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Sample records for main thoracic idiopathic

  1. Assessment of spontaneous correction of lumbar curve after fusion of the main thoracic in Lenke 1 adolescent idiopathic scoliosis☆

    PubMed Central

    Mizusaki, Danilo; Gotfryd, Alberto Ofenhejm

    2016-01-01

    Objective To evaluate the clinical and radiographic response of the lumbar curve after fusion of the main thoracic, in patients with adolescent idiopathic scoliosis of Lenke type 1. Methods Forty-two patients with Lenke 1 adolescent idiopathic scoliosis who underwent operations via the posterior route with pedicle screws were prospectively evaluated. Clinical measurements (size of the hump and translation of the trunk in the coronal plane, by means of a plumb line) and radiographic measurements (Cobb angle, distal level of arthrodesis, translation of the lumbar apical vertebral and Risser) were made. The evaluations were performed preoperatively, immediately postoperatively and two years after surgery. Results The mean Cobb angle of the main thoracic curve was found to have been corrected by 68.9% and the lumbar curve by 57.1%. Eighty percent of the patients presented improved coronal trunk balance two years after surgery. In four patients, worsening of the plumb line measurements was observed, but there was no need for surgical intervention. Less satisfactory results were observed in patients with lumbar modifier B. Conclusions In Lenke 1 patients, fusion of the thoracic curve alone provided spontaneous correction of the lumbar curve and led to trunk balance. Less satisfactory results were observed in curves with lumbar modifier B, and this may be related to overcorrection of the main thoracic curve. PMID:26962505

  2. Idiopathic Thoracic Spontaneous Spinal Epidural Hematoma.

    PubMed

    Aycan, Abdurrahman; Ozdemir, Seymen; Arslan, Harun; Gonullu, Edip; Bozkına, Cemal

    2016-01-01

    A 33-year-old male patient experienced temporary sensory loss and weakness in the right lower extremity one month prior to admission. The patient was admitted to a private clinic with a three-day history of acute onset of sensory loss and weakness in both lower extremities and was treated and followed up with a prediagnosis of transverse myelitis and the Guillain-Barre syndrome (GBS). The patient was subsequently transferred to our clinic and the neurologic examination revealed paraplegia in both lower extremities, positive bilateral Babinski signs, and hypesthesia below the T10 dermatome with saddle anesthesia. The patient had urinary incontinence and thoracic magnetic resonance imaging (MRI) showed an image of a mass compressing the medulla. PMID:27088028

  3. Idiopathic Thoracic Spontaneous Spinal Epidural Hematoma

    PubMed Central

    Aycan, Abdurrahman; Ozdemir, Seymen; Gonullu, Edip; Bozkına, Cemal

    2016-01-01

    A 33-year-old male patient experienced temporary sensory loss and weakness in the right lower extremity one month prior to admission. The patient was admitted to a private clinic with a three-day history of acute onset of sensory loss and weakness in both lower extremities and was treated and followed up with a prediagnosis of transverse myelitis and the Guillain-Barre syndrome (GBS). The patient was subsequently transferred to our clinic and the neurologic examination revealed paraplegia in both lower extremities, positive bilateral Babinski signs, and hypesthesia below the T10 dermatome with saddle anesthesia. The patient had urinary incontinence and thoracic magnetic resonance imaging (MRI) showed an image of a mass compressing the medulla. PMID:27088028

  4. Spontaneous resolution of idiopathic thoracic spinal cord herniation: case report.

    PubMed

    Samuel, Nardin; Goldstein, Christina L; Santaguida, Carlo; Fehlings, Michael G

    2015-09-01

    Spinal cord herniation is a relatively rare but increasingly recognized clinical entity, with fewer than 200 cases reported in the literature to date. The etiology of this condition remains unknown, and surgery is used as the primary treatment to correct the herniation and consequent spinal cord compromise. Some patients without clinical progression have been treated with nonoperative measures, including careful follow-up and symptomatic physical therapy. To date, however, there has been no published report on the resolution of spinal cord herniation without surgical intervention. The patient in the featured case is a 58-year-old man who presented with mild thoracic myelopathy and imaging findings consistent with idiopathic spinal cord herniation. Surprisingly, updated MRI studies, obtained to better delineate the pathology, showed spontaneous resolution of the herniation. Subsequent MRI 6 months later revealed continued resolution of the previous spinal cord herniation. This is the first report of spontaneous resolution of a spinal cord herniation in the literature. At present, the treatment of this disorder is individualized, with microsurgical correction used in patients with progressive neurological impairment. The featured case highlights the potential variability in the natural history of this condition and supports considering an initial trial of nonoperative management for patients with mild, nonprogressive neurological deficits. PMID:26023901

  5. Pulmonary function after less invasive anterior instrumentation and fusion for idiopathic thoracic scoliosis

    PubMed Central

    2013-01-01

    Purpose Standard thoracotomy for anterior instrumentation and fusion of the thoracic spine in idiopathic scoliosis may have detrimental effects on pulmonary function. In this study we describe a less invasive anterior surgical technique and show the pre- and postoperative pulmonary function with a minimum follow-up of 2 years. Methods Twenty patients with Lenke type 1 adolescent thoracic idiopathic scoliosis were treated with anterior spinal fusion and instrumentation. The mean preoperative Cobb angle of the thoracic curve was 53° ± 5.8. Pulmonary function tests (PFT) and radiographic evaluation was performed. Results The mean postoperative correction in Cobb angle of the thoracic curve was 27° ± 8.2 (49%). The mean preoperative FEV1 was 2.81 ± 0.43 L, which increased to 3.14 ± 0.50 L at 2 years postoperatively (P = 0.000). The mean FEV1% did not change (89.60 ± 7.49% preoperatively, versus 90.53 ± 5.95% at 2 years follow-up, P = 0.467). The TLC increased from 4.62 ± 0.62 L preoperatively to 5.17 ± 0.63 L at 2 years follow-up (P = 0.000). The FEV1% at two years of follow-up improved to 104% of the FEV1% predicted value. The FEV1 improved to 97% of the FEV1 predicted value. Conclusion Anterior spinal fusion for idiopathic scoliosis by means of a minimal open thoracotomy proved to be a safe surgical technique that resulted in an improvement of pulmonary function. Our results are similar to those of thoracoscopic procedures reported in literature. PMID:23965278

  6. Surgical management of thoracic idiopathic spinal cord herniation. Technical case report and review.

    PubMed

    Payer, Michael; Zumsteg, Dominik; De Tribolet, Nicolas; Wetzel, Stephan

    2016-08-01

    Idiopathic spinal cord herniation (ISCH) is a rare spinal disease, in which chronic cerebrospinal fluid pulsations push the arachnoid and adjacent thoracic spinal cord region through an antero-lateral dural defect of congenital, post-traumatic, or inflammatory/erosive origin. Symptomatic patients commonly present around the 5th decade of life with slowly progressive myelopathy. Diagnosis relies on high-resolution magnetic resonance imaging. Stable mild cases may be observed, whereas in progressive symptomatic situations, surgical spinal cord reposition and dural defect repair with a dural patch is the preferred treatment. We present a case of ISCH at T5/6 and a review the literature. PMID:27221089

  7. Effects of Multilevel Facetectomy and Screw Density on Postoperative Changes in Spinal Rod Contour in Thoracic Adolescent Idiopathic Scoliosis Surgery

    PubMed Central

    Kokabu, Terufumi; Sudo, Hideki; Abe, Yuichiro; Ito, Manabu; Ito, Yoichi M.; Iwasaki, Norimasa

    2016-01-01

    Flattening of the preimplantation rod contour in the sagittal plane influences thoracic kyphosis (TK) restoration in adolescent idiopathic scoliosis (AIS) surgery. The effects of multilevel facetectomy and screw density on postoperative changes in spinal rod contour have not been documented. This study aimed to evaluate the effects of multilevel facetectomy and screw density on changes in spinal rod contour from before implantation to after surgical correction of thoracic curves in patients with AIS prospectively. The concave and convex rod shapes from patients with thoracic AIS (n = 49) were traced prior to insertion. Postoperative sagittal rod shape was determined by computed tomography. The angle of intersection of the tangents to the rod end points was measured. Multiple stepwise linear regression analysis was used to identify variables independently predictive of change in rod contour (Δθ). Average Δθ at the concave and convex side were 13.6° ± 7.5° and 4.3° ± 4.8°, respectively. The Δθ at the concave side was significantly greater than that of the convex side (P < 0.0001) and significantly correlated with Risser sign (P = 0.032), the preoperative main thoracic Cobb angle (P = 0.031), the preoperative TK angle (P = 0.012), and the number of facetectomy levels (P = 0.007). Furthermore, a Δθ at the concave side ≥14° significantly correlated with the postoperative TK angle (P = 0.003), the number of facetectomy levels (P = 0.021), and screw density at the concave side (P = 0.008). Rod deformation at the concave side suggests that corrective forces acting on that side are greater than on the convex side. Multilevel facetectomy and/or screw density at the concave side have positive effects on reducing the rod deformation that can lead to a loss of TK angle postoperatively. PMID:27564683

  8. Effects of Multilevel Facetectomy and Screw Density on Postoperative Changes in Spinal Rod Contour in Thoracic Adolescent Idiopathic Scoliosis Surgery.

    PubMed

    Kokabu, Terufumi; Sudo, Hideki; Abe, Yuichiro; Ito, Manabu; Ito, Yoichi M; Iwasaki, Norimasa

    2016-01-01

    Flattening of the preimplantation rod contour in the sagittal plane influences thoracic kyphosis (TK) restoration in adolescent idiopathic scoliosis (AIS) surgery. The effects of multilevel facetectomy and screw density on postoperative changes in spinal rod contour have not been documented. This study aimed to evaluate the effects of multilevel facetectomy and screw density on changes in spinal rod contour from before implantation to after surgical correction of thoracic curves in patients with AIS prospectively. The concave and convex rod shapes from patients with thoracic AIS (n = 49) were traced prior to insertion. Postoperative sagittal rod shape was determined by computed tomography. The angle of intersection of the tangents to the rod end points was measured. Multiple stepwise linear regression analysis was used to identify variables independently predictive of change in rod contour (Δθ). Average Δθ at the concave and convex side were 13.6° ± 7.5° and 4.3° ± 4.8°, respectively. The Δθ at the concave side was significantly greater than that of the convex side (P < 0.0001) and significantly correlated with Risser sign (P = 0.032), the preoperative main thoracic Cobb angle (P = 0.031), the preoperative TK angle (P = 0.012), and the number of facetectomy levels (P = 0.007). Furthermore, a Δθ at the concave side ≥14° significantly correlated with the postoperative TK angle (P = 0.003), the number of facetectomy levels (P = 0.021), and screw density at the concave side (P = 0.008). Rod deformation at the concave side suggests that corrective forces acting on that side are greater than on the convex side. Multilevel facetectomy and/or screw density at the concave side have positive effects on reducing the rod deformation that can lead to a loss of TK angle postoperatively. PMID:27564683

  9. Treatment of idiopathic chylothorax in 14 dogs by ligation of the thoracic duct and partial pericardiectomy.

    PubMed

    Carobbi, B; White, R A S; Romanelli, G

    The outcome and complications associated with thoracic duct ligation combined with partial pericardiectomy in 14 dogs with idiopathic chylothorax were investigated retrospectively. Nine of the dogs were treated in the uk and five in Italy. All of them were reassessed clinically four weeks after surgery and the respiratory function and any pleural fluid accumulation were evaluated; they were followed up by telephone contact for at least six months. Eleven of the dogs were clinically normal and had no radiographic signs of pleural effusion when reassessed after four weeks. Two showed radiographic signs of a minor accumulation of pleural fluid but were clinically normal; when reassessed after three months they showed similar radiographic signs and clinical findings; but after four months there was no evidence of pleural effusion. One dog had a major complication that required a second surgical intervention. PMID:19103616

  10. Idiopathic pulmonary haemosiderosis: spectrum of thoracic imaging findings in the adult patient.

    PubMed

    Khorashadi, L; Wu, C C; Betancourt, S L; Carter, B W

    2015-05-01

    Idiopathic pulmonary haemosiderosis (IPH) is a rare disease characterized by alveolar capillary haemorrhage resulting in deposition and accumulation of haemosiderin in the lungs. Although its precise pathophysiology remains unclear, several hypotheses have been proposed to explain the aetiology of the disorder, including autoimmune, environmental, allergic, and genetic theories. IPH is typically diagnosed in childhood, usually before the age of 10 years; however, this entity may be encountered in older patients given the greater awareness of the diagnosis, availability and utilization of advanced imaging techniques, and improved treatment and survival. The classic presentation of IPH consists of the triad of haemoptysis, iron-deficiency anaemia, and pulmonary opacities on chest radiography. The diagnosis is usually confirmed via bronchoscopy with bronchoalveolar lavage (BAL), at which time haemosiderin-laden macrophages referred to as siderophages, considered pathognomonic for IPH, may be identified. However, lung biopsy may ultimately be necessary to exclude other disease processes. For children with IPH, the disease course is severe and the prognosis is poor. However, adults generally have a longer disease course with milder symptoms and the prognosis is more favourable. Specific imaging features, although non-specific in isolation, may be identified on thoracic imaging studies, principally chest radiography and CT, depending on the phase of disease (acute or chronic). Recognition of these findings is important to guide appropriate clinical management. PMID:25515792

  11. Transient long thoracic nerve injury during posterior spinal fusion for adolescent idiopathic scoliosis: A report of two cases.

    PubMed

    Tsirikos, Athanasios I; Al-Hourani, Khalid

    2013-11-01

    We present the transient long thoracic nerve (LTN) injury during instrumented posterior spinal arthrodesis for idiopathic scoliosis. The suspected mechanism of injury, postoperative course and final outcome is discussed. The LTN is susceptible to injury due to its long and relatively superficial course across the thoracic wall through direct trauma or tension. Radical mastectomies with resection of axillary lymph nodes, first rib resection to treat thoracic outlet syndrome and cardiac surgery can be complicated with LTN injury. LTN injury producing scapular winging has not been reported in association with spinal deformity surgery. We reviewed the medical notes and spinal radiographs of two adolescent patients with idiopathic scoliosis who underwent posterior spinal arthrodesis and developed LTN neuropraxia. Scoliosis surgery was uneventful and intraoperative spinal cord monitoring was stable throughout the procedure. Postoperative neurological examination was otherwise normal, but both patients developed winging of the scapula at 4 and 6 days after spinal arthrodesis, which did not affect shoulder function. Both patients made a good recovery and the scapular winging resolved spontaneously 8 and 11 months following surgery with no residual morbidity. We believe that this LTN was due to positioning of our patients with their head flexed, tilted and rotated toward the contralateral side while the arm was abducted and extended. The use of heavy retractors may have also applied compression or tension to the nerve in one of our patients contributing to the development of neuropraxia. This is an important consideration during spinal deformity surgery to prevent potentially permanent injury to the nerve, which can produce severe shoulder dysfunction and persistent pain. PMID:24379470

  12. Idiopathic tension mediastinal emphysema cured by video-assisted thoracic surgery: a case report

    PubMed Central

    Li, Xiao-Ping; Zhang, Liang; Li, Lei; Yang, Bo; Zhang, Wei-Dong

    2015-01-01

    Mediastinal emphysema is a status of gas retention in mediastinum, usually caused by airway system injury or esophagus rupture. Healthy people without trauma and basic disease emerging unexplained mediastinal emphysema are called idiopathic mediastinal emphysema. While tension pneumomediastinum is an exceptional and potentially lethal condition, the increased intramediastinal pressure with a severe oppression of heart, vena cava and pulmonary vein leads to impaired central venous return through the cavae system, restricted right heart diastolic filling, and collapse of the cardiac chambers, resulting in reduced stroke volume, cardiac output and dysaemia. This young people-prone disease was initially reported by Hamman in 1939 and usually has a good prognosis so that no special treatments were needed. In this study, however, we presented a young boy with idiopathic tension mediastinal emphysema combined with dysaemia and respiratory failure. Fortunately, he was finally successful treated by VATS after heteropathy and incision on suprasternal fossae. PMID:26770618

  13. Surgical approaches for the management of idiopathic thoracic scoliosis and the indications for combined anterior-posterior technique.

    PubMed

    Rauzzino, M J; Shaffrey, C I; Wagner, J; Nockels, R; Abel, M

    1999-05-15

    The indications for surgical intervention in patients with idiopathic scoliosis have been well defined. The goals of surgery are to achieve fusion and arrest progressive curvature while restoring normal coronal and sagittal balance. As first introduced by Harrington, posterior fusion, the gold standard of treatment, has a proven record of success. More recently, anterior techniques for performing fusion procedures via either a thoracotomy or a retroperitoneal approach have been popularized in attempts to achieve better correction of curvature, preserve motion segments, and avoid some of the complications of posterior fusion such as the development of the flat-back syndrome. Anterior instrumentation alone, although effective, can be kyphogenic and has been shown to be associated with complications such as pseudarthrosis and instrumentation failure. Performing a combined approach in patients with scoliosis and other deformities has become an increasingly popular procedure to achieve superior correction of deformity and to minimize later complications. Indications for a combined approach (usually consisting of anterior release, arthrodesis with or without use of instrumentation, and posterior segmental fusion) include: prevention of crankshaft phenomenon in juvenile or skeletally immature adolescents; correction of large curves (75 degrees ) or excessively rigid curves in skeletally mature or immature patients; correction of curves with large sagittal-plane deformities such as thoracic kyphosis (> 90 degrees ) or thoracic lordosis (> 20 degrees ); and correction of thoracolumbar curves that need to be fused to the sacrum. Surgery may be performed either in a staged proceedure or, more commonly, in a single sitting. The authors discuss techniques for combined surgery and complication avoidance. PMID:17031912

  14. An Official American Thoracic Society Research Statement: Noninfectious Lung Injury after Hematopoietic Stem Cell Transplantation: Idiopathic Pneumonia Syndrome

    PubMed Central

    Panoskaltsis-Mortari, Angela; Griese, Matthias; Madtes, David K.; Belperio, John A.; Haddad, Imad Y.; Folz, Rodney J.; Cooke, Kenneth R.

    2011-01-01

    Rationale: Acute lung dysfunction of noninfectious etiology, known as idiopathic pneumonia syndrome (IPS), is a severe complication following hematopoietic stem cell transplantation (HSCT). Several mouse models have been recently developed to determine the underlying causes of IPS. A cohesive interpretation of experimental data and their relationship to the findings of clinical research studies in humans is needed to better understand the basis for current and future clinical trials for the prevention/treatment of IPS. Objectives: Our goal was to perform a comprehensive review of the preclinical (i.e., murine models) and clinical research on IPS. Methods: An ATS committee performed PubMed and OVID searches for published, peer-reviewed articles using the keywords “idiopathic pneumonia syndrome” or “lung injury” or “pulmonary complications” AND “bone marrow transplant” or “hematopoietic stem cell transplant.” No specific inclusion or exclusion criteria were determined a priori for this review. Measurements and Main Results: Experimental models that reproduce the various patterns of lung injury observed after HSCT have identified that both soluble and cellular inflammatory mediators contribute to the inflammation engendered during the development of IPS. To date, 10 preclinical murine models of the IPS spectrum have been established using various donor and host strain combinations used to study graft-versus-host disease (GVHD). This, as well as the demonstrated T cell dependency of IPS development in these models, supports the concept that the lung is a target of immune-mediated attack after HSCT. The most developed therapeutic strategy for IPS involves blocking TNF signaling with etanercept, which is currently being evaluated in clinical trials. Conclusions: IPS remains a frequently fatal complication that limits the broader use of allogeneic HSCT as a successful treatment modality. Faced with the clinical syndrome of IPS, one can categorize the

  15. Thoracic and lumbar vertebral bone mineral density changes in a natural occurring dog model of diffuse idiopathic skeletal hyperostosis.

    PubMed

    De Decker, Steven; Lam, Richard; Packer, Rowena M A; Gielen, Ingrid M V L; Volk, Holger A

    2015-01-01

    Ankylosing spinal disorders can be associated with alterations in vertebral bone mineral density (BMD). There is however controversy about vertebral BMD in patients wuse idiopathic skeletal hyperostosis (DISH). DISH in Boxer dogs has been considered a natural occurring disease model for DISH in people. The purpose of this study was to compare vertebral BMD between Boxers with and without DISH. Fifty-nine Boxers with (n=30) or without (n=29) DISH that underwent computed tomography were included. Vertebral BMD was calculated for each thoracic and lumbar vertebra by using an earlier reported and validated protocol. For each vertebral body, a region of interest was drawn on the axial computed tomographic images at three separate locations: immediately inferior to the superior end plate, in the middle of the vertebral body, and superior to the inferior end plate. Values from the three axial slices were averaged to give a mean Hounsfield Unit value for each vertebral body. Univariate statistical analysis was performed to identify factors to be included in a multivariate model. The multivariate model including all dogs demonstrated that vertebral DISH status (Coefficient 24.63; 95% CI 16.07 to 33.19; p <0.001), lumbar vertebrae (Coefficient -17.25; 95% CI -23.42 to -11.09; p < 0.01), and to a lesser extent higher age (Coefficient -0.56; 95% CI -1.07 to -0.05; p = 0.03) were significant predictors for vertebral BMD. When the multivariate model was repeated using only dogs with DISH, vertebral DISH status (Coefficient 20.67; 95% CI, 10.98 to 30.37; p < 0.001) and lumbar anatomical region (Coefficient -38.24; 95% CI, -47.75 to -28.73; p < 0.001) were again predictors for vertebral BMD but age was not. The results of this study indicate that DISH can be associated with decreased vertebral BMD. Further studies are necessary to evaluate the clinical importance and pathophysiology of this finding. PMID:25898128

  16. Thoracic and Lumbar Vertebral Bone Mineral Density Changes in a Natural Occurring Dog Model of Diffuse Idiopathic Skeletal Hyperostosis

    PubMed Central

    De Decker, Steven; Lam, Richard; Packer, Rowena M. A.; Gielen, Ingrid M. V. L.; Volk, Holger A.

    2015-01-01

    Ankylosing spinal disorders can be associated with alterations in vertebral bone mineral density (BMD). There is however controversy about vertebral BMD in patients wuse idiopathic skeletal hyperostosis (DISH). DISH in Boxer dogs has been considered a natural occurring disease model for DISH in people. The purpose of this study was to compare vertebral BMD between Boxers with and without DISH. Fifty-nine Boxers with (n=30) or without (n=29) DISH that underwent computed tomography were included. Vertebral BMD was calculated for each thoracic and lumbar vertebra by using an earlier reported and validated protocol. For each vertebral body, a region of interest was drawn on the axial computed tomographic images at three separate locations: immediately inferior to the superior end plate, in the middle of the vertebral body, and superior to the inferior end plate. Values from the three axial slices were averaged to give a mean Hounsfield Unit value for each vertebral body. Univariate statistical analysis was performed to identify factors to be included in a multivariate model. The multivariate model including all dogs demonstrated that vertebral DISH status (Coefficient 24.63; 95% CI 16.07 to 33.19; p <0.001), lumbar vertebrae (Coefficient -17.25; 95% CI -23.42 to -11.09; p < 0.01), and to a lesser extent higher age (Coefficient -0.56; 95% CI -1.07 to -0.05; p = 0.03) were significant predictors for vertebral BMD. When the multivariate model was repeated using only dogs with DISH, vertebral DISH status (Coefficient 20.67; 95% CI, 10.98 to 30.37; p < 0.001) and lumbar anatomical region (Coefficient -38.24; 95% CI, -47.75 to -28.73; p < 0.001) were again predictors for vertebral BMD but age was not. The results of this study indicate that DISH can be associated with decreased vertebral BMD. Further studies are necessary to evaluate the clinical importance and pathophysiology of this finding. PMID:25898128

  17. Accuracy of free-hand placement of thoracic pedicle screws in adolescent idiopathic scoliosis: how much of a difference does surgeon experience make?

    PubMed

    Samdani, Amer F; Ranade, Ashish; Sciubba, Daniel M; Cahill, Patrick J; Antonacci, M Darryl; Clements, David H; Betz, Randal R

    2010-01-01

    The use of thoracic pedicle screws for the treatment of adolescent idiopathic scoliosis (AIS) has gained widespread popularity. However, the placement of pedicle screws in the deformed spine poses unique challenges, and surgeons experience a learning curve. The in vivo accuracy as determined by computed tomography (CT) of placement of thoracic pedicle screws in the deformed spine as a function of surgeon experience is unknown. We undertook a retrospective review to determine the effect of surgeon experience on the accuracy of thoracic pedicle screw placement in AIS. In 2005, we started to obtain routine postoperative CT scans on patients undergoing a spinal fusion. From a database of these patients, we selected AIS patients, who underwent a posterior spinal fusion. Fifteen consecutive patients for each of the following three groups stratified by attending surgeon experience were selected (N = 45): A) less than 20 cases of all pedicle screw constructs for AIS (surgeons <2 years of practice), B) 20-50 cases (surgeons 2-5 years of practice), and C) greater than 50 cases (surgeons greater than 5 years of practice). Intraoperative evaluation of all screws included probing of the pedicle screw tract, neurophysiologic monitoring, and fluoroscopic confirmation. A total of 856 thoracic pedicle screws were studied. Postoperative CT scans were evaluated by two spine surgeons and a consensus read established as follows: (1) In: intraosseous placement or 2-mm breach, either medial or lateral. Of the 856 screws, 104 demonstrated a >2-mm breach, for an overall rate of 12.1% (medial = 55, lateral = 49, P = 0.67). When the breach rates were stratified by surgeon experience, there was a trend toward decreased rate of breach for the most experienced surgeons, although this did not attain statistical significance (Group A: 12.7%, Group B: 12.9%, Group C: 10.8%, P = 0.58). However, the most experienced group (C) had a markedly decreased rate of medial

  18. Changes of concave and convex rib-vertebral angle, angle difference and angle ratio in patients with right thoracic adolescent idiopathic scoliosis.

    PubMed

    Canavese, Federico; Turcot, Katia; Holveck, Jerôme; Farhoumand, Agnés Dahl; Kaelin, André

    2011-01-01

    The aim of this study is to describe the radiological changes in rib-vertebral angles (RVAs), rib-vertebral angle differences (RVADs), and rib-vertebral angle ratios (RVARas) in patients with untreated right thoracic adolescent idiopathic scoliosis and to compare with the normal subjects. The concave and convex RVA from T1 to T12, the RVADs and the RVARas were measured on AP digital radiographs of 44 female patients with right convex idiopathic scoliosis and 14 normal females. Patients were divided into three groups: normal subjects (group 1), scoliotic patients with Cobb's angle equal or <30° (group 2) and scoliotic patients with Cobb's angle over 30° (group 3). Overall values (mean ± SD) of the RVAs on the concave side were 90.5° ± 17° in group 1, 90.3° ± 15.8° in group 2 and 88.8° ± 15.4° in group 3. On the convex side, values were 90.0° ± 17.3° in group 1, 86.3° ± 13.7° in group 2 and 80.7° ± 14.4° in group 3. Overall values (mean ± SD) of the RVADs at all levels were 0.5° ± 0.7° in group 1, 4.0° ± 4.8° in group 2 and 8.0° ± 4.0° in group 3. The RVARa values (mean ± SD) at all levels was 1.008° ± 0.012° in group 1, 1.041° ± 0.061° in group 2 and 1.102° ± 0.151° in group 3. RVAD and RVARa values in the scoliotic segment were greater in patients with untreated scoliosis over 30° than in patients with an untreated deformity of <30° or normal subjects. A significant effect between groups was observed for the RVA, RVAD and RVARa variables. Measurement of RVA, RVAD and RVARa should not only be performed at and around the apex of a thoracic spinal deformity, but also extended to the whole thoracic spine. PMID:20811755

  19. The Superiority of Intraoperative O-arm Navigation-assisted Surgery in Instrumenting Extremely Small Thoracic Pedicles of Adolescent Idiopathic Scoliosis

    PubMed Central

    Liu, Zhen; Jin, Mengran; Qiu, Yong; Yan, Huang; Han, Xiao; Zhu, Zezhang

    2016-01-01

    Abstract To investigate the accuracy of O-arm navigation-assisted screw insertion in extremely small thoracic pedicles and to compare it with free-hand pedicle screw insertion in adolescent idiopathic scoliosis (AIS). A total of 344 pedicle screws were inserted in apical region (defined as 2 vertebrae above and below the apex each) of 46 AIS patients (age range 13–18 years) with O-arm navigation and 712 screws were inserted in 92 AIS patients (age range 11–17 years) with free-hand technique. According to the narrowest diameter orthogonal to the long axis of the pedicle on a trajectory entering the vertebral body on preoperative computed tomography, the pedicles were classified into large (>3 mm) and small (≤3 mm) subgroups. Furthermore, a subset of extremely small pedicles (≤2 mm in the narrowest diameter) was specifically discussed. Screw accuracy was categorized as grade 0: no perforation, grade 1: perforation by less than 2 mm, grade 2: perforation by 2 to 4 mm, grade 3: perforation over 4 mm. In the O-arm group, the mean thoracic pedicle diameters were 2.23 mm (range 0.7–2.9 mm) and 3.48 mm (3.1–7.1 mm) for small and large pedicles, respectively. In the free-hand group, the small and large thoracic pedicle diameters were 2.42 mm (range 0.6–2.9 mm) and 3.75 mm (3.1–6.9 mm), respectively. The overall accuracies of screw insertion in large and small thoracic pedicles (grade 0, 1) were significantly higher in O-arm group (large: 93.8%, 210/224, small: 91.7%, 110/120) than those of free-hand group (large: 84.9%, 353/416, small: 78.4%, 232/296) (P < 0.05). Importantly, the overall accuracy of screw placement in extremely small pedicles was significantly higher in the O-arm group (84.3%, 48/57) compared with 62.7% (79/126) in free-hand group (P < 0.05), and the incidence of medial perforation was significantly lower in O-arm group (11.1%, 1/9) compared with 17.0% (8/47) in free-hand group (P < 0.05). The O

  20. Idiopathic hypersomnia

    MedlinePlus

    Hypersomnia - idiopathic; Drowsiness - idiopathic; Somnolence - idiopathic ... extremely sleepy. It is different from narcolepsy because idiopathic hypersomnia does not usually involve suddenly falling asleep (sleep ...

  1. Idiopathic scoliosis.

    PubMed

    Yaman, Onur; Dalbayrak, Sedat

    2014-01-01

    Scoliosis refers to curves exceeding 10 degrees observed through posterioanterior direct radiography. In fact, the diagnosis for idiopathic scoliosis is accepted to exclude already available causes. The aim of this paper was to review the etiopathogenesis, classification systems and the treatment management of idiopathic scoliosis. A search in the National Library of Medicine (Pubmed) database using the key words 'idiopathic' and 'scoliosis' was performed. For the literature review, papers concerning the etiopathogenesis, classification and treatment were selected among these articles. A search in the National Library of Medicine (Pubmed) database using the key words 'idiopathic' and 'scoliosis' yielded 4518 articles published between 1947 and 2013. The main hypothesis put forward included genetic factors, hormonal factors, bone and connective tissue anomalies. King, Lenke, Coonrad and Peking Union Medical College (PUMC) classifications were the main classification systems for idiopathic scoliosis. Exercise, bracing and anterior, posterior or combined surgery when indicated are the choices for the treatment. Every idiopathic scoliosis case has to be managed to its own characteristics. It is the post-operative appearance that the surgeons are perhaps the least interested but the adolescent patients the most interested in. The aim of scoliosis surgery is to restore the spine without neurological deficit. PMID:25269032

  2. Ossification of thoracic ligamenta flava

    SciTech Connect

    Kudo, S.; Minoru, O.; Russell, W.J.

    1983-07-01

    Although ligamentum flavum ossification (LFO) often occurs in normal persons, there are no reports of its detection on lateral chest radiographs made during screening examinations. Review of 1,744 consecutive lateral chest radiographs identified LFO in 6.2% of males and 4.8% of females. LFO occurred mainly at the intervertebral segments from T9-T10 through T12-L1. Most prevalent was the hook-shaped LFO, protruding inferoirly from the inferior facets into the projections of the intervertabral foramina. Though LFO can cause severe neurologic symptoms, none of the affected persons in this study reported such symptoms. LFO was first visualized radiographically when the subjects were 20-40 years old, and it may be a physiologic condition. The LFO in these cases existed independent of thoracic posterior longitudinal ligament ossification, diffuse idiopathic skeletal hyperostosis, and degenerative osteoarthritis.

  3. Could CCI or FBCI Fully Eliminate the Impact of Curve Flexibility When Evaluating the Surgery Outcome for Thoracic Curve Idiopathic Scoliosis Patient? A Retrospective Study

    PubMed Central

    Ni, Haijian; Zhu, Xiaodong; Li, Ming

    2015-01-01

    Purpose To clarify if CCI or FBCI could fully eliminate the influence of curve flexibility on the coronal correction rate. Methods We reviewed medical record of all thoracic curve AIS cases undergoing posterior spinal fusion with all pedicle screw systems from June 2011 to July 2013. Radiographical data was collected and calculated. Student t test, Pearson correlation analysis and linear regression analysis were used to analyze the data. Results 60 were included in this study. The mean age was 14.7y (10-18y) with 10 males (17%) and 50 females (83%). The average Risser sign was 2.7. The mean thoracic Cobb angle before operation was 51.9°. The mean bending Cobb angle was 27.6° and the mean fulcrum bending Cobb angle was 17.4°. The mean Cobb angle at 2 week after surgery was 16.3°. The Pearson correlation coefficient r between CCI and BFR was -0.856(P<0.001), and between FBCI and FFR was -0.728 (P<0.001). A modified FBCI (M-FBCI) = (CR-0.513)/BFR or a modified CCI (M-CCI) = (CR-0.279)/FFR was generated by curve estimation has no significant correlation with FFR (r=-0.08, p=0.950) or with BFR (r=0.123, p=0.349). Conclusions Fulcrum-bending radiographs may better predict the outcome of AIS coronal correction than bending radiographs in thoracic curveAIS patients. Neither CCI nor FBCI can fully eliminate the impact of curve flexibility on the outcome of correction. A modified CCI or FBCI can better evaluating the corrective effects of different surgical techniques or instruments. PMID:25984945

  4. BENDING RADIOGRAPHS AS A PREDICTIVE FACTOR IN SURGICAL CORRECTION OF ADOLESCENT IDIOPATHIC SCOLIOSIS

    PubMed Central

    Gotfryd, Alberto Ofenhejm; Franzin, Fernando José; Poletto, Patrícia Rios; de Laura, Alexandre Spertini; da Silva, Luis Carlos Ferreira

    2015-01-01

    Objective: To evaluate the use of x-rays in dorsal decubitus, as a predictive factor for surgical correction of the main thoracic curve using pedicle screws, on patients with idiopathic adolescent scoliosis. Method: Twenty patients with idiopathic adolescent scoliosis of Lenke types 1A and 1B who were operated using a technique only involving pedicle screws by means of the posterior route were evaluated clinically and radiographically. The curve flexibility was calculated by means of active supine lateral oblique radiographs. The postoperative values for the main thoracic curve were included in a mathematical equation proposed by Cheung et al., with the aim of predicting the expected angular result from the surgical correction. The difference between the expected and actual postoperative results was then investigated regarding its statistical significance. Results: There was statistical significance for all the cases studied, between the values predicted before the operation and the radiographic findings immediately after the operation (p < 0.005). Conclusions: It is possible to predict the percentage surgical correction of the main thoracic curve that will be achieved using pedicle screws in patients with idiopathic adolescent scoliosis of Lenke types 1A and 1B, by means of preoperative supine oblique radiographs. PMID:27027056

  5. Thoracic emergencies.

    PubMed

    Worrell, Stephanie G; Demeester, Steven R

    2014-02-01

    This article discusses thoracic emergencies, including the anatomy, pathophysiology, clinical presentation, examination, diagnosis, technique, management, and treatment of acute upper airway obstruction, massive hemoptysis, spontaneous pneumothorax, and pulmonary empyema. PMID:24267505

  6. Idiopathic anaphylaxis.

    PubMed

    Greenberger, Paul A

    2007-05-01

    Idiopathic anaphylaxis is a prednisone-responsive condition without external cause, but it can coexist with food-, medication-, or exercise-induced anaphylaxis. Mast cell activation may occur at night or after foods that have been eaten with impunity many times previously. Idiopathic anaphylaxis can be classified into frequent (if there are six or more episodes per year or two episodes in the last 2 months) or infrequent (if episodes occur less often). Idiopathic anaphylaxis-generalized consists of urticaria or angioedema associated with severe respiratory distress, syncope or hypotension, and gastrointestinal symptoms. Idiopathic anaphylaxis-angioedema consists of massive tongue enlargement or severe pharyngeal or laryngeal swelling with urticaria or peripheral angioedema. The differential diagnosis of idiopathic anaphylaxis is reviewed, and treatment approaches are presented. PMID:17493503

  7. Study on Treatment with Respect to Idiopathic Scoliosis

    NASA Astrophysics Data System (ADS)

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

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

  8. Idiopathic hypersomnia

    MedlinePlus

    ... page, please enable JavaScript. Idiopathic hypersomnia is a sleep disorder in which a person is excessively sleepy ( hypersomnia ) ... other potential causes of excessive daytime sleepiness. Other sleep disorders that may cause daytime sleepiness include: Narcolepsy Obstructive ...

  9. Adolescent Idiopathic Scoliosis

    PubMed Central

    Choudhry, Muhammad Naghman; Ahmad, Zafar; Verma, Rajat

    2016-01-01

    Background: Scoliosis refers to deviation of spine greater than 10 degrees in the coronal plane. Idiopathic Scoliosis is the most common spinal deformity that develops in otherwise healthy children. The sub types of scoliosis are based on the age of the child at presentation. Adolescent idiopathic scoliosis (AIS) by definition occurs in children over the age of 10 years until skeletal maturity. Objective: The objective of this review is to outline the features of AIS to allow the physician to recognise this condition and commence early treatment, thereby optimizing patient outcome. Method: A thorough literature search was performed using available databases, including Pubmed and Embase, to cover important research published covering AIS. Conclusion: AIS results in higher incidence of back pain and discontent with body image. Curves greater than 50 degrees in thoracic region and greater than 30 degrees in lumbar region progress at a rate of 0.5 to 1 degree per year into adulthood. Curves greater than 60 degrees can lead to pulmonary functional deficit. Therefore once the disease is recognized, effective treatment should be instituted to address the deformity and prevention of its long-term sequelae. PMID:27347243

  10. Thoracic aortic aneurysm

    MedlinePlus

    Aortic aneurysm - thoracic; Syphilitic aneurysm; Aneurysm - thoracic aortic ... The most common cause of a thoracic aortic aneurysm is hardening of the ... with high cholesterol, long-term high blood pressure, or who ...

  11. Thoracic actinomycosis

    PubMed Central

    Slade, P. R.; Slesser, B. V.; Southgate, J.

    1973-01-01

    Six cases of pulmonary infection with Actinomyces Israeli and one case of infection with Nocardia asteroides are described. The incidence of thoracic actinomycosis has declined recently and the classical presentation with chronic discharging sinuses is now uncommon. The cases described illustrate some of the forms which the disease may take. Actinomycotic infection has been noted, not infrequently, to co-exist with bronchial carcinoma and a case illustrating this association is described. Sputum cytology as practised for the diagnosis of bronchial carcinoma has helped to identify the fungi in the sputum. Treatment is discussed, particularly the possible use of oral antibiotics rather than penicillin by injection. Images PMID:4568119

  12. Idiopathic hypersomnia.

    PubMed

    Billiard, Michel; Sonka, Karel

    2016-10-01

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

  13. Thoracic textilomas: CT findings*

    PubMed Central

    Machado, Dianne Melo; Zanetti, Gláucia; Araujo, Cesar Augusto; Nobre, Luiz Felipe; Meirelles, Gustavo de Souza Portes; Pereira e Silva, Jorge Luiz; Guimarães, Marcos Duarte; Escuissato, Dante Luiz; Souza, Arthur Soares; Hochhegger, Bruno; Marchiori, Edson

    2014-01-01

    OBJECTIVE: The aim of this study was to analyze chest CT scans of patients with thoracic textiloma. METHODS: This was a retrospective study of 16 patients (11 men and 5 women) with surgically confirmed thoracic textiloma. The chest CT scans of those patients were evaluated by two independent observers, and discordant results were resolved by consensus. RESULTS: The majority (62.5%) of the textilomas were caused by previous heart surgery. The most common symptoms were chest pain (in 68.75%) and cough (in 56.25%). In all cases, the main tomographic finding was a mass with regular contours and borders that were well-defined or partially defined. Half of the textilomas occurred in the right hemithorax and half occurred in the left. The majority (56.25%) were located in the lower third of the lung. The diameter of the mass was ≤ 10 cm in 10 cases (62.5%) and > 10 cm in the remaining 6 cases (37.5%). Most (81.25%) of the textilomas were heterogeneous in density, with signs of calcification, gas, radiopaque marker, or sponge-like material. Peripheral expansion of the mass was observed in 12 (92.3%) of the 13 patients in whom a contrast agent was used. Intraoperatively, pleural involvement was observed in 14 cases (87.5%) and pericardial involvement was observed in 2 (12.5%). CONCLUSIONS: It is important to recognize the main tomographic aspects of thoracic textilomas in order to include this possibility in the differential diagnosis of chest pain and cough in patients with a history of heart or thoracic surgery, thus promoting the early identification and treatment of this postoperative complication. PMID:25410842

  14. Predictors of curve flexibility in adolescent idiopathic scoliosis: a retrospective study of 100 patients.

    PubMed

    Ameri, Ebrahim; Behtash, Hamid; Mobini, Bahram; Daraie, Ariasb

    2015-01-01

    Curve flexibility in adolescent idiopathic scoliosis (AIS) was one of the major concerns of spinal surgeons since the evolution of surgical correction techniques. In this respect, many tried to identify which criteria denote more rigid curve. In the present study, we aimed toward determining important factors influencing AIS curve flexibility on supine bending films. We assessed radiographs of 100 patients with AIS for direction of curves, number of involved vertebrae, apical vertebral translation and rotation, magnitude of main thoracic curve and T5-T12 kyphosis. Statistical analysis performed via stepwise linear regression model with these variables plus age and sex against flexibility index. According to regression analysis, there was a clear relationship between flexibility indexes (FI) and magnitude of main thoracic curve at all (P<0.001). When we consider flexible curves (FI>50%) against rigid curves, apical vertebral rotation was a major determinant of curve flexibility also (P<0.001). Adolescent idiopathic scoliosis curves with larger Cobb's angle and apical vertebral rotation show less flexibility on supine bending films. PMID:25796026

  15. Thoracic pedicle subtraction osteotomy in the treatment of severe pediatric deformities.

    PubMed

    Bakaloudis, Georgios; Lolli, Francesco; Di Silvestre, Mario; Greggi, Tiziana; Astolfi, Stefano; Martikos, Konstantinos; Vommaro, Francesco; Barbanti-Brodano, Giovanni; Cioni, Alfredo; Giacomini, Stefano

    2011-05-01

    The traditional surgical treatment of severe spinal deformities, both in adult and pediatric patients, consisted of a 360° approach. Posterior-based spinal osteotomy has recently been reported as a useful and safe technique in maximizing kyphosis and/or kyphoscoliosis correction. It obviates the deleterious effects of an anterior approach and can increase the magnitude of correction both in the coronal and sagittal plane. There are few reports in the literature focusing on the surgical treatment of severe spinal deformities in large pediatric-only series (age <16 years old) by means of a posterior-based spinal osteotomy, with no consistent results on the use of a single posterior-based thoracic pedicle subtraction osteotomy in the treatment of such challenging group of patients. The purpose of the present study was to review our operative experience with pediatric patients undergoing a single level PSO for the correction of thoracic kyphosis/kyphoscoliosis in the region of the spinal cord (T12 and cephalad), and determine the safety and efficacy of posterior thoracic pedicle subtraction osteotomy (PSO) in the treatment of severe pediatric deformities. A retrospective review was performed on 12 consecutive pediatric patients (6 F, 6 M) treated by means of a posterior thoracic PSO between 2002 and 2006 in a single Institution. Average age at surgery was 12.6 years (range, 9-16), whereas the deformity was due to a severe juvenile idiopathic scoliosis in seven cases (average preoperative main thoracic 113°; 90-135); an infantile idiopathic scoliosis in two cases (preoperative main thoracic of 95° and 105°, respectively); a post-laminectomy kypho-scoliosis of 95° (for a intra-medullar ependimoma); an angular kypho-scoliosis due to a spondylo-epiphisary dysplasia (already operated on four times); and a sharp congenital kypho-scoliosis (already operated on by means of a anterior-posterior in situ fusion). In all patients a pedicle screws instrumentation was used

  16. [Idiopathic calcium nephrolithiasis: therapeutic aspects].

    PubMed

    Jaeger, P; Portmann, L; Burckhardt, P

    1983-11-26

    The 75% of the renal stone formers have a so-called idiopathic calcium urolithiasis. The majority of these patients, however, do have a detectable biochemical disorder such as hypercalciuria, hyperuricosuria or hyperoxaluria. A high fluid intake unequivocally represents the first step in the therapeutic approach to these patients. Nevertheless, the detection of any type of biochemical disturbance is of great importance since the addition of a specific therapy will then become possible. Patients with absorptive idiopathic hypercalciuria will be advised to decrease their intake of dairy products as a function of the degree of calcium hyperabsorption, and simultaneously the major dietary sources of oxalate such as chocolate, spinach, rhubarb and asparagus will be eliminated; neutral orthophosphates (3-4 times 500 mg/d) or a thiazide, resp. an analogue as chlorthalidone (50 mg/d) are reasonable alternatives. Renal idiopathic hypercalciuria should be treated, according to the authors, with chlorthalidone (50 mg/d), with or without allopurinol (300 mg/d) depending on the presence of concomitant hyperuricosuria. Patients with dietary idiopathic hypercalciuria should be advised to better equilibrate the various components of their dietary intake. Finally, patients with isolated idiopathic hyperuricosuria whose disease would remain active despite a high fluid intake should receive allopurinol (300 mg/d). The treatment of isolated idiopathic hyperoxaluria is not yet well established. Two main arguments favor this so to say "tailored" approach to the idiopathic stone former: first, some metabolic disturbances are causally related to a particularly active and severe urolithiasis, whereas others are less so; second, the lack of efficacy of some types of treatment appears more and more to be due to insufficient screening of the patients before starting a given treatment. PMID:6658421

  17. Society of Thoracic Surgeons

    MedlinePlus

    ... With Its Intense Demands New Website from The Society of Thoracic Surgeons Puts the Power of Information ... Hotel Discount for STS Members Copyright © 2016 The Society of Thoracic Surgeons. All rights reserved. Expanded Proprietary ...

  18. Blunt thoracic trauma.

    PubMed

    Weyant, Michael J; Fullerton, David A

    2008-01-01

    Blunt thoracic trauma represents a significant portion of trauma admissions to hospitals in the United States. These injuries are encountered by physicians in many specialities such as emergency medicine, pediatrics, general surgery and thoracic surgery. Accurate diagnosis and treatment improves the chances of favorable outcomes and it is desirable for all treating physicians to have current knowledge of all aspects of blunt thoracic trauma. Cardiothoracic surgeons often treat the most severe forms of blunt thoracic injuries and we review the aspects of blunt thoracic trauma that are pertinent to the practicing cardiothoracic surgeon. PMID:18420123

  19. Pathology of Idiopathic Interstitial Pneumonias

    PubMed Central

    Hashisako, Mikiko; Fukuoka, Junya

    2015-01-01

    The updated classification of idiopathic interstitial pneumonias (IIPs) in 2013 by American Thoracic Society/European Respiratory Society included several important revisions to the categories described in the 2002 classification. In the updated classification, lymphoid interstitial pneumonia (LIP) was moved from major to rare IIPs, pleuroparenchymal fibroelastosis (PPFE) was newly included in the rare IIPs, acute fibrinous and organizing pneumonia (AFOP) and interstitial pneumonias with a bronchiolocentric distribution are recognized as rare histologic patterns, and unclassifiable IIP (UCIP) was classified as an IIP. However, recent reports indicate the areas of concern that may require further evaluation. Here, we describe the histopathologic features of the updated IIPs and their rare histologic patterns and also point out some of the issues to be considered in this context. PMID:26949346

  20. Juvenile Idiopathic Arthritis

    MedlinePlus

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

  1. Idiopathic cardiomegaly*

    PubMed Central

    1968-01-01

    Cardiomyopathies are certain heart diseases of unknown etiology and pathogenesis, occurring mostly in tropical and subtropical areas, where they constitute a major clinical problem and sometimes a public health problem. The need for international co-operation in the study of such forms of heart disease has long been recognized and WHO convened informal meetings of investigators on various aspects of the subject in 1964, 1965 and 1966. Out of these have arisen co-operative studies co-ordinated by WHO. In November 1967 a fourth informal meeting was held in Kingston, Jamaica, to review the following topics: the progress reports from all co-operating laboratories; the different types of cardiomyopathies; past experience with cardiac registries, and the diagnostic importance of coronary angiography. Steps were taken towards the formulation of a standard terminology, since too many confusing names are currently employed to mean “cardiomegaly of unknown origin”. A common name, “idiopathic cardiomegaly”, was therefore suggested for future use. The account presented here was prepared by Dr Z. Fejfar, Chief Medical Officer, Cardiovascular Diseases, World Health Organization, Geneva, on behalf of the other participants and is a précis of some of the information that was exchanged, some of the views that were expressed and of the suggestions that were made. PMID:4235740

  2. [Genetics of idiopathic epilepsies].

    PubMed

    Weber, Y G; Lerche, H

    2013-02-01

    Idiopathic epilepsies are genetically determined. They are characterized by the observed seizure types, an age-dependent onset, electroencephalographic criteria and concomitant symptoms, such as movement disorders or developmental delay. The main subtypes are the idiopathic (i) generalized, (ii) the focal epilepsies including the benign syndromes of early childhood and (iii) the epileptic encephalopathies as well as the fever-associated syndromes. In recent years, an increasing number of mutations have been identified in genes encoding ion channels, proteins associated to the vesical synaptic cycle or proteins involved in energy metabolism. These mechanisms are pathophysiologically plausible as they influence neuronal excitability. The large number of genetic defects in epilepsy complicates the genetic diagnostic analysis but novel genetic methods are available covering all known genes at a reasonable price. The proof of a genetic defect leads to a definitive diagnosis, is important for the prognostic and genetic counselling and may influence therapeutic decisions in some cases, so that genetic diagnostic testing is becoming increasingly more important and meaningful in many cases in daily clinical practice. PMID:23392265

  3. Emergency Thoracic US: The Essentials.

    PubMed

    Wongwaisayawan, Sirote; Suwannanon, Ruedeekorn; Sawatmongkorngul, Sorravit; Kaewlai, Rathachai

    2016-01-01

    Acute thoracic symptoms are common among adults visiting emergency departments in the United States. Adults with these symptoms constitute a large burden on the overall resources used in the emergency department. The wide range of possible causes can make a definitive diagnosis challenging, even after clinical evaluation and initial laboratory testing. In addition to radiography and computed tomography, thoracic ultrasonography (US) is an alternative imaging modality that can be readily performed in real time at the patient's bedside to help diagnose many thoracic diseases manifesting acutely and in the trauma setting. Advantages of US include availability, relatively low cost, and lack of ionizing radiation. Emergency thoracic US consists of two main parts, lung and pleura US and focused cardiac US, which are closely related. Acoustic mismatches among aerated lungs, pleura, chest wall, and pathologic conditions produce artifacts useful for diagnosis of pneumothorax and pulmonary edema and help in detection of subpleural, pleural, and chest wall pathologic conditions such as pneumonia, pleural effusion, and fractures. Visual assessment of cardiac contractility and detection of right ventricular dilatation and pericardial effusion at focused cardiac US are critical in patients presenting with acute dyspnea and trauma. Additional US examinations of the inferior vena cava for noninvasive volume assessment and of the groin areas for detection of deep venous thrombosis are often performed at the same time. This multiorgan US approach can provide valuable information for emergency treatment of both traumatic and nontraumatic thoracic diseases involving the lungs, pleura, chest wall, heart, and vascular system. Online supplemental material is available for this article. (©)RSNA, 2016. PMID:27035835

  4. [Video-assisted thoracic surgery, lung transplantation and mediastinitis: major issues in thoracic surgery in 2010].

    PubMed

    Borro, José M; Moreno, Ramón; Gómez, Ana; Duque, José Luis

    2011-01-01

    We reviewed the major issues in thoracic surgery relating to the advances made in our specialty in 2010. To do this, the 43(rd) Congress of the Spanish Society of Pneumology and Thoracic Surgery held in La Coruña and the articles published in the Society's journal, Archivos de Bronconeumología, were reviewed. The main areas of interest were related to the development of video-assisted thoracic surgery, lung transplantation and descending mediastinitis. The new tumor-node-metastasis (TNM) classification (7(th) edition), presented last year, was still a topical issue this year. The First Forum of Thoracic Surgeons and the Update in Thoracic Surgery together with the Nurses' Area have constituted an excellent teaching program. PMID:21300211

  5. Optimization Correction Strength Using Contra Bending Technique without Anterior Release Procedure to Achieve Maximum Correction on Severe Adult Idiopathic Scoliosis

    PubMed Central

    Rahyussalim, Ahmad Jabir; Saleh, Ifran; Purnaning, Dyah; Kurniawati, Tri

    2016-01-01

    Adult scoliosis is defined as a spinal deformity in a skeletally mature patient with a Cobb angle of more than 10 degrees in the coronal plain. Posterior-only approach with rod and screw corrective manipulation to add strength of contra bending manipulation has correction achievement similar to that obtained by conventional combined anterior release and posterior approach. It also avoids the complications related to the thoracic approach. We reported a case of 25-year-old male adult idiopathic scoliosis with double curve. It consists of main thoracic curve of 150 degrees and lumbar curve of 89 degrees. His curve underwent direct contra bending posterior approach using rod and screw corrective manipulation technique to achieve optimal correction. After surgery the main thoracic Cobb angle becomes 83 degrees and lumbar Cobb angle becomes 40 degrees, with 5 days length of stay and less than 800 mL blood loss during surgery. There is no complaint at two months after surgery; he has already come back to normal activity with good functional activity. PMID:27064801

  6. Optimal management of idiopathic scoliosis in adolescence

    PubMed Central

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

    2013-01-01

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

  7. Optimal management of idiopathic scoliosis in adolescence.

    PubMed

    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

  8. Idiopathic Intracranial Hypertension (Pseudotumor Cerebri)

    MedlinePlus

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

  9. [Thoracic outlet syndrome].

    PubMed

    Sonoo, Masahiro

    2014-12-01

    Thoracic outlet syndrome (TOS) is a well-known disorder, but its definition has been disputed. TOS is differentiated into five distinct disorders: arterial vascular, venous vascular, traumatic neurovascular, true neurologic (TN-TOS), and nonspecific TOS. TN-TOS is caused by compression of the lower plexus (T1>C8 roots and/or lower trunk) by a fibrous band. The most frequent presenting symptoms are insidious-onset atrophy and weakness of the intrinsic hand muscles, predominantly in the thenar eminence and radial digital flexors. Numbness and sensory loss are usually present, mainly in the ulnar forearm, although severe pain or pain/paresthesia proximal to the elbow can occur; however, sensory symptoms or signs can be absent in some patients. Nerve conduction studies are pathognomonic and show the loss or severe attenuation of the sensory nerve action potential (SNAP) of the medial antebrachial cutaneous nerve. Additionally, they show a severely depressed median compound muscle action potential (CMAP) and, subsequently, a depressed ulnar CMAP and SNAP. TN-TOS is a rare disorder, although its incidence may be higher than previously believed. Hirayama disease is an important differential diagnosis. Nonspecific TOS, which is mainly diagnosed by provocative maneuvers, corresponds to the classical concept of TOS. However, this concept is now challenged and the existence of nonspecific TOS is doubted. PMID:25475030

  10. Thoracic outlet anatomy (image)

    MedlinePlus

    ... spinal vertebra to the rib. There may be pain in the neck and shoulders, and numbess in the last 3 fingers and inner forearm. Thoracic outlet syndrome is usually treated with physical therapy which helps ...

  11. Thoracic Outlet Syndrome

    MedlinePlus

    Thoracic outlet syndrome (TOS) causes pain in the shoulder, arm, and neck. It happens when the nerves or blood vessels just below your ... vein is compressed, your hand might be sensitive to cold, or turn pale or bluish. Your arm ...

  12. Idiopathic headshaking: is it still idiopathic?

    PubMed

    Pickles, Kirstie; Madigan, John; Aleman, Monica

    2014-07-01

    The clinical syndrome of equine idiopathic headshaking (HSK) was first described in the veterinary literature over 100 years ago, and the disorder continues to be a cause of substantial distress for the horse, frustration for the owner and therapeutic challenge for the veterinarian. This review presents a summary of the current knowledge of clinical signs, signalment, aetiopathogenesis, anatomy, diagnosis and treatment of idiopathic HSK. Recent advances in understanding the pathogenesis of the disease will be discussed with reference to human trigeminal neuralgia, along with the implications this may have for potential therapies. PMID:24821361

  13. [Idiopathic Pulmonary Fibrosis].

    PubMed

    Prasse, A

    2015-10-01

    Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pneumonia and a disease of the elderly. Cigarette smoking and longterm exposure to substances harming alveolar epithelial cells are risk factors for the development of IPF. There is also evidence for a genetic susceptibility. IPF is defined as the idiopathic variant of Usual Interstitial Pneumonitis (UIP). Diagnosis of IPF is complex and based on the exclusion of other diseases associated with an UIP pattern. The only cure is lung transplantation. In the last years there was a breakthrough in the treatment of IPF. With pirfenidone and nintedanib there are now two compounds approved for the treatment of IPF. PMID:26444136

  14. [Idiopathic pulmonary trunk aneurysm].

    PubMed

    Uehara, Mayuko; Kuroda, Yosuke; Ohori, Syunsuke; Mawatari, Toru; Morishita, Kiyofumi

    2010-07-01

    Pulmonary trunk aneurysm is generally associated with congenital cardiac defects, pulmonary hypertension, or infection. Idiopathic pulmonary trunk aneurysm without any associated diseases is a rare lesion and has seldom been reported. Here, we report a case of a 68-year-old woman with idiopathic pulmonary trunk aneurysm. The maximum diameter of the aneurysm was 53 mm while she was 142 cm in height. We successfully performed aneurysmorrhaphy and her postoperative course was uneventful. Aneurysmorrhaphy was an effective technique for idiopathic pulmonary trunk aneurysm without pulmonary hypertention. PMID:20662238

  15. Idiopathic cardiomegaly in Africa.

    PubMed

    Ikeme, A C

    1976-01-01

    Idiopathic cardiomegaly is probably the commonest single diagnosis other than hypertension made in tropical and subtropical African cardiovascular practice. Understanding of the nature of this disease has been hampered by failure to recognize the possibility that the term "idiopathic cardiomegaly" may embrace several disease entities. Evidence suggests that many factors, sometimes acting singly, but often acting in combination, may be responsible for the genesis of so-called idiopathic myocardial failure. The future attitude to research should not be one of excluding well-defined forms from the concept of idiopathic cardiomegaly, but one of clinicopathological classification, which should be a prelude to the search, within each moiety of this group of disorders, for a specific or dominant etiological factor. PMID:829042

  16. Idiopathic Pulmonary Fibrosis

    MedlinePlus

    ... the NHLBI on Twitter. What Is Idiopathic Pulmonary Fibrosis? Pulmonary fibrosis (PULL-mun-ary fi-BRO-sis) is a ... time. The formation of scar tissue is called fibrosis. As the lung tissue thickens, your lungs can' ...

  17. Juvenile idiopathic arthritis

    MedlinePlus

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

  18. Idiopathic Scrotal Calcinosis.

    PubMed

    Killedar, Madhura Milind; Shivani, Aslam A; Shinde, Usha

    2016-08-01

    Idiopathic scrotal calcinosis (ISC) is a rare benign condition which presents with multiple, asymptomatic, and painless nodules on the scrotal skin wall. The lesions have been attributed as sebaceous cysts, calcified steatocystoma, fibroma, atheroma, and xanthoma. Shapiro et al. reviewed the histologic data and found no evidence of an epithelial lining, residual cysts, and lipid or organisms, and concluded that the calcification was idiopathic introducing the term "idiopathic scrotal calcinosis." We have studied four cases of idiopathic scrotal calcinosis, one of which had scrotal calcinosis involving the whole of the scrotum. He presented with painless multiple nodules over the scrotum. He was subjected for surgery with SOS skin grafting, but as the scrotal skin is so lax, primary closure is easily possible. In all our four cases, primary closure was easily possible. PMID:27574356

  19. Idiopathic calcified myocardial mass

    PubMed Central

    Patterson, David; Gibson, Derek; Gomes, Ricardo; McDonald, Lawson; Olsen, Eckhardt; Parker, John; Ross, Donald

    1974-01-01

    Patterson, D., Gibson, D., Gomes, R., McDonald, L., Olsen, E., Parker, J., and Ross, D. (1974).Thorax,29, 589-594. Idiopathic calcified myocardial mass. Myocardial calcification can be subdivided into three groups—metastatic, dystrophic or an extension inwards from the pericardium. This case in which the calcified myocardial mass was initially delineated by radiography and by echocardiography and subsequently removed does not fit into any subdivision and has been termed idiopathic. Images PMID:4279467

  20. Thoracic spine x-ray

    MedlinePlus

    Vertebral radiography; X-ray - spine; Thoracic x-ray; Spine x-ray; Thoracic spine films; Back films ... Gillard JH, Schaefer-Prokop CM, eds. Grainger & Allison's Diagnostic Radiology: A Textbook of Medical Imaging . 6th ed. New ...

  1. Understanding Thoracic Outlet Syndrome

    PubMed Central

    Freischlag, Julie

    2014-01-01

    The diagnosis of thoracic outlet syndrome was once debated in the world of vascular surgery. Today, it is more understood and surprisingly less infrequent than once thought. Thoracic outlet syndrome (TOS) is composed of three types: neurogenic, venous, and arterial. Each type is in distinction to the others when considering patient presentation and diagnosis. Remarkable advances have been made in surgical approach, physical therapy, and rehabilitation of these patients. Dedicated centers of excellence with multidisciplinary teams have been developed and continue to lead the way in future research. PMID:25140278

  2. A neglected point in surgical treatment of adolescent idiopathic scoliosis: Variations in the number of vertebrae.

    PubMed

    Hu, Zongshan; Zhang, Zhen; Zhao, Zhihui; Zhu, Zezhang; Liu, Zhen; Qiu, Yong

    2016-08-01

    Inaccurate identification of vertebral levels is the main cause of wrong-site spine surgery which is performed by nearly half of the spine surgeons. Unusual anatomy and failure to verify the surgical level on radiographs have been commonly reported. We aimed at investigating the variations in vertebral number in adolescent idiopathic scoliosis (AIS) patients and thus to raise awareness of the possibility for wrong-level spinal surgery and to make a comparison with normal adolescents. A cohort of 657 AIS patients and 248 normal adolescents, presented to our center from June 2008 to February 2013, who met the inclusion criteria, were recruited. Radiographs were reviewed to identify the number of thoracic or lumbar vertebrae and the presence of a lumbosacral transitional vertebra. In the AIS group, 70 (10.6%) patients had variations in the number of thoracic and/or lumbar vertebrae. Remarkably, the prevalence of variations in male subjects was significantly higher than that in female subjects (P < 0.05). Thirty-seven patients (5.6%) had an atypical number of thoracic vertebrae, with 33 having 11 thoracic vertebrae and 4 patients having 13. Forty-eight patients (7.3%) had an atypical number of lumbar vertebrae, with 14 having 4 lumbar vertebrae and 34 patients having 6. Multilevel vertebral anomalies were present in 2.3% of the patients (15 of 657). A variation in the number of vertebrae had been identified in 1.7% (11) of the reports by the radiologist. In the normal group, 27 (10.9%) subjects showed variations in the vertebral number. There was no significant difference in the prevalence of atypical numbers of vertebral number between the AIS and normal groups (P > 0.05). Therefore, we concluded that variations in the number of thoracic-lumbar vertebrae were found in up to10.6% of AIS patients. Identification of variations in the number of vertebrae is crucial to serve to decrease the risk of wrong-level surgery. PMID:27559975

  3. Acute surgical management in idiopathic intracranial hypertension

    PubMed Central

    Zakaria, Zaitun; Fenton, Eoin; Sattar, Muhammad Taufiq

    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. PMID:23239783

  4. Genetic polymorphisms and idiopathic generalized epilepsies.

    PubMed

    Lucarini, Nazzareno; Verrotti, Alberto; Napolioni, Valerio; Bosco, Guido; Curatolo, Paolo

    2007-09-01

    In recent years, progress in understanding the genetic basis of idiopathic generalized epilepsies has proven challenging because of their complex inheritance patterns and genetic heterogeneity. Genetic polymorphisms offer a convenient avenue for a better understanding of the genetic basis of idiopathic generalized epilepsy by providing evidence for the involvement of a given gene in these disorders, and by clarifying its pathogenetic mechanisms. Many of these genes encode for some important central nervous system ion channels (KCNJ10, KCNJ3, KCNQ2/KCNQ3, CLCN2, GABRG2, GABRA1, SCN1B, and SCN1A), while many others encode for ubiquitary enzymes that play crucial roles in various metabolic pathways (HP, ACP1, ME2, LGI4, OPRM1, GRIK1, BRD2, EFHC1, and EFHC2). We review the main genetic polymorphisms reported in idiopathic generalized epilepsy, and discusses their possible functional significance in the pathogenesis of seizures. PMID:17765802

  5. Radiology of thoracic diseases

    SciTech Connect

    Swensen, S.J.; Pugatch, R.D.

    1989-01-01

    This book presents the essential clinical and radiologic findings of a wide variety of thoracic diseases. The authors include conventional, CT and MR images of each disease discussed. In addition, they present practical differential diagnostic considerations for most of the radiographic findings or patterns portrayed.

  6. [Thoracic oncology: annual review].

    PubMed

    Sculier, J-P; Berghmans, T; Meert, A-P

    2013-01-01

    The objective of this paper is to review the literature published in 2011-12 in the field of thoracic oncology. Are discussed because of new original publications: epidemiology, screening, pulmonary nodule, diagnosis and assessment, treatment of lung cancer non-small cell, small cell lung cancer, prognosis, palliative care and end of life, organization of care, mesothelioma. PMID:23755717

  7. [Single Port Thoracic Surgery and Reduced Port Thoracic Surgery].

    PubMed

    Onodera, Ken; Noda, Masafumi

    2016-07-01

    Single port thoracic surgery, reduced port surgery and needlescopic surgery attract attention as one of the minimally invasive surgery in thoracic surgery recently. Single port thoracic surgery was advocated by Rocco in 2004, it was reported usefulness of single port thoracic surgery for primary spontaneous pneumothorax. The surgical procedure as single (or reduced) port thoracic surgery is roughly divided into the following. One is operated with instruments inserted from the single extended incision, and the other is operated with instruments punctured without extending incision. It is not generally complicated procedures in single port thoracic surgery. Primary spontaneous pneumothorax and biopsy for lung and pleura are considered the surgical indication for single (or reduced) port surgery. It is revealed that single port surgery for primary spontaneous pneumothorax is less invasive than conventional surgery. Single port and reduced port thoracic surgery will spread furthermore in the future. PMID:27440029

  8. Dobosiewicz method physiotherapy for idiopathic scoliosis.

    PubMed

    Dobosiewicz, Krystyna; Durmala, Jacek; Kotwicki, Tomasz

    2008-01-01

    The method developed since 1979, comprises active 3-dimensional auto-correction, concerning the primary curve mobilization towards the correction of the curvature, with special emphasis on the kyphotization of the thoracic spine, carried on in closed kinematic chains, and developed on a symmetrically positioned pelvis and shoulder girdle, followed by active stabilization of the corrected position, and endured as postural habit. The positions for exercising and the movements involved are described in details. Small, moderate and important curves can be managed with DoboMed, however the effectiveness of the therapy depends on the curve flexibility and patient's compliance. DoboMed has been used as a single therapy or together with bracing, as well as preparation for scoliosis surgery. The published results demonstrated that the DoboMed has a positive influence on inhibition of the curve progression in idiopathic scoliosis, the improvement of respiratory functions, assessed by the spirometric values, and the general exercise efficiency evaluated using ergospirometry. PMID:18401093

  9. [Thoracic nocardiosis - a clinical report].

