Altering Hirsutism Through Ovulation Induction in Women With Polycystic Ovary Syndrome

PubMed Central

Roth, Lauren W.; Huang, Hao; Legro, Richard S.; Diamond, Michael P.; Coutifaris, Christos; Carson, Sandra A.; Steinkampf, Michael P.; Carr, Bruce R.; McGovern, Peter G.; Cataldo, Nicholas A.; Gosman, Gabriella G.; Nestler, John E.; Myers, Evan R.; Zhang, Heping; Schlaff, William D.

2012-01-01

Objective Many women with polycystic ovary syndrome (PCOS) experience infertility and hirsutism and often seek treatment for both concurrently. We investigated whether women who ovulate in response to treatment with clomiphene citrate), metformin, or both would have greater improvement in hirsutism compared to those who did not ovulate. Methods This is a secondary analysis evaluating the change in Ferriman-Gallwey score for the hirsute women (n = 505, 80.7%) from the Pregnancy in Polycystic Ovary Syndrome 1 study. This was a prospective, randomized, doubled-blind trial of 626 women with PCOS and infertility recruited from 12 university sites. They were treated with clomiphene citrate, metformin, or both (combination) for up to six cycles, and hirsutism evaluators were blinded to group assignment. Results There was a significant decrease in the Ferriman-Gallwey score between baseline and completion of the study in each of the three individual groups (clomiphene citrate, p=0.024; metformin, p=0.005; combination, p<0.001). There was no significant difference in the degree to which the hirsutism score changed when comparing the three groups (p=0.44). The change in hirsutism was not associated with the duration of treatment or with the presence or absence of ovulation. Conclusions In infertile hirsute women with PCOS, treatment with clomiphene citrate, metformin, or both for up to 6 cycles does not alter hirsutism. Clinical Trial Registration ClinicalTrials.gov, www.clinicaltrials.gov, NCT00068861. PMID:22617579

Quality of life, mental health and self-esteem in hirsute adolescent females.

PubMed

Drosdzol, Agnieszka; Skrzypulec, Violetta; Plinta, Ryszard

2010-09-01

The aim of the study was to evaluate the influence of hirsutism on general quality of life, self-esteem and the prevalence of anxiety and depressive symptoms among adolescent girls. Fifty adolescent females with hirsutism, aged 13-18 years, were enrolled in the research group. The control group comprised 50 non-hirsute adolescents. A specific questionnaire was used as the research tool. It included self-evaluation inventories: Short Form-36 Health Survey Version 2, Hospital Anxiety and Depression Scale and Rosenberg Self-Esteem Scale. Quality of life indices for hirsute girls scored lower than for the controls and statistically significantly so with regard to physical functioning (p = 0.04), general health (p = 0.002) and social functioning (p = 0.007). Anxiety was diagnosed in 26% in the group of hirsute girls as compared with 10% of the controls (p = 0.03). The study analysis revealed more clinically significant problems of low self-esteem in hirsute adolescents compared with non-hirsute girls (14% vs. 2%). Hirsutism is associated with a decreased quality of life, a higher prevalence of anxiety disorder and lower self-esteem in adolescent females. The mother's level of education is associated with the quality of life in adolescent girls.

Alternative diagnostic criteria for idiopathic hypersomnia: A 32-hour protocol.

PubMed

Evangelista, Elisa; Lopez, Régis; Barateau, Lucie; Chenini, Sofiene; Bosco, Adriana; Jaussent, Isabelle; Dauvilliers, Yves

2018-02-01

To assess the diagnostic value of extended sleep duration on a controlled 32-hour bed rest protocol in idiopathic hypersomnia (IH). One hundred sixteen patients with high suspicion of IH (37 clear-cut IH according to multiple sleep latency test criteria and 79 probable IH), 32 with hypersomnolence associated with a comorbid disorder (non-IH), and 21 controls underwent polysomnography, modified sleep latency tests, and a 32-hour bed rest protocol. Receiver operating characteristic curves were used to find optimal total sleep time (TST) cutoff values on various periods that discriminate patients from controls. TST was longer in patients with clear-cut IH than other groups (probable IH, non-IH, and controls) and in patients with probable IH than non-IH and controls. The TST cutoff best discriminating clear-cut IH and controls was 19 hours for the 32-hour recording (sensitivity = 91.9%, specificity = 85.7%) and 12 hours (100%, 85.7%) for the first 24 hours. The 19-hour cutoff displayed a specificity and sensitivity of 91.9% and 81.2% between IH and non-IH patients. Patients with IH above the 19-hour cutoff were overweight, had more sleep inertia, and had higher TST on all periods compared to patients below 19 hours, whereas no differences were found for the 12-hour cutoff. An inverse correlation was found between the mean sleep latency and TST during 32-hour recording in IH patients. In standardized and controlled stringent conditions, the optimal cutoff best discriminating patients from controls was 19 hours over 32 hours, allowing a clear-cut phenotypical characterization of major interest for research purposes. Sleepier patients on the multiple sleep latency test were also the more severe in terms of extended sleep. Ann Neurol 2018;83:235-247. © 2018 American Neurological Association.

Social support: an important factor for quality of life in women with hirsutism.

PubMed

Ekbäck, Maria Palmetun; Lindberg, Magnus; Benzein, Eva; Årestedt, Kristofer

2014-12-20

Women with hirsutism have reported imparied health and health-related quality of life (HRQoL). Social support is a factor that might increase HRQoL in chronic diseases, but little is known about this association among women with hirsutism. The aim of the study was to describe social support and explore its association with HRQoL among women with hirsutism. A further aim was to compare HRQoL in women with hirsutism with a Swedish normal population. A questionnaire including socio-demographic questions, Short-Form Health Survey (SF-36), the Multidimensional Scale of Perceived Social Support (MSPSS), and a self-estimation of hairiness using the Ferriman-Gallway scale (F-G scale) was answered by 127 women with hirsutism. Multiple regression analyses showed significant associations between social support and all health dimensions in the SF-36, also after the model was adjusted for age, hairiness and body mass index. Compared to the normal Swedish population, women with hirsutism reported significantly lower HRQoL in all dimensions of the SF-36 (p < 0.01). There is a significant positive association between social support and HRQoL, demonstrating its importance for the ability to adapt to problems associated with hirsutism. As women with hirsutism reported poorer HRQoL compared to the normal population, social support may be a factor to consider in clinical practice.

Prevalence of functional disorders of androgen excess in unselected premenopausal women: a study in blood donors.

PubMed

Sanchón, Raúl; Gambineri, Alessandra; Alpañés, Macarena; Martínez-García, M Ángeles; Pasquali, Renato; Escobar-Morreale, Héctor F

2012-04-01

The polycystic ovary syndrome (PCOS) is one of the most common endocrine disorders in women. On the contrary, the prevalences of other disorders of androgen excess such as idiopathic hyperandrogenism and idiopathic hirsutism remain unknown. We aimed to obtain an unbiased estimate of the prevalence in premenopausal women of (i) signs of androgen excess and (ii) PCOS, idiopathic hyperandrogenism and idiopathic hirsutism. A multicenter prevalence survey included 592 consecutive premenopausal women (393 from Madrid, Spain and 199 from Bologna, Italy) reporting spontaneously for blood donation. Immediately before donation, we conducted clinical and biochemical phenotyping for androgen excess disorders. We determined the prevalence of (i) hirsutism, acne and alopecia as clinical signs of androgen excess and (ii) functional disorders of androgen excess, including PCOS, defined by the National Institute of Child Health and Human Development/National Institute of Health criteria, idiopathic hyperandrogenism and idiopathic hirsutism. Regarding clinical signs of hyperandrogenism, hirsutism and acne were equally frequent [12.2% prevalence; 95% confidence interval (CI): 9.5-14.8%], whereas alopecia was uncommon (1.7% prevalence, 95% CI: 0.7-2.7%). Regarding functional disorders of androgen excess, PCOS and idiopathic hirsutism were equally frequent (5.4% prevalence, 95% CI: 3.6-7.2) followed by idiopathic hyperandrogenism (3.9% prevalence, 95% CI: 2.3-5.4). Clinical signs of hyperandrogenism and functional disorders of androgen excess show a high prevalence in premenopausal women. The prevalences of idiopathic hyperandrogenism and idiopathic hirsutism are similar to that of PCOS, highlighting the need for further research on the pathophysiology, consequences for health and clinical implications of these functional forms of androgen excess.

A comparison of idiopathic hypersomnia and narcolepsy-cataplexy using self report measures and sleep diary data.

PubMed Central

Bruck, D; Parkes, J D

1996-01-01

Eighteen patients with idiopathic hypersomnia (IH) were compared with 50 patients with the narcoleptic syndrome of cataplexy and daytime sleepiness (NLS) using self report questionnaires and a diary of sleep/wake patterns. The IH group reported more consolidated nocturnal sleep, a lower propensity to nap, greater refreshment after naps, and a greater improvement in excessive daytime sleepiness since onset than the NLS group. In IH, the onset of excessive daytime sleepiness was predominantly associated with familial inheritance or a viral illness. Two variable--number of reported awakenings during nocturnal sleep and the reported change in sleepiness since onset--provided maximum discrimination between the IH and NLS groups. Confusional arousals, extended naps or nocturnal sleep, autonomic nervous system dysfunction, low ratings of medication effectiveness, or side effects of medication were not associated differentially with either IH or NLS. PMID:8778267

Use of subcutaneous flumazenil preparations for the treatment of idiopathic hypersomnia: A case report.

PubMed

Kelty, Erin; Martyn, Vlad; O'Neil, George; Hulse, Gary

2014-07-01

Idiopathic hypersomnia (IH) is a rare sleep disorder, recently hypothesized to be related to the production of a molecule that facilitates the binding of gamma-aminobutyric acid (GABA) to the GABA receptor. This paper reports on the treatment of a patient with IH who was treated with a 96-hour continuous low dose (4 mg/day) infusion of a benzodiazepine antagonist, flumazenil, followed by a slow-release flumazenil implant. The use of flumazenil mitigated the patient's IH symptoms including excessive daytime sleepiness, sleep drunkenness and self-reported cognitive problems. The case both provides a possible treatment and system for short (subcutaneous (SC) administration) and longer term (implant) flumazenil delivery. Current data supports the need for further research into the use of flumazenil for the treatment of IH and to develop long term flumazenil delivery systems. © The Author(s) 2014.

Effects of pitolisant, a histamine H3 inverse agonist, in drug-resistant idiopathic and symptomatic hypersomnia: a chart review.

PubMed

Leu-Semenescu, Smaranda; Nittur, Nandy; Golmard, Jean-Louis; Arnulf, Isabelle

2014-06-01

To evaluate the benefits and risks of pitolisant (a wake-enhancing drug that increases the histamine release in the brain by blocking presynaptic H3 histamine reuptake) in patients with idiopathic (IH) and symptomatic (SH) hypersomnia plus sleepiness refractory to available stimulants (modafinil, methylphenidate, mazindol, sodium oxybate, and d-amphetamine). Through retrospective analyses of patient files, the benefit (the score from the Epworth Sleepiness Scale [ESS], authorization renewal) and tolerance (side-effects) of pitolisant were assessed. A total of 78 patients with IH (n=65%, 78% women) and SH (n=13%, 54% women) received pitolisant 5-50 mg once per day over the course of five days to 37 months. The median (interquartile range) ESS scores of patients with IH decreased from 17 (15.5-18.5) to 14 (12-17). There were 36% responders (ESS fall of > or =3). The improvement in ESS score (-1.9±2.6) was different from 0 in IH without long sleep time (P<0.002) and in IH with a long sleep time (P<0.0001), but not in SH. Forty-four (63%) patients with IH and 12 (77%) patients with SH stopped pitolisant, mostly due to a lack of efficacy. Side-effects included gastrointestinal pain (15.4%), increased appetite and weight gain (14.1%), headache (12.8%), insomnia (11.5%), and anxiety (9%), as well as exceptional reports of depression and persistent genital arousal. Pitolisant had a long-term favorable benefit/risk ratio in 23-38% of drug-resistant patients with IH and SH, suggesting that histamine neurons can be stimulated in severe idiopathic and symptomatic hypersomnia. Copyright © 2014 Elsevier B.V. All rights reserved.

Polysomnographic study of nocturnal sleep in idiopathic hypersomnia without long sleep time.

PubMed

Pizza, Fabio; Ferri, Raffaele; Poli, Francesca; Vandi, Stefano; Cosentino, Filomena I I; Plazzi, Giuseppe

2013-04-01

We investigated nocturnal sleep abnormalities in 19 patients with idiopathic hypersomnia without long sleep time (IH) in comparison with two age- and sex- matched control groups of 13 normal subjects (C) and of 17 patients with narcolepsy with cataplexy (NC), the latter considered as the extreme of excessive daytime sleepiness (EDS). Sleep macro- and micro- (i.e. cyclic alternating pattern, CAP) structure as well as quantitative analysis of EEG, of periodic leg movements during sleep (PLMS), and of muscle tone during REM sleep were compared across groups. IH and NC patients slept more than C subjects, but IH showed the highest levels of sleep fragmentation (e.g. awakenings), associated with a CAP rate higher than NC during lighter sleep stages and lower than C during slow wave sleep respectively, and with the highest relative amount of A3 and the lowest of A1 subtypes. IH showed a delta power in between C and NC groups, whereas muscle tone and PLMS had normal characteristics. A peculiar profile of microstructural sleep abnormalities may contribute to sleep fragmentation and, possibly, EDS in IH. © 2012 European Sleep Research Society.

REANALYZING THE MODIFIED FERRIMAN-GALLWEY SCORE: IS THERE A SIMPLER METHOD FOR ASSESSING THE EXTENT OF HIRSUTISM?

PubMed Central

Cook, Heather; Brennan, Kathleen; Azziz, Ricardo

2011-01-01

Objective To determine whether assessing the extent of terminal hair growth in a subset of the traditional 9 areas included in the modified Ferriman-Gallwey (mFG) score can serve as a simpler predictor of total body hirsutism when compared to the full scoring system, and to determine if this new model can accurately distinguish hirsute from non-hirsute women. Design Cross-sectional analysis Setting Two tertiary care academic referral centers. Patients 1951 patients presenting for symptoms of androgen excess. Interventions History and physical examination, including mFG score. Main Outcome Measures Total body hirsutism. Results A regression model using all nine body areas indicated that the combination of upper abdomen, lower abdomen and chin was the best predictor of the total full mFG score. Using this subset of three body areas is accurate in distinguishing true hirsute from non-hirsute women when defining true hirsutism as mFG>7. Conclusion Scoring terminal hair growth only on the chin and abdomen can serve as a simple, yet reliable predictor of total body hirsutism when compared to full body scoring using the traditional mFG system. PMID:21924716

Altered dynamics in the circadian oscillation of clock genes in dermal fibroblasts of patients suffering from idiopathic hypersomnia.

PubMed

Lippert, Julian; Halfter, Hartmut; Heidbreder, Anna; Röhr, Dominik; Gess, Burkhard; Boentert, Mathias; Osada, Nani; Young, Peter

2014-01-01

From single cell organisms to the most complex life forms, the 24-hour circadian rhythm is important for numerous aspects of physiology and behavior such as daily periodic fluctuations in body temperature and sleep-wake cycles. Influenced by environmental cues - mainly by light input -, the central pacemaker in the thalamic suprachiasmatic nuclei (SCN) controls and regulates the internal clock mechanisms which are present in peripheral tissues. In order to correlate modifications in the molecular mechanisms of circadian rhythm with the pathophysiology of idiopathic hypersomnia, this study aimed to investigate the dynamics of the expression of circadian clock genes in dermal fibroblasts of idiopathic hypersomniacs (IH) in comparison to those of healthy controls (HC). Ten clinically and polysomnographically proven IH patients were recruited from the department of sleep medicine of the University Hospital of Muenster. Clinical diagnosis was done by two consecutive polysomnographies (PSG) and Multiple Sleep Latency Test (MSLT). Fourteen clinical healthy volunteers served as control group. Dermal fibroblasts were obtained via punch biopsy and grown in cell culture. The expression of circadian clock genes was investigated by semiquantitative Reverse Transcriptase-PCR qRT-PCR analysis, confirming periodical oscillation of expression of the core circadian clock genes BMAL1, PER1/2 and CRY1/2. The amplitude of the rhythmically expressed BMAL1, PER1 and PER2 was significantly dampened in dermal fibroblasts of IH compared to HC over two circadian periods whereas the overall expression of only the key transcriptional factor BMAL1 was significantly reduced in IH. Our study suggests for the first time an aberrant dynamics in the circadian clock in IH. These findings may serve to better understand some clinical features of the pathophysiology in sleep - wake rhythms in IH.

Altered Dynamics in the Circadian Oscillation of Clock Genes in Dermal Fibroblasts of Patients Suffering from Idiopathic Hypersomnia

PubMed Central

Lippert, Julian; Halfter, Hartmut; Heidbreder, Anna; Röhr, Dominik; Gess, Burkhard; Boentert, Mathias; Osada, Nani; Young, Peter

2014-01-01

From single cell organisms to the most complex life forms, the 24-hour circadian rhythm is important for numerous aspects of physiology and behavior such as daily periodic fluctuations in body temperature and sleep-wake cycles. Influenced by environmental cues – mainly by light input -, the central pacemaker in the thalamic suprachiasmatic nuclei (SCN) controls and regulates the internal clock mechanisms which are present in peripheral tissues. In order to correlate modifications in the molecular mechanisms of circadian rhythm with the pathophysiology of idiopathic hypersomnia, this study aimed to investigate the dynamics of the expression of circadian clock genes in dermal fibroblasts of idiopathic hypersomniacs (IH) in comparison to those of healthy controls (HC). Ten clinically and polysomnographically proven IH patients were recruited from the department of sleep medicine of the University Hospital of Muenster. Clinical diagnosis was done by two consecutive polysomnographies (PSG) and Multiple Sleep Latency Test (MSLT). Fourteen clinical healthy volunteers served as control group. Dermal fibroblasts were obtained via punch biopsy and grown in cell culture. The expression of circadian clock genes was investigated by semiquantitative Reverse Transcriptase-PCR qRT-PCR analysis, confirming periodical oscillation of expression of the core circadian clock genes BMAL1, PER1/2 and CRY1/2. The amplitude of the rhythmically expressed BMAL1, PER1 and PER2 was significantly dampened in dermal fibroblasts of IH compared to HC over two circadian periods whereas the overall expression of only the key transcriptional factor BMAL1 was significantly reduced in IH. Our study suggests for the first time an aberrant dynamics in the circadian clock in IH. These findings may serve to better understand some clinical features of the pathophysiology in sleep – wake rhythms in IH. PMID:24454829

What Is IH (Intracranial Hypertension)?

MedlinePlus

... Store What is IH? What is IH? Intracranial hypertension literally means that the pressure of cerebrospinal fluid ( ... is too high. “Intracranial” means “within the skull.” “Hypertension” means “high fluid pressure.” To understand how this ...

Comparison of flutamide and spironolactone in the treatment of hirsutism: a randomized controlled trial.

PubMed

Cusan, L; Dupont, A; Gomez, J L; Tremblay, R R; Labrie, F

1994-02-01

To compare the clinical efficacy and safety of the pure antiandrogen flutamide and the steroidal derivative spironolactone in the treatment of hirsutism in women. Fifty-three premenopausal women suffering from moderate to severe hirsutism were randomized into two groups and received either flutamide or spironolactone in association with a triphasic oral contraceptive (OC) pill. Hirsutism, acne, seborrhea, alopecia, and side effects were monitored monthly for a treatment period of 9 months and a follow-up after treatment period of 6 months. Blood samples were taken at each visit for assessment of endocrine, biochemical, and hematologic parameters. After only 6 months of therapy, flutamide caused a maximal reduction in the hirsutism score to a value within almost normal range; during the same period, spironolactone caused only a 30% reduction of the hirsutism score. Whereas flutamide caused a dramatic (80%) decrease in total acne, seborrhea, and hair loss score after only 3 months of therapy, spironolactone caused only a 50% reduction in acne and seborrhea, with no significant effect on the hair loss score. Four patients in the spironolactone group but only one in the flutamide group stopped the medication because of adverse side effects. The present data obtained in a randomized prospective study clearly demonstrate that the pure antiandrogen flutamide is superior to spironolactone in the treatment of female hirsutism and its related androgen-dependent symptoms and signs in women.

Long-Term Response of Hirsutism and Other Hyperandrogenic Symptoms to Combination Therapy in Polycystic Ovary Syndrome.

PubMed

Ezeh, Uche; Huang, Andy; Landay, Melanie; Azziz, Ricardo

2018-06-07

Polycystic ovary syndrome (PCOS) affects 5%-15% of women and is the most common cause of hirsutism. Data on the time-course of improvement to suppressive therapy and predictors of that response in PCOS are lacking. The objectives of our study are to determine the long-term response and identify predictors of response in PCOS women treated with suppressive therapy, including spironolactone (SPL) + oral contraceptives (OCs). Retrospective cross-sectional analysis of 200 women with PCOS (1990 NIH criteria) treated with suppressive therapy in general, and a subgroup of 138 subjects treated with OCP+SPL who had been prospectively included in a biorepository. Main outcome measure included improvement rate per 100 person-month of follow-up for hirsutism, menstrual irregularity and acne measured qualitatively as "feeling better", and changes in the severity of hirsutism quantified by modified Ferriman-Gallwey [mF-G] score. During a mean follow-up of 34.2 months, 85.1%, 82.7%, and 79.3% of patients reported improvement in hirsutism, menstrual dysfunction, and acne, respectively. The modified Ferriman-Gallwey (mF-G) hirsutism score improved by 59.9%. The net reduction in mF-G score and the percent of patients reporting improvement in hirsutism were greater for OC+SPL than for either drug alone, with no difference in the percent of patients free of adverse effects. Among those treated with OC+SPL (n = 138), the initial mF-G and sex hormone-binding globulin (SHBG) independently predicted successful therapy for hirsutism. There is a high rate of patient satisfaction with suppressive therapy in PCOS. The efficacy of suppressive therapy for hirsutism was greater with OC+SPL than with either drug alone. Successful treatment of hirsutism with combination OC+SPL requires at least 6 months of therapy, with the proportion of satisfied patients continuing to increase with treatment duration. The probability of patient satisfaction with OC+SPL treatment for hirsutism can be

Vertebral Fractures and Bone Mineral Density in Patients With Idiopathic Hypoparathyroidism on Long-Term Follow-Up.

PubMed

Chawla, Himika; Saha, Soma; Kandasamy, Devasenathipathy; Sharma, Raju; Sreenivas, Vishnubhatla; Goswami, Ravinder

2017-01-01

Bone mineral density (BMD) is increased in idiopathic hypoparathyroidism (IH). Parathyroid hormone (PTH) deficiency, hypocalcemic seizures, and anticonvulsants could compromise skeletal health in IH. We assessed vertebral fractures (VFs) and related factors in IH and change in BMD during follow-up. VFs were assessed by morphometry. BMD was assessed by dual-energy X-ray absorptiometery at the lumbar spine, hip, and forearm. Change in BMD was assessed in a subset after a 10-year follow-up. The endocrine clinic of All India Institute of Medical Sciences, New Delhi, India. Included were 104 patients with IH and 64 healthy controls. Hypocalcemia, hyperphosphatemia, normal kidney function, and low serum PTH levels were used to diagnose IH. VFs were seen in 18.3% of patients with IH and 4.7% of controls (odds ratio, 4.54; 95% confidence interval, 1.28 to 16.04). Use of anticonvulsants and menopause were significantly associated (P < 0.05) with VF. Mean BMD at lumbar spine and hip were higher by 21.4% and 8.6%, respectively, in IH than in controls (P < 0.001), respectively. BMD significantly increased during follow-up at all sites. Change in BMD correlated with maintenance of the serum calcium/phosphorus ratio during follow-up. Despite increased BMD, prevalence of vertebral-fractures is greater in patients with IH, especially in postmenopausal women and those on anticonvulsant therapy. Copyright © 2017 by the Endocrine Society

Benefits and risk of sodium oxybate in idiopathic hypersomnia versus narcolepsy type 1: a chart review.

PubMed

Leu-Semenescu, Smaranda; Louis, Pauline; Arnulf, Isabelle

2016-01-01

Few stimulants have been evaluated for the treatment of idiopathic hypersomnia (IH). Sodium oxybate (indicated in narcolepsy type 1, NT1) has not been tested in IH patients. The aim of this study is to retrospectively evaluate the benefit/risk ratio of sodium oxybate in IH versus NT1 using a chart review. We reviewed the files of 46 patients with IH (35.7 ± 12.6 years old, 78% women) and 47 patients with NT1 (44.1 ± 18 years old, 47% women) and evaluated the benefits of sodium oxybate using the Epworth sleepiness scale (ESS) and a four-point scale assessing the global benefit, sleep inertia, sleepiness, sleep duration, and sleep onset latency. The spontaneously reported side effects were collected. Sodium oxybate was prescribed at a lower dose in IH than in NT1 (4.3 ± 2.2 vs. 6.6 ± 2.8 g/night, p <0.0001) patients after having tried more (3.2 ± 1.4 vs. 2.2 ± 1, p <0.0001) stimulants, but it produced a similar ESS change (-3.5 ± 4.5 vs. -3.2 ± 4.2 points) in the IH and NT1 groups. Severe morning inertia was improved in 24/34 (71%) patients with IH. During the follow-up period (15.8 months in IH vs. 35 months in NT1 groups), 53% IH and 68% NT1 patients dropped out. The side effects were as frequent in the IH group as in the NT1 group (67% vs. 52%), but nausea (40% vs. 13%) and dizziness (34.3% vs. 4.3%) were more frequent in the IH group. The benefit/risk ratio of sodium oxybate in IH- was similar to NT1-associated sleepiness, with additional benefits on severe morning inertia, despite using smaller doses in more refractory patients. Copyright © 2015 Elsevier B.V. All rights reserved.

The effects of Transcranial Direct Current Stimulation (tDCS) on Idiopathic Hypersomnia: a pilot study.

PubMed

Galbiati, Andrea; Abutalebi, Jubin; Iannaccone, Sandro; Borsa, Virginia Maria; Musteata, Stela; Zucconi, Marco; Giora, Enrico; Ferini-Strambi, Luigi

2016-04-01

Idiopathic Hypersomnia (IH) is a rare sleep disorder characterised by excessive daytime sleepiness (EDS) that leads to invalidating daytime consequences. Till now the treatment of IH has mirrored that of sleepiness in narcolepsy, and it is mainly focused on symptoms' management. We employed an anodal transcranic Direct Current Stimulation (tDCS) treatment in order to induce a shift toward arousal in IH patients' cortex during the day. Every patients underwent a 4 weeks treatment (3 stimulations per week, for a total of 12 stimulations over a period of 28 days) with an assessment at the baseline and after treatment aimed to the evaluation of subjective daytime sleepiness, neurocognitive functions, and attentional domain tested by means of the Attentional Network Task (ANT). The dependent variables of the ANT are accuracy and reaction times, which represent the objective outcome of our study. A significant effect of tDCS' treatment in reducing EDS was found. Besides the amelioration in subjective EDS,  an objective improvement in RTs in all conditions of the ANT, in particular in the more difficult component, was observed. Our results indicate that tDCS may foster the management of EDS in IH, improving also the attentional domain.

Central Disorders of Hypersomnolence: Focus on the Narcolepsies and Idiopathic Hypersomnia.

PubMed

Khan, Zeeshan; Trotti, Lynn Marie

2015-07-01

The central disorders of hypersomnolence are characterized by severe daytime sleepiness, which is present despite normal quality and timing of nocturnal sleep. Recent reclassification distinguishes three main subtypes: narcolepsy type 1, narcolepsy type 2, and idiopathic hypersomnia (IH), which are the focus of this review. Narcolepsy type 1 results from loss of hypothalamic hypocretin neurons, while the pathophysiology underlying narcolepsy type 2 and IH remains to be fully elucidated. Treatment of all three disorders focuses on the management of sleepiness, with additional treatment of cataplexy in those patients with narcolepsy type 1. Sleepiness can be treated with modafinil/armodafinil or sympathomimetic CNS stimulants, which have been shown to be beneficial in randomized controlled trials of narcolepsy and, quite recently, IH. In those patients with narcolepsy type 1, sodium oxybate is effective for the treatment of both sleepiness and cataplexy. Despite these treatments, there remains a subset of hypersomnolent patients with persistent sleepiness, in whom alternate therapies are needed. Emerging treatments for sleepiness include histamine H3 antagonists (eg, pitolisant) and possibly negative allosteric modulators of the gamma-aminobutyric acid-A receptor (eg, clarithromycin and flumazenil).

Effect of basal ganglia calcification on its glucose metabolism and dopaminergic function in idiopathic hypoparathyroidism.

PubMed

Modi, Sagar; Arora, Geetanjali; Bal, Chandra Shekhar; Sreenivas, Vishnubhatla; Kailash, Suparna; Sagar, Rajesh; Goswami, Ravinder

2015-10-01

The functional significance of basal ganglia calcification (BGC) in idiopathic hypoparathyroidism (IH) is not clear. To assess the effect of BGC on glucose metabolism and dopaminergic function in IH. (18) F-FDG and (99m) Tc-TRODAT-1 nuclear imaging were performed in 35 IH patients with (n = 26) and without (n = 9) BGC. Controls were subjects without hypoparathyroidism or BGC (nine for (18) F-FDG and 12 for (99m) Tc-TRODAT-1). Relationship of the glucose metabolism and dopaminergic function was assessed with the neuropsychological and biochemical abnormalities. (18) F-FDG uptake in IH patients with calcification at caudate and striatum was less than that of IH patients without calcification (1·06 ± 0·13 vs 1·24 ± 0·09, P = <0·0001 and 1·06 ± 0·09 vs 1·14 ± 0·08, P = 0·03, respectively). (18) F-FDG uptake did not correlate with neuropsychological dysfunctions. (18) F-FDG uptake in IH without BGC was significantly lower than that of controls. The mean (99m) Tc-TRODAT-1 uptake at basal ganglia was comparable between IH with and without BGC and between IH without BGC and controls. Serum calcium-phosphorus ratio maintained by the patients correlated with (18) F-FDG uptake at striatum (r = 0·57, P = 0·001). For every 0·1 unit reduction in calcium-phosphorus ratio, (18) F-FDG uptake decreased by 2·5 ± 0·68% (P = 0·001). BGC was associated with modest reduction (15%) in (18) F-FDG uptake at basal ganglia in IH but did not affect dopaminergic function. (18) F-FDG uptake did not correlate with neuropsychological dysfunctions. Interestingly, chronic hypocalcaemia-hyperphosphataemia also contributed to reduction in (18) F-FDG uptake which was independent of BGC. © 2014 John Wiley & Sons Ltd.

Nocturnal sleep architecture in idiopathic hypersomnia: a systematic review and meta-analysis.

PubMed

Plante, David T

2018-05-01

Current sleep medicine nosology places increased importance on nocturnal polysomnographic sleep recordings in the diagnosis of central nervous system disorders of hypersomnolence, particularly idiopathic hypersomnia (IH). Determine what differences in sleep staging and architecture exist between IH and healthy controls using meta-analysis. Systematic review identified relevant studies that included nocturnal polysomnography data for IH and healthy control groups. Meta-analysis compared standardized mean differences (Hedge's g) for total sleep time (TST), sleep onset latency (SOL), sleep efficiency (SE), rapid eye movement (REM) sleep percentage, slow wave sleep (SWS) percentage, and REM latency (REML). Moderator analyses were also conducted for variables with significant heterogeneity among studies. The meta-analysis included 10 studies. Relative to controls, IH demonstrated increased TST (pooled g = 0.92; 95% CI: 0.46 to 1.38, p < 0.0001) and REM percentage (pooled g = 0.36, 95% CI: 0.09 to 0.64, p = 0.01), decreased SOL (pooled g = -0.46; 95% CI: -0.81 to -0.12, p = 0.009) and SWS percentage (pooled g = -0.28, 95% CI: -0.50 to -0.07, p = 0.01), without significant differences in SE (pooled g = 0.03; 95% CI: -0.32 to 0.38, p = 0.86) or REML (pooled g = 0.14, 95% CI: -0.21 to 0.49, p = 0.42). Moderator analysis demonstrated a significant effect of sex on SE, with a higher proportion of women to men significantly predicting lower SE between in IH and controls (p < 0.0001). IH is associated with several changes in sleep staging and architecture relative to healthy persons, including alterations in REM and SWS not currently delineated in nosological constructs. Further research is indicated to clarify how these findings are related the pathophysiology of IH and related disorders. Copyright © 2017 Elsevier B.V. All rights reserved.

Prevalence of coeliac disease in idiopathic hypoparathyroidism and effect of gluten-free diet on calcaemic control.

PubMed

Saha, Soma; Saini, Savita; Makharia, Govind K; Datta Gupta, Siddhartha; Goswami, Ravinder

2016-04-01

Patients with idiopathic hypoparathyroidism (IH) require variable doses of calcium and 1-α-(OH)D. The reasons for such variability are not clear. As autoimmune mechanisms may play a role in IH, there is a possibility of coexistent coeliac disease with calcium/vitamin D malabsorption. We assessed the prevalence of coeliac disease and antitissue transglutaminase autoantibodies (anti-tTGAbs) in IH and analysed the effect of a gluten-free diet on calcaemic control. A total of 171 patients with IH and 126 healthy controls were screened for anti-tTGAb. IH patients with anti-tTGAb >20 RU/ml underwent duodenoscopy and intestinal biopsy; those with biopsy-proven coeliac disease were followed up on a gluten-free diet. Eleven of 171 (6·4%) patients with IH and seven of 126 (5·6%) controls had anti-tTGAb (P = 0·81). There was no difference in the clinical and biochemical parameters at diagnosis and during long-term follow-up of 7·2 ± 4·8 year (mean serum total calcium = 1·88 ± 0·16 vs 1·82 ± 0·36 mmol/l, P = 0·52; phosphorus = 1·81 ± 0·17 vs 1·87 ± 0·36 mmol/l, P = 0·53) in IH patients with and without anti-tTGAb. Although CaSRAb positivity was comparable in the two groups, IH patients with anti-tTGAb had higher TPOAb positivity (45·5% vs 12·8%, P = 0·02). Coeliac disease was diagnosed in only 2/9 patients with IH on biopsy, both of whom showed improved calcaemic control with a gluten-free diet. The prevalence of coeliac autoimmunity (6·4%) and coeliac disease (1·2%) in patients with IH seems to be similar to that in the general population. Notwithstanding this modest prevalence, it is important to be aware of the potential occurrence of coeliac disease with IH and the beneficial effect of a gluten-free diet on calcium control. © 2015 John Wiley & Sons Ltd.

Is acne a sign of androgen excess disorder or not?

PubMed

Uysal, Gulsum; Sahin, Yılmaz; Unluhizarci, Kursad; Ferahbas, Ayten; Uludag, Semih Zeki; Aygen, Ercan; Kelestimur, Fahrettin

2017-04-01

Acne is not solely a cosmetic problem. The clinical importance of acne in the estimation of androgen excess disorders is controversial. Recently, the Amsterdam ESHRE/ASRM-sponsored third PCOS Consensus Workshop Group suggested that acne is not commonly associated with hyperandrogenemia and therefore should not be regarded as evidence of hyperandrogenemia. Our aim was to investigate whether acne is a sign of androgen excess disorder or not. This is a cross sectional study that was performed in a university hospital involving 207 women, aged between 18 and 45 years, suffering mainly from acne. The women were assigned as polycystic ovary syndrome (PCOS), idiopathic hirsutism (IH), idiopathic hyperandrogenemia (IHA). Women with acne associated with any of the androgen excess disorders mentioned above were named as hyperandrogenemia associated acne (HAA). Women with acne but without hirsutism and hyperandrogenemia and having ovulatory cycles were named as "isolated acne". Serum luteinizing hormone, follicle stimulating hormone, estradiol, progesterone, 17-hydroxyprogesterone, dehydroepiandrosterone-sulfate (DHEAS), androstenedione, total testosterone and lipid levels were measured. Acne score was similar between the women with isolated acne and HAA. The most common cause for acne was PCOS and only 28% of the women had isolated acne. 114 (55%) women had at least one raised serum androgen level. In this study, 72% of acneic women had clinical and/or biochemical hyperandrogenemia. In contrast to the suggestion of ESHRE/ASRM-sponsored third PCOS Consensus Workshop Group, our data indicate that the presence of androgen excess disorders should be evaluated in women presenting with acne. Copyright © 2017 Elsevier B.V. All rights reserved.

Long-term outcome of cataract surgery in patients with idiopathic hypoparathyroidism and its relationship with their calcemic status.

PubMed

Saha, Soma; Gantyala, Shiva Prasad; Aggarwal, Sameer; Sreenivas, Vishnubhatla; Tandon, Radhika; Goswami, Ravinder

2017-07-01

Cataract is a cardinal manifestation of hypoparathyroidism. Although patients with hypoparathyroidism require cataract surgery at a younger age than individuals without hypoparathyroidism, there is limited information on the outcome of this surgery. We assessed long-term complications of cataract surgery in patients with idiopathic hypoparathyroidism (IH) and its relationship with their clinical and biochemical parameters. Twenty-seven patients with IH and 25 nonhypoparathyroid controls with a minimum follow-up of 2 years after cataract surgery were assessed for visual acuity, intraocular pressure, lens centricity, Nd:YAG laser capsulotomy, and the severity of posterior capsular opacification (PCO) and anterior capsular opacification. High-resolution optical slit-lamp images were analyzed by an ophthalmologist. Patients with IH had cataract surgery at a younger age than controls (34.0 ± 16.4 years vs 58.0 ± 11.2 years, P < 0.001). A higher proportion of IH patients had dense white PCO (75.0 % vs 39.4 %, P = 0.004), Nd:YAG laser capsulotomy (44.2 % vs 10.0 %, P = 0.001), anterior capsular opacification (97.7 % vs 84.2 %, P = 0.03), and a decentric lens (28.3% vs 2.6 %, P = 0.001) at a comparable time after surgery (8.6 ± 6.1 years vs 8.7 ± 6.8 years, P = 0.85). On regression analysis, the severity of PCO in IH correlated only with male sex and not with other factors, including serum total calcium and inorganic phosphorus levels at the baseline and during follow-up. To conclude, patients with IH are likelier than individuals without IH to develop PCO and to require Nd:YAG laser capsulotomy after cataract surgery. Proper precautions should be taken during surgery to minimize this complication in IH.

FFT-enhanced IHS transform method for fusing high-resolution satellite images

USGS Publications Warehouse

Ling, Y.; Ehlers, M.; Usery, E.L.; Madden, M.

2007-01-01

Existing image fusion techniques such as the intensity-hue-saturation (IHS) transform and principal components analysis (PCA) methods may not be optimal for fusing the new generation commercial high-resolution satellite images such as Ikonos and QuickBird. One problem is color distortion in the fused image, which causes visual changes as well as spectral differences between the original and fused images. In this paper, a fast Fourier transform (FFT)-enhanced IHS method is developed for fusing new generation high-resolution satellite images. This method combines a standard IHS transform with FFT filtering of both the panchromatic image and the intensity component of the original multispectral image. Ikonos and QuickBird data are used to assess the FFT-enhanced IHS transform method. Experimental results indicate that the FFT-enhanced IHS transform method may improve upon the standard IHS transform and the PCA methods in preserving spectral and spatial information. ?? 2006 International Society for Photogrammetry and Remote Sensing, Inc. (ISPRS).

Ontogeny of Gonadotropin-Inhibitory Hormone (GnIH) in the cichlid fish Cichlasoma dimerus.

PubMed

Di Yorio, María P; Sallemi, Julieta E; Toledo Solís, Francisco J; Pérez Sirkin, Daniela I; Delgadin, Tomás H; Tsutsui, Kazuyoshi; Vissio, Paula G

2018-05-13

RFamide peptides are expressed in the early stages of development in most vertebrates. Gonadotropin-inhibitory hormone (GnIH) belongs to the RFamide family, and its role in reproduction has been widely studied in adult vertebrates, ranging from fish to mammals. As only three reports evaluated GnIH during development, the aim of this study was to characterise the ontogeny of GnIH in a fish model, Cichlasoma dimerus. We detected the presence of two GnIH-immunoreactive (GnIH-ir) cell clusters with spatial and temporal differences. One cluster was observed by 3 days post-hatching (dph) in the nucleus olfacto-retinalis (NOR) and the other in the nucleus posterioris periventricularis by 14 dph. The number of GnIH-ir neurons increased in both nuclei, whereas their size increased only in the NOR from hatchling to juvenile stages. These changes occurred from the moment larvae started feeding exogenously and during development and differentiation of gonadal primordia. We showed by double-label immunofluorescence that only GnIH-ir neurons in the NOR co-expressed GnRH3 associated peptide. In addition, GnIH-ir fibre density increased in all brain regions from 5 dph. GnIH-ir fibres were also detected in the retina, optic tract and optic tectum, suggesting that GnIH acts as a neuromodulator of photoreception and the integration of different sensory modalities. Also, there were GnIH-ir fibres in the pituitary from 14 dph, which were in close association with somatotropes. Moreover, GnIH-ir fibres were observed in the saccus vasculosus from 30 dph, suggesting a potential role of GnIH in the modulation of its function. Finally, we found that gnih was expressed from 1 dph, and that the pattern of variation of its transcript levels was in accordance with that of cell number. Present results are the starting point for the study of new GnIH roles during development. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

Contribution of GnIH Research to the Progress of Reproductive Neuroendocrinology

PubMed Central

Tsutsui, Kazuyoshi; Ubuka, Takayoshi; Son, You Lee; Bentley, George E.; Kriegsfeld, Lance J.

2015-01-01

Since the discovery of gonadotropin-releasing hormone (GnRH) in mammals at the beginning of the 1970s, it was generally accepted that GnRH is the only hypothalamic neuropeptide regulating gonadotropin release in mammals and other vertebrates. In 2000, however, gonadotropin-inhibitory hormone (GnIH), a novel hypothalamic neuropeptide that actively inhibits gonadotropin release, was discovered in quail. Numerous studies over the past decade and a half have demonstrated that GnIH serves as a key player regulating reproduction across vertebrates, acting on the brain and pituitary to modulate reproductive physiology and behavior. In the latter case, recent evidence indicates that GnIH can regulate reproductive behavior through changes in neurosteroid, such as neuroestrogen, biosynthesis in the brain. This review summarizes the discovery of GnIH, and the contributions to GnIH research focused on its mode of action, regulation of biosynthesis, and how these findings advance our understanding of reproductive neuroendocrinology. PMID:26635728

[18F]Fludeoxyglucose-Positron Emission Tomography Evidence for Cerebral Hypermetabolism in the Awake State in Narcolepsy and Idiopathic Hypersomnia.

PubMed

Dauvilliers, Yves; Evangelista, Elisa; de Verbizier, Delphine; Barateau, Lucie; Peigneux, Philippe

2017-01-01

Changes in structural and functional central nervous system have been reported in narcolepsy, with large discrepancies between studies. No study has investigated yet spontaneous brain activity at wake in idiopathic hypersomnia (IH). We compared relative changes in regional brain metabolism in two central hypersomnia conditions with different clinical features, namely narcolepsy type 1 (NT1) and IH, and in healthy controls. Sixteen patients [12 males, median age 30 years (17-78)] with NT1, nine patients [2 males, median age 27 years (20-60)] with IH and 19 healthy controls [16 males, median age 36 years (17-78)] were included. 18 F-fludeoxyglucose positron emission tomography (PET) was performed in all drug-free subjects under similar conditions and instructions to stay in a wake resting state. We found increased metabolism in the anterior and middle cingulate and the insula in the two pathological conditions as compared to healthy controls. The reverse contrast failed to evidence hypometabolism in patients vs. controls. Comparisons between patient groups were non-significant. At sub-statistical threshold, we found higher right superior occipital gyrus glucose metabolism in narcolepsy and higher middle orbital cortex and supplementary motor area metabolism in IH, findings that require further confirmation. There is significant hypermetabolism in narcolepsy and IH in the wake resting state in a set of brain regions constitutive of the salience cortical network that may reflect a compensatory neurocircuitry activity secondary to sleepiness. Metabolic differences between the two disorders within the executive-control network may be a signature of abnormally functioning neural system leading to persistent drowsiness typical of IH.

Design of four-beam IH-RFQ linear accelerator

NASA Astrophysics Data System (ADS)

Ikeda, Shota; Murata, Aki; Hayashizaki, Noriyosu

2017-09-01

The multi-beam acceleration method is an acceleration technique for low-energy high-intensity heavy ion beams, which involves accelerating multiple beams to decrease space charge effects, and then integrating these beams by a beam funneling system. At the Tokyo Institute of Technology a two beam IH-RFQ linear accelerator was developed using a two beam laser ion source with direct plasma injection scheme. This system accelerated a carbon ion beam with a current of 108 mA (54 mA/channel × 2) from 5 up to 60 keV/u. In order to demonstrate that a four-beam IH-RFQ linear accelerator is suitable for high-intensity heavy ion beam acceleration, we have been developing a four-beam prototype. A four-beam IH-RFQ linear accelerator consists of sixteen RFQ electrodes (4 × 4 set) with stem electrodes installed alternately on the upper and lower ridge electrodes. As a part of this development, we have designed a four-beam IH-RFQ linear accelerator using three dimensional electromagnetic simulation software and beam tracking simulation software. From these simulation results, we have designed the stem electrodes, the center plate and the side shells by evaluating the RF properties such as the resonance frequency, the power loss and the electric strength distribution between the RFQ electrodes.

Feasibility and usefulness of ultrasonography in idiopathic intracranial hypertension or secondary intracranial hypertension.

PubMed

Lochner, Piergiorgio; Brio, Francesco; Zedde, Maria Luisa; Sanguigni, Sandro; Coppo, Lorenzo; Nardone, Raffaele; Naldi, Andrea; Sola, Daniele; Stolz, Erwin

2016-06-02

Transorbital sonography (TOS) has been proven to be able to non-invasively detect elevated intracranial pressure. In this condition TOS shows an increase in optic nerve sheath diameter (ONSD). It has been suggested that internal jugular vein valve insufficiency (IJVVI) may represent a factor contributing to the pathogenesis of idiopathic intracranial hypertension (IIH). The aim of this study was to investigate whether patients with IIH or secondary IH have higher ONSD values and higher frequency of IJVVI compared to subjects without IH. Twenty-one patients with newly diagnosed IIH or secondary IH were prospectively evaluated and compared with 21 age, gender and BMI-matched controls. Experienced vascular sonographers used B-mode TOS to evaluate ONSD, optic nerve diameter (OND) and IJVVI. CSF opening pressures were also measured. ONSD values were significantly higher in patients (6.50 ± 0.67) than controls (5.73 ± 0.66; p < 0.0001). No differences were found in OND values between patients (2.99 ± 0.26) and controls (2.93 ± 0.41; p = 0.574). No correlation was demonstrated between ONSD and CSF opening pressure (r = 0,086) (p = 0.73). No difference in frequency of IJVVI between patients (11/42 valves, 26 %) and controls (9/42, 21 %) was observed (p = 0.777). Increased ONSD values detected by TOS support the diagnosis of IH. Our results do not support the hypothesis of a venous congestion as a potential factor contributing to the pathogenesis of IIH. Not applicable. Observational, non-interventional study.

Reversible pressure-induced crystal-amorphous structural transformation in ice Ih

NASA Astrophysics Data System (ADS)

English, Niall J.; Tse, John S.

2014-08-01

Molecular dynamics (MD) simulation of depressurised high-density amorphous ice (HDA) at 80 K and at negative pressures has been performed. Over several attempts, HDA recrystallised to a form close to hexagonal ice Ih, albeit with some defects. The results support the hypothesis that compression of ice-Ih to HDA is a reversible first-order phase transition, with a large hysteresis. Therefore, it would appear that LDA is not truly amorphous. The elastic energy estimated from the area of the hysteresis loop is ca. 4.5 kJ/mol, in some way consistent with experimentally-determined accumulated successive heats of transformations from recovered HDA → ice Ih.

Ih Current Is Necessary to Maintain Normal Dopamine Fluctuations and Sleep Consolidation in Drosophila

PubMed Central

Gonzalo-Gomez, Alicia; Turiegano, Enrique; León, Yolanda; Molina, Isabel; Torroja, Laura; Canal, Inmaculada

2012-01-01

HCN channels are becoming pharmacological targets mainly in cardiac diseases. But apart from their well-known role in heart pacemaking, these channels are widely expressed in the nervous system where they contribute to the neuron firing pattern. Consequently, abolishing Ih current might have detrimental consequences in a big repertoire of behavioral traits. Several studies in mammals have identified the Ih current as an important determinant of the firing activity of dopaminergic neurons, and recent evidences link alterations in this current to various dopamine-related disorders. We used the model organism Drosophila melanogaster to investigate how lack of Ih current affects dopamine levels and the behavioral consequences in the sleep∶activity pattern. Unlike mammals, in Drosophila there is only one gene encoding HCN channels. We generated a deficiency of the DmIh core gene region and measured, by HPLC, levels of dopamine. Our data demonstrate daily variations of dopamine in wild-type fly heads. Lack of Ih current dramatically alters dopamine pattern, but different mechanisms seem to operate during light and dark conditions. Behaviorally, DmIh mutant flies display alterations in the rest∶activity pattern, and altered circadian rhythms. Our data strongly suggest that Ih current is necessary to prevent dopamine overproduction at dark, while light input allows cycling of dopamine in an Ih current dependent manner. Moreover, lack of Ih current results in behavioral defects that are consistent with altered dopamine levels. PMID:22574167

Ih current is necessary to maintain normal dopamine fluctuations and sleep consolidation in Drosophila.

PubMed

Gonzalo-Gomez, Alicia; Turiegano, Enrique; León, Yolanda; Molina, Isabel; Torroja, Laura; Canal, Inmaculada

2012-01-01

HCN channels are becoming pharmacological targets mainly in cardiac diseases. But apart from their well-known role in heart pacemaking, these channels are widely expressed in the nervous system where they contribute to the neuron firing pattern. Consequently, abolishing Ih current might have detrimental consequences in a big repertoire of behavioral traits. Several studies in mammals have identified the Ih current as an important determinant of the firing activity of dopaminergic neurons, and recent evidences link alterations in this current to various dopamine-related disorders. We used the model organism Drosophila melanogaster to investigate how lack of Ih current affects dopamine levels and the behavioral consequences in the sleep:activity pattern. Unlike mammals, in Drosophila there is only one gene encoding HCN channels. We generated a deficiency of the DmIh core gene region and measured, by HPLC, levels of dopamine. Our data demonstrate daily variations of dopamine in wild-type fly heads. Lack of Ih current dramatically alters dopamine pattern, but different mechanisms seem to operate during light and dark conditions. Behaviorally, DmIh mutant flies display alterations in the rest:activity pattern, and altered circadian rhythms. Our data strongly suggest that Ih current is necessary to prevent dopamine overproduction at dark, while light input allows cycling of dopamine in an Ih current dependent manner. Moreover, lack of Ih current results in behavioral defects that are consistent with altered dopamine levels.

Characterization of REM sleep without atonia in patients with narcolepsy and idiopathic hypersomnia using AASM scoring manual criteria.

PubMed

DelRosso, Lourdes M; Chesson, Andrew L; Hoque, Romy

2013-07-15

The AASM Manual for the Scoring of Sleep and Associated Events (Manual) has provided standardized definitions for tonic and phasic REM sleep without atonia (RSWA). This study used Manual criteria to characterize REM sleep in patients with narcolepsy and idiopathic hypersomnia (IH). A retrospective review of PSG data from ICSD-2 defined patients with narcolepsy or IH, performed by two board certified sleep medicine physicians. Data compiled included REM sleep epochs and the presence in REM sleep of epochs scored as sustained muscle activity (tonic), and excessive transient muscle activity (phasic) as defined by Manual criteria. PSG data from 8 narcolepsy patients (mean age: 27.5 years; age range: 11-55) showed mean ± standard deviation values for: total REM sleep epochs 205 ± 46.1; RSWA/ phasic epochs 56.1 ± 25.4; and RSWA/tonic epochs 15.0 ± 10.7. PSG data from 8 IH patients (mean age: 33.1 years; age range: 20-57) showed mean ± standard deviation values of total REM sleep epochs 163.8 ± 67.9; RSWA/phasic epochs 6.2 ± 3.5; and RSWA/tonic epochs 0.2 ± 0.4. Comparison revealed intergroup differences in phasic REM sleep (p < 0.01) and tonic REM sleep (p < 0.01) were significantly increased in narcoleptics compared to IH. Our retrospective analysis showed that RSWA phasic activity and RSWA tonic activity are significantly increased in patients meeting ICSD-2 criteria for narcolepsy compared to patients meeting ICSD-2 criteria for IH. This robust difference, with further validation, could be useful as electrophysiological criteria differentiating the two disorders and understanding the physiological differences.

40 CFR 721.10267 - [5,6]Fullerene-C60-Ih.

Code of Federal Regulations, 2014 CFR

2014-07-01

... CONTROL ACT SIGNIFICANT NEW USES OF CHEMICAL SUBSTANCES Significant New Uses for Specific Chemical Substances § 721.10267 [5,6]Fullerene-C60-Ih. (a) Chemical substance and significant new uses subject to reporting. (1) The chemical substance identified as [5,6]Fullerene-C60-Ih (PMN P-09-54; CAS No. 99685-96-8...

Efficacy of a new oral contraceptive containing drospirenone and ethinyl estradiol in the long-term treatment of hirsutism.

