Epiretinal membrane: a treatable cause of visual disability in myotonic dystrophy type 1.

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Kersten, Hannah M; Roxburgh, Richard H; Child, Nicholas; Polkinghorne, Philip J; Frampton, Chris; Danesh-Meyer, Helen V

2014-01-01

A wide range of ocular abnormalities have been documented to occur in patients with myotonic dystrophy type 1. The objectives of this study were to investigate the macular and optic nerve morphology using optical coherence tomography in patients with myotonic dystrophy type 1. A total of 30 myotonic dystrophy type 1 patients and 28 controls were recruited for participation. All participants underwent a thorough ophthalmologic examination, including spectral-domain optical coherence tomography of the macula and retinal nerve fibre layer. Images were reviewed by a retinal specialist ophthalmologist, masked to the diagnosis of the participants. Average macular thickness was significantly greater in the myotonic dystrophy group compared to controls [327.3 μm vs. 308.5 μm (p Visual acuity was reduced due to the presence of epiretinal membrane in six patient eyes and none of the control eyes. The presence of an epiretinal membrane was significantly correlated with increasing age in the patient group. We report an increased prevalence of epiretinal membrane in the myotonic dystrophy type 1 group. This may be a previously under-recognised form of visual impairment in this group. Epiretinal membranes can be treated surgically. We suggest that, in addition to a comprehensive clinical examination, optical coherence tomography examination is implemented as part of an ophthalmological assessment for the myotonic dystrophy type 1 patient with reduced visual acuity.

Recurrence rate and need for reoperation after surgery with or without internal limiting membrane removal for the treatment of the epiretinal membrane.

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De Novelli, Fernando José; Goldbaum, Mauro; Monteiro, Mario Luiz Ribeiro; Aggio, Fabio Bom; Nóbrega, Mario Junqueira; Takahashi, Walter Yukihiko

2017-01-01

To compare the recurrence rate and need for reoperation after epiretinal membrane surgery with and without removal of the internal limiting membrane. In this retrospective study, 125 patients operated for epiretinal membrane removal were evaluated, with a minimum 6-month follow-up. Removal of the epiretinal membrane (ERM) was performed in 78 patients, while 47 had removal of the epiretinal membrane associated with internal limiting membrane peeling (ERM + ILM). The mean age in the ERM group was 65.8 years old, ranging from 41 to 80 years old. In the ERM + ILM group, the mean age was 67.2 years old, ranging from 52 to 82 years old. The mean preoperative visual acuity in the ERM group was 20/80p, and in the ERM + ILM group, it was 20/80. The mean postoperative visual acuity in both groups was 20/30. The mean preoperative macular thickness in the ERM group was 467 µm ranging from 281 to 663 µm; in the ERM + ILM group, the preoperative macular thickness was 497 µm, ranging from 172 to 798 µm. After surgery, a reduction in macular thickness was observed in both groups. In the ERM group, the mean macular thickness reduction was 361 ± 101. µm, whereas in the ERM + ILM group, it was 367 ± 75.2 µm. Twenty-two patients presented with a recurrence of epiretinal membrane, of which 16 (20.5%) were from the ERM group and 6 (12.8%) were from the ERM + ILM group (p = 0.39); one patient (2%) was retreated in the ERM + ILM group, whereas 5 patients (6%) where retreated in the ERM group. We postulate that ILM peeling for the treatment of epiretinal membrane is not a relevant factor either for visual recovery or macular thickness reduction, but it may reduce the recurrence and reoperation rate.

miRNAs in the vitreous humor of patients affected by idiopathic epiretinal membrane and macular hole.

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Andrea Russo

Full Text Available The aim of the present study was to assess the expression of miRNAs in the Vitreous Humor (VH of patients with Macular Hole (MH and Epiretinal Membrane (ERM compared to a control group.In this prospective, comparative study, 2-ml of VH was extracted from the core of the vitreous chamber in consecutive patients who underwent standard vitrectomy for ERM and MH. RNA was extracted and TaqMan® Low Density Arrays (TLDAs were used to profile the transcriptome of 754 miRNAs. Results were validated by single TaqMan® assays. Finally, we created a biological network of differentially expressed miRNA targets and their nearest neighbors.Overall 10 eyes with MH, 16 eyes with idiopathic ERM and 6 controls were enrolled in the study. Profiling data identified 5 miRNAs differentially expressed in patients affected by MH and ERM with respect to controls. Four were downregulated (miR-19b, miR-24, miR-155, miR-451 and 1 was downregulated (miR-29a; TaqMan® assays of the VH of patients affected by MH and ERM, with respect to controls, showed that the most differentially expressed were miR-19b (FC -9.13, p:<0.00004, mir-24 (FC -7.52, p:<0.004 and miR-142-3p (FC -5.32, p:<0.011. Our network data showed that deregulation of differentially expressed miRNAs induces an alteration of several pathways associated with genes involved in both MH and ERM.The present study suggests that disregulation of miR-19b, miR-24 and miR-142-3p, might be related to the alterations that characterize patients affected by MH and ERM.

Comparison of the Effectiveness of Pars Plana Vitrectomy with and without Internal Limiting Membrane Peeling for Idiopathic Retinal Membrane Removal: A Meta-Analysis

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Hanhan Liu

2015-01-01

Full Text Available We conducted a meta-analysis of published retrospective studies and compared the effectiveness of pars plana vitrectomy with and without internal limiting membrane (ILM peeling for idiopathic epiretinal membrane (IERM. The results revealed that patients in the IERM+ILM peeling group had better BCVA after surgery within 12 months than those in IERM peeling group. But patients in the IERM peeling group showed better BCVA in the 18th month. More retrospective studies or randomized controlled trials are required to investigate and compare the long-term effect of IERM removal with and without ILM peeling.

Comparison of the Effectiveness of Pars Plana Vitrectomy with and without Internal Limiting Membrane Peeling for Idiopathic Retinal Membrane Removal: A Meta-Analysis

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Liu, Hanhan; Zuo, Shanru; Ding, Chun; Dai, Xunzhang; Zhu, Xiaohua

2015-01-01

We conducted a meta-analysis of published retrospective studies and compared the effectiveness of pars plana vitrectomy with and without internal limiting membrane (ILM) peeling for idiopathic epiretinal membrane (IERM). The results revealed that patients in the IERM+ILM peeling group had better BCVA after surgery within 12 months than those in IERM peeling group. But patients in the IERM peeling group showed better BCVA in the 18th month. More retrospective studies or randomized controlled trials are required to investigate and compare the long-term effect of IERM removal with and without ILM peeling. PMID:26693348

Heavy trypan blue staining of epiretinal membranes: an alternative to infracyanine green

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Oberstein, Sarit Y. Lesnik; Mura, Marco; Tan, Stevie H.; de Smet, Marc D.

2007-01-01

BACKGROUND: By using dyes, it is easier to identify the extent of an epiretinal membrane (ERM) or the inner limiting membrane (ILM) during surgery. Trypan blue (TB) stains ERM and ILM weakly, but with less apparent toxicity than other intraocular dyes. Its main drawback in vitreoretinal surgery is

Changes in Retinal Thickness after Vitrectomy for Epiretinal Membrane with and without Internal Limiting Membrane Peeling.

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Obata, Shumpei; Fujikawa, Masato; Iwasaki, Keisuke; Kakinoki, Masashi; Sawada, Osamu; Saishin, Yoshitsugu; Kawamura, Hajime; Ohji, Masahito

2017-01-01

To investigate anatomic changes in retinal thickness (RT) and functional changes after vitrectomy for idiopathic epiretinal membranes (ERMs) with and without internal limiting membrane (ILM) peeling. The medical records of 100 eyes of 96 patients with ERM who underwent vitrectomy and ERM removal were reviewed retrospectively. The RT was measured by optical coherence tomography, and the area was divided into 9 sections. The best-corrected visual acuity (BCVA), 9 RT areas, and incidence rates of recurrent ERM were compared between the groups with and without ILM peeling before the operation and 12 months postoperatively. Thirty-nine eyes that underwent vitrectomy with ILM peeling and 61 eyes that underwent vitrectomy without ILM peeling met the inclusion criteria. There were no significant differences between the groups in the BCVA and any of the RTs before the operation and 12 months postoperatively. The ERMs recurred in 8 (20.5%) of 39 eyes and 26 (42.6%) of 61 eyes in the groups with and without ILM peeling, respectively, with a difference that reached significance (p = 0.02) 12 months postoperatively. Vitrectomy for ERM affects the BCVA or the RTs 12 months postoperatively. Additional ILM peeling does not affect them, but it might reduce the ERM recurrence rate. © 2016 S. Karger AG, Basel.

Management of macular epiretinal membrane by vitrectomy and intravitreal triamcinolone.

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Shukla, Dhananjay

2014-04-01

A patient underwent successful vitrectomy for macular epiretinal membrane with anatomical and functional improvement. 10 weeks later, there was a recurrence of macular edema with corresponding visual decline. An intravitreal injection of triamcinolone acetonide not only restored the macular anatomy but also improved the visual outcome beyond that achieved after surgery.

Epiretinal membrane removal in patients with Stargardt disease

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Muna Bhende

2015-01-01

Full Text Available Epiretinal membranes (ERMs in Stargardt disease have been known to undergo spontaneous separation in children. Results of surgical intervention in adult patients with Stargardt disease have rarely been reported. A retrospective review of results of surgical intervention for ERM causing visual impairment in two adult patients of Stargardt disease was carried out. Both patients developed ERM in one eye during their follow-up period with the resultant drop in their preexisting visual acuity. Postsurgery, restoration of foveal contour with some improvement in visual acuity was observed in both patients. No adverse effect of surgery was noted.

Eccentric Macular Hole after Pars Plana Vitrectomy for Epiretinal Membrane Without Internal Limiting Membrane Peeling: A Case Report.

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Garnavou-Xirou, Christina; Xirou, Tina; Kabanarou, Stamatina; Gkizis, Ilias; Velissaris, Stavros; Chatziralli, Irini

2017-12-01

Postoperative eccentric macular hole formation is an uncommon complication after pars plana vitrectomy (PPV) without internal limiting membrane (ILM) peeling for the treatment of epiretinal membrane (ERM). We present a case of eccentric macular hole formation after PPV for ERM without ILM peeling. A 68-year-old male patient presented with ERM and visual acuity of 6/24 in his left eye. He underwent 23-gauge PPV without ILM peeling for treatment of ERM. One week postoperatively the retina was attached and the epiretinal membrane was successfully removed, while visual acuity was 6/9. One month after PPV, a single eccentric retinal hole below the macula was detected using fundoscopy and subsequently confirmed by optical coherence tomography. At this time the visual acuity was 6/9 and the patient reported no symptoms. No further intervention was attempted and at the 9-month follow-up, the visual acuity and the size of the eccentric macular hole remained stable. Eccentric macular holes can be developed after PPV even without ILM peeling and are usually managed conservatively by observation.

SURGICAL REMOVAL OF EPIRETINAL MEMBRANE WITH AND WITHOUT REMOVAL OF INTERNAL LIMITING MEMBRANE: Comparative Study of Visual Acuity, Features of Optical Coherence Tomography, and Recurrence Rate.

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De Novelli, Fernando J; Goldbaum, Mauro; Monteiro, Mario L R; Bom Aggio, Fabio; Takahashi, Walter Y

2017-12-05

To study and compare visual acuity, foveal thickness, outer limiting layer, ellipsoid zone, and recurrence rate in patients undergoing removal of the epiretinal membrane with and without the removal of the internal limiting membrane (ILM). Sixty-three patients who had the epiretinal membrane removed by a single surgeon were randomly assigned into 2 groups: Group 1 without additional removal of the ILM and Group 2 with removal of the ILM. Patients were followed up and evaluated at the first month, third month, and sixth month, postoperatively. Patients from both groups had a gradual improvement in their vision over time. There was no significant difference in the improvement in visual acuity between the two groups. About tomographic assessment of alterations, no significant differences were found between the groups; however, Group 1 had a higher relapse rate (17%) compared with Group 2 (3.6%) (P = 0.09). Epiretinal membrane removal with and without ILM peeling shows similar functional and anatomical improvements, but the group in which the ILM was not removed seemed to have a higher recurrence rate.

Epiretinal membrane surgery for combined hamartoma of the retina and retinal pigment epithelium: role of multimodal analysis

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Bruè C

2013-01-01

Full Text Available Claudia Bruè, Andrea Saitta, Michele Nicolai, Cesare Mariotti, Alfonso GiovanniniOphthalmology, Department of Neuroscience, Marche Polytechnic University, Ancona, ItalyBackground: The purpose of this study was to evaluate the role of spectral domain optical coherence tomography (SD-OCT, MP-1 microperimetry, and fundus autofluorescence imaging for planning surgical procedures in combined hamartomas of the retina and retinal pigment epithelium (CHR-RPE and following epiretinal membrane removal.Methods: In an interventional retrospective case series, six consecutive subjects with CHR-RPE underwent vitrectomy and epiretinal membrane peeling, with 4 years of follow-up. Each underwent complete ophthalmic examination, including best corrected visual acuity, fundus examination, fundus fluorescein angiography, SD-OCT, MP-1, and fundus autofluorescence at one, 6, 12, and 48 months.Results: Six eyes from six subjects with CHR-RPE were studied (mean age 31 ± 14 years. All patients were phakic and five were male (83.3%. Lesions were unilateral, ie, three macular, two juxtapapillary and macular, and one pericentral. Preoperative best corrected visual acuity was 0.3 ± 0.08 Snellen, with significant improvement to 0.9 ± 0.17 Snellen (P = 0.001 at 4 years of follow-up. Mean retinal sensitivity within the central 20° field improved from 16.6 ± 1.84 dB to 18.8 ± 0.96 dB (P = 0.07. There was also a statistically significant reduction in the visual defect (P = 0.04. SD-OCT demonstrated that the epiretinal membranes were completely removed in all but one patient, with significantly decreased macular edema on follow-up at one, 6, 12, and 48 months (P = 0.001. A positive correlation was shown between preoperative macular sensitivity and postoperative best corrected visual acuity. Fundus autofluorescence demonstrated a block in background autofluorescence at the site of the lesion, and hyperautofluorescsence at the edematous retina overlain by the epiretinal

Possible role for fundus autofluorescence as a predictive factor for visual acuity recovery after epiretinal membrane surgery.

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Brito, Pedro N; Gomes, Nuno L; Vieira, Marco P; Faria, Pedro A; Fernandes, Augusto V; Rocha-Sousa, Amândio; Falcão-Reis, Fernando

2014-02-01

To study the potential association between fundus autofluorescence, spectral-domain optical coherence tomography, and visual acuity in patients undergoing surgery because of epiretinal membranes. Prospective, interventional case series including 26 patients submitted to vitrectomy because of symptomatic epiretinal membranes. Preoperative evaluation consisted of a complete ophthalmologic examination, autofluorescence, and spectral-domain optical coherence tomography. Studied variables included foveal autofluorescence (fov.AF), photoreceptor inner segment/outer segment (IS/OS) junction line integrity, external limiting membrane integrity, central foveal thickness, and foveal morphology. All examinations were repeated at the first, third, and sixth postoperative months. The main outcome measures were logarithm of minimal angle resolution visual acuity, fov.AF integrity, and IS/OS integrity. All cases showing a continuous IS/OS line had an intact fov.AF, whereas patients with IS/OS disruption could have either an increased area of foveal hypoautofluorescence or an intact fov.AF, with the latter being associated with IS/OS integrity recovery in follow-up spectral-domain optical coherence tomography imaging. The only preoperative variables presenting a significant correlation with final visual acuity were baseline visual acuity (P = 0.047) and fov.AF grade (P = 0.023). Recovery of IS/OS line integrity after surgery, in patients with preoperative IS/OS disruption and normal fov.AF, can be explained by the presence of a functional retinal pigment epithelium-photoreceptor complex, supporting normal photoreceptor activity. Autofluorescence imaging provides a functional component to the study of epiretinal membranes, complementing the structural information obtained with optical coherence tomography.

Reflectivity and thickness analysis of epiretinal membranes using spectral-domain optical coherence tomography

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Ajay E. Kuriyan

2016-01-01

Full Text Available AIM: To compare thickness and reflectivity spectral domain optical coherence tomography (SD-OCT findings in patients with idiopathic epiretinal membranes (ERMs, before and after ERM peeling surgery, with normal controls. METHODS: A retrospective study analyzed SD-OCTs of eyes with ERMs undergoing ERM peeling surgery by one surgeon from 2008 to 2010 and normal control eyes. SD-OCTs were analyzed using a customized algorithm to measure reflectivity and thickness. The relationship between the SD-OCT findings and best corrected visual acuity (BCVA outcomes was also studied. RESULTS: Thirty-four ERM eyes and 12 normal eyes were identified. Preoperative eyes had high reflectivity and thickness of the group of layers from the internal limiting membrane (ILM to the retinal pigment epithelium (RPE and the group of layers from the ILM to the external limiting membrane (ELM. The values of reflectivity of these two groups of layers decreased postoperatively, but were still higher than normal eyes. In contrast, preoperative eyes had lower reflectivity of two 10×15 pixel regions of interest (ROIs incorporating: 1 ELM + outer nuclear layer (ONL and 2 photoreceptor layer (PRL + RPE, compared to controls. The values of reflectivity of these ROIs increased postoperatively, but were still lower than normal controls. A larger improvement in BCVA postoperatively was correlated with a greater degree of abnormal preoperative reflectivity and thickness findings. CONCLUSION: Quantitative differences in reflectivity and thickness between preoperative, postoperative, and normal SD-OCTs allow assessment of changes in the retina secondary to ERM. Our study identified hyperreflective inner retina changes and hyporeflective outer retina changes in patients with ERMs. SD-OCT quantitative measures of reflectivity and/or thickness of specific groups of retinal layers and/or ROIs correlate with improvement in BCVA.

Gene Expression of IGF1, IGF1R, and IGFBP3 in Epiretinal Membranes of Patients with Proliferative Diabetic Retinopathy: Preliminary Study

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Dorota Romaniuk

2013-01-01

Full Text Available The molecular mechanism formation of secondary epiretinal membranes (ERMs after proliferative diabetic retinopathy (PDR or primary idiopathic ERMs is still poorly understood. Therefore, the present study focused on the assessment of IGF1, IGF1R, and IGFBP3 mRNA levels in ERMs and PBMCs from patients with PDR. The examined group comprised 6 patients with secondary ERMs after PDR and the control group consisted of 11 patients with idiopathic ERMs. Quantification of IGF1, IGF1R, and IGFBP3 mRNAs was performed by real-time QRT-PCR technique. In ERMs, IGF1 and IGF1R mRNA levels were significantly higher in patients with diabetes compared to control subjects. In PBMCs, there were no statistically significant differences of IGF1, IGF1R, and IGFBP3 expression between diabetic and nondiabetic patients. In conclusion, our study indicated IGF1 and IGF1R differential expression in ERMs, but not in PBMCs, of diabetic and nondiabetic patients, suggesting that these factors can be involved in the pathogenesis or progression of proliferative vitreoretinal disorders. This trial is registered with NCT00841334.

20 g PPV with indocyanine green-assisted ILM peeling versus 23 g PPV with brilliant blue G-assisted ILM peeling for epiretinal membrane.

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Manousaridis, Kleanthis; Peter, Silvia; Mennel, Stefan

2016-06-01

To compare the anatomical and visual outcomes of 20 gauge (g) pars plana vitrectomy (PPV) with indocyanine green (ICG)-assisted internal limiting membrane (ILM) peeling and 23 g PPV with brilliant blue G (BBG)-assisted ILM peeling for idiopathic epiretinal membrane (ERM). 38 eyes of 38 patients with idiopathic ERM were included. They were divided in two groups: group 1 (18 eyes) underwent 20 g PPV with ICG-assisted ILM peeling and group 2 (20 eyes) 23 g PPV with BBG-assisted ILM peeling. Postoperative best-corrected visual acuity (BCVA) and central macular thickness (CMT) were compared. Average BCVA in group 1 improved significantly from 0.60 logarithm of the minimal angle of resolution (log MAR) at baseline to 0.3 log MAR postoperatively. Average BCVA in group 2 improved significantly from 0.60 log MAR at baseline to 0.3 log MAR postoperatively. Mean CMT reduced significantly from 473 to 375 μm in group 1 and from 486 to 396 μm in group 2. There were no significant differences in the BCVA and CMT between the groups. Both surgical methods appeared to be safe and provided similar anatomical and visual outcomes.

Functional recovery following vitreoretinal surgery

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Scheerlinck, LME

2016-01-01

The general aim of this thesis was to evaluate functional outcome following vitreoretinal surgery for idiopathic epiretinal membranes (iERM) and rhegmatogenous retinal detachment (RRD). Idiopathic epiretinal membrane Pars plana vitrectomy with removal of the iERM is considered to be the standard

Corneal endothelial cell loss and corneal biomechanical characteristics after two-step sequential or combined phaco-vitrectomy surgery for idiopathic epiretinal membrane

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Hamoudi, Hassan; Christensen, Ulrik Correll; La Cour, Morten

2017-01-01

with non-contact specular microscopy. Pachymetry [central cornea thickness (CCT)], keratometry and cornea volume (CV) were measured with Pentacam Scheimpflug camera. Primary outcome was change in CED after 12 months; secondary outcomes were changes in CCT and CV after 12 months. RESULTS: Sixty-two eyes......PURPOSE: To assess the impact of sequential and combined surgery [cataract surgery and 23-gauge pars plana vitrectomy (PPV) with peeling] on corneal endothelium cell density (CED) and corneal biomechanical characteristics. METHODS: Phakic eyes with epiretinal membrane (ERM) were prospectively...... allocated to (i) cataract surgery and subsequent PPV (CAT group), (ii) PPV and subsequent cataract surgery (VIT group) or (iii) phacovitrectomy (COMBI group). Eyes were examined at baseline, 1 month after each surgery, and at 3 and 12 months follow-up. Corneal endothelium cell density (CED) was assessed...

Value of internal limiting membrane peeling in surgery for idiopathic macular hole stage 2 and 3: a randomised clinical trial

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Christensen, U C; Krøyer, K; Sander, B

2009-01-01

AIM: To determine the effect of internal limiting membrane (ILM) peeling on anatomical and functional success rates in stage 2 and 3 idiopathic macular hole surgery (MHS). METHODS: Randomised clinical trial of stage 2 and 3 idiopathic macular hole without visible epiretinal fibrosis and with less...... than 1 year's duration of symptoms. Eyes were randomised to (1) vitrectomy alone without retinal surface manipulation, (2) vitrectomy plus 0.05% isotonic Indocyanine Green (ICG)-assisted ILM peeling or (3) vitrectomy plus 0.15% Trypan Blue (TB)-assisted ILM peeling. Main outcomes were hole closure...... after 3 and 12 months and best-corrected visual acuity after 12 months. RESULTS: 78 eyes were enrolled. Primary closure rates were significantly higher with ILM peeling than without peeling for both stage 2 holes (ICG peeling 100%, non-peeling 55%, p = 0.014) and for stage 3 holes (ICG peeling 91%, TB...

Retinal thickness after vitrectomy and internal limiting membrane peeling for macular hole and epiretinal membrane

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Kumagai, Kazuyuki; Ogino, Nobuchika; Furukawa, Mariko; Hangai, Masanori; Kazama, Shigeyasu; Nishigaki, Shirou; Larson, Eric

2012-01-01

Purpose To determine the retinal thickness (RT), after vitrectomy with internal limiting membrane (ILM) peeling, for an idiopathic macular hole (MH) or an epiretinal membrane (ERM). Also, to investigate the effect of a dissociated optic nerve fiber layer (DONFL) appearance on RT. Methods A non-randomized, retrospective chart review was performed for 159 patients who had successful closure of a MH, with (n = 148), or without (n = 11), ILM peeling. Also studied were 117 patients who had successful removal of an ERM, with (n = 104), or without (n = 13), ILM peeling. The RT of the nine Early Treatment Diabetic Retinopathy Study areas was measured by spectral domain optical coherence tomography (SD-OCT). In the MH-with-ILM peeling and ERM-with-ILM peeling groups, the RT of the operated eyes was compared to the corresponding areas of normal fellow eyes. The inner temporal/inner nasal ratio (TNR) was used to assess the effect of ILM peeling on RT. The effects of DONFL appearance on RT were evaluated in only the MH-with-ILM peeling group. Results In the MH-with-ILM peeling group, the central, inner nasal, and outer nasal areas of the retina of operated eyes were significantly thicker than the corresponding areas of normal fellow eyes. In addition, the inner temporal, outer temporal, and inner superior retina was significantly thinner than in the corresponding areas of normal fellow eyes. Similar findings were observed regardless of the presence of a DONFL appearance. In the ERM-with-ILM peeling group, the retina of operated eyes was significantly thicker in all areas, except the inner and outer temporal areas. In the MH-with-ILM peeling group, the TNR was 0.86 in operated eyes, and 0.96 in fellow eyes (P peeling group, the TNR was 0.84 in operated eyes, and 0.95 in fellow eyes (P peeling group was 0.98, which was significantly greater than that of the MH-with-ILM peeling group (P peeling group was 0.98, which was significantly greater than that of ERM-with-ILM peeling

Efficacy of vitrectomy and epiretinal membrane peeling in eyes with dry age-related macular degeneration

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Mason, III, John; Patel,Shyam

2015-01-01

John O Mason III,1,2 Shyam A Patel11Department of Ophthalmology, University of Alabama School of Medicine, Birmingham, AL, USA; 2Retina Consultants of Alabama, Callahan Eye Foundation Hospital, Birmingham, AL, USAObjective: To study the efficacy of epiretinal membrane (ERM) peeling in eyes with dry age-related macular degeneration (AMD).Methods: We retrospectively analyzed patient charts on 17 eyes (16 patients) that underwent ERM peeling with a concurrent diagnosis of dry AMD.Results: Eyes w...

Retinal Changes Induced by Epiretinal Tangential Forces

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Mario R. Romano

2015-01-01

Full Text Available Two kinds of forces are active in vitreoretinal traction diseases: tangential and anterior-posterior forces. However, tangential forces are less characterized and classified in literature compared to the anterior-posterior ones. Tangential epiretinal forces are mainly due to anomalous posterior vitreous detachment (PVD, vitreoschisis, vitreopapillary adhesion (VPA, and epiretinal membranes (ERMs. Anomalous PVD plays a key role in the formation of the tangential vectorial forces on the retinal surface as consequence of gel liquefaction (synchysis without sufficient and fast vitreous dehiscence at the vitreoretinal interface. The anomalous and persistent adherence of the posterior hyaloid to the retina can lead to vitreomacular/vitreopapillary adhesion or to a formation of avascular fibrocellular tissue (ERM resulting from the proliferation and transdifferentiation of hyalocytes resident in the cortical vitreous remnants after vitreoschisis. The right interpretation of the forces involved in the epiretinal tangential tractions helps in a better definition of diagnosis, progression, prognosis, and surgical outcomes of vitreomacular interfaces.

Refractive changes after lens-sparing vitrectomy for macular hole and epiretinal membrane

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Muto T

2017-08-01

Full Text Available Tetsuya Muto,1 Tomoharu Nishimura,1 Takefumi Yamaguchi,2 Makoto Chikuda,1 Shigeki Machida1 1Department of Ophthalmology, Dokkyo Medical University Koshigaya Hospital, Koshigaya, Japan; 2Department of Ophthalmology, Tokyo Dental College Ichikawa General Hospital, Ichikawa, Japan Purpose: Cataract progression after lens-sparing vitrectomy might differ according to original posterior segment diseases. Our objective was to analyze the refractive values after lens-sparing vitrectomy for macular hole (MH and epiretinal membrane (ERM. Materials and methods: We reviewed the medical records of 25 MH patients (25 eyes and 23 ERM patients (23 eyes who underwent lens-sparing vitrectomy. Refractive changes in both groups were compared. All patients underwent 20-gauge three-port pars plana vitrectomy. Fluid–air exchange was performed during vitrectomy only in the MH group. The results were analyzed using the unpaired t-test, chi-square test, or Fisher exact probability test, and multivariate analysis. Results: There were no significant differences in the patient’s age (P=0.45. The myopia progression rate (D/month was higher in the MH group after surgery than that in the ERM group (P=0.035. MH group had more females (P=0.043, longer surgical time (P<0.001, and higher frequencies of surgical adjuvants use (triamcinolone acetonide, P=0.019; brilliant blue G, P<0.001. The myopia progression rate in the MH group (R=0.568, P<0.001 correlated with female gender. However, no correlation was observed between longer surgical time and the use of surgical adjuvants. Conclusion: The rate of myopia progression was higher in the MH group. Fluid–air exchange and gender may affect the rate of myopia progression. Keywords: cataract, vitrectomy, macular hole, epiretinal membrane

CHARACTERISTICS OF EPIRETINAL MEMBRANE REMNANT EDGE BY OPTICAL COHERENCE TOMOGRAPHY AFTER PARS PLANA VITRECTOMY.

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Gaber, Raouf; You, Qi Sheng; Muftuoglu, Ilkay Kilic; Alam, Mostafa; Tsai, Frank F; Mendoza, Nadia; Freeman, William R

2017-11-01

To evaluate the incidence, characteristics, and the progression of epiretinal membrane (ERM) remnant edge seen by optical coherence tomography after ERM peeling. A retrospective chart review was conducted for 86 eyes of 85 consecutive patients who were diagnosed with ERM and underwent pars plana vitrectomy for epiretinal membrane peeling between 2013 and 2014. Data collected and analyzed included age, gender, preoperative and postoperative visual acuity, use of indocyanine green dye to stain internal limiting membrane, tamponade used after vitrectomy, ERM edge boundaries, presence of cystoid macular edema, and central foveal thickness. An ERM remnant edge was detected in 33/86 study eyes (38.4%) at the first postoperative optical coherence tomography scan. Compared with those without an ERM remnant, patients with an ERM remnant after surgery were significantly older at baseline and had a higher incidence of ERM recurrence at their last visit. They were not significantly different in terms of gender, preoperative and postoperative visual acuity, reduction of central foveal thickness from baseline, proportion of eyes with preoperative ERM elevation on optical coherence tomography, presence of macular edema before surgery, intraoperative use of indocyanine green staining for ILM peeling, or tamponade used. Based on the edge morphology, we classified the ERM remnant into three types: Type 1 was flat and blended with the retina (14/33 eyes, 42.4%), Type 2 was flat but stepped (17/33 eyes, 51.5%), and Type 3 was elevated (2/33 eyes, 6.0%). A significantly higher risk of ERM recurrence was seen in Type 2 and Type 3 ERM remnants (75% and 100%, respectively) than Type 1 ERM remnants (10%). An ERM remnant edge was detected by optical coherence tomography after ERM peeling in 38.4% of eyes. The presence of a postoperative ERM edge was associated with a higher risk of ERM recurrence, particularly in Type 2 and Type 3 ERM remnants.

PERIPAPILLARY RETINAL NERVE FIBER THICKNESS CHANGES AFTER VITRECTOMY FOR EPIRETINAL MEMBRANE IN EYES WITH AND WITHOUT VITREOUS DETACHMENT.

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Mariotti, Cesare; Nicolai, Michele; Longo, Antonio; Viti, Francesca; Bambini, Elisa; Saitta, Andrea; Pirani, Vittorio; Orsini, Emanuele; Baruffa, Daniela; Reibaldi, Michele

2017-12-01

To compare the changes in postoperative peripapillary retinal nerve fiber layer (p-RNFL) thickness after vitrectomy for epiretinal membrane in eyes with preexisting posterior vitreous detachment (PVD) and eyes with surgically induced PVD. This study included consecutive patients who underwent 25-gauge vitrectomy for epiretinal membrane. Eyes were divided, according to intraoperative PVD status, into a preexisting PVD group and surgically induced PVD group. Best-corrected visual acuity, p-RNFL thickness, and central retinal thickness were performed before and at 1, 3, and 6 months after surgery. One hundred and twenty eyes of 120 patients were enrolled: 64 eyes in the preexisting PVD group and 56 eyes in the surgically induced PVD group. In the preexisting PVD group at 6 months, the mean global p-RNFL thickness did not change, whereas it was reduced in the temporal sector (P = 0.034). In the surgically induced PVD group at 6 months, significant decreases were observed in global p-RNFL thickness (P = 0.027), temporal (P = 0.021), temporal inferior (P = 0.030), and nasal inferior sectors (P = 0.010). At 6 months, the two groups differed significantly in temporal (P PVD.

Internal Limiting Membrane Peeling to Prevent Post-vitrectomy Epiretinal Membrane Development in Retinal Detachment.

Science.gov (United States)

Akiyama, Kunihiko; Fujinami, Kaoru; Watanabe, Ken; Tsunoda, Kazushige; Noda, Toru

2016-11-01

To determine the efficacy of internal limiting membrane (ILM) peeling during vitrectomy for rhegmatogenous retinal detachment (RRD) regarding post-vitrectomy epiretinal membrane (ERM) development and visual outcomes. Retrospective, interventional, comparative case series. Setting: Institutional. One hundred and two consecutive eyes with RRD treated with vitrectomy and followed for at least 6 months. ILM was peeled without using dye such as indocyanine green (ICG). Observational Procedures: Patients were divided into 2 groups based on postoperative ERM development: Group 1, 81 eyes without ERM formation; Group 2, 21 eyes with ERM development. Patients also were divided into 2 subgroups: those with and without ILM peeling (58 and 44 eyes, respectively). Statistical analyses were performed between the 2 groups with/without ERM formation and between the 2 subgroups with/without ILM peeling for 5 preoperative factors including foveal involvement of the RRD, 4 intraoperative factors including ILM peeling, baseline best-corrected visual acuity (BCVA), and final BCVA. An association of ILM peeling with ERM prevention and the influence of ILM peeling on visual outcomes. ILM peeling was significantly (P peeling. ILM peeling without ICG staining during the initial vitrectomy for RRDs may prevent postoperative ERM formation with favorable visual outcomes. Copyright © 2016 Elsevier Inc. All rights reserved.

Morphision: A method for subjective evaluation of metamorphopsia in patients with unilateral macular pathology (i.e., full thickness macular hole and epiretinal membrane

Directory of Open Access Journals (Sweden)

Marta Ugarte

2013-01-01

Full Text Available Background: Lack of clinical tests to quantify spatial components of distortion in patients with full thickness macular holes (FTMH and epiretinal membranes (ERM. Aim: To develop a test for subjective evaluation of visual distortion in the central visual field around fixation in patients with unilateral FTMH or ERM. Settings and Design: Prospective case-control study carried out at tertiary referral center. Materials and Methods: Twenty-five patients with unilateral macular disease (13 macular epiretinal membranes, 12 full-thickness macular holes, and nine controls (without ocular pathology underwent ophthalmological examination with logMAR ETDRS visual acuity, near vision and contrast sensitivity assessed. Macular optical coherence tomography and metamorphopsia assessment using Morphision test was also carried out. This test consists of a set of modified Amsler charts for detection, identification, and subjective quantification of visual distortion in the central visual field around fixation. Morphision test content and construct validity, and reliability (test-retest method were evaluated. Sixteen patients completed an unstructured survey on test performance and preference. Results: Every patient with unilateral FTMH or ERM identified a particular chart using Morphision test (content validity. None of the normal subjects without symptoms of metamorphopsia identified any distortion (construct validity. Test-retest showed a 100% consistency for frequency and 67% for amplitude. The mean amplitude difference between measurements was 0.02 degrees (SD = 0.038. The coefficient of repeatability was 0.075. There was a correlation between Morphision amplitude score and visual acuity and contrast sensitivity, individually. Conclusions: Morphision test allowed detection and subjective quantification of metamorphopsia in the clinical setting in our patients with unilateral macular epiretinal membranes and full thickness macular holes.

Effect of internal limiting membrane peeling on the development of epiretinal membrane after pars plana vitrectomy for primary rhegmatogenous retinal detachment.

Science.gov (United States)

Nam, Ki Yup; Kim, Jung Yeul

2015-05-01

To investigate the difference in the occurrence of postoperative epiretinal membranes (ERMs) in vitrectomy for rhegmatogenous retinal detachment with and without peeling of the internal limiting membrane (ILM). The medical records of the 135 patients, who underwent vitrectomy for primary rhegmatogenous retinal detachment from November 2007 to August 2011, were analyzed retrospectively. Of the subjects, 70 patients underwent ILM peeling during the surgery and 65 did not. The best-corrected visual acuity, fundus photograph, and optical coherence tomography were collected 3, 6, and 12 months postoperatively. The relationship between ILM peeling and the preoperative findings of rhegmatogenous retinal detachment and development of a postoperative ERM was analyzed. No ERM occurred in the ILM peeling group, whereas an ERM occurred in 14 of 65 patients who underwent vitrectomy without ILM peeling (21.5%). This difference was significant (P peeling group and was significantly higher 12 months postoperatively (P = 0.03). Internal limiting membrane peeling seems to prevent the occurrence of a postoperative ERM in patients with primary rhegmatogenous retinal detachment.

Five-Year Outcomes of Surgically Treated Symptomatic Epiretinal Membranes With and Without Internal Limiting Membrane Peeling.

Science.gov (United States)

Sultan, Harris; Wykoff, Charles C; Shah, Ankoor R

2018-05-01

The authors evaluated long-term postoperative visual outcomes and recurrence rates following surgery for symptomatic epiretinal membrane (ERM) ± internal limiting membrane (ILM) peeling. This was a retrospective, consecutive case series of 78 patients undergoing vitrectomy for symptomatic ERM between 1/2010 and 4/2012 with follow-up through at least postoperative year 5 (POY5). Outcomes included visual acuity (VA) (Snellen VA converted to logMAR), central retinal thickness (CRT; μm), and ERM recurrence. Subgroup analysis evaluated outcomes related to ILM peeling. Subgroup analysis based on ILM peeling did not find VA (20/50 [0.430 logMAR ± 0.061 logMAR; mean ± SD] vs. 20/60 [0.518 logMAR ± 0.128 logMAR] for ILM vs. non-ILM peeling respectively; P = .513) nor macular thickness (355 μm ± 13 μm vs. 360 μm ± 42 μm; P = .410) to be significantly different at POY5. Recurrence requiring surgery with and without ILM peeling was not statistically significantly different at POY5 (1.6% and 11.8%; P = .118). Vitrectomy for symptomatic ERM led to improved visual and anatomic outcomes with sustained benefit through 5 years. ILM peeling was was associated with reduced ERM recurrence, but this benefit was not statistically significant at POY5. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:296-302.]. Copyright 2018, SLACK Incorporated.

Effects of internal limiting membrane peeling during vitrectomy for macula-off primary rhegmatogenous retinal detachment.

