which is currently being tested in the Yakima Basin using upper Yakima stock of spring chinook salmon. Supplementation success in the Yakima Basin is defined relative to four topic areas: natural production, genetics, ecological interactions, and harvest (Busack et al. 1997). The success of spring chinook salmon supplementation in the Yakima Basin is dependent, in part, upon fish culture practices and favorable physical and biological conditions in the natural environment (Busack et al. 1997; James et al. 1999; Pearsons et al., 2003; Pearsons et al. 2004). Shortfalls in either of these two topics (i.e., failure in culturing many fish that have high long-term fitness or environmental conditions that constrain spring chinook salmon production) will cause supplementation success to be limited. For example, inadvertent selection or propagation of spring chinook that residualize or precocially mature may hinder supplementation success. Spring chinook salmon that residualize (do not migrate during the normal migration period) may have lower survival rates than migrants and, additionally, may interact with wild fish and cause unacceptable impacts to non-target taxa. Large numbers of precocials (nonanadromous spawners) may increase competition for females and significantly skew ratios of offspring sired by nonanadromous males, which could result in more nonanadromous spring chinook in future generations. Conditions in the natural environment may also limit the success of spring chinook supplementation. For example, intra or interspecific competition may constrain spring chinook salmon production. Spring chinook salmon juveniles may compete with each other for food or space or compete with other species that have similar ecological requirements. Monitoring of spring chinook salmon residuals, precocials, prey abundance, carrying capacity, and competition will help researchers

'TrueCoinc' software utility for calculation of the true coincidence correction

International Nuclear Information System (INIS)

Sudar, S.

2002-01-01

The true coincidence correction plays an important role in the overall accuracy of the γ ray spectrometry especially in the case of present-day high volume detectors. The calculation of true coincidence corrections needs detailed nuclear structure information. Recently these data are available in computerized form from the Nuclear Data Centers through the Internet or on a CD-ROM of the Table of Isotopes. The aim has been to develop software for this calculation, using available databases for the levels data. The user has to supply only the parameters of the detector to be used. The new computer program runs under the Windows 95/98 operating system. In the framework of the project a new formula was prepared for calculating the summing out correction and calculation of the intensity of alias lines (sum peaks). The file converter for reading the ENDSF-2 type files was completed. Reading and converting the original ENDSF was added to the program. A computer accessible database of the X rays energies and intensities was created. The X ray emissions were taken in account in the 'summing out' calculation. Calculation of the true coincidence 'summing in' correction was done. The output was arranged to show independently two types of corrections and to calculate the final correction as multiplication of the two. A minimal intensity threshold can be set to show the final list only for the strongest lines. The calculation takes into account all the transitions, independently of the threshold. The program calculates the intensity of X rays (K, L lines). The true coincidence corrections for X rays were calculated. The intensities of the alias γ lines were calculated. (author)

A case of idiopathic omental hemorrhage

Directory of Open Access Journals (Sweden)

Toshimitsu Hosotani

2016-05-01

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

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

Science.gov (United States)

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

2014-04-30

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

DEHP exposure in utero disturbs sex determination and is potentially linked with precocious puberty in female mice

International Nuclear Information System (INIS)

Wang, Yongan; Yang, Qing; Liu, Wei; Yu, Mingxi; Zhang, Zhou; Cui, Xiaoyu

2016-01-01

Human's ubiquitous exposure to di (2-ethylhexyl) phthalate (DEHP) is thought to be associated with female reproductive toxicity. Previous studies found that DEHP inhibited follicle growth and decreased estradiol levels in adult female mice. However, limited information is available on the link between in utero DEHP exposure and ovarian development in female mouse offspring. The present study evaluates the disturbances in regulatory genes involved in female sex determination and the ovarian outcomes in fetal and postnatal female mice treated with in utero DEHP exposure. Pregnant mice were exposed to DEHP by gavage, with the dosage regime beginning at human relevant exposure levels. After in utero DEHP exposure, increased follicular atresia was observed in the female pups at postnatal days (PND) 21. Foxl2 expression was significantly upregulated, and Fst was significantly downregulated by DEHP above 2 mg/kg/d at PND 1 and 21. This suggests that lesion of granulosa cell differentiation and disturbance of follicle development in postnatal female mice. The expression of Cyp11a1 and Star were significantly downregulated by in utero DEHP exposure, indicating effects on estradiol biosynthesis. The female sex determination pathway was disturbed in fetus by DEHP at 2 mg/kg/d and above during the critical time window of sex determination causing significant upregulation of Foxl2, Wnt4, β-catenin and Fst. Furthermore, the increased expression of Wnt4 was supported by whole-mount in situ hybridization (WISH). These results suggest a possible association between in utero DEHP exposure and precocious puberty in the postnatal life of mice offspring, where disturbance of the sex determination regulating pathway acted as an important mechanism. - Highlights: • Maternal exposure to di (2-ethylhexyl) phthalate disturbs fetus sex determination. • DEHP upregulated Foxl2 expression potentially disturbs postnatal granulosa cell differentiation. • DEHP accelerated medulla

DEHP exposure in utero disturbs sex determination and is potentially linked with precocious puberty in female mice

Energy Technology Data Exchange (ETDEWEB)

Wang, Yongan [Key Laboratory of Industrial Ecology and Environmental Engineering (MOE), School of Environmental Science and Technology, Dalian University of Technology, Dalian, Liaoning 116024 (China); Yang, Qing [School of Life Science and Biotechnology, Dalian University of Technology, Dalian, Liaoning 116024 (China); Liu, Wei, E-mail: liu_wei@dlut.edu.cn [Key Laboratory of Industrial Ecology and Environmental Engineering (MOE), School of Environmental Science and Technology, Dalian University of Technology, Dalian, Liaoning 116024 (China); Yu, Mingxi; Zhang, Zhou; Cui, Xiaoyu [Key Laboratory of Industrial Ecology and Environmental Engineering (MOE), School of Environmental Science and Technology, Dalian University of Technology, Dalian, Liaoning 116024 (China)

2016-09-15

Human's ubiquitous exposure to di (2-ethylhexyl) phthalate (DEHP) is thought to be associated with female reproductive toxicity. Previous studies found that DEHP inhibited follicle growth and decreased estradiol levels in adult female mice. However, limited information is available on the link between in utero DEHP exposure and ovarian development in female mouse offspring. The present study evaluates the disturbances in regulatory genes involved in female sex determination and the ovarian outcomes in fetal and postnatal female mice treated with in utero DEHP exposure. Pregnant mice were exposed to DEHP by gavage, with the dosage regime beginning at human relevant exposure levels. After in utero DEHP exposure, increased follicular atresia was observed in the female pups at postnatal days (PND) 21. Foxl2 expression was significantly upregulated, and Fst was significantly downregulated by DEHP above 2 mg/kg/d at PND 1 and 21. This suggests that lesion of granulosa cell differentiation and disturbance of follicle development in postnatal female mice. The expression of Cyp11a1 and Star were significantly downregulated by in utero DEHP exposure, indicating effects on estradiol biosynthesis. The female sex determination pathway was disturbed in fetus by DEHP at 2 mg/kg/d and above during the critical time window of sex determination causing significant upregulation of Foxl2, Wnt4, β-catenin and Fst. Furthermore, the increased expression of Wnt4 was supported by whole-mount in situ hybridization (WISH). These results suggest a possible association between in utero DEHP exposure and precocious puberty in the postnatal life of mice offspring, where disturbance of the sex determination regulating pathway acted as an important mechanism. - Highlights: • Maternal exposure to di (2-ethylhexyl) phthalate disturbs fetus sex determination. • DEHP upregulated Foxl2 expression potentially disturbs postnatal granulosa cell differentiation. • DEHP accelerated medulla

Value judgments and the true self.

Science.gov (United States)

Newman, George E; Bloom, Paul; Knobe, Joshua

2014-02-01

The belief that individuals have a "true self" plays an important role in many areas of psychology as well as everyday life. The present studies demonstrate that people have a general tendency to conclude that the true self is fundamentally good--that is, that deep inside every individual, there is something motivating him or her to behave in ways that are virtuous. Study 1 finds that observers are more likely to see a person's true self reflected in behaviors they deem to be morally good than in behaviors they deem to be bad. Study 2 replicates this effect and demonstrates observers' own moral values influence what they judge to be another person's true self. Finally, Study 3 finds that this normative view of the true self is independent of the particular type of mental state (beliefs vs. feelings) that is seen as responsible for an agent's behavior.

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

Directory of Open Access Journals (Sweden)

Juliana Molina

2012-10-01

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

The CXC chemokine cCAF stimulates precocious deposition of ECM molecules by wound fibroblasts, accelerating development of granulation tissue

Directory of Open Access Journals (Sweden)

Li Qi-Jing

2002-06-01

Full Text Available Abstract Background During wound repair, fibroblasts orchestrate replacement of the provisional matrix formed during clotting with tenascin, cellular fibronectin and collagen III. These, in turn, are critical for migration of endothelial cells, keratinocytes and additional fibroblasts into the wound site. Fibroblasts are also important in the deposition of collagen I during scar formation. The CXC chemokine chicken Chemotactic and Angiogenic Factor (cCAF, is highly expressed by fibroblasts after wounding and during development of the granulation tissue, especially in areas where extracellular matrix (ECM is abundant. We hypothesized that cCAF stimulates fibroblasts to produce these matrix molecules. Results Here we show that this chemokine can stimulate precocious deposition of tenascin, fibronectin and collagen I, but not collagen III. Studies in culture and in vivo show that tenascin stimulation can also be achieved by the N-terminal 15 aas of the protein and occurs at the level of gene expression. In contrast, stimulation of fibronectin and collagen I both require the entire molecule and do not involve changes in gene expression. Fibronectin accumulation appears to be linked to tenascin production, and collagen I to decreased MMP-1 levels. In addition, cCAF is chemotactic for fibroblasts and accelerates their migration. Conclusions These previously unknown functions for chemokines suggest that cCAF, the chicken orthologue of human IL-8, enhances healing by rapidly chemoattracting fibroblasts into the wound site and stimulating them to produce ECM molecules, leading to precocious development of granulation tissue. This acceleration of the repair process may have important application to healing of impaired wounds.

Exercise therapy in juvenile idiopathic arthritis

NARCIS (Netherlands)

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

2008-01-01

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

CELSR2 is a candidate susceptibility gene in idiopathic scoliosis

DEFF Research Database (Denmark)

Einarsdottir, Elisabet; Grauers, Anna; Wang, Jingwen

2017-01-01

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

Acute Idiopathic Scrotal Edema

Directory of Open Access Journals (Sweden)

Micheál Breen

2013-01-01

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

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

Science.gov (United States)

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

2015-05-01

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

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

Directory of Open Access Journals (Sweden)

Yonghui Xia

2013-05-01

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

Glymphatic MRI in idiopathic normal pressure hydrocephalus.

Science.gov (United States)

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

2017-10-01

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

Idiopathic Scoliosis from Psychopathological and Mind-Body Medicine Perspectives.

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

2016-12-01

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

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

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

2001-02-01

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

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

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Francesco Zulian

2012-01-01

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

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

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Mahesh Jayachandran

2013-01-01

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

Comparison of learning ability and memory retention in altricial (Bengalese finch, Lonchura striata var. domestica) and precocial (blue-breasted quail, Coturnix chinensis) birds using a color discrimination task.

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Ueno, Aki; Suzuki, Kaoru

2014-02-01

The present study sought to assess the potential application of avian models with different developmental modes to studies on cognition and neuroscience. Six altricial Bengalese finches (Lonchura striata var. domestica), and eight precocial blue-breasted quails (Coturnix chinensis) were presented with color discrimination tasks to compare their respective faculties for learning and memory retention within the context of the two developmental modes. Tasks consisted of presenting birds with discriminative cues in the form of colored feeder lids, and birds were considered to have learned a task when 80% of their attempts at selecting the correctly colored lid in two consecutive blocks of 10 trials were successful. All of the finches successfully performed the required experimental tasks, whereas only half of the quails were able to execute the same tasks. In the learning test, finches required significantly fewer trials than quails to learn the task (finches: 13.5 ± 9.14 trials, quails: 45.8 ± 4.35 trials, P memory retention tests, which were conducted 45 days after the learning test, finches retained the ability to discriminate between colors correctly (95.0 ± 4.47%), whereas quails did not retain any memory of the experimental procedure and so could not be tested. These results suggested that altricial and precocial birds both possess the faculty for learning and retaining discrimination-type tasks, but that altricial birds perform better than precocial birds in both faculties. The present findings imply that developmental mode is an important consideration for assessing the suitability of bird species for particular experiments. © 2013 Japanese Society of Animal Science.

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

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

2017-09-01

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

Treatment of idiopathic FSGS with adrenocorticotropic hormone gel.

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

2013-12-01

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

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

NARCIS (Netherlands)

Kouwenhoven, J.W.M.

2007-01-01

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

[Biologic therapy in idiopathic inflammatory myopathy].

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

2014-09-15

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

Idiopathic Harlequin syndrome – case report

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Marcelina Grochowiec

2015-09-01

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

Time course of recovery of idiopathic vocal fold paralysis.

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

2018-01-01

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

Bilateral versus single lung transplant for idiopathic pulmonary fibrosis.

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

2014-10-01

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

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

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MR Etemadifar

2005-05-01

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

Growth factors in idiopathic pulmonary fibrosis: relative roles

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Allen Jeremy T

2001-11-01

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

Update on diagnosis and treatment of idiopathic pulmonary fibrosis

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

2015-01-01

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

Update on diagnosis and treatment of idiopathic pulmonary fibrosis

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José Baddini-Martinez

2015-10-01

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

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

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Imoto, S; Kitaya, T; Kodama, T; Hasebe, T; Mukai, K

1997-08-01

We report a case of idiopathic granulomatous mastitis in a 35-year-old Japanese woman, who came to our hospital complaining of a tender mass in her right breast. Because the results of initial aspiration cytology were considered highly suspicious for carcinoma, modified radical mastectomy was performed. However, the final histological diagnosis was granulomatous lobular mastitis with no evidence of malignancy. Idiopathic granulomatous mastitis is a rare inflammatory breast disease of unknown etiology. Since the clinical manifestations are similar to those of mammary carcinoma, this condition has been misdiagnosed as carcinoma and treated as such. A review of the literature revealed that idiopathic granulomatous mastitis has tended to occur in young patients with a history of childbirth or oral contraceptive usage. Clinical or imaging diagnosis has often been difficult. Complete resection or corticosteroid therapy can be recommended as the optimal treatment. Since 38% of patients experience recurrence, long-term follow-up is indicated.

Idiopathic Chronic Parotitis: Imaging Findings and Sialendoscopic Response.