PubMed

Batukan, Cem; Muderris, Iptisam Ipek

2006-02-01

This study represents long term clinical and biochemical results and the response of different body parts to medical therapy with oral ethinyl estradiol/drospirenone combination in hirsute patients with or without polycystic ovary syndrome (PCOS). Prospective, open, controlled clinical study. Outpatients at Erciyes University Medical School. Fifty women with moderate to severe hirsutism were recruited. Two women were lost to follow-up. Women were treated with 3 mg of drospirenone and 30 microg of ethinyl estradiol for 12 cycles. Hirsutism was assessed at 6-month intervals using the Ferriman-Gallwey (F-G) scoring system. Serum FSH, LH, total and free testosterone (T), androstenedione (A), dehydroepiandrosterone sulfate (DHEAS), estradiol (E2), and sex-hormone binding globulin (SHBG) levels at 6 and 12 months of therapy were compared with baseline values. Total mean FG score declined by 67% and 78% after 6 and 12 months, respectively. Improvement was most prominent on the chest and abdomen, followed by the upper lip and chin. The lowest effect was observed on the back and arms. Serum levels of total and free T and A decreased, whereas SHBG levels increased significantly after 6 and 12 months when compared with baseline levels. Drospirenone/ethinyl estradiol combination exerts significant antiandrogenic activity and is effective in improving facial hirsutism. The beneficial effect is most obvious after six cycles and continues thereafter at a slower rate.

Fulminant bilateral papilloedema during low-dose steroid taper in a child with systemic idiopathic arthritis treated with tocilizumab.

PubMed

Burstzyn, Lulu; Levin, Simon; Rotenberg, Brian; Van Hooren, Tamara; Leung, Andrew; Berard, Roberta; Ardelean, Daniela S

2017-01-01

Systemic juvenile idiopathic arthritis (SJIA) is one of the most severe forms of arthritis that affects children younger than 16 years of age at onset. SJIA often requires corticosteroids to control the inflammation. However, long-term corticosteroid use may have adverse effects, including intracranial hypertension (IH). Biologic therapies have been used as corticosteroid sparing agents. We report the first case of a child with steroid-dependent SJIA treated with tocilizumab, an IL-6 receptor monoclonal antibody, who developed fulminant IH, bilateral papilloedema and vision loss when oral prednisone was weaned from 2 to 1 mg per day. Despite repeated lumbar punctures and high dose acetazolamide, he required urgent unilateral optic nerve sheath fenestration (ONSF). This endoscopic surgical intervention released the pressure exerted by the cerebrospinal fluid on the optic nerve and stopped the progression of vision loss. Nine weeks after the diagnosis of bilateral papilloedema, his vision was completely restored in one eye and partially recovered in the contralateral one. Long-term treatment with corticosteroids even at very low dose and tocilizumab may predispose to severe IH, papilloedema and vision loss. The role that tocilizumab might have played in this case in unclear. Early recognition and prompt treatment of papilloedema is crucial in avoiding permanent vision loss. Fulminant papilloedema in an immunocompromised child carries additional significant challenges. Early ONSF is a safe and effective intervention in refractory papilloedema. Children with severe papilledema secondary to IH should be managed by a multidisciplinary team in tertiary centres.

Assessing new terminal body and facial hair growth during pregnancy: toward developing a simplified visual scoring system for hirsutism.

PubMed

Yang, Yabo; Han, Yang; Wang, Wenjun; Du, Tao; Li, Yu; Zhang, Jianping; Yang, Dongzi; Zhao, Xiaomiao

2016-02-01

To study the distribution and progression of terminal hair growth in pregnant women and to determine the feasibility of a simplified scoring system for assessing hirsutism. Prospective follow-up observational study. Academic hospital. A total of 115 pregnant women (discovery cohort) and 1,159 women with polycystic ovary syndrome (PCOS) (validation cohort). Facial and body terminal hair growth assessed by modified Ferriman and Gallwey score system (mFG score), and total testosterone (TT) level detected by liquid chromatography with tandem mass spectrometry. Degree of facial and body terminal hair growth. The serum TT level and mFG score increased as pregnancy progressed. Both the prospective study and receiver operating characteristics curve indicated that the body areas with the greatest contribution to hirsutism (defined as an mFG score ≥5) with new terminal hair growth were the upper lip, lower back, lower abdomen, and thigh. A simplified mFG scoring system (sFG) was developed, and a cutoff value of ≥3 was defined as hirsutism. Pregnant hirsute women were distinguished from nonhirsute women with an accuracy of 95.2%, sensitivity of 96.8%, and specificity of 94.3% for detecting hirsutism. This was further validated in the PCOS population with a sensitivity, specificity, and positive predictive value of 97.6%, 96.4%, and 96.4%, respectively. This study suggests that the upper lip, lower back, lower abdomen, and thigh may be an effective simplified combination of the mFG system for the evaluation of excess hair growth in Chinese women. ChiCTR-OCH-14005012. Copyright © 2016 American Society for Reproductive Medicine. Published by Elsevier Inc. All rights reserved.

The IHS diagnostic X-ray equipment radiation protection program

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

Knapp, A.; Byrns, G.; Suleiman, O.

The Indian Health Service (IHS) operates or contracts with Tribal groups to operate 50 hospitals and approximately 165 primary ambulatory care centers. These facilities contain approximately 275 medical and 800 dental diagnostic x-ray machines. IHS environmental health personnel in collaboration with the Food and Drug Administration's (FDA) Center for Devices and Radiological Health (CDRH) developed a diagnostic x-ray protection program including standard survey procedures and menu-driven calculations software. Important features of the program include the evaluation of equipment performance collection of average patient entrance skin exposure (ESE) measurements for selected procedures, and quality assurance. The ESE data, collected using themore » National Evaluation of X-ray Trends (NEXT) protocol, will be presented. The IHS Diagnostic X-ray Radiation Protection Program is dynamic and is adapting to changes in technology and workload.« less

40 CFR 721.10267 - [5,6]Fullerene-C60-Ih.

Code of Federal Regulations, 2013 CFR

2013-07-01

... CONTROL ACT SIGNIFICANT NEW USES OF CHEMICAL SUBSTANCES Significant New Uses for Specific Chemical Substances § 721.10267 [5,6]Fullerene-C60-Ih. (a) Chemical substance and significant new uses subject to reporting.(1) The chemical substance identified as [5,6]Fullerene-C60-Ih (PMN P-09-54;CAS No. 99685-96-8) is...

Laser-assisted hair removal for facial hirsutism in women: A review of evidence.

PubMed

Lee, Chun-Man

2018-06-01

Poly cystic ovarian syndrome (PCOS) has been described as the common diagnosis for hirsutism in women. Facial hirsutism is by far the most distressing symptom of hyperandrogenism in women with PCOS. A statistically significant improvement in psychological well-being has been reported in patients with PCOS allocated for laser-assisted hair removal. The theory of selective photothermolysis has revolutionized laser hair removal in that it is effective and safe, when operated by sufficiently trained and experienced professionals. Long-pulsed ruby (694 nm), long-pulsed alexandrite (755 nm), diode (800-980 nm), and long-pulsed Nd:YAG (1064 nm) are commercially available laser devices for hair removal most widely studied. This article will introduce the fundamentals and mechanism of action of lasers in hair removal, in a contemporary literature review looking at medium to long term efficacy and safety profiles of various laser hair removal modalities most widely commercially available to date.

Effect of cortisol on gonadotropin inhibitory hormone (GnIH) in the cinnamon clownfish, Amphiprion melanopus.

PubMed

Choi, Young Jae; Habibi, Hamid R; Kil, Gyung-Suk; Jung, Min-Min; Choi, Cheol Young

2017-04-01

Hypothalamic peptides, gonadotropin-releasing hormone (GnRH) and gonadotropin inhibitory hormone (GnIH), play pivotal roles in the control of reproduction and gonadal maturation in fish. In the present study we tested the possibility that stress-mediated reproductive dysfunction in teleost may involve changes in GnRH and GnIH activity. We studied expression of brain GnIH, GnIH-R, seabream GnRH (sbGnRH), as well as circulating levels of follicle stimulating hormone (FSH), and luteinizing hormone (LH) in the cinnamon clownfish, Amphiprion melanopus. Treatment with cortisol increased GnIH mRNA level, but reduced sbGnRH mRNA and circulating levels of LH and FSH in cinnamon clownfish. Using double immunofluorescence staining, we found expression of both GnIH and GnRH in the diencephalon region of cinnamon clownfish brain. These findings support the hypothesis that cortisol, an indicator of stress, affects reproduction, in part, by increasing GnIH in cinnamon clownfish which contributes to hypothalamic suppression of reproductive function in A. melanopus, a protandrous hermaphroditic fish. Copyright © 2017 Elsevier Inc. All rights reserved.

High prevalence of hirsutism and menstrual disorders in obese adolescent girls and adolescent girls with type 1 diabetes mellitus despite different hormonal profiles.

PubMed

Samara-Boustani, Dinane; Colmenares, Ana; Elie, Caroline; Dabbas, Myriam; Beltrand, Jacques; Caron, Virgile; Ricour, Claude; Jacquin, Paul; Tubiana-Rufi, Nadia; Levy-Marchal, Claire; Delcroix, Christine; Martin, Delphine; Benadjaoud, Lila; Jacqz Aigrain, Evelyne; Trivin, Christine; Laborde, Kathleen; Thibaud, Elisabeth; Robert, Jean-Jacques; Polak, Michel

2012-02-01

To compare the pubertal development, the hormonal profiles and the prevalence of hirsutism and menstrual disorders in obese adolescent girls and adolescent girls with type 1 diabetes mellitus (T1DM). Data were collected from 96 obese adolescent girls and 78 adolescent girls with T1DM at Tanner stage IV or V, whose ages ranged between 11.9 and 17.9 years. High prevalence of hirsutism and menstrual disorder was found in the obese adolescent girls (36.5 and 42% respectively) and the adolescent girls with T1DM (21 and 44% respectively). The obese girls were significantly younger at pubarche, thelarche and menarche than the girls with T1DM. Hirsutism in the obese girls and those with T1DM was associated with hyperandrogenaemia and a raised free androgen index (FAI). When the cause of the raised FAI was investigated in both the groups of girls with hirsutism, the raised FAI in the obese girls was due to low serum sex hormone-binding globulin (SHBG) levels. In contrast, the raised FAI of the girls with T1DM and hirsutism was due to hyperandrogenaemia. Menstrual disorders in the T1DM girls were associated also with hyperandrogenaemia unlike obese girls. Hirsutism and menstrual disorders are common in obese adolescent girls and adolescent girls with T1DM. Although hyperandrogenaemia is present in both groups of girls, the androgenic profiles of the two groups differ. The hyperandrogenaemia in the obese girls is primarily due to their decreased serum SHBG levels, whereas the hyperandrogenaemia in the girls with T1DM is due to their increased androgen production.

42 CFR 137.206 - Why does the IHS need this information?

Code of Federal Regulations, 2010 CFR

2010-10-01

... 42 Public Health 1 2010-10-01 2010-10-01 false Why does the IHS need this information? 137.206 Section 137.206 Public Health PUBLIC HEALTH SERVICE, DEPARTMENT OF HEALTH AND HUMAN SERVICES INDIAN HEALTH SERVICE, DEPARTMENT OF HEALTH AND HUMAN SERVICES TRIBAL SELF-GOVERNANCE Operational Provisions Health Status Reports § 137.206 Why does the IHS...

25 CFR 900.67 - Will there be a uniform data set for all IHS programs?

Code of Federal Regulations, 2013 CFR

2013-04-01

... 25 Indians 2 2013-04-01 2013-04-01 false Will there be a uniform data set for all IHS programs... EDUCATION ASSISTANCE ACT Programmatic Reports and Data Requirements § 900.67 Will there be a uniform data set for all IHS programs? IHS will work with Indian tribe or tribal organization representatives to...

25 CFR 900.67 - Will there be a uniform data set for all IHS programs?

Code of Federal Regulations, 2010 CFR

2010-04-01

... 25 Indians 2 2010-04-01 2010-04-01 false Will there be a uniform data set for all IHS programs... EDUCATION ASSISTANCE ACT Programmatic Reports and Data Requirements § 900.67 Will there be a uniform data set for all IHS programs? IHS will work with Indian tribe or tribal organization representatives to...

25 CFR 900.67 - Will there be a uniform data set for all IHS programs?

Code of Federal Regulations, 2011 CFR

2011-04-01

... 25 Indians 2 2011-04-01 2011-04-01 false Will there be a uniform data set for all IHS programs... EDUCATION ASSISTANCE ACT Programmatic Reports and Data Requirements § 900.67 Will there be a uniform data set for all IHS programs? IHS will work with Indian tribe or tribal organization representatives to...

25 CFR 900.67 - Will there be a uniform data set for all IHS programs?

Code of Federal Regulations, 2012 CFR

2012-04-01

... 25 Indians 2 2012-04-01 2012-04-01 false Will there be a uniform data set for all IHS programs... EDUCATION ASSISTANCE ACT Programmatic Reports and Data Requirements § 900.67 Will there be a uniform data set for all IHS programs? IHS will work with Indian tribe or tribal organization representatives to...

25 CFR 900.67 - Will there be a uniform data set for all IHS programs?

Code of Federal Regulations, 2014 CFR

2014-04-01

... 25 Indians 2 2014-04-01 2014-04-01 false Will there be a uniform data set for all IHS programs... EDUCATION ASSISTANCE ACT Programmatic Reports and Data Requirements § 900.67 Will there be a uniform data set for all IHS programs? IHS will work with Indian tribe or tribal organization representatives to...

Presence of strong association of the major histocompatibility complex (MHC) class I allele HLA-A*26:01 with idiopathic hypoparathyroidism.

PubMed

Goswami, Ravinder; Singh, Archana; Gupta, Nandita; Rani, Rajni

2012-09-01

The pathogenesis of isolated hypoparathyroidism, also referred to as idiopathic hypoparathyroidism (IH), is not clear. There is a paucity of information related to the immunogenetic basis of the disease due to its rarity. A recurrent theme of several autoimmune disorders is aberrant antigen presentation. We investigated for the association of alleles of the human leukocyte antigen (HLA) class I and II loci with IH. A total of 134 patients with IH and 902 healthy controls from the same ethnic background participated in the study. There was a significant increase of HLA class I alleles HLA-A*26:01 [P < 1.71 × 10(-34); odds ratio (OR) = 9.29; 95% confidence interval (CI) = 6.08-14.16] and HLA-B*08:01 (P < 8.19 × 10(-6); OR = 2.59; 95% CI = 1.63-4.04) in patients with IH compared to healthy controls. However, the association of A*26:01 was primary because B*08:01 was in linkage disequilibrium with A*26:01. Although the major histocompatibility complex (MHC) is very polymorphic, several alleles of HLA loci share key residues at anchor positions in the peptide binding pockets such that similar peptides may be presented by different MHC molecules encoded by the same locus. These allelic forms with similar anchoring amino acids have been clustered in supertypes. An analysis of HLA-A locus supertypes A01, A02, A03, and A04 revealed that supertype A01 was significantly increased (P < 9.18 × 10(-9); OR = 2.95) in IH compared to controls. However, this increase in the supertype A01 was contributed by A*26:01 because 68.7% of the A01 samples had A*26:01. Other alleles of the supertype did not show any significant differences. The strong association of HLA-A*26:01 suggests an important role of MHC class I-mediated presentation of autoantigenic peptides to CD8(+) cytotoxic T cells in the pathogenesis of IH. These data provide evidence for the autoimmune etiology of IH akin to other autoimmune disorders like type 1 diabetes and rheumatoid arthritis.

Can laser treatment improve quality of life of hirsute women?

PubMed Central

Alizadeh, Narges; Ayyoubi, Sharad; Naghipour, Mohammadreza; Hassanzadeh, Rasool; Mohtasham-Amiri, Zahra; Zaresharifi, Shirin; Gharaei Nejad, Kaveh

2017-01-01

Background Hirsutism can have negative impacts on psychosocial aspects of women’s lives and reduce their quality of life (QOL). The aim of this study was to assess the QOL of these women during laser treatment. Patients and methods Eighty-eight women with unwanted facial hair underwent laser therapy. Each patient completed a questionnaire consisting of a modified Dermatology Life Quality Index (DLQI) and visual analog scale (VAS) before the first, third, and fifth sessions of laser therapy. Interval between the sessions was 4–6 weeks. Statistical analyses were done using SPSS software version18. Results The DLQI scores before treatment, and at third and fifth sessions were 7.75±2.36, 5.55±1.88, and 4.14±0.64, respectively (P<0.0001). Also, VAS scores had a decreasing trend between the first and second treatment sessions as the mean patient VAS score fell from 10±0.04 to 5.53±2.41 (P<0.0001). The DLQI scores were significantly different according to areas of hair growth and number of involved areas. There were no significant differences with regard to response to treatment and mean of DLQI score according to the level of education, marital status, and employment status. Conclusion Hair removal with laser therapy can improve the QOL in hirsute women. Also, socioeconomic status does not affect the satisfaction rate of laser therapy for hair removal. PMID:29089786

Whole brain inhomogeneous magnetization transfer (ihMT) imaging: Sensitivity enhancement within a steady-state gradient echo sequence.

PubMed

Mchinda, Samira; Varma, Gopal; Prevost, Valentin H; Le Troter, Arnaud; Rapacchi, Stanislas; Guye, Maxime; Pelletier, Jean; Ranjeva, Jean-Philippe; Alsop, David C; Duhamel, Guillaume; Girard, Olivier M

2018-05-01

To implement, characterize, and optimize an interleaved inhomogeneous magnetization transfer (ihMT) gradient echo sequence allowing for whole-brain imaging within a clinically compatible scan time. A general framework for ihMT modelling was developed based on the Provotorov theory of radiofrequency saturation, which accounts for the dipolar order underpinning the ihMT effect. Experimental studies and numerical simulations were performed to characterize and optimize the ihMT-gradient echo dependency with sequence timings, saturation power, and offset frequency. The protocol was optimized in terms of maximum signal intensity and the reproducibility assessed for a nominal resolution of 1.5 mm isotropic. All experiments were performed on healthy volunteers at 1.5T. An important mechanism driving signal optimization and leading to strong ihMT signal enhancement that relies on the dynamics of radiofrequency energy deposition has been identified. By taking advantage of the delay allowed for readout between ihMT pulse bursts, it was possible to boost the ihMT signal by almost 2-fold compared to previous implementation. Reproducibility of the optimal protocol was very good, with an intra-individual error < 2%. The proposed sensitivity-boosted and time-efficient steady-state ihMT-gradient echo sequence, implemented and optimized at 1.5T, allowed robust high-resolution 3D ihMT imaging of the whole brain within a clinically compatible scan time. Magn Reson Med 79:2607-2619, 2018. © 2017 International Society for Magnetic Resonance in Medicine. © 2017 International Society for Magnetic Resonance in Medicine.

CSF histamine contents in narcolepsy, idiopathic hypersomnia and obstructive sleep apnea syndrome.

PubMed

Kanbayashi, Takashi; Kodama, Tohru; Kondo, Hideaki; Satoh, Shinsuke; Inoue, Yuichi; Chiba, Shigeru; Shimizu, Tetsuo; Nishino, Seiji

2009-02-01

To (1) replicate our prior result of low cerebrospinal fluid (CSF) histamine levels in human narcolepsy in a different sample population and to (2) evaluate if histamine contents are altered in other types of hypersomnia with and without hypocretin deficiency. Cross sectional studies. Sixty-seven narcolepsy subjects, 26 idiopathic hypersomnia (IHS) subjects, 16 obstructive sleep apnea syndrome (OSAS) subjects, and 73 neurological controls were included. All patients were Japanese. Diagnoses were made according to ICSD-2. We found significant reductions in CSF histamine levels in hypocretin deficient narcolepsy with cataplexy (mean +/- SEM; 176.0 +/- 25.8 pg/mL), hypocretin non-deficient narcolepsy with cataplexy (97.8 +/- 38.4 pg/mL), hypocretin non-deficient narcolepsy without cataplexy (113.6 +/- 16.4 pg/mL), and idiopathic hypersomnia (161.0 +/- 29.3 pg/ mL); the levels in OSAS (259.3 +/- 46.6 pg/mL) did not statistically differ from those in the controls (333.8 +/- 22.0 pg/mL). Low CSF histamine levels were mostly observed in non-medicated patients; significant reductions in histamine levels were evident in non-medicated patients with hypocretin deficient narcolepsy with cataplexy (112.1 +/- 16.3 pg/ mL) and idiopathic hypersomnia (143.3 +/- 28.8 pg/mL), while the levels in the medicated patients were in the normal range. The study confirmed reduced CSF histamine levels in hypocretin-deficient narcolepsy with cataplexy. Similar degrees of reduction were also observed in hypocretin non-deficient narcolepsy and in idiopathic hypersomnia, while those in OSAS (non central nervous system hypersomnia) were not altered. The decrease in histamine in these subjects were more specifically observed in non-medicated subjects, suggesting CSF histamine is a biomarker reflecting the degree of hypersomnia of central origin.

Preferential inhibition of Ih in rat trigeminal ganglion neurons by an organic blocker.

PubMed

Janigro, D; Martenson, M E; Baumann, T K

1997-11-15

The potency and specificity of a novel organic Ih current blocker DK-AH 268 (DK, Boehringer) was studied in cultured rat trigeminal ganglion neurons using whole-cell patch-clamp recording techniques. In neurons current-clamped at the resting potential, the application of 10 microM DK caused a slight hyperpolarization of the membrane potential and a small increase in the threshold for action potential discharge without any major change in the shape of the action potential. In voltage-clamped neurons, DK caused a reduction of a hyperpolarization-activated current. Current subtraction protocols revealed that the time-dependent, hyperpolarization-activated currents blocked by 10 microM DK or external Cs+ (3 mM) had virtually identical activation properties, suggesting that DK and Cs+ caused blockade of the same current, namely Ih. The block of Ih by DK was dose-dependent. At the intermediate and higher concentrations of DK (10 and 100 microM) a decrease in specificity was observed so that time-independent, inwardly rectifying and noninactivating, voltage-gated outward potassium currents were also reduced by DK but to a much lesser extent than the time-dependent, hyperpolarization-activated currents. Blockade of the time-dependent, hyperpolarization-activated currents by DK appeared to be use-dependent since it required hyperpolarization for the effect to take place. Relief of DK block was also aided by membrane hyperpolarization. Since both the time-dependent current blocked by DK and the Cs+-sensitive time-dependent current behaved as Ih, we conclude that 10 microM DK can preferentially reduce Ih without a major effect on other potassium currents. Thus, DK may be a useful agent in the investigation of the function of Ih in neurons.

Comparison of clinical characteristics among narcolepsy with and without cataplexy and idiopathic hypersomnia without long sleep time, focusing on HLA-DRB1( *)1501/DQB1( *)0602 finding.

PubMed

Sasai, Taeko; Inoue, Yuichi; Komada, Yoko; Sugiura, Tatsuki; Matsushima, Eisuke

2009-10-01

Clinical characteristics of narcolepsy without cataplexy (NA w/o CA) and its relation to positivity of HLA-DRB1( *)1501/DQB1( *)0602 remain unclarified. We investigated clinical features of NA w/o CA, particularly addressing HLA-DRB1( *)1501/DQB1( *)0602. Comparisons of the Epworth Sleepiness Scale (ESS), multiple sleep latency test (MSLT) variables, rapid eye movement (REM)-related symptoms, and treatment response to psychostimulant medication were made for four patient groups (narcolepsy with cataplexy; NA-CA, NA w/o CA HLA-positive, NA w/o CA HLA-negative, and idiopathic hypersomnia without long sleep time; IHS w/o LST). Mean sleep latency was significantly shorter and the rate of reduction of ESS after medication was lower in both NA-CA and NA w/o CA HLA-positive groups than those in the IHS w/o LST group. Among the three narcoleptic groups, the NA w/o CA HLA-negative group showed the lowest REM latency and the highest reduction rate of ESS after treatment. Neither these subjective and objective sleepiness measures nor the treatment response measure was significantly different between this group and the IHS w/o LST group. In NA w/o CA, HLA-positivity might affect hypersomnia severity and REM propensity. The NA w/o CA HLA-negative group and the IHS w/o LST group exhibit equivalent hypersomnia severity.

Photoabsorption of the molecular IH cation at the iodine 3 d absorption edge

NASA Astrophysics Data System (ADS)

Klumpp, Stephan; Guda, Alexander A.; Schubert, Kaja; Mertens, Karolin; Hellhund, Jonas; Müller, Alfred; Schippers, Stefan; Bari, Sadia; Martins, Michael

2018-03-01

Yields of atomic iodine Iq + (q ≥2 ) fragments resulting from photoexcitation and photoionization of the target ions IH+ and I+ have been measured in the photon-energy range 610-680 eV, which comprises the thresholds for iodine 3 d ionization. The measured ion-yield spectra show two strong and broad resonance features due to the excitation of the 3 d3 /2 ,5 /2 electrons into ɛ f states rather similar for both parent ions. In the 3 d pre-edge range, excitations into (n p π ) -like orbitals and into an additional σ* orbital are found for IH+, which have been identified by comparison of the atomic I+ and molecular IH+ data and with the help of (time-dependent) density functional theory (DFT) and atomic Hartree-Fock calculations. The (5 p π ) orbital is almost atomlike, whereas all other resonances of the IH+ primary ion show a more pronounced molecular character, which is deduced from the chemical shifts of the resonances and the theoretical analysis.

Activation of Ih and TTX-sensitive sodium current at subthreshold voltages during CA1 pyramidal neuron firing

PubMed Central

Yamada-Hanff, Jason

2015-01-01

We used dynamic clamp and action potential clamp techniques to explore how currents carried by tetrodotoxin-sensitive sodium channels and HCN channels (Ih) regulate the behavior of CA1 pyramidal neurons at resting and subthreshold voltages. Recording from rat CA1 pyramidal neurons in hippocampal slices, we found that the apparent input resistance and membrane time constant were strongly affected by both conductances, with Ih acting to decrease apparent input resistance and time constant and sodium current acting to increase both. We found that both Ih and sodium current were active during subthreshold summation of artificial excitatory postsynaptic potentials (EPSPs) generated by dynamic clamp, with Ih dominating at less depolarized voltages and sodium current at more depolarized voltages. Subthreshold sodium current—which amplifies EPSPs—was most effectively recruited by rapid voltage changes, while Ih—which blunts EPSPs—was maximal for slow voltage changes. The combined effect is to selectively amplify rapid EPSPs. We did similar experiments in mouse CA1 pyramidal neurons, doing voltage-clamp experiments using experimental records of action potential firing of CA1 neurons previously recorded in awake, behaving animals as command voltages to quantify flow of Ih and sodium current at subthreshold voltages. Subthreshold sodium current was larger and subthreshold Ih was smaller in mouse neurons than in rat neurons. Overall, the results show opposing effects of subthreshold sodium current and Ih in regulating subthreshold behavior of CA1 neurons, with subthreshold sodium current prominent in both rat and mouse CA1 pyramidal neurons and additional regulation by Ih in rat neurons. PMID:26289465

Morphological relationship between GnIH and GnRH neurons in the brain of the Neotropical cichlid fishCichlasoma dimerus.

PubMed

Di Yorio, María P; Pérez Sirkin, Daniela I; Muñoz-Cueto, José A; Delgadin, Tomás H; Tsutsui, Kazuyoshi; Somoza, Gustavo M; Vissio, Paula G

2018-06-15

Reproduction is regulated by the hypothalamic-pituitary-gonadal axis. The first neuropeptide identified that regulates this function was the decapeptide gonadotropin-releasing hormone (GnRH). Nowadays, in gnatostomates, a number of GnRH variants have been identified and classified into three different types: GnRH1, GnRH2, and GnRH3. Almost 30 years later, a new peptide that inhibits gonadotropin synthesis and secretion was discovered and thus named as gonadotropin-inhibitory hormone (GnIH). In avians and mammals, the interaction and regulation between GnRH and GnIH neurons has been widely studied; however, in other vertebrate groups there is little information about the relationship between these neurons. In previous works, three GnRH variants and a GnIH propeptide were characterized in Cichlasoma dimerus, and it was demonstrated that GnIH inhibited gonadotropins release in this species. Because no innervation was detected at the pituitary level, we speculate that GnIH would inhibit gonadotropins via GnRH. Thus, the aim of the present study was to evaluate the anatomical relationship between neurons expressing GnIH and the three GnRH variants by double labelling confocal immunofluorescence in adults of C. dimerus. Our results showed no apparent contacts between GnIH and GnRH1, fiber to fiber interactions between GnIH and GnRH2, and co-localization of GnIH and GnRH3 variant in neurons of the nucleus olfacto-retinalis. In conclusion, whether GnIH regulates the expression or secretion of GnRH1 in this species, an indirect modulation seems more plausible. Moreover, the present results suggest an interaction between GnIH and GnRH2 systems. Finally, new clues were provided to investigate the role of nucleus olfacto-retinalis cells and putative GnIH and GnRH3interactions in the modulation of the reproductive network in teleost fish. Copyright © 2018. Published by Elsevier Inc.

Association of polycystic ovary syndrome susceptibility single nucleotide polymorphism rs2479106 and PCOS in Caucasian patients with PCOS or hirsutism as referral diagnosis.

PubMed

Eriksen, Mette B; Brusgaard, Klaus; Andersen, Marianne; Tan, Qihua; Altinok, Magda L; Gaster, Michael; Glintborg, Dorte

2012-07-01

Polycystic ovary syndrome (PCOS) is the most common endocrine disease among premenopausal women. A recent study found association between three single nucleotide polymorphisms (SNPs) and PCOS in a cohort of Han Chinese women. To investigate the association between rs13405728 (LHCGR gene), rs13429458 (THADA gene) and rs2479106 (DENND1A gene), PCOS, hirsutism and metabolic and hormonal parameters in a well characterized cohort of Caucasian patients of Danish descendant with PCOS or hirsutism. Patients underwent clinical examination, hormone analyses, oral glucose tolerance test and transvaginal ultrasound. Genetic variation was tested using allelic discrimination by real-time PCR. 268 patients referred to The Department of Endocrinology, Odense University Hospital, Denmark with PCOS or hirsutism between 1997 and 2011. Two hundred and forty-eight healthy females were included as controls. Genotype distributions and allele frequencies of rs13405728, rs13429458, and rs2479106 were comparable in patients and controls. The rs2479106 G allele was associated with a decreased PCOS susceptibility. None of the SNPs were associated with hirsutism or increased metabolic parameters. The rs2479106 G allele was associated with decreased PCOS susceptibility, thus confirming previously reported findings of association between rs2479106 and PCOS. Metabolic and hormonal parameters were comparable between genotypes of rs13405728 and rs2479106. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

Cardiovascular risk factors and events in women with androgen excess.

PubMed

Macut, D; Antić, I B; Bjekić-Macut, J

2015-03-01

Androgen excess (AE) was approximated to be present in 7% of the adult population of women. Polycystic ovary syndrome (PCOS) is the most prevalent among them, followed by idiopathic hirsutism (IH), congenital adrenal hyperplasia (CAH), hyperandrogenic insulin-resistant acanthosis nigricans (HAIRAN) syndrome, and androgen-secreting neoplasms (ASNs). Increased cardiovascular risk was implicated in women with AE. Serum testosterone independently increases risk for cardiovascular disease (CVD), and correlates even with indices of subclinical atherosclerosis in various populations of postmenopausal women. Hyperandrogenism in PCOS is closely related to the aggravation of abdominal obesity, and together with insulin resistance forming the metabolic core for the development of CVD. However, phenotypic variability of PCOS generates significant influence on the cardiometabolic risks. Numerous risk factors in PCOS lead to 5-7 times higher risk for CVD and over 2-fold higher risk for coronary heart disease and stroke. However, issue on the cardiometabolic risk in postmenopausal women with hyperandrogenic history is still challenging. There is a significant overlapping in the CVD characteristics of women with PCOS and variants of CAH. Relevant clinical data on the prevalence and cardiometabolic risk and events in women with IH, HAIRAN syndrome or ASNs are scarce. The effects of various oral contraceptives (OCs) and antiandrogenic compounds on metabolic profile are varying, and could be related to the selected populations and different therapy regiments mainly conducted in women with PCOS. It is assumed relation of OCs containing antiandrogenic progestins to the increased risk of cardiovascular and thromboembolic events.

Expression of a functional hyperpolarization-activated current (Ih) in the mouse nucleus reticularis thalami.

PubMed

Rateau, Y; Ropert, N

2006-05-01

The GABAergic neurons of the nucleus reticularis thalami (nRT) express the type 2 hyperpolarization-activated cAMP-sensitive (HCN2) subunit mRNA, but surprisingly, they were reported to lack the hyperpolarization-activated (Ih) current carried by this subunit. Using the voltage-clamp recordings in the thalamocortical slice preparation of the newborn and juvenile mice (P6-P23), we demonstrate that, in the presence of 1 mM barium (Ba2+), the nRT neurons express a slow hyperpolarization-activated inward current, suggesting that the Ih is present but masked in control conditions by K+ leak currents. We investigate the identity of the hyperpolarization-activated current in the nRT by studying its physiological and pharmacological profile in presence of Ba2+. We show that it has voltage- and time-dependent properties typical of the Ih, that it is blocked by cesium and ZD7288, two blockers of the Ih, and that it is carried both by the K+ and Na+ ions. We could also alter the gating characteristics of the hyperpolarization-activated current in the nRT by adding a nonhydrolysable analogue of cAMP to the pipette solution. Finally, using the current-clamp recording, we showed that blocking the hyperpolarization-activated current induced an hyperpolarization correlated with an increase of the R(in) of the nRT neurons. In conclusion, our results demonstrate that the nRT neurons express the Ih with slow kinetics similar to those described for the homomeric HCN2 channels, and we show that the Ih of the nRT contributes to the excitability of the nRT neurons in normal conditions.

Structure and energetics of extended defects in ice Ih

NASA Astrophysics Data System (ADS)

Silva Junior, Domingos L.; de Koning, Maurice

2012-01-01

We consider the molecular structure and energetics of extended defects in proton-disordered hexagonal ice Ih. Using plane-wave density functional theory (DFT) calculations, we compute the energetics of stacking faults and determine the structure of the 30∘ and 90∘ partial dislocations on the basal plane. Consistent with experimental data, the formation energies of all fully reconstructed stacking faults are found to be very low. This is consistent with the idea that basal-plane glide dislocations in ice Ih are dissociated into partial dislocations separated by an area of stacking fault. For both types of partial dislocation we find a strong tendency toward core reconstruction through pairwise hydrogen-bond reformation. In the case of the 30∘ dislocation, the pairwise hydrogen-bond formation leads to a period-doubling core structure equivalent to that seen in zinc-blende semiconductor crystals. For the 90∘ partial we consider two possible core reconstructions, one in which the periodicity of the structure along the core remains unaltered and another in which it is doubled. The latter is preferred, although the energy difference between both is rather small, so that a coexistence of both reconstructions appears plausible. Our results imply that a mobility theory for dislocations on the basal plane in ice Ih should be based on the idea of reconstructed partial dislocations.

Photoinduced Charge Transfer and Electrochemical Properties of Triphenylamine Ih-Sc3N@C80 Donor-Acceptor Conjugates

PubMed Central

Pinzón, Julio R.; Gasca, Diana C.; Shankara, Gayathri. S; Bottari, Giovanni; Torres, Tomás; Guldi, Dirk M.; Echegoyen, Luis

2009-01-01

Two isomeric [5,6]-pyrrolidine-Ih-Sc3N@C80 electron donor acceptor conjugates containing triphenylamine (TPA) as the donor system were synthesized. Electrochemical and photophysical studies of the novel conjugates were made and compared with those of their C60 analogues, in order to determine i) the effect of the linkage position (N-substituted versus 2-substituted pyrrolidine) of the donor system in the formation of photoinduced charge separated states, ii) the thermal stability towards the retro-cycloaddition reaction and iii) the effect of changing C60 for Ih-Sc3N@C80 as the electron acceptor. It was found that when the donor is connected to the pyrrolidine nitrogen atom, the resulting dyad produces a significantly longer lived radical pair than the corresponding 2-substituted isomer for both the C60 and Ih-Sc3N@C80 dyads. In addition to that, the N-substituted TPA-Ih-Sc3N@C80 dyad has much better thermal stability than the 2-subtituted one. Finally, the Ih-Sc3N@C80 dyads have considerably longer lived charge separated states than their C60 analogues, thus approving the advantage of using Ih-Sc3N@C80 instead of C60 as the acceptor for the construction of fullerene based donor acceptor conjugates. These findings are important for the design and future application of Ih-Sc3N@C80 dyads as materials for the construction of plastic organic solar cells. PMID:19445462

A prospective randomized trial comparing low-dose ethinyl estradiol and drospirenone 24/4 combined oral contraceptive vs. ethinyl estradiol and drospirenone 21/7 combined oral contraceptive in the treatment of hirsutism.

PubMed

Oner, Gokalp; Muderris, Iptisam Ipek

2011-11-01

A prospective randomized trial was conducted to compare the clinical efficacy of two oral contraceptives containing drospirenone in the treatment of hirsutism in women. Fifty women with moderate to severe hirsutism were recruited. Three women were lost to follow-up. Twenty-four patients received oral 0.03 mg ethinyl estradiol and 3 mg drospirenone 21/7 regimen (Group 1) for 6 months. Another group of 23 patients received oral 0.02 mg ethinyl estradiol and 3 mg drospirenone 24/4 regimen (Group 2) for 6 months. Hirsutism was assessed after 6 months using the Ferriman-Gallwey (F-G) scoring system. Hormonal levels after 6 months of both therapies were compared with baseline values and each other. An improvement in the F-G scores for hirsutism (mean±SD) was observed in Group 1 (17.3±5.2 to 8.7±2.5, p<.001) and in Group 2 (17.5±4.8 to 7.9±2.8, p<.001). Pre- and post-treatment hirsutism scores were comparable between the groups (p>.05). Total and free testosterone levels decreased significantly after the therapy in both groups. The sex hormone-binding globulin levels increased significantly in both groups during the 6-month period. The treatment of hirsutism with both combined oral contraceptives (COCs) containing drospirenone offered comparable effects and was well tolerated. Copyright © 2011 Elsevier Inc. All rights reserved.

Transvaginal ultrasound appearances of the ovary in normal women and hirsute women with oligomenorrhoea.

PubMed

Fox, R

1999-02-01

The transvaginal ultrasound appearances of the ovary were determined in women with clinical and endocrine features of polycystic ovarian disease (PCOD) and apparently normal women. At scan the number of small follicles were counted and ovarian volume was calculated. The maximum width of the ovarian cortex was also measured. Blood was sent for measurement of LH, FSH and testosterone. The women with oligomenorrhoea were scanned at random and the normal women were seen within the first 5 days of the start of menstruation. There were significant differences between median values for the 2 groups in terms of number of small follicles, ovarian volume and stromal width; the ovaries of the hirsute women had more follicles, were of larger volume, and had greater stromal width. The 2 ranges for number of follicles did overlap, however. Four hirsute oligomenorrhoeic women had a normal number of follicles; all 4 had the several clinical and endocrine features indicative of PCOD. These data suggest that the classical ultrasound features of PCOD are not consistently present and that the absence of increased follicularity at scan should not necessarily deter clinicians from making the functional diagnosis of PCOD.

New Equations for the Sublimation Pressure and Melting Pressure of H2O Ice Ih

NASA Astrophysics Data System (ADS)

Wagner, Wolfgang; Riethmann, Thomas; Feistel, Rainer; Harvey, Allan H.

2011-12-01

New reference equations, adopted by the International Association for the Properties of Water and Steam (IAPWS), are presented for the sublimation pressure and melting pressure of ice Ih as a function of temperature. These equations are based on input values derived from the phase-equilibrium condition between the IAPWS-95 scientific standard for thermodynamic properties of fluid H2O and the equation of state of H2O ice Ih adopted by IAPWS in 2006, making them thermodynamically consistent with the bulk-phase properties. Compared to the previous IAPWS formulations, which were empirical fits to experimental data, the new equations have significantly less uncertainty. The sublimation-pressure equation covers the temperature range from 50 K to the vapor-liquid-solid triple point at 273.16 K. The ice Ih melting-pressure equation describes the entire melting curve from 273.16 K to the ice Ih-ice III-liquid triple point at 251.165 K. For completeness, we also give the IAPWS melting-pressure equation for ice III, which is slightly adjusted to agree with the ice Ih melting-pressure equation at the corresponding triple point, and the unchanged IAPWS melting-pressure equations for ice V, ice VI, and ice VII.

CSF Histamine Contents in Narcolepsy, Idiopathic Hypersomnia and Obstructive Sleep Apnea Syndrome

PubMed Central

Kanbayashi, Takashi; Kodama, Tohru; Kondo, Hideaki; Satoh, Shinsuke; Inoue, Yuichi; Chiba, Shigeru; Shimizu, Tetsuo; Nishino, Seiji

2009-01-01

Study Objective: To (1) replicate our prior result of low cerebrospinal fluid (CSF) histamine levels in human narcolepsy in a different sample population and to (2) evaluate if histamine contents are altered in other types of hypersomnia with and without hypocretin deficiency. Design: Cross sectional studies. Setting and Patients: Sixty-seven narcolepsy subjects, 26 idiopathic hypersomnia (IHS) subjects, 16 obstructive sleep apnea syndrome (OSAS) subjects, and 73 neurological controls were included. All patients were Japanese. Diagnoses were made according to ICSD-2. Results: We found significant reductions in CSF histamine levels in hypocretin deficient narcolepsy with cataplexy (mean ± SEM; 176.0 ± 25.8 pg/mL), hypocretin non-deficient narcolepsy with cataplexy (97.8 ± 38.4 pg/mL), hypocretin non-deficient narcolepsy without cataplexy (113.6 ± 16.4 pg/mL), and idiopathic hypersomnia (161.0 ± 29.3 pg/mL); the levels in OSAS (259.3 ± 46.6 pg/mL) did not statistically differ from those in the controls (333.8 ± 22.0 pg/mL). Low CSF histamine levels were mostly observed in non-medicated patients; significant reductions in histamine levels were evident in non-medicated patients with hypocretin deficient narcolepsy with cataplexy (112.1 ± 16.3 pg/mL) and idiopathic hypersomnia (143.3 ± 28.8 pg/mL), while the levels in the medicated patients were in the normal range. Conclusion: The study confirmed reduced CSF histamine levels in hypocretin-deficient narcolepsy with cataplexy. Similar degrees of reduction were also observed in hypocretin non-deficient narcolepsy and in idiopathic hypersomnia, while those in OSAS (non central nervous system hypersomnia) were not altered. The decrease in histamine in these subjects were more specifically observed in non-medicated subjects, suggesting CSF histamine is a biomarker reflecting the degree of hypersomnia of central origin. Citation: Kanbayashi T; Kodama T; Kondo H; Satoh S; Inoue Y; Chiba S; Shimizu T; Nishino S. CSF

Of PCOS symptoms, hirsutism has the most significant impact on the quality of life of Iranian women.

PubMed

Khomami, Mahnaz Bahri; Tehrani, Fahimeh Ramezani; Hashemi, Somayeh; Farahmand, Maryam; Azizi, Fereidoun

2015-01-01

Polycystic ovary syndrome is a common endocrine disorder affecting women both physically and psychologically and can lead to a poor quality of life compared to their normal counterparts. The aim of the present study was to assess the impact of various clinical features of polycystic ovary syndrome on the health-related quality of life of Iranian women diagnosed with this syndrome. A total of 796 women diagnosed with polycystic ovary syndrome, aged 15-49 years, completed the questionnaires, interviews, and medical assessments required for this study. A reliable and validated Persian version of the health-related quality of life questionnaire for polycystic ovary syndrome patients was used. Linear regression models were used to assess the association between the symptoms of polycystic ovary syndrome and health-related quality of life. The mean age of participants was 28.02 years. 35.4% of the subjects were classified as overweight or obese. Hirsutism, was reported to have the strongest impact on the patients' health-related quality of life, followed in descending order by body mass index, irregular menses and infertility. The relative level of hirsutism was directly proportional to decrease in health-related quality of life score (p<0.001). The results of the study found that hirsutism had the strongest impact on the health-related quality of life measures in Iranian women diagnosed with polycystic ovary syndrome. Health care officials need to evaluate in depth the effect of each clinical feature of polycystic ovary syndrome separately and design management strategies, keeping in mind the psychological and physical manifestations.

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

PubMed

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

2010-09-01

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

High dendritic expression of Ih in the proximity of the axon origin controls the integrative properties of nigral dopamine neurons.

PubMed

Engel, Dominique; Seutin, Vincent

2015-11-15

The hyperpolarization-activated cation current Ih is expressed in dopamine neurons of the substantia nigra, but the subcellular distribution of the current and its role in synaptic integration remain unknown. We used cell-attached patch recordings to determine the localization profile of Ih along the somatodendritic axis of nigral dopamine neurons in slices from young rats. Ih density is higher in axon-bearing dendrites, in a membrane area close to the axon origin, than in the soma and axon-lacking dendrites. Dual current-clamp recordings revealed a similar contribution of Ih to the waveform of single excitatory postsynaptic potentials throughout the somatodendritic domain. The Ih blocker ZD 7288 increased the temporal summation in all dendrites with a comparable effect in axon- and non-axon dendrites. The strategic position of Ih in the proximity of the axon may influence importantly transitions between pacemaker and bursting activities and consequently the downstream release of dopamine. Dendrites of most neurons express voltage-gated ion channels in their membrane. In combination with passive properties, active currents confer to dendrites a high computational potential. The hyperpolarization-activated cation current Ih present in the dendrites of some pyramidal neurons affects their membrane and integration properties, synaptic plasticity and higher functions such as memory. A gradient of increasing h-channel density towards distal dendrites has been found to be responsible for the location independence of excitatory postsynaptic potential (EPSP) waveform and temporal summation in cortical and hippocampal pyramidal cells. However, reports on other cell types revealed that smoother gradients or even linear distributions of Ih can achieve homogeneous temporal summation. Although the existence of a robust, slowly activating Ih current has been repeatedly demonstrated in nigral dopamine neurons, its subcellular distribution and precise role in synaptic integration

Hyperpolarization-activated current (I(h)) in vestibular calyx terminals: characterization and role in shaping postsynaptic events.

PubMed

Meredith, Frances L; Benke, Tim A; Rennie, Katherine J

2012-12-01

Calyx afferent terminals engulf the basolateral region of type I vestibular hair cells, and synaptic transmission across the vestibular type I hair cell/calyx is not well understood. Calyces express several ionic conductances, which may shape postsynaptic potentials. These include previously described tetrodotoxin-sensitive inward Na(+) currents, voltage-dependent outward K(+) currents and a K(Ca) current. Here, we characterize an inwardly rectifying conductance in gerbil semicircular canal calyx terminals (postnatal days 3-45), sensitive to voltage and to cyclic nucleotides. Using whole-cell patch clamp, we recorded from isolated calyx terminals still attached to their type I hair cells. A slowly activating, noninactivating current (I(h)) was seen with hyperpolarizing voltage steps negative to the resting potential. External Cs(+) (1-5 mM) and ZD7288 (100 μM) blocked the inward current by 97 and 83 %, respectively, confirming that I(h) was carried by hyperpolarization-activated, cyclic nucleotide gated channels. Mean half-activation voltage of I(h) was -123 mV, which shifted to -114 mV in the presence of cAMP. Activation of I(h) was well described with a third order exponential fit to the current (mean time constant of activation, τ, was 190 ms at -139 mV). Activation speeded up significantly (τ=136 and 127 ms, respectively) when intracellular cAMP and cGMP were present, suggesting that in vivo I(h) could be subject to efferent modulation via cyclic nucleotide-dependent mechanisms. In current clamp, hyperpolarizing current steps produced a time-dependent depolarizing sag followed by either a rebound afterdepolarization or an action potential. Spontaneous excitatory postsynaptic potentials (EPSPs) became larger and wider when I(h) was blocked with ZD7288. In a three-dimensional mathematical model of the calyx terminal based on Hodgkin-Huxley type ionic conductances, removal of I(h) similarly increased the EPSP, whereas cAMP slightly decreased simulated EPSP size

Diurnal and nocturnal cardiovascular variability and heart rate arousal response in idiopathic hypersomnia.

PubMed

Sforza, Emilia; Roche, Frédéric; Barthélémy, Jean Claude; Pichot, Vincent

2016-08-01

Autonomic nervous system dysfunction has been described in narcolepsy with cataplexy affecting sympathetic functions. In this study we analyzed whether altered diurnal and nocturnal cardiovascular control is present in idiopathic hypersomnia (IH). Fourteen drug-free patients aged 26.2 ± 7 years and 14 age-matched controls were examined. Clinical data, 24-h polysomnography, heart rate (HR) variability, and the HR response to spontaneous arousal were available. Sleep macrostructure was comparable between controls and patients, with the latter having significantly longer sleep time, a higher number of sleep cycles (p < 0.0001), and low sleep efficiency (p < 0.01). The HR variability indices did not differ between groups, except for the rise of high frequency (HF) and HFnu in patients (p < 0.05) associated with blunted sympathetic indices (p < 0.01). These parasympathetic alterations were present for light, slow wave, and rapid eye-movement sleep and persisted for all sleep cycles. Compared to controls, the HR arousal response was significantly higher (p < 0.01) in patients starting before the arousal onset and persisting into the post-arousal period. In IH patients a dysfunction of the parasympathetic activity during awake and sleep and an altered autonomic response to arousals are present. These findings suggest an impaired parasympathetic function that may explain some vegetative symptoms present in this type of central hypersomnia. Copyright © 2016 Elsevier B.V. All rights reserved.

The Evolution of Ih C_60 Vibrational Modes in Planar Polymerized C_60.

NASA Astrophysics Data System (ADS)

Adams, G. B.; Page, J. B.

2001-03-01

We have used first-principles local-orbital-based molecular dynamics(O.F. Sankey and D.J. Niklewski, Phys. Rev. B40), 3979 (1989). to simulate a wide variety of planar polymers of C_60, including the orthorhombic (O), tetrahedral (T), and rhombohedral (R) polymers which have been reported experimentally. It has been customary to assume that the vibrational modes of the polymers are moderately perturbed Ih C_60 vibrational modes.(See, for example V.A. Davydov et al.), Phys. Rev. B61, 11936 (2000) or V.C. Long et al., Phys. Rev. B 61, 13191 (2000). To test this assumption, we have expanded the polymer vibrational eigenvectors in the eigenvectors of Ih C_60, thus determining quantitatively the percentage contribution of each Ih C_60 mode to each polymer vibrational mode. We find that for many polymer modes the assumption is not justified. We report our results for selected Raman- and IR-active vibrational modes of the observed polymers.

Green light inhibits GnRH-I expression by stimulating the melatonin-GnIH pathway in the chick brain.

PubMed

Zhang, L; Chen, F; Cao, J; Dong, Y; Wang, Z; Hu, M; Chen, Y

2017-05-01

To study the mechanism by which monochromatic light affects gonadotrophin-releasing hormone (GnRH) expression in chicken hypothalamus, a total of 192 newly-hatched chicks were divided into intact, sham-operated and pinealectomy groups and exposed to white (WL), red (RL), green (GL) and blue (BL) lights using a light-emitting diode system for 2 weeks. In the GL intact group, the mRNA and protein levels of GnRH-I in the hypothalamus, the mean cell area and mean cell optical density (OD) of GnRH-I-immunoreactive (-ir) cells of the nucleus commissurae pallii were decreased by 13.2%-34.5%, 5.7%-39.1% and 9.9%-17.3% compared to those in the chicks exposed to the WL, RL and BL, respectively. GL decreased these factors related to GnRH-I expression and the effect of GL was not observed in pinealectomised birds. However, the mRNA and protein levels of hypothalamic gonadotrophin-inhibitory hormone (GnIH) and GnIH receptor (GnIHR), the mean cell area and mean cell OD of the GnIH-ir cells of the paraventricularis magnocellularis, and the plasma melatonin concentration in the chicks exposed to GL were increased by 18.6%-49.2%, 21.1%-60.0% and 8.6%-30.6% compared to the WL, RL and BL intact groups, respectively. The plasma melatonin concentration showed a negative correlation with GnRH-I protein and a positive correlation with GnIH and GnIHR proteins. Protein expression of both GnRH-I and GnIHR showed a negative correlation in the hypothalamus. After pinealectomy, GnRH-I expression increased, whereas plasma melatonin concentration, GnIH and GnIHR expression decreased, and there were no significant differences among the WL, RL, GL and BL groups. Double-labelled immunofluorescence showed that GnIH axon terminals were near GnRH-I neurones, some GnRH-I neurones coexpressed with GnIHR and GnIH neurones coexpressed with melatonin receptor subtype quinone reductase 2. These results demonstrate that green light inhibits GnRH-I expression by increasing melatonin secretion and stimulating

Second-order many-body perturbation study of ice Ih

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

He, Xiao; Sode, Olaseni; Xantheas, Sotiris S.

2012-11-28

Ice Ih is arguably the most important molecular crystal in nature, yet our understanding of its structural and dynamical properties is still incomplete. To explain the origin of two peaks in the hydrogen-bond-stretching region of the inelastic neutron scattering (INS) spectra, the existence of two types of hydrogen bonds with strengths differing by a factor of two was previously hypothesized. We present first-principles calculations based on diagrammatic many-body perturbation theory of the structures and vibrational spectra of ice Ih, which suggest that the observed spectral features arise from the directionality or anisotropy of the hydrogen-bond stretching vibrations rather than theirmore » vastly different force constants, disproving the previous hypothesis. Our calculations also reproduce the infrared and Raman spectra, the variation of INS spectra with deuterium concentration, and the anomaly of heat capacities at low temperatures, together rendering our calculations a paradigm for "crystals from first principles" as envisioned by Maddox.« less

The evaluation of the relationship between some related hormone levels and diet in obese or overweight patients with hirsutism: A randomized clinical trial

PubMed Central

Krouni, Atefeh; Forouhari, Sedighe; Namavarjahromi, Bahia; Dabbaghmanesh, Mohammad Hossein; Shayan, Arezoo; Sepasi, Sara; Zare, Atefeh

2017-01-01

Introduction: Hirsutism is a common disorder that has remarkable physical and mental effects on individuals. No appropriate diet has yet specified for individuals with hirsutism. The present study was carried out to examine the effect of high-fibre, low-caloric balanced diet on some related hormone levels in obese or overweight women with hirsutism who had referred to clinics affiliated with Shiraz University of Medical Sciences. Materials and Methods: The present study was a clinical trial that was carried out on 47 obese or overweight women with hirsutism in 2014. The women were randomly assigned to an intervention group and a control group that, respectively, consumed a high-fiber, low-caloric balanced diet and a normal diet for 3 months. A demographic characteristics questionnaire and a researcher-designed diet questionnaire were filled out by the two groups before the intervention. Before and 12 weeks after the intervention, body mass index (BMI) was measured and blood samples (on the 3–5 days of menstruation) were collected. Factors of luteinizing hormone, follice stimulating hormone, sex hormone binding globulin, dehydroepiandrosterone sulfate, low-density lipoproteins, high-density lipoproteins (HDL), fasting blood sugar (FBS), CHOL, prolactin, triglycerides, insulin, 17-hydroxyprogesterone, and free androstenedione testosterone were measured. The collected data were analyzed through t-test, Chi-square, and intergroup analysis using SPSS 22.0. Results: The mean age of the participating women was 27.23 ± 5.42 years. After the study, the level of FBS and insulin in the intervention group dropped while they increased in the control group. Moreover, the postintervention level of BMI in the intervention group on average decreased 1.89 units while it rose by 0.3 units in the control group, and there was a significant difference between the two groups (P < 0.001). Conclusion: The results of the present study showed that consuming high-fiber diet by obese or

Idiopathic anaphylaxis.