Science.gov (United States)

Blanco-Teijeiro, María José; Bande Rodriguez, Manuel; Mansilla Cuñarro, Raquel; Paniagua Fernández, Laura; Ruiz-Oliva Ruiz, Francisco; Piñeiro Ces, Antonio

2018-03-01

To determine the effectiveness of internal limiting membrane peeling during vitrectomy for macula-off primary rhegmatogenous retinal detachment in the prevention of postoperative epiretinal membrane formation and achievement of good visual outcomes and to identify preoperative and intraoperative risk factors for epiretinal membrane formation. We retrospectively analyzed data from 62 eyes of 62 consecutive patients with macula-off primary rhegmatogenous retinal detachment who underwent vitrectomy with (n = 30) or without (n = 32) internal limiting membrane peeling between January 2014 and March 2016 and were followed up for at least 12 months. The effects of internal limiting membrane peeling on visual outcomes and postoperative recovery of the macular structure were determined. We subsequently divided patients into an epiretinal membrane group and a non-epiretinal membrane group and assessed the effects of various preoperative and intraoperative factors on postoperative epiretinal membrane formation. Postoperative epiretinal membrane developed in 10 patients in the no internal limiting membrane peeling group and three patients in the internal limiting membrane peeling group. Postoperative visual acuity significantly improved in both groups. Epiretinal membrane formation was found to be correlated with a higher number of retinal breaks. Our results suggest that internal limiting membrane peeling during macula-off primary rhegmatogenous retinal detachment surgery can reduce the occurrence of postoperative epiretinal membrane, is safe, and results in favorable visual outcomes.

DETRIMENTAL EFFECTS OF ACTIVE INTERNAL LIMITING MEMBRANE PEELING DURING EPIRETINAL MEMBRANE SURGERY: Microperimetric Analysis.

Science.gov (United States)

Deltour, Jean-Baptiste; Grimbert, Pierre; Masse, Helene; Lebreton, Olivier; Weber, Michel

2017-03-01

The aim of the study was to assess the microperimetric consequences of active internal limiting membrane (ILM) peeling during idiopathic epimacular membrane (IEMM) surgery. This retrospective monocentric study included 32 eyes of 31 consecutive patients who underwent IEMM surgery. Internal limiting membrane integrity was assessed by ILM Blue staining after IEMM removal: peeling was spontaneous (Group S) or active (Group A). Preprocedure and postprocedure (1 and 6 months) examinations were performed using visual acuity determination, spectral domain optical coherence tomography and microperimetry. Twenty-two eyes had an "active ILM peeling" and 10 a "spontaneous ILM peeling." Both groups had comparable and significant improvements in visual acuity 6 months after surgery (+1.82 lines [+9 letters] [Group A] and +1.51 lines [+8 letters] [Group S], P peeling has progressively become generalized in IEMM surgery to reduce recurrences. This additional procedure does not change the postoperative visual acuity but increases the development of deeper microscotomas. The real impact on the quality of vision remains unclear. Active ILM peeling in IEMM surgery may be responsible for visual impairment related to its microtraumatic effects.

Refractive changes after vitrectomy and phacovitrectomy for macular hole and epiretinal membrane

DEFF Research Database (Denmark)

Hamoudi, Hassan; La Cour, Morten

2013-01-01

a role in refraction, including measurement of the axial length, changes in the effective lens position and the anterior chamber depth, the use of intraocular gas tamponade, the formula for intraocular lens (IOL) power calculation, and the IOL type. Most phakic eyes with macular hole or epiretinal...

Internal limiting membrane peeling or not: a systematic review and meta-analysis of idiopathic macular pucker surgery.

Science.gov (United States)

Fang, Xiao-Ling; Tong, Yao; Zhou, Ya-Li; Zhao, Pei-Quan; Wang, Zhao-Yang

2017-11-01

To determine whether internal limiting membrane (ILM) peeling improves anatomical and functional outcomes in idiopathic macular pucker (IMP)/epiretinal membrane (ERM) surgery in this systematic review and meta-analysis. We searched the PubMed, Medline, Web of Science, Cochrane, Ovid MEDLINE, ClinicalTrials.gov and CNKI databases for studies published before 15 September 2016. The eligibility criteria included studies comparing ILM peeling versus no-peeling for IMP surgery. Thirteen articles (10 retrospective cohort studies, 1 prospective cohort study and 2 randomised controlled trials (RCTs)) were included in the review. Primary outcomes: no differences were observed in the best-corrected visual acuity (BCVA) or central macular thickness (CMT) at 12 months; however, lower ERM recurrence (OR, 0.13; 95% CI 0.04 to 0.41; p=0.0004) and reoperation rates (OR, 0.10; 95% CI 0.02 to 0.49; p=0.004) that favoured ILM peeling were observed at the final follow-up. no difference was observed in BCVA at 3, 6 months, the final follow-up or in CMT at 3, 6 months, the final follow-up. Significantly increased CMT, which favoured ILM peeling, was observed at the final follow-up (p=0.002) in the RCTs. ILM peeling yielded greater anatomical success, but no improvement in functional outcomes as the treatment of choice for patients undergoing IMP surgery. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

Efficacy of vitrectomy and epiretinal membrane peeling in eyes with dry age-related macular degeneration.

Science.gov (United States)

Mason, John O; Patel, Shyam A

2015-01-01

To study the efficacy of epiretinal membrane (ERM) peeling in eyes with dry age-related macular degeneration (AMD). We retrospectively analyzed patient charts on 17 eyes (16 patients) that underwent ERM peeling with a concurrent diagnosis of dry AMD. Eyes with concurrent dry AMD and with a good preoperative best-corrected visual acuity (BCVA) (better than or equal to 20/50) had a statistically significant mean BCVA improvement at 6 months after ERM peeling. There was a statistical increase in mean BCVA from 20/95 to 20/56 in dry AMD eyes, and no eyes showed worsening in BCVA at 6 months or at most recent follow-up. Five/seventeen (29.4%) eyes had cataract formation or progression. There were no other complications, reoperations, or reoccurrences. ERM peeling in eyes with dry AMD may show significant improvement, especially in eyes with good preoperative BCVA. The procedure is relatively safe with low complications and reoccurrences.

Inner retinal thinning after Brilliant Blue G-assisted internal limiting membrane peeling for vitreoretinal interface disorders.

Science.gov (United States)

Ambiya, Vikas; Goud, Abhilash; Khodani, Mitali; Chhablani, Jay

2017-04-01

The aim of this study was to evaluate ganglion cell layer and nerve fiber layer thickness after Brilliant Blue G (BBG)-assisted internal limiting membrane (ILM) peeling for vitreomacular disorders. Retrospective analysis of spectral domain optical coherence tomography (SD-OCT) of 42 eyes of 42 patients, who underwent pars plana vitrectomy with BBG-assisted ILM peeling, was performed. Inclusion criteria were idiopathic macular hole, idiopathic vitreomacular traction, and idiopathic epiretinal membrane. Key exclusion criteria were vitreoretinal interface abnormalities secondary to any other diseases, follow-up period of less than 3 months, and any other associated retinal pathology. Average, minimum, and sectoral ganglion cell, and inner plexiform layers (GCIPL) and retinal nerve fiber layer (RNFL) parameters were collected. Changes in these parameters from baseline to 3- and 6-month visits after surgery were analyzed. At 3 months after surgery, we found a statistically significant reduction in the average GCIPL thickness (P = 0.031) and also in the superior sectors (P peeling for vitreomacular interface disorders leads to thinning of the inner retina including GCIPL and RNFL. These structural changes should be correlated with retinal function tests to explore the pros and cons of this surgical step.

Combination therapy with rituximab, low-dose cyclophosphamide, and prednisone for idiopathic membranous nephropathy: a case series.

Science.gov (United States)

Cortazar, Frank B; Leaf, David E; Owens, Charles T; Laliberte, Karen; Pendergraft, William F; Niles, John L

2017-02-01

Membranous nephropathy is a common cause of the nephrotic syndrome. Treatment with standard regimens fails to induce complete remission in most patients. We evaluated the efficacy of combination therapy with rituximab, low-dose, oral cyclophosphamide, and an accelerated prednisone taper (RCP) for the treatment of idiopathic membranous nephropathy. We analyzed 15 consecutive patients with idiopathic membranous nephropathy treated with RCP at Massachusetts General Hospital. Seven patients (47%) received RCP as initial therapy, and the other eight patients (53%) received RCP for relapsing or refractory disease. All patients had at least 1 year of follow-up. The co-primary outcomes were attainment of partial and complete remission. Partial remission was defined as a urinary protein to creatinine ratio (UPCR) RCP resulted in high rates of complete remission. Larger studies evaluating this regimen are warranted.

Kidney injury molecule-1 and neutrophil gelatinase-associated lipocalin as prognostic markers in idiopathic membranous nephropathy.

Science.gov (United States)

Maas, Rutger Jh; van den Brand, Jan Ajg; Waanders, Femke; Meijer, Esther; Goor van, Harry; Peters, Hilde P; Hofstra, Julia M; Wetzels, Jack Fm

2016-01-01

Urinary excretion of alpha-1-microglobulin and beta-2-microglobulin reflects tubular damage and predicts outcome in patients with idiopathic membranous nephropathy with reasonable accuracy. Urinary kidney injury molecule-1 and neutrophil gelatinase-associated lipocalin are novel biomarkers of tubular damage. We investigated if these markers could improve prediction of outcome in idiopathic membranous nephropathy. We measured kidney injury molecule-1 and neutrophil gelatinase-associated lipocalin in urine samples from patients with idiopathic membranous nephropathy, who had nephrotic proteinuria and normal renal function. Excretion of alpha-1-microglobulin and beta-2-microglobulin had been measured previously. Progression was defined as a serum creatinine rise >30%, a rise in serum creatinine to an absolute value of ≥135 µmol/L, or a clinical decision to start immunosuppressive therapy. Remission was defined as proteinuria 50% reduction from baseline. Sixty-nine patients were included. Median follow-up was 35 months (interquartile range 18-63 months). Progression occurred in 30 patients (44%), and spontaneous remission in 36 (52%). Kidney injury molecule-1 and neutrophil gelatinase-associated lipocalin excretion rates were significantly correlated with each other, and with alpha-1-microglobulin and beta-2-microglobulin. The areas under the receiver operating characteristic curves for progression were 0.75 (0.62-0.87) for kidney injury molecule-1 and 0.74 (0.62-0.87) for neutrophil gelatinase-associated lipocalin. In multivariate analysis with either alpha-1-microglobulin and beta-2-microglobulin, kidney injury molecule-1 and neutrophil gelatinase-associated lipocalin did not independently predict outcome. Kidney injury molecule-1 and neutrophil gelatinase-associated lipocalin excretion rates correlated with excretion rates of other tubular damage markers and predicted outcome in patients with idiopathic membranous nephropathy. They did not add prognostic value

Anti-phospholipase A receptor antibodies correlate with clinical status in idiopathic membranous nephropathy

NARCIS (Netherlands)

Hofstra, J.M.; Beck Jr., L.H.; Beck, D.M.; Wetzels, J.F.M.; Salant, D.J.

2011-01-01

BACKGROUND AND OBJECTIVES: Circulating autoantibodies against the M-type phospholipase A(2) receptor (anti-PLA(2)R) were recently identified in the majority of patients in the United States with idiopathic membranous nephropathy (iMN). The objectives of this study were to assess the prevalence of

A Multicenter Randomized Controlled Trial of Rituximab versus Cyclosporine in the Treatment of Idiopathic Membranous Nephropathy (MENTOR).

Science.gov (United States)

Fervenza, Fernando C; Canetta, Pietro A; Barbour, Sean J; Lafayette, Richard A; Rovin, Brad H; Aslam, Nabeel; Hladunewich, Michelle A; Irazabal, Maria V; Sethi, Sanjeev; Gipson, Debbie S; Reich, Heather N; Brenchley, Paul; Kretzler, Matthias; Radhakrishnan, Jai; Hebert, Lee A; Gipson, Patrick E; Thomas, Leslie F; McCarthy, Ellen T; Appel, Gerald B; Jefferson, J Ashley; Eirin, Alfonso; Lieske, John C; Hogan, Marie C; Greene, Eddie L; Dillon, John J; Leung, Nelson; Sedor, John R; Rizk, Dana V; Blumenthal, Samuel S; Lasic, Lada B; Juncos, Luis A; Green, Dollie F; Simon, James; Sussman, Amy N; Philibert, David; Cattran, Daniel C

2015-01-01

Idiopathic membranous nephropathy remains the leading cause of nephrotic syndrome in Caucasian adults. Immunosuppressive therapy with cyclosporine (CSA) is often successful in reducing proteinuria, but its use is associated with a high relapse rate. Rituximab, a monoclonal antibody that specifically targets CD20 on the surface of B-cells, is effective in achieving a complete remission of proteinuria in patients with idiopathic membranous nephropathy. However, whether rituximab is as effective as CSA in inducing and maintaining complete or partial remission of proteinuria in these patients is unknown. The membranous nephropathy trial of rituximab (MENTOR) hypothesizes that B-cell targeting with rituximab is non-inferior to CSA in inducing long-term remission of proteinuria. Patients with idiopathic membranous nephropathy, proteinuria ≥5 g/24 h, and a minimum of 3 months of Angiotensin-II blockade will be randomized into a 12-month treatment period with i.v. rituximab, 1,000 mg (2 infusions, 14 days apart; repeated at 6 months if a substantial reduction in proteinuria (equal to or >25%) is seen at 6 months) or oral CSA 3.5-5 mg/kg/day for 6 months (continued for another 6 months if a substantial reduction in proteinuria (equal to or >25%) is seen at 6 months). The efficacy of treatment will be assessed by the remission status (based on changes in proteinuria) at 24 months from randomization. Patient safety will be assessed via collection of adverse event data and evaluation of pre- and posttreatment laboratory data. At the 6-month post-randomization visit, patients who have been randomized to either CSA or rituximab but who do not have a reduction in proteinuria ≥25% (confirmed on repeat measurements within 2 weeks) will be considered treatment failures and exit the study. This study will test for the first time whether treatment with rituximab is non-inferior to CSA in inducing long-term remission (complete or partial) of proteinuria in patients with idiopathic

Changes in macular pigment optical density after membrane peeling.

Science.gov (United States)

Romano, Mario R; Cennamo, Gilda; Grassi, Piergiacomo; Sparnelli, Federica; Allegrini, Davide; Cennamo, Giovanni

2018-01-01

To highlight the differences in macular pigment optical density (MPOD) between eyes with vitreoretinal interface syndrome and healthy control eyes, to assess the changes in MPOD in eyes treated with macular peeling, to investigate the relationships between MPOD changes and measures of retinal sensitivity such as best corrected visual acuity (BCVA) and microperimetry. In this cross-sectional comparative study, 30 eyes affected by idiopathic epiretinal membrane (iERM, 15eyes) or full-thickness macular hole (FTMH, 15eyes) were compared with 60 eyes from 30 healthy age-matched patients. MPOD values (mean MPOD, maximum MPOD, MPOD area, and MPOD volume) were measured in a range of 4°-7° of eccentricity around the fovea, using the one-wavelength reflectometry method (Visucam 200, Carl-Zeiss Meditec). Patients affected by iERM and FTMH were treated with vitrectomy and epiretinal membrane-inner limiting membrane (ERM-ILM) peeling, with follow-up examinations performed preoperatively and 6 months postoperatively. The main outcome measures were the differences in MPOD between eyes with vitreoretinal interface syndrome and healthy eyes, changes in MPOD after ERM-ILM peeling, and relationships between MPOD and functional changes. Mean MPOD differed significantly between control eyes and those with iERM (P = .0001) or FTMH (P = .0006). The max MPOD and MPOD area increased, but not significantly. After peeling, the only significant change in MPOD was in MPOD volume (P = .01). In the ERM group, postoperative mean MPOD correlated significantly with best-corrected visual acuity (r = .739, P = .002). MPOD was reduced in patients with iERM or FTMH compared with healthy eyes. We found a significant correlation between the mean postoperative MPOD and postoperative BCVA, hypothesizing that the postoperative increase in mean MPOD could be due to a change in distribution for unfolding and expansion of the fovea after the peeling. MOPD may be considered as a prognostic factor associated

Lung transplantation for idiopathic pulmonary arterial hypertension on intraoperative and postoperatively prolonged extracorporeal membrane oxygenation provides optimally controlled reperfusion and excellent outcome.

Science.gov (United States)

Moser, Bernhard; Jaksch, Peter; Taghavi, Shahrokh; Muraközy, Gabriella; Lang, Georg; Hager, Helmut; Krenn, Claus; Roth, Georg; Faybik, Peter; Bacher, Andreas; Aigner, Clemens; Matilla, José R; Hoetzenecker, Konrad; Hacker, Philipp; Lang, Irene; Klepetko, Walter

2018-01-01

Lung transplantation for idiopathic pulmonary arterial hypertension has the highest reported postoperative mortality of all indications. Reasons lie in the complexity of treatment of these patients and the frequent occurrence of postoperative left ventricular failure. Transplantation on intraoperative extracorporeal membrane oxygenation support instead of cardiopulmonary bypass and even more the prolongation of extracorporeal membrane oxygenation into the postoperative period helps to overcome these problems. We reviewed our experience with this concept. All patients undergoing bilateral lung transplantation for idiopathic pulmonary arterial hypertension on intraoperative extracorporeal membrane oxygenation with or without prophylactic extracorporeal membrane oxygenation prolongation into the postoperative period between January 2000 and December 2014 were retrospectively analysed. Forty-one patients entered the study. Venoarterial extracorporeal membrane oxygenation support was prolonged into the postoperative period for a median of 2.5 days (range 1-40). Ninety-day, 1-, 3- and 5-year survival rates for the patient collective were 92.7%, 90.2%, 87.4% and 87.4%, respectively. When compared with 31 patients with idiopathic pulmonary arterial hypertension transplanted in the same period of time without prolongation of extracorporeal membrane oxygenation into the postoperative period, the results compared favourably (83.9%, 77.4%, 77.4%, and 77.4%; P = 0.189). Furthermore, these results are among the best results ever reported for this particularly difficult patient population. Bilateral lung transplantation for idiopathic pulmonary arterial hypertension with intraoperative venoarterial extracorporeal membrane oxygenation support seems to provide superior outcome compared with the results reported about the use of cardiopulmonary bypass. Prophylactic prolongation of venoarterial extracorporeal membrane oxygenation into the early postoperative period provides

Kidney injury molecule-1 and neutrophil gelatinase-associated lipocalin as prognostic markers in idiopathic membranous nephropathy

NARCIS (Netherlands)

Maas, Rutger J. H.; van den Brand, Jan A. J. G.; Waanders, Femke; Meijer, Esther; van Goor, Harry; Peters, Hilde P.; Hofstra, Julia M.; Wetzels, Jack F. M.

Background: Urinary excretion of alpha-1-microglobulin and beta-2-microglobulin reflects tubular damage and predicts outcome in patients with idiopathic membranous nephropathy with reasonable accuracy. Urinary kidney injury molecule-1 and neutrophil gelatinase-associated lipocalin are novel

Postoperative eccentric macular holes after vitrectomy and internal limiting membrane peeling.

Science.gov (United States)

Brouzas, Dimitrios; Dettoraki, Maria; Lavaris, Anastasios; Kourvetaris, Dimitrios; Nomikarios, Nikolaos; Moschos, Marilita M

2017-06-01

The purpose of this study was to describe the incidence, clinical characteristics, and outcome of eccentric macular holes presenting after vitrectomy and internal limiting membrane (ILM) peeling for the treatment of macular pathology and discuss the pathogenesis of holes formation. A retrospective, noncomparative, interventional case-series study of five patients who developed eccentric macular holes postoperatively following vitrectomy in 198 consecutive patients who underwent ILM peeling for idiopathic macular hole and epiretinal membrane formation between 2008 and 2015. Five patients (2.5 %) developed full-thickness eccentric macular holes postoperatively. Three patients presented with a single eccentric macular hole, one patient had an eccentric hole after a failed idiopathic macular hole surgery and one patient developed four eccentric macular holes. The mean diameter of the holes was 584 μm (range 206-1317 μm) and the average time of holes formation after vitrectomy was 27.7 weeks (range 1-140 weeks). Postoperative best-corrected visual acuity ranged from "counting fingers" to 20/25. The eyes with the holes distant from the fovea had the best final visual acuity. No further intervention was attempted and no complications occurred. The mean follow-up time was 26.8 months. The postoperative macular holes after vitrectomy and ILM peeling were variable in number, size, and time of appearance but remained stable and were not associated with any complications. The pathogenesis of macular holes is most consistent with contraction of the residual ILM or secondary epimacular proliferation probably stimulated by ILM peeling.

Single-dose Rituximab Therapy for Refractory Idiopathic Membranous Nephropathy: A Single-center Experience

OpenAIRE

Katsuno, Takayuki; Ozaki, Takenori; Kim, Hangsoo; Kato, Noritoshi; Suzuki, Yasuhiro; Akiyama, Shinichi; Ishimoto, Takuji; Kosugi, Tomoki; Tsuboi, Naotake; Ito, Yasuhiko; Maruyama, Shoichi

2017-01-01

To date, a recognized treatment for refractory membranous nephropathy (MN) has not been established. Recently, several reports have indicated the efficacy of rituximab as a novel treatment option. However, only a few published accounts exist of rituximab therapy for idiopathic MN (IMN) in the Asian population. We present the cases of three IMN patients who were treated with single-dose rituximab after they showed no response to conventional therapies, including corticosteroids, cyclosporine, ...

Novel 'heavy' dyes for retinal membrane staining during macular surgery: multicenter clinical assessment

NARCIS (Netherlands)

Veckeneer, Marc; Mohr, Andreas; Alharthi, Essam; Azad, Rajvardhan; Bashshur, Ziad F.; Bertelli, Enrico; Bejjani, Riad A.; Bouassida, Brahim; Bourla, Dan; Crespo, Iñigo Corcóstegui; Fahed, Charbel; Fayyad, Faisal; Mura, Marco; Nawrocki, Jerzy; Rivett, Kelvin; Scharioth, Gabor B.; Shkvorchenko, Dmitry O.; Szurman, Peter; van Wijck, Hein; Wong, Ian Y.; Wong, David S. H.; Frank, Johannes; Oellerich, Silke; Bruinsma, Marieke; Melles, Gerrit R. J.

2014-01-01

Purpose: To evaluate the feasibility of two novel heavy' dye solutions for staining the internal limiting membrane (ILM) and epiretinal membranes (ERMs), without the need for a prior fluid-air exchange, during macular surgery. Methods: In this prospective nonrandomized multicenter cohort study, the

Extraction of retinal tacks from subjects implanted with an epiretinal visual prosthesis.

Science.gov (United States)

de Juan, Eugene; Spencer, Rand; Barale, Pierre-Olivier; da Cruz, Lyndon; Neysmith, Jordan

2013-10-01

Retinal tacks, first developed for the treatment of complex retinal detachments, have more recently been used for the fixation of epiretinal electrode arrays as part of implanted visual prostheses. Here, we report on the clinical experience of extracting four such tacks after chronic implantation. The ability to safely extract retinal tacks ensures that epiretinal devices can be repositioned or removed if necessary. Custom-built, titanium alloy retinal tacks were mechanically removed from the posterior coats after prolonged implantation (up to 19 months). The resulting wound was characterized by clinical evaluation, fundus photography, and fluorescein angiography while being monitored for stability over time. The wounds were also compared to earlier published reports of the healing response around retinal tacks in human subjects. Tack extraction was accomplished successfully, without complication, in all four subjects. The wound site was readily identified by pale scar tissue. No change in the wound size or appearance was noted over many months of post-operative observation (up to 22 months after explant). No adverse effects on overall ocular health were detected. Extraction of retinal tacks from subjects implanted with epiretinal prostheses can be performed without significant complication. The long-term healing response appears to be stable and localized in eyes afflicted with retinitis pigmentosa or choroideremia. There was also minimal, if any, impact on the local circulatory system. These cases suggest that the use of retinal tacks for anchoring epiretinal visual prostheses does not preclude safe repositioning or removal of the device more than a year after implant.

Abnormal reticuloendothelial function in patients with active vasculitis and idiopathic membranous glomerulopathy

Energy Technology Data Exchange (ETDEWEB)

van der Woude, F J; Piers, D A; van der Giessen, M; Hoedemaeker, P J; Hauw The, T; van der Hem, G K

1983-02-01

Reticuloendothelial function was assessed in 11 patients with systemic lupus erythematosus, 8 patients with Wegener's granulomatosus, and 20 patients with idiopathic membranous sup(99m)Tc-labeled heat-damaged red blood cells. With this method organ uptake could be measured by quantitative scintigraphy. There was no relation between the Tsub(1/2) of the blood disappearance curve and the Tsub(1/2) of the splenic uptake curve. The Tsub(1/2) of the blood disappearance curve was normal in all the patient groups. However, there was a significant shift from spleen to liver uptake in patients with active systemic lupus erythematosus, active Wegener's granulomatosus, and membranous glomerulopathy in comparison with a control group. There was no relation with age, level of circulating immune complexes, complement level, kidney function, or immunosuppressive treatment. We conclude that an increase of the liver component of reticuloendothelial function may compensate abnormalities in splenic function. This stresses the importance of quantitative scanning to detect such abnormalities. The study provides evidence for disease related hyposplenism in patients with active systemical lupus erythematosus, active Wegener's granulamatosus, and membranous glomerulopathy.

Abnormal reticuloendothelial function in patients with active vasculitis and idiopathic membranous glomerulopathy

International Nuclear Information System (INIS)

Woude, F.J. van der; Piers, D.A.; Giessen, M. van der; Hoedemaeker, P.J.; Hauw The, T.; Hem, G.K. van der; Rijksuniversiteit Groningen; Rijksuniversiteit Groningen

1983-01-01

Reticuloendothelial function was assessed in 11 patients with systemic lupus erythematosus, 8 patients with Wegener's granulomatosus, and 20 patients with idiopathic membranous sup(99m)Tc-labeled heat-damaged red blood cells. With this method organ uptake could be measured by quantitative scintigraphy. There was no relation between the Tsub(1/2) of the blood disappearance curve and the Tsub(1/2) of the splenic uptake curve. The Tsub(1/2) of the blood disappearance curve was normal in all the patient groups. However, there was a significant shift from spleen to liver uptake in patients with active systemic lupus erythematosus, active Wegener's granulomatosus, and membranous glomerulopathy in comparison with a control group. There was no relation with age, level of circulating immune complexes, complement level, kidney function, or immunosuppressive treatment. We conclude that an increase of the liver component of reticuloen-dothelial function may compensate abnormalities in splenic function. This stresses the importance of quantitative scanning to detect such abnormalities. The study provides evidence for disaase related hyposplenism in patients with active systemical lupus erythematosus, active Wegener's granulamatosus, and membranous glomerulopathy. (orig.)

Advances in clinical application of optical coherence tomography in vitreomacular interface disease

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Xiao-Li Xing

2013-08-01

Full Text Available Vitreous macular interface disease mainly includes vitreomacular traction syndrome, idiopathic macular epiretinal membrane and idiopathic macular hole. Optical coherence tomography(OCTas a new tool that provides high resolution biopsy cross section image non traumatic imaging inspection, has a unique high resolution, no damage characteristics, and hence clinical widely used, vitreous macular interface for clinical disease diagnosis, differential diagnosis and condition monitoring and quantitative evaluation, treatment options, etc provides important information and reference value. Vitreous macular interface disease in OCT image of anatomical morphology characteristics, improve the clinical on disease occurrence and development of knowledge. We reviewed the advances in the application of OCT in vitreomacular interface disease.

LONG-TERM RETROSPECTIVE ANALYSIS OF VISUAL ACUITY AND OPTICAL COHERENCE TOPOGRAPHIC CHANGES AFTER SINGLE VERSUS DOUBLE PEELING DURING VITRECTOMY FOR MACULAR EPIRETINAL MEMBRANES.

Science.gov (United States)

Jung, Jesse J; Hoang, Quan V; Ridley-Lane, Megan L; Sebrow, Dov B; Dhrami-Gavazi, Elona; Chang, Stanley

2016-11-01

To determine the long-term effect of internal limiting membrane with associated epiretinal membrane (ERM) peeling versus single peeling alone in terms of best-corrected visual acuity and anatomical outcomes on spectral-domain optical coherence tomography. This retrospective comparative cohort study of patients who had follow-up of >1 year and underwent surgery for ERM by a single surgeon (S.C.) from January 1, 2008 to December 31, 2012 compared cases in which the internal limiting membrane was stained with brilliant blue G to facilitate double peeling (n = 42) and single peeling (n = 43) of the ERM alone for up to 3 years of follow-up. For continuous variables, an independent two-tailed t-test was performed. For binary variables, the Fisher's exact test was performed. Statistical significance was defined as P peeling group were more likely to have ERM remaining in the central fovea postoperatively (P = 0.0020, becoming significant by postoperative Year 1, P = 0.022) and less likely to develop inner retinal dimpling (P = 0.000, becoming significant by postoperative Month 3, P = 0.015). At 3 years, central foveal thickness had decreased in the single-peeling group by -136.9 µm and by -84.1 μm in the double-peeling group, which was not significantly different (P = 0.08). Mean best-corrected visual acuity improved in both the groups at all time points. There was no statistically significant difference between the 2 groups at 3 years (P = 0.44; single-peeling group, 0.32 ± 0.42, Snellen 20/42; double-peeling group, 0.23 ± 0.27, Snellen 20/34). Brilliant blue G-assisted internal limiting membrane peeling for ERM results in a more thorough removal of residual ERM around the paracentral fovea. However, there is no difference in long-term best-corrected visual acuity at 3 years and a greater likelihood of inner retinal dimpling.

Epiretinal transplantation of human bone marrow mesenchymal stem cells rescues retinal and vision function in a rat model of retinal degeneration.

Science.gov (United States)

Tzameret, Adi; Sher, Ifat; Belkin, Michael; Treves, Avraham J; Meir, Amilia; Nagler, Arnon; Levkovitch-Verbin, Hani; Rotenstreich, Ygal; Solomon, Arieh S

2015-09-01

Vision incapacitation and blindness associated with incurable retinal degeneration affect millions of people worldwide. In this study, 0.25×10(6) human bone marrow stem cells (hBM-MSCs) were transplanted epiretinally in the right eye of Royal College Surgeons (RCS) rats at the age of 28 days. Epiretinally transplanted cells were identified as a thin layer of cells along vitreous cavity, in close proximity to the retina or attached to the lens capsule, up to 6 weeks following transplantation. Epiretinal transplantation delayed photoreceptor degeneration and rescued retinal function up to 20 weeks following cell transplantation. Visual functions remained close to normal levels in epiretinal transplantation rats. No inflammation or any other adverse effects were observed in transplanted eyes. Our findings suggest that transplantation of hBM-MSCs as a thin epiretinal layer is effective for treatment of retinal degeneration in RCS rats, and that transplanting the cells in close proximity to the retina enhances hBM-MSC therapeutic effect compared with intravitreal injection. Copyright © 2015. Published by Elsevier B.V.

Microvascular changes after vitrectomy with internal limiting membrane peeling: an optical coherence tomography angiography study.

Science.gov (United States)

Mastropasqua, Leonardo; Borrelli, Enrico; Carpineto, Paolo; Toto, Lisa; Di Antonio, Luca; Mattei, Peter A; Mastropasqua, Rodolfo

2017-06-19

To evaluate superficial capillary plexus (SCP) changes occurring after internal limiting membrane (ILM) peeling for the treatment of idiopathic epiretinal membrane (ERM). A total of 15 eyes of 15 patients affected by idiopathic ERM (eight males and seven females; mean age 59.8 ± 9.6 years) were enrolled in the study. Patients were treated with pars plana vitrectomy followed by ERM and ILM peeling. Subjects were evaluated at baseline and at the week-1 and month-1 follow-up visits. At each visit, patients were evaluated with a complete ophthalmologic evaluation, which included imaging with optical coherence tomography angiography. Overall, the SCP vessel density was 43.0 ± 3.0% at baseline and was stable throughout the follow-up (40.0 ± 4.0% at week-1 and 41.0 ± 4.0% at month-1 follow-up visits; p = 0.087 and p = 0.426, respectively). Nevertheless, the SCP vessel density was reduced at week-1 visit in both the superior and inferior sectors. In these sectors, the superficial vessel density was still reduced at the month-1 follow-up visit. We observed a reduction in the SCP vessel density occurring after pars plana vitrectomy with ILM peeling. The reduction is referred to those areas where other changes (e.g., swelling of the arcuate nerve fiber layer) have been already described. In theory, superficial vessel density modifications may be due to the direct surgical trauma to the inner retina, where the superficial plexus is contained, during the ILM grasping.

Pars plana vitrectomy combined with internal limiting membrane peeling for recurrent macular edema due to branch retinal vein occlusion after antivascular endothelial growth factor treatments

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Shirakata Y

2016-02-01

Full Text Available Yukari Shirakata,1 Kouki Fukuda,1 Tomoyoshi Fujita,1 Yuki Nakano,1 Hiroyuki Nomoto,2 Hidetaka Yamaji,3 Fumio Shiraga,4 Akitaka Tsujikawa1 1Department of Ophthalmology, Faculty of Medicine, Kagawa University, Miki-cho, 2Nomoto Eye Clinic, Himeji, 3Department of Ophthalmology, Shirai Eye Hospital, Mitoyo, 4Department of Ophthalmology, Okayama University, Okayama, Japan Purpose: To evaluate the anatomic and functional outcomes of pars plana vitrectomy combined with internal limiting membrane peeling for recurrent macular edema (ME due to branch retinal vein occlusion (BRVO after intravitreal injections of antivascular endothelial growth factor (anti-VEGF agents. Methods: Twenty-four eyes of 24 patients with treatment-naive ME from BRVO were treated with intravitreal injections of anti-VEGF agents. Recurred ME was treated with pars plana vitrectomy combined with internal limiting membrane peeling. Results: After the surgery, ME was significantly reduced at 1 month (P=0.031 and the reduction increased with time (P=0.007 at the final visit. With the reduction in ME, treated eyes showed a slow improvement in visual acuity (VA. At the final visit, improvement in VA was statistically significant compared with baseline (P=0.048. The initial presence of cystoid spaces, serous retinal detachment, or subretinal hemorrhage under the fovea, as well as retinal perfusion status, showed no association with VA improvement. However, the presence of epiretinal membrane showed a significant association with the visual recovery. Although eyes without epiretinal membrane showed visual improvement (-0.10±0.32 in logarithm of the minimum angle of resolution [logMAR], eyes with epiretinal membrane showed greater visual improvement (-0.38±0.12 in logMAR, P=0.012. Conclusion: For recurrent ME due to BRVO after anti-VEGF treatment, particularly when accompanied by epiretinal membrane, pars plana vitrectomy combined with internal limiting membrane peeling might be a

[Long-term outcome of vitrectomy combined with internal limiting membrane peeling for idiopathic macular holes].

Science.gov (United States)

Yamamoto, Kaori; Hori, Sadao

2011-01-01

To elucidate the long-term outcome of internal limiting membrane (ILM) peeling on visual function during vitrectomy for idiopathic macular holes using scanning laser ophthalmoscope (SLO) microperimetry. Prospective uncontrolled study. We studied 31 eyes (29 patients) with idiopathic macular holes. All patients underwent vitrectomy with ILM peeling. The SLO microperimetry was performed preoperatively, and once a year for 3 years postoperatively to detect scotomas in and around the macular holes, and both within and in close vicinity to the areas of ILM peeling. Closure of macular holes after one surgery was confirmed in all cases except for 2 with second surgery. The visual acuity by logarithmic minimum angle of resolution (logMAR) averaged 0.71 +/- 0.36 before surgery, 0.23 +/- 0.31 one year, 0.14 +/- 0.27 two years and 0.12 +/- 0.26 three years after surgery. There was significant improvement up to 2 years after the surgery. All scotomas detected before surgery in the holes, and 77.4% of those detected around the holes decreased gradually. No scotomas were detected in or around the area of ILM peeling either before or after surgery. ILM peeling in vitrectomy for idiopathic macular holes successfully improved visual acuity and did not influence retinal sensitivity in and around the area of ILM peeling. The scotomas detected in and around the holes before surgery gradually reduced or disappeared.

Multiple extrafoveal macular holes following internal limiting membrane peeling

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Hussain N

2018-05-01

Full Text Available Nazimul Hussain, Sandip Mitra Department of Ophthalmology, Al Zahra Hospital, Sharjah, United Arab Emirates Objective: Internal limiting membrane (ILM peeling has been the standard of treatment for macular holes. Besides, causing retinal nerve fiber layer surface abnormality, postoperative extrafoveal multiple retinal holes is a rare phenomenon following ILM peeling. We report an unusual complication of eight extrafoveal macular holes occurring following ILM peeling.Case presentation: A 60-year-old male presented with complaints of decreased and distorted vision in the right eye. He was diagnosed as having epiretinal membrane with lamellar macular hole. He underwent 23G pars plana vitrectomy, brilliant blue assisted ILM peeling and fluid gas exchange. Intraoperatively, ILM was found to be adherent to the underlying neurosensory retina. One month after cataract surgery, he underwent YAG capsulotomy in the right eye. He complained of visual distortion. His fundus evaluation in the right eye showed multiple (eight extrafoveal retinal holes temporal to the macula clustered together.Conclusion: This case demonstrated that peeling of ILM, especially when it is adherent to the underlying neurosensory retina, may cause unwanted mechanical trauma to the inner retina. Glial apoptosis and neuronal degeneration may presumably play a role in delayed appearance of multiple (eight extrafoveal macular holes, which has not been reported earlier. Keywords: internal limiting membrane, lamellar macular hole, full thickness macular holes, epiretinal membrane

Changes in metamorphopsia in daily life after successful epiretinal membrane surgery and correlation with M-CHARTS score

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Kinoshita T

2015-02-01

Full Text Available Takamasa Kinoshita,1,2 Hiroko Imaizumi,1 Hirotomo Miyamoto,1 Utako Okushiba,1 Yuki Hayashi,2 Takashi Katome,2 Yoshinori Mitamura2 1Department of Ophthalmology, Sapporo City General Hospital, Sapporo, Japan; 2Department of Ophthalmology, Institute of Health Biosciences, The University of Tokushima Graduate School, Tokushima, Japan Purpose: To determine the correlation between the changes in metamorphopsia in daily life environment and the M-CHARTS scores after epiretinal membrane (ERM removal, and to determine the criterion for determining whether clinically significant changes in the metamorphopsia score have occurred in M-CHARTS. Methods: We studied 65 eyes undergoing vitrectomy for unilateral ERM. Self-administered questionnaires were used to examine the metamorphopsia in their daily life. The degree of metamorphopsia was determined by M-CHARTS. The receiver operating characteristic curve was used to determine the best predictor of the changes in metamorphopsia in daily life. To determine the reproducibility of the M-CHARTS score, another set of 56 eyes with ERM was tested twice on two different days. Results: The postoperative changes in the logarithm of the M-CHARTS score was defined as M2-value. The area under the receiver operating characteristic curve for the M2-value as a predictor of the changes in metamorphopsia in daily life was larger than area under the receiver operating characteristic curve obtained for any other parameter. The optimal cutoff value was -0.4. The 95% limits of agreement between test and retest measurements had a reproducibility of ±0.3 logarithm of the M-CHARTS score. Taking into account not only the reproducibility but also the consistency with the subjective changes, we determined the criterion for clinically significant changes in the M-CHARTS scores as a change of the M2-value by ≥0.4. Conclusion: Evaluating the changes in the M-CHARTS scores in logarithmic form is favorable not only theoretically but also

Visualizing Macular Structures During Membrane Peeling Surgery With an Intraoperative Spectral-Domain Optical Coherence Tomography Device.

