WorldWideScience

Sample records for haemorrhagic telangiectasia patients

  1. Hereditary haemorrhagic telangiectasia

    DEFF Research Database (Denmark)

    Kjeldsen, A D; Vase, P; Green, A

    1999-01-01

    Hereditary haemorrhagic telangiectasia (HHT) is a dominantly inherited disease characterized by telangiectatic lesions. The disease manifestations are variable and include epistaxis, gastrointestinal bleeding, pulmonary arteriovenous malformations and cerebral arteriovenous malformations. Early...

  2. Treatment of Laryngeal Telangiectatic Lesions in a Patient Diagnosed with Hereditary Haemorrhagic Telangiectasia

    DEFF Research Database (Denmark)

    Kjeldsen, Anette Drøhse; Printz, Trine; Slot Mehlum, Camilla

    2015-01-01

    Abstract We here present a case concerning a 69 year old female patient with Hereditary Haemorrhagic Telangiectasia (HHT). She was suffering from hoarseness due to a telangiectatic lesion on the right vocal cord. The lesion was treated with laser and the voice improved markedly, which is document...

  3. Quality of life in patients with hereditary haemorrhagic telangiectasia (HHT).

    Science.gov (United States)

    Zarrabeitia, Roberto; Fariñas-Álvarez, Concepción; Santibáñez, Miguel; Señaris, Blanca; Fontalba, Ana; Botella, Luisa María; Parra, José Antonio

    2017-01-23

    There are very few studies about general quality of life parameters, standards for the description of health status and comparison with general population data on patients with Hereditary hemorrhagic telangiectasia (HHT), a rare disease in which epistaxis is a cardinal symptom. To assess the quality of life in a population of Spanish patients with HHT and compare it with the general population. Between January 1 st 2005 and December 31 st 2013, 187 adult patients diagnosed with HHT who were admitted to the HHT Unit of the Hospital Sierrallana, completed on their first visit, the EuroQol 5D-3L (five dimensions and three levels) quality of life descriptive test and the visual analog scale (VAS). The numerical social index value was also determined and the subjective effect of the nasal epistaxis on their quality of life was estimated classified as mild, moderate or severe. Patients with HHT had greater problems than the general population in the five dimensions of the EuroQol 5D-3L, particularly considering pain/discomfort and anxiety/depression. In the VAS and the social index value, patients with HHT also scored lower than the general population, particularly older patients, males, and patients with HHT2. They also had values similar to those of populations with chronic illnesses. The subjective perception of the severity of epistaxis correlated strongly with the VAS and social index values. The quality of life of patients with HHT, estimated using the EuroQol 5D-3L scale, is affected across all dimensions. The scores are similar to those seen in cases of other chronic diseases. Older patients, males and the carriers of the ACVRL1 mutation generally have worse scores on these scales. The VAS and the social index value are index that correlate well with the severity of the clinical symptoms associated mainly with epistaxis.

  4. Clinical symptoms according to genotype amongst patients with hereditary haemorrhagic telangiectasia

    DEFF Research Database (Denmark)

    Kjeldsen, A D; Møller, T R; Brusgaard, K

    2005-01-01

    BACKGROUND: Hereditary haemorrhagic telangiectasia (HHT) is a dominantly inherited disease, characterized by a wide variety of clinical manifestations, including epistaxis, gastrointestinal (GI) bleeding, pulmonary arteriovenous malformations (PAVMs) and neurological symptoms. HHT is a genetically...... patients had experienced more severe GI bleeding than HHT2 patients. There was no significant difference in severity of epistaxis or age at debut. Finally the mortality over a 90-month observation period was not significantly increased....

  5. National mutation study among Danish patients with hereditary haemorrhagic telangiectasia

    DEFF Research Database (Denmark)

    Tørring, P M; Brusgaard, K; Ousager, L B

    2014-01-01

    carry mutations in the ENG, ACVRL1 or SMAD4 genes. Here, we report on the genetic heterogeneity in the Danish national HHT population and address the prevalence of pulmonary arteriovenous malformations (PAVM). Probands of 107 apparently unrelated families received genetic testing, including sequencing...... and multiplex ligation-dependent probe amplification (MLPA) analyses of ENG, ACVRL1 and SMAD4. These 107 families included 320 patients confirmed to have HHT either clinically or genetically. In 89% of the probands (n=95), a mutation was identified. We detected 64 unique mutations of which 27 (41%) were novel....... Large deletions were identified in ENG and ACVRL1. The prevalence of PAVM was 52.3% in patients with an ENG mutation and 12.9% in the ACVRL1 mutation carriers. We diagnosed 80% of the patients clinically, fulfilling the Curaçao criteria, and those remaining were diagnosed by genetic testing...

  6. Clinical features of Hereditary Haemorrhagic Telangiectasia

    NARCIS (Netherlands)

    Hosman, A.E.

    2017-01-01

    Hereditary Haemorrhagic Telangiectasia (HHT), also known as Rendu-Osler-Weber disease (ROW), is an autosomal dominant disease with multi-systemic vascular dysplasia characterized by mucocutaneous telangiectasia, arteriovenous malformations and recurrent spontaneous epistaxis (nosebleeds). Most cases

  7. Follow-up of Thalidomide treatment in patients with Hereditary Haemorrhagic Telangiectasia.

    Science.gov (United States)

    Hosman, A; Westermann, C J J; Snijder, R; Disch, F; Mummery, C L; Mager, J J

    2015-12-01

    Patients with a hereditary vascular disorder called Rendu-Osler-Weber syndrome (Hereditary Haemorrhagic Telangiectasia, HHT) haemorrhage easily due to weak-walled vessels. Haemorrhage in lungs or brain can be fatal but patients suffer most from chronic and prolonged nosebleeds (epistaxis), the frequency and intensity of which increases with age. Several years ago, it was discovered serendipitously that the drug Thalidomide had beneficial effects on the disease symptoms in several of a small group of HHT patients: epistaxis and the incidence of anaemia were reduced and patients required fewer blood transfusions. In addition, they reported a better quality of life. However, Thalidomide has significant negative side effects, including neuropathy and fatigue. We followed up all HHT patients in the Netherlands who had been taking Thalidomide at the time the original study was completed to find out (i) how many had continued taking Thalidomide and for how long (ii) the nature and severity of any side-effects and (iii) whether side-effects had influenced their decision to continue taking Thalidomide. Only a minority of patients had continued taking the drug despite its beneficial effects on their symptoms and that the side effects were the primary reason to stop. Despite symptom reduction, alternative treatments are still necessary for epistaxis in HHT patients and a large-scale clinical trial is not justified although incidental use in the most severely affected patients can be considered.

  8. Evidence-based management of epistaxis in hereditary haemorrhagic telangiectasia.

    Science.gov (United States)

    Syed, I; Sunkaraneni, V S

    2015-05-01

    There are currently no guidelines in the UK for the specific management of hereditary haemorrhagic telangiectasia related epistaxis. The authors aimed to review the literature and provide an algorithm for the management of hereditary haemorrhagic telangiectasia related epistaxis. The Medline and Embase databases were interrogated on 15 November 2013 using the search items 'hereditary haemorrhagic telangiectasia' (title), 'epistaxis' (title) and 'treatment' (title and abstract), and limiting the search to articles published in English. A total of 46 publications were identified, comprising 1 systematic review, 2 randomised, controlled trials, 27 case series, 9 case reports, 4 questionnaire studies and 3 in vitro studies. There is a lack of high-level evidence for the use of many of the available treatments for the specific management of epistaxis in hereditary haemorrhagic telangiectasia. Current management should be based on a multidisciplinary team approach involving both a hereditary haemorrhagic telangiectasia physician and an ENT surgeon, especially when systemic therapy is being considered. The suggested treatment algorithm considers that the severity of epistaxis merits intervention at different levels of the treatment ladder. The patient should be assessed using a reproducible validated assessment tool, for example an epistaxis severity score, to guide treatment. More research is required, particularly in the investigation of topical agents targeting the development and fragility of telangiectasiae in hereditary haemorrhagic telangiectasia.

  9. [Hereditary haemorrhagic telangiectasia diagnosed in connection with a traffic accident].

    Science.gov (United States)

    Sivapalan, Pradeesh; Demény, Ann Kathrin; Almind, Merete; Kjeldsen, Anette Drøhse

    2014-02-17

    Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant disorder characterized by vascular dysplasia and haemorrhage. It is manifested by mucocutaneous telangiec-tases and arteriovenous malformations in organs such as lungs, liver and brain. We present a case of HHT. A 16-year-old patient with a history of recurrent epistaxis was admitted to the local hospital with chest pain and desaturation. A CT scan revealed pulmonary arteriovenous malformations.

  10. Hereditary haemorrhagic telangiectasia: a cause of preventable morbidity and mortality.

    LENUS (Irish Health Repository)

    Brady, A P

    2012-01-31

    Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant condition whose effects are mediated through deficient blood vessel formation and regeneration, with multisystem involvement. Patients are usually aware of resulting skin telangiectasia and epistaxis, but are also exposed to dangers posed by occult vascular malformations in other organs. About 15-35% of HHT patients have pulmonary AVMs (PAVMs), 10% have cerebral AVMs (CAVMs), 25-33% suffer significant GI blood loss from GI tract telangiectasia, and an unknown but high percentage have liver involvement. In total, 10% of affected individuals die prematurely or suffer major disability from HHT, largely because of bleeding from CAVMs and PAVMs, or paradoxical embolization through PAVMs. Screening for and early intervention to treat occult PAVMs and CAVMs can largely eliminate these risks, and should be undertaken in a specialist centre. The National HHT Center in The Mercy University Hospital in Cork is the referral centre for HHT screening in Ireland.

  11. Global Gene Expression Profiling of Telangiectasial Tissue from Patients with Hereditary Haemorrhagic Telangiectasia

    DEFF Research Database (Denmark)

    Tørring, Pernille M; Larsen, Martin Jakob; Kjeldsen, Anette D

    2015-01-01

    and arteriovenous malformations in visceral organs, primarily the lungs, brain and liver. The most common symptom in HHT is epistaxis originating from nasal telangiectasia, which can be difficult to prevent and can lead to severe anaemia. The clinical manifestations of HHT are extremely variable, even within family...

  12. Neurovascular phenotypes in hereditary haemorrhagic telangiectasia patients according to age. Review of 50 consecutive patients aged 1 day-60 years

    International Nuclear Information System (INIS)

    Krings, T.; Ozanne, A.; Chng, S.M.; Alvarez, H.; Lasjaunias, P.L.; Rodesch, G.

    2005-01-01

    Hereditary haemorrhagic telangiectasia (HHT) is inherited as an autosomal dominant trait with varying penetrance and expressivity. Some of the most devastating consequences of this disease result from cerebral vascular malformations that manifest themselves in either arteriovenous fistulae (AVF), small nidus-type arteriovenous malformations (AVM) or micro-AVMs with a nidus less than 1 cm in size. The purpose of this study was to compare the phenotypes of CNS-manifestations of HHT with the age of the patient. The charts and angiographic films of 50 patients diagnosed with HHT according to the Curacao criteria were retrospectively evaluated concerning age of onset of symptoms, or, if not applicable of first consultation. The files were reviewed for clinical presentation, family and personal history, while the patients' angiograms were analysed with respect to the number of lesions (single and multiple), the location (superficial supratentorial, deep supratentorial, infratentorial, and spinal), and type of lesion (fistulous AVM, nidus-type AVM, and micro-AVM). A total of 75 central nervous system manifestations of HHT were found. Lesions included seven spinal cord AVFs that were all present in the paediatric age group (mean age: 2.2 years), 34 cerebral AV fistulae, all but two affected patients were less than 6 years (mean age 3.0). Sixteen nidus type AVMs (mean age: 23.1 years) and 18 micro-AVMs (mean age: 31.8 years) were found. HHT displays an age-related penetrance of clinical manifestations. Since members of the same family can present with completely different phenotypes of this disease there seems to be no relationship between the type of mutation and the phenotype of the disease. Since there seems to be a continuum of vascular abnormalities (from large fistulous areas to small AVMs and micro-AVMs) associated with HHT, the most likely determinating factor of the HHT phenotype is the timing of the revealing event in relation to the maturity of the vessel

  13. Bevacizumab: an option for refractory epistaxis in hereditary haemorrhagic telangiectasia.

    Science.gov (United States)

    Amann, Arno; Steiner, Normann; Gunsilius, Eberhard

    2015-08-01

    Recurrent epistaxis in hereditary haemorrhagic telangiectasia (HHT) patients significantly decreases their quality of life. Treatment in therapy refractory patients is limited although various options have been tested so far. Herein, one patient is described that was treated for HHT for over 20 years with only intermediate benefits. As epistaxis duration and frequency increased continuously, bevacizumab 5 mg/kg was administered every 2 weeks. During the time of treatment (six doses) and up to 3 month afterwards clinical symptoms, blood pressure, cardiac output, pulmonary arterial hypertension (PAH), bleeding duration and frequency were assessed as criteria for treatment benefit. Duration and frequency of epistaxis decreased immediately after the first application resulting in reduced need of blood transfusions. After completion of six cycles, a further decrease in frequency and duration of bleeding was noted. Cardiac output and PAH decreased or remained stable, respectively, during time and after treatment. No increase in blood pressure could be found but a significant increase in heart rate was experienced after completion of all six applications. Unfortunately, the patient died due to a cerebral abscess. Bevacizumab led to an improvement of HHT related epistaxis, refractory to other treatments.

  14. Neurologic Manifestation as Initial Presentation in a Case of Hereditary Haemorrhagic Telangiectasia

    Directory of Open Access Journals (Sweden)

    Yeow Kwan Teo

    2010-01-01

    Full Text Available Hereditary Haemorrhagic Telangiectasia (HHT, or Osler-Weber-Rendu syndrome is an uncommon autosomal dominant multi-organ condition of vascular dysplasias. We describe a 19 year old Indian female who presented with cerebral abscess secondary to paradoxical emboli from pulmonary arteriovenous malformations (PAVMs associated with HHT. Cerebral, pulmonary, hepatic and gastrointestinal involvement can be life-threatening and it is important to have lifelong follow-ups on these patients.

  15. Research on Potential Biomarkers in Hereditary Haemorrhagic Telangiectasia

    Directory of Open Access Journals (Sweden)

    Luisa Maria Botella

    2015-03-01

    Full Text Available Hereditary Hemorrhagic Telangiectasia (HHT is a genetically heterogeneous disorder, involving mutations in two predominant genes known as Endoglin (ENG; HHT1 and Activin receptor like kinase 1 (ACVRL1/ALK1; HHT2, as well as in some less frequent genes, such as MADH4/SMAD4 (JP-HHT or BMP9/GDF2 (HHT5. The diagnosis of HHT patients currently remains at the clinical level, according to the Curaçao criteria, whereas the molecular diagnosis is used to confirm or rule out suspected HHT cases, especially when a well characterized index case is present in the family or in an isolated population. Unfortunately, many suspected patients do not present a clear HHT diagnosis or do not show pathogenic mutations in HHT genes, prompting the need to investigate additional biomarkers of the disease. Here, several HHT biomarkers and novel methodological approaches developed during the last years will be reviewed. On one hand, products detected in plasma or serum samples: soluble proteins (VEGF, TGF-β1, soluble endoglin, angiopoietin-2 and microRNA variants (miR-27a, miR-205, miR-210. On the other hand, differential HHT gene expression fingerprinting, Next Generation Sequencing (NGS of a panel of genes involved in HHT, and infrared spectroscopy combined with Artificial Neural Network (ANN patterns will also be reviewed. All these biomarkers might help to improve and refine HHT diagnosis by distinguishing from the non-HHT population.

  16. Experience of the Irish National Centre for hereditary haemorrhagic telangiectasia 2003-2008.

    LENUS (Irish Health Repository)

    Ni Bhuachalla, C F

    2012-01-31

    Hereditary haemorrhagic telangiectasia (HHT) is a group of autosomal dominant disorders of vascular structure. The Irish National Centre for HHT at the Mercy University Hospital, Cork, Ireland was founded in 2003. From 2003 to 2008, screening of 164 patients with contrast echocardiography, thoracic computerised tomography (CT) and cerebral magnetic resonance imaging (MRI) has identified 88 patients with definite HHT, 72 (82%) of whom had epistaxis, 70 (80%) had telangiectasia and 81 (92%) had a first-degree relative with HHT. We sought to describe the manifestations of HHT in an Irish population and to determine differences between internationally reported data. The HHT patient database was analysed to describe demographics, clinical manifestations and interventional procedures performed in all referred patients. Contrast echocardiography and\\/or CT were performed in 86 patients with definite HHT, identifying 27 patients (31%) with pulmonary arteriovenous malformations (pAVMs). Nineteen patients with single or multiple pAVMs had 28 embolisation procedures performed, with 1-6 pAVMs embolised per procedure. Cerebral MRI was performed in 78 (89%) patients and 2 (2.3%) had cerebral arteriovenous malformations (cAVMs). HHT prevalence is thought to be 1 in 2500-8000, suggesting that there are many undiagnosed cases in Irish patients. Internationally published data suggest a prevalence of 15-35% for pAVMs and 10-23% for cAVMs in patients with HHT. While the prevalence of pAVMs in our group is consistent with these data, the prevalence of cAVMs is considerably lower, suggesting that Irish patients with HHT may differ genotypically and phenotypically from those in other countries.

  17. Pulmonary arteriovenous malformations in hereditary haemorrhagic telangiectasia. Correlations between computed tomography findings and cerebral complications

    Energy Technology Data Exchange (ETDEWEB)

    Etievant, Johan; Si-Mohamed, Salim; Vinurel, Nicolas; Revel, Didier [Hospices Civils de Lyon, Hopital Cardiologique Louis Pradel, Departement d' Imagerie Cardiaque et Thoracique, Diagnostique et Interventionnelle, Bron (France); Universite Claude Bernard Lyon 1, Villeurbanne (France); Dupuis-Girod, Sophie [Hospices Civils de Lyon, Hopital Femme-Mere-Enfant, Service de Genetique, Centre de Reference pour la maladie de Rendu-Osler, Lyon (France); Decullier, Evelyne [Universite Claude Bernard Lyon 1, Villeurbanne (France); Hospices Civils de Lyon, Pole Information Medicale Evaluation Recherche, Lyon (France); Gamondes, Delphine [Hospices Civils de Lyon, Hopital Cardiologique Louis Pradel, Departement d' Imagerie Cardiaque et Thoracique, Diagnostique et Interventionnelle, Bron (France); Khouatra, Chahera [Hospices Civils de Lyon, Hopital Cardiologique Louis Pradel, Service de pneumologie - Centre des Maladies Orphelines Pulmonaires, Lyon (France); Cottin, Vincent [Universite Claude Bernard Lyon 1, Villeurbanne (France); Hospices Civils de Lyon, Hopital Cardiologique Louis Pradel, Service de pneumologie - Centre des Maladies Orphelines Pulmonaires, Lyon (France)

    2018-03-15

    Computed tomography (CT) is the modality of choice to characterise pulmonary arteriovenous malformations (PAVMs) in patients with hereditary haemorrhagic telangiectasia (HHT). Our objective was to determine if CT findings were associated with frequency of brain abscess and ischaemic stroke. This retrospective study included patients with HHT-related PAVMs. CT results, i.e. PAVM presentation (unique, multiple, disseminated or diffuse), the number of PAVMs and the largest feeding artery size, were correlated to prevalence of ischaemic stroke and brain abscess. All CTs were reviewed in consensus by two radiologists. Of 170 patients, 73 patients had unique (42.9 %), 49 multiple (28.8 %), 36 disseminated (21.2 %) and 12 diffuse (7.1 %) PAVMs. Fifteen patients presented with brain abscess; 26 patients presented with ischaemic stroke. The number of PAVMs was significantly correlated with brain abscess (11.5 vs. 6.2, respectively; p=0.025). The mean diameter of the largest feeding artery was significantly correlated with ischaemic stroke frequency (4.9 vs. 3.2 mm, respectively; p=0.0098). The number of PAVMs correlated significantly with risk of brain abscess, and a larger feeding artery significantly with more ischaemic strokes. These findings can lead to a better recognition and management of the PAVMs at risk of cerebral complications. (orig.)

  18. Reproducibility of right-to-left shunt quantification using transthoracic contrast echocardiography in hereditary haemorrhagic telangiectasia.

    Science.gov (United States)

    Vorselaars, V M M; Velthuis, S; Huitema, M P; Hosman, A E; Westermann, C J J; Snijder, R J; Mager, J J; Post, M C

    2018-04-01

    Transthoracic contrast echocardiography (TTCE) is recommended for screening of pulmonary arteriovenous malformations (PAVMs) in hereditary haemorrhagic telangiectasia. Shunt quantification is used to find treatable PAVMs. So far, there has been no study investigating the reproducibility of this diagnostic test. Therefore, this study aimed to describe inter-observer and inter-injection variability of TTCE. We conducted a prospective single centre study. We included all consecutive persons screened for presence of PAVMs in association with hereditary haemorrhagic telangiectasia in 2015. The videos of two contrast injections per patient were divided and reviewed by two cardiologists blinded for patient data. Pulmonary right-to-left shunts were graded using a three-grade scale. Inter-observer and inter-injection agreement was calculated with κ statistics for the presence and grade of pulmonary right-to-left shunts. We included 107 persons (accounting for 214 injections) (49.5% male, mean age 45.0 ± 16.6 years). A pulmonary right-to-left shunt was present in 136 (63.6%) and 131 (61.2%) injections for observer 1 and 2, respectively. Inter-injection agreement for the presence of pulmonary right-to-left shunts was 0.96 (95% confidence interval (CI) 0.9-1.0) and 0.98 (95% CI 0.94-1.00) for observer 1 and 2, respectively. Inter-injection agreement for pulmonary right-to-left shunt grade was 0.96 (95% CI 0.93-0.99) and 0.95 (95% CI 0.92-0.98) respectively. There was disagreement in right-to-left shunt grade between the contrast injections in 11 patients (10.3%). Inter-observer variability for presence and grade of the pulmonary right-to-left shunt was 0.95 (95% CI 0.91-0.99) and 0.97 (95% CI 0.95-0.99) respectively. TTCE has an excellent inter-injection and inter-observer agreement for both the presence and grade of pulmonary right-to-left shunts.

  19. Endoplasmic reticulum quality control is involved in the mechanism of endoglin-mediated hereditary haemorrhagic telangiectasia.

    Directory of Open Access Journals (Sweden)

    Bassam R Ali

    Full Text Available Hereditary haemorrhagic telangiectasia (HHT is an autosomal dominant genetic condition affecting the vascular system and is characterised by epistaxis, arteriovenous malformations and mucocutaneous and gastrointestinal telangiectases. This disorder affects approximately 1 in 8,000 people worldwide. Significant morbidity is associated with this condition in affected individuals, and anaemia can be a consequence of repeated haemorrhages from telangiectasia in the gut and nose. In the majority of the cases reported, the condition is caused by mutations in either ACVRL1 or endoglin genes, which encode components of the TGF-beta signalling pathway. Numerous missense mutations in endoglin have been reported as causative defects for HHT but the exact underlying cellular mechanisms caused by these mutations have not been fully established despite data supporting a role for the endoplasmic reticulum (ER quality control machinery. For this reason, we examined the subcellular trafficking of twenty-five endoglin disease-causing missense mutations. The mutant proteins were expressed in HeLa and HEK293 cell lines, and their subcellular localizations were established by confocal fluorescence microscopy alongside the analysis of their N-glycosylation profiles. ER quality control was found to be responsible in eight (L32R, V49F, C53R, V125D, A160D, P165L, I271N and A308D out of eleven mutants located on the orphan extracellular domain in addition to two (C363Y and C382W out of thirteen mutants in the Zona Pellucida (ZP domain. In addition, a single intracellular domain missense mutant was examined and found to traffic predominantly to the plasma membrane. These findings support the notion of the involvement of the ER's quality control in the mechanism of a significant number, but not all, missense endoglin mutants found in HHT type 1 patients. Other mechanisms including loss of interactions with signalling partners as well as adverse effects on functional

  20. Efficacy and safety of thalidomide for the treatment of severe recurrent epistaxis in hereditary haemorrhagic telangiectasia: results of a non-randomised, single-centre, phase 2 study.

    Science.gov (United States)

    Invernizzi, Rosangela; Quaglia, Federica; Klersy, Catherine; Pagella, Fabio; Ornati, Federica; Chu, Francesco; Matti, Elina; Spinozzi, Giuseppe; Plumitallo, Sara; Grignani, Pierangela; Olivieri, Carla; Bastia, Raffaella; Bellistri, Francesca; Danesino, Cesare; Benazzo, Marco; Balduini, Carlo L

    2015-11-01

    Hereditary haemorrhagic telangiectasia is a genetic disease that leads to multiregional angiodysplasia. Severe recurrent epistaxis is the most common presentation, frequently leading to severe anaemia. Several therapeutic approaches have been investigated, but they are mostly palliative and have had variable results. We aimed to assess the efficacy of thalidomide for the reduction of epistaxis in patients with hereditary haemorrhagic telangiectasia that is refractory to standard therapy. We recruited patients aged 17 years or older with hereditary haemorrhagic telangiectasia who had severe recurrent epistaxis refractory to minimally invasive surgical procedures into an open-label, phase 2, non-randomised, single-centre study at IRCCS Policlinico San Matteo Foundation (Pavia, Italy). We gave patients thalidomide at a starting dose of 50 mg/day orally. If they had no response, we increased the thalidomide dose by 50 mg/day increments every 4 weeks, until a response was seen, up to a maximum dose of 200 mg/day. After patients had achieved a response, they continued treatment for 8-16 additional weeks. The primary endpoint was the efficacy of thalidomide measured as the percentage of patients who had reductions of at least one grade in the frequency, intensity, or duration of epistaxis. We followed up patients each month to assess epistaxis severity score and transfusion need, and any adverse events were reported. We included all patients who received any study drug and who participated in at least one post-baseline assessment in the primary efficacy population. The safety population consisted of all patients who received any dose of study treatment. This trial is registered with ClinicalTrials.gov, number NCT01485224. Between Dec 1, 2011, and May 12, 2014, we enrolled 31 patients. Median follow-up was 15·9 months (IQR 10·1-22·3). Three (10%, 95% CI 2-26) patients had a complete response, with bleeding stopped, 28 (90%, 95% CI 74-98) patients had partial responses

  1. Bladder Wall Telangiectasia in a Patient with Ataxia-Telangiectasia and How to Manage?

    Directory of Open Access Journals (Sweden)

    Fatma Deniz Aygün

    2015-01-01

    Full Text Available Ataxia-telangiectasia (A-T is a rare neurodegenerative, inherited disease causing severe morbidity. Oculocutaneous telangiectasias are almost constant findings among the affected cases as telangiectasia is considered the main clinical finding for diagnosis. Vascular abnormalities in organs have been reported infrequently but bladder wall telangiectasias are extremely rare. We aimed to report recurrent hemorrhage from bladder wall telangiectasia in a 9-year-old boy with A-T who had received intravenous cyclophosphamide for non-Hodgkin’s lymphoma. Since A-T patients are known to be more susceptible to chemical agents, we suggested that possibly cyclophosphamide was the drug which induced bladder wall injury in this patient.

  2. PI3 kinase inhibition improves vascular malformations in mouse models of hereditary haemorrhagic telangiectasia.

    Science.gov (United States)

    Ola, Roxana; Dubrac, Alexandre; Han, Jinah; Zhang, Feng; Fang, Jennifer S; Larrivée, Bruno; Lee, Monica; Urarte, Ana A; Kraehling, Jan R; Genet, Gael; Hirschi, Karen K; Sessa, William C; Canals, Francesc V; Graupera, Mariona; Yan, Minhong; Young, Lawrence H; Oh, Paul S; Eichmann, Anne

    2016-11-29

    Activin receptor-like kinase 1 (ALK1) is an endothelial serine-threonine kinase receptor for bone morphogenetic proteins (BMPs) 9 and 10. Inactivating mutations in the ALK1 gene cause hereditary haemorrhagic telangiectasia type 2 (HHT2), a disabling disease characterized by excessive angiogenesis with arteriovenous malformations (AVMs). Here we show that inducible, endothelial-specific homozygous Alk1 inactivation and BMP9/10 ligand blockade both lead to AVM formation in postnatal retinal vessels and internal organs including the gastrointestinal (GI) tract in mice. VEGF and PI3K/AKT signalling are increased on Alk1 deletion and BMP9/10 ligand blockade. Genetic deletion of the signal-transducing Vegfr2 receptor prevents excessive angiogenesis but does not fully revert AVM formation. In contrast, pharmacological PI3K inhibition efficiently prevents AVM formation and reverts established AVMs. Thus, Alk1 deletion leads to increased endothelial PI3K pathway activation that may be a novel target for the treatment of vascular lesions in HHT2.

  3. Hereditary haemorrhagic telangiectasia: study of hepatic vascular alterations with multi-detector row helical CT and reconstruction programs

    International Nuclear Information System (INIS)

    Memeo, Maurizio; Stabile Ianora, Amato Antonio; Scaldapane, Arnaldo; Rotondo, Antonio; Angelelli, Giuseppe; Suppressa, Patrizia; Cirulli, Anna; Sabba', Carlo

    2005-01-01

    Purpose: To evaluate hepatic alterations in patients affected by Hereditary Haemorrhagic Telangiectasia (HHT) by using multidetector row helical CT (MDCT) and new reconstruction programs. Materials and methods: An MDCT multiphasic study of the liver was performed in 105 consecutive patients: 89 considered to be affected by HHT and 16 with suspicion of disease alone. The scan delay was determined by using a test bolus of contrast material. The CT examination was performed with a triphasic technique (double arterial phase and portal venous phase). multiplanar and angiographic reconstructions were then obtained, and the images checked for the presence of shunts, hepatic perfusion disorders, vascular lesions (telangiectasis and large confluent vascular masses), indirect signs of portal hypertension, and anatomical vascular variants. Results: Hepatic vascular alterations were found in 78/105 cases (67/89) patients affected by HHT and 11/16 patients with clinical suspicion alone). Therefore HHT diagnosis was excluded in 5 patients. 78/100 (78%) patients with HHT had intrahepatic vascular alterations: arterioportal shunts in 40/78 (51.2%) arteriosystemic shunts in 16/78 (20.5%) and both shunt types in 22/78 (28.3%). Intraparenchymal perfusion disorders were found in 46/78 (58.9%) patients. Telangiectasis were recognised in 50/78 (64.1%) patients. Large confluent vascular masses (LCVMs) were identified in 20/78 (25.6%) patients. indirect signs of portal hypertension were found in 46/78 (58.9%) cases. Variant hepatic arterial anatomy was present in 38/100 cases (38%). Conclusions: Multiphasic MDCT and the new reconstruction programs enable the identification and characterisation of the complex vascular alterations typical of HHT [it

  4. Living with hereditary haemorrhagic telangiectasia: coping and psychological distress - a cross-sectional study.

    Science.gov (United States)

    Geirdal, Amy Østertun; Dheyauldeen, Sinan; Bachmann-Harildstad, Gregor; Heimdal, Ketil

    2013-02-01

    The purpose of this study was to examine the relationship between coping strategies measured by Coping Orientation to Problems Experienced Scale (COPE) and psychological distress measured by Hospital Anxiety and Depression Scale (HADS) and Becks Hopelessness Scale (BHS) in individuals living with Hereditary hemorrhagic telangiectasia (HHT) and to examine if coping strategies might have a mediating role between experienced illness and psychological distress. HHT is mainly caused by mutations in the ENG- or ALK1-genes and associated with a shorter life span. 90% of patients have recurrent nosebleeds. 66 individuals affected of HHT participated in this cross-sectional study, completing questions due to demographic variables, Experience of illness, COPE, BHS and HADS. X(2) test, bivariate correlations with Pearson r and hierarchical multiple regression were used using PASW 18. Experience of illness made the highest variance in anxiety, depression and hopelessness and the coping strategy "behavioral disengagement" seems to have a mediating role between nose bleedings, being afraid of complications, satisfied with life and psychological distress. Experience of illness is of big importance in psychological distress in individuals affected of HHT, and behavioral disengagement explained the actual relationship between experience of illness and psychological distress.

  5. Gastrointestinal bleeding in patients with hereditary hemorrhagic telangiectasia

    DEFF Research Database (Denmark)

    Kjeldsen, A D; Kjeldsen, J

    2000-01-01

    Gastrointestinal bleeding occurs in a number of patients with hereditary hemorrhagic telangiectasia (HHT) and may lead to a high transfusion need. The aim of this study was to estimate the occurrence and severity of gastrointestinal bleeding in a geographically well defined HHT population....

  6. Hereditary haemorrhagic telangiectasia: study of hepatic vascular alterations with multi-detector row helical CT and reconstruction programs; Telangiectasia emorragica ereditaria: TC multidetettore multifasica e programmi di ricostruzione nello studio delle alterazioni vascolari epatiche

    Energy Technology Data Exchange (ETDEWEB)

    Memeo, Maurizio; Stabile Ianora, Amato Antonio; Scaldapane, Arnaldo; Rotondo, Antonio; Angelelli, Giuseppe [Policlinico Universitario, Bari (Italy). DiMIMP Sezione di Diagnostica per Immagini; Suppressa, Patrizia; Cirulli, Anna; Sabba' , Carlo [Policlinico Universitario, Bari (Italy). Centro Interdipartimentale per lo studio dell' HHT

    2005-02-01

    Purpose: To evaluate hepatic alterations in patients affected by Hereditary Haemorrhagic Telangiectasia (HHT) by using multidetector row helical CT (MDCT) and new reconstruction programs. Materials and methods: An MDCT multiphasic study of the liver was performed in 105 consecutive patients: 89 considered to be affected by HHT and 16 with suspicion of disease alone. The scan delay was determined by using a test bolus of contrast material. The CT examination was performed with a triphasic technique (double arterial phase and portal venous phase). multiplanar and angiographic reconstructions were then obtained, and the images checked for the presence of shunts, hepatic perfusion disorders, vascular lesions (telangiectasis and large confluent vascular masses), indirect signs of portal hypertension, and anatomical vascular variants. Results: Hepatic vascular alterations were found in 78/105 cases (67/89) patients affected by HHT and 11/16 patients with clinical suspicion alone). Therefore HHT diagnosis was excluded in 5 patients. 78/100 (78%) patients with HHT had intrahepatic vascular alterations: arterioportal shunts in 40/78 (51.2%) arteriosystemic shunts in 16/78 (20.5%) and both shunt types in 22/78 (28.3%). Intraparenchymal perfusion disorders were found in 46/78 (58.9%) patients. Telangiectasis were recognised in 50/78 (64.1%) patients. Large confluent vascular masses (LCVMs) were identified in 20/78 (25.6%) patients. indirect signs of portal hypertension were found in 46/78 (58.9%) cases. Variant hepatic arterial anatomy was present in 38/100 cases (38%). Conclusions: Multiphasic MDCT and the new reconstruction programs enable the identification and characterisation of the complex vascular alterations typical of HHT. [Italian] Scopo: Valutare le alterazioni epatiche nei pazienti affetti da Telangiectasia Emorraica Ereditaria (TEE) utilizzando una TC multidetettore (TCMD) ed in nuovi programmi di ricostruzione. Materiale e metodi: E' stato eseguito uno

  7. Efficiency of laser treatment in patients with hereditary hemorrhagic telangiectasia

    DEFF Research Database (Denmark)

    Jørgensen, Gita; Lange, Bibi; Wanscher, Jens Højberg

    2011-01-01

    Earlier studies have shown the effect of laser treatment on epistaxis in patients with hereditary hemorrhagic telangiectasia (HHT). At the present time, only very few prospective trials have been performed, and many studies are based on patients' subjective assessment of the severity of epistaxis....... This prospective study measures the objective effect of laser treatment in HHT patients with mild to moderate epistaxis. We introduce an objective measure to assess the severity of epistaxis: the bleeding time (BT). Before and after treatment, the quality of life, as measured by the patient, was assessed...

  8. Motor pathway degeneration in young ataxia telangiectasia patients: A diffusion tractography study

    Directory of Open Access Journals (Sweden)

    Ishani Sahama

    2015-01-01

    Conclusions: Whole tract analysis of the corticomotor, corticospinal and somatosensory pathways in ataxia telangiectasia showed significant white matter degeneration along the entire length of motor circuits, highlighting that ataxia–telangiectasia gene mutation impacts the cerebellum and multiple other motor circuits in young patients.

  9. Risk of subarachnoid haemorrhage in first degree relatives of patients with subarachnoid haemorrhage

    DEFF Research Database (Denmark)

    Gaist, D; Vaeth, M; Tsiropoulos, I

    2000-01-01

    OBJECTIVE: To estimate the risk of occurrence of subarachnoid haemorrhage in first degree relatives (parents, siblings, children) of patients with subarachnoid haemorrhage. DESIGN: Population based cohort study using data from the Danish National Discharge Registry and the Central Person Registry......, standardised for age, sex, and calendar period. This process was repeated for patients discharged from neurosurgery units, as diagnoses from these wards had high validity (93%). RESULTS: 18 patients had a total of 19 first degree relatives with subarachnoid haemorrhage during the study period, corresponding...... to a standardised incidence ratio of 2.9 (95% confidence interval 1.9 to 4.6). Patients discharged from neurosurgery wards had a higher standardised incidence ratio (4.5, 2.7 to 7.3). CONCLUSIONS: First degree relatives of patients with subarachnoid haemorrhage have a threefold to fivefold increased risk...

  10. Hepatic and pancreatic involvement in hereditary hemorrhagic telangiectasia: quantitative and qualitative evaluation with 64-section CT in asymptomatic adult patients

    Energy Technology Data Exchange (ETDEWEB)

    Barral, Matthias; Sirol, Marc; Hamzi, Lounis; Gayat, Etienne; Boudiaf, Mourad [Hopital Lariboisiere-APHP, Department of Abdominal Imaging, Paris (France); Place, Vinciane [Hopital Lariboisiere-APHP, Department of Abdominal Imaging, Paris (France); Universite Diderot-Paris 7, UFR de Medecine, Paris (France); Borsik, Michel [Hopital Lariboisiere-APHP, Deparment of Ear, Nose and Throat, Paris (France); Soyer, Philippe [Hopital Lariboisiere-APHP, Department of Abdominal Imaging, Paris (France); Universite Diderot-Paris 7, UFR de Medecine, Paris (France); Unite 965 INSERM/Paris7, Hopital Lariboisiere-APHP, Paris (France)

    2012-01-15

    To analyse quantitatively and qualitatively asymptomatic hepatic and pancreatic involvement in hereditary haemorrhagic telangiectasia (HHT) using 64-section helical CT. The 64-section helical CT examinations of 19 patients with HHT (8 men, 11 women; mean age, 58.6 years) were quantitatively and qualitatively analysed and compared to those of 19 control subjects who were matched for age and sex. Comparisons were made using univariate analysis. Dilated and tortuous intrahepatic arterial branches was the most discriminating independent variable (P < 0.0001) and had the highest specificity (100%; 19/19; 95%CI: 82%-100%) and accuracy (97%; 37/38; 95%CI: 86%-100%) for the diagnosis of HHT. Heterogeneous enhancement of hepatic parenchyma, intrahepatic telangiectases, hepatic artery to hepatic vein shunting, hepatic artery enlargement (i.e. diameter > 6.5 mm) and portal vein enlargement (i.e. diameter > 13 mm) were other variables that strongly correlated with the presence of HHT. Intrapancreatic telangiectases and arteriovenous malformations were found in 42% and 16% of patients with HHT, respectively. Liver and pancreatic involvement in asymptomatic HHT patients is associated with myriad suggestive findings on 64-section helical CT. It can be anticipated that familiarity with these findings would result in more confident diagnosis of HHT. (orig.)

  11. Trends in monitoring patients with aneurysmal subarachnoid haemorrhage

    DEFF Research Database (Denmark)

    Springborg, J B; Frederiksen, H-J; Eskesen, V

    2005-01-01

    After aneurysmal subarachnoid haemorrhage (SAH), the clinical outcome depends upon the primary haemorrhage and a number of secondary insults in the acute post-haemorrhagic period. Some secondary insults are potentially preventable but prevention requires prompt recognition of cerebral or systemic...... implemented monitoring system provides answers but it also raises valuable new questions challenging our current understanding of the brain injury after SAH....... complications. Currently, several neuro-monitoring techniques are available; this review describes the most frequently used techniques and discusses indications for their use, and their value in diagnosis and prognosis. None of the techniques, when considered in isolation, has proved sufficient after SAH...... with the conventional monitoring systems, for example intracranial pressure measurements, transcranial Doppler ultrasound and modern neuro-imaging, direct assessment of biochemical markers by intracerebral microdialysis is promising in the advancement of neurointensive care of patients with SAH. A successfully...

  12. Establishment of cell lines derived from ataxia telangiectasia and xeroderma pigmentosum patients with high radiation sensitivity

    International Nuclear Information System (INIS)

    Hashimoto, Tomoko; Furuyama, Jun-ichi; Nakano, Yoshiro; Owada, M. Koji; Kakunaga, Takeo

    1986-01-01

    Four human fibroblast cell lines, three of which were derived from a patient with ataxia telangiectasia and the other from a patient with xeroderma pigmentosum, were established after transfection with cloned SV40 DNA. These 4 cell lines showed some phenotypes characteristic of neoplastically transformed cells, and had a human karyotype with heteromorphisms identical to those of the parental fibroblasts. Their sensitivity to the cytotoxic effects of γ-rays or ultraviolet irradiation was as high as those of their parental fibroblasts. (Auth.)

  13. Macular telangiectasia type 2 (MacTel) in a 34-year-old patient.

    Science.gov (United States)

    Nicolai, Heleen; Wirix, Mieke; Spielberg, Leigh; Leys, Anita

    2014-09-23

    We report macular telangiectasia type 2 (MacTel) in a 34-year-old man, the youngest patient so far published with MacTel type 2. The patient presented with metamorphopsia and impaired reading ability. Diagnosis was based on bilateral abnormal macular autofluorescence, perifoveal telangiectasia with fluorescein angiographic hyperfluorescence without cystoid oedema, a small foveal avascular zone, asymmetric configuration of the foveal pit, disruptions in the inner segment/outer segment layer and hyper-reflective haze and spots in the outer nuclear layer. Although MacTel usually manifests with a slowly progressive decrease in visual acuity in the fifth to seventh decades of life, younger patients may occasionally be diagnosed with the disease. Awareness of subtle signs of the condition is essential for early diagnosis. 2014 BMJ Publishing Group Ltd.

  14. Dysphonia and vocal fold telangiectasia in hereditary hemorrhagic telangiectasia.

    Science.gov (United States)

    Chang, Joseph; Yung, Katherine C

    2014-11-01

    This case report is the first documentation of dysphonia and vocal fold telangiectasia as a complication of hereditary hemorrhagic telangiectasia (HHT). Case report of a 40-year-old man with HHT presenting with 2 years of worsening hoarseness. Hoarseness corresponded with a period of anticoagulation. Endoscopy revealed vocal fold scarring, vocal fold telangiectasias, and plica ventricular is suggestive of previous submucosal vocal fold hemorrhage and subsequent counterproductive compensation with ventricular phonation. Hereditary hemorrhagic telangiectasia may present as dysphonia with vocal fold telangiectasias and place patients at risk of vocal fold hemorrhage. © The Author(s) 2014.

  15. Gastric outlet obstruction due to adenocarcinoma in a patient with Ataxia-Telangiectasia syndrome: a case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Hammond Sue

    2009-03-01

    Full Text Available Abstract Background Ataxia-Telangiectasia syndrome is characterized by progressive cerebellar dysfunction, conjuctival and cutaneous telangiectasias, severe immune deficiencies, premature aging and predisposition to cancer. Clinical and radiographic evaluation for malignancy in ataxia-telangiectasia patients is usually atypical, leading to delays in diagnosis. Case presentation We report the case of a 20 year old ataxia-telangiectasia patient with gastric adenocarcinoma that presented as complete gastric outlet obstruction. Conclusion A literature search of adenocarcinoma associated with ataxia-telangiectasia revealed 6 cases. All patients presented with non-specific gastrointestinal complaints suggestive of ulcer disease. Although there was no correlation between immunoglobulin levels and development of gastric adenocarcinoma, the presence of chronic gastritis and intestinal metaplasia seem to lead to the development of gastric adenocarcinoma. One should consider adenocarcinoma in any patient with ataxia-telangiectasia who presents with non-specific gastrointestinal complaints, since this can lead to earlier diagnosis.

  16. Hashimoto thyroiditis associated with ataxia telangiectasia.

    Science.gov (United States)

    Patiroglu, Turkan; Gungor, Hatice Eke; Unal, Ekrem; Kurtoglu, Selim; Yikilmaz, Ali; Patiroglu, Tahir

    2012-01-01

    Ataxia telangiectasia is a rare genetic disease characterized by neurological manifestations, infections, and cancers. In addition to these cardinal features, different autoimmune diseases can be seen in patients with ataxia telangiectasia. Although there were reports of positive autoimmune thyroid antibodies associated with ataxia telangiectasia, to our knowledge, we report the first cases of nodular Hashimoto thyroiditis in two patients with ataxia telangiectasia in the English medical literature. These cases illustrate that despite the rarity of nodular Hashimoto thyroiditis associated with ataxia telangiectasia, physicians should be aware of this possibility. Furthermore, thyroid examination of patient with ataxia telangiectasia is recommended for early diagnosis.

  17. Haemorrhagic cholecystitis in a newly anticoagulated patient.

    Science.gov (United States)

    Kinnear, Ned; Hennessey, Derek Barry; Thomas, Rebecca

    2017-04-12

    A 74-year-old man undergoing rehabilitation after pneumonia developed right upper quadrant abdominal pain. Five days earlier he had been commenced on apixaban for a new diagnosis of atrial fibrillation. Ultrasound and CT scans revealed an acalculous grossly thickened gallbladder, with high attenuation non-echogenic material both within and surrounding the structure. Active contrast extravasation was seen at the neck. On laparotomy, a perforated internally bleeding gallbladder containing a single calculus was found, with significant free blood within the abdomen. After cholecystectomy, the patient recovered slowly in hospital before nursing home placement. © 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.

  18. Embolotherapy for pulmonary arteriovenous malformations in patients with hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome)

    DEFF Research Database (Denmark)

    Andersen, P E; Kjeldsen, A D; Oxhøj, H

    1998-01-01

    experienced an improved functional level. One patient experienced severe pleurisy and another a rise in temperature following treatment, but otherwise no symptomatic complications were observed. CONCLUSION: Embolotherapy is a definitive treatment for PAVMs: it is very effective with a high success rate......PURPOSE: To evaluate the clinical results of embolization of pulmonary arteriovenous malformations (PAVMs) in patients with hereditary hemorrhagic telangiectasia (HHT), the Rendu-Osler-Weber syndrome. MATERIAL AND METHODS: Twelve patients in the county of Fyn, Denmark, were treated...... with transcatheter embolization of 20 PAVMs using 12 detachable silicone balloons and 26 steel coils. RESULTS: All PAVMs were completely occluded and we observed a significant rise in PaO2 after treatment and a significant decrease in right-to-left shunt estimated by contrast echocardiography. All patients...

  19. Sugammadex reversal of rocuronium-induced neuromuscular block in a patient with ataxia-telangiectasia

    International Nuclear Information System (INIS)

    Kang, E.; Jung, J.W.

    2015-01-01

    A 17-year-old adolescent with ataxia-telangiectasia was scheduled to have laparoscopic colectomy for a resection of colon cancer. He had symptoms and signs of dyspnea, generalized dystonia, dysmetria, ataxia, and telangiectasia on the orbit. General anesthesia was performed, and rocuronium 30 mg was administered for muscle relaxation. Deep neuromuscular block (post-tetanic count: 0-8) was maintained for 95 minutes without additional rocuronium. On completion of surgery, sugammadex 80 mg was injected and train-of-four ratio was 0.93 at 210 seconds after administration. The tracheal tube was removed 5 min after the end of surgery. He recovered full spontaneous respiration and voluntary movements within 1 minute after extubation. After the surgery, he transferred to the intensive care unit and discharged 14 days after the surgery without any concrete problem. The reversal of rocuronium induced neuromuscular block by sugammadex was fast, complete, and recovered to the initial preoperative level of neuromuscular function in this patient. (author)

  20. Ischaemic strokes in patients with pulmonary arteriovenous malformations and hereditary hemorrhagic telangiectasia: associations with iron deficiency and platelets.

    Directory of Open Access Journals (Sweden)

    Claire L Shovlin

    Full Text Available BACKGROUND: Pulmonary first pass filtration of particles marginally exceeding ∼7 µm (the size of a red blood cell is used routinely in diagnostics, and allows cellular aggregates forming or entering the circulation in the preceding cardiac cycle to lodge safely in pulmonary capillaries/arterioles. Pulmonary arteriovenous malformations compromise capillary bed filtration, and are commonly associated with ischaemic stroke. Cohorts with CT-scan evident malformations associated with the highest contrast echocardiographic shunt grades are known to be at higher stroke risk. Our goal was to identify within this broad grouping, which patients were at higher risk of stroke. METHODOLOGY: 497 consecutive patients with CT-proven pulmonary arteriovenous malformations due to hereditary haemorrhagic telangiectasia were studied. Relationships with radiologically-confirmed clinical ischaemic stroke were examined using logistic regression, receiver operating characteristic analyses, and platelet studies. PRINCIPAL FINDINGS: Sixty-one individuals (12.3% had acute, non-iatrogenic ischaemic clinical strokes at a median age of 52 (IQR 41-63 years. In crude and age-adjusted logistic regression, stroke risk was associated not with venous thromboemboli or conventional neurovascular risk factors, but with low serum iron (adjusted odds ratio 0.96 [95% confidence intervals 0.92, 1.00], and more weakly with low oxygen saturations reflecting a larger right-to-left shunt (adjusted OR 0.96 [0.92, 1.01]. For the same pulmonary arteriovenous malformations, the stroke risk would approximately double with serum iron 6 µmol/L compared to mid-normal range (7-27 µmol/L. Platelet studies confirmed overlooked data that iron deficiency is associated with exuberant platelet aggregation to serotonin (5HT, correcting following iron treatment. By MANOVA, adjusting for participant and 5HT, iron or ferritin explained 14% of the variance in log-transformed aggregation-rate (p = 0

  1. MDCT diagnostic work-up of patients with subarachnoid haemorrhage

    International Nuclear Information System (INIS)

    Phanis, S.; Nikolopoulos, P.; Veliou, K.; Tsampoulas, C.; Efremidis, S.

    2006-01-01

    Full text: Our presentation is intended to analyze our experience over the last two years, in the expeditious work up of patients with subarachnoid haemorrhage, as well as patients whose clinical symptoms combined with radiological findings are highly suspicious of an aneurysm, acute arterial occlusion, cerebral vascular malformation, venous occlusion, dissection of the internal carotid artery or vertebral artery. 276 CT Angiographies of the brain have been performed and recorded over the last two years in our Department, on a 16 Detector Philips MX8000IDT Computed Tomograph. The patients are referred to our Department from the Emergency Department of our Hospital, as well as from Hospitals all over Northwestern Greece. The CT Angiographies are performed with examination protocols, which we will analyze thoroughly in our presentation. The primary data are then studied in workstations away from the main console of the Computed Tomograph. The study of the primary data consists in multiplanar reconstructions with the Maximum Intensity Algorithm, as well as Volume Rendering and three Dimensional Reconstruction. The stages we follow for these procedures will be presented as well as the procedures for the removal of bone structures, with the intention of the best possible angiographic results. Of the 276 patients 40 were diagnosed with an aneurysm,19 with a venous angioma, 2 with a vascular malformation, 3 with venous occlusion and 2 with dissection, a total of 66/276 with aneurysms and vascular malformations. These patients were subsequently referred to the Neurosurgery Clinic or to the Department of Interventional Radiology for further treatment. The remaining 210 patients, underwent in the following days of their hospitalization a Digital Subtraction Angiography, and in 16 (7.6%) of them a small aneurysm, 3-5 mm was diagnosed. The aneurysm was detected in a second reading of the CT Angiography in 8 patients (3.8%). The CT Angiography in patients with subarachnoid

  2. Brainstem Auditory Evoked Potentials in Patients with Subarachnoid Haemorrhage

    Directory of Open Access Journals (Sweden)

    Mikhail Matveev

    2009-10-01

    Full Text Available Objective. The aim of the present study is to typify BAEPs configurations of patients with different location of lesions caused by subarachnoid haemorrhage (SAH and the ensuing complications, in view of assessing the auditory-brainstem system disturbance.Methods. The typization was performed by comparing BAEPs with standard patterns from two sets of types of BAEPs by ipsilateral and binaural stimulation and by cross-stimulation.Results. 94 BAEPs were used for collection of normal referential values: for the absolute latencies and the absolute amplitudes of waves I, II, III, IV and V; for inter-peak latencies I-III, II-III, III-V, I-V and II-V; for amplitude ratios I/V and III/V. 146 BAEPs of patients with mild SAH and 55 from patients with severe SAH, were typified. In 5 types of BAEPs out of a total of 11, the percentage of the potentials in patients with mild SAH and severe SAH differed significantly (p<0.01.Conclusions. The use of sets of types of BAEPs by ipsilateral, binaural and cross-stimulation correctly classifies the potentials in patients with mild and severe SAH.

  3. Hereditary hemorrhagic telangiectasia in children: endovascular treatment of neurovascular malformations. Results in 31 patients

    International Nuclear Information System (INIS)

    Krings, T.; Chng, S.M.; Ozanne, A.; Alvarez, H.; Lasjaunias, P.L.; Rodesch, G.

    2005-01-01

    Hereditary hemorrhagic telangiectasia (HHT) is a heterogeneous disease that can present with a variety of clinical manifestations. The neurovascular complications of this disease, especially in children, may be potentially devastating. The purpose of this article was to review the therapeutic results of endovascular treatment of neurovascular malformations in children. A total of 31 patients under the age of 16 were included in this retrospective analysis. All children were treated in a single center. Twenty children presented with 28 arteriovenous (AV) fistulae, including seven children with spinal AV fistulae and 14 children with cerebral AV fistulae (one child had both a spinal and cerebral fistulae). Eleven children had small nidus-type AV malformations. All embolizations were performed employing superselective glue injection. Follow-up ranged between 3 and 168 months (mean 66 months). A total of 115 feeding vessels were embolized in 81 single sessions, resulting in a mean overall occlusion rate of the malformation of 77.4% (ranging from 30 to 100%). Two of 31 patients (6.5%) died as a direct complication of the embolization procedure; two patients (6.5%) had a persistent new neurological deficit; eight patients (26.7%) were clinically unchanged following the procedure; in 13 patients (41.9%) an amelioration of symptoms but no cure could be achieved; and six patients (19.4%) were completely asymptomatic following the endovascular procedure. (orig.)

  4. Thalidomide for Epistaxis in Patients with Hereditary Hemorrhagic Telangiectasia: A Preliminary Study.

    Science.gov (United States)

    Fang, Jia; Chen, Xiaomeng; Zhu, Bijun; Ye, Haibo; Zhang, Weitian; Guan, Jian; Su, Kaiming

    2017-08-01

    To evaluate the effectiveness of thalidomide for epistaxis in hereditary hemorrhagic telangiectasia (HHT), 7 HHT patients with recurrent epistaxis were treated with thalidomide at an initial dose of 50 mg/d, gradually increasing to 100 mg/d if needed. The Epistaxis Severity Score (ESS) was used to evaluate the treatment effects. Patients reported that epistaxis improved 1 to 3 weeks after starting thalidomide. The mean ESS before treatment, at the end of treatment, and 3 months after stopping treatment was 5.03 ± 2.05, 0.90 ± 0.84 ( P = .003), and 1.98 ± 1.33 ( P = .006), respectively. Four patients reported mild to moderate side effects, including drowsiness, dizziness, constipation, nausea, and peripheral neuropathy. Two patients stopped the treatment because of adverse effects. Those results showed that thalidomide may be a treatment choice for recurrent epistaxis in HHT patients, although the side effects should be considered. Further study should focus on guidelines for dosing and course and investigate how to reduce the adverse effects.

  5. Are patients with severe epistaxis caused by hereditary hemorrhagic telangiectasia satisfied with nostril closure surgery?

    Science.gov (United States)

    Ichimura, Keiichi; Kikuchi, Hisashi; Imayoshi, Shoichiro; Yamauchi, Tomohiko; Ishikawa, Kotaro

    2012-02-01

    Recurrent epistaxis as a manifestation of hereditary hemorrhagic telangiectasia (HHT) is usually difficult to control. Although no treatment is regarded to be completely efficacious, nostril closure is considered a modality of choice for the most severe cases. The cessation of airflow resulting from this procedure can stop bleeding by minimizing risk factors. However, loss of nasal functions is a disadvantage of nostril closure. We conducted a questionnaire survey of patients who underwent nostril closure surgery, regarding the effects and disadvantages of the operation. Seven patients were asked questions on issues including frequency and severity of epistaxis pre- and post-operatively, satisfaction of treatment, and impairment in daily living activities. Most patients reported complete cessation of bleeding. Some still had bleeding, but the frequency and severity were far lower. No transfusions were required in any of the cases. Patients reported some disadvantages, for example, respiratory, olfactory, and phonatory issues. Six out of seven patients were very satisfied with the outcome of surgery. Nostril closure surgery can remarkably reduce frequency and volume of epistaxis. Our survey indicated that satisfactory results were achieved. However, difficulties caused by complete nasal obstruction varied. Thus, individualized coping strategies are required. Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.

  6. Hepatic artery embolization for treatment of patients with hereditary hemorrhagic telangiectasia and symptomatic hepatic vascular malformations

    Energy Technology Data Exchange (ETDEWEB)

    Chavan, Ajay [Hannover Medical School, Department of Diagnostic Radiology, Hannover (Germany); Klinikum Oldenburg, Department of Radiology and Nuclear Medicine, Oldenburg (Germany); Caselitz, Martin; Wagner, Siegfried; Manns, Michael [Hannover Medical School, Department of Gastroenterology and Hepatology, Hannover (Germany); Gratz, Karl-Friedrich [Hannover Medical School, Department of Nuclear Medicine, Hannover (Germany); Lotz, Joachim; Kirchhoff, Timm; Galanski, Michael [Hannover Medical School, Department of Diagnostic Radiology, Hannover (Germany); Piso, Plinio [Hannover Medical School, Department of Abdominal and Transplantation Surgery, Hannover (Germany)

    2004-11-01

    At present there is no established therapy for treating patients with hereditary hemorrhagic telangiectasia (HHT) and symptomatic hepatic involvement. We present the results of a prospective study with 15 consecutive patients who were treated with staged hepatic artery embolization (HAE). Branches of the hepatic artery were selectively catheterized and embolized in stages using polyvinyl alcohol particles (PVA) and platinum microcoils or steel macrocoils. Prophylactic antibiotics, analgesics and anti-emetics were administered after every embolization. Clinical symptomatology and cardiac output were assessed before and after therapy as well as at the end of follow-up (median 28 months; range 10-136 months). Five patients had abdominal pain and four patients had symptoms of portal hypertension. The cardiac output was raised in all patients, with cardiac failure being present in 11 patients. After treatment, pain resolved in all five patients, and portal hypertension improved in two of the four patients. The mean cardiac output decreased significantly (P<0.001) from 12.57{+-}3.27 l/min pre-treatment to 8.36{+-}2.60 l/min at the end of follow-up. Symptoms arising from cardiac failure resolved or improved markedly in all but one patient. Cholangitis and/or cholecystitis occurred in three patients of whom two required a cholecystectomy. One patient with pre-existent hepatic cirrhosis died as a complication of the procedure. Staged HAE yields long-term relief of clinical symptoms in patients with HHT and hepatic involvement. Patients with pre-existing hepatic cirrhosis may be poor candidates for HAE. (orig.)

  7. Quality of life in patients with hereditary hemorrhagic telangiectasia in Norway: a population based study.

    Science.gov (United States)

    Geirdal, Amy Østertun; Dheyauldeen, Sinan; Bachmann-Harildstad, Gregor; Heimdal, Ketil

    2012-06-01

    Hereditary hemorrhagic telangiectasia (HHT) is a rare, autosomal dominant disease characterized by the presence of recurrent epistaxis and small characteristic malformations of the peripheral blood vessels near the surface of the skin or mucosal linings. Arteriovenous malformations (AVM) of the lung, liver, and CNS are also known clinical findings. The purpose of this study was to examine quality of life (QoL) in patients with HHT in Norway. Sixty-six affected patients (39 women and 27 men) were included. QoL on overall-, health-related (HR-QoL), and disease-specific levels were measured with Cantril's Ladder (CL), Short Form 36 (SF-36), and a Symptom-specific QoL question in HHT patients (SFB-HHT-Q), respectively. Comparisons were made between patients and an age and gender adjusted normative sample from the Norwegian population (N = 990). Overall, the results reflected that several HHT disease-related variables were associated with reduced QoL on all three levels; overall QoL (CL), HR-QoL (SF36) as well as disease-specific QoL (SFB-HHT-Q), while demographic variables impacted HR-QoL in HHT patients. Compared to the normative sample, all subscales of SF36, but bodily pain, were significantly poorer in the HHT patients. HHT disease variables had the strongest association with QoL compared to demographic variables. The results substantiate that disease severity is associated with poorer QoL in this patients. Pain contributed independently to all levels of QoL. Copyright © 2012 Wiley Periodicals, Inc.

  8. Vertical transmission of macular telangiectasia type 2.

    Science.gov (United States)

    Delaere, Lien; Spielberg, Leigh; Leys, Anita M

    2012-01-01

    The purpose of this study was to report vertical transmission of macular telangiectasia type 2 and type 2 diabetes mellitus in 3 families. In this retrospective interventional case series, the charts of patients with inherited macular telangiectasia type 2 were reviewed. A large spectrum of presentations of macular telangiectasia type 2 was observed and has been studied with different techniques including best-corrected visual acuity, microperimetry, confocal blue reflectance fundus autofluorescence, fluorescein angiography, and time domain and spectral domain optical coherence tomography. Vertical transmission of macular telangiectasia type 2 and associated type 2 diabetes mellitus is described in 3 families. Symptomatic as well as asymptomatic eyes with macular telangiectasia type 2 were identified. In 2 families, a mother and son experienced visual loss and were diagnosed with macular telangiectasia type 2. All 4 patients had type 2 diabetes. Diabetic retinopathy was observed in one mother and her son. In the third family, the index patient was diagnosed macular telangiectasia type 2 after complaints of metamorphopsia. She and her family members had type 2 diabetes mellitus, and further screening of her family revealed familial macular telangiectasia type 2. None of the patients were treated for macular telangiectasia type 2. Macular telangiectasia type 2 may be more common than previously assumed, as vision can remain preserved and patients may go undiagnosed. Screening of family members is indicated, and detection of mild anomalies is possible using fundus autofluorescence and spectral domain optical coherence tomography.

  9. Body composition, muscle strength and hormonal status in patients with ataxia telangiectasia: a cohort study.

    Science.gov (United States)

    Pommerening, H; van Dullemen, S; Kieslich, M; Schubert, R; Zielen, S; Voss, S

    2015-12-09

    Ataxia-telangiectasia (A-T) is a devastating human autosomal recessive disorder that causes progressive cerebellar ataxia, immunodeficiency, premature aging, chromosomal instability and increased cancer risk. Affected patients show growth failure, poor weight gain, low body mass index (BMI), myopenia and increased fatigue during adolescence. The prevalence of alterations in body composition, muscle strength and hormonal status has not been well described in classical A-T patients. Additionally, no current guidelines are available for the assessment and management of these changes. We analyzed body composition, manual muscle strength and hormonal status in 25 A-T patients and 26 age-matched, healthy controls. Bioelectrical impedance analysis (BIA) was performed to evaluate the body composition, fat-free mass (FFM), body cell mass (BCM), extracellular matrix (ECM), phase angle (PhA), fat mass (FM) and ECM to BCM ratio. Manual muscle strength was measured using a hydraulic hand dynamometer. The BMI, FFM and PhA were significantly lower in A-T patients than in controls (BMI 16.56 ± 3.52 kg/m(2) vs. 19.86 ± 3.54 kg/m(2); Z-Score: -1.24 ± 1.29 vs. 0.05 ± 0.92, p body composition, characterized by depleted BMI, PhA and BCM; by the need to sit in a wheelchair; by altered hormone levels; and by poor muscle strength, is a major factor underlying disease progression and increased fatigue in A-T patients. ClinicalTrials.gov NCT02345200.