PubMed

Fenny, Nana; Grammer, Leslie C

2015-05-01

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

Transition mechanism of sH to filled-ice Ih structure of methane hydrate under fixed pressure condition

NASA Astrophysics Data System (ADS)

Kadobayashi, H.; Hirai, H.; Ohfuji, H.; Kojima, Y.; Ohishi, Y.; Hirao, N.; Ohtake, M.; Yamamoto, Y.

2017-10-01

The phase transition mechanism of methane hydrate from sH to filled-ice Ih structure was examined using a combination of time-resolved X-ray diffractometry (XRD) and Raman spectroscopy in conjunction with charge-coupled device (CCD) camera observation under fixed pressure conditions. Prior to time-resolved Raman experiments, the typical C-H vibration modes and their pressure dependence of three methane hydrate structures, fluid methane and solid methane were measured using Raman spectroscopy to distinguish the phase transitions of methane hydrates from decomposition to solid methane and ice VI or VII. Experimental results by XRD, Raman spectroscopy and CCD camera observation revealed that the structural transition of sH to filled-ice Ih occurs through a collapse of the sH framework followed by the release of fluid methane that is then gradually incorporated into the filled-ice Ih to reconstruct its structure. These observations suggest that the phase transition of sH to filled-ice Ih takes place by a typical reconstructive mechanism.

A crystalline anionic complex of scandium nitride endometallofullerene: experimental observation of single-bonded (Sc3N@Ih-C80−)2 dimers†

PubMed Central

Konarev, Dmitri V.; Zorina, Leokadiya V.; Khasanov, Salavat S.; Popov, Alexey A.; Otsuka, Akihiro; Yamochi, Hideki; Saito, Gunzi; Lyubovskaya, Rimma N.

2017-01-01

Reduction of scandium nitride clusterfullerene, Sc3N@Ih-C80, by sodium fluorenone ketyl in the presence of cryptand[2,2,2] allows the crystallization of the {cryptand[2,2,2](Na+)}2(Sc3N@Ih-C80−)2·2.5C6H4Cl2 (1) salt. The Sc3N@Ih-C80•− radical anions are dimerized to form single-bonded (Sc3N@Ih-C80−)2 dimers. PMID:27511304

Transforming wealth: using the inverse hyperbolic sine (IHS) and splines to predict youth's math achievement.

PubMed

Friedline, Terri; Masa, Rainier D; Chowa, Gina A N

2015-01-01

The natural log and categorical transformations commonly applied to wealth for meeting the statistical assumptions of research may not always be appropriate for adjusting for skewness given wealth's unique properties. Finding and applying appropriate transformations is becoming increasingly important as researchers consider wealth as a predictor of well-being. We present an alternative transformation-the inverse hyperbolic sine (IHS)-for simultaneously dealing with skewness and accounting for wealth's unique properties. Using the relationship between household wealth and youth's math achievement as an example, we apply the IHS transformation to wealth data from US and Ghanaian households. We also explore non-linearity and accumulation thresholds by combining IHS transformed wealth with splines. IHS transformed wealth relates to youth's math achievement similarly when compared to categorical and natural log transformations, indicating that it is a viable alternative to other transformations commonly used in research. Non-linear relationships and accumulation thresholds emerge that predict youth's math achievement when splines are incorporated. In US households, accumulating debt relates to decreases in math achievement whereas accumulating assets relates to increases in math achievement. In Ghanaian households, accumulating assets between the 25th and 50th percentiles relates to increases in youth's math achievement. Copyright © 2014 Elsevier Inc. All rights reserved.

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

PubMed

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

2015-01-01

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

[Regulation of pyloric rhythm by I(A) and I(h) in crayfish stomatogastric ganglion].

PubMed

Kuang, Guo-Hui; Liu, Yi-Hui; Ren, Wei

2012-06-25

The stomatogastric ganglion (STG) of shellfish includes 30 neurons and produces pyloric rhythms. It is the common model to study central pattern generator (CPG). Regulation of pyloric rhythms not only is related to the property of single neurons in STG but also depends on the connections and property of the whole neuronal network. It has been found that transient potassium current (I(A)) and hyperpolarization-activated cation current (I(h)) exist in certain types of neurons of STG. However, roles played by these two currents in maintaining and regulating the pyloric rhythms are unknown. In the present study, in vitro electrophysiological recordings were performed on crayfish STG to examine the role played by I(A) and I(h) in regulation of pyloric rhythm. 4AP (2 mmol/L), a specific inhibitor of I(A), caused a decrease in pyloric cycle (P < 0.01), an increase in PD (pyloric dilator) ratio, a decrease in PY (pyloric) ratio (P < 0.01) and delay of phases of LP and PY firing. ZD7288 (100 μmol/L), a specific inhibitor of I(h), caused a decrease in pyloric cycle (P < 0.01), an increase in PD ratio (P < 0.01), an increase in LP (lateral pyloric) ratio (P < 0.01), a decrease in PY ratio (P < 0.01) and delay of phases of LP and PY firing. These results indicate that I(A) and I(h) play important roles in regulating pyloric rhythms in crayfish STG.

Comparison between sequentional treatment with diode and alexandrite lasers versus alexandrite laser alone in the treatment of hirsutism.

PubMed

Nilforoushzadeh, Mohammad Ali; Naieni, Farahnaz Fatemi; Siadat, Amir Hossein; Rad, Leila

2011-11-01

Laser systems that are commonly used for the treatment of hirsutism include the ruby laser (694 nm), the diode laser (800 nm), the alexandrite laser (755 nm) and the Nd:YAG laser (1084 nm). The diode laser and alexandrite laser are considered effective in treatment of hirsutism in dark-skinned patients. The response of hairs to these laser systems is variable and not complete. In this study, we compared the efficacy of these two laser systems for permanent hair removal. This was a randomized, controlled clinical trial that was performed with women of the age range 15-45 years old. After obtaining informed consent, the samples were randomized into two groups using random allocation software. The first group was treated with alexandrite laser alone (four sessions, two months apart). The second group was treated sequentially with diode laser for the first two sessions and alexandrite laser for the next two sessions. Overall, 111 patients (57 patients in the alexandrite laser group and 54 patients in the sequential diode-alexandrite laser group) were evaluated. There was no significant difference regarding mean of hair reduction between the two groups during the courses of treatment. Except for the first session, there was no significant difference regarding percent of patient satisfaction between the two groups (P value >0.05). Comparison between the two groups showed no significant difference one month, three months and six months after the last treatment (P value >0.05). Regarding the results of our study, there is no significant difference between sequential treatment with diode and alexandrite lasers versus alexandrite laser alone in the treatment of hirsutism. We suggest that in further studies, the efficacy of sequential treatment with other laser systems is evaluated against single treatment methods.

Distinct perinatal features of the hyperpolarization-activated non-selective cation current Ih in the rat cortical plate

PubMed Central

2012-01-01

Background During neocortical development, multiple voltage- and ligand-gated ion channels are differentially expressed in neurons thereby shaping their intrinsic electrical properties. One of these voltage-gated ion channels, the hyperpolarization-activated cyclic nucleotide-gated (HCN) channel and its current Ih, is an important regulator of neuronal excitability. Thus far, studies on an early Ih appearance in rodent neocortex are missing or conflicting. Therefore, we focused our study on perinatal neocortical Ih and its properties. Results In the perinatal rat neocortex we observed a rapid increase in the number of neurons exhibiting Ih. Perinatal Ih had unique properties: first, a pronounced cAMP sensitivity resulting in a marked shift of the voltage sufficient for half-maximum activation of the current towards depolarized voltages and second, an up to 10 times slower deactivation at physiological membrane potentials when compared to the one at postnatal day 30. The combination of these features was sufficient to suppress membrane resonance in our in silico and in vitro experiments. Although all four HCN subunits were present on the mRNA level we only detected HCN4, HCN3 and HCN1 on the protein level at P0. HCN1 protein at P0, however, appeared incompletely processed. At P30 glycosilated HCN1 and HCN2 dominated. By in silico simulations and heterologous co-expression experiments of a ‘slow’ and a ‘fast’ Ih conducting HCN channel subunit in HEK293 cells, we mimicked most characteristics of the native current, pointing to a functional combination of subunit homo- or heteromeres. Conclusion Taken together, these data indicate a HCN subunit shift initiated in the first 24 hours after birth and implicate a prominent perinatal role of the phylogenetically older HCN3 and/or HCN4 subunits in the developing neocortex. PMID:22694806

Modafinil in the treatment of idiopathic hypersomnia without long sleep time--a randomized, double-blind, placebo-controlled study.

PubMed

Mayer, Geert; Benes, Heike; Young, Peter; Bitterlich, Marion; Rodenbeck, Andrea

2015-02-01

In 2010 the European Medicines Agency withdrew the indication of modafinil for the treatment of obstructive sleep apnea, shift work sleep disorder and for idiopathic hypersomnia (IH). In uncontrolled studies, modafinil has been reported to be efficacious in the treatment of sleep disorders. We therefore performed a randomized, placebo-controlled study with the aim of proving the efficacy of modafinil treatment in these patients. Drug-free IH patients without long sleep according to ICSD2 criteria, age >18 years and disease duration >2 years were included. After a washout phase, patients at baseline received placebo or 100 mg modafinil in the morning and at noon over 3 weeks, followed by 1 week without medication. At each visit the Epworth Sleepiness Scale (ESS) and Clinical Global Impression (CGI) rating scale were performed. At baseline and on days 8 and 21 four Maintenance of Wakefulness Tests (MWTs)/day or per day were performed. Patients kept a sleep-wake diary throughout the study. Between 2009 and 2011 three sleep centres recruited 33 participants. Compared to placebo, modafinil decreased sleepiness significantly and improved mean sleep latency in the MWT non-significantly. The CGI improved significantly from baseline to the last visit on treatment. The most frequent adverse events were headaches and gastrointestinal disorders; skin and psychiatric reactions were not reported. The number of reported naps and duration of daytime sleepiness decreased significantly. Total sleep time of nocturnal sleep was slightly reduced. The sleep diaries showed increases in feeling refreshed in the morning; the diurnal diaries showed significant improvement of performance and of exhaustion. Modafinil is an effective and safe medication in the treatment of IH. Adverse events are mild to moderate. © 2014 European Sleep Research Society.

Predicting the melting temperature of ice-Ih with only electronic structure information as input.

PubMed

Pinnick, Eric R; Erramilli, Shyamsunder; Wang, Feng

2012-07-07

The melting temperature of ice-Ih was calculated with only electronic structure information as input by creating a problem-specific force field. The force field, Water model by AFM for Ice and Liquid (WAIL), was developed with the adaptive force matching (AFM) method by fitting to post-Hartree-Fock quality forces obtained in quantum mechanics∕molecular mechanics calculations. WAIL predicts the ice-Ih melting temperature to be 270 K. The model also predicts the densities of ice and water, the temperature of maximum density of water, the heat of vaporizations, and the radial distribution functions for both ice and water in good agreement with experimental measurements. The non-dissociative WAIL model is very similar to a flexible version of the popular TIP4P potential and has comparable computational cost. By customizing to problem-specific configurations with the AFM approach, the resulting model is remarkably more accurate than any variants of TIP4P for simulating ice-Ih and water in the temperature range from 253 K and 293 K under ambient pressure.

Elastic wave speeds and moduli in polycrystalline ice Ih, si methane hydrate, and sll methane-ethane hydrate

USGS Publications Warehouse

Helgerud, M.B.; Waite, W.F.; Kirby, S.H.; Nur, A.

2009-01-01

We used ultrasonic pulse transmission to measure compressional, P, and shear, S, wave speeds in laboratory-formed polycrystalline ice Ih, si methane hydrate, and sll methane-ethane hydrate. From the wave speed's linear dependence on temperature and pressure and from the sample's calculated density, we derived expressions for bulk, shear, and compressional wave moduli and Poisson's ratio from -20 to 15??C and 22.4 to 32.8 MPa for ice Ih, -20 to 15??C and 30.5 to 97.7 MPa for si methane hydrate, and -20 to 10??C and 30.5 to 91.6 MPa for sll methane-ethane hydrate. All three materials had comparable P and S wave speeds and decreasing shear wave speeds with increasing applied pressure. Each material also showed evidence of rapid intergranular bonding, with a corresponding increase in wave speed, in response to pauses in sample deformation. There were also key differences. Resistance to uniaxial compaction, indicated by the pressure required to compact initially porous samples, was significantly lower for ice Ih than for either hydrate. The ice Ih shear modulus decreased with increasing pressure, in contrast to the increase measured in both hydrates ?? 2009.

25 CFR 900.96 - How can Indian tribes or tribal organizations learn about BIA and IHS excess property?

Code of Federal Regulations, 2010 CFR

2010-04-01

... 25 Indians 2 2010-04-01 2010-04-01 false How can Indian tribes or tribal organizations learn about BIA and IHS excess property? 900.96 Section 900.96 Indians BUREAU OF INDIAN AFFAIRS, DEPARTMENT OF THE... Property § 900.96 How can Indian tribes or tribal organizations learn about BIA and IHS excess property...

Service Networks and Patterns of Utilization: Mental Health Programs, Indian Health Service (IHS). Volume 1: Overview and Recommendations.

ERIC Educational Resources Information Center

Attneave, Carolyn L.; Beiser, Morton

Constituting an overview of a 10-volume report on the historical development and contemporary activities (1966-1973) of each of the 8 administrative Area Offices of the Indian Health Service (IHS) Mental Health Programs, this volume includes: the methods used for data collection (personal interviews with both past and present IHS key officials,…

25 CFR 900.96 - How can Indian tribes or tribal organizations learn about BIA and IHS excess property?

Code of Federal Regulations, 2011 CFR

2011-04-01

... 25 Indians 2 2011-04-01 2011-04-01 false How can Indian tribes or tribal organizations learn about BIA and IHS excess property? 900.96 Section 900.96 Indians BUREAU OF INDIAN AFFAIRS, DEPARTMENT OF THE... Property § 900.96 How can Indian tribes or tribal organizations learn about BIA and IHS excess property...

Path-integral simulation of ice Ih: The effect of pressure

NASA Astrophysics Data System (ADS)

Herrero, Carlos P.; Ramírez, Rafael

2011-12-01

The effect of pressure on structural and thermodynamic properties of ice Ih has been studied by means of path-integral molecular dynamics simulations at temperatures between 50 and 300 K. Interatomic interactions were modeled by using the effective q-TIP4P/F potential for flexible water. Positive (compression) and negative (tension) pressures have been considered, which allowed us to approach the limits for the mechanical stability of this solid water phase. We have studied the pressure dependence of the crystal volume, bulk modulus, interatomic distances, atomic delocalization, and kinetic energy. The spinodal point at both negative and positive pressures is derived from the vanishing of the bulk modulus. For P<0, the spinodal pressure changes from -1.38 to - 0.73 GPa in the range from 50 to 300 K. At positive pressure the spinodal is associated with ice amorphization, and at low temperatures it is found to be between 1.1 and 1.3 GPa. Quantum nuclear effects cause a reduction of the metastability region of ice Ih.

Health-Related Quality of Life Among Drug-Naïve Patients with Narcolepsy with Cataplexy, Narcolepsy Without Cataplexy, and Idiopathic Hypersomnia Without Long Sleep Time

PubMed Central

Ozaki, Akiko; Inoue, Yuichi; Nakajima, Toru; Hayashida, Kenichi; Honda, Makoto; Komada, Yoko; Takahashi, Kiyohisa

2008-01-01

Objective: To evaluate the health-related quality life (HRQOL) of drugnaïve patients with narcolepsy with cataplexy (NA with CA), narcolepsy without cataplexy (NA without CA) and idiopathic hypersomnia without long sleep time (IHS without LST), and to explore the factors influencing the HRQOL. Factors associated with the occurrence of automobile accidents are also discussed. Methods: A total of 137 consecutive drug naïve patients who met the criteria of the 2nd edition of the International Classification of Sleep Disorders (NA with CA, n = 28; NA without CA, n = 27; IHS without LST, n = 82) were enrolled. The patients were asked to fill out questionnaires, including the SF-36, Epworth Sleepiness Scale (ESS), sociodemographic variables, and items regarding driving habits and the experiences related to automobile accidents. Results: All 3 diagnostic groups had significantly lower scores in most SF-36 domains compared with Japanese normative data. Significant differences among the 3 diagnostic groups were not observed. Specific factors in SF-36 domains were not found with multiple linear regression analyses, while disease duration was positively correlated with mental health among all subjects. Among the patients reporting driving habits, ESS score (≥16) was positively associated with the experience of automobile accidents. Conclusions: Our results indicated that HRQOL decreases in drugnaïve patients with hypersomnia, but neither disease category nor severity of the disorder appears as an associated factor. Increased severity of hypersomnia, however, was thought to play an important role in the occurrence of automobile accidents. Citation: Ozaki A; Inoue Y; Nakajima T; Hayashida K; Honda M; Komada Y; Takahashi K. Health-related quality of life among drug-naïve patients with narcolepsy with cataplexy, narcolepsy without cataplexy, and idiopathic hypersomnia without long sleep time. J Clin Sleep Med 2008;4(6):572-578. PMID:19110887

Variants in the 5alpha-reductase type 1 and type 2 genes are associated with polycystic ovary syndrome and the severity of hirsutism in affected women.

PubMed

Goodarzi, Mark O; Shah, Nissar A; Antoine, Heath J; Pall, Marita; Guo, Xiuqing; Azziz, Ricardo

2006-10-01

Despite the importance of dihydrotestosterone in androgen action, polymorphisms in the genes for the two isoforms of 5alpha-reductase (SRD5A1 and SRD5A2) have not been evaluated as risk factors for polycystic ovary syndrome (PCOS). The objective of the study was to test the hypothesis that haplotypes in the SRD5A1 and SRD5A2 genes are risk factors for PCOS and the severity of hirsutism in affected women. PCOS and control subjects were genotyped for seven single-nucleotide polymorphisms in SRD5A1 and eight single-nucleotide polymorphisms in SRD5A2. Haplotypes were determined and tested for association with PCOS diagnosis and component phenotypes. Subjects were recruited from the reproductive endocrinology clinic at the University of Alabama at Birmingham; control subjects were recruited from the general surrounding community. Genotyping took place at Cedars-Sinai Medical Center in Los Angeles. A total of 287 White women with PCOS and 187 controls participated. SRD5A1 and SRD5A2 genotype, quantitative hirsutism score, and hormonal and metabolic phenotypes were assessed. Haplotypes within both genes were associated with PCOS risk. The Leu allele of the Val89Leu variant in SRD5A2 was associated with protection against PCOS; this allele is known to modestly reduce 5alpha-reductase activity. Haplotypes in SRD5A1 but not SRD5A2 were also associated with the degree of hirsutism in affected women. This study presents genetic evidence suggesting an important role of both isoforms of 5alpha-reductase in the pathogenesis of PCOS. That only SRD5A1 haplotypes were associated with hirsutism suggests that only this isoform is important in the hair follicle.

Full-scale bonded concrete overlay on IH-30 in Ft. Worth, Texas

DOT National Transportation Integrated Search

2000-10-01

This report presents the research and recommendations regarding the rehabilitation of an urban section of IH-30 in west Fort Worth, Texas. Bonded concrete overlays have proven to be a viable solution for rehabilitation of heavily traveled pavement se...

Ice Ih anomalies: Thermal contraction, anomalous volume isotope effect, and pressure-induced amorphization.

PubMed

Salim, Michael A; Willow, Soohaeng Yoo; Hirata, So

2016-05-28

Ice Ih displays several anomalous thermodynamic properties such as thermal contraction at low temperatures, an anomalous volume isotope effect (VIE) rendering the volume of D2O ice greater than that of H2O ice, and a pressure-induced transition to the high-density amorphous (HDA) phase. Furthermore, the anomalous VIE increases with temperature, despite its quantum-mechanical origin. Here, embedded-fragment ab initio second-order many-body perturbation (MP2) theory in the quasiharmonic approximation (QHA) is applied to the Gibbs energy of an infinite, proton-disordered crystal of ice Ih at wide ranges of temperatures and pressures. The quantum effect of nuclei moving in anharmonic potentials is taken into account from first principles without any empirical or nonsystematic approximation to either the electronic or vibrational Hamiltonian. MP2 predicts quantitatively correctly the thermal contraction at low temperatures, which is confirmed to originate from the volume-contracting hydrogen-bond bending modes (acoustic phonons). It qualitatively reproduces (but underestimates) the thermal expansion at higher temperatures, caused by the volume-expanding hydrogen-bond stretching (and to a lesser extent librational) modes. The anomalous VIE is found to be the result of subtle cancellations among closely competing isotope effects on volume from all modes. Consequently, even ab initio MP2 with the aug-cc-pVDZ and aug-cc-pVTZ basis sets has difficulty reproducing this anomaly, yielding qualitatively varied predictions of the sign of the VIE depending on such computational details as the choice of the embedding field. However, the temperature growth of the anomalous VIE is reproduced robustly and is ascribed to the librational modes. These solid-state MP2 calculations, as well as MP2 Born-Oppenheimer molecular dynamics, find a volume collapse and a loss of symmetry and long-range order in ice Ih upon pressure loading of 2.35 GPa or higher. Concomitantly, rapid softening of

Ice Ih anomalies: Thermal contraction, anomalous volume isotope effect, and pressure-induced amorphization

NASA Astrophysics Data System (ADS)

Salim, Michael A.; Willow, Soohaeng Yoo; Hirata, So

2016-05-01

Ice Ih displays several anomalous thermodynamic properties such as thermal contraction at low temperatures, an anomalous volume isotope effect (VIE) rendering the volume of D2O ice greater than that of H2O ice, and a pressure-induced transition to the high-density amorphous (HDA) phase. Furthermore, the anomalous VIE increases with temperature, despite its quantum-mechanical origin. Here, embedded-fragment ab initio second-order many-body perturbation (MP2) theory in the quasiharmonic approximation (QHA) is applied to the Gibbs energy of an infinite, proton-disordered crystal of ice Ih at wide ranges of temperatures and pressures. The quantum effect of nuclei moving in anharmonic potentials is taken into account from first principles without any empirical or nonsystematic approximation to either the electronic or vibrational Hamiltonian. MP2 predicts quantitatively correctly the thermal contraction at low temperatures, which is confirmed to originate from the volume-contracting hydrogen-bond bending modes (acoustic phonons). It qualitatively reproduces (but underestimates) the thermal expansion at higher temperatures, caused by the volume-expanding hydrogen-bond stretching (and to a lesser extent librational) modes. The anomalous VIE is found to be the result of subtle cancellations among closely competing isotope effects on volume from all modes. Consequently, even ab initio MP2 with the aug-cc-pVDZ and aug-cc-pVTZ basis sets has difficulty reproducing this anomaly, yielding qualitatively varied predictions of the sign of the VIE depending on such computational details as the choice of the embedding field. However, the temperature growth of the anomalous VIE is reproduced robustly and is ascribed to the librational modes. These solid-state MP2 calculations, as well as MP2 Born-Oppenheimer molecular dynamics, find a volume collapse and a loss of symmetry and long-range order in ice Ih upon pressure loading of 2.35 GPa or higher. Concomitantly, rapid softening of

Juvenile Idiopathic Arthritis

MedlinePlus

... Is Juvenile Idiopathic Arthritis the same as Juvenile Rheumatoid Arthritis? Yes, Juvenile Idiopathic Arthritis (JIA) is a new ... of chronic inflammatory diseases that affect children. Juvenile Rheumatoid Arthritis (JRA) is the older term that was used ...

Idiopathic Intracranial Hypertension (Pseudotumor Cerebri)

MedlinePlus

... Asked Questions Español Condiciones Chinese Conditions Idiopathic Intracranial Hypertension (Pseudotumor Cerebri) En Español Read in Chinese What is idiopathic intracranial hypertension? Idiopathic intracranial hypertension (IIH) is a disorder that ...

Neural Versus Gonadal GnIH: Are they Independent Systems? A Mini-Review.

PubMed

Bentley, George E; Wilsterman, Kathryn; Ernst, Darcy K; Lynn, Sharon E; Dickens, Molly J; Calisi, Rebecca M; Kriegsfeld, Lance J; Kaufer, Daniela; Geraghty, Anna C; viviD, Dax; McGuire, Nicolette L; Lopes, Patricia C; Tsutsui, Kazuyoshi

2017-12-01

Based on research in protochordates and basal vertebrates, we know that communication across the first endocrine axes likely relied on diffusion. Because diffusion is relatively slow, rapid responses to some cues, including stress-related cues, may have required further local control of axis outputs (e.g., steroid hormone production by the gonads). Despite the evolution of much more efficient circulatory systems and complex nervous systems in vertebrates, production of many "neuro"transmitters has been identified outside of the hypothalamus across the vertebrate phylogeny and these neurotransmitters are known to locally regulate endocrine function. Our understanding of tissue-specific neuropeptide expression and their role coordinating physiological/behavioral responses of the whole organism remains limited, in part, due to nomenclature and historic dogma that ignores local regulation of axis output. Here, we review regulation of gonadotropin-inhibitory hormone (GnIH) across the reproductive axis in birds and mammals to bring further attention to context-dependent disparities and similarities in neuropeptide production by the brain and gonads. We find that GnIH responsiveness to cues of stress appears conserved across species, but that the response of specific tissues and the direction of GnIH regulation varies. The implications of differential regulation across tissues remain unclear in most studies, but further work that manipulates and contrasts function in different tissues has the potential to inform us about both organism-specific function and endocrine axis evolution. © The Author 2017. Published by Oxford University Press on behalf of the Society for Integrative and Comparative Biology. All rights reserved. For permissions please email: journals.permissions@oup.com.

Juvenile idiopathic arthritis.

PubMed

Boros, Christina; Whitehead, Ben

2010-09-01

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

Complete genome sequence of Pseudomonas rhizosphaerae IH5T (=DSM 16299T), a phosphate-solubilizing rhizobacterium for bacterial biofertilizer.

PubMed

Kwak, Yunyoung; Jung, Byung Kwon; Shin, Jae-Ho

2015-01-10

Pseudomonas rhizosphaerae IH5(T) (=DSM 16299(T)), isolated from the rhizospheric soil of grass growing in Spain, has been reported as a novel species of the genus Pseudomonas harboring insoluble phosphorus solubilizing activity. To understanding the multifunctional biofertilizer better, we report the complete genome sequence of P. rhizosphaerae IH5(T). Copyright © 2014 Elsevier B.V. All rights reserved.

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

PubMed

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

2016-01-01

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

42 CFR 136.416 - When should the IHS deny employment or dismiss an employee?

Code of Federal Regulations, 2013 CFR

2013-10-01

... violence; sexual assault, molestation, exploitation, contact, or prostitution; crimes against persons; or... Protection and Family Violence Prevention § 136.416 When should the IHS deny employment or dismiss an...

42 CFR 136.416 - When should the IHS deny employment or dismiss an employee?

Code of Federal Regulations, 2014 CFR

2014-10-01

... violence; sexual assault, molestation, exploitation, contact, or prostitution; crimes against persons; or... Protection and Family Violence Prevention § 136.416 When should the IHS deny employment or dismiss an...

42 CFR 136.416 - When should the IHS deny employment or dismiss an employee?

Code of Federal Regulations, 2010 CFR

2010-10-01

... violence; sexual assault, molestation, exploitation, contact, or prostitution; crimes against persons; or... Protection and Family Violence Prevention § 136.416 When should the IHS deny employment or dismiss an...

42 CFR 136.416 - When should the IHS deny employment or dismiss an employee?

Code of Federal Regulations, 2012 CFR

2012-10-01

... violence; sexual assault, molestation, exploitation, contact, or prostitution; crimes against persons; or... Protection and Family Violence Prevention § 136.416 When should the IHS deny employment or dismiss an...

42 CFR 136.416 - When should the IHS deny employment or dismiss an employee?

Code of Federal Regulations, 2011 CFR

2011-10-01

... violence; sexual assault, molestation, exploitation, contact, or prostitution; crimes against persons; or... Protection and Family Violence Prevention § 136.416 When should the IHS deny employment or dismiss an...

Identification and localization of gonadotropin-inhibitory hormone (GnIH) orthologs in the hypothalamus of the red-eared slider turtle, Trachemys scripta elegans.

PubMed

Ukena, Kazuyoshi; Iwakoshi-Ukena, Eiko; Osugi, Tomohiro; Tsutsui, Kazuyoshi

2016-02-01

Gonadotropin-inhibitory hormone (GnIH) was discovered in 2000 as a novel hypothalamic neuropeptide that inhibited gonadotropin release in the Japanese quail. GnIH and its orthologs have a common C-terminal LPXRFamide (X=L or Q) motif, and have been identified in vertebrates from agnathans to humans, apart from reptiles. In the present study, we characterized a cDNA encoding GnIH orthologs in the brain of the red-eared slider turtle. The deduced precursor protein consisted of 205 amino-acid residues, encoding three putative peptide sequences that included the LPXRFamide motif at their C-termini. In addition, the precursor sequence was most similar to those of avian species. Immunoaffinity purification combined with mass spectrometry confirmed that three mature peptides were produced in the brain. In situ hybridization and immunohistochemistry showed that turtle GnIH-containing cells were restricted to the periventricular hypothalamic nucleus. Immunoreactive fibers were densely distributed in the median eminence. Thus, GnIH and related peptides may act on the pituitary to regulate pituitary hormone release in turtles as well as other vertebrates. Copyright © 2015 Elsevier Inc. All rights reserved.

42 CFR 137.95 - May a Self-Governance Tribe purchase goods and services from the IHS on a reimbursable basis?

Code of Federal Regulations, 2010 CFR

2010-10-01

... 42 Public Health 1 2010-10-01 2010-10-01 false May a Self-Governance Tribe purchase goods and... SELF-GOVERNANCE Funding Acquisition of Goods and Services from the Ihs § 137.95 May a Self-Governance Tribe purchase goods and services from the IHS on a reimbursable basis? Yes, a Self-Governance Tribe may...

Idiopathic hypersomnia.

PubMed

Billiard, Michel; Sonka, Karel

2016-10-01

Idiopathic hypersomnia continues to evolve from the concept of "sleep drunkenness" introduced by Bedrich Roth in Prague in 1956 and the description of idiopathic hypersomnia with two forms, polysymptomatic and monosymptomatic, by the same Bedrich Roth in 1976. The diagnostic criteria of idiopathic hypersomnia have varied with the successive revisions of the International classifications of sleep disorders, including the recent 3rd edition. No epidemiological studies have been conducted so far. Disease onset occurs most often during adolescence or young adulthood. A familial background is often present but rigorous studies are still lacking. The key manifestation is hypersomnolence. It is often accompanied by sleep of long duration and debilitating sleep inertia. Polysomnography (PSG) followed by a multiple sleep latency test (MSLT) is mandatory, as well as a 24 h PSG or a 2-wk actigraphy in association with a sleep log to ensure a total 24-h sleep time longer than or equal to 66O minutes, when the mean sleep latency on the MSLT is longer than 8 min. Yet, MSLT is neither sensitive nor specific and the polysomnographic diagnostic criteria require continuous readjustment and biologic markers are still lacking. Idiopathic hypersomnia is most often a chronic condition though spontaneous remission may occur. The condition is disabling, sometimes even more so than narcolepsy type 1 or 2. Based on neurochemical, genetic and immunological analyses as well as on exploration of the homeostatic and circadian processes of sleep, various pathophysiological hypotheses have been proposed. Differential diagnosis involves a number of diseases and it is not yet clear whether idiopathic hypersomnia and narcolepsy type 2 are not the same condition. Until now, the treatment of idiopathic hypersomnia has mirrored that of the sleepiness of narcolepsy type 1 or 2. The first randomized, double-blind, placebo-controlled trials of modafinil have just been published, as well as a double

Characterization of an MPS I-H Knock-In Mouse that Carries a Nonsense Mutation Analogous to the Human IDUA-W402X Mutation

PubMed Central

Wang, Dan; Shukla, Charu; Liu, Xiaoli; Schoeb, Trenton R.; Clarke, Lorne A.; Bedwell, David M.; Keeling, Kim M.

2009-01-01

Here we report the characterization of a knock-in mouse model for the autosomal recessive disorder mucopolysaccharidosis type I-Hurler (MPS I-H), also known as Hurler syndrome. MPS I-H is the most severe form of α-L-iduronidase deficiency. α-L-iduronidase (encoded by the IDUA gene) is a lysosomal enzyme that participates in the degradation of dermatan sulfate and heparan sulfate. Using gene replacement methodology, a nucleotide change was introduced into the mouse Idua locus that resulted in a nonsense mutation at codon W392. The Idua-W392X mutation is analogous to the human IDUA-W402X mutation commonly found in MPS I-H patients. We found that the phenotype in homozygous Idua-W392X mice closely correlated with the human MPS I-H disease. Homozygous W392X mice showed no detectable α-L-iduronidase activity. We observed a defect in GAG degradation as evidenced by an increase in sulfated GAGs excreted in the urine and stored in multiple tissues. Histology and electron microscopy also revealed evidence of GAG storage in all tissues examined. Additional assessment revealed bone abnormalities and altered metabolism within the Idua-W392X mouse. This new mouse will provide an important tool to investigate therapeutic approaches for MPS I-H that cannot be addressed using current MPS I-H animal models. PMID:19751987

Early metformin therapy (age 8-12 years) in girls with precocious pubarche to reduce hirsutism, androgen excess, and oligomenorrhea in adolescence.

PubMed

Ibáñez, Lourdes; López-Bermejo, Abel; Díaz, Marta; Marcos, Maria Victoria; de Zegher, Francis

2011-08-01

Girls with a combined history of low(-normal) birth weight (LBW) and precocious pubarche (PP) are at high risk to develop polycystic ovary syndrome (PCOS). The objective of the study was to compare the capacity of early vs. late metformin treatment to prevent adolescent PCOS. This was a randomized, open-label study over 7 yr. The study was conducted at a university hospital. Thirty-eight LBW-PP girls were followed up from the mean age 8 until age 15 yr. Early metformin (study yr 1-4; age 8-12 yr) vs. late metformin (yr 6; age 13-14 yr). Measures included height; weight; hirsutism score; menstrual cycle; endocrine-metabolic screening (fasting; follicular phase); C-reactive protein; body composition (absorptiometry); abdominal fat partitioning (magnetic resonance imaging); ovarian morphology (ultrasound); PCOS (National Institutes of Health and Androgen Excess Society definitions) after yr 7 (all girls thus untreated for at least 1 yr). None of the girls dropped out of the study. At age 15 yr, early-metformin girls were taller (4 cm), were in a less proinflammatory state, and had less central fat due to reductions in visceral and hepatic fat. Hirsutism, androgen excess, oligomenorrhea, and PCOS were between 2- and 8-fold more prevalent in late- than early-treated girls. Abdominal adiposity was the first variable to diverge (at age 8-10 yr) between girls without vs. with PCOS at age 15 yr. In LBW-PP girls, early metformin therapy was found to prevent or delay the development of hirsutism, androgen excess, oligomenorrhea, and PCOS more effectively than late metformin. The time window of late childhood and early puberty may be more critical for the development, and thus for the prevention, of adolescent PCOS than the first years beyond menarche.

Revealing topographic lineaments through IHS enhancement of DEM data. [Digital Elevation Model

NASA Technical Reports Server (NTRS)

Murdock, Gary

1990-01-01

Intensity-hue-saturation (IHS) processing of slope (dip), aspect (dip direction), and elevation to reveal subtle topographic lineaments which may not be obvious in the unprocessed data are used to enhance digital elevation model (DEM) data from northwestern Nevada. This IHS method of lineament identification was applied to a mosiac of 12 square degrees using a Cray Y-MP8/864. Square arrays from 3 x 3 to 31 x 31 points were tested as well as several different slope enhancements. When relatively few points are used to fit the plane, lineaments of various lengths are observed and a mechanism for lineament classification is described. An area encompassing the gold deposits of the Carlin trend and including the Rain in the southeast to Midas in the northwest is investigated in greater detail. The orientation and density of lineaments may be determined on the gently sloping pediment surface as well as in the more steeply sloping ranges.

Evaluation of the Effect of Modafinil on Cognitive Functions in Patients with Idiopathic Hypersomnia with P300.

PubMed

Yaman, Mehmet; Karakaya, Fatıma; Aydin, Tuğçe; Mayda, Hasan; Güzel, Hail İbrahim; Kayaalp, Dilek

2015-06-27

Modafinil is a well-tolerated psychostimulant drug with low addictive potential that is used to treat patients with narcolepsy and other excessive sleepiness. Whereas favorable effects of modafinil on cognitive functions have been shown in a large number of studies, there are very few reports presenting the effects of modafinil electrophysiologically. The aim of this study was to investigate the effects of modafinil on auditory P300 latency and amplitude electrophysiologically. Eighteen patients (age range: 16-48 years) with a diagnosis of idiopathic hypersomnia (IH) were included in the present study. As a standard treatment, 200 mg/day modafinil was administered to each patient. The P300 auditory test was performed for each patient before and at the end of 1 week of modafinil treatment. After 1 week of modafinil treatment, mean P300 latencies (at all electrode sites) were significantly lower than the latencies before the treatment (P values for Fz, Cz and Pz recording sites were 0.039, 0.002, and 0.004, respectively). An increase in the P300 amplitudes was detected only at the Fz recording site, but not at Cz or Pz recording sites (P values for Fz, Cz, and Pz recording sites were 0.014, 0.100, and 0.05, respectively). One week of modafinil treatment improved the cognitive performance, alertness, and executive functions in IH patients. Our electrophysiologically obtained findings provide further confirmation for previous reports in which modafinil has been shown to exert favorable effects on cognitive performance, alertness, and executive functions.

Evaluation of the Effect of Modafinil on Cognitive Functions in Patients with Idiopathic Hypersomnia with P300

PubMed Central

Yaman, Mehmet; Karakaya, Fatıma; Aydin, Tuğçe; Mayda, Hasan; Güzel, Hail İbrahim; Kayaalp, Dilek

2015-01-01

Background Modafinil is a well-tolerated psychostimulant drug with low addictive potential that is used to treat patients with narcolepsy and other excessive sleepiness. Whereas favorable effects of modafinil on cognitive functions have been shown in a large number of studies, there are very few reports presenting the effects of modafinil electrophysiologically. The aim of this study was to investigate the effects of modafinil on auditory P300 latency and amplitude electrophysiologically. Material/Methods Eighteen patients (age range: 16–48 years) with a diagnosis of idiopathic hypersomnia (IH) were included in the present study. As a standard treatment, 200 mg/day modafinil was administered to each patient. The P300 auditory test was performed for each patient before and at the end of 1 week of modafinil treatment. Results After 1 week of modafinil treatment, mean P300 latencies (at all electrode sites) were significantly lower than the latencies before the treatment (P values for Fz, Cz and Pz recording sites were 0.039, 0.002, and 0.004, respectively). An increase in the P300 amplitudes was detected only at the Fz recording site, but not at Cz or Pz recording sites (P values for Fz, Cz, and Pz recording sites were 0.014, 0.100, and 0.05, respectively). Conclusions One week of modafinil treatment improved the cognitive performance, alertness, and executive functions in IH patients. Our electrophysiologically obtained findings provide further confirmation for previous reports in which modafinil has been shown to exert favorable effects on cognitive performance, alertness, and executive functions. PMID:26116438

Ice Ih anomalies: Thermal contraction, anomalous volume isotope effect, and pressure-induced amorphization

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

Salim, Michael A.; Willow, Soohaeng Yoo; Hirata, So, E-mail: sohirata@illinois.edu

Ice Ih displays several anomalous thermodynamic properties such as thermal contraction at low temperatures, an anomalous volume isotope effect (VIE) rendering the volume of D{sub 2}O ice greater than that of H{sub 2}O ice, and a pressure-induced transition to the high-density amorphous (HDA) phase. Furthermore, the anomalous VIE increases with temperature, despite its quantum-mechanical origin. Here, embedded-fragment ab initio second-order many-body perturbation (MP2) theory in the quasiharmonic approximation (QHA) is applied to the Gibbs energy of an infinite, proton-disordered crystal of ice Ih at wide ranges of temperatures and pressures. The quantum effect of nuclei moving in anharmonic potentials ismore » taken into account from first principles without any empirical or nonsystematic approximation to either the electronic or vibrational Hamiltonian. MP2 predicts quantitatively correctly the thermal contraction at low temperatures, which is confirmed to originate from the volume-contracting hydrogen-bond bending modes (acoustic phonons). It qualitatively reproduces (but underestimates) the thermal expansion at higher temperatures, caused by the volume-expanding hydrogen-bond stretching (and to a lesser extent librational) modes. The anomalous VIE is found to be the result of subtle cancellations among closely competing isotope effects on volume from all modes. Consequently, even ab initio MP2 with the aug-cc-pVDZ and aug-cc-pVTZ basis sets has difficulty reproducing this anomaly, yielding qualitatively varied predictions of the sign of the VIE depending on such computational details as the choice of the embedding field. However, the temperature growth of the anomalous VIE is reproduced robustly and is ascribed to the librational modes. These solid-state MP2 calculations, as well as MP2 Born–Oppenheimer molecular dynamics, find a volume collapse and a loss of symmetry and long-range order in ice Ih upon pressure loading of 2.35 GPa or higher. Concomitantly

Smoking, Alcohol, Drug Use, Abuse and Dependence in Narcolepsy and Idiopathic Hypersomnia: A Case-Control Study

PubMed Central

Barateau, Lucie; Jaussent, Isabelle; Lopez, Régis; Boutrel, Benjamin; Leu-Semenescu, Smaranda; Arnulf, Isabelle; Dauvilliers, Yves

2016-01-01

Study Objectives: Basic experiments support the impact of hypocretin on hyperarousal and motivated state required for increasing drug craving. Our aim was to assess the frequencies of smoking, alcohol and drug use, abuse and dependence in narcolepsy type 1 (NT1, hypocretin-deficient), narcolepsy type 2 (NT2), idiopathic hypersomnia (IH) (non-hypocretin-deficient conditions), in comparison to controls. We hypothesized that NT1 patients would be less vulnerable to drug abuse and addiction compared to other hypersomniac patients and controls from general population. Methods: We performed a cross-sectional study in French reference centres for rare hypersomnia diseases and included 450 adult patients (median age 35 years; 41.3% men) with NT1 (n = 243), NT2 (n = 116), IH (n = 91), and 710 adult controls. All participants were evaluated for alcohol consumption, smoking habits, and substance (alcohol and illicit drug) abuse and dependence diagnosis during the past year using the Mini International Neuropsychiatric Interview. Results: An increased proportion of both tobacco and heavy tobacco smokers was found in NT1 compared to controls and other hypersomniacs, despite adjustments for potential confounders. We reported an increased regular and frequent alcohol drinking habit in NT1 versus controls but not compared to other hypersomniacs in adjusted models. In contrast, heavy drinkers were significantly reduced in NT1 versus controls but not compared to other hypersomniacs. The proportion of patients with excessive drug use (codeine, cocaine, and cannabis), substance dependence, or abuse was low in all subgroups, without significant differences between either hypersomnia disorder categories or compared with controls. Conclusions: We first described a low frequency of illicit drug use, dependence, or abuse in patients with central hypersomnia, whether Hcrt-deficient or not, and whether drug-free or medicated, in the same range as in controls. Conversely, heavy drinkers were

Degree of facial and body terminal hair growth in unselected black and white women: toward a populational definition of hirsutism.

PubMed

DeUgarte, Catherine Marin; Woods, K S; Bartolucci, Alfred A; Azziz, Ricardo

2006-04-01

Hirsutism (i.e. facial and body terminal hair growth in a male-like pattern in women) is the principal clinical sign of hyperandrogenism, although its definition remains unclear. The purposes of the present study were to define 1) the degree of facial and body terminal hair, as assessed by the modified Ferriman-Gallwey (mFG) score, in unselected women from the general population; 2) the effect of race (Black and White) on the same; and 3) the normative cutoff values. We conducted a prospective observational study at a tertiary academic medical center. Participants included 633 unselected White (n = 283) and Black (n = 350) women presenting for a preemployment physical exam. Interventions included history and physical examination. Terminal body hair growth was assessed using the mFG scoring system; nine body areas were scored from 0-4 for terminal hair growth distribution. The mFG scores were not normally distributed; although cluster analysis failed to identify a natural cutoff value or clustering of the population, principal component and univariate analyses denoted two nearly distinct clusters that occurred above and below an mFG value of 2, with the bulk of the scores below. Overall, an mFG score of at least 3 was observed in 22.1% of all subjects (i.e. the upper quartile); of these subjects, 69.3% complained of being hirsute, compared with 15.8% of women with an mFG score below this value, and similar to the proportion of women with an mFG score of at least 8 who considered themselves to be hirsute (70.0%). Overall, there were no significant differences between Black and White women. Our data indicate that the prevalence and degree of facial and body terminal hair growth, as assessed by the mFG score, is similar in Black and White women and that an mFG of at least 3 signals the population of women whose hair growth falls out of the norm.

Comparative Study of Diode Laser Versus Neodymium-Yttrium Aluminum: Garnet Laser Versus Intense Pulsed Light for the Treatment of Hirsutism.

PubMed

Puri, Neerja

2015-01-01

Lasers are widely used for the treatment of hirsutism. But the choice of the right laser for the right skin type is very important. Before starting with laser therapy, it is important to assess the skin type, the fluence, the pulse duration and the type of laser to be used. To compare the efficacy and side effects of Diode laser, Neodymium-yttrium aluminum - garnet (Nd: YAG) laser and intense pulsed light (IPL) on 30 female patients of hirsutism. Thirty female patients with hirsutism were selected for a randomised controlled study. The patients were divided into three groups of 10 patients each. In group I patients diode laser was used, in group II patients long pulsed Nd: YAG laser was used and in group III, IPL was used. The patients were evaluated and result graded according to a 4-point scale as excellent, >75% reduction; good, 50-75% reduction; fair; 25-50% reduction; and poor, <25% reduction in hair density. It was seen that the percentage of hair reduction after two sessions of treatment was maximum (40%) in the diode laser group, followed by 35% hair reduction in the Nd: Yag laser group and 10% hair reduction in the IPL group. The percentage of hair reduction after four sessions of treatment was maximum (64%) in the diode laser group, followed by 62% hair reduction in the Nd: Yag laser group and 48% hair reduction in the IPL group. The percentage of hair reduction after eight sessions of treatment was maximum (92%) in the diode laser group, followed by 90% hair reduction in the Nd: YAG group and 70% hair reduction in the IPL group. To conclude for the Indian skin with dark hairs, the diode laser still stands the test of time. But, since the diode laser has a narrow margin of safety, proper pre and post-procedure cooling is recommended. Although, the side effects of Nd: YAG laser are less as compared to the diode laser, it is less efficacious as compared to the diode laser.

Idiopathic Inflammatory Myopathies

PubMed Central

Dimachkie, Mazen M.; Barohn, Richard J.

2012-01-01

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

Biodegradation of Cyclohexylamine by Brevibacterium oxydans IH-35A

PubMed Central

Iwaki, Hiroaki; Shimizu, Masatake; Tokuyama, Tai; Hasegawa, Yoshie

1999-01-01

A bacterial strain capable of growing on cyclohexylamine (CHAM) was isolated by using enrichment and isolation techniques. The strain isolated, strain IH-35A, was classified as a member of the genus Brevibacterium. The results of growth and enzyme studies are consistent with degradation of CHAM via cyclohexanone (CHnone), 6-hexanolactone, 6-hydroxyhexanoate, and adipate. Cell extracts obtained from this strain grown on CHAM contained CHAM oxidase, and the model for CHAM oxidation by this enzyme was similar to the model for deamino oxidation of amine by amine oxidase. PMID:10224025

a Novel Ihs-Ga Fusion Method Based on Enhancement Vegetated Area

NASA Astrophysics Data System (ADS)

Niazi, S.; Mokhtarzade, M.; Saeedzadeh, F.

2015-12-01

Pan sharpening methods aim to produce a more informative image containing the positive aspects of both source images. However, the pan sharpening process usually introduces some spectral and spatial distortions in the resulting fused image. The amount of these distortions varies highly depending on the pan sharpening technique as well as the type of data. Among the existing pan sharpening methods, the Intensity-Hue-Saturation (IHS) technique is the most widely used for its efficiency and high spatial resolution. When the IHS method is used for IKONOS or QuickBird imagery, there is a significant color distortion which is mainly due to the wavelengths range of the panchromatic image. Regarding the fact that in the green vegetated regions panchromatic gray values are much larger than the gray values of intensity image. A novel method is proposed which spatially adjusts the intensity image in vegetated areas. To do so the normalized difference vegetation index (NDVI) is used to identify vegetation areas where the green band is enhanced according to the red and NIR bands. In this way an intensity image is obtained in which the gray values are comparable to the panchromatic image. Beside the genetic optimization algorithm is used to find the optimum weight parameters in order to gain the best intensity image. Visual and statistical analysis proved the efficiency of the proposed method as it significantly improved the fusion quality in comparison to conventional IHS technique. The accuracy of the proposed pan sharpening technique was also evaluated in terms of different spatial and spectral metrics. In this study, 7 metrics (Correlation Coefficient, ERGAS, RASE, RMSE, SAM, SID and Spatial Coefficient) have been used in order to determine the quality of the pan-sharpened images. Experiments were conducted on two different data sets obtained by two different imaging sensors, IKONOS and QuickBird. The result of this showed that the evaluation metrics are more promising for

Semiquantitative assessment of hirsutism in 850 PCOS patients and 2,988 controls in China.

PubMed

Wong, Meifong; Zhao, Xiaomiao; Hong, Yu; Yang, Dongzi

2014-01-01

There is considerable individual and racial variation in the degree and pattern of body hair among PCOS patients. The purposes of this study were to define: 1) a suitable standard of hirsutism for Chinese women with PCOS and the general Chinese population; 2) the characteristics of hair distribution and degree in Chinese women with PCOS and the general population; and 3) the correlation of PCOS and FG score in Chinese women. This retrospective study in Chinese women with PCOS in the reproductive centre of Sun Yat-Sen Memorial Hospital, Sun Yat-Sen University, comprised 850 patients with PCOS and 2,988 members of the general population. It was conducted crosssectionally by interview, physical examination, ultrasound scan, and modified Ferriman-Gallwey score. Eight hundred and fifty Chinese women with PCOS (aged 20-41) underwent an interview, blood drawing, physical and ultrasound examination. Body hair at nine sites (lip, chin, arm, thigh, chest, upper belly, lower belly, upper back and lower back) were evaluated using the scoring system described by Ferriman and Gallwey; 2,988 healthy women (aged 20-45) underwent an interview, physical examination, ultrasound scan and FG score evaluation by trained gynaecologists. Terminal body hair growth was assessed using the mFG scoring system in Chinese women with PCOS and the control group; nine body areas were scored from 0-4 for terminal hair growth distribution. Our findings showed that of the 850 patients, 367 had a FG score equal to or greater than 5 points [43.2% (367/850)], 282 patients had a FG score equal to or greater than 6 points [33.18% (282/850)], and 21% had a score of at least 8 points. Nobody's FG score was equal to or greater than 24 points. Hirsutism was significantly higher in PCOS patients (score ≥ 5 = 43.2%) than in the general population (score ≥ 5 = 10%). The lip is the most common place (score 0-3) where terminal hair grows in 850 PCOS patients and the general population. Next came the upper back

Dielectric constant and low-frequency infrared spectra for liquid water and ice Ih within the E3B model

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

Shi, L.; Ni, Y.; Drews, S. E. P.

2014-08-28

Two intrinsic difficulties in modeling condensed-phase water with conventional rigid non-polarizable water models are: reproducing the static dielectric constants for liquid water and ice Ih, and generating the peak at about 200 cm{sup −1} in the low-frequency infrared spectrum for liquid water. The primary physical reason for these failures is believed to be the missing polarization effect in these models, and consequently various sophisticated polarizable water models have been developed. However, in this work we pursue a different strategy and propose a simple empirical scheme to include the polarization effect only on the dipole surface (without modifying a model's intermolecularmore » interaction potential). We implement this strategy for our explicit three-body (E3B) model. Our calculated static dielectric constants and low-frequency infrared spectra are in good agreement with experiment for both liquid water and ice Ih over wide temperature ranges, albeit with one fitting parameter for each phase. The success of our modeling also suggests that thermal fluctuations about local minima and the energy differences between different proton-disordered configurations play minor roles in the static dielectric constant of ice Ih. Our analysis shows that the polarization effect is important in resolving the two difficulties mentioned above and sheds some light on the origin of several features in the low-frequency infrared spectra for liquid water and ice Ih.« less

Dielectric constant and low-frequency infrared spectra for liquid water and ice Ih within the E3B model.