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Leisser, Christoph; Hackl, Christoph; Hirnschall, Nino; Luft, Nikolaus; Döller, Birgit; Draschl, Petra; Rigal, Karl; Findl, Oliver

2016-04-01

The aim of this study was to examine the quality of intraoperative visualization of the posterior hyaloid, epiretinal membrane (ERM), inner limiting membrane (ILM), and hyporeflective subfoveal zone with a commercially available, microscope-integrated spectral-domain OCT setup (mi-SD-OCT) (Rescan 700; Carl Zeiss Meditec AG, Germany). Twenty patients prospectively scheduled for pars plana vitrectomy with membrane peeling due to an idiopathic ERM were included. Standard 23-gauge, three-port pars plana vitrectomy with membrane peeling and staining of the ERM with a trypan blue-based chromovitrectomy dye was performed in all cases. Intraoperative SD-OCT was performed before and after peeling and visualization of the posterior hyaloid, ERM, ILM, and presence of subfoveal hyporeflective zones were examined. OCT follow-ups were performed 2 days and 3 months after surgery. The study was approved by the local ethics committee of the city of Vienna. Successful intraoperative visualization of ERM by mi-SD-OCT was possible in all cases. The posterior hyaloid and ILM could not be seen in the mi-SD-OCT scans, whereas an intraoperative subfoveal hyporeflective zone presented in 35% of cases. In 12.5% an independent subfoveal hyporeflective zone presented postoperatively. Visual acuity improved in 93.8% of patients after surgery. mi-SD-OCT appears to be a valuable tool for intraoperative visualization of the ERM and offers immediate visualization of retinal anatomy during peeling. Therefore, it adds to the understanding of intraoperative traumatic changes due to the peeling procedure. Copyright 2016, SLACK Incorporated.

Spontaneous release of epiretinal membrane in a young weight-lifting athlete by presumed central rupture and centrifugal pull

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Mansour AM

2014-11-01

Full Text Available Ahmad M Mansour,1,2 Hana A Mansour,3 J Fernando Arevalo4,5 1Department of Ophthalmology, Rafic Hariri University Hospital, Beirut, Lebanon; 2Department of Ophthalmology, American University of Beirut, Beirut, Lebanon; 3Department of Biology, American University of Beirut, Beirut, Lebanon; 4Retina Department, The King Khaled Eye Specialist Hospital, Riyadh, Kingdom of Saudi Arabia; 5Retina Department, Wilmer Eye Institute, The Johns Hopkins University, Baltimore, MD, USA Abstract: This patient presented for surgery at the age of 32 years, 14 months after his initial complaint of metamorphopsia and visual loss in the right eye. Past tests demonstrated a whitish epiretinal membrane (ERM with translucent stress lines over a thickened macula. Visual acuity was found on last presentation to be normal with minimal alteration on Amsler grid testing. A torn ERM was found in the center with left-over ERM temporally and rolled-over ERM nasally at the site of the epicenter with no posterior vitreous detachment. Visual recovery occurred gradually over several days 2 months prior to presentation apparently following heavy weight-lifting with a sensation of severe eye pressure. Sequential funduscopy and optical coherence tomography scans demonstrated the peeling of an ERM accompanied by normalization of foveal thickness. Valsalva maneuver had put excessive tension on ERM which tore in its center at the weakest line with gradual contraction of the ERM away from the fovea towards the peripapillary area. This is a new mechanism of self-separation of ERM induced by Valsalva. ERM in young subjects is subject to rupture and subsequent separation by tangential traction. There are three mechanisms for spontaneous separation of ERM: 1 posterior vitreous detachment with pulling of ERM by detaching vitreous (most common in adults; 2 the contracting forces of the immature ERM become stronger than its adhesions to the retina resulting in slow tangential traction on the

Vitrectomy with internal limiting membrane (ILM) peeling versus vitrectomy with no peeling for idiopathic full-thickness macular hole (FTMH)

DEFF Research Database (Denmark)

Spiteri Cornish, Kurt; Lois, Noemi; Scott, Neil

2013-01-01

Several observational studies have suggested the potential benefit of internal limiting membrane (ILM) peeling to treat idiopathic full-thickness macular hole (FTMH). However, no strong evidence is available on the potential benefit(s) of this surgical manoeuvre and uncertainty remains among...... vitreoretinal surgeons about the indication for peeling the ILM, whether to use it in all cases or in long-standing and/or larger holes. ...

Two Cases of Severe Degeneration of the Macula Following Vitrectomy with Indocyanine Green-Assisted Internal Limiting Membrane Peeling for Idiopathic Macular Hole

OpenAIRE

Inoue, Junji; Sakuma, Toshiro; Kiyokawa, Masatoshi; Kobayashi, Yasuhiko; Takebayashi, Hiroshi; Mizota, Atsushi; Tanaka, Minoru

2008-01-01

We describe three eyes of two cases of severe degeneration of the macula following vitrectomy with indocyanine green-assisted internal limiting membrane peeling for idiopathic macular hole. We need to remember the possibility of these complications and have to select the procedures that are safest to use for macular hole surgery.

Idiopathic pulmonary fibrosis patient supported with extracorporeal membrane oxygenation for 403 days while waiting for a lung transplant: A case report

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Nao Umei, M.D.

Full Text Available According to the Extracorporeal Life Support Organization, the average duration of veno-venous extracorporeal membrane oxygenation (V-V ECMO in adults with acute respiratory failure is 10.5–13.5 days. Some patients on V-V ECMO may not recover in such a short period of time, and recently, there have been more reports of prolonged V-V ECMO. However, we do not know how long it is feasible to wait for native lung recovery or lung transplant (LTx with the use of ECMO. We describe a patient with acute exacerbation of idiopathic pulmonary fibrosis supported by ECMO for 403 days while waiting for a LTx. In this case, we kept the patient awake, and he was communicating frequently with his family. We changed the membrane oxygenator 23 times and the cannula 10 times without complication. However, we terminated the treatment on day 403 of ECMO because there was no access site for cannula insertion due to blockage by a venous thrombotic occlusion, making it impossible to continue this bridge to lung transplantation. It has become possible to maintain patients on ECMO for extended periods of time, but it is difficult to manage ECMO for more than one year without the development of a more durable lung support system. Keywords: Lung transplantation, Extracorporeal membrane oxygenation, Idiopathic pulmonary fibrosis

Bilateral versus single lung transplant for idiopathic pulmonary fibrosis.

Science.gov (United States)

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

2014-10-01

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

Thickness of the Macula, Retinal Nerve Fiber Layer, and Ganglion Cell Layer in the Epiretinal Membrane: The Repeatability Study of Optical Coherence Tomography.

Science.gov (United States)

Lee, Haeng-Jin; Kim, Min-Su; Jo, Young-Joon; Kim, Jung-Yeul

2015-07-01

To analyze the repeatability of measurements of the thicknesses of the macula, retinal nerve fiber layer (RNFL), and ganglion cell inner plexiform layer (GCIPL) using spectral-domain optical coherence tomography (SD-OCT) in the epiretinal membrane (ERM). The prospective study analyzed patients who visited our retinal clinic from June 2013 to January 2014. An experienced examiner measured the thicknesses twice using macular cube 512 × 128 and optic disc cube 200 × 200 scans. The repeatability of the thicknesses of the macula, RNFL, and GCIPL were compared using the intraclass correlation coefficient (ICC) of two groups based on the central macular thickness (group A, ≤ 450 μm; group B, > 450 μm). A total of 88 patients were analyzed. The average thicknesses of the central macula, RNFL, and GCIPL were 256.5, 96.6, and 84.4 μm, respectively, in the normal fellow eye and 412.3, 94.6, and 56.7 μm in the affected eye. The ICCs of the central macula, RNFL, and GCIPL were 0.995, 0.994, and 0.996, respectively, for the normal fellow eye and 0.991, 0.973, and 0.881 for the affected eye. The average thicknesses of the central macula, RNFL, and GCIPL in group A were 360.9, 93.5, and 63.4 μm, respectively, and the ICCs were 0.997, 0.987, and 0.995. The thicknesses in group B were 489.5, 96.2, and 46.6 μm, respectively, and the ICCs were 0.910, 0.942, and 0.603, significantly lower repeatability compared with group A (P macula.

The Spatial Extent of Epiretinal Electrical Stimulation in the Healthy Mouse Retina

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Zohreh Hosseinazdeh

2017-07-01

Full Text Available Background/Aims: Retinal prostheses use electrical stimulation to restore functional vision to patients blinded by retinitis pigmentosa. A key detail is the spatial pattern of ganglion cells activated by stimulation. Therefore, we characterized the spatial extent of network-mediated electrical activation of retinal ganglion cells (RGCs in the epiretinal monopolar electrode configuration. Methods: Healthy mouse RGC activities were recorded with a micro-electrode array (MEA. The stimuli consisted of monophasic rectangular cathodic voltage pulses and cycling full-field light flashes. Results: Voltage tuning curves exhibited significant hysteresis, reflecting adaptation to electrical stimulation on the time scale of seconds. Responses decreased from 0 to 300 µm, and were also dependent on the strength of stimulation. Applying the Rayleigh criterion to the half-width at half-maximum of the electrical point spread function suggests a visual acuity limit of no better than 20/946. Threshold voltage showed only a modest increase across these distances. Conclusion: The existence of significant hysteresis requires that future investigations of electrical retinal stimulation control for such long-memory adaptation. The spread of electrical activation beyond 200 µm suggests that neighbouring electrodes in epiretinal implants based on indirect stimulation of RGCs may be indiscriminable at interelectrode spacings as large as 400 µm.

Visual Outcomes of Pars Plana Vitrectomy with Epiretinal Membrane Peeling in Patients with Asteroid Hyalosis: A Matched Cohort Study.

Science.gov (United States)

Mouna, Ali; Berrod, Jean-Paul; Conart, Jean-Baptiste

2017-01-01

The study aimed to evaluate outcomes of epiretinal membrane (ERM) peeling in patients with asteroid hyalosis (AH) and to compare them with those from controls without AH. This is a retrospective matched cohort study of 1,104 consecutive patients who underwent surgery for ERM between January 2004 and February 2014. Patients with AH were included in the study group and were matched for preoperative visual acuity, age, gender, date of surgery, and axial length with control patients without AH selected from the same cohort. The best-corrected visual acuity (BCVA) and central macular thickness (CMT) on optical coherence tomography were measured at baseline and postoperatively with a minimum follow-up period of 12 months. A total of 44 patients were included in the AH group and 44 in the control group. The mean initial BCVA was 0.53 ± 0.21 LogMAR for the AH group vs. 0.49 ± 0.20 LogMAR for the control group, and the mean initial CMT was 419 ± 74 vs. 423 ± 75 µm, respectively. During the follow-up, no significant difference was found regarding the final BCVA at 6 months (0.23 ± 0.14 vs. 0.24 ± 0.17) LogMAR (p = 0.87) and 12 months (0.16 ± 0.09 vs. 0.17 ± 0.12) LogMAR (p = 0.92), despite a tendency toward slower visual recovery for the AH group at 1 month, with a mean BCVA of 0.36 ± 0.12 vs. 0.28 ± 0.18 LogMAR (p = 0.08). No difference was found regarding the progression of CMT at 1.6 and 12 months with a mean CMT of 396 ± 47 vs. 378 ± 55 µm (p = 0.39), 356 ± 39 vs. 365 ± 41 µm (p = 0.48), and 349 ± 68 vs. 352 ± 53 µm (p = 0.87), respectively. Vitrectomy with ERM peeling in patients with AH was beneficial and showed similar functional and anatomical outcomes in both groups. AH does not seem to affect visual improvement or the complication rate after ERM peeling. Therefore, the indications for vitrectomy in case of ERM should not be prompted by the presence of AH. © 2017 S. Karger AG, Basel.

Vitrectomy with Internal Limiting Membrane Peeling versus No Peeling for Idiopathic Full-Thickness Macular Hole

DEFF Research Database (Denmark)

Spiteri Cornish, Kurt; Lois, Noemi; Scott, Neil W

2014-01-01

OBJECTIVE: To determine whether internal limiting membrane (ILM) peeling improves anatomic and functional outcomes of full-thickness macular hole (FTMH) surgery when compared with the no-peeling technique. DESIGN: Systematic review and individual participant data (IPD) meta-analysis undertaken...... under the auspices of the Cochrane Eyes and Vision Group. Only randomized controlled trials (RCTs) were included. PARTICIPANTS AND CONTROLS: Patients with idiopathic stage 2, 3, and 4 FTMH undergoing vitrectomy with or without ILM peeling. INTERVENTION: Macular hole surgery, including vitrectomy and gas...... endotamponade with or without ILM peeling. MAIN OUTCOME MEASURES: Primary outcome was best-corrected distance visual acuity (BCdVA) at 6 months postoperatively. Secondary outcomes were BCdVA at 3 and 12 months; best-corrected near visual acuity (BCnVA) at 3, 6, and 12 months; primary (after a single surgery...

Vascular Displacement in Idiopathic Macular Hole after Single-layered Inverted Internal Limiting Membrane Flap Surgery.

Science.gov (United States)

Lee, Jae Jung; Lee, In Ho; Park, Keun Heung; Pak, Kang Yeun; Park, Sung Who; Byon, Ik Soo; Lee, Ji Eun

2017-08-01

To compare vascular displacement in the macula after surgical closure of idiopathic macular hole (MH) after single-layered inverted internal limiting membrane (ILM) flap technique and conventional ILM removal. This retrospective study included patients who underwent either vitrectomy and ILM removal only or vitrectomy with single-layered inverted ILM flap for idiopathic MH larger than 400 μm from 2012 to 2015. A customized program compared the positions of the retinal vessels in the macula between preoperative and postoperative photographs. En face images of 6 × 6 mm optical coherence tomography volume scans were registered to calculate the scale. Retinal vessel displacement was measured as a vector value by comparing its location in 16 sectors of a grid partitioned into eight sectors in two rings (inner, 2 to 4 mm; outer, 4 to 6 mm). The distance and angle of displacement were calculated as an average vector and were compared between the two groups for whole sectors, inner ring, outer ring, and for each sector. Twenty patients were included in the ILM flap group and 22 in the ILM removal group. There were no statistical differences between the groups for baseline characteristics. The average displacement in the ILM flap group and the ILM removal group was 56.6 μm at -3.4° and 64.9 μm at -2.7°, respectively, for the whole sectors (p = 0.900), 76.1 μm at -1.1° and 87.3 μm at -0.9° for the inner ring (p = 0.980), and 37.4 μm at -8.2° and 42.7 μm at -6.3° for the outer ring (p = 0.314). There was no statistical difference in the displacement of each of the sectors. Postoperative topographic changes showed no significant differences between the ILM flap and the ILM removal group for idiopathic MH. The single-layered ILM flap technique did not appear to cause additional displacement of the retinal vessels in the macula. © 2017 The Korean Ophthalmological Society

Evaluation of a new multifunctional vitreoretinal twister for use in advanced proliferative diabetic retinopathy

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Dikran G Hovaghimian

2016-01-01

Conclusion Epiretinal membrane dissection with the new multifunctional retinal twister appears to be a safe and effective technique in the management of epiretinal membranes. The new multifunctional retinal twister proved its efficacy and success in grasping, holding, twisting, pulling, and dissecting epiretinal membranes; further, the bipolar diathermy attachment for quick management of retinal bleeders was a great advantage for shortening the surgical time and providing a bloodless working field. The encouraging anatomic and functional outcome with the least complications met with during surgery favors its safe use. The twister proved to be a useful adjunct in the armamentarium of retinal microsurgical instruments.

Increase in average foveal thickness after internal limiting membrane peeling

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Kumagai K

2017-04-01

Full Text Available Kazuyuki Kumagai,1 Mariko Furukawa,1 Tetsuyuki Suetsugu,1 Nobuchika Ogino2 1Department of Ophthalmology, Kami-iida Daiichi General Hospital, 2Department of Ophthalmology, Nishigaki Eye Clinic, Aichi, Japan Purpose: To report the findings in three cases in which the average foveal thickness was increased after a thin epiretinal membrane (ERM was removed by vitrectomy with internal limiting membrane (ILM peeling.Methods: The foveal contour was normal preoperatively in all eyes. All cases underwent successful phacovitrectomy with ILM peeling for a thin ERM. The optical coherence tomography (OCT images were examined before and after the surgery. The changes in the average foveal (1 mm thickness and the foveal areas within 500 µm from the foveal center were measured. The postoperative changes in the inner and outer retinal areas determined from the cross-sectional OCT images were analyzed.Results: The average foveal thickness and the inner and outer foveal areas increased significantly after the surgery in each of the three cases. The percentage increase in the average foveal thickness relative to the baseline thickness was 26% in Case 1, 29% in Case 2, and 31% in Case 3. The percentage increase in the foveal inner retinal area was 71% in Case 1, 113% in Case 2, and 110% in Case 3, and the percentage increase in foveal outer retinal area was 8% in Case 1, 13% in Case 2, and 18% in Case 3.Conclusion: The increase in the average foveal thickness and the inner and outer foveal areas suggests that a centripetal movement of the inner and outer retinal layers toward the foveal center probably occurred due to the ILM peeling. Keywords: internal limiting membrane, optical coherence tomography, average foveal thickness, epiretinal membrane, vitrectomy

Smoking is a risk factor for the progression of idiopathic membranous nephropathy.

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Makoto Yamaguchi

Full Text Available BACKGROUND: Multiple studies have shown cigarette smoking to be a risk factor for chronic kidney disease. However, it is unknown whether smoking similarly increases the risk for progression of membranous nephropathy. METHODS: This study used the Nagoya Nephrotic Syndrome Cohort Study (N-NSCS, including 171 patients with idiopathic membranous nephropathy (IMN from 10 nephrology centers in Japan. The dose-response relationships between cigarette smoking and the outcomes were assessed by using multivariate Cox proportional hazards models adjusted for clinically relevant factors. The primary outcome was a 30% decline in the estimated glomerular filtration rate (eGFR. The secondary outcome was first complete remission (CR of proteinuria. RESULTS: During the observation period (median, 37 months; interquartile range, 16-71 months, 37 (21.6% patients developed a 30% decline in eGFR and 2 (1.2% progressed to ESRD. CR occurred in 103 (60.2% patients. Multivariate Cox proportional hazards models revealed current smoking (adjusted hazard ratio [HR], 7.81 [95% confidence interval (CI, 3.17-19.7], female sex (adjusted HR, 3.58 [95% CI, 1.87-8.00], older age (adjusted HR, 1.71 [95% CI, 1.13-2.62] per 10 years, the number of cigarettes smoked daily (adjusted HR, 1.61 [95% CI, 1.23-2.09] per 10 cigarettes daily, and cumulative smoking of ≥40 pack-years (adjusted HR, 5.56 [95% CI, 2.17-14.6] to be associated with a 30% decline in eGFR. However, smoking was not associated with CR. CONCLUSION: Smoking is a significant and dose-dependent risk factor for IMN progression. All patients with IMN who smoke should be encouraged to quit.

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

International Nuclear Information System (INIS)

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

2003-01-01

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

INTRASURGICAL MICROSCOPE-INTEGRATED SPECTRAL DOMAIN OPTICAL COHERENCE TOMOGRAPHY-ASSISTED MEMBRANE PEELING.

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Falkner-Radler, Christiane I; Glittenberg, Carl; Gabriel, Max; Binder, Susanne

2015-10-01

To evaluate microscope-integrated intrasurgical spectral domain optical coherence tomography during macular surgery in a prospective monocenter study. Before pars plana vitrectomy and before, during, and after membrane peeling, 512 × 128 macular cube scans were performed using a Carl Zeiss Meditec Cirrus high-definition OCT system adapted to the optical pathway of a Zeiss OPMI VISU 200 surgical microscope and compared with retinal staining. The study included 51 patients with epiretinal membranes, with 8 of those having additional lamellar macular holes, 11 patients with vitreomacular traction, and 8 patients with full-thickness macular holes. Intraoperative spectral domain optical coherence tomography allowed performing membrane peeling without using retinal dyes in 40% of cases (28 of 70 patients). No residual membranes were found in 94.3% of patients (66 of 70 patients) in intrasurgical spectral domain optical coherence tomography and subsequent (re)staining. In patients with vitreomacular traction, intrasurgical spectral domain optical coherence tomography scans facilitated decisions on the need for an intraocular tamponade after membrane peeling. Intraoperative spectral domain optical coherence tomography was comparable with retinal dyes in confirming success after membrane peeling. However, the visualization of flat membranes was better after staining.

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

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

2010-09-01

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

Clinical outcomes of double staining and additional ILM peeling during ERM surgery.

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Oh, Ha Na; Lee, Joo Eun; Kim, Hyun Woong; Yun, Il Han

2013-08-01

To assess the clinical outcomes in idiopathic epiretinal membrane (ERM) patients after vitrectomy and ERM removal with or without additional indocyanine green (ICG)-assisted internal limiting membrane (ILM) peeling. The medical records of 43 patients with an idiopathic ERM that underwent vitrectomy and ERM removal between July 2007 and April 2010 were reviewed. The patients were divided into two groups: triamcinolone-assisted simple ERM peeling only (group A, n = 23) and triamcinolone-assisted ERM peeling followed by ICG staining and peeling of the remaining internal ILM (group B, n = 20). No difference was found between the two groups in terms of visual acuity, macular thickness, P1 amplitude or implicit time on multifocal-electroretinogram (mfERG) at six and 12 months postoperatively. In group B, ICG staining after ERM peeling demonstrated that the ILM had been removed together with the ERM in 12 eyes (60%), and all 12 eyes showed punctate retinal hemorrhages during ERM peeling. There was no recurrence of an ERM in either group. Additional procedures involving ICG staining and ILM peeling during ERM surgery do not appear to have an additive effect on the clinical outcomes in terms of visual acuity, retinal function based on mfERG, or recurrence rate.

Ten-Year Follow-up of a Blind Patient Chronically Implanted with Epiretinal Prosthesis Argus I.

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Yue, Lan; Falabella, Paulo; Christopher, Punita; Wuyyuru, Varalakshmi; Dorn, Jessy; Schor, Paulo; Greenberg, Robert J; Weiland, James D; Humayun, Mark S

2015-12-01

The Argus I implant is the first-generation epiretinal prosthesis approved for an investigational clinical trial by the United States Food and Drug Administration. Herein we report testing results obtained from a 10-year follow-up to study the physiologic effects of the bioelectronic visual implant after prolonged chronic electrical stimulation. Case report. One man, 55 years of age when enrolled in the study, underwent surgical implantation of the Argus I in June 2004, followed by periodic tests from July 2004 through June 2014, spanning a total of 10 years. The decade-long follow-up consisted of implant system performance tests, subject visual function evaluation, and implant-retina interface analysis. Changes in electrode impedance and perceptual threshold over the time course; subject's performance on visual function task, orientation, and mobility tests; and optical coherence tomography data, fundus imaging, and fluorescein angiography results for the assessment of subject's implant-retina physical interface. Electrically elicited phosphenes were present 10 years after implantation of an epiretinal stimulator. The test subject not only was able to perceive phosphenes, but also could perform visual tasks at rates well above chance. This decade-long follow-up report provides further support for the use of retinal prostheses as a long-lasting treatment for some types of blindness. Copyright © 2015 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.

Functional and Morphological Correlations before and after Video-Documented 23-Gauge Pars Plana Vitrectomy with Membrane and ILM Peeling in Patients with Macular Pucker.

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Mayer, Wolfgang J; Fazekas, Clara; Schumann, Ricarda; Wolf, Armin; Compera, Denise; Kampik, Anselm; Haritoglou, Christos

2015-01-01

Purpose. To assess functional and morphological alterations following video-documented surgery for epiretinal membranes. Methods. Forty-two patients underwent video-documented 23-gauge vitrectomy with peeling of epiretinal (ERM) and inner limiting membrane (ILM). Patient assessment was performed before and 3 and 6 months including best corrected visual acuity (BCVA), slit lamp biomicroscopy, SD-OCT, and central 2° and 18° microperimetry. In addition, all video-documented areas of peeling on the retinal surface were evaluated postoperatively using an additional focal 2° microperimetry. Retinal sensitivity and BCVA were correlated with morphological changes (EZ and ELM) in the foveal region and in regions of membrane peeling. Results. Overall, BCVA increased from 0.6 (±0.2) to 0.2 (±0.2) logMAR after 6 months with an increase in retinal sensitivity (17.9 ± 2.7 dB to 26.8 ± 3.1 dB, p peeling areas (p peeling and overall retinal sensitivity regarding visual acuity gain could be observed after 6 months (p > 0.05). In contrast, overall postoperative retinal sensitivity was significantly decreased in patients with a visual acuity gain lower than 2 lines (p peeling due to the use of intraocular forceps may affect the outer retinal structure. Nevertheless, these changes seem to have no significant impact on postoperative functional outcome.

COMPARISON OF RETINAL PATHOLOGY VISUALIZATION IN MULTISPECTRAL SCANNING LASER IMAGING.

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Meshi, Amit; Lin, Tiezhu; Dans, Kunny; Chen, Kevin C; Amador, Manuel; Hasenstab, Kyle; Muftuoglu, Ilkay Kilic; Nudleman, Eric; Chao, Daniel; Bartsch, Dirk-Uwe; Freeman, William R

2018-03-16

To compare retinal pathology visualization in multispectral scanning laser ophthalmoscope imaging between the Spectralis and Optos devices. This retrospective cross-sectional study included 42 eyes from 30 patients with age-related macular degeneration (19 eyes), diabetic retinopathy (10 eyes), and epiretinal membrane (13 eyes). All patients underwent retinal imaging with a color fundus camera (broad-spectrum white light), the Spectralis HRA-2 system (3-color monochromatic lasers), and the Optos P200 system (2-color monochromatic lasers). The Optos image was cropped to a similar size as the Spectralis image. Seven masked graders marked retinal pathologies in each image within a 5 × 5 grid that included the macula. The average area with detected retinal pathology in all eyes was larger in the Spectralis images compared with Optos images (32.4% larger, P < 0.0001), mainly because of better visualization of epiretinal membrane and retinal hemorrhage. The average detection rate of age-related macular degeneration and diabetic retinopathy pathologies was similar across the three modalities, whereas epiretinal membrane detection rate was significantly higher in the Spectralis images. Spectralis tricolor multispectral scanning laser ophthalmoscope imaging had higher rate of pathology detection primarily because of better epiretinal membrane and retinal hemorrhage visualization compared with Optos bicolor multispectral scanning laser ophthalmoscope imaging.

Idiopathic portal hypertension

International Nuclear Information System (INIS)

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

1996-01-01

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

Vitrectomy with internal limiting membrane peeling versus no peeling for idiopathic full-thickness macular hole.

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Spiteri Cornish, Kurt; Lois, Noemi; Scott, Neil W; Burr, Jennifer; Cook, Jonathan; Boachie, Charles; Tadayoni, Ramin; la Cour, Morten; Christensen, Ulrik; Kwok, Alvin K H

2014-03-01

To determine whether internal limiting membrane (ILM) peeling improves anatomic and functional outcomes of full-thickness macular hole (FTMH) surgery when compared with the no-peeling technique. Systematic review and individual participant data (IPD) meta-analysis undertaken under the auspices of the Cochrane Eyes and Vision Group. Only randomized controlled trials (RCTs) were included. Patients with idiopathic stage 2, 3, and 4 FTMH undergoing vitrectomy with or without ILM peeling. Macular hole surgery, including vitrectomy and gas endotamponade with or without ILM peeling. Primary outcome was best-corrected distance visual acuity (BCdVA) at 6 months postoperatively. Secondary outcomes were BCdVA at 3 and 12 months; best-corrected near visual acuity (BCnVA) at 3, 6, and 12 months; primary (after a single surgery) and final (after >1 surgery) macular hole closure; need for additional surgical interventions; intraoperative and postoperative complications; patient-reported outcomes (PROs) (EuroQol-5D and Vision Function Questionnaire-25 scores at 6 months); and cost-effectiveness. Four RCTs were identified and included in the review. All RCTs were included in the meta-analysis; IPD were obtained from 3 of the 4 RCTs. No evidence of a difference in BCdVA at 6 months was detected (mean difference, -0.04; 95% confidence interval [CI], -0.12 to 0.03; P=0.27); however, there was evidence of a difference in BCdVA at 3 months favoring ILM peeling (mean difference, -0.09; 95% CI, -0.17 to-0.02; P=0.02). There was evidence of an effect favoring ILM peeling with regard to primary (odds ratio [OR], 9.27; 95% CI, 4.98-17.24; Ppeeling was found to be highly cost-effective. Available evidence supports ILM peeling as the treatment of choice for patients with idiopathic stage 2, 3, and 4 FTMH. Copyright © 2014 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.

Idiopathic anaphylaxis.

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Fenny, Nana; Grammer, Leslie C

2015-05-01

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

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

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

2015-01-01

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

Retinal pigment epithelial atrophy following indocyanine green dye-assisted surgery for serous macular detachment

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Hussain Nazimul

2008-01-01

Full Text Available To report subretinal migration of indocyanine green dye (ICG and subsequent retinal pigment epithelial (RPE atrophy during macular surgery for serous macular detachment. A 65-year-old woman presented with residual epiretinal membrane and serous detachment of the macula following vitreoretinal surgery for epiretinal membrane. She underwent resurgery with ICG-assisted internal limiting membrane peeling and intraocular tamponade. Intraoperatively a large area of subretinal ICG was seen with subsequent RPE mottling and atrophy of the macula in the area involved during follow-up. This case demonstrates that subretinal migration of ICG is possible and can be toxic to RPE.

Fibrous membranes in diabetic retinopathy and bevacizumab.

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Pattwell, David M; Stappler, Theodor; Sheridan, Carl; Heimann, Heinrich; Gibran, Syed K; Wong, David; Hiscott, Paul

2010-01-01

The purpose of this study was to determine the histopathologic characteristics of bevacizumab-treated human proliferative diabetic retinopathy (PDR) membranes with particular regard to membrane vasculature as a step toward addressing the effects of the drug on PDR membranes. Intravitreous injection of bevacizumab, an antivascular endothelial growth factor monoclonal antibody, has recently been advocated as an adjunct in surgery for PDR. In this context, a clinically observed decrease in PDR epiretinal membrane vascularity (vascular regression) occurs from 24 hours to 48 hours after injection, but the exact mechanisms of drug action are unknown. A consecutive series of seven PDR membrane specimens that had been removed sequentially from seven bevacizumab-treated patients were studied retrospectively. The membrane specimens were examined using light microscopic methods, including immunohistochemistry. Five of the seven membranes were clinically avascular (one contained "ghost" vessels) and did not hemorrhage during excision. Of these 5 specimens, which included 1 removed 7 days after a total of 6 intravitreous injections of 1.25 mg bevacizumab, 4 contained histologically detectable capillaries (1 did not). These blood vessels were lined by endothelial cells as determined by immunohistochemistry for the endothelial markers CD31 and CD34. The two remaining membranes were clinically and histologically still vascularized despite bevacizumab treatment. All the specimens also contained smooth muscle actin-containing fibroblastic cells within the collagenous stroma. The findings do not support the concept that the clinical phenomenon of vascular regression in PDR membranes after bevacizumab injection in the vitreous is resulting from obliteration of the membrane blood vessels. Another mechanism appears to be involved in at least some patients, possibly a vasoconstrictive response. Such a mechanism might explain reversal of the effects of bevacizumab that has been reported

INDUCTION OF MACULAR DETACHMENT FOR THE TREATMENT OF PERSISTENT OR RECURRENT IDIOPATHIC MACULAR HOLES.

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Szigiato, Andrei-Alexandru; Gilani, Fatimah; Walsh, Mark K; Mandelcorn, Efrem D; Muni, Rajeev H

2016-09-01

To analyze the efficacy of induced macular detachment for the treatment of persistent or recurrent idiopathic macular holes after treatment with one or more standard pars plana vitrectomies (PPVs) with internal limiting membrane peeling. This study is a retrospective consecutive case series of 10 patients who underwent a PPV with subretinal balanced salt solution injection from 2011 to 2014 to treat persistent or recurrent idiopathic macular holes. All patients had previously undergone PPV with internal limiting membrane peeling. Visual acuity, ocular examination findings, and optical coherence tomographic images were reviewed preoperatively and postoperatively to assess the anatomical and visual outcomes of this procedure. Nine of the 10 patients who underwent the procedure had closure of their macular holes postoperatively (90%) and remained closed 6 months postoperatively. Most patients reported a subjective visual improvement. A mean objective visual improvement of 16 letters (Early Treatment Diabetic Retinopathy Study, 0.324 logMAR) was seen between preoperative and 6-month postoperative assessments of all patients (pre = 1.490, post = 1.166; P = 0.022). Subgroup analysis of patients with successful closure revealed 20 letters of improvement (0.398 logMAR) in visual acuity (pre = 1.491, post = 1.093; P = 0.004). There were no intraoperative or postoperative complications. In eyes with persistent or recurrent idiopathic macular holes after standard PPV with internal limiting membrane peeling, repeat PPV with subretinal balanced salt solution injection to create a macular detachment may be a viable surgical treatment option. Our results show improved anatomical and visual outcomes postoperatively that compare favorably to other case series describing various surgical treatments for these challenging cases.

[Clinical features and expression of PLA(2)R in renal tissue with idiopathic membranous nephropathy in children].

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Dong, Y F; Sun, L W; Zhang, B; Kuang, X Y; Niu, X L; Kang, Y L; Hao, S; Wang, P; Li, Z; Zhu, G H; Huang, W Y; Wu, Y

2018-03-02

Objective: To explore the clinical features and expression of PLA(2)R in renal tissue of children with idiopathic membranous nephropathy. Methods: Retrospective study was performed in patients with membranous nephropathy diagnosed through renal biopsy and the follow-up time was at least half a year in Shanghai Children ' s Hospital from January 2010 to February 2017. We compared their clinicopathological and pathological findings of IMN. Indirect immunofluorescence assay was used to detect glomerular PLA(2)R expression. We analyzed the differences of clinical features between the PLA(2)R negative and positive groups. T test, rank-sum test and Fisher exact test were used. Results: Eleven cases had hematuria and proteinuria, 9 cases presented with nephrotic syndrome, and 2 cases showed isolated proteinuria. Of the 22 cases of children with IMN, 16 patients had complete remission (complete remission rate was 72.8%), and 22 patients had partial remission. The renal function of all cases was normal and in all cases the estimated glomerular filtration rate was > 90 ml/(min·1.73m(2)). Of 22 cases with IMN, 7 cases were PLA(2)R-positive in renal tissue and 15 cases were PLA(2)R-negative. The age of positive group (10 years old) was older than the negative group (6 years old)( Z= -2.483, PPLA(2)R in renal tissue of children with IMN was about 32%. The age of PLA(2)R positive group was older than the negative group. And the time of urine protein turning to negative in positive group was longer than that in the negative group.

Treatment of idiopathic FSGS with adrenocorticotropic hormone gel.

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

2013-12-01

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

Adolescent Idiopathic Scoliosis

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Safak Ekinci

2014-06-01

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

Design and validation of a foldable and photovoltaic wide-field epiretinal prosthesis.

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Ferlauto, Laura; Airaghi Leccardi, Marta Jole Ildelfonsa; Chenais, Naïg Aurelia Ludmilla; Gilliéron, Samuel Charles Antoine; Vagni, Paola; Bevilacqua, Michele; Wolfensberger, Thomas J; Sivula, Kevin; Ghezzi, Diego

2018-03-08

Retinal prostheses have been developed to fight blindness in people affected by outer retinal layer dystrophies. To date, few hundred patients have received a retinal implant. Inspired by intraocular lenses, we have designed a foldable and photovoltaic wide-field epiretinal prosthesis (named POLYRETINA) capable of stimulating wireless retinal ganglion cells. Here we show that within a visual angle of 46.3 degrees, POLYRETINA embeds 2215 stimulating pixels, of which 967 are in the central area of 5 mm, it is foldable to allow implantation through a small scleral incision, and it has a hemispherical shape to match the curvature of the eye. We demonstrate that it is not cytotoxic and respects optical and thermal safety standards; accelerated ageing shows a lifetime of at least 2 years. POLYRETINA represents significant progress towards the improvement of both visual acuity and visual field with the same device, a current challenging issue in the field.

Idiopathic chondrolysis - diagnostic difficulties

International Nuclear Information System (INIS)

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

1984-01-01

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

3D finite element modeling of epiretinal stimulation: Impact of prosthetic electrode size and distance from the retina.

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Sui, Xiaohong; Huang, Yu; Feng, Fuchen; Huang, Chenhui; Chan, Leanne Lai Hang; Wang, Guoxing

2015-05-01

A novel 3-dimensional (3D) finite element model was established to systematically investigate the impact of the diameter (Φ) of disc electrodes and the electrode-to-retina distance on the effectiveness of stimulation. The 3D finite element model was established based on a disc platinum stimulating electrode and a 6-layered retinal structure. The ground electrode was placed in the extraocular space in direct attachment with sclera and treated as a distant return electrode. An established criterion of electric-field strength of 1000 Vm-1 was adopted as the activation threshold for RGCs. The threshold current (TC) increased linearly with increasing Φ and electrode-to-retina distance and remained almost unchanged with further increases in diameter. However, the threshold charge density (TCD) increased dramatically with decreasing electrode diameter. TCD exceeded the electrode safety limit for an electrode diameter of 50 µm at an electrode-to-retina distance of 50 to 200 μm. The electric field distributions illustrated that smaller electrode diameters and shorter electrode-to-retina distances were preferred due to more localized excitation of RGC area under stimulation of different threshold currents in terms of varied electrode size and electrode-to-retina distances. Under the condition of same-amplitude current stimulation, a large electrode exhibited an improved potential spatial selectivity at large electrode-to-retina distances. Modeling results were consistent with those reported in animal electrophysiological experiments and clinical trials, validating the 3D finite element model of epiretinal stimulation. The computational model proved to be useful in optimizing the design of an epiretinal stimulating electrode for prosthesis.