    PubMed

    Vale, Artur; Guerra, Miguel; Martins, Daniel; Lameiras, Angelina; Miranda, José; Vouga, Luís

    2014-01-01

    Nocardia genus microorganisms are ubiquitous, Gram positive aerobic bacterias, responsible for disease mainly in immunocompromised hosts, with cellular immune response commitment. Inhalation is the main form of transmition and pulmonary disease is the most frequent presentation. Dissemination may occur by contiguity and also via hematogenous. The clinical and imaging presentation is not specific, and diagnosis is obtained after identification of Nocardia bacteria in biological samples. Since there are no reliable studies that indicate the best therapeutic option, treatment should be individualized and based on antimicrobial susceptibility testing. Surgical drainage should also be considered in all patients. The authors present a clinical case of a patient with thoracic nocardiosis, and make a short literature review on the theme. PMID:25596394

  10. History and current status of mini-invasive thoracic surgery

    PubMed Central

    He, Jianxing

    2011-01-01

    Mini-invasive thoracic technique mainly refers to a technique involving the significant reduction of the chest wall access-related trauma. Notably, thoracoscope is the chief representative. The development of thoracoscope technique is characterized by: developing from direct peep to artificial lighting, then combination with image and video technique in equipments; technically developing from diagnostic to therapeutic approaches; developing from simpleness to complexity in application scope; and usually developing together with other techniques. At present, the widely used mini-invasive thoracic surgery refers to the mini-open thoracic surgery performed mainly by using some instruments to control target tissues and organs based on the vision associated with multi-limb coordination, which may be hand-assisted if necessary. The mini-invasive thoracic surgery consists of three approaches including video-assisted thoracic surgery (VATS), video-assisted Hybrid and hand-assisted VATS. So far the mini-invasive thoracic technique has achieved great advances due to the development in instruments of mini-invasive thoracic surgery which has the following features: instruments of mini-invasive thoracic surgery appear to be safe and practical, and have successive improvement and diversification in function; the specific instruments of open surgeries has been successively developed into dedicated instruments of endoscopic surgery; the application of endoscopic mechanical suture device generates faster fragmentation and reconstruction of organ tissues; the specific delicated instruments of endoscopic surgery have rapid development and application; and the simple instruments structurally similar to the conventional instruments are designed according to the mini-incison. In addition, the mini-invasive thoracic technique is widely used in five aspects including diseases of pleura membrane and chest wall, lung diseases, esophageal diseases, mediastinal diseases and heart diseases

  11. Idiopathic Interstitial Pneumonia

    PubMed Central

    Flaherty, Kevin R.; Andrei, Adin-Cristian; King, Talmadge E.; Raghu, Ganesh; Colby, Thomas V.; Wells, Athol; Bassily, Nadir; Brown, Kevin; du Bois, Roland; Flint, Andrew; Gay, Steven E.; Gross, Barry H.; Kazerooni, Ella A.; Knapp, Robert; Louvar, Edmund; Lynch, David; Nicholson, Andrew G.; Quick, John; Thannickal, Victor J.; Travis, William D.; Vyskocil, James; Wadenstorer, Frazer A.; Wilt, Jeffrey; Toews, Galen B.; Murray, Susan; Martinez, Fernando J.

    2007-01-01

    Rationale: Treatment and prognoses of diffuse parenchymal lung diseases (DPLDs) varies by diagnosis. Obtaining a uniform diagnosis among observers is difficult. Objectives: Evaluate diagnostic agreement between academic and community-based physicians for patients with DPLDs, and determine if an interactive approach between clinicians, radiologists, and pathologists improved diagnostic agreement in community and academic centers. Methods: Retrospective review of 39 patients with DPLD. A total of 19 participants reviewed cases at 2 community locations and 1 academic location. Information from the history, physical examination, pulmonary function testing, high-resolution computed tomography, and surgical lung biopsy was collected. Data were presented in the same sequential fashion to three groups of physicians on separate days. Measurements and Main Results: Each observer's diagnosis was coded into one of eight categories. A κ statistic allowing for multiple raters was used to assess agreement in diagnosis. Interactions between clinicians, radiologists, and pathologists improved interobserver agreement at both community and academic sites; however, final agreement was better within academic centers (κ = 0.55–0.71) than within community centers (κ = 0.32–0.44). Clinically significant disagreement was present between academic and community-based physicians (κ = 0.11–0.56). Community physicians were more likely to assign a final diagnosis of idiopathic pulmonary fibrosis compared with academic physicians. Conclusions: Significant disagreement exists in the diagnosis of DPLD between physicians based in communities compared with those in academic centers. Wherever possible, patients should be referred to centers with expertise in diffuse parenchymal lung disorders to help clarify the diagnosis and provide suggestions regarding treatment options. PMID:17255566

  12. [Thoracic actinomycosis: three cases].

    PubMed

    Herrak, L; Msougar, Y; Ouadnouni, Y; Bouchikh, M; Benosmane, A

    2007-09-01

    Actinomycosis is a rare condition which, in the thoracic localisation, can mimic cancer or tuberculosis. We report a series of three case of thoracic actinomycosis treated in the Ibn Sina University Thoracic Surgery Unit in Rabat, Morocco. CASE N degrees 1: This 45-year-old patient presented a tumefaction on the left anterior aspect of the chest. Physical examination identified a parietal mass with fistulisation to the skin. Radiography demonstrated a left pulmonary mass. Transparietal puncture led to the pathological diagnosis of actinomycosis. The patient was given medical treatment and improved clinically and radiographically. CASE N degrees 2: This 68-year-old patient presented repeated episodes of hemoptysis. The chest x-ray revealed atelectasia of the middle lobe and bronchial fibroscopy demonstrated the presence of a bud in the middle lobar bronchus. Biopsies were negative. The patient underwent surgery and the histology examination of the operative specimen revealed pulmonary actinomycosis. The patient recovered well clinically and radiographically with antibiotic therapy. CASE N degrees 3: This 56-year-old patient presented cough and hemoptysis. Physical examination revealed a left condensation and destruction of the left lung was noted on the chest x-ray. Left pleuropulmonectomy was performed. Histological analysis of the surgical specimen identified associated Aspergillus and Actinomyces. The outcome was favorable with medical treatment. The purpose of this work was to recall the radiological, clinical, histological, therapeutic, outcome aspects of this condition and to relate the problems of differential diagnosis when can suggest other diseases. PMID:17978739

  13. Idiopathic Inflammatory Myopathies

    PubMed Central

    Dimachkie, Mazen M.; Barohn, Richard J.

    2012-01-01

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

  14. Nonintubated anesthesia for thoracic surgery

    PubMed Central

    Wang, Bei

    2014-01-01

    Nonintubated thoracic surgery has been used in procedures including pleura, lungs and mediastinum. Appropriate anesthesia techniques with or without sedation allow thoracic surgery patients to avoid the potential risks of intubated general anesthesia, particularly for the high-risk patients. However, nonintubated anesthesia for thoracic surgery has some benefits as well as problems. In this review, the background, indication, perioperative anesthetic consideration and management, and advantages and disadvantages are discussed and summarized. PMID:25589994

  15. Nonmalignant Adult Thoracic Lymphatic Disorders.

    PubMed

    Itkin, Maxim; McCormack, Francis X

    2016-09-01

    The thoracic lymphatic disorders are a heterogeneous group of uncommon conditions that are associated with thoracic masses, interstitial pulmonary infiltrates, and chylous complications. Accurate diagnosis of the thoracic lymphatic disorders has important implications for the newest approaches to management, including embolization and treatment with antilymphangiogenic drugs. New imaging techniques to characterize lymphatic flow, such as dynamic contrast-enhanced magnetic resonance lymphangiogram, are redefining approaches to disease classification and therapy. PMID:27514588

  16. Thoracic Endometriosis Syndrome: A Veritable Pandora's Box.

    PubMed

    Nair, Sobha S; Nayar, Jayashree

    2016-04-01

    Thoracic endometriosis syndrome is a rare disorder characterised by the presence of functioning endometrial tissue in pleura, lung parenchyma, airways, and/or encompasses mainly four clinical entities-catamenial pneumothorax, catamenial haemothorax, catamenial haemoptysis and lung nodules. The cases were studied retrospectively by reviewing the records at Amrita Institute of Medical Sciences, for duration of five years i.e., form March 2010-2014 and analysed for the clinical presentation and management of thoracic endometriosis syndrome. Catamenial breathlessness was the main symptom. Pneumothorax and pleural effusion were the findings on investigations. Histopathology report of endometriosis was present in three cases (50%). Conditions with excess oestrogen like endometriosis, fibroid, adenomyosis were diagnosed in these patients by pelvic scan. After the initial supportive treatment with hormones, pleurodesis, hysterectomy and lung decortication were the treatment modalities. Two cases that had multiple recurrences were diagnosed as disseminated TES. They underwent combined treatment of surgery and hormones. PMID:27190904

  17. Thoracic trauma in horses.

    PubMed

    Sprayberry, Kim A; Barrett, Elizabeth J

    2015-04-01

    Traumatic injuries involving the thorax can be superficial, necessitating only routine wound care, or they may extend to deeper tissue planes and disrupt structures immediately vital to respiratory and cardiac function. Diagnostic imaging, especially ultrasound, should be considered part of a comprehensive examination, both at admission and during follow-up. Horses generally respond well to diligent monitoring, intervention for complications, and appropriate medical or surgical care after sustaining traumatic wounds of the thorax. This article reviews the various types of thoracic injury and their management. PMID:25770070

  18. Genetics Home Reference: adolescent idiopathic scoliosis

    MedlinePlus

    ... Understand Genetics Home Health Conditions adolescent idiopathic scoliosis adolescent idiopathic scoliosis Enable Javascript to view the expand/ ... boxes. Download PDF Open All Close All Description Adolescent idiopathic scoliosis is an abnormal curvature of the ...

  19. Thoracic spine x-ray

    MedlinePlus

    Vertebral radiography; X-ray - spine; Thoracic x-ray; Spine x-ray; Thoracic spine films; Back films ... The test is done in a hospital radiology department or in the health care provider's office. You will lie on the x-ray table in different positions. If the x-ray ...

  20. Aneurysms: thoracic aortic aneurysms.

    PubMed

    Chun, Kevin C; Lee, Eugene S

    2015-04-01

    Thoracic aortic aneurysms (TAAs) have many possible etiologies, including congenital heart defects (eg, bicuspid aortic valves, coarctation of the aorta), inherited connective tissue disorders (eg, Marfan, Ehlers-Danlos, Loeys-Dietz syndromes), and degenerative conditions (eg, medial necrosis, atherosclerosis of the aortic wall). Symptoms of rupture include a severe tearing pain in the chest, back, or neck, sometimes associated with cardiovascular collapse. Before rupture, TAAs may exert pressure on other thoracic structures, leading to a variety of symptoms. However, most TAAs are asymptomatic and are found incidentally during imaging for other conditions. Diagnosis is confirmed with computed tomography scan or echocardiography. Asymptomatic TAAs should be monitored with imaging at specified intervals and patients referred for repair if the TAAs are enlarging rapidly (greater than 0.5 cm in diameter over 6 months for heritable etiologies; greater than 0.5 cm over 1 year for degenerative etiologies) or reach a critical aortic diameter threshold for elective surgery (5.5 cm for TAAs due to degenerative etiologies, 5.0 cm when associated with inherited syndromes). Open surgery is used most often to treat asymptomatic TAAs in the ascending aorta and aortic arch. Asymptomatic TAAs in the descending aorta often are treated medically with aggressive blood pressure control, though recent data suggest that endovascular procedures may result in better long-term survival rates. PMID:25860136

  1. An idiopathic gigantomastia.

    PubMed

    Cho, Min Jeng; Yang, Jung-Hyun; Choi, Hyeon-Gon; Kim, Wan Seop; Yu, Yeong-Beom; Park, Kyoung Sik

    2015-03-01

    Gigantomastia is a rare condition characterized by excessive breast growth. It has been reported that the majority of gigantomastia cases occur during either pregnancy or puberty. We were presented with a rare case of gigantomastia associated with neither pregnancy nor puberty, and successfully treated it with reduction mammaplasty and free nipple graft. This idiopathic gigantomastia is the very first case in Korea, and adds to the worldwide total of 9 reported cases. PMID:25741497

  2. An idiopathic gigantomastia

    PubMed Central

    Cho, Min Jeng; Choi, Hyeon-Gon; Kim, Wan Seop; Yu, Yeong-Beom; Park, Kyoung Sik

    2015-01-01

    Gigantomastia is a rare condition characterized by excessive breast growth. It has been reported that the majority of gigantomastia cases occur during either pregnancy or puberty. We were presented with a rare case of gigantomastia associated with neither pregnancy nor puberty, and successfully treated it with reduction mammaplasty and free nipple graft. This idiopathic gigantomastia is the very first case in Korea, and adds to the worldwide total of 9 reported cases. PMID:25741497

  3. Idiopathic Polydactylous Longitudinal Erythronychia

    PubMed Central

    2011-01-01

    Objective: To describe the clinical features of idiopathic polydactylous longitudinal erythronychia. Introduction: Longitudinal erythronychia presents as a linear red band on the nail plate. Idiopathic polydactylous longitudinal erythronychia is a rarely described manifestation of longitudinal erythronychia in which one or more linear red bands present on the nails of multiple digits without any associated subungual malignant tumor, dermatological condition, or systemic disease. Methods: As part of a total body skin examination, the fingernails and toenails were evaluated for linear red bands. Results: One or more asymptomatic linear red bands (longitudinal erythronychia) was observed on multiple digits of the hands in one percent (3 men of 134 men and 112 women) of patients examined during a period of 75 days. The author also noted similar changes of his own nails. Between 3 to 10 digits were affected. Multiple linear red bands per nail were usually narrow (less than 1mm wide), whereas a single band on a nail often ranged from 4 to 6mm wide. The intensity of an individual wider linear red band was position-dependent in three individuals in whom the distal portion appeared less prominent when the affected digit was held upward above the level of the patient's heart—pseudolongitudinal erythronychia. Other nail changes in these patients included distal subungual hyperkeratosis, fissuring at the free end of the nail, leukonychia, red lunula, and splinter hemorrhages. Discussion: Idiopathic polydactylous longitudinal erythronychia is a benign, usually asymptomatic, condition of undetermined etiology characterized by one or more linear red bands originating at the proximal nail fold or distal lunula and extending to the free edge of the nail. It appears to be more prevalent in men over 50 years of age and its incidence was noted to be one percent of adults attending a dermatology clinic. Patients are either unaware of the nail changes or seek medical attention because

  4. Thoracic outlet syndrome.

    PubMed

    Ozoa, Glenn; Alves, Daniel; Fish, David E

    2011-08-01

    Of the many clinical entities involving the neck region, one of the most intriguing is thoracic outlet syndrome (TOS). TOS is an array of disorders that involves injury to the neurovascular structures in the cervicobrachial region. A classification system based on etiology, symptoms, clinical presentation, and anatomy is supported by most physicians. The first type of TOS is vascular, involving compression of either the subclavian artery or vein. The second type is true neurogenic TOS, which involves injury to the brachial plexus. Finally, the third and most controversial type is referred to as disputed neurogenic TOS. This article aims to provide the reader some understanding of the pathophysiology, workup, and treatment of this fascinating clinical entity. PMID:21824588

  5. Vascular Thoracic Outlet Syndrome.

    PubMed

    Hussain, Mohamad Anas; Aljabri, Badr; Al-Omran, Mohammed

    2016-01-01

    Two distinct terms are used to describe vascular thoracic outlet syndrome (TOS) depending on which structure is predominantly affected: venous TOS (due to subclavian vein compression) and arterial TOS (due to subclavian artery compression). Although the venous and arterial subtypes of TOS affect only 3% and <1% of all TOS patients respectively, the diagnostic and management approaches to venous and arterial TOS have undergone considerable evolution due to the recent emergence of minimally invasive endovascular techniques such as catheter-directed arterial and venous thrombolysis, and balloon angioplasty. In this review, we discuss the anatomical factors, etiology, pathogenesis and clinical presentation of vascular TOS patients. In addition, we use the most up to date observational evidence available to provide a contemporary approach to the diagnosis and management of venous TOS and arterial TOS patients. PMID:27568153

  6. Helicobacter pylori-negative, non-steroidal anti-inflammatory drug: negative idiopathic ulcers in Asia.

    PubMed

    Iijima, Katsunori; Kanno, Takeshi; Koike, Tomoyuki; Shimosegawa, Tooru

    2014-01-21

    Since the discovery of Helicobacter pylori (H. pylori) infection in the stomach, the bacteria infection and non-steroidal anti-inflammatory drugs (NSAIDs) use had been considered to be the 2 main causes of peptic ulcers. However, there have been recent reports of an increase in the proportion of peptic ulcers without these known risk factors; these are termed idiopathic peptic ulcers. Such trend was firstly indicated in 1990s from some reports in North America. In Asia, numerous studies reported that idiopathic ulcers accounted for a small percentage of all ulcers in the 1990s, but in the 2000s, multiple studies reported that the proportion of idiopathic ulcers had reached 10%-30%, indicating that the incidence of idiopathic ulcers in Asia has also been rising in recent years. While a decline in H. pylori infection rates of general population in Asia is seen as the main reason for the increased incidence of idiopathic ulcers, it is also possible that the absolute number of idiopathic ulcer cases has increased. Advanced age, serious systemic complication, and psychological stress are considered to be the potential risk factors for idiopathic ulcers. Management of idiopathic ulcers is challenging, at present, because there is no effective preventative measure against recurrence in contrast with cases of H. pylori-positive ulcers and NSAIDs-induced ulcers. As it is expected that H. pylori infection rates in Asia will decline further in the future, measures to treat idiopathic ulcers will also likely become more important. PMID:24574744

  7. The European educational platform on thoracic surgery

    PubMed Central

    Rocco, Gaetano; Venuta, Federico

    2014-01-01

    As the largest scientific organisation world-wide exclusively dedicated to general thoracic surgery (GTS), the European Society of Thoracic Surgeons (ESTS) recognized that one of its priorities is education. The educational platform designed ESTS addresses not only trainees, but also confirmed thoracic surgeons. The two main aims are (I) to prepare trainees to graduation and to the certification by the European Board of Thoracic Surgery and (II) to offer opportunities for continuous medical education in the perspective of life-long learning and continuous professional development to certified thoracic surgeons. It is likely that recertification will become an obligation during the coming decade. At its inception, the platform differentiated two different events. A 6-day course emphasizing on theoretic knowledge was created in Antalya in 2007. The same year, a 2-day school oriented to practical issues with hands-on in the animal lab was launched in Antalya. These two teaching tracks need further development. In the knowledge track, we intend to organize highly specialized 2-day courses to deepen insight into theoretical questions. The skill track will be implemented by specialized courses for high technology such as tracheal surgery, ECMO, robotics or chest wall reconstruction. In order to promote tomorrows’ leadership, we created an academic competence track giving an insight into medical communication, methodology and management. We also had to respond to an increasing demand from the Russian speaking countries, where colleagues may face problems to attend western meetings, and where the language bareer may be a major impediment. We initiated a Russian school with three events yearly in 2012. Contemporary teaching must be completed with an e-learning platform, which is currently under development. The school activities are organized by the educational committee, which is headed by the ESTS Director of Education, assisted by coordinators of the teaching tracks

  8. The European educational platform on thoracic surgery.

    PubMed

    Massard, Gilbert; Rocco, Gaetano; Venuta, Federico

    2014-05-01

    As the largest scientific organisation world-wide exclusively dedicated to general thoracic surgery (GTS), the European Society of Thoracic Surgeons (ESTS) recognized that one of its priorities is education. The educational platform designed ESTS addresses not only trainees, but also confirmed thoracic surgeons. The two main aims are (I) to prepare trainees to graduation and to the certification by the European Board of Thoracic Surgery and (II) to offer opportunities for continuous medical education in the perspective of life-long learning and continuous professional development to certified thoracic surgeons. It is likely that recertification will become an obligation during the coming decade. At its inception, the platform differentiated two different events. A 6-day course emphasizing on theoretic knowledge was created in Antalya in 2007. The same year, a 2-day school oriented to practical issues with hands-on in the animal lab was launched in Antalya. These two teaching tracks need further development. In the knowledge track, we intend to organize highly specialized 2-day courses to deepen insight into theoretical questions. The skill track will be implemented by specialized courses for high technology such as tracheal surgery, ECMO, robotics or chest wall reconstruction. In order to promote tomorrows' leadership, we created an academic competence track giving an insight into medical communication, methodology and management. We also had to respond to an increasing demand from the Russian speaking countries, where colleagues may face problems to attend western meetings, and where the language bareer may be a major impediment. We initiated a Russian school with three events yearly in 2012. Contemporary teaching must be completed with an e-learning platform, which is currently under development. The school activities are organized by the educational committee, which is headed by the ESTS Director of Education, assisted by coordinators of the teaching tracks and

  9. Glucocorticoid pharmacogenetics in pediatric idiopathic nephrotic syndrome.

    PubMed

    Cuzzoni, Eva; De Iudicibus, Sara; Franca, Raffaella; Stocco, Gabriele; Lucafò, Marianna; Pelin, Marco; Favretto, Diego; Pasini, Andrea; Montini, Giovanni; Decorti, Giuliana

    2015-01-01

    Idiopathic nephrotic syndrome represents the most common type of primary glomerular disease in children: glucocorticoids (GCs) are the first-line therapy, even if considerable interindividual differences in their efficacy and side effects have been reported. Immunosuppressive and anti-inflammatory effects of these drugs are mainly due to the GC-mediated transcription regulation of pro- and anti-inflammatory genes. This mechanism of action is the result of a complex multistep pathway that involves the glucocorticoid receptor and several other proteins, encoded by polymorphic genes. Aim of this review is to highlight the current knowledge on genetic variants that could affect GC response, particularly focusing on children with idiopathic nephrotic syndrome. PMID:26419298

  10. Idiopathic infantile hypercalcaemia--a continuing enigma.

    PubMed Central

    Martin, N D; Snodgrass, G J; Cohen, R D

    1984-01-01

    Seventy six children with documented Fanconi-type idiopathic infantile hypercalcaemia were studied and compared with 41 with the Williams-Beuren syndrome. Clinical comparison showed, as expected, very close similarities but also considerable differences, particularly in the severity of feeding problems and the degree of failure to thrive. The estimated incidence of idiopathic infantile hypercalcaemia alone has remained constant for the past 20 years, at approximately 18 cases per year in the United Kingdom (1 per 47 000 total live births). Long term morbidity in these children is mainly due to mental handicap and arteriopathy, but hypertension (29%), kyphoscoliosis (19%), hyperacusis (75%), and obesity (50%) may be added complications. In one child, hypercalcaemia recurred during adolescence but this seems to be excessively rare. More detailed investigation before treatment is required to discover the aetiology of hypercalcaemia in this condition. Images Fig. 2 PMID:6465928

  11. The European general thoracic surgery database project

    PubMed Central

    Brunelli, Alessandro

    2014-01-01

    The European Society of Thoracic Surgeons (ESTS) Database is a free registry created by ESTS in 2001. The current online version was launched in 2007. It runs currently on a Dendrite platform with extensive data security and frequent backups. The main features are a specialty-specific, procedure-specific, prospectively maintained, periodically audited and web-based electronic database, designed for quality control and performance monitoring, which allows for the collection of all general thoracic procedures. Data collection is the “backbone” of the ESTS database. It includes many risk factors, processes of care and outcomes, which are specially designed for quality control and performance audit. The user can download and export their own data and use them for internal analyses and quality control audits. The ESTS database represents the gold standard of clinical data collection for European General Thoracic Surgery. Over the past years, the ESTS database has achieved many accomplishments. In particular, the database hit two major milestones: it now includes more than 235 participating centers and 70,000 surgical procedures. The ESTS database is a snapshot of surgical practice that aims at improving patient care. In other words, data capture should become integral to routine patient care, with the final objective of improving quality of care within Europe. PMID:24868445

  12. The European general thoracic surgery database project.

    PubMed

    Falcoz, Pierre Emmanuel; Brunelli, Alessandro

    2014-05-01

    The European Society of Thoracic Surgeons (ESTS) Database is a free registry created by ESTS in 2001. The current online version was launched in 2007. It runs currently on a Dendrite platform with extensive data security and frequent backups. The main features are a specialty-specific, procedure-specific, prospectively maintained, periodically audited and web-based electronic database, designed for quality control and performance monitoring, which allows for the collection of all general thoracic procedures. Data collection is the "backbone" of the ESTS database. It includes many risk factors, processes of care and outcomes, which are specially designed for quality control and performance audit. The user can download and export their own data and use them for internal analyses and quality control audits. The ESTS database represents the gold standard of clinical data collection for European General Thoracic Surgery. Over the past years, the ESTS database has achieved many accomplishments. In particular, the database hit two major milestones: it now includes more than 235 participating centers and 70,000 surgical procedures. The ESTS database is a snapshot of surgical practice that aims at improving patient care. In other words, data capture should become integral to routine patient care, with the final objective of improving quality of care within Europe. PMID:24868445

  13. [Juvenile idiopathic epiretinal membrane].

    PubMed

    Kontopoulou, K; Krause, S; Fili, S; Hayvazov, S; Schilling, H; Kohlhaas, M

    2016-07-01

    Idiopathic epiretinal membrane (iERM) is very rare in adolescent patients. The pathogenesis remains unclear although the role of hyalocytes is of major importance. The clinical features in young patients are different from those in older patients. We describe a case of iERM in a 15-year-old girl who presented with metamorphopsia of the right eye. This case report presents the basis for the decision for surgical treatment as well as the clinical features at follow-up examination 9 months after surgery. PMID:26458892

  14. Idiopathic gingival fibromatosis

    PubMed Central

    Dani, Nitin Hemchandra; Khanna, Dinkar Parveen; Bhatt, Vaibhavi Hitesh; Joshi, Chaitanya Pradeep

    2015-01-01

    Idiopathic gingival fibromatosis (IGF) is a rare hereditary condition characterized by slowly progressive, nonhemorrhagic, fibrous enlargement of maxillary and mandibular keratinized gingiva caused by increase in submucosal connective tissue elements, mostly associated with some syndrome. This case report describes a case of nonsyndromic generalized IGF in an 18-year-old male patient who presented with generalized gingival enlargement. The enlarged tissue was surgically removed by internal bevel gingivectomy and ledge and wedge procedure. The patient was regularly monitored clinically for improvement in his periodontal condition as well as for any recurrence of gingival overgrowth. PMID:26941525

  15. [Idiopathic granulomatous mastitis].

    PubMed

    Hello, M; Néel, A; Graveleau, J; Masseau, A; Agard, C; Caillon, J; Hamidou, M

    2013-06-01

    Idiopathic granulomatous mastitis (IGM) is a rare localized granulomatosis of unknown aetiology that usually affects women of childbearing age. It often mimics breast carcinoma or abscess. Histopathologic evaluation and elimination of the others aetiologies of granuloma play a crucial role in the diagnosis. Its etiopathogeny remains poorly understood, but Corynebacteria might be involved. The disease course is usually protracted, with a significant impact on quality of life. The management of IGM remains controversial, but corticosteroids are usually the first-line treatment. PMID:22981187

  16. [Thoracic endometriosis: A difficult diagnosis].

    PubMed

    Hagneré, P; Deswarte, S; Leleu, O

    2011-09-01

    Thoracic endometriosis is a rare disease, which presents in women at a mean age of 35 years, later than for pelvic endometriosis. There are no known predisposing factors for the condition and its pathogenesis is not yet clearly established. The symptoms always appear in connection with the periods of the person affected by the condition, occurring within 24-48 h after the start of menstruation. Catamenial pneumothorax is the most common clinical entity. It is associated with pelvic endometriosis in 30-50% of cases. Thoracoscopy, preferably performed during menstruation, allows full inspection of the diaphragm and the pleural cavity for defects in the diaphragm, endometrial nodules and bullae. The level of CA 125 is often elevated but this is not a reliable or specific marker. Medical treatment is aimed at blocking the action of estrogen on the endometrium and ectopic endometrial implants. GnRH analogues or danazol are the preferred treatments. Surgery to repair and strengthen the diaphragm and/or resect nodules or bullae also has a role, supplemented by pleurodesis to prevent further pneumothorax or effusions. The main risk is recurrence, and thus the current usual practice is to combine surgery, immediately followed by hormone therapy focusing on GnRH analogues. PMID:21943537

  17. Invasive diagnostic techniques in idiopathic interstitial pneumonias.

    PubMed

    Poletti, Venerino; Ravaglia, Claudia; Gurioli, Carlo; Piciucchi, Sara; Dubini, Alessandra; Cavazza, Alberto; Chilosi, Marco; Rossi, Andrea; Tomassetti, Sara

    2016-01-01

    Fibrosing interstitial lung diseases (f-ILDs) represent a heterogeneous group of disorders in which the aetiology may be identified or, not infrequently, remain unknown. Establishing a correct diagnosis of a distinct f-ILD requires a multidisciplinary approach, integrating clinical profile, physiological and laboratory data, radiological appearance and, when appropriate, histological findings. Surgical lung biopsy is still considered the most important diagnostic tool as it is able to provide lung samples large enough for identification of complex patterns such as usual interstitial pneumonitis (UIP) and nonspecific interstitial pneumonitis. However, this procedure is accompanied by significant morbidity and mortality. Bronchoalveolar lavage is still a popular diagnostic tool allowing identification of alternative diagnoses in patients with suspected idiopathic pulmonary fibrosis (IPF) when an increase in lymphocytes is detected. Conventional transbronchial lung biopsy has a very low sensitivity in detecting the UIP pattern and its role in this clinical-radiological context is marginal. The introduction of less invasive methods such as transbronchial cryobiopsy show great promise to clinical practice as they can be used to obtain samples large enough to morphologically support a diagnosis of IPF or other idiopathic interstitial pneumonias, along with fewer complications. Recent advances in the field suggest that less invasive methods of lung sampling, without significant side effects, in combination with other diagnostic methods could replace the need for surgical lung biopsy in the future. Indeed, these new multidisciplinary procedures may become the main diagnostic work-up method for patients with suspected idiopathic interstitial pneumonia. PMID:26682637

  18. Idiopathic Retroperitoneal Fibrosis.

    PubMed

    Vaglio, Augusto; Maritati, Federica

    2016-07-01

    Idiopathic retroperitoneal fibrosis (RPF), reviewed herein, is a rare fibro-inflammatory disease that develops around the abdominal aorta and the iliac arteries, and spreads into the adjacent retroperitoneum, where it frequently causes ureteral obstruction and renal failure. The clinical phenotype of RPF is complex, because it can be associated with fibro-inflammatory disorders involving other organs, is considered part of the spectrum of IgG4-related disease, and often arises in patients with other autoimmune conditions. Obstructive uropathy is the most common complication, although other types of renal involvement may occur, including stenosis of the renal arteries and veins, renal atrophy, and different types of associated GN. Environmental and genetic factors contribute to disease susceptibility, whereas the immunopathogenesis of RPF is mediated by different immune cell types that eventually promote fibroblast activation. The diagnosis is made on the basis of computed tomography or magnetic resonance imaging, and positron emission tomography is a useful tool in disease staging and follow-up. Treatment of idiopathic RPF aims at relieving ureteral obstruction and inducing disease regression, and includes the use of glucocorticoids, combined or not with other traditional immunosuppressants. However, biologic therapies such as the B cell-depleting agent rituximab are emerging as potentially efficacious agents in difficult-to-treat cases. PMID:26860343

  19. [Thoracic duct collaterals of lymphatic and pulmonary origin. Anatomy and chylothorax after pulmonary surgery].

    PubMed

    Riquet, M; Hidden, G; Debesse, B

    1989-01-01

    Dye injection of lung segments reveals the existence of lymphatic drainage of the lungs generally into cervical venous confluents and more rarely into the arch of the thoracic duct in the neck and also occasionally into the thoracic duct in the mediastinum. Direct drainage of the lymph into the thoracic duct was observed in 10 cases out of a series of 589 injections of lung segments in adult cadavers. In one half of cases, the thoracic duct was injected from the left suprabronchial lymph node chain, the origin of the left recurrent chain, and in one quarter of cases from the lateral anteroposterior right major azygos and left azygo-aortic lymph node chains, not recognised by the classical authors. More rarely, direct lymphatic collaterals drained certain segments of the lower lobes into the thoracic duct via the triangular ligament. Analysis of cases of chylothorax occurring after lung resection and observed in the authors' department or in the literature reveals that most of them can be attributed to a chyle leak from one of these pulmonary lymph collaterals. These pathways are probably also involved in the development of medical or idiopathic chylothorax. PMID:2686514

  20. Idiopathic laryngotracheal stenosis.

    PubMed

    Costantino, Christina L; Mathisen, Douglas J

    2016-03-01

    Idiopathic laryngotracheal stenosis (ILTS) is a rare inflammatory disease of unknown etiology. Infectious, traumatic and immunologic processes must first be excluded. The majority of patients affected are female who present with progressive symptoms of upper airway obstruction, which can extend over a number of years. ILTS is characterized by short segment, circumferential stenotic lesions, located particularly at the level of the cricoid. Bronchoscopic evaluation is essential for establishing the diagnosis and operative planning. Various temporizing interventions have historically been utilized, including dilation and laser ablation, for symptomatic management. However these interventions have demonstrated diminishing returns and poor long-term outcomes. Patients with ILTS should be considered early for definitive surgical intervention to minimize complications and optimize outcomes. Laryngotracheal resection and reconstruction is a viable intervention, which has demonstrated good long-term results and low recurrence rates for this patient population. PMID:26981272

  1. Chronic Idiopathic Thrombocytogenic Purpura

    PubMed Central

    Pineo, G. F.

    1984-01-01

    Chronic idiopathic thrombocytopenic purpura (ITP) is a relatively common cause of an acquired hemostatic defect. It is important for family physicians to recognize this disorder, because of its insidious onset and the fact that it most commonly affects women of childbearing age. Chronic ITP is due to an antibody in the plasma which attaches to platelets and leads to their destruction in the reticuloendothelial system. The antibody can cross the placenta and affect the fetus. Although the condition may not disappear, in the vast majority of patients it can be controlled with current therapy, including prednisone, splenectomy and immunosuppressive agents. Although the mortality rate is low, patients with severe thrombocytopenia may have significant bleeding problems requiring special measures such as platelet transfusions, intravenous gammaglobulin, plasmapheresis and emergency splenectomy. Upon diagnosis, these patients should be referred to a large, specialized centre. PMID:21279099

  2. Idiopathic laryngotracheal stenosis

    PubMed Central

    Costantino, Christina L.

    2016-01-01

    Idiopathic laryngotracheal stenosis (ILTS) is a rare inflammatory disease of unknown etiology. Infectious, traumatic and immunologic processes must first be excluded. The majority of patients affected are female who present with progressive symptoms of upper airway obstruction, which can extend over a number of years. ILTS is characterized by short segment, circumferential stenotic lesions, located particularly at the level of the cricoid. Bronchoscopic evaluation is essential for establishing the diagnosis and operative planning. Various temporizing interventions have historically been utilized, including dilation and laser ablation, for symptomatic management. However these interventions have demonstrated diminishing returns and poor long-term outcomes. Patients with ILTS should be considered early for definitive surgical intervention to minimize complications and optimize outcomes. Laryngotracheal resection and reconstruction is a viable intervention, which has demonstrated good long-term results and low recurrence rates for this patient population. PMID:26981272

  3. Idiopathic Pulmonary Fibrosis.

    PubMed

    Martin, Maria D; Chung, Jonathan H; Kanne, Jeffrey P

    2016-05-01

    Idiopathic pulmonary fibrosis (IPF) is the most common fibrosing lung disease and is associated with a very poor prognosis. IPF manifests histopathologically as usual interstitial pneumonia (UIP) and as subpleural and basal predominant reticulation with honeycombing on high-resolution computed tomography (HRCT) of the chest. When a high-confidence radiologic diagnosis of UIP is made on HRCT, surgical biopsy is rarely required. Therefore, radiologists should recognize a UIP pattern on HRCT as well as recognize other patterns of fibrosing lung disease such as nonspecific interstitial pneumonia or chronic hypersensitivity pneumonitis, both of which can be mistaken for UIP. This article reviews the clinical, CT, and histopathologic features of IPF, discusses the impact of CT findings on prognosis, and describes complications associated with IPF. PMID:27043425

  4. The idiopathic hypereosinophilic syndrome.

    PubMed Central

    Alfaham, M A; Ferguson, S D; Sihra, B; Davies, J

    1987-01-01

    A 14 year old girl with idiopathic hypereosinophilic syndrome is described. In addition to weight loss, anaemia, amenorrhoea, general lethargy, anorexia, mouth ulcers, blisters of hands and feet, and petechial skin rash, she had features of involvement of the cardiovascular system as the major complication. She responded well to treatment. After a comprehensive search of the published reports 18 cases of this syndrome were identified in children under 16 years. Fifteen of these children had involvement of the cardiovascular system as the major source of their morbidity and mortality. Summary of the clinical details and laboratory, biopsy, and necropsy findings of the involvement of the various organ systems of the 18 children is presented. PMID:3619478

  5. Adolescent idiopathic scoliosis.

    PubMed

    Cheng, Jack C; Castelein, René M; Chu, Winnie C; Danielsson, Aina J; Dobbs, Matthew B; Grivas, Theodoros B; Gurnett, Christina A; Luk, Keith D; Moreau, Alain; Newton, Peter O; Stokes, Ian A; Weinstein, Stuart L; Burwell, R Geoffrey

    2015-01-01

    Adolescent idiopathic scoliosis (AIS) is the most common form of structural spinal deformities that have a radiological lateral Cobb angle - a measure of spinal curvature - of ≥10(°). AIS affects between 1% and 4% of adolescents in the early stages of puberty and is more common in young women than in young men. The condition occurs in otherwise healthy individuals and currently has no recognizable cause. In the past few decades, considerable progress has been made towards understanding the clinical patterns and the three-dimensional pathoanatomy of AIS. Advances in biomechanics and technology and their clinical application, supported by limited evidence-based research, have led to improvements in the safety and outcomes of surgical and non-surgical treatments. However, the definite aetiology and aetiopathogenetic mechanisms that underlie AIS are still unclear. Thus, at present, both the prevention of AIS and the treatment of its direct underlying cause are not possible. PMID:27188385

  6. Juvenile idiopathic arthritis

    PubMed Central

    Bhatt, Krupa H; Karjodkar, Freny R; Sansare, Kaustubh; Patil, Darshana

    2014-01-01

    Juvenile Idiopathic Arthritis (JIA) is the most chronic musculoskeletal disease of pediatric population. The chronic course of disease has a great impact on oral health. Temporomandibular joint is involved in JIA causing limited mouth opening with progressive open bite, retrognathia, microgenia and bird like appearance. Joints of upper and lower extremities are also involved. Effect on upper limb function leads to difficulty with fine motor movements required for brushing and flossing. This increases incidence of caries and periodontal disease in children. The cause of JIA is still poorly understood and none of the available drugs for JIA can cure the disease. However, prognosis has improved as a result of progress in disease classification and management. The dental practitioner should be familiar with the symptoms and oral manifestations of JIA to help manage as multidisciplinary management is essential. PMID:24808703

  7. Idiopathic Flushing with Dysesthesia

    PubMed Central

    Fogelman, Joshua P.; Ashinoff, Robin; Soter, Nicholas A.

    2015-01-01

    Objective: The purpose of this study was to analyze the efficacy and safety of the 585nm pulsed dye laser for the treatment of idiopathic flushing with dysesthesia. Design: This was a retrospective study of patients treated with a 585nm pulsed dye laser with fluences ranging from 3.5 to 7.5J/cm2 (purpura threshold fluences), a pulse duration of 450μsec, and a spot size of 5 or 10mm. Setting: The Ronald 0. Perelman Department of Dermatology at New York University Medical Center. Participants: Ten adult subjects who presented with flushing with dysesthesia. Measurements: Participants subjectively evaluated the decrease in dysesthesia and the number of flushing episodes. The objective response to treatment was evaluated by a single physician using pre- and postoperative photographs. The severity of postoperative erythema was compared with baseline using an ordinal scale ranging from zero (resolution of erythema) to four (76-100% of baseline erythema). Results: The mean number of treatments received by the subjects was seven. The mean fluence was 6.66J/cm2. Subjectively, 100 percent of subjects reported a decrease in dysethesia and the number of flushing episodes. Objectively, subjects demonstrated at least a 62.5-percent reduction in erythema. Conclusion: Laser surgery provided subjective relief of dysesthesia and decreased the number of flushing episodes with a greater than 62-percent objective reduction in the severity of erythema. The 585nm pulsed dye laser is a safe, efficacious treatment for the signs and symptoms of idiopathic flushing with dysesthesia. PMID:26345489

  8. Non-intubated thoracic surgery—A survey from the European Society of Thoracic Surgeons

    PubMed Central

    Sorge, Roberto; Akopov, Andrej; Congregado, Miguel; Grodzki, Tomasz

    2015-01-01

    Background A survey amongst the European Society of Thoracic Surgeons (ESTS) members has been performed to investigate the currents trends, rates of adoption as well as potential for future expansion of non-intubated thoracic surgery (NITS) performed under spontaneous ventilation. Methods A 14-question-based questionnaire has been e-mailed to ESTS members. To facilitate the completion of the questionnaire, questions entailed either quantitative or multiple-choice answers. Investigated issues included previous experience with NITS and number of procedures performed, preferred types of anesthesia protocols (i.e., thoracic epidural anesthesia, intercostal or paravertebral blocks, laryngeal mask, use of additional sedation), type of procedures, ideal candidates for NITS, main advantages and technical disadvantages. Non-univocal answer to multiple-choice questions was permitted. Results Out of 105 responders, 62 reported an experience with NITS. The preferred types of anesthesia were intercostal blocks with (59%) or without (50%) sedation, followed by laryngeal mask with sedation (43%) and thoracic epidural anesthesia with sedation (20%). The most frequently performed procedures included thoracoscopic management of recurrent pleural effusion (98%), pleural decortication for empyema thoracis and lung biopsy for interstitial lung disease (26% each); pericardial window and mediastinal biopsy (20% each). More complex procedures such as lobectomy, lung volume reduction surgery and thymectomy have been performed by a minority of responders (2% each). Poor-risk patients due to co-morbidities (70%) and patients with poor pulmonary function (43%) were considered the ideal candidates. Main advantages included faster, recovery (67%), reduced morbidity (59%) and shorter hospital stay with decreased costs (43% each). Reported technical disadvantages included coughing (59%) and poor maneuverability due to diaphragmatic and lung movements (56%). Overall, 69% of responders indicated

  9. Symptomatic Thoracic Spinal Cord Herniation: Case Series and Technical Report

    PubMed Central

    Hawasli, Ammar H.; Ray, Wilson Z.; Wright, Neill M.

    2014-01-01

    Background and Importance Idiopathic spinal cord herniation (ISCH) is an uncommon condition located predominantly in the thoracic spine and often associated with a remote history of a major traumatic injury. ISCH has an incompletely described presentation and unknown etiology. There is no consensus on treatment algorithm and surgical technique, and there is little data on clinical outcomes. Clinical Presentation In this case series and technical report, we describe the atypical myelopathy presentation, remote history of traumatic injury, radiographic progression, treatment, and outcomes of 5 patients treated at Washington University for symptomatic ISCH. A video showing surgical repair is presented. In contrast to classic compressive myelopathy symptomology, ISCH patients presented with an atypical myelopathy, characterized by asymmetric motor and sensory deficits and early-onset urinary incontinence. Clinical deterioration correlated with progressive spinal cord displacement and herniation observed on yearly spinal imaging in a patient imaged serially due to multiple sclerosis. Finally compared to compressive myelopathy in the thoracic spine, surgical treatment of ISH led to rapid improvement despite long duration of symptoms. Conclusion Symptomatic ISCH presents with atypical myelopathy and slow temporal progression and can be successfully managed with surgical repair. PMID:24871148

  10. Thoracic Radiculopathy due to Rare Causes

    PubMed Central

    2016-01-01

    Thoracic radiculopathy represents an uncommon spinal disorder that is frequently overlooked in the evaluation of thoracic, or abdominal pain syndrome. The clinical representation of this uncommon disorder is often atypical. With many differential diagnoses to consider, it is not surprising that the cause of thoracic radiculopathy is often not discovered for months, or years, after the symptoms arise. We report two rare cases of thoracic radiculopathy; one case was caused by extraskeletal Ewing sarcoma (EES) along the thoracic paraspinal area, and the other by foraminal stenosis, due to a bony spur of the thoracic vertebra. As such, thoracic radiculopathy should be considered in the diagnosis of patients with thoracic and abdominal pain, especially if initial diagnostic studies are inconclusive. PMID:27446792

  11. [Thoracic endometriosis and catamenial pneumothorax].

    PubMed

    Voskresenskiĭ, O V; Smoliar, A N; Damirov, M M; Galankina, I E; Zhelev, I G

    2014-01-01

    It was analyzed own experience of diagnosis and treatment of catamenial (menstrual) pneumothorax and thoracic endometriosis and literature review. It is shown that catamenial pneumothorax has specific clinical and instrumental signs allowing to establish the diagnosis before surgery. It was proposed surgical treatment including the removal of trans diaphragmatic way of pneumothorax development, removal of thoracic endometriosis and the establishment of reliable pleurodesis. It was demonstrated that this volume of surgery can be successfully implemented by using of thoracoscopic access. Relapse prevention includes hormonal therapy for the 6 months after surgery under the supervision of an obstetrician-gynecologist. PMID:25484144

  12. Idiopathic pulmonary fibrosis in a Christmas Island nuclear test veteran.

    PubMed

    Parfrey, H; Babar, J; Fiddler, C A; Chilvers, E R

    2010-01-01

    We describe the case of a 71-year-old man with idiopathic pulmonary fibrosis (usual interstitial pneumonia (UIP) pattern) diagnosed on clinical, radiological and lung function criteria, in accordance with the American Thoracic Society/European Respiratory Society consensus criteria (2000), who had been in close proximity to three atmospheric nuclear bomb blasts during military service in 1957. He does not have clubbing and clinically and radiologically his lung disease is stable. He also has bladder carcinoma and carotid arteriosclerosis, both recognised consequences of radiation injury. This is the first reported case of UIP in a nuclear test veteran. Awareness of this potential association is important given the current attempts of the British Nuclear Test Veterans Association to gain compensation for claimed injuries. PMID:22797205

  13. Idiopathic pulmonary fibrosis in a Christmas Island nuclear test veteran

    PubMed Central

    Parfrey, H; Babar, J; Fiddler, CA; Chilvers, ER

    2010-01-01

    We describe the case of a 71-year-old man with idiopathic pulmonary fibrosis (usual interstitial pneumonia (UIP) pattern) diagnosed on clinical, radiological and lung function criteria, in accordance with the American Thoracic Society/European Respiratory Society consensus criteria (2000), who had been in close proximity to three atmospheric nuclear bomb blasts during military service in 1957. He does not have clubbing and clinically and radiologically his lung disease is stable. He also has bladder carcinoma and carotid arteriosclerosis, both recognised consequences of radiation injury. This is the first reported case of UIP in a nuclear test veteran. Awareness of this potential association is important given the current attempts of the British Nuclear Test Veterans Association to gain compensation for claimed injuries. PMID:22797205

  14. [A case of thoracic actinomycosis].

    PubMed

    Denisova, O A; Cherniavskaia, G M; Beloborodova, É I; Topol'nitskiĭ, E B; Iakimenko, Iu V; Chernogoriuk, G É; Beloborodova, E V; Strezh, Iu A; Vil'danova, L R

    2014-01-01

    A case of thoracic actinomycosis manifest as round shadow in the lung is described. Diagnosis was based on the presence of actinomycetes in a transthoracic lung biopsy sample. Treatment for 3 months resulted in recovery. No relapse was documented during 1 year follow-up period. PMID:25265662

  15. How Is Idiopathic Pulmonary Fibrosis Treated?

    MedlinePlus

    ... the NHLBI on Twitter. How Is Idiopathic Pulmonary Fibrosis Treated? Doctors may prescribe medicines, oxygen therapy , pulmonary ... PR), and lung transplant to treat idiopathic pulmonary fibrosis (IPF). Medicines Currently, no medicines are proven to ...

  16. Idiopathic pulmonary arterial hypertension.

    PubMed

    Souza, Rogerio; Jardim, Carlos; Humbert, Marc

    2013-10-01

    Idiopathic pulmonary arterial hypertension (IPAH), formerly called primary pulmonary hypertension, is a rare disease (incidence and prevalence rates of approximately one and six cases per million inhabitants, respectively) with different clinical phenotypes. A group of diverse conditions manifest pulmonary arterial hypertension (PAH) and share similar pathological and/or clinical findings with IPAH. By definition, IPAH is diagnosed only after alternative diagnoses have been ruled out. Extensive investigation is needed to determine if PAH is associated with thyroid diseases, infectious diseases, autoimmune conditions, exposure to certain drugs (particularly anorexigens), certain genetic mutations, and so on. The presence of genetic abnormalities and risk factors (such as specific drug exposures) reinforces the "multiple hit" concept for the development of pulmonary hypertension. Fortunately, within the past two decades, therapeutic options have become available for IPAH, resulting in improved survival and clinical outcomes. At least seven different compounds have been registered for PAH treatment. However, even with aggressive PAH-specific therapy, mortality rates remain high (∼40% at 5 years). Given the high mortality rates, the use of combinations of agents that work by different pathways has been advocated (either as "add-on" therapy or initial "up front" therapy). Further, new therapeutic agents and treatment strategies are on the near horizon, aiming to further improve survival from the remarkable progress already seen. PMID:24037625

  17. Idiopathic Inflammatory Myopathies

    PubMed Central

    Barohn, Richard J.; Amato, Anthony

    2014-01-01

    The idiopathic inflammatory myopathies (IIM) consist of rare heterogenous autoimmune disorders that present with marked proximal and symmetric muscle weakness, except for distal and asymmetric weakness in inclusion body myositis (IBM). Besides frequent creatine kinase (CK) elevation, the electromyogram confirms the presence of an irritative myopathy. Extramuscular involvement affects a significant number of cases with interstitial lung disease (ILD), cutaneous in dermatomyositis (DM), systemic or joint manifestations and increased risk of malignancy especially in DM. Myositis specific autoantibodies influence phenotype of the IIM. Jo-1 antibodies are frequently associated with ILD and the newly described HMG-CoA reductase antibodies are characteristic of autoimmune necrotizing myopathy (NM). Muscle pathology ranges from inflammatory exudates of variable distribution, to intact muscle fiber invasion, necrosis, phagocytosis and in the case of IBM rimmed vacuoles and protein deposits. Despite many similarities, the IIM are a quite heterogeneous from the histopathological and pathogenetic standpoints in addition to some clinical and treatment-response difference. The field has witnessed significant advances in our understanding of pathophysiology and treatment of these rare disorders. In this review, we focus on DM, polymyositis (PM) and NM and examine current and promising therapies. The reader interested in more details on IBM is referred to the corresponding chapter in this issue. PMID:25037081

  18. Idiopathic inflammatory myositis.

    PubMed

    Tieu, Joanna; Lundberg, Ingrid E; Limaye, Vidya

    2016-02-01

    Knowledge on idiopathic inflammatory myopathy (IIM) has evolved with the identification of myositis-associated and myositis-specific antibodies, development of histopathological classification and the recognition of how these correlate with clinical phenotype and response to therapy. In this paper, we outline key advances in diagnosis and histopathology, including the more recent identification of antibodies associated with immune-mediated necrotising myopathy (IMNM) and inclusion body myositis (IBM). Ongoing longitudinal observational cohorts allow further classification of these patients with IIM, their predicted clinical course and response to specific therapies. Registries have been developed worldwide for this purpose. A challenging aspect in IIM, a multisystem disease with multiple clinical subtypes, has been defining disease status and clinically relevant improvement. Tools for assessing activity and damage are now recognised to be important in determining disease activity and guiding therapeutic decision-making. The International Myositis Assessment and Clinical Studies (IMACS) group has developed such tools for use in research and clinical settings. There is limited evidence for specific treatment strategies in IIM. With significant development in the understanding of IIM and improved classification, longitudinal observational cohorts and trials using validated outcome measures are necessary, to provide important information for evidence-based care in the clinical setting. PMID:27421222

  19. Endovascular Repair of Thoracic Aortic Aneurysms

    PubMed Central

    Findeiss, Laura K.; Cody, Michael E.

    2011-01-01

    Degenerative aneurysms of the thoracic aorta are increasing in prevalence; open repair of descending thoracic aortic aneurysms is associated with high rates of morbidity and mortality. Repair of isolated descending thoracic aortic aneurysms using stent grafts was introduced in 1995, and in an anatomically suitable subgroup of patients with thoracic aortic aneurysm, repair with endovascular stent graft provides favorable outcomes, with decreased perioperative morbidity and mortality relative to open repair. The cornerstones of successful thoracic endovascular aneurysm repair are appropriate patient selection, thorough preprocedural planning, and cautious procedural execution, the elements of which are discussed here. PMID:22379281

  20. Strategies to treat thoracic aortitis and infected aortic grafts.

    PubMed

    Kahlberg, A; Melissano, G; Tshomba, Y; Leopardi, M; Chiesa, R

    2015-04-01

    Infectious thoracic aortitis is a rare disease, especially since the incidence of syphilis and tuberculosis has dropped in western countries. However, the risk to develop an infectious aortitis and subsequent mycotic aneurysm formation is still present, particularly in case of associated endocarditis, sepsis, and in immunosuppressive disorders. Moreover, the number of surgical and endovascular thoracic aortic repairs is continuously increasing, and infective graft complications are observed more frequently. Several etiopathogenetic factors may play a role in thoracic aortic and prosthetic infections, including hematogenous seeding, local bacterial translocation, and iatrogenous contamination. Also, fistulization of the esophagus or the bronchial tree is commonly associated with these diseases, and it represents a critical event requiring a multidisciplinary management. Knowledge on underlying micro-organisms, antibiotic efficacy, risk factors, and prevention strategies has a key role in the management of this spectrum of infectious diseases involving the thoracic aorta. When the diagnosis of a mycotic aneurysm or a prosthetic graft infection is established, treatment is demanding, often including a number of surgical options. Patients are usually severely compromised by sepsis, and in most cases they are considered unfit for surgery for general clinical conditions or local concerns. Thus, results of different therapeutic strategies for infectious diseases of the thoracic aorta are still burdened with very high morbidity and mortality. In this manuscript, we review the literature regarding the main issues related to thoracic infectious aortitis and aortic graft infections, and we report our personal series of patients surgically treated at our institution for these conditions from 1993 to 2014. PMID:25608572

  1. Idiopathic Bilateral External Jugular Vein Thrombosis

    PubMed Central

    Hindi, Zakaria; Fadel, Ehab

    2015-01-01

    Patient: Male, 21 Final Diagnosis: Idiopathic bilateral external jugular vein thrombosis Symptoms: Face engorgement • neck swelling Medication: — Clinical Procedure: None Specialty: Hematology Objective: Unknown ethiology Background: Vein thrombosis is mainly determined by 3 factors, which constitute a triad called Virchow’s triad: hypercoagulability, stasis, and endothelial injury. Venous thrombosis commonly occurs in the lower extremities since most of the blood resides there and flows against gravity. The veins of the lower extremities are dependent on intact valves and fully functional leg muscles. However, in case of valvular incompetency or muscular weakness, thrombosis and blood stasis will occur as a result. In contrast, the veins of the neck, specially the jugulars, have distensible walls which allow flexibility during respiration. In addition, the blood directly flows downward towards the heart. Nevertheless, many case reports mentioned the thrombosis of internal jugular veins and external jugular veins with identified risk factors. Jugular vein thrombosis has previously been associated in the literature with a variety of medical conditions, including malignancy. Case Report: This report is of a case of idiopathic bilateral external jugular vein thrombosis in a 21 year-old male construction worker of Southeast Asian origin with no previous medical history who presented with bilateral facial puffiness of gradual onset over 1 month. Doppler ultrasound and computed tomography were used in the diagnosis. Further work-up showed no evidence of infection or neoplasia. The patient was eventually discharged on warfarin. The patient was assessed after 6 months and his symptoms had resolved completely. Conclusions: Bilateral idiopathic external jugular veins thrombosis is extremely rare and can be an indicator of early malignancy or hidden infection. While previous reports in the literature have associated jugular vein thrombosis with malignancy, the present

  2. Torsional deformity of apical vertebra in adolescent idiopathic scoliosis.

    PubMed

    Kotwicki, Tomasz; Napiontek, Marek

    2002-01-01

    CT scans of structural thoracic idiopathic scoliosis were reviewed in nine patients admitted to our department for scoliosis surgery. The apical vertebra scans were chosen and the following parameters were evaluated: 1) alpha angle formed by the axis of vertebra and the axis of spinous process 2) beta concave and beta convex angle between the spinous process and the left and right transverse process, respectively, 3) gamma concave and gamma convex angle between the axis of vertebra and the left and right transverse process, respectively, 4) the rotation angle to the sagittal plane. The constant deviation of the spinous process towards the convex side of the curve was observed. The vertebral body itself was distorted towards the concavity of the curve. The angle between the spinous process and the transverse process was smaller on the convex side of the curve. The torsional, intravertebral deformity of the apical vertebra was a factor acting in the direction opposite to the rotation, in the sense to reduce the deformity of the spine in idiopathic scoliosis. PMID:15456062

  3. Idiopathic Sporadic Onychomadesis of Toenails

    PubMed Central

    Nitayavardhana, Sunatra

    2016-01-01

    Onychomadesis is a clinical sign of nail plate separation due to transient or permanent arrest of nail matrix activities. Onychomadesis can be considered as a severe form of Beau's line. This condition usually occurs after trauma, causal diseases, or medications, yet it rarely occurs as an idiopathic condition. We report a case of a 38-year-old Thai female who developed recurrence onychomadesis in several toenails in the absence of predisposing factors or associated conditions. To the best of our knowledge, our patient is the first reported case of idiopathic onychomadesis limited to toenails. PMID:27437152

  4. Diffuse idiopathic skeletal hyperostosis of the spine in a nine-year-old cat.

    PubMed

    Bossens, K; Bhatti, S; Van Soens, I; Gielen, I; Van Ham, L

    2016-01-01

    A nine-year-old intact female domestic shorthair cat was evaluated for paraparesis, ataxia and severe spinal hyperaesthesia. Neurological examination indicated a T3-L3 spinal cord segment lesion. Computed tomography of the thoracolumbar and lumbosacral vertebral column was performed. This showed contiguous smooth new bone formation ventral and lateral to the vertebrae extending from the cranial thoracic area to the lumbosacral junction and appearing similar to canine diffuse idiopathic skeletal hyperostosis. There was also marked dorsolateral stenosis of the vertebral canal at the level of T4-T5 because of degenerative changes of the facet joints. To the authors' knowledge, this is the first published report of feline diffuse idiopathic skeletal hyperostosis. PMID:26011748

  5. Reoperation for thoracic outlet syndrome.

    PubMed

    Sessions, R T

    1989-01-01

    The clinical history and operative findings in a group of 60 patients who underwent reoperation for thoracic outlet syndrome (TOS) are presented. The patients were severely disabled by arm, shoulder, and neck pain and presented with physical findings pointing to scar fixation of the brachial plexus in the neck (upper tract recurrence) or at the thoracic outlet (lower tract recurrence). The causes of recurrence of TOS as discovered at operation are outlined. Basic principles governing the surgical management of recurrent TOS are elimination of the known causes of recurrence, thorough neurolysis of the brachial plexus, and coverage of the nerves with healthy fat. The role of an expanded PTFE surgical membrane (Gortex) as an adjunct to prevent recurrent scarring is discussed. The surgeon who operates on patients with recurrent TOS must be capable of managing the potential intraoperative complications of severe nerve injury and life threatening bleeding. PMID:2745532

  6. Nanotechnology applications in thoracic surgery.

    PubMed

    Hofferberth, Sophie C; Grinstaff, Mark W; Colson, Yolonda L

    2016-07-01

    Nanotechnology is an emerging, rapidly evolving field with the potential to significantly impact care across the full spectrum of cancer therapy. Of note, several recent nanotechnological advances show particular promise to improve outcomes for thoracic surgical patients. A variety of nanotechnologies are described that offer possible solutions to existing challenges encountered in the detection, diagnosis and treatment of lung cancer. Nanotechnology-based imaging platforms have the ability to improve the surgical care of patients with thoracic malignancies through technological advances in intraoperative tumour localization, lymph node mapping and accuracy of tumour resection. Moreover, nanotechnology is poised to revolutionize adjuvant lung cancer therapy. Common chemotherapeutic drugs, such as paclitaxel, docetaxel and doxorubicin, are being formulated using various nanotechnologies to improve drug delivery, whereas nanoparticle (NP)-based imaging technologies can monitor the tumour microenvironment and facilitate molecularly targeted lung cancer therapy. Although early nanotechnology-based delivery systems show promise, the next frontier in lung cancer therapy is the development of 'theranostic' multifunctional NPs capable of integrating diagnosis, drug monitoring, tumour targeting and controlled drug release into various unifying platforms. This article provides an overview of key existing and emerging nanotechnology platforms that may find clinical application in thoracic surgery in the near future. PMID:26843431

  7. Use of a Vertical Expandable Prosthetic Titanium Rib in Children With Thoracic Insufficiency Syndrome and Scoliosis.

    PubMed

    Watts, Stephanie L

    2016-04-01

    Thoracic insufficiency syndrome is the inability of the thorax to support normal respiration or lung growth. One treatment to address the thoracic deformities associated with the syndrome is placement of a vertical expandable prosthetic titanium rib. The goal is to restore thoracic volume, symmetry, and function and to allow for growth of the thorax in skeletally immature children. The main topics include indications for placement of a titanium rib, postoperative nursing care, potential complications, long-term follow-up, quality-of-life issues, and implications for critical care nurses and advanced practice nurses. (Critical Care Nurse.2016;36[2]:52-61). PMID:27037339

  8. Idiopathic CD4 Lymphocytopenia

    PubMed Central

    Régent, Alexis; Autran, Brigitte; Carcelain, Guislaine; Cheynier, Rémi; Terrier, Benjamin; Charmeteau-De Muylder, Bénédicte; Krivitzky, Alain; Oksenhendler, Eric; Costedoat-Chalumeau, Nathalie; Hubert, Pascale; Lortholary, Olivier; Dupin, Nicolas; Debré, Patrice; Guillevin, Loïc; Mouthon, Luc

    2014-01-01

    Abstract Idiopathic CD4 T lymphocytopenia (ICL) is a rare and severe condition with limited available data. We conducted a French multicenter study to analyze the clinical and immunologic characteristics of a cohort of patients with ICL according to the Centers for Disease Control criteria. We recruited 40 patients (24 female) of mean age 44.2 ± 12.2 (19–70) years. Patients underwent T-lymphocyte phenotyping and lymphoproliferation assay at diagnosis, and experiments related to thymic function and interferon (IFN)-γ release by natural killer (NK) cell were performed. Mean follow-up was 6.9 ± 6.7 (0.14–24.3) years. Infectious, autoimmune, and neoplastic events were recorded, as were outcomes of interleukin 2 therapy. In all, 25 patients had opportunistic infections (12 with human papillomavirus infection), 14 had autoimmune symptoms, 5 had malignancies, and 8 had mild or no symptoms. At the time of diagnosis, the mean cell counts were as follows: mean CD4 cell count: 127/mm3 (range, 4–294); mean CD8: 236/mm3 (range, 1–1293); mean CD19: 113/mm3 (range, 3–547); and mean NK cell count: 122/mm3 (range, 5–416). Most patients had deficiency in CD8, CD19, and/or NK cells. Cytotoxic function of NK cells was normal, and patients with infections had a significantly lower NK cell count than those without (p = 0.01). Patients with autoimmune manifestations had increased CD8 T-cell count. Proliferation of thymic precursors, as assessed by T-cell rearrangement excision circles, was increased. Six patients died (15%). CD4 T-cell count <150/mm3 and NK cell count <100/mm3 were predictors of death. In conclusion, ICL is a heterogeneous disorder often associated with deficiencies in CD8, CD19, and/or NK cells. Long-term prognosis may be related to initial CD4 and NK cell deficiency. PMID:24646462

  9. Reversal of childhood idiopathic scoliosis in an adult, without surgery: a case report and literature review

    PubMed Central

    2009-01-01

    Background Some patients with mild or moderate thoracic scoliosis (Cobb angle <50-60 degrees) suffer disproportionate impairment of pulmonary function associated with deformities in the sagittal plane and reduced flexibility of the spine and chest cage. Long-term improvement in the clinical signs and symptoms of childhood onset scoliosis in an adult, without surgical intervention, has not been documented previously. Case presentation A diagnosis of thoracic scoliosis (Cobb angle 45 degrees) with pectus excavatum and thoracic hypokyphosis in a female patient (DOB 9/17/52) was made in June 1964. Immediate spinal fusion was strongly recommended, but the patient elected a daily home exercise program taught during a 6-week period of training by a physical therapist. This regime was carried out through 1992, with daily aerobic exercise added in 1974. The Cobb angle of the primary thoracic curvature remained unchanged. Ongoing clinical symptoms included dyspnea at rest and recurrent respiratory infections. A period of multimodal treatment with clinical monitoring and treatment by an osteopathic physician was initiated when the patient was 40 years old. This included deep tissue massage (1992-1996); outpatient psychological therapy (1992-1993); a daily home exercise program focused on mobilization of the chest wall (1992-2005); and manipulative medicine (1994-1995, 1999-2000). Progressive improvement in chest wall excursion, increased thoracic kyphosis, and resolution of long-standing respiratory symptoms occurred concomitant with a >10 degree decrease in Cobb angle magnitude of the primary thoracic curvature. Conclusion This report documents improved chest wall function and resolution of respiratory symptoms in response to nonsurgical approaches in an adult female, diagnosed at age eleven years with idiopathic scoliosis. PMID:20003501

  10. Effects of the Schroth exercise on the Cobb's angle and vital capacity of patients with idiopathic scoliosis that is an operative indication.

    PubMed

    Kim, Kyoung-Don; Hwangbo, Pil-Neo

    2016-03-01

    [Purpose] The purpose of this study was to investigate the effects of the Schroth exercise on the Cobb's angle and vital capacity of patients with growing idiopathic scoliosis, an operative indication. [Subjects] Five idiopathic scoliosis patients with a Cobb's angle of the thoracic vertebra of 40 degrees or higher and Risser sign stage 3 or higher. [Methods] The Schroth exercise was applied 3 times a week for 12 weeks. We measured the thoracic trunk inclination, Cobb's angle, and vital capacity before and after the exercise program. [Results] The thoracic trunk rotation angle decreased from 11.86 ± 3.32° to 4.90 ± 1.91° on average, the thoracic Cobb's angle decreased from 42.40 ± 7.86° to 26.0 ± 3.65° on average, and the vital capacity also increased from 2.83 ± 1.23° to 4.04° ± 1.67° on average. All these effects were significant. [Conclusion] The 12-week Schroth exercise caused significant effects in the thoracic trunk inclination, Cobb's angle, and vital capacity. The conservative treatment method was found to be effective even at a 40 degree or higher Cobb's angle. In the future, universal exercise approach methods and preventive training for the treatment of scoliosis should be developed further. PMID:27134385

  11. Vertebral Body Stapling versus Bracing for Patients with High-Risk Moderate Idiopathic Scoliosis

    PubMed Central

    Cuddihy, Laury; Danielsson, Aina J.; Cahill, Patrick J.; Samdani, Amer F.; Grewal, Harsh; Richmond, John M.; Mulcahey, M. J.; Gaughan, John P.; Antonacci, M. Darryl; Betz, Randal R.