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

2015-01-01

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

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

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

1994-12-15

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

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

International Nuclear Information System (INIS)

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

1994-01-01

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

Characterization of patients diagnosed with central precocious puberty starting treatment with leuprolide acetate, at the Hospital Nacional de Ninos in the period January 2004 to December 2009

International Nuclear Information System (INIS)

Viquez Viquez, Natalia

2013-01-01

Clinical characteristics and laboratory and cabinet findings (hormone levels, bone age, pelvic ultrasound) were determined in a sample of 39 patients diagnosed with central precocious puberty (CPP) who started treatment with leuprolide acetate (LPA). The cross-sectional observational study was developed at the Hospital Nacional de Ninos 'Dr. Carlos Saenz Herrera' during the period 2004 and 2009. The chronological age of patients with CPP was specified at the time of diagnosis and to start treatment with LPA. Epidemiological characteristics (sex, origin) were described in patients with CPP. Bone age with respect to chronological age was identified in patients with CPP. Sexual maturity of patients with CPP was established by the Tanner scale. The hormone levels of estrogen, testosterone, luteinizing hormone and follicle stimulating hormone were specified in patients with CPP. The etiology was determined in patients with CPP treated with LPA. The adverse effects of treatment with LPA were described. Anthropometric measurements were detailed. The heredo-familial antecedents of precocious puberty were established in patients with diagnosis of CPP. The size of the pelvic organs was evaluated by pelvic ultrasound [es

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

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Ameri E

2012-11-01

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

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

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Gür, Gözde; Ayhan, Cigdem; Yakut, Yavuz

2017-06-01

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

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

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Raman R, Thulasi; Manimaran, D

2016-01-01

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

Idiopathic multiple aneurysm of external carotid artery

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Saravanan Balachandran

2014-01-01

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

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

LENUS (Irish Health Repository)

2012-02-01

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

An 11-month-old girl with central precocious puberty caused by hypothalamic hamartoma

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Da Young Yoon

2016-12-01

Full Text Available Central precocious puberty (CPP is caused by premature activation of the hypothalamic-gonadal axis, and must be treated adequately. In particular, CPP that occurs at a relatively young age or in boys is likely to be caused by an organic lesion. Hypothalamic hamartoma (HH is the most common organic cause of CPP. The present case report describes an 11-month-old female infant who presented with vaginal bleeding and rapidly progressive secondary sex characteristics from the age of 6 months. She was diagnosed with CPP following the detection of HH via magnetic resonance imaging. The infant girl was successfully treated with gonadotropin-releasing hormone agonist. After 6 months, her breast had regressed and clinical and radiological follow-up demonstrated stable findings with no evidence of tumor growth or secondary sexual characteristics until the fourth year after the initiation of treatment. This patient is the one of the youngest infants presenting with CPP and HH in Korea; treatment was successful over a relatively long follow-up period.

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

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G. Raghu

2008-12-01

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

Factors that predict a positive response on gonadotropin-releasing hormone stimulation test for diagnosing central precocious puberty in girls

Directory of Open Access Journals (Sweden)

Junghwan Suh

2013-12-01

Full Text Available PurposeThe rapid increase in the incidence of precocious puberty in Korea has clinical and social significance. Gonadotropin-releasing hormone (GnRH stimulation test is required to diagnose central precocious puberty (CPP, however this test is expensive and time-consuming. This study aimed to identify factors that can predict a positive response to the GnRH stimulation test.MethodsClinical and laboratory parameters, including basal serum luteinizing hormone (LH, follicle-stimulating hormone (FSH, and estradiol (E2, were measured in 540 girls with clinical signs of CPP.ResultsTwo hundred twenty-nine of 540 girls with suspected CPP had a peak serum LH level higher than 5 IU/L (the CPP group. The CPP group had advanced bone age (P<0.001, accelerated yearly growth rate (P<0.001, increased basal levels of LH (P=0.02, FSH (P<0.001, E2 (P=0.001, and insulin-like growth factor-I levels (P<0.001 compared to the non-CPP group. In contrast, body weight (P<0.001 and body mass index (P<0.001 were lower in the CPP group. Although basal LH was significantly elevated in the CPP group compared to the non-CPP group, there was considerable overlap between the 2 groups. Cutoff values of basal LH (0.22 IU/L detected CPP with 87.8% sensitivity and 20.9% specificity.ConclusionNo single parameter can predict a positive response on the GnRH stimulation test with both high sensitivity and specificity. Therefore, multiple factors should be considered in evaluation of sexual precocity when deciding the timing of the GnRH stimulation test.

Treatment of Refractory Idiopathic Supraorbital Neuralgia Using Percutaneous Pulsed Radiofrequency.

Science.gov (United States)

Luo, Fang; Lu, Jingjing; Ji, Nan

2018-02-26

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

Intervention levels in a precocious detection program for breast cancer and evaluation of four participant units; Niveles de intervencion en un programa de deteccion precoz del cancer de mama y evaluacion de cuatro unidades participantes

Energy Technology Data Exchange (ETDEWEB)

Carrera M, F.; Velazquez M, S.; Manzano M, F.J.; Sanchez S, J. [Hospital `Juan Ramon Jimenez` Ronda Norte s/n 21005. Huelva, Espana (Spain)

1998-12-31

It is presented the basis to make a cost benefit analysis for a breast cancer precocious detection program and consequently the keys for its optimization from the radiological point of view. Taking this as a reference it is made an exhaustive quality control to four mammographic unities which were participating or they were candidates to participate in a breast cancer precocious detection program. Also it is presented its results. It is followed the protocol for quality control in mammography in Spain obtaining values for the measurement of twelve interesting parameters. It should be maintained the standard breast dose about 1 mGy/ image. It should be available a 24 x 30 cm portacassete and considering the utilization of a single projection by breast. (Author)

Spring Chinook Salmon Interactions Indices and Residual/Precocial Monitoring in the Upper Yakima Basin; Yakima/Klickitat Fisheries Project Monitoring and Evaluation, 2001-2002 Annual Report.

Energy Technology Data Exchange (ETDEWEB)

Pearsons, Todd N.; James, Brenda B.; Johnson, Christopher L. (Washington Department of Fish and Wildlife, Olympia, WA)

2003-05-01

This report examines some of the factors that can influence the success of supplementation, which is currently being tested in the Yakima Basin using upper Yakima stock of spring chinook salmon. Supplementation success in the Yakima Basin is defined relative to four topic areas: natural production, genetics, ecological interactions, and harvest (Busack et al. 1997). The success of spring chinook salmon supplementation in the Yakima Basin is dependent, in part, upon fish culture practices and favorable physical and biological conditions in the natural environment (Busack et al. 1997). Shortfalls in either of these two topics (i.e., failure in culturing many fish that have high long-term fitness or environmental conditions that constrain spring chinook salmon production) will cause supplementation success to be limited. For example, inadvertent selection or propagation of spring chinook that residualize or precocially mature may hinder supplementation success. Spring chinook salmon that residualize (do not migrate during the normal migration period) may have lower survival rates than migrants and, additionally, may interact with wild fish and cause unacceptable impacts to non-target taxa. Large numbers of precocials (nonanadromous spawners) may increase competition for females and significantly skew ratios of offspring sired by nonanadromous males, which could result in more nonanadromous spring chinook in future generations. Conditions in the natural environment may also limit the success of spring chinook supplementation. For example, intra or interspecific competition may constrain spring chinook salmon production. Spring chinook salmon juveniles may compete with each other for food or space or compete with other species that have similar ecological requirements. Monitoring of spring chinook salmon residuals, precocials, prey abundance, carrying capacity, and competition will help researchers interpret why supplementation is working or not working (Busack et al

Familial Idiopathic Cranial Neuropathy in a Chinese Family.

Science.gov (United States)

Zhang, Li; Liang, Jianfeng; Yu, Yanbing

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

Idiopathic brain herniation. A report of two paediatric cases.

Science.gov (United States)

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

2014-10-01

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

Idiopathic pulmonary fibrosis misdiagnosed as sputum-negative pulmonary tuberculosis.

Science.gov (United States)

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

2016-01-01

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

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

Science.gov (United States)

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

2013-11-01

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

Idiopathic fibrosing pancreatitis: A cause of obstructive jaundice in childhood

International Nuclear Information System (INIS)

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

1988-01-01

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

Idiopathic fibrosing pancreatitis: A cause of obstructive jaundice in childhood

Energy Technology Data Exchange (ETDEWEB)

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

1988-01-01

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

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

DEFF Research Database (Denmark)

Heiligenhaus, Arnd; Foeldvari, Ivan; Edelsten, Clive

2012-01-01

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

Thrombotic thrombocytopenic purpura (TTP or Moschowitz syndrome: a true hematologic emergency

Directory of Open Access Journals (Sweden)

Deborah Melis

2012-01-01

Full Text Available Introduction: Thrombotic thrombocytopenic purpura (TTP is a thrombotic microangiopathy caused by congenital or inherited disorders involving the processing of the ultra-large forms of von Willebrand factor. As a result, platelet-rich microthrombi form in the small arterial vessels of various organs, particularly those of the brain, heart, and kidneys. The idiopathic autoimmune form of TTP is the most common. There are various subgroups of acquired TTP associated with HIV infection, sepsis, pregnancy, autoimmune disease, various disseminated malignancies, and drugs. If not promptly treated, TTP is associated with high mortality, making it a true medical emergency. Materials and methods: The article is based on a review of the literature published between January and October of 2009. Its aim is to clarify the diagnosis, treatment, and follow-up of TTP. Results: Diagnostic criteria include the presence of microangiopathic hemolytic anemia associated with thrombocytopenia in the absence of other obvious causes. Assays of ADAMTS13 activity and titration of acquired antibodies against this enzyme are indicated in the follow-up of disease and as prognostic indicators. Treatment centers around daily plasma exchange associated with immunosuppressant drug therapy, particularly steroids and more recently the monoclonal anti-CD20 antibody rituximab. Discussion: Despite improved treatment, TTP is still associated with significant mortality (10—20%, particularly when plasma exchange is initiated late. Relapse also occurs in a substantial proportion of patients (10—40% although the frequency of this outcome may be reduced by rituximab therapy.

Precocious Puberty or Premature Thelarche: Analysis of a Large Patient Series in a Single Tertiary Center with Special Emphasis on 6- to 8-Year-Old Girls

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Tero Varimo

2017-08-01

Full Text Available IntroductionWe describe the etiology, MRI findings, and growth patterns in girls who had presented with signs of precocious puberty (PP, i.e., premature breast development or early menarche. Special attention was paid to the diagnostic findings in 6- to 8-year-olds.Materials and methodsWe reviewed the medical records of 149 girls (aged 0.7–10.3 years who had been evaluated for PP in the Helsinki University Hospital between 2001 and 2014.ResultsIn 6- to 8-year-old girls, PP was most frequently caused by idiopathic gonadotropin-releasing hormone (GnRH-dependent PP (60% and premature thelarche (PT; 39%. The former subgroup grew faster (8.7 ± 2.0 cm/year, n = 58 than the girls with PT (7.0 ± 1.1 cm/year, n = 32 (P < 0.001, and the best discrimination for GnRH-dependent PP was achieved with a growth velocity cut-off value of 7.0 cm/year (sensitivity 92% and specificity 58% [area under the curve 0.82, 95% confidence interval (CI 0.73–0.91, P < 0.001]. Among asymptomatic and previously healthy 6- to 8-year-old girls with GnRH-dependent PP, one (1.7%, 95% CI 0.3–9.7% had a pathological brain MRI finding requiring surgical intervention (craniopharyngioma. In girls younger than 3 years, the most frequent cause of breast development was PT, and, in 3- to 6-year-olds, GnRH-dependent PP.ConclusionIn 6- to 8-year-old girls, analysis of growth velocity is helpful in differentiating between PT and GnRH-dependent PP. Although the frequency of clinically relevant intracranial findings in previously healthy, asymptomatic 6- to 8-year-old girls was low, they can present without any signs or symptoms, which favors routine MRI imaging also in this age group.

Idiopathic Hypoparathyroidism Mimicking Ankylosing Spondylitis: A Case Report

Directory of Open Access Journals (Sweden)

Ozge Illeez Memetoglu

2016-01-01

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

Idiopathic non-cirrhotic portal hypertension: a review

NARCIS (Netherlands)

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

2015-01-01

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

Diffuse idiopathic skeletal hyperostosis in ancient clergymen.

NARCIS (Netherlands)

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

2007-01-01

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

Diffuse idiopathic skeletal hyperostosis in ancient clergymen

NARCIS (Netherlands)

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

2007-01-01

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

Drug therapy for chronic idiopathic axonal polyneuropathy

NARCIS (Netherlands)

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

2017-01-01

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

Drug therapy for chronic idiopathic axonal polyneuropathy

NARCIS (Netherlands)

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

2017-01-01

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

Acute Lymphoblastic Leukaemia presenting as Juvenile Idiopathic ...

African Journals Online (AJOL)

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

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

International Nuclear Information System (INIS)

Nagano, Tatsuya; Kotani, Yoshikazu; Fujii, Osamu

2012-01-01

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

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

Energy Technology Data Exchange (ETDEWEB)

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

2016-09-15

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

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

International Nuclear Information System (INIS)

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

2016-01-01

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

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.

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.

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

Science.gov (United States)

LeBauer, Aaron; Brtalik, Robert; Stowe, Katherine

2008-10-01

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

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

International Nuclear Information System (INIS)

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

2008-01-01

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

Idiopathic Noncirrhotic Portal Hypertension: An Appraisal

Science.gov (United States)

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

2016-01-01

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

A case of idiopathic portalhypertension

International Nuclear Information System (INIS)

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

1982-01-01

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

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...

Syphilis mimicking idiopathic intracranial hypertension

DEFF Research Database (Denmark)

Yri, Hanne; Wegener, Marianne; Jensen, Rigmor

2011-01-01

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

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

Directory of Open Access Journals (Sweden)

Byung Sun Kim

2013-12-01

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

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

Science.gov (United States)

Marimuthu, Vidhya; Krishnamurthy, Sriram; Rajappa, Medha

2017-11-15

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

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

Science.gov (United States)

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

2014-01-01

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

Idiopathic Thrombocytopenic Purpura Misdiagnosed as Hereditary Angioedema

DEFF Research Database (Denmark)

Andersen, Michelle Fog; Bygum, Anette

2015-01-01

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

Histological characteristics of diffuse idiopathic skeletal hyperostosis

NARCIS (Netherlands)

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

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

High-dose acetylcysteine in idiopathic pulmonary fibrosis

NARCIS (Netherlands)

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

2005-01-01

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

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

Science.gov (United States)

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

2016-01-01

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

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

International Nuclear Information System (INIS)

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

2003-01-01

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

Idiopathic trigeminal neuropathy in a poodle

Directory of Open Access Journals (Sweden)

Carlos Eduardo Aparicio

2010-12-01

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

Contemporary Management of Idiopathic Laryngotracheal Stenosis.

Science.gov (United States)

Donahoe, Laura; Keshavjee, Shaf

2018-05-01

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

Measurement of true density

International Nuclear Information System (INIS)

Carr-Brion, K.G.; Keen, E.F.

1982-01-01

System for determining the true density of a fluent mixture such as a liquid slurry, containing entrained gas, such as air comprises a restriction in pipe through which at least a part of the mixture is passed. Density measuring means such as gamma-ray detectors and source measure the apparent density of the mixture before and after its passage through the restriction. Solid-state pressure measuring devices are arranged to measure the pressure in the mixture before and after its passage through the restriction. Calculating means, such as a programmed microprocessor, determine the true density from these measurements using relationships given in the description. (author)

Clinical characteristics and outcomes of familial and idiopathic ...

African Journals Online (AJOL)

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

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

African Journals Online (AJOL)

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

Successful treatment of idiopathic pulmonary capillaritis with intravenous cyclophosphamide.

LENUS (Irish Health Repository)

Flanagan, Frances

2013-03-01

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

MRI findings in acute idiopathic transverse myelopathy in children

International Nuclear Information System (INIS)

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

2003-01-01

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

Chronic idiopathic intestinal pseudo-obstruction in an English bulldog.

Science.gov (United States)

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

2001-05-01

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

Idiopathic intracranial hypertension and transverse sinus stenoses

DEFF Research Database (Denmark)

Skyrman, Simon; Fytagoridis, Anders; Andresen, Morten

2013-01-01

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

Biological agents in polyarticular juvenile idiopathic arthritis

DEFF Research Database (Denmark)

Amarilyo, Gil; Tarp, Simon; Foeldvari, Ivan

2016-01-01

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

Adolescent idiopathic scoliosis: Indications and efficacy of nonoperative treatment

Directory of Open Access Journals (Sweden)

Federico Canavese

2011-01-01

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

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

International Nuclear Information System (INIS)

Wang Guohong; Xu Ruiji; Zhang Zhongshu; Wang Youji

2005-01-01

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

Serum inhibin A and inhibin B in central precocious puberty before and during treatment with GnRH agonists

DEFF Research Database (Denmark)

Sehested, A; Andersson, A M; Müller, J

2000-01-01

both gonadotropins and estradiol levels become suppressed. We therefore investigated serum levels of inhibin A and inhibin B in girls with CPP at diagnosis and during treatment in order to test the hypothesis that inhibin secretion would increase and decrease in parallel with the activation......Serum levels of the gonadal hormones inhibin A and inhibin B are undetectable or low in prepubertal girls, and rise during puberty. In girls with central precocious puberty (CPP) the hypothalamic-pituitary-gonadal axis is prematurely activated, if the girl is thereafter treated with GnRH agonists...... and suppression of the hypothalamic-pituitary-gonadal axis. Serum levels of inhibin A and inhibin B were significantly (p 0.0005) elevated in 42 girls at diagnosis of CPP (inhibin A: 7 pg/ml (...

