Sample records for hamartomas dos ductos

  1. Pancreatic Lipomatous Hamartoma: A Hitherto Unrecognized Variant.

    PubMed

    Tanaka, Mariko; Ushiku, Tetsuo; Ikemura, Masako; Takazawa, Yutaka; Igari, Toru; Shimizu, Michio; Yamaguchi, Hiroshi; Fukushima, Noriyoshi; Sakuma, Kei; Arita, Junichi; Sakamoto, Yoshihiro; Hasegawa, Kiyoshi; Watadani, Takeyuki; Nakai, Yousuke; Koike, Kazuhiko; Fukayama, Masashi

    2018-05-04

    Pancreatic masses consisting of lipomatous components clinically include lipoma, liposarcoma, lipomatous pseudohypertrophy of the pancreas, fat-containing neoplasms such as perivascular epithelioid cell tumor, and malignant neoplasm with lipoid degeneration. We present pancreatic lipomatous hamartoma, which has not been reported hitherto. A solid pancreatic mass was detected from a computed tomographic scan check-up in each of 3 cases of Japanese men. Macroscopically, well-demarcated solid lipomatous masses were detected at the uncus, body, and tail of the pancreas, respectively. Microscopically, the masses predominantly consisted of mature adipocytes with no atypia, but contained characteristics components of pancreatic hamartoma, such as small ducts, a well-preserved acinar structure, and/or fibrous stroma. On the basis of the unique features, lack of islets and absence of periductal elastic fibers, these tumors are a distinct variant of pancreatic hamartoma. Furthermore, high-mobility group AT-hook 2 expression in the fibro-adipocytes of this tumor indicated that these cells are an integral component of the pancreatic lipomatous hamartoma. Consequently, the unique tumors described herein are pancreatic lipomatous hamartoma, which must be discriminated from other lipomatous lesions of the pancreas.

  2. Fibrolipomatous hamartoma: pathognomonic on MR imaging.

    PubMed

    Marom, E M; Helms, C A

    1999-05-01

    To assess the MR imaging characteristics, presenting symptoms, age and nerve distribution of fibrolipomatous hamartoma. A computer search was performed of the term fibrolipomatous hamartoma through the musculoskeletal section MR imaging results at our institution from June 7, 1996 to January 21, 1998 followed by a search of the terms lipomatous hamartoma, median nerve, surrounding fat, increased fatty signal, coaxial, and neuroma. MR images and medical files were retrospectively reviewed by two experienced musculoskeletal radiologists for imaging characteristics, nerve and age distribution as well as for history of trauma. In addition three consultation cases from outside institutions were added for determination of image characteristics. Ten fibrolipomatous hamartomas were identified: eight in the median nerve, one in the ulnar nerve and one in the sciatic nerve. Mean age was 32.3 years (range 4-75 years, SD 21 years). Imaging characteristics were serpiginous low-intensity structures representing thickened nerve fascicles, surrounded by evenly distributed fat, high signal intensity on T1-weighted sequences and low signal intensity on T2-weighted sequences. The amount of fat varied; however, distribution in eight cases (80%) was predominantly between nerve fibers rather than surrounding them peripherally. All had a coaxial-cable-like appearance on axial planes and a spaghetti-like appearance on coronal planes that was not seen in any other type of median nerve abnormality imaged during the study period. The MR imaging characteristics of fibrolipomatous hamartoma are pathognomonic, obviating the need for biopsy for diagnosis.

  3. Leiomyomatous hamartoma of the incisive papilla.

    PubMed

    Corrêa, L; Lotufo, M; Martins, M T; Sugaya, N; de Sousa, S C

    2001-01-01

    A case of unusual hamartoma in a six-year-old otherwise healthy Brazilian girl is reported, with emphasis on histological and immunohistochemical features. A mass observed in the incisive papilla was detected whose appearance was similar to congenital epulis or fibroma. Histological findings showed interlacing fascicles of large spindle cells resembling smooth muscle cells. Immunohistochemical staining for desmin and for smooth-muscle actin was positive. The histological diagnosis was leiomyomatous hamartoma, based on clinical and microscopic observations.

  4. Difficulties in the management of mesenchymal hamartomas.

    PubMed

    Karpelowsky, Jonathan Saul; Pansini, Andrea; Lazarus, Colin; Rode, Heinz; Millar, Alistair J W

    2008-10-01

    Mesenchymal hamartoma of the liver is an entity with a varied presentation and frequent initial delay in diagnosis. The macroscopic appearance too is quite heterogeneous with solid, cystic and mixed variants being present with varying degrees of vascularity. Management will depend on presentation and expertise available. We look at a single centre experience with the mesenchymal hamartomas. Retrospective patient record review of the past 30 years, 1976-2006. Seventeen patients aged 1 day to 15 years were identified, with a histopathological diagnosis of mesenchymal hamartoma of the liver. The anatomical location in the liver was 12 in the right liver and the 5 in the left. All patients presented with abdominal distension, eight had significant anorexia and or vomiting. Ultrasound scan was done in all patients. Findings were that of a mass and or cysts. The cysts were multiple in all cases but one and were interspersed with solid elements. Calcification was noted in only two of the patients. Operative approaches were six right hepatectomy, four wedge excision, seven tumour excisions by division of its pedicle; two of these were done laparoscopically, by cyst drainage and excision of the solid component. The tumours were all confirmed as mesenchymal hamartomas; size ranged from 412 to 2,230 g. Complications included three related to misdiagnosis (hydatid disease, and hepatoblastoma). Intraoperative problems consisted of preoperative bleeding resulting in an on-table hypovolaemic arrest and in a second case a bile duct injury. Postoperative problems consisted of an initial incomplete resection, with residual tumour on the IVC. There was rapid regrowth of tumour and death after a second exploration. Two children developed fluid collections requiring re-exploration and drainage. The surviving children have been followed up for a median time period of 4 months (range 1 month-11 years) and are well. Although hamartomas of the liver are histologically benign, their

  5. Skin hamartoma on the hand of a newborn infant.

    PubMed

    Kaga, Akimune; Itabashi, Chieko; Kanda, Susumu; Suzuki, Yutaka; Tanabu, Muneyuki; Kure, Shigeo

    2013-06-01

    Skin hamartoma is an extremely rare disease on the hand in newborn infants. Reported herein is the case of a newborn infant who presented with a skin hamartoma on the hand. The patient was a girl born at 37 weeks of gestational age. The mass was seen on her proximal left thumb at birth. The mass had a spherical diameter of 4 cm and was pedunculated. One the day after birth, the stalk on the mass was ligated in the neonatal intensive care unit. The mass was diagnosed as skin hamartoma on histopathology. At 1 year of age, the child had good hand function, and no recurrence of the neoplasia was evident. © 2013 The Authors. Pediatrics International © 2013 Japan Pediatric Society.

  6. Cecal vascular hamartoma causing recurrent colic in an Arabian mare.

    PubMed

    Nolf, Marie; Maninchedda, Ugo; Belluco, Sara; Lepage, Olivier; Cadoré, Jean-Luc

    2014-06-01

    A 5-year-old mare was treated for recurrent colic and weight loss by surgical removal of an intraluminal cecal mass. Microscopic examination revealed vascular hamartoma. A 6-month follow-up showed an improvement in the general condition of the mare. Vascular hamartoma should be one of the differential diagnoses for weight loss and colic.

  7. Fibrolipomatous hamartoma of the inferior calcaneal nerve (Baxter nerve).

    PubMed

    Zeng, Rong; Frederick-Dyer, Katherine; Ferguson, N Lynn; Lewis, James; Fu, Yitong

    2012-09-01

    Fibrolipomatous hamartoma (FLH) is a rare, benign lesion of the peripheral nerves most frequently involving the median nerve and its digital branches (80 %). Pathognomonic MR features of FLH such as coaxial-cable-like appearance on axial planes and a spaghetti-like appearance on coronal planes have been described by Marom and Helms, obviating the need for diagnostic biopsy. We present a case of fibrolipomatous hamartoma of the inferior calcaneal nerve (Baxter nerve) with associated subcutaneous fat proliferation.

  8. [Eccrine angiomatous hamartoma: a report of 2 cases].

    PubMed

    Batalla, A; Rosón, E; Flórez, A; Troncoso, A; de la Torre, C

    2011-05-01

    Eccrine angiomatous hamartoma is a rare, benign tumor characterized by a proliferation of eccrine and vascular structures. We present 2 cases and review the characteristics of this disorder. The first patient was a 33-year-old woman who consulted for a brownish lesion on her back associated with local hyperhidrosis. The second patient was a 25-year-old man with an asymptomatic erythematous lesion on his left palm. In both patients a diagnosis of eccrine angiomatous hamartoma was made based on the histological findings. Eccrine angiomatous hamartoma is usually present at birth or develops during childhood. It is typically a solitary lesion and signs and symptoms can vary; diagnosis is therefore based on histological study. The most common site is on the distal parts of limbs. The lesions tend to be asymptomatic, but there may be associated pain and hyperhidrosis. Treatment is not usually necessary except in cases with persistent symptoms, excessive sweating, or cosmetic concerns. Copyright © 2010 Elsevier España, S.L. y AEDV. All rights reserved.

  9. NOVEL PRERETINAL HAIR PIN-LIKE VESSEL IN RETINAL ASTROCYTIC HAMARTOMA WITH VITREOUS HEMORRHAGE.

    PubMed

    Soeta, Megumi; Arai, Yusuke; Takahashi, Hidenori; Fujino, Yujiro; Tanabe, Tatsuro; Inoue, Yuji; Kawashima, Hidetoshi

    2018-01-01

    To report a case of retinal astrocytic hamartoma with vitreous hemorrhage and a hair pin-like vessel adhering to a posterior vitreous membrane. A 33-year-old man with a retinal astrocytic hamartoma presented with vitreous hemorrhage 5 times. Multimodal imaging, including fundus photography, fluorescein angiography, optical coherence tomography, and B-mode ultrasonography. Multimodal imaging demonstrated a novel hair pin-like vessel that adhered to the posterior vitreous membrane. Some cases of retinal astrocytic hamartoma with vitreous hemorrhage may be related to structure abnormalities of tumor vessels.

  10. Bilateral Multifocal Hamartoma of the Chest Wall in an Infant

    PubMed Central

    Yilmaz, Erdem; Erol, Oguz Bulent; Pekcan, Melih; Gundogdu, Gokcen; Bilgic, Bilge; Gun, Feryal; Yekeler, Ensar

    2015-01-01

    Summary Background Hamartoma of the thoracic wall is a rare benign tumor that occurs in infancy and can be mistaken for a malignancy due to its clinical and imaging features. Hamartomas are extrapleural soft tissue lesions that cause rib expansion and destruction and appear on imaging as cystic areas with fluid levels and calcification. They can cause scoliosis, pressure on the neighboring lung parenchyma and mediastinal displacement. While conservative treatment is recommended in asymptomatic cases, growing lesions require surgical excision. Case Report In this report, we present the imaging findings in a 3-month-old infant that presented with a firm swelling in the chest wall and was histopathologically confirmed to have a bilateral multifocal hamartoma. Conclusions Radiological imaging methods are important for accurate diagnosis of this very rare condition that can be confused with a malignancy. PMID:26082822

  11. Malignant neurocristic hamartoma: a tumor distinct from conventional melanoma and malignant blue nevus.

    PubMed

    Linskey, Katy R; Dias-Santagata, Dora; Nazarian, Rosalynn M; Le, Long P; Lam, Quynh; Bellucci, Kirsten S W; Robinson-Bostom, Leslie; Mihm, Martin C; Hoang, Mai P

    2011-10-01

    Neurocristic hamartomas are rare pigmented lesions comprised of melanocytes, Schwann cells, and pigmented dendritic spindle cells that involve the skin and soft tissue. Malignant transformation can rarely arise within neurocristic hamartomas. Up to date, there has been only 1 series of 7 cases of malignant neurocristic hamartomas (MNHs), with 3 cases that developed metastases. We present the histology and clinical course of 3 additional cases of MNH, 2 of which were metastatic. CD117 was strongly positive in all cases with available archival materials--the tumors and background neurocristic hamartoma of 3 cases, and 1 lymph node metastasis; however, KIT sequencing for exons 11, 13, 17, and 18 was negative. Mutational analyses of recurrent mutations of 17 cancer genes, including BRAF and KIT, were also negative. Although our series is small, KIT overexpression in MNH does not seem to correlate with gene mutation. The lack of BRAF, NRAS, GNAQ, and KIT mutations seems to support the notion that MNH may be distinct from conventional melanoma and from other dermal melanomas, such as malignant blue nevi and melanoma arising in congenital nevi.

  12. Bile duct hamartomas (von Mayenburg complexes) mimicking liver metastases from bile duct cancer: MRC findings

    PubMed Central

    Nagano, Yasuhiko; Matsuo, Kenichi; Gorai, Katsuya; Sugimori, Kazuya; Kunisaki, Chikara; Ike, Hideyuki; Tanaka, Katsuaki; Imada, Toshio; Shimada, Hiroshi

    2006-01-01

    We present a case of a 72-year-old man with a common bile duct cancer, who was initially believed to have multiple liver metastases based on computed tomography findings, and in whom magnetic resonance cholangiography (MRC) revealed a diagnosis of bile duct hamartomas. At exploration for pancreaticoduodenectomy, liver palpation revealed disseminated nodules at the surface of the liver. These nodules showed gray-white nodular lesions of about 0.5 cm in diameter scattered on the surface of both liver lobes, which were looked like multiple liver metastases from bile duct cancer. Frozen section of the liver biopsy disclosed multiple bile ducts with slightly dilated lumens embedded in the collagenous stroma characteristics of multiple bile duct hamartomas (BDHs). Only two reports have described the MRC features of bile duct hamartomas. Of all imaging procedures, MRC provides the most relevant features for the imaging diagnosis of bile duct hamartomas. PMID:16534895

  13. Gingival leiomyomatous hamartoma of the maxilla: a rare entity

    PubMed Central

    Raghunath, Vandana; Manjunatha, Bhari Sharanesha; Al-Thobaiti, Yasser

    2016-01-01

    Hamartoma is a tumour-like malformation appearing as a focal overgrowth of normal cells. Leiomyomatous hamartomas (LHs) are rare in the oral cavity and commonly seen in the Japanese and less than 40 cases have been reported in the Japanese and English literature. The clinical differential diagnoses are irritational (traumatic) fibroma and congenital epulis. It has to be differentiated histopathologically from its neoplastic counterparts and mesenchymomas. Hence, we report such a case of LHs, which presented as a sessile gingival growth occurring in the midline in a 15-year-old girl. The final diagnosis was based on the histopathological appearance which was confirmed by immunohistochemical staining of various markers. A review of the literature of previous cases was also carried out. PMID:27161203

  14. Gingival leiomyomatous hamartoma of the maxilla: a rare entity.

    PubMed

    Raghunath, Vandana; Manjunatha, Bhari Sharanesha; Al-Thobaiti, Yasser

    2016-05-09

    Hamartoma is a tumour-like malformation appearing as a focal overgrowth of normal cells. Leiomyomatous hamartomas (LHs) are rare in the oral cavity and commonly seen in the Japanese and less than 40 cases have been reported in the Japanese and English literature. The clinical differential diagnoses are irritational (traumatic) fibroma and congenital epulis. It has to be differentiated histopathologically from its neoplastic counterparts and mesenchymomas. Hence, we report such a case of LHs, which presented as a sessile gingival growth occurring in the midline in a 15-year-old girl. The final diagnosis was based on the histopathological appearance which was confirmed by immunohistochemical staining of various markers. A review of the literature of previous cases was also carried out. 2016 BMJ Publishing Group Ltd.

  15. Congenital smooth muscle hamartoma of the palpebral conjunctiva.

    PubMed

    Mora, L Evelyn; Rodríguez-Reyes, Abelardo A; Vera, Ana M; Rubio, Rosa Isela; Mayorquín-Ruiz, Mariana; Salcedo, Guillermo

    2012-01-01

    Smooth muscle hamartoma is defined as a disorganized focus or an overgrowth of mature smooth muscle, generally with low capacity of autonomous growth and benign behavior. The implicated tissues are mature and proliferate in a disorganized fashion. A healthy 5-day-old Mexican boy was referred to the authors' hospital in México city for evaluation of a "cystic" lesion of the right eye that had been noted since birth. The pregnancy and delivery were unremarkable. On physical examination, there was a reddish-pink soft lesion with a tender "cystic" appearance, which was probably emerging from the upper eyelid conjunctiva, which measured 2.7 cm in its widest diameter and transilluminated. Ultrasound imaging revealed an anterior "cystic" lesion with normally formed phakic eye. An excisional biopsy was performed, and the lesion was dissected from the upper tarsal subconjunctival space. Subsequent histologic and immunohistochemical findings were consistent with the diagnosis of congenital smooth muscle hamartoma (CSMH) of the tarsal conjunctiva. The authors' research revealed that only one case of CSMH localized in the conjunctiva (Roper GJ, Smith MS, Lueder GT. Congenital smooth muscle hamartoma of the conjunctival fornix. Am J Ophthalmol. 1999;128:643-4) has been reported to date in the literature. To the best of the authors' knowledge, this current case would be the second case reported of CSMH in this anatomic location. Therefore, the authors' recommendation is to include CSMH in the differential diagnosis of a cystic mass that presents in the fornix and palpebral conjunctiva.

  16. Comparison of Color Fundus Photography, Infrared Fundus Photography, and Optical Coherence Tomography in Detecting Retinal Hamartoma in Patients with Tuberous Sclerosis Complex.

    PubMed

    Bai, Da-Yong; Wang, Xu; Zhao, Jun-Yang; Li, Li; Gao, Jun; Wang, Ning-Li

    2016-05-20

    A sensitive method is required to detect retinal hamartomas in patients with tuberous sclerosis complex (TSC). The aim of the present study was to compare the color fundus photography, infrared imaging (IFG), and optical coherence tomography (OCT) in the detection rate of retinal hamartoma in patients with TSC. This study included 11 patients (22 eyes) with TSC, who underwent color fundus photography, IFG, and spectral-domain OCT to detect retinal hamartomas. TSC1 and TSC2RESULTS: The mean age of the 11 patients was 8.0 ± 2.1 years. The mean spherical equivalent was -0.55 ± 1.42 D by autorefraction with cycloplegia. In 11 patients (22 eyes), OCT, infrared fundus photography, and color fundus photography revealed 26, 18, and 9 hamartomas, respectively. The predominant hamartoma was type I (55.6%). All the hamartomas that detected by color fundus photography or IFG can be detected by OCT. Among the methods of color fundus photography, IFG, and OCT, the OCT has higher detection rate for retinal hamartoma in TSC patients; therefore, OCT might be promising for the clinical diagnosis of TSC.

  17. Case of fibrolipomatous hamartoma of the digital nerve without macrodactyly.

    PubMed

    Nanno, Mitsuhiko; Sawaizumi, Takuya; Takai, Shinro

    2011-01-01

    Fibrolipomatous hamartoma of nerves without macrodactyly is a rare lesion characterized by fibrofatty proliferation causing epineural and perineural fibrosis with fatty infiltration around the nerve bundles. We report an unusual case of fibromatous hamartoma of the ulnar digital nerve of the thumb in a 43-year-old woman. Magnetic resonance imaging revealed a large fusiform mass along the nerve. The findings were unusual and pathognomonic and included a coaxial cable-like appearance on axial sections and a spaghettilike appearance on coronal sections on both T1- and T2-weighted images; these findings were useful for the diagnosis and preoperative evaluation of this lesion. Surgical exploration revealed a yellow, cordlike mass of the digital nerve enlarged by fat. Gross excision could not be done without extensive damage to the nerve. Therefore, a limited excision with biopsy of the fibrolipomatous tissue around the nerve bundles was performed. The histological appearance was consistent with fibrolipomatous hamartoma. There was no recurrence of the mass and no neurological deficit 3 years after surgery. Some authors have suggested that invasive excision can cause catastrophic sensory or motor deficits because of the extensive fatty infiltration of the nerve fascicles. In conclusion, the recommended treatment for this lesion is limited excision with only biopsy to confirm the diagnosis.

  18. Surgical treatment in combined hamartoma of the retina and retinal pigment epithelium.

    PubMed

    Sánchez-Vicente, J L; Rueda-Rueda, T; Llerena-Manzorro, L; Molina-Socola, F E; Contreras-Díaz, M; Szewc, M; Vital-Berral, C; Alfaro-Juárez, A; Medina-Tapia, A; López-Herrero, F; González-García, L; Muñoz-Morales, A

    2017-03-01

    The case is presented of a 39 year-old man with a combined hamartoma of the retina and retinal pigment epithelium, who experienced progressive visual loss and worsening of metamorphopsia. The patient underwent vitrectomy and epiretinal component peeling, with improvement in visual acuity, metamorphopsia, and retinal architecture, assessed by optical coherence tomography. Selected patients with combined hamartomas of the retina and retinal pigment epithelium may benefit from surgical management. Copyright © 2016 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.

  19. Comparison of Color Fundus Photography, Infrared Fundus Photography, and Optical Coherence Tomography in Detecting Retinal Hamartoma in Patients with Tuberous Sclerosis Complex

    PubMed Central

    Bai, Da-Yong; Wang, Xu; Zhao, Jun-Yang; Li, Li; Gao, Jun; Wang, Ning-Li

    2016-01-01

    Background: A sensitive method is required to detect retinal hamartomas in patients with tuberous sclerosis complex (TSC). The aim of the present study was to compare the color fundus photography, infrared imaging (IFG), and optical coherence tomography (OCT) in the detection rate of retinal hamartoma in patients with TSC. Methods: This study included 11 patients (22 eyes) with TSC, who underwent color fundus photography, IFG, and spectral-domain OCT to detect retinal hamartomas. TSC1 and TSC2 mutations were tested in eight patients. Results: The mean age of the 11 patients was 8.0 ± 2.1 years. The mean spherical equivalent was −0.55 ± 1.42 D by autorefraction with cycloplegia. In 11 patients (22 eyes), OCT, infrared fundus photography, and color fundus photography revealed 26, 18, and 9 hamartomas, respectively. The predominant hamartoma was type I (55.6%). All the hamartomas that detected by color fundus photography or IFG can be detected by OCT. Conclusion: Among the methods of color fundus photography, IFG, and OCT, the OCT has higher detection rate for retinal hamartoma in TSC patients; therefore, OCT might be promising for the clinical diagnosis of TSC. PMID:27174333

  20. Videothoracoscopic identification of chondromatous hamartoma of the lung

    PubMed Central

    Bohanes, Tomàš; Szkorupa, Marek; Klein, Jiří; Zapletalová, Jana; Chudáček, Josef; Vomáčková, Katherine; Vrba, Radek

    2013-01-01

    Introduction The main disadvantage of a videothoracoscopic procedure is the lack of touch sensation. The probability of easily finding the lesion is usually estimated according to computed tomography (CT). Aim To find useful parameters of location of chondromatous hamartoma of the lung parenchyma in relation to its size to assess the probability of successful search via a videothoracoscopic approach only. Material and methods A group of 55 patients operated on for chondromatous hamartoma of the lung at the First Department of Surgery in Olomouc from January 2006 to June 2011 was analyzed. Initially, the tumor's longest diameter and its nearest distance to the pleural surface were measured on CT scans. Subsequently, the surgery began using the videothoracoscopic approach. A short thoracotomy with direct palpation followed when videothoracoscopy failed. Results No significant differences in age, sex and side of localization between the group with and without successful videothoracoscopic detection were found. A significant difference was found in the median size (p = 0.026) and the depth of the tumor (p < 0.0001) and in the calculated index “tumor size/depth” (p < 0.0001). Deeper analysis revealed that the parameters “depth” and “index size/depth” are considered to be good predictors but the parameter “size” is not a suitable predictor. Conclusions The main predictors of successful videothoracoscopic detection of lung chondromatous hamartoma are considered to be the depth of the tumor in the lung parenchyma with a cut-off value ≤ 7.5 mm and the index “size/depth” with a cut-off value ≥ 1.54; the tumor size is not considered to be a good predictor. PMID:23837099

  1. MRI diagnosis of fibrolipomatous hamartoma of the median nerve and associated macrodystrophia lipomatosa.

    PubMed

    Chiang, Chia-Ling; Tsai, Meng-Yuan; Chen, Clement Kuen-Huang

    2010-09-01

    Fibrolipomatous hamartoma is an uncommon congenital disorder, which is characterized by disproportionate hyperplasia of adipose tissue infiltrating along the perineurium, the epineurium and the affected nerve trajectory. We present a case of combined fibrolipomatous hamartoma and macrodystrophia lipomatosa of the median nerve. The involved sites included the left palm, wrist and forearm. Part of the patient's middle finger had been amputated due to previous macrodystrophia lipomatosa; however, the lesion continued to enlarge and was accompanied by numbness. Magnetic resonance imaging demonstrated a typical fibrolipomatous hamartoma with high signal intensity of fat on both T1-weighted and T2-weighted images, characteristic coaxial cable appearance on axial images, and spaghetti appearance on sagittal images. A similar skipped lesion at the median nerve of the middle forearm was also noted. To the best of our knowledge, this has not been reported in the English literature. Copyright © 2010 Elsevier. Published by Elsevier B.V. All rights reserved.

  2. Hamartomas from patients with tuberous sclerosis show loss of heterozygosity for chromosome 9q34

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

    Green, A.J.; Sepp, T.; Yates, J.R.W.

    1994-09-01

    We have previously shown allele loss in hamartomas from cases of tuberous sclerosis (TSC) for markers in the region of the recently characterized TSC2 gene on chromosome 16p13.3. Germline deletions in the TSC2 gene have been shown in 5% of patients with TSC. These data strongly suggest that the TSC2 gene acts as a tumor suppressor gene. We hypothesised that hamartomas from patients with TSC can also show allele loss for markers on chromosome 9q34 in the region of the TSC1 gene. We studied 7 hamartomas (3 renal angiomyolipomas, 3 giant cell astrocytomas, and a cardiac rhabdomyoma) from 7 casesmore » of TSC, none of which showed allele loss for markers on chromosome 16p13.3. Eight microsatellite markers were analyzed, comprising from centromeric to telomeric, ASS - D9S64 - D9S149 -D9S150 - DBH - D9S66 - D9S114 - D9S67. Two hamartomas (one renal angiomyolipoma and one giant cell astrocytoma) showed allele loss for at least two markers. The region of allele loss involved the TSC1 locus, but did not include D9S149 or D9S67. We have shown allele loss in two of seven TSC hamartomas in the region of the TSC1 gene on 9q34. Based on this deletion mapping, we suggest that the TSC1 gene on 9a34, like the TSC2 gene, acts as a tumor suppressor gene.« less

  3. Gamma Knife radiosurgery for hypothalamic hamartoma preserves endocrine functions.

    PubMed

    Castinetti, Frederic; Brue, Thierry; Morange, Isabelle; Carron, Romain; Régis, Jean

    2017-06-01

    Gamma Knife radiosurgery (GK) is an effective treatment for hypothalamic hamartoma. No precise data are available on the risk of endocrine side effects of this treatment. In this study, 34 patients with hypothalamic hamartoma (HH) were followed prospectively at the Department of Endocrinology, La Timone Hospital, Marseille, France, for a mean follow-up of >2 years (mean ± standard deviation [SD] 3.6 ± 2 years). Initial pre- and post-GK radiosurgery evaluations were performed, including weight, body mass index (BMI), and a complete endocrinological workup. At diagnosis, eight patients presented with central precocious puberty at a mean age of 5.4 ± 2.4 years. At the time of GK (mean age 18.2 ± 11.1 years), two patients previously treated with surgery presented with luteinizing hormone/follicle-stimulating hormone (LH/FSH) deficiency. After GK, only one patient presented with a new thyrotropin-stimulating hormone (TSH) deficiency, 2 years after the procedure. The other pituitary axes remained normal in all but two patients (who had LH/FSH deficiency prior to GK). There was no significant difference between pre- and post-GK mean BMI (26.9 vs. 25.1 kg/m 2 , p = 0.59). To conclude, in this group of 34 patients, GK did not induce major endocrinologic side effects reported with all the other surgical techniques in the literature. It is, thus, a safe and effective procedure in the treatment of hypothalamic hamartoma. Wiley Periodicals, Inc. © 2017 International League Against Epilepsy.

  4. Peripapillar retinal hamartoma associated with tuberous sclerosis. Case report.

    PubMed

    Hernández Pardines, F; Núñez Márquez, S; Fernández Montalvo, L; Serra Verdú, M C; Juárez Marroquí, A

    2018-03-01

    Tuberous sclerosis is a rare multisystemic disease with an autosomal dominant inheritance pattern. There are few documented cases in the literature of retinal hamartomas (astrocytomas) with aggressive progression in the context of this disease. A report is presented on a case of a 31 year-old male with unknown history of ophthalmic or systemic conditions, who referred to a history of 6 months of blurred vision in his right eye. This was caused by a unilateral retinal hamartoma due to an undiagnosed tuberous sclerosis. Multidisciplinary management, with the cooperation of Internal Medicine and the Oncology Department, is needed in these cases, as well as genetic counselling for affected patients. Complications are directly related to increased tumour size. Treatment does not seem to have any influence on the natural history of the disease. Copyright © 2017 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.

  5. Folliculosebaceous cystic hamartoma with a neural component: an immunohistochemical study.

    PubMed

    Toyoda, M; Morohashi, M

    1997-07-01

    We present a case of follicular cystic hamartoma, a distinctive cutaneous malformation characterized by marked overgrowth of folliculosebaceous units accompanied by appreciable mesenchymal alterations, including fibroplasia, increased vascular components, and numerous adipocytes. A conspicuous feature of our case is an aggregation of thick trespassing nerve bundles in the deep portion of the neoplasm. An immunohistochemical study revealed the nerve bundles were immunoreactive for the general neuronal marker protein gene product 9.5. The nerves, however, stained negatively with antibodies against neuropeptides such as calcitonin gene-related peptide, substance P, vasoactive intestinal polypeptide, and neuropeptide Y, all of which are known to be contained in normal cutaneous nerves. The presence of these aberrant nerve bundles devoid of the normal expression of neuropeptides supports the concept that folliculosebaceous cystic hamartoma is a true, tumor-like malformation characterized by abnormal overgrowth of normal components of the skin.

  6. Retinoic acid-induced lumbosacral neural tube defects: myeloschisis and hamartoma.

    PubMed

    Cai, WeiSong; Zhao, HongYu; Guo, JunBin; Li, Yong; Yuan, ZhengWei; Wang, WeiLin

    2007-05-01

    To observe the morphological features of the lumbosacral neural tube defects (NTDs) induced by all-trans retinoic acid (atRA) and to explore the pathogenesis of these defects. Rat embryos with lumbosacral NTDs were obtained by treating pregnant rats with administration of atRA. Rat embryos were obtained by cesarean. Fetuses were sectioned and stained with hematoxylin-eosin (H&E). Relevant structures including caudal neural tube were examined. In the atRA-treated rats, about 48% embryos showed lumbosacral NTDs. There appeared a dorsally and rostrally situated, neural-plate-like structure (myeloschisis) and a ventrally and caudally located cell mass containing multiple canals (hamartoma) in the lumbosacral NTDs induced by atRA. Retinoic acid could disturb the notochord and tail bud development in the process of primary and secondary neurulation in rat embryos, which cause lumbosacral NTDs including myeloschisis and hamartoma. The morphology is very similar to that happens in humans.

  7. Syndrome of fetal gigantism, renal hamartomas, and nephroblastomatosis with Wilms' tumor.

    PubMed

    Perlman, M; Levin, M; Wittels, B

    1975-04-01

    A new case of the fetal gigantism-renal hamartomas-nephroblastomatosis syndrome is described, in which a Wilms' tumor occurred. It is considered that this observation provides strong evidence for the interrelationship between renal dysplasia and renal neoplasia.

  8. Congenital Eccrine Angiomatous Hamartoma: A Rare Entity Revisited

    PubMed Central

    Mendiratta, Vibhu; Malik, Meenu; Agrawal, Mahima; Jain, Manjula; Gupta, Brijnandan

    2018-01-01

    Eccrine angiomatous hamartoma (EAH) is a rare benign malformation characterized by eccrine and vascular components. It usually presents at birth or during early infancy or childhood on lower extremities as a nodule or plaque. We report a case of asymptomatic tumoral swelling over the left leg in a 5-month-old Indian infant since birth. This was diagnosed as EAH on histopathology and confirmed on immunohistochemistry. PMID:29854641

  9. Hybrid eccrine gland and hair follicle hamartoma: a new entity of adnexal nevus.

    PubMed

    Luo, Di-Qing; Huang, Chang-Zheng; Xie, Wen-Lin; Xu, Feng-Feng; Mo, Li-Qiu

    2015-02-01

    Eccrine nevus shows increase in number or size of eccrine glands, whereas hair follicle nevus is composed of densely packed normal vellus hairs, and eccrine-pilar angiomatous nevus reveals increase of eccrine, pilar, and angiomatous structures. No case with increased number of both eccrine glands and hair follicles only in the dermis has been previously reported. A 10-month-old girl presented with cutaneous hamartoma with overlying skin hyperpigmentation on her left hypochondrium since 3 months of age, in whom the lesion was completely excised. Histopathology demonstrated evidently increased number of both eccrine glands and hair follicles in the dermis with reactive hyperplasia of collagen fibers. No recurrence occurred after the tumor was completely excised. A term "hybrid eccrine gland and hair follicle hamartoma" is proposed for this unique lesion.

  10. Nasal Chondromesenchymal Hamartoma in a Child

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

    Finitsis, Stefanos; Giavroglou, Constantinos; Potsi, Stamatia, E-mail: matinapotsi@hotmail.co

    Nasal chondromesenchymal hamartoma (NCMH) is a benign tumor that was described in 1998. The occurrence of this lesion in the nasal cavity of infants and children is especially rare, with only 21 cases reported in the international literature. We report a 12-month-old boy with respiratory distress due to nasal obstruction. Computed tomographic scan and magnetic resonance imaging examination demonstrated a soft-tissue mass obstructing the left nasal cavity. Digital subtraction angiography and preoperative superselective embolization with microparticles were also performed. The tumor was completely resected surgically. Histopathology and immunohistochemical analyses of the tumor disclosed a NCMH. The imaging characteristics of themore » tumor are described and the radiology literature is reviewed.« less

  11. Lipofibromatous Hamartoma of the Plantar Nerve An Extremely Rare Localization.

    PubMed

    Mert, Murat; Hacısalihoglu, Payam

    2018-03-01

    Lipofibromatous hamartoma (LFH) is a rare, benign, tumor-like soft-tissue lesion that affects the peripheral nerves and forms a palpable neurogenic mass. Lipofibromatous hamartoma is associated with pain and sensory and/or motor deficits in the area of innervation of the affected nerve. This report describes a rare case of LFH of the plantar nerve. A 48-year-old woman presented to our outpatient orthopedic clinic with pain and a burning sensation on her left foot. The patient had a history of Morton's neuroma and had undergone a tarsal tunnel operation 2 years earlier at another center. None of her symptoms was alleviated by two previous operations. Magnetic resonance imaging with contrast revealed tenosynovitis of the flexor hallucis longus tendon and signal changes at deep tissue planes of the foot at the levels of the second and third toes, on the dorsal site and subcutaneous soft-tissue planes, suggesting edema and Morton's neuroma. The lesion was excised under spinal anesthesia, and histopathologic examination of the specimen revealed a diagnosis of LFH. The patient was discharged without any symptoms and her foot was normal at 8-month outpatient follow-up, with no indications of postoperative complications and/or recurrence.

  12. Aspiration cytology of mesenchymal hamartoma of the chest wall: a case report and literature review.

    PubMed

    Taweevisit, Mana; Trinavarat, Panruethai; Thorner, Paul Scott

    2014-10-01

    Mesenchymal hamartoma of the chest wall is a rare tumor-like lesion of infancy and childhood. The few available descriptions of the findings on fine needle aspiration list spindle-shaped cells and cartilage or chondromyxoid material as essential features for this diagnosis. An aggressive appearance on imaging studies and a lack of familiarity with this lesion, can lead the pathologist to misdiagnose the cytologic findings as malignancy. We reported a 5-month-old male presenting with a mass of the right chest wall progressively for 2 months. Radiologic studies showed a mixed solid and cystic mass originating from the third, fourth and fifth ribs, and a diagnosis of malignancy was favored. Fine needle aspiration recovered only spindle-shaped cells and a few multinucleated giant cells of osteoclast type. After a review of the imaging, a diagnosis of mesenchymal hamartoma of the chest wall was raised. This diagnosis was confirmed by pathologic examination of the subsequently resected mass. This is the sixth report of a mesenchymal hamartoma of the chest wall diagnosed by fine needle aspiration. This case illustrates that this diagnosis can be suspected in the absence of cartilage or chondromyxoid material, given appropriate clinical and radiologic findings. © 2014 Wiley Periodicals, Inc.

  13. Surgical management of giant Brunner's gland hamartoma: case report and literature review

    PubMed Central

    Stewart, Zoe A; Hruban, Ralph H; Fishman, Elliot F; Wolfgang, Christopher L

    2009-01-01

    Brunner's gland hamartomas (BGH) are uncommon benign tumors of the duodenum forming mature Brunner's glands. We report here an unusual case of a giant BGH that was not amenable to endoscopic or surgical local resection thus requiring a pancreaticoduodenectomy for extirpation. The relevant literature is discussed. PMID:19725968

  14. Amelogenesis imperfecta associated with dental follicular-like hamartomas and generalised gingival enlargement.

    PubMed

    O'Connell, S; Davies, J; Smallridge, J; Vaidyanathan, M

    2014-10-01

    Amelogenesis imperfecta (AI) is an inherited disorder characterised by generalised defects of dental enamel, but has been associated with other dental and medical conditions. It affects the appearance and structure of teeth, both in the primary and secondary dentition. AI in the presence of dental follicular hamartomas and gingival hyperplasia is rare and the management presents several challenges to the clinician. This article describes a case of a girl who presented to the paediatric department at the age of 7 years complaining of discomfort when eating and that she was unhappy with the appearance of her anterior teeth. The patient was born in the UK but she and her family were African and of Kenyan origin. She was otherwise fit and well. Investigations included clinical, radiographic and pathological examination as well as cone beam computed tomography imaging and X-ray Microtomography of extracted primary teeth. A diagnosis of AI in the presence of dental follicular hamartomas and generalised gingival hyperplasia was made, which had resulted in the delayed eruption of permanent teeth and an associated anterior open bite. There was no family history of dental defects. Initial treatment included preventative advice and the application of preformed metal crowns on all primary molars. Extraction of all remaining primary incisors was carried out followed by gingivectomy around the maxillary permanent incisors, mandibular central incisors and maxillary left second primary molar. Composite resin reconstruction of all permanent incisors and mandibular primary canines was complicated by the poor quality of enamel. Orthodontic extrusion of the anterior incisors was carried out to improve surface area for bonding with some success. A multidisciplinary team managed this case and decided that no surgical intervention of the dental follicular hamartomas was warranted. The patient coped well with treatment and attended for regular review over an 8-year period. She was

  15. Multifocal demyelinating motor neuropathy and hamartoma syndrome associated with a de novo PTEN mutation

    PubMed Central

    Bansagi, Boglarka; Phan, Vietxuan; Baker, Mark R.; O'Sullivan, Julia; Jennings, Matthew J.; Whittaker, Roger G.; Müller, Juliane S.; Duff, Jennifer; Griffin, Helen; Miller, James A.L.; Gorman, Grainne S.; Lochmüller, Hanns; Chinnery, Patrick F.; Roos, Andreas; Swan, Laura E.

    2018-01-01

    Objective To describe a patient with a multifocal demyelinating motor neuropathy with onset in childhood and a mutation in phosphatase and tensin homolog (PTEN), a tumor suppressor gene associated with inherited tumor susceptibility conditions, macrocephaly, autism, ataxia, tremor, and epilepsy. Functional implications of this protein have been investigated in Parkinson and Alzheimer diseases. Methods We performed whole-exome sequencing in the patient's genomic DNA validated by Sanger sequencing. Immunoblotting, in vitro enzymatic assay, and label-free shotgun proteomic profiling were performed in the patient's fibroblasts. Results The predominant clinical presentation of the patient was a childhood onset, asymmetric progressive multifocal motor neuropathy. In addition, he presented with macrocephaly, autism spectrum disorder, and skin hamartomas, considered as clinical criteria for PTEN-related hamartoma tumor syndrome. Extensive tumor screening did not detect any malignancies. We detected a novel de novo heterozygous c.269T>C, p.(Phe90Ser) PTEN variant, which was absent in both parents. The pathogenicity of the variant is supported by altered expression of several PTEN-associated proteins involved in tumorigenesis. Moreover, fibroblasts showed a defect in catalytic activity of PTEN against the secondary substrate, phosphatidylinositol 3,4-trisphosphate. In support of our findings, focal hypermyelination leading to peripheral neuropathy has been reported in PTEN-deficient mice. Conclusion We describe a novel phenotype, PTEN-associated multifocal demyelinating motor neuropathy with a skin hamartoma syndrome. A similar mechanism may potentially underlie other forms of Charcot-Marie-Tooth disease with involvement of the phosphatidylinositol pathway. PMID:29720545

  16. Hypothalamic hamartoma: Epileptogenesis beyond the lesion?

    PubMed

    Scholly, Julia; Staack, Anke Maren; Kahane, Philippe; Scavarda, Didier; Régis, Jean; Hirsch, Edouard; Bartolomei, Fabrice

    2017-06-01

    The discovery of intrinsic epileptogenicity of the hypothalamic hamartoma (HH) marked a new area in understanding the associated clinical syndrome, often manifesting as progressive epileptic encephalopathy. However, therapeutic procedures targeting the HH proved to be inefficient to cure seizures in up to 50% of cases, whereas in cases with partial improvement, the electroclinical patterns of persisting seizures suggest an involvement of distant cortical regions. The concept of kindling-like secondary epileptogenesis has been suggested as a possible underlying mechanism. Yet the role of the hypothalamic lesion in the pathophysiology of the syndrome remains debatable. In the Strasbourg-Kork series, the best outcomes were obtained when the duration of epilepsy before endoscopic HH surgery did not exceed 10 years. In two patients with HH ablation followed at a later time by a temporal lobectomy, only this second surgical step allowed complete seizure freedom. These findings suggest the existence of an independent, third stage of secondary epileptogenesis in human. In the Grenoble series, stereotactic intracerebral recordings (stereo electroencephalography [SEEG]) of five HH cases demonstrated that gelastic/dacrystic seizures were correlated with discharges within the HH, whereas other seizure types were related to discharges affecting cortical regions, which sometimes seemed to be triggered by HH. In the Marseille series, two cases explored by SEEG provided evidence of extended epileptogenicity outside the limits of the HH, forming complex epileptogenic networks, with HH still triggering clusters of neocortical seizures in the first, but not obligatory involved in spontaneous seizures in the second case. Taken together, our data argue for the existence of dynamic ictal network organization, with possible "kindling-like" relationships between the HH and the neocortex or widespread epileptogenesis. Despite the existence of secondary epileptogenesis, the epileptogenic

  17. Multifocal demyelinating motor neuropathy and hamartoma syndrome associated with a de novo PTEN mutation.

    PubMed

    Bansagi, Boglarka; Phan, Vietxuan; Baker, Mark R; O'Sullivan, Julia; Jennings, Matthew J; Whittaker, Roger G; Müller, Juliane S; Duff, Jennifer; Griffin, Helen; Miller, James A L; Gorman, Grainne S; Lochmüller, Hanns; Chinnery, Patrick F; Roos, Andreas; Swan, Laura E; Horvath, Rita

    2018-05-22

    To describe a patient with a multifocal demyelinating motor neuropathy with onset in childhood and a mutation in phosphatase and tensin homolog ( PTEN ), a tumor suppressor gene associated with inherited tumor susceptibility conditions, macrocephaly, autism, ataxia, tremor, and epilepsy. Functional implications of this protein have been investigated in Parkinson and Alzheimer diseases. We performed whole-exome sequencing in the patient's genomic DNA validated by Sanger sequencing. Immunoblotting, in vitro enzymatic assay, and label-free shotgun proteomic profiling were performed in the patient's fibroblasts. The predominant clinical presentation of the patient was a childhood onset, asymmetric progressive multifocal motor neuropathy. In addition, he presented with macrocephaly, autism spectrum disorder, and skin hamartomas, considered as clinical criteria for PTEN-related hamartoma tumor syndrome. Extensive tumor screening did not detect any malignancies. We detected a novel de novo heterozygous c.269T>C, p.(Phe90Ser) PTEN variant, which was absent in both parents. The pathogenicity of the variant is supported by altered expression of several PTEN-associated proteins involved in tumorigenesis. Moreover, fibroblasts showed a defect in catalytic activity of PTEN against the secondary substrate, phosphatidylinositol 3,4-trisphosphate. In support of our findings, focal hypermyelination leading to peripheral neuropathy has been reported in PTEN-deficient mice. We describe a novel phenotype, PTEN-associated multifocal demyelinating motor neuropathy with a skin hamartoma syndrome. A similar mechanism may potentially underlie other forms of Charcot-Marie-Tooth disease with involvement of the phosphatidylinositol pathway. Copyright © 2018 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.

  18. Fibrolipomatous Hamartoma of the Median Nerve with Macrodystrophia Lipomatosa.

    PubMed

    Azeemuddin, Muhammad; Waheed, Adeel A; Khan, Noman; Sayani, Raza; Ahmed, Anwar

    2018-03-09

    Fibrolipomatous hamartoma (FLH) is a rare congenital condition that presents with a benign overgrowth of the bone and fibroadipose tissue termed as macrodystrophia lipomatosa (MDL). Although commonly seen in the median nerve, other peripheral nerves can be involved. Diagnosis can be made on magnetic resonance imaging (MRI) due to the characteristic coaxial cable appearance on axial images and the spaghetti appearance on sagittal images. Histology shows mature adipose and fibrous tissue infiltrating the epineural and perineural compartments. Multiple or debulking surgeries are often needed, with an emphasis on cosmetic aspects. We present one such case in which wide margin excision and sural nerve graft were carried out.

  19. Basaloid Follicular Hamartoma

    PubMed Central

    Cooper, Hassie; Hogan, Daniel J.; Miller, Richard; Heaphy, Michael; Spencer, James

    2018-01-01

    Introduction: Basaloid follicular hamartoma (BFH) is a rare, benign neoplasm of the hair follicle, characterized by multiple brown papules involving the face, scalp, and trunk. It is described by multiple clinical forms, and can present as localized or generalized. Diagnosis is made histologically via biopsy, which is important in order to distinguish BFH from basal cell carcinoma (BCC) or other malignant epithelial neoplasms. Correct diagnosis allows for the avoidance of unnecessary surgeries to remove benign lesions. While benign, lesions can be cosmetically unacceptable. Case Report: A 68-year-old man with a two-year history of brown, homogenous papules on his face presented to discuss treatment options. A physical examination revealed hundreds of dark brown, 1- to 3mm verrucous papules distributed throughout the face. Two punch biopsies revealed histologic features consistent with BFH. Discussion: BFHs classically present with multiple 1- to 2mm tan-to-brown-colored papules distributed on the face, scalp, neck, axilla, trunk, and pubic area. Differential diagnoses can include nevus sebaceous, lichen striatus, linear epidermal nevus, and basal cell nevus. BFH arises from a mutation in the patch gene, the same gene thought to cause nevoid BCC syndrome. Histologic examination of BFH lesions is essential to diagnosis. No standard of care exists for BFH; treatment options remain limited. This patient was treated with three rounds of pulsed dye laser (PDL) therapy and showed marked improvement in the treated areas. The authors propose PDL to be a safe, effective, and novel cosmetic treatment for BFH and potentially other adnexal tumors. PMID:29607000

  20. Evolution of an Astrocytic Hamartoma of the Optic Nerve Head in a Patient with Retinitis Pigmentosa - Photographic Documentation over 2 Years of Follow-Up.

    PubMed

    Loukianou, Eleni; Kisma, Nacima; Pal, Bishwanathan

    2011-02-02

    To report photographically the evolution of an astrocytic hamartoma of the left optic nerve head over a 2-year follow-up in a patient with retinitis pigmentosa. A 14-year-old boy was seen in the medical retina clinic with a 3-year history of night blindness. Best corrected visual acuity was 6/18 in both eyes. Colour vision was normal in both eyes and confrontation fields showed peripheral constriction. Fundus examination revealed bone spicule pigmentary changes at the retinal mid periphery typical of retinitis pigmentosa and superficial globules at the margins of both optic nerve heads. Electrodiagnostic tests confirmed moderately severe rod cone dystrophy with macular involvement bilaterally. Two years later, the ocular examination was unchanged except for the appearance of the optic nerve head lesion in the left eye. There was an increase in the size of the lesion which was diagnosed as an astrocytic hamartoma. Further investigations were recommended to exclude neurofibromatosis and tuberous sclerosis. Astrocytic hamartomas of the optic nerve head and optic nerve head drusen have both been described in patients with retinitis pigmentosa. They can be a diagnostic dilemma although drusen are more common (10%). To differentiate these two entities it is very important to document any growth during the follow-up period which is suggestive of astrocytic hamartoma rather than optic disc drusen.

  1. Hypothalamic hamartoma with precocious puberty: a case report.

    PubMed

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

    2012-07-01

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

  2. Respiratory epithelial adenomatoid hamartoma in a dog.

    PubMed

    Leroith, Tanya; Binder, Ellen M; Graham, A Heather; Duncan, Robert B

    2009-11-01

    A 6-month-old, intact, male Weimaraner dog presented to the veterinary teaching hospital for bilateral mucopurulent ocular and nasal discharge that began at approximately 10 weeks of age. A computed tomography scan showed an expansile soft-tissue mass involving both frontal sinuses, the ethmoid regions, and nasal cavities with lysis of the maxillary turbinates and hyperostosis of the walls of the frontal sinus. The dog was euthanized after complications during a trephination and biopsy procedure. At necropsy, a large, tan, papillary, gelatinous mass filled the entire nasal cavity and frontal sinus. The mass was composed of large fronds of loose fibrovascular stroma covered by a single layer of pseudostratified, columnar, ciliated epithelium and intermixed goblet cells. The cells occasionally formed glandular structures that were continuous with the surface epithelium. The mass was diagnosed as a respiratory epithelial adenomatoid hamartoma based on the morphologic appearance.

  3. Evolution of an Astrocytic Hamartoma of the Optic Nerve Head in a Patient with Retinitis Pigmentosa – Photographic Documentation over 2 Years of Follow-Up

    PubMed Central

    Loukianou, Eleni; Kisma, Nacima; Pal, Bishwanathan

    2011-01-01

    Aim To report photographically the evolution of an astrocytic hamartoma of the left optic nerve head over a 2-year follow-up in a patient with retinitis pigmentosa. Methods A 14-year-old boy was seen in the medical retina clinic with a 3-year history of night blindness. Best corrected visual acuity was 6/18 in both eyes. Colour vision was normal in both eyes and confrontation fields showed peripheral constriction. Fundus examination revealed bone spicule pigmentary changes at the retinal mid periphery typical of retinitis pigmentosa and superficial globules at the margins of both optic nerve heads. Electrodiagnostic tests confirmed moderately severe rod cone dystrophy with macular involvement bilaterally. Results Two years later, the ocular examination was unchanged except for the appearance of the optic nerve head lesion in the left eye. There was an increase in the size of the lesion which was diagnosed as an astrocytic hamartoma. Further investigations were recommended to exclude neurofibromatosis and tuberous sclerosis. Conclusion Astrocytic hamartomas of the optic nerve head and optic nerve head drusen have both been described in patients with retinitis pigmentosa. They can be a diagnostic dilemma although drusen are more common (10%). To differentiate these two entities it is very important to document any growth during the follow-up period which is suggestive of astrocytic hamartoma rather than optic disc drusen. PMID:21347192

  4. Generalized smooth muscle hamartoma with multiple congenital anomalies without the "Michelin tire baby" phenotype.

    PubMed

    Janicke, Elise C; Nazareth, Michael R; Rothman, Ilene L

    2014-01-01

    We report a patient with generalized smooth muscle hamartoma who presented with many of the variety of congenital anomalies that have been reported in babies with multiple symmetric circumferential rings of folded skin known as Michelin tire baby (MTB) syndrome, but our patient did not show the MTB phenotype. This constellation of findings in the absence of the MTB phenotype has not been previously reported. © 2014 Wiley Periodicals, Inc.

  5. Juvenile X-linked retinoschisis presenting as juxtapapillary retinal fold mimicking combined hamartoma of the retina and retinal pigment epithelium.

    PubMed

    Pointdujour-Lim, Renelle; Say, Emil Anthony T; Shields, Carol L

    2017-04-01

    A 21-month-old boy presumptively diagnosed with combined hamartoma of the retina and retinal pigment epithelium was found to have juvenile X-linked retinoschisis with vitreomacular traction and prominent retinal folding. Copyright © 2017 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.

  6. [Mucosal Schwann cells hamartoma: Review of a recently described entity].

    PubMed

    García-Molina, Francisco; Ruíz-Macia, José Antonio; Sola, Joaquin

    Neural lesions of the colon may be masses (schwannomas and neurofibromas) or, more frequently, small polyps including perineuromas, ganglioneuromas and granular cell tumors. Some neural lesions are associated with congenital syndromes (neurofibromatosis-1, multiple endocrine neoplasia-2B). Recently, a new entity has been described named mucosal Schwann cell hamartoma, consisting of an intramucosal neural proliferation; to date, less than forty cases have been reported. We report a further case in a patient from whom a polyp was extirpated during colonoscopy screening. Histologically, the polyp showed a lamina propia that contained spindle-shaped cells of neural aspect which could only be identified after a histochemical and immunohistochemical study. Copyright © 2017 Sociedad Española de Anatomía Patológica. Publicado por Elsevier España, S.L.U. All rights reserved.

  7. Canine mesenchymal hamartoma of the eyelid.

    PubMed

    Kafarnik, Christiane; Calvarese, Sara; Dubielzig, Richard R

    2010-03-01

    Characterization of a benign disease syndrome involving the eyelids of dogs, describing the signalment, clinical appearance, anatomic location, and pathologic features. The records and submitted tissue of 10 dogs with mesenchymal hamartomatous lesions of the periocular connective tissues were retrieved from the Comparative Ocular Pathology Laboratory of Wisconsin (COPLOW) database. The submitted tissue in each case was stained with hematoxilyn & eosin and Masson's Trichrome stain and examined histopathologically. Clinical information was collected from the submission requests. The clinical history, treatment, and follow-up are described in more detail for one of the 10 dogs. Seven different breeds, including four Golden Retrievers, were represented. The ages of affected dogs ranged from 6 to 11 years. Eight of 10 lesions were located at the temporal canthus, ranging in diameter from 0.6 to 3 cm. Clinically, the masses were subcutaneous, firm, lobular soft-tissue growths, which were in some cases adherent to the underlying orbital rim, and in others, freely palpable between the skin and conjunctiva of the eyelid. Histologically, all had distinct margins but were not encapsulated and contained normal appearing collagen-rich connective tissue with some adipose tissue. Five also contained fully differentiated skeletal muscle tissue arranged in poorly formed aggregates or as individual muscle fibers. Mesenchymal hamartoma of the eyelid has not been previously described. The mass has a predisposition to occur at the temporal canthus and should be included in the list of differential diagnoses of benign eyelid masses in dogs.

  8. Rhabdomyomatous mesenchymal hamartoma presenting as a skin tag in the sternoclavicular area.

    PubMed

    Solis-Coria, Araceli; Vargas-González, Roberto; Sotelo-Avila, Cirilo

    2007-01-01

    Rhabdomyomatous mesenchymal hamartoma (RMH) is a rare congenital lesion of the dermis and subdermis. It has been described predominantly in newborns, with 30 cases reported in the English literature. Typically, it appears as a skin tag, papule, nodule or a mass involving the face or sternal notch. A 28-day-old girl presented with a 1.4 x 0.8 cm soft skin tag in the right sternoclavicular area. Physical examination revealed no congenital anomalies. A shaved biopsy showed that the core of the lesion contained striated muscle fibers mixed with hair follicles and sebaceous and eccrine glands. Thin epidermis lined the outside of the tag. We report a patient with a RMH in a site not previously reported and discuss the differential diagnosis.

  9. Are the methylenetetrahydrofolate reductase 1298 and 677 gene polymorphisms related to optic glioma and hamartoma risk in neurofibromatosis type 1 patients?

    PubMed

    Tanyıldız, Hikmet Gülşah; Yeşil, Şule; Bozkurt, Ceyhun; Çandır, Mehmet Onur; Akpınar-Tekgündüz, Sibel; Toprak, Şule; Yüksel, Deniz; Şahin, Gürses

    2016-01-01

    The methylenetetrahydrofolate reductase (MTHFR) gene plays a key role in carcinogenesis through its effects on DNA synthesis and methylation and also has a significant role in the etiology of many disorders, such as diabetes, migraine, and cardiovascular disease. Neurofibromatoses (NF) are autosomal dominant inherited diseases that can affect tissues such as bone and skin and predispose individuals to tumor development in various parts of the nervous system or body. Optic nerve glioma and brain tumors are common in children with NF, and leukemia and lymphoma incidence is also higher than normal. We therefore aimed to investigate the possible relationship between the MTHFR gene polymorphism and accompanying tumors such as neurofibroma, hamartoma, and optic glioma in children with NF1 found to have the MTHFR 677 and MTHFR 1298 gene polymorphism in this study. We included 55 pediatric patients diagnosed with NF1 between 2005 and 2014 in the study group. The control group included 44 healthy subjects without acute or chronic disease findings. A significant relationship was found between the MTHFR A1298C polymorphism and the incidence of optic glioma (p=0.014) (AA vs. AC: OR 11, 95% CI 1.27-95.17; AA vs. CC: OR 7.33, 95% CI 0.35-150.70). We also found a significant relationship between the MTHFR C1298C polymorphism and the incidence of hamartoma (p=0.019) (AA vs. AC: OR 2.12, 95% CI 0.662-6.809; p=0.203). Epilepsy incidence was high in subjects with MTHFR C677C. The MTHFR A1298C, C1298C, and C677C gene polymorphisms can be associated with a higher optic glioma, hamartoma, and epilepsy incidence, respectively, in patients diagnosed with neurofibromatosis type 1.

  10. Successful treatment of hyperphagia by resection of a hypothalamic hamartoma.

    PubMed

    Esquenazi, Yoshua; Sandberg, David I; Rekate, Harold L

    2013-06-01

    Hypothalamic hamartomas (HHs) are benign lesions that are often associated with central precocious puberty and may present with gelastic seizures. Treatment modalities for HH include medical therapy with long-term gonadotropin-releasing hormone analogs or resection. The authors report the case of a 7-year-old girl who was diagnosed with an HH due to precocious puberty and was treated medically with a gonadotropin-releasing hormone analog for 3 years. Despite normalization of her plasma levels of luteinizing hormone, follicle-stimulating hormone, and estradiol and arrest of her precocious puberty, the patient developed progressive weight gain associated with extreme hyperphagia and morbid obesity by the age of 10 years. Her compulsive eating patterns were refractory to counseling and other interventions attempted by her parents and physicians. After resection of the HH, her hyperphagia resolved and her weight stabilized. To the authors' knowledge, this is the first report describing resection of an HH for the purpose of treating hyperphagia and obesity.

  11. The microbiome in PTEN hamartoma tumor syndrome.

    PubMed

    Byrd, Victoria; Getz, Ted; Padmanabhan, Roshan; Arora, Hans; Eng, Charis

    2018-03-01

    Germline PTEN mutations defining PTEN hamartoma tumor syndrome (PHTS) confer heritable predisposition to breast, endometrial, thyroid and other cancers with known age-related risks, but it remains impossible to predict if any individual will develop cancer. In the general population, gut microbial dysbiosis has been linked to cancer, yet is unclear whether these are associated in PHTS patients. In this pilot study, we aimed to characterize microbial composition of stool, urine, and oral wash from 32 PTEN mutation-positive individuals using 16S rRNA gene sequencing. PCoA revealed clustering of the fecal microbiome by cancer history ( P  = 0.03, R 2  = 0.04). Fecal samples from PHTS cancer patients had relatively more abundant operational taxonomic units (OTUs) from family Rikenellaceae and unclassified members of Clostridia compared to those from non-cancer patients, whereas families Peptostreptococcaceae, Enterobacteriaceae, and Bifidobacteriaceae represented relatively more abundant OTUs among fecal samples from PHTS non-cancer patients. Functional metagenomic prediction revealed enrichment of the folate biosynthesis, genetic information processing and cell growth and death pathways among fecal samples from PHTS cancer patients compared to non-cancer patients. We found no major shifts in overall diversity and no clustering by cancer history among oral wash or urine samples. Our observations suggest the utility of an expanded study to interrogate gut dysbiosis as a potential cancer risk modifier in PHTS patients. © 2018 The authors.

  12. Oral-facial-digital syndrome type 1 with hypothalamic hamartoma and Dandy-Walker malformation.

    PubMed

    Azukizawa, Takayuki; Yamamoto, Masahito; Narumiya, Seirou; Takano, Tomoyuki

    2013-04-01

    We report a 1-year-old girl with oral-facial-digital syndrome type 1 with multiple malformations of the oral cavity, face, digits, and central nervous system, including agenesis of the corpus callosum, the presence of intracerebral cysts, and agenesis of the cerebellar vermis, which is associated with the subarachnoid space separating the medial sides of the cerebellar hemispheres. This child also had a hypothalamic hamartoma and a Dandy-Walker malformation, which have not been reported previously. The clinical features, including cerebral malformations, in several types of oral-facial-digital syndrome, overlap with each other. Further accumulation of new case reports and identification of new genetic mutations in oral-facial-digital syndrome may provide novel and important insights into the genetic mechanisms of this syndrome. Copyright © 2013 Elsevier Inc. All rights reserved.

  13. Congenital Hypothalamic "Hamartoblastoma" Versus "Hamartoma": Suggestions for Neuropathologic Terminology Emanating From a Mid-gestational Autopsy Case of Pallister-Hall Syndrome.

    PubMed

    Dunham, C; McFadden, D; Dahlgren, L; Butler, B; Hamilton, S; McKinnon, M

    2018-01-01

    Pallister-Hall syndrome (PHS) is a rare malformative disorder that is due to truncating functional repressor mutations in GLI3. Since the seminal publication in 1980, hypothalamic tumors have been recognized to be a cardinal feature of PHS. In their original description of the neuropathologic features of PHS, Clarren et al. coined the term "hamartoblastoma" to characterize what they deemed to be a dual malformative and neoplastic mass of the hypothalamus. In subsequent published cases/series of PHS, the term "hamartoma" was often substituted for hamartoblastoma given what appeared to be a benign natural history of this lesion. Additional confusion in the literature has ensued since most hypothalamic hamartomas (HH) encountered on the clinical neuropathology service are "isolated" in nature (ie, no other congenital malformations) and present in a very different and stereotypical fashion with gelastic seizures and/or precocious puberty. While genomic investigations of isolated HH have begun to uncover a mutational profile of these cases, GLI3 mutations have only been recognized in a small subset of isolated HH. Herein, we describe the autopsy findings from a 21-week gestational age fetus with features of PHS. Moreover, we provide a detailed description of the hypothalamic tumor affecting this fetus and propose a novel subclassification of HH, distinguishing syndromic from isolated forms based upon the presence or absence of neocortical-like areas.

  14. PTCH1 Germline Mutations and the Basaloid Follicular Hamartoma Values in the Tumor Spectrum of Basal Cell Carcinoma Syndrome (NBCCS).

    PubMed

    Ponti, Giovanni; Manfredini, Marco; Pastorino, Lorenza; Maccaferri, Monia; Tomasi, Aldo; Pellacani, Giovanni

    2018-01-01

    Nevoid basal cell carcinoma syndrome (NBCCS) is an autosomal dominantly inherited disorder characterized by multiple basal cell carcinomas (BCC), odontogenic tumors and various skeletal anomalies. Basaloid follicular hamartomas (BFHs) constitute rare neoplasms that can be detected in sporadic and familial settings as in the Basaloid Follicular Hamartoma Syndrome (BFHS). Although BFHS shares clinical, histopathological and genetic overlapping with the NBCCS, they are still considered two distinctive entities. The aim of our single-institution study was the analysis of a cohort of PTCH1-mutated patients in order to define clinical and biomolecular relationship between NBCCS and BFHs. In our study we evaluated PTCH1 gene-carrier probands affected by NBCCS to detect the incidence of BFHs and their correlation with this rare syndrome. Among probands we recognized 4 patients with BFHs. We found 15 germline PTCH1 mutations, uniformly distributed across the PTCH1 gene. Six of them had familial history of NBCCS, two of them were novel and have not been described previously. NBCCS and BFHS may be the same genetic entity and not two distinctive syndromes. The inclusion of BFH in the NBCCS cutaneous tumor spectrum might be useful for the recognition of misdiagnosed NBCCS cases that could benefit from tailored surveillance strategies. Copyright© 2018, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.

  15. Nasal Cavity Masses Resembling Chondro-osseous Respiratory Epithelial Adenomatoid Hamartomas in 3 Dogs.

    PubMed

    LaDouceur, E E B; Michel, A O; Lindl Bylicki, B J; Cifuentes, F F; Affolter, V K; Murphy, B G

    2016-05-01

    Chondro-osseous respiratory epithelial adenomatoid hamartomas (COREAHs) are rare tumors in the nasal cavity of people, which have not been described in other species. COREAHs in people are minimally invasive and rarely recur following excision. Histologically, these tumors are composed of disorganized, mature, nasal turbinate tissue that is organized into polypoid growths. These growths are lined by respiratory epithelium, contain glandular elements, and are organized around central cores of chondro-osseous matrix. This report describes 3 cases of dogs with nasal tumors that have histomorphology similar to that of COREAH in people. The tumors were all identified within the nasal cavity and were associated with regional bony lysis of the turbinates and surrounding skull bones, a feature that has not been reported in COREAH in people. There was no evidence of metastasis or extension beyond the nasal cavity in any of the 3 cases. © The Author(s) 2015.

  16. AKT activation promotes PTEN hamartoma tumor syndrome–associated cataract development

    PubMed Central

    Sellitto, Caterina; Li, Leping; Gao, Junyuan; Robinson, Michael L.; Lin, Richard Z.; Mathias, Richard T.; White, Thomas W.

    2013-01-01

    Mutations in the human phosphatase and tensin homolog (PTEN) gene cause PTEN hamartoma tumor syndrome (PHTS), which includes cataract development among its diverse clinical pathologies. Currently, it is not known whether cataract formation in PHTS patients is secondary to other systemic problems, or the result of the loss of a critical function of PTEN within the lens. We generated a mouse line with a lens-specific deletion of Pten (PTEN KO) and identified a regulatory function for PTEN in lens ion transport. Specific loss of PTEN in the lens resulted in cataract. PTEN KO lenses exhibited a progressive age-related increase in intracellular hydrostatic pressure, along with, increased intracellular sodium concentrations, and reduced Na+/K+-ATPase activity. Collectively, these defects lead to lens swelling, opacities and ultimately organ rupture. Activation of AKT was highly elevated in PTEN KO lenses compared to WT mice. Additionally, pharmacological inhibition of AKT restored normal Na+/K+-ATPase activity in primary cultured lens cells and reduced lens pressure in intact lenses from PTEN KO animals. These findings identify a direct role for PTEN in the regulation of lens ion transport through an AKT-dependent modulation of Na+/K+-ATPase activity, and provide a new animal model to investigate cataract development in PHTS patients. PMID:24270425

  17. Fibrous hamartoma of infancy: a clinical pathological analysis of seventeen cases

    PubMed Central

    Yu, Guojun; Wang, Yibing; Wang, Guangjun; Zhang, Dawei; Sun, Yong

    2015-01-01

    To discuss the clinical and pathological features, differential diagnosis and prognosis of fibrous hamartoma of infancy (FHI), seventeen FHI specimens were analyzed with H&E staining and strepavidin peroxidase (SP) immunohistochemistry to detect distinguishing tissue markers. The long-term outcomes of select cases were also obtained. Among the 17 patients (13 males, 4 females, average age 16 months), FHI manifested as a subcutaneous painless mass, primarily on the back of the neck, the upper arms and buttocks. One recurrence was noted among six follow-up cases. The tumors consisted of three main components: fibrous connective tissue; mature fat; and undifferentiated mesenchymal tissue. Immunohistochemistry revealed that fibrous connective tissue was positive for SMA and actin, mature fat tissue was positive for S-100 protein, and undifferentiated mesenchymal tissue was positive for CD34 and was partially positive for actin and SMA. The tumors were negative for desmin, NSE, bcl-2, β-catenin and Ki-67. In brief, FHI is a benign, fibroblastic/myofibroblastic proliferative lesion. Defined histologic features of FHI as presented here would distinguish FHI from similar invasive tumors including infant fibromatosis, calcifying aponeurotic fibroma, fibrous fatty tumor and embryonal rhabdomyosarcoma. Once clearly identified, FHI is curable with complete resection. PMID:26045872

  18. Fibrous hamartoma of infancy: a clinical pathological analysis of seventeen cases.

    PubMed

    Yu, Guojun; Wang, Yibing; Wang, Guangjun; Zhang, Dawei; Sun, Yong

    2015-01-01

    To discuss the clinical and pathological features, differential diagnosis and prognosis of fibrous hamartoma of infancy (FHI), seventeen FHI specimens were analyzed with H&E staining and strepavidin peroxidase (SP) immunohistochemistry to detect distinguishing tissue markers. The long-term outcomes of select cases were also obtained. Among the 17 patients (13 males, 4 females, average age 16 months), FHI manifested as a subcutaneous painless mass, primarily on the back of the neck, the upper arms and buttocks. One recurrence was noted among six follow-up cases. The tumors consisted of three main components: fibrous connective tissue; mature fat; and undifferentiated mesenchymal tissue. Immunohistochemistry revealed that fibrous connective tissue was positive for SMA and actin, mature fat tissue was positive for S-100 protein, and undifferentiated mesenchymal tissue was positive for CD34 and was partially positive for actin and SMA. The tumors were negative for desmin, NSE, bcl-2, β-catenin and Ki-67. In brief, FHI is a benign, fibroblastic/myofibroblastic proliferative lesion. Defined histologic features of FHI as presented here would distinguish FHI from similar invasive tumors including infant fibromatosis, calcifying aponeurotic fibroma, fibrous fatty tumor and embryonal rhabdomyosarcoma. Once clearly identified, FHI is curable with complete resection.

  19. Bilateral respiratory epithelial adenomatoid hamartoma of the olfactory cleft penetrating into the endocranium.

    PubMed

    Mladina, Ranko; Skitarelić, Neven; Poje, Gorazd; Vuković, Katarina

    2011-09-01

    Respiratory epithelial adenomatoid hamartomas (REAHs) of the nose and paranasal sinuses are relatively rare. These tumors usually do not extend over the boundaries of the nose and sinuses. The authors presented a 65-year-old man experiencing progressive hyposmia, followed by intermittent stubborn headache. The symptoms lasted for almost 2 years and were getting worse very slowly. Fiberendoscopy showed relatively discrete polypoid tissue occupying the olfactory cleft bilaterally. The computed tomography and magnetic resonance imaging suggested the possible lack of the cribriform plate and the unity and uniformity of the tissues located both in the endocranium and high in the nasal cavity. The clinical picture resembled very much a esthesineuroblastoma.The patient underwent endoscopic sinus surgery under the general hypotensive anesthesia. Frozen sections during the surgery showed REAH. The entire tumor was removed in a piece meal way, including both olfactory bulbs because they were involved within the pathologic tissue as well.This case showed that REAH could also be a locally aggressive process, penetrating even into the endocranium.

  20. BRAF V600E mutational status in bile duct adenomas and hamartomas.

    PubMed

    Pujals, Anaïs; Bioulac-Sage, Paulette; Castain, Claire; Charpy, Cécile; Zafrani, Elie Serge; Calderaro, Julien

    2015-10-01

    Bile duct adenomas (BDA) and bile duct hamartomas (BDH) are benign bile duct lesions considered neoplastic or secondary to ductal plate malformation, respectively. We have reported previously a high prevalence of BRAF V600E mutations detected by allele-specific polymerase chain reaction assay in BDA, and suggested that BDA may be precursors to a subset of intrahepatic cholangiocarcinomas harbouring V600E mutations. The aim of the present study was to assess the existence of BRAF V600E mutations, using immunohistochemical methods, in additional BDA as well as in BDH. Fifteen BDA and 35 BDH were retrieved from the archives of the pathology departments of two French university hospitals. All cases were reviewed by two pathologists specialized in liver diseases. BRAF V600E mutational status was investigated by immunohistochemistry. Mutated BRAF mutant protein was detected in 53% of the BDA and in none of the cases of BDH. Our findings suggest that BDA and BDH are different processes, and that BDA represent true benign neoplasms. They also support the hypothesis that mutated BDA might precede the development of the subset of intrahepatic cholangiocarcinomas harbouring BRAF V600E mutations. © 2015 John Wiley & Sons Ltd.

  1. Status gelasticus after temporal lobectomy: ictal FDG-PET findings and the question of dual pathology involving hypothalamic hamartomas.

    PubMed

    Palmini, Andre; Van Paesschen, Wim; Dupont, Patrick; Van Laere, Koen; Van Driel, Guido

    2005-08-01

    To present the first ictal fluorodeoxyglucose-positron emission tomography (FDG-PET) evidence of the hypothalamic origin of gelastic seizures in a patient with a hypothalamic hamartoma (HH) and to raise the issue of true dual pathology related to this entity. Ictal FDG-PET was acquired during an episode of status gelasticus with preserved consciousness, in a patient previously operated on for complex partial seizures (CPSs) due to a temporal lobe epileptogenic cyst. Ictal hypermetabolism was localized to the region of the HH during the status gelasticus. CPSs had been completely eliminated after temporal lobe surgery. Ictal FDG-PET independently confirmed that gelastic seizures in patients with HH do originate in the diencephalic lesion. An HH may coexist with another epileptogenic lesion, in a context of dual pathology.

  2. Broad spectrum of neuropsychiatric phenotypes associated with white matter disease in PTEN hamartoma tumor syndrome.

    PubMed

    Balci, Tugce B; Davila, Jorge; Lewis, Denice; Boafo, Addo; Sell, Erick; Richer, Julie; Nikkel, Sarah M; Armour, Christine M; Tomiak, Eva; Lines, Matthew A; Sawyer, Sarah L

    2018-01-01

    White matter lesions have been described in patients with PTEN hamartoma tumor syndrome (PHTS). How these lesions correlate with the neurocognitive features associated with PTEN mutations, such as autism spectrum disorder (ASD) or developmental delay, has not been well established. We report nine patients with PTEN mutations and white matter changes on brain magnetic resonance imaging (MRI), eight of whom were referred for reasons other than developmental delay or ASD. Their clinical presentations ranged from asymptomatic macrocephaly with normal development/intellect, to obsessive compulsive disorder, and debilitating neurological disease. To our knowledge, this report constitutes the first detailed description of PTEN-related white matter changes in adult patients and in children with normal development and intelligence. We present a detailed assessment of the neuropsychological phenotype of our patients and discuss the relationship between the wide array of neuropsychiatric features and observed white matter findings in the context of these individuals. © 2017 Wiley Periodicals, Inc.

  3. Hydrometrocolpos, postaxial polydactyly, and hypothalamic hamartoma in a patient with confirmed Pallister-Hall syndrome: a clinical overlap with McKusick-Kaufman syndrome.