    2015-01-01

    Purpose. We report a comparison study of vertebral body stapling (VBS) versus a matched bracing cohort for immature patients with moderate (25 to 44°) idiopathic scoliosis (IS). Methods. 42 of 49 consecutive patients (86%) with IS were treated with VBS and followed for a minimum of 2 years. They were compared to 121 braced patients meeting identical inclusion criteria. 52 patients (66 curves) were matched according to age at start of treatment (10.6 years versus 11.1 years, resp. [P = 0.07]) and gender. Results. For thoracic curves 25–34°, VBS had a success rate (defined as curve progression <10°) of 81% versus 61% for bracing (P = 0.16). In thoracic curves 35–44°, VBS and bracing both had a poor success rate. For lumbar curves, success rates were similar in both groups for curves measuring 25–34°. Conclusion. In this comparison of two cohorts of patients with high-risk (Risser 0-1) moderate IS (25–44°), in smaller thoracic curves (25–34°) VBS provided better results as a clinical trend as compared to bracing. VBS was found not to be effective for thoracic curves ≥35°. For lumbar curves measuring 25–34°, results appear to be similar for both VBS and bracing, at 80% success. PMID:26618169

  12. Thoracic spine sports-related injuries.

    PubMed

    Menzer, Heather; Gill, G Keith; Paterson, Andrew

    2015-01-01

    Although sports-related injuries to the thoracic spine are relatively uncommon, they are among the most feared due to the potential for catastrophic neurologic injury. The increased biomechanical support of the thoracic spine makes injuries in this region particularly rare compared with the cervical and lumbar spine. As a result, thoracic spine injuries can be missed easily, difficult to diagnose, and problematic to treat. Recognition of mechanism and awareness of injury patterns help physicians determine a diagnosis and create an index of suspicion for unstable thoracic spine injuries. Aggressive full-contact sports receive the most attention for spinal injury; however several sports with repetitive loading of the spine can cause severe injuries, including rowing, gymnastics, and golf. The goal of this article was to provide an overview of the unique anatomic and biomechanical features of the thoracic spine and to discuss some of the more common thoracic injuries that can affect athletes. PMID:25574880

  13. TEVAR: Endovascular Repair of the Thoracic Aorta

    PubMed Central

    Nation, David A.; Wang, Grace J.

    2015-01-01

    The development of thoracic endovascular aortic repair (TEVAR) has allowed a minimally invasive approach for management of an array of thoracic aortic pathologies. Initially developed specifically for exclusion of thoracic aortic aneurysms, TEVAR is now used as an alternative to open surgery for a variety of disease pathologies due to the lower morbidity of this approach. Advances in endograft technology continue to broaden the applications of this technique. PMID:26327745

  14. Thoracic Endometriosis Syndrome: A Veritable Pandora’s Box

    PubMed Central

    Nayar, Jayashree

    2016-01-01

    Thoracic endometriosis syndrome is a rare disorder characterised by the presence of functioning endometrial tissue in pleura, lung parenchyma, airways, and/or encompasses mainly four clinical entities–catamenial pneumothorax, catamenial haemothorax, catamenial haemoptysis and lung nodules. The cases were studied retrospectively by reviewing the records at Amrita Institute of Medical Sciences, for duration of five years i.e., form March 2010-2014 and analysed for the clinical presentation and management of thoracic endometriosis syndrome. Catamenial breathlessness was the main symptom. Pneumothorax and pleural effusion were the findings on investigations. Histopathology report of endometriosis was present in three cases (50%). Conditions with excess oestrogen like endometriosis, fibroid, adenomyosis were diagnosed in these patients by pelvic scan. After the initial supportive treatment with hormones, pleurodesis, hysterectomy and lung decortication were the treatment modalities. Two cases that had multiple recurrences were diagnosed as disseminated TES. They underwent combined treatment of surgery and hormones. PMID:27190904

  15. Practical genetics of thoracic aortic aneurysm.

    PubMed

    Elefteriades, John A; Pomianowski, Pawel

    2013-01-01

    This chapter will provide a practical look at the rapidly evolving field regarding the genetics of thoracic aortic aneurysm. It will start with a look at the history of the genetics of thoracic aortic aneurysm and will then move on to elucidating the discovery of familial patterns of thoracic aortic aneurysm. We will next review the Mendelian genetics of transmission of thoracic aortic aneurysm. We will move on to the molecular genetics at the DNA level and finish with a discussion of the molecular genetics at the RNA level, including a promising investigational "RNA Signature" test that we have been developing at Yale. PMID:23993238

  16. Traumatic injury of the thoracic duct.

    PubMed

    Guzman, A E; Rossi, L; Witte, C L; Smyth, S

    2002-03-01

    Injuries to the thoracic duct are infrequent but may become life-threatening when chylous leakage persists. This report describes 6 patients with such injuries in whom the leakage resolved spontaneously in one, was corrected using microsurgical lymphatic repair or lymphatic-venous anastomosis in two, successfully treated either by ligation of the thoracic duct or insertion of a peritoneovenous shunt in two, and was eventually controlled after bilateral pleurodesis and thoracic duct ligation by insertion of a peritoneo-venous shunt in one. Conventional lymphography is superior to lymphoscintigraphy and is usually required to document disruption of the thoracic duct. PMID:11939572

  17. Subacromial space in adult patients with thoracic hyperkyphosis and in healthy volunteers.

    PubMed

    Gumina, Stefano; Di Giorgio, Giantony; Postacchini, Franco; Postacchini, Roberto

    2008-02-01

    The assumption that subacromial space decreases in patients with thoracic hyperkyphosis arises from sporadic and personal observations. The purpose of this study was to compare width of subacromial space calculated on radiographs and CT scans of a high number of patients with thoracic hyperkyphosis that registered on exams of healthy volunteers. We measured the subacromial space, using Petersson's method, on radiographs of 47 patients with idiopathic or acquired thoracic hyperkyphosis and of 175 healthy shoulder volunteers. Both groups were further distinguished considering gender and age. Females with hyperkyphosis were also divided in two subgroups: those with a kyphotic curve of less (24 patients) or more (19 patients) than 50 degrees , respectively. Subacromial space of all patients and of 21 volunteers was also evaluated using CT. Acromio-humeral space was less wide in patients with hyperkyphosis with respect to coeval volunteers of the same gender; in females and in subjects older than 60. Subacromial width of females with hyperkyphosis whose curve was more than 50 degrees was significantly narrower (p<0.05) than that measured on radiograms or CT scans of females with a less severe spinal deformity. Decrease of subacromial space may be attributed to less posterior tilting of the scapula and to dyskinesis of the scapular movement. Scapular malposition causes an anomalous orientation of the acromion that may contribute to subacromial impingement. Patients with thoracic hyperkyphosis greater than 50 degrees had a subacromial space narrower than that measured in patients with a less severe kyphosis. This suggests that subacromial width is directly related to severity of thoracic kyphosis. Because hyperkyphosis of patients with osteoporotic vertebral fractures may worsen over the time, subacromial decompression could give only temporary shoulder pain relief. PMID:18320381

  18. [Completion pneumonectomy combined with graft replacement of thoracic aortic aneurysm by simple clamping].

    PubMed

    Shimamoto, A; Takao, M; Kanemitsu, S; Fujinaga, K; Yan, G; Cruz, B P; Onoda, K; Shimono, T; Shimpo, H; Namikawa, S; Yuasa, H; Yada, I

    1999-01-01

    A 59-years-old male patient who had left upper lobe partial resection 30 years ago. He was seen at the family physician because of cough. A chest X-ray was showing an abnormal mass shadow measuring 3 x 4 cm in left lower lobe like honey comb. And squamous cell carcinoma (SCC) was detected in his sputum. He was diagnosed as primary lung cancer and introduced to our department to have operation. Chest CT-scan was showing lung tumor suspected SCC measuring 4.3 x 2.6 cm in segment 8 faced chest wall. At the same time, we detected thoracic aortic aneurysm and subcarinal lymph node, but could not see where the boundary is, so it was hard to distinguish between parietal thrombus with thoracic aortic aneurysm and swelling subcarinal lymph node. We decided it swelling subcarinal lymph node by three-dimensional treated CT-scan. Aortic angiography was showing proximal descending aortic aneurysm measuring diameter was 4.5 cm. Abdominal CT-scan was showing infrarenal abdominal aortic aneurysm measuring diameter was 5.5 cm. He was diagnosed as primary lung cancer (It. S8, SCC) (cT2N2M0, Stage IIIB), thoracic aortic aneurysm, abdominal aortic aneurysm, and idiopathic pulmonary fibrosis, and had completion pneumonectomy (R 2 b) for primary lung cancer and graft replacement with aneurysm dissection for thoracic aortic aneurysm without extracorporeal circulation. In this operation, we could find swelling subcarinal lymph node measuring 5 x 3 cm instead of parietal thrombus with thoracic aortic aneurysm. Pathological examination diagnosed middle differential SCC and no metastasis from dissected lymph node (PT2N0M0, Stage I A). PMID:10024802

  19. Comparative Analysis of Interval, Skipped, and Key-vertebral Pedicle Screw Strategies for Correction in Patients With Lenke Type 1 Adolescent Idiopathic Scoliosis.

    PubMed

    Wang, Fei; Xu, Xi-Ming; Lu, Yanghu; Wei, Xian-Zhao; Zhu, Xiao-Dong; Li, Ming

    2016-03-01

    Pedicle screw constructs have become the mainstay for surgical correction in patients with spinal deformities. To reduce or avoid the risk of pedicle screw-based complications and to decrease the costs associated with pedicle screw instrumentation, some authors have introduced interval, skipped, and key-vertebral pedicle screw strategies for correction. However, there have been no comparisons of outcomes among these 3 pedicle screw-placement strategies.The aim of this study was to compare the correlative clinical outcomes of posterior correction and fusion with pedicle screw fixation using these 3 surgical strategies.Fifty-six consecutive patients with Lenke type 1 adolescent idiopathic scoliosis were included in this study. Twenty patients were treated with the interval pedicle screw strategy (IPSS), 20 with the skipped pedicle screw strategy (SPSS), and 16 with the key-vertebral pedicle screw strategy (KVPSS). Coronal and sagittal radiographs were analyzed before surgery, at 1 week after surgery, and at the last follow-up after surgery.There were no significant differences among the 3 groups regarding preoperative radiographic parameters. No significant difference was found between the IPSS and SPSS groups in correction of the main thoracic curve (70.8% vs 70.0%; P = 0.524). However, there were statistically significant differences between the IPSS and KVPSS groups (70.8% vs 64.9%) and between the SPSS and KVPSS groups (70.0% vs 64.9%) in correction of the main thoracic curve (P < 0.001 for both). Additionally, there were no significant differences among the 3 strategies for sagittal parameters at the immediate postoperative and last postoperative follow-up periods, though there were significant differences in the Cobb angle between the preoperative and immediate postoperative periods among the 3 groups, but not between the immediate postoperative and last follow-up periods. The amount of hospital charges in the SPSS group was significantly higher than

  20. Possibilities of scar treatment after thoracic surgery.

    PubMed

    Maragakis, M; Willital, G H; Michel, G; Görtelmeyer, R

    1995-01-01

    During a ten year observation period it was found that scar formation after thoracic surgery is influenced by various factors: metabolism, operative technique and factors of a general nature. On the basis of these findings, a study was carried out to investigate the effect of the scar-specific Contractubex gel (Merz+Co., D-Frankfurt/Main), containing 10% onion extract, 50 U sodium heparin per g of gel and 1% allantoin, in the treatment of children who underwent thoracic surgery and to evaluate its effect on scar development. Before and during the six-month treatment period, both macromorphology and scar colour were assessed; furthermore, a global evaluation of the therapeutic result was made. Additionally, the scars were characterized after a six-month treatment-free follow-up period. The results of 38 Contractubex-treated and 27 untreated patients were compared. In the treated scars, the global evaluation of the therapeutic result was better than in the untreated scars. In the Contractubex group, the rating was "good" and "very good" in 84% of cases, as compared to 59% of the untreated cases. In the treated group, the increase in scar size was markedly lower than in the untreated patients. The treated scars showed a tendency towards quicker paling than the untreated scars. In the treated group, the conversion of primary physiological scars to unphysiological scars (hypertrophic or keloidal scars) was less frequent than in the untreated group. The tolerability of the product was very good in 37 of the 38 treated patients, and good in one patient. All scar-specific effects of Contractubex continued to persist after the end of treatment. PMID:8846750

  1. Perceptions, experiences and needs of patients with idiopathic pulmonary fibrosis

    PubMed Central

    Duck, Annette; Spencer, Lisa G; Bailey, Simon; Leonard, Colm; Ormes, Jennifer; Caress, Ann-Louise

    2015-01-01

    Aims To understand the perceptions, needs and experiences of patients with Idiopathic Pulmonary Fibrosis. Background Idiopathic pulmonary fibrosis is a progressive interstitial lung disease, with a mean life expectancy similar to some forms of cancer of 2–4 years from diagnosis. Unlike the cancer literature, which is rich with studies exploring the needs of their disease group, few publications exist on patient needs with this severe fibrotic lung disease. Design A Qualitative study which took place between 2007–2012. Methods Seventeen patients with a multidisciplinary team confirmed diagnosis of Idiopathic Pulmonary Fibrosis, with moderate to advanced disease severity and six of their informal carers were interviewed. An interview topic guide was developed by the researchers and service user group. The interviews were audio-recorded, semi-structured and took place at a regional respiratory and lung transplant centre in North West England. Interviews were transcribed verbatim and data analysed using Framework Analysis. Findings Three main themes were identified: ‘Struggling to get a diagnosis’; ‘Loss of the life I previously had’; and ‘Living with Idiopathic Pulmonary Fibrosis’. Patients reported struggling to get a diagnosis and coping with a life-limiting, rapidly progressive illness with no good treatment and few support structures. Conclusions There is an urgent need for a better understanding of the difficulties faced by people with Idiopathic Pulmonary Fibrosis and their carers. This can be used to develop better supportive care in the United Kingdom and ultimately improve the quality of life of these patients. PMID:25533573

  2. Genetics and Idiopathic Interstitial Pneumonias.

    PubMed

    Chu, Sarah G; El-Chemaly, Souheil; Rosas, Ivan O

    2016-06-01

    Significant progress has been made in elucidating the genetics of parenchymal lung diseases, particularly idiopathic interstitial pneumonias (IIPs). IIPs are a heterogeneous group of diffuse interstitial lung diseases of uncertain etiology, diagnosed only after known causes of interstitial lung disease have been excluded. Idiopathic pulmonary fibrosis is the most common IIP. Through candidate gene approaches and genome wide association studies, much light has been shed on the genetic origins of IIPs, enhancing our understanding of risk factors and pathogenesis. However, significant work remains to be accomplished in identifying novel genetic variants and characterizing the function of validated candidate genes in lung pathobiology, their interplay with environmental factors, and ultimately translating these discoveries to patient care. PMID:27231858

  3. Treatment of Idiopathic Membranous Nephropathy

    PubMed Central

    Austin, Howard A.

    2012-01-01

    Exciting progress recently has been made in our understanding of idiopathic membranous nephropathy, as well as treatment of this disease. Here, we review important advances regarding the pathogenesis of membranous nephropathy. We will also review the current approach to treatment and its limitations and will highlight new therapies that are currently being explored for this disease including Rituximab, mycophenolate mofetil, and adrenocorticotropic hormone, with an emphasis on results of the most recent clinical trials. PMID:22859855

  4. Evolution of thoracic surgery in Canada

    PubMed Central

    Deslauriers, Jean; Griffith Pearson, F; Nelems, Bill

    2015-01-01

    BACKGROUND: Canada’s contributions toward the 21st century’s practice of thoracic surgery have been both unique and multilayered. Scattered throughout are tales of pioneers where none had gone before, where opportunities were greeted by creativity and where iconic figures followed one another. OBJECTIVE: To describe the numerous and important achievements of Canadian thoracic surgeons in the areas of surgery for pulmonary tuberculosis, thoracic oncology, airway surgery and lung transplantation. METHOD: Information was collected through reading of the numerous publications written by Canadian thoracic surgeons over the past 100 years, interviews with interested people from all thoracic surgery divisions across Canada and review of pertinent material form the archives of several Canadian hospitals and universities. RESULTS: Many of the developments occurred by chance. It was the early and specific focus on thoracic surgery, to the exclusion of cardiac and general surgery, that distinguishes the Canadian experience, a model that is now emerging everywhere. From lung transplantation in chimera twin calves to ex vivo organ preservation, from the removal of airways to tissue regeneration, and from intensive care research to complex science, Canadians have excelled in their commitment to research. Over the years, the influence of Canadian thoracic surgery on international practice has been significant. CONCLUSIONS: Canada spearheaded the development of thoracic surgery over the past 100 years to a greater degree than any other country. From research to education, from national infrastructures to the regionalization of local practices, it happened in Canada.

  5. The Thoracic Shape of Hominoids

    PubMed Central

    Chan, Lap Ki

    2014-01-01

    In hominoids, the broad thorax has been assumed to contribute to their dorsal scapular position. However, the dorsoventral diameter of their cranial thorax was found in one study to be longer in hominoids. There are insufficient data on thoracic shape to explain the relationship between broad thorax and dorsal scapular position. The current study presents data on multilevel cross-sectional shape and volume distribution in a range of primates. Biplanar radiographs of intact fluid-preserved cadavers were taken to measure the cross-sectional shape of ten equally spaced levels through the sternum (called decisternal levels) and the relative volume of the nine intervening thoracic segments. It was found that the cranial thorax of hominoids is larger and broader (except in the first two decisternal levels) than that of other primates. The cranial thorax of hominoids has a longer dorsoventral diameter because the increase in dorsoventral diameter caused by the increase in the volume of the cranial thorax overcompensates for the decrease caused by the broadening of the cranial thorax. The larger and broader cranial thorax in hominoids can be explained as a locomotor adaptation for scapular gliding and as a respiratory adaptation for reducing the effects of orthograde posture on ventilation-perfusion inequality. PMID:24818026

  6. Thoracic organ transplantation: laboratory methods.

    PubMed

    Patel, Jignesh K; Kobashigawa, Jon A

    2013-01-01

    Although great progress has been achieved in thoracic organ transplantation through the development of effective immunosuppression, there is still significant risk of rejection during the early post-transplant period, creating a need for routine monitoring for both acute antibody and cellular mediated rejection. The currently available multiplexed, microbead assays utilizing solubilized HLA antigens afford the capability of sensitive detection and identification of HLA and non-HLA specific antibodies. These assays are being used to assess the relative strength of donor specific antibodies; to permit performance of virtual crossmatches which can reduce the waiting time to transplantation; to monitor antibody levels during desensitization; and for heart transplants to monitor antibodies post-transplant. For cell mediated immune responses, the recent development of gene expression profiling has allowed noninvasive monitoring of heart transplant recipients yielding predictive values for acute cellular rejection. T cell immune monitoring in heart and lung transplant recipients has allowed individual tailoring of immunosuppression, particularly to minimize risk of infection. While the current antibody and cellular laboratory techniques have enhanced the ability to manage thoracic organ transplant recipients, future developments from improved understanding of microchimerism and graft tolerance may allow more refined allograft monitoring techniques. PMID:23775735

  7. The thoracic shape of hominoids.

    PubMed

    Chan, Lap Ki

    2014-01-01

    In hominoids, the broad thorax has been assumed to contribute to their dorsal scapular position. However, the dorsoventral diameter of their cranial thorax was found in one study to be longer in hominoids. There are insufficient data on thoracic shape to explain the relationship between broad thorax and dorsal scapular position. The current study presents data on multilevel cross-sectional shape and volume distribution in a range of primates. Biplanar radiographs of intact fluid-preserved cadavers were taken to measure the cross-sectional shape of ten equally spaced levels through the sternum (called decisternal levels) and the relative volume of the nine intervening thoracic segments. It was found that the cranial thorax of hominoids is larger and broader (except in the first two decisternal levels) than that of other primates. The cranial thorax of hominoids has a longer dorsoventral diameter because the increase in dorsoventral diameter caused by the increase in the volume of the cranial thorax overcompensates for the decrease caused by the broadening of the cranial thorax. The larger and broader cranial thorax in hominoids can be explained as a locomotor adaptation for scapular gliding and as a respiratory adaptation for reducing the effects of orthograde posture on ventilation-perfusion inequality. PMID:24818026

  8. Bilateral internal thoracic artery grafting

    PubMed Central

    2013-01-01

    The effectiveness of the left internal mammary artery graft to the anterior descending coronary artery as a surgical strategy has been shown to improve the survival rate and decrease the risk of adverse cardiac events in patients undergoing coronary bypass surgery. These clinical benefits appear to be related to the superior short and long-term patency rates of the internal thoracic artery graft. Although the advantages of using of both internal thoracic arteries (ITA) for bypass grafting have taken longer to prove, recent results from multiple data sets now support these findings. The major advantage of bilateral ITA grafting appears to be improved survival rate, while the disadvantages of complex ITA grafting include the increased complexity of operation, and an increased risk of wound complications. While these short-term disadvantages have been mitigated in contemporary surgical practice, they have not eliminated. Bilateral ITA grafting should be considered the procedure of choice for patients undergoing coronary bypass surgery that have a predicted survival rate of longer than ten years. PMID:23977627

  9. Evaluation of the effectiveness of thoracic sympathectomy in the treatment of primary hyperhidrosis of hands and armpits using the measurement of skin resistance

    PubMed Central

    Jabłoński, Sławomir; Rzepkowska-Misiak, Beata; Piskorz, Łukasz; Brocki, Marian; Wcisło, Szymon; Smigielski, Jacek; Kordiak, Jacek

    2012-01-01

    Introduction Hyperhidrosis is excessive sweating beyond the needs of thermoregulation. It is disease which mostly affects young people, often carrying a considerable amount of socio-economic implications. Thoracic sympathectomy is now considered to be the "gold standard" in the treatment of idiopathic hyperhidrosis of hands and armpits. Aim Assessment of early effectiveness of thoracic sympathectomy using skin resistance measurements performed before surgery and in the postoperative period. Material and methods A group of 20 patients with idiopathic excessive sweating of hands and the armpit was enrolled in the study. Patients underwent two-stage thoracic sympathectomy with resection of Th2-Th4 ganglions. The skin resistance measurements were made at six previously designated points on the day of surgery and the first day after the operation. Results In all operated patients we obtained complete remission of symptoms on the first day after the surgery. Inhibition of sweating was confirmed using the standard starch iodine (Minor) test. At all measurement points we obtained a statistically significant increase of skin resistance, assuming p < 0.05. To check whether there is a statistically significant difference in the results before and after surgery we used sequence pairs Wilcoxon test. Conclusions Thoracic sympathectomy is an effective curative treatment for primary hyperhidrosis of hands and armpits. Statistically significant increase of skin resistance in all cases is a good method of assessing the effectiveness of the above surgery in the early postoperative period. PMID:23256019

  10. From “awake” to “monitored anesthesia care” thoracic surgery: A 15 year evolution

    PubMed Central

    Mineo, Tommaso C; Tacconi, Federico

    2014-01-01

    Although general anesthesia still represents the standard when performing thoracic surgery, the interest toward alternative methods is increasing. These have evolved from the employ of just local or regional analgesia techniques in completely alert patients (awake thoracic surgery), to more complex protocols entailing conscious sedation and spontaneous ventilation. The main rationale of these methods is to prevent serious complications related to general anesthesia and selective ventilation, such as tracheobronchial injury, acute lung injury, and cardiovascular events. Trends toward shorter hospitalization and reduced overall costs have also been indicated in preliminary reports. Monitored anesthesia care in thoracic surgery can be successfully employed to manage diverse oncologic conditions, such as malignant pleural effusion, peripheral lung nodules, and mediastinal tumors. Main non-oncologic indications include pneumothorax, emphysema, pleural infections, and interstitial lung disease. Furthermore, as the familiarity with this surgical practice has increased, major operations are now being performed this way. Despite the absence of randomized controlled trials, there is preliminary evidence that monitored anesthesia care protocols in thoracic surgery may be beneficial in high-risk patients, with non-inferior efficacy when compared to standard operations under general anesthesia. Monitored anesthesia care in thoracic surgery should enter the armamentarium of modern thoracic surgeons, and adequate training should be scheduled in accredited residency programs. PMID:26766966

  11. The Idiopathic Intracranial Hypertension Treatment Trial

    PubMed Central

    Wall, Michael; Kupersmith, Mark J.; Kieburtz, Karl D.; Corbett, James J.; Feldon, Steven E.; Friedman, Deborah I.; Katz, David M.; Keltner, John L.; Schron, Eleanor B.; McDermott, Michael P.

    2015-01-01

    IMPORTANCE To our knowledge, there are no large prospective cohorts of untreated patients with idiopathic intracranial hypertension (IIH) to characterize the disease. OBJECTIVE To report the baseline clinical and laboratory features of patients enrolled in the Idiopathic Intracranial Hypertension Treatment Trial. DESIGN, SETTING, AND PARTICIPANTS We collected data at baseline from questionnaires, examinations, automated perimetry, and fundus photography grading. Patients (n = 165) were enrolled from March 17, 2010, to November 27, 2012, at 38 academic and private practice sites in North America. All participants met the modified Dandy criteria for IIH and had a perimetric mean deviation between −2 dB and −7 dB. All but 4 participants were women. MAIN OUTCOMES AND MEASURES Baseline and laboratory characteristics. RESULTS The mean (SD) age of our patients was 29.0 (7.4) years and 4 (2.4%) were men. The average (SD) body mass index (calculated as weight in kilograms divided by height in meters squared) was 39.9 (8.3). Headache was the most common symptom (84%). Transient visual obscurations occurred in 68% of patients, back pain in 53%, and pulse synchronous tinnitus in 52%. Only 32% reported visual loss. The average (SD) perimetric mean deviation in the worst eye was −3.5 (1.1) dB, (range, −2.0 to −6.4 dB) and in the best eye was −2.3 (1.1) dB (range, −5.2 to 0.8 dB). A partial arcuate visual field defect with an enlarged blind spot was the most common perimetric finding. Visual acuity was 85 letters or better (20/20) in 71% of the worst eyes and 77% of the best eyes. Quality of life measures, including the National Eye Institute Visual Function Questionnaire–25 and the Short Form–36 physical and mental health summary scales, were lower compared with population norms. CONCLUSIONS AND RELEVANCE The Idiopathic Intracranial Hypertension Treatment Trial represents the largest prospectively analyzed cohort of untreated patients with IIH. Our data show

  12. Effects of consecutive application of stretching, Schroth, and strengthening exercises on Cobb’s angle and the rib hump in an adult with idiopathic scoliosis

    PubMed Central

    Yang, Jae-Man; Lee, Jung-Hoon; Lee, Dae-Hee

    2015-01-01

    [Purpose] To report the effects of consecutive application of stretching, Schroth, and strengthening exercises in an adult with idiopathic scoliosis. [Subject] A 26-year-old woman with idiopathic scoliosis, Cobb’s angle of 20.51°, and back pain. [Methods] The exercise program consisted of 3 sessions: 10 minutes of stretching exercises, 20 minutes of Schroth exercises, and 10 minutes of strengthening exercises. This program was implemented 3 times a week, for 8 weeks. [Results] The thoracic Cobb’s angle decreased from 20.51° to 16.35°, and the rib hump decreased from 15° to 9°. [Conclusion] Consecutive application of stretching, Schroth, and strengthening exercises may help reduce Cobb’s angle and the rib hump in adults with idiopathic scoliosis. PMID:26355577

  13. [Idiopathic granulomatous mastitis treated with steroids and methotrexate].

    PubMed

    Peña-Santos, Genaro; Ruiz-Moreno, José Luis

    2011-06-01

    Idiopathic granulomatous mastitis is a rare inflammatory breast disease of unknown etiology. It manifests as breast mass of 6 cm on average (range 2-10 cm), often in upper outer quadrant of left breast, in another quadrant, right or bilateral breast. Clinical diagnosis by ultrasound or mammography and fine needle aspiration confuses with carcinoma; histopathology (gold standard) confirm the diagnosis after ruling out causes of granulomatous inflammation, mainly tuberculosis. Steroid treatment achieve complete remission, but adverse reactions and relapses after the descent and suspension. Methotrexate or azathioprine is added from the start to maintain remission. We report three cases of idiopathic granulomatous mastitis diagnosis and treatment based on prednisone until clinical improvement and methotrexate as maintenance therapy. Complete remission was obtained in three patients. The rheumatologist knows and handles autoimmune/inflammatory with these drugs, therefore, is suggested the multidisciplinary treatment of this disease with oncologists and gynecologists. PMID:21966829

  14. Familial Idiopathic Cranial Neuropathy in a Chinese Family.

    PubMed

    Zhang, Li; Liang, Jianfeng; Yu, Yanbing

    2016-01-01

    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. PMID:27161475

  15. [Thoracic actinomycosis versus bronchial cancer].

    PubMed

    Brombacher-Frey, I; Wöckel, W; Kreusser, T

    1992-01-01

    We report on 4 thoracic actinomycoses; in three of these four cases a bronchial carcinoma was suspected, and in case No. 2 this carcinoma had been considered to be in a very advanced and inoperable stage. A man of 51 years of age was in a generally run-down condition. He also noticed that his sputum was tinged with blood. The x-ray film showed a large space-occupying growth at the right lung hilus. Repeated perbronchial biopsies of the focus did not yield any diagnosis. Actinomycosis was identified histologically only in the tissue samples obtained via thoracotomy. After a three-month penicillin course the hilar shadow receded. A 61-year old male patient was transferred to our Pneumological Hospital, being strongly suspected of suffering from an extensive bronchial carcinoma, and having multiple intrathoracic space-occupying growths as well as pleural effusions, a pericardial effusion, and an infiltration of the left thoracic wall with fistula formation; however, histological examination of skin biopsies revealed that he was suffering from actinomycosis. Antibiotic therapy cured him completely in a six-month course. In a man of 32 years of age who had been indulging for many years in a severe abuse of nicotin, we suspected a central bronchial carcinoma on the basis of his x-ray, but histology of the tissue taken from the space-occupying growth via diagnostic thoracotomy revealed that this patient, too, suffered from actinomycosis. Complete recession occurred after several months of antibiotic treatment. A woman of 82 years had been an inpatient for several months in another hospital because of relapsing pleuropneumonias on the right side. She was transferred to us as an outpatient after a renewed relapse. We conducted a transcutaneous fine-needle biopsy of the right indurating pleural effusion. A few actinomyces filaments were seen on histological examination of the purulent exudate. Hence, actinomycosis was confirmed. After antibiotic therapy the finding receded

  16. [Preliminary study on the action of hypopressive gymnastics in the treatment of idiopathic scoliosis].

    PubMed

    Caufriez, Marcel; Fernández-Domínguez, Juan Carlos; Brynhildsvoll, Nils

    2011-01-01

    Our goal is to describe the results of a hypopressive gymnastics (HG) program applied to 3 children with idiopathic scoliosis. Three children (ages ranging from 8 to 15 years) suffering from idiopathic scoliosis were recruited for this study. Thoracic or thoracolumbar curves showed between 15° and 40° Cobb degrees. The evolutionary character of the curves had been confirmed. A study of different clinical and radiological parameters was carried out to compare the measurements before and after (3 months later) the application of a 5 HG daily exercises programme: A radiological study of the spine was carried out to compare the measurements of the vertebral rotation. A radiological study, a plumb line and a measuring tape were used to assess the vertebral tilt in this study. A Scoliometer was used to measure the deformation of the rib cage (gibbosity). The most significant results were: a trend to reach stabilisation in the vertebral tilt and rotation, and stabilisation of gibbosity, which probably might improve the respiratory function of these subjects. Finally, the performance of an ordinary HG exercise program shows a trend to control and stabilise dorsal idiopathic scoliosis progression. PMID:22019528

  17. Idiopathic pulmonary hemosiderosis in adults: a case report and review of the literature.

    PubMed

    Tzouvelekis, Argyris; Ntolios, Paschalis; Oikonomou, Anastasia; Koutsopoulos, Anastasios; Sivridis, Efthimios; Zacharis, George; Kaltsas, Kostantinos; Boglou, Panagiotis; Mikroulis, Dimitrios; Bouros, Demosthenes

    2012-01-01

    Idiopathic pulmonary hemosiderosis is a very rare condition rarely affecting adults and causing recurrent episodes of diffuse alveolar haemorrhage that may lead to lung fibrosis. Due to lack of pathognomonic findings, IPH diagnosis is established upon exclusion of all other possible causes of DAH in combination with specific pathologic findings revealing bland alveolar haemorrhage with absence of vasculitis and/or accumulation of immune complexes within lung parenchyma. Here we describe a rare case of idiopathic pulmonary hemosiderosis in an otherwise healthy 27-year-old Greek male patient with relapsing episodes of fever accompanied by general fatigue and discomfort. He was at this time point a light smoker and had been hospitalised once in the past for similar symptoms. His iron deficiency anemia coupled with chest high-resolution computed tomography and bronchoalveolar lavage revealed findings compatible with diffuse alveolar hemorrhage. After excluding all other sources of bleeding through extensive gastrointestinal workup and thorough immunologic profile, video-assisted thoracic lung biopsy was performed and the diagnosis of Idiopathic Pulmonary Hemosiderosis was established. Patient was treated with high doses of oral corticosteroids, leading to clinical response. We highlight the need for vigilance by the respiratory physician for the presence of DAH, a challenging, acute condition requiring early recognition along with identification of the underlying syndrome and appropriate treatment to achieve optimal results. PMID:22851975

  18. Influence of pelvic asymmetry and idiopathic scoliosis in adolescents on postural balance during sitting.

    PubMed

    Jung, Ji-Yong; Cha, Eun-Jong; Kim, Kyung-Ah; Won, Yonggwan; Bok, Soo-Kyung; Kim, Bong-Ok; Kim, Jung-Ja

    2015-01-01

    The effects of pelvic asymmetry and idiopathic scoliosis on postural balance during sitting were studied by measuring inclination angles, pressure distribution, and electromyography. Participants were classified into a control group, pelvic asymmetry group, scoliosis group, and scoliosis with pelvic asymmetry and then performed anterior, posterior, left, and right pelvic tilting while sitting on the unstable board for 5 seconds to assess their postural balance. Inclination and obliquity angles between the groups were measured by an accelerometer located on the unstable board. Pressure distribution (maximum force and peak pressure) was analyzed using a capacitive seat sensor. In addition, surface electrodes were attached to the abdominal and erector spinae muscles of each participant. Inclination and obliquity angles increased more asymmetrically in participants with both pelvic asymmetry and scoliosis than with pelvic asymmetry or scoliosis alone. Maximum forces and peak pressures of each group showed an asymmetrical pressure distribution caused by the difference in height between the left and right pelvis and curve type of the patients' spines when performing anterior, posterior, left, and right pelvic tilting while sitting. Muscle contraction patterns of external oblique, thoracic erector spinae, lumbar erector spinae, and lumbar multifidus muscles may be influenced by spine curve type and region of idiopathic scoliosis. Asymmetrical muscle activities were observed on the convex side of scoliotic patients and these muscle activity patterns were changed by the pelvic asymmetry. From these results, it was confirmed that pelvic asymmetry and idiopathic scoliosis cause postural asymmetry, unequal weight distribution, and muscular imbalance during sitting. PMID:26406054

  19. Thoracic Wall Reconstruction in Advanced Breast Tumours

    PubMed Central

    Daigeler, A.; Harati, K.; Goertz, O.; Hirsch, T.; Behr, B.; Lehnhardt, M.; Kolbenschlag, J.

    2014-01-01

    In advanced mammary tumours, extensive resections, sometimes involving sections of the thoracic wall, are often necessary. Plastic surgery reconstruction procedures offer sufficient opportunities to cover even large thoracic wall defects. Pedicled flaps from the torso but also free flap-plasties enable, through secure defect closure, the removal of large, ulcerated, painful or bleeding tumours with moderate donor site morbidity. The impact of thoracic wall resection on the respiratory mechanism can be easily compensated for and patientsʼ quality of life in the palliative stage of disease can often be improved. PMID:24976636

  20. Nintedanib in idiopathic pulmonary fibrosis.

    PubMed

    Woodcock, H V; Maher, T M

    2015-06-01

    Idiopathic pulmonary fibrosis (IPF) conveys a median survival of 3 years and until recently has lacked effective therapies. Nintedanib, an orally available, small-molecule tyrosine kinase inhibitor with selectivity for vascular endothelial growth factor (VEGF), platelet-derived growth factor (PDGF) and fibroblast growth factor (FGF) receptors has recently been shown, in two pivotal phase III studies, to effectively slow IPF disease progression. Consequently, nintedanib was given accelerated approval by the FDA in October 2014 for the treatment of IPF. This monograph explores the preclinical rationale for the antifibrotic role of nintedanib and provides an overview of the available data on pharmacokinetics, efficacy and safety. PMID:26261848

  1. [The Details of Thermoesthesia-and-Algesthesia State in Patients with Idiopathic Scoliosis of III-IV Degree].

    PubMed

    Shchurova, E N; Riabykh, S O; Kobyzev, A E; Ochirova, P V

    2016-01-01

    Sensitivity in patients with adolescent idiopathic scoliosis remains insufficiently studied. The details of thermoesthesia-and-algesthesia state were studied in adolescent patients with III-IV Degree idiopathic scoliosis. Thermoesthesia-and-algesthesia was evaluated in Th1-S1 dermatomes on the right and on the left using an electric esthesiometer. Thermoesthesia-and-algesthesia disorders of different severity degree have been established to be observed in all patients in all the dermatomes studied, manifesting themselves as hypesthesia, hyperesthesia, and thermoesthesia absence. The disorders of thermoesthesia-and-algesthesia were not of local character; but they went beyond the level of the spine deformity. Maximum negative changes in thermoesthesia-and-algesthesia were registered in the dermatomes corresponding to the apex of thoracic spine deformity (Th8, Th9, Th10) where the lowest percentage of patients with normal algesthesia thresholds is observed being combined with the decreased number of patients with thermoesthesia thresholds within the norm. PMID:27188152

  2. Management of a Left Internal Thoracic Artery Graft Injury during Left Thoracotomy for Thoracic Surgery.

    PubMed

    Oates, Matthew; Yadav, Sumit; Saxena, Pankaj

    2016-07-01

    There have been some recent reports on the surgical treatment of lung cancer in patients following previous coronary artery bypass graft surgery. Use of internal thoracic artery graft is a gold standard in cardiac surgery with superior long-term patency. Left internal thoracic artery graft is usually patent during left lung resection in patients who present to the surgeon with an operable lung cancer. We have presented our institutional experience with left-sided thoracic surgery in patients who have had previous coronary artery surgery with a patent internal thoracic artery graft. PMID:26907619

  3. Modern impact of video assisted thoracic surgery

    PubMed Central

    D’Amico, Thomas A.

    2014-01-01

    With advancement in technology, experience and training over the last two decades, video assisted thoracic surgery (VATS) has become widely accepted and utilized all over the world. VATS started as a diagnostic tool in the early 1990s, technique of VATS lobectomy evolved and became safer over the next 10-15 years and now it is being used for more advanced and hybrid operations. VATS has contributed to the development of minimally invasive surgical interventions for other thoracic disorders like mediastinal tumors and esophageal cancer as well. This article looks at the advantages of VATS, technique advancements and its applications in other thoracic operations and its influence on the present and future of thoracic surgery. PMID:25379201

  4. Thoracic and abdominal blastomycosis in a horse.

    PubMed

    Toribio, R E; Kohn, C W; Lawrence, A E; Hardy, J; Hutt, J A

    1999-05-01

    A 5-year-old Quarter Horse mare was examined because of lethargy, fever, and weight loss of 1 month's duration. Thoracic auscultation revealed decreased lung sounds cranioventrally. Thoracic ultrasonography revealed bilateral anechoic areas with hyperechoic strands, consistent with pleural effusion and fibrin tags. A large amount of free fluid was evident during abdominal ultrasonography. Abnormalities included anemia, hyperproteinemia, hyperglobulinemia, hyperfibrinogenemia, and hypoalbuminemia. Thoracic radiography revealed alveolar infiltrates in the cranial and caudoventral lung fields. A cavitary mass, consistent with an abscess, could be seen caudodorsal to the crura of the diaphragm. Ultrasonographic evaluation of this area revealed a hypoechoic mass with septations. Bilateral thoracocentesis was performed. Bacterial culture of the pleural fluid did not yield growth, but Blastomyces dermatitidis was isolated from pleural fluid, abdominal fluid, and an aspirate of the abscess. The mare was euthanatized, and a diagnosis of thoracic and abdominal blastomycosis was confirmed at necropsy. PMID:10319179

  5. Correlation between quantitative EEG and MRI in idiopathic generalized epilepsy.

    PubMed

    Betting, Luiz E; Li, Li M; Lopes-Cendes, Iscia; Guerreiro, Marilisa M; Guerreiro, Carlos A M; Cendes, Fernando

    2010-09-01

    The objective of this study was to investigate the relationship between the focal discharges sometimes observed in the electroencephalogram of patients with idiopathic generalized epilepsies and subtle structural magnetic resonance imaging abnormalities. The main hypothesis to be assessed is that focal discharges may arise from areas of structural abnormality which can be detected by quantitative neuroimaging. Focal discharges were used for quantitative electroencephalogram source detection. Neuroimaging investigations consisted of voxel-based morphometry and region of interest volumetry. For voxel-based morphometry, volumetric MRI were acquired and processed. The images of each patient were individually compared with a control group. Statistical analysis was used to detect differences in gray matter volumes. Region of interest-based morphometry was automatically performed and used essentially to confirm voxel-based morphometry findings. The localization of the focal discharges on the electroencephalogram was compared to the neuroimaging results. Twenty-two patients with idiopathic generalized epilepsies were evaluated. Gray matter abnormalities were detected by voxel-based morphometry analysis in 77% of the patients. There was a good concordance between EEG source detection and voxel-based morphometry. On average, the nearest voxels detected by these methods were 19 mm (mm) apart and the most statistically significant voxels were 34 mm apart. This study suggests that in some cases subtle gray matter abnormalities are associated with focal epileptiform discharges observed in the electroencephalograms of patients with idiopathic generalized epilepsies. PMID:20082332

  6. Axial length in unilateral idiopathic central serous chorioretinopathy

    PubMed Central

    Moon, Hoseok; Lee, Dae Yeong; Nam, Dong Heun

    2016-01-01

    AIM To evaluate the axial length (AXL) in unilateral idiopathic central serous chorioretinopathy (CSC). METHODS This retrospective case-control study was comprised of a consecutive case series of 35 patients with acute unilateral idiopathic CSC, and age- and sex-matched 50 control eyes. AXL of both eyes of unilateral CSC patients and the control eyes were investigated. AXL was measured by ultrasonic biometry, and the adjusted AXL was calculated for CSC eyes as measured AXL plus differences of foveal thickness between CSC and normal fellow eyes in millimeters. The main outcome measures were comparison of AXL between CSC, fellow and control eyes. RESULTS The mean age of 35 CSC patients was 45.5y, and 31 males were included. The adjusted AXL of CSC eyes was 23.52 mm, and the AXL of fellow eyes was 23.46 mm, and of control eyes 23.94 mm. The AXL of both CSC and fellow eyes were significantly shorter than control eyes (CSC vs control, P=0.044; fellow vs control, P=0.026). There was no statistically significant difference in AXL between CSC and fellow eyes. CONCLUSION In unilateral idiopathic CSC, the AXL of CSC and fellow eyes are shorter than that of control eyes. Short AXL may be related with choroidal circulation abnormality in CSC. PMID:27275428

  7. Idiopathic epilepsy and school achievement.

    PubMed

    Sturniolo, M G; Galletti, F

    1994-05-01

    Forty one children (20 boys, 21 girls) aged 6-10.8 years (mean age 8.6 years) who were affected with idiopathic epilepsy underwent neuropsychological (Wechsler Intelligence Scale for Children, Bender test) and behavioural assessment (Personality Inventory for Children; this was also used in a matched control group). Further information was obtained by teachers' reports. School underachievement occurred in 25 children (61%). Statistical analysis showed no influence of sex, social background, age of onset, seizure type, duration of illness, features seen on electroencephalography, and treatment. School failure was due to poor performance in almost all academic fields, and was associated with higher visuomotor impairment; children showing good school performance had a higher mean IQ and less visuomotor impairment. The behaviour of children with epilepsy who had a good academic performance did not differ from that of their healthy peers. Emotional maladjustment (social skill impairment, depression, poor motivation, and low self esteem) was associated with poor school performance. Such problems, that may complicate the course of idiopathic epilepsy and require an appropriate educational programme, should be carefully considered by the clinician. PMID:8017966

  8. Uniportal video-assisted thoracic (VATS) lobectomy.

    PubMed

    Sihoe, Alan D L

    2016-03-01

    Uniportal video assisted thoracic surgery (VATS) has become one of the most exciting new developments in minimally invasive thoracic surgery in recent years. While the debate over its purported advantages continues, this chapter instead focuses on the technical aspects of performing a lobectomy via the uniportal approach. Using clear medical illustrations to show how each step is performed, the key tips and tricks are laid out for the beginner hoping to learn the technique. PMID:27134841

  9. Uniportal video-assisted thoracic (VATS) lobectomy

    PubMed Central

    2016-01-01

    Uniportal video assisted thoracic surgery (VATS) has become one of the most exciting new developments in minimally invasive thoracic surgery in recent years. While the debate over its purported advantages continues, this chapter instead focuses on the technical aspects of performing a lobectomy via the uniportal approach. Using clear medical illustrations to show how each step is performed, the key tips and tricks are laid out for the beginner hoping to learn the technique. PMID:27134841

  10. Nonintubated anesthesia in thoracic surgery: general issues

    PubMed Central

    Castillo, Maria

    2015-01-01

    Anesthetic management for awake thoracic surgery (ATS) is more difficult than under general anesthesia (GA), being technically extremely challenging for the anesthesiologist. Therefore, thorough preparation and vigilance are paramount for successful patient management. In this review, important considerations of nonintubated anesthesia for thoracic surgery are discussed in view of careful patient selection, anesthetic preparation, potential perioperative difficulties and the management of its complications. PMID:26046051

  11. Hemoptysis as the Presenting Clinical Sign of a T8-T9 Spine Fracture with Diffuse Idiopathic Skeletal Hyperostosis Changes

    PubMed Central

    Pollina, John; Dimopoulos, Vassilios G.

    2016-01-01

    Diffuse idiopathic skeletal hyperostosis (DISH) is a noninflammatory degenerative disease that affects multiple spine levels and, in combination with osteoporosis, makes vertebrae more prone to fractures, especially in elderly people. We describe a rare case of thoracic fracture in an ankylosed spine in which hemoptysis was the only clinical sign. The patient (age in the early 80s) presented with chest pain and a cough associated with hemoptysis. The patient had no complaints of back pain and no neurological symptoms. Computed tomography (CT) angiography of the chest revealed changes consistent with DISH, with fractures at the T8 and T9 vertebra as well as lung hemorrhage or contusion in the right lung base. CT and magnetic resonance imaging of the thoracic spine showed similar findings, with a recent T8-T9 fracture and DISH changes. The patient underwent percutaneous pedicle screw fixation from T7 to T11 and remained neurologically intact with an uneventful postoperative course. PMID:27418984

  12. Exercise therapy for patients with diffuse idiopathic skeletal hyperostosis.

    PubMed

    Al-Herz, Adeeba; Snip, Jan Paul; Clark, Bruce; Esdaile, John M

    2008-02-01

    We evaluated the effect of exercise therapy on back pain, spinal range of motion (ROM), and disability in persons with diffuse idiopathic skeletal hyperostosis (DISH). Persons with symptomatic DISH received a daily exercise program for 24 weeks consisting of mobility, stretching, and strengthening exercises for the cervical, thoracic, and lumbar spine. It included 14 supervised sessions over 8 weeks. Outcomes included visual analogue scales (VAS) for pain, stiffness, and fatigue, 13 spinal measurements, the neck pain and disability scale, the Quebec back pain disability scale, the Bath Spondylitis Functional Index, and the MACTAR patient preference scale. Assessments were made at baseline, 8 weeks, and 24 weeks. Fifteen of 17 completed the study. Comparing week 24 with baseline, Schober's test improved significantly (p = 0.02), and VAS stiffness and left finger-to-floor test demonstrated a trend to improvement (p = 0.07 each). The physical measures, which were expected to improve with the exercise program, all moved in the direction expected, but had p values > 0.10. At 24 weeks, eight (53.3%) participants rated their status as improved, three (20%) as unchanged, and four (27%) were unsure about the benefit. The exercise program designed for DISH and tested in this study led to small improvements in physical measures which achieved significance only for lumbosacral flexion. PMID:17885726

  13. Cytomegalovirus Immunoglobulin After Thoracic Transplantation

    PubMed Central

    Grossi, Paolo; Mohacsi, Paul; Szabolcs, Zoltán; Potena, Luciano

    2016-01-01

    Abstract Cytomegalovirus (CMV) is a highly complex pathogen which, despite modern prophylactic regimens, continues to affect a high proportion of thoracic organ transplant recipients. The symptomatic manifestations of CMV infection are compounded by adverse indirect effects induced by the multiple immunomodulatory actions of CMV. These include a higher risk of acute rejection, cardiac allograft vasculopathy after heart transplantation, and potentially bronchiolitis obliterans syndrome in lung transplant recipients, with a greater propensity for opportunistic secondary infections. Prophylaxis for CMV using antiviral agents (typically oral valganciclovir or intravenous ganciclovir) is now almost universal, at least in high-risk transplants (D+/R−). Even with extended prophylactic regimens, however, challenges remain. The CMV events can still occur despite antiviral prophylaxis, including late-onset infection or recurrent disease, and patients with ganciclovir-resistant CMV infection or who are intolerant to antiviral therapy require alternative strategies. The CMV immunoglobulin (CMVIG) and antiviral agents have complementary modes of action. High-titer CMVIG preparations provide passive CMV-specific immunity but also exert complex immunomodulatory properties which augment the antiviral effect of antiviral agents and offer the potential to suppress the indirect effects of CMV infection. This supplement discusses the available data concerning the immunological and clinical effects of CMVIG after heart or lung transplantation. PMID:26900989

  14. Unexplained childhood anaemia: idiopathic pulmonary hemosiderosis.

    PubMed

    Siu, K K; Li, Rever; Lam, S Y

    2015-04-01

    This report demonstrates pulmonary haemorrhage as a differential cause of anaemia. Idiopathic pulmonary hemosiderosis is a rare disease in children; it is classically described as a triad of haemoptysis, pulmonary infiltrates on chest radiograph, and iron-deficiency anaemia. However, anaemia may be the only presenting feature of idiopathic pulmonary hemosiderosis in children due to occult pulmonary haemorrhage. In addition, the serum ferritin is falsely high in idiopathic pulmonary hemosiderosis which increases the diagnostic difficulty. We recommend that pulmonary haemorrhage be suspected in any child presenting with iron-deficiency anaemia and persistent bilateral pulmonary infiltrates. PMID:25904566

  15. MAINE POPULATION

    EPA Science Inventory

    MEPOP250 depicts Maine's 1950-1990 population data by town or Census in unorganized territories. Populations were compiled from US Census Bureau data where available or from Maine Municipal Information (mainly for older records). Unorganized towns with very low or zero pop...

  16. Cough in idiopathic pulmonary fibrosis.

    PubMed

    van Manen, Mirjam J G; Birring, Surinder S; Vancheri, Carlo; Cottin, Vincent; Renzoni, Elisabetta A; Russell, Anne-Marie; Wijsenbeek, Marlies S

    2016-09-01

    Many patients with idiopathic pulmonary fibrosis (IPF) complain of chronic refractory cough. Chronic cough is a distressing and disabling symptom with a major impact on quality of life. During recent years, progress has been made in gaining insight into the pathogenesis of cough in IPF, which is most probably "multifactorial" and influenced by mechanical, biochemical and neurosensory changes, with an important role for comorbidities as well. Clinical trials of cough treatment in IPF are emerging, and cough is increasingly included as a secondary end-point in trials assessing new compounds for IPF. It is important that such studies include adequate end-points to assess cough both objectively and subjectively. This article summarises the latest insights into chronic cough in IPF. It describes the different theories regarding the pathophysiology of cough, reviews the different methods to assess cough and deals with recent and future developments in the treatment of cough in IPF. PMID:27581827

  17. Antidepressants in chronic idiopathic urticaria.

    PubMed

    Yasharpour, Michelle R; Randhawa, Inderpal

    2011-01-01

    Chronic idiopathic urticaria (CIU) is a common disease estimated to affect 0.1% of the population and can be very difficult to treat. Many psychotropic medications have been reported to be successful in treating refractory CIU. The purpose of this article was to discuss the pathophysiology of chronic urticaria and provide practicing allergists and dermatologists alternative treatment options in the management of refractory CIU, especially in those who have concurrent psychiatric comorbidity. A review was performed of pertinent literature pertaining to the pathophysiology of CIU and the many psychotropic medications reportedly successful in disease management. Although more research is needed, this article serves to broaden the mind of the physician treating CIU. PMID:22221435

  18. Epidemiology of idiopathic pulmonary fibrosis

    PubMed Central

    Ley, Brett; Collard, Harold R

    2013-01-01

    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. PMID:24348069

  19. Epigenomics of idiopathic pulmonary fibrosis

    PubMed Central

    Yang, Ivana V

    2012-01-01

    Idiopathic pulmonary fibrosis (IPF) is a complex lung disease of unknown etiology. Development of IPF is influenced by both genetic and environmental factors. Gene-expression profiling studies have taught us quite a bit about the biology of this fatal disease, but epigenetic marks may be the missing link that connects the environmental exposure in genetically predisposed individuals to transcriptome changes associated with the development of IPF. This review will begin with an introduction to the disease, followed by brief summaries of studies of gene expression in IPF and epigenetic marks associated with exposures relevant to IPF. The majority of the discussion will focus on epigenetic studies conducted so far in IPF, the limitations, challenges and future directions in this field. PMID:22449190

  20. Molecular etiology of idiopathic cardiomyopathy

    PubMed Central

    Arimura, T; Hayashi, T; Kimura, A

    2007-01-01

    Summary Idiopathic cardiomyopathy (ICM) is a primary cardiac disorder associated with abnormalities of ventricular wall thickness, size of ventricular cavity, contraction, relaxation, conduction and rhythm. Over the past two decades, molecular genetic analyses have revealed that mutations in the various genes cause ICM and such information concerning the genetic basis of ICM enables us to speculate the pathogenesis of this heterogeous cardiac disease. This review focuses on the molecular pathogenesis, i.e., genetic abnormalities and functional alterations due to the mutations especially in sarcomere/cytoskeletal components, in three characteristic features of ICM, hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM) and restrictive cardiomyopathy (RCM). Understanding the functional abnormalities of the sarcomere/cytoskeletal components, in ICM, has unraveled the function of these components not only as a contractile unit but also as a pivot for transduction of biochemical signals. PMID:18646564

  1. Genetics Home Reference: idiopathic pulmonary fibrosis

    MedlinePlus

    ... However, the course of the disease is highly variable; some affected people become seriously ill within a ... idiopathic pulmonary fibrosis: an observational cohort study with independent validation. Lancet Respir Med. 2014 Jul;2(7): ...

  2. Diagnosis and classification of idiopathic inflammatory myopathies.

    PubMed

    Lundberg, I E; Miller, F W; Tjärnlund, A; Bottai, M

    2016-07-01

    The idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of diseases, collectively termed myositis, sharing symptoms of muscle weakness, fatigue and inflammation. Other organs are frequently involved, supporting the notion that these are systemic inflammatory diseases. The IIMs can be subgrouped into dermatomyositis, polymyositis and inclusion body myositis. The myositis-specific autoantibodies (MSAs) identify other and often more distinct clinical phenotypes, such as the antisynthetase syndrome with antisynthetase autoantibodies and frequent interstitial lung disease and anti-SRP and anti-HMGCR autoantibodies that identify necrotizing myopathy. The MSAs are important both to support myositis diagnosis and to identify subgroups with different patterns of extramuscular organ involvement such as interstitial lung disease. Another cornerstone in the diagnostic procedure is muscle biopsy to identify inflammation and to exclude noninflammatory myopathies. Treatment effect and prognosis vary by subgroup. To develop new and better therapies, validated classification criteria that identify distinct subgroups of myositis are critical. The lack of such criteria was the main rationale for the development of new classification criteria for IIMs, which are summarized in this review; the historical background regarding previous diagnostic and classification criteria is also reviewed. As the IIMs are rare diseases with a prevalence of 10 in 100 000 individuals, an international collaboration was essential, as was the interdisciplinary effort including experts in adult and paediatric rheumatology, neurology, dermatology and epidemiology. The new criteria have been developed based on data from more than 1500 patients from 47 centres worldwide and are based on clinically easily available variables. PMID:27320359

  3. Thoracoscopic Ligation of the Thoracic Duct

    PubMed Central

    Teixeira, Julio A.

    2000-01-01

    Objective: When nonoperative treatment of chylothorax fails, thoracic duct ligation is usually performed through a thoracotomy. We describe two cases of persistent chylothorax, in a child and an adult, successfully treated with thoracoscopic ligation of the thoracic duct. Methods: A 4-year-old girl developed a right chylothorax following a Fontan procedure. Aggressive nonoperative management failed to eliminate the persistent chyle loss. A 72-year-old insulin-dependent diabetic man was involved in a motor vehicle accident, in which he sustained multiple fractured ribs, a right hemopneumothorax, a right femoral shaft fracture, and a T-11 thoracic vertebral fracture. Subsequently, he developed a right chylothorax, which did not respond to nonoperative management. Both patients were successfully treated with thoracoscopic ligation of the thoracic duct. Results: The child had significant decrease of chyle drainage following surgery. Increased drainage that appeared after the introduction of full feedings five days postoperatively was controlled with the somatostatin analog octreotide. The chest tube was removed two weeks after surgery. After two years' follow-up, she has had no recurrence of chylothorax. The adult had no chyle drainage following surgery. He was maintained on a medium-chain triglyceride diet postoperatively for two weeks. The chest tube was removed four days after surgery. After six months' follow-up, he has had no recurrence of chylothorax. Conclusions: Thoracoscopic ligation of the thoracic duct provides a safe and effective treatment of chylothorax and may avoid thoracotomy and its associated morbidity. PMID:10987402

  4. MAINE AQUIFERS

    EPA Science Inventory

    AQFRS24 contains polygons of significant aquifers in Maine (glacial deposits that are a significant ground water resource) mapped at a scale 1:24,000. This statewide coverage was derived from aquifer boundaries delineated and digitized by the Maine Geological Survey from data com...

  5. Rapid regression of exudative maculopathy in idiopathic retinitis, vasculitis, aneurysms and neuroretinitis syndrome after intravitreal ranibizumab.

    PubMed

    Marín-Lambíes, Cristina; Gallego-Pinazo, Roberto; Salom, David; Navarrete, Javier; Díaz-Llopis, Manuel

    2012-05-01

    The idiopathic retinitis, vasculitis, aneurysms and neuroretinitis syndrome is a rare retinal vascular disorder characterized by multiple leaking aneurysmal dilations, retinal vasculitis, neuroretinitis and peripheral vascular ischemia. Visual loss mainly occurs due to the development of retinal neovascularization and/or exudative maculopathy. Although the treatment of choice has not yet been established, retinal photocoagulation seems to be the best option to control the disease and to prevent its progression. Herein, we report a case of idiopathic retinitis, vasculitis, aneurysms and neuroretinitis syndrome with both retinal neovascularization and macular exudation successfully managed with intravitreal ranibizumab (Lucentis(®)) as adjunctive therapy to retinal photocoagulation. PMID:22949913

  6. Idiopathic brachial neuritis in a child: A case report and review of the literature

    PubMed Central

    Jain, Shikha; Bhatt, Girish Chandra; Rai, Nirendra; Bhan, Bhavna Dhingra

    2014-01-01

    Brachial neuritis is a rare disease in children, affecting mainly the lower motor neurons of the brachial plexus and/or individual nerves or nerve branches. We report a case of idiopathic brachial plexus neuritis in a 2½-year-old female child admitted with acute respiratory distress and given antibiotic therapy following which she developed weakness of the left hand. She was diagnosed as a case of idiopathic brachial plexus neuritis and was given supportive care. Although, the association with antibiotic therapy in this case could be incidental, indeed it is intriguing and requires further studies. PMID:25624937

  7. Idiopathic brachial neuritis in a child: A case report and review of the literature.

    PubMed

    Jain, Shikha; Bhatt, Girish Chandra; Rai, Nirendra; Bhan, Bhavna Dhingra

    2014-01-01

    Brachial neuritis is a rare disease in children, affecting mainly the lower motor neurons of the brachial plexus and/or individual nerves or nerve branches. We report a case of idiopathic brachial plexus neuritis in a 2½-year-old female child admitted with acute respiratory distress and given antibiotic therapy following which she developed weakness of the left hand. She was diagnosed as a case of idiopathic brachial plexus neuritis and was given supportive care. Although, the association with antibiotic therapy in this case could be incidental, indeed it is intriguing and requires further studies. PMID:25624937

  8. Patterns of cortical thinning in idiopathic rapid eye movement sleep behavior disorder.

    PubMed

    Rahayel, Shady; Montplaisir, Jacques; Monchi, Oury; Bedetti, Christophe; Postuma, Ronald B; Brambati, Simona; Carrier, Julie; Joubert, Sven; Latreille, Véronique; Jubault, Thomas; Gagnon, Jean-François

    2015-04-15

    Idiopathic rapid eye movement sleep behavior disorder is a parasomnia that is a risk factor for dementia with Lewy bodies and Parkinson's disease. Brain function impairments have been identified in this disorder, mainly in the frontal and posterior cortical regions. However, the anatomical support for these dysfunctions remains poorly understood. We investigated gray matter thickness, gray matter volume, and white matter integrity in patients with idiopathic rapid eye movement sleep behavior disorder. Twenty-four patients with polysomnography-confirmed idiopathic rapid eye movement sleep behavior disorder and 42 healthy individuals underwent a 3-tesla structural and diffusion magnetic resonance imaging examination using corticometry, voxel-based morphometry, and diffusion tensor imaging. In the patients with idiopathic rapid eye movement sleep behavior disorder, decreased cortical thickness was observed in the frontal cortex, the lingual gyrus, and the fusiform gyrus. Gray matter volume was reduced in the superior frontal sulcus only. Patients showed no increased gray matter thickness or volume. Diffusion tensor imaging analyses revealed no significant white matter differences between groups. Using corticometry in patients with idiopathic rapid eye movement sleep behavior disorder, several new cortical regions with gray matter alterations were identified, similar to those reported in dementia with Lewy bodies and Parkinson's disease. These findings provide some anatomical support for previously identified brain function impairments in this disorder. PMID:24676967

  9. The use of four-dimensional computed tomography to diagnose costoclavicular impingement causing thoracic outlet syndrome

    PubMed Central

    Troupis, John M; Bell, Simon N

    2014-01-01

    Thoracic outlet syndrome is caused by compression of the neurovascular structures crossing the interscalene triangle, costoclavicular space or retropectoralis minor space. The costoclavicular space is the most frequent site of arterial compression and is mainly a result of anatomical variations and masses occupying the costoclavicular space causing a compression effect on the vascular or neural structures within it. We present a case of thoracic outlet syndrome caused by dynamic impingement of the clavicle and the second rib diagnosed by four-dimensional computed tomography scanning.

  10. Penetrating injuries to the thoracic great vessels.

    PubMed

    Demetriades, D

    1997-01-01

    Penetrating injuries to the thoracic great vessels have been diagnosed with increased frequency because of the escalating use of automatic weapons. The overall incidence is 5.3% of gunshot wounds and 2% of stab wounds to the chest. Most of these patients reach the hospital dead or in severe shock. The overall mortality of thoracic aortic injuries is higher than 90% and in subclavian vascular injuries higher than 65%. In the prehospital phase, the "scoop and run" policy offers the best chances of survival and no attempts should be made for any form of stabilization. Investigations should be reserved only for fairly stable patients. Angiography, color flow Doppler, and transesophageal echocardiography may be useful in selected cases. Patients in cardiac arrest or imminent cardiac arrest may benefit from an emergency room thoracotomy. The surgical approach to specific thoracic great vessels is described. PMID:9271743

  11. European institutional accreditation of general thoracic surgery.

    PubMed

    Brunelli, Alessandro; Falcoz, Pierre Emmanuel

    2014-05-01

    To improve standardization of general thoracic surgery (GTS) practice across Europe, the European Society of Thoracic Surgeons (ESTS) has implemented a program of Institutional Accreditation. We reviewed the methods and rules of engagement of this program. A composite performance score (CPS) including outcome and process indicators is used to measure institutional performance and assess eligibility for accreditation. Eligible units are invited to participate and accept a local audit performed by an external auditors team composed by data inspectors and thoracic surgeons. In addition to data quality, a series of structural, procedural and qualification characteristics are inspected. Once the visit is complete, the team will produce an audit report to be sent to the members of the database committee for deliberation on the institutional accreditation of that unit. The Database committee will send an executive report to the ESTS Executive Committee for their final decision on the accreditation. PMID:24868447

  12. Acute Shingles after Resection of Thoracic Schwannoma

    PubMed Central

    Muesse, Jason L.; Blackmon, Shanda H.; Harris, Richard L.; Kim, Min P.

    2012-01-01

    Herpes zoster is relatively uncommon after surgery in immunocompetent patients. To our knowledge, there have been no reports of herpes zoster after the resection of a thoracic schwannoma. We report the case of a 48-year-old woman in whom acute shingles developed after the video-assisted thoracic surgical resection of a posterior mediastinal schwannoma adjacent to the 4th thoracic vertebral body. The patient recovered after receiving timely antiviral therapy. Rash and pain are common in patients who have wound infections and contact dermatitis after surgery, so the possible reactivation of varicella virus might not be prominent in the surgeon's mind. This case serves as a reminder that viral infections such as shingles should be considered in the differential diagnosis of postoperative erythema and pain. PMID:22740749

  13. Pleural abnormalities: thoracic ultrasound to the rescue!

    PubMed Central

    Pathmanathan, Sega; Lakshminarayana, Umesh B.; Avery, Gerard R.; Kastelik, Jack A.; Morjaria, Jaymin B.