GENETIC PREDICTORS OF IDIOPATHIC SICK SINUS SYNDROME

Directory of Open Access Journals (Sweden)

A. A. Chernova

2012-01-01

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

Towards a new taxonomy of idiopathic orofacial pain.

Science.gov (United States)

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

2005-08-01

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

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

DEFF Research Database (Denmark)

Gideon, P; Sørensen, P S; Thomsen, C

1995-01-01

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

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

Directory of Open Access Journals (Sweden)

Moore Peter

2010-02-01

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

The True Scholar.

Science.gov (United States)

Bellah, Robert N.

2000-01-01

This essay argues for ethical inquiry as the essence of true scholarship. Individual sections address: pure reason versus ethics, the current "age of money" in the university, rational choice theory, and the fatal flaw in rational choice theory that all human actions cannot be explained by it. (DB)

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

Science.gov (United States)

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

2011-01-01

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

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

Science.gov (United States)

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

2017-01-01

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

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

Science.gov (United States)

Shiu, Jr-Rung; Chao, Hsun-Chin; Luo, Chih-Cheng; Lai, Ming-Wei; Kong, Man-Shan; Chen, Shih-Yen; Chen, Chien-Chang; Wang, Chao-Jan

2010-08-01

There are no available data for outcomes in children's idiopathic superior mesenteric artery syndrome (SMAS) strictly treated conservatively. The aim of the study was to evaluate clinical and nutritional outcome in children with idiopathic SMAS. A 1-year prospective observation study of effects of treatment and outcome was performed in 27 children (8 boys, 19 girls) with idiopathic SMAS who underwent an upper gastrointestinal (UGI) series, ultrasound measurement of the aortomesenteric angle, treatment, clinical assessment, growth evaluation, and regular clinical visits for more than 12 months. Mean age of the patients was 11.77 +/- 2.15 years. The major clinical complaints were postprandial pain or fullness (88.9%), vomiting (55.6%), and early satiety (51.9%). Eight patients (29.6%) had weight loss. The UGI series revealed typical features of SMAS. The aortomesenteric angle on ultrasound was 10 degrees to 19 degrees. The height of most patients (92.6%) was above the 10th percentile, whereas 15 (55.6%) patients weighed below the 10th percentile. Six patients underwent surgical intervention (3 for obstruction and 3 for persistent anorexia with weight loss), and their clinical symptoms and weight status improved steadily during the follow-up months. Among the 21 patients not subject to surgical intervention, 11 (52.4%) experienced a reduction of symptoms >50% after 3 months of treatment, and weight-for-age percentile increased significantly after 6 months of treatment. Overall, a significant increase in the weight-for-age status was seen in the patients with surgical treatment or with medication only after 6 and 12 months of treatment. An aortomesenteric angle children with idiopathic SMAS. A duodenojejunostomy can effectively relieve the obstructive symptoms, such as anorexia, and improve nutritional status, whereas long-term medical treatment may aid in relieving the clinical symptoms, promoting appetite, and improving nutritional status in pediatric patients with

Precision Medicine: The New Frontier in Idiopathic Pulmonary Fibrosis.

Science.gov (United States)

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

2016-06-01

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

An unusual case of idiopathic multiple invasive cervical resorption

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Sathya Prakash Reddy Kesary

2014-01-01

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

The efficacy of flutamide, an antiandrogen in idiopathic hirsutism

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Somani V

1998-01-01

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

Idiopathic granulomatous mastitis: in search of a therapeutic paradigm.

Science.gov (United States)

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

2007-08-01

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

Idiopathic Pulmonary Calcification and Ossification in an Elderly ...

African Journals Online (AJOL)

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

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

International Nuclear Information System (INIS)

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

2004-01-01

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

Your True Freedom

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Jena Rausch

2015-02-01

Full Text Available Your True Freedom is about my journey teaching inmates the fundamental truths of self worth, self acceptance and self love--through writing, mindfulness meditation and emotional healing. It is a journey that continues to enlighten me and to heal and free the inmates with whom I work.

Systemic True False

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):. (a). (b). (c). (d). Answer: True systemics are (b & c); False Systemics (a & d). For more examples, see (10 and 11). Na2O2. NaCl. Na. HCl heat /excess. (O). 300°C electrolysis. (molten). NaOH. NaCl. Na. HCl. H2O electrolysis. (solution). NaCl.

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

NARCIS (Netherlands)

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

2014-01-01

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

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

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Mimura Tatsuya

2010-12-01

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

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.

Unusual Presentation Of Idiopathic Retroperitoneal Fibrosis: Case ...

African Journals Online (AJOL)

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

Idiopathic granulomatous lobular mastitis.

Science.gov (United States)

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

2012-02-01

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

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

Science.gov (United States)

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

2015-09-01

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

Idiopathic intracranial hypertension with altered consciousness in a ...

African Journals Online (AJOL)

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

Löffler's endomyocarditis in the idiopathic hypereosinophilic syndrome

NARCIS (Netherlands)

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

1999-01-01

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

Treatment of Resistant Idiopathic Hiccups with Pulse Radio ...

African Journals Online (AJOL)

2017-09-14

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

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

Science.gov (United States)

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

2017-05-01

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

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

Science.gov (United States)

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

2014-03-01

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

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

Science.gov (United States)

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

2015-01-01

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

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

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Juliana C. Ferreira

2011-01-01

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

Anterior Overgrowth in Primary Curves, Compensatory Curves and Junctional Segments in Adolescent Idiopathic Scoliosis.

Science.gov (United States)

Schlösser, Tom P C; van Stralen, Marijn; Chu, Winnie C W; Lam, Tsz-Ping; Ng, Bobby K W; Vincken, Koen L; Cheng, Jack C Y; Castelein, René M

2016-01-01

Although much attention has been given to the global three-dimensional aspect of adolescent idiopathic scoliosis (AIS), the accurate three-dimensional morphology of the primary and compensatory curves, as well as the intervening junctional segments, in the scoliotic spine has not been described before. A unique series of 77 AIS patients with high-resolution CT scans of the spine, acquired for surgical planning purposes, were included and compared to 22 healthy controls. Non-idiopathic curves were excluded. Endplate segmentation and local longitudinal axis in endplate plane enabled semi-automatic geometric analysis of the complete three-dimensional morphology of the spine, taking inter-vertebral rotation, intra-vertebral torsion and coronal and sagittal tilt into account. Intraclass correlation coefficients for interobserver reliability were 0.98-1.00. Coronal deviation, axial rotation and the exact length discrepancies in the reconstructed sagittal plane, as defined per vertebra and disc, were analyzed for each primary and compensatory curve as well as for the junctional segments in-between. The anterior-posterior difference of spinal length, based on "true" anterior and posterior points on endplates, was +3.8% for thoracic and +9.4% for (thoraco)lumbar curves, while the junctional segments were almost straight. This differed significantly from control group thoracic kyphosis (-4.1%; P<0.001) and lumbar lordosis (+7.8%; P<0.001). For all primary as well as compensatory curves, we observed linear correlations between the coronal Cobb angle, axial rotation and the anterior-posterior length difference (r≥0.729 for thoracic curves; r≥0.485 for (thoraco)lumbar curves). Excess anterior length of the spine in AIS has been described as a generalized growth disturbance, causing relative anterior spinal overgrowth. This study is the first to demonstrate that this anterior overgrowth is not a generalized phenomenon. It is confined to the primary as well as the

Segmental torso masses in adolescent idiopathic scoliosis.

Science.gov (United States)

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

2014-08-01

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

Acute surgical management in idiopathic intracranial hypertension.

LENUS (Irish Health Repository)

Zakaria, Zaitun

2012-01-01

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

Idiopathic thrombocytopenic purpura

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L Kayal

2014-01-01

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

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

DEFF Research Database (Denmark)

Grauers, Anna; Wang, Jingwen; Einarsdottir, Elisabet

2015-01-01

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

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...

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

Science.gov (United States)

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

2017-08-01

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

Graves' disease and idiopathic intracranial hypertension

OpenAIRE

Manish Gutch; Annesh Bhattacharjee; Sukriti Kumar; Durgesh Pushkar

2017-01-01

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

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

Science.gov (United States)

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

2009-11-01

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

Clinical efficiency of cyclosporine in chronic idiopathic urticaria in adults

Directory of Open Access Journals (Sweden)

V.I. Petrov

2010-06-01

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

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

International Nuclear Information System (INIS)

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

2000-01-01

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

Idiopathic hypertrophic subaortic stenosis. I

International Nuclear Information System (INIS)

Kvam, G.

1980-01-01

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

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

International Nuclear Information System (INIS)

Hiraoka, Kotaro; Meguro, Kenichi; Mori, Etsuro

2008-01-01

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

Idiopathic granulomatous mastitis | Rubin | SA Journal of Radiology

African Journals Online (AJOL)

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

Idiopathic Oesophageal Dysmotility Disorder: Stridor Secondary to Megaesophagus

Directory of Open Access Journals (Sweden)

B. G. Natesh

2013-01-01

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

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

Science.gov (United States)

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

2018-03-07

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

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

Science.gov (United States)

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

2011-01-01

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

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

DEFF Research Database (Denmark)

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

2015-01-01

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

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

International Nuclear Information System (INIS)

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

2009-01-01

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

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

Science.gov (United States)

2013-01-01

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

New protocetid whale from the middle eocene of pakistan: birth on land, precocial development, and sexual dimorphism.

Directory of Open Access Journals (Sweden)

Philip D Gingerich

Full Text Available BACKGROUND: Protocetidae are middle Eocene (49-37 Ma archaeocete predators ancestral to later whales. They are found in marine sedimentary rocks, but retain four legs and were not yet fully aquatic. Protocetids have been interpreted as amphibious, feeding in the sea but returning to land to rest. METHODOLOGY/PRINCIPAL FINDINGS: Two adult skeletons of a new 2.6 meter long protocetid, Maiacetus inuus, are described from the early middle Eocene Habib Rahi Formation of Pakistan. M. inuus differs from contemporary archaic whales in having a fused mandibular symphysis, distinctive astragalus bones in the ankle, and a less hind-limb dominated postcranial skeleton. One adult skeleton is female and bears the skull and partial skeleton of a single large near-term fetus. The fetal skeleton is positioned for head-first delivery, which typifies land mammals but not extant whales, evidence that birth took place on land. The fetal skeleton has permanent first molars well mineralized, which indicates precocial development at birth. Precocial development, with attendant size and mobility, were as critical for survival of a neonate at the land-sea interface in the Eocene as they are today. The second adult skeleton is the most complete known for a protocetid. The vertebral column, preserved in articulation, has 7 cervicals, 13 thoracics, 6 lumbars, 4 sacrals, and 21 caudals. All four limbs are preserved with hands and feet. This adult is 12% larger in linear dimensions than the female skeleton, on average, has canine teeth that are 20% larger, and is interpreted as male. Moderate sexual dimorphism indicates limited male-male competition during breeding, which in turn suggests little aggregation of food or shelter in the environment inhabited by protocetids. CONCLUSIONS/SIGNIFICANCE: Discovery of a near-term fetus positioned for head-first delivery provides important evidence that early protocetid whales gave birth on land. This is consistent with skeletal

True self-alienation positively predicts reports of mindwandering.

Science.gov (United States)

Vess, Matthew; Leal, Stephanie A; Hoeldtke, Russell T; Schlegel, Rebecca J; Hicks, Joshua A

2016-10-01

Two studies assessed the relationship between feelings of uncertainty about who one truly is (i.e., true self-alienation) and self-reported task-unrelated thoughts (i.e., mindwandering) during performance tasks. Because true self-alienation is conceptualized as the subjective disconnect between conscious awareness and actual experience, we hypothesized that greater feelings of true self-alienation would positively relate to subjective reports of mindwandering. Two convergent studies supported this hypothesis. Moreover, this relationship could not consistently be accounted for by the independent influence of other aspects of authenticity, negative mood, mindfulness, or broad personality dimensions. These findings suggest that individual differences in true self-alienation are reliably associated with subjective reports of mindwandering. The implications of these findings for the true self-alienation construct, the ways that personality relates to mindwandering, and future research directions focused on curtailing mindwandering and improving performance and achievement are discussed. Copyright © 2016 Elsevier Inc. All rights reserved.

Knowledge as Fact-Tracking True Belief

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Fred Adams

Full Text Available ABSTRACT Drawing inspiration from Fred Dretske, L. S. Carrier, John A. Barker, and Robert Nozick, we develop a tracking analysis of knowing according to which a true belief constitutes knowledge if and only if it is based on reasons that are sensitive to the fact that makes it true, that is, reasons that wouldn’t obtain if the belief weren’t true. We show that our sensitivity analysis handles numerous Gettier-type cases and lottery problems, blocks pathways leading to skepticism, and validates the epistemic closure thesis that correct inferences from known premises yield knowledge of the conclusions. We discuss the plausible views of Ted Warfield and Branden Fitelson regarding cases of knowledge acquired via inference from false premises, and we show how our sensitivity analysis can account for such cases. We present arguments designed to discredit putative counterexamples to sensitivity analyses recently proffered by Tristan Haze, John Williams and Neil Sinhababu, which involve true statements made by untrustworthy informants and strange clocks that sometimes display the correct time while running backwards. Finally, we show that in virtue of employing the paradox-free subjunctive conditionals codified by Relevance Logic theorists instead of the paradox-laden subjunctive conditionals codified by Robert Stalnaker and David Lewis.

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

African Journals Online (AJOL)

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

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

DEFF Research Database (Denmark)

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

2016-01-01

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

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

International Nuclear Information System (INIS)

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

1991-01-01

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

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

Science.gov (United States)

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

2015-03-01

Discrete potentials (DPs) have been recorded and targeted as the site of ablation of the outflow tract arrhythmias. The aim of the present study was to investigate the significance of DPs with respect to mapping and ablation for idiopathic outflow tract premature ventricular contractions (PVCs) or ventricular tachycardias (VTs). Seventeen consecutive patients with idiopathic right or left ventricular outflow tract PVCs/VTs who underwent radiofrequency catheter ablation were included. Intracardiac electrograms during the mapping and ablation were analysed. During sinus rhythm, sharp high-frequency DPs that displayed double or multiple components were recorded following or buried in the local ventricular electrograms in all of the 17 patients, peak amplitude 0.51 ± 0.21 mV. The same potential was recorded prior to the local ventricular potential of the PVCs/VTs. Spontaneous reversal of the relationship of the DPs to the local ventricular electrogram during the arrhythmias was noted. The DPs were related to a region of low voltage showed by intracardiac high-density contact mapping. At the sites with DPs, lower unipolar and bipolar ventricular voltage of sinus beats were noted compared with the adjacent regions without DPs (unipolar: 6.1 ± 1.8 vs. 8.3 ± 2.3 mV, P Discrete potentials were not present in seven controls. Discrete potentials and related low-voltage regions were common in idiopathic outflow tract ventricular arrhythmias. Discrete potential- and substrate-guided ablation strategy will help to reduce the recurrence of idiopathic outflow tract arrhythmias. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2014. For permissions please email: journals.permissions@oup.com.