    PubMed

    Kos, Sebastian; Roth, Katharina; Korinth, Dirk; Zeilinger, Georg; Eich, Georg

    2008-08-01

    We present a preterm-born girl with polydactyly of both hands and massive hydrometrocolpos, the latter due to vaginal atresia. This association led initially to the diagnosis of McKusick-Kaufman syndrome (MKKS). However, additional features, including characteristic radiographic findings of the hands and a large hypothalamic tumour, presumably a hamartoma, favoured the diagnosis of Pallister-Hall syndrome (PHS), which was then genetically confirmed by detection of a GLI3 mutation (Q717X). This is the second genetically confirmed case revealing the previously described association of PHS with hydrometrocolpos due to vaginal atresia as a clinical overlap with MKKS.

  4. A recessive form of extreme macrocephaly and mild intellectual disability complements the spectrum of PTEN hamartoma tumour syndrome.

    PubMed

    Schwerd, Tobias; Khaled, Andrea V; Schürmann, Manfred; Chen, Hannah; Händel, Norman; Reis, André; Gillessen-Kaesbach, Gabriele; Uhlig, Holm H; Abou Jamra, Rami

    2016-06-01

    PTEN hamartoma tumour syndrome (PHTS) is caused by heterozygous variants in PTEN and is characterised by tumour predisposition, macrocephaly, and cognition impairment. Bi-allelic loss of PTEN activity has not been reported so far and animal models suggest that bi-allelic loss of PTEN activity is embryonically lethal. Here, we report the identification of a novel homozygous variant in PTEN, NM_000314.4; c.545T>C; p.Leu182Ser, in two adolescent siblings with severe macrocephaly and mild intellectual disability. The variant is predicted to be damaging and is associated with significantly increased phospho-S6 downstream of PTEN. The absence of tumours in the two homozygous siblings as well as lack of symptoms of PHTS in the heterozygous carriers of the family suggest that this particular variant is functionally hypomorphic rather than deleterious.

  5. A recessive form of extreme macrocephaly and mild intellectual disability complements the spectrum of PTEN hamartoma tumour syndrome

    PubMed Central

    Schwerd, Tobias; Khaled, Andrea V; Schürmann, Manfred; Chen, Hannah; Händel, Norman; Reis, André; Gillessen-Kaesbach, Gabriele; Uhlig, Holm H; Abou Jamra, Rami

    2016-01-01

    PTEN hamartoma tumour syndrome (PHTS) is caused by heterozygous variants in PTEN and is characterised by tumour predisposition, macrocephaly, and cognition impairment. Bi-allelic loss of PTEN activity has not been reported so far and animal models suggest that bi-allelic loss of PTEN activity is embryonically lethal. Here, we report the identification of a novel homozygous variant in PTEN, NM_000314.4; c.545T>C; p.Leu182Ser, in two adolescent siblings with severe macrocephaly and mild intellectual disability. The variant is predicted to be damaging and is associated with significantly increased phospho-S6 downstream of PTEN. The absence of tumours in the two homozygous siblings as well as lack of symptoms of PHTS in the heterozygous carriers of the family suggest that this particular variant is functionally hypomorphic rather than deleterious. PMID:26443266

  6. Cytokeratin 20 expression in basaloid follicular hamartoma and infundibulocystic basal cell carcinoma.

    PubMed

    Honarpisheh, Hedieh; Glusac, Earl J; Ko, Christine J

    2014-12-01

    Tumors with similar or identical histopathologic features have been termed basaloid follicular hamartoma (BFH) or infundibulocystic basal cell carcinoma (BCC). BCC typically lacks immunoreactivity with cytokeratin 20 (CK20) and pleckstrin homology-like domain, family A, member 1 protein (PHLDA1). A series of BFH and infundibulocystic BCC were investigated to determine the pattern of CK20 and PHLDA1 labeling in these lesions. Thirty-six samples of BFH (n = 14) and infundibulocystic BCC (n = 22) were collected. CK20 and PHLDA1 staining was performed and evaluated. All the lesions were small (average of 3 mm), well circumscribed, and composed of basaloid to squamoid cells arranged in islands resembling ramifying rootlets with interspersed horn cysts. CK20-positive cells were present in all 36 cases (average, 22/mm(2)), throughout the tumor, including deeper portions, irrespective of original diagnosis. Six of thirty cases (20%; 5 infundibulocystic BCC, 1 BFH) were focally PHLDA1 positive. Findings on hematoxylin and eosin staining and those of CK20 staining in BFH and infundibulocystic BCC were similar, and in most cases were indistinguishable. The CK20 labeling was similar to that of trichoepithelioma. The findings add a degree of support to the argument that BFH and infundibulocystic BCC represent the same lesion and, further, a benign one. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  7. Orbital fibrous dysplasia with soft tissue hamartoma--a variant of Mazabraud's syndrome.

    PubMed

    Santos, C T; Choo, C T; Loh, A H L

    2008-01-01

    Mazabraud's syndrome was first described, by Mazabraud et al. as a rare disease, associated with single or multiple intramuscular myxomas with monostotic or polyostotic forms of fibrous dyplasia, Reports of this syndrome is rare and usually associated with myoxomatas. The authors would want to report the occurrence of this rare case associated with a hamartoma as basis for record and future studies. An adult male came to the institution 4 years ago complaining of a right unilateral proptosis that he had since childhood. However, proptosis progressed in a month's time accompanied by right eye redness. Right eye was congested with a palpable firm mass on the right lower lid. Best corrected visual acuity was 6/15-2. Pupillary examination revealed the presence of RAPD on the right eye. Exophthalmometry revealed a 10 mm proptosis. EOMS were restricted on the right eye and diplopia was noted in all gazes. Ishihara test showed 14/15 for the right eye. CT SCAN and MRI confirmed a mild inflammatory extraconal or intramuscular mass in the right obito-retrobulbar region. Bony changes seen in the right zygoma, sphenoid bone and squamous part of the temporal bone with extensive bone mineralization. The patient underwent a right lateral orbitotomy with biopsy and debulking of the inferior orbital mass. Histopathology revealed a hamartomatous lesion. Due to the associated soft tissue lesion with multiple craniofacial bone changes, this case may be considered a variant of Mazabraud's Syndrome. The associated hamatomatous lesion may be a rare occurrence that should be noted.

  8. Gamma knife surgery for epilepsy related to hypothalamic hamartomas.

    PubMed

    Régis, J; Bartolomei, F; de Toffol, B; Genton, P; Kobayashi, T; Mori, Y; Takakura, K; Hori, T; Inoue, H; Schröttner, O; Pendl, G; Wolf, A; Arita, K; Chauvel, P

    2000-12-01

    Drug-resistant epilepsy associated with hypothalamic hamartomas (HHs) can be cured by microsurgical resection of the lesions. Morbidity and mortality rates for microsurgery in this area are significant. Gamma knife surgery (GKS) is less invasive and seems to be well adapted for this indication. To evaluate the safety and efficacy of GKS to treat this uncommon pathological condition, we organized a multicenter retrospective study. Ten patients were treated in seven different centers. The follow-up periods were more than 12 months for eight patients, with a median follow-up period of 28 months (mean, 35 mo; range, 12-71 mo). All patients had severe drug-resistant epilepsy, including frequent gelastic and generalized tonic or tonicoclonic attacks. The median age was 13.5 years (range, 1-32 yr; mean, 14 yr) at the time of GKS. Three patients experienced precocious puberty. All patients had sessile HHs. The median marginal dose was 15.25 Gy (range, 12-20 Gy). Two patients were treated two times (at 19 and 49 mo) because of insufficient efficacy. All patients exhibited improvement. Four patients were seizure-free, one experienced rare nocturnal seizures, one experienced some rare partial seizures but no more generalized attacks, and two exhibited only improvement, with reductions in the frequency of seizures but persistence of some rare generalized seizures. Two patients, now seizure-free, were considered to exhibit insufficient improvement after the first GKS procedure and were treated a second time. A clear correlation between efficacy and dose was observed in this series. The marginal dose was more than 17 Gy for all patients in the successful group and less than 13 Gy for all patients in the "improved" group. No side effects were reported, except for poikilothermia in one patient. Behavior was clearly improved for two patients (with only slight improvements in their epilepsy). Complete coverage of the HHs did not seem to be mandatory, because the dosimetry spared a

  9. Undifferentiated (embryonal) liver sarcoma: synchronous and metachronous occurrence with neoplasms other than mesenchymal liver hamartoma.

    PubMed

    Gasljevic, Gorana; Lamovec, Janez; Jancar, Janez

    2011-08-01

    Undifferentiated (embryonal) liver sarcoma (UELS) is a rare tumor that typically occurs in children. The association of UELS with neoplasm other than mesenchymal liver hamartoma is exceedingly rare. The aim of the study was to report 3 cases of UELS, 2 of them being interesting because of their association with another neoplasm, vaginal embryonal rhabdomyosarcoma in a teenage girl and B-acute lymphoblastic leukemia in a middle-aged woman. Besides, one of our cases of UELS, in a 58-year-old woman, is an extremely rare presentation of such a tumor in a middle-aged adult. The patient's clinical features, therapy, and pathologic results were reviewed; immunohistochemical analysis and, in 2 cases, electron microscopy were performed. In this study, all 3 patients were females aged 13, 13, and 58 years. Histopathologic evaluation of resected liver tumors confirmed the diagnosis of UELS in all of them. In 2 of the cases, metachronous occurrence of UELS with vaginal embryonal rhabdomyosarcoma in a teenage girl and B-acute lymphoblastic leukemia in a middle-aged woman is described. Careful clinical analysis, histologic studies, and immunohistochemistry are mandatory to distinguish UELS from other hepatic malignancies with similar or overlapping features and to exclude the possibility of other tumors that may be considered in the differential diagnosis. The association of UELS with another neoplasm is exceedingly rare. Copyright © 2011 Elsevier Inc. All rights reserved.

  10. Hypothalamic hamartoma: is the epileptogenic zone always hypothalamic? Arguments for independent (third stage) secondary epileptogenesis.

    PubMed

    Scholly, Julia; Valenti, Maria-Paola; Staack, Anke M; Strobl, Karl; Bast, Thomas; Kehrli, Pierre; Steinhoff, Bernhard J; Hirsch, Edouard

    2013-12-01

    Gelastic seizures associated with hypothalamic hamartomas (HHs) are a clinicoradiologic syndrome presenting with a variety of symptoms, including pharmacoresistant epilepsy with multiple seizure types, electroencephalography (EEG) abnormalities, precocious puberty, behavioral disturbances, and progressive cognitive deterioration. Surgery in adults provides seizure freedom in only one third of patients. The poor results of epilepsy surgery could be explained by an extrahypothalamic epileptogenic zone. The existence of an independent, secondary epileptogenic area with persistent seizures after resection of the presumably primary lesion supports the concept of a "hypothalamic plus" epilepsy. "Hypothalamic plus" epilepsy could be related to either an extrahypothalamic structural lesion (visible on magnetic resonance imaging [MRI] or on neuropathology) or if the former is absent, to a functional alteration with enhanced epileptogenic properties due to a process termed secondary epileptogenesis. We report two patients with gelastic seizures with HH (gelastic seizures isolated or associated with dyscognitive seizures of temporal origin). Both patients underwent two-step surgery: first an endoscopic resection of the HH, followed at a later time by temporal lobectomy. Both patients became seizure-free only after the temporal lobectomy. In both cases, neuropathology failed to demonstrate a significant structural lesion in the temporal lobe. To our knowledge, for the first time, these two cases suggest the existence of independent secondary epileptogenesis in humans. Wiley Periodicals, Inc. © 2013 International League Against Epilepsy.

  11. DOS.

    ERIC Educational Resources Information Center

    Traven, Bill

    1988-01-01

    Discusses using the DOS PATH command (for MS-DOS) to enable the microcomputer user to move from directory to directory on a hard drive. Lists the commands to be programed, gives examples, and explains the use of each. (MVL)

  12. Four-year follow-up study in a NF1 boy with a focal pontine hamartoma.

    PubMed

    Parisi, Pasquale; Persechino, Severino; Paolino, Maria Chiara; Nicita, Francesco; Torrente, Isabella; Bozzao, Alessandro; Villa, Maria Pia

    2013-02-11

    Neurofibromatosis is a collective name for a group of genetic conditions in which benign tumours affect the nervous system. Type 1 is caused by a genetic mutation in the NF1 gene (OMIM 613113) and symptoms can vary dramatically between individuals, even within the same family. Some people have very mild skin changes, whereas others suffer severe medical complications. The condition usually appears in childhood and is diagnosed if two of the following are present: six or more café-au-lait patches larger than 1.5 cm in diameter, axillary or groin freckling, 2 or more Lisch nodules (small pigmented areas in the iris of the eye), 2 or more neurofibromas, optic pathway gliomas, bone dysplasia, and a first-degree family relative with Neurofibromatosis type 1. The pattern of inheritance is autosomal dominant, however, half of all NF1 cases are 'sporadic' and there is no family history. Neurofibromatosis type 1 is an extremely variable condition whose morbidity and mortality is largely dictated by the occurrence of the many complications that may involve any of the body systems. We describe a family affected by NF1 in whom genetic molecular analysis identified the same mutation in the son and father. Routine MRI showed pontine focal lesions in the eight-year-old son, though not in the father. We performed a four years follow-up study and at follow-up pontine hamartoma size remained unchanged in the son, and the father showed still no brain lesions, confirming thus an intra-familial phenotype variability.

  13. DOS-32 System Manual

    DOT National Transportation Integrated Search

    1973-06-01

    This manual describes the internal workings of the Disk Operating System (DOS-32 for the Noneywell H - 632 computer. DOS - 32 is a core resident, one user, console oriented operating system written primarily in FORTRAN. A companion document DOS - 32 ...

  14. DOS-32 User's Manual

    DOT National Transportation Integrated Search

    1973-01-01

    This manual describes the internal workings of the Disk Operating System (DOS-32 for the Noneywell H - 632 computer. DOS - 32 is a core resident, one user, console oriented operating system written primarily in FORTRAN. A companion document DOS - 32 ...

  15. Immune dysregulation in patients with PTEN hamartoma tumor syndrome: Analysis of FOXP3 regulatory T cells.

    PubMed

    Chen, Hannah H; Händel, Norman; Ngeow, Joanne; Muller, James; Hühn, Michael; Yang, Huei-Ting; Heindl, Mario; Berbers, Roos-Marijn; Hegazy, Ahmed N; Kionke, Janina; Yehia, Lamis; Sack, Ulrich; Bläser, Frank; Rensing-Ehl, Anne; Reifenberger, Julia; Keith, Julia; Travis, Simon; Merkenschlager, Andreas; Kiess, Wieland; Wittekind, Christian; Walker, Lisa; Ehl, Stephan; Aretz, Stefan; Dustin, Michael L; Eng, Charis; Powrie, Fiona; Uhlig, Holm H

    2017-02-01

    Patients with heterozygous germline mutations in phosphatase and tensin homolog deleted on chromosome 10 (PTEN) experience autoimmunity and lymphoid hyperplasia. Because regulation of the phosphoinositide 3-kinase (PI3K) pathway is critical for maintaining regulatory T (Treg) cell functions, we investigate Treg cells in patients with heterozygous germline PTEN mutations (PTEN hamartoma tumor syndrome [PHTS]). Patients with PHTS were assessed for immunologic conditions, lymphocyte subsets, forkhead box P3 (FOXP3) + Treg cell levels, and phenotype. To determine the functional importance of phosphatases that control the PI3K pathway, we assessed Treg cell induction in vitro, mitochondrial depolarization, and recruitment of PTEN to the immunologic synapse. Autoimmunity and peripheral lymphoid hyperplasia were found in 43% of 79 patients with PHTS. Immune dysregulation in patients with PHTS included lymphopenia, CD4 + T-cell reduction, and changes in T- and B-cell subsets. Although total CD4 + FOXP3 + Treg cell numbers are reduced, frequencies are maintained in the blood and intestine. Despite pathogenic PTEN mutations, the FOXP3 + T cells are phenotypically normal. We show that the phosphatase PH domain leucine-rich repeat protein phosphatase (PHLPP) downstream of PTEN is highly expressed in normal human Treg cells and provides complementary phosphatase activity. PHLPP is indispensable for the differentiation of induced Treg cells in vitro and Treg cell mitochondrial fitness. PTEN and PHLPP form a phosphatase network that is polarized at the immunologic synapse. Heterozygous loss of function of PTEN in human subjects has a significant effect on T- and B-cell immunity. Assembly of the PTEN-PHLPP phosphatase network allows coordinated phosphatase activities at the site of T-cell receptor activation, which is important for limiting PI3K hyperactivation in Treg cells despite PTEN haploinsufficiency. Copyright © 2016 The Authors. Published by Elsevier Inc. All rights

  16. DOS cones along atomic chains

    NASA Astrophysics Data System (ADS)

    Kwapiński, Tomasz

    2017-03-01

    The electron transport properties of a linear atomic chain are studied theoretically within the tight-binding Hamiltonian and the Green’s function method. Variations of the local density of states (DOS) along the chain are investigated. They are crucial in scanning tunnelling experiments and give important insight into the electron transport mechanism and charge distribution inside chains. It is found that depending on the chain parity the local DOS at the Fermi level can form cone-like structures (DOS cones) along the chain. The general condition for the local DOS oscillations is obtained and the linear behaviour of the local density function is confirmed analytically. DOS cones are characterized by a linear decay towards the chain which is in contrast to the propagation properties of charge density waves, end states and Friedel oscillations in one-dimensional systems. We find that DOS cones can appear due to non-resonant electron transport, the spin-orbit scattering or for chains fabricated on a substrate with localized electrons. It is also shown that for imperfect chains (e.g. with a reduced coupling strength between two neighboring sites) a diamond-like structure of the local DOS along the chain appears.

  17. Self-regulating the effortful "social dos".

    PubMed

    Cortes, Kassandra; Kammrath, Lara K; Scholer, Abigail A; Peetz, Johanna

    2014-03-01

    In the current research, we explored differences in the self-regulation of the personal dos (i.e., engaging in active and effortful behaviors that benefit the self) and in the self-regulation of the social dos (engaging in those same effortful behaviors to benefit someone else). In 6 studies, we examined whether the same trait self-control abilities that predict task persistence on personal dos would also predict task persistence on social dos. That is, would the same behavior, such as persisting through a tedious and attentionally demanding task, show different associations with trait self-control when it is framed as benefitting the self versus someone else? In Studies 1-3, we directly compared the personal and social dos and found that trait self-control predicted self-reported and behavioral personal dos but not social dos, even when the behaviors were identical and when the incentives were matched. Instead, trait agreeableness--a trait linked to successful self-regulation within the social domain--predicted the social dos. Trait self-control did not predict the social dos even when task difficulty increased (Study 4), but it did predict the social don'ts, consistent with past research (Studies 5-6). The current studies provide support for the importance of distinguishing different domains of self-regulated behaviors and suggest that social dos can be successfully performed through routes other than traditional self-control abilities. (PsycINFO Database Record (c) 2014 APA, all rights reserved).

  18. Eye Involvement in TSC

    MedlinePlus

    ... what we see to the brain via the optic nerve. Retinal and optic nerve involvement in TSC are well known today, ... hamartomas (non-cancerous tumors) of the retina or optic nerve. The most common type of retinal hamartoma ...

  19. Identification of a YAC spanning the translocation breakpoints in uterine leiomyomata, pulmonary chondroid hamartoma, and lipoma: Physical mapping of the 12q14-q15 breakpoint region in uterine leiomyomata

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

    Fejzo, M.S.; Yoon, S.J.; Kucherlapati, R.S.

    1995-03-20

    Uterine leiomyomata are the most common tumors in women and can cause abnormal uterine bleeding, pelvic pain, and infertility. Approximately 200,000 hysterectomies are performed annually in the U.S. to relieve patients of the medical sequelae of these benign neoplasms. Our efforts have focused on cloning the t(12;14)(q14-q15;q23-q24) breakpoint in uterine leiomyoma to further our understanding of the biology of these tumors. Thirty-nine YACs and six cosmids mapping to 12q14-q15 have been mapped by fluorescence in situ hybridization to tumor metaphase chromosomes containing a t(12;14). One YAC spanned the translocation breakpoint and was mapped to tumor metaphases from a pulmonary chondroidmore » hamartoma containing a t(12;14)(q14-q15;q23-q24) and a lipoma containing a t(12;15)(q15;q24); this YAC also spanned the breakpoint in these two tumors, suggesting that the same gene on chromosome 12 may be involved in the pathobiology of these distinct benign neoplasms. 41 refs., 2 figs., 1 tab.« less

  20. Hair follicle nevus in a distribution following Blaskho's lines.

    PubMed

    Germain, Marguerite; Smith, Kathleen J

    2002-05-01

    We present a case of a rare follicular hamartoma, a hair follicle nevus. Although most previous reports have been solitary lesions, the case we are reporting had multiple papules and nodules following Blaskho's lines. There have been no reports of malignancies arising in these hamartomas.

  1. Teaching Bibliometric Analysis and MS/DOS Commands.

    ERIC Educational Resources Information Center

    Dou, Henri; And Others

    1988-01-01

    Outlines the steps involved in bibliometric studies, and demonstrates the ability to execute simple studies on microcomputers by downloading files using only the capability of MS/DOS. Detailed illustrations of the MS/DOS commands used are provided. (eight references) (CLB)

  2. 48 CFR 632.702-70 - DOS policy.

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... 48 Federal Acquisition Regulations System 4 2010-10-01 2010-10-01 false DOS policy. 632.702-70... REQUIREMENTS CONTRACT FINANCING Contract Funding 632.702-70 DOS policy. The Department's policy is to provide... incremental funding of cost-reimbursement contracts. Fixed-price, labor-hour, and time-and-materials contracts...

  3. Disk Operating System--DOS. Teacher Packet. Learning Activity Packets.

    ERIC Educational Resources Information Center

    Oklahoma State Dept. of Vocational and Technical Education, Stillwater. Curriculum and Instructional Materials Center.

    The Learning Activity Packets (LAPs) contained in this manual are designed to assist the beginning user in understanding DOS (Disk Operating System). LAPs will not work with any version below DOS Version 3.0 and do not address the enhanced features of versions 4.0 or higher. These elementary activities cover only the DOS commands necessary to…

  4. Epilepsy related to hypothalamic hamartomas: surgical management with special reference to gamma knife surgery.

    PubMed

    Régis, Jean; Scavarda, Didier; Tamura, Manabu; Nagayi, Mariko; Villeneuve, Nathalie; Bartolomei, Fabrice; Brue, Thierry; Dafonseca, David; Chauvel, Patrick

    2006-08-01

    A large spectrum of surgical techniques can be proposed to young patients presenting with hypothalamic hamartomas (HH) associated with severe epilepsy. The aim of this report is to point on some clinical and anatomical parameters supposed to influence the choice of the surgical approach and to emphasize the specific role of radiosurgery. We reviewed both our experience and the recent literature based on a Pubmed search. Lateral pterional, midline frontal through the lamina terminalis, transcallosal interforniceal approaches, endoscopic treatment through the foramen of Monro, disconnecting surgery, radiofrequency ablation, brachytherapy and gamma knife surgery (GKS) were all considered. Mortality, morbidity, and efficacy of each of these techniques were compared. Specific limits, difficulties, and constraints were taken into account. Our experience of radiosurgery is based on a prospective trial which enrolled 60 patients with HH and associated severe epilepsy between October 1999 and December 2005. Several surgical techniques can lead to a real reversal of the epileptic encephalopathy. The main factors for the decision-making process are the age, the size of the lesion and its anatomical type (according to our original classification), the severity of the epilepsy, and the severity of the cognitive/psychiatric comorbidity. In our prospective trial (GKS), 27 patients have a follow-up superior to 3 years. Among those, 59.2% have an excellent result with a dramatic behavioral and cognitive improvement and are completely seizure-free (37%) or have only rare non-disabling seizures (22.2%). No permanent neurological complication has been observed so far; three patients have presented a transient poïkilothermia. GKS is clearly the safer approach for these difficult patients. Young patients with severe epilepsy and comorbidity must be operated on using a curative approach as early as possible. Very large type VI or mixed type with a large component above the floor of the

  5. 33 CFR 104.255 - Declaration of Security (DoS).

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... and for handling DoS requests from a facility or other vessel. (b) At MARSEC Level 1, the Master or... engaging in a vessel-to-vessel activity, prior to the activity, the respective Masters, VSOs, or their... DoS for the period of the vessel-to-vessel activity. Upon the vessel-to-vessel activity and prior to...

  6. 33 CFR 104.255 - Declaration of Security (DoS).

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... and for handling DoS requests from a facility or other vessel. (b) At MARSEC Level 1, the Master or... engaging in a vessel-to-vessel activity, prior to the activity, the respective Masters, VSOs, or their... DoS for the period of the vessel-to-vessel activity. Upon the vessel-to-vessel activity and prior to...

  7. 27 CFR 9.175 - Dos Rios.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... of this chapter, “Dos Rios” is a term of viticultural significance. (b) Approved Maps. The..., T22N, R14W, west of Eberle Ridge, (Iron Peak Quadrangle); and (15) Proceed generally southeast along...

  8. 27 CFR 9.175 - Dos Rios.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... of this chapter, “Dos Rios” is a term of viticultural significance. (b) Approved Maps. The..., T22N, R14W, west of Eberle Ridge, (Iron Peak Quadrangle); and (15) Proceed generally southeast along...

  9. Hair follicle nevus of the abdominal skin: an unusual extracephalic presentation.

    PubMed

    Jedrych, Jaroslaw; Akilov, Oleg; Gehris, Robin; Ho, Jonhan

    2014-01-01

    Hair follicle nevus (HFN) is a rare hamartoma typically diagnosed on the face of infants, where it may clinically mimic an accessory tragus. We report a 6-month-old boy who presented with a congenital fleshy, bilobed papule in the midline of his upper abdomen that upon excision was classified as an HFN based on detailed histopathologic examination. Our report documents a previously undescribed extracephalic location of the HFN and therefore expands the spectrum of clinical presentations of this rare hamartoma. © 2014 Wiley Periodicals, Inc.

  10. Unique Regulation of the DosR Regulon in the Beijing Lineage of Mycobacterium tuberculosis.

    PubMed

    Domenech, Pilar; Zou, Jason; Averback, Alexandra; Syed, Nishath; Curtis, Daniele; Donato, Samuel; Reed, Michael B

    2017-01-15

    The DosR regulon, a set of 48 genes normally expressed in Mycobacterium tuberculosis under conditions that inhibit aerobic respiration, is controlled via the DosR-DosS/DosT two-component system. While the regulon requires induction in most M. tuberculosis isolates, for members of the Beijing lineage, its expression is uncoupled from the need for signaling. In our attempts to understand the mechanistic basis for this uncoupling in the Beijing background, we previously reported the identification of two synonymous single-nucleotide polymorphisms (SNPs) within the adjacent Rv3134c gene. In the present study, we have interrogated the impact of these SNPs on dosR expression in wild-type strains, as well as a range of dosR-dosS-dosT mutants, for both Beijing and non-Beijing M. tuberculosis backgrounds. In this manner, we have unequivocally determined that the C601T dosR promoter SNP is the sole requirement for the dramatic shift in the pattern of DosR regulon expression seen in this globally important lineage. Interestingly, we also show that DosT is completely nonfunctional within these strains. Thus, a complex series of evolutionary steps has led to the present-day Beijing DosR phenotype that, in turn, potentially confers a fitness advantage in the face of some form of host-associated selective pressure. Mycobacterium tuberculosis strains of the Beijing lineage have been described as being of enhanced virulence compared to other lineages, and in certain regions, they are associated with the dramatic spread of multidrug-resistant tuberculosis (TB). In terms of trying to understand the functional basis for these broad epidemiological phenomena, it is interesting that, in contrast to the other major lineages, the Beijing strains all constitutively overexpress members of the DosR regulon. Here, we identify the mutational events that led to the evolution of this unique phenotype. In addition, our work highlights the fact that important phenotypic differences exist between

  11. America Inc.: John Dos Passos'"USA" as Professional Writing Textbook.

    ERIC Educational Resources Information Center

    Di Renzo, Anthony

    While working as a special consultant for General Mills in 1948, John Dos Passos wrote a report explaining the latest scientific research and technological advancements and how the postwar economy was affecting General Mills and the cereal market. General Mills, using a real writer for a corporate freelance, profited from Dos Passos' expertise and…

  12. Izabel dos Santos and the training of the health workers.

    PubMed

    Paiva, Carlos Henrique Assunção

    2015-06-01

    This article discusses the career of Izabel dos Santos (1927-2010) as a means of examining the connections between health schools and agendas in contemporary Brazil. The article highlights dos Santos's training and her work in the Serviço Especial de Saúde Pública (SESP- Special Public Health Service), the Pan American Health Organization (PAHO) and in the formulation and implementation of national training programs for human resources within the area of health from the late 1970s onwards. The article highlights dos Santos's central role in the formulation and implementation of training policies for health workers, especially nursing technicians and assistants, and demonstrates how she occupies an important place in the history of Brazilian public health.

  13. Sedimentation History of Lago Dos Bocas, Puerto Rico, 1942-2005

    USGS Publications Warehouse

    Soler-López, Luis R.

    2007-01-01

    The Lago Dos Bocas Dam, located in the municipality of Utuado in north central Puerto Rico, was constructed in 1942 for hydroelectric power generation. The reservoir had an original storage capacity of 37.50 million cubic meters and a drainage area of 440 square kilometers. In 1948, the construction of the Lago Caonillas Dam on the Rio Caonillas branch of Lago Dos Bocas reduced the natural sediment-contributing drainage area to 310 square kilometers; therefore, the Lago Caonillas Dam is considered an effective sediment trap. Sedimentation in Lago Dos Bocas reservoir has reduced the storage capacity from 37.50 million cubic meters in 1942 to 17.26 million cubic meters in 2005, which represents a storage loss of about 54 percent. The long-term annual water-storage capacity loss rate remained nearly constant at about 320,000 cubic meters per year to about 1997. The inter-survey sedimentation rate between 1997 and 1999, however, is higher than the long-term rate at about 1.09 million cubic meters per year. Between 1999 and 2005 the rate is lower than the long-term rate at about 0.13 million cubic meters per year. The Lago Dos Bocas effective sediment-contributing drainage area had an average sediment yield of about 1,400 cubic meters per square kilometer per year between 1942 and 1997. This rate increased substantially by 1999 to about 4,600 cubic meters per square kilometer per year, probably resulting from the historical magnitude floods caused by Hurricane Georges in 1998. Recent data indicate that the Lago Dos Bocas drainage area sediment yield decreased substantially to about 570 cubic meters per square kilometer per year, which is much lower than the 1942-1997 area normalized sedimentation rate of 1,235 cubic meters per square kilometer per year. The impact of Hurricane Georges on the basin sediment yield could have been the cause of this change, since the magnitude of the floods could have nearly depleted the Lago Dos Bocas drainage area of easily erodible and

  14. Measurements of degree of sensitization (DoS) in aluminum alloys using EMAT ultrasound.

    PubMed

    Li, Fang; Xiang, Dan; Qin, Yexian; Pond, Robert B; Slusarski, Kyle

    2011-07-01

    Sensitization in 5XXX aluminum alloys is an insidious problem characterized by the gradual formation and growth of beta phase (Mg(2)Al(3)) at grain boundaries, which increases the susceptibility of alloys to intergranular corrosion (IGC) and intergranular stress-corrosion cracking (IGSCC). The degree of sensitization (DoS) is currently quantified by the ASTM G67 Nitric Acid Mass Loss Test, which is destructive and time consuming. A fast, reliable, and non-destructive method for rapid detection and the assessment of the condition of DoS in AA5XXX aluminum alloys in the field is highly desirable. In this paper, we describe a non-destructive method for measurements of DoS in aluminum alloys with an electromagnetic acoustic transducer (EMAT). AA5083 aluminum alloy samples were sensitized at 100°C with processing times varying from 7days to 30days. The DoS of sensitized samples was first quantified with the ASTM 67 test in the laboratory. Both ultrasonic velocity and attenuation in sensitized specimens were then measured using EMAT and the results were correlated with the DoS data. We found that the longitudinal wave velocity was almost a constant, independent of the sensitization, which suggests that the longitudinal wave can be used to determine the sample thickness. The shear wave velocity and especially the shear wave attenuation are sensitive to DoS. Relationships between DoS and the shear velocity, as well as the shear attenuation have been established. Finally, we performed the data mining to evaluate and improve the accuracy in the measurements of DoS in aluminum alloys with EMAT. Copyright © 2010 Elsevier B.V. All rights reserved.

  15. Safety and efficacy of Gamma Knife radiosurgery in hypothalamic hamartomas with severe epilepsies: A prospective trial in 48 patients and review of the literature.

    PubMed

    Régis, Jean; Lagmari, Medhi; Carron, Romain; Hayashi, Motohiro; McGonigal, Aileen; Daquin, Géraldine; Villeneuve, Nathalie; Laguitton, Virginie; Bartolomei, Fabrice; Chauvel, Patrick

    2017-06-01

    Epilepsies associated with hypothalamic hamartomas (HHs) are frequently drug resistant with severe psychiatric and cognitive comorbidities. We performed a prospective trial to evaluate the safety and efficacy of Gamma Knife radiosurgery (GKS). Between October 1999 and October 2007, a total of 57 patients were investigated, included and treated by GKS in Timone University Hospital. Preoperative workup and 3-year postoperative evaluation consisted of seizure diary, neuropsychological, psychiatric, endocrinologic, visual field, and visual acuity examinations. Follow-up of >3 years was available for 48 patients. Topologic type was type I in 11 patients, type II in 15, type III in 17, type IV in one, type V in one, type VI in one, and mixed type in 2. The median marginal dose was 17 Gy (min 14 and max 25 Gy). The median target volume was 398 mm 3 (28-1,600 mm 3 ). Due to partial results, 28 patients (58.3%) required a second treatment. The median follow-up was 71 months (36-153 months). At last follow-up, the rate of Engel class I outcome was 39.6%, Engel class II was 29.2% (I+II 68.8%), and Engel class III was 20%. Global psychiatric comorbidity was considered cured in 28%, improved in 56%, stable in 8%, and continued to worsen in 8%. No permanent neurologic side effect was reported (in particular, no memory deficit). Nondisabling transient poikilothermia was observed in three patients (6.2%). A transient increase of seizure frequency was reported in 8 patients (16.6%) with a median duration of 30 days (9-90 days). Microsurgery was proposed because of insufficient efficacy of GKS in seven patients (14.5%) with a postoperative Engel class I-II in 28.6%. This prospective trial demonstrates very good long-term safety and efficacy of GKS for 2 patients. Beyond seizure reduction, the improvement of psychiatric and cognitive comorbidities along with better school performance and social functioning, being better socially integrated, having friends having a social life

  16. Familial Pallister-Hall in adulthood.

    PubMed

    Talsania, Mitali; Sharma, Rohan; Sughrue, Michael E; Scofield, R Hal; Lim, Jonea

    2017-10-01

    Pallister Hall syndrome is autosomal dominant disorder usually diagnosed in infants and children. Current diagnostic criteria include presence of hypothalamic hamartoma, post axial polydactyly and positive family history, but the disease has variable manifestations. Herein we report Pallister Hall syndrome diagnosed in a family where both patients were adults. A 59 year old man developed seizures 4 years prior to our evaluation of him, at which time imaging showed a hypothalamic hamartoma. The seizures were controlled medically. He did well until he had visual changes after a traumatic head injury. Repeat MRI showed slight expansion of the mass with formal visual field testing demonstrating bitemporal hemianopsia. There was no evidence of pituitary dysfunction except for large urine volume. He underwent surgery to debulk the hamartoma and the visual field defects improved. There was no hypopituitarism post-operatively, and the polydyspia resolved. His 29 year old daughter also had seizures and hypothalamic hamartoma. Both patients had had polydactyly with prior surgical correction in childhood. The daughter underwent genetic testing, which revealed a previously undescribed heterozygous single base pair deletion in exon 13 of the GLI3 gene causing a frameshift mutation. Further investigation into family history revealed multiple members in previous generations with polydactyly and/or seizures. Pallister-Hall syndrome is caused by an inherited autosomal dominant or de novo mutation in GLI3 gene. This rare syndrome has not had prevalence defined, however. Generally, diagnoses are made in the pediatric population. Our report adds to the few cases detected in adulthood.

  17. "DOS for Managers." Management Training Series.

    ERIC Educational Resources Information Center

    Marion County Schools, Fairmont, WV.

    A plan is provided for a lesson on disk operating systems (DOS) for managers. Twenty-five lesson objectives are listed, followed by suggestions for learning activities and special resources. In the presentation section, key points and content are provided for 25 instructional topics that correspond to the 25 lesson objectives. The topics are as…

  18. A DOS Primer for Librarians: Part II.

    ERIC Educational Resources Information Center

    Beecher, Henry

    1990-01-01

    Provides an introduction to DOS commands and strategies for the effective organization and use of hard disks. Functions discussed include the creation of directories and subdirectories, enhanced copying, the assignment of disk drives, and backing up the hard disk. (CLB)

  19. Delineation and Diagnostic Criteria of Oral-Facial-Digital Syndrome Type VI

    PubMed Central

    2012-01-01

    Oral-Facial-Digital Syndrome type VI (OFD VI) represents a rare phenotypic subtype of Joubert syndrome and related disorders (JSRD). In the original report polydactyly, oral findings, intellectual disability, and absence of the cerebellar vermis at post-mortem characterized the syndrome. Subsequently, the molar tooth sign (MTS) has been found in patients with OFD VI, prompting the inclusion of OFD VI in JSRD. We studied the clinical, neurodevelopmental, neuroimaging, and genetic findings in a cohort of 16 patients with OFD VI. We derived the following inclusion criteria from the literature: 1) MTS and one oral finding and polydactyly, or 2) MTS and more than one typical oral finding. The OFD VI neuroimaging pattern was found to be more severe than in other JSRD subgroups and includes severe hypoplasia of the cerebellar vermis, hypoplastic and dysplastic cerebellar hemispheres, marked enlargement of the posterior fossa, increased retrocerebellar collection of cerebrospinal fluid, abnormal brainstem, and frequently supratentorial abnormalities that occasionally include characteristic hypothalamic hamartomas. Additionally, two new JSRD neuroimaging findings (ascending superior cerebellar peduncles and fused thalami) have been identified. Tongue hamartomas, additional frenula, upper lip notch, and mesoaxial polydactyly are specific findings in OFD VI, while cleft lip/palate and other types of polydactyly of hands and feet are not specific. Involvement of other organs may include ocular findings, particularly colobomas. The majority of the patients have absent motor development and profound cognitive impairment. In OFD VI, normal cognitive functions are possible, but exceptional. Sequencing of known JSRD genes in most patients failed to detect pathogenetic mutations, therefore the genetic basis of OFD VI remains unknown. Compared with other JSRD subgroups, the neurological findings and impairment of motor development and cognitive functions in OFD VI are significantly

  20. Documenting 35 years of land cover change: Lago Cachet Dos drainage, Chile

    USGS Publications Warehouse

    Friesen, Beverly A.; Nimick, David A.; Mcgrath, Daniel; Cole, Christopher J.; Wilson, Earl M.; Noble, Suzanne M.; Fahey, Mark J.; Leidich, Jonathan; O'Kuinghttons Villena, Jorge I.

    2015-01-01

    The U.S. Geological Survey (USGS) Special Applications Science Center is monitoring temporal changes at the Colonia Glacier and Lago Cachet Dos, Northern Patagonia Icefield of southern Chile. This location is one of the newest international sites in the USGS Global Fiducial Program (GFP)—a program which provides systematic monitoring of dynamic and environmentally critical areas with high-resolution imagery (http://gfp.usgs.gov/). In 2008, Lago Cachet Dos began experiencing glacial lake outburst floods (GLOFs) during which the entire pool of water (about 200 million cubic meters) rapidly drains from the lake and flows south-southeast through the Colonia Glacier. These catastrophic events cause massive erosion of valley-fill deposits and consequent upstream expansion of Lago Cachet Dos towards Lago Cachet Uno.  Panchromatic and multispectral images for 1979, 2007, and 2014 highlight the dramatic changes that have occurred at this site over a 35-year period. The lake was smallest in 1979, when the Colonia Glacier was at its maximum extent during the study period. Between 1979 and 2007, the glacier shrank causing an increase in the surface area of the lake. The size of the lake increased substantially, from 2.98 square kilometers (km2) in 1979 to 4.41 km2 in 2014, primarily due to erosion of valley-fill deposits upstream of its northern edge by the 15 GLOFs that occurred between April 2008 and February 2014. Ongoing studies of the Colonia Glacier and Lago Cachet Dos are focused on providing real-time monitoring of Lago Cachet Dos lake levels, understanding the history of advances and retreats of the Colonia Glacier, and determining the physical mechanisms and hazards associated with the GLOFs that come from Lago Cachet Dos.

  1. New tumor entities in the 4th edition of the World Health Organization classification of head and neck tumors: Nasal cavity, paranasal sinuses and skull base.

    PubMed

    Thompson, Lester D R; Franchi, Alessandro

    2018-03-01

    The World Health Organization recently published the 4th edition of the Classification of Head and Neck Tumors, including several new entities, emerging entities, and significant updates to the classification and characterization of tumor and tumor-like lesions, specifically as it relates to nasal cavity, paranasal sinuses, and skull base in this overview. Of note, three new entities (NUT carcinoma, seromucinous hamartoma, biphenotypic sinonasal sarcoma,) were added to this section, while emerging entities (SMARCB1-deficient carcinoma and HPV-related carcinoma with adenoid cystic-like features) and several tumor-like entities (respiratory epithelial adenomatoid hamartoma, chondromesenchymal hamartoma) were included as provisional diagnoses or discussed in the setting of the differential diagnosis. The sinonasal tract houses a significant diversity of entities, but interestingly, the total number of entities has been significantly reduced by excluding tumor types if they did not occur exclusively or predominantly at this site or if they are discussed in detail elsewhere in the book. Refinements to nomenclature and criteria were provided to sinonasal papilloma, borderline soft tissue tumors, and neuroendocrine neoplasms. Overall, the new WHO classification reflects the state of current understanding for many relatively rare neoplasms, with this article highlighting the most significant changes.

  2. DataPlus™ - a revolutionary applications generator for DOS hand-held computers

    Treesearch

    David Dean; Linda Dean

    2000-01-01

    DataPlus allows the user to easily design data collection templates for DOS-based hand-held computers that mimic clipboard data sheets. The user designs and tests the application on the desktop PC and then transfers it to a DOS field computer. Other features include: error checking, missing data checks, and sensor input from RS-232 devices such as bar code wands,...

  3. Non-invasive tissue temperature measurements based on quantitative diffuse optical spectroscopy (DOS) of water.

    PubMed

    Chung, S H; Cerussi, A E; Merritt, S I; Ruth, J; Tromberg, B J

    2010-07-07

    We describe the development of a non-invasive method for quantitative tissue temperature measurements using Broadband diffuse optical spectroscopy (DOS). Our approach is based on well-characterized opposing shifts in near-infrared (NIR) water absorption spectra that appear with temperature and macromolecular binding state. Unlike conventional reflectance methods, DOS is used to generate scattering-corrected tissue water absorption spectra. This allows us to separate the macromolecular bound water contribution from the thermally induced spectral shift using the temperature isosbestic point at 996 nm. The method was validated in intralipid tissue phantoms by correlating DOS with thermistor measurements (R=0.96) with a difference of 1.1+/-0.91 degrees C over a range of 28-48 degrees C. Once validated, thermal and hemodynamic (i.e. oxy- and deoxy-hemoglobin concentration) changes were measured simultaneously and continuously in human subjects (forearm) during mild cold stress. DOS-measured arm temperatures were consistent with previously reported invasive deep tissue temperature studies. These results suggest that DOS can be used for non-invasive, co-registered measurements of absolute temperature and hemoglobin parameters in thick tissues, a potentially important approach for optimizing thermal diagnostics and therapeutics.

  4. Rhinencephalon changes in tuberous sclerosis complex.

    PubMed

    Manara, Renzo; Brotto, Davide; Bugin, Samuela; Pelizza, Maria Federica; Sartori, Stefano; Nosadini, Margherita; Azzolini, Sara; Iaconetta, Giorgio; Parazzini, Cecilia; Murgia, Alessandra; Peron, Angela; Canevini, Paola; Labriola, Francesca; Vignoli, Aglaia; Toldo, Irene

    2018-06-17

    Despite complex olfactory bulb embryogenesis, its development abnormalities in tuberous sclerosis complex (TSC) have been poorly investigated. Brain MRIs of 110 TSC patients (mean age 11.5 years; age range 0.5-38 years; 52 female; 26 TSC1, 68 TSC2, 8 without mutation identified in TSC1 or TSC2, 8 not tested) were retrospectively evaluated. Signal and morphological abnormalities consistent with olfactory bulb hypo/aplasia or with olfactory bulb hamartomas were recorded. Cortical tuber number was visually assessed and a neurological severity score was obtained. Patients with and without rhinencephalon abnormalities were compared using appropriate parametric and non-parametric tests. Eight of110 (7.2%) TSC patients presented rhinencephalon MRI changes encompassing olfactory bulb bilateral aplasia (2/110), bilateral hypoplasia (2/110), unilateral hypoplasia (1/110), unilateral hamartoma (2/110), and bilateral hamartomas (1/110); olfactory bulb hypo/aplasia always displayed ipsilateral olfactory sulcus hypoplasia, while no TSC patient harboring rhinencephalon hamartomas had concomitant forebrain sulcation abnormalities. None of the patients showed overt olfactory deficits or hypogonadism, though young age and poor compliance hampered a proper evaluation in most cases. TSC patients with rhinencephalon changes had more cortical tubers (47 ± 29.1 vs 26.2 ± 19.6; p = 0.006) but did not differ for clinical severity (p = 0.45) compared to the other patients of the sample. Olfactory bulb and/or forebrain changes are not rare among TSC subjects. Future studies investigating clinical consequences in older subjects (anosmia, gonadic development etc.) will define whether rhinencephalon changes are simply an imaging feature among the constellation of TSC-related brain changes or a feature to be searched for possible implications in the management of TSC subjects.

  5. First-principle calculation of the electronic structure, DOS and effective mass TlInSe2

    NASA Astrophysics Data System (ADS)

    Ismayilova, N. A.; Orudzhev, G. S.; Jabarov, S. H.

    2017-05-01

    The electronic structure, density of states (DOS), effective mass are calculated for tetragonal TlInSe2 from first principle in the framework of density functional theory (DFT). The electronic structure of TlInSe2 has been investigated by Quantum Wise within GGA. The calculated band structure by Hartwigsen-Goedecker-Hutter (HGH) pseudopotentials (psp) shows both the valence band maximum and conduction band minimum located at the T point of the Brillouin zone. Valence band maximum at the T point and the surrounding parts originate mainly from 6s states of univalent Tl ions. Bottom of the conduction band is due to the contribution of 6p-states of Tl and 5s-states of In atoms. Calculated DOS effective mass for holes and electrons are mDOS h∗ = 0.830m e, mDOS h∗ = 0.492m e, respectively. Electron effective masses are fairly isotropic, while the hole effective masses show strong anisotropy. The calculated electronic structure, density of states and DOS effective masses of TlInSe2 are in good agreement with existing theoretical and experimental results.

  6. Sedimentation survey of Lago Dos Bocas, Utuado, Puerto Rico, January 2010

    USGS Publications Warehouse

    Soler-López, Luis R.

    2014-01-01

    Lago Dos Bocas reservoir was completed in 1942 to provide water for hydroelectric power generation along the northern coast of Puerto Rico. The reservoir had an original storage capacity of 37.50 million cubic meters (Mm3). The dam is located about 9 kilometers (km) northeast of the town of Utuado, immediately downstream of the original confluence of the Río Grande de Arecibo and the Río Caonillas (fig. 1). The Puerto Rico Electric Power Authority (PREPA) owns and operates the Lago Dos Bocas reservoir, and since 1996, the reservoir has become an essential part of the Puerto Rico Aqueduct and Sewer Authority (PRASA) North Coast Superaqueduct Project. The Superaqueduct is supplied by controlled releases for hydroelectric power generation that replenish the public-supply raw-water intake pool located about 10 km downstream from the Lago Dos Bocas Dam (fig. 1). As of 2005, the Superaqueduct supplies about 4.03 cubic meters per second (m3/s) (348,192 cubic meters per day [m3/d]) of potable water to communities along the northern coast, from Arecibo to the San Juan metropolitan area. Because of the importance of the reservoir to the North Coast Superaqueduct, the U.S. Geological Survey (USGS), in cooperation with PRASA, conducted a sedimentation survey of Lago Dos Bocas in January 2009. The results of this survey were used to estimate the useful life and the firm yield of the reservoir, and evaluate the need to dredge the reservoir.

  7. Observational Assessment of Preschool Disruptive Behavior, Part II: validity of the Disruptive Behavior Diagnostic Observation Schedule (DB-DOS).

    PubMed

    Wakschlag, Lauren S; Briggs-Gowan, Margaret J; Hill, Carri; Danis, Barbara; Leventhal, Bennett L; Keenan, Kate; Egger, Helen L; Cicchetti, Domenic; Burns, James; Carter, Alice S

    2008-06-01

    To examine the validity of the Disruptive Behavior Diagnostic Observation Schedule (DB-DOS), a new observational method for assessing preschool disruptive behavior. A total of 327 behaviorally heterogeneous preschoolers from low-income environments comprised the validation sample. Parent and teacher reports were used to identify children with clinically significant disruptive behavior. The DB-DOS assessed observed disruptive behavior in two domains, problems in Behavioral Regulation and Anger Modulation, across three interactional contexts: Examiner Engaged, Examiner Busy, and Parent. Convergent and divergent validity of the DB-DOS were tested in relation to parent and teacher reports and independently observed behavior. Clinical validity was tested in terms of criterion and incremental validity of the DB-DOS for discriminating disruptive behavior status and impairment, concurrently and longitudinally. DB-DOS scores were significantly associated with reported and independently observed behavior in a theoretically meaningful fashion. Scores from both DB-DOS domains and each of the three DB-DOS contexts contributed uniquely to discrimination of disruptive behavior status, concurrently and predictively. Observed behavior on the DB-DOS also contributed incrementally to prediction of impairment over time, beyond variance explained by meeting DSM-IV disruptive behavior disorder symptom criteria based on parent/teacher report. The multidomain, multicontext approach of the DB-DOS is a valid method for direct assessment of preschool disruptive behavior. This approach shows promise for enhancing accurate identification of clinically significant disruptive behavior in young children and for characterizing subtypes in a manner that can directly inform etiological and intervention research.

  8. 33 CFR 105.245 - Declaration of Security (DoS).

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ...) of this section. (e) At MARSEC Levels 1 and 2, FSOs of facilities that frequently interface with the... part to implement a DoS with the VSO prior to any vessel-to-facility interface when he or she deems it...

  9. 33 CFR 105.245 - Declaration of Security (DoS).

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ...) of this section. (e) At MARSEC Levels 1 and 2, FSOs of facilities that frequently interface with the... part to implement a DoS with the VSO prior to any vessel-to-facility interface when he or she deems it...

  10. Oncogenes on my mind: ERK and MTOR signaling in cognitive diseases.

    PubMed

    Krab, Lianne C; Goorden, Susanna M I; Elgersma, Ype

    2008-10-01

    Defects in rat sarcoma viral oncogene homolog (RAS)-extracellular signal regulated kinase (ERK) and phosphatidylinositol 3-kinase (PI3K)-mammalian target of rapamycin (MTOR) signaling pathways have recently been shown to cause several genetic disorders classified as neuro-cardio-facial-cutaneous (NCFC) and Hamartoma syndromes. Although these pathways are well-known players in cell proliferation and cancer, their role in cognitive function is less appreciated. Here, we focus on the cognitive problems associated with mutations in the RAS-ERK and PI3K-MTOR signaling pathways and on the underlying mechanisms revealed by recent animal studies. Cancer drugs have been shown to reverse the cognitive deficits in mouse models of NCFC and Hamartoma syndromes, raising hopes for clinical trials.

  11. International PPB Registry for PPB, DICER1 and Associated Conditions

    ClinicalTrials.gov

    2017-12-18

    Pleuropulmonary Blastoma; Sertoli-Leydig Cell Tumor; DICER1 Syndrome; Cystic Nephroma; Wilms Tumor; Pineoblastoma; Renal Sarcoma; Nodular Hyperplasia of Thyroid; Nasal Chondromesenchymal Hamartoma; Ciliary Body Medulloepithelioma; Neuroblastoma; Pituitary Cancer; Embryonal Rhabdomyosarcoma

  12. A hybrid protection approaches for denial of service (DoS) attacks in wireless sensor networks

    NASA Astrophysics Data System (ADS)

    Gunasekaran, Mahalakshmi; Periakaruppan, Subathra

    2017-06-01

    Wireless sensor network (WSN) contains the distributed autonomous devices with the sensing capability of physical and environmental conditions. During the clustering operation, the consumption of more energy causes the draining in battery power that leads to minimum network lifetime. Hence, the WSN devices are initially operated on low-power sleep mode to maximise the lifetime. But, the attacks arrival cause the disruption in low-power operating called denial of service (DoS) attacks. The conventional intrusion detection (ID) approaches such as rule-based and anomaly-based methods effectively detect the DoS attacks. But, the energy consumption and false detection rate are more. The absence of attack information and broadcast of its impact to the other cluster head (CH) leads to easy DoS attacks arrival. This article combines the isolation and routing tables to detect the attack in the specific cluster and broadcasts the information to other CH. The intercommunication between the CHs prevents the DoS attacks effectively. In addition, the swarm-based defence approach is proposed to migrate the fault channel to normal operating channel through frequency hop approaches. The comparative analysis between the proposed table-based intrusion detection systems (IDSs) and swarm-based defence approaches with the traditional IDS regarding the parameters of transmission overhead/efficiency, energy consumption, and false positive/negative rates proves the capability of DoS prediction/prevention in WSN.

  13. Colorectal cancer risk in hamartomatous polyposis syndromes

    PubMed Central

    Campos, Fábio Guilherme; Figueiredo, Marleny Novaes; Martinez, Carlos Augusto Real

    2015-01-01

    Colorectal cancer (CRC) is a major cause of morbidity and mortality around the world, and approximately 5% of them develop in a context of inherited mutations leading to some form of familial colon cancer syndromes. Recognition and characterization of these patients have contributed to elucidate the genetic basis of CRC. Polyposis Syndromes may be categorized by the predominant histological structure found within the polyps. The aim of the present paper is to review the most important clinical features of the Hamartomatous Polyposis Syndromes, a rare group of genetic disorders formed by the peutz-Jeghers syndrome, juvenil polyposis syndrome and PTEN Hamartoma Tumor Syndrome (Bannayan-Riley-Ruvalacaba and Cowden Syndromes). A literature search was performed in order to retrieve the most recent and important papers (articles, reviews, clinical cases and clinical guidelines) regarding the studied subject. We searched for terms such as “hamartomatous polyposis syndromes”, “Peutz-Jeghers syndrome”, “juvenile polyposis syndrome”, “juvenile polyp”, and “PTEN hamartoma tumour syndrome” (Cowden syndrome, Bananyan-Riley-Ruvalcaba). The present article reports the wide spectrum of disease severity and extraintestinal manifestations, with a special focus on their potential to develop colorectal and other neoplasia. In the literature, the reported colorectal cancer risk for Juvenile Polyposis, Peutz-Jeghers and PTEN Hamartoma Tumor Syndromes are 39%-68%, 39%-57% and 18%, respectively. A review regarding cancer surveillance recommendations is also presented. PMID:25848489

  14. Perspectivas Futuras para o Observatório do Pico dos Dias

    NASA Astrophysics Data System (ADS)

    Bruch, Albert

    2004-02-01

    Com o Observatório Gemini plenamente operacional e o telescópio SOAR iniciando suas operações em breve, a astronomia observacional brasileira encontra-se no auge de uma transformação profunda que terá um impacto grave no Observatório do Pico dos Dias - OPD. Refletimos aqui sobre a natureza desse impacto e estratégias para manter a competitividade do OPD. Não queremos apresentar receitas prontas, mas idéias que poderão servir como base de discussão sobre o uso inteligente dos telescópios do OPD como parte do conjunto de instrumentos disponíveis à comunidade astronômica brasileira.

  15. A Direct Method to Extract Transient Sub-Gap Density of State (DOS) Based on Dual Gate Pulse Spectroscopy

    NASA Astrophysics Data System (ADS)

    Dai, Mingzhi; Khan, Karim; Zhang, Shengnan; Jiang, Kemin; Zhang, Xingye; Wang, Weiliang; Liang, Lingyan; Cao, Hongtao; Wang, Pengjun; Wang, Peng; Miao, Lijing; Qin, Haiming; Jiang, Jun; Xue, Lixin; Chu, Junhao

    2016-06-01

    Sub-gap density of states (DOS) is a key parameter to impact the electrical characteristics of semiconductor materials-based transistors in integrated circuits. Previously, spectroscopy methodologies for DOS extractions include the static methods, temperature dependent spectroscopy and photonic spectroscopy. However, they might involve lots of assumptions, calculations, temperature or optical impacts into the intrinsic distribution of DOS along the bandgap of the materials. A direct and simpler method is developed to extract the DOS distribution from amorphous oxide-based thin-film transistors (TFTs) based on Dual gate pulse spectroscopy (GPS), introducing less extrinsic factors such as temperature and laborious numerical mathematical analysis than conventional methods. From this direct measurement, the sub-gap DOS distribution shows a peak value on the band-gap edge and in the order of 1017-1021/(cm3·eV), which is consistent with the previous results. The results could be described with the model involving both Gaussian and exponential components. This tool is useful as a diagnostics for the electrical properties of oxide materials and this study will benefit their modeling and improvement of the electrical properties and thus broaden their applications.

  16. FlexyDos3D: a deformable anthropomorphic 3D radiation dosimeter: radiation properties

    NASA Astrophysics Data System (ADS)

    De Deene, Y.; Skyt, P. S.; Hil, R.; Booth, J. T.

    2015-02-01

    Three dimensional radiation dosimetry has received growing interest with the implementation of highly conformal radiotherapy treatments. The radiotherapy community faces new challenges with the commissioning of image guided and image gated radiotherapy treatments (IGRT) and deformable image registration software. A new three dimensional anthropomorphically shaped flexible dosimeter, further called ‘FlexyDos3D’, has been constructed and a new fast optical scanning method has been implemented that enables scanning of irregular shaped dosimeters. The FlexyDos3D phantom can be actuated and deformed during the actual treatment. FlexyDos3D offers the additional advantage that it is easy to fabricate, is non-toxic and can be molded in an arbitrary shape with high geometrical precision. The dosimeter formulation has been optimized in terms of dose sensitivity. The influence of the casting material and oxygen concentration has also been investigated. The radiophysical properties of this new dosimeter are discussed including stability, spatial integrity, temperature dependence of the dosimeter during radiation, readout and storage, dose rate dependence and tissue equivalence. The first authors Y De Deene and P S Skyt made an equivalent contribution to the experimental work presented in this paper.

  17. Computer-Assisted Instruction to Teach DOS Commands: A Pilot Study.

    ERIC Educational Resources Information Center

    McWeeney, Mark G.

    1992-01-01

    Describes a computer-assisted instruction (CAI) program used to teach DOS commands. Pretest and posttest results for 65 graduate students using the program are reported, and it is concluded that the CAI program significantly aided the students. Sample screen displays for the program and several questions from the pre/posttest are included. (nine…

  18. 76 FR 58074 - 30-Day Notice of Proposed Information Collection: DS-7001 and DS-7005, DOS-Sponsored Academic...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-09-19

    ...-7001 and DS- 7005, DOS-Sponsored Academic Exchange Program Application, OMB Control Number 1405-0138.... Title of Information Collection: DOS-Sponsored Academic Exchange Program Application. OMB Control Number... Academic Exchange Program. Estimated Number of Respondents: 7160 (For DS-7001, 3842 estimated; for DS-7005...

  19. 76 FR 25733 - 60-Day Notice of Proposed Information Collection: DS-7001 and DS-7005, DOS-Sponsored Academic...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-05-05

    ... and DS- 7005, DOS-Sponsored Academic Exchange Program Application, OMB Control Number 1405-0138 ACTION... Collection: DOS-Sponsored Academic Exchange Program Application. OMB Control Number: 1405-0138. Type of... Cultural Affairs, ECA/A/E/EUR. Form Number: DS-7001, DS-7005. Respondents: Applicants for the Academic...

  20. Adaptive Suspicious Prevention for Defending DoS Attacks in SDN-Based Convergent Networks

    PubMed Central

    Dao, Nhu-Ngoc; Kim, Joongheon; Park, Minho; Cho, Sungrae

    2016-01-01

    The convergent communication network will play an important role as a single platform to unify heterogeneous networks and integrate emerging technologies and existing legacy networks. Although there have been proposed many feasible solutions, they could not become convergent frameworks since they mainly focused on converting functions between various protocols and interfaces in edge networks, and handling functions for multiple services in core networks, e.g., the Multi-protocol Label Switching (MPLS) technique. Software-defined networking (SDN), on the other hand, is expected to be the ideal future for the convergent network since it can provide a controllable, dynamic, and cost-effective network. However, SDN has an original structural vulnerability behind a lot of advantages, which is the centralized control plane. As the brains of the network, a controller manages the whole network, which is attractive to attackers. In this context, we proposes a novel solution called adaptive suspicious prevention (ASP) mechanism to protect the controller from the Denial of Service (DoS) attacks that could incapacitate an SDN. The ASP is integrated with OpenFlow protocol to detect and prevent DoS attacks effectively. Our comprehensive experimental results show that the ASP enhances the resilience of an SDN network against DoS attacks by up to 38%. PMID:27494411

  1. Adaptive Suspicious Prevention for Defending DoS Attacks in SDN-Based Convergent Networks.

    PubMed

    Dao, Nhu-Ngoc; Kim, Joongheon; Park, Minho; Cho, Sungrae

    2016-01-01

    The convergent communication network will play an important role as a single platform to unify heterogeneous networks and integrate emerging technologies and existing legacy networks. Although there have been proposed many feasible solutions, they could not become convergent frameworks since they mainly focused on converting functions between various protocols and interfaces in edge networks, and handling functions for multiple services in core networks, e.g., the Multi-protocol Label Switching (MPLS) technique. Software-defined networking (SDN), on the other hand, is expected to be the ideal future for the convergent network since it can provide a controllable, dynamic, and cost-effective network. However, SDN has an original structural vulnerability behind a lot of advantages, which is the centralized control plane. As the brains of the network, a controller manages the whole network, which is attractive to attackers. In this context, we proposes a novel solution called adaptive suspicious prevention (ASP) mechanism to protect the controller from the Denial of Service (DoS) attacks that could incapacitate an SDN. The ASP is integrated with OpenFlow protocol to detect and prevent DoS attacks effectively. Our comprehensive experimental results show that the ASP enhances the resilience of an SDN network against DoS attacks by up to 38%.

  2. Paratesticular cysts with benign epithelial proliferations of wolffian origin.

    PubMed

    Nistal, Manuel; González-Peramato, Pilar; Serrano, Alvaro; Vega-Perez, Maria; De Miguel, Maria P; Regadera, Javier

    2005-08-01

    Paratesticular cysts with benign epithelial proliferations (BEPs) are rare. Only 10 cases were found in a series of 431 paratesticular cysts and were classified as follows: cystadenoma, 5; papilloma, 2; and hamartoma, 3. Four cystadenomas showed multiple papillae lined by CD10+ epithelial cells with hyperchromatic nuclei. The remaining lesion showed areas with a microcystic, glandular, cribriform pattern, with small, benign glands without atypia. Urothelial papilloma presented BEPs with cytokeratin (CK) 7+ and CD10+ and CK20- umbrella-like cells. The mural papilloma was lined by proliferative cylindrical cells exhibiting strong CK7 and CD10 expression. The 3 Wolffian hamartomas were characterized by strongly CD10+ epithelium surrounded by smooth muscle cells. The consistent CD10 expression in BEPs of paratesticular cysts suggests a Wolffian origin. The differential diagnosis of paratesticular cysts with BEP vs metastatic prostatic and primary borderline or malignant tumors is discussed.

  3. Research on a Denial of Service (DoS) Detection System Based on Global Interdependent Behaviors in a Sensor Network Environment

    PubMed Central

    Song, Jae-gu; Jung, Sungmo; Kim, Jong Hyun; Seo, Dong Il; Kim, Seoksoo

    2010-01-01

    This research suggests a Denial of Service (DoS) detection method based on the collection of interdependent behavior data in a sensor network environment. In order to collect the interdependent behavior data, we use a base station to analyze traffic and behaviors among nodes and introduce methods of detecting changes in the environment with precursor symptoms. The study presents a DoS Detection System based on Global Interdependent Behaviors and shows the result of detecting a sensor carrying out DoS attacks through the test-bed. PMID:22163475

  4. The trap DOS in small molecule organic semiconductors: A quantitative comparison of thin-film transistors with single crystals

    NASA Astrophysics Data System (ADS)

    Kalb, Wolfgang; Haas, Simon; Pernstich, Kurt; Mathis, Thomas; Batlogg, Bertram

    2010-03-01

    Our study shows that it is possible to reach one of the ultimate goals of organic electronics: organic field-effect transistors can be produced with trap densities as low as in the bulk of single crystals. Several analytical methods to calculate the spectral density of localized states in the band gap (trap DOS) from measured data were used to clarify, if the different methods lead to similar results. We then compared quantitatively trap DOS information from the literature, correcting for differences due to different calculation methods. In the bulk of single crystals the trap DOS is lower by several orders of magnitude than in thin films. The compilation of all data strongly suggests that structural defects at grain boundaries are the main cause of ``fast'' traps in TFT's made with vacuum-evaporated pentacene. For high-performance transistors made with small molecule semiconductors such as rubrene it is essential to reduce the dipolar disorder caused by water adsorbed on the gate dielectric. We will discuss to what degree band broadening due to the thermal fluctuations of the intermolecular transfer integral is reflected in the trap DOS very close (<0.15 eV) to the mobility edge.

  5. Calcium Isotopic Anomalies in the Allende CAIs and the Angrite Angra dos Reis

    NASA Astrophysics Data System (ADS)

    Chen, H. W.; Chen, J. C.; Lee, T.; Shen, J. J.

    2010-03-01

    Both negative 48Ca and 50Ti anomalies of the Angrite Angra dos Reis was identified in this study, and the result supported previous study of correlated negative 54Cr and 50Ti anomalies in achondrites.

  6. Low-Budget, Cost-Effective OCR: Optical Character Recognition for MS-DOS Micros.

    ERIC Educational Resources Information Center

    Perez, Ernest

    1990-01-01

    Discusses optical character recognition (OCR) for use with MS-DOS microcomputers. Cost effectiveness is considered, three types of software approaches to character recognition are explained, hardware and operation requirements are described, possible library applications are discussed, future OCR developments are suggested, and a list of OCR…

  7. 48 CFR 653.219-70 - DOS form DS-1910, Small Business Agency Review-Actions Above the Simplified Acquisition Threshold.

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... 48 Federal Acquisition Regulations System 4 2010-10-01 2010-10-01 false DOS form DS-1910, Small Business Agency Review-Actions Above the Simplified Acquisition Threshold. 653.219-70 Section 653.219-70 Federal Acquisition Regulations System DEPARTMENT OF STATE CLAUSES AND FORMS FORMS Prescription of Forms 653.219-70 DOS form DS-1910, Small...

  8. 48 CFR 653.219-70 - DOS form DS-1910, Small Business Agency Review-Actions Above the Simplified Acquisition Threshold.

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ... 48 Federal Acquisition Regulations System 4 2011-10-01 2011-10-01 false DOS form DS-1910, Small Business Agency Review-Actions Above the Simplified Acquisition Threshold. 653.219-70 Section 653.219-70 Federal Acquisition Regulations System DEPARTMENT OF STATE CLAUSES AND FORMS FORMS Prescription of Forms 653.219-70 DOS form DS-1910, Small...

  9. 48 CFR 653.219-70 - DOS form DS-1910, Small Business Agency Review-Actions Above the Simplified Acquisition Threshold.

    Code of Federal Regulations, 2014 CFR

    2014-10-01

    ... 48 Federal Acquisition Regulations System 4 2014-10-01 2014-10-01 false DOS form DS-1910, Small Business Agency Review-Actions Above the Simplified Acquisition Threshold. 653.219-70 Section 653.219-70 Federal Acquisition Regulations System DEPARTMENT OF STATE CLAUSES AND FORMS FORMS Prescription of Forms 653.219-70 DOS form DS-1910, Small...

  10. 48 CFR 653.219-70 - DOS form DS-1910, Small Business Agency Review-Actions Above the Simplified Acquisition Threshold.

    Code of Federal Regulations, 2013 CFR

    2013-10-01

    ... 48 Federal Acquisition Regulations System 4 2013-10-01 2013-10-01 false DOS form DS-1910, Small Business Agency Review-Actions Above the Simplified Acquisition Threshold. 653.219-70 Section 653.219-70 Federal Acquisition Regulations System DEPARTMENT OF STATE CLAUSES AND FORMS FORMS Prescription of Forms 653.219-70 DOS form DS-1910, Small...

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

    Gutierrez, O.; Schwartz, S.I.

    This book describes the diagnosis of liver tumors. Topics considered include general considerations, hepatocellular carcinoma, hepatoblastoma, cholangiocarcinoma, mesenchyomoma, sarcoma, hemangioma, hepatic cell adenoma, focal nodular hyperlasia (FNH), hamartoma, echinococcus cyst, abscess, AV fistula, hepatic artery aneurysm, metastatic carcinoma-colon, metastatic cholangiocarcinoma, metastatic melanoma, metastatic merkel cell and extrahepatic tumor.

  12. Using satellite images to monitor glacial-lake outburst floods: Lago Cachet Dos drainage, Chile

    USGS Publications Warehouse

    Friesen, Beverly A.; Cole, Christopher J.; Nimick, David A.; Wilson, Earl M.; Fahey, Mark J.; McGrath, Daniel J.; Leidich, Jonathan

    2015-01-01

    During 2008–2013, 14 GLOFs were released from Lago Cachet Dos and created environmental and safety concerns for downstream residents and to infrastructure. If GLOFs and the consequent headward erosion continue, the moraine that creates Lago Cachet Uno could be destabilized and breached, and the two lakes could merge. If the two lakes become connected, the volume of future GLOFs likely would be greater and thus cause longer and (or) more extensive flooding downstream. Additional GLOFs from Lago Cachet Dos are expected in the future, and continued environmental monitoring could provide an early warning system as well as scientific information that could increase our understanding of GLOFs and their consequences. GLOFs occur in glaciated areas around the world and remote sensing technologies can allow researchers to better understand—and potentially predict—future GLOF events.

  13. DoS detection in IEEE 802.11 with the presence of hidden nodes

    PubMed Central

    Soryal, Joseph; Liu, Xijie; Saadawi, Tarek

    2013-01-01

    The paper presents a novel technique to detect Denial of Service (DoS) attacks applied by misbehaving nodes in wireless networks with the presence of hidden nodes employing the widely used IEEE 802.11 Distributed Coordination Function (DCF) protocols described in the IEEE standard [1]. Attacker nodes alter the IEEE 802.11 DCF firmware to illicitly capture the channel via elevating the probability of the average number of packets transmitted successfully using up the bandwidth share of the innocent nodes that follow the protocol standards. We obtained the theoretical network throughput by solving two-dimensional Markov Chain model as described by Bianchi [2], and Liu and Saadawi [3] to determine the channel capacity. We validated the results obtained via the theoretical computations with the results obtained by OPNET simulator [4] to define the baseline for the average attainable throughput in the channel under standard conditions where all nodes follow the standards. The main goal of the DoS attacker is to prevent the innocent nodes from accessing the channel and by capturing the channel’s bandwidth. In addition, the attacker strives to appear as an innocent node that follows the standards. The protocol resides in every node to enable each node to police other nodes in its immediate wireless coverage area. All innocent nodes are able to detect and identify the DoS attacker in its wireless coverage area. We applied the protocol to two Physical Layer technologies: Direct Sequence Spread Spectrum (DSSS) and Frequency Hopping Spread Spectrum (FHSS) and the results are presented to validate the algorithm. PMID:25685510

  14. DoS detection in IEEE 802.11 with the presence of hidden nodes.

    PubMed

    Soryal, Joseph; Liu, Xijie; Saadawi, Tarek

    2014-07-01

    The paper presents a novel technique to detect Denial of Service (DoS) attacks applied by misbehaving nodes in wireless networks with the presence of hidden nodes employing the widely used IEEE 802.11 Distributed Coordination Function (DCF) protocols described in the IEEE standard [1]. Attacker nodes alter the IEEE 802.11 DCF firmware to illicitly capture the channel via elevating the probability of the average number of packets transmitted successfully using up the bandwidth share of the innocent nodes that follow the protocol standards. We obtained the theoretical network throughput by solving two-dimensional Markov Chain model as described by Bianchi [2], and Liu and Saadawi [3] to determine the channel capacity. We validated the results obtained via the theoretical computations with the results obtained by OPNET simulator [4] to define the baseline for the average attainable throughput in the channel under standard conditions where all nodes follow the standards. The main goal of the DoS attacker is to prevent the innocent nodes from accessing the channel and by capturing the channel's bandwidth. In addition, the attacker strives to appear as an innocent node that follows the standards. The protocol resides in every node to enable each node to police other nodes in its immediate wireless coverage area. All innocent nodes are able to detect and identify the DoS attacker in its wireless coverage area. We applied the protocol to two Physical Layer technologies: Direct Sequence Spread Spectrum (DSSS) and Frequency Hopping Spread Spectrum (FHSS) and the results are presented to validate the algorithm.

  15. Análise dos Conceitos Astronômicos Apresentados por Professores de Algumas Escolas Estaduais Brasileiras

    NASA Astrophysics Data System (ADS)

    Voelzke, Marcos Rincon; Gonzaga, Edson Pereira

    2011-12-01

    A razão para o desenvolvimento deste trabalho baseia-se no fato de que muitos professores da Educação Básica (EB) não lidam com conceitos relacionados à astronomia, e quando o fazem eles simplesmente seguem livros didáticos que podem conter erros conceituais. Como é de conhecimento geral a astronomia é um dos conteúdos a serem ensinados na EB fazendo parte dos Parâmetros Curriculares Nacionais e das Propostas Curriculares do Estado de São Paulo, mas é um fato, que vários pesquisadores apontam, a existência de muitos problemas no ensino da astronomia. Com o propósito de minimizar algumas dessas deficiências foi realizado um trabalho de pesquisa com a utilização de questionários pré e pós pesquisa, para tanto foi desenvolvido um Curso de Extensão Universitária para professores da Diretoria de Ensino Regional (DE) que abrange Mauá, Ribeirão Pires e Rio Grande da Serra (no Estado de São Paulo) com os seguintes objetivos: levantar concepções alternativas; subsidiar os professores por meio de palestras, debates e workshops, e verificar o sucesso da aprendizagem após o curso, adotando-se como referência, para a análise dos resultados, os dicionários de Língua Portuguesa (FERREIRA, 2004) e Enciclopédico de Astronomia e Astronáutica (MOURĀO, 1995). Portanto, dezesseis questões foram aplicadas antes e após o curso, assim pode-se verificar após a pesquisa que 100,0% dos professores sabiam os nomes das fases da Lua, 97,0% entenderam que o Sistema Solar é composto por oito planetas, 78,1% foram capazes de explicar como ocorre um eclipse lunar, um eclipse solar e um solstício, 72,7% sabiam como explicar a ocorrência das estações do ano; 64,5% explicaram corretamente a ocorrência do equinócio, 89,7% foram capazes de definir adequadamente o termo cometa; 63,6% definiram asteróide, 54,5% meteoro, 58,1% galáxia, e 42,4% planeta. Os resultados obtidos indicam uma aprendizagem significativa por parte dos participantes.