    2013-01-01

    Diaphragmatic hernias that are diagnosed in adulthood may be traumatic or congenital in nature. Therefore, respiratory specialists need to be aware of the presentation of patients with these conditions. In this report, we describe a case series of patients with congenital and traumatic diaphragmatic hernias and highlight a varied range of their presentations. Abnormalities were noted in the thorax on the chest radiographs, but it was unclear as to the nature of the anomaly. The findings on thoracic ultrasound conducted by a pulmonologist helped to direct appropriate investigations avoiding unnecessary interventions. Instead of pleural effusions, consolidation or collapse, thoracic computed tomography demonstrated diaphragmatic hernias which were managed either conservatively or by surgery. There is increasing evidence that pulmonary specialists should be trained in thoracic ultrasonography to identify pleural pathology as well as safely conducting pleural-based interventions. PMID:23819018

  14. Pleural abnormalities: thoracic ultrasound to the rescue!

    PubMed

    Aslam, Imran; Pathmanathan, Sega; Lakshminarayana, Umesh B; Avery, Gerard R; Kastelik, Jack A; Morjaria, Jaymin B

    2013-07-01

    Diaphragmatic hernias that are diagnosed in adulthood may be traumatic or congenital in nature. Therefore, respiratory specialists need to be aware of the presentation of patients with these conditions. In this report, we describe a case series of patients with congenital and traumatic diaphragmatic hernias and highlight a varied range of their presentations. Abnormalities were noted in the thorax on the chest radiographs, but it was unclear as to the nature of the anomaly. The findings on thoracic ultrasound conducted by a pulmonologist helped to direct appropriate investigations avoiding unnecessary interventions. Instead of pleural effusions, consolidation or collapse, thoracic computed tomography demonstrated diaphragmatic hernias which were managed either conservatively or by surgery. There is increasing evidence that pulmonary specialists should be trained in thoracic ultrasonography to identify pleural pathology as well as safely conducting pleural-based interventions. PMID:23819018

  15. Change of paradigm in thoracic radionecrosis management.

    PubMed

    Dast, S; Assaf, N; Dessena, L; Almousawi, H; Herlin, C; Berna, P; Sinna, R

    2016-06-01

    Classically, muscular or omental flaps are the gold standard in the management of thoracic defects following radionecrosis debridement. Their vascular supply and antibacterial property was supposed to enhance healing compared with cutaneous flaps. The evolution of reconstructive surgery allowed us to challenge this dogma. Therefore, we present five consecutive cases of thoracic radionecrosis reconstructed with cutaneous perforator flaps. In four patients, we performed a free deep inferior epigastric perforator (DIEP) flap and one patient had a thoracodorsal perforator (TDAP) flap. Median time healing was 22.6 days with satisfactory cutaneous covering and good aesthetic results. There were no flap necrosis, no donor site complications. We believe that perforator flaps are a new alternative, reliable and elegant option that questions the dogma of muscular flaps in the management of thoracic radionecrosis. PMID:26831037

  16. Thoracic spinal trauma and associated injuries: should early spinal decompression be considered?

    PubMed

    Petitjean, M E; Mousselard, H; Pointillart, V; Lassie, P; Senegas, J; Dabadie, P

    1995-08-01

    The relative benefits of conservative or surgical treatment in thoracic spinal trauma are still controversial. Owing to its anatomic relations, thoracic spinal trauma is specific regarding neurologic prognosis, the high incidence of associated injuries, and surgical management. Over a 30-month period, 49 patients sustained thoracic spinal trauma with neurologic impairment. The authors review population characteristics, associated injuries, and surgical management, and underline the high incidence of associated injuries, in particular, blunt chest trauma. In their opinion, early spinal decompression has no indication in complete paraplegia. Concerning partial paraplegia, early surgery may enhance neurologic recovery. Nevertheless, they suggest three main criteria in deciding whether or not to perform surgery early: the existence of residual spinal compression, the degree of neurologic impairment, and the presence of potential hemorrhagic lesions or blunt chest trauma, especially pulmonary contusion. PMID:7674409

  17. Endoscopic procedures of the upper-thoracic sympathetic chain. A review.

    PubMed

    Drott, C; Göthberg, G; Claes, G

    1993-02-01

    The upsurge of endoscopic surgical procedures now includes procedures of the thoracic sympathetic chain. The number of articles on this issue is rapidly increasing. This article reviews the indications for as well as the technique, complications, side effects, and results of endoscopic upper-thoracic sympathetic ablation. Since 1977, nearly 900 cases have been described in the literature. The main indication is usually hyperhidrosis. The described techniques vary in detail, but the common denominators are simplicity, expedience, minimal surgical trauma, few complications, and low cost compared with standard methods of open surgery. The results are excellent, durable, and stand well compared with results of previous open techniques. Due to the overwhelming advantages of endoscopic methods, we can foresee an increasing adoption of these techniques and a subsequent relegation of the various open surgical procedures of the upper-thoracic sympathetic chain. PMID:8431126

  18. Genetics Home Reference: familial thoracic aortic aneurysm and dissection

    MedlinePlus

    ... Home Health Conditions familial TAAD familial thoracic aortic aneurysm and dissection Enable Javascript to view the expand/ ... Open All Close All Description Familial thoracic aortic aneurysm and dissection ( familial TAAD ) involves problems with the ...

  19. The History of Duke Thoracic Surgery.

    PubMed

    Smith, Peter K; Mulvihill, Michael S; D'Amico, Thomas A

    2015-01-01

    Since 1931, Duke Thoracic Surgery has been defined by excellence in patient care, research, and the education of leaders in surgery. In this work, the history, contributions, historic figures, and current structure of the program are reviewed. The program has cultivated a commitment to surgical investigation and training that persists to the present day. This commitment is manifest by the program's contributions to the field of cardiothoracic surgery, from the fundamental investigation of the coronary circulation and the development of the heat exchanger for myocardial preservation, to large-scale clinical trials in cardiac and thoracic surgery. PMID:26811042

  20. CT of nontraumatic thoracic aortic emergencies.

    PubMed

    Bhalla, Sanjeev; West, O Clark

    2005-10-01

    Computed tomography (CT), especially multidetector row CT (MDCT), is often the preferred imaging test used for evaluation of nontraumatic thoracic aortic abnormalities. Unenhanced images, usually followed by contrast-enhanced arterial imaging, allow for rapid detailed aortic assessment. Understanding the spectrum of acute thoracic aortic conditions which may present similarly (aortic dissection, aneurysm rupture, penetrating atherosclerotic ulcer, intramural hematoma) will ensure that patients are diagnosed and treated appropriately. Familiarity with imaging protocols and potential mimics will prevent confusion of normal anatomy and variants with aortic disease. PMID:16274000

  1. Practical management of Idiopathic Pulmonary Fibrosis.

    PubMed

    Kishaba, Tomoo

    2015-01-01

    Idiopathic Pulmonary Fibrosis (IPF) is relentless progressive interstitial lung disease (ILD) of unknown etiology. Main pathogenesis is aberrant recovery of epithelial injury and collagen deposition. Majority of IPF patients have been elderly men with smokers. However, there are important differential diagnosis such as fibrotic non-specific interstitial pneumonia (NSIP), Connective Tissue Disease (CTD) associated ILD, chronic hypersensitivity pneumonia (CHP). Clinical point of view, non-productive cough and progressive exertional dyspnea are main symptoms. In addition, scalene muscle hypertrophy, fine crackles and finger clubbing are key findings. Serum marker such as lactate deydrogenase (LDH), Krebs von den Lungeng-6 (KL-6) are sensitive for ILD detection and activity. Pulmonary function test and 6 minute walk test (6MWT) are quite meaningful physiological examination. Serial change of forced vital capacity 6MWT distance predict mortality of IPF. International IPF guideline published recently and highlighted on the importance of high resolution computed tomography (HRCT) findings. Key findings of IPF are honeycombing, traction bronchiectasis and subpleural reticular opacity. IPF is chronic progressive disease. Therefore, tracing disease behavior is crucial and unifying clinical, physiological, imaging information over time provide useful information for physicians.In management, many candidate agent failed to have positive result. Pirfenidone which is anti-fibrotic agent showed to slow the decline of vital capacity and prevent of acute exacerbation. Molecular agent such as nintedanib is promising agent for prevention of progression of IPF. In this review, we review the clinical information of IPF and IPF guideline. Lastly, we show the clinical algorithm of this devastated disease. PMID:26278687

  2. Perspective: Update on Idiopathic Intracranial Hypertension

    PubMed Central

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

    2011-01-01

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

  3. Mayo Clinic: An Institutional History of General Thoracic Surgery.

    PubMed

    Gillaspie, Erin A; Nichols, Francis C; Allen, Mark S

    2015-01-01

    The Mayo Clinic was started in Rochester, MN after a 1883 tornado disaster. The Mayo brothers, William and Charles began thoracic surgical procedures early in their career. Dr. Samuel Robinson is recognized as the first thoracic surgeon at Mayo. He was followed by Drs. Harrington and Claret who became famous surgeons. Many other notable surgeons have help to build the thoracic surgical practice into what is today a world renown center of excellence in thoracic surgery. PMID:26811041

  4. Genetics of idiopathic generalized epilepsies.

    PubMed

    Gardiner, Mark

    2005-01-01

    The idiopathic generalized epilepsies (IGEs) are considered to be primarily genetic in origin. They encompass a number of rare mendelian or monogenic epilepsies and more common forms which are familial but manifest as complex, non-mendelian traits. Recent advances have demonstrated that many monogenic IGEs are ion channelopathies. These include benign familial neonatal convulsions due to mutations in KCNQ2 or KCNQ3, generalized epilepsy with febrile seizures plus due to mutations in SCN1A, SCN2A, SCN1B, and GABRG2, autosomal-dominant juvenile myoclonic epilepsy (JME) due to a mutation in GABRA1 and mutations in CLCN2 associated with several IGE sub-types. There has also been progress in understanding the non-mendelian IGEs. A haplotype in the Malic Enzyme 2 gene, ME2, increases the risk for IGE in the homozygous state. Five missense mutations have been identified in EFHC1 in 6 of 44 families with JME. Rare sequence variants have been identified in CACNA1H in sporadic patients with childhood absence epilepsy in the Chinese Han population. These advances should lead to new approaches to diagnosis and treatment. PMID:16302872

  5. Seizures of idiopathic generalized epilepsies.

    PubMed

    Durón, Reyna M; Medina, Marco T; Martínez-Juárez, Iris E; Bailey, Julia N; Perez-Gosiengfiao, Katerina Tanya; Ramos-Ramírez, Ricardo; López-Ruiz, Minerva; Alonso, María Elisa; Ortega, Ramón H Castro; Pascual-Castroviejo, Ignacio; Machado-Salas, Jesús; Mija, Lizardo; Delgado-Escueta, Antonio V

    2005-01-01

    Idiopathic generalized epilepsies (IGEs) comprise at least 40% of epilepsies in the United States, 20% in Mexico, and 8% in Central America. Here, we review seizure phenotypes across IGE syndromes, their response to treatment and advances in molecular genetics that influence nosology. Our review included the Medline database from 1945 to 2005 and our prospectively collected Genetic Epilepsy Studies (GENESS) Consortium database. Generalized seizures occur with different and similar semiologies, frequencies, and patterns, ages at onset, and outcomes in different IGEs, suggesting common neuroanatomical pathways for seizure phenotypes. However, the same seizure phenotypes respond differently to the same treatments in different IGEs, suggesting different molecular defects across syndromes. De novo mutations in SCN1A in sporadic Dravet syndrome and germline mutations in SCN1A, SCN1B, and SCN2A in generalized epilepsies with febrile seizures plus have unraveled the heterogenous myoclonic epilepsies of infancy and early childhood. Mutations in GABRA1, GABRG2, and GABRB3 are associated with absence seizures, while mutations in CLCN2 and myoclonin/EFHC1 substantiate juvenile myoclonic epilepsy as a clinical entity. Refined understanding of seizure phenotypes, their semiology, frequencies, and patterns together with the identification of molecular lesions in IGEs continue to accelerate the development of molecular epileptology. PMID:16302874

  6. Scrotal calcinosis: idiopathic or dystrophic?

    PubMed

    Dubey, Suparna; Sharma, Rajeev; Maheshwari, Veena

    2010-01-01

    Scrotal calcinosis is a rare benign local process characterized by multiple, painless, hard scrotal nodules in the absence of any systemic metabolic disorder. Histological examination reveals extensive deposition of calcium in the dermis, which may be surrounded by histiocytes and an inflammatory giant cell reaction. Numerous theories have been propounded to explain the pathogenesis of this condition, but the principal debate revolves around whether the calcium is deposited at the site of previous epithelial cysts or the calcified nodules are purely idiopathic. This is the largest study of scrotal calcinosis to date with 100 cases, on which clinical, biochemical, radiological, cytopathological, and histopathological examinations were conducted. The histological picture shows a continuous spectrum of changes ranging from intact epithelial cysts (41.0%) - both normal and inflamed; through inflamed cysts containing calcific material in the lumen but with intact cyst wall (53.0%); calcified inflamed cysts with partial epithelial lining (11.0%); to 'naked' calcium deposits lying in the dermis (100%), sometimes compressing surrounding collagen fibres to form a pseudocyst (56.0%). The presence of normal values of calcium and phosphorus along with this spectrum of changes in histology both support the theory that these form by dystrophic calcification of epithelial cysts in a progression that involves inflammation, rupture, calcification and obliteration of the cyst wall. PMID:20178701

  7. Familial idiopathic normal pressure hydrocephalus.

    PubMed

    Huovinen, Joel; Kastinen, Sami; Komulainen, Simo; Oinas, Minna; Avellan, Cecilia; Frantzen, Janek; Rinne, Jaakko; Ronkainen, Antti; Kauppinen, Mikko; Lönnrot, Kimmo; Perola, Markus; Pyykkö, Okko T; Koivisto, Anne M; Remes, Anne M; Soininen, Hilkka; Hiltunen, Mikko; Helisalmi, Seppo; Kurki, Mitja; Jääskeläinen, Juha E; Leinonen, Ville

    2016-09-15

    Idiopathic normal pressure hydrocephalus (iNPH) is a late-onset surgically alleviated, progressive disease. We characterize a potential familial subgroup of iNPH in a nation-wide Finnish cohort of 375 shunt-operated iNPH-patients. The patients were questionnaired and phone-interviewed, whether they have relatives with either diagnosed iNPH or disease-related symptomatology. Then pedigrees of all families with more than one iNPH-case were drawn. Eighteen patients (4.8%) from 12 separate pedigrees had at least one shunt-operated relative whereas 42 patients (11%) had relatives with two or more triad symptoms. According to multivariate logistic regression analysis, familial iNPH-patients had up to 3-fold risk of clinical dementia compared to sporadic iNPH patients. This risk was independent from diagnosed Alzheimer's disease and APOE ε4 genotype. This study describes a familial entity of iNPH offering a novel approach to discover the potential genetic characteristics of iNPH. Discovered pedigrees offer an intriguing opportunity to conduct longitudinal studies targeting potential preclinical signs of iNPH. PMID:27538594

  8. MAINE HYDROGRAPHY

    EPA Science Inventory

    Hydronet_me24 and Hydropoly_me24 depict Maine's hydrography data, based on 8-digit hydrological unit codes (HUC's) at the 1:24,000 scale. Some New Hampshire and New Brunswick hydrography data are also included. The NHD hydrography data was compiled from previous ArcIn...

  9. MAINE WOODLOTS

    EPA Science Inventory

    MEOWN250 describes industrial, non-industrial, and public woodlot ownership in Maine at 1:250,000 scale. Industrial owners are those having at least one primary wood processing facility. Non-industrial owners are those with no primary wood processing facility. Public ownership...

  10. Maine Ingredients

    ERIC Educational Resources Information Center

    Waters, John K.

    2009-01-01

    This article features Maine Learning Technology Initiative (MLTI), the nation's first-ever statewide 1-to-1 laptop program which marks its seventh birthday by expanding into high schools, providing an occasion to celebrate--and to examine the components of its success. The plan to put laptops into the hands of every teacher and student in grades 7…

  11. Burnei’s anterior transthoracic retropleural approach of the thoracic spine: a new operative technique in the treatment of spinal disorders

    PubMed Central

    Gavriliu, TS; Japie, EM; Ghiță, RA; Hamei, Ș; Dughilă, C; Țiripa, IL; Elnayef, T

    2015-01-01

    Background: Up to the middle of the last century, the thoracic spine, especially in its upper part, has been considered an unapproachable site, a no-man’s land, but the constant evolution of medicine imposed techniques of the spine at these levels in order to solve a large area of pathology (infectious, tumoral, traumatic, and last but not least, deformative). This way, a series of anterior approaches allowed surgeons to gain access to the anterior part of the spine and the posterior mediastinum. The approaches described by Hodgson, Mirbaha or transthoracic transpleural approach (T4-T11), are enumerated. The idea to allow a more visible and extensive approach, but to avoid respiratory issues due to the lesion of the pleura, led to the description of a new anterior approach by Burnei in 2000. Material and method: Burnei’s approach represents an anterior approach to the thoracic spine, being a transthoracic and retropleural one. This approach allows a large area of spinal pathology due to infectious, traumatic, tumoral and degenerative (idiopathic or congenital scoliosis) causes. Statistically, this approach has been performed more frequently in cases of spinal instrumentation after diskectomy, in order to perform a partial correction of severe, rigid idiopathic scoliosis with more than 70 degrees Cobb and in cases of congenital scoliosis for hemivertebra resection and somatic synthesis to correct the scoliotic curve. Results: This kind of anterior approach allows the surgeon a large visibility of the anterior thoracic spine, diskectomies of up to 5 levels to tender the curve of the deformity and to ensure somatic or/ and transpedicular synthesis of up to 6 thoracic vertebrae. By performing a thoracotomy involving the resection of the posterior arches of the ribs, a thoracoplasty is also ensured with functional and aesthetic effects, by ameliorating the thoracic hump due to the scoliotic deformity. Conclusions: Burnei’s approach joins all the other anterior

  12. Therapeutic options in idiopathic burning mouth syndrome: literature review.

    PubMed

    Miziara, Ivan; Chagury, Azis; Vargas, Camila; Freitas, Ludmila; Mahmoud, Ali

    2015-01-01

    Introduction Burning mouth syndrome (BMS) is characterized by a burning sensation in the tongue, palate, lips, or gums of no well-defined etiology. The diagnosis and treatment for primary BMS are controversial. No specific laboratory tests or diagnostic criteria are well established, and the diagnosis is made by excluding all other possible disorders. Objective To review the literature on the main treatment options in idiopathic BMS and compare the best results of the main studies in 15 years. Data Synthesis We conducted a literature review on PubMed/MEDLINE, SciELO, and Cochrane-BIREME of work in the past 15 years, and only selected studies comparing different therapeutic options in idiopathic BMS, with preference for randomized and double-blind controlled studies. Final Comments Topical clonazepam showed good short-term results for the relief of pain, although this was not presented as a definitive cure. Similarly, α-lipoic acid showed good results, but there are few randomized controlled studies that showed the long-term results and complete remission of symptoms. On the other hand, cognitive therapy is reported as a good and lasting therapeutic option with the advantage of not having side effects, and it can be combined with pharmacologic therapy. PMID:25992157

  13. Therapeutic Options in Idiopathic Burning Mouth Syndrome: Literature Review

    PubMed Central

    Miziara, Ivan; Chagury, Azis; Vargas, Camila; Freitas, Ludmila; Mahmoud, Ali

    2014-01-01

    Introduction Burning mouth syndrome (BMS) is characterized by a burning sensation in the tongue, palate, lips, or gums of no well-defined etiology. The diagnosis and treatment for primary BMS are controversial. No specific laboratory tests or diagnostic criteria are well established, and the diagnosis is made by excluding all other possible disorders. Objective To review the literature on the main treatment options in idiopathic BMS and compare the best results of the main studies in 15 years. Data Synthesis We conducted a literature review on PubMed/MEDLINE, SciELO, and Cochrane-BIREME of work in the past 15 years, and only selected studies comparing different therapeutic options in idiopathic BMS, with preference for randomized and double-blind controlled studies. Final Comments Topical clonazepam showed good short-term results for the relief of pain, although this was not presented as a definitive cure. Similarly, α-lipoic acid showed good results, but there are few randomized controlled studies that showed the long-term results and complete remission of symptoms. On the other hand, cognitive therapy is reported as a good and lasting therapeutic option with the advantage of not having side effects, and it can be combined with pharmacologic therapy. PMID:25992157

  14. Pleuroparenchymal fibroelastosis: is it also an idiopathic entity?

    PubMed

    Portillo, Karina; Guasch Arriaga, Ignasi; Ruiz-Manzano, Juan

    2015-10-01

    Pleuroparenchymal fibroelastosis (PPFE) is a rare disease that has been recently included in the updated consensus on idiopathic interstitial pneumonias. It shares some clinical features with other chronic interstitial pneumonias (dyspnea, dry cough), and is radiologically characterized by pleural and subpleural parenchymal fibrosis and elastosis, mainly in the upper lobes. The main histological findings include pleural fibrosis and prominent subpleural and parenchymal fibroelastosis. Its characterization is based on the increasing number of cases reported in the literature, so several aspects of the etiology, pathogenesis and natural history are still unknown. Although some cases have been described as idiopathic, PPFE has been reported as a complication after bone marrow transplantation, lung transplantation and chemotherapy, especially with alkylating agents.Spontaneous or iatrogenic pneumothorax is a frequently reported complication of invasive diagnostic tests for identifying PPFE. The disease course is variable, ranging from slow progression to rapid clinical deterioration. No treatment has shown evidence of efficacy, and lung transplantation remains the only option for patients who fulfill the diagnostic criteria for this option. Recognizing and disseminating the specific features of PPFE is essential to raise the level of clinical suspicion for this entity, and to implement appropriate multidisciplinary diagnostic management. PMID:26099364

  15. Functional chiral asymmetry in descending thoracic aorta.

    PubMed

    Frazin, L J; Lanza, G; Vonesh, M; Khasho, F; Spitzzeri, C; McGee, S; Mehlman, D; Chandran, K B; Talano, J; McPherson, D

    1990-12-01

    To determine whether rotational blood flow or chiral asymmetry exists in the human descending thoracic aorta, we established the ability of color Doppler ultrasound to detect rotational flow in a tornado tube model of a vortex descending fluid column. In a model of the human aortic arch with a pulse duplicator, color Doppler was then used to demonstrate that rotational flow occurs first in the transverse arch and then in the proximal descending thoracic aorta. With the use of color Doppler esophageal echocardiography, 53 patients (age range, 25-78 years; mean age, 56.4 years) were prospectively examined for rotational flow in the descending thoracic aorta. At 10 cm superior to retro-left ventricular position, 22 of 38 patients (58%) revealed rotational flow with obvious diastolic counterclockwise rotation but less obvious systolic clockwise rotation. At 5 cm superior to retro-left ventricular position, 29 of 46 patients (63%) revealed rotational flow with a tendency toward systolic clockwise and diastolic counterclockwise rotation. At the retro-left ventricular position, 47 of 53 patients (89%) revealed rotational flow, usually of a clockwise direction, occurring in systole. Our data suggest that aortic flow is not purely pulsatile and axial but has a rotational component. Rotational flow begins in the aortic arch and is carried through to the descending thoracic aorta, where flow is chirally asymmetric with systolic clockwise and diastolic counterclockwise components. These data demonstrate an aortic rotational flow component that may have physiological implications for organ perfusion. PMID:2242523

  16. Odor-associated idiopathic anaphylaxis. A case report.

    PubMed

    Saunders, R L; Halpern, G M; Gershwin, M E

    1995-01-01

    A 44 year old woman is described who appears to have idiopathic anaphylaxis triggered by chemical odors. Her case and a general discussion of anaphylaxis are presented. The known causes of anaphylaxis and a discourse on idiopathic anaphylaxis are given. The treatment of idiopathic anaphylaxis is discussed. PMID:7631593

  17. Chronic pain and the thoracic spine.

    PubMed

    Louw, Adriaan; Schmidt, Stephen G

    2015-07-01

    In recent years there has been an increased interest in pain neuroscience in physical therapy.1,2 Emerging pain neuroscience research has challenged prevailing models used to understand and treat pain, including the Cartesian model of pain and the pain gate.2-4 Focus has shifted to the brain's processing of a pain experience, the pain neuromatrix and more recently, cortical reorganisation of body maps.2,3,5,6 In turn, these emerging theories have catapulted new treatments, such as therapeutic neuroscience education (TNE)7-10 and graded motor imagery (GMI),11,12 to the forefront of treating people suffering from persistent spinal pain. In line with their increased use, both of these approaches have exponentially gathered increasing evidence to support their use.4,10 For example, various randomised controlled trials and systematic reviews have shown that teaching patients more about the biology and physiology of their pain experience leads to positive changes in pain, pain catastrophization, function, physical movement and healthcare utilisation.7-10 Graded motor imagery, in turn, has shown increasing evidence to help pain and disability in complex pain states such as complex regional pain syndrome (CRPS).11,12 Most research using TNE and GMI has focussed on chronic low back pain (CLBP) and CRPS and none of these advanced pain treatments have been trialled on the thoracic spine. This lack of research and writings in regards to the thoracic spine is not unique to pain science, but also in manual therapy. There are, however, very unique pain neuroscience issues that skilled manual therapists may find clinically meaningful when treating a patient struggling with persistent thoracic pain. Utilising the latest understanding of pain neuroscience, three key clinical chronic thoracic issues will be discussed - hypersensitisation of intercostal nerves, posterior primary rami nerves mimicking Cloward areas and mechanical and sensitisation issues of the spinal dura in the

  18. Cerebrospinal Fluid Leakage after Thoracic Decompression

    PubMed Central

    Hu, Pan-Pan; Liu, Xiao-Guang; Yu, Miao

    2016-01-01

    Objective: The objective of this study is to review cerebrospinal fluid leakage (CSFL) after thoracic decompression and describe its regular and special features. Data Sources: Literature cited in this review was retrieved from PubMed and Medline and was primarily published during the last 10 years. “Cerebrospinal fluid”, “leakage”, “dural tears”, and “thoracic decompression” were the indexed terms. Relevant citations in the retrieved articles were also screened to include more data. Study Selection: All retrieved literature was scrutinized, and four categories were recorded: incidence and risk factors, complications, treatment modalities, and prognosis. Results: CSFL is much more frequent after thoracic decompression than after cervical and lumbar spinal surgeries. Its occurrence is related to many clinical factors, especially the presence of ossified ligaments and the adhesion of the dural sac. While its impact on the late neurological recovery is currently controversial, CSFL increases the risk of other perioperative complications, such as low intracranial pressure symptoms, infection, and vascular events. The combined use of primary repairs during the operation and conservative treatment postoperatively is generally effective for most CSFL cases, whereas lumbar drains and reoperations should be implemented as rescue options for refractory cases only. Conclusions: CSFL after thoracic decompression has not been specifically investigated, so the present study provides a systematic and comprehensive review of the issue. CSFL is a multi-factor-related complication, and pathological factors play a decisive role. The importance of CSFL is in its impact on the increased risk of other complications during the postoperative period. Methods to prevent these complications are in need. In addition, though the required treatment resources are not special for CSFL after thoracic decompression, most CSFL cases are conservatively curable, and surgeons should be

  19. Chronic pain and the thoracic spine

    PubMed Central

    Louw, Adriaan; Schmidt, Stephen G.

    2015-01-01

    In recent years there has been an increased interest in pain neuroscience in physical therapy.1,2 Emerging pain neuroscience research has challenged prevailing models used to understand and treat pain, including the Cartesian model of pain and the pain gate.2–4 Focus has shifted to the brain's processing of a pain experience, the pain neuromatrix and more recently, cortical reorganisation of body maps.2,3,5,6 In turn, these emerging theories have catapulted new treatments, such as therapeutic neuroscience education (TNE)7–10 and graded motor imagery (GMI),11,12 to the forefront of treating people suffering from persistent spinal pain. In line with their increased use, both of these approaches have exponentially gathered increasing evidence to support their use.4,10 For example, various randomised controlled trials and systematic reviews have shown that teaching patients more about the biology and physiology of their pain experience leads to positive changes in pain, pain catastrophization, function, physical movement and healthcare utilisation.7–10 Graded motor imagery, in turn, has shown increasing evidence to help pain and disability in complex pain states such as complex regional pain syndrome (CRPS).11,12 Most research using TNE and GMI has focussed on chronic low back pain (CLBP) and CRPS and none of these advanced pain treatments have been trialled on the thoracic spine. This lack of research and writings in regards to the thoracic spine is not unique to pain science, but also in manual therapy. There are, however, very unique pain neuroscience issues that skilled manual therapists may find clinically meaningful when treating a patient struggling with persistent thoracic pain. Utilising the latest understanding of pain neuroscience, three key clinical chronic thoracic issues will be discussed – hypersensitisation of intercostal nerves, posterior primary rami nerves mimicking Cloward areas and mechanical and sensitisation issues of the spinal dura in

  20. Non-intubated anesthesia in thoracic surgery—technical issues

    PubMed Central

    2015-01-01

    Performing awake thoracic surgery (ATS) is technically more challenging than thoracic surgery under general anesthesia (GA), but it can result in a greater benefit for the patient. Local wound infiltration and lidocaine administration in the pleural space can be considered for ATS. More invasive techniques are local wound infiltration with wound catheter insertion, thoracic wall blocks, selective intercostal nerve blockade, thoracic paravertebral blockade and thoracic epidural analgesia, offering the advantage of a catheter placement which can also be continued for postoperative analgesia. PMID:26046050

  1. Nonsurgical Management of Adolescent Idiopathic Scoliosis.

    PubMed

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

    2016-08-01

    Pediatric patient visits for spinal deformity are common. Most of these visits are for nonsurgical management of scoliosis, with approximately 600,000 visits for adolescent idiopathic scoliosis (AIS) annually. Appropriate management of scoliotic curves that do not meet surgical indication parameters is essential. Renewed enthusiasm for nonsurgical management of AIS (eg, bracing, physical therapy) exists in part because of the results of the Bracing in Adolescent Idiopathic Scoliosis Trial, which is the only randomized controlled trial available on the use of bracing for AIS. Bracing is appropriate for idiopathic curves between 20° and 40°, with successful control of these curves reported in >70% of patients. Patient adherence to the prescribed duration of wear is essential to maximize the effectiveness of the brace. The choice of brace type must be individualized according to the deformity and the patient's personality as well as the practice setting and brace availability. PMID:27388720

  2. Idiopathic Nonviral Cryoglobulinemia Treated Successfully With Rituximab.

    PubMed

    Kamel, Mahmoud; Thajudeen, Bijin; Bracamonte, Erika; Madhrira, Machaiah

    2016-01-01

    Cryoglobulinemia is a systemic inflammatory syndrome that generally involves small-to-medium vessel vasculitis due to cryoglobulin-containing immune complexes. The therapeutic management of idiopathic cryoglobulinemic vasculitis has yet to be defined because no study has evaluated the best strategies. However, treatment of severe vasculitis is traditionally based on a combination of corticosteroids and immunosuppressants or plasmapheresis, and more recently rituximab. We report a case of 77-year-old female patient diagnosed with idiopathic cryoglobulinemia, treated successfully with 6 months prednisone tapering and 2 doses of rituximab (1 g each dose). After receiving the above-mentioned treatment, her creatinine went back to normal with resolution of proteinuria and hematuria, normalization of serum complements, and significant improvement in her clinical picture. We conclude that rituximab could be an effective treatment for idiopathic cryoglobulnemia. PMID:24914502

  3. Long-term three-dimensional changes of the spine after posterior spinal instrumentation and fusion in adolescent idiopathic scoliosis.

    PubMed

    Papin, P; Labelle, H; Delorme, S; Aubin, C E; de Guise, J A; Dansereau, J

    1999-01-01

    This is a prospective study comparing the short- and long-term three-dimensional (3D) changes in shape, length and balance of the spine after spinal instrumentation and fusion in a group of adolescents with idiopathic scoliosis. The objective of the study was to evaluate the stability over time of the postoperative changes of the spine after instrumentation with multi rod, hook and screw instrumentation systems. Thirty adolescents (average age: 14.5+/-1.6 years) undergoing surgery by a posterior approach had computerized 3D reconstructions of the spine done at an average of 3 days preoperatively (stage I), and 2 months (stage II) and 2,5 years (stage III) after surgery, using a digital multi-planar radiographic technique. Stages I, II and III were compared using various geometrical parameters of spinal length, curve severity, and orientation. Significant improvement of curve magnitude between stages I and II was documented in the frontal plane for thoracic and lumbar curves, as well as in the orientation of the plane of maximum deformity, which was significantly shifted towards the sagittal plane in thoracic curves. However, there was a significant loss of this correction between stages II and III. Slight changes were noted in apical vertebral rotation, in thoracic kyphosis and in lumbar lordosis. Spinal length and height were significantly increased at stage II, but at long-term follow-up spinal length continued to increase while spinal height remained similar. These results indicate that although a significant 3D correction can be obtained after posterior instrumentation and fusion, a significant loss of correction and an increase in spinal length occur in the years following surgery, suggesting that a crankshaft phenomenon may be an important factor altering the long-term 3D correction after posterior instrumentation of the spine for idiopathic scoliosis. PMID:10190849

  4. Uncertainty Quantification applied to flow simulations in thoracic aortic aneurysms

    NASA Astrophysics Data System (ADS)

    Boccadifuoco, Alessandro; Mariotti, Alessandro; Celi, Simona; Martini, Nicola; Salvetti, Maria Vittoria

    2015-11-01

    The thoracic aortic aneurysm is a progressive dilatation of the thoracic aorta causing a weakness in the aortic wall, which may eventually cause life-threatening events. Clinical decisions on treatment strategies are currently based on empiric criteria, like the aortic diameter value or its growth rate. Numerical simulations can give the quantification of important indexes which are impossible to be obtained through in-vivo measurements and can provide supplementary information. Hemodynamic simulations are carried out by using the open-source tool SimVascular and considering patient-specific geometries. One of the main issues in these simulations is the choice of suitable boundary conditions, modeling the organs and vessels not included in the computational domain. The current practice is to use outflow conditions based on resistance and capacitance, whose values are tuned to obtain a physiological behavior of the patient pressure. However it is not known a priori how this choice affects the results of the simulation. The impact of the uncertainties in these outflow parameters is investigated here by using the generalized Polynomial Chaos approach. This analysis also permits to calibrate the outflow-boundary parameters when patient-specific in-vivo data are available.

  5. Idiopathic non-specific interstitial pneumonia.

    PubMed

    Belloli, Elizabeth A; Beckford, Rosemarie; Hadley, Ryan; Flaherty, Kevin R

    2016-02-01

    Non-specific interstitial pneumonia (NSIP) is an interstitial lung disease that may be idiopathic or secondary to connective tissue disease, toxins or numerous other causes. Idiopathic NSIP is a rare diagnosis and requires exclusion of these other possible causes. Patients typically present in mid-adulthood with dyspnoea, cough and often constitutional symptoms including fever and fatigue. The disease has a female predominance, and more than 50% of patients have never smoked. Physical exam features mild hypoxaemia and inspiratory rales. Pulmonary function tests demonstrate restriction and a low diffusing capacity for carbon monoxide. High-resolution computed tomography abnormalities include predominantly lower lobe subpleural reticular changes, traction bronchiectasis and ground-glass opacities; honeycombing is rarely seen. An evaluation of the underlying pathology is necessary for a firm diagnosis. Histologically, alveolar and interstitial mononuclear cell inflammation and fibrosis are seen in a temporally uniform pattern with preserved underlying alveolar architecture. NSIP must be differentiated from other parenchymal lung diseases including idiopathic pulmonary fibrosis and hypersensitivity pneumonitis. A thorough exposure history and assessment for underlying connective tissue diseases are highly important, as positive findings in these categories would likely denote a case of secondary NSIP. A multi-disciplinary discussion that includes pulmonologist(s), radiologist(s) and pathologist(s) assists in reaching a consensus diagnosis and improves diagnostic accuracy. Treatment of idiopathic NSIP, although not well proven, is generally instituted in the form of immunosuppression. Prognosis is favourable compared with idiopathic pulmonary fibrosis, although the diagnosis still carries an attributable mortality. Herein we will summarize the clinical characteristics and management of idiopathic NSIP. PMID:26564810

  6. Complications corner: Anterior thoracic disc surgery with dural tear/CSF fistula and low-pressure pleural drain led to severe intracranial hypotension

    PubMed Central

    Oudeman, Eline A.; Tewarie, Rishi D. S. Nandoe; Jöbsis, G. Joost; Arts, Mark P.; Kruyt, Nyika D.

    2015-01-01

    Background: Thoracic disc surgery can lead to a life-threatening complication: intracranial hypotension due to a subarachnoid-pleural fistula. Case Description: We report a 63-year-old male with paraparesis due to multiple herniated thoracic discs, with compressive myelopathy. The patient required a circumferential procedure including a laminectomy/fusion followed by an anterior thoracic decompression to address both diffuse idiopathic skeletal hyperostosis (DISH) anteriorly and posterior stenosis. The postoperative course was complicated by severe intracranial hypotension attributed to the erroneous placement of a low-pressure drain placed in the pleural cavity instead of a lumbar drain; this resulted in subdural hematoma's necessitating subsequent surgery. Conclusion: Severe neurological deterioration occurring after thoracic decompressive surgery may rarely be attributed to intracranial hypotension due to a subarachnoid-pleural fistula. Patients should be treated with external lumbar drainage of cerebrospinal fluid for 3–5 days rather than a low-pressure pleural drain to avoid the onset of intracranial hypotension leading to symptomatic subdural hematomas. PMID:26005575

  7. Echocardiographic and Hemodynamic Predictors of Mortality in Idiopathic Pulmonary Fibrosis

    PubMed Central

    Rivera-Lebron, Belinda N.; Forfia, Paul R.; Kreider, Maryl; Lee, James C.; Holmes, John H.

    2013-01-01

    Background: Idiopathic pulmonary fibrosis (IPF) can lead to the development of pulmonary hypertension, which is associated with an increased risk of death. In pulmonary arterial hypertension, survival is directly related to the capacity of the right ventricle to adapt to elevated pulmonary vascular load. The relative importance of right ventricular function in IPF is not well understood. Our objective was to evaluate right ventricular echocardiographic and hemodynamic predictors of mortality in a cohort of patients with IPF referred for lung transplant evaluation. Methods: We performed a retrospective cohort study of 135 patients who met 2011 American Thoracic Society/European Respiratory Society criteria for IPF and who were evaluated for lung transplantation at the Hospital of the University of Pennsylvania. Results: Right ventricle:left ventricle diameter ratio (hazard ratio [HR], 4.5; 95% CI, 1.7-11.9), moderate to severe right atrial and right ventricular dilation (HR, 2.9; 95% CI, 1.4-5.9; and HR, 2.7; 95% CI, 1.4-5.4, respectively) and right ventricular dysfunction (HR, 5.5; 95% CI, 2.6-11.5) were associated with an increased risk of death. Higher pulmonary vascular resistance was also associated with increased mortality (HR per 1 Wood unit, 1.3; 95% CI, 1.1-1.5). These risk factors were independent of age, sex, race, height, weight, FVC, and lung transplantation status. Other hemodynamic indices, such as mean pulmonary artery pressure and cardiac index, were not associated with outcome. Conclusions: Right-sided heart size and right ventricular dysfunction measured by echocardiography and higher pulmonary vascular resistance by invasive hemodynamic assessment predict mortality in patients with IPF evaluated for lung transplantation. PMID:23450321

  8. Dry needling for the management of thoracic spine pain.

    PubMed

    Fernández-de-Las-Peñas, César; Layton, Michelle; Dommerholt, Jan

    2015-07-01

    Thoracic spine pain is as disabling as neck and low back pain without receiving the same level of attention in the scientific literature. Among the different structures that can refer pain to the thoracic spine, muscles often play a relevant role. Trigger points (TrPs) from neck, shoulder and spinal muscles can induce pain in the region of the thoracic spine. There is a lack of evidence reporting the presence of TrPs in the region of the thoracic spine, but clinical evidence suggests that TrPs can be a potential source of thoracic spine pain. The current paper discusses the role of TrPs in the thoracic spine and dry needling (DN) for the management of TrPs in the thoracic multifidi and longissimus thoracis. This paper also includes a brief discussion of the application of DN in other tissues such as tendons, ligaments and scars. PMID:26309385

  9. Dry needling for the management of thoracic spine pain

    PubMed Central

    Fernández-de-las-Peñas, César; Layton, Michelle; Dommerholt, Jan

    2015-01-01

    Thoracic spine pain is as disabling as neck and low back pain without receiving the same level of attention in the scientific literature. Among the different structures that can refer pain to the thoracic spine, muscles often play a relevant role. Trigger points (TrPs) from neck, shoulder and spinal muscles can induce pain in the region of the thoracic spine. There is a lack of evidence reporting the presence of TrPs in the region of the thoracic spine, but clinical evidence suggests that TrPs can be a potential source of thoracic spine pain. The current paper discusses the role of TrPs in the thoracic spine and dry needling (DN) for the management of TrPs in the thoracic multifidi and longissimus thoracis. This paper also includes a brief discussion of the application of DN in other tissues such as tendons, ligaments and scars. PMID:26309385

  10. Idiopathic pulmonary fibrosis: an Australian perspective.

    PubMed

    Prasad, J; Holland, A E; Glaspole, I; Westall, G

    2016-06-01

    Idiopathic pulmonary fibrosis is a progressive interstitial lung disease of unknown aetiology with a dismal median survival of 3 years. Patients typically develop progressive dyspnoea and increasing exercise limitation. With a rising incidence and prevalence, an unpredictable disease course and limited treatment options, it is rapidly becoming an important public health concern. To date, lung transplantation has been the sole viable hope for treatment for those who qualify. However, the landscape of idiopathic pulmonary fibrosis management is changing, with the recent emergence of novel pharmacotherapy shown to have a favourable influence on the natural history of this disease. PMID:27257148

  11. Idiopathic Parkinson's disease: epidemiology, diagnosis and management.

    PubMed Central

    Ben-Shlomo, Y; Sieradzan, K

    1995-01-01

    Since the introduction of levodopa therapy for idiopathic Parkinson's disease over 20 years ago, there has been an awakening of research interest in this chronic neuro-degenerative disorder. This paper describes current understanding of the role of genetic and environmental factors in the aetiology of idiopathic Parkinson's disease and problems associated with both diagnosis and management. It briefly outlines both pharmacological and non-pharmacological options for treatment. Despite an increasing armoury of available treatments, the optimum management for this condition remains controversial. PMID:7619574

  12. Acute Paraplegia due to Thoracic Hematomyelia.

    PubMed

    Akpınar, Aykut; Celik, Bahattin; Canbek, Ihsan; Karavelioğlu, Ergun

    2016-01-01

    Spontaneous intraspinal intramedullary hemorrhage is a rare entity with the acute onset of neurologic symptoms. The etiology of idiopathic spontaneous hematomyelia (ISH) is unknown, and there are few published case reports. Hematomyelia is mostly associated with trauma, but the other nontraumatic etiologies are vascular malformations, tumors, bleeding disorders, syphilis, syrinx, and myelitis. MRI is a good choice for early diagnosis. Hematomyelia usually causes acute spinal cord syndrome due to the compression and destruction of the spinal cord. A high-dose steroid treatment and surgical decompression and evacuation of hematoma are the urgent solution methods. We present idiopathic spontaneous hematomyelia of a previously healthy 80-year-old male with a sudden onset of back pain and paraplegia. PMID:27478663

  13. Acute Paraplegia due to Thoracic Hematomyelia

    PubMed Central

    Celik, Bahattin; Canbek, Ihsan; Karavelioğlu, Ergun

    2016-01-01

    Spontaneous intraspinal intramedullary hemorrhage is a rare entity with the acute onset of neurologic symptoms. The etiology of idiopathic spontaneous hematomyelia (ISH) is unknown, and there are few published case reports. Hematomyelia is mostly associated with trauma, but the other nontraumatic etiologies are vascular malformations, tumors, bleeding disorders, syphilis, syrinx, and myelitis. MRI is a good choice for early diagnosis. Hematomyelia usually causes acute spinal cord syndrome due to the compression and destruction of the spinal cord. A high-dose steroid treatment and surgical decompression and evacuation of hematoma are the urgent solution methods. We present idiopathic spontaneous hematomyelia of a previously healthy 80-year-old male with a sudden onset of back pain and paraplegia. PMID:27478663

  14. Video-Assisted Thoracoscopic Surgery Plus Lumbar Mini-Open Surgery for Adolescent Idiopathic Scoliosis

    PubMed Central

    Chong, Hyon Su; Kim, Hak Sun; Ankur, Nanda; Kho, Phillip Anthony; Kim, Sung Jun; Kim, Do Yeon; Park, Jin Oh; Moon, Seong Hwan; Lee, Hwan Mo

    2011-01-01

    Purpose The objectives of this study are to describe the outcome of adolescent idiopathic scoliosis (AIS) patients treated with Video Assisted Thoracoscopic Surgery (VATS) plus supplementary minimal incision in the lumbar region for thoracic and lumbar deformity correction and fusion. Materials and Methods This is a case series of 13 patients treated with VATS plus lumbar mini-open surgery for AIS. A total of 13 patients requiring fusions of both the thoracic and lumbar regions were included in this study: 5 of these patients were classified as Lenke type 1A and 8 as Lenke type 5C. Fusion was performed using VATS up to T12 or L1 vertebral level. Lower levels were accessed via a small mini-incision in the lumbar area to gain access to the lumbar spine via the retroperitoneal space. All patients had a minimum follow-up of 1 year. Results The average number of fused vertebrae was 7.1 levels. A significant correction in the Cobb angle was obtained at the final follow-up (p = 0.001). The instrumented segmental angle in the sagittal plane was relatively well-maintained following surgery, albeit with a slight increase. Scoliosis Research Society-22 (SRS-22) scores were noted have significantly improved at the final follow-up (p < 0.05). Conclusion Indications for the use of VATS may be extended from patients with localized thoracic scoliosis to those with thoracolumbar scoliosis. By utilizing a supplementary minimal incision in the lumbar region, a satisfactory deformity correction may be accomplished with minimal post-operative scarring. PMID:21155045

  15. A unified concept of idiopathic orofacial pain: clinical features.

    PubMed

    Woda, A; Pionchon, P

    1999-01-01

    The main features of atypical facial pain, stomatodynia, atypical odontalgia, and masticatory muscle and temporomandibular joint (TMJ) disorders are compared in this article, which included a search of articles indexed in MEDLINE. The fact that their terminology has been the subject of many debates can be considered a consequence of taxonomic difficulties and uncertainties. Epidemiologic studies indicate marked female predominance for all types of idiopathic orofacial pain. There is also a difference in the age of maximal prevalence between masticatory muscle and TMJ disorders and the other entities. The clinical presentations display several symptoms in common. Pain is oral, perioral, or facial and does not follow a nervous pathway. It has been present for the last 4 to 6 months or has returned periodically in the same form over a period of several months or years. The pain is continuous, has no major paroxysmal character, and is present throughout all or part of the day. It is generally absent during sleep. Clinical, radiographic, or laboratory examination does not reveal any obvious organic cause of pain. There is also a frequent presence of certain psychologic factors, personality traits, or life events. Based on these shared characteristics, a unified concept is proposed. Each of these entities belongs to a group of idiopathic orofacial pain and could be expressed in either the jaws, the buccal mucosa, the teeth, the masticatory muscles, or the TMJ. PMID:10823031

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

    PubMed Central

    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 of a malignant lesion; however, fine-needle aspiration cytology (FNAC) revealed features of granulomatous mastitis, and the subsequent histology of the excised lump confirmed the diagnosis of IGLM. Conclusions IGLM should be considered as one of the differential diagnoses when granulomas are encountered in breast FNAC and biopsy. A definitive diagnosis of IGLM can be made by identifying its characteristic histomorphology and ruling out other causes for granulomatous inflammation. An exact diagnosis is essential since the treatment for different granulomatous conditions of the breast varies. PMID:27437133

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

    PubMed Central

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

    2016-01-01

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

  18. Resistin in idiopathic inflammatory myopathies

    PubMed Central

    2012-01-01

    Introduction The purpose of this study was to evaluate and compare the serum levels and local expression of resistin in patients with idiopathic inflammatory myopathies to controls, and to determine the relationship between resistin levels, inflammation and disease activity. Methods Serum resistin levels were determined in 42 patients with inflammatory myopathies and 27 healthy controls. The association among resistin levels, inflammation, global disease activity and muscle strength was examined. The expression of resistin in muscle tissues from patients with inflammatory myopathies and healthy controls was evaluated. Gene expression and protein release from resistin-stimulated muscle and mononuclear cells were assessed. Results In patients with inflammatory myopathies, the serum levels of resistin were significantly higher than those observed in controls (8.53 ± 6.84 vs. 4.54 ± 1.08 ng/ml, P < 0.0001) and correlated with C-reactive protein (CRP) levels (r = 0.328, P = 0.044) and myositis disease activity assessment visual analogue scales (MYOACT) (r = 0.382, P = 0.026). Stronger association was observed between the levels of serum resistin and CRP levels (r = 0.717, P = 0.037) as well as MYOACT (r = 0.798, P = 0.007), and there was a trend towards correlation between serum resistin and myoglobin levels (r = 0.650, P = 0.067) in anti-Jo-1 positive patients. Furthermore, in patients with dermatomyositis, serum resistin levels significantly correlated with MYOACT (r = 0.667, P = 0.001), creatine kinase (r = 0.739, P = 0.001) and myoglobin levels (r = 0.791, P = 0.0003) and showed a trend towards correlation with CRP levels (r = 0.447, P = 0.067). Resistin expression in muscle tissue was significantly higher in patients with inflammatory myopathies compared to controls, and resistin induced the expression of interleukins (IL)-1β and IL-6 and monocyte chemoattractant protein (MCP)-1 in mononuclear cells but not in myocytes. Conclusions The results of this study

  19. [Digital thoracic radiology: devices, image processing, limits].

    PubMed

    Frija, J; de Géry, S; Lallouet, F; Guermazi, A; Zagdanski, A M; De Kerviler, E

    2001-09-01

    In a first part, the different techniques of digital thoracic radiography are described. Since computed radiography with phosphore plates are the most commercialized it is more emphasized. But the other detectors are also described, as the drum coated with selenium and the direct digital radiography with selenium detectors. The other detectors are also studied in particular indirect flat panels detectors and the system with four high resolution CCD cameras. In a second step the most important image processing are discussed: the gradation curves, the unsharp mask processing, the system MUSICA, the dynamic range compression or reduction, the soustraction with dual energy. In the last part the advantages and the drawbacks of computed thoracic radiography are emphasized. The most important are the almost constant good quality of the pictures and the possibilities of image processing. PMID:11567193

  20. Thoracic Aortic Dissection: Are Matrix Metalloproteinases Involved?

    PubMed Central

    Zhang, Xiaoming; Shen, Ying H.; LeMaire, Scott A.

    2010-01-01

    Thoracic aortic dissection, one of the major diseases affecting the aorta, carries a very high mortality rate. Improving our understanding of the pathobiology of this disease may help us develop medical treatments to prevent dissection and subsequent aneurysm formation and rupture. Dissection is associated with degeneration of the aortic media. Recent studies have shown increased expression and activation of a family of proteolytic enzymes—called matrix metalloproteinases (MMPs)—in dissected aortic tissue, suggesting that MMPs may play a major role in this disease. Inhibition of MMPs may be beneficial in reducing MMP-mediated aortic damage associated with dissection. This article reviews the recent literature and summarizes our current understanding of the role of MMPs in the pathobiology of thoracic aortic dissection. The potential importance of MMP inhibition as a future treatment of aortic dissection is also discussed. PMID:19476747

  1. Acute Aortic Syndromes and Thoracic Aortic Aneurysm

    PubMed Central

    Ramanath, Vijay S.; Oh, Jae K.; Sundt, Thoralf M.; Eagle, Kim A.

    2009-01-01

    Acute and chronic aortic diseases have been diagnosed and studied by physicians for centuries. Both the diagnosis and treatment of aortic diseases have been steadily improving over time, largely because of increased physician awareness and improvements in diagnostic modalities. This comprehensive review discusses the pathophysiology and risk factors, classification schemes, epidemiology, clinical presentations, diagnostic modalities, management options, and outcomes of various aortic conditions, including acute aortic dissection (and its variants intramural hematoma and penetrating aortic ulcers) and thoracic aortic aneurysms. Literature searches of the PubMed database were conducted using the following keywords: aortic dissection, intramural hematoma, aortic ulcer, and thoracic aortic aneurysm. Retrospective and prospective studies performed within the past 20 years were included in the review; however, most data are from the past 15 years. PMID:19411444

  2. Endovascular repair of thoracic aortic aneurysms.

    PubMed

    Cartes-Zumelzu, F; Lammer, J; Kretschmer, G; Hoelzenbein, T; Grabenwöger, M; Thurnher, S

    2000-03-01

    The standard technique for the treatment of descending thoracic aortic aneurysms is elective open surgical repair with graft interposition. This standard approach, although steadily improving, is associated with high morbidity and substantial mortality rates and implies a major surgical procedure with lateral thoracotomy, use of cardiopulmonary bypass, long operation times and a variety of peri- and postoperative complications. This and the success of the first endoluminal treatment of abdominal aortic aneurysms by Parodi et al. prompted the attention to be thrown on the treatment of descending thoracic aortic aneurysms with endoluminal stent-grafts in many large centres. The aim of this new minimally invasive technique is to exclude the aneurysm from blood flow and in consequence to avoid pressure stress on the aneurysmatic aortic wall, by avoiding a large open operation with significant perioperative morbidity. The potentially beneficial effect of this new treatment approach was evaluated in the course of this study. PMID:10875224

  3. Selective Arterial Embolization of Idiopathic Priapism

    SciTech Connect

    Cohen, Gary S.; Braunstein, Larry; Ball, David S.; Roberto, Paul J.; Reich, Jeffrey; Hanno, Phillip

    1996-11-15

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

  4. Idiopathic Arterial Calcification of Infancy: Case Report

    PubMed Central

    Attia, Tarek Hamed; Abd Alhamed, Mohamed Maisara; Selim, Mohamed Fouad; Haggag, Mohamed Salah; Fathalla, Diaa

    2015-01-01

    Idiopathic arterial calcification of infancy is a rare autosomal recessive disease, characterized by deposition of calcium along the internal elastic membrane of arteries, accompanied by fibrous thickening of the intima which causes luminal narrowing. Here we are reporting a case of idiopathic arterial calcification of infancy in a Saudi female newborn of non-consanguineous pregnant woman who had polyhydramnios. The newborn baby had severe respiratory distress, systemic hypertension and persistent pulmonary hypertension of newborn. She was admitted to Neonatal Intensive Care Unit, where she was ventilated and proper treatment was provided. Molecular genetic testing was positive for mutations of ectonucleotide pyrophosphatase/phosphodiesterase1 gene which is reported in 80% of cases of Idiopathic arterial calcification of infancy. The baby died at about 5 month of age because of myocardial ischemia and cardiorespiratory arrest. Idiopathic Arterial Calcification of Infancy should be considered in any newborn who presented with persistent pulmonary hypertension of newborn, severe systemic hypertension and echogenic vessels on any radiological study. Calcifications of large and medium-sized arteries are important diagnostic finding. PMID:27252793

  5. Idiopathic Aortic Root to Right Atrial Fistula.

    PubMed

    Campisi, Salvatore; Cluzel, Armand; Vola, Marco; Fuzellier, Jean Francois

    2016-06-01

    An aorta to right atrium fistula is rare. We report a case of idiopathic aortic root to right atrial fistula with right heart failure and review the literature. doi: 10.1111/jocs.12751 (J Card Surg 2016;31:373-375). PMID:27109166

  6. Mineral Oil Aspiration Related Juvenile Idiopathic Arthritis

    PubMed Central

    Nelson, Andrew D.; Fischer, Philip R.; Reed, Ann M.; Wylam, Mark E.

    2015-01-01

    We describe the development of rheumatoid factor-positive migratory polyarthritis in a 5-year-old male who had been administered bidaily oral mineral oil as a laxative since birth. Minor respiratory symptoms, radiographic and bronchoscopic findings were consistent with chronic lipoid pneumonia. We speculate that immune sensitization to mineral oil promoted the clinical syndrome of juvenile idiopathic arthritis. PMID:26171269

  7. Mineral Oil Aspiration Related Juvenile Idiopathic Arthritis.

    PubMed

    Nelson, Andrew D; Fischer, Philip R; Reed, Ann M; Wylam, Mark E

    2015-01-01

    We describe the development of rheumatoid factor-positive migratory polyarthritis in a 5-year-old male who had been administered bidaily oral mineral oil as a laxative since birth. Minor respiratory symptoms, radiographic and bronchoscopic findings were consistent with chronic lipoid pneumonia. We speculate that immune sensitization to mineral oil promoted the clinical syndrome of juvenile idiopathic arthritis. PMID:26171269

  8. Idiopathic Arterial Calcification of Infancy: Case Report.

    PubMed

    Attia, Tarek Hamed; Abd Alhamed, Mohamed Maisara; Selim, Mohamed Fouad; Haggag, Mohamed Salah; Fathalla, Diaa

    2015-11-01

    Idiopathic arterial calcification of infancy is a rare autosomal recessive disease, characterized by deposition of calcium along the internal elastic membrane of arteries, accompanied by fibrous thickening of the intima which causes luminal narrowing. Here we are reporting a case of idiopathic arterial calcification of infancy in a Saudi female newborn of non-consanguineous pregnant woman who had polyhydramnios. The newborn baby had severe respiratory distress, systemic hypertension and persistent pulmonary hypertension of newborn. She was admitted to Neonatal Intensive Care Unit, where she was ventilated and proper treatment was provided. Molecular genetic testing was positive for mutations of ectonucleotide pyrophosphatase/phosphodiesterase1 gene which is reported in 80% of cases of Idiopathic arterial calcification of infancy. The baby died at about 5 month of age because of myocardial ischemia and cardiorespiratory arrest. Idiopathic Arterial Calcification of Infancy should be considered in any newborn who presented with persistent pulmonary hypertension of newborn, severe systemic hypertension and echogenic vessels on any radiological study. Calcifications of large and medium-sized arteries are important diagnostic finding. PMID:27252793

  9. Imaging diagnosis--canine thoracic mesothelioma.

    PubMed

    Echandi, Rita L; Morandi, Federica; Newman, Shelley J; Holford, Amy

    2007-01-01

    A 12-year-old neutered female Pembroke Welsh Corgi had a 2-month history of a progressive, productive cough nonresponsive to therapy. Mild pleural effusion, right middle lung lobe collapse, and multiple subpleural nodular lesions were detected in thoracic radiographs and computed tomography (CT) images. Histopathologic diagnosis of the pleural nodules was mesothelioma. Mesothelioma should be considered in patients where pleural masses are detected in radiographs or CT images. PMID:17508511

  10. Thoracic Cavernous Lymphangioma Provoking Massive Chyloptysis

    PubMed Central

    Ferguson, Robert; Hodges, Jeffrey; Harness-Brumley, Cayce; Girod, Carlos; Bartolome, Sonja

    2013-01-01

    Chyloptysis is a relatively rare embodiment of disease that encompasses a lengthy differential and provides many diagnostic and therapeutic challenges. Presented here is the case of a young woman with massive chyloptysis due to a thoracic cavernous lymphangioma arising in the peripartum period. The severity of her condition mandated the use of cardiopulmonary bypass to resect her lymphangioma. We believe that the extent of her symptoms, etiology of disease, and surgical management represent a unique scenario in the literature. PMID:26425583

  11. Managing Dissections of the Thoracic Aorta

    PubMed Central

    WONG, DANIEL R.; LEMAIRE, SCOTT A.; COSELLI, JOSEPH S.

    2010-01-01

    Thoracic aortic dissection is associated with substantial morbidity and mortality, and it requires timely and accurate diagnosis and treatment. Long-term antihypertensive therapy remains critical for the treatment of this disease. Surgical intervention, although still a formidable undertaking, has evolved to better address both acute and chronic dissection, and the results have improved. Basic and clinical research, as well as technological advances, have increased our understanding of this challenging disease state. PMID:18481490

  12. Current Trend of Robotic Thoracic and Cardiovascular Surgeries in Korea: Analysis of Seven-Year National Data

    PubMed Central

    Kang, Chang Hyun; Bok, Jin San; Lee, Na Rae; Kim, Young Tae; Lee, Seon Heui; Lim, Cheong

    2015-01-01

    Background Robotic surgery is an alternative to minimally invasive surgery. The aim of this study was to report on current trends in robotic thoracic and cardiovascular surgical techniques in Korea. Methods Data from the National Evidence-based Healthcare Collaborating Agency (NECA) between January 2006 and June 2012 were used in this study, including a total of 932 cases of robotic surgeries reported to NECA. The annual trends in the case volume, indications for robotic surgery, and distribution by hospitals and surgeons were analyzed in this study. Results Of the 932 cases, 591 (63%) were thoracic operations and 340 (37%) were cardiac operations. The case number increased explosively in 2007 and 2008. However, the rate of increase regained a steady state after 2011. The main indications for robotic thoracic surgery were pulmonary disease (n=271, 46%), esophageal disease (n=199, 34%), and mediastinal disease (n=117, 20%). The main indications for robotic cardiac surgery were valvular heart disease (n=228, 67%), atrial septal defect (n=79, 23%), and cardiac myxoma (n=27, 8%). Robotic thoracic and cardiovascular surgeries were performed in 19 hospitals. Three large volume hospitals performed 94% of the case volume of robotic cardiac surgery and 74% of robotic thoracic surgery. Centralization of robotic operation was significantly (p<0.0001) more common in cardiac surgery than in thoracic surgery. A total of 39 surgeons performed robotic surgeries. However, only 27% of cardiac surgeons and 23% of thoracic surgeons performed more than 10 cases of robotic surgery. Conclusion Trend analysis of robotic and cardiovascular operations demonstrated a gradual increase in the surgical volume in Korea. Meanwhile, centralization of surgical cases toward specific surgeons in specific hospitals was observed. PMID:26509124

  13. Presentation of parathyroid adenoma with genu valgum and thoracic deformities.

    PubMed

    Zil-E-Ali, Ahsan; Latif, Aiza; Rashid, Anam; Malik, Asim; Khan, Haseeb Ahmed

    2016-01-01

    Parathyroid adenoma is the main cause of primary hyperparathyroidism. It is usually asymptomatic and occurs more commonly in adults. It presents with raised parathormone (PTH) and Ca+ levels in serum. Its presentation in adolescence is rare. We report one such incidence of a 14 years old girl who presented with bone pains short stature, and generalized muscle wasting. She was found to have genu valgum at the knee joint, pectus carniatum, scoliosis and cystic changes in pelvis and calvarium. Biochemical investigations and parathyroid Tc-99mMIBI scan confirmed the diagnosis of a parathyroid adenoma. The gland was removed by parathyroidectomy. Till date 12 such cases are reported and none had thoracic, vertebral or calvarium involvement. PMID:26712192

  14. A Decade of Discovery in the Genetic Understanding of Thoracic Aortic Disease.

    PubMed

    Andelfinger, Gregor; Loeys, Bart; Dietz, Hal

    2016-01-01

    Aortic aneurysms are responsible for a significant number of all deaths in Western countries. In this review we provide a perspective on the important progress made over the past decade in the understanding of the genetics of this condition, with an emphasis on the more frequent forms of vascular smooth muscle and transforming growth factor β (TGF-β) signalling alterations. For several nonsyndromic and syndromic forms of thoracic aortic disease, a genetic basis has now been identified, with 3 main pathomechanisms that have emerged: perturbation of the TGF-β signalling pathway, disruption of the vascular smooth muscle cell (VSMC) contractile apparatus, and impairment of extracellular matrix synthesis. Because smooth muscle cells and proteins of the extracellular matrix directly regulate TGF-β signalling, this latter pathway emerges as a key component of thoracic aortic disease initiation and progression. These discoveries have revolutionized our understanding of thoracic aortic disease and provided inroads toward gene-specific stratification of treatment. Last, we outline how these genetic findings are translated into novel pharmaceutical approaches for thoracic aortic disease. PMID:26724507

  15. Main Report

    PubMed Central

    2006-01-01

    scientific literature. The criteria were distributed among three main categories for each condition: The availability and characteristics of the screening test;The availability and complexity of diagnostic services; andThe availability and efficacy of treatments related to the conditions. A survey process utilizing a data collection instrument was used to gather expert opinion on the conditions in the first tier of the assessment. The data collection format and survey provided the opportunity to quantify expert opinion and to obtain the views of a diverse set of interest groups (necessary due to the subjective nature of some of the criteria). Statistical analysis of data produced a score for each condition, which determined its ranking and initial placement in one of three categories (high scoring, moderately scoring, or low scoring/absence of a newborn screening test). In the second tier of these analyses, the evidence base related to each condition was assessed in depth (e.g., via systematic reviews of reference lists including MedLine, PubMed and others; books; Internet searches; professional guidelines; clinical evidence; and cost/economic evidence and modeling). The fact sheets reflecting these analyses were evaluated by at least two acknowledged experts for each condition. These experts assessed the data and the associated references related to each criterion and provided corrections where appropriate, assigned a value to the level of evidence and the quality of the studies that established the evidence base, and determined whether there were significant variances from the survey data. Survey results were subsequently realigned with the evidence obtained from the scientific literature during the second-tier analysis for all objective criteria, based on input from at least three acknowledged experts in each condition. The information from these two tiers of assessment was then considered with regard to the overriding principles and other technology or condition

  16. CT and MRI in the Evaluation of Thoracic Aortic Diseases

    PubMed Central

    2013-01-01

    Computed tomography (CT) and magnetic resonance imaging (MRI) are the most commonly used imaging examinations to evaluate thoracic aortic diseases because of their high spatial and temporal resolutions, large fields of view, and multiplanar imaging reconstruction capabilities. CT and MRI play an important role not only in the diagnosis of thoracic aortic disease but also in the preoperative assessment and followup after treatment. In this review, the CT and MRI appearances of various acquired thoracic aortic conditions are described and illustrated. PMID:24396601

  17. Thoracic Endovascular Stent Graft Repair of Middle Aortic Syndrome.

    PubMed

    Kim, Joung Taek; Lee, Mina; Kim, Young Sam; Yoon, Yong Han; Baek, Wan Ki

    2016-09-01

    Middle aortic syndrome is a rare disease defined as a segmental narrowing of the distal descending thoracic or abdominal aorta. A thoracoabdominal bypass or endovascular treatment is the choice of treatment. Endovascular therapy consists of a balloon dilatation and stent implantation. Recently, thoracic endovascular aortic repair has been widely used in a variety of aortic diseases. We report a case of middle aortic syndrome treated with a thoracic endovascular stent graft. PMID:27549552

  18. Diffuse idiopathic skeletal hyperostosis in ancient clergymen

    PubMed Central

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

    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 are present. The etiology of DISH is unknown but previous studies have shown a strong association with obesity and insulin-independent diabetes mellitus. DISH can lead to back pain, dysphagia, myelopathy, musculoskeletal impairment and grossly unstable spine fractures after minor trauma. In archeological studies a high prevalence of DISH has been demonstrated in ancient clergymen. The present study describes the pathological changes of human remains excavated from the abbey court (Pandhof) in the city of Maastricht, The Netherlands. Human remains of 51 individuals buried between 275 and 1795 ce were excavated and examined. The remains were investigated according to a standardized physical anthropological report and individuals demonstrating ossification of spinal ligaments and/or multiple peripheral enthesopathies were included in the study group. The authors reviewed all available material and after reaching consensus, each abnormality found was given a diagnosis and subsequently recorded. After examination, 28 individuals were considered to be adult males; 11 adult females; three adults of indeterminate sex and nine individuals were of sub adult age. The mean age at death for adults was 36.8 years. Seventeen adult individuals (40.4% of all adults), displayed ossifications of at least four contiguous spinal levels and/or multiple enthesopathies of the appendicular skeleton and were therefore, assigned the diagnosis DISH. The mean age of these individuals was 49.5 ± 13.0 years. In at least three of these individuals, DISH had led to extensive ossification and subsequent ankylosis of axial and peripheral skeletal structures. In this population of (presumably

  19. Idiopathic Calcinosis Cutis of the Penis

    PubMed Central

    Tschen, Jaime A.