PubMed

Shi, L; Ni, Y; Drews, S E P; Skinner, J L

2014-08-28

Two intrinsic difficulties in modeling condensed-phase water with conventional rigid non-polarizable water models are: reproducing the static dielectric constants for liquid water and ice Ih, and generating the peak at about 200 cm(-1) in the low-frequency infrared spectrum for liquid water. The primary physical reason for these failures is believed to be the missing polarization effect in these models, and consequently various sophisticated polarizable water models have been developed. However, in this work we pursue a different strategy and propose a simple empirical scheme to include the polarization effect only on the dipole surface (without modifying a model's intermolecular interaction potential). We implement this strategy for our explicit three-body (E3B) model. Our calculated static dielectric constants and low-frequency infrared spectra are in good agreement with experiment for both liquid water and ice Ih over wide temperature ranges, albeit with one fitting parameter for each phase. The success of our modeling also suggests that thermal fluctuations about local minima and the energy differences between different proton-disordered configurations play minor roles in the static dielectric constant of ice Ih. Our analysis shows that the polarization effect is important in resolving the two difficulties mentioned above and sheds some light on the origin of several features in the low-frequency infrared spectra for liquid water and ice Ih.

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

PubMed

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

2017-06-01

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

Hirsutism, virilism, polycystic ovarian disease, and the steroid-gonadotropin-feedback system: a career retrospective.

PubMed

Mahesh, Virendra B

2012-01-01

This career retrospective describes how the initial work on the mechanism of hormone action provided the tools for the study of hirsutism, virilism, and polycystic ovarian disease. After excessive ovarian and or adrenal androgen secretion in polycystic ovarian disease had been established, the question whether the disease was genetic or acquired, methods to manage hirsutism and methods for the induction of ovulation were addressed. Recognizing that steroid gonadotropin feedback was an important regulatory factor, initial studies were done on the secretion of LH and FSH in the ovulatory cycle. This was followed by the study of basic mechanisms of steroid-gonadotropin feedback system, using castration and steroid replacement and the events surrounding the natural onset of puberty. Studies in ovariectomized rats showed that progesterone was a pivotal enhancer of estrogen-induced gonadotropin release, thus accounting for the preovulatory gonadotropin surge. The effects of progesterone were manifested by depletion of the occupied estrogen receptors of the anterior pituitary, release of hypothalamic LHRH, and inhibition of enzymes that degrade LHRH. Progesterone also promoted the synthesis of FSH in the pituitary. The 3α,5α-reduced metabolite of progesterone brought about selective LH release and acted using the GABA(A) receptor system. The 5α-reduced metabolite of progesterone brought about selective FSH release; the ability of progesterone to bring about FSH release was dependent on its 5α-reduction. The GnRH neuron does not have steroid receptors; the steroid effect was shown to be mediated through the excitatory amino acid glutamate, which in turn stimulated nitric oxide. These observations led to the replacement of the long-accepted belief that ovarian steroids acted directly on the GnRH neuron by the novel concept that the steroid feedback effect was exerted at the glutamatergic neuron, which in turn regulated the GnRH neuron. The neuroprotective effects of

Comparative Study of Diode Laser Versus Neodymium-Yttrium Aluminum: Garnet Laser Versus Intense Pulsed Light for the Treatment of Hirsutism

PubMed Central

Puri, Neerja

2015-01-01

Introduction: Lasers are widely used for the treatment of hirsutism. But the choice of the right laser for the right skin type is very important. Before starting with laser therapy, it is important to assess the skin type, the fluence, the pulse duration and the type of laser to be used. Aims: To compare the efficacy and side effects of Diode laser, Neodymium-yttrium aluminum — garnet (Nd: YAG) laser and intense pulsed light (IPL) on 30 female patients of hirsutism. Materials and Methods: Thirty female patients with hirsutism were selected for a randomised controlled study. The patients were divided into three groups of 10 patients each. In group I patients diode laser was used, in group II patients long pulsed Nd: YAG laser was used and in group III, IPL was used. The patients were evaluated and result graded according to a 4-point scale as excellent, >75% reduction; good, 50-75% reduction; fair; 25-50% reduction; and poor, <25% reduction in hair density. Results: It was seen that the percentage of hair reduction after two sessions of treatment was maximum (40%) in the diode laser group, followed by 35% hair reduction in the Nd: Yag laser group and 10% hair reduction in the IPL group. The percentage of hair reduction after four sessions of treatment was maximum (64%) in the diode laser group, followed by 62% hair reduction in the Nd: Yag laser group and 48% hair reduction in the IPL group. The percentage of hair reduction after eight sessions of treatment was maximum (92%) in the diode laser group, followed by 90% hair reduction in the Nd: YAG group and 70% hair reduction in the IPL group. Conclusions: To conclude for the Indian skin with dark hairs, the diode laser still stands the test of time. But, since the diode laser has a narrow margin of safety, proper pre and post-procedure cooling is recommended. Although, the side effects of Nd: YAG laser are less as compared to the diode laser, it is less efficacious as compared to the diode laser. PMID:26157309

Interpretation of magnetization transfer from inhomogeneously broadened lines (ihMT) in tissues as a dipolar order effect within motion restricted molecules

NASA Astrophysics Data System (ADS)

Varma, G.; Girard, O. M.; Prevost, V. H.; Grant, A. K.; Duhamel, G.; Alsop, D. C.

2015-11-01

Comparison of off-resonance saturation with single and dual frequency irradiation indicates a contribution of inhomogeneously broadened lines to magnetization transfer in tissues. This inhomogeneous magnetization transfer (ihMT) phenomenon can be exploited to produce images that highlight tissues containing myelin, in vivo. Here, a model for ihMT is described that includes dipolar order effects from magnetization associated with motion-restricted macromolecules. In this model, equal irradiation at positive and negative frequency offsets eliminates dipolar order and achieves greater saturation than irradiation at a single offset frequency using the same power. Fitting of mouse and human volunteer brain data at different irradiation powers and offset frequencies was performed to assess the relevance of the model and approximate tissue parameters. A key parameter in determining ihMT signal was found to be the relaxation time T1D associated with the dipolar order reservoir and the fraction f of the semi-solid, bound magnetization that possessed a nonzero T1D. Indeed, better fits of myelinated tissue were achieved when assuming f ≠ 1. From such fits, estimated T1Ds of mice in the white matter, (34 ± 14)ms, were much longer than in muscle, T1D = (1 ± 1)ms and the average f from white matter volunteer data was 2.2 times greater than that in grey matter. The combination of f and longer T1Ds was primarily responsible for the much higher ihMT in myelinated tissues, and provided explanation for the species variation. This dipolar order ihMT model should help guide future research, pulse sequence optimization, and clinical applications.

On the nature of the solvated electron in ice Ih.

PubMed

de Koning, Maurice; Fazzio, Adalberto; da Silva, Antônio José Roque; Antonelli, Alex

2016-02-14

The water-solvated excess electron (EE) is a key chemical agent whose hallmark signature, its asymmetric optical absorption spectrum, continues to be a topic of debate. While nearly all investigation has focused on the liquid-water solvent, the fact that the crystalline-water solvated EE shows a very similar visible absorption pattern has remained largely unexplored. Here, we present spin-polarized density-functional theory calculations subject to periodic boundary conditions of the interplay between an EE and a number of intrinsic lattice defects in ice Ih. Our results show that the optical absorption signatures in the presence of three unsaturated hydrogen bonds (HB) are very similar to those observed experimentally. Its low-energy side can be attributed to transitions between the EE ground state and a single localized excited level, in a picture that is different from that for the liquid solvent, where this portion has been associated with hydrogen-like s → p excitations. The blue tail, on the other hand, relates to transitions between the EE ground state and delocalized excited states, which is in line with the bound-to-continuum transition interpretations for the EE in liquid water. Finally, we find that, depending on the number of dangling HBs participating in the EE trap, its charge density may spontaneously break the spin degeneracy through exchange interactions with the surrounding electrons, displaying the many-electron quantum nature of the EE problem in ice Ih.

42 CFR 136.412 - What questions must the IHS ask as part of the background investigation?

Code of Federal Regulations, 2011 CFR

2011-10-01

... offense, under Federal, State, or Tribal law involving crimes of violence; sexual assault, molestation... Child Protection and Family Violence Prevention § 136.412 What questions must the IHS ask as part of the...

42 CFR 136.412 - What questions must the IHS ask as part of the background investigation?

Code of Federal Regulations, 2014 CFR

2014-10-01

... offense, under Federal, State, or Tribal law involving crimes of violence; sexual assault, molestation... Child Protection and Family Violence Prevention § 136.412 What questions must the IHS ask as part of the...

42 CFR 136.412 - What questions must the IHS ask as part of the background investigation?

Code of Federal Regulations, 2012 CFR

2012-10-01

... offense, under Federal, State, or Tribal law involving crimes of violence; sexual assault, molestation... Child Protection and Family Violence Prevention § 136.412 What questions must the IHS ask as part of the...

42 CFR 136.412 - What questions must the IHS ask as part of the background investigation?

Code of Federal Regulations, 2010 CFR

2010-10-01

... offense, under Federal, State, or Tribal law involving crimes of violence; sexual assault, molestation... Child Protection and Family Violence Prevention § 136.412 What questions must the IHS ask as part of the...

42 CFR 136.412 - What questions must the IHS ask as part of the background investigation?

Code of Federal Regulations, 2013 CFR

2013-10-01

... offense, under Federal, State, or Tribal law involving crimes of violence; sexual assault, molestation... Child Protection and Family Violence Prevention § 136.412 What questions must the IHS ask as part of the...

Microstructural analysis of the thermal annealing of ice-Ih using EBSD

NASA Astrophysics Data System (ADS)

Hidas, Károly; Tommasi, Andréa; Mainprice, David; Chauve, Thomas; Barou, Fabrice; Montagnat, Maurine

2017-04-01

Rocks deformed in the middle crust and deeper in the Earth typically remain at high temperature for extended time spans after the cessation of deformation. This results in annealing of the deformation microstructure by a series of thermally activated, diffusion-based processes, namely: recovery and static recrystallization, which may also modify the crystal preferred orientation (CPO) or texture. Understanding the effects of annealing on the microstructure and CPO is therefore of utmost importance for the interpretation of the microstructures and for the estimation of the anisotropy of physical properties of lower crustal and mantle rocks. Ice-Ih -the typical form of water ice on the Earth's surface, with hexagonal crystal symmetry- deforms essentially by glide of dislocations on the basal plane [1], thus it has high viscoplastic anisotropy, which induces strong heterogeneity of stresses and strains at both the intra- and intergranular scales [2-3]. This behavior makes ice-Ih an excellent analog material for silicate minerals that compose the Earth. In situ observations of the evolution of the microstructures and CPO during annealing enable the study of the interplay between the various physical processes involved in annealing (recovery, nucleation, grain growth). They also allow the analysis of the impact of the preexisting deformation microstructures on the microstructural and CPO evolution during annealing. Here we studied the evolution of the microstructure of ice-Ih during static recrystallization by stepwise annealing experiments. We alternated thermal annealing and electron backscatter diffraction (EBSD) analyses on polycrystalline columnar ice-Ih pre-deformed in uniaxial compression at temperature of -7 °C to strains of 3.0-5.2. Annealing experiments were carried out at -5 °C and -2 °C up to a maximum of 3.25 days, typically in 5-6 steps. EBSD crystal orientation maps obtained after each annealing step permit the description of microstructural changes

Health-related quality of life among drug-naïve patients with narcolepsy with cataplexy, narcolepsy without cataplexy, and idiopathic hypersomnia without long sleep time.

PubMed

Ozaki, Akiko; Inoue, Yuichi; Nakajima, Toru; Hayashida, Kenichi; Honda, Makoto; Komada, Yoko; Takahashi, Kiyohisa

2008-12-15

To evaluate the health-related quality life (HRQOL) of drug-naïve patients with narcolepsy with cataplexy (NAwith CA), narcolepsy without cataplexy (NA without CA) and idiopathic hypersomnia without long sleep time (IHS without LST), and to explore the factors influencing the HRQOL. Factors associated with the occurrence of automobile accidents are also discussed. A total of 137 consecutive drug naïve patients who met the criteria of the 2nd edition of the International Classification of Sleep Disorders (NA with CA, n = 28; NA without CA, n = 27; IHS without LST, n = 82) were enrolled. The patients were asked to fill out questionnaires, including the SF-36, Epworth Sleepiness Scale (ESS), sociodemographic variables, and items regarding driving habits and the experiences related to automobile accidents. All 3 diagnostic groups had significantly lower scores in most SF-36 domains compared with Japanese normative data. Significant differences among the 3 diagnostic groups were not observed. Specific factors in SF-36 domains were not found with multiple linear regression analyses, while disease duration was positively correlated with mental health among all subjects. Among the patients reporting driving habits, ESS score (> or =16) was positively associated with the experience of automobile accidents. Our results indicated that HRQOL decreases in drug-naïve patients with hypersomnia, but neither disease category nor severity of the disorder appears as an associated factor. Increased severity of hypersomnia, however, was thought to play an important role in the occurrence of automobile accidents.

Natural history of idiopathic diabetes insipidus.

PubMed

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

2011-10-01

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

An acute hemolytic transfusion reaction due to anti-IH in a patient with sickle cell disease.

PubMed

Campbell, S A; Shirey, R S; King, K E; Ness, P M

2000-07-01

A hemolytic transfusion reaction (HTR) due to anti-IH is reported in a patient with sickle cell disease (SCD). An 18-year-old woman with SCD and a complete phenotype on file had been identified as group B-positive with negative antibody-screening tests and had received 1 unit of packed RBCs. Ten days later, she was readmitted in painful crisis with a Hb of 4.2 g per dL. Antibody-screening tests and panel cells were positive at all test phases with a negative autocontrol, which suggested alloantibodies. Phenotypically matched group O RBCs were issued emergently. After the transfusion of 100 mL, the patient had an HTR with chills, fever, and tachycardia and laboratory findings of hemoglobinemia, hemoglobinuria, and negative DATs. A high-titer, IgM anti-IH with a high thermal amplitude (reactive with group O, but not group B RBCs at 37 degrees C) was identified. Autologous RBCs appeared to have normal I antigen expression, but less H antigen than pooled group B RBCs. She was given group B RBCs, uneventfully, by use of a blood warmer. This is a rare case of anti-IH as the cause of a HTR, as a serologic problem that may be seen in SCD, and as an autoantibody that may mimic an alloantibody. Ironically, this HTR resulted from the effort to provide phenotypically matched RBCs, which necessitated the selection of group O RBCs.

Vitamin D deficiency in chronic idiopathic urticaria.

PubMed

Movahedi, Masoud; Tavakol, Marzieh; Hirbod-Mobarakeh, Armin; Gharagozlou, Mohammad; Aghamohammadi, Asghar; Tavakol, Zahra; Momenzadeh, Kaveh; Nabavi, Mohammad; Dabbaghzade, Abbas; Mosallanejad, Asieh; Rezaei, Nima

2015-04-01

Chronic urticaria is the most common skin diseases, characterized by chronic cutaneous lesions which severely debilitates patients in several aspects of their everyday life. Vitamin D is known to exert several actions in the immune system and to influence function and differentiation of mast cells, central role players in the pathogenesis of chronic idiopathic urticaria. This study was performed to evaluate the relationship between vitamin D levels and susceptibility to chronic idiopathic urticaria. One hundred and fourteen patients with chronic idiopathic urticaria were recruited in this study along with one hundred and eighty seven sex-matched and age-matched healthy volunteers as the control group. For each patient, urticaria activity score was calculated and autologous serum skin test was done. Vitamin D metabolic statue was measured in serum as 25 hydroxyvitamin D using enzyme immunoassay method. Patients with chronic idiopathic urticaria significantly showed lower levels of vitamin D. Vitamin D deficiency was significantly associated with increased susceptibility to chronic idiopathic urticaria. There was a significant positive correlation between vitamin D levels and urticaria activity score. This study showed that patients with chronic idiopathic urticaria had reduced levels of vitamin D, while vitamin D deficiency could increase susceptibility to chronic idiopathic urticaria.

Delicious Low GL space foods by using Low GI materials -IH and Vacuum cooking -

NASA Astrophysics Data System (ADS)

Katayama, Naomi; Nagasaka, Sanako; Murasaki, Masahiro; Space Agriculture Task Force, J.

Enough life-support systems are necessary to stay in space for a long term. The management of the meal for astronauts is in particular very important. When an astronaut gets sick in outer space, it means death. To astronauts, the delicious good balance space foods are essential for their work. This study was aimed at making balance space foods menu for the healthy space-life. The kitchen utensil has a limit in the space environment. And a method to warm is only heater without fire. Therefore purpose of this study, we make the space foods which make by using vacuum cooking device and the IH heater We made space foods menu to referred to Japanese nutrition standard in 2010. We made space foods menu which are using "brown rice, wheat, soy bean, sweet potato and green-vegetable" and " loach and insects which are silkworm pupa, snail, mud snail, turmait, fly, grasshopper, bee". We use ten health adults as subjects. Ten subjects performed the sensory test of the questionnaire method. There was the sensuality examination in the item of "taste, a fragrance, color, the quantity" and acquired a mark at ten points of perfect scores.. We could make the space foods which we devised with vacuum cooking and IH deliciously. As a result of sensuality examination, the eight points in ten points of perfect scores was appeared. This result showed, our space food menu is delicious. We can store these space foods with a refrigerator for 20 days by making vacuum cooking. This thing is at all important result so that a save is enabled when surplus food was done in future by performing vacuum cooking. We want to make delicious space foods menu with vacuum cooking and IH heater more in future.

American Indian Veterans' Views about Their Choices in Health Care: VA, IHS, and Medicare

ERIC Educational Resources Information Center

Reifel, Nancy; Bayhylle, Ruth; Harada, Nancy; Villa, Valentine

2009-01-01

Legislation during the past three decades has gradually drawn Indian Health Service (IHS)-funded clinics into the mainstream of the US medical care environment. The Indian Self-Determination and Education Reform Act of 1973 and its Indian Education Amendments of 1984 began a movement away from federal management of health services to local tribal…

Idiopathic ventricular tachycardia and fibrillation.

PubMed

Belhassen, B; Viskin, S

1993-06-01

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

42 CFR 137.294 - What is the typical IHS environmental review process for construction projects?

Code of Federal Regulations, 2013 CFR

2013-10-01

... 42 Public Health 1 2013-10-01 2013-10-01 false What is the typical IHS environmental review process for construction projects? 137.294 Section 137.294 Public Health PUBLIC HEALTH SERVICE, DEPARTMENT OF HEALTH AND HUMAN SERVICES INDIAN HEALTH SERVICE, DEPARTMENT OF HEALTH AND HUMAN SERVICES TRIBAL...

42 CFR 137.294 - What is the typical IHS environmental review process for construction projects?

Code of Federal Regulations, 2014 CFR

2014-10-01

... 42 Public Health 1 2014-10-01 2014-10-01 false What is the typical IHS environmental review process for construction projects? 137.294 Section 137.294 Public Health PUBLIC HEALTH SERVICE, DEPARTMENT OF HEALTH AND HUMAN SERVICES INDIAN HEALTH SERVICE, DEPARTMENT OF HEALTH AND HUMAN SERVICES TRIBAL...

42 CFR 137.294 - What is the typical IHS environmental review process for construction projects?

Code of Federal Regulations, 2012 CFR

2012-10-01

... 42 Public Health 1 2012-10-01 2012-10-01 false What is the typical IHS environmental review process for construction projects? 137.294 Section 137.294 Public Health PUBLIC HEALTH SERVICE, DEPARTMENT OF HEALTH AND HUMAN SERVICES INDIAN HEALTH SERVICE, DEPARTMENT OF HEALTH AND HUMAN SERVICES TRIBAL...

Relation between adolescent idiopathic scoliosis and morphologic somatotypes.

PubMed

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

1997-11-01

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

Perspective: Update on Idiopathic Intracranial Hypertension

PubMed Central

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

2011-01-01

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

Extensive clinical experience: relative prevalence of different androgen excess disorders in 950 women referred because of clinical hyperandrogenism.

PubMed

Carmina, E; Rosato, F; Jannì, A; Rizzo, M; Longo, R A

2006-01-01

We undertook this study to estimate the prevalence of the various androgen excess disorders using the new criteria suggested for the diagnosis of polycystic ovary syndrome (PCOS). The study was performed at two endocrine departments at the University of Palermo (Palermo, Italy). The records of all patients referred between 1980 and 2004 for evaluation of clinical hyperandrogenism were reevaluated. All past diagnoses were reviewed using the actual diagnostic criteria. To be included in this study, the records of the patients had to present the following available data: clinical evaluation of hyperandrogenism, body weight and height, testosterone (T), free T, dehydroepiandrosterone sulfate, 17-hydroxyprogesterone, progesterone, and pelvic sonography. A total of 1226 consecutive patients were seen during the study period, but only the scores of 950 patients satisfied all criteria and were reassessed for the diagnosis. The prevalence of androgen excess disorders was: PCOS, 72.1% (classic anovulatory patients, 56.6%; mild ovulatory patients, 15.5%), idiopathic hyperandrogenism, 15.8%; idiopathic hirsutism, 7.6%; 21-hydroxylase-deficient nonclassic adrenal hyperplasia, 4.3%; and androgen-secreting tumors, 0.2%. Compared with other androgen excess disorders, patients with PCOS had increased body weight whereas nonclassic adrenal hyperplasia patients were younger and more hirsute and had higher serum levels of T, free T, and 17-hydroxyprogesterone. Classic PCOS is the most common androgen excess disorder. However, mild androgen excess disorders (ovulatory PCOS and idiopathic hyperandrogenism) are also common and, in an endocrine setting, include about 30% of patients with clinical hyperandrogenism.

Molecular identification of StAR and 3βHSD1 and characterization in response to GnIH stimulation in protogynous hermaphroditic grouper (Epinephelus coioides).

PubMed

Wang, Qingqing; Qi, Xin; Tang, Haipei; Guo, Yin; Li, Shuisheng; Li, Gaofei; Yang, Xiaoli; Zhang, Haifa; Liu, Xiaochun; Lin, Haoran

2017-04-01

Gonadal steroids are critical factors in reproduction and sex reverse process. StAR (steroidogenic acute regulatory protein), transferring the cholesterol from the outer mitochondrial membrane to the inner membrane, is the rate-limiting factor of steroidogenesis. 3βHSD (3β-hydroxysteroid dehydrogenase/Δ5-Δ4 isomerase), converting Δ5-steroids into Δ4-steroids, is an important oxidoreductase in steroidogenesis. In the present study, StAR and 3βHSD1 were cloned and characterized from protogynous orange-spotted grouper. StAR cDNA contains an 861bp open reading frame (ORF), encoding a predicted protein of 286 amino acids, and the ORF of 3βHSD1 was 1125bp, encoding a predicted protein of 374 amino acids. The transcript of StAR was mainly expressed in gonad, while 3βHSD1 mRNA was predominantly detected in brain and gonad. In the previous study, we found the expression of GnIH mRNA level in male, as well as in 17 alpha-methyltestosterone (MT)-induced male fish was significantly higher than in female fish, this indicating that GnIH/GnIHR signaling might be involved in the regulation of sex reversal and male maintenance. In order to figure out the function of GnIH in steroidogenesis, the expression of StAR and 3βHSD1 regulated by GnIH was examined. In vitro study showed that treatment of cultured ovary fragments with gGnIH peptides significantly stimulated the expression of StAR and 3βHSD1. In addition, the mRNA levels of StAR and 3βHSD1 were significantly increased after intraperitoneal injection (i.p.) with gGnIH peptides. Moreover, during MT-induced sex change from female to male, the levels of StAR mRNA significantly increased by 5.2, 24.8 and 353.5 folds, and that of 3βHSD1 mRNA by 3.5, 32.5 and 55.4 folds at the 2nd, 4th and 6th week after MT implantation, respectively. Collectively, our results indicate that GnIH may be involved in the regulation of sex reversal or male maintenance by stimulating the expression of StAR and 3βHSD1 in protogynous grouper

42 CFR 137.304 - May Self-Governance Tribes buy back environmental services from the IHS?

Code of Federal Regulations, 2011 CFR

2011-10-01

... 42 Public Health 1 2011-10-01 2011-10-01 false May Self-Governance Tribes buy back environmental...-GOVERNANCE Construction Nepa Process § 137.304 May Self-Governance Tribes buy back environmental services from the IHS? Yes, Self-Governance Tribes may “buy back” project related services in their construction...

Different sleep onset criteria at the multiple sleep latency test (MSLT): an additional marker to differentiate central nervous system (CNS) hypersomnias.

PubMed

Pizza, Fabio; Vandi, Stefano; Detto, Stefania; Poli, Francesca; Franceschini, Christian; Montagna, Pasquale; Plazzi, Giuseppe

2011-03-01

Excessive daytime sleepiness (EDS) has different correlates in non-rapid eye movement (NREM) [idiopathic hypersomnia (IH) without long sleep time] and REM sleep [narcolepsy without cataplexy (NwoC) and narcolepsy with cataplexy (NC)]-related hypersomnias of central origin. We analysed sleep onset characteristics at the multiple sleep latency test (MSLT) applying simultaneously two sleep onset criteria in 44 NC, seven NwoC and 16 IH consecutive patients referred for subjective EDS complaint. Sleep latency (SL) at MSLT was assessed both as the time elapsed to the occurrence of a single epoch of sleep Stage 1 NREM (SL) and of unequivocal sleep [three sleep Stage 1 NREM epochs or any other sleep stage epoch, sustained SL (SusSL)]. Idiopathic hypersomnia patients showed significantly (P<0.0001) longer SusSL than SL (7.7±2.5 versus 5.6±1.3 min, respectively) compared to NwoC (5.8±2.5 versus 5.3±2.2 min) and NC patients (4.1±3 versus 3.9±3 min). A mean difference threshold between SusSL and SL ≥27 s reached a diagnostic value to discriminate IH versus NC and NwoC sufferers (sensitivity 88%; specificity 82%). Moreover, NC patients showed better subjective sleepiness perception than NwoC and IH cases in the comparison between naps with or without sleep occurrence. Simultaneous application of the two widely used sleep onset criteria differentiates IH further from NC and NwoC patients: IH fluctuate through a wake-Stage 1 NREM sleep state before the onset of sustained sleep, while NC and NwoC shift abruptly into a sustained sleep. The combination of SusSL and SL determination at MSLT should be tested as an additional objective differential criterion for EDS disorders. © 2010 European Sleep Research Society.

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

PubMed

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

2018-05-01

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

Idiopathic Hypersomnia: A Study of 77 Cases

PubMed Central

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

2007-01-01

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

77 FR 67657 - Request for Public Comment: 30-Day Proposed Information Collection: Indian Health Service (IHS...

Federal Register 2010, 2011, 2012, 2013, 2014

2012-11-13

... Practice, and Local Effort (BPPPLE) Form.'' Need and Use of Information Collection: The IHS goal is to.../Disease Prevention, Nursing, and Dental) have developed a centralized program database of best practices, promising Practices and local efforts and resources. This database was previously referred as OSCAR, but the...

Aetiology of idiopathic granulomatous mastitis.

PubMed

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.

Aetiology of idiopathic granulomatous mastitis

PubMed Central

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

2014-01-01

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. PMID:25516860

Intermittent Hypoxia Increases the Severity of Bleomycin-Induced Lung Injury in Mice.

PubMed

Gille, Thomas; Didier, Morgane; Rotenberg, Cécile; Delbrel, Eva; Marchant, Dominique; Sutton, Angela; Dard, Nicolas; Haine, Liasmine; Voituron, Nicolas; Bernaudin, Jean-François; Valeyre, Dominique; Nunes, Hilario; Besnard, Valérie; Boncoeur, Emilie; Planès, Carole

2018-01-01

Severe obstructive sleep apnea (OSA) with chronic intermittent hypoxia (IH) is common in idiopathic pulmonary fibrosis (IPF). Here, we evaluated the impact of IH on bleomycin- (BLM-) induced pulmonary fibrosis in mice. C57BL/6J mice received intratracheal BLM or saline and were exposed to IH (40 cycles/hour; FiO 2 nadir: 6%; 8 hours/day) or intermittent air (IA). In the four experimental groups, we evaluated (i) survival; (ii) alveolar inflammation, pulmonary edema, lung oxidative stress, and antioxidant enzymes; (iii) lung cell apoptosis; and (iv) pulmonary fibrosis. Survival at day 21 was lower in the BLM-IH group ( p < 0.05). Pulmonary fibrosis was more severe at day 21 in BLM-IH mice, as assessed by lung collagen content ( p = 0.02) and histology. At day 4, BLM-IH mice developed a more severe neutrophilic alveolitis, ( p < 0.001). Lung oxidative stress was observed, and superoxide dismutase and glutathione peroxidase expression was decreased in BLM-IH mice ( p < 0.05 versus BLM-IA group). At day 8, pulmonary edema was observed and lung cell apoptosis was increased in the BLM-IH group. These results show that exposure to chronic IH increases mortality, lung inflammation, and lung fibrosis in BLM-treated mice. This study raises the question of the worsening impact of severe OSA in IPF patients.

77 FR 52748 - 60-Day Proposed Information Collection: Indian Health Service (IHS) Sharing What Works-Best...

Federal Register 2010, 2011, 2012, 2013, 2014

2012-08-30

... Form (OMB Form No. 0917-0034). Need and Use of Information Collection: The IHS goal is to raise the... Prevention (HP/DP), Nursing, and Dental) have developed a centralized program database of Best/Promising Practices and Local Efforts (BPPPLE) and resources. The purpose of this collection is to develop a database...

Acute Idiopathic Scrotal Edema: Systematic Literature Review.

PubMed

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

2018-06-01

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

Friction at ice-Ih / water interfaces

NASA Astrophysics Data System (ADS)

Louden, Patrick B.; Gezelter, J. Daniel

We present evidence that the prismatic and secondary prism facets of ice-Ih crystals possess structural features that alter the effective hydrophilicity of the ice / water interface. This is shown through molecular dynamics simulations of solid-liquid friction, where the prismatic { 10 1 0 } , secondary prism { 11 2 0 } , basal { 0001 } , and pyramidal { 20 2 1 } facets are drawn through liquid water. We find that the two prismatic facets exhibit differential solid-liquid friction coefficients when compared with the basal and pyramidal facets. These results are complemented by a model solid/liquid interface with tunable hydrophilicity. These simulations provide evidence that the two prismatic faces have a significantly smaller effective surface area in contact with the liquid water. The ice / water interfacial widths for all four crystal facets are similar (using both structural and dynamic measures), and were found to be independent of the shear rate. Additionally, decomposition of orientational time correlation functions show position-dependence for the short- and longer-time decay components close to the interface. Support for this project was provided by the National Science Foundation under Grant CHE-1362211. Computational time was provided by the Center for Research Computing (CRC) at the University of Notre Dame.

Idiopathic hypersomnia: a study of 77 cases.

PubMed

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

2007-10-01

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

[Idiopathic facial paralysis in children].

PubMed

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

2015-05-01

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

Nuclear quantum fluctuations in ice I(h).

PubMed

Moreira, Pedro Augusto Franco Pinheiro; de Koning, Maurice

2015-10-14

We discuss the role of nuclear quantum fluctuations in ice Ih, focusing on the hydrogen-bond (HB) structure and the molecular dipole-moment distribution. For this purpose we carry out DFT-based first-principles molecular dynamics and path-integral molecular dynamics simulations at T = 100 K. We analyze the HB structure in terms of a set of parameters previously employed to characterize molecular structures in the liquid phase and compute the molecular dipole moments using the maximally-localized Wannier functions. The results show that the protons experience very large digressions driven by quantum fluctuations, accompanied by major rearrangements in the electronic density. As a result of these protonic quantum fluctuations the molecular dipole-moment distribution is substantially broadened as well as shifted to a larger mean value when compared to the results obtained when such fluctuations are neglected. In terms of dielectric constants, the reconciliation between the greater mean dipole moment and experimental indications that the dielectric constant of H2O ice is lower than that of D2O ice would indicate that the topology of the HB network is sensitive to protonic quantum fluctuations.

42 CFR 137.401 - What role does Tribal consultation play in the IHS annual budget request process?

Code of Federal Regulations, 2010 CFR

2010-10-01

... 42 Public Health 1 2010-10-01 2010-10-01 false What role does Tribal consultation play in the IHS annual budget request process? 137.401 Section 137.401 Public Health PUBLIC HEALTH SERVICE, DEPARTMENT OF HEALTH AND HUMAN SERVICES INDIAN HEALTH SERVICE, DEPARTMENT OF HEALTH AND HUMAN SERVICES TRIBAL SELF...

42 CFR 137.275 - May Self-Governance Tribes include IHS construction programs in a construction project agreement...

Code of Federal Regulations, 2013 CFR

2013-10-01

... 42 Public Health 1 2013-10-01 2013-10-01 false May Self-Governance Tribes include IHS construction... OF HEALTH AND HUMAN SERVICES TRIBAL SELF-GOVERNANCE Construction Purpose and Scope § 137.275 May Self... agreement? Yes, Self-Governance Tribes may choose to assume construction programs in a construction project...

42 CFR 137.275 - May Self-Governance Tribes include IHS construction programs in a construction project agreement...

Code of Federal Regulations, 2010 CFR

2010-10-01

... 42 Public Health 1 2010-10-01 2010-10-01 false May Self-Governance Tribes include IHS construction... OF HEALTH AND HUMAN SERVICES TRIBAL SELF-GOVERNANCE Construction Purpose and Scope § 137.275 May Self... agreement? Yes, Self-Governance Tribes may choose to assume construction programs in a construction project...

42 CFR 137.275 - May Self-Governance Tribes include IHS construction programs in a construction project agreement...

Code of Federal Regulations, 2011 CFR

2011-10-01

... 42 Public Health 1 2011-10-01 2011-10-01 false May Self-Governance Tribes include IHS construction... OF HEALTH AND HUMAN SERVICES TRIBAL SELF-GOVERNANCE Construction Purpose and Scope § 137.275 May Self... agreement? Yes, Self-Governance Tribes may choose to assume construction programs in a construction project...

42 CFR 137.275 - May Self-Governance Tribes include IHS construction programs in a construction project agreement...

Code of Federal Regulations, 2014 CFR

2014-10-01

... 42 Public Health 1 2014-10-01 2014-10-01 false May Self-Governance Tribes include IHS construction... OF HEALTH AND HUMAN SERVICES TRIBAL SELF-GOVERNANCE Construction Purpose and Scope § 137.275 May Self... agreement? Yes, Self-Governance Tribes may choose to assume construction programs in a construction project...

42 CFR 137.275 - May Self-Governance Tribes include IHS construction programs in a construction project agreement...

Code of Federal Regulations, 2012 CFR

2012-10-01

... 42 Public Health 1 2012-10-01 2012-10-01 false May Self-Governance Tribes include IHS construction... OF HEALTH AND HUMAN SERVICES TRIBAL SELF-GOVERNANCE Construction Purpose and Scope § 137.275 May Self... agreement? Yes, Self-Governance Tribes may choose to assume construction programs in a construction project...

Juvenile idiopathic arthritis

MedlinePlus

... www.ncbi.nlm.nih.gov/pubmed/21452260 . Long AR, Rouster-Stevens KA. The role of exercise therapy ... nlm.nih.gov/pubmed/21131338 . Wu EY, Bryan AR, Rabinovich CE. Juvenile idiopathic arthritis. In: Kliegman RM, ...

The Swedish version of the multidimensional scale of perceived social support (MSPSS)--a psychometric evaluation study in women with hirsutism and nursing students.

PubMed

Ekbäck, Maria; Benzein, Eva; Lindberg, Magnus; Arestedt, Kristofer

2013-10-10

The Multidimensional Scale of Perceived Social Support (MSPSS) is a short instrument, developed to assess perceived social support. The original English version has been widely used. The original scale has demonstrated satisfactory psychometric properties in different settings, but no validated Swedish version has been available. The aim was therefore to translate, adapt and psychometrically evaluate the Multidimensional Scale of Perceived Social Support for use in a Swedish context. In total 281 participants accepted to join the study, a main sample of 127 women with hirsutism and a reference sample of 154 nursing students. The MSPSS was translated and culturally adapted according to the rigorous official process approved by WHO. The psychometric evaluation included item analysis, evaluation of factor structure, known-group validity, internal consistency and reproducibility. The original three-factor structure was reproduced in the main sample of women with hirsutism. An equivalent factor structure was demonstrated in a cross-validation, based on the reference sample of nursing students. Known-group validity was supported and internal consistency was good for all scales (α = 0.91-0.95). The test-retest showed acceptable to very good reproducibility for the items (κw = 0.58-0.85) and the scales (ICC = 0.89-0.92; CCC = 0.89-0.92). The Swedish version of the MSPSS is a multidimensional scale with sound psychometric properties in the present study sample. The simple and short format makes it a useful tool for measuring perceived social support.

Contribution of Ih to the relative facilitation of synaptic responses induced by carbachol in the entorhinal cortex during repetitive stimulation of the parasubiculum.

PubMed

Sparks, D W; Chapman, C A

2014-10-10

Neurons in the superficial layers of the entorhinal cortex provide the hippocampus with the majority of its cortical sensory input, and also receive the major output projection from the parasubiculum. This puts the parasubiculum in a position to modulate the activity of entorhinal neurons that project to the hippocampus. These brain areas receive cholinergic projections that are active during periods of theta- and gamma-frequency electroencephalographic (EEG) activity. The purpose of this study was to investigate how cholinergic receptor activation affects the strength of repetitive synaptic responses at these frequencies in the parasubiculo-entorhinal pathway and the cellular mechanisms involved. Whole-cell patch-clamp recordings of rat layer II medial entorhinal neurons were conducted using an acute slice preparation, and responses to 5-pulse trains of stimulation at theta- and gamma-frequency delivered to the parasubiculum were recorded. The cholinergic agonist carbachol (CCh) suppressed the amplitude of single synaptic responses, but also produced a relative facilitation of synaptic responses evoked during stimulation trains. The N-methyl-d-aspartate (NMDA) glutamate receptor blocker APV did not significantly reduce the relative facilitation effect. However, the hyperpolarization-activated cationic current (Ih) channel blocker ZD7288 mimicked the relative facilitation induced by CCh, suggesting that CCh-induced inhibition of Ih could produce the effect by increasing dendritic input resistance (Rin). Inward-rectifying and leak K(+) currents are known to interact with Ih to affect synaptic excitability. Application of the K(+) channel antagonist Ba(2+) depolarized neurons and enhanced temporal summation, but did not block further facilitation of train-evoked responses by ZD7288. The Ih-dependent facilitation of synaptic responses can therefore occur during reductions in inward-rectifying potassium current (IKir) associated with dendritic depolarization. Thus, in

Intraspinal anomalies in early-onset idiopathic scoliosis.

PubMed

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

2017-06-01

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

Angiopoietin-2 polymorphism in women with idiopathic recurrent miscarriage.

PubMed

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.

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

PubMed

Yamada, S; Ishikawa, M; Yamamoto, K

2016-07-01

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

Intestinal Volvulus in Idiopathic Steatorrhea

PubMed Central

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

1963-01-01

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

Analysis of the role of the low threshold currents IT and Ih in intrinsic delta oscillations of thalamocortical neurons

PubMed Central

Amarillo, Yimy; Mato, Germán; Nadal, Marcela S.

2015-01-01

Thalamocortical neurons are involved in the generation and maintenance of brain rhythms associated with global functional states. The repetitive burst firing of TC neurons at delta frequencies (1–4 Hz) has been linked to the oscillations recorded during deep sleep and during episodes of absence seizures. To get insight into the biophysical properties that are the basis for intrinsic delta oscillations in these neurons, we performed a bifurcation analysis of a minimal conductance-based thalamocortical neuron model including only the IT channel and the sodium and potassium leak channels. This analysis unveils the dynamics of repetitive burst firing of TC neurons, and describes how the interplay between the amplifying variable mT and the recovering variable hT of the calcium channel IT is sufficient to generate low threshold oscillations in the delta band. We also explored the role of the hyperpolarization activated cationic current Ih in this reduced model and determine that, albeit not required, Ih amplifies and stabilizes the oscillation. PMID:25999847

Analysis of the role of the low threshold currents IT and Ih in intrinsic delta oscillations of thalamocortical neurons.

PubMed

Amarillo, Yimy; Mato, Germán; Nadal, Marcela S

2015-01-01

Thalamocortical neurons are involved in the generation and maintenance of brain rhythms associated with global functional states. The repetitive burst firing of TC neurons at delta frequencies (1-4 Hz) has been linked to the oscillations recorded during deep sleep and during episodes of absence seizures. To get insight into the biophysical properties that are the basis for intrinsic delta oscillations in these neurons, we performed a bifurcation analysis of a minimal conductance-based thalamocortical neuron model including only the IT channel and the sodium and potassium leak channels. This analysis unveils the dynamics of repetitive burst firing of TC neurons, and describes how the interplay between the amplifying variable mT and the recovering variable hT of the calcium channel IT is sufficient to generate low threshold oscillations in the delta band. We also explored the role of the hyperpolarization activated cationic current Ih in this reduced model and determine that, albeit not required, Ih amplifies and stabilizes the oscillation.

Smoking, Alcohol, Drug Use, Abuse and Dependence in Narcolepsy and Idiopathic Hypersomnia: A Case-Control Study.

PubMed

Barateau, Lucie; Jaussent, Isabelle; Lopez, Régis; Boutrel, Benjamin; Leu-Semenescu, Smaranda; Arnulf, Isabelle; Dauvilliers, Yves

2016-03-01

Basic experiments support the impact of hypocretin on hyperarousal and motivated state required for increasing drug craving. Our aim was to assess the frequencies of smoking, alcohol and drug use, abuse and dependence in narcolepsy type 1 (NT1, hypocretin-deficient), narcolepsy type 2 (NT2), idiopathic hypersomnia (IH) (non-hypocretin-deficient conditions), in comparison to controls. We hypothesized that NT1 patients would be less vulnerable to drug abuse and addiction compared to other hypersomniac patients and controls from general population. We performed a cross-sectional study in French reference centres for rare hypersomnia diseases and included 450 adult patients (median age 35 years; 41.3% men) with NT1 (n = 243), NT2 (n = 116), IH (n = 91), and 710 adult controls. All participants were evaluated for alcohol consumption, smoking habits, and substance (alcohol and illicit drug) abuse and dependence diagnosis during the past year using the Mini International Neuropsychiatric Interview. An increased proportion of both tobacco and heavy tobacco smokers was found in NT1 compared to controls and other hypersomniacs, despite adjustments for potential confounders. We reported an increased regular and frequent alcohol drinking habit in NT1 versus controls but not compared to other hypersomniacs in adjusted models. In contrast, heavy drinkers were significantly reduced in NT1 versus controls but not compared to other hypersomniacs. The proportion of patients with excessive drug use (codeine, cocaine, and cannabis), substance dependence, or abuse was low in all subgroups, without significant differences between either hypersomnia disorder categories or compared with controls. We first described a low frequency of illicit drug use, dependence, or abuse in patients with central hypersomnia, whether Hcrt-deficient or not, and whether drug-free or medicated, in the same range as in controls. Conversely, heavy drinkers were rare in NT1 compared to controls but not to other

Nonsurgical Management of Adolescent Idiopathic Scoliosis.

PubMed

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

2016-08-01

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

A method based on IHS cylindrical transform model for quality assessment of image fusion

NASA Astrophysics Data System (ADS)

Zhu, Xiaokun; Jia, Yonghong

2005-10-01

Image fusion technique has been widely applied to remote sensing image analysis and processing, and methods for quality assessment of image fusion in remote sensing have also become the research issues at home and abroad. Traditional assessment methods combine calculation of quantitative indexes and visual interpretation to compare fused images quantificationally and qualitatively. However, in the existing assessment methods, there are two defects: on one hand, most imdexes lack the theoretic support to compare different fusion methods. On the hand, there is not a uniform preference for most of the quantitative assessment indexes when they are applied to estimate the fusion effects. That is, the spatial resolution and spectral feature could not be analyzed synchronously by these indexes and there is not a general method to unify the spatial and spectral feature assessment. So in this paper, on the basis of the approximate general model of four traditional fusion methods, including Intensity Hue Saturation(IHS) triangle transform fusion, High Pass Filter(HPF) fusion, Principal Component Analysis(PCA) fusion, Wavelet Transform(WT) fusion, a correlation coefficient assessment method based on IHS cylindrical transform is proposed. By experiments, this method can not only get the evaluation results of spatial and spectral features on the basis of uniform preference, but also can acquire the comparison between fusion image sources and fused images, and acquire differences among fusion methods. Compared with the traditional assessment methods, the new methods is more intuitionistic, and in accord with subjective estimation.

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

PubMed

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

2016-05-25

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

Production of fiberboard using corn stalk pretreated with white-rot fungus Trametes hirsute by hot pressing without adhesive.

PubMed

Wu, Jianguo; Zhang, Xin; Wan, Jilin; Ma, Fuying; Tang, Yong; Zhang, Xiaoyu

2011-12-01

Corn stalk pretreated with white-rot fungus Trametes hirsute was used to produce fiberboard by hot pressing without adhesive. The moduli of rupture and elasticity of the corn-stalk-based fiberboard were increased 3.40- and 8.87-fold when bio-pretreated rather than untreated corn stalk was used. Fourier transform infra-red spectroscopy, X-ray diffraction, and chemical analysis showed that bio-pretreated corn stalk increased the mechanical properties of the fiberboard because it had more than twice the number of hydroxyl group, an 18% higher crystallinity, and twice the polysaccharide content of untreated corn stalk. Its laccase content was 4.65 ± 0.38 U/g. Corn stalk-based fiberboard production did not require adhesives, thus eliminating a potential source of toxic emissions such as formaldehyde gas. Copyright © 2011 Elsevier Ltd. All rights reserved.

IgG abnormality in narcolepsy and idiopathic hypersomnia.

PubMed

Tanaka, Susumu; Honda, Makoto

2010-03-05

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

Pathology of idiopathic non-cirrhotic portal hypertension.

PubMed

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.

[Adolescent idiopathic scoliosis].

PubMed

2016-12-01

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

Idiopathic pulmonary fibrosis.

PubMed

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

2017-02-23

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

Idiopathic toe walking.

PubMed

Oetgen, Matthew E; Peden, Sean

2012-05-01

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

CLINICAL CHARACTERISTICS OF IDIOPATHIC FOVEOMACULAR RETINOSCHISIS.

PubMed

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

2016-08-01

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

Optimal management of idiopathic scoliosis in adolescence

PubMed Central

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

2013-01-01

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

Computer-Based Patient Records: Better Planning and Oversight by VA, DOD, and IHS Would Enhance Health Data Sharing

DTIC Science & Technology

2001-04-01

IHS), could share information technology (IT) and patient medical information to provide greater continuity of care, accelerate VA eligibility... patient medical information to provide greater continuity of care, accelerate VA eligibility determinations, and save software development costs.1 In...system, which primarily includes information on patient hospital admission and discharge, patient medications , laboratory results, and radiology

Service Networks and Patterns of Utilization: Mental Health Programs, Indian Health Service (IHS). Volume 5: Billings Area, 1963-1973.

ERIC Educational Resources Information Center

Attneave, Carolyn L.; Beiser, Morton

The fifth volume in a 10-volume report on the historical development (1966-1973) of the 8 administrative Area Offices of the Indian Health Service (IHS) Mental Health Programs, this report presents information on the Billings Area Office. Included in this document are: (1) General Description (geography, demography, and transportation facilities…

Treatment of the idiopathic scoliosis with brace and physiotherapy.

PubMed

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.

Acute exacerbation of idiopathic pulmonary fibrosis triggered by Aspergillus empyema.

PubMed

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

2018-01-01

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

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

PubMed

Segal, Laura; Discepola, Marino

2005-12-01

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

Guidelines for the medical treatment of idiopathic pulmonary fibrosis.

PubMed

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.

Maternal asthma and idiopathic preterm labor.

PubMed

Kramer, M S; Coates, A L; Michoud, M C; Dagenais, S; Moshonas, D; Davis, G M; Hamilton, E F; Nuwayhid, B; Joshi, A K; Papageorgiou, A

1995-11-15

Previous studies suggest that women with asthma are at increased risk of preterm birth. Moreover, drugs (especially beta-agonists) used to treat asthma are also used to treat preterm labor. The authors carried out a case-control study of 555 women from three hospital centers with idiopathic preterm labor (< 37 weeks), including two overlapping (i.e., non-mutually exclusive) subsamples: cases with early idiopathic preterm labor (< 34 weeks) and cases with idiopathic recurrent preterm labor (< 37 weeks plus a previous history of preterm delivery or second-trimester miscarriage). Controls were matched to cases according to race and smoking history prior to and during pregnancy. All subjects responded in person to questions about atopic, respiratory, obstetric, and sociodemographic histories. Subjects in the early and recurrent preterm labor subsamples were also asked to undergo spirometric testing with methacholine challenge 6-12 weeks after delivery. Cases were significantly more likely to report histories of asthma symptoms and physician-diagnosed asthma (matched odds ratios of 2-3) than controls, particularly those cases with recurrent preterm labor. No significant associations were observed, however, with methacholine responsiveness. These results could not be explained by residual confounding by smoking or other variables, nor by selective recall of asthma symptoms and histories by cases. Women with asthma are at increased risk of idiopathic preterm labor. The fact that no such association was seen with methacholine responsiveness suggests that nonatopic, noncholinergic mechanisms may link bronchial and uterine smooth muscle lability.

Altered Regional Cerebral Blood Flow in Idiopathic Hypersomnia.

PubMed

Boucetta, Soufiane; Montplaisir, Jacques; Zadra, Antonio; Lachapelle, Francis; Soucy, Jean-Paul; Gravel, Paul; Dang-Vu, Thien Thanh

2017-10-01

Idiopathic hypersomnia is characterized by excessive daytime sleepiness, despite normal or long sleep time. Its pathophysiological mechanisms remain unclear. This pilot study aims at characterizing the neural correlates of idiopathic hypersomnia using single photon emission computed tomography. Thirteen participants with idiopathic hypersomnia and 16 healthy controls were scanned during resting wakefulness using a high-resolution single photon emission computed tomography scanner with 99mTc-ethyl cysteinate dimer to assess cerebral blood flow. The main analysis compared regional cerebral blood flow distribution between the two groups. Exploratory correlations between regional cerebral blood flow and clinical characteristics evaluated the functional correlates of those brain perfusion patterns. Significance was set at p < .05 after correction for multiple comparisons. Participants with idiopathic hypersomnia showed regional cerebral blood flow decreases in medial prefrontal cortex and posterior cingulate cortex and putamen, as well as increases in amygdala and temporo-occipital cortices. Lower regional cerebral blood flow in the medial prefrontal cortex was associated with higher daytime sleepiness. These preliminary findings suggest that idiopathic hypersomnia is characterized by functional alterations in brain areas involved in the modulation of vigilance states, which may contribute to the daytime symptoms of this condition. The distribution of regional cerebral blood flow changes was reminiscent of the patterns associated with normal non-rapid-eye-movement sleep, suggesting the possible presence of incomplete sleep-wake transitions. These abnormalities were strikingly distinct from those induced by acute sleep deprivation, suggesting that the patterns seen here might reflect a trait associated with idiopathic hypersomnia rather than a non-specific state of sleepiness. © Sleep Research Society 2017. Published by Oxford University Press on behalf of the Sleep

Idiopathic Hypersomnia: Clinical Features and Response to Treatment

PubMed Central

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

2009-01-01

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

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

PubMed

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

2012-10-01

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

Brain MRI findings in patients with idiopathic hypersomnia.

PubMed

Trotti, Lynn Marie; Bliwise, Donald L

2017-06-01

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

42 CFR 136.418 - What should the IHS do if an individual has been charged with an offense but the charge is...

Code of Federal Regulations, 2010 CFR

2010-10-01

... SERVICE, DEPARTMENT OF HEALTH AND HUMAN SERVICES INDIAN HEALTH Indian Child Protection and Family Violence Prevention § 136.418 What should the IHS do if an individual has been charged with an offense but the charge...

Evidence for net renal tubule oxalate secretion in patients with calcium kidney stones

PubMed Central

Zisman, Anna L.; Asplin, John R.; Worcester, Elaine M.; Coe, Fredric L.

2011-01-01

Little is known about the renal handling of oxalate in patients with idiopathic hypercalciuria (IH). To explore the role of tubular oxalate handling in IH and to evaluate whether differences exist between IH and normal controls, we studied 19 IH subjects, 8 normal subjects, and 2 bariatric stone formers (BSF) during a 1-day General Clinical Research Center protocol utilizing a low-oxalate diet. Urine and blood samples were collected at 30- to 60-min intervals while subjects were fasting and after they ate three meals providing known amounts of calcium, phosphorus, sodium, protein, oxalate, and calories. Plasma oxalate concentrations and oxalate-filtered loads were similar between patients (includes IH and BSF) and controls in both the fasting and fed states. Urinary oxalate excretion was significantly higher in patients vs. controls regardless of feeding state. Fractional excretion of oxalate (FEOx) was >1, suggesting tubular secretion of oxalate, in 6 of 19 IH and both BSF, compared with none of the controls (P < 0.00001). Adjusted for water extraction along the nephron, urine oxalate rose more rapidly among patients than normal subjects with increases in plasma oxalate. Our findings identify tubular secretion of oxalate as a key mediator of hyperoxaluria in calcium stone formers, potentially as a means of maintaining plasma oxalate in a tight range. PMID:21123489

Idiopathic granulomatous mastitis: an institutional experience

PubMed Central

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

2017-01-01

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

Idiopathic granulomatous mastitis: a diagnostic dilemma for the breast radiologist.

PubMed

Sripathi, Smiti; Ayachit, Anurag; Bala, Archana; Kadavigere, Rajagopal; Kumar, Sandeep

2016-08-01

Idiopathic granulomatous mastitis is a chronic inflammatory disease of the breast, which is often difficult to differentiate both clinically and radiologically from infectious aetiologies such as tuberculosis, fungal infections, and also from malignancy, thus posing a diagnostic dilemma. We present a pictorial review of the commonly encountered imaging findings in idiopathic granulomatous mastitis on mammography and ultrasound. Mammographic and ultrasound findings of histopathologically proven cases of granulomatous mastitis are discussed. Idiopathic granulomatous mastitis has varied and non-specific appearances on ultrasound and mammography. Histopathology is essential to establish diagnosis. • Idiopathic granulomatous mastitis often poses a diagnostic dilemma for the radiologist by mimicking malignancy. • It has varied and non-specific appearances on mammography and ultrasound. • Histopathology is mandatory to establish the diagnosis and decide management.