  16. SH3 domain-mediated binding of the Drk protein to Dos is an important step in signaling of Drosophila receptor tyrosine kinases.

    PubMed

    Feller, Stephan M; Wecklein, Heike; Lewitzky, Marc; Kibler, Eike; Raabe, Thomas

    2002-08-01

    Activation of the Sevenless (Sev) receptor tyrosine kinase (RTK) in the developing Drosophila eye is required for the specification of the R7 photoreceptor cell fate. Daughter of Sevenless (Dos), a putative multi-site adaptor protein, is a substrate of the Sev kinase and is known to associate with the tyrosine phosphatase Corkscrew (Csw). Binding of Csw to Dos depends on the Csw Src homology 2 (SH2) domains and is an essential step for signaling by the Sev RTK. Dos, however, lacks a recognizable phosphotyrosine interaction domain and it was previously unclear how it is recruited to the Sev receptor. Here it is shown that the SH2/SH3 domain adaptor protein Drk can provide this link. Drk binds with its SH2 domain to the autophosphorylated Sev receptor while the C-terminal SH3 domain is able to associate with Dos. The Drk SH3 domain binding motifs on Dos were mapped to two sites which do not conform the known Drk SH3 domain binding motif (PxxPxR) but instead have the consensus PxxxRxxKP. Mutational analysis in vitro and in vivo provided evidence that both Drk binding sites fulfil an important function in the context of Sev and Drosophila epidermal growth factor receptor mediated signaling processes.

  17. The spectrum of skin biopsies and excisions in a pediatric skin center.

    PubMed

    Theiler, Martin; Neuhaus, Kathrin; Kerl, Katrin; Weibel, Lisa

    2017-12-01

    Little is known about the spectrum of pediatric skin disorders requiring biopsy/excision, their indication, impact on further management, and the accuracy of clinical diagnosis. We aimed to address these questions in the patient population seen at our Swiss University referral center for Pediatric Dermatology and Plastic Surgery. All skin biopsies/excisions performed in patients aged ≤ 16 years over a period of 2 years were retrospectively analyzed. A total of 506 samples were included. The majority of biopsies/excisions (n = 413, 82%) was performed for tumors, cysts, and hamartomas and 18% for other skin conditions. Malignant tumors were found in 12 samples (2%) from four patients. In 121 (24%) patients, the histopathology had an important impact on patient management. In 80 (16%) cases, the pathology did not match with the clinical diagnosis. In 382 (75%) cases, excision was the treatment of choice. Of these, the indication for surgery was based on patient's request in 181 (47%) cases. Surgical interventions for pediatric skin disorders are performed for diagnostic and therapeutic reasons. In this cohort, histopathology was essential for treatment in one quarter of cases. Skin tumors, cysts, and hamartomas often require excision during childhood, with families' request and esthetic considerations playing an important role. What is Known: • The spectrum of pediatric skin conditions has been studied in outpatient, inpatient, and emergency settings. • In contrast, no data exist on the spectrum of pediatric skin disorders undergoing biopsy/excision specifically. What is New: • We analyze biopsies/excisions in children, focusing on diagnosis, indication, and impact on patient management. • Surgical interventions for skin disorders in children are often performed for tumors and hamartomas with esthetic considerations playing a relevant role. If used for diagnostic purposes, they are often performed to confirm or rule out severe skin disease.

  18. Entre Dos Mundos/Between Two Worlds: Youth Violence Prevention for Acculturating Latino Families

    ERIC Educational Resources Information Center

    Smokowski, Paul R.; Bacallao, Martica

    2009-01-01

    Objective: This study evaluated the efficacy of Entre Dos Mundos/Between Two Worlds (EDM) prevention for Latino adolescents. Method: In an experimental trial to compare implementation formats, 41 Latino families were randomly assigned to EDM action-oriented skills training groups, and 47 families were randomly assigned to unstructured EDM support…

  19. Quality control and assurance for validation of DOS/I measurements

    NASA Astrophysics Data System (ADS)

    Cerussi, Albert; Durkin, Amanda; Kwong, Richard; Quang, Timothy; Hill, Brian; Tromberg, Bruce J.; MacKinnon, Nick; Mantulin, William W.

    2010-02-01

    Ongoing multi-center clinical trials are crucial for Biophotonics to gain acceptance in medical imaging. In these trials, quality control (QC) and assurance (QA) are key to success and provide "data insurance". Quality control and assurance deal with standardization, validation, and compliance of procedures, materials and instrumentation. Specifically, QC/QA involves systematic assessment of testing materials, instrumentation performance, standard operating procedures, data logging, analysis, and reporting. QC and QA are important for FDA accreditation and acceptance by the clinical community. Our Biophotonics research in the Network for Translational Research in Optical Imaging (NTROI) program for breast cancer characterization focuses on QA/QC issues primarily related to the broadband Diffuse Optical Spectroscopy and Imaging (DOS/I) instrumentation, because this is an emerging technology with limited standardized QC/QA in place. In the multi-center trial environment, we implement QA/QC procedures: 1. Standardize and validate calibration standards and procedures. (DOS/I technology requires both frequency domain and spectral calibration procedures using tissue simulating phantoms and reflectance standards, respectively.) 2. Standardize and validate data acquisition, processing and visualization (optimize instrument software-EZDOS; centralize data processing) 3. Monitor, catalog and maintain instrument performance (document performance; modularize maintenance; integrate new technology) 4. Standardize and coordinate trial data entry (from individual sites) into centralized database 5. Monitor, audit and communicate all research procedures (database, teleconferences, training sessions) between participants ensuring "calibration". This manuscript describes our ongoing efforts, successes and challenges implementing these strategies.

  20. A mouse model of TSC1 reveals sex-dependent lethality from liver hemangiomas, and up-regulation of p70S6 kinase activity in Tsc1 null cells.

    PubMed

    Kwiatkowski, David J; Zhang, Hongbing; Bandura, Jennifer L; Heiberger, Kristina M; Glogauer, Michael; el-Hashemite, Nisreen; Onda, Hiroaki

    2002-03-01

    Tuberous sclerosis (TSC) is a autosomal dominant genetic disorder caused by mutations in either TSC1 or TSC2, and characterized by benign hamartoma growth. We developed a murine model of Tsc1 disease by gene targeting. Tsc1 null embryos die at mid-gestation from a failure of liver development. Tsc1 heterozygotes develop kidney cystadenomas and liver hemangiomas at high frequency, but the incidence of kidney tumors is somewhat lower than in Tsc2 heterozygote mice. Liver hemangiomas were more common, more severe and caused higher mortality in female than in male Tsc1 heterozygotes. Tsc1 null embryo fibroblast lines have persistent phosphorylation of the p70S6K (S6K) and its substrate S6, that is sensitive to treatment with rapamycin, indicating constitutive activation of the mTOR-S6K pathway due to loss of the Tsc1 protein, hamartin. Hyperphosphorylation of S6 is also seen in kidney tumors in the heterozygote mice, suggesting that inhibition of this pathway may have benefit in control of TSC hamartomas.

  1. RighTime: A real time clock correcting program for MS-DOS-based computer systems

    NASA Technical Reports Server (NTRS)

    Becker, G. Thomas

    1993-01-01

    A computer program is described which effectively eliminates the misgivings of the DOS system clock in PC/AT-class computers. RighTime is a small, sophisticated memory-resident program that automatically corrects both the DOS system clock and the hardware 'CMOS' real time clock (RTC) in real time. RighTime learns what corrections are required without operator interaction beyond the occasional accurate time set. Both warm (power on) and cool (power off) errors are corrected, usually yielding better than one part per million accuracy in the typical desktop computer with no additional hardware, and RighTime increases the system clock resolution from approximately 0.0549 second to 0.01 second. Program tools are also available which allow visualization of RighTime's actions, verification of its performance, display of its history log, and which provide data for graphing of the system clock behavior. The program has found application in a wide variety of industries, including astronomy, satellite tracking, communications, broadcasting, transportation, public utilities, manufacturing, medicine, and the military.

  2. Reagent-based DOS: developing a diastereoselective methodology to access spirocyclic- and fused heterocyclic ring systems.

    PubMed

    Damerla, V Surendra Babu; Tulluri, Chiranjeevi; Gundla, Rambabu; Naviri, Lava; Adepally, Uma; Iyer, Pravin S; Murthy, Y L N; Prabhakar, Nampally; Sen, Subhabrata

    2012-10-01

    Herein, we report a diversity-oriented-synthesis (DOS) approach for the synthesis of biologically relevant molecular scaffolds. Our methodology enables the facile synthesis of fused N-heterocycles, spirooxoindolones, tetrahydroquinolines, and fused N-heterocycles. The two-step sequence starts with a chiral-bicyclic-lactam-directed enolate-addition/substitution step. This step is followed by a ring-closure onto the built-in scaffold electrophile, thereby leading to stereoselective carbocycle- and spirocycle-formation. We used in silico tools to calibrate our compounds with respect to chemical diversity and selected drug-like properties. We evaluated the biological significance of our scaffolds by screening them in two cancer cell-lines. In summary, our DOS methodology affords new, diverse scaffolds, thereby resulting in compounds that may have significance in medicinal chemistry. Copyright © 2012 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.

  3. The Intraplate Seismogenic Zone of Porto dos Gauchos in the Amazon Craton, Brazil

    NASA Astrophysics Data System (ADS)

    Barros, L. V.; Assumpcao, M. S.; Quintero, R. Q.

    2007-05-01

    The Porto dos Gauchos Seismogenic Zone (PGSZ), in the center north of Mato Grosso State, in the contact between the southern Amazonian Craton and northern Parecis Basin, represents one of the most important area of seismic activity in Brazil, with the largest magnitude ever observed in the stable continent of the South America plate (6.2 mb on January 31, 1955). Focal mechanism studies indicated a pure reverse faulting regime with compressional SHmax. oriented in SE-NW direction. After the 1955 earthquake, located in Serra do Tombador, a recurrent seismicity has been detected in Porto dos Gauchos, 100 km northeast of Serra do Tombador. No recent events have been detected in the area of the 1955 epicenter, suggesting a long recurrence time or mislocation of Serra do Tombador earthquake. The Porto dos Gauchos recurrent seismicity has been observed since 1959, when a 4.5 macroseismic estimated magnitude was felt by local inhabitants settled in that remote area two years earlier. In subsequent years, with deployment of regional stations in Brazilian Amazon region earthquakes were detected in 1981 (3.8 mb), 1989 (3.3 mb), 1993 (3.8 mb), 1996 (4.4 mb), 1997 (3.3 mb), and finally on March 10, 1998 (5.1 mb). The aftershocks of 1998 main shock were studied with a local network with up to eight 3- component stations. Such network detected more than 2500 events until December of 2002, when the network was deactivated, but only 100 were accurately located. Based on this set of events and a controlled source experiment we determined a 1-D velocity model for the area, a composite focal mechanism with P wave polarities, spectral analysis studies to estimate the source dimension, stress drop and moment magnitudes for the main shock and some others events of the set. On March 23, 2005 another shock occurred in the same seismogenic area of Porto dos Gauchos, with magnitude 4.7 to 5.0. One week later five stations were installed again to monitor the aftershock activity, detecting

  4. A natural product based DOS library of hybrid systems.

    PubMed

    Prabhu, Ganesh; Agarwal, Shalini; Sharma, Vijeta; Madurkar, Sanjay M; Munshi, Parthapratim; Singh, Shailja; Sen, Subhabrata

    2015-05-05

    Here we described a natural product inspired modular DOS strategy for the synthesis of a library of hybrid systems that are structurally and stereochemically disparate. The main scaffold is a pyrroloisoquinoline motif, that is synthesized from tandem Pictet-Spengler lactamization. The structural diversity is generated via "privileged scaffolds" that are attached at the appropriate site of the motif. Screening of the library compounds for their antiplasmodial activity against chloroquine sensitive 3D7 cells indicated few compounds with moderate activity (20-50 μM). A systematic comparison of structural intricacy between the library members and a natural product dataset obtained from ZINC(®) revealed comparable complexity. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

  5. Fast interrupt platform for extended DOS

    NASA Technical Reports Server (NTRS)

    Duryea, T. W.

    1995-01-01

    Extended DOS offers the unique combination of a simple operating system which allows direct access to the interrupt tables, 32 bit protected mode access to 4096 MByte address space, and the use of industry standard C compilers. The drawback is that fast interrupt handling requires both 32 bit and 16 bit versions of each real-time process interrupt handler to avoid mode switches on the interrupts. A set of tools has been developed which automates the process of transforming the output of a standard 32 bit C compiler to 16 bit interrupt code which directly handles the real mode interrupts. The entire process compiles one set of source code via a make file, which boosts productivity by making the management of the compile-link cycle very simple. The software components are in the form of classes written mostly in C. A foreground process written as a conventional application which can use the standard C libraries can communicate with the background real-time classes via a message passing mechanism. The platform thus enables the integration of high performance real-time processing into a conventional application framework.

  6. Use of Remote Sensing and Local Knowledge for Geoconservation of Regiao dos Lagos, Brazil

    NASA Astrophysics Data System (ADS)

    Avelar, S.; Vasconcelos, G.; Mansur, K. L.; Anjos, S. C.

    2013-12-01

    A series of lagoons can be found along the coastline of Rio de Janeiro, in the so-called Regiao dos Lagos. The lagoons differ in size, physicochemical, sedimentological and biological characteristics. Rare examples of litifying microbialites that produce stromatolites, the oldest fossils on Earth, can be found living in this lagoon system. The occurrence of stromatolites in the region is of great scientific interest because it enables the study of possible analogues of the earliest life on Earth. However, this region has been suffering from intense human activities and degradations. Geoconservation planning requires an assessment of the characteristics of the region and its potential threats. The primary goal of this study is to assess physical environmental changes and anthropogenic impacts over the last four decades in Regiao dos Lagos. Using a broad integrative assessment combining remote sensing, GIS, field studies and local knowledge of communities, land-cover and land-use classes were identified, as well as the main human activities impacting the environment. The seasonal and weekend tourism and urban sprawl in this coastal area of Rio de Janeiro triggers the occupation of new areas and the removal of natural vegetation, especially on lagoon margins. This disorderly occupation by an ever increasing population, with both legal and illegal constructions and the subsequent overload of the local infrastructure, e.g. increase of electrical energy consumption, volume of vehicles, pollution in air, water and soil and problems with water supply and wastewater treatment, are hastening the gradual degradation of the lake ecosystem. The main driving forces to environmental changes over the last four decades in Regiao dos Lagos were the change of dense vegetation, saline and bare soil classes into built-up areas, adding to the poor waste treatment and inadequate sewage disposal. This analysis provides a basis for a better control of anthropogenic impacts and

  7. Document image archive transfer from DOS to UNIX

    NASA Technical Reports Server (NTRS)

    Hauser, Susan E.; Gill, Michael J.; Thoma, George R.

    1994-01-01

    An R&D division of the National Library of Medicine has developed a prototype system for automated document image delivery as an adjunct to the labor-intensive manual interlibrary loan service of the library. The document image archive is implemented by a PC controlled bank of optical disk drives which use 12 inch WORM platters containing bitmapped images of over 200,000 pages of medical journals. Following three years of routine operation which resulted in serving patrons with articles both by mail and fax, an effort is underway to relocate the storage environment from the DOS-based system to a UNIX-based jukebox whose magneto-optical erasable 5 1/4 inch platters hold the images. This paper describes the deficiencies of the current storage system, the design issues of modifying several modules in the system, the alternatives proposed and the tradeoffs involved.

  8. The extratemporal lobe epilepsies in the epilepsy monitoring unit

    PubMed Central

    Dash, Deepa; Tripathi, Manjari

    2014-01-01

    Extratemporal lobe epilepsies (ETLE) are characterized by the epileptogenic foci outside the temporal lobe. They have a wide spectrum of semiological presentation depending upon the site of origin. They can arise from frontal, parietal, occipital lobes and from hypothalamic hamartoma. We discuss in this review the semiology of different types of ETLE encountered in the epilepsy monitoring unit. PMID:24791090

  9. Cystic tumors of the liver: A practical approach

    PubMed Central

    Poggio, Paolo Del; Buonocore, Marco

    2008-01-01

    Biliary cyst tumors (cystadenoma and cystadeno-carcinoma) are an indication for liver resection. They account for only 5% of all solitary cystic lesions of the liver, but differential diagnosis with multiloculated or complicated biliary cysts, atypical hemangiomas, hamartomas and lymphangiomas may be difficult. The most frequent challenge is to differentiate biliary cyst tumors from hemorrhagic cysts. Computerized tomography (CT) and magnetic resonance imaging (MRI) are often not diagnostic and in these cases fine needle aspiration (FNA) is used to confirm the presence of atypical biliary cells. FNA, however, lacks adequate sensitivity and specificity and should always be used in conjunction with imaging. Pre-operative differentiation of cystadenoma from cystadenocarcinoma is impossible and surgery must be performed if a biliary cyst tumor is suspected. When multiple cystic lesions are observed throughout the liver parenchyma, it is important to exclude liver metastasis, of which colonic cancer is the most common primary site. Multiple biliary hamartomas (von Meyenburg complex) can appear as a mixture of solid and cystic lesions and can be confused with cystic metastasis. Strong and uniform T2 hyperintensity on MRI is usually diagnostic, but occasionally a percutaneous biopsy may be required. PMID:18595127

  10. Neurofibromatosis: an update of ophthalmic characteristics and applications of optical coherence tomography

    PubMed Central

    Abdolrahimzadeh, Barmak; Piraino, Domenica Carmen; Albanese, Giorgio; Cruciani, Filippo; Rahimi, Siavash

    2016-01-01

    Neurofibromatosis (NF) is a multisystem disorder and tumor predisposition syndrome caused by genetic mutation on chromosome 17-17q11.2 in NF type 1 (NF1), and on chromosome 22-22q12.2 in NF type 2. The disorder is characterized by considerable heterogeneity of clinical expression. NF1 is the form with the most characteristic ocular manifestations. Lisch nodules of the iris are among the well-known diagnostic criteria for the disease. Glaucoma and associated globe enlargement have been described in a significant proportion of patients with NF1 and orbital–facial involvement. Optic nerve glioma may cause strabismus and proptosis, and palpebral neurofibroma may reach considerable size and occasionally show malignant transformation. Near infrared reflectance has greatly contributed to enhancing our knowledge on choroidal alterations in NF1. Indeed, some authors have proposed to include these among the diagnostic criteria. Optical coherence tomography has given new insight on retinal alterations and is a noninvasive tool in the management of optic nerve gliomas in children. Ocular manifestations in NF type 2 can range from early-onset cataracts in up to 80% of cases to optic nerve hamartomas and combined pigment epithelial and retinal hamartomas. PMID:27257370

  11. Nevus Lipomatosus Cutaneous Superficialis With Perifollicular Fibromas.

    PubMed

    Anzai, Alessandra; Halpern, Ilana; Rivitti-Machado, Maria Cecília

    2015-09-01

    Nevus lipomatosus cutaneous superficialis (NLCS) is a rare cutaneous hamartoma characterized by groups of mature fat cells in the dermis. The authors report a case of a dark-skinnned, 6-year-old, Brazilian girl with 1 lesion in the right upper chest, extending to the neck, since 1 year of age. The dermatological examination consisted of skin-colored and yellowish follicular papules, isolated and grouped into a papillomatous plaque. The biopsy revealed ectopic mature adipose tissue in the dermis, compatible with NLCS, perifollicular well-circumscribed fibroblast and collagen proliferation, and decreased elastic fibers in the papillary and upper reticular dermis. The finding of perifollicular fibrosis in NLCS is not common, having been first described in 2003. The perifollicular fibrosis is characteristic of other rare hamartoma, the perifollicular fibroma, but is also present in fibrofolliculomas or trichodiscomas, angiofibromas, and fibrotic papules on the face of tuberous sclerosis and postinflammatory fibrosis. This case is very similar to the first described case of NLCS with perifollicular fibrosis, and the authors believe that this is the second case of this entity, but they propose to name it NCLS with perifollicular fibromas, which better describes the nature of this lesion.

  12. “Click, Click, Cyclize”: A DOS Approach to Sultams Utilizing Vinyl Sulfonamide Linchpins

    PubMed Central

    Zhou, Aihua; Rayabarapu, Dinesh; Hanson, Paul R.

    2009-01-01

    A diversity-oriented synthesis (DOS) strategy termed “Click, Click, Cyclize” is reported. This approach relies on functional group (FG) pairing between a vinyl sulfonamide and an array of functional groups to synthesize skeletally diverse sultams. Several FG pairing pathways on central tertiary vinyl sulfonamide linchpins have been developed including intramolecular Heck, aza-Michael, ring-closing enyne metathesis, Pauson—Khand, and chemoselective oxidation/Baylis—Hillman reactions. PMID:19115841

  13. Structural Basis for TSC-1 TSC-2 Complex Formation

    DTIC Science & Technology

    2008-03-01

    mental retardation and autism . Brain lesions include cerebral cortical tubers, subependymal nodules, and retinal hamartomas. Patients often develop...Rheb) (6,7). Rheb activates the mammalian target of Rapamycin ( mTOR ) and inactivates B-Raf (7). Activation of mTOR leads to increased protein...activity and activation of mTOR . Several unrelated patients with tuberous sclerosis have point mutations in the TSC2 GAP domain that influence the

  14. Qual o Papel da Escola e da Mídia na Obtenção dos Conhecimentos Astronômicos?

    NASA Astrophysics Data System (ADS)

    da Cunha, W. S.; Voelzke, M. R.; Amaral, L. H.

    2007-08-01

    É amplamente reconhecido que as pessoas em geral têm grande fascínio e interesse pela Astronomia. Por outro lado, o conteúdo dessa área incluído no ensino formal está longe de ser abrangente e suficiente para suprir a demanda. Esse interesse permite aperfeiçoar a divulgação e o ensino não formal de Astronomia através da mídia aliando a adequação do conteúdo à expectativa das pessoas. A reforma do Currículo Básico da Escola Pública da maioria dos Estados brasileiros tem introduzido Astronomia desde a pré-escola até o Ensino Médio. Num estudo realizado com 1180 alunos do Ensino Médio de seis escolas estaduais, revelou-se através da aplicação de um formulário contendo questões que abordavam conhecimentos básicos em Astronomia, como foram adquiridos tais conhecimentos e à infra-estrutura da escola. Para 66,1% dos entrevistados o professor já fez alguma apresentação a respeito de Astronomia. O presente estudo revelou também que 54,4% dos alunos entrevistados adquiriram seus conhecimentos astronômicos na escola, 18,4% através da televisão e 27,1% afirmaram que seus conhecimentos foram obtidos através de outros tipos de mídia (internet, livros didáticos, filmes e revistas). Embora o estudo revele claramente que há certo equilíbrio no meio em que os alunos obtiveram seus conhecimentos astronômicos, a mídia busca não somente sua difusão, mas a disponibilização desse conhecimento de maneira correta, que às vezes, não é atingido, apresentando conceitos sem fundamentação e incorretos. Por outro lado, no dia-a-dia escolar, é comum encontrar professores que vêm para as aulas com concepções prévias, que podem diferir substancialmente das idéias a serem ensinadas, dificultando o aprendizado de futuros conceitos científicos. Uma avaliação criteriosa, avaliando as dificuldades dos professores em determinar o sentido correto dos conceitos e observando a metodologia que utilizam no ensino, poderá proporcionar uma melhor

  15. An MS-DOS-based program for analyzing plutonium gamma-ray spectra

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

    Ruhter, W.D.; Buckley, W.M.

    1989-09-07

    A plutonium gamma-ray analysis system that operates on MS-DOS-based computers has been developed for the International Atomic Energy Agency (IAEA) to perform in-field analysis of plutonium gamma-ray spectra for plutonium isotopics. The program titled IAEAPU consists of three separate applications: a data-transfer application for transferring spectral data from a CICERO multichannel analyzer to a binary data file, a data-analysis application to analyze plutonium gamma-ray spectra, for plutonium isotopic ratios and weight percents of total plutonium, and a data-quality assurance application to check spectral data for proper data-acquisition setup and performance. Volume 3 contains the software listings for these applications.

  16. On securing wireless sensor network--novel authentication scheme against DOS attacks.

    PubMed

    Raja, K Nirmal; Beno, M Marsaline

    2014-10-01

    Wireless sensor networks are generally deployed for collecting data from various environments. Several applications specific sensor network cryptography algorithms have been proposed in research. However WSN's has many constrictions, including low computation capability, less memory, limited energy resources, vulnerability to physical capture, which enforce unique security challenges needs to make a lot of improvements. This paper presents a novel security mechanism and algorithm for wireless sensor network security and also an application of this algorithm. The proposed scheme is given to strong authentication against Denial of Service Attacks (DOS). The scheme is simulated using network simulator2 (NS2). Then this scheme is analyzed based on the network packet delivery ratio and found that throughput has improved.

  17. A case of cord capillary hemangioma of the spleen: a recently proven true neoplasm.

    PubMed

    Tajima, Shogo; Koda, Kenji

    2015-05-01

    Cord capillary hemangioma (CCH) of the spleen is an extremely rare lesion. Once classified under splenic hamartoma, CCH is now differentiated from hamartoma, as its clonality has recently been proven. Herein, we present the case of an incidentally found CCH, measuring 6 × 5.5 × 5 cm, in a 45-year-old man. He underwent splenectomy and has been recurrence-free for 8 years. Macroscopically, the cut surface of the mass showed a central stellate scar with peripheral interspersed reddish areas of variable sizes and intervening fibrous bands. Microscopically, the mass was well demarcated from the splenic tissue. The macroscopic reddish areas were found to correspond to nodules of various sizes, which were predominantly composed of CD34-expressing capillaries without the presence of CD8-expressing sinuses. The relative proportion of these two types of vessels distinguishes CCH from sclerosing angiomatoid nodular transformation (SANT), as SANT shows the apparent presence of CD8-expressing sinuses. In longstanding cases of SANT, sinuses might become ambiguous and the differences between CCH and SANT might become subtle. Nonetheless, immunohistochemistry should be performed when these conditions are suspected, since accurate distinction is usually achieved as a result. © 2015 Japanese Society of Pathology and Wiley Publishing Asia Pty Ltd.

  18. Ocular Alterations in a Rare Case of Segmental Neurofibromatosis Type 1 with a Non-Classified Mutational Variant of the NF-1 Gene.

    PubMed

    Abdolrahimzadeh, Solmaz; Piraino, Domenica Carmen; Plateroti, Rocco; Scuderi, Gianluca; Recupero, Santi Maria

    2016-06-01

    Neurofibromatosis type 1 (NF-1) is an autsomal dominant disorder which can occasionally result from somatic mosaicism and manifest as segmental forms of the disease. A 37-year-old woman with ascertained NF-1, based on clinical diagnostic criteria and genetic analysis, was referred for ophthalmological evaluation. Genetic analysis, magnetic resonance imaging (MRI), complete ophthalmological examination, and near infrared reflectance (NIR) images at 815 nm of the retina were obtained. Genetic analysis revealed a non-classified mutational variant of the NF-1 gene identified as NM_000267.3:c2084T > C (p.Leu695Pro.T). MRI demonstrated non-symptomatic bilateral optic nerve gliomas. The only cutaneous sign was a subcutaneous neurofibroma of the posterior cervical region. Slit-lamp examination showed bilateral Lisch nodules. NIR images of the retina did not show any choroidal hamartomas. We report a rare case of segmental neurofibromatosis with a non-classified mutational variant of the NF-1 gene described in only one previous case in the literature. The patient presented with clinical features of NF-1 localized to the head and neck region, compatible with diagnosis of segmental NF-1. Interestingly, ocular manifestations included bilateral optic nerve gliomas and Lisch nodules, but no choroidal hamartomas.

  19. Fat-containing soft-tissue masses in children.

    PubMed

    Sheybani, Elizabeth F; Eutsler, Eric P; Navarro, Oscar M

    2016-12-01

    The diagnosis of soft-tissue masses in children can be difficult because of the frequently nonspecific clinical and imaging characteristics of these lesions. However key findings on imaging can aid in diagnosis. The identification of macroscopic fat within a soft-tissue mass narrows the differential diagnosis considerably and suggests a high likelihood of a benign etiology in children. Fat can be difficult to detect with sonography because of the variable appearance of fat using this modality. Fat is easier to recognize using MRI, particularly with the aid of fat-suppression techniques. Although a large portion of fat-containing masses in children are adipocytic tumors, a variety of other tumors and mass-like conditions that contain fat should be considered by the radiologist confronted with a fat-containing mass in a child. In this article we review the sonographic and MRI findings in the most relevant fat-containing soft-tissue masses in the pediatric age group, including adipocytic tumors (lipoma, angiolipoma, lipomatosis, lipoblastoma, lipomatosis of nerve, and liposarcoma); fibroblastic/myofibroblastic tumors (fibrous hamartoma of infancy and lipofibromatosis); vascular anomalies (involuting hemangioma, intramuscular capillary hemangioma, phosphate and tensin homologue (PTEN) hamartoma of soft tissue, fibro-adipose vascular anomaly), and other miscellaneous entities, such as fat necrosis and epigastric hernia.

  20. Differentiating the mTOR inhibitors everolimus and sirolimus in the treatment of tuberous sclerosis complex

    PubMed Central

    MacKeigan, Jeffrey P.; Krueger, Darcy A.

    2015-01-01

    Tuberous sclerosis complex (TSC) is a genetic autosomal dominant disorder characterized by benign tumor-like lesions, called hamartomas, in multiple organ systems, including the brain, skin, heart, kidneys, and lung. These hamartomas cause a diverse set of clinical problems based on their location and often result in epilepsy, learning difficulties, and behavioral problems. TSC is caused by mutations within the TSC1 or TSC2 genes that inactivate the genes' tumor-suppressive function and drive hamartomatous cell growth. In normal cells, TSC1 and TSC2 integrate growth signals and nutrient inputs to downregulate signaling to mammalian target of rapamycin (mTOR), an evolutionarily conserved serine-threonine kinase that controls cell growth and cell survival. The molecular connection between TSC and mTOR led to the clinical use of allosteric mTOR inhibitors (sirolimus and everolimus) for the treatment of TSC. Everolimus is approved for subependymal giant cell astrocytomas and renal angiomyolipomas in patients with TSC. Sirolimus, though not approved for TSC, has undergone considerable investigation to treat various aspects of the disease. Everolimus and sirolimus selectively inhibit mTOR signaling with similar molecular mechanisms, but with distinct clinical profiles. This review differentiates mTOR inhibitors in TSC while describing the molecular mechanisms, pathogenic mutations, and clinical trial outcomes for managing TSC. PMID:26289591

  1. Transitional cell carcinoma of the retrorectal space arisen in tailgut cyst: a case report and review of the literature.

    PubMed

    Vinciguerra, Gian Luca Rampioni; Mercantini, Paolo; La Torre, Marco; Pilozzi, Emanuela; Ziparo, Vincenzo; Vecchione, Andrea

    2014-05-01

    Tailgut cysts, also known as retrorectal cystic hamartomas, are congenital lesions derived by an abnormal remnant of the postanal primitive hindgut, consisting of unilocular or multilocular cysts usually lined by squamous, transitional, or glandular epithelium. Malignant transformation is an uncommon event, and it mainly involves the neuroendocrine or glandular epithelium; other histotypes are sporadic. Here, we report, for the first time, the clinicopathological features of a transitional cell carcinoma that arose in a tailgut cyst.

  2. Congenital Panfollicular Nevus in a 6-Month-Old Girl.

    PubMed

    Duan, Linna; Antaya, Richard J; Ko, Christine J; Narayan, Deepak

    2016-01-01

    A 6-month-old girl presented with an irregular nodule on the right lateral neck. The lesion, which superficially resembled a lymphangioma or mastocytoma, had been present since birth but had recently been growing. The patient was referred for surgical resection. Microscopic findings were compatible with a recently described rare hamartoma termed "panfollicular nevus," which is a well-circumscribed lesion containing aberrantly differentiated, clustered hair follicles in high density. © 2016 Wiley Periodicals, Inc.

  3. Brunner’s gland adenoma of duodenum: report of two cases

    PubMed Central

    Lu, Li; Li, Ruotong; Zhang, Guojing; Zhao, Zhicheng; Fu, Weihua; Li, Weidong

    2015-01-01

    Brunner’s gland adenoma is a rare tumor of the duodenum and might also be an unusual cause of gastrointestinal bleeding or obstruction. The pathogenesis of Brunner gland hamartoma of the duodenum is unknown. We report two cases of Brunner’s gland adenoma. Surgical resection was carried out because the tumor size was big in both cases and one accompanied with bleeding. Pathological examination revealed submucosal nodular hyperplasia of the Brunner’s glands. PMID:26261670

  4. Combinatorial Therapy Approaches for NF2-Deficient Meningiomas

    DTIC Science & Technology

    2013-06-01

    led to the formation of hepatocellular carcinoma and bile duct hamartoma, strongly suggesting a role for theHippo pathway in carcinogenesis. The core...CNS Oncology, Vol. 1, No. 2, Pages 113-115, 2012 mouse liver , Zhang and colleagues defined a functional role between Merlin/NF2 tumor...suppressor and the Hippo pathway. The inactivation of Nf2 in the mouse liver led to YAP1 activation and to the formation of hepatocellular carcinoma [6

  5. Sm-Nd-Pu timepieces in the Angra dos Reis meteorite

    NASA Technical Reports Server (NTRS)

    Lugmair, G. W.; Marti, K.

    1977-01-01

    Isotope ratios and age estimations are presented for the ultramafic pyroxenite Angra dos Reis meteorite. A pyroxene-phosphate internal isochron age of 4.55 plus or minus 0.04 eons was obtained, in agreement with reported Pb-Pb ages. It is suggested that Sm-146 decay led to an increase in the ratio of Nd-142 to Nd-144 in pyroxene, which is greater than the ratio of the Nd isotopes in phosphates. The effect of Pu-244 on the fission xenon components in pyroxene and phosphate is examined. The ratios of Pu-244 to Nd, U-238, and Th-232 are compared for pyroxene and phosphate separates. The exposure age of the meteorite, as obtained by the Kr-81 to Kr-83 method, was found to be 55.5 plus or minus 1.2 million years.

  6. The influence of climate change on wine production - the case of the Touriga Nacional grape variety (Quinta dos Termos, Portugal)

    NASA Astrophysics Data System (ADS)

    Fonseca, João

    2017-04-01

    The regional and local climate, heavily influenced by global climate change, has strong implications for agriculture. Wine production which has specific characteristics in terms of climate and soil is undoubtedly one of the economic activities strongly influenced by climate change. Quinta dos Termos located in Beira Interior (Belmonte, Portugal) is the largest wine producer in the DOC Beira Interior region, producing premium to hiper premium wines of excellence, marketed at both national and international levels, and cultivates the vineyards according to the rules of Integrated Crop Management. Moreover, grapes are free from herbicides, pesticides or any other chemicals that can be harmful to the environment and health. These factors have contributed to the socio-economic development of the region, creating wealth, favoring employment and promoting tourism. The quality of the wines produced by Quinta dos Termos result from its terroir, given its granite region, the sun exposure, the wind protection, the atmospheric humidity and temperature, the soil water content, the mineralogical/organic composition and soil porosity. These factors favor unique conditions for the cultivation of Touriga Nacional grape variety, which is recognized by its extremely complex color and aroma, which allows the production of wines with great balance and a good ageing potential. Touriga Nacional, a red grape variety of Portuguese origin with high qualitative excellence and reputation and much appreciated worldwide, is versatile to several types of soils and resistant to high thermal amplitudes. Nevertheless, the climatic changes that has been gradually verified, the type of crop management, and in particular the reputation of Touriga Nacional grape variety, may be compromised in the long term, given that these characteristics are strongly influenced by the climate and soil. Aware of that, Quinta dos Termos has been performing a monitoring of the vineyards in terms of pedological treatment

  7. A Influência da Escola e da Mídia na Obtenção dos Conhecimentos Astronômicos

    NASA Astrophysics Data System (ADS)

    da Cunha, W. S.; Voelzke, M. R.; Amaral, L. H.

    2006-08-01

    O universo em que vivemos possui segredos jamais imaginados pelo homem, fruto da pequena proporção que ocupamos diante de um cosmos infinito. A astronomia funciona como um instrumento que sacia a sede humana em desvendar esses segredos, estudando as galáxias, quasares, pulsares além da existência de todos os elementos que preenchem o espaço cósmico, o vácuo, não mais vazio como se pensou em outros tempos. A mídia explora esse campo de forma mística e também científica. A difusão adequada e dentro dos padrões educacionais vigentes, de conceitos astronômicos, se faz necessária, devido sua presença nos conteúdos escolares, centros de ciência e na mídia, de modo geral. A reforma do Currículo Básico da Escola Pública da maioria dos Estados brasileiros introduziu conceitos astronômicos desde a pré-escola até o Ensino Médio. Num estudo realizado com 1.032 alunos do Ensino Médio de seis escolas estaduais, revelou-se através da aplicação de um questionário que abordava conheciment os básicos em astronomia, como estes foram adquiridos e qual era a infra-estrutura oferecida pela escola: para 66,2% dos entrevistados o professor já fez alguma apresentação a respeito de astronomia. O presente estudo revelou também que 44,5% dos alunos entrevistados adquiriram seus conhecimentos astronômicos na escola, 28,1% através da televisão e 27,4% afirmaram que seus conhecimentos foram obtidos através de outros tipos de mídia (internet, filmes e revistas). Embora o estudo revele claramente que há certo equilíbrio na forma em que os alunos obtiveram seus conhecimentos astronômicos, a mídia ainda explora e muitas vezes desperta o interesse pela astronomia com assuntos que não fazem parte de nosso cotidiano como, por exemplo, o homem na Lua, os projetos espaciais e a primeira viagem de um astronauta brasileiro no espaço. Porém, em certos casos, a mídia pode promover conhecimentos incompletos ou mesmo equivocados sobre os conceitos que envolvam

  8. Odontomas in two long-finned ocellaris clownfish ( Amphiprion ocellaris).

    PubMed

    Vorbach, Bryan S; Wolf, Jeffrey C; Yanong, Roy P

    2018-01-01

    Two adult long-finned ocellaris clownfish ( Amphiprion ocellaris) from 2 different aquaculture facilities were examined at the University of Florida, Tropical Aquaculture Laboratory for oral masses. Incisional biopsies were obtained from the masses under anesthesia, and histologic examination revealed both to be odontomas. Most benign odontomas are classified as hamartomas (disorganized proliferations of tissues found normally at the site of origin). Odontomas have previously been reported in wild teleost fish in association with viral infections and pollution.

  9. [Laparoscopic aspect and clinical relevance of cholangiofibromas].

    PubMed

    Henning, H; Friedrich, K; Lüders, C J

    1982-12-01

    In a current series of 1160 laparoscopies in 38 cases (3.2%) we found cholangiofibromas. The picture of single, sporadic and multiple cholangiofibromas as well as cholangiofibromatosis is both described and documented by laparoscopic photography. Clinically it seems remarkable that only patients with alcohol- or drug-induced liver injury showed such alterations. The prevalence of cholangiofibromas in toxic liver damage suggests that we are here not dealing with embryonal aberrations (hamartomas) but apparently with cellular inductive phenomena.

  10. Observações no âmbito dos "additional programs" do satélite COROT

    NASA Astrophysics Data System (ADS)

    Janot Pacheco, E.

    2003-08-01

    O satélite Fraco-europeu COROT fará fotometria de altissima precisão (pretende-se atingir uma parte em um milhão), grande campo (3x3 graus) e por longos períodos, de duas regiões pré-determinadas do céu, com 10 graus de raio. Suas finalidades básicas serão estudos em sismologia estelar e a procura de exoplanetas. A comunidade astronômica brasileira participará dessa missão espacial, com direitos iguais aos dos parceiros europeus. Isso se deve a que o satélite utilizará a estação de recepção de dados de Natal (INPE), 5 a 6 brasileiros participarão das equipes de software e cientistas do país atuarão na fase de pré-lançamento. Apresentamos nesta comunicação sugestões para a preparação de propostas de observações com COROT, no âmbito dos Programas Adicionais, que contemplam outros projetos que não de sismologia ou exoplanetas. As últimas definições técnicas e decisões tomadas na 4th Corot Week de junho último serão igualmente apresentadas, em particular quanto às regiões de observação escolhidas e quanto aos procedimentos a seguir para se propor observações.

  11. Prevalence and predictors of orthorexia nervosa among German students using the 21-item-DOS.

    PubMed

    Depa, Julia; Schweizer, Jenny; Bekers, Sandra-Kristin; Hilzendegen, Carolin; Stroebele-Benschop, Nanette

    2017-03-01

    Orthorexia nervosa (ON) describes the constant pathological preoccupation with "healthy" nutrition. The current results regarding the prevalence of ON differ widely possibly because of invalid measurement tools. This study aimed to investigate ON prevalence in a sample of German students and to examine age, gender, semester, and nutritional knowledge as potential predictors of ON by comparing nutrition science (NS) with economics (ES) students. A total of 446 university students participated in the survey (NS 188, ES 268). ON was determined using the 21-item-DOS, which is a well-constructed, validated, and reliability-tested questionnaire. Age, gender, and semester were also assessed. Of the total sample, 3.3 % were classified as having ON and 9.0 % were at risk of developing ON. Older students scored significantly higher on the subscale "avoidance of additives" compared with younger students and students of lower semester suffered significantly more often from ON than students of higher semester. In addition, comparing field of study showed no significant difference in the prevalence of ON or the risk of developing ON between female NS and ES students. However, mean values for the three DOS subscales were higher among female NS students, albeit far below values indicating pathological behavior. The prevalence of ON appears to be low in this sample of German university students. Female NS students do not seem to have higher prevalence of ON or risk of developing ON.

  12. Studies of Brazilian meteorites. III - Origin and history of the Angra dos Reis achondrite

    NASA Technical Reports Server (NTRS)

    Prinz, M.; Keil, K.; Hlava, P. F.; Berkley, J. L.; Gomes, C. B.; Curvello, W. S.

    1977-01-01

    The mineral composition of the Angra dos Reis meteorite, which fell in 1869, is described. This achondrite contains phases reported in a meteorite for the first time. Petrofabric analysis shows that fassaite has a preferred orientation and lineation, which is interpreted as being due to cumulus processes, possibly the effect of post-depositional magmatic current flow or laminar flow of a crystalline mush. The mineral chemistry indicates crystallization from a highly silica-undersaturated melt at low pressure. Several aspects of the mineral composition are discussed with reference to the implications of crystallization conditions.

  13. The Secret List of Dos and Don'ts for Filmmaking

    NASA Astrophysics Data System (ADS)

    Kramer, N.

    2012-12-01

    Science is a massive black box to billions of people who walk the streets. However, the process of filmmaking can be equally as mystifying. As with the development of many scientific experiments, the process starts on a napkin at a restaurant…but then what? The road to scientific publication is propelled by a canonical list of several dos and don't that fit most situations. An equally useful list exists for up-and-coming producers. The list streamlines efforts, optimizes your use of the tools at your fingertips and enhances impact. Many fundamentals can be learned from books, but during this talk we will project and discuss several examples of best practices, from honing a story, to identifying audience appeal, filming, editing and the secrets of inexpensively acquiring expert help. Whether your goal is a two-minute webisode or a 90 minute documentary, these time-tested practices, with a little awareness, can give life to your films.;

  14. Perfil de temperatura dos funis magnetosféricos de estrelas T Tauri com aquecimento alfvênico

    NASA Astrophysics Data System (ADS)

    Vasconcelos, M. J.

    2003-08-01

    Estrelas T Tauri Clássicas são objetos jovens circundados por discos de gás e poeira e que apresentam uma intensa atividade magnética. Seu espectro mostra linhas de emissão alargadas que são razoavelmente reproduzidas nos modelos de acresção magnetosférica. No entanto, o perfil de temperatura dos funis magnéticos é desconhecido. Aquecimento magnético compressional e difusão ambipolar foram considerados para estas estruturas, porém as temperaturas obtidas não são suficientes para explicar as observações. Neste trabalho, examinamos o aquecimento gerado pelo amortecimento de ondas Alfvén através de quatro mecanismos, os amortecimentos não-linear, turbulento, viscoso-resistivo e colisional como função da freqüência da onda. Inicialmente, a temperatura é ajustada para reproduzir as observações e o grau de turbulência requerido para que o mecanismo seja viável é calculado. Os resultados mostram que este é compatível com os dados observacionais. Apresentam-se, também, resultados preliminares do cálculo auto-consistente do perfil de temperatura dos funis, levando-se em conta fontes de aquecimento Alfvênica e fontes de resfriamento.

  15. Radiosurgery for epilepsy: Systematic review and International Stereotactic Radiosurgery Society (ISRS) practice guideline.

    PubMed

    McGonigal, Aileen; Sahgal, Arjun; De Salles, Antonio; Hayashi, Motohiro; Levivier, Marc; Ma, Lijun; Martinez, Roberto; Paddick, Ian; Ryu, Samuel; Slotman, Ben J; Régis, Jean

    2017-11-01

    While there are many reports of radiosurgery for treatment of drug-resistant epilepsy, a literature review is lacking. The aim of this systematic review is to summarize current literature on the use of stereotactic radiosurgery (RS) for treatment of epilepsy. Literature search was performed using various combinations of the search terms "radiosurgery", "stereotactic radiosurgery", "Gamma Knife", "epilepsy" and "seizure", from 1990 until October 2015. Level of evidence was assessed according to the PRISMA guidelines. Fifty-five articles fulfilled inclusion criteria. Level 2 evidence (prospective studies) was available for the clinical indications of mesial temporal lobe epilepsy (MTLE) and hypothalamic hamartoma (HH) treated by Gamma Knife (GK) RS. For remaining indications including corpus callosotomy as palliative treatment, epilepsy related to cavernous malformation and extra-temporal epilepsy, only Level 4 data was available (case report, prospective observational study, or retrospective case series). No Level 1 evidence was available. Based on level 2 evidence, RS is an efficacious treatment to control seizures in MTLE, possibly resulting in superior neuropsychological outcomes and quality of life metrics in selected subjects compared to microsurgery. RS has a better risk-benefit ratio for small hypothalamic hamartomas compared to surgical methods Delayed therapeutic effect resulting in ongoing seizures is associated with morbidity and mortality risk. Lack of level 1 evidence precludes the formation of guidelines at present. Copyright © 2017 Elsevier B.V. All rights reserved.

  16. Surgical management of gingival overgrowth associated with Cowden sydrome: a case report and current understanding.

    PubMed

    Feitosa, Daniela da Silva; Santamaria, Mauro Pedrine; Casati, Márcio Zaffalon; Sallum, Enilson Antonio; Nociti Júnior, Francisco Humberto; de Toledo, Sérgio

    2011-05-01

    Cowden syndrome, also known as multiple hamartoma syndrome, is a rare autosomal dominant disorder characterized by multiple hamartomas and a high risk of development of malignancy. Oral findings, such as papillomatous lesions and fibromas, are common features; however, a periodontal phenotype has not been reported previously. Therefore, this report presents a case of gingival overgrowth associated with Cowden syndrome, its successful surgical management, and the 12-month follow-up results. Additionally, we discuss the implications for clinicians. A 23-year-old woman was referred to the Department of Periodontics, Piracicaba Dental School, presenting with generalized gingival overgrowth. A detailed dental and medical history and clinical examination confirmed the systemic diagnosis of Cowden syndrome. Histology, radiographs, and clinical data document the entire clinical approach and follow-up. Clinically, there were minor signs of recurrence of gingival overgrowth in a 12-month period after gingivectomy; however, papular lesions reappeared in keratinized gingiva immediately after healing. No signs of bone loss related to the systemic condition were observed radiographically. Histologically, a dense connective tissue with a moderate chronic inflammatory infiltrate and epithelial acanthosis, which is characteristic of gingival hyperplasia, were demonstrated. Gingival overgrowth may occur as an oral phenotype related to Cowden syndrome and can be successfully treated by means of external bevel gingivectomy, followed by regular maintenance therapy, contributing to the patient's well-being, both functionally and esthetically.

  17. Three different clinical faces of the same histopathological entity: hair follicle nevus, trichofolliculoma and accessory tragus*

    PubMed Central

    Karabulut, Yasemin Yuyucu; Şenel, Engin; Karabulut, Hacı Halil; Dölek, Yasemin

    2015-01-01

    BACKGROUND Hair follicle nevus is a rare, congenital hamartoma with follicular differentiation characterized histologically by numerous, tiny, mature hair follicles. Trichofolliculoma, the histopathological features of which are quite similar to those of hair follicle nevus, is also a hamartoma that differs from hair follicle. Accessory tragus is a relatively common, benign congenital abnormality of the external ear with an incidence rate of 1 to 10 per 1,000 live births. OBJECTIVE This study seeks to assess the discriminatory value of currently available, histological criteria in the differential diagnosis of hair follicle nevus, accessory tragi and trichofolliculoma. METHODS Twenty-one patients comprising 9 cases of hair follicle nevus, 8 accessory tragi patients and 4 trichofolliculoma cases, were recruited to perform the study. RESULTS There were 10 males and 11 females in the study group. No significant difference was observed between the three study groups in terms of age, gender or histopathological parameters such as density of hair follicles, subcutaneous fat score and presence of connective tissue framework. Cartilaginous component was seen in 8 cases that were diagnosed as accessory tragi, while central cyst and radiating hair follicles were seen in 4 cases which were diagnosed as trichofolliculoma. CONCLUSION The results of our study showed that diagnostic discrimination of these diseases could be made only with the clinicopathologic correlation because of their clinical and histopathological similarities. PMID:26375221

  18. Three different clinical faces of the same histopathological entity: hair follicle nevus, trichofolliculoma and accessory tragus.

    PubMed

    Karabulut, Yasemin Yuyucu; Şenel, Engin; Karabulut, Hacı Halil; Dölek, Yasemin

    2015-01-01

    Hair follicle nevus is a rare, congenital hamartoma with follicular differentiation characterized histologically by numerous, tiny, mature hair follicles. Trichofolliculoma, the histopathological features of which are quite similar to those of hair follicle nevus, is also a hamartoma that differs from hair follicle. Accessory tragus is a relatively common, benign congenital abnormality of the external ear with an incidence rate of 1 to 10 per 1,000 live births. This study seeks to assess the discriminatory value of currently available, histological criteria in the differential diagnosis of hair follicle nevus, accessory tragi and trichofolliculoma. Twenty-one patients comprising 9 cases of hair follicle nevus, 8 accessory tragi patients and 4 trichofolliculoma cases, were recruited to perform the study. There were 10 males and 11 females in the study group. No significant difference was observed between the three study groups in terms of age, gender or histopathological parameters such as density of hair follicles, subcutaneous fat score and presence of connective tissue framework. Cartilaginous component was seen in 8 cases that were diagnosed as accessory tragi, while central cyst and radiating hair follicles were seen in 4 cases which were diagnosed as trichofolliculoma. The results of our study showed that diagnostic discrimination of these diseases could be made only with the clinicopathologic correlation because of their clinical and histopathological similarities.

  19. Age and isotopic relationships among the angrites Lewis Cliff 86010 and Angra dos Reis

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

    Lugmair, G.W.; Galer, S.J.G.

    1992-04-01

    Results of a wide-ranging isotopic investigation of the unique Antarctican angrite LEW-86010 (LEW) are presented, together with a reassessment of the type angrite Angra dos Reis (ADOR). The principal objectives of this study are to obtain precise radiometric ages, initial Sr isotopic compositions, and to search for the erstwhile presence of the short-lived nuclei {sup 146}Sm and {sup 26}Al via their daughter products. The isotopic compositions of Sm, U, Ca, and Ti were also measured. This allows a detailed appraisal to be made of the relations between, and the genealogy of, these two angrites.

  20. Chemotaxis cluster 1 proteins form cytoplasmic arrays in Vibrio cholerae and are stabilized by a double signaling domain receptor DosM.

    PubMed

    Briegel, Ariane; Ortega, Davi R; Mann, Petra; Kjær, Andreas; Ringgaard, Simon; Jensen, Grant J

    2016-09-13

    Nearly all motile bacterial cells use a highly sensitive and adaptable sensory system to detect changes in nutrient concentrations in the environment and guide their movements toward attractants and away from repellents. The best-studied bacterial chemoreceptor arrays are membrane-bound. Many motile bacteria contain one or more additional, sometimes purely cytoplasmic, chemoreceptor systems. Vibrio cholerae contains three chemotaxis clusters (I, II, and III). Here, using electron cryotomography, we explore V. cholerae's cytoplasmic chemoreceptor array and establish that it is formed by proteins from cluster I. We further identify a chemoreceptor with an unusual domain architecture, DosM, which is essential for formation of the cytoplasmic arrays. DosM contains two signaling domains and spans the two-layered cytoplasmic arrays. Finally, we present evidence suggesting that this type of receptor is important for the structural stability of the cytoplasmic array.

  1. 48 CFR 653.219-70 - DOS form DS-1910, Small Business Agency Review-Actions Above the Simplified Acquisition Threshold.

    Code of Federal Regulations, 2012 CFR

    2012-10-01

    ... 48 Federal Acquisition Regulations System 4 2012-10-01 2012-10-01 false DOS form DS-1910, Small Business Agency Review-Actions Above the Simplified Acquisition Threshold. 653.219-70 Section 653.219-70... Threshold. As prescribed in 619.501(c), DS-1910 is prescribed for use in documenting set-aside decisions...

  2. T.D.S. spectroscopic databank for spherical tops: DOS version

    NASA Astrophysics Data System (ADS)

    Tyuterev, V. G.; Babikov, Yu. L.; Tashkun, S. A.; Perevalov, V. I.; Nikitin, A.; Champion, J.-P.; Wenger, C.; Pierre, C.; Pierre, G.; Hilico, J.-C.; Loete, M.

    1994-10-01

    T.D.S. (Traitement de Donnees Spectroscopiques or Tomsk-Dijon-Spectroscopy project) is a computer package concerned with high resolution spectroscopy of spherical top molecules like CH4, CF4, SiH4, SiF4, SnH4, GeH4, SF6, etc. T.D.S. contains information, fundamental spectroscopic data (energies, transition moments, spectroscopic constants) recovered from comprehensive modeling and simultaneous fitting of experimental spectra, and associated software written in C. The T.D.S. goal is to provide an access to all available information on vibration-rotation molecular states and transitions including various spectroscopic processes (Stark, Raman, etc.) under extended conditions based on extrapolations of laboratory measurements using validated theoretical models. Applications for T.D.S. may include: education/training in molecular physics, quantum chemistry, laser physics; spectroscopic applications (analysis, laser spectroscopy, atmospheric optics, optical standards, spectroscopic atlases); applications to environment studies and atmospheric physics (remote sensing); data supply for specific databases; and to photochemistry (laser excitation, multiphoton processes). The reported DOS-version is designed for IBM and compatible personal computers.

  3. Video movie making using remote procedure calls and 4BSD Unix sockets on Unix, UNICOS, and MS-DOS systems

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

    Robertson, D.W.; Johnston, W.E.; Hall, D.E.

    1990-03-01

    We describe the use of the Sun Remote Procedure Call and Unix socket interprocess communication mechanisms to provide the network transport for a distributed, client-server based, image handling system. Clients run under Unix or UNICOS and servers run under Unix or MS-DOS. The use of remote procedure calls across local or wide-area networks to make video movies is addressed.

  4. Diverted organic synthesis (DOS): accessing a new, natural product inspired, neurotrophically active scaffold through an intramolecular Pauson-Khand reaction.

    PubMed

    Mehta, Goverdhan; Samineni, Ramesh; Srihari, Pabbaraja; Reddy, R Gajendra; Chakravarty, Sumana

    2012-09-14

    Drawing inspiration from the impressive neurotrophic activity exhibited by the natural product paecilomycine A, we have designed a new natural product-like scaffold employing an intramolecular Pauson-Khand reaction. Several compounds based on the new designer scaffold exhibited promising neurotrophic activity and are worthy of further biological evaluation. Our findings also highlight the importance of a DOS strategy in creating useful therapeutical leads.

  5. AGGRESSIVE RETINAL ASTROCYTOMAS IN FOUR PATIENTS WITH TUBEROUS SCLEROSIS COMPLEX

    PubMed Central

    Shields, Jerry A; Eagle, Ralph C; Shields, Carol L; Marr, Brian P

    2004-01-01

    ABSTRACT Objective To report the clinical and histopathologic findings of retinal astrocytic tumors that showed progressive growth in four patients with tuberous sclerosis complex (TSC). Methods Four young children each developed an enlarging retinal neoplasm that eventually necessitated enucleation of the affected eye. The systemic findings, clinical course, and histopathologic findings were reviewed. Results Each patient had a progressively enlarging retinal mass associated with a total exudative retinal detachment and neovascular glaucoma. Enucleation was necessary in each case because the affected eye became blind and painful. The mean patient age at enucleation was 7 years, and the median age was 3 years. At the time of enucleation the tumors ranged from 10 to 20 mm in basal diameter and from 10 to 25 mm in thickness. Histopathologic studies of each eye revealed a giant cell astrocytoma that had produced a total exudative retinal detachment. The tumor cells showed positive immunoreactivity to neuron-specific enolase and glial fibrillary acidic protein. The retinal neoplasms in these cases were identical histopathologically to the subependymal giant cell astrocytoma that typifies TSC in the brain. One tumor filled the entire eye and perforated the globe. Although the lesions simulated retinoblastoma clinically, each patient had ocular and systemic findings of TSC, supporting the diagnosis of astrocytic hamartoma. Conclusions Although retinal astrocytic lesions of TSC generally are stationary, they can sometimes grow relentlessly and cause severe ocular complications. Patients with retinal astrocytic hamartomas should have serial ophthalmic evaluations because of this possibility. PMID:15747752

  6. Measured oxygen fugacities of the Angra dos Reis achondrite as a function of temperature

    USGS Publications Warehouse

    Brett, R.; Stephen, Huebner J.; Sato, M.

    1977-01-01

    Measurements of the oxygen fugacity (f{hook}O2) as a function of temperature (T) were made on an interior bulk sample of the cumulate achondrite, Angra dos Reis. Data clustered between the f{hook}O2-T relationship of the iron-wu??stite assemblage and 1.2 log atm units above iron-wu??stite. Interpretation of the data indicates that, throughout most of the cooling history of the meteorite, f{hook}O2 values were defined by equilibria involving iron-bearing species at values close to the f{hook}O2 of the assemblage iron-wu??stite. Measured f{hook}O2 data are compatible with crystallization and cooling at pressures greater than 50 bars. ?? 1977.

  7. A holistic approach to a rare pathology.

    PubMed

    Lieberman, Morton; Velez, Ines; Mejia, Lina; Siegel, Michael A

    2010-01-01

    A case of cystic hygroma (CH), a congenital lymph-filled multicystic hamartoma of the neck, with a holistic approach to patient management is presented. The stigma of the CH imposed by attending physicians, dentists, parents, teachers and peers affected the psychological development of this patient since her diagnosis in childhood. Although surgery relieves the threat of airway obstruction and may improve the esthetic appearance of the CH, the patient may be isolated and traumatized by the ignorance and superstitions of the individuals she encounters on a daily basis.

  8. [Congenital linear nevus sebaceus].

    PubMed

    Linnemann, Anders; Bygum, Anette; Fenger-Grøn, Jesper

    2011-09-05

    An unusual case of nevus sebaceous is described. Nevus sebaceous is a congenital epidermal hamartoma of the skin and the predilection site is the head or neck. In this case the nevus followed the lines of Blaschko along the back of the left lower extremity. The linear lesion seemed papulovesicular which caused suspicion of incontinentia pigmenti or infection, and the boy received antimicrobial treatment until a biopsy revealed the correct diagnosis. We wish to emphasize this clinical picture to spare the patient and relatives from unnecessary tests, treatment and concern.

  9. Hair Follicle Nevus Located on the Chin of an Infant: Case Report and Review of Literature.

    PubMed

    Larson, Krista N; O'Shea, Patrick; Zedek, Daniel C; Morrell, Dean S

    2016-01-01

    Hair follicle nevi are rare, benign, congenital hamartomas that usually occur in the distribution of the first brachial arch. Histopathologically, the distinction between hair follicle nevus, trichofolliculoma, and accessory tragus has recently come into question, and it may be that they are all on a spectrum of the same condition. We report the case of a 7-day-old boy who presented with a "tag"-like lesion on his midline chin that had been present since birth. Biopsy of the lesion proved it to be a hair follicle nevus. © 2016 Wiley Periodicals, Inc.

  10. Lack of normal MR enhancement of the pituitary gland: findings in three siblings with combined pituitary hormone deficiency.

    PubMed

    Falcone, S; Sanchez, J; Quencer, R M

    1998-02-01

    We present the MR appearance of the sella turcica in three sibling dwarfs with combined pituitary hormone deficiency in which MR images revealed a peripheral curvilinear band of enhancement about the pituitary gland in all three patients, a normal-size pituitary gland in two siblings, a mildly enlarged pituitary gland in one sibling, and a thin infundibulum and a normal posterior pituitary bright spot in all three siblings. Possible antecedents include an abnormal vascular supply, pituitary gland replacement by a nonfunctioning adenoma, a proteinaceous cyst, or a hamartoma.

  11. Investigação dos perfís temporais de alta resolução de explosões solares tipo-III decimétricas

    NASA Astrophysics Data System (ADS)

    Cecatto, J. R.; Fernandes, F. C. R.; Sawant, H. S.; Madsen, F. R. H.

    2003-08-01

    Explosões solares tipo-III indicam a presença de feixes de elétrons acelerados durante "flares" solares. Sua investigação fornece informações tanto sobre os processos de aceleração de partículas quanto das características do agente causador e do local de aceleração. Explosões tipo-III decimétricas são geradas por feixes de elétrons viajando através de arcos magnéticos densos da baixa coroa solar. Os perfís temporais destes fenômenos, quando tomados com alta resolução, informam sobre os mecanismos de aceleração de partículas do feixe e o meio de transporte da energia liberada a partir da região de aceleração. Usando o Brazilian Solar Spectroscope (BSS), em operação no INPE, foram registradas dezenas de explosões tipo-III decimétricas, dentro da faixa de 2050-2250 MHz com alta resolução temporal (20 ms), em 13 de setembro de 2001, entre 13:00 e 16:10 UT. Foram selecionadas 10 explosões isoladas para uma investigação estatística detalhada de seus perfís temporais, em todos os cerca de 50 canais de freqüência. Os resultados indicam que cerca de 70% dos perfís temporais são complexos tanto durante a subida quanto descida. Os 30% restantes indicam que os perfís da subida podem ser bem representados, na maioria dos casos, por um processo não-linear e uma parcela significativa por processos lineares. Os perfís temporais da descida são dominados por um decaimento não-linear. Neste trabalho, será efetuada uma análise dos perfís temporais, tanto durante a subida quanto descida do fluxo, para as explosões selecionadas, em termos dos prováveis mecanismos de aceleração e relaxamento. 2

  12. Análise dos fatores de risco relacionados às amputações maiores e menores de membros inferiores em hospital terciário

    PubMed Central

    de Jesus-Silva, Seleno Glauber; de Oliveira, João Pedro; Brianezi, Matheus Henrique Colepicolo; Silva, Melissa Andreia de Moraes; Krupa, Arturo Eduardo; Cardoso, Rodolfo Souza

    2017-01-01

    Resumo Contexto As amputações dos membros inferiores, sejam definidas como maiores ou menores, são um grave problema de saúde, com altos índices de morbimortalidade e de relevante impacto social. Diferentes características clínicas dos pacientes parecem estar relacionadas aos diferentes tipos de amputação realizados. Objetivos Analisar os fatores de risco presentes em pacientes submetidos a amputações de membros inferiores em hospital terciário. Métodos Estudo retrospectivo, transversal, envolvendo 109 pacientes submetidos a amputação de membro inferior em um período de 31 meses, através da análise de gênero e idade, 15 dados clínicos e cinco parâmetros laboratoriais presentes no momento da admissão. Os dados foram submetidos a estatística descritiva e comparativa através do teste t de Student não pareado (para variáveis numéricas), e dos testes de Mann-Whitney e exato de Fisher (para variáveis categóricas). Resultados Das 109 amputações realizadas, 59 foram maiores e 50 menores. A maioria dos pacientes era do gênero masculino (65%), e a média de idade foi de 65 anos (mín. 39, máx. 93). Dentre os fatores de risco observados, idade avançada, acidente vascular encefálico, isquemia, sepse e níveis baixos de hemoglobina e hematócrito estavam estatisticamente mais relacionados às amputações maiores (p < 0,05). Diabetes melito, neuropatia e pulsos distais palpáveis foram fatores mais associados às amputações menores. Conclusões Os níveis das amputações de membros inferiores estão relacionados a diferentes fatores de risco. Os quadros isquêmicos mais graves e de maior morbidade estiveram associados a amputações maiores, enquanto a neuropatia e perfusão preservada, mais relacionados às amputações menores. PMID:29930618

  13. Buffer zone monitoring plan for the Dos Rios subdivision, Gunnison, Colorado

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

    NONE

    1996-02-01

    This report presents a plan for water quality monitoring at the Dos Rios subdivision (Units 2, 3, and the Island Unit) that is intended to satisfy the informational needs of residents who live southwest (downgradient) of the former Gunnison processing site. Water quality monitoring activities described in this report are designed to protect the public from residual contamination that entered the ground water as a result of previous uranium milling operations. Requirements presented in this monitoring plan are also included in the water sampling and analysis plan (WSAP) for the Gunnison Uranium Mill Tailings Remedial Action (UMTRA) Project site. Themore » Gunnison WSAP is a site-specific document prepared by the U.S. Department of Energy (DOE) that provides background, guidance, and justification for future ground water sampling and analysis activities for the UMTRA Project Gunnison processing and disposal sites. The WSAP will be updated annually, as additional water quality data are collected and interpreted, to provide ongoing protection for public health and the environment.« less

  14. 2.3 Å X-ray Structure of the Heme-Bound GAF Domain of Sensory Histidine Kinase DosT of Mycobacterium tuberculosis†

    PubMed Central

    Podust, Larissa M.; Ioanoviciu, Alexandra; Ortiz de Montellano, Paul R.

    2009-01-01

    Mycobacterium tuberculosis responds to the changes in environmental conditions through a two-component signaling system that detects reduced O2 tension and NO and CO exposures via the heme-binding GAF domains of two sensory histidine kinases, DosT and DevS, and the transcriptional regulator DosR. We report the first x-ray structure of the DosT heme-bound GAF domain (GAFDosT) in both oxy and deoxy forms determined to a resolution of 2.3 Å. In GAFDosT, heme binds in an orientation orthogonal to that in the PAS domains via a highly conserved motif including invariant H147 as a proximal heme axial ligand. On the distal side, invariant Y169 is in stacking interactions with the heme with its long axis parallel and the plane of the ring orthogonal to the heme plane. In one of the two protein monomers in an asymmetric unit, O2 binds as a second axial ligand to the heme iron, and is stabilized via an H-bond to the OH-group of Y169. The structure reveals two small tunnel-connected cavities and a pore on the protein surface that suggest a potential route for O2 access to the sensing pocket. The limited conformational differences observed between differently heme iron-ligated GAFDosT monomers in the asymmetric unit may result from crystal lattice limitations since atmospheric oxygen binding likely occurs in the crystal as a result of x-ray induced Fe3+ photoreduction during diffraction data collection. Determination of the GAFDosT structure sets up a framework in which to address ligand-recognition, discrimination, and signal propagation schemes in the heme-based GAF domains of biological sensors. PMID:18980385

  15. En la búsqueda de características en eyecciones coronales de masa que discriminen entre dos paradigmas físicos en modelos de ECMs

    NASA Astrophysics Data System (ADS)

    Paissan, G.; Stenborg, G.; Rovira, M.

    Se conocen tres diferentes fenómenos de gran escala que ocurren en la atmósfera solar, denominados eyecciones coronales de masa (ECMs), protuberancias eruptivas y grandes fulguraciones de dos bandas. Estos fenómenos están estrechamente relacionados y podrían ser distintas manifestaciones de un único proceso físico. Las ECMs son definidas como eyecciones de gran escala de masa y flujo magnético desde la baja corona al espacio interplanetario. Desde su descubrimiento en los '70, muchos modelos han sido propuestos para explicar su origen y evolución. La explicación física de las ECMs es un tema de debate intenso. No obstante, los modelos pueden sintetizarse en dos grandes grupos: 1) los modelos de inyección de flujo y 2) los modelos de almacenamiento y liberación. En este trabajo, se presentan los estudios realizados con una serie de eventos observados con el coronógrafo MICA (Mirror Coronograph for Argentina), el telescopio en H-alfa HASTA (H-alpha Solar Telescope for Argentina) y los coronógrafos C2 y C3 de la sonda SOHO (Solar and Heliospheric Observatory). Los eventos que pudieron ser identificados como ECMs son contrastados dentro del esquema de los dos paradigmas teóricos propuestos.

  16. 32 CFR Appendix C to Part 197 - Procedures for the Department of State (DoS) Foreign Relations of the United States (FRUS) Series

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... 32 National Defense 2 2012-07-01 2012-07-01 false Procedures for the Department of State (DoS) Foreign Relations of the United States (FRUS) Series C Appendix C to Part 197 National Defense Department of Defense (Continued) OFFICE OF THE SECRETARY OF DEFENSE (CONTINUED) MISCELLANEOUS HISTORICAL...

  17. 32 CFR Appendix C to Part 197 - Procedures for the Department of State (DoS) Foreign Relations of the United States (FRUS) Series

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... 32 National Defense 2 2013-07-01 2013-07-01 false Procedures for the Department of State (DoS) Foreign Relations of the United States (FRUS) Series C Appendix C to Part 197 National Defense Department of Defense (Continued) OFFICE OF THE SECRETARY OF DEFENSE (CONTINUED) MISCELLANEOUS HISTORICAL...

  18. 32 CFR Appendix C to Part 197 - Procedures for the Department of State (DoS) Foreign Relations of the United States (FRUS) Series

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... 32 National Defense 2 2014-07-01 2014-07-01 false Procedures for the Department of State (DoS) Foreign Relations of the United States (FRUS) Series C Appendix C to Part 197 National Defense Department of Defense (Continued) OFFICE OF THE SECRETARY OF DEFENSE (CONTINUED) MISCELLANEOUS HISTORICAL...

  19. Use of a frameless computed tomography-guided stereotactic biopsy system for nasal biopsy in five dogs.

    PubMed

    Kuhlman, Gregory M; Taylor, Amanda R; Thieman-Mankin, Kelley M; Griffin, Jay; Cook, Audrey K; Levine, Jonathan M

    2016-04-15

    5 dogs (median age, 9 years; median body weight, 31 kg [68.2 lb]) with undefined nasal masses were examined after undergoing CT of the head and nasal biopsy via a rostral rhinoscopic or unaided (blind) approach because histologic results for collected biopsy specimens (inflammatory, necrotic, or hemorrhagic disease) suggested the specimens were nonrepresentative of the underlying disease process identified via CT (aggressive or malignant disease). Clinical signs at the time dogs were evaluated included open-mouth breathing, sneezing, or unilateral epistaxis. Histologic findings pertaining to the original biopsy specimens were suggestive of benign processes such as inflammation. In an attempt to obtain better representative specimens, a frameless CT-guided stereotactic biopsy system (CTSBS) was used to collect additional biopsy specimens from masses within the nasal and sinus passages of the dogs. The second set of biopsy specimens was histologically evaluated. Histologic evaluation of biopsy specimens collected via the CTSBS revealed results suggestive of malignant neoplasia (specifically, chondrosarcoma, hemangiopericytoma, or undifferentiated sarcoma) for 3 dogs, mild mixed-cell inflammation for 1 dog, and hamartoma for 1 dog. No complications were reported. These findings resulted in a change in treatment recommendations for 3 dogs and confirmed that no additional treatment was required for 1 dog (with hamartoma). For the remaining dog, in which CT findings and clinical history were strongly suggestive of neoplasia, the final diagnosis was rhinitis. Biopsy specimens were safely collected from masses within the nasal and sinus passages of dogs by use of a frameless CTSBS, allowing a definitive diagnosis that was unachievable with other biopsy approaches.

  20. En bloc resection for treatment of tumor-induced osteomalacia: a case presentation and a systematic review.

    PubMed

    Meng, Tong; Zhou, Wang; Li, Bo; Yin, Huabin; Li, Zhenxi; Zhou, Lei; Kong, Jinhai; Yan, Wangjun; Yang, Xinghai; Liu, Tielong; Song, Dianwen; Xiao, Jianru

    2015-05-08

    Tumor-induced osteomalacia (TIO) is a rare disorder, which is commonly found in craniofacial locations and in the extremities. To the best of our knowledge, only 16 cases have been described in the spine, and this is the first report to describe a case of patient with TIO in the thoracic spine combined with a mesenchymal hamartoma which had confused the therapeutic strategies to date. We report the case of a 60-year-old patient with hypophosphatemia and presented with limb weakness. Treating with phosphate did not correct the hypophosphatemia and an (111)In pentetreotide scintigraphy (octreotide scan) revealed an increased uptake at the right forearm. The tumor was resected totally, and the histopathology revealed a mesenchymal hamartoma, but we noticed that hypophosphatemia was not corrected after the tumor resection. Then a whole-body magnetic resonance imaging (WB-MRI) was performed and the results revealed tumorous tissues at the right T1 vertebral pedicle. The tumor was removed with an en bloc method, and the pathology showed phosphaturic mesenchymal tumor. Follow-up at 1 year after surgery revealed no recurrence, and the serum phosphorus level of the patient was normal. Tumor-induced osteomalacia is exceedingly rare with only 16 cases in spine published in the literature. It is difficult to find and leads to years of suffering debilitating complications. In this regard, the WB-MRI is a better method to locate the real tumor. Treating with phosphate can only relieve symptoms, and a complete surgical removal remains the gold standard treatment.

  1. A novel mouse model of tuberous sclerosis complex (TSC): eye-specific Tsc1-ablation disrupts visual-pathway development

    PubMed Central

    Jones, Iwan; Hägglund, Anna-Carin; Törnqvist, Gunilla; Nord, Christoffer; Ahlgren, Ulf; Carlsson, Leif

    2015-01-01

    ABSTRACT Tuberous sclerosis complex (TSC) is an autosomal dominant syndrome that is best characterised by neurodevelopmental deficits and the presence of benign tumours (called hamartomas) in affected organs. This multi-organ disorder results from inactivating point mutations in either the TSC1 or the TSC2 genes and consequent activation of the canonical mammalian target of rapamycin complex 1 signalling (mTORC1) pathway. Because lesions to the eye are central to TSC diagnosis, we report here the generation and characterisation of the first eye-specific TSC mouse model. We demonstrate that conditional ablation of Tsc1 in eye-committed progenitor cells leads to the accelerated differentiation and subsequent ectopic radial migration of retinal ganglion cells. This results in an increase in retinal ganglion cell apoptosis and consequent regionalised axonal loss within the optic nerve and topographical changes to the contra- and ipsilateral input within the dorsal lateral geniculate nucleus. Eyes from adult mice exhibit aberrant retinal architecture and display all the classic neuropathological hallmarks of TSC, including an increase in organ and cell size, ring heterotopias, hamartomas with retinal detachment, and lamination defects. Our results provide the first major insight into the molecular etiology of TSC within the developing eye and demonstrate a pivotal role for Tsc1 in regulating various aspects of visual-pathway development. Our novel mouse model therefore provides a valuable resource for future studies concerning the molecular mechanisms underlying TSC and also as a platform to evaluate new therapeutic approaches for the treatment of this multi-organ disorder. PMID:26449264

  2. A novel mouse model of tuberous sclerosis complex (TSC): eye-specific Tsc1-ablation disrupts visual-pathway development.