Introversion, the prevalent trait of adolescents with idiopathic scoliosis: an observational study

Directory of Open Access Journals (Sweden)

Elisabetta D’Agata

2017-11-01

Full Text Available Abstract Background A large number of studies about adolescents with idiopathic scoliosis focus on health-related quality of life (HRQOL. However, only a few articles aim at evaluating the personality of these patients. Therefore, the purpose of the present research is to assess the personality traits of adolescents with idiopathic scoliosis and their relationship with HRQOL. Our hypothesis is that adolescents with idiopathic scoliosis present the principal personality trait of introversion, defined as self-reliance and inhibition in social relationships. Methods This was a cross-sectional study. The examined group consisted of 43 patients (only 4 boys, mean age = 14.3 (SD = 2.23. On the day of the visit, HRQOL tools (Scoliosis Research Society-22 Questionnaire (SRS-22 and Trunk Appearance Perception Scale (TAPS and a personality test (16 Personality Factors-Adolescent Personality Questionnaire (16PF-APQ were completed; in addition, a posterior-anterior radiography was performed. Correlations among demographic and medical data and HRQOL and personality tests were assessed. Results Results for SRS-22 were as follows: Function 4.5 (SD = .4, Pain 4.3 (SD = .5, Self-image 3.6 (SD = .7, Mental Health 3.8. (SD = .7, and Subtotal 4.2 (SD = .7. Mean TAPS was 3.5 (SD = .6. In personality, the lowest values were assessed for Extroversion (M = 29.4, SD = 24.7 and Self-reliance (M = 71, SD = 25.3. Independence was negatively related to Self-image (r = −.51, Mental Health (r = −.54, and Subtotal SRS-22 (r = −.60 (p < .01. Conclusions Adolescents with idiopathic scoliosis presented a common style of personality, characterized by social inhibition (introversion, preference for staying alone, and being self-sufficient (self-reliance. Specific programs in promoting social abilities may help adolescent patients with idiopathic scoliosis in finding a way to express themselves and to become more sociable. Correlational studies

How innate is locomotion in precocial animals? A study on the early development of spatio-temporal gait variables and gait symmetry in piglets.

Science.gov (United States)

Vanden Hole, Charlotte; Goyens, Jana; Prims, Sara; Fransen, Erik; Ayuso Hernando, Miriam; Van Cruchten, Steven; Aerts, Peter; Van Ginneken, Chris

2017-08-01

Locomotion is one of the most important ecological functions in animals. Precocial animals, such as pigs, are capable of independent locomotion shortly after birth. This raises the question whether coordinated movement patterns and the underlying muscular control in these animals is fully innate or whether there still exists a rapid maturation. We addressed this question by studying gait development in neonatal pigs through the analysis of spatio-temporal gait characteristics during locomotion at self-selected speed. To this end, we made video recordings of piglets walking along a corridor at several time points (from 0 h to 96 h). After digitization of the footfalls, we analysed self-selected speed and spatio-temporal characteristics (e.g. stride and step lengths, stride frequency and duty factor) to study dynamic similarity, intralimb coordination and interlimb coordination. To assess the variability of the gait pattern, left-right asymmetry was studied. To distinguish neuromotor maturation from effects caused by growth, both absolute and normalized data (according to the dynamic similarity concept) were included in the analysis. All normalized spatio-temporal variables reached stable values within 4 h of birth, with most of them showing little change after the age of 2 h. Most asymmetry indices showed stable values, hovering around 10%, within 8 h of birth. These results indicate that coordinated movement patterns are not entirely innate, but that a rapid neuromotor maturation, potentially also the result of the rearrangement or recombination of existing motor modules, takes place in these precocial animals. © 2017. Published by The Company of Biologists Ltd.

MRI evaluation of multifidus muscles in adolescent idiopathic scoliosis

International Nuclear Information System (INIS)

Chan Yu-Leung; King, A.D.; Griffith, J.F.; Metreweli, C.; Cheng, J.C.Y.; Guo Xia

1999-01-01

Background. The role of the multifidus muscles in the initiation and progression of curve in adolescent idiopathic scoliosis is not fully understood and controversy exists as to the side of the abnormality. Objective. To evaluate on MRI the multifidus muscles at the apex of the major curve in adolescent idiopathic scoliosis to ascertain if the multifidus muscles on the convex or concave side are abnormal and the relationship to curve severity. Materials and methods. Forty-six patients with adolescent idiopathic scoliosis, separated into two groups, were studied using a 1.5-T MR scanner with the synergy spine coil, employing a modified STIR (short tau inversion recovery) axial sequence obtained at the apex of the major scoliotic curve. Results. No hyperintense signal change was demonstrated in the convex side multifidus muscles in any patient. In group I, 16 of 18 patients with severe or rapidly progressive curve showed increase in signal intensity in the multifidus muscle on the concave side of the apex of the curve. In group II, of the 15 patients with mild curve (Cobb angle 10-30 ), 4 had increased signal intensity in the multifidus muscle on the concave side; of the 13 with more severe curve (Cobb angle greater than 30 ), 10 had increase in multifidus signal intensity on the concave side. Conclusions. The concave-side multifidus muscle at the apex of a scoliotic curve was morphologically abnormal. A significant association between abnormal signal change and curve severity was also established. (orig.)

Idiopathic toe-walking in children, adolescents and young adults: a matter of local or generalised stiffness?

NARCIS (Netherlands)

Engelbert, Raoul; Gorter, Jan Willem; Uiterwaal, Cuno; van de Putte, Elise; Helders, Paul

2011-01-01

Idiopathic Toe Walking (ITW) is present in children older than 3 years of age still walking on their toes without signs of neurological, orthopaedic or psychiatric diseases. ITW has been estimated to occur in 7% to 24% of the childhood population. To study associations between Idiopathic Toe Walking

The pattern of juvenile idiopathic arthritis; a retrospective Egyptian ...

African Journals Online (AJOL)

Ehab

subtype specially at rural areas which differed from Western and Gulf countries pattern. ... version 18) and the following statistical measures ..... the results of Abdwani et al27 at 2015 (an Omani ... Ravelli A, Martini A. Juvenile idiopathic arthritis.

An Update on Idiopathic Intracranial Hypertension

OpenAIRE

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

2010-01-01

Idiopathic intracranial hypertension (IIH) is a condition of unknown etiology often encountered in neurologic practice. It produces non-localizing symptoms and signs of raised intracranial pressure and, when left untreated, can result in severe irreversible visual loss. It most commonly occurs in obese women of childbearing age, but it can also occur in children, men, non-obese adults, and older adults. While it is frequently associated with obesity, it can be associated with other conditions...

Case of twin pregnancy complicated by idiopathic thrombocytopenic ...

African Journals Online (AJOL)

2016-05-09

May 9, 2016 ... Idiopathic thrombocytopenic purpura (ITP) is an acquired thrombocytopenia without other clear cause of thrombocytopenia. It is not common in a singleton pregnancy and less common in twin pregnancy. We report a 33‑year‑old ITP pluripara whose first pregnancy was uneventful. She carried twin ...

Progressive multifocal leucoencephalopathy in a patient with idiopathic CD4+ lymphocytopenia.

LENUS (Irish Health Repository)

Moloney, F

2012-03-01

Progressive multifocal leucoencephalopathy (PML) is an opportunistic, demyelinating neurological disease caused by reactivation of the JC polyomavirus. PML occurs almost exclusively in immunosuppressed individuals, with only isolated case reports of PML occurring in patients without apparent immunosuppression. Idiopathic CD4+ lymohocytopenia (ICL) is a syndrome defined by the Centre for Disease Control and Prevention as a CD4+ count <300 cells\\/uL or <20% of total T cell count on >1 occasion, with no evidence of human immunodeficiency virus (HIV) infection, and the absence of other known immunodeficiency or therapy associated with lymphocytopenia. We describe a case of PML occurring in a patient with idiopathic CD4+ lymphocytopenia.

In search of true friendship

Directory of Open Access Journals (Sweden)

Luciana Karine de Souza

2008-01-01

Full Text Available The present text proposes a discussion on the concept of true friendship. The argument is grounded mostly on Aristotle's Nicomachean Ethics, Owen Flanagan's ethics as human ecology, and on contemporary authors' works about the Greek philosopher's concept of friendship. Given that human beings flourish through 1 exercising capacities, 2 being moral, and 3 having true friendships, difficulties to establish the level of trust required by true friendships turns the search itself (for them morally valid.O presente texto propõe uma discussão sobre o conceito de amizade verdadeira. A argumentação que fundamenta o trabalho baseia-se em grande parte na Ética a Nicômaco de Aristóteles, na ética como ecologia humana de Owen Flanagan, e em autores contemporâneos sobre o conceito de amizade do filósofo grego. Considerando-se que seres humanos encontram a felicidade mediante 1 o exercício de suas capacidades, 2 ser um ente moral, e 3 ter amizades verdadeiras, as dificuldades no estabelecimento do nível de amizade requerido nas amizades verdadeiras torna a busca (por elas, por si só, algo moralmente válido.

Comparison of plasma, liver, and skeletal muscle carnitine concentrations in cats with idiopathic hepatic lipidosis and in healthy cats.

Science.gov (United States)

Jacobs, G; Cornelius, L; Keene, B; Rakich, P; Shug, A

1990-09-01

Concentrations of total, free, and esterified carnitine were determined in plasma, liver, and skeletal muscle from cats with idiopathic hepatic lipidosis and compared with values from healthy cats. The mean concentrations of plasma, liver, and skeletal muscle total carnitine; plasma and skeletal muscle free carnitine; and plasma and liver esterified carnitine were greater (P less than 0.05) in cats with idiopathic hepatic lipidosis than in control cats. The mean for the ratio of free/total carnitine in plasma and liver was lower (P less than 0.05) in cats with idiopathic hepatic lipidosis than in control cats. These data suggest that carnitine deficiency does not contribute to the pathogenesis of feline idiopathic hepatic lipidosis.

[A case of cutaneous extramedullary hematopoiesis associated with idiopathic myelofibrosis].

Science.gov (United States)

Corella, F; Barnadas, M A; Bordes, R; Curell, R; Espinosa, I; Vergara, C; Alomar, A

2008-05-01

Cutaneous extramedullary hematopoiesis is a rare manifestation of chronic myeloproliferative processes, mainly chronic idiopathic myelofibrosis. In adults, it manifests as macules, papules, nodules, and ulcers on the trunk. The lesions usually appear soon after diagnosis and the possibility of a relationship between splenectomy and the appearance of extramedullary foci of hematopoiesis is still debated. Diagnosis is based on histopathology showing an infiltrate with different combinations of myeloid and erythroid cell precursors and megakaryocytes. Symptomatic treatment is provided alongside treatment of the underlying disease. We report a new case associated with chronic idiopathic myelofibrosis in which foci of cutaneous extramedullary hematopoiesis were observed 9 years after initial diagnosis. The lesions were progressive and the patient went on to develop acute myeloid leukemia.

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....

Outcomes of recalcitrant idiopathic epistaxis in children: Septoplasty as a surgical treatment.

Science.gov (United States)

Levi, Jessica M; McKee-Cole, Katherine M; Barth, Patrick C; Brody, Robert M; Reilly, James S

2016-12-01

Our objective was to measure short- and long-term outcomes of children presenting with recalcitrant idiopathic epistaxis. The study was an 11-year (2000-2011) retrospective chart review of children evaluated and treated for epistaxis. A retrospective review of patients with diagnostic International Classification of Diseases, Ninth Revision code 784.7 (epistaxis) and 21.5/21.88 (septoplasty) was completed reviewing age at presentation, type of surgery, and number of bleeding events prior to and after surgery. In our cohort, almost 100% of children with idiopathic recurrent epistaxis responded to topical treatments. About 0.2% were refractory (20/9239), and 90% of those (18/20) resolved with either a traditional septoplasty (14/20) or modified septoplasty (6/20) without cartilage excision, with a mean follow-up of 35 months. One of the patients who did not show resolution was found to have Von Willebrand disease, which likely contributed to this outcome. Septoplasty surgery, with or without cartilage removal, appears beneficial for refractory idiopathic epistaxis in children. 4 Laryngoscope, 126:2833-2837, 2016. © 2016 The American Laryngological, Rhinological and Otological Society, Inc.

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...

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

Science.gov (United States)

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

2016-03-01

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

Structural white matter abnormalities in patients with idiopathic dystonia

NARCIS (Netherlands)

Bonilha, Leonardo; de Vries, Paulien M.; Vincent, Diana J.; Rorden, Chris; Morgan, Paul S.; Hurd, Mark W.; Besenski, Nada; Bergmann, Kenneth J.; Hinson, Vanessa K.

2007-01-01

We investigated whether structural white matter abnormalities, in the form of disruption of axonal coherence and integrity as measured with diffusion tensor imaging (DTI), constitute an underlying pathological mechanism of idiopathic dystonia (ID), independent of genotype status. We studied seven

Evaluation of classification systems for nonspecific idiopathic orbital inflammation

NARCIS (Netherlands)

Bijlsma, Ward R.; van 't Hullenaar, Fleur C.; Mourits, Maarten P.; Kalmann, Rachel

2012-01-01

To systematically analyze existing classification systems for idiopathic orbital inflammation (IOI) and propose and test a new best practice classification system. A systematic literature search was conducted to find all studies that described and applied a classification system for IOI.

Ultrasonography and color Doppler in juvenile idiopathic arthritis

DEFF Research Database (Denmark)

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

2012-01-01

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

Prognosis in canine idiopathic immune-mediated haemolytic anaemia

NARCIS (Netherlands)

Piek, C.J.

2011-01-01

Canine idiopathic immune-mediated haemolytic anaemia (iIMHA) is one of the most frequently occurring immune-mediated diseases in dogs. A gel-based Coombs' test was shown to perform equally well as a classical Coombs' test. Since the gel-based Coombs' test can be commercially produced and is easy and

Chancre of the eyelid as manifestation of primary syphilis, and precocious chorioretinitis and uveitis in an HIV-infected patient: a case report

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Cillino Salvatore

2012-09-01

Full Text Available Abstract Background Ocular syphilis is often difficult to diagnose because of the wide variation in clinical features. HIV co-infection may further complicate the picture. Case presentation Herein the authors report an unusual primary syphilitic ocular lesion in a 45-year-old Italian HIV-infected bisexual man who presented with a unilateral eyelid lesion. Associated precocious signs and symptoms in the posterior segment of both eyes, bilateral chorioretinitis and uveitis, are described. Intravenous penicillin and steroid treatment produced a rapid improvement in clinical status and complete resolution. Conclusions Careful questioning about sexual behavior is crucial for unmasking unusual features of ocular syphilis in HIV-infected subjects.

The Brazilian version of the SRS-22r questionnaire for idiopathic scoliosis

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Paula M. F. Camarini

2013-10-01

Full Text Available BACKGROUND: The SRS-22r questionnaire is a well-accepted instrument used to measure health-related quality of life in patients with idiopathic scoliosis. No validated tool exists in Brazil for idiopathic scoliosis, and the use of the SRS-22r in non-English Laguage contries requires its transcultural adaptation. OBJECTIVE: The objective of this study was to culturally adapt the translated Brazilian version of the SRS-22r questionnaire and to determine its reliability using statistical tests for internal consistency and test-retest reliability. METHOD: The transcultural adaptation process was carried out according to the recommendations of the American Academy of Orthopedic Surgeons. The pre-final version was administered to 44 patients with idiopathic scoliosis. The mean age of the participants was 18.93 years and the mean curve magnitude was 54.6°. A subgroup of 30 volunteers completed the questionnaire a second time one week later to determine the scale's reproducibility. Internal consistency was determined using Cronbach's alpha coefficient, and the test-retest reliability was determined using the Intraclass Correlation Coefficient (ICC. RESULTS: No floor effects were observed using the Brazilian version of the SRS-22r. Ceiling effects were observed in the Pain and Satisfaction with Management domains. The internal consistency values were very good for 3 domains and good for 2 domains. The ICC values were excellent for all domains. CONCLUSIONS: The high values of internal consistency and ICC reproducibility suggest that this version of the questionnaire can be used in Brazilian patients with idiopathic scoliosis.