Idiopathic Retroperitoneal Hematoma

African Journals Online (AJOL)

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

TOPOGRAPHIC CHANGES OF THE MACULA AFTER CLOSURE OF IDIOPATHIC MACULAR HOLE.

Science.gov (United States)

Pak, Kang Yeun; Park, Keun Heung; Kim, Kyong Ho; Park, Sung Who; Byon, Ik Soo; Kim, Hyun Woong; Chung, In Young; Lee, Joo Eun; Lee, Sang Joon; Lee, Ji Eun

2017-04-01

To investigate retinal displacement in the macula after surgical closure of idiopathic macular hole and to identify factors correlated with displacement. This retrospective multicenter study included 73 eyes of 73 patients having idiopathic macular hole. A custom program was developed to compare the position of the retinal vessels in the macula between preoperative and postoperative photographs. En face images of a 6 mm × 6 mm optical coherence tomography volume scans were registered to calculate the scale. A grid comprising 16 sectors in 2 rings (inner; 2-4 mm and outer; 4-6 mm) was superimposed. The displacement of the retinal vessels was measured as a vector value by comparing the location of the retinal vessels in each sector. The correlation between displacement and various clinical parameters was analyzed. The average displacement was 57.2 μm at an angle of -3.3° (nasal and slightly inferior). Displacement was larger in the inner ring (79.2 μm) than in the outer ring (35.3 μm, P macula was displaced centripetally, nasally, and slightly inferiorly after surgical closure of idiopathic macular hole. Hole closure, contraction of the nerve fiber layer, and gravity are the suggested mechanisms of macular displacement caused by internal limiting membrane peeling.

Genetics Home Reference: adolescent idiopathic scoliosis

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

Multiple extrafoveal macular holes following internal limiting membrane peeling.

Science.gov (United States)

Hussain, Nazimul; Mitra, Sandip

2018-01-01

Internal limiting membrane (ILM) peeling has been the standard of treatment for macular holes. Besides, causing retinal nerve fiber layer surface abnormality, postoperative extrafoveal multiple retinal holes is a rare phenomenon following ILM peeling. We report an unusual complication of eight extrafoveal macular holes occurring following ILM peeling. A 60-year-old male presented with complaints of decreased and distorted vision in the right eye. He was diagnosed as having epiretinal membrane with lamellar macular hole. He underwent 23G pars plana vitrectomy, brilliant blue assisted ILM peeling and fluid gas exchange. Intraoperatively, ILM was found to be adherent to the underlying neurosensory retina. One month after cataract surgery, he underwent YAG capsulotomy in the right eye. He complained of visual distortion. His fundus evaluation in the right eye showed multiple (eight) extrafoveal retinal holes temporal to the macula clustered together. This case demonstrated that peeling of ILM, especially when it is adherent to the underlying neurosensory retina, may cause unwanted mechanical trauma to the inner retina. Glial apoptosis and neuronal degeneration may presumably play a role in delayed appearance of multiple (eight) extrafoveal macular holes, which has not been reported earlier.

Immunosuppressive treatment for nephrotic idiopathic membranous nephropathy: a meta-analysis based on Chinese adults.

Directory of Open Access Journals (Sweden)

Guoqiang Xie

Full Text Available Idiopathic membranous nephropathy (IMN is the most common pathological type for nephrotic syndrome in adults in western countries and China. The benefits and harms of immunosuppressive treatment in IMN remain controversial.To assess the efficacy and safety of different immunosuppressive agents in the treatment of nephrotic syndrome caused by IMN.PubMed, EMBASE, Cochrane Library and wanfang, weipu, qinghuatongfang, were searched for relevant studies published before December 2011. Reference lists of nephrology textbooks, review articles were checked. A meta-analysis of randomized controlled trials (RCTs meeting the criteria was performed using Review Manager.17 studies were included, involving 696 patients. Calcineurin inhibitors had a better effect when compared to alkylating agents, on complete remission (RR 1.61, 95% CI 1.13, to 2.30 P = 0.008, partial or complete remission (effective (CR/PR, RR 1.29, 95% CI 1.09 to 1.52 P = 0.003, and fewer side effects. Among calcineurin inhibitors, tacrolimus (TAC was shown statistical significance in inducing more remissions. When compared to cyclophosphamide (CTX, leflunomide (LET showed no beneficial effect, mycophenolate mofetil (MMF showed significant beneficial on effectiveness (CR/PR, RR: 1.41, 95% CI 1.16 to 1.72 P = 0.0006 but not significant on complete remission (CR, RR: 1.38, 95% CI 0.89 to 2.13 P = 0.15.This analysis based on Chinese adults and short duration RCTs suggested calcineurin inhibitors, especially TAC, were more effective in proteinuria reduction in IMN with acceptable side effects. Long duration RCTs were needed to confirm the long-term effects of those agents in nephrotic IMN.

Evaluation of the dual staining technique for complete removal of idiopathic epimacular membranes

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Ahmed M Abdel Hadi

2015-01-01

In conclusion, dual staining starting with TA effectively led to adequate visualization and removal of the posterior hyaloid as well as the fibrous component of the idiopathic EMM. The subsequent BBG administration in an air filled vitreous (the dry technique helped selective removal of the ILM with no deleterious effects on the functional or the anatomical outcomes of the procedure as demonstrated by the significant improvement in both the BCVA and the CMT 6 months after surgery.

Juvenile Idiopathic Arthritis

Directory of Open Access Journals (Sweden)

Kenan Barut

2017-04-01

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

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

Science.gov (United States)

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

2018-03-07

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

Atypical idiopathic inflammatory demyelinating lesions

DEFF Research Database (Denmark)

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

2013-01-01

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

Retinal displacement toward optic disc after internal limiting membrane peeling for idiopathic macular hole.

Science.gov (United States)

Ishida, Masahiro; Ichikawa, Yoshikazu; Higashida, Rieko; Tsutsumi, Yorihisa; Ishikawa, Atsushi; Imamura, Yutaka

2014-05-01

To examine the retinal displacement following successful macular hole (MH) surgery with internal limiting membrane (ILM) peeling and gas tamponade, and to determine the correlation between the extent of displacement and the basal MH size. Retrospective, interventional, observational case series. The medical records of consecutive patients with an idiopathic MH that had undergone vitrectomy with ILM peeling and gas tamponade were studied. The distances between the optic disc and the intersection of 2 retinal vessels located nasal or temporal to the fovea were measured manually preoperatively (A), and 2 weeks and 1, 3, 6, and 12 months postoperatively (B), on the fundus autofluorescence or near-infrared images. The basal and minimum diameters of the MHs were measured in the spectral-domain optical coherence tomographic images. The correlations between the ratio of the retinal displacement (A - B/A) and basal diameters of the MHs were determined. Twenty-one eyes of 21 patients (9 men, mean age: 64.6 ± 8.4 years) were studied. Ten eyes (47.6%) had stage 2 MH, 9 eyes (42.9%) had stage 3 MH, and 2 eyes (9.5%) had stage 4 MH. The temporal retinal vessels were displaced 260.8 ± 145.8 μm toward the optic disc at 2 weeks postoperatively, which was significantly greater than the 91.1 ± 89.7 μm of the nasal retinal vessels (paired t test, P displacement in the temporal field at 2 weeks was significantly correlated with the basal diameter of the MH (Spearman's rank correlation coeffieient = -0.476, P = .033. The greater displacement of the temporal retina than the nasal retina toward the optic disc postoperatively suggests that the temporal retina is more flexible and can be retracted toward the optic disc during the MH closure. Copyright © 2014 Elsevier Inc. All rights reserved.

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

Science.gov (United States)

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

2014-04-01

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

Idiopathic Intracranial Hypertension (Pseudotumor Cerebri)

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

Idiopathic ventricular tachycardia and fibrillation.

Science.gov (United States)

Belhassen, B; Viskin, S

1993-06-01

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

Antiphospholipase A2 Receptor Autoantibodies: A Comparison of Three Different Immunoassays for the Diagnosis of Idiopathic Membranous Nephropathy

Directory of Open Access Journals (Sweden)

Astrid Behnert

2014-01-01

Full Text Available Background. The recent identification of circulating autoantibodies directed towards the M-type phospholipase A2 receptor (PLA2R has been a major advancement in the serological diagnosis of idiopathic membranous nephropathy (IMN, a common cause of nephrotic syndrome in adults. The goal of this study was to compare the performance characteristics of two commercial assays as well as the first addressable laser bead immunoassay (ALBIA developed for the detection of anti-PLA2R antibodies. Methods. Serum samples of 157 IMN patients and 142 controls were studied. Samples were tested by a cell based immunofluorescence assay (CBA-IFA, Euroimmun, Germany, by ELISA (Euroimmun, and by a novel ALBIA employing an in vivo expressed recombinant human PLA2R. Results. Overall, the three assays showed significant qualitative and quantitative correlation. As revealed by receiver operating characteristic analysis, the ALBIA correlated better with the CBA-IFA than the ELISA (P=0.0003. The clinical sensitivities/specificities for IMN were 60.0% (51.0–68.5%/98.6% (95.0–99.8% and 56.2% (47.2–64.8%/100.0% (97.4–100.0% for ALBIA and CBA-IFA, respectively. Conclusion. The ALBIA represents a promising assay for the detection of anti-PLA2R antibodies showing similar performance to the CBA-IFA and the advantage of ease of use and suitability for high throughput, rapid turnaround times, and multiplexing.

Single night postoperative prone posturing in idiopathic macular hole surgery.

LENUS (Irish Health Repository)

2012-02-01

Purpose. To evaluate the role of postoperative prone posturing for a single night in the outcome of trans pars plana vitrectomy (TPPV) with internal limiting membrane (ILM) peel and 20% perfluoroethane (C2F6) internal tamponade for idiopathic macular hole. Methods. This prospective trial enrolled 14 eyes in 14 consecutive patients with idiopathic macular hole. All eyes underwent TPPV with vision blue assisted ILM peeling with and without phacoemulsification and intraocular lens (IOL) for macular hole. Intraocular gas tamponade (20% C2F6) was used in all cases with postoperative face-down posturing overnight and without specific posturing afterwards. LogMAR visual acuity, appearance by slit-lamp biomicroscopy, and ocular coherence tomography (OCT) scans were compared preoperatively and postoperatively to assess outcome. Results. Among 14 eyes recruited, all eyes were phakic; 50% of patients underwent concurrent phacoemulsification with IOL. The macular holes were categorized preoperatively by OCT appearance, 4 (28.57%) were stage 2, 7 (50%) were stage 3, and 3 (21.43%) were stage 4. Mean macular hole size was 0.35 disk diameters. Symptoms of macular hole had been present for an average of 6.5 months. All holes (100%) were closed 3 and 6 months postoperatively. Mean visual acuity (logMAR) was improved to 0.61 at 3 months and was stable at 6 months after the surgery. None of the eyes had worse vision postoperatively. Conclusions. Vitrectomy with ILM peeling and 20% C2F6 gas with a brief postoperative 1 night prone posturing regimen is a reasonable approach to achieve anatomic closure in idiopathic macular hole. Concurrent cataract extraction did not alter outcomes and was not associated with any additional complications.

Philadelphia Telemedicine Glaucoma Detection and Follow-Up Study

Science.gov (United States)

2017-05-02

Glaucoma; Glaucoma Suspect; Diabetic Retinopathy; Ocular Hypertension; Cataract; Branch Retinal Vein Occlusion; Branch Retinal Arterial Occlusion; Central Retinal Vein Occlusion; Central Retinal Artery Occlusion; Epi-retinal Membrane; Macular Degeneration; Drusen; Loss of Vision

Genetics Home Reference: idiopathic pulmonary fibrosis

Science.gov (United States)

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

Aetiology of idiopathic granulomatous mastitis.

Science.gov (United States)

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

2014-12-16

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

Retinal Damage Induced by Internal Limiting Membrane Removal

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Rachel Gelman

2015-01-01

Full Text Available The internal limiting membrane (ILM, the basement membrane of the Müller cells, serves as the interface between the vitreous body and the retinal nerve fiber layer. It has a fundamental role in the development, structure, and function of the retina, although it also is a pathologic component in the various vitreoretinal disorders, most notably in macular holes. It was not until understanding of the evolution of idiopathic macular holes and the advent of idiopathic macular hole surgery that the idea of adjuvant ILM peeling in the treatment of tractional maculopathies was explored. Today intentional ILM peeling is a commonly applied surgical technique among vitreoretinal surgeons as it has been found to increase the rate of successful macular hole closure and improve surgical outcomes in other vitreoretinal diseases. Though ILM peeling has refined surgery for tractional maculopathies, like all surgical procedures it is not immune to perioperative risk. The essential role of the ILM to the integrity of the retina and risk of trauma to retinal tissue spurs suspicion with regard to its routine removal. Several authors have investigated the retinal damage induced by ILM peeling and these complications have been manifested across many different diagnostic studies.

Membranous nephropathy: A review on the pathogenesis, diagnosis, and treatment

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Wei Ling Lai

2015-02-01

Full Text Available In adults, membranous nephropathy (MN is a major cause of nephrotic syndrome. However, the etiology of approximately 75% of MN cases is idiopathic. Secondary causes of MN are autoimmune diseases, infection, drugs, and malignancy. The pathogenesis of MN involves formation of immune complex in subepithelial sites, but the definite mechanism is still unknown. There are three hypotheses about the formation of immune complex, including preformed immune complex, in situ immune-complex formation, and autoantibody against podocyte membrane antigen. The formation of immune complex initiates complement activation, which subsequently leads to glomerular damage. Recently, the antiphospholipase A2 receptor antibody was found to be associated with idiopathic MN. This finding may be useful in the diagnosis and prognosis of MN. The current treatment includes best supportive care, which consists of the use of angiotensin-converting enzyme inhibitors/angiotensin II receptor blockers, lipid-lowering agents, and optimal control of blood pressure. Immunosuppressive agents should be used for patients who suffer from refractory proteinuria or complications associated with nephrotic syndrome. Existing evidence supports the use of a combination of steroid and alkylating agents. This article reviews the epidemiology, pathogenesis, diagnosis, and the treatment of MN.

Idiopathic pulmonary fibrosis: treatment update.

LENUS (Irish Health Repository)

O'Connell, Oisin J

2011-11-01

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

Retrospective panoramic radiographic analysis for idiopathic osteosclerosis in Indians

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Srikanth H Srivathsa

2016-01-01

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

[Idiopathic facial paralysis in children].

Science.gov (United States)

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

2015-05-01

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

Total fertilization failure and idiopathic subfertility

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Goverde Angelique J

2009-01-01

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

Idiopathic membranous nephropathy in pediatric patients: presentation, response to therapy, and long-term outcome

Directory of Open Access Journals (Sweden)

Valderrama Elsa

2007-08-01

Full Text Available Abstract Background Idiopathic membranous nephropathy (IMN is one of the most common causes of primary nephrotic syndrome in adults. However, it is a relatively rare entity in the pediatric population and there is a paucity of data about the incidence, prognosis, and optimal treatment of IMN in children and adolescents. We conducted this study to evaluate pediatric patients with IMN in order to clarify the presentation, response to therapy, and clinical outcome. Methods A retrospective chart review was performed on patients identified with biopsy-proven IMN between 1988–2005. Patients with systemic lupus erythematosus or hepatitis-related lesions were excluded. The following data were tabulated: age, gender, ethnicity, presenting clinical and laboratory findings, proteinuria in a first morning urine specimen, estimated glomerular filtration rate (GFRe, histopathology, type and duration of treatment, and clinical status at final evaluation. Results 13 cases of IMN were identified out of 460 renal biopsies performed for evaluation of primary kidney disease during the study interval. Mean age was 9.6 ± 4.6, gender 6 M:7 F, ethnicity 8 W:2 B:3 H. At the initial visit hematuria was present in 9 patients, edema in 5, nephrotic-range proteinuria in 5, and hypertension in 3. Mean urinary protein:creatinine ratio 3.3 ± 2.5 and all patients had a normal GFRe. Classic glomerular findings of IMN were seen in all renal specimens, with concomitant interstitial changes in 2 cases. Treatment included an angiotensin converting enzyme inhibitor or angiotensin receptor blocker in 11 cases. Most patients were also given immunosuppressive medications – prednisone in 10, a calcineurin inhibitor in 5, and mycophenolate mofetil or azathioprine in 3 patients. At the last follow-up, 42 ± 35 months after the diagnostic biopsy, 7 children were hypertensive and the urine protein:creatinine ratio was 2.3 ± 3.1. The mean GFRe was 127 ± 57 mL/min/m2. Three patients

Drug therapy for chronic idiopathic axonal polyneuropathy

NARCIS (Netherlands)

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

2004-01-01

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

Evaluation of secondary surgery to enlarge the peeling of the internal limiting membrane following the failed surgery of idiopathic macular holes.

Science.gov (United States)

Che, Xin; He, Fanglin; Lu, Linna; Zhu, Dongqing; Xu, Xiaofang; Song, Xin; Fan, Xianqun; Wang, Zhiliang

2014-03-01

The aim of the present study was to evaluate the clinical results of pars plana vitrectomy (PPV) combined with the surgical enlargement of internal limiting membrane (ILM) peeling in patients who had previously undergone failed idiopathic macular hole (IMH) surgery. In the study, 134 eyes from 130 IMH patients who had received PPV combined with ILM peeling surgery (2 disk diameters) were analyzed. Within this cohort, 14 eyes had IMHs that were not closed, of which 13 eyes underwent a second surgery involving enlargement of the ILM peeling. The extent of the ILM peeling was increased to the vascular arcades of the posterior fundus in the secondary surgery. Of the 13 eyes that underwent secondary surgery, five were in stage III and nine were in stage IV. The second surgery successfully achieved IMH closure in 61.5% (8/13) of the eyes. The IMH was completely closed following surgery and the logMAR vision increased from 0.98 to 0.84 (P=0.013) in the 8 successfully treated cases. The surgical enlargement of ILM peeling closed the IMHs and improved vision in the majority of patients. In addition, the procedures were safe. Therefore, the results of the present study indicate that enlargement of ILM peeling may be an effective therapy for patients who have previously undergone the failed surgical correction of an IMH.

Adolescent Idiopathic Scoliosis

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

Quantitative electromyographic characteristics of idiopathic unilateral vocal fold paralysis.

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

2016-11-01

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

[The temporomandibular joint in juvenile idiopathic arthritis: what radiologists need to look for on magnetic resonance imaging].

Science.gov (United States)

De La Hoz Polo, M; Navallas, M

2014-01-01

The term "juvenile idiopathic arthritis" (JIA) encompasses a group of arthritis of unknown cause with onset before the age of 16 years that last for at least 6 weeks. The prevalence of temporomandibular joint involvement in published series ranges from 17% to 87%. Temporomandibular joint involvement is difficult to detect clinically, so imaging plays a key role in diagnosis and monitoring treatment. MRI is the technique of choice for the study of arthritis of the temporomandibular joint because it is the most sensitive technique for detecting acute synovitis and bone edema. Power Doppler ultrasonography can also detect active synovitis by showing the hypervascularization of the inflamed synovial membrane, but it cannot identify bone edema. This article describes the MRI technique for evaluating the temporomandibular joint in patients with juvenile idiopathic arthritis, defines the parameters to look for, and illustrates the main findings. Copyright © 2013 SERAM. Published by Elsevier Espana. All rights reserved.

Syphilis mimicking idiopathic intracranial hypertension

DEFF Research Database (Denmark)

Yri, Hanne; Wegener, Marianne; Jensen, Rigmor

2011-01-01

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

Idiopathic Gingival Fibromatosis: Case Report and Its Management

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Prashant P. Jaju

2009-01-01

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

Intraspinal anomalies in early-onset idiopathic scoliosis.

Science.gov (United States)

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

2017-06-01

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

Idiopathic epileptic syndromes and cognition.

Science.gov (United States)

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

2006-01-01

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

Idiopathic granulomatous mastitis

International Nuclear Information System (INIS)

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

2009-01-01

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

Angiopoietin-2 polymorphism in women with idiopathic recurrent miscarriage.

Science.gov (United States)

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

2003-10-01

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

[Drusen characteristics after internal limiting membrane peeling].

Science.gov (United States)

Lehmann, F; Jenisch, T; Helbig, H; Gamulescu, M A

2015-05-01

There are some reports showing isolated cases of drusen regression after pars plana vitrectomy (ppV) with peeling of the internal limiting membrane (iLM). Drusen characteristics after iLM peeling was investigated in this study. The data of 527 patients who had received iLM peeling between 2004 and 2012 were retrospectively collected and those patients with retinal drusen were selected for the study. Fundus photographs before and after vitrectomy due to a macular hole or epiretinal gliosis were compared and drusen arrangement in the peeling site was analyzed. The aim of the study was to show whether there was drusen regression 2-5 months after surgery. Out of the 527 patients 11 showed central macular drusen, 4 with confluent large drusen (> 63 µm diameter) and 7 with small hard drusen (≤ 63 µm diameter). One patient showed drusen regression after iLM peeling without any changes in the other eye and all other patients showed no differences in the drusen findings (n = 6) or even some additional drusen (n = 4) without drusen alterations in the other eye. The results of this study could not confirm some reports showing drusen regression after iLM peeling in the peeling site in general and there was only one single case of central drusen regression.

Acute exacerbation of idiopathic pulmonary fibrosis triggered by Aspergillus empyema

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Atsushi Suzuki

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

Aberrant tryptophan transport in cultured fibroblast from patients with Male Idiopathic Osteoporosis: An in vitro study

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Ylva Pernow

2018-06-01

Full Text Available It has been demonstrated, that long-term chronic tryptophan deficiency, results in decreased serotonin synthesis, which may lead to low bone mass and low bone formation. Findings from studies in male patients with idiopathic osteoporosis suggested a decreased transport of tryptophan in erythrocytes of osteoporotic patients, indicating that serotonin system defects may be involved in the etiology of low bone mass. Tryptophan is the precursor of serotonin, and a disturbed transport of tryptophan is implicated in altered serotonin synthesis. However, no study has investigated the tryptophan transport kinetics in MIO patients. The aim of this study is to investigate the kinetic parameters of tryptophan transport in fibroblasts derived from MIO patients compared to age and sex matched controls.Fibroblast cells were cultured from skin biopsies obtained from 14 patients diagnosed with Male Idiopathic Osteoporosis and from 13 healthy age-sex matched controls, without a diagnosis of osteoporosis. Transport of the amino acid tryptophan across the cell membrane was measured by the cluster tray method. The kinetic parameters, maximal transport capacity (Vmax and affinity constant (Km were determined by using the Lineweaver-Burke plot equation.The results of this study have shown a significantly lower mean value for Vmax (p=0.0138 and lower Km mean value (p=0.0009 of tryptophan transport in fibroblasts of MIO patients compared to the control group. A lower Vmax implied a decreased tryptophan transport availability in MIO patients.In conclusion, reduced cellular tryptophan availability in MIO patients might result in reduced brain serotonin synthesis and its endogenous levels in peripheral tissues, and this may contribute to low bone mass/formation. The findings of the present study could contribute to the etiology of idiopathic osteoporosis and for the development of novel approaches for diagnosis, treatment and management strategies of MIO. Keywords: Male

COMPARATIVE ANALYSIS OF RETINAL REATTACHMENT SURGERY WITH OR WITHOUT INTERNAL LIMITING MEMBRANE PEELING TO PREVENT POSTOPERATIVE MACULAR PUCKER.

Science.gov (United States)

Forlini, Matteo; Date, Purva; Ferrari, Luisa Micelli; Lorusso, Massimo; Lecce, Gabriella; Verdina, Tommaso; Neri, Giovanni; Benatti, Caterina; Rossini, Paolo; Bratu, Adriana; DʼEliseo, Domenico; Ferrari, Tommaso Micelli; Cavallini, Gian Maria

2017-07-18

To determine whether internal limiting membrane (ILM) peeling during pars plana vitrectomy for rhegmatogenous retinal detachment reduces the incidence of epiretinal membrane (ERM) formation. In this retrospective study, preoperative, intraoperative, and postoperative data from all eyes undergoing pars plana vitrectomy for rhegmatogenous retinal detachment between January 2007 and December 2013 was analyzed. All cases with at least 1-year of follow-up were included. Data collection included vision, intraoperative complications, occurrence of ERM, and spectral domain optical coherence tomography characteristics. The OCTs were retrieved for all eyes and were graded by a single masked grader. Out of 159 eyes recruited, ILM peeling was done in 78 eyes (49%). Overall occurrence of ERM was 20%. Seven eyes (9%) in ILM peeling group and 25 eyes in the non-ILM peeling group (31%) showed ERM (P = 0.001). Postoperative vision was significantly better in eyes that had ILM peeling (0.48 ± 0.4 logarithm of the minimum angle of resolution [20/63] vs. 0.77 ± 0.6 logarithm of the minimum angle of resolution [20/125], P = 0.003). In multivariable models adjusting for type of tamponade, ILM peeling reduced the likelihood of ERM formation by 75% (P = 0.01). Internal limiting membrane peeling during pars plana vitrectomy for rhegmatogenous retinal detachment significantly reduces ERM formation in the postoperative period and is associated with better visual and anatomical outcomes.

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

Science.gov (United States)

Yamada, S; Ishikawa, M; Yamamoto, K

2016-07-01

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

Intestinal Volvulus in Idiopathic Steatorrhea

Science.gov (United States)

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

1963-01-01

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

Idiopathic scoliosis; a biomechanical and functional anatomical study.

NARCIS (Netherlands)

Veldhuizen, Albert Gerrit

1985-01-01

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

Pathology of idiopathic non-cirrhotic portal hypertension.

Science.gov (United States)

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

2018-04-12

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

Patellofemoral morphometry in patients with idiopathic patellofemoral pain syndrome

International Nuclear Information System (INIS)

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

2010-01-01

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

Idiopathic pulmonary fibrosis.

Science.gov (United States)

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

2017-02-23

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

Evidence for cognitive vestibular integration impairment in idiopathic scoliosis patients

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Mercier Pierre

2009-08-01

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

Optimal management of idiopathic scoliosis in adolescence

Science.gov (United States)

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

2013-01-01

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

Is idiopathic recurrent pancreatitis attributed to small stones?

OpenAIRE

Chow, Wai-Keung; Peng, Yen-Chun

2013-01-01

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

Treatment of the idiopathic scoliosis with brace and physiotherapy.

Science.gov (United States)

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

2009-01-01

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

Correlations between M-CHARTS and PHP findings and subjective perception of metamorphopsia in patients with macular diseases.

Science.gov (United States)

Arimura, Eiko; Matsumoto, Chota; Nomoto, Hiroki; Hashimoto, Shigeki; Takada, Sonoko; Okuyama, Sachiko; Shimomura, Yoshikazu

2011-01-05

To assess the correlations between a patient's subjective perception of metamorphopsia and the clinical measurements of metamorphopsia by M-CHARTS and PreView PHP (PHP). The authors designed a 10-item questionnaire focusing on the symptoms of metamorphopsia and verified its validity with a Rasch analysis. M-CHARTS measured the minimum visual angle of a dotted line needed to detect metamorphopsia, and PHP used the hyperacuity function for detection. Subjects were 39 patients with idiopathic epiretinal membrane (ERM), 22 patients with idiopathic macular hole (M-hole), 19 patients with age-related macular degeneration (AMD), and 51 healthy controls. Rasch analysis suggested the elimination of one question. The nine-item questionnaire score significantly correlated to the M-CHARTS score in ERM (r = 0.59; P = 0.0004) but not in M-hole and to the PHP result in AMD (r = -0.29; P = 0.04) but not in ERM. Eighty percent of ERM patients with greater horizontal M-CHARTS score subjectively perceived horizontal metamorphopsia more often. M-CHARTS showed better sensitivities than PHP in both ERM (89% vs. 42%) and AMD (74% vs. 68%) and better specificity (100% vs. 71%) in healthy controls. Rasch analysis indicated that the present form of the questionnaire is better suited for moderate to severe cases of metamorphopsia than for mild cases. The questionnaire appears to be a valid assessment of patient subjective perception of metamorphopsia and can be used to supplement the clinical measurements of metamorphopsia by M-CHARTS and PHP in patients with macular diseases.

PATTERN OF INHERITANCE OF IDIOPATHIC HYPERCALCIURIA IN TWO FAMILIES

Directory of Open Access Journals (Sweden)

A. Nickavar

2006-09-01

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

Patellofemoral morphometry in patients with idiopathic patellofemoral pain syndrome

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

2010-07-15

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

Guidelines for the medical treatment of idiopathic pulmonary fibrosis.

Science.gov (United States)

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

2017-05-01

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

Family history of idiopathic REM behavior disorder

DEFF Research Database (Denmark)

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

2013-01-01

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

Computed tomography in the assessment of idiopathic spontaneous pneumothorax

International Nuclear Information System (INIS)

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

1991-01-01

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

Idiopathic scrotal elephantiasis.

Science.gov (United States)

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

2005-02-01

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

Understanding idiopathic intracranial hypertension

DEFF Research Database (Denmark)

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

2016-01-01

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

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

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

2009-01-01

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

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

African Journals Online (AJOL)

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

Optimal management of idiopathic scoliosis in adolescence

Directory of Open Access Journals (Sweden)

Kotwicki T

2013-07-01

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

Retinal thinning after internal limiting membrane peeling for idiopathic macular hole.

Science.gov (United States)

Imamura, Yutaka; Ishida, Masahiro

2018-03-01

To determine the changes in retinal thickness and whether they correlate with the size of the macular hole (MH) after vitrectomy with internal limiting membrane peeling. Retrospective, interventional case series METHODS: Consecutive patients with an MH and undergoing pars plana vitrectomy with internal limiting membrane peeling were studied. The retinal thicknesses in the inner 4 sectors as defined by the Early Treatment of Diabetic Retinopathy Study were measured using spectral-domain optical coherence tomography (SD-OCT) before and at 2 weeks and 1, 3, 6, and 12 months after the surgery. The basal and minimum diameters of the MHs were measured. The correlations between the retinal thicknesses and the size of the MH were determined. Thirty-three eyes of 32 consecutive patients (18 women; mean age, 64.2 ± 8.8 years) with an MH were studied. Thirteen eyes had a stage-2 MH; 12 eyes, a stage-3 MH; and 8 eyes, a stage-4 MH. The mean retinal thickness in the temporal sector was 362.8 ± 29.9 µm preoperatively, 337.9 ± 20.6 µm at 2 weeks postoperatively, and 307.6 ± 20.2 µm at 12 months postoperatively (P peeling.

Idiopathic short stature

Directory of Open Access Journals (Sweden)

Vlaški Jovan

2013-01-01

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

Perceived health status in self-reported adolescent idiopathic scoliosis

DEFF Research Database (Denmark)

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

2010-01-01

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

Clinical and Polysomnographic Comparison between Narcolepsy without Cataplexy and Idiopathic Hypersomnia

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Tae Won Kim

2012-10-01

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

Idiopathic linear IgA bullous dermatosis: prognostic factors based on a case series of 72 adults.

Science.gov (United States)

Gottlieb, J; Ingen-Housz-Oro, S; Alexandre, M; Grootenboer-Mignot, S; Aucouturier, F; Sbidian, E; Tancrede, E; Schneider, P; Regnier, E; Picard-Dahan, C; Begon, E; Pauwels, C; Cury, K; Hüe, S; Bernardeschi, C; Ortonne, N; Caux, F; Wolkenstein, P; Chosidow, O; Prost-Squarcioni, C

2017-07-01

Linear IgA bullous dermatosis (LABD) is a clinically and immunologically heterogeneous, subepidermal, autoimmune bullous disease (AIBD), for which the long-term evolution is poorly described. To investigate the clinical and immunological characteristics, follow-up and prognostic factors of adult idiopathic LABD. This retrospective study, conducted in our AIBD referral centre, included adults, diagnosed between 1995 and 2012, with idiopathic LABD, defined as pure or predominant IgA deposits by direct immunofluorescence. Clinical, histological and immunological findings were collected from charts. Standard histology was systematically reviewed, and indirect immunofluorescence (IIF) on salt-split skin (SSS) and immunoblots (IBs) on amniotic membrane extracts using anti-IgA secondary antibodies were performed, when biopsies and sera obtained at diagnosis were available. Prognostic factors for complete remission (CR) were identified using univariate and multivariate analyses. Of the 72 patients included (median age 54 years), 60% had mucous membrane (MM) involvement. IgA IIF on SSS was positive for 21 of 35 patients tested; 15 had epidermal and dermal labellings. Immunoelectron microscopy performed on the biopsies of 31 patients labelled lamina lucida (LL) (26%), lamina densa (23%), anchoring-fibril zone (AFz) (19%) and LL+AFz (23%). Of the 34 IgA IBs, 22 were positive, mostly for LAD-1/LABD97 (44%) and full-length BP180 (33%). The median follow-up was 39 months. Overall, 24 patients (36%) achieved sustained CR, 19 (29%) relapsed and 35% had chronic disease. CR was significantly associated with age > 70 years or no MM involvement. No prognostic immunological factor was identified. Patients with LABD who are < 70 years old and have MM involvement are at risk for chronic evolution. © 2017 British Association of Dermatologists.

Clinical and cost-effectiveness of internal limiting membrane peeling for patients with idiopathic full thickness macular hole. Protocol for a Randomised Controlled Trial: FILMS (Full-thickness macular hole and Internal Limiting Membrane peeling Study

Directory of Open Access Journals (Sweden)

Cook Jonathan

2008-11-01

Full Text Available Abstract Background A full-thickness macular hole (FTMH is a common retinal condition associated with impaired vision. Randomised controlled trials (RCTs have demonstrated that surgery, by means of pars plana vitrectomy and post-operative intraocular tamponade with gas, is effective for stage 2, 3 and 4 FTMH. Internal limiting membrane (ILM peeling has been introduced as an additional surgical manoeuvre to increase the success of the surgery; i.e. increase rates of hole closure and visual improvement. However, little robust evidence exists supporting the superiority of ILM peeling compared with no-peeling techniques. The purpose of FILMS (Full-thickness macular hole and Internal Limiting Membrane peeling Study is to determine whether ILM peeling improves the visual function, the anatomical closure of FTMH, and the quality of life of patients affected by this disorder, and the cost-effectiveness of the surgery. Methods/Design Patients with stage 2–3 idiopathic FTMH of less or equal than 18 months duration (based on symptoms reported by the participant and with a visual acuity ≤ 20/40 in the study eye will be enrolled in this FILMS from eight sites across the UK and Ireland. Participants will be randomised to receive combined cataract surgery (phacoemulsification and intraocular lens implantation and pars plana vitrectomy with postoperative intraocular tamponade with gas, with or without ILM peeling. The primary outcome is distance visual acuity at 6 months. Secondary outcomes include distance visual acuity at 3 and 24 months, near visual acuity at 3, 6, and 24 months, contrast sensitivity at 6 months, reading speed at 6 months, anatomical closure of the macular hole at each time point (1, 3, 6, and 24 months, health related quality of life (HRQOL at six months, costs to the health service and the participant, incremental costs per quality adjusted life year (QALY and adverse events. Discussion FILMS will provide high quality evidence on the

Pulmonary tuberculosis in patients with idiopathic pulmonary fibrosis

Energy Technology Data Exchange (ETDEWEB)

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

2004-11-01

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

Pulmonary tuberculosis in patients with idiopathic pulmonary fibrosis

International Nuclear Information System (INIS)

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

2004-01-01

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

Hypercalcemia in idiopathic myelofibrosis

DEFF Research Database (Denmark)

Voss, A; Schmidt, K; Hasselbalch, H

1992-01-01

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

Repressive coping and alexithymia in idiopathic environmental intolerance

DEFF Research Database (Denmark)

Skovbjerg, Sine; Zachariae, Robert; Rasmussen, Alice

2010-01-01

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

Sleepiness in Idiopathic REM Sleep Behavior Disorder and Parkinson Disease.

Science.gov (United States)

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

2015-10-01

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

Obesity is a significant susceptibility factor for idiopathic AA amyloidosis.

Science.gov (United States)

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

2018-03-01

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

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

African Journals Online (AJOL)

2015-12-03

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

Allelic variation of the FRMD7 gene in congenital idiopathic nystagmus.

Science.gov (United States)

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

2007-09-01

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

Epiretinal proliferation in lamellar macular holes and full-thickness macular holes: clinical and surgical findings.

Science.gov (United States)

Lai, Tso-Ting; Chen, San-Ni; Yang, Chung-May

2016-04-01

To report the clinical findings and surgical outcomes of lamellar macular holes (LMH) with or without lamellar hole-associated epiretinal proliferation (LHEP), and those of full-thickness macular holes (FTMH) presenting with LHEP. From 2009 to 2013, consecutive cases of surgically treated LMH, and all FTMH cases with LHEP were reviewed, given a follow-up time over 1 year. In the LMH group (43 cases), those with LHEP (19 cases) had significantly thinner bases and larger openings than those without (24 cases). The rate of disrupted IS/OS line was higher in the LHEP subgroup preoperatively (68.4 % vs 37.5 %), but similar between subgroups postoperatively (36.8 % and 33.3 %). The preoperative and postoperative visual acuity showed no significant difference between two subgroups. In the FTMH group (13 cases), the average hole size was 219.2 ± 92.1 μm. Permanent or transient spontaneous hole closure was noted in 69.2 % of cases. An intact IS-OS line was found in only 23 % of cases at the final follow-up. In the LMH group, LHEP was associated with a more severe defect but didn't affect surgical outcomes. In the FTMH group, spontaneous hole closure was frequently noted. Despite small holes, disruption of IS-OS line was common after hole closure.

Idiopathic Scoliosis

Directory of Open Access Journals (Sweden)

Jens Ivar Brox

2014-07-01

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

Efficacy of transdermal nitroglycerine in idiopathic pre-term labour.

Science.gov (United States)

Shaikh, Shahida; Shaikh, Abdul Hameed; Akhter, Saleem; Isran, Basma

2012-01-01

To determine the efficacy of transdermal Nitroglycerine patch in idiopathic pre-term labour and foetomaternal outcome. This quasi-experimental study was conducted at the Obstetrics Unit-II of Shaikh Zayed Hospital for Women, Chandka Medical College, Shaheed Mohtarma Benazir Bhutto Medical University, Larkana, from Jan 1 to June 30, 2010. Sixtyfive pregnant women at 28-34 weeks of gestation were recruited after they met the selection criteria based on non-probability consecutive sampling. Initially, 73 patients were selected, but 65 of them completed the treatment, while 8 patients refused to continue. Patients diagnosed with pre-term labour were given glyceryl trinitrate (GTN) 5 mg/12 hours transdermal patch which was applied on the anterior abdominal wall. The second patch of same dose was given after 12 hours. Arrest of labour, prolongation of pregnancy in days or weeks along with side effects of the agent were monitored. Patients were followed till delivery to know the foeto-maternal outcome. Dramatic effects were seen in around 60 (92.3%), of the total patients who had felt relief from premature labour pains within the first hour and only 5 (7.6%) patients could not go beyond 24 hours, as among them 3 (4.61%) had previous uterine scar and 2 (3.07%) developed ruptured membranes after 12 hours of admission and their babies also could not survive. Mean pregnancy prolongation was 15.35 +/- 9.45 days (min: 4 max: 35), so delivery was deferred up to 48 hours, 3 to 7 days and more than 7 days in 4 (6.15%), 6 (9.23%) and 50 (76.92%) respectively. Glyceryl trinitrate, trans dermal patch is effective and safe tocolytic in idiopathic preterm labour. By prolonging pregnancy it improves neonatal outcome.