  10. Human iPSC-Derived Cerebellar Neurons from a Patient with Ataxia-Telangiectasia Reveal Disrupted Gene Regulatory Networks

    Directory of Open Access Journals (Sweden)

    Sam P. Nayler

    2017-10-01

    Full Text Available Ataxia-telangiectasia (A-T is a rare genetic disorder caused by loss of function of the ataxia-telangiectasia-mutated kinase and is characterized by a predisposition to cancer, pulmonary disease, immune deficiency and progressive degeneration of the cerebellum. As animal models do not faithfully recapitulate the neurological aspects, it remains unclear whether cerebellar degeneration is a neurodevelopmental or neurodegenerative phenotype. To address the necessity for a human model, we first assessed a previously published protocol for the ability to generate cerebellar neuronal cells, finding it gave rise to a population of precursors highly enriched for markers of the early hindbrain such as EN1 and GBX2, and later more mature cerebellar markers including PTF1α, MATH1, HOXB4, ZIC3, PAX6, and TUJ1. RNA sequencing was used to classify differentiated cerebellar neurons generated from integration-free A-T and control induced pluripotent stem cells. Comparison of RNA sequencing data with datasets from the Allen Brain Atlas reveals in vitro-derived cerebellar neurons are transcriptionally similar to discrete regions of the human cerebellum, and most closely resemble the cerebellum at 22 weeks post-conception. We show that patient-derived cerebellar neurons exhibit disrupted gene regulatory networks associated with synaptic vesicle dynamics and oxidative stress, offering the first molecular insights into early cerebellar pathogenesis of ataxia-telangiectasia.

  11. Intensive care management of patients with severe intracerebral haemorrhage after endovascular treatment of brain arteriovenous malformations

    International Nuclear Information System (INIS)

    Keller, E.; Yonekawa, Y.; Imhof, H.G.; Tanaka, M.; Valavanis, Anton

    2002-01-01

    We studied the impact of emergency neurosurgery and intensive care on the outcome for patients with severe intracerebral haemorrhage after endovascular treatment of brain arteriovenous malformations (AVMs). We reviewed the case notes of 18 patients with severe haemorrhage after embolisation of a brain AVM between 1986 and 2001. During this period the treatment changed: before 1993, these patients were not surgically treated, and they died, while after 1994, all patients underwent emergency surgery. We established a standardised protocol for emergency treatment and intensive care in May 1998, and emergency surgery was performed as soon as possible after the onset of symptoms of haemorrhage. Postoperative intensive care was according to a standardised regime. During these 15 years, 24 out of 605 patients undergoing 1066 interventions had a haemorrhage during or after the procedure, of which 18 were severe (3% of patients, 1.7% of interventions). All patients had a severe clinical deficit (mean Glasgow coma scale 4.2); eight had uni- or bilateral mydriasis. From 1989 to April 1998 four (31%) of 13 patients died, one (7.5%) remained in a vegetative state and eight (61.5%) made a good recovery. All five patients treated between 1998 and 2001 had a favourable outcome. The mean time from onset of the symptoms of haemorrhage to reaching the operation room was 129 min between 1989 and 1998 and 24 min between 1998 and 2001. Standardised emergency treatment and intensive care with early resuscitation, minimal radiological exploration before rapid surgery improved the outcome. A short time between the onset of the symptoms of haemorrhage and evacuation of the haematoma may be the most important factor for a favourable outcome. (orig.)

  12. The effects of epistaxis on health-related quality of life in patients with hereditary hemorrhagic telangiectasia.

    Science.gov (United States)

    Merlo, Christian A; Yin, Linda X; Hoag, Jeffrey B; Mitchell, Sally E; Reh, Douglas D

    2014-11-01

    Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease mainly characterized by epistaxis in more than 96% of patients. Recently, a validated questionnaire known as the HHT Epistaxis Severity Score (ESS) was developed. However, little is known about the relationship between epistaxis and quality of life. We hypothesize that epistaxis severity is a major factor predicting health-related quality of life (HR-QoL) in HHT patients. This is a cross-sectional study. The ESS questionnaire and Medical Outcomes Study 36-item short form (SF-36) were administered to subjects through an Internet survey. All participants had a definitive diagnosis of HHT through Curaçao criteria or genetic testing. Demographic information, genetics, and extensive histories were also collected. Descriptive analyses were performed with calculations of means and standard deviations (SDs) for continuous variables and proportions for categorical variables. Linear regressions were then performed to assess the association between HR-QoL and ESS. A total of 604 subjects participated between April and August 2008. All patients reported epistaxis, 285 (47.2%) had telangiectasias, and 545 (90.2%) had a family history of HHT; 167 (27.6%) patients had mild epistaxis (ESS epistaxis (≥4 ESS epistaxis (ESS ≥7). Patients with severe epistaxis had lower scores for both the Physical Component Summary (PCS) and the Mental Component Summary (MCS) of HR-QoL when compared to those with mild epistaxis (p epistaxis. © 2014 ARS-AAOA, LLC.

  13. Intracranial haemorrhage

    African Journals Online (AJOL)

    of the brain the haemorrhage is referred to as an .... The bleed is in the left basal ganglia most often originating in the putamen. Fig. 3. This 26-year-old patient presented with sudden-onset headache, right-sided ..... Early surgery versus initial.

  14. Probiotics and infective endocarditis in patients with hereditary hemorrhagic telangiectasia: a clinical case and a review of the literature.

    Science.gov (United States)

    Boumis, Evangelo; Capone, Alessandro; Galati, Vincenzo; Venditti, Carolina; Petrosillo, Nicola

    2018-02-01

    In the last decades, probiotics have been widely used as food supplements because of their putative beneficial health effects. They are generally considered safe but rare reports of serious infections caused by bacteria included in the definition of probiotics raise concerns on their potential pathogenic role in patients with particular predisposing factors. Patients with hereditary hemorrhagic telangiectasia (HHT) are exposed to infections because of telangiectasias and arteriovenous malformations (AVMs). We describe what is, to our knowledge, the first case of infective endocarditis (IE) caused by Lactobacillus rhamnosus in a patient with HHT. A systematic review of the relevant medical literature is presented. A patient with HHT and an aortic bioprosthesis was admitted because of prolonged fever not responding to antibiotics. The patient had a history of repeated serious infections with hospitalizations and prolonged use of antibiotics, and used to assume large amounts of different commercial products containing probiotics. Weeks before the onset of symptoms the patient had been treated with nasal packings and with surgical closure of a nasal bleeding site because of recurrent epistaxis. A diagnosis of IE of the aortic bioprosthesis was made. All blood coltures were positive for L. rhamnosus. The patients responded to a cycle of 6 weeks of amoxicillin/clavulanate plus gentamicin. A systematic review of IE linked to consumption of probiotics, and of infective endocarditis in patients with HHT was conducted. 10 cases of IE linked to probiotics consumption and 6 cases of IE in patients with HHT were found. Consumption of probiotics can pose a risk of serious infections in patients with particular predisposing factors. Patients with HHT can be considered at risk because of their predisposition to infections. Prophylaxis with antibiotics before nasal packings in patients with HHT can be considered.

  15. Erythropoietin in the cerebrospinal fluid of patients with aneurysmal subarachnoid haemorrhage originates from the brain

    DEFF Research Database (Denmark)

    Springborg, Jacob Bertram; Sonne, Bjarne; Frederiksen, Hans Jørgen

    2003-01-01

    Recent years' research has revealed a specific, neuroprotective erythropoietin (EPO) system in the central nervous system (CNS) that is upregulated by hypoxia. The presence and dynamics of EPO in the cerebrospinal fluid (CSF) of patients with subarachnoid haemorrhage (SAH) has not been investigated...

  16. Aneurysm occlusion in elderly patients with aneurysmal subarachnoid haemorrhage : a cost-utility analysis

    NARCIS (Netherlands)

    Koffijberg, H.; Buskens, E.; Rinkel, G. J. E.

    Background Aneurysm occlusion after subarachnoid haemorrhage (SAH) aims to improve outcome by reducing the rebleeding risk. With increasing age, overall prognosis decreases, and the complications of aneurysm occlusion increase. The balance of risks for aneurysm occlusion in elderly SAH patients in

  17. Amplatzer Vascular Plugs Versus Coils for Embolization of Pulmonary Arteriovenous Malformations in Patients with Hereditary Hemorrhagic Telangiectasia

    Energy Technology Data Exchange (ETDEWEB)

    Tau, Noam, E-mail: taunoam@gmail.com; Atar, Eliyahu [Rabin Medical Center – Beilinson and HaSharon Campuses, Department of Diagnostic Imaging (Israel); Mei-Zahav, Meir [Schneider Children’s Medical Center of Israel, Department of Pulmonology and National HHT Center (Israel); Bachar, Gil N. [Rabin Medical Center – Beilinson and HaSharon Campuses, Department of Diagnostic Imaging (Israel); Dagan, Tamir; Birk, Einat; Bruckheimer, Elchanan [Schneider Children’s Medical Center of Israel, Institute of Pediatric Cardiology (Israel)

    2016-08-15

    PurposeCoil embolization of pulmonary arteriovenous malformations (PAVMs) has a high re-canalization/re-perfusion rate. Embolization with Amplatzer plugs has been previously described, but the long-term efficacy is not established. This study reports the experience of a referral medical center with the use of coils and Amplatzer plugs for treating PAVMs in patients with hereditary hemorrhagic telangiectasia.MethodsThe study was approved by the Institutional Review Board with waiver of informed consent. The cohort included all patients who underwent PAVM embolization in 2004–2014 for whom follow-up imaging scans were available. The medical files were retrospectively reviewed for background data, embolization method (coils, Amplatzer plugs, both), and complications. Re-canalization of treated PAVMs was assessed from intrapulmonary angiograms (following percutaneous procedures) or computed tomography angiograms. Fisher’s exact test and Pearson Chi-squared test or t test were used for statistical analysis, with significance at p < 0.05.Results16 patients met the study criteria. Imaging scans were available for 63 of the total 110 PAVMs treated in 41 procedures. Coils were used for embolization in 37 PAVMs, Amplatzer plugs in 21, and both in five. Median follow-up time was 7.7 years (range 1.4–18.9). Re-canalization was detected in seven vessels, all treated with coils; there were no cases of re-canalization in plug-occluded vessels (p = 0.0413).ConclusionThe use of Amplatzer plugs for the embolization of PAVMs in patients with hemorrhagic telangiectasia is associated with a significantly lower rate of re-canalization of feeding vessels than coils. Long-term prospective studies are required to confirm these findings.

  18. Amplatzer Vascular Plugs Versus Coils for Embolization of Pulmonary Arteriovenous Malformations in Patients with Hereditary Hemorrhagic Telangiectasia

    International Nuclear Information System (INIS)

    Tau, Noam; Atar, Eliyahu; Mei-Zahav, Meir; Bachar, Gil N.; Dagan, Tamir; Birk, Einat; Bruckheimer, Elchanan

    2016-01-01

    PurposeCoil embolization of pulmonary arteriovenous malformations (PAVMs) has a high re-canalization/re-perfusion rate. Embolization with Amplatzer plugs has been previously described, but the long-term efficacy is not established. This study reports the experience of a referral medical center with the use of coils and Amplatzer plugs for treating PAVMs in patients with hereditary hemorrhagic telangiectasia.MethodsThe study was approved by the Institutional Review Board with waiver of informed consent. The cohort included all patients who underwent PAVM embolization in 2004–2014 for whom follow-up imaging scans were available. The medical files were retrospectively reviewed for background data, embolization method (coils, Amplatzer plugs, both), and complications. Re-canalization of treated PAVMs was assessed from intrapulmonary angiograms (following percutaneous procedures) or computed tomography angiograms. Fisher’s exact test and Pearson Chi-squared test or t test were used for statistical analysis, with significance at p < 0.05.Results16 patients met the study criteria. Imaging scans were available for 63 of the total 110 PAVMs treated in 41 procedures. Coils were used for embolization in 37 PAVMs, Amplatzer plugs in 21, and both in five. Median follow-up time was 7.7 years (range 1.4–18.9). Re-canalization was detected in seven vessels, all treated with coils; there were no cases of re-canalization in plug-occluded vessels (p = 0.0413).ConclusionThe use of Amplatzer plugs for the embolization of PAVMs in patients with hemorrhagic telangiectasia is associated with a significantly lower rate of re-canalization of feeding vessels than coils. Long-term prospective studies are required to confirm these findings.

  19. Pituitary apoplexy with optic tract oedema and haemorrhage in a patient with idiopathic thrombocytopenic purpura

    International Nuclear Information System (INIS)

    Lenthall, R.; Jaspan, T.

    2001-01-01

    Bilateral optic tract oedema, left optic tract haemorrhage and subarachnoid haemorrhage occurred in a 70-year-old man with pituitary apoplexy associated with idiopathic thrombocytopenic purpura. Left optic tract haemorrhage was confirmed on MRI. (orig.)

  20. The production and repair of double strand breaks in cells from normal humans and patients with ataxia telangiectasia

    International Nuclear Information System (INIS)

    Lehman, A.R.; Stevens, S.

    1977-01-01

    The production and repair of double strand breaks induced by γ-rays in the DNA of human fibroblasts have been measured by sedimentation in sucrose gradients under non-denaturing conditions. Unirradiated DNA formed a rapidly sedimenting gel. Low doses of radiation released freely sedimenting DNA molecules from this gel. Higher doses reduced the rate of sedimentation of the free DNA due to the introduction of double strand breaks. The breakage efficiency was 1 break/1.3x10 10 daltons of DNA/krad. Postirradiation incubation after a high dose of radiation resulted in an increase in molecular weight of the free DNA molecules, and after a low dose the rapidly-sedimenting gel was reformed. These data suggest that double strand breaks are repaired in human fibroblasts. No significant differences were found between fibroblasts from two normal donors and four patients with the radiosensitive disorder, ataxia telangiectasia, in either the production or repair of double strand breaks

  1. Patients with an inherited syndrome characterized by immunodeficiency, microcephaly, and chromosomal instability: genetic relationship to ataxia telangiectasia

    International Nuclear Information System (INIS)

    Jaspers, N.G.; Taalman, R.D.; Baan, C.

    1988-01-01

    Fibroblast cultures from six unrelated patients having a familial type of immunodeficiency combined with microcephaly, developmental delay, and chromosomal instability were studied with respect to their response to ionizing radiation. The cells from five of them resembled those from individuals with ataxia telangiectasia (AT) in that they were two to three times more radiosensitive on the basis of clonogenic cell survival. In addition, after exposure to either X-rays or bleomycin, they showed an inhibition of DNA replication that was less pronounced than that in normal cells and characteristic of AT fibroblasts. However, the patients are clinically very different from AT patients, not showing any signs of neurocutaneous symptoms. Genetic complementation studies in fused cells, with the radioresistant DNA synthesis used as a marker, showed that the patients' cells could complement representatives of all presently known AT complementation groups. Furthermore, they were shown to constitute a genetically heterogeneous group as well. It is concluded that these patients are similar to AT patients with respect to cytological parameters. The clinical differences between these patients and AT patients are a reflection of genetic heterogeneity. The data indicate that the patients suffer from a chromosome-instability syndrome that is distinct from AT

  2. Changes in self-efficacy, collective efficacy and patient outcome following interprofessional simulation training on postpartum haemorrhage.

    Science.gov (United States)

    Egenberg, Signe; Øian, Pål; Eggebø, Torbjørn Moe; Arsenovic, Mirjana Grujic; Bru, Lars Edvin

    2017-10-01

    To examine whether interprofessional simulation training on management of postpartum haemorrhage enhances self-efficacy and collective efficacy and reduces the blood transfusion rate after birth. Postpartum haemorrhage is a leading cause of maternal morbidity and mortality worldwide, although it is preventable in most cases. Interprofessional simulation training might help improve the competence of health professionals dealing with postpartum haemorrhage, and more information is needed to determine its potential. Multimethod, quasi-experimental, pre-post intervention design. Interprofessional simulation training on postpartum haemorrhage was implemented for midwives, obstetricians and auxiliary nurses in a university hospital. Training included realistic scenarios and debriefing, and a measurement scale for perceived postpartum haemorrhage-specific self-efficacy, and collective efficacy was developed and implemented. Red blood cell transfusion was used as the dependent variable for improved patient outcome pre-post intervention. Self-efficacy and collective efficacy levels were significantly increased after training. The overall red blood cell transfusion rate did not change, but there was a significant reduction in the use of ≥5 units of blood products related to severe bleeding after birth. The study contributes to new knowledge on how simulation training through mastery and vicarious experiences, verbal persuasion and psychophysiological state might enhance postpartum haemorrhage-specific self-efficacy and collective efficacy levels and thereby predict team performance. The significant reduction in severe postpartum haemorrhage after training, indicated by reduction in ≥5 units of blood transfusions, corresponds well with the improvement in collective efficacy, and might reflect the emphasis on collective efforts to counteract severe cases of postpartum haemorrhage. Interprofessional simulation training in teams may contribute to enhanced prevention and

  3. Henoch-Schönlein purpura with intracerebral haemorrhage in an adult patient: a case report

    Directory of Open Access Journals (Sweden)

    Karamadoukis Lazarus

    2008-06-01

    Full Text Available Abstract Introduction Henoch-Schönlein purpura is a small vessel vasculitis that affects mainly the skin, joints, gastrointestinal tract and kidneys. The central nervous system is also occasionally affected, although the majority of patients experience only mild symptoms such as headaches and behavioural changes. Intracerebral haemorrhage is a rare complication of Henoch-Schönlein purpura that so far has mainly been described in children and young adolescence. Case presentation We describe a 42-year-old man with Henoch-Schönlein purpura who developed an acute intracerebral haemorrhage that coincided with a reactivation of his vasculitis and the development of renal failure following discontinuation of steroids. In this patient, both the Henoch-Schönlein purpura and his neurological symptoms were successfully treated with intravenous cyclophosphamide and methylprednisolone, followed by a short course of oral cyclophosphamide and long-term oral prednisolone. His renal function also recovered sufficiently not to require renal replacement therapy. Conclusion The management of Henoch-Schönlein nephritis remains unclear, especially in the presence of severe complications such as intracerebral haemorrhage. We describe a successful outcome in such a patient.

  4. Influence of temporary nasal occlusion (tNO) on epistaxis frequency in patients with hereditary hemorrhagic telangiectasia (HHT).

    Science.gov (United States)

    Wirsching, Kornelia E C; Haubner, Frank; Kühnel, Thomas S

    2017-04-01

    The objective of the study was to evaluate the influence of temporary nasal occlusion (tNO) with hypoallergenic tape on the frequency and severity of epistaxis in patients with hereditary hemorrhagic telangiectasia (HHT). This prospective observational study included 20 HHT patients who were undergoing Nd:YAG laser therapy at regular intervals. Over a 3-month period, laser therapy was supplemented by tNO with hypoallergenic tape for 5 h/day on average. On a 0-10 numeric rating scale, the patients reported significantly greater satisfaction in epistaxis terms after tNO treatment, with mean scores of 5 before and 7 after 3-month tNO (p = 0.05). The Epistaxis Severity Score also fell significantly from a median of 3.59-2.43 after 3-month tNO compared with laser therapy alone (p = 0.01). The patients' hemoglobin levels remained stable during the study (median: 12.2 g/dL before tNO; median: 11.7 g/dL after tNO; p = 0.387). Overall, the present study confirms the positive influence of tNO on epistaxis in HHT patients and on subjective satisfaction. This simple and inexpensive strategy is therefore a helpful option, especially in addition to regular Nd:YAG laser therapy, and is recommended by the authors.

  5. Recurrent gastrointestinal hemorrhage in treatment with dasatinib in a patient showing SMAD4 mutation with acute lymphoblastic leukemia Philadelphia positive and juvenile polyposis hereditary hemorrhagic telangiectasia syndrome

    Directory of Open Access Journals (Sweden)

    Chiara Sartor

    2013-07-01

    Full Text Available We report a case of a patient affected by juvenile polyposis and hereditary hemorrhagic telangiectasia linked to a SMAD4 mutation who developed acute lymphoblastic leukemia positive for the Philadelphia chromosome translocation and with a complex karyotype. During the treatment with the tyrosine kinase inhibitor dasatinib the patient presented recurrent severe gastrointestinal hemorrhages linked to the genetic background and aggravated by thrombocytopenia.

  6. Acute variceal haemorrhage in the United Kingdom: patient characteristics, management and outcomes in a nationwide audit.

    Science.gov (United States)

    Jairath, Vipul; Rehal, Sunita; Logan, Richard; Kahan, Brennan; Hearnshaw, Sarah; Stanworth, Simon; Travis, Simon; Murphy, Michael; Palmer, Kelvin; Burroughs, Andrew

    2014-05-01

    Despite advances in treatment, acute variceal haemorrhage remains life-threatening. To describe contemporary characteristics, management and outcomes of patients with cirrhosis and acute variceal haemorrhage and risk factors for rebleeding and mortality. Multi-centre clinical audit conducted in 212 UK hospitals. In 526 cases of acute variceal haemorrhage, 66% underwent endoscopy within 24h with 64% (n=339) receiving endoscopic therapy. Prior to endoscopy, 57% (n=299) received proton pump inhibitors, 44% (n=232) vasopressors and 27% (n=144) antibiotics. 73% (n=386) received red cell transfusion, 35% (n=184) fresh frozen plasma and 14% (n=76) platelets, with widely varying transfusion thresholds. 26% (n=135) experienced further bleeding and 15% (n=80) died by day 30. The Model for End Stage Liver Disease score was the best predictor of mortality (area under the receiver operating curve=0.74, Prisk stratification tools are required to identify patients needing more intensive support. Copyright © 2013 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

  7. Abnormal peri-operative haemorrhage in asymptomatic patients is ...

    African Journals Online (AJOL)

    Accordingly, we undertook two studies to detertnine whether it could be justified in patients without a history of abnormal bleeding. In the first of these, 45 of 159 patients were excluded because of aspirin ingestion and a further 3 because of positive bleeding history so that prothrombin time, activated partial thromboplastin ...

  8. Nutritional status of patients with ataxia-telangiectasia: A case for early and ongoing nutrition support and intervention.

    Science.gov (United States)

    Ross, Lynda J; Capra, Sandra; Baguley, Brenton; Sinclair, Kate; Munro, Kate; Lewindon, Peter; Lavin, Martin

    2015-08-01

    Ataxia-telangiectasia (A-T) is a rare genomic syndrome resulting in severe disability. Chronic childhood disorders can profoundly influence growth and development. Nutrition-related issues in A-T are not well described, and there are no nutritional guidelines. This study investigated the nutrition-related characteristics and behaviours of Australian A-T patients attending a national clinic. A cross-sectional analysis of 13 A-T patients (nine females; aged: 4-23 years): nutritional status was assessed by anthropometric and body cell mass (BCM) calculations. Parents reported their child's diet history and physical and behavioural factors that affect nutrition including fatigue and need for assistance. Ten (77%) had short stature (height for age z scores nutritional barriers as chronic tiredness and the need for care giver assistance with meals. This study confirms profound malnutrition in Australian A-T patients. Poor intakes and diet quality suggest the need for early nutrition intervention. Ongoing support for families and early discussions on tube feeding are required to address changing needs in childhood and likely nutritional decline into adulthood. A prospective study is required to assess feasibility and effectiveness of nutrition interventions in young people with A-T. © 2015 The Authors. Journal of Paediatrics and Child Health © 2015 Paediatrics and Child Health Division (Royal Australasian College of Physicians).

  9. [Ataxia telangiectasia: review of 13 new cases].

    Science.gov (United States)

    Valbuena, O; Póo, P; Campistol, J; Vernet, A; Fernández-Alvarez, E; Sierra, I; Gean, E

    1996-01-01

    We report the review of 13 patients who were diagnosed of ataxia telangiectasia before 6 years of age. All of them manifested cerebelous ataxia, oculocutaneus telangiectasias (11), sinopulmonary infections (9), dystonia (9), oculomotor apraxia (9) and Burkitt linfoma (1). We analyse the most common presentation of the disease in early stages and the complementary studies performed. The prompt diagnosis allow us a better control of infections, malignant process and finally the possibility of genetic counseling.

  10. Abnormal peri-operative haemorrhage in asymptomatic patients is ...

    African Journals Online (AJOL)

    history so that prothrotnbin titne, activated partial throtnboplastin titne, bleeding .... was used as a screen in 12 000 patients and was unable to identify the ... 1982; 117: 48-51. 2. Barber A, Green D, Galluzzo T, Chung-Hsin T. The bleeding time.

  11. Frequency of cerebral infarction and haemorrhage in the patients of stroke

    International Nuclear Information System (INIS)

    Shah, A.N.; Ataullah, S.

    2009-01-01

    Stroke is rapidly developing phenomena of symptoms and signs of focal, and at times global, loss of cerebral function with no apparent cause other than that of vascular origin. The Objective was to know the frequency of cerebral infarction and haemorrhage in one hundred patients of stroke in a period of one year. Data was collected by consecutive sampling technique. Total one hundred patients of stroke were collected for the study. They were assessed through a detailed history of hypertension, diabetes mellitus, smoking, previous stroke, transient ischemic attack (TIA), previous myocardial infarction, angina, atrial fibrillation, alcohol intake, drugs used for hypertension/diabetes mellitus. Blood pressure was recorded at arrival and 24 hours after admission. There were 70% males and 30% females. Twenty percent of the patients were in the age range of 51-60 years, 26% of the patients were in the age range of 61-70 years and 18% were in the age range of 71-80 years. Cerebral infarction was present in 72% patients while cerebral haemorrhage was present in 28% patients. Hypertension was the most common risk factor among these stroke patients. Average blood pressure was 180/100 mmHg. Cerebral infarction is the commonest form of stroke. Hypertension is the leading risk factor in stroke patients. (author)

  12. Ataxia telangiectasia: a review

    Directory of Open Access Journals (Sweden)

    Cynthia Rothblum-Oviatt

    2016-11-01

    Full Text Available Abstract Definition of the disease Ataxia telangiectasia (A-T is an autosomal recessive disorder primarily characterized by cerebellar degeneration, telangiectasia, immunodeficiency, cancer susceptibility and radiation sensitivity. A-T is often referred to as a genome instability or DNA damage response syndrome. Epidemiology The world-wide prevalence of A-T is estimated to be between 1 in 40,000 and 1 in 100,000 live births. Clinical description A-T is a complex disorder with substantial variability in the severity of features between affected individuals, and at different ages. Neurological symptoms most often first appear in early childhood when children begin to sit or walk. They have immunological abnormalities including immunoglobulin and antibody deficiencies and lymphopenia. People with A-T have an increased predisposition for cancers, particularly of lymphoid origin. Pulmonary disease and problems with feeding, swallowing and nutrition are common, and there also may be dermatological and endocrine manifestations. Etiology A-T is caused by mutations in the ATM (Ataxia Telangiectasia, Mutated gene which encodes a protein of the same name. The primary role of the ATM protein is coordination of cellular signaling pathways in response to DNA double strand breaks, oxidative stress and other genotoxic stress. Diagnosis The diagnosis of A-T is usually suspected by the combination of neurologic clinical features (ataxia, abnormal control of eye movement, and postural instability with one or more of the following which may vary in their appearance: telangiectasia, frequent sinopulmonary infections and specific laboratory abnormalities (e.g. IgA deficiency, lymphopenia especially affecting T lymphocytes and increased alpha-fetoprotein levels. Because certain neurological features may arise later, a diagnosis of A-T should be carefully considered for any ataxic child with an otherwise elusive diagnosis. A diagnosis of A-T can be confirmed by the

  13. Splenic Involvement in Hereditary Hemorrhagic Telangiectasia

    Directory of Open Access Journals (Sweden)

    Susumu Takamatsu

    2016-01-01

    Full Text Available A 33-year-old man who presented with prolonged epigastric pain was referred to our hospital. He had experienced recurrent epistaxis and had a family history of hereditary hemorrhagic telangiectasia. Computed tomography and magnetic resonance imaging revealed splenomegaly and a 9 cm hypervascular mass in his spleen. Computed tomography also showed a pulmonary arteriovenous malformation and heterogeneous enhancement of the liver parenchyma, suggesting the presence of arteriosystemic shunts and telangiectases. Based on these findings, the patient was definitely diagnosed with hereditary hemorrhagic telangiectasia according to Curaçao criteria. He underwent splenectomy, and his symptoms disappeared after surgery. Pathological examination of the resected specimen revealed that the hypervascular lesion of the spleen was not a tumor but was composed of abnormal vessels associated with hereditary hemorrhagic telangiectasia. Symptomatic splenic involvement may be a rare manifestation of hereditary hemorrhagic telangiectasia but can be revealed by imaging modalities.

  14. Comparison of Selected Parameters of Redox Homeostasis in Patients with Ataxia-Telangiectasia and Nijmegen Breakage Syndrome

    Directory of Open Access Journals (Sweden)

    Barbara Pietrucha

    2017-01-01

    Full Text Available This study compared the antioxidant status and major lipophilic antioxidants in patients with ataxia-telangiectasia (AT and Nijmegen breakage syndrome (NBS. Total antioxidant status (TAS, total oxidant status (TOS, oxidative stress index (OSI, and concentrations of coenzyme Q10 (CoQ10 and vitamins A and E were estimated in the plasma of 22 patients with AT, 12 children with NBS, and the healthy controls. In AT patients, TAS (median 261.7 μmol/L was statistically lower but TOS (496.8 μmol/L was significantly elevated in comparison with the healthy group (312.7 μmol/L and 311.2 μmol/L, resp.. Tocopherol (0.8 μg/mL and CoQ10 (0.1 μg/mL were reduced in AT patients versus control (1.4 μg/mL and 0.3 μg/mL, resp.. NBS patients also displayed statistically lower TAS levels (290.3 μmol/L, while TOS (404.8 μmol/L was comparable to the controls. We found that in NBS patients retinol concentration (0.1 μg/mL was highly elevated and CoQ10 (0.1 μg/mL was significantly lower in comparison with those in the healthy group. Our study confirms disturbances in redox homeostasis in AT and NBS patients and indicates a need for diagnosing oxidative stress in those cases as a potential disease biomarker. Decreased CoQ10 concentration found in NBS and AT indicates a need for possible supplementation.

  15. Absceso intramedular en paciente con enfermedad de Rendu-Osler-Weber Spinal abscess in a patient with hereditary hemorrhagic telangiectasia

    Directory of Open Access Journals (Sweden)

    Lorena V Maldonado

    2007-12-01

    Full Text Available La enfermedad de Rendu-Osler-Weber es una enfermedad autosómica dominante que se manifiesta por telangiectasias en piel y mucosas y malformaciones arteriovenosas en diversos órganos. El compromiso neurológico puede ocurrir por la presencia de malformaciones arterio-venosas cerebrales, hemorragia intracraneal, y más habitualmente por accidentes cerebrovasculares isquémicos y abscesos cerebrales secundarios a embolia paradojal, en pacientes con malformaciones arteriovenosas pulmonares. El absceso intramedular es una entidad rara, inusual como complicación de la enfermedad de Rendu-Osler-Weber. Presentamos el caso de una mujer de 56 años de edad, con antecedentes familiares de enfermedad de Rendu-Osler- Weber, que ingresó a Terapia Intensiva con cuadriplejia aguda e hipoxemia, en quien se diagnosticó fístulas arteriovenosas pulmonares y un absceso intramedular cervical.Rendu-Osler-Weber syndrome is an autosomal dominant disorder characterized by multiple skin and mucosal telangiectasis and multiorgan arteriovenous malformations. Neurological manifestations may occur because of cerebral arteriovenous malformations, intracranial hemorrhage, and most commonly by ischemic stroke and brain abscess secondary to paradoxical embolization in patients with pulmonary arteriovenous malformations. Intramedullary abscess is a rare, unusual condition, in Rendu-Osler-Weber syndrome. We report the case of a 56 years old woman, with a familial history of Rendu-Osler-Weber syndrome, admitted to intensive care with acute quadriplegia and hypoxemia. Our diagnosis was pulmonary arteriovenous malformations and intramedullary abscess.

  16. [Paradoxical brain embolism mediated through a pulmonary arteriovenous malformation with hereditary hemorrhagic telangiectasia in a Japanese patient].

    Science.gov (United States)

    Takeda, June; Todo, Kenichi; Yamamoto, Shiro; Yamagami, Hiroshi; Kawamoto, Michi; Kohara, Nobuo

    2012-01-01

    We report a case of paradoxical brain embolism mediated through a pulmonary arteriovenous malformation (PAVM) with hereditary hemorrhagic telangiectasia (HHT). A 25-year-old right handed man was admitted to our hospital after sudden headache and visual field abnormality. In neurologic examinations, he had left superior-quadrantanopsia. Laboratory findings showed iron deficiency anemia. Diffusion weighted images disclosed a high-signal-intensity area in the right occipito-temporal lobe, and intraarterial digital subtraction cerebral angiography revealed occlusion of the right posterior cerebral artery. Transesophageal echocardiography revealed continuous right-to-left shunt. We confirmed his history of spontaneous recurrent epistaxis and the first-degree relatives with epistaxis or PAVM. A contrast enhanced CT scan of the chest revealed a PAVM. The diagnosis of paradoxical brain embolism mediated through the PAVM with HHT was, thus, established. The PAVM was occluded by using embolization coils successfully. In Asian countries, the prevalence of PAVM with HHT is thought to be lower than in European countries. We should carefully take medical and family histories, especially epistaxis, in a young stroke patient.

  17. Retroperitoneal Haematoma in a Patient with Dengue Haemorrhagic Fever: A Rare Case Report.

    Science.gov (United States)

    Singh, Jasminder; Singh, Harpreet; Sukhija, Gagandeep; Jagota, Ruchi; Bala, Saroj

    2016-11-01

    Dengue Haemorrhagic Fever (DHF) has diverse manifestations ranging from asymptomatic petechial skin haemorrhages to life threatening cerebral, pulmonary, gastrointestinal and genitourinary haemorrhages. However, the association of spontaneous retroperitoneal haematomas with DHF is not well documented in literature. We report a rare case of spontaneous retroperitoneal haematoma complicating DHF.

  18. Tranexamic acid for epistaxis in hereditary hemorrhagic telangiectasia patients: a European cross-over controlled trial in a rare disease.

    Science.gov (United States)

    Gaillard, S; Dupuis-Girod, S; Boutitie, F; Rivière, S; Morinière, S; Hatron, P-Y; Manfredi, G; Kaminsky, P; Capitaine, A-L; Roy, P; Gueyffier, F; Plauchu, H

    2014-09-01

    Hereditary hemorrhagic telangiectasia (HHT) is a genetic disorder associated with abnormal angiogenesis and disabling epistaxis. Tranexamic acid (TA) has been widely used in the treatment of these severe bleeds but with no properly designed trial. To demonstrate the efficacy of TA in epistaxis in HHT patients and to explore its safety of use. A randomized, placebo-controlled, double-blind, cross-over trial was conducted. Participants were randomized to receive TA (3 g a day) then placebo or the opposite sequence. The main analysis compared intra-individual mean duration of epistaxis under TA vs. placebo on a log scale. The primary outcome was the mean duration of epistaxis per month, assessed with specific grids to be completed by participants. The number of epistaxis episodes was recorded as a secondary outcome. A total of 118 randomized patients contributed to the statistical analysis. The mean duration of epistaxis per month was significantly shorter with TA than placebo (0.19 on the log scale; SD = 0.07; P = 0.005), corresponding to a decrease of 17.3% (15.7 min) in the duration of epistaxis per month (CI 95%, 5.5-27.6). The median number of epistaxis episodes per month was 22.1 episodes in the placebo arm vs. 23.3 episodes in the TA arm. No thrombophlebitis was observed. In the ATERO study, we demonstrated a significant decrease in the duration of epistaxis in HHT patients taking TA. No safety issues were recorded in our cohort of patients. © 2014 International Society on Thrombosis and Haemostasis.

  19. Magnetic resonance angiography compared to intra-arterial digital subtraction angiography in patients with subarachnoid haemorrhage

    International Nuclear Information System (INIS)

    Gouliamos, A.; Gotsis, E.; Vlahos, L.; Samara, C.; Kapsalaki, E.; Rologis, D.; Kapsalakis, Z.; Papavasiliou, C.

    1992-01-01

    In order to evaluate the sensitivity and specificity of magnetic resonance angiography (MRA) in spontaneous subarachnoid haemorrhage, 14 patients with recent haemorrhage verified by CT or lumbar puncture were investigated with both selective intra-arterial digital subtraction angiography (IA-DSA) and MRA by two independent teams, each having the same preangiographic information. The results were compared with each other and whenever possible (all positive cases except one) with those of surgical intervention. Seven patients were identified by MRA and IA-DSA as having a single aneurysm on the circle of Willis, 1 an aneurysm of the posterior inferior cerebellar artery 1 an aneurysm of the internal carotid artery (siphon) and 2 patients with two aneurysms on the circle of Willis. MRA and IA-DSA both failed to demonstrate aneurysms in 2 cases. Three patients had negative results on both methods and no surgical intervention was attempted. The aneurysms ranged from 0.3 to 1.5 cm in size. In most cases there was agreement between MRA and DSA, leading us to believe that, if the proper protocols are followed, MRA is a powerful alternative to other established methods in the detection of intracranial aneurysms. At this stage it will not replace IA-DSA prior to surgery, but the ability to obtain various projections using 3D MRA may improve surgical planning. (orig.)

  20. Magnetic resonance angiography compared to intra-arterial digital subtraction angiography in patients with subarachnoid haemorrhage

    Energy Technology Data Exchange (ETDEWEB)

    Gouliamos, A. (Dept. of Radiology, Athens Univ. (Greece)); Gotsis, E. (Diagnostic and Research Inst. Encephalos, Athens (Greece)); Vlahos, L. (Dept. of Radiology, Athens Univ. (Greece)); Samara, C. (Dept. of Radiology, Athens Univ. (Greece)); Kapsalaki, E. (Diagnostic and Research Inst. Encephalos, Athens (Greece)); Rologis, D. (Dept. of Neurosurgery, Athens General Hospital (Greece)); Kapsalakis, Z. (Diagnostic and Research Inst. Encephalos, Athens (Greece)); Papavasiliou, C. (Dept. of Radiology, Athens Univ. (Greece))

    1992-12-01

    In order to evaluate the sensitivity and specificity of magnetic resonance angiography (MRA) in spontaneous subarachnoid haemorrhage, 14 patients with recent haemorrhage verified by CT or lumbar puncture were investigated with both selective intra-arterial digital subtraction angiography (IA-DSA) and MRA by two independent teams, each having the same preangiographic information. The results were compared with each other and whenever possible (all positive cases except one) with those of surgical intervention. Seven patients were identified by MRA and IA-DSA as having a single aneurysm on the circle of Willis, 1 an aneurysm of the posterior inferior cerebellar artery 1 an aneurysm of the internal carotid artery (siphon) and 2 patients with two aneurysms on the circle of Willis. MRA and IA-DSA both failed to demonstrate aneurysms in 2 cases. Three patients had negative results on both methods and no surgical intervention was attempted. The aneurysms ranged from 0.3 to 1.5 cm in size. In most cases there was agreement between MRA and DSA, leading us to believe that, if the proper protocols are followed, MRA is a powerful alternative to other established methods in the detection of intracranial aneurysms. At this stage it will not replace IA-DSA prior to surgery, but the ability to obtain various projections using 3D MRA may improve surgical planning. (orig.)

  1. Superselective arterial embolisation with a liquid polyvinyl alcohol copolymer in patients with acute gastrointestinal haemorrhage

    Energy Technology Data Exchange (ETDEWEB)

    Lenhart, Markus; Schneider, Hans [Sozialstiftung Bamberg, Department of Diagnostic and Interventional Radiology, Bamberg (Germany); Paetzel, Christian [Klinikum Weiden, Department of Radiology, Weiden (Germany); Sackmann, Michael [Sozialstiftung Bamberg, Department of Gastroenterology, Bamberg (Germany); Jung, Ernst Michael; Schreyer, Andreas G.; Feuerbach, Stefan; Zorger, Niels [University of Regensburg, Department of Radiology, Regensburg (Germany)

    2010-08-15

    To evaluate the results of emergency embolisation in acute arterial bleeding of the gastrointestinal tract with a liquid polyvinyl alcohol copolymer from two centres. We retrospectively analysed 16 cases (15 patients) of acute arterial bleeding of the gastrointestinal tract where emergency embolotherapy was performed by using the copolymer when acute haemorrhage was not treatable with endoscopic techniques alone. Cause of haemorrhage and technical and clinical success were documented. Arterial embolotherapy was successful in all 16 cases. The technical success rate was 100%. The cause of bleeding was pancreatitis in four, graft-versus-host disease (GVHD) of the colon in three, malignancy in three, angiodysplasia in two, ulcer in two and panarteritis no dosa and trauma in one each. There were no procedure-related complications. No bowel necrosis occurred because of embolisation. In 13 cases, the patients were discharged in good condition (81%); the three patients with GVHD died because of the underlying disease. The copolymer seems to have great potential in embolotherapy of acute arterial gastrointestinal bleeding. In our series none of the patients had rebleeding at the site of embolisation and no clinically obvious bowel necrosis occurred. (orig.)

  2. Superselective arterial embolisation with a liquid polyvinyl alcohol copolymer in patients with acute gastrointestinal haemorrhage

    International Nuclear Information System (INIS)

    Lenhart, Markus; Schneider, Hans; Paetzel, Christian; Sackmann, Michael; Jung, Ernst Michael; Schreyer, Andreas G.; Feuerbach, Stefan; Zorger, Niels

    2010-01-01

    To evaluate the results of emergency embolisation in acute arterial bleeding of the gastrointestinal tract with a liquid polyvinyl alcohol copolymer from two centres. We retrospectively analysed 16 cases (15 patients) of acute arterial bleeding of the gastrointestinal tract where emergency embolotherapy was performed by using the copolymer when acute haemorrhage was not treatable with endoscopic techniques alone. Cause of haemorrhage and technical and clinical success were documented. Arterial embolotherapy was successful in all 16 cases. The technical success rate was 100%. The cause of bleeding was pancreatitis in four, graft-versus-host disease (GVHD) of the colon in three, malignancy in three, angiodysplasia in two, ulcer in two and panarteritis no dosa and trauma in one each. There were no procedure-related complications. No bowel necrosis occurred because of embolisation. In 13 cases, the patients were discharged in good condition (81%); the three patients with GVHD died because of the underlying disease. The copolymer seems to have great potential in embolotherapy of acute arterial gastrointestinal bleeding. In our series none of the patients had rebleeding at the site of embolisation and no clinically obvious bowel necrosis occurred. (orig.)

  3. Retrospective audit of the investigation of patients with suspected acute subarachnoid haemorrhage

    International Nuclear Information System (INIS)

    Muhammed, O.; Teubnet, D.; Jones, D.N.; Slavotinek, J.P.

    2010-01-01

    Recommended investigational care (RIC) of emergency department (ED) patients with suspected subarachnoid haemorrhage comprises lumbar puncture (LP) to detect xanthochromia if the preceding CT scan is negative. Retrospective audit of the investigational care of 100 consecutive ED patients presenting with possible subarachnoid haemorrhage. Of the 100 patients, 91 had negative CT, and 36 (39.6%) of these patients had an LP performed to detect xanthochromia (i.e. RIC). Fifty-five of 91 (60.4%) patients did not receive RIC. Of the 55 patients who did not receive RIC, 25 (45.5%) had a documented senior clinical decision not to perform an LP; 15 (27.3%) had no documented reason; five (9.1%) refused consent; two (3.6%) had an LP but no xanthochromia requested, one patient did not have an LP because of technical issues, six patients underwent CT angiography (CTA), and one patient underwent magnetic resonance angiography (MRA), in the absence of a LP, following a negative CT. Two patients underwent CTA following a negative xanthochromia result. Patients admitted to the emergency extended care unit had 6.85 times the odds of receiving RIC (95% CI 2.20-21.4). Fifty-five (55) of 91 (60%) ED patients did not receive RIC. Fifteen of the 55 did not have any documented justification for not performing an LP with xanthochromia testing. Admission to an emergency extended care unit was a predictor of receiving RIC. Inappropriate use of CTA and MRA was identified. These findings have important implications for patient safety. Multifaceted strategies are required to close this evidence-practice gap.

  4. Juvenil polypose-syndrom og hereditær hæmoragisk telangiektasi hos en patient med SMAD4-mutation

    DEFF Research Database (Denmark)

    Jelsig, Anne Marie; Tørring, Pernille Mathiesen; Wikman, Friedrik

    2014-01-01

    Germ line mutations in SMAD4 can cause both juvenile polyposis syndrome and hereditary haemorrhagic telangiectasia syndrome. In this case we present a 37-year-old man with a frameshift mutation in SMAD4. The patient had multiple polyps in the gastrointestinal tract and was diagnosed with colon ca...... cancer at the age of 21 and gastro-oesophageal junction cancer at the age of 37. Furthermore the patient had telangiectasias and recurrent epistaxis....

  5. The Increase of The Mean Platelet Volume in Patients With Intracerebral Haemorrhage

    Directory of Open Access Journals (Sweden)

    Adalet Arıkanoğlu

    2012-06-01

    Full Text Available OBJECTIVE: The mean platelet volume (MPV is a biomarker of platelet function and activity. The influence of platelet function disorders on the aetiology of intracerebral haemorrhages (ICH and mortality is not clear yet. The purpose of this study is to investigate the change in the MPV values in patients with ICH and to observe its influence on mortality in a retrospective manner. METHODS: Sixty-six patients with intracerebral haemorrhage (32 males, 34 females; mean age: 61.9± 16.9 were enrolled in the study. Patients with ICH were divided into two groups as those who died within the first 10 days and those who survived. The MPV values and the haematoma volumes were compared between the groups. Also, the MPV values and platelet counts of the patients with ICH were compared with the values of healthy volunteers from similar age and sex groups (27 males, 17 females; mean age: 59.9 ±3.2. RESULTS: The MPV values of the patients with ICH measured within 24 hours following the intracerebral haemorrhage (8.33 ± 1.27 fl/mL were statistically significantly higher than the MPV values of the control group (7.76 ± 1.14 fl/mL (p=0.018. The platelet counts of the patients with ICH also measured within the first 24 hours (235.8±94.9 x103/mL were statistically significantly lower than the platelet counts of the control group (279.1 ± 94.9 x103/mL (p=0.022. No statistically significant difference in terms of the MPV values and platelet counts was observed between the patients with ICH who died within the first 10 days and those who survived (p>0.05. However, the difference observed in the haematoma volume between the patients with ICH who died within the first 10 days (31.1 ±33.7 ml and those who survived (8.7± 13.4 ml was statistically significant (p<0.001. No correlation was found between the haematoma volume and the MPV value in the patients with ICH. CONCLUSION: The increase observed in the mean platelet volume in patients with ICH may point to a

  6. Very early disease manifestations of macular telangiectasia type 2

    NARCIS (Netherlands)

    Issa, P.C.; Heeren, T.F.C.; Kupitz, E.H.; Holz, F.G.; Berendschot, T.T.J.M.

    Background: To report very early morphologic and functional alterations in patients with macular telangiectasia type 2. Methods: Patients with asymmetric disease manifestations, in whom retinal alterations characteristic for macular telangiectasia type 2 were present in one but not in the apparently

  7. [Patients with subarachnoid haemorrhage in poor grade neurological status: Study of prognostic factors].

    Science.gov (United States)

    Sosa-Pérez, Coralia; Morera-Molina, Jesús; Espino-Postigo, Carlos; Jiménez-O'Shanahan, Aruma

    2015-01-01

    To evaluate and predict factors influencing prognosis and/or clinical outcome at 6 months in patients with spontaneous subarachnoid haemorrhage, World Federation of Neurosurgical Societies (WFNS) grades iv and v. This was a retrospective study of a consecutive series of 394 patients admitted to our hospital with clinical and radiological diagnosis of spontaneous subarachnoid haemorrhage, from 1 January 1999 to 30 June 2009. We selected 121 patients who met the criteria of being in WFNS grades iv or v before treatment; 3 patients were excluded due to loss of tracking. The outcome variable was assessed 6 months after the event using the Glasgow Outcome Scale. A P value<.05 was considered statistically significant. One hundred and twenty-one patients were included in the statistical analysis. The average age of the patients in the series was 54 years (14-92). Patients who had a mean Glasgow Coma Scale lower than 7 points (P<.0001), those who were grade v (P<.0001) in the pre-treatment WFNS scale and those with pupillary disorder (P=.002) had a worse clinical outcome. Likewise, those with associated intraparenchymal hematoma (P=.020) and those not receiving any treatment (P=.020) were also associated with a poor clinical outcome. These results were statistically significant. Patients admitted with a WFNS grade v and/or presenting pupil disorder and/or intraparenchymal hematoma were associated with worse clinical outcomes. Copyright © 2014 Sociedad Española de Neurocirugía. Published by Elsevier España. All rights reserved.

  8. Effect of Topical Intranasal Therapy on Epistaxis Frequency in Patients With Hereditary Hemorrhagic Telangiectasia: A Randomized Clinical Trial.

    Science.gov (United States)

    Whitehead, Kevin J; Sautter, Nathan B; McWilliams, Justin P; Chakinala, Murali M; Merlo, Christian A; Johnson, Maribeth H; James, Melissa; Everett, Eric M; Clancy, Marianne S; Faughnan, Marie E; Oh, S Paul; Olitsky, Scott E; Pyeritz, Reed E; Gossage, James R

    2016-09-06

    Epistaxis is a major factor negatively affecting quality of life in patients with hereditary hemorrhagic telangiectasia (HHT; also known as Osler-Weber-Rendu disease). Optimal treatment for HHT-related epistaxis is uncertain. To determine whether topical therapy with any of 3 drugs with differing mechanisms of action is effective in reducing HHT-related epistaxis. The North American Study of Epistaxis in HHT was a double-blind, placebo-controlled randomized clinical trial performed at 6 HHT centers of excellence. From August 2011 through March 2014, there were 121 adult patients who met the clinical criteria for HHT and had experienced HHT-related epistaxis with an Epistaxis Severity Score of at least 3.0. Follow-up was completed in September 2014. Patients received twice-daily nose sprays for 12 weeks with either bevacizumab 1% (4 mg/d), estriol 0.1% (0.4 mg/d), tranexamic acid 10% (40 mg/d), or placebo (0.9% saline). The primary outcome was median weekly epistaxis frequency during weeks 5 through 12. Secondary outcomes included median duration of epistaxis during weeks 5 through 12, Epistaxis Severity Score, level of hemoglobin, level of ferritin, need for transfusion, emergency department visits, and treatment failure. Among the 121 patients who were randomized (mean age, 52.8 years [SD, 12.9 years]; 44% women with a median of 7.0 weekly episodes of epistaxis [interquartile range {IQR}, 3.0-14.0]), 106 patients completed the study duration for the primary outcome measure (43 were women [41%]). Drug therapy did not significantly reduce epistaxis frequency (P = .97). After 12 weeks of treatment, the median weekly number of bleeding episodes was 7.0 (IQR, 4.5-10.5) for patients in the bevacizumab group, 8.0 (IQR, 4.0-12.0) for the estriol group, 7.5 (IQR, 3.0-11.0) for the tranexamic acid group, and 8.0 (IQR, 3.0-14.0) for the placebo group. No drug treatment was significantly different from placebo for epistaxis duration. All groups had a significant

  9. Prevention of haematoma progression by tranexamic acid in intracerebral haemorrhage patients with and without spot sign on admission scan

    DEFF Research Database (Denmark)

    Ovesen, Christian; Jakobsen, Janus Christian; Gluud, Christian

    2018-01-01

    OBJECTIVE: We present the statistical analysis plan of a prespecified Tranexamic Acid for Hyperacute Primary Intracerebral Haemorrhage (TICH)-2 sub-study aiming to investigate, if tranexamic acid has a different effect in intracerebral haemorrhage patients with the spot sign on admission compared...... to spot sign negative patients. The TICH-2 trial recruited above 2000 participants with intracerebral haemorrhage arriving in hospital within 8 h after symptom onset. They were included irrespective of radiological signs of on-going haematoma expansion. Participants were randomised to tranexamic acid...... versus matching placebo. In this subgroup analysis, we will include all participants in TICH-2 with a computed tomography angiography on admission allowing adjudication of the participants' spot sign status. RESULTS: Primary outcome will be the ability of tranexamic acid to limit absolute haematoma...

  10. Open trial of cimetidine in the prevention of upper gastro-intestinal haemorrhage in patients with severe intracranial injury.

    Science.gov (United States)

    Mouawad, E; Deloof, T; Genette, F; Vandesteene, A

    1983-01-01

    The present study evaluates the efficacy of Cimetidine in the prevention of clinically important gastro-intestinal haemorrhage in patients suffering from severe head injury. Fifty patients (39 males and 11 females) were included in the study. We excluded from the trial patients on anticoagulant therapy or concomitant non-steroid anti-inflammatory agents, pregnant and lactating women, and patients with previous histories of peptic ulcer disease.

  11. Radiosensitivity in ataxia-telangiectasia

    International Nuclear Information System (INIS)

    Lavin, M.F.; Khanna, K.K.; Watters, D.

    1998-01-01

    Full text: Radiosensitivity is a major hallmark of the human genetic disorder ataxia-telangiectasia. This hypersensitivity to ionizing radiation has been demonstrated in vitro after exposure of patients to therapeutic thought to be the major factor contculture. Clearly an understanding of the nature of the molecular defect in ataxia-telangiectasia will be of considerable assistance in delineating additional pathways that determine cellular radiosensitivity/radioresistance. Furthermore, since patients with this syndrome are also predisposed to developing a number of leukaemias and lymphomas the possible connection between radiosensitivity and cancer predisposition is of interest. Now that the gene (ATM) responsible for this genetic disease has been cloned and identified, progress is being made in determining the role of the ATM protein in mediating the effects of cellular exposure to ionizing radiation and other forms of redox stress. Proteins such as the product of the tumour suppressor gene p53 and the proto-oncogene c-Abl (a protein tyrosine kinase) have been shown to interact with ATM. Since several intermediate steps in both the p53 and c-Abl pathways, activated by ionizing radiation, are known it will be possible to map the position of ATM in these pathways and describe its mechanism of action. What are the clinical implications of understanding the molecular basis of the defect in ataxia-telangiectasia? As outlined above since radiosensitivity is a universal characteristic of A-T understanding the mechanism of action of ATM will provide additional information or radiation signalling in human cells. With this information it may be possible to sensitize tumour cells to radiation and thus increase the therapeutic benefit of radiotherapy. This might involve the use of small molecules that would interfere with the normal ATM controlled pathways and thus sensitize cells to radiation or alternatively it might involve the efficient introduction of ATM anti-sense c

  12. Radiosensitivity in ataxia-telangiectasia

    Energy Technology Data Exchange (ETDEWEB)

    Lavin, M.F. [Royal Brisbane Hospital, QLD (Australia). Queensland Institute of Medical Research and The Department of Surgery; Khanna, K.K.; Watters, D. [Royal Brisbane Hospital, QLD (Australia). Queensland Institute of Medical Research

    1998-12-31

    Full text: Radiosensitivity is a major hallmark of the human genetic disorder ataxia-telangiectasia. This hypersensitivity to ionizing radiation has been demonstrated in vitro after exposure of patients to therapeutic thought to be the major factor contculture. Clearly an understanding of the nature of the molecular defect in ataxia-telangiectasia will be of considerable assistance in delineating additional pathways that determine cellular radiosensitivity/radioresistance. Furthermore, since patients with this syndrome are also predisposed to developing a number of leukaemias and lymphomas the possible connection between radiosensitivity and cancer predisposition is of interest. Now that the gene (ATM) responsible for this genetic disease has been cloned and identified, progress is being made in determining the role of the ATM protein in mediating the effects of cellular exposure to ionizing radiation and other forms of redox stress. Proteins such as the product of the tumour suppressor gene p53 and the proto-oncogene c-Abl (a protein tyrosine kinase) have been shown to interact with ATM. Since several intermediate steps in both the p53 and c-Abl pathways, activated by ionizing radiation, are known it will be possible to map the position of ATM in these pathways and describe its mechanism of action. What are the clinical implications of understanding the molecular basis of the defect in ataxia-telangiectasia? As outlined above since radiosensitivity is a universal characteristic of A-T understanding the mechanism of action of ATM will provide additional information or radiation signalling in human cells. With this information it may be possible to sensitize tumour cells to radiation and thus increase the therapeutic benefit of radiotherapy. This might involve the use of small molecules that would interfere with the normal ATM controlled pathways and thus sensitize cells to radiation or alternatively it might involve the efficient introduction of ATM anti-sense c

  13. Effect of magnesium treatment and glucose levels on delayed cerebral ischemia in patients with subarachnoid hemorrhage : A substudy of the Magnesium in Aneurysmal Subarachnoid Haemorrhage trial (MASH-II)

    NARCIS (Netherlands)

    Leijenaar, Jolien F.; Dorhout Mees, Sanne M.; Algra, Ale; van den Bergh, Walter M.; Rinkel, Gabriel J. E.

    2015-01-01

    Background: Magnesium treatment did not improve outcome in patients with aneurysmal subarachnoid haemorrhage in the Magnesium in Aneurysmal Subarachnoid Haemorrhage II trial. We hypothesized that high glucose levels may have offset a potential beneficial effect to prevent delayed cerebral ischemia.

  14. Trans-anastomotic porto-portal varices in patients with gastrointestinal haemorrhage

    International Nuclear Information System (INIS)

    Mitchell, A.W.M.; Jackson, J.E.

    2000-01-01

    AIM: Porto-portal varices are commonly seen in patients with segmental extra-hepatic portal hypertension and develop to provide a collateral circulation around an area of portal venous obstruction. It is not well recognized that such communications may also develop across surgical anastomoses and be the source of gastrointestinal haemorrhage. The possible mode of development of such communications has not been previously discussed. MATERIALS AND METHODS: Over a 3-year period between 1995 and 1998, porto-portal varices were demonstrated across surgical anastomoses in four patients who were referred for the investigation of acute (two), acute-on-chronic (one) and chronic gastrointestinal bleeding (one). Their medical notes and the findings at angiography were reviewed. RESULTS: Three patients had segmental portal hypertension due to extra-hepatic portal vein (one) or superior mesenteric vein (two) stenosis/occlusion. One patient had mild portal hypertension due to hepatic fibrosis secondary to congenital biliary atresia. At angiography all patients were shown to have varices crossing previous surgical anastomoses. These varices were presumed to be the cause of bleeding in three of the four patients; the site of bleeding in the fourth individual was not determined. CONCLUSIONS: Trans-anastomotic porto-portal varices are rare. They develop in the presence of extra-hepatic portal hypertension and presumably arise within peri-anastomotic inflammatory tissue. Such varices may be difficult to manage and their prognosis is poor when bleeding occurs. Mitchell, A.W.M., Jackson, J.E. (2000)

  15. Haemorrhage from Pancreatic Pseudocysts Presenting as Upper Gastrointestinal Haemorrhage

    Directory of Open Access Journals (Sweden)

    Giuseppe Garcea

    2004-04-01

    Full Text Available Haemorrhage is a rare but frequently fatal complication of pancreatic pseudocysts. The high mortality associated with pancreatic haemorrhage makes prompt and aggressive management essential. Occasionally, haemorrhage may present atypically, leading to delay in its diagnosis and management. This report details a case of pancreatic haemorrhage presenting as an upper gastrointestinal bleed and discusses the subsequent management. When managing patients with pancreatic pseudocysts who present with the stigmata of upper gastrointestinal bleeding, the possibility that the bleeding originates from the pancreas must always be borne in mind.

  16. Subclinical haemorrhagic tendency exists in patients with β-thalassaemia major in early childhood

    Directory of Open Access Journals (Sweden)

    Abhishek Maiti

    2012-02-01

    Full Text Available BackgroundAlterations of coagulation profile have been reported in patients with β-thalassaemia major (β-TM.MethodTo investigate this in the paediatric population, we studied haemostatic parameters in pre-transfusion blood samples from 50 non-splenectomised transfusion-dependent children with β-TM (mean age 6±2.5 years and in blood from 25 healthy controls.ResultsLaboratory evaluation showed thrombocytopenia in 40%, prolongation of prothrombin time (PT in 12% and prolongation of activated partial thromboplastin time (APTT in 6% of the patients. Mean values for PT, APTT and platelet count (PC were all raised in the patient population compared with the controls. The alteration of coagulation status was significant for PT (p value 0.05. No significant liner correlation could be identified between PT, APTT, PC of the patients and interval between transfusions (in days or days since last transfusion.ConclusionThe findings from this study suggest that a subclinical haemorrhagic tendency exists in patients with β-TM at a very early age. The intrinsic pathway appears to be more affected than the extrinsic pathway.

  17. [Neonatal subgaleal haemorrhage; a potential life-threatening extracranial haemorrhage

    NARCIS (Netherlands)

    Fuijkschot, J.; Antonius, T.A.J.; Meijers, P.W.; Vrancken, S.L.A.G.

    2008-01-01

    A female neonate delivered at term developed hypovolemic shock due to a subgaleal haemorrhage, i.e. extracranial bleeding between the galea aponeurotica and the cranial periosteum. The subgaleal haemorrhage was most likely the result of a traumatic vacuum extraction. The patient was treated with

  18. Factors associated with poor outcome for aneurysmal subarachnoid haemorrhage in a series of 334 patients.

    Science.gov (United States)

    Rivero Rodríguez, D; Scherle Matamoros, C; Fernández Cúe, L; Miranda Hernández, J L; Pernas Sánchez, Y; Pérez Nellar, J

    This study evaluates care-related sociodemographic, clinical, and imaging factors and influences associated with outcome at discharge in patients with aneurismal subarachnoid haemorrhage. Retrospective cohort study in 334 patients treated at Hospital Hermanos Ameijeiras in Havana, Cuba between October 2005 and June 2014. Logistic regression analysis determined that the following factors were associated with higher risk of poor outcome: age older than 65 years (OR 3.51, 95% CI 1.79-5.7, P=.031), female sex (OR 2.17, 95% CI 1.22-3.84, P=.0067), systolic hypertension (OR 4.82, 95% CI 2.27-9.8, P=.0001), and hyperglycaemia at admission (OR 3.93, 95% CI 2.10-7.53, P=.0003). Certain complications were also associated with poor prognosis, including respiratory infection (OR 2.73, 95% CI 1.27-5.85, P=.0085), electrolyte disturbances (OR 3.33, 95% CI 1.33-8.28, P=.0073), hydrocephalus (OR 2.21, 95% CI 1.05-4.63, P=.0039), rebleeding (OR 16.50, 95% CI 8.24-41.24, P=.0000), symptomatic vasospasm (OR 19.00, 95% CI 8.86-41.24, P=.0000), cerebral ischaemia (OR 3.82, 95% CI 1.87-7.80, P=.000) and multiplex rebleeding (OR 6.69, 95% CI 1.35-36.39, P=.0019). Grades of iii and iv on the World Federation of Neurological Surgeons (OR 2.09, 95% CI 1.12-3.91, P=.0021) and Fisher scales (OR 5.18, 95% CI 2.65-10.29, P=.0008) were also related to poor outcome. Outcome of aneurysmal subarachnoid haemorrhage was related to age, sex, clinical status at admission to the stroke unit, imaging findings according to the Fisher scale, blood pressure, glycaemia and such complications as electrolyte disturbances, hydrocephalus, rebleeding, and multiplex rebleeding. Copyright © 2014 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.