Idiopathic scrotal elephantiasis.

PubMed

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

2005-02-01

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

Thalamocortical neurons display suppressed burst-firing due to an enhanced Ih current in a genetic model of absence epilepsy.

PubMed

Cain, Stuart M; Tyson, John R; Jones, Karen L; Snutch, Terrance P

2015-06-01

Burst-firing in distinct subsets of thalamic relay (TR) neurons is thought to be a key requirement for the propagation of absence seizures. However, in the well-regarded Genetic Absence Epilepsy Rats from Strasbourg (GAERS) model as yet there has been no link described between burst-firing in TR neurons and spike-and-wave discharges (SWDs). GAERS ventrobasal (VB) neurons are a specific subset of TR neurons that do not normally display burst-firing during absence seizures in the GAERS model, and here, we assessed the underlying relationship of VB burst-firing with Ih and T-type calcium currents between GAERS and non-epileptic control (NEC) animals. In response to 200-ms hyperpolarizing current injections, adult epileptic but not pre-epileptic GAERS VB neurons displayed suppressed burst-firing compared to NEC. In response to longer duration 1,000-ms hyperpolarizing current injections, both pre-epileptic and epileptic GAERS VB neurons required significantly more hyperpolarizing current injection to burst-fire than those of NEC animals. The current density of the Hyperpolarization and Cyclic Nucleotide-activated (HCN) current (Ih) was found to be increased in GAERS VB neurons, and the blockade of Ih relieved the suppressed burst-firing in both pre-epileptic P15-P20 and adult animals. In support, levels of HCN-1 and HCN-3 isoform channel proteins were increased in GAERS VB thalamic tissue. T-type calcium channel whole-cell currents were found to be decreased in P7-P9 GAERS VB neurons, and also noted was a decrease in CaV3.1 mRNA and protein levels in adults. Z944, a potent T-type calcium channel blocker with anti-epileptic properties, completely abolished hyperpolarization-induced VB burst-firing in both NEC and GAERS VB neurons.

Service Networks and Patterns of Utilization: Mental Health Programs, Indian Health Service (IHS). Volume 9: Portland Area, 1966-1973.

ERIC Educational Resources Information Center

Attneave, Carolyn L.; Beiser, Morton

The ninth volume in a 10-volume report on the historical development (1966-1973) of the 8 administrative Area Offices of the Indian Health Service (IHS) Mental Health Programs, this report presents information on the Portland Area Office. Included in this document are: (1) The Context (early history of the Oregon Territory, geography and tribal…

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

PubMed

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

2009-01-01

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

[Physical therapy for idiopathic scoliosis].

PubMed

Steffan, K

2015-11-01

The objective is the description and summary of the current state of idiopathic scoliosis treatment with physical therapy based on new scientific knowledge and concluded from more than 15 years of experience as a leading physician in two well-known clinics specializing in the conservative treatment of scoliosis. Based on current scientific publications on physical therapy in scoliosis treatment and resulting from the considerable personal experience gained working with conservative treatment and consulting scoliosis patients (as inpatients and outpatients), the current methods of physical therapy have been compared and evaluated. Physical therapy according to Schroth and Vojta therapy are at present the most common and effective methods in the physical treatment of idiopathic scoliosis. These methods can be applied during inpatient or outpatient treatment or intensified in the practice of specialized therapists. As there are only a few scientific studies on this subject, the author's findings are based mainly on his own experiences of the conservative treatment of idiopathic scoliosis. Athough these experiences are the results of over 15 years of working in the field of therapy, and the Schroth method in combination with corrective bracing presents highly promising results, it would nevertheless be desirable to conduct detailed scientific studies to verify the effectiveness of conservative treatment.

Service Networks and Patterns of Utilization: Mental Health Programs, Indian Health Service (IHS). Volume 8: Phoenix Area, 1966-1974.

ERIC Educational Resources Information Center

Attneave, Carolyn L.; Beiser, Morton

The eighth volume in a 10-volume report on the historical development (1966-1973) of the 8 administrative Area Offices of the Indian Health Service (IHS) Mental Health Programs, this report presents information on the Phoenix Area Office and the Tucson Sub-Area Office. Included in this document are: (1) The Context: Political and Geographic (the…

[Vitrectomy for idiopathic and secondary preretinal macular membrane].

PubMed

Oficjalska-Młyńczak, Jolanta; Jamrozy-Witkowska, Agnieszka

2004-01-01

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

Idiopathic hypersomnia: clinical features and response to treatment.

PubMed

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

2009-12-15

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

42 CFR 410.46 - Physician and other practitioner services furnished in or at the direction of an IHS or Indian...

Code of Federal Regulations, 2010 CFR

2010-10-01

... in or at the direction of an IHS or Indian tribal hospital or clinic: Scope and conditions. 410.46... tribal hospital or clinic: Scope and conditions. (a) Medicare Part B pays, in accordance with the physician fee schedule, for services furnished in or at the direction of a hospital or outpatient clinic...

42 CFR 136.404 - What does the Indian Child Protection and Family Violence Prevention Act require of the IHS and...

Code of Federal Regulations, 2010 CFR

2010-10-01

... 42 Public Health 1 2010-10-01 2010-10-01 false What does the Indian Child Protection and Family Violence Prevention Act require of the IHS and Indian Tribes or Tribal organizations receiving funds under... Protection and Family Violence Prevention § 136.404 What does the Indian Child Protection and Family Violence...

Idiopathic inflammatory myopathies overlapping with systemic diseases

PubMed Central

Lepreux, Sébastien; Hainfellner, Johannes A.; Vital, Anne

2018-01-01

A muscle biopsy is currently requested to assess the diagnosis of an idiopathic inflammatory myopathy overlapping with a systemic disease. During the past few years, the classification of inflammatory myopathy subtypes has been revisited progressively on the basis of correlations between clinical phenotypes, autoantibodies and histological data. Several syndromic entities are now more clearly defined, and the aim of the present review is to clarify the contribution of muscle biopsy in a setting of idiopathic inflammatory myopathies overlapping with systemic diseases. PMID:29154752

Obesity is a significant susceptibility factor for idiopathic AA amyloidosis.

PubMed

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

2018-03-01

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

Placental villous hypermaturation is associated with idiopathic preterm birth

PubMed Central

Morgan, Terry K.; Tolosa, Jorge E.; Mele, Lisa; Wapner, Ronald J.; Spong, Catherine Y.; Sorokin, Yoram; Dudley, Donald J.; Peaceman, Alan M.; Mercer, Brian M.; Thorp, John M.; O’Sullivan, Mary Jo; Ramin, Susan M.; Rouse, Dwight J.; Sibai, Baha

2014-01-01

Objective Pregnancy complications such as intra-amniotic infection, preeclampsia, and fetal intrauterine growth restriction (IUGR) account for most cases of preterm birth (PTB), but many spontaneous PTB cases do not have a clear etiology. We hypothesize that placental insufficiency may be a potential cause of idiopathic PTB. Methods Secondary analysis of 82 placental samples from women with PTB obtained from a multicenter trial of repeat versus single antenatal corticosteroids. Samples were centrally reviewed by a single placental pathologist masked to clinical outcomes. The histopathologic criterion for infection was the presence of acute chorioamnionitis defined as neutrophils marginating into the chorionic plate. Placental villous hypermaturation (PVH) was defined as a predominance of terminal villi (similar to term placenta) with extensive syncytial knotting. Idiopathic PTB comprised a group without another known etiology such as preeclampsia, IUGR or infection. Results Acute chorioamnionitis was observed in 33/82 (40%) cases. Other known causes of PTB were reported in 18/82 (22%). The remaining 31/82 (38%) were idiopathic. The frequency of PVH in idiopathic PTB (26/31=84%) was similar to cases with IUGR or preeclampsia (16/ 18=89%), but significantly more common than PVH in the group with acute chorioamnionitis (10/33=30%) (p<0.001). Conclusions PVH, which is a histologic marker of relative placental insufficiency, is a common finding in idiopathic PTB. PMID:23130816

Allelic variation of the FRMD7 gene in congenital idiopathic nystagmus.

PubMed

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.

Study on Treatment with Respect to Idiopathic Scoliosis

NASA Astrophysics Data System (ADS)

Takeuchi, Kenzen; Azegami, Hideyuki; Murachi, Shunji; Kitoh, Junzoh; Ishida, Yoshito; Kawakami, Noriaki; Makino, Mitsunori

A hypothesis that the thoracic idiopathic scoliosis is buckling phenomenon of the fourth mode induced by the growth of thoracic vertebral bodies was presented in the previous work by the authors using numerical simulations with finite element model of the spine. If the hypothesis is acceptable, sensitivity function with respect to the critical growth of thoracic vertebrae on the maximization problem of buckling load with the fourth buckling mode gives us useful information to improve and develop treatments for the idiopathic scoliosis. The numerical results analyzed by the finite element method demonstrated that the sensitivity function is high at the articular capsules of the intervertebral joints, the intervertebral disks, the costotransverse joints and the constovertebral joints around the apex of the curvature in the case of the thoracic idiopathic scoliosis.

Idiopathic orbital inflammatory disease successfully treated with rituximab.

PubMed

Schafranski, Marcelo Derbli

2009-02-01

We report a case of a 66-year-old Caucasian female with a diagnosis of idiopathic orbital inflammatory disease (OID) refractory to azathioprine therapy. The coexistence of diabetes mellitus represented a relative contraindication to chronic prednisone use. After two infusions of rituximab, a chimeric anti-CD20+ antibody, ophthalmic signs and symptoms remarkably improved. To our knowledge, this is the first case of idiopathic OID successfully treated with rituximab.

Pathogenesis, Newly Recognized Etiologies, and Management of Idiopathic Anaphylaxis

PubMed Central

Kuhlen, James L.; Virkud, Yamini V.

2018-01-01

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

77 FR 5087 - Notice of Intent To Prepare an Environmental Impact Statement; USH 51, IH 39/90 East of Stoughton...

Federal Register 2010, 2011, 2012, 2013, 2014

2012-02-01

... Environmental Impact Statement; USH 51, IH 39/90 East of Stoughton to USH 12/18 (Madison South Beltline Highway... between Interstate 39/90 east of the City of Stoughton and US 12/18 (Madison South Beltline Highway). The.../18 (Madison South Beltline Highway), north of the Village of McFarland. The study will also examine a...

Argentinean adaptation of the Social Skills Inventory IHS-Del-Prette.

PubMed

Olaz, Fabián Orlando; Medrano, Leonardo; Greco, María Eugenia; Del Prette, Zilda Aparecida Pereira

2009-11-01

We present the results of the adaptation of the IHS-Del-Prette (Inventario de Habilidades Sociales, in English, Social Skills Inventory) to a sample of Argentinean college students. Firstly, we addressed the backward translation and carried out an equivalence study of the Portuguese and Spanish versions of the scale. The results showed the two versions were equivalent, as we obtained correlations lower than .50 in only 5 items. Secondly, we performed item analysis by calculating discrimination indexes and item-total correlations. Results indicated that the items are sensitive to differentiate between high and low social-skill groups. Exploratory factor analysis carried out with a sample of 602 college students yielded five factors that explained 26.5% of the total variance, although our data did not completely match the original factor structure. We also obtained moderate alpha values for the subscales, but high reliability for the total scale. Lastly, group differences between males and females are presented to provide evidence of validity. We discuss the implications of the results and present future lines of inquiry.

MR venography in idiopathic intracranial hypertension: unappreciated and misunderstood

PubMed Central

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

2004-01-01

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

Microstructural evolution during thermal annealing of ice-Ih

NASA Astrophysics Data System (ADS)

Hidas, Károly; Tommasi, Andréa; Mainprice, David; Chauve, Thomas; Barou, Fabrice; Montagnat, Maurine

2017-06-01

We studied the evolution of the microstructure of ice-Ih during static recrystallization by stepwise annealing experiments. We alternated thermal annealing and electron backscatter diffraction (EBSD) analyses on polycrystalline columnar ice pre-deformed in uniaxial compression at temperature of -7 °C to macroscopic strains of 3.0-5.2. Annealing experiments were carried out at -5 °C and -2 °C up to a maximum of 3.25 days, typically in 5-6 steps. EBSD crystal orientation maps obtained after each annealing step permit the description of microstructural changes. Decrease in average intragranular misorientation at the sample scale and modification of the misorientation across subgrain boundaries provide evidence for recovery from the earliest stages of annealing. This initial evolution is similar for all studied samples irrespective of their initial strain or annealing temperature. After an incubation period ≥1.5 h, recovery is accompanied by recrystallization (nucleation and grain boundary migration). Grain growth proceeds at the expense of domains with high intragranular misorientations, consuming first the most misorientated parts of primary grains. Grain growth kinetics fits the parabolic growth law with grain growth exponents in the range of 2.4-4.0. Deformation-induced tilt boundaries and kink bands may slow down grain boundary migration. They are stable features during early stages of static recrystallization, only erased by normal growth, which starts after >24 h of annealing.

Idiopathic scrotal calcinosis.

PubMed

Celik, Orcun; Ipekci, Tumay; Kazimoglu, Hatem

2013-12-01

Idiopathic scrotal calcinosis is a rare scrotal benign disease. Its distinct features are painless, non-pruritic, semi-soft palpable calcific transdermal nodules. We report a 42-year-old-man with asymptomatic multiple calcified scrotal skin nodules for 10 years. Under spinal anesthesia, the affected scrotal skin was excised and the nodules removed. We aim to explain the etiology, pathophysiology, diagnosis, and treatment modalities of this rare disease.

Inelastic properties of ice Ih at low temperatures and high pressures

USGS Publications Warehouse

Kirby, S.H.; Durham, W.B.; Beeman, M.L.; Heard, H.C.; Daley, M.A.

1987-01-01

The aim of our research programme is to explore the rheological behavior of H2O ices under conditions appropriate to the interiors of the icy satellites of the outer planets in order to give insight into their deformation. To this end, we have performed over 100 constant-strain-rate compression tests at pressures to 500 MPa and temperatures as low as 77 K. At P > 30 MPa, ice Ih fails by a shear instability producing faults in the maximum shear stress orientation and failure strength typically is independent of pressure. This unusual faulting behavior is thought to be connected with phase transformations localized in the shear zones. The steady-state strength follows rheological laws of the thermally-activated power-law type, with different flow law parameters depending on the range of test temperatures. The flow laws will be discussed with reference to the operating deformation mechanisms as deduced from optical-scale microstructures and comparison with other work.

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

PubMed

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

2017-09-01

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

Idiopathic hemifacial spasm responsive to zonisamide: a case report.

PubMed

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

2009-01-01

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

Testicular atrophy and reproductive quiescence in photorefractory and scotosensitive quail: Involvement of hypothalamic deep brain photoreceptors and GnRH-GnIH system.

PubMed

Banerjee, Somanshu; Chaturvedi, Chandra Mohini

2017-10-01

Birds time their daily and seasonal activities in synchronization with circadian and annual periodicities in the environment, which is mainly provided by changes in photoperiod/day length conditions. Photoperiod appears to act at the level of eye, pineal and encephalic/deep brain photoperception and thus entrain the hypothalamic clock as well as reproductive circuitry in different avian species. In this article our focus of study is to elucidate out the underlying molecular mechanism of modulation of the hypothalamic reproductive circuitry following the photoperception through the hypothalamic photoreceptor cells and the subsequent alteration in the reproductive responses in quail, kept under different simulated photoperiodic conditions. Present study investigated the different simulated photoperiodic conditions induced hypothalamic DBP-GnRH-GnIH system mediated translation of photoperiodic information and subsequent exhibition of differential photosexual responses (scoto-/photo-sensitivity and refractoriness) in Japanese quail, Coturnix coturnix japonica. Paired testes weight and paired testicular volume increased 15.9 and 22.6-fold respectively in scotorefractory quail compare to that of scotosensitive phase and 12.8 and 24.3-fold in photosensitive quail compare to that of photorefractory phase. The pineal/eye melatonin (through melatonin receptor subtype Mel 1c R) and hypothalamic deep brain photoreceptor (DBPs) cells directly modulate the hypothalamic GnRH-I/II and GnIH system and thus exhibit testicular stimulation or regression in response to different photoperiodic conditions (PS, PR, SS and SR). The hypothalamic alteration of DBP(s) and GnRH-GnIH system thus may induce the testicular stimulation in PS and SR quail and testicular regression in SS and PR quail. Copyright © 2017 Elsevier B.V. All rights reserved.

Idiopathic central diabetes Insipidus.

PubMed

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.

Idiopathic thoracic transdural intravertebral spinal cord herniation

PubMed Central

Turel, Mazda K; Wewel, Joshua T; Kerolus, Mena G; O'Toole, John E

2017-01-01

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. PMID:29021685

Multiple idiopathic external apical root resorption: report of four cases.

PubMed

Cholia, S S; Wilson, P H R; Makdissi, J

2005-07-01

Multiple idiopathic external root resorption is an unusual condition that may present in a cervical or an apical form. In this article, we review the published literature relating to multiple idiopathic external apical root resorption and present four clinical cases. We consider the aetiology of this condition and discuss the various treatment options.

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

PubMed

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

2015-01-01

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

Disease of the year: juvenile idiopathic arthritis--differential diagnosis.

PubMed

Hu-Torres, Sandra; Foster, C Stephen

2014-02-01

The purpose of this review is to comprehensively explain the differential diagnosis of juvenile idiopathic arthritis-associated uveitis. Web-based literature review. Main diagnostic decisions are made through a thorough anterior segment exam and a comprehensive exploration of past medical and family history. High clinical suspicion of other uveitic entities occurring in children is necessary and must be excluded by the practitioner before immediate diagnosis of juvenile idiopathic arthritis is made.

[Coincidence of juvenile idiopathic arthritis and multiple sclerosis: case report].

PubMed

Puszczewicz, Mariusz J; Tuchocka-Piotrowska, Aleksandra; Majewski, Dominik; Kołczewska, Aleksandra

2006-01-01

Juvenile idiopathic arthritis is a systemic pathology of connective tissue characterized by a chronic inflammatory process with an autoimmune background whereas multiple sclerosis is a demyelination disease with an important role of immune disorders in its pathogenesis. The etiology in both cases remains unknown. The coincidence of juvenile idiopathic arthritis and multiple sclerosis was described a just a few patients. We now report on a 31-year-old woman with juvenile idiopathic arthritis and multiple sclerosis. In the present case, the main problem was to find the right proper medication for a very, aggressive course of multiple sclerosis and for arthritis. Treatment with interferon-beta and methylprednisolone led to remission with just minor side-effects.

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

PubMed

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

2002-01-01

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

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

PubMed

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).

PubMed

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

2018-06-01

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

Idiopathic hypertrophic pachymeningitis presenting with occipital neuralgia.

PubMed

Auboire, Laurent; Boutemy, Jonathan; Constans, Jean Marc; Le Gallou, Thomas; Busson, Philippe; Bienvenu, Boris

2015-03-01

Although occipital neuralgia is usually caused by degenerative arthropathy, nearly 20 other aetiologies may lead to this condition. We present the first case report of hypertrophic pachymeningitis revealed by isolated occipital neuralgia. Idiopathic hypertrophic pachymeningitis is a plausible cause of occipital neuralgia and may present without cranial-nerve palsy. There is no consensus on the treatment for idiopathic hypertrophic pachymeningitis, but the usual approach is to start corticotherapy and then to add immunosuppressants. When occipital neuralgia is not clinically isolated or when a first-line treatment fails, another disease diagnosis should be considered. However, the cost effectiveness of extended investigations needs to be considered.

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

PubMed

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

2018-04-01

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

Genetics Home Reference: idiopathic pulmonary fibrosis

MedlinePlus

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

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

PubMed

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.

Giant scrotal elephantiasis: an idiopathic case.

PubMed

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

2010-01-01

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

Idiopathic hypersomnia: a report of three adolescent-onset cases in a two-generation family.

PubMed

Janácková, Sona; Motte, Jacques; Bakchine, Serge; Sforza, Emilia

2011-04-01

Idiopathic hypersomnia is an uncommon sleep disorder characterized by prolonged sleep time and excessive daytime sleepiness without cataplexy. This study concerned a case of familial occurrence. The proband expressed an idiopathic hypersomnia with long sleep time at the age of 12 years. Clinical interview and ad libitum polysomnographic study did not reveal any symptoms of narcolepsy or other sleep disorders. Family history revealed that a 20-year-old sister had experienced symptoms of hypersomnia from the age of 16 and their mother had been diagnosed with idiopathic hypersomnia previously. The diagnosis of idiopathic hypersomnia with long sleep time was confirmed in the sister by clinical interview and ad libitum polysomnography. Human leukocyte antigen (HLA) did not reveal the DQB1-0602 phenotype in the proband and relatives. This report confirms the hypothesis of a genetic predisposition in idiopathic hypersomnia.

Screening for Adolescent Idiopathic Scoliosis: US Preventive Services Task Force Recommendation Statement.

PubMed

Grossman, David C; Curry, Susan J; Owens, Douglas K; Barry, Michael J; Davidson, Karina W; Doubeni, Chyke A; Epling, John W; Kemper, Alex R; Krist, Alex H; Kurth, Ann E; Landefeld, C Seth; Mangione, Carol M; Phipps, Maureen G; Silverstein, Michael; Simon, Melissa A; Tseng, Chien-Wen

2018-01-09

Adolescent idiopathic scoliosis, a lateral curvature of the spine of unknown cause with a Cobb angle of at least 10°, occurs in children and adolescents aged 10 to 18 years. Idiopathic scoliosis is the most common form and usually worsens during adolescence before skeletal maturity. Severe spinal curvature may be associated with adverse long-term health outcomes (eg, pulmonary disorders, disability, back pain, psychological effects, cosmetic issues, and reduced quality of life). Early identification and effective treatment of mild scoliosis could slow or stop curvature progression before skeletal maturity, thereby improving long-term outcomes in adulthood. To update the 2004 US Preventive Services Task Force (USPSTF) recommendation on screening for idiopathic scoliosis in asymptomatic adolescents. The USPSTF reviewed the evidence on the benefits and harms of screening for and treatment of adolescent idiopathic scoliosis. The USPSTF found no direct evidence on screening for adolescent idiopathic scoliosis and health outcomes and no evidence on the harms of screening. The USPSTF found inadequate evidence on treatment with exercise and surgery. It found adequate evidence that treatment with bracing may slow curvature progression in adolescents with mild or moderate curvature severity (Cobb angle <40° to 50°); however, evidence on the association between reduction in spinal curvature in adolescence and long-term health outcomes in adulthood is inadequate. The USPSTF found inadequate evidence on the harms of treatment. Therefore, the USPSTF concludes that the current evidence is insufficient and that the balance of benefits and harms of screening for adolescent idiopathic scoliosis cannot be determined. The USPSTF concludes that the current evidence is insufficient to assess the balance of benefits and harms of screening for adolescent idiopathic scoliosis in children and adolescents aged 10 to 18 years. (I statement).

Anomalous diffusion of water molecules at grain boundaries in ice Ih.

PubMed

Moreira, Pedro Augusto Franco Pinheiro; Veiga, Roberto Gomes de Aguiar; Ribeiro, Ingrid de Almeida; Freitas, Rodrigo; Helfferich, Julian; de Koning, Maurice

2018-05-23

Using ab initio and classical molecular dynamics simulations, we study pre-melting phenomena in pristine coincident-site-lattice grain boundaries (GBs) in proton-disordered hexagonal ice Ih at temperatures just below the melting point Tm. Concerning pre-melt-layer thicknesses, the results are consistent with the available experimental estimates for low-disorder impurity-free GBs. With regard to molecular mobility, the simulations provide a key new insight: the translational motion of the water molecules is found to be subdiffusive for time scales from ∼10 ns up to at least 0.1 μs. Moreover, the fact that the anomalous diffusion occurs even at temperatures just below Tm where the bulk supercooled liquid still diffuses normally suggests that it is related to the confinement of the GB pre-melt layers by the surrounding crystalline environment. Furthermore, we show that this behavior can be characterized by continuous-time random walk models in which the waiting-time distributions decay according to power-laws that are very similar to those describing dynamics in glass-forming systems.

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

PubMed

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

2011-01-01

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

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

PubMed

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

2015-11-01

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

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

PubMed

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).

Pelvic confined idiopathic retroperitoneal fibrosis mimicking a large tumor.

PubMed

Salemis, N S; Tsiambas, E; Tsohataridis, E

2009-01-01

Idiopathic retroperitoneal fibrosis (IRF) entirely confined to the pelvic cavity is an extremely rare clinical entity. Herein, is described the case of a 36- year old male who presented with clinical and imaging manifestations of a large pelvic tumor. Exploratory laparotomy revealed a large mass in the right pelvis originating from the retroperitoneal space, displacing the right iliac vessels, the right ureter and the urinary bladder completely to the left. A laborious resection of the mass measuring 14 x 10cm was performed. Histopathological examination and detailed immunohistochemistry analysis were suggestive of idiopathic retroperitoneal fibrosis with no evidence of malignancy. This is a very rare case regarding localization and clinical presentation of idiopathic retroperitoneal fibrosis. We conclude that IRF should be included in the differential diagnosis of patients presenting with a pelvic mass even if there is no involvement of the typical para aortic area.

Locomotor skills and balance strategies in adolescents idiopathic scoliosis.

PubMed

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

2007-01-01

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

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

PubMed

Yamada, Shigeki; Ishikawa, Masatsune; Yamamoto, Kazuo

2017-01-01

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

Idiopathic pulmonary fibrosis: evolving concepts.

PubMed

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.

[Physiopathology of idiopathic hypersomnia. Current studies and new orientations].

PubMed

Billiard, M; Rondouin, G; Espa, F; Dauvilliers, Y; Besset, A

2001-11-01

In 1976 Bedrich Roth coined the term "idiopathic hypersomnia" and described two forms of the disease, one monosymptomatic, manifested only by excessive daytime sleepiness, and one polysymptomatic, characterized by excessive daytime sleepiness, nocturnal sleep of abnormally long duration and signs of "sleep drunkenness" on awakening. In comparison with that of narcolepsy, the pathophysiology of idiopathic hypersomnia remains poorly known. There are two main reasons for that: the absence of clinical and polysomnographic criteria pathognomonic or at least characteristic of the condition, as the cataplexies and the sleep onset REM periods of narcolepsy, and also the absence of a natural animal model comparable with the canine model of narcolepsy. The first investigations have stressed the frequent familial pattern of idiopathic hypersomnia. Later on biochemical assays have been performed in the CSF with results in favour of a dysfunction of noradrenergic systems. In the light of the two process model of sleep regulation in which sleep propensity is determined by a homeostatic process S and a circadian process C and of the later three-process model of regulation in which sleepiness/alertness are simulated by the combined action of a homeostatic process, a circadian process and sleep inertia, we suggest that idiopathic hypersomnia is not a pathological entity in itself, but rather the consequence of chronic sleep deprivation in very long sleepers.

Interventional Radiologic Treatment for Idiopathic Portal Hypertension

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

Hirota, Shozo; Ichikawa, Satoshi; Matsumoto, Shinichi

1999-07-15

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

Neurodevelopment in preschool idiopathic toe-walkers.

PubMed

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.

Idiopathic noncirrhotic portal hypertension: current perspectives.

PubMed

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.

Ethanol Effects on Dopaminergic Ventral Tegmental Area Neurons During Block of Ih: Involvement of Barium-Sensitive Potassium Currents

PubMed Central

McDaid, John; McElvain, Maureen A.; Brodie, Mark S.

2008-01-01

The dopaminergic neurons of the ventral tegmental area (DA VTA neurons) are important for the rewarding and reinforcing properties of drugs of abuse, including ethanol. Ethanol increases the firing frequency of DA VTA neurons from rats and mice. Because of a recent report on block of ethanol excitation in mouse DA VTA neurons with ZD7288, a selective blocker of the hyperpolarization-activated cationic current Ih, we examined the effect of ZD7288 on ethanol excitation in DA VTA neurons from C57Bl/6J and DBA/2J mice and Fisher 344 rats. Ethanol (80 mM) caused only increases in firing rate in mouse DA VTA neurons in the absence of ZD7288, but in the presence of ZD7288 (30 μM), ethanol produced a more transient excitation followed by a decrease of firing. This same biphasic phenomenon was observed in DA VTA neurons from rats in the presence of ZD7288 only at very high ethanol concentrations (160–240 mM) but not at lower pharmacologically relevant concentrations. The longer latency ethanol-induced inhibition was not observed in DA VTA neurons from mice or rats in the presence of barium (100 μM), which blocks G protein–linked potassium channels (GIRKs) and other inwardly rectifying potassium channels. Ethanol may have a direct effect to increase an inhibitory potassium conductance, but this effect of ethanol can only decrease the firing rate if Ih is blocked. PMID:18614756

An ether -à-go-go K+ current, Ih-eag, contributes to the hyperpolarization of human fusion-competent myoblasts

PubMed Central

Bijlenga, Philippe; Occhiodoro, Teresa; Liu, Jian-Hui; Bader, Charles R; Bernheim, Laurent; Fischer-Lougheed, Jacqueline

1998-01-01

Two early signs of human myoblast commitment to fusion are membrane potential hyperpolarization and concomitant expression of a non-inactivating delayed rectifier K+ current, IK(NI). This current closely resembles the outward K+ current elicited by rat ether-à-go-go (r-eag) channels in its range of potential for activation and unitary conductance.It is shown that activation kinetics of IK(NI), like those of r-eag, depend on holding potential and on [Mg2+]o, and that IK(NI), like r-eag, is reversibly inhibited by a rise in [Ca2+].Forced expression of an isolated human ether-à-go-go K+ channel (h-eag) cDNA in undifferentiated myoblasts generates single-channel and whole-cell currents with remarkable similarity to IK(NI).h-eag current (Ih-eag) is reversibly inhibited by a rise in [Ca2+]i, and the activation kinetics depend on holding potential and [Mg2+]o.Forced expression of h-eag hyperpolarizes undifferentiated myoblasts from −9 to −50 mV, the threshold for the activation of both Ih-eag and IK(NI). Similarly, the higher the density of IK(NI), the more hyperpolarized the resting potential of fusion-competent myoblasts.It is concluded that h-eag constitutes the channel underlying IK(NI) and that it contributes to the hyperpolarization of fusion-competent myoblasts. To our knowledge, this is the first demonstration of a physiological role for a mammalian eag K+ channel. PMID:9763622

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

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

Pochis, W.T.; Krasnow, A.Z.; Collier, B.D.

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 oftenmore » needed for accurate differential diagnosis.« less

Spinal Manipulative Therapy for Adolescent Idiopathic Scoliosis: A Systematic Review.

PubMed

Théroux, Jean; Stomski, Norman; Losco, Christine Dominique; Khadra, Christelle; Labelle, Hubert; Le May, Sylvie

The purpose of this study was to perform a systematic review of clinical trials of spinal manipulative therapy for adolescent idiopathic scoliosis. Search strategies were developed for PubMed, CINHAL, and CENTRAL databases. Studies were included through June 2016 if they were prospective trials that evaluated spinal manipulative therapy (eg, chiropractic, osteopathic, physical therapy) for adolescent idiopathic scoliosis. Data were extracted and assessed by 2 independent reviewers. Cochrane risk of bias tools were used to assess the quality of the included studies. Data were reported qualitatively because heterogeneity prevented statistical pooling. Four studies satisfied the inclusion criteria and were critically appraised. The findings of the included studies indicated that spinal manipulative therapy might be effective for preventing curve progression or reducing Cobb angle. However, the lack of controls and small sample sizes precluded robust estimation of the interventions' effect sizes. There is currently insufficient evidence to establish whether spinal manipulative therapy may be beneficial for adolescent idiopathic scoliosis. The results of the included studies suggest that spinal manipulative therapy may be a promising treatment, but these studies were all at substantial risk of bias. Further high-quality studies are warranted to conclusively determine if spinal manipulative therapy may be effective in the management of adolescent idiopathic scoliosis. Copyright © 2017. Published by Elsevier Inc.

STELLATE NONHEREDITARY IDIOPATHIC FOVEOMACULAR RETINOSCHISIS ACCOMPANIED BY CONTRALATERAL PERIPHERAL RETINOSCHISIS.

PubMed

Ahmed, Daniel; Stattin, Martin; Glittenberg, Carl; Krebs, Ilse; Ansari-Shahrezaei, Siamak

2017-01-16

To present a patient with stellate nonhereditary idiopathic foveomacular retinoschisis on one eye and peripheral retinoschisis without foveal affection on the other eye. A case report with complete workup of family history and clinical examination, including multimodal imaging with optical coherence tomography and angiography, fluorescein angiography, and infrared fundus imaging. Genetic testing for gene mutation XRLS1 was performed. A white woman with unremarkable medical history presented with stellate foveal splitting of the outer plexiform layer on the right eye and peripheral splitting of the outer plexiform layer on both eyes. All known allegeable trigger factors for the existence of a hereditary or acquired foveomacular retinoschisis were ruled out either by clinical presentation or genetic testing. This led to the diagnosis of stellate nonhereditary idiopathic foveomacular retinoschisis with central involvement only present on one eye. Although peripheral schisis of the outer plexiform layer is often concomitant with central splitting in X-linked juvenile retinoschisis, this is the first known report of nonhereditary cleavage of the outer plexiform layer of the peripheral retina without central affection in a patient with documented stellate nonhereditary idiopathic foveomacular retinoschisis on the other eye. These findings suggest an accurate bilateral examination of the peripheral retina while confirming the diagnose of stellate nonhereditary idiopathic foveomacular retinoschisis.

Genetic (idiopathic) epilepsy with photosensitive seizures includes features of both focal and generalized seizures.

PubMed

Xue, Jiao; Gong, Pan; Yang, Haipo; Liu, Xiaoyan; Jiang, Yuwu; Zhang, Yuehua; Yang, Zhixian

2018-04-19

Clinically, some patients having genetic (idiopathic) epilepsy with photosensitive seizures were difficult to be diagnosed. We aimed to discuss whether the genetic (idiopathic) epilepsy with photosensitive seizures is a focal entity, a generalized entity or a continuum. Twenty-two patients with idiopathic epilepsies and photoconvulsive response (PCR) were retrospectively recruited. In the medical records, the seizure types included "generalized tonic-clonic seizures (GTCS)" in 15, "partial secondarily GTCS (PGTCS)" in 3, partial seizures (PS) in 3, myoclonic seizures in 2, eyelid myoclonus in one, and only febrile seizures in one. Seizure types of PCR included GTCS (1/22), PGTCS (6/22), PS (9/22), electrical seizures (ES) (3/22) and GTCS/PGTCS (3/22). Combined the medical history with PCR results, they were diagnosed as: idiopathic (photosensitive) occipital lobe epilepsy (I(P)OE) in 12, genetic (idiopathic) generalized epilepsy (GGE) in one, GGE/I(P)OE in 5, pure photosensitive seizure in one, and epilepsy with undetermined generalized or focal seizure in 3. So, the dichotomy between generalized and focal seizures might have been out of date regarding to pathophysiological advances in epileptology. To some extent, it would be better to recognize the idiopathic epilepsy with photosensitive seizures as a continuum between focal and generalized seizures.

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

PubMed Central

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

2016-01-01

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

McArdle's disease: A differential diagnosis of idiopathic toe walking.

PubMed

Pomarino, David; Martin, Stephan; Pomarino, Andrea; Morigeau, Stefanie; Biskup, Saskia

2018-06-01

Idiopathic toe walking (ITW) is a pathological gait pattern in which children walk on their tip toes with no orthopedic or neurological reason. Physiological characteristics of the gastrocnemius muscles, the Achilles tendon, and the foot of toe walkers differ from subjects with a plantigrade walking pattern. McArdle's disease is characterized by the inability to break down muscle glycogen. It is an autosomal-recessive condition, characterized by low exercise tolerance, muscular atrophy at the shoulder girdle, episodes of myoglobinuria after vigorous physical activities and the occurrence of the second wind phenomenon. The aim of this review is to present the case studies of two subjects who were originally diagnosed as idiopathic toe walkers, but were then found to have McArdle's disease. This review will describe some physical characteristics that distinguish McArdle´s disease from Idiopathic toe walkers.

French consensus. Idiopathic hypersomnia: Investigations and follow-up.

PubMed

Leu-Semenescu, S; Quera-Salva, M-A; Dauvilliers, Y

Idiopathic hypersomnia is a rare, central hypersomnia, recently identified and to date of unknown physiopathology. It is characterised by a more or less permanent, excessive daytime sleepiness, associated with long and unrefreshing naps. Night-time sleep is of good quality, excessive in quantity, associated with sleep inertia in the subtype previously described as "with long sleep time". Diagnosis of idiopathic hypersomnia is complex due to the absence of a quantifiable biomarker, the heterogeneous symptoms, which overlap with the clinical picture of type 2 narcolepsy, and its variable evolution over time. Detailed evaluation enables other frequent causes of somnolence, such as depression or sleep deprivation, to be eliminated. Polysomnography and multiple sleep latency tests (MSLT) are essential to rule out other sleep pathologies and to objectify excessive daytime sleepiness. Sometimes the MSLT do not show excessive sleepiness, hence a continued sleep recording of at least 24hours is necessary to show prolonged sleep (>11h/24h). In this article, we propose recommendations for the work-up to be carried out during diagnosis and follow-up for patients suffering from idiopathic hypersomnia. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

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

PubMed

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

2016-01-01

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

Idiopathic noncirrhotic portal hypertension: current perspectives

PubMed Central

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. PMID:27555800

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

PubMed

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.

Selective Arterial Embolization of Idiopathic Priapism

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

Cohen, Gary S.; Braunstein, Larry; Ball, David S.

1996-11-15

We report a case of idiopathic priapism that was only identified as high-flow or arterial priapism after drainage of the corpora cavernosa. Following failure of conservative and surgical treatment attempts, two consecutive embolizations of a unilateral penile artery were performed with gelgoam particles.

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

PubMed

Kume, Akito; Kume, Hideaki

2010-06-01

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

Significance of Myositis Autoantibody in Patients with Idiopathic Interstitial Lung Disease

PubMed Central

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

2015-01-01

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

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

PubMed

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

2015-05-01

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

Self-diffusion of polycrystalline ice Ih under confining pressure: Hydrogen isotope analysis using 2-D Raman imaging

NASA Astrophysics Data System (ADS)

Noguchi, Naoki; Kubo, Tomoaki; Durham, William B.; Kagi, Hiroyuki; Shimizu, Ichiko

2016-08-01

We have developed a high-resolution technique based on micro Raman spectroscopy to measure hydrogen isotope diffusion profiles in ice Ih. The calibration curve for quantitative analysis of deuterium in ice Ih was constructed using micro Raman spectroscopy. Diffusion experiments using diffusion couples composed of dense polycrystalline H2O and D2O ice were carried out under a gas confining pressure of 100 MPa (to suppress micro-fracturing and pore formation) at temperatures from 235 K to 245 K and diffusion times from 0.2 to 94 hours. Two-dimensional deuterium profiles across the diffusion couples were determined by Raman imaging. The location of small spots of frost from room air could be detected from the shapes of the Raman bands of OH and OD stretching modes, which change because of the effect of the molar ratio of deuterium on the molecular coupling interaction. We emphasize the validity for screening the impurities utilizing the coupling interaction. Some recrystallization and grain boundary migration occurred in recovered diffusion couples, but analysis of two-dimensional diffusion profiles of regions not affected by grain boundary migration allowed us to measure a volume diffusivity for ice at 100 MPa of (2.8 ± 0.4) ×10-3exp[ -57.0 ± 15.4kJ /mol RT ] m2 /s (R is the gas constant, T is temperature). Based on ambient pressure diffusivity measurements by others, this value indicates a high (negative) activation volume for volume diffusivity of -29.5 cm3/mol or more. We can also constrain the value of grain boundary diffusivity in ice at 100 MPa to be <104 that of volume diffusivity.

Glymphatic MRI in idiopathic normal pressure hydrocephalus

PubMed Central

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

2017-01-01

Abstract 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

Glymphatic MRI in idiopathic normal pressure hydrocephalus.

PubMed

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

PubMed Central

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 mastitis, initially mistaken for mastitis as well as breast carcinoma, and successfully treated with a course of corticosteroids. Conclusion There is no clear clinical consensus regarding the ideal therapeutic management of idiopathic granulomatous mastitis. Treatment options include expectant management with spontaneous remission, corticosteroid therapy, immunosuppressive agents and extensive surgery for refractory cases. PMID:17662130

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

PubMed

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

2016-04-01

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

Idiopathic gingival fibromatosis rehabilitation: a case report with two-year followup.

PubMed

Jayachandran, Mahesh; Kapoor, Shalini; Mahesh, Rethi

2013-01-01

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.

Treatment of idiopathic FSGS with adrenocorticotropic hormone gel.

PubMed

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

2013-12-01

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

[Biologic therapy in idiopathic inflammatory myopathy].

PubMed

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

2014-09-15

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

Bilateral versus single lung transplant for idiopathic pulmonary fibrosis.

PubMed

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

2014-10-01

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

On the phase diagram of water with density functional theory potentials: the melting temperature of Ice I-h with the Perdew-Burke-Ernzerhof and Becke-Lee-Yang-Parr functionals

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

Yoo, Soohaeng; Zeng, Xiao Cheng; Xantheas, Sotiris S.

2009-06-11

The melting temperature (Tm) of ice Ih was determined from constant enthalphy (NPH) Born-Oppenheimer Molecular Dynamics (BOMD) simulations to be 417±3 K for the Perdew-Burke-Ernzerhof (PBE) and 411±4 K for the Becke-Lee-Yang-Parr (BLYP) density functionals using a coexisting ice (Ih)-liquid phase at constant pressures of P = 2,500 and 10,000 bar and a density ρ = 1 g/cm3, respectively. This suggests that ambient condition simulations at ρ = 1 g/cm3 will rather describe a supercooled state that is overstructured when compared to liquid water. This work was supported by the US Department of Energy Office of Basic Energy Sciences' Chemicalmore » Sciences program. Pacific Northwest National Laboratory is operated by Battelle for the US Department of Energy.« less

Update on diagnosis and treatment of idiopathic pulmonary fibrosis

PubMed Central

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

Ethinylestradiol/Chlormadinone acetate for use in dermatological disorders.

PubMed

Gómez Vázquez, Mercedes; Navarra Amayuelas, Ramón; Lamarca, Marta; Baquedano, Laura; Romero Ruiz, Sebastián; Vilar-Checa, Eduardo; Iniesta, Maria D

2011-09-06

The diagnosis and management of four cases of dermatological disorders, most of which are related to the endocrine disorder of androgen excess, are presented. Combined oral contraceptives (COCs) may be useful when well-tolerated hormonal therapy and/or when contraception is required. A female patient with androgenetic alopecia or female pattern balding, without underlying hyperandrogenism, was treated with ethinylestradiol/chlormadinone acetate (EE/CMA) 0.03 mg/2 mg for 6 months and experienced stabilization of hair loss (case report 1). A patient who had previously received a COC for an irregular menstrual pattern but again experienced irregular menses and also acne after stopping treatment was diagnosed with acne associated with polycystic ovary syndrome (PCOS) [case report 2]. After 6 month's treatment with EE/CMA 0.03 mg/2 mg, this patient had fewer acne lesions and became eumenorrheic. A third patient who had excess hair since childhood was diagnosed with idiopathic hirsutism (no underlying gynecological or endocrinological disorder was found) and was treated with EE/CMA 0.03 mg/2 mg (case report 3). Less hair growth was reported after 6 months' treatment. Case report 4 describes a patient who presented with oligomenorrhea and acne. She was diagnosed with PCOS with acne, seborrhea and mild hirsutism. Treatment with EE/CMA 0.03 mg/2 mg for 6 months resulted in improvements in her facial acne, seborrhea and hirsutism; she also became eumenorrheic. These four cases illustrate that EE/CMA may be a useful and well tolerated treatment option in the management of patients with dermatological disorders with or without hyperandrogenization.

Epidemiology of polycystic ovary syndrome: a cross sectional study of university students at An-Najah national university-Palestine.

PubMed

Musmar, Samar; Afaneh, Asma; Mo'alla, Hafsa

2013-05-20

Polycystic Ovary Syndrome (PCOS) is the most common gynecological endocrinopathy in women of reproductive age. Despite its heavy burden on female reproduction and general health, there is no study regarding PCOS prevalence in Palestine. This study aims to establish prevalence of PCOS among female university students at An-Najah National University-Palestine and to explore its possible risk factors. A cross sectional study was conducted on 137 female students using convenience sampling method for age group (18-24) years. PCOS cases were identified according to the National Institute of health (NIH) criteria through clinical interview and assessment for participants at the University clinics. Menstrual irregularities regarding cycle and flow were identified and clinical hyperandrogenism was assessed as the self-reported degree of hirsutism using the modified Ferriman Gallwey (mF-G) scoring method of more than 8 score. Biochemical hyperandrogenism for girls with menstrual irregularities was assessed by measuring free testosterone level. Data were analyzed using SPSS version 17 applying descriptive methods; different risk factor relationships were estimated using bivariate analysis and multivariate logistic regression. The estimated prevalence of PCOS was 7.3%, acne was the only studied risk factor among others to be statistically significantly related to PCOS patients (OR=8.430, P-value=0.015). Clinical Hirsutism was found in 27% of participants, 70% of whom had idiopathic hirsutism. Prevalence of PCOS in Palestine seems to be relatively high but similar to other Mediterranean statistics. We recommend further studies using wider age group and larger sample for all parts of Palestine in order to generalize results.

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

PubMed

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

1997-08-01

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

Idiopathic Chronic Parotitis: Imaging Findings and Sialendoscopic Response.

PubMed

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

2015-01-01

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

[Linkage analysis in an extended multigenerational family segregating for idiopathic epilepsy].

PubMed

Palacio, L G; Sánchez, J L; Jiménez, M E; Rivera-Valencia, D; Jiménez-Ramírez, I; Arcos, O M

Linkage analyses enable us to identify the loci that bestow susceptibility to certain diseases which are assumed to have a genetic aetiology by determining the cosegregation of alleles of specific markers within families. The aim of this study was to determine whether there is generalised idiopathic epilepsy (GIE) susceptibility in the 8q22.1 -q24.23, 16p13.3 and 21q22.3 regions within an extended multigenerational family belonging to the Paisa community in Antioquia, a genetic isolate located in Colombia segregating for GIE with a strong capacity for detecting linkage. A family with a number of individuals affected by idiopathic epilepsy who visited the Instituto Neurológico de Antioquia was selected for study. An affected individual was required to have been diagnosed by a neurologist as suffering from non-myoclonic idiopathic epilepsy or partial idiopathic epilepsy. All patients suspected of suffering from idiopathic epilepsy were submitted to video monitoring in order to characterise the seizures electroencephalographically. Of the 106 individuals in this family that were included in the family tree, 76 were genotyped, 15 of whom were affected by generalised clonic tonic seizures and six were considered to be possibly affected. Results of the lod score were significantly negative for all the markers in relation to each model that was considered. The possibility of the genes located in the 8q22.1 -q24.23, 16p13.3 and 21q22.3 regions being responsible for the familial aggregation of GIE in this family was ruled out, which is in accordance with claims made in previous studies conducted on other families.

"Idiopathic" mental retardation and new chromosomal abnormalities

PubMed Central

2010-01-01

Mental retardation is a heterogeneous condition, affecting 1-3% of general population. In the last few years, several emerging clinical entities have been described, due to the advent of newest genetic techniques, such as array Comparative Genomic Hybridization. The detection of cryptic microdeletion/microduplication abnormalities has allowed genotype-phenotype correlations, delineating recognizable syndromic conditions that are herein reviewed. With the aim to provide to Paediatricians a combined clinical and genetic approach to the child with cognitive impairment, a practical diagnostic algorithm is also illustrated. The use of microarray platforms has further reduced the percentage of "idiopathic" forms of mental retardation, previously accounted for about half of total cases. We discussed the putative pathways at the basis of remaining "pure idiopathic" forms of mental retardation, highlighting possible environmental and epigenetic mechanisms as causes of altered cognition. PMID:20152051

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

PubMed

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

2012-05-01

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

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

PubMed

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

2015-05-01

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

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

PubMed

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

2017-09-01

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

Hypercalciuric Bone Disease

NASA Astrophysics Data System (ADS)

Favus, Murray J.

2008-09-01

Hypercalciuria plays an important causal role in many patients with calcium oxalate (CaOx) stones. The source of the hypercalciuria includes increased intestinal Ca absorption and decreased renal tubule Ca reabsorption. In CaOx stone formers with idiopathic hypercalciuria (IH), Ca metabolic balance studies have revealed negative Ca balance and persistent hypercalciuria in the fasting state and during low dietary Ca intake. Bone resorption may also contribute to the high urine Ca excretion and increase the risk of bone loss. Indeed, low bone mass by DEXA scanning has been discovered in many IH patients. Thiazide diuretic agents reduce urine Ca excretion and may increase bone mineral density (BMD), thereby reducing fracture risk. Dietary Ca restriction that has been used unsuccessfully in the treatment of CaOx nephrolithiasis in the past may enhance negative Ca balance and accelerate bone loss. DEXA scans may demonstrate low BMD at the spine, hip, or forearm, with no predictable pattern. The unique pattern of bone histologic changes in IH differs from other causes of low DEXA bone density including postmenopausal osteoporosis, male hypogonadal osteoporosis, and glucocorticoid-induced osteoporosis. Hypercalciuria appears to play an important pathologic role in the development of low bone mass, and therefore correction of urine Ca losses should be a primary target for treatment of the bone disease accompanying IH.

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

PubMed

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

2016-03-18

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

Treatment of Refractory Idiopathic Supraorbital Neuralgia Using Percutaneous Pulsed Radiofrequency.

PubMed

Luo, Fang; Lu, Jingjing; Ji, Nan

2018-02-26

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

Different fates of excessive daytime sleepiness: survival analysis for remission.

PubMed

Kim, T; Lee, J H; Lee, C S; Yoon, I Y

2016-07-01

Excessive daytime sleepiness (EDS) is a symptom frequently presented in sleep clinics. Only a paucity of data has addressed clinical courses of sleep disorders with EDS. Therefore, we sought to compare clinical outcomes of patients presenting EDS. A retrospective observational study was performed in the setting of sleep laboratory and outpatient department in a university hospital. One hundred and eight patients who presented EDS underwent polysomnography and multiple sleep latency test. Each patient was diagnosed as one of the following four categories: (1) narcolepsy with cataplexy (N + C; n = 29); (2) narcolepsy without cataplexy (N - C; n = 22); (3) idiopathic hypersomnia (IH; n = 24); and (4) subjective hypersomnolence (SH; n = 33) with mean sleep latency >8 min. Remission of EDS and treatment response were determined based on clinical evaluation. Kaplan-Meier survival analysis was performed. Remission rates were significantly different (P < 0.001, overall log-rank test) among four groups except those between N - C and IH (P = 0.489). While N + C showed no remission, predicted remission rates of N - C and IH group were 44.6% at 5 years and 32.5% at 5.5 years after diagnosis. The predicted remission rate of SH group was 71.7% at 3 years after diagnosis. The similarity of clinical courses between N - C and IH suggests that N - C may be more related to IH compared to N + C. Considering different clinical courses among EDS patients, thorough evaluation of EDS should be warranted before starting treatment. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

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

PubMed

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) (p<0.0001), working memory deficits (p<0.003), auditory verbal long-term memory deficits (p<0.0021), and more frequent psychosocial symptoms (p<0.0001). The severity of auditory verbal memory 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.

Familial Idiopathic Cranial Neuropathy in a Chinese Family.

PubMed

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.

Compatibility of amino acids in ice Ih and high-pressure phases: implications for the origin of life

NASA Astrophysics Data System (ADS)

Hao, J.; Giovenco, E.; Pedreira-Segade, U.; Montagnac, G.; Daniel, I.

2017-12-01

Icy environments may have been common on the early Earth due to the faint young sun. Previous studies have proposed that the formation of large icy bodies in the early ocean could concentrate the building blocks of life in eutectic fluids and therefore facilitate the polymerization of monomers. This hypothesis is based on the untested assumption that organic molecules are virtually incompatible in ice Ih. In this study, we conducted freezing experiments to explore the partitioning behavior of selected amino acids (glycine, L-alanine, L-proline, and L-phenylalanine) between ice Ih and aqueous solutions analogous to seawater. We let ice crystals grow slowly from a few seeds in equilibrium with the solution and used Raman spectroscopy to analyze in situ the relative concentrations of amino acids in the ice and aqueous solution. During freezing, there was no precipitation of amino acid crystals, indicating that the concentrations in solution never reached their solubility limit, even when the droplet was mostly frozen. Analyses of the Raman spectra of ice and eutectic solution showed that considerable amounts of amino acids existed in the ice phase with partition coefficients ranging between 0.2 and 0.5. This study also explored the partitioning of amino acids between other phases of ice (ice VI and ice VII) and solutions at high pressures and observed similar results. These observations implied little incompatibility of amino acids in ice during the freezing of the solutions, rendering the hypothesis of a cold origin of life unwarranted. However, incorporation into ice could significantly improve the efficiency of extraterrestrial transport of small organics. Therefore, this study supports the hypothesis of extraterrestrial delivery of organic molecules in the icy comets and asteroids to the primitive Earth as suggested by an increasing number of independent observations.

Idiopathic pulmonary fibrosis misdiagnosed as sputum-negative pulmonary tuberculosis.

PubMed

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.

Physical examination in adolescent idiopathic scoliosis.

PubMed

Diab, Mohammad

2007-04-01

The following distinguish the physical examination in scoliosis: it is extensive, it is revealing, and it influences treatment. Throughout this discussion, reference frequently is made to evaluation for underlying neural disease. Idiopathic scoliosis is a diagnosis of exclusion, and a neural etiology of spinal deformity must be ruled out in every case.