    PubMed

    Jones, Iwan; Hägglund, Anna-Carin; Törnqvist, Gunilla; Nord, Christoffer; Ahlgren, Ulf; Carlsson, Leif

    2015-12-01

    Tuberous sclerosis complex (TSC) is an autosomal dominant syndrome that is best characterised by neurodevelopmental deficits and the presence of benign tumours (called hamartomas) in affected organs. This multi-organ disorder results from inactivating point mutations in either the TSC1 or the TSC2 genes and consequent activation of the canonical mammalian target of rapamycin complex 1 signalling (mTORC1) pathway. Because lesions to the eye are central to TSC diagnosis, we report here the generation and characterisation of the first eye-specific TSC mouse model. We demonstrate that conditional ablation of Tsc1 in eye-committed progenitor cells leads to the accelerated differentiation and subsequent ectopic radial migration of retinal ganglion cells. This results in an increase in retinal ganglion cell apoptosis and consequent regionalised axonal loss within the optic nerve and topographical changes to the contra- and ipsilateral input within the dorsal lateral geniculate nucleus. Eyes from adult mice exhibit aberrant retinal architecture and display all the classic neuropathological hallmarks of TSC, including an increase in organ and cell size, ring heterotopias, hamartomas with retinal detachment, and lamination defects. Our results provide the first major insight into the molecular etiology of TSC within the developing eye and demonstrate a pivotal role for Tsc1 in regulating various aspects of visual-pathway development. Our novel mouse model therefore provides a valuable resource for future studies concerning the molecular mechanisms underlying TSC and also as a platform to evaluate new therapeutic approaches for the treatment of this multi-organ disorder. © 2015. Published by The Company of Biologists Ltd.

  3. Católicos, fidelidade conjugal e AIDS: entre a cruz da doutrina moral e as espadas do cotidiano sexual dos adeptos1

    PubMed Central

    Rios, Luis Felipe; de Aquino, Francisca Luciana; Muñoz-Laboy, Miguel; Oliveira, Cinthia; Parker, Richard

    2009-01-01

    Neste artigo discutimos a visão da Igreja Católica sobre sexualidade na interface com a epidemia do HIV/AIDS. Nossa reflexão está embasada em pesquisa etnográfica que envolveu dois meses de observação participante do cotidiano de católicos de um bairro popular da Região Metropolitana do Recife, além de contar com entrevistas a onze dos leigos engajados nos serviços religiosos da igreja do bairro e a oito sacerdotes que realizam seus trabalhos religiosos em outras localidades. Nelas abordamos diferentes temáticas relacionadas ao enfrentamento da epidemia da AIDS. Nesse contexto, conjugalidade e fidelidade se afiguram como importantes analisadores de como aqueles lidam com a epidemia, em uma variedade de re-descrições práticas e de re-interpretações conceptuais das assertivas do discurso moral religioso – ainda que, muitos impasses permaneçam em aberto em termos das prerrogativas da Igreja e seus possíveis rebatimentos na saúde sexual dos adeptos. PMID:21765650

  4. Geochemistry and petrogenesis of the Mesoarchean granites from the Canaã dos Carajás area, Carajás Province, Brazil: Implications for the origin of Archean granites

    NASA Astrophysics Data System (ADS)

    Feio, G. R. L.; Dall'Agnol, R.

    2012-12-01

    Four Mesoarchean (2.93 to 2.83 Ga) granite units, which encompass the Canaã dos Carajás, Bom Jesus, Cruzadão and Serra Dourada granites, were recognized in the Canaã dos Carajás area of the Archean Carajás Province. The Mesoarchean units are composed dominantly of biotite leucomonzogranites. They are compared with the Neoarchean Planalto suite (2.73 Ga) which encompasses biotite-hornblende monzogranites to syenogranites. The Canaã dos Carajás, Bom Jesus and the variety of the Cruzadão granite with higher (La/Yb)N are geochemically more akin to the calc-alkaline granites, whereas the other varieties of the Cruzadão granite are transitional between calc-alkaline and alkaline granites. The Serra Dourada granite has an ambiguous geochemical character with some features similar to those of calc-alkaline granites and other peraluminous granites. The Planalto granites have ferroan character, are similar geochemically to reduced A-type granites and show a strong geochemical contrast with the Mesoarchean studied granites. The Mesoarchean granites described in the Canaã dos Carajás area are geochemically distinct to those of the Rio Maria domain of the Carajás Province. The Canaã dos Carajás and Bom Jesus granites are similar to the high-Ca granites, whereas the Cruzadão and Serra Dourada are more akin to the low-CaO granites of the Yilgarn craton. The geochemical characteristics of the Mesoarchean studied granites approach those of the biotite granite group of Dharwar but the latter are enriched in HFSE and HREE compared to the Canaã dos Carajás granites. The Neoarchean Planalto suite granite has no counterpart in the Mesoarchean Rio Maria domain of the Carajás Province, neither in the Yilgarn and Dharwar cratons. Geochemical modeling suggests that partial melting of a source similar in composition to an Archean basaltic andesite of the Carajás Province could give origin to the Bom Jesus and Cruzadão granites. In the case of the Bom Jesus granite the

  5. Familial bilateral periventricular nodular heterotopia mimics tuberous sclerosis.

    PubMed Central

    Jardine, P E; Clarke, M A; Super, M

    1996-01-01

    A mother and daughter with an initial diagnosis of tuberous sclerosis are described. The daughter presented with partial seizures at the age of 8 months. Computed tomography showed uncalcified periventricular nodules which on magnetic resonance imaging were ovoid, almost contiguous, of grey matter density, and did not enhance with gadolinium. Brain imaging of her asymptomatic mother was similar. Absence of severe mental retardation, extracranial hamartomas, and depigmented patches distinguishes familial bilateral periventricular nodular heterotopia (FNH) from tuberous sclerosis. FNH is probably inherited as an X linked dominant with lethality in males. Images Figure 1 Figure 2 PMID:8787433

  6. Cerebriform connective tissue nevus of lumbar.

    PubMed

    Chen, Jinbo; Chen, Liuqing; Duan, Yiqun; Li, Dongsheng; Dong, Bilin

    2015-02-01

    Connective tissue nevi represents a kind of hamartoma, and coalescence of the lesions in a cerebriform mode in the lumbar region without Proteus syndrome is rarely seen. Here, we report a 26-year-old woman presenting with nodules and plaques in her left lumbar region of 26 years in duration. Histopathological examination and Masson-trichrome stain showed increased dermal collagen bundles in a haphazard array. The diagnosis of connective tissue nevi was made. This is the first case report on cerebriform connective tissue nevi without Proteus syndrome in the lumbar region. © 2014 Japanese Dermatological Association.

  7. Cross-sectional Imaging Review of Tuberous Sclerosis.

    PubMed

    Krishnan, Anant; Kaza, Ravi K; Vummidi, Dharshan R

    2016-05-01

    Tuberous sclerosis complex (TSC) is a multisystem, genetic disorder characterized by development of hamartomas in the brain, abdomen, and thorax. It results from a mutation in one of 2 tumor suppressor genes that activates the mammalian target of rapamycin pathway. This article discusses the origins of the disorder, the recently updated criteria for the diagnosis of TSC, and the cross-sectional imaging findings and recommendations for surveillance. Familiarity with the diverse radiological features facilitates diagnosis and helps in treatment planning and monitoring response to treatment of this multisystem disorder. Copyright © 2016 Elsevier Inc. All rights reserved.

  8. Nevus sebaceous revisited.

    PubMed

    Moody, Megan N; Landau, Jennifer M; Goldberg, Leonard H

    2012-01-01

    Nevus sebaceous of Jadassohn is a hamartoma with a combination of abnormalities of the epidermis, hair follicles, and sebaceous and apocrine glands. Herein, we discuss the results of an extensive literature review on the topic of nevus sebaceous with a particular focus on the debate about the necessity for prophylactic excision. We also focus on the documentation of associated malignant tumors that were reported to develop within NS. In addition to reporting the number and types of neoplasms, we documented the recommendations of all authors for therapeutic handling of these nevi. © 2011 Wiley Periodicals, Inc.

  9. Rare earth elements in Angra dos Reis and Lewis Cliff 86010, two meteorites with similar but distinct magma evolutions

    NASA Technical Reports Server (NTRS)

    Crozaz, Ghislaine; Mckay, Gordon

    1990-01-01

    Data are presented on ion microprobe measurements of REE and selected trace element abundances in individual grains of merrillite, fassaite, olivine, kirschsteinite, and plagioclase of Lewis Cliff 86010 (LEW 86010) meteorite and in merrillite and fassaite grains of Angra dos Reis (ADOR). Results show a close relationship between the two meteorites and support a magmatic origin for LEW 86010. However, the measurements indicate that, despite numerous common characteristics, the two meteorites must have been produced in separate magmatic events involving similar but distinct processes and parent melts.

  10. 32 CFR Appendix C to Part 197 - Procedures for the Department of State (DoS) Foreign Relations of the United States (FRUS) Series

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 32 National Defense 2 2011-07-01 2011-07-01 false Procedures for the Department of State (DoS) Foreign Relations of the United States (FRUS) Series C Appendix C to Part 197 National Defense Department... RESEARCH IN THE FILES OF THE OFFICE OF THE SECRETARY OF DEFENSE (OSD) Pt. 197, App. C Appendix C to Part...

  11. Albumin expression distinguishes bile duct adenomas from metastatic adenocarcinoma.

    PubMed

    Moy, Andrea P; Arora, Kshitij; Deshpande, Vikram

    2016-09-01

    Bile duct adenomas may be difficult to distinguish from metastatic carcinomas, particularly well-differentiated pancreatic ductal adenocarcinoma. Prior studies have evaluated the utility of various immunohistochemical markers, although these markers are notable for low sensitivity and/or specificity. The aim of this study was to investigate the utility of albumin and BRAFV600E expression in distinguishing between metastatic pancreatic adenocarcinoma and bile duct adenoma. We studied 26 bile duct adenomas, three bile duct hamartomas, and 158 pancreatic ductal adenocarcinomas. Branched-chain in-situ hybridization (bISH) for albumin was performed; bISH is based on the branched DNA technology, wherein signal amplification is achieved via a series of sequential steps. Additionally, BRAFV600E immunohistochemistry (IHC) was performed on a subset of cases. Twenty-three of 25 (92%) bile duct adenomas were positive for albumin; 18 (72%) showed diffuse staining, and five showed focal staining (20%), including two challenging examples. Two bile duct hamartomas also stained positively. All pancreatic adenocarcinomas were negative for albumin. Seven of 16 (44%) bile duct adenomas and five of 106 (5%) pancreatic ductal adenocarcinomas were positive for BRAFV600E by IHC. The sensitivity and specificity of expression of albumin, as detected by bISH, for distinguishing bile duct adenomas from metastatic pancreatic adenocarcinomas were 92% and 100%, respectively; the sensitivity and specificity of BRAFV600E IHC for distinguishing bile duct adenomas from metastatic pancreatic adenocarcinomas were 43.8% and 95.3%, respectively. Diagnostically challenging examples of bile duct adenoma may be distinguished from metastatic pancreatic adenocarcinoma by the use of albumin bISH. © 2016 John Wiley & Sons Ltd.

  12. Balancing Proliferation and Connectivity in PTEN-associated Autism Spectrum Disorder.

    PubMed

    Tilot, Amanda K; Frazier, Thomas W; Eng, Charis

    2015-07-01

    Germline mutations in PTEN, which encodes a widely expressed phosphatase, was mapped to 10q23 and identified as the susceptibility gene for Cowden syndrome, characterized by macrocephaly and high risks of breast, thyroid, and other cancers. The phenotypic spectrum of PTEN mutations expanded to include autism with macrocephaly only 10 years ago. Neurological studies of patients with PTEN-associated autism spectrum disorder (ASD) show increases in cortical white matter and a distinctive cognitive profile, including delayed language development with poor working memory and processing speed. Once a germline PTEN mutation is found, and a diagnosis of phosphatase and tensin homolog (PTEN) hamartoma tumor syndrome made, the clinical outlook broadens to include higher lifetime risks for multiple cancers, beginning in childhood with thyroid cancer. First described as a tumor suppressor, PTEN is a major negative regulator of the phosphatidylinositol 3-kinase/protein kinase B/mammalian target of rapamycin (mTOR) signaling pathway-controlling growth, protein synthesis, and proliferation. This canonical function combines with less well-understood mechanisms to influence synaptic plasticity and neuronal cytoarchitecture. Several excellent mouse models of Pten loss or dysfunction link these neural functions to autism-like behavioral abnormalities, such as altered sociability, repetitive behaviors, and phenotypes like anxiety that are often associated with ASD in humans. These models also show the promise of mTOR inhibitors as therapeutic agents capable of reversing phenotypes ranging from overgrowth to low social behavior. Based on these findings, therapeutic options for patients with PTEN hamartoma tumor syndrome and ASD are coming into view, even as new discoveries in PTEN biology add complexity to our understanding of this master regulator.

  13. Pineal region tumors: computed tomographic-pathologic spectrum

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

    Futrell, N.N.; Osborn, A.G.; Cheson. B.D.

    While several computed tomographic (CT) studies of posterior third ventricular neoplasms have included descriptions of pineal tumors, few reports have concentrated on these uncommon lesions. Some authors have asserted that the CT appearance of many pineal tumors is virtually pathognomonic. A series of nine biopsy-proved pineal gland and eight other presumed tumors is presented that illustrates their remarkable heterogeneity in both histopathologic and CT appearance. These tumors included germinomas, teratocarcinomas, hamartomas, and other varieties. They had variable margination, attentuation, calcification, and suprasellar extension. Germinomas have the best response to radiation therapy. Biopsy of pineal region tumors is now feasible andmore » is recommended for treatment planning.« less

  14. Non-malignant Fibrosing Tumors in the Pediatric Hand: A Clinicopathologic Case Review

    PubMed Central

    Baumholtz, Michael A.; Popek, Edwina; Schneider, Adam M.

    2008-01-01

    Non-malignant fibrosing tumors in the pediatric hand or juvenile fibromatoses are clinically challenging because of their relatively infrequent occurrence and because of the variety of names associated with these diseases. We conducted a review of a personal case series of pediatric patients with these tumors and discuss here the more common histologic types and clinical characteristics of the disease spectrum in the context of the available published literature. All histologic samples were reviewed by a single pathologist. Infantile myofibromatosis, fibrous hamartoma of infancy, juvenile aponeurotic fibromatosis, palmar fibromatosis (Dupuytren’s type), infantile digital fibromatosis (Reye’s tumor), fibroma of the tendon sheath, and melorheostosis represent the encountered lesions. PMID:19048350

  15. Cystic lung disease in birt-hogg-dubé syndrome: a case series of three patients.

    PubMed

    Kilincer, Abidin; Ariyurek, Orhan Macit; Karabulut, Nevzat

    2014-06-01

    Birt-Hogg-Dubé syndrome is characterized by clinical manifestations such as hamartomas of the skin, renal tumors and lung cysts with spontaneous pneumothoraces. Patients with Birt-Hogg-Dubé syndrome may present with only multiple lung cysts. We report the chest computerized tomography (CT) features of three patients with Birt-Hogg-Dubé syndrome. Each patient had multiple lung cysts of various sizes according to chest CT evaluation, most of which were located in lower lobes and related to pleura. The identification of unique characteristics in the chest CT of patients with Birt-Hogg-Dubé syndrome may provide an efficient mechanism for diagnosis.

  16. Proteus syndrome*

    PubMed Central

    Rocha, Ritha de Cássia Capelato; Estrella, Mariani Paulino Soriano; do Amaral, Danielle Mechereffe; Barbosa, Angela Marques; de Abreu, Marilda Aparecida Milanez Morgado

    2017-01-01

    Proteus syndrome is a rare syndrome characterized by disproportionate overgrowth of limbs, multiple hamartomas, and vascular malformations. The cerebriform connective tissue nevi, also called cerebriform plantar hyperplasia, are present in most patients, and is the main characteristic of the syndrome. If present, even alone, they can be considered as a pathognomonic sign. This article reports a classic case of Proteus syndrome in a 2-year-old male patient who began to show a discrete asymmetry of the right hemibody in relation to the left one after birth, which increased over the months. He also showed cerebriform plantar hyperplasia and Port-wine stains, among other alterations. PMID:29166516

  17. BENIGN TUMORS AND TUMOR-LIKE LESIONS OF THE PANCREAS

    PubMed Central

    Basturk, Olca; Askan, Gokce

    2017-01-01

    Synopsis The pancreas is a complex organ that may give rise to large number of neoplasms and non-neoplastic lesions. This article will focus on benign neoplasms such as serous neoplasms as well as tumor-like (pseudotumoral) lesions that may be mistaken for neoplasm not only by clinicians and radiologists, but also by pathologists. The family of pancreatic pseudotumors, by a loosely defined conception of that term, includes a variety of lesions including heterotopia, hamartoma, and lipomatous pseudohypertrophy. Autoimmue pancreatitis (covered in chronic pancreatitis chapter) and paraduodenal (“groove”) pancreatitis may also lead to pseudotumor formation. Knowledge of these entities will help in making an accurate diagnosis. PMID:27926363

  18. Congenital panfollicular nevus: report of a new entity.

    PubMed

    Finn, Laura S; Argenyi, Zsolt B

    2005-01-01

    The various forms of non-melanocytic nevi (hamartomas) are usually encountered in pediatric patients, and nevus sebaceous of Jadassohn is the most common to have undifferentiated pilosebaceous units. We report a unique congenital follicular nevus that fails to meet the criteria of any previously described follicular neoplasm, despite the plethora of alternatives. Clinically considered a syringocystadenoma papilliferum, the excised lesion contained multiple dermal nodules that exhibited nearly all stages of follicular differentiation. The periodicity of the follicular proliferations was akin to normal terminal hair, and a prominent perifollicular sheath surrounded each. This benign lesion of abortive hair follicles was unassociated with any established genodermatous syndrome or other adnexal neoplasm.

  19. The Caenorhabditis elegans EGL-15 Signaling Pathway Implicates a DOS-Like Multisubstrate Adaptor Protein in Fibroblast Growth Factor Signal Transduction

    PubMed Central

    Schutzman, Jennifer L.; Borland, Christina Z.; Newman, John C.; Robinson, Matthew K.; Kokel, Michelle; Stern, Michael J.

    2001-01-01

    EGL-15 is a fibroblast growth factor receptor in the nematode Caenorhabditis elegans. Components that mediate EGL-15 signaling have been identified via mutations that confer a Clear (Clr) phenotype, indicative of hyperactivity of this pathway, or a suppressor-of-Clr (Soc) phenotype, indicative of reduced pathway activity. We have isolated a gain-of-function allele of let-60 ras that confers a Clr phenotype and implicated both let-60 ras and components of a mitogen-activated protein kinase cascade in EGL-15 signaling by their Soc phenotype. Epistasis analysis indicates that the gene soc-1 functions in EGL-15 signaling by acting either upstream of or independently of LET-60 RAS. soc-1 encodes a multisubstrate adaptor protein with an amino-terminal pleckstrin homology domain that is structurally similar to the DOS protein in Drosophila and mammalian GAB1. DOS is known to act with the cytoplasmic tyrosine phosphatase Corkscrew (CSW) in signaling pathways in Drosophila. Similarly, the C. elegans CSW ortholog PTP-2 was found to be involved in EGL-15 signaling. Structure-function analysis of SOC-1 and phenotypic analysis of single and double mutants are consistent with a model in which SOC-1 and PTP-2 act together in a pathway downstream of EGL-15 and the Src homology domain 2 (SH2)/SH3-adaptor protein SEM-5/GRB2 contributes to SOC-1-independent activities of EGL-15. PMID:11689700

  20. Asymptomatic multinodular splenoma (splenic hamartoma) in a child with sickle cell anemia

    PubMed Central

    Elenga, Narcisse; Labbé, Sylvain; Leduc, Nicolas; Sika, Anicet; Cuadro, Emma; Long, Laurence; Njuieyon, Falucar; Kom-Tchameni, Rémi; Basset, Thierry

    2017-01-01

    Splenoma is a rare and benign malformation usually fortuitously diagnosed during imaging, surgery or, unfortunately, at autopsy. Although splenoma was first described in 1861, its association with hematological pathology is a very unusual condition in children. We report the case of an asymptomatic splenoma in an 8-year-old boy with sickle cell anemia, whose diagnosis was confirmed after conventional splenectomy. PMID:28744165

  1. Morphometric and bioacoustic data on three species of Pseudopaludicola Miranda-Ribeiro, 1926 (Anura: Leptodactylidae: Leiuperinae) described from Chapada dos Guimarães, Mato Grosso, Brazil, with the revalidation of Pseudopaludicola ameghini (Cope, 1887).

    PubMed

    Pansonato, André; Strüssmann, Christine; Mudrek, Jessica Rhaiza; Martins, Itamar Alves

    2013-01-01

    Due to minute size, overall morphological similarities, scarcity of diagnostic characters after preservation, and usual sympatric or even syntopic occurrence of two or more species of Pseudopaludicola, the taxonomy of the genus is not yet a matter of consensus. Three species in the genus Pseudopaludicola Miranda-Ribeiro, 1926 were described by Cope in 1887, based on material obtained at Chapada dos Guimarães, mid-western state of Mato Grosso, Brazil. One of these species, Pseudopaludicola ameghini, was subsequently synonymized to P. mystacalis. In this paper we present morphological and bioacoustic evidences supporting a full specific status for the three sympatric species of Pseudopaludicola described from Chapada dos Guimarães, including Pseudopaludicola ameghini Cope, 1887.

  2. Functional imaging in differentiating bronchial masses: an initial experience with a combination of (18)F-FDG PET-CT scan and (68)Ga DOTA-TOC PET-CT scan.

    PubMed

    Kumar, Arvind; Jindal, Tarun; Dutta, Roman; Kumar, Rakesh

    2009-10-01

    To evaluate the role of combination of (18)F-FDG PET-CT scan and (68)Ga DOTA-TOC PET-CT scan in differentiating bronchial tumors observed in contrast enhanced computed tomography scan of chest. Prospective observational study. Place of study: All India Institute of Medical Sciences, New Delhi, India. 7 patients with bronchial mass detected in computed tomography scan of the chest were included in this study. All patients underwent (18)F-FDG PET-CT scan, (68)Ga DOTA-TOC PET-CT scan and fiberoptic bronchoscope guided biopsy followed by definitive surgical excision. The results of functional imaging studies were analyzed and the results are correlated with the final histopathology of the tumor. Histopathological examination of 7 bronchial masses revealed carcinoid tumors (2 typical, 1 atypical), inflammatory myofibroblastic tumor (1), mucoepidermoid carcinoma (1), hamartoma (1), and synovial cell sarcoma (1). The typical carcinoids had mild (18)F-FDG uptake and high (68)Ga DOTA-TOC uptake. Atypical carcinoid had moderate uptake of (18)F-FDG and high (68)Ga DOTA-TOC uptake. Inflammatory myofibroblastic tumor showed high uptake of (18)F-FDG and no uptake of (68)Ga DOTA-TOC. Mucoepidermoid carcinoma showed mild (18)F-FDG uptake and no (68)Ga DOTA-TOC uptake. Hamartoma showed no uptake on either scans. Synovial cell sarcoma showed moderate (18)F-FDG uptake and mild focal (68)Ga DOTA-TOC uptake. This initial experience with the combined use of (18)F-FDG and (68)Ga DOTA-TOC PET-CT scan reveals different uptake patterns in various bronchial tumors. Bronchoscopic biopsy will continue to be the gold standard; however, the interesting observations made in this study merits further evaluation of the utility of the combination of (18)F-FDG PET-CT scan and (68)Ga DOTA-TOC PET-CT scan in larger number of patients with bronchial masses.

  3. A novel germline mutation (c.A527G) in STK11 gene causes Peutz-Jeghers syndrome in a Chinese girl: A case report.

    PubMed

    Zhao, Zi-Ye; Jiang, Yu-Liang; Li, Bai-Rong; Yang, Fu; Li, Jing; Jin, Xiao-Wei; Sun, Shu-Han; Ning, Shou-Bin

    2017-12-01

    Peutz-Jeghers syndrome (PJS) is a Mendelian autosomal dominant disease caused by mutations in the tumor suppressor gene, serine/threonine kinase 11 (STK11). The features of this syndrome include gastrointestinal (GI) hamartomas, melanin spots on the lips and the extremities, and an increased risk of developing cancer. Early onset of disease is often characterized by mucocutaneous pigmentation and intussusception due to GI polyps in childhood. A girl with a positive family history grew oral pigmentation at 1 and got intussusception by small bowel hamartomas at 5. She was diagnosed with PJS based on oral pigmentation and a positive family history of PJS. Enteroscopy was employed to treat the GI polyps. Sanger sequencing was used to investigate STK11 mutation in this family. A large jejunal polyp together with other smaller ones was resected, and the girl recovered uneventfully. We discovered a heterozygous substitution in STK11, c.A527G in exon 4, in the girl and her father who was also a PJS patient, and the amine acid change was an aspartic acid-glycine substitution in codon 176. This mutation was not found in other healthy family members and 50 unrelated non-PJS controls, and it is not recorded in databases, which prove it a novel mutation. Evolutionary conservation analysis of amino acid residues showed this aspartic acid is a conserved one between species, and protein structure prediction by SWISS-MODEL indicated an obvious change in local structure. In addition, PolyPhen-2 score for this mutation is 1, which indicates it probably damaging. PJS can cause severe complication like intussusception in young children, and early screening for small bowel may be beneficial for these patients. The mutation of STK11 found in this girl is a novel one, which enlarges the spectrum of STK11. Our analysis supported it a causative one in PJS.

  4. The 2015 World Health Organization Classification of Lung Tumors: Impact of Genetic, Clinical and Radiologic Advances Since the 2004 Classification.

    PubMed

    Travis, William D; Brambilla, Elisabeth; Nicholson, Andrew G; Yatabe, Yasushi; Austin, John H M; Beasley, Mary Beth; Chirieac, Lucian R; Dacic, Sanja; Duhig, Edwina; Flieder, Douglas B; Geisinger, Kim; Hirsch, Fred R; Ishikawa, Yuichi; Kerr, Keith M; Noguchi, Masayuki; Pelosi, Giuseppe; Powell, Charles A; Tsao, Ming Sound; Wistuba, Ignacio

    2015-09-01

    The 2015 World Health Organization (WHO) Classification of Tumors of the Lung, Pleura, Thymus and Heart has just been published with numerous important changes from the 2004 WHO classification. The most significant changes in this edition involve (1) use of immunohistochemistry throughout the classification, (2) a new emphasis on genetic studies, in particular, integration of molecular testing to help personalize treatment strategies for advanced lung cancer patients, (3) a new classification for small biopsies and cytology similar to that proposed in the 2011 Association for the Study of Lung Cancer/American Thoracic Society/European Respiratory Society classification, (4) a completely different approach to lung adenocarcinoma as proposed by the 2011 Association for the Study of Lung Cancer/American Thoracic Society/European Respiratory Society classification, (5) restricting the diagnosis of large cell carcinoma only to resected tumors that lack any clear morphologic or immunohistochemical differentiation with reclassification of the remaining former large cell carcinoma subtypes into different categories, (6) reclassifying squamous cell carcinomas into keratinizing, nonkeratinizing, and basaloid subtypes with the nonkeratinizing tumors requiring immunohistochemistry proof of squamous differentiation, (7) grouping of neuroendocrine tumors together in one category, (8) adding NUT carcinoma, (9) changing the term sclerosing hemangioma to sclerosing pneumocytoma, (10) changing the name hamartoma to "pulmonary hamartoma," (11) creating a group of PEComatous tumors that include (a) lymphangioleiomyomatosis, (b) PEComa, benign (with clear cell tumor as a variant) and (c) PEComa, malignant, (12) introducing the entity pulmonary myxoid sarcoma with an EWSR1-CREB1 translocation, (13) adding the entities myoepithelioma and myoepithelial carcinomas, which can show EWSR1 gene rearrangements, (14) recognition of usefulness of WWTR1-CAMTA1 fusions in diagnosis of epithelioid

  5. Efficacy of 99mTc-EDDA/HYNIC-TOC scintigraphy in differential diagnosis of solitary pulmonary nodules.

    PubMed

    Płachcińska, Anna; Mikołajczak, Renata; Maecke, Helmut; Kozak, Józef; Michalski, Andrzej; Rzeszutek, Katarzyna; Kuśmierek, Jacek

    2004-10-01

    Fifty consecutive patients with solitary pulmonary nodules (SPN) on chest radiographs were studied scintigraphically after the administration of a somatostatin analog 99mTc-EDDA/HYNIC-TOC. The activity amounted to 740-925 MBq and a single photon emission computed tomography (SPECT) technique was applied. Verification of the nodule etiology was based on histology or cytology and bacteriology. As additional criterion for nodule benignity, its stable size in a chest radiograph for at least 3 years was accepted. In 31 patients, malignant etiologies of nodules were found. The diagnoses included: 11 adenocarcinomas, 6 squamous-cell carcinomas, 2 large-cell carcinomas, 6 nonsmall-cell lung cancers (NSCLC) of unspecified, more detailed morphology, 2 small-cell lung cancers (SCLC), 2 typical carcinoids, and 2 metastatic tumors: leiomyosarcoma and malignant melanoma. In 19 patients, the following benign tumors were diagnosed: 6 tuberculomas, 2 other granulomas, 4 hamartomas, 2 nonspecific inflammatory infiltrates, 1 abscess, 1 peripheral carcinoid of morphological characteristics of a benign tumor, 1 ectopic lesion of thyroid tissue, and 2 benign tumors of unspecified etiology, with stable size over 3 and 5 years. Positive scintigraphic results were obtained in 28 of 31 patients (90%) with malignant SPNs; among these there were 26 of 27 (96%) cases of primary pulmonary carcinoma. The remaining 2 false-negative cases included metastatic tumors: liposarcoma and melanoma. Among 19 benign lesions, 15 (79%) did not accumulate the radiopharmaceutical. The remaining 4 tumors visible on scintigrams included: 1 tuberculoma, 1 hamartoma, 1 abscess, and 1 case of nonestablished diagnosis (with stable size over 3 years). In conclusion, scintigraphy with 99mTc-EDDA/HYNIC-TOC appears to be an effective procedure for differentiation between malignant and benign SPNs.

  6. Nasopharyngeal glial heterotopia with delayed postoperative meningitis.

    PubMed

    Maeda, Kenichi; Furuno, Kenji; Chong, Pin Fee; Morioka, Takato

    2017-06-22

    A male infant, who underwent radical resection of a large glial heterotopia at the nasopharynx at 8 days, developed delayed postoperative bacterial meningitis at 9 months. Neuroradiological examination clearly demonstrated that meningitis had occurred because of the intracranial and extracranial connections, which were scarcely seen in the perioperative period. A transsphenoidal extension of hypothalamic hamartoma is possible because the connection started from the right optic nerve, running through the transsphenoidal canal in the sphenoid bone and terminating at the recurrent mass in the nasopharyngeal region. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  7. Congenital Becker's nevus with a familial association.

    PubMed

    Book, S E; Glass, A T; Laude, T A

    1997-01-01

    Becker's nevus is a unilateral, hyperpigmented cutaneous hamartoma usually with hypertrichosis. It occurs predominantly in boys, becoming apparent during adolescence, although several cases of congenital Becker's nevus have been reported. Rarely it may be familial and as such is transmitted in an autosomal dominant pattern. We report a 16-month-old black boy with a hyperpigmented patch on his right shoulder and upper pectoral area that extended down his arm. The patient's father has a similar lesion with hair on his left shoulder which has been present since childhood. Histology of the child's lesion was consistent with Becker's nevus. We believe this to be the first reported case of a congenital Becker's nevus with a familial association.

  8. Geology and geochemistry of Pelagatos, Cerro del Agua, and Dos Cerros monogenetic volcanoes in the Sierra Chichinautzin Volcanic Field, south of México City

    NASA Astrophysics Data System (ADS)

    Agustín-Flores, Javier; Siebe, Claus; Guilbaud, Marie-Noëlle

    2011-04-01

    This study focuses on the geology and geochemistry of three closely-spaced monogenetic volcanoes that are located in the NE sector of the Sierra Chichinautzin Volcanic Field near México City. Pelagatos (3020 m.a.s.l.) is a small scoria cone (0.0017 km 3) with lava flows (0.036 km 3) that covered an area of 4.9 km 2. Cerro del Agua scoria cone (3480 m.a.s.l., 0.028 km 3) produced several lava flows (0.24 km 3) covering an area of 17.6 km 2. Dos Cerros is a lava shield which covers an area of 80.3 km 2 and is crowned by two scoria cones: Tezpomayo (3080 m.a.s.l., 0.022 km 3) and La Ninfa (3000 m.a.s.l., 0.032 km 3). The eruptions of Cerro del Agua and Pelagatos occurred between 2500 and 14,000 yr BP. The Dos Cerros eruption took place close to 14,000 yr BP as constrained by radiocarbon dating. Rocks from these three volcanoes are olivine-hypersthene normative basaltic andesites and andesites with porphyritic, aphanitic, and glomeroporphyritic textures. Their mineral assemblages include olivine, clinopyroxene, and orthopyroxene phenocrysts (≤ 10 vol.%) embedded in a trachytic groundmass which consists mainly of plagioclase microlites and glass. Pelagatos rocks also present quartz xenocrysts. Due to their high Cr and Ni contents, and high Mg#s, Pelagatos rocks are considered to be derived from primitive magmas, hence the importance of this volcano for understanding petrogenetic processes in this region. Major and trace element abundances and petrography of products from these volcanoes indicate a certain degree of crystal fractionation during ascent to the surface. However, the magmas that formed the volcanoes evolved independently from each other and are not cogenetically related. REE, HFSE, LILE, and isotopic (Sr, Nd, and Pb) compositions point towards a heterogeneous mantle source that has been metasomatized by aqueous/melt phases from the subducted Cocos slab. There is no clear evidence of important crustal contributions in the compositions of Pelagatos and

  9. Novas determinações dos parâmetros atmosféricos das estrelas anãs brancas DA

    NASA Astrophysics Data System (ADS)

    Giovannini, O.; Costa, A. F. M.; Kepler, S. O.

    2003-08-01

    Nós temos selecionado uma amostra de estrelas anãs brancas DA (atmosfera de hidrogênio) a partir dos índices de Strömgren cujos valores estão próximos a região de instabilidade das estrelas anãs brancas DA variáveis, as chamadas estrelas DAV ou ZZ Ceti. O objetivo é determinar os parâmetros fundamentais (temperatura efetiva, Teff, aceleração da gravidade, log g, e massa) destas estrelas para verificar quais os parâmetros estelares estão envolvidos com o mecanismo de pulsação das estrelas DAV. Nós obtemos, até agora, mais de 120 espectros óticos de estrelas DA. Entre as estrelas selecionadas há 20 estrelas variáveis (DAV). Assim, podemos verificar se existem ou não estrelas não variáveis dentro da faixa de instabilidade das estrelas ZZ Ceti. Neste trabalho nós apresentamos a determinação dos parâmetros atmosféricos (temperatura efetiva, Teff, e aceleração da gravidade, log g) das estrelas anãs brancas DA usando os novos modelos de atmosfera ML2/a = 0.6. Estes modelos têm sido utilizados recentemente por fornecerem uma excelente consistência interna na determinação das temperaturas nas regiões do ultra-violeta e ótico. Os parâmetros atmosféricos são determinados espectroscopicamente através da comparação do fluxo de energia das linhas de Balmer (Hb à H9) entre os espectros observados e sintéticos (gerados pelos modelos de atmosfera). As temperaturas obtidas com os novos modelos são, em geral, menores (~ 1000 K menos) que as temperaturas determinadas anteriormente, com modelos ML1. Os valores de log g não mudaram significativamente (menos de 10%). A faixa de instabilidade das DAVs está entre 11000 e 13000 K, consistente com dados de outros autores.

  10. Endocrine disruptors in water filters used in the Rio dos Sinos Basin region, Southern Brazil.

    PubMed

    Furtado, C M; von Mühlen, C

    2015-05-01

    The activated carbon filter is used in residences as another step in the treatment of drinking water, based on a physical-chemical process to absorb pollutants that are not removed in conventional treatment. Endocrine disruptors (EDCs) are exogenous substances or mixtures of substances that acts on the endocrine system similarly to the endogenously produced hormones, triggering malfunctions and harmful changes to human and animal health. The objective of the present work was to study EDCs through semi-quantitative analysis of residential water filters collected in the region of Rio dos Sinos basin, focusing on two specific classes: hormones and phenols. The solid phase extraction principle was used for the extraction of compounds and gas chromatography coupled with mass spectrometry for the separation and characterization of EDCs. Four samples of residential filters collected from public water distribution and artesian wells, from the cities of Novo Hamburgo and São Leopoldo were analysed. Using the developed methodology, it was possible to detect and comparatively quantify selected EDCs in all studied samples, which indicates the presence of these contaminants in drinking water from different sources.

  11. Avaliação dos efeitos do subgalato de bismuto na proliferação de miofibroblastos

    PubMed Central

    de Lima, Rubianne Ligório; Sampaio, Cláudia Paraguaçu; Seidel, Karin Caroline; Branco, Melina; Sobreiro, Rafaela Mabile

    2016-01-01

    Resumo Contexto O subgalato de bismuto é um metal pesado e insolúvel, utilizado por suas propriedades adstringentes e hemostáticas. Objetivo Avaliar os efeitos do subgalato de bismuto na cicatrização mediante observação de miofibroblastos em pele de ratos. Métodos Foram utilizados 60 ratos da linhagem Wistar, que receberam uma ferida no dorso da pele. Os animais foram divididos em dois grupos: controle (aplicação diária de cloreto de sódio a 0,9%) e experimental (aplicação diária de 0,5 mg de subgalato de bismuto). Cada grupo foi subdividido em três subgrupos, que foram reoperados para retirada da ferida em 3, 7 e 14 dias. Foi realizada coloração de hematoxilina eosina, picrosirius e imuno-histoquímica para avaliar contagem de miofibroblastos, resposta inflamatória e síntese de colágeno. Resultados Não foi encontrada diferença entre os grupos controle e experimento com relação ao processo inflamatório – subgrupos 3 dias (p = 1), 7 dias (p = 0,474) e 14 dias (p = 303). A avaliação dos colágenos tipo I e III no grupo-controle não demonstrou benefícios de cicatrização – 3 dias (p = 0,436), 7 dias (p = 0,853) e 14 dias (p = 0,436); já no grupo experimental, houve aumento dos colágenos tipos I e III nos subgrupos 3 e 14 dias (p = 0,005). A imuno-histoquímica confirmou os resultados encontrados na coloração hematoxilina eosina, na qual a área de miofibroblastos entre os subgrupos, nos grupos experimental (p = 0,4) e controle (p = 0,336), foi indiferente. Conclusão A utilização do subgalato de bismuto em ferida de pele de ratos não evidenciou benefícios na cicatrização, ou seja, não houve diferença na fibroplasia quando comparados os grupos experimental e controle. PMID:29930592

  12. Epidemiological study of hepatitis B and C in a municipality with rural characteristics: Cássia dos Coqueiros, State of São Paulo, Brazil.

    PubMed

    Melo, Laura Valdiane Luz; Silva, Marcondes Alves Barbosa da; Perdoná, Gleici da Silva Castro; Nascimento, Margarida Maria Passeri; Secaf, Marie; Monteiro, Rosane Aparecida; Martinelli, Ana de Lourdes Candolo; Passos, Afonso Dinis Costa

    2015-01-01

    Hepatitis B and C viral infections remain an important cause of global morbidity and mortality. Studies have been conducted in population groups of large cities, leaving gaps in the knowledge regarding the situation in small municipalities. We aimed to measure the prevalence of hepatitis B and C markers and presence of infection-associated factors. All inhabitants of Cássia dos Coqueiros aged ≥18 years who agreed to participate in the research were included. We collected blood as well as information via a questionnaire between March 2011 and December 2013. Univariate and multivariate analyses were conducted. Among the 1,001 participants, 41 (4.1%) participants had a serological profile of hepatitis B viral exposure, and only one (0.1%) participant was considered a virus carrier. The frequency of isolated antibody to hepatitis B virus surface antigen (anti-HBs) markers was 17.8% for the overall population. In the multivariate analysis, hepatitis B virus (HBV) infection was associated with age, birth outside the State of São Paulo, history of hepatitis, ≥2 sexual partners in the last 6 months, and tattoos. Four (0.4%) participants had a serological profile of hepatitis C viral exposure. However, after confirmation using viral ribonucleic acid (RNA) evaluation, only one (0.1%) individual remained positive. The positivity rates for hepatitis B and C were low, despite greater sexual freedom and the recent emergence of illicit drugs, as observed by the health personnel working in Cássia dos Coqueiros.

  13. Feeding ecology of the lizard Tropidurus oreadicus Rodrigues 1987 (Tropiduridae) at Serra dos Carajás, Pará state, northern Brazil.

    PubMed

    Rocha, C F D; Siqueira, C C

    2008-02-01

    Tropidurus species commonly prey on arthropods, but they may also feed on vertebrates and plant material. The lizard Tropidurus oreadicus (Tropiduridae) is common in open vegetation habitats and generally has sexual dimorphism. In this study we analyzed the diet of T. oreadicus at Serra dos Carajás, Pará, in the north of Brazil. Snout-vent length (SVL) and jaw width (JW) were taken for 34 lizards. There was a significant difference in SVL and in JW, with males being larger than females. All lizards analyzed contained food in their stomachs. The diet of T. oreadicus at Serra dos Carajás was characterized by the consumption of a relative wide spectrum of food item categories (21 types of items), consisting of arthropods, part of one vertebrate and plant material, which characterizes the diet of a generalist predator. Volumetrically, the most important items in the diet of both sexes of T. oreadicus were flowers (M = 61.7%; F = 33%) and orthopterans (M = 1.7%; F = 3.5%). Ants were the most frequently consumed (100% for both sexes) and the most numerous (M = 94.5%; F = 89.4%) food item. Flowers also were frequently consumed (M = 91.7%; F = 54.5%), with their relative consumption differing significantly between sexes. There was not a significant sexual difference in prey volume, neither in number of preys per stomach, nor in type of prey ingested. There was no relationship between lizard jaw width and the mean volume of prey. The data showed that T. oreadicus is a relatively generalist lizard in terms of diet and that consumes large volumes of plant material, especially flowers of one species of genus Cassia.

  14. SU-F-T-330: Characterization of the Clinically Released ScandiDos Discover Diode Array for In-Vivo Dose Monitoring

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

    Saenz, D; Gutierrez, A

    Purpose: The ScandiDos Discover has obtained FDA clearance and is now clinically released. We studied the essential attenuation and beam hardening components as well as tested the diode array’s ability to detect changes in absolute dose and MLC leaf positions. Methods: The ScandiDos Discover was mounted on the heads of an Elekta VersaHD and a Varian 23EX. Beam attenuation measurements were made at 10 cm depth for 6 MV and 18 MV beam energies. The PDD(10) was measured as a metric for the effect on beam quality. Next, a plan consisting of two orthogonal 10 × 10 cm2 fields wasmore » used to adjust the dose per fraction by scaling monitor units to test the absolute dose detection sensitivity of the Discover. A second plan (conformal arc) was then delivered several times independently on the Elekta VersaHD. Artificially introduced MLC position errors in the four central leaves were then added. The errors were incrementally increased from 1 mm to 4 mm and back across seven control points. Results: The absolute dose measured at 10 cm depth decreased by 1.2% and 0.7% for 6 MV and 18 MV beam with the Discover, respectively. Attenuation depended slightly on the field size but only changed the attenuation by 0.1% across 5 × 5 cm{sup 2} and 20 − 20 cm{sup 2} fields. The change in PDD(10) for a 10 − 10 cm{sup 2} field was +0.1% and +0.6% for 6 MV and 18 MV, respectively. Changes in monitor units from −5.0% to 5.0% were faithfully detected. Detected leaf errors were within 1.0 mm of intended errors. Conclusion: A novel in-vivo dosimeter monitoring the radiation beam during treatment was examined through its attenuation and beam hardening characteristics. The device tracked with changes in absolute dose as well as introduced leaf position deviations.« less

  15. [Therapeutic update in tuberous sclerosis complex: the role of mTOR pathway inhibitors].

    PubMed

    Ruiz-Falcó Rojas, M Luz

    2012-05-21

    Tuberous sclerosis complex is an autosomal dominant disease, with variable expressivity and multisystemic involvement, which is characterised by the growth of benign tumours called hamartomas. The organs that are most commonly affected are the brain, skin, kidneys, eyes, heart and lungs. Of all the children with this disease, 85% present neurological manifestations that, due to their severity, are the main cause of morbidity and mortality. The most significant neurological manifestations are epilepsy, autism spectrum disorders and mental retardation. It has been shown that in tuberous sclerosis complex the genes TSC1 and TSC2 alter the mTOR enzyme cascade, which sets off inhibition of this pathway. The possibility of resorting to treatments applied at the origin, thus inhibiting this pathway, is currently being evaluated.

  16. Connective Tissue Nevi: A Review of the Literature.

    PubMed

    Arora, Harleen; Falto-Aizpurua, Leyre; Cortés-Fernandez, Andrea; Choudhary, Sonal; Romanelli, Paolo

    2017-05-01

    Connective tissue nevi (CTN) are hamartomas of the dermis, with the 3 main components being collagen, elastin, and proteoglycans. Each subtype can present as a solitary lesion or multiple lesions. They could present as part of systemic diseases or inherited disorders. This article provides a comprehensive literature review of the different types of CTN, their clinical presentations, associations, and treatment options. Treatment options for 56 lesions were reviewed. Fifty-two percent of lesions were present in males, and the age range at the time of presentation was wide (1.6-80 years). Management varied according to CTN subtypes. Most lesions (14) received topical or intralesional treatment with corticosteroids, followed by surgical removal of lesions (12), whereas the remaining lesions were clinically monitored.

  17. Primary mesenchymal or mixed-cell-origin lung tumors in four dogs.

    PubMed

    Watson, A D; Young, K M; Dubielzig, R R; Biller, D S

    1993-03-15

    Primary lung tumors of mesenchymal or mixed cell origin were diagnosed in 4 dogs with clinical and radiographic abnormalities indicating an intrathoracic mass. Each dog had 1 large intrapulmonary lesion, and 1 dog also had nodules scattered throughout all lung lobes. Two dogs were euthanatized; 1 had a biphasic pulmonary blastoma; and the other had a pulmonary chondroblastic osteosarcoma with intrapulmonary metastases. The masses in the other 2 dogs were hamartomas (lipomatous in 1, microcystic in the other), which were resected. Both dogs survived more than 1 year after surgery. Primary lung tumors are uncommon in dogs and are generally malignant (adenocarcinomas or carcinomas). Tumors of connective tissue or mixed cell origin are rare, but the outcome is potentially good after surgical removal.

  18. Adenomatoid odontogenic tumor with clear cell changes.

    PubMed

    Mohanty, Neeta; Routray, Samapika; Swain, Niharika; Ingale, Yashwant

    2014-01-01

    Adenomatoid odontogenic tumor (AOT) has a limited biological profile and been an attention-grabbing tumor for a century for its origin. Though described earlier, it was widely accepted after Harbitz from Norway reported about this uncommon benign tumor in 1915. There has been a long debate as whether this tumor is a hamartoma or a neoplasm. Here, we present a case of AOT in a 20-year-old female with details of clinical, radiological and histological features along with clear cell changes, signifying AOT to be more aggressive in nature than assessed from earlier literature. Thus, we did an extensive search of PubMed literature on AOT with all its histopathological features associated until date to find the report of clear cell changes yet.

  19. A new species of Rock-Dwelling Scinax Wagler (Anura: Hylidae) from Chapada dos Veadeiros, Central Brazil.

    PubMed

    Araujo-Vieira, Katyuscia; Brandão, Reuber Albuquerque; Faria, Daniele Carvalho Do Carmo

    2015-02-02

    A new species of the Scinax ruber clade is described from Chapada dos Veadeiros region, Central Brazil. The new species is diagnosed by having SVL 21.9-27.7 mm in males and 26.7-31.7 mm in females; snout acuminate in dorsal view and rounded in profile; medium-sized tympanum; vocal sac single, median, subgular, that does not reach the pectoral region; iris iridescent yellow, with some thin, darker reticulations; tadpoles with ventral oral disc; P-3 regular and unmodified as a labial arm; absence of keratinized and colored plates on the sides of the lower jaw-sheath; presence of a keratinized and colored spur on each side behind the lower jaw-sheath; dorsolateral eyes, ventrally invisible; and advertisement call composed of 8-14 notes each with 4-18 pulses, and duration of 290-420 ms. The new species uses temporary creeks in rock meadows above 1.000 m a.s.l. and males calls from rock outcrops. The dorsal color pattern enables this species to camouflage in this kind of surfaces. 

  20. Geochemical and isotopic constraints on the tectonic setting of Serra dos Carajas belt, eastern Para, Brazil

    NASA Technical Reports Server (NTRS)

    Olszewski, W. J., Jr.; Gibbs, A. K.; Wirth, K. R.

    1986-01-01

    The lower part of the Serra dos Carajas belt is the metavolcanic and metasedimentary Grao para Group (GPG). The GPG is thought to unconformably overlie the older (but undated) Xingu Complex, composed of medium and high-grade gneisses and amphibolite and greenstone belts. The geochemical data indicate that the GPG has many features in common with ancient and modern volcanic suites erupted through continental crust. The mafic rocks clearly differ from those of most Archean greenstone belts, and modern MORB, IAB, and hot-spot basalts. The geological, geochemical, and isotopic data are all consistent with deposition on continental crust, presumably in a marine basin formed by crustal extension. The isotopic data also suggest the existence of depleted mantle as a source for the parent magmas of the GPG. The overall results suggest a tectonic environment, igneous sources, and petrogenesis similar to many modern continental extensional basins, in contrast to most Archean greenstone belts. The Hammersley basin in Australia and the circum-Superior belts in Canada may be suitable Archean and Proterozoic analogues, respectively.

  1. Hepatitis A seroprevalence in public school children in Campos dos Goytacazes, Rio de Janeiro State, Brazil, prior to the introduction of the hepatitis A universal childhood vaccination.

    PubMed

    Kury, Charbell Miguel; Pinto, Marcelo Alves; Silva, Jaquelline Pereira da; Cruz, Oswaldo Gonçalves; Vitral, Claudia Lamarca

    2016-11-01

    This cross-sectional study was carried out between August 2011 and July 2012 in the city of Campos dos Goytacazes in Rio de Janeiro State, Brazil. Dried blood spot samples were collected on filter paper from 919 individuals between the ages of 1 and 19 and were tested for antibodies against the hepatitis A virus (anti-HAV). The total prevalence was 20.7%, while 94.7% of children under the age of 5 were found to be susceptible to HAV infection. The prevalence of anti-HAV increased with age, reaching 33.3% among individuals aged between 15 and 19, thereby indicating that this municipality has a low level of endemicity for hepatitis A. Age, non-white skin color, accustomed to swimming in the river and more than five people living at home were the factors that were associated with an increase in the chance of a positive anti-HAV result. Mother's education level (secondary or tertiary) was considered a protective factor for HAV infection. The data obtained showed that a large proportion of the children from Campos dos Goytacazes were at risk of HAV infection, which should be minimized with the introduction of the vaccination program against hepatitis A that was launched in the municipality in 2011.

  2. A estabilidade dos PAHS em função da energia da radiação interestelar nas faixas UV e raios-X

    NASA Astrophysics Data System (ADS)

    Pinotti, R.; Costa, R. K.; Boechat-Roberty, H. M.; Lago, A.; Souza, G. B.

    2003-08-01

    A nebulosa CRL 618, uma proto-nebulosa planetária cuja nuvem molecular espessa envolve uma estrela B0, contém uma grande quantidade de C2H2 e CH4. Estas moléculas são consideradas os tijolos da criação de grandes moléculas carbonadas como os Hidrocarbonetos Policíclicos Aromáticos (PAHs). Esta nebulosa, por estar exposta a intensos campos de UV e Raios-X, é uma região de fotodissociação molecular que propicia a formação de novas moléculas, confirmada pela presença de C4H2 e C6H6 (Benzeno), que é a unidade básica dos PAHs. Atribui-se a esta família de moléculas orgânicas duas propriedades fundamentais, a resistência para sobreviver ao campo de radiação UV interestelar e a geração das bandas de emissão não identificadas (UIR) observadas no infravermelho. No entanto, alguns autores questionam a resistência dos PAHs ao campo de radiação UV interestelar. Empregando a técnica de Espectrometria de Massas por Tempo de Vôo, no modo de coincidência fotoelétron-fotoíon, estudamos a ionização e fragmentação das seguintes moléculas: Benzeno, Benzeno deuterado, Naftaleno, Antraceno e Fenantreno. Utilizamos uma fonte de Hélio monocromática em 21,21 eV (584,5 Å) e a radiação Síncroton do Laboratório Nacional de Luz Síncroton (LNLS) em diferentes energias nas proximidades da borda do C 1s ( 290 eV). Comprovamos a estabilidade dos PAHs sob ação de UV (21,21 eV), onde eles apresentam um baixo nível de fotodissociação, produzindo fragmentos ionizados com rendimento total na ordem de 5 por cento em relação ao íon molecular pai. Entretanto, em altas energias, na faixa de Raios-X, a quebra destas moléculas torna-se mais intensa, com a produção de muitos fragmentos. Como uma das rotas de fragmentação do Naftaleno é [(C10H8) = > (C6H6+) + (C4H2) + (e-)], e como temos as evidências observacionais da existência do C4H2 e C6H6 na nebulosa CRL 618, sugerimos que este ambiente também possui o Naftaleno.

  3. [The Arabic influence in the "Colóquios dos simples e drogas da India" of Garcia da Orta].

    PubMed

    Ricordel, Joëlle

    2015-09-01

    The "Colóquios dos simples e drogas he cousas medicinais de Índia" (Conversations on the simples, drugs and medicinal substances of India) (1563) of Garcia da Orta is a botanical and pharmacognosy book. The author is a Portuguese physician who studied in the Spanish universities and practiced medicine mainly in India. He studies in short chapters presented in the form of dialogues about sixty simples. Sources to which he refers are indicative of a "classical" training, but also the mark of a curious and open mind to different cultures. The Arabic sources are numerous and mainly concern the identification of substances by abundant synonyms of their names in foreign languages and different medicinal uses that may have been done by the ancient physicians. However, Da Orta is critical with respect to these sources, seeking contradictions and differences of opinion among authors. He confronts them with the oral information collected thanks to a wide network of contacts.

  4. A novel germline mutation (c.A527G) in STK11 gene causes Peutz–Jeghers syndrome in a Chinese girl

    PubMed Central

    Zhao, Zi-Ye; Jiang, Yu-Liang; Li, Bai-Rong; Yang, Fu; Li, Jing; Jin, Xiao-Wei; Sun, Shu-Han; Ning, Shou-Bin

    2017-01-01

    Abstract Rationale: Peutz–Jeghers syndrome (PJS) is a Mendelian autosomal dominant disease caused by mutations in the tumor suppressor gene, serine/threonine kinase 11 (STK11). The features of this syndrome include gastrointestinal (GI) hamartomas, melanin spots on the lips and the extremities, and an increased risk of developing cancer. Early onset of disease is often characterized by mucocutaneous pigmentation and intussusception due to GI polyps in childhood. Patient concerns: A girl with a positive family history grew oral pigmentation at 1 and got intussusception by small bowel hamartomas at 5. Diagnoses: She was diagnosed with PJS based on oral pigmentation and a positive family history of PJS. Interventions: Enteroscopy was employed to treat the GI polyps. Sanger sequencing was used to investigate STK11 mutation in this family. Outcomes: A large jejunal polyp together with other smaller ones was resected, and the girl recovered uneventfully. We discovered a heterozygous substitution in STK11, c.A527G in exon 4, in the girl and her father who was also a PJS patient, and the amine acid change was an aspartic acid-glycine substitution in codon 176. This mutation was not found in other healthy family members and 50 unrelated non-PJS controls, and it is not recorded in databases, which prove it a novel mutation. Evolutionary conservation analysis of amino acid residues showed this aspartic acid is a conserved one between species, and protein structure prediction by SWISS-MODEL indicated an obvious change in local structure. In addition, PolyPhen-2 score for this mutation is 1, which indicates it probably damaging. Lessons: PJS can cause severe complication like intussusception in young children, and early screening for small bowel may be beneficial for these patients. The mutation of STK11 found in this girl is a novel one, which enlarges the spectrum of STK11. Our analysis supported it a causative one in PJS. PMID:29245219

  5. Treatment of true precocious puberty with a potent luteinizing hormone-releasing factor agonist: effect on growth, sexual maturation, pelvic sonography, and the hypothalamic-pituitary-gonadal axis.

    PubMed

    Styne, D M; Harris, D A; Egli, C A; Conte, F A; Kaplan, S L; Rivier, J; Vale, W; Grumbach, M M

    1985-07-01

    We used the LHRH agonist D-Trp6-Pro6-N-ethylamide LHRH (LHRH-A) to treat 19 children (12 girls and 7 boys) with true precocious puberty. Fourteen patients had idiopathic true precocious puberty, 4 had a hamartoma of the tuber cinereum, and 1 had a hypothalamic astrocytoma. Basal gonadotropin secretion and responses to native LHRH decreased within 1 week of initiation LHRH-A therapy, and sex steroid secretion decreased within 2 weeks to or within the prepubertal range. Ultrasonographic evaluation of the uterus indicated a postmenarchal size and shape in all 11 girls studied before treatment, which reverted to prepubertal size and configuration in 5 girls during LHRH-A therapy. The enlarged ovaries decreased in size and the multiple ovarian follicular cysts regressed. Sexual characteristics ceased advancing or reverted toward the prepubertal state in all patients receiving therapy for 6-36 months. All 5 girls with menarche before therapy had no further menses. Three girls had hot flashes after LHRH-A-induced reduction of the plasma estradiol concentration. Height velocity, SDs above the mean height velocity for age, and SDs above the mean height for age decreased during LHRH-A therapy; the velocity of skeletal maturation decreased after 12 months of LHRH-A therapy and was sustained during continued therapy over 18-36 months. In 4 patients, a subnormal growth rate (less than 4.5 cm/yr) occurred during LHRH-A therapy. Six patients had cutaneous reactions of LHRH-A, but no demonstrable circulating antibodies to LHRH-A. In 2 patients in whom LHRH-A therapy was discontinued because of skin reactions, precocious sexual maturation resumed at the previous rate for the ensuing 6-12 months; subsequently, they were desensitized to LHRH-A, and during a second course of therapy, their secondary sexual development and sex steroid levels again quickly decreased. LHRH-A proved an effective and safe treatment for true precocious puberty in boys as well as girls with central

  6. Tumoral, quasitumoral and pseudotumoral lesions of the superficial and somatic soft tissue: new entities and new variants of old entities recorded during the last 25 years. Part XII: appendix.

    PubMed

    Bisceglia, M; Spagnolo, D; Galliani, C; Fisher, C; Suster, S; Kazakov, D V; Cooper, K; Michal, M

    2006-08-01

    In an eleven part series published in Pathologica, we have presented various tumoral, quasitumoral and pseudotumoral lesions of the superficial and somatic soft tissue (ST), which emerged as new entities or as variants of established entities during the last quarter of a century. Detailed clinicomorphological and differential diagnostic features of approximately sixty entities were chosen on the basis of their clinical significance and morphologic distinctiveness. The series included fibrous and myofibroblastic tumors (e.g. solitary fibrous tumor, high grade classic and pigmented dermatofibrosarcoma protuberans, inflammatory myofibroblastic tumor and myofibrosarcomas), fibromyxoid and fibrohistiocytic neoplasms (e.g., Evans' tumor, phosphaturic mesenchymal tumor, inflammatory myxohyaline tumor), special adipocytic/vascular/and smooth muscle lesions (e.g., chondroid lipoma, Dabska's tumor, ST hemangioblastoma, lipoleiomyosarcoma), epithelioid mesenchymal malignancies of diverse lineages (e.g., epithelioid liposarcoma, proximal-type epithelioid sarcoma, neuroendocrine extraskeletal chondromyxoid sarcoma), ST Ewing's tumor and peripheral nerve sheath tumors (perineuriomas and pigmented and rosetting tumors of the schwannoma/neurofibroma group), extranodal dendritic or histiocytic proliferative processes (follicular dendritic cell sarcoma, Rosai-Dorfman disease, Castleman's disease, and plexiform xanthomatous tumor), and tumors with myoepithelial differentiation. The section devoted to selected pseudotumoral entities considered representatives of the hamartoma group (neural fibrolipomatous hamartoma, ectopic hamartomatous thymoma, rudimentary meningocele), metabolic diseases (amyloid tumor, nephrogenic fibrosing dermopathy, tophaceous pseudogout, pseudoinfiltrative parathyromatosis), stromal tissue reactions to trauma (fibroosseous pseudotumors of digits) and infections (bacillary angiomatosis), and normal organs (glomus coccygeum). To conclude the descriptive phase

  7. 99mTc-EDDA/HYNIC-TOC scintigraphy in the differential diagnosis of solitary pulmonary nodules.

    PubMed

    Płachcińska, Anna; Mikołajczak, Renata; Maecke, Helmut R; Michalski, Andrzej; Rzeszutek, Katarzyna; Kozak, Józef; Kuśmierek, Jacek

    2004-07-01

    Forty-three consecutive patients with solitary pulmonary nodules (SPNs) on chest radiographs were studied scintigraphically after administration of the somatostatin analogue (99m)Tc-EDDA/HYNIC-TOC. The objective of the study was to assess the usefulness of the procedure for differentiation of SPNs as malignant or benign. The administered activity was 740-925 MBq, and a single-photon emission computed tomography imaging technique was employed. Verification of the nodule aetiology was based on histology or cytology and bacteriology. A stable tumour size on chest radiography for at least 3 years was accepted as an additional criterion of benignity. In 29 patients, nodules were found to be malignant. The diagnoses included ten adenocarcinomas, five squamous cell carcinomas, two large cell carcinomas, six non-small cell lung cancers without specification of the more detailed morphology, two small cell lung cancers, two typical carcinoids and two metastatic tumours (leiomyosarcoma and malignant melanoma). In 14 patients the following benign tumours were diagnosed: four tuberculomas, one other granuloma, three hamartomas, one non-specific inflammatory infiltrate, one abscess, one peripheral carcinoid with the morphological characteristics of a benign tumour, one ectopic lesion of thyroid tissue and two benign tumours of unspecified aetiology with a stable size over 3 and 5 years respectively. Positive scintigraphic results were obtained in 26 of the 29 patients (90%) with malignant SPNs; among these, 24 of the 25 (96%) cases of primary pulmonary carcinoma yielded positive results. The remaining two false negative cases were the metastatic tumours, liposarcoma and melanoma. Of the 14 benign lesions, ten (71%) did not accumulate the radiopharmaceutical. The remaining four benign tumours that were visible on scintigrams comprised one tuberculoma, one hamartoma, one abscess and one case in which the diagnosis could not be established (the tumour had a stable size over 3 years

  8. [Manifestatations of violence in adolescents in public schools in the municipality of Jaboatão dos Guararapes].

    PubMed

    Melo, Monica Cristina Batista de; Barros, Erika Neves de; Almeida, Andréa Maria Lages Gomes de

    2011-10-01

    Adolescence is a period in human development characterized by profound biological, psychological and social modifications affecting both adolescents and their entire social and family environment. This stage, which has good character forming opportunities, is also permeated by various risks, including violence. Nowadays, violence is the main cause of death among adolescents and is considered a public health concern. This research sought to investigate manifestations of violence in adolescents from public schools of the municipality of Jaboatão dos Guararapes. This is a qualitative study, in which the data obtained from a questionnaire was analyzed using the content analysis technique. The results show that the main manifestations of violence involve harm to others. Drug usage was revealed as the main motive behind physical violence. The deployment of more policemen was the strategy most cited and strengthening the family unit was the most commented preventative measure. In conclusion, the importance of further discussion about disguised manifestations of violence is clear as this would contribute to reflections on new prevention proposals and greater visibility of the phenomenon.

  9. Collision Tumor between Trichofolliculoma and Melanocytic Nevus.

    PubMed

    Bolte, Christel; Cullen, Roberto; Sazunic, Ivo

    2017-01-01

    Trichofolliculoma (TF) is a hamartomatous hair follicle-related tumor, clinically described as a dome-shaped papule with a central pore crossed by one or more silky white hairs. Histologically, it described as a cystic cavity containing keratinous debris, hair shaft fragments, and numerous hair follicles arising from its linings. Collision or compound tumors are a coexistence of two or more identifiable tumors in the same lesion. We present a case of a 47-year-old man with a lesion on his left cheek clinically characterized as a TF. However, the histopathological study reveals a collision tumor involving a TF and a melanocytic nevus. Collision tumors involving melanocytic nevi and hair follicle-related tumors have been previously reported, such as desmoplastic trichoepithelioma, epidermoid cyst, folliculosebaceous cystic hamartoma, and trichoadenoma.

  10. Familial Investigations of Childhood Cancer Predisposition

    ClinicalTrials.gov

    2018-01-03

    Acute Leukemia; Adenomatous Polyposis; Adrenocortical Carcinoma; AML; BAP1 Tumor Predisposition Syndrome; Carney Complex; Choroid Plexus Carcinoma; Constitutional Mismatch Repair Deficiency Syndrome; Diamond-Blackfan Anemia; DICER1 Syndrome; Dyskeratosis Congenita; Emberger Syndrome; Familial Acute Myeloid Leukemia; Familial Adenomatous Polyposis; Fanconi Anemia; Familial Cancer; Familial Wilms Tumor; Familial Neuroblastoma; GIST; Hereditary Breast and Ovarian Cancer; Hereditary Paraganglioma-Pheochromocytoma Syndrome; Hodgkin Lymphoma; Juvenile Polyposis; Li-Fraumeni Syndrome; Lynch Syndrome; MDS; Melanoma Syndrome; Multiple Endocrine Neoplasia Type 1; Multiple Endocrine Neoplasia Type 2; Neuroblastoma; Neurofibromatosis Type 1; Neurofibromatosis Type II; Nevoid Basal Cell Carcinoma Syndrome; Non Hodgkin Lymphoma; Noonan Syndrome and Other Rasopathy; Overgrowth Syndromes; Pancreatic Cancer; Peutz-Jeghers Syndrome; Pheochromocytoma/Paraganglioma; PTEN Hamartoma Tumor Syndrome; Retinoblastoma; Rhabdoid Tumor Predisposition Syndrome; Rhabdomyosarcoma; Rothmund-Thomson Syndrome; Tuberous Sclerosis; Von Hippel-Lindau Disease

  11. Michelin tire baby syndrome: a review of the literature and a proposal for diagnostic criteria with adoption of the name circumferential skin folds syndrome.

    PubMed

    Rothman, Ilene L

    2014-01-01

    The term Michelin tire baby (MTB), named for the cartoon mascot of the Michelin Tire Company, has been used to describe babies with multiple symmetric circumferential rings of folded skin. In those reported with this phenotype who had skin biopsies, pathology has shown nevus lipomatosis, smooth muscle hamartoma, degenerative collagen, and scarring. Others did not undergo biopsy or had normal skin. Many individuals with the MTB phenotype have had a variety of other congenital anomalies. I review the literature on MTB and the history of the designation Michelin tire baby Syndrome (MTBS). Because the term MTBS has been poorly defined or not defined at all, I propose strict criteria for diagnosis. In doing so, it is recommended that the syndrome be renamed to avoid further confusion. © 2014 Wiley Periodicals, Inc.

  12. Determinação de elementos próprios dos asteróides troianos: comparação entre as teorias semi-analítica e sintética

    NASA Astrophysics Data System (ADS)

    Roig, F.; Beaugé, C.

    2003-08-01

    Além do cálculo semi-analítico de elementos próprios dos asteróides Troianos (Beaugé & Roig 2001, Icarus 153, 391), recentemente foi apresentado um novo conjunto destes elementos próprios determinado através de uma teoria sintética (Knenezevic & Milani 2003, comunicação pessoal). As bases de dados contendo estas determinações estão disponiveis na pagina web do Asteroid Dynamical Site (http://hamilton.dm.unipi.it/cgi-bin/astdys/astibo). Nesta comunicação apresentamos os primeiros resultados de um estudo comparativo entre ambos conjuntos de elementos próprios, analisando suas vantagens e desvantagens, assim como os limites de precisão de cada conjunto. Mostramos que os elementos próprios sintéticos são mais precisos que os smi-analíticos para grandes amplitudes de libração do ângulo s = l-lJup, embora acontece o contrario para os corpos cuja amplitude de libração é muito pequena. Finalmente discutimos a influencia destes erros na determinação de familias de asteroides e da estrutura resonante em torno dos pontos Lagrangeanos L4 e L5.

  13. Potential of DosR and Rpf antigens from Mycobacterium tuberculosis to discriminate between latent and active tuberculosis in a tuberculosis endemic population of Medellin Colombia.

    PubMed

    Arroyo, Leonar; Marín, Diana; Franken, Kees L M C; Ottenhoff, Tom H M; Barrera, Luis F

    2018-01-08

    Tuberculosis (TB) remains one of the most deadly infectious diseases. One-third to one-fourth of the human population is estimated to be infected with Mycobacterium tuberculosis (Mtb) without showing clinical symptoms, a condition called latent TB infection (LTBI). Diagnosis of Mtb infection is based on the immune response to a mixture of mycobacterial antigens (PPD) or to Mtb specific ESAT-6/CFP10 antigens (IGRA), highly expressed during the initial phase of infection. However, the immune response to PPD and IGRA antigens has a low power to discriminate between LTBI and PTB. The T-cell response to a group of so-called latency (DosR-regulon-encoded) and Resuscitation Promoting (Rpf) antigens of Mtb has been proved to be significantly higher in LTBI compared to active TB across many populations, suggesting their potential use as biomarkers to differentiate latent from active TB. PBMCs from a group LTBI (n = 20) and pulmonary TB patients (PTB, n = 21) from an endemic community for TB of the city of Medellín, Colombia, were in vitro stimulated for 7 days with DosR- (Rv1737c, Rv2029c, and Rv2628), Rpf- (Rv0867c and Rv2389c), the recombinant fusion protein ESAT-6-CFP10 (E6-C10)-, or PPD-antigen. The induced IFNγ levels detectable in the supernatants of the antigen-stimulated cells were then used to calculate specificity and sensitivity in discriminating LTBI from PTB, using different statistical approaches. IFNγ production in response to DosR and Rpf antigens was significantly higher in LTBI compared to PTB. ROC curve analyses of IFNγ production allowed differentiation of LTBI from PTB with areas under the curve higher than 0.70. Furthermore, Multiple Correspondence Analysis (MCA) revealed that LTBI is associated with higher levels of IFNγ in response to the different antigens compared to PTB. Analysis based on decision trees showed that the IFNγ levels produced in response to Rv2029c was the leading variable that best-classified disease status. Finally

  14. Geochemical and isotopic evidence for the petrogenesis and emplacement tectonics of the Serra dos Órgãos batholith in the Ribeira belt, Rio de Janeiro, Brazil

    NASA Astrophysics Data System (ADS)

    Machado, Rômulo; Philipp, Ruy Paulo; McReath, Ian; Peucat, Jean Jacques

    2016-07-01

    The Serra dos Órgãos batholith in the State of Rio de Janeiro (Brazil) is a NE-SW-trending elongated body that occupies ca. 5000 km2 in plan view. It is a foliated intrusion, especially at its borders and is crosscut by syn-magmatic shear zones, with foliations that are moderately-to steeply-dipping to the northwest and moderately-to shallow-dipping in the center and to the southeast, in a configuration of a large laccolith. It was emplaced between 560 and 570 Ma, during an extensional episode that was part of a series of events that comprise the Brasiliano Orogeny in SE Brazil, and which includes deformation, metamorphism and granite intrusion during the interval between 630 and 480 Ma. The two main rock types in the batholith are biotite-hornblende monzogranite, and biotite leucogranite, with subordinate tonalite, granodiorite, diorite, quartz diorite (enclaves), aplite and pegmatite. Harker-type diagrams help show two rock groups with similar trends of evolution: a dioritic and a granitic. The first one is tholeiitic, whereas the second is calc-alkaline, with medium-to high-K calc-alkaline affinity and metaluminous to slightly peraluminous character. In both groups strong decrease in Al2O3, MgO, FeOT and CaO relative to silica contents are observed, which is compatible with trends of fractional crystallization involving clinopyroxene and/or hornblende, plagioclase, opaque minerals, apatite, microcline and biotite. The Sr and Nd isotopic data suggest recycling of a Paleoproterozoic crust as an important petrological process to generate the batholith rocks. Geothermometry (amphibole composition) and geobarometry (saturation in zircon and apatite) indicate that most of the batholith solidified at mid to lower crustal levels at about 750 °C and between 5 and 5.5 kbar. We consider that Serra dos Órgãos crustal protoliths underwent melting caused by the interaction with hotter mafic magma at the base of the crust. These two magmas, with distinct initial

  15. Could a Collision Between a Ghost Galaxy and the Milky Way be the Origin of the VPOS or DoS?

    NASA Astrophysics Data System (ADS)

    Bohórquez, O. A.; Casas, A. R.

    2018-01-01

    At present within the area of astrophysics there are a number of unresolved problems, including the origin of the satellite galaxies of the Milky Way. Most of these galaxies are characterized as dwarf spheroidal galaxies. The large majority of them is distributed in a disk-like structure which is arranged almost perpendicular to the plane of the Galaxy, this structure is known as disk of satellites (DoS) or Vast Polar structure of Satellite galaxies (VPoS). So far there is not a model that fully reproduces the amount and spatial distribution of these galaxies. However there have been several proposed for the solutions, one of which suggests that these originated in the collision of two disk galaxies billions of years ago. Using the Gadget2 software, we have performed N-bodies numerical simulations of the collision between two disk galaxies that could give rise to disk of Milky Way satellites.

  16. mTOR, a Potential Target to Treat Autism Spectrum Disorder.

    PubMed

    Sato, Atsushi

    2016-01-01

    Mammalian target of rapamycin (mTOR) is a key regulator in various cellular processes, including cell growth, gene expression, and synaptic functions. Autism spectrum disorder (ASD) is frequently accompanied by monogenic disorders, such as tuberous sclerosis complex, phosphatase and tensin homolog tumor hamartoma syndrome, neurofibromatosis 1, and fragile X syndrome, in which mTOR is hyperactive. Mutations in the genes involved in the mTOR-mediated signaling pathway have been identified in some cases of syndromic ASD. Evidences indicate a pathogenic role for hyperactive mTOR-mediated signaling in ASD associated with these monogenic disorders, and mTOR inhibitors are a potential pharmacotherapy for ASD. Abnormal synaptic transmission through metabotropic glutamate receptor 5 may underlie in a part of ASD associated with hyperactive mTOR-mediated signaling. In this review, the relationship between mTOR and ASD is discussed.