  19. Amino Acids in Cerebrospinal Fluid of Patients with Aneurysmal Subarachnoid Haemorrhage: An Observational Study

    Directory of Open Access Journals (Sweden)

    Bartosz Sokół

    2017-08-01

    Full Text Available BackgroundThe authors are aware of only one article investigating amino acid concentrations in cerebrospinal fluid (CSF in patients with ruptured intracranial aneurysms, and this was published 31 years ago. Since then, both management of subarachnoid haemorrhage (SAH and amino acid assay techniques have seen radical alterations, yet the pathophysiology of SAH remains unclear.ObjectiveTo analyse the pattern of concentrations of amino acids and related compounds in patients with different outcomes following aneurysmal SAH.Methods49 CSF samples were collected from 23 patients on days 0–3, 5, and 10 post-SAH. Concentrations of 33 amino acids and related compounds were assayed by liquid chromatography tandem mass spectrometry in patients with good [Glasgow Outcome Scale (GOS 1–3] and poor (GOS 4–5 outcome.ResultsOf the 33 compounds assayed, only hydroxyproline and 3-aminoisobutyric acid appeared not to increase significantly following SAH. In poor outcome patients, we found significantly higher concentrations of aspartic acid (p = 0.038, glutamic acid (p = 0.038, and seven other compounds on days 0–3 post-SAH; glutamic acid (p = 0.041 on day 5 post-SAH, and 2-aminoadipic acid (p = 0.033 on day 10 post-SAH. The most significant correlation with GOS at 3 months was found for aminoadipic acid on day 10 post-SAH (cc = −0.81.ConclusionAneurysmal rupture leads to a generalised increase of amino acids and related compounds in CSF. The patterns differ between good and poor outcome cases. Increased excitatory amino acids are strongly indicative of poor outcome.

  20. Prehospital antiplatelet use and functional status on admission of patients with non-haemorrhagic moyamoya disease: a nationwide retrospective cohort study (J-ASPECT study)

    OpenAIRE

    Onozuka, Daisuke; Hagihara, Akihito; Nishimura, Kunihiro; Kada, Akiko; Nakagawara, Jyoji; Ogasawara, Kuniaki; Ono, Junichi; Shiokawa, Yoshiaki; Aruga, Toru; Miyachi, Shigeru; Nagata, Izumi; Toyoda, Kazunori; Matsuda, Shinya; Suzuki, Akifumi; Kataoka, Hiroharu

    2016-01-01

    Objectives To elucidate the association between antiplatelet use in patients with non-haemorrhagic moyamoya disease before hospital admission and good functional status on admission in Japan. Design Retrospective, multicentre, non-randomised, observational study. Setting Nationwide registry data in Japan. Participants A total of 1925 patients with non-haemorrhagic moyamoya disease admitted between 1 April 2012 and 31 March 2014 in Japan. Main outcome measure We performed propensity score-matc...

  1. Combined Acute Haemolytic and Secondary Angle Closure Glaucoma following Spontaneous Intraocular Haemorrhages in a Patient on Warfarin

    Directory of Open Access Journals (Sweden)

    Walter Andreatta

    2016-11-01

    Full Text Available Background: To report the first described case of combined haemolytic and acute angle closure glaucoma secondary to spontaneous intraocular haemorrhages in a patient on excessive anticoagulation. To the best of our knowledge, this is the first case reported in the literature presenting with raised intraocular pressure due to both mechanisms. Case Description: A 90-year-old woman presented with acute pain and reduction in vision in the left eye. Her intraocular pressure (IOP was 55 mm Hg. There were red tinted blood cells in the anterior chamber giving it a reddish hue. The patient was known to have advanced wet macular degeneration. She was taking oral warfarin for atrial fibrillation. Her international normalised ratio (INR was 7.7. B-scan ultrasound of posterior segment showed vitreous and suprachoroidal haemorrhages. An ultrabiomicroscopic examination confirmed open angles. A diagnosis of haemolytic glaucoma secondary to intraocular haemorrhages was made. The IOP was controlled medically. Warfarin was withdrawn and oral vitamin K therapy was initiated leading to a rapid INR reduction. Three days later, her anterior chamber became progressively shallower causing a secondary acute angle closure which was managed medically. After 2 months, the left IOP was well-controlled without any medications and the eye was not inflamed. Her vision in that eye remained perception of light. Conclusion: Patients with suprachoroidal haemorrhages should be closely monitored as they might subsequently develop acute angle closure despite an initially open angle and well-controlled INR and IOP. Excessive anticoagulation needs to be prevented to minimise the risk of sight-threatening complications.

  2. Imaging of Hereditary Hemorrhagic Telangiectasia

    International Nuclear Information System (INIS)

    Carette, Marie-France; Nedelcu, Cosmina; Tassart, Marc; Grange, Jean-Didier; Wislez, Marie; Khalil, Antoine

    2009-01-01

    This pictorial review is based on our experience of the follow-up of 120 patients at our multidisciplinary center for hereditary hemorrhagic telangiectasia (HHT). Rendu-Osler-Weber disease or HHT is a multiorgan autosomal dominant disorder with high penetrance, characterized by epistaxis, mucocutaneous telangiectasis, and visceral arteriovenous malformations (AVMs). The research on gene mutations is fundamental and family screening by clinical examination, chest X-ray, research of pulmonary shunting, and abdominal color Doppler sonography is absolutely necessary. The angioarchitecture of pulmonary AVMs can be studied by unenhanced multidetector computed tomography; however, all other explorations of liver, digestive bowels, or brain require administration of contrast media. Magnetic resonance angiography is helpful for central nervous system screening, in particular for the spinal cord, but also for pulmonary, hepatic, and pelvic AVMs. Knowledge of the multiorgan involvement of HHT, mechanism of complications, and radiologic findings is fundamental for the correct management of these patients.

  3. Potentiation by caffeine of x-ray damage to cultured human skin fibroblasts from normal subjects and ataxia-telangiectasia patients

    International Nuclear Information System (INIS)

    Furcinitti, P.S.

    1983-01-01

    Caffeine was found to potentiate x-ray-induced killing of human diploid fibroblasts from a normal subject and an ataxia-telangiectasia (AT) patient when it was present at 2 mM concentration for 30 to 66 h postirradiation. The dose-modifying factor for caffeine-treated normal cells had an average value of 1.26 +- 0.13 which did not vary significantly with treatment time or x-ray dose. The dose-modifying factor for caffeine-treated AT cells was 1.12 +- 0.12 at 30 h, rose to 1.66 +- 0.17 at 41 h, and decreased to 1.31 +- 0.13 at 66 h. Thus no clear difference was observed between these two cell strains' susceptibility to postirradiation caffeine treatment

  4. Renal impairment in stroke patients: A comparison between the haemorrhagic and ischemic variants [version 1; referees: 2 approved, 1 not approved

    Directory of Open Access Journals (Sweden)

    Pratyush Shrestha

    2017-08-01

    Full Text Available Background: Renal impairment is regularly seen in hospitalized stroke patients, affecting the outcome of patients, as well as causing difficulties in their management. A prospective cohort study was conducted to assess the trend of renal function in hospitalized ischemic and haemorrhagic stroke patients. The incidence of renal impairment in these subgroups, the contributing factors and the need for renal replacement in renal impaired patients was evaluated. Methods: Alternate day renal function testing was performed in hospitalized stroke patients. Estimated glomerular filtration rate (e-GFR was calculated and the trend of renal function in the two stroke subgroups (haemorrhagic and ischemic was assessed, with renal impairment defined as e-GFR < 60mL/ minute per 1.73m2. Results: Among 52 patients, 25 had haemorrhagic stroke (mean age 59.81 ± 14.67 and 27 had ischemic stroke (mean age 56.12 ± 13.08. The mean e-GFR (mL/minute per 1.732m2 at admission in the haemorrhagic stroke subgroup was 64.79 ± 25.85 compared to 86.04 ± 26.09 in the ischemic stroke subgroup (p=0.005. Sixteen out of 25 (64% patients in the haemorrhagic stroke subgroup and 9 out of 27 (33.3% patients in the ischemic subgroup developed renal impairment (p=0.27. The location of the bleed (p=0.8, volume of hematoma (p=0.966 and surgical intervention (p=0.4 did not predispose the patients to renal impairment. One out of 16 patients with haemorrhagic stroke (who eventually died, and 2 out of 9 patients with ischemic stroke required renal replacement. Conclusion: Renal impairment is commonly seen in stroke patients, more so in patients who suffered haemorrhagic strokes.  The impairment, however, is transient and rarely requires renal replacement therapy.

  5. Topical application of timolol decreases the severity and frequency of epistaxis in patients who have previously undergone nasal dermoplasty for hereditary hemorrhagic telangiectasia.

    Science.gov (United States)

    Ichimura, Keiichi; Kikuchi, Hisashi; Imayoshi, Shoichiro; Dias, Mari Shimada

    2016-08-01

    Hereditary hemorrhagic telangiectasia (HHT) is widely known to cause bleeding that is difficult to control because of the associated vascular wall fragility. Although nasal dermoplasty results in decreased severity and frequency of nasal bleeding in patients with HHT, it does not eradicate epistaxis because this procedure cannot cover the entire nasal cavity. Residual bleeding warrants additional effective therapy. Preliminary reports on the use of β-adrenergic blockers for treating epistaxis in patients with HHT encouraged us to examine their effects in HHT patients who had previously undergone nasal dermoplasty but still complained of epistaxis. We performed a prospective topical timolol, a nonselective beta blocker, application study involving 12 HHT patients who had undergone nasal dermoplasty. The observation period lasted for 3 months. There was one improperly enrolled case in which timolol administration was discontinued. The mean score of bleeding intensity and that of bleeding frequency were markedly reduced after treatment. Two patients who had required transfusions before treatment did not need them afterward, and patients were generally satisfied with the treatment. Topical timolol application was effective in decreasing epistaxis. Although no adverse effects were observed in the properly selected patients, there are contraindications to timolol application that should be kept in mind when applying this treatment. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  6. Haemorrhagic pituitary tumours

    International Nuclear Information System (INIS)

    Lazaro, C.M.; Philippine General Hospital, Manila; Guo, W.Y.; Sami, M.; Hindmarsch, T.; Ericson, K.; Hulting, A.L.; Wersaell, J.

    1994-01-01

    In a group of 69 patients with pituitary tumours, 12 were found to have evidence of intratumoral haemorrhage on MRI, characterized by high signal intensity on short TR/TE sequences. This was verified in all but 1 patient. The majority of the bleedings occurred in macroadenomas. Five (42%) were prolactinomas and 4 (33%) were non-functioning adenomas. There were 2 GH- and 1 ACTH-secreting tumours. All 5 patients with prolactinomas were on bromocriptine medication. Two of the patients had a clinical picture of pituitary apoplexy. The haemorrhage was not large enough to prompt surgery in any of the patients. However, surgical verification of the diagnosis was obtained in 5 cases, while 6 patients were examined with follow-up MRI. (orig.)

  7. Reduced CBF recovery detected by longitudinal 3D-SSP SPECT analyses predicts outcome of postoperative patients after subarachnoid haemorrhage.

    Science.gov (United States)

    Mutoh, Tatsushi; Totsune, Tomoko; Takenaka, Shunsuke; Tatewaki, Yasuko; Nakagawa, Manabu; Suarez, Jose I; Taki, Yasuyuki; Ishikawa, Tatsuya

    2018-02-01

    The aim of this study was to evaluate the impact of cerebral blood flow (CBF) recovery obtained from brain single-photon emission computed tomography (SPECT) images on postoperative outcome after aneurysmal subarachnoid haemorrhage (SAH). Twenty-nine patients who had undergone surgical clipping for ruptured anterior communicating artery aneurysms were analyzed prospectively. Routine measurements of CBF were performed using technetium-99 m hexamethyl propyleneamine oxine SPECT on days 4 and 14 after SAH. Regional voxel data analyzed by three dimensional stereotactic surface projection (3D-SSP) were compared between patients and age-matched normal database (NDB). In 3D-SSP analysis of all patients, cortical hypoperfusion around the surgical site in bilateral frontal lobes was evident on day 4 (P SSP SPECT image analyses can be a potential predictor of poor prognosis in postoperative patients after SAH. © 2017 John Wiley & Sons Australia, Ltd.

  8. Aneurysmal sub-arachnoid haemorrhage in patients with Noonan syndrome: a report of two cases and review of neurovascular presentations in this syndrome

    International Nuclear Information System (INIS)

    Dineen, R.A.; Lenthall, R.K.

    2004-01-01

    To date, intracranial aneurysms have been reported in two patients with Noonan syndrome. We report two unrelated patients with Noonan syndrome who presented with sub-arachnoid haemorrhage secondary to intracranial aneurysms. These cases are discussed and other cerebrovascular abnormalities that have been described in association with Noonan syndrome are reviewed. Ultrastructural and genetic factors contributing to these associations are considered. (orig.)

  9. Aneurysmal sub-arachnoid haemorrhage in patients with Noonan syndrome: a report of two cases and review of neurovascular presentations in this syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Dineen, R.A.; Lenthall, R.K. [Department of Radiology, University Hospital, Queen' s Medical Centre, NG7 2UH, Nottingham (United Kingdom)

    2004-04-01

    To date, intracranial aneurysms have been reported in two patients with Noonan syndrome. We report two unrelated patients with Noonan syndrome who presented with sub-arachnoid haemorrhage secondary to intracranial aneurysms. These cases are discussed and other cerebrovascular abnormalities that have been described in association with Noonan syndrome are reviewed. Ultrastructural and genetic factors contributing to these associations are considered. (orig.)

  10. Ataxia-telangiectasia: future prospects

    Directory of Open Access Journals (Sweden)

    Chaudhary MW

    2014-09-01

    Full Text Available Mohammed Wajid Chaudhary, Raidah Saleem Al-Baradie Pediatric Neurology, Neurosciences Centre, King Fahad Specialist Hospital, Dammam, Kingdom of Saudi Arabia Abstract: Ataxia-telangiectasia (A-T is an autosomal recessive multi-system disorder caused by mutation in the ataxia-telangiectasia mutated gene (ATM. ATM is a large serine/threonine protein kinase, a member of the phosphoinositide 3-kinase-related protein kinase (PIKK family whose best-studied function is as master controller of signal transduction for the DNA damage response (DDR in the event of double strand breaks (DSBs. The DDR rapidly recognizes DNA lesions and initiates the appropriate cellular programs to maintain genome integrity. This includes the coordination of cell-cycle checkpoints, transcription, translation, DNA repair, metabolism, and cell fate decisions, such as apoptosis or senescence. DSBs can be generated by exposure to ionizing radiation (IR or various chemical compounds, such as topoisomerase inhibitors, or can be part of programmed generation and repair of DSBs via cellular enzymes needed for the generation of the antibody repertoire as well as the maturation of germ cells. AT patients have immunodeficiency, and are sterile with gonadal dysgenesis as a result of defect in meiotic recombination. In the cells of nervous system ATM has additional role in vesicle dynamics as well as in the maintenance of the epigenetic code of histone modifications. Moderate levels of ATM are associated with prolonged lifespan through resistance to oxidative stress. ATM inhibitors are being viewed as potential radiosensitizers as part of cancer radiotherapy. Though there is no cure for the disease at present, glucocorticoids have been shown to induce alternate splicing site in the gene for ATM partly restoring its activity, but their most effective timing in the disease natural history is not yet known. Gene therapy is promising but large size of the gene makes it technically difficult

  11. The effectiveness and safety of antifibrinolytics in patients with acute intracranial haemorrhage: statistical analysis plan for an individual patient data meta-analysis

    OpenAIRE

    Ker, Katharine; Prieto-Merino, David; Sprigg, Nikola; Mahmood, Abda; Bath, Philip; Kang Law, Zhe; Flaherty, Katie; Roberts, Ian

    2017-01-01

    Introduction: The Antifibrinolytic Trialists Collaboration aims to increase knowledge about the effectiveness and safety of antifibrinolytic treatment by conducting individual patient data (IPD) meta-analyses of randomised trials. This article presents the statistical analysis plan for an IPD meta-analysis of the effects of antifibrinolytics for acute intracranial haemorrhage. Methods: The protocol for the IPD meta-analysis has been registered with PROSPERO (CRD42016052155). We will conduct a...

  12. Cortical spreading ischaemia is a novel process involved in ischaemic damage in patients with aneurysmal subarachnoid haemorrhage

    DEFF Research Database (Denmark)

    Dreier, Jens P; Major, Sebastian; Manning, Andrew

    2009-01-01

    flow and electrocorticography were simultaneously recorded in 417 CSDs. Isolated CSDs occurred in 12 patients and were associated with either physiological, absent or inverse haemodynamic responses. Whereas the physiological haemodynamic response caused tissue hyperoxia, the inverse response led......, causing either transient hyperperfusion (physiological haemodynamic response) in healthy tissue; or hypoperfusion [inverse haemodynamic response = cortical spreading ischaemia (CSI)] in tissue at risk for progressive damage, which has so far only been shown experimentally. Here, we performed a prospective......, multicentre study in 13 patients with aneurysmal subarachnoid haemorrhage, using novel subdural opto-electrode technology for simultaneous laser-Doppler flowmetry (LDF) and direct current-electrocorticography, combined with measurements of tissue partial pressure of oxygen (ptiO(2)). Regional cerebral blood...

  13. Brain abscesses and hereditary hemorrhagic telangiectasia

    International Nuclear Information System (INIS)

    Vives, Daniel A.; Bauni, Carlos E.; Mendoza, Monica E.

    2003-01-01

    Rendu-Osler-Weber disease or Hereditary Hemorrhagic Telangiectasia (HHT) is a generalized familial angiodysplastic disorder. The neurological manifestations of this entity are due to Central Nervous System vascular lesions or to complications of other visceral lesions such as pulmonary arteriovenous fistulae. This report describes two patients (males, 40 and 61 years old), with brain abscesses associated with HHT. The CT, MRI and Angiographic findings as well as the therapeutic approach are analyzed. Patients with brain abscess of unknown origin must be evaluated for the presence of lung vascular malformation in association with HHT. (author)

  14. Prehospital antiplatelet use and functional status on admission of patients with non-haemorrhagic moyamoya disease: a nationwide retrospective cohort study (J-ASPECT study).

    Science.gov (United States)

    Onozuka, Daisuke; Hagihara, Akihito; Nishimura, Kunihiro; Kada, Akiko; Nakagawara, Jyoji; Ogasawara, Kuniaki; Ono, Junichi; Shiokawa, Yoshiaki; Aruga, Toru; Miyachi, Shigeru; Nagata, Izumi; Toyoda, Kazunori; Matsuda, Shinya; Suzuki, Akifumi; Kataoka, Hiroharu; Nakamura, Fumiaki; Kamitani, Satoru; Nishimura, Ataru; Kurogi, Ryota; Sayama, Tetsuro; Iihara, Koji

    2016-03-15

    To elucidate the association between antiplatelet use in patients with non-haemorrhagic moyamoya disease before hospital admission and good functional status on admission in Japan. Retrospective, multicentre, non-randomised, observational study. Nationwide registry data in Japan. A total of 1925 patients with non-haemorrhagic moyamoya disease admitted between 1 April 2012 and 31 March 2014 in Japan. We performed propensity score-matched analysis to examine the association between prehospital antiplatelet use and no significant disability on hospital admission, as defined by a modified Rankin Scale score of 0 or 1. Propensity-matched patients who received prehospital antiplatelet drugs were associated with a good outcome on hospital admission (OR adjusted for all covariates, 3.82; 95% CI 1.22 to 11.99) compared with those who did not receive antiplatelet drugs prior to hospital admission. Prehospital antiplatelet use was significantly associated with good functional status on hospital admission among patients with non-haemorrhagic moyamoya disease in Japan. Our results suggest that prehospital antiplatelet use should be considered when evaluating outcomes of patients with non-haemorrhagic moyamoya disease. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

  15. Prehospital antiplatelet use and functional status on admission of patients with non-haemorrhagic moyamoya disease: a nationwide retrospective cohort study (J-ASPECT study)

    Science.gov (United States)

    Onozuka, Daisuke; Hagihara, Akihito; Nishimura, Kunihiro; Kada, Akiko; Nakagawara, Jyoji; Ogasawara, Kuniaki; Ono, Junichi; Shiokawa, Yoshiaki; Aruga, Toru; Miyachi, Shigeru; Nagata, Izumi; Toyoda, Kazunori; Matsuda, Shinya; Suzuki, Akifumi; Kataoka, Hiroharu; Nakamura, Fumiaki; Kamitani, Satoru; Nishimura, Ataru; Kurogi, Ryota; Sayama, Tetsuro; Iihara, Koji

    2016-01-01

    Objectives To elucidate the association between antiplatelet use in patients with non-haemorrhagic moyamoya disease before hospital admission and good functional status on admission in Japan. Design Retrospective, multicentre, non-randomised, observational study. Setting Nationwide registry data in Japan. Participants A total of 1925 patients with non-haemorrhagic moyamoya disease admitted between 1 April 2012 and 31 March 2014 in Japan. Main outcome measure We performed propensity score-matched analysis to examine the association between prehospital antiplatelet use and no significant disability on hospital admission, as defined by a modified Rankin Scale score of 0 or 1. Results Propensity-matched patients who received prehospital antiplatelet drugs were associated with a good outcome on hospital admission (OR adjusted for all covariates, 3.82; 95% CI 1.22 to 11.99) compared with those who did not receive antiplatelet drugs prior to hospital admission. Conclusions Prehospital antiplatelet use was significantly associated with good functional status on hospital admission among patients with non-haemorrhagic moyamoya disease in Japan. Our results suggest that prehospital antiplatelet use should be considered when evaluating outcomes of patients with non-haemorrhagic moyamoya disease. PMID:27008684

  16. Diode laser for the treatment of telangiectasias following hemangioma involution.

    Science.gov (United States)

    Cerrati, Eric W; O, Teresa M; Chung, Hoyun; Waner, Milton

    2015-02-01

    Infantile hemangiomas are well known for their rapid growth during the first 6 to 9 months of life, followed by a spontaneous but slow involution. The standard of care is to treat these lesions at an early age with propranolol to expedite the involution process; however, surgery still remains an active component in the management. Medical treatment with propranolol or natural involution will often result in residual telangiectasias. We evaluated the efficacy of using a diode laser as a treatment for telangiectasias following cervicofacial infantile hemangioma involution. Case series with chart review. Tertiary care hospital and practice specializing in the care of vascular anomalies. Twenty patients, aged 4 months to 11 years (average 2.69 years), underwent treatment with a 532-nm diode laser to treat the residual telangiectasias following hemangioma involution. All procedures were performed in the operating room. To assess the efficacy, we independently evaluated pre- and posttreatment digital photographs and ranked them on a 0- to 4-point scale (0 = no change and 4 = complete response). Adverse reactions were also recorded. The telangiectasias showed considerable improvement following treatment. In more than half of the patients treated, the affected area demonstrated a complete response. No adverse reactions were noted. A 532-nm diode laser effectively treats the remaining telangiectasias following hemangioma involution. Whether used independently or in conjunction with other treatment modalities, the diode laser should be part of the surgical armamentarium when treating infantile hemangiomas. © American Academy of Otolaryngology—Head and Neck Surgery Foundation 2014.

  17. Predicting the risks of venous thromboembolism versus post-pancreatectomy haemorrhage: analysis of 13 771 NSQIP patients

    Science.gov (United States)

    Tzeng, Ching-Wei D; Katz, Matthew H G; Lee, Jeffrey E; Fleming, Jason B; Pisters, Peter W T; Vauthey, Jean-Nicolas; Aloia, Thomas A

    2014-01-01

    Background The fear of an early post-pancreatectomy haemorrhage (PPH) may prevent surgeons from prescribing post-operative venous thromboembolism (VTE) chemoprophylaxis. The primary hypothesis of this study was that the national post-pancreatectomy early PPH rate was lower than the rate of VTE. The secondary hypothesis was that patients at high risk for post-discharge VTE could be identified, potentially facilitating the selective use of extended chemoprophylaxis. Patients and methods All elective pancreatectomies were identified in the 2005 to 2010 American College of Surgeons-National Surgical Quality Improvement Program (ACS-NSQIP) database. Factors associated with 30-day rates of (pre-versus post-discharge) VTE, early PPH (transfusions > 4 units within 72 h) and return to the operating room (ROR) with PPH were analysed. Results Pancreaticoduodenectomies (PD) and distal pancreatectomies (DP) numbered 9140 (66.4%) and 4631 (33.6%) out of 13 771 pancreatectomies, respectively. Event rates included: VTE (3.1%), PPH (1.1%) and ROR+PPH (0.7%). PD and DP had similar VTE rates (P > 0.05) with 31.9% of VTE occurring post-discharge. Independent risk factors for late VTE included obesity [odds ratio (OR), 1.5], age ≥ 75 years (OR, 1.8), DP (OR, 2.4) and organ space infection (OR, 2.1) (all P pancreatectomy VTE outnumber early haemorrhagic complications, which are rare. The fear of PPH should not prevent routine and timely post-pancreatectomy VTE chemoprophylaxis. Because one-third of VTE occur post-discharge, high-risk patients may benefit from post-discharge chemoprophylaxis. PMID:23869628

  18. Emergency percutaneous transcatheter embolisation of acute arterial haemorrhage.

    LENUS (Irish Health Repository)

    Keeling, A N

    2010-09-01

    The purpose of this study was to review indications, source of haemorrhage, method of embolisation and clinical outcome in patients referred to Interventional Radiology for the emergency management of acute arterial haemorrhage.

  19. Effect of magnesium treatment and glucose levels on delayed cerebral ischemia in patients with subarachnoid hemorrhage : a substudy of the Magnesium in Aneurysmal Subarachnoid Haemorrhage trial (MASH-II)

    NARCIS (Netherlands)

    Leijenaar, Jolien F.; Mees, Sanne M. Dorhout; Algra, Ale; van den Bergh, Walter M.; Rinkel, Gabriel J. E.

    2015-01-01

    BackgroundMagnesium treatment did not improve outcome in patients with aneurysmal subarachnoid haemorrhage in the Magnesium in Aneurysmal Subarachnoid Haemorrhage II trial. We hypothesized that high glucose levels may have offset a potential beneficial effect to prevent delayed cerebral ischemia. We

  20. Likelihood of aneurysmal subarachnoid haemorrhage in patients with normal unenhanced CT, CSF xanthochromia on spectrophotometry and negative CT angiography.

    Science.gov (United States)

    Rana, A K; Turner, H E; Deans, K A

    2013-01-01

    Patients with suspected subarachnoid haemorrhage, a normal noncontrast computed tomography (CT) and cerebrospinal fluid (CSF) evidence of haemoglobin breakdown products often undergo CT angiography (CTA). If this is normal, then invasive catheter angiography may be offered. In current clinical practice, haemoglobin breakdown products are detected by spectrophotometry rather than visible xanthochromia, and CTA is performed on multidetector scanners. The aim of this study was to determine if such patients should still have a catheter angiography, given the associated risks. Patients positive for CSF spectrophotometry (n=26) were retrospectively identified from the clinical biochemistry information system and imaging data from the electronic radiology records were reviewed. Discharge letters were consulted to relate the biochemistry and radiology results to the final diagnosis. 15 patients with CT angiography were found. Nine patients had normal CT angiography. No causative aneurysms had been missed. One patient had small, coincidental aneurysms missed on initial reading of the CTA. The likelihood of a clinically significant aneurysm in a patient who is CT negative, lumbar puncture positive and CTA negative is low. Double reporting of negative CT angiograms may be advisable.

  1. The effectiveness and safety of antifibrinolytics in patients with acute intracranial haemorrhage: statistical analysis plan for an individual patient data meta-analysis [version 2; referees: 2 approved

    Directory of Open Access Journals (Sweden)

    Katharine Ker

    2018-02-01

    Full Text Available Introduction: The Antifibrinolytic Trialists Collaboration aims to increase knowledge about the effectiveness and safety of antifibrinolytic treatment by conducting individual patient data (IPD meta-analyses of randomised trials. This article presents the statistical analysis plan for an IPD meta-analysis of the effects of antifibrinolytics for acute intracranial haemorrhage. Methods: The protocol for the IPD meta-analysis has been registered with PROSPERO (CRD42016052155. We will conduct an individual patient data meta-analysis of randomised controlled trials with 1000 patients or more assessing the effects of antifibrinolytics in acute intracranial haemorrhage. We will assess the effect on two co-primary outcomes: 1 Death in hospital within 30 days of randomisation, and 2 Death  or dependency at final follow-up within 90 days of randomisation. The co-primary outcomes will be limited to patients treated within three hours of injury or stroke onset. We will report treatment effects using odds ratios and 95% confidence intervals. We use logistic regression models to examine how the effect of antifibrinolytics vary by time to treatment, severity of intracranial bleeding, and age. We will also examine the effect of antifibrinolytics on secondary outcomes including death, dependency, vascular occlusive events, seizures, and neurological outcomes. Secondary outcomes will be assessed in all patients irrespective of time of treatment. All analyses will be conducted on an intention-to-treat basis. Conclusions: This IPD meta-analysis will examine important clinical questions about the effects of antifibrinolytic treatment in patients with intracranial haemorrhage that cannot be answered using aggregate data. With IPD we can examine how effects vary by time to treatment, bleeding severity, and age, to gain better understanding of the balance of benefit and harms on which to base recommendations for practice.

  2. The effectiveness and safety of antifibrinolytics in patients with acute intracranial haemorrhage: statistical analysis plan for an individual patient data meta-analysis [version 1; referees: 2 approved

    Directory of Open Access Journals (Sweden)

    Katharine Ker

    2017-12-01

    Full Text Available Introduction: The Antifibrinolytic Trialists Collaboration aims to increase knowledge about the effectiveness and safety of antifibrinolytic treatment by conducting individual patient data (IPD meta-analyses of randomised trials. This article presents the statistical analysis plan for an IPD meta-analysis of the effects of antifibrinolytics for acute intracranial haemorrhage. Methods: The protocol for the IPD meta-analysis has been registered with PROSPERO (CRD42016052155. We will conduct an individual patient data meta-analysis of randomised controlled trials with 1000 patients or more assessing the effects of antifibrinolytics in acute intracranial haemorrhage. We will assess the effect on two co-primary outcomes: 1 death in hospital at end of trial follow-up, and 2 death in hospital or dependency at end of trial follow-up. The co-primary outcomes will be limited to patients treated within three hours of injury or stroke onset. We will report treatment effects using odds ratios and 95% confidence intervals. We use logistic regression models to examine how the effect of antifibrinolytics vary by time to treatment, severity of intracranial bleeding, and age. We will also examine the effect of antifibrinolytics on secondary outcomes including death, dependency, vascular occlusive events, seizures, and neurological outcomes. Secondary outcomes will be assessed in all patients irrespective of time of treatment. All analyses will be conducted on an intention-to-treat basis. Conclusions: This IPD meta-analysis will examine important clinical questions about the effects of antifibrinolytic treatment in patients with intracranial haemorrhage that cannot be answered using aggregate data. With IPD we can examine how effects vary by time to treatment, bleeding severity, and age, to gain better understanding of the balance of benefit and harms on which to base recommendations for practice.

  3. The effectiveness and safety of antifibrinolytics in patients with acute intracranial haemorrhage: statistical analysis plan for an individual patient data meta-analysis.

    Science.gov (United States)

    Ker, Katharine; Prieto-Merino, David; Sprigg, Nikola; Mahmood, Abda; Bath, Philip; Kang Law, Zhe; Flaherty, Katie; Roberts, Ian

    2017-01-01

    Introduction : The Antifibrinolytic Trialists Collaboration aims to increase knowledge about the effectiveness and safety of antifibrinolytic treatment by conducting individual patient data (IPD) meta-analyses of randomised trials. This article presents the statistical analysis plan for an IPD meta-analysis of the effects of antifibrinolytics for acute intracranial haemorrhage. Methods : The protocol for the IPD meta-analysis has been registered with PROSPERO (CRD42016052155). We will conduct an individual patient data meta-analysis of randomised controlled trials with 1000 patients or more assessing the effects of antifibrinolytics in acute intracranial haemorrhage. We will assess the effect on two co-primary outcomes: 1) death in hospital at end of trial follow-up, and 2) death in hospital or dependency at end of trial follow-up. The co-primary outcomes will be limited to patients treated within three hours of injury or stroke onset. We will report treatment effects using odds ratios and 95% confidence intervals. We use logistic regression models to examine how the effect of antifibrinolytics vary by time to treatment, severity of intracranial bleeding, and age. We will also examine the effect of antifibrinolytics on secondary outcomes including death, dependency, vascular occlusive events, seizures, and neurological outcomes. Secondary outcomes will be assessed in all patients irrespective of time of treatment. All analyses will be conducted on an intention-to-treat basis. Conclusions : This IPD meta-analysis will examine important clinical questions about the effects of antifibrinolytic treatment in patients with intracranial haemorrhage that cannot be answered using aggregate data. With IPD we can examine how effects vary by time to treatment, bleeding severity, and age, to gain better understanding of the balance of benefit and harms on which to base recommendations for practice.

  4. Manuseio anestésico de paciente portador de telangiectasia hemorrágica hereditária (síndrome de Rendu-Osler-Weber: relato de caso Manoseo anestésico de paciente portador de telangiectasia hemorrágica hereditaria (síndrome de Rendu - Osler - Weber: relato de caso Anesthetic management of a patient with hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome: case report

    Directory of Open Access Journals (Sweden)

    Alexandre Palmeira Goulart

    2009-02-01

    characterized by mucocutaneous and visceral vascular dysplasia associated with frequent episodes of epistaxis and gastrointestinal bleeding. The objective of this report was to describe the anesthesia of a patient with this syndrome. CASE REPORT: A 25 years old male patient underwent surgical correction of an orbital fracture. He had the triad of recurrent epistaxis, family history, and telangiectasia, and had been diagnosed with HHT. Pulmonary, brain, or gastrointestinal tract vascular malformations were not detected in the preoperative investigation. The patient underwent total venous anesthesia one hour after the administration of an antifibrinolytic drug. Bleeding was considered normal for this type of surgery, and hemodynamic instability or the need of perioperative blood transfusion was not detected. The patient was extubated in the operating room; he was transferred to the room after 60 minutes and discharged from the hospital after 24 hours. CONCLUSIONS: Hereditary hemorrhagic telangiectasia is an autosomal dominant disorder that leads to mucocutaneous and visceral vascular dysplasia. Perioperative blood loss can be greater than expected in patients with this syndrome. Since bleeding does not result from a defect in the coagulation cascade but from the surgical exposure of malformed vascular structures, perioperative conduct includes the use of antifibrinolytics, adequate homeostasis, and induced hypotension in the absence of contraindications. Pre-anesthetic evaluation should include the search for brain, lung, and gastrointestinal vascular malformation.

  5. [Hereditary hemorrhagic telangiectasia presenting with hematuria and severe anemia].

    Science.gov (United States)

    Paz, A; Goren, E; Segal, M

    1995-07-01

    A patient with hereditary hemorrhagic telangiectasia was admitted with hematuria and severe anemia after mild recurrent episodes of epistaxis. Telangiectasias were found in the skin and buccal and nasal mucosa. No defect in the coagulation mechanism was found; thrombocyte count and function were normal. On cystoscopy, tortuous engorged vessels, some actively bleeding, were seen in the trigonal mucosa. Biopsy showed enlarged vessels in the lamina propria. Electrocoagulation of the bleeding vessels stopped hematuria, but 6 months later it recurred. This time Nd-YAG laser was used to stop the bleeding after electrocoagulation was ineffective.

  6. Predictors of symptomatic intracranial haemorrhage in patients with an ischaemic stroke with neurological deterioration after intravenous thrombolysis.

    Science.gov (United States)

    James, Brandon; Chang, Andrew D; McTaggart, Ryan A; Hemendinger, Morgan; Mac Grory, Brian; Cutting, Shawna M; Burton, Tina M; Reznik, Michael E; Thompson, Bradford; Wendell, Linda; Mahta, Ali; Siket, Matthew; Madsen, Tracy E; Sheth, Kevin N; Nouh, Amre; Furie, Karen L; Jayaraman, Mahesh V; Khatri, Pooja; Yaghi, Shadi

    2018-02-27

    Early neurological deterioration prompting urgent brain imaging occurs in nearly 15% of patients with ischaemic stroke receiving intravenous tissue plasminogen activator (tPA). We aim to determine risk factors associated with symptomatic intracranial haemorrhage (sICH) in patients with ischaemic stroke undergoing emergent brain imaging for early neurological deterioration after receiving tPA. We abstracted data from our prospective stroke database and included all patients receiving tPA for ischaemic stroke between 1 March 2015 and 1 March 2017. We then identified patients with neurological deterioration who underwent urgent brain imaging prior to their per-protocol surveillance imaging and divided patients into two groups: those with and without sICH. We compared baseline demographics, clinical variables, in-hospital treatments and functional outcomes at 90 days between the two groups. We identified 511 patients who received tPA, of whom 108 (21.1%) had an emergent brain CT. Of these patients, 17.5% (19/108) had sICH; 21.3% (23/108) of emergent scans occurred while tPA was infusing, though only 4.3% of these scans (1/23) revealed sICH. On multivariable analyses, the only predictor of sICH was a change in level of consciousness (OR 6.62, 95% CI 1.64 to 26.70, P=0.008). Change in level of consciousness is associated with sICH among patients undergoing emergent brain imaging after receiving tPA. In this group of patients, preparation of tPA reversal agents while awaiting brain imaging may reduce reversal times. Future studies are needed to study the cost-effectiveness of this approach. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  7. Differential expression of circulating microRNAs in blood and haematoma samples from patients with intracerebral haemorrhage.

    Science.gov (United States)

    Wang, Jialu; Zhu, Ying; Jin, Feng; Tang, Ling; He, Zhenwei; He, Zhiyi

    2016-06-01

    To measure the differential expression of microRNAs (miRNAs) in peripheral blood samples from patients with intracerebral haemorrhage (ICH) and to measure the levels of hsa-miR-21-5p in peripheral blood and haematoma samples from patients with ICH. This case-control study enrolled individuals with ICH in the putamen treated by craniotomy and age- and sex-matched healthy control subjects. Serum miRNA expression profiles were determined in the patient and control groups using miRNA polymerase chain reaction (PCR) arrays. The ICH-related miRNA hsa-miR-21-5p was selected and its differential expression was assessed in peripheral blood and haematoma specimens from patients with ICH compared with peripheral blood samples controls using real-time PCR. Seven patients and five control subjects were included in the miRNA expression profile analysis; and 31 patients and 22 control subjects provided samples for the real-time PCR of hsa-miR-21-5p expression. A total of 59 miRNAs were significantly downregulated in patients with ICH. Relative hsa-miR-21-5p levels of 0.43 and 0.31 for peripheral blood and haematoma samples, respectively, were obtained in the patient group compared with the control subjects. Hsa-miR-21-5p levels were significantly reduced in both peripheral blood and haematoma samples in patients with ICH. © The Author(s) 2016.

  8. Intraventricular Transplantation of Autologous Bone Marrow Mesenchymal Stem Cells via Ommaya Reservoir in Persistent Vegetative State Patients after Haemorrhagic Stroke: Report of Two Cases & Review of the Literature

    Directory of Open Access Journals (Sweden)

    Fauzi AA

    2016-11-01

    Full Text Available Background: One of the most devastating diseases, stroke, is a leading cause of death and disability worldwide with severe emotional and economic consequences. The purpose of this article is mainly to report the effect of intraventricular transplantation via an Ommaya reservoir using autologous bone marrow mesenchymal stem cells (BM-MSCs in haemorrhagic stroke patients. Case Presentations: Two patients, aged 51 and 52, bearing sequels of haemorrhagic stroke were managed by intraventricular transplantation of BM-MSCs obtained from their own bone marrow. Before the procedure, both patients were bedridden, tracheostomised, on nasogastric (NG tube feeding and in hemiparesis. The cells were transplanted intraventricularly (20 x 106 cells/2.5 ml using an Ommaya reservoir, and then repeated transplantations were done after 1 and 2 months consecutively. The safety and efficacy of the procedures were evaluated 3, 6 and 12 months after treatment. The National Institute of Health Stroke Scale (NIHSS was used to evaluate the patients' neurological status before and after treatment. No adverse events derived from the procedures or transplants were observed in the one-year follow-up period, and the neurological status of both patients improved after treatment. Conclusions: Our report demonstrates that the intraventricular transplantation of BM-MSCs via an Ommaya reservoir is safe and it improves the neurological status of post-haemorrhagic stroke patients. The repeated transplantation procedure is easier and safer to perform via a subcutaneously implanted Ommaya reservoir.

  9. Early CT signs in patients with acute middle cerebral artery occlusion: incidence of contrast staining and haemorrhagic transformations after intra-arterial reperfusion therapy

    International Nuclear Information System (INIS)

    Nakano, S.; Iseda, T.; Yoneyama, T.; Wakisaka, S.

    2006-01-01

    AIM: To investigate how often early computed tomography (CT) signs are associated with blood-brain barrier (BBB) disruption and result in haemorrhagic transformations. MATERIALS AND METHODS: Serial CT findings were prospectively evaluated in 61 patients with acute middle cerebral artery (MCA) occlusion who underwent initial CT examination within 3 h of stroke onset and who were treated with intra-arterial reperfusion therapy within 6 h of stroke onset. Obscuration of the margin of the lentiform nucleus and loss of the insular ribbon were evaluated as early CT signs in the deep MCA territories. Cortical effacement was also evaluated. BBB disruption was defined as contrast medium staining in post-therapeutic CT just after reperfusion therapy. The relationship between pre-therapeutic early CT signs and post-therapeutic contrast staining or haemorrhagic transformations was investigated. RESULTS: The frequency of early CT signs in the deep MCA territories was significantly higher than that of cortical effacement (68.9 versus 27.9%). There were significant correlations between pre-therapeutic early CT signs and post-therapeutic contrast staining in both the deep and superficial MCA territories. Compared with early CT signs in the deep MCA territories, cortical effacement had a significantly higher incidence of post-therapeutic contrast staining (54.8 versus 82.4%). Although not statistically significant, cortical effacement tended to develop into haemorrhagic transformations. There was no correlation between early CT signs in the deep MCA territories and haemorrhagic transformations. CONCLUSION: Cortical effacement may be an advanced CT sign with BBB disruption and potential risk for haemorrhagic transformations. The presence of early CT signs in the deep MCA territories may not be a contraindication of reperfusion therapy

  10. Preventive Antibiotics and Delayed Cerebral Ischaemia in Patients with Aneurysmal Subarachnoid Haemorrhage Admitted to the Intensive Care Unit.

    Science.gov (United States)

    Gathier, Celine S; Oostdijk, Evelien A; Rinkel, Gabriel J E; Dorhout Mees, Sanne M; Vergouwen, Mervyn D I; de Smet, Anne Marie G A; van de Beek, Diederik; Vandertop, W Peter; Verbaan, Dagmar; Algra, Ale; Bonten, Marc J M; van den Bergh, Walter M

    2016-02-01

    Delayed cerebral ischemia (DCI) is an important contributor to poor outcome after aneurysmal subarachnoid haemorrhage (aSAH). Development of DCI is multifactorial, and inflammation, with or without infection, is one of the factors independently associated with development of DCI and poor outcome. We thus postulated that preventive antibiotics might be associated with a reduced risk of DCI and subsequent poor outcome in aSAH patients. We performed a retrospective cohort-study in intensive care units (ICU) of three university hospitals in The Netherlands. We included consecutive aSAH patients with minimal ICU stay of 72 h who received either preventive antibiotics (SDD: selective digestive tract decontamination including systemic cefotaxime or SOD: selective oropharyngeal decontamination) or no preventive antibiotics. DCI was defined as a new hypodensity on CT with no other explanation than DCI. Hazard ratio's (HR) for DCI and risk ratio's (RR) for 28-day case-fatality and poor outcome at 3 months were calculated, with adjustment (aHR/aRR) for clinical condition on admission, recurrent bleeding, aneurysm treatment modality and treatment site. Of 459 included patients, 274 received preventive antibiotics (SOD or SDD) and 185 did not. With preventive antibiotics, the aHR for DCI was 1.0 (95% CI 0.6-1.8), the aRR for 28-day case-fatality was 1.1 (95% CI 0.7-1.9) and the aRR for poor functional outcome 1.2 (95% CI 1.0-1.4). Preventive antibiotics were not associated with reduced risk of DCI or poor outcome in aSAH patients in the ICU.

  11. Difference in aneurysm characteristics between patients with familial and sporadic aneurysmal subarachnoid haemorrhage

    NARCIS (Netherlands)

    Mensing, Liselore A.; Rinkel, Gabriel J E; Vlak, Monique H M; Van Der Schaaf, Irene C.; Ruigrok, Ynte M.

    2016-01-01

    Object Patients with familial intracranial aneurysms (IA) have a higher risk of rupture than patients with sporadic IA. We compared geometric and morphological risk factors for aneurysmal rupture between patients with familial and sporadic aneurysmal subarachnoid hemorrhage (aSAH) to analyse if

  12. Calcium antagonists for aneurysmal subarachnoid haemorrhage

    NARCIS (Netherlands)

    Dorhout Mees, S. M.; Rinkel, G. J. E.; Feigin, V. L.; Algra, A.; van den Bergh, W. M.; Vermeulen, M.; van Gijn, J.

    2007-01-01

    BACKGROUND: Secondary ischaemia is a frequent cause of poor outcome in patients with subarachnoid haemorrhage (SAH). Its pathogenesis has been incompletely elucidated, but vasospasm probably is a contributing factor. Experimental studies have suggested that calcium antagonists can prevent or reverse

  13. DNA strand breakage repair in ataxia telangiectasia fibroblast-like cells

    Energy Technology Data Exchange (ETDEWEB)

    Vincent, Jr, R A; Sheridan, III, R B; Huang, P C [Johns Hopkins Univ., Baltimore, Md. (USA). Dept. of Environmental and Biophysical Sciences

    1975-12-01

    Human diploid fibroblast-like cells derived from four patients with the genetic disease ataxia telangiectasia and from two non-mutant donors were examined for the repair of x-ray induced strand breaks in DNA. The ataxia telangiectasia cultures showed no significant differences from the non-mutant cultures in the kinetics and extent of strand repair. This suggests that the increased spontaneous and x-ray induced chromatid aberrations observed in ataxia telangiectasia cells are not caused by a defect in the repair of single strand breaks as might be suspected from a general model of aberration production.

  14. Diagnostic criteria for hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome)

    DEFF Research Database (Denmark)

    Shovlin, C L; Guttmacher, A E; Buscarini, E

    2000-01-01

    Hereditary Hemorrhagic Telangiectasia (HHT) is easily recognized in individuals displaying the classical triad of epistaxis, telangiectasia, and a suitable family history, but the disease is more difficult to diagnosis in many patients. Serious consequences may result if visceral arteriovenous ma...... in this disorder. These criteria may be refined as molecular diagnostic tests become available in the next few years....... of the HHT Foundation International, Inc., we present consensus clinical diagnostic criteria. The four criteria (epistaxes, telangiectasia, visceral lesions and an appropriate family history) are carefully delineated. The HHT diagnosis is definite if three criteria are present. A diagnosis of HHT cannot...

  15. Outcome after emergency surgery without angiography in patients with intracerebral haemorrhage after aneurysm rupture

    DEFF Research Database (Denmark)

    Bergdal, Ove; Springborg, Jacob; Hauerberg, John

    2009-01-01

    BACKGROUND: Patients with large intracerebral haematomas (ICH) from aneurysm rupture often present in a poor clinical condition and have a poor prognosis. Time delay for preoperative angiography might in some cases be unappealing. We evaluated the outcome after immediate haematoma removal...... and aneurysm occlusion without preoperative angiography. METHODS: We retrospectively identified 13 consecutive patients. We recorded clinical data and evaluated mortality and morbidity with the Glasgow Outcome Score (GOS) and Telephone Interview of Cognitive Status (TICS). FINDINGS: At follow up seven...... of thirteen patients had favourable outcome assessed by GOS. Three patients had severe disability and three patients died. None of the survived patients interviewed had impaired cognition. CONCLUSIONS: In patients presented in a critical state with aneurysmal ICH, emergency haematoma removal and aneurysm...

  16. Prevention of cardiovascular events in Asian patients with ischaemic stroke at high risk of cerebral haemorrhage (PICASSO): a multicentre, randomised controlled trial.

    Science.gov (United States)

    Kim, Bum Joon; Lee, Eun-Jae; Kwon, Sun U; Park, Jong-Ho; Kim, Yong-Jae; Hong, Keun-Sik; Wong, Lawrence K S; Yu, Sungwook; Hwang, Yang-Ha; Lee, Ji Sung; Lee, Juneyoung; Rha, Joung-Ho; Heo, Sung Hyuk; Ahn, Sung Hwan; Seo, Woo-Keun; Park, Jong-Moo; Lee, Ju-Hun; Kwon, Jee-Hyun; Sohn, Sung-Il; Jung, Jin-Man; Navarro, Jose C; Kang, Dong-Wha

    2018-06-01

    The optimal treatment for patients with ischaemic stroke with a high risk of cerebral haemorrhage is unclear. We assessed the efficacy and safety of cilostazol versus aspirin, with and without probucol, in these patients. In this randomised, controlled, 2 × 2 factorial trial, we enrolled patients with ischaemic stroke with a history of or imaging findings of intracerebral haemorrhage or two or more microbleeds from 67 centres in three Asian countries. Patients were randomly assigned (1:1:1:1) to receive oral cilostazol (100 mg twice a day), aspirin (100 mg once a day), cilostazol plus probucol (250 mg twice a day), or aspirin plus probucol with centralised blocks stratified by centre. Cilostazol versus aspirin was investigated double-blinded; probucol treatment was open-label, but the outcome assessor was masked to assignment. The co-primary outcomes were incidence of the composite of stroke, myocardial infarction, or vascular death (efficacy) and incidence of haemorrhagic stroke (safety), which were assessed in intention-to-treat and modified intention-to-treat populations. Efficacy was analysed with a non-inferiority test and a superiority test if non-inferiority was satisfied. Safety was assessed with a superiority test only. This trial is registered with ClinicalTrials.gov, NCT01013532. Between Aug 1, 2009, and Aug 31, 2015, we randomly assigned 1534 patients to one of the four study groups, of whom 1512 were assessed for the co-primary endpoints. During a median follow-up of 1·9 years (IQR 1·0-3·0), the incidence of composite vascular events was 4·27 per 100 person-years in patients who received cilostazol and 5·33 per 100 person-years in patients who received aspirin (HR 0·80, 95% CI 0·57-1·11; non-inferiority p=0·0077; superiority p=0·18). Incidence of cerebral haemorrhage was 0·61 per 100 person-years in patients who received cilostazol and 1·20 per 100 person-years in those who received aspirin (HR 0·51, 97·5% CI 0·20-1·27; superiority

  17. MRA versus digital subtraction angiography in acute subarachnoid haemorrhage: a blinded multireader study of prospectively recruited patients

    International Nuclear Information System (INIS)

    Jaeger, H.R.; Hausmann, O.; Moseley, I.F.; Taylor, W.J.; Mansmann, U.; Partzsch, U.

    2000-01-01

    We performed a blinded multireader study comparing MR angiography (MRA) with digital subtraction angiography (DSA) in 34 prospectively recruited patients who presented with acute subarachnoid haemorrhage (SAH). Two observers independently reviewed the MRA and DSA studies some months after clinical presentation. Presence of an aneurysm was rated on a 4-point confidence scale. Cases in which the initial interpretation of the observers varied were jointly reviewed to reach a consensus opinion. DSA was deliberately chosen not to represent the reference standard and the clinical course and surgical findings were used to explain significant differences between the consensus readings of MRA and DSA. Diagnostic confidence and interobserver agreement were, overall, higher on DSA than on MRA studies (κ DSA = 0.64 versus κ MRA = 0.52 with 95 % CI for Δ = κ DSA -κ MRA [-0.06, 0.31]). With both methods, discrepancies between observers were due to aneurysms overlooked rather than false-positive readings by one observer. Diagnostic accuracy therefore improved when the readings of the two observers were combined, particularly for MRA. Intermethod agreement was only fair and similar for both readers (κ reader 1 = 0.37 versus κ reader 2 = 0.32 with 95 % CI for Δ = κ reader 1 -κ reader 2 [-0.02, 0.11]). Both interobserver and intermethod agreements improved when the data were analysed on a per-study (positive or negative study) rather than on a per-aneurysm basis. Differences in the consensus reading were due to five aneurysms (four single and one multiple) detected only with MRA and five (two single and three multiple) detected only with DSA. MRA and DSA should be regarded as complementary in the investigation of patients with acute SAH. DSA can no longer be regarded as the reference standard. (orig.)

  18. Clinical practice guidelines in intracerebral haemorrhage.

    Science.gov (United States)

    Rodríguez-Yáñez, M; Castellanos, M; Freijo, M M; López Fernández, J C; Martí-Fàbregas, J; Nombela, F; Simal, P; Castillo, J; Díez-Tejedor, E; Fuentes, B; Alonso de Leciñana, M; Alvarez-Sabin, J; Arenillas, J; Calleja, S; Casado, I; Dávalos, A; Díaz-Otero, F; Egido, J A; Gállego, J; García Pastor, A; Gil-Núñez, A; Gilo, F; Irimia, P; Lago, A; Maestre, J; Masjuan, J; Martínez-Sánchez, P; Martínez-Vila, E; Molina, C; Morales, A; Purroy, F; Ribó, M; Roquer, J; Rubio, F; Segura, T; Serena, J; Tejada, J; Vivancos, J

    2013-05-01

    Intracerebral haemorrhage accounts for 10%-15% of all strokes; however it has a poor prognosis with higher rates of morbidity and mortality. Neurological deterioration is often observed during the first hours after onset and determines poor prognosis. Intracerebral haemorrhage, therefore, is a neurological emergency which must be diagnosed and treated properly as soon as possible. In this guide we review the diagnostic procedures and factors that influence the prognosis of patients with intracerebral haemorrhage and we establish recommendations for the therapeutic strategy, systematic diagnosis, acute treatment and secondary prevention for this condition. Copyright © 2011 Sociedad Española de Neurología. Published by Elsevier Espana. All rights reserved.

  19. Risk of haemorrhage from transurethral prostatectomy in acetylsalicylic acid and NSAID-treated patients

    DEFF Research Database (Denmark)

    Wierød, F S; Frandsen, N J; Jacobsen, J D

    1998-01-01

    the unselected clinical records of all patients undergoing TUR-P in the Department of Urology at Hvidovre Hospital (during 1992-1994) with special focus on the use of ASA and non-steroidal anti-inflammatory drugs (NSAIDs). In total, 457 records were examined: 99 patients on ASA/NSAID received 42 units of blood......, while 358 patients free from such medication received 68 units of blood, a significantly smaller amount (p = 0.0390). We conclude that ASA and NSAIDs increase the risk of bleeding during and after TUR-P, and we recommend the withdrawal of these drugs for one week before TUR-P....

  20. Genetic and cellular features of ataxia telangiectasia

    Energy Technology Data Exchange (ETDEWEB)

    Taylor, A M.R.; Byrd, P J; McConville, C M; Thacker, S [Birmingham Univ. (United Kingdom). CRC Dept. of Cancer Studies

    1994-01-01

    Ataxia telangiectasia (AT) is a developmental disorder in which many organ systems are affected. The children are recognized by a progressive cerebellar deterioration. The gene for AT has now been localized to a region of chromosome 11q22-23 of no more than 3Mb in size and its product appears to be involved directly or indirectly in some form of DNA recombination. Patients and their cells are unusually sensitive to ionizing radiation and various radiometric drugs. Observations on the progressive nature of the disorder, with loss of selected cells or failure to develop normally, might be compatible with the pathological effect of an inability to correctly regulate apoptosis in some cell lineages. While this is an intriguing speculation, there is, at present, no evidence for such a defect in AT. (author).

  1. Complementation analysis of ataxia-telangiectasia

    International Nuclear Information System (INIS)

    Jaspers, N.G.; Painter, R.B.; Paterson, M.C.; Kidson, C.; Inoue, T.

    1985-01-01

    In a number of laboratories genetic analysis of ataxia-telangiectasia (AT) has been performed by studying the expression of the AT phenotype in fused somatic cells or mixtures of cell-free extracts from different patients. Complementation of the defective response to ionizing radiation was observed frequently, considering four different parameters for radiosensitivity in AT. The combined results from studies on cultured fibroblasts or lymphoblastoid cells from 17 unrelated families revealed the presence of at least four and possibly nine complementation groups. These findings suggest that there is an extensive genetic heterogeneity in AT. More extensive studies are needed for an integration of these data and to provide a set of genetically characterized cell strains for future research of the AT genetic defect

  2. Viral Haemorrhagic Septicaemia Virus

    DEFF Research Database (Denmark)

    Olesen, Niels Jørgen; Skall, Helle Frank

    2013-01-01

    This chapter covers the genetics (genotypes and serotypes), clinical signs, host species, transmission, prevalence, diagnosis, control and prevention of viral haemorrhagic septicaemia virus.......This chapter covers the genetics (genotypes and serotypes), clinical signs, host species, transmission, prevalence, diagnosis, control and prevention of viral haemorrhagic septicaemia virus....

  3. Cell biological study on ataxia-telangiectasia

    International Nuclear Information System (INIS)

    Ohta, Shigeru; Katsura, Tadahiko; Shimada, Morimi; Shima, Akihiro; Chishiro, Hiroko; Kasahara, Yoshitaka.

    1985-01-01

    Diagnosis of ataxia-telangiectasia (AT) has largely been dependent on the clinical findings such as cerebellar ataxia, telangiectasia, and immunological deficiency. However, diagnosis of AT by these ordinary criteria is sometimes not sufficient because of the lack of immunological abnormalities. We examined three cases of AT by ordinary clinical criteria and also by X-ray sensitivity of cultured skin fibroblasts. Case 1, a 9-year-old boy, revealed typical clinical features of AT. However, he had no abnormality in serum IgA or IgE. Case 2, a 10-year-old boy, showed decreased serum IgA level. Case 3, a 19-year-old female, had typical clinical features of AT with normal serum IgA, and developed papillary adenocarcinoma of thyroid which was surgically removed. Fibroblast strains derived from these three cases of AT and from the parents of Case 3 were examined with regard to X-ray sensitivity. Three fibroblast strains derived from AT patients (AT homozygotes) showed remarkable hypersensitivity to X-ray. Fibroblast strains derived from the parents (AT heterozygotes) of Case 3, however, showed normal X-ray sensitivity. Recently, AT fibroblasts have been known to show hypersensitivity also to some mutagen like neocarzinostazin as reported by Shiloh et al. Fibroblasts from Case 3 revealed hypersensitivity to neocarzinostazin. However, the sensitivity of the strains from AT heterozygotes (the parents of Case 3) showed no apparent difference from that of control cells. The assay system for mutagen is quite unstable and proper conditioning of the seeding cell number is important for the carrier detection. However, the diagnosis of AT homozygotes was definitely established by X-ray irradiation to cultured fibroblasts from patients. (author)

  4. Subarachnoid Haemorrhage and Sports.

    Science.gov (United States)

    Sousa Nanji, Liliana; Melo, Teresa P; Canhão, Patrícia; Fonseca, Ana Catarina; Ferro, José Manuel

    2015-01-01

    Some cases of subarachnoid haemorrhage (SAH) have been associated with vigorous physical activity, including sports. Our research aimed to describe the association between SAH and sports and to identify the types of sports that were more frequently found as precipitating factors in a tertiary single-centre SAH register. We retrieved information from a prospectively collected SAH registry and reviewed discharge notes of acute SAH patients admitted to the Stroke Unit of Hospital de Santa Maria, Lisbon, between 1995 and 2014. Out of 738 patients included in the analysis, 424 (57.5%) cases of SAH were preceded by physical activity. Nine cases (1.2%) were associated with sports, namely running (2 cases), aerobics (2 cases), cycling, body balance, dance, surf and windsurf. Patients with SAH while practicing sports were younger than controls (average age 43.1 vs. 57.0 years; p = 0.007). In 1 patient, there was a report of trauma to the neck. Patients in the sports group only had Hunt and Hess scale grades 1 (11.1%) or 2 (88.9%) at admission, while patients in the control group had a wider distribution in severity. Our findings indicate that SAH precipitated by sports is not very frequent and is uncommonly related to trauma. Patients who suffered SAH associated with sports were younger and apparently had a milder clinical presentation.

  5. Fresh frozen plasma versus prothrombin complex concentrate in patients with intracranial haemorrhage related to vitamin K antagonists (INCH)

    DEFF Research Database (Denmark)

    Steiner, Thorsten; Poli, Sven; Griebe, Martin

    2016-01-01

    BACKGROUND: Haematoma expansion is a major cause of mortality in intracranial haemorrhage related to vitamin K antagonists (VKA-ICH). Normalisation of the international normalised ratio (INR) is recommended, but optimum haemostatic management is controversial. We assessed the safety and efficacy ...

  6. Erythropoietin in patients with aneurysmal subarachnoid haemorrhage: a double blind randomised clinical trial

    DEFF Research Database (Denmark)

    Springborg, J B; Møller, C; Gideon, P

    2007-01-01

    but preliminarily terminated because of a lower than expected inclusion rate. However, 73 patients were randomised to treatment with EPO (500 IU/kg/day for three days) or placebo. The primary endpoint was Glasgow Outcome Score at six months. We further studied surrogate measures of secondary ischaemia, i...

  7. Stroke and recurrent haemorrhage associated with antithrombotic treatment after gastrointestinal bleeding in patients with atrial fibrillation

    DEFF Research Database (Denmark)

    Staerk, Laila; Lip, Gregory Y H; Olesen, Jonas B

    2015-01-01

    with better outcomes for all cause mortality and thromboembolism compared with patients who did not resume treatment. This was despite an increased longitudinal associated risk of bleeding. FUNDING, COMPETING INTERESTS, DATA SHARING: This study was supported by a grant from Boehringer-Ingelheim. Competing...

  8. [Re-bleeding predictors in patients with aneurysmal subarachnoid haemorrhage and delayed neurosurgical treatment].

    Science.gov (United States)

    Rivero Rodríguez, Dannys; Scherle Matamoros, Claudio; Fernández Cúe, Leda; Miranda Hernández, José Luis; Pernas Sánchez, Yanelis; Pérez Nellar, Jesús

    2016-01-01

    To evaluate the re-bleeding predictors in patients with delayed treatment of aneurysmal subarachnoid hemorrhage. A prospective cohort study enrolled 261 patients with aneurysmal subarachnoid hemorrhage, attending in Hermanos Ameijeiras Hospital from October 2005, and June 2014. An increased re-bleeding risk in the multivariate analysis was associated with grade III (OR 2.01; 95% CI; 1.06-3.84) and grade IV (OR 3.84; 95% CI; 2.06-7.31) on World Federation Neurological Surgeon (WFNS) scale; grade III (OR 2.04; 95% CI; 1.01-4.13) and grade IV (OR 2.12, 95% CI; 1.05-4.28) on the Fischer scale, aneurism location in posterior circulation (OR 2.45, 95% CI; 1.33-4.44), and anterior communicant artery (OR 1.57, 95% CI;1.00-2.46). Hypertension history was present in 60.9% (159 patients) and was also associated with risk of re-bleeding (OR 2.70, 95% CI; 1.00-7.30). Blood pressure, haematocrit, glycemic, aneurysm size, multiple aneurysms, and location in the middle cerebral artery, do not show any relationship. Hypertension history, poor grade (III and IV) on WFNS and Fisher scale and aneurysm location were independent risks factors of re-bleeding in patients with delayed aneurysmal treatment. Copyright © 2014 Sociedad Española de Neurocirugía. Published by Elsevier España. All rights reserved.

  9. Stroke and recurrent haemorrhage associated with antithrombotic treatment after gastrointestinal bleeding in patients with atrial fibrillation

    DEFF Research Database (Denmark)

    Staerk, Laila; Lip, Gregory Y.H.; Olesen, Jonas B.

    2015-01-01

    Study question What are the risks of all cause mortality, thromboembolism, major bleeding, and recurrent gastrointestinal bleeding associated with restarting antithrombotic treatment after gastrointestinal bleeding in patients with atrial fibrillation? Methods This Danish cohort study (1996...... to avoid confounding from use of previously prescribed drugs on discharge. Risks of all cause mortality, thromboembolism, major bleeding, and recurrent gastrointestinal bleeding were estimated with competing risks models and time dependent multiple Cox regression models. Study answer and limitations 4602...... gastrointestinal bleeding. 27.1% (n=924) of patients did not resume antithrombotic treatment. Compared with non-resumption of treatment, a reduced risk of all cause mortality was found in association with restart of oral anticoagulation (hazard ratio 0.39, 95% confidence interval 0.34 to 0.46), an antiplatelet...