A case of idiopathic interstitial pneumonia

OpenAIRE

豊見山, 寛; 兼島, 洋; 下地, 克佳; 金城, 勇徳; 中富, 昌夫; 小張, 一峰; 松井, 克明; Tomiyama, Hiroshi; Kaneshima, Hiroshi; Shimoji, Katsuyoshi; Kinjo, Yutoku; Nakatomi, Masao; Kobari, Kazumine; Matsui, Katsuaki; 琉球大学医学部第一内科

1982-01-01

A case of idiopathic interstitial pneumonia was reported. A 53-year-old man was admitted to our hospital with his complaint of shortness of breath. On physical examination clubbing of the fingers was noticed and velcro rales was heard on the bilateral lower back. On laboratory data no remarkable finding was revealed. Chest roentgenogram showed diffuse reticulonodular shadow and small ring shadow of the bilateral lower lung fields. On pulmonary function test decline of VC and DLco was revealed...

Idiopathic fibrosing pancreatitis: a rare cause of chronic obstructive jaundice in childhood

International Nuclear Information System (INIS)

Park, Hye Seong; Kim, Hyun Sook; Kim, Hack Hee; Kim, Ok Hwa; Kim, Choon Yul; Bahk, Yong Whee

1992-01-01

We report a 14-months-old infant who had obstructive jaundice caused by idiopathic fibrosing pancreatitis. Ultrasonography and abdominal computed tomography showed dilatation of the intrahepatic ducts, common bile duct, and the pancreatic duct. Diffuse swelling of the pancreas was also noted on CT. At laparotomy, the head portion of the pancreas revealed a stony hard consistency, and proliferation of fibrotic tissue was confirmed pathologically. Idiopathic fibrosing pancreatitis is a very rare disease entity in childhood, but should be considered in the differential diagnosis of obstructive jaundice in children who demonstrate bile duct and pancreatic duct dilatation and/or diffuse pancreas swelling

Idiopathic Scrotal Hematoma in Neonate: A Case Report and Review of the Literature

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Bioku Muftau Jimoh

2014-01-01

Full Text Available Neonatal scrotal hematoma is a rare genitourinary emergency. Some cases have underlining aetiologic factors such as testicular torsion, adrenal hemorrhage, or birth trauma, and others are idiopathic. Previously, immediate scrotal exploration was considered imperative for diagnosis and treatment. With good imaging techniques, some patients are managed nonoperatively. We report a case of idiopathic scrotal hematoma in a neonate. He was managed conservatively with clinical and radiological follow-up. There was complete resolution of hematoma within two months, thus, avoiding unnecessary exploration.

Handwriting difficulties in juvenile idiopathic arthritis: a pilot study

NARCIS (Netherlands)

Haberfehlner, Helga; Visser, Bart; Daffertshofer, Andreas; van Rossum, Marion Aj; Roorda, Leo D.; van der Leeden, Marike; Dekker, Joost; Hoeksma, Agnes F.

2011-01-01

The aim of the present study was to describe handwriting difficulties of primary school children with juvenile idiopathic arthritis (JIA), and to investigate possible correlations with hand function and writing performance. In a cross-sectional approach, 15 children with JIA and reported handwriting

Disrupted thalamic prefrontal pathways in patients with idiopathic dystonia

NARCIS (Netherlands)

Bonilha, Leonardo; de Vries, Paulien M.; Hurd, Mark W.; Rorden, Chris; Morgan, Paul S.; Besenski, Nada; Bergmann, Kenneth J.; Hinson, Vanessa K.

There are quantifiable abnormalities in water diffusion properties of the white matter in thalamic and prefrontal areas in patients with idiopathic dystonia (ID). However, it is unclear which pathways are disrupted in these patients. Using probabilistic tractography of high resolution DTI, we

Fahr’s syndrome and idiopathic hypoparathyroidism: A case report

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Marinković Dejan M.

2017-01-01

Full Text Available Introduction. Fahr´s syndrome is a rare, slowly progressive, neurodegenerative disorder, characterised by extensive, bilateral, and symmetrical basal ganglia calcification. It is associated with neuropsychiatric manifestations and gradually progressive cognitive impairment. Fahr's syndrome is the secondary form of brain calcification that is caused by various metabolic, infectious, or degenerative diseases. Case report. We presented a middle-aged male with Fahr's syndrome due to primary idiopathic hypoparathyroidism. Clinical diagnosis was based on signs and symptoms of hypocalcemia, progressive neuropsychiatric illnesses, laboratory evidence of hypoparathyroidism, and radiological signs of calcifications in the basal ganglia. The patient improved after only a few days of intravenous rehydration and calcium substitution, followed by oral supplemental calcitriol. Conclusion. Timely recognition of idiopathic and iatrogenic hypoparathyroidism allows appropriate treatment that can prevent the development and clinical manifestations of Fahr´s syndrome and potentially slow its progression.

Idiopathic interstitial pneumonias. From classification to diagnostic work-up

International Nuclear Information System (INIS)

Mueller-Mang, C.; Bankier, A.; Stiebellehner, L.; Schmid, K.

2007-01-01

Idiopathic interstitial pneumonias (IIP) comprise seven entities with distinct histologic patterns. In their idiopathic form IIP are rare diseases. They are, nevertheless, considered prototypes of the much more common secondary interstitial pneumonias. The advent of high-resolution computed tomography (HRCT) has had a profound impact on the imaging of IIP, because the detailed delineation of the lung anatomy allows a close correlation between the histologic patterns of IIP and the CT features. On the basis of CT morphology and in the correct clinical context, the radiologist can achieve an accurate diagnosis in many cases. However, due to overlap between the various entities, complementary lung biopsy is recommended in virtually all cases. This article reviews the CT pattern of IIP and offers relevant clinical and histological information for the purpose of enabling the radiologist to understand and participate in the multidisciplinary concept of IIP. (orig.) [de

Analysis of scapular muscle EMG activity in patients with idiopathic neck pain: a systematic review.

Science.gov (United States)

Castelein, Birgit; Cools, Ann; Bostyn, Emma; Delemarre, Jolien; Lemahieu, Trees; Cagnie, Barbara

2015-04-01

It is proposed that altered scapular muscle function can contribute to abnormal loading of the cervical spine. However, it is not clear if patients with idiopathic neck pain show altered activity of the scapular muscles. The aim of this paper was to systematically review the literature regarding the differences or similarities in scapular muscle activity, measured by electromyography ( = EMG), between patients with chronic idiopathic neck pain compared to pain-free controls. Case-control (neck pain/healthy) studies investigating scapular muscle EMG activity (amplitude, timing and fatigue parameters) were searched in Pubmed and Web of Science. 25 articles were included in the systematic review. During rest and activities below shoulder height, no clear differences in mean Upper Trapezius ( = UT) EMG activity exist between patients with idiopathic neck pain and a healthy control group. During overhead activities, no conclusion for scapular EMG amplitude can be drawn as a large variation of results were reported. Adaptation strategies during overhead tasks are not the same between studies. Only one study investigated timing of the scapular muscles and found a delayed onset and shorter duration of the SA during elevation in patients with idiopathic neck pain. For scapular muscle fatigue, no definite conclusions can be made as a wide variation and conflicting results are reported. Further high quality EMG research on scapular muscles (broader than the UT) is necessary to understand/draw conclusions on how scapular muscles react in the presence of idiopathic neck pain. Copyright © 2015 Elsevier Ltd. All rights reserved.

Idiopathic granulomatous hypophysitis: clinical and imaging features

Energy Technology Data Exchange (ETDEWEB)

Vasile, M. [Service de Radiologie, Hopital Saint-Antoine, 75 - Paris (France); Marsot-Dupuch, K. [Service de Radiologie, Hopital Saint-Antoine, 75 - Paris (France); Kujas, M. [Service d`Histologie Embryologie Cytogenetique, Hopital Pitie-Salpetriere, 75 - Paris (France); Brunereau, L. [Service de Radiologie, Hopital Saint-Antoine, 75 - Paris (France); Bouchard, P. [Service d`Histologie Embryologie Cytogenetique, Hopital Pitie-Salpetriere, 75 - Paris (France); Comoy, J. [Service de Neurochirurgie, Hopital Kremlin Bicetre, 94 (France); Tubiana, J.M. [Service de Radiologie, Hopital Saint-Antoine, 75 - Paris (France)

1997-01-01

Idiopathic pituitary granuloma is a rare disorder similar to lymphocytic adenohypophysitis. Few cases have been reported. We report a new histologically case proven with MRI. The patterns of clinical and radiological presentation and the management of this disorder are discussed. MRI findings suggestive of this condition include an intensely enhancing pituitary mass, associated with dural enhancement. Steroid therapy may be suggested avoiding unnecessary surgery. (orig.)

Comparison of true metabolisable energy and true amino acid availability between normal maize and quality protein maize (Shandan 17

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Mei Li Zhang

2010-01-01

Full Text Available A precision-fed assay was conducted to determine true metabolisable energy and true amino acid digestibility in Chinese quality protein maize (QPM compared with normal maize (NM. Thirty adult roosters, kept in individual cages, were made to fast for 48h and then tub-fed 50g QPM or NM per bird and their excreta was colleted for the subsequent 48h. Additional fifteen roosters were made to fast in order to estimate endogenous losses of energy and amino acids (AA in excreta. Gross energy of the two types of maize were similar; the lysine content in NM and QPM were 0.27% and 0.41% (DM basis, respectively. True metabolisable energy (TME and true metabolisable energy nitrogen corrected (TMEn values of QPM and NM were not significantly different (P>0.05. Digestibility of some AA, including lysine and methionine, in QPM were higher than those in NM (P<0.05. The results of this study indicated that the nutritive value of QPM might be higher than that of NM.

A de novo nonsense PDGFB mutation causing idiopathic basal ganglia calcification with laryngeal dystonia.

Science.gov (United States)

Nicolas, Gaël; Jacquin, Agnès; Thauvin-Robinet, Christel; Rovelet-Lecrux, Anne; Rouaud, Olivier; Pottier, Cyril; Aubriot-Lorton, Marie-Hélène; Rousseau, Stéphane; Wallon, David; Duvillard, Christian; Béjot, Yannick; Frébourg, Thierry; Giroud, Maurice; Campion, Dominique; Hannequin, Didier

2014-10-01

Idiopathic basal ganglia calcification (IBGC) is characterized by brain calcification and a wide variety of neurologic and psychiatric symptoms. In families with autosomal dominant inheritance, three causative genes have been identified: SLC20A2, PDGFRB, and, very recently, PDGFB. Whereas in clinical practice sporadic presentation of IBGC is frequent, well-documented reports of true sporadic occurrence are rare. We report the case of a 20-year-old woman who presented laryngeal dystonia revealing IBGC. Her healthy parents' CT scans were both normal. We identified in the proband a new nonsense mutation in exon 4 of PDGFB, c.439C>T (p.Gln147*), which was absent from the parents' DNA. This mutation may result in a loss-of-function of PDGF-B, which has been shown to cause IBGC in humans and to disrupt the blood-brain barrier in mice, resulting in brain calcification. The c.439C>T mutation is located between two previously reported nonsense mutations, c.433C>T (p.Gln145*) and c.445C>T (p.Arg149*), on a region that could be a hot spot for de novo mutations. We present the first full demonstration of the de novo occurrence of an IBGC-causative mutation in a sporadic case.

Identification of a novel idiopathic epilepsy locus in Belgian Shepherd dogs

DEFF Research Database (Denmark)

Seppälä, Eija H.; Koskinen, Lotta L.E.; Gulløv, Christina Hedal

2012-01-01

Epilepsy is the most common neurological disorder in dogs, with an incidence ranging from 0.5% to up to 20% in particular breeds. Canine epilepsy can be etiologically defined as idiopathic or symptomatic. Epileptic seizures may be classified as focal with or without secondary generalization...... collected 159 cases and 148 controls and confirmed the presence of epilepsy through epilepsy questionnaires and clinical examinations. The MRI was normal while interictal EEG revealed abnormalities and variable foci in the clinically examined affected dogs. A genome-wide association study using Affymetrix...... mutation. It would establish the affected breed as a novel therapeutic model, help to develop a DNA test for breeding purposes and introduce a novel candidate gene for human idiopathic epilepsies....

Are we simplifying balance evaluation in adolescent idiopathic scoliosis?

Science.gov (United States)

Pasha, Saba; Baldwin, Keith

2018-01-01

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

MRI evaluation of multifidus muscles in adolescent idiopathic scoliosis

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Chan Yu-Leung; King, A.D.; Griffith, J.F.; Metreweli, C. [Dept. of Diagnostic Radiology and Organ Imaging, Chinese University of Hong Kong, Prince of Wales Hospital, Shatin (Hong Kong); Cheng, J.C.Y.; Guo Xia [Orthopaedics and Traumatology, Chinese University of Hong Kong, Prince of Wales Hospital, Shatin (Hong Kong)

1999-05-01

Background. The role of the multifidus muscles in the initiation and progression of curve in adolescent idiopathic scoliosis is not fully understood and controversy exists as to the side of the abnormality. Objective. To evaluate on MRI the multifidus muscles at the apex of the major curve in adolescent idiopathic scoliosis to ascertain if the multifidus muscles on the convex or concave side are abnormal and the relationship to curve severity. Materials and methods. Forty-six patients with adolescent idiopathic scoliosis, separated into two groups, were studied using a 1.5-T MR scanner with the synergy spine coil, employing a modified STIR (short tau inversion recovery) axial sequence obtained at the apex of the major scoliotic curve. Results. No hyperintense signal change was demonstrated in the convex side multifidus muscles in any patient. In group I, 16 of 18 patients with severe or rapidly progressive curve showed increase in signal intensity in the multifidus muscle on the concave side of the apex of the curve. In group II, of the 15 patients with mild curve (Cobb angle 10-30 ), 4 had increased signal intensity in the multifidus muscle on the concave side; of the 13 with more severe curve (Cobb angle greater than 30 ), 10 had increase in multifidus signal intensity on the concave side. Conclusions. The concave-side multifidus muscle at the apex of a scoliotic curve was morphologically abnormal. A significant association between abnormal signal change and curve severity was also established. (orig.) With 2 figs., 3 tabs., 25 refs.

Radionuclide therapy for true polycythemia

International Nuclear Information System (INIS)

Afanasieva, N.I.; Grushka, G.V.; Vasiliev, L.Ya.

2005-01-01

Full text: True polycythemia (Vaquez' disease) is the disease of the hemopoietic system of an unknown origin accompanied by increase of erythrocyte count in the peripheral blood with absolute and relative increase in the mass of circulating erythrocytes. True polycythemia is treated with bloodletting and myelosuppressing drugs. This treatment is not always effective. A special place among myelo-depressants is occupied by P-32. Its main therapeutic effect is mediated by the participation in the metabolic processes. Besides DNA damage with beta-radiation, P-32 incorporation by DNA followed by decay and transformation to a stable sulphur isotope (S-32) results in the damage of the structure of nucleic acids, which is main mechanism of cellular proliferation inhibition. Therefore, P-32 is considered a powerful myelosuppressive agent. The indication to P-32 administration is true polycythemia, which, in contrast to secondary polycythemia accompanying numerous diseases, requires special cytostatic treatment. The study involved 152 patients with true polycythemia (76 men and 76 women aged 48) treated with P-32. The treatment was administered in case when bloodletting proved to be ineffective and hematocrit level exceeded 75%. P-32 was administered orally in 100 ml of 10% glucose solution on an empty stomach at a dose of 37-111 MBq with a 4-10-day intervals (mean 6 days). The blood count was checked for 12 weeks. If the number of thrombocytes and leukocytes decreased less than by 25% and the level of hematocrit did not normalize, repeated P-32 treatments were performed. In 13 (8.6%) patients, haematocrit parameters became normal after a single P-32 administration, in 15 (9.9%) patients two treatments with P-32 were necessary to normalize the blood count. In 134 (88.1%) cases P-32 treatment was administered more then two times (maximum 10 treatments). Total P-32 activity ranged from 37 to 740 MBq. Of all the patients who were administered P- 32 treatment, hematological

Omalizumab for the Treatment of Chronic Idiopathic Urticaria: Systematic Review of the Literature.