Periostin, discovered by nano-flow liquid chromatography and mass spectrometry, is a novel marker of diabetic retinopathy

Energy Technology Data Exchange (ETDEWEB)

Takada, Michiya [Division of Diabetes, Metabolism and Endocrinology, Department of Internal Medicine, Showa University School of Medicine, Tokyo (Japan); Ban, Yoshiyuki, E-mail: yshyban@yahoo.co.jp [Division of Diabetes, Metabolism and Endocrinology, Department of Internal Medicine, Showa University School of Medicine, Tokyo (Japan); Yamamoto, Gou [Department of Oral Pathology and Diagnosis, School of Dentistry, Showa University, Tokyo (Japan); Ueda, Toshihiko; Saito, Yuta; Nishimura, Eiichi; Fujisawa, Kunimi; Koide, Ryohei [Department of Ophthalmology, Showa University School of Medicine, Tokyo (Japan); Mizutani, Masakazu; Kozawa, Tadahiko; Shiraishi, Yuji [Kozawa Eye Hospital and Diabetes Center, Ibaraki-ken (Japan); Bando, Yasuhiko [Biosys Technologies, Inc., Meguro, Tokyo (Japan); Tachikawa, Tetsuhiko [Department of Oral Pathology and Diagnosis, School of Dentistry, Showa University, Tokyo (Japan); Hirano, Tsutomu [Division of Diabetes, Metabolism and Endocrinology, Department of Internal Medicine, Showa University School of Medicine, Tokyo (Japan)

2010-08-20

Research highlights: {yields} In proliferative membrane and epiretinal membrane specimens, the numbers of proteins are 225 and 154, respectively, and 123 proteins are common to both. {yields} Periostin and thrombospondin-1 proteins are unique to the proliferative membrane specimens. {yields} The expression of periostin is significantly up-regulated in proliferative membrane specimens. -- Abstract: Diabetes can lead to serious microvascular complications including proliferative diabetic retinopathy (PDR), the leading cause of blindness in adults. Recent studies using gene array technology have attempted to apply a hypothesis-generating approach to elucidate the pathogenesis of PDR, but these studies rely on mRNA differences, which may or may not be related to significant biological processes. To better understand the basic mechanisms of PDR and to identify potential new biomarkers, we performed shotgun liquid chromatography (LC)/tandem mass spectrometry (MS/MS) analysis on pooled protein extracts from neovascular membranes obtained from PDR specimens and compared the results with those from non-vascular epiretinal membrane (ERM) specimens. We detected 226 distinct proteins in neovascular membranes and 154 in ERM. Among these proteins, 102 were specific to neovascular membranes and 30 were specific to ERM. We identified a candidate marker, periostin, as well as several known PDR markers such as pigment epithelium-derived factor (PEDF). We then performed RT-PCR using these markers. The expression of periostin was significantly up-regulated in proliferative membrane specimens. Periostin induces cell attachment and spreading and plays a role in cell adhesion. Proteomic analysis by LC/MS/MS, which permits accurate quantitative comparison, was useful in identifying new candidates such as periostin potentially involved in the pathogenesis of PDR.

Periostin, discovered by nano-flow liquid chromatography and mass spectrometry, is a novel marker of diabetic retinopathy

International Nuclear Information System (INIS)

Takada, Michiya; Ban, Yoshiyuki; Yamamoto, Gou; Ueda, Toshihiko; Saito, Yuta; Nishimura, Eiichi; Fujisawa, Kunimi; Koide, Ryohei; Mizutani, Masakazu; Kozawa, Tadahiko; Shiraishi, Yuji; Bando, Yasuhiko; Tachikawa, Tetsuhiko; Hirano, Tsutomu

2010-01-01

Research highlights: → In proliferative membrane and epiretinal membrane specimens, the numbers of proteins are 225 and 154, respectively, and 123 proteins are common to both. → Periostin and thrombospondin-1 proteins are unique to the proliferative membrane specimens. → The expression of periostin is significantly up-regulated in proliferative membrane specimens. -- Abstract: Diabetes can lead to serious microvascular complications including proliferative diabetic retinopathy (PDR), the leading cause of blindness in adults. Recent studies using gene array technology have attempted to apply a hypothesis-generating approach to elucidate the pathogenesis of PDR, but these studies rely on mRNA differences, which may or may not be related to significant biological processes. To better understand the basic mechanisms of PDR and to identify potential new biomarkers, we performed shotgun liquid chromatography (LC)/tandem mass spectrometry (MS/MS) analysis on pooled protein extracts from neovascular membranes obtained from PDR specimens and compared the results with those from non-vascular epiretinal membrane (ERM) specimens. We detected 226 distinct proteins in neovascular membranes and 154 in ERM. Among these proteins, 102 were specific to neovascular membranes and 30 were specific to ERM. We identified a candidate marker, periostin, as well as several known PDR markers such as pigment epithelium-derived factor (PEDF). We then performed RT-PCR using these markers. The expression of periostin was significantly up-regulated in proliferative membrane specimens. Periostin induces cell attachment and spreading and plays a role in cell adhesion. Proteomic analysis by LC/MS/MS, which permits accurate quantitative comparison, was useful in identifying new candidates such as periostin potentially involved in the pathogenesis of PDR.

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

International Nuclear Information System (INIS)

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

2002-01-01

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

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

African Journals Online (AJOL)

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

MUSCLE DISORDERS IN ADOLESCENT IDIOPATHIC SCOLIOSIS: LITERATURE REVIEW

OpenAIRE

Débora Pinheiro Lédio Alves; Barbara de Araújo

2016-01-01

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

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

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Mihas Constantinos

2006-05-01

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

Tamoxifen is a potent antioxidant modulator for sperm quality in patients with idiopathic oligoasthenospermia.

Science.gov (United States)

Guo, Li; Jing, Jun; Feng, Yu-Ming; Yao, Bing

2015-09-01

To explore the new mechanisms of tamoxifen (TAM) in the treatment for patients with idiopathic oligoasthenospermia-antioxidation. In a prospective, randomized, controlled clinical trial, 120 cases of idiopathic oligoasthenospermia were enrolled and randomly assigned to the indomethacin group (n = 60) treated with indomethacin (25 mg, bid) and TAM group (n = 60) treated with TAM (10 mg, bid) for 3 months. Before and after treatment, we evaluated semen parameters, serum malondialdehyde (MDA) and total antioxidant capacity (TAC), seminal plasma MDA and TAC, spermatozoa intracellular reactive oxygen species (ROS), sperm succinate dehydrogenase (SDH) activity, sperm mitochondrial membrane potential (MMP), and sperm adenosine triphosphate (ATP) content. The independent t test and one-way repeated measures analysis of variance were used to compare the variables between and within two groups. In the indomethacin group, the percentage of progressive motile sperms, total motility, sperm MMP, and ATP content were increased significantly after 3-month treatment (P sperm count, sperm concentration, the percentage of progressive motile sperms, total motility, serum and seminal plasma TAC, sperm MMP, and ATP content were significantly improved or increased (P sperm count, sperm concentration, serum TAC, seminal plasma TAC, spermatozoa intracellular ROS, and sperm SDH activity. TAM treatment can significantly improve sperm quality, which is achieved through alleviating oxidative stress, improving sperm mitochondrial functionality, and subsequently increasing sperm motility.

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

LENUS (Irish Health Repository)

Loveman, Emma

2015-01-01

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

Idiopathic hemifacial spasm responsive to zonisamide: a case report.

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

2009-01-01

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

Pulmonary function in children with idiopathic scoliosis

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Tsiligiannis Theofanis

2012-03-01

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

Magnetic resonance imaging of shoulders with idiopathic adhesive capsulitis: reliability of measures

Energy Technology Data Exchange (ETDEWEB)

Lefevre-Colau, Marie-Martine; Fayad, Fouad; Rannou, Francois; Demaille-Wlodyka, Samantha; Mayoux-Benhamou, Marie-Anne; Poiraudeau, Serge; Revel, Michel [Universite Rene Descartes, Department of Physical and Rehabilitation Medicine, Hopital Cochin (AP-HP), Paris (France); Drape, Jean-Luc; Diche, Thierry; Minvielle, Francois [Hopital Cochin (AP-HP), Department of Radiology B, Paris (France); Fermanian, Jacques [Universite Rene Descartes, Department of Biostatistics, Hopital Necker (AP-HP), Paris (France)

2005-12-01

The magnetic resonance imaging (MRI) findings in idiopathic adhesive capsulitis (AC) were compared with those of contralateral healthy shoulders and the reliability of measures assessed. Twenty-six consecutive patients (26 AC and 14 healthy shoulders) were prospectively assessed. The main measurements were thickness of the joint capsule and synovial membrane in the axillary recess and rotator interval in T1-weighted spin-echo sequence enhanced with intravenous (IV) gadolinium chelate (Gd-chelate). Reliability was studied by use of the intraclass correlation coefficient (ICC). The mean thickness of the axillary recess on the coronal plane was 9.0{+-}2.2 mm in AC shoulders and 0.4{+-}0.7 mm in healthy shoulders. The mean thickness of the rotator interval on the sagittal plane was 8.4{+-}2.8 in AC shoulders and 0.6{+-}0.8 mm in healthy shoulders. Interobserver reliability was good for the axillary recess, with ICC values of 0.84 for the coronal plane, and good for the rotator interval, with ICC values of 0.80 for the sagittal plane. MRI with IV Gd-chelate injection can show, with acceptable reliability, signal and thickness abnormalities of the shoulder joint capsule and synovial membrane in AC. (orig.)

Magnetic resonance imaging of shoulders with idiopathic adhesive capsulitis: reliability of measures

International Nuclear Information System (INIS)

Lefevre-Colau, Marie-Martine; Fayad, Fouad; Rannou, Francois; Demaille-Wlodyka, Samantha; Mayoux-Benhamou, Marie-Anne; Poiraudeau, Serge; Revel, Michel; Drape, Jean-Luc; Diche, Thierry; Minvielle, Francois; Fermanian, Jacques

2005-01-01

The magnetic resonance imaging (MRI) findings in idiopathic adhesive capsulitis (AC) were compared with those of contralateral healthy shoulders and the reliability of measures assessed. Twenty-six consecutive patients (26 AC and 14 healthy shoulders) were prospectively assessed. The main measurements were thickness of the joint capsule and synovial membrane in the axillary recess and rotator interval in T1-weighted spin-echo sequence enhanced with intravenous (IV) gadolinium chelate (Gd-chelate). Reliability was studied by use of the intraclass correlation coefficient (ICC). The mean thickness of the axillary recess on the coronal plane was 9.0±2.2 mm in AC shoulders and 0.4±0.7 mm in healthy shoulders. The mean thickness of the rotator interval on the sagittal plane was 8.4±2.8 in AC shoulders and 0.6±0.8 mm in healthy shoulders. Interobserver reliability was good for the axillary recess, with ICC values of 0.84 for the coronal plane, and good for the rotator interval, with ICC values of 0.80 for the sagittal plane. MRI with IV Gd-chelate injection can show, with acceptable reliability, signal and thickness abnormalities of the shoulder joint capsule and synovial membrane in AC. (orig.)

Pediatric Idiopathic Intracranial Hypertension Presenting With Sensorineural Hearing Loss.

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

2015-12-01

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

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

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Allard Paul

2006-10-01

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

Idiopathic intracranial hypertension in pediatric patients

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Nada Jirásková

2008-11-01

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

Single photon emission computed tomography in children with idiopathic seizures

International Nuclear Information System (INIS)

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

1991-01-01

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

Intermediate uveitis: pattern of etiology, complications, treatment and outcome in a tertiary academic center.

Science.gov (United States)

Ness, Thomas; Boehringer, Daniel; Heinzelmann, Sonja

2017-04-27

Patients with intermediate uveitis (IU) represent a heterogenous group characterized by a wide spectrum of etiologies and regional differences. Aim of the study was to analyze the characteristics of patients with IU examined in an academic center in Germany. We conducted a retrospective analysis of the clinical records of all patients with intermediate uveitis referred to the Eye Center, University of Freiburg from 2007 to 2014. Diagnosis followed the Standardization in Uveitis Nomenclature (SUN) criteria. Data analysis included: etiology of IU, demographics, complications, treatment and visual acuity. We identified 159 patients with intermediate uveitis during that period. Mean age at diagnosis was 35 years. Most are female (64%), and the mean duration of IU was 6.1 years (range 1 month - 35 years). Etiology of IU was idiopathic in 59%. Multiple sclerosis (MS) (20%) and sarcoidosis (10%) were frequent systemic causes of IU. Other etiologies including infectious diseases (tuberculosis, borreliosis) or immune-mediated conditions (eg, after vaccination) were present in 11%. The pattern of complications included macular edema (CME) (36%), cataract (24%), secondary glaucoma (7%), and epiretinal membrane formation (19%). Periphlebitis and optic neuritis were more frequent in conjunction with MS. Treatment comprised local and systemic steroids, immunosuppressive agents, biologics, and surgery. Best corrected visual acuity was better than 20/25 in 60% of the eyes after more than 10 years of follow-up. In our German academic center, most IU cases were idiopathic or associated with MS or sarcoidosis. In contrast to other countries, infectious cases were rare. Patients' overall visual prognosis is favorable even when the duration of IU has been long and and despite numerous complications.

Idiopathic central diabetes Insipidus.

Science.gov (United States)

Grace, Mary; Balachandran, Venu; Menon, Sooraj

2011-10-01

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

Venous Thromboembolism and Risk of Idiopathic Interstitial Pneumonia A Nationwide Study

DEFF Research Database (Denmark)

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

2010-01-01

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

Idiopathic thoracic transdural intravertebral spinal cord herniation

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Mazda K Turel

2017-01-01

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

Idiopathic facial pain related with dental implantation

Directory of Open Access Journals (Sweden)

Tae-Geon Kwon

2016-06-01

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

27-GAUGE VITRECTOMY WOUND INTEGRITY: A Randomized Pilot Study Comparing Angled Versus Straight Entry in Fluid-Filled Vitrectomized Eyes.

Science.gov (United States)

Khan, M Ali; Durrani, Alia K; Hsu, Jason; Regillo, Carl D

2018-04-01

To compare clinical outcomes using angled versus straight trocar insertion during 27-gauge pars plana vitrectomy for epiretinal membrane. Pilot randomized controlled trial. Thirty eyes of 30 patients undergoing 27-gauge pars plana vitrectomy with membrane peeling for epiretinal membrane were randomized 1:1 to receive angled or straight trocar insertion. Intraocular pressure (IOP) and postoperative wound-related complications were compared. Fifteen eyes were randomized to each the angled and straight incision groups. No significant difference in phakic status (P = 0.71) or preoperative IOP (15.1 ± 3.4 vs. 14.6 ± 3.0 mmHg, P = 0.67) existed between groups. On postoperative Day 1, eyes in the straight group had lower IOP compared with the angled group (11.8 ± 3.9 vs. 15.3 ± 5.2 mmHg, P = 0.04) and a relative decrease in IOP compared with preoperative values (11.8 ± 3.9 vs. 15.1 ± 3.4 mmHg, P straight group) and serous choroidal detachment (1 eye, angled group). Eyes with straight incisions had transiently lower IOP on postoperative Day 1, possibly suggestive of subclinical wound leak in the very early postoperative period. Overall, rates of hypotony and sclerotomy-related complications were similarly low between wound construction strategies.

Internal limiting membrane peeling in macula-off retinal detachment complicated by grade B proliferative vitreoretinopathy.

Science.gov (United States)

Foveau, Pauline; Leroy, Bertrand; Berrod, Jean-Paul; Conart, Jean-Baptiste

2018-04-02

To investigate the clinical benefit of internal limiting membrane (ILM) peeling as a surgical adjunct in the repair of primary retinal detachment (RD) complicated by grade B proliferative vitreoretinopathy (PVR). Retrospective, interventional, comparative case series. SETTING: Institutional. 75 consecutive patients who underwent vitrectomy for primary macula off RD complicated by grade B PVR. Patients were divided into an ILM peeling (group P) and a no ILM peeling (group NP). Anatomical success rate, best-corrected visual acuity, and spectral-domain optical coherence tomography (SD-OCT) characteristics were collected at 1 and 6 months. In all, 37 eyes with ILM peeling were included in group P and 38 eyes without ILM peeling were included in group NP. The anatomical success rate after single surgery was higher in group P (89%) than in group NP (66%, p=0.03). Mean final visual acuity was 0.41 ±0.40 logMAR in group P versus 0.43 ±0.22 logMAR in group NP (p=0.82). We found no epiretinal membrane (ERM) formation in group P, whereas five cases of ERM (20%) were detected in group NP (p=0.012). The two groups did not differ in terms of cystoid macular edema occurrence, macular thickness, or photoreceptor damage. ILM peeling during vitrectomy in macula off RD complicated by grade B PVR reduces the need for a second surgery for re-detachment or macular pucker. Copyright © 2018. Published by Elsevier Inc.

Idiopathic megarectum in children.

Science.gov (United States)

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

2001-02-01

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

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

NARCIS (Netherlands)

Schlösser, T.P.C.

2014-01-01

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

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

NARCIS (Netherlands)

Janssen, M.M.A.

2011-01-01

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

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

Directory of Open Access Journals (Sweden)

Diego Rey

2015-02-01

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

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

Science.gov (United States)

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

2015-01-01

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

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

Science.gov (United States)

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

2016-03-18

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

Nasal bacterial colonization in cases of idiopathic epistaxis in children.

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Kamble, Payal; Saxena, Sonal; Kumar, Sunil

2015-11-01

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

Antioxidant therapy in idiopathic oligoasthenoteratozoospermia

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Ahmad Majzoub

2017-01-01

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

Calcium Sensing Receptor Mutations Implicated in Pancreatitis and Idiopathic Epilepsy Syndrome Disrupt an Arginine-rich Retention Motif

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Stepanchick, Ann; McKenna, Jennifer; McGovern, Olivia; Huang, Ying; Breitwieser, Gerda E.

2010-01-01

Calcium sensing receptor (CaSR) mutations implicated in familial hypocalciuric hypercalcemia, pancreatitis and idiopathic epilepsy syndrome map to an extended arginine-rich region in the proximal carboxyl terminus. Arginine-rich motifs mediate endoplasmic reticulum retention and/or retrieval of multisubunit proteins so we asked whether these mutations, R886P, R896H or R898Q, altered CaSR targeting to the plasma membrane. Targeting was enhanced by all three mutations, and Ca2+-stimulated ERK1/2 phosphorylation was increased for R896H and R898Q. To define the role of the extended arginine-rich region in CaSR trafficking, we independently determined the contributions of R890/R891 and/or R896/K897/R898 motifs by mutation to alanine. Disruption of the motif(s) significantly increased surface expression and function relative to wt CaSR. The arginine-rich region is flanked by phosphorylation sites at S892 (protein kinase C) and S899 (protein kinase A). The phosphorylation state of S899 regulated recognition of the arginine-rich region; S899D showed increased surface localization. CaSR assembles in the endoplasmic reticulum as a covalent disulfide-linked dimer and we determined whether retention requires the presence of arginine-rich regions in both subunits. A single arginine-rich region within the dimer was sufficient to confer intracellular retention comparable to wt CaSR. We have identified an extended arginine-rich region in the proximal carboxyl terminus of CaSR (residues R890 - R898) which fosters intracellular retention of CaSR and is regulated by phosphorylation. Mutation(s) identified in chronic pancreatitis and idiopathic epilepsy syndrome therefore increase plasma membrane targeting of CaSR, likely contributing to the altered Ca2+ signaling characteristic of these diseases. PMID:20798521

Idiopathic gingival fibromatosis

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Sujata Rath

2011-01-01

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

Epidemiology of idiopathic pulmonary fibrosis

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Ley B

2013-11-01

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

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

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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).

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

2018-03-01

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

Idiopathic Canalicular Inflammatory Disease: New Disease Description of Clinical Patterns, Investigations, Management, and Outcomes.

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Ali, Mohammad Javed

2018-01-25

The objective of this perspective is to present a separate disease description of "idiopathic canalicular inflammatory disease" and outline the diagnostic criteria and early experiences with its investigations and management. Retrospective case series of 44 canaliculi of 22 eyes of 11 patients presenting at a tertiary care Dacryology service over a period of 2 years with typical clinical patterns of inflammatory canaliculitis and its outcomes were studied. All the patients underwent microbiological work-up with culture and sensitivity, dacryoendoscopy imaging, serial Fourier domain ocular coherence tomography, and collagen vascular profiles. Stages in the evolution of the disease were studied. All patients were treated initially with topical steroids followed by punctal dilatation and placement of mini-monoka stents. Five patients in addition had a small biopsy from the inflamed portion of the vertical canaliculus. Stents were extubated at 6 weeks. Forty-four canaliculi were diagnosed to have idiopathic canalicular inflammatory disease during the study period. There was a female preponderance (81.8%, 9/11) and the mean age at presentation was 57 years. All patients presented with unilateral epiphora without any discharge, pain, or swelling. Collagen vascular profiles and screening for autoimmune diseases were negative. Clinical picture ranged from stages 1 to 5, consisting of edema, progressive centripetal vascularization, pouting of vascularized mucosa, membrane formation, and progressive scarring. The presentation begins in 1 eye and usually involves the other eye at a mean of 6 months. Ocular coherence tomography and dacryoendoscopy were of adjunctive value in the diagnosis. Histopathological examination was suggestive of a chronic inflammation. All patients had relentless progression to end-stage disease, although delayed significantly by steroids and monoka intubation. Idiopathic canalicular inflammatory disease has a distinct and typical clinical behavior and

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

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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.

Endoscope-Assisted and Controlled Argus II Epiretinal Prosthesis Implantation in Late-Stage Retinitis Pigmentosa: A Report of 2 Cases.

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Özmert, Emin; Demirel, Sibel

2016-01-01

Several different approaches for restoring sight in subjects who are blind due to outer retinal degeneration are currently under investigation, including stem cell therapy, gene therapy, and visual prostheses. Although many different types of visual prostheses have shown promise, to date, the Argus II Epiretinal Prosthesis System, developed in a clinical setting over the course of 10 years, is the world's first and only retinal prosthesis that has been approved by the United States Food and Drug Administration (FDA) and has been given the CE-Mark for sale within the European Economic Area (EEA). The incidence of serious adverse events from Argus II implantation decreased over time after minor changes in the implant design and improvements in the surgical steps used for the procedure had been made. In order to further decrease the scleral incision-related complications and enhance the assessment of the tack position and the contact between the array and the inner macular surface, we used an ophthalmic endoscope during the regular course of Argus II implantation surgery in 2 patients with late-stage retinitis pigmentosa in an attempt to improve the anatomical and functional outcomes.

Endoscope-Assisted and Controlled Argus II Epiretinal Prosthesis Implantation in Late-Stage Retinitis Pigmentosa: A Report of 2 Cases

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Emin Özmert

2016-12-01

Full Text Available Several different approaches for restoring sight in subjects who are blind due to outer retinal degeneration are currently under investigation, including stem cell therapy, gene therapy, and visual prostheses. Although many different types of visual prostheses have shown promise, to date, the Argus II Epiretinal Prosthesis System, developed in a clinical setting over the course of 10 years, is the world’s first and only retinal prosthesis that has been approved by the United States Food and Drug Administration (FDA and has been given the CE-Mark for sale within the European Economic Area (EEA. The incidence of serious adverse events from Argus II implantation decreased over time after minor changes in the implant design and improvements in the surgical steps used for the procedure had been made. In order to further decrease the scleral incision-related complications and enhance the assessment of the tack position and the contact between the array and the inner macular surface, we used an ophthalmic endoscope during the regular course of Argus II implantation surgery in 2 patients with late-stage retinitis pigmentosa in an attempt to improve the anatomical and functional outcomes.

Management of severe idiopathic oligohydramnios: is antepartum transabdominal amnioinfusion really a treatment option?

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Kozinszky, Zoltan; Pásztor, Norbert; Vanya, Melinda; Sikovanyecz, János; Pál, Attila

2013-03-01

The aim of this study was an evaluation of the role of antepartum transabdominal amnioinfusion (APTA) in the management of severe idiopathic oligohydramnios with a view to improving the pregnancy outcome. The study comprised an analysis of 20 pregnant women with severe oligohydramnios who were treated with APTA in 2009 and 2012. The pregnancy outcomes and the complications of the procedure were analysed. The mean gestational age at first treatment was 22 weeks 3 days. The preprocedure amniotic fluid index of <5 cm was restored by the treatment to 8 cm. More than a half of the pregnancies (66.7%, 8/12) treated with APTA finished with spontaneous abortion in the second trimester, mostly due to rupture of the membranes (as a consequence of retroamniotic filling with saline in four cases). The later the oligohydramnios developed, the higher the probability of a significant prolongation of the gestation. Although APTA is a useful procedure in the management of severe oligohydramnios, it may be followed by a relatively high rate of rupture of the membranes, particularly in the second trimester. Paradoxically, a higher volume of infused saline into the amniotic cavity is associated with a significantly lower risk of rupture.

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

OpenAIRE

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

2016-01-01

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

Idiopathic incapacitates in infancy and childhood

International Nuclear Information System (INIS)

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

2003-01-01

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

Genetics Home Reference: idiopathic inflammatory myopathy

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

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

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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.

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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.

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

International Nuclear Information System (INIS)

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

2004-01-01

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

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

International Nuclear Information System (INIS)

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

1990-01-01

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

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

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

1990-05-01

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

A CASE OF IDIOPATHIC PULMONARY ARTERIAL HYPERTENSION IN MALE

OpenAIRE

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

2016-01-01

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

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

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

2013-03-01

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

Locomotor skills and balance strategies in adolescents idiopathic scoliosis.

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

2007-01-01

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

MUSCLE DISORDERS IN ADOLESCENT IDIOPATHIC SCOLIOSIS: LITERATURE REVIEW

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Débora Pinheiro Lédio Alves

2016-03-01

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

Idiopathic unilateral vocal-fold paralysis in the adult.

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

2018-02-02

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

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

Science.gov (United States)

Yamada, Shigeki; Ishikawa, Masatsune; Yamamoto, Kazuo

2017-01-01

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

Alteration of placental haemostatic mechanisms in idiopathic intrauterine growth restriction

Directory of Open Access Journals (Sweden)

Jaime Eduardo Bernal Villegas

2012-08-01

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

IgG4 anti-phospholipase A2 receptor might activate lectin and alternative complement pathway meanwhile in idiopathic membranous nephropathy: an inspiration from a cross-sectional study.

Science.gov (United States)

Yang, Yang; Wang, Chao; Jin, Liping; He, Fagui; Li, Changchun; Gao, Qingman; Chen, Guanglei; He, Zhijun; Song, Minghui; Zhou, Zhuliang; Shan, Fujun; Qi, Ka; Ma, Lu

2016-08-01

The deposition of IgG4 of antibodies against phospholipase A2 receptor (anti-PLA2R) is predominating in the kidneys of patients with idiopathic membranous nephropathy, while its predictive value has not been determined. It was a retrospective study, and 438 patients were included. Serum samples of two time points [before intervention (baseline) and after 1.5-year treatment (endpoint)] were detected for total and IgG4 anti-PLA2R. IgG4 IgG4 subclass and the achievement of CR; (3) bi-negativity of IgG4 has a high accuracy of predicting CR compared with total antibodies; (4) in patients of bi-positivity, those achieving CR showed lower MASP-1/2, MBL, C3a, C5a, FB, Ba and Bb than patients failing to achieve CR; (5) the titers of endpoint and decrease in Ba and Bb were associated with improvement of 24 h-UP in those of bi-positivity; and (6) the decrease in Ba was a significant factor for achieving CR in those of bi-positivity. Continuous IgG4 negativity was a useful tool to predict the achievement of CR; however, in patients of continuous IgG4 positivity, those with lower activation of lectin and alternative pathways would still more probably achieve CR.

Idiopathic pulmonary fibrosis: evolving concepts.

Science.gov (United States)

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

2014-08-01

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

Idiopathic intracranial hypertension

DEFF Research Database (Denmark)

Yri, Hanne M; Jensen, Rigmor H

2015-01-01

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

Genetics Home Reference: juvenile idiopathic arthritis

Science.gov (United States)

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

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

International Nuclear Information System (INIS)

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

2016-01-01

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

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

Energy Technology Data Exchange (ETDEWEB)

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

2016-06-15

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

A comparative study on OCT before and after the operation for vitreomacular traction syndrome

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Shu-Qi Song

2017-07-01

Full Text Available AIM: To make a contrast and then analyze the difference of optical coherence tomography(OCTbefore and after vitreomacular traction syndrome(VTSwas performed. METHODS: The clinical date of 11 evaluable eyes of 11 patients with VTS who were diagnosed by OCT and underwent 25G vitreous surgery from January 2013 to January 2014 were retrospectively analyzed. Patients were followed up for an average of 6mo, to observe the visual acuity and OCT examination of the patient before and after operation. We compared the changes of retinal thickness and local morphology before and after operation.RESULTS: After vitreous retraction, 6 eyes improved, 2 eyes do not improve. One eye received macular membrane traction, in the operation the macular epiretinal membrane peeling, retrial membrane stripping and the triamcinolone acetonide intravitreal injection were given, but after the operation, the vision does not improve. Two eyes received vitreous combined with retinal macular membrane traction. In the operation, macular epiretinal membrane stripping was given, after the operation, visual acuity improved. The proportion of those with visual acuity of 0.1 or more increased from 46% before to 73% after the operation. Before operations, the mean central macular thickness was 619.27±195.13μm, 239.12±143.84μm after, which decreased significantly(PCONCLUSION: Vitrectomy can effectively relieve the vitreous traction of the macula, and can prevent further decline in visual acuity and reduce macular edema as well as improve the visual acuity of some patients. So, OCT has important guiding significance on the diagnosis and prognosis of this group.

Microfabrication of an Implantable silicone Microelectrode array for an epiretinal prosthesis

Energy Technology Data Exchange (ETDEWEB)

Maghribi, Mariam Nader [Univ. of California, Davis, CA (United States)

2003-06-10

Millions of people suffering from diseases such as retinitis pigmentosa and macular degeneration are legally blind due to the loss of photoreceptor function. Fortunately a large percentage of the neural cells connected to the photoreceptors remain viable, and electrical stimulation of these cells has been shown to result in visual perception. These findings have generated worldwide efforts to develop a retinal prosthesis device, with the hope of restoring vision. Advances in microfabrication, integrated circuits, and wireless technologies provide the means to reach this challenging goal. This dissertation describes the development of innovative silicone-based microfabrication techniques for producing an implantable microelectrode array. The microelectrode array is a component of an epiretinal prosthesis being developed by a multi-laboratory consortium. This array will serve as the interface between an electronic imaging system and the human eye, directly stimulating retinal neurons via thin film conducting traces. Because the array is intended as a long-term implant, vital biological and physical design requirements must be met. A retinal implant poses difficult engineering challenges due to the size of the intraocular cavity and the delicate retina. Not only does it have to be biocompatible in terms of cytotoxicity and degradation, but it also has to be structurally biocompatible, with regard to smooth edges and high conformability; basically mimicking the biological tissue. This is vital to minimize stress and prevent physical damage to the retina. Also, the device must be robust to withstand the forces imposed on it during fabrication and implantation. In order to meet these biocompatibility needs, the use of non-conventional microfabrication materials such as silicone is required. This mandates the enhancement of currently available polymer-based fabrication techniques and the development of new microfabrication methods. Through an iterative process, devices

Interventional Radiologic Treatment for Idiopathic Portal Hypertension

International Nuclear Information System (INIS)

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

1999-01-01

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

Neurodevelopment in preschool idiopathic toe-walkers.

Science.gov (United States)

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

2017-09-01

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

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

African Journals Online (AJOL)

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

Serum anti-PLA2R antibody as a diagnostic biomarker of idiopathic membranous nephropathy: The optimal cut-off value for Chinese patients.

Science.gov (United States)

Liu, Yipeng; Li, Xuan; Ma, Chaoqun; Wang, Ping; Liu, Ju; Su, Hong; Zhuo, Hao; Kong, Xianglei; Xu, Dayu; Xu, Dongmei

2018-01-01

The M-type phospholipase A2 receptor (PLA2R) is a specific target autoantigen identified in idiopathic membranous nephropathy (IMN). The autoantibody against PLA2R (anti-PLA2R) may be used to diagnose IMN. However, the appropriate diagnosis cut-off value for Chinese patients with IMN has not been established. In total, 119 patients who underwent renal biopsy (57 patients with IMN and 62 patients with non-IMN glomerulonephritis) and 22 healthy individuals were recruited for our observation study from Qianfoshan Hospital between September 2011 and March 2016. The serum concentration of anti-PLA2R was measured using a quantitative enzyme-linked immunosorbent assay (ELISA). The sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and receiver operating characteristic (ROC) curve of anti-PLA2R in diagnosing IMN were analysed based on the ELISA detection. The sensitivity, specificity, PPV, and NPV of anti-PLA2R in the diagnosis of IMN in the Chinese patients were 82.5, 75, 69.1, and 86.3% for the 2RU/ml cut-off value; 78.9, 91.7, 86.5, and 86.5% for the 2.6RU/ml cut-off value; 59.6, 95.2, 89.5, and 77.7% for the 14RU/ml cut-off value; 50.9, 96.4, 90.6, and 74.3% for the 20RU/ml cut-off value; and 47.4, 97.6, 93.1, and 73.2% for the 40RU/ml cut-off value, respectively. The area under the ROC curve was 0.879. The cut-off value of 2.6RU/ml is recommended for the use of anti-PLA2R for the diagnosis of IMN in Chinese patients based on the ELISA. Copyright © 2017. Published by Elsevier B.V.

Idiopathic noncirrhotic portal hypertension: current perspectives.

Science.gov (United States)

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

2016-01-01

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

Increased Granulocyte Heparanase Activity in Neutrophils from Patients with Lupus Nephritis and Idiopathic Membranous Nephropathy.

Science.gov (United States)

Szymczak, Maciej; Kuźniar, Jakub; Kopeć, Wacław; Żabińska, Marcelina; Marchewka, Zofia; Kościelska-Kasprzak, Katarzyna; Klinger, Marian

2017-02-01

Heparanase is a β-glucuronidase that cleaves sugar chains of heparan sulfate proteoglycans. It is believed that heparanase may be involved in the pathogenesis of proteinuria. The aim of this study was to assess the significance of heparanase in the pathogenesis of particular glomerulonephritis types. The evaluation of heparanase activity in serum, urine, and granulocytes and superoxide dismutase (SOD) activity in granulocytes of patients with lupus nephritis (n = 17), membranous nephropathy (n = 11), IgA nephropathy (n = 12), focal and segmental glomerulosclerosis (n = 18), mesangiocapillary glomerulonephritis (n = 12) and in 19 healthy volunteers were performed. The heparanase activity in granulocytes of patients with lupus nephritis and membranous nephropathy was higher than heparanase activity in granulocytes in the control group (p = 0.02 in both cases). This is the first observation of this phenomenon. There was no difference between SOD activity in granulocytes of patients with all assessed types of glomerulonephritis and the control group. A positive correlation between heparanase activity in urine and double-strain DNA antibodies (r = 0.51; p = 0.04), and reverse correlations between heparanase in urine and hemolytic activity of the complement (r = -0.57; p = 0.03) in the lupus nephritis group, and between heparanase activity in granulocytes and serum total protein level (r = -0.69; p = 0.02) in membranous nephropathy were observed. Increase in heparanase activity without changes in superoxide dismutase activity in the granulocytes from patients with lupus nephritis and membranous nephropathy was observed. It may be used as one of the markers of these disease activities.

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

OpenAIRE

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

2007-01-01

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

Pirfenidone treatment in idiopathic pulmonary fibrosis

DEFF Research Database (Denmark)

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

2016-01-01

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

Morphological features in eyes with endophthalmitis after cataract surgery – histopathology and optical coherence tomography assessment

DEFF Research Database (Denmark)

Solborg Bjerrum, Søren; Prause, Jan Ulrik; Fuchs, Helle Josefine

2016-01-01

limiting membrane (ILM) of the retina (14 eyes versus 3 eyes, p = 0.015) and a higher degree of retinal atrophy temporal to the fovea (13 eyes versus 1 eye, p = 0.013) compared to fellow eyes. The histopathological analyses showed the formation of epiretinal membranes, derangement of all retinal layers...... with a reduced number of nuclei in the nuclear layers, loss of photoreceptor outer segments and massive retinal gliosis. CONCLUSIONS: Optical coherence tomography scans of the retina and histopathology analyses provide insights in the pathological process occurring in PE....

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

NARCIS (Netherlands)

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

2012-01-01

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

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

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Galina V. Pyatakova

2016-12-01

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

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

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O. Yu. Konopel'ko

2013-01-01

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

INFORMATIVITY OF SPECTRAL OPTICAL COHERENT TOMOGRAPHY IN AGGRESSIVE POSTERIOR RETINOPATHY OF PREMATURITY

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A. V. Tereshchenko

2017-01-01

Full Text Available The purpose: to evaluate the informativity of optical coherence tomography in patients with aggressive posterior retinopathy of prematurity. Patients and methods. spectral optical coherence tomography using portable device iVue-100 with a removable camera (Optovue, USA was held in 32 children (64 eyes with aggressive posterior retinopathy of prematurity with a gestational period 26–31 week. Results. Children with aggressive posterior retinopathy of prematurity at the stage of early clinical manifestations, in addition to the indication that the immaturity of the retina, according to the spectral optical coherence tomography revealed only a few areas of epiretinal proliferation, which are not visualized with a digital retinoscopy and binocular indirect ophthalmoscopy. When the process is more pronounced in children with retinopathy of prematurity aggressive rear stage manifestation already determined multiple zones epiretinal proliferation as a "mushroom" and "flake" conglomerates with rear zone hyaloid membrane had an uneven seal. Coarser structural disorders of the retina and the vitreoretinal interface have been identified in patients with advancedstage aggressive posterior retinopathy of prematurity. We determined the shaft extraretinal proliferation as a "comb", as well as portions of epiretinal proliferation on the border of vascularized and avascular retina, which tended to merge, and the formation of massive hyperreflection complexes, lifted back hyaloid membrane, which was not only uneven sealed, but in some places is stratified. Conclusion. Despite the complexity of the procedure and the complexity of its implementation, the data obtained are particularly valuable and informative because they allow to complement the clinical picture and objectify it. It helps to choose the optimal tactics and improvement of a differentiated approach to the treatment of aggressive posterior retinopathy of prematurity.