  17. Rare desmoplastic trichilemmoma associated with sebaceous nevus.

    PubMed

    Jardim, Márcio Martins Lobo; Souza, Bruno de Castro E; Fraga, Renata Cavanellas; Fraga, Rafael Cavanellas

    2017-01-01

    Nevus sebaceous of Jadassohn is a congenital hamartoma that usually affects the scalp and face. Several benign or malignant neoplasias may develop in the lesion and the most common are trichoblastoma, syringocystadenoma papilliferum, and basal cell carcinoma. Trichilemmoma is a benign solid tumor originating from external sheath cells of pilosebaceous follicles. When it is characterized by a central zone of desmoplasia, it is called desmoplastic trichilemmoma. We report a case of a 58-year-old patient who developed a tumor in a sebaceous nevus. We performed a total excision of the lesion. Histopathological diagnosis was compatible with desmoplastic trichilemmoma. Our literature review reveals that the occurrence of trichilemmoma desmoplastic is unusual. Moreover, it can mimic an invasive carcinoma on histological and clinical examinations. This fact confirms the importance of reporting the occurrence of this rare cancer in a nevus sebaceous of Jadassohn.

  18. Histologic Mimics of Basal Cell Carcinoma.

    PubMed

    Stanoszek, Lauren M; Wang, Grace Y; Harms, Paul W

    2017-11-01

    - Basal cell carcinoma (BCC) is the most common human malignant neoplasm and is a frequently encountered diagnosis in dermatopathology. Although BCC may be locally destructive, it rarely metastasizes. Many diagnostic entities display morphologic and immunophenotypic overlap with BCC, including nonneoplastic processes, such as follicular induction over dermatofibroma; benign follicular tumors, such as trichoblastoma, trichoepithelioma, or basaloid follicular hamartoma; and malignant tumors, such as sebaceous carcinoma or Merkel cell carcinoma. Thus, misdiagnosis has significant potential to result in overtreatment or undertreatment. - To review key features distinguishing BCC from histologic mimics, including current evidence regarding immunohistochemical markers useful for that distinction. - Review of pertinent literature on BCC immunohistochemistry and differential diagnosis. - In most cases, BCC can be reliably diagnosed by histopathologic features. Immunohistochemistry may provide useful ancillary data in certain cases. Awareness of potential mimics is critical to avoid misdiagnosis and resulting inappropriate management.

  19. The abortive form of Bourneville-Pringle syndrome.

    PubMed

    Giusti, C

    2002-01-01

    To present a 26-year-old woman affected by the abortive form of Bourneville-Pringle syndrome. To our knowledge, this disease is unusual since only very few cases have been reported in the scientific literature at this time. Visual acuity was 20/20 in both eyes. No relevant ocular abnormalities were observed excepting two retinal hamartomas, a smaller one in the nasal midperiphery of the right eye and a larger one located along the super-temporal retinal vessels of the left eye. Classical signs of Bourneville-Pringle disease, such as mental retardation and epilepsy, were absent whereas a slight facial adenoma sebaceum and renal cysts represented the solely systemic manifestations of the disease. This case report confirms that retinal phakomata are a typical manifestation of Tuberous Sclerosis, even in the absence of a detected involvement of the brain.

  20. mTOR, a Potential Target to Treat Autism Spectrum Disorder

    PubMed Central

    Sato, Atsushi

    2016-01-01

    Mammalian target of rapamycin (mTOR) is a key regulator in various cellular processes, including cell growth, gene expression, and synaptic functions. Autism spectrum disorder (ASD) is frequently accompanied by monogenic disorders, such as tuberous sclerosis complex, phosphatase and tensin homolog tumor hamartoma syndrome, neurofibromatosis 1, and fragile X syndrome, in which mTOR is hyperactive. Mutations in the genes involved in the mTOR-mediated signaling pathway have been identified in some cases of syndromic ASD. Evidences indicate a pathogenic role for hyperactive mTOR-mediated signaling in ASD associated with these monogenic disorders, and mTOR inhibitors are a potential pharmacotherapy for ASD. Abnormal synaptic transmission through metabotropic glutamate receptor 5 may underlie in a part of ASD associated with hyperactive mTOR-mediated signaling. In this review, the relationship between mTOR and ASD is discussed. PMID:27071790

  1. Implementing a knowledge translation intervention in long-term care: feasibility results from the Vitamin D and Osteoporosis Study (ViDOS).

    PubMed

    Kennedy, Courtney C; Thabane, Lehana; Ioannidis, George; Adachi, Jonathan D; Papaioannou, Alexandra

    2014-12-01

    To evaluate the feasibility of implementing an interdisciplinary, multifaceted knowledge translation intervention within long-term care (LTC) and to identify any challenges that should be considered in designing future studies. Cluster randomized controlled trial. Forty LTC homes across the province of Ontario, Canada. LTC teams composed of physicians, nurses, pharmacists, and other staff. Cluster-level feasibility measures, including recruitment, retention, data completion, and participation in the intervention. A process evaluation was completed by directors of care indicating which process/policy changes had been implemented. Recruitment and retention rates were 22% and 63%, respectively. Good fidelity with the intervention was achieved, including attendance at educational meetings. After ViDOS, 7 process indicators were being newly implemented by more than 50% of active intervention homes. Despite recruitment and retention challenges, the multifaceted intervention produced a number of policy/process changes and had good intervention fidelity. This study is registered at ClinicalTrials.gov NCT01398527. Copyright © 2014. Published by Elsevier Inc.

  2. Factores socio-económicos asociados a la percepción de situación socioeconómica entre adultos mayores de dos países latinoamericanos

    PubMed Central

    Brenes-Camacho, Gilbert

    2014-01-01

    El objetivo principal del artículo es estudiar la asociación entre la percepción subjetiva sobre la situación económica propia y una serie de medidas objetivas de bienestar socioeconómico –fuentes de ingresos, tenencia de vivienda, nivel educativo y transferencias familiares informales de dinero- entre adultos mayores de dos países Latinoamericanos: México y Costa Rica. Los datos se obtienen de las primeras rondas de dos encuestas sobre envejecimiento: CRELES para Costa Rica y ENASEM para México. La variable dependiente más importante se obtiene de las respuestas a las pregunta “¿Cómo califica su situación económica actual?” en Costa Rica y “¿Diría usted que su situación económica es…?” en México. Para ambas encuestas, las respuestas se codificaron en forma binaria; el código 0 representa las categorías Excelente, Muy buena y Buena, y el código 1 representa a las categorías Regular y Mala. Se encontró que el ingreso por jubilación es un importante determinante de la percepción de bienestar en ambos países. En Costa Rica, el ingreso del cónyuge y la tenencia de vivienda son importantes predictores de la percepción de bienestar, mientras que en México, los ingresos por transferencias están fuertemente asociados con dicha percepción. PMID:25360057

  3. ESR dating of Smilodon populator from Toca de Cima dos Pilão, Piauí, Brazil.

    PubMed

    Kinoshita, Angela; Mollemberg, Michelle; Santana, William; G Figueiredo, Ana Maria; Guidon, Niede; Fátima da Luz, Maria de; Guérin, Claude; Baffa, Oswaldo

    2017-02-01

    Smilodon is a genus of big cats that lived from the early to the late Pleistocene in regions extending from North to South America. The fossil records of the "saber-toothed cats" are uneven, with some taxa being quite abundant in certain regions. In Brazil, Smilodon populator is a well-known species whose remains, although scarce in comparison to other large mammals, are found all across the country. In particular, there are multiple records of this species in the region of the Serra da Capivara National Park. This area was home to a rich Pleistocene-Holocene fauna, including many mammals. Here, we report on the Electron Spin Resonance dating of a Smilodon populator tooth found in "Toca de Cima dos Pilão", located in the surroundings of the Serra da Capivara National Park. The equivalent dose found after exponential fitting of dose-response curve was (2.7±0.3)x10 2 Gy. Neutron Activation Analysis was used to determine the concentration of radioisotopes present in the sample and in the sediment to calculate the internal and external dose rates. The result of age found is 93±9ka, which confirmed the presence of this species in Serra da Capivara National Park in the late Pleistocene. Copyright © 2016 Elsevier Ltd. All rights reserved.

  4. Morphology and morphometry of upland lakes over lateritic crust, Serra dos Carajás, southeastern Amazon region.

    PubMed

    Silva, Marcio S DA; Guimarães, José T F; Souza Filho, Pedro W M; Nascimento Júnior, Wilson; Sahoo, Prafulla K; Costa, Francisco R DA; Silva Júnior, Renato O; Rodrigues, Tarcísio M; Costa, Marlene F DA

    2018-01-01

    High-resolution satellite images, digital elevation models, bathymetric and sedimentological surveys coupled with statistical analysis were used to understand the physical environment and discuss their influence on water quality of the five upland lakes of Serra Sul dos Carajás, southeast Amazonia. The lakes have mid-altitude ranges (elevation), very small (catchment) and shallow to very shallow (central basins). Based on the length, area and volume, Violão and TI (Três Irmãs)-3 lakes may present large vertical movements of the water due to wind action and weakly stratified waters. Trophic conditions based on depth and shore development (Ld) parameters must be used with caution, since Amendoim Lake is relatively deep, but it is oligotrophic to ultra-oligotrophic. Ld values suggest that the lakes are circular to subcircular and are likely formed by solution process, as also suggested by volume development. TI-2 Lake is only presenting convex central basin and has highest dynamic ratio (DR), thus it may have high sedimentation and erosion rates. Based on the relationship between studied parameters, morphometric index and DR likely influence temperature and dissolved oxygen of waters of TI-2 Lake due to its depth profile and wind-induced surface mixing. Nevertheless, water quality parameters are controlled by catchment characteristics of the lakes.

  5. Gastrointestinal parasites among felids inhabiting the Serra dos Órgãos National Park, Rio de Janeiro, Brazil.

    PubMed

    Dib, Laís Verdan; Cronemberger, Cecília; Pereira, Fabiane de Aguiar; Bolais, Paula Forain; Uchôa, Claudia Maria Antunes; Bastos, Otilio Machado Pereira; Amendoeira, Maria Regina Reis; Barbosa, Alynne da Silva

    2018-05-24

    This study aimed to investigate the species of felids that inhabit the Serra dos Órgãos National Park (Parnaso) and gastrointestinal parasites at various stages of their life cycles in the feces of these animals. Between 2013 and 2015, felid feces were collected from trails in Parnaso. The sampling points were georeferenced. A total of 82 fecal samples were processed, of which 79 were collected on the ground, two from captured felids and one from a necropsied animal. All samples underwent coproparasitological techniques. Samples collected from the environment underwent additional trichological analysis. Fur patterns corresponding to Leopardus guttulus, Leopardus pardalis, Leopardus wiedii and Puma yagouaroundi were observed in 32 of the samples collected on the soil. High frequency of potentially parasitic evolving forms (88.6%) was observed in felid feces, particularly eggs of the family Diphyllobothriidae (68.6%). Besides, were also detected, eggs of superfamily Ascaridoidea (42.9%), nematode larvae (28.6%), eggs of order Strongylida (28.6%), Capillaria sp. (8.6%), Trichuris sp. (8.6%), eggs of order Spirurida (2.9%), unsporulated coccidian oocysts (8.6%) and Eimeria sp. (2.9%). Felid feces presented higher frequency of polyparasitism (60%) than monoparasitism (28.6%).

  6. Teenage pregnancies in Mozambique: the experience of "Servicios Amigos dos Adolescentes" clinics in Beira.

    PubMed

    Pizzol, Damiano; Di Gennaro, Francesco; Boscardin, Chiara; Putoto, Giovanni; Cuppini, Elena; Pita, Graciana; George, Alexandra; Monno, Laura; Saracino, Annalisa; Da Dalt, Liviana; De Palma, Angela

    2018-03-01

    The purpose of this article is to provide insights into the demand for pregnancy-related health services by adolescent girls and young women in Mozambique. We analysed the patient registers for the first year of operation (2014) of the Servicios Amigos dos Adolescentes (SAAJ) [Friendly Services for Adolescents] clinics in Beira, Mozambique. These registers provide details of the service demands of, and services provided to the 8 290 adolescent girls and young women who accessed the 6 SAAJ clinics in 2014. Analysis of that record, with disaggregation of the patients according to age (9 years or less; 10-14; 15-19; 20-24; 25 and older), show that 3 021 (36%) were pregnant or had previously been pregnant; most being girls in the 15-19 age band (59%). Being pregnant or having been pregnant previously was associated with dropping out of school. Of all the girls and women, 60% agreed to HIV testing and counselling; the HIV prevalence rate amongst this group was 4-5% amongst adolescents and 25% amongst women 25 years and older. A minority of the girls and women who were pregnant or had been pregnant previously agreed to HIV testing and counselling. Notwithstanding the limitations for analysis, the results were alarming: substantially high HIV prevalence rates were indicated (2% amongst 10-14 year old girls; 8% amongst 15-19 year olds; 10% amongst 20-24 year olds; and 28% amongst >24 year olds). The data from the SAAJ clinics and results pertain only to conditions in Beira. However, as the first empirical assessment of pregnancy-related service demand amongst adolescent girls and young women in the country and involving a relatively large sample, we contend that this study affirms the need for expansion of sexual and reproductive health (SRH) services, including HIV services, for adolescent girls and young women in Mozambique.

  7. Lessons Learned from Sleep Education in Schools: A Review of Dos and Don'ts

    PubMed Central

    Blunden, Sarah; Rigney, Gabrielle

    2015-01-01

    Study Objectives: Sleep duration and quality are associated with negative neuropsychological and psychosocial outcomes in children and adolescents. However, community awareness of this is low and sleep education programs in schools are attempting to address this issue. Several studies now exist assessing the efficacy of these sleep education programs for improving sleep knowledge, sleep hygiene and sleep patterns. This paper presents these sleep education programs, most particularly, it presents the strengths and weaknesses of the current available studies in the hope that this can identify areas where future sleep education programs can improve. Methods: A systematic search of all school-based sleep education studies in adolescents was undertaken. Studies were scrutinized for author, teacher and participant comment regarding strengths and limitations of each study, which were then extracted and summarized. Results: Two specific types of sleep education programs emerged from the review, those that sought to change sleep behavior and those that sought simply to disseminate information. Issues that dictated the strength or weakness of a particular study including who delivers the program, the theoretical basis, the tools utilized to measure sleep patterns, the content, and their capacity to engage students were assessed. Sleep education was considered important by teachers, students and parents alike. Conclusions: Future sleep education programs need to take into account lessons learned from previous sleep education efforts in order to maximize the potential for sleep education programs to improve the sleep health of our young people. Commentary: A commentary on this article appears in this issue on page 595. Citation: Blunden S, Rigney G. Lessons learned from sleep education in schools: a review of dos and don'ts. J Clin Sleep Med 2015;11(6):671–680. PMID:25766709

  8. Multicentric primary extramammary Paget disease: a Toker cell disorder?

    PubMed

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

    2014-07-01

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

  9. Geology, hydrology, and water quality of the Tracy-Dos Palos area, San Joaquin Valley, California

    USGS Publications Warehouse

    Hotchkiss, W.R.; Balding, G.O.

    1971-01-01

    The Tracy-Dos Palos area includes about 1,800 square miles on the northwest side of the San Joaquin Valley. The Tulare Formation of Pliocene and Pleistocene age, terrace deposits of Pleistocene age, and alluvium and flood-basin deposits of Pleistocene and Holocene age constitute the fresh ground-water reservoir Pre-Tertiary and Tertiary sedimentary and crystalline rocks, undifferentiated, underlie the valley and yield saline water. Hydrologically most important, the Tulare Formation is divided into a lower water-bearing zone confined by the Corcoran Clay Member and an upper zone that is confined, semiconfined, and unconfined in different parts of the area. Alluvium and flood-basin deposits are included in the upper zone. Surficial alluvium and flood-basin deposits contain a shallow water-bearing zone. Lower zone wells were flowing in 1908, but subsequent irrigation development caused head declines and land subsidence. Overdraft in both zones ended in 1951 with import of surface water. Bicarbonate water flows into the area from the Sierra Nevada and Diablo Range. Diablo Range water is higher in sulfate, chloride, and dissolved solids. Upper zone water averages between 400 and 1,200 mg/l (milligrams per liter) dissolved solids and water hardness generally exceeds 180 mg/l as calcium carbonate. Nitrate, fluoride, iron, and boron occur in excessive concentrations in water from some wells. Dissolved constituents in lower zone water generally are sodium chloride and sodium sulfate with higher dissolved solids concentration than water from the upper zone. The foothills of the Diablo Range provide favorable conditions for artificial recharge, but shallow water problems plague about 50 percent of the area and artificial recharge is undesirable at this time.

  10. Holocene history of a lake filling and vegetation dynamics of the Serra Sul dos Carajás, southeast Amazonia.

    PubMed

    Guimarães, José T F; Sahoo, Prafulla K; Souza-Filho, Pedro W M; Figueiredo, Mariana M J Costa DE; Reis, Luiza S; Silva, Marcio S DA; Rodrigues, Tarcísio M

    2017-07-24

    Down-core changes in sedimentary facies, elemental geochemistry, pollen, spore, δ13C, δ15N and radiocarbon records from a filled lake, named R4, of the Serra Sul dos Carajás were used to study the relationship between the paleomorphological and paleoecological processes and their significance for Holocene paleoclimatology of the southeast Amazonia. The sediment deposition of the R4 lake started around 9500 cal yr BP. Increase of detrital components from 9500 to 7000 cal yr BP suggests high weathering of surrounding catchment rocks and soils, and deposition into the lake basin under mudflows. At that time, montane savanna and forest formation were already established suggesting predominance of wet climate. However, from 7000 to 3000 cal yr BP, a decline of detrital input occurred. Also, forest formation and pteridophytes were declined, while palms and macrophytes were remained relatively stable, indicating that water levels of the lake is likely dropped allowing the development of plants adapted to subaerial condition under drier climate conditions. After 3000 cal yr BP, eutrophication and low accommodation space lead to high lake productivity and the final stage of the lake filling respectively, and forest formation may has acquired its current structure, which suggests return of wetter climate conditions.

  11. A novel mutation of the glomulin gene in an Italian family with autosomal dominant cutaneous glomuvenous malformations.

    PubMed

    Borroni, Riccardo G; Narula, Nupoor; Diegoli, Marta; Grasso, Maurizia; Concardi, Monica; Rosso, Renato; Cerica, Alessandra; Brazzelli, Valeria; Arbustini, Eloisa

    2011-12-01

    Glomuvenous malformations (GVM) are hamartomas characterized histologically by glomus cells, which should be distinguished from glomus tumors. Familial GVM are rare, often present as multiple lesions, and exhibit familial aggregation, with autosomal dominant transmission. GVM are caused by mutations of the glomulin (GLMN) gene on chromosome 1p21-p22. Their development is thought to follow the 'two-hit' hypothesis, with a somatic mutation required in addition to the inherited germline mutation. We describe a novel GLMN mutation in an Italian family with GVM in which some members present with the less commonly observed phenotype of solitary lesions. A second somatic 'hit' mutation in GLMN was not discovered in our family. We further provide histological, immunohistochemical and electron microscopic data exhibiting the classic features of GVM. The diagnosis of GVM is critical because of distinction from venous malformations and blue rubber bleb nevus syndrome, which may demonstrate clinical similarities but require different treatment. © 2011 John Wiley & Sons A/S.

  12. Neoplastic and Nonneoplastic Cutaneous Tumors of Dogs in Grenada, West Indies

    PubMed Central

    Chikweto, A.; McNeil, P.; Bhaiyat, M. I.; Stone, D.; Sharma, R. N.

    2011-01-01

    This retrospective survey was undertaken between 2002 and 2007 on samples from dogs residing in Grenada. The objectives of the study were to identify the most common histologic types of canine cutaneous tumors, determine the relative frequency of each tumor type, and compare results to reports from other regions. In a series of 225 skin masses examined, the proportion of neoplasms was 72% whereas nonneoplastic tumors accounted for 15.6%, and inflammatory conditions constituted 12.4%. There were 10 types of nonneoplastic tumors with hamartomas being the most common (28.5%), followed by sebaceous hyperplasia (25.7%) and fibroepithelial polyps (22.8%). The 10 most common cutaneous neoplasms were hemangiosarcomas (19.1%), histiocytomas (8.6%), melanocytomas (8%), mast cell tumors (6.8%), lipomas (6.8%), hemangiopericytomas (6.2%), papillomas (5.6%), fibrosarcomas (5.6%), hemangiomas (4.9%), and squamous cell carcinomas (4.3%). Tumors of vascular origin and transmissible venereal tumors were more common in dogs in our study than reported from other regions. PMID:23738097

  13. Seizures presenting as incessant laughter: a case of gelastic epilepsy.

    PubMed

    Holmes, Christina M; Goldman, Mitchell J

    2012-12-01

    Gelastic seizures are defined as seizure activity manifesting as laughter inappropriate to the situation, with supporting evidence on electroencephalogram or magnetic resonance imaging. Gelastic seizures are most commonly reported in patients with hypothalamic hamartomas causing precocious puberty. The differential diagnosis of incessant laughter is important to recognize in the Emergency Department, as some conditions warrant immediate treatment and others require further diagnostic work-up with implications for the entire family. The background and pathophysiology of gelastic epilepsy will be discussed. The case of a previously healthy girl with acute onset of incessant laughter is reported. This patient was diagnosed with a clinical case of gelastic seizures. This case illustrates the importance of recognizing this form of seizures for accurate treatment and follow-up. This case report illustrates the importance of a broad differential for a patient presenting emergently with uncontrollable laughter. Gelastic epilepsy is relatively rare but requires further work-up and often may require chronic therapy. Copyright © 2012 Elsevier Inc. All rights reserved.

  14. A 28-Year-Old Male Patient with Nail Tumors, Skin Lesions, and Epilepsy

    PubMed Central

    Balak, Deepak M.W.; Zonnenberg, Bernard A.; Spitzer-Naaijkens, Juliette M.J.; Hulshof, Mieke M.

    2017-01-01

    Background Tuberous sclerosis complex (TSC) is an autosomal dominant disorder characterized by the development of benign hamartomas in multiple organs. Most patients with TSC exhibit cutaneous manifestations. Methods We report a 28-year-old patient with multiple pink papules at the proximal nail fold of several toes. Results Histopathological analysis of a biopsy of a papule was consistent with an ungual fibroma. Histopathological analysis of a biopsy of an elevated skin-colored plaque at the lower back was diagnostic for a Shagreen patch. These findings were consistent with a clinical diagnosis of TSC. This patient was subsequently referred to a multidisciplinary TSC clinic for further screening, which revealed a giant cell astrocytoma and multiple subependymal tubers. Annual monitoring was recommended. The skin lesions were treated with topical rapamycin ointment. Conclusions Recognizing dermatological manifestations of TSC is of importance to allow early diagnosis. TSC should be considered as a differential diagnosis in the case of ungual fibromas, even in older patients. PMID:28203158

  15. A 28-Year-Old Male Patient with Nail Tumors, Skin Lesions, and Epilepsy.

    PubMed

    Balak, Deepak M W; Zonnenberg, Bernard A; Spitzer-Naaijkens, Juliette M J; Hulshof, Mieke M

    2017-01-01

    Tuberous sclerosis complex (TSC) is an autosomal dominant disorder characterized by the development of benign hamartomas in multiple organs. Most patients with TSC exhibit cutaneous manifestations. We report a 28-year-old patient with multiple pink papules at the proximal nail fold of several toes. Histopathological analysis of a biopsy of a papule was consistent with an ungual fibroma. Histopathological analysis of a biopsy of an elevated skin-colored plaque at the lower back was diagnostic for a Shagreen patch. These findings were consistent with a clinical diagnosis of TSC. This patient was subsequently referred to a multidisciplinary TSC clinic for further screening, which revealed a giant cell astrocytoma and multiple subependymal tubers. Annual monitoring was recommended. The skin lesions were treated with topical rapamycin ointment. Recognizing dermatological manifestations of TSC is of importance to allow early diagnosis. TSC should be considered as a differential diagnosis in the case of ungual fibromas, even in older patients.

  16. Identificabilidade e estabilidade dos parâmetros no método Grade of Membership (GoM): considerações metodológicas e práticas

    PubMed Central

    Guedes, Gilvan Ramalho; Caetano, André Junqueira; Machado, Carla Jorge; Brondízio, Eduardo Sonewend

    2011-01-01

    O método Grade of Membership (GoM) tem sido cada vez mais utilizado por demógrafos brasileiros e tem a vantagem de possuir um parâmetro que mensura a heterogeneidade individual, com base nas correlações não-observáveis entre as categorias de resposta das variáveis de interesse, gerando um medida do grau de pertencimento de cada indivíduo a perfis extremos. Alguns autores, contudo, chamam atenção para questões importantes na calibragem dos modelos finais que utilizam o programa GoM versão 3.4, como o problema de identificabilidade – soluções múltiplas para parâmetros estimados. Neste artigo, é sugerido um procedimento capaz de identificar um modelo final com solução única que descreva os tipos puros mais fidedignos à base de dados, em uma tentativa de otimização. Para ilustrar esse processo, utilizou-se uma base de dados correspondente a um levantamento econômico e sociodemográfico de uma população de pequenos agricultores residentes ao longo da Rodovia Transamazônica, no Estado do Pará. Também identificou-se a existência de instabilidade nos parâmetros estimados pelo programa GoM 3.4, sendo proposto um método de estabilização de seus valores. Com esses procedimentos combinados, os usuários do programa GoM 3.4 poderão descrever sua base de dados de forma mais adequada e responder às críticas sobre questões de identificabilidade e estabilidade dos modelos resultantes. Essas soluções empíricas são relevantes por afetarem cálculos de prevalência e de incidência de eventos de interesse, além de trazerem consequências importantes sobre o ponto e o momento corretos para intervenções de políticas públicas ou de planejamento prospectivo em análises de projeção. PMID:21709732

  17. Endocrine tumours in neurofibromatosis type 1, tuberous sclerosis and related syndromes

    PubMed Central

    Lodish, Maya B.; Stratakis, Constantine A.

    2010-01-01

    Neurofibromatosis type 1 (NF-1) and tuberous sclerosis complex (TSC) are two familial syndromes known as phakomatoses that may be associated with endocrine tumors. These hereditary cutaneous conditions affect the central nervous system and are characterized by the development of hamartomas. Over the past 20 years, there have been major advances in our understanding of the molecular basis of these diseases. Both NF-1 and TSC are disorders of unregulated progression through the cell cycle, in which causative genes behave as characteristic tumor suppressor genes. The pathogenesis of these familial syndromes is linked by the shared regulation of a common pathway, the protein kinase mammalian target of rapamycin (mTOR). Additional related disorders that also converge on the mTOR pathway include Peutz-Jeghers syndrome and Cowden syndrome. All of these inherited cancer syndromes are associated with characteristic skin findings that offer a clue to their recognition and treatment. The discovery of mTOR inhibitors has led to a possible new therapeutic modality for patients with endocrine tumors as part of these familial syndromes. PMID:20833335

  18. [Features of skin graft in pediatric plastic surgery].

    PubMed

    Depoortère, C; François, C; Belkhou, A; Duquennoy-Martinot, V; Guerreschi, P

    2016-10-01

    Skin graft is a skin tissue fragment transferred from a donor site to a receiving site with a spontaneous revascularization. Basic process of plastic surgery, skin graft known in children, specific, warnings and refinements. It finds its indication in many pediatric cases: integumental diseases (neavus, hamartoma), acute burns and scars, traumatic loss of substance or surgically induced, congenital malformations of the hands and feet, etc. Specific skin graft techniques in children are developed: donor sites, sampling technique and procedure, early postoperative care. Especially in children, the scalp is a perfect site for split skin graft and technique is actively developed. Refinements and special cases are discussed: use of dermal matrices, allografts, xenografts, negative pressure therapy, prior skin expansion of the donor site. Results of skin graft in children are exposed: taking of graft, growth and shrinkage, pigmentation. Skin graft sometimes allows to stay the complex movement and get the best final benefit, permanent or at least temporary, in a growing being. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  19. Birt-Hogg-Dube Syndrome with a Novel Mutation in the FLCN Gene.

    PubMed

    Kayhan, Gulsum; Yılmaz Demirci, Nilgun; Turktas, Haluk; Ergun, Mehmet Ali

    2017-10-01

    Birt-Hogg-Dube syndrome (BHDS) is an autosomal dominant disease characterized by hair follicle hamartomas, kidney tumors, and spontaneous pneumothorax; its cause is a heterozygous mutation in the FLCN gene. Colorectal polyps and carcinoma have also been reported in BHDS. FLCN mutations can be detected in patients with isolated primary spontaneous pneumothorax (PSP), so PSP may present as part of BHDS. The aim of this report is to enhance awareness that patients presenting with spontaneous PSP should be evaluated for FLCN mutations. A 44-year-old woman with PSP and her parents were analyzed for FLCN mutations. One of the patient's paternal aunts had a PSP and two of her paternal aunts had colon cancer diagnosed at early ages. A novel in-frame deletion in the FLCN gene, c.932_933delCT (P311Rfs*78), was detected in the proband and in her unaffected father. We recommend that molecular analysis of the FLCN gene be performed in patients with PSP and their families, and that mutation carriers be examined for kidney and colon tumors.

  20. Primary intestinal lymphangiectasia treated with rapamycin in a child with tuberous sclerosis complex (TSC).

    PubMed

    Pollack, Sarah F; Geffrey, Alexandra L; Thiele, Elizabeth A; Shah, Uzma

    2015-09-01

    Primary intestinal lymphangiectasia (PIL) is a rare protein-losing enteropathy characterized by a congenital malformation of the lymphatic vessels of the small intestine causing insufficient drainage and leakage of lymph fluid. Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder characterized by benign hamartomas in multiple organ systems. While the lymphatic system has been implicated in TSC through lymphangioleiomyomatosis (LAM) and lymphedema, this paper reports the first case of PIL in TSC, a female patient with a TSC2 mutation. She developed persistent and significant abdominal distension with chronic diarrhea during her first year of life. Due to lack of treatment options and the involvement of the mTOR pathway in TSC, a trial of an mTOR inhibitor, rapamycin, was initiated. This treatment was highly effective, with improvement in clinical symptoms of PIL as well as abnormal laboratory values including VEGF-C, which was elevated to over seven times the normal upper limit before treatment. This case suggests that PIL is a rare manifestation of TSC, warranting the use of mTOR inhibitors in future studies. © 2015 Wiley Periodicals, Inc.

  1. Carmakila: An effective management by kshara karma

    PubMed Central

    Shindhe, Pradeep; Kiran, Mutnali

    2013-01-01

    Epidermal nevi are hamartomas that are characterized by hyperplasia of epidermis and adnexal structures. These nevi may be classified into a number of distinct variants, which are based on clinical morphology, extent of involvement, and the predominant epidermal structure in the lesion. Variants include verrucous epidermal nevus, nevus sebaceous, nevus comedonicus, eccrine nevus, apocrine nevus, Becker's nevus, and white sponge nevus. A 22-year-old girl approached us with complaints of blackish-colored hard growth, increasing in size over the right post-auricular region since 5 years. Ksharakarma is a procedure that involves the most important surgical, para-surgical, and critical-care procedures like incision, excision, scraping, and hemostatic locally (pratisaraneeya) and generally (panneya). Pratisaraneeya kshara is prepared with herbo-mineral medicines having an average pH of 13, possessing penetrating, corrosive, scraping, and healing properties, and are evidently indicated for external application in charmakīla. For the present case, kshara karma was preferred for application as the lesion was bigger in size and the results were appreciable clinically. PMID:24250149

  2. Carmakila: An effective management by kshara karma.

    PubMed

    Shindhe, Pradeep; Kiran, Mutnali

    2013-07-01

    Epidermal nevi are hamartomas that are characterized by hyperplasia of epidermis and adnexal structures. These nevi may be classified into a number of distinct variants, which are based on clinical morphology, extent of involvement, and the predominant epidermal structure in the lesion. Variants include verrucous epidermal nevus, nevus sebaceous, nevus comedonicus, eccrine nevus, apocrine nevus, Becker's nevus, and white sponge nevus. A 22-year-old girl approached us with complaints of blackish-colored hard growth, increasing in size over the right post-auricular region since 5 years. Ksharakarma is a procedure that involves the most important surgical, para-surgical, and critical-care procedures like incision, excision, scraping, and hemostatic locally (pratisaraneeya) and generally (panneya). Pratisaraneeya kshara is prepared with herbo-mineral medicines having an average pH of 13, possessing penetrating, corrosive, scraping, and healing properties, and are evidently indicated for external application in charmakīla. For the present case, kshara karma was preferred for application as the lesion was bigger in size and the results were appreciable clinically.

  3. Endocrine tumours in neurofibromatosis type 1, tuberous sclerosis and related syndromes.

    PubMed

    Lodish, Maya B; Stratakis, Constantine A

    2010-06-01

    Neurofibromatosis type 1 (NF-1) and tuberous sclerosis complex (TSC) are two familial syndromes known as phakomatoses that may be associated with endocrine tumours. These hereditary cutaneous conditions affect the central nervous system and are characterised by the development of hamartomas. Over the past 20 years, there have been major advances in our understanding of the molecular basis of these diseases. Both NF-1 and TSC are disorders of unregulated progression through the cell cycle, in which causative genes behave as tumour suppressor genes. The pathogenesis of these familial syndromes is linked by the shared regulation of a common pathway, the protein kinase mammalian target of rapamycin (mTOR). Additional related disorders that also converge on the mTOR pathway include Peutz-Jeghers syndrome and Cowden syndrome. All of these inherited cancer syndromes are associated with characteristic skin findings that offer a clue to their recognition and treatment. The discovery of mTOR inhibitors has led to a possible new therapeutic modality for patients with endocrine tumours as part of these familial syndromes. Published by Elsevier Ltd.

  4. The effect of sebocytes cultured from nevus sebaceus on hair growth.

    PubMed

    Lee, Weon Ju; Cha, Hyun Wuk; Lim, Hyun Jung; Lee, Seok-Jong; Kim, Do Won

    2012-10-01

    Sebaceous glands are known to affect hair growth. Nevus sebaceus, a sebaceous gland hamartomas, presents as hairless patches. In this study, cultures of nevus sebaceus sebocytes (NSS) and normal scalp hair follicle sebocytes (NS) were used in performance of microarray, RT-PCR, western blot assay and immunofluorescence staining. NSS- and NS-conditioned media were also added to the culture of outer root sheath cells (ORSCs), dermal papilla cells (DPCs) or normal scalp hair follicle sebocytes. Results of this study showed a decrease in the survival rate of ORSCs and DPCs and hair growth in the NSS-conditioned medium-treated group, compared with the control and NS-conditioned medium-treated groups. An increase in expression of fibroblast growth factor (FGF)-5, Dickkopf-1 and inflammatory cytokines and a decrease in expression of Wnt10b and Lef1 were observed. In conclusion, NSS showed an increase in expression of hair growth-suppressing bioactive factors, including FGF-5, and a decrease in expression of hair growth-stimulating factors. © 2012 John Wiley & Sons A/S.

  5. Pediatric living donor liver transplantation for congenital hepatic fibrosis using a mother's graft with von Meyenburg complex: A case report.

    PubMed

    Yamada, Naoya; Sanada, Yukihiro; Katano, Takumi; Tashiro, Masahisa; Hirata, Yuta; Okada, Noriki; Ihara, Yoshiyuki; Miki, Atsushi; Sasanuma, Hideki; Urahashi, Taizen; Sakuma, Yasunaru; Mizuta, Koichi

    2016-11-28

    This is the first report of living donor liver transplantation (LDLT) for congenital hepatic fibrosis (CHF) using a mother's graft with von Meyenburg complex. A 6-year-old girl with CHF, who suffered from recurrent gastrointestinal bleeding, was referred to our hospital for liver transplantation. Her 38-year-old mother was investigated as a living donor and multiple biliary hamartoma were seen on her computed tomography and magnetic resonance imaging scan. The mother's liver function tests were normal and she did not have any organ abnormality, including polycystic kidney disease. LDLT using the left lateral segment (LLS) graft from the donor was performed. The donor LLS graft weighed 250 g; the graft recipient weight ratio was 1.19%. The operation and post-operative course of the donor were uneventful and she was discharged on post-operative day (POD) 8. The graft liver function was good, and the recipient was discharged on POD 31. LDLT using a graft with von Meyenburg complex is safe and useful. Long-term follow-up is needed with respect to graft liver function and screening malignant tumors.

  6. Discoveries and controversies in cutaneous mosaicism.

    PubMed

    Castori, Marco; Tadini, Gianluca

    2016-06-01

    Genetic mosaicism is thought to be a common phenomenon in inherited skin disorders. It is the leading molecular mechanism explaining cutaneous hamartomas and nevoid disorders, skin manifestations of most X-linked genodermatoses and specific forms of clinical variability and topographic distribution in autosomal skin disorders. The developmental (in utero) origin and timing dependence are two major attributes for the current definition of cutaneous mosaicism. Chromosomal mosaicism, lyonization in X-linked genodermatoses, and various types of mosaicism (i.e. type 1, type 2 and revertant mosaicism) in autosomal skin disorders are mechanisms well defined at the molecular level. All these concepts have been fully included in the current medical terminology in dermatology and genetics. Mitotic crossing-over, paradominant inheritance, monoallelic expression of autosomal traits and mosaicism in acquired skin disorders remain without a formal molecular proof and still represent sources of debate in the scientific community. This review summarizes current concepts, discoveries and controversies in the field of cutaneous mosaicism for practitioners and clinical researchers to enhance their understanding of such a underestimated clinical phenomenon and its biological basis.

  7. [Governance, sustainability, and equity in the health program for the municipality of São José dos Pinhais, Brazil].

    PubMed

    Bueno, Roberto Eduardo; Moysés, Simone Tetu; Bueno, Paula Alexandra Reis; Moysés, Samuel Jorge

    2013-12-01

    To analyze the Final Report of the VIII Health Conference and the São José dos Pinhais City Health Program for 2010-2013 and investigate whether these documents addressed the themes of sustainability, governance, and equity and the interfaces between these themes--government policies, power balance, and inclusive processes/impacting results--that make up the Concept Model for Human Development and Health Promotion developed by the authors. This case study analyzed 331 proposals approved for incorporation in the City Health Program. The six thematical categories of the Concept Model were analyzed using ATLAS Ti 5.0 software. The proposals were classified according to the number of themes and interfaces of the Concept Model: full health proposals contained all six categories; partial proposals contained three categories; and incipient proposals contained one category. Of 331 proposals approved, 162 (49%) contemplated the six thematical categories and were classified as full health promotion proposals. Ninety-five (29%) contemplated three categories (partial health promotion). Of these, 38 (12%) addressed Governance, Sustainability, and Government Policies, 33 (10%) addressed Governance, Power Balance, and Equity and 24 (7%) addressed Equity, Inclusive Processes/Impact Results, and Sustainability. Finally, 74 (22%) proposals contemplated only one category and were classified as incipient: 36 (11%) addressed Governance, 27 (8%) addressed sustainability, and 11 (3%) addressed equity. Based on the fact that 49% of the proposals approved were classified as full health promotion, it is considered that the effectiveness of social control and popular participation in the construction of health policies at the local level contritute to the promotion of health in the city.

  8. Nitrogen and Phosphorous Flow in Atlantic Forest Covered Watersheds on the Oceanic and Continental Slopes at Serra dos Órgãos mountain, Southeast of Brazil

    NASA Astrophysics Data System (ADS)

    Vidal, M. M.; De Souza, P.; De Mello, W. Z.; Damaceno, I.; Bourseau, L.; Rodrigues, R. D. A.; Mattos, B. B.

    2017-12-01

    Concentration of nutrients above natural levels are found even at remote or protected environments due to atmospheric transportation from biomass burning emissions, urban and industrial areas. This study evaluate N and P atmospheric deposition at the oceanic and continental slopes of Serra dos Órgãos mountain, which are influenced by the pollutants emission from the Metropolitan Region of Rio de Janeiro. Flux of dissolved forms of N and P were measured in three watersheds in headwaters of Piabanha basin, southeastern Brazil, to understand the dynamics of the biogeochemical processes of these elements, related to anthropic influences of atmospheric inputs and export via stream flow. Samples of bulk precipitation (weekly; n=47) and stream water (monthly; n=13) were collected along one year (Sept 2014 - Sept 2015). During that period the annual rainfall in the oceanic slope (2163 mm) was the double of the continental one. It is important to stress that the rainfall in the oceanic slope was 13 % and 28% in 2014/15, respectively, lower than the long term average. Atmospheric deposition of total dissolved nitrogen (TDN) on the oceanic and continental slopes were, respectively, 15 and 8.6 kg N ha-1 year-1. The TDN outputs by stream water were 5-7 times lower in oceanic slope and 28 times lower on the continental one. The relative contribution of dissolved organic nitrogen (DON; 65%-70%) was higher than the one of dissolved inorganic nitrogen (DIN; 30-35%) to TDN deposition. Atmospheric deposition of total dissolved phosphorus (TDP) in oceanic and continental slopes were 1.4 and 0.95 kg P ha-1 year-1. Dissolved Organic Phosphorus (DOP; 89-96%) was higher than the inorganic one (PO43-; 5-11%). TDP outputs were 2-4 times lower, regarding to atmospheric contribution. The contribution of DOP (73-77 %) was higher than DIP (23-27 %). Results show variations in quantities and forms of N and P species due to natural and anthropogenic processes which contribute to the cycling of

  9. Earth and Heavens: Two Separate Universes? (Spanish Title: Tierra y Cielos: ¿Dos Universos Separados?) Terra e Céus: Dois Universos Separados?

    NASA Astrophysics Data System (ADS)

    Carrascosa, Jaime; Gil-Pérez, Daniel; Solbes, Jordi; Vilches, Amparo

    2005-12-01

    complexidade, considerando, entre outros aspectos: as situações problemáticas relevantes que levaram à construção dos conhecimentos, as dificuldades de todo tipo que se teve que enfrentar nessa construção e, muito em particular,a necessidade de se superar as "evidências do senso comum" que estabeleciam uma suposta barreira intransponível entre a Terra e os Céus e que foram um obstáculo durante séculos para o desenvolvimento científico. Neste trabalho apresentamos um programa de atividades para o estudo da gravitação nos últimos anos do ensino médio, orientado a favorecer a participação dos estudantes, em alguma medida, na reconstrução deste processo que constituiu a denominada primeira grande revolução científica e a nos aproximarmos do estudo de uma das ciências mais antigas, a Astronomia, que hoje continua despertando grande interesse.

  10. U-Pb zircon geochronologycal investigation on the Morro dos Seis Lagos Carbonatite Complex and associated Nb deposit (Amazonas, Brazil)

    NASA Astrophysics Data System (ADS)

    Rossoni, Marco B.; Bastos Neto, Artur C.; Souza, Valmir S.; Marques, Juliana C.; Dantas, Elton; Botelho, Nilson F.; Giovannini, Arthur L.; Pereira, Vitor P.

    2017-12-01

    We present results of U-Pb dating (by MC-ICP-MS) of zircons from samples that cover all of the known lithotypes in the Seis Lagos Carbonatite Complex and associated lateritic mineralization (the Morro dos Seis Lagos Nb deposit). The host rock (gneiss) yielded an age of 1828 ± 09 Ma interpreted as the crystallization time of this unit. The altered feldspar vein in the same gneiss yielded an age of 1839 ± 29 Ma. Carbonatite samples provided 3 groups of ages. The first group comprises inherited zircons with ages compatible with the gneissic host rock: 1819 ± 10 Ma (superior intercept), 1826 ± 5 Ma (concordant age), and 1812 ± 27 Ma (superior intercept), all from the Orosirian. The second and the third group of ages are from the same carbonatite sample: the superior intercept age of 1525 ± 21 Ma (MSWD = 0.77) and the superior intercept age of 1328 ± 58 Ma (MSWD = 1.4). The mineralogical study indicates that the ∼1.3 Ga zircons have affinity with carbonatite. It is, however, a tendence rather than a well-defined result. The data allow state that the age of 1328 ± 58 Ma represents the maximum age of the carbonatite. Without the same certainty, we consider that the data suggest that this age may be the carbonatite age, whose emplacement would have been related to the evolution of the K'Mudku belt. The best age obtained in laterite samples (a superior intercept age of 1828 ± 12 Ma) is considered the age of the main source for the inherited zircons related to the gneissic host rock.

  11. Dacrystic seizures: demographic, semiologic, and etiologic insights from a multicenter study in long-term video-EEG monitoring units.

    PubMed

    Blumberg, Julie; Fernández, Iván Sánchez; Vendrame, Martina; Oehl, Bernhard; Tatum, William O; Schuele, Stephan; Alexopoulos, Andreas V; Poduri, Annapurna; Kellinghaus, Christoph; Schulze-Bonhage, Andreas; Loddenkemper, Tobias

    2012-10-01

    To provide an estimate of the frequency of dacrystic seizures in video-electroencephalography (EEG) long-term monitoring units of tertiary referral epilepsy centers and to describe the clinical presentation of dacrystic seizures in relationship to the underlying etiology. We screened clinical records and video-EEG reports for the diagnosis of dacrystic seizures of all patients admitted for video-EEG long-term monitoring at five epilepsy referral centers in the United States and Germany. Patients with a potential diagnosis of dacrystic seizures were identified, and their clinical charts and video-EEG recordings were reviewed. We included only patients with: (1) stereotyped lacrimation, sobbing, grimacing, yelling, or sad facial expression; (2) long-term video-EEG recordings (at least 12 h); and (3) at least one brain magnetic resonance imaging (MRI) study. Nine patients (four female) with dacrystic seizures were identified. Dacrystic seizures were identified in 0.06-0.53% of the patients admitted for long-term video-EEG monitoring depending on the specific center. Considering our study population as a whole, the frequency was 0.13%. The presence of dacrystic seizures without other accompanying clinical features was found in only one patient. Gelastic seizures accompanied dacrystic seizures in five cases, and a hypothalamic hamartoma was found in all of these five patients. The underlying etiology in the four patients with dacrystic seizures without gelastic seizures was left mesial temporal sclerosis (three patients) and a frontal glioblastoma (one patient). All patients had a difficult-to-control epilepsy as demonstrated by the following: (1) at least three different antiepileptic drugs were tried in each patient, (2) epilepsy was well controlled with antiepileptic drugs in only two patients, (3) six patients were considered for epilepsy surgery and three of them underwent a surgical/radiosurgical or radioablative procedure. Regarding outcome, antiepileptic drugs

  12. Long seismic activity in the Porto dos Gaúchos Seismic Zone(PGSZ) - Amazon Craton Brazil

    NASA Astrophysics Data System (ADS)

    Barros, L. V.; Bowen, B. M. D.; Schmidt, K.

    2017-12-01

    The largest earthquake ever observed in the stable continental interior of the South American plate occurred in Serra do Tombador (ST), Mato Grosso state - Brazil, on January 31, 1955 with magnitude 6.2 m b . Since then no other earthquake has been located near the 1955 epicenter. However, in Porto dos Gaúchos (PG), 100 km northeast of ST, a recurrent seismicity has been observed since 1959. Both ST and PG are located in the Phanerozoic Parecis basin whose sediments overlies the crystalline basement of Amazon craton. Two magnitude 5 earthquakes occurred in PG, in 1998 and 2005 with intensities up to VI and V, respectively. These two main shocks were followed by aftershock sequences, studied by local seismic networks, last up today, almost 30 years later, period in which it was detected more than seven thousand of seismic events. Both sequences occurred in the same WSW-ENE oriented fault zone with right-lateral strike-slip mechanisms. The epicentral zone is near the northern border of Parecis basin, where there are buried grabens, generally trending WNW-ESE, such as the deep Mesoproterozoic Caiabis graben which lies partly beneath the Parecis basin. The seismogenic fault is located in a basement high, which is probably related with the same seismogenic feature responsible for the earthquakes in PGSZ. The 1955 earthquake, despite the uncertainty in its epicenter, does not seem to be directly related to any buried graben either. The seismicity in the PGSZ, therefore, is not directly related to rifted crust.Not considering the possibility of miss location in the ST earthquake, its isolated occurrence - from the perspective of new studies on intraplate seismicity - lead us to think that the PGSZ was activated by stresses released by the earthquake of 1955 and that the seismogenic fault of ST would have closed a cycle of activity. This would explain its seismic quiescence. However, other studies are necessary to prove this hypothesis, such as the measurement of the

  13. Queda dos homicídios em São Paulo, Brasil: uma análise descritiva

    PubMed Central

    Peres, Maria Fernanda Tourinho; Vicentin, Diego; Nery, Marcelo Batista; de Lima, Renato Sérgio; de Souza, Edinilsa Ramos; Cerda, Magdalena; Cardia, Nancy; Adorno, e Sérgio

    2012-01-01

    Objetivo Descrever a evolução da mortalidade por homicídios no Município de São Paulo segundo tipo de arma, sexo, raça ou cor, idade e áreas de exclusão/inclusão social entre 1996 e 2008. Métodos Estudo ecológico de série temporal. Os dados sobre óbitos ocorridos no Município foram coletados da base de dados do Programa de Aprimoramento das Informações sobre Mortalidade, seguindo a Classificação Internacional de Doenças, Décima Revisão (CID-10). Foram calculadas as taxas de mortalidade por homicídio (TMH) para a população total, por sexo, raça ou cor, faixa etária, tipo de arma e área de exclusão/inclusão social. As TMH foram padronizadas por idade pelo método direto. Foram calculados os percentuais de variação no período estudado. Para as áreas de exclusão/inclusão social foram calculados os riscos relativos de morte por homicídio. Resultados As TMH apresentaram queda de 73,7% entre 2001 e 2008. Foi observada redução da TMH em todos os grupos analisados, mais pronunciada em homens (−74,5%), jovens de 15 a 24 anos (−78,0%) e moradores de áreas de exclusão social extrema (−79,3%). A redução ocorreu, sobretudo, nos homicídios cometidos com armas de fogo (−74,1%). O risco relativo de morte por homicídio nas áreas de exclusão extrema (tendo como referência áreas com algum grau de exclusão social) foi de 2,77 em 1996, 3,9 em 2001 e 2,13 em 2008. Nas áreas de alta exclusão social, o risco relativo foi de 2,07 em 1996 e 1,96 em 2008. Conclusões Para compreender a redução dos homicídios no Município, é importante considerar macrodeterminantes que atingem todo o Município e todos os subgrupos populacionais e microdeterminantes que atuam localmente, influenciando de forma diferenciada os homicídios com armas de fogo e os homicídios na população jovem, no sexo masculino e em residentes em áreas de alta exclusão social. PMID:21390415

  14. Genesis and morphological evolution of coastal talus-platforms (fajãs) with lagoons: The case study of the newly-formed Fajã dos Milagres (Corvo Island, Azores)

    NASA Astrophysics Data System (ADS)

    Melo, Carlos S.; Ramalho, Ricardo S.; Quartau, Rui; Hipólito, Ana; Gil, Artur; Borges, Paulo A.; Cardigos, Frederico; Ávila, Sérgio P.; Madeira, José; Gaspar, João L.

    2018-06-01

    Supratidal talus-platforms are low-relief subaerial accumulations of debris produced by mass wasting along high coastal cliffs, being particularly abundant at reefless volcanic islands subjected to high wave energy. Known as "fajãs" across the Portuguese-speaking Atlantic archipelagos, these coastal features, on rare occasions, may exhibit lagoons, constituting sites of high geological, biological, landscape, and social value. Whilst the origin of fajãs is firmly established as being the product of coastal landslides, little is known about the processes that shape fajãs with lagoons. In particular, doubts still remain concerning whether fajãs featuring lagoons are a fortuitous product of mass wasting, or result from marine reworking (by waves and currents) after emplacement. On October 30, 2012, a coastal landslide ( 0.001 km3) occurred on Corvo Island, Azores Archipelago, forming a nearshore gravel islet that later migrated to the island's coast, resulting in a fajã with an ephemeral lagoon (Fajã dos Milagres). This event provided a unique opportunity to study the generation and development of fajãs with lagoons, and therefore a 3-year survey was carried out to record its evolution. This GIS-based study used aerial oblique photography and satellite optical imagery, complemented with a land survey for a more precise topographic reconstruction. Analysis of data concerning bathymetry, precipitation, and wave regime was also employed to investigate the driving forces behind the morphodynamic evolution of the deposit. "Fajã dos Milagres" evolved very rapidly, through an evolutionary pattern with five main stages: 1) "islet stage"; 2) "gravel spit stage"; 3) "early lagoon stage"; 4) "mature lagoon stage"; and 5) "fajã (without lagoon) stage". Our reconstructions show that, for fajãs with lagoons to be formed, several factors should converge: a) presence of high coastal cliffs, made up of composite volcanic sequences, capable of producing large landslides that

  15. Proceedings of the 2010 National Toxicology Program Satellite Symposium

    PubMed Central

    Adams, E. Terence; Auerbach, Scott; Blackshear, Pamela E.; Bradley, Alys; Gruebbel, Margarita M.; Little, Peter B.; Malarkey, David; Maronpot, Robert; McKay, Jennifer S.; Miller, Rodney A.; Moore, Rebecca R.; Morrison, James P.; Nyska, Abraham; Ramot, Yuval; Rao, Deepa; Suttie, Andrew; Wells, Monique Y.; Willson, Gabrielle A.; Elmore, Susan A.

    2011-01-01

    The 2010 annual National Toxicology Program (NTP) Satellite Symposium, entitled “Pathology Potpourri,” was held in Chicago, Illinois, in advance of the scientific symposium sponsored jointly by the Society of Toxicologic Pathology (STP) and the International Federation of Societies of Toxicologic Pathologists (IFSTP). The goal of the annual NTP Symposium is to present current diagnostic pathology or nomenclature issues to the toxicologic pathology community. This article presents summaries of the speakers' presentations, including diagnostic or nomenclature issues that were presented, along with select images that were used for voting or discussion. Some topics covered during the symposium included a comparison of rat and mouse hepatocholangiocarcinoma, a comparison of cholangiofibrosis and cholangiocarcinoma in rats, a mixed pancreatic neoplasm with acinar and islet cell components, an unusual preputial gland tumor, renal hyaline glomerulopathy in rats and mice, eosinophilic substance in the nasal septum of mice, INHAND nomenclature for proliferative and nonproliferative lesions of the CNS/PNS, retinal gliosis in a rat, fibroadnexal hamartoma in rats, intramural plaque in a mouse, a treatment-related chloracne-like lesion in mice, and an overview of mouse ovarian tumors. PMID:21177527

  16. [Autism, epilepsy and tuberous sclerosis complex: a functional model linked to mTOR pathway].

    PubMed

    García-Peñas, Juan José; Carreras-Sááez, Inmaculada

    2013-02-22

    Tuberous sclerosis complex (TSC) is an autosomal dominant disorder that results from mutations in the TSC1 or TSC2 genes and is associated with hamartoma formation in multiple organ systems. Brain disorders are the origin of more frequent and severe problems and include infantile spasms, intractable epilepsy, brain tumors, cognitive disabilities, and autism. TSC1 or TSC2 encoded proteins modulate cell function via the mTOR signaling cascade and serve as keystones in regulating cell growth and proliferation. AIM. To review the etiopathogenic mechanisms and the natural course of the association of autism and epilepsy in TSC. Both the clinical and the neuroimaging findings of TSC, including early onset epilepsy and the localization of cortical tubers in the temporal lobes, and the molecular understanding of the mTOR signaling pathway, not only involved in cell growth, but also in synaptogenesis, synaptic plasticity and neuronal functioning, have suggested a multimodal origin of autism in these patients. A greater understanding of the pathogenetic mechanisms underlying autism in TSC could help in devising targeted and potentially more effective treatment strategies. Antagonism of the mTOR pathway with rapamycin and everolimus may provide new therapeutic options for these TSC patients.

  17. Total colonic aganglionosis and imperforate anus in a severely affected infant with Pallister-Hall syndrome.

    PubMed

    Li, Mindy H; Eberhard, Moriah; Mudd, Pamela; Javia, Luv; Zimmerman, Robert; Khalek, Nahla; Zackai, Elaine H

    2015-03-01

    Pallister-Hall syndrome is a complex malformation syndrome characterized by a wide range of anomalies including hypothalamic hamartoma, polydactyly, bifid epiglottis, and genitourinary abnormalities. It is usually caused by truncating frameshift/nonsense and splicing mutations in the middle third of GLI3. The clinical course ranges from mild to lethal in the neonatal period. We present the first patient with Pallister-Hall syndrome reported with total colonic aganglionosis, a rare form of Hirschsprung disease with poor long-term outcome. The patient also had an imperforate anus, which is the third individual with Pallister-Hall syndrome reported with both Hirschsprung disease and an imperforate anus. Molecular testing via amniocentesis showed an apparently de novo novel nonsense mutation c.2641 C>T (p.Gln881*). His overall medical course was difficult and was complicated by respiratory failure and pan-hypopituitarism. Invasive care was ultimately withdrawn, and the patient expired at three months of age. This patient's phenotype was complex with unusual gastrointestinal features ultimately leading to a unfavorable prognosis and outcome, highlighting the range of clinical severity in patients with Pallister-Hall syndrome. © 2015 Wiley Periodicals, Inc.

  18. MRI, volumetry, 1H spectroscopy, and cerebropetal blood flowmetry in childhood idiopathic anatomic megalencephaly.

    PubMed

    Koudijs, Suzanne M; van der Grond, Jeroen; Hoogendoorn, Mechteld L C; Hulshoff Pol, Hilleke E; Schnack, Hugo G; Witkamp, Theo D; Gooskens, Rob H J M; van Nieuwenhuizen, Onno; Braun, Kees P J

    2006-08-01

    To evaluate cerebral abnormalities in childhood idiopathic anatomic megalencephaly (MC) by means of different magnetic resonance (MR) modalities. MRI, volumetry, spectroscopy, and cerebropetal blood flowmetry were performed in six children with idiopathic anatomic MC, and seven volunteers. MRI revealed an increased ventricular system in five of six patients. A thalamic hamartoma was found in one patient and a Chiari I malformation was found in two. Volumetric analysis showed a disproportional increase of ventricular volume but normal subarachnoid cerebrospinal fluid (CSF) volume. Supratentorial volume was disproportionally increased compared to cerebellar volume. Intracranial volume correlated significantly with skull circumference. MR spectroscopy (MRS) N-acetyl aspartate/choline (NAA/Cho) peak ratios in WM were significantly higher in patients than in controls. Choline/creatine (Cho/Cr) peak ratios in WM were significantly lower in patients. Cortical gray matter (GM) MRS ratios were unaltered. Cerebropetal flow was increased in MC, possibly related to increased brain volume. This study reveals associated developmental anomalies for idiopathic anatomic MC. A relative ventriculomegaly was found, which should not be misinterpreted as true hydrocephalus. In contrast to metabolic MC, MRS showed no severe disturbances. Total intracranial volume is correlated to skull circumference and cerebropetal blood flow.

  19. mTOR and vascular remodeling in lung diseases: current challenges and therapeutic prospects.

    PubMed

    Goncharova, Elena A

    2013-05-01

    Mammalian target of rapamycin (mTOR) is a major regulator of cellular metabolism, proliferation, and survival that is implicated in various proliferative and metabolic diseases, including obesity, type 2 diabetes, hamartoma syndromes, and cancer. Emerging evidence suggests a potential critical role of mTOR signaling in pulmonary vascular remodeling. Remodeling of small pulmonary arteries due to increased proliferation, resistance to apoptosis, and altered metabolism of cells forming the pulmonary vascular wall is a key currently irreversible pathological feature of pulmonary hypertension, a progressive pulmonary vascular disorder with high morbidity and mortality. In addition to rare familial and idiopathic forms, pulmonary hypertension is also a life-threatening complication of several lung diseases associated with hypoxia. This review aims to summarize our current knowledge and recent advances in understanding the role of the mTOR pathway in pulmonary vascular remodeling, with a specific focus on the hypoxia component, a confirmed shared trigger of pulmonary hypertension in lung diseases. We also discuss the emerging role of mTOR as a promising therapeutic target and mTOR inhibitors as potential pharmacological approaches to treat pulmonary vascular remodeling in pulmonary hypertension.

  20. New insights into genotype–phenotype correlation for GLI3 mutations

    PubMed Central

    Démurger, Florence; Ichkou, Amale; Mougou-Zerelli, Soumaya; Le Merrer, Martine; Goudefroye, Géraldine; Delezoide, Anne-Lise; Quélin, Chloé; Manouvrier, Sylvie; Baujat, Geneviève; Fradin, Mélanie; Pasquier, Laurent; Megarbané, André; Faivre, Laurence; Baumann, Clarisse; Nampoothiri, Sheela; Roume, Joëlle; Isidor, Bertrand; Lacombe, Didier; Delrue, Marie-Ange; Mercier, Sandra; Philip, Nicole; Schaefer, Elise; Holder, Muriel; Krause, Amanda; Laffargue, Fanny; Sinico, Martine; Amram, Daniel; André, Gwenaelle; Liquier, Alain; Rossi, Massimiliano; Amiel, Jeanne; Giuliano, Fabienne; Boute, Odile; Dieux-Coeslier, Anne; Jacquemont, Marie-Line; Afenjar, Alexandra; Van Maldergem, Lionel; Lackmy-Port-Lis, Marylin; Vincent- Delorme, Catherine; Chauvet, Marie-Liesse; Cormier-Daire, Valérie; Devisme, Louise; Geneviève, David; Munnich, Arnold; Viot, Géraldine; Raoul, Odile; Romana, Serge; Gonzales, Marie; Encha-Razavi, Ferechte; Odent, Sylvie; Vekemans, Michel; Attie-Bitach, Tania

    2015-01-01

    The phenotypic spectrum of GLI3 mutations includes autosomal dominant Greig cephalopolysyndactyly syndrome (GCPS) and Pallister–Hall syndrome (PHS). PHS was first described as a lethal condition associating hypothalamic hamartoma, postaxial or central polydactyly, anal atresia and bifid epiglottis. Typical GCPS combines polysyndactyly of hands and feet and craniofacial features. Genotype–phenotype correlations have been found both for the location and the nature of GLI3 mutations, highlighting the bifunctional nature of GLI3 during development. Here we report on the molecular and clinical study of 76 cases from 55 families with either a GLI3 mutation (49 GCPS and 21 PHS), or a large deletion encompassing the GLI3 gene (6 GCPS cases). Most of mutations are novel and consistent with the previously reported genotype–phenotype correlation. Our results also show a correlation between the location of the mutation and abnormal corpus callosum observed in some patients with GCPS. Fetal PHS observations emphasize on the possible lethality of GLI3 mutations and extend the phenotypic spectrum of malformations such as agnathia and reductional limbs defects. GLI3 expression studied by in situ hybridization during human development confirms its early expression in target tissues. PMID:24736735

  1. New insights into genotype-phenotype correlation for GLI3 mutations.

    PubMed

    Démurger, Florence; Ichkou, Amale; Mougou-Zerelli, Soumaya; Le Merrer, Martine; Goudefroye, Géraldine; Delezoide, Anne-Lise; Quélin, Chloé; Manouvrier, Sylvie; Baujat, Geneviève; Fradin, Mélanie; Pasquier, Laurent; Megarbané, André; Faivre, Laurence; Baumann, Clarisse; Nampoothiri, Sheela; Roume, Joëlle; Isidor, Bertrand; Lacombe, Didier; Delrue, Marie-Ange; Mercier, Sandra; Philip, Nicole; Schaefer, Elise; Holder, Muriel; Krause, Amanda; Laffargue, Fanny; Sinico, Martine; Amram, Daniel; André, Gwenaelle; Liquier, Alain; Rossi, Massimiliano; Amiel, Jeanne; Giuliano, Fabienne; Boute, Odile; Dieux-Coeslier, Anne; Jacquemont, Marie-Line; Afenjar, Alexandra; Van Maldergem, Lionel; Lackmy-Port-Lis, Marylin; Vincent-Delorme, Catherine; Chauvet, Marie-Liesse; Cormier-Daire, Valérie; Devisme, Louise; Geneviève, David; Munnich, Arnold; Viot, Géraldine; Raoul, Odile; Romana, Serge; Gonzales, Marie; Encha-Razavi, Ferechte; Odent, Sylvie; Vekemans, Michel; Attie-Bitach, Tania

    2015-01-01

    The phenotypic spectrum of GLI3 mutations includes autosomal dominant Greig cephalopolysyndactyly syndrome (GCPS) and Pallister-Hall syndrome (PHS). PHS was first described as a lethal condition associating hypothalamic hamartoma, postaxial or central polydactyly, anal atresia and bifid epiglottis. Typical GCPS combines polysyndactyly of hands and feet and craniofacial features. Genotype-phenotype correlations have been found both for the location and the nature of GLI3 mutations, highlighting the bifunctional nature of GLI3 during development. Here we report on the molecular and clinical study of 76 cases from 55 families with either a GLI3 mutation (49 GCPS and 21 PHS), or a large deletion encompassing the GLI3 gene (6 GCPS cases). Most of mutations are novel and consistent with the previously reported genotype-phenotype correlation. Our results also show a correlation between the location of the mutation and abnormal corpus callosum observed in some patients with GCPS. Fetal PHS observations emphasize on the possible lethality of GLI3 mutations and extend the phenotypic spectrum of malformations such as agnathia and reductional limbs defects. GLI3 expression studied by in situ hybridization during human development confirms its early expression in target tissues.

  2. Castleman's disease of the spleen.

    PubMed

    Mantas, Dimitrios; Damaskos, Christos; Dailiani, Panagiota; Samarkos, Michael; Korkolopoulou, Penelope

    2017-06-01

    Castleman's disease (CD), also known as giant or angiofolicular lymphoid hyperplasia or lymphoid hamartoma, is a group of atypical lymphoproliferative disorders that share common lymph node histological features and may be localized either to a single lymph node (unicentric) or occur systemically (multicentric). Herein, we present a rare case of a of 75-year-old female patient who was referred to our department and after a thorough work-up, underwent splenectomy with synchronous resection of an accessory spleen, splenic artery lymph nodes, and splenic hilar lymph nodes due to splenic involvement in a multicentric CD. The pathology of the specimens led to the conclusion that it was a case of polycentric HHV-8-positive CD, affecting the spleen, the accessory spleen, and the lymph nodes. Incidence of this rare condition is believed to be approximately 0.001-0.05%. CD has been linked to the human immunodeficiency virus (HIV), human herpes virus 8 (HHV-8), and is associated with malignancies. The pathogenesis mechanism is considered to be a dysregulation and hypersecretion of cytokines, either idiopathic or secondary to a viral infection, with the latter considered the most frequent. Solid organ involvement is very rare as is splenic involvement.

  3. Hair Follicle Nevus With Features of Comedo Nevus: An Expanding Spectrum.

    PubMed

    Nagarajan, Priyadharsini; Bartholomew, Timothy S; Allen, Carl M; Peters, Sara B

    2016-06-01

    Hair follicle nevus (HFN) is a rare hamartomatous lesion of the folliculosebaceous unit, with or without admixed fibroadipose or muscular tissue. It typically has a congenital presentation in the preauricular area of infants and is frequently confused with an accessory tragus. Acquired tumors with similar histopathologic features have been described infrequently during adolescence and adult life. We report yet another unique presentation of this unusual lesion in a 4-year-old girl who had a long-standing tumor of the nasal columella that started growing rapidly after trauma. Histopathologic examination revealed increased numbers of hair follicles, some of which were associated with diminutive sebaceous glands, with no associated central cystic structure. In addition, the infundibula of the follicles were dilated and filled with keratinous debris. Although these hamartomas are common in the head and neck region, to our knowledge, this is the first report of a HFN at this anatomic location. In addition, this tumor has an overall architecture of a HFN but is accompanied by features of a comedo nevus. We also present a review of the literature and summarize the current diagnostic criteria for HFN.

  4. Histologic characteristics of gastric polyps in Korea: Emphasis on discrepancy between endoscopic forceps biopsy and endoscopic mucosal resection specimen

    PubMed Central

    Yoon, Won Jae; Lee, Dong Ho; Jung, Yong Jin; Jeong, Ji Bong; Kim, Ji Won; Kim, Byeong Gwan; Lee, Kook Lae; Lee, Kwang Hyuck; Park, Young Soo; Hwang, Jin-Hyeok; Kim, Jin-Wook; Kim, Nayoung; Lee, Jun Kyu; Jung, Hyun Chae; Yoon, Yong Bum; Song, In Sung

    2006-01-01

    AIM: To investigate histological characteristics of gastric polyps in the Korean population. METHODS: We reviewed endoscopic photographs and medical records of patients with gastric polyps who underwent endoscopic mucosal resection from April 1996 through February 2003. RESULTS: A total of 85 gastric polyps from 74 patients were reviewed. Male-to-female ratio was 1:1.96. Mean age was 59.9 ± 10.8 years. Multiple polyps were observed in 10.8%. Gastric polyps occurred most frequently in the antrum (58.8%). Pathological results on resected specimens were as follows: tubular adenoma 45.9%, hyperplastic polyp 31.8%, inflammatory polyp 9.4%, hamartoma 3.5%, fundic gland polyp 2.4%, tubulovillous adenoma 2.4%, adenocarcinoma 2.4%, dysplasia 1.1%, and mucosal pseudolipomatosis 1.1%. Discrepancy rate between endoscopic biopsy and pathology of resected specimens was 27.1%. There was no relationship between the size of the polyp and concordance rate. CONCLUSION: There is considerable discrepancy in histologic findings between endoscopic forceps biopsy and resected specimens. Approaches to review of the histology of an entire polyp should be performed, especially when an adenoma is suspected. PMID:16810753

  5. Analysis of the Unified Health System funding and expenditure in the municipalities of the "Rota dos Bandeirantes" health region, State of São Paulo, Brazil.

    PubMed

    Santos, João Alves Dos; Mendes, Áquilas Nogueira; Pereira, Antônio Carlos; Paranhos, Luiz Renato

    2017-04-01

    The national scenario of lack of resources in the Brazilian Unified Health System (SUS) has led to major differences in the municipalities funding models. Thus, this study aims to analyze SUS funding and expenditure in seven cities of the Rota dos Bandeirantes health region, State of São Paulo, SP, Brazil, from 2009 to 2012. Settled expenditure indicators were collected from the Public Health Budgets Information System (SIOPS) for analysis, showing descriptive data with absolute and relative frequency calculations. We identified that the per capita income available for the city of Barueri is almost tenfold that of the city of Carapicuíba, and that Barueri's health expenditure per capita is more than double that of the regional average and almost fivefold that of Carapicuíba. The Federal Government is responsible for 95.4% of all funding to municipalities. Most of the available income of the municipalities in the region include their own taxes and state transfers. All the municipalities showed a significant positive trend, both for available income and health expenditure. The regional average of own revenue spent on health is 27.3%. Carapicuíba achieved a level of 37.5%, which is much higher than the minimum of 15% required by the Federal Constitution.

  6. Um Breve Balanço dos Estudos em Astronomia e Educação no Brasil no Período de 2010 a 2013

    NASA Astrophysics Data System (ADS)

    Goncalves, Erica de Oliveira; Kern, C.

    2014-10-01

    No Brasil, as pesquisas em ensino de astronomia para a Educação Básica vem ganhando destaque. Posto como importante área do conhecimento para estudantes e professores, os estudos em astronomia conquistam espaços nos documentos oficiais da educação e nos currículos escolares. Diante desse cenário, fez-se, neste trabalho, um mapeamento no banco de dados da Biblioteca Digital Brasileira de Teses e Dissertações , com base nas palavras-chave "astronomia" e "educação" no período de 2010 a 2013. Para compor o que aqui denominamos de balanço da área de estudo, foram selecionados trabalhos e analisados os títulos, os resumos, as considerações finais e as referências, bem como identificamos as fontes epistemológicas correntes nas pesquisas de pós-graduação no período supracitado. Identificou-se, na maior parte dos trabalhos pesquisados, referenciais teóricos relacionados & agrave; área de física, ciências e astronomia que envolvem discussões sobre currículo e práticas pedagógicas vinculados ao ensino de astronomia no ensino fundamental e médio da Educação Bãsica e nos cursos de formação de professores.

  7. Simultaneous lidar observation of peculiar sporadic K and Na layers at São José dos Campos (23.1°S, 45.9°W), Brazil

    NASA Astrophysics Data System (ADS)

    Jiao, Jing; Yang, Guotao; Cheng, Xuewu; Liu, Zhengkuan; Wang, Jihong; Yan, Zhenzhong; Wang, Chi; Batista, Paulo; Pimenta, Alexandre; Andrioli, Vânia; Denardini, C. M.

    2018-04-01

    We present the first simultaneous observation of mesopause sodium (Na) and potassium (K) layer by a lidar which has Na and K channel simultaneously at the South Hemisphere site, São José dos Campos (23.1°S, 45.9°W). Measurements reported here were conducted on two nights with 3.5 and 8 h of observations in November 2016. On 20 November 2016, sporadic layers in both Na and K layer occurred above 100 km, and the higher layers corresponded well with sporadic E (Es) layer. And the density of Na at 100-105 km is higher than that at main layer around 90 km, but K density around 100 km is at least 3 times smaller compared with the K density around 90 km for the similar period. On 21 November 2016, both sporadic layers occurred in main layer height with obvious descending variations with time, which seems like tidal induced. Notably, the peak K/Na ratio slowly increased with time. And Na layer and K layer showed different processes along with time with K density reaching its maximum 1 h later than that of Na. Correlations of Na/K density, Es, and winds were also discussed.

  8. Prevalence of intestinal parasites versus knowledge, attitudes, and practices of inhabitants of low-income communities of Campos dos Goytacazes, Rio de Janeiro State, Brazil.

    PubMed

    de Moraes Neto, Antonio Henrique A; Pereira, Adriana P M F; Alencar, Maria de Fátima L; Souza, Paulo R B; Dias, Rodrigo C; Fonseca, Juliana G; Santos, Clóvis P; Almeida, João C A

    2010-07-01

    Intestinal parasites are the causative agents of common infections responsible for significant public health problems in developing countries and generally linked to lack of sanitation, safe water, and improper hygiene. More than two billion people throughout the world live with unrelenting illness due to intestinal parasitic infections (IPIs). The purposes of this study are to assess knowledge, attitudes, and practices on IPIs and investigate the relationship with prevalence of intestinal parasites among a low-income group of inhabitants from two communities of the Travessão District area, Campos dos Goytacazes, north of Rio de Janeiro State, Brazil. The two communities are known as "Parque Santuário," which is an urban slum with miserable living conditions, and "Arraial," where the socioeconomic and educational levels are better, neither having a sanitary infrastructure with an excreta collection system. Questionnaires revealed that both communities had local and specific codification to denominate the intestinal parasites and present correct knowledge on the theme but ignored some aspects of IPI transmission, with the Arraial population being better informed (p < 0.05). The overall prevalence of IPIs in Parque Santuário (49.7%) was greater than in Arraial (27.2%) (p < 0.001; prevalence ratio/95% confidence interval 1.83/1.50-2.23). This study reports the real IPI situation in the Travessão District and also reinforces the need to continue the investigation on the impact of combined prophylactic methods, educational measures, and socioeconomic and sanitary improvements by governmental authorities and the local popular organization.

  9. [Evaluation of four dark object atmospheric correction methods based on ZY-3 CCD data].