    2012-01-01

    Background: Calcinosis cutis—the deposition of insoluble calcium salts in the skin and the soft tissue—occurs in the following five settings: calciphylaxis, dystrophic, iatrogenic, idiopathic, and metastatic. Idiopathic calcinosis cutis of the penis is rare. Purpose: This paper describes a man with idiopathic calcinosis cutis of the penis, summarizes the clinical features of previously reported men with this condition, and also reviews dystrophic, iatrogenic, and metastatic penile calcinosis. Methods: A 27-year-old Pakistan man presented with concurrent, asymptomatic, individual nodules on the right mid-ventral penile shaft and left side of scrotum and two additional papules on the right side of the scrotum. Evaluation and treatment included the excision of all lesions. Reports of patients with penile calcinosis were identified using a medical search engine (PubMed Central) and referenced citations from the published papers on this subject. Results: Microscopic examination of the patient's nodules showed idiopathic and dystrophic calcinosis cutis of the penis and scrotum, respectively; the scrotal papules were fibroepithelial polyps. Including this individual, idiopathic calcinosis cutis of the penis has only been reported in 11 men. It presents as either an asymptomatic nodule (5 patients) or multiple lesions (6 patients) of less than one-year duration, on either the penile shaft (distal in 4 patients, mid in 2 patients, both in 1 patient, and site unspecified in 1 patient) or the prepuce (3 patients) of uncircumcised men less than 30 years of age. Concurrent scrotal calcification was noted in two patients. Dermal deposits of calcium are found in the dermis—often with surrounding histiocytes and multinucleated giant cells; concurrent features of dystrophic penile shaft calcification, such as calcium within syringomas or transepidermal elimination of calcium through eccrine sweat ducts, was only noted in two men. The nodules do not recur following excision

  20. Treatment of uveitis associated with juvenile idiopathic arthritis.

    PubMed

    Bou, Rosa; Iglesias, Estíbaliz; Antón, Jordi

    2014-08-01

    Chronic anterior uveitis affects 10-30 % of patients with juvenile idiopathic arthritis (JIA) and is still a cause of blindness in childhood. In most patients it is asymptomatic, bilateral, and recurrent, so careful screening and early diagnosis are important to obtain the best long-term prognosis. The treatment of chronic uveitis associated with JIA is challenging. Initial treatment is based on topical steroids and mydriatic drops. Methotrexate is the most common first-line immunomodulatory drug used. For refractory patients, biologicals, mainly the anti-tumor-necrosis-factor (TNF) drugs adalimumab and infliximab, have been revealed to be effective and have changed the outcome for these patients. Collaboration between pediatric rheumatologists and ophthalmologists is important for the successful diagnosis and treatment of patients with uveitis associated with JIA. PMID:24938442

  1. Extraskeletal symptoms and comorbidities of diffuse idiopathic skeletal hyperostosis

    PubMed Central

    Terzi, Rabia

    2014-01-01

    Diffuse idiopathic skeletal hyperostosis (DISH) is a non-inflammatory disease characterized by calcification and ossification of soft tissues, mainly enthesis and spinal ligaments. The clinical presentation primarily includes spinal involvement-induced pain and range of motion. Although rare, life-threatening gastrointestinal, respiratory or neurological events or severe morbidity due to bone compression on the adjacent structures may develop. There is a limited amount of data on DISH-related events in the literature. In recent years, comorbid metabolic disorders are of great interest in patients with DISH. The early diagnosis of these conditions as well as rare entities allows an effective multidisciplinary approach for the treatment of DISH. In this article, we review extraskeletal symptoms and associated comorbidities in patients with DISH. PMID:25232544

  2. Idiopathic Systemic Capillary Leak Syndrome: A Case Report

    PubMed Central

    Yardimci, Bulent; Kazancioglu, Rumeyza

    2016-01-01

    Introduction Idiopathic systemic capillary leak syndrome (ISCLS) is rarely seen, and presents with recurrent episodes of hypotension, shock, hemoconcentration, and hypoproteinemia. The main pathology is the dysfunction of the vascular endothelium, and it is characterized by an increase of capillary permeability that is accompanied by the loss of intravascular fluid and protein. Case Presentation We present a 58-year-old female who presented with peripheral edema, leg pain, and syncope at the emergency department. Interestingly demyemilising neuropathy, which is a rare finding, ensued on day 4. She is still being treated using intravenous immunoglobulin therapy. Conclusions The early signs and symptoms of ISCLS may be subtle; therefore the diagnosis can easily be missed and prompt treatment of the syndrome may be postponed. Thus, the clinician must consider ISCLS in differential diagnosis in cases of hypotension, hemoconcentration, and hypoalbuminemia. PMID:27195144

  3. Extraskeletal symptoms and comorbidities of diffuse idiopathic skeletal hyperostosis.

    PubMed

    Terzi, Rabia

    2014-09-16

    Diffuse idiopathic skeletal hyperostosis (DISH) is a non-inflammatory disease characterized by calcification and ossification of soft tissues, mainly enthesis and spinal ligaments. The clinical presentation primarily includes spinal involvement-induced pain and range of motion. Although rare, life-threatening gastrointestinal, respiratory or neurological events or severe morbidity due to bone compression on the adjacent structures may develop. There is a limited amount of data on DISH-related events in the literature. In recent years, comorbid metabolic disorders are of great interest in patients with DISH. The early diagnosis of these conditions as well as rare entities allows an effective multidisciplinary approach for the treatment of DISH. In this article, we review extraskeletal symptoms and associated comorbidities in patients with DISH. PMID:25232544

  4. Favourable outcome in idiopathic ventricular fibrillation with treatment aimed at prevention of high sympathetic tone and suppression of inducible arrhythmias.

    PubMed Central

    Crijns, H. J.; Wiesfeld, A. C.; Posma, J. L.; Lie, K. I.

    1995-01-01

    OBJECTIVE--In the absence of an obvious cause for cardiac arrest, patients with idiopathic ventricular fibrillation are difficult to manage. A subset of patients has inducible arrhythmias. In others sympathetic excitation plays a role in the onset of the cardiac arrest. This study evaluates a prospective stepped care approach in the management of idiopathic ventricular fibrillation, with therapy first directed at induced arrhythmias and secondly at adrenergic trigger events. SETTING--University Hospital. PATIENTS--10 consecutive patients successfully resuscitated from idiopathic ventricular fibrillation. INTERVENTIONS--Programmed electrical stimulation to determine inducibility, followed by serial drug treatment. Assessment of pre-arrest physical activity and mental stress status by interview, followed by beta blockade. Cardioverter-defibrillator implantation in non-inducible patients not showing significant arrest related sympathetic excitation. MAIN OUTCOME MEASURE--Recurrent cardiac arrest or ventricular tachycardia. RESULTS--Five patients were managed with serial drug treatment and four with beta blockade. In one patient a defibrillator was implanted. During a median follow up of 2.8 years (range 6 to 112 months) no patient died or experienced defibrillator shocks. One patient had a recurrence of a well tolerated ventricular tachycardia on disopyramide. CONCLUSIONS--Idiopathic ventricular fibrillation may be related to enhanced sympathetic activation. Prognosis may be favourable irrespective of the method of treatment. Whether the present approach enhances prognosis of idiopathic ventricular fibrillation remains to be determined. However, it may help to avoid potentially hazardous antiarrhythmic drugs or obviate the need for implantation of cardioverter-defibrillators. PMID:7488456

  5. Idiopathic noncirrhotic portal hypertension: current perspectives.

    PubMed

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

    2016-01-01

    The term idiopathic noncirrhotic portal hypertension (INCPH) has been recently proposed to replace terms, such as hepatoportal sclerosis, idiopathic portal hypertension, incomplete septal cirrhosis, and nodular regenerative hyperplasia, used to describe patients with a hepatic presinusoidal cause of portal hypertension of unknown etiology, characterized by features of portal hypertension (esophageal varices, nonmalignant ascites, porto-venous collaterals), splenomegaly, patent portal, and hepatic veins and no clinical and histological signs of cirrhosis. Physicians should learn to look for this condition in a number of clinical settings, including cryptogenic cirrhosis, a disease known to be associated with INCPH, drug administration, and even chronic alterations in liver function tests. Once INCPH is clinically suspected, liver histology becomes mandatory for the correct diagnosis. However, pathologists should be familiar with the histological features of INCPH, especially in cases in which histology is not only requested to exclude liver cirrhosis. PMID:27555800

  6. Genetic Aspects of Congenital and Idiopathic Scoliosis

    PubMed Central

    Giampietro, Philip F.

    2012-01-01

    Congenital and idiopathic scoliosis represent disabling conditions of the spine. While congenital scoliosis (CS) is caused by morphogenic abnormalities in vertebral development, the cause(s) for idiopathic scoliosis is (are) likely to be varied, representing alterations in skeletal growth, neuromuscular imbalances, disturbances involving communication between the brain and spine, and others. Both conditions are characterized by phenotypic and genetic heterogeneities, which contribute to the difficulties in understanding their genetic basis that investigators face. Despite the differences between these two conditions there is observational and experimental evidence supporting common genetic mechanisms. This paper focuses on the clinical features of both CS and IS and highlights genetic and environmental factors which contribute to their occurrence. It is anticipated that emerging genetic technologies and improvements in phenotypic stratification of both conditions will facilitate improved understanding of the genetic basis for these conditions and enable targeted prevention and treatment strategies. PMID:24278672

  7. Acute idiopathic polyneuropathy in the dog.

    PubMed

    Northington, J W; Brown, M J; Farnbach, G C; Steinberg, S A

    1981-08-15

    From among a large group of dogs with acute tetraparesis, we identified 10 dogs with a distinct peripheral nerve disorder. Prior to the onset of signs, all of the dogs had been healthy, and none was known to have been exposed to a neurotoxin or raccoon bite. Weakness, with hypoactive or absent segmental reflexes, became progressively worse for 1 to 21 days. Results of electromyography and nerve conduction studies invariably were compatible with a diagnosis of polyneuropathy that predominantly affected proximal nerve segments. Appearance of nerve biopsy specimens and the short time course for functional recovery suggested a demyelinative component to the disorder. The extent of recovery was variable but often rapid and complete in dogs that did not succumb to complications in the early period. Corticosteroid therapy did not demonstrably influence the outcome. This acute idiopathic polyneuropathy in the dog shares many clinical and pathologic features with idiopathic polyradiculoneuritis (Coonhound paralysis). PMID:6270046

  8. Management of severe and rigid idiopathic scoliosis.

    PubMed

    Teixeira da Silva, Luis Eduardo Carelli; de Barros, Alderico Girão Campos; de Azevedo, Gustavo Borges Laurindo

    2015-07-01

    Frequently, severe idiopathic scoliosis patients are first seen in a spine centre after years of deformity evolution, presenting with large curves, severe rib hump, shoulder and trunk imbalance and cardiorespiratory complications related to neglected scoliosis. Severe rigid idiopathic scoliosis has <25% of correction on bending films and major curve over 90°. Adequate mobilization of this type of deformity is necessary to achieve maximal correction, often requiring more extensive surgical intervention, with care taken to avoid clinical and neurological complications. Halo traction, internal temporary distraction, releases, osteotomies and apical vertebral resection are often used in combination to achieve optimal results. Indications must be tailored by surgeons considering resources, deformity characteristics and patient's profile. Vertebral resection procedures may have potential neurological and clinical risks and should be one of the last treatment options performed by experienced surgical team. Neuromonitoring is essential during these procedures. PMID:26033753

  9. Idiopathic head tremor in English bulldogs.

    PubMed

    Guevar, Julien; De Decker, Steven; Van Ham, Luc M L; Fischer, Andrea; Volk, Holger A

    2014-02-01

    Idiopathic head tremor (IHT) syndrome is a recognized but poorly characterized movement disorder in English bulldogs (EBs). The data analyzed were collected via a detailed online questionnaire and video recordings. Thirty-eight percent of the population demonstrated IHT. The first presentation was early in life. There was no sex or neutered status predisposition. The condition disappeared with time in 50% of the cases. The direction of the head movement was vertical or horizontal. The number of episodes per day and the duration of the episodes were greatly variable. The majority of episodes occurred at rest. Most of the episodes were unpredictable. And there was no alteration of the mental status for most dogs during the episodes. Stress has been reported as a suspected trigger factor. IHT in EBs can be considered an idiopathic paroxysmal movement disorder. PMID:24375621

  10. [Juvenile idiopathic arthritis: Definition and classification].

    PubMed

    Deslandre, C

    2016-04-01

    Juvenile idiopathic arthritis (JIA) is a group of diseases defined by the presence of arthritis of more than 6weeks duration in patients aged less than 16years and with unknown etiology. The international classification based on clinical and biological criteria define each type of JIA: systemic, oligoarticular, polyarticular with and without rheumatoid factor, enthesitis-related arthritis, and psoriatic arthritis. However, some discussions persist concerning systemic-onset juvenile idiopathic arthritis, whose clinical symptoms and pathogenic mechanisms are quite similar to those observed in autoinflammatory diseases, arthritis with antinuclear factors (poly- and oligoarticular) that could be considered as a homogenous group, and a family history of psoriasis that frequently led to unclassified arthritis. Better knowledge of the pathogenic mechanisms should improve the initial clinical classification with more homogeneous groups of patients and reduce the number of unclassified cases of arthritis. PMID:26968301

  11. Idiopathic noncirrhotic portal hypertension: current perspectives

    PubMed Central

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

    2016-01-01

    The term idiopathic noncirrhotic portal hypertension (INCPH) has been recently proposed to replace terms, such as hepatoportal sclerosis, idiopathic portal hypertension, incomplete septal cirrhosis, and nodular regenerative hyperplasia, used to describe patients with a hepatic presinusoidal cause of portal hypertension of unknown etiology, characterized by features of portal hypertension (esophageal varices, nonmalignant ascites, porto-venous collaterals), splenomegaly, patent portal, and hepatic veins and no clinical and histological signs of cirrhosis. Physicians should learn to look for this condition in a number of clinical settings, including cryptogenic cirrhosis, a disease known to be associated with INCPH, drug administration, and even chronic alterations in liver function tests. Once INCPH is clinically suspected, liver histology becomes mandatory for the correct diagnosis. However, pathologists should be familiar with the histological features of INCPH, especially in cases in which histology is not only requested to exclude liver cirrhosis. PMID:27555800

  12. Video-Assisted Thoracic Sympathectomy for Hyperhidrosis.

    PubMed

    Milanez de Campos, Jose Ribas; Kauffman, Paulo; Gomes, Oswaldo; Wolosker, Nelson

    2016-08-01

    By the 1980s, endoscopy was in use by some groups in sympathetic denervation of the upper limbs with vascular indications. Low morbidity, cosmetic results, reduction in the incidence of Horner syndrome, and the shortened time in hospital made video-assisted thoracic sympathectomy (VATS) better accepted by those undergoing treatment for hyperhidrosis. Over the last 25 years, this surgical procedure has become routine in the treatment of hyperhidrosis, leading to a significant increase in the number of papers on the subject in the literature. PMID:27427529

  13. Thoracic Outlet Syndrome Following Breast Implant Rupture

    PubMed Central

    Caplash, Yugesh; Giri, Pratyush; Kearney, Daniel; Wagstaff, Marcus

    2015-01-01

    Summary: We present a patient with bilateral breast implant rupture who developed severe locoregional silicone granulomatous lymphadenopathy. Poly Implant Prothese silicone implants had been used for bilateral breast augmentation 5 years prior. Extracapsular implant rupture and bilateral axillary lymphadenopathy indicated explantation, capsulectomy, and selective lymph node excision. Histology demonstrated silicone lymphadenopathy with no evidence of malignancy. Over the subsequent 12 months, she developed progressive locoregional lymphadenopathy involving bilateral cervical, axillary, and internal mammary groups, resulting in bilateral thoracic outlet syndrome. We report the unusual presentation, progression, and the ultimate surgical management of this patient. PMID:25878942

  14. Dynamic thoracoplasty for asphyxiating thoracic dystrophy.

    PubMed

    Kaddoura, I L; Obeid, M Y; Mroueh, S M; Nasser, A A

    2001-11-01

    The life-saving procedures to expand the chests of infants born with Jeune asphyxiating thoracic dystrophy provide a static solution incapable of responding to the growth demands of thriving patients. We describe an instrument that provided a dynamic solution for an infant, where an initial methyl methacrylate midsternotomy spacer placed at 4 months of age was followed at 11 months with recurrence of his difficulties. At 8 months after the second operation the patient was stable and thriving with no recurrence of symptoms. The instrument modifications, limitations, and possible complications are described. PMID:11722089

  15. Video-assisted thoracic surgery complications

    PubMed Central

    Kozak, Józef

    2014-01-01

    Video-assisted thoracic surgery (VATS) is a miniinvasive technique commonly applied worldwide. Indications for VATS are very broad and include the diagnosis of mediastinal, lung and pleural diseases, as well as large resection procedures such as pneumonectomy. The most frequent complication is prolonged postoperative air leak. The other significant complications are bleeding, infections, postoperative pain and recurrence at the port site. Different complications of VATS procedures can occur with variable frequency in various diseases. Despite the large number of their types, such complications are rare and can be avoided through the proper selection of patients and an appropriate surgical technique. PMID:25561984

  16. Levocetirizine for chronic idiopathic urticaria: a review.

    PubMed

    Ducharme, Erin E; Weinberg, Jeffrey M

    2009-03-01

    Only recently available on the US market, levocetirizine has emerged as an effective new option in the treatment of chronic idiopathic urticaria (CIU). Levocetirizine is the active isomer of cetirizine and demonstrates twice the affinity for the H1 receptor compared with the parent compound. In the treatment of CIU, levocetirizine has consistently demonstrated high response rates, fast onset, and a favorable side effect profile. PMID:19271371

  17. [Persistent idiopathic facial pain and atypical odontalgia].

    PubMed

    Gaul, Charly; Ettlin, Dominik; Pfau, Doreen B

    2013-01-01

    The terms 'persistent idiopathic facial pain' (PIFP) and 'atypical odontalgia' (AO) are currently used as exclusion diagnoses for chronic toothache and chronic facial pain. Knowledge about these pain conditions in medical and dental practices is of crucial importance for the prevention of iatrogenic tissue damage by not-indicated invasive interventions, such as endodontic treatment and tooth extraction. In the present paper, etiology and pathogenesis, differential diagnostic criteria, and diagnostic approaches will be explained and relevant therapeutic principles will be outlined. PMID:23916270

  18. Clomiphene Effects on Idiopathic Premature Ejaculation

    PubMed Central

    Ketabchi, Ali Asghar

    2015-01-01

    Background: Premature ejaculation (PE) is the inability to delay ejaculation, occurring sooner than they or their partner would like during sexual activities. PE is a challenging problem that can affect sexual enjoyment and may harm relationships of couples and affect their quality of life. In idiopathic PE, several helpful techniques and medicines are recommended, but none of them has yielded satisfactory results. Objectives: Our objective in this study was to evaluate the efficacy and safety of clomiphene as a selective estrogen receptor modulator on the treatment of idiopathic PE. Patients and Methods: In a randomized clinical trial, 178 married men with idiopathic PE defined according to the Diagnostic and Statistical Manual of Mental Disorders Third Revised Version (DSM-III-R) who referred to urology clinics over a 10-month period in 2012 were randomized into two groups, namely the study (clomiphene) and control (placebo) groups. They completed self-administered questionnaires that included intravaginal ejaculatory latency time (IELT), erectile dysfunction indexes, quality of life (QOL), sociodemographic characteristics, lifestyle, and medical illness. After 6 months of intervention, all data were compared with the baseline data and between the groups. Results: Within the 10-month study course, 126 patients (70.8%) completed this study. After intervention and comparison of the results between the two groups, IELT, sexual indexes, and QOL improved in the study group, but significant differences were observed only in the IELT and QOL findings. Conclusions: Clomiphene seems to be useful in the pharmacological treatment of PE compared to the placebo. PMID:26543830

  19. Interventional Radiologic Treatment for Idiopathic Portal Hypertension

    SciTech Connect

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

    1999-07-15

    Purpose: To evaluate the usefulness of interventional radiological treatment for idiopathic portal hypertension. Methods: Between 1995 and 1998, we performed an interventional radiological treatment in five patients with idiopathic portal hypertension, four of whom had refused surgery and one of whom had undergone surgery. Three patients with gastroesophageal varices (GEV) were treated by partial splenic embolization (PSE), one patient with esophageal varices (EV) and massive ascites by transjugular intrahepatic portosytemic shunt (TIPS) and PSE, and one patient with GEV by percutaneous transhepatic obliteration (PTO). Midterm results were analyzed in terms of the effect on esophageal and/or gastric varices. Results: 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.

  20. Pulmonary function in children with idiopathic scoliosis

    PubMed Central

    2012-01-01

    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. PMID:22445133

  1. Thoracic Paravertebral Block, Multimodal Analgesia, and Monitored Anesthesia Care for Breast Cancer Surgery in Primary Lateral Sclerosis

    PubMed Central

    Fernandes, Anthony

    2016-01-01

    Objective. Primary lateral sclerosis (PLS) is a rare idiopathic neurodegenerative disorder affecting upper motor neurons and characterized by spasticity, muscle weakness, and bulbar involvement. It can sometimes mimic early stage of more common and fatal amyotrophic lateral sclerosis (ALS). Surgical patients with a history of neurodegenerative disorders, including PLS, may be at increased risk for general anesthesia related ventilatory depression and postoperative respiratory complications, abnormal response to muscle relaxants, and sensitivity to opioids, sedatives, and local anesthetics. We present a case of a patient with PLS and recent diagnosis of breast cancer who underwent a simple mastectomy surgery uneventfully under an ultrasound guided thoracic paravertebral block, multimodal analgesia, and monitored anesthesia care. Patient reported minimal to no pain or discomfort in the postoperative period and received no opioids for pain management before being discharged home. In patients with PLS, thoracic paravertebral block and multimodal analgesia can provide reliable anesthesia and effective analgesia for breast surgery with avoidance of potential risks associated with general anesthesia, muscle paralysis, and opioid use. PMID:27200193

  2. The correlation between calcaneal valgus angle and asymmetrical thoracic-lumbar rotation angles in patients with adolescent scoliosis.

    PubMed

    Park, Jaeyong; Lee, Sang Gil; Bae, Jongjin; Lee, Jung Chul

    2015-12-01

    [Purpose] This study aimed to provide a predictable evaluation method for the progression of scoliosis in adolescents based on quick and reliable measurements using the naked eye, such as the calcaneal valgus angle of the foot, which can be performed at public facilities such as schools. [Subjects and Methods] Idiopathic scoliosis patients with a Cobb's angle of 10° or more (96 females, 22 males) were included in this study. To identify relationships between factors, Pearson's product-moment correlation coefficient was computed. The degree of scoliosis was set as a dependent variable to predict thoracic and lumbar scoliosis using ankle angle and physique factors. Height, weight, and left and right calcaneal valgus angles were set as independent variables; thereafter, multiple regression analysis was performed. This study extracted variables at a significance level (α) of 0.05 by applying a stepwise method, and calculated a regression equation. [Results] Negative correlation (R=-0.266) was shown between lumbar lordosis and asymmetrical lumbar rotation angles. A correlation (R=0.281) was also demonstrated between left calcaneal valgus angles and asymmetrical thoracic rotation angles. [Conclusion] Prediction of scoliosis progress was revealed to be possible through ocular inspection of the calcaneus and Adams forward bending test and the use of a scoliometer. PMID:26834376

  3. Low-tech rehabilitation and management of a 64 year old male patient with acute idiopathic onset of costochondritis

    PubMed Central

    Hudes, Karen

    2008-01-01

    Objective This study was conducted to discuss the treatment and management of a patient presenting with acute idiopathic costochondritis. Case 64 year old male patient presenting with acute anterior chest pain of one week duration. Treatment High-velocity low-amplitude thrust manipulation was used to the zygapophyseal joints of the thoracic spine, costotransverse, and costochondral joints involved. Acupuncture, ischemic compression, cross fibre friction massage techniques, and cryotherapy were utilized on the local area of pain. Specific exercise prescription (low tech rehabilitation) was also utilized. Improvement of pain reported on the Visual Analog Scale was noted with a complete resolution of the condition at the conclusion of treatment. No recurrences were reported on an eleven month follow up of the patient. Conclusion Conservative management, including manipulation and exercise prescription, may be beneficial in the treatment of benign costochondritis. PMID:19066696

  4. Thoracic-pelvic dysostosis: a 'new' autosomal dominant form.

    PubMed Central

    Bankier, A; Danks, D M

    1983-01-01

    A form of thoracic and pelvic dysostosis is reported in a mother and her son. The short ribs caused respiratory distress in the baby and raised the possibility of asphyxiating thoracic dystrophy (ATD). The radiological features, however, distinguish this benign condition from ATD and other described skeletal dysplasias. Images PMID:6620328

  5. Anomalous azygos vein: a potential danger during endoscopic thoracic sympathectomy.

    PubMed

    Sieunarine, K; May, J; White, G H; Harris, J P

    1997-08-01

    A report of a patient with an azygos lobe and an associated anomalous azygos vein covering the upper thoracic sympathetic chain. This anomaly poses a significant risk during the procedure of endoscopic thoracic sympathectomy. A chest X-ray is useful in detecting this anomaly and alerting the surgeon to potential problems. PMID:9287933

  6. Surgical efficacy of minimally invasive thoracic discectomy.

    PubMed

    Elhadi, Ali M; Zehri, Aqib H; Zaidi, Hasan A; Almefty, Kaith K; Preul, Mark C; Theodore, Nicholas; Dickman, Curtis A

    2015-11-01

    We aimed to determine the clinical indications and surgical outcomes for thoracoscopic discectomy. Thoracic disc disease is a rare degenerative process. Thoracoscopic approaches serve to minimize tissue injury during the approach, but critics argue that this comes at the cost of surgical efficacy. Current reports in the literature are limited to small institutional patient series. We systematically identified all English language articles on thoracoscopic discectomy with at least two patients, published from 1994 to 2013 on MEDLINE, Science Direct, and Google Scholar. We analyzed 12 articles that met the inclusion criteria, five prospective and seven retrospective studies comprising 545 surgical patients. The overall complication rate was 24% (n=129), with reported complications ranging from intercostal neuralgia (6.1%), atelectasis (2.8%), and pleural effusion (2.6%), to more severe complications such as pneumonia (0.8%), pneumothorax (1.3%), and venous thrombosis (0.2%). The average reported postoperative follow-up was 20.5 months. Complete resolution of symptoms was reported in 79% of patients, improvement with residual symptoms in 10.2%, no change in 9.6%, and worsening in 1.2%. The minimally invasive endoscopic approaches to the thoracic spine among selected patients demonstrate excellent clinical efficacy and acceptable complication rates, comparable to the open approaches. Disc herniations confined to a single level, with small or no calcifications, are ideal for such an approach, whereas patients with calcified discs adherent to the dura would benefit from an open approach. PMID:26206758

  7. Developing the academic thoracic surgeon: teaching surgery.

    PubMed

    Baumgartner, W A; Greene, P S

    2000-04-01

    Teaching surgery can be a very gratifying experience for those of us involved in academic thoracic surgery. Fundamentals of a good residency program require that patients should always be placed in the highest priority. However, the residency program should also be committed to teaching as a priority. Creating the proper operating room environment is essential for optimal conduct of the operation. This environment is similar to that of the airline industry, which is known as crew or cockpit resource management. The design of a teaching program needs to have evaluation as one of its key elements. In addition to resident evaluation, it is also important to have faculty evaluation by the residents. The goal of any residency program should be to foster the development of the future leaders in our specialty. The information contained within this article represents the art and science of teaching thoracic surgery as applied by the faculty in the Division of Cardiac Surgery at The Johns Hopkins Hospital. PMID:10727957

  8. The thoracic anterior spinal cord adhesion syndrome

    PubMed Central

    Taylor, T R; Dineen, R; White, B; Jaspan, T

    2012-01-01

    Objectives This study included a series of middle-aged male and female patients who presented with chronic anterior hemicord dysfunction progressing to paraplegia. Imaging of anterior thoracic cord displacement by either a dural adhesion or a dural defect with associated cord herniation is presented. Methods This is a retrospective review of cases referred to a tertiary neuroscience centre over a 19-year period. Imaging series were classified by two experienced neuroradiologists against several criteria and correlated with clinical examination and/or findings at surgery. Results 16 cases were available for full review. Nine were considered to represent adhesions (four confirmed surgically) and four to represent true herniation (three confirmed surgically). In the three remaining cases the diagnosis was radiologically uncertain. Conclusion The authors propose “thoracic anterior spinal cord adhesion syndrome” as a novel term to describe this patient cohort and suggest appropriate clinicoradiological features for diagnosis. Several possible aetiologies are also suggested, with disc rupture and inflammation followed by disc resorption and dural pocket formation being a possible mechanism predisposing to herniation at the extreme end of a clinicopathological spectrum. PMID:22665931

  9. Severe thoracic impalement injury: Survival in a case with delayed surgical definitive care.

    PubMed

    Lunca, Sorinel; Morosanu, Corneliu; Alexa, Ovidiu; Pertea, Mihaela

    2015-03-01

    Impalement injuries are rare and among the most spectacular and dramatic traumatic lesions. The survival of a patient with a thoracic impalement injury is an extremely rare event. The objective of this study was to present the case of a 24-year-old male patient with a severe thoracic impalement injury successfully treated despite his late arrival in our hospital. A log in 12 cm diameter penetrated his right thorax producing injuries of the right main bronchus, right pulmonary lobe, right subclavian artery as well as extensive parietal lesions. Definitive surgical repair of these lesions was performed more than seven hours after trauma. The management principles contributing to the successful outcome that we would like to emphasize are: rapid transportation and reaction of the trauma team, minimal manipulation of the impaling object, removal of the log as one piece under direct vision in the operating room, ventilatory support, extensive debridement, and lavage associated with appropriate antibiotherapy. PMID:25904279

  10. Anti-TNF therapy for juvenile idiopathic arthritis-related uveitis

    PubMed Central

    Semeraro, Francesco; Arcidiacono, Barbara; Nascimbeni, Giuseppe; Angi, Martina; Parolini, Barbara; Costagliola, Ciro

    2014-01-01

    Juvenile idiopathic arthritis-related uveitis is the most common type of uveitis in childhood and one of the main causes of visual impairment in children. The introduction of biological treatment has widened the range of therapeutic options for children with uveitis refractory to standard nonbiologic immunosuppressants. Data from clinical trials suggest that both adalimumab and infliximab have demonstrated effectiveness and safety in open-label studies, although no large, randomized, controlled trials have been reported so far. The role of etanercept in treating juvenile idiopathic arthritis-related uveitis is not yet well defined. In our experience, anti-tumor necrosis factor therapy has been shown to be more effective than steroids and/or methotrexate in treating uveitis. Up to now, tumor necrosis factor blocking compounds have been reserved for the treatment of the most severe cases of refractory uveitis, and larger prospective clinical trials are required in order to better assess the safety of these new compounds. PMID:24711694

  11. Accuracy of robot-assisted pedicle screw placement for adolescent idiopathic scoliosis in the pediatric population.

    PubMed

    Macke, Jeremy J; Woo, Raymund; Varich, Laura

    2016-06-01

    This is a retrospective review of pedicle screw placement in adolescent idiopathic scoliosis (AIS) patients under 18 years of age who underwent robot-assisted corrective surgery. Our primary objective was to characterize the accuracy of pedicle screw placement with evaluation by computed tomography (CT) after robot-assisted surgery in AIS patients. Screw malposition is the most frequent complication of pedicle screw placement and is more frequent in AIS. Given the potential for serious complications, the need for improved accuracy of screw placement has spurred multiple innovations including robot-assisted guidance devices. No studies to date have evaluated this robot-assisted technique using CT exclusively within the AIS population. Fifty patients were included in the study. All operative procedures were performed at a single institution by a single pediatric orthopedic surgeon. We evaluated the grade of screw breach, the direction of screw breach, and the positioning of the patient for preoperative scan (supine versus prone). Of 662 screws evaluated, 48 screws (7.2 %) demonstrated a breach of greater than 2 mm. With preoperative prone position CT scanning, only 2.4 % of screws were found to have this degree of breach. Medial malposition was found in 3 % of screws, a rate which decreased to 0 % with preoperative prone position scanning. Based on our results, we conclude that the proper use of image-guided robot-assisted surgery can improve the accuracy and safety of thoracic pedicle screw placement in patients with adolescent idiopathic scoliosis. This is the first study to evaluate the accuracy of pedicle screw placement using CT assessment in robot-assisted surgical correction of patients with AIS. In our study, the robot-assisted screw misplacement rate was lower than similarly constructed studies evaluating conventional (non-robot-assisted) procedures. If patients are preoperatively scanned in the prone position, the misplacement rate is further

  12. Thoracic size-selective sampling of fibres: performance of four types of thoracic sampler in laboratory tests.

    PubMed

    Jones, A D; Aitken, R J; Fabriès, J F; Kauffer, E; Liden, G; Maynard, A; Riediger, G; Sahle, W

    2005-08-01

    The counting of fibres on membrane filters could be facilitated by using size-selective samplers to exclude coarse particulate and fibres that impede fibre counting. Furthermore, the use of thoracic size selection would also remove the present requirement to discriminate fibres by diameter during counting. However, before thoracic samplers become acceptable for sampling fibres, their performance with fibres needs to be determined. This study examines the performance of four thoracic samplers: the GK2.69 cyclone, a Modified SIMPEDS cyclone, the CATHIA sampler (inertial separation) and the IOM thoracic sampler (porous foam pre-selector). The uniformity of sample deposit on the filter samples, which is important when counts are taken on random fields, was examined with two sizes of spherical particles (1 and 10 microm) and a glass fibre aerosol with fibres spanning the aerodynamic size range of the thoracic convention. Counts by optical microscopy examined fields on a set scanning pattern. Hotspots of deposition were detected for one of the thoracic samplers (Modified SIMPEDS with the 10 microm particles and the fibres). These hotspots were attributed to the inertial flow pattern near the port from the cyclone pre-separator. For the other three thoracic samplers, the distribution was similar to that on a cowled sampler, the current standard sampler for fibres. Aerodynamic selection was examined by comparing fibre concentration on thoracic samples with those measured on semi-isokinetic samples, using fibre size (and hence calculated aerodynamic diameter) and number data obtained by scanning electron microscope evaluation in four laboratories. The size-selection characteristics of three thoracic samplers (GK2.69, Modified SIMPEDS and CATHIA) appeared very similar to the thoracic convention; there was a slight oversampling (relative to the convention) for d(ae) < 7 microm, but that would not be disadvantageous for comparability with the cowled sampler. Only the IOM

  13. The Prevalence of Idiopathic Scoliosis in Eleven Year-Old Korean Adolescents: A 3 Year Epidemiological Study

    PubMed Central

    Lee, Jin-Young; Moon, Seong-Hwan; Kim, Han Jo; Suh, Bo-Kyung; Nam, Ji Hoon; Jung, Jae Kyun; Lee, Hwan-Mo

    2014-01-01

    Purpose School screening allows for early detection and early treatment of scoliosis, with the purpose of reducing the number of patients requiring surgical treatment. Children between 10 and 14 years old are considered as good candidates for school screening tests of scoliosis. The purpose of the present study was to assess the epidemiological findings of idiopathic scoliosis in 11-year-old Korean adolescents. Materials and Methods A total of 37856 11-year-old adolescents were screened for scoliosis. There were 17110 girls and 20746 boys. Adolescents who were abnormal by Moiré topography were subsequently assessed by standardized clinical and radiological examinations. A scoliotic curve was defined as 10° or more. Results The prevalence of scoliosis was 0.19% and most of the curves were small (10° to 19°). The ratio of boys to girls was 1:5.5 overall. Sixty adolescents (84.5%) exhibited single curvature. Thoracolumbar curves were the most common type of curve identified, followed by thoracic and lumbar curves. Conclusion The prevalence of idiopathic scoliosis among 11-year-old Korean adolescents was 0.19%. PMID:24719147

  14. Effects of Exercise on Spinal Deformities and Quality of Life in Patients with Adolescent Idiopathic Scoliosis

    PubMed Central

    Anwer, Shahnawaz; Alghadir, Ahmad; Abu Shaphe, Md.; Anwar, Dilshad

    2015-01-01

    Objectives. This systematic review was conducted to examine the effects of exercise on spinal deformities and quality of life in patients with adolescent idiopathic scoliosis (AIS). Data Sources. Electronic databases, including PubMed, CINAHL, Embase, Scopus, Cochrane Register of Controlled Trials, PEDro, and Web of Science, were searched for research articles published from the earliest available dates up to May 31, 2015, using the key words “exercise,” “postural correction,” “posture,” “postural curve,” “Cobb's angle,” “quality of life,” and “spinal deformities,” combined with the Medical Subject Heading “scoliosis.” Study Selection. This systematic review was restricted to randomized and nonrandomized controlled trials on AIS published in English language. The quality of selected studies was assessed by the PEDro scale, the Cochrane Collaboration's tool, and the Grading of Recommendations Assessment, Development, and Evaluation System (GRADE). Data Extraction. Descriptive data were collected from each study. The outcome measures of interest were Cobb angle, trunk rotation, thoracic kyphosis, lumbar kyphosis, vertebral rotation, and quality of life. Data Synthesis. A total of 30 studies were assessed for eligibility. Six of the 9 selected studies reached high methodological quality on the PEDro scale. Meta-analysis revealed moderate-quality evidence that exercise interventions reduce the Cobb angle, angle of trunk rotation, thoracic kyphosis, and lumbar lordosis and low-quality evidence that exercise interventions reduce average lateral deviation. Meta-analysis revealed moderate-quality evidence that exercise interventions improve the quality of life. Conclusions. A supervised exercise program was superior to controls in reducing spinal deformities and improving the quality of life in patients with AIS. PMID:26583083

  15. Headache arising from idiopathic changes in CSF pressure.

    PubMed

    Ducros, Anne; Biousse, Valérie

    2015-06-01

    New onset of sudden or progressive headache can have various causes, including disorders of intracranial pressure (ICP). Headache is the most common-and often the presenting-symptom of both intracranial hypertension and intracranial hypotension syndromes, which can be symptomatic or idiopathic. Despite the widespread availability of diagnostic tests, including ocular ophthalmoscopy, neuroimaging, and measurement of CSF pressure, delays in diagnosis or misdiagnosis of idiopathic intracranial hypertension and spontaneous intracranial hypotension remain common. If left untreated, idiopathic intracranial hypertension and spontaneous intracranial hypotension produce highly disabling headaches, and threaten vision, hearing, and in rare cases, brain function and life. To improve the diagnosis of idiopathic intracranial hypertension and spontaneous intracranial hypotension, changes in the overall diagnostic strategy for headaches will be necessary in most care centres. Improved understanding of CSF physiology and the mechanisms of idiopathic intracranial hypertension and spontaneous intracranial hypotension will guide the development of new treatments. PMID:25987284

  16. Multifocal thoracic chordoma mimicking a paraganglioma.

    PubMed

    Conzo, Giovanni; Gambardella, Claudio; Pasquali, Daniela; Ciancia, Giuseppe; Avenia, Nicola; Pietra, Cristina Della; Napolitano, Salvatore; Palazzo, Antonietta; Mauriello, Claudio; Parmeggiani, Domenico; Pettinato, Guido; Napolitano, Vincenzo; Santini, Luigi

    2013-01-01

    Chordoma of thoracic vertebras is a very rare locally invasive neoplasm with low grade malignancy arising from embryonic notochordal remnants. Radical surgery remains the cornerstone of the treatment. We describe a case of multifocal T1-T2 chordoma, without bone and disc involvement, incidentally misdiagnosed as a paraganglioma, occurring in a 47-year-old male asymptomatic patient. Neoplasm was radically removed by an endocrine surgeon through a right extended cervicotomy. A preoperative reliable diagnosis of chordoma, as in the reported case, is often difficult. Radical surgery can provide a favorable outcome but, given the high rates of local recurrence of this neoplasm, a strict and careful follow-up is recommended. Although very rare, chordoma should be suggested in the differential diagnosis of the paravertebral cervical masses of unknown origin. Spine surgeon consultation and a FNB should be routinely included in the multidisciplinary preoperative work-up of these neoplasms. PMID:24125991

  17. Respiratory Displacement of the Thoracic Aorta: Physiological Phenomenon With Potential Implications for Thoracic Endovascular Repair

    SciTech Connect

    Weber, Tim Frederik; Tetzlaff, Ralf; Rengier, Fabian; Geisbuesch, Philipp; Kopp-Schneider, Annette; Boeckler, Dittmar; Eichinger, Monika; Kauczor, Hans-Ulrich; Tengg-Kobligk, Hendrik von

    2009-07-15

    The purpose of this study was to assess the magnitude and direction of respiratory displacement of the ascending and descending thoracic aorta during breathing maneuvers. In 11 healthy nonsmokers, dynamic magnetic resonance imaging was performed in transverse orientation at the tracheal bifurcation during maximum expiration and inspiration as well as tidal breathing. The magnitude and direction of aortic displacement was determined relatively to resting respiratory position for the ascending (AA) and descending (DA) aorta. To estimate a respiratory threshold for occurrence of distinct respiratory aortic motion, the latter was related to the underlying change in anterior-posterior thorax diameter. Compound displacement between maximum expiration and inspiration was 24.3 {+-} 6.0 mm for the AA in the left anterior direction and 18.2 {+-} 5.5 mm for the DA in the right anterior direction. The mean respiratory thorax excursion during tidal breathing was 8.9 {+-} 2.8 mm. The respiratory threshold, i.e., the increase in thorax diameter necessary to result in respiratory aortic displacement, was estimated to be 15.7 mm. The data suggest that after a threshold of respiratory thorax excursion is exceeded, respiration is accompanied by significant displacement of the thoracic aorta. Although this threshold may not be reached during tidal breathing in the majority of individuals, segmental differences during forced respiration impact on aortic geometry, may result in additional extrinsic forces on the aortic wall, and may be of significance for aortic prostheses designed for thoracic endovascular aortic repair.

  18. Idiopathic Polyhydramnios: Severity and Perinatal Morbidity.

    PubMed

    Wiegand, Samantha L; Beamon, Carmen J; Chescheir, Nancy C; Stamilio, David

    2016-06-01

    Objective To estimate the association between the severity of idiopathic polyhydramnios and adverse outcomes. Study Design Retrospective cohort study of deliveries at one hospital from 2000 to 2012 with an amniotic fluid index (AFI) measurement ≥24 + 0 weeks' gestation. Pregnancies complicated by diabetes, multiples, or fetal anomalies were excluded. Exposure was the degree of polyhydramnios: normal (AFI 5-24 cm), mild (≥ 24-30 cm), and moderate-severe (> 30 cm). Primary outcomes were perinatal mortality, neonatal intensive care unit (NICU) admission, and postpartum hemorrhage. Results There were 10,536 pregnancies: 10,188 with a normal AFI, 274 mild (78.74%), and 74 moderate-severe polyhydramnios (21.26%). Adverse outcomes were increased with idiopathic polyhydramnios: NICU admission (adjusted odds ratio [AOR] 3.71, 95% confidence interval [CI] 2.77-4.99), postpartum hemorrhage (AOR 15.81, 95% CI 7.82-31.96), macrosomia (AOR 3.41, 95% CI 2.61-4.47), low 5-minute Apgar score (AOR 2.60, 95% CI 1.57-4.30), and cesarean (AOR 2.16, 95% CI 1.74-2.69). There were increasing odds of macrosomia (mild: AOR 3.19, 95% CI 2.36-4.32; moderate-severe: AOR 4.44, 95% CI 2.53-7.79) and low 5-minute Apgar score (mild: AOR 2.24, 95% CI 1.23-4.08; moderate-severe: AOR 3.93, 95% CI 1.62-9.55) with increasing severity of polyhydramnios. Conclusion Idiopathic polyhydramnios is independently associated with increased risks of morbidity. There appears to be a dose-response relationship for neonatal macrosomia and low 5-minute Apgar score risks. PMID:26862725

  19. Congenital thoracic lordosis and scoliosis in a cat.

    PubMed

    Lee, Maris S; Taylor, Jim; Lefbom, Bonnie

    2014-08-01

    A 10-week-old domestic shorthair kitten was referred for intermittent episodes of dyspnea, cyanosis and a suspected congenital thoracic anomaly. Physical examination showed an obvious palpable concavity in the caudal thoracic spine. Thoracic radiographs showed severe caudal thoracic lordosis from T5 to T13 with a Cobb angle of -77°, a centroid lordosis angle of -68°, a vertebral index of 6.3 and a flattened sternum. Severe loss of vital capacity was suspected and surgical correction of the thoracic deformity was to be performed in two separate stages, the first being surgical ventral distraction on the sternum to increase thoracic volume and rigid fixation with an external splint. The second stage, if required, would be surgical correction of the spinal deformity to also increase thoracic volume. The initial stage of surgery was performed and postoperative radiographs showed a vertebral index of 10.3. The kitten suffered a left sided pneumothorax in recovery and died from cardiorespiratory arrest despite immediate pleural drainage and cardiopulmonary resuscitation. Treatment recommendations that may benefit future case management are discussed. PMID:24393777

  20. Idiopathic Pulmonary Fibrosis: Diagnosis and Clinical Manifestations

    PubMed Central

    Nakamura, Yutaro; Suda, Takafumi

    2015-01-01

    Idiopathic pulmonary fibrosis (IPF) is a parenchymal lung disease characterized by progressive interstitial fibrosis. The clinical course of IPF can be unpredictable and may be punctuated by acute exacerbations. Although much progress is being made in unraveling the mechanisms underlying IPF, effective therapy for improving survival remains elusive. Longitudinal disease profiling, especially in terms of clinical manifestations in a large cohort of patients, should lead to proper management of the patients and development of new treatments for IPF. Appropriate multidisciplinary assessment in ongoing registries is required to achieve this. This review summarizes the current status of the diagnosis and clinical manifestations of IPF.

  1. Idiopathic lipoid pneumonia successfully treated with prednisolone.

    PubMed

    Lococo, Filippo; Cesario, Alfredo; Porziella, Venanzio; Mulè, Antonino; Petrone, Gianluigi; Margaritora, Stefano; Granone, Pierluigi

    2012-01-01

    Lipoid pneumonia (LP) is a rare type of pneumonia that is radiologically characterized by lung infiltrates, although imaging alone may not be diagnostic. We describe an unusual 61-year-old patient with idiopathic LP presenting as a solitary pulmonary nodule mimicking lung cancer because of its rapid growth. After treatment with oral prednisone, a control chest radiogram indicated complete normalization of the radiologic features. This case shows that LP should be considered in the diagnostic assessment of any undefined pulmonary mass, after malignancy has been pathologically excluded. PMID:21419490

  2. Renal involvement in idiopathic hypereosinophic syndrome

    PubMed Central

    Shehwaro, Nathalie; Langlois, Anne Lyse; Gueutin, Victor; Izzedine, Hassane

    2013-01-01

    The hypereosinophilic syndromes (HESs) are a group of disorders marked by the sustained overproduction of eosinophils, in which eosinophilic infiltration and mediator release cause damage to multiple organs. In idiopathic HES, the underlying cause of hypereosinophilia (HE) remains unknown despite thorough aetiological work-up. Kidney disease is thought to be rare in HES. Renal manifestations described include eosinophilic interstitial nephritis, various types of glomerulopathies, thrombotic microangiopathy (TMA) and electrolyte disturbances. The diagnosis must be made in time, because a recovery of renal function can be obtained if treatment is initiated promptly. PMID:26064485

  3. Idiopathic Juvenile Osteoporosis: A Case Report.

    PubMed

    Altan, Halenur; Tosun, Gül; Şen, Yaşar

    2015-08-01

    Idiopathic Juvenile Osteoporosis (IJO) is a very rare disease, self restrictive and shows marked, spontaneous improvement during adolescence. The major clinical features were pain with difficulty walking, growth retardation, oral and dental abnormalities with radiographically porous bone structure. A 13-year-old male referred to paediatric dentistry clinic for toothache. The observations made with extra-intraoral clinic examination that one revealed short and skinny stature, diffuse caries in deciduous teeth, abraded lower incisor, deep bite and dysmorphic appearance in permanent incisor. This report emphasizes the recognized features of IJO as well as describes facio-dental findings that could aid in the diagnosis and management of these patients. PMID:26436063

  4. "Nocturnal seizures" in idiopathic pulmonary arterial hypertension.

    PubMed

    Izzo, Anthony; McSweeney, Julia; Kulik, Thomas; Khatwa, Umakanth; Kothare, Sanjeev V

    2013-10-15

    The usual differential diagnoses of nocturnal events in children include parasomnias, nocturnal seizures, nocturnal reflux (Sandifer syndrome), hypnic jerks, periodic limb movements of sleep, and sleep disordered breathing. We report a previously healthy young girl who presented to the sleep clinic for evaluation of nocturnal events which were diagnosed as medically refractory nocturnal seizures. It was not until a syncopal event occurred in the daytime, which prompted referral for cardiac evaluation, the diagnosis of idiopathic pulmonary arterial hyper-tension (IPAH) was made. Sleep physicians should consider IPAH in the differential diagnosis of nocturnal events in children. PMID:24127156

  5. Idiopathic Thrombocytopenic Purpura Misdiagnosed as Hereditary Angioedema

    PubMed Central

    Andersen, Michelle Fog; Bygum, Anette

    2015-01-01

    Hereditary angioedema is a rare, but potentially life-threatening genetic disorder that results from an autosomal dominant trait. It is characterized by acute, recurrent attacks of severe local edema, most commonly affecting the skin and mucosa. Swelling in hereditary angioedema patients does 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 of hereditary angioedema. The case illustrates how clinicians can have difficulties in handling patients with rare diseases, especially in the emergency care setting. PMID:26819784

  6. Idiopathic Juvenile Osteoporosis: A Case Report

    PubMed Central

    Tosun, Gül; Şen, Yaşar

    2015-01-01

    Idiopathic Juvenile Osteoporosis (IJO) is a very rare disease, self restrictive and shows marked, spontaneous improvement during adolescence. The major clinical features were pain with difficulty walking, growth retardation, oral and dental abnormalities with radiographically porous bone structure. A 13-year-old male referred to paediatric dentistry clinic for toothache. The observations made with extra-intraoral clinic examination that one revealed short and skinny stature, diffuse caries in deciduous teeth, abraded lower incisor, deep bite and dysmorphic appearance in permanent incisor. This report emphasizes the recognized features of IJO as well as describes facio-dental findings that could aid in the diagnosis and management of these patients. PMID:26436063

  7. Dystonia Associated with Idiopathic Slow Orthostatic Tremor

    PubMed Central

    Kobylecki, Christopher; Silverdale, Monty A.; Dick, Jeremy P. R.; Kellett, Mark W.; Marshall, Andrew G.

    2015-01-01

    Background We aimed to characterize the clinical and electrophysiological features of patients with slow orthostatic tremor. Case Report The clinical and neurophysiological data of patients referred for lower limb tremor on standing were reviewed. Patients with symptomatic or primary orthostatic tremor were excluded. Eight patients were identified with idiopathic slow 4–8 Hz orthostatic tremor, which was associated with tremor and dystonia in cervical and upper limb musculature. Coherence analysis in two patients showed findings different to those seen in primary orthostatic tremor. Discussion Slow orthostatic tremor may be associated with dystonia and dystonic tremor. PMID:26877891

  8. Idiopathic pulmonary hemosiderosis complicated by Down syndrome.

    PubMed

    Watanabe, Hirofumi; Ayusawa, Mamoru; Kato, Masataka; Chou, Ami; Komori, Akiko; Abe, Yuriko; Matsumura, Masaharu; Kamiyama, Hiroshi; Izumi, Hiroyuki; Takahashi, Shori

    2015-10-01

    We report the case of a 9-year-old girl with Down syndrome (DS) diagnosed with idiopathic pulmonary hemosiderosis (IPH). Although acute pneumonia complicated by hemolytic anemia was suspected, IPH was finally diagnosed on bronchoscopy. Treatment with prednisolone achieved good clinical response. An association between IPH and DS was not able to be identified, but immunological issues in DS may contribute to the onset of IPH. Recurrent and intractable respiratory symptoms with marked infiltrative shadows in the bilateral lungs and complicated by severe anemia in patients with DS should suggest IPH. PMID:26508184

  9. Systemic Juvenile Idiopathic Arthritis: Diagnosis and Management.

    PubMed

    Kumar, Sathish

    2016-04-01

    Systemic juvenile idiopathic arthritis (sJIA) is an inflammatory condition characterized by fever, lymphadenopathy, arthritis, rash and serositis. In sJIA, systemic inflammation has been associated with dysregulation of the innate immune system, suggesting that it is an autoinflammatory disorder. IL-1 and IL-6 play a major role in the pathogenesis of sJIA and treatment with IL-1 and IL-6 inhibitors has shown to be highly effective. Recent data suggests that early cytokine blockage might abrogate chronic, destructive, therapy resistant arthritis phase, reflecting a potential "window of opportunity" in the care of children with sJIA. PMID:26916892

  10. Robotic thoracic surgery: The state of the art

    PubMed Central

    Kumar, Arvind; Asaf, Belal Bin

    2015-01-01

    Minimally invasive thoracic surgery has come a long way. It has rapidly progressed to complex procedures such as lobectomy, pneumonectomy, esophagectomy, and resection of mediastinal tumors. Video-assisted thoracic surgery (VATS) offered perceptible benefits over thoracotomy in terms of less postoperative pain and narcotic utilization, shorter ICU and hospital stay, decreased incidence of postoperative complications combined with quicker return to work, and better cosmesis. However, despite its obvious advantages, the General Thoracic Surgical Community has been relatively slow in adapting VATS more widely. The introduction of da Vinci surgical system has helped overcome certain inherent limitations of VATS such as two-dimensional (2D) vision and counter intuitive movement using long rigid instruments allowing thoracic surgeons to perform a plethora of minimally invasive thoracic procedures more efficiently. Although the cumulative experience worldwide is still limited and evolving, Robotic Thoracic Surgery is an evolution over VATS. There is however a lot of concern among established high-volume VATS centers regarding the superiority of the robotic technique. We have over 7 years experience and believe that any new technology designed to make minimal invasive surgery easier and more comfortable for the surgeon is most likely to have better and safer outcomes in the long run. Our only concern is its cost effectiveness and we believe that if the cost factor is removed more and more surgeons will use the technology and it will increase the spectrum and the reach of minimally invasive thoracic surgery. This article reviews worldwide experience with robotic thoracic surgery and addresses the potential benefits and limitations of using the robotic platform for the performance of thoracic surgical procedures. PMID:25598601

  11. Rare case of thoracic kidney detected by renal scintigraphy.

    PubMed

    Natarajan, Aravintho; Agrawal, Archi; Purandare, Nilendu; Shah, Sneha; Rangarajan, Venkatesh

    2016-01-01

    Intrathoracic kidney is a rare congenital abnormality with lowest frequency among all renal ectopias. Patients with thoracic kidneys are usually asymptomatic, and the condition is usually discovered incidentally during radiological evaluation for other conditions or during thoracic surgery. We report a case of a 62-year-old male who was referred to our department for renal scintigraphy for a nonvisualized left kidney on ultrasonography report. Both Tc-99m dimercaptosuccinic acid and diethylenetriaminepentaacetic acid scans revealed a left thoracic kidney which was confirmed by CT scan of the thorax and abdomen. PMID:27385896

  12. Rare case of thoracic kidney detected by renal scintigraphy

    PubMed Central

    Natarajan, Aravintho; Agrawal, Archi; Purandare, Nilendu; Shah, Sneha; Rangarajan, Venkatesh

    2016-01-01

    Intrathoracic kidney is a rare congenital abnormality with lowest frequency among all renal ectopias. Patients with thoracic kidneys are usually asymptomatic, and the condition is usually discovered incidentally during radiological evaluation for other conditions or during thoracic surgery. We report a case of a 62-year-old male who was referred to our department for renal scintigraphy for a nonvisualized left kidney on ultrasonography report. Both Tc-99m dimercaptosuccinic acid and diethylenetriaminepentaacetic acid scans revealed a left thoracic kidney which was confirmed by CT scan of the thorax and abdomen. PMID:27385896

  13. Trans-arterial Onyx Embolization of a Functional Thoracic Paraganglioma

    PubMed Central

    Chacón-Quesada, Tatiana; Maud, Alberto; Ramos-Duran, Luis; Torabi, Alireza; Fitzgerald, Tamara; Akle, Nassim; Cruz Flores, Salvador; Trier, Todd

    2015-01-01

    Paragangliomas are rare tumors of the endocrine system. They are highly vascular and in some cases hormonally active, making their management challenging. Although there is strong evidence of the safety and effectiveness of preoperative embolization in the management of spinal tumors, only five cases have been reported in the setting of thoracic paragangliomas. We present the case of a 19-year-old man with a large, primary, functional, malignant paraganglioma of the thoracic spine causing a vertebral fracture and spinal cord compression. To our knowledge this is the first report of preoperative trans-arterial balloon augmented Onyx embolization of a thoracic paraganglioma. PMID:25763296

  14. Implementing effective and sustainable multidisciplinary clinical thoracic oncology programs

    PubMed Central

    Freeman, Richard K.; Krasna, Mark J.

    2015-01-01

    Three models of care are described, including two models of multidisciplinary care for thoracic malignancies. The pros and cons of each model are discussed, the evidence supporting each is reviewed, and the need for more (and better) research into care delivery models is highlighted. Key stakeholders in thoracic oncology care delivery outcomes are identified, and the need to consider stakeholder perspectives in designing, validating and implementing multidisciplinary programs as a vehicle for quality improvement in thoracic oncology is emphasized. The importance of reconciling stakeholder perspectives, and identify meaningful stakeholder-relevant benchmarks is also emphasized. Metrics for measuring program implementation and overall success are proposed. PMID:26380186

  15. Chimeric Anterolateral Thigh Flap for Total Thoracic Esophageal Reconstruction.

    PubMed

    Ruiz-Moya, Alejandro; Segura-Sampedro, Juan J; Sicilia-Castro, Domingo; Carvajo-Pérez, Francisco; Gómez-Cía, Tomás; Vázquez-Medina, Antonio; Ibáñez-Delgado, Francisco

    2016-01-01

    Gastric pull-up is generally the first choice for a total thoracic esophageal reconstruction. Malfunction of this gastric conduit is uncommon, but devastating when it occurs: it causes marked comorbidity to the patient, preventing oral intake and worsening quality of life. Secondary salvage thoracic esophageal reconstruction surgery is usually performed with free or pedicled jejunum flaps or colon interposition. We present a case of a total thoracic esophageal reconstruction with an externally monitored chimeric anterolateral thigh flap, extending from the cervical esophagus to the retrosternal gastroplasty remnant. Intestinal reconstructive techniques were not an available option for this patient. PMID:26694271

  16. Thoracic and lumbar extradural structure examined by extraduroscope.

    PubMed

    Igarashi, T; Hirabayashi, Y; Shimizu, R; Saitoh, K; Fukuda, H

    1998-08-01

    We examined the extradural space using a flexible extraduroscope in 113 patients undergoing extradural anaesthesia. Patients were classified into two groups to receive either thoracic or lumbar extradural anaesthesia as needed for perioperative analgesia. The extraduroscopy showed that the thoracic extradural space becomes widely patent after injecting a given amount of air and that the amount of fatty and fibrous connective tissue is less in the thoracic extradural space compared with the lumbar extradural space. We suggest that differences between the structure of these two vertebral regions may affect the spread of local anaesthetics in the extradural space. PMID:9813508

  17. Nearly Asymptomatic Eight-Month Thoracic Aortic Dissection

    PubMed Central

    Kumar, Arjun; Kumar, Krishan; Zeltser, Roman; Makaryus, Amgad N.

    2016-01-01

    Thoracic aortic dissection is a rare, but lethal, medical condition that is either misdiagnosed as a myocardial infarction or overlooked completely. Though thoracic aortic dissections are commonly diagnosed in patients exhibiting sharp chest pain, there are some notable cases where patients do not report the expected severity of pain. We report a unique case of a patient with a thoracic aortic dissection who was initially nearly asymptomatic for eight months, in order to heighten awareness, highlight diagnosis protocol, and improve prognosis for this commonly misdiagnosed, but fatal, condition. PMID:27257400

  18. Chronic Thoracic Aortic Aneurysm Presenting 29 Years following Trauma

    PubMed Central

    Miller, Sarah; Kumar, Prashant; Van den Bosch, Rene; Khanafer, Adib

    2015-01-01

    Blunt, nonpenetrating injuries of the thoracic aorta are uncommon and associated with a high mortality rate within the first hour. Aortic injury is missed in 1-2% of patients that survive to hospital, and a chronic thoracic aortic aneurysm may subsequently form. We present a case in which a chronic thoracic aortic aneurysm was diagnosed 29 years following a significant motor vehicle accident. We discuss the epidemiology, presentation, and management of this uncommon consequence of blunt, nonpenetrating aortic injury. Our case illustrates an important clinical lesson; a past medical history of trauma should not be overlooked at any patient assessment. PMID:26351610

  19. Cardio-thoracic surgical patients' experience on bedside nursing handovers: Findings from a qualitative study.

    PubMed

    Lupieri, Giulia; Creatti, Chiara; Palese, Alvisa

    2016-08-01

    The purpose of this study was to describe the experiences of postoperative cardio-thoracic surgical patients experiencing nursing bedside handover. A descriptive qualitative approach was undertaken. A purposeful sampling technique was adopted, including 14 patients who went through cardio-thoracic surgery and witnessed at least two bedside handovers. The study was performed in a Cardio-thoracic ICU localised in a Joint Commission International accredited Academic Hospital in north-eastern Italy from August to November 2014. The experience of patients participating at the bedside handover is based on four main themes: (1) 'discovering a new nursing identity', (2) 'being apparently engaged in a bedside handover', (3) 'experiencing the paradox of confidentiality' and (4) 'having the situation under control'. With the handover performed at the bedside in a postoperative setting, two interconnected potential effects may be achieved with regard to patients, nurses and the nursing profession. Nurses have a great opportunity to express their closeness to patients and to promote awareness of the important growth that nursing has achieved over the years as a profession and discipline. Therefore, patients may better perceive nursing competence and feel safer during the postoperative care pathway. They can appreciate nurses' humanity in caring and trust their competence and professionalism. PMID:27080568

  20. Caffeine restriction has no role in the management of patients with symptomatic idiopathic ventricular premature beats.

    PubMed Central

    Newby, D. E.; Neilson, J. M.; Jarvie, D. R.; Boon, N. A.

    1996-01-01

    OBJECTIVE: To assess the role of caffeine restriction in the management of patients with symptomatic idiopathic ventricular premature beats. DESIGN: A randomised, double blind, 6 week intervention trial incorporating dietary caffeine restriction, caffeinated coffee, and decaffeinated coffee. SETTING: Cardiac outpatient clinic. PATIENTS: 13 patients with symptomatic frequent idiopathic ventricular premature beats. MAIN OUTCOME MEASURES: Weekly measures of serum caffeine concentration, coffee consumption, visual analogue score of palpitations, and 24 hour ventricular premature beat frequency. RESULTS: The interventions achieved significant alterations in serum caffeine concentrations (P < 0.001) which correlated with coffee consumption (r = 0.70; P < 0.001). Visual analogue palpitation scores showed a small, but significant correlation with ventricular premature beat frequencies (r = 0.34; P = 0.003). However, there were no significant changes in palpitation scores or ventricular premature beat frequencies during the intervention weeks and no significant correlations were found between these variables and serum caffeine concentrations. CONCLUSIONS: Caffeine restriction has no role in the management of patients referred with symptomatic idiopathic ventricular premature beats. PMID:8983684

  1. Systemic-onset juvenile idiopathic arthritis.

    PubMed

    Cimaz, Rolando

    2016-09-01

    Systemic-onset juvenile idiopathic arthritis (SoJIA) is a systemic inflammatory disease which has up to now been classified as a category of juvenile idiopathic arthritis. However, in this context, systemic inflammation has been associated with dysregulation of the innate immune system, suggesting that it may rather be part of the spectrum of autoinflammatory disorders. The disease is in fact unique with regard to the other JIA categories, in terms of clinical manifestations, prognosis, and response to conventional immunosuppressant therapies. It is characterized clinically by fever, lymphadenopathy, arthritis, rash, and serositis. IL-1 and IL-6 play a major role in the pathogenesis of SoJIA, and treatment with IL-1 and IL-6 inhibitors has shown to be highly effective. However, complications of SoJIA, including macrophage activation syndrome, limitations in functional outcome by arthritis and long-term damage from chronic inflammation continue to be a major issue in patients' care. Recent advances on the pathogenesis and treatment have revolutionized the care and prognosis of this potentially life-threatening pediatric condition. PMID:27392503

  2. Idiopathic Noncirrhotic Portal Hypertension: An Appraisal

    PubMed Central

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

    2016-01-01

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

  3. Idiopathic incus necrosis: Analysis of 4 cases.

    PubMed

    Kansu, Leyla; Yilmaz, Ismail; Akdogan, Volkan; Avci, Suat; Ozluoglu, Levent

    2013-02-01

    We evaluated ossicular chain reconstruction in patients with idiopathic incus necrosis who have conductive hearing loss and an intact ear drum. The study included four patients (3 women and 1 man; the ages of the patients were 22, 31, 35, and 56 years, respectively) with unilateral conductive hearing loss, no history of chronic serous otitis media, an intact ear drum, normal middle ear mucosa, and necrosis of the long processes of the incus. On preoperative pure tone audiometry, air-bone gaps were 24, 25, 38, and 33 dB. Bilateral tympanometry and temporal bone computed tomography results were normal. All 4 patients underwent an exploratory tympanotomy. During the operation, the mucosa of the middle ear was normal, with a mobile stapes foot plate and malleus. No evidence of any granulation tissue was found; however, necrosis of the incus long processes was seen. For ossicular reconstruction, we used tragal cartilage between the incus and the stapes in 1 patient; in the other 3 patients, glass ionomer bone cement was used (an interposition cartilage graft also was used in the patients who received the glass ionomer bone cement). In all patients, air-bone gaps under 20 dB were established in the first year after surgery. In the ossicular disorders within the middle ear, the incus is the most commonly affected ossicle. While, the most common cause of these disorders is chronic otitis media, it may be idiopathic rarely. Several ossicular reconstruction techniques have been used to repair incudostapedial discontinuity. PMID:23460219

  4. Possible development of idiopathic sclerosing encapsulating peritonitis.

    PubMed

    Yanagi, H; Kusunoki, M; Yamamura, T

    1999-01-01

    We report a rare case of idiopathic sclerosing encapsulating peritonitis (SEP). During a laparotomy before undergoing a distal gastrectomy with Billroth II reconstruction for early gastric cancer, the patient was found to have a membranous encapsulation wrapping each small bowel loop, unlike peritoneal encapsulation or typical SEP. He had complained of persistent heartburn, distension and diarrhea for 2 months in the post-operative course. The second laparotomy, which was performed to improve prolonged transit, revealed typical SEP with a thick and fibrotic membrane that encased the small bowel entirely. Stripping of the sclerosing encasing membrane, separation of the adherent loops of the proximal small bowel, and Braun's anastomosis were performed. The patient complained of epigastric fullness and diarrhea after he was relieved from the complete bowel obstruction for 45 days post-operatively. Trimebutine maleate was administrated 5 months after the second operation and this markedly improved his symptoms. This case might reflect the developmental process of idiopathic SEP. In addition, the use of a motility regulator may improve symptoms related to the abnormal intestinal motility by this disease. PMID:10228820

  5. Medical Therapy in Idiopathic Pulmonary Fibrosis.

    PubMed

    Antoniou, Katerina M; Wuyts, Wim; Wijsenbeek, Marlies; Wells, Athol U

    2016-06-01

    Medical therapy for idiopathic fibrosis remains controversial. Idiopathic pulmonary fibrosis (IPF) was uniformly a disease that progressed inexorably, typically leading to death within 3 to 5 years from onset of symptoms. Until recently, lung transplantation was the only effective transplant option. Within the past decade, several placebo-controlled trials failed to show benefit in patients with IPF. However, within the past 2 years, two novel antifibrotic agents (pirfenidone and nintedanib) were approved by the Food and Drug Administration (FDA) in the United States and European Medicines Agency (EMA) based upon pivotal studies that showed benefit (specifically slowing of the rate of disease progression) with both agents. Short-term outcomes (12 months) showed less deterioration of physiological parameters (e.g., change in forced vital capacity), although survival benefit has not convincingly been established with either agent. Nonetheless, these agents bring a glimmer of hope to patients with this deadly disease. The appropriate indications for initiating therapy, best candidates for therapy, and possible role for combination therapy remain controversial. Additional studies using agents that attenuate or abrogate profibrotic cytokines and chemokines may provide even further improvement in the future. PMID:27231861

  6. Idiopathic osteoarthritis and contracture: causal implications

    PubMed Central

    Jones, P; Alexander, C; Stewart, J; Lynskey, N

    2005-01-01

    Objective: To use the known association of idiopathic osteoarthritis with contracture as a means of searching for its cause. There are currently two theories concerning this association, one assuming that the contracture is a consequence of the osteoarthritis and the other that it precedes and causes the osteoarthritis. This study tested both theories. Methods: Flexion ranges in the 12 finger joints were obtained by goniometric measurement in two samples of normal female subjects, one group with a mean age of 22 years (25 subjects) and one with a mean age of 45 years (50 subjects). The results were compared with the known regional prevalence of osteoarthritis in the finger joints of women. Results: The older group showed evidence of reduced flexion range consistent with development of contracture in the extensor mechanism of the fingers. The distribution of the contracture showed a strong negative correlation with the regional prevalence of osteoarthritis. Conclusions: An early dorsal contracture develops in the fingers of normal subjects, but it is neither a consequence of nor the cause of digital osteoarthritis. The most parsimonious explanation for the association is that both contracture and idiopathic osteoarthritis are independent consequences of failure to use the full movement range. If this hypothesis is correct, the disease could be preventable. PMID:15647431

  7. Idiopathic Fatal Pancytopenia: A Case Report

    PubMed Central

    Tilak, Vijai

    2016-01-01

    Pancytopenia is defined as decrease in red blood cells, white blood cells and platelets. Many disease processes involve the bone marrow primarily or secondarily resulting in pancytopenia. A 55-year-old male presented with generalized body weakness and few episodes of malena for last one year. Physical and systemic examination was unremarkable. CBC report revealed pancytopenia. Other haematological parameters were within normal limit. Stool for occult blood was positive. USG and CECT abdomen showed no abnormality. The patient was evaluated for any evidence of malignancy but no clue was found. Bone marrow examination was done as patient was having pancytopenia. Bone marrow smears, clot sections and bone marrow biopsy was normal. Immunohistochemistry and cytogenetics study was unremarkable. Patient was admitted in hospital for 1 month and his condition rapidly deteriorated. The cause of pancytopenia remained unexplained and therefore it was named as Idiopathic fatal pancytopenia. “Idiopathic Fatal Pancytopenia (IFP)” is an emerging new entity with a grave prognosis. We wish to sensitize the medical community and the scientists to this rapidly fatal condition. PMID:27504300

  8. Acute and subacute idiopathic interstitial pneumonias.

    PubMed

    Taniguchi, Hiroyuki; Kondoh, Yasuhiro

    2016-07-01

    Idiopathic interstitial pneumonias (IIPs) may have an acute or subacute presentation, or acute exacerbation may occur in a previously subclinical or unrecognized chronic IIP. Acute or subacute IIPs include acute interstitial pneumonia (AIP), cryptogenic organizing pneumonia (COP), nonspecific interstitial pneumonia (NSIP), acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) and AE-NSIP. Interstitial lung diseases (ILDs) including connective tissue disease (CTD) associated ILD, hypersensitivity pneumonitis, acute eosinophilic pneumonia, drug-induced lung disease and diffuse alveolar haemorrhage need to be differentiated from acute and subacute IIPs. Despite the severe lack of randomized controlled trials for the treatment of acute and subacute IIPs, the mainstream treatment remains corticosteroid therapy. Other potential therapies reported in the literature include corticosteroids and immunosuppression, antibiotics, anticoagulants, neutrophil elastase inhibitor, autoantibody-targeted treatment, antifibrotics and hemoperfusion therapy. With regard to mechanical ventilation, patients in recent studies with acute and subacute IIPs have shown better survival than those in previous studies. Therefore, a careful value-laden decision about the indications for endotracheal intubation should be made for each patient. Noninvasive ventilation may be beneficial to reduce ventilator associated pneumonia. PMID:27123874

  9. Current concepts and controversies on adolescent idiopathic scoliosis: Part I

    PubMed Central

    Sud, Alok; Tsirikos, Athanasios I

    2013-01-01

    Adolescent idiopathic scoliosis is the most common spinal deformity encountered by General Orthopaedic Surgeons. Etiology remains unclear and current research focuses on genetic factors that may influence scoliosis development and risk of progression. Delayed diagnosis can result in severe deformities which affect the coronal and sagittal planes, as well as the rib cage, waistline symmetry, and shoulder balance. Patient's dissatisfaction in terms of physical appearance and mechanical back pain, as well as the risk for curve deterioration are usually the reasons for treatment. Conservative management involves mainly bracing with the aim to stop or slow down scoliosis progression during growth and if possible prevent the need for surgical treatment. This is mainly indicated in young compliant patients with a large amount of remaining growth and progressive curvatures. Scoliosis correction is indicated for severe or progressive curves which produce significant cosmetic deformity, muscular pain, and patient discontent. Posterior spinal arthrodesis with Harrington instrumentation and bone grafting was the first attempt to correct the coronal deformity and replace in situ fusion. This was associated with high pseudarthrosis rates, need for postoperative immobilization, and flattening of sagittal spinal contour. Segmental correction techniques were introduced along with the Luque rods, Harri-Luque, and Wisconsin systems. Correction in both coronal and sagittal planes was not satisfactory and high rates of nonunion persisted until Cotrel and Dubousset introduced the concept of global spinal derotation. Development of pedicle screws provided a powerful tool to correct three-dimensional vertebral deformity and opened a new era in the treatment of scoliosis. PMID:23682172

  10. Idiopathic interstitial pneumonias: progress in classification, diagnosis, pathogenesis and management.

    PubMed Central

    King, Talmadge E.