  10. White-centred retinal haemorrhages (Roth spots).

    OpenAIRE

    Ling, R.; James, B.

    1998-01-01

    Roth spots (white-centred retinal haemorrhages) were classically described as septic emboli lodged in the retina of patients with subacute bacterial endocarditis. Indeed many have considered Roth spots pathognomonic for this condition. More recent histological evidence suggests, however, that they are not foci of bacterial abscess. Instead, they are nonspecific and may be found in many other diseases. A review of the histology and the pathogenesis of these white-centred haemorrhages will be p...

  11. Initial clinical experience with dual-layer detector spectral CT in patients with acute intracerebral haemorrhage: A single-centre pilot study.

    Directory of Open Access Journals (Sweden)

    Soo Buem Cho

    Full Text Available The purpose of this study was to investigate the clinical feasibility of spectral analyses using dual-layer detector spectral computed tomography (CT in acute intracerebral haemorrhage (ICH.We retrospectively reviewed patients with acute ICH who underwent CT angiography on a dual-layer detector spectral CT scanner. A spectral data analysis was performed to detect contrast enhancement in or adjacent to acute ICH by using spectral image reconstructions including monoenergetic (MonoE, virtual noncontrast (VNC, and iodine overlay fusion images. We also acquired a spectral plot to assess material differentiation within lesions.Among the 30 patients, the most common cause of acute ICH was chronic hypertension (18/30, 60% followed by trauma (5/30, 16.7%, brain tumour (3/30, 10%, Moyamoya disease (2/30, 6.7%, and haemorrhagic diathesis from anticoagulation therapy (2/30, 6.7%. Of 30 patients, 13 showed suboptimal iodine suppression in the subcalvarial spaces on VNC images compared with true noncontrast images. The CT angiographic spot sign within the acute ICH was detected in four patients (4/30, 13.3%. All three tumours were metastatic and included lung cancer (n = 2 and hepatocellular carcinoma (n = 1 which showed conspicuous delineation of an enhancing tumour portion in the spectral analysis. Spectral analyses allowed the discrimination of acute haemorrhage and iodine with enhanced lesion visualization on the MonoE images obtained at lower keVs (less than 70 keV and spectral plot.Even though the image quality of VNC is perceived to be inferior, it is feasible to evaluate acute ICH in clinical settings using dual-layer detector spectral CT. The MonoE images taken at lower keVs were useful for depicting contrast enhancing lesion, and spectral plot might be helpful for material differentiation in patients with acute ICH.

  12. Initial clinical experience with dual-layer detector spectral CT in patients with acute intracerebral haemorrhage: A single-centre pilot study.

    Science.gov (United States)

    Cho, Soo Buem; Baek, Hye Jin; Ryu, Kyeong Hwa; Moon, Jin Il; Choi, Bo Hwa; Park, Sung Eun; Bae, Kyungsoo; Jeon, Kyung Nyeo; Kim, Dong Wook

    2017-01-01

    The purpose of this study was to investigate the clinical feasibility of spectral analyses using dual-layer detector spectral computed tomography (CT) in acute intracerebral haemorrhage (ICH). We retrospectively reviewed patients with acute ICH who underwent CT angiography on a dual-layer detector spectral CT scanner. A spectral data analysis was performed to detect contrast enhancement in or adjacent to acute ICH by using spectral image reconstructions including monoenergetic (MonoE), virtual noncontrast (VNC), and iodine overlay fusion images. We also acquired a spectral plot to assess material differentiation within lesions. Among the 30 patients, the most common cause of acute ICH was chronic hypertension (18/30, 60%) followed by trauma (5/30, 16.7%), brain tumour (3/30, 10%), Moyamoya disease (2/30, 6.7%), and haemorrhagic diathesis from anticoagulation therapy (2/30, 6.7%). Of 30 patients, 13 showed suboptimal iodine suppression in the subcalvarial spaces on VNC images compared with true noncontrast images. The CT angiographic spot sign within the acute ICH was detected in four patients (4/30, 13.3%). All three tumours were metastatic and included lung cancer (n = 2) and hepatocellular carcinoma (n = 1) which showed conspicuous delineation of an enhancing tumour portion in the spectral analysis. Spectral analyses allowed the discrimination of acute haemorrhage and iodine with enhanced lesion visualization on the MonoE images obtained at lower keVs (less than 70 keV) and spectral plot. Even though the image quality of VNC is perceived to be inferior, it is feasible to evaluate acute ICH in clinical settings using dual-layer detector spectral CT. The MonoE images taken at lower keVs were useful for depicting contrast enhancing lesion, and spectral plot might be helpful for material differentiation in patients with acute ICH.

  13. Hereditary hemorrhagic telangiectasia: progress of diagnostic imaging and vascular therapeutic embolization

    International Nuclear Information System (INIS)

    Lu Chuan; Liu Zuoqin

    2008-01-01

    Hereditary haemorrhagic telangiectasia (HHT) is a genetic autosomal-dominant disorder characterized by the presence of epistaxis, vascular telangiectasis in mucosal and cutaneous tissues, with visceral lesions and family history. However, many specialists or radiologists are still in lack of appreciation concerning the full range of consequences in diagnosis and their family relationship resulting the poor recognition of the disease. Understanding the diagnostic imaging and therapeutic measure for HHT will be critical, because of the continuous growth and risk existance of these arteriovenous malformations arousing early diagnosis, proper treatment, adequate follow-up and screening of the family. (authors)

  14. Computed tomography of the brain in predicting outcome of traumatic intracranial haemorrhage in Malaysian patients

    International Nuclear Information System (INIS)

    Azian, A.A.; Nurulazman, A.A.; Shuaib, I.L.; Mahayidin, M.; Ariff, A.R.; Naing, N.N.; Abdullah, J.

    2001-01-01

    Head injury is a significant economic, social and medical problem all over the world. Road accidents are the most frequent cause of head injury in Malaysia which highest risk in the young (15 to 24 years old). The associated outcomes include good recovery, possibility of death for the severely injured, which may cause disruption of the lives of their family members. It is important to predict the outcome as it will provide sound information to assist clinicians in Malaysia in providing prognostic information to patients and their families, to assess the effectiveness of different modes of treatment in promoting recovery and to document the significance of head injury as a public health problem. Results. A total of 103 cases with intracranial hemorrhage i.e. intracerebral hemorrhage, extradural hemorrhage, subdural hemorrhage, intraventricular hemorrhage, hemorrhagic contusion and subarachnoid hemorrhage, following motor vehicle accidents was undertaken to study factors contributing to either good or poor outcome according to the Glasgow outcome scale. Patients below 12 years of age were excluded. The end point of the study was taken at 24 months post injury. The selected variables were incorporated into models generated by logistic regression techniques of multivariate analysis to see the significant predictors of outcome as well as the correlation between the CT findings with GCS. Conclusion. Significant predictors of outcome were GCS on arrival in the accident emergency department, pupillary reflex and the CT scan findings. The CT predictors of outcome include ICH, EDH, IVH, present of SAH, site of ICH, volumes of EDH and SDH as well as midline shift. (author)

  15. Churg-Strauss Syndrome as an Unusual Aetiology of Stroke with Haemorrhagic Transformation in a Patient with No Cardiovascular Risk Factors

    Directory of Open Access Journals (Sweden)

    Tiina Sairanen

    2011-01-01

    Full Text Available Background: We present here a case of haemorrhagic brain infarction in a middle-aged and physically active male, who had never smoked. This case report aims to remind the internist and neurologist to bear in mind unusual aetiologies of brain infarcts in patients without classical cardiovascular risk factors. Case Description: A 49-year-old male with pulmonary asthma and a prior history of nasal polyps had a wake-up stroke with left-sided symptoms and speech disturbance. A head MRI and MR angiography revealed a recent haemorrhagic infarct in the right putamen and corona radiata. The left hemiparesis progressed to sensory-motor hemiplegia on the 4th day. In the head CT, it was shown that the haemorrhagic infarct had progressed to a large haematoma. A pansinusitis was also diagnosed. The aetiological investigations revealed a minor atrial septal defect (ASD with shunting and a heterozygotic clotting factor V R506Q mutation. A remarkable blood eosinophilia of 9.80 E9/l (42% together with fever, sinusitis, wide-spread bilateral nodular pulmonary infiltrates that did not respond to wide-spectrum antimicrobial treatment, positive anti-neutrophilic cytoplasmic antibodies, a high myeloperoxidase antibody level and slightly positive anti-proteinase 3 antibodies suggested the diagnosis of Churg-Strauss syndrome. These inflammatory symptoms and findings promptly responded to treatment with corticosteroids and cyclophosphamide. Conclusions: Even after the concomitant findings of the low risk factors, i.e. small ASD and heterozygotic clotting factor mutation, continued search for the final aetiology of stroke revealed Churg-Strauss syndrome, which was the key to the treatment.

  16. Churg-Strauss Syndrome as an Unusual Aetiology of Stroke with Haemorrhagic Transformation in a Patient with No Cardiovascular Risk Factors

    Science.gov (United States)

    Sairanen, Tiina; Kanerva, Mari; Valanne, Leena; Lyytinen, Jukka; Pekkonen, Eero

    2011-01-01

    Background We present here a case of haemorrhagic brain infarction in a middle-aged and physically active male, who had never smoked. This case report aims to remind the internist and neurologist to bear in mind unusual aetiologies of brain infarcts in patients without classical cardiovascular risk factors. Case Description A 49-year-old male with pulmonary asthma and a prior history of nasal polyps had a wake-up stroke with left-sided symptoms and speech disturbance. A head MRI and MR angiography revealed a recent haemorrhagic infarct in the right putamen and corona radiata. The left hemiparesis progressed to sensory-motor hemiplegia on the 4th day. In the head CT, it was shown that the haemorrhagic infarct had progressed to a large haematoma. A pansinusitis was also diagnosed. The aetiological investigations revealed a minor atrial septal defect (ASD) with shunting and a heterozygotic clotting factor V R506Q mutation. A remarkable blood eosinophilia of 9.80 E9/l (42%) together with fever, sinusitis, wide-spread bilateral nodular pulmonary infiltrates that did not respond to wide-spectrum antimicrobial treatment, positive anti-neutrophilic cytoplasmic antibodies, a high myeloperoxidase antibody level and slightly positive anti-proteinase 3 antibodies suggested the diagnosis of Churg-Strauss syndrome. These inflammatory symptoms and findings promptly responded to treatment with corticosteroids and cyclophosphamide. Conclusions Even after the concomitant findings of the low risk factors, i.e. small ASD and heterozygotic clotting factor mutation, continued search for the final aetiology of stroke revealed Churg-Strauss syndrome, which was the key to the treatment. PMID:21468361

  17. Hepatic telangiectasia in Osler's disease treated with arterial embolization

    Energy Technology Data Exchange (ETDEWEB)

    Goethlin, J H; Nordgard, K; Jonsson, K; Nyman, U

    1982-02-01

    Hepatic hereditary telangiectasia in 2 females was treated with hepatic artery embolization. In one patient both peripheral and central arterial occlusion was performed; the patient died of massive gastro-intestinal bleeding 2 months later. Autopsy showed extensive regions with necrosis in the right liver lobe. In the next patient only central occlusion of the right hepatic artery was performed and the arterio-venous shunting in the left liver lobe left remaining. After a long reconvalescence period the patient recovered completely. It is advocated to centrally occlude only that hepatic artery supplying the most affected parts of the liver in Osler's disease. Thus extensive necrosis with possible ensuing death may be avoided.

  18. Ebolavirus and Haemorrhagic Syndrome

    Directory of Open Access Journals (Sweden)

    Gerald A. Matua

    2015-05-01

    Full Text Available The Ebola virus is a highly virulent, single-stranded ribonucleic acid virus which affects both humans and apes and has fast become one of the world’s most feared pathogens. The virus induces acute fever and death, with haemorrhagic syndrome occurring in up to 90% of patients. The known species within the genus Ebolavirus are Bundibugyo, Sudan, Zaïre, Reston and Taï Forest. Although endemic in Africa, Ebola has caused worldwide anxiety due to media hype and concerns about its international spread, including through bioterrorism. The high fatality rate is attributed to unavailability of a standard treatment regimen or vaccine. The disease is frightening since it is characterised by rapid immune suppression and systemic inflammatory response, causing multi-organ and system failure, shock and often death. Currently, disease management is largely supportive, with containment efforts geared towards mitigating the spread of the virus. This review describes the classification, morphology, infective process, natural ecology, transmission, epidemic patterns, diagnosis, clinical features and immunology of Ebola, including management and epidemic containment strategies.

  19. Ebolavirus and Haemorrhagic Syndrome.

    Science.gov (United States)

    Matua, Gerald A; Van der Wal, Dirk M; Locsin, Rozzano C

    2015-05-01

    The Ebola virus is a highly virulent, single-stranded ribonucleic acid virus which affects both humans and apes and has fast become one of the world's most feared pathogens. The virus induces acute fever and death, with haemorrhagic syndrome occurring in up to 90% of patients. The known species within the genus Ebolavirus are Bundibugyo, Sudan, Zaïre, Reston and Taï Forest. Although endemic in Africa, Ebola has caused worldwide anxiety due to media hype and concerns about its international spread, including through bioterrorism. The high fatality rate is attributed to unavailability of a standard treatment regimen or vaccine. The disease is frightening since it is characterised by rapid immune suppression and systemic inflammatory response, causing multi-organ and system failure, shock and often death. Currently, disease management is largely supportive, with containment efforts geared towards mitigating the spread of the virus. This review describes the classification, morphology, infective process, natural ecology, transmission, epidemic patterns, diagnosis, clinical features and immunology of Ebola, including management and epidemic containment strategies.

  20. Haemorrhage in pregnancy: information given to women in Chiradzulu (Malawi

    Directory of Open Access Journals (Sweden)

    H Kapyepye

    2006-09-01

    Full Text Available Advising women on , haemorrhage in pregnancy could, be viewed, as an integral aspect of maternal health care in M alawi. The WHO (1999 confirmed, that haemorrhage in pregnancy was not only a direct reason for maternal mortality but also a major cause of maternal death. The question on the nature of information that midwives and traditional birth attendants (referred to as TBA’s in the Chiradzulu district in Malawi gave with regard to haemorrhage in pregnancy, therefore arose. Research available focused on the women’s knowledge about the complications of pregnancy but not on the nature of information women received from midwives and TBA’s. This study explored and described the nature of information that was given to rural women in the Chiradzulu district by the midwives and TBA’s regarding haemorrhage in pregnancy. The findings revealed that although both the midwives and TBA’s included important information about haemorrhage in pregnancy, there were deficiencies in some critical areas. Examples of these deficiencies were the definition of haemorrhage in pregnancy; the predisposing factors for antepartum and postpartum haemorrhage and deficiencies in the nature of information on the management and referral of haemorrhaging patients. The findings provided insights into the nature of the information that was provided to the women regarding haemorrhage in pregnancy in the Chiradzulu district in Malawi. Thereafter guidelines were developed for the provision of this information. Finally a follow-up study was recommended after implementation of these guidelines in the district to evaluate the change in the nature of the information communicated to patients regarding haemorrhage by midwives and TBA’s. In this study, haemorrhage during pregnancy referred to the perinatal phase, including antepartum, intrapartum and postpartum haemorrhage.

  1. CLINICAL APPROACH TO HEREDITARY HEMORRHAGIC TELANGIECTASIA

    Directory of Open Access Journals (Sweden)

    Mary Hachmeriyan

    2013-11-01

    Full Text Available Background: Hereditary hemorrhagic telangiectasia (HHT or Rendu-Osler-Weber disease is a rare syndrome, inherited as an autosomal dominant trait with incidence of 1/10000. The clinical manifestations are due to vascular malformations and predisposition to hemorrhages in different organs, the leading symptom being recurrent epistaxis. If diagnosed with HHT, the patient and his relatives and especially children have to be screened for occult vascular malformations.Case report: A 30 years old woman was treated for cerebral stroke, epistaxis, anemia, arterio-venous malformations for over 6 months. Only at this point she was diagnosed with HHT, after noticing the typical mucosal changes. Focused family history revealed symptoms of HHT in her only child, her father, aunt and two cousins The child was screened for occult vascular malformations – attainment of the nasal mucosa, lungs, gastrointestinal system, liver and brain. Pulmonary and gastrointestinal arterio-venous malformations were proven.Conclusion: Any case of recurrent epistaxis should be evaluated for HHT. After confirmation of the diagnosis every patient and close relatives have to be screened for attainment of other organs and followed up in order to prevent severe life threatening complications.

  2. Prophylactic ethamsylate for periventricular haemorrhage.

    OpenAIRE

    Cooke, R W; Morgan, M E

    1984-01-01

    Drug prophylaxis with ethamsylate for periventricular haemorrhage in very low birthweight infants significantly reduced the incidence of periventricular haemorrhage in survivors. A reduction in abnormalities at follow up and in insertion of ventriculoperitoneal shunts was also noted.

  3. Desmopressin Acetate in Intracranial Haemorrhage

    Directory of Open Access Journals (Sweden)

    Thomas Kapapa

    2014-01-01

    Full Text Available Introduction. The secondary increase in the size of intracranial haematomas as a result of spontaneous haemorrhage or trauma is of particular relevance in the event of prior intake of platelet aggregation inhibitors. We describe the effect of desmopressin acetate as a means of temporarily stabilising the platelet function. Patients and Methods. The platelet function was analysed in 10 patients who had received single (N=4 or multiple (N=6 doses of acetylsalicylic acid and 3 patients (control group who had not taken acetylsalicylic acid. All subjects had suffered intracranial haemorrhage. Analysis was performed before, half an hour and three hours after administration of desmopressin acetate. Statistical analysis was performed by applying a level of significance of P≤0.05. Results. (1 Platelet function returned to normal 30 minutes after administration of desmopressin acetate. (2 The platelet function worsened again after three hours. (3 There were no complications related to electrolytes or fluid balance. Conclusion. Desmopressin acetate can stabilise the platelet function in neurosurgical patients who have received acetylsalicylic acid prior to surgery without causing transfusion-related side effects or a loss of time. The effect is, however, limited and influenced by the frequency of drug intake. Further controls are needed in neurosurgical patients.

  4. Catastrophic cerebral antiphospholipid syndrome presenting as cerebral infarction with haemorrhagic transformation after sudden withdrawal of warfarin in a patient with primary antiphospholipid syndrome

    Science.gov (United States)

    Wani, Abdul Majid; Hussain, Waleed Mohd; Mejally, Mousa Ali Al; Ali, Khaled Shawkat; Raja, Sadeya Hanif; Maimani, Wael Al; Bafaraj, Mazen G; Bashraheel, Ashraf; Akhtar, Mubeena; Khoujah, Amer Mohd

    2010-01-01

    Catastrophic antiphospholipid syndrome (APS) is caused by thrombotic vascular occlusions that affect both small and large vessels, producing ischaemia in the affected organs. The “catastrophic” variant of the antiphospholipid syndrome (cAPS) develops over a short period of time. Although patients with cAPS represent <1% of all patients with APS, they are usually life threatening with a 50% mortality rate. A strong association with concomitant infection is thought to act as the main trigger of microthromboses in cAPS. Several theories have been proposed to explain these physiopathological features. Some of them suggest the possibility of molecular mimicry between components of infectious microorganisms and natural anticoagulants, which might be involved in the production of cross-reacting antiphospholipid antibodies. We present a case of catastrophic cerebral APS characterised by massive temporal lobe infarction and subsequent haemorrhagic transformation after sudden withdrawal of warfarin. PMID:22242060

  5. Olivary degeneration after cerebellar or brain stem haemorrhage: MRI

    Energy Technology Data Exchange (ETDEWEB)

    Uchino, A. (Dept. of Radiology, Kyushu Univ. Hospital, Fukuoka (Japan) Dept. of Radiology, Kyushu Rosai Hospital, Kitakyushu (Japan)); Hasuo, K. (Dept. of Radiology, Kyushu Univ. Hospital, Fukuoka (Japan)); Uchida, K. (Dept. of Radiology, Kyushu Rosai Hospital, Kitakyushu (Japan)); Matsumoto, S. (Dept. of Radiology, Kyushu Univ. Hospital, Fukuoka (Japan)); Tsukamoto, Y. (Dept. of Radiology, Kyushu Rosai Hospital, Kitakyushu (Japan)); Ohno, M. (Dept. of Radiology, Kyushu Rosai Hospital, Kitakyushu (Japan)); Masuda, K. (Dept. of Radiology, Kyushu Univ. Hospital, Fukuoka (Japan))

    1993-05-01

    Magnetic resonance (MR) images of seven patients with olivary degeneration caused by cerebellar or brain stem haemorrhages were reviewed. In four patients with cerebellar haemorrhage, old haematomas were identified as being located in the dentate nucleus; the contralateral inferior olivary nuclei were hyperintense on proton-density- and T2-weighted images. In two patients with pontine haemorrhages, the old haematomas were in the tegmentum and the ipsilateral inferior olivary nuclei, which were hyperintense. In one case of midbrain haemorrhage, the inferior olivary nuclei were hyperintense bilaterally. The briefest interval from the ictus to MRI was 2 months. Hypertrophic olivary nuclei were observed only at least 4 months after the ictus. Olivary degeneration after cerebellar or brain stem haemorrhage should not be confused with ischaemic, neoplastic, or other primary pathological conditions of the medulla. (orig.)

  6. Haemorrhagic necrosis of the grey matter of the spinal cord due to accidental injection of iopamidol in a patient with multiple neurofibromas; a clinico-pathological study

    International Nuclear Information System (INIS)

    Roos, R.A.C.; Wintzen, A.R.; Voormolen, J.H.C.; Vielvoye, G.J.; Bots, G.T.A.M.

    1988-01-01

    Cervical laminectomy was performed in a 34-year old man with multiple spinal neurofibromas because of a slowly progressive medullary compression. Four weeks later a rapid deterioration necessitated iopamidol myelography by left lateral cervical puncture at C2 level. Despite the establishment of adequate spinal fluid contact, resulting in imaging of the subarachnoid space, part of the contrast medium entered the spinal cord, thus delineating a syrinx from the upper cervical extending to the upper thoracic level. After the puncture the patient developed triplegia, involving the left arm and both legs and a paresis of the right arm. He died from aspiration pneumonia. Autopsy revealed haemorrhagic necrosis of the spinal grey matter. This adverse effect of myelography is argued to have been conditioned by the extreme immobility and displacement of the spinal cord due to the presence of multiple neurofibromas. The deterioration four weeks after the operation was probably caused by a further compression of the spinal cord. (orig.)

  7. Comparative human cellular radiosensitivity: I. The effect of SV40 transformation and immortalisation on the gamma-irradiation survival of skin derived fibroblasts from normal individuals and from ataxia-telangiectasia patients and heterozygotes.

    Science.gov (United States)

    Arlett, C F; Green, M H; Priestley, A; Harcourt, S A; Mayne, L V

    1988-12-01

    We have compared cell killing following 60Co gamma irradiation in 22 primary human fibroblast strains, nine SV40-immortalized human fibroblast lines and seven SV40-transformed pre-crisis human fibroblast cultures. We have examined material from normal individuals, from ataxia-telangiectasia (A-T) patients and from A-T heterozygotes. We have confirmed the greater sensitivity of A-T derived cells to gamma radiation. The distinction between A-T and normal cells is maintained in cells immortalized by SV40 virus but the immortal cells are more gamma radiation resistant than the corresponding primary fibroblasts. Cells transformed by plasmids (pSV3gpt and pSV3neo) expressing SV40 T-antigen, both pre- and post-crisis, show this increased resistance, indicating that it is expression of SV40 T-antigen, rather than immortalization per se which is responsible for the change. We use D0, obtained from a straight line fit, and D, estimated from a multitarget curve, as parameters to compare radiosensitivity. We suggest that both have their advantages; D0 is perhaps more reproducible, but D is more realistic when comparing shouldered and non-shouldered data.

  8. Evaluation of a metal artifact reduction algorithm applied to post-interventional flat detector CT in comparison to pre-treatment CT in patients with acute subarachnoid haemorrhage

    Energy Technology Data Exchange (ETDEWEB)

    Mennecke, Angelika; Svergun, Stanislav; Doerfler, Arnd; Struffert, Tobias [University of Erlangen-Nuremberg, Department of Neuroradiology, Erlangen (Germany); Scholz, Bernhard [Siemens Healthcare GmbH, Forchheim (Germany); Royalty, Kevin [Siemens Medical Solutions, USA, Inc., Hoffman Estates, IL (United States)

    2017-01-15

    Metal artefacts can impair accurate diagnosis of haemorrhage using flat detector CT (FD-CT), especially after aneurysm coiling. Within this work we evaluate a prototype metal artefact reduction algorithm by comparison of the artefact-reduced and the non-artefact-reduced FD-CT images to pre-treatment FD-CT and multi-slice CT images. Twenty-five patients with acute aneurysmal subarachnoid haemorrhage (SAH) were selected retrospectively. FD-CT and multi-slice CT before endovascular treatment as well as FD-CT data sets after treatment were available for all patients. The algorithm was applied to post-treatment FD-CT. The effect of the algorithm was evaluated utilizing the pre-post concordance of a modified Fisher score, a subjective image quality assessment, the range of the Hounsfield units within three ROIs, and the pre-post slice-wise Pearson correlation. The pre-post concordance of the modified Fisher score, the subjective image quality, and the pre-post correlation of the ranges of the Hounsfield units were significantly higher for artefact-reduced than for non-artefact-reduced images. Within the metal-affected slices, the pre-post slice-wise Pearson correlation coefficient was higher for artefact-reduced than for non-artefact-reduced images. The overall diagnostic quality of the artefact-reduced images was improved and reached the level of the pre-interventional FD-CT images. The metal-unaffected parts of the image were not modified. (orig.)

  9. Evaluation of a metal artifact reduction algorithm applied to post-interventional flat detector CT in comparison to pre-treatment CT in patients with acute subarachnoid haemorrhage

    International Nuclear Information System (INIS)

    Mennecke, Angelika; Svergun, Stanislav; Doerfler, Arnd; Struffert, Tobias; Scholz, Bernhard; Royalty, Kevin

    2017-01-01

    Metal artefacts can impair accurate diagnosis of haemorrhage using flat detector CT (FD-CT), especially after aneurysm coiling. Within this work we evaluate a prototype metal artefact reduction algorithm by comparison of the artefact-reduced and the non-artefact-reduced FD-CT images to pre-treatment FD-CT and multi-slice CT images. Twenty-five patients with acute aneurysmal subarachnoid haemorrhage (SAH) were selected retrospectively. FD-CT and multi-slice CT before endovascular treatment as well as FD-CT data sets after treatment were available for all patients. The algorithm was applied to post-treatment FD-CT. The effect of the algorithm was evaluated utilizing the pre-post concordance of a modified Fisher score, a subjective image quality assessment, the range of the Hounsfield units within three ROIs, and the pre-post slice-wise Pearson correlation. The pre-post concordance of the modified Fisher score, the subjective image quality, and the pre-post correlation of the ranges of the Hounsfield units were significantly higher for artefact-reduced than for non-artefact-reduced images. Within the metal-affected slices, the pre-post slice-wise Pearson correlation coefficient was higher for artefact-reduced than for non-artefact-reduced images. The overall diagnostic quality of the artefact-reduced images was improved and reached the level of the pre-interventional FD-CT images. The metal-unaffected parts of the image were not modified. (orig.)

  10. White-centred retinal haemorrhages (Roth spots).

    Science.gov (United States)

    Ling, R; James, B

    1998-10-01

    Roth spots (white-centred retinal haemorrhages) were classically described as septic emboli lodged in the retina of patients with subacute bacterial endocarditis. Indeed many have considered Roth spots pathognomonic for this condition. More recent histological evidence suggests, however, that they are not foci of bacterial abscess. Instead, they are nonspecific and may be found in many other diseases. A review of the histology and the pathogenesis of these white-centred haemorrhages will be provided, along with the work-up of the differential diagnosis.

  11. DNA-repair synthesis in ataxia telangiectasia lymphoblastoid cells

    Energy Technology Data Exchange (ETDEWEB)

    Ford, M.D.; Houldsworth, J.; Lavin, M.F. (Queensland Univ., Brisbane (Australia). Dept. of Biochemistry)

    1981-12-01

    The ability of a number of Epstein-Barr virus-transformed lymphoblastoid cells from ataxia telangiectasia (AT) patients to repair ..gamma..-radiation damage to DNA was determined. All of these AT cells were previously shown to be hypersensitive to ..gamma..-radiation. Two methods were used to determine DNA-repair synthesis: isopycnic gradient analysis and a method employing hydroxyurea to inhibit semiconservative DNA synthesis. Control, AT heterozygote and AT homozygote cells were demonstrated to have similar capacities for repair of radiation damage to DNA. In addition at high radiation doses (10-40 krad) the extent of inhibition of DNA synthesis was similar in the different cell types.

  12. Antithrombotic drugs and subarachnoid haemorrhage risk

    DEFF Research Database (Denmark)

    Pottegård, A; García Rodríguez, L A; Poulsen, F R

    2015-01-01

    The study objective was to investigate the relationship between use of antithrombotic drugs and subarachnoid haemorrhage (SAH). We identified patients discharged from Danish neurosurgery units with a first-ever SAH diagnosis in 2000 to 2012 (n=5,834). For each case, we selected 40 age-, sex...

  13. Genetics Home Reference: hereditary hemorrhagic telangiectasia

    Science.gov (United States)

    ... Central OMIM: JUVENILE POLYPOSIS/HEREDITARY HEMORRHAGIC TELANGIECTASIA SYNDROME McDonald J, Bayrak-Toydemir P, Pyeritz RE. Hereditary hemorrhagic ... 10.1097/GIM.0b013e3182136d32. Review. Citation on PubMed McDonald J, Wooderchak-Donahue W, VanSant Webb C, Whitehead ...

  14. Hereditary hemorrhagic telangiectasia clinical and molecular genetics

    NARCIS (Netherlands)

    Letteboer, T.G.W.

    2010-01-01

    Hereditary hemorrhagic telangiectasia (HHT) or Rendu-Osler-Weber (ROW) syndrome is an autosomal dominant disease characterized by vascular malformations in multiple organ systems. HHT has an age-related penetrance and variable clinical expression. The clinical symptoms are caused by direct

  15. Ebola haemorrhagic fever

    Science.gov (United States)

    Feldmann, Heinz; Geisbert, Thomas W

    2012-01-01

    Ebola viruses are the causative agents of a severe form of viral haemorrhagic fever in man, designated Ebola haemorrhagic fever, and are endemic in regions of central Africa. The exception is the species Reston Ebola virus, which has not been associated with human disease and is found in the Philippines. Ebola virus constitutes an important local public health threat in Africa, with a worldwide effect through imported infections and through the fear of misuse for biological terrorism. Ebola virus is thought to also have a detrimental effect on the great ape population in Africa. Case-fatality rates of the African species in man are as high as 90%, with no prophylaxis or treatment available. Ebola virus infections are characterised by immune suppression and a systemic inflammatory response that causes impairment of the vascular, coagulation, and immune systems, leading to multiorgan failure and shock, and thus, in some ways, resembling septic shock. PMID:21084112

  16. Pulmonary function in adolescents with ataxia telangiectasia.

    Science.gov (United States)

    McGrath-Morrow, Sharon; Lefton-Greif, Maureen; Rosquist, Karen; Crawford, Thomas; Kelly, Amber; Zeitlin, Pamela; Carson, Kathryn A; Lederman, Howard M

    2008-01-01

    Pulmonary complications are common in adolescents with ataxia telangiectasia (A-T), however objective measurements of lung function may be difficult to obtain because of underlying bulbar weakness, tremors, and difficulty coordinating voluntary respiratory maneuvers. To increase the reliability of pulmonary testing, minor adjustments were made to stabilize the head and to minimize leaks in the system. Fifteen A-T adolescents completed lung volume measurements by helium dilution. To assess for reproducibility of spirometry testing, 10 A-T adolescents performed spirometry on three separate occasions. Total lung capacity (TLC) was normal or just mildly decreased in 12/15 adolescents tested. TLC correlated positively with functional residual capacity (FRC), a measurement independent of patient effort (R2=0.71). The majority of individuals had residual volumes (RV) greater than 120% predicted (10/15) and slow vital capacities (VC) less than 70% predicted (9/15). By spirometry, force vital capacity (FVC) and forced expiratory volume in 1 sec (FEV1) values were reproducible in the 10 individuals who underwent testing on three separate occasions (R=0.97 and 0.96 respectively). Seven of the 10 adolescents had FEV1/FVC ratios>90%. Lung volume measurements from A-T adolescents revealed near normal TLC values with increased RV and decreased VC values. These findings indicate a decreased ability to expire to residual volume rather then a restrictive defect. Spirometry was also found to be reproducible in A-T adolescents suggesting that spirometry testing may be useful for tracking changes in pulmonary function over time in this population. Copyright (c) 2007 Wiley-Liss, Inc.

  17. A comparison of patient dose levels between 3/4 vessel conventional angiography and computed tomography angiography during examinations to investigate subarachnoid haemorrhage

    International Nuclear Information System (INIS)

    Spanton, David; Strudwick, Ruth M.

    2007-01-01

    The aim of this study was to investigate and compare the levels of ionising radiation dose received by patients whilst undergoing radiological examination for Subarachnoid haemorrhage by conventional angiography (single and bi plane) and computed tomography angiography. The results obtained from previous examinations have been compared to consider which method of investigation delivers the lowest ionising radiation dose to the patient. Consideration was also given to comparing single plane angiography to bi plane angiography as empirical evidence suggested that radiologists received no formal training and only a small amount of informal training on newly installed equipment at the hospital in which the research was carried out. Would this lead to patients being inadvertently exposed to increased radiation as radiologists familiarised themselves with the equipment? The dose received by 30 patients examined for SAH by each modality was converted to effective dose (mSv) for comparison. These results were then further compared by removing the lowest and highest recorded doses to eliminate any bias that may have been caused by skewed data. The results showed that CTA consistently delivered a lower dose to patients than single or bi plane angiography and that bi plane delivered a lower mean average dose than single plane angiography, with or without any skewed data

  18. Streptococcus sanguinis meningitis following endoscopic ligation for oesophageal variceal haemorrhage.

    Science.gov (United States)

    Liu, Yu-Ting; Lin, Chin-Fu; Lee, Ya-Ling

    2013-05-01

    We report a case of acute purulent meningitis caused by Streptococcus sanguinis after endoscopic ligation for oesophageal variceal haemorrhage in a cirrhotic patient without preceding symptoms of meningitis. Initial treatment with flomoxef failed. The patient was cured after 20 days of intravenous penicillin G. This uncommon infection due to S. sanguinis adds to the long list of infectious complications among patients with oesophageal variceal haemorrhage.

  19. Dual-energy CT for detection of contrast enhancement or leakage within high-density haematomas in patients with intracranial haemorrhage

    Energy Technology Data Exchange (ETDEWEB)

    Watanabe, Yoshiyuki; Tsukabe, Akio; Kunitomi, Yuki; Nishizawa, Mitsuo; Arisawa, Atsuko; Tanaka, Hisashi; Tomiyama, Noriyuki [Osaka University Graduate School of Medicine, Department of Diagnostic and Interventional Radiology, Suita, Osaka (Japan); Yoshiya, Kazuhisa; Shimazu, Takeshi [Osaka University Graduate School of Medicine, Department of Traumatology and Acute Critical Medicine, Osaka (Japan)

    2014-04-15

    Our study aimed to elucidate the diagnostic performance of dual-energy CT (DECT) in the detection of contrast enhancement in intracranial haematomas (ICrH) with early phase dual-energy computed tomography angiography (CTA) and compare the results with those obtained by delayed CT enhancement. Thirty-six patients with ICrH were retrospectively included in this study. All patients had undergone single-energy non-contrast CT and contrast-enhanced dual-source DECT. DECT images were post-processed with commercial software, followed by obtaining iodine images and virtual non-contrast images and generating combined images that created the impression of 120-kVp images. Two neuroradiologists, blinded to the patients' data, reviewed two reading sessions: session A (non-contrast CT and combined CT) and session B (non-contrast CT, combined CT, and iodine images) for detection of contrast enhancement in the haematomas. Contrast leakage or enhancement was detected in 23 (57.5 %) out of 40 haemorrhagic lesions in 36 patients on delayed CT. Three enhanced lesions were depicted only in the DECT iodine images. The sensitivity, specificity, positive predictive value, and negative predictive value of session A were 82.6, 94.1, 95.0, and 80.0 %, respectively, and those of session B were 95.7, 94.1, 95.7, and 94.1 %, respectively. DECT emphasised the iodine enhancement and facilitated the detection of contrast enhancement or leakage. (orig.)

  20. Dual-energy CT for detection of contrast enhancement or leakage within high-density haematomas in patients with intracranial haemorrhage.

    Science.gov (United States)

    Watanabe, Yoshiyuki; Tsukabe, Akio; Kunitomi, Yuki; Nishizawa, Mitsuo; Arisawa, Atsuko; Tanaka, Hisashi; Yoshiya, Kazuhisa; Shimazu, Takeshi; Tomiyama, Noriyuki

    2014-04-01

    Our study aimed to elucidate the diagnostic performance of dual-energy CT (DECT) in the detection of contrast enhancement in intracranial haematomas (ICrH) with early phase dual-energy computed tomography angiography (CTA) and compare the results with those obtained by delayed CT enhancement. Thirty-six patients with ICrH were retrospectively included in this study. All patients had undergone single-energy non-contrast CT and contrast-enhanced dual-source DECT. DECT images were post-processed with commercial software, followed by obtaining iodine images and virtual non-contrast images and generating combined images that created the impression of 120-kVp images. Two neuroradiologists, blinded to the patients' data, reviewed two reading sessions: session A (non-contrast CT and combined CT) and session B (non-contrast CT, combined CT, and iodine images) for detection of contrast enhancement in the haematomas. Contrast leakage or enhancement was detected in 23 (57.5 %) out of 40 haemorrhagic lesions in 36 patients on delayed CT. Three enhanced lesions were depicted only in the DECT iodine images. The sensitivity, specificity, positive predictive value, and negative predictive value of session A were 82.6, 94.1, 95.0, and 80.0 %, respectively, and those of session B were 95.7, 94.1, 95.7, and 94.1 %, respectively. DECT emphasised the iodine enhancement and facilitated the detection of contrast enhancement or leakage.

  1. Dual-energy CT for detection of contrast enhancement or leakage within high-density haematomas in patients with intracranial haemorrhage

    International Nuclear Information System (INIS)

    Watanabe, Yoshiyuki; Tsukabe, Akio; Kunitomi, Yuki; Nishizawa, Mitsuo; Arisawa, Atsuko; Tanaka, Hisashi; Tomiyama, Noriyuki; Yoshiya, Kazuhisa; Shimazu, Takeshi

    2014-01-01

    Our study aimed to elucidate the diagnostic performance of dual-energy CT (DECT) in the detection of contrast enhancement in intracranial haematomas (ICrH) with early phase dual-energy computed tomography angiography (CTA) and compare the results with those obtained by delayed CT enhancement. Thirty-six patients with ICrH were retrospectively included in this study. All patients had undergone single-energy non-contrast CT and contrast-enhanced dual-source DECT. DECT images were post-processed with commercial software, followed by obtaining iodine images and virtual non-contrast images and generating combined images that created the impression of 120-kVp images. Two neuroradiologists, blinded to the patients' data, reviewed two reading sessions: session A (non-contrast CT and combined CT) and session B (non-contrast CT, combined CT, and iodine images) for detection of contrast enhancement in the haematomas. Contrast leakage or enhancement was detected in 23 (57.5 %) out of 40 haemorrhagic lesions in 36 patients on delayed CT. Three enhanced lesions were depicted only in the DECT iodine images. The sensitivity, specificity, positive predictive value, and negative predictive value of session A were 82.6, 94.1, 95.0, and 80.0 %, respectively, and those of session B were 95.7, 94.1, 95.7, and 94.1 %, respectively. DECT emphasised the iodine enhancement and facilitated the detection of contrast enhancement or leakage. (orig.)

  2. Clinical and genetic features of ataxia-telangiectasia

    International Nuclear Information System (INIS)

    Bundey, S.

    1994-01-01

    There are several variants of ataxia-telangiectasia (A-T): classical A-T with marked radiation sensitivity; classical A-T with intermediate levels of radiation sensitivity; mild A-T with intermediate levels of radiation sensitivity; A-T without telangiectasia; A-T without oculomoto apraxia; and A-T with microcephaly. These disorders are probably caused by different allelic mutations, because affected sibs resemble the index patients, and because there is an association of certain haplo-types of 11q22-23 with specific phenotypes. The Nijmegen Breakage Syndrome, with its lack of ataxia, seems on clinical grounds to be a different disorder. Although A-T is almost always inherited as an autosomal recessive, there are some unusual features; an unexpectedly low parental consanguinity rate, an incidence in sibs that is < 0.25, and occurrence of disease in many different races and in the offspring of mixed race unions. Moreover, looking at haplotypes from 63 UK patients, there is a remarkably low incidence of homozygosity. An autosomal recessive condition that is deficient in parental consanguinity, and in homozygosity for the region around the gene, can be explained by J.H. Edwards' hypothesis that homozygosity for alleles at a neighbouring locus are lethal early in embryogenesis. Other possible mechanisms to explain the unusual genetic features are discussed. (author)

  3. Emergency admissions for major haemorrhage associated with direct oral anticoagulants.

    Science.gov (United States)

    Bouget, Jacques; Oger, Emmanuel

    2015-12-01

    To describe the population admitted in an emergency department of a teaching hospital for severe bleeding associated with direct oral anticoagulants (DOAC). During a three-year period (2012-2014) patients older than 16 years were prospectively identified by haemorrhagic symptoms from computerised requests. At least one of the following criteria defined major haemorrhage: haemorrhagic shock, unstable haemodynamic, need for transfusion or haemostatic procedure, or a life threatening location. Fifty four patients, 23 receiving dabigatran, 30 rivaroxaban and one apixaban were included, 2 in 2012, 35 in 2013 and 17 in 2014. Median age was 84 years (range 63-99) with a sex ratio of 1.16. Haemorrhagic complications were gastrointestinal (n=27), intracranial (n=12) or miscellaneous (n=15). Indication of DOAC was stroke prevention in atrial fibrillation in 49 cases and deep vein thrombosis in 5 cases. Hospitalization was required for 45 patients (83%) with a mean length of stay of 8.5 days. Sixteen patients needed intensive care. Reversal therapy was prescribed in 11 patients. At 1 month, overall mortality was 24%, reaching 41.7% for intracranial haemorrhage. Among surviving patients, DOAC was stopped in 10 cases, continued in 17 patients and switched for other antithrombotic in 17 patients. Our study contributes to the post marketing surveillance of major haemorrhagic complications associated with DOAC. It takes part to the knowledge about the course of this severe event in emergencies. Careful awareness in risk benefit assessment, especially in elderly, is needed. Copyright © 2015 Elsevier Ltd. All rights reserved.

  4. Germline mutations in BMP9 are not identified in a series of Danish and French patients with hereditary hemorrhagic telangiectasia

    DEFF Research Database (Denmark)

    Tørring, P. M.; Dupuis-Girod, S.; Giraud, S

    2016-01-01

    undiscovered HHT causative genes. A new vascular-anomaly syndrome caused by mutations in BMP9 has recently been published. Three patients suspected of HHT, with familial nose bleedings and dermal manifestations not characteristic for HHT, were described. Although, it was concluded that these patients probably...

  5. Postpartum haemorrhage: prevention

    OpenAIRE

    Chelmow, David

    2008-01-01

    Loss of more than 500 mL of blood is usually caused by failure of the uterus to contract fully after delivery of the placenta, and occurs in over 10% of deliveries with a 1% mortality rate worldwide. Other causes of postpartum haemorrhage include retained placental tissue, lacerations to the genital tract and coagulation disorders.Uterine atony is more likely in women who have had a general anaesthetic or oxytocin, an over-distended uterus, a prolonged or precipitous labour, or who are of ...

  6. Postpartum haemorrhage: prevention

    OpenAIRE

    Chelmow, David

    2011-01-01

    Loss of more than 500 mL of blood is usually caused by failure of the uterus to contract fully after delivery of the placenta, and occurs in over 10% of deliveries, with a 1% mortality worldwide. Other causes of postpartum haemorrhage include retained placental tissue, lacerations to the genital tract, and coagulation disorders.Uterine atony is more likely in women who have had a general anaesthetic or oxytocin, an over-distended uterus, a prolonged or precipitous labour, or who are of hig...

  7. ATM (ataxia-telangiectasia mutated) abnormality and diseases

    International Nuclear Information System (INIS)

    Takagi, Masatoshi; Nakata, Shinichiro; Mizutani, Shuki

    2007-01-01

    Ataxia-Telangiectasia (A-T) is an autosomal recessive inherited disease due to mutation of ATM gene on chromosome 11q22.3, with major symptoms of ataxia, telangiectasia, immunodeficiency and frequent complication of cancer, and the cells have characters of chromosomal break, high sensitivity to radiation and inappropriate continuation of DNA synthesis after radiation. This review describes past and present studies of ATM functions with clinical features in the following order: Clinical symptoms and epidemiology; ATM gene mutation in A-T patients, mainly by frame-shift (80-90%); ATM, whose gene consisted from 66 exons (150 kb), functions in phosphoinositide-3-kinase related kinase family which protecting cells from stress and integrating their system, at response to DNA double strand break, and in the cell cycle checkpoints at G1/S, S and G2/M phases; ATM nonsense/missense mutations in embryonic cells leading to carcinogenesis and role of ATM in the suppression of carcinogenesis in somatic cells; Chromosomal translocation which relating to carcinogenesis, by functional defect of ATM; and Other functions of ATM in neuronal growth, immunodeficiency, carbohydrate and lipid metabolism, early senescence, and virus infection. ATM is thus an essential molecule to maintain growth and homeostasis. (T.I.)

  8. Prevention of haematoma progression by tranexamic acid in intracerebral haemorrhage patients with and without spot sign on admission scan: a statistical analysis plan of a pre-specified sub-study of the TICH-2 trial.

    Science.gov (United States)

    Ovesen, Christian; Jakobsen, Janus Christian; Gluud, Christian; Steiner, Thorsten; Law, Zhe; Flaherty, Katie; Dineen, Rob A; Bath, Philip M; Sprigg, Nikola; Christensen, Hanne

    2018-06-13

    We present the statistical analysis plan of a prespecified Tranexamic Acid for Hyperacute Primary Intracerebral Haemorrhage (TICH)-2 sub-study aiming to investigate, if tranexamic acid has a different effect in intracerebral haemorrhage patients with the spot sign on admission compared to spot sign negative patients. The TICH-2 trial recruited above 2000 participants with intracerebral haemorrhage arriving in hospital within 8 h after symptom onset. They were included irrespective of radiological signs of on-going haematoma expansion. Participants were randomised to tranexamic acid versus matching placebo. In this subgroup analysis, we will include all participants in TICH-2 with a computed tomography angiography on admission allowing adjudication of the participants' spot sign status. Primary outcome will be the ability of tranexamic acid to limit absolute haematoma volume on computed tomography at 24 h (± 12 h) after randomisation among spot sign positive and spot sign negative participants, respectively. Within all outcome measures, the effect of tranexamic acid in spot sign positive/negative participants will be compared using tests of interaction. This sub-study will investigate the important clinical hypothesis that spot sign positive patients might benefit more from administration of tranexamic acid compared to spot sign negative patients. Trial registration ISRCTN93732214 ( http://www.isrctn.com ).

  9. Haemorrhage in intracranial tuber- culosis

    African Journals Online (AJOL)

    CASE REPORT. 16. SA JOURNAL OF RADIOLOGY • July 2005. Haemorrhage in intracranial tuber- culosis. M Modi. FCRad (SA), MMed. Department of Radiation Sciences ... wall where granulomatous inflamma- tion (Fig. 2, arrow) was present in the adventitia. A specific site of origin for the subarachnoid haemorrhage was.

  10. 20-year follow-up study of Danish HHT patients-survival and causes of death

    DEFF Research Database (Denmark)

    Kjeldsen, Anette; Aagaard, Katrine Saldern; Tørring, Pernille Mathiesen

    2016-01-01

    in the TGF-β pathway which is responsible for angiogenesis. Modulations of angiogenesis may influence cancer rates. The objective of the study was to evaluate 20-year survival according to HHT subtype, as well as to evaluate differences in causes of death comparing HHT patients and controls. We also wanted......BACKGROUND: Hereditary Haemorrhagic Telangiectasia (HHT) is a dominantly inheritable disorder, with a wide variety of clinical manifestations due to presence of multiple arteriovenous manifestations. The most common mutations are found in HHT1 (ENG) and HHT2 (ACVRL1) patients, causing alterations...

  11. Intracerebral haemorrhage risk in microbleed-positive ischaemic stroke patients with atrial fibrillation: Preliminary meta-analysis of cohorts and anticoagulation decision schema.

    Science.gov (United States)

    Charidimou, Andreas; Boulouis, Gregoire; Shams, Sara; Calvet, David; Shoamanesh, Ashkan

    2017-07-15

    Whether ischaemic stroke patients with atrial fibrillation (AF) and cerebral microbleeds (CMBs) on MRI can be safely anticoagulated is a hotly debated topic. We performed a systematic review and meta-analysis of published aggregate data, to investigate the risk of subsequent intracerebral haemorrhage (ICH) based on CMBs presence in this stroke population, generally considered for oral anticoagulation. We also suggest a decision-making schema for anticoagulation use in this setting. We searched PubMed for relevant observational studies. Random effects models with DerSimonian-Laird weights were used to investigated the association between CMBs presence at baseline MRI and ICH or ischaemic stroke during follow-up. Four studies, with slightly heterogeneous design, including 990 ischaemic stroke patients were pooled in a meta-analysis (crude CMBs prevalence: 25%; 95%CI: 17%-33%). The median follow-up ranged between 17 and 37months. The future symptomatic ICH rate was 1.6% (16/990), while recurrent ischaemic stroke rate was 5.9% (58/990). Baseline CMB presence was associated with increased risk of symptomatic ICH during follow-up compared to patients without CMBs (OR: 4.16; 95%CI: 1.54-11.25; p=0.005). There was no association between CMBs presence and recurrent ischaemic stroke risk. We have shown that the presence of CMBs in cohorts of ischaemic stroke patients, most with AF on warfarin, is associated with a 4-fold increase in subsequent ICH (but not ischaemic stroke) risk (Class III evidence). These pooled estimates are useful for future trials design. We propose a simple data-driven anticoagulation schema which awaits validation and refinement as new prospective data are accumulated. Copyright © 2017 Elsevier B.V. All rights reserved.

  12. Long non-coding RNA expression profiles in hereditary haemorrhagic telangiectasia

    DEFF Research Database (Denmark)

    Tørring, Pernille M; Larsen, Martin Jakob; Kjeldsen, Anette D

    2014-01-01

    transcriptome, we wanted to assess whether lncRNAs play a role in the molecular pathogenesis of HHT manifestations. By microarray technology, we profiled lncRNA transcripts from HHT nasal telangiectasial and non-telangiectasial tissue using a paired design. The microarray probes were annotated using the GENCODE...... v.16 dataset, identifying 4,810 probes mapping to 2,811 lncRNAs. Comparing HHT telangiectasial tissue with HHT non-telangiectasial tissue, we identified 42 lncRNAs that are differentially expressed (qUsing GREAT, a tool that assumes cis-regulation, we showed that differently expressed lncRNAs...... to the TGF-β signalling pathway. The exact mechanism of how haploinsufficiency of ENG and ACVRL1 leads to HHT manifestations remains to be identified. As long non-coding RNAs (lncRNAs) are increasingly recognized as key regulators of gene expression and constitute a sizable fraction of the human...

  13. An Unusual Case of Bilateral Vitreous Haemorrhage following Snake Bite

    Directory of Open Access Journals (Sweden)

    Vipul Bhandari

    2013-01-01

    Full Text Available A-45-year-old man presented to us with diminution of vision in both eye following snake bite. On examination vision in the right eye (RE was 6/36 and vision in left eye (LE was hand movement positive and fundus examination revealed a subhyloid haemorrhage, left eye showed vitreous haemorrhage. Patient was advised bed rest, vitamin C tablets and oral steroids.

  14. [Use of "the Middlesex Elderly Assessment of Mental State" scale in the assessment of cognitive functioning in patients with aneurysmal subarachnoid haemorrhage].

    Science.gov (United States)

    Chyza, Karolina Julia; Polityńska, Barbara; Kochanowicz, Jan; Lewko, Janusz

    2007-03-01

    It is now well established that cognitive deficits are a frequent consequence of aneurysmal subarachnoid haemorrhage (SAH). The cognitive status in the acute phase of the illness may provide valuable prognostic information in relation to the effects of the proposed treatment and long-term functioning of the patient. A prerequisite for this task is the identification of instruments that might prove useful in the diagnosis of neuropsychological deficits in patients with SAH. For these purposes we used The Middlesex Elderly Assessment of Mental State (MEAMS) in order to assess the cognitive deficits consequent upon SAH. To assess the cognitive functioning of patients undergoing treatment for SAH of aneurysmal origin in the acute stage of the illness, using a modified form of the MEAMS. 49 patients participated in the study, none of whom had a previous history of neurological or psychiatric illness. The age of the patients ranged between 23-70 years. 35 (71%) patients received surgical treatment (clipping of the aneurysm neck) and in 14 (29%) the aneurysm was embolised. The patients were assessed on two occasions: the first on admission to the Neurosurgery department following the SAH, and on the second, following treatment to secure the aneurysm. A modified version of the MEAMS in two parallel versions was used in the assessment. The results obtained were evaluated with reference to a control group. A range of cognitive impairments was identified with the aid of the MEAMS in patients undergoing treatment for aneurysmal SAH. These included deficits in visual and auditory memory, executive, perceptual and visuo-spatial functions together with the tendency to perseverate. In those patients who underwent surgery, deficits were observed in the following areas: disorientation in relation to self, time and place; perceptual, memory and visuo-spatial impairments. The results obtained indicate that the Middlesex Elderly Assessment of Mental State, in the form used in the

  15. Hereditary hemorrhagic telangiectasia: genetics and molecular diagnostics in a new era

    Directory of Open Access Journals (Sweden)

    Jamie eMcDonald

    2015-01-01

    Full Text Available Hereditary hemorrhagic telangiectasia (HHT is a vascular dysplasia characterized by telangiectases and arteriovenous malformations (AVMs in particular locations described in consensus clinical diagnostic criteria published in 2000. Two genes in the transforming growth factor-beta (TGF-β signaling pathway, ENG and ACVRL1, were discovered almost two decades ago, and mutations in these genes have been reported to cause up to 85% of HHT. In our experience, approximately 96% of individuals with HHT have a mutation in these two genes, when published (Curaçao diagnostic criteria for HHT are strictly applied. More recently, two additional genes in the same pathway, SMAD4 and GDF2, have been identified in a much smaller number of patients with a similar or overlapping phenotype to HHT. Yet families still exist with compelling evidence of a hereditary telangiectasia disorder, but no identifiable mutation in a known gene. Recent availability of whole exome and genome testing has created new opportunities to facilitate gene discovery, identify genetic modifiers to explain clinical variability, and potentially define an increased spectrum of hereditary telangiectasia disorders. An expanded approach to molecular diagnostics for inherited telangiectasia disorders that incorporates a multi-gene next generation sequencing (NGS HHT panel is proposed.

  16. Postpartum haemorrhage: a preventable cause of maternal mortality

    International Nuclear Information System (INIS)

    Shaheen, B.; Hassan, L.

    2007-01-01

    To assess the preventable predictors of severe postpartum haemorrhage and the adverse outcome associated with it. All the admitted patients who developed severe postpartum haemorrhage (>1500 ml) were included in the study. Clinical and sociodemographic data was obtained along with results of investigations to categorize the complications encountered. Odds ratio (OR) and 95% confidence intervals were determined. During the study period, 75 out of 4683 obstetrical admissions, developed severe postpartum haemorrhage (1.6 %). About 65% of the patients were admitted with some other complications including obstructed labour, antepartum haemorrhage and eclampsia. The risk factors were grand multiparity (OR=3.4), pre-eclampsia (OR=2.75), antepartum haemorrhage (OR=13.35), active labour of more than 10 hours (OR=46.92), twin delivery (OR=3.25), instrumental delivery (OR=8.62) and caesarean section (OR=9.74). Maternal mortality in these cases was 2.66% and residual morbidity being 40%. Birth attendant other than doctor and delivery outside the study unit were significantly associated with the adverse outcome in these patients. Maternal outcome associated with postpartum haemorrhage is a function of care given during labour and postnatal period with early diagnosis and management of the complication and its risk factors, being the key of good maternal outcome. (author)

  17. DNA synthesis in ataxia telangiectasia

    NARCIS (Netherlands)

    N.G.J. Jaspers (Nicolaas)

    1985-01-01

    textabstractAfter the discovery that cultured cells from AT patients are hypersensitive to ionizing radiation the suggestion was made that AT-could be the 1 X-ray-analogue 1 of xeroderma pigmentosum. The latter syndrome (XP) is characterized by hypersensitivity to short-wave UV-radiation, caused by

  18. Postpartum haemorrhage: prevention.

    Science.gov (United States)

    Chelmow, David

    2011-04-04

    Loss of more than 500 mL of blood following childbirth is usually caused by failure of the uterus to contract fully after delivery of the placenta, and occurs in over 10% of deliveries, with a 1% mortality rate worldwide. Other causes of postpartum haemorrhage include retained placental tissue, lacerations to the genital tract, and coagulation disorders. Uterine atony is more likely in women who have had a general anaesthetic or oxytocin, an over-distended uterus, a prolonged or precipitous labour, or who are of high parity. We conducted a systematic review and aimed to answer the following clinical questions: What are the effects of non-drug interventions and of drug interventions to prevent primary postpartum haemorrhage? We searched: Medline, Embase, The Cochrane Library, and other important databases up to March 2010 (Clinical Evidence reviews are updated periodically; please check our website for the most up-to-date version of this review). We included harms alerts from relevant organisations such as the US Food and Drug Administration (FDA) and the UK Medicines and Healthcare products Regulatory Agency (MHRA). We found 40 systematic reviews, RCTs, or observational studies that met our inclusion criteria. We performed a GRADE evaluation of the quality of evidence for interventions. In this systematic review we present information relating to the effectiveness and safety of the following interventions: active management of the third stage of labour, carboprost injection, controlled cord traction, ergot compounds (ergometrine/methylergotamine), immediate breastfeeding, misoprostol (oral, rectal, sublingual, or vaginal), oxytocin, oxytocin plus ergometrine combinations, prostaglandin E2 compounds, and uterine massage.

  19. Traumatic primary brain stem haemorrhage

    International Nuclear Information System (INIS)

    Andrioli, G.C.; Zuccarello, M.; Trincia, G.; Fiore, D.L.; De Caro, R.

    1983-01-01

    We report 36 cases of post-traumatic 'primary brain stem haemorrhage' visualized by the CT scan and confirmed at autopsy. Clinical experience shows that many technical factors influence the inability to visualize brain stem haemorrhages. Experimental injection of fresh blood into the pons and midbrain of cadavers shows that lesions as small as 0.25 ml in volume may be visualized. The volume and the anatomical configuration of traumatic lesions of the brain stem extended over a rostro-caudal direction, and their proximity to bony structures at the base of the skull are obstacles to the visualization of brain stem haemorrhages. (Author)

  20. DNA synthesis in ataxia telangiectasia

    OpenAIRE

    Jaspers, Nicolaas

    1985-01-01

    textabstractAfter the discovery that cultured cells from AT patients are hypersensitive to ionizing radiation the suggestion was made that AT-could be the 1 X-ray-analogue 1 of xeroderma pigmentosum. The latter syndrome (XP) is characterized by hypersensitivity to short-wave UV-radiation, caused by a reduced ability to properly remove UV-induced DNA damage. The evidence for a DNA repair defect in AT cells is not as strong as in the case of XP (see section 2.2.5 of this thesis). Different XP p...

  1. Systematic Evaluation of Desmopressin Applied to the Patients with Aneurysmal Subarachnoid Haemorrhage in the light of the Literature.

    Science.gov (United States)

    Karaarslan, Numan; Yilmaz, Ibrahim; Akgun, Feride Sinem; Caliskan, Tezcan; Ozbek, Hanefi; Ates, Ozkan

    2018-04-09

    This study discusses the management of patients with the syndrome of inappropriate antidiuretic hormone secretion (SIADH), developed after subarachnoid hemorrhage, in a comparative manner in light of the literature. Without country or language restrictions, articles with high evidential value found in electronic databases were compared to our patients' data. After the literature review, three articles were included for systematic evaluation. Desmopressin was applied to the patients for the treatment of hyponatremia, volume contraction, and negative sodium balance caused by SIADH. However, it was not used for preventing re-bleeding. To prevent the development of this complication (SIADH), the use of desmopressin, an analogue of vasopressin, is important in routine clinical practice.

  2. ERS statement on the multidisciplinary respiratory management of ataxia telangiectasia

    Directory of Open Access Journals (Sweden)

    Jayesh M. Bhatt

    2015-12-01

    Full Text Available Ataxia telangiectasia (A-T is a rare, progressive, multisystem disease that has a large number of complex and diverse manifestations which vary with age. Patients with A-T die prematurely with the leading causes of death being respiratory diseases and cancer. Respiratory manifestations include immune dysfunction leading to recurrent upper and lower respiratory infections; aspiration resulting from dysfunctional swallowing due to neurodegenerative deficits; inefficient cough; and interstitial lung disease/pulmonary fibrosis. Malnutrition is a significant comorbidity. The increased radiosensitivity and increased risk of cancer should be borne in mind when requesting radiological investigations. Aggressive proactive monitoring and treatment of these various aspects of lung disease under multidisciplinary expertise in the experience of national multidisciplinary clinics internationally forms the basis of this statement on the management of lung disease in A-T. Neurological management is outwith the scope of this document.

  3. Intracerebral haemorrhage after carotid endarterectomy

    DEFF Research Database (Denmark)

    Schroeder, T; Sillesen, H; Boesen, J

    1987-01-01

    Among 662 consecutive carotid endarterectomies eight cases of postoperative ipsilateral intracerebral haemorrhage were identified, occurring into brain areas which, preoperatively were without infarction. As blood pressures across the stenosis were routinely measured during surgery, the internal...

  4. Effect of magnesium treatment and glucose levels on delayed cerebral ischemia in patients with subarachnoid hemorrhage: a substudy of the Magnesium in Aneurysmal Subarachnoid Haemorrhage trial (MASH-II).

    Science.gov (United States)

    Leijenaar, Jolien F; Dorhout Mees, Sanne M; Algra, Ale; van den Bergh, Walter M; Rinkel, Gabriel J E

    2015-10-01

    Magnesium treatment did not improve outcome in patients with aneurysmal subarachnoid haemorrhage in the Magnesium in Aneurysmal Subarachnoid Haemorrhage II trial. We hypothesized that high glucose levels may have offset a potential beneficial effect to prevent delayed cerebral ischemia. We investigated if magnesium treatment led to less delayed cerebral ischemia and if glucose levels interacted with magnesium treatment in the Magnesium in Aneurysmal Subarachnoid Haemorrhage II trial. To investigate the effect of magnesium treatment on occurrence of delayed cerebral ischemia and the interaction between glucose levels and magnesium treatment in subarachnoid hemorrhage patients. The Magnesium in Aneurysmal Subarachnoid Haemorrhage was a phase III randomized placebo-controlled trial assessing the effect of magnesium sulphate on clinical outcome in aneurysmal subarachnoid hemorrhage patients. For the current study, we included only the patients admitted to the University Medical Centre-Utrecht. We calculated hazard ratios for occurrence of delayed cerebral ischemia in patients treated with magnesium vs. placebo for the entire study population, and separately in the subgroups of patients with high and low mean fasting and mean daily glucose levels until onset of delayed cerebral ischemia. We used the cross-product of magnesium and glucose in the regression analysis to evaluate whether an interaction between magnesium and glucose existed. We included 616 patients: 307 received magnesium and 309 placebo; 156 patients had delayed cerebral ischemia. Hazard ratio for magnesium on occurrence of delayed cerebral ischemia was 1·0 (95% confidence interval: 0·7-1·4). Results were similar in patients with low or high fasting or daily glucose levels. We found no interactions between magnesium treatment and high fasting (P = 0·54) and daily glucose (P = 0·60). Magnesium treatment did not reduce the risk of delayed cerebral ischemia in patients with aneurysmal

  5. Clinical performance and patient outcome after simulation-based training in prevention and management of postpartum haemorrhage : An educational intervention study in a low-resource setting

    NARCIS (Netherlands)

    Nelissen, Ellen; Ersdal, Hege; Mduma, Estomih; Evjen-Olsen, Bjorg; Twisk, Jos; Broerse, Jacqueline; van Roosmalen, Jos; Stekelenburg, Jelle

    2017-01-01

    Background: Postpartum haemorrhage (PPH) is a major cause of maternal mortality. Prevention and adequate treatment are therefore important. However, most births in low-resource settings are not attended by skilled providers, and knowledge and skills of healthcare workers that are available are low.