    PubMed

    Guo, Hong; Gu, Xing-fa; Xie, Yong; Yu, Tao; Gao, Hai-liang; Wei, Xiang-qin; Liu, Qi-yue

    2014-08-01

    The present paper performed the evaluation of four dark-object subtraction(DOS) atmospheric correction methods based on 2012 Inner Mongolia experimental data The authors analyzed the impacts of key parameters of four DOS methods when they were applied to ZY-3 CCD data The results showed that (1) All four DOS methods have significant atmospheric correction effect at band 1, 2 and 3. But as for band 4, the atmospheric correction effect of DOS4 is the best while DOS2 is the worst; both DOS1 and DOS3 has no obvious atmospheric correction effect. (2) The relative error (RE) of DOS1 atmospheric correction method is larger than 10% at four bands; The atmospheric correction effect of DOS2 works the best at band 1(AE (absolute error)=0.0019 and RE=4.32%) and the worst error appears at band 4(AE=0.0464 and RE=19.12%); The RE of DOS3 is about 10% for all bands. (3) The AE of atmospheric correction results for DOS4 method is less than 0. 02 and the RE is less than 10% for all bands. Therefore, the DOS4 method provides the best accuracy of atmospheric correction results for ZY-3 image.

  10. An innovative method of pediatric chest wall reconstruction using Surgisis and swinging rib technique.

    PubMed

    Oliveira, Carol; Zamakhshary, Mohammed; Alfadda, Tariq; Alhabshan, Fahad; Alshalaan, Hisham; Miller, Stephen; Kim, Peter C W

    2012-05-01

    Herein, we describe a new surgical approach for chest wall reconstruction using a native supporting rib and Surgisis. A retrospective review of 3 cases from 2 tertiary pediatric health care centers presenting with chest wall defects in the neonatal period was performed. Perioperative data were collected. Two chest wall deformities were diagnosed at birth (Poland syndrome and cleft sternum). One patient was diagnosed prenatally with a mediastinal mass. The first infant had absent ribs 2 through 9. He underwent chest wall reconstruction at 4 weeks of life because of difficulty weaning from ventilation related to paradoxical breathing. The hamartoma of the second asymptomatic patient was removed at 6 weeks. The third patient's V-shaped sternal defect encompassed through the upper two thirds of the sternum and was repaired at 6 months of age with intraoperative transesophageal echocardiogram monitoring. In all cases, Surgisis (collagen matrix) was used as an onlay patch. In 2 cases, a swinging rib acted supportive. Neither patient had intraoperative complications. Surgisis is useful in pediatric chest wall reconstruction, particularly in combination with swinging ribs. The capacity for adaptation to the child's growth of this approach is crucial. Short-term safety is shown, but long-term assessment is required. Copyright © 2012 Elsevier Inc. All rights reserved.

  11. Dual pathology proximal median nerve compression of the forearm.

    PubMed

    Murphy, Siun M; Browne, Katherine; Tuite, David J; O'Shaughnessy, Michael

    2013-12-01

    We report an unusual case of synchronous pathology in the forearm- the coexistence of a large lipoma of the median nerve together with an osteochondroma of the proximal ulna, giving rise to a dual proximal median nerve compression. Proximal median nerve compression neuropathies in the forearm are uncommon compared to the prevalence of distal compression neuropathies (eg Carpal Tunnel Syndrome). Both neural fibrolipomas (Refs. 1,2) and osteochondromas of the proximal ulna (Ref. 3) in isolation are rare but well documented. Unlike that of a distal compression, a proximal compression of the median nerve will often have a definite cause. Neural fibrolipoma, also called fibrolipomatous hamartoma are rare, slow-growing, benign tumours of peripheral nerves, most often occurring in the median nerve of younger patients. To our knowledge, this is the first report of such dual pathology in the same forearm, giving rise to a severe proximal compression of the median nerve. In this case, the nerve was being pushed anteriorly by the osteochondroma, and was being compressed from within by the intraneural lipoma. This unusual case highlights the advantage of preoperative imaging as part of the workup of proximal median nerve compression. Copyright © 2013 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.

  12. Hepatic perivascular epithelioid cell tumor: Case report and brief literature review.

    PubMed

    Tang, Da; Wang, Jianmin; Tian, Yuepeng; Li, Qiuguo; Yan, Haixiong; Wang, Biao; Xiong, Li; Li, Qinglong

    2016-12-01

    Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal neoplasm which expresses both myogenic and melanocytic markers. PEComas are found in a variety locations in the body, but up to now only approximately 30 cases about hepatic perivascular epithelioid cell tumor are reported in English language worldwide. A 32-year-old woman was admitted in our hospital with intermittent right upper quadrant pain for 1 month and recent (1 day) progressive deterioration. Based on the results of the laboratory examinations and the findings of the computed tomography, the diagnosis of hepatic hamartoma or the hepatocecullar carcinoma with hemorrhage was made. The patient underwent a segmentectomy of the liver, and the finally diagnosis of hepatic PEComa was made with immunohistochemical confirmation with HMB-45 and SMA. There is no clinical or radiographic evidence of recurrence 9 months after surgery. This kind of tumor is extremely rare and the natural history of PEComa is uncertain, as the treatment protocol for hepatic PEComa has not reached a consensus. But the main treatment of the disease may be surgical resection. Only after long term follow-up can we know whether the tumor is benign or malignant. It appears that longer clinical follow-up is necessary in all patients with hepatic PEComas.

  13. Role of the Tsc1-Tsc2 complex in signaling and transport across the cell membrane in the fission yeast Schizosaccharomyces pombe.

    PubMed Central

    Matsumoto, Sanae; Bandyopadhyay, Amitabha; Kwiatkowski, David J; Maitra, Umadas; Matsumoto, Tomohiro

    2002-01-01

    Heterozygous inactivation of either human TSC1 or TSC2 causes tuberous sclerosis (TSC), in which development of benign tumors, hamartomas, occurs via a two-hit mechanism. In this study, fission yeast genes homologous to TSC1 and TSC2 were identified, and their protein products were shown to physically interact like the human gene products. Strains lacking tsc1(+) or tsc2(+) were defective in uptake of nutrients from the environment. An amino acid permease, which is normally positioned on the plasma membrane, aggregated in the cytoplasm or was confined in vacuole-like structures in Deltatsc1 and Deltatsc2 strains. Deletion of tsc1(+) or tsc2(+) also caused a defect in conjugation. When a limited number of the cells were mixed, they conjugated poorly. The conjugation efficiency was improved by increased cell density. Deltatsc1 cells were not responsive to a mating pheromone, P-factor, suggesting that Tsc1 has an important role in the signal cascade for conjugation. These results indicate that the fission yeast Tsc1-Tsc2 complex plays a role in the regulation of protein trafficking and suggest a similar function for the human proteins. We also show that fission yeast Int6 is involved in a similar process, but functions in an independent genetic pathway. PMID:12136010

  14. DOS/Russian - US program overview

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

    Pifer, M.L.

    1996-04-01

    This paper describes the organization and describes the objectives for the science and technology agreements between the U.S.A. and Russia. The Gore-Chernomyrdin Commission objectives and the Civilian Research and the Development Foundation for the Independent States of the former Soviet Union (CRDF) are discussed.

  15. The Role of Climatic Conditions in Controlling Observed Variability of Timing and Peak Discharge of Glacial Lake Outburst Floods: Lago Cachet Dos, Chile

    NASA Astrophysics Data System (ADS)

    Jacquet, J.; McCoy, S. W.; McGrath, D.; Nimick, D.; Friesen, B.; Fahey, M. J.; Leidich, J.; Okuinghttons, J.

    2016-12-01

    The sudden release of water from an ice-dammed lake poses substantial hazard to the downstream environment, but predicting the timing and magnitude of such an event is difficult. We use a series of high-resolution discharge measurements from a glacier-dammed lake, Lago Cachet Dos (LC2), during outburst events to evaluate the environmental conditions that influence the timing of initiation and peak discharge of observed glacial lake outburst floods (GLOFs). Since April 2008, 20 GLOFs have initiated out of LC2, located on the eastern edge of the Northern Patagonia Icefield, Chile and flooded areas along the Rio Colonia- Rio Baker system. GLOF frequency has averaged 2-3 events annually and peak discharges exiting LC2 have ranged widely from 2,000 to >15,000 m3 s-1. Although some LC2 GLOFs are consistent with global compilations relating peak discharge to lake volume, large deviations from the global trend and large intra-event variability are striking and call into question the predictive ability of simple empirical scaling equations. To evaluate the environmental conditions that lead to variability in observed peak discharge, we use a variation of the theoretical model of Nye (1976), which describes the process of englacial conduit evolution as a competition between thermally induced conduit growth and viscous flow of ice causing conduit collapse. We show that, consistent with theory, initial lake volume, lake temperature, and the rate of meltwater input into the glacially dammed lake all influence the peak discharge of measured GLOFs. Consequently, evolving climatic conditions of a region can greatly influence the potential hazard of GLOFs. Our results suggest that more accurate predictions of GLOF timing and magnitude from ice dammed lakes can be made by incorporating additional measurements of environmental conditions.

  16. Use of Optical Storage Devices as Shared Resources in Local Area Networks

    DTIC Science & Technology

    1989-09-01

    13 3. SERVICE CALLS FOR MS-DOS CD-ROM EXTENSIONS . 14 4. MS-DOS PRIMITIVE GROUPS ....................... 15 5. RAM USAGE FOR VARIOUS LAN...17 2. Service Call Translation to DOS Primitives ............. 19 3. MS-DOS Device Drivers ............................. 21 4. MS-DOS/ROM...directed to I/O devices will be referred to as primitive instruction groups). These primitive instruction groups include keyboard, video, disk, serial

  17. Impact of natural climate change and historical land use on vegetation cover and geomorphological process dynamics in the Serra dos Órgãos mountain range in Rio de Janeiro State, Brazil

    NASA Astrophysics Data System (ADS)

    Nehren, U.; Sattler, D.; Heinrich, J.

    2010-03-01

    The Serra dos Órgãos mountain range in the hinterland of Rio de Janeiro contains extensive remnants of the Atlantic Forest (Mata Atlântica) biome, which once covered about 1.5 million km² from Northeast to South Brazil and further inland to Paraguay and Argentina. As a result of historical deforestation and recent land use intensification processes today only 5 to 8% of the original Atlantic Forest remains. Despite the dramatic habitat loss and a high degree of forest fragmentation, the remnants are among the Earth’s most diverse habitats in terms of species richness. Furthermore, they are characterized by a high level of endemism. Therefore, the biome is considered a "hotspot of biodiversity". In the last years many efforts have been taken to investigate the Mata Atlântica biome in different spatial and time scales and from different scientific perspectives. We are working in the Atlantic Forest of Rio de Janeiro since 2004 and focus in our research particularly on Quaternary landscape evolution and landscape history. By means of landscape and soil archives we reconstruct changes in the landscape system, which are mainly the result of Quaternary climate variability, young tectonic uplift and human impact. The findings throw light on paleoecological conditions in the Late Quaternary and the impact of pre-colonial and colonial land use practices on these landscapes. In this context, a main focus is set on climate and human-driven changes of the vegetation cover and its consequences for the geomorphological process dynamics, in particular erosion and sedimentation processes. Research methods include geomorphological field studies, interpretation of satellite images, physical and chemical sediment and soil analyses as well as relative and absolute dating (Feo/Fed ratio and 14C dating). For the Late Quaternary landscape evolution, the findings are compared with results from paleoclimatic and paloecological investigations in Southeast and South Brazil using other

  18. A Diverging DOS Strategy Using an Allene-Containing Tryptophan Scaffold and a Library Design that Maximizes Biologically Relevant Chemical Space While Minimizing the Number of Compounds

    PubMed Central

    Painter, Thomas O.; Wang, Lirong; Majumder, Supriyo; Xie, Xiang-Qun; Brummond, Kay M.

    2011-01-01

    A diverging diversity-oriented synthesis (DOS) strategy using an allene-containing tryptophan as a key starting material was investigated. An allene-yne substituted derivative of tryptophan 12 gave indolylmethylazabicyclooctadiene 17 when subjected to a microwave-assisted allenic [2 + 2] cycloaddition reaction. This same tryptophan-derived precursor afforded an indolylmethyldihydrocyclopentapyridinone 14 when subjected to a rhodium(I)-catalyzed cyclocarbonylation reaction and an indolylmethylpyrrolidinocyclopentenones 16 when reacted with molybdenum hexacarbonyl. Construction of allenic tetrahydro-β-carboline scaffolds via a Pictet-Spengler reaction and subsequent silver(I)-catalyzed cycloisomerization afforded tetrahydroindolizinoindoles (21). Attachment of allene and alkyne groups to the tetrahydro-β-carboline followed by a microwave-assisted allenic [2 + 2] cycloaddition reaction provided tetrahydrocyclobutaindoloquinolizinones 24 and the tetrahydrocyclopentenone indolizinoindolone 26 when reacted with molybdenum hexacarbonyl. These six scaffolds were used as a template for the construction of a virtual library of 11,748 compounds employing 44 indoles, 12 aldehydes, and 51 alkynes. Diversity analyses using a combination of cell-based chemistry space computations using BCUT (Burden (B) CAS (C) Pearlman at the University of Texas (UT)) metrics and Tanimoto coefficient (Tc) similarity calculations using two-dimensional (2D) fingerprints showed that the compounds in the virtual library occupied new chemical space when compared to the 327,000 compounds in the molecular libraries small molecule repository (MLSMR). A subset of fifty-three compounds was identified from the virtual library using the DVS package of Sybyl 8.0; this subset represents the most diverse compounds within the chemical space defined by these compounds and will be synthesized and screened for biological activity. PMID:21332123

  19. Ultrafast wavelength multiplexed broad bandwidth digital diffuse optical spectroscopy for in vivo extraction of tissue optical properties

    NASA Astrophysics Data System (ADS)

    Torjesen, Alyssa; Istfan, Raeef; Roblyer, Darren

    2017-03-01

    Frequency-domain diffuse optical spectroscopy (FD-DOS) utilizes intensity-modulated light to characterize optical scattering and absorption in thick tissue. Previous FD-DOS systems have been limited by large device footprints, complex electronics, high costs, and limited acquisition speeds, all of which complicate access to patients in the clinical setting. We have developed a new digital DOS (dDOS) system, which is relatively compact and inexpensive, allowing for simplified clinical use, while providing unprecedented measurement speeds. The dDOS system utilizes hardware-integrated custom board-level direct digital synthesizers and an analog-to-digital converter to generate frequency sweeps and directly measure signals utilizing undersampling at six wavelengths modulated at discrete frequencies from 50 to 400 MHz. Wavelength multiplexing is utilized to achieve broadband frequency sweep measurements acquired at over 97 Hz. When compared to a gold-standard DOS system, the accuracy of optical properties recovered with the dDOS system was within 5.3% and 5.5% for absorption and reduced scattering coefficient extractions, respectively. When tested in vivo, the dDOS system was able to detect physiological changes throughout the cardiac cycle. The new FD-dDOS system is fast, inexpensive, and compact without compromising measurement quality.

  20. Tritium ((3)H) as a tracer for monitoring the dispersion of conservative radionuclides discharged by the Angra dos Reis nuclear power plants in the Piraquara de Fora Bay, Brazil.

    PubMed

    de Carvalho Gomes, Franciane; Godoy, José Marcus; de Carvalho, Zenildo Lara; de Souza, Elder Magalhães; Rodrigues Silva, José Ivan; Tadeu Lopes, Ricardo

    2014-10-01

    Presently, two nuclear power plants operate in Brazil. Both are located at Itaorna beach, Angra dos Reis, approximately 133 km from Rio de Janeiro city. The reactor cooling circuits require the input of seawater, which is later discharged through a pipeline into the adjacent Piraquara de Fora Cove. The radioactive effluents undergo ion-exchange treatment prior to their release in batches, causing the enrichment of (3)H relative to other radionuclides in the discharged waters. Under steady state conditions, the (3)H gradient in the Piraquara de Fora waters can be used to determine the dependence of the dilution factor on the distance from the discharge point. The present work describes experiments carried out at the reactor site during batch release episodes, including time series sampling at the discharge point and surface seawater sampling every 250 m to a distance of 1250 m, after a double distillation, the (3)H concentration was measured by liquid scintillation counting applying a Quantulus liquid scintillation spectrometer. The obtained results showed a linear relationship between the (3)H concentration and distance from the discharge point. At 1250 m from the discharge point a dilution index of 1:15 was measured which fits the expected value based on modeling. Copyright © 2014 Elsevier Ltd. All rights reserved.

  1. Improving Public Reporting and Data Validation for Complex Surgical Site Infections After Coronary Artery Bypass Graft Surgery and Hip Arthroplasty

    PubMed Central

    Calderwood, Michael S.; Kleinman, Ken; Murphy, Michael V.; Platt, Richard; Huang, Susan S.

    2014-01-01

    Background  Deep and organ/space surgical site infections (D/OS SSI) cause significant morbidity, mortality, and costs. Rates are publicly reported and increasingly used as quality metrics affecting hospital payment. Lack of standardized surveillance methods threaten the accuracy of reported data and decrease confidence in comparisons based upon these data. Methods  We analyzed data from national validation studies that used Medicare claims to trigger chart review for SSI confirmation after coronary artery bypass graft surgery (CABG) and hip arthroplasty. We evaluated code performance (sensitivity and positive predictive value) to select diagnosis codes that best identified D/OS SSI. Codes were analyzed individually and in combination. Results  Analysis included 143 patients with D/OS SSI after CABG and 175 patients with D/OS SSI after hip arthroplasty. For CABG, 9 International Classification of Diseases, 9th Revision (ICD-9) diagnosis codes identified 92% of D/OS SSI, with 1 D/OS SSI identified for every 4 cases with a diagnosis code. For hip arthroplasty, 6 ICD-9 diagnosis codes identified 99% of D/OS SSI, with 1 D/OS SSI identified for every 2 cases with a diagnosis code. Conclusions  This standardized and efficient approach for identifying D/OS SSI can be used by hospitals to improve case detection and public reporting. This method can also be used to identify potential D/OS SSI cases for review during hospital audits for data validation. PMID:25734174

  2. Microglandular adenosis of the breast: a deceptive and still misterious benign lesion.

    PubMed

    Foschini, Maria P; Eusebi, Vincenzo

    2018-06-24

    Microglandular adenosis of the breast (MA), a benign glandular proliferation, was originally described about 35years ago. The lesion, is constituted by small glands all of the same size. Glands are lined by one layer of cuboidal epithelial cells encircled by basal lamina without any evidence of interposed myoepithelial elements. Cells are positive for low weight keratins and S-100 protein and negative for ER, PR and HER 2. Since then, in the years, several malignant lesions all showing microglandular architecture have been regarded either as a precursor or an equivalent manifestation of MA. The latter has been associated to a large number of malignancies that include DCIS, LCIS, ademyoepithelioma, high grade basal like carcinoma, adenoid cystic carcinoma, matrix producing carcinoma, invasive duct carcinoma NOS, spindle cell carcinoma, not to mention acinic cell carcinoma. None of the above tumors were identical to MA. Differences mainly rested not only on the specific structure of the small glands but also on the cytological composition and immunohistochemical features of different lesions. Here a review of the features of MA together with the differential diagnosis with lesions showing microglandular structure is discussed. MA shows similarities with a lesion named microglandular hamartoma/adenosis (MH) of the nasal cavity. The relation of the two similar lesions is discussed. Copyright © 2018. Published by Elsevier Inc.

  3. Fibroblastic connective tissue nevus: Clinicopathological and immunohistochemical study of 14 cases.

    PubMed

    Pennacchia, Ilaria; Kutzner, Heinz; Kazakov, Dmitry V; Mentzel, Thomas

    2017-10-01

    We present herein a series of 14 lesions showing overlapping features with the newly defined benign cutaneous mesenchymal neoplasm labeled as fibroblastic connective tissue nevus (FCTN). Total of 8 patients were male and 5 were female, ranging in age from 1 to 56 years. Lesions appeared as isolated nodules or plaques on the trunk (7 cases), the limbs (4 cases) and the neck (2 cases). Histologically, all cases were composed of bundles of bland spindle cells of fibroblastic/myofibroblastic lineage irregularly branching within the reticular dermis and along fibrous septa in the subcutis. Adnexal structures and dermal adipocytes were entrapped by the fascicles, the epidermis was often papillomatous and elastic fibers were decreased and fragmented. Expression of CD34 and ASMA was found in 8 and 7 cases, respectively. Follow-up was available for 7 patients (mean follow-up, 5 years; range, 1-10 years). None of the cases metastasized or recurred, even when incompletely excised. The differential diagnosis of FCTN is broad and includes hypertrophic scar, dermatofibroma, dermatomyofibroma, pilar leiomyoma, plaque-stage DFSP, CD34-positive plaque-like dermal fibroma, fibroblastic-predominant plexiform fibrohistiocytic tumor, lipofibromatosis, superficial desmoid fibromatosis and fibrous hamartoma of infancy, of which it represents probably the monophasic variant. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  4. Hepatic perivascular epithelioid cell tumor

    PubMed Central

    Tang, Da; Wang, Jianmin; Tian, Yuepeng; Li, Qiuguo; Yan, Haixiong; Wang, Biao; Xiong, Li; Li, Qinglong

    2016-01-01

    Abstract Rational: Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal neoplasm which expresses both myogenic and melanocytic markers. PEComas are found in a variety locations in the body, but up to now only approximately 30 cases about hepatic perivascular epithelioid cell tumor are reported in English language worldwide. Patient concerns: A 32-year-old woman was admitted in our hospital with intermittent right upper quadrant pain for 1 month and recent (1 day) progressive deterioration. Diagnoses: Based on the results of the laboratory examinations and the findings of the computed tomography, the diagnosis of hepatic hamartoma or the hepatocecullar carcinoma with hemorrhage was made. Interventions: The patient underwent a segmentectomy of the liver, and the finally diagnosis of hepatic PEComa was made with immunohistochemical confirmation with HMB-45 and SMA. Outcomes: There is no clinical or radiographic evidence of recurrence 9 months after surgery. Lessons: This kind of tumor is extremely rare and the natural history of PEComa is uncertain, as the treatment protocol for hepatic PEComa has not reached a consensus. But the main treatment of the disease may be surgical resection. Only after long term follow-up can we know whether the tumor is benign or malignant. It appears that longer clinical follow-up is necessary in all patients with hepatic PEComas. PMID:28002331

  5. Hair and Scalp Disorders in a Tuscan Pediatric Dermatological Outpatient Clinic: A Clinical and Epidemiological Evaluation.

    PubMed

    Conti, Rossana; Colucci, Roberta; Arunachalam, Meena; Berti, Samantha; Fabroni, Caterina; De Martino, Maurizio; Dragoni, Federica; Lazzeri, Linda; Pisaneschi, Lisa; Moretti, Silvia

    2016-01-01

    The aim of this study was to evaluate the clinical and epidemiological profile of hair and scalp disorders in children referred to the Pediatric Dermatology Outpatient Clinic. We performed a retrospective study of children with hair loss problems or scalp diseases who turned to the Pediatric Dermatology Service, Anna Meyer Pediatric Hospital, Florence, Italy, from January 1, 2009, to December 31, 2009. Demographics, personal and familial medical history, laboratory tests, clinical examination, final diagnosis and therapeutic interventions were obtained from the manual chart review. Of the 2,640 children who had access to the Pediatric Dermatology Service, 190 (7.19%) had a hair or scalp disorder. Among the 190 children, 60 (31.57%) presented with nonscarring alopecia, 56 (29.47%) had benign neoplasias, hamartomas or vascular malformations of the scalp, 51 (26.84%) had scalp inflammatory diseases, 14 (7.36%) had scarring alopecia, 5 (2.63%) had infections and 2 (1.05%) had infestation of the scalp. A case of constitutional hypertrichosis (0.52%) and also a case (0.52%) of lamellar ichthyosis were diagnosed. Our results underline that hair and scalp diseases represent an important percentage of admittances to a dermatological pediatric outpatient clinic. The variety and complexity of the diseases observed in this study included diseases commonly found also in adulthood. © 2015 S. Karger AG, Basel.

  6. Novel Histopathological Patterns in Cortical Tubers of Epilepsy Surgery Patients with Tuberous Sclerosis Complex.

    PubMed

    Mühlebner, Angelika; van Scheppingen, Jackelien; Hulshof, Hanna M; Scholl, Theresa; Iyer, Anand M; Anink, Jasper J; van den Ouweland, Ans M W; Nellist, Mark D; Jansen, Floor E; Spliet, Wim G M; Krsek, Pavel; Benova, Barbora; Zamecnik, Josef; Crino, Peter B; Prayer, Daniela; Czech, Thomas; Wöhrer, Adelheid; Rahimi, Jasmin; Höftberger, Romana; Hainfellner, Johannes A; Feucht, Martha; Aronica, Eleonora

    2016-01-01

    Tuberous Sclerosis Complex (TSC) is a genetic hamartoma syndrome frequently associated with severe intractable epilepsy. In some TSC patients epilepsy surgery is a promising treatment option provided that the epileptogenic zone can be precisely delineated. TSC brain lesions (cortical tubers) contain dysmorphic neurons, brightly eosinophilic giant cells and white matter alterations in various proportions. However, a histological classification system has not been established for tubers. Therefore, the aim of this study was to define distinct histological patterns within tubers based on semi-automated histological quantification and to find clinically significant correlations. In total, we studied 28 cortical tubers and seven samples of perituberal cortex from 28 TSC patients who had undergone epilepsy surgery. We assessed mammalian target of rapamycin complex 1 (mTORC1) activation, the numbers of giant cells, dysmorphic neurons, neurons, and oligodendrocytes, and calcification, gliosis, angiogenesis, inflammation, and myelin content. Three distinct histological profiles emerged based on the proportion of calcifications, dysmorphic neurons and giant cells designated types A, B, and C. In the latter two types we were able to subsequently associate them with specific features on presurgical MRI. Therefore, these histopathological patterns provide consistent criteria for improved definition of the clinico-pathological features of cortical tubers identified by MRI and provide a basis for further exploration of the functional and molecular features of cortical tubers in TSC.

  7. Novel Histopathological Patterns in Cortical Tubers of Epilepsy Surgery Patients with Tuberous Sclerosis Complex

    PubMed Central

    Hulshof, Hanna M.; Scholl, Theresa; Iyer, Anand M.; Anink, Jasper J.; van den Ouweland, Ans M. W.; Nellist, Mark D.; Jansen, Floor E.; Spliet, Wim G. M.; Krsek, Pavel; Benova, Barbora; Zamecnik, Josef; Crino, Peter B.; Prayer, Daniela; Czech, Thomas; Wöhrer, Adelheid; Rahimi, Jasmin; Höftberger, Romana; Hainfellner, Johannes A.; Feucht, Martha; Aronica, Eleonora

    2016-01-01

    Tuberous Sclerosis Complex (TSC) is a genetic hamartoma syndrome frequently associated with severe intractable epilepsy. In some TSC patients epilepsy surgery is a promising treatment option provided that the epileptogenic zone can be precisely delineated. TSC brain lesions (cortical tubers) contain dysmorphic neurons, brightly eosinophilic giant cells and white matter alterations in various proportions. However, a histological classification system has not been established for tubers. Therefore, the aim of this study was to define distinct histological patterns within tubers based on semi-automated histological quantification and to find clinically significant correlations. In total, we studied 28 cortical tubers and seven samples of perituberal cortex from 28 TSC patients who had undergone epilepsy surgery. We assessed mammalian target of rapamycin complex 1 (mTORC1) activation, the numbers of giant cells, dysmorphic neurons, neurons, and oligodendrocytes, and calcification, gliosis, angiogenesis, inflammation, and myelin content. Three distinct histological profiles emerged based on the proportion of calcifications, dysmorphic neurons and giant cells designated types A, B, and C. In the latter two types we were able to subsequently associate them with specific features on presurgical MRI. Therefore, these histopathological patterns provide consistent criteria for improved definition of the clinico-pathological features of cortical tubers identified by MRI and provide a basis for further exploration of the functional and molecular features of cortical tubers in TSC. PMID:27295297

  8. Germline Mutations in BMPR1A/ALK3 Cause a Subset of Cases of Juvenile Polyposis Syndrome and of Cowden and Bannayan-Riley-Ruvalcaba Syndromes*

    PubMed Central

    Zhou, Xiao-Ping; Woodford-Richens, Kelly; Lehtonen, Rainer; Kurose, Keisuke; Aldred, Micheala; Hampel, Heather; Launonen, Virpi; Virta, Sanno; Pilarski, Robert; Salovaara, Reijo; Bodmer, Walter F.; Conrad, Beth A.; Dunlop, Malcolm; Hodgson, Shirley V.; Iwama, Takeo; Järvinen, Heikki; Kellokumpu, Ilmo; Kim, J. C.; Leggett, Barbara; Markie, David; Mecklin, Jukka-Pekka; Neale, Kay; Phillips, Robin; Piris, Juan; Rozen, Paul; Houlston, Richard S.; Aaltonen, Lauri A.; Tomlinson, Ian P. M.; Eng, Charis

    2001-01-01

    Juvenile polyposis syndrome (JPS) is an inherited hamartomatous-polyposis syndrome with a risk for colon cancer. JPS is a clinical diagnosis by exclusion, and, before susceptibility genes were identified, JPS could easily be confused with other inherited hamartoma syndromes, such as Bannayan-Riley-Ruvalcaba syndrome (BRRS) and Cowden syndrome (CS). Germline mutations of MADH4 (SMAD4) have been described in a variable number of probands with JPS. A series of familial and isolated European probands without MADH4 mutations were analyzed for germline mutations in BMPR1A, a member of the transforming growth-factor β–receptor superfamily, upstream from the SMAD pathway. Overall, 10 (38%) probands were found to have germline BMPR1A mutations, 8 of which resulted in truncated receptors and 2 of which resulted in missense alterations (C124R and C376Y). Almost all available component tumors from mutation-positive cases showed loss of heterozygosity (LOH) in the BMPR1A region, whereas those from mutation-negative cases did not. One proband with CS/CS-like phenotype was also found to have a germline BMPR1A missense mutation (A338D). Thus, germline BMPR1A mutations cause a significant proportion of cases of JPS and might define a small subset of cases of CS/BRRS with specific colonic phenotype. PMID:11536076

  9. Ligand binding to the Fe(III)-protoporphyrin IX complex of phosphodiesterase from Escherichia coli (Ec DOS) markedly enhances catalysis of cyclic di-GMP: roles of Met95, Arg97, and Phe113 of the putative heme distal side in catalytic regulation and ligand binding.

    PubMed

    Tanaka, Atsunari; Shimizu, Toru

    2008-12-16

    Phosphodiesterase (Ec DOS) from Escherichia coli is a gas-sensor enzyme in which binding of gas molecules, such as O(2), CO, and NO, to the Fe(II)-protoporphyrin IX complex in the sensor domain stimulates phosphodiesterase activity toward cyclic-di-GMP. In this study, we report that external axial ligands, such as cyanide or imidazole, bind to Fe(III)-protoporphyrin IX in the sensor domain and induce a 10- to 11-fold increase (from 8.1 up to 86 min(-1)) in catalysis, which is more substantial than that (6.3 to 7.2-fold) observed for other gas-stimulated Fe(II) heme-bound enzymes. Catalytic activity (50 min(-1)) of the heme-free mutant, H77A, was comparable to that of the ligand-stimulated enzymes. Accordingly, we propose that the heme at the sensor domain inhibits catalysis and that ligand binding to the heme iron complex releases this catalytic suppression. Furthermore, mutations of Met95, Arg97, and Phe113 at the putative heme distal side suppressed the ligand effects on catalysis. The rate constants (19,000 x 10(-5) microM(-1)min(-1)) for cyanide binding to the M95A and M95L mutants of the full-length enzyme were 633-fold higher than that to wild-type Ec DOS (30 x 10(-5) microM(-1)min(-1)). The absorption spectrum of the F113Y mutant suggests that the Tyr O(-) group directly coordinates to the Fe(III) complex and that the cyanide binding rate to the mutant is very slow, compared with those of the wild-type and other mutant proteins. We observed a similar trend in the binding behavior of imidazole to full-length mutant enzymes. Therefore, while Met95 and Phe113 are not direct axial ligands for the Fe(III) complex, catalytic, spectroscopic, and ligand binding evidence suggests that these residues are located in the vicinity of the heme.

  10. Influence of Ca2+ on the mechanosensitivity of the hair cells in the lateral line organs of Necturus maculosus.

    PubMed

    Jørgensen, F

    1983-08-01

    The degree of synchronization (DOS) between the afferent spike activity from one stitch of the lateral line of Necturus maculosus (in vivo) and the mechanical stimulation of one neuromast of the same stitch was measured under different circumstances. The DOS was found to be independent of changes in the concentration of monovalent cations (Na+, K+ and choline+) in the bulk solution at high Ca concentration (1 mM). DOS was also independent of the Ca concentration in the range 1 mM-1 microM in Tris-HCl buffer, but was markedly reduced at Ca = 10 microM in MOPS-KOH buffer. The reduced DOS, however, could be restored by addition of 10-20 mM KCl. 5 mM of 4-aminopyridine did not influence the DOS at high Ca concentration, but completely reduced DOS at Ca = 10 microM. D600 (a methoxy derivative of verapamil) decreased DOS both at high and low Ca concentration.

  11. The influence of cumulus cells during in vitro fertilization of buffalo (Bubalus bubalis) denuded oocytes that have undergone vitrification.

    PubMed

    Attanasio, Laura; De Rosa, Anna; De Blasi, Marina; Neglia, Gianluca; Zicarelli, Luigi; Campanile, Giuseppe; Gasparrini, Bianca

    2010-11-01

    The aim of this work was to evaluate whether providing a support of cumulus cells during IVF of buffalo denuded oocytes submitted to vitrification-warming enhances their fertilizing ability. In vitro matured denuded oocytes were vitrified by Cryotop in 20% EG + 20% of DMSO and 0.5 M sucrose and warmed into decreasing concentrations of sucrose (1.25 M-0.3M). Oocytes that survived vitrification were fertilized: 1) in the absence of a somatic support (DOs); 2) in the presence of bovine cumulus cells in suspension (DOs+susp); 3) on a bovine cumulus monolayer (DOs+monol); and 4) with intact bovine COCs in a 1:1 ratio (DOs+COCs). In vitro matured oocytes were fertilized and cultured to the blastocyst stage as a control. An increased cleavage rate was obtained from DOs+COCs (60.9%) compared to DOs, DOs+susp (43.6 and 38.4, respectively; P < 0.01) and DOs+monol (47.5%; P < 0.05). Interestingly, cleavage rate of DOs+COCs was similar to that of fresh control oocytes (67.8%). However, development to blastocysts significantly decreased in all vitrification groups compared to the control (P < 0.01). In conclusion the co-culture with intact COCs during IVF completely restores fertilizing capability of buffalo denuded vitrified oocytes, without improving blastocyst development. Copyright © 2010 Elsevier Inc. All rights reserved.

  12. Antibiotic and Duration of Perioperative Prophylaxis Predicts Surgical Site Infection in Head and Neck Surgery.

    PubMed

    Langerman, Alexander; Thisted, Ronald; Hohmann, Samuel; Howell, Michael

    2016-06-01

    To examine the effect of giving antibiotics on the day of surgery (DOS) vs DOS and first postoperative day (DOS+1) for prophylaxis against surgical site infection (SSI) in clean-contaminated head and neck surgery (CCHNS). Retrospective multi-institution analysis using University HealthSystem Consortium data. A multivariate logistic regression model of 8836 discharge records from patients undergoing CCHNS was used to determine the odds of SSI for antibiotic agent/duration combinations. Ninety-two academic and affiliated medical centers from 2008 to 2011. Ampicillin/sulbactam, clindamycin, cefazolin + metronidazole, and cefazolin alone were the most common antibiotics. For patients receiving antibiotics only on DOS, there was no significant difference in odds of SSI based on antibiotic choice. When given on the DOS and DOS+1, patients receiving ampicillin/sulbactam had a reduction in odds of SSI by over two-thirds (odds ratio [OR], 0.28 [95% confidence interval, 0.13-0.61], P = .001, compared with ampicillin/sulbactam on DOS only), whereas this effect was not seen with clindamycin (1.82 [0.93-3.56], P = .078, compared with clindamycin on DOS only). Prolonging clindamycin beyond the DOS was associated with a higher odds of SSI compared with DOS-only ampicillin/sulbactam (OR, 2.66; 95% CI, 1.33-5.30; P = .006). These relationships held in a subset of physicians and hospitals that used multiple different regimens. DOS+1 regimens were not associated with an increased odds of antibiotic-induced complications. Prolonging ampicillin/sulbactam beyond the day of surgery may have a protective effect against SSI, and 1 or more days of ampicillin/sulbactam may be preferable to multiple days of clindamycin. New randomized trials are needed to define the ideal regimen for CCHNS. © American Academy of Otolaryngology—Head and Neck Surgery Foundation 2016.

  13. Dissolved organic sulfur in the ocean: Biogeochemistry of a petagram inventory

    NASA Astrophysics Data System (ADS)

    Ksionzek, Kerstin B.; Lechtenfeld, Oliver J.; McCallister, S. Leigh; Schmitt-Kopplin, Philippe; Geuer, Jana K.; Geibert, Walter; Koch, Boris P.

    2016-10-01

    Although sulfur is an essential element for marine primary production and critical for climate processes, little is known about the oceanic pool of nonvolatile dissolved organic sulfur (DOS). We present a basin-scale distribution of solid-phase extractable DOS in the East Atlantic Ocean and the Atlantic sector of the Southern Ocean. Although molar DOS versus dissolved organic nitrogen (DON) ratios of 0.11 ± 0.024 in Atlantic surface water resembled phytoplankton stoichiometry (sulfur/nitrogen ~ 0.08), increasing dissolved organic carbon (DOC) versus DOS ratios and decreasing methionine-S yield demonstrated selective DOS removal and active involvement in marine biogeochemical cycles. Based on stoichiometric estimates, the minimum global inventory of marine DOS is 6.7 petagrams of sulfur, exceeding all other marine organic sulfur reservoirs by an order of magnitude.

  14. Stratigraphy of fluvial sediment sequences and their palaeoenvironmental information in the foreland of the Serra dos Órgãos, southeastern Brazil

    NASA Astrophysics Data System (ADS)

    Kirchner, André; Nehren, Udo; Heinrich, Jürgen

    2013-04-01

    In the hinterland of Rio de Janeiro city the rivers Guapiaçu, Macacu and Iconha originate in the Serra dos Órgãos mountain range and drain into the Atlantic Ocean. Since their channelization in the 1950s, higher flow velocities caused an incision of the rivers into the valley fills. These circumstances provide the possibility to study the alluvial deposits along the streams during low water level and allow conclusions on palaeoenvironmental change and landscape history. Sedimentological investigations of 13 exposures as well as AMS 14C measurements were carried out to investigate sediment properties and reconstruct the sedimentation history within the floodplains. These results enable to distinguish three different facies units. A late Pleistocene Unit I can be detected at the base of the observed exposures and consists of clast-supported fine to coarse gravels. It can be assumed that the gravel bodies were formed by a climatically induced erosional-depositional cycle within a braided river system. The gravels are overlaid by Unit II, a grayish to bluish loam mainly of mid-Holocene age. During generally drier climates these loams have been deposited during high water stages or flooding events as a splay facies proximal to the rivers. A reduced flow competence and relatively stable morphodynamic conditions are assumed for that period. Unit III accumulated in the late Holocene typically consists of several meters of planar or cross bedded sands to fine gravels, interfingered by loamy inclusions, buried peat bogs and organic debris. Fining-upward sequences can be frequently studied within Unit III which were completed by loamy sediments in the uppermost parts of the exposures. The increased flow competence from Unit II to Unit III seems to be a fluvial response to the increased humidity of the late Holocene as well as the enhancement of El Niño-Southern Oscillation (ENSO). Heavy rainfall likely caused higher sediment supply from the steep slopes as well as a

  15. Simplified expressions that incorporate finite pulse effects into coherent two-dimensional optical spectra.

    PubMed

    Do, Thanh Nhut; Gelin, Maxim F; Tan, Howe-Siang

    2017-10-14

    We derive general expressions that incorporate finite pulse envelope effects into a coherent two-dimensional optical spectroscopy (2DOS) technique. These expressions are simpler and less computationally intensive than the conventional triple integral calculations needed to simulate 2DOS spectra. The simplified expressions involving multiplications of arbitrary pulse spectra with 2D spectral response function are shown to be exactly equal to the conventional triple integral calculations of 2DOS spectra if the 2D spectral response functions do not vary with population time. With minor modifications, they are also accurate for 2D spectral response functions with quantum beats and exponential decay during population time. These conditions cover a broad range of experimental 2DOS spectra. For certain analytically defined pulse spectra, we also derived expressions of 2D spectra for arbitrary population time dependent 2DOS spectral response functions. Having simpler and more efficient methods to calculate experimentally relevant 2DOS spectra with finite pulse effect considered will be important in the simulation and understanding of the complex systems routinely being studied by using 2DOS.

  16. Techniques of remote sensing applied to the environmental analysis of part of an aquifer located in the São José dos Campos Region sp, Brazil.

    PubMed

    Bressan, Mariana Affonseca; Dos Anjos, Célio Eustáquio

    2003-05-01

    The anthropogenic activity on the surface can modify and introduce new mechanisms of recharging the groundwater system, modifying the tax, the frequency and the quality of recharge of underground waters. The understanding of these mechanisms and the correct evaluation of such modifications are fundamental in determining the vulnerability of groundwater contamination. The groundwater flow of the South Paraíba Compartment, in the region of São José dos Campos, São Paulo, is directly related to structural features of the Taubaté Basin and, therefore, the analysis of its behaviour enhances the understanding of tectonic structure. The methodology adopted for this work consists in pre-processing and processing of the satellite images, visual interpretation of HSI products, field work and data integration. The derivation of the main structural features was based on visual analysis of the texture elements of drainage, and the relief in sedimentary and crystalline rocks. Statistical analysis of the feature densities and the metric-geometric relations between the analysed elements have been conducted. The crystalline rocks, on which the sediments were laying, conditions and controls the structural arrangement of sedimentary formations. The formation of the South Paraíba Grabén is associated with Cenozoic distensive movement which reactivated old features of crust weakness and generated previous cycles with normal characteristics. The environmental analysis is based on the integration of the existing methodology to characterise vulnerability of an universal pollutant and density fracture zone. The digital integration was processed using GIS (Geographic Information System) to delineate five defined vulnerability classes. The hydrogeological settings were analysed in each thematic map and, using fuzzy logic, an index for each different vulnerability class was compiled. Evidence maps could be combined in a series of steps using map algebra.

  17. DYNAMIC OSCILLATORY STRETCHING EFFICACY ON HAMSTRING EXTENSIBILITY AND STRETCH TOLERANCE: A RANDOMIZED CONTROLLED TRIAL

    PubMed Central

    Tee, Jason Cameron; Stewart, Aimee

    2017-01-01

    Background While static stretch (SS), proprioceptive neuromuscular facilitation (PNF) and oscillatory physiological mobilization techniques are documented to have positive effects on a range of motion (ROM), there are no reports on the effect of dynamic oscillatory stretching (DOS), a technique that combines these three techniques, on hamstring extensibility. Purpose To determine whether DOS improves hamstring extensibility and stretch tolerance to a greater degree than SS in asymptomatic young participants. Study Design Randomized Controlled Trial. Methods Sixty participants (47 females, 13 males, mean age 22 ± 1 years, height 166 ± 6 centimeters, body mass 67.6 ± 9.7 kg) completed a passive straight leg (SLR) to establish hamstring extensibility and stretch tolerance as perceived by participants, using a visual analogue scale (VAS). Participants were randomly assigned to one of two treatment groups (SS or DOS) or a placebo control (20 per group). Tests were repeated immediately following and one hour after each intervention. Data were assessed using a two-way repeated measure analysis of variance (ANOVA) and Tukey's post hoc test. Results Immediately post-intervention, there was a significant improvement in the hamstring extensibility as measured by the SLR in both the SS and DOS groups, with the DOS group exhibiting a significantly greater increase than the SS group (Control 73 ± 12°, SS 86 ± 8°, DOS 94 ± 11°, p < 0.001). One hour post-intervention, hamstring extensibility in the DOS group remained elevated, while the SS group no longer differed from the control group (Control 73 ± 12°, SS 80 ± 8°, DOS 89 ± 12°, p = 0.001). Furthermore, the stretch tolerance remained significantly elevated for the SS group, but there was no difference between the control and DOS groups, (Control 4.6 ± 1.3, SS 5.9 ± 0.8, DOS 4.3 ± 1.0 AU, p < 0.001). Conclusion DOS was more effective than SS at achieving

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

    Busbey, A.B.

    A number of methods and products, both hardware and software, to allow data exchange between Apple Macintosh computers and MS-DOS based systems. These included serial null modem connections, MS-DOS hardware and/or software emulation, MS-DOS disk-reading hardware and networking.

  19. Balancing Act

    Cancer.gov

    Part of being an Active, More Powerful You means finding balance in your daily life: taking on the Must-dos and finding time for some Should Dos and Want-to-Dos. Sometimes, emotions and commitments can come into play and upset the balance.

  20. Accurate description of charge transport in organic field effect transistors using an experimentally extracted density of states

    NASA Astrophysics Data System (ADS)

    Roelofs, W. S. C.; Mathijssen, S. G. J.; Janssen, R. A. J.; de Leeuw, D. M.; Kemerink, M.

    2012-02-01

    The width and shape of the density of states (DOS) are key parameters to describe the charge transport of organic semiconductors. Here we extract the DOS using scanning Kelvin probe microscopy on a self-assembled monolayer field effect transistor (SAMFET). The semiconductor is only a single monolayer which has allowed extraction of the DOS over a wide energy range, pushing the methodology to its fundamental limit. The measured DOS consists of an exponential distribution of deep states with additional localized states on top. The charge transport has been calculated in a generic variable range-hopping model that allows any DOS as input. We show that with the experimentally extracted DOS an excellent agreement between measured and calculated transfer curves is obtained. This shows that detailed knowledge of the density of states is a prerequisite to consistently describe the transfer characteristics of organic field effect transistors.

  1. 77 FR 14581 - Notice of Meeting of the Cultural Property Advisory Committee

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-03-12

    ... DEPARTMENT OF STATE [Public Notice 7767; Guatemala Docket No. DOS-2012-0011; Mali Docket No. DOS... Government of the Republic of Guatemala Concerning the Imposition of Import Restrictions on Archaeological Objects and Materials from the Pre- Columbian Cultures of Guatemala (MOU) [Docket No. DOS-2012-0011] and...

  2. Whenever You Use a Computer You Are Using a Program Called an Operating System.

    ERIC Educational Resources Information Center

    Cook, Rick

    1984-01-01

    Examines design, features, and shortcomings of eight disk-based operating systems designed for general use that are popular or most likely to affect the future of microcomputing. Included are the CP/M family, MS-DOS, Apple DOS/ProDOS, Unix, Pick, the p-System, TRSDOS, and Macintosh/Lisa. (MBR)

  3. 76 FR 76476 - Notice of Meeting of the Cultural Property Advisory Committee

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-12-07

    ... DEPARTMENT OF STATE [Public Notice 7658; Cyprus Docket No. DOS-2011-0135; Peru Docket No. DOS-2011... Ritual Ethnological Material [Docket No. DOS-2011-0135], and with Peru Memorandum of Understanding Between the Government of the United States of America and the Government of the Republic of Peru...

  4. Glioneuronal Heterotopia Presenting As a Cerebellopontine angle Tumor of the cranial Nerve VIII, Case Report.

    PubMed

    Peris-Celda, M; Giannini, C; Diehn, F E; Eckel, L J; Neff, B A; Van Gompel, J J

    2018-04-03

    Vestibular schwannomas and meningiomas account for the great majority of lesions arising in the cerebellopontine angle (CPA). In this report, we present a case of glioneuronal heterotopia, also known as glioneuronal hamartoma, arising from the VIII cranial nerve, which is an extremely uncommon lesion. Important radiologic and surgical aspects are reviewed, which may help in early recognition and intraoperative decision making when these lesions are encountered. A healthy 29-year-old female presented with intermittent right facial numbness. Magnetic resonance imaging (MRI) showed an incidental minimally enhancing cerebellopontine angle lesion on the right VII-VIII cranial nerve complex. The patient declined serial observation and opted for operative intervention for resection. Intraoperatively, the lesion resembled neural tissue and was continuous with the VIII cranial nerve. Pathological analysis demonstrated mature glioneuronal tissue consistent with hamartomatous brain tissue. The patient maintained normal hearing and facial nerve function after surgery. Radiologic, surgical and pathological characteristics are described. Ectopic glioneuronal tissue of the VIII cranial nerve is a rare non-neoplastic lesion, and should be considered in the differential diagnosis of unusual appearing intracanalicular and cerebellopontine angle lesions. The congenital and benign nature of this entity makes observation a valid option for these cases, although they are so infrequent that they are often presumptively managed as vestibular schwannomas. Attempts to radically resect these lesions may result in higher rates of hearing loss or facial palsy due to their continuity with the cranial nerves. Copyright © 2018 Elsevier Inc. All rights reserved.

  5. [Clinical and genetic study patients with tuberous sclerosis complex].

    PubMed

    Rubilar, Carla; López, Francisca; Troncoso, Mónica; Barrios, Andrés; Herrera, Luisa

    2017-02-01

    Tuberous sclerosis complex (TSC) is a multisystem autosomal dominant disease caused by mutations in the tumor suppressor genes TSC1 or TSC2. To characterize clinically and genetically patients diagnosed with TSC. Descriptive study of clinical records of 42 patients from a pediatric neuropsychiatry department diagnosed with TSC and genetic study in 21 of them. The exon 15 of TSC1 gene and exons 33, 36 and 37 of TSC2 gene were amplified by polymerase chain reaction and sequenced. The relationship between the mutations found with the severity and clinical course were analyzed. In 61.9% of the patients the symptoms began before 6 months of age. The initial most frequent manifestations of TSC were new onset of seizures (73.8%) and the detection of cardiac rhabdomyomas (16.6%). During the evolution of the disease all patients had neurological involvement; 92.9% had epilepsy. All patients presented hypomelanotic spots, 47.6% facial angiofibromas, 23.8% Shagreen patch, 47.6 heart rhabdomyomas and 35.7% retinal hamartomas. In the genetic study of 21 patients two heterozygous pathogenic mutations in TSC1 and one in TSC2 genes were identified. The latter had a more severe clinical phenotype. Neurological and dermatological manifestations were the most frequent ones in patients with TSC. Two pathogenic mutations in TSC1 and one in TSC2 genes were identified. The patient with TSC2 mutation manifested a more severe clinical phenotype.

  6. A 35-year-old woman presenting with an unusual post-traumatic leiomyoma of the nipple: a case report.

    PubMed

    Pavlidis, Leonidas; Vakirlis, Efstratios; Spyropoulou, Georgia-Alexandra; Pramateftakis, Manousos Georgios; Dionyssiou, Dimitris; Demiri, Efterpi

    2013-02-19

    Leiomyoma of the mammary papilla is one of the most uncommon nipple tumors with only 50 cases reported in the literature until now. To the best of our knowledge we present the first report of a nipple leiomyoma that originated from a traumatic abrasion caused by breastfeeding. A 35-year-old healthy Caucasian female with a cauliflower-like tender and pink nodular mass that was approximately 10mm in diameter presented to our out-patients department. The patient suggested that the mass originated from a traumatic abrasion caused by breastfeeding three years ago and it has been slowly growing ever since.An excision biopsy was performed. The histological and immunohistochemical examination confirmed the diagnosis of leiomyoma. There were no postoperative complications or any sign of local recurrence four years postoperatively. Leiomyoma of the mammary papilla is a rare benign neoplasm that usually appears as a solid tender nodule. Differential diagnosis comprises breast carcinoma, leiomyosarcoma and myoid hamartoma. The recommended treatment is complete excision of the tumor with histologically confirmed tumor-free margins otherwise recurrence is possible. A detailed history of the patient's disease can reveal the original etiology. This is an original case report that will have particular interest to plastic surgeons, dermatologists, and pathologists. The pathogenetic mechanism was trauma of the nipple. According to our review of the literature this particular information has never been reported and we think that it may advance our knowledge of this very infrequent tumor.

  7. Cardiac Pathology and Molecular Epidemiology by Avian Leukosis Viruses in Japan

    PubMed Central

    Nakamura, Sayuri; Ochiai, Kenji; Ochi, Akihiro; Yabushita, Hiroki; Abe, Asumi; Kishi, Sayaka; Sunden, Yuji; Umemura, Takashi

    2014-01-01

    Epidemiological studies suggest that retroviruses, including human immunodeficiency virus type 1, are associated with cardiomyopathy and myocarditis, but a causal relationship remains to be established. We encountered unusual cardiomyocyte hypertrophy and mitosis in Japanese native fowls infected with subgroup A of the avian leukosis viruses (ALVs-A), which belong to the genus Alpharetrovirus of the family Retroviridae and mainly induce lymphoid neoplasm in chickens. The affected hearts were evaluated by histopathology and immunohistochemistry, viral isolation, viral genome sequencing and experimental infection. There was non-suppurative myocarditis in eighteen fowls and seven of them had abnormal cardiomyocytes, which were distributed predominantly in the left ventricular wall and showed hypertrophic cytoplasm and atypical large nuclei. Nuclear chains and mitosis were frequently noted in these cardiomyocytes and immunohistochemistry for proliferating cell nuclear antigen supported the enhancement of mitotic activity. ALVs were isolated from all affected cases and phylogenic analysis of envSU genes showed that the isolates were mainly classified into two different clusters, suggesting viral genome diversity. In ovo experimental infection with two of the isolates was demonstrated to cause myocarditis and cardiomyocyte hypertrophy similar to those in the naturally occurring lesions and cardiac hamartoma (rhabdomyoma) in a shorter period of time (at 70 days of age) than expected. These results indicate that ALVs cause myocarditis as well as cardiomyocyte abnormality in chickens, implying a pathogenetic mechanism different from insertional mutagenesis and the existence of retrovirus-induced heart disorder. PMID:24466146

  8. Clinicopathologic analysis of 21 cases of nevus sebaceus: a retrospective study.

    PubMed

    Simi, C M; Rajalakshmi, T; Correa, Marjorie

    2008-01-01

    Nevus sebaceus (NS), otherwise designated as 'organoid nevus', involves proliferative changes of the sebaceous glands, sweat glands, and the hair follicles. It displays a range of appearances, depending on the lesion's age. To study the histopathological features of NS and correlate these with clinical findings. All skin biopsy specimens over a 12-year period from 1995 to 2007 which had a diagnosis of NS were included. Clinical data with follow-up notes and histopathology were reviewed. Half of the cases had a verrucous clinical appearance, while the rest presented as papules, plaques, or patches. All the cases showed immature hair follicles, and 24% of cases showed immature sebaceous glands. Normal terminal hair follicles were characteristically absent in the lesion. Nineteen percent of the cases showed dilated apocrine glands, and 14% showed hyperplasia of eccrine glands. Epidermal changes in the form of acanthosis, papillomatosis, and hyperkeratosis were seen in 86% of cases. Dilated keratin-filled infundibula were observed in 24% of cases. One case was associated with a squamous cell carcinoma. Nevus sebaceus is a cutaneous hamartoma, consisting of various elements indigenous to the organ. Normal terminal hair follicles are characteristically absent in the lesion although the same may be seen in rest of the epidermis, a feature of diagnostic importance, not usually highlighted in literature. The divergent differentiation observed in NS is consistent with the common embryologic origin of the folliculosebaceous-apocrine unit and should not mislead the pathologist.

  9. Nevus sebaceus: a clinicopathological study of 168 cases and review of the literature.

    PubMed

    Kamyab-Hesari, Kambiz; Seirafi, Hassan; Jahan, Shahin; Aghazadeh, Nessa; Hejazi, Pardis; Azizpour, Arghavan; Goodarzi, Azadeh

    2016-02-01

    Nevus sebaceus is a hamartoma composed of abnormal epidermal and dermal components with clinical and histopathological features that change with aging. Prophylactic excision of the lesion was advised historically considering its potential for giving rise to malignant tumors. However, whether early prophylactic excision should be performed has been questioned recently as most of the tumors have been found to be benign in origin. The histopathologic features of 168 patients with nevus sebaceus were reviewed to determine the frequency of different histologic features as well as associated neoplastic growths. Acanthosis, papillomatosis, and basal layer pigmentation were the most frequent findings. Hyperplastic changes of the sebaceous glands became obvious during the second decade and were present in 60% of the specimens from individuals older than 20. Inflammatory infiltrate was almost invariably present. Skin adnexal changes were frequently noted, including sebaceous gland hyperplasia (93.5%) and primitive hair follicles (76.8%). In 88.5% of scalp lesions, markedly decreased terminal hair was observed. Ectopic apocrine glands were present in 55.4% of specimens, while 24.4% showed anomalous ductal sweat gland structures resembling eccrine hyperplasia. Neoplastic changes were detected in nine cases, including four trichoblastomas, three tricholemmomas, and two syringocystadenoma papilliferums. Development of malignancies in sebaceus nevi is a rare phenomenon, and decision for excision of the lesion should be made after thorough evaluation of the pros and cons. © 2015 The International Society of Dermatology.

  10. Multiple cavernous malformations presenting in a patient with Poland syndrome: A case report

    PubMed Central

    2011-01-01

    Introduction Poland syndrome is a congenital disorder related to chest and hand anomalies on one side of the body. Its etiology remains unclear, with an ipsilateral vascular alteration (of unknown origin) to the subclavian artery in early embryogenesis being the currently accepted theory. Cavernous malformations are vascular hamartomas, which have been linked to a genetic etiology, particularly in familial cases, which commonly present with multiple lesions. Our case report is the first to describe multiple cavernous malformations associated with Poland syndrome, further supporting the vascular etiology theory, but pointing to a genetic rather than a mechanistic factor disrupting blood flow in the corresponding vessels. Case presentation A 41-year-old Caucasian man with Poland syndrome on the right side of his body presented to our hospital with a secondary generalized seizure and was found to have multiple cavernous malformations distributed in his brain, cerebellum, and brain stem, with a predominance of lesions in the left hemisphere. Conclusion The distribution of cavernous malformations in the left hemisphere and the right-sided Poland syndrome in our patient could not be explained by a mechanistic disruption of one of the subclavian arteries. A genetic alteration, as in familial cavernous malformations, would be a more appropriate etiologic diagnosis of Poland syndrome in our patient. Further genetic and pathological studies of the involved blood vessels in patients with Poland syndrome could lead to a better understanding of the disease. PMID:21933407

  11. Do children with tuberous sclerosis complex have superior musical skill?--A unique tendency of musical responsiveness in children with TSC.

    PubMed

    Matsuyama, Kumi; Ohsawa, Isao; Ogawa, Toyoaki

    2007-04-01

    Tuberous sclerosis complex (TSC) is an autosomal dominant disorder that manifests with symptoms that might include mental retardation, epilepsy, skin lesions, and hamartomas in the heart, brain, and kidneys. Anecdotal reports have characterized children with TSC as having high music responsiveness despite their developmental delay. This study is intended to investigate this putative musical skill of children with TSC and to elucidate the presence of non-delayed facets of their development. This study examined 11 children with TSC: 10 children with DSM-IV autism and 92 healthy children who participated as control subjects. Correlation was examined between results obtained using Non-Verbal MMRC, which is a validated musical responsiveness battery, and results of a scientifically accepted standardized pediatric developmental test: the New Edition of the Kyoto Scale of Psychological Development. Inter-rater reliability among the three raters was also assessed. The rhythm or melody score on the Non-Verbal MMRC and DA among children with TSC showed no significant correlation. In contrast, a significant correlation was found among normal children and those with autism. Moreover, the inter-rater reliability was good. The results demonstrate that children with TSC show high responsiveness to musical stimuli despite otherwise delayed development (e.g., language, cognition, motor skills). This report is the first stating that children with TSC have a unique tendency in terms of correlation between music and developmental age. These findings indicate a non-delayed area of TSC children's development and suggest the use of music as therapeutic intervention.

  12. 28 CFR 51.20 - Form of submissions.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... megabyte MS-DOS formatted diskettes; 5 1/4″ 1.2 megabyte MS-DOS formatted floppy disks; nine-track tape... provided in hard copy. (c) All magnetic media shall be clearly labeled with the following information: (1... a disk operating system (DOS) file, it shall be formatted in a standard American Standard Code for...

  13. A Semi-Analytical Extraction Method for Interface and Bulk Density of States in Metal Oxide Thin-Film Transistors

    PubMed Central

    Chen, Weifeng; Wu, Weijing; Zhou, Lei; Xu, Miao; Wang, Lei; Peng, Junbiao

    2018-01-01

    A semi-analytical extraction method of interface and bulk density of states (DOS) is proposed by using the low-frequency capacitance–voltage characteristics and current–voltage characteristics of indium zinc oxide thin-film transistors (IZO TFTs). In this work, an exponential potential distribution along the depth direction of the active layer is assumed and confirmed by numerical solution of Poisson’s equation followed by device simulation. The interface DOS is obtained as a superposition of constant deep states and exponential tail states. Moreover, it is shown that the bulk DOS may be represented by the superposition of exponential deep states and exponential tail states. The extracted values of bulk DOS and interface DOS are further verified by comparing the measured transfer and output characteristics of IZO TFTs with the simulation results by a 2D device simulator ATLAS (Silvaco). As a result, the proposed extraction method may be useful for diagnosing and characterising metal oxide TFTs since it is fast to extract interface and bulk density of states (DOS) simultaneously. PMID:29534492

  14. 76 FR 59240 - Airworthiness Directives; Empresa Brasileira de Aeronáutica S.A. (EMBRAER) Airplanes

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-09-26

    ... Support, Av. Brig. Faria Lima, 2170, Sao Jose dos Campos-SP, CEP: 12227-901--PO Box: 36/2, Brasil.... Brig. Faria Lima, 2170, Sao Jose dos Campos--SP, CEP: 12227-901--PO Box: 36/2, Brasil; telephone: ++55... dos Campos--SP, CEP: 12227-901--PO Box: 36/2, Brasil; telephone: ++55 12 3927-5383; fax: ++55 12 3927...

  15. Daughter of sevenless is a substrate of the phosphotyrosine phosphatase Corkscrew and functions during sevenless signaling.

    PubMed

    Herbst, R; Carroll, P M; Allard, J D; Schilling, J; Raabe, T; Simon, M A

    1996-06-14

    The SH2 domain-containing phosphotyrosine phosphatase Corkscrew (CSW) is an essential component of the signaling pathway initiated by the activation of the sevenless receptor tyrosine kinase (SEV) during Drosophila eye development. We have used genetic and biochemical approaches to identify a substrate for CSW. Expression of a catalytically inactive CSW was used to trap CSW in a complex with a 115 kDa tyrosine-phosphorylated substrate. This substrate was purified and identified as the product of the daughter of sevenless (dos) gene. Mutations of dos were identified in a screen for dominant mutations which enhance the phenotype caused by overexpression of inactive CSW during photoreceptor development. Analysis of dos mutations indicates that DOS is a positive component of the SEV signaling pathway and suggests that DOS dephosphorylation by CSW may be a key event during signaling by SEV.

  16. Calculating the trap density of states in organic field-effect transistors from experiment: A comparison of different methods

    NASA Astrophysics Data System (ADS)

    Kalb, Wolfgang L.; Batlogg, Bertram

    2010-01-01

    The spectral density of localized states in the band gap of pentacene (trap DOS) was determined with a pentacene-based thin-film transistor from measurements of the temperature dependence and gate-voltage dependence of the contact-corrected field-effect conductivity. Several analytical methods to calculate the trap DOS from the measured data were used to clarify, if the different methods lead to comparable results. We also used computer simulations to further test the results from the analytical methods. Most methods predict a trap DOS close to the valence-band edge that can be very well approximated by a single exponential function with a slope in the range of 50-60 meV and a trap density at the valence-band edge of ≈2×1021eV-1cm-3 . Interestingly, the trap DOS is always slightly steeper than exponential. An important finding is that the choice of the method to calculate the trap DOS from the measured data can have a considerable effect on the final result. We identify two specific simplifying assumptions that lead to significant errors in the trap DOS. The temperature dependence of the band mobility should generally not be neglected. Moreover, the assumption of a constant effective accumulation-layer thickness leads to a significant underestimation of the slope of the trap DOS.

  17. Thin films for gas sensors

    NASA Astrophysics Data System (ADS)

    Pires, Jose Miguel Alves Correia

    Nos ultimos anos tem-se assistido a um aumento dos investimentos na investigacao de novos materiais para aplicacao em sensores. Apesar de ja existir um bom numero de dispositivos explorados comercialmente, muitas vezes, quer devido aos elevados custos de producao, quer devido a uma crescente exigencia do ponto de vista das caracteristicas de funcionamento, continua a ser necessario procurar novos materiais ou novas formas de producao que permitam baixar os custos e melhorar o desempenho dos dispositivos. No campo dos sensores de gases tem-se verificado continuos avancos nos ultimos anos. Continua todavia a ser necessario conhecer melhor, tanto os processos de producao dos materiais, como os mecanismos que regulam a sensibilidade dos dispositivos aos gases, de modo a orientar adequadamente a investigacao dos novos materiais, nomeadamente no que se refere a optimizacao dos parâmetros que nao satisfazem ainda os requisitos do mercado. Um dos materiais que tem mostrado melhores qualidades para aplicacao em sensores de gases de tipo resistivo e o dioxido de estanho. Este material tem sido produzido sob diversas formas e usando diferentes tecnicas, como sejam: sol-gel [1], pulverizacao catodica (sputtering) por magnetrao [2-4], sinterizacao de pos [5, 6], ablacao laser [7] ou RGTO [8]. Os resultados obtidos revelam que as caracteristicas dos dispositivos sao muito dependentes das tecnicas usadas na sua producao. A deposicao usando sputtering reactivo por magnetrao e uma tecnica que permite obter filmes finos de oxido de estanho com diferentes caracteristicas, quer do ponto de vista da estrutura, quer da composicao, e por isso, tambem, com diferentes sensibilidades aos gases. No âmbito deste trabalho, foram produzidos filmes de SnO2 usando sputtering DC reactivo com diferentes condicoes de deposicao. Os substratos usados foram lâminas de vidro e o alvo foi estanho com 99.9% de pureza. Foi estudada a influencia da atmosfera de deposicao, da pressao parcial do O2, da

  18. Comment on “Dissolved organic sulfur in the ocean: Biogeochemistry of a petagram inventory”

    NASA Astrophysics Data System (ADS)

    Dittmar, Thorsten; Stubbins, Aron; Ito, Takamitsu; Jones, Daniel C.

    2017-05-01

    Ksionzek et al. (Reports, 28 October 2016, p. 456) provide important data describing the distribution of dissolved organic sulfur (DOS) in the Atlantic Ocean. Here, we show that mixing between water masses is sufficient to explain the observed distribution of DOS, concluding that the turnover time of refractory DOS that Ksionzek et al. present cannot be deduced from their data.

  19. JPRS Report, West Europe, Reference Aid, Acronyms and Abbreviations of Portugal

    DTIC Science & Technology

    1988-09-02

    Internacional, Ltd. AML Auto Metralhadora Ligeira AN Assembleia Nacional ANA Aeroportos e Navegacao Aerea ANASD ^ Associacao Nacional dos...de Informacoes ANIC ^ Associacao Nacional dos Industriais de Conservas ANIMEE Associac’ao Nacional dos Industriais de Material Electrico e...Produtos Electricos SIPIP ^ Servico de Informacao Previa ao Investidor Potencial SIRP ^ Sistema de Informac’oes da Repüblica Portuguesa SIS

  20. Establishing the Empirical Relationship Between Non-Science Majoring Undergraduate Learners' Spatial Thinking Skills and Their Conceptual Astronomy Knowledge. (Spanish Title: Estableciendo Una Relación Empírica Entre el Razonamiento Espacial de los Estudiantes de Graduación de Carreras no Científicas y su Conocimento Conceptual de la Astronomía.) Estabelecendo Uma Relação Empírica Entre o RacioCínio Espacial dos Estudantes de Graduação EM Carreiras Não Científicas e Seu Conhecimento Conceitual da Astronomia

    NASA Astrophysics Data System (ADS)

    Heyer, Inge; Slater, Stephanie J.; Slater, Timothy F.

    2013-12-01

    The astronomy education community has tacitly assumed that learning astronomy is a conceptual domain resting upon spatial thinking skills. As a first step to formally identify an empirical relationship, undergraduate students in a non-major introductory astronomy survey class at a mediumsized, Ph.D. granting, mid-western US university were given pre- and post-astronomy conceptual diagnostics and spatial reasoning diagnostics, Instruments used were the "Test Of Astronomy Standards" and "What Do You Know?" Using only fully matched data for analysis, our sample consisted of 86 undergraduate non-science majors. Students' normalized gains for astronomy surveys were low at .26 and .13 respectively. Students' spatial thinking was measured using an instrument designed specifically for this study. Correlations between the astronomy instruments' pre- to post-course gain scores and the spatial assessment instrument show moderate to strong relationships suggesting the relationship between spatial reasoning and astronomy ability can explain about 25% of the variation in student achievement. La comunidad de educación en astronomía ha supuesto de forma tácita que el aprendizaje de la astronomía consiste en un dominio conceptual fundamentado en el razonamiento espacial. Como un primer paso para identificar formalmente una relación empírica entre estas dos cosas, utilizamos como muestra los estudantes de graduación de carreras no científicas de un curso experimental en una universidad norteamericana del medioeste de porte mediano con programa de Doctorado em curso, en el cual estos estudiantes se sometieron a un diagnóstico de razonamiento espacial y conceptos astronómicos antes e después del mismo. Las herramientas utilizadas fueron el Test Of Astronomy Standards (TOAST) y el cuestionario What do you know? Utilizando solo los datos completamente consistentes para este análisis, nuestra muestra consistió en 86 estudantes de graduación. Las mejoras, depués de

  1. Privileged structures: efficient chemical "navigators" toward unexplored biologically relevant chemical spaces.

    PubMed

    Kim, Jonghoon; Kim, Heejun; Park, Seung Bum

    2014-10-22

    In the search for new therapeutic agents for currently incurable diseases, attention has turned to traditionally "undruggable" targets, and collections of drug-like small molecules with high diversity and quality have become a prerequisite for new breakthroughs. To generate such collections, the diversity-oriented synthesis (DOS) strategy was developed, which aims to populate new chemical space with drug-like compounds containing a high degree of molecular diversity. The resulting DOS-derived libraries have been of great value for the discovery of various bioactive small molecules and therapeutic agents, and thus DOS has emerged as an essential tool in chemical biology and drug discovery. However, the key challenge has become how to design and synthesize drug-like small-molecule libraries with improved biological relevancy as well as maximum molecular diversity. This Perspective presents the development of privileged substructure-based DOS (pDOS), an efficient strategy for the construction of polyheterocyclic compound libraries with high biological relevancy. We envisioned the specific interaction of drug-like small molecules with certain biopolymers via the incorporation of privileged substructures into polyheterocyclic core skeletons. The importance of privileged substructures such as benzopyran, pyrimidine, and oxopiperazine in rigid skeletons was clearly demonstrated through the discovery of bioactive small molecules and the subsequent identification of appropriate target biomolecule using a method called "fluorescence difference in two-dimensional gel electrophoresis". Focusing on examples of pDOS-derived bioactive compounds with exceptional specificity, we discuss the capability of privileged structures to serve as chemical "navigators" toward biologically relevant chemical spaces. We also provide an outlook on chemical biology research and drug discovery using biologically relevant compound libraries constructed by pDOS, biology-oriented synthesis, or

  2. A visual and semi-quantitative assessment of (99m)Tc-EDDA/HYNIC-TOC scintigraphy in differentiation of solitary pulmonary nodules.

    PubMed

    Płachcińska, Anna; Mikołajczak, Renata; Kozak, Józef; Rzeszutek, Katarzyna; Kuśmierek, Jacek

    2004-01-01

    The aim of the study was the assessment of the clinical usefulness of scintigraphy with (99m)Tc-EDDA/HYNIC-TOC for purposes of a differential diagnosis of SPNs by means of a visual inspection and semi-quantitative assessment of uptake intensity of the radiopharmaceutical (RPh). In 53 patients (32 males and 21 females at the ages between 38 and 78 years, mean value 57) with SPN on chest radiographs or CT scans, of diameters from 1 to 5.5 (mean 2.3) cm a SPECT acquisition was performed, 2-4 h after administration of 740 MBq of RPh. Additionally, aiming at the implementation of a correction of a partial volume effect resulting from finite resolution of this technique, the measurement of the resolution of this technique was performed on an thorax phantom. Scintigraphic studies were inspected visually visually and semi-quantitatively, restoring real concentration of the RPh in nodules in comparison with the peritumoral background (tumour-to-background ratio) by the application of resolution recovery coefficients for the respective nodule diameters. The threshold values of tumour-to-background ratio providing optimal differentiation between malignant and benign nodules of sizes smaller and larger than 2 cm in diameter were determined. Verification of scintigraphic results was based on pathological examinations of tumour samples (histopathology or cytology) and in some cases on bacteriological studies. The additional criterion of tumour benignity was accepted, based on its stable size in a time interval no shorter than 3 years. In 32 patients the following malignant tumours were diagnosed: 12 adenocarcinomas, 6 squamous cell carcinomas, 6 non-small cell lung cancers of unspecified more detailed morphology, 2 large cell carcinomas, 2 small cell lung cancers, 2 carcinoids and 2 metastatic lesions (malignant melanoma and leiomyosarcoma). In 21 patients benign etiologies were found: 6 tuberculomas, 2 other granuloma, 4 hamartomas, 2 non-specific inflammatory infiltrate, 1

  3. Pepita Talks Twice = Pepita habla dos veces.

    ERIC Educational Resources Information Center

    Lachtman, Ofelia Dumas

    This illustrated bilingual children's book (English and Spanish), relates the story of Pepita, a young Hispanic girl who is fluent in both Spanish and English. Pepita always helps when asked to talk for others in Spanish and English. But one day Pepita decides she doesn't want to help anymore. She wants to hurry and get home from school before her…

  4. Semiconducting molecular crystals: Bulk in-gap states modified by structural and chemical defects

    NASA Astrophysics Data System (ADS)

    Haas, S.; Krellner, C.; Goldmann, C.; Pernstich, K. P.; Gundlach, D. J.; Batlogg, B.

    2007-03-01

    Charge transport in organic molecular crystals is strongly influenced by the density of localized in-gap states (traps). Thus, a profound knowledge of the defect states' origin is essential. Temperature-dependent space-charge limited current (TD-SCLC) spectroscopy was used as a powerful tool to quantitatively study the density of states (DOS) in high-quality rubrene and pentacene single crystals. In particular, changes of the DOS due to intentionally induced chemical and structural defects were monitored. For instance, the controlled exposure of pentacene and rubrene to x-ray radiation results in a broad over-all increase of the DOS. Namely, the ionizing radiation induces a variety of both chemical and structural defects. On the other hand, exposure of rubrene to UV-excited oxygen is reflected in a sharp peak in the DOS, whereas in a similar experiment with pentacene oxygen acts as a dopant, and possible defects are metastable on the time-scale of the measurement, thus leaving the extracted DOS virtually unchanged.