40 CFR 721.10271 - 3′H-Cyclopropa[1,9][5,6]fullerene-C60-Ih-3′-butanoic acid, 3′-phenyl-, methyl ester.

Code of Federal Regulations, 2013 CFR

2013-07-01

... CHEMICAL SUBSTANCES Significant New Uses for Specific Chemical Substances § 721.10271 3′H-Cyclopropa[1,9][5,6]fullerene-C60-Ih-3′-butanoic acid, 3′-phenyl-, methyl ester. (a) Chemical substance and significant new uses subject to reporting. (1) The chemical substance identified as 3′H-Cyclopropa[1,9][5,6...

40 CFR 721.10271 - 3′H-Cyclopropa[1,9][5,6]fullerene-C60-Ih-3′-butanoic acid, 3′-phenyl-, methyl ester.

Code of Federal Regulations, 2014 CFR

2014-07-01

... CHEMICAL SUBSTANCES Significant New Uses for Specific Chemical Substances § 721.10271 3′H-Cyclopropa[1,9][5,6]fullerene-C60-Ih-3′-butanoic acid, 3′-phenyl-, methyl ester. (a) Chemical substance and significant new uses subject to reporting. (1) The chemical substance identified as 3′H-Cyclopropa[1,9][5,6...

Idiopathic paraproteinaemia. I. Studies in an animal model--the ageing C57BL/KaLwRij mouse.

PubMed Central

Radl, J; Hollander, C F; van den Berg, P; de Glopper, E

1978-01-01

A search for a suitable animal model for studies on idiopathic paraproteinaemia showed that an age-dependent increase in the appearance of homogeneous immunoglobulins in serum was common to all of the seven mouse strains investigated to date. The highest frequency was found in C57Bl/KaLwRij mice. Further investigations in this strain demonstrated that, except for some quantitative differences, most of the features of human and C57BL Mouse idiopathic paraproteinaemia were essentially the same. No clear-cut correlation was found between the idiopathic paraproteinaemia and, in the old C57B1 mice, a rather frequently occurring reticulum cell sarcoma B and amyloidosis. The mouse idiopathic paraproteinaemia can be regarded as an analogue of the human idiopathic paraproteinaemia and therefore as a suitable model for further experimental studies. PMID:367647

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

PubMed

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

2016-09-01

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.

Prediction of pulmonary hypertension in idiopathic pulmonary fibrosis☆

PubMed Central

Zisman, David A.; Ross, David J.; Belperio, John A.; Saggar, Rajan; Lynch, Joseph P.; Ardehali, Abbas; Karlamangla, Arun S.

2007-01-01

Summary Background Reliable, noninvasive approaches to the diagnosis of pulmonary hypertension in idiopathic pulmonary fibrosis are needed. We tested the hypothesis that the forced vital capacity to diffusing capacity ratio and room air resting pulse oximetry may be combined to predict mean pulmonary artery pressure (MPAP) in idiopathic pulmonary fibrosis. Methods Sixty-one idiopathic pulmonary fibrosis patients with available right-heart catheterization were studied. We regressed measured MPAP as a continuous variable on pulse oximetry (SpO2) and percent predicted forced vital capacity (FVC) to percent-predicted diffusing capacity ratio (% FVC/% DLco) in a multivariable linear regression model. Results Linear regression generated the following equation: MPAP = −11.9+0.272 × SpO2+0.0659 × (100−SpO2)2+3.06 × (% FVC/% DLco); adjusted R2 = 0.55, p<0.0001. The sensitivity, specificity, positive predictive and negative predictive value of model-predicted pulmonary hypertension were 71% (95% confidence interval (CI): 50–89%), 81% (95% CI: 68–92%), 71% (95% CI: 51–87%) and 81% (95% CI: 68–94%). Conclusions A pulmonary hypertension predictor based on room air resting pulse oximetry and FVC to diffusing capacity ratio has a relatively high negative predictive value. However, this model will require external validation before it can be used in clinical practice. PMID:17604151

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

PubMed

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.

Idiopathic Noncirrhotic Portal Hypertension: An Appraisal

PubMed Central

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

Treatment of Idiopathic FSGS with Adrenocorticotropic Hormone Gel

PubMed Central

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

2013-01-01

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

Central Disorders of Hypersomnolence

PubMed Central

Khan, Zeeshan

2015-01-01

The central disorders of hypersomnolence are characterized by severe daytime sleepiness, which is present despite normal quality and timing of nocturnal sleep. Recent reclassification distinguishes three main subtypes: narcolepsy type 1, narcolepsy type 2, and idiopathic hypersomnia (IH), which are the focus of this review. Narcolepsy type 1 results from loss of hypothalamic hypocretin neurons, while the pathophysiology underlying narcolepsy type 2 and IH remains to be fully elucidated. Treatment of all three disorders focuses on the management of sleepiness, with additional treatment of cataplexy in those patients with narcolepsy type 1. Sleepiness can be treated with modafinil/armodafinil or sympathomimetic CNS stimulants, which have been shown to be beneficial in randomized controlled trials of narcolepsy and, quite recently, IH. In those patients with narcolepsy type 1, sodium oxybate is effective for the treatment of both sleepiness and cataplexy. Despite these treatments, there remains a subset of hypersomnolent patients with persistent sleepiness, in whom alternate therapies are needed. Emerging treatments for sleepiness include histamine H3 antagonists (eg, pitolisant) and possibly negative allosteric modulators of the gamma-aminobutyric acid-A receptor (eg, clarithromycin and flumazenil). PMID:26149554

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

PubMed

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

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

PubMed Central

2014-01-01

Background 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. Methods 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. Results 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). Conclusions 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

Recurrent urethral obstruction secondary to idiopathic renal hematuria in a puppy.

PubMed

Hawthorne, J C; deHaan, J J; Goring, R L; Randall, S R; Kennedy, F S; Stone, E; Zimmerman, K M; McAbee, S W

1998-01-01

A seven-month-old, neutered male Catahoula leopard dog cross was presented for recurrent urethral obstruction and intermittent hematuria. After exploratory laparotomy and ventral cystotomy, unilateral idiopathic renal hematuria was diagnosed based on gross observation of hematuria from the left ureteral catheter. The hematuria resolved after nephrectomy of the left kidney. The histopathological diagnosis was multifocal, acute congestion and intratubular hemorrhage. Although idiopathic renal hematuria has been described previously, this puppy was unique because the hematuria caused recurrent, complete urethral obstruction.

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

PubMed

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.

Circulating irisin and chemerin levels as predictors of seizure control in children with idiopathic epilepsy.

PubMed

Elhady, Marwa; Youness, Eman R; Gafar, Heba S; Abdel Aziz, Ali; Mostafa, Rehab S I

2018-06-02

Irisin and chemerin peptides expression are triggered by hypoxia and involved in activation of inflammatory cascades in various organs including the brain; however, their role in epilepsy is not fully illustrated. This study aims to explore the predictive role of irisin and chemerin for seizure control in children with idiopathic epilepsy. This cross-sectional comparative study included 50 children with idiopathic epilepsy; 25 of them had controlled seizures over the previous 6 months and 30 age- and sex-matched healthy children as controls. Epilepsy characteristics, seizure severity Chalfont score, and response to medications were assessed in relation to serum irisin and chemerin levels. In comparison to healthy controls, serum chemerin and irisin levels were significantly higher in children with idiopathic epilepsy especially those with uncontrolled seizures. Serum chemerin and irisin levels had significant positive correlation with seizure severity Chalfont score and the duration of epilepsy. Elevated Chalfont score (OR 3.19), serum chemerin (OR 2.01), and irisin (OR 2.03) are predictors of uncontrolled seizures. Circulating chemerin and irisin have 80% and 76% sensitivity and 88% and 92% specificity at cutoff point > 191.38 ng/ml and > 151.2 ng/ml respectively for prediction of uncontrolled seizures in children with idiopathic epilepsy. Elevated circulating level of irisin and chemerin may predict poor seizure control in children with idiopathic epilepsy suggesting the role of hypoxia-triggered neuroinflammation in the pathogenesis of childhood idiopathic epilepsy.

Gastric mucin expression in Helicobacter pylori-related, nonsteroidal anti-inflammatory drug-related and idiopathic ulcers

PubMed Central

Boltin, Doron; Halpern, Marisa; Levi, Zohar; Vilkin, Alex; Morgenstern, Sara; Ho, Samuel B; Niv, Yaron

2012-01-01

AIM: To determine the pattern of secreted mucin expression in Helicobacter pylori (H. pylori)-related, nonsteroidal anti-inflammatory drug (NSAID)-related and idiopathic gastric ulcers. METHODS: We randomly selected 92 patients with H. pylori-associated (n = 30), NSAID-associated (n = 18), combined H. pylori and NSAID-associated gastric ulcers (n = 24), and patients with idiopathic gastric ulcers (n = 20). Immunohistochemistry for T-cell CD4/CD8, and for mucin 5AC (MUC5AC) and mucin 6 (MUC6), was performed on sections of the mucosa from the ulcer margin. Inflammation score was assessed according to the Sydney system. RESULTS: MUC5AC was expressed on the surface epithelium (98.9%) and neck glands (98.9%) with minimal expression in the deep glands (6.5%). MUC6 was strongly expressed in the deep glands (97.8%), variable in the neck glands (19.6%) and absent in the surface epithelium (0%). The pattern of mucin expression in idiopathic ulcer margins was not different from the expression in ulcers associated with H. pylori, NSAIDs, or combined H. pylori and NSAIDs. CD4/CD8 ratio was higher in H. pylori-positive patients (P = 0.009). Idiopathic ulcers are associated with hospitalized patients and have higher bleeding and mortality rates. CONCLUSION: Idiopathic ulcers have a unique clinical profile. Gastric mucin expression in idiopathic gastric ulcers is unchanged compared with H. pylori and/or NSAID-associated ulcers. PMID:22969235

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

PubMed

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

1998-09-01

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

Idiopathic Paroxysmal Ventricular Tachycardia in Infants and Children

ERIC Educational Resources Information Center

Hernandez, Antonio; And Others

1975-01-01

Laboratory tests including blood count serum electrolyte measures, and electroencephalograms were performed on seven children ages 1 day to 18 years with recurrent attacks of rapid heart action known as idiopathic paroxysmal ventricular tachycardia. (CL)

Diagnosis of narcolepsy and idiopathic hypersomnia. An update based on the International classification of sleep disorders, 2nd edition.

PubMed

Billiard, Michel

2007-10-01

Defining the precise nosological limits of narcolepsy and idiopathic hypersomnia is an ongoing process dating back to the first description of the two conditions. The most recent step forward has been done within the preparation of the second edition of the "International classification of sleep disorders" published in June 2005. Appointed by Dr Emmanuel Mignot, the Task Force on "Hypersomnias of central origin, not due to a circadian rhythm sleep disorder, sleep related breathing disorder, or other causes of disturbed nocturnal sleep" thoroughly revisited the nosology of narcolepsy and of idiopathic hypersomnia. Narcolepsy is now distinguished into three different entities, narcolepsy with cataplexy, narcolepsy without cataplexy and narcolepsy due to medical condition, and idiopathic hypersomnia into two entities, idiopathic hypersomnia with long sleep time and idiopathic hypersomnia without long sleep time. Nevertheless there are still a number of pending issues. What are the limits of narcolepsy without cataplexy? Is there a continuum in the pathophysiology of narcolepsy with and without cataplexy? Should sporadic and familial forms of narcolepsy with cataplexy appear as subgroups in the classification? Are idiopathic hypersomnia with long sleep time and idiopathic hypersomnia without long sleep time, two forms of the same condition or two different conditions? Is there a pathophysiological relationship between narcolepsy without cataplexy and idiopathic hypersomnia without long sleep time?

Assessment of sensorimotor control in adults with surgical correction for idiopathic scoliosis.

PubMed

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

2016-10-01

This study aims at verifying if impaired sensorimotor control observed in adolescents and young adults with scoliosis is also present in adult patients who underwent surgery to reduce their spine deformation. The study included ten healthy adults and ten adults with idiopathic scoliosis who underwent surgery to reduce their spine deformation. Galvanic vestibular stimulation was delivered to assess sensorimotor control. Vertical forces under each foot and horizontal displacement of the upper body were measured before, during and after stimulation. Balance control was assessed by calculating the root mean square values of kinematic and kinetic variables. The amplitude of the vestibular-evoked postural response was 3.4 % (0.8-6.0 %) and 4.5 % (-0.4 to 9.5 %) of the maximal range of motion. Therefore, spine surgery did not limit the postural response. Patients with idiopathic scoliosis exhibited larger body sway than the healthy controls during and immediately after vestibular stimulation. The maximal normalized lateral displacement of the body was 0.85 and 0.40 cm/m and maximal normalized vertical force was 0.78 vs. 0.39 N/kg, for idiopathic scoliosis and healthy groups, respectively. This result suggests that dysfunctional sensorimotor integration is still present even in adult idiopathic scoliosis that underwent spine deformation correction.

Idiopathic slow transit constipation and megacolon are not associated with neurturin mutations.

PubMed

Chen, B; Knowles, C H; Scott, M; Anand, P; Williams, N S; Milbrandt, J; Tam, P K H

2002-10-01

Chronic idiopathic slow-transit constipation (ISTC) and idiopathic megacolon (IMC) are early-onset gastrointestinal motility disorders of unknown aetiology. The gene encoding the neurotrophic factor neurturin may be a candidate for these disorders, as neurturin-deficient mice have a similar enteric phenotype. In the present study, we tested this hypothesis. Genomic DNA from 26 cases of chronic idiopathic STC [with a family history of constipation in 15 (58%) and Hirschsprung's disease in two (8%)], and five cases of IMC [two familial (40%)] was screened by direct DNA sequencing using the fluorescent dideoxy terminator method. Results were compared with published sequence data and 24 control DNAs. Our results revealed several previously unreported common sequence polymorphisms, but overall frequencies were comparable between patients and controls. We conclude that mutation of neurturin is not a frequent cause of ISTC or IMC.

[Juvenile idiopathic arthritis with dry synovitis: clinical case and review of literature].

PubMed

Dias, Bruno Leonardo Scofano; Imamura, Erica Ueno; Izumi, Ana Paula; Pinheiro, Lúcia Virgínia de Melo; Borigato, Eliana Valverde Magro

2009-01-01

Juvenile idiopathic arthritis is a term that encompasses all forms of arthritis that begin before the age of 16 years, persist for more than 6 weeks and are of unknown cause. Dry synovitis is still not completely understood nor commonly described. It is associated with juvenile idiopathic arthritis and must be considered in patients with minimal swelling but pain and stiffness along with flexion contractures as well as other evidence of an inflammatory process (lab changes and/or other symptoms, such as uveitis or rash), and often follow a destructive course. The authors present a case of a brazilian child with a rheumatoid factor- negative polyarthritis compatible with the subtype dry synovitis, who had great clinical and functional improvement after participation in rehabilitation activities and beginning of pharmacological treatment usually used in Juvenile idiopathic arthritis, including immunossuppressive therapy.

Inhibition of ileal bile acid transporter: An emerging therapeutic strategy for chronic idiopathic constipation.

PubMed

Mosińska, Paula; Fichna, Jakub; Storr, Martin

2015-06-28

Chronic idiopathic constipation is a common disorder of the gastrointestinal tract that encompasses a wide profile of symptoms. Current treatment options for chronic idiopathic constipation are of limited value; therefore, a novel strategy is necessary with an increased effectiveness and safety. Recently, the inhibition of the ileal bile acid transporter has become a promising target for constipation-associated diseases. Enhanced delivery of bile acids into the colon achieves an accelerated colonic transit, increased stool frequency, and relief of constipation-related symptoms. This article provides insight into the mechanism of action of ileal bile acid transporter inhibitors and discusses their potential clinical use for pharmacotherapy of constipation in chronic idiopathic constipation.

Is There a Rationale behind Pharmacotherapy in Idiopathic Gynecomastia?

PubMed

Kasielska-Trojan, Anna; Danilewicz, Marian; Antoszewski, Bogusław

2018-05-17

The aim of this research was to analyze digit ratio in relation to estrogen receptor (ER) and progesterone receptor (PR) expression and to verify digit ratio (2D: 4D) as a marker of ER and PR overexpression in the male breast. This study included 35 patients who underwent breast reduction due to the idiopathic form of gynecomastia. The average age of the studied individuals was 25.7 years (SD = 7.8). ER and PR expression was detected in breasts, and digit ratios were calculated in patients with idiopathic gynecomastia. ER expression did not correlate with the right (p = 0.51) and left 2D: 4D (p = 0.97). Also, there was no correlation between PR expression and 2D: 4D. A lack of correlation between these variables may result from the fact that the analyzed group of men with idiopathic gynecomastia was small in number, but at the same time, it appeared to be homogenous in these aspects (positive ER and/or PR expression and high digit ratio). High digit ratio in men with gynecomastia may tend to be a marker of overexpression of ER and PR. This may justify an early use of tamoxifen in men with gynecomastia and a high digit ratio. © 2018 S. Karger AG, Basel.

Adolescent idiopathic scoliosis: Indications and efficacy of nonoperative treatment

PubMed Central

Canavese, Federico; Kaelin, André

2011-01-01

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. PMID:21221217

Adults With Idiopathic Scoliosis Diagnosed at Youth Experience Similar Physical Activity and Fracture Rate as Controls.

PubMed

Diarbakerli, Elias; Grauers, Anna; Danielsson, Aina; Gerdhem, Paul

2017-04-01

Cross-sectional. To describe physical activity level and fracture rates in adults with idiopathic scoliosis, diagnosed before maturity, and to compare with a control group. A previous study found a lower level of sporting activities in adults treated for idiopathic scoliosis compared with controls. Other studies have shown a lower bone mass in adults with idiopathic scoliosis compared with controls. One thousand two hundred seventy-eight adults (aged 18-71 yr) with idiopathic scoliosis and 214 controls (aged 18-70 yr) were included and answered the International Physical Activity Questionnaire - Short Form (IPAQ-SF) and questions about previous fractures. The three scoliosis treatment groups (untreated n = 360, brace n = 460, and surgically treated n = 458) were compared. Furthermore, a comparison based on onset (juvenile n = 169 or adolescent n = 976) was performed. Achieved weekly moderate activity level and metabolic equivalent task (MET) minutes/week were assessed for patients and controls. Statistical comparisons were made with analysis of covariance with adjustments for age, body mass index, and sex. The proportion achieving weekly moderate activity level was 962 out of 1278 for individuals with idiopathic scoliosis (75%) and 157 out of 214 (73%) for controls (P = 0.40). The scoliosis patients reported 2016 MET-minutes/week (median value) and the controls 2456 (P = 0.06). Fracture rates did not differ (P = 0.72). Fewer surgically treated individuals achieved moderate activity level (P = 0.046) compared with the untreated and the previously braced individuals. No difference was seen regarding MET-minutes/week (P = 0.86). No differences were seen between individuals with a juvenile onset compared with individuals with an adolescent onset (all P ≥ 0.05). Adults with idiopathic scoliosis have similar physical activity level and do not sustain more fractures compared with controls. Adults with surgically treated

The influence of thermoplastic thoraco lumbo sacral orthoses on standing balance in subjects with idiopathic scoliosis.

PubMed

Khanal, Minoo; Arazpour, Mokhtar; Bahramizadeh, Mahmood; Samadian, Mohammad; Hutchins, Stephen W; Kashani, Reza Vahab; Mardani, Mohammad A; Tari, Hossein Vahid; Aboutorabi, Atefeh; Curran, Sarah; Sadeghi, Heidar

2016-08-01

Idiopathic scoliosis patients have postural equilibrium problems. The objective of this study was to assess postural control in subjects with idiopathic scoliosis following a 4-month intervention in an unbraced position. Quasi-experimental. Eight healthy girls and eight girls with idiopathic scoliosis took part. A Kistler force platform was used with a frequency of 100 Hz for recording data. The center of pressure was recorded in different positions out of brace for scoliosis and healthy subjects. Test conditions were single limb and double limb stance, with eyes open and closed, and foam and rigid surfaces. The data reflected a weak balance of idiopathic scoliosis subjects compared to healthy subjects. After 1 and 4 months of wearing the brace, center of pressure and center of gravity sway increased in the majority of the tests, although there were no significant differences in any of the test conditions (p > 0.05). While the center of pressure sway in medio-lateral direction decreased after 4 months of wearing a brace, in other variables center of pressure and center of gravity sway increased. Idiopathic scoliosis patients have weak balance in comparison to healthy subjects. In addition, following a period of 4 months of wearing a brace, balance parameters in the scoliosis subjects did not improve. The results show that we need more follow-up of orthoses wearing in idiopathic scoliosis subjects and suggest more studies at least 1-year follow-up to identify the efficiency of brace wear on balance. Scoliosis can alter postural stability and balance performance during quiet standing. Spinal deformity can alter a subject's ability to compensate for postural changes and cause gait deviations. This study investigated balance differences between the healthy and idiopathic scoliosis patients and the results of thoraco lumbo sacral orthosis brace wear. It might provide some new insight into the conservative treatment of idiopathic scoliosis patients for

Towards a new taxonomy of idiopathic orofacial pain.

PubMed

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.

Lack of evidence for central sensitization in idiopathic, non-traumatic neck pain: a systematic review.

PubMed

Malfliet, Annaleen; Kregel, Jeroen; Cagnie, Barbara; Kuipers, Mandy; Dolphens, Mieke; Roussel, Nathalie; Meeus, Mira; Danneels, Lieven; Bramer, Wichor M; Nijs, Jo

2015-01-01

Chronic neck pain is a common problem with a poorly understood pathophysiology. Often no underlying structural pathology can be found and radiological imaging findings are more related to age than to a patient's symptoms. Besides its common occurrence, chronic idiopathic neck pain is also very disabling with almost 50% of all neck pain patients showing moderate disability at long-term follow-up. Central sensitization (CS) is defined as "an amplification of neural signaling within the central nervous system that elicits pain hypersensitivity," "increased responsiveness of nociceptive neurons in the central nervous system to their normal or subthreshold afferent input," or "an augmentation of responsiveness of central neurons to input from unimodal and polymodal receptors." There is increasing evidence for involvement of CS in many chronic pain conditions. Within the area of chronic idiopathic neck pain, there is consistent evidence for the presence and clinical importance of CS in patients with traumatic neck pain, or whiplash-associated disorders. However, the majority of chronic idiopathic neck pain patients are unrelated to a traumatic injury, and hence are termed chronic idiopathic non-traumatic neck pain. When comparing whiplash with idiopathic non-traumatic neck pain, indications for different underlying mechanisms are found. The goal of this article was to review the existing scientific literature on the role of CS in patients with chronic idiopathic non-traumatic neck pain. Systematic review. All selected studies were case control studies. A systematic search of existing, relevant literature was performed via the electronic databases Medline, Embase, Web of Science, Cinahl, PubMed, and Google Scholar. All titles and abstracts were checked to identify relevant articles. An article was considered eligible if it met following inclusion criteria: (1) participants had to be human adults (> 18 years) diagnosed with idiopathic non-traumatic chronic (present for at

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

PubMed

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

2014-05-29

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

Sleep apnea, daytime somnolence, and idiopathic dizziness--a novel association.

PubMed

Sowerby, Leigh J; Rotenberg, Brian; Brine, Meggan; George, Charles F P; Parnes, Lorne S

2010-06-01

To determine if an association exists between sleep apnea, daytime somnolence, and chronic idiopathic dizziness. Case-control study of new patients presenting to a tertiary neuro-otologic practice. A total of 46 subjects with idiopathic dizziness (ID), 20 positive controls with dizziness (benign paroxysmal positional vertigo [BPV]), and 69 negative controls with hearing loss (HL) but no dizziness were enrolled. Participants who were patients diagnosed with the above conditions and who met all other inclusion criteria completed a sleep questionnaire and had a complete physical exam and investigations to establish or exclude a neuro-otologic diagnosis. They were subsequently evaluated for risk of symptomatic sleep disturbance based on the Epworth Sleepiness Scale (ESS), the Berlin Questionnaire, and the Multivariable Apnea Risk Index (MAP). Statistical analysis was carried out using SPSS (SPSS Inc., Chicago, IL). There was no significant demographic difference among the groups in terms of age, sex, body mass index, neck size, alcohol consumption, or smoking. Using a cutoff of both 10 and 12 on the ESS, the ID were more likely to have significant daytime somnolence than the HL group, with a likelihood ratio (LR) of 7.8 for the ESS 12 score (P = .021) and 7.1 for the ESS 10 score (P = .029). Using the MAP score, a statistically significant difference between the ID group and both the BPV group (LR 3.99, P = .046) and the HL group (LR 5.46, P = .019) was found. This study suggests that a previously undescribed link between idiopathic dizziness, daytime somnolence, and sleep apnea might exist. Prospective investigation is warranted to determine whether treatment of any sleep issues resolves symptoms of idiopathic dizziness.

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

PubMed

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

2015-08-01

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

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

ERIC Educational Resources Information Center

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…

Precise measurement of dielectric anisotropy in ice Ih at 39 GHz

NASA Astrophysics Data System (ADS)

Matsuoka, Takeshi; Fujita, Shuji; Morishima, Shigenori; Mae, Shinji

1997-03-01

The dielectric permittivities parallel and perpendicular to the c axis (optic axis) of ice Ih were measured using an open resonator at 39 GHz in the temperature range 194-262 K. The dielectric anisotropy in ice at microwave frequencies is important for understanding remote sensing data in polar regions, obtained by ice radar and satellite-born microwave radar and radiometer. The measured samples were natural single-crystal ice collected from Mendenhall Glacier, Alaska. A very precise measurement was achieved by detecting two resonant peaks, one from the ordinary component and the other from the extraordinary component, simultaneously, from one sample. The real part of dielectric anisotropy, Δɛ'=ɛ∥c'-ɛ⊥c', at 39 GHz was 0.0339±0.0007 (1.07%±0.02%) at 252 K and slightly depended on temperature. Reference measurements at 1 MHz using parallel plate electrodes were also carried out. The measured dielectric anisotropy at microwave frequencies agrees very well with the value at 1 MHz. The absolute values of ɛ∥c' and ɛ⊥c' at 39 GHz were, respectively, smaller than those at 1 MHz and the difference was about 0.044 at 252 K. The results suggest that a small dispersion exists between GHz and MHz frequencies, but there is no frequency dependence in the value of anisotropy.

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

PubMed

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.

Acute effects of spinal bracing on scapular kinematics in adolescent idiopathic scoliosis.

PubMed

Gur, Gozde; Turgut, Elif; Ayhan, Cigdem; Baltaci, Gul; Yakut, Yavuz

2017-08-01

Bracing is the most common nonsurgical treatment for adolescent idiopathic scoliosis. Spinal braces affect glenohumeral and scapulothoracic motion because they restrict trunk movements. However, the potential spinal-bracing effects on scapular kinematics are unknown. The present study aimed to investigate the acute effects of spinal bracing on scapular kinematics in adolescent idiopathic scoliosis. Scapular kinematics, including scapular internal/external rotation, posterior/anterior tilting, and downward/upward rotation during scapular plane elevation, were evaluated in 27 in-brace and out-of-brace adolescent idiopathic scoliosis patients with a three-dimensional electromagnetic tracking system. Data on the position and orientation of the scapula at 30°, 60°, 90°, and 120° humerothoracic elevation were used for statistical comparisons. The paired t-test was used to assess the differences between the mean values of in-brace and out-of-brace conditions. The in-brace condition showed significantly increased (P<0.05) scapular anterior tilting and decreased internal rotation in the resting position on the convex and concave sides; increased scapular downward rotation at 120° humerothoracic elevation on the convex side and at 30°, 60°, 90°, and 120° humerothoracic elevation on the concave side; increased scapular anterior tilt at 30°, 60°, 90°, and 120° humerothoracic elevation on the convex and concave sides; and decreased (P<0.05) maximal humerothoracic elevation of the arm. Spinal bracing affects scapular kinematics. Observed changes in scapular kinematics with brace may also affect upper extremity function for adolescents with idiopathic scoliosis. Therefore, clinicians should include assessments of the glenohumeral and scapulothoracic joints when designing rehabilitation protocols for patients with adolescent idiopathic scoliosis. Copyright © 2017 Elsevier Ltd. All rights reserved.

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

PubMed Central

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

2014-01-01

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

Idiopathic granulomatous mastitis: in search of a therapeutic paradigm.

PubMed

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.

Precision Medicine: The New Frontier in Idiopathic Pulmonary Fibrosis.

PubMed

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.

Comorbidity between central disorders of hypersomnolence and immune-based disorders.

PubMed

Barateau, Lucie; Lopez, Régis; Arnulf, Isabelle; Lecendreux, Michel; Franco, Patricia; Drouot, Xavier; Leu-Semenescu, Smaranda; Jaussent, Isabelle; Dauvilliers, Yves

2017-01-03

To assess and compare the frequencies of personal and family history of autoimmune diseases (AID), autoinflammatory disorders (ID), and allergies in a population of patients, adults and children, with narcolepsy type 1 (NT1), narcolepsy type 2 (NT2), and idiopathic hypersomnia (IH), 3 central hypersomnia disorders, and healthy controls. Personal and family history of AID, ID, and allergies were assessed by questionnaire and medical interview in a large cohort of 450 consecutive adult patients (206 NT1, 106 NT2, 138 IH) and 95 pediatric patients (80 NT1) diagnosed according to the third International Classification of Sleep Disorders criteria in national reference centers for narcolepsy in France and 751 controls (700 adults, 51 children) from the general population. Ten adults with NT1 (4.9%) had a comorbid AID vs 3.4% of adult controls, without between-group differences in adjusted models. AID frequency did not differ between children with NT1 and controls. Conversely, compared with controls, AID frequency was higher in adults with NT2 (p = 0.002), whereas ID (p = 0.0002) and allergy (p = 0.003) frequencies were higher in adults with IH. A positive family history of AID was found in the NT1 group and of ID in the IH group. NT1 is not associated with increased risk of comorbid immune disorders, in favor of a potentially unique pathophysiology. Conversely, compared with controls, the frequency of autoimmune diseases was higher in adults with NT2, whereas allergies and autoinflammatory disorders were more common in adults with IH, suggesting an immune dysregulation mechanism in these conditions. © 2016 American Academy of Neurology.

Possible association of FAS and FASLG polymorphisms with the risk of idiopathic azoospermia in southeast Turkey.

PubMed

Balkan, Mahmut; Atar, Murat; Erdal, Mehmet Emin; Rustemoğlu, Aydin; Yildiz, Ismail; Gunesacar, Ramazan; Hatipoğlu, Namık Kemal; Bodakçi, Mehmet Nuri; Ay, Ozlem Izci; Çevik, Kenan

2014-06-01

To investigate the association of the genetic variants of FAS/FASLG cell death pathway genes in male infertility, we genotyped the FAS -670A/G, -1377G/A, and FASLG -124A/G single-nucleotide polymorphisms (SNPs) by real-time polymerase chain reaction in 108 infertile men with idiopathic azoospermia and in 125 proven fertile controls. The distribution of genotypes and alleles for SNPs at FAS -1377G/A and FASLG -124A/G loci were determined not to be statistically different between the case and control groups. However, the genotype frequencies of SNPs, FAS -670AA and FAS -670AG, were found to be significantly different between the case and control groups. Whereas the FAS -670AA genotype might be regarded as a higher predisposition for idiopathic azoospermia, FAS -670AG could be interpreted to mean that this genotype provides protection against idiopathic azoospermia. The study of combined genotype and haplotype frequencies has found statistically significant differences between case and control subjects for some combinations. The AA-GG binary genotype for the FAS670 and FAS1377 loci couple, in particular, may have a high degree of predisposition to idiopathic azoospermia. Our results suggest that FAS -670A/G SNP may be a genetic predisposing factor of idiopathic azoospermia among southeastern Anatolian men. Larger studies are needed to verify these findings. Furthermore, our data indicated a possible linkage between the FAS and FASLG genes and idiopathic azoospermia.

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

PubMed Central

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

2012-01-01

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

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

PubMed

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

2015-10-01

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

Idiopathic granulomatous lobular mastitis.

PubMed

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.

PubMed

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.

A geriatric patient with diffuse idiopathic skeletal hyperostosis

PubMed Central

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

2010-01-01

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

Imaging of juvenile idiopathic arthritis. Part I: Clinical classifications and radiographs

PubMed Central

Matuszewska, Genowefa; Gietka, Piotr; Płaza, Mateusz; Walentowska-Janowicz, Marta

2016-01-01

Juvenile idiopathic arthritis is the most common autoimmune systemic disease of the connective tissue affecting individuals at the developmental age. Radiography is the primary modality employed in the diagnostic imaging in order to identify changes typical of this disease entity and rule out other bone-related pathologies, such as neoplasms, posttraumatic changes, developmental defects and other forms of arthritis. The standard procedure involves the performance of comparative joint radiographs in two planes. Radiographic changes in juvenile idiopathic arthritis are detected in later stages of the disease. Bone structures are assessed in the first place. Radiographs can also indirectly indicate the presence of soft tissue inflammation (i.e. in joint cavities, sheaths and bursae) based on swelling and increased density of the soft tissue as well as dislocation of fat folds. Signs of articular cartilage defects are also seen in radiographs indirectly – based on joint space width changes. The first part of the publication presents the classification of juvenile idiopathic arthritis and discusses its radiographic images. The authors list the affected joints as well as explain the spectrum and specificity of radiographic signs resulting from inflammatory changes overlapping with those caused by the maturation of the skeletal system. Moreover, certain dilemmas associated with the monitoring of the disease are reviewed. The second part of the publication will explain issues associated with ultrasonography and magnetic resonance imaging, which are more and more commonly applied in juvenile idiopathic arthritis for early detection of pathological features as well as the disease complications. PMID:27679726

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

PubMed Central

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

Sensory Impairment and Head Circumference in Fragile X Syndrome, Down Syndrome and Idiopathic Intellectual Disability.

ERIC Educational Resources Information Center

Turk, Jeremy; Patton, Michael

2000-01-01

Eighteen boys with fragile X syndrome were compared with 42 with idiopathic intellectual disability, and 45 with Down syndrome. Boys with Down syndrome had more sensory problems and smaller head circumferences than normal. Head circumferences of boys with fragile X syndrome and with idiopathic intellectual disability were larger than normal.…

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

PubMed Central

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

2012-01-01

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

Subjective symptoms in idiopathic hypersomnia: beyond excessive sleepiness.

PubMed

Vernet, Cyrille; Leu-Semenescu, Smaranda; Buzare, Marie-Annick; Arnulf, Isabelle

2010-12-01

Patients with idiopathic hypersomnia never feel fully alert despite a normal or long sleep night. The spectrum of the symptoms is insufficiently studied. We interviewed 62 consecutive patients with idiopathic hypersomnia (with a mean sleep latency lower than 8 min or a sleep time longer than 11 h) and 50 healthy controls using a questionnaire on sleep, awakening, sleepiness, alertness and cognitive, psychological and functional problems during daily life conditions. Patients slept 3 h more on weekends, holidays and in the sleep unit than on working days. In the morning, the patients needed somebody to wake them, or to be stressed, while routine, light, alarm clocks and motivation were inefficient. Three-quarters of the patients did not feel refreshed after short naps. During the daytime, their alertness was modulated by the same external conditions as controls, but they felt more sedated in darkness, in a quiet environment, when listening to music or conversation. Being hyperactive helped them more than controls to resist sleepiness. They were more frequently evening-type and more alert in the evening than in the morning. The patients were able to focus only for 1 h (versus 4 h in the controls). They complained of attention and memory deficit. Half of them had problems regulating their body temperature and were near-sighted. Mental fatigability, dependence on other people for awakening them, and a reduced benefit from usually alerting conditions (except being hyperactive or stressed) seem to be more specific of the daily problems of patients with idiopathic hypersomnia than daytime sleepiness. © 2010 European Sleep Research Society.

Shuttle Return-to-Flight IH-108 Aerothermal Test at CUBRC - Flow Field Calibration and CFD

NASA Technical Reports Server (NTRS)

Lau, Kei Y.; Holden, M. S.

2011-01-01

This paper discusses one specific aspect of the Shuttle Retrun-To-Flight IH-108 Aerothermal Test at Calspan-University of Buffalo Research Center (CUBRC), the test flow field calibration. It showed the versatility of the CUBRC Large Energy National Shock Tunnel (LENS) II wind tunnel for an aerothermal test with unique and demanding requirements. CFD analyses were used effectively to extend the test range at the low end of the Mach range. It demonstrated how ground test facility and CFD synergy can be utilitzed iteratively to enhance the confidence in the fedility of both tools. It addressed the lingering concerns of the aerothermal community on use of inpulse facility and CFD analysis. At the conclusion of the test program, members from the NASA Marshall (MSFC), CUBRC and USA (United Space Alliance) Consultants (The Grey Beards) were asked to independently verify the flight scaling data generated by Boeing for flight certification of the re-designed external tank (ET) components. The blind test comparison showed very good results.

Cisapride stimulates contraction of idiopathic megacolonic smooth muscle in cats.

PubMed

Hasler, A H; Washabau, R J

1997-01-01

We have previously shown that cisapride, a substituted piperidinyl benzamide, stimulates contraction of healthy feline colonic smooth muscle. The purpose of the present investigation was to determine the effect of cisapride on feline idiopathic megacolonic smooth muscle function. Longitudinal smooth muscle strips from ascending and descending colon were obtained from cats with idiopathic megacolon, suspended in a 1.5 mM Ca(2+)-HEPES buffer solution (37 degrees C, 100% O2, pH 7.4), attached to isometric force transducers, and stretched to optimal muscle length (Lo). Control responses were obtained at each muscle site with acetylcholine (10(-8) to 10(-4) M), substance P (10(-11) to 10(-7) M), or potassium chloride (10 to 80 mM). Muscles were then stimulated with cumulative (10(-9) to 10(-6) M) doses of cisapride in the absence or presence of tetrodotoxin (10(-6) M) and atropine (10(-6) M), or in a 0 calcium HEPES buffer solution. In cats with idiopathic megacolon, cisapride stimulated contractions of longitudinal smooth muscle from both the ascending and the descending colon. Cisapride-induced contractions were similar in magnitude to those induced by substance P and acetylcholine in the ascending colon, but were less than those observed in the descending colon. Cisapride-induced contractions in megacolonic smooth muscle were only partially inhibited by tetrodotoxin and atropine, but were virtually abolished by removal of extracellular calcium. We concluded that cisapride-induced contractions of feline megacolonic smooth muscle are largely smooth muscle mediated and dependent on influx of extracellular calcium. Cisapride-induced contractions in megacolonic smooth muscle are only partially dependent on enteric cholinergic nerves. Thus, cisapride may be useful in the treatment of cats with idiopathic megacolon.

Idiopathic Intracranial Hypertension in Children and Adolescents: An Update.

PubMed

Cleves-Bayon, Catalina

2018-03-01

Idiopathic intracranial hypertension (IIH), previously known as pseudotumor cerebri syndrome (PTC) is a serious neurological disorder that can lead to irreversible visual loss. Predominantly a disorder affecting women in reproductive years, the pediatric population is not spared. In the past few years, the condition has been redefined, due to new accepted values for opening pressure in children and advances in neuroimaging. Emerging techniques in ophthalmology are being increasingly used to monitor disease in these patients. And, although the treatment tools have not changed in several years, important evidence for efficacy for acetazolamide finally came to light in recent years in the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT). This review article provides an overview on recent advances in diagnosis, evaluation and treatment of IIH. © 2017 American Headache Society.

Precision Medicine: The New Frontier in Idiopathic Pulmonary Fibrosis

PubMed Central

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

2016-01-01

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. PMID:26991475

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

PubMed

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.

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

PubMed

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) (P<0.01). Focal segmental glomerulosclerosis (FSGS) was the commonest histopathological condition, seen in 13 (43.3%). Children with overt hypothyroidism (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.

The consequences of idiopathic partial epilepsies in relation to neuropsychological functioning: a closer look at the associated mathematical disability.

PubMed

Sart, Z Hande; Demirbilek, Veysi; Korkmaz, Bariş; Slade, Peter D; Dervent, Ayşin; Townes, Brenda D

2006-03-01

Although the seizure prognosis is mostly favorable in idiopathic partial epilepsies, there is some empirical evidence showing that subtle neuropsychological impairments, with a consequent risk of academic underachievement, are not rare. We investigated neuropsychological functioning including attention, memory, visuomotor ability, and executive functioning with a closer look at the associated mathematical ability in patients with idiopathic partial epilepsies. A battery of age-appropriate, neuropsychological and mathematics achievement tests was administered to 30 participants with idiopathic partial epilepsy [13 children with benign epilepsy with centrotemporal spikes (BECTS), 17 children with idiopathic childhood occipital epilepsies (ICOE)], and to 30 healthy participants matched for age, sex, handedness, and socioeconomic status. Results did not support any impairment in overall neuropsychological functioning in participants with idiopathic partial epilepsies, whereas, isolated deficits did exist. The mean performance of the IPE group was significantly lower than the control group in six out of 12, neuropsychological measures: drawing (p < 0.01), digit span (p < 0.05), verbal learning (p < 0.01), object assembly (p < 0.01), similarities (p < 0.05), and vocabulary (p < 0.001). Results suggested that one should be cautious regarding neuropsychological and academic prognosis in the so-called benign idiopathic partial epilepsies of childhood.

SOLITARY IDIOPATHIC CHOROIDITIS IN THE SETTING OF EXTENSIVE ANIMAL EXPOSURE.

PubMed

Kumar, Vivek; Khoo, Chloe T L; Shields, Carol L

2016-01-01

To describe solitary idiopathic choroiditis in the setting of extensive animal exposure. A 56-year-old asymptomatic female equestrian with an extensive history of exposure to horses and dogs and a trapper of wild animals and rodents was discovered to have an amelanotic choroidal mass in the macular region and referred for suspicious atypical nevus. Funduscopy revealed a deep yellow mass with overlying retinal pigment epithelial thinning and without visible subretinal fluid or lipofuscin. Mild hyperautofluorescence represented unmasking of scleral autofluorescence. Ultrasonography showed a 1.8-mm-thick echodense lesion. Enhanced depth imaging-optical coherence tomography disclosed a dense, elevated scleral mass with "volcanic" configuration, demonstrating choroidal compression and trace overlying subretinal fluid. These features were consistent with solitary idiopathic choroiditis/scleritis. Systemic evaluation for standard cat-related bartonellosis, tuberculosis, sarcoidosis, and syphilis were negative. Horse-, dog-, and rodent-related bartonellosis testing was not available. Observation was advised, and the findings remained stable at 6 months. Solitary idiopathic choroiditis is best imaged on enhanced depth imaging-optical coherence tomography as a scleral lesion with "volcanic" configuration and often secondary to previous Bartonella infection. Serologic positivity for cat-related Bartonella decays over time, and testing for horse-, dog-, or rodent-related Bartonella is not commonly used.

[Effects of temporal lobe epilepsy and idiopathic epilepsy on cognitive function and emotion in children].

PubMed

Yang, Xiao-Yan; Long, Li-Li; Xiao, Bo

2016-07-01

To investigate the effects of temporal lobe epilepsy and idiopathic epilepsy on cognitive function and emotion in children and the risk factors for cognitive impairment. A retrospective analysis was performed for the clinical data of 38 children with temporal lobe epilepsy and 40 children with idiopathic epilepsy. The controls were 42 healthy children. All subjects received the following neuropsychological tests: Montreal Cognitive Assessment (MoCA) scale, verbal fluency test, digit span test, block design test, Social Anxiety Scale for Children (SASC), and Depression Self-rating Scale for Children (DSRSC). Compared with the control group, the temporal lobe epilepsy and idiopathic epilepsy groups showed significantly lower scores of MoCA, verbal fluency, digit span, and block design (P<0.05) and significantly higher scores on SASC and DSRSC (P<0.05). Compared with the idiopathic epilepsy group, the temporal lobe epilepsy group showed significantly lower scores of MoCA, verbal fluency, digit span, and block design (P<0.05) and significantly higher scores on SASC and DSRSC (P<0.05). In the temporal lobe epilepsy group, MoCA score was negatively correlated with SASC score, DSRSC score, and seizure frequency (r=-0.571, -0.529, and -0.545 respectively; P<0.01). In the idiopathic epilepsy group, MoCA score was also negatively correlated with SASC score, DSRSC score, and seizure frequency (r=-0.542, -0.487, and -0.555 respectively; P<0.01). Children with temporal lobe epilepsy and idiopathic epilepsy show impaired whole cognition, verbal fluency, memory, and executive function and have anxiety and depression, which are more significant in children with temporal lobe epilepsy. High levels of anxiety, depression, and seizure frequency are risk factors for impaired cognitive function.

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

PubMed

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 < 0.05). No significant differences were observed for other measurements between the groups; however, trends toward greater improvement were observed in the stabilization group. Core stabilization training in addition to traditional exercises was more effective than traditional exercises alone in the correction of vertebral rotation and reduction of pain in 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.

Pediatric and adult vision restoration after optic nerve sheath decompression for idiopathic intracranial hypertension.

PubMed

Bersani, Thomas A; Meeker, Austin R; Sismanis, Dimitrios N; Carruth, Bryant P

2016-06-01

To compare presentations of idiopathic intracranial hypertension and efficacy of optic nerve sheath decompression between adult and pediatric patients, a retrospective cohort study was completed All idiopathic intracranial hypertension patients undergoing optic nerve sheath decompression by one surgeon between 1991 and 2012 were included. Pre-operative and post-operative visual fields, visual acuity, color vision, and optic nerve appearance were compared between adult and pediatric (<18 years) populations. Outcome measures included percentage of patients with complications or requiring subsequent interventions. Thirty-one adults (46 eyes) and eleven pediatric patients (18 eyes) underwent optic nerve sheath decompression for vision loss from idiopathic intracranial hypertension. Mean deviation on visual field, visual acuity, color vision, and optic nerve appearance significantly improved across all subjects. Pre-operative mean deviation was significantly worse in children compared to adults (p=0.043); there was no difference in mean deviation post-operatively (p=0.838). Significantly more pediatric eyes (6) presented with light perception only or no light perception than adult eyes (0) (p=0.001). Pre-operative color vision performance in children (19%) was significantly worse than in adults (46%) (p=0.026). Percentage of patients with complications or requiring subsequent interventions did not differ between groups. The consistent improvement after surgery and low rate of complications suggest optic nerve sheath decompression is safe and effective in managing vision loss due to adult and pediatric idiopathic intracranial hypertension. Given the advanced pre-operative visual deficits seen in children, one might consider a higher index of suspicion in diagnosing, and earlier surgical intervention in treating pediatric idiopathic intracranial hypertension.

Effect of pirfenidone on mortality: pooled analyses and meta-analyses of clinical trials in idiopathic pulmonary fibrosis.

PubMed

Nathan, Steven D; Albera, Carlo; Bradford, Williamson Z; Costabel, Ulrich; Glaspole, Ian; Glassberg, Marilyn K; Kardatzke, David R; Daigl, Monica; Kirchgaessler, Klaus-Uwe; Lancaster, Lisa H; Lederer, David J; Pereira, Carlos A; Swigris, Jeffrey J; Valeyre, Dominique; Noble, Paul W

2017-01-01

In clinical trials of idiopathic pulmonary fibrosis, rates of all-cause mortality are low. Thus prospective mortality trials are logistically very challenging, justifying the use of pooled analyses or meta-analyses. We did pooled analyses and meta-analyses of clinical trials of pirfenidone versus placebo to determine the effect of pirfenidone on mortality outcomes over 120 weeks. We did a pooled analysis of the combined patient populations of the three global randomised phase 3 trials of pirfenidone versus placebo-Clinical Studies Assessing Pirfenidone in Idiopathic Pulmonary Fibrosis: Research of Efficacy and Safety Outcomes (CAPACITY 004 and 006; trial durations 72-120 weeks) and Assessment of Pirfenidone to Confirm Efficacy and Safety in Idiopathic Pulmonary Fibrosis (ASCEND 016; 52 weeks)-for all-cause mortality, treatment-emergent all-cause mortality, idiopathic-pulmonary-fibrosis-related mortality, and treatment-emergent idiopathic-pulmonary-fibrosis-related mortality at weeks 52, 72, and 120. We also did meta-analyses of these data and data from two Japanese trials of pirfenidone versus placebo-Shionogi Phase 2 (SP2) and Shionogi Phase 3 (SP3; trial durations 36-52 weeks). At week 52, the relative risk of death for all four mortality outcomes was significantly lower in the pirfenidone group than in the placebo group in the pooled population (all-cause mortality hazard ratio [HR] 0·52 [95% CI 0·31-0·87; p=0·0107]; treatment-emergent all-cause mortality 0·45 [0·24-0·83; 0·0094]; idiopathic-pulmonary-fibrosis-related mortality 0·35 [0·17-0·72; 0·0029]; treatment-emergent idiopathic-pulmonary-fibrosis-related mortality 0·32 [0·14-0·76; 0·0061]). Consistent with the pooled analysis, meta-analyses for all-cause mortality at week 52 also showed a clinically relevant and significant risk reduction in the pirfenidone group compared with the placebo group. Over 120 weeks, we noted significant differences in the pooled analysis favouring pirfenidone

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

PubMed

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

PubMed Central

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

2011-01-01

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

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

PubMed

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

2009-01-01

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

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

PubMed Central

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

Idiopathic orthostatic hypotension treated with levodopa and MAO inhibitor: a preliminary report

PubMed Central

Sharpe, J.; Marquez-Julio, A.; Ashby, P.

1972-01-01

The clinical and pathophysiological features of a case of idiopathic orthostatic hypotension (Shy-Drager syndrome) are presented. Recent reports on the pathological findings in this condition indicate that there may be a defect in catecholamine synthesis in the pigmented brain stem nuclei and sympathetic ganglia similar to that in idiopathic parkinsonism. On this basis a new form of therapy using levodopa combined with MAO inhibition is derived. The results of a trial of this therapy, which produced improvements in both the hypotension and in the extrapyramidal features of the disease, are reported. PMID:5056115

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

PubMed

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

1994-10-01

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

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

PubMed

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

2017-08-01

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

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

PubMed

D'Agata, Elisabetta; Sánchez-Raya, Judith; Bagó, Juan

2017-01-01

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. 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 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). 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 between personality and HRQOL need to be

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

PubMed

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

2010-08-01

There are no available data for outcomes in children's idiopathic superior mesenteric artery syndrome (SMAS) strictly treated conservatively. The aim of the study was to evaluate clinical and nutritional outcome in children with idiopathic SMAS. A 1-year prospective observation study of effects of treatment and outcome was performed in 27 children (8 boys, 19 girls) with idiopathic SMAS who underwent an upper gastrointestinal (UGI) series, ultrasound measurement of the aortomesenteric angle, treatment, clinical assessment, growth evaluation, and regular clinical visits for more than 12 months. Mean age of the patients was 11.77 +/- 2.15 years. The major clinical complaints were postprandial pain or fullness (88.9%), vomiting (55.6%), and early satiety (51.9%). Eight patients (29.6%) had weight loss. The UGI series revealed typical features of SMAS. The aortomesenteric angle on ultrasound was 10 degrees to 19 degrees. The height of most patients (92.6%) was above the 10th percentile, whereas 15 (55.6%) patients weighed below the 10th percentile. Six patients underwent surgical intervention (3 for obstruction and 3 for persistent anorexia with weight loss), and their clinical symptoms and weight status improved steadily during the follow-up months. Among the 21 patients not subject to surgical intervention, 11 (52.4%) experienced a reduction of symptoms >50% after 3 months of treatment, and weight-for-age percentile increased significantly after 6 months of treatment. Overall, a significant increase in the weight-for-age status was seen in the patients with surgical treatment or with medication only after 6 and 12 months of treatment. An aortomesenteric angle <20 degrees is a constant phenomenon in children with idiopathic SMAS. A duodenojejunostomy can effectively relieve the obstructive symptoms, such as anorexia, and improve nutritional status, whereas long-term medical treatment may aid in relieving the clinical symptoms, promoting appetite, and improving

Idiopathic Parkinson's disease and depression: a psychosomatic view.

PubMed Central

Todes, C J

1984-01-01

The link between idiopathic Parkinson's disease and depression is examined in the light of psychosomatic theory. A view of the condition is offered as a manifestation of chronic emotional disorder in an organic sense. Predisposition arises from bereavement and/or maternal failure in early emotional development. PMID:6707677

Isolated upper eyelid retraction: a sign of idiopathic inflammatory orbital disease.

PubMed

Shome, Debraj; Toshniwal, Svetlana; Jain, Vandana; Natarajan, Sundaram; Vemuganti, Geeta K

2008-01-01

A 41-year-old woman was examined for left upper eyelid retraction. Remaining ocular and systemic examination was unremarkable. Orbital CT demonstrated an ill-defined, extraconal, superior orbital soft-tissue mass involving the levator palpebrae superioris muscle. Incisional biopsy with histopathology demonstrated idiopathic orbital inflammation. The patient was started on a gradually tapering dose of oral steroids, for 6 weeks. On follow-up, the eyelid retraction had resolved. We report this case to demonstrate that idiopathic inflammatory orbital disease, localized to the superior orbit, may cause isolated upper eyelid retraction without associated proptosis. This condition resolves with medical therapy, leading to symmetrical palpebral apertures.

Comparison of plasma, liver, and skeletal muscle carnitine concentrations in cats with idiopathic hepatic lipidosis and in healthy cats.

PubMed

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.

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

PubMed

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

2016-03-01

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

The Use of IL-1 Receptor Antagonist (Anakinra) in Idiopathic Recurrent Pericarditis: A Narrative Review

PubMed Central

Baskar, Shankar; Klein, Allan L.; Zeft, Andrew

2016-01-01

Recurrent pericarditis is a complication of acute pericarditis in 20–30% of the patients and is usually idiopathic in nature. The underlying pathogenesis of this condition remains unclear, although immune-mediated mechanisms seem likely. A subgroup of these patients with refractory symptoms can be challenging to manage, and multiple immunosuppressive medications have been used without consistent benefit. Anakinra, an interleukin-1 receptor antagonist, has been used in treatment of rheumatoid arthritis and autoinflammatory syndromes. Preliminary evidence suggests that anakinra could be a promising therapy for idiopathic recurrent pericarditis. In this narrative review, we summarize the current understanding of the etiopathogenesis of idiopathic recurrent pericarditis, mechanism of action of anakinra, and the preliminary evidence, supporting the use of anakinra in pericarditis. PMID:26942035

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

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

Bhatt, M.H.; Snow, B.J.; Martin, W.R.