  6. Characterizing amide proton transfer imaging in haemorrhage brain lesions using 3T MRI

    Energy Technology Data Exchange (ETDEWEB)

    Jeong, Ha-Kyu [Philips Korea, Seoul (Korea, Republic of); Korea Basic Science Institute, Chungcheongbuk-do (Korea, Republic of); Han, Kyunghwa [Yonsei University College of Medicine, Department of Radiology and Research Institute of Radiological Science, Seodaemun-gu, Seoul (Korea, Republic of); Yonsei University College of Medicine, Yonsei Biomedical Research Institute, Seoul (Korea, Republic of); Zhou, Jinyuan [Johns Hopkins University School of Medicine, Division of MRI Research, Department of Radiology, Baltimore, MD (United States); Zhao, Yansong [Philips Healthcare, MR Clinical Science, Cleveland, OH (United States); Choi, Yoon Seong; Lee, Seung-Koo; Ahn, Sung Soo [Yonsei University College of Medicine, Department of Radiology and Research Institute of Radiological Science, Seodaemun-gu, Seoul (Korea, Republic of)

    2017-04-15

    The aim of this study was to characterize amide proton transfer (APT)-weighted signals in acute and subacute haemorrhage brain lesions of various underlying aetiologies. Twenty-three patients with symptomatic haemorrhage brain lesions including tumorous (n = 16) and non-tumorous lesions (n = 7) were evaluated. APT imaging was performed and analyzed with magnetization transfer ratio asymmetry (MTR{sub asym}). Regions of interest were defined as the enhancing portion (when present), acute or subacute haemorrhage, and normal-appearing white matter based on anatomical MRI. MTR{sub asym} values were compared among groups and components using a linear mixed model. MTR{sub asym} values were 3.68 % in acute haemorrhage, 1.6 % in subacute haemorrhage, 2.65 % in the enhancing portion, and 0.38 % in normal white matter. According to the linear mixed model, the distribution of MTR{sub asym} values among components was not significantly different between tumour and non-tumour groups. MTR{sub asym} in acute haemorrhage was significantly higher than those in the other regions regardless of underlying pathology. Acute haemorrhages showed high MTR{sub asym} regardless of the underlying pathology, whereas subacute haemorrhages showed lower MTR{sub asym} than acute haemorrhages. These results can aid in the interpretation of APT imaging in haemorrhage brain lesions. (orig.)

  7. A randomized, placebo-controlled pilot study of patients with spontaneous intraventricular haemorrhage treated with intraventricular thrombolysis.

    Science.gov (United States)

    King, Nicolas K K; Lai, Jin Li; Tan, Li Bing; Lee, Kah Keow; Pang, Boon Chuan; Ng, Ivan; Wang, Ernest

    2012-07-01

    Intraventricular hemorrhage (IVH) occurring after spontaneous intracerebral hemorrhage (ICH) is an independent risk factor for mortality. The use of intraventricular urokinase (Uk) to reduce intraventricular blood clot volume and improve outcome was investigated. Patients with IVH requiring external ventricular drainage were recruited and randomized into a double-blind placebo controlled study. Assessments of collected cerebrospinal fluid (CSF) haemoglobin (Hb) and serial CT scans were performed. The study outcomes were: infection rates, length of stay in the intensive care unit, survival, National Institutes of Health Stroke Scale score; and modified Rankin Scale scores. Our results showed an increase in both the drained CSF Hb concentration in patients treated with Uk compared to placebo and in the rate of resolution clot volume. No differences were found in the other outcome measures but there was a trend towards lowered mortality in the group treated with Uk. Therefore, intraventricular Uk resulted in faster resolution of IVH with no adverse events. Copyright © 2011 Elsevier Ltd. All rights reserved.

  8. Ataxia-telangiectasia: the pattern of cerebellar atrophy on MRI

    International Nuclear Information System (INIS)

    Tavani, F.; Zimmerman, R.A.; Gatti, R.; Bingham, P.; Berry, G.T.; Sullivan, K.

    2003-01-01

    We describe MRI of the brain in 19 patients with ataxia-telangiectasia (AT) and correlate the appearances with the degree of neurologic deficit. We examined 10 male and nine female patients; 17 were aged between 2 and 12 years (mean 8 years) but a woman and her brother were 35 and 38 years old, and had a variant of AT. Ataxia was the first recognized sign of the disease in every patient. We detected the following patterns of cerebellar atrophy: in the youngest patient, aged 2 years, the study was normal; in the five next youngest patients 3-7 years of age, the lateral cerebellum and superior vermis showed the earliest changes of atrophy; and all but one of the other patients had moderate to marked diffuse atrophy of vermis and cerebellar hemispheres. There were 12 patients aged 9 years and above; one, who was normal, was 9 years old. The five patients who at the time of examination were unable to walk all had diffuse atrophy involving both vermis and cerebellar hemispheres. (orig.)

  9. The value of MRI in angiogram-negative intracranial haemorrhage

    Energy Technology Data Exchange (ETDEWEB)

    Renowden, S.A. (Dept. of Neuroradiology, Radcliffe Infirmary, Oxford (United Kingdom)); Molyneux, A.J. (Dept. of Neuroradiology, Radcliffe Infirmary, Oxford (United Kingdom)); Anslow, P. (Dept. of Neuroradiology, Radcliffe Infirmary, Oxford (United Kingdom)); Byrne, J.V. (Dept. of Neuroradiology, Radcliffe Infirmary, Oxford (United Kingdom))

    1994-08-01

    In one year, cerebral angiograms were performed for intracranial haemorrhage (ICH) on 334 patients. No cause for haemorrhage could be identified in 41 (12 %), 30 of whom had predominantly subarachnoid (SAH) and 11 predominantly parenchymal haemorrhage (PH). These patients were prospectively examined by cranial MRI 1-6 weeks after the ictus. The MRI studies were positive in 7 patients (17 %). In the 30 patients examined after SAH, 2 studies were positive, showing an aneurysm in one case and a brain stem lesion of uncertain aetiology in the other. In those examined after PH, cavernous angiomas were shown in 2, a tumour in 1 and a vascular malformation in another; useful diagnostic information was thus obtained in 36 % of this group. (orig.)

  10. Using the ultra-long pulse width pulsed dye laser and elliptical spot to treat resistant nasal telangiectasia.

    Science.gov (United States)

    Madan, Vishal; Ferguson, Janice

    2010-01-01

    Thick linear telangiectasia on the ala nasi and nasolabial crease can be resistant to treatment with the potassium-titanyl-phosphate (KTP) laser and the traditional round spot on a pulsed dye laser (PDL). We evaluated the efficacy of a 3 mm x 10 mm elliptical spot using the ultra-long pulse width on a Candela Vbeam(R) PDL for treatment of PDL- and KTP laser-resistant nasal telangiectasia. Nasal telangiectasia resistant to PDL (12 patients) and KTP laser (12 patients) in 18 patients were treated with a 3 mm x 10 mm elliptical spot on the ultra-long pulse pulsed dye laser (ULPDL) utilising long pulse width [595 nm, 40 ms, double pulse, 30:20 dynamic cooling device (DCD)]. Six patients had previously received treatment with both PDL and KTP laser prior to ULPDL (40 treatments, range1-4, mean 2.2). Complete clearance was seen in ten patients, and eight patients displayed more than 80% improvement after ULPDL treatment. Self-limiting purpura occurred with round spot PDL and erythema with KTP laser and ULPDL. Subtle linear furrows along the treatment sites were seen in three patients treated with the KTP laser. ULPDL treatment delivered using a 3 mm x 10 mm elliptical spot was non-purpuric and highly effective in the treatment of nasal telangiectasia resistant to KTP laser and PDL.

  11. Changes in the level of cardiac troponine and disorders in pulmonary gas exchange as predictors of short- and long-term outcomes of patients with aneurysm subarachnoid haemorrhage.

    Science.gov (United States)

    Burzyńska, Małgorzata; Uryga, Agnieszka; Kasprowicz, Magdalena; Kędziora, Jarosław; Szewczyk, Ewa; Woźniak, Jowita; Jarmundowicz, Włodzimierz; Kübler, Andrzej

    2017-12-01

    Cardiopulmonary abnormalities are common after aneurysmal subarachnoid haemorrhage (aSAH). However, the relationship between short- and long-term outcome is poorly understood. In this paper, we present how cardiac troponine elevations (cTnI) and pulmonary disorders are associated with short- and long-term outcomes assessed by the Glasgow Outcome Scale (GOS) and Extended Glasgow Outcome Scale (GOSE). A total of 104 patients diagnosed with aSAH were analysed in the study. The non-parametric U Mann-Whitney test was used to evaluate the difference between good (GOS IV-V, GOSE V-VIII) and poor (GOS I-III, GOSE I-IV) outcomes in relation to cTnI elevation and pulmonary disorders. Outcome was assessed at discharge from the hospital, and then followed up 6 and 12 months later. Pulmonary disorders were determined by the PaO 2 /FiO 2 ratio and radiography. The areas under the ROC curves (AUCs) were used to determine the predictive power of these factors. In the group with good short-term outcomes cTnI elevation on the second day after aSAH was significantly lower (p = .00007) than in patients with poor short-term outcomes. The same trend was observed after 6 months, although there were different results 12 months from the onset (p = .024 and n.s., respectively). A higher peak of cTnI was observed in the group with a pathological X-ray (p = .008) and pathological PaO 2 /FiO 2 ratio (p ≪ .001). cTnI was an accurate predictor of short-term outcomes (AUC = 0.741, p ≪ .001) and the outcome after 6 months (AUC = 0.688, p = .015). The results showed that cardiopulmonary abnormalities perform well as predictive factors for short- and long-term outcomes after aSAH.

  12. Late onset retinoblastoma presenting with vitreous haemorrhage

    DEFF Research Database (Denmark)

    Bagger, Mette; Prause, Jan Ulrik; Heegard, Steffen

    2012-01-01

    in the retina. A vascularized gelatinous mass was revealed after vitrectomy. Later the patient developed white cysts in the anterior chamber and histological findings were indicative of a retinoblastoma. The patient was enucleated and the diagnosis of retinoblastoma was confirmed. Intraocular surgery in young...... people with unknown retinoblastoma enhances the risk of metastasis development, orbital recurrence and death. Unexplained vitreous haemorrhage can obscure the view of a tumour but ultrasonic findings of a retinal mass calls for further imaging e.g. through MRI. The case illustrates the importance...

  13. Valved or valveless ventriculoperitoneal shunting in the treatment of post-haemorrhagic hydrocephalus

    DEFF Research Database (Denmark)

    Andreasen, Trine Hjorslev; Holst, Anders Vedel; Lilja, Alexander

    2016-01-01

    BACKGROUND: Implant infection and obstruction are major complications for ventriculoperitoneal shunts in patients with post-haemorrhagic hydrocephalus. In an effort to (1) reduce the incidence of these complications, (2) reduce the rate of shunt failure and (3) shorten the duration of neurosurgical...... hospitalisation, we have implemented valveless ventriculoperitoneal shunts at our department for adult patients with post-haemorrhagic hydrocephalus and haemorrhagic cerebrospinal fluid at the time of shunt insertion. METHODS: All adult patients (>18 years old) treated for post-haemorrhagic hydrocephalus.......3 %, p = 0.02), but a higher rate of overdrainage (10.3 % vs 2.6 %, p = 0.04). CONCLUSION: The use of a valveless shunting for patients with post-haemorrhagic hydrocephalus results in shorter duration of neurosurgical hospitalisation and lower rate of shunt infection, although these advantages should...

  14. Super selective transcatheter angiographic embolization: an effective and prophylactic treatment for massive obstetric haemorrhage

    International Nuclear Information System (INIS)

    Zhou Yiming; Zhai Renyou; Qian Xiaojun; Wei Baojie; Gao Kun; Zhang Shilong; Liu Jinmei; Zhang Qiuhong; Jiang Lei

    2008-01-01

    Objective: To discuss the effect and safety of transcatheter angiographic embolization (TAE)for managing massive obstetric haemorrhage. Methods: 17 cases of obstetric massive haemorrhage or with haemorrhage tendency were treated with TAE. Among them 14 cases had haemorrhage already, including 10 cases after abortion, caesarean section or normal labor and other 4 of hydatidiform mole. 3 cases with obstetric haemorrhage tendency included 2 cases of placenta praevia and 1 case of cervical pregnancy. Selective catheterization into bilateral uterine arteries or internal iliac arteries for DSA, showed the cause and location of the haemorrhage and then embolized with gelfoam sponge chips (1-3 mm) or Polyvinyl Alcohol(PVA); and part of the cases with MTX through uterine arterial perfusion. Results: The successful rate of catheterization was 100%. The achievement in 14 cases showed no active haemorrhage immediately after the procedure and no vaginal bleeding after 1-5 days. In 3 prophylactic cases before abortion or uterine curettage, obstetric massive haemorrhage occurred in 1 case, but not so in other 2 cases. Conclusions: TAE is an effective treatment for obstetric massive haemorrhage, with the advantages of minimal trauma, fast and definite treating effect and less complications. Prophylactical application for high risk patients can reduce the bleeding and mortality. (authors)

  15. An epistaxis severity score for hereditary hemorrhagic telangiectasia.

    Science.gov (United States)

    Hoag, Jeffrey B; Terry, Peter; Mitchell, Sally; Reh, Douglas; Merlo, Christian A

    2010-04-01

    Hereditary hemorrhagic telangiectasia (HHT)-related epistaxis leads to alterations in social functioning and quality of life. Although more than 95% experience epistaxis, there is considerable variability of severity. Because no standardized method exists to measure epistaxis severity, the purpose of this study was to determine factors associated with patient-reported severity to develop a severity score. Prospective, survey-based study. HHT care providers and a focus group of patients were interviewed to determine epistaxis-associated factors. From this, an electronic survey was developed and administered to patients with HHT. Descriptive analyses were performed with calculations of means and medians for continuous and proportions for categorical variables. Multiple ordinal logistic and linear regression models were developed to determine risk factors for epistaxis severity. Nine hundred respondents from 21 countries were included. Eight hundred fifty-five (95%) subjects reported epistaxis. The mean (standard deviation) age was 52.1 (13.9) years, and 61.4% were female. Independently associated risk factors for self-reported epistaxis severity included epistaxis frequency (odds ratio [OR] 1.57), duration (OR 2.17), intensity (OR 2.45), need for transfusion (OR 2.74), anemia (OR 1.44), and aggressiveness of treatment required (OR 1.53, P epistaxis severity in patients with HHT include frequency, duration, and intensity of episodes; invasiveness of prior therapy required to stop epistaxis; anemia; and the need for blood transfusion. From these factors, an epistaxis severity score will be presented.

  16. Prospective study of sentinel headache in aneurysmal subarachnoid haemorrhage.

    NARCIS (Netherlands)

    Linn, F.H.H.; Wijdicks, E.F.M.; Graaf, Y. van der; Weerdesteyn-van Vliet, F.A.C.; Bartelds, A.I.M.; Gijn, J. van

    1994-01-01

    Retrospective surveys of patients with subarachnoid haemorrhage suggest that minor episodes with sudden headache (warning leaks) may precede rupture of an aneurysm, and that early recognition and surgery might lead to improved outcome. We studied 148 patients with sudden and severe headache

  17. Comparison of serum lipid profile in ischaemic and haemorrhagic stroke

    International Nuclear Information System (INIS)

    Mehmood, A.; Sharif, M.A.

    2010-01-01

    To compare serum lipid profile between patients of ischaemic and haemorrhagic strokes. Study Design: Cross sectional, comparative study. Place and Duration of Study: Military Hospital, Rawalpindi, from August 2004 to February 2005. Methodology: Patients with diagnosis of stroke comprising 100 consecutive patients each of ischaemic and haemorrhagic strokes were included in the study while patients on lipid lowering therapy were excluded from study. To determine the subtype of stroke, clinical examination followed by CT scan of brain was done. A serum sample after 8 hours of overnight fasting was taken on the next day of admission for both groups of patients. Total serum cholesterol, triglycerides, LDL cholesterol, VLDL-cholesterol and HDL-cholesterol was determined, using enzymatic colorimetric method. Statistical analysis was done by comparison of lipid profile in two subgroups, using proportion test for any significant difference. Results: The mean age at presentation of patients with stroke was 64.2+-12 years with a male to female ratio of 3.6:1. In 100 ischaemic stroke patients, raised serum total cholesterol was seen in 42, triglyceride in 04, LDL-cholesterol in 05 and VLDL-cholesterol in 07 patients. Serum HDL-cholesterol was below the normal reference in 31 cases. On the other hand, serum total cholesterol and triglycerides was raised in 05 patients each, LDL-cholesterol in 09 and VLDL-cholesterol in 03 patients of haemorrhagic stroke. Serum HDL-cholesterol was below normal in 04 patients of haemorrhagic stroke. On comparison, there were significantly greater number of patients with raised serum cholesterol and low HDL-cholesterol in ischaemic stroke than haemorrhagic stroke (p < 0.05). No statistical significance was found on comparing serum values of ischaemic and haemorrhagic stroke for triglycerides, LDL-cholesterol and VLDL-cholesterol. Conclusion: Ischaemic stroke patients had high serum total cholesterol and lower HDL-cholesterol levels as compared to

  18. Factors affecting the occurrence of symptomatic intracerebral haemorrhage after intravenous thrombolysis depending on the haemorrhage definition.

    Science.gov (United States)

    Sledzińska-Dźwigał, M; Sobolewski, Piotr; Szczuchniak, W

    2013-01-01

    Symptomatic intracerebral haemorrhage (sICH) remains the most feared complication of systemic thrombolysis in patients with ischaemic stroke. The aim of the study was to analyze the impact of different factors on the occurrence of sICH, depending on definition used. We retrospectively evaluated the influence of several factors on the occurrence of sICH (according to definitions used in ECASS2, SITS-MOST and NINDS studies) in 200 patients treated with systemic thrombolysis from 2006 to 2011. Multivariate analysis of impact of individual variables on the occurrence of haemorrhagic transformation (HT) and parenchymal haemorrhage type 2 (PH2) were performed. Haemorrhagic transformation occurred in 35 cases (17.5%). SICH was found in 10 cases according to ECASS2, in 7 cases according to SITS and in 13 cases according to NINDS. Older age was related to higher risk of sICH, regardless which definition was used (ECASS2: p = 0.014, SITS-MOST: p = 0.048, NINDS: p = 0.008), and female sex was related to higher risk of sICH according to NINDS and ECASS2 definition (p = 0.002 and p = 0.04, respectively). Blood glucose level and high NIHSS score (> 14 pts) were found as risk factor of sICH in ECASS2 definition (p = 0.044 and p = 0.03, respectively). In multivariate logistic regression higher NIHSS scores were associated with HT independent of age, gender and glucose level (p = 0.012). Multivariate analysis showed no impact of age, gender, severity of stroke and glucose level on presence of PH2. Definition of sICH can determine variables that are related to a high risk of this complication. In our study most factors correlated with sICH using the ECASS2 definition.

  19. Massive retroperitoneal haemorrhage after extracorporeal shock wave lithotripsy (ESWL).

    Science.gov (United States)

    Inoue, Hiromasa; Kamphausen, Thomas; Bajanowski, Thomas; Trübner, Kurt

    2011-01-01

    A 76-year-old male suffering from nephrolithiasis developed a shock syndrome 5 days after extracorporal shock wave lithotripsy (ESWL). CT scan of the abdomen showed massive haemorrhage around the right kidney. Although nephrectomy was performed immediately, the haemorrhage could not be controlled. Numerous units of erythrocytes were transfused, but the patient died. The autopsy revealed massive retroperitoneal haemorrhage around the right kidney. The kidney showed a subcapsular haematoma and a rupture of the capsule. The right renal artery was dissected. The inferior vena cava was lacerated. Accordingly, a hemorrhagic shock as the cause of death was determined, which might mainly have resulted from the laceration of the inferior vena cava due to ESWL. ESWL seems to be a relatively non-invasive modality, but one of its severe complications is perirenal hematoma. The injuries of the blood vessels might have been caused by excessive shock waves. Subsequently, anticoagulation therapy had been resumed 3 days after EWSL, which might have triggered the haemorrhage. Physicians should note that a haemorrhage after an ESWL can occur and they should pay attention to the postoperative management in aged individuals especially when they are under anticoagulation therapy.

  20. THE PREVALENCE OF INTRAVENTRICULAR HAEMORRHAGE

    African Journals Online (AJOL)

    Esem

    haemorrhage in preterm infants with birth weight 1.5kg and less was 34.2% in ... case fatality rate was 85.7% for those with grade 4 in the first three days of life. ... with case fatality rates of more than 45% (2007 and 2008 NICU ward statistics).

  1. Imaging study of lymphoreticular tumor development in ataxia-telangiectasia and Nijmegen breakage syndrome

    International Nuclear Information System (INIS)

    Martinez-Leon, M. I.; Ceres-Ruiz, L.; Cuesta, M. A.; Garcia-Martin, F. J.

    2003-01-01

    Ataxia-telangiectasia (AT), or Louis-Bar syndrome, is an autosomal recessive illness characterized by progressive cerebellar ataxia, oculo-cutaneous telangiectasia, immunodeficiency combined with susceptibility to sinopulmonary infections and high incidence of neoplastic development. Nijmegen breakage syndrome (NBS) is a variant of AT, is also an autosomal recessive illness that presents cerebellar ataxia, as well as combined immunodeficiency and a tendency toward tumor development. Contrary to Louis-Bar syndrome, it doesn't present telangiectasia and exhibits a characteristics phenotype (short stature, bird-like face and microcephaly). Both entities are classified as syndrome of chromosomal instability or chromosomal fragility, a group which also includes Bloom syndrome and Fanconi anemia. All of these show an increase in the frequency of neoplastic pathologies, mainly lymphoid tumors. We present three patients,two with AT and one with NBS, who developed different lymphoma types in the course of the illness. We highlight the most outstanding aspects from a clinical-radiological point of view. (Author) 17 refs

  2. Uterine artery embolization: an effective treatment for intractable obstetric haemorrhage

    Energy Technology Data Exchange (ETDEWEB)

    Hong, T.-M.; Tseng, H.-S. E-mail: hstseng@vghtpe.gov.tw; Lee, R.-C.; Wang, J.-H.; Chang, C.-Y

    2004-01-01

    AIM: To present the findings of uterine artery embolization (UAE) in the management of obstetric haemorrhage. MATERIALS AND METHODS: From October 1999 to February 2003, 10 women with postpartum haemorrhage (n=7) and post-abortion haemorrhage with placenta accreta (n=3), were referred to our department for pelvic angiography and possible arterial embolization. RESULTS: Angiography revealed engorged and tortuous uterine arteries in all patients; and contrast medium extravasation in three patients. Eight patients (three with and five without detectable active bleeding) then underwent bilateral UAE. Medium-sized (250-355 {mu}m) polyvinyl alcohol particles were injected via a coaxial catheter into the uterine arteries, followed by gelatin sponge pieces via a 4 F Cobra catheter. Microcoil devascularization was also performed in the two patients with visible, active bleeding. The vaginal bleeding resolved in all patients, without any ischaemic complications. At follow-up, all patients who underwent UAE had normal menstruation; three of them subsequently gave birth to full-term healthy babies. CONCLUSION: Selective UAE by the coaxial method is safe and effective to control obstetric haemorrhage, with the potential to preserve fertility.

  3. Spontaneous haemorrhage and rupture of third ventricular colloid cyst.

    LENUS (Irish Health Repository)

    Ogbodo, Elisha

    2012-01-01

    Acute bleeding within a colloid cyst of the third ventricle represents a rare event causing sudden increase in the cyst volume that may lead to acute hydrocephalus and rapid neurological deterioration. We report a case of spontaneous rupture of haemorrhagic third ventricular colloid cyst and its management. A 77-year-old ex-smoker presented with unsteady gait, incontinence and gradually worsening confusion over a 3-week period. Brain CT scan findings were highly suggestive of a third ventricular colloid cyst with intraventricular rupture. He underwent cyst excision and histopathology, which confirmed the radiological diagnosis with evidence of haemorrhage within the cyst. A ventriculo peritoneal shunt was performed for delayed hydrocephalus. Surgical management of these patients must include emergency ventriculostomy followed by prompt surgical removal of the haemorrhagic cyst.

  4. What are the current therapeutic options for haemorrhagic strokes

    International Nuclear Information System (INIS)

    Khan, M.; Kamal, A.K.

    2010-01-01

    The Factor Seven for Acute Haemorrhagic Stroke Trial (FAST) and Intensive Blood Pressure Reduction in Acute Cerebral Haemorrhage trial (INTERACT). Intracerebral haemorrhage (ICH) is the most devastating form of stroke carrying a mortality of up to 40% at one month. In our part of the world the levels of uncontrolled hypertension pose a greater risk of ICH for our population where ICH is about 30% of all strokes compared to 14% in developed countries. There is evidence to suggest that haematoma expansion occurs in as many as 70% of patients. This expansion translates into increased disability and death. Most of this expansion is within the initial three hours. Also early elevation of blood pressure (BP) is very common after ICH and many studies have highlighted an association between elevated BP post ICH and poor outcomes. The reason for this is postulated to be an increase in both the size of the haematoma and perilesional oedema. (author)

  5. Ataxia telangiectasia: LET dependence of cellular inactivation

    International Nuclear Information System (INIS)

    Blakely, E.A.; Tobias, C.A.

    1984-01-01

    Human Ataxia telangiectasia cells (AT 2SF line) have been irradiated in vitro under aerobic and hypoxic conditions with heavy-ion beams accelerated at the Berkeley Bevalac as a part of a study to characterize the radiation responses of genetically sensitive and resistant cell lines to high LET radiations. Results from track-segment exposures to neon, silicon, argon and iron ion beams accelerated to initial energies of from 225 to 670 MeV/amu provided an LET range between 30 to 1,000 KeV/μm. The data indicate: (1) The sensitivity of AT cells increases with increasing LET, similar to resistant human lines (e.g., T-1 cells). However, due to efficient repair, T-1 cells are more resistant than AT cells at LET values below 200 keV/μm; (2) Maximum cell kill occurs for both lines at 100-200 keV/μm; at higher LET the sensitivity of the two lines approach each other; (3) There is only small variation in the sensitivity of AT cells to particles of various atomic numbers at the same LET; differences are more pronounced in the LET domain between 50 and 200 keV/μm; and (4) AT cells have slightly lower OER values than T-1 cells in the range of LET studied below 200 keV/μm

  6. Glutamine deprivation induces interleukin-8 expression in ataxia telangiectasia fibroblasts.

    Science.gov (United States)

    Kim, Min-Hyun; Kim, Aryung; Yu, Ji Hoon; Lim, Joo Weon; Kim, Hyeyoung

    2014-05-01

    To investigate whether glutamine deprivation induces expression of inflammatory cytokine interleukin-8 (IL-8) by determining NF-κB activity and levels of oxidative indices (ROS, reactive oxygen species; hydrogen peroxide; GSH, glutathione) in fibroblasts isolated from patients with ataxia telangiectasia (A-T). We used A-T fibroblasts stably transfected with empty vector (Mock) or with human full-length ataxia telangiectasia mutated (ATM) cDNA (YZ5) and mouse embryonic fibroblasts (MEFs) transiently transfected with ATM small interfering RNA (siRNA) or with non-specific control siRNA. The cells were cultured with or without glutamine or GSH. ROS levels were determined using a fluorescence reader and confocal microscopy. IL-8 or murine IL-8 homolog, keratinocyte chemoattractant (KC), and hydrogen peroxide levels in the medium were determined by enzyme-linked immunosorbent assay and colorimetric assay. GSH level was assessed by enzymatic assay, while IL-8 (KC) mRNA level was measured by reverse transcription-polymerase chain reaction (RT-PCR) and/or quantitative real-time PCR. NF-κB DNA-binding activity was determined by electrophoretic mobility shift assay. Catalase activity and ATM protein levels were determined by O2 generation and Western blotting. While glutamine deprivation induced IL-8 expression and increased NF-κB DNA-binding activity in Mock cells, both processes were decreased by treatment of cells with glutamine or GSH or both glutamine and GSH. Glutamine deprivation had no effect on IL-8 expression or NF-κB DNA-binding activity in YZ5 cells. Glutamine-deprived Mock cells had higher oxidative stress indices (increases in ROS and hydrogen peroxide, reduction in GSH) than glutamine-deprived YZ5 cells. In Mock cells, glutamine deprivation-induced oxidative stress indices were suppressed by treatment with glutamine or GSH or both glutamine and GSH. GSH levels and catalase activity were lower in Mock cells than YZ5 cells. MEFs transfected with ATM siRNA and

  7. Telangiectasia hemorrágica hereditária e malformações arteriovenosas pulmonares - Embolização com rolhão vascular Amplatzer Hereditary hemorrhagic telangiectasia and pulmonary arteriovenous malformations - Embolization with Amplatzer vascular plug

    Directory of Open Access Journals (Sweden)

    Cláudia Sofia Santos

    2009-03-01

    Full Text Available As malformações arteriovenosas pulmonares (MAVP estão associadas a telangiectasia hemorrágica hereditária em cerca de 70% dos casos, podendo cursar com complicações neurológicas graves decorrentes do embolismo paradoxal potencial. A terapêutica das malformações é realizada no sentido de prevenir estas complicações. Descreve-se o caso clínico de uma doente com duas MAVP, no contexto de telangiectasia hemorrágica hereditária,submetida a embolização com o rolhão vascular Amplatzer. Este novo dispositivo é cada vez mais utilizado nestas situações, salientando-se algumas das suas características e vantagens em relação a outras técnicas de embolização mais frequentemente utilizadas.Pulmonary arteriovenous malformations (PAVMs are associated to hereditary hemorrhagic telangiectasia in about 70% of the cases. PAVMs are associated to serious neurologic complications, secondary to inherent paradoxical embolisms. Treatment of the malformations is done to prevent these complications. The authors report a clinical case of a patient with two PAVMs, with hereditary hemorrhagic telangiectasia, who was treated by embolization with na Amplatzer vascular plug, a new device increasingly used in these situations. The authors emphasise some of its characteristics and advantages when compared with other embolization techniques most commonly used.

  8. Tranexamic acid for control of haemorrhage in acute promyelocytic leukaemia

    NARCIS (Netherlands)

    Avvisati, G.; ten Cate, J. W.; Büller, H. R.; Mandelli, F.

    1989-01-01

    In a double-blind study, 12 consecutive patients with acute promyelocytic leukaemia were randomised either to tranexamic acid (TA group) or to placebo (control group) for 6 days to see whether inhibition of fibrinolysis would reduce haemorrhage and transfusion requirements. The total study period

  9. Haemorrhage in the labyrinth caused by anticoagulant therapy: case report

    International Nuclear Information System (INIS)

    Callonnec, F.; Gerardin, E.; Thiebot, J.; Marie, J.P.; Andrieu Guitrancourt, J.; Marsot-Dupuch, K.

    1999-01-01

    We report a patient who experienced a severe vertiginous episode with bilateral tinnitus and progressive right-sided hearing loss. She had Marfan's disease and was on anticoagulant treatment. The fluid in the labyrinth gave higher signal than cerebrospinal fluid on T1-weighted images, suggesting haemorrhage. The radiological follow-up is discussed. (orig.)

  10. Haemorrhage in the labyrinth caused by anticoagulant therapy: case report

    Energy Technology Data Exchange (ETDEWEB)

    Callonnec, F; Gerardin, E; Thiebot, J [Department of Radiology, Rouen University Hospital, 1 rue de Germont, F-76031 Rouen cedex (France); Marie, J P; Andrieu Guitrancourt, J [Department of Otolaryngology, Rouen University Hospital (France); Marsot-Dupuch, K [Department of Radiology, St. Antoine, Paris University Hospital (France)

    1999-06-01

    We report a patient who experienced a severe vertiginous episode with bilateral tinnitus and progressive right-sided hearing loss. She had Marfan`s disease and was on anticoagulant treatment. The fluid in the labyrinth gave higher signal than cerebrospinal fluid on T1-weighted images, suggesting haemorrhage. The radiological follow-up is discussed. (orig.) With 2 figs., 11 refs.

  11. Hypopituitarism after subarachnoid haemorrhage, do we know enough?

    NARCIS (Netherlands)

    L. Khajeh (Ladbon); K. Blijdorp (Karin); S.J.C.M.M. Neggers (Bas); G.M. Ribbers (Gerard); D.W.J. Dippel (Diederik); F. van Kooten (Fop)

    2014-01-01

    textabstractBackground: Fatigue, slowness, apathy and decrease in level of activity are common long-term complaints after a subarachnoid haemorrhage (SAH). They resemble the symptoms frequently found in patients with endocrine dysfunction. Pituitary dysfunction may be the result of SAH or its

  12. An evaluation of the severity and progression of epistaxis in hereditary hemorrhagic telangiectasia 1 versus hereditary hemorrhagic telangiectasia 2.

    Science.gov (United States)

    Hunter, Benjamin N; Timmins, Benjamin H; McDonald, Jamie; Whitehead, Kevin J; Ward, P Daniel; Wilson, Kevin F

    2016-04-01

    Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular dysplasia whose hallmark symptom is spontaneous recurrent epistaxis. Two major genetic subtypes of this syndrome are HHT1 and HHT2. Severity of epistaxis ranges from occasional low-volume bleeding to frequent large-volume hemorrhage. This study evaluated the severity and progression of epistaxis in HHT1 versus HHT2. Retrospective cohort study. A retrospective chart review was performed for 183 genotyped HHT patients seen at our center from 2010 to 2013. Data collected included epistaxis severity score (ESS), age of epistaxis onset, number and type of treatments, age at which treatments were sought, complete blood count values, ferritin, number of telangiectases, blood transfusions, iron therapy history, and patient demographics. 115 subjects with HHT2 were compared to 68 with HHT1. Subjects with HHT2 had a higher ESS compared to HHT1 (P = .043) and a later age of onset of epistaxis (P = .005). HHT2 subjects were more likely to use oral iron (P = .032) and were more likely to seek interventions to control their epistaxis (P = .029). HHT2 is associated with more severe epistaxis and a subsequent higher rate of interventions, requiring more aggressive therapy as compared to HHT1. 4. © 2015 The American Laryngological, Rhinological and Otological Society, Inc.

  13. Three-dimensional reconstruction and volumetry of intracranial haemorrhage and its mass effect

    International Nuclear Information System (INIS)

    Strik, H.M.; Baehr, M.; Borchert, H.; Fels, C.; Knauth, M.; Rienhoff, O.; Verhey, J.F.

    2005-01-01

    Intracerebral haemorrhage still causes considerable disability and mortality. The studies on conservative and operative management are inconclusive, probably due to inexact volumetry of the haemorrhage. We investigated whether three-dimensional (3-D), voxel-based volumetry of the haemorrhage and its mass effect is feasible with routine computed tomography (CT) scans. The volumes of the haemorrhage, ventricles, midline shift, the intracranial volume and ventricular compression in CT scans of 12 patients with basal ganglia haemorrhage were determined with the 3-D slicer software. Indices of haemorrhage and intracranial or ventricular volume were calculated and correlated with the clinical data. The intended measures could be determined with an acceptable intra-individual variability. The 3-D volumetric data tended to correlate better with the clinical course than the conventionally assessed distance of midline shift and volume of haemorrhage. 3-D volumetry of intracranial haemorrhage and its mass effect is feasible with routine CT examination. Prospective studies should assess its value for clinical studies on intracranial space-occupying diseases. (orig.)

  14. Defect in radiation signal transduction in ataxia-telangiectasia

    International Nuclear Information System (INIS)

    Lavin, M.F.

    1994-01-01

    Exposure of mammalian cells to ionizing radiation causes a delay in progression through the cycle at several checkpoints. Cells from patients with ataxia-telangiectasia (A-T) ignore these checkpoint controls postirradiation. The tumour suppressor gene product p53 plays a key role at the G 1 /S checkpoint preventing the progression of cells into S phase. The induction of p53 by radiation is reduced and/or delayed in A-T cells, which appears to account for the failure of delay at the G 1 /S checkpoint. We have investigated further this defect in radiation signal transduction in A-T. While the p53 response was defective after radiation, agents that interfered with cell cycle progression such as mimosine, aphidicolin and deprivation of serum led to a normal p53 response in A-T cells. None of these agents caused breaks in DNA, as determined by pulse-field gel electrophoresis, in order to elicit the response. Since this pathway is mediated by protein kinases, we investigated the activity of several of these enzymes in control and A-T cells. Ca +2 -dependent and -independent protein kinase C activities were increased by radiation to the same extent in the two cell types, a variety of serine/threonine protein kinase activities were approximately the same and anti-tyrosine antibodies failed to reveal any differences in protein phosphorylation between A-T and control cells. (author)

  15. Diagnostic accuracy of guys Hospital stroke score (allen score) in acute supratentorial thrombotic/haemorrhagic stroke

    International Nuclear Information System (INIS)

    Zulfiqar, A.; Toori, K. U.; Khan, S. S.; Hamza, M. I. M.; Zaman, S. U.

    2006-01-01

    A consecutive series of 103 patients, 58% male with mean age of 62 year (range 40-75 years), admitted with supratentorial stroke in our teaching hospital were studied. All patients had Computer Tomography scan brain done after clinical evaluation and application of Allen stroke score. Computer Tomography Scan confirmed thrombotic stroke in 55 (53%) patients and haemorrhagic stroke in 48 (47%) patients. Out of the 55 patients with definitive thrombotic stroke on Computer Tomography Scan, Allen stroke score suggested infarction in 67%, haemorrhage in 6% and remained inconclusive in 27% of cases. In 48 patients with definitive haemorrhagic stroke on Computer Tomography Scan, Allen stroke score suggested haemorrhage in 60%, infarction in 11% and remained inconclusive in 29% of cases. The overall accuracy of Allen stroke score was 66%. (author)

  16. Haemorrhagic Fevers, Viral

    Science.gov (United States)

    ... plan 2015: West Africa Ebola outbreak Health worker Ebola infections in Guinea, Liberia and Sierra Leone Infection prevention and control guidance for care of Ebola patients Publications, technical guidance on Ebola Related topics ...

  17. Late haemorrhagic disease of the newborn.

    Science.gov (United States)

    Zengin, Emine; Sarper, Nazan; Türker, Gülcan; Corapçioğlu, Funda; Etuş, Volkan

    2006-09-01

    Late haemorrhagic disease of the newborn (HDN) can occur owing to a lack of vitamin K prophylaxis, as a manifestation of an underlying disorder or idiopatically from the 8th day to 12 weeks after birth. Eight infants admitted to Kocaeli University Hospital with nine episodes of late HDN between January 2002 and April 2005 were evaluated retrospectively from hospital records. The median age at presentation was 46 (26-111) days. All the infants were born at full-term to healthy mothers and were exclusively breast-fed. All had an uneventful perinatal history, except one who had meconium aspiration. Four patients had received no vitamin K prophylaxis and another three had uncertain histories. At presentation, six had intracranial bleeding and the remainder had bleeding either from the venepuncture site or the gastro-intestinal tract. The presenting signs and symptoms were irritability, vomiting, bulging or full fontanelle, convulsions and diminished or absent neonatal reflexes. Galactosaemia was detected in a 2-month-old infant with prolonged jaundice. There was no surgery-related mortality or complications but one survived for only 2 days on ventilatory support following surgery. Only one of the six survivors had severe neurological sequelae. Late HDN frequently presents with intracranial haemorrhage, leading to high morbidity and mortality. HDN can be the manifestation of an underlying metabolic disorder. Vitamin K prophylaxis of the newborn should be routine in developing countries.

  18. Hereditary Hemorrhagic Telangiectasia Presenting as High Output Cardiac Failure during Pregnancy

    Directory of Open Access Journals (Sweden)

    Tareq Goussous

    2009-01-01

    Full Text Available High-output cardiac failure secondary to hepatic involvement is a rare complication of hereditary hemorrhagic telangiectasia (HHT. Here we report a 43-year-old woman who presented at 29 weeks gestation of her second pregnancy with complications of right-sided heart failure and preterm labor. After delivery via cesarean section, the patient was found to have intrahepatic arteriovenous malformations through non-invasive imaging. Subsequently, a family history of vascular malformations and epistaxis was elucidated and a diagnosis of HHT was made. This case is presented, along with a review of the literature and discussion of hepatic involvement in HHT with particular focus on the pregnant patient.

  19. Perimetric demonstration of spontaneous visual field recovery following occipital lobe haemorrhage.

    Science.gov (United States)

    Lin, Siying; George, Badie Z; Wilson-Holt, Nicholas J

    2013-08-29

    A 45-year-old patient on lifelong warfarin therapy after a metal aortic valve replacement developed a homonymous visual field defect following an occipital lobe haemorrhage. The patient received only conservative management and yet described continued improvement in her visual field defect for up to 20 months following the initial cerebral insult. We present the first conclusive illustrative documentation of visual recovery in a patient with an occipital lobe haemorrhage with sequential automated perimetric assessments over an extended period of time.

  20. Case Report: Neuro-Imaging Findings in Ataxia Telangiectasia

    Directory of Open Access Journals (Sweden)

    Farhad Mahvelati

    2004-06-01

    Full Text Available Ataxia Telangiectasia (AT is an autosomal recessive inherited disorder in which cutaneous and scleral Telangiectasia, cerebellar ataxia and immunodeficiency occur. There is a high incidence of development of malignant tumors, mainly lymphomas. Cerebellar atrophy is the most prominent abnormality and is shown better by magnetic resonance imaging (MRI than CT-Scan. Intracranial hemorrhage occurs rarely. We report a 7 years old boy who admitted for recurrent pulmonary infections. His examination showed ataxic gait with decreased deep tendon reflexes in lower extremities. He had telangiectasia in the eyes and his speech was slurred and difficult. Brain MRI showed cerebellar atrophy with diffuse hyperintensity in white matter, most prominent in occipital region, which was suggestive of leukodystrophy. This white matter change was not reported before in AT.

  1. Relationship between lunar cycle and haemorrhagic complication rate in surgery.

    Science.gov (United States)

    Raposio, Edoardo; Caruana, Giorgia; Santi, Pierluigi; Cafiero, Ferdinando

    2017-08-01

    The aim of this study was to evaluate a possible relationship between lunar cycles and haemorrhagic complication rate in surgery. The possible relationship between moon phases and surgical outcome was tested by evaluating the haemorrhagic complication rate for 18,760 patients who underwent surgery between January 2001 and December 2008 at the National Institute for Cancer Research in Genoa. A total of 103 lunar phases were considered using Chi-square (χ 2 ) test analysis, and patients were allocated a surgery date. One hundred and sixty-seven haemorrhagic complications were observed. Three hundred and nine new moon phase days were analysed and 12 incidences of complications detected, with a 3.9% complication rate per day. In the waxing moon phase, 1184.5 d were analysed with 68 incidences of complications at a daily rate of 5.7%. In the full moon phase there was a 4.9% complication rate per day (15 incidences in 309 d), whereas in the waning moon phase, the 6% percentage rate per day resulted from 72 incidences in 1184.5 d. No statistically significant correlations were found between moon cycles and postoperative haemorrhagic complications (p = .50).

  2. CT and MRI of haemorrhage into intracranial neuromas

    Energy Technology Data Exchange (ETDEWEB)

    Asari, S. (Dept. of Neurological Surgery, Okayama Univ. Medical School, Okayama City (Japan)); Katayama, S. (Dept. of Neurological Surgery, Okayama Univ. Medical School, Okayama City (Japan)); Itoh, T. (Dept. of Neurological Surgery, Okayama Univ. Medical School, Okayama City (Japan)); Tsuchida, S. (Dept. of Neurological Surgery, Okayama Univ. Medical School, Okayama City (Japan)); Ohmoto, T. (Dept. of Neurological Surgery, Okayama Univ. Medical School, Okayama City (Japan))

    1993-04-01

    Six patients with haemorrhage into intracranial neuromas were studied by computed tomography (CT) and magnetic resonance imaging (MRI) at 0.5 T with spin-echo pulse sequences. The nature of the tumour and the presence of a haematoma were confirmed by surgery and microscopic examination in all cases. Four neuromas arose from the acoustic nerves and two from the trigeminal. Four of the six patients suffered from sudden onset or rapid worsening of symptoms including headache, vertigo and/or hemifacial motor and sensory disturbances. CT in the acute stage revealed a hyperdense area or a fluid-fluid level (FFL). The hyperdense area disappeared on CT repeated in the chronic stage. On MRI in subacute and chronic stages the haemorrhage showed hyperintensity on both T1 and T2 weighting in five cases examined between 16 and 46 days after the onset, and isointensity on T1 weighting and an FFL on T2 weighting in one case examined 12 days after the onset of symptoms. A well-defined low intensity rim indicating prior haemorrhage was observed on T2-weighted images in three cases. MRI was more effectie than CT in detecting haemorrhage into the tumours and in staging it. (orig.)

  3. [Ebola haemorrhagic fever.

    DEFF Research Database (Denmark)

    Fabiansen, C.; Kronborg, G.; Thybo, S.

    2008-01-01

    This review presents the latest findings on ebola. Ebola presents one of the highest case-fatality rates of all infectious diseases, and in 2007 outbreaks were observed first in the Democratic Republic of Congo and later in Uganda with a new subtype. Accumulating evidence suggests that fruit bats...... are a likely reservoir for the ebola virus. The frequency of filovirus outbreaks in Central Africa is increasing and the potential for introduction and patient care in Denmark is evaluated Udgivelsesdato: 2008/11/24...

  4. Decompressive craniectomy in aneurysmal subarachnoid haemorrhage for hematoma or oedema versus secondary infarction

    NARCIS (Netherlands)

    Goedemans, Taco; Verbaan, Dagmar; Coert, Bert A.; Sprengers, Marieke E. S.; van den Berg, René; Vandertop, W. Peter; van den Munckhof, Pepijn

    2017-01-01

    Decompressive craniectomy (DC) has been proposed as lifesaving treatment in aneurysmal subarachnoid haemorrhage (aSAH) patients with elevated intracranial pressure (ICP). However, data is sparse and controversy exists whether the underlying cause of elevated ICP influences neurological outcome. The

  5. Surgical Trial in Lobar Intracerebral Haemorrhage (STICH II Protocol

    Directory of Open Access Journals (Sweden)

    Rowan Elise N

    2011-05-01

    Full Text Available Abstract Background Within the spectrum of spontaneous intracerebral haemorrhage there are some patients with large or space occupying haemorrhage who require surgery for neurological deterioration and others with small haematomas who should be managed conservatively. There is equipoise about the management of patients between these two extremes. In particular there is some evidence that patients with lobar haematomas and no intraventricular haemorrhage might benefit from haematoma evacuation. The STICH II study will establish whether a policy of earlier surgical evacuation of the haematoma in selected patients will improve outcome compared to a policy of initial conservative treatment. Methods/Design an international multicentre randomised parallel group trial. Only patients for whom the treating neurosurgeon is in equipoise about the benefits of early craniotomy compared to initial conservative treatment are eligible. All patients must have a CT scan confirming spontaneous lobar intracerebral haemorrhage (≤1 cm from the cortex surface of the brain and 10-100 ml in volume. Any clotting or coagulation problems must be corrected and randomisation must take place within 48 hours of ictus. With 600 patients, the study will be able to demonstrate a 12% benefit from surgery (2p Stratified randomisation is undertaken using a central 24 hour randomisation service accessed by telephone or web. Patients randomised to early surgery should have the operation within 12 hours. Information about the status (Glasgow Coma Score and focal signs of all patients through the first five days of their trial progress is also collected in addition to another CT scan at about five days (+/- 2 days. Outcome is measured at six months via a postal questionnaire to the patient. Primary outcome is death or severe disability defined using a prognosis based 8 point Glasgow Outcome Scale. Secondary outcomes include: Mortality, Rankin, Barthel, EuroQol, and Survival. Trial

  6. Haemorrhage and intestinal lymphoma

    Directory of Open Access Journals (Sweden)

    Attilia M. Pizzini

    2013-04-01

    Full Text Available Background: The prevalence of coeliac disease is around 1% in general population but this is often unrecognised. The classical presentation of adult coeliac disease is characterized by diarrhoea and malabsorption syndrome, but atypical presentations are probably more common and are characterized by iron deficiency anaemia, weight loss, fatigue, infertility, arthralgia, peripheral neuropathy and osteoporosis. Unusual are the coagulation disorders (prevalence 20% and these are due to vitamin K malabsorption (prolonged prothrombin time. Clinical case: A 64-year-old man was admitted to our Department for an extensive spontaneous haematoma of the right leg. He had a history of a small bowel resection for T-cell lymphoma, with a negative follow-up and he didn’t report any personal or familiar history of bleeding. Laboratory tests showed markedly prolonged prothrombin (PT and partial-thromboplastin time (PTT, corrected by mixing studies, and whereas platelet count and liver tests was normal. A single dose (10 mg of intravenous vitamin K normalized the PT. Several days before the patient had been exposed to a superwarfarin pesticide, but diagnostic tests for brodifacoum, bromadiolone or difenacoum were negative. Diagnosis of multiple vitamin K-dependent coagulationfactor deficiencies (II, VII, IX, X due to intestinal malabsorption was made and coeliac disease was detected. Therefore the previous lymphoma diagnosis might be closely related to coeliac disease. Conclusions: A gluten free diet improves quality of life and restores normal nutritional and biochemical status and protects against these complications.

  7. Excision of gamma-ray induced thymine lesions by preparations from ataxia telangiectasia fibroblasts

    Energy Technology Data Exchange (ETDEWEB)

    Remsen, J F; Cerutti, P A [Florida Univ., Gainesville (USA). Inst. of Food and Agricultural Sciences; Florida Univ., Gainesville (USA). Dept. of Biochemistry)

    1977-04-01

    The capacity of whole cell sonicates of skin fibroblasts of normal individuals and patients with the autosomal recessive disease Ataxia telangiectasia (AT) to remove aerobic gamma-ray products of the 5,6-dihydroxydihydrothymine type (tsub(O/sub 2/)sup(..gamma..)) from exogenous DNA substrates was investigated. All four AT strains (AT CRL 1312, AT CRL 1343, AT GM 367, AT 4BI) possessed normal capabilities to excise tsub(O/sub 2/)sup(..gamma..) from irradiated bacteriophage DNA and irradiated chromatin isolated from normal and AT-skin fibroblasts.

  8. Ataxia Telangiectasia - A Report of a case in Port Harcourt

    African Journals Online (AJOL)

    TNHJOURNALPH

    ocularand cutaneous telangiectasia, and a predisposition to ... complications because early management with monitoring of lung ... Blymphoid malignancy and hypersensitivity to ionizing radiation. 4 Data on the prevalence of A-T in Nigeria is limited. Few cases of A-T have been reported in Nigeria. 5 The prevalence.

  9. Filoviral haemorrhagic fevers: A threat to Zambia?

    Directory of Open Access Journals (Sweden)

    Katendi Changula

    2012-06-01

    Full Text Available Filoviral haemorrhagic fevers (FVHF are caused by agents belonging to Filoviridae family, Ebola and Marburg viruses. They are amongst the most lethal pathogens known to infect humans. Incidence of FVHF outbreaks are increasing, with affected number of patients on the rise. Whilst there has been no report yet of FVHF in Zambia, its proximity to Angola and Democratic Republic of Congo, which have recorded major outbreaks, as well as the open borders, increased trade and annual migration of bats between these countries, puts Zambia at present and increased risk. Previous studies have indicated bats as potential reservoir hosts for filoviruses. An increasing population with an increasing demand for resources has forced incursion into previously uninhabited land, potentially bringing them into contact with unknown pathogens, reservoir hosts and/or amplifying hosts. The recent discovery of a novel arenavirus, Lujo, highlights the potential that every region, including Zambia, has for being the epicentre or primary focus for emerging and re-emerging infections. It is therefore imperative that surveillance for potential emerging infections, such as viral haemorrhagic fevers be instituted. In order to accomplish this surveillance, rapid detection, identification and monitoring of agents in patients and potential reservoirs is needed. International co-operation is the strategy of choice for the surveillance and fight against emerging infections. Due to the extensive area in which filoviral infections can occur, a regional approach to surveillance activities is required, with regional referral centres. There is a need to adopt shared policies for the prevention and control of infectious diseases. There is also need for optimisation of currently available tests and development of new diagnostic tests, in order to have robust, highly sensitive and specific diagnostic tests that can be used even where there are inadequate laboratories and diagnostic services.

  10. Hepatitis C in haemorrhagic obstetrical emergencies

    International Nuclear Information System (INIS)

    Khaskheli, M.; Baloch, S.

    2014-01-01

    Objective: To determine the maternal health and fetal outcome in hepatitis C with obstetrical haemorrhagic emergencies. Study Design: An observational study. Place and Duration of Study: Department of Obstetrics and Gynaecology Unit-I, Liaquat University of Medical and Health Sciences Hospital, Hyderabad, Sindh, from January 2009 to December 2010. Methodology: All the women admitted during the study period with different obstetrical haemorrhagic emergencies were included. On virology screening, hepatitis C screening was done on all. The women with non-haemorrhagic obstetrical emergencies were excluded. Studied variables included demographic characteristics, the nature of obstetrical emergency, haemorrhagic conditions and maternal and fetal morbidity and mortality. The data was analyzed on SPSS version 20. Results: More frequent obstetrical haemorrhagic emergencies were observed with hepatitis C positive in comparison with hepatitis C negative cases including post-partum haemorrhage in 292 (80.88%) and ante-partum haemorrhage in 69 (19.11%) cases. Associated morbidities seen were disseminated intravascular coagulation in 43 (11.91%) and shock in 29 (8.03%) cases with hepatitis C positive. Fetal still birth rate was 37 (10.24%) in hepatitis C positive cases. Conclusion: Frequency of maternal morbidity and mortality and perinatal mortality was high in obstetrical haemorrhagic emergencies with hepatitis C positive cases. (author)

  11. Bilateral spontaneous adrenal haemorrhage complicating acute pancreatitis

    International Nuclear Information System (INIS)

    Pianta, M.; Varma, D. K.

    2007-01-01

    Bilateral adrenal haemorrhage is an event that mandates prompt diagnosis and treatment to prevent primary adrenocortical insufficiency and potential death. Presentation can be non-specific and incidentally diagnosed with imaging alone, primarily CT. We present a case of acute pancreatitis with spontaneous bilateral adrenal haemorrhage and briefly discuss imaging and treatment implications

  12. Ultrasonically detectable cerebellar haemorrhage in preterm infants.

    LENUS (Irish Health Repository)

    McCarthy, Lisa Kenyon

    2011-07-01

    To determine the frequency and pattern of cerebellar haemorrhage (CBH) on routine cranial ultrasound (cUS) imaging in infants of ≤32 weeks gestation, and to investigate how extremely preterm infants with CBH differ from those with severe intraventricular haemorrhage (IVH).

  13. Patch: platelet transfusion in cerebral haemorrhage: study protocol for a multicentre, randomised, controlled trial

    Directory of Open Access Journals (Sweden)

    Dijkgraaf Marcel G

    2010-03-01

    Full Text Available Abstract Background Patients suffering from intracerebral haemorrhage have a poor prognosis, especially if they are using antiplatelet therapy. Currently, no effective acute treatment option for intracerebral haemorrhage exists. Limiting the early growth of intracerebral haemorrhage volume which continues the first hours after admission seems a promising strategy. Because intracerebral haemorrhage patients who are on antiplatelet therapy have been shown to be particularly at risk of early haematoma growth, platelet transfusion may have a beneficial effect. Methods/Design The primary objective is to investigate whether platelet transfusion improves outcome in intracerebral haemorrhage patients who are on antiplatelet treatment. The PATCH study is a prospective, randomised, multi-centre study with open treatment and blind endpoint evaluation. Patients will be randomised to receive platelet transfusion within six hours or standard care. The primary endpoint is functional health after three months. The main secondary endpoints are safety of platelet transfusion and the occurrence of haematoma growth. To detect an absolute poor outcome reduction of 20%, a total of 190 patients will be included. Discussion To our knowledge this is the first randomised controlled trial of platelet transfusion for an acute haemorrhagic disease. Trial registration The Netherlands National Trial Register (NTR1303

  14. Laparoscopic management of massive spontaneous external haemorrhage from the umbilical varix due to recanalisation of the paraumbilical vein in a patient with ′Child′s Class A′ liver cirrhosis

    Directory of Open Access Journals (Sweden)

    Sanoop K Zachariah

    2012-01-01

    Full Text Available Spontaneous external haemorrhage from the umbilical varix is an extremely rare complication of portal hypertension. Bleeding is usually into the peritoneal cavity and the treatment involves urgent laparotomy and ligation of the bleeding varices. We describe a cirrhotic 38-year-old man who presented with spontaneous external haemorrhage from the umbilical varix which was successfully managed laparoscopically by in-situ distal clipping and proximal transcutaneous ligation of the recanalised paraumbilical veins. We therefore feel that laparoscopy can be safely and effectively employed to control external haemorrhage from the umbilical varix associated with liver cirrhosis. This novel technique can help avoid a laparotomy and also help preserve the umbilicus.

  15. Classification of non-aneurysmal subarachnoid haemorrhage: CT correlation to the clinical outcome

    International Nuclear Information System (INIS)

    Nayak, S.; Kunz, A.B.; Kieslinger, K.; Ladurner, G.; Killer, M.

    2010-01-01

    Aim: To propose a new computed tomography (CT)-based classification system for non-aneurysmal subarachnoid haemorrhage (SAH), which predicts patients' discharge clinical outcome and helps to prioritize appropriate patient management. Methods and materials: A 5-year, retrospective, two-centre study was carried out involving 1486 patients presenting with SAH. One hundred and ninety patients with non-aneurysmal SAH were included in the study. Initial cranial CT findings at admission were correlated with the patients' discharge outcomes measured using the Modified Rankin Scale (MRS). A CT-based classification system (type 1-4) was devised based on the topography of the initial haemorrhage pattern. Results: Seventy-five percent of the patients had type 1 haemorrhage and all these patients had a good clinical outcome with a discharge MRS of ≤1. Eight percent of the patients presented with type 2 haemorrhage, 62% of which were discharged with MRS of ≤1 and 12% of patients had MRS 3 or 4. Type 3 haemorrhage was found in 10%, of which 16% had good clinical outcome, but 53% had moderate to severe disability (MRS 3 and 4) and 5% were discharged with severe disability (MRS 5). Six percent of patients presented with type 4 haemorrhage of which 42% of the patients had moderate to severe disability (MRS 3 and 4), 42% had severe disability and one-sixth of the patients died. Highly significant differences were found between type 1(1a and 1b) and type 2 (p = 0.003); type 2 and type 3 (p = 0.002); type 3 and type 4 (p = 0.001). Conclusion: Haemorrhages of the type 1 category are usually benign and do not warrant an extensive battery of clinical and radiological investigations. Type 2 haemorrhages have a varying prognosis and need to be investigated and managed along similar lines as that of an aneurysmal haemorrhage with emphasis towards radiological investigation. Type 3 and type 4 haemorrhages need to be extensively investigated to find an underlying cause.

  16. Endovascular control of haemorrhagic urological emergencies: an observational study

    Directory of Open Access Journals (Sweden)

    Thorpe Peter

    2006-09-01

    Full Text Available Abstract Background Transarterial embolisation (TAE is an effective method in control of haemorrhage irrespective of the nature of urological emergency. As the technique and technology have evolved, it is now possible to perform highly selective embolisation. The aim of this study was to critically appraise feasibility and efficacy of therapeutic TAE in control of haemorrhagic urological emergencies using selective and non-selective embolisation. Specifically, we aimed to assess the impact of timing of embolisation on the requirement of blood transfusion and long-term morphological and functional follow-up of embolised organs. Methods This is a single institutional observational study carried out between March 1992 and March 2006. Records of all patients who underwent selective and non-selective angioembolisation to control bleeding in urological emergencies were reviewed. Data on success rate, periprocedural complications, timing of embolisation, requirement of blood transfusion and the long-term morphological and functional outcomes of embolised organs was recorded. Results Fourteen patients underwent endovascular control of bleeding as a result of trauma, iatrogenic injury and spontaneous perinephric haemorrhage during a period of 14 years. All these patients would have required emergency open surgery without the option of embolisation procedure. The mean time between the first presentation and embolisation was 22 hours (range 30 minutes to 60 hours. Mean pre-embolisation transfusion requirement was 6.8 units (range 0–22 units. None of the patients with successful embolisation required post-procedural blood transfusion. Permanent haemostasis was achieved in all but one patient, who required emergency nephrectomy. There were no serious procedure related post-embolisation complications. Conclusion Endovascular control using transarterial angioembolisation is an effective method for managing haematuria or haemorrhage in urological emergencies

  17. Intranasal topical estrogen in the management of epistaxis in hereditary hemorrhagic telangiectasia.

    Science.gov (United States)

    Minami, Kazuhiko; Haji, Tomoyuki

    2016-01-01

    Application of topical estriol ointment is an effective treatment for hereditary hemorrhagic telangiectasia (HHT) epistaxis. HHT is an autosomal-dominant disease characterized by epistaxis in more than 96% of patients. Management of this major symptom, epistaxis, has not been standardized. This study reports experience with topical application of estriol in patients with HHT. Five patients with a confirmed diagnosis of HHT who first visited the hospital between 2012 and 2013 received 0.1% estriol ointment and were guided to apply the ointment twice daily to the anterior part of both nasal cavities. Severity of epistaxis was valued using epistaxis severity score (ESS) before and 3 months after initiating therapy. Five patients (three males, two females) received treatment. After the initiation of treatment, intensity and frequency of epistaxis became moderate in all patients. ESS decreased significantly from pre- to post-treatment (p = 0.043). No adverse events were recorded during follow-up.

  18. Acute haemorrhagic oedema of infancy with bullae and koebnerisation

    OpenAIRE

    Mohd Sazlly Lim, S; Shamsudin, N

    2014-01-01

    A 5-month-old Malay boy presented with purpuric papules and plaques on the face and extremities accompanied by fever, coryzal symptoms and bilateral lower limb oedema. There were also bullous linear purpuric lesions on the right upper limb. Blood and culture tests were normal. Histopathological tests showed leucocytoclastic vasculitis, confirming the diagnosis of acute haemorrhagic oedema of infancy. The patient achieved complete recovery after 2 weeks with no recurrence.

  19. Acute haemorrhagic oedema of infancy with bullae and koebnerisation

    Directory of Open Access Journals (Sweden)

    Norashikin Shamsudin

    2014-08-01

    Full Text Available A 5-month-old Malay boy presented with purpuric papules and plaques on the face and extremities accompanied by fever, coryzal symptoms and bilateral lower limb oedema. There were also bullous linear purpuric lesions on the right upper limb. Blood and culture tests were normal. Histopathological tests showed leucocytoclastic vasculitis, confirming the diagnosis of acute haemorrhagic oedema of infancy. The patient achieved complete recovery after 2 weeks with no recurrence.

  20. [Carbetocin versus Oxytocin during caesarean section for preventing postpartum haemorrhage].