  5. Death obsession in Palestinians.

    PubMed

    Abdel-Khalek, Ahmed M; Al-Arja, Nahida S; Abdalla, Taysir

    2006-04-01

    The authors explored death obsession level and correlates among a sample (N = 601) of Palestinians living in the city of Beit Jala, the village of Al-Khader, and the Aida refugee camp in the Bethlehem area. They live in war conditions; the houses of half of them have been demolished. The Death Obsession Scale (DOS) was administered. Its alpha reliability was .92, denoting high internal consistency. Among women, it yielded 1 factor, (General Death Obsession), whereas among men it yielded 3 factors: Death Rumination, Death Dominance, and Death Idea Repetition. Palestinian men and women attained significantly lower DOS mean scores than participants from 4 Arab countries: Egypt, Kuwait, Syria, and Lebanon in 7 out of 8 comparisons. However, Palestinian women had significantly higher DOS mean score than their Spanish, American and British counterparts, whereas Palestinian men had significantly higher mean DOS score than Spanish peers. The low DOS scores of Palestinians, in proportion to other Arab samples, may reflect their adaptation to strife and violence.

  6. Aging Degradation of Austenitic Stainless Steel Weld Probed by Electrochemical Method and Impact Toughness Evaluation

    NASA Astrophysics Data System (ADS)

    Singh, Raghuvir; Das, Goutam; Mahato, B.; Singh, P. K.

    2017-03-01

    The present study discriminates the spinodal decomposition and G-phase precipitation in stainless steel welds by double loop electrochemical potentio-kinetic reactivation method and correlates it with the degradation in toughness property. The welds produced with different heat inputs were aged up to 10,000 hours at 673 K to 723 K (400 to 450 °C) and evaluated subsequently for the degree of sensitization (DOS) and impact toughness. The DOS values obtained were attributed to the spinodal decomposition and precipitation of G-phase. Study shows that the DOS correlates well with the impact toughness of the 304LN weld. Prolonged aging at 673 K and 723 K (400 °C and 450 °C) increased the DOS values while the impact toughness was decreased. The weld fabricated at 1 kJ/mm of heat input, produced higher DOS, compared to that at 3 kJ/mm. The geometrical location along the weld is shown to influence the DOS; higher values were obtained at the root than at the topside of the weld. Vermicular and columnar microstructure, in addition to the spinodal decomposition and G-phase precipitation, observed in the root side of the weld appear risky for the impact toughness.

  7. The Brazilian Malaria Vector Anopheles (Kerteszia) Cruzii: Life Stages and Biology (Diptera: Culicidae)

    DTIC Science & Technology

    1991-11-01

    Mosquitos no litoral paranaense. I - Idade fisioldgica de no Parque National da Serra dos Orgaos, Anopheles cruzii (Diptera, Culicidae). Arq. Estado do...no Parque National da Peryassii, A.G. 1908. OS culicideos do Brazil. Serra dos Grgaos, Estado do Rio de Janeiro, Inst. de Manguinhos, Rio de Janeiro...Kerteszia no litoral Guimar%es, A.E. and V.N.M. Victoria. 1986. do estado de Santa Catarina. Rev. Bras. Mosquitos no Parque National da Serra dos

  8. Development of Less Toxic Treatment Strategies for Metastatic and Drug-Resistant Breast Cancer Using Noninvasive Optical Monitoring

    DTIC Science & Technology

    2017-09-01

    models has been evaluated, with one good option, the Py230 cell lines, as our choice for use in future studies . We have conducted the first study ... Study of Progressive Resistance Major Task 6: dDOS fabrication Subtask 15: Design /Fabricate dDOS system and new custom dDOS probe 6-24 Dr...until year 3 of the study , based on current accrual trends with our clinical collaborators at the Boston Medical Center for different projects, the

  9. Familial occurrence of thymoma and autoimmune diseases with the constitutional translocation t(14;20)(q24.1;p12.3).

    PubMed

    Nicodème, Frédéric; Geffroy, Sandrine; Conti, Massimo; Delobel, Bruno; Soenen, Valérie; Grardel, Nathalie; Porte, Henri; Copin, Marie-Christine; Laï, Jean-Luc; Andrieux, Joris

    2005-10-01

    Thymomas are low-grade epithelial cancers of the thymus whose prevalence varies between 0.1/100,000 and 0.4/100,000. Familial occurrence of thymoma is very rare. We studied a family bearing the constitutional chromosome translocation t(14;20)(q24;p12), 3 of whose members had a thymoma. In this family, among 27 patients, 11 had the translocation: 3 had thymoma and 4 others had 5 different autoimmune diseases: type 1 diabetes mellitus, Graves' disease, pernicious anemia, primitive Sjögren disease, and autoimmune pancytopenia. FISH studies allowed us to be more specific about the translocation breakpoints. The 14q24 breakpoint was in intron 5 of RAD51L1, and the 20p12 breakpoint was 100 kb telomeric to BMP2. RAD51L1 is a tumor-suppressor gene belonging to the RAD51 family, already implicated in many tumors (uterine leiomyomas, pseudo-Meigs syndromes, pulmonary chondroid hamartomas) and involved in recombinational repair of DNA double-strand breaks. BMP2 belongs to the TGFbeta superfamily, and the BMP2-BMP4 genes are involved in thymocyte differentiation by blocking progression from CD4-CD8- to CD4+CD8+ while maintaining a sufficient pool of immature precursors. Dysregulation of RAD51L1 and/or BMP2 may explain this familial occurrence of thymomas and autoimmune diseases. Using QRT-PCR, we studied the expression of BMP2 in 20 sporadic thymomas and found various levels of expression that may be associated with autoimmune diseases.

  10. Neuromuscular choristoma: characteristic magnetic resonance imaging findings and association with post-biopsy fibromatosis.

    PubMed

    Niederhauser, Blake D; Spinner, Robert J; Jentoft, Mark E; Everist, Brian M; Matsumoto, Jane M; Amrami, Kimberly K

    2013-04-01

    To describe imaging characteristics of neuromuscular choristomas (NMC) and to differentiate them from fibrolipomatous hamartomas (FLH). Clinical and imaging characteristics of six patients with biopsy-proven NMC and six patients with FLH were reviewed by musculoskeletal, a pediatric, and two in-training radiologists with a literature review to define typical magnetic resonance imaging features by consensus. Five radiology trainees blinded to cases and naive to the diagnosis of NMC and a musculoskeletal-trained radiologist rated each lesion as having more than or less than 50% intralesional fat, as well as an overall impression using axial T1 images. Sensitivity, specificity, accuracy, and interobserver agreement kappa were determined. Typical features of NMC include smoothly tapering, fusiform enlargement of the sciatic nerve or brachial plexus elements with T1 and T2 signal characteristics closely following those of muscle. Longitudinal bands of intervening low T1 and T2 signal were often present and likely corresponded to fibrous tissue by pathology. Four of five patients with long-term follow-up (80%) developed aggressive fibromatosis after percutaneous or surgical biopsy. Nerve fascicle thickening often resulted in a "coaxial cable" appearance similar to classic FLH, however, using a cutoff of <50% intralesional fat allowed for differentiation with 100% sensitivity by all reviewers and 100% specificity when all imaging features were utilized for impressions. Agreement was excellent with all differentiating methods (kappa 0.861-1.0). NMC can be confidently differentiated from FLH and malignancies using characteristic imaging and clinical features. When a diagnosis is made, biopsy should be avoided given frequent complication by aggressive fibromatosis.

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

    PubMed

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

    2015-04-02

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

  12. Somatic gain-of-function mutations in PIK3CA in patients with macrodactyly.

    PubMed

    Rios, Jonathan J; Paria, Nandina; Burns, Dennis K; Israel, Bonnie A; Cornelia, Reuel; Wise, Carol A; Ezaki, Marybeth

    2013-02-01

    Macrodactyly is a discrete congenital anomaly consisting of enlargement of all tissues localized to the terminal portions of a limb, typically within a 'nerve territory'. The classic terminology for this condition is 'lipofibromatous hamartoma of nerve' or Type I macrodactyly. The peripheral nerve, itself, is enlarged both in circumference and in length. It is not related to neurofibromatosis (NF1), nor is it associated with vascular malformations, such as in the recently reported CLOVES syndrome. The specific nerve pathophysiology in this form of macrodactyly has not been well described and a genetic etiology for this specific form of enlargement is unknown. To identify the genetic cause of macrodactyly, we used whole-exome sequencing to identify somatic mutations present in the affected nerve of a single patient. We confirmed a novel mutation in PIK3CA (R115P) present in the patient's affected nerve tissue but not in blood DNA. Sequencing PIK3CA exons identified gain-of-function mutations (E542K, H1047L or H1047R) in the affected tissue of five additional unrelated patients; mutations were absent in blood DNA available from three patients. Immunocytochemistry confirmed AKT activation in cultured cells from the nerve of a macrodactyly patient. Additionally, we found that the most abnormal structure within the involved nerve in a macrodactylous digit is the perineurium, with additional secondary effects on the axon number and size. Thus, isolated congenital macrodactyly is caused by somatic activation of the PI3K/AKT cell-signaling pathway and is genetically and biochemically related to other overgrowth syndromes.

  13. Smooth muscle tumors of soft tissue and non-uterine viscera: biology and prognosis.

    PubMed

    Miettinen, Markku

    2014-01-01

    Smooth muscle tumors are here considered an essentially dichotomous group composed of benign leiomyomas and malignant leiomyosarcomas. Soft tissue smooth muscle tumors with both atypia and mitotic activity are generally diagnosed leiomyosarcomas acknowledging potential for metastasis. However, lesions exist that cannot be comfortably placed in either category, and in such cases the designation 'smooth muscle tumor of uncertain biologic potential' is appropriate. The use of this category is often necessary with limited sampling, such as needle core biopsies. Benign smooth muscle tumors include smooth muscle hamartoma and angioleiomyoma. A specific category of leiomyomas are estrogen-receptor positive ones in women. These are similar to uterine leiomyomas and can occur anywhere in the abdomen and abdominal wall. Leiomyosarcomas can occur at any site, although are more frequent in the retroperitoneum and proximal extremities. They are recognized by likeness to smooth muscle cells but can undergo pleomorphic evolution ('dedifferentiation'). Presence of smooth muscle actin is nearly uniform and desmin-positivity usual. This and the lack of KIT expression separate leiomyosarcoma from GIST, an important problem in abdominal soft tissues. EBV-associated smooth muscle tumors are a specific subcategory occurring in AIDS or post-transplant patients. These tumors can have incomplete smooth muscle differentiation but show nuclear EBER as a diagnostic feature. In contrast to many other soft tissue tumors, genetics of smooth muscle tumors are poorly understood and such diagnostic testing is not yet generally applicable in this histogenetic group. Leiomyosarcomas are known to be genetically complex, often showing 'chaotic' karyotypes including aneuploidy or polyploidy, and no recurrent tumor-specific translocations have been detected.

  14. Epilepsy surgery in children: outcomes and complications.

    PubMed

    Kim, Seung-Ki; Wang, Kyu-Chang; Hwang, Yong-Seung; Kim, Ki Joong; Chae, Jong Hee; Kim, In-One; Cho, Byung-Kyu

    2008-04-01

    Ideal epilepsy surgery would eliminate seizures without causing any functional deficits. The aim of the present study was to assess seizure outcomes and complications after epilepsy surgery in children with intractable epilepsy. Data obtained in 134 children (75 boys and 59 girls) age 17 years or younger who underwent epilepsy surgery at Seoul National University Children's Hospital between 1993 and 2005 were retrospectively reviewed. Epilepsy surgery included temporal resection (59 cases), extratemporal resection (56 cases), functional hemispherectomy (7 cases), callosotomy (9 cases), multiple subpial transection (1 case), and disconnection of a hamartoma (2 cases). The mean follow-up duration was 62.3 months (range 12-168 months). The overall seizure-free rate was 69% (93 of 134 cases). The seizure-free rate was significantly higher in children who underwent temporal resection than in those in whom extratemporal resection was performed (88 vs 55%, p < 0.05). The most frequent causes of treatment failure were related to the absence of structural abnormality demonstrated on magnetic resonance imaging, development-associated disease, widespread disease documented by postoperative electroencephalography, and limited resection due to the presence of functional cortex. There were no postoperative deaths. Visual field defects were the most common complication after temporal resection (22% [13 of 59 cases]), whereas hemiparesis (mostly transient) was the most common morbidity after extratemporal resection (18% [10 of 56 cases]). Epilepsy surgery is an effective and safe therapeutic modality in childhood. In children with extratemporal epilepsy, more careful interpretation of clinical and investigative data is needed to achieve favorable seizure outcome.

  15. Clinical management of tuberous sclerosis complex over the lifetime of a patient.

    PubMed

    Frost, Michael; Hulbert, John

    2015-01-01

    Tuberous sclerosis complex (TSC) is a genetic disorder characterized by nonmalignant tumors (hamartomas) that can occur in various organ systems, including the brain, kidneys, lungs, skin, eyes, and heart. Clinical manifestations of TSC can occur at any age, thereby making the diagnosis difficult. No typical disease presentation is known, and the clinical presentation usually differs between pediatric and adult patients. Furthermore, variable penetrance of the genetic mutation causes a range of disease severity from very mild to severe, and affected individuals can go undetected for years because many of the clinical manifestations of TSC lack specificity. Once a diagnosis is made, TSC management strategies should be tailored to address the symptoms and risks most relevant to the age of the patient. Improved understanding of the genetic basis of TSC and of the central issue of mTOR overactivation has led to use of pharmacotherapies such as the mTOR inhibitors everolimus and sirolimus in the treatment of TSC disease. In Phase II and III studies, everolimus has demonstrated efficacy and safety in the treatment of both brain (subependymal giant cell astrocytoma) and renal (angiomyolipoma) manifestations associated with TSC. It is important to bear in mind that TSC is a lifelong condition, and for those diagnosed as children, a continuum of care will be needed as they transition from pediatric to adult health services. Clearly identifying the likely differences among diagnosis, monitoring, and management of pediatric and adult patients with TSC is an important step in enabling efficiencies to be maximized without compromising the care provided to patients.

  16. Clinical management of tuberous sclerosis complex over the lifetime of a patient

    PubMed Central

    Frost, Michael; Hulbert, John

    2015-01-01

    Tuberous sclerosis complex (TSC) is a genetic disorder characterized by nonmalignant tumors (hamartomas) that can occur in various organ systems, including the brain, kidneys, lungs, skin, eyes, and heart. Clinical manifestations of TSC can occur at any age, thereby making the diagnosis difficult. No typical disease presentation is known, and the clinical presentation usually differs between pediatric and adult patients. Furthermore, variable penetrance of the genetic mutation causes a range of disease severity from very mild to severe, and affected individuals can go undetected for years because many of the clinical manifestations of TSC lack specificity. Once a diagnosis is made, TSC management strategies should be tailored to address the symptoms and risks most relevant to the age of the patient. Improved understanding of the genetic basis of TSC and of the central issue of mTOR overactivation has led to use of pharmacotherapies such as the mTOR inhibitors everolimus and sirolimus in the treatment of TSC disease. In Phase II and III studies, everolimus has demonstrated efficacy and safety in the treatment of both brain (subependymal giant cell astrocytoma) and renal (angiomyolipoma) manifestations associated with TSC. It is important to bear in mind that TSC is a lifelong condition, and for those diagnosed as children, a continuum of care will be needed as they transition from pediatric to adult health services. Clearly identifying the likely differences among diagnosis, monitoring, and management of pediatric and adult patients with TSC is an important step in enabling efficiencies to be maximized without compromising the care provided to patients. PMID:29388579

  17. Premature Pubarche before One Year of Age: Distinguishing between Mini-Puberty Variants and Precocious Puberty

    PubMed Central

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

    2015-01-01

    Background The aim of this study was to facilitate the distinction between the benign “mini-puberty of early infancy” and precocious puberty (PP). Material/Methods We compared 59 patients (21 boys and 38 girls) seen for pubic hair development before one year of age diagnosed as mini-puberty to 13 patients (2 boys) in whom pubertal development before one year revealed a PP. Results The boys with mini-puberty presented with pubic hair development and prepubertal testicular volume, with low plasma testosterone concentrations. Their gonadotropin responses to gonadotropin releasing hormone (GnRH) test showed predominant luteinising hormone increase in 9/13. The girls presented with pubic hair development that was accompanied by breast development in 47% of cases, with low plasma estradiol concentrations. Their gonadotropin responses showed predominant follicle-stimulating hormone increase in the 17 evaluated. The patients with PP had organic central PP (5 hypothalamic hamartoma) or idiopathic central PP (n=6), or peripheral PP (one ovarian tumor and one congenital adrenal hyperplasia). The diagnosis was challenging only in 3 girls with idiopathic central PP presenting with prepubertal plasma estradiol concentrations and responses to GnRH test. Conclusions The diagnosis of PP was easily determined based on the clinical presentation and the pubertal concentrations of testosterone in boys or of estradiol in girls, as was the diagnosis of central or peripheral origin of PP based on gonadotropin response to the GnRH test. Once PP is excluded, these patients need careful follow–up and physician consultation is needed if clinical pubertal signs progress. PMID:25832117

  18. Prevalence of benign disease in patients undergoing resection for suspected lung cancer.

    PubMed

    Smith, Michael A; Battafarano, Richard J; Meyers, Bryan F; Zoole, Jennifer Bell; Cooper, Joel D; Patterson, G Alexander

    2006-05-01

    In this era of expanded lung cancer screening, accurate differentiation of benign from malignant lesions remains an important problem. We sought to characterize our experience with focal pulmonary lesions suggestive of lung cancer and subsequently proven benign on surgical resection. A retrospective analysis was performed on 1,560 patients who underwent resection for focal pulmonary lesions at our institution from January 1995 to December 2002. Computed tomography and pathology reports were reviewed for all patients. Fluorine-18-fluorodeoxyglucose positron emission tomography studies were performed on 43 patients. Benign processes were found on pathologic examination in 140 patients (9%). Resection was accomplished by thoracotomy in 103 patients (74%), video-assisted thoracoscopy in 36 patients (26%), and sternotomy in 1 patient (0.7%). Seventy patients (50%) underwent mediastinoscopy before resection. There was 1 (0.7%) perioperative death. Pathologic diagnoses from the pulmonary resections revealed granulomatous inflammation in 91 patients (65%), hamartoma in 17 patients (12%), pneumonia or pneumonitis in 14 patients (10%), fibrosis in 5 patients (4%), and other in 13 patients (9%). Fluorine-18-fluorodeoxyglucose positron emission tomography imaging suggested malignancy in 22 of 43 patients and benign lesion in 20 of 43 patients (1 study was not interpretable). Thirty-eight patients underwent needle biopsy before surgery. Of these, 29 samples were nondiagnostic, 5 samples were negative, and 4 samples were considered positive for malignancy. Despite thorough clinical assessment, advanced imaging technology, and needle biopsy, many patients continue to undergo surgery for benign disease. Aggressive attempts to diagnose and treat early stage lung cancer must be tempered with this understanding.

  19. Superconductivity in Bismuth. A New Look at an Old Problem

    PubMed Central

    2016-01-01

    To investigate the relationship between atomic topology, vibrational and electronic properties and superconductivity of bismuth, a 216-atom amorphous structure (a-Bi216) was computer-generated using our undermelt-quench approach. Its pair distribution function compares well with experiment. The calculated electronic and vibrational densities of states (eDOS and vDOS, respectively) show that the amorphous eDOS is about 4 times the crystalline at the Fermi energy, whereas for the vDOS the energy range of the amorphous is roughly the same as the crystalline but the shapes are quite different. A simple BCS estimate of the possible crystalline superconducting transition temperature gives an upper limit of 1.3 mK. The e-ph coupling is more preponderant in a-Bi than in crystalline bismuth (x-Bi) as indicated by the λ obtained via McMillan’s formula, λc = 0.24 and experiment λa = 2.46. Therefore with respect to x-Bi, superconductivity in a-Bi is enhanced by the higher values of λ and of eDOS at the Fermi energy. PMID:26815431

  20. Superconductivity in Bismuth. A New Look at an Old Problem.

    PubMed

    Mata-Pinzón, Zaahel; Valladares, Ariel A; Valladares, Renela M; Valladares, Alexander

    2016-01-01

    To investigate the relationship between atomic topology, vibrational and electronic properties and superconductivity of bismuth, a 216-atom amorphous structure (a-Bi216) was computer-generated using our undermelt-quench approach. Its pair distribution function compares well with experiment. The calculated electronic and vibrational densities of states (eDOS and vDOS, respectively) show that the amorphous eDOS is about 4 times the crystalline at the Fermi energy, whereas for the vDOS the energy range of the amorphous is roughly the same as the crystalline but the shapes are quite different. A simple BCS estimate of the possible crystalline superconducting transition temperature gives an upper limit of 1.3 mK. The e-ph coupling is more preponderant in a-Bi than in crystalline bismuth (x-Bi) as indicated by the λ obtained via McMillan's formula, λc = 0.24 and experiment λa = 2.46. Therefore with respect to x-Bi, superconductivity in a-Bi is enhanced by the higher values of λ and of eDOS at the Fermi energy.

  1. Are All-Solid-State Lithium-Ion Batteries Really Safe?-Verification by Differential Scanning Calorimetry with an All-Inclusive Microcell.

    PubMed

    Inoue, Takao; Mukai, Kazuhiko

    2017-01-18

    Although all-solid-state lithium-ion batteries (ALIBs) have been believed as the ultimate safe battery, their true character has been an enigma so far. In this paper, we developed an all-inclusive-microcell (AIM) for differential scanning calorimetry (DSC) analysis to clarify the degree of safety (DOS) of ALIBs. Here AIM possesses all the battery components to work as a battery by itself, and DOS is determined by the total heat generation ratio (ΔH) of ALIB compared with the conventional LIB. When DOS = 100%, the safety of ALIB is exactly the same as that of LIB; when DOS = 0%, ALIB reaches the ultimate safety. We investigated two types of LIB-AIM and three types of ALIB-AIM. Surprisingly, all the ALIBs exhibit one or two exothermic peaks above 250 °C with 20-30% of DOS. The exothermic peak is attributed to the reaction between the released oxygen from the positive electrode and the Li metal in the negative electrode. Hence, ALIBs are found to be flammable as in the case of LIBs. We also attempted to improve the safety of ALIBs and succeeded in decreasing the DOS down to ∼16% by incorporating Ketjenblack into the positive electrode as an oxygen scavenger. Based on ΔH as a function of voltage window, a safety map for LIBs and ALIBs is proposed.

  2. Combination of broadband diffuse optical spectroscopy with magnetic resonance imaging

    NASA Astrophysics Data System (ADS)

    Merritt, Sean Isaiah

    Broadband diffuse optical spectroscopy (DOS) is an emerging optical technique used to measure absorption and scattering of bulk tissue non-invasively within the near-infrared (600--1050 nm). The ultimate aim of my advisors group is for broadband DOS to become an established medical diagnostic technique used clinically on various tissue types including breast, muscle and bone. The specific goal for my research is to use established magnetic resonance (MR) techniques for the purpose of continued development and validation of broadband DOS. The initial studies carried out were a validation of broadband DOS through a direct comparison with MRI. Both techniques are sensitive to signals produced by water and lipids in tissue. There is also sensitivity to blood flow, which MRI measures using exogenous contrast agents and broadband DOS is sensitive through measurement of total hemoglobin content (THC) and tissue oxygen saturation (StO2). These validation studies were compared initially in a rat tumor model in which both techniques were used simultaneously. A qualitative correlation was found between the MR images of water content and blood perfusion compared with the DOS water and THC values. A more quantitative comparison was made between measuring absolute water and lipid content in phantoms and in human tissue, which showed a strong correlation. The in vivo study also validated that broadband DOS was interrogating bone marrow in the tibia. The second half of this thesis is focused on developing new capabilities of broadband DOS and the MRI literature is used as a guide. When a water molecule hydrogen bonds to another molecule, the absorption spectrum in the near-infrared which is due to the vibrational overtone of the OH bond will change. The expected changes were observed in tissue and an algorithm was developed to fit for a tissue bound water parameter. Also, as tissue temperature changes, the fraction of water bound to other water molecules changes and can be used to

  3. pH dependence of cyanide binding to the ferric heme domain of the direct oxygen sensor from Escherichia coli and the effect of alkaline denaturation.

    PubMed

    Bidwai, Anil K; Ok, Esther Y; Erman, James E

    2008-09-30

    The spectrum of the ferric heme domain of the direct oxygen sensor protein from Escherichia coli ( EcDosH) has been measured between pH 3.0 and 12.6. EcDosH undergoes acid denaturation with an apparent p K a of 4.24 +/- 0.05 and a Hill coefficient of 3.1 +/- 0.6 and reversible alkaline denaturation with a p K a of 9.86 +/- 0.04 and a Hill coefficient of 1.1 +/- 0.1. Cyanide binding to EcDosH has been investigated between pH 4 and 11. The EcDosH-cyanide complex is most stable at pH 9 with a K D of 0.29 +/- 0.06 microM. The kinetics of cyanide binding are monophasic between pH 4 and 8. At pH >or=8.5, the reaction is biphasic with the fast phase dependent upon the cyanide concentration and the slow phase independent of cyanide. The slow phase is attributed to conversion of denatured EcDosH to the native state, with a pH-independent rate of 0.052 +/- 0.006 s (-1). The apparent association rate constant for cyanide binding to EcDosH increases from 3.6 +/- 0.1 M (-1) s (-1) at pH 4 to 520 +/- 20 M (-1) s (-1) at pH 11. The dissociation rate constant averages (8.6 +/- 1.3) x 10 (-5) s (-1) between pH 5 and 9, increasing to (1.4 +/- 0.1) x 10 (-3) s (-1) at pH 4 and (2.5 +/- 0.1) x 10 (-3) s (-1) at pH 12.2. The mechanism of cyanide binding is consistent with preferential binding of the cyanide anion to native EcDosH. The reactions of imidazole and H 2O 2 with ferric EcDosH were also investigated and show little reactivity.

  4. 78 FR 67018 - Airworthiness Directives; Embraer S.A. Airplanes

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-11-08

    ... dos Campos, Sao Paulo, Brasil; phone: (+55 12) 3927-1000; Fax: (+55 12) 3927-6600, Ext. 1448; email..., CEP: 12227-901, Sao Jose dos Campos, Sao Paulo, Brasil; phone: (+55 12) 3927-1000; Fax: (+55 12) 3927...

  5. Contribution to the scintillation detection optimization in double phase detectors for direct detection of dark matter

    NASA Astrophysics Data System (ADS)

    Balan, Catalin

    Na ultima decada, foram feitos grandes progressos no desenvolvimento dos detetores de detecao direta das particulas que constituem a materia negra. Com estrategias do aumento gradual do volume do alvo e, simultaneamente, de reducao dos niveis de fundo, a experiencia XENON obteve resultados muito bons e perspetivas promissoras para a detecao de materia negra. Tarefas relativas a analise de dados experimentais adquiridos com o detetor de dupla fase em uso, assim como as simulacoes do campo eletrico, desenvolvimento, montagem e testes para o proximo detetor XENON1T, assim como a participacao regular na manutencao geral e monitorizacao do prototipo atual XENON100 no LNGS, constituiram o plano de trabalhos para as atividades de investigacao do presente doutoramento e a minha contribuicao para a otimizacao da detecao de cintilacao nos detetores da experiencia XENON. A necessidade de alcancar niveis elevados de sensibilidade, requer inovacao em todos os aspetos fisicos do detetor, assim como a reducao de todas as fontes de radioatividade que contribuem para o fundo. O modo mais indicado de operacao para os detetores com enchimento a Xe no estado liquido e gasoso envolve a medicao da cintilacao primaria e da secundaria provenientes da interacao das particulas no Xe liquido. A razao entre estes dois sinais permite diferenciar claramente a maior parte dos eventos correspondentes as fundo dos eventos correspondentes a WIMPs. Deste modo, a leitura dos sinais correspondentes a cintilacao e de extrema importancia. A amplitude do sinal de cintilacao antes dos fotossensores e maximizada atraves da otimizacao de varios parametros, tais como a geometria do alvo do detetor, a transparencia das grelhas dos eletrodos, a uniformidade do ganho em cintilacao secundaria e a utilizacao de material reflectivo para cobrir as superficies que nao sao fotossensiveis.

  6. Production and characterization of a camelid single domain antibody-urease enzyme conjugate for the treatment of cancer.

    PubMed

    Tian, Baomin; Wong, Wah Yau; Hegmann, Elda; Gaspar, Kim; Kumar, Praveen; Chao, Heman

    2015-06-17

    A novel immunoconjugate (L-DOS47) was developed and characterized as a therapeutic agent for tumors expressing CEACAM6. The single domain antibody AFAIKL2, which targets CEACAM6, was expressed in the Escherichia coli BL21 (DE3) pT7-7 system. High purity urease (HPU) was extracted and purified from Jack bean meal. AFAIKL2 was activated using N-succinimidyl [4-iodoacetyl] aminobenzoate (SIAB) as the cross-linker and then conjugated to urease. The activation and conjugation reactions were controlled by altering pH. Under these conditions, the material ratio achieved conjugation ratios of 8-11 antibodies per urease molecule, the residual free urease content was practically negligible (<2%), and high purity (>95%) L-DOS47 conjugate was produced using only ultradiafiltration to remove unreacted antibody and hydrolyzed cross-linker. L-DOS47 was characterized by a panel of analytical techniques including SEC, IEC, Western blot, ELISA, and LC-MS(E) peptide mapping. As the antibody-urease conjugate ratio increased, a higher binding signal was observed. The specificity and cytotoxicity of L-DOS47 was confirmed by screening in four cell lines (BxPC-3, A549, MCF7, and CEACAM6-transfected H23). BxPC-3, a CEACAM6-expressing cell line was found to be most susceptible to L-DOS47. L-DOS47 is being investigated as a potential therapeutic agent in human phase I clinical studies for nonsmall cell lung cancer.

  7. Feasibility of outpatient total hip and knee arthroplasty in unselected patients

    PubMed Central

    Gromov, Kirill; Kjærsgaard-Andersen, Per; Revald, Peter; Kehlet, Henrik; Husted, Henrik

    2017-01-01

    Background and purpose The number of patients who are suitable for outpatient total hip and knee arthroplasty (THA and TKA) in an unselected patient population remains unknown. The purpose of this prospective 2-center study was to identify the number of patients suitable for outpatient THA and TKA in an unselected patient population, to investigate the proportion of patients who were discharged on the day of surgery (DOS), and to identify reasons for not being discharged on the DOS. Patients and methods All consecutive, unselected patients who were referred to 2 participating centers and who were scheduled for primary THA and TKA were screened for eligibility for outpatient surgery with discharge to home on DOS. If patients did not fulfill the discharge criteria, the reasons preventing discharge were noted. Odds factors with relative risk intervals for not being discharged on DOS were identified while adjusting for age, sex, ASA score, BMI and distance to home. Results Of the 557 patients who were referred to the participating surgeons during the study period, 54% were potentially eligible for outpatient surgery. Actual DOS discharge occurred in 13–15% of the 557 patients. Female sex and surgery late in the day increased the odds of not being discharged on the DOS. Interpretation This study shows that even in unselected THA and TKA patients, same-day discharge is feasible in about 15% of patients. Future studies should evaluate safety aspects and economic benefits. PMID:28426262

  8. Experimental and theoretical studies of vibrational density of states in Fe3O4 single-crystalline thin films

    NASA Astrophysics Data System (ADS)

    Handke, B.; Kozłowski, A.; Parliński, K.; Przewoźnik, J.; Ślęzak, T.; Chumakov, A. I.; Niesen, L.; Kąkol, Z.; Korecki, J.

    2005-04-01

    This paper presents experimental and theoretical studies of lattice vibrations in a single-crystalline Fe3O4(001) thin film. The investigations were carried out in order to see how the lattice dynamics changes at the Verwey transition. Vibrational densities of states (DOS) were obtained from nuclear inelastic scattering (NIS) of synchrotron radiation in the temperature range 25 to 296 K, while theoretical DOS were calculated ab initio within density functional theory. Experimental phonon density of states shows good agreement with calculated DOS, reproducing both the general features of main line groups as well as the groups’ structure. This is also in qualitative accord with heat capacity data, provided that experimental DOS is augmented with that calculated for oxygen atoms. We have observed a gradual change in the NIS raw data as well as the relevant DOS while lowering the temperature. In particular, the main peak in the energy region 15-25 meV shows increasing splitting on cooling. The Lamb-Mössbauer factor calculated in the course of DOS evaluation shows a pronounced drop in the vicinity of the Verwey transition that may be partly connected to the observed abrupt lowering of the count rate at approximately 7 meV for T

  9. HEC-RAS 2.2 for backwater and scour analysis - phase one

    DOT National Transportation Integrated Search

    2000-09-01

    The Kansas Department of Transportation (KDOT) and most bridge consultants in Kansas have been using the DOS-WSPRO program and the KDOT scour spreadsheets to perform bridge hydraulics and scour analysis for the past several years. Unfortunately, DOS-...

  10. Effect of O2 plasma treatment on density-of-states in a-IGZO thin film transistors

    NASA Astrophysics Data System (ADS)

    Ding, Xingwei; Huang, Fei; Li, Sheng; Zhang, Jianhua; Jiang, Xueyin; Zhang, Zhilin

    2017-01-01

    This work reports an efficient route for enhancing the performance of amorphous InGaZnO (a-IGZO) thin film transistors (TFT). The mobility was greatly improved by about 38% by means of O2 plasma treatment. Temperature-stress was carried out to investigate the stability and extract the parameters related to activation energy ( E a) and density-of-states (DOS). The DOS was calculated on the basis of the experimentally obtained E a, which can explain the experimental observation. A lower activation energy ( E a, 0.72 eV) and a smaller DOS were obtained in the O2 plasma treatment TFT based on the temperature-dependent transfer curves. The results showed that temperature stability and electrical properties enhancements in a-IGZO thin film transistors were attributed to the smaller DOS. [Figure not available: see fulltext.

  11. Influence of addition of degassed water on bulk nanobubbles.

    PubMed

    Tuziuti, Toru; Yasui, Kyuichi; Kanematsu, Wataru

    2018-05-01

    The effects of the addition of degassed water on bulk nanobubbles (ultrafine bubbles) of air in liquid water were investigated by measuring the volumetric concentration and size distribution at different dissolved air degree of saturation (DOS) values. The proportion of degassed water mixed with water containing bulk nanobubbles was increased to prepare samples having lower DOS values. It was found that the volumetric concentration of nanobubbles mostly decreased and the average nanobubble size became larger as the DOS was decreased. In our proposed mechanism, smaller nanobubbles are selectively dissolved into the surrounding liquid by Laplace pressure due to surface tension as the DOS is reduced. These results demonstrate that stable bulk nanobubbles are present even in water undersaturated with gas. The role of nanobubble under an ultrasound is also discussed. Copyright © 2018 Elsevier B.V. All rights reserved.

  12. Charge Transport Properties in Disordered Organic Semiconductor as a Function of Charge Density: Monte Carlo Simulation

    NASA Astrophysics Data System (ADS)

    Shukri, Seyfan Kelil

    2017-01-01

    We have done Kinetic Monte Carlo (KMC) simulations to investigate the effect of charge carrier density on the electrical conductivity and carrier mobility in disordered organic semiconductors using a lattice model. The density of state (DOS) of the system are considered to be Gaussian and exponential. Our simulations reveal that the mobility of the charge carrier increases with charge carrier density for both DOSs. In contrast, the mobility of charge carriers decreases as the disorder increases. In addition the shape of the DOS has a significance effect on the charge transport properties as a function of density which are clearly seen. On the other hand, for the same distribution width and at low carrier density, the change occurred on the conductivity and mobility for a Gaussian DOS is more pronounced than that for the exponential DOS.

  13. Magnetic disorder in superconductors: Enhancement by mesoscopic fluctuations

    NASA Astrophysics Data System (ADS)

    Burmistrov, I. S.; Skvortsov, M. A.

    2018-01-01

    We study the density of states (DOS) and the transition temperature Tc in a dirty superconducting film with rare classical magnetic impurities of an arbitrary strength described by the Poissonian statistics. We take into account that the potential disorder is a source of mesoscopic fluctuations of the local DOS, and, consequently, of the effective strength of magnetic impurities. We find that these mesoscopic fluctuations result in a nonzero DOS for all energies in the region of the phase diagram where without this effect the DOS is zero within the standard mean-field theory. This mechanism can be more efficient in filling the mean-field superconducting gap than rare fluctuations of the potential disorder (instantons). Depending on the magnetic impurity strength, the suppression of Tc by spin-flip scattering can be faster or slower than in the standard mean-field theory.

  14. Incorporating structural analysis in a quantum dot Monte-Carlo model

    NASA Astrophysics Data System (ADS)

    Butler, I. M. E.; Li, Wei; Sobhani, S. A.; Babazadeh, N.; Ross, I. M.; Nishi, K.; Takemasa, K.; Sugawara, M.; Peyvast, Negin; Childs, D. T. D.; Hogg, R. A.

    2018-02-01

    We simulate the shape of the density of states (DoS) of the quantum dot (QD) ensemble based upon size information provided by high angle annular dark field scanning transmission electron microscopy (HAADF STEM). We discuss how the capability to determined the QD DoS from micro-structural data allows a MonteCarlo model to be developed to accurately describe the QD gain and spontaneous emission spectra. The QD DoS shape is then studied, with recommendations made via the effect of removing, and enhancing this size inhomogeneity on various QD based devices is explored.

  15. Acoustic Purcell Effect for Enhanced Emission

    NASA Astrophysics Data System (ADS)

    Landi, Maryam; Zhao, Jiajun; Prather, Wayne E.; Wu, Ying; Zhang, Likun

    2018-03-01

    We observe that our experimentally measured emission power enhancement of a speaker inside a previously proposed metacavity agrees with our numerically calculated enhancement of the density of states (DOS) of the source-cavity system. We interpret the agreement by formulating a relation between the emitted sound power and the acoustic DOS. The formulation is an analog to Fermi's golden rule in quantum emission. The formulation complements the radiation impedance theory in traditional acoustics for describing sound emission. Our study bridges the gap between acoustic DOS and the acoustic Purcell effect for sound emission enhancement.

  16. 78 FR 12256 - Airworthiness Directives; Embraer S.A. Airplanes

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-02-22

    .... Brigadeiro Faria Lima, 2170--Putim--12227-901 S[atilde]o Jose dos Campos--SP-- BRASIL; telephone +55 12 3927... Lima, 2170--Putim--12227-901 S[atilde]o Jose dos Campos--SP--BRASIL; telephone +55 12 3927-5852 or +55...

  17. 77 FR 63272 - Airworthiness Directives; Embraer S.A. Airplanes

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-10-16

    ... Faria Lima, 2170--Putim-12227-901 S[atilde]o Jose dos Campos-SP-- BRASIL; telephone +55 12 3927-5852 or... Section (PC 060), Av. Brigadeiro Faria Lima, 2170-Putim-12227-901 S[atilde]o Jose dos Campos-SP-BRASIL...

  18. 77 FR 36224 - Airworthiness Directives; Empresa Brasileira de Aeronautica S.A. (EMBRAER) Airplanes

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-06-18

    ... dos Campos--SP--BRASIL; telephone +55 12 3927-5852 or +55 12 3309-0732; fax +55 12 3927-7546; email.... Brigadeiro Faria Lima, 2170-- Putim--12227-901 S[atilde]o Jose dos Campos--SP--BRASIL; telephone +55 12 3927...

  19. 78 FR 66258 - Airworthiness Directives; Empresa Brasileira de Aeronautica S.A. (EMBRAER) Airplanes

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-11-05

    ..., 2170--Putim--12227-901 S[atilde]o Jose dos Campos--SP--BRASIL; telephone +55 12 3927-5852 or +55 12... Faria Lima, 2170-- Putim--12227-901 S[atilde]o Jose dos Campos--SP--BRASIL; telephone +55 12 3927-5852...

  20. 77 FR 72776 - Airworthiness Directives; Embraer S.A. Airplanes

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-12-06

    .... Brigadeiro Faria Lima, 2170--Putim--12227-901 S[atilde]o Jose dos Campos-SP- BRASIL; telephone +55 12 3927...--Putim--12227-901 S[atilde]o Jose dos Campos--SP--BRASIL; telephone +55 12 3927-5852 or +55 12 3309-0732...

  1. 75 FR 68684 - Airworthiness Directives; Empresa Brasileira de Aeronautica S.A. (EMBRAER) Model EMB-500 Airplanes

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-11-09

    ..., Sao Jose dos Campos--SP, CEP: 12227-901--P.O. Box: 38/2, BRASIL, telephone: ++55 12 3927-5383; fax... Support, Av. Brig. Farina Lima, 2170, Sao Jose dos Campos--SP, CEP: 12227-901--P.O. Box: 38/2, BRASIL...

  2. The Relationship Between Preoperative and Primary Care Blood Pressure Among Veterans Presenting from Home for Surgery. Is There Evidence for Anesthesiologist-Initiated Blood Pressure Referral?

    PubMed Central

    Schonberger, Robert B.; Burg, Matthew M.; Holt, Natalie; Lukens, Carrie L.; Dai, Feng; Brandt, Cynthia

    2011-01-01

    Background American College of Cardiology/American Heart Association guidelines describe the perioperative evaluation as “a unique opportunity to identify patients with hypertension,” however factors such as anticipatory stress or medication noncompliance may induce a bias toward higher blood pressure, leaving clinicians unsure about how to interpret preoperative hypertension. Information describing the relationship between preoperative intake blood pressure and primary care measurements could help anesthesiologists make primary care referrals for improved blood pressure control in an evidence-based fashion. We hypothesized that the preoperative examination provides a useful basis for initiating primary care blood pressure referral. Methods We analyzed retrospective data on 2807 patients who arrived from home for surgery and who were subsequently evaluated within 6 months after surgery in the primary care center of the same institution. After descriptive analysis, we conducted multiple linear regression analysis to identify day-of-surgery (DOS) factors associated with subsequent primary care blood pressure. We calculated the sensitivity, specificity, and positive and negative predictive value of different blood pressure referral thresholds using both a single-measurement and a two-stage screen incorporating recent preoperative and DOS measurements for identifying patients with subsequently elevated primary care blood pressure. Results DOS systolic blood pressure (SBP) was higher than subsequent primary care SBP by a mean bias of 5.5mmHg (95% limits of agreement +43.8 to −32.8). DOS diastolic blood pressure (DBP) was higher than subsequent primary care DBP by a mean bias of 1.5mmHg (95% limits of agreement +13.0 to −10.0). Linear regression of DOS factors explained 19% of the variability in primary care SBP and 29% of the variability in DBP. Accounting for the observed bias, a two-stage SBP referral screen requiring preoperative clinic SBP≥140mmHg and DOS

  3. Face mask ventilation--the dos and don'ts.

    PubMed

    Wood, Fiona E; Morley, Colin J

    2013-12-01

    Face mask ventilation provides respiratory support to newly born or sick infants. It is a challenging technique and difficult to ensure that an appropriate tidal volume is delivered because large and variable leaks occur between the mask and face; airway obstruction may also occur. Technique is more important than the mask shape although the size must appropriately fit the face. The essence of the technique is to roll the mask on to the face from the chin while avoiding the eyes, with a finger and thumb apply a strong even downward pressure to the top of the mask, away from the stem and sloped sides or skirt of the mask, place the other fingers under the jaw and apply a similar upward pressure. Preterm infants require continuous end-expiratory pressure to facilitate lung aeration and maintain lung volume. This is best done with a T-piece device, not a self-inflating or flow-inflating bag. Copyright © 2013 Elsevier Ltd. All rights reserved.

  4. Dos Hermanas Chicanas: Overcoming Barriers to Professional Advancement

    ERIC Educational Resources Information Center

    Prospero, Moises

    2007-01-01

    Women and ethnic minorities face steep barriers to professional advancement, and those who rise to the executive level typically use a variety of strategies to overcome obstacles in their way. This study first reviewed the literature on barriers to professional advancement for women and ethnic minorities and the strategies that they report using…

  5. Los Dos Mundos: Rural Mexican Americans, Another America.

    ERIC Educational Resources Information Center

    Baker, Richard

    This book explores race relations between Mexican Americans and Anglo Americans in "Middlewest," a fictitious name for an actual rural Idaho community with the highest proportion of Mexican Americans in the state. Many Mexican Americans in this predominantly agricultural area are current or former migrant workers. The first chapter…

  6. Dos and Don'ts of Writing Presidential Contracts

    ERIC Educational Resources Information Center

    Fain, Paul

    2006-01-01

    In the not-so-distant past, an employment agreement between a university president and a governing board was often nothing more than a handshake. Today, formal contracts are requirements for presidents, with boards and presidents typically relying on lawyers to extensively document terms of employment, salary and benefits, and, perhaps most…

  7. Web-Based Mathematics: Some "Dos" and "Don'ts"

    ERIC Educational Resources Information Center

    Loong, Esther Yook-Kin

    2011-01-01

    This case study describes an "out of field" teacher's use of the Internet to teach a range of mathematical topics in a modified Year 8 mathematics class. It highlights the importance of three factors for implementing a discernible web-based teaching strategy: appropriate choice of web objects, effective "virtual" pedagogy, and technical support…

  8. 78 FR 33201 - Airworthiness Directives; Embraer S.A. Airplanes

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-06-04

    .... Brigadeiro Faria Lima, 2170-Putim-12227-901 S[atilde]o Jose dos Campos-SP-BRASIL; telephone +55 12 3927-5852...[atilde]o Jose dos Campos-SP-BRASIL; telephone +55 12 3927-5852 or +55 12 3309-0732; fax +55 12 3927-7546...

  9. 77 FR 39159 - Airworthiness Directives; Empresa Brasileria de Aeronáutica S.A. (EMBRAER) Airplanes

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-07-02

    ... Lima, 2170, S[atilde]o Jos[eacute] dos Campos--SP, CEP: 12227-901--PO Box 36/2, BRASIL; fax ++55 12.... Brigadeiro Faria Lima, 2170, S[atilde]o Jos[eacute] dos Campos--SP, CEP: 12227-901--P.O. Box 36/2, BRASIL...

  10. Two regimes in the magnetic field response of superconducting MgB2

    NASA Astrophysics Data System (ADS)

    Kohen, A.; Giubileo, F.; Proslier, Th.; Bobba, F.; Cucolo, A. M.; Sacks, W.; Noat, Y.; Troianovski, A.; Roditchev, D.

    2007-05-01

    Using Scanning Tunneling Microscope at low temperature we explore the superconducting phase diagram in the π-band of the two-band superconductor MgB2. In this band the peculiar shape of the local tunneling spectra and their dynamics in the magnetic field reveal the complex character of the quasiparticle density of states (DOS). The gap in the DOS is first rapidly filled with states in raising the magnetic field up to 0.5 T and then slowly approaches the normal state value: the gap is observed up to 2 T. Such a change in the DOS dynamics suggests the existence of two terms in the DOS of the π-band: a first one, reflecting an intrinsic superconductivity in the band and a second one, originating from an inter-band coupling to the σ-band. Our findings allow a deeper understanding of the unique phase diagram of MgB2.

  11. Dissecting the protein architecture of DNA-binding transcription factors in bacteria and archaea.

    PubMed

    Rivera-Gómez, Nancy; Martínez-Núñez, Mario Alberto; Pastor, Nina; Rodriguez-Vazquez, Katya; Perez-Rueda, Ernesto

    2017-08-01

    Gene regulation at the transcriptional level is a central process in all organisms where DNA-binding transcription factors play a fundamental role. This class of proteins binds specifically at DNA sequences, activating or repressing gene expression as a function of the cell's metabolic status, operator context and ligand-binding status, among other factors, through the DNA-binding domain (DBD). In addition, TFs may contain partner domains (PaDos), which are involved in ligand binding and protein-protein interactions. In this work, we systematically evaluated the distribution, abundance and domain organization of DNA-binding TFs in 799 non-redundant bacterial and archaeal genomes. We found that the distributions of the DBDs and their corresponding PaDos correlated with the size of the genome. We also identified specific combinations between the DBDs and their corresponding PaDos. Within each class of DBDs there are differences in the actual angle formed at the dimerization interface, responding to the presence/absence of ligands and/or crystallization conditions, setting the orientation of the resulting helices and wings facing the DNA. Our results highlight the importance of PaDos as central elements that enhance the diversity of regulatory functions in all bacterial and archaeal organisms, and our results also demonstrate the role of PaDos in sensing diverse signal compounds. The highly specific interactions between DBDs and PaDos observed in this work, together with our structural analysis highlighting the difficulty in predicting both inter-domain geometry and quaternary structure, suggest that these systems appeared once and evolved with diverse duplication events in all the analysed organisms.

  12. Evidence for abiotic sulfurization of marine dissolved organic matter in sulfidic environments

    NASA Astrophysics Data System (ADS)

    Pohlabeln, A. M.; Niggemann, J.; Dittmar, T.

    2016-02-01

    Sedimentary organic matter abiotically sulfurizes in sulfidic marine environments. Here we hypothesize that sulfurization also affects dissolved organic matter (DOM), and that sulfidic marine environments are sources of dissolved organic sulfur (DOS) to the ocean. To test these hypotheses we studied solid-phase extractable (SPE) DOS in the Black Sea at various water column depths (oxic and anoxic) and in sediment porewaters from the German Wadden Sea. The concentration and molecular composition of SPE-DOS from these sites and from the oxic water columns of the North Sea (Germany) and of the North Pacific were compared. In support of our hypotheses, SPE-DOS concentrations were elevated in sulfidic waters compared to oxic waters. For a detailed molecular characterization of SPE-DOS, selective wet-chemical alteration experiments targeting different sulfur-containing functional groups were applied prior to Fourier-transform ion cyclotron resonance mass spectrometry (FT-ICR-MS). These experiments included harsh hydrolysis, selective derivatization of thiols, oxidation, and deoxygenation to test for thioesters, sulfonic acid esters, alkylsulfates, thiols, non-aromatic thioethers, and sulfoxides. Additionally, collision-induced fragmentation experiments were applied to test for sulfonic acids. The tests revealed that the sulfonic acid group was the main structural feature in SPE-DOS, independent of the environmental conditions of the sampling site. Only in Wadden Sea anoxic porewater also non-aromatic thioethers were found which are presumably not stable in oxic waters. The findings from our field studies were confirmed in laboratory experiments, where we abiotically sulfurized marine and algal-derived DOM under conditions similar to that in anoxic marine sediments.

  13. Toward noninvasive detection and monitoring of malaria with broadband diffuse optical spectroscopy

    NASA Astrophysics Data System (ADS)

    Campbell, Chris; Tromberg, Bruce J.; O'Sullivan, Thomas D.

    2018-02-01

    Despite numerous advances, malaria continues to kill nearly half a million people globally every year. New analytical methods and diagnostics are critical to understanding how treatments under development affect the lifecycle of malaria parasites. A biomarker that has been gaining interest is the "malaria pigment" hemozoin. This byproduct of hemoglobin digestion by the parasite has a unique spectral signature but is difficult to differentiate from hemoglobin and other tissue chromophores. Hemozoin can be detected in blood samples, but only utilizing approaches that require specialized training and facilities. Diffuse optical spectroscopy (DOS) is a noninvasive sensing technique that is sensitive to near-infrared absorption and scattering and capable of probing centimeter-deep volumes of tissue in vivo. DOS is relatively low-cost, does not require specialized training and thus potentially suitable for use in low-resource settings. In this work, we assess the potential of DOS to detect and quantify the presence of hemozoin noninvasively and at physiologically relevant levels. We suspended synthetic hemozoin in Intralipid-based tissue-simulating phantoms in order to mimic malaria infection in multiply-scattering tissue. Using a fiber probe that combines frequency-domain and continuous-wave broadband DOS (650-1000 nm), we detected hemozoin concentrations below 250 ng/ml, which corresponds to parasitemia sensitivities comparable to modern rapid diagnostic tests. We used the experimental variability to simulate and estimate the sensitivity of DOS to hemozoin in tissue that includes hemoglobin, water, and lipid under various tissue oxygen saturation levels. The results indicate that with increased precision, it may be possible to detect Hz noninvasively with DOS.

  14. Disk Operating System User's Guide

    DOT National Transportation Integrated Search

    1972-05-01

    This document serves the purpose of bringing together in one place most of the information a user needs to use the DDP-516 Disk Operating System, (DOS). DOS is a core resident, one user, console-oriented operating system which allows the user to cont...

  15. ANÁLISE DA INSERÇÃO DOS TEMAS DE HUMANIDADES E ÉTICA, COM METODOLOGIA DE APRENDIZAGEM BASEADA EM PROBLEMAS, EM CURRICULO MÉDICO INTEGRADO EM ESCOLA PÚBLICA NO DISTRITO FEDERAL, BRASIL

    PubMed Central

    Novaes, Maria Rita Carvalho Garbi; Novaes, Luiz Carlos Garcez; Guilhem, Dirce; Lolas, Fernando; Silveira, Carla; Guiotti, Murilo

    2009-01-01

    Objetivo Realizar uma análise da inserção da ética e humanidades no currículo do Curso de Medicina da Escola Superior em Ciências da Saúde - ESCS, escola pública do Distrito Federal, Brasil, de forma a contribuir com o processo de gestão curricular. Metodologia O Estudo é de coorte e documental. Foram pesquisados 37 termos relacionados à ética e 36 referentes à humanização nos objetivos educacionais e conteúdo dos módulos temáticos, habilidades e atitudes e interação ensino-serviço-comunidade, de 1a a 4a série e no programa do internato no currículo (ano 2006) e no projeto pedagógico do Curso de Medicina (2001). Resultados Maior inserção da humanização, ética e bioética na 1a e 2a série, quando comparado à inserção na 3a e 4a série e no internato, (IC95%-α=0,034, pvalue=0,007). Unidade de habilidades e atitudes: freqüência das 3 temáticas no currículo da 1a a 4a séries (IC95%-α=0,026, pvalue=0,013). Quando comparada a inserção entre o internato e as quatro primeiras séries, observa-se que nestas a inserção da temática humanização é superior (IC95%-α=0,042, pvalue=0,029). Conclusão O currículo desenvolvido no ano de 2006 na ESCS apresentou correlação com o projeto pedagógico do curso e contemplou a temática de forma abrangente, em todas as séries e internato. PMID:20396594

  16. Finite-size Scaling of the Density of States in Photonic Band Gap Crystals

    NASA Astrophysics Data System (ADS)

    Hasan, Shakeeb Bin; Mosk, Allard P.; Vos, Willem L.; Lagendijk, Ad

    2018-06-01

    The famous vanishing of the density of states (DOS) in a band gap, be it photonic or electronic, pertains to the infinite-crystal limit. In contrast, all experiments and device applications refer to finite crystals, which raises the question: Upon increasing the linear size L of a crystal, how fast does the DOS approach the infinite-crystal limit? We present a theory for finite crystals that includes Bloch-mode broadening due to the presence of crystal boundaries. Our results demonstrate that the DOS for frequencies inside a band gap has a 1 /L scale dependence for crystals in one, two and three dimensions.

  17. Extraction of the defect density of states in microcrystalline silicon from experimental results and simulation studies

    NASA Astrophysics Data System (ADS)

    Tibermacine, T.; Merazga, A.; Ledra, M.; Ouhabab, N.

    2015-09-01

    The constant photocurrent method in the ac-mode (ac-CPM) is used to determine the defect density of states (DOS) in hydrogenated microcrystalline silicon (μc-Si:H) prepared by very high frequency plasma-enhanced chemical vapor deposition (VHF-PECVD). The absorption coefficient spectrum (ac-α(hv)), is measured under ac-CPM conditions at 60 Hz. The measured ac-α(hv) is converted by the CPM spectroscopy into a DOS distribution covering a portion in the lower energy range of occupied states. We have found that the density of valence band-tail states falls exponentially towards the gap with a typical band-tail width of 63 meV. Independently, computer simulations of the ac-CPM are developed using a DOS model that is consistent with the measured ac-α(hv) in the present work and a previously measured transient photocurrent (TPC) for the same material. The DOS distribution model suggested by the measurements in the lower and in the upper part of the energy-gap, as well as by the numerical modelling in the middle part of the energy-gap, coincide reasonably well with the real DOS distribution in hydrogenated microcrystalline silicon because the computed ac-α(hv) is found to agree satisfactorily with the measured ac-α(hv).

  18. Intraoperative optical assessment of photodynamic therapy response of superficial oral squamous cell carcinoma

    NASA Astrophysics Data System (ADS)

    Rohrbach, Daniel J.; Rigual, Nestor; Arshad, Hassan; Tracy, Erin C.; Cooper, Michelle T.; Shafirstein, Gal; Wilding, Gregory; Merzianu, Mihai; Baumann, Heinz; Henderson, Barbara W.; Sunar, Ulas

    2016-01-01

    This study investigated whether diffuse optical spectroscopy (DOS) measurements could assess clinical response to photodynamic therapy (PDT) in patients with head and neck squamous cell carcinoma (HNSCC). In addition, the correlation between parameters measured with DOS and the crosslinking of signal transducer and activator of transcription 3 (STAT3), a molecular marker for PDT-induced photoreaction, was investigated. Thirteen patients with early stage HNSCC received the photosensitizer 2-[1-hexyloxyethyl]-2-devinylpyropheophorbide-a (HPPH) and DOS measurements were performed before and after PDT in the operating room (OR). In addition, biopsies were acquired after PDT to assess the STAT3 crosslinking. Parameters measured with DOS, including blood volume fraction, blood oxygen saturation (StO2), HPPH concentration (cHPPH), HPPH fluorescence, and blood flow index (BFI), were compared to the pathologic response and the STAT3 crosslinking. The best individual predictor of pathological response was a change in cHPPH (sensitivity=60%, specificity=100%), while discrimination analysis using a two-parameter classifier (change in cHPPH and change in StO2) classified pathological response with 100% sensitivity and 100% specificity. BFI showed the best correlation with the crosslinking of STAT3. These results indicate that DOS-derived parameters can assess the clinical response in the OR, allowing for earlier reintervention if needed.

  19. Tunnelling spectroscopy of gate-induced superconductivity in MoS2

    NASA Astrophysics Data System (ADS)

    Costanzo, Davide; Zhang, Haijing; Reddy, Bojja Aditya; Berger, Helmuth; Morpurgo, Alberto F.

    2018-06-01

    The ability to gate-induce superconductivity by electrostatic charge accumulation is a recent breakthrough in physics and nanoelectronics. With the exception of LaAlO3/SrTiO3 interfaces, experiments on gate-induced superconductors have been largely confined to resistance measurements, which provide very limited information about the superconducting state. Here, we explore gate-induced superconductivity in MoS2 by performing tunnelling spectroscopy to determine the energy-dependent density of states (DOS) for different levels of electron density n. In the superconducting state, the DOS is strongly suppressed at energy smaller than the gap Δ, which is maximum (Δ 2 meV) for n of 1 × 1014 cm-2 and decreases monotonously for larger n. A perpendicular magnetic field B generates states at E < Δ that fill the gap, but a 20% DOS suppression of superconducting origin unexpectedly persists much above the transport critical field. Conversely, an in-plane field up to 10 T leaves the DOS entirely unchanged. Our measurements exclude that the superconducting state in MoS2 is fully gapped and reveal the presence of a DOS that vanishes linearly with energy, the explanation of which requires going beyond a conventional, purely phonon-driven Bardeen-Cooper-Schrieffer mechanism.

  20. Development and Characterization of a Camelid Single Domain Antibody–Urease Conjugate That Targets Vascular Endothelial Growth Factor Receptor 2

    PubMed Central

    Tian, Baomin; Wong, Wah Yau; Uger, Marni D.; Wisniewski, Pawel; Chao, Heman

    2017-01-01

    Angiogenesis is the process of new blood vessel formation and is essential for a tumor to grow beyond a certain size. Tumors secrete the pro-angiogenic factor vascular endothelial growth factor, which acts upon local endothelial cells by binding to vascular endothelial growth factor receptors (VEGFRs). In this study, we describe the development and characterization of V21-DOS47, an immunoconjugate that targets VEGFR2. V21-DOS47 is composed of a camelid single domain anti-VEGFR2 antibody (V21) and the enzyme urease. The conjugate specifically binds to VEGFR2 and urease converts endogenous urea into ammonia, which is toxic to tumor cells. Previously, we developed a similar antibody–urease conjugate, L-DOS47, which is currently in clinical trials for non-small cell lung cancer. Although V21-DOS47 was designed from parameters learned from the generation of L-DOS47, additional optimization was required to produce V21-DOS47. In this study, we describe the expression and purification of two versions of the V21 antibody: V21H1 and V21H4. Each was conjugated to urease using a different chemical cross-linker. The conjugates were characterized by a panel of analytical techniques, including SDS-PAGE, size exclusion chromatography, Western blotting, and LC-MSE peptide mapping. Binding characteristics were determined by ELISA and flow cytometry assays. To improve the stability of the conjugates at physiologic pH, the pIs of the V21 antibodies were adjusted by adding several amino acid residues to the C-terminus. For V21H4, a terminal cysteine was also added for use in the conjugation chemistry. The modified V21 antibodies were expressed in the E. coli BL21 (DE3) pT7 system. V21H1 was conjugated to urease using the heterobifunctional cross-linker succinimidyl-[(N-maleimidopropionamido)-diethyleneglycol] ester (SM(PEG)2), which targets lysine resides in the antibody. V21H4 was conjugated to urease using the homobifunctional cross-linker, 1,8-bis(maleimido)diethylene glycol (BM

  1. In vivo prediction of goat kids body composition from the deuterium oxide dilution space determined by isotope-ratio mass spectrometry.

    PubMed

    Lerch, S; Lastel, M L; Grandclaudon, C; Brechet, C; Rychen, G; Feidt, C

    2015-09-01

    Deuterium oxide dilution space (DOS) determination is one of the most accurate methods for in vivo estimation of ruminant body composition. However, the time-consuming vacuum sublimation of blood preceding infrared spectroscopy analysis, which is traditionally used to determine deuterium oxide (DO) concentration, limits its current use. The use of isotope-ratio mass spectrometry (IRMS) to determine the deuterium enrichment and thus quantify DO in plasma could counteract this limitation by reducing the sample preparation for plasma deproteinisation through centrifugal filters. The aim of this study was to validate the DOS technique using IRMS in growing goat kids to establish in vivo prediction equations of body composition. Seventeen weaned male Alpine goat kids (8.6 wk old) received a hay-based diet supplemented with 2 types of concentrates providing medium ( = 9) or high ( = 8) energy levels. Kids were slaughtered at 14.0 ( = 1, medium-energy diet), 17.2 ( = 4, medium-energy diet, and = 4, high-energy diet), or 21.2 wk of age ( = 4, medium-energy diet, and = 4, high-energy diet). Two days before slaughter, DOS was determined after an intravenous injection of 0.2 g DO/kg body mass (BM) and the resulting study of DO dilution kinetics from 4 plasma samples (+5, +7, +29, and +31 h after injection). The deuterium enrichment was analyzed by IRMS. After slaughter, the gut contents were discarded, the empty body (EB) was minced, and EB water, lipid, protein, ash, and energy contents were measured by chemical analyses. Prediction equations for body components measured postmortem were computed from in vivo BM and DOS. The lack of postmortem variation of fat-free EB composition was confirmed (mean of 75.3% [SD 0.6] of water), and the proportion of lipids in the EB tended ( = 0.06) to be greater for the high-energy diet (13.1%) than for the medium-energy diet (11.1%). There was a close negative relationship (residual CV [rCV] = 3.9%, = 0.957) between EB water and lipid

  2. Early detection of chemotherapy-refractory patients by monitoring textural alterations in diffuse optical spectroscopic images.

    PubMed

    Sadeghi-Naini, Ali; Vorauer, Eric; Chin, Lee; Falou, Omar; Tran, William T; Wright, Frances C; Gandhi, Sonal; Yaffe, Martin J; Czarnota, Gregory J

    2015-11-01

    Changes in textural characteristics of diffuse optical spectroscopic (DOS) functional images, accompanied by alterations in their mean values, are demonstrated here for the first time as early surrogates of ultimate treatment response in locally advanced breast cancer (LABC) patients receiving neoadjuvant chemotherapy (NAC). NAC, as a standard component of treatment for LABC patient, induces measurable heterogeneous changes in tumor metabolism which were evaluated using DOS-based metabolic maps. This study characterizes such inhomogeneous nature of response development, by determining alterations in textural properties of DOS images apparent at early stages of therapy, followed later by gross changes in mean values of these functional metabolic maps. Twelve LABC patients undergoing NAC were scanned before and at four times after treatment initiation, and tomographic DOS images were reconstructed at each time. Ultimate responses of patients were determined clinically and pathologically, based on a reduction in tumor size and assessment of residual tumor cellularity. The mean-value parameters and textural features were extracted from volumetric DOS images for several functional and metabolic parameters prior to the treatment initiation. Changes in these DOS-based biomarkers were also monitored over the course of treatment. The measured biomarkers were applied to differentiate patient responses noninvasively and compared to clinical and pathologic responses. Responding and nonresponding patients demonstrated different changes in DOS-based textural and mean-value parameters during chemotherapy. Whereas none of the biomarkers measured prior the start of therapy demonstrated a significant difference between the two patient populations, statistically significant differences were observed at week one after treatment initiation using the relative change in contrast/homogeneity of seven functional maps (0.001

  3. Perfil dos Professores de Ciências Naturais do Recôncavo da Bahia - Alunos da Disciplina Terra e Universo no Curso de Ciências Naturais do Parfor

    NASA Astrophysics Data System (ADS)

    Lima, S. R.; Cerqueira Júnior, W.; Dutra, G.

    2011-12-01

    Este trabalho foi desenvolvido pelo projeto Astronomia no Recôncavo da Bahia, no Centro de Formação de Professores da Universidade Federal do Recôncavo da Bahia. Traçamos o perfil de um grupo de professores que lecionam conteúdos de Ciências Naturais no recôncavo, alunos do curso de Licenciatura em Ciências Naturais, oferecido dentro do Plano Nacional de Formação de Professores da Educação Básica. Nosso objetivo era avaliar se eles estão preparados para trabalhar conteúdos de Astronomia e identificar suas dificuldades. Os resultados serviram para orientar o professor da disciplina “Terra e Universo”, oferecida no segundo semestre de 2010. Durante a primeira aula da disciplina Terra e Universo, os alunos responderam a um questionário contendo questões abertas e fechadas, divididas em duas partes. A primeira procurando caracterizar profissionalmente os alunos enquanto professores da rede pública da região do Recôncavo Sul da Bahia e uma segunda parte procurando identificar conhecimentos básicos em Astronomia. Os resultados indicam uma predominância de professores do sexo feminino, com idade superior aos 40 anos, pardos e sem formação específica para o ensino de ciências. A maioria leciona há mais de 15 anos para turmas do 1º ao 5º ano, alguns lecionam para turmas de 6º ao 9º ano. Quase todos nunca participaram de um curso de formação continuada em Astronomia. Além disso, não estão habituados a ler revistas especializadas e nem livros com esta temática. Os que procuram ensinar temas voltados para a Astronomia têm, no livro didático, a maior fonte de informação sobre o assunto. As respostas também indicam uma deficiência em conteúdos básicos como a compreensão da esfericidade da Terra, noções de verticalidade e gravidade, incapacidade de identificar a Terra como um planeta, no Sistema Solar, em uma galáxia, no Universo. Estes resultados ressaltam a importância de disciplinas de Astronomia básica na formação dos

  4. Deep Learning Method for Denial of Service Attack Detection Based on Restricted Boltzmann Machine.

    PubMed

    Imamverdiyev, Yadigar; Abdullayeva, Fargana

    2018-06-01

    In this article, the application of the deep learning method based on Gaussian-Bernoulli type restricted Boltzmann machine (RBM) to the detection of denial of service (DoS) attacks is considered. To increase the DoS attack detection accuracy, seven additional layers are added between the visible and the hidden layers of the RBM. Accurate results in DoS attack detection are obtained by optimization of the hyperparameters of the proposed deep RBM model. The form of the RBM that allows application of the continuous data is used. In this type of RBM, the probability distribution of the visible layer is replaced by a Gaussian distribution. Comparative analysis of the accuracy of the proposed method with Bernoulli-Bernoulli RBM, Gaussian-Bernoulli RBM, deep belief network type deep learning methods on DoS attack detection is provided. Detection accuracy of the methods is verified on the NSL-KDD data set. Higher accuracy from the proposed multilayer deep Gaussian-Bernoulli type RBM is obtained.

  5. Effective Patient-Physician Communication Based on Osteopathic Philosophy in Caring for Elderly Patients.

    PubMed

    Noll, Donald R; Ginsberg, Terrie; Elahi, Abdul; Cavalieri, Thomas A

    2016-01-01

    The objective of this article is to discuss effective communication strategies between elderly patients and their physicians from the perspective of osteopathic heritage. The patient-physician communication styles of Andrew Taylor Still, MD, DO, and early osteopathic physicians (ie, DOs) may have influenced how DOs today communicate with their patients. Historical literature describes how Still would discuss with his patients the causes of their health problems using analogies and language they would understand, and how, when caring for a patient at the end of life, he empathically provided emotional support for both patients and their families. Early DOs advocated setting clear expectations for patients regarding clinical outcomes and carefully listening to patients to build trust. The Osteopathic Oath, which calls for the DO to view the patient as a friend, may also affect patient-physician communication. Early osteopathic philosophy and culture, as modeled by Dr Still in his approach to elderly patients, should inspire today's DOs in their communication with their elderly patients.

  6. Density of photon states in dye-doped chiral nematic liquid crystal cells in the presence of losses and gain.

    PubMed

    Mavrogordatos, Th K; Morris, S M; Castles, F; Hands, P J W; Ford, A D; Coles, H J; Wilkinson, T D

    2012-07-01

    We calculate the density of photon states (DOS) of the normal modes in dye-doped chiral nematic liquid crystal (LC) cells in the presence of various loss mechanisms. Losses and gain are incorporated into the transmission characteristics through the introduction of a small imaginary part in the dielectric constant perpendicular and along the director, for which we assume no frequency dispersion. Theoretical results are presented on the DOS in the region of the photonic band gap for a range of values of the loss coefficient and different values of the optical anisotropy. The obtained values of the DOS at the photonic band gap edges predict a reversal of the dominant modes in the structure. Our results are found to be in good agreement with the experimentally obtained excitation thresholds in chiral nematic LC lasers. The behavior of the DOS is also discussed for amplifying LC cells providing additional insight to the lasing mechanism of these structures.

  7. Diversity-oriented synthetic strategy for developing a chemical modulator of protein-protein interaction

    NASA Astrophysics Data System (ADS)

    Kim, Jonghoon; Jung, Jinjoo; Koo, Jaeyoung; Cho, Wansang; Lee, Won Seok; Kim, Chanwoo; Park, Wonwoo; Park, Seung Bum

    2016-10-01

    Diversity-oriented synthesis (DOS) can provide a collection of diverse and complex drug-like small molecules, which is critical in the development of new chemical probes for biological research of undruggable targets. However, the design and synthesis of small-molecule libraries with improved biological relevance as well as maximized molecular diversity represent a key challenge. Herein, we employ functional group-pairing strategy for the DOS of a chemical library containing privileged substructures, pyrimidodiazepine or pyrimidine moieties, as chemical navigators towards unexplored bioactive chemical space. To validate the utility of this DOS library, we identify a new small-molecule inhibitor of leucyl-tRNA synthetase-RagD protein-protein interaction, which regulates the amino acid-dependent activation of mechanistic target of rapamycin complex 1 signalling pathway. This work highlights that privileged substructure-based DOS strategy can be a powerful research tool for the construction of drug-like compounds to address challenging biological targets.

  8. Screening for germline phosphatase and tensin homolog-mutations in suspected Cowden syndrome and Cowden syndrome-like families among uterine cancer patients

    PubMed Central

    TZORTZATOS, GERASIMOS; ARAVIDIS, CHRISTOS; LINDBLOM, ANNIKA; MINTS, MIRIAM; THAM, EMMA

    2015-01-01

    Cowden syndrome (CS) is an autosomal dominant disorder characterized by multiple hamartomas in the breast, thyroid and endometrium, with a prevalence of 1 per 250,000. Females with CS have a 21–28% lifetime risk of developing uterine cancer. Germline mutations in the phosphatase and tensin homolog (PTEN) gene, a tumor suppressor gene, are responsible for 30–80% of CS cases. PTEN is a nine-exon gene, located on chromosome 10q23.3, which encodes the 403 amino acid PTEN protein. It negatively regulates the phosphoinositide 3-kinase/protein kinase B/mammalian target of rapamycin pathway, affecting various cellular processes and signaling pathways. The present study examined whether PTEN mutations are present in CS-like families with uterine cancer (UC). UC patients underwent surgery at Karolinska University Hospital, Stockholm, Sweden (2008–2012). Pedigrees were analyzed and 54 unrelated CS-like families were identified. CS-like families were defined as having at least one occurrence of uterine cancer and one of breast cancer, as well as at least one additional Cowden-associated tumor (uterine, breast, thyroid, colon or kidney cancer) in the same individual or in first-degree relatives. Genomic DNA was amplified using polymerase chain reaction, and DNA sequencing analysis of all nine exons of the PTEN gene was conducted. No germline PTEN mutations or polymorphisms were identified. Germline PTEN mutations are rare in CS-like families with uterine cancer, therefore, genetic screening must be restricted to patients that meet the strict National Comprehensive Cancer Network criteria. Gynecologists must be aware of the CS criteria and identify potential cases of CS in females where uterine cancer is the sentinel cancer. PMID:25789042

  9. Birt-Hogg-Dubé syndrome: Clinical and molecular aspects of recently identified kidney cancer syndrome.

    PubMed

    Hasumi, Hisashi; Baba, Masaya; Hasumi, Yukiko; Furuya, Mitsuko; Yao, Masahiro

    2016-03-01

    Birt-Hogg-Dubé syndrome is an autosomal dominantly inherited disease that predisposes patients to develop fibrofolliculoma, lung cysts and bilateral multifocal renal tumors, histologically hybrid oncocytic/chromophobe tumors, chromophobe renal cell carcinoma, oncocytoma, papillary renal cell carcinoma and clear cell renal cell carcinoma. The predominant forms of Birt-Hogg-Dubé syndrome-associated renal tumors, hybrid oncocytic/chromophobe tumors and chromophobe renal cell carcinoma are typically less aggressive, and a therapeutic principle for these tumors is a surgical removal with nephron-sparing. The timing of surgery is the most critical element for postoperative renal function, which is one of the important prognostic factors for Birt-Hogg-Dubé syndrome patients. The folliculin gene (FLCN) that is responsible for Birt-Hogg-Dubé syndrome was isolated as a novel tumor suppressor for kidney cancer. Recent studies using murine models for FLCN, a protein encoded by the FLCN gene, and its two binding partners, folliculin-interacting protein 1 (FNIP1) and folliculin-interacting protein 2 (FNIP2), have uncovered important roles for FLCN, FNIP1 and FNIP2 in cell metabolism, which include AMP-activated protein kinase-mediated energy sensing, Ppargc1a-driven mitochondrial oxidative phosphorylation and mTORC1-dependent cell proliferation. Birt-Hogg-Dubé syndrome is a hereditary hamartoma syndrome, which is triggered by metabolic alterations under a functional loss of FLCN/FNIP1/FNIP2 complex, a critical regulator of kidney cell proliferation rate; a mechanistic insight into the FLCN/FNIP1/FNIP2 pathway could provide us a basis for developing new therapeutics for kidney cancer. © 2015 The Japanese Urological Association.