1991-06-01

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 lossmore » 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.« less

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

PubMed

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

2016-02-01

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

Crohn's disease mistaken for long-standing idiopathic mesenteric panniculitis: A case report and management algorithm.

PubMed

Nuzzo, Alexandre; Zappa, Magaly; Cazals-Hatem, Dominique; Bouhnik, Yoram

2016-09-01

Mesenteric panniculitis (MP) is mostly an associated sign of an intra-abdominal or systemic inflammatory primary disease. Nevertheless, etiological and differential diagnosis of idiopathic MP can be challenging when an associate primary cause is not in the foreground. We report here the case of an isolated small bowel Crohn's disease, long time considered as idiopathic MP. This patient presented to our department with a 10-year history of acute abdominal symptoms evolving with flare-up and remission. A diagnosis of idiopathic MP was made based on compatible CT-scan features along with normal laboratory tests and upper and lower bowel endoscopies. As symptoms recurred, a steroid course was proposed which dramatically improved his condition for years. Finally, an explorative laparoscopy was performed because of concern of malignancy when he returned to our unit with a steroid refractory flare-up and weight loss, along with MP nodes growing up to 10 mm. Crohn's disease was eventually diagnosed, based on histopathological middle-gut bowel resection and numerous granulomas in mesenteric nodes without necrosis. This case emphasizes the importance of excluding inflammatory intestinal lesions before making the diagnosis of idiopathic MP (fecal calprotectin, magnetic resonance enterography, wireless capsule endoscopy).

The clinical and molecular genetic features of idiopathic infantile periodic alternating nystagmus

PubMed Central

Thomas, Mervyn G.; Crosier, Moira; Lindsay, Susan; Kumar, Anil; Thomas, Shery; Araki, Masasuke; Talbot, Chris J.; McLean, Rebecca J.; Surendran, Mylvaganam; Taylor, Katie; Leroy, Bart P.; Moore, Anthony T.; Hunter, David G.; Hertle, Richard W.; Tarpey, Patrick; Langmann, Andrea; Lindner, Susanne; Brandner, Martina

2011-01-01

Periodic alternating nystagmus consists of involuntary oscillations of the eyes with cyclical changes of nystagmus direction. It can occur during infancy (e.g. idiopathic infantile periodic alternating nystagmus) or later in life. Acquired forms are often associated with cerebellar dysfunction arising due to instability of the optokinetic-vestibular systems. Idiopathic infantile periodic alternating nystagmus can be familial or occur in isolation; however, very little is known about the clinical characteristics, genetic aetiology and neural substrates involved. Five loci (NYS1-5) have been identified for idiopathic infantile nystagmus; three are autosomal (NYS2, NYS3 and NYS4) and two are X-chromosomal (NYS1 and NYS5). We previously identified the FRMD7 gene on chromosome Xq26 (NYS1 locus); mutations of FRMD7 are causative of idiopathic infantile nystagmus influencing neuronal outgrowth and development. It is unclear whether the periodic alternating nystagmus phenotype is linked to NYS1, NYS5 (Xp11.4-p11.3) or a separate locus. From a cohort of 31 X-linked families and 14 singletons (70 patients) with idiopathic infantile nystagmus we identified 10 families and one singleton (21 patients) with periodic alternating nystagmus of which we describe clinical phenotype, genetic aetiology and neural substrates involved. Periodic alternating nystagmus was not detected clinically but only on eye movement recordings. The cycle duration varied from 90 to 280 s. Optokinetic reflex was not detectable horizontally. Mutations of the FRMD7 gene were found in all 10 families and the singleton (including three novel mutations). Periodic alternating nystagmus was predominantly associated with missense mutations within the FERM domain. There was significant sibship clustering of the phenotype although in some families not all affected members had periodic alternating nystagmus. In situ hybridization studies during mid-late human embryonic stages in normal tissue showed restricted

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

PubMed

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 < 0.05; bipolar: 0.62 ± 0.45 vs. 1.03 ± 0.60 mV, P < 0.05). The targeted DPs were still present in 12 patients after successful elimination of the ectopies. 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.

Androgen excess in women: experience with over 1000 consecutive patients.

PubMed

Azziz, R; Sanchez, L A; Knochenhauer, E S; Moran, C; Lazenby, J; Stephens, K C; Taylor, K; Boots, L R

2004-02-01

The objective of the present study was to estimate the prevalence of the different pathological conditions causing clinically evident androgen excess and to document the degree of long-term success of suppressive and/or antiandrogen hormonal therapy in a large consecutive population of patients. All patients presenting for evaluation of symptoms potentially related to androgen excess between October 1987 and June 2002 were evaluated, and the data were maintained prospectively in a computerized database. For the assessment of therapeutic response, a retrospective review of the medical chart was performed, after the exclusion of those patients seeking fertility therapy only, or with inadequate follow-up or poor compliance. A total of 1281 consecutive patients were seen during the study period. Excluded from analysis were 408 patients in whom we were unable to evaluate hormonal status, determine ovulatory status, or find any evidence of androgen excess. In the remaining population of 873 patients, the unbiased prevalence of androgen-secreting neoplasms was 0.2%, 21-hydroxylase-deficient classic adrenal hyperplasia (CAH) was 0.6%, 21-hydroxylase-deficient nonclassic adrenal hyperplasia (NCAH) was 1.6%, hyperandrogenic insulin-resistant acanthosis nigricans (HAIRAN) syndrome was 3.1%, idiopathic hirsutism was 4.7%, and polycystic ovary syndrome (PCOS) was 82.0%. Fifty-nine (6.75%) patients had elevated androgen levels and hirsutism but normal ovulation. A total of 257 patients were included in the assessment of the response to hormonal therapy. The mean duration of follow-up was 33.5 months (range, 6-155). Hirsutism improved in 86%, menstrual dysfunction in 80%, acne in 81%, and hair loss in 33% of patients. The major side effects noted were irregular vaginal bleeding (16.1%), nausea (13.0%), and headaches (12.6%); only 36.6% of patients never complained of side effects. In this large study of consecutive patients presenting with clinically evident androgen excess

Improving cancer care for American Indians with cervical cancer in the Indian Health Service (IHS) system - Navigation may not be enough.

PubMed

Dockery, Lauren E; Motwani, Anita; Ding, Kai; Doescher, Mark; Dvorak, Justin D; Moore, Kathleen N; Holman, Laura L

2018-04-01

Patient navigation programs have been shown to positively impact cancer outcomes for minority populations. Little is known regarding the effects of these programs on American Indian (AI) populations. The purpose of this study is to characterize the impact of a patient navigation program on AI cervical cancer patients at a tertiary care center. A retrospective review of all AI cervical cancer patients receiving navigation services and a cohort of AI patients treated prior to navigation services was performed. Additional comparisons were made between those with and without Indian Health Service (IHS) funding. Summary statistics were used to describe demographic, clinical characteristics, treatment, and survivorship across groups. Of 55 patients identified, 34 received navigation and 21 did not. In navigated patients, median age was 46years (27-80years) compared with 42years (17-68years) in pre-navigation patients (p=0.53). There was no difference between stage at diagnosis (p=0.73). No difference was noted in treatment received between groups (p=0.48). Distance traveled for treatment between groups did not differ (p=0.46). Median time to initiation of treatment was not different between groups, 30.5days vs. 27.5days (p=0.18). Among patients with IHS funding, navigation services did not alter time to initiation of treatment (p=0.57), and there was no difference in completion of prescribed therapy between groups (92% navigated vs 100% pre-navigation). Navigation services for AI cervical cancer patients did not alter initiation or completion of treatment. Navigation programs may provide less tangible benefits to AI cervical cancer patients and further study is warranted. Copyright © 2017 Elsevier Inc. All rights reserved.

Adolescent idiopathic scoliosis and the single-nucleotide polymorphism of the growth hormone receptor and IGF-1 genes.

PubMed

Yang, Yong; Wu, Zhihong; Zhao, Taimao; Wang, Hai; Zhao, Dong; Zhang, Jianguo; Wang, Yipeng; Ding, Yaozhong; Qiu, Guixing

2009-06-01

The etiology of adolescent idiopathic scoliosis is undetermined despite years of research. A number of hypotheses have been postulated to explain its development, including growth abnormalities. The irregular expression of growth hormone and insulin-like growth factor-1 (IGF-1) may disturb hormone metabolism, result in a gross asymmetry, and promote the progress of adolescent idiopathic scoliosis. Initial association studies in complex diseases have demonstrated the power of candidate gene association. Prior to our study, 1 study in this field had a negative result. A replicable study is vital for reliability. To determine the relationship of growth hormone receptor and IGF-1 genes with adolescent idiopathic scoliosis, a population-based association study was performed. Single nucleotide polymorphisms with potential function were selected from candidate genes and a distribution analysis was performed. A conclusion was made confirming the insufficiency of an association between adolescent idiopathic scoliosis and the single-nucleotide polymorphism of the growth hormone receptor and IGF-1 genes in Han Chinese.

Diffuse idiopathic hyperplasia of the sternocleidomastoid muscle in a child

PubMed Central

Pal, Kamalesh; Bhat, Nisar; Moghazy, Khaled; Mitra, DK; Hegazi, Mohammed

2009-01-01

Unilateral diffuse or localized enlargement of the sternocleidomastoid muscle (SCM) is an event commonly seen in infancy, and is popularly known as ‘sternocleidomastoid tumor’. The condition, which usually spontaneously resolves with or without physiotherapy, is due to a hematoma following a difficult labor. The muscle regains its elasticity and complete function. In some infants it resolves with fibromatous changes in the muscle leading to shortening, fibrosis and finally culminating in torticollis. We describe a case of idiopathic diffuse enlargement of unilateral SCM in a 12-year-old child without any functional compromise or torticollis. The histopathological and clinical characteristics differentiating it from more commonly described sternocleidomastoid tumor or fibromatosis coli are described. We believe this is the first case report of idiopathic hyperplasia of SCM. PMID:19847086

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

PubMed

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

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

PubMed Central

2014-01-01

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

Spinal alignment in surgical, multisegmental, transpedicular correction of adolescent idiopathic scoliosis

PubMed Central

Nowakowski, Andrzej; Dworak, Lechosław B.; Kubaszewski, Łukasz; Kaczmarczyk, Jacek

2012-01-01

Summary The objective of this study was to discuss the variables influencing alignment mechanisms of the spine, with particular consideration of post-surgical alignment in adolescent idiopathic scoliosis. The analysis is based on information currently available in the literature, and on the authors’ own experience, which includes surgical material from over 2200 cases of idiopathic scoliosis. Over 50% of cases of adolescent idiopathic scoliosis are decompensated before surgical treatment. Spinal alignment is most significantly influenced by the position of the pelvis. Surgical restoration of lumbar lordosis is more important than attempting to restore thoracic kyphosis in the sagittal plane. The sagittal profile has an essential impact on spinal alignment. The same curves in the coronal plane can have various 3-dimensional configurations. Clinical difficulties in the assessment of thoracic kyphosis and lumbar lordosis result from the fact that they undergo constant change with age. Thoracic hypokyphosis diagnosed before surgery is a very frequent symptom of curve progression. The presence of proximal (thoraco-thoracic) and distal (thoraco-lumbar) junctional kyphosis is very important for planning the scope of spondylodesis. The natural tendency of the spine for alignment (compensation) after surgery nowadays occurs more naturally by applying derotational forces through pedicle screws, compared to the distraction devices (eg, Harrington rod) used in the past. PMID:23229319

Flow void of cerebrospinal fluid in idiopathic normal pressure hydrocephalus of the elderly: can it predict outcome after shunting?

PubMed

Krauss, J K; Regel, J P; Vach, W; Jüngling, F D; Droste, D W; Wakhloo, A K

1997-01-01

We investigate the predictive value of cerebrospinal fluid (CSF) flow void on outcome after shunting in a prospective series of patients with idiopathic normal pressure hydrocephalus (NPH). The degree and extension of CSF flow void were examined on T2-weighted magnetic resonance imaging scans of 37 elderly patients with idiopathic NPH who underwent subsequent shunting. The degree of flow void was assessed in comparison with the signal of large cerebral arteries. The extension was evaluated via the calculation of sum scores for the occurrence of flow void in different locations of the ventricular system. Those parameters were not considered in the decision to perform shunting. CSF flow void in the aqueduct and the adjacent third and fourth ventricles of the 37 patients with idiopathic NPH was compared with that of 37 age-matched control patients. CSF flow void scores in patients with idiopathic NPH were investigated for correlations between postoperative outcome scores and ventricular width indices. No difference was found between the occurrence of aqueductal CSF flow void in patients with idiopathic NPH and the control group. A significant difference, however, was noted for the extension of the CSF flow void, which was greater in the NPH group. Postoperative improvement was found in 33 of 37 patients with idiopathic NPH at a mean follow-up of 15.6 months. Only small, statistically not significant correlations were found between CSF flow void and postoperative outcome. Flow void sum scores, however, correlated significantly with ventricular width indices. The degree and extension of CSF flow void on T2-weighted magnetic resonance imaging scans have little predictive value for outcome after shunting in patients with idiopathic NPH. The greater extension of the CSF flow void in patients with NPH is most likely related to increased ventricular width. It is not useful to consider CSF flow void findings on conventional magnetic resonance imaging scans in making the

Effects of Low-frequency Current Sacral Dermatome Stimulation on Idiopathic Slow Transit Constipation.

PubMed

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.

Outcome of Radiofrequency Catheter Ablation as a non-pharmacological therapy for idiopathic Ventricular Tachycardia.

PubMed

Samore, Naseer Ahmed; Imran Majeed, Syed Muhammad; Kayani, Azhar Mahmud; Bhalli, Muhammad Asif; Shabbir, Muhammad

2009-09-01

To determine the outcome of Radiofrequency Catheter Ablation (RFCA) as a non-pharmacological curative therapy for idiopathic Ventricular Tachycardia (VT) and to identify procedure-related complications. Descriptive study. The Armed Forces Institute of Cardiology and National Institute of Heart Diseases, Rawalpindi, from February 2001 to October 2008. Ninety eight consecutive patients with idiopathic VT, resistant to drug therapy, who underwent Electrophysiology Studies (EPS) radiofrequency catheter ablation were enrolled. Clinical and electrophysiological variables were recorded and a descriptive analysis was done. Out of the 98 patients, 79 were males (80.6%). The mean age was 33.29+11.93 years. Modes of presentation were sustained VT, Repetitive Monomorphic VT (RMVT), Non-sustained VT (NSVT) and Ventricular Premature Beats (VPBs). Right Ventricular Outflow Tract (RVOT) VT was found in 37 patients, 37 had Idiopathic Left Ventricular Tachycardia (ILVT), 20 had Left Ventricular Outflow Tract (LVOT) VT, and Inflow Right Ventricular Tachycardia (IRVT) was found in 7 patients. Other sites of origin of VT were infrequent. Eight patients had dual morphologies of VT. Atrioventricular Nodal Re-entry Tachycardia (AVNRT) was found in 8 patients. RFCA was successful in abolishing inducible VT in 88 patients. One patient developed complete AV block requiring a permanent pacemaker. Results of this study confirm a high degree of success and safety of radiofrequency catheter ablation as curative therapy for idiopathic ventricular tachycardia.

CONTRAIS: CONservative TReatment for Adolescent Idiopathic Scoliosis: a randomised controlled trial protocol.

PubMed

Abbott, Allan; Möller, Hans; Gerdhem, Paul

2013-09-05

Idiopathic scoliosis is a three-dimensional structural deformity of the spine that occurs in children and adolescents. Recent reviews on bracing and exercise treatment have provided some evidence for effect of these interventions. The purpose of this study is to improve the evidence base regarding the effectiveness of conservative treatments for preventing curve progression in idiopathic scoliosis. Previously untreated girls and boys with idiopathic scoliosis, 9 to 17 years of age with at least one year of remaining growth and a curve Cobb angle of 25-40 degrees will be included. A total of 135 participants will be randomly allocated in groups of 45 patients each to receive one of the three interventions. All three groups will receive a physical activity prescription according to the World Health Organisation recommendations. One group will additionally wear a hyper-corrective night-time brace. One group will additionally perform postural scoliosis-specific exercises. Participation in the study will last until the curve has progressed, or until cessation of skeletal growth. OUTCOME variables will be measured every 6 months. The primary outcome variable, failure of treatment, is defined as progression of the Cobb angle more than 6 degrees, compared to the primary x-ray, seen on two consecutive spinal standing x-rays taken with 6 months interval. Secondary outcome measures include the SRS-22r and EQ5D-Y quality of life questionnaires, the International Physical Activity Questionnaire (IPAQ) short form, and Cobb angle at end of the study. This trial will evaluate which of the tested conservative treatment approaches that is the most effective for patients with adolescent idiopathic scoliosis. NCT01761305.

Eliminating the use of allogeneic blood products in adolescent idiopathic scoliosis surgery.

PubMed

Berney, Mark J; Dawson, Peter H; Phillips, Margaret; Lui, Darren F; Connolly, Paul

2015-07-01

The aim of this study was to compare transfusion requirements in patients before and after the introduction of tranexamic acid as standard in patients undergoing spinal surgery for idiopathic scoliosis in a national orthopaedic hospital. A retrospective chart review of 56 idiopathic scoliosis patients who underwent posterior spinal instrumentation and fusion between 2009 and 2013 at our institution. Preoperative, intraoperative, and postoperative data were measured. Patients who received tranexamic acid as standard (n = 31) showed a trend towards a decrease in transfusion requirements compared with those who received no tranexamic acid (n = 25). These patients had a statistically significant decrease in operative time (223 vs 188 min, p = 0.005), and estimated intraoperative blood loss was reduced by nearly 50% in the tranexamic acid group. They also had an associated reduced decrease in haemoglobin between preoperative and postoperative levels (4 vs 5 g/dL, p = 0.01). Since February 2012, no patient has required intraoperative or postoperative allogeneic blood product transfusion in this hospital. The routine use of antifibrinolytic medications in patients undergoing surgery for adolescent idiopathic scoliosis has effectively eliminated the need for allogeneic blood products.

Associations Between Neuropsychological, Neurobehavioral and Emotional Functioning and Either Narcolepsy or Idiopathic Hypersomnia in Children and Adolescents.

PubMed

Ludwig, Beris; Smith, Simon; Heussler, Helen

2018-04-15

Narcolepsy and idiopathic hypersomnia are chronic neurological sleep disorders characterized by hypersomnolence or excessive daytime sleepiness. This review aims to systematically examine the scientific literature on the associations between narcolepsy and idiopathic hypersomnia and their effect on intellectual functioning, academic achievement, behavior, and emotion. Published studies that examined those associations in children and adolescents were included. Studies in which children or adolescents received a clinical diagnosis, and in which the associated function was measured with at least one objective instrument were included. Twenty studies published between 1968 and 2017 were eligible for inclusion in this review. There does not appear to be a clear association between intellectual functioning and narcolepsy or idiopathic hypersomnia; however, limited research is an obstacle to obtaining generalizability. The variability in results from studies investigating associations between academic achievement and these two hypersomnolence disorders suggests that further research using standardized and validated assessment instruments is required to determine if there is an association. Behavior and emotion appear to be significantly affected by narcolepsy. Only two studies included populations of children and adolescents with idiopathic hypersomnia. Further research using larger populations of children and adolescents with narcolepsy or idiopathic hypersomnia while utilizing standardized and validated instruments is required, because the effect of these conditions of hypersomnolence varies and is significant for each individual. © 2018 American Academy of Sleep Medicine.

Anti-TNF therapy for juvenile idiopathic arthritis-related uveitis

PubMed Central

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.

PubMed

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.

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

PubMed

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

2003-01-01

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

Long-term follow-up of amitriptyline treatment for idiopathic cough.

PubMed

Ryan, Marisa A; Cohen, Seth M

2016-12-01

To evaluate short- and long-term treatment outcomes of amitriptyline for idiopathic cough. Retrospective chart review and anonymous survey of a patient cohort. We evaluated a cohort of adults treated for cough at the Duke Voice Care Center with amitriptyline over a 2- to 3-year period. We characterized demographics, symptoms, treatment variability, and treatment effects from chart review and survey responses. We performed univariate analysis comparing cough improvement to age, gender, symptoms of throat irritation, cough duration, concurrent speech therapy, and dosage. Eighty-nine percent were taking the medication at the first clinical follow-up at a mean 2.6 months, and overall, 67% reported ≥50% improvement. No statistically significant predictors of cough improvement with medication were identified. At follow-up 2 to 3 years later, overall, 53% reported ≥50% improvement, with only 34% of patients still taking amitriptyline. After 2 to 3 years, 65% of patients had titrated the medication to effect, and 33% restarted the medication. Occurrence of side effects was the most frequent reason for stopping the medication. Amitriptyline can be an effective and well-tolerated part of short- and long-term management of idiopathic cough in adults. Titrating the dose and restarting are often necessary. Larger studies and randomized control trials are needed to better understand the outcomes of using amitriptyline to treat idiopathic cough. 4 Laryngoscope, 126:2758-2763, 2016. © 2016 The American Laryngological, Rhinological and Otological Society, Inc.

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

PubMed

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

2015-01-01

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

Western blot immunoassay for HSP-70 antibodies in idiopathic tinnitus: a preliminary report.

PubMed

Savastano, Marina; Celadin, Marilena; Pittoni, Marina; Plebani, Mario; Marioni, Gino

2006-03-01

Our preliminary study investigated the role of nonspecific immunologic tests and immunoassay for heat shock protein 70 (HSP-70) in supporting the possibility of an autoimmune inner ear process determining idiopathic tinnitus. Thirty-six consecutive patients with idiopathic tinnitus without other otologic or autoimmune diseases and 20 healthy blood donor subjects underwent determinations of circulating immune complexes (CICs) and other nonspecific immunologic factors and immunoassay for HSP-70. The mean CIC values were 4.2 microg/mL in the tinnitus patients and 0.9 microg/mL in the control group (p = .012). Thirteen of the 36 tinnitus patients and none of the control group were HSP-70-positive. Ten of the 13 HSP-70-positive patients had CIC values higher than normal. In the tinnitus group, the mean CIC values were 6.9 microg/mL and 2.6 microg/mL in the HSP-70-positive and -negative subgroups, respectively (p = .024). It may be hypothesized that in a significant number of cases, idiopathic tinnitus could be induced by immune response to inner ear-specific HSP-70.

Prognostic value of cardiovascular magnetic resonance imaging measurements corrected for age and sex in idiopathic pulmonary arterial hypertension.

PubMed

Swift, Andrew J; Rajaram, Smitha; Campbell, Michael J; Hurdman, Judith; Thomas, Steve; Capener, Dave; Elliot, Charlie; Condliffe, Robin; Wild, Jim M; Kiely, David G

2014-01-01

There are limited data on the prognostic value of cardiovascular magnetic resonance measurements in idiopathic pulmonary arterial hypertension, with no studies investigating the impact of correction of cardiovascular magnetic resonance indices for age and sex on prognostic value. Consecutive patients with idiopathic pulmonary arterial hypertension underwent cardiovascular magnetic resonance imaging at 1.5T. Steady-state free precession cardiac volumes and mass measurements were corrected for age, sex, and body surface area according to reference data and prognostic significance assessed. A total of 80 patients with idiopathic pulmonary arterial hypertension were identified, and 23 patients died during the mean follow-up of 32±14 months. Corrected for age, sex, and body surface area, right ventricular end-systolic volume (P=0.004) strongly predicted mortality, independent of World Health Organization functional class, mean right atrial pressure, cardiac index, and mixed venous oxygen saturations. Consideration should be given to correcting cardiovascular magnetic resonance measures for age, sex, and body surface area, particularly given the changing demographics of patients with idiopathic pulmonary arterial hypertension. Corrected right ventricular end-systolic volume is a strong prognostic marker in idiopathic pulmonary arterial hypertension, independent of invasively derived measurements, mean right atrial pressure cardiac index, and mixed venous oxygen saturations.

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

PubMed

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

2006-01-01

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

Observation and Early Intervention in Mild Idiopathic Scoliosis via Corrective Exercises in Growing Children.

PubMed

Sy, Ng

2016-01-01

Idiopathic scoliosis afflicts 2-3% of the population. For mild curvatures, observation is the treatment of choice. Though this passive "wait and see" approach has been used for many years, the practice is inconsistent among different countries. In Anglo-Saxon countries where scoliosis specific exercises are not practised, observation is indicated for curvatures below 25° in growing children and adolescents. In countries, such as France, Germany, Italy and Poland where scoliosis specific corrective exercises are employed, only patients with no signs of maturity and with curvatures below 15° are treated by observation. Patients with curvatures between 15 - 25° are treated by scoliosis specific exercises. In view of the unpredictability of the progression of scoliosis curvatures in immature patients and the lack of knowledge of long term biomechanical repercussions of mild idiopathic scoliosis on lumbar spine and lower extremities, it is proposed that active intervention through scoliosis specific exercises rather than passive observation be employed in the treatment of mild adolescent idiopathic scoliosis.

[Idiopathic scrotal elephantiasis. A case report].

PubMed

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.

Facial morphology in children and adolescents with juvenile idiopathic arthritis and moderate to severe temporomandibular joint involvement.

PubMed

Hsieh, Yuh-Jia; Darvann, Tron A; Hermann, Nuno V; Larsen, Per; Liao, Yu-Fang; Bjoern-Joergensen, Jens; Kreiborg, Sven

2016-02-01

The aims of this study were to (1) assess lateral facial morphology in children and adolescents with juvenile idiopathic arthritis and moderate to severe temporomandibular joint (TMJ) involvement, (2) compare the lateral facial morphology of these subjects with and without TMJ involvement using cephalograms and 3-dimensional (3D) facial photographs, and (3) compare and correlate the results of the 3D photographic and cephalometric analyses. Sixty patients with juvenile idiopathic arthritis were included and grouped as follows: group 1, juvenile idiopathic arthritis patients without TMJ involvement; group 2, juvenile idiopathic arthritis patients with moderate to severe unilateral TMJ involvement; and group 3, juvenile idiopathic arthritis patients with moderate to severe bilateral TMJ involvement. Lateral cephalograms were used to assess and compare lateral facial morphologies between the groups. Lateral projections of oriented 3D photographs were superimposed on the lateral cephalograms. The results of the lateral 3D photographic analysis were correlated with those of lateral cephalometric analysis. Group 3 showed the most severe growth disturbances, including more retrognathic mandible and retruded chin, steep occlusal and mandibular planes, and more hyperdivergent type (P <0.01). Group 2 showed similar growth disturbances, but to a lesser extent than did group 3. Photographic variables were significantly correlated with the soft tissue and skeletal variables of cephalograms (0.5 < r < 0.9; P <0.001). Subjects with juvenile idiopathic arthritis and unilateral or bilateral moderate to severe TMJ involvement had significant growth disturbances. Early intervention is recommended for these patients to prevent unfavorable facial development. Furthermore, with proper orientation, 3D photographs can be used as an alternative to conventional lateral cephalograms and 2-dimensional photographs. Copyright © 2016 American Association of Orthodontists. Published by Elsevier

Idiopathic Juxtafoveolar Retinal Telangiectasis: A Current Review

PubMed Central

Nowilaty, Sawsan R.; Al-Shamsi, Hanan N.; Al-Khars, Wajeeha

2010-01-01

Idiopathic juxtafoveolar retinal telangiectasis (IJFT), also known as parafoveal telangiectasis or idiopathic macular telangiectasia, refers to a heterogeneous group of well-recognized clinical entities characterized by telangiectatic alterations of the juxtafoveolar capillary network of one or both eyes, but which differ in appearance, presumed pathogenesis, and management strategies. Classically, three groups of IJFT are identified. Group I is unilateral easily visible telangiectasis occurring predominantly in males, and causing visual loss as a result of macular edema. Group II, the most common, is bilateral occurring in both middle-aged men and women, and presenting with telangiectasis that is more difficult to detect on biomicroscopy, but with characteristic and diagnostic angiographic and optical coherence tomography features. Vision loss is due to retinal atrophy, not exudation, and subretinal neovascularization is common. Group III is very rare characterized predominantly by progressive obliteration of the perifoveal capillary network, occurring usually in association with a medical or neurologic disease. This paper presents a current review of juxtafoveolar retinal telangiectasis, reviewing the classification of these entities and focusing primarily on the two most common types encountered in clinical practice, i.e., groups I and II, describing their clinical features, histopathology, natural history, complications, latest results from imaging modalities and functional studies, differential diagnosis, and treatment modalities. PMID:20844678

Idiopathic sclerosing encapsulating peritonitis (or abdominal cocoon).

PubMed

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.

Manual therapy as a conservative treatment for adolescent idiopathic scoliosis: a systematic review

PubMed Central

Romano, Michele; Negrini, Stefano

2008-01-01

Background 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. Methods 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. Results 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. Conclusion 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. PMID:18211702

Manual therapy as a conservative treatment for adolescent idiopathic scoliosis: a systematic review.

PubMed

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.

Cancer Risk after Cyclophosphamide Treatment in Idiopathic Membranous Nephropathy

PubMed Central

van Dijk, Peter R.; Hofstra, Julia M.; Wetzels, Jack F.M.

2014-01-01

Background and objectives Cyclophosphamide treatment improves renal survival in patients with idiopathic membranous nephropathy. However, use of cyclophosphamide is associated with cancer. The incidence of malignancies in patients with idiopathic membranous nephropathy was evaluated, and the cancer risk associated with cyclophosphamide use was estimated. Design, setting, participants, & measurements Patients who attended the clinic were included prospectively from 1995 on. A crude incidence ratio for the occurrence of malignancy was calculated. Incidence ratios were subsequently standardized to potential confounders. Latency between cyclophosphamide therapy and the occurrence of cancer was estimated by stratifying for time since the start of treatment. Finally, Poisson regression was used to obtain a multiple adjusted incidence ratio and investigate the dose–response relationship between cyclophosphamide and cancer. Results Data were available for 272 patients; the mean age was 51 years, and 70% of the patients were men. Median follow-up was 6.0 years (interquartile range=3.6–9.5), and 127 patients were treated with cyclophosphamide. Cancer incidence was 21.2 per 1000 person-years in treated patients compared with 4.6 per 1000 person-years in patients who did not receive cyclophosphamide, resulting in crude and adjusted incidence ratios of 4.6 (95% confidence interval, 1.5 to 18.8) and 3.2 (95% confidence interval, 1.0 to 9.5), respectively. Conclusion Cyclophosphamide therapy in idiopathic membranous nephropathy gives a threefold increase in cancer risk. For the average patient, this finding translates into an increase in annual risk from approximately 0.3% to 1.0%. The increased risk of malignancy must be balanced against the improved renal survival. PMID:24855280

Are we simplifying balance evaluation in adolescent idiopathic scoliosis?

PubMed

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), p<0.05. Frontal Cobbs, apical rotations, distal kyphosis, pelvic incidence, sacral slope, sagittal vertical axis, and frontal balance 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.

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

PubMed

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

2015-04-01

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

Specific CAPN10 gene haplotypes influence the clinical profile of polycystic ovary patients.

PubMed

Gonzalez, Alejandro; Abril, Eduardo; Roca, Alfredo; Aragón, Maria José; Figueroa, Maria José; Velarde, Pilar; Ruiz, Rocío; Fayez, Omar; Galán, José Jorge; Herreros, José Antonio; Real, Luis Miguel; Ruiz, Agustín

2003-11-01

Recently, several research groups have evaluated CAPN10 gene in polycystic ovarian syndrome (PCOS) patients and other phenotypes, including hirsutism or intermediate phenotypes of PCOS. Molecular genetic analysis of CAPN10 gene indicates that different alleles may play a role in PCOS susceptibility and could be associated with idiopathic hirsutism. However, these observations are not exempt from controversy, because independent studies cannot replicate these preliminary findings. We present a haplotype-phenotype correlation study of CAPN10 haplotypes in 148 women showing ecographically detected polycystic ovaries (PCO) combined with one or more of these clinical symptoms: amenorrhea or severe oligomenorrhea, hyperandrogenism, and anovulatory infertility, as well as 93 unrelated controls. We have reconstructed and analyzed 482 CAPN10 haplotypes in patients and controls. We detected the association of UCSNP-44 allele with PCO phenotype in the Spanish population (P = 0.02). In addition, we identified several CAPN10 alleles associated to phenotypic differences observed between PCO patients, such as the presence of hypercholesterolemia (haplotype 1121, P = 0.005), presence of hyperandrogenic features (P = 0.05), and familial cancer incidence (haplotype 1111, P = 0.0005). Our results confirm the association of UCSNP-44 allele with PCO phenotype in the Spanish population. Moreover, we have identified novel candidate risk alleles and genotypes, within CAPN10 gene, that could be associated with important phenotypic and prognosis differences observed in PCOS patients.

Effects of Low-frequency Current Sacral Dermatome Stimulation on Idiopathic Slow Transit Constipation

PubMed Central

Kim, Jin-Seop; Yi, Seung-Ju

2014-01-01

[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. PMID:25013277

The Idiopathic Intracranial Hypertension Treatment Trial

PubMed Central

Wall, Michael; Kupersmith, Mark J.; Kieburtz, Karl D.; Corbett, James J.; Feldon, Steven E.; Friedman, Deborah I.; Katz, David M.; Keltner, John L.; Schron, Eleanor B.; McDermott, Michael P.

2015-01-01

IMPORTANCE To our knowledge, there are no large prospective cohorts of untreated patients with idiopathic intracranial hypertension (IIH) to characterize the disease. OBJECTIVE To report the baseline clinical and laboratory features of patients enrolled in the Idiopathic Intracranial Hypertension Treatment Trial. DESIGN, SETTING, AND PARTICIPANTS We collected data at baseline from questionnaires, examinations, automated perimetry, and fundus photography grading. Patients (n = 165) were enrolled from March 17, 2010, to November 27, 2012, at 38 academic and private practice sites in North America. All participants met the modified Dandy criteria for IIH and had a perimetric mean deviation between −2 dB and −7 dB. All but 4 participants were women. MAIN OUTCOMES AND MEASURES Baseline and laboratory characteristics. RESULTS The mean (SD) age of our patients was 29.0 (7.4) years and 4 (2.4%) were men. The average (SD) body mass index (calculated as weight in kilograms divided by height in meters squared) was 39.9 (8.3). Headache was the most common symptom (84%). Transient visual obscurations occurred in 68% of patients, back pain in 53%, and pulse synchronous tinnitus in 52%. Only 32% reported visual loss. The average (SD) perimetric mean deviation in the worst eye was −3.5 (1.1) dB, (range, −2.0 to −6.4 dB) and in the best eye was −2.3 (1.1) dB (range, −5.2 to 0.8 dB). A partial arcuate visual field defect with an enlarged blind spot was the most common perimetric finding. Visual acuity was 85 letters or better (20/20) in 71% of the worst eyes and 77% of the best eyes. Quality of life measures, including the National Eye Institute Visual Function Questionnaire–25 and the Short Form–36 physical and mental health summary scales, were lower compared with population norms. CONCLUSIONS AND RELEVANCE The Idiopathic Intracranial Hypertension Treatment Trial represents the largest prospectively analyzed cohort of untreated patients with IIH. Our data show

Causes of gynaecomastia in young adult males and factors associated with idiopathic gynaecomastia.

PubMed

Ersöz, Halil önder; Onde, Mehmet Emin; Terekeci, Hakan; Kurtoglu, Soner; Tor, Hayati

2002-10-01

Gynaecomastia is a common clinical condition. Persistent pubertal or late onset idiopathic gynaecomastia is the leading cause of gynaecomastia in different series. The aim of this study was the assessment of the prevalence and characteristics of different causes of gynaecomastia in young adult males, and evaluation of the factors associated with idiopathic gynaecomastia. Fifty-three male patients (mean age 22.04 +/- 2.22, range 19-29), who had been admitted to our outpatient clinics with gynaecomastia as the main presenting symptom were enrolled in the study. Patients were evaluated with breast palpation, breast ultrasonography, anthropometric measurements and sex steroid levels. Secondary causes of gynaecomastia were ruled out. Thirty age-matched healthy individuals were also studied as healthy control group. Idiopathic gynaecomastia was diagnosed in 31 of 53 patients (58%), with 17 (32%) persistent pubertal and 14 (24%) late onset course. Other causes of gynaecomastia were hypogonadism in 13 cases (25%), hyperprolactinaemia in five (9%), chronic liver disease in two (4%), and drug induced (prolonged use of H2 antagonists) in two (4%). Patients with idiopathic gynaecomastia, either pubertal or late onset, were compared with the healthy control group in order to find out associated factors. Anthropometric measurements revealed a significant increase in body weight and body mass index (BMI) in the patient group compared with healthy controls (72.4 +/- 13.3 vs. 63.6 +/- 7.9 kg, p = 0.0086 and 25.2 +/- 4.0 vs. 21.5 +/- 2.7 kg/m2, p = 0.0001). Total skin fold thickness (SFT) of four different regions were also higher in the patient group (50.9 +/- 22.1 vs. 32.6 +/- 10.2 mm, p = 0.0006) indicating a higher body fat percentage. Total serum testosterone (4.76 +/- 1.31 vs. 5.70 +/- 1.06 microg/mL, p = 0.0038) and luteinizing hormone (LH) (4.80 +/- 1.92 vs. 7.32 +/- 1.90 mIU/mL, p < 0.0001) levels were significantly lower in the patient group while oestradiol levels were

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

PubMed

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

2008-01-01

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

Conservative management of idiopathic scoliosis--guidelines based on SOSORT 2006 Consensus.

PubMed

Kotwicki, Tomasz; Durmała, Jacek; Czaprowski, Dariusz; Głowacki, Maciej; Kołban, Maciej; Snela, Sławomir; Sliwiński, Zbigniew; Kowalski, Ireneusz M

2009-01-01

Idiopathic scoliosis, defined as a lateral curvature of the spine of above 10 degrees (Cobb angle), is seen in 2-3% of the growing age population, while curves above 20 degrees , requiring conservative treatment, are found in 0.3-0.5%. In our observation, both under-treatment of progressive curves and over-treatment of stable cases are common during conservative management of scoliosis. A model of therapeutic management is presented based on the experience of Polish clinicians specialising in the treatment of scoliosis as well as the effects of work of a panel of experts of SOSORT (Society on Scoliosis Orthopaedic and Rehabilitation Treatment). The model comprises the indications for conservative treatment according to age, curve type and size and Risser grading. The aetiology, classifications, usefulness of the Lonstein and Carlson factor of progression and other methods of determining the probability of scoliosis progression, as well as the psychological aspects of conservative management are presented. Based on the knowledge of the natural history of idiopathic scoliosis, factors of progression and on the SOSORT experts' opinion, guidelines are proposed for clinicians treating children and adolescents with idiopathic scoliosis, including the timing and course of brace treatment and the types of exercises. Uniform practical guidelines developed by experts may represent an essential step towards establishing standards of conservative scoliosis care in our country.

Correlates of the peak height velocity in girls with idiopathic scoliosis.

PubMed

Sanders, James O; Browne, Richard H; Cooney, Timothy E; Finegold, David N; McConnell, Sharon J; Margraf, Susan A

2006-09-15

Prospective longitudinal. Determine correlates of the peak height velocity (PHV) in girls with idiopathic scoliosis. Only identifiable retrospectively, the PHV is the most useful known maturity marker in idiopathic scoliosis. Clinically useful correlates are needed to make PHV timing helpful. A total of 24 immature girls with idiopathic scoliosis were followed with serial heights, sexual staging, skeletal ages, spinal radiographs, insulin-like growth factor (IGF)-1, IGF binding protein-3, dehydroepiandrosterone sulfate, estradiol, bone-specific alkaline phosphatase, and osteocalcin levels. These markers were correlated to PHV timing. There were 14 girls who had identifiable growth peaks that averaged 10.5 +/- 1.8 cm/y at age 11.7 +/- 1 years. At the PHV, all girls were Risser 0 with open triradiate cartilages. On a skeletal age radiograph, digital uncapped phalangeal epiphyses were indicative of pre-PHV and fused epiphyses of post-PHV. Capped but unfused epiphyses were indeterminate. Tanner stage 1 for breast strongly indicates pre-PHV. Stage 3 for breast and pubic hair occurred at or after the PHV, and stage 4 always occurred after PHV. Higher IGF-1 and estradiol levels after PHV are potentially discriminatory. The PHV occurs during Risser 0 with open triradiate cartilages. If triradiate cartilages are open, then Tanner stages, IGF-1, estradiol levels, and the appearance of the epiphyses on a skeletal age radiograph are useful in determining status before or after PHV.

Consanguineous marriage and increased risk of idiopathic congenital talipes equinovarus: a case-control study in a rural area.

PubMed

Sahin, Orcun; Yildirim, Cengiz; Akgun, Rahmi C; Haberal, Bahtiyar; Yazici, Ayse C; Tuncay, Ismail C

2013-01-01

The purpose of this study is to evaluate if there is any relationship between consanguineous marriages and idiopathic congenital talipes equinovarus (CTEV). A case-control study on CTEV screening was conducted in a rural eastern city of Turkey between 2009 and 2011 and a total of 28 cases (infants with idiopathic CTEV) and 575 controls (healthy infants) were recruited. Sociodemographic status of the infants, including gestational age and birth weights, maternal characteristics and, if any, the degree of consanguinity, were recorded. As an inclusion criterion, only singleton, full-term, live births were accepted. A backward stepwise logistic regression model was used to evaluate the relationship between idiopathic CTEV and parental consanguinity. Unadjusted and adjusted odds ratios (OR) with 95% confidence interval (CI) were calculated. Among maternal and infant characteristics, significant risk factors for idiopathic CTEV in the regression analysis were work status (employed), consanguineous marriage, sex (male), and gestational age (>42 wk). Babies born to first-cousin parents had >4 times the risk of idiopathic CTEV [OR, 4.138, (95% CI, 1.484, 11.538)] and the risk for those born to distant relatives was 2.9 times higher [OR, 2.941, (95% CI, 1.070, 8.087)] than for children of unrelated parents. Consanguineous marriage was significantly associated with an increased risk of idiopathic CTEV. This association remained significant even after adjusting for potential confounding variables. To obtain more accurate results, a population-based screening study with an increased number of cases and controls should be performed in future studies. Case-control study investigating the effect of a patient characteristic on the outcome of disease (level-III).

CONTRAIS: CONservative TReatment for Adolescent Idiopathic Scoliosis: a randomised controlled trial protocol

PubMed Central

2013-01-01

Background Idiopathic scoliosis is a three-dimensional structural deformity of the spine that occurs in children and adolescents. Recent reviews on bracing and exercise treatment have provided some evidence for effect of these interventions. The purpose of this study is to improve the evidence base regarding the effectiveness of conservative treatments for preventing curve progression in idiopathic scoliosis. Methods/design Patients: Previously untreated girls and boys with idiopathic scoliosis, 9 to 17 years of age with at least one year of remaining growth and a curve Cobb angle of 25–40 degrees will be included. A total of 135 participants will be randomly allocated in groups of 45 patients each to receive one of the three interventions. Interventions: All three groups will receive a physical activity prescription according to the World Health Organisation recommendations. One group will additionally wear a hyper-corrective night-time brace. One group will additionally perform postural scoliosis-specific exercises. Outcome: Participation in the study will last until the curve has progressed, or until cessation of skeletal growth. Outcome variables will be measured every 6 months. The primary outcome variable, failure of treatment, is defined as progression of the Cobb angle more than 6 degrees, compared to the primary x-ray, seen on two consecutive spinal standing x-rays taken with 6 months interval. Secondary outcome measures include the SRS-22r and EQ5D-Y quality of life questionnaires, the International Physical Activity Questionnaire (IPAQ) short form, and Cobb angle at end of the study. Discussion This trial will evaluate which of the tested conservative treatment approaches that is the most effective for patients with adolescent idiopathic scoliosis. Trial registration NCT01761305 PMID:24007599

Manual Therapy in the Treatment of Idiopathic Scoliosis. Analysis of Current Knowledge.

PubMed

Czaprowski, Dariusz

2016-10-28

Apart from the recommended specific physiotherapy, the treatment of idiopathic scoliosis (IS) also incorporates non-specific manual therapy (NMT). The aim of this paper is to assess the efficacy of NMT (manual therapy, chiropractic, osteopathy) used in the treatment of children and adolescents with IS. The study analysed systematic reviews (Analysis 1) and other recent scientific publications (Analysis 2). Analysis 1 encompassed papers on the use of NMT in patients with IS. Works concerning specific physiotherapy (SP) or bracing (B) and other types of scoliosis were excluded from the analysis. Inclusion criteria for Analysis 2 were: treatment with NMT; subjects aged 10-18 years with IS. The following types of papers were excluded: works analysing NMT combined with SP or B, reports concerning adult pa tients, analyses of single cases and publications included in Analysis 1. Analysis 1: six systematic reviews contained 6 papers on the efficacy of NMT in the treatment of IS. The results of these studies are contradictory, ranging from Cobb angle reduction to no treatment effects whatsoever. The papers analysed are characterised by poor methodological quality: small group sizes, incomplete descriptions of the study groups, no follow-up and no control groups. Analysis 2: in total, 217 papers were found. None of them met the criteria set for the analysis. 1. Few papers verifying the efficacy of manual therapy, chiropractic and osteopathy in the treatment of idiopathic scoliosis have been published to date. 2. The majority are experimental studies with poor methodology or observational case studies. 3. At present, the efficacy of non-specific manual therapy in the treatment of patients with idiopathic scoliosis cannot be reliably evaluated. 4. It is necessary to conduct further research based on appropriate methods (prospective, rando mi s ed, controlled studies) in order to reliably assess the usefulness of non-specific manual therapy in the treatment of idiopathic

Echocardiography-based spectrum of severe tricuspid regurgitation: the frequency of apparently idiopathic tricuspid regurgitation.

PubMed

Mutlak, Diab; Lessick, Jonathan; Reisner, Shimon A; Aronson, Doron; Dabbah, Salim; Agmon, Yoram

2007-04-01

The cause of tricuspid valve (TV) regurgitation (TR) occasionally remains unclear. The objectives of our study were to define the causal spectrum of severe TR diagnosed by echocardiography at a tertiary medical center and to assess the relative frequency and determine the clinical and echocardiographic characteristics of TR without an apparent cause (idiopathic TR). Consecutive patients with severe TR were identified by the echocardiography laboratory computerized database. The echocardiographic reports of all patients were reviewed and the causes of TR were determined. The echocardiographic studies and medical charts were reviewed in patients without an obvious cause of TR. Of 242 consecutive patients diagnosed with severe TR, organic TV disease was evident in 23 patients (9.5%) and significant pulmonary hypertension (estimated pulmonary artery systolic pressure > 50 mm Hg) in an additional 157 patients (64.9%). After further excluding patients with various confounding factors, possibly associated with occult organic TV disease or significant pulmonary hypertension, 23 patients (9.5%) had severe TR without an apparent cause. Of these, TV coaptation appeared relatively intact, allowing adequate estimation of pulmonary artery pressure, in 15 patients (6.2% of all patients with severe TR; idiopathic TR group). Patients with idiopathic TR were older (76 +/- 10 years), with a high frequency of atrial fibrillation (93%), and prominent TV annular dilatation. After excluding multiple potential causes of TR, severe TR is occasionally idiopathic. Annular dilatation (secondary to aging, atrial fibrillation, or other causes) is the likely mechanism of TR in these patients.

Ultrasound-guided corticosteroid injection of the subtalar joint for treatment of juvenile idiopathic arthritis.

PubMed

Young, Cody M; Horst, Deanna M; Murakami, James W; Shiels, William E

2015-07-01

The subtalar joint is commonly affected in children with juvenile idiopathic arthritis and is challenging to treat percutaneously. To describe the technique for treating the subtalar joint with US-guided corticosteroid injections in children and young adults with juvenile idiopathic arthritis and to evaluate the safety of the treatment. We retrospectively analyzed 122 patients (age 15 months-29 years) with juvenile idiopathic arthritis who were referred by a pediatric rheumatologist for corticosteroid injection therapy for symptoms related to the hindfoot or ankle. In these patients the diseased subtalar joint was targeted for therapy, often in conjunction with adjacent affected joints or tendon sheaths of the ankle. We used a protocol based on age, weight and joint for triamcinolone hexacetonide or triamcinolone acetonide dose prescription. We describe the technique for successful treatment of the subtalar joint. A total of 241 subtalar joint corticosteroid injections were performed under US guidance, including 68 repeat injections for recurrent symptoms in 26 of the 122 children and young adults. The average time interval between repeat injections was 24.8 months (range 2.2-130.7 months, median 14.2 months). Subcutaneous tissue atrophy and skin hypopigmentation were the primary complications observed. These complications occurred in 3.9% of the injections. With appropriate training and practice, the subtalar joint can be reliably and safely targeted with US-guided corticosteroid injection to treat symptoms related to juvenile idiopathic arthritis.

Idiopathic autoimmune hemolytic anemia due to lecithin overdose: a case report

PubMed Central

2009-01-01

Introduction Idiopathic Autoimmune Hemolytic Anemia is a potentially fatal condition which requires prompt and potent treatment. Diagnosis of idiopathic autoimmune hemolytic anemia requires both serologic evidence of autoantibody presence and hemolysis. Although most of the times it is considered idiopathic, several underlying causes have been identified, like autoimmune and connective tissue diseases, viral infections, drugs or hyper function of the immune system. To our knowledge, this is the first case in the international literature describing lecithin-induced autoimmune hemolytic anemia. Case Presentation This case report is to highlight a rare but dangerous adverse reaction to overdose of lecithin. A 38 year old white female from Greece, presented to our emergency room with progressive fatigue over a period of ten days and icteric discoloration of her skin and conjunctiva. The patient had been taking lecithin supplements (1200 mg, 3 capsules a day) over a period of ten days for weight loss. She reports that the last 3 days, prior to the examination, she took 5 capsules/day, so that the supplement would take effect more rapidly. Her past medical, social and family history showed no disturbance. Relatives of the patient were requested to submit any blood-tests taken over a period of 20 days prior to the onset of symptoms caused by Lecithin. All tests proved that all functions were within normal scale. Her physical examination revealed pallor and jaundice without palpable hepatosplenomegaly. Blood biochemistry tests showed total bilirubin 7.5 mg/dl, with indirect bilirubin 6.4 mg/dl and complete blood count showed hemoglobin 7.6 g/dl with blood levels 21.4%. Conclusion In every case of idiopathic autoimmune hemolytic anemia the administration of pharmaceutical substances should always be examined, except for the standard reasons that cause it. In this case the cause of hemolysis was attributed to the excessive intake of lecithin capsules for the loss of body

Surgical repair of idiopathic scrotal elephantiasis.

PubMed

Zacharakis, Evangelos; Dudderidge, Tim; Zacharakis, Emmanouil; Ioannidis, Evangelos

2008-02-01

Scrotal lymphedema (scrotal elephantiasis) is uncommon outside of filariasis endemic regions. We present a case of a 65-year-old with idiopathic lymphedema of the scrotum and functional impairment of the penis. The patient underwent surgical excision of the edematous subcutaneous tissues and plastic reconstruction of his penis and scrotum. Three years later, the patient showed no signs of local recurrence, had complete restoration of urinary and sexual function and was extremely satisfied with the result. Surgical management was an effective strategy in the management of scrotal lymphedema in this case.

[Idiopathic inflammatory bowel disease - advancements in surgical treatment].

PubMed

Ulrych, J; Krška, Z

2012-10-01

Treatment of idiopathic inflammatory bowel disease is constantly developing. Biological therapy has become a standard part of conservative treatment, and gene and cell therapy of these diseases is in preclinical phase. Surgical therapy also offers some progress in the treatment, such as the increasingly preferred laparoscopic approach offering the numerous benefits of minimally invasive surgery or a tendency to perform stapled anastomosis. A retrospective analysis of patients with a diagnosis of idiopathic inflammatory bowel operated on at the First Department of Surgery, General University Hospital in the years 2007-2011 was performed. Within this period, 179 patients diagnosed with Crohns disease were operated on. 30 patients underwent acute operation and 149 patients were indicated for elective surgery. In the same period, 40 patients with ulcerative colitis were indicated for surgery, of whom 22 patients for acute surgery and 18 for elective surgery. Multidisciplinary approach in the treatment of patients with inflammatory bowel disease is crucial and patients should be treated in specialized centres. New possibilities of conservative treatment and progress in surgical therapy mutually correlate, and thus the choice of a correct therapeutic procedure requires specific cooperation between the surgeon and the gastroenterologist.

Idiopathic hypercalciuria and formation of calcium renal stones

PubMed Central

Coe, Fredric L.; Worcester, Elaine M.; Evan, Andrew P.

2018-01-01

The most common presentation of nephrolithiasis is idiopathic calcium stones in patients without systemic disease. Most stones are primarily composed of calcium oxalate and form on a base of interstitial apatite deposits, known as Randall’s plaque. By contrast some stones are composed largely of calcium phosphate, as either hydroxyapatite or brushite (calcium monohydrogen phosphate), and are usually accompanied by deposits of calcium phosphate in the Bellini ducts. These deposits result in local tissue damage and might serve as a site of mineral overgrowth. Stone formation is driven by supersaturation of urine with calcium oxalate and brushite. The level of supersaturation is related to fluid intake as well as to the levels of urinary citrate and calcium. Risk of stone formation is increased when urine citrate excretion is <400 mg per day, and treatment with potassium citrate has been used to prevent stones. Urine calcium levels >200 mg per day also increase stone risk and often result in negative calcium balance. Reduced renal calcium reabsorption has a role in idiopathic hypercalciuria. Low sodium diets and thiazide-type diuretics lower urine calcium levels and potentially reduce the risk of stone recurrence and bone diseas PMID:27452364

Nephrotic syndrome complicated by idiopathic central diabetes insipidus.