Science.gov (United States)

Tonacci, Alessandro; Billeci, Lucia; Pioggia, Giovanni; Navarra, Michele; Gangemi, Sebastiano

2017-04-01

Omalizumab is recombinant humanized monoclonal antibody to immunoglobulin E. Guidelines for the treatment of chronic idiopathic urticaria (also known as chronic spontaneous urticaria) recommend the use of omalizumab as third-line therapy in addition to high doses of histamine receptor type 1 (H 1 ) antihistamines when they are unsuccessful as first- and second-line therapy. We performed a systematic review of the literature to identify studies that evaluated the efficacy of omalizumab for the treatment of chronic idiopathic urticaria, in both controlled and real-world settings, to assess its potential role as a preferred therapy. The PubMed, ScienceDirect, LILACS (Latin American and Caribbean Health Sciences Literature), and Google Scholar databases were searched between January 1, 2000, and November 21, 2016. The search was limited to articles published in peer-reviewed journals in the English language, and 29 studies were included in this review. Omalizumab 300 mg administered every 4 weeks appears to be the most effective and safe dosage, with a rapid response time, for the treatment of chronic idiopathic urticaria, with few minor adverse effects, and appears to be safe in the offspring of pregnant patients who received the drug. However, as published studies of omalizumab are sparse, future studies are warranted. When findings are confirmed in larger studies, due to its efficacy, safety, and increased benefit/cost ratio, omalizumab could become the preferred method of treatment for chronic idiopathic urticaria in patients unresponsive to H 1 antihistamines. © 2017 Pharmacotherapy Publications, Inc.

TOPOGRAPHIC CHANGES OF THE MACULA AFTER CLOSURE OF IDIOPATHIC MACULAR HOLE.

Science.gov (United States)

Pak, Kang Yeun; Park, Keun Heung; Kim, Kyong Ho; Park, Sung Who; Byon, Ik Soo; Kim, Hyun Woong; Chung, In Young; Lee, Joo Eun; Lee, Sang Joon; Lee, Ji Eun

2017-04-01

To investigate retinal displacement in the macula after surgical closure of idiopathic macular hole and to identify factors correlated with displacement. This retrospective multicenter study included 73 eyes of 73 patients having idiopathic macular hole. A custom program was developed to compare the position of the retinal vessels in the macula between preoperative and postoperative photographs. En face images of a 6 mm × 6 mm optical coherence tomography volume scans were registered to calculate the scale. A grid comprising 16 sectors in 2 rings (inner; 2-4 mm and outer; 4-6 mm) was superimposed. The displacement of the retinal vessels was measured as a vector value by comparing the location of the retinal vessels in each sector. The correlation between displacement and various clinical parameters was analyzed. The average displacement was 57.2 μm at an angle of -3.3° (nasal and slightly inferior). Displacement was larger in the inner ring (79.2 μm) than in the outer ring (35.3 μm, P macula was displaced centripetally, nasally, and slightly inferiorly after surgical closure of idiopathic macular hole. Hole closure, contraction of the nerve fiber layer, and gravity are the suggested mechanisms of macular displacement caused by internal limiting membrane peeling.

Exercise therapy in juvenile idiopathic arthritis: a Cochrane Review

NARCIS (Netherlands)

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

2008-01-01

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

Providence nighttime bracing, in treatment of adolescent idiopathic scoliosis

DEFF Research Database (Denmark)

Simony, A.; Beuschau, Inge; Quisth, Lena

2015-01-01

Introduction: Since 2008 the non-surgical treatment of adolescent idiopathic scoliosis (AIS) in the southern part of Denmark, went from full-time bracing with Boston brace, to Providence night-time bracing. Methods: Since 2008, skeletally immature patients diagnosed with AIS and a primary curve w...

Effects of Low-frequency Current Sacral Dermatome Stimulation on Idiopathic Slow Transit Constipation.

Science.gov (United States)

Kim, Jin-Seop; Yi, Seung-Ju

2014-06-01

[Purpose] This study aimed to determine whether low-frequency current therapy can be used to reduce the symptoms of idiopathic slow transit constipation (ISTC). [Subjects] Fifteen patients (ten male and five female) with idiopathic slow transit constipation were enrolled in the present study. [Results] Bowel movements per day, bowel movements per week, and constipation assessment scale scores significantly improved after low-frequency current simulation of S2-S3. [Conclusion] Our results show that stimulation with low-frequency current of the sacral dermatomes may offer therapeutic benefits for a subject of patients with ISTC.

Diagnostic value of optical coherence tomography for intracranial pressure in idiopathic intracranial hypertension

DEFF Research Database (Denmark)

Skau, M; Yri, H; Sander, B

2013-01-01

BACKGROUND: Idiopathic intracranial hypertension (IIH) is a condition of raised intracranial pressure (ICP) in the absence of space-occupying lesions or other known etiology. It primarily affects young obese females, and potentially causes permanent visual loss due to papilledema and secondary...... optic atrophy. The aim of this study was to evaluate the diagnostic value of optical coherence tomography (OCT) as a marker for CSF opening pressure in patients with idiopathic intracranial hypertension (IIH). METHODS: We conducted a case-control study of 20 newly diagnosed, 21 long-term IIH patients...

Wind turbines and idiopathic symptoms

DEFF Research Database (Denmark)

Blanes-Vidal, Victoria; Schwartz, Joel

2016-01-01

Whether or not wind turbines pose a risk to human health is a matter of heated debate. Personal reactions to other environmental exposures occurring in the same settings as wind turbines may be responsible of the reported symptoms. However, these have not been accounted for in previous studies. We...... investigated whether there is an association between residential proximity to wind turbines and idiopathic symptoms, after controlling for personal reactions to other environmental co-exposures. We assessed wind turbine exposures in 454 residences as the distance to the closest wind turbine (Dw) and number...... of wind turbines

True ternary fission in 310126X

International Nuclear Information System (INIS)

Banupriya, B.; Vijayaraghavan, K.R.; Balasubramaniam, M.

2015-01-01

All possible combinations are minimized by the two dimensional minimization process and minimized with respect to neutron numbers and proton numbers of the fragments. Potential energy is low and Q - value is high at true ternary fission region. It shows that true ternary mode is the dominant mode in the ternary fission of superheavy nuclei. Also, the results show that the fragments with neutron magic numbers are the dominant one in the ternary fission of superheavy nuclei whereas the fragments with proton magic numbers are the dominant one in the ternary fission of heavy nuclei

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

Science.gov (United States)

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

2013-09-01

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

Toxocara Seroprevalence in Patients with Idiopathic Parkinson's Disease: Chance Association or Coincidence?

Directory of Open Access Journals (Sweden)

Tuncay Çelik

2013-01-01

Full Text Available Most cases of idiopathic Parkinson disease (IPD are believed to be due to a combination of genetic and environmental factors. The purpose of this study is to investigate the relationship between toxocariasis and Parkinson disease (PD. Patients were selected from people who were admitted to the Movement Disorders Branch, Neurology Department of Elazığ University Faculty of Medicine Elazığ, Turkey. We studied specific IgG antibodies against Toxocara canis (T. canis in 50 patients with idiopathic Parkinson and 50 healthy volunteers. We investigated the clinical history of three patients infected with T. canis. We also studied specific IgG antibodies against Toxoplasma gondii in these groups. Antibodies anti-Toxocara canis were found in 3 idiopathic PD (6% (P=0.121 and antibody titer was not found in control. A patient had history of the presence of dog in current dog ownership. We did not detect any statistically significant association between T. canis and IPD. But, we believe that further comprehensive studies are required for understanding whether there is a causal relation between toxocariasis and PD. We didn’t find possible association between Toxoplasma gondii and IPD (P=0.617.

Idiopathic Intracranial Hypertension – Pathophysiology Based on Case Series

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Ljubisavljević Srdjan

2016-09-01

Full Text Available According to the definition, idiopathic intracranial hypertension (IIH is a pathological state characterized by an increase in intracranial pressure; however, there are no obvious intracranial pathological processes. The pathophysiology of this disorder is not clear, although there are many reports related to it.

The aetiology of idiopathic scoliosis : biomechanical and neuromuscular factors

NARCIS (Netherlands)

Veldhuizen, AG; Webb, PJ

The aetiology of adolescent idiopathic scoliosis (AIS) remains an enigma. In the literature there are two opinions: one believes a deviating growth pattern is responsible for the condition - patients with AIS lend to be growing faster/be taller - while the other opinion assumes that the growth

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

Treatment of Juvenile Idiopathic Arthritis-Associated Uveitis

OpenAIRE

Oray, Merih; Tu?al-Tutkun, ?lknur

2016-01-01

Pediatric uveitis may be a serious health problem because of the lifetime burden of vision loss due to severe complications if the problem is not adequately treated. Juvenile idiopathic arthritis (JIA)-associated uveitis is characterized by insidious onset and potentially blinding chronic anterior uveitis. Periodic ophthalmologic screening is of utmost importance for early diagnosis of uveitis. Early diagnosis and proper immunomodulatory treatment are essential for good visual progno...

Correlation of Seminal Plasma Total Antioxidant Capacity and Malondialdehyde Levels With Sperm Parameters in Men With Idiopathic Infertility

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Fazeli

2016-01-01

Full Text Available Background Oxidative stress is the result of an imbalance between the production and scavenging of reactive oxygen species (ROS. Recently, oxidative stress has been introduced as a major cause of male infertility. Objectives The aim of the present study was to determine the correlation between total antioxidant capacity (TAC and malondialdehyde (MDA as markers of oxidative stress in relation to idiopathic male infertility and sperm parameters. Patients and Methods This case control study was conducted using 35 men with idiopathic infertility and 34 men with proven fertility. Seminal plasma TAC and MDA were measured by ferric reducing ability of plasma (FRAP and thiobarbituric acid (TBA reaction methods, respectively. Results Seminal TAC levels were significantly lower and seminal MDA levels were significantly higher in men with idiopathic infertility than in fertile men (P < 0.0001 and P = 0.004, respectively. A positive correlation was shown between sperm motility, sperm morphology, and TAC levels in men with idiopathic infertility (P = 0.002 and P = 0.002, respectively. In addition, there was a correlation between sperm motility and TAC levels in fertile men (P = 0.005. There was no correlation between sperm count and TAC levels in either men with idiopathic infertility or in fertile men. Negative correlations were observed between MDA levels and sperm motility, morphology, and sperm count only in men with idiopathic infertility (P = 0.003, P = 0.001, and P = 0.006, respectively. Conclusions Our results show that oxidative stress could play an important role in male infertility as well as in sperm motility and sperm morphology.

Idiopathic Transverse Myelitis and Neuromyelitis Optica: Clinical Profiles, Pathophysiology and Therapeutic Choices

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Awad, Amer; Stüve, Olaf

2011-01-01

Transverse myelitis is a focal inflammatory disorder of the spinal cord which may arise due to different etiologies. Transverse myelitis may be idiopathic or related/secondary to other diseases including infections, connective tissue disorders and other autoimmune diseases. It may be also associated with optic neuritis (neuromyelitis optica), which may precede transverse myelitis. In this manuscript we review the pathophysiology of different types of transverse myelitis and neuromyelitis optica and discuss diagnostic criteria for idiopathic transverse myelitis and risk of development of multiple sclerosis after an episode of transverse myelitis. We also discuss treatment options including corticosteroids, immunosuppressives and monoclonal antibodies, plasma exchange and intravenous immunoglobulins. PMID:22379456

Anti-TNF therapy for juvenile idiopathic arthritis-related uveitis

Science.gov (United States)

Semeraro, Francesco; Arcidiacono, Barbara; Nascimbeni, Giuseppe; Angi, Martina; Parolini, Barbara; Costagliola, Ciro

2014-01-01

Juvenile idiopathic arthritis-related uveitis is the most common type of uveitis in childhood and one of the main causes of visual impairment in children. The introduction of biological treatment has widened the range of therapeutic options for children with uveitis refractory to standard nonbiologic immunosuppressants. Data from clinical trials suggest that both adalimumab and infliximab have demonstrated effectiveness and safety in open-label studies, although no large, randomized, controlled trials have been reported so far. The role of etanercept in treating juvenile idiopathic arthritis-related uveitis is not yet well defined. In our experience, anti-tumor necrosis factor therapy has been shown to be more effective than steroids and/or methotrexate in treating uveitis. Up to now, tumor necrosis factor blocking compounds have been reserved for the treatment of the most severe cases of refractory uveitis, and larger prospective clinical trials are required in order to better assess the safety of these new compounds. PMID:24711694

Anti-TNF therapy for juvenile idiopathic arthritis-related uveitis.

Science.gov (United States)

Semeraro, Francesco; Arcidiacono, Barbara; Nascimbeni, Giuseppe; Angi, Martina; Parolini, Barbara; Costagliola, Ciro

2014-01-01

Juvenile idiopathic arthritis-related uveitis is the most common type of uveitis in childhood and one of the main causes of visual impairment in children. The introduction of biological treatment has widened the range of therapeutic options for children with uveitis refractory to standard nonbiologic immunosuppressants. Data from clinical trials suggest that both adalimumab and infliximab have demonstrated effectiveness and safety in open-label studies, although no large, randomized, controlled trials have been reported so far. The role of etanercept in treating juvenile idiopathic arthritis-related uveitis is not yet well defined. In our experience, anti-tumor necrosis factor therapy has been shown to be more effective than steroids and/or methotrexate in treating uveitis. Up to now, tumor necrosis factor blocking compounds have been reserved for the treatment of the most severe cases of refractory uveitis, and larger prospective clinical trials are required in order to better assess the safety of these new compounds.

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

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

2017-01-01

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

Adult-onset photosensitivity: clinical significance and epilepsy syndromes including idiopathic (possibly genetic) photosensitive occipital epilepsy.

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Koutroumanidis, Michalis; Tsirka, Vasiliki; Panayiotopoulos, Chrysostomos

2015-09-01

To evaluate the clinical associations of adult-onset photosensitivity, we studied the clinical and EEG data of patients who were referred due to a possible first seizure and who had a photoparoxysmal response on their EEG. Patients with clinical evidence of photosensitivity before the age of 20 were excluded. Of a total of 30 patients, four had acute symptomatic seizures, two had vasovagal syncope, and 24 were diagnosed with epilepsy. Nine of the 24 patients had idiopathic (genetic) generalized epilepsies and predominantly generalized photoparoxysmal response, but also rare photically-induced seizures, while 15 had exclusively, or almost exclusively, reflex photically-induced occipital seizures with frequent secondary generalization and posterior photoparoxysmal response. Other important differences included a significantly older age at seizure onset and paucity of spontaneous interictal epileptic discharges in patients with photically-induced occipital seizures; only a quarter of these had occasional occipital spikes, in contrast to the idiopathic (genetic) generalized epilepsy patients with typically generalized epileptic discharges. On the other hand, both groups shared a positive family history of epilepsy, common seizure threshold modulators (such as tiredness and sleep deprivation), normal neurological examination and MRI, a generally benign course, and good response to valproic acid. We demonstrated that photosensitivity can first occur in adult life and manifest, either as idiopathic (possibly genetic) photosensitive occipital epilepsy with secondary generalization or as an EEG, and less often, a clinical/EEG feature of idiopathic (genetic) generalized epilepsies. Identification of idiopathic photosensitive occipital epilepsy fills a diagnostic gap in adult first-seizure epileptology and is clinically important because of its good response to antiepileptic drug treatment and fair prognosis.

Idiopathic focal epilepsies: the "lost tribe".