    Science.gov (United States)

    Pizzagalli, F; Agasse, J; Marpeau, L

    2015-05-01

    The aim of the study was to compare the effectiveness of Carbetocin versus Oxyotcin during caesarean section for preventing postpartum haemorrhage. Prospective observational study (before/after design). Five hundred and forty patients who received an injection of Oxytocin were compared to 262 patients with single injection of 100 micrograms of Carbetocin. The primary outcome was to compare the differential hematocrit level between pre- and postoperative blood samples. The secondary outcome was to compare differential hemoglobin level and the use of complementary therapies for postpartum haemorrhage. We did not find any difference between the Oxytocin and Carbetocin groups on differential hematocrit level. There was no difference between the groups regarding the use of additionnal therapies (Sulproston injections, blood transfusions and surgery methods). The rate of postpartum haemorrhage was similar in the two groups (18.7% vs 21.6%; P=0.33). We found a lower percentage of patients with differential of hemoglobin level between 2 g/dL and 4 g/dL in the Carbetocin group (6.5% vs 15.6%, Poxytocin. Carbetocin seems to reduce the need for postoperative intravenous iron injection. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

  1. The brain in hereditary hemorrhagic telangiectasia with pulmonary AVM: The clinical importance of paradoxical embolism

    International Nuclear Information System (INIS)

    Kim, W.S.; Kinnison, M.L.; Charnas, L.; Rosenbaum, A.E.; White, R.I. Jr.

    1986-01-01

    Despite the literature's emphasis on the presence of intracerebral vascular malformations in hereditary hemorrhagic telangiectasia (HHT), few cases have been documented. The authors prospectively evaluated brain CT scans in 29 consecutive patients with pulmonary arteriovenous malformations (AVMs) and known HHT. Fifty-five percent of the CT scans revealed the signs of embolic phenomena: infarctions (n=11) and intracerebral abscesses (n=5). In only one patient was a small enhancing vascular lesion seen. They conclude that embolic phenomena rather than vascular malformations cause the majority of abnormal CT findings in patients with HHT and pulmonary AVMs. Infarctions and intracerebral abscesses probably result from paradoxical embolization through the AVMs in the lungs. The therapeutic implications seem clear, since 55% of these patients are potentially treatable by balloon occlusion of the pulmonary AVMs

  2. Imaging study of lymphoreticular tumor development in ataxia-telangiectasia and Nijmegen breakage syndrome; Estudio por imagen del desarrollo de tumores linforreticulares en la ataxia telangiectasia y el sindrome de Nijmegen

    Energy Technology Data Exchange (ETDEWEB)

    Martinez-Leon, M. I.; Ceres-Ruiz, L.; Cuesta, M. A.; Garcia-Martin, F. J. [Hospital Materno-Infantil C.H.U. Carlos Haya. Malaga (Spain)

    2003-07-01

    Ataxia-telangiectasia (AT), or Louis-Bar syndrome, is an autosomal recessive illness characterized by progressive cerebellar ataxia, oculo-cutaneous telangiectasia, immunodeficiency combined with susceptibility to sinopulmonary infections and high incidence of neoplastic development. Nijmegen breakage syndrome (NBS) is a variant of AT, is also an autosomal recessive illness that presents cerebellar ataxia, as well as combined immunodeficiency and a tendency toward tumor development. Contrary to Louis-Bar syndrome, it doesn't present telangiectasia and exhibits a characteristics phenotype (short stature, bird-like face and microcephaly). Both entities are classified as syndrome of chromosomal instability or chromosomal fragility, a group which also includes Bloom syndrome and Fanconi anemia. All of these show an increase in the frequency of neoplastic pathologies, mainly lymphoid tumors. We present three patients,two with AT and one with NBS, who developed different lymphoma types in the course of the illness. We highlight the most outstanding aspects from a clinical-radiological point of view. (Author) 17 refs.

  3. The Development of Ataxia Telangiectasia Mutated Kinase Inhibitors

    Czech Academy of Sciences Publication Activity Database

    Andrs, M.; Kobarecny, J.; Nepovimova, E.; Jun, D.; Hodný, Zdeněk; Moravcová, Simona; Hanzlíková, Hana; Kuca, K.

    2014-01-01

    Roč. 14, č. 10 (2014), s. 805-811 ISSN 1389-5575 R&D Projects: GA MŠk(CZ) CZ.1.07/2.3.00/30.0044 Grant - others:MH CZ - DRO (University Hospital Hradec Kralove(CZ) 00179906 Institutional support: RVO:68378050 Keywords : Ataxia telangiectasia mutated * cancer * chemosensitization * DNA damage response Subject RIV: EB - Genetics ; Molecular Biology Impact factor: 2.903, year: 2014

  4. Hereditary hemorrhagic telangiectasia with bilateral pulmonary vascular malformations: A case report

    Directory of Open Access Journals (Sweden)

    Lončarević Olivera

    2016-01-01

    Full Text Available Introduction. Hereditary hemorrhagic telangiectasia (HHT also known as Osler-Weber-Rendu syndrome is an autosomal dominant disease that occurs due to vascular dysplasia associated with the disorder in the signaling pathway of transforming growth factor β (TGF-β. The clinical consequence is a disorder of blood vessels in multiple organ systems with the existence of telangiectasia which causes dilation of capillaries and veins, are present from birth and are localized on the skin and mucosa of the mouth, respiratory, gastrointestinal and urinary tract. They can make a rupture with consequent serious bleeding that can end up with fatal outcome. Since there is a disruption of blood vessels of more than one organic system, the diagnosis is very complex and requires a multidisciplinary approach. Case report. We reported a 40-year-old female patient with a long-time evolution of problems, who was diagnosed and treated at the Clinic for Lung Diseases of the Military Medical Academy in Belgrade, Serbia, because of bilaterally pulmonary arteriovenous malformations associated with HHT. Embolization was performed in two acts, followed with normalization of clinical, radiological and functional findings with the cessation of hemoptysis, effort intolerance with a significant improvement of the quality of life. Conclusion. HHT is a rare dominant inherited multisystem disease that requires multidisciplinary approach to diagnosis and treatment. Embolization is the method of choice in the treatment of arteriovenous malformations with minor adverse effects and very satisfying therapeutic effect.

  5. Therapeutic management of Crimean-Congo haemorrhagic fever.

    Science.gov (United States)

    de la Calle-Prieto, Fernando; Martín-Quirós, Alejandro; Trigo, Elena; Mora-Rillo, Marta; Arsuaga, Marta; Díaz-Menéndez, Marta; Arribas, José Ramón

    2017-06-29

    Crimean-Congo haemorrhagic fever has been reported in more than 30 countries in Africa, Asia, the Middle East and Eastern Europe, with an increasing incidence in recent years, especially in Europe. Because no specific treatments have demonstrated efficacy, supportive treatment is essential, as well as the provision of a centre with the appropriate means to guarantee the safety of its healthcare professionals. Laboratory monitoring of thrombocytopenia, severe coagulopathy or liver failure is of critical importance. Patients with Crimean-Congo haemorrhagic fever should be admitted to High Level Isolation Units where appropriate biocontainment procedures can prevent nosocomial transmission through infected fluids or accidents with contaminated material. In case of high-risk exposures, early administration of ribavirin should be considered. Copyright © 2017. Publicado por Elsevier España, S.L.U.

  6. Dengue Haemorrhagic Encephalitis: Rare Case Report with Review of Literature.

    Science.gov (United States)

    Kutiyal, Aditya Singh; Malik, Chetanya; Hyanki, Gitika

    2017-07-01

    Dengue is an endemic arboviral infection prevalent especially in tropical countries including Southern and Southeast Asia. Central Nervous System (CNS) involvement in dengue infection is uncommon. Haemorrhagic encephalitis is a rare presentation in dengue. This is a case of a 58-year-old male who presented with fever, petechial rash and altered sensorium. Dengue serology IgM was reactive and MRI brain was suggestive of haemorrhagic encephalitis. Patient was managed in Intensive Care Unit (ICU) but eventually succumbed to his illness. We report this fatal outcome of a common viral infection with unusual neurological presentation to propose an association between dengue and neurotropism and the need to look at dengue infection beyond its classical features.

  7. Nutritional management in Ebola haemorrhagic fever

    Directory of Open Access Journals (Sweden)

    Kamon Chaiyasit

    2015-06-01

    Full Text Available Ebola haemorrhagic fever is a viral infection causing a major health problem worldwide. In this short article, the authors briefly review and discuss on the nutritional management (energy, protein, fat and micronutrient in management of Ebola infection.

  8. Aetiology and treatment of severe postpartum haemorrhage

    DEFF Research Database (Denmark)

    Edwards, Hellen

    2017-01-01

    This thesis is comprised of three studies focusing on severe postpartum haemorrhage (PPH). PPH is a major cause of maternal morbidity and mortality worldwide. Risk factors include retained placenta, prolonged duration of the third stage of labour, previous caesarean section, and operative vaginal...... delivery. Occurrence and development of PPH are, however, unpredictable and can sometimes give rise to massive haemorrhage or even hysterectomy and maternal death. Severe haemorrhage can lead to coagulopathy causing further haemorrhage and requiring substitution with blood transfusions. The aim...... had a cardiac arrest, and a total of 128 women (52%) required a hysterectomy. Hysterectomy was associated with increased blood loss, increased number of blood transfusions, a higher fresh frozen plasma to red blood cell ratio (p=0.010), and an increased number of red blood cells before first platelet...

  9. Hypopituitarism is uncommon after aneurysmal subarachnoid haemorrhage

    DEFF Research Database (Denmark)

    Klose, Marianne; Brennum, Jannick; Poulsgaard, Lars

    2010-01-01

    Aneurysmal subarachnoid haemorrhage (SAH) has recently been reported as a common cause of chronic hypopituitarism, and introduction of routine neuroendocrine screening has been advocated. We aimed at estimating the risk of hypopituitarism after SAH using strict criteria including confirmatory...

  10. Treatment of nonneovascular idiopathic macular telangiectasia type 2 with intravitreal ranibizumab: results of a phase II clinical trial.

    Science.gov (United States)

    Toy, Brian C; Koo, Euna; Cukras, Catherine; Meyerle, Catherine B; Chew, Emily Y; Wong, Wai T

    2012-05-01

    To evaluate the safety and preliminary efficacy of intravitreal ranibizumab for nonneovascular idiopathic macular telangiectasia Type 2. Single-center, open-label Phase II clinical trial enrolling five participants with bilateral nonneovascular idiopathic macular telangiectasia Type 2. Intravitreal ranibizumab (0.5 mg) was administered every 4 weeks in the study eye for 12 months with the contralateral eye observed. Outcome measures included changes in best-corrected visual acuity, area of late-phase leakage on fluorescein angiography, and retinal thickness on optical coherence tomography. The study treatment was well tolerated and associated with few adverse events. Change in best-corrected visual acuity at 12 months was not significantly different between treated study eyes (0.0 ± 7.5 letters) and control fellow eyes (+2.2 ± 1.9 letters). However, decreases in the area of late-phase fluorescein angiography leakage (-33 ± 20% for study eyes, +1 ± 8% for fellow eyes) and in optical coherence tomography central subfield retinal thickness (-11.7 ± 7.0% for study eyes and -2.9 ± 3.5% for fellow eyes) were greater in study eyes compared with fellow eyes. Despite significant anatomical responses to treatment, functional improvement in visual acuity was not detected. Intravitreal ranibizumab administered monthly over a time course of 12 months is unlikely to provide a general and significant benefit to patients with nonneovascular idiopathic macular telangiectasia Type 2.

  11. European research priorities for intracerebral haemorrhage

    DEFF Research Database (Denmark)

    Steiner, Thorsten; Petersson, Jesper; Al-Shahi Salman, Rustam

    2011-01-01

    Over 2 million people are affected by intracerebral haemorrhage (ICH) worldwide every year, one third of them dying within 1 month, and many survivors being left with permanent disability. Unlike most other stroke types, the incidence, morbidity and mortality of ICH have not declined over time...... and disability. The European Research Network on Intracerebral Haemorrhage EURONICH is a multidisciplinary academic research collaboration that has been established to define current research priorities and to conduct large clinical studies on all aspects of ICH....

  12. Conservative management of primary postpartum haemorrhage

    International Nuclear Information System (INIS)

    Naqvi, S.; Makhdoom, T.

    2004-01-01

    Severe postpartum haemorrhage (PPH) is a serious obstetrical emergency. Two cases of severe PPH due to uterine atony are described which were managed by uterine packing with sterile ribbon gauze by vaginal route under general anesthesia. Along with supportive measures, it resulted in marked improvement in controlling haemorrhage and infectious morbidity. This is an effective treatment for severe PPH and should be practiced at tertiary care level in woman who wishes to preserve fertility. (author)

  13. Pregnancy and mesenchimal dysplasias (Marfan syndrome, Ehlers-Danlos syndrome, hereditary hemorrhagic telangiectasia).

    Science.gov (United States)

    Radetskaya, L S; Makatsariya, A D; Bitsadze, V O; Khizroeva, J K

    2018-07-01

    The objective of this article is to attract the attention of clinical physicians to the rare but extremely relevant clinical pathology of mesenchymal dysplasias (Marfan syndrome, Ehlers-Danlos syndrome, hereditary hemorrhagic telangiectasia) and especially specific characteristics of such diseases during pregnancy. Connective tissue pathology can cover different organs and systems, symptoms of the same disease can vary in different patients thus making diagnostics significantly difficult. Here clinical diagnostic criteria and methods of molecular diagnostics of diseases are described. The pathogenesis of mesenchymal dysplasias is not currently well understood. For the patients with mesenchymal dysplasias pregnancy is fraught with high risk of life-threatening complications. The preferred delivery method for such patients is caesarean section.

  14. Management of symptomatic thrombocytopenia associated with dengue haemorrhagic fever

    International Nuclear Information System (INIS)

    Jameel, T.; Saleem, I.U.; Mehmood, K.; Tanvir, I.; Saadia, A.

    2010-01-01

    Introduction: Immune - mediated destruction of platelets is thought to be the mechanism of thrombocytopenia seen after the viraemic phase of dengue haemorrhagic fever (DHF). Immuno - suppressants such as steroids, immune globulin and Anti D immune globulin are effective in the treatment of this type of immune thrombocytopenic purpura. Objective: To evaluate the efficacy of oral Prednisolone in the rate of resolution of thrombocytopenia and monitoring of complications in patients recovering from Dengue haemorrhagic fever. Method: A controlled study was carried out on diagnosed cases Dengue haemorrhagic patients presenting with sever thrombocytopenia and symptoms like confluent ecchymosis, epistaxis and purpuric rashes. In study was conducted in Ittefaq hospital (trust) Lahore, during the period of October to December 2008. Treatment group received steroids in two forms i.e. first line therapy prednisolone (1 mg / kg) orally or as second line therapy of initial I/V high dose (prednisolone) in pulse doses i.e. 40 mg / bd for four days and later oral prednisolone as in first line therapy with omeprazole 20 mg / bd in addition to standard treatment. Control group received standard supportive care only. Results: A total of 341 suspected patients were admitted in hospital. Serological diagnosis was confirmed in 166 patients. CBC revealed platelet count . 100 x 109 / l in 106 patients. A group of symptomatic febrile patients have platelet count < 20 x 109 / l was selected for therapeutic intervention. first line therapy (oral prednisolone was stated in 43 patients. In Fourteen patients second line therapy (high dose dexamethasone pulse) therapy was instituted. Seven of them attained complete response whereas two patients achieved partial response. Four patients were shifted to Anti D therapy. Three deaths occurred during our study. Rest of all the patients improved and were discharged in due course of time. Conclusion: This small scale preliminary study shows promising

  15. Haemorrhage in intracerebral arteriovenous malformations: detection with MRI and comparison with clinical history

    Energy Technology Data Exchange (ETDEWEB)

    Prayer, L. (Dept. of Radiology, and MR Inst., Univ. of Vienna (Austria)); Wimberger, D (Dept. of Radiology, and MR Inst., Univ. of Vienna (Austria) Dept. of Neurology, Univ. of Vienna (Austria)); Stiglbauer, R. (Dept. of Radiology, and MR Inst., Univ. of Vienna (Austria)); Kramer, J. (Dept. of Radiology, and MR Inst., Univ. of Vienna (Austria)); Richling, B. (Dept. of Neurosurgery, Univ. of Vienna (Austria)); Bavinzski, G. (Dept. of Neurosurgery, Univ. of Vienna (Austria)); Czech, T. (Dept. of Neurosurgery, Univ. of Vienna (Austria)); Imhof, H. (Dept. of Radiology, and MR Inst., Univ. of Vienna (Austria))

    1993-01-01

    Fifty-one patients with 59 angiographically proven cerebral arteriovenous malformations (AVMs) were examined by high-field MRI to detect blood breakdown products. Results were correlated with the history of intracranial bleeding. Evidence of previous episodes of haemorrhage was seen in 10 of 12 patients (83.3%) with verified bleeding, 4 of 9 patients (44.4%) with symptoms which could suggest bleeding and in 6 of 30 patients (20%) with negative histories. Because of the known rebleeding rate and the increased risk of associated complications, identification of the subgroup who had had haemorrhage and should therefore be considered for surgery may be beneficial. MRI can make a contribution to management by demonstrating prior haemorrhage in patients with an inadequate clinical history. (orig.)

  16. Post eclamptic aneurysmal rupture subarachnoid haemorrhage diagnosed in the puerperium

    International Nuclear Information System (INIS)

    Coolen, Teresa

    2006-01-01

    The incidence of subarachnoid and/or intracerebral haemorrhage in women during pregnancy is rare. The risk depends on the stage of pregnancy, but seems to be highest during the late third trimester, during delivery and in the puerperium. Headache can be a symptom of both preeclampsia, subarachnoid haemorrhage and other pathologies or conditions. It is essential for pregnant women with a suspected ruptured aneurysm to be investigated and treated without delay, irrespective of fear of harm to the foetus, to avoid complications from aneurysm rupture. This case study presents a 39-year-old woman who was 35 weeks and 3 days pregnant with known preeclampsia. She endured a headache for the three days leading up to the delivery with associated diplopia on the third day, but these symptoms were thought to be related to her preeclampsia. Over the three hours following childbirth, her headache became more severe and she suffered from vomiting, loss of vision, torticollis and seizures. Computed tomography (CT) of her head revealed a subarachnoid haemorrhage while CT angiography of the Circle of Willis failed to reveal an aneurysm and 4-vessel angiography only demonstrated an area slightly suspicious for the presence of an aneurysm. 3D rotational angiography clearly demonstrated a 1-2 mm aneurysm superior to the left terminal internal carotid artery. In this case, 3D rotational angiography proved to be a valuable additional technique. This patient underwent surgery for her ruptured aneurysm and has made an excellent recovery

  17. Immediate post-partum haemorrhage: Epidemiological aspects and maternal prognosis at South N’djamena District Hospital (Chad

    Directory of Open Access Journals (Sweden)

    Gabkika Bray Madoue

    2015-05-01

    Full Text Available Background: Post-partum haemorrhage defined as blood loss after delivery over 500mls, affects all countries and is the commonest cause of maternal mortality. It is a frequent obstetric emergency in developing countries. Objective: To identify the causes of post-partum haemorrhage and identify adequate management of immediate post-partum haemorrhage and thus reduce maternal mortality. Patients and methods: This was a prospective and descriptive study of one year from 1st January 2014 to 31stDecember 2014 conducted at South N’Djamena district hospital. Before including a patient in our survey her consent was obtained after explaining to her the need for the survey. All consenting patients with post-partum haemorrhage were included. Data were analyzed using SPSS17.0. Results: We recorded 100 cases of post-partum haemorrhage among 6815 deliveries giving an incidence of 1.47%. The average age of the women was 25.0 years. The majority of deliveries (90% were vaginal. The main cause of immediate post-partum haemorrhage was a third stage of labour bleeding (66% followed by genital lesions (32%. The management was medical (uterotonic drug, fluid replacement and blood transfusion, obstetric (manual removal of placenta or clot, and surgical (suture of lesions, vascular ligature and hysterectomy. There were two maternal deaths (2%. Conclusion: Post-partum haemorrhage is often fatal in our region. Preventive measures and efficient management can help to improve maternal prognosis.

  18. Allelic Dropout in the ENG Gene, Affecting the Results of Genetic Testing in Hereditary Hemorrhagic Telangiectasia

    DEFF Research Database (Denmark)

    Tørring, Pernille M; Kjeldsen, A.D.; Ousager, L.B.

    2012-01-01

    Background: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal-dominant vascular disorder with three disease-causing genes identified to date: ENG, ACVRL1, and SMAD4. We report an HHT patient with allelic dropout that on routine sequence analysis for a known mutation in the family (c.817......-3T>G in ENG) initially seemed to be homozygous for the mutation. Aim: To explore the possibility of allelic dropout causing a false result in this patient. Methods: Mutation analysis of additional family members was performed and haplotype analysis carried out. New primers were designed to reveal...... the presence of a possible sequence variant, which could explain the presumed allelic dropout. Results: Allelic dropout caused by a six-nucleotide duplication close to the standard reverse primer was the assumed cause of a false homozygous diagnosis. Conclusion: Sequence variants outside of the primer regions...

  19. [Mutations of ACVRL1 gene in a pedigree with hereditary hemorrhagic telangiectasia].

    Science.gov (United States)

    Luo, Jie-wei; Chen, Hui; Yang, Liu-qing; Zhu, Ai-lan; Wu, Yan-an; Li, Jian-wei

    2008-06-01

    To identify the activin A receptor type II-like 1 gene (ACVRL1) mutations in a Chinese family with hereditary hemorrhagic telangiectasia (HHT2). The exons 3, 7 and 8 of ACVRL1 gene of the proband and her five family members were amplified by polymerase chain reaction (PCR), and the PCR products were sequenced. The proband had obvious telangiectasis of gastric mucosa, and small arteriovenous fistula in the right kidney. All the patients in the HHT2 family had iterative epistaxis or bleeding in other sites, and had telangiectasis of nasal mucosa, tunica mucosa oris and finger tips. ACVRL1 gene analysis confirmed that there is frameshift mutation caused by deletion of G145 in exon 3 in the 4 patients, but the mutation is absent in 2 members without HHT2. The HHT2 family is caused by a 145delG mutation of ACVRL1 gene, resulting in frameshift and a new stop codon at codon 53.

  20. Frequency of causes of primary postpartum haemorrhage in a tertiary care hospital

    International Nuclear Information System (INIS)

    Munir, S.I.

    2015-01-01

    Objective: To study the frequency of causes of primary postpartum hemorrhage (PPH) in women managed in a tertiary care Hospital. material and Methods: The study was conducted in the department of Obstetrics and Gynecology, Unit - 1, Lady Willingdon Hospital King Edward Medical University, Lahore from July 2013 to December 2013. All the women having postpartum haemorrhage after vaginal delivery in the labour room or referred with primary postpartum haemorrhage were included in the study and were evaluated to see the frequency of postpartum haemorrhage as well as the causes of PPH in women being treated in a tertiary care unit. Results: During the study period 1344 women delivered in unit I and 250 patients developed postpartum haemorrhage giving frequency of primary postpartum haemorrhage 18.60%. Majority of the women 29.6% (n = 74) were between 26 - 30 years of age, mean and SD was 28.43 ± 4.76 years. The gestation of 55.2% (n = 138) patients was between 37 - 40 weeks. The frequency of postpartum haemorrhage in booked women during antenatal period was recorded as 25.2% (n = 63) while 74.8% (n = 187) were not booked in any health facility. Among patients who developed PPH, uterine atony was the most common cause 57.6% (n = 144), followed by genital tract teats which was 29.2% (n = 73). The rest of the causes of PPH were retained placenta in 10% (n = 25), uterine rupture in 3.6% (n = 9) and uterine inversion in 1.6% (n = 4). Conclusions: Postpartum haemorrhage is still a leading but preventable cause of maternal morbidity and mortality in our country due to under utilization of health facilities, the major cause is uterine atony followed by perineal tears. (author)

  1. Current diagnostic approaches to subarachnoid haemorrhage

    International Nuclear Information System (INIS)

    U-King-Im, Jean Marie; Koo, Brendan; Trivedi, Rikin A.; Higgins, Nicholas J.; Tay, Keng Y.; Cross, Justin J.; Antoun, Nagui M.; Gillard, Jonathan H.

    2005-01-01

    Over the past decade, significant advances have been made in the field of subarachnoid haemorrhage (SAH). Prompt diagnosis with high-resolution CT and intensive critical care support remain key aspects of good patient management. Early identification and definitive treatment of underlying ruptured aneurysms is generally advocated to reduce the risk of re-bleeding, a complication with high mortality and morbidity. Although intra-arterial digital subtraction angiography (DSA) is still considered the gold standard for sourcing aneurysms, CT angiography, especially with the evolution of multi-slice technology, is slowly gaining acceptance as a rapid, accessible and minimally invasive method which appears likely to replace DSA as first-line modality in the future. Furthermore, the advent of Guglielmi detachable coils and the ISAT trial have revolutionised the treatment of ruptured aneurysms, with a significant trend towards endovascular coiling away from operative clipping. Improvements in clinical experience, coiling technology and assistive devices now allow interventionalists to potentially treat the majority of aneurysms, including wide-necked or complex lesions. The uncertain long-term results of coiling, however, still fuel strong debate and controversy. This review summarises current diagnostic approaches to SAH from a radiological perspective, with an emphasis on aneurysmal SAH and an evidence-based approach to the role of imaging and interventional radiology in diagnosis, treatment and follow-up. (orig.)

  2. Current diagnostic approaches to subarachnoid haemorrhage

    Energy Technology Data Exchange (ETDEWEB)

    U-King-Im, Jean Marie; Koo, Brendan; Trivedi, Rikin A.; Higgins, Nicholas J.; Tay, Keng Y.; Cross, Justin J.; Antoun, Nagui M.; Gillard, Jonathan H. [Addenbrooke' s Hospital, University Department of Radiology, Cambridge (United Kingdom)

    2005-06-01

    Over the past decade, significant advances have been made in the field of subarachnoid haemorrhage (SAH). Prompt diagnosis with high-resolution CT and intensive critical care support remain key aspects of good patient management. Early identification and definitive treatment of underlying ruptured aneurysms is generally advocated to reduce the risk of re-bleeding, a complication with high mortality and morbidity. Although intra-arterial digital subtraction angiography (DSA) is still considered the gold standard for sourcing aneurysms, CT angiography, especially with the evolution of multi-slice technology, is slowly gaining acceptance as a rapid, accessible and minimally invasive method which appears likely to replace DSA as first-line modality in the future. Furthermore, the advent of Guglielmi detachable coils and the ISAT trial have revolutionised the treatment of ruptured aneurysms, with a significant trend towards endovascular coiling away from operative clipping. Improvements in clinical experience, coiling technology and assistive devices now allow interventionalists to potentially treat the majority of aneurysms, including wide-necked or complex lesions. The uncertain long-term results of coiling, however, still fuel strong debate and controversy. This review summarises current diagnostic approaches to SAH from a radiological perspective, with an emphasis on aneurysmal SAH and an evidence-based approach to the role of imaging and interventional radiology in diagnosis, treatment and follow-up. (orig.)

  3. Efficacy of Intravitreal Bevacizumab in Treatment of Proliferative Type 2 Idiopathic Juxtafoveal Telangiectasia

    Directory of Open Access Journals (Sweden)

    Ökkeş Baz

    2017-06-01

    Full Text Available Objectives: To evaluate the effectiveness of intravitreal bevacizumab (IVB in patients with subretinal neovascularization secondary to type 2 juxtafoveal telangiectasia. Materials and Methods: Ten eyes of 10 patients were included in this retrospective study. All cases were treated with IVB (1.25 mg bevacizumab. Visual acuity and slit-lamp anterior and posterior segment examinations were performed at each visit. Central macular thickness (CMT and intraretinal/subretinal fluid were evaluated via spectral domain optical coherence tomography (OCT. Loss of a line in visual acuity chart and presence of fluid on OCT were defined as criteria for repeated treatment. Results: The mean age of patients was 66.0±7.0 years (56-75. The mean follow-up time was 54.7±16.0 month (24-72. The mean BCVA was 0.62±0.35 (0.00-1.00 logMAR at baseline and 0.54±0.35 (0.00-1.00 logMAR at final exam (p=0.03. The mean CMT was 251±25.4 µm at baseline and 239±39.3 µm at final exam (p=0.01. Patients received an average of 1.7±1.0 IVB injections during follow-up. At baseline, all cases had intraretinal/subretinal fluid. There was no fluid at final examination of all cases. Conclusion: IVB treatment may be effective in the treatment of subretinal neovascularization secondary to type 2 juxtafoveal telangiectasia.

  4. Hereditary hemorrhagic telangiectasia and pregnancy: potential adverse events and pregnancy outcomes

    Directory of Open Access Journals (Sweden)

    Bari O

    2017-05-01

    Full Text Available Omar Bari,1 Philip R Cohen2 1School of Medicine, University of California San Diego, La Jolla, CA, USA; 2Department of Dermatology, University of California San Diego, La Jolla, CA, USA Abstract: Hereditary hemorrhagic telangiectasia (HHT is an autosomal dominant condition with a prevalence of ~1 in 5,000 individuals. The pathophysiology of this condition centers on the lack of capillary beds between arterioles and venules, leading to direct contact between these vessels. This results in telangiectases on characteristic locations such as the face, fingers, mouth, and nasal mucosa. Visceral arteriovenous malformations (AVMs are also observed in many patients, and these are most commonly seen in the brain, gastrointestinal tract, and lungs. Liver AVMs are present in many patients with HHT, though these individuals are usually asymptomatic; however, liver AVMs may lead to serious complications, such as high output cardiac failure. Diagnosis of HHT hinges upon fulfilling three out of four criteria: family history of the condition, mucocutaneous telangiectases, spontaneous and recurrent episodes of epistaxis, and visceral AVMs. Management is guided by international consensus guidelines and targets patients’ specific AVMs. Prognosis is good, though severe complications including hemorrhage and paradoxical emboli are possible. Novel therapeutics are being explored in clinical trials; bevacizumab and pazopanib inhibit angiogenesis, while thalidomide bolsters blood vessel maturation. Pregnancy in patients with HHT is considered high risk. While the majority of pregnancies proceed normally, severe complications have been reported in some women with HHT; these include heart failure, intracranial hemorrhage, pulmonary hemorrhage, and stroke. Such complications occur most often in the second and third trimesters when maternal changes such as peripheral vasodilation and increased cardiac output are at their maximum. Awareness of the diagnosis of HHT has

  5. Treatment of Hereditary Hemorrhagic Telangiectasia-Related Epistaxis.

    Science.gov (United States)

    Sautter, Nathan B; Smith, Timothy L

    2016-06-01

    Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease with an incidence of 1:5000. Recurrent, spontaneous epistaxis is the most common presenting symptom. Severity of epistaxis varies widely, from mild, self-limited nosebleeds to severe, life-threatening nasal hemorrhage. Treatment of HHT-related epistaxis presents a challenge to the otolaryngologist due to the recurrent, persistent nature of epistaxis often requiring multiple treatments. Treatment modalities range from conservative topical therapies to more aggressive surgical treatments. Copyright © 2016 Elsevier Inc. All rights reserved.

  6. Embolization for non-variceal upper gastrointestinal tract haemorrhage: A systematic review

    Energy Technology Data Exchange (ETDEWEB)

    Mirsadraee, S.; Tirukonda, P.; Nicholson, A. [Department of Radiology, Leeds General Infirmary, Leeds (United Kingdom); Everett, S.M. [Department of Gastroenterology, Leeds General Infirmary, Leeds (United Kingdom); McPherson, S.J., E-mail: simon.mcpherson@leedsth.nhs.u [Department of Radiology, Leeds General Infirmary, Leeds (United Kingdom)

    2011-06-15

    Aim: To assess the published evidence on the endovascular treatment of non-variceal upper gastrointestinal haemorrhage. Materials and methods: An Ovid Medline search of published literature was performed (1966-2009). Non-English literature, experimental studies, variceal haemorrhage and case series with fewer than five patients were excluded. The search yielded 1888 abstracts. Thirty-five articles were selected for final analysis. Results: The total number of pooled patients was 927. The technical and clinical success of embolization ranged from 52-100% and 44-100%, respectively. The pooled mean technical/clinical success rate in primary upper gastrointestinal tract haemorrhage (PUGITH) only, trans-papillary haemorrhage (TPH) only, and mixed studies were 84%/67%, 93%/89%, and 93%/64%, respectively. Clinical outcome was adversely affected by multi-organ failure, shock, corticosteroids, transfusion, and coagulopathy. The anatomical source of haemorrhage and procedural variables did not affect the outcome. A successful embolization improved survival by 13.3 times. Retrospective comparison with surgery demonstrated equivalent mortality and clinical success, despite embolization being applied to a more elderly population with a higher prevalence of co-morbidities. Conclusions: Embolization is effective in this very difficult cohort of patients with outcomes similar to surgery.

  7. Cellular and molecular response to irradiation in ataxia telangiectasia and in Fanconi's anemia

    International Nuclear Information System (INIS)

    Ridet, A.; Guillouf, C.; Duchaud, E.; Moustacchi, E.; Rosselli, F.

    1997-01-01

    Ataxia telangiectasia (AT) and Fanconi anemia (FA) are recessive genetic diseases featuring chromosomal instability, increased predisposition to cancer and in vitro hypersensitivity to ionizing radiation (AT) or DNA cross-linking agents (FA). Moreover, an in vivo hypersensitivity to γ-rays exposure was reported in both syndromes. Cellular response to irradiation includes growth arrest (cell cycle modification) and cell death (by apoptosis or necrosis). Since it is generally accepted that apoptosis modulates cellular sensitivity to genotoxic stress, it was of interest to investigate the contribution of apoptosis in determining FA and AT responses to DNA Damaging Agents. The results support the contention that the in vivo hypersensitivity to radiation in these syndromes is not related to a higher rate of apoptotic cells but could be to a higher necrotic response triggering inflammatory reactions in the patients affected by this syndromes. (authors)

  8. Cellular and molecular response to irradiation in ataxia telangiectasia and in Fanconi`s anemia

    Energy Technology Data Exchange (ETDEWEB)

    Ridet, A.; Guillouf, C.; Duchaud, E.; Moustacchi, E.; Rosselli, F. [Institut Curie-Recherche, UMR 218, CNRS, 75 - Paris (France)

    1997-03-01

    Ataxia telangiectasia (AT) and Fanconi anemia (FA) are recessive genetic diseases featuring chromosomal instability, increased predisposition to cancer and in vitro hypersensitivity to ionizing radiation (AT) or DNA cross-linking agents (FA). Moreover, an in vivo hypersensitivity to {gamma}-rays exposure was reported in both syndromes. Cellular response to irradiation includes growth arrest (cell cycle modification) and cell death (by apoptosis or necrosis). Since it is generally accepted that apoptosis modulates cellular sensitivity to genotoxic stress, it was of interest to investigate the contribution of apoptosis in determining FA and AT responses to DNA Damaging Agents. The results support the contention that the in vivo hypersensitivity to radiation in these syndromes is not related to a higher rate of apoptotic cells but could be to a higher necrotic response triggering inflammatory reactions in the patients affected by this syndromes. (authors)

  9. Spontaneous subarachnoid haemorrhage and outcome--results from Tan Tock Seng Hospital, Singapore.

    Science.gov (United States)

    Wong, S H; Yeo, T T; Seow, W T; Tan, K K; Ong, P L

    1999-07-01

    To ascertain the number of cases of spontaneous (aneurysmal) subarachnoid haemorrhage presenting to Tan Tock Seng Hospital, Singapore, over a one year period, the demographics of the patients involved, their treatment and their eventual outcome. A retrospective study from June 1995 to June 1996. There were 62 patients admitted over this period with an average of 5 patients per month. Their ages ranged from 9 to 85 years with a mean of 54 years. All 62 patients underwent 4-vessel cerebral angiograms. Forty-three patients (69%) underwent clipping of their aneurysms. Twelve patients (19%) had negative angiograms. Four patients (6%) underwent coiling of their aneurysms via interventional neuroradiology techniques. Patients with subarachnoid haemorrhage of Grades 1 to 3 on the WFNS (World Federation of Neurological Surgeons) grading had a favourable outcome (Glasgow Outcome Score of 4 and 5) in 85% of the cases. The overall mortality rate for the operated group (all grades) was 11%. However for the group with good WFNS grading, namely the Grade 1 to 2 groups, there were no deaths. Twenty-four percent of patients developed clinically symptomatic vasospasm. Eighteen percent of patients required ventriculo-peritoneal shunting for hydrocephalus secondary to the subarachnoid haemorrhage. The overall management mortality (operated and non-operated cases) was 14% for proven aneurysmal and angiographically-negative spontaneous subarachnoid haemorrhage. These results are comparable to that of other reputable centers reported in the literature.

  10. Mortality from trauma haemorrhage and opportunities for improvement in transfusion practice.

    Science.gov (United States)

    Stanworth, S J; Davenport, R; Curry, N; Seeney, F; Eaglestone, S; Edwards, A; Martin, K; Allard, S; Woodford, M; Lecky, F E; Brohi, K

    2016-03-01

    The aim of this study was to describe the prevalence, patterns of blood use and outcomes of major haemorrhage in trauma. This was a prospective observational study from 22 hospitals in the UK, including both major trauma centres and smaller trauma units. Eligible patients received at least 4 units of packed red blood cells (PRBCs) in the first 24 h of admission with activation of the massive haemorrhage protocol. Case notes, transfusion charts, blood bank records and copies of prescription/theatre charts were accessed and reviewed centrally. Study outcomes were: use of blood components, critical care during hospital stay, and mortality at 24 h, 30 days and 1 year. Data were used to estimate the national trauma haemorrhage incidence. A total of 442 patients were identified during a median enrolment interval of 20 (range 7-24) months. Based on this, the national incidence of trauma haemorrhage was estimated to be 83 per million. The median age of patients in the study cohort was 38 years and 73·8 per cent were men. The incidence of major haemorrhage increased markedly in patients aged over 65 years. Thirty-six deaths within 24 h of admission occurred within the first 3 h. At 24 h, 79 patients (17·9 per cent) had died, but mortality continued to rise even after discharge. Patients who received a cumulative ratio of fresh frozen plasma to PRBCs of at least 1 : 2 had lower rates of death than those who received a lower ratio. There were delays in administration of blood. Platelets and cryoprecipitate were either not given, or transfused well after initial resuscitation. There is a high burden of trauma haemorrhage that affects all age groups. Research is required to understand the reasons for death after the first 24 h and barriers to timely transfusion support. © 2016 BJS Society Ltd Published by John Wiley & Sons Ltd.

  11. Fulminant intravascular lymphomatosis mimicking acute haemorrhagic leukoencephalopathy.

    Science.gov (United States)

    Marino, D; Sicurelli, F; Cerase, A; Tripodi, S; Cintorino, M; Lazzi, S; Federico, A

    2012-09-15

    Intravascular lymphomatosis (IVL) is a rare non-Hodgkin's lymphoma, usually of B cell lineage, characterized by massive angiotropic growth. The clinical presentation of IVL may include changes in mental status, non-localizing neurological deficits, seizures, fever of unknown origin and skin changes. Because of its rarity and the absence of specific diagnostic procedures except for cerebral biopsy, diagnosis is often postmortem. Brain MRI usually shows non-specific abnormalities. The purpose of this case report is to increase the knowledge of clinical and neuroimaging features of IVL by describing the findings observed in a 71-year-old patient. A 71-year-old male was admitted for right hemiparesis, acute cognitive impairment and febricula. A bone marrow biopsy resulted normal. He then developed a rapid progressive impairment of his mental status and left hemisoma motor seizures. Brain CT and MRI were interpreted as consistent with acute haemorrhagic leukoencephalopathy (AHLE), including multiple areas of restricted diffusion without gadolinium enhancement and a small focal area of gadolinium enhancement in the left temporal lobe white matter. The patient died within a few days and the autopsy led to the diagnosis of IVL. IVL may present with a variety of clinical signs and symptoms, including stroke and hemiparesis. IVL may mimic AHLE at brain MRI. However, the evidence of multiple areas of restricted diffusion without gadolinium enhancement and of a small area of gadolinium enhancement could have led to the correct diagnosis. IVL should be added to the differential diagnosis of AHLE at brain MRI. Copyright © 2012 Elsevier B.V. All rights reserved.

  12. Atm reactivation reverses ataxia telangiectasia phenotypes in vivo.

    Science.gov (United States)

    Di Siena, Sara; Campolo, Federica; Gimmelli, Roberto; Di Pietro, Chiara; Marazziti, Daniela; Dolci, Susanna; Lenzi, Andrea; Nussenzweig, Andre; Pellegrini, Manuela

    2018-02-22

    Hereditary deficiencies in DNA damage signaling are invariably associated with cancer predisposition, immunodeficiency, radiation sensitivity, gonadal abnormalities, premature aging, and tissue degeneration. ATM kinase has been established as a central player in DNA double-strand break repair and its deficiency causes ataxia telangiectasia, a rare, multi-system disease with no cure. So ATM represents a highly attractive target for the development of novel types of gene therapy or transplantation strategies. Atm tamoxifen-inducible mouse models were generated to explore whether Atm reconstitution is able to restore Atm function in an Atm-deficient background. Body weight, immunodeficiency, spermatogenesis, and radioresistance were recovered in transgenic mice within 1 month from Atm induction. Notably, life span was doubled after Atm restoration, mice were protected from thymoma and no cerebellar defects were observed. Atm signaling was functional after DNA damage in vivo and in vitro. In summary, we propose a new Atm mouse model to investigate novel therapeutic strategies for ATM activation in ataxia telangiectasia disease.

  13. Defective fluid shear stress mechanotransduction mediates hereditary hemorrhagic telangiectasia

    Science.gov (United States)

    Baeyens, Nicolas; Larrivée, Bruno; Ola, Roxana; Hayward-Piatkowskyi, Brielle; Dubrac, Alexandre; Huang, Billy; Ross, Tyler D.; Coon, Brian G.; Min, Elizabeth; Tsarfati, Maya; Tong, Haibin; Eichmann, Anne

    2016-01-01

    Morphogenesis of the vascular system is strongly modulated by mechanical forces from blood flow. Hereditary hemorrhagic telangiectasia (HHT) is an inherited autosomal-dominant disease in which arteriovenous malformations and telangiectasias accumulate with age. Most cases are linked to heterozygous mutations in Alk1 or Endoglin, receptors for bone morphogenetic proteins (BMPs) 9 and 10. Evidence suggests that a second hit results in clonal expansion of endothelial cells to form lesions with poor mural cell coverage that spontaneously rupture and bleed. We now report that fluid shear stress potentiates BMPs to activate Alk1 signaling, which correlates with enhanced association of Alk1 and endoglin. Alk1 is required for BMP9 and flow responses, whereas endoglin is only required for enhancement by flow. This pathway mediates both inhibition of endothelial proliferation and recruitment of mural cells; thus, its loss blocks flow-induced vascular stabilization. Identification of Alk1 signaling as a convergence point for flow and soluble ligands provides a molecular mechanism for development of HHT lesions. PMID:27646277

  14. Intrapituitary fluid levels following haemorrhage: MRI appearances in 13 cases

    International Nuclear Information System (INIS)

    Lenthall, R.K.; Dean, J.R.; Jeffree, M.A.; Bartlett, J.R.

    1999-01-01

    Demonstration of fluid levels on MRI is well recognised in cerebral haematomas, tumours and cysts. The occurrence of fluid levels within haemorrhagic pituitary tumours has not previously been described in detail. Evidence of haemorrhage was identified in 27 of 125 pituitary tumours. Fluid levels occurred in 13 of these haemorrhagic tumours. No association with histological type was identified. Recognised risk factors for haemorrhage were identified in half of the cases. (orig.) (orig.)

  15. C-arm flat detector computed tomography parenchymal blood volume imaging: the nature of parenchymal blood volume parameter and the feasibility of parenchymal blood volume imaging in aneurysmal subarachnoid haemorrhage patients

    International Nuclear Information System (INIS)

    Kamran, Mudassar; Byrne, James V.

    2015-01-01

    C-arm flat detector computed tomography (FDCT) parenchymal blood volume (PBV) measurements allow assessment of cerebral haemodynamics in the neurointerventional suite. This paper explores the feasibility of C-arm computed tomography (CT) PBV imaging and the relationship between the C-arm CT PBV and the MR-PWI-derived cerebral blood volume (CBV) and cerebral blood flow (CBF) parameters in aneurysmal subarachnoid haemorrhage (SAH) patients developing delayed cerebral ischemia (DCI). Twenty-six patients with DCI following aneurysmal SAH underwent a research C-arm CT PBV scan using a biplane angiography system and contemporaneous MR-PWI scan as part of a prospective study. Quantitative whole-brain atlas-based volume-of-interest analysis in conjunction with Pearson correlation and Bland-Altman tests was performed to explore the agreement between C-arm CT PBV and MR-derived CBV and CBF measurements. All patients received medical management, while eight patients (31 %) underwent selective intra-arterial chemical angioplasty. Colour-coded C-arm CT PBV maps were 91 % sensitive and 100 % specific in detecting the perfusion abnormalities. C-arm CT rPBV demonstrated good agreement and strong correlation with both MR-rCBV and MR-rCBF measurements; the agreement and correlation were stronger for MR-rCBF relative to MR-rCBV and improved for C-arm CT PBV versus the geometric mean of MR-rCBV and MR-rCBF. Analysis of weighted means showed that the C-arm CT PBV has a preferential blood flow weighting (∼60 % blood flow and ∼40 % blood volume weighting). C-arm CT PBV imaging is feasible in DCI following aneurysmal SAH. PBV is a composite perfusion parameter incorporating both blood flow and blood volume weightings. That PBV has preferential (∼60 %) blood flow weighting is an important finding, which is of clinical significance when interpreting the C-arm CT PBV maps, particularly in the setting of acute brain ischemia. (orig.)

  16. C-arm flat detector computed tomography parenchymal blood volume imaging: the nature of parenchymal blood volume parameter and the feasibility of parenchymal blood volume imaging in aneurysmal subarachnoid haemorrhage patients

    Energy Technology Data Exchange (ETDEWEB)

    Kamran, Mudassar; Byrne, James V. [University of Oxford, Nuffield Department of Surgical Sciences, Oxford (United Kingdom)

    2015-09-15

    C-arm flat detector computed tomography (FDCT) parenchymal blood volume (PBV) measurements allow assessment of cerebral haemodynamics in the neurointerventional suite. This paper explores the feasibility of C-arm computed tomography (CT) PBV imaging and the relationship between the C-arm CT PBV and the MR-PWI-derived cerebral blood volume (CBV) and cerebral blood flow (CBF) parameters in aneurysmal subarachnoid haemorrhage (SAH) patients developing delayed cerebral ischemia (DCI). Twenty-six patients with DCI following aneurysmal SAH underwent a research C-arm CT PBV scan using a biplane angiography system and contemporaneous MR-PWI scan as part of a prospective study. Quantitative whole-brain atlas-based volume-of-interest analysis in conjunction with Pearson correlation and Bland-Altman tests was performed to explore the agreement between C-arm CT PBV and MR-derived CBV and CBF measurements. All patients received medical management, while eight patients (31 %) underwent selective intra-arterial chemical angioplasty. Colour-coded C-arm CT PBV maps were 91 % sensitive and 100 % specific in detecting the perfusion abnormalities. C-arm CT rPBV demonstrated good agreement and strong correlation with both MR-rCBV and MR-rCBF measurements; the agreement and correlation were stronger for MR-rCBF relative to MR-rCBV and improved for C-arm CT PBV versus the geometric mean of MR-rCBV and MR-rCBF. Analysis of weighted means showed that the C-arm CT PBV has a preferential blood flow weighting (∼60 % blood flow and ∼40 % blood volume weighting). C-arm CT PBV imaging is feasible in DCI following aneurysmal SAH. PBV is a composite perfusion parameter incorporating both blood flow and blood volume weightings. That PBV has preferential (∼60 %) blood flow weighting is an important finding, which is of clinical significance when interpreting the C-arm CT PBV maps, particularly in the setting of acute brain ischemia. (orig.)

  17. Post-transfusion hypertension, convulsion and intracranial haemorrhage in beta-thalassemia major

    International Nuclear Information System (INIS)

    Masood, S.A.; Zaidi, A.

    2012-01-01

    The haematologic disorder b-thalassemia major is common in Pakistan. We describe a patient with undiagnosed thalassemia presenting with hypertension and convulsions and found to have cerebral haemorrhage on neuro-imaging. He had been transfused 2 weeks before this illness. Our experience is similar to a few case reports described in literature that were found to have cerebral haemorrhages post-mortem after a similar clinical presentation. All patients had a blood transfusion within 2 weeks prior to the presentation so association with transfusion has been proposed. We have reviewed the several mechanisms presented and discussed the findings. (author)

  18. Improvement after treatment of hydrocephalus in aneurysmal subarachnoid haemorrhage : implications for grading and prognosis

    NARCIS (Netherlands)

    ter Laan, M; Mooij, JJA

    Two patients with aneurysmal subarachnoid haemorrhage and hydrocephalus are presented. On admission they scored E1M4V1 and E1M3Vtube on the Glasgow Coma Scale. The first patient recovered to E3M5Vtube after treatment of hydrocpehalus by extraventricular drainage. The second recovered to E2M5Vtube

  19. Combination therapy of hyaluronic acid mesotherapic injections and sclerotherapy for treatment of lower leg telangiectasia without major venous insufficiency: a preliminary clinical study.

    Science.gov (United States)

    Iannitti, Tommaso; Rottigni, Valentina; Torricelli, Federica; Palmieri, Beniamino

    2014-04-01

    Telangiectasia is the dilation of dermal capillaries mainly due to hypertension and vein insufficiency. Treatments of choice for this condition are sclerotherapy with foam liquid or intradermal fiber optic laser energy delivery. The aim of this study was to assess the efficacy of a new therapeutic approach consisting in the use of polymerized hyaluronic acid mesotherapic injections following sclerotherapy in the areas of the skin affected by telangiectasia in patients without major vein insufficiency. A total of 20 women, aged between 19 and 64 years, affected by recurrent lower leg telangiectasia, were included in this study. Patients were preliminarily submitted to echo color Doppler sonography to rule out severe saphenofemoral valve and lower limb major vein insufficiency. All patients underwent 3 sessions a month of polidocanol 1% capillary injections for 2 months. This was followed by 0.1 ml cross-linked hyaluronic acid introduction in the polidocanol 1% needle track. A total of 50 mesotherapic injections (0.05 ml each) were performed on the skin surface where an ice pack was previously applied for 4 to 5 minutes. A follow-up visit was performed at 3 months. The results, based on photographic examination, were rated as follows: poor improvement (0%-50%), good improvement (51%-75%), and very good improvement (76%-100%). The side effects of the clinical procedure, in terms of pain, itching, paresthesia, ecchymosis, and relapse of telangiectasia over the treated skin surface, as well as a persisting pigmentation in the injection spots and induced benefits related to leg heaviness and comfort, were recorded. In total, 6 patients displayed a slight venous insufficiency, 3 patients displayed patent venous insufficiency, and 11 patients did not show any venous insufficiency. Before treatment, itching was present in 18 out of 20 patients, paresthesia in 15 out of 20 patients, ecchymosis in 16 out of 20 patients, and leg heaviness in 15 out of 20 patients. At the 3

  20. The need for repeat angiography in subarachnoid haemorrhage

    International Nuclear Information System (INIS)

    Urbach, H.; Solymosi, L.; Zentner, J.

    1998-01-01

    This study was designed to assess the necessity for a second angiogram study in patients in whom initial angiography after primary subarachnoid haemorrhage (SAH) was negative. During a 12-year period, 122 of 694 patients (17.5 %) had negative initial angiograms. CT, available for 98 patients, showed a preponderance of subarachnoid blood in the perimesencephalic cisterns in 50 of 73 patients (68.5 %) in whom blood was visible on CT. Angiography, repeated in 67 patients, revealed an aneurysm in 4 (6 %): 2 had an aneurysm of the anterior communicating artery, 1 of the posterior inferior cerebellar artery, and 1 of the P2 segment of the posterior cerebral artery. CT showed subarachnoid blood in the interpeduncular and ambient cisterns in this last case, and a preponderance of subarachnoid blood outside the perimesencephalic cisterns in the remaining 3 patients. (orig.)

  1. The need for repeat angiography in subarachnoid haemorrhage

    Energy Technology Data Exchange (ETDEWEB)

    Urbach, H; Solymosi, L [Department of Neuroradiology, University of Bonn, Sigmund-Freud-Strasse 25, D-53127 Bonn (Germany); Zentner, J [Department of Neurosurgery, University of Bonn, Sigmund-Freud-Strasse 25, D-53127 Bonn (Germany)

    1998-01-01

    This study was designed to assess the necessity for a second angiogram study in patients in whom initial angiography after primary subarachnoid haemorrhage (SAH) was negative. During a 12-year period, 122 of 694 patients (17.5 %) had negative initial angiograms. CT, available for 98 patients, showed a preponderance of subarachnoid blood in the perimesencephalic cisterns in 50 of 73 patients (68.5 %) in whom blood was visible on CT. Angiography, repeated in 67 patients, revealed an aneurysm in 4 (6 %): 2 had an aneurysm of the anterior communicating artery, 1 of the posterior inferior cerebellar artery, and 1 of the P2 segment of the posterior cerebral artery. CT showed subarachnoid blood in the interpeduncular and ambient cisterns in this last case, and a preponderance of subarachnoid blood outside the perimesencephalic cisterns in the remaining 3 patients. (orig.) With 2 figs., 1 tab., 32 refs.

  2. Initial presentation of a giant gastrointestinal stromal tumour of the stomach with recurrent spontaneous intra-peritoneal haemorrhage

    Directory of Open Access Journals (Sweden)

    Margarida Vinagreiro

    2015-01-01

    Discussion and conclusion: GISTs are uncommon and rarely present with spontaneous intra-peritoneal haemorrhage, which may be life threatening. In our understanding, this is the first reported case of the reviewed literature presenting with a chronic hemoperitoneum, due to recurrent brisk episodes of tumour haemorrhage. Tumour rupture and large tumour size are two poor independent prognostic tumour factors for recurrence. Despite this, the patient remains free of disease after surgery and instituted adjuvant imatinib mesylate.

  3. Total Body Opacification 'Technique Neonatal Adrenal Haemorrhage

    African Journals Online (AJOL)

    1971-12-11

    Dec 11, 1971 ... A case is reported illustrating the possible usefulness of total body opacification in the diagnosis of neonatal adrenal haemorrhage. To derive maximum benefit from this principle, the routine use of an early film coupled with high dosage is urged whenever an intravenous pyelogram is performed for ...

  4. Cyclophosphamide induced Haemorrhagic Cystitis; a review of ...

    African Journals Online (AJOL)

    Cyclophosphamide is an akylating agent widely used in the management of both malignant and non neoplastic disorders. We undertook this review to assess the advancement in knowledge regarding the aetiopathogenesis and current management approaches of haemorrhagic cystitis resulting from the use of ...

  5. Sanitation of viral haemorrhagic septicaemia (VHS)

    DEFF Research Database (Denmark)

    Olesen, Niels Jørgen

    1998-01-01

    A sanitation programme for stamping-out viral haemorrhagic septicaemia (VHS) was implemented in Denmark in 1965. The programme has resulted in a dramatic reduction in the number of infected rainbow trout farms, from approximate to 400 to 26. The programme is carried out on a voluntary basis...

  6. The Prevalence of Intraventricular Haemorrhage and Associated ...

    African Journals Online (AJOL)

    The Prevalence of Intraventricular Haemorrhage and Associated Risk Factors in Preterm Neonates in the Neonatal Intensive Care Unit at the University Teaching ... any-IVH generated at the time of analysis was used in determining the prevalence of IVH and also as the dependent variable in multivariate logistic regression.

  7. Uterine artery embolization for the management of secondary postpartum haemorrhage associated with placenta accreta

    International Nuclear Information System (INIS)

    Li, X.; Wang, Z.; Chen, J.; Shi, H.; Zhang, X.; Pan, J.; Liu, W.; Yang, N.; Jin, Z.; Lang, J.

    2012-01-01

    Aim: To evaluate the efficacy and safety of uterine artery embolization for the management of secondary postpartum haemorrhage associated with placenta accreta. Materials and methods: Between January 2005 and August 2011, 45 women with placenta accreta, which was discovered during delivery, were managed conservatively in Peking Union Medical College Hospital. They did not experience severe bleeding during delivery. Ten patients (mean age 31 ± 6.4 years) developed secondary postpartum haemorrhage and underwent uterine artery embolization. The complications, control of haemorrhage, and outcome of the placenta left inside the uterus were retrospectively reviewed. Results: All patients underwent transcatheter embolization of bilateral uterine arteries. The median time between delivery and uterine artery embolization was 11 days (range 3–76 days). The technical success rate of embolization was 100%. Bleeding was controlled in all patients during follow-up (11 ± 6.9 months; range 3–24 months), and no further bleeding occurred. One patient developed lower-extremity deep venous thrombosis after uterine artery embolization, and no other major complications occurred. The placentae that were left inside the uteri gradually decreased in size during follow-up, except in one case. Nine patients resumed normal menstruation. One patient subsequently became pregnant and had an uneventful intrauterine pregnancy carried to term. Conclusion: Uterine artery embolization is safe and effective for the management of secondary postpartum haemorrhage associated with placenta accreta.

  8. A double-blind, randomized study comparing pure chromated glycerin with chromated glycerin with 1% lidocaine and epinephrine for sclerotherapy of telangiectasias and reticular veins.

    Science.gov (United States)

    Kern, Philippe; Ramelet, Albert-Adrien; Wutschert, Robert; Mazzolai, Lucia

    2011-11-01

    Chromated glycerin (CG) is an effective, although painful, sclerosing agent for telangiectasias and reticular leg veins treatment. To determine pain level and relative efficacy of pure or one-third lidocaine-epinephrine 1% mixed chromated glycerin in a prospective randomized double-blind trial. Patients presenting with telangiectasias and reticular leg veins on the lateral aspect of the thigh (C(1A) or (S) E(P) A(S) P(N1) ) were randomized to receive pure CG or CG mixed with one-third lidocaine-epinephrine 1% (CGX) treatment. Lower limb photographs were taken before and after treatment and analyzed by blinded expert reviewers for efficacy assessment (visual vein disappearance). Patients' pain and satisfaction were assessed using visual analogue scales. Data from 102 of 110 randomized patients could be evaluated. Patient pain scores were significantly higher when pure CG was used than with CGX (psclerotherapy pain without affecting efficacy when treating telangiectasias and reticular leg veins. © 2011 by the American Society for Dermatologic Surgery, Inc.

  9. Liquor circulation disturbance after subarachnoid haemorrhages - comparative pneumoencephalography and liquor scintigraphic investigations

    International Nuclear Information System (INIS)

    Menzel, J.; Georgi, P.; Krastel, A.; Deutsches Krebsforschungszentrum, Heidelberg

    1976-01-01

    Haemorrhages into the subarachnoid space often lead to instant blocking of the liquor circulation pathways with an acute increase of the intracranial pressure and acute venticular enlargement. These liquor circulation disturbances may be diagnosed by liquor scintiscanning as well as by pneumoencephalography. 165 patients were examined by both methods. The following results were obtained: liquor circulation disturbances after subarachnoid bleeding are frequent, they should be expected in 33% of all cases after spontaneous subarachnoid haemorrhages and in 68% of the cases after traumatic subarachnoid haemorrhages. The most severe form of liquor circulation distrubance may also be diagnosed by liquor scintiscanning as well as by pneumoencephalography. Liquor scintiscanning is the more exact method in cases with transitory ventricular reflux, while lumbar pneumoencephalography, in this series, is the method of choice when it comes to documenting the extent of the hydrocephalus. (GSE) [de

  10. Cutaneous granulomatosis and combined immunodeficiency revealing Ataxia-Telangiectasia: a case report

    OpenAIRE

    Folgori, Laura; Scarselli, Alessia; Angelino, Giulia; Ferrari, Francesca; Antoccia, Antonio; Chessa, Luciana; Finocchi, Andrea

    2010-01-01

    Abstract Ataxia-telangiectasia (A-T) is a complex multisystem disorder characterized by progressive neurological impairment, variable immunodeficiency and oculo-cutaneous telangiectasia. A-T is a member of chromosomal breakage syndromes and it is caused by a mutation in the ataxia-telangiectasia mutated (ATM) gene. Because of a wide clinical heterogeneity, A-T is often difficult to diagnose in children. We report an unusual case of a 3-year-old boy affected by A-T who presented exclusively wi...

  11. Severe postpartum haemorrhage from ruptured pseudoaneurysm: successful treatment with transcatheter arterial embolization

    Energy Technology Data Exchange (ETDEWEB)

    Soyer, Philippe; Fargeaudou, Yann; Boudiaf, Mourad; Le Dref, Olivier; Rymer, Roland [Hopital Lariboisiere-AP-HP Universite Paris 7, Department of Abdominal Imaging, Paris cedex 10 (France); Morel, Olivier [Hopital Lariboisiere-AP-HP Universite Paris 7, Department of Obstetrics, Paris cedex 10 (France)

    2008-06-15

    The purpose of this retrospective study was to evaluate the role of transcatheter arterial embolization in the management of severe postpartum haemorrhage due to a ruptured pseudoaneurysm and to analyse the clinical symptoms that may suggest a pseudoaneurysm as a cause of postpartum haemorrhage. A retrospective search of our database disclosed seven women with severe postpartum haemorrhage in whom angiography revealed the presence of a uterine or vaginal artery pseudoaneurysm and who were treated using transcatheter arterial embolization. Clinical files were reviewed for possible clinical findings that could suggest pseudoaneurysm as a cause of bleeding. Angiography revealed extravasation of contrast material in five out of seven patients. Transcatheter arterial embolization allowed to control the bleeding in all patients and subsequently achieve vaginal suture in four patients with vaginal laceration. No complications related to transcatheter arterial embolization were noted. Only two patients had uterine atony, and inefficiency of sulprostone was observed in all patients. Transcatheter arterial embolization is an effective and secure technique for the treatment of severe postpartum haemorrhage due to uterine or vaginal artery pseudoaneurysm. Ineffectiveness of suprostone and absence of uterine atony should raise the possibility of a ruptured pseudoaneurysm. (orig.)

  12. Severe postpartum haemorrhage from ruptured pseudoaneurysm: successful treatment with transcatheter arterial embolization

    International Nuclear Information System (INIS)

    Soyer, Philippe; Fargeaudou, Yann; Boudiaf, Mourad; Le Dref, Olivier; Rymer, Roland; Morel, Olivier

    2008-01-01

    The purpose of this retrospective study was to evaluate the role of transcatheter arterial embolization in the management of severe postpartum haemorrhage due to a ruptured pseudoaneurysm and to analyse the clinical symptoms that may suggest a pseudoaneurysm as a cause of postpartum haemorrhage. A retrospective search of our database disclosed seven women with severe postpartum haemorrhage in whom angiography revealed the presence of a uterine or vaginal artery pseudoaneurysm and who were treated using transcatheter arterial embolization. Clinical files were reviewed for possible clinical findings that could suggest pseudoaneurysm as a cause of bleeding. Angiography revealed extravasation of contrast material in five out of seven patients. Transcatheter arterial embolization allowed to control the bleeding in all patients and subsequently achieve vaginal suture in four patients with vaginal laceration. No complications related to transcatheter arterial embolization were noted. Only two patients had uterine atony, and inefficiency of sulprostone was observed in all patients. Transcatheter arterial embolization is an effective and secure technique for the treatment of severe postpartum haemorrhage due to uterine or vaginal artery pseudoaneurysm. Ineffectiveness of suprostone and absence of uterine atony should raise the possibility of a ruptured pseudoaneurysm. (orig.)

  13. Telangiectasia hemorrágica hereditária: ácido tranexâmico no tratamento de úlcera plantar Hereditary hemorrhagic telangiectasia: tranexamic acid for plantar ulcer

    Directory of Open Access Journals (Sweden)

    Gabriella Corrêa de Albuquerque

    2005-12-01

    Full Text Available Relato de um caso de úlcera plantar por fístula arteriovenosa em paciente portador de telangiectasia hemorrágica hereditária ou doença de Rendu-Osler-Weber tratado com ácido tranexâmico. Este fármaco é utilizado para tratamento de epistaxe, referindo-se o principal achado deste artigo ao uso eficaz desse medicamento na terapia de úlceras plantares hemorrágicas. São descritos os aspectos fisiopatológicos e clínicos da doença e as propriedades antifibrinolíticas do ácido tranexâmico. Este foi bem tolerado e apresentou evidências de eficácia na utilização para controle do sangramento e cicatrização da úlcera.Case report of one patient with Hereditary Hemorrhagic Telangiectasia, also known as Rendu-Osler-Weber syndrome, treated with Tranexamic Acid for arteriovenous plantar ulcer. This drug has proved effective in controlling epistaxis, but the main point of this report is to expose the success use of this medication in the therapy of skin bleeding ulcer. The pathophysiologic and clinical features of the disease are reviewed and also the pharmacological aspects of the antifibrinolytic drugs. This drug was well tolerated by the patient and show evidence of good activity in the bleeding and healed the ulcer.