PubMed

Konomoto, Takao; Tanaka, Etsuko; Imamura, Hideaki; Orita, Mayuko; Sawada, Hirotake; Nunoi, Hiroyuki

2014-05-01

There is ongoing discussion regarding the mechanisms underlying edema formation in nephrotic syndrome (NS). Many studies published in the last decade reported that primary renal sodium retention was a major factor in edema formation. However, many of the factors influencing edema formation in NS remain unclear, including the role of arginine vasopressin (AVP). We report a 12-year-old boy with steroid-dependent NS complicated by idiopathic central diabetes insipidus (CDI). He did not develop edema during his first relapse of NS after developing CDI, despite having hypoalbuminemia. He had polydipsia, polyuria, low urine osmolality, and a low serum arginine AVP level. His fractional excretion of sodium was only slightly low. Endocrinological testing and magnetic resonance imaging revealed idiopathic CDI. After starting desmopressin therapy, he developed edema when his NS relapsed. This is the first known reported case of NS in a patient with CDI. The findings suggest that appropriate AVP secretion in response to an increase in serum osmolality caused by renal sodium retention is necessary for excess extracellular fluid accumulation in NS. Further investigation is needed to more fully understand the role of AVP in edema formation in NS.

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

PubMed

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

2016-07-01

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

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

PubMed

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

2012-09-01

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

Familial idiopathic pulmonary fibrosis. Evidence of lung inflammation in unaffected family members

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

Bitterman, P.B.; Rennard, S.I.; Keogh, B.A.

1986-05-22

We evaluated 17 clinically unaffected members of three families with an autosomal dominant form of idiopathic pulmonary fibrosis for evidence of alveolar inflammation. Each person in the study was examined by gallium-67 scanning for a general estimate of pulmonary inflammation, and by bronchoalveolar lavage for characterization of the types of recovered cells and their state of activation. Eight of the 17 subjects had evidence of alveolar inflammation on the lavage studies. Supporting data included increased numbers of neutrophils and activated macrophages that released one or more neutrophil chemoattractants, and growth factors for lung fibroblasts--findings similar to those observed in patientsmore » with overt idiopathic pulmonary fibrosis. Four of these eight also had a positive gallium scan; in all the other clinically unaffected subjects the scan was normal. During a follow-up of two to four years in seven of the eight subjects who had evidence of inflammation, no clinical evidence of pulmonary fibrosis has appeared. These results indicate that alveolar inflammation occurs in approximately half the clinically unaffected family members at risk of inheriting autosomal dominant idiopathic pulmonary fibrosis. Whether these persons with evidence of pulmonary inflammation but no fibrosis will proceed to have clinically evident pulmonary fibrosis is not yet known.« less

Bilateral transverse sinus stenosis in patients with tinnitus.

PubMed

Chiarella, G; Bono, F; Cassandro, C; Lopolito, M; Quattrone, A; Cassandro, E

2012-08-01

Tinnitus is a frequent complaint in patients affected by intracranial hypertension (IH). Recently, some studies have reported an association between idiopathic intracranial hypertension (IIH) and bilateral transverse sinus stenosis (BTSS). We investigated the relationship between BTSS and monosymptomatic tinnitus, regardless of its clinical characteristics, in subjects without clinical evidence of IH. We selected 78 subjects (all women, mean age 49.5 ± 10.36) affected by tinnitus, without clinical history of audiological and otological pathologies, enrolled among outpatients of the Institute of Audiology and Phoniatrics in Catanzaro, Italy, over a 2 year period. All subjects underwent psychometric evaluation, psychoacoustic assessment, neurological and ophthalmological examination, cerebral magnetic resonance venography (MRV) and brain magnetic resonance imaging (MRI). MRV identified BTSS in 17.9% (14 patients). In the BTSS group, tinnitus was bilateral/central in 21%, and monolateral in the remaining patients (50% left; 29% right ear). It was more frequently pulsating in the BTSS group, but 64.9% of BTSS subjects described their tinnitus as stable. No features of tinnitus showed statistical significance in association with BTSS. In BTSS subjects, we found values suggesting IH by lumbar puncture (LP) in 40% of cases. In these patients, LP gave immediate improvement of tinnitus. The association between BTSS and tinnitus, regardless of its features, must be considered when other causes of tinnitus are excluded.

Early prediction of medication refractoriness in children with idiopathic epilepsy based on scalp EEG analysis.

PubMed

Lin, Lung-Chang; Ouyang, Chen-Sen; Chiang, Ching-Tai; Yang, Rei-Cheng; Wu, Rong-Ching; Wu, Hui-Chuan

2014-11-01

Refractory epilepsy often has deleterious effects on an individual's health and quality of life. Early identification of patients whose seizures are refractory to antiepileptic drugs is important in considering the use of alternative treatments. Although idiopathic epilepsy is regarded as having a significantly lower risk factor of developing refractory epilepsy, still a subset of patients with idiopathic epilepsy might be refractory to medical treatment. In this study, we developed an effective method to predict the refractoriness of idiopathic epilepsy. Sixteen EEG segments from 12 well-controlled patients and 14 EEG segments from 11 refractory patients were analyzed at the time of first EEG recordings before antiepileptic drug treatment. Ten crucial EEG feature descriptors were selected for classification. Three of 10 were related to decorrelation time, and four of 10 were related to relative power of delta/gamma. There were significantly higher values in these seven feature descriptors in the well-controlled group as compared to the refractory group. On the contrary, the remaining three feature descriptors related to spectral edge frequency, kurtosis, and energy of wavelet coefficients demonstrated significantly lower values in the well-controlled group as compared to the refractory group. The analyses yielded a weighted precision rate of 94.2%, and a 93.3% recall rate. Therefore, the developed method is a useful tool in identifying the possibility of developing refractory epilepsy in patients with idiopathic epilepsy.

Corticosteroid treatment and timing of surgery in idiopathic granulomatous mastitis confusing with breast carcinoma.

PubMed

Erozgen, Fazilet; Ersoy, Yeliz E; Akaydin, Murat; Memmi, Naim; Celik, Aysun Simsek; Celebi, Fatih; Guzey, Deniz; Kaplan, Rafet

2010-09-01

Idiopathic granulomatous mastitis (IGM) is an uncommon chronic inflammatory lesion of the breast with an uncertain optimal treatment regimen, the physical examination, and radiologic features of which may be confused with breast carcinoma. In this study, we aimed to describe the clinicopathologic characteristics of 33 patients who admitted to our breast policlinic and took the diagnosis of granulomatous (idiopathic and non-idiopathic) mastitis, and report the place of corticosteroids and the timing of surgery in the treatment of patients with IGM. The clinical features of 33 patients who presented to our breast policlinic with the complaint of breast mass and reached the final diagnosis of GM between March 2005 and October 2009 were reported. The most common symptoms were mass (n: 27) and pain (n: 11). Ultrasonography (USG) and biopsy were performed in all of the patients. Mammography (MMG) was performed in 9, and magnetic resonance imaging (MRI) in 10 patients. The diagnosis of idiopathic lobular granulomatous mastitis (ILGM) was made in 25 patients and tuberculous mastitis (non-idiopathic GM) in the remaining 8 patients. Twenty-four patients received steroid treatment except one who was pregnant. After giving birth, she also received steroids. One of the patients who developed recurrence after 11 months repeated the steroid therapy. Eight patients with tuberculous mastitis were placed on a regimen of antituberculosis therapy for 6 months. In the diagnosis of IGM, physical examination, USG, MMG, and even MRI alone may sometimes not be enough. They should be discussed altogether and the treatment should begin after definitive histopathologic result. Fine needle aspiration biopsy for cytology will result in a high level of diagnostic accuracy, however, core biopsy will reinforce the exact result. Corticosteroid therapy has been shown to be efficacious for IGM, but in the existence of complications such as abscess formation, fistulae, and persistent wound infection

[Regulation of airway stem cell proliferation in idiopathic pulmonary fibrosis].

PubMed

Yang, S X; Wu, Q; Sun, X; Li, X; Li, K; Xu, L; Li, Y; Zhang, Q Y; Zhang, Y C; Chen, H Y

2016-09-01

To investigate the effect of fibroblasts on regulating airway stem cell proliferation in idiopathic pulmonary fibrosis. Lung cell suspension was prepared from β-actin-GFP mice. Airway stem cells were obtained by fluorescence activated cell sorting and co-cultured with lung fibroblasts. The fibroblasts were treated with TGF-β inhibitor SB43142. The expression of growth factors FGF1/2 and the effect of FGF1/2 on stem cell proliferation were observed. The cloning efficiency of airway stem cells, when co-cultured with normal lung fibroblast cells for 8 days, was (3.5±1.1)%, while the cloning efficiency was reduced to (0.04±0.04)% when co-cultured with lung fibroblasts from idiopathic pulmonary fibrosis patients. The difference between the 2 groups was statistically significant(P=0.002 5). TGF-β receptor inhibitor SB431542 increased lung fibroblast growth factors FGF1/2 expression.FGF1 mRNA expression was increased to the experimental group 0.005 5 from 0.000 2 in the control group.FGF2 mRNA expression of the amount raised to the experimental group 0.000 15 from 0.000 8 in the control group.FGF1/2 promoted the growth of airway stem cells. After FGF1/2 was co-cultured with normal lung fibroblast cells for 8 days, the cloning efficiency of airway stem cells was (0.3±0.1)%. During the development of idiopathic pulmonary fibrosis, fibroblast secreted FGF1/2 regulate airway stem cell proliferation.

Computational modeling of venous sinus stenosis in idiopathic intracranial hypertension

PubMed Central

Levitt, Michael R; McGah, Patrick M; Moon, Karam; Albuquerque, Felipe C; McDougall, Cameron G; Kalani, M Yashar S; Kim, Louis J; Aliseda, Alberto

2016-01-01

Background and Purpose Idiopathic intracranial hypertension has been associated with dural venous sinus stenosis in some patients, but the hemodynamic environment of the dural venous sinuses has not been quantitatively described. Here, we present the first such computational fluid dynamics model using patient-specific blood pressure measurements. Materials and Methods Six patients with idiopathic intracranial hypertension and at least one stenosis or atresia at the transverse-sigmoid sinus junction underwent MRV followed by cerebral venography and manometry throughout the dural venous sinuses. Patient-specific computational fluid dynamics models were created using MRV anatomy, with venous pressure measurements as boundary conditions. Blood flow and wall shear stress were calculated for each patient. Results Computational models of dural venous sinuses were successfully reconstructed in all six patients with patient-specific boundary conditions. Three patients demonstrated a pathologic pressure gradient (≥ 8 mm Hg) across four dural venous sinus stenoses. Small sample size precludes statistical comparisons, but average overall flow throughout the dural venous sinuses of patients with pathologic pressure gradients was higher than in those without (1041.00 ± 506.52 vs. 358.00 ± 190.95 mL/min). Wall shear stress was also higher across stenoses in patients with pathologic pressure gradients (37.66 ± 48.39 vs 7.02 ± 13.60 Pa). Conclusion The hemodynamic environment of the dural venous sinuses can be computationally modeled using patient-specific anatomy and physiological measurements in patients with idiopathic intracranial hypertension. There was substantially higher blood flow and wall shear stress in patients with pathological pressure gradients. PMID:27197986

Quantum path integral simulation of isotope effects in the melting temperature of ice Ih.

PubMed

Ramírez, R; Herrero, C P

2010-10-14

The isotope effect in the melting temperature of ice Ih has been studied by free energy calculations within the path integral formulation of statistical mechanics. Free energy differences between isotopes are related to the dependence of their kinetic energy on the isotope mass. The water simulations were performed by using the q-TIP4P/F model, a point charge empirical potential that includes molecular flexibility and anharmonicity in the OH stretch of the water molecule. The reported melting temperature at ambient pressure of this model (T=251 K) increases by 6.5±0.5 and 8.2±0.5 K upon isotopic substitution of hydrogen by deuterium and tritium, respectively. These temperature shifts are larger than the experimental ones (3.8 and 4.5 K, respectively). In the classical limit, the melting temperature is nearly the same as that for tritiated ice. This unexpected behavior is rationalized by the coupling between intermolecular interactions and molecular flexibility. This coupling makes the kinetic energy of the OH stretching modes larger in the liquid than in the solid phase. However, the opposite behavior is found for intramolecular modes, which display larger kinetic energy in ice than in liquid water.

Buckling and bone modeling as factors in the development of idiopathic scoliosis.

PubMed

Goto, Manabu; Kawakami, Noriaki; Azegami, Hideyuki; Matsuyama, Yukihiro; Takeuchi, Kenzen; Sasaoka, Ryu

2003-02-15

Computational analysis using the finite-element method was used to examine a possible etiology of idiopathic scoliosis. To compare changes in the coronal and the transverse planes of idiopathic thoracic scoliosis with changes produced in a finite-element buckling model, and to investigate the influence of bone modeling on the buckling spine. Although it is now widely accepted that growth is related strongly to the onset and progression of scoliosis, the pathomechanism or etiology of idiopathic scoliosis still is not clear. A previous study showed that a buckling phenomenon caused by anterior spinal overgrowth can produce scoliosis, and that the fourth buckling mode matched the clinical characteristics associated with the thoracic type of idiopathic scoliosis. The fourth buckling mode occurs when the first, second, and third buckling modes are prevented. The spinal finite-element model used in this study consisted of 68,582 elements and 84,603 nodes. The transverse changes seen in the computed tomography images of 41 patients with idiopathic thoracic scoliosis (apex, T8; average Cobb angle, 52.5 degrees) were compared with those produced in the fourth buckling mode. Bone modeling (bone formation and resorption) was simulated as heat deformation caused by changes in temperature. The bone formation and resorption were simulated, respectively, by positive and negative volume changes in proportion to the stress that occurred in the buckling spine. Computed tomography images of scoliosis show that as the scoliosis becomes more severe, the thoracic cage decreases on the convex side of the curve and increases on the concave side. The opposite thoracic cage deformation was obtained in the fourth buckling mode. In patients with scoliosis, the sternum essentially remains in its original position with respect to the vertebrae, but in the linear buckling model, it shifted in the direction of vertebral body rotation. In contrast to clinical data, the incremental deformation

Ultrasound-guided corticosteroid injection therapy for juvenile idiopathic arthritis: 12-year care experience.

PubMed

Young, Cody M; Shiels, William E; Coley, Brian D; Hogan, Mark J; Murakami, James W; Jones, Karla; Higgins, Gloria C; Rennebohm, Robert M

2012-12-01

Intra-articular corticosteroid injections are a safe and effective treatment for patients with juvenile idiopathic arthritis. The potential scope of care in ultrasound-guided corticosteroid therapy in children and a joint-based corticosteroid dose protocol designed to optimize interdisciplinary care are not found in the current literature. The purpose of this study was to report the spectrum of care, technique and safety of ultrasound-guided corticosteroid injection therapy in patients with juvenile idiopathic arthritis and to propose an age-weight-joint-based corticosteroid dose protocol. A retrospective analysis was performed of 198 patients (ages 21 months to 28 years) referred for treatment of juvenile idiopathic arthritis with corticosteroid therapy. Symptomatic joints and tendon sheaths were treated as prescribed by the referring rheumatologist. An age-weight-joint-based dose protocol was developed and utilized for corticosteroid dose prescription. A total of 1,444 corticosteroid injections (1,340 joints, 104 tendon sheaths) were performed under US guidance. Injection sites included small, medium and large appendicular skeletal joints (upper extremity 497, lower extremity 837) and six temporomandibular joints. For patients with recurrent symptoms, 414 repeat injections were performed, with an average time interval of 17.7 months (range, 0.5-101.5 months) between injections. Complications occurred in 2.6% of injections and included subcutaneous tissue atrophy, skin hypopigmentation, erythema and pruritis. US-guided corticosteroid injection therapy provides dynamic, precise and safe treatment of a broad spectrum of joints and tendon sheaths throughout the entire pediatric musculoskeletal system. An age-weight-joint-based corticosteroid dose protocol is effective and integral to interdisciplinary care of patients with juvenile idiopathic arthritis.

Risk Factors for Neurodegeneration in Idiopathic REM sleep Behavior Disorder: A Multicenter Study

PubMed Central

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, p<0.001), with similar sex distribution. Neither caffeine, smoking, nor alcohol exposure predicted conversion. 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

Long-Term Effects of Untreated Adolescent Idiopathic Scoliosis: A Review of the Literature

PubMed Central

Karavidas, Nikos; Moramarco, Marc; Moramarco, Kathryn

2016-01-01

Currently, adolescent idiopathic scoliosis (AIS) is principally regarded as benign, but some researchers have cited serious or extreme effects, including severe pain, cardiopulmonary compromise, social isolation, and even early death. Therefore, exploration of the long-term effects of AIS, the most common type of idiopathic scoliosis, is warranted. The purpose of this review was to examine the long-term studies on the natural history of AIS and/or reviews concerning the long-term effects of untreated AIS. A PubMed search was conducted using the key words idiopathic scoliosis, long-term effects and idiopathic scoliosis, natural history. For further analysis, references cited in those studies were reviewed for additional, related evidence not retrieved in the initial PubMed search. A review of the pertinent bibliography showed that older natural history studies did not distinguish between late-onset scoliosis (referred to in this paper as AIS) and early-onset scoliosis (EOS). The more recent studies offer such important distinction and reach to the general conclusion that untreated AIS does not lead to severe consequences with respect to signs and symptoms of scoliosis. It is possible that earlier studies may have included patient populations with EOS, leading to the perception of untreated scoliosis as having an unusually high morbidity rate. Studies on the long-term effects of AIS that specifically excluded EOS patients conclude that AIS is a benign disorder. This indicates that for research and reporting purposes, it is important to distinguishing between AIS and EOS. This will allow the practitioner and patient and their families to decide on an optimal treatment plan based on the most appropriate prognosis. PMID:27994795

Idiopathic Granulomatous Mastitis Associated with Corynebacterium Sp. Infection

PubMed Central

Lee, Yun Sun; Balfour, John

2011-01-01

Idiopathic granulomatous mastitis (IGM) is a rare inflammatory condition of the breast. The etiology and treatments options of IGM remain controversial. Previous case reports have suggested that Corynebacterium sp., a gram-positive bacillus endogenous to the skin, may be associated with IGM. In the present report, we describe the first case of IGM with a positive culture for Corynebacterium sp. reported in the United States. PMID:21857740

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

PubMed

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

1996-03-01

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

Comparing the Roles of EUS, ERCP and MRCP in Idiopathic Acute Recurrent Pancreatitis.

PubMed

Safari, Mohammad Taghi; Miri, Mohammad Bager; Ebadi, Shahram; Shahrokh, Shabnam; Mohammad Alizadeh, Amir Houshang

2016-01-01

Acute recurrent pancreatitis (ARP) is defined as more than two attacks of acute pancreatitis with complete or almost complete resolution of symptoms and signs of pancreatitis between episodes. The initial evaluation fails to detect the cause of ARP in 10%-30% of patients, whose condition is classified as idiopathic ARP. Endoscopic ultrasound (EUS) has gained increasing attention as a useful imaging modality for the pancreas and the extrahepatic biliary tree. The close proximity of the pancreas to the digestive tract allows EUS to obtain detailed images of this organ. This review aims to record pancreaticobiliary endoscopic ultrasound (EUS) and other imaging modalities in the clinical management of patients with idiopathic ARP.

Idiopathic restrictive cardiomyopathy is part of the clinical expression of cardiac troponin I mutations

PubMed Central

Mogensen, Jens; Kubo, Toru; Duque, Mauricio; Uribe, William; Shaw, Anthony; Murphy, Ross; Gimeno, Juan R.; Elliott, Perry; McKenna, William J.

2003-01-01

Restrictive cardiomyopathy (RCM) is an uncommon heart muscle disorder characterized by impaired filling of the ventricles with reduced volume in the presence of normal or near normal wall thickness and systolic function. The disease may be associated with systemic disease but is most often idiopathic. We recognized a large family in which individuals were affected by either idiopathic RCM or hypertrophic cardiomyopathy (HCM). Linkage analysis to selected sarcomeric contractile protein genes identified cardiac troponin I (TNNI3) as the likely disease gene. Subsequent mutation analysis revealed a novel missense mutation, which cosegregated with the disease in the family (lod score: 4.8). To determine if idiopathic RCM is part of the clinical expression of TNNI3 mutations, genetic investigations of the gene were performed in an additional nine unrelated RCM patients with restrictive filling patterns, bi-atrial dilatation, normal systolic function, and normal wall thickness. TNNI3 mutations were identified in six of these nine RCM patients. Two of the mutations identified in young individuals were de novo mutations. All mutations appeared in conserved and functionally important domains of the gene. PMID:12531876

Torsional deformity of apical vertebra in adolescent idiopathic scoliosis.

PubMed

Kotwicki, Tomasz; Napiontek, Marek

2002-01-01

CT scans of structural thoracic idiopathic scoliosis were reviewed in nine patients admitted to our department for scoliosis surgery. The apical vertebra scans were chosen and the following parameters were evaluated: 1) alpha angle formed by the axis of vertebra and the axis of spinous process 2) beta concave and beta convex angle between the spinous process and the left and right transverse process, respectively, 3) gamma concave and gamma convex angle between the axis of vertebra and the left and right transverse process, respectively, 4) the rotation angle to the sagittal plane. The constant deviation of the spinous process towards the convex side of the curve was observed. The vertebral body itself was distorted towards the concavity of the curve. The angle between the spinous process and the transverse process was smaller on the convex side of the curve. The torsional, intravertebral deformity of the apical vertebra was a factor acting in the direction opposite to the rotation, in the sense to reduce the deformity of the spine in idiopathic scoliosis.

Anti-adalimumab antibodies in juvenile idiopathic arthritis-related uveitis.

PubMed

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 (p<0.001), uveitis 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 <12 AU/ml, higher serum trough levels did not associate with better control of uveitis (p=0.86). Adalimumab treatment might be better guided by monitoring anti-adalimumab antibody formation in treating JIA-related uveitis.

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

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

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

2002-12-15

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

Elevated Vitamin D Receptor Levels in Genetic Hypercalciuric Stone-Forming Rats Are Associated With Downregulation of Snail

PubMed Central

Bai, Shaochun; Wang, Hongwei; Shen, Jikun; Zhou, Randal; Bushinsky, David A; Favus, Murray J

2010-01-01

Patients with idiopathic hypercalciuria (IH) and genetic hypercalciuric stone-forming (GHS) rats, an animal model of IH, are both characterized by normal serum Ca, hypercalciuria, Ca nephrolithiasis, reduced renal Ca reabsorption, and increased bone resorption. Serum 1,25-dihydroxyvitamin D [1,25(OH)2D] levels are elevated or normal in IH and are normal in GHS rats. In GHS rats, vitamin D receptor (VDR) protein levels are elevated in intestinal, kidney, and bone cells, and in IH, peripheral blood monocyte VDR levels are high. The high VDR is thought to amplify the target-tissue actions of normal circulating 1,25(OH)2D levels to increase Ca transport. The aim of this study was to elucidate the molecular mechanisms whereby Snail may contribute to the high VDR levels in GHS rats. In the study, Snail gene expression and protein levels were lower in GHS rat tissues and inversely correlated with VDR gene expression and protein levels in intestine and kidney cells. In human kidney and colon cell lines, ChIP assays revealed endogenous Snail binding close to specific E-box sequences within the human VDR promoter region, whereas only one E-box specifically bound Snail in the rat promoter. Snail binding to rat VDR promoter E-box regions was reduced in GHS compared with normal control intestine and was accompanied by hyperacetylation of histone H3. These results provide evidence that elevated VDR in GHS rats likely occurs because of derepression resulting from reduced Snail binding to the VDR promoter and hyperacetylation of histone H3. © 2010 American Society for Bone and Mineral Research. PMID:19929616

Idiopathic constipation: A challenging but manageable problem.

PubMed

Bischoff, Andrea; Brisighelli, Giulia; Dickie, Belinda; Frischer, Jason; Levitt, Marc A; Peña, Alberto

2017-10-10

A protocol to treat idiopathic constipation is presented. A contrast enema is performed in every patient and, when indicated, patients are initially submitted to a "clean out" protocol. All patients are started on a Senna-based laxative. The initial dosage is empirically determined and adjusted daily, during a one week period, based on history and abdominal radiographs, until the amount of Senna that empties the colon is reached. The management is considered successful when patients empty their colon daily and stop soiling. If the laxatives dose provokes abdominal cramping, distension, and vomiting, without producing bowel movements, patients are considered nonmanageable. From 2005 to 2012, 215 patients were treated. 121 (56%) were males. The average age was 8.2years (range: 1-20). 160 patients (74%) presented encopresis. 67 patients (32%) needed a clean out. After one week, 181 patients (84%) achieved successful management, with an average Senna dose of 67mg (range: 5-175mg). In 34 patients (16%) the treatment was unsuccessful: 19 were nonmanageable, 3 noncompliant, and 12 continued soiling. At a later follow-up (median: 329days) the success rate for 174 patients was 81%. We designed a successful protocol to manage idiopathic constipation. The key points are clean out before starting laxatives, individual adjustments of laxative, and radiological monitoring of colonic emptying. Level IV. Copyright © 2017 Elsevier Inc. All rights reserved.

Uveitis in juvenile idiopathic arthritis.

PubMed

Heiligenhaus, Arnd; Minden, Kirsten; Föll, Dirk; Pleyer, Uwe

2015-02-06

Juvenile idiopathic arthritis (JIA) is the most common systemic disease causing uveitis in childhood, with a prevalence of 10 per 100 000 persons. JIA often takes a severe inflammatory course, and its complications often endanger vision. This review is based on pertinent articles retrieved by a selective literature search up to 18 August 2014 and on the current interdisciplinary S2k guideline on the diagnostic evaluation and anti-inflammatory treatment of juvenile idiopathic uveitis. Uveitis arises in roughly 1 in 10 patients with JIA. Regular eye check-ups should be performed starting as soon as JIA is diagnosed. 75-80% of patients are girls; antinuclear antibodies are found in 70-90%. The risk to vision is higher if JIA begins in the preschool years. As for treatment, only a single, small-scale randomized controlled trial (RCT) and a small number of prospective trials have been published to date. Topical corticosteroids should be given as the initial treatment. Systemic immunosuppression is needed if irritation persists despite topical corticosteroids, if new complications arise, or if the topical steroids have to be given in excessively high doses or have unacceptable side effects. If the therapeutic effect remains inadequate, conventional and biological immune modulators can be given as add-on (escalation) therapy. Treatment lowers the risk of uveitis and its complications and thereby improves the prognosis for good visual function. Severely affected patients should be treated in competence centers to optimize their long-term outcome. Multidisciplinary, individualized treatment is needed because of the chronic course of active inflammation and the ensuing high risk of complications that can endanger vision. Future improvements in therapy will be aided by prospective, population-based registries and by basic research on biomarkers for the prediction of disease onset, prognosis, tissue damage, and therapeutic response.

Idiopathic granulomatous mastitis associated with risperidone-induced hyperprolactinemia

PubMed Central

2012-01-01

Idiopathic granulomatous mastitis (IGM) is a rare inflammatory breast disease. The etiology and treatment options of IGM remain controversial. Previous case reports have suggested that hyperprolactinemia may be associated with IGM. In the present report, we describe the first case of IGM associated with risperidone-induced hyperprolactinemia. Virtual slides The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/8120093785928228 PMID:22221904

Evaluation of Fitness and the Balance Levels of Children with a Diagnosis of Juvenile Idiopathic Arthritis: A Pilot Study

PubMed Central

Maggio, Maria Cristina; Corsello, Giovanni; Messina, Giuseppe; Iovane, Angelo; Palma, Antonio

2017-01-01

Background: Juvenile idiopathic arthritis is a main cause of physical disability and has high economic costs for society. The purpose of this study was to assess the fitness levels and the postural and balance deficits with a specific test battery. Methods: Fifty-six subjects were enrolled in this study. Thirty-nine healthy subjects were included in the control group and seventeen in the juvenile idiopathic arthritis group. All subjects were evaluated using a posturography system. The fitness level was evaluated with a battery of tests (Abalakov test, sit-up test, hand grip test, backsaver sit and reach, the toe touch test). An unpaired t-test was used to determine differences. Pearson’s correlation coefficient was used to evaluate the correlation between the tests. Results: The battery of tests demonstrated that subjects in the juvenile idiopathic arthritis group have lower fitness levels compared to the control group. The juvenile idiopathic arthritis group showed low postural control with respect to the control group. Pearson analysis of the juvenile idiopathic arthritis group data showed significant correlations between variables. Pearson’s results from the control group data showed a similar trend. Conclusions: The results suggest that the battery of tests used could be an appropriate tool. However, we highlight that these conclusions need to be supported by other studies with a larger population scale. PMID:28753965

Evaluation of Fitness and the Balance Levels of Children with a Diagnosis of Juvenile Idiopathic Arthritis: A Pilot Study.

PubMed

Patti, Antonino; Maggio, Maria Cristina; Corsello, Giovanni; Messina, Giuseppe; Iovane, Angelo; Palma, Antonio

2017-07-19

Background: Juvenile idiopathic arthritis is a main cause of physical disability and has high economic costs for society. The purpose of this study was to assess the fitness levels and the postural and balance deficits with a specific test battery. Methods: Fifty-six subjects were enrolled in this study. Thirty-nine healthy subjects were included in the control group and seventeen in the juvenile idiopathic arthritis group. All subjects were evaluated using a posturography system. The fitness level was evaluated with a battery of tests (Abalakov test, sit-up test, hand grip test, backsaver sit and reach, the toe touch test). An unpaired t -test was used to determine differences. Pearson's correlation coefficient was used to evaluate the correlation between the tests. Results: The battery of tests demonstrated that subjects in the juvenile idiopathic arthritis group have lower fitness levels compared to the control group. The juvenile idiopathic arthritis group showed low postural control with respect to the control group. Pearson analysis of the juvenile idiopathic arthritis group data showed significant correlations between variables. Pearson's results from the control group data showed a similar trend. Conclusions: The results suggest that the battery of tests used could be an appropriate tool. However, we highlight that these conclusions need to be supported by other studies with a larger population scale.

Idiopathic recurrent pericarditis as an immune-mediated disease: current insights into pathogenesis and emerging treatment options.

PubMed

Imazio, Massimo

2014-11-01

Idiopathic recurrent pericarditis affects 30-50% of patients with a previous attack of pericarditis. The etiopathogenesis is incompletely understood and most cases remain idiopathic with a presumed immune-mediated pathogenesis. The mainstay of therapy is aspirin or a nonsteroidal anti-inflammatory drug plus colchicine and the possible adjunct of a low-to-moderate dose of a corticosteroid in more difficult cases. Colchicine as an adjunct to anti-inflammatory therapy reduces by 50% the subsequent recurrent rate. For true refractory cases with failure of standard combination therapies, new and emerging options especially include human intravenous immunoglobulins and biological agents (i.e., anakinra). The outcome of idiopathic recurrent pericarditis is good with a negligible risk of developing constrictive pericarditis. Thus, it is important to reassure patients on their prognosis, explaining the nature of the disease and the likely course. Moreover, therapeutic choices should include less toxic agents and favor cheaper drugs whenever possible.

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

PubMed

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

2013-01-01

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

Histiocytes in familial and infection-induced/idiopathic hemophagocytic syndromes may exhibit phenotypic differences.

PubMed

Buckley, P J; O'Laughlin, S; Komp, D M

1992-01-01

Familial hemophagocytic syndrome (FHS) and infection-associated hemophagocytic syndrome (IAHS) usually present with fever, pancytopenia, hepatosplenomegaly, signs of hepatic dysfunction, bleeding diathesis, and neurological manifestations. FHS is almost uniformly fatal, and IAHS is associated with high mortality. The only distinguishing characteristics are lack of family history and association with infection in the latter. Despite this, sporadic cases of FHS and culture-negative examples of IAHS (idiopathic HS) can be difficult to distinguish and the distinction may have important implications for treatment and family planning. We evaluated the immunophenotype of the macrophages (M phi s) in frozen tissue sections from three cases of hemophagocytic syndrome using a very large panel of monocyte/M phi-associated monoclonal antibodies and an immunoperoxidase technique. The clinical and laboratory features suggested that two were examples of FHS (one with strong family history) and that the third was IAHS/idiopathic HS. The results supported the clinical impressions by showing that the antigenic phenotypes of the FHS cases were nearly identical and different from that of the case of presumed IAHS/idiopathic HS. Specifically, M phi s from the FHS cases expressed complement receptors, 1, 2, and 3 (CD35, CD21, and CD11b, respectively), the monocyte antigen CD36, and the "activation" antigens CD25 (IL2-R) and CD30 (Ki-1), while those from the IAHS/idiopathic case did not. These studies also demonstrated that the M phi s in these cases exhibited some phenotypic differences from those in control tissues, that is, expression of the pan-M phi antigen CD14, the M phi subset antigen identified by antibody G16/1, complement receptors, certain monocyte antigens, and M phi "activation" antigens.(ABSTRACT TRUNCATED AT 250 WORDS)

Experiences of employment among young people with juvenile idiopathic arthritis: a qualitative study.

PubMed

Hanson, Helen; Hart, Ruth I; Thompson, Ben; McDonagh, Janet E; Tattersall, Rachel; Jordan, Alison; Foster, Helen E

2018-08-01

This study explored expectations and experiences of employment among young people with juvenile idiopathic arthritis and the role of health professionals in promoting positive employment outcomes. Semistructured interviews (n = 13) and three focus groups (n = 9, n = 4, n = 3) were conducted with young people (16-25 y) and adults (26-31 y) with juvenile idiopathic arthritis and semistructured interviews (n = 9) were conducted with health professionals. Transcripts were analyzed thematically. Young people with juvenile idiopathic arthritis have concerns about employers' attitudes toward employees with long-term health conditions and lack knowledge of antidiscrimination legislation. Young people not in education, employment or training identify arthritis as a key barrier. Challenges associated with arthritis (e.g., pain, psychological distress) may not be visible to employers. Decisions about disclosing arthritis are challenging and cause anxiety. Young people associate good disease management and access to flexible and convenient care with their capacity to succeed in employment. Psychosocial and vocational interventions have benefited some young people but are not routinely available. Low expectations of employers may affect young people's decisions about disclosure and seeking appropriate support in the work place. Health professionals can equip young people with knowledge and skills to negotiate appropriate support, through signposting to antidiscrimination information and offering practice of transferable skills such as disclosure in consultations. Implications for rehabilitation Young people with juvenile idiopathic arthritis encounter challenges with regard to employment; many lack the knowledge and skills to negotiate appropriate support from employers. Rehabilitation professionals could play a more substantial role in equipping them with relevant knowledge and skills by signposting to antidiscrimination information and nurturing

Non-surgical interventions for adolescents with idiopathic scoliosis: an overview of systematic reviews.

PubMed

Płaszewski, Maciej; Bettany-Saltikov, Josette

2014-01-01

Non-surgical interventions for adolescents with idiopathic scoliosis remain highly controversial. Despite the publication of numerous reviews no explicit methodological evaluation of papers labeled as, or having a layout of, a systematic review, addressing this subject matter, is available. Analysis and comparison of the content, methodology, and evidence-base from systematic reviews regarding non-surgical interventions for adolescents with idiopathic scoliosis. Systematic overview of systematic reviews. Articles meeting the minimal criteria for a systematic review, regarding any non-surgical intervention for adolescent idiopathic scoliosis, with any outcomes measured, were included. Multiple general and systematic review specific databases, guideline registries, reference lists and websites of institutions were searched. The AMSTAR tool was used to critically appraise the methodology, and the Oxford Centre for Evidence Based Medicine and the Joanna Briggs Institute's hierarchies were applied to analyze the levels of evidence from included reviews. From 469 citations, twenty one papers were included for analysis. Five reviews assessed the effectiveness of scoliosis-specific exercise treatments, four assessed manual therapies, five evaluated bracing, four assessed different combinations of interventions, and one evaluated usual physical activity. Two reviews addressed the adverse effects of bracing. Two papers were high quality Cochrane reviews, Three were of moderate, and the remaining sixteen were of low or very low methodological quality. The level of evidence of these reviews ranged from 1 or 1+ to 4, and in some reviews, due to their low methodological quality and/or poor reporting, this could not be established. Higher quality reviews indicate that generally there is insufficient evidence to make a judgment on whether non-surgical interventions in adolescent idiopathic scoliosis are effective. Papers labeled as systematic reviews need to be considered in terms

An inquiry into chiropractors' intention to treat adolescent idiopathic scoliosis: a telephone survey.

PubMed

Feise, R J

2001-01-01

The primary aim of this study was to (1) determine the clinical management approach of practicing chiropractors with regard to patients with adolescent idiopathic scoliosis and (2) measure the response rate of a telephone survey. A survey instrument was developed and pretested, and a case-specific clinical vignette was generated for a hypothetical typical 12-year-old girl with adolescent idiopathic scoliosis. The instrument addressed 3 domains: the specific management of idiopathic scoliosis, elements guiding the general selection of treatment recommendations, and demographics of respondents. The sample frame consisted of 62,000 US chiropractors, of whom 165 were randomly selected for the survey sample. Interviews were conducted by telephone through use of the tested survey instrument. The response rate was 69% (114/165). Of the 51 nonrespondents, 15 did not have a listed business telephone number and 24 were not in active practice. The response rate of those who met the inclusion criteria (practicing chiropractor with a listed telephone number) was 90% (114/126). The gender, chiropractic college, and years in practice of respondents in this survey were similar to those of respondents in 3 other national surveys. In general, the respondents would provide 6 months of "intensive" chiropractic therapy, then follow the patient for 4 years (near skeletal maturity). Eighty-two percent of respondents named diversified technique as their primary adjustive treatment, 87% would use exercise, and 30% would use electric muscle stimulation as an adjunct to manual therapy. Most surveyed chiropractors would use similar methods (frequency and length of treatment, manipulation technique, and exercise) in the treatment of patients with adolescent idiopathic scoliosis. A high response rate to a national survey can be achieved through use of telephone contact.

Psychologic management of brace therapy for patients with idiopathic scoliosis.

PubMed

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

2005-03-01

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

Prevalence and incidence of juvenile idiopathic arthritis: a systematic review.

PubMed

Thierry, Sigrid; Fautrel, Bruno; Lemelle, Irène; Guillemin, Francis

2014-03-01

To conduct a systematic literature review on incidence and prevalence of juvenile idiopathic arthritis and to estimate these figures in Europe for 2010. Articles on incidence or prevalence of juvenile idiopathic arthritis were searched in Medline. Pooled incidence and prevalence were calculated overall, by gender, age, classification and arthritis categories. We used the available age and gender pooled rates to standardize the incidence and prevalence on the 2010 European population and estimate the number of cases in Europe in 2010. Forty-three articles (33 on incidence, 29 on prevalence) were included. Incidence rates varied from 1.6 to 23 and prevalence from 3.8 to 400/100,000. Pooled incidence and prevalence were higher for girls (10.0 [9.4-10.7] and 19.4 [18.3-20.6]/100,000) than boys (5.7 [5.3-6.2] and 11.0 [10.2-11.9]/100,000). Oligoarthritis was the most frequent form (pooled incidence rate 3.7 [3.5-3.9] and prevalence 16.8 [15.9-17.7]/100,000). The direct standardized incidence rate was 8.2 [7.5-9.0] and prevalence 70.2 [62.9-78.1]/100,000. In Europe in 2010, the estimated number of incident cases was 6896 [5481-8578] and 59,175 [44,256-76,983] prevalent cases. Incidence and prevalence varied greatly among published reports of juvenile idiopathic arthritis, which may be explained by methodological issues, classification used, and time. Estimating the number of affected children can be useful, especially with the new treatment possibilities. Copyright © 2013 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

Idiopathic ventricular outflow tract arrhythmias from the great cardiac vein: challenges and risks of catheter ablation.

PubMed

Steven, D; Pott, C; Bittner, A; Sultan, A; Wasmer, K; Hoffmann, B A; Köbe, J; Drewitz, I; Milberg, P; Lueker, J; Mönnig, G; Servatius, H; Willems, S; Eckardt, L

2013-11-20

Catheter ablation for idiopathic ventricular arrhythmia is well established but epicardial origin, proximity to coronary arteries, and limited accessibility may complicate ablation from the venous system in particular from the great cardiac vein (GCV). Between April 2009 and October 2010 14 patients (56 ± 15 years; 9 male) out of a total group of 117 patients with idiopathic outflow tract tachycardias were included undergoing ablation for idiopathic VT or premature ventricular contractions (PVC) originating from GCV. All patients in whom the PVC arose from the GCV were subject to the study. In these patients angiography of the left coronary system was performed with the ablation catheter at the site of earliest activation. Successful ablation was performed in 6/14 (43%) and long-term success was achieved in 5/14 (36%) patients. In 4/14 patients (28.6%) ablation was not performed. In another 4 patients (26.7%), ablation did not abolish the PVC/VT. In the majority, the anatomical proximity to the left coronary system prohibited effective RF application. In 3 patients RF application resulted in a coronary spasm with complete regression as revealed in repeat coronary angiography. A relevant proportion idiopathic VT/PVC can safely be ablated from the GCV without significant permanent coronary artery stenosis after RF application. Our data furthermore demonstrate that damage to the coronary artery system is likely to be transient. © 2013.

Comparing the Roles of EUS, ERCP and MRCP in Idiopathic Acute Recurrent Pancreatitis

PubMed Central

Safari, Mohammad Taghi; Miri, Mohammad Bager; Ebadi, Shahram; Shahrokh, Shabnam; Alizadeh, Amir Houshang Mohammad

2016-01-01

Acute recurrent pancreatitis (ARP) is defined as more than two attacks of acute pancreatitis with complete or almost complete resolution of symptoms and signs of pancreatitis between episodes. The initial evaluation fails to detect the cause of ARP in 10%–30% of patients, whose condition is classified as idiopathic ARP. Endoscopic ultrasound (EUS) has gained increasing attention as a useful imaging modality for the pancreas and the extrahepatic biliary tree. The close proximity of the pancreas to the digestive tract allows EUS to obtain detailed images of this organ. This review aims to record pancreaticobiliary endoscopic ultrasound (EUS) and other imaging modalities in the clinical management of patients with idiopathic ARP. PMID:27375362

Anticyclic citrullinated peptide antibodies in juvenile idiopathic arthritis.

PubMed

Brunner, Jürgen K H; Sitzmann, Friedrich C

2006-01-01

Anticyclic citrullinated peptide (anti-CCP) antibodies have been detected in patients with juvenile idiopathic arthritis (JRA), particularly in those with polyarticular JIA. We analyzed the presence of anti-CCP antibodies of the IgG class in sera of patients with defined juvenile idiopathic arthritis (JIA) of various subgroups. One hundred and fifty-nine serum samples were investigated. Forty-five patients were diagnosed with JIA (15 male and 30 female) aged 1.9-17.3 years (median 12.9, mean 11.0). Thirty-eight samples were taken from patients suffering from other autoimmunopathies and 34 patients with other underlying diseases were taken at different time points in their disease course. Under 42 samples were taken from patients with noninflammatory diseases. Enzyme-linked immunosorbent assay (ELISA) was used for the detection of anti-CCP antibodies. Anti-CCP antibodies were found in 6.9% of all samples and in 4.4% patients with JIA. Disease duration and medication did not differ significantly between anti-CCP positive and negative patients. A review of the literature and our own results shows that anti-CCP antibodies can be detected in the sera of only some patients with JIA. Routine determination of anti-CCP cannot be recommended.

Pleuroparenchymal fibroelastosis: is it also an idiopathic entity?

PubMed

Portillo, Karina; Guasch Arriaga, Ignasi; Ruiz-Manzano, Juan

2015-10-01

Pleuroparenchymal fibroelastosis (PPFE) is a rare disease that has been recently included in the updated consensus on idiopathic interstitial pneumonias. It shares some clinical features with other chronic interstitial pneumonias (dyspnea, dry cough), and is radiologically characterized by pleural and subpleural parenchymal fibrosis and elastosis, mainly in the upper lobes. The main histological findings include pleural fibrosis and prominent subpleural and parenchymal fibroelastosis. Its characterization is based on the increasing number of cases reported in the literature, so several aspects of the etiology, pathogenesis and natural history are still unknown. Although some cases have been described as idiopathic, PPFE has been reported as a complication after bone marrow transplantation, lung transplantation and chemotherapy, especially with alkylating agents.Spontaneous or iatrogenic pneumothorax is a frequently reported complication of invasive diagnostic tests for identifying PPFE. The disease course is variable, ranging from slow progression to rapid clinical deterioration. No treatment has shown evidence of efficacy, and lung transplantation remains the only option for patients who fulfill the diagnostic criteria for this option. Recognizing and disseminating the specific features of PPFE is essential to raise the level of clinical suspicion for this entity, and to implement appropriate multidisciplinary diagnostic management. Copyright © 2015 SEPAR. Published by Elsevier Espana. All rights reserved.

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

PubMed

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 (P<0.05) and 2 years postoperatively (P<0.001) than juveniles; however, both groups reported improved mental health (P<0.001) and self-image (P<0.01) postoperatively. Mental health 2 years postoperatively was predicted by preoperative self-image (P<0.05), mental health (P<0.001), and main thoracic Cobb angle (P<0.05) in the juvenile group. Within the adolescent group, mental health 2 years postoperatively was predicted by preoperative mental health (P<0.001); self-image 2 years postoperatively was predicted by preoperative mental health (P<0.01) and self-image (P<0.001). Self-image and mental health are significantly improved after spine deformity correction in juveniles and adolescents with idiopathic scoliosis. However, consistent with normative development

Management of idiopathic normal-pressure hydrocephalus: a multiinstitutional study conducted in Japan.

PubMed

Mori, K

2001-12-01

A cooperative study was undertaken to identify factors that could be used to predict a favorable outcome after extracranial cerebrospinal fluid (CSF) diversion (shunting) in patients with suspected idiopathic normal-pressure hydrocephalus (NPH). Questionnaires concerning patients with suspected idiopathic NPH were sent to 14 members of the Committee for Scientific Research on Intractable Hydrocephalus, sponsored by the Ministry of Health and Welfare of Japan. After the questionnaires were returned, a retrospective analysis of the responses was undertaken. To be included in the study, patients had to be 65 years of age or older and had to have undergone surgery between October 1995 and October 1998. Clinical measures included degrees of gait disturbance, dementia, and urinary incontinence as evaluated before. 3 months after, and 3 years after shunt placement. Diagnostic tests in various combinations included lumbar puncture in which CSF was withdrawn; intracranial pressure monitoring; measurements of CSF outflow resistance, level of serum alpha-1-antichymotrypsin, cerebral arteriovenous differences of oxygen content, and cerebral blood flow; and computerized tomography cisternography. In this study, 120 patients were identified as having idiopathic NPH and these patients underwent placement of shunts. A ventriculoperitoneal shunt with a programmable valve was used in two thirds of the patients. At the end of 3 months (early assessment), there was an 80% overall rate of clinical improvement, which dropped to 73.3% of the 105 patients who could be evaluated at the end of the 3-year study. Of the three variables, gait disturbance was most improved, both at early and late testing periods. Shunt complications occurred in 22 (18.3%) of the patients. Patients suspected of having idiopathic NPH did not form a homogeneous group, making it difficult to select those who would most likely respond to CSF diversion. Of the diagnostic studies, the most reliable result was

Trunk rotational strength asymmetry in adolescents with idiopathic scoliosis: an observational study

PubMed Central

McIntire, Kevin L; Asher, Marc A; Burton, Douglas C; Liu, Wen

2007-01-01

Background Recent reports have suggested a rotational strength weakness in rotations to the concave side in patients with idiopathic scoliosis. There have been no studies presenting normative values of female adolescent trunk rotational strength to which a comparison of female adolescents with idiopathic scoliosis could be made. The purpose of this study was to determine trunk rotational strength asymmetry in a group of female adolescents with AIS and a comparison group of healthy female adolescents without scoliosis. Methods Twenty-six healthy adolescent females served as the healthy group (HG) (average age 14 years) and fourteen otherwise healthy adolescent females with idiopathic scoliosis served as the idiopathic scoliosis group (ISG) (average age 13.5 years, average Cobb 28°). Participant's isometric trunk rotational strength was measured in five randomly ordered trunk positions: neutral, 18° and 36° of right and left pre-rotation. Rotational strength asymmetry was compared within each group and between the two groups using several different measures. Results The HG showed strength asymmetry in the 36° pre-rotated trunk positions when rotating towards the midline (p < 0.05). The ISG showed strength asymmetry when rotating towards the concavity of their primary curve from the neutral position (p < 0.05) and when rotating towards the concavity from the 18° (p < 0.05) and 36° (p < 0.05) concave pre-rotated positions. The ISG is significantly weaker than the HG when rotating away from the midline toward the concave (ISG)-left (HG) side from the concave/left pre-rotated 18° (p < 0.05) and 36° (p < 0.05) positions. Conclusion The AIS females were found to be significantly weaker when contracting toward their main curve concavity in the neutral and concave pre-rotated positions compared to contractions toward the convexity. These weaknesses were also demonstrated when compared to the group of healthy female adolescent controls. Possible mechanisms for the

Idiopathic granulomatous mastitis: a mimicking disease in a pregnant woman: a case report

PubMed Central

2013-01-01

Background Idiopathic granulomatous mastitis is a rare, benign, inflammatory chronic condition of unclear etiology. This case is reported because it illustrates how idiopathic granulomatous mastitis can mimic other diseases, making it difficult to associate the presenting symptoms and the correct diagnosis; This disease is a challenge for clinicians to diagnose, manage and avoid iatrogenic complications, and requires consultation with experts in several specialties. Case presentation The patient was 30 years old, South-American, eleven weeks pregnant, and with an apparent infectious mastitis. She presented with progressive worsening of her breast symptoms and multiple negative laboratory tests. She suffered different side effects from several prescribed treatments and endured a prolonged recovery. The article emphasizes the need for ruling out common pathologies to arrive at the correct diagnosis such as bacterial and fungal infections; granulomatous conditions like tuberculosis and sarcoidosis; and inflammatory breast carcinoma. It also describes frequently used pharmacological and supplementary forms of treatment for patients with this condition. Conclusion Idiopathic granulomatous mastitis is a rare unusual condition of unknown etiology. Pathological confirmation is required for its diagnosis and optimal management is still unclear. The presentation and management of this case is intended to advance its awareness to physicians from different specialties. PMID:23497626

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

PubMed

Noll, Christine; Steitz, Vanessa; Daentzer, Dorothea

2017-01-01

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

The Immune Response and the Pathogenesis of Idiopathic Inflammatory Myositis: a Critical Review.

PubMed

Ceribelli, Angela; De Santis, Maria; Isailovic, Natasa; Gershwin, M Eric; Selmi, Carlo

2017-02-01

The pathogenesis of idiopathic inflammatory myositis (IIMs, including polymyositis and dermatomyositis) remains largely enigmatic, despite advances in the study of the role played by innate immunity, adaptive immunity, genetic predisposition, and environmental factors in an orchestrated response. Several factors are involved in the inflammatory state that characterizes the different forms of IIMs which share features and mechanisms but are clearly different with respect to the involved sites and characteristics of the inflammation. Cellular and non-cellular mechanisms of both the immune and non-immune systems have been identified as key regulators of inflammation in polymyositis/dermatomyositis, particularly at different stages of disease, leading to the fibrotic state that characterizes the end stage. Among these, a special role is played by an interferon signature and complement cascade with different mechanisms in polymyositis and dermatomyositis; these differences can be identified also histologically in muscle biopsies. Numerous cellular components of the adaptive and innate immune response are present in the site of tissue inflammation, and the complexity of idiopathic inflammatory myositis is further supported by the involvement of non-immune mechanisms such as hypoxia and autophagy. The aim of this comprehensive review is to describe the major pathogenic mechanisms involved in the onset of idiopathic inflammatory myositis and to report on the major working hypothesis with therapeutic implications.

Time for a change: is idiopathic pulmonary fibrosis still idiopathic and only fibrotic?

PubMed Central

Wolters, Paul J; Blackwell, Timothy S; Eickelberg, Oliver; Loyd, James E; Kaminski, Naftali; Jenkins, Gisli; Maher, Toby M; Molina-Molina, Maria; Noble, Paul W; Raghu, Ganesh; Richeldi, Luca; Schwarz, Marvin I; Selman, Moises; Wuyts, Wim A; Schwartz, David A

2018-01-01

Idiopathic pulmonary fibrosis (IPF) is a progressive, irreversible, and typically fatal lung disease characterised by subpleural fibrosis, subepithelial fibroblast foci, and microscopic honeycombing. Although understanding of the pathogenic mechanisms continues to evolve, evidence indicates that distal airway and alveolar epithelial cells are central drivers of the disease. In this Viewpoint, we review the history of naming and classifications used to define the disease now referred to as IPF, in the context of understanding the clinical presentation, causes, and pathogenesis of the disease. We aim to generate discussion on whether, given the substantial progress made in understanding the clinical, genetic, cellular, and molecular mechanisms involved in the development of IPF, a change of name should be considered. To initiate this discussion, we offer new suggestions to update the name of this disease and new approaches to classify all forms of pulmonary fibrosis. PMID:29413083

Clinical Aspects of Idiopathic Inflammatory Bowel Disease: A Review for Pathologists.

PubMed

Lee, Hwajeong; Westerhoff, Maria; Shen, Bo; Liu, Xiuli

2016-05-01

-Idiopathic inflammatory bowel disease manifests with different clinical phenotypes showing varying behavior and risk for neoplasia. The clinical questions that are posed to pathologists differ depending on phase of the disease and the clinical circumstances. Understanding the clinical aspects of the dynamic disease process will enhance the role of pathology in optimizing the care of patients with inflammatory bowel disease. -To review clinical and surgical aspects of inflammatory bowel disease that are relevant to practicing pathologists. -The literature was reviewed. -Diagnosis and management of inflammatory bowel disease require an integrated evaluation of clinical, endoscopic, radiologic, and pathologic features. Therefore, close interaction between clinicians and pathologists is crucial. Having this team approach improves understanding of the pertinent clinical and surgical aspects of the disease and assists in the recognition of unusual presentation of variants, as well as mimics of idiopathic inflammatory bowel disease, by pathologists.