Science.gov (United States)

Pal, Deb K; Ferrie, Colin; Addis, Laura; Akiyama, Tomoyuki; Capovilla, Giuseppe; Caraballo, Roberto; de Saint-Martin, Anne; Fejerman, Natalio; Guerrini, Renzo; Hamandi, Khalid; Helbig, Ingo; Ioannides, Andreas A; Kobayashi, Katsuhiro; Lal, Dennis; Lesca, Gaetan; Muhle, Hiltrud; Neubauer, Bernd A; Pisano, Tiziana; Rudolf, Gabrielle; Seegmuller, Caroline; Shibata, Takashi; Smith, Anna; Striano, Pasquale; Strug, Lisa J; Szepetowski, Pierre; Valeta, Thalia; Yoshinaga, Harumi; Koutroumanidis, Michalis

2016-09-01

The term idiopathic focal epilepsies of childhood (IFE) is not formally recognised by the ILAE in its 2010 revision (Berg et al., 2010), nor are its members and boundaries precisely delineated. The IFEs are amongst the most commonly encountered epilepsy syndromes affecting children. They are fascinating disorders that hold many "treats" for both clinicians and researchers. For example, the IFEs pose many of the most interesting questions central to epileptology: how are functional brain networks involved in the manifestation of epilepsy? What are the shared mechanisms of comorbidity between epilepsy and neurodevelopmental disorders? How do focal EEG discharges impact cognitive functioning? What explains the age-related expression of these syndromes? Why are EEG discharges and seizures so tightly locked to slow-wave sleep? In the last few decades, the clinical symptomatology and the respective courses of many IFEs have been described, although they are still not widely appreciated beyond the specialist community. Most neurologists would recognise the core syndromes of IFE to comprise: benign epilepsy of childhood with centro-temporal spikes or Rolandic epilepsy (BECTS/RE); Panayiotopoulos syndrome; and the idiopathic occipital epilepsies (Gastaut and photosensitive types). The Landau-Kleffner syndrome and the related (idiopathic) epilepsy with continuous spikes and waves in sleep (CSWS or ESES) are also often included, both as a consequence of the shared morphology of the interictal discharges and their potential evolution from core syndromes, for example, CSWS from BECTS. Atypical benign focal epilepsy of childhood also has shared electro-clinical features warranting inclusion. In addition, a number of less well-defined syndromes of IFE have been proposed, including benign childhood seizures with affective symptoms, benign childhood epilepsy with parietal spikes, benign childhood seizures with frontal or midline spikes, and benign focal seizures of adolescence. The

Idiopathic urolithiasis in Tunisian children: A report of 134 cases

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A Alaya

2013-01-01

Full Text Available We evaluated the metabolic and the nutritional aspects of 134 urolithiasis children in order to outline the characteristics of idiopathic urolithiasis in children. This prospective study group of 134 children (56 females, 78 males with renal calculi was evaluated. The age range of the patients was six months to 16 years. A dietary survey was performed on every child. All patients were investigated with respect to stone localization and serum and urine risk factors. Statistical analysis of data was carried out using software SPSS 11.0 for Windows. Hypercalciuria was the most common risk factor detected in this group (28.3%. A decrease of water intake was noted in all age groups, especially in the rural area (549.6 mL/day vs. 1150.6 mL/day, and there was an increase in animal protein intake in 17 cases (mean: 1.9 g/kg. In addition, increased intake of starchy foods and food with high oxalate content (sorgum were detected in the ten to 16 years age group (51% of our study. Calcium oxalate monohydrate represents the principal component of idiopathic stone (58.2%, which is more frequent in children (68% than in infants (51.7% (P <0.02. The major etiology of idiopathic urolithiasis highlights the influence of dietary habit in stone formers in our country. The increased occurrence of calcium oxalate stones in school age children confirms the change in the etiology of urolithiasis according to age.

Analysis of T Cell Subsets in Adult Primary/Idiopathic Minimal Change Disease: A Pilot Study

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Francisco Salcido-Ochoa

2017-01-01

Full Text Available Aim. To characterise infiltrating T cells in kidneys and circulating lymphocyte subsets of adult patients with primary/idiopathic minimal change disease. Methods. In a cohort of 9 adult patients with primary/idiopathic minimal change recruited consecutively at disease onset, we characterized (1 infiltrating immune cells in the kidneys using immunohistochemistry and (2 circulating lymphocyte subsets using flow cytometry. As an exploratory analysis, association of the numbers and percentages of both kidney-infiltrating immune cells and the circulating lymphocyte subsets with kidney outcomes including deterioration of kidney function and proteinuria, as well as time to complete clinical remission up to 48 months of follow-up, was investigated. Results. In the recruited patients with primary/idiopathic minimal change disease, we observed (a a dominance of infiltrating T helper 17 cells and cytotoxic cells, comprising cytotoxic T cells and natural killer cells, over Foxp3+ Treg cells in the renal interstitium; (b an increase in the circulating total CD8+ T cells in peripheral blood; and (c an association of some of these parameters with kidney function and proteinuria. Conclusions. In primary/idiopathic minimal change disease, a relative numerical dominance of effector over regulatory T cells can be observed in kidney tissue and peripheral blood. However, larger confirmatory studies are necessary.

Lung microbiome and disease progression in idiopathic pulmonary fibrosis: an analysis of the COMET study.

Science.gov (United States)

Han, MeiLan K; Zhou, Yueren; Murray, Susan; Tayob, Nabihah; Noth, Imre; Lama, Vibha N; Moore, Bethany B; White, Eric S; Flaherty, Kevin R; Huffnagle, Gary B; Martinez, Fernando J

2014-07-01

The role of the lung microbiome in the pathogenesis of idiopathic pulmonary fibrosis is unknown. We investigated whether unique microbial signatures were associated with progression of idiopathic pulmonary fibrosis. Patients (aged 35-80 years) with idiopathic pulmonary fibrosis within 4 years of diagnosis from the Correlating Outcomes with biochemical Markers to Estimate Time-progression (COMET) in idiopathic pulmonary fibrosis study were followed up for a maximum of 80 weeks. Progression-free survival was defined as time to death, acute exacerbation, lung transplant, or decrease in forced vital capacity (FVC) of 10% or greater or decrease in diffusion capacity of the lung (DLCO) of 15% or greater. DNA was isolated from 55 samples of bronchoscopic alveolar lavage. 454 pyrosequencing was used to assign operational taxonomic units (OTUs) to bacteria based on a 3% sequence divergence. Adjusted Cox models were used to identify OTUs that were significantly associated with progression-free survival at a pidiopathic pulmonary fibrosis is associated with the presence of specific members within the Staphylococcus and Streptococcus genera. Additional research will be needed to identify the specific bacterial species and to ascertain whether this is a causal association. National Institutes of Health. Copyright © 2014 Elsevier Ltd. All rights reserved.

Eisenmenger syndrome and idiopathic pulmonary arterial hypertension: do parenchymal lung changes reflect aetiology?

International Nuclear Information System (INIS)

Griffin, N.; Allen, D.; Wort, J.; Rubens, M.; Padley, S.

2007-01-01

Aim: To document the pulmonary vascular changes on thin-section computed tomography (CT) in patients with Eisenmenger syndrome and idiopathic pulmonary arterial hypertension, and to determine whether there is any correlation with pulmonary arterial pressures or the aetiology of pulmonary hypertension. Material and methods: From the National Pulmonary Hypertension Database, we identified eight patients with idiopathic pulmonary arterial hypertension and 20 patients with Eisenmenger syndrome (secondary to a ventriculoseptal defect) who had also undergone contrast-enhanced thin-section CT. CT studies were reviewed for the presence of centrilobular nodules, mosaicism, neovascularity, and bronchial artery hypertrophy. Haemodynamic data were also reviewed. Results: Centrilobular nodules, mosaicism, and neovascularity were seen in both patient groups (p > 0.05). A significantly higher number of enlarged bronchial arteries were seen in patients with Eisenmenger syndrome. There was no correlation with pulmonary arterial pressures. Conclusion: Patients with idiopathic pulmonary arterial hypertension and Eisenmenger syndrome demonstrated similar pulmonary vascular changes on CT. These changes did not predict the underlying cause of pulmonary hypertension or its severity

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

Science.gov (United States)

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

2015-01-01

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

Eisenmenger syndrome and idiopathic pulmonary arterial hypertension: do parenchymal lung changes reflect aetiology?

Energy Technology Data Exchange (ETDEWEB)

Griffin, N. [Royal Brompton and Harefield NHS Trust, London (United Kingdom)]. E-mail: nyreegriffin@hotmail.com; Allen, D. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Wort, J. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Rubens, M. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Padley, S. [Royal Brompton and Harefield NHS Trust, London (United Kingdom)

2007-06-15

Aim: To document the pulmonary vascular changes on thin-section computed tomography (CT) in patients with Eisenmenger syndrome and idiopathic pulmonary arterial hypertension, and to determine whether there is any correlation with pulmonary arterial pressures or the aetiology of pulmonary hypertension. Material and methods: From the National Pulmonary Hypertension Database, we identified eight patients with idiopathic pulmonary arterial hypertension and 20 patients with Eisenmenger syndrome (secondary to a ventriculoseptal defect) who had also undergone contrast-enhanced thin-section CT. CT studies were reviewed for the presence of centrilobular nodules, mosaicism, neovascularity, and bronchial artery hypertrophy. Haemodynamic data were also reviewed. Results: Centrilobular nodules, mosaicism, and neovascularity were seen in both patient groups (p > 0.05). A significantly higher number of enlarged bronchial arteries were seen in patients with Eisenmenger syndrome. There was no correlation with pulmonary arterial pressures. Conclusion: Patients with idiopathic pulmonary arterial hypertension and Eisenmenger syndrome demonstrated similar pulmonary vascular changes on CT. These changes did not predict the underlying cause of pulmonary hypertension or its severity.

The Role of Diffusion-Weighted MRI in Differentiation of Idiopathic Acute Transverse Myelitis and Acute Spinal Cord Infarction

International Nuclear Information System (INIS)

Kim, Yeo Goon; Lee, Joon Woo; Kim, Jae Hyoung; Kang, Heung Sik; Park, Kyung Seok

2011-01-01

To compare the diffusion characteristics of idiopathic acute transverse myelitis (ATM) and acute spinal cord infarction (SCI). Diffusion-weighted images (DWI) and an apparent diffusion coefficient (ADC) map were prospectively obtained from patients diagnosed with myelopathy between February 2006 and April 2009. Inclusion criteria included 1) the presence of an intramedullary T2-high signal intensity and 2) a final diagnosis of idiopathic ATM or SCI established by one neurologist. In total, 13 patients (M : F = 8 : 5; mean age, 39.5 years; range, 29-50 years) with idiopathic ATM and seven patients (M : F = 2 : 5; mean age, 58 years; range, 48-75 years) with SCI were included in this study. Two radiologists evaluated the DWIs and ADC map in consensus. The extent of the cord signal change was also evaluated on T2-weighted sagittal images. Among the 16 patients with ATM, 14 patients showed iso-signal on an ADC map, but one case showed restricted diffusion and another showed increased diffusion on the ADC map. Among the seven patients with SCI, five patients showed restricted diffusion. Idiopathic ATM usually does not demonstrate restricted diffusion, which can be a clue to differentiate it from SCI. However, idiopathic ATM with larger segment involvement can show focal diffusion restriction.

The spread of true and false news online.

Science.gov (United States)

Vosoughi, Soroush; Roy, Deb; Aral, Sinan

2018-03-09

We investigated the differential diffusion of all of the verified true and false news stories distributed on Twitter from 2006 to 2017. The data comprise ~126,000 stories tweeted by ~3 million people more than 4.5 million times. We classified news as true or false using information from six independent fact-checking organizations that exhibited 95 to 98% agreement on the classifications. Falsehood diffused significantly farther, faster, deeper, and more broadly than the truth in all categories of information, and the effects were more pronounced for false political news than for false news about terrorism, natural disasters, science, urban legends, or financial information. We found that false news was more novel than true news, which suggests that people were more likely to share novel information. Whereas false stories inspired fear, disgust, and surprise in replies, true stories inspired anticipation, sadness, joy, and trust. Contrary to conventional wisdom, robots accelerated the spread of true and false news at the same rate, implying that false news spreads more than the truth because humans, not robots, are more likely to spread it. Copyright © 2018 The Authors, some rights reserved; exclusive licensee American Association for the Advancement of Science. No claim to original U.S. Government Works.

Idiopathic pulmonary fibrosis

Directory of Open Access Journals (Sweden)

Noble Paul W

2008-03-01

Full Text Available Abstract Idiopathic pulmonary fibrosis (IPF is a non-neoplastic pulmonary disease that is characterized by the formation of scar tissue within the lungs in the absence of any known provocation. IPF is a rare disease which affects approximately 5 million persons worldwide. The prevalence is estimated to be slightly greater in men (20.2/100,000 than in women (13.2/100,000. The mean age at presentation is 66 years. IPF initially manifests with symptoms of exercise-induced breathless and dry coughing. Auscultation of the lungs reveals early inspiratory crackles, predominantly located in the lower posterior lung zones upon physical exam. Clubbing is found in approximately 50% of IPF patients. Cor pulmonale develops in association with end-stage disease. In that case, classic signs of right heart failure may be present. Etiology remains incompletely understood. Some environmental factors may be associated with IPF (cigarette smoking, exposure to silica and livestock. IPF is recognized on high-resolution computed tomography by peripheral, subpleural lower lobe reticular opacities in association with subpleural honeycomb changes. IPF is associated with a pathological lesion known as usual interstitial pneumonia (UIP. The UIP pattern consists of normal lung alternating with patches of dense fibrosis, taking the form of collagen sheets. The diagnosis of IPF requires correlation of the clinical setting with radiographic images and a lung biopsy. In the absence of lung biopsy, the diagnosis of IPF can be made by defined clinical criteria that were published in guidelines endorsed by several professional societies. Differential diagnosis includes other idiopathic interstitial pneumonia, connective tissue diseases (systemic sclerosis, polymyositis, rheumatoid arthritis, forme fruste of autoimmune disorders, chronic hypersensitivity pneumonitis and other environmental (sometimes occupational exposures. IPF is typically progressive and leads to significant

Imaging diagnosis--positive contrast peritoneographic features of true diaphragmatic hernia.

Science.gov (United States)

Choi, Jihye; Kim, Hyunwook; Kim, Mieun; Yoon, Junghee

2009-01-01

A true diaphragmatic hernia is a congenital diaphragmatic malformation that can appear identical to a peritoneopericardial diaphragmatic hernia (PPDH). True diaphragmatic hernias are rare in dogs. Herein we describe the use of positive contrast peritoneography for diagnosis of a true diaphragmatic hernia in two dogs.

Spring Chinook Salmon Interactions Indices and Residual/Precocial Monitoring in the Upper Yakima Basin; Yakima/Klickitat Fisheries Project Monitoring and Evaluation Report 5 of 7, 2003-2004 Annual Report.

Energy Technology Data Exchange (ETDEWEB)

Pearsons, Todd N.; Johnson, Christopher L.; James, Brenda B. (Washington Department of Fish and Wildlife, Olympia, WA)

2004-05-01

This report examines some of the factors that can influence the success of supplementation, which is currently being tested in the Yakima Basin using upper Yakima stock of spring chinook salmon. Supplementation success in the Yakima Basin is defined relative to four topic areas: natural production, genetics, ecological interactions, and harvest (Busack et al. 1997). The success of spring chinook salmon supplementation in the Yakima Basin is dependent, in part, upon fish culture practices and favorable physical and biological conditions in the natural environment (Busack et al. 1997; James et al. 1999; Pearsons et al., 2003). Shortfalls in either of these two topics (i.e., failure in culturing many fish that have high long-term fitness or environmental conditions that constrain spring chinook salmon production) will cause supplementation success to be limited. For example, inadvertent selection or propagation of spring chinook that residualize or precocially mature may hinder supplementation success. Spring chinook salmon that residualize (do not migrate during the normal migration period) may have lower survival rates than migrants and, additionally, may interact with wild fish and cause unacceptable impacts to non-target taxa. Large numbers of precocials (nonanadromous spawners) may increase competition for females and significantly skew ratios of offspring sired by nonanadromous males, which could result in more nonanadromous spring chinook in future generations. Conditions in the natural environment may also limit the success of spring chinook supplementation. For example, intra or interspecific competition may constrain spring chinook salmon production. Spring chinook salmon juveniles may compete with each other for food or space or compete with other species that have similar ecological requirements. Monitoring of spring chinook salmon residuals, precocials, prey abundance, carrying capacity, and competition will help researchers interpret why supplementation

[Idiopathic scrotal elephantiasis. A case report].