  10. Robotic surgery for benign duodenal tumors.

    PubMed

    Downs-Canner, Stephanie; Van der Vliet, Wald J; Thoolen, Stijn J J; Boone, Brian A; Zureikat, Amer H; Hogg, Melissa E; Bartlett, David L; Callery, Mark P; Kent, Tara S; Zeh, Herbert J; Moser, A James

    2015-02-01

    Benign duodenal and periampullary tumors are uncommon lesions requiring careful attention to their complex anatomic relationships with the major and minor papillae as well as the gastric outlet during surgical intervention. While endoscopy is less morbid than open resection, many lesions are not amenable to endoscopic removal. Robotic surgery offers technical advantages above traditional laparoscopy, and we demonstrate the safety and feasibility of this approach for a variety of duodenal lesions. We performed a retrospective review of all robotic duodenal resections between April 2010 and December 2013 from two institutions. Demographic, clinicopathologic, and operative details were recorded with special attention to the post-operative course. Twenty-six patients underwent robotic duodenal resection for a variety of diagnoses. The majority (88 %) were symptomatic at presentation. Nine patients underwent transduodenal ampullectomy, seven patients underwent duodenal resection, six patients underwent transduodenal resection of a mass, and four patients underwent segmental duodenal resection. Median operative time was 4 h with a median estimated blood loss of 50 cm(3) and no conversions to an open operation. The rate of major Clavien-Dindo grades 3-4 complications was 15 % at post-operative days 30 and 90 without mortality. Final pathology demonstrated a median tumor size of 2.9 cm with a final histologic diagnoses of adenoma (n = 13), neuroendocrine tumor (n = 6), gastrointestinal stromal tumor (GIST) (n = 2), lipoma (n = 2), Brunner's gland hamartoma (n = 1), leiomyoma (n = 1), and gangliocytic paraganglioma (n = 1). Robotic duodenal resection is safe and feasible for benign and premalignant duodenal tumors not amenable to endoscopic resection.

  11. Somatic gain-of-function mutations in PIK3CA in patients with macrodactyly

    PubMed Central

    Rios, Jonathan J.; Paria, Nandina; Burns, Dennis K.; Israel, Bonnie A.; Cornelia, Reuel; Wise, Carol A.; Ezaki, Marybeth

    2013-01-01

    Macrodactyly is a discrete congenital anomaly consisting of enlargement of all tissues localized to the terminal portions of a limb, typically within a ‘nerve territory’. The classic terminology for this condition is ‘lipofibromatous hamartoma of nerve’ or Type I macrodactyly. The peripheral nerve, itself, is enlarged both in circumference and in length. It is not related to neurofibromatosis (NF1), nor is it associated with vascular malformations, such as in the recently reported CLOVES syndrome. The specific nerve pathophysiology in this form of macrodactyly has not been well described and a genetic etiology for this specific form of enlargement is unknown. To identify the genetic cause of macrodactyly, we used whole-exome sequencing to identify somatic mutations present in the affected nerve of a single patient. We confirmed a novel mutation in PIK3CA (R115P) present in the patient's affected nerve tissue but not in blood DNA. Sequencing PIK3CA exons identified gain-of-function mutations (E542K, H1047L or H1047R) in the affected tissue of five additional unrelated patients; mutations were absent in blood DNA available from three patients. Immunocytochemistry confirmed AKT activation in cultured cells from the nerve of a macrodactyly patient. Additionally, we found that the most abnormal structure within the involved nerve in a macrodactylous digit is the perineurium, with additional secondary effects on the axon number and size. Thus, isolated congenital macrodactyly is caused by somatic activation of the PI3K/AKT cell-signaling pathway and is genetically and biochemically related to other overgrowth syndromes. PMID:23100325

  12. Constitutional FLCN mutations in patients with suspected Birt-Hogg-Dubé syndrome ascertained for non-cutaneous manifestations.

    PubMed

    Maffé, A; Toschi, B; Circo, G; Giachino, D; Giglio, S; Rizzo, A; Carloni, A; Poletti, V; Tomassetti, S; Ginardi, C; Ungari, S; Genuardi, M

    2011-04-01

    Birt-Hogg-Dubé syndrome (BHDS) is characterized by a clinical triad including cutaneous hamartomas originating from hair follicles, lung cysts/pneumothorax, and kidney tumors. Inactivating mutations of the tumor suppressor gene FLCN are identified in most families with BHDS. Usually, patients are referred for genetic examination by dermatologists because of the presence of typical multiple skin tumors with or without additional symptoms. However, because of phenotypic variability and incomplete penetrance, the clinical presentation of BHDS is not yet fully defined. Criteria for genetic testing and diagnosis that take into account variable manifestations have recently been proposed by the European BHD Consortium. We sequenced the FLCN gene coding region in a series of 19 patients selected for kidney and/or lung manifestations. Overall, FLCN mutations were found in 9 of 19 (47%) families and were detected only in probands who had either >2 components of the clinical triad or a single component (renal or pulmonary) along with a family history of another main BHDS manifestation. Typical cutaneous lesions were present only in 8 of 21 FLCN mutation carriers aged >20 years identified in the mutation-positive families. In addition, we provide clinical and molecular evidence that parotid oncocytoma, so far reported in six BHDS cases, is associated with this condition, based on the observation of a patient with bilateral parotid involvement and marked reduction of the wild-type FLCN allele signal in tumor DNA. Overall, the results obtained in this study contribute to the definition of the phenotypic characteristics that should be considered for BHDS diagnosis and FLCN mutation testing. © 2010 John Wiley & Sons A/S.

  13. Co2/Erbium:YAG/Dye laser combination: an effective and successful treatment for angiofibromas in tuberous sclerosis.

    PubMed

    Fioramonti, Paolo; De Santo, Liliana; Ruggieri, Martina; Carella, Sara; Federico, Lo Torto; Onesti, Maria Giuseppina; Scuderi, Nicolò

    2014-02-01

    Tuberous sclerosis, an autosomal dominant neurocutaneous syndrome seen in approximately 1 in 6,000 people worldwide, is characterized by the appearance of hamartomas in multiple organs. The classic clinical triad consists of angiofibromas, epilepsy, and developmental delay. Dermatologic disorder is one of the main characteristics. Angiofibromas, a common form of presentation, causes significant cosmetic and medical problems. The current treatment for skin lesions is laser therapy. The carbon dioxide (CO2) laser has been used satisfactorily in treating these lesions, but several studies have demonstrated a high percentage of recurrences. Erbium:yttrium-aluminum-garnet (YAG) laser treatment has been used to resurface skin abnormalities in patients with dermatologic conditions. The dye laser as an alternative uses the principles of selective photothermolysis and is very effective in treating the vascular component of tuberous sclerosis. The use of all these lasers to treat skin lesions in patients affected by tuberous sclerosis has never been described in the literature. A retrospective study, conducted from 2007 to May 2013, investigated 13 patients who had tuberous sclerosis treated with an erbium:YAG/CO2/dye laser combination. All the patients showed great improvement of their skin lesions. The results were evident immediately after the first treatment. No patient experienced complications or recurrence. The combined use of the erbium:YAG/Dye/CO2 laser is a safe and effective treatment for skin lesions in patients affected by tuberous sclerosis. This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

  14. One gene - many endocrine and metabolic syndromes: PTEN-opathies and precision medicine.

    PubMed

    Yehia, Lamis; Eng, Charis

    2018-05-23

    An average of 10% of all cancers (range 1-40%) are caused by heritable mutations and over the years, have become powerful models for precision medicine practice. Furthermore, such cancer predisposition genes for seemingly rare syndromes have turned out to help explain mechanisms of sporadic carcinogenesis and often inform normal development. The tumor suppressor PTEN encodes a ubiquitously expressed phosphatase that counteracts the PI3K/AKT/mTOR cascade - one of the most critical growth-promoting signaling pathways. Clinically, individuals with germline PTEN mutations have diverse phenotypes and fall under the umbrella term PTEN hamartoma tumor syndrome (PHTS). PHTS encompasses four clinically distinct allelic overgrowth syndromes, namely Cowden, Bannayan-Riley-Ruvalcaba, Proteus, and Proteus-like syndromes. Relatedly, mutations in other genes encoding components of the PI3K/AKT/mTOR pathway downstream of PTEN also predispose patients to partially overlapping clinical manifestations, with similar effects as PTEN malfunction. We refer to these syndromes as "PTEN-opathies." As a tumor suppressor and key regulator of normal development, PTEN dysfunction can cause a spectrum of phenotypes including benign overgrowths, malignancies, metabolic, and neurodevelopmental disorders. Relevant to clinical practice, the identification of PTEN mutations in patients not only establishes a PHTS molecular diagnosis, but also informs on more accurate cancer risk assessment and medical management of those patients and affected family members. Importantly, timely diagnosis is key, as early recognition allows for preventative measures such as high-risk screening and surveillance even prior to cancer onset. This review highlights the translational impact that the discovery of PTEN has had on the diagnosis, management, and treatment of PHTS.

  15. PI3K/AKT pathway mutations cause a spectrum of brain malformations from megalencephaly to focal cortical dysplasia

    PubMed Central

    Mirzaa, Ghayda M.; Ishak, Gisele E.; O'Roak, Brian J.; Hiatt, Joseph B.; Roden, William H.; Gunter, Sonya A.; Christian, Susan L.; Collins, Sarah; Adams, Carissa; Rivière, Jean-Baptiste; St-Onge, Judith; Ojemann, Jeffrey G.; Shendure, Jay; Hevner, Robert F.; Dobyns, William B.

    2015-01-01

    Malformations of cortical development containing dysplastic neuronal and glial elements, including hemimegalencephaly and focal cortical dysplasia, are common causes of intractable paediatric epilepsy. In this study we performed multiplex targeted sequencing of 10 genes in the PI3K/AKT pathway on brain tissue from 33 children who underwent surgical resection of dysplastic cortex for the treatment of intractable epilepsy. Sequencing results were correlated with clinical, imaging, pathological and immunohistological phenotypes. We identified mosaic activating mutations in PIK3CA and AKT3 in this cohort, including cancer-associated hotspot PIK3CA mutations in dysplastic megalencephaly, hemimegalencephaly, and focal cortical dysplasia type IIa. In addition, a germline PTEN mutation was identified in a male with hemimegalencephaly but no peripheral manifestations of the PTEN hamartoma tumour syndrome. A spectrum of clinical, imaging and pathological abnormalities was found in this cohort. While patients with more severe brain imaging abnormalities and systemic manifestations were more likely to have detected mutations, routine histopathological studies did not predict mutation status. In addition, elevated levels of phosphorylated S6 ribosomal protein were identified in both neurons and astrocytes of all hemimegalencephaly and focal cortical dysplasia type II specimens, regardless of the presence or absence of detected PI3K/AKT pathway mutations. In contrast, expression patterns of the T308 and S473 phosphorylated forms of AKT and in vitro AKT kinase activities discriminated between mutation-positive dysplasia cortex, mutation-negative dysplasia cortex, and non-dysplasia epilepsy cortex. Our findings identify PI3K/AKT pathway mutations as an important cause of epileptogenic brain malformations and establish megalencephaly, hemimegalencephaly, and focal cortical dysplasia as part of a single pathogenic spectrum. PMID:25722288

  16. Fiscal Year 2011 Afghanistan Infrastructure Fund Projects Are Behind Schedule and Lack Adequate Sustainment Plans

    DTIC Science & Technology

    2012-07-30

    interconnected subset ofUSATD Economic Support Fund ( ESl -") projects, which arc essential to meet program ol~jcctives. Under the program, DOS and US AID...with conunent: The congressional reporting requirements in the KDAA cover AIF funded projects, whether executed hy DoD or DOS. ESl ’-tunded projects

  17. 78 FR 19077 - Forwarding of Asylum Applications to the Department of State

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-03-29

    ... Department of State (DOS), Bureau of Democracy, Human Rights, and Labor. Currently, EOIR forwards to DOS all... form of post-traumatic stress or depression that affects long-term memory, making credibility... time of dwindling resources, both human and monetary, the Department has determined that it is best to...

  18. Choosing the Right Database Management Program.

    ERIC Educational Resources Information Center

    Vockell, Edward L.; Kopenec, Donald

    1989-01-01

    Provides a comparison of four database management programs commonly used in schools: AppleWorks, the DOS 3.3 and ProDOS versions of PFS, and MECC's Data Handler. Topics discussed include information storage, spelling checkers, editing functions, search strategies, graphs, printout formats, library applications, and HyperCard. (LRW)

  19. 48 CFR 609.406-3 - Procedures.

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... statement from each DOS organizational element affected by the debarment action as to the impact of a... is responsible for making the transcribed record of the hearing, unless the contractor and the fact... decision to each DOS organizational element affected by the decision and to the General Services...

  20. Geochemistry and genesis of the angrites

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

    Mittlefehldt, D.W.; Lindstrom, M.M.

    1990-11-01

    The angrites Angra dos Reis, LEW86010, and LEW87051 are petrologically and compositionally similar achondrites. All angrites have high FeO/MnO ratios of 80-94 and very low CI normalized Na/Sm ratios of 0.001-0.003. High abundances of oxidized Fe and low abundances of moderately volatile Na most likely resulted from parent body processes, such as magmatic outgassing, rather than nebular processes. All angrites have fractionated Ca/Al ratios, with Angra dos Reis exhibiting the most extreme ratio (3.1 {times} CI). For Angra dos Reis, cumulus material may be the cause of the high Ca/Al ratio. Refractory element abundances of LEW86010 and LEW87051 show similarmore » patterns, while Angra dos Reis has both greater enrichments in these elements and more fractionated patterns. Compositional and petrologic constraints indicate that LEW86010 and LEW87051 are related via olivine control. The refractory element abundances and mg{number sign} of LEW86010 can be approximated by removal of olivine from LEW87051, suggesting that LEW86010 may be a residual melt from a LEW87051-like precursor. Alternatively, LEW87051 may have formed via olivine accumulation from a LEW86010-like precursor. The differences between the LEW86010-LEW87051 duo and Angra dos Reis suggest that either the angrite parent body was heterogeneous or that Angra dos Reis was formed on a separate parent body. Based on FeO/MnO ratios and normative mineralogies, the angrite parent body(ies) may be similar in bulk composition to one of the carbonaceous chondrite groups, particularly CI-CM-CO.« less

  1. A Concepção de Universo entre Alunos do Ensino Médio de São Paulo e suas Fontes de Aquisição

    NASA Astrophysics Data System (ADS)

    Araújo, M. A. A.; Elias, D. C. N.; Amaral, L. H.; Araújo, M. S. T.; Voelzke, M. R.

    2006-08-01

    Nesse trabalho procurou-se identificar por meio de um questionário as concepções de Universo, de espaço e tempo que sustentam a visão de mundo de um grupo de 270 estudantes de Ensino Médio, pertencentes a três escolas de São Paulo. As questões relacionadas aos conhecimentos prévios dos estudantes permitiram constatar que há pouco conhecimento acerca dos temas investigados, destacando-se que apenas 20% dos alunos foram capazes de relacionar as semanas com as fases da lua, enquanto 28% associaram as estações do ano à inclinação do eixo de rotação da Terra e 23% tinham noções das distâncias entre objetos celestes próximos da Terra. Enquanto 56% conseguiram relacionar o Big Bang com a origem do Universo, verificou-se que 37% reconheciam ano-luz como unidade de distância e 60% concebiam o Sol como uma estrela. No que se refere às fontes de aquisição que proporcionaram esses conhecimentos, apesar de 60% dos alunos indicarem a escola como principal fonte dos conhecimentos de Ast! ronomia, verificou-se claramente que para a maioria dos alunos seus conceitos ainda são inadequados, havendo necessidade de aprimoramento da abordagem desses conteúdos, pois apesar de popular, a Astronomia ainda é veiculada de maneira pouco esclarecedora e com imprecisões. Nesse contexto, são discutidas algumas possíveis contribuições que podem ser dadas para o ensino de Astronomia pelo uso das ferramentas computacionais nas escolas.

  2. Coulomb gap triptych in a periodic array of metal nanocrystals.

    PubMed

    Chen, Tianran; Skinner, Brian; Shklovskii, B I

    2012-09-21

    The Coulomb gap in the single-particle density of states (DOS) is a universal consequence of electron-electron interaction in disordered systems with localized electron states. Here we show that in arrays of monodisperse metallic nanocrystals, there is not one but three identical adjacent Coulomb gaps, which together form a structure that we call a "Coulomb gap triptych." We calculate the DOS and the conductivity in two- and three-dimensional arrays using a computer simulation. Unlike in the conventional Coulomb glass models, in nanocrystal arrays the DOS has a fixed width in the limit of large disorder. The Coulomb gap triptych can be studied via tunneling experiments.

  3. Scanning tunneling spectroscopy of MoS2 monolayer in presence of ethanol gas

    NASA Astrophysics Data System (ADS)

    Hosseini, Seyed Ali; Iraji zad, Azam; Berahman, Masoud; Aghakhani Mahyari, Farzaneh; Shokouh, Seyed Hossein Hosseini

    2018-04-01

    Due to high surface to volume ratio and tunable band gap, two dimensional (2D) layered materials such as MoS2, is good candidate for gas sensing applications. This research mainly focuses on variation of Density of States (DOS) of MoS2 monolayes caused by ethanol adsorption. The nanosheets are synthesized by liquid exfoliation, and then using Scanning Tunneling Spectroscopy (STS) and Density Functional Theory (DFT), local electronic characteristic such as DOS and band gap in non-vacuum condition are analyzed. The results show that ethanol adsorption enhances DOS and deform orbitals near the valence and conduction bands that increase transport of carriers on the sheet.

  4. EVALUATION OF POSTGRADUATES STRICTO SENSU: MONITORING POLICY FOR INTERNATIONAL GRADUATES.

    PubMed

    Lima, Wilma Terezinha Anselmo

    2015-01-01

    Search for references in relationship to international alumni on the website of the postgraduate programs of all postgraduate courses at Ribeirão Preto Medical School - FMRP. Verify with more attention to the ones with 5, 6 and 7 notes, and also the same search on the website of courses with notes 5, 6 and 7 of CAPES - Medicine III. Of the 22 programs of FMRP only three had any information on the site about the destiny of the postgraduates; they were: Surgical Clinics, Genetics, and Basic and Applied Immunology. Programs in the area of ​​Medicine III, notes 5, 6 and 7, only Ophthalmology and Visual Programs and Translational Sciences Surgery, both of UNIFESP, presented such information. It is urgent: to create project and funding evaluation mechanisms that are approved by different sources; to stimulate more efficient controls in relation to teachers and their students who participate in these projects; and to stimulate the interaction of teachers and students with the institution and the program. Verificar a existência de referências aos egressos internacionais nos sites dos programas de pós-graduação de todos os cursos de pós-graduação da Faculdade de Medicina de Ribeirão Preto - FMRP. Verificar com mais atenção aos cursos notas 5, 6 e 7, e também a mesma busca nos sites dos cursos com notas 5, 6 e 7 da Medicina III da CAPES. Dos 22 programas da FMRP apenas três tinham no site alguma informação sobre o destino dos egressos, foram eles: Clínica Cirúrgica, Genética e Imunologia Básica e Aplicada. Dos programas da área de Medicina III, notas 5, 6 e 7 apenas os programas de Oftalmologia e Ciências Visuais e Cirurgia Translacional, ambos da UNIFESP, apresentavam informações sobre o destino dos seus egressos. É urgente criar mecanismos de avaliação para os projetos de incentivo e fomento à pesquisa dos diferentes órgãos; estimular controles mais eficientes e atualizados em relação aos docentes e seus respectivos discentes que

  5. 78 FR 45490 - Department of State Acquisition Regulation

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-07-29

    ... being added due to the need to obtain current data to support the Department of State (DOS) financial statements. From a financial accounting perspective, DOS must have a way of keeping track of its capital... DOSAR 652.245- 71, Accounting for Government Property, requests quarterly reporting of U.S. Department...

  6. Measuring Student Learning in Social Justice Courses: The Diversity and Oppression Scale

    ERIC Educational Resources Information Center

    Windsor, Liliane Cambraia; Shorkey, Clay; Battle, DuWayne

    2015-01-01

    The Diversity and Oppression Scale (DOS) is a standardized instrument measuring self-reported student learning about diversity and oppression based on requirements of the Council on Social Work Education. DOS was tested with social work students in 2 major North American universities. Factor structure was examined using exploratory factor analysis…

  7. Clearing a Path: The 16-Bit Operating System Jungle Offers Confusion, Not Standardization.

    ERIC Educational Resources Information Center

    Pournelle, Jerry

    1984-01-01

    Discusses the design and limited uses of the Pascal, MS-DOS, CP/M, and PC-DOS operating systems as standard operating systems for 16-bit microprocessors, especially with the more sophisticated microcomputers currently being developed. Advantages and disadvantages of Unix--a multitasking, multiuser operating system--as a standard operating system…

  8. Towards Quantifying Programmable Logic Controller Resilience Against Intentional Exploits

    DTIC Science & Technology

    2012-03-22

    may improve the SCADA system’s resilience against DoS and man-in-the-middle ( MITM ) attacks. DoS attacks may be mitigated by using the redundant...paths available on the network links. MITM attacks may be mitigated by the data integrity checks associated with the middleware. Figure 4 illustrates

  9. Is There a Disk of Satellites around the Milky Way?

    NASA Astrophysics Data System (ADS)

    Maji, Moupiya; Zhu, Qirong; Marinacci, Federico; Li, Yuexing

    2017-07-01

    The “disk of satellites” (DoS) around the Milky Way is a highly debated topic with conflicting interpretations of observations and their theoretical models. We perform a comprehensive analysis of all of the dwarfs detected in the Milky Way and find that the DoS structure depends strongly on the plane identification method and the sample size. In particular, we demonstrate that a small sample size can artificially produce a highly anisotropic spatial distribution and a strong clustering of the angular momentum of the satellites. Moreover, we calculate the evolution of the 11 classical satellites with proper motion measurements and find that the thin DoS in which they currently reside is transient. Furthermore, we analyze two cosmological simulations using the same initial conditions of a Milky-Way-sized galaxy, an N-body run with dark matter only, and a hydrodynamic one with both baryonic and dark matter, and find that the hydrodynamic simulation produces a more anisotropic distribution of satellites than the N-body one. Our results suggest that an anisotropic distribution of satellites in galaxies can originate from baryonic processes in the hierarchical structure formation model, but the claimed highly flattened, coherently rotating DoS of the Milky Way may be biased by the small-number selection effect. These findings may help resolve the contradictory claims of DoS in galaxies and the discrepancy among numerical simulations.

  10. Influence of co-culture with denuded oocytes during in vitro maturation on fertilization and developmental competence of cumulus-enclosed porcine oocytes in a defined system.

    PubMed

    Appeltant, Ruth; Somfai, Tamás; Kikuchi, Kazuhiro; Maes, Dominiek; Van Soom, Ann

    2016-04-01

    Co-culture of cumulus-oocyte complexes (COCs) with denuded oocytes (DOs) during in vitro maturation (IVM) was reported to improve the developmental competence of oocytes via oocyte-secreted factors in cattle. The aim of the present study was to investigate if addition of DOs during IVM can improve in vitro fertilization (IVF) and in vitro culture (IVC) results for oocytes in a defined in vitro production system in pigs. The maturation medium was porcine oocyte medium supplemented with gonadotropins, dbcAMP and β-mercaptoethanol. Cumulus-oocyte complexes were matured without DOs or with DOs in different ratios (9 COC, 9 COC+16 DO and 9 COC+36 DO). Consequently; oocytes were subjected to IVF as intact COCs or after denudation to examine if DO addition during IVM would affect cumulus or oocyte properties. After fertilization, penetration and normal fertilization rates of zygotes were not different between all tested groups irrespective of denudation before IVF. When zygotes were cultured for 6 days, no difference could be observed between all treatment groups in cleavage rate, blastocyst rate and cell number per blastocyst. In conclusion, irrespective of the ratio, co-culture with DOs during IVM did not improve fertilization parameters and embryo development of cumulus-enclosed porcine oocytes in a defined system. © 2015 Japanese Society of Animal Science.

  11. Fine structure of striations observed in barium plasma injections in the magnetospheric cleft

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

    Simons, D.J.; Eastman, T.E.; Pongratz, M.B.

    1976-01-01

    In January and November of 1975, the Los Alamos Scientific Laboratory sponsored four high altitude shaped charge barium plasma injections in the magnetospheric cleft region. These experiments were TORDO UNO (January 6), TORDO DOS (January 11), PERIQUITO UNO (November 25), and PERIQUITO DOS (November 28). All four injections took place near 500 km altitude, and optical data were taken from two aircraft and a ground station. The TORDO DOS and the PERIQUITO experiments showed rapid formation of striations (within one minute after injection), and fast horizontal spreading in contrast with TORDO UNO. In PERIQUITO DOS, the debris cloud spread magneticallymore » east-west with a small net northerly motion. TORDO UNO shows very rapid poleward motion, and the remaining two events resulted in magnetically east-west horizontal spreading, with no noticeable poleward motion. Striations observed in the PERIQUITO DOS experiment separate in opposite directions with relative velocities of up to 3 km/sec. These field-aligned structures appear to form in sheets of approximately constant magnetic latitude. Significant spatial variations occur on a scale of less than 200 meters. Spatial frequency power spectra across these striations have been determined at various times. Observations of the debris cloud and the fast barium streak show strong field-aligned coherency of striation fine structure, indicating a field line mapping of transverse electric fields and gradients.« less

  12. Early detection of chemotherapy-refractory patients by monitoring textural alterations in diffuse optical spectroscopic images

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

    Sadeghi-Naini, Ali; Falou, Omar; Czarnota, Gregory J., E-mail: Gregory.Czarnota@sunnybrook.ca

    2015-11-15

    Purpose: Changes in textural characteristics of diffuse optical spectroscopic (DOS) functional images, accompanied by alterations in their mean values, are demonstrated here for the first time as early surrogates of ultimate treatment response in locally advanced breast cancer (LABC) patients receiving neoadjuvant chemotherapy (NAC). NAC, as a standard component of treatment for LABC patient, induces measurable heterogeneous changes in tumor metabolism which were evaluated using DOS-based metabolic maps. This study characterizes such inhomogeneous nature of response development, by determining alterations in textural properties of DOS images apparent at early stages of therapy, followed later by gross changes in mean valuesmore » of these functional metabolic maps. Methods: Twelve LABC patients undergoing NAC were scanned before and at four times after treatment initiation, and tomographic DOS images were reconstructed at each time. Ultimate responses of patients were determined clinically and pathologically, based on a reduction in tumor size and assessment of residual tumor cellularity. The mean-value parameters and textural features were extracted from volumetric DOS images for several functional and metabolic parameters prior to the treatment initiation. Changes in these DOS-based biomarkers were also monitored over the course of treatment. The measured biomarkers were applied to differentiate patient responses noninvasively and compared to clinical and pathologic responses. Results: Responding and nonresponding patients demonstrated different changes in DOS-based textural and mean-value parameters during chemotherapy. Whereas none of the biomarkers measured prior the start of therapy demonstrated a significant difference between the two patient populations, statistically significant differences were observed at week one after treatment initiation using the relative change in contrast/homogeneity of seven functional maps (0.001 < p < 0.049), and mean value of

  13. Yours, Mine or Ours: What Counts as Innovation?

    ERIC Educational Resources Information Center

    Cooksey, Ray W.

    2011-01-01

    In this paper, I argue that research and development organizations (R&DOs) have particular perspectives on what counts as an innovation whereas the potential adopting users usually have quite different often diverse perspectives. If these "worldviews" do not overlap or speak to each other, then what R&DOs consider innovative might constitute a…

  14. The Challenges for Physicians of Demonstrating Continuing Competence in the Changing World of Medical Regulation: Osteopathic Pediatrician Case Report

    ERIC Educational Resources Information Center

    Langenau, Erik E.; Gimpel, John R.

    2012-01-01

    The current system of continuing medical education, maintenance of certification, and renewal of medical licenses can be quite burdensome and inefficient for all practicing physicians: medical doctors (M.D.s) and doctors of osteopathic medicine (D.O.s). D.O.s have opportunities for residency training and specialty certification which are not…

  15. RESPSYST: An Interactive Microcomputer Program for Education.

    ERIC Educational Resources Information Center

    Boyle, Joseph

    1985-01-01

    RESPSYST is a computer program (written in BASICA and using MS-DOS/PC-DOS microcomputers) incorporating more than 20 of the factors that determine gas transport by the cardio-respiratory system. The five-part program discusses most of these factors, provides several question/answer sections, and relies heavily on graphics to demonstrate…

  16. A new species of Eimeria Schneider, 1875 from the Serra dos Órgãos National Park, Rio de Janeiro, Brazil, with notes on its endogenous development in the montane grass mouse, Akodon montensis Thomas, 1913 (Rodentia: Sigmodontinae).

    PubMed

    de Santana Miglionico, Marcos Tobias; Viana, Lúcio André; Barbosa, Helene Santos; Mota, Ester Maria; da Costa Neto, Sócrates Fraga; Frazão-Teixeira, Edwards; D'Andrea, Paulo Sergio

    2018-02-01

    A total of 53 specimens of the montane grass mouse, Akodon montensis Thomas, 1913 were collected in the Serra dos Órgãos National Park (SONP) in November 2014 and July 2015. The fecal material was analyzed, and a prevalence of 7.5% was recorded for a new coccidian species of the genus Eimeria Schneider, 1875, with part of its endogenous development recorded in the small intestine. The oocysts of a new coccidian species of genus Eimeria are ellipsoidal to subspherical. The wall is bi-layered, c. 1.5 μm (1.3-1.6 μm) thick, outer layer rough. Oocyst (n = 126) mean length is 25.3 μm (21.0-28.0 μm), with a width of 20.2 μm (17.0-22.0 μm) and mean length/width (L:W) ratio of 1.3 (1.2-1.4). Polar granule is present, with the oocyst residuum as a large spherical to subspherical globule. Sporocyst shape (n = 126) is ellipsoidal, with a mean length of 11.8 μm (9.3-14.4 μm), width of 7.9 μm (6.7-9.3 μm), and mean L:W ratio of 1.5 (1.4-1.7). Sporocysts with nipple-like Stieda body and sub-Stieda body are absent. A sporocyst residuum formed by several globules, usually along the sporocyst wall. This is the first record of Eimeria in the montane grass mouse from Brazil.

  17. Como os Alunos do Ensino Médio da Rede Estadual de São Paulo obtém Conhecimentos Astronômicos?

    NASA Astrophysics Data System (ADS)

    da Cunha, W. S.; Voelzke, M. R.; Amaral, L. H.

    2005-08-01

    Atualmente vivencia-se um mundo globalizado onde os computadores e a internet permitiram um acesso rápido e seguro a todo tipo de informação e conhecimento. O presente trabalho visa analisar a maneira pela qual alunos de segundo grau da rede estadual da cidade de São Paulo obtiveram, caso tenham, conhecimentos básicos de astronomia quanto aos fenômenos celestes que os rodeiam, tais como a sucessão dos dias e das estações do ano, além de questioná~los sobre fatos genéricos tais como: o que vem a ser o Sol, o Big Bang, o que ocasionou a extinção dos dinossauros. Para tanto foi elaborado um formulário constando de questões de múltipla escolha, o qual foi aplicado no primeiro colegial diurno da Escola Estadual Guilherme de Almeida. Num espaço amostral de 44 alunos constatou-se que 41% dos alunos adquiriram seus conhecimentos astronômicos na escola e 59% através da mídia em geral. Neste mesmo espaço amostral apenas 11% dos alunos usaram computadores na escola, 41% na residência, 5% no trabalho e 43% não utilizaram. O presente estudo revelou também que para 50% dos alunos o professor jamais utilizou um programa de computador a respeito de astronomia ou fez alguma apresentação sobre o tema. Embora em sua fase inicial este estudo revela claramente que a maioria dos alunos não obtém na escola seus conhecimentos astronômicos, estes provém de fontes não especificamente didático-pedagógicas tais como filmes e revistas populares que não raramente geram conhecimentos incompletos e em muitos casos inclusive falhos.

  18. 8 CFR 212.6 - Border crossing identification cards.

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ... or pleasure without a visa and passport, must apply to the DOS on Form DS-156, Visitor Visa... border crossing card (or similar stamp in a passport). (i) A Canadian citizen or other person sharing... crossing card (or similar stamp in a passport) issued by the DOS prior to April 1, 1998, that does not...

  19. 8 CFR 212.6 - Border crossing identification cards.

    Code of Federal Regulations, 2013 CFR

    2013-01-01

    ... or pleasure without a visa and passport, must apply to the DOS on Form DS-156, Visitor Visa... border crossing card (or similar stamp in a passport). (i) A Canadian citizen or other person sharing... crossing card (or similar stamp in a passport) issued by the DOS prior to April 1, 1998, that does not...

  20. 8 CFR 212.6 - Border crossing identification cards.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... or pleasure without a visa and passport, must apply to the DOS on Form DS-156, Visitor Visa... border crossing card (or similar stamp in a passport). (i) A Canadian citizen or other person sharing... crossing card (or similar stamp in a passport) issued by the DOS prior to April 1, 1998, that does not...

  1. 8 CFR 212.6 - Border crossing identification cards.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... or pleasure without a visa and passport, must apply to the DOS on Form DS-156, Visitor Visa... border crossing card (or similar stamp in a passport). (i) A Canadian citizen or other person sharing... crossing card (or similar stamp in a passport) issued by the DOS prior to April 1, 1998, that does not...

  2. 8 CFR 212.6 - Border crossing identification cards.

    Code of Federal Regulations, 2014 CFR

    2014-01-01

    ... or pleasure without a visa and passport, must apply to the DOS on Form DS-156, Visitor Visa... border crossing card (or similar stamp in a passport). (i) A Canadian citizen or other person sharing... crossing card (or similar stamp in a passport) issued by the DOS prior to April 1, 1998, that does not...

  3. tRNA-mediated codon-biased translation in mycobacterial hypoxic persistence

    NASA Astrophysics Data System (ADS)

    Chionh, Yok Hian; McBee, Megan; Babu, I. Ramesh; Hia, Fabian; Lin, Wenwei; Zhao, Wei; Cao, Jianshu; Dziergowska, Agnieszka; Malkiewicz, Andrzej; Begley, Thomas J.; Alonso, Sylvie; Dedon, Peter C.

    2016-11-01

    Microbial pathogens adapt to the stress of infection by regulating transcription, translation and protein modification. We report that changes in gene expression in hypoxia-induced non-replicating persistence in mycobacteria--which models tuberculous granulomas--are partly determined by a mechanism of tRNA reprogramming and codon-biased translation. Mycobacterium bovis BCG responded to each stage of hypoxia and aerobic resuscitation by uniquely reprogramming 40 modified ribonucleosides in tRNA, which correlate with selective translation of mRNAs from families of codon-biased persistence genes. For example, early hypoxia increases wobble cmo5U in tRNAThr(UGU), which parallels translation of transcripts enriched in its cognate codon, ACG, including the DosR master regulator of hypoxic bacteriostasis. Codon re-engineering of dosR exaggerates hypoxia-induced changes in codon-biased DosR translation, with altered dosR expression revealing unanticipated effects on bacterial survival during hypoxia. These results reveal a coordinated system of tRNA modifications and translation of codon-biased transcripts that enhance expression of stress response proteins in mycobacteria.

  4. tRNA-mediated codon-biased translation in mycobacterial hypoxic persistence

    PubMed Central

    Chionh, Yok Hian; McBee, Megan; Babu, I. Ramesh; Hia, Fabian; Lin, Wenwei; Zhao, Wei; Cao, Jianshu; Dziergowska, Agnieszka; Malkiewicz, Andrzej; Begley, Thomas J.; Alonso, Sylvie; Dedon, Peter C.

    2016-01-01

    Microbial pathogens adapt to the stress of infection by regulating transcription, translation and protein modification. We report that changes in gene expression in hypoxia-induced non-replicating persistence in mycobacteria—which models tuberculous granulomas—are partly determined by a mechanism of tRNA reprogramming and codon-biased translation. Mycobacterium bovis BCG responded to each stage of hypoxia and aerobic resuscitation by uniquely reprogramming 40 modified ribonucleosides in tRNA, which correlate with selective translation of mRNAs from families of codon-biased persistence genes. For example, early hypoxia increases wobble cmo5U in tRNAThr(UGU), which parallels translation of transcripts enriched in its cognate codon, ACG, including the DosR master regulator of hypoxic bacteriostasis. Codon re-engineering of dosR exaggerates hypoxia-induced changes in codon-biased DosR translation, with altered dosR expression revealing unanticipated effects on bacterial survival during hypoxia. These results reveal a coordinated system of tRNA modifications and translation of codon-biased transcripts that enhance expression of stress response proteins in mycobacteria. PMID:27834374

  5. A Visão do Universo Segundo a Concepção de um Grupo de Alunos do Ensino Médio de São Paulo

    NASA Astrophysics Data System (ADS)

    Araújo, M. A. A.; Elias, D. C. N.; Amaral, L. H.; Araújo, M. S. T.; Voelzke, M. R.

    2005-12-01

    Nesse trabalho foi identificada por meio de um questionário a visão de mundo sobre o Universo espaço e tempo entre 270 estudantes de Ensino Médio de três escolas de São Paulo. Constatou-se pouco conhecimento dos temas investigados sendo que apenas 20% dos alunos relacionaram as semanas com as fases da lua enquanto 28% associaram as estações do ano à inclinação do eixo de rotação da Terra e 23% tinham noções das distâncias entre objetos celestes próximos da Terra. Enquanto 56% conseguiram relacionar o Big Bang com a origem do Universo verificou-se que 37% reconheciam ano-luz como unidade de distância e 60% reconheciam o Sol como uma estrela. Apesar de 60% dos alunos indicarem a escola como principal fonte dos conhecimentos de astronomia seus conceitos ainda são inadequados havendo necessidade de aprimoramento da abordagem desses conteúdos pois apesar de popular a astronomia é veiculada de maneira pouco esclarecedora e com imprecissões.

  6. Observation of self-regulating response in Li xM yMn 2-yO 4 (M=Mn, Ni): A study using density functional theory

    NASA Astrophysics Data System (ADS)

    Ragavendran, K.; Sherwood, Daniel; Emmanuel, Bosco

    2009-02-01

    Density functional theory is used to understand the response of the transition metal-oxygen octahedra in Li xMn 2O 4 and Li xNi 0.5Mn 1.5O 4 to lithium intercalation and de-intercalation. Electronic structure computations on these compounds for x=0, 0.5 and 1 indicate that the 3d DOS of Mn is almost unaffected to variations in x. On the other hand, the oxygen 2p-DOS and to a lesser extent Ni 3d DOS are found to be sensitive to perturbation. The observations are explained on the grounds of self-regulating response, characteristic of systems having localized d states that communicate with a covalent manifold.

  7. [Slaves and citizens on Ilha Grande: the dawning of the Republic came slowly].

    PubMed

    Schnoor, Eduardo Cavalcanti

    2012-12-01

    The article introduces Ilha Grande, a district in the municipality of Angra dos Reis, and describes its role within the subsistence market economy and the memory of slavery as found in the Angra dos Reis Civil Records. According to the Almanaque Laemmert, this district had the largest number of slaves in the municipality as of the 1870s. Most owners had few slaves, and the majority of landholdings were small. The Angra dos Reis Civil Records did not forget slavery after May 1888. A number of birth and death notifications describe the persons in question as former slaves. Even in the twentieth century, clerks continued to describe various republican citizens as ex-slaves.

  8. Coulomb gap: how a metal film becomes an insulator

    PubMed

    Butko; DiTusa; Adams

    2000-02-14

    Electron tunneling measurements of the density of states (DOS) in ultrathin Be films reveal that a correlation gap mediates their insulating behavior. In films with sheet resistance R<5000 Omega the correlation singularity appears as the usual perturbative ln(V) zero bias anomaly (ZBA) in the DOS. As R is increased further, however, the ZBA grows and begins to dominate the DOS spectrum. This evolution continues until a nonperturbative |V| Efros-Shklovskii Coulomb gap spectrum finally emerges in the highest R films. Transport measurements of films which display this gap are well described by a universal variable range hopping law R(T) = (h/2e(2))exp(T0/T)(1/2).

  9. Transforming the Department of State: Adapting Operational Capacities and Capabilities to the Post-9/11 Reality, Evolving Interagency Responsibilities, and the Challenges of the 21st Century

    DTIC Science & Technology

    2006-04-14

    combination of the Strategic Plan, MPPs, and BPPs goals, along with performance, and accountability plans. Three other documents are inherently important...DOS Strategic Plan, Mission Performance Plan (MPP), Bureau Performance Plan ( BPP ). NSC representation, POLADs, Embassy Country Teams, and...the world. DOS Strategic Plan, Mission Performance Plan (MPP), Bureau Performance Plan ( BPP ). NSC representation, POLADs, Exchange

  10. 8 CFR 286.9 - Fee for processing applications and issuing documentation at land border Ports-of-Entry.

    Code of Federal Regulations, 2014 CFR

    2014-01-01

    ...), Arrival/Departure Record, for admission at a land border Port-of-Entry must remit the required fee for... Card, issued by the DOS, or a passport and combined B-1/B-2 visa and non-biometric BCC (or similar stamp in a passport) issued by the DOS, who is required to be issued Form I-94, Arrival/Departure Record...

  11. Interannual variability of dust-mass loading and composition of dust deposited on snow cover in the San Juan Mountains, CO, USA: Insights into effects on snow melt

    NASA Astrophysics Data System (ADS)

    Goldstein, H. L.; Reynolds, R. L.; Derry, J.; Kokaly, R. F.; Moskowitz, B. M.

    2017-12-01

    Dust deposited on snow cover (DOS) in the American West can enhance snow-melt rates and advance the timing of melting, which together can result in earlier-than-normal runoff and overall smaller late-season water supplies. Understanding DOS properties and how they affect the absorption of solar radiation can lead to improved snow-melt models by accounting for important dust components. Here, we report on the interannual variability of DOS-mass loading, particle size, organic matter, and iron mineralogy, and their correspondences to laboratory-measured reflectance of samples from the Swamp Angel Study Plot in the San Juan Mountains, Colorado, USA. Samples were collected near the end of spring in water year 2009 (WY09) and from WY11-WY16, when dust layers deposited throughout the year had merged into one layer at the snow surface. Dust-mass loading on snow ranged 2-64 g/m2, mostly as particles with median sizes of 13-33 micrometers. Average reflectance values of DOS varied little across total (0.4 to 2.50 µm) and visible (0.4 to 0.7 µm) wavelengths at 0.30-0.45 and 0.19-0.27, respectively. Reflectance values lacked correspondence to particle-size. Total reflectance values inversely corresponded to concentrations of (1) organic matter content (4-20 weight %; r2 = 0.71) that included forms of black carbon and locally derived material such as pollen, and (2) magnetite (0.05 to 0.13 weight %; r2 = 0.44). Magnetite may be a surrogate for related dark, light-absorbing minerals. Concentrations of crystalline ferric oxide minerals (hematite+goethite) based on magnetic properties at room-temperature did not show inverse association to visible reflectance values. These ferric oxide measures, however, did not account for the amounts of nano-sized ferric oxides known to exist in these samples. Quantification of such nano-sized particles is required to evaluate their possible effects on visible reflectance. Nonetheless, our results emphasize that reflectance values of year

  12. Is There a Disk of Satellites around the Milky Way?

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

    Maji, Moupiya; Zhu, Qirong; Li, Yuexing

    2017-07-01

    The “disk of satellites” (DoS) around the Milky Way is a highly debated topic with conflicting interpretations of observations and their theoretical models. We perform a comprehensive analysis of all of the dwarfs detected in the Milky Way and find that the DoS structure depends strongly on the plane identification method and the sample size. In particular, we demonstrate that a small sample size can artificially produce a highly anisotropic spatial distribution and a strong clustering of the angular momentum of the satellites. Moreover, we calculate the evolution of the 11 classical satellites with proper motion measurements and find thatmore » the thin DoS in which they currently reside is transient. Furthermore, we analyze two cosmological simulations using the same initial conditions of a Milky-Way-sized galaxy, an N -body run with dark matter only, and a hydrodynamic one with both baryonic and dark matter, and find that the hydrodynamic simulation produces a more anisotropic distribution of satellites than the N -body one. Our results suggest that an anisotropic distribution of satellites in galaxies can originate from baryonic processes in the hierarchical structure formation model, but the claimed highly flattened, coherently rotating DoS of the Milky Way may be biased by the small-number selection effect. These findings may help resolve the contradictory claims of DoS in galaxies and the discrepancy among numerical simulations.« less

  13. Transport and NMR characteristics of the skutterudite-related compound Ca3Rh4Sn13

    NASA Astrophysics Data System (ADS)

    Tseng, C. W.; Kuo, C. N.; Li, B. S.; Wang, L. M.; Gippius, A. A.; Kuo, Y. K.; Lue, C. S.

    2018-02-01

    We report the electronic properties of the Yb3Rh4Sn13-type single crystalline Ca3Rh4Sn13 by means of the electrical resistivity, Hall coefficient, Seebeck coefficient, thermal conductivity, as well as 119Sn nuclear magnetic resonance (NMR) measurements. The negative sign of the Hall coefficient and Seebeck coefficient at low temperatures suggests that the n-type carriers dominate the electrical transport in Ca3Rh4Sn13, in contrast to the observations in Sr3Rh4Sn13 which has a p-type conduction. Such a finding indicates a significant difference in the electronic features between these two stannides. Furthermore, we analyzed the temperature-dependent 119Sn NMR spin-lattice relaxation rate for Ca3Rh4Sn13, (Sr0.7Ca0.3)3Rh4Sn13, and Sr3Rh4Sn13 to examine the change of the electronic Fermi-level density of states (DOS) in (Sr1-xCax)3Rh4Sn13. It indicates that the Sn 5s partial Fermi-level DOS enhances with increasing the Ca content, being consistent with the trend of the superconducting temperature. Since the total Fermi-level DOS usually obeys the same trend of the partial Fermi-level DOS, the NMR analysis provides microscopic evidence for the correlation between the electronic DOS and superconductivity of the (Sr1-xCax)3Rh4Sn13 system.

  14. Percepção Astronômica de Alunos do Ensino Médio da Rede Estadual de São Paulo

    NASA Astrophysics Data System (ADS)

    de Oliveira, E. F.; Voelzke, M. R.; Amaral, L. H.

    2005-08-01

    Embora a astronomia seja uma das ciências mais antigas da humanidade e muitos dos conceitos astronômicos serem populares, principalmente nesta época de alta globalização do conhecimento por intermédio de eficientes meios de comunicação e de obtenção da informação, notadamente através da internet, observa-se que uma parcela significativa dos estudantes encontra-se à margem dessas informações. O presente trabalho visa analisar o nível de conhecimento básico dos alunos de Ensino Médio da rede estadual da cidade de Suzano quanto aos fenômenos astronômicos que os rodeiam, tais como a sucessão dos dias e das estações do ano, além de questioná-los sobre fatos genéricos tais como: quais são os astros que se encontram mais próximos do planeta Terra, o que vem a ser o Sol, o Big Bang, um ano-luz, uma estrela cadente, a estrela de Bélem e o que ocasionou a extinção dos dinossauros. Para tanto foi elaborado um formulário constando de questões de múltipla escolha, o qual foi aplicado no primeiro colegial noturno da Escola Estadual Batista Renzi. Num espaço amostral de 34 alunos constatou-se que apenas 29,4% compreendiam a sucessão dos dias da semana, que apenas 20,6% explicaram corretamente as estações do ano, que apenas 20,6% tinham idéia de quais são os objetos celestes mais próximos da Terra, em contraposição 67,6% sabiam classificar corretamente o Sol como estrela, 55,9% relacionavam o Big Bang à origem do universo, apenas 20,6% identificavam um ano-luz como unidade de distância, 32,4% reconheciam uma estrela cadente como meteoro, 41,2% consideravam a estrela de Belém como um cometa e 50,0% explicaram corretamente a extinção dos dinossauros. A presente análise será expandida para as demais classes de primeiro colegial, não somente do período noturno, mas também do diurno da Escola Estadual Batista Renzi, bem como o formulário será devidamente ampliado. Já nesta primeira fase nota-se claramente o pequeno discernimento

  15. Treating rheumatic diseases in pregnancy: dos and don'ts

    PubMed Central

    Lockshin, M D

    2006-01-01

    This paper presents a critical appraisal of the current evidence and recommendations regarding the use of pharmaceuticals and biologicals in pregnancy. Clinical experience is often at variance with published recommendations as studies have not taken into account the differences in physiology in the various stages of gestation and between animal and human pregnancies. Physicians should bear in mind that pregnancy causes disruption in several organ systems; the effects of an intervention may depend on the stage of gestation; and some effects of interventions in pregnancy may not manifest until adulthood. PMID:17038475

  16. Treating rheumatic diseases in pregnancy: dos and don'ts.

    PubMed

    Lockshin, M D

    2006-11-01

    This paper presents a critical appraisal of the current evidence and recommendations regarding the use of pharmaceuticals and biologicals in pregnancy. Clinical experience is often at variance with published recommendations as studies have not taken into account the differences in physiology in the various stages of gestation and between animal and human pregnancies. Physicians should bear in mind that pregnancy causes disruption in several organ systems; the effects of an intervention may depend on the stage of gestation; and some effects of interventions in pregnancy may not manifest until adulthood.

  17. DOS Design/Application Tools System/Segment Specification. Volume 3

    DTIC Science & Technology

    1990-09-01

    consume the same information to obtain that information without "manual" translation by people. Solving the information management problem effectively...and consumes ’ even more information than centralized development. Distributed systems cannot be developed successfully by experiment without...human intervention because all tools consume input from and produce output to the same repository. New tools are easily absorbed into the environment

  18. Dos and Don'ts in Pregnancy: Truths and Myths.

    PubMed

    Fox, Nathan S

    2018-04-01

    Pregnancy is a time of excitement and anxiety. The reality for pregnant women is that their actions could affect their pregnancies and their fetuses. As such, they need to know what they should and should not do to minimize risk and optimize outcomes. Whereas this advice used to come from doctors, a few books, and some family and friends, in the age of the internet, women are now bombarded with information and recommendations, which are often confusing at best and conflicting at worst. The objective of this review is to present current, evidence-based recommendations for some of the things that pregnant women should and should not routinely do during pregnancy.

  19. An Ounce of Prevention Beats a Pound of Cure: Resourcing the State Department to Defend the Nation

    DTIC Science & Technology

    2010-04-01

    17 The DOS lacks the resources to lead USG efforts .....................................................25 Summary...failing states 5) The DOS lacks the resources to lead USG efforts After compelling the reader to accept these five arguments, a disparity in US foreign...world‟s most powerful nation. The nation-states in which these terrorists operate lack the ability to monitor or disrupt the operations of these

  20. Precipitation Phenomena: Deformation and Aging. Proceedings of an International Conference Held in Conjunction with the 1988 World Materials Congress, Chicago, Illinois, USA, 24-30 September 1988

    DTIC Science & Technology

    1988-01-01

    corrosion and stress analytical methods. corrosion cracking (SCC) in certain aqueous 3 EXPERIMENTAL PROCEDURE treatments were performed using variable time...properly oriented with the applied uniaxial approach a comparable EPR-DOS in the control stress are influenced. Deformation may specimens. EPR-DOS values...corrosion and stress corrosion. Atteridge, Sensitization Development Deformation above 20% prior strain, however, in Austenitic Stainless Steel: II. induces

  1. User's guide for SYSTUM-1 (Version 2.0): A simulator of growth trends in young stands under management in California and Oregon

    Treesearch

    Martin W. Ritchie; Robert F. Powers

    1993-01-01

    SYSTUM-1 is an individual-tree/distance-independent simulator developed for use in young plantations in California and southern Oregon. The program was developed to run under the DOS operating system and requires DOS 3.0 or higher running on an 8086 or higher processor. The simulator is designed to provide a link with existing PC-based simulators (CACTOS and ORGANON)...

  2. 8 CFR 286.9 - Fee for processing applications and issuing documentation at land border Ports-of-Entry.

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    .../Departure Record, for admission at a land border Port-of-Entry must remit the required fee for issuance of... Card, issued by the DOS, or a passport and combined B-1/B-2 visa and non-biometric BCC (or similar stamp in a passport) issued by the DOS, who is required to be issued Form I-94, Arrival/Departure Record...

  3. 8 CFR 286.9 - Fee for processing applications and issuing documentation at land border Ports-of-Entry.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    .../Departure Record, for admission at a land border Port-of-Entry must remit the required fee for issuance of... Card, issued by the DOS, or a passport and combined B-1/B-2 visa and non-biometric BCC (or similar stamp in a passport) issued by the DOS, who is required to be issued Form I-94, Arrival/Departure Record...

  4. 8 CFR 286.9 - Fee for processing applications and issuing documentation at land border Ports-of-Entry.

    Code of Federal Regulations, 2013 CFR

    2013-01-01

    .../Departure Record, for admission at a land border Port-of-Entry must remit the required fee for issuance of... Card, issued by the DOS, or a passport and combined B-1/B-2 visa and non-biometric BCC (or similar stamp in a passport) issued by the DOS, who is required to be issued Form I-94, Arrival/Departure Record...

  5. 8 CFR 286.9 - Fee for processing applications and issuing documentation at land border Ports-of-Entry.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    .../Departure Record, for admission at a land border Port-of-Entry must remit the required fee for issuance of... Card, issued by the DOS, or a passport and combined B-1/B-2 visa and non-biometric BCC (or similar stamp in a passport) issued by the DOS, who is required to be issued Form I-94, Arrival/Departure Record...

  6. Tunneling study of cavity grade Nb : possible magnetic scattering at the surface.

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

    Prolier, T.; Zasadzinski, J. F.; Cooley, L.

    Tunneling spectroscopy was performed on Nb pieces prepared by the same processes used to etch and clean superconducting radio frequency (SRF) cavities. Air exposed, electropolished Nb exhibited a surface superconducting gap {Delta} = 1.55 meV, which is characteristic of a clean, bulk Nb. However, the tunneling density of states (DOS) was significantly broadened. The Nb pieces, which were treated with the same mild baking used to improve the Q slope in SRF cavities, reveal a sharper DOS. Good fits to the DOS were obtained by using the Shiba theory, suggesting that magnetic scattering of quasiparticles is the origin of themore » gapless surface superconductivity and a heretofore unrecognized contributor to the Q-slope problem of Nb SRF cavities.« less

  7. Development of a Response Planner using the UCT Algorithm for Cyber Defense

    DTIC Science & Technology

    2013-03-01

    writer2l, guess passwd r2l, imap r2l, ipsweep probe, land dos, loadmodule u2r, multihop r2l, neptune dos, nmap probe, perl u2r, phf r2l, pod dos, portsweep...2646 10 pod 201 11 back 956 12 guess passwd 53 Item Type Count 13 ftp write 8 14 multihop 7 15 rootkit 10 16 buffer overflow 30 17 imap 11 18...pod 0 0 0 87 6 11 0 0 64 33 0 0 0 0 k = back 908 0 0 0 0 0 0 0 0 0 47 0 1 0 l = guess passwd 0 0 0 42 3 0 1 0 0 0 0 5 1 0 m = buffer overflow 0 0 17

  8. Luminescent sensing of Cu2+, CrO42- and photocatalytic degradation of methyl violet by Zn(II) metal-organic framework (MOF) having 5,5‧-(1H-2,3,5-triazole-1,4-diyl)diisophthalic acid ligand

    NASA Astrophysics Data System (ADS)

    Wu, Weiping; Li, Baohong; Gu, Chuying; Wang, Jun; Singh, Amita; Kumar, Abhinav

    2017-11-01

    A porous Zn(II) metal-organic framework (MOF) [Zn(H2L)(4,4‧-bipy)0.5]n (1) has been selected and its luminescence sensing for cations and anions as well as the photocatalytic property against methyl violet have been explored. Luminescence studies indicated that 1 could be an efficient multifunctional fluorescent material for highly sensitive detection of metal cation Cu2+ and anions CrO42-. The luminescence intensity of 1 was found to decrease proportionately with increase in the concentration of Cu2+ and CrO42-. Furthermore, the photocatalytic property of 1 for degradation of the methyl violet (MV) have been explored and a possible photocatalytic mechanism have been proposed using density of states (DOS) and partial DOS (pDOS) calculations.

  9. Carbohydrates in diversity-oriented synthesis: challenges and opportunities.

    PubMed

    Lenci, E; Menchi, G; Trabocchi, A

    2016-01-21

    Over the last decade, Diversity-Oriented Synthesis (DOS) has become a new paradigm for developing large collections of structurally diverse small molecules as probes to investigate biological pathways, and to provide a larger array of the chemical space. Drug discovery and chemical biology are taking advantage of DOS approaches to exploit highly-diverse and complex molecular platforms, producing advances in both target and ligand discovery. In this view, carbohydrates are attractive building blocks for DOS libraries, due to their stereochemical diversity and high density of polar functional groups, thus offering many possibilities for chemical manipulation and scaffold decoration. This review will discuss research contributions and perspectives on the application of carbohydrate chemistry to explore the accessible chemical space through appendage, stereochemical and scaffold diversity.

  10. A comprehensive functional analysis of PTEN mutations: implications in tumor- and autism-related syndromes.

    PubMed

    Rodríguez-Escudero, Isabel; Oliver, María D; Andrés-Pons, Amparo; Molina, María; Cid, Víctor J; Pulido, Rafael

    2011-11-01

    The PTEN (phosphatase and tensin homolog) phosphatase is unique in mammals in terms of its tumor suppressor activity, exerted by dephosphorylation of the lipid second messenger PIP(3) (phosphatidylinositol 3,4,5-trisphosphate), which activates the phosphoinositide 3-kinase/Akt/mTOR (mammalian target of rapamycin) oncogenic pathway. Loss-of-function mutations in the PTEN gene are frequent in human cancer and in the germline of patients with PTEN hamartoma tumor-related syndromes (PHTSs). In addition, PTEN is mutated in patients with autism spectrum disorders (ASDs), although no functional information on these mutations is available. Here, we report a comprehensive in vivo functional analysis of human PTEN using a heterologous yeast reconstitution system. Ala-scanning mutagenesis at the catalytic loops of PTEN outlined the critical role of residues within the P-catalytic loop for PIP(3) phosphatase activity in vivo. PTEN mutations that mimic the P-catalytic loop of mammalian PTEN-like proteins (TPTE, TPIP, tensins and auxilins) affected PTEN function variably, whereas tumor- or PHTS-associated mutations targeting the PTEN P-loop produced complete loss of function. Conversely, Ala-substitutions, as well as tumor-related mutations at the WPD- and TI-catalytic loops, displayed partial activity in many cases. Interestingly, a tumor-related D92N mutation was partially active, supporting the notion that the PTEN Asp92 residue might not function as the catalytic general acid. The analysis of a panel of ASD-associated hereditary PTEN mutations revealed that most of them did not substantially abrogate PTEN activity in vivo, whereas most of PHTS-associated mutations did. Our findings reveal distinctive functional patterns among PTEN mutations found in tumors and in the germline of PHTS and ASD patients, which could be relevant for therapy.

  11. Germ-line mutations in the neurofibromatosis 2 gene: Correlations with disease severity and retinal abnormalities

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

    Parry, D.M.; Kaiser-Kupfer, M.; Eldridge, R.

    Neurofibromatosis 2 (NF2) features bilateral vestibular schwannomas, other benign neural tumors, and cataracts. Patients in some families develop many tumors at an early age and have rapid clinical progression, whereas in other families, patients may not have symptoms until much later and vestibular schwannomas may be the only tumors. The NF2 gene has been cloned from chromosome 22q; most identified germ-line mutations result in a truncated protein and severe NF2. To look for additional mutations and clinical correlations, we used SSCP analysis to screen DNA from 32 unrelated patients. We identified 20 different mutations in 21 patients (66%): 10 nonsensemore » mutations, 2 frameshifts, 7 splice-site mutations, and 1 large in-frame deletion. Clinical information on 47 patients from the 21 families included ages at onset and at diagnosis, numbers of meningiomas, spinal and skin tumors, and presence of cataracts and retinal abnormalities. We compared clinical findings in patients with nonsense or frameshift mutations to those with splice-site mutations. When each patient was considered as an independent random event, the two groups differed (P {le} .05) for nearly every variable. Patients with nonsense or frameshift mutations were younger at onset and at diagnosis and had a higher frequency and mean number of tumors, supporting the correlation between nonsense and frameshift mutations and severe NF2. When each family was considered as an independent random event, statistically significant differences between the two groups were observed only for mean ages at onset and at diagnosis. A larger data set is needed to resolve these discrepancies. We observed retinal hamartomas and/or epiretinal membranes in nine patients from five families with four different nonsense mutations. This finding, which may represent a new genotype-phenotype correlation, merits further study. 58 refs., 2 tabs.« less

  12. [Evolution of ideas and techniques, and future prospects in epilepsy surgery].

    PubMed

    Mathon, B; Bédos-Ulvin, L; Baulac, M; Dupont, S; Navarro, V; Carpentier, A; Cornu, P; Clemenceau, S

    2015-02-01

    The aim of this article was to review and evaluate the published literature related to the outcome of epilepsy surgery, while placing it in an historical perspective, and to describe the future prospects in this field. Temporal lobe surgery achieves seizure freedom in about 70% of cases. Seizure outcome is similar in the pediatric population. Extratemporal resections impart good results to 40% to 60% of patients, with a better prognosis in the case of frontal lobe surgery. Pediatric hemispherotomy leads to seizure control in about 80% of children. Radiosurgery used as a treatment for temporal mesial epilepsy has an outcome quite similar to that obtained with surgical resection, but provides a neuropsychological advantage. Radiosurgery is also effective in 60% of children treated for seizures related to hypothalamic hamartoma. Regarding palliative surgery, callosotomy and multiple subpial transections show satisfactory outcomes in over 60% of cases. Neuromodulation techniques (vagus nerve stimulation and bilateral stimulation of the anterior nucleus of the thalamus) allow a 50% reduction of seizures in half of patients. Transcranial magnetic stimulation combined with electroencephalography seems a promising technique because of its diagnostic, prognostic and therapeutic applications. Transcranial ultrasound stimulation, which can reversibly control neuronal activity, is also under consideration. Concerning neuromodulation, trigeminal nerve stimulation may become an alternative to vagus nerve stimulation; while other targets of deep brain stimulation are being evaluated. Also, the possibility of coupling SEEG seizure focus detection with concomitant laser or radiofrequency focus destruction is under development. Constant evolution of epilepsy surgery has improved patient outcomes over time. Current research and development axes suggest the continuation of this trend and a reduction of the invasiveness of surgical procedures. Copyright © 2014 Elsevier Masson SAS. All

  13. Germline disruption of Pten localization causes enhanced sex-dependent social motivation and increased glial production.

    PubMed

    Tilot, Amanda K; Gaugler, Mary K; Yu, Qi; Romigh, Todd; Yu, Wanfeng; Miller, Robert H; Frazier, Thomas W; Eng, Charis

    2014-06-15

    PTEN Hamartoma Tumor Syndrome (PHTS) is an autosomal-dominant genetic condition underlying a subset of autism spectrum disorder (ASD) with macrocephaly. Caused by germline mutations in PTEN, PHTS also causes increased risks of multiple cancers via dysregulation of the PI3K and MAPK signaling pathways. Conditional knockout models have shown that neural Pten regulates social behavior, proliferation and cell size. Although much is known about how the intracellular localization of PTEN regulates signaling in cancer cell lines, we know little of how PTEN localization influences normal brain physiology and behavior. To address this, we generated a germline knock-in mouse model of cytoplasm-predominant Pten and characterized its behavioral and cellular phenotypes. The homozygous Pten(m3m4) mice have decreased total Pten levels including a specific drop in nuclear Pten and exhibit region-specific increases in brain weight. The Pten(m3m4) model displays sex-specific increases in social motivation, poor balance and normal recognition memory-a profile reminiscent of some individuals with high functioning ASD. The cytoplasm-predominant protein caused cellular hypertrophy limited to the soma and led to increased NG2 cell proliferation and accumulation of glia. The animals also exhibit significant astrogliosis and microglial activation, indicating a neuroinflammatory phenotype. At the signaling level, Pten(m3m4) mice show brain region-specific differences in Akt activation. These results demonstrate that differing alterations to the same autism-linked gene can cause distinct behavioral profiles. The Pten(m3m4) model is the first murine model of inappropriately elevated social motivation in the context of normal cognition and may expand the range of autism-related behaviors replicated in animal models. © The Author 2014. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  14. The non-neuronal and nonmuscular effects of botulinum toxin: an opportunity for a deadly molecule to treat disease in the skin and beyond.

    PubMed

    Grando, S A; Zachary, C B

    2018-05-01

    There is growing evidence that botulinum neurotoxins (BoNTs) exhibit biological effects on various human cell types with a host of associated clinical implications. This review aims to provide an update on the non-neuronal and nonmuscular effects of botulinum toxin. We critically analysed recent reports on the structure and function of cellular signalling systems subserving biological effects of BoNTs. The BoNT receptors and intracellular targets are not unique for neurotransmission. They have been found in both neuronal and non-neuronal cells, but there are differences in how BoNT binds to, and acts on, neuronal vs. non-neuronal cells. The non-neuronal cells that express one or more BoNT/A-binding proteins, and/or cleavage target synaptosomal-associated protein 25, include: epidermal keratinocytes; mesenchymal stem cells from subcutaneous adipose; nasal mucosal cells; urothelial cells; intestinal, prostate and alveolar epithelial cells; breast cell lines; neutrophils; and macrophages. Serotype BoNT/A can also elicit specific biological effects in dermal fibroblasts, sebocytes and vascular endothelial cells. Nontraditional applications of BoNT have been reported for the treatment of the following dermatological conditions: hyperhidrosis, Hailey-Hailey disease, Darier disease, inversed psoriasis, aquagenic palmoplantar keratoderma, pachyonychia congenita, multiple eccrine hydrocystomas, eccrine angiomatous hamartoma, eccrine sweat gland naevi, congenital eccrine naevus, Raynaud phenomenon and cutaneous leiomyomas. Experimental studies have demonstrated the ability of BoNT/A to protect skin flaps, facilitate wound healing, decrease thickness of hypertrophic scars, produce an anti-ageing effect, improve a mouse model of psoriasiform dermatitis, and have also revealed extracutaneous effects of BoNT arising from its anti-inflammatory and anticancer properties. BoNTs have a much wider range of applications than originally understood, and the individual cellular responses

  15. PI3K/AKT pathway mutations cause a spectrum of brain malformations from megalencephaly to focal cortical dysplasia.

    PubMed

    Jansen, Laura A; Mirzaa, Ghayda M; Ishak, Gisele E; O'Roak, Brian J; Hiatt, Joseph B; Roden, William H; Gunter, Sonya A; Christian, Susan L; Collins, Sarah; Adams, Carissa; Rivière, Jean-Baptiste; St-Onge, Judith; Ojemann, Jeffrey G; Shendure, Jay; Hevner, Robert F; Dobyns, William B

    2015-06-01

    Malformations of cortical development containing dysplastic neuronal and glial elements, including hemimegalencephaly and focal cortical dysplasia, are common causes of intractable paediatric epilepsy. In this study we performed multiplex targeted sequencing of 10 genes in the PI3K/AKT pathway on brain tissue from 33 children who underwent surgical resection of dysplastic cortex for the treatment of intractable epilepsy. Sequencing results were correlated with clinical, imaging, pathological and immunohistological phenotypes. We identified mosaic activating mutations in PIK3CA and AKT3 in this cohort, including cancer-associated hotspot PIK3CA mutations in dysplastic megalencephaly, hemimegalencephaly, and focal cortical dysplasia type IIa. In addition, a germline PTEN mutation was identified in a male with hemimegalencephaly but no peripheral manifestations of the PTEN hamartoma tumour syndrome. A spectrum of clinical, imaging and pathological abnormalities was found in this cohort. While patients with more severe brain imaging abnormalities and systemic manifestations were more likely to have detected mutations, routine histopathological studies did not predict mutation status. In addition, elevated levels of phosphorylated S6 ribosomal protein were identified in both neurons and astrocytes of all hemimegalencephaly and focal cortical dysplasia type II specimens, regardless of the presence or absence of detected PI3K/AKT pathway mutations. In contrast, expression patterns of the T308 and S473 phosphorylated forms of AKT and in vitro AKT kinase activities discriminated between mutation-positive dysplasia cortex, mutation-negative dysplasia cortex, and non-dysplasia epilepsy cortex. Our findings identify PI3K/AKT pathway mutations as an important cause of epileptogenic brain malformations and establish megalencephaly, hemimegalencephaly, and focal cortical dysplasia as part of a single pathogenic spectrum. © The Author (2015). Published by Oxford University Press

  16. Distribution of cysts of Strongyluris sp. (Nematoda) in the pallial system of Achatina fulica Bowdich, 1822 from Vila Dois Rios and Vila do Abraão, Ilha Grande, Angra dos Reis, Rio de Janeiro.

    PubMed

    Oliveira, J L; Santos, S B

    2018-04-19

    This work aimed to assessing Strongyluris sp. cysts distribution pattern in the several inner organs from pallial system of Achatina fulica Bowdich, 1822. Also we verified if there is a relationship between the mollusk size and the number of specimens from parasites collected from two touristic villages in Ilha Grande (Angra dos Reis, Rio de Janeiro state): Vila Dois Rios (VDR) and Vila do Abraão (ABR). The samples were obtained through a field work conducted bimonthly during 2007, 2008, 2010, and 2011, at both locations. Height and width were measured from shells collected, and the all specimens were classified in different classes: class 1 - <4.0 cm, class 2 - 4.1-9.0 cm and class 3 - < 9.0 cm. After the specimens were dissected in order to find and count the number cysts in the pallial system. In specimens from both locations, the pulmonary and secondary veins showed a high number of cysts. No significance difference was found both in the abundance of cysts among the specimens in ABR (p=0.138) and VDR (p=0.181). Achatina fulica showed different intensities of cyst infection based on the size classes: the class-3 specimens, at both locations, showed the greatest cyst average (ABR Anova F= 3.8; p=0.02); (VDR T of Student T= -2.04; p=0.04). The results suggested that the highest number of cysts in the vascularized area in pallial system of A. fulica was a consequence of a greater hemolymph circulation in that area, delivering more nutrients for larvae development. We think that bigger individuals host a higher number of cysts, as they usually present a larger biomass and a larger area of the pallial system, allowing an efficient parasite colonization. Other possible explanation could be the long exposure of the molluscs of class 3 to the parasites, which allowed a longer time to the larvae to allocate themselves.

  17. Trap density of states in n-channel organic transistors: variable temperature characteristics and band transport

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

    Cho, Joung-min, E-mail: cho.j.ad@m.titech.ac.jp; Akiyama, Yuto; Kakinuma, Tomoyuki

    2013-10-15

    We have investigated trap density of states (trap DOS) in n-channel organic field-effect transistors based on N,N ’-bis(cyclohexyl)naphthalene diimide (Cy-NDI) and dimethyldicyanoquinonediimine (DMDCNQI). A new method is proposed to extract trap DOS from the Arrhenius plot of the temperature-dependent transconductance. Double exponential trap DOS are observed, in which Cy-NDI has considerable deep states, by contrast, DMDCNQI has substantial tail states. In addition, numerical simulation of the transistor characteristics has been conducted by assuming an exponential trap distribution and the interface approximation. Temperature dependence of transfer characteristics are well reproduced only using several parameters, and the trap DOS obtained from the simulatedmore » characteristics are in good agreement with the assumed trap DOS, indicating that our analysis is self-consistent. Although the experimentally obtained Meyer-Neldel temperature is related to the trap distribution width, the simulation satisfies the Meyer-Neldel rule only very phenomenologically. The simulation also reveals that the subthreshold swing is not always a good indicator of the total trap amount, because it also largely depends on the trap distribution width. Finally, band transport is explored from the simulation having a small number of traps. A crossing point of the transfer curves and negative activation energy above a certain gate voltage are observed in the simulated characteristics, where the critical V{sub G} above which band transport is realized is determined by the sum of the trapped and free charge states below the conduction band edge.« less

  18. Microbial contamination of dental unit waterlines and effect on quality of indoor air.

    PubMed

    Kadaifciler, Duygu Göksay; Cotuk, Aysin

    2014-06-01

    The microbiological quality in dental unit waterlines (DUWLs) is considered to be important because patients and dental staff with suppressed immune systems are regularly exposed to water and aerosols generated from dental units (DUs). Opportunistic pathogens like Pseudomonas, Legionella, Candida, and Aspergillus can be present in DUWLs, while during consultations, bioaerosols can be dispersed in the air, thus resulting in effects on microbiological quality of indoor air. This present study represents microbiological air and water quality in dental offices (DOs) and also concerns the relationship between the quality of DO air and dental unit water. This study aimed to assess both the microbial quality of dental unit water and the indoor air in 20 DOs and to survey the effect on the quality of the indoor air with the existing microorganisms in dental unit water. Fourteen out of 20 (70 %) DUWLs were found to be contaminated with a high number of aerobic mesophilic heterotrophic bacteria. In terms of bacterial air contamination levels, in 90 % of DOs, a medium level (<500 colony-forming units (CFU)/m(3)) of contamination was determined, while in terms of microfungal air contamination, in all DOs, a low level (<100 CFU/m(3)) of contamination was determined. Potential infection or allergen agents, such as Pseudomonas, Micrococcus, Staphylococcus, Alternaria, Cladosporium, Penicillium, Aspergillus, and Paecilomyces were isolated from water and air samples. This study's determination of contamination sources and evaluation of microbial load in DOs could contribute to the development of quality control methods in the future.

  19. Preparing to Win in a Complex World: Institutionalizing Interagency Cooperation at the Tactical Level

    DTIC Science & Technology

    2016-06-10

    inefficiencies have even escalated to cases where soldiers in the brigades experienced challenges and delays in obtaining official passports . Despite...USARAF and RAF BCTs supporting DoS coordinated and approved security cooperation activities, DoS passport procedures have denied many soldiers from...draw from, in order to support sometimes late notice security cooperation activities. As a result, brigades often 84 put in blanket official passport

  20. Role of the treating surgeon in the consent process for elective refractive surgery.

    PubMed

    Schallhorn, Steven C; Hannan, Stephen J; Teenan, David; Schallhorn, Julie M

    2016-01-01

    To compare patient's perception of consent quality, clinical and quality-of-life outcomes after laser vision correction (LVC) and refractive lens exchange (RLE) between patients who met their treating surgeon prior to the day of surgery (PDOS) or on the day of surgery (DOS). Retrospective, comparative case series. Optical Express, Glasgow, UK. Patients treated between October 2015 and June 2016 (3972 LVC and 979 RLE patients) who attended 1-day and 1-month postoperative aftercare and answered a questionnaire were included in this study. All patients had a thorough preoperative discussion with an optometrist, watched a video consent, and were provided with written information. Patients then had a verbal discussion with their treating surgeon either PDOS or on the DOS, according to patient preference. Preoperative and 1-month postoperative visual acuity, refraction, preoperative, 1-day and 1-month postoperative questionnaire were compared between DOS and PDOS patients. Multivariate regression model was developed to find factors associated with patient's perception of consent quality. Preoperatively, 8.0% of LVC and 17.1% of RLE patients elected to meet their surgeon ahead of the surgery day. In the LVC group, 97.5% of DOS and 97.2% of PDOS patients indicated they were properly consented for surgery ( P =0.77). In the RLE group, 97.0% of DOS and 97.0% of PDOS patients stated their consent process for surgery was adequate ( P =0.98). There was no statistically significant difference between DOS and PDOS patients in most of the postoperative clinical or questionnaire outcomes. Factors predictive of patient's satisfaction with consent quality were postoperative satisfaction with vision (46.7% of explained variance), difficulties with night driving, close-up vision or outdoor/sports activities (25.4%), visual phenomena (12.2%), dry eyes (7.5%), and patient's satisfaction with surgeon's care (8.2%). Perception of quality of consent was comparable between patients that