[Results of amnioinfusion in pregnancies with oligohydramnios and non-ruptured fetal membranes].

Science.gov (United States)

Zamłyński, Jacek; Bodzek, Piotr; Olejek, Anita; Grettka, Krzysztof; Mańka, Grzegorz

2003-01-01

Authors analysed results of amnioinfusion in the group of 65 pregnant women with oligohydramnios and non-ruptured fetal membranes. Amnioinfusions were the most efficient in the pregnancies with oligohydramnios and asymmetric hypotrophy, idiopathic oligohydramnios or oligohydramnios with pathological FCG. The group with oligohydramnios and fetal malformations showed the worst results of the treatment. The findings confirm mainly the diagnostic character of the method before the end of 25 week of pregnancy because during period the oligohydramnios usually occurs together with lethal fetus malformations. The amnioinfusion in the cases of fetal hypotrophy and pregnancy-induced hypertension seems to be the prophylactic procedure.

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

DEFF Research Database (Denmark)

Lisse, I; Hasselbalch, H; Junker, P

1991-01-01

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

Atherosclerosis in Juvenile Idiopathic Arthritis

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Ewa Jednacz

2012-01-01

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

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

Science.gov (United States)

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

2015-07-01

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

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

Science.gov (United States)

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

2016-01-01

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

A case of idiopathic omental hemorrhage

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Toshimitsu Hosotani

2016-05-01

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

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

Science.gov (United States)

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

2014-04-30

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

Cost-effectiveness of internal limiting membrane peeling versus no peeling for patients with an idiopathic full-thickness macular hole: results from a randomised controlled trial.

Science.gov (United States)

Ternent, Laura; Vale, Luke; Boachie, Charles; Burr, Jennifer M; Lois, Noemi

2012-03-01

To determine whether internal limiting membrane (ILM) peeling is cost-effective compared with no peeling for patients with an idiopathic stage 2 or 3 full-thickness macular hole. A cost-effectiveness analysis was performed alongside a randomised controlled trial. 141 participants were randomly allocated to receive macular-hole surgery, with either ILM peeling or no peeling. Health-service resource use, costs and quality of life were calculated for each participant. The incremental cost per quality-adjusted life year (QALY) gained was calculated at 6 months. At 6 months, the total costs were on average higher (£424, 95% CI -182 to 1045) in the No Peel arm, primarily owing to the higher reoperation rate in the No Peel arm. The mean additional QALYs from ILM peel at 6 months were 0.002 (95% CI 0.01 to 0.013), adjusting for baseline EQ-5D and other minimisation factors. A mean incremental cost per QALY was not computed, as Peeling was on average less costly and slightly more effective. A stochastic analysis suggested that there was more than a 90% probability that Peeling would be cost-effective at a willingness-to-pay threshold of £20,000 per QALY. Although there is no evidence of a statistically significant difference in either costs or QALYs between macular hole surgery with or without ILM peeling, the balance of probabilities is that ILM Peeling is likely to be a cost-effective option for the treatment of macular holes. Further long-term follow-up data are needed to confirm these findings.

Membranous glomerulopathy and massive cervical lymphadenopathy due to immunoglobulin G4-disease

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Kamel El-Reshaid

2017-01-01

Full Text Available A 32-year-old male presented with acute and severe nephrotic syndrome as well as massive right cervical lymphadenopathy for <2 years. Computed tomography scan of the chest, abdomen, and pelvis did not reveal any lymphadenopathy. Histopathology and immunohistochemical testing of his lymph node biopsy showed infiltrate enriched with immunoglobulin G4 (IgG4-positive plasma cells. His kidney biopsy showed granular membranous deposits of IgG4 in the basement membrane without interstitial infiltrate. Antiphospholipid 2 receptor antibodies were absent excluding its "idiopathic" nature. Since he was allergic to rituximab, he was treated with corticosteroids for two months and a combination of tacrolimus and mycophenolate. His lymphadenopathy disappeared, and his proteinuria abated. The dose of the latter two medications was reduced to half after four months and will be maintained for a minimum of two years to prevent relapse of his disease.

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

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Juliana Molina

2012-10-01

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

Exercise therapy in juvenile idiopathic arthritis

NARCIS (Netherlands)

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

2008-01-01

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

CELSR2 is a candidate susceptibility gene in idiopathic scoliosis

DEFF Research Database (Denmark)

Einarsdottir, Elisabet; Grauers, Anna; Wang, Jingwen

2017-01-01

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

Acute Idiopathic Scrotal Edema

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Micheál Breen

2013-01-01

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

Prospective Retinal and Optic Nerve Vitrectomy Evaluation (PROVE study: findings at 3 months

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Reddy RK

2013-09-01

Full Text Available Rahul K Reddy,1 Maziar Lalezary,1 Stephen J Kim,1 Jeffrey A Kammer,1 Rachel W Kuchtey,1 Edward F Cherney,1 Franco M Recchia,2 Karen M Joos,1 Anita Agarwal,1 Janice C Law11Department of Ophthalmology, Vanderbilt University School of Medicine, Nashville, TN, USA; 2Tennessee Retina, PC, Nashville, TN, USABackground: The purpose of this paper is to report the 3-month findings of the Prospective Retinal and Optic Nerve Vitrectomy Evaluation (PROVE study.Methods: Eighty eyes of 40 participants undergoing vitrectomy were enrolled. Participants underwent baseline evaluation of the study (surgical and fellow (control eye that included: intraocular pressure, central corneal thickness, gonioscopy, cup-to-disc ratio measurement, color fundus and optic disc photography, automated perimetry, and optical coherence tomography of the macula and optic nerve. Evaluation was repeated at 3 months. Main outcome measures were changes in macula and retinal nerve fiber layer (RNFL thickness and intraocular pressure.Results: All participants completed follow-up. Mean cup-to-disc ratio of study and fellow eyes at baseline was 0.43 ± 0.2 and 0.46 ± 0.2, respectively, and 13% of participants had undiagnosed narrow angles. There was no significant change in intraocular pressure, cup-to-disc ratio, or pattern standard deviation in study eyes compared with baseline or fellow eyes at 3 months. Vision improved in all study eyes at 3 months compared with baseline (P = 0.013, but remained significantly worse than fellow eyes (P < 0.001. Central subfield and temporal peripapillary RNFL thickness were significantly greater in eyes with epiretinal membrane (P < 0.05, and resolution after surgery correlated with visual improvement (P < 0.05.Conclusion: The 3-month results do not indicate any increased risk for open-angle glaucoma but suggest that a relatively high percentage of eyes may be at risk of angle closure glaucoma. Temporal RNFL thickness and central subfield were increased

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

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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.

Plasma membrane wounding and repair in pulmonary diseases.

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Cong, Xiaofei; Hubmayr, Rolf D; Li, Changgong; Zhao, Xiaoli

2017-03-01

Various pathophysiological conditions such as surfactant dysfunction, mechanical ventilation, inflammation, pathogen products, environmental exposures, and gastric acid aspiration stress lung cells, and the compromise of plasma membranes occurs as a result. The mechanisms necessary for cells to repair plasma membrane defects have been extensively investigated in the last two decades, and some of these key repair mechanisms are also shown to occur following lung cell injury. Because it was theorized that lung wounding and repair are involved in the pathogenesis of acute respiratory distress syndrome (ARDS) and idiopathic pulmonary fibrosis (IPF), in this review, we summarized the experimental evidence of lung cell injury in these two devastating syndromes and discuss relevant genetic, physical, and biological injury mechanisms, as well as mechanisms used by lung cells for cell survival and membrane repair. Finally, we discuss relevant signaling pathways that may be activated by chronic or repeated lung cell injury as an extension of our cell injury and repair focus in this review. We hope that a holistic view of injurious stimuli relevant for ARDS and IPF could lead to updated experimental models. In addition, parallel discussion of membrane repair mechanisms in lung cells and injury-activated signaling pathways would encourage research to bridge gaps in current knowledge. Indeed, deep understanding of lung cell wounding and repair, and discovery of relevant repair moieties for lung cells, should inspire the development of new therapies that are likely preventive and broadly effective for targeting injurious pulmonary diseases. Copyright © 2017 the American Physiological Society.

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

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Yonghui Xia

2013-05-01

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

Glymphatic MRI in idiopathic normal pressure hydrocephalus.

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

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

2001-02-01

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

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

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Francesco Zulian

2012-01-01

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

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

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Mahesh Jayachandran

2013-01-01

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

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

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

2017-09-01

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

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

NARCIS (Netherlands)

Kouwenhoven, J.W.M.

2007-01-01

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

[Biologic therapy in idiopathic inflammatory myopathy].

Science.gov (United States)

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

2014-09-15

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

Idiopathic Harlequin syndrome – case report

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Marcelina Grochowiec

2015-09-01

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

Time course of recovery of idiopathic vocal fold paralysis.

Science.gov (United States)

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

2018-01-01

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

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

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MR Etemadifar

2005-05-01

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

Growth factors in idiopathic pulmonary fibrosis: relative roles

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Allen Jeremy T

2001-11-01

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

Update on diagnosis and treatment of idiopathic pulmonary fibrosis

Science.gov (United States)

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

2015-01-01

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

Update on diagnosis and treatment of idiopathic pulmonary fibrosis

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José Baddini-Martinez

2015-10-01

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

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

Science.gov (United States)

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

1997-08-01

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

Idiopathic Chronic Parotitis: Imaging Findings and Sialendoscopic Response.

Science.gov (United States)

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

2015-01-01

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

Optimal management of idiopathic macular holes

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Madi HA

2016-01-01

Full Text Available Haifa A Madi,1,* Ibrahim Masri,1,* David H Steel1,2 1Sunderland Eye Infirmary, Sunderland, 2Institute of Genetic Medicine, Newcastle University, International Centre for Life, Newcastle, UK *These authors contributed equally to this work Abstract: This review evaluates the current surgical options for the management of idiopathic macular holes (IMHs, including vitrectomy, ocriplasmin (OCP, and expansile gas use, and discusses key background information to inform the choice of treatment. An evidence-based approach to selecting the best treatment option for the individual patient based on IMH characteristics and patient-specific factors is suggested. For holes without vitreomacular attachment (VMA, vitrectomy is the only option with three key surgical variables: whether to peel the inner limiting membrane (ILM, the type of tamponade agent to be used, and the requirement for postoperative face-down posturing. There is a general consensus that ILM peeling improves primary anatomical hole closure rate; however, in small holes (<250 µm, it is uncertain whether peeling is always required. It has been increasingly recognized that long-acting gas and face-down positioning are not always necessary in patients with small- and medium-sized holes, but large (>400 µm and chronic holes (>1-year history are usually treated with long-acting gas and posturing. Several studies on posturing and gas choice were carried out in combination with ILM peeling, which may also influence the gas and posturing requirement. Combined phacovitrectomy appears to offer more rapid visual recovery without affecting the long-term outcomes of vitrectomy for IMH. OCP is licensed for use in patients with small- or medium-sized holes and VMA. A greater success rate in using OCP has been reported in smaller holes, but further predictive factors for its success are needed to refine its use. It is important to counsel patients realistically regarding the rates of success with

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

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

1994-12-15

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

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

International Nuclear Information System (INIS)

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

1994-01-01

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

Clinicopathologic correlation of chorioretinitis sclopetaria.

Science.gov (United States)

Dubovy, S R; Guyton, D L; Green, W R

1997-01-01

To report the clinicopathologic features in the eye of a patient who sustained a traumatic chorioretinal rupture from a gunshot wound to the orbit, chorioretinitis sclopetaria, with clinical follow up of more than 20 years. The patient was studied ophthalmoscopically and by fluorescein angiography after the trauma and was seen intermittently thereafter. The eyes were obtained postmortem; sections of the central portion of the right eye, including the macula and optic nerve head, and the inferior cap were examined by light microscopy. Histopathologic study of the right eye showed partial loss of the nerve fiber and ganglion cell layers in the macular area, temporal peripapillary and macular loss of the photoreceptors with hypertrophy and hyperplasia of the retinal pigment epithelium, an epiretinal membrane, and three defects in Bruch's membrane. Inferiorly, there was a 5-mm defect in choroid, Bruch's membrane, and retina. These structures were replaced by a loose and dense fibrous connective tissue. The sclera and a long posterior ciliary nerve remained intact. A thin fibrovascular tissue from the choroid extended into the subretinal space where it was covered by retinal pigment epithelium and thickened basement membrane in the posterior aspect of the inferior lesion. Marked hemiatrophy of the optic nerve was present. The clinicopathologic features of chorioretinitis sclopetaria include direct traumatic chorioretinal rupture followed by marked fibrovascular proliferation with variable replacement of choroid and retina with no retinal detachment. Posteriorly, indirect macular choroidal ruptures with hyperplasia and migration of the retinal pigment epithelium into the retina and choroid, epiretinal membrane formation, loss of photoreceptors, and marked hemiatrophy of the optic nerve were present.

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

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Ameri E

2012-11-01

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

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

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Gür, Gözde; Ayhan, Cigdem; Yakut, Yavuz

2017-06-01

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

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

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Raman R, Thulasi; Manimaran, D

2016-01-01

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

Idiopathic multiple aneurysm of external carotid artery

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Saravanan Balachandran

2014-01-01

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

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

LENUS (Irish Health Repository)

2012-02-01

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

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

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G. Raghu

2008-12-01

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

Altered Decorin and Smad Expression in Human Fetal Membranes in PPROM1

Science.gov (United States)

Horgan, Casie E.; Roumimper, Hailey; Tucker, Richard; Lechner, Beatrice E.

2014-01-01

ABSTRACT Humans with Ehlers-Danlos syndrome, a subtype of which is caused by abnormal decorin expression, are at increased risk of preterm birth due to preterm premature rupture of fetal membranes (PPROM). In the mouse model, the absence of decorin leads to fetal membrane abnormalities, preterm birth, and dysregulation of decorin's downstream pathway components, including the transcription factor p-Smad-2. However, the role of decorin and p-Smad-2 in idiopathic human PPROM is unknown. Fetal membranes from 20–25 pregnancies per group were obtained as a cross-sectional sample of births at one institution between January 2010 and December 2012. The groups were term, preterm without PPROM, and preterm with PPROM. Immunohistochemical analysis of fetal membranes was performed for decorin and p-Smad-2 using localization and quantification assessment. Decorin expression is developmentally regulated in fetal membranes and is decreased in preterm birth with PPROM compared to preterm birth without PPROM. In preterm with PPROM samples, the presence of infection is associated with significant decorin downregulation compared to preterm with PPROM samples without infection. The preterm with PPROM group exhibited decreased p-Smad-2 staining compared to both the term controls and the preterm-without-PPROM group. Our findings suggest that dysregulation of decorin and its downstream pathway component p-Smad-2 occurs in fetal membranes during the second trimester in pathological pregnancies, thus supporting a role for decorin and p-Smad-2 in the pathophysiology of fetal membranes and adverse pregnancy outcomes. These findings may lead to the discovery of new targets for the diagnosis and treatment of PPROM. PMID:25232019

Treatment of Refractory Idiopathic Supraorbital Neuralgia Using Percutaneous Pulsed Radiofrequency.

Science.gov (United States)

Luo, Fang; Lu, Jingjing; Ji, Nan

2018-02-26

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

Familial Idiopathic Cranial Neuropathy in a Chinese Family.

Science.gov (United States)

Zhang, Li; Liang, Jianfeng; Yu, Yanbing

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

Idiopathic brain herniation. A report of two paediatric cases.

Science.gov (United States)

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

2014-10-01

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

Idiopathic pulmonary fibrosis misdiagnosed as sputum-negative pulmonary tuberculosis.

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

2016-01-01

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

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

Science.gov (United States)

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

2013-11-01

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

Idiopathic fibrosing pancreatitis: A cause of obstructive jaundice in childhood

International Nuclear Information System (INIS)

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

1988-01-01

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

Idiopathic fibrosing pancreatitis: A cause of obstructive jaundice in childhood

Energy Technology Data Exchange (ETDEWEB)

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

1988-01-01

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

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

DEFF Research Database (Denmark)

Heiligenhaus, Arnd; Foeldvari, Ivan; Edelsten, Clive

2012-01-01

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

Complications of Macular Peeling

Science.gov (United States)

Asencio-Duran, Mónica; Manzano-Muñoz, Beatriz; Vallejo-García, José Luis; García-Martínez, Jesús

2015-01-01

Macular peeling refers to the surgical technique for the removal of preretinal tissue or the internal limiting membrane (ILM) in the macula for several retinal disorders, ranging from epiretinal membranes (primary or secondary to diabetic retinopathy, retinal detachment…) to full-thickness macular holes, macular edema, foveal retinoschisis, and others. The technique has evolved in the last two decades, and the different instrumentations and adjuncts have progressively advanced turning into a safer, easier, and more useful tool for the vitreoretinal surgeon. Here, we describe the main milestones of macular peeling, drawing attention to its associated complications. PMID:26425351

IDIOPATHIC SCLEROSING ENCAPSULATING PERITONITIS CAUSING ACUTE INTESTINAL OBSTRUCTION AND GANGRENE: A CASE REPORT

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Nava

2016-04-01

Full Text Available INTRODUCTION Sclerosing encapsulating peritonitis (SEP is a relatively rare cause of intestinal obstruction resulting from encasement of variable lengths of bowel by dense fibro-collagenous membrane. It is more common in young females, and shows tropical and sub-tropical distribution. The idiopathic cases of SEP, which lack any identifiable cause from clinical, radiological and histopathological findings, are also reported under the descriptive term “abdominal cocoon syndrome”. SEP presents with acute or sub-acute intestinal obstruction with or without a mass. In the era of laparoscopic surgery, inadvertent damage to the small bowel at insertion of the trocar and cannula can occur by being unaware of this condition resulting in unnecessary bowel resection. Persistent untreated SEP may advance to bowel gangrene or intestinal perforation, representing life threatening conditions. We report the clinical presentation of a 75-year-old female presenting with signs of intestinal obstruction whose imaging findings revealed abdominal cocoon with bowel gangrene leading to perforation and the same confirmed at surgery. Surgical excision of the fibrotic sac encasing the bowel, resection of gangrenous bowel segment and end ileostomy was performed. Histopathology of the excised membrane confirmed sclerosing encapsulating peritonitis. To our knowledge, only a few cases of abdominal cocoon with perforation have been reported in literature so far. Radiologists should be aware of this relatively rare cause of intestinal obstruction, its imaging findings and complications, as preoperative diagnosis will prevent delay and aid in treatment planning to the surgeon. Identification of soft tissue density membrane encasing congregated small bowel loops into a single area on computed-tomography gives diagnostic clue. Surgical excision of sac, release of bowel loops and adhesions with partial intestinal resection when necessary is the treatment.

Idiopathic Hypoparathyroidism Mimicking Ankylosing Spondylitis: A Case Report

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Ozge Illeez Memetoglu

2016-01-01

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

Idiopathic non-cirrhotic portal hypertension: a review

NARCIS (Netherlands)

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

2015-01-01

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

Diffuse idiopathic skeletal hyperostosis in ancient clergymen.

NARCIS (Netherlands)

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

2007-01-01

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

Diffuse idiopathic skeletal hyperostosis in ancient clergymen

NARCIS (Netherlands)

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

2007-01-01

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

Drug therapy for chronic idiopathic axonal polyneuropathy

NARCIS (Netherlands)

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

2017-01-01

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

Drug therapy for chronic idiopathic axonal polyneuropathy

NARCIS (Netherlands)

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

2017-01-01

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

Acute Lymphoblastic Leukaemia presenting as Juvenile Idiopathic ...

African Journals Online (AJOL)

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

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

International Nuclear Information System (INIS)

Nagano, Tatsuya; Kotani, Yoshikazu; Fujii, Osamu

2012-01-01

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

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

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

2016-09-15

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

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

International Nuclear Information System (INIS)

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

2016-01-01

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

Impact of juvenile idiopathic arthritis on schooling

Directory of Open Access Journals (Sweden)

Bouaddi Ilham

2013-01-01

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

Analysis of the Juvenile Idiopathic Arthritis Immunization Schedule

Directory of Open Access Journals (Sweden)

L. S. Namazova-Baranova

2016-01-01

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

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

Science.gov (United States)

LeBauer, Aaron; Brtalik, Robert; Stowe, Katherine

2008-10-01

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

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

International Nuclear Information System (INIS)

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

2008-01-01

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

Idiopathic Noncirrhotic Portal Hypertension: An Appraisal

Science.gov (United States)

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

2016-01-01

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

A case of idiopathic portalhypertension

International Nuclear Information System (INIS)

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

1982-01-01

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

Juvenile idiopathic arthritis-associated uveitis

OpenAIRE

Clarke, Sarah; Sen, Ethan; Ramanan, Athimalaipet

2016-01-01

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

Syphilis mimicking idiopathic intracranial hypertension

DEFF Research Database (Denmark)

Yri, Hanne; Wegener, Marianne; Jensen, Rigmor

2011-01-01

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

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

Directory of Open Access Journals (Sweden)

Byung Sun Kim

2013-12-01

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

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

Science.gov (United States)

Marimuthu, Vidhya; Krishnamurthy, Sriram; Rajappa, Medha

2017-11-15

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

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

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

2014-01-01

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

Idiopathic Thrombocytopenic Purpura Misdiagnosed as Hereditary Angioedema

DEFF Research Database (Denmark)

Andersen, Michelle Fog; Bygum, Anette

2015-01-01

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

Histological characteristics of diffuse idiopathic skeletal hyperostosis

NARCIS (Netherlands)

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

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

High-dose acetylcysteine in idiopathic pulmonary fibrosis

NARCIS (Netherlands)

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

2005-01-01

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

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

Science.gov (United States)

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

2016-01-01

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

Idiopathic trigeminal neuropathy in a poodle

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Carlos Eduardo Aparicio

2010-12-01

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

Contemporary Management of Idiopathic Laryngotracheal Stenosis.

Science.gov (United States)

Donahoe, Laura; Keshavjee, Shaf

2018-05-01

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

Clinical characteristics and outcomes of familial and idiopathic ...

African Journals Online (AJOL)

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

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

African Journals Online (AJOL)

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

Successful treatment of idiopathic pulmonary capillaritis with intravenous cyclophosphamide.

LENUS (Irish Health Repository)

Flanagan, Frances

2013-03-01

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

MRI findings in acute idiopathic transverse myelopathy in children

International Nuclear Information System (INIS)

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

2003-01-01

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

Chronic idiopathic intestinal pseudo-obstruction in an English bulldog.

Science.gov (United States)

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

2001-05-01

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

Idiopathic intracranial hypertension and transverse sinus stenoses

DEFF Research Database (Denmark)

Skyrman, Simon; Fytagoridis, Anders; Andresen, Morten

2013-01-01

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

Biological agents in polyarticular juvenile idiopathic arthritis

DEFF Research Database (Denmark)

Amarilyo, Gil; Tarp, Simon; Foeldvari, Ivan

2016-01-01

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

Combination therapy of low-fluence photodynamic therapy and intravitreal ranibizumab for choroidal neovascular membrane in choroidal osteoma

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Rodney J Morris

2011-01-01

Full Text Available Choroidal osteoma is an unusual form of intraocular calcification seen in otherwise healthy eyes. It is a benign idiopathic osseous tumor of the choroid, typically seen in young females. Choroidal neovascular membrane (CNVM is a complication seen in one-third of these patients and carries a poor visual outcome. We report a case of a 25-year-old hyperthyroid female with choroidal osteoma and subfoveal CNVM in her left eye which was successfully treated using low-fluence photodynamic therapy (PDT with verteporfin followed by a single injection of intravitreal ranibizumab.

Adolescent idiopathic scoliosis: Indications and efficacy of nonoperative treatment

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Federico Canavese

2011-01-01

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

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

International Nuclear Information System (INIS)

Wang Guohong; Xu Ruiji; Zhang Zhongshu; Wang Youji

2005-01-01

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

Optimal management of idiopathic macular holes.

Science.gov (United States)

Madi, Haifa A; Masri, Ibrahim; Steel, David H

2016-01-01

This review evaluates the current surgical options for the management of idiopathic macular holes (IMHs), including vitrectomy, ocriplasmin (OCP), and expansile gas use, and discusses key background information to inform the choice of treatment. An evidence-based approach to selecting the best treatment option for the individual patient based on IMH characteristics and patient-specific factors is suggested. For holes without vitreomacular attachment (VMA), vitrectomy is the only option with three key surgical variables: whether to peel the inner limiting membrane (ILM), the type of tamponade agent to be used, and the requirement for postoperative face-down posturing. There is a general consensus that ILM peeling improves primary anatomical hole closure rate; however, in small holes (holes, but large (>400 µm) and chronic holes (>1-year history) are usually treated with long-acting gas and posturing. Several studies on posturing and gas choice were carried out in combination with ILM peeling, which may also influence the gas and posturing requirement. Combined phacovitrectomy appears to offer more rapid visual recovery without affecting the long-term outcomes of vitrectomy for IMH. OCP is licensed for use in patients with small- or medium-sized holes and VMA. A greater success rate in using OCP has been reported in smaller holes, but further predictive factors for its success are needed to refine its use. It is important to counsel patients realistically regarding the rates of success with intravitreal OCP and its potential complications. Expansile gas can be considered as a further option in small holes with VMA; however, larger studies are required to provide guidance on its use.

GENETIC PREDICTORS OF IDIOPATHIC SICK SINUS SYNDROME

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A. A. Chernova

2012-01-01

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

Towards a new taxonomy of idiopathic orofacial pain.

Science.gov (United States)

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

2005-08-01

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

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

DEFF Research Database (Denmark)

Gideon, P; Sørensen, P S; Thomsen, C

1995-01-01

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

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

Directory of Open Access Journals (Sweden)

Moore Peter

2010-02-01

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

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

Science.gov (United States)

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

2011-01-01

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

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

Science.gov (United States)

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

2017-01-01

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

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

Science.gov (United States)

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

2010-08-01

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

Precision Medicine: The New Frontier in Idiopathic Pulmonary Fibrosis.

Science.gov (United States)

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

2016-06-01

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

An unusual case of idiopathic multiple invasive cervical resorption

Directory of Open Access Journals (Sweden)

Sathya Prakash Reddy Kesary

2014-01-01

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

The efficacy of flutamide, an antiandrogen in idiopathic hirsutism

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Somani V

1998-01-01

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

Idiopathic granulomatous mastitis: in search of a therapeutic paradigm.

Science.gov (United States)

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

2007-08-01

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

Idiopathic Pulmonary Calcification and Ossification in an Elderly ...

African Journals Online (AJOL)

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

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

International Nuclear Information System (INIS)

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

2004-01-01

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

Cyclosporine utilization in Idiopathic Nephrotic Syndrome in children

International Nuclear Information System (INIS)

Saeed, B.; Sheriff, S.; Ossman, Mohd Imad

2006-01-01

The treatment of steroid-resistant focal segmental glomerulosclerosis (FSGS) imposes one the most perplexing and frustrating problems on nephrologists. Cyclosporine A (CsA) is widely considered as the treatment of choice for steroid-resistant or dependent nephrotic children. We reviewed the clinical outcome in children with idiopathic nephrotic syndrome (INS) under CsA treatment. A total of 22 children presented with either steroid resistant nephrotic syndrome (SRNS) (14 children), or steroid-dependent nephrotic syndrome (SDNS) (8 children) during the period from August 2002 to February 2005; the mean age for both groups was 7.6 years (range: 23 months-15 years). Renal histology showed FSGS in 14 (63%) patients, minimal change disease (MCD) in 4(18%), diffuse mesangial glomerulonphritis (MesGN) in three (13.6%), and membranous glomerulonephritis (MGN) in two (6.8%). Treatment with CsA in combination with alternate-day prednisolone induced remission in 15(68%) patients; 9(60%) patients had complete remission and six (40%) partial remission. Seven (50%) patients in SRNS group responded to CsA treatment; two (14.2%) patients had complete remission and 5 (35.7%) had partial remission. Seven ( 87.5%) children in SDNS group had complete remission and one (13.5%) had partial remission. We conclude that this study demonstrates the efficacy of CsA in inducing remission in the steroid dependent is higher than in the steroid resistant nephrotic children. We believe that CsA is probably a good alternative therapy in this population. (author)

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

NARCIS (Netherlands)

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

2014-01-01

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

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

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Mimura Tatsuya

2010-12-01

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

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

Directory of Open Access Journals (Sweden)

E. I. Alexeeva

2013-01-01

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

Unusual Presentation Of Idiopathic Retroperitoneal Fibrosis: Case ...

African Journals Online (AJOL)

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

Idiopathic granulomatous lobular mastitis.

Science.gov (United States)

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

2012-02-01

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

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

Science.gov (United States)

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

2015-09-01

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

Idiopathic intracranial hypertension with altered consciousness in a ...

African Journals Online (AJOL)

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

Löffler's endomyocarditis in the idiopathic hypereosinophilic syndrome

NARCIS (Netherlands)

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

1999-01-01

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

Treatment of Resistant Idiopathic Hiccups with Pulse Radio ...

African Journals Online (AJOL)

2017-09-14

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

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

Science.gov (United States)

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

2017-05-01

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

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

Science.gov (United States)

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

2014-03-01

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

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

Science.gov (United States)

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

2015-01-01

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

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

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Juliana C. Ferreira

2011-01-01

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

Segmental torso masses in adolescent idiopathic scoliosis.

Science.gov (United States)

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

2014-08-01

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

Acute surgical management in idiopathic intracranial hypertension.

LENUS (Irish Health Repository)

Zakaria, Zaitun

2012-01-01

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

Idiopathic thrombocytopenic purpura

Directory of Open Access Journals (Sweden)

L Kayal

2014-01-01

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

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

DEFF Research Database (Denmark)

Grauers, Anna; Wang, Jingwen; Einarsdottir, Elisabet

2015-01-01

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

Non-HLA gene polymorphisms in juvenile idiopathic arthritis

DEFF Research Database (Denmark)

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

2017-01-01

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

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

Science.gov (United States)

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

2017-08-01

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

Graves' disease and idiopathic intracranial hypertension

OpenAIRE

Manish Gutch; Annesh Bhattacharjee; Sukriti Kumar; Durgesh Pushkar

2017-01-01

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

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

Science.gov (United States)

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

2009-11-01

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

Clinical efficiency of cyclosporine in chronic idiopathic urticaria in adults

Directory of Open Access Journals (Sweden)

V.I. Petrov

2010-06-01

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

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

International Nuclear Information System (INIS)

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

2000-01-01

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

Idiopathic hypertrophic subaortic stenosis. I

International Nuclear Information System (INIS)

Kvam, G.

1980-01-01

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

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

International Nuclear Information System (INIS)

Hiraoka, Kotaro; Meguro, Kenichi; Mori, Etsuro

2008-01-01

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

Idiopathic granulomatous mastitis | Rubin | SA Journal of Radiology

African Journals Online (AJOL)

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

Idiopathic Oesophageal Dysmotility Disorder: Stridor Secondary to Megaesophagus

Directory of Open Access Journals (Sweden)

B. G. Natesh

2013-01-01

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

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

Science.gov (United States)

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

2011-01-01

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

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

DEFF Research Database (Denmark)

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

2015-01-01

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

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

International Nuclear Information System (INIS)

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

2009-01-01

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

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

Science.gov (United States)

2013-01-01

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

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

African Journals Online (AJOL)

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

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

DEFF Research Database (Denmark)

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

2016-01-01

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

Value of internal limiting membrane peeling in surgery for idiopathic macular hole and the correlation between function and retinal morphology

DEFF Research Database (Denmark)

Christensen, Ulrik Correll

2009-01-01

conducted a randomized clinical trial including 78 pseudophakic patients with idiopathic macular hole stages 2 and 3. Patients were randomly assigned to macular hole surgery consisting of (i) vitrectomy alone without instrumental retinal surface contact (non-peeling), (ii) vitrectomy plus 0.05% isotonic ICG...... rate than surgery without ILM peeling (95% versus 45%). The overall functional results confirm that surgery for macular hole generally leads to favourable visual results, with two-thirds of eyes regaining reading vision (>or=20/40). Macular hole surgery can be considered a safe procedure with a low...... incidence of sight-threatening adverse events; the retinal detachment rate was 2.2%. Visual outcomes in eyes with primary hole closure were not significantly different between the intervention groups; however, for the stage 2 subgroup with primary macular hole closure, there was a trend towards a better...

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

International Nuclear Information System (INIS)

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

1991-01-01

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

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

Science.gov (United States)

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

2015-03-01

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

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

Directory of Open Access Journals (Sweden)

Elisabetta D’Agata

2017-11-01

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

MRI evaluation of multifidus muscles in adolescent idiopathic scoliosis

International Nuclear Information System (INIS)

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

1999-01-01

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

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

NARCIS (Netherlands)

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

2011-01-01

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

The pattern of juvenile idiopathic arthritis; a retrospective Egyptian ...

African Journals Online (AJOL)

Ehab

subtype specially at rural areas which differed from Western and Gulf countries pattern. ... version 18) and the following statistical measures ..... the results of Abdwani et al27 at 2015 (an Omani ... Ravelli A, Martini A. Juvenile idiopathic arthritis.

An Update on Idiopathic Intracranial Hypertension

OpenAIRE

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

2010-01-01

Idiopathic intracranial hypertension (IIH) is a condition of unknown etiology often encountered in neurologic practice. It produces non-localizing symptoms and signs of raised intracranial pressure and, when left untreated, can result in severe irreversible visual loss. It most commonly occurs in obese women of childbearing age, but it can also occur in children, men, non-obese adults, and older adults. While it is frequently associated with obesity, it can be associated with other conditions...

Case of twin pregnancy complicated by idiopathic thrombocytopenic ...

African Journals Online (AJOL)

2016-05-09

May 9, 2016 ... Idiopathic thrombocytopenic purpura (ITP) is an acquired thrombocytopenia without other clear cause of thrombocytopenia. It is not common in a singleton pregnancy and less common in twin pregnancy. We report a 33‑year‑old ITP pluripara whose first pregnancy was uneventful. She carried twin ...

Progressive multifocal leucoencephalopathy in a patient with idiopathic CD4+ lymphocytopenia.

LENUS (Irish Health Repository)

Moloney, F

2012-03-01

Progressive multifocal leucoencephalopathy (PML) is an opportunistic, demyelinating neurological disease caused by reactivation of the JC polyomavirus. PML occurs almost exclusively in immunosuppressed individuals, with only isolated case reports of PML occurring in patients without apparent immunosuppression. Idiopathic CD4+ lymohocytopenia (ICL) is a syndrome defined by the Centre for Disease Control and Prevention as a CD4+ count <300 cells\\/uL or <20% of total T cell count on >1 occasion, with no evidence of human immunodeficiency virus (HIV) infection, and the absence of other known immunodeficiency or therapy associated with lymphocytopenia. We describe a case of PML occurring in a patient with idiopathic CD4+ lymphocytopenia.

Comparison of plasma, liver, and skeletal muscle carnitine concentrations in cats with idiopathic hepatic lipidosis and in healthy cats.

Science.gov (United States)

Jacobs, G; Cornelius, L; Keene, B; Rakich, P; Shug, A

1990-09-01

Concentrations of total, free, and esterified carnitine were determined in plasma, liver, and skeletal muscle from cats with idiopathic hepatic lipidosis and compared with values from healthy cats. The mean concentrations of plasma, liver, and skeletal muscle total carnitine; plasma and skeletal muscle free carnitine; and plasma and liver esterified carnitine were greater (P less than 0.05) in cats with idiopathic hepatic lipidosis than in control cats. The mean for the ratio of free/total carnitine in plasma and liver was lower (P less than 0.05) in cats with idiopathic hepatic lipidosis than in control cats. These data suggest that carnitine deficiency does not contribute to the pathogenesis of feline idiopathic hepatic lipidosis.

[A case of cutaneous extramedullary hematopoiesis associated with idiopathic myelofibrosis].

Science.gov (United States)

Corella, F; Barnadas, M A; Bordes, R; Curell, R; Espinosa, I; Vergara, C; Alomar, A

2008-05-01

Cutaneous extramedullary hematopoiesis is a rare manifestation of chronic myeloproliferative processes, mainly chronic idiopathic myelofibrosis. In adults, it manifests as macules, papules, nodules, and ulcers on the trunk. The lesions usually appear soon after diagnosis and the possibility of a relationship between splenectomy and the appearance of extramedullary foci of hematopoiesis is still debated. Diagnosis is based on histopathology showing an infiltrate with different combinations of myeloid and erythroid cell precursors and megakaryocytes. Symptomatic treatment is provided alongside treatment of the underlying disease. We report a new case associated with chronic idiopathic myelofibrosis in which foci of cutaneous extramedullary hematopoiesis were observed 9 years after initial diagnosis. The lesions were progressive and the patient went on to develop acute myeloid leukemia.

Decreased retinal sensitivity after internal limiting membrane peeling for macular hole surgery.

Science.gov (United States)

Tadayoni, Ramin; Svorenova, Ivana; Erginay, Ali; Gaudric, Alain; Massin, Pascale

2012-12-01

To compare the retinal sensitivity and frequency of microscotomas found by spectral domain optical coherence tomography (SD-OCT) combined with scanning laser ophthalmoscopy (SLO) microperimetry after idiopathic macular hole closure, in eyes that underwent internal limiting membrane (ILM) peeling and eyes that did not. This was a retrospective, non-randomised, comparative study. Combined SD-OCT and SLO microperimetry was performed in 16 consecutive eyes after closure of an idiopathic macular hole. A customised microperimetry pattern with 29 measurement points was used. The ILM was peeled in 8/16 eyes. The main outcome measure was mean retinal sensitivity. Mean retinal sensitivity (in dB) was lower after peeling: 9.80 ± 2.35 dB with peeling versus 13.19 ± 2.92 without (p=0.0209). Postoperative microscotomas were significantly more frequent after ILM peeling: 11.3 ± 6.6 points with retinal sensitivity below 10 dB in eyes that underwent peeling versus 2.9 ± 4.6 in those that did not (p=0.0093). These results suggest that ILM peeling may reduce retinal sensitivity, and significantly increase the incidence of microscotomas. Until a prospective trial confirming or not these results, it seems justified to avoid peeling the ILM when its potential benefit seems minor or unproved, and when peeling is carried out, to limit the surface peeled to the bare minimum.