Science.gov (United States)

López-Caballero, Ignacio; Sánchez-Ruvalcaba, Itzel; Sánchez-Martinez, Luis Carlos; Hernández-Ordoñez, Octavio; Gómez-Lara, Miguel; Flores-Carrillo, Víctor

2014-01-01

Penoscrotal lymphedema (scrotal elephantiasis) is a condition that has been described in areas in which filariasis is endemic. This paper presents a case of a 45-year-old man with idiopathic lymphedema isolated to the scrotum. After acquired causes of lymphedema were ruled out, the patient was treated with scrotectomy and penoscrotal reconstruction. Currently, the patient is receiving follow-up care without evidence of recurrence. Penoscrotal lymphedema may cause symptoms of weakness, immobility and emotional disturbance. Surgery procedure provides a satisfactory cosmetical and functional outcome.

Body composition in children with juvenile idiopathic arthritis: effect ...

African Journals Online (AJOL)

Introduction: the aim of this study was to evaluate the relationship between macronutrient intake, body composition (lean body mass and fat mass) and bone mineral content in Moroccan children with juvenile idiopathic arthritis (JIA). Methods: a cross-sectional study, conducted between May 2010 and June 2011, covering ...

Hyperoxaluria in idiopathic calcium nephrolithiasis--what are the limits?

DEFF Research Database (Denmark)

Osther, P J

1999-01-01

OBJECTIVE: The object of this study was to investigate the role for measurement of 24-h renal oxalate excretion in the evaluation of idiopathic calcium stone formers. MATERIALS AND METHODS: Renal excretion rates of oxalate and creatinine were measured in 24-h urines in 46 consecutive male recurrent...

Idiopathic sclerosing encapsulating peritonitis (or abdominal cocoon).

Science.gov (United States)

Serafimidis, Costas; Katsarolis, Ioannis; Vernadakis, Spyros; Rallis, George; Giannopoulos, George; Legakis, Nikolaos; Peros, George

2006-02-13

Idiopathic sclerosing encapsulating peritonitis (or abdominal cocoon) is a rare cause of small bowel obstruction, especially in adult population. Diagnosis is usually incidental at laparotomy. We discuss one such rare case, outlining the fact that an intra-operative surprise diagnosis could have been facilitated by previous investigations. A 56 year-old man presented in A&E department with small bowel ileus. He had a history of 6 similar episodes of small bowel obstruction in the past 4 years, which resolved with conservative treatment. Pre-operative work-up did not reveal any specific etiology. At laparotomy, a fibrous capsule was revealed, in which small bowel loops were encased, with the presence of interloop adhesions. A diagnosis of abdominal cocoon was established and extensive adhesiolysis was performed. The patient had an uneventful recovery and follow-up. Idiopathic sclerosing encapsulating peritonitis, although rare, may be the cause of a common surgical emergency such as small bowel ileus, especially in cases with attacks of non-strangulating obstruction in the same individual. A high index of clinical suspicion may be generated by the recurrent character of small bowel ileus combined with relevant imaging findings and lack of other plausible etiologies. Clinicians must rigorously pursue a preoperative diagnosis, as it may prevent a "surprise" upon laparotomy and result in proper management.

Risk Factors for Neurodegeneration in Idiopathic REM sleep Behavior Disorder: A Multicenter Study

Science.gov (United States)

Postuma, RB; Iranzo, A; Hogl, B; Arnulf, I; Ferini-Strambi, L; Manni, R; Miyamoto, T.; Oertel, W; Dauvilliers, Y; Ju, Y; Puligheddu, M; Sonka, K; Pelletier, A; Santamaria, J; Frauscher, B; Leu-Semenescu, S; Zucconi, M; Terzaghi, M; Miyamoto, M.; Unger, MM; Carlander, B; Fantini, ML; Montplaisir, JY

2018-01-01

Objective To assess whether risk factors for Parkinson’s disease and Dementia with Lewy bodies increase rate of defined neurodegenerative disease in idiopathic REM sleep behavior disorder Methods 12 centers administered a detailed questionnaire assessing risk factors for neurodegenerative synucleinopathy to patients with idiopathic REM sleep behavior disorder. Variables included demographics, lifestyle factors, pesticide exposures, occupation, co-morbid conditions, medication use, family history, and autonomic/motor symptoms. After 4-years follow-up, patients were assessed for dementia or parkinsonism. Disease risk was assessed with Kaplan-Meier analysis, and epidemiologic variables were compared between convertors and those still idiopathic using logistic regression. Results Of 305 patients, follow-up information was available for 279, of whom 93 (33.3%) developed defined neurodegenerative disease. Disease risk was 25% at 3 years, and 41% after 5 years. Patients who converted were older (difference=4.5 years, pconversion. Although occupation was similar between groups, those who converted had a lower likelihood of pesticide exposure (occupational insecticide=2.3% vs. 9.0%). Convertors were more likely to report family history of dementia (OR=2.09), without significant differences in Parkinson’s disease or sleep disorders. Medication exposures and medical history were similar between groups. Autonomic and motor symptoms were more common among those who converted. Risk factors for primary dementia and parkinsonism were generally similar, except for a notably higher clonazepam use in dementia convertors (OR=2.6). Interpretation Patients with idiopathic RBD are at very high risk of neurodegenerative synucleinopathy. Risk factor profiles between convertors and non-convertors have both important commonalities and differences. PMID:25767079

Manual therapy as a conservative treatment for adolescent idiopathic scoliosis: a systematic review.

Science.gov (United States)

Romano, Michele; Negrini, Stefano

2008-01-22

The treatment of adolescent idiopathic scoliosis is contingent upon many variables. Simple observation is enough for less serious curvatures, but for very serious cases surgical intervention could be proposed. Between these there is a wide range of different treatments. Manual therapy is commonly used: the aim of this paper is to verify the data existing in the literature on the efficacy of this approach. A systematic review of the scientific literature published internationally has been performed. We have included in the term manual therapy all the manipulative and generally passive techniques performed by an external operator. In a more specific meaning, osteopathic, chiropractic and massage techniques have been considered as manipulative therapeutic methods. We performed our systematic research in Medline, Embase, Cinhal, Cochrane Library, Pedro with the following terms: idiopathic scoliosis combined with chiropractic; manipulation; mobilization; manual therapy; massage; osteopathy; and therapeutic manipulation. The criteria for inclusion were as follows: Any kind of research; diagnosis of adolescent idiopathic scoliosis; patients treated exclusively by one of the procedures established as a standard for this review (chiropractic manipulation, osteopathic techniques, massage); and outcome in Cobb degrees. We founded 145 texts, but only three papers were relevant to our study. However, no one of the three satisfied all the required inclusion criteria because they were characterized by a combination of manual techniques and other therapeutic approaches. The lack of any kind of serious scientific data does not allow us to draw any conclusion on the efficacy of manual therapy as an efficacious technique for the treatment of Adolescent idiopathic scoliosis.

Hippocampal size is related to short-term true and false memory, and right fusiform size is related to long-term true and false memory.

Science.gov (United States)

Zhu, Bi; Chen, Chuansheng; Loftus, Elizabeth F; He, Qinghua; Lei, Xuemei; Dong, Qi; Lin, Chongde

2016-11-01

There is a keen interest in identifying specific brain regions that are related to individual differences in true and false memories. Previous functional neuroimaging studies showed that activities in the hippocampus, right fusiform gyrus, and parahippocampal gyrus were associated with true and false memories, but no study thus far has examined whether the structures of these brain regions are associated with short-term and long-term true and false memories. To address that question, the current study analyzed data from 205 healthy young adults, who had valid data from both structural brain imaging and a misinformation task. In the misinformation task, subjects saw the crime scenarios, received misinformation, and took memory tests about the crimes an hour later and again after 1.5 years. Results showed that bilateral hippocampal volume was associated with short-term true and false memories, whereas right fusiform gyrus volume and surface area were associated with long-term true and false memories. This study provides the first evidence for the structural neural bases of individual differences in short-term and long-term true and false memories.

A new look at the etiopathogenesis ofadolescent idiopathic scoliosis

Directory of Open Access Journals (Sweden)

Maciej Brzęczek

2016-06-01

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

MANAGEMENT OF CHRONIC IDIOPATHIC THROMBOTIC THROMBOCYTOPENIC PURPURA WITH SPLENECTOMY

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Mojca Modic

2002-07-01

Full Text Available Background. Treatment of thrombotic thrombocytopenic purpura (TTP with plasma exchange (PE has reduced mortality rates from 90% in untreated patients to less than 20%. Despite primary effective PE, relapses occur in up to 40% of patients. Beside PE, second line treatment options are corticosteroids, vincristine, defibrotide, prostacycline. We describe two patients with relapsing TTP treated successfully with splenectomy.Patients and methods. Case 1. A 20–year female was diagnosed in October 2000 with idiopathic chronic relapsing TTP. First episode of PE plus methylprednisolone treatment led to complet remission. Subsequent relapses occured 3, 6 and 8 months after first TTP episode. Every relaps was effectivelly treated with PE and methylprednisolone. Because of more and more frequent TTP relapses laparoscopic splenectomy was performed. Currently, 7 months after splenectomy she is in complet TTP remission.Case 2. A 18–year female, was diagnosed in January 1997 with idiopathic TTP. After PE and methylprednisolone complete remission was established. Relaps occured after nearly three years. Later on, TTP got chronic course with neurological clinical symptoms and plateled drops as soon as PE treatment was withdrawn. Besides PE and methylprednisolone she received also defibrotide. Because of chronic, PE dependant TTP, laparoscopic splenectomy was performed. Today, 15 months after splenectomy she is in complete TTP remission.Conclusions. In idiopathic relapsing and chronic TTP, PE treatment only is usually not sufficient for durable remission. Second line TTP treatment drugs have limited efficacy. Based on our experience in patients with relapsing and/or chronic TTP, laparoscopic splenectomy should be considered as a first treatment option, especialy in junger patients.

True ternary fission of 252Cf

International Nuclear Information System (INIS)

Vijayaraghavan, K.R.; Balasubramaniam, M.; Oertzen, W. von

2014-01-01

Splitting of heavy radioactive nucleus into three fragments is known as ternary fission. If the size of the fragments are almost equal it is referred to as true ternary fission. Recently, Yu. V. Pyatkov et al observed/reported the experimental observation of true ternary fission in 252 Cf. In this work, the possibilities of different true ternary fission modes of 252 Cf through potential energy surface (PES) calculations based on three cluster model (TCM) are discussed. In TCM a condition on the mass numbers of the fission fragments is implied as A 1 ≥ A 2 ≥ A 3 in order to avoid repetition of combinations. Due to this condition, the values of Z 3 vary from 0 to 36 and Z 2 vary from 16 to 51. Of the different pairs having similar (Z 2 , Z 3 ) with different potential energy, a pair possessing minimum potential energy is chosen. Thus identified favourable combinations are plotted. For the PES calculations the arrangement of the fragments is considered in the order of A 1 +A 2 +A 3 . i.e. the heavy and the lightest fragments are kept at the ends. It is seen that the deepest minimum in the PES occurs for Z 3 =2 labelled as (Z 2 ; 2) indicating He accompanied breakup as the most favourable one. Of which, the breakup with Z 2 around 46 to 48 is the least (shown by dashed (Z 1 = 50) and dotted (Z 1 = 52) lines indicating a constant Z 1 value). The other notable minima in the PES are labelled and they correspond to the (Z 2 , Z 3 ) pairs viz., (20, 20), (28, 20), (28, 28) and (32, 32). Of these four minima, the first three are associated with the magic numbers 20 and 28. For Z 3 =20, there are two minimums at (20,20) and (28,20) among them (28,20) is the lowest minimum through which the minimum-path passes, and it is the ternary decay observed by Yu. V. Pyatkov et al. The fourth minima is the most interesting due to the fact that it corresponds to true ternary fission mode with Z 2 =32, Z 3 =32 and Z 1 =34. The minimum potential energy path also goes through this true

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

Science.gov (United States)

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

2015-04-01

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

MR-Venography Using High Resolution True-FISP

Energy Technology Data Exchange (ETDEWEB)

Spuentrup, E. [Technische Hochschule Aachen (Germany). Klinik fuer Radiologische Diagnostik; Beth Israel Deaconess Medical Center, Boston, MA (United States). Dept. of Medicine; Harvard Medical School, Boston, MA (United States); Buecker, A.; Guenther, R.W. [Technische Hochschule Aachen (Germany). Klinik fuer Radiologische Diagnostik; Stuber, M. [Beth Israel Deaconess Medical Center, Boston, MA (United States). Dept. of Medicine; Harvard Medical School, Boston, MA (United States); Philips Med. Syst., Best (Netherlands)

2001-08-01

A new fast MR-venography approach using a high resolution True-FISP imaging sequence was investigated in 20 patients suffering from 23 deep vein thromboses. Diagnosis was proven by X-ray venography, CT or ultrasound examination. The presented technique allowed for clear thrombus visualization with a high contrast to the surrounding blood pool even in calf veins. Acquisition time was less than 10 minutes for imaging the pelvis and the legs. No contrast media was needed. The presented high resolution True-FISP MR-veography is a promising non-invasive, fast MR-venography approach for detection of deep venous thrombosis. (orig.) [German] Eine neue schnelle, oertlich hochaufgeloeste MR-Phlebographietechnik mit einer axialen True-FISP Bildgebungssequenz wurde an 20 Patienten mit 23 nach-gewiesenen tiefen Beinvenenthrombosen untersucht. Die Befunde wurden mit einer konventionellen Roentgenphlebographie, einer CT oder einer Sonographie gesichert. Die vorgestellte Technik erlaubte in allen Faellen eine Thrombusdarstellung mit hohem Kontrast zum umgebenden venoesen Blut, wobei aufgrund der hohen Ortsaufloesung auch die Unterschenkelvenen beurteilt werden konnten. Die Datenaufnahmezeit zur Untersuchung des Beckens und der Beine betrug weniger als 10 Minuten. Kontrastmittel wurde nicht benoetigt. Die vorgestellte MR-Phlebographietechnik unter Verwendung einer oertlich hochauf-geloesten True-FISP Sequenz ist eine neue, vielversprechende, nicht-invasive Technik zur Diagnostik der tiefen Bein- und Beckenvenenthrombose. (orig.)

Pulmonary function vascular index predicts prognosis in idiopathic interstitial pneumonia

NARCIS (Netherlands)

Corte, Tamera J.; Wort, Stephen J.; MacDonald, Peter S.; Edey, Anthony; Hansell, David M.; Renzoni, Elisabetta; Maher, Toby M.; Nicholson, Andrew G.; Bandula, Steven; Bresser, Paul; Wells, Athol U.

2012-01-01

Background and objective: Pulmonary hypertension (PH) is associated with increased mortality in fibrotic idiopathic interstitial pneumonia (IIP). We hypothesize that baseline KCO (diffusing capacity of carbon monoxide/alveolar volume) and 6-month decline in KCO reflect PH, thus predicting mortality

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

Unravelling the incidence and etiology of chronic idiopathic axonal polyneuropathy

NARCIS (Netherlands)

Visser, N.A.

2016-01-01

Chronic idiopathic axonal polyneuropathy (CIAP) is a sensory or sensorimotor polyneuropathy that has a slowly progressive course without severe disability. CIAP is diagnosed in a significant proportion of patients with polyneuropathy, but precise figures on the incidence of polyneuropathy and CIAP

International Veterinary Epilepsy Task Force's current understanding of idiopathic epilepsy of genetic or suspected genetic origin in purebred dogs

DEFF Research Database (Denmark)

Hülsmeyer, Velia-Isabel; Fischer, Andrea; Mandigers, Paul J. J.

2015-01-01

Canine idiopathic epilepsy is a common neurological disease affecting both purebred and crossbred dogs. Various breed-specific cohort, epidemiological and genetic studies have been conducted to date, which all improved our knowledge and general understanding of canine idiopathic epilepsy, and in ...

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

Directory of Open Access Journals (Sweden)

Y. M. Spivakovskiy

2015-01-01

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

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