    2004-01-01

    The idiopathic interstitial pneumonias are a heterogeneous group of poorly understood diseases with often devastating consequences for those afflicted. Subclassification of the idiopathic interstitial pneumonia based on clinical-radiological-pathological criteria has highlighted important pathogenic, therapeutic and prognostic implications. The most critical distinction is the presence of usual interstitial pneumonia, the histopathological pattern seen in idiopathic pulmonary fibrosis. Idiopathic pulmonary fibrosis has a worse response to therapy and prognosis. New insight into the pathophysiology of usual interstitial pneumonia suggests a distinctly fibroproliferative process, and antifibrotic therapies show promise. While the clinical and radiographic diagnosis of idiopathic interstitial pneumonias can be made confidently in some cases, many patients require surgical lung biopsy to determine their underlying histopathology. A structured, clinical-radiological-pathological approach to the diagnosis of the idiopathic interstitial pneumonias, with particular attention to the identification of idiopathic pulmonary fibrosis, insures proper therapy, enhances prognostication, and allows for further investigation of therapies aimed at distinct pathophysiology. Images Fig. 2 Fig. 4 Fig. 5 Fig. 6 PMID:17060957

  11. Thoracic and respirable particle definitions for human health risk assessment

    EPA Science Inventory

    Provides estimates of the thoracic and respirable fractions, for adults and children during typical activities during both nasal and oral inhalation, that may be used in the design of experimental studies and interpretation of evidence of health effects.

  12. Robotic thoracic surgery: from the perspectives of European chest surgeons

    PubMed Central

    2014-01-01

    Although thoracic surgery is one of the fastest growing programs, the results of robotic thoracic surgery reports are presented very rarely. In this manuscript, the development of robotic thoracic surgery programs in Europe and the initial results are discussed. Several European countries lead the development of robotic surgery in the world, especially for lung cancer surgery and for thymus—thymoma surgery. Yet, we may not recognize any major advantage in the outcome when compared to video-assisted thoracic surgery (VATS). But, certainly, the superior capabilities of the intraoperative instrumentation of robotic surgery will be beneficial. More experience in robotic surgery may provide superior results in oncological, physiological and life quality measurements. PMID:24868438

  13. Successful treatment of an infected thoracic endovascular stent graft.

    PubMed

    Sueda, Taijiro; Takahashi, Shinya; Katayama, Keijiro; Imai, Katsuhiko

    2016-05-01

    A 70-year-old man with a chronic type B aortic dissection was treated with two stent grafts deployed in the descending thoracic aorta. The patient was re-admitted to the hospital at 16 months after thoracic endovascular stent grafting because of a high fever. A blood culture showed sepsis due to a Staphylococcus species. A CT scan showed an increase in the size of the thrombosed false lumen. Complete excision of the infected descending aortic wall and infected stent graft were performed. The descending thoracic aorta was reconstructed using a rifampicin-bonded Dacron graft and omental wrapping. The combination of in situ graft replacement using a rifampicin-bonded graft and omental wrapping is considered an effective treatment for thoracic stent graft infection. PMID:24990657

  14. [Neurological Signs and Symptoms of True Neurogenic Thoracic Outlet Syndrome].

    PubMed

    Higashihara, Mana; Konoeda, Fumie; Sonoo, Masahiro

    2016-05-01

    Thoracic outlet syndrome (TOS) is a well-known disorder, but many aspects of its pathology, including its definition, has been disputed. True neurogenic TOS (TN-TOS) is a rare but well-defined clinical condition. TN-TOS results from the compression of the C8/T1 roots (dominant for the T1 root) or the proximal lower trunk of the brachial plexus by a fibrous band. The band extends from the first rib to either the tip of an elongated C7 transverse process or a rudimentary cervical rib. The most common presenting symptoms of TN-TOS are insidious-onset atrophy and weakness of the intrinsic hand muscles, predominantly in the thenar eminence and radial digit flexors. Nerve conduction studies demonstrate pathognomonic findings: severely attenuated compound muscle action potential of the abductor pollicis brevis muscle, and usually, loss of the sensory nerve action potential of the medial antebrachial cutaneous nerve. Numbness and sensory loss are typically observed, mainly in the medial forearm, although they are usually mild, and may be absent in some patients. Severe pain or paresthesia proximal to the elbow is not observed. The classical concept of TOS underlie nonspecific neurogenic TOS. It has been primarily diagnosed using provocative maneuvers. However, there is controversy regarding its pathological conceptualization and existence, as objective evidence of the disease is still lacking. PMID:27156505

  15. Idiopathic, aseptic, effusive, fibrinous, nonconstrictive pericarditis with tamponade in a standardbred filly.

    PubMed

    Robinson, J A; Marr, C M; Reef, V B; Sweeney, R W

    1992-11-15

    A Standardbred filly was admitted for evaluation of pleuritis and pneumonia. Heart rate was 80 to 120 beats/min, and the pulse was barely palpable. Thoracic and abdominal ultrasonography and echocardiography revealed substantial pericardial effusion with cardiac tamponade, fibrinous pericarditis, pleural effusion, and ascites. Initial electrocardiography revealed normal sinus rhythm with decreased amplitude of the QRS complexes consistent with pericardial effusion. Following thoracentesis, echocardiogram-guided pericardiocentesis was performed. Bacterial culture yielded no growth from any of the fluids, and bacteria were not seen on cytologic examination. Initial treatment included broad-spectrum antibiotic treatments, IV fluid therapy, and anti-inflammatory agent administration. On the basis of negative culture results, an immune-mediated cause was considered, and dexamethasone was instituted in a decreasing dosage regimen. Pericardial effusion, ventral edema, and ascites began to resolve within 3 days after beginning dexamethasone treatment. Thirty days following discharge, the filly was reexamined, and at that time, the prognosis for athletic performance was considered good so the horse was returned to race training. The final diagnosis in this case was idiopathic, effusive, nonconstrictive pericarditis with tamponade. Early identification, clinical understanding, and application of knowledge of the pathophysiologic mechanisms of pericarditis in horses, combined with use of diagnostic aids such as ultrasonography and aggressive therapy consisting of effusion drainage, pericardial lavage, antibiotics that penetrate the pericardium, and corticosteroids when indicated are critical for a successful outcome in horses with pericarditis. PMID:1289343

  16. [Criteria for treatment of idiopathic scoliosis between 40 degrees and 50 degrees. Surgical vs. conservative therapy].

    PubMed

    Hopf, C

    2000-06-01

    The treatment of idiopathic scoliosis over 40 degrees (Cobb) during the growth period is under discussion concerning the indication for conservative or surgical treatment. Curve progression depends on the degree of the frontal and sagittal deformity, vertebral rotation, rigidity of the curve, the skeletal age, the age and sex of the patient, the familial frequency of scoliosis and the location of the curve. In scoliosis over 40 degrees progression is fast and the possibilities for successful conservative brace treatment are reduced during the growth period. Progression occurs more frequently in thoracic and double major scolioses, especially in young patients (Risser sign 0 and 1). Predictive factors of a successful brace treatment are the correction of scoliosis and rotation; deterioration of both during the brace treatment leads to poor results. Evaluating the flexibility of the sagittal profile is important, as is primary correction of 30-50% in the brace during the 3 months. Operative correction of small primary curves reduces the fusion length, operation time, and blood loss and is followed by a reduction in loading on the adjacent vertebral segments in comparison to the long fusions necessary in more structural and double major scolioses. So far it is not possible to make an equivalent judgment of the frequency of the "crankshaft" phenomenon and the treatment necessary in young patients (Rissersign 0 and 1) treated by dorsal instrumentation alone, but temporary brace treatment may be considered in those cases. PMID:10929330

  17. MR Analysis of Regional Brain Volume in Adolescent Idiopathic Scoliosis: Neurological Manifestation of a Systemic Disease

    PubMed Central

    Liu, Tianming; Chu, Winnie C.W.; Young, Geoffrey; Li, Kaiming; Yeung, Benson H.Y.; Guo, Lei; Man, Gene C.W.; Lam, Wynnie W.M.; Wong, Stephen T.C.; Cheng, Jack C.Y.

    2008-01-01

    Purpose To investigate whether regional brain volumes in adolescent idiopathic scoliosis (AIS) patients differ from matched control subjects as AIS subjects are reported to have poor performance on combined visual and proprioceptive testing and impaired postural balance in previous studies. Materials and Methods Twenty AIS female patients with typical right-convex thoracic curve (age range,11−18 years; mean, 14.1 years) and 26 female controls (mean age, 14.8 years) underwent three-dimensional magnetization prepared rapid acquisition gradient echo (3D-MPRAGE) MR imaging. Volumes of 99 preselected neuroanatomical regions were compared by statistical parametric mapping and atlas-based hybrid warping. Results Analysis of variance statistics revealed significant mean volumetric differences in 22 brain regions between AIS and controls. Ten regions were larger in AIS including the left frontal gyri and white matter in left frontal, parietal, and temporal regions, corpus callosum and brainstem. Twelve regions were smaller in AIS, including right-sided descending white matter tracts (anterior and posterior limbs of the right internal capsule and the cerebral peduncle) and deep nucleus (caudate), bilateral perirhinal cortices, left hippocampus and amygdala, bilateral precuneus gyri, and left middle and inferior occipital gyri. Conclusion Regional brain volume difference in AIS subjects may help to explain neurological abnormalities in this group. PMID:18302230

  18. [The influence of intraoperative oxygen inhalation on patients with idiopathic pulmonary fibrosis].

    PubMed

    Sekine, Yasuo; Ko, Eitetsu

    2011-03-01

    Idiopathic pulmonary fibrosis (IPF) is a high risk factor for acute exacerbation of interstitial pneumonia (IP) after pulmonary resection. Other risk factors for inducing IP exacerbation are thought to be intraoperative inhalation of high concentration of oxygen, high pressure mechanical ventilation, major thoracic surgery, massive blood transfusion and preoperative chemotherapy and irradiation. The prophylactic strategy for this phenomenon has not been established, although mechanical ventilation by low pressure and low oxygen concentration, minimum invasive surgery and prophylactic administration of steroid, ulinastatin and sivelestat sodium hydride are performed. Acute exacerbation of IP is the same concept with acute lung injury (ALI)/acute respiratory distress syndrome (ARDS). This pulmonary injury is closely associated with reactive oxygen species (ROS). In particular, high concentration of oxygen induces excessive production of ROS. ROS stimulates alveolar macrophages and neutrophils to release inflammatory cytokines, such as TNF-alpha, IL-8, IFN-gamma, IL-6 and IL-1beta. These cytokines injure pulmonary endothelium and alveolus, and atelectasis, pulmonary hemorrhage, lung edema, hyalinization and alveolar thickness occur, and this is a manifestation of ALL Therefore, although there is no evidence, high pressure ventilation and inhalation of high oxygen concentration during anesthesia should be avoided. PMID:21485100

  19. The multidisciplinary approach in the diagnosis of idiopathic pulmonary fibrosis: a patient case-based review.

    PubMed

    Tomassetti, Sara; Piciucchi, Sara; Tantalocco, Paola; Dubini, Alessandra; Poletti, Venerino

    2015-03-01

    Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic, progressively fibrosing interstitial pneumonia that is associated with a significantly worse prognosis than other forms of chronic interstitial pneumonia. An early and accurate diagnosis of IPF is important to enable the initiation of disease-specific therapies, which have the potential to reduce disease progression, and the avoidance of inappropriate and potentially harmful drugs. Establishing an accurate diagnosis of IPF can be challenging. Recent studies and international guidelines advocate the importance of a multidisciplinary team (MDT) in the initial diagnostic assessment of patients with suspected IPF. Typical MDT members include a pulmonologist, a radiologist and a pathologist, with further input from a thoracic surgeon, a rheumatologist, a specialist nurse and an occupational physician where appropriate. Multidisciplinary diagnosis is considered the gold standard because it can improve the accuracy of diagnosis of IPF, avoid unnecessary testing (e.g. lung biopsy), and optimise patient management. Here we highlight the strengths and limitations of the multidisciplinary approach to IPF diagnosis through MDT discussion of two patient cases. PMID:25726558

  20. Salmonella osteomyelitis of the thoracic spine: an unusual presentation.

    PubMed

    Gupta, S K; Pandit, A; White, D G; Evans, P D

    2004-02-01

    A case of thoracic vertebral osteomyelitis due to Salmonella enteritidis phage type 2 in an immunocompetent patient is reported. The patient initially presented with abdominal, urinary and chest symptoms, which were followed by a large pleural effusion. The infection was successfully treated with ciprofloxacin. This is the only case of salmonella thoracic vertebral osteomyelitis in an immunocompetent patient reported in the English literature. PMID:14970302

  1. Clinical pathway for thoracic surgery in the United States

    PubMed Central

    Wei, Benjamin

    2016-01-01

    The paradigm for postoperative care for thoracic surgical patients in the United States has shifted with efforts to reduce hospital length of stay and improve quality of life. The increasing usage of minimally invasive techniques in thoracic surgery has been an important part of this. In this review we will examine our standard practices as well as the evidence behind both general contemporary postoperative care principles and those specific to certain operations. PMID:26941967

  2. Double migration of a schwannoma of thoracic spine

    PubMed Central

    Khan, Robert Ahmed; Rahman, Asifur; Bhandari, Paawan Bahadur; Khan, SIM Khairun Nabi

    2013-01-01

    Mobile intraspinal tumours have rarely been reported. In most cases, mobile tumours such as schwannomas or ependymomas were located in the cauda equina. Perusal of the literature revealed only two reports of mobile schwannomas in the cervical and thoracic regions. We report a case of thoracic schwannoma which migrated twice in successive operations resulting in negative exploration in the expected area. The aim of this report is to remind the surgeons about the possibility of migration of intradural-extramedullary tumour. PMID:23349180

  3. Idiopathic Calcium Nephrolithiasis And Hypercalciuria: The Role Of Genes

    NASA Astrophysics Data System (ADS)

    Gambaro, Giovanni; Abaterusso, Cataldo

    2007-04-01

    Idiopathic calcium nephrolithiasis and hypercalciuria are multifactorial disease conditions, the pathogenesis of which involves the interaction of environmental and individual factors. Data support a strong role of genes in the pathogenesis of these two conditions. Findings obtained in monogenic disorders characterized by renal calcium stones, and/or hypercalciuria, and/or nephrocalcinosis have proposed a number of genes as candidate genes in the pathogenesis of the common idiopathic calcium nephrolithiasis and hypercalciuria. The physiological role of these genes, and findings in monogenic disorders and idiopathic, multifactorial disorders will be presented.

  4. Idiopathic granulomatous interstitial nephritis responsive to mycophenolate mofetil therapy.

    PubMed

    Leeaphorn, Napat; Stokes, Michael B; Ungprasert, Patompong; Lecates, William

    2014-04-01

    Granulomatous interstitial nephritis (GIN) is a rare histologic disease. Various causes have been reported in the literature, including drugs, sarcoidosis, and infections. Other incidents have no discernible cause and are identified as idiopathic. We report a 68-year-old white man who presented with acute kidney injury and was given a diagnosis of idiopathic GIN. Mycophenolate mofetil treatment was elected because of steroid toxicity. He responded well to mycophenolate mofetil and has been in remission for more than 3 years. To our knowledge, this is the first report of successful treatment with mycophenolate mofetil of an adult patient with idiopathic GIN. PMID:24315767

  5. Idiopathic pleuroparenchymal fibroelastosis, a new idiopathic interstitial pneumonia: A case report.

    PubMed

    Hurtado, Enrique Javier Soto; González, Maria Luisa Amaya; Soto, Maria Del Mar Elena; Rueda, Francisco Jose Cabello; Nadal, Francisco Javier Pérez; Cantero, Alberto Ruíz

    2016-08-01

    Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare disease, idiopathic in most of the cases described in the literature. We report the case of a 55-year-old patient, non-smoker with tuberculosis treated in his youth, starting with progressive dyspnoea and cough, with radiographic abnormalities consisting of pleural thickening, bronchiectasis and structural distortion predominantly in the upper lobes. Due to functional impairment and persistent radiographic abnormalities, biopsy by video-assisted thoracoscopic surgical was decided. The presence of striking elastosis, absence of interstitial damage and abrupt boundary of the fibrous lesion with healthy lung allowed the diagnosis of IPPFE. Currently, the patient has no specific treatment and is in follow-up in the Transplant Unit. PMID:26810762

  6. [Thoracic duct valves in man and albino rat].

    PubMed

    Petrenko, V M; Kruglov, S V

    2004-01-01

    The aim of this research was to determine the number, structure and position of valves along the whole length of thoracic duct in man and albino rat. The study was performed using the material obtained from 30 human corpses of men and women aged 17 to 60 years with no history of cardiovascular pathology and from 60 outbred albino rats of both sexes aged 5 to 12 months. Material was fixed in 10% neutral formalin. Anterior wall of thoracic duct was longitudinally dissected. Thoracic duct of 30 rats was stained with gallocyanin-chrome alum, in the remaining animals it was studied after its injection with blue Gerota's mass. In most cases, the valves consisted of two semilunar cusps. Thoracic duct was found to contain on the average 14.7+/-0.2 valves in man and 11.9+/-0.2 valves in rat. Most of all valves were found in the upper (anterior) 1/3 of the thoracic duct, least--in its middle part. This seems to be determined by anatomo-topographic features of the different parts of thoracic duct. PMID:15839250

  7. Normal and abnormal spine and thoracic cage development

    PubMed Central

    Canavese, Federico; Dimeglio, Alain

    2013-01-01

    Development of the spine and thoracic cage consists of a complex series of events involving multiple metabolic processes, genes and signaling pathways. During growth, complex phenomena occur in rapid succession. This succession of events, this establishment of elements, is programmed according to a hierarchy. These events are well synchronized to maintain harmonious limb, spine and thoracic cage relationships, as growth in the various body segments does not occur simultaneously at the same magnitude or rate. In most severe cases of untreated progressive early-onset spinal deformities, respiratory insufficiency and pulmonary and cardiac hypertension (cor pulmonale), which characterize thoracic insufficiency syndrome (TIS), can develop, sometimes leading to death. TIS is the inability of the thorax to ensure normal breathing. This clinical condition can be linked to costo-vertebral malformations (e.g., fused ribs, hemivertebrae, congenital bars), neuromuscular diseases (e.g., expiratory congenital hypotonia), Jeune or Jarcho-Levin syndromes or to 50% to 75% fusion of the thoracic spine before seven years of age. Complex spinal deformities alter normal growth plate development, and vertebral bodies become progressively distorted, perpetuating the disorder. Therefore, many scoliotic deformities can become growth plate disorders over time. This review aims to provide a comprehensive review of how spinal deformities can affect normal spine and thoracic cage growth. Previous conceptualizations are integrated with more recent scientific data to provide a better understanding of both normal and abnormal spine and thoracic cage growth. PMID:24147251

  8. Results of hemivertebrectomy and fusion for symptomatic thoracic disc herniation.

    PubMed

    Debnath, U K; McConnell, J R; Sengupta, D K; Mehdian, S M H; Webb, J K

    2003-06-01

    We retrospectively analysed ten consecutive patients (age range 32-77 years) treated surgically from 1994 to 1999 for symptomatic thoracic disc herniation between the 6th and 12th thoracic discs. Clinically, eight patients had varying grades of back pain and eight patients had paraparesis. Radiography showed calcification in 50% of the herniated discs. Two patients had two-level thoracic disc herniation. Hemivertebrectomy followed by discectomy and fusion was carried out in all patients. Instrumentation with cages was performed in eight patients and bone grafting alone in two patients. The average follow-up was 24 months (range 13-36 months). Six patients had an excellent or good outcome, three had a fair outcome and one had a poor outcome. One patient had atelectasis, which recovered within 2 days of surgery. Another patient had developed complete paraplegia, detected at surgery by SSEPs, and underwent resurgery following magnetic resonance (MR) scan with complete corpectomy and instrumented fusion. At 2 years, she had a functional recovery. The patient with poor outcome had undergone a previous discectomy at T9/10. He developed severe back pain and generalised hyper-reflexia following corpectomy and fusion for disc herniation at T10/11. We advocate anterior transthoracic discectomy following partial corpectomy for symptomatic thoracic disc herniation between the 6th and 12th thoracic discs. This procedure offers improved access to the thoracic disc for an instrumented fusion, which is likely to decrease the risk of iatrogenic injury to the spinal cord. PMID:12800003

  9. Donor to recipient sizing in thoracic organ transplantation.

    PubMed

    Eberlein, Michael; Reed, Robert M

    2016-03-24

    Donor-to-recipient organ size matching is a critical aspect of thoracic transplantation. In the United States potential recipients for lung transplant and heart transplant are listed with limitations on donor height and weight ranges, respectively. Height is used as a surrogate for lung size and weight is used as a surrogate for heart size. While these measures are important predictors of organ size, they are crude surrogates that fail to incorporate the influence of sex on organ size. Independent of other measures, a man's thoracic organs are approximately 20% larger than a woman's. Lung size can be better estimated using the predicted total lung capacity, which is derived from regression equations correcting for height, sex and age. Similarly, heart size can be better estimated using the predicted heart mass, which adjusts for sex, age, height, and weight. These refined organ sizing measures perform better than current sizing practice for the prediction of outcomes after transplantation, and largely explain the outcome differences observed after sex-mismatch transplantation. An undersized allograft is associated with worse outcomes. In this review we examine current data pertaining to size-matching in thoracic transplantation. We advocate for a change in the thoracic allocation mechanism from a height-or-weight-based strategy to a size-matching process that utilizes refined estimates of organ size. We believe that a size-matching approach based on refined estimates of organ size would optimize outcomes in thoracic transplantation without restricting or precluding patients from thoracic transplantation. PMID:27011913

  10. Imaging in thoracic oncology: case studies from Multidisciplinary Thoracic Tumor Board

    PubMed Central

    Reddy, Rishindra M.; Lin, Jules; Arenberg, Douglas A.; Speers, Corey; Hayman, James A.; Kong, Fengming P.; Orringer, Mark B.; Kalemkerian, Gregory P.

    2013-01-01

    Abstract Multidisciplinary tumor board conferences foster collaboration among health care providers from a variety of specialties and help to facilitate optimal patient care. Generally, the clinical questions revolve around the best options for establishing a diagnosis, staging the disease and directing treatment. This article describes and illustrates the clinical scenarios of three patients who were presented at our thoracic Tumor Board, focusing on management issues and the role of imaging. These patients had invasive thymoma; concurrent small cell lung cancer and non-small cell lung cancer; and esophageal cancer with celiac lymph node metastases, respectively. PMID:24325879

  11. Quantitative normal thoracic anatomy at CT.

    PubMed

    Matsumoto, Monica M S; Udupa, Jayaram K; Tong, Yubing; Saboury, Babak; Torigian, Drew A

    2016-07-01

    Automatic anatomy recognition (AAR) methodologies for a body region require detailed understanding of the morphology, architecture, and geographical layout of the organs within the body region. The aim of this paper was to quantitatively characterize the normal anatomy of the thoracic region for AAR. Contrast-enhanced chest CT images from 41 normal male subjects, each with 11 segmented objects, were considered in this study. The individual objects were quantitatively characterized in terms of their linear size, surface area, volume, shape, CT attenuation properties, inter-object distances, size and shape correlations, size-to-distance correlations, and distance-to-distance correlations. A heat map visualization approach was used for intuitively portraying the associations between parameters. Numerous new observations about object geography and relationships were made. Some objects, such as the pericardial region, vary far less than others in size across subjects. Distance relationships are more consistent when involving an object such as trachea and bronchi than other objects. Considering the inter-object distance, some objects have a more prominent correlation, such as trachea and bronchi, right and left lungs, arterial system, and esophagus. The proposed method provides new, objective, and usable knowledge about anatomy whose utility in building body-wide models toward AAR has been demonstrated in other studies. PMID:27065241

  12. Selective lung intubation during paediatric thoracic surgeries.

    PubMed

    Mixa, V; Nedomova, B; Rygl, M

    2016-01-01

    Selective lung intubation is a necessary prerequisite for the completion of most interventions comprising thoracotomy and thoracoscopy. In paediatric care, our site uses Univent tubes for children up to the age of three years and double-lumen tubes (DLT) for children from 6-8 years of age. In younger children, we usually use regular endotracheal intubation, with the lung being held in the hemithorax position being operated on using a surgical retractor. The article presents the analysis of 860 thoracic surgeries, of which 491 comprised selective intubation (Univent 57 cases, DLT 434 cases). The use of the aforementioned devices is connected with certain complications. Univent tube can be connected with intraoperative dislocation of the obturating balloon (29.8%) and balloon perforation (5.2%). DLT insertion may be connected with failure of tube fitting. In 84 cases we had to repeat DLT insertion (20.6%). In 8 cases we were not able to insert DLT at all (1.8%). Standard use of selective intubation methods in paediatric patients from two years of age improved the conditions for surgical interventions (Tab. 2, Fig. 2, Ref. 19). PMID:27546541

  13. [Intraoperative risk management during thoracic procedures].

    PubMed

    Tsuzaki, Koichi

    2009-05-01

    Risk management in clinical practice is an impor part of medical audit. Although, medical audit consists of monitoring, data collection, peer review and establishing standards, these four steps should be regarded as a series of cyclical process. As a general rule, this concept should be applied to any field of clinical medicine and will contribute to the development of sound quality control scheme. Several complications are known to occur in thoracic anesthesia, especially in one-lung ventilation. For example, malposition of double-lumen endotracheal tube, severe hypoxia and higher airway pressure are relatively common problems, and it would be better for us to prepare for these adverse events beforehand. Auscultation, fiber-optic visualization and proper ventilatory management (eg. lower tidal volume with dependent lung PEEP, alveolar recruitment maneuver, application of CPAP to non-dependent lung) are the recommended technique required to correct these abnormalities. When life-threatening hypoxia is imminent, we should convert to two-lung ventilation without any delay. In this regard, verbal communication between surgical teams should be kept on even ground, each playing key roles in the management of such a critical situation. PMID:19462793

  14. Cytomegalovirus Immunoglobulin After Thoracic Transplantation: An Overview.

    PubMed

    Grossi, Paolo; Mohacsi, Paul; Szabolcs, Zoltán; Potena, Luciano

    2016-03-01

    Cytomegalovirus (CMV) is a highly complex pathogen which, despite modern prophylactic regimens, continues to affect a high proportion of thoracic organ transplant recipients. The symptomatic manifestations of CMV infection are compounded by adverse indirect effects induced by the multiple immunomodulatory actions of CMV. These include a higher risk of acute rejection, cardiac allograft vasculopathy after heart transplantation, and potentially bronchiolitis obliterans syndrome in lung transplant recipients, with a greater propensity for opportunistic secondary infections. Prophylaxis for CMV using antiviral agents (typically oral valganciclovir or intravenous ganciclovir) is now almost universal, at least in high-risk transplants (D+/R-). Even with extended prophylactic regimens, however, challenges remain. The CMV events can still occur despite antiviral prophylaxis, including late-onset infection or recurrent disease, and patients with ganciclovir-resistant CMV infection or who are intolerant to antiviral therapy require alternative strategies. The CMV immunoglobulin (CMVIG) and antiviral agents have complementary modes of action. High-titer CMVIG preparations provide passive CMV-specific immunity but also exert complex immunomodulatory properties which augment the antiviral effect of antiviral agents and offer the potential to suppress the indirect effects of CMV infection. This supplement discusses the available data concerning the immunological and clinical effects of CMVIG after heart or lung transplantation. PMID:26900989

  15. Molecular Mechanisms of Thoracic Aortic Dissection

    PubMed Central

    Wu, Darrell; Shen, Ying H.; Russell, Ludivine; Coselli, Joseph S.; LeMaire, Scott A.

    2013-01-01

    Thoracic aortic dissection (TAD) is a highly lethal vascular disease. In many patients with TAD, the aorta progressively dilates and ultimately ruptures. Dissection formation, progression, and rupture cannot be reliably prevented pharmacologically because the molecular mechanisms of aortic wall degeneration are poorly understood. The key histopathologic feature of TAD is medial degeneration, a process characterized by smooth muscle cell depletion and extracellular matrix degradation. These structural changes have a profound impact on the functional properties of the aortic wall and can result from excessive protease-mediated destruction of the extracellular matrix, altered signaling pathways, and altered gene expression. Review of the literature reveals differences in the processes that lead to ascending versus descending and sporadic versus hereditary TAD. These differences add to the complexity of this disease. Although tremendous progress has been made in diagnosing and treating TAD, a better understanding of the molecular, cellular, and genetic mechanisms that cause this disease is necessary to developing more effective preventative and therapeutic treatment strategies. PMID:23856125

  16. Endovascular Management of Thoracic Aortic Aneurysms

    SciTech Connect

    Fattori, Rossella Russo, Vincenzo; Lovato, Luigi; Buttazzi, Katia; Rinaldi, Giovanni

    2011-12-15

    The overall survival of patients with thoracic aortic aneurysm (TAA) has improved significantly in the past few years. Endovascular treatment, proposed as an alternative to surgery, has been considered a therapeutic innovation because of its low degree of invasiveness, which allows the treatment of even high-surgical risk patients with limited complications and mortality. A major limitation is the lack of adequate evidence regarding long-term benefit and durability because follow-up has been limited to just a few years even in the largest series. The combination of endovascular exclusion with visceral branch revascularization for the treatment of thoraco-abdominal aortic aneurysms involving the visceral aorta has also been attempted. As an alternative, endografts with branches represent a technological evolution that allows treatment of complex anatomy. Even if only small numbers of patients and short follow-up are available, this technical approach, which has with limited mortality (<10%) and paraplegia rates, to expand endovascular treatment to TAA seems feasible. With improved capability to recognize proper anatomy and select clinical candidates, the choice of endovascular stent-graft placement may offer a strategy to optimize management and improve prognosis.

  17. Hemoptysis as a first symptom of endoleak after thoracic endovascular aortic repair, which caused aortic rupture and required complex management

    PubMed Central

    Warot, Marcin; Burchard, Paweł; Paschke, Łukasz; Łysiak, Zuzanna; Chęciński, Paweł

    2013-01-01

    Thoracic endovascular aortic repair (TEVAR) has become the most common procedure in the treatment of thoracic aorta aneurysms. However, potential long-term complications of this technique could be life-threatening. Hemoptysis is a common symptom of lung tumor, especially cancer. On the other hand, hemoptysis can also be caused by bronchitis, tuberculosis, mycosis, and trauma. In this case report, we present a patient with hemoptysis and lung tumor suggesting lung cancer, which was a unique symptom of type IA endoleak after TEVAR and led to rupture of the thoracic aneurysm. It was decided to perform next an endovascular procedure due to the severe state of the patient. Next the thoracotomy was performed because drainage of the left pleural cavity was unsuccessful. In the last stage bronchoscopy was needed to remove the thrombus, which occluded the left main bronchi. Successful management has led to the patient's full recovery. Despite justified popularity of endovascular procedures in the treatment of thoracic aorta aneurysms, we should remember about potential long-term complications. Hemoptysis could be a unique symptom of the endoleak after TEVAR and treatment of such complications could be complex and demanding. PMID:23837105

  18. Chronic idiopathic urticaria and anxiety symptoms.

    PubMed

    Barbosa, Filipe; Freitas, João; Barbosa, António

    2011-10-01

    Chronic idiopathic urticaria (CIU) is a frequently disabling disease with a negative influence on the quality of life, and can cause psychopathological symptoms, such as anxiety. Our aim is to study further anxiety symptoms on CIU patients. Both CIU patients and the control group were studied by means of validated scales for psychopathology symptoms, psychological variables and quality of life. In this study, we reported high levels of anxiety symptoms. We found statistically significant correlations between anxiety symptoms, some personality dimensions, insecure attachment styles, alexithymia and with some quality of life dimensions. CIU patients exhibit high levels of psychological distress that could potentiate difficulties at several domains, namely social, emotional, general health perception and interpersonal relationships. PMID:21459916

  19. Chronic idiopathic urticaria: treatment with omalizumab.

    PubMed

    Naaman, Sandra; Sussman, Gordon

    2014-01-01

    Chronic idiopathic urticaria (CIU) is a common autoimmune skin condition characterized by spontaneously recurring hives for 6 weeks or longer. The new terminology used for CIU in most countries including Canada is chronic spontaneous urticaria (CSU). CSU is associated with significant psychosocial morbidity with a markedly negative impact on overall quality of life. Conventional approaches with antihistamines, even at high doses, is effective in about 50% of patients suffering from CSU. A new treatment option, omalizumab, a humanized monoclonal antibody against the Fc domain of IgE, has undergone the scrutiny of randomized research studies evaluating the efficacy in CSU. This editorial reviews mechanisms of action of omalizumab, efficacy, cost and potential side effect profile. Omalizumab has emerged as a very promising treatment option for patients with CSU. Future research is necessary to establish standardized protocols related to dosing as well as monitoring possible adverse effects of long-term treatment. PMID:25807072

  20. Idiopathic granulomatous mastitis: presentation, investigation and management.

    PubMed

    Benson, John R; Dumitru, Dorin

    2016-06-01

    Idiopathic granulomatous mastitis (IGM) is a rare chronic inflammatory condition of the breast which although benign can mimic carcinoma. Establishing a diagnosis can be challenging and requires a high index of suspicion with exclusion of infective and autoimmune breast diseases. IGM is characterized histologically by noncaseating granulomas which are of a lobulo-centric pattern and often associated with microabscess formation. Management of confirmed cases remains controversial with proponents of initial surgical or medical therapies - each has its associated problems which can be worse than the original symptoms of IGM. However, many patients require more than one modality of treatment to completely resolve IGM lesions and careful judgment is necessary to ensure optimal type and sequencing of treatments. PMID:27067146

  1. An Update on Idiopathic Intracranial Hypertension

    PubMed Central

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

    2013-01-01

    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, such as obstructive sleep apnea and transverse cerebral venous sinus stenoses. Recent identification of subgroups at high risk for irreversible visual loss, including black patients, men, and patients with fulminant forms of IIH, help guide the optimal management and follow-up. Ongoing studies of venous anatomy and physiology in IIH patients, as well as a recently begun randomized clinical treatment trial, should provide more insights into this common yet poorly understood syndrome of isolated intracranial hypertension. PMID:20944524

  2. Idiopathic intracranial hypertension presenting as postpartum headache.

    PubMed

    Mathew, Mariam; Salahuddin, Ayesha; Mathew, Namitha R; Nandhagopal, Ramachandiran

    2016-01-01

    Postpartum headache is described as headache and neck or shoulder pain during the first 6 weeks after delivery. Common causes of headache in the puerperium are migraine headache and tension headache; other causes include pre-eclampsia/eclampsia, post-dural puncture headache, cortical vein thrombosis, subarachnoid hemorrhage, posterior reversible leukoencephalopathy syndrome, brain tumor, cerebral ischemia, meningitis, and so forth. Idiopathic intracranial hypertension (IIH) is a rare cause of postpartum headache. It is usually associated with papilledema, headache, and elevated intracranial pressure without any focal neurologic abnormality in an otherwise healthy person. It is more commonly seen in obese women of reproductive age group, but rare during pregnancy and postpartum. We present a case of IIH who presented to us 18 days after cesarean section with severe headache and was successfully managed. PMID:26818168

  3. Acute Exacerbations of Idiopathic Pulmonary Fibrosis

    PubMed Central

    Collard, Harold R.; Moore, Bethany B.; Flaherty, Kevin R.; Brown, Kevin K.; Kaner, Robert J.; King, Talmadge E.; Lasky, Joseph A.; Loyd, James E.; Noth, Imre; Olman, Mitchell A.; Raghu, Ganesh; Roman, Jesse; Ryu, Jay H.; Zisman, David A.; Hunninghake, Gary W.; Colby, Thomas V.; Egan, Jim J.; Hansell, David M.; Johkoh, Takeshi; Kaminski, Naftali; Kim, Dong Soon; Kondoh, Yasuhiro; Lynch, David A.; Müller-Quernheim, Joachim; Myers, Jeffrey L.; Nicholson, Andrew G.; Selman, Moisés; Toews, Galen B.; Wells, Athol U.; Martinez, Fernando J.

    2007-01-01

    The natural history of idiopathic pulmonary fibrosis (IPF) has been characterized as a steady, predictable decline in lung function over time. Recent evidence suggests that some patients may experience a more precipitous course, with periods of relative stability followed by acute deteriorations in respiratory status. Many of these acute deteriorations are of unknown etiology and have been termed acute exacerbations of IPF. This perspective is the result of an international effort to summarize the current state of knowledge regarding acute exacerbations of IPF. Acute exacerbations of IPF are defined as acute, clinically significant deteriorations of unidentifiable cause in patients with underlying IPF. Proposed diagnostic criteria include subjective worsening over 30 days or less, new bilateral radiographic opacities, and the absence of infection or another identifiable etiology. The potential pathobiological roles of infection, disordered cell biology, coagulation, and genetics are discussed, and future research directions are proposed. PMID:17585107

  4. Malignant Hyperthermia and Idiopathic HyperCKemia.

    PubMed

    Kasi, Pashtoon Murtaza

    2011-01-01

    Malignant hyperthermia (MH) is a rare but life-threatening condition that is more frequently encountered and discussed within the anesthesia literature. Here we through a case specifically discuss the susceptibility of individuals and/or families with asymptomatic unexplained elevations of creatine kinase (CK), also frequently referred to as hyperCKemia or idiopathic hyperCKemia (IHCK) in recent reports. The clinical implications would be to underscore the importance of this as a susceptibility to developing MH and highlight the importance of genetic susceptibility testing in such cases. Anesthesiologists and critical care intensivists as well as primary care physicians should keep this in mind when seeing patients with asymptomatic hyperCKemia and potentially inform them about the possibility of developing MH if exposed to triggering agents. Genetic susceptibility testing should be considered if available and family members should also receive nontriggering agents when undergoing anesthesia and wear Medic Alert tags. PMID:22162697

  5. A Mathematical Model of Idiopathic Pulmonary Fibrosis

    PubMed Central

    Hao, Wenrui; Marsh, Clay; Friedman, Avner

    2015-01-01

    Idiopathic pulmonary fibrosis (IPF) is a disease of unknown etiology, and life expectancy of 3-5 years after diagnosis. The incidence rate in the United States is estimated as high as 15 per 100,000 persons per year. The disease is characterized by repeated injury to the alveolar epithelium, resulting in inflammation and deregulated repair, leading to scarring of the lung tissue, resulting in progressive dyspnea and hypoxemia. The disease has no cure, although new drugs are in clinical trials and two agents have been approved for use by the FDA. In the present paper we develop a mathematical model based on the interactions among cells and proteins that are involved in the progression of the disease. The model simulations are shown to be in agreement with available lung tissue data of human patients. The model can be used to explore the efficacy of potential drugs. PMID:26348490

  6. MUC5B and Idiopathic Pulmonary Fibrosis.

    PubMed

    Yang, Ivana V; Fingerlin, Tasha E; Evans, Christopher M; Schwarz, Marvin I; Schwartz, David A

    2015-11-01

    Idiopathic pulmonary fibrosis (IPF), a fatal disease that is a result of complex interactions between genetics and the environment, has limited treatment options. We have identified the MUC5B promoter polymorphism and other common genetic variants that in aggregate explain roughly one-third of disease risk. The MUC5B promoter polymorphism is the strongest and the most replicated genetic risk factor for IPF, appears to be protective and predictive in this disease, and is likely involved in disease pathogenesis through an increase in MUC5B expression in terminal bronchi and honeycombed cysts. Expression of MUC5B is also highly correlated with expression of cilium genes in IPF lung. Our work suggests that mucociliary dysfunction in the distal airway may play a role in the development of progressive fibroproliferative lung disease. In addition, our work has important implications for secondary prevention, early detection, and future early and personalized treatment based on genetic profiles. PMID:26595739

  7. Cardiac manifestations of idiopathic pulmonary fibrosis

    PubMed Central

    Agrawal, Abhinav; Verma, Isha; Shah, Varun; Agarwal, Abhishek; Sikachi, Rutuja R

    2016-01-01

    Summary Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, parenchymal disease of the lung with an estimated prevalence of 14–43 per 100,000. Patient usually presents with coughing and exertional dyspnea, which can lead to acute respiratory failure. IPF has been associated with various co-morbidities such as lung cancer, emphysema, obstructive sleep apnea (OSA), GERD and multiple cardiovascular consequences. The cardiovascular manifestations of IPF include pulmonary hypertension, heart failure, coronary artery disease, cardiac arrhythmias & cardiac manifestations of drugs used to treat IPF. This review will outline evidence of the association between IPF and cardiovascular conditions and attempt to provide insights into the underlying pathophysiology. We also discuss the impact of these cardiovascular diseases on patients with IPF including increased morbidity and mortality. PMID:27195188

  8. Cardiac manifestations of idiopathic pulmonary fibrosis.

    PubMed

    Agrawal, Abhinav; Verma, Isha; Shah, Varun; Agarwal, Abhishek; Sikachi, Rutuja R

    2016-05-01

    Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, parenchymal disease of the lung with an estimated prevalence of 14-43 per 100,000. Patient usually presents with coughing and exertional dyspnea, which can lead to acute respiratory failure. IPF has been associated with various co-morbidities such as lung cancer, emphysema, obstructive sleep apnea (OSA), GERD and multiple cardiovascular consequences. The cardiovascular manifestations of IPF include pulmonary hypertension, heart failure, coronary artery disease, cardiac arrhythmias & cardiac manifestations of drugs used to treat IPF. This review will outline evidence of the association between IPF and cardiovascular conditions and attempt to provide insights into the underlying pathophysiology. We also discuss the impact of these cardiovascular diseases on patients with IPF including increased morbidity and mortality. PMID:27195188

  9. Isolated unilateral idiopathic transient hypoglossal nerve palsy

    PubMed Central

    Ahmed, Syed Viqar; Akram, Muhammad Saqub

    2014-01-01

    A 52-year-old Caucasian man presented with sudden onset of difficulty in moving his tongue to the left with preceding left-sided headache with no neck pain. Earlier, he had self-limiting chest infection without rashes or tonsillar enlargement. His medical and surgical history was unremarkable with no recent trauma. Oral examination revealed difficulty in protruding his tongue to the left with muscle bulk loss and fasciculation on the same side, suggesting left hypoglossal nerve palsy. Examination of the rest of the cranial nerves and nervous system was normal. The patient's oropharyngeal and laryngeal examination was unremarkable with no cervical lymphadenopathy. He had normal laboratory investigations and cerebrospinal fluid examination. Extensive imaging of the head, neck and chest failed to reveal any pathology. Further review by an otorhinologist and rheumatologist ruled out any other underlying pathology. He made a good recovery without treatment. English literature search revealed very few cases of idiopathic, transient, unilateral hypoglossal nerve palsy. PMID:24969070

  10. [Idiopathic pulmonary fibrosis and lung cancer].

    PubMed

    Yoshimura, Akinobu; Kudoh, Syoji

    2003-02-01

    It is widely known that patients with idiopathic pulmonary fibrosis (IPF) are frequently associated with lung cancer. Although a complication with lung cancer is an important prognostic factor for IPF, standard treatments for lung cancer cannot be given because of IPF. Especially, the administration of many anticancer agents is limited by a complication with IPF, which is recognized as a risk factor for the development of fatal lung injury in cancer chemotherapy. Epidemiological studies reveal that cigarette smoking and occupational and environmental exposure to toxic substances are common risk factors for both IPF and lung cancer. It has been assumed that metaplasia in fibrous lesions is pathologically a precancerous lesion, but it is necessary to prove several genetic abnormalities in the process of carcinogenesis in order to clarify that. Currently, several genetic abnormalities in IPF, including in p53, K-ras, FHIT and transforming growth factor (TGF)-beta 1 type II receptor, have been reported. PMID:12610869

  11. White Matter Microstructure in Idiopathic Craniocervical Dystonia

    PubMed Central

    Pinheiro, Giordanna L. S.; Guimarães, Rachel P.; Piovesana, Luiza G.; Campos, Brunno M.; Campos, Lidiane S.; Azevedo, Paula C.; Torres, Fabio R.; Amato-Filho, Augusto C.; França, Marcondes C.; Lopes-Cendes, Iscia; Cendes, Fernando; D’Abreu, Anelyssa

    2015-01-01

    Background Dystonias are hyperkinetic movement disorders characterized by involuntary muscle contractions resulting in abnormal torsional movements and postures. Recent neuroimaging studies in idiopathic craniocervical dystonia (CCD) have uncovered the involvement of multiple areas, including cortical ones. Our goal was to evaluate white matter (WM) microstructure in subjects with CCD using diffusion tensor imaging (DTI) analysis. Methods We compared 40 patients with 40 healthy controls. Patients were then divided into subgroups: cervical dystonia, blepharospasm, blepharospasm + oromandibular dystonia, blepharospasm + oromandibular dystonia + cervical dystonia, using tract-based spatial statistics. We performed a region of interest-based analysis and tractography as confirmatory tests. Results There was no significant difference in the mean fractional anisotropy (FA) and mean diffusivity (MD) between the groups in any analysis. Discussion The lack of DTI changes in CCD suggests that the WM tracts are not primarily affected. PMID:26056610

  12. Managing comorbidities in idiopathic pulmonary fibrosis

    PubMed Central

    Fulton, Blair G; Ryerson, Christopher J

    2015-01-01

    Major risk factors for idiopathic pulmonary fibrosis (IPF) include older age and a history of smoking, which predispose to several pulmonary and extra-pulmonary diseases. IPF can be associated with additional comorbidities through other mechanisms as either a cause or a consequence of these diseases. We review the literature regarding the management of common pulmonary and extra-pulmonary comorbidities, including chronic obstructive pulmonary disease, lung cancer, pulmonary hypertension, venous thromboembolism, sleep-disordered breathing, gastroesophageal reflux disease, coronary artery disease, depression and anxiety, and deconditioning. Recent studies have provided some guidance on the management of these diseases in IPF; however, most treatment recommendations are extrapolated from studies of non-IPF patients. Additional studies are required to more accurately determine the clinical features of these comorbidities in patients with IPF and to evaluate conventional treatments and management strategies that are beneficial in non-IPF populations. PMID:26451121

  13. Idiopathic pulmonary hemosiderosis - a diagnostic challenge.

    PubMed

    Bakalli, Ilirjana; Kota, Luljeta; Sala, Durim; Celaj, Ermela; Kola, Elmira; Lluka, Robert; Sallabanda, Sashenka

    2014-01-01

    Idiopathic pulmonary hemosiderosis is a rare disorder that can occur at any age and is characterized by the triad of hemoptysis, iron deficiency anemia and diffuse pulmonary infiltrates. The clinical course is exceedingly variable especially in children and a substantial proportion of this age group is undiagnosed. It is probably due to the fact that iron deficiency anemia may be the first and the only manifestation of IPH, preceding other symptoms and signs by several months and IPH is not considered as a rare cause of anemia, unless the typical triad is present. We present a case of IPH in a 13-year-old girl, treated for several months with persistent iron deficiency anemia, without response to therapy. PMID:24708654

  14. [Adolescent idiopathic scoliosis : Guideline for practical application].

    PubMed

    Seifert, J; Thielemann, F; Bernstein, P

    2016-06-01

    Juvenile or adolescent idiopathic scoliosis is a relatively common spinal deformity, with an incidence of more than 1 %. Early diagnosis can lead to successful therapy. In the case of pathological clinical findings, the anteroposterior X‑ray of the whole spine leads the way to the correct grading, according to Cobb angle measurement. Depending on the individual risk of progression, brace treatment will be started with a Cobb angle range of 20-25°. Important predictors of therapeutic success are sufficient primary corrective power and patient compliance. COBB angles of 40-50° usually lead to the recommendation for surgery, which is performed as either anterior or posterior spinal fusion in skeletally mature adolescents, depending on the grade of the deformity according to Lenke's classification. To achieve the best possible results, it is recommended that both conservative and surgical treatments are carried out by scoliosis specialists. PMID:27241514

  15. Genetics Home Reference: familial idiopathic basal ganglia calcification

    MedlinePlus

    ... in regulating phosphate levels within the body (phosphate homeostasis) by transporting phosphate across cell membranes. The SLC20A2 ... link familial idiopathic basal ganglia calcification with phosphate homeostasis. Nat Genet. 2012 Feb 12;44(3):254- ...

  16. [Idiopathic facial pain--Part 1: Definition, classification and etiology].

    PubMed

    Reher, P; Harris, M

    1998-01-01

    The present paper introduces a new concept in the physiopathology and treatment of the called 'temporomandibular dysfunction'. This paper was divided into two parts, this first explaining its definition, aetiology and hypothesis of the idiopathic facial pain. The second part, will mention its treatment, prognosis and recommendations. Under this new view, this 'dysfunction' is part of the called Idiopathic Facial Pain. The idiopathic facial pain is didactically subdivided in (1) facial artromialgia, (2) atypical facial pain, (3) atypical odontalgia and (4) oral dysestesia. Occlusal factors are less important in its aetiology, and psychological and biochemical factors are more recognised. Correct diagnosis is essential, and a full history, including psychological and social aspects is very important. The present management is based on a working model developed in this department and in other world centres, and is revolutionising the clinical management of the patients with idiopathic facial pain. PMID:9922499

  17. Long term combination treatment for severe idiopathic pulmonary arterial hypertension

    PubMed Central

    Affuso, Flora; Cirillo, Plinio; Ruvolo, Antonio; Carlomagno, Guido; Fazio, Serafino

    2010-01-01

    We report the long-term follow-up of 3 cases of severe idiopathic pulmonary arterial hypertension, in whom tadalafil plus sitaxentan combination therapy improved the clinical condition and exercise performance without any relevant adverse event. PMID:21160759

  18. Idiopathic Paroxysmal Ventricular Tachycardia in Infants and Children

    ERIC Educational Resources Information Center

    Hernandez, Antonio; And Others

    1975-01-01

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

  19. The use of a portable digital thoracic suction Thopaz drainage system for the management of a persistent spontaneous secondary pneumothorax in a patient with underlying interstitial lung disease.

    PubMed

    Jenkins, William S A; Hall, David P; Dhaliwal, Kev; Hill, Adam T; Hirani, Nik

    2012-01-01

    We present the case of a 68-year-old woman who presented in extremis with a secondary pneumothorax with a past history of severe idiopathic pulmonary fibrosis. Following insertion of a 32F intercostal drain, she developed a persistent broncho-pleural fistula and became dependent on negative-pressure wall-mounted suction to prevent respiratory compromise. She declined definitive surgical intervention and was therefore managed conservatively. After adhering to the wall-mounted suction method for 49 days, we obtained for use a portable digital thoracic drainage system previously used only in the cardiothoracic postoperative patient. This electronically delivered, negative-pressure drainage system induced radiographic improvement within 24 h, and allowed the patient to mobilise for the first time since admission. The patient was discharged home with the Thopaz drain in situ 8 weeks after placing it, and the drain was removed successfully with a resolved pneumothorax 20 weeks after her initial presentation. PMID:22684832

  20. [Idiopathic headache in childhood and adolescence].

    PubMed

    Karwautz, A; Wöber-Bingöl, C; Wöber, C

    1993-12-01

    This review of the literature covers classification, epidemiology and clinical aspects of idiopathic headache in childhood and adolescence. In addition, pathogenetic models taking into account the complex involvement of organic, psychological and psychosocial factors are critically reviewed. A general pathogenetic model of migraine may be characterized by a given predisposition, various co-factors which enhance the tendency, and finally, trigger mechanisms which induce an attack. No generally accepted model exists for tension-type headache. In assessing the importance of various factors thought to be related to idiopathic headache, it is necessary to differentiate between causal relation, unspecific association, and coincidence. The aim of this review is to present potential factors influencing headache in childhood and adolescence and to discuss these factors critically with regard to their etiopathogenetic importance. Organic factors seem to be most important in migraine, whereas psychological and (psycho)social factors may influence any type of headache. Briefly, migraine in childhood and adolescence seems to be definitively associated with vegetative dysfunction, abdominal symptoms and hormonal factors and possibly with allergic reactions, whereas a relation to epilepsy can be excluded. There is absolutely no evidence for a typical personality of migraine patients. Various psychic reactions, however, are important in all types of headache. Depression and anxiety in young headache patients seem to be related generally to pain, but not specifically to headache. However, school problems, learning disabilities and stress coping behaviour seem to be related directly to recurrent headache. Additionally, there is evidence that the prevalence of headache is associated with low economic status. PMID:8114976

  1. Microcatheter Embolization of Intractable Idiopathic Epistaxis

    SciTech Connect

    Leppaenen, Martti; Seppaenen, Seppo; Laranne, Jussi; Kuoppala, Katriina

    1999-11-15

    Purpose: To assess the efficacy and safety of microcatheter embolization in the treatment of intractable idiopathic epistaxis. Methods: Thirty-seven patients underwent microcatheter embolization in 1991-1998. We evaluated retrospectively the technical and clinical outcome, the number of complications, the duration of embolization in each case, and the number of blood transfusions needed. All embolizations were done with biplane digital subtraction angiography (DSA) equipment. The procedure was carried out under local anesthesia using transfemoral catheterization, except in one case where the translumbar route was used. Tracker 18 or 10 microcatheters were advanced as far as possible to the distal branches of the sphenopalatine artery. Polyvinyl alcohol (PVA) particles were used for embolization in most cases, while platinum coils or a combination of these two materials were occasionally used. The primary outcome was always assessed immediately by angiography. Follow-up data were obtained from patient records, by interviewing patients on the telephone or by postal questionnaires when necessary. The mean follow-up time was 21 months. Results: The embolization was technically successful in all 37 cases. A curative outcome was achieved in 33 cases (89%). The mean duration of the procedure was 110 min. Four patients (8%) had mild transient complications, but no severe or persistent complications were encountered. Twenty-three patients needed a blood transfusion. Slight rebleeding occurred in three patients during the follow-up; all responded to conservative treatment. One patient suffered two episodes of rebleeding within 2 months after primary embolization. Re-embolizations successfully stopped the bleeding. Conclusion: Embolization is the primary invasive modality for treating intractable idiopathic epistaxis. It proved both safe and effective over a relatively long follow-up.

  2. Impact of juvenile idiopathic arthritis on schooling

    PubMed Central

    2013-01-01

    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<0.0001), while 33% of children with JIA were unable to attend school due to their condition. The students with JIA who were able to attend school were absent much more often than controls (63% compared to 20%), with a highly significant p value (p<0.0001). Slightly less than half of the JIA patients (48.5%) failed in their schooling. In univariate analysis, there was an association between absenteeism and tender joints (p=0.02), disease activity score (DAS28) (p=0.007), Childhood Health Assessment Questionnaire (CHAQ) (p=0.01), and erythrocyte sedimentation rate (ESR) (p=0.03). In multivariate analysis, the only association persisted between DAS28 and absenteeism. 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. PMID:23289498

  3. Idiopathic corporeal hemihypertrophy associated with hemihypertrichosis.

    PubMed

    Maniar, S; Azzi, K; Iraqi, H; El Hassan Garbi, M; Chraibi, A; Gaouzi, A

    2011-02-01

    The hemihypertrophy or hemihyperplasy is a rare congenital abnormality, characterized by an asymmetric growth of the limbs, the trunk, and the face or half of the entire body. It may be isolated or be part of several syndromes including Beckwith-Wiedemann syndrome, Klippel-Trenaunay-Weber syndrome, Silver-Russell syndrome and Proteus syndrome. In its isolated form, it is called idiopathic. The latter may be associated with several anomalies including dermatological and urogenital abnormalities with increased risk of developing embryonal tumors. We report the case of a 22-month-old infant, who was referred by his pediatrician at the age of 15 months for a corporeal hemihypertrophy associated with hemihypertrichosis. In his medical history, a second degree parental consanguinity and a hypospadias in the father and a paternal uncle were found. Clinical examination found a weight and a size greater than chronological age (3 standard deviations), a hemihypertrophy of entire left side with a difference of length and diameter between the left and right limbs of 2 cm. The hemihypertrichosis was widespread in the left body and the genital examination found a hypospadias. Biological and radiological assessments did not show any abnormality, with the exception of an initially high plasma testosterone level, which gradually normalized. Thus, the diagnosis of idiopathic hemihypertrophy with congenital hemihypertrichosis was retained. This is the fourth case reported in the literature. Its management is similar to all hemihypertrophies, which consists of an initial assessment to detect an embryonic tumor, followed by a monitoring protocol including an abdominal and renal ultrasound every 6 months until the age of 8, determination of alpha-feto-protein every 6 to 12 weeks until the age of 4 years to track the development of the two most frequent tumors: Wilms tumor and hepatoblastoma. The hemihypertrophy associated with hemihypertrichosis has been exceptionally reported and the

  4. Idiopathic focal epilepsies: the "lost tribe".

    PubMed

    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

  5. Analgesia in post-thoracotomy patients: Comparison between thoracic epidural and thoracic paravertebral blocks

    PubMed Central

    Mukherjee, Maitreyee; Goswami, Anupam; Gupta, Sampa Dutta; Sarbapalli, Debabrata; Pal, Ranabir; Kar, Sumit

    2010-01-01

    Background: Acute postoperative pain can cause detrimental effects on multiple organ systems, leading to chronic pain syndromes. Objective: To compare thoracic epidural block (TEB) and paravertebral block (PVB) for relief of postoperative pain in adult patients undergoing thoracotomy. Materials and Methods: In this randomized, single-blinded, prospective study, 60 adult patients of both sexes, belonging to ASA physical status I and II, were scheduled for elective thoracotomy under general anesthesia. They were randomly divided into two groups, A and B of 30 each, who were comparable in terms of demographic parameters and body weight. Group A received TEB and Group B received PVB. All the patients underwent thoracotomy under general anesthesia using a uniform standard anesthetic technique. Thirty minutes before the anticipated end of skin suture, blocks were activated in both the groups with 7.5 ml for TEB and 15 ml for thoracic PVB of 0.25% bupivacaine, along with 1 ml of fentanyl for postoperative analgesia. Results: Patients receiving PVB for postoperative analgesia experienced better analgesia than those receiving TEB from the immediate postoperative period that lasted longer. Intragroup comparison showed that in the cases receiving TEB, there was a significant statistical difference in preoperative and postoperative values with regard to the mean systolic blood pressure (SBP), mean arterial pressure and mean pulse rate. However, in patients receiving PVB, significant difference in preoperative and postoperative values was seen in mean SBP only. Conclusions: We observed longer duration of analgesia with PVB compared to TEB. PMID:25885234

  6. High spatial and temporal resolution 4D FEM simulation of the thoracic bioimpedance using MRI scans

    NASA Astrophysics Data System (ADS)

    Ulbrich, Mark; Marleaux, Bastian; Mühlsteff, Jens; Schoth, Felix; Koos, Ralf; Teichmann, Daniel; Leonhardt, Steffen

    2013-04-01

    In this work, a finite element model was created using MRI scans of the main author to analyze sources of the dynamic thoracic bioimpedance. This model can be used to identify limitations of impedance cardiography (ICG) in practice. Heart beat (8.3 ms temporal resolution) and aortic wave propagation (2.6 ms temporal resolution) were implemented. The static volume contains all major organs of the thorax in high spatial resolution. Simulations were successfully conducted and a high correlation (r = 0.9) between the simulated aortic ICG signal and a measured signal of the same subject was obtained.

  7. Sleepiness in Idiopathic REM Sleep Behavior Disorder and Parkinson Disease

    PubMed Central

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

    2015-01-01

    Objective: 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. Methods: 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. Results: 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 < 0.0001). They reached the levels of 114 patients with Parkinson disease (ESS: 8.7 ± 4.8), whether they had (n = 78) or did not have (n = 36) concomitant RBD. The severity of sleepiness in idiopathic RBD correlated with younger age, but not with 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). Conclusions: 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. Citation: Arnulf I, Neutel D, Herlin B, Golmard JL, Leu-Semenescu S, Cochen de Cock V, Vidailhet M. Sleepiness in idiopathic REM sleep behavior disorder and Parkinson disease. SLEEP 2015;38(10):1529–1535. PMID:26085299

  8. [Unilateral idiopathic gingival fibromatosis--a case report].

    PubMed

    Łazarz-Bartyzel, Katarzyna; Gawron, Katarzyna; Darczuki, Dagmara; Chomyszyn-Gajewska, Maria

    2016-01-01

    Gingival fibromatosis is a painless gingival overgrowth. It may result in difficulties with proper dental hygiene keeping, mastication and occlusion. Herein, a case of a 10-year-old patient was described. The patient reported to the Department of Periodontology and Oral Medicine of the Jagiellonian University Medical College in Krakow due to the problems with permanent teeth eruption (23-26), chewing and dental hygiene maintaining. Based on medical history, clinical examination, diagnostic tests and histopathological study of gingival tissue biopsies the patient was diagnosed with unilateral idiopathic gingival fibromatosis. After oral cavity hygienization, patient un- derwent dental surgery procedures by gingivectomy and gingivoplasty. The follow-up examination 2 and 6 months post operation showed un- eventful healing, proper tooth eruption, improved oral hygiene and chewing function. Twelve months post surgery no recurrence was noted. Due to the etiological diversity of gingival lesions occurring as an overgrowth, accurate medical history, clinical examination, laboratory tests and histopathological study are needed. Accurate diagnos- tics is crucial mainly to exclude he- matological and oncological diseases. Gingivectomy being the "gold method" of gingival fibromatosis treatment was effective and sufficient to cure the case presented in this article. PMID:27526431

  9. The lived experience with idiopathic pulmonary fibrosis: a qualitative study.

    PubMed

    Overgaard, Dorthe; Kaldan, Gudrun; Marsaa, Kristoffer; Nielsen, Thyge Lynghøj; Shaker, Saher Burhan; Egerod, Ingrid

    2016-05-01

    The disease course in idiopathic pulmonary fibrosis (IPF) is variable, but patients experience a progressive decline in lung function and increased symptom burden leading to death. Little is known about the patients' experience and their needs during the disease course or about the burden on family caregivers. Both patients and family caregivers face an altered life as the disease progresses. The aim of our study was to increase knowledge of life with IPF for patients and family caregivers.This study had a qualitative descriptive design using in-depth dyadic interviews with IPF patients (n=25) and family caregivers (n=24). We used the five-step analysis from the framework method and analysed the data on three levels: the patient, the family caregivers and couple level.The following six themes emerged as the main results: information and disclosure, reactional dyssynchrony, perpetual vigilance, emotional ambivalence, gradual and tacit role shift, and adapted coping strategies.Our findings suggest that IPF patients need information at the time of diagnosis, but some issues should be paced as the disease progresses. A palliation plan was demanded by patients and their caregivers. Further efforts are required to provide palliative care to IPF patients starting at the time of diagnosis. PMID:26846831

  10. Idiopathic intracranial hypertension in the Middle East: A growing concern

    PubMed Central

    Almarzouqi, Sumayya J.; Morgan, Michael L.; Lee, Andrew G.