  14. Treatment of epistaxis in hereditary hemorrhagic telangiectasia with tranexamic acid - a double-blind placebo-controlled cross-over phase IIIB study.

    Science.gov (United States)

    Geisthoff, Urban W; Seyfert, Ulrich T; Kübler, Marcus; Bieg, Birgitt; Plinkert, Peter K; König, Jochem

    2014-09-01

    Epistaxis is the most frequent manifestation in hereditary hemorrhagic telangiectasia, in which no optimal treatment exists. It can lead to severe anemia and reduced quality of life. Positive effects of tranexamic acid, an antifibrinolytic drug, have been reported on epistaxis related to this disorder. We sought to evaluate the efficacy of treating nosebleeds in hereditary hemorrhagic telangiectasia with tranexamic acid. In a randomized, double-blind, placebo controlled, cross-over phase IIIB study, 1 gram of tranexamic acid or placebo was given orally 3 times daily for 3 months for a total of 6 months. 22 patients were included in the intention-to-treat analysis. Hemoglobin levels, the primary outcome measure, did not change significantly (p=0.33). The secondary outcome measure was epistaxis score and patients reported a statistically significant reduction in nosebleeds, equaling a clinically relevant 54% diminution (p=0.0031), as compared to the placebo period. No severe side effects were observed. Tranexamic acid reduces epistaxis in patients with hereditary hemorrhagic telangiectasia. (Clinical trial registration numbers: BfArM 141 CHC 9008-001 and ClinicalTrials.gov NCT01031992). Copyright © 2014 Elsevier Ltd. All rights reserved.

  15. Prevention of postpartum haemorrhage with the oxytocin analogue carbetocin.

    Science.gov (United States)

    Rath, Werner

    2009-11-01

    better cardiovascular side effect profile compared with syntometrine. In addition to being an effective treatment for the prevention of postpartum haemorrhage following caesarean delivery, carbetocin may also become the drug of choice for postpartum haemorrhage prevention after vaginal delivery in high-risk women and those who suffer from hypertensive disorders in pregnancy. Preeclampsia is still a contraindication to the administration of carbetocin in the EU, and further studies would be required to assess the cardiovascular effects of carbetocin before it can be advocated for routine use in preeclamptic patients. Further research is required to assess whether prophylactic carbetocin is superior to conventional uterotonic agents following vaginal delivery in low-risk women.

  16. Addison's disease due to bilateral post-traumatic adrenal haemorrhage: CT and MR findings

    International Nuclear Information System (INIS)

    Wilms, G.; Tits, J.; Vanstraelen, D.; Marchal, G.; Rigauts, H.; Baert, A.L.

    1991-01-01

    In a patient with Addison's disease, who gave a history of a severe car accident, CT showed bilateral enlargement of the adrenal glands. MRI, by demonstrating paramagnetic T1 shortening due to methaemoglobin and haemosiderin on T2-weighted images, confirmed the haemorrhagic nature of the lesions. (orig.)

  17. Is bacterial colonisation of the tonsillar fossa a factor in post-tonsillectomy haemorrhage?

    NARCIS (Netherlands)

    Stephens, J. C.; Georgalas, C.; Kyi, M.; Ghufoor, K.

    2008-01-01

    OBJECTIVES: To identify if there is a link between bacterial colonisation of the tonsillar fossa and post-tonsillectomy haemorrhage. STUDY DESIGN AND SETTING: Prospective non-interventional study of 105 patients who underwent tonsillectomy during a seven-month period. The study took place in a

  18. Nasal closure for the treatment of epistaxis secondary to hereditary hemorrhagic telangiectasia.

    Science.gov (United States)

    Sena Esteves, Sara; Cardoso, Carla; Silva, Ana; Abrunhosa, José; Almeida E Sousa, Cecília

    Hereditary hemorrhagic telangiectasia (HHT), also known by the eponym Osler-Weber-Rendu syndrome, is an autosomal dominant disorder characterised by the presence of multiple arteriovenous malformations (AVMs) affecting multiple organs. Many procedures have been used for epistaxis control in patients with this disorder. The objective of this study was to report the treatment of severe HHT-related epistaxiswith the modified Young's procedure. We describe the treatment of 4 patients with severe blood-transfusion-dependent epistaxis who underwent a modified Young's procedure in a tertiary hospital. The nasal closure was bilateral and complete in all cases. All patients were followed for 12 months or longer. The procedure was well tolerated and complete cessation of bleeding was achieved in all the patients. Young's technique is a safe surgical procedure, well tolerated by patients with severe epistaxis and HHT. Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. All rights reserved.

  19. Terapia alternativa para microvarizes e telangiectasias com uso de agulha Alternative therapy for microvarices and telangiectasias with use of needle

    Directory of Open Access Journals (Sweden)

    Raimundo Rosendo de Oliveira

    2007-03-01

    Full Text Available CONTEXTO: O desenvolvimento de terapia alternativa à convencional para a destruição de microvarizes e telangiectasias sem o uso de produtos químicos tem como objetivo reduzir os efeitos colaterais, faz uso de agulha para lise mecânica dos vasos e tem como modelo experimental galinhas da linhagem Lohmann Brown. OBJETIVO: Elaborar uma nova técnica, desenvolvendo um tratamento alternativo, sem uso de produtos químicos, objetivando a redução dos efeitos colaterais. MÉTODOS: Foram utilizadas 30 galinhas da linhagem Lohmann Brown, sendo que 15 foram submetidas ao método convencional de tratamento de microvarizes e telangiectasias (grupo-controle e as outras 15 receberam o tratamento experimental proposto (grupo experimental. O grupo experimental foi tratado com agulha de lise vascular, percorrendo todo o trajeto dos vasos escolhidos em punções escalonadas até que todo o vaso ser atingido. O grupo-controle foi tratado com oleato de monoetanolamina e glicose a 50%, puncionando-se o vaso com agulha 13 x 3 mm e injetando-se, em média, 0,3 mL da solução em cada vaso. RESULTADOS: Dos 50 vasos tratados no grupo experimental, dois apresentaram recidiva total, cinco apresentaram recidiva parcial, e 43 apresentaram destruição (lise satisfatória; enquanto que, no grupo-controle, dos 51 vasos tratados, quatro apresentaram recidiva total, 12, recidiva parcial, 22, destruição satisfatória, e em 13 ocorreu endurecimento de trajeto. CONCLUSÃO: O presente estudo demonstrou que o método experimental proposto, com uso de agulha de lise vascular, possui mais eficiência no tratamento de microvarizes se comparado com o método convencional, devido à redução das recidivas e à ausência de hipercromia de trajeto endurecido.BACKGROUND: The development of an alternative to the conventional therapy for microvarices and telangiectasias without using chemical products aims at reducing side effects, using a needle for mechanical lysis of vessels. It

  20. CT and MR imaging of splenic leiomyoma in a child with ataxia telangiectasia

    Energy Technology Data Exchange (ETDEWEB)

    Coskun, M. [Dept. of Radiology, Hacettepe University Hospital, Ankara (Turkey); Aydingoez, Ue. [Dept. of Radiology, Hacettepe University Hospital, Ankara (Turkey); Tacal, T. [Dept. of Radiology, Hacettepe University Hospital, Ankara (Turkey); Ariyuerek, M. [Dept. of Radiology, Hacettepe University Hospital, Ankara (Turkey); Demirkazik, F. [Dept. of Radiology, Hacettepe University Hospital, Ankara (Turkey); Oguzkurt, L. [Dept. of Radiology, Hacettepe University Hospital, Ankara (Turkey)

    1995-02-01

    Computed tomographic and magnetic resonance imaging findings of a splenic leiomyoma in an 8-year-old boy with ataxia telangiectasia are presented. This is the first reported case of a splenic leiomyoma in the literature. (orig.)

  1. CT and MR imaging of splenic leiomyoma in a child with ataxia telangiectasia

    International Nuclear Information System (INIS)

    Coskun, M.; Aydingoez, Ue.; Tacal, T.; Ariyuerek, M.; Demirkazik, F.; Oguzkurt, L.

    1995-01-01

    Computed tomographic and magnetic resonance imaging findings of a splenic leiomyoma in an 8-year-old boy with ataxia telangiectasia are presented. This is the first reported case of a splenic leiomyoma in the literature. (orig.)

  2. Telangiectasia hemorrágica hereditária e malformações arteriovenosas pulmonares – Embolização com rolhão vascular Amplatzer

    Directory of Open Access Journals (Sweden)

    Cláudia Sofia Santos

    2009-03-01

    Full Text Available Resumo: As malformações arteriovenosas pulmonares (MAVP estão associadas a telangiectasia hemorrágica hereditária em cerca de 70% dos casos, podendo cursar com complicações neurológicas graves decorrentes do embolismo paradoxal potencial. A terapêutica das malformações é realizada no sentido de prevenir estas complicações.Descreve-se o caso clínico de uma doente com duas MAVP, no contexto de telangiectasia hemorrágica hereditária, submetida a embolização com o rolhão vascular Amplatzer. Este novo dispositivo é cada vez mais utilizado nestas situações, salientando-se algumas das suas características e vantagens em relação a outras técnicas de embolização mais frequentemente utilizadas. Abstract: Pulmonary arteriovenous malformations (PAVMs are associated to hereditary hemorrhagic telangiectasia in about 70% of the cases. PAVMs are associated to serious neurologic complications, secondary to inherent paradoxical embolisms. Treatment of the malformations is done to prevent these complications.The authors report a clinical case of a patient with two PAVMs, with hereditary hemorrhagic telangiectasia, who was treated by embolization with an Amplatzer vascular plug, a new device increasingly used in these situations. The authors emphasise some of its characteristics and advantages when compared with other embolization techniques most commonly used. Palavras-chave: Malformação arteriovenosa pulmonar, telangiectasia hemorrágica hereditária, rolhão vascular Amplatzer, Key-words: Pulmonary arteriovenous malformations, hereditary hemorrhagic telangiectasia, Amplatzer vascular plug

  3. Brain glucose metabolism in adults with ataxia-telangiectasia and their asymptomatic relatives.

    Science.gov (United States)

    Volkow, Nora D; Tomasi, Dardo; Wang, Gene-Jack; Studentsova, Yana; Margus, Brad; Crawford, Thomas O

    2014-06-01

    Ataxia-telangiectasia is a recessive genetic disorder (ATM is the mutated gene) of childhood with severe motor impairments and whereas homozygotes manifest the disorder, heterozygotes are asymptomatic. Structural brain imaging and post-mortem studies in individuals with ataxia-telangiectasia have reported cerebellar atrophy; but abnormalities of motor control characteristic of extrapyramidal dysfunction suggest impairment of broader motor networks. Here, we investigated possible dysfunction in other brain areas in individuals with ataxia-telangiectasia and tested for brain changes in asymptomatic relatives to assess if heterozygocity affects brain function. We used positron emission tomography and (18)F-fluorodeoxyglucose to measure brain glucose metabolism (quantified as µmol/100 g/min), which serves as a marker of brain function, in 10 adults with ataxia-telangiectasia, 19 non-affected adult relatives (12 siblings, seven parents) and 29 age-matched healthy controls. Statistical parametric mapping and region of interest analyses were used to compare individuals with ataxia-telangiectasia, asymptomatic relatives, and unrelated controls. We found that participants with ataxia-telangiectasia had lower metabolism in cerebellar hemispheres (14%, P brain stimulation. Our finding of decreased metabolism in vermis and hippocampus of asymptomatic relatives suggests that heterozygocity influences the function of these brain regions. Published by Oxford University Press on behalf of the Guarantors of Brain 2014. This work is written by US Government employees and is in the public domain in the US.

  4. A rare cause of fatal intracranial haemorrhage.

    LENUS (Irish Health Repository)

    Neligan, A

    2012-01-31

    INTRODUCTION: We report the case of a 53-year-old farmer with a 5-day history of severe headache, photophobia and neck stiffness. Full blood count (platelets 173), coagulation screen were normal throughout. Liver function tests remained normal apart from an elevated gamma-GT (156). CT Brain was normal. CSF analysis showed a WCC of 454\\/mm(3) (60% lymphocytes), elevated CSF protein (1.42 g\\/l) and a normal CSF glucose. He was commenced on IV antibiotics and IV acyclivor and improved. On day 3 of admission, he complained of a sudden severe headache, became unresponsive (GCS 3\\/15). INVESTIGATIONS: CT Brain showed a massive left intraventricular haemorrhage. He died 4 days later. Subsequent serum serology for leptospirosis was positive. A repeat sample taken 4 days post-admission, showed a rising IgM indicating active leptospirosis. Detailed pathological examination confirmed intracerebral haemorrhage with normal cerebral vasculature. CONCLUSION: Leptospirosis is a rare cause of intracerebral haemorrhage even in the absence of coagulopathy.

  5. Retinal haemorrhage in infants with pertussis.

    Science.gov (United States)

    Raoof, Naz; Pereira, Susana; Dai, Shuan; Neutze, Jocelyn; Grant, Cameron Charles; Kelly, Patrick

    2017-12-01

    It has been hypothesised that paroxysmal coughing in infantile pertussis (whooping cough) could produce retinal haemorrhages identical to those seen in abusive head trauma. We aimed to test this hypothesis. This is a prospective study of infants hospitalised with pertussis in Auckland, New Zealand, from 2009 to 2014. The clinical severity of pertussis was categorised. All infants recruited had retinal examination through dilated pupils by the paediatric ophthalmology service using an indirect ophthalmoscope. Forty-eight infants with pertussis, aged 3 weeks to 7 months, were examined after a mean of 18 days of coughing. Thirty-nine had severe pertussis and nine had mild pertussis. All had paroxysmal cough, and all were still coughing at the time of examination. No retinal haemorrhages were seen. We found no evidence to support the hypothesis that pertussis may cause the pattern of retinal haemorrhages seen in abusive head trauma in infants. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  6. THROMBOCYTOPENIA IN DENGUE HAEMORRHAGIC FEVER

    Directory of Open Access Journals (Sweden)

    I Wayan Putu Sutirta-Yasa

    2013-04-01

    Full Text Available The incidence and geographical distribution of dengue has gradually increased during the past decade. Today, dengue is considered one of the most important arthropod-borne viral diseasases in humans in term of morbidity and mortality. Dengue infection   a potential life-threatening dengue hemorrhagic fever (DHF / dengue shock syndrome(DSS, characterized by thrombocytopenia and increased vascular permiability. Thrombocytopenia causes bleeding, but in   DHF patients with thrombocytopenia do not always develop bleeding manifestation. The pathogenesis of thrombocytopenia are not cleared. Multiple factors  may be involved in the machanisms leading to thrombocytopenia in DHF/DSS patients.

  7. Nd:YAG laser hyaloidotomy for valsalva pre-macular haemorrhage.

    LENUS (Irish Health Repository)

    Kirwan, R P

    2012-02-01

    AIM: To report a case of successful drainage of a large pre-macular haemorrhage using laser photo-disruption of the posterior hyaloid membrane. MATERIALS AND METHODS: A case report. RESULTS: A 47-year-old man presented acutely to our emergency department complaining of a 24-h history of sudden onset, painless and persistent loss of vision in his left eye. Immediately before noticing this loss of vision, he had been vomiting violently from excessive alcohol intake. The left visual acuity was counting fingers. Dilated fundoscopy of the left eye revealed a large pre-macular haemorrhage which was 14 disc diametres in size. Clotting investigations were normal. A diagnosis of valsalva retinopathy was made and the patient elected to receive a prompt neodymium-doped yttrium aluminium garnet (Nd:YAG) laser posterior hyaloidotomy as an outpatient. At 1 week follow-up, the haemorrhage had drained completely into the vitreous space revealing a healthy macula and the visual acuity had improved to 6\\/12 unaided. At 6-month follow-up the left visual acuity stabilised at 6\\/9 unaided. CONCLUSION: Nd:YAG laser posterior hyaloidotomy is a useful outpatient procedure for successful clearance of large pre-macular haemorrhages that offers patients rapid recovery of visual acuity and the avoidance of more invasive intraocular surgery.

  8. The role of haemorrhage and exudate detection in automated grading of diabetic retinopathy.

    Science.gov (United States)

    Fleming, Alan D; Goatman, Keith A; Philip, Sam; Williams, Graeme J; Prescott, Gordon J; Scotland, Graham S; McNamee, Paul; Leese, Graham P; Wykes, William N; Sharp, Peter F; Olson, John A

    2010-06-01

    Automated grading has the potential to improve the efficiency of diabetic retinopathy screening services. While disease/no disease grading can be performed using only microaneurysm detection and image-quality assessment, automated recognition of other types of lesions may be advantageous. This study investigated whether inclusion of automated recognition of exudates and haemorrhages improves the detection of observable/referable diabetic retinopathy. Images from 1253 patients with observable/referable retinopathy and 6333 patients with non-referable retinopathy were obtained from three grading centres. All images were reference-graded, and automated disease/no disease assessments were made based on microaneurysm detection and combined microaneurysm, exudate and haemorrhage detection. Introduction of algorithms for exudates and haemorrhages resulted in a statistically significant increase in the sensitivity for detection of observable/referable retinopathy from 94.9% (95% CI 93.5 to 96.0) to 96.6% (95.4 to 97.4) without affecting manual grading workload. Automated detection of exudates and haemorrhages improved the detection of observable/referable retinopathy.

  9. Severe Crimean-Congo haemorrhagic fever presented with massive retroperitoneal haemorrhage that recovered without antiviral treatment

    DEFF Research Database (Denmark)

    Gharabaghi, Mehrnaz Asadi; Chinikar, Sadegh; Ghiasi, Seyyed Mojtaba

    2011-01-01

    Crimean-Congo haemorrhagic fever (CCHF) is a tickborne viral zoonosis with up to 50% mortality in humans caused by CCHF virus belonging to the genus Nairovirus, family Bunyaviridae. The geographical distribution of CCHF cases corresponds closely with the distribution of principle tick vectors...... that is species of Hyaloma. The disease presents with non-specific febrile symptoms, but progress to a serious haemorrhagic syndrome that, soon after, a full blown multi organ failure may develop with prominent features of liver damage and bleeding diathesis. The authors present a case of a 39-year-old man...

  10. Efficacy of b-lynch brace suture in postpartum haemorrhage

    International Nuclear Information System (INIS)

    Tarique, S.; Wazir, S.; Moeen, G.

    2011-01-01

    Massive uncontrolled haemorrhage after childbirth is the leading cause of maternal death in developing countries. Postpartum haemorrhage is traditionally defined as blood loss of more than 500 ml after vaginal delivery and more than 1000 ml after caesarean section, but intraoperative estimation of blood loss is inaccurate. Uterine atony alone accounts for 75 - 90% of PPH. To estimate the effectiveness and safety of B-Lynch brace Suture in the management of primary postpartum haemorrhage (PPH). (author)

  11. Adult adrenal haemorrhage: an unrecognised complication of renal vein thrombosis

    Energy Technology Data Exchange (ETDEWEB)

    Loke, T.K.L. E-mail: lokekl@ha.org.hk

    2001-07-01

    There are many predisposing factors for neonatal adrenal haemorrhage but the causative factors are different in adults. Several cases of neonatal adrenal haemorrhage have been reported in association with renal vein thrombosis. This complication has not been documented in the adults. The presence of an adrenal mass in the setting of renal vein thrombosis should raise the possibility of adrenal haemorrhage even though this is extremely uncommon in adults.

  12. Effectiveness of Per Rectal Misoprostol Versus Intramuscular Oxytocin for Prevention of Primary Postpartum Haemorrhage.

    Science.gov (United States)

    Asmat, Raheela; Ashraf, Tasneem; Asmat, Fazila; Asmat, Shakila; Asmat, Nagina

    2017-01-01

    To compare the effectiveness of per rectal misoprostol over oxytocin in primary postpartum haemorrhage (PPH). Randomised controlled trial study. Gynaecology and Obstetrics Department, Unit IV, Bolan Medical Complex Hospital, Quetta, from September 2013 to February 2014. Emergency obstetric patients receiving per rectal misoprostol (800 µgm) were named as group 'A' and those receiving 10 units oxytocin intramuscularly were labelled as group 'B'. The patients were followed within 24 hours of spontaneous vaginal deliveries. Pads soaked were used to assess the amount of blood loss. A total of 1,678 patients were included in the study. The mean age of patients in group-A was 29.11 years while the mean age of patients in group-B was 29.16 years. One hundred and twenty-three (14.66%) patients in group-A and 120 (14.31%) patients in group-B had PPH. Among the total 1,678 patients, 243 (14.49%) had postpartum haemorrhage among whom 24 (9.88%) had major haemorrhage with a blood loss ≥1000 mL. Among the sub-group (839 patients) administered misoprostol had 123 (14.66%) patients with blood loss greater than 500 mL and the rest 716 patients (85.34%) had blood loss less than 500 mL. The sub-group administered oxytocin have 120 (14.31%) out of 839 patients with postpartum haemorrhage while 719 (85.69%) had blood loss less than 500 mL. Active management of 3rd stage of labour with per rectal misoprostol administration was as effective as intramuscular oxytocin. Both were equally effective to reduce PPH and the subsequent need for surgical interventions.

  13. Prospective randomized trial of sclerotherapy vs standard treatment for epistaxis due to hereditary hemorrhagic telangiectasia.

    Science.gov (United States)

    Boyer, Holly; Fernandes, Patricia; Le, Chap; Yueh, Bevan

    2015-05-01

    Our previous studies have demonstrated the tolerability and low side-effect profile of office-based sclerotherapy with sodium tetradecyl sulfate (STS) for treating recurrent epistaxis due to hereditary hemorrhagic telangiectasia (HHT). The objective of this study was to use a prospective randomized trial to determine the effectiveness of sclerotherapy with STS vs standard treatment. This prospective randomized trial (conducted from November 1, 2011, through January 31, 2014) involved 17 patients with recurrent epistaxis due to HHT. We defined standard treatment as continuation of any treatment that the patient had previously undergone, such as moisturization, packing, and cautery. We used a crossover design, so study participants were randomized to either sclerotherapy or standard treatment during the first time period, and then to the other during the second period. The primary outcome measure was frequency and severity of epistaxis, as measured by the epistaxis severity score (ESS). The ESS is a 10-point scale, with higher scores corresponding to more bleeding. After controlling for treatment order, bleeding was substantially better controlled after sclerotherapy; the ESS after sclerotherapy was nearly one point lower than after standard treatment (-0.95, 1-sided p = 0.027). Treatment order, baseline ESS, the number of lesions, moisturization practices, and a history of previous blood transfusions did not significantly affect the results. This trial demonstrated that sclerotherapy with STS (vs standard treatment) significantly reduced epistaxis due to HHT. © 2015 ARS-AAOA, LLC.

  14. The effect of bevacizumab (Avastin) treatment on epistaxis in hereditary hemorrhagic telangiectasia.

    Science.gov (United States)

    Simonds, Jana; Miller, Frank; Mandel, Jess; Davidson, Terence M

    2009-05-01

    Determine the effectiveness of treating epistaxis in hereditary hemorrhagic telangiectasia (HHT) with potassium titanyl phosphate (KTP) laser cautery combined with submucosal injection of 100 mg of bevacizumab. Retrospective pilot study. Bevacizumab was injected throughout the nasal cavity following KTP laser treatment in 10 patients (bevacizumab/KTP group) and compared to nine patients previously treated with KTP laser alone (KTP group). Epistaxis frequency and severity, blood transfusion requirement, intravenous iron supplementation, emergency department visit frequency, and quality of life within 1 month and 1 year pre- and postsurgery were analyzed. Benefit was defined as less than three nosebleeds per week, with less than 10 minutes to stop each nosebleed, and no blood transfusions. The pre- and postsurgery data were analyzed within and between the two groups. The groups were comparable in age and gender. Significant benefit was found in frequency of epistaxis (P epistaxis is superior to KTP laser treatment alone. It significantly decreases frequency and severity of nosebleeds and blood transfusion requirements, and significantly improves work ability and quality of life.

  15. The ATM gene and the radiobiology of ataxia-telangiectasia

    International Nuclear Information System (INIS)

    Jorgensen, T.J.; Shiloh, Y.

    1996-01-01

    Ataxia-telangiectasia (A-T) is the classic human genetic disease involving severe ionizing radiation sensitivity and as such has been intensely studied by radiation biologists over the years. Unlike its counterpart for UV light sensitivity -xeroderma pigmentosum - A-T has no obvious DNA repair defect; and there has been much speculation as to the mechanism underlying the altered radioresponses associated with this disease. The gene defective in A-T (ATM) has recently been cloned, and its primary coding sequence determined. The primary sequence of the ATM protein suggests that it has some regulatory functions related to cellular radioresponse and maintenance of genomic stability, and shares these functions with a growing family of other proteins in various organisms. At this juncture it is appropriate to review our current knowledge about the radiobiology of A-T and reflect on the possible radiobiological mechanisms that are suggested by the ATM gene itself. This article will attempt briefly to review current knowledge about the radiobiology of A-T and to introduce new speculations about underlying radiobiological mechanisms that are suggested by the primary amino acid sequence of the predicted ATM gene product. (Author)

  16. Automatic telangiectasia analysis in dermoscopy images using adaptive critic design.

    Science.gov (United States)

    Cheng, B; Stanley, R J; Stoecker, W V; Hinton, K

    2012-11-01

    Telangiectasia, tiny skin vessels, are important dermoscopy structures used to discriminate basal cell carcinoma (BCC) from benign skin lesions. This research builds off of previously developed image analysis techniques to identify vessels automatically to discriminate benign lesions from BCCs. A biologically inspired reinforcement learning approach is investigated in an adaptive critic design framework to apply action-dependent heuristic dynamic programming (ADHDP) for discrimination based on computed features using different skin lesion contrast variations to promote the discrimination process. Lesion discrimination results for ADHDP are compared with multilayer perception backpropagation artificial neural networks. This study uses a data set of 498 dermoscopy skin lesion images of 263 BCCs and 226 competitive benign images as the input sets. This data set is extended from previous research [Cheng et al., Skin Research and Technology, 2011, 17: 278]. Experimental results yielded a diagnostic accuracy as high as 84.6% using the ADHDP approach, providing an 8.03% improvement over a standard multilayer perception method. We have chosen BCC detection rather than vessel detection as the endpoint. Although vessel detection is inherently easier, BCC detection has potential direct clinical applications. Small BCCs are detectable early by dermoscopy and potentially detectable by the automated methods described in this research. © 2011 John Wiley & Sons A/S.

  17. Type I IFN-related NETosis in ataxia telangiectasia and Artemis deficiency.

    Science.gov (United States)

    Gul, Ersin; Sayar, Esra Hazar; Gungor, Bilgi; Eroglu, Fehime Kara; Surucu, Naz; Keles, Sevgi; Guner, Sukru Nail; Findik, Siddika; Alpdundar, Esin; Ayanoglu, Ihsan Cihan; Kayaoglu, Basak; Geckin, Busra Nur; Sanli, Hatice Asena; Kahraman, Tamer; Yakicier, Cengiz; Muftuoglu, Meltem; Oguz, Berna; Cagdas Ayvaz, Deniz Nazire; Gursel, Ihsan; Ozen, Seza; Reisli, Ismail; Gursel, Mayda

    2017-11-16

    Pathological inflammatory syndromes of unknown etiology are commonly observed in ataxia telangiectasia (AT) and Artemis deficiency. Similar inflammatory manifestations also exist in patients with STING-associated vasculopathy in infancy (SAVI). We sought to test the hypothesis that the inflammation-associated manifestations observed in patients with AT and Artemis deficiency stem from increased type I IFN signature leading to neutrophil-mediated pathological damage. Cytokine/protein signatures were determined by ELISA, cytometric bead array, or quantitative PCR. Stat1 phosphorylation levels were determined by flow cytometry. DNA species accumulating in the cytosol of patients' cells were quantified microscopically and flow cytometrically. Propensity of isolated polymorhonuclear granulocytes to form neutrophil extracellular traps (NETs) was determined using fluorescence microscopy and picogreen assay. Neutrophil reactive oxygen species levels and mitochondrial stress were assayed using fluorogenic probes, microscopy, and flow cytometry. Type I and III IFN signatures were elevated in plasma and peripheral blood cells of patients with AT, Artemis deficiency, and SAVI. Chronic IFN production stemmed from the accumulation of DNA in the cytoplasm of AT and Artemis-deficient cells. Neutrophils isolated from patients spontaneously produced NETs and displayed indicators of oxidative and mitochondrial stress, supportive of their NETotic tendencies. A similar phenomenon was also observed in neutrophils from healthy controls exposed to patient plasma samples or exogeneous IFN-α. Type I IFN-mediated neutrophil activation and NET formation may contribute to inflammatory manifestations observed in patients with AT, Artemis deficiency, and SAVI. Thus, neutrophils represent a promising target to manage inflammatory syndromes in diseases with active type I IFN signature. Copyright © 2017 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights

  18. Computational Intelligence Method for Early Diagnosis Dengue Haemorrhagic Fever Using Fuzzy on Mobile Device

    Directory of Open Access Journals (Sweden)

    Salman Afan

    2014-03-01

    Full Text Available Mortality from Dengue Haemorrhagic Fever (DHF is still increasing in Indonesia particularly in Jakarta. Diagnosis of the dengue shall be made as early as possible so that first aid can be given in expectation of decreasing death risk. The Study will be conducted by developing expert system based on Computational Intelligence Method. On the first year, study will use the Fuzzy Inference System (FIS Method to diagnose Dengue Haemorrhagic Fever particularly in Mobile Device consist of smart phone. Expert system application which particularly using fuzzy system can be applied in mobile device and it is useful to make early diagnosis of Dengue Haemorrhagic Fever that produce outcome faster than laboratory test. The evaluation of this application is conducted by performing accuracy test before and after validation using data of patient who has the Dengue Haemorrhagic Fever. This expert system application is easy, convenient, and practical to use, also capable of making the early diagnosis of Dengue Haemorraghic to avoid mortality in the first stage.

  19. Early cryoprecipitate for major haemorrhage in trauma: a randomised controlled feasibility trial.

    Science.gov (United States)

    Curry, N; Rourke, C; Davenport, R; Beer, S; Pankhurst, L; Deary, A; Thomas, H; Llewelyn, C; Green, L; Doughty, H; Nordmann, G; Brohi, K; Stanworth, S

    2015-07-01

    Low fibrinogen (Fg) concentrations in trauma haemorrhage are associated with poorer outcomes. Cryoprecipitate is the standard source for Fg administration in the UK and USA and is often given in the later stages of transfusion therapy. It is not known whether early cryoprecipitate therapy improves clinical outcomes. The primary aim of this feasibility study was to determine whether it was possible to administer cryoprecipitate, within 90 min of admission to hospital. Secondary aims were to evaluate laboratory measures of Fg and clinical outcomes including thrombotic events, organ failure, length of hospital stay and mortality. This was an unblinded RCT, conducted at two civilian UK major trauma centres of adult trauma patients (age ≥16 yrs), with active bleeding and requiring activation of the major haemorrhage protocol. Participants were randomised to standard major haemorrhage therapy (STANDARD) (n=22), or to standard haemorrhage therapy plus two early pools of cryoprecipitate (CRYO) (n=21). 85% (95% CI: 69-100%) CRYO participants received cryoprecipitate within 90 min, median time 60 min (IQR: 57-76) compared with 108 min (67-147), CRYO and STANDARD arms respectively (P=0.002). Fg concentrations were higher in the CRYO arm and were maintained above 1.8 g litre(-1) at all time-points during active haemorrhage. All-cause mortality at 28 days was not significantly different (P=0.14). Early Fg supplementation using cryoprecipitate is feasible in trauma patients. This study supports the need for a definitive RCT to determine the effect of early Fg supplementation on mortality and other clinical outcomes. ISRCTN55509212. © The Author 2015. Published by Oxford University Press on behalf of the British Journal of Anaesthesia. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  20. Dengue Haemorrhagic Fever presenting as Acute Abdomen

    OpenAIRE

    Al-Araimi, Hanaa; Al-Jabri, Amal; Mehmoud, Arshad; Al-Abri, Seif

    2011-01-01

    We describe a case of a 38 year-old Sri Lankan female who was referred to the surgeon on call with a picture of acute abdomen. She presented with a three-day history of fever, headache, abdominal pain and diarrhoea; however, the physical examination was not consistent with acute abdomen. Her platelet count was 22 ×109/L. A diagnosis of dengue haemorrhagic fever (DHF) was made and dengue serology was positive. Dengue epidemics have been associated with a variety of gastrointestinal symptoms an...

  1. Haemorrhagic SLE In A Young Male

    Directory of Open Access Journals (Sweden)

    Rajagopal R

    2002-01-01

    Full Text Available Systemic lupus erythematous (SLE is a systemic autoimmune disease that tends to occur in early adult life. The peak age of onset of the first symptom or sign in females is about 38 years and later in men, at about 44 years. Females outnumber men in this illness in a ratio of about 8 : 1. Cutaneous lesions in male have not been properly investigated and some studies in male with SLE have shown that the illness may present with atypical skin lesions. A case of SLE in a 20 year male who developed sudden onset of haemorrhagic vesiculobullous butterfly rash is described.

  2. Bipallidal haemorrhage after ethylene glycol intoxication

    Energy Technology Data Exchange (ETDEWEB)

    Caparros-Lefebvre, D.; Policard, J.; Rigal, M. [CHU Pointe a Pitre, Service de Neurologie, Lille (France); Sengler, C. [CHU Pointe a Pitre, Laboratoire de Pharmaco-Toxicologie, Guadeloupe (France); Benabdallah, E. [CHU Pointe a Pitre, Service de Radiologie, Guadeloupe (France); Colombani, S. [Centre d' Imagerie medicale, Martinique (France)

    2005-02-01

    Acute or subacute bipallidal lesion, an uncommon radiological feature produced by metabolic disorders or poisoning, has never been attributed to ethylene glycol (EG) intoxication. This 50-year-old Afro-Caribbean alcoholic man had unexplained loss of consciousness. Blood tests showed osmolar gap. Drug screening was positive for EG at 6.06 mmol/l. Brain CT revealed bilateral pallidal haemorrhage. Pallidal haematoma, which could be related to deposition of oxalate crystals issued from EG metabolism, should lead to toxicological screening. (orig.)

  3. Bipallidal haemorrhage after ethylene glycol intoxication

    International Nuclear Information System (INIS)

    Caparros-Lefebvre, D.; Policard, J.; Rigal, M.; Sengler, C.; Benabdallah, E.; Colombani, S.

    2005-01-01

    Acute or subacute bipallidal lesion, an uncommon radiological feature produced by metabolic disorders or poisoning, has never been attributed to ethylene glycol (EG) intoxication. This 50-year-old Afro-Caribbean alcoholic man had unexplained loss of consciousness. Blood tests showed osmolar gap. Drug screening was positive for EG at 6.06 mmol/l. Brain CT revealed bilateral pallidal haemorrhage. Pallidal haematoma, which could be related to deposition of oxalate crystals issued from EG metabolism, should lead to toxicological screening. (orig.)

  4. European research priorities for intracerebral haemorrhage

    DEFF Research Database (Denmark)

    Steiner, Thorsten; Petersson, Jesper; Al-Shahi Salman, Rustam

    2011-01-01

    and disability. The European Research Network on Intracerebral Haemorrhage EURONICH is a multidisciplinary academic research collaboration that has been established to define current research priorities and to conduct large clinical studies on all aspects of ICH........ No standardised diagnostic workup for the detection of the various underlying causes of ICH currently exists, and the evidence for medical or surgical therapeutic interventions remains limited. A dedicated European research programme for ICH is needed to identify ways to reduce the burden of ICH-related death...

  5. A rare case of Weil's disease with alveolar haemorrhage

    Directory of Open Access Journals (Sweden)

    Abhiram Chakrabarti

    2014-05-01

    Full Text Available Leptospirosis, a disease of protean manifestations occurs sporadically throughout the year with a peak seasonal incidence during the rainy season mimicking other febrile viral illness. In the rare case, the disease leads to renal and hepatic involvement with hemorrhage which may be associated with multisystem organ dysfunction in form of pulmonary, cardiac and central nervous system, when it is known as Weil's disease. Rarely haemorrhagic manifestations are assosciated. Early diagnosis is important as sometimes the disease may be life threatening. Proper antibiotics results in dramatic improvement. We hereby presented a case that had clinical features of Weil's disease with cough, dyspnoea and haemoptysis. Leptospirosis was detected on ELISA testing. Patient was cured rapidly with antibiotics.

  6. A rare case of Weil's disease with alveolar haemorrhage.

    Science.gov (United States)

    Chakrabarti, Abhiram; Nandy, Manab; Pal, Dipankar; Mallik, Sudesna

    2014-05-01

    Leptospirosis, a disease of protean manifestations occurs sporadically throughout the year with a peak seasonal incidence during the rainy season mimicking other febrile viral illness. In the rare case, the disease leads to renal and hepatic involvement with hemorrhage which may be associated with multisystem organ dysfunction in form of pulmonary, cardiac and central nervous system, when it is known as Weil's disease. Rarely haemorrhagic manifestations are assosciated. Early diagnosis is important as sometimes the disease may be life threatening. Proper antibiotics results in dramatic improvement. We hereby presented a case that had clinical features of Weil's disease with cough, dyspnoea and haemoptysis. Leptospirosis was detected on ELISA testing. Patient was cured rapidly with antibiotics.

  7. Pre-emptive treatment with fibrinogen concentrate for postpartum haemorrhage

    DEFF Research Database (Denmark)

    Wikkelsø, A J; Edwards, H M; Afshari, A

    2015-01-01

    BACKGROUND: In early postpartum haemorrhage (PPH), a low concentration of fibrinogen is associated with excessive subsequent bleeding and blood transfusion. We hypothesized that pre-emptive treatment with fibrinogen concentrate reduces the need for red blood cell (RBC) transfusion in patients...... and the fibrinogen concentration at inclusion. The primary outcome was RBC transfusion up to 6 weeks postpartum. Secondary outcomes were total blood loss, total amount of blood transfused, occurrence of rebleeding, haemoglobin ... concentrate, thereby significantly increasing fibrinogen concentration compared with placebo by 0.40 g litre(-1) (95% confidence interval, 0.15-0.65; P=0.002). Postpartum blood transfusion occurred in 25 (20%) of the fibrinogen group and 26 (22%) of the placebo group (relative risk, 0.95; 95% confidence...

  8. Progress towards the treatment of Ebola haemorrhagic fever.

    Science.gov (United States)

    Ströher, Ute; Feldmann, Heinz

    2006-12-01

    Being highly pathogenic for human and nonhuman primates and the subject of former weapon programmes makes Ebola virus one of the most feared pathogens worldwide today. Due to a lack of licensed pre- and postexposure intervention, the current response depends on rapid diagnostics, proper isolation procedures and supportive care of case patients. Consequently, the development of more specific countermeasures is of high priority for the preparedness of many nations. Over the past years, enhanced research efforts directed to better understand virus replication and pathogenesis have identified potential new targets for intervention strategies. The authors discuss the most promising therapeutic approaches for Ebola haemorrhagic fever as judged by their efficacy in animal models. The current development in this field encourages discussions on how to move some of the experimental approaches towards clinical application.

  9. Crimean-Congo Haemorrhagic Fever in Kosova : a fatal case report

    Directory of Open Access Journals (Sweden)

    Raka Lul

    2006-10-01

    Full Text Available Abstract Crimean-Congo haemorrhagic fever (CCHF is an often fatal viral infection described in about 30 countries around the world. The authors report a fatal case of Crimean-Congo hemorrhagic fever (CCHF observed in a patient from Kosova. The diagnosis of CCHF was confirmed by reverse transcription-PCR. Late diagnosis decreased the efficacy of treatment and patient died due to severe complications of infection.

  10. Ultrastructural and clinical evidence of subretinal debris accumulation in type 2 macular telangiectasia.

    Science.gov (United States)

    Cherepanoff, Svetlana; Killingsworth, Murray C; Zhu, Meidong; Nolan, Timothy; Hunyor, Alex P; Young, Stephanie H; Hageman, Gregory S; Gillies, Mark C

    2012-11-01

    To describe subretinal debris found on ultrastructural examination in an eye with macular telangiectasia (MacTel) type 2 and on optical coherence tomography (OCT) in a subset of patients with MacTel type 2. Blocks from the mid-periphery and temporal perifovea of an eye with clinically documented MacTel type 2 were examined with electron microscopy (EM). Cases came from the Sydney centre of the MacTel project and the practices of the authors. On EM examination, subretinal debris was found in the perifovea with accumulation of degenerate photoreceptor elements in the subretinal space. Despite the substantial subretinal debris, there was minimal retinal pigment epithelial (RPE) reaction. Focal defects were seen in the inner limiting membrane in the perifovea. Of the 65 Sydney MacTel project participants, three (5%) had prominent yellow material at the fovea. OCT revealed smooth mounds between the RPE and the ellipsoid region. The material was hyperautofluorescent. This study suggests that subretinal accumulation of photoreceptor debris may be a feature of MacTel type 2. Ultrastructural and OCT evidence of disease beyond the vasculature, involving photoreceptors and Muller cells, is presented.

  11. Impaired recovery and mutagenic SOS-like responses in ataxia telangiectasia cells

    Energy Technology Data Exchange (ETDEWEB)

    Hilgers, G. (Universite Libre de Bruxelles (Belgium) Rijksuniversiteit Leiden (Netherlands)); Abrahams, P.J. (Rijksuniversiteit Leiden (Netherlands)); Chen, Y.Q. (Universite Libre de Bruxelles (Belgium)); Schouten, R. (Rijksuniversiteit Leiden (Netherlands)); Cornelis, J.J. (Universite Libre de Bruxelles (Belgium) Institut Pasteur, 75 - Paris (France)); Lowe, J.E. (Sussex Univ., Brighton (UK)); Eb, A.J. van der (Rijksuniversiteit Leiden (Netherlands)); Rommelaere, J. (Universite Libre de Bruxelles (Belgium) Institut Pasteur, 75 - Paris (France))

    1989-01-01

    Radiosensitive fibroblasts from patients with ataxia telangiectasia (AT) were studied for their proficiency in two putative eukaryotic SOS-like responses, namely the enhanced reactivation (ER) and enhanced mutagenesis of damaged viruses infecting pre-irradiated versus mock-treated cells. A previous report indicated that, unlike normal human cells, a line of AT fibroblasts (AT5BIVA) could not be induced to express ER of damaged parvovirus H-1, a single-stranded DNA virus, by UV- or X-irradiation. In the present study, AT5BIVA fibroblasts were also distinguished from normal cells by the inability of the former to achieve enhanced mutagenesis of damaged H-1 virus upon cell UV-irradiation. In contrast, dose-response and time-course experiments revealed normal levels of ER of Herpes simplex virus 1, a double-stranded DNA virus, in X- or UV-irradiated AT5BIVA cells. Taken together, these data point to a possible deficiency of AT cells in a conditioned mutagenic process that contributes to a greater extent to the recovery of damaged single-stranded than double-stranded DNA. Such a defect may concern the replication of damaged DNA or the generation of signals promoting the latter process and may be related to the lack of radiation-induced delay that is typical of AT cell DNA synthesis. (author).

  12. Impaired recovery and mutagenic SOS-like responses in ataxia telangiectasia cells

    International Nuclear Information System (INIS)

    Hilgers, G.; Abrahams, P.J.; Chen, Y.Q.; Schouten, R.; Cornelis, J.J.; Lowe, J.E.; Eb, A.J. van der; Rommelaere, J.

    1989-01-01

    Radiosensitive fibroblasts from patients with ataxia telangiectasia (AT) were studied for their proficiency in two putative eukaryotic SOS-like responses, namely the enhanced reactivation (ER) and enhanced mutagenesis of damaged viruses infecting pre-irradiated versus mock-treated cells. A previous report indicated that, unlike normal human cells, a line of AT fibroblasts (AT5BIVA) could not be induced to express ER of damaged parvovirus H-1, a single-stranded DNA virus, by UV- or X-irradiation. In the present study, AT5BIVA fibroblasts were also distinguished from normal cells by the inability of the former to achieve enhanced mutagenesis of damaged H-1 virus upon cell UV-irradiation. In contrast, dose-response and time-course experiments revealed normal levels of ER of Herpes simplex virus 1, a double-stranded DNA virus, in X- or UV-irradiated AT5BIVA cells. Taken together, these data point to a possible deficiency of AT cells in a conditioned mutagenic process that contributes to a greater extent to the recovery of damaged single-stranded than double-stranded DNA. Such a defect may concern the replication of damaged DNA or the generation of signals promoting the latter process and may be related to the lack of radiation-induced delay that is typical of AT cell DNA synthesis. (author)

  13. Effects of hyperthermia and ionizing radiation in normal and ataxia telangiectasia human fibroblast lines

    International Nuclear Information System (INIS)

    Mitchel, R.E.J.; Chan, A.; Smith, B.P.; Child, S.D.; Paterson, M.C.

    1984-01-01

    The effects of 45 0 C hyperthermia and γ radiation have been studied in three normal human fibroblast lines (GM38, GM730, WI38) and compared to the effects in two lines derived from patients with the hereditary disease ataxia telangiectasia (AR3BI, AT5BI). All lines, both normal and γ-sensitive AT, showed a similar resistance to killing by heat alone, suggesting that the defect responsible for the increased radiation sensitivity in AT lines does not confer increased heat sensitivity. Shouldered survival curves were obtained in each case indicating the ability to accumulate sublethal heat damage. All normal and AT cell lines exhibited increased resistance to the lethal effects of heat in response to a thermal stress, indicating that the defect that causes radiosensitivity in AT cell lines does not prevent the induction of thermotolerance. It was hypothesized that in normal cells, this heat treatment inactivates the process which is already defective in AT lines, and that this process may be required for the proper rejoining of double-strand breaks produced during the repair of other radiation-induced lesions

  14. NAD+ Replenishment Improves Lifespan and Healthspan in Ataxia Telangiectasia Models via Mitophagy and DNA Repair.

    Science.gov (United States)

    Fang, Evandro Fei; Kassahun, Henok; Croteau, Deborah L; Scheibye-Knudsen, Morten; Marosi, Krisztina; Lu, Huiming; Shamanna, Raghavendra A; Kalyanasundaram, Sumana; Bollineni, Ravi Chand; Wilson, Mark A; Iser, Wendy B; Wollman, Bradley N; Morevati, Marya; Li, Jun; Kerr, Jesse S; Lu, Qiping; Waltz, Tyler B; Tian, Jane; Sinclair, David A; Mattson, Mark P; Nilsen, Hilde; Bohr, Vilhelm A

    2016-10-11

    Ataxia telangiectasia (A-T) is a rare autosomal recessive disease characterized by progressive neurodegeneration and cerebellar ataxia. A-T is causally linked to defects in ATM, a master regulator of the response to and repair of DNA double-strand breaks. The molecular basis of cerebellar atrophy and neurodegeneration in A-T patients is unclear. Here we report and examine the significance of increased PARylation, low NAD + , and mitochondrial dysfunction in ATM-deficient neurons, mice, and worms. Treatments that replenish intracellular NAD + reduce the severity of A-T neuropathology, normalize neuromuscular function, delay memory loss, and extend lifespan in both animal models. Mechanistically, treatments that increase intracellular NAD + also stimulate neuronal DNA repair and improve mitochondrial quality via mitophagy. This work links two major theories on aging, DNA damage accumulation, and mitochondrial dysfunction through nuclear DNA damage-induced nuclear-mitochondrial signaling, and demonstrates that they are important pathophysiological determinants in premature aging of A-T, pointing to therapeutic interventions. Published by Elsevier Inc.

  15. Primary postpartum haemorrhage at the university of Port Harcourt ...

    African Journals Online (AJOL)

    Background: Postpartum haemorrhage (PPH) is a leading global cause of severe maternal morbidity and mortality. Approximately 14 million women suffer postpartum haemorrhage annually and at least 128,000 of these women bleed to death. Most of these deaths, which occur within four hours of delivery and are as a ...

  16. Home therapy with continuous infusion of factor VIII after minor surgery or serious haemorrhage.

    Science.gov (United States)

    Varon, D; Schulman, S; Bashari, D; Martinowitz, U

    1996-10-01

    Administration of factor VIII (F VIII) concentrates by continuous infusion is now routinely used at several haemophilia centers but almost exclusively for hospitalized patients. We evaluated various aspects of home therapy with continuous infusion of an immunoaffinity purified F VIII concentrate (Monoclate P®, Armour) in patients who would normally have been treated with high doses in bolus injections or with continuous infusion as in-patients. Twenty haemophilia A patients, eight after minor surgery and 12 for serious haemorrhage, received continuous infusion with undiluted F VIII by a minipump for a mean of 0.9 days in the hospital, followed by 3.3 days at home. Infusion bags were exchanged every 2.5 days. No haemorrhagic complications occurred, and five haemorrhages that had been resistant to treatment with bolus injections responded promptly to the continuous infusion. There were no technical problems and patient compliance and acceptance was good. We find this mode of therapy safe, efficacious and convenient for the patients as well as for the staff.

  17. The minimal important difference of the epistaxis severity score in hereditary hemorrhagic telangiectasia.

    Science.gov (United States)

    Yin, Linda X; Reh, Douglas D; Hoag, Jeffrey B; Mitchell, Sally E; Mathai, Stephen C; Robinson, Gina M; Merlo, Christian A

    2016-05-01

    Hereditary hemorrhagic telangiectasia (HHT) is a disease of abnormal angiogenesis, causing epistaxis in over 96% of patients. The Epistaxis Severity Score (ESS) was developed as a standardized measurement of nasal symptoms among HHT patients. The minimal important difference (MID) of a disease index estimates the smallest change that a patient and clinician would identify as important. This study aims to establish the MID of the ESS in a diverse population of HHT patients. Retrospective cross-sectional study in patients with a diagnosis of HHT using Curacao criteria or genetic testing. The ESS questionnaire and Medical Outcomes Study 36-Item Short Form (SF-36) were administered to participants recruited through the HHT Foundation Web site. Demographics and relevant medical histories were collected from all participants. An anchor-based method using a change of 5 in the Physical Component Summary (PCS) of the SF-36 and a distributional method were used to estimate the MID. A total of 604 subjects were recruited between April and August 2008. All participants reported epistaxis. An increasing ESS in the study cohort showed a significant negative correlation to the PCS (r = -0.43, P < 0.001). The MID was determined to be 0.41 via the anchor-based approach and 1.01 via the distribution-based approach, giving a mean MID of 0.71. Using both the anchor-based and distribution-based approaches, the estimated MID for the ESS in HHT is 0.71. Further implications include key metrics to help guide treatment responses in clinical care and essential information to calculate power and sample size for future clinical trials. 4. Laryngoscope, 126:1029-1032, 2016. © 2015 The American Laryngological, Rhinological and Otological Society, Inc.

  18. Molecular diagnosis of Haemorrhagic Septicaemia - A Review

    Directory of Open Access Journals (Sweden)

    Ranjan Rajeev

    2011-08-01

    Full Text Available Pasteurella multocida is associated with hemorrhagic septicaemia in cattle and buffaloes, pneumonic pasteurellosis in sheep and goats, fowl cholera in poultry, atrophic rhinitis in pigs and snuffles in rabbits. Haemorrhagic septicaemia is caused by Pasteurella multocida type B:2, B:2,5 and B:5 in Asian countries and type E:2 in African countries. Pasteurella multocida have five types of capsular serotype i.e. type A, B, D, E and F. Diagnosis of the disease is mainly based on the clinical sign and symptom, post mortem findings. Confirmatory diagnosis is done by isolation and identification of causative agent. A variety of laboratory diagnostic techniques have been developed over the years for pasteurellosis and used routinely in the laboratory. Among these techniques molecular techniques of diagnosis is most important. This technique not only gives diagnosis but it also provides information regarding capsular type of Pasteurella multocida. Techniques which are used for molecular diagnosis of haemorrhagic septicaemia are PCR based diagnosis, Restriction endonuclease analysis (REA, Ribotyping, Colony hybridization assay, Filled alternation gel electrophoresis (FAGE, Detection of Pasteurella multocida by Real Time PCR. Among these techniques real time PCR is most sensitive and specific. [Vet. World 2011; 4(4.000: 189-192

  19. Endovascular management of delayed post-pancreatectomy haemorrhage

    Energy Technology Data Exchange (ETDEWEB)

    Pottier, Edwige [Beaujon Hospital, University Hospitals Paris Nord Val de Seine, Department of Radiology, Clichy, Hauts-de-Seine (France); Ronot, Maxime; Vilgrain, Valerie [Beaujon Hospital, University Hospitals Paris Nord Val de Seine, Department of Radiology, Clichy, Hauts-de-Seine (France); University Paris Diderot, Paris (France); INSERM U1149, centre de recherche biomedicale Bichat-Beaujon, CRB3, Paris (France); Gaujoux, Sebastien; Cesaretti, Manuela; Barbier, Louise [APHP, University Hospitals Paris Nord Val de Seine, Beaujon, Department of Surgery, Clichy, Hauts-de-Seine (France); Sauvanet, Alain [University Paris Diderot, Paris (France); APHP, University Hospitals Paris Nord Val de Seine, Beaujon, Department of Surgery, Clichy, Hauts-de-Seine (France)

    2016-10-15

    To assess the patient outcome after endovascular treatment of delayed post-pancreatectomy haemorrhage (PPH) as first-line treatment. Between January 2005 and November 2013, all consecutive patients referred for endovascular treatment of PPH were included. Active bleeding, pseudoaneurysms, collections and the involved artery were recorded on pretreatment CT. Endovascular procedures were classified as technical success (source of bleeding identified on angiogram and treated), technical failure (source of bleeding identified but incompletely treated) and abstention (no abnormality identified, no treatment performed). Factors associated with rebleeding were analysed. Sixty-nine patients (53 men) were included (mean 59 years old (32-75)). Pretreatment CT showed 27 (39 %) active bleeding. In 22 (32 %) cases, no involved artery was identified. Technical success, failure and abstention were observed in 48 (70 %), 9 (13 %) and 12 patients (17 %), respectively. Thirty patients (43 %) experienced rebleeding. Rebleeding rates were 29 %, 58 % and 100 % in case of success, abstention and failure (p < 0.001). Treatment failure/abstention was the only factor associated with rebleeding. Overall, 74 % of the patients were successfully treated by endovascular procedure(s) alone. After a first endovascular procedure for PPH, the rebleeding rate is high and depends upon the success of the procedure. Most patients are successfully treated by endovascular approach(es) alone. (orig.)

  20. Endovascular management of delayed post-pancreatectomy haemorrhage

    International Nuclear Information System (INIS)

    Pottier, Edwige; Ronot, Maxime; Vilgrain, Valerie; Gaujoux, Sebastien; Cesaretti, Manuela; Barbier, Louise; Sauvanet, Alain

    2016-01-01

    To assess the patient outcome after endovascular treatment of delayed post-pancreatectomy haemorrhage (PPH) as first-line treatment. Between January 2005 and November 2013, all consecutive patients referred for endovascular treatment of PPH were included. Active bleeding, pseudoaneurysms, collections and the involved artery were recorded on pretreatment CT. Endovascular procedures were classified as technical success (source of bleeding identified on angiogram and treated), technical failure (source of bleeding identified but incompletely treated) and abstention (no abnormality identified, no treatment performed). Factors associated with rebleeding were analysed. Sixty-nine patients (53 men) were included (mean 59 years old (32-75)). Pretreatment CT showed 27 (39 %) active bleeding. In 22 (32 %) cases, no involved artery was identified. Technical success, failure and abstention were observed in 48 (70 %), 9 (13 %) and 12 patients (17 %), respectively. Thirty patients (43 %) experienced rebleeding. Rebleeding rates were 29 %, 58 % and 100 % in case of success, abstention and failure (p < 0.001). Treatment failure/abstention was the only factor associated with rebleeding. Overall, 74 % of the patients were successfully treated by endovascular procedure(s) alone. After a first endovascular procedure for PPH, the rebleeding rate is high and depends upon the success of the procedure. Most patients are successfully treated by endovascular approach(es) alone. (orig.)

  1. Magnetic resonance imaging of central nervous system haemorrhage

    International Nuclear Information System (INIS)

    Silberstein, M.; Hennessy, O.

    1993-01-01

    The variable magnetic resonance imaging appearances of central nervous system haemorrhage, both intra- and extra-axial, are described. These will vary with the type of image contrast (T1 or T2 weighting), the nature of the imaging sequence (spin-echo or gradient-echo) and the time from onset of haemorrhage. Magnetic resonance imaging is a useful technique for imaging haemorrhage in the central nervous system as it yields temporal information about haematoma development, and it is the only non-invasive means of imaging intraspinal haemorrhage. However, in the imaging of haematomas within 24 h of onset and in subarachnoid haemorrhage computed tomography is the investigation of choice. 13 refs., 6 figs

  2. Soetomo score: score model in early identification of acute haemorrhagic stroke

    Directory of Open Access Journals (Sweden)

    Moh Hasan Machfoed

    2016-06-01

    Full Text Available Aim of the study: On financial or facility constraints of brain imaging, score model is used to predict the occurrence of acute haemorrhagic stroke. Accordingly, this study attempts to develop a new score model, called Soetomo score. Material and methods: The researchers performed a cross-sectional study of 176 acute stroke patients with onset of ≤24 hours who visited emergency unit of Dr. Soetomo Hospital from July 14th to December 14th, 2014. The diagnosis of haemorrhagic stroke was confirmed by head computed tomography scan. There were seven predictors of haemorrhagic stroke which were analysed by using bivariate and multivariate analyses. Furthermore, a multiple discriminant analysis resulted in an equation of Soetomo score model. The receiver operating characteristic procedure resulted in the values of area under curve and intersection point identifying haemorrhagic stroke. Afterward, the diagnostic test value was determined. Results: The equation of Soetomo score model was (3 × loss of consciousness + (3.5 × headache + (4 × vomiting − 4.5. Area under curve value of this score was 88.5% (95% confidence interval = 83.3–93.7%. In the Soetomo score model value of ≥−0.75, the score reached the sensitivity of 82.9%, specificity of 83%, positive predictive value of 78.8%, negative predictive value of 86.5%, positive likelihood ratio of 4.88, negative likelihood ratio of 0.21, false negative of 17.1%, false positive of 17%, and accuracy of 83%. Conclusions: The Soetomo score model value of ≥−0.75 can identify acute haemorrhagic stroke properly on the financial or facility constrains of brain imaging.

  3. Rejoining of DNA double-strand breaks in human fibroblasts and its impairment in one ataxia telangiectasia and two Fanconi strains

    International Nuclear Information System (INIS)

    Coquerelle, T.M.; Weibezahn, K.F.

    1981-01-01

    Using the technique of neutral elution through polycarbonate filters as a measure of DNA length, and hence of the number of double-strand breaks incurred as a result of radiation damage, we found that normal human fibroblasts rejoin 50% of all breaks within only 3 min (37 degrees C). This fast rejoining was impaired in fibroblasts from one patient with Ataxia telangiectasia and in fibroblasts from two patients with Fanconi's anemia. Also the number of residual breaks after several hours of repair was higher than in control cells. Other cases with the same diseases were normal in their rejoining of double-strand breaks

  4. Cutaneous granulomatosis and combined immunodeficiency revealing Ataxia-Telangiectasia: a case report

    Directory of Open Access Journals (Sweden)

    Antoccia Antonio

    2010-04-01

    Full Text Available Abstract Ataxia-telangiectasia (A-T is a complex multisystem disorder characterized by progressive neurological impairment, variable immunodeficiency and oculo-cutaneous telangiectasia. A-T is a member of chromosomal breakage syndromes and it is caused by a mutation in the ataxia-telangiectasia mutated (ATM gene. Because of a wide clinical heterogeneity, A-T is often difficult to diagnose in children. We report an unusual case of a 3-year-old boy affected by A-T who presented exclusively with extensive cutaneous granulomatosis and severe combined immunodeficiency, without neurological abnormalities, at the time of diagnosis. This case clearly emphasizes the variable presentation of A-T syndrome and highlights the difficulties in the early diagnosis of A-T. A-T should be considered in children with evidence of combined humoral and cellular immunodeficiency associated with unexplained skin granulomatous lesions, even in the absence of the classic features of this syndrome.

  5. Health risks for ataxia-telangiectasia mutated heterozygotes : a systematic review, meta-analysis and evidence-based guideline

    NARCIS (Netherlands)

    van Os, N J H; Roeleveld, N; Weemaes, C M R; Jongmans, M C J; Janssens, G O; Taylor, A M R; Hoogerbrugge, N; Willemsen, Michel A A P

    Ataxia-telangiectasia (AT) is an autosomal recessive neurodegenerative disorder with immunodeficiency and an increased risk of developing cancer, caused by mutations in the ataxia-telangiectasia mutated (ATM) gene. Logically, blood relatives may also carry a pathogenic ATM mutation. Female carriers

  6. ROLE OF HYSTEROSCOPY IN THE MANAGEMENT OF SECONDARY POSTPARTUM HAEMORRHAGE

    Directory of Open Access Journals (Sweden)

    Krupa Patalay

    2016-03-01

    Full Text Available INTRODUCTION Secondary postpartum haemorrhage [PPH] or puerperal haemorrhage, though rare can sometimes cause severe morbidity needing prolonged hospitalisation. Majority of the cases can be managed medically, a few of them requiring surgical interventions. With retained placental tissue being a common cause, emptying the uterus in the puerperium can be difficult and dangerous too as the wall is soft and perforation chances are high. Hysteroscopic evaluation of the puerperal uterus gives us a better picture of the retained bits of placental tissue, and helps in complete evacuation of the tissue without causing much trauma to the fragile uterine wall. It is also more specific than ultrasonogram [USG] to rule out the presence of retained tissue. MATERIAL AND METHODS 17 patients who had secondary PPH and did not respond to the initial medical management were included in the study. Hysteroscopy was done in these cases. Definitive pathology was found in 12 cases; 7 cases had polypoidal tissue [retained bits of placenta], 3 cases had placental tissue adherent to the caesarean scar [placenta accreta] and 2 cases had submucous fibroids. RESULTS In cases which had retained placental bits, the tissue could be visualised, its exact location noted and the entire tissue could be removed without inciting much trauma to the uterine wall. In cases with placenta accreta, gentle extraction of the adherent tissue could be done without traumatising the scar. In one case which had multiple fibroids with irregular uterine cavity, hysteroscopy helped in localising the retained tissue. CONCLUSION Hysteroscopy is a useful modality in managing cases of secondary PPH, who have persistent bleeding in the postpartum period.

  7. Acute subarachnoid haemorrhage: Is a negative CT angiogram enough?

    International Nuclear Information System (INIS)

    MacKinnon, A.D.; Clifton, A.G.; Rich, P.M.

    2013-01-01

    Aim: To determine the negative predictive value of 16 channel multisection computed tomography angiography (CTA) for detecting aneurysms in spontaneous subarachnoid haemorrhage (SAH), using digital subtraction angiography (DSA) as the reference standard. Materials and methods: The prospectively collected cerebral angiogram database of Department of Neuroradiology, Atkinson Morley Regional Neuroscience Centre was used to identify 200 consecutive patients who had undergone DSA for SAH. Of these, 176 had undergone CTA prior to DSA. Clinical details and radiology reports were correlated and images of positive investigations reviewed. Results: DSA showed one or more cerebral aneurysms in 105 (60%) patients. These were correctly reported on CTA in 100. CTA was reported negative for aneurysms in 74 patients. Of these five were false negative and had aneurysms detected on DSA. In the CTA/DSA negative group, 11 (16%) patients had classical perimesencephalic clinical syndrome and blood distribution. There were two false positives at CTA. For ruptured cerebral aneurysms, CTA had 95.2% sensitivity, 97.2% specificity, 98.1% positive predictive value, and 93.2% negative predictive value. Conclusion: The sensitivity and negative predictive value of CTA for ruptured aneurysms remains imperfect. Continued use of DSA is recommended in most patients with a negative CTA after acute SAH. Confirmation of a negative CTA result with DSA may not be routinely required in patients with perimesencephalic syndrome

  8. Dyspnea with anemia turned out to be a case of hereditary hemorrhagic telangiectasia

    Directory of Open Access Journals (Sweden)

    Amitabha Sengupta

    2013-01-01

    Full Text Available Hereditary hemorrhagic telangiectasia (HHT is a rare autosomal dominant inherited disorder of the vascular system. It can be asymptomatic but when symptomatic most common presentation being epistaxis. It can involve any organs of the body like lungs, skin, liver brain, GI mucosa etc. We are reporting a case of HHT presented to us with dyspnea and severe anemia. He had arteriovenous malformations of different visceral organs and telangiectasia of skin along with presence of similar history in first-degree relatives.