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

DEFF Research Database (Denmark)

Berntson, Lillemor; Wernroth, Lisa; Fasth, Anders

2007-01-01

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

Outcomes of recalcitrant idiopathic epistaxis in children: Septoplasty as a surgical treatment.

Science.gov (United States)

Levi, Jessica M; McKee-Cole, Katherine M; Barth, Patrick C; Brody, Robert M; Reilly, James S

2016-12-01

Our objective was to measure short- and long-term outcomes of children presenting with recalcitrant idiopathic epistaxis. The study was an 11-year (2000-2011) retrospective chart review of children evaluated and treated for epistaxis. A retrospective review of patients with diagnostic International Classification of Diseases, Ninth Revision code 784.7 (epistaxis) and 21.5/21.88 (septoplasty) was completed reviewing age at presentation, type of surgery, and number of bleeding events prior to and after surgery. In our cohort, almost 100% of children with idiopathic recurrent epistaxis responded to topical treatments. About 0.2% were refractory (20/9239), and 90% of those (18/20) resolved with either a traditional septoplasty (14/20) or modified septoplasty (6/20) without cartilage excision, with a mean follow-up of 35 months. One of the patients who did not show resolution was found to have Von Willebrand disease, which likely contributed to this outcome. Septoplasty surgery, with or without cartilage removal, appears beneficial for refractory idiopathic epistaxis in children. 4 Laryngoscope, 126:2833-2837, 2016. © 2016 The American Laryngological, Rhinological and Otological Society, Inc.

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

OpenAIRE

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

2016-01-01

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

Determinants of 6-minute walk distance in patients with idiopathic pulmonary fibrosis undergoing lung transplant evaluation.

Science.gov (United States)

Porteous, Mary K; Rivera-Lebron, Belinda N; Kreider, Maryl; Lee, James; Kawut, Steven M

2016-03-01

Little is known about the physiologic determinants of 6-minute walk distance in idiopathic pulmonary fibrosis. We investigated the demographic, pulmonary function, echocardiographic, and hemodynamic determinants of 6-minute walk distance in patients with idiopathic pulmonary fibrosis evaluated for lung transplantation. We performed a cross-sectional analysis of 130 patients with idiopathic pulmonary fibrosis who completed a lung transplantation evaluation at the Hospital of the University of Pennsylvania between 2005 and 2010. Multivariable linear regression analysis was used to generate an explanatory model for 6-minute walk distance. After adjustment for age, sex, race, height, and weight, the presence of right ventricular dilation was associated with a decrease of 50.9 m (95% confidence interval [CI], 8.4-93.3) in 6-minute walk distance ([Formula: see text]). For each 200-mL reduction in forced vital capacity, the walk distance decreased by 15.0 m (95% CI, 9.0-21.1; [Formula: see text]). For every increase of 1 Wood unit in pulmonary vascular resistance, the walk distance decreased by 17.3 m (95% CI, 5.1-29.5; [Formula: see text]). Six-minute walk distance in idiopathic pulmonary fibrosis depends in part on circulatory impairment and the degree of restrictive lung disease. Future trials that target right ventricular morphology, pulmonary vascular resistance, and forced vital capacity may potentially improve exercise capacity in patients with idiopathic pulmonary fibrosis.

Structural white matter abnormalities in patients with idiopathic dystonia

NARCIS (Netherlands)

Bonilha, Leonardo; de Vries, Paulien M.; Vincent, Diana J.; Rorden, Chris; Morgan, Paul S.; Hurd, Mark W.; Besenski, Nada; Bergmann, Kenneth J.; Hinson, Vanessa K.

2007-01-01

We investigated whether structural white matter abnormalities, in the form of disruption of axonal coherence and integrity as measured with diffusion tensor imaging (DTI), constitute an underlying pathological mechanism of idiopathic dystonia (ID), independent of genotype status. We studied seven

Evaluation of classification systems for nonspecific idiopathic orbital inflammation

NARCIS (Netherlands)

Bijlsma, Ward R.; van 't Hullenaar, Fleur C.; Mourits, Maarten P.; Kalmann, Rachel

2012-01-01

To systematically analyze existing classification systems for idiopathic orbital inflammation (IOI) and propose and test a new best practice classification system. A systematic literature search was conducted to find all studies that described and applied a classification system for IOI.

Ultrasonography and color Doppler in juvenile idiopathic arthritis

DEFF Research Database (Denmark)

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

2012-01-01

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

Prognosis in canine idiopathic immune-mediated haemolytic anaemia

NARCIS (Netherlands)

Piek, C.J.

2011-01-01

Canine idiopathic immune-mediated haemolytic anaemia (iIMHA) is one of the most frequently occurring immune-mediated diseases in dogs. A gel-based Coombs' test was shown to perform equally well as a classical Coombs' test. Since the gel-based Coombs' test can be commercially produced and is easy and

The epithelium in idiopathic pulmonary fibrosis: breaking the barrier

Directory of Open Access Journals (Sweden)

Ana eCamelo

2014-01-01

Full Text Available Idiopathic pulmonary fibrosis is a progressive disease of unknown etiology characterised by a dysregulated wound healing response that leads to fatal accumulation of fibroblasts and extracellular matrix in the lung, which compromises tissue architecture and lung function capacity. Injury to type II alveolar epithelial cells is thought to be the key event for the initiation of the disease, and so far both genetic factors, such as mutations in telomerase and MUC5b genes as well as environmental components, like cigarette smoking, exposure to asbestos and viral infections have been implicated as potential initiating triggers. The injured epithelium then enters a state of senescence-associated secretory phenotype whereby it produces both pro-inflammatory and pro-fibrotic factors that contribute to the wound healing process in the lung. Immune cells, like macrophages and neutrophils as well as activated myofibroblasts then perpetuate this cascade of epithelial cell apoptosis and proliferation by release of pro-fibrotic TGF-β and continuous deposition of extracellular matrix stiffens the basement membrane, altogether having a deleterious impact on epithelial cell function. In this review we describe the role of the epithelium as both a physical and immunological barrier between environment and self in the homeostatic versus diseased lung and explore the potential mechanisms of epithelial cell injury and the impact of loss of epithelial cell permeability and function on cytokine production, inflammation and myofibroblast activation in the fibrotic lung.

The Brazilian version of the SRS-22r questionnaire for idiopathic scoliosis

Directory of Open Access Journals (Sweden)

Paula M. F. Camarini

2013-10-01

Full Text Available BACKGROUND: The SRS-22r questionnaire is a well-accepted instrument used to measure health-related quality of life in patients with idiopathic scoliosis. No validated tool exists in Brazil for idiopathic scoliosis, and the use of the SRS-22r in non-English Laguage contries requires its transcultural adaptation. OBJECTIVE: The objective of this study was to culturally adapt the translated Brazilian version of the SRS-22r questionnaire and to determine its reliability using statistical tests for internal consistency and test-retest reliability. METHOD: The transcultural adaptation process was carried out according to the recommendations of the American Academy of Orthopedic Surgeons. The pre-final version was administered to 44 patients with idiopathic scoliosis. The mean age of the participants was 18.93 years and the mean curve magnitude was 54.6°. A subgroup of 30 volunteers completed the questionnaire a second time one week later to determine the scale's reproducibility. Internal consistency was determined using Cronbach's alpha coefficient, and the test-retest reliability was determined using the Intraclass Correlation Coefficient (ICC. RESULTS: No floor effects were observed using the Brazilian version of the SRS-22r. Ceiling effects were observed in the Pain and Satisfaction with Management domains. The internal consistency values were very good for 3 domains and good for 2 domains. The ICC values were excellent for all domains. CONCLUSIONS: The high values of internal consistency and ICC reproducibility suggest that this version of the questionnaire can be used in Brazilian patients with idiopathic scoliosis.

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

Science.gov (United States)

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

2016-02-01

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

A case of idiopathic interstitial pneumonia

OpenAIRE

豊見山, 寛; 兼島, 洋; 下地, 克佳; 金城, 勇徳; 中富, 昌夫; 小張, 一峰; 松井, 克明; Tomiyama, Hiroshi; Kaneshima, Hiroshi; Shimoji, Katsuyoshi; Kinjo, Yutoku; Nakatomi, Masao; Kobari, Kazumine; Matsui, Katsuaki; 琉球大学医学部第一内科

1982-01-01

A case of idiopathic interstitial pneumonia was reported. A 53-year-old man was admitted to our hospital with his complaint of shortness of breath. On physical examination clubbing of the fingers was noticed and velcro rales was heard on the bilateral lower back. On laboratory data no remarkable finding was revealed. Chest roentgenogram showed diffuse reticulonodular shadow and small ring shadow of the bilateral lower lung fields. On pulmonary function test decline of VC and DLco was revealed...

Idiopathic fibrosing pancreatitis: a rare cause of chronic obstructive jaundice in childhood

International Nuclear Information System (INIS)

Park, Hye Seong; Kim, Hyun Sook; Kim, Hack Hee; Kim, Ok Hwa; Kim, Choon Yul; Bahk, Yong Whee

1992-01-01

We report a 14-months-old infant who had obstructive jaundice caused by idiopathic fibrosing pancreatitis. Ultrasonography and abdominal computed tomography showed dilatation of the intrahepatic ducts, common bile duct, and the pancreatic duct. Diffuse swelling of the pancreas was also noted on CT. At laparotomy, the head portion of the pancreas revealed a stony hard consistency, and proliferation of fibrotic tissue was confirmed pathologically. Idiopathic fibrosing pancreatitis is a very rare disease entity in childhood, but should be considered in the differential diagnosis of obstructive jaundice in children who demonstrate bile duct and pancreatic duct dilatation and/or diffuse pancreas swelling

Idiopathic Scrotal Hematoma in Neonate: A Case Report and Review of the Literature

Directory of Open Access Journals (Sweden)

Bioku Muftau Jimoh

2014-01-01

Full Text Available Neonatal scrotal hematoma is a rare genitourinary emergency. Some cases have underlining aetiologic factors such as testicular torsion, adrenal hemorrhage, or birth trauma, and others are idiopathic. Previously, immediate scrotal exploration was considered imperative for diagnosis and treatment. With good imaging techniques, some patients are managed nonoperatively. We report a case of idiopathic scrotal hematoma in a neonate. He was managed conservatively with clinical and radiological follow-up. There was complete resolution of hematoma within two months, thus, avoiding unnecessary exploration.

Handwriting difficulties in juvenile idiopathic arthritis: a pilot study

NARCIS (Netherlands)

Haberfehlner, Helga; Visser, Bart; Daffertshofer, Andreas; van Rossum, Marion Aj; Roorda, Leo D.; van der Leeden, Marike; Dekker, Joost; Hoeksma, Agnes F.

2011-01-01

The aim of the present study was to describe handwriting difficulties of primary school children with juvenile idiopathic arthritis (JIA), and to investigate possible correlations with hand function and writing performance. In a cross-sectional approach, 15 children with JIA and reported handwriting

Disrupted thalamic prefrontal pathways in patients with idiopathic dystonia

NARCIS (Netherlands)

Bonilha, Leonardo; de Vries, Paulien M.; Hurd, Mark W.; Rorden, Chris; Morgan, Paul S.; Besenski, Nada; Bergmann, Kenneth J.; Hinson, Vanessa K.

There are quantifiable abnormalities in water diffusion properties of the white matter in thalamic and prefrontal areas in patients with idiopathic dystonia (ID). However, it is unclear which pathways are disrupted in these patients. Using probabilistic tractography of high resolution DTI, we

Fahr’s syndrome and idiopathic hypoparathyroidism: A case report

Directory of Open Access Journals (Sweden)

Marinković Dejan M.

2017-01-01

Full Text Available Introduction. Fahr´s syndrome is a rare, slowly progressive, neurodegenerative disorder, characterised by extensive, bilateral, and symmetrical basal ganglia calcification. It is associated with neuropsychiatric manifestations and gradually progressive cognitive impairment. Fahr's syndrome is the secondary form of brain calcification that is caused by various metabolic, infectious, or degenerative diseases. Case report. We presented a middle-aged male with Fahr's syndrome due to primary idiopathic hypoparathyroidism. Clinical diagnosis was based on signs and symptoms of hypocalcemia, progressive neuropsychiatric illnesses, laboratory evidence of hypoparathyroidism, and radiological signs of calcifications in the basal ganglia. The patient improved after only a few days of intravenous rehydration and calcium substitution, followed by oral supplemental calcitriol. Conclusion. Timely recognition of idiopathic and iatrogenic hypoparathyroidism allows appropriate treatment that can prevent the development and clinical manifestations of Fahr´s syndrome and potentially slow its progression.

Idiopathic interstitial pneumonias. From classification to diagnostic work-up

International Nuclear Information System (INIS)

Mueller-Mang, C.; Bankier, A.; Stiebellehner, L.; Schmid, K.

2007-01-01

Idiopathic interstitial pneumonias (IIP) comprise seven entities with distinct histologic patterns. In their idiopathic form IIP are rare diseases. They are, nevertheless, considered prototypes of the much more common secondary interstitial pneumonias. The advent of high-resolution computed tomography (HRCT) has had a profound impact on the imaging of IIP, because the detailed delineation of the lung anatomy allows a close correlation between the histologic patterns of IIP and the CT features. On the basis of CT morphology and in the correct clinical context, the radiologist can achieve an accurate diagnosis in many cases. However, due to overlap between the various entities, complementary lung biopsy is recommended in virtually all cases. This article reviews the CT pattern of IIP and offers relevant clinical and histological information for the purpose of enabling the radiologist to understand and participate in the multidisciplinary concept of IIP. (orig.) [de

Analysis of scapular muscle EMG activity in patients with idiopathic neck pain: a systematic review.

Science.gov (United States)

Castelein, Birgit; Cools, Ann; Bostyn, Emma; Delemarre, Jolien; Lemahieu, Trees; Cagnie, Barbara

2015-04-01

It is proposed that altered scapular muscle function can contribute to abnormal loading of the cervical spine. However, it is not clear if patients with idiopathic neck pain show altered activity of the scapular muscles. The aim of this paper was to systematically review the literature regarding the differences or similarities in scapular muscle activity, measured by electromyography ( = EMG), between patients with chronic idiopathic neck pain compared to pain-free controls. Case-control (neck pain/healthy) studies investigating scapular muscle EMG activity (amplitude, timing and fatigue parameters) were searched in Pubmed and Web of Science. 25 articles were included in the systematic review. During rest and activities below shoulder height, no clear differences in mean Upper Trapezius ( = UT) EMG activity exist between patients with idiopathic neck pain and a healthy control group. During overhead activities, no conclusion for scapular EMG amplitude can be drawn as a large variation of results were reported. Adaptation strategies during overhead tasks are not the same between studies. Only one study investigated timing of the scapular muscles and found a delayed onset and shorter duration of the SA during elevation in patients with idiopathic neck pain. For scapular muscle fatigue, no definite conclusions can be made as a wide variation and conflicting results are reported. Further high quality EMG research on scapular muscles (broader than the UT) is necessary to understand/draw conclusions on how scapular muscles react in the presence of idiopathic neck pain. Copyright © 2015 Elsevier Ltd. All rights reserved.

Idiopathic granulomatous hypophysitis: clinical and imaging features

Energy Technology Data Exchange (ETDEWEB)

Vasile, M. [Service de Radiologie, Hopital Saint-Antoine, 75 - Paris (France); Marsot-Dupuch, K. [Service de Radiologie, Hopital Saint-Antoine, 75 - Paris (France); Kujas, M. [Service d`Histologie Embryologie Cytogenetique, Hopital Pitie-Salpetriere, 75 - Paris (France); Brunereau, L. [Service de Radiologie, Hopital Saint-Antoine, 75 - Paris (France); Bouchard, P. [Service d`Histologie Embryologie Cytogenetique, Hopital Pitie-Salpetriere, 75 - Paris (France); Comoy, J. [Service de Neurochirurgie, Hopital Kremlin Bicetre, 94 (France); Tubiana, J.M. [Service de Radiologie, Hopital Saint-Antoine, 75 - Paris (France)

1997-01-01

Idiopathic pituitary granuloma is a rare disorder similar to lymphocytic adenohypophysitis. Few cases have been reported. We report a new histologically case proven with MRI. The patterns of clinical and radiological presentation and the management of this disorder are discussed. MRI findings suggestive of this condition include an intensely enhancing pituitary mass, associated with dural enhancement. Steroid therapy may be suggested avoiding unnecessary surgery. (orig.)

Identification of a novel idiopathic epilepsy locus in Belgian Shepherd dogs

DEFF Research Database (Denmark)

Seppälä, Eija H.; Koskinen, Lotta L.E.; Gulløv, Christina Hedal

2012-01-01

Epilepsy is the most common neurological disorder in dogs, with an incidence ranging from 0.5% to up to 20% in particular breeds. Canine epilepsy can be etiologically defined as idiopathic or symptomatic. Epileptic seizures may be classified as focal with or without secondary generalization...... collected 159 cases and 148 controls and confirmed the presence of epilepsy through epilepsy questionnaires and clinical examinations. The MRI was normal while interictal EEG revealed abnormalities and variable foci in the clinically examined affected dogs. A genome-wide association study using Affymetrix...... mutation. It would establish the affected breed as a novel therapeutic model, help to develop a DNA test for breeding purposes and introduce a novel candidate gene for human idiopathic epilepsies....

Are we simplifying balance evaluation in adolescent idiopathic scoliosis?

Science.gov (United States)

Pasha, Saba; Baldwin, Keith

2018-01-01

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

MRI evaluation of multifidus muscles in adolescent idiopathic scoliosis

Energy Technology Data Exchange (ETDEWEB)

Chan Yu-Leung; King, A.D.; Griffith, J.F.; Metreweli, C. [Dept. of Diagnostic Radiology and Organ Imaging, Chinese University of Hong Kong, Prince of Wales Hospital, Shatin (Hong Kong); Cheng, J.C.Y.; Guo Xia [Orthopaedics and Traumatology, Chinese University of Hong Kong, Prince of Wales Hospital, Shatin (Hong Kong)

1999-05-01

Background. The role of the multifidus muscles in the initiation and progression of curve in adolescent idiopathic scoliosis is not fully understood and controversy exists as to the side of the abnormality. Objective. To evaluate on MRI the multifidus muscles at the apex of the major curve in adolescent idiopathic scoliosis to ascertain if the multifidus muscles on the convex or concave side are abnormal and the relationship to curve severity. Materials and methods. Forty-six patients with adolescent idiopathic scoliosis, separated into two groups, were studied using a 1.5-T MR scanner with the synergy spine coil, employing a modified STIR (short tau inversion recovery) axial sequence obtained at the apex of the major scoliotic curve. Results. No hyperintense signal change was demonstrated in the convex side multifidus muscles in any patient. In group I, 16 of 18 patients with severe or rapidly progressive curve showed increase in signal intensity in the multifidus muscle on the concave side of the apex of the curve. In group II, of the 15 patients with mild curve (Cobb angle 10-30 ), 4 had increased signal intensity in the multifidus muscle on the concave side; of the 13 with more severe curve (Cobb angle greater than 30 ), 10 had increase in multifidus signal intensity on the concave side. Conclusions. The concave-side multifidus muscle at the apex of a scoliotic curve was morphologically abnormal. A significant association between abnormal signal change and curve severity was also established. (orig.) With 2 figs., 3 tabs., 25 refs.

Autologous transplantation of the internal limiting membrane for refractory macular holes.

Science.gov (United States)

Morizane, Yuki; Shiraga, Fumio; Kimura, Shuhei; Hosokawa, Mio; Shiode, Yusuke; Kawata, Tetsuhiro; Hosogi, Mika; Shirakata, Yukari; Okanouchi, Toshio

2014-04-01

To determine the effectiveness of autologous transplantation of the internal limiting membrane (ILM) for refractory macular holes. Prospective, interventional case series. Ten eyes of 10 consecutive patients who underwent autologous transplantation of the ILM for the treatment of refractory macular holes were studied. The primary diseases in these patients were large idiopathic macular holes that had existed for more than 1 year (4 eyes), a traumatic macular hole (1 eye), myopic foveoschisis (2 eyes), foveoschisis resulting from pit-macular syndrome (2 eyes), and proliferative diabetic retinopathy (1 eye). Apart from the 5 eyes with idiopathic or traumatic macular holes, macular holes developed in the other 5 eyes after initial vitrectomies with ILM removal. In all eyes, regular macular hole surgery failed to achieve closure. The main outcome measures used in this study were macular hole closure and best-corrected visual acuity (BCVA). Macular holes were closed successfully in 9 eyes (90%) after autologous transplantation of the ILM. The postoperative BCVAs were significantly better than the preoperative BCVAs (P = .007, paired t test). Postoperative BCVAs improved by more than 0.2 logarithm of the minimal angle of resolution units in 8 eyes (80%) and were unchanged in 2 eyes (20%). Although this is a pilot study, the results suggest that autologous transplantation of the ILM may contribute to improved anatomic and visual outcomes in the treatment of refractory macular holes and may warrant further investigation. Copyright © 2014 Elsevier Inc. All rights reserved.

Omalizumab for the Treatment of Chronic Idiopathic Urticaria: Systematic Review of the Literature.

Science.gov (United States)

Tonacci, Alessandro; Billeci, Lucia; Pioggia, Giovanni; Navarra, Michele; Gangemi, Sebastiano

2017-04-01

Omalizumab is recombinant humanized monoclonal antibody to immunoglobulin E. Guidelines for the treatment of chronic idiopathic urticaria (also known as chronic spontaneous urticaria) recommend the use of omalizumab as third-line therapy in addition to high doses of histamine receptor type 1 (H 1 ) antihistamines when they are unsuccessful as first- and second-line therapy. We performed a systematic review of the literature to identify studies that evaluated the efficacy of omalizumab for the treatment of chronic idiopathic urticaria, in both controlled and real-world settings, to assess its potential role as a preferred therapy. The PubMed, ScienceDirect, LILACS (Latin American and Caribbean Health Sciences Literature), and Google Scholar databases were searched between January 1, 2000, and November 21, 2016. The search was limited to articles published in peer-reviewed journals in the English language, and 29 studies were included in this review. Omalizumab 300 mg administered every 4 weeks appears to be the most effective and safe dosage, with a rapid response time, for the treatment of chronic idiopathic urticaria, with few minor adverse effects, and appears to be safe in the offspring of pregnant patients who received the drug. However, as published studies of omalizumab are sparse, future studies are warranted. When findings are confirmed in larger studies, due to its efficacy, safety, and increased benefit/cost ratio, omalizumab could become the preferred method of treatment for chronic idiopathic urticaria in patients unresponsive to H 1 antihistamines. © 2017 Pharmacotherapy Publications, Inc.

Exercise therapy in juvenile idiopathic arthritis: a Cochrane Review

NARCIS (Netherlands)

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

2008-01-01

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

Providence nighttime bracing, in treatment of adolescent idiopathic scoliosis

DEFF Research Database (Denmark)

Simony, A.; Beuschau, Inge; Quisth, Lena

2015-01-01

Introduction: Since 2008 the non-surgical treatment of adolescent idiopathic scoliosis (AIS) in the southern part of Denmark, went from full-time bracing with Boston brace, to Providence night-time bracing. Methods: Since 2008, skeletally immature patients diagnosed with AIS and a primary curve w...

INTERNAL LIMITING MEMBRANE PEELING IN MACULAR HOLE SURGERY; WHY, WHEN, AND HOW?

Science.gov (United States)

Chatziralli, Irini P; Theodossiadis, Panagiotis G; Steel, David H W

2018-05-01

To review the current rationale for internal limiting membrane (ILM) peeling in macular hole (MH) surgery and to discuss the evidence base behind why, when, and how surgeons peel the ILM. Review of the current literature. Pars plana vitrectomy is an effective treatment for idiopathic MH, and peeling of the ILM has been shown to improve closure rates and to prevent postoperative reopening. However, some authors argue against ILM peeling because it results in a number of changes in retinal structure and function and may not be necessary in all cases. Furthermore, the extent of ILM peeling optimally performed and the most favorable techniques to remove the ILM are uncertain. Several technique variations including ILM flaps, ILM scraping, and foveal sparing ILM peeling have been described as alternatives to conventional peeling in specific clinical scenarios. Internal limiting membrane peeling improves MH closure rates but can have several consequences on retinal structure and function. Adjuvants to aid peeling, instrumentation, technique, and experience may all alter the outcome. Hole size and other variables are important in assessing the requirement for peeling and potentially its extent. A variety of evolving alternatives to conventional peeling may improve outcomes and need further study.

Effects of Low-frequency Current Sacral Dermatome Stimulation on Idiopathic Slow Transit Constipation.

Science.gov (United States)

Kim, Jin-Seop; Yi, Seung-Ju

2014-06-01

[Purpose] This study aimed to determine whether low-frequency current therapy can be used to reduce the symptoms of idiopathic slow transit constipation (ISTC). [Subjects] Fifteen patients (ten male and five female) with idiopathic slow transit constipation were enrolled in the present study. [Results] Bowel movements per day, bowel movements per week, and constipation assessment scale scores significantly improved after low-frequency current simulation of S2-S3. [Conclusion] Our results show that stimulation with low-frequency current of the sacral dermatomes may offer therapeutic benefits for a subject of patients with ISTC.

Diagnostic value of optical coherence tomography for intracranial pressure in idiopathic intracranial hypertension

DEFF Research Database (Denmark)

Skau, M; Yri, H; Sander, B

2013-01-01

BACKGROUND: Idiopathic intracranial hypertension (IIH) is a condition of raised intracranial pressure (ICP) in the absence of space-occupying lesions or other known etiology. It primarily affects young obese females, and potentially causes permanent visual loss due to papilledema and secondary...... optic atrophy. The aim of this study was to evaluate the diagnostic value of optical coherence tomography (OCT) as a marker for CSF opening pressure in patients with idiopathic intracranial hypertension (IIH). METHODS: We conducted a case-control study of 20 newly diagnosed, 21 long-term IIH patients...

Wind turbines and idiopathic symptoms

DEFF Research Database (Denmark)

Blanes-Vidal, Victoria; Schwartz, Joel

2016-01-01

Whether or not wind turbines pose a risk to human health is a matter of heated debate. Personal reactions to other environmental exposures occurring in the same settings as wind turbines may be responsible of the reported symptoms. However, these have not been accounted for in previous studies. We...... investigated whether there is an association between residential proximity to wind turbines and idiopathic symptoms, after controlling for personal reactions to other environmental co-exposures. We assessed wind turbine exposures in 454 residences as the distance to the closest wind turbine (Dw) and number...... of wind turbines

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

Science.gov (United States)

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

2013-09-01

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

Toxocara Seroprevalence in Patients with Idiopathic Parkinson's Disease: Chance Association or Coincidence?

Directory of Open Access Journals (Sweden)

Tuncay Çelik

2013-01-01

Full Text Available Most cases of idiopathic Parkinson disease (IPD are believed to be due to a combination of genetic and environmental factors. The purpose of this study is to investigate the relationship between toxocariasis and Parkinson disease (PD. Patients were selected from people who were admitted to the Movement Disorders Branch, Neurology Department of Elazığ University Faculty of Medicine Elazığ, Turkey. We studied specific IgG antibodies against Toxocara canis (T. canis in 50 patients with idiopathic Parkinson and 50 healthy volunteers. We investigated the clinical history of three patients infected with T. canis. We also studied specific IgG antibodies against Toxoplasma gondii in these groups. Antibodies anti-Toxocara canis were found in 3 idiopathic PD (6% (P=0.121 and antibody titer was not found in control. A patient had history of the presence of dog in current dog ownership. We did not detect any statistically significant association between T. canis and IPD. But, we believe that further comprehensive studies are required for understanding whether there is a causal relation between toxocariasis and PD. We didn’t find possible association between Toxoplasma gondii and IPD (P=0.617.

Idiopathic Intracranial Hypertension – Pathophysiology Based on Case Series

Directory of Open Access Journals (Sweden)

Ljubisavljević Srdjan

2016-09-01

Full Text Available According to the definition, idiopathic intracranial hypertension (IIH is a pathological state characterized by an increase in intracranial pressure; however, there are no obvious intracranial pathological processes. The pathophysiology of this disorder is not clear, although there are many reports related to it.

The aetiology of idiopathic scoliosis : biomechanical and neuromuscular factors

NARCIS (Netherlands)

Veldhuizen, AG; Webb, PJ

The aetiology of adolescent idiopathic scoliosis (AIS) remains an enigma. In the literature there are two opinions: one believes a deviating growth pattern is responsible for the condition - patients with AIS lend to be growing faster/be taller - while the other opinion assumes that the growth

Exercise testing and fitness training in juvenile idiopathic arthritis

NARCIS (Netherlands)

Singh-Grewal, D.

2010-01-01

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

Treatment of Juvenile Idiopathic Arthritis-Associated Uveitis

OpenAIRE

Oray, Merih; Tu?al-Tutkun, ?lknur

2016-01-01

Pediatric uveitis may be a serious health problem because of the lifetime burden of vision loss due to severe complications if the problem is not adequately treated. Juvenile idiopathic arthritis (JIA)-associated uveitis is characterized by insidious onset and potentially blinding chronic anterior uveitis. Periodic ophthalmologic screening is of utmost importance for early diagnosis of uveitis. Early diagnosis and proper immunomodulatory treatment are essential for good visual progno...

Correlation of Seminal Plasma Total Antioxidant Capacity and Malondialdehyde Levels With Sperm Parameters in Men With Idiopathic Infertility

Directory of Open Access Journals (Sweden)

Fazeli

2016-01-01

Full Text Available Background Oxidative stress is the result of an imbalance between the production and scavenging of reactive oxygen species (ROS. Recently, oxidative stress has been introduced as a major cause of male infertility. Objectives The aim of the present study was to determine the correlation between total antioxidant capacity (TAC and malondialdehyde (MDA as markers of oxidative stress in relation to idiopathic male infertility and sperm parameters. Patients and Methods This case control study was conducted using 35 men with idiopathic infertility and 34 men with proven fertility. Seminal plasma TAC and MDA were measured by ferric reducing ability of plasma (FRAP and thiobarbituric acid (TBA reaction methods, respectively. Results Seminal TAC levels were significantly lower and seminal MDA levels were significantly higher in men with idiopathic infertility than in fertile men (P < 0.0001 and P = 0.004, respectively. A positive correlation was shown between sperm motility, sperm morphology, and TAC levels in men with idiopathic infertility (P = 0.002 and P = 0.002, respectively. In addition, there was a correlation between sperm motility and TAC levels in fertile men (P = 0.005. There was no correlation between sperm count and TAC levels in either men with idiopathic infertility or in fertile men. Negative correlations were observed between MDA levels and sperm motility, morphology, and sperm count only in men with idiopathic infertility (P = 0.003, P = 0.001, and P = 0.006, respectively. Conclusions Our results show that oxidative stress could play an important role in male infertility as well as in sperm motility and sperm morphology.

Idiopathic Transverse Myelitis and Neuromyelitis Optica: Clinical Profiles, Pathophysiology and Therapeutic Choices

Science.gov (United States)

Awad, Amer; Stüve, Olaf

2011-01-01

Transverse myelitis is a focal inflammatory disorder of the spinal cord which may arise due to different etiologies. Transverse myelitis may be idiopathic or related/secondary to other diseases including infections, connective tissue disorders and other autoimmune diseases. It may be also associated with optic neuritis (neuromyelitis optica), which may precede transverse myelitis. In this manuscript we review the pathophysiology of different types of transverse myelitis and neuromyelitis optica and discuss diagnostic criteria for idiopathic transverse myelitis and risk of development of multiple sclerosis after an episode of transverse myelitis. We also discuss treatment options including corticosteroids, immunosuppressives and monoclonal antibodies, plasma exchange and intravenous immunoglobulins. PMID:22379456

Anti-TNF therapy for juvenile idiopathic arthritis-related uveitis

Science.gov (United States)

Semeraro, Francesco; Arcidiacono, Barbara; Nascimbeni, Giuseppe; Angi, Martina; Parolini, Barbara; Costagliola, Ciro

2014-01-01

Juvenile idiopathic arthritis-related uveitis is the most common type of uveitis in childhood and one of the main causes of visual impairment in children. The introduction of biological treatment has widened the range of therapeutic options for children with uveitis refractory to standard nonbiologic immunosuppressants. Data from clinical trials suggest that both adalimumab and infliximab have demonstrated effectiveness and safety in open-label studies, although no large, randomized, controlled trials have been reported so far. The role of etanercept in treating juvenile idiopathic arthritis-related uveitis is not yet well defined. In our experience, anti-tumor necrosis factor therapy has been shown to be more effective than steroids and/or methotrexate in treating uveitis. Up to now, tumor necrosis factor blocking compounds have been reserved for the treatment of the most severe cases of refractory uveitis, and larger prospective clinical trials are required in order to better assess the safety of these new compounds. PMID:24711694

Anti-TNF therapy for juvenile idiopathic arthritis-related uveitis.

Science.gov (United States)

Semeraro, Francesco; Arcidiacono, Barbara; Nascimbeni, Giuseppe; Angi, Martina; Parolini, Barbara; Costagliola, Ciro

2014-01-01

Juvenile idiopathic arthritis-related uveitis is the most common type of uveitis in childhood and one of the main causes of visual impairment in children. The introduction of biological treatment has widened the range of therapeutic options for children with uveitis refractory to standard nonbiologic immunosuppressants. Data from clinical trials suggest that both adalimumab and infliximab have demonstrated effectiveness and safety in open-label studies, although no large, randomized, controlled trials have been reported so far. The role of etanercept in treating juvenile idiopathic arthritis-related uveitis is not yet well defined. In our experience, anti-tumor necrosis factor therapy has been shown to be more effective than steroids and/or methotrexate in treating uveitis. Up to now, tumor necrosis factor blocking compounds have been reserved for the treatment of the most severe cases of refractory uveitis, and larger prospective clinical trials are required in order to better assess the safety of these new compounds.

Anti-adalimumab antibodies in juvenile idiopathic arthritis-related uveitis.

Science.gov (United States)

Leinonen, Sanna T; Aalto, Kristiina; Kotaniemi, Kaisu M; Kivelä, Tero T

2017-01-01

To evaluate the association of adalimumab trough levels and anti-adalimumab antibodies with activity of uveitis in juvenile idiopathic arthritis-related uveitis. This was a retrospective observational case series in a clinical setting at the Department of Ophthalmology, Helsinki University Hospital, Finland in 2014-2016. Thirty-one paediatric patients with chronic anterior juvenile idiopathic arthritis-related uveitis in 58 eyes and who had been on adalimumab ≥6 months were eligible for the study. Uveitis activity during adalimumab treatment, adalimumab trough levels and anti-adalimumab antibody levels were recorded. Anti-adalimumab antibody levels ≥12 AU /ml were detected in nine patients (29%). This level of anti-adalimumab antibodies was associated with a higher grade of uveitis (puveitis that was not in remission (p=0.001) and with lack of concomitant methotrexate therapy (p=0.043). In patients with anti-adalimumab antibody levels uveitis (p=0.86). Adalimumab treatment might be better guided by monitoring anti-adalimumab antibody formation in treating JIA-related uveitis.

Adult-onset photosensitivity: clinical significance and epilepsy syndromes including idiopathic (possibly genetic) photosensitive occipital epilepsy.

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Koutroumanidis, Michalis; Tsirka, Vasiliki; Panayiotopoulos, Chrysostomos

2015-09-01

To evaluate the clinical associations of adult-onset photosensitivity, we studied the clinical and EEG data of patients who were referred due to a possible first seizure and who had a photoparoxysmal response on their EEG. Patients with clinical evidence of photosensitivity before the age of 20 were excluded. Of a total of 30 patients, four had acute symptomatic seizures, two had vasovagal syncope, and 24 were diagnosed with epilepsy. Nine of the 24 patients had idiopathic (genetic) generalized epilepsies and predominantly generalized photoparoxysmal response, but also rare photically-induced seizures, while 15 had exclusively, or almost exclusively, reflex photically-induced occipital seizures with frequent secondary generalization and posterior photoparoxysmal response. Other important differences included a significantly older age at seizure onset and paucity of spontaneous interictal epileptic discharges in patients with photically-induced occipital seizures; only a quarter of these had occasional occipital spikes, in contrast to the idiopathic (genetic) generalized epilepsy patients with typically generalized epileptic discharges. On the other hand, both groups shared a positive family history of epilepsy, common seizure threshold modulators (such as tiredness and sleep deprivation), normal neurological examination and MRI, a generally benign course, and good response to valproic acid. We demonstrated that photosensitivity can first occur in adult life and manifest, either as idiopathic (possibly genetic) photosensitive occipital epilepsy with secondary generalization or as an EEG, and less often, a clinical/EEG feature of idiopathic (genetic) generalized epilepsies. Identification of idiopathic photosensitive occipital epilepsy fills a diagnostic gap in adult first-seizure epileptology and is clinically important because of its good response to antiepileptic drug treatment and fair prognosis.

Idiopathic focal epilepsies: the "lost tribe".

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Pal, Deb K; Ferrie, Colin; Addis, Laura; Akiyama, Tomoyuki; Capovilla, Giuseppe; Caraballo, Roberto; de Saint-Martin, Anne; Fejerman, Natalio; Guerrini, Renzo; Hamandi, Khalid; Helbig, Ingo; Ioannides, Andreas A; Kobayashi, Katsuhiro; Lal, Dennis; Lesca, Gaetan; Muhle, Hiltrud; Neubauer, Bernd A; Pisano, Tiziana; Rudolf, Gabrielle; Seegmuller, Caroline; Shibata, Takashi; Smith, Anna; Striano, Pasquale; Strug, Lisa J; Szepetowski, Pierre; Valeta, Thalia; Yoshinaga, Harumi; Koutroumanidis, Michalis

2016-09-01

The term idiopathic focal epilepsies of childhood (IFE) is not formally recognised by the ILAE in its 2010 revision (Berg et al., 2010), nor are its members and boundaries precisely delineated. The IFEs are amongst the most commonly encountered epilepsy syndromes affecting children. They are fascinating disorders that hold many "treats" for both clinicians and researchers. For example, the IFEs pose many of the most interesting questions central to epileptology: how are functional brain networks involved in the manifestation of epilepsy? What are the shared mechanisms of comorbidity between epilepsy and neurodevelopmental disorders? How do focal EEG discharges impact cognitive functioning? What explains the age-related expression of these syndromes? Why are EEG discharges and seizures so tightly locked to slow-wave sleep? In the last few decades, the clinical symptomatology and the respective courses of many IFEs have been described, although they are still not widely appreciated beyond the specialist community. Most neurologists would recognise the core syndromes of IFE to comprise: benign epilepsy of childhood with centro-temporal spikes or Rolandic epilepsy (BECTS/RE); Panayiotopoulos syndrome; and the idiopathic occipital epilepsies (Gastaut and photosensitive types). The Landau-Kleffner syndrome and the related (idiopathic) epilepsy with continuous spikes and waves in sleep (CSWS or ESES) are also often included, both as a consequence of the shared morphology of the interictal discharges and their potential evolution from core syndromes, for example, CSWS from BECTS. Atypical benign focal epilepsy of childhood also has shared electro-clinical features warranting inclusion. In addition, a number of less well-defined syndromes of IFE have been proposed, including benign childhood seizures with affective symptoms, benign childhood epilepsy with parietal spikes, benign childhood seizures with frontal or midline spikes, and benign focal seizures of adolescence. The

Idiopathic urolithiasis in Tunisian children: A report of 134 cases

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A Alaya

2013-01-01

Full Text Available We evaluated the metabolic and the nutritional aspects of 134 urolithiasis children in order to outline the characteristics of idiopathic urolithiasis in children. This prospective study group of 134 children (56 females, 78 males with renal calculi was evaluated. The age range of the patients was six months to 16 years. A dietary survey was performed on every child. All patients were investigated with respect to stone localization and serum and urine risk factors. Statistical analysis of data was carried out using software SPSS 11.0 for Windows. Hypercalciuria was the most common risk factor detected in this group (28.3%. A decrease of water intake was noted in all age groups, especially in the rural area (549.6 mL/day vs. 1150.6 mL/day, and there was an increase in animal protein intake in 17 cases (mean: 1.9 g/kg. In addition, increased intake of starchy foods and food with high oxalate content (sorgum were detected in the ten to 16 years age group (51% of our study. Calcium oxalate monohydrate represents the principal component of idiopathic stone (58.2%, which is more frequent in children (68% than in infants (51.7% (P <0.02. The major etiology of idiopathic urolithiasis highlights the influence of dietary habit in stone formers in our country. The increased occurrence of calcium oxalate stones in school age children confirms the change in the etiology of urolithiasis according to age.