    2014-01-01

    Idiopathic Intracranial Hypertension (IIH) is a disorder of increased intracranial pressure without any identifiable etiology. It is defined by elevated intracranial pressure (ICP) with normal neuroimaging and normal cerebrospinal fluid (CSF) contents. IIH typically affects young obese women and produces symptoms and signs related to high ICP. Headache and blurred vision are the most common symptoms, and papilledema is the major clinical sign. In this review we examine the epidemiology and demographic features of IIH in Middle Eastern countries and compare and contrast them with the published IIH literature from Western countries. The incidence of IIH in several Middle East countries has been estimated at 2.02–2.2/100,000 in the general population, which is higher than the Western rate. Obesity is a major risk factor globally and it is associated with an increased risk of severe vision loss due to IIH. There has been an increase in obesity prevalence in the Middle East countries mainly affecting the Gulf Council Countries (GCC), which parallels increased industrial development. This rise may be contributing to the increasing incidence of IIH in these countries. Other risk factors may also be contributing to IIH in Middle East countries and the differences and similarities to Western IIH merit further study. PMID:25859136

  11. Mutant GABA(A) receptor subunits in genetic (idiopathic) epilepsy.

    PubMed

    Hirose, Shinichi

    2014-01-01

    The γ-aminobutyric acid receptor type A (GABAA receptor) is a ligand-gated chloride channel that mediates major inhibitory functions in the central nervous system. GABAA receptors function mainly as pentamers containing α, β, and either γ or δ subunits. A number of antiepileptic drugs have agonistic effects on GABAA receptors. Hence, dysfunctions of GABAA receptors have been postulated to play important roles in the etiology of epilepsy. In fact, mutations or genetic variations of the genes encoding the α1, α6, β2, β3, γ2, or δ subunits (GABRA1, GABRA6, GABRB2, GABRB3, GABRG2, and GABRD, respectively) have been associated with human epilepsy, both with and without febrile seizures. Epilepsy resulting from mutations is commonly one of following, genetic (idiopathic) generalized epilepsy (e.g., juvenile myoclonic epilepsy), childhood absence epilepsy, genetic epilepsy with febrile seizures, or Dravet syndrome. Recently, mutations of GABRA1, GABRB2, and GABRB3 were associated with infantile spasms and Lennox-Gastaut syndrome. These mutations compromise hyperpolarization through GABAA receptors, which is believed to cause seizures. Interestingly, most of the insufficiencies are not caused by receptor gating abnormalities, but by complex mechanisms, including endoplasmic reticulum (ER)-associated degradation, nonsense-mediated mRNA decay, intracellular trafficking defects, and ER stress. Thus, GABAA receptor subunit mutations are now thought to participate in the pathomechanisms of epilepsy, and an improved understanding of these mutations should facilitate our understanding of epilepsy and the development of new therapies. PMID:25194483

  12. Investigation of pulsatile flowfield in healthy thoracic aorta models.

    PubMed

    Wen, Chih-Yung; Yang, An-Shik; Tseng, Li-Yu; Chai, Jyh-Wen

    2010-02-01

    Cardiovascular disease is the primary cause of morbidity and mortality in the western world. Complex hemodynamics plays a critical role in the development of aortic dissection and atherosclerosis, as well as many other diseases. Since fundamental fluid mechanics are important for the understanding of the blood flow in the cardiovascular circulatory system of the human body aspects, a joint experimental and numerical study was conducted in this study to determine the distributions of wall shear stress and pressure and oscillatory WSS index, and to examine their correlation with the aortic disorders, especially dissection. Experimentally, the Phase-Contrast Magnetic Resonance Imaging (PC-MRI) method was used to acquire the true geometry of a normal human thoracic aorta, which was readily converted into a transparent thoracic aorta model by the rapid prototyping (RP) technique. The thoracic aorta model was then used in the in vitro experiments and computations. Simulations were performed using the computational fluid dynamic (CFD) code ACE+((R)) to determine flow characteristics of the three-dimensional, pulsatile, incompressible, and Newtonian fluid in the thoracic aorta model. The unsteady boundary conditions at the inlet and the outlet of the aortic flow were specified from the measured flowrate and pressure results during in vitro experiments. For the code validation, the predicted axial velocity reasonably agrees with the PC-MRI experimental data in the oblique sagittal plane of the thoracic aorta model. The thorough analyses of the thoracic aorta flow, WSSs, WSS index (OSI), and wall pressures are presented. The predicted locations of the maxima of WSS and the wall pressure can be then correlated with that of the thoracic aorta dissection, and thereby may lead to a useful biological significance. The numerical results also suggest that the effects of low WSS and high OSI tend to cause wall thickening occurred along the inferior wall of the aortic arch and the

  13. Association of exposure to phenols and idiopathic male infertility.

    PubMed

    Chen, Minjian; Tang, Rong; Fu, Guangbo; Xu, Bin; Zhu, Pengfei; Qiao, Shanlei; Chen, Xiaojiao; Xu, Bo; Qin, Yufeng; Lu, Chuncheng; Hang, Bo; Xia, Yankai; Wang, Xinru

    2013-04-15

    Widespread human exposure to phenols has been documented recently, and some phenols which are potential endocrine disruptors have demonstrated adverse effects on male reproduction in animal and in vitro studies. However, implications about exposure to phenols and male infertility are scarce in humans. Case-control study of 877 idiopathic infertile men and 713 fertile controls was conducted. Urinary levels of bisphenol A, benzophenone-3, pentachlorophenol, triclosan, 4-tert-octylphenol (4-t-OP), 4-n-octylphenol (4-n-OP) and 4-n-nonylphenol (4-n-NP) and semen parameters were measured. After multivariate adjustment, we found 4-t-OP, 4-n-OP and 4-n-NP exposure was associated with idiopathic male infertility (p-value for trend: <0.0001, 0.014 and 0.001, respectively). Aside from these associations, 4-t-OP and 4-n-NP exposure was also associated with idiopathic male infertility with abnormal semen parameters. Moreover, we observed significant associations between sum alkylphenols (APs) exposure and idiopathic male infertility. There were no relationships between exposure to other phenols and idiopathic male infertility in the present study. Our study provides the first evidence that exposure to APs (4-t-OP, 4-n-OP and 4-n-NP) is associated with idiopathic male infertility. PMID:23435201

  14. Surgical treatment of chronic idiopathic thrombocytopenic purpura: results in 107 cases

    SciTech Connect

    Cola, B.; Tonielli, E.; Sacco, S.; Brulatti, M.; Franchini, A.

    1986-07-01

    Between 1972 and 1985, 107 patients with chronic Idiopathic Thrombocytopenic Purpura underwent splenectomy. Platelet life span and sites of sequestration were studied with labelled platelets and external scanning. Medical treatment was always of scarce and transient effectiveness and had considerable side effects. Splenectomy had minimal complications and mortality and caused no hazard of overwhelming sepsis in adults. The results of splenectomy were very satisfying, especially when platelet sequestration was mainly splenic (remission in about 90% of patients). Surgical treatment is at present the most effective in patients with chronic ITP.

  15. Recommendations for research studies on treatment of idiopathic scoliosis: Consensus 2014 between SOSORT and SRS non-operative management committee.

    PubMed

    Negrini, Stefano; Hresko, Timothy M; O'Brien, Joseph P; Price, Nigel

    2015-01-01

    The two main societies clinically dealing with idiopathic scoliosis are the Scoliosis Research Society (SRS), founded in 1966, and the international Society on Scoliosis Orthopedic and Rehabilitation Treatment (SOSORT), started in 2004. Inside the SRS, the Non-Operative Management Committee (SRS-NOC) has the same clinical interest of SOSORT, that is the Orthopaedic and Rehabilitation (or Non-Operative, or conservative) Management of idiopathic scoliosis patients. The aim of this paper is to present the results of a Consensus among the best experts of non-operative treatment of Idiopathic Scoliosis, as represented by SOSORT and SRS, on the recommendation for research studies on treatment of Idiopathic Scoliosis. The goal of the consensus statement is to establish a framework for research with clearly delineated inclusion criteria, methodologies, and outcome measures so that future meta- analysis or comparative studies could occur. A Delphi method was used to generate a consensus to develop a set of recommendations for clinical studies on treatment of Idiopathic Scoliosis. It included the development of a reference scheme, which was judged during two Delphi Rounds; after this first phase, it was decided to develop the recommendations and 4 other Delphi Rounds followed. The process finished with a Consensus Meeting, that was held during the SOSORT Meeting in Wiesbaden, 8-10 May 2014, moderated by the Presidents of SOSORT (JP O'Brien) and SRS (SD Glassman) and by the Chairs of the involved Committees (SOSORT Consensus Committee: S Negrini; SRS Non-Operative Committee: MT Hresko). The Boards of the SRS and SOSORT formally accepted the final recommendations. The 18 Recommendations focused: Research needs (3), Clinically significant outcomes (4), Radiographic outcomes (3), Other key outcomes (Quality of Life, adherence to treatment) (2), Standardization of methods of non-operative research (6). PMID:25780381

  16. Imaging the pre- and postsynaptic side of striatal dopaminergic synapses in idiopathic cervical dystonia: a SPECT study using [123I] epidepride and [123I] beta-CIT.

    PubMed

    Naumann, M; Pirker, W; Reiners, K; Lange, K W; Becker, G; Brücke, T

    1998-03-01

    There is increasing evidence that a dysfunction of the dopaminergic system may be involved in the pathogenesis of idiopathic dystonia. To visualize possible alterations of the pre- and postsynaptic side of striatal dopaminergic synapses, SPECT studies using the radiotracers [123I] epidepride and [123I] beta-CIT were performed in 10 patients with idiopathic cervical dystonia. Eleven age- and sex-matched subjects served as controls. [123I] Epidepride is a new highly affine marker of D2 receptors, and [123I] beta-CIT binds to dopamine transporters on dopaminergic nerve endings. [123I] Epidepride binding was significantly reduced in both striata of dystonia patients compared with controls (p < 0.05). In contrast, striatal [123I beta-CIT uptake did not differ from controls. We conclude that dopaminergic dysfunction in idiopathic focal dystonia mainly involves postsynaptic mechanisms and suggest a disturbance of the indirect pathway of the motor circuit resulting in a disinhibited thalamocortical stimulation. PMID:9539347

  17. Towards a comprehensive CT image segmentation for thoracic organ radiation dose estimation and reporting

    NASA Astrophysics Data System (ADS)

    Lorenz, Cristian; Ruppertshofen, Heike; Vik, Torbjörn; Prinsen, Peter; Wiegert, Jens

    2014-03-01

    Administered dose of ionizing radiation during medical imaging is an issue of increasing concern for the patient, for the clinical community, and for respective regulatory bodies. CT radiation dose is currently estimated based on a set of very simplifying assumptions which do not take the actual body geometry and organ specific doses into account. This makes it very difficult to accurately report imaging related administered dose and to track it for different organs over the life of the patient. In this paper this deficit is addressed in a two-fold way. In a first step, the absorbed radiation dose in each image voxel is estimated based on a Monte-Carlo simulation of X-ray absorption and scattering. In a second step, the image is segmented into tissue types with different radio sensitivity. In combination this allows to calculate the effective dose as a weighted sum of the individual organ doses. The main purpose of this paper is to assess the feasibility of automatic organ specific dose estimation. With respect to a commercially applicable solution and respective robustness and efficiency requirements, we investigated the effect of dose sampling rather than integration over the organ volume. We focused on the thoracic anatomy as the exemplary body region, imaged frequently by CT. For image segmentation we applied a set of available approaches which allowed us to cover the main thoracic radio-sensitive tissue types. We applied the dose estimation approach to 10 thoracic CT datasets and evaluated segmentation accuracy and administered dose and could show that organ specific dose estimation can be achieved.

  18. Regional interdependence and manual therapy directed at the thoracic spine.

    PubMed

    McDevitt, Amy; Young, Jodi; Mintken, Paul; Cleland, Josh

    2015-07-01

    Thoracic spine manipulation is commonly used by physical therapists for the management of patients with upper quarter pain syndromes. The theoretical construct for using thoracic manipulation for upper quarter conditions is a mainstay of a regional interdependence (RI) approach. The RI concept is likely much more complex and is perhaps driven by a neurophysiological response including those related to peripheral, spinal cord and supraspinal mechanisms. Recent evidence suggests that thoracic spine manipulation results in neurophysiological changes, which may lead to improved pain and outcomes in individuals with musculoskeletal disorders. The intent of this narrative review is to describe the research supporting the RI concept and its application to the treatment of individuals with neck and/or shoulder pain. Treatment utilizing both thrust and non-thrust thoracic manipulation has been shown to result in improvements in pain, range of motion and disability in patients with upper quarter conditions. Research has yet to determine optimal dosage, techniques or patient populations to which the RI approach should be applied; however, emerging evidence supporting a neurophysiological effect for thoracic spine manipulation may negate the need to fully answer this question. Certainly, there is a need for further research examining both the clinical efficacy and effectiveness of manual therapy interventions utilized in the RI model as well as the neurophysiological effects resulting from this intervention. PMID:26309384

  19. Mechanism of the formation for thoracic impedance change.

    PubMed

    Kuang, Ming-Xing; Xiao, Qiu-Jin; Cui, Chao-Ying; Kuang, Nan-Zhen; Hong, Wen-Qin; Hu, Ai-Rong

    2010-03-01

    The purpose of this study is to investigate the mechanism of the formation for thoracic impedance change. On the basis of Ohm's law and the electrical field distribution in the cylindrical volume conductor, the formula about the thoracic impedance change are deduced, and they are demonstrated with the model experiment. The results indicate that the thoracic impedance change caused by single blood vessel is directly proportional to the ratio of the impedance change to the basal impedance of the blood vessel itself, to the length of the blood vessel appearing between the current electrodes, and to the basal impedance between two detective electrodes on the chest surface, while it is inversely proportional to the distance between the blood vessel and the line joining two detective electrodes. The thoracic impedance change caused by multiple blood vessels together is equal to the algebraic addition of all thoracic impedance changes resulting from the individual blood vessels. That is, the impedance changes obey the principle of adding scalars in the measurement of the electrical impedance graph. The present study can offer the theoretical basis for the waveform reconstruction of Impedance cardiography (ICG). PMID:20336823

  20. Endovascular aortic injury repair after thoracic pedicle screw placement.

    PubMed

    Pesenti, S; Bartoli, M A; Blondel, B; Peltier, E; Adetchessi, T; Fuentes, S

    2014-09-01

    Our objective was to describe the management and prevention of thoracic aortic injuries caused by a malposition of pedicle screws in corrective surgery of major spine deformities. Positioning pedicle screws in thoracic vertebras by posterior approach exposes to the risk of injury of the elements placed ahead of the thoracic spine, as the descending thoracic aorta. This complication can result in a cataclysmic bleeding, needing urgent vascular care, but it can also be totally asymptomatic, resulting in the long run in a pseudoaneurysm, justifying the systematic removal of the hardware. We report the case of a 76-year-old woman who underwent spinal correction surgery for thoraco-lumbar degenerative kypho-scoliosis. Immediately after the surgery, a thoracic aortic injury caused by the left T7 pedicle screw was diagnosed. The patient underwent a two-step surgery. The first step was realized by vascular surgeons and aimed to secure the aortic wall by short endovascular aortic grafting. During the second step, spine surgeons removed the responsible screw by posterior approach. The patient was discharged in a rehabilitation center 7 days after the second surgery. When such a complication occurs, a co-management by vascular and spine surgeons is necessary to avoid major complications. Endovascular management of this kind of vascular injuries permits to avoid an open surgery that have a great rate of morbi-mortality in frail patients. Nowadays, technologies exist to prevent this kind of event and may improve the security when positioning pedicle screws. PMID:25023930

  1. Surgical treatment analysis of idiopathic esophageal achalasia

    PubMed Central

    de AQUINO, José Luis Braga; SAID, Marcelo Manzano; PEREIRA, Douglas Rizzanti; do AMARAL, Paula Casals; LIMA, Juliana Carolina Alves; LEANDRO-MERHI, Vânia Aparecida

    2015-01-01

    Background Idiopathic esophageal achalasia is an inflammatory disease of unknown origin, characterized by aperistalsis of the esophageal body and failure of the lower esophageal sphincter in response to swallowing, with consequent dysphagia. Aim To demonstrate the results of surgical therapy in these patients, evaluating the occurred local and systemic complications. Methods Were studied retrospectively 32 patients, 22 of whom presented non-advanced stage of the disease (Stage I/II) and 10 with advanced disease (Stage III/IV). All of them had the clinical conditions to be submitted to surgery. The diagnoses were done by clinical, endoscopic, cardiological, radiological and esophageal manometry analysis. Pre-surgical evaluation was done with a questionnaire based on the most predisposing factors in the development of the disease and the surgical indication was based on the stage of the disease. Results The patients with non-advanced stages were submitted to cardiomyotomy with fundoplication, wherein in the post-surgical early assessment, only one (4,4%) presented pulmonary infection, but had a good outcome. In patients with advanced disease, seven were submitted to esophageal mucosectomy preserving the muscular layer, wherein one patient (14,2%) presented dehiscence of gastric cervical esophagus anastomosis as well as pulmonary infection; all of these complications were resolved with proper specific treatment; the other three patients with advanced stage were submitted to transmediastinal esophagectomy; two of them presented hydropneumothorax with good evolution, and one of them also presented fistula of the cervical esophagogastric anastomosis, but with spontaneous healing after conservative treatment and nutritional support. The two patients with fistula of the cervical anastomosis progressed to stenosis, with good results after endoscopic dilations. In the medium and long term assessment done in 23 patients, all of them reported improvement in life quality, with

  2. Deadly dozen: dealing with the 12 types of thoracic injuries.

    PubMed

    Cipolle, Mark; Rhodes, Michael; Tinkoff, Glen

    2012-09-01

    Although most thoracic trauma may be treated non-operatively, major thoracic trauma accounts for 25% of trauma deaths. Except for provision of a definitive airway and/or relief of a tension pneumothorax with a needle decompression, the vast majority of thoracic trauma is best served with "load and go," high-flow oxygen, placement of an IV line and administration of crystalloid solutions as the clinical scenario would indicate. Understanding the mechanism of injury is helpful in establishing both prehospital and in-hospital management priorities. Patients who sustain a single penetrating wound to the chest have the best survivability after a resuscitative thoracotomy. Practicing chest assessment skills is vital to being a good prehospital provider. Ultrasound, NIRS tissue oxygenation and telemedicine will likely become more commonly employed as prehospital monitoring options. PEEP, or "over bagging," may exacerbate a simple or open pneumothorax, converting it to a tension pneumothorax. PMID:23342703

  3. Neurogenic thoracic outlet and pectoralis minor syndromes in children.

    PubMed

    Sanders, Richard J; Annest, Stephen J; Goldson, Edward

    2013-07-01

    Brachial plexus compression (BPC) occurs above the clavicle as neurogenic thoracic outlet syndrome (NTOS) and below as neurogenic pectoralis minor syndrome (NPMS). It was recently noted that 75% of the adults seen for NTOS also had NPMS and in some this was the only diagnosis. This is also true in children but has not yet been reported. Because surgical treatment of NPMS is a minimum risk operation for pectoralis minor tenotomy (PMT), recognition of NPMS and distinguishing it from NTOS becomes important. In this study, 40 operations, 20 PMT and 20 NTOS procedures, were performed. Success rate for PMT was 85% and for thoracic outlet decompression was 70%. It was concluded that in children, as in adults, BPC is more often due to combined NTOS and NPMS. Surgical PMT should be considered first as the treatment of choice for children with NPMS. Thoracic outlet decompression is available if PMT is unsuccessful. PMID:23503361

  4. A comparison of thoracic or lumbar patient-controlled epidural analgesia methods after thoracic surgery

    PubMed Central

    2014-01-01

    Background We aimed to compare patient-controlled thoracic or lumbar epidural analgesia methods after thoracotomy operations. Methods One hundred and twenty patients were prospectively randomized to receive either thoracic epidural analgesia (TEA group) or lumbar epidural analgesia (LEA group). In both groups, epidural catheters were administered. Hemodynamic measurements, visual analog scale scores at rest (VAS-R) and after coughing (VAS-C), analgesic consumption, and side effects were compared at 0, 2, 4, 8, 16, and 24 hours postoperatively. Results The VAS-R and VAS-C values were lower in the TEA group in comparison to the LEA group at 2, 4, 8, and 16 hours after surgery (for VAS-R, P = 0.001, P = 0.01, P = 0.008, and P = 0.029, respectively; and for VAS-C, P = 0.035, P = 0.023, P = 0.002, and P = 0.037, respectively). Total 24-hour analgesic consumption was different between groups (175 +/- 20 mL versus 185 +/- 31 mL; P = 0.034). The comparison of postoperative complications revealed that the incidence of hypotension (21/57, 36.8% versus 8/63, 12.7%; P = 0.002), bradycardia (9/57, 15.8% versus 2/63, 3.2%; P = 0.017), atelectasis (1/57, 1.8% versus 7/63, 11.1%; P = 0.04), and the need for intensive care unit (ICU) treatment (0/57, 0% versus 5/63, 7.9%; P = 0.03) were lower in the TEA group in comparison to the LEA group. Conclusions TEA has beneficial hemostatic effects in comparison to LEA after thoracotomies along with more satisfactory pain relief profile. PMID:24885545

  5. Donor to recipient sizing in thoracic organ transplantation

    PubMed Central

    Eberlein, Michael; Reed, Robert M

    2016-01-01

    Donor-to-recipient organ size matching is a critical aspect of thoracic transplantation. In the United States potential recipients for lung transplant and heart transplant are listed with limitations on donor height and weight ranges, respectively. Height is used as a surrogate for lung size and weight is used as a surrogate for heart size. While these measures are important predictors of organ size, they are crude surrogates that fail to incorporate the influence of sex on organ size. Independent of other measures, a man’s thoracic organs are approximately 20% larger than a woman’s. Lung size can be better estimated using the predicted total lung capacity, which is derived from regression equations correcting for height, sex and age. Similarly, heart size can be better estimated using the predicted heart mass, which adjusts for sex, age, height, and weight. These refined organ sizing measures perform better than current sizing practice for the prediction of outcomes after transplantation, and largely explain the outcome differences observed after sex-mismatch transplantation. An undersized allograft is associated with worse outcomes. In this review we examine current data pertaining to size-matching in thoracic transplantation. We advocate for a change in the thoracic allocation mechanism from a height-or-weight-based strategy to a size-matching process that utilizes refined estimates of organ size. We believe that a size-matching approach based on refined estimates of organ size would optimize outcomes in thoracic transplantation without restricting or precluding patients from thoracic transplantation. PMID:27011913

  6. Prospective Evaluation of Thoracic Ultrasound in the Detection of Pneumothorax

    NASA Technical Reports Server (NTRS)

    Schwarz, K. W.; Hamilton, D. R.; Kirkpatrick, A. W.; Billica, R. D.; Williams, D. R.; Diebel, L. N.; Sargysan, A. E.; Dulchavsky, S. A.

    2000-01-01

    Introduction: Pneumothorax (PTX) occurs commonly in trauma patients and is confirmed by examination and radiography. Thoracic ultrasound (VIS) has been suggested as an alternative method for rapidly diagnosing PTX when X-ray is unavailable as in rural, military, or space flight settings; however, its accuracy and specificity are not known. Methods: We evaluated the accuracy of thoracic U/S detection of PTX compared to radiography in stable, emergency patients with a high suspicion of PTX at a Level-l trauma center over a 6-month period. Following University and NASA Institutional Review Board approval, informed consent was obtained from patients with penetrating or blunt chest trauma, or with a history consistent with PTX. Whenever possible, the presence or absence of the " lung sliding" sign or the "comet tail" artifact were determined by U/S in both hemithoraces by residents instructed in thoracic U/S before standard radiologic verification of PTX. Results were recorded on data sheets for comparison to standard radiography. Results: Thoracic VIS had a 94% sensitivity; two PTX could not be reliably diagnosed due to subcutaneous air; the true negative rate was 100%. In one patient, the VIS exam was positive while X ray did not confirm PTX; a follow-up film 1 hour later demonstrated a small PTX. The average time for bilateral thoracic VIS examination was 2 to 3 minutes. Conclusions: Thoracic ultrasound reliably diagnoses pneumothorax. Presence of the "lung sliding" sign conclusively excludes pneumothorax. Expansion of the FAST examination to include the thorax should be investigated.

  7. Three-dimensional reconstruction of thoracic structures: based on Chinese Visible Human.

    PubMed

    Wu, Yi; Luo, Na; Tan, Liwen; Fang, Binji; Li, Ying; Xie, Bing; Liu, Kaijun; Chu, Chun; Li, Min; Zhang, Shaoxiang

    2013-01-01

    We managed to establish three-dimensional digitized visible model of human thoracic structures and to provide morphological data for imaging diagnosis and thoracic and cardiovascular surgery. With Photoshop software, the contour line of lungs and mediastinal structures including heart, aorta and its ramus, azygos vein, superior vena cava, inferior vena cava, thymus, esophagus, diaphragm, phrenic nerve, vagus nerve, sympathetic trunk, thoracic vertebrae, sternum, thoracic duct, and so forth were segmented from the Chinese Visible Human (CVH)-1 data set. The contour data set of segmented thoracic structures was imported to Amira software and 3D thorax models were reconstructed via surface rendering and volume rendering. With Amira software, surface rendering reconstructed model of thoracic organs and its volume rendering reconstructed model were 3D reconstructed and can be displayed together clearly and accurately. It provides a learning tool of interpreting human thoracic anatomy and virtual thoracic and cardiovascular surgery for medical students and junior surgeons. PMID:24369489

  8. Retrospective evaluation of CTV to PTV margins using CyberKnife in patients with thoracic tumors.

    PubMed

    Floriano, Alejandro; García, Rafael; Moreno, Ramon; Sánchez-Reyes, Alberto

    2014-01-01

    The objectives of this study were to estimate global uncertainty for patients with thoracic tumors treated in our center using the CyberKnife VSI after placement of fiducial markers and to compare our findings with the standard CTV to PTV margins used to date. Datasets for 16 patients (54 fractions) treated with the CyberKnife and the Synchrony Respiratory Tracking System were analyzed retrospectively based on CT planning, tracking information, and movement data generated and saved in the logs files by the system. For each patient, we analyzed all the main uncertainty sources and assigned a value. We also calculated an expanded global uncertainty to ensure a robust estimation of global uncertainty and to enable us to determine the position of 95% of the CTV points with a 95% confidence level during treatment. Based on our estimation of global uncertainty and compared with our general mar- gin criterion (5 mm in all three directions: superior/inferior [SI], anterior/posterior [AP], and lateral [LAT]), 100% were adequately covered in the LAT direction, as were 94% and 94% in the SI and AP directions. We retrospectively analyzed the main sources of uncertainty in the CyberKnife process patient by patient. This individualized approach enabled us to estimate margins for patients with thoracic tumors treated in our unit and compare the results with our standard 5 mm margin.  PMID:25493508

  9. Non-intubated video-assisted thoracic surgery management of secondary spontaneous pneumothorax

    PubMed Central

    Bolufer, Sergio; Navarro-Martinez, Jose; Lirio, Francisco; Corcoles, Juan Manuel; Rodriguez-Paniagua, Jose Manuel

    2015-01-01

    Secondary spontaneous pneumothorax (SSP) is serious entity, usually due to underlying disease, mainly chronic obstructive pulmonary disease (COPD). Its morbidity and mortality is high due to the pulmonary compromised status of these patients, and the recurrence rate is almost 50%, increasing mortality with each episode. For persistent or recurrent SSP, surgery under general anesthesia (GA) and mechanical ventilation (MV) with lung isolation is the gold standard, but ventilator-induced damages and dependency, and postoperative pulmonary complications are frequent. In the last two decades, several groups have reported successful results with non-intubated video-assisted thoracic surgery (NI-VATS) with thoracic epidural anesthesia (TEA) and/or local anesthesia under spontaneous breathing. Main benefits reported are operative time, operation room time and hospital stay reduction, and postoperative respiratory complications decrease when comparing to GA, thus encouraging for further research in these moderate to high risk patients many times rejected for the standard regimen. There are also reports of special situations with satisfactory results, as in contralateral pneumonectomy and lung transplantation. The aim of this review is to collect, analyze and discuss all the available evidence, and seek for future lines of investigation. PMID:26046045

  10. The role of radiation therapy in thoracic tumors.

    PubMed

    Kong, Feng-Ming Spring; Zhao, Lujun; Hayman, James A

    2006-04-01

    Radiation plays an important role in the treatment of thoracic tumors. During the last 10 years there have been several major advances in thoracic RT including the incorporation of concurrent chemotherapy and the application of con-formal radiation-delivery techniques (eg, stereotactic RT, three-dimensional conformal RT, and intensity-modulated RT) that allow radiation dose escalation. Radiation as a local measure remains the definitive treatment of medically inoperable or surgically unresectable disease in NSCLC and part of a multimodality regimen for locally advanced NSCLC, limited stage SCLC, esophageal cancer, thymoma, and mesothelioma. PMID:16730299

  11. Thoracic outlet syndrome after corrective surgery for pectus excavatum.

    PubMed

    Donders, H P; Geelen, J A

    1988-02-01

    Two patients are described who developed a thoracic outlet syndrome (TOS) after undergoing Ravitch's operation for the correction of pectus excavatum. In one case the syndrome developed a few days after surgery, whereas in the second patient it manifested more gradually. A third patient presented with latent TOS and pectus excavatum. It is recommended that prior to the correction of pectus excavatum, the patient should be examined to detect signs of neurovascular compression due to latent thoracic outlet syndrome. After surgery the possibility of this complication should be kept in mind to avoid permanent lesions of the cervicobrachial plexus. PMID:3352940

  12. Reasons to participate in European Society of Thoracic Surgeons database

    PubMed Central

    2015-01-01

    The process of data collection inevitably involves costs at various levels. Nevertheless, this effort is essential to base our knowledge and the consequent decision making on solid foundations. The European Society of Thoracic Surgeons (ESTS) database collects a large amount of data on general thoracic surgery derived from about 60 units representative of 11 nations. Since its beginning in 2001, the ESTS database has contributed to increase the knowledge and the quality of care in our specialty. The present paper illustrates the ultimate finalities and the obtained results of this data collection, providing a broad overview of the motivations to participate to the ESTS database. PMID:25984355

  13. Aortoesophageal fistula after thoracic endovascular aortic repair and transthoracic embolization.

    PubMed

    Riesenman, Paul J; Farber, Mark A; Mauro, Matthew A; Selzman, Craig H; Feins, Richard H

    2007-10-01

    Endografts are more commonly being used to treat thoracic aortic aneurysms and other vascular lesions. Endoleaks are a potential complication of this treatment modality and can be associated with aneurysmal sac expansion and rupture. This case report presents a patient who developed a type IA endoleak after endograft repair of a descending thoracic aneurysm. The endoleak was successfully treated through computed tomographic-guided transthoracic embolization, although the patient experienced lower extremity paraparesis postprocedurally. The patient's endovascular repair was complicated by the development of an aortoesophageal fistula and endograft infection necessitating operative débridement and endograft explantation. PMID:17903656

  14. Skeletal Dysplasias That Cause Thoracic Insufficiency in Neonates

    PubMed Central

    İpek, Mehmet Sah; Akgul Ozmen, Cihan

    2016-01-01

    Abstract Skeletal dysplasias are a heterogeneous group of conditions associated with various abnormalities of the skeleton. Some of them are perinatally lethal and can be diagnosed at birth. Lethality is usually due to thoracic underdevelopment and lung hypoplasia. A correct diagnosis and typing of the skeletal disorder is essential for the prognosis as is genetic counseling of the family. A retrospective review of 12 cases of clinico-radiologic diagnosis of skeletal dysplasia, leading to thoracic insufficiency, was conducted. We aimed to make differential diagnosis with special emphasis on radiological findings, and to emphasize the importance of parental counseling. PMID:27057899

  15. Dissecting thoracic aortic aneurysm associated with tuberculous pleural effusion

    PubMed Central

    Im, Kyong Shil; Choi, Min Kyung; Jeon, Yong Kyoung

    2016-01-01

    We present the case of thoracic aortic aneurysm associated with the tuberculous pleural effusion. An 82-year-old woman underwent emergency stent graft under a diagnosis of dissecting thoracic aortic aneurysm. Preoperative computed tomography revealed right pleural effusion supposed to the hemothorax caused by the dissecting aneurysm. But, the effusion was sanguineous color fluid and it was determined to result from pulmonary tuberculosis. The medical team was exposed to the pulmonary tuberculosis; fortunately no one became infected. Physicians should be aware of the possibility of an infected aortic aneurysm and prepare for pathogen transmission. PMID:27499987

  16. "Lower limbs revascularization from supraceliac and thoracic aorta".

    PubMed

    Wistuba, Mariel Riedemann; Alonso-Pérez, Manuel; Al-Sibbai, Amer Zanabili; González-Gay, Mario; Alvarez Marcos, Francisco; Camblor, Lino A; Llaneza-Coto, José Manuel

    2015-07-01

    Revascularization of femoral arteries from descending thoracic or supraceliac aorta is an uncommon procedure, in part because of the popularization of the technically easier extra-anatomic bypasses. However, using those aortic levels as the source of the bypass inflow is a useful alternative in selected patients with aortoiliac disease, with excellent results. We report long-term results in 4 patients with revascularization from thoracic aorta and another 2 cases from aorta at supraceliac level. This technique should be considered as a good alternative in patients with adverse abdominal conditions or with a severely diseased infrarenal aorta due to heavy calcification. PMID:25771745

  17. Preoperative Factors Predicting Intraoperative Blood Loss in Female Patients With Adolescent Idiopathic Scoliosis

    PubMed Central

    Li, Chao; Yang, Mingyuan; Wang, Chao; Wang, Chuanfeng; Fan, Jianping; Chen, Ziqiang; Wei, Xianzhao; Zhang, Guoyou; Bai, Yushu; Zhu, Xiaodong; Xie, Yang; Li, Ming

    2015-01-01

    Abstract In this article, a retrospective analysis of 161 female patients with adolescent idiopathic scoliosis (AIS) is performed who underwent posterior correction and fusion using all-pedicle screw instrument. The aim of this article is to find out preoperative factors that influence intraoperative blood loss (IOBL) in female patients with AIS. The IOBL in posterior correction and fusion surgery for patients with idiopathic scoliosis greatly varies. The variables affecting the IOBL also greatly vary among different studies. Medical records of all female patients with AIS who underwent posterior correction and fusion operations using the all-pedicle screw system in our hospital from January 2012 to January 2014 were reviewed. Patients with irregular menstruation, who underwent osteotomy, and using coagulants were excluded. Preoperative clinical data, including patient age, height, weight, Risser sign, day after last menstruation, major curve Cobb angle, fulcrum-bending Cobb angle, curve flexibility index, sagittal thoracic Cobb angle, sagittal lumbar Cobb angle, albumin, hemoglobin, platelet, activated partial thromboplastic time (APTT), prothrombin time, thrombin time, fibrinogen, fusion level, menstrual phase, and blood type, were collected. Data were further analyzed using multiple linear regression with forward elimination. A total of 161 patients were included in this study. The mean IOBL was 933.98 ± 158.10 mL (500–2000 mL). Forward selection showed that fulcrum-bending Cobb angle, fusion level, Risser sign, APTT, fibrinogen, and menstrual phase were the preoperative factors that influenced the IOBL in female patients with AIS. Equation of IOBL was built by multiple linear regression: IOBL = −966.228 + 54.738 Risser sign + 18.910 fulcrum-bending Cobb angle + 114.737 fibrinogen + 21.386 APTT − 71.312 team 2 − 177.985 team 3 − 165.082 team 4 + 53.470 fusion level. R = 0.782. Operation for patients with AIS was featured by large IOBL

  18. Not all chronic urticaria is "idiopathic"!

    PubMed

    Greaves, M W; O'Donnell, B F

    1998-02-01

    The aetiology of chronic urticaria in the majority of patients is elusive. The cause of physical urticarias (dermographism, delayed pressure urticaria, cold urticaria) is unknown. We have identified a subset of chronic "idiopathic" urticaria patients, representing approximately 30% of the total in which the disease is caused by the presence of IgG autoantibodies against the high affinity IgE receptor (FcepsilonRIalpha). This functional autoantibody stimulates normal (albeit vicarious) activation of mast cells and basophils via FcepsilonRI, causing whealing and angioedema. Anti-FcepsilonRI-positive patients are recognized by a combination of autologous serum skin testing and evoked histamine release from basophils of normal human donors. Autoantibody-positive and -negative patients are clinically indistinguishable and are treated routinely by combinations of H1 and occasionally H2-antihistamines in both cases. However, severely affected patients who are anti-FcepsilonRI-positive can also be treated by non-specific immunotherapy (plasma pheresis, intravenous immunoglobulin, cyclosporin. PMID:9517916

  19. Chronic idiopathic urticaria and Graves' disease.

    PubMed

    Ruggeri, R M; Imbesi, S; Saitta, S; Campennì, A; Cannavò, S; Trimarchi, F; Gangemi, S

    2013-01-01

    Chronic urticaria is a common condition characterized by recurrent episodes of mast cell-driven wheal and flare-type skin reactions lasting for more than 6 weeks. In about 75% of cases, the underlying causes remain unknown, and the term chronic idiopathic urticaria (CIU) is used to emphasize that wheals develop independently of identified external stimuli. Although CIU affects about 1.0% of the general population, its etiopathogenesis is not yet well understood. It is now widely accepted that in many cases CIU should be regarded as an autoimmune disorder caused by circulating and functionally active IgG autoantibodies specific for the IgE receptor (FceRI) present on mast cells and basophils or for IgE itself. The well-known association of CIU with other autoimmune processes/diseases represents further indirect evidence of its autoimmune origin. Autoimmune thyroid diseases, especially autoimmune thyroiditis, represent the most frequently investigated diseases in association with CIU. Here we review this topic with particular regard to the association between Graves' disease and CIU. The possible pathogenetic mechanisms and the clinical implications of such an association are discussed. PMID:23609949

  20. Idiopathic hypersomnia in an aircrew member.

    PubMed

    Withers, B G; Loube, D I; Husak, J P

    1999-08-01

    In aviation, it is essential that all aircrew members remain alert and contribute, by their observations and actions, to flight safety. Especially in helicopter operations, crewmembers riding in the rear of the aircraft play an integral role in many aspects of flight, such as take-offs, landings, turns, formation flights, hazard avoidance, situational awareness, military operations, and crew coordination. We present the case of a helicopter crew chief with idiopathic hypersomnia, briefly review the disorder, and give the recent U.S. military aviation experience with sleep disorders. Flight surgeons and aeromedical examiners should be active in considering and diagnosing sleep-related disorders as the aviator or crewmember may not be aware of the disease or may not volunteer the history. A directed history is important in making the diagnosis, as are reports from family and other aircrew members. Referral to a sleep specialist is required in performing objective sleep studies, establishing the diagnosis, recommending treatment, and providing a prognosis. Many sleep disorders are treatable and aeromedically waiverable. PMID:10447054

  1. [Glycosaminoglycans dysbolism in pathogenesis of idiopathic varicocele].

    PubMed

    Shenberger, A B; Bashkatov, S A; Parfenova, T I; Sufiiarova, R Sh

    2008-01-01

    Ultrasound dopplerography (UD) was used to examine 50 patients with idiopathic varicocele (IV) and 20 healthy subjects. Such parameters were assessed as age, severity of IV, its duration, scrotal vessels UD data, vein diameter of the pampiniform plexus on the left, reflux at the height of the Valsalva test, uronic acids (UA), glycosaminoglycanes (GAG) and their fractions (hialuronic acid--HA, chondroitin sulphates--CS, heparansulphates--HS). UA and GAG fraction composition was studied with ion exchange chromatography. Calculations were made of mean values, standard deviations, Student's t-criterion, Pirson's correlation coefficient. It was found that HA and UA concentrations were high. In young patients HA metabolism was disturbed more, in adult patients--disturbance of UA metabolism. Two equal negative moderately strong links were revealed between the patients' age, HS and total GAG: r1 = r2 = -0.56; CI of UA in the patients--21.97-59.09 mcg/ml, in the control group --23.70- 43.42 mcg/ml. With age, the organism adapts to the disease and spontaneous regress may occur. In UA concentration in the serum above 43.42 mcg/ml surgical treatment is indicated, if it is lower--conservative treatment and follow-up. PMID:19069496

  2. Lymphoedema caused by idiopathic lymphatic thrombus.

    PubMed

    Hara, Hisako; Mihara, Makoto; Seki, Yukio; Koshima, Isao

    2013-12-01

    Primary lymphoedema includes some diseases whose genetic anomaly is detected and others whose pathology is unknown. In this article, we report a lymphatic thrombus found in a limb with lymphoedema during lymphatico-venous anastomosis (LVA). A 32-year-old man was aware of oedema in his left calcar pedis 3 years previously, which appeared without any trigger. Indocyanine green lymphography indicated lymphatic stasis in the left calf and thigh region, and we performed LVA for the patient. During the operation, we found yellow vessels, which were thought to be lymphatic vessels filled with a yellow solid substance, just beneath the superficial fascia at the left ankle. Pathological examination of the thrombi revealed hyaline material mixed with cell components. The cells were categorised as lymphatic endothelial cells, as they were positive for podoplanin. There was no evidence of malignancy. Causes of idiopathic lymphatic thrombus such as this may be one of the causes of so-called primary lymphoedema, and evaluation of such cases may be the first step towards elucidating the mechanisms involved in the development of primary lymphoedema. PMID:23643778

  3. Idiopathic Granulomatous Hypophysitis Mimicking Pituitary Abscess

    PubMed Central

    Kong, Xiangyi; Wang, Renzhi; Yang, Yi; Wu, Huanwen; Su, Changbao; Ma, Wenbin; Li, Yongning; Xing, Bing; Lian, Wei; Xu, Zhiqin; Yao, Yong; Ren, Zuyuan

    2015-01-01

    Abstract Idiopathic granulomatous hypophysitis (IGH) is a rare inflammatory disease of the pituitary that commonly presents with enlargement of the pituitary gland. Clinically and radiologically, IGH is a rare sellar entity easily to be misdiagnosed as a pituitary adenoma. Through such a case, we aim to present this rarity and emphasize the importance to correctly diagnose confusing pituitary lesions comprehensively by clinical presentations, radiological signs, and biopsy. We present an uncommon case of IGH in a 19-year-old man. The patient was admitted to the hospital with severe headache, vomiting, and vision's sharp decline. Magnetic resonance imaging showed a sellar lesion with obvious cystic change and ring enhancement. The disease course including diagnosis and treatment was presented and analyzed in detail. The pertinent literature is reviewed regarding this uncommon entity. The patient underwent surgical exploration and partial resection via the transsphenoidal approach. The pathologic findings suggested IGH giving no significant evidences of systemic granulomatous disease and venereal disease. Large dose methylprednisolone was then used. The pituitary function recovered, but there was no apparent improvement of his vision. IGH is a rarely occurred inflammatory disease of unknown etiology. It is difficult to diagnose preoperatively and is often misdiagnosed. Although rare, IGH should be kept in mind in terms of differential diagnosis of sellar region lesions. PMID:26181544

  4. [Juvenile idiopathic arthritis and oral health].

    PubMed

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

    2016-01-01

    Juvenile idiopathic arthritis (JIA) is the most common autoimmune inflammatory disease of connective tissue in children. It is characterized by progressive joint destruction which causes preserved changes in the musculoskeletal system. The literature describes fully clinical symptoms and radiological images in different subtypes of JIA. However, there is still a limited number of studies reporting on the medical condition of the oral cavity of ill children. JIA can affect hard and soft tissues of the oral cavity by: the general condition of the child's health, arthritis of the upper limbs, as the result of the pharmacotherapy, changes in secretion and composition of saliva, inflammation of the temporomandibular joint and facial deformity. The study summarizes the available literature on the condition of the teeth and periodontal and oral hygiene in the course of JIA. The presence of diverse factors that modify the oral cavity, such as facial growth, functioning of salivary glands, or the supervision and care provided by adults, prevents clear identification if JIA leads to severe dental caries and periodontal disease. Despite conflicting results in studies concerning the clinical oral status, individuals with JIA require special attention regarding disease prevention and maintenance of oral health. PMID:27180959

  5. The treatment of idiopathic pulmonary fibrosis.

    PubMed

    Woodcock, Hannah V; Maher, Toby M

    2014-01-01

    Idiopathic pulmonary fibrosis (IPF) is a progressive and invariably fatal disease with a median survival of less than three years from diagnosis. The last decade has seen an exponential increase in clinical trial activity in IPF and this in turn has led to important developments in the treatment of this terrible disease. Previous therapeutic approaches based around regimens including corticosteroids and azathioprine have, when tested in randomized clinical trials, been shown to be harmful in IPF. By contrast, compounds with anti-fibrotic actions have been shown to be beneficial. Subsequently, the novel anti-fibrotic agent pirfenidone has, in many parts of the world, become the first treatment ever to be licensed for use in IPF. This exciting development, coupled with ongoing clinical trials of a range of other novel compounds, is bringing hope to patients and their clinicians and raises the prospect that, in the future, it may become possible to successfully arrest the development of progressive scarring in IPF. PMID:24669297

  6. Molecular mechanisms in progressive idiopathic pulmonary fibrosis.

    PubMed

    Steele, Mark P; Schwartz, David A

    2013-01-01

    There is clear evidence that environmental exposures and genetic predisposition contribute to the pathogenesis of idiopathic pulmonary fibrosis (IPF). Cigarette smoking increases the risk of developing IPF several-fold, as do other exposures such as metal-fume and wood-dust exposure. Occupations that increase the risk of IPF are agricultural work, hairdressing, and stone polishing, supporting the role of environmental exposure in disease pathogenesis. Genetic predisposition to IPF is evident from its familial aggregation and the fact that pulmonary fibrosis develops in several rare genetic disorders. Mutations in surfactant proteins lead to pulmonary fibrosis and are associated with endoplasmic reticulum stress in alveolar type II epithelial cells. Mutations in telomerase have been found in several families with IPF, and shortened telomeres are found in sporadic cases of IPF. A common variant in mucin 5B predisposes to both familial and sporadic IPF and is present in the majority of cases, indicating sporadic IPF occurs in those with genetic predisposition. PMID:23020878

  7. The laboratory profile in idiopathic intracranial hypertension.

    PubMed

    Pollak, Lea; Zohar, Efrat; Glovinsky, Yoseph; Huna-Baron, Ruth

    2015-07-01

    While overweight and female gender play an undisputable role in the pathogenesis of idiopathic intracranial hypertension (IIH), the contribution of other factors is still unclear. We have evaluated the laboratory findings of patients with IIH in an attempt to find the influence of abnormalities on the disease course. Included were 82 females after menarche and males older than 18 years who were followed up for at least 1 year. A wide range of laboratory parameters were examined at the time of presentation. The most frequent abnormal laboratory findings were elevated C reactive protein (CRP) (51 %), thrombophilia (31 %), increased plasma cortisol levels (29 %) and elevated lactate dehydrogenase (LDH) (20 %). Patients with elevated CRP and patients with thrombophilia had an unfavorable visual outcome. Increased cortisol levels and abnormal calcium correlated with a higher rate of recurrence. The visual outcome of patients with elevated LDH was better than those with normal LDH. It seems that certain metabolic, inflammatory and coagulation abnormalities may influence the course of IIH. If confirmed in further studies, these findings could contribute to elucidation of the etiology and prognosis of IIH. PMID:25596710

  8. Osteoporosis in men with idiopathic hypogonadotropic hypogonadism

    SciTech Connect

    Finkelstein, J.S.; Klibanski, A.; Neer, R.M.; Greenspan, S.L.; Rosenthal, D.I.; Crowley, W.F. Jr.

    1987-03-01

    To assess the effect of testosterone deficiency on skeletal integrity in men, we determined bone density in 23 hypogonadal men with isolated gonadotropin-releasing hormone deficiency and compared those values with ones from controls. Cortical bone density, as assessed by single-photon absorptiometry of the nondominant radius, ranged from 0.57 to 0.86 g/cm2 (mean +/- SE, 0.71 +/- 0.02) in patients with fused epiphyses and from 0.57 to 0.67 g/cm2 (mean, 0.61 +/- 0.01) in patients with open epiphyses, both of which were significantly (p less than 0.001) lower than normal. Spinal trabecular bone density, as assessed by computed tomography, was similarly decreased (p less than 0.0001) and ranged from 42 to 177 mg K2HPO4/cm3 (mean, 112 +/- 7). Cortical bone density was at least 2 SD below normal in 16 of 23 men, and 8 men had spinal bone densities below the fracture threshold of 80 to 100 mg K2HPO4/cm3. Osteopenia was equally severe in men with immature and mature bone ages, suggesting that abnormal bone development plays an important role in the osteopenia of men with idiopathic hypogonadotropic hypogonadism.

  9. Idiopathic encapsulating peritonitis: report of two cases.

    PubMed

    Da Luz, Magda Maria Profeta; Barral, Sumara Marques; Barral, Carlyle Marques; Bechara, Cristiane De Souza; Lacerda-Filho, Antônio

    2011-12-01

    This report presents two cases of young males who developed the rare idiopathic form of sclerosing encapsulating peritonitis (SEP) presented as partial bowel obstruction, both diagnosed during surgical treatment, with satisfactory outcomes. Sclerosing encapsulating peritonitis is a rare and enigmatic condition, characterized by intraperitoneal fibrosclerosis, which causes intestinal obstruction. It is a chronic entity with a poorly elucidated pathophysiology, leading to the constitution of a thick white nacreous fibrosis membrane that wraps the bowel in a concertina-like fashion with some adhesions configuring an intra-abdominal cocoon. Sclerosing encapsulating peritonitis is reported in a wide variety of patients, including those who have undergone peritoneal dialysis, young adolescent girls, cirrhotic patients after peritoneal-venous shunting, and patients treated with β-blockers. Nevertheless, the etiology of SEP remains obscure. This entity presents many difficulties in preoperative diagnosis because of its peculiar characteristics. Recognition of the SEP results in proper management and prevents unnecessary bowel resection. Regardless of cause, the treatment of the obstruction is surgical, with dissection of the encasing membrane from the intestine and separation of adherent loops of small bowel until they are laid free and returned to their normal configuration. The prognosis after appropriate surgical therapy is good, but depends on coexisting diseases. PMID:21969199

  10. Trigeminal neuralgia and persistent idiopathic facial pain.

    PubMed

    Obermann, Mark; Holle, Dagny; Katsarava, Zaza

    2011-11-01

    Trigeminal neuralgia (TN) and persistent idiopathic facial pain (PIFP) are two of the most puzzling orofacial pain conditions and affected patients are often very difficult to treat. TN is characterized by paroxysms of brief but severe pain followed by asymptomatic periods without pain. In some patients a constant dull background pain may persist. This constant dull pain sometimes makes the distinction from PIFP difficult. PIFP is defined as continuous facial pain, typically localized in a circumscribed area of the face, which is not accompanied by any neurological or other lesion identified by clinical examination or clinical investigations. The pain usually does not stay within the usual anatomic boundaries of the trigeminal nerve distribution and is a diagnosis of exclusion. Epidemiologic evidence on TN, and even more so on PIFP, is quite scarce, but generally both conditions are considered to be rare diseases. The etiology and underlying pathophysiology of TN, and more so PIFP, remain unknown. Treatment is based on only few randomized controlled clinical trials and insufficiently evaluated surgical procedures. PMID:22014140

  11. Prucalopride: A Review in Chronic Idiopathic Constipation.

    PubMed

    Garnock-Jones, Karly P

    2016-01-01

    Prucalopride (Resolor®), a highly selective serotonin 5-HT4 receptor agonist, is indicated in the European Economic Area for the treatment of adults with chronic idiopathic constipation (CIC) in whom laxatives have failed to provide adequate relief. This article reviews the pharmacological properties of prucalopride and its clinical efficacy and tolerability in patients with CIC. In five well-designed, 12-week trials in patients with CIC, oral prucalopride 2 mg/day was significantly more effective than placebo at improving bowel function, including the number of bowel movements and a range of other constipation symptoms, as well as health-related quality of life and patient satisfaction; however, no significant differences in bowel function measures were observed between prucalopride and placebo in a 24-week trial. Oral PEG-3350 + electrolytes reconstituted powder was found to be noninferior but not superior to prucalopride according to primary endpoint data from a 4-week, controlled-environment trial. Prucalopride was generally well tolerated in clinical trials; the most common adverse events were headache, diarrhoea, nausea and abdominal pain. No cardiovascular safety issues have arisen with prucalopride treatment. Although further long-term and comparative data would be beneficial, prucalopride provides an additional treatment option for patients with CIC. PMID:26628294

  12. Macrophages - silent enemies in juvenile idiopathic arthritis.

    PubMed

    Świdrowska-Jaros, Joanna; Orczyk, Krzysztof; Smolewska, Elżbieta

    2016-01-01

    The inflammatory response by secretion of cytokines and other mediators is postulated as one of the most significant factors in the pathophysiology of juvenile idiopathic arthritis (JIA). The effect of macrophage action depends on the type of their activation. Classically activated macrophages (M1) are responsible for release of molecules crucial for joint inflammation. Alternatively activated macrophages (M2) may recognize self antigens by scavenger receptors and induce the immunological reaction leading to autoimmune diseases such as JIA. Molecules essential for JIA pathophysiology include: TNF-α, the production of which precedes synovial inflammation in rheumatoid arthritis; IL-1 as a key mediator of synovial damage; chemotactic factors for macrophages IL-8 and MCP-1; IL6, the level of which correlates with the radiological joint damage; MIF, promoting the secretion of TNF-α and IL-6; CCL20 and HIF, significant for the hypoxic synovial environment in JIA; GM-CSF, stimulating the production of macrophages; and IL-18, crucial for NK cell functions. Recognition of the role of macrophages creates the potential for a new therapeutic approach. PMID:27383571

  13. PROFILEing idiopathic pulmonary fibrosis: rethinking biomarker discovery.

    PubMed

    Maher, Toby M

    2013-06-01

    Despite major advances in the understanding of the pathogenesis of idiopathic pulmonary fibrosis (IPF), diagnosis and management of the condition continue to pose significant challenges. Clinical management of IPF remains unsatisfactory due to limited availability of effective drug therapies, a lack of accurate indicators of disease progression, and an absence of simple short-term measures of therapeutic response. The identification of more accurate predictors of prognosis and survival in IPF would facilitate counseling of patients and their families, aid communication among clinicians, and would guide optimal timing of referral for transplantation. Improvements in molecular techniques have led to the identification of new disease pathways and a more targeted approach to the development of novel anti-fibrotic agents. However, despite an increased interest in biomarkers of IPF disease progression there are a lack of measures that can be used in early phase clinical trials. Careful longitudinal phenotyping of individuals with IPF together with the application of novel omics-based technology should provide important insights into disease pathogenesis and should address some of the major issues holding back drug development in IPF. The PROFILE (Prospective Observation of Fibrosis in the Lung Clinical Endpoints) study is a currently enrolling, prospective cohort study designed to tackle these issues. PMID:23728868

  14. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia syndrome.

    PubMed

    Rossi, Giulio; Cavazza, Alberto; Spagnolo, Paolo; Sverzellati, Nicola; Longo, Lucia; Jukna, Agita; Montanari, Gloria; Carbonelli, Cristiano; Vincenzi, Giada; Bogina, Giuseppe; Franco, Renato; Tiseo, Marcello; Cottin, Vincent; Colby, Thomas V

    2016-06-01

    The term diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) may be used to describe a clinico-pathological syndrome, as well as an incidental finding on histological examination, although there are obvious differences between these two scenarios. According to the World Health Organization, the definition of DIPNECH is purely histological. However, DIPNECH encompasses symptomatic patients with airway disease, as well as asymptomatic patients with neuroendocrine cell hyperplasia associated with multiple tumourlets/carcinoid tumours. DIPNECH is also considered a pre-neoplastic lesion in the spectrum of pulmonary neuroendocrine tumours, because it is commonly found in patients with peripheral carcinoid tumours.In this review, we summarise clinical, physiological, radiological and histological features of DIPNECH and critically discuss recently proposed diagnostic criteria. In addition, we propose that the term "DIPNECH syndrome" be used to indicate a sufficiently distinct patient subgroup characterised by respiratory symptoms, airflow obstruction, mosaic attenuation with air trapping on chest imaging and constrictive obliterative bronchiolitis, often with nodular proliferation of neuroendocrine cells with/without tumourlets/carcinoid tumours on histology. Surgical lung biopsy is the diagnostic gold standard. However, in the appropriate clinical and radiological setting, transbronchial lung biopsy may also allow a confident diagnosis of DIPNECH syndrome. PMID:27076588

  15. Mineralogical microanalysis of idiopathic pulmonary fibrosis

    SciTech Connect

    Monso, E.; Tura, J.M.; Marsal, M.; Morell, F.; Pujadas, J.; Morera, J. )

    1990-05-01

    A mineralogical analysis of lung tissue was conducted on 25 samples from patients who had been diagnosed as having idiopathic pulmonary fibrosis (IPF). Scanning electron microscopy (SEM) at low magnification and energy-dispersive x-ray analysis (EDXA) was used. In all samples, the surface silicon/sulfur (Si/S) ratio was calculated. The Si/S ratio for 25 samples of normal lung and 6 samples of pneumoconiotic lung was also determined (upper limit of normal Si/S ratio = 0.3). The difference between the Si/S ratio in the group with IPF and group with normal lung tissue was significantly significant (p less than .007, Wilcoxon test). Six of 12 patients with a previous diagnosis of IPF and a Si/S ratio greater than 0.3 had an exposure history that could imply inhalation of silica/silicates, and the correct diagnosis for these patients is most probably pneumoconiosis. The silica/silicate deposits detected in patients with IPF, and who had a ratio and no past exposure to dusts, could be either a cause or an effect of the disease.

  16. Management of Children with Juvenile Idiopathic Arthritis.

    PubMed

    Viswanathan, Vijay; Murray, Kevin J

    2016-01-01

    Juvenile idiopathic arthritis (JIA) comprises a group of heterogeneous disorders of chronic arthritis in childhood and remains the commonest pediatric rheumatic disease associated with significant long-term morbidity. Advances in understanding of the pathogenesis, better definition of disease control/remission measures, and the arrival of biological agents have improved the outcomes remarkably. Methotrexate (Mtx) remains the first-line disease modifying (DMARD) therapy for most children with JIA due to its proven efficacy and safety. Sulphosalazine (SSz) (especially for enthesitis) and leflunomide may also have a secondary role. Tumor necrosis factor inhibitors (TNF-I), alone or in combination with Mtx have shown tremendous benefit in children with polyarticular JIA, enthesitis related arthritis (ERA) and psoriatic arthritis. Tocilizumab appears very efficacious in systemic arthritis and abatacept and tocilizumab also appear to benefit polyarticular JIA; the role of rituximab remains unclear, though clearly beneficial in adult RA. TNF-I with Mtx is also effective in uveitis associated with JIA. Biologicals have demonstrated an impressive safety record in children with JIA, although close monitoring for rare but potentially dangerous adverse events, such as tuberculosis and other infections; paradoxical development of additional autoimmune diseases; and possibly an increased risk of cancers is warranted. PMID:26639461

  17. Urinary neopterin in idiopathic retinal vasculitis

    PubMed Central

    Palmer, H; Giovannoni, G; Stanford, M; Wallace, G; Graham, E

    2001-01-01

    AIMS—To determine whether urinary neopterin:creatinine (UNC) ratios relate to disease activity in idiopathic retinal vasculitis (RV).
METHODS—18 patients with RV were prospectively recruited into a year long longitudinal study. Patients collected first morning urine samples on a weekly basis and on the same day completed a diary which documented their subjective view of RV activity and any concurrent infection. They were examined in clinic on a 6-8 weekly basis and an objective assessment was made of RV disease activity. 14 healthy controls collected urine samples in the same way.
RESULTS—UNC ratios were significantly higher in patients than in controls (p=0.004, Mann-Whitney U test). UNC ratios were significantly higher when, according to their diaries, the patients had a subjective flare up of RV (p=0.001, Mann-Whitney U test). Subjective increased RV activity occurred more often when the patients had a concurrent infection (p<0.0001, χ2 test). There was no significant difference in the UNC ratio between objective clinical relapse and non-relapse of RV. There was moderate agreement between the clinical assessment and patients' subjective impression of RV activity (κ=0.48).
CONCLUSIONS—Higher neopterin levels reflect cell mediated disease that occurs in RV, but UNC ratios are not recommended as a means of monitoring clinical disease activity in RV.

 PMID:11133708

  18. Idiopathic Granulomatous Mastitis Associated with Erythema Nodosum

    PubMed Central

    Kalaycı, Tuğçe Özlem; Koruyucu, Melike Bedel; Apaydın, Melda; Etit, Demet; Varer, Makbule

    2016-01-01

    Background: Idiopathic granulomatous mastitis (IGM) is an uncommon benign chronic inflammatory breast disease, and erythema nodosum (EN) is an extremely rare systemic manifestation of IGM. Here, we report a rare case of IGM accompanied by EN. Case Report: A 32-year-old patient was admitted to our clinic with a history of a tender mass in the right breast. On physical examination, the right breast contained a hard, tender mass in the lower half with indrawing of the nipple. She had florid EN affecting both legs. She was evaluated with mammography, ultrasound, power Doppler ultrasound, non-enhancing magnetic resonance imaging (MRI), dynamic contrast-enhanced MRI, fine needle aspiration biopsy (FNAB) and excisional biopsy. Time-intensity curves showed a type II pattern on dynamic contrast-enhanced MRI, which has an intermediate probability for malignancy. The FNAB reported a benign cytology suggestive of a granulomatous inflammation, which was also supported by the histopathological findings. A partial mastectomy was performed following medical treatment. There was no recurrence at 1-year follow-up. Conclusion: IGM should be considered in the differential diagnosis of EN. Although histopathological examination remains the only method for the definite diagnosis of IGM, MRI can be helpful in the diagnosis or differentiation of benign lesions from malignant ones. PMID:27403395

  19. Idiopathic Inflammatory Myopathies: Clinical Approach and Management

    PubMed Central

    Malik, Asma; Hayat, Ghazala; Kalia, Junaid S.; Guzman, Miguel A.

    2016-01-01

    Idiopathic inflammatory myopathies (IIM) are a group of chronic, autoimmune conditions affecting primarily the proximal muscles. The most common types are dermatomyositis (DM), polymyositis (PM), necrotizing autoimmune myopathy (NAM), and sporadic inclusion body myositis (sIBM). Patients typically present with sub-acute to chronic onset of proximal weakness manifested by difficulty with rising from a chair, climbing stairs, lifting objects, and combing hair. They are uniquely identified by their clinical presentation consisting of muscular and extramuscular manifestations. Laboratory investigations, including increased serum creatine kinase (CK) and myositis specific antibodies (MSA) may help in differentiating clinical phenotype and to confirm the diagnosis. However, muscle biopsy remains the gold standard for diagnosis. These disorders are potentially treatable with proper diagnosis and initiation of therapy. Goals of treatment are to eliminate inflammation, restore muscle performance, reduce morbidity, and improve quality of life. This review aims to provide a basic diagnostic approach to patients with suspected IIM, summarize current therapeutic strategies, and provide an insight into future prospective therapies. PMID:27242652

  20. [Idiopathic Progressive Subglottic Stenosis: Surgical Techniques].

    PubMed

    Hoetzenecker, K; Schweiger, T; Klepetko, W

    2016-09-01

    Idiopathic subglottic stenosis is a disease characterized by slow, progressive scarring and constriction of the subglottic airway. It almost always occurs in females between the 3rd and 5th decade of life. Symptoms are frequently misinterpreted as asthma and patients are referred for endoscopic evaluation only when asthma medications fail to alleviate their symptoms. Treatment options can be divided into endoscopic and open surgical techniques. Microlaryngoscopic scar reduction by laser followed by balloon dilation usually delivers good short-term results. However, the majority of patients will experience restenosis within a short period of time. Open surgical correction techniques are based on a complete removal of the affected airway segment. This must be combined with various extended resection techniques in patients with advanced stenosis. Depending on the extent and severity of the stenosis the following surgical techniques are required: standard cricotracheal resection (Grillo's technique), cricoplasty with dorsal and lateral mucosaplasty, or a combination of resection and enlargement techniques using rib cartilage grafts. In experienced centres, success rates of over 95 % are reported with good functional outcome of voice and deglutition. PMID:27607884

  1. Psychological functioning in idiopathic short stature.

    PubMed

    Noeker, Meinolf

    2011-01-01

    Living with idiopathic short stature (ISS) may entail significant risks to psychological functioning and quality of life. Apparent inconsistency among study findings can be resolved if methodological differences among study designs are taken into account (i.e., definition of particular endpoints, sample selection from clinic or population, source of report, specific or generic assessment instruments, statistical control of confounders). Some individuals fail and others succeed in mastering the challenges of ISS. The principles of multifinality and equifinality may explain the emergence of a broad variation of individuals with ISS as a result of an interaction of the individual medical and auxological features on the one side, and psychosocial risk and protective factors on the other. As a result, patients may show heterogeneous developmental outcomes ranging from clinical psychopathology to development of resilience. A taxonomy of four distinct pathways of adaptation to ISS is delineated as a basis for case formulation and treatment planning. Psychological intervention in ISS includes counseling, cognitive-behavioral therapy and assertiveness training to improve psychological functioning via enhancement of target coping behaviors for critical situations. PMID:21912169

  2. Factors associated with behavioral problems in children with idiopathic epilepsy.

    PubMed

    Dafoulis, Vaios; Kalyva, Efrosini

    2012-06-01

    The present study examined whether the perceived behavioral problems of children with idiopathic epilepsy differed from those of healthy controls according to parent proxy-reports and which factors are associated with these problems. The parents of 106 children with idiopathic epilepsy and 305 healthy controls aged 6-9 years old completed the Vanderbilt ADHD Diagnostic Parent Rating Scale and the Strengths and Difficulties Questionnaire. The 106 children with idiopathic epilepsy were also interviewed using the K-SADS-PL. The parents of children with idiopathic epilepsy reported more hyperactivity, emotional and conduct problems than the parents of healthy controls, as well as less prosocial behavior. Parents detected no differences in peer problems, inattention, oppositional/defiant disorder, and anxiety/depression. Age of onset of epilepsy (later), the number of administered antiepileptic drugs (polytherapy), and gender (male) predicted behavioral problems in children with idiopathic epilepsy. The frequency of seizures was associated with behavioral problems, while age was not. Finally, children with benign focal epilepsy were rated by their parents as having less behavioral problems than children with generalized epilepsy. PMID:22348790

  3. Prevalence of Celiac Disease in Turkish Children with Idiopathic Epilepsy

    PubMed Central

    Işikay, Sedat; Hizli, Şamil; Yilmaz, Kutluhan

    2014-01-01

    Objective: This study has examined the prevalence of celiac disease in Turkish children with idiopathic epilepsy. Methods: Children with idiopathic epilepsy were screened for celiac disease using the IgA anti-tissue transglutaminase antibody and compared with the healthy control group in order to find the association of celiac disease (CD) with idiopathic epilepsy. Upper gastrointestinal endoscopy and small intestinal biopsies were offered to all antibody-positive patients. Findings : A total of 214 children with the diagnosis of idiopathic epilepsy and 166 healthy children as control group were studied. Of the patients recruited, 55.1% had generalized epilepsy, and 44.9% had partial epilepsy. In 33 patients with partial epilepsy, electroclinical features were consistent with a diagnosis of childhood partial epilepsy with occipital paroxysms (CPEO). Two of 33 patients with CPEO had positive IgA anti-tissue transglutaminase antibodies in serology. Pathological examination of small intestinal biopsy specimens showed total villous atrophy in both of them. The prevalence of celiac disease among children with idiopathic epilepsy and CPEO was 0.9% and 6%, respectively. Conclusion: The results of the present study revealed that prevalence of CD is increased in children with epilepsy. On the other hand, as high as 6% prevalence of CD among patients with CPEO found in this study should be kept in mind and the clinicians should be aware of this association. PMID:25562021

  4. Idiopathic Nephrotic Syndrome and Atopy: Is There a Common Link?

    PubMed Central

    Abdel-Hafez, Maher; Shimada, Michiko; Lee, Pui Y.; Johnson, Richard J.; Garin, Eduardo H.

    2010-01-01

    Numerous reports during the last 60 years have reported a strong association between idiopathic nephrotic syndrome and atopic disorders. Idiopathic nephrotic syndrome can be precipitated by allergic reactions and has been associated with both aeroallergens (pollens, mold, and dust) and food allergies. Patients with idiopathic nephrotic syndrome also may show increased serum immunoglobulin E (IgE) levels. A review of the literature suggests that although some idiopathic nephrotic syndrome cases may be associated with allergies, evidence that it is a type of allergic disorder or can be induced by a specific allergen is weak. Rather, it is likely that the proteinuria and increased IgE levels in patients with idiopathic nephrotic syndrome are caused by increased levels of interleukin 13 observed in these patients. Recent studies suggest that interleukin 13, a known stimulator of IgE response, may mediate proteinuria in patients with minimal change disease because of its ability to directly induce CD80 expression on the podocyte. PMID:19556042

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

    PubMed

    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. PMID:24792314

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

    PubMed Central

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

    2013-01-01

    In recent years, the improvement of technology and the increase in knowledge have shifted several strongly held paradigms. This is particularly true in gastroenterology, and specifically in the field of the so-called “functional” or “idiopathic” disease, where conditions thought for decades to be based mainly on alterations of visceral perception or aberrant psychosomatic mechanisms have, in fact, be reconducted to an organic basis (or, at the very least, have shown one or more demonstrable abnormalities). This is particularly true, for instance, for irritable bowel syndrome, the prototype entity of “functional” gastrointestinal disorders, where low-grade inflammation of both mucosa and myenteric plexus has been repeatedly demonstrated. Thus, researchers have also investigated other functional/idiopathic gastrointestinal disorders, and found that some organic ground is present, such as abnormal neurotransmission and myenteric plexitis in esophageal achalasia and mucosal immune activation and mild eosinophilia in functional dyspepsia. Here we show evidence, based on our own and other authors’ work, that chronic constipation has several abnormalities reconductable to alterations in the enteric nervous system, abnormalities mainly characterized by a constant decrease of enteric glial cells and interstitial cells of Cajal (and, sometimes, of enteric neurons). Thus, we feel that (at least some forms of) chronic constipation should no more be considered as a functional/idiopathic gastrointestinal disorder, but instead as a true enteric neuropathic abnormality. PMID:23864772

  7. The inter-connection between fibrosis and microvascular remodeling in idiopathic pulmonary fibrosis: Reality or just a phenomenon

    PubMed Central

    Mlika, Mona; Bacha, Saoussen; Braham, Emna; El Mezni, Faouzi

    2015-01-01

    Background Idiopathic pulmonary fibrosis is the most frequent interstitial disease with the worst prognosis. It is characterized by an uncontrolled fibrosis which is difficult to manage. The pathogenesis of this disease remains unclear with many theories resulting in multiple target therapies. The relation between fibrosis and vascular remodeling has been debated in the literature with different results that may seem contradictory. Aim We target to evaluate the connection between fibrosis and vascular remodeling in usual interstitial pneumonia. Material and methods 26 cases of idiopathic pulmonary fibrosis were reviewed by 2 pathologists and the diagnosis of UIP was retained according to the American Thoracic Society's criteria. Fibrotic changes and vascular remodeling were evaluated blindly. The fibrotic changes were classified as severe, intermediate and mild. Vascular occlusion was graded in 4 grades extending from medial hypertrophy (grade 1) to plexiform lesions of the vascular wall (grade 4). Results We noticed that severe degrees of fibrosis were correlated with severe grades of vascular obstruction. In fact, our 26 cases were classified as severe fibrosis in 11 cases with grade IV vascular lesions in 6 cases, intermediate fibrosis in 12 cases with grade II vascular lesions in 8 cases and mild fibrosis in 3 cases with grade I vascular lesions in all cases. Conclusion Many theories have been reported concerning the UIP's pathogenesis. Recently, many authors reported that the primum movens of these lesions was an epithelial/endothelial injury which induces uncontrolled fibrosis and microvascular remodeling using different pathways. This puts emphasis on the necessity of multi-target therapies in order to improve the management of this fatal disease. PMID:27222781

  8. Low kV settings CT angiography (CTA) with low dose contrast medium volume protocol in the assessment of thoracic and abdominal aorta disease: a feasibility study

    PubMed Central

    Talei Franzesi, C; Fior, D; Bonaffini, P A; Minutolo, O; Sironi, S

    2015-01-01

    Objective: To assess the diagnostic quality of low dose (100 kV) CT angiography (CTA), by using ultra-low contrast medium volume (30 ml), for thoracic and abdominal aorta evaluation. Methods: 67 patients with thoracic or abdominal vascular disease underwent multidetector CT study using a 256 slice scanner, with low dose radiation protocol (automated tube current modulation, 100 kV) and low contrast medium volume (30 ml; 4 ml s−1). Density measurements were performed on ascending, arch, descending thoracic aorta, anonymous branch, abdominal aorta, and renal and common iliac arteries. Radiation dose exposure [dose–length product (DLP)] was calculated. A control group of 35 patients with thoracic or abdominal vascular disease were evaluated with standard CTA protocol (automated tube current modulation, 120 kV; contrast medium, 80 ml). Results: In all patients, we correctly visualized and evaluated main branches of the thoracic and abdominal aorta. No difference in density measurements was achieved between low tube voltage protocol (mean attenuation value of thoracic aorta, 304 HU; abdominal, 343 HU; renal arteries, 331 HU) and control group (mean attenuation value of thoracic aorta, 320 HU; abdominal, 339; renal arteries, 303 HU). Radiation dose exposure in low tube voltage protocol was significantly different between thoracic and abdominal low tube voltage studies (490 and 324 DLP, respectively) and the control group (thoracic DLP, 1032; abdomen, DLP 1078). Conclusion: Low-tube-voltage protocol may provide a diagnostic performance comparable with that of the standard protocol, decreasing radiation dose exposure and contrast material volume amount. Advances in knowledge: Low-tube-voltage-setting protocol combined with ultra-low contrast agent volume (30 ml), by using new multidetector-row CT scanners, represents a feasible diagnostic tool to significantly reduce the radiation dose delivered to patients and to preserve renal function

  9. Clinical and laboratory correlates of lung disease and cancer in adults with idiopathic hypogammaglobulinaemia.

    PubMed

    Brent, J; Guzman, D; Bangs, C; Grimbacher, B; Fayolle, C; Huissoon, A; Bethune, C; Thomas, M; Patel, S; Jolles, S; Alachkar, H; Kumaratne, D; Baxendale, H; Edgar, J D; Helbert, M; Hambleton, S; Arkwright, P D

    2016-04-01

    Idiopathic hypogammaglobulinaemia, including common variable immune deficiency (CVID), has a heterogeneous clinical phenotype. This study used data from the national UK Primary Immune Deficiency (UKPID) registry to examine factors associated with adverse outcomes, particularly lung damage and malignancy. A total of 801 adults labelled with idiopathic hypogammaglobulinaemia and CVID aged 18-96 years from 10 UK cities were recruited using the UKPID registry database. Clinical and laboratory data (leucocyte numbers and serum immunoglobulin concentrations) were collated and analysed using uni- and multivariate statistics. Low serum immunoglobulin (Ig)G pre-immunoglobulin replacement therapy was the key factor associated with lower respiratory tract infections (LRTI) and history of LRTI was the main factor associated with bronchiectasis. History of overt LRTI was also associated with a significantly shorter delay in diagnosis and commencing immunoglobulin replacement therapy [5 (range 1-13 years) versus 9 (range 2-24) years]. Patients with bronchiectasis started immunoglobulin replacement therapy significantly later than those without this complication [7 (range 2-22) years versus 5 (range 1-13) years]. Patients with a history of LRTI had higher serum IgG concentrations on therapy and were twice as likely to be on prophylactic antibiotics. Ensuring prompt commencement of immunoglobulin therapy in patients with idiopathic hypogammaglobulinaemia is likely to help prevent LRTI and subsequent bronchiectasis. Cancer was the only factor associated with mortality. Overt cancer, both haematological and non-haematological, was associated with significantly lower absolute CD8(+) T cell but not natural killer (NK) cell numbers, raising the question as to what extent immune senescence, particularly of CD8(+) T cells, might contribute to the increased risk of cancers as individuals age. PMID:26646609

  10. Synthetic ACTH in High Risk Patients with Idiopathic Membranous Nephropathy: A Prospective, Open Label Cohort Study

    PubMed Central

    van de Logt, Anne-Els; Beerenhout, Charles H.; Brink, Hans S.; van de Kerkhof, Jos J.; Wetzels, Jack F.; Hofstra, Julia M.

    2015-01-01

    New therapeutic agents are warranted in idiopathic membranous nephropathy. Synthetic ACTH may be advantageous with reported remission rates up to 85% and few side effects. We conducted a prospective open label cohort study from 2008 till 2010 (NCT00694863). We prospectively selected patients with idiopathic membranous nephropathy and high risk for progression (defined as βeta-2-microglobulin (β2m) excretion of >500 ng/min). For comparison, we selected matched historical controls treated with cyclophosphamide. The prospectively selected patients received intramuscular injections of synthetic ACTH during 9 months (maximal dose 1 mg twice a week). The primary endpoints concerned the feasibility and incidence of remissions as a primary event. Secondary endpoints included side effects of treatment and the incidence of remissions and relapses at long-term follow-up. Twenty patients (15 men) were included (age 54±14 years, serum creatinine 104 μmol/l [IQR 90–113], urine protein:creatinine ratio 8.7 g/10 mmol creatinine [IQR 4.3–11.1]). Seventeen patients (85%) completed treatment. 97% of injections were administered correctly. Cumulative remission rate was 55% (complete remission in 4 patients, partial remission 7 patients). In a group of historical controls treated with cyclophosphamide and steroids, 19 of 20 patients (95%) developed a remission (complete remission in 13 patients, partial remission in 6 patients) (p<0.01). The main limitation of our study is its small size and the use of a historical control group. We show that treatment with intramuscular injections of synthetic ACTH is feasible. Our data suggest that synthetic ACTH is less effective than cyclophosphamide in inducing a remission in high risk patients with idiopathic membranous nephropathy. The use of synthetic ACTH was also associated with many adverse events. Therefore, we advise against synthetic ACTH as standard treatment in membranous nephropathy. Trial Registration ClinicalTrials.gov NCT

  11. Radiological and clinical outcome of screw placement in adolescent idiopathic scoliosis: evaluation with low-dose computed tomography

    PubMed Central

    Ohlin, Acke; Strömbeck, Anita; Maly, Pavel; Sundgren, Pia C.

    2009-01-01

    Posterior corrective surgery using “all pedicle screw construct” carries risk of neurovascular complications. The study aims were to assess the screw placement in patients with adolescent idiopathic scoliosis using CT with low-radiation dose, and to evaluate the clinical outcome in patients with misplaced pedicle screws. CTs of 49 consecutive patients (873 screws, 79% thoracic) were retrospectively evaluated by two independent radiologists. A new grading system was developed to distinguish between lateral, medial and anterior cortical perforations, endplate perforation and foraminal perforation. The grading system is based on whether the cortical violation is partial or total rather than on mm-basis. The overall rate of screw misplacement was 17% (n = 149): 8% were laterally placed and 6.1% were medially placed. The rates of anterior cortical, endplate and foraminal perforation were 1.5, 0.9, and 0.5%, respectively. Lateral cortical perforation was more frequent in the thoracic spine (P = 0.005), whereas other types of misplacement including medial cortical perforation were more frequent on the left and the concave side of scoliotic curves (P = 0.002 and 0.003). No neurovascular complications were reported. The association between the occurrence of screw misplacement and the Cobb angle was statistically significant (P = 0.037). Misplacements exceeding half screw diameter should be classified as unacceptable. Low-dose CT implies exposing these young individuals to a significantly lower radiation dose than do other protocols used in daily clinical practice. We recommend using low-dose CT and the grading system proposed here in the postoperative assessment of screw placement. PMID:19888607

  12. Survey of Thoracic CT Protocols and Technical Parameters in Korean Hospitals: Changes before and after Establishment of Thoracic CT Guideline by Korean Society of Thoracic Radiology in 2008

    PubMed Central

    2016-01-01

    We retrospectively reviewed the thoracic CT scan protocols and technical parameters obtained from hospitals in Korea, one group during May 2007 (n = 100) and the other group during January 2012 (n = 173), before and after the establishment of the thoracic CT Guideline in 2008. Each group was also divided into two subgroups according to the health care delivery level, i.e. the “A” subgroup from primary and the “B” subgroup from secondary and tertiary care hospitals. When comparing the data from 2007 and 2012, the tube current decreased from 179.1 mAs to 137.2 mAs. The scan interval decreased from 6.4 mm to 4.8 mm. Also, the insufficient scan range decreased from 19.0% to 8.7%, and the suboptimal quality scans decreased from 33.0% to 5.2%. Between groups A and B, group B had lower tube voltages, smaller scan thicknesses, and smaller scan intervals. However, group B had more phase numbers. In terms of the suboptimal quality scans, a decrease was seen in both groups. In conclusion, during the five-year time period between 2007 and 2012, a reduction in the tube current values was seen. And the overall image quality improved over the same time period. We assume that these changes are attributed to the implementation of the thoracic CT guideline in 2008. PMID:26908985

  13. Hirschsprung's disease and idiopathic megacolon in adults and adolescents.

    PubMed Central

    Barnes, P R; Lennard-Jones, J E; Hawley, P R; Todd, I P

    1986-01-01

    The distinction between Hirschsprung's disease and idiopathic megacolon in childhood dates from the classic clinical, radiological, and histological studies of Bodian, Stephens, and Ward. This article describes clinical experience over 15 years of 94 patients in whom megacolon of these two types was recognised for the first time after the age of 10, to illustrate the problems of diagnosis and treatment in later years. Just as it is now recognised that patients with the clinical characteristics of Hirschsprung's disease may have one of several abnormalities of the myenteric plexus, including not only absence of ganglion cells, but also patchy or zonal loss, abnormal neurones or neuronal dysplasia, so idiopathic megacolon may also be a heterogeneous group of cases. This paper suggests on clinical grounds that those patients with idiopathic megacolon whose symptoms start in childhood differ from those whose symptoms develop in later years. Images Fig. 1 Fig. 2 Fig. 6 PMID:3699562

  14. Effects of erythromycin in chronic idiopathic intestinal pseudo-obstruction.

    PubMed

    Minami, T; Nishibayashi, H; Shinomura, Y; Matsuzawa, Y

    1996-12-01

    The prokinetic effects of erythromycin, a macrolide antibiotic, on the gastrointestinal tract as a motilin receptor agonist and its potential value for the treatment of gastrointestinal motility disorders have recently attracted interest. The effects of erythromycin on the clinical symptoms and gastrointestinal motility of patients with chronic idiopathic pseudo-obstruction have not been investigated extensively. We presented a case of chronic idiopathic intestinal pseudo-obstruction, in a 67-year-old man in whom oral erythromycin (900 mg/day) dramatically improved postprandial abdominal distention, nausea, and vomiting. Other agents with prokinetic effects on intestinal motility, i.e., cisapride, domperidone, metoclopramide, and trimebutine maleate did not have a favorable effect. Gastric emptying, measured by the sulfamethizole method; and intestinal transit, evaluated using radio-opaque markers, were markedly improved by treatment with erythromycin. Our experience suggests that the prokinetic effects of erythromycin may be of therapeutic value in chronic idiopathic intestinal pseudo-obstruction. PMID:9027652

  15. The role of gastroesophageal reflux in idiopathic pulmonary fibrosis.

    PubMed

    Raghu, Ganesh

    2003-08-18

    Fibroblast foci are indicative of idiopathic pulmonary fibrosis and appear to be a cellular attempt to repair the damaged alveolus. Although this progressive, often fatal, clinical syndrome is thought to be dependent on alveolar injury of unknown origin, significant clinical and preclinical evidence points to gastric acid as a causative harmful agent. Graded instillation of various forms of acid in several animal models resulted in aspiration-induced lung injury, including pulmonary fibrosis in pigs. Moreover, compelling clinical data suggest that a high percentage of patients with idiopathic pulmonary fibrosis also experience abnormal esophageal acid exposure, without necessarily experiencing the typical symptoms of gastroesophageal reflux disease (GERD). Aggressive, long-term therapeutic trials of patients with GERD and evaluation of the therapeutic effects on pulmonary disease will allow determination of the real influences of abnormal esophageal acid exposure in the development of idiopathic pulmonary fibrosis. PMID:12928077

  16. Smoking-related idiopathic interstitial pneumonia: A review.

    PubMed

    Margaritopoulos, George A; Harari, Sergio; Caminati, Antonella; Antoniou, Katerina M

    2016-01-01

    For many years, cigarette smoking has been considered as the leading cause of chronic obstructive pulmonary disease and lung cancer. Recently, however, it has also been associated with the development of diffuse interstitial lung diseases. In the latest classification of the major idiopathic interstitial pneumonias (IIP), the term smoking-related IIP has been introduced, including two entities, namely desquamative interstitial pneumonia (DIP) and respiratory bronchiolitis-interstitial lung disease (RB-ILD). Other entities in which smoking has a definite or suggested role include pulmonary Langerhan's cell histiocytosis, smoking-related interstitial fibrosis, combined pulmonary fibrosis and emphysema syndrome and idiopathic pulmonary fibrosis. In this review, we will focus on the mechanisms of smoking-related lung damage and on the clinical aspects of these disorders with the exception of idiopathic pulmonary fibrosis, which will be reviewed elsewhere in this review series. PMID:26138798

  17. Gamma knife radiosurgery for medically refractory idiopathic trigeminal neuralgia.

    PubMed

    Kang, J H; Yoon, Y S; Kang, D W; Chung, S S; Chang, J W

    2008-01-01

    Gamma knife radiosurgery (GKS) has been generally considered as a viable therapeutic option for the management of medically refractory idiopathic trigeminal neuralgia (TN). We reviewed our experience with GKS in patients with TN. Between Feb 1996 and May 2006, 77 patients with medical refractory idiopathic TN were treated using GKS. Thirty-six patients who had undergone other previous procedures, previous GKS, or had brain stem lesion, atypical symptoms, were excluded from this study. Pain improvement was achieved in 38 of the patients with TN (pain response rate 92.7%). Twenty-three patients were pain free and 15 had reduced pain. There were no serious complications. We think that GKS is an effective treatment option for patients with medical refractory idiopathic TN. PMID:18642631

  18. Hereditary Influence in Thoracic Aortic Aneurysm and Dissection.

    PubMed

    Isselbacher, Eric M; Lino Cardenas, Christian Lacks; Lindsay, Mark E

    2016-06-14

    Thoracic aortic aneurysm is a potentially life-threatening condition in that it places patients at risk for aortic dissection or rupture. However, our modern understanding of the pathogenesis of thoracic aortic aneurysm is quite limited. A genetic predisposition to thoracic aortic aneurysm has been established, and gene discovery in affected families has identified several major categories of gene alterations. The first involves mutations in genes encoding various components of the transforming growth factor beta (TGF-β) signaling cascade (FBN1, TGFBR1, TGFBR2, TGFB2, TGFB3, SMAD2, SMAD3 and SKI), and these conditions are known collectively as the TGF-β vasculopathies. The second set of genes encode components of the smooth muscle contractile apparatus (ACTA2, MYH11, MYLK, and PRKG1), a group called the smooth muscle contraction vasculopathies. Mechanistic hypotheses based on these discoveries have shaped rational therapies, some of which are under clinical evaluation. This review discusses published data on genes involved in thoracic aortic aneurysm and attempts to explain divergent hypotheses of aneurysm origin. PMID:27297344

  19. Normal Range of Thoracic Kyphosis in Male School Children

    PubMed Central

    Shamsi, MohammadBagher; Veisi, Korosh; Karimi, Loghman; Sarrafzadeh, Javad; Najafi, Farid

    2014-01-01

    Background. Although there are frequent studies about normal range of thoracic kyphosis, there is still a controversy about the exact values of this curve. In nine reported studies on 10 to 20 years of age boys, the value ranged from 25.1° to 53.3°. Objective for the Study. The aim of the present research was investigation of normal ranges of thoracic kyphosis in school children in Kermanshah, western Iran. Methods. 582 male students aged 13 to 18 years old using cluster random sampling were recruited from schools in Kermanshah city, 97 students for each age. Thoracic curves were measured using the flexicurve method. Results. Mean thoracic kyphosis for whole population was 35.49° SD 7.83 and plus or minus two standard deviations ranged from 19.83° to 51.15°. It increased gradually from 13 to 16 and then there was a little decrease to 18 years. Mean values for each age (13–16) were 13 (34.41 SD 7.47°), 14 (34.86 SD 8.29°), 15 (35.79 SD 7.93°), 16 (36.49 SD 7.85°), 17 (35.84 SD 8.33°), and 18 (35.55 SD 7.07°). Conclusions. Our results are in agreement with previous reports and can be used as normal values for local and regional purposes. PMID:24967122

  20. Xanthorrhizol induces endothelium-independent relaxation of rat thoracic aorta.

    PubMed

    Campos, M G; Oropeza, M V; Villanueva, T; Aguilar, M I; Delgado, G; Ponce, H A

    2000-06-01

    Xanthorrhizol, a bisabolene isolated from the medicinal plant Iostephane heterophylla, was assayed on rat thoracic aorta rings to elucidate its effect and likely mechanism of action, by measuring changes of isometric tension. Xanthorrhizol (1, 3, 10, 30 and 100 microg/mL) significantly inhibited precontractions induced by KCI-; (60mM), noradrenaline (10(-6) M) or CaCl2 (1.0 mM). Increasing concentrations of external calcium antagonized the inhibitory effect on KCl-induced contractions. The vasorelaxing effect of xanthorrhizol was not affected by indomethacin (10 microM) or L-NAME (100 microM) in intact rat thoracic aorta rings precontracted by noradrenaline, which suggested that the effect was not mediated through either endothelium-derived prostacyclin (PGI2) or nitric oxide release from endothelial cells. Endothelium removal did not affect the relaxation induced by xanthorrhizol on rat thoracic aorta rings, discarding the participation of any substance released by the endothelium. Xanthorrhizol inhibitory effect was greater on KCI- and CaCl2-induced contractions than on those induced by noradrenaline. Xanthorrhizol inhibitory effect in rat thoracic aorta is likely explained for interference with calcium availability by inhibiting calcium influx through both voltage- and receptor-operated channels. PMID:10983876

  1. [Pulmological aspects of diagnosis of thoracic pain (author's transl)].

    PubMed

    Meier-Sydow, J

    1980-05-16

    A number of medical disciplines are involved in the diagnosis and therapy of thoracic pain. The origin may be somatic or visceral. Individual diseases are discussed in particular such as myalgia epidemica, intercostal neuralgia, herpes zoster, pleuritis and pneumonia, pulmonary embolism, pneumothorax, mediastinal emphysema, mediastinitis, pulmonary hypertension and the hyperventilation syndrome. Differential diagnosis is also referred to. PMID:6771586

  2. Thoracoscopic Pericardial Window Creation and Thoracic Duct Ligation in Neonates

    PubMed Central

    Ouzounian, Steven P.; Napoleon, Lori; Permut, Lester C.; Golombek, Sergio G.

    2003-01-01

    Objective: We describe 2 newborn infants with persistent pericardial effusion treated with thoracoscopic pericardial window and thoracic duct ligation. Methods: Patient 1 was a premature female newborn who presented with severe cardiac anomalies, including dextrocardia. She was treated with pulmonary artery banding and pacemaker placement for complete cardiac block. Postoperatively, she developed pericarditis with persistent symptomatic pericardial effusion. She did not improve despite pericardial drain placement. She was treated with a thoracoscopic pericardial window. Patient 2 was a newborn male who presented with cardiac tamponade secondary to congenital chylopericardium. He did not respond to pericardial drain placement or medical management with fasting, total parenteral nutrition, and octreotide. He was treated with thoracoscopic pericardial window and thoracic duct ligation. Results: Patient 1 improved rapidly. The pericardial effusion disappeared. The chest tube was removed 5 days following surgery. She died 6 weeks later of a cardiac arrhythmia secondary to pacemaker failure. The pericardial effusion had resolved. Patient 2 responded to the pericardial window and thoracic duct ligation. He was discharged 10 days following the procedure. Conclusions: Thoracoscopy provides an excellent approach to the pericardium. Pericardial windows and biopsy can be safely performed with this approach. The thoracic duct can be easily identified and ligated even in small babies. Recovery can be fast with minimal postoperative discomfort. Cosmetic results are excellent and length of hospitalization is minimized. PMID:14626403

  3. Video-assisted thoracoscopic surgery for acute thoracic trauma

    PubMed Central

    Goodman, Michael; Lewis, Jaime; Guitron, Julian; Reed, Michael; Pritts, Timothy; Starnes, Sandra

    2013-01-01

    Background: Operative intervention for thoracic trauma typically requires thoracotomy. We hypothesized that thoracoscopy may be safely and effectively utilized for the acute management of thoracic injuries. Materials and Methods: The Trauma Registry of a Level I trauma center was queried from 1999 through 2010 for all video-assisted thoracic procedures within 24 h of admission. Data collected included initial vital signs, operative indication, intraoperative course, and postoperative outcome. Results: Twenty-three patients met inclusion criteria: 3 (13%) following blunt injury and 20 (87%) after penetrating trauma. Indications for urgent thoracoscopy included diaphragmatic/esophageal injury, retained hemothorax, ongoing hemorrhage, and open/persistent pneumothorax. No conversions to thoracotomy were required and no patient required re-operation. Mean postoperative chest tube duration was 2.9 days and mean length of stay was 5.6 days. Conclusion: Video-assisted thoracoscopic surgery is safe and effective for managing thoracic trauma in hemodynamically stable patients within the first 24 h post-injury. PMID:23723618

  4. Research and education in thoracic surgery: the European trainees’ perspective

    PubMed Central

    Ilonen, Ilkka K.

    2015-01-01

    Thoracic surgery training within Europe is diverse and a consensus may help to harmonise the training. Currently, training for thoracic surgery compromises thoracic, cardiothoracic and aspects of general surgical training. The recognition of specialist degrees should be universal and equal. Between different nations significant differences in training exist, especially in general surgery rotations and in the role of oesophageal surgery. The European board examination for thoracic surgery is one of the key ways to achieve harmonisation within the European Union (EU) and internationally. Further support and encouragement may be beneficial to promote diverse and engaging fellowships and clinical exchange programmes between nations. International fellowships may even benefit young residents, in both clinical and academic settings. Many studies currently would benefit from multi-centre and multi-national design, enhancing the results and giving better understanding of clinical scenarios. Educational content provided by independent organisations should be more recognised as an integral part in both resident training and continuing development throughout surgeons’ careers. During annual society meetings, trainees should have some sessions that are aimed at enhancing their training and establishing networks of international peers. PMID:25984356

  5. Video-assisted thoracic surgery for mediastinal extramedullary haematopoiesis.

    PubMed Central

    Ng, C. S. H.; Wan, S.; Lee, T. W.; Sihoe, A. D. L.; Wan, I. Y. P.; Arifi, A. A.; Yim, A. P. C.

    2002-01-01

    Extramedullary haematopoiesis is a rare cause of an intrathoracic mass. We report a case of posterior mediastinal extramedullary haematopoietic mass in a 50-year-old man who presented with non-specific symptoms and a paravertebral mass on chest X-ray. Diagnosis was achieved by using video-assisted thoracic surgery. Images Figure 1 Figure 2 PMID:12092864

  6. [Unusual fatal thoracic injury due to glass splinters].

    PubMed

    Korman, V; Soral, A

    1976-05-01

    The authors report on a case of unusual lethal penetrating puncture-incised thoracic wound in a male aged 33 following an incidental fall into the glass panel of a glass-panelled door. The glass splinters of the bre. The case as well as the pertinent literatury data and to the diagnostic problems pertaining to the determination of the injurious mechanism. PMID:1013734

  7. Thoracic vertebral osteomyelitis: an unusual complication of Crohn's disease.

    PubMed

    Ajayi, Olushola; Mayooran, Nithiananthan; Iqbal, Nasir

    2014-01-01

    Vertebral osteomyelitis complicating Crohn's disease is a rare occurrence and mostly occurred in patients with Crohn's disease complicated by an abscess or fistulising disease. We report a case of thoracic vertebral osteomyelitis, occurring in a refractory Crohn's disease without contiguous abscess or fistula with the bowel. PMID:24916975

  8. New applications of magnetic resonance imaging for thoracic oncology.

    PubMed

    Ohno, Yoshiharu

    2014-02-01

    Since the clinical introduction of magnetic resonance imaging (MRI), the chest has been one of its most challenging applications, and since the 1980s many physicists and radiologists have been trying to evaluate images for various lung diseases as well as mediastinal and pleural diseases. However, thoracic MRI could not yield image quality sufficient for a convincing diagnosis within an acceptable examination time, so MRI did not find acceptance as a substitute for computed tomography (CT) and other modalities. Until the 2000, thoracic MRI was generally used only for select, minor clinical indications. Within the past decade, however, technical advances in sequencing, scanners and coils, adaptation of parallel imaging techniques, utilization of contrast media, and development of postprocessing tools have been developed. In addition, pulmonary functional MRI has been extensively researched, and MR is being assessed as a new research and diagnostic tool for pulmonary diseases. State-of-the art thoracic MRI now has the potential as a substitute for traditional imaging techniques and/or to play a complimentary role in patient management. In this review, we focus on these advances in MRI for thoracic oncologic imaging, especially for pulmonary nodule assessment, lung cancer staging, mediastinal tumor diagnosis and malignant mesothelioma evaluation, prediction of postoperative lung function, and prediction or evaluation of therapeutic effectiveness. We also discuss the potential and limitations of these advances for routine clinical practice in comparison with other modalities such as CT, positron emission tomography (PET), PET/CT, or nuclear medicine studies. PMID:24481757

  9. Optimal management of idiopathic macular holes

    PubMed Central

    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 (<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 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. PMID:26834454

  10. Chronic idiopathic urticaria: prevalence and clinical course.

    PubMed

    Kulthanan, Kanokvalai; Jiamton, Sukhum; Thumpimukvatana, Narumol; Pinkaew, Sumruay

    2007-05-01

    The purpose of our study was to assess the prevalence and clinical course of patients with chronic idiopathic urticaria (CIU), as well as possible causes or associated findings, laboratory findings and the duration of the disease in patients with chronic urticaria (CU). We retrospectively reviewed the 450 case record forms of patients with CU and/or angioedema who attended the Department of Dermatology, Siriraj Hospital, during the period 2000-2004. Of 450 patients with CU, 337 patients (75%) were diagnosed as CIU. Forty-three patients (9.5%) had physical urticaria, while 17 patients (3.8%) had infectious causes. Other possible causes were food, thyroid diseases, atopy, drugs, dyspepsia and collagen vascular diseases. In eighty-nine percent of patients, no abnormalities were detected at the time of physical examination. The most common abnormal laboratory finding was minimal elevation of the erythrocyte sedimentary rate (42%). In 61 patients, autologous serum skin tests had been done. Fifteen patients (24.5%) had positive results i.e. autoimmune urticaria. Anti-thyroglobulin and anti-microsomal antibodies were positive in 16 % and 12% of CIU patients respectively. After 1 year from the onset of the symptoms, 34.5% of CIU patients were free of symptoms and after 1.2 years from the onset of the symptoms, 56.5% of autoimmune urticaria patients were free of symptoms. The median disease duration of CIU and autoimmune urticaria were 390 days and 450 days respectively. Our study provided an overview of CU and CIU in a large series of Thai patients, based on etiological aspects and clinical courses. PMID:17408437

  11. The Etiology of Juvenile Idiopathic Arthritis.

    PubMed

    Rigante, Donato; Bosco, Annalisa; Esposito, Susanna

    2015-10-01

    Over the years, the commonly used term to describe juvenile idiopathic arthritis (JIA) has changed. By definition, JIA includes all types of arthritis with no apparent cause, lasting more than 6 weeks, in patients aged less than 16 years at onset. JIA pathogenesis is still poorly understood: the interaction between environmental factors and multiple genes has been proposed as the most relevant working mechanism to the development of JIA. The concept that various microbes that colonize or infect not only the mucosal surfaces, like the oral cavity, but also the airways and gut might trigger autoimmune processes, resulting in chronic arthritides, and JIA was first drafted at the outset of last century. JIA development might be initiated and sustained by the exposure to environmental factors, including infectious agents which affect people at a young age, depending on the underlying genetic predisposition to synovial inflammation. Many data from patients with JIA suggest a scenario in which different external antigens incite multiple antigen-specific pathways, cytotoxic T cell responses, activation of classical complement cascade, and production of proinflammatory cytokines. In this review, emphasis is paid not only to the potential role of parvovirus B19 and Epstein-Barr virus in primis but also to the general involvement of different bacteria as Salmonella spp., Shigella spp., Campylobacter spp., Mycoplasma pneumoniae, Chlamydophila pneumoniae, Bartonella henselae, and Streptococcus pyogenes for the development of immune-mediated arthritides during childhood. No unequivocal evidence favoring or refuting these associations has been clearly proved, and today, the strict definition of JIA etiology remains unknown. The infection can represent a random event in a susceptible individual, or it can be a necessary factor in JIA development, always in combination with a peculiar genetic background. Further studies are needed in order to address the unsolved questions

  12. Risk Factors for Idiopathic Optic Neuritis Recurrence

    PubMed Central

    Zhang, Yu-Jiao; Li, Kaijun; He, Jian-Feng

    2014-01-01

    Background Approximately 30–50% of idiopathic optic neuritis (ION) patients experience one or multiple episodes of recurrence. The aim of this study was to search for risk factors for ION recurrence. Methods Clinical data on hospitalized patients diagnosed with ION between January 2003 and January 2011 at the First Affiliated Hospital of Guangxi Medical University were retrospectively collected. Univariate and multivariate analyses were performed on factors that might cause ION recurrence. In total, 115 ION cases (32 recurrent and 83 non-recurrent cases) with complete data were analyzed. The length of the follow-up period ranged from 12 to 108 months (median: 42 months). Results The univariate analysis showed that the recurrence rate for unilateral ION was higher than that for bilateral ION (40% vs. 12%, p = 0.001). Underlying diseases had a significant impact on recurrence (p<0.001): the recurrence rates due to neuromyelitis optica (NMO), multiple sclerosis (MS), demyelinating lesions alone of the central nervous system, and unknown causes were 89%, 70%, 41%, and 8.7%, respectively. The multivariate analysis showed that the factors causing relatively high recurrence rates included NMO (odds ratio [OR], 73.5; 95% confidence interval [CI], 7.3 to 740.9), MS (OR, 33.9; 95% CI, 5.2 to 222.2), and demyelinating lesions alone (OR, 8.9; 95% CI, 2.3 to 34.4), unilateral involvement (OR, 5.7; 95% CI, 1.5 to 21.3), relatively low initial glucocorticoid dosage (equivalent to ≤100 mg prednisone/day) (OR, 4.3; 95% CI, 1.0 to 17.9). Conclusion Underlying diseases, laterality (unilateral or bilateral), and initial glucocorticoid dosage are important risk factors of ION recurrence. Clinical physicians are advised to treat ION patients with a sufficient dose of glucocorticoid in the initial treatment stage to reduce the recurrence risk. PMID:25255372

  13. Recent advances in understanding idiopathic pulmonary fibrosis

    PubMed Central

    Daccord, Cécile; Maher, Toby M.

    2016-01-01

    Despite major research efforts leading to the recent approval of pirfenidone and nintedanib, the dismal prognosis of idiopathic pulmonary fibrosis (IPF) remains unchanged. The elaboration of international diagnostic criteria and disease stratification models based on clinical, physiological, radiological, and histopathological features has improved the accuracy of IPF diagnosis and prediction of mortality risk. Nevertheless, given the marked heterogeneity in clinical phenotype and the considerable overlap of IPF with other fibrotic interstitial lung diseases (ILDs), about 10% of cases of pulmonary fibrosis remain unclassifiable. Moreover, currently available tools fail to detect early IPF, predict the highly variable course of the disease, and assess response to antifibrotic drugs. Recent advances in understanding the multiple interrelated pathogenic pathways underlying IPF have identified various molecular phenotypes resulting from complex interactions among genetic, epigenetic, transcriptional, post-transcriptional, metabolic, and environmental factors. These different disease endotypes appear to confer variable susceptibility to the condition, differing risks of rapid progression, and, possibly, altered responses to therapy. The development and validation of diagnostic and prognostic biomarkers are necessary to enable a more precise and earlier diagnosis of IPF and to improve prediction of future disease behaviour. The availability of approved antifibrotic therapies together with potential new drugs currently under evaluation also highlights the need for biomarkers able to predict and assess treatment responsiveness, thereby allowing individualised treatment based on risk of progression and drug response. This approach of disease stratification and personalised medicine is already used in the routine management of many cancers and provides a potential road map for guiding clinical care in IPF. PMID:27303645

  14. Adult idiopathic scoliosis: the tethered spine.

    PubMed

    Whyte Ferguson, Lucy

    2014-01-01

    This article reports on an observational and treatment study using three case histories to describe common patterns of muscle and fascial asymmetry in adults with idiopathic scoliosis (IS) who have significant scoliotic curvatures that were not surgically corrected and who have chronic pain. Rather than being located in the paraspinal muscles, the myofascial trigger points (TrPs) apparently responsible for the pain were located at some distance from the spine, yet referred pain to locations throughout the thoracolumbar spine. Asymmetries in these muscles appear to tether the spine in such a way that they contribute to scoliotic curvatures. Evaluation also showed that each of these individuals had major ligamentous laxity and this may also have contributed to development of scoliotic curvatures. Treatment focused on release of TrPs found to refer pain into the spine, release of related fascia, and correction of related joint dysfunction. Treatment resulted in substantial relief of longstanding chronic pain. Treatment thus validated the diagnostic hypothesis that myofascial and fascial asymmetries were to some extent responsible for pain in adults with significant scoliotic curvatures. Treatment of these patterns of TrPs and muscle and fascial asymmetries and related joint dysfunction was also effective in relieving pain in each of these individuals after they were injured in auto accidents. Treatment of myofascial TrPs and asymmetrical fascial tension along with treatment of accompanying joint dysfunction is proposed as an effective approach to treating both chronic and acute pain in adults with scoliosis that has not been surgically corrected. PMID:24411157

  15. Transitioning from Idiopathic to Explainable Autoimmune Hepatitis.

    PubMed

    Czaja, Albert J

    2015-10-01

    Autoimmune hepatitis lacks an identifiable cause, and its diagnosis requires the exclusion of etiologically defined diseases that resemble it. Insights into its pathogenesis are moving autoimmune hepatitis from an idiopathic to explainable disease, and the goal of this review is to describe the insights that are hastening this transition. Two types of autoimmune hepatitis are justified by serological markers, but they also have distinctive genetic associations (DRB1 and DQB1 genes) and autoantigens. DRB1 alleles are the principal susceptibility factors in white adults, and a six amino acid sequence encoded in the antigen-binding groove of class II molecules of the major histocompatibility complex can influence the selection of autoantigens. Polymorphisms, including variants of SH2B3 and CARD10 genes, may affect immune reactivity and disease severity. The cytochrome mono-oxygenase, CYP2D6, is the autoantigen associated with type 2 autoimmune hepatitis, and it shares homologies with multiple viruses that might promote self-intolerance by molecular mimicry. Chemokines, especially CXCL9 and CXCL10, orchestrate the migration of effector cells to sites of injury and are associated with disease severity. Cells of the innate and adaptive immune responses promote tissue damage, and possible deficiencies in the number and function of regulatory T cells may facilitate the injurious process. Receptor-mediated apoptosis is the principal mechanism of hepatocyte loss, and cell-mediated and antibody-dependent mechanisms of cytotoxicity also contribute. Insights that explain autoimmune hepatitis will allow triggering exogenous antigens to be characterized, risk management to be improved, prognostic indices to be refined, and site-specific therapeutic interventions to emerge. PMID:25999246

  16. Immune Complexes in Juvenile Idiopathic Arthritis

    PubMed Central

    Moore, Terry L.

    2016-01-01

    Juvenile idiopathic arthritis (JIA) reflects a group of clinically heterogeneous, autoimmune disorders in children characterized by chronic arthritis and hallmarked by elevated levels of circulating immune complexes (CICs) and associated complement activation by-products in their sera. Immune complexes (ICs) have been detected in patients’ sera with JIA utilizing a variety of methods, including the anti-human IgM affinity column, C1q solid-phase assay, polyethylene glycol precipitation, Staphylococcal Protein A separation method, anti-C1q/C3 affinity columns, and FcγRIII affinity method. As many as 75% of JIA patients have had IC detected in their sera. The CIC proteome in JIA patients has been examined to elucidate disease-associated proteins that are expressed in active disease. Evaluation of these ICs has shown the presence of multiple peptide fragments by SDS-PAGE and 2-DE. Subsequently, all isotypes of rheumatoid factor (RF), isotypes of anti-cyclic citrullinated peptide (CCP) antibodies, IgG, C1q, C4, C3, and the membrane attack complex (MAC) were detected in these IC. Complement activation and levels of IC correlate with disease activity in JIA, indicating their role in the pathophysiology of the disease. This review will summarize the existing literature and discuss the role of possible protein modification that participates in the generation of the immune response. We will address the possible role of these events in the development of ectopic germinal centers that become the secondary site of plasma cell development in JIA. We will further address possible therapeutic modalities that could be instituted as a result of the information gathered by the presence of ICs in JIA. PMID:27242784

  17. Objective cough frequency in Idiopathic Pulmonary Fibrosis

    PubMed Central

    2010-01-01

    Background Cough is a common presenting symptom in patients with Idiopathic Pulmonary Fibrosis (IPF). This study measured cough rates in IPF patients and investigated the association between cough and measures of health related quality of life and subjective cough assessments. In addition, IPF cough rates were related to measures of physiological disease severity and compared to cough rates in health and other respiratory conditions. Methods Nineteen IPF patients, mean age 70.8 years ± 8.6, five female (26.3%) were studied. Subjects performed full pulmonary function testing, 24 hour ambulatory cough recordings, completed a cough related quality of life questionnaire (Leicester Cough Questionnaire) and subjectively scored cough severity with a visual analogue scale. Ambulatory cough recordings were manually counted and reported as number of coughs per hour. Results The 24hr cough rates were high (median 9.4, range 1.5-39.4), with day time rates much higher than night time (median 14.6, range 1.9-56.6 compared to 1.9, range 0-19.2, p = 0.003). Strong correlations were found between objective cough frequency and both the VAS (day r = 0.80, p < 0.001, night r = 0.71, p = 0.001) and LCQ (r = -0.80, p < 0.001), but not with measures of pulmonary function. Cough rates in IPF were higher than healthy subjects (p < 0.001) and asthma patients (p < 0.001) but similar to patients with chronic cough (p = 0.33). Conclusions This study confirms objectively that cough is a major, very distressing and disabling symptom in IPF patients. The strong correlations between objective cough counts and cough related quality of life measures suggest that in IPF patient's, perception of cough frequency is very accurate. PMID:20565979

  18. Bullous variant of idiopathic central serous chorioretinopathy

    PubMed Central

    Sahu, D.; Namperumalsamy, P; Hilton, G.; de Sousa, N. F

    2000-01-01

    BACKGROUND—Spontaneous bullous serous retinal detachment (RD) with subretinal exudation complicating idiopathic central serous chorioretinopathy (ICSC) is a rare and infrequently described clinical entity. Clinical observations are described on this variant form in 11 patients, the largest series reported to date.
METHODS—13 eyes of 11 Indian patients having this entity were followed up clinically and angiographically for 12-24 months (retrospective, longitudinal). None of the patients had any previous history of other diseases nor were they on any medications. Four eyes received laser treatment (group A); nine eyes were not treated (group B).
RESULTS—All 11 patients were male, aged 23-49 years (median 37 years). The clinical and photographic records revealed subretinal exudation and inferior bullous serous RD complicating ICSC with evidence of large, single or multiple, leaking retinal pigment epithelial detachments (PEDs) in all the cases. In group A, resolution of serous RD occurred in 12 weeks (median) with a visual recovery of ⩾20/30 in three out of four eyes while in group B resolution of serous retinal detachment was observed in 14 weeks (median) with eight out of nine eyes achieving a visual acuity of ⩾20/30. Subretinal fibrosis developed in two eyes in group A and none of the eyes in group B.
CONCLUSION—The disease is an exaggerated form of ICSC and can occur spontaneously without any history of corticosteroid therapy. Recognition of this atypical presentation is important to avoid inappropriate treatment. These observations suggest that with respect to the duration of the disease and the final visual outcome laser therapy offers no additional benefit over the natural course of this variant form of ICSC.

 PMID:10781512

  19. Immune Complexes in Juvenile Idiopathic Arthritis.

    PubMed

    Moore, Terry L

    2016-01-01

    Juvenile idiopathic arthritis (JIA) reflects a group of clinically heterogeneous, autoimmune disorders in children characterized by chronic arthritis and hallmarked by elevated levels of circulating immune complexes (CICs) and associated complement activation by-products in their sera. Immune complexes (ICs) have been detected in patients' sera with JIA utilizing a variety of methods, including the anti-human IgM affinity column, C1q solid-phase assay, polyethylene glycol precipitation, Staphylococcal Protein A separation method, anti-C1q/C3 affinity columns, and FcγRIII affinity method. As many as 75% of JIA patients have had IC detected in their sera. The CIC proteome in JIA patients has been examined to elucidate disease-associated proteins that are expressed in active disease. Evaluation of these ICs has shown the presence of multiple peptide fragments by SDS-PAGE and 2-DE. Subsequently, all isotypes of rheumatoid factor (RF), isotypes of anti-cyclic citrullinated peptide (CCP) antibodies, IgG, C1q, C4, C3, and the membrane attack complex (MAC) were detected in these IC. Complement activation and levels of IC correlate with disease activity in JIA, indicating their role in the pathophysiology of the disease. This review will summarize the existing literature and discuss the role of possible protein modification that participates in the generation of the immune response. We will address the possible role of these events in the development of ectopic germinal centers that become the secondary site of plasma cell development in JIA. We will further address possible therapeutic modalities that could be instituted as a result of the information gathered by the presence of ICs in JIA. PMID:27242784

  20. The Use of Cotrel-Dubousset Instrumentation in the Treatment of Adult Idiopathic Scoliosis

    PubMed Central

    Skubic, John W.; Kostuik, John P.; Suh, Paul B.; Salo, Paul

    1991-01-01

    Between November 1985 and April 1988, forty-nine adults (forty-three females) underwent correction of their spinal deformity secondary to idiopathic scoliosis with Cotrel-Dubousset Instrumentation (CDI). Average age at the time of surgery was forty-three years (twenty-one to seventy-five years) with nineteen patients older than fifty years. Twelve patients had previous spinal fusions and nineteen patients required staged surgery. Follow up averaged thirty-four months (range twenty-four to fifty months). Average preoperative scoliosis was 60 degrees (range 26 to 115 degrees), and at final follow-up averaged 35 degrees (forty-two per cent correction). Previously unfused patients averaged 50 per cent correction. Sagittal curve correction was most dramatic in the hypokyphotic thoracic or hypolordotic lumbar spine, averaging 173 and 133 per cent improvement respectively. For lumbar curves especially, surgical technique depended on the flexibility of the curve, sagittal alignment, and on the need to fuse to the sacrum as determined by preoperative lumbosacral discography and pain reproduction. Twentyeight complications occurred in twenty patients. Loss of fixation due to lumbar lamina fracture and hook pull out occurred in four patients over age fifty who had a staged anterior release. Supplementing distal lumbar fixation with pedicle screws in this category of patients appears to be a viable solution. One nerve root neuropraxia attributable to the instrumentation resolved. Pain relief occurred in 82 per cent. ImagesFigures 1-A-B-C-DFigures 2-A, 2-B, 2-C and 2-DFigures 3-A, 3-B, 3-C and 3-DFigures 4-A, 4-B, 4-C and 4-DFigures 5-A, 5-B, 5-C and 5-DFigures 6-A, 6-B, 6-C and 6-DFigures 7-A, 7-B and 7-C

  1. Treatment of Idiopathic Clubfoot in the Ponseti Era and Beyond.

    PubMed

    Chu, Alice; Lehman, Wallace B

    2015-12-01

    The initial treatment of idiopathic clubfoot was mostly surgical for the 1980s/1990s. In the latter half of the 1990s, there was a surge of interest in the Ponseti method of casting after the publication of Dr. Ponseti's 30-year results. Many authors have since shown correction rates in the high 90th percentile, rendering posteromedial release surgery almost obsolete. The success of the Ponseti method has been brought internationally and extrapolated to more and more difficult cases, such as idiopathic or syndromic, primary or recurrence. This new trend will create a different subset of complications. PMID:26589078

  2. Update on diagnosis and treatment of idiopathic pulmonary fibrosis.

    PubMed

    Baddini-Martinez, José; Baldi, Bruno Guedes; Costa, Cláudia Henrique da; 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

  3. Idiopathic Gingival Fibromatosis: Case Report and Review of the Literature.

    PubMed

    Ko, Yen Chen K; Farr, Jeffrey B; Yoon, Angela; Philipone, Elizabeth

    2016-06-01

    Gingival fibromatosis (GF) is a condition characterized by a progressive, normal colored enlargement of the gingiva caused by an increase in the size of submucosal connective tissue. Both familial and idiopathic variants of the condition exist. The authors present a case report of a 38-year-old African American man who presented with an impressive overgrowth of the maxillary and mandibular gingivae, subsequently diagnosed as idiopathic GF. In this report, the authors will review the etiologies, treatment, and clinical and histological findings of GF, review similar cases found in the literature, and discuss the differential diagnosis for diffuse gingival enlargements. PMID:27043333

  4. Disseminated eosinophilic disease resembling idiopathic hypereosinophilic syndrome in a dog.

    PubMed

    Aroch, I; Perl, S; Markovics, A

    2001-09-29

    True idiopathic hypereosinophilic syndrome has been described in human beings and cats, but not in dogs. The syndrome is characterised by prolonged unexplained peripheral mature eosinophilia, the infiltration of many organs by eosinophils, organ dysfunction and a fatal outcome. This paper describes an idiopathic disseminated eosinophilic disease in a dog involving various organs, manly the heart and the lungs, accompanied by a leukemoid eosinophilic response, and a fatal outcome. The histopathological findings included the infiltration of the myocardium, lung parenchyma, liver, spleen, lymph nodes and skeletal muscles with eosiniphils. PMID:11601516

  5. Update on diagnosis and treatment of idiopathic pulmonary fibrosis

    PubMed Central

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

    2015-01-01

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

  6. Clinical Analysis and Management of Acquired Idiopathic Generalized Anhidrosis.

    PubMed

    Satoh, Takahiro

    2016-01-01

    Acquired idiopathic generalized anhidrosis (AIGA) is a sweating disorder characterized by inadequate sweating in response to heat stimuli such as high temperature, humidity, and physical exercise. Patients exhibit widespread nonsegmental hypohidrosis/anhidrosis without any apparent cause, but the palms, soles, and axillae are rarely affected. Heat stroke readily develops due to increased body temperature. AIGA commonly affects young males. Approximately 30-60% of patients show complications of cholinergic urticaria, also known as idiopathic pure sudomotor failure or hypohidrotic cholinergic urticaria. Systemic corticosteroids are the most effective therapy, although recurrence is not uncommon. PMID:27584965

  7. Alpha1-adrenoceptors in the guinea pig thoracic aorta.

    PubMed

    Yamamoto, Y; Koike, K

    1999-01-01

    In the present study, we tried to determine which alpha1-adrenoceptor subtypes are involved in the guinea pig thoracic aorta by using in vitro functional analysis. In first, we tried to estimate the pA2 values of some key alpha1-adrenoceptor antagonists (prazosin, 5-methylurapidil, WB4101, BMY7378 and tamsulosin) against responses to norepinephrine in the thoracic aorta of guinea pigs. The concentration-response curves of norepinephrine were rightward shifted by the presence of prazosin, 5 methylurapidil, WB4101, BMY7378 and tamsulosin. The pA2 values for these antagonists against norepinephrine were 7.83, 7.78, 8.20, 5.73 and 9.57, respectively. In second, we tried to compare the estimated pA2 values obtained in the present study with reported pKi and pA2 values for cloned and native alpha1-adrenoceptor subtypes. In rabbit mesenteric artery, trigone, urethra, prostate and human lower urinary tract which were proposed to contain the putative alpha1L-adenoceptor, we obtained the good correlation for the pA2 values reported in these tissues with pA2 values estimated in guinea pig thoracic aorta. Moreover, regression lines were close to the line of identity. These results suggest that the alpha1-adenoceptors mediating contraction of guinea pig thoracic aorta are similar pharmacologically to the putative alpha1L-adenoceptor subtype in rabbit mesenteric artery, trigone, urethra, prostate and human lower urinary tract. As a final point, guinea pig thoracic aorta may be able to use as a tool to develop the new alpha1-adrenoceptor antagonist which is therapeutically advantageous in the treatment of urinary tract obstruction (e. g., in benign prostatic hyperplasia). PMID:10733154

  8. Penetrating thoracic injury with retained foreign body: can video-assisted thoracic surgery take up the leading role in acute management?

    PubMed Central

    Yu, Peter S. Y.; Chan, Herman H. M.; Lau, Rainbow W. H.; Capili, Freddie G.; Underwood, Malcolm J.

    2016-01-01

    Video-assisted thoracic surgery (VATS) is widely adopted in acute management of patient with thoracic trauma, but its use in penetrating thoracic injuries with retained foreign bodies were rarely reported. We described three of such cases using VATS as the first line approach. Identification of injuries, control of bleeders, clot evacuation, resection of damaged lung parenchyma and safe retrieval of foreign bodies were all performed via complete VATS within short operative time. Patient were uneventfully discharged during early post-operative period. We suggest that, for haemodynamically stable patients, VATS offers a safe and minimally-invasive alternative to conventional thoracotomy for penetrating thoracic injury with retained foreign bodies. PMID:27621884

  9. Penetrating thoracic injury with retained foreign body: can video-assisted thoracic surgery take up the leading role in acute management?

    PubMed

    Yu, Peter S Y; Chan, Herman H M; Lau, Rainbow W H; Capili, Freddie G; Underwood, Malcolm J; Wan, Innes Y P

    2016-08-01

    Video-assisted thoracic surgery (VATS) is widely adopted in acute management of patient with thoracic trauma, but its use in penetrating thoracic injuries with retained foreign bodies were rarely reported. We described three of such cases using VATS as the first line approach. Identification of injuries, control of bleeders, clot evacuation, resection of damaged lung parenchyma and safe retrieval of foreign bodies were all performed via complete VATS within short operative time. Patient were uneventfully discharged during early post-operative period. We suggest that, for haemodynamically stable patients, VATS offers a safe and minimally-invasive alternative to conventional thoracotomy for penetrating thoracic injury with retained foreign bodies. PMID:27621884

  10. Impact of Nonvascular Thoracic MR Imaging on the Clinical Decision Making of Thoracic Surgeons: A 2-year Prospective Study.

    PubMed

    Ackman, Jeanne B; Gaissert, Henning A; Lanuti, Michael; Digumarthy, Subba R; Shepard, Jo-Anne O; Halpern, Elkan F; Wright, Cameron D

    2016-08-01

    Purpose To determine the impact of nonvascular thoracic magnetic resonance (MR) imaging on the clinical decision making and diagnostic certainty of thoracic surgeons. Materials and Methods Seven thoracic surgeons at Massachusetts General Hospital, an academic quaternary referral hospital, participated in this 2-year, prospective, institution review board-approved, HIPAA-compliant pre- and post-MR imaging survey study after completing a one-time demographic survey. Between July 16, 2013, and July 13, 2015, each time a thoracic surgeon ordered a nonvascular thoracic MR imaging study via radiology order entry, he or she was sent a link to the pre-test survey that ascertained the clinical rationale for MR imaging, the clinical management plan if MR imaging was not an option, and pre-test diagnostic certainty. Upon completion of the MR imaging report, the surgeon was sent a link to the post-test survey assessing if/how MR imaging changed clinical management, the surgeon's comfort with the clinical management plan, and post-test diagnostic certainty. Data were analyzed with Student t, Wilcoxon, and McNemar tests. Results A total of 99 pre- and post-test surveys were completed. Most MR imaging studies (64 of 99 [65%]) were requested because of indeterminate computed tomographic findings. The use of MR imaging significantly reduced the number of planned surgical interventions (P < .001), modified the surgical approach in 54% (14 of 26) of surgical cases, and increased surgeon comfort with the patient management plan in 95% (94 of 99) of cases. Increased diagnostic certainty as a result of MR imaging was highly significant (P < .0001). In 21% (21 of 99) of cases, definitive MR imaging results warranted no further follow-up or clinical care. Conclusion In appropriate cases, assessment with nonvascular thoracic MR imaging substantially affects the clinical decision making and diagnostic certainty of thoracic surgeons. (©) RSNA, 2016 Online supplemental material is available

  11. Effect of thoracic and cervical joint mobilization on pulmonary function in stroke patients

    PubMed Central

    Jang, Sang-Hun; Bang, Hyun-Soo

    2016-01-01

    [Purpose] This study aimed to conduct thoracic and cervical mobilization in stroke patients and determine its effects on respiratory function. [Subjects and Methods] Twenty-one stroke patients were studied. Subjects were divided into a control group (control group, n=11) who did not undergo thoracic and cervical joint mobilization, and an experimental group (thoracic and cervical mobilization group, n=10) who underwent thoracic and cervical joint mobilization. Forced vital capacity and forced expiratory volume in the first second, well-known indicators of respiratory capabilities, were measured. Peak cough flow was measured as an indicator of cough capability. [Results] After the exercise, respiratory function in the thoracic and cervical mobilization group showed statistically significant improvements demonstrated by increases in forced vital capacity, forced expiratory volume in the first second, and peak cough flow. [Conclusion] The findings indicate that thoracic and cervical mobilization can improve the thoracic movements of stroke patients resulting in improved pulmonary function. PMID:26957769

  12. Near-Infrared Fluorescence Imaging of Thoracic Duct Anatomy and Function in Open Surgery and Video-Assisted Thoracic Surgery

    PubMed Central

    Ashitate, Yoshitomo; Tanaka, Eiichi; Stockdale, Alan; Choi, Hak Soo; Frangioni, John V.

    2011-01-01

    Objective Chylothorax resulting from damage to the thoracic duct is often difficult to identify and repair. We hypothesized that near-infrared (NIR) fluorescent light could provide sensitive, real-time, high-resolution intraoperative imaging of thoracic duct anatomy and function. Methods In 16 rats (n=16), four potential NIR fluorescent lymphatic tracers were compared in terms of signal strength and imaging time: indocyanine green (ICG), the carboxylic acid of CW800, ICG adsorbed to human serum albumin (HSA), and CW800 conjugated covalently to HSA (HSA800). The optimal agent was validated in eight pigs approaching the size of humans, n = 6 by open surgery using the Fluorescence-Assisted Resection and Exploration (FLARE) imaging system and n = 2 by video-assisted thoracoscopic surgery (VATS) using the minimally invasive imaging system (m-FLARE). Lymphatic tracer injection site, dose, and timing were optimized. Results For signal strength, sustained imaging time, and clinical translatability, the best lymphatic tracer was ICG, which is already FDA-approved for other indications. In pigs, a simple subcutaneous injection of ICG into the lower leg, at a dose ≥36 μg/kg, provided thoracic duct imaging with an onset of 5 min after injection, sustained imaging for at least 60 min after injection, and a signal-to-background ratio ≥2. Using this technology, normal thoracic duct flow, collateral flow, injury models, and repair models could all be observed under direct visualization. Conclusions NIR fluorescent light could provide sensitive, sustained, real-time imaging of thoracic duct anatomy and function during both open surgery and VATS in animal models. PMID:21477818

  13. An empiric treatment for idiopathic oligozoospermia revisited: a 20-year investigative saga.

    PubMed

    Koukkou, E; Billa, E; Kapolla, N; Pappa, A; Venaki, E; Andreou, L; Nicopoulou, S C; Adamopoulos, D A

    2012-10-01

    A series of studies aiming at introducing an effective treatment for idiopathic oligozoospermia was conducted in a step-wise fashion spanning over a 20-year period. The concept was that co-administration of an accessory gland-stimulating androgen, testosterone undecanoate (40 mg t.i.d.) and the FSH raising anti-oestrogen tamoxifen citrate (10 mg b.i.d.) may improve sperm parameters. A prerequisite for such an effect was the demonstration that testosterone undecanoate had no suppressing action on pituitary-testicular axis. In this context, initial studies demonstrated no change in basal or stimulated gonadotrophin and testosterone secretion in short- or long-term protocols. Two subsequent trials with this combination showed a marked improvement of sperm parameters and pregnancy incidence, with a seasonal variation noted in response to treatment, this being higher during the cold seasons of autumn and winter. Regarding the mechanism of testosterone undecanoate's action, a recent study from our unit showed that its administration resulted in a marked rise of serum DHT levels. Because this steroid is an epididymal function promoter, it appears that its contribution in the combination is mediated mainly through its DHT raising effect. By and large, this empiric approach for the treatment of idiopathic oligozoospermia was satisfactorily documented after a 20-year investigative saga. PMID:22946848

  14. Explanatory and pragmatic perspectives regarding idiopathic physical symptoms and related syndromes.

    PubMed

    Engel, Charles C

    2006-03-01

    In recent years, research-methods literature mainly addressing controlled clinical trials has arisen regarding explanatory and pragmatic treatment trials. Explanatory trials tend to examine causal mechanisms and questions of efficacy and value internal validity (creating optimal study conditions) over generalizability (using study results to understand treatment effects in real-life patient populations). In contrast, pragmatic trials value "external relevance" (generalizability) of study results over "internal elegance" so that clinicians and health policymakers can better understand how treatments might impact their patients and policies. This review draws inspiration from these contrasting explanatory and pragmatic perspectives and develops them for clinical and research pertaining to idiopathic physical symptoms and related syndromes (eg, somatization disorder, chronic fatigue syndrome, multiple chemical sensitivities, irritable bowel syndrome). Explanatory and pragmatic perspectives are used to examine these idiopathies with regard to causation, case definition, labels, and treatment. It is concluded that idiopathic symptom syndromes are fundamentally pragmatic clinical and research challenges. For epidemiologic and methodologic reasons, the complex explanations for these syndromes remain largely elusive. Even so, scientific and clinical pragmatism offers the opportunity to reduce disagreement between competing medical disciplines and between clinicians and affected patients with regard to irreconcilable etiologic questions and to remain evidence-based in the care of patients. PMID:16575379

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

    PubMed

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

    2015-01-01

    Machrophage activation syndrome (MAS) is a rare and potentially fatal disease, commonly associated with chronic rheumatic diseases, mainly juvenile idiopathic arthritis. It is included in the group of secondary forms of haemophagocytic syndrome, and other causes are lymphoproliferative diseases and infections. Its most important clinical and laboratorial manifestations are non-remitting fever, splenomegaly, bleeding, impairment of liver function, cytopenias, hypoalbuminemia, hypertriglyceridemia, hypofibrinogenemia and hyperferritinemia. The treatment needs to be started quickly, and the majority of cases have a good response with corticosteroids and cyclosporine. The Epstein-Barr virus is described as a possible trigger for many cases of MAS, especially in these patients in treatment with tumor necrosis factor (TNF) blockers. In these refractory cases, etoposide (VP16) should be administered, associated with corticosteroids and cyclosporine. Our objective is to describe a rare case of MAS probably due to EBV infection in a subject with systemic-onset juvenile idiopathic arthritis, which achieved complete remission of the disease after therapy guided by 2004-HLH protocol. PMID:25575650

  16. Craniofacial surgical management of a patient with systematic juvenile idiopathic arthritis and Crohn's disease.

    PubMed

    Kasfikis, Georgios; Georgios, Kasfikis; Antoniades, Helias; Helias, Antoniades; Kyrgidis, Athanassios; Athanassios, Kyrgidis; Markovitsi, Eleni; Eleni, Markovitsi; Antoniades, Konstantinos; Konstantinos, Antoniades

    2009-05-01

    Juvenile idiopathic arthritis (JIA) is the most common chronic inflammatory disease in early age. It affects one or more joints, lasts more than 3 weeks, and appears in patients younger than 16 years. Juvenile idiopathic arthritis is classified according to the International League of Associations for Rheumatology consensus depending on the number of affected joints in the beginning of the disease. When JIA affects the temporomandibular joint, the development of the mandible is constrained. Patients show a tendency toward retrognathism and a vertical facial development pattern. The purpose of this study was to present a rare case of a young teenager who experienced JIA and Crohn's disease at the same time. The patient was referred to the hospital for aesthetic and functional problems, mainly convex facial profile and obstructive sleep apnea caused by the craniofacial abnormality. The patient was treated by sagittal split mandibular advancement osteotomy and advancement genioplasty. The mechanisms of obstructive sleep apnea development and the surgical treatment through osteotomies are commentated on. The surgical outcome is functionally and aesthetically favorable and solid 2 years after the operation. Surgical management of the craniofacial region can be a problem-solving treatment modality for patients with juvenile arthritis. PMID:19461338

  17. Identification of Clinical Phenotypes in Idiopathic Interstitial Pneumonia with Pulmonary Emphysema.

    PubMed

    Sato, Suguru; Tanino, Yoshinori; Misa, Kenichi; Fukuhara, Naoko; Nikaido, Takefumi; Uematsu, Manabu; Fukuhara, Atsuro; Wang, Xintao; Ishida, Takashi; Munakata, Mitsuru

    2016-01-01

    Objective Since the term "combined pulmonary fibrosis and emphysema" (CPFE) was first proposed, the co-existence of pulmonary fibrosis and pulmonary emphysema (PE) has drawn considerable attention. However, conflicting results on the clinical characteristics of patients with both pulmonary fibrosis and PE have been published because of the lack of an exact definition of CPFE. The goal of this study was thus to clarify the clinical characteristics and phenotypes of idiopathic interstitial pneumonia (IIP) with PE. Methods We retrospectively analyzed IIP patients who had been admitted to our hospital. Their chest high-resolution computed tomography images were classified into two groups according to the presence of PE. We then performed a cluster analysis to identify the phenotypes of IIP patients with PE. Results Forty-four (53.7%) out of 82 patients had at least mild emphysema in their bilateral lungs. The cluster analysis separated the IIP patients with PE into three clusters. The overall survival rate of one cluster that consisted of mainly idiopathic pulmonary fibrosis (IPF) patients was significantly worse than those of the other clusters. Conclusion Three different phenotypes can be identified in IIP patients with PE, and IPF with PE is a distinct clinical phenotype with a poor prognosis. PMID:27301501

  18. Radiographic detection of thoracic lesions in adult cows: A retrospective study of 42 cases (1995–2002)

    PubMed Central

    Masseau, Isabelle; Fecteau, Gilles; Breton, Luc; Hélie, Pierre; Beauregard, Guy; Blond, Laurent

    2008-01-01

    Medical records of 42 cows that underwent both thoracic radiographic and postmortem examinations within a period of 7 days were reviewed to develop an evaluation grid to interpret bovine thoracic radiographs and to determine the sensitivity and the specificity of thoracic radiographs for detection of thoracic lesions, based on postmortem examination. Most cows (64%) had clinical signs of respiratory disease, whereas 19% showed signs of cardiac problems. The sensitivity and specificity of radiographs for identifying cows with thoracic lesions were 94% and 50%, respectively. In this study, with a prevalence of thoracic lesions of 86%, the positive- and negative-predictive values were 92% and 57%, respectively. This study provides an evaluation grid that allows standardization of the reading of bovine thoracic radiographs and the identification of most thoracic lesions. Bovine thoracic radiographs are useful in detecting thoracic lesions in cows. PMID:18390098

  19. Strontium oral load test in children with idiopathic hypercalciuria.

    PubMed

    Fernández, Porfirio; Santos, Fernando; Sotorrío, Pilar; Mayordomo, Juan; Ferrero, Luis

    2007-09-01

    Increased intestinal calcium absorption may play an important role in the pathogenesis of idiopathic hypercalciuria in children. Calcium absorption was assessed by an oral strontium load test in 22 prepubertal children (13 male) with idiopathic hypercalciuria, urinary calcium excretion 6.48 +/- 0.60 mg/kg per day (range 4.12-13.40 mg/kg per day), and ten healthy, young, normocalciuric controls (six male). After administration of 2.65 mg/kg of strontium chloride (SrCl(2)), the serum concentrations of strontium at 30 min, 60 min, 120 min, 240 min, and the fraction of the absorbed dose (FAD%) at 30 min, 60 min and 240 min, were similar in both groups. FAD% at 120 min was lower (P < 0.05) in hypercalciuric children than in controls (11.84 +/- 0.96% vs 15.87 +/- 1.77%). Values of the area under the curve were not different between both groups. In children with idiopathic hypercalciuria, serum basal intact parathyroid hormone (PTH) (r = -0.59, P = 0.004) and the 1,25-dihydroxyvitamin D/PTH ratio (r = 0.65; P = 0.001) were correlated with the serum concentration of strontium at 60 min. The study reported here provides, for the first time, the results of a strontium oral load test in children with idiopathic hypercalciuria. With this method no major alterations of intestinal calcium absorption were found in this disorder. PMID:17541793

  20. Idiopathic systemic AA-amyloidosis in a skunk (Mephitis mephitis).

    PubMed

    Elhensheri, Mohamed; Linke, Reinhold P; Blankenburg, Anja; Beineke, Andreas

    2012-03-01

    This report describes a case of systemic amyloidosis in a captive striped skunk. At necropsy, bilateral alopecia, as well as reno-, hepato-, and splenomegaly were present. Congo red staining and immunohistochemistry revealed depositions of AA-amyloid in different organs. The lack of a predisposing disease is suggestive of idiopathic systemic AA-amyloidosis. PMID:22448530