Analysis of T Cell Subsets in Adult Primary/Idiopathic Minimal Change Disease: A Pilot Study

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Francisco Salcido-Ochoa

2017-01-01

Full Text Available Aim. To characterise infiltrating T cells in kidneys and circulating lymphocyte subsets of adult patients with primary/idiopathic minimal change disease. Methods. In a cohort of 9 adult patients with primary/idiopathic minimal change recruited consecutively at disease onset, we characterized (1 infiltrating immune cells in the kidneys using immunohistochemistry and (2 circulating lymphocyte subsets using flow cytometry. As an exploratory analysis, association of the numbers and percentages of both kidney-infiltrating immune cells and the circulating lymphocyte subsets with kidney outcomes including deterioration of kidney function and proteinuria, as well as time to complete clinical remission up to 48 months of follow-up, was investigated. Results. In the recruited patients with primary/idiopathic minimal change disease, we observed (a a dominance of infiltrating T helper 17 cells and cytotoxic cells, comprising cytotoxic T cells and natural killer cells, over Foxp3+ Treg cells in the renal interstitium; (b an increase in the circulating total CD8+ T cells in peripheral blood; and (c an association of some of these parameters with kidney function and proteinuria. Conclusions. In primary/idiopathic minimal change disease, a relative numerical dominance of effector over regulatory T cells can be observed in kidney tissue and peripheral blood. However, larger confirmatory studies are necessary.

Lung microbiome and disease progression in idiopathic pulmonary fibrosis: an analysis of the COMET study.

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Han, MeiLan K; Zhou, Yueren; Murray, Susan; Tayob, Nabihah; Noth, Imre; Lama, Vibha N; Moore, Bethany B; White, Eric S; Flaherty, Kevin R; Huffnagle, Gary B; Martinez, Fernando J

2014-07-01

The role of the lung microbiome in the pathogenesis of idiopathic pulmonary fibrosis is unknown. We investigated whether unique microbial signatures were associated with progression of idiopathic pulmonary fibrosis. Patients (aged 35-80 years) with idiopathic pulmonary fibrosis within 4 years of diagnosis from the Correlating Outcomes with biochemical Markers to Estimate Time-progression (COMET) in idiopathic pulmonary fibrosis study were followed up for a maximum of 80 weeks. Progression-free survival was defined as time to death, acute exacerbation, lung transplant, or decrease in forced vital capacity (FVC) of 10% or greater or decrease in diffusion capacity of the lung (DLCO) of 15% or greater. DNA was isolated from 55 samples of bronchoscopic alveolar lavage. 454 pyrosequencing was used to assign operational taxonomic units (OTUs) to bacteria based on a 3% sequence divergence. Adjusted Cox models were used to identify OTUs that were significantly associated with progression-free survival at a pidiopathic pulmonary fibrosis is associated with the presence of specific members within the Staphylococcus and Streptococcus genera. Additional research will be needed to identify the specific bacterial species and to ascertain whether this is a causal association. National Institutes of Health. Copyright © 2014 Elsevier Ltd. All rights reserved.

Eisenmenger syndrome and idiopathic pulmonary arterial hypertension: do parenchymal lung changes reflect aetiology?

International Nuclear Information System (INIS)

Griffin, N.; Allen, D.; Wort, J.; Rubens, M.; Padley, S.

2007-01-01

Aim: To document the pulmonary vascular changes on thin-section computed tomography (CT) in patients with Eisenmenger syndrome and idiopathic pulmonary arterial hypertension, and to determine whether there is any correlation with pulmonary arterial pressures or the aetiology of pulmonary hypertension. Material and methods: From the National Pulmonary Hypertension Database, we identified eight patients with idiopathic pulmonary arterial hypertension and 20 patients with Eisenmenger syndrome (secondary to a ventriculoseptal defect) who had also undergone contrast-enhanced thin-section CT. CT studies were reviewed for the presence of centrilobular nodules, mosaicism, neovascularity, and bronchial artery hypertrophy. Haemodynamic data were also reviewed. Results: Centrilobular nodules, mosaicism, and neovascularity were seen in both patient groups (p > 0.05). A significantly higher number of enlarged bronchial arteries were seen in patients with Eisenmenger syndrome. There was no correlation with pulmonary arterial pressures. Conclusion: Patients with idiopathic pulmonary arterial hypertension and Eisenmenger syndrome demonstrated similar pulmonary vascular changes on CT. These changes did not predict the underlying cause of pulmonary hypertension or its severity

Conservative treatment of idiopathic spontaneous pneumoperitoneum in a bedridden patient: a case report.

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Tanaka, Ryo; Kameyama, Hitoshi; Nagahashi, Masayuki; Kanda, Tatsuo; Ichikawa, Hiroshi; Hanyu, Takaaki; Ishikawa, Takashi; Kobayashi, Takashi; Sakata, Jun; Kosugi, Shin-Ichi; Wakai, Toshifumi

2015-01-01

Idiopathic spontaneous pneumoperitoneum is a rare condition that is characterized by intraperitoneal gas for which no clear etiology has been identified. We report here a case of idiopathic spontaneous pneumoperitoneum, which was successfully managed by conservative treatment. A 77-year-old woman who was bedridden with speech disability as a sequela of brain hemorrhage presented at our hospital with a 1-day history of abdominal distention. On physical examination, she had stable vital signs and slight epigastric tenderness on deep palpation without any other signs of peritonitis. A chest radiograph and computed tomography showed that a large amount of free gas extended into the upper abdominal cavity. Esophagogastroduodenoscopy revealed no perforation of the upper gastrointestinal tract. The patient was diagnosed with idiopathic spontaneous pneumoperitoneum, and conservative treatment was selected. The abdominal distension rapidly disappeared, and the patient resumed oral intake on the 5th hospital day without deterioration of symptoms. Knowledge of this rare disease and accurate diagnosis with findings of clinical imaging might contribute towards refraining from unnecessary laparotomy.

Eisenmenger syndrome and idiopathic pulmonary arterial hypertension: do parenchymal lung changes reflect aetiology?

Energy Technology Data Exchange (ETDEWEB)

Griffin, N. [Royal Brompton and Harefield NHS Trust, London (United Kingdom)]. E-mail: nyreegriffin@hotmail.com; Allen, D. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Wort, J. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Rubens, M. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Padley, S. [Royal Brompton and Harefield NHS Trust, London (United Kingdom)

2007-06-15

Aim: To document the pulmonary vascular changes on thin-section computed tomography (CT) in patients with Eisenmenger syndrome and idiopathic pulmonary arterial hypertension, and to determine whether there is any correlation with pulmonary arterial pressures or the aetiology of pulmonary hypertension. Material and methods: From the National Pulmonary Hypertension Database, we identified eight patients with idiopathic pulmonary arterial hypertension and 20 patients with Eisenmenger syndrome (secondary to a ventriculoseptal defect) who had also undergone contrast-enhanced thin-section CT. CT studies were reviewed for the presence of centrilobular nodules, mosaicism, neovascularity, and bronchial artery hypertrophy. Haemodynamic data were also reviewed. Results: Centrilobular nodules, mosaicism, and neovascularity were seen in both patient groups (p > 0.05). A significantly higher number of enlarged bronchial arteries were seen in patients with Eisenmenger syndrome. There was no correlation with pulmonary arterial pressures. Conclusion: Patients with idiopathic pulmonary arterial hypertension and Eisenmenger syndrome demonstrated similar pulmonary vascular changes on CT. These changes did not predict the underlying cause of pulmonary hypertension or its severity.

The Role of Diffusion-Weighted MRI in Differentiation of Idiopathic Acute Transverse Myelitis and Acute Spinal Cord Infarction

International Nuclear Information System (INIS)

Kim, Yeo Goon; Lee, Joon Woo; Kim, Jae Hyoung; Kang, Heung Sik; Park, Kyung Seok

2011-01-01

To compare the diffusion characteristics of idiopathic acute transverse myelitis (ATM) and acute spinal cord infarction (SCI). Diffusion-weighted images (DWI) and an apparent diffusion coefficient (ADC) map were prospectively obtained from patients diagnosed with myelopathy between February 2006 and April 2009. Inclusion criteria included 1) the presence of an intramedullary T2-high signal intensity and 2) a final diagnosis of idiopathic ATM or SCI established by one neurologist. In total, 13 patients (M : F = 8 : 5; mean age, 39.5 years; range, 29-50 years) with idiopathic ATM and seven patients (M : F = 2 : 5; mean age, 58 years; range, 48-75 years) with SCI were included in this study. Two radiologists evaluated the DWIs and ADC map in consensus. The extent of the cord signal change was also evaluated on T2-weighted sagittal images. Among the 16 patients with ATM, 14 patients showed iso-signal on an ADC map, but one case showed restricted diffusion and another showed increased diffusion on the ADC map. Among the seven patients with SCI, five patients showed restricted diffusion. Idiopathic ATM usually does not demonstrate restricted diffusion, which can be a clue to differentiate it from SCI. However, idiopathic ATM with larger segment involvement can show focal diffusion restriction.

Idiopathic pulmonary fibrosis

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Noble Paul W

2008-03-01

Full Text Available Abstract Idiopathic pulmonary fibrosis (IPF is a non-neoplastic pulmonary disease that is characterized by the formation of scar tissue within the lungs in the absence of any known provocation. IPF is a rare disease which affects approximately 5 million persons worldwide. The prevalence is estimated to be slightly greater in men (20.2/100,000 than in women (13.2/100,000. The mean age at presentation is 66 years. IPF initially manifests with symptoms of exercise-induced breathless and dry coughing. Auscultation of the lungs reveals early inspiratory crackles, predominantly located in the lower posterior lung zones upon physical exam. Clubbing is found in approximately 50% of IPF patients. Cor pulmonale develops in association with end-stage disease. In that case, classic signs of right heart failure may be present. Etiology remains incompletely understood. Some environmental factors may be associated with IPF (cigarette smoking, exposure to silica and livestock. IPF is recognized on high-resolution computed tomography by peripheral, subpleural lower lobe reticular opacities in association with subpleural honeycomb changes. IPF is associated with a pathological lesion known as usual interstitial pneumonia (UIP. The UIP pattern consists of normal lung alternating with patches of dense fibrosis, taking the form of collagen sheets. The diagnosis of IPF requires correlation of the clinical setting with radiographic images and a lung biopsy. In the absence of lung biopsy, the diagnosis of IPF can be made by defined clinical criteria that were published in guidelines endorsed by several professional societies. Differential diagnosis includes other idiopathic interstitial pneumonia, connective tissue diseases (systemic sclerosis, polymyositis, rheumatoid arthritis, forme fruste of autoimmune disorders, chronic hypersensitivity pneumonitis and other environmental (sometimes occupational exposures. IPF is typically progressive and leads to significant

[Idiopathic scrotal elephantiasis. A case report].

Science.gov (United States)

López-Caballero, Ignacio; Sánchez-Ruvalcaba, Itzel; Sánchez-Martinez, Luis Carlos; Hernández-Ordoñez, Octavio; Gómez-Lara, Miguel; Flores-Carrillo, Víctor

2014-01-01

Penoscrotal lymphedema (scrotal elephantiasis) is a condition that has been described in areas in which filariasis is endemic. This paper presents a case of a 45-year-old man with idiopathic lymphedema isolated to the scrotum. After acquired causes of lymphedema were ruled out, the patient was treated with scrotectomy and penoscrotal reconstruction. Currently, the patient is receiving follow-up care without evidence of recurrence. Penoscrotal lymphedema may cause symptoms of weakness, immobility and emotional disturbance. Surgery procedure provides a satisfactory cosmetical and functional outcome.

Body composition in children with juvenile idiopathic arthritis: effect ...

African Journals Online (AJOL)

Introduction: the aim of this study was to evaluate the relationship between macronutrient intake, body composition (lean body mass and fat mass) and bone mineral content in Moroccan children with juvenile idiopathic arthritis (JIA). Methods: a cross-sectional study, conducted between May 2010 and June 2011, covering ...

Hyperoxaluria in idiopathic calcium nephrolithiasis--what are the limits?

DEFF Research Database (Denmark)

Osther, P J

1999-01-01

OBJECTIVE: The object of this study was to investigate the role for measurement of 24-h renal oxalate excretion in the evaluation of idiopathic calcium stone formers. MATERIALS AND METHODS: Renal excretion rates of oxalate and creatinine were measured in 24-h urines in 46 consecutive male recurrent...

Idiopathic sclerosing encapsulating peritonitis (or abdominal cocoon).

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Serafimidis, Costas; Katsarolis, Ioannis; Vernadakis, Spyros; Rallis, George; Giannopoulos, George; Legakis, Nikolaos; Peros, George

2006-02-13

Idiopathic sclerosing encapsulating peritonitis (or abdominal cocoon) is a rare cause of small bowel obstruction, especially in adult population. Diagnosis is usually incidental at laparotomy. We discuss one such rare case, outlining the fact that an intra-operative surprise diagnosis could have been facilitated by previous investigations. A 56 year-old man presented in A&E department with small bowel ileus. He had a history of 6 similar episodes of small bowel obstruction in the past 4 years, which resolved with conservative treatment. Pre-operative work-up did not reveal any specific etiology. At laparotomy, a fibrous capsule was revealed, in which small bowel loops were encased, with the presence of interloop adhesions. A diagnosis of abdominal cocoon was established and extensive adhesiolysis was performed. The patient had an uneventful recovery and follow-up. Idiopathic sclerosing encapsulating peritonitis, although rare, may be the cause of a common surgical emergency such as small bowel ileus, especially in cases with attacks of non-strangulating obstruction in the same individual. A high index of clinical suspicion may be generated by the recurrent character of small bowel ileus combined with relevant imaging findings and lack of other plausible etiologies. Clinicians must rigorously pursue a preoperative diagnosis, as it may prevent a "surprise" upon laparotomy and result in proper management.

Risk Factors for Neurodegeneration in Idiopathic REM sleep Behavior Disorder: A Multicenter Study

Science.gov (United States)

Postuma, RB; Iranzo, A; Hogl, B; Arnulf, I; Ferini-Strambi, L; Manni, R; Miyamoto, T.; Oertel, W; Dauvilliers, Y; Ju, Y; Puligheddu, M; Sonka, K; Pelletier, A; Santamaria, J; Frauscher, B; Leu-Semenescu, S; Zucconi, M; Terzaghi, M; Miyamoto, M.; Unger, MM; Carlander, B; Fantini, ML; Montplaisir, JY

2018-01-01

Objective To assess whether risk factors for Parkinson’s disease and Dementia with Lewy bodies increase rate of defined neurodegenerative disease in idiopathic REM sleep behavior disorder Methods 12 centers administered a detailed questionnaire assessing risk factors for neurodegenerative synucleinopathy to patients with idiopathic REM sleep behavior disorder. Variables included demographics, lifestyle factors, pesticide exposures, occupation, co-morbid conditions, medication use, family history, and autonomic/motor symptoms. After 4-years follow-up, patients were assessed for dementia or parkinsonism. Disease risk was assessed with Kaplan-Meier analysis, and epidemiologic variables were compared between convertors and those still idiopathic using logistic regression. Results Of 305 patients, follow-up information was available for 279, of whom 93 (33.3%) developed defined neurodegenerative disease. Disease risk was 25% at 3 years, and 41% after 5 years. Patients who converted were older (difference=4.5 years, pconversion. Although occupation was similar between groups, those who converted had a lower likelihood of pesticide exposure (occupational insecticide=2.3% vs. 9.0%). Convertors were more likely to report family history of dementia (OR=2.09), without significant differences in Parkinson’s disease or sleep disorders. Medication exposures and medical history were similar between groups. Autonomic and motor symptoms were more common among those who converted. Risk factors for primary dementia and parkinsonism were generally similar, except for a notably higher clonazepam use in dementia convertors (OR=2.6). Interpretation Patients with idiopathic RBD are at very high risk of neurodegenerative synucleinopathy. Risk factor profiles between convertors and non-convertors have both important commonalities and differences. PMID:25767079

Manual therapy as a conservative treatment for adolescent idiopathic scoliosis: a systematic review.

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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.

A new look at the etiopathogenesis ofadolescent idiopathic scoliosis

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Maciej Brzęczek

2016-06-01

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

MANAGEMENT OF CHRONIC IDIOPATHIC THROMBOTIC THROMBOCYTOPENIC PURPURA WITH SPLENECTOMY

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Mojca Modic

2002-07-01

Full Text Available Background. Treatment of thrombotic thrombocytopenic purpura (TTP with plasma exchange (PE has reduced mortality rates from 90% in untreated patients to less than 20%. Despite primary effective PE, relapses occur in up to 40% of patients. Beside PE, second line treatment options are corticosteroids, vincristine, defibrotide, prostacycline. We describe two patients with relapsing TTP treated successfully with splenectomy.Patients and methods. Case 1. A 20–year female was diagnosed in October 2000 with idiopathic chronic relapsing TTP. First episode of PE plus methylprednisolone treatment led to complet remission. Subsequent relapses occured 3, 6 and 8 months after first TTP episode. Every relaps was effectivelly treated with PE and methylprednisolone. Because of more and more frequent TTP relapses laparoscopic splenectomy was performed. Currently, 7 months after splenectomy she is in complet TTP remission.Case 2. A 18–year female, was diagnosed in January 1997 with idiopathic TTP. After PE and methylprednisolone complete remission was established. Relaps occured after nearly three years. Later on, TTP got chronic course with neurological clinical symptoms and plateled drops as soon as PE treatment was withdrawn. Besides PE and methylprednisolone she received also defibrotide. Because of chronic, PE dependant TTP, laparoscopic splenectomy was performed. Today, 15 months after splenectomy she is in complete TTP remission.Conclusions. In idiopathic relapsing and chronic TTP, PE treatment only is usually not sufficient for durable remission. Second line TTP treatment drugs have limited efficacy. Based on our experience in patients with relapsing and/or chronic TTP, laparoscopic splenectomy should be considered as a first treatment option, especialy in junger patients.

Do all patients with idiopathic pulmonary fibrosis warrant a trial of therapeutic intervention? A pro-con perspective.

Science.gov (United States)

Moodley, Yuben; Corte, Tamera; Richeldi, Luca; King, Talmadge E

2015-04-01

Idiopathic pulmonary fibrosis (IPF) is an incurable condition that is characterized by progressive pulmonary fibrosis, architectural distortion of the lung and loss of gas exchange units. Until recently, there was no effective treatment for this condition. However, there were two landmark trials published earlier this year, which have changed the management of this condition. Pirfenidone (Assessment of Pirfenidone to Confirm Efficacy and Safety in Idiopathic Pulmonary Fibrosis trial) and nintedanib (Efficacy and Safety of Nintedanib in Idiopathic Pulmonary Fibrosis-1 and -2 trials) have both demonstrated positive outcomes in patients with IPF. In this perspective, we critically discuss the role of these agents in IPF and in the broader pulmonary fibrosis population. © 2015 Asian Pacific Society of Respirology.

Pulmonary function vascular index predicts prognosis in idiopathic interstitial pneumonia

NARCIS (Netherlands)

Corte, Tamera J.; Wort, Stephen J.; MacDonald, Peter S.; Edey, Anthony; Hansell, David M.; Renzoni, Elisabetta; Maher, Toby M.; Nicholson, Andrew G.; Bandula, Steven; Bresser, Paul; Wells, Athol U.

2012-01-01

Background and objective: Pulmonary hypertension (PH) is associated with increased mortality in fibrotic idiopathic interstitial pneumonia (IIP). We hypothesize that baseline KCO (diffusing capacity of carbon monoxide/alveolar volume) and 6-month decline in KCO reflect PH, thus predicting mortality

Impact of Juvenile Idiopathic Arthritis Associated Uveitis in Early Adulthood

NARCIS (Netherlands)

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

2016-01-01

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

Impact of Juvenile Idiopathic Arthritis Associated Uveitis in Early Adulthood

NARCIS (Netherlands)

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

2016-01-01

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

Impact of juvenile idiopathic arthritis associated uveitis in early adulthood

NARCIS (Netherlands)

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

2016-01-01

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

Unravelling the incidence and etiology of chronic idiopathic axonal polyneuropathy

NARCIS (Netherlands)

Visser, N.A.

2016-01-01

Chronic idiopathic axonal polyneuropathy (CIAP) is a sensory or sensorimotor polyneuropathy that has a slowly progressive course without severe disability. CIAP is diagnosed in a significant proportion of patients with polyneuropathy, but precise figures on the incidence of polyneuropathy and CIAP

International Veterinary Epilepsy Task Force's current understanding of idiopathic epilepsy of genetic or suspected genetic origin in purebred dogs

DEFF Research Database (Denmark)

Hülsmeyer, Velia-Isabel; Fischer, Andrea; Mandigers, Paul J. J.

2015-01-01

Canine idiopathic epilepsy is a common neurological disease affecting both purebred and crossbred dogs. Various breed-specific cohort, epidemiological and genetic studies have been conducted to date, which all improved our knowledge and general understanding of canine idiopathic epilepsy, and in ...

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

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Y. M. Spivakovskiy

2015-01-01

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

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

Science.gov (United States)

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

2014-05-24

Juvenile idiopathic arthritis is a relatively common chronic disease of childhood, and is associated with persistent morbidity and extra-articular complications, one of the most common being uveitis. The introduction of biologic therapies, particularly those blocking the inflammatory mediator tumor necrosis factor-α, provided a new treatment option for juvenile idiopathic arthritis patients who were refractory to standard therapy such as non-steroidal anti-inflammatory drugs, corticosteroids and/or methotrexate. The first case was a 2-year-old girl with juvenile idiopathic arthritis and uveitis who failed to respond to treatment with anti-inflammatories, low-dose corticosteroids and methotrexate, and had growth retardation. Adalimumab 24 mg/m2 every 2 weeks and prednisone 0.5 mg/kg/day were added to methotrexate therapy; steroid tapering and withdrawal started after 1 month. After 2 months the patient showed good control of articular and ocular manifestations, and she remained in remission for 1 year, receiving adalimumab and methotrexate with no side effects, and showing significant improvement in growth. Case 2 was a 9-year-old boy with an 8-year history of juvenile idiopathic arthritis and uveitis that initially responded to infliximab, but relapse occurred after 2 years off therapy. After switching to adalimumab, and adjusting doses of both adalimumab and methotrexate based on body surface area, the patient showed good response and corticosteroids were tapered and withdrawn after 6 months; the patient remained in remission taking adalimumab and methotrexate. The final case was a 5-year-old girl with juvenile idiopathic arthritis for whom adalimumab was added to methotrexate therapy after three flares of uveitis. The patient had two subsequent episodes of uveitis that responded well to local therapy, but was then free of both juvenile idiopathic arthritis and uveitis symptoms, allowing methotrexate and then adalimumab to be stopped; the patient remained in drug

IDIOPATHIC LARYN GEAL STENOSIS - A VERY RARE CASE

OpenAIRE

Sudip Kumar; Ruma; Rajesh; Arvind Kumar; Subhendu

2014-01-01

A 35 year old lady presented in the Out Patient Department with cough , dyspnea and gradual hoarseness for last 5 years. After proper history taking and thorough clinical examination , diagnosis of Laryngeal Stenosis was made. Subsequently by excluding important causes of Laryngeal Stenosis like trauma , chronic infection , tuberculosis and other granulomatous diseases , the diagnosis of Idiopathic Laryngeal Stenosis was established KEYWORDS ...

[Normal lung volumes in patients with idiopathic pulmonary fibrosis and emphysema].

Science.gov (United States)

Casas, Juan Pablo; Abbona, Horacio; Robles, Adriana; López, Ana María

2008-01-01

Pulmonary function tests in idiopathic pulmonary fibrosis characteristically show a restrictive pattern, resulting from reduction of pulmonary compliance due to diffuse fibrosis. Conversely, an obstructive pattern with hyperinflation results in emphysema by loss of elastic recoil, expiratory collapse of the peripheral airways and air trapping. Previous reports suggest that when both diseases coexist, pulmonary volumes are compensated and a smaller than expected reduction or even normal lung volumes can be found. We report 4 male patients of 64, 60, 73 and 70 years, all with heavy cigarette smoking history and progressive breathlessness. Three of them had severe limitation in their quality of life. All four showed advanced lung interstitial involvement, at high resolution CT scan, fibrotic changes predominantly in the subpleural areas of lower lung fields and concomitant emphysema in the upper lobes. Emphysema and pulmonary fibrosis was confirmed by open lung biopsy in one patient. The four patients showed normal spirometry and lung volumes with severe compromise of gas exchange and poor exercise tolerance evaluated by 6 minute walk test. Severe pulmonary arterial hypertension was also confirmed in three patients. Normal lung volumes does not exclude diagnosis of idiopathic pulmonary fibrosis in patients with concomitant emphysema. The relatively preserved lung volumes may underestimate the severity of idiopathic pulmonary fibrosis and attenuate its effects on lung function parameters.

Idiopathic hypereosinophilic syndrome involving the liver: CT features vs. peripheral eosinophilia

Energy Technology Data Exchange (ETDEWEB)

Kim, Kyung Sook; Lee, Moon Gyu; Won, Young Chul; Lee, Eun Hye; Noh, Han Na; Ha, Hyun Kwon; Kim, Pyo Nyun; Auh, Yong Ho [Ulsan Univ. College of Medicine, Seoul (Korea, Republic of)

1997-10-01

To correlate CT features with peripheral eosinophilia in patients with idiopathic hypereosinophilic syndrome involving the liver. During the last three years, features of liver involvement in nine of 20 patients with idiopathic hypereosinophilic syndrome were evaluated on CT. The shape and distribution of intrahepatic low densities and the presence of hepatomegaly and/or splenomegaly were reviewed on CT, and the percentage of eosinophils in peripheral blood was also determined. In seven cases, interval change in hepatic lesion and the percentage of eosinophils were reviewed on follow-up examination. On initial CT, varying low-density patterns were seen in the liver in all cases; hepatomegaly was seen in four cases, and hepatosplenomegaly in two. The percentage of eosinophils was 89% in a case with diffuse patch low densities in the liver, 65-85% in three cases with numerous nodular low density lesions, 12-29% in four cases with multiple (below ten) nodular or small geographic hypodense lesions, and 24% in a case with a single nodular hypodense lesion. On follow-up CT, seven patients showed a decrease in the percentage of eosinophils, and in six, improved intrahepatic low densities were seen. On CT, intrahepatic low densities were seen in patients with idiopathic hypereosinophilic syndrome, and these were distributed more extensively when peripheral eosinophilia was more severe. With improvement in peripheral eosinophilia, the low densities also improved.

Idiopathic hypereosinophilic syndrome involving the liver: CT features vs. peripheral eosinophilia

International Nuclear Information System (INIS)

Kim, Kyung Sook; Lee, Moon Gyu; Won, Young Chul; Lee, Eun Hye; Noh, Han Na; Ha, Hyun Kwon; Kim, Pyo Nyun; Auh, Yong Ho

1997-01-01

To correlate CT features with peripheral eosinophilia in patients with idiopathic hypereosinophilic syndrome involving the liver. During the last three years, features of liver involvement in nine of 20 patients with idiopathic hypereosinophilic syndrome were evaluated on CT. The shape and distribution of intrahepatic low densities and the presence of hepatomegaly and/or splenomegaly were reviewed on CT, and the percentage of eosinophils in peripheral blood was also determined. In seven cases, interval change in hepatic lesion and the percentage of eosinophils were reviewed on follow-up examination. On initial CT, varying low-density patterns were seen in the liver in all cases; hepatomegaly was seen in four cases, and hepatosplenomegaly in two. The percentage of eosinophils was 89% in a case with diffuse patch low densities in the liver, 65-85% in three cases with numerous nodular low density lesions, 12-29% in four cases with multiple (below ten) nodular or small geographic hypodense lesions, and 24% in a case with a single nodular hypodense lesion. On follow-up CT, seven patients showed a decrease in the percentage of eosinophils, and in six, improved intrahepatic low densities were seen. On CT, intrahepatic low densities were seen in patients with idiopathic hypereosinophilic syndrome, and these were distributed more extensively when peripheral eosinophilia was more severe. With improvement in peripheral eosinophilia, the low densities also improved

Idiopathic global unclassified ectrodactyly – A case report | Udigwe ...

African Journals Online (AJOL)

Congenital digital limb anomalies are not very common and are usually associated with congenital syndromes. They present enormous challenges to the managing team due to the intricacies involved while the parents go through a lot of anxiety. The case presented here is an isolated idiopathic form of digital anomalies ...

Idiopathic central precocious puberty in a Nigerian boy | Ojukwu ...

African Journals Online (AJOL)

A case of idiopathic central precocious puberty, a rare condition is reported in a 21/2 year-old Nigerian boy. He presented with progressive genital growth, a growth spurt, advanced skeletal maturation, and inappropriately high serum concentrations of pituitary and gonadal sex homones for his age. There was no family ...

Consanguineous marriage and increased risk of idiopathic congenital talipes equinovarus: a case-control study in a rural area.

Science.gov (United States)

Sahin, Orcun; Yildirim, Cengiz; Akgun, Rahmi C; Haberal, Bahtiyar; Yazici, Ayse C; Tuncay, Ismail C

2013-01-01

The purpose of this study is to evaluate if there is any relationship between consanguineous marriages and idiopathic congenital talipes equinovarus (CTEV). A case-control study on CTEV screening was conducted in a rural eastern city of Turkey between 2009 and 2011 and a total of 28 cases (infants with idiopathic CTEV) and 575 controls (healthy infants) were recruited. Sociodemographic status of the infants, including gestational age and birth weights, maternal characteristics and, if any, the degree of consanguinity, were recorded. As an inclusion criterion, only singleton, full-term, live births were accepted. A backward stepwise logistic regression model was used to evaluate the relationship between idiopathic CTEV and parental consanguinity. Unadjusted and adjusted odds ratios (OR) with 95% confidence interval (CI) were calculated. Among maternal and infant characteristics, significant risk factors for idiopathic CTEV in the regression analysis were work status (employed), consanguineous marriage, sex (male), and gestational age (>42 wk). Babies born to first-cousin parents had >4 times the risk of idiopathic CTEV [OR, 4.138, (95% CI, 1.484, 11.538)] and the risk for those born to distant relatives was 2.9 times higher [OR, 2.941, (95% CI, 1.070, 8.087)] than for children of unrelated parents. Consanguineous marriage was significantly associated with an increased risk of idiopathic CTEV. This association remained significant even after adjusting for potential confounding variables. To obtain more accurate results, a population-based screening study with an increased number of cases and controls should be performed in future studies. Case-control study investigating the effect of a patient characteristic on the outcome of disease (level-III).

Manual Therapy in the Treatment of Idiopathic Scoliosis. Analysis of Current Knowledge.

Science.gov (United States)

Czaprowski, Dariusz

2016-10-28

Apart from the recommended specific physiotherapy, the treatment of idiopathic scoliosis (IS) also incorporates non-specific manual therapy (NMT). The aim of this paper is to assess the efficacy of NMT (manual therapy, chiropractic, osteopathy) used in the treatment of children and adolescents with IS. The study analysed systematic reviews (Analysis 1) and other recent scientific publications (Analysis 2). Analysis 1 encompassed papers on the use of NMT in patients with IS. Works concerning specific physiotherapy (SP) or bracing (B) and other types of scoliosis were excluded from the analysis. Inclusion criteria for Analysis 2 were: treatment with NMT; subjects aged 10-18 years with IS. The following types of papers were excluded: works analysing NMT combined with SP or B, reports concerning adult pa tients, analyses of single cases and publications included in Analysis 1. Analysis 1: six systematic reviews contained 6 papers on the efficacy of NMT in the treatment of IS. The results of these studies are contradictory, ranging from Cobb angle reduction to no treatment effects whatsoever. The papers analysed are characterised by poor methodological quality: small group sizes, incomplete descriptions of the study groups, no follow-up and no control groups. Analysis 2: in total, 217 papers were found. None of them met the criteria set for the analysis. 1. Few papers verifying the efficacy of manual therapy, chiropractic and osteopathy in the treatment of idiopathic scoliosis have been published to date. 2. The majority are experimental studies with poor methodology or observational case studies. 3. At present, the efficacy of non-specific manual therapy in the treatment of patients with idiopathic scoliosis cannot be reliably evaluated. 4. It is necessary to conduct further research based on appropriate methods (prospective, rando mi s ed, controlled studies) in order to reliably assess the usefulness of non-specific manual therapy in the treatment of idiopathic

Ball tip method for thoracic pedicle screw placement in patients with adolescent idiopathic scoliosis

International Nuclear Information System (INIS)

Watanabe, Kota; Matsumoto, Morio; Iizuka, Shingo

2008-01-01

The purpose of this study was to evaluate the efficacy of ball tip method for thoracic pedicle screw placements in idiopathic scoliosis patients. 24 patients with adolescent idiopathic scoliosis were included in this study. Conventional method was performed in 12 patients. Ball tip method was performed in 12 patients. Accuracy of the pedicle screw placement was evaluated based on the postoperative CT. In the ball tip method, a probe which was consisted of ball tip with flexible shaft was used. After removing of cortical bone at a starting point, the probe was inserted manually or sometimes with gently tapping by hammer. During the maneuver, the probe will gradually progress into cancellous bone in the pedicle, without perforating cortical bone in the pedicle. Following expansion of the hole by a rigid gear shift probe, screw was placed in the pedicle. 65.1% of screws were located within pedicle in the conventional group and 86.5% in the ball tip group. 5.3% of screws were located out of pedicle within 2 mm in the conventional group and 8.2% in the ball tip group. 15.8% of screws were located out of pedicle beyond 2 mm and 1.8% in the ball tip group. The ball tip method enhanced the accuracy of thoracic pedicle screw placements in adolescent idiopathic scoliosis patients. The ball tip method may be effective for accurate pedicle screw placement in patients with adolescent idiopathic scoliosis. (author)

Idiopathic thrombocytopenic purpura during pregnancy

Directory of Open Access Journals (Sweden)

Tânia Regina Padovani

2012-04-01

Full Text Available ABSTRACT This essay is based on a medical case of idiopathic thrombocytopenic purpura (ITP during pregnancy. The cause of ITP is unknown, who suffer from this disorder, generate antibodies that destroy thrombocytes from their blood. ITP affects women of childbearing age and is associated to maternal and fetal complications. The management of a pregnant patient is difficult and requires the combined care of an obstetrician, a hematologist, and a neonatologist. The main therapeutic options for ITP in pregnant women include glucocorticoids and intravenous immunoglobulin. Splenectomy may be (performed in refractory cases. There is no concerning the management and treatment of pregnant women.

Surgical repair of idiopathic scrotal elephantiasis.

Science.gov (United States)

Zacharakis, Evangelos; Dudderidge, Tim; Zacharakis, Emmanouil; Ioannidis, Evangelos

2008-02-01

Scrotal lymphedema (scrotal elephantiasis) is uncommon outside of filariasis endemic regions. We present a case of a 65-year-old with idiopathic lymphedema of the scrotum and functional impairment of the penis. The patient underwent surgical excision of the edematous subcutaneous tissues and plastic reconstruction of his penis and scrotum. Three years later, the patient showed no signs of local recurrence, had complete restoration of urinary and sexual function and was extremely satisfied with the result. Surgical management was an effective strategy in the management of scrotal lymphedema in this case.

Potential novel targets: Protease-activated receptors in idiopathic pulmonary fibrosis

NARCIS (Netherlands)

Lin, C.

2015-01-01

Idiopathic pulmonary fibrosis (IPF) is the most devastating diffuse fibrosing lung disease of unknown etiology. IPF patients suffer from severe breathlessness caused by decreasing lung compliance eventually leading to respiratory failure and death. The prognosis of IPF is devastating: there is only

Lysyl oxidases regulate fibrillar collagen remodelling in idiopathic pulmonary fibrosis

NARCIS (Netherlands)

Tjin, Gavin; White, Eric S; Faiz, Alen; Sicard, Delphine; Tschumperlin, Daniel J; Mahar, Annabelle; Kable, Eleanor P W; Burgess, Janette K

2017-01-01

Idiopathic pulmonary fibrosis (IPF) is a progressive scarring disease of the lung with feweffective therapeutic options. Structural remodelling of the extracellular matrix [i.e. collagen cross-linkingmediated by the lysyl oxidase (LO) family of enzymes (LOX, LOXL1-4)] might contribute to disease

The adolescent experience in Juvenile Idiopathic Arthritis: A narrative approach

NARCIS (Netherlands)

Fuchs, C.E.

2013-01-01

This dissertation focused on the self-experience of adolescents with juvenile idiopathic arthritis (JIA) or chronic fatigue syndrome (CFS). Although the etiology and nosology of JIA and CFS are fundamentally different, some commonalities in the emotional experience of adolescents dealing with these

Idiopathic omental hemorrhage: A case report and review of the literature

Directory of Open Access Journals (Sweden)

Jiro Kimura

2016-01-01

Conclusion: Idiopathic omental hemorrhage is a rare cause of an acute abdomen, which may develop after eating. Omentectomy is preferred to ligation or transcatheter arterial embolization to rule out an underlying malignancy or aneurysm.