  9. Role of chromatin structure modulation by the histone deacetylase inhibitor trichostatin A on the radio-sensitivity of ataxia telangiectasia

    Energy Technology Data Exchange (ETDEWEB)

    Meschini, Roberta, E-mail: meschini@unitus.it; Morucci, Elisa; Berni, Andrea; Lopez-Martinez, Wilner; Palitti, Fabrizio

    2015-07-15

    Highlights: • Role of chromatin compaction on chromosomal instability. • Reduced radiation-induced clastogenicity in Ataxia telangiectasia cell lines. • Histone tails hyperacetylation reduces heterochromatin content favouring DSBs repair. - Abstract: At present, a lot is known about biochemical aspects of double strand breaks (DBS) repair but how chromatin structure affects this process and the sensitivity of DNA to DSB induction is still an unresolved question. Ataxia telangiectasia (A-T) patients are characterised by very high sensitivity to DSB-inducing agents such as ionising radiation. This radiosensitivity is revealed with an enhancement of chromosomal instability as a consequence of defective DNA repair for a small fraction of breaks located in the heterochromatin, where they are less accessible. Besides, recently it has been reported that Ataxia Telangiectasia Mutated (ATM) mediated signalling modifies chromatin structure. In order to study the impact of chromatin compaction on the chromosomal instability of A-T cells, the response to trichostatin-A, an histone deacetylase inhibitor, in normal and A-T lymphoblastoid cell lines was investigated testing its effect on chromosomal aberrations, cell cycle progression, DNA damage and repair after exposure to X-rays. The results suggest that the response to both trichostatin-A pre- and continuous treatments is independent of the presence of either functional or mutated ATM protein, as the reduction of chromosomal damage was found also in the wild-type cell line. The presence of trichostatin-A before exposure to X-rays could give rise to prompt DNA repair functioning on chromatin structure already in an open conformation. Differently, trichostatin-A post-treatment causing hyperacetylation of histone tails and reducing the heterochromatic DNA content might diminish the requirement for ATM and favour DSBs repair reducing chromosomal damage only in A-T cells. This fact could suggest that trichostatin-A post

  10. Risk Factors for Intracranial Haemorrhage in Accidents Associated with the Shower or Bathtub.

    Directory of Open Access Journals (Sweden)

    Thomas C Sauter

    Full Text Available There has been little research on bathroom accidents. It is unknown whether the shower or bathtub are connected with special dangers in different age groups or whether there are specific risk factors for adverse outcomes.This cross-sectional analysis included all direct admissions to the Emergency Department at the Inselspital Bern, Switzerland from 1 January 2000 to 28 February 2014 after accidents associated with the bathtub or shower. Time, age, location, mechanism and diagnosis were assessed and special risk factors were examined. Patient groups with and without intracranial bleeding were compared with the Mann-Whitney U test.The association of risk factors with intracranial bleeding was investigated using univariate analysis with Fisher's exact test or logistic regression. The effects of different variables on cerebral bleeding were analysed by multivariate logistic regression.Two hundred and eighty (280 patients with accidents associated with the bathtub or shower were included in our study. Two hundred and thirty-five (235 patients suffered direct trauma by hitting an object (83.9% and traumatic brain injury (TBI was detected in 28 patients (10%. Eight (8 of the 27 patients with mild traumatic brain injuries (GCS 13-15, (29.6% exhibited intracranial haemorrhage. All patients with intracranial haemorrhage were older than 48 years and needed in-hospital treatment. Patients with intracranial haemorrhage were significantly older and had higher haemoglobin levels than the control group with TBI but without intracranial bleeding (p<0.05 for both.In univariate analysis, we found that intracranial haemorrhage in patients with TBI was associated with direct trauma in general and with age (both p<0.05, but not with the mechanism of the fall, its location (shower or bathtub or the gender of the patient. Multivariate logistic regression analysis identified only age as a risk factor for cerebral bleeding (p<0.05; OR 1.09 (CI 1.01;1.171.In patients

  11. Infrastructure and clinical practice for the detection and management of trauma-associated haemorrhage and coagulopathy

    DEFF Research Database (Denmark)

    Driessen, Annemariek; Schäfer, N; Albrecht, V

    2015-01-01

    of trauma-associated haemorrhage and coagulopathy, we have conducted a web-based survey amongst the delegates to the 15th European Congress of Trauma and Emergency Surgery (ECTES) and the 2nd World Trauma (WT) Congress held in Frankfurt, Germany, 25-27 May 2014. RESULTS: 446/1,540 delegates completed...... frequently used strategies for early detection and monitoring of bleeding trauma patients with coagulopathy. Only 30% of the respondents declared to use extended coagulation assays to better characterise the bleeding and coagulopathy prompted by more individualised treatment concepts. Most trauma centres (69...... intake of anticoagulants including "new oral anticoagulants" and platelet inhibitors as an increasing threat to bleeding trauma patients. CONCLUSIONS: This study confirms differences in infrastructure, logistics and clinical practice for the detection and management of trauma-haemorrhage and trauma...

  12. Efficacy of intra-arterial nimodipine in the treatment of cerebral vasospasm complicating subarachnoid haemorrhage

    Energy Technology Data Exchange (ETDEWEB)

    Hui, C. [Department of Diagnostic Imaging, Monash Medical Centre, Clayton, Vic. (Australia)]. E-mail: cathryn.hui@southernhealth.org.au; Lau, K.P. [Department of Diagnostic Imaging, Monash Medical Centre, Clayton, Vic. (Australia)

    2005-09-01

    AIM: To examine the efficacy and safety of nimodipine as an alternative to papaverine for the treatment of cerebral vasospasm following subarachnoid haemorrhage. METHODS: We retrospectively reviewed the procedure reports, anaesthetic records, clinical charts and CT and angiographic images of 9 patients who had received intra-arterial nimodipine; 1 of these patients received both nimodipine and papaverine. The difference in arterial luminal diameter before and after treatment was calculated as a percentage change. RESULTS: The average dose of nimodipine administered per vessel was 3.3 mg. The mean increase in arterial diameter was 66.6% in the vasospastic segment. There was no significant change in blood pressure of any of the subjects during endovascular treatment of vasospasm. CONCLUSION: Intra-arterial nimodipine is effective in improving angiographic vasospasm complicating subarachnoid haemorrhage. Further studies aimed at examining the clinical benefits of nimodipine are warranted, particularly in view of the low risk of adverse side effects of nimopidine when compared with papaverine.

  13. Bilateral adrenal haemorrhage associated with heparin-induced thrombocytopaenia during treatment of Fournier gangrene.

    Science.gov (United States)

    Tattersall, Timothy Lee; Thangasamy, Isaac A; Reynolds, Jamie

    2014-10-14

    We present a case of bilateral adrenal haemorrhage (BAH) associated with heparin-induced thrombocytopaenia (HIT) in a 61-year-old man admitted to hospital for the treatment of Fournier's gangrene. He presented to hospital with scrotal swelling and fever, and developed spreading erythaema and a gangrenous scrotum. His scrotum was surgically debrided and intravenous broad-spectrum antibiotics were administered. Unfractionated heparin was given postoperatively for venous thromboembolism prophylaxis. The patient deteriorated clinically 8-11 days postoperatively with delirium, chest pain and severe hypertension followed by hypotension and thrombocytopaenia. Abdominal CT scan revealed bilateral adrenal haemorrhage. Antibodies to the heparin-platelet factor 4 complex were present. HIT-associated BAH was diagnosed and heparin was discontinued. Intravenous bivalirudin and hydrocortisone were started, with rapid improvement in clinical status. BAH is a rare complication of HIT and should be considered in the postoperative patient with unexplained clinical deterioration. 2014 BMJ Publishing Group Ltd.

  14. Automation of CT-based haemorrhagic stroke assessment for improved clinical outcomes: study protocol and design.

    Science.gov (United States)

    Chinda, Betty; Medvedev, George; Siu, William; Ester, Martin; Arab, Ali; Gu, Tao; Moreno, Sylvain; D'Arcy, Ryan C N; Song, Xiaowei

    2018-04-19

    Haemorrhagic stroke is of significant healthcare concern due to its association with high mortality and lasting impact on the survivors' quality of life. Treatment decisions and clinical outcomes depend strongly on the size, spread and location of the haematoma. Non-contrast CT (NCCT) is the primary neuroimaging modality for haematoma assessment in haemorrhagic stroke diagnosis. Current procedures do not allow convenient NCCT-based haemorrhage volume calculation in clinical settings, while research-based approaches are yet to be tested for clinical utility; there is a demonstrated need for developing effective solutions. The project under review investigates the development of an automatic NCCT-based haematoma computation tool in support of accurate quantification of haematoma volumes. Several existing research methods for haematoma volume estimation are studied. Selected methods are tested using NCCT images of patients diagnosed with acute haemorrhagic stroke. For inter-rater and intrarater reliability evaluation, different raters will analyse haemorrhage volumes independently. The efficiency with respect to time of haematoma volume assessments will be examined to compare with the results from routine clinical evaluations and planimetry assessment that are known to be more accurate. The project will target the development of an enhanced solution by adapting existing methods and integrating machine learning algorithms. NCCT-based information of brain haemorrhage (eg, size, volume, location) and other relevant information (eg, age, sex, risk factor, comorbidities) will be used in relation to clinical outcomes with future project development. Validity and reliability of the solution will be examined for potential clinical utility. The project including procedures for deidentification of NCCT data has been ethically approved. The study involves secondary use of existing data and does not require new consent of participation. The team consists of clinical neuroimaging

  15. Viral haemorrhagic fever and vascular alterations.

    Science.gov (United States)

    Aleksandrowicz, P; Wolf, K; Falzarano, D; Feldmann, H; Seebach, J; Schnittler, H

    2008-02-01

    Pathogenesis of viral haemorrhagic fever (VHF) is closely associated with alterations of the vascular system. Among the virus families causing VHF, filoviruses (Marburg and Ebola) are the most fatal, and will be focused on here. After entering the body, Ebola primarily targets monocytes/macrophages and dendritic cells. Infected dendritic cells are largely impaired in their activation potency, likely contributing to the immune suppression that occurs during filovirus infection. Monocytes/macrophages, however, immediately activate after viral contact and release reasonable amounts of cytokines that target the vascular system, particularly the endothelial cells. Some underlying molecular mechanisms such as alteration of the vascular endothelial cadherin/catenin complex, tyrosine phosphorylation, expression of cell adhesion molecules, tissue factor and the effect of soluble viral proteins released from infected cells to the blood stream will be discussed.

  16. Does carbetocin for prevention of postpartum haemorrhage at caesarean section provide clinical or financial benefit compared with oxytocin?

    Science.gov (United States)

    Higgins, L; Mechery, J; Tomlinson, A J

    2011-11-01

    Postpartum haemorrhage is a major cause of maternal morbidity and mortality worldwide. A recent Cochrane review of carbetocin (long-acting oxytocin analogue) concluded that its use decreased additional uterotonic requirements, however, no included studies compared its use against intravenous bolus oxytocin. The majority of studies of carbetocin have considered its use in vaginal delivery; no studies have examined the economic implications of its use. This study describes a clinical and financial evaluation undertaken at a United Kingdom District General Hospital surrounding the introduction of carbetocin for prophylaxis against postpartum haemorrhage at caesarean deliveries. A range of clinical outcomes were observed including frequency of postpartum haemorrhage, estimated blood loss, transfusion requirements, change in haemoglobin or haemodynamics, use of additional uterotonics and perioperative recovery. Finally, a composite financial analysis was performed. No clinically significant benefit was found, however associated costs increased by £18.52/patient.

  17. Phylogenetic analysis in a recent controlled outbreak of Crimean-Congo haemorrhagic fever in the south of Iran, December 2008

    DEFF Research Database (Denmark)

    Chinikar, S; Ghiasi, Seyed Mojtaba; Mojtaba Ghiasi, S

    2010-01-01

    Crimean-Congo haemorrhagic fever (CCHF) is a viral zoonotic disease with a high mortality rate in humans. The CCHF virus is transmitted to humans through the bite of Ixodid ticks or contact with blood or tissues of CCHF patients or infected livestock. In December 2008, a re-emerging outbreak...... of CCHF occurred in the southern part of Iran. Five people were hospitalised with sudden fever and haemorrhaging, and CCHF was confirmed by RT-PCR and serological assays. One of the cases had a fulminant course and died. Livestock was identified as the source of infection; all animals in the incriminated...

  18. ELECTROCARDIOGRAPHIC CHANGES OBSERVED IN HAEMORRHAGIC AND ISCHAEMIC CEREBROVASCULAR DISEASES

    Directory of Open Access Journals (Sweden)

    Channappa

    2016-03-01

    Full Text Available INTRODUCTION Cardiac abnormalities are relatively common after acute neurologic injury. Disturbances can vary in severity from transient ECG abnormalities to profound myocardial injury and dysfunction. CNS is involved in the generation of cardiac arrhythmias and dysfunction even in an otherwise normal myocardium. AIM To find out proportion of ECG changes observed in ischaemic and haemorrhagic stroke. MATERIALS AND METHODS The Electrocardiographs of 100 patients with acute stroke were studied to find out the types of ECG abnormalities among different types of stroke. RESULTS In our study, the most common ECG abnormalities associated with stroke were prolonged QTc interval, ST-T segment abnormalities, prominent U wave and arrhythmias. Trop-I was positive in 12.8% patients with ECG changes. Statistical significance was found in association with Trop-I positivity and ST depression. CONCLUSION Usually patients with heart disease present with arrhythmias and Ischaemic like ECG changes. But these changes are also seen most often in the patients with presenting with stroke who didn’t have any past history of heart disease. This shows that arrhythmias and ischaemic ECG abnormalities are primarily evolved due to central nervous system disorders.

  19. Antifibrinolytic drugs for treating primary postpartum haemorrhage.

    Science.gov (United States)

    Shakur, Haleema; Beaumont, Danielle; Pavord, Sue; Gayet-Ageron, Angele; Ker, Katharine; Mousa, Hatem A

    2018-02-20

    Postpartum haemorrhage (PPH) - heaving bleeding within the first 24 hours after giving birth - is one of the main causes of death of women after childbirth. Antifibrinolytics, primarily tranexamic acid (TXA), have been shown to reduce bleeding in surgery and safely reduces mortality in trauma patients with bleeding without increasing the risk of adverse events.An earlier Cochrane review on treatments for primary PPH covered all the various available treatments - that review has now been split by types of treatment. This new review concentrates only on the use of antifibrinolytic drugs for treating primary PPH. To determine the effectiveness and safety of antifibrinolytic drugs for treating primary PPH. We searched Cochrane Pregnancy and Childbirth's Trials Register, ClinicalTrials.gov, the WHO International Clinical Trials Registry Platform (ICTRP) (28 May 2017) and reference lists of retrieved studies. Randomised controlled trials (RCTs), including cluster-randomised trials of antifibrinolytic drugs (aprotinin, TXA, epsilon-aminocaproic acid (EACA) and aminomethylbenzoic acid, administered by whatever route) for primary PPH in women.Participants in the trials were women after birth following a pregnancy of at least 24 weeks' gestation with a diagnosis of PPH, regardless of mode of birth (vaginal or caesarean section) or other aspects of third stage management.We have not included quasi-randomised trials, or cross-over studies. Studies reported as abstracts have not been included if there was insufficient information to allow assessment of risk of bias.In this review we only identified studies looking at TXA. Two review authors independently extracted data from each study using an agreed form. We entered data into Review Manager software and checked for accuracy.For key review outcomes, we rated the quality of the evidence as 'high', 'moderate', 'low' or 'very low' according to the GRADE approach. Three trials (20,412 women) met our inclusion criteria. Two trials

  20. Sinonasal quality of life outcomes following laser treatment of epistaxis related to hereditary hemorrhagic telangiectasia.

    Science.gov (United States)

    Kuan, Edward C; Peng, Kevin A; Thompson, Christopher F; Suh, Jeffrey D; Wang, Marilene B

    2017-04-01

    Hereditary hemorrhagic telangiectasia (HHT) is an inherited vascular disorder which manifests as recurrent, episodic, and potentially debilitating epistaxis. In this study, we aim to (1) characterize baseline sinonasal symptoms for HHT patients and to (2) analyze changes in sinonasal symptoms before and after laser surgical treatment for HHT. We performed a retrospective chart review of sinonasal outcome test-22 (SNOT-22) scores before and after one or more laser surgical treatments for HHT-related epistaxis between January 1, 2010 and December 1, 2015 at a tertiary academic medical center with an HHT Foundation-approved Center of Excellence. Preoperative and all subsequent postoperative SNOT-22 scores (short-term, epistaxis. Mean preoperative, short-term postoperative, and long-term postoperative SNOT-22 scores were 34.6 ± 5.4, 33.9 ± 5.5, and 18.8 ± 4.6, respectively. When analyzing subcategory scores, there was a significant improvement in the rhinologic domain from short-term to long-term postoperatively (13.5 vs. 7.3; p = 0.004), in the non-rhinologic otolaryngic domain from short-term to long-term postoperatively (2.8 vs. 1.7; p = 0.014), and in the psychological domain from preoperative and short-term postoperative to long-term postoperatively (12.2 and 10.0 vs. 6.0; p = 0.015 and 0.01, respectively). Following laser surgery for HHT-related epistaxis, patients' rhinologic symptoms worsened on the short run postoperatively but improved over time. The main benefit of laser treatment appears to be long-term improvement in psychological factors. This study once again underscores the important role of the otolaryngologist in managing sinonasal manifestations of HHT.

  1. Design and Experimental Evaluation of a Non-Invasive Microwave Head Imaging System for Intracranial Haemorrhage Detection.

    Directory of Open Access Journals (Sweden)

    A T Mobashsher

    Full Text Available An intracranial haemorrhage is a life threatening medical emergency, yet only a fraction of the patients receive treatment in time, primarily due to the transport delay in accessing diagnostic equipment in hospitals such as Magnetic Resonance Imaging or Computed Tomography. A mono-static microwave head imaging system that can be carried in an ambulance for the detection and localization of intracranial haemorrhage is presented. The system employs a single ultra-wideband antenna as sensing element to transmit signals in low microwave frequencies towards the head and capture backscattered signals. The compact and low-profile antenna provides stable directional radiation patterns over the operating bandwidth in both near and far-fields. Numerical analysis of the head imaging system with a realistic head model in various situations is performed to realize the scattering mechanism of haemorrhage. A modified delay-and-summation back-projection algorithm, which includes effects of surface waves and a distance-dependent effective permittivity model, is proposed for signal and image post-processing. The efficacy of the automated head imaging system is evaluated using a 3D-printed human head phantom with frequency dispersive dielectric properties including emulated haemorrhages with different sizes located at different depths. Scattered signals are acquired with a compact transceiver in a mono-static circular scanning profile. The reconstructed images demonstrate that the system is capable of detecting haemorrhages as small as 1 cm3. While quantitative analyses reveal that the quality of images gradually degrades with the increase of the haemorrhage's depth due to the reduction of signal penetration inside the head; rigorous statistical analysis suggests that substantial improvement in image quality can be obtained by increasing the data samples collected around the head. The proposed head imaging prototype along with the processing algorithm demonstrates

  2. Familial study of ataxia telangiectasia. Heterozygotes identification on the basis of sensitivity of gamma-irradiated cultures to caffeine post-treatment

    Energy Technology Data Exchange (ETDEWEB)

    Pawlak, A.L.; Kotecki, M. [Polska Akademia Nauk, Poznan (Poland). Zaklad Genetyki Czlowieka; Ignatowicz, R. [Centrum Zdrowia Dziecka, Warsaw (Poland)

    1994-12-31

    The effects of caffeine (CF), {gamma}-irradiation + CF post-treatment on chromosomal aberrations were studied in lymphocyte cultures from a patient with ataxia telangiectasia (AT), his parents and brother. In the studied family both the homozygotes and the obligatory heterozygotes of AT showed increased sensitivity to CF post-treatment. Individual differences in sensitivity to {gamma}-irradiation + CF post-treatment proved to be correlated with the sensitivity of non-irradiated cells to CF treatment, but not to {gamma}-irradiation. (author). 19 refs, 1 fig., 1 tab.

  3. Intense pulsed light vs. long-pulsed dye laser treatment of telangiectasia after radiotherapy for breast cancer: a randomized split-lesion trial of two different treatments

    DEFF Research Database (Denmark)

    Nymann, P.; Hedelund, L.; Hædersdal, Merete

    2009-01-01

    Background Chronic radiodermatitis is a common sequela of treatment for breast cancer and potentially a psychologically distressing factor for the affected women. Objectives To evaluate the efficacy and adverse effects of treatments with a long-pulsed dye laser (LPDL) vs. intense pulsed light (IPL......); the interventions were randomly assigned to left/right or upper/lower halves. Primary end-points were reduction in telangiectasia, patient satisfaction and preferred treatment. Secondary end-points were pain and adverse effects. Efficacy was registered by blinded photographic evaluations 3 months after the final...

  4. Familial study of ataxia telangiectasia. Heterozygotes identification on the basis of sensitivity of gamma-irradiated cultures to caffeine post-treatment

    International Nuclear Information System (INIS)

    Pawlak, A.L.; Kotecki, M.

    1994-01-01

    The effects of caffeine (CF), γ-irradiation + CF post-treatment on chromosomal aberrations were studied in lymphocyte cultures from a patient with ataxia telangiectasia (AT), his parents and brother. In the studied family both the homozygotes and the obligatory heterozygotes of AT showed increased sensitivity to CF post-treatment. Individual differences in sensitivity to γ-irradiation + CF post-treatment proved to be correlated with the sensitivity of non-irradiated cells to CF treatment, but not to γ-irradiation. (author). 19 refs, 1 fig., 1 tab

  5. Malformações arteriovenosas pulmonares – Associação a telangiectasia hemorrágica hereditária. Casos clínicos e rastreio familiar

    Directory of Open Access Journals (Sweden)

    Diva Ferreira

    2006-07-01

    Full Text Available Resumo: As malformações arteriovenosas pulmonares são raras e mais de metade dos casos surgem em associação a telangiectasia hemorrágica hereditária.Faz-se uma revisão teórica sobre a apresentação clínica, abordagem diagnóstica, terapêutica e prognóstico destas malformações vasculares. Estão associadas a morbilidade e mortalidade consideráveis, pelo que se preconiza o seu tratamento, assim como o rastreio dos familiares directos quando se identificam malformações arteriovenosas pulmonares num doente com telangiectasia hemorrágica hereditária. Ainda não existem estudos prospectivos que estabeleçam o melhor plano de estudo do doente e seus familiares.Descrevem-se dois casos clínicos de malformações arteriovenosas pulmonares no contexto familiar de telangiectasia hemorrágica hereditária. A identificação das doentes implicou o rastreio familiar que permitiu detectar malformações arteriovenosas pulmonares em dois familiares e excluir o envolvimento pulmonar em quatro familiares com a doença.Rev Port Pneumol 2006; XII (4: 383-399 Abstract: Pulmonary arteriovenous malformations are a rare disorder associated to hereditary hemorrhagic telangiectasia in over50 % of the cases.Clinical presentation, diagnostic work-up, therapeutic options and prognosis are reviewed by the authors. Pulmonary arteriovenous malformations are known to have considerable morbidity and mortality, their treatment being advisable as well as their screening among family members, especially if the index case is diagnosed with both pulmonary arteriovenous malformations and hereditary hemorrhagic telangiectasia. To this moment prospective studies establishing the best diagnostic work-up for the patients and their families are lacking.The authors report two pulmonary arteriovenous malformations cases in a family with hereditary hemorrhagic telangiectasia. Patient’s diagnosis led

  6. Value of the quantity and distribution of subarachnoid haemorrhage on CT in the localization of ruptured cerebral aneurysm

    International Nuclear Information System (INIS)

    Karttunen, A.I.; Jartti, P.H.; Haapea, M.; Ukkola, V.A.; Sajanti, J.

    2003-01-01

    Computed tomography (CT) is the 'gold standard' for detecting subarachnoid haemorrhage (SAH) and digital subtraction angiography (DSA) for visualising the vascular pathology. We studied retrospectively 180 patients with subarachnoid haemorrhage (SAH) who underwent first non-enhanced computed tomography (CT), then digital subtraction angiography (DSA) and finally operative aneurysm clipping. Our aim was to assess if the location of the ruptured aneurysm could be predicted on the basis of the quantity and distribution of haemorrhage on the initial CT scan. 180 patients with SAH were retrospectively studied. All the CT and DSA examinations were performed at the same hospital. CT was performed within 24 hours after the initial haemorrhage. DSA was performed alter the CT, within 48 hours after the initial haemorrhage. Two neuroradiologists, blind to the DSA results, analysed and scored independently the quantity and distribution of the haemorrhage and predicted the site of the ruptured aneurysm on the basis of the non-enhanced CT. DSA provided the location of the ruptured aneurysm. All the patients were operated upon, and the location of the ruptured aneurysm was determined. The overall reliability value (r,-value) between the two neuroradiologists for locating all ruptured aneurysms was 0.780. The corresponding value for the right MCA was 0.911, that for the left MCA 0.877 and that for the AcoA 0.736. Not all of the rc-values were calculated, either because the location of the rupture was constant or because the number of ruptures in the vessel was too small. Subarachnoid haemorrhage with a parenchymal hematoma is an excellent predictor of the site of the ruptured aneurysm with a statistical significance of p = 0.003. The quantity and pattern of the blood clot an CT within the day of onset of SAH is a reliable and quick cool for locating a ruptured MCA or AcoA aneurysm. It is not, however, reliable for locating other ruptured aneurysms. Subarachnoid haemorrhage with a

  7. Deterministic rather than stochastic factors explain most of the variation in the expression of skin telangiectasia after radiotherapy

    International Nuclear Information System (INIS)

    Safwat, Akmal; Bentzen, Soeren M.; Turesson, Ingela; Hendry, Jolyon H.

    2002-01-01

    Background: The large patient-to-patient variability in the grade of normal tissue injury after a standard course of radiotherapy is well established clinically. A better understanding of this individual variation may provide valuable insights into the pathogenesis of radiation damage and the prospects of predicting the outcome. Purpose: To estimate the relative importance of the stochastic vs. patient-related components of variability in the expression of radiation-induced normal tissue damage. Methods and Materials: The study data were selected from the dose fractionation studies of Turesson in Gothenburg. Patients treated with bilateral internal mammary fields, who completed at least 10 years of follow-up, were included. The material included 22 different fractionation schedules (11 on each side). Telangiectasia was graded on an arbitrary 6-point scale using clinical photographs of the irradiated fields. For each field, in each patient, a curve showing the grade of telangiectasia as a function of time was constructed. A measure of radioresponsiveness was obtained from the difference between the area under the curve (AUC) for a specific field in an individual patient minus the mean AUC of fields receiving the same dose fractionation schedule. As a confirmatory procedure, the same analysis was repeated with a weighted area under the curve (WAUC) approach, in which the time spent at or above each of the 5 nonzero grades was calculated for each field in each patient. These times were used as explanatory variables in a linear regression analysis of biological equivalent dose to establish statistically the weight of each grade providing the optimal relationship between dose and effect. Using these regression coefficients, the weighted area under the grade-time curve (WAUC) was estimated. Results: The AUC was significantly correlated with the isoeffective dose in 2-Gy fractions (ID2). An analysis of variance components, using the maximum likelihood method, showed that

  8. Splenic arteriovenous malformation manifested by thrombocytopenia in hereditary hemorrhagic telangiectasia: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Kwon, Hee Jin; Choi, Jong Cheol; Oh, Jong Yeong; Cho, Jin Han; Kang, Myong Jin; Lee, Jin Hwa; Yoon, Seong Kuk; Nam, Kyeong Jin [College of Medicine, Dong-A University, Busan (Korea, Republic of)

    2008-09-15

    Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant inherited disease characterized by epistaxis, telangiectases and visceral arteriovenous malformations (AVMs). The involvement of the gastrointestinal tract, liver, lung and cerebrum for HHT has been described, whereas little is known about AVMs of the spleen. We report here the radiological findings of a case of a splenic AVM manifested by thrombocytopenia in HHT.

  9. Splenic arteriovenous malformation manifested by thrombocytopenia in hereditary hemorrhagic telangiectasia: a case report

    International Nuclear Information System (INIS)

    Kwon, Hee Jin; Choi, Jong Cheol; Oh, Jong Yeong; Cho, Jin Han; Kang, Myong Jin; Lee, Jin Hwa; Yoon, Seong Kuk; Nam, Kyeong Jin

    2008-01-01

    Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant inherited disease characterized by epistaxis, telangiectases and visceral arteriovenous malformations (AVMs). The involvement of the gastrointestinal tract, liver, lung and cerebrum for HHT has been described, whereas little is known about AVMs of the spleen. We report here the radiological findings of a case of a splenic AVM manifested by thrombocytopenia in HHT

  10. Severe acute haemorrhagic liver failure in a neonate with a favourable spontaneous outcome

    International Nuclear Information System (INIS)

    Cavet, Madeleine; Balu, Marie; Garel, Catherine; Ducou le Pointe, Hubert; Mitanchez, Delphine; Alexandre, Marie; Renolleau, Sylvain; Pariente, Daniele

    2008-01-01

    Acute liver failure in neonates is rare and is frequently associated with an unfavourable outcome. There is no curative treatment other than liver transplantation. Screening for viral, metabolic, toxic or vascular disease is essential to assess the prognosis and to guide specific treatment. Hepatic haemorrhage in neonates is often associated with bacterial infection, trauma and coagulopathies. We present a unique case of neonatal acute liver failure and multifocal massive haemorrhagic intrahepatic lesions of traumatic origin, documented by US and MRI. The patient made a spontaneous recovery. Clinical, biological and imaging outcome was excellent despite the apparent severity of the initial features. The only possible aetiology was a difficult caesarean delivery for mild fetal macrosomia. (orig.)

  11. Severe acute haemorrhagic liver failure in a neonate with a favourable spontaneous outcome

    Energy Technology Data Exchange (ETDEWEB)

    Cavet, Madeleine; Balu, Marie; Garel, Catherine; Ducou le Pointe, Hubert [Universite Pierre et Marie Curie Paris VI, Service de Radiologie, Hopital d' enfants Armand-Trousseau, Paris (France); Mitanchez, Delphine; Alexandre, Marie [Universite Pierre et Marie Curie Paris VI, Service de Neonatologie, Hopital d' enfants Armand-Trousseau, Paris (France); Renolleau, Sylvain [Universite Pierre et Marie Curie Paris VI, Service de Reanimation, Hopital d' enfants Armand-Trousseau, Paris (France); Pariente, Daniele [Hopital de Bicetre, Service de Radiologie Pediatrique, Paris (France)

    2008-10-15

    Acute liver failure in neonates is rare and is frequently associated with an unfavourable outcome. There is no curative treatment other than liver transplantation. Screening for viral, metabolic, toxic or vascular disease is essential to assess the prognosis and to guide specific treatment. Hepatic haemorrhage in neonates is often associated with bacterial infection, trauma and coagulopathies. We present a unique case of neonatal acute liver failure and multifocal massive haemorrhagic intrahepatic lesions of traumatic origin, documented by US and MRI. The patient made a spontaneous recovery. Clinical, biological and imaging outcome was excellent despite the apparent severity of the initial features. The only possible aetiology was a difficult caesarean delivery for mild fetal macrosomia. (orig.)

  12. Serial CT and MRI of ischaemic cerebral infarcts: frequency and clinical impact of haemorrhagic transformation

    Energy Technology Data Exchange (ETDEWEB)

    Mayer, T.E.; Brueckmann, H. [Muenchen Univ. (Germany). Abt. fuer Neuroradiologie; Schulte-Altedorneburg, G. [Dept. of Radiology/Neuroradiology, University Medical School of Luebeck (Germany); Droste, D.W. [Dept. of Neurology, University Medical School of Luebeck (Germany)

    2000-04-01

    The frequency, predisposing factors and clinical consequences of haemorrhagic infarcts and damaged blood-brain barrier as shown by contrast enhancement (CE) in ischaemic cerebral infarcts are controversial. We prospectively compared the sensitivity of CT and MRI to haemorrhagic transformation (HT) and CE. We also wished to investigate the clinical significance of HT and factors possibly associated with it. We studied 36 patients with acute ischaemic infarcts in the middle cerebral artery territory during the first 2 weeks after the ictus. After CT and rating of the neurological deficit on admission, serial examinations with clinical neuromonitoring, contrast-enhanced CT and MRI were done on the same day. The occurrence and severity of HT were correlated with CE, stroke mechanism, infarct size, development of neurological deficits and antithrombotic treatment. The frequency of HT detected by MRI was 80 %. CE usually preceded HT or was seen simultaneously. MRI had a higher sensitivity than CT to HT and CE. Severity of HT was positively correlated with infarct size (P < 0.01). HT had no influence on patient's neurological status. Neither the type of antithrombotic treatment nor the stroke mechanism was associated with the severity of HT. No parenchymal haemorrhage occurred. (orig.)

  13. Duodenal Wedge Resection for Large Gastrointestinal Stromal Tumour Presenting with Life-Threatening Haemorrhage

    Directory of Open Access Journals (Sweden)

    Alexander Shaw

    2013-01-01

    Full Text Available Background. Duodenal gastrointestinal stromal tumours (GISTs are an uncommon malignancy of the gastrointestinal (GI tract. We present a case of life-threatening haemorrhage caused by a large ulcerating duodenal GIST arising from the third part of the duodenum managed by a limited duodenal wedge resection. Case Presentation. A 61-year-old patient presented with acute life-threatening gastrointestinal bleeding. After oesophagogastroduodenoscopy failed to demonstrate the source of bleeding, a 5 cm ulcerating exophytic mass originating from the third part of the duodenum was identified at laparotomy. A successful limited wedge resection of the tumour mass was performed. Histopathology subsequently confirmed a duodenal GIST. The patient remained well at 12-month followup with no evidence of local recurrence or metastatic spread. Conclusion. Duodenal GISTs can present with life-threatening upper GI haemorrhage. In the context of acute haemorrhage, even relatively large duodenal GISTs can be treated by limited wedge resection. This is a preferable alternative to duodenopancreatectomy with lower morbidity and mortality but comparable oncological outcome.

  14. The role of dual energy CT in differentiating between brain haemorrhage and contrast medium after mechanical revascularisation in acute ischaemic stroke

    International Nuclear Information System (INIS)

    Tijssen, M.P.M.; Stadler, A.A.R.; Zwam, W. van; Graaf, R. de; Postma, A.A.; Hofman, P.A.M.; Oostenbrugge, R.J. van; Klotz, E.; Wildberger, J.E.

    2014-01-01

    To assess the feasibility of dual energy computed tomography (DE-CT) in intra-arterially treated acute ischaemic stroke patients to discriminate between contrast extravasation and intracerebral haemorrhage. Thirty consecutive acute ischaemic stroke patients following intra-arterial treatment were examined with DE-CT. Simultaneous imaging at 80 kV and 140 kV was employed with calculation of mixed images. Virtual unenhanced non-contrast (VNC) images and iodine overlay maps (IOM) were calculated using a dedicated brain haemorrhage algorithm. Mixed images alone, as ''conventional CT'', and DE-CT interpretations were evaluated and compared with follow-up CT. Eight patients were excluded owing to a lack of follow-up or loss of data. Mixed images showed intracerebral hyperdense areas in 19/22 patients. Both haemorrhage and residual contrast material were present in 1/22. IOM suggested contrast extravasation in 18/22 patients; in 16/18 patients this was confirmed at follow-up. The positive predictive value (PPV) of mixed imaging alone was 25 %, with a negative predictive value (NPV) of 91 % and accuracy of 63 %. The PPV for detection of haemorrhage with DE-CT was 100 %, with an NPV of 89 % and accuracy improved to 89 %. Dual energy computed tomography improves accuracy and diagnostic confidence in early differentiation between intracranial haemorrhage and contrast medium extravasation in acute stroke patients following intra-arterial revascularisation. (orig.)

  15. The role of dual energy CT in differentiating between brain haemorrhage and contrast medium after mechanical revascularisation in acute ischaemic stroke

    Energy Technology Data Exchange (ETDEWEB)

    Tijssen, M.P.M.; Stadler, A.A.R.; Zwam, W. van; Graaf, R. de; Postma, A.A. [Maastricht University Medical Centre, Department of Radiology, P.O. Box 5800, Maastricht (Netherlands); Hofman, P.A.M. [Maastricht University Medical Centre, Department of Radiology, P.O. Box 5800, Maastricht (Netherlands); Maastricht University, MhENS School for Mental Health and Neuroscience, Maastricht (Netherlands); Oostenbrugge, R.J. van [Maastricht University Medical Centre, Department of Neurology, P.O. Box 5800, Maastricht (Netherlands); Maastricht University, CARIM School for Cardiovascular Diseases, P.O. Box 616, Maastricht (Netherlands); Klotz, E. [Siemens Healthcare Sector, Computed Tomography, Forchheim (Germany); Wildberger, J.E. [Maastricht University Medical Centre, Department of Radiology, P.O. Box 5800, Maastricht (Netherlands); Maastricht University, CARIM School for Cardiovascular Diseases, P.O. Box 616, Maastricht (Netherlands)

    2014-04-15

    To assess the feasibility of dual energy computed tomography (DE-CT) in intra-arterially treated acute ischaemic stroke patients to discriminate between contrast extravasation and intracerebral haemorrhage. Thirty consecutive acute ischaemic stroke patients following intra-arterial treatment were examined with DE-CT. Simultaneous imaging at 80 kV and 140 kV was employed with calculation of mixed images. Virtual unenhanced non-contrast (VNC) images and iodine overlay maps (IOM) were calculated using a dedicated brain haemorrhage algorithm. Mixed images alone, as ''conventional CT'', and DE-CT interpretations were evaluated and compared with follow-up CT. Eight patients were excluded owing to a lack of follow-up or loss of data. Mixed images showed intracerebral hyperdense areas in 19/22 patients. Both haemorrhage and residual contrast material were present in 1/22. IOM suggested contrast extravasation in 18/22 patients; in 16/18 patients this was confirmed at follow-up. The positive predictive value (PPV) of mixed imaging alone was 25 %, with a negative predictive value (NPV) of 91 % and accuracy of 63 %. The PPV for detection of haemorrhage with DE-CT was 100 %, with an NPV of 89 % and accuracy improved to 89 %. Dual energy computed tomography improves accuracy and diagnostic confidence in early differentiation between intracranial haemorrhage and contrast medium extravasation in acute stroke patients following intra-arterial revascularisation. (orig.)

  16. Influencing the practice and outcome in acute upper gastrointestinal haemorrhage. Steering Committee of the National Audit of Acute Upper Gastrointestinal Haemorrhage.

    Science.gov (United States)

    Rockall, T A; Logan, R F; Devlin, H B; Northfield, T C

    1997-11-01

    To assess changes in practice and outcome in acute upper gastrointestinal haemorrhage following the feedback of data, the reemphasis of national guidelines, and specific recommendations following an initial survey. A prospective, multicentre, audit cycle. Forty five hospitals from three health regions participated in two phases of the audit cycle. Phase I: 2332 patients with acute upper gastrointestinal haemorrhage; phase II: 1625 patients with upper gastrointestinal haemorrhage. Patients were evaluated with respect to management (with reference to the recommendations in the national guidelines), mortality, and length of hospital stay. Following the distribution of data from the first phase of the National Audit and the formulation of specific recommendations for improving practice, the proportion of hospitals with local guidelines or protocols for the management of upper gastrointestinal haemorrhage rose from 71% (32/45) to 91% (41/45); 12 of the 32 hospitals with guidelines during the first phase revised their guidelines following the initial survey. There was a small but significant increase in the proportion of all patients who underwent endoscopy (from 81% to 86%), the proportion who underwent endoscopy within 24 hours of admission (from 50% to 56%), and the use of central venous pressure monitoring in patients with organ failure requiring blood transfusion or those with profound shock (from 30% to 43%). There was, however, no change in the use of high dependency beds or joint medical/surgical management in high risk cases. There was no significant change in crude or risk standardised mortality (13.4% in the first phase and 14.4% in the second phase). Although many of the participating hospitals have made efforts to improve practice by producing or updating guidelines or protocols, there has been only a small demonstrable change in some areas of practice during the National Audit. The failure to detect any improvement in mortality may reflect this lack of

  17. Chronic hypopituitarism is uncommon in survivors of aneurysmal subarachnoid haemorrhage.

    Science.gov (United States)

    Hannon, M J; Behan, L A; O'Brien, M M; Tormey, W; Javadpour, M; Sherlock, M; Thompson, C J

    2015-01-01

    The incidence of hypopituitarism after aneurysmal subarachnoid haemorrhage (SAH) is unclear from the conflicting reports in the literature. As routine neuroendocrine screening for hypopituitarism for all patients would be costly and logistically difficult, there is a need for precise data on the frequency of hypopituitarism and on factors which might predict the later development of pituitary dysfunction. We aimed to: (i) Establish the incidence of long-term hypopituitarism in patients with aneurysmal SAH. (ii) Determine whether data from patients' acute admission with SAH could predict the occurrence of long-term hypopituitarism. One hundred patients were studied prospectively from the time of presentation with acute SAH. Plasma cortisol, plasma sodium and a variety of clinical and haemodynamic parameters were sequentially measured for the first 12 days of their acute admission. Forty-one patients then underwent dynamic pituitary testing at median 15 months following SAH (range 7-30 months), with insulin tolerance test (ITT) or, if contraindicated, a glucagon stimulation test (GST) plus short synacthen test (SST). If symptoms of cranial diabetes insipidus (CDI) were present, a water deprivation test was also performed. Forty-one patients attended for follow-up dynamic pituitary testing. Although 14 of 100 had acute glucocorticoid deficiency immediately following SAH, only two of 41 had long-term adrenocorticotrophic hormone (ACTH) deficiency and four of 41 had growth hormone (GH) deficiency. None were hypothyroid or gonadotrophin deficient. None had chronic CDI or hyponatraemia. There was no association between acute glucocorticoid deficiency, acute CDI or acute hyponatraemia and long-term pituitary dysfunction. Both anterior and posterior hypopituitarism are very uncommon following SAH and are not predicted by acute clinical, haemodynamic or endocrinological parameters. Routine neuroendocrine screening is not justified in SAH patients. © 2014 John Wiley & Sons

  18. Dengue fever and dengue haemorrhagic fever in adolescents and adults.

    Science.gov (United States)

    Tantawichien, Terapong

    2012-05-01

    Dengue fever (DF) is endemic in tropical and subtropical zones and the prevalence is increasing across South-east Asia, Africa, the Western Pacific and the Americas. In recent years, the spread of unplanned urbanisation, with associated substandard housing, overcrowding and deterioration in water, sewage and waste management systems, has created ideal conditions for increased transmission of the dengue virus in tropical urban centres. While dengue infection has traditionally been considered a paediatric disease, the age distribution of dengue has been rising and more cases have been observed in adolescents and adults. Furthermore, the development of tourism in the tropics has led to an increase in the number of tourists who become infected, most of whom are adults. Symptoms and risk factors for dengue haemorrhagic fever (DHF) and severe dengue differ between children and adults, with co-morbidities and incidence in more elderly patients associated with greater risk of mortality. Treatment options for DF and DHF in adults, as for children, centre round fluid replacement (either orally or intravenously, depending on severity) and antipyretics. Further data are needed on the optimal treatment of adult patients.

  19. Olivary degeneration after intracranial haemorrhage or trauma: follow-up MRI

    Energy Technology Data Exchange (ETDEWEB)

    Suzuki, M.; Takashima, T.; Ueda, F.; Fujinaga, Y.; Horichi, Y. [Kanazawa Univ. (Japan). School of Medicine; Yamashita, J. [Department of Neurosurgery, Kanazawa Univ. (Japan)

    1999-01-01

    We studied serial MRI appearances of transneuronal degeneration in the inferior olives, retrospectively analysing follow-up images of five patients, three with head injury and two with brain stem haemorrhage. We performed 13 MRI studies 4 days to 2 years 7 months after the accident. All but one of the patients exhibited bilateral olivary high signal on T2-weighted images. The interval between causal event and appearance of olivary changes was 2-4 months, images 4 days to 1.5 months after the accidents revealing no changes. Olivary enlargement was observed in four patients 2-4 months after ictus. (orig.) (orig.) With 2 figs., 1 tab., 10 refs.

  20. Transfusion of blood and blood component therapy for postpartum haemorrhage at a tertiary referral center

    International Nuclear Information System (INIS)

    Hussain, N.; Shah, T.; Shah, N.; Khan, N.H.

    2011-01-01

    Objective: To determine the practice of transfusion of blood and blood products in cases of postpartum haemorrhage, at a tertiary referral center. Methods: A retrospective study was conducted where medical records were reviewed for women, who either delivered or were admitted in labour suite with diagnosis of postpartum haemorrhage. The study period extended from Jan 2008 to Oct 2009. During a period of 22 months, records were reviewed for transfusion of blood and blood products in above group of women. Data were analyzed for descriptive statistics. Results: During the study period, a total of 4744 patients were admitted in the labour suite. A total of 113 (2.36%) women were diagnosed with Post partum haemorrhage. Uterine atony was the commonest cause of PPH, followed by genital tract trauma. A total of 81(71%) women received transfusion of blood and blood components (1.6%). The mean blood loss was 1088 ml (+- 584ml). Transfusion of blood and blood component therapy was significantly more in women who underwent caesarean section, compared to those women who delivered vaginally. There was one case of acute tubular necrosis due to PPH, and seven maternal deaths. The mean hospital stay was of +- 3 days. Conclusion: In this hospital based study, the prevalence of PPH was 2.36 +- %, and the rate of transfusion of blood and blood products was 1.6%.

  1. Complications from office sclerotherapy for epistaxis due to hereditary hemorrhagic telangiectasia (HHT or Osler-Weber-Rendu).

    Science.gov (United States)

    Hanks, John E; Hunter, David; Goding, George S; Boyer, Holly C

    2014-05-01

    The aim of this study was to identify and evaluate adverse clinical outcomes following office-based sclerotherapy using sodium tetradecyl sulfate (STS) for epistaxis due to hereditary hemorrhagic telangiectasias (HHT or Osler-Weber-Rendu). A retrospective chart review of 36 adult patients treated with STS sclerotherapy for severe and/or recurrent epistaxis due to HHT was performed. A total of 153 separate treatment sessions were analyzed. Each patient underwent an average of 4.3 sessions with an average of 7 intralesional injections per session. Bleeding during the procedure was experienced by 8 patients with a maximum reported blood loss of 200 mL in 1 patient, but less than 50 mL in all others. Seven patients reported some postinjection pain, which included nasal, cheek, and eye pain. Nasal congestion, sneezing, and vasovagal responses were each noted to occur 2 times. No complications of postprocedural visual loss, deep venous thrombosis/pulmonary embolus, transient ischemic attack (TIA)/stroke, or anaphylaxis were encountered. Conventional therapies used in the management of HHT-related epistaxis, such as laser coagulation, septodermoplasty, selective arterial embolization, and Young's occlusion each have specific associated complications, including worsened epistaxis, septal perforation, foul odor, nasal crusting, and compromised nasal breathing. STS is a safe office-based treatment option for HHT-mediated epistaxis that is associated with exceedingly few of the aforementioned serious sequelae. © 2014 ARS-AAOA, LLC.

  2. Novel brain arteriovenous malformation mouse models for type 1 hereditary hemorrhagic telangiectasia.

    Directory of Open Access Journals (Sweden)

    Eun-Jung Choi

    Full Text Available Endoglin (ENG is a causative gene of type 1 hereditary hemorrhagic telangiectasia (HHT1. HHT1 patients have a higher prevalence of brain arteriovenous malformation (AVM than the general population and patients with other HHT subtypes. The pathogenesis of brain AVM in HHT1 patients is currently unknown and no specific medical therapy is available to treat patients. Proper animal models are crucial for identifying the underlying mechanisms for brain AVM development and for testing new therapies. However, creating HHT1 brain AVM models has been quite challenging because of difficulties related to deleting Eng-floxed sequence in Eng(2fl/2fl mice. To create an HHT1 brain AVM mouse model, we used several Cre transgenic mouse lines to delete Eng in different cell-types in Eng(2fl/2fl mice: R26CreER (all cell types after tamoxifen treatment, SM22α-Cre (smooth muscle and endothelial cell and LysM-Cre (lysozyme M-positive macrophage. An adeno-associated viral vector expressing vascular endothelial growth factor (AAV-VEGF was injected into the brain to induce focal angiogenesis. We found that SM22α-Cre-mediated Eng deletion in the embryo caused AVMs in the postnatal brain, spinal cord, and intestines. Induction of Eng deletion in adult mice using R26CreER plus local VEGF stimulation induced the brain AVM phenotype. In both models, Eng-null endothelial cells were detected in the brain AVM lesions, and formed mosaicism with wildtype endothelial cells. However, LysM-Cre-mediated Eng deletion in the embryo did not cause AVM in the postnatal brain even after VEGF stimulation. In this study, we report two novel HHT1 brain AVM models that mimic many phenotypes of human brain AVM and can thus be used for studying brain AVM pathogenesis and testing new therapies. Further, our data indicate that macrophage Eng deletion is insufficient and that endothelial Eng homozygous deletion is required for HHT1 brain AVM development.

  3. Acute myocardial infarction: susceptibility-weighted cardiac MRI for the detection of reperfusion haemorrhage at 1.5 T

    International Nuclear Information System (INIS)

    Durighel, G.; Tokarczuk, P.F.; Karsa, A.; Gordon, F.; Cook, S.A.; O'Regan, D.P.

    2016-01-01

    Aim: To assess whether susceptibility-weighted imaging (SWI) provides better image contrast for the detection of haemorrhagic ischaemia–reperfusion injury in the heart. Materials and methods: Thirty patients (all men; mean age 53 years) underwent cardiac magnetic resonance imaging (MRI) within 7 days of primary percutaneous intervention for acute ST elevation myocardial infarction (STEMI). Multiple gradient-echo T2* sequences with magnitude and phase reconstructions were acquired. A high-pass filtered phase map was used to create a mask for the SWI reconstructions. The difference in image contrast was assessed in those patients with microvascular obstruction. A mixed effects regression model was used to test the effect of echo time and reconstruction method on phase and contrast-to-noise ratio (CNR). Medians and interquartile ranges (IQR) are reported. Results: T2* in haemorrhagic infarcts was shorter than in non-haemorrhagic infarcts (33.5 ms [24.9–43] versus 49.9 ms [44.6–67.6]; p=0.0007). The effect of echo time on phase was significant (p<0.0001), as was the effect of haemorrhage on phase (p=0.0016). SWI reconstruction had a significant effect on the CNR at all echo times (echoes 1–5, p<0.0001; echo 6, p=0.01; echo 7, p=0.02). The median echo number at which haemorrhage was first visible was less for SWI compared to source images (echo 2 versus echo 5, p=0.0002). Conclusion: Cardiac SWI improves the contrast between myocardial haemorrhage and the surrounding tissue following STEMI and has potential as a new tool for identifying patients with ischaemia–reperfusion injury. - Highlights: • Cardiac susceptibility-weighted imaging (SWI) is feasible at 1.5T. • Combining phase and modulus data allows blood products to be seen at shorter echo times. • This sequence improves visualisation of reperfusion myocardial haemorrhage.

  4. Ataxia telangiectasia derived iPS cells show preserved x-ray sensitivity and decreased chromosomal instability

    OpenAIRE

    Fukawatase, Yoshihiro; Toyoda, Masashi; Okamura, Kohji; Nakamura, Ken-ichi; Nakabayashi, Kazuhiko; Takada, Shuji; Yamazaki-Inoue, Mayu; Masuda, Akira; Nasu, Michiyo; Hata, Kenichiro; Hanaoka, Kazunori; Higuchi, Akon; Takubo, Kaiyo; Umezawa, Akihiro

    2014-01-01

    Ataxia telangiectasia is a neurodegenerative inherited disease with chromosomal instability and hypersensitivity to ionizing radiation. iPS cells lacking ATM (AT-iPS cells) exhibited hypersensitivity to X-ray irradiation, one of the characteristics of the disease. While parental ataxia telangiectasia cells exhibited significant chromosomal abnormalities, AT-iPS cells did not show any chromosomal instability in vitro for at least 80 passages (560 days). Whole exome analysis also showed a compa...

  5. Procedure-related haemorrhage in embolisation of intracranial aneurysms with Guglielmi detachable coils

    Energy Technology Data Exchange (ETDEWEB)

    Kwon, B.J.; Kim, K.H. [Department of Radiology, Seoul National University College of Medicine, 28 Yongon-Dong, Chongno-Ku, 110-744, Seoul (Korea); Han, M.H.; Chang, K.H. [Department of Radiology, Seoul National University College of Medicine, 28 Yongon-Dong, Chongno-Ku, 110-744, Seoul (Korea); Clinical Research Institute, Seoul National University Hospital, 28 Yongon-Dong, Chongno-Ku, 110-744, Seoul (Korea); Institute of Radiation Medicine, Seoul National University Medical Research Centre, 28 Yongon-Dong, Chongno-Ku, 110-744, Seoul (Korea); Seoul National University College of Medicine (Korea); Oh, C.W. [Department of Neurosurgery, Seoul National University College of Medicine, 28 Yongon-Dong, Chongno-Ku, 110-744, Seoul (Korea)

    2003-08-01

    We reviewed the haemorrhagic complications of the endovascular treatment of intracranial aneurysms, in terms of frequency, pre-embolisation clinical status, clinical and radiological manifestations, management and prognosis. In 275 patients treated for 303 aneurysms over 7 years we had seven (one man and six women - 2.3%) with haemorrhage during or immediately after endovascular treatment. All procedures were performed with a standardised protocol of heparinisation and anaesthesia. Four had ruptured aneurysms, two at the tip of the basilar artery, and one ach on the internal carotid and posterior cerebral artery, treated after 12, 5, 14, and 2 days, respectively, three were in Hunt and Hess grade 2 and one in grade 1. Bleeding occurred during coiling in three, after placement of at least four coils, and during manipulation of the guidewire to enter the aneurysm in the fourth. Haemorrhage was manifest as extravasation of contrast medium, with a sudden rise in systolic blood pressure in three patients. The other three patients had unruptured aneurysms; they had stable blood pressure and angiographic findings during the procedure, but one, under sedation, had seizures immediately after insertion of four coils, and the other two had seizures, headache and vomiting on the day following the procedure. Heparin reversal with protamine sulphate was started promptly started when bleeding was detected in four patients, and the embolisation was completed with additional coils in three. Emergency ventricular drainage was performed in the two patients with ruptured aneurysm and one with an unruptured aneurysm who had abnormal neurological responses or hydrocephalus. The bleeding caused a third nerve palsy in one patient, which might have been due to ischaemia and progressively improved. (orig.)

  6. Phospho-SMC1 in-Cell ELISA based Detection of Ataxia Telangiectasia

    Directory of Open Access Journals (Sweden)

    Majid Zaki dizaji

    2016-12-01

    Full Text Available BackgroundAtaxia telangiectasia (A-T is a common genetically inherited cause of early childhood-onset ataxia. The infrequency of this disease, vast phenotype variation, disorders with features similar to those of A-T, and lack of definite laboratory test, make diagnosis difficult.  In addition, there is no rapid reliable laboratory method for identifying A-T heterozygotes, who susceptible to ionizing radiation (IR, atherosclerosis, diabetes, and cancers. We used SMC1pSer966 (pSMC1 in-cell colorimetric ELISA to diagnosis and screen in A-T families.Materials and Methods: With informed consent, 2cc peripheral blood was collected from the 15 A-T patients, their parents, and 24 healthy controls with no family history of malignancy, diabetes, and atherosclerosis. Extracted peripheral blood mononuclear cells (PBMCs were cultured in poly-L-Lysine treated 96-well plate with density of 70,000 cells per well. SMC1 phosphorylation was evaluated with cell-based ELISA kit 1 hour after 5 Gy IR and the pSMC1data normalized with Glyceraldehyde-3-phosphate dehydrogenase (GAPDH.Results: SMC1 phosphorylation was significantly low in A-T`s PBMC (mean + standard deviation [SD]: 0.075 + 0.034 in comparison to carriers (mean + SD: 0.190 + 0.060 and healthy controls (mean + SD: 0.312 +0.081, but unluckily could only discriminate A-T patients (Area Under the Curve -receiver operating characteristic [AUC-ROC]: 1.00, 1.00-1.00. This method in spite of rapidness and simplicity showed poor imprecision (22.49% coefficient of variation [CV] for intraday imprecision.Conclusion: It seems pSMC1 assessment by in-cell ELISA can be used for detection of A-T patients, but it may not sensitive enough for identification of carriers. This ELISA test is very simple, rapid, and requires less than 2cc blood. Thus it may be proposed for the early differential diagnosis of A-T as an alternative method.

  7. [Fatal alveolar haemorrhage following a "bang" of cannabis].

    Science.gov (United States)

    Grassin, F; André, M; Rallec, B; Combes, E; Vinsonneau, U; Paleiron, N

    2011-09-01

    The new methods of cannabis consumption (home made water pipe or "bang") may be responsible for fatal respiratory complications. We present a case, with fatal outcome, of a man of 19 years with no previous history other than an addiction to cannabis using "bang". He was admitted to intensive care with acute dyspnoea. A CT scan showed bilateral, diffuse alveolar shadowing. He was anaemic with an Hb of 9.3g/l. Bronchoalveolar lavage revealed massive alveolar haemorrhage. Investigations for infection and immunological disorder were negative and toxicology was negative except for cannabis. Antibiotic treatment was given and favourable progress allowed early discharge. Death occurred 15 days later due to alveolar haemorrhage following a further "bang" of cannabis. Autopsy showed toxic alveolar haemorrhage. The probable mechanism is pulmonary damage due to acid anhydrides released by the incomplete combustion of cannabis in contact with plastic. These acids have a double effect on the lungs: a direct toxicity with severe inflammation of the mucosa leading to alveolar haemorrhage and subsequently the acid anhydrides may lead to the syndrome of intra-alveolar haemorrhage and anaemia described in occupational lung diseases by Herbert in Oxford in 1979. It manifests itself by haemoptysis and intravascular haemolysis. We draw attention to the extremely serious potential consequences of new methods of using cannabis, particularly the use of "bang" in homemade plastic materials. Copyright © 2011 SPLF. Published by Elsevier Masson SAS. All rights reserved.

  8. Hemorrhage-adjusted iron requirements, hematinics and hepcidin define hereditary hemorrhagic telangiectasia as a model of hemorrhagic iron deficiency.

    Directory of Open Access Journals (Sweden)

    Helen Finnamore

    Full Text Available Iron deficiency anemia remains a major global health problem. Higher iron demands provide the potential for a targeted preventative approach before anemia develops. The primary study objective was to develop and validate a metric that stratifies recommended dietary iron intake to compensate for patient-specific non-menstrual hemorrhagic losses. The secondary objective was to examine whether iron deficiency can be attributed to under-replacement of epistaxis (nosebleed hemorrhagic iron losses in hereditary hemorrhagic telangiectasia (HHT.The hemorrhage adjusted iron requirement (HAIR sums the recommended dietary allowance, and iron required to replace additional quantified hemorrhagic losses, based on the pre-menopausal increment to compensate for menstrual losses (formula provided. In a study population of 50 HHT patients completing concurrent dietary and nosebleed questionnaires, 43/50 (86% met their recommended dietary allowance, but only 10/50 (20% met their HAIR. Higher HAIR was a powerful predictor of lower hemoglobin (p = 0.009, lower mean corpuscular hemoglobin content (p<0.001, lower log-transformed serum iron (p = 0.009, and higher log-transformed red cell distribution width (p<0.001. There was no evidence of generalised abnormalities in iron handling Ferritin and ferritin(2 explained 60% of the hepcidin variance (p<0.001, and the mean hepcidinferritin ratio was similar to reported controls. Iron supplement use increased the proportion of individuals meeting their HAIR, and blunted associations between HAIR and hematinic indices. Once adjusted for supplement use however, reciprocal relationships between HAIR and hemoglobin/serum iron persisted. Of 568 individuals using iron tablets, most reported problems completing the course. For patients with hereditary hemorrhagic telangiectasia, persistent anemia was reported three-times more frequently if iron tablets caused diarrhea or needed to be stopped.HAIR values, providing an indication of

  9. Dual-energy CT of the brain: Comparison between DECT angiography-derived virtual unenhanced images and true unenhanced images in the detection of intracranial haemorrhage.

    Science.gov (United States)

    Bonatti, Matteo; Lombardo, Fabio; Zamboni, Giulia A; Pernter, Patrizia; Pozzi Mucelli, Roberto; Bonatti, Giampietro

    2017-07-01

    To evaluate the diagnostic performance of virtual non-contrast (VNC) images in detecting intracranial haemorrhages (ICHs). Sixty-seven consecutive patients with and 67 without ICH who underwent unenhanced brain CT and DECT angiography were included. Two radiologists independently evaluated VNC and true non-contrast (TNC) images for ICH presence and type. Inter-observer agreement for VNC and TNC image evaluation was calculated. Sensitivity and specificity of VNC images for ICH detection were calculated using Fisher's exact test. VNC and TNC images were compared for ICH extent (qualitatively and quantitatively) and conspicuity assessment. On TNC images 116 different haemorrhages were detected in 67 patients. Inter-observer agreement ranged from 0.98-1.00 for TNC images and from 0.86-1.00 for VNC images. VNC sensitivity ranged from 0.90-1, according to the different ICH types, and specificity from 0.97-1. Qualitatively, ICH extent was underestimated on VNC images in 11.9% of cases. Haemorrhage volume did not show statistically significant differences between VNC and TNC images. Mean haemorrhage conspicuity was significantly lower on VNC images than on TNC images for both readers (p < 0.001). VNC images are accurate for ICH detection. Haemorrhages are less conspicuous on VNC images and their extent may be underestimated. • VNC images represent a reproducible tool for detecting ICH. • ICH can be identified on VNC images with high sensitivity and specificity. • Intracranial haemorrhages are less conspicuous on VNC images than on TNC images. • Intracranial haemorrhages extent may be underestimated on VNC images.

  10. Effectiveness of rectal misoprostol in cessation of Post Partum Haemorrhage

    International Nuclear Information System (INIS)

    Nasreen, S.; Baqai, S.; Iftikhar, S.

    2018-01-01

    Objective: To determine the efficacy of rectal misoprostol in management of Post Partum Haemorrhage in third stage of labour. Study Design: Interventional study. Place and Duration of Study: Gynaecology and obstetrics department at PNS Shifa Hospital Karachi, from Sep 2012 to Mar 2013. Material and Methods: All singleton and multiple pregnancies of gestation 37-42 weeks, who presented in labour room to deliver and had prolonged second stage of labour (n=112) were enrolled in the study. Patients, who were having coagulopathy, abruption, placenta previa, and allergy to prostaglandins were excluded from the study. Third stage was managed actively according to hospital standard routine. Hemoglobin was measured at the time of admission and repeated after delivery in patients having PPH. Blood was estimated by weighing all gauzes and packs. If blood loss more than 500ml one hour after delivery and all traumatic causes and retained placenta were excluded, 600 mu g (3 tablets) of misoprostol were given per rectally, which were inserted up to a digit depth. After one hour total amount of blood loss was calculated. The data were then entered in a proforma and analyzed. Results: Majority (44.6%) of the women were 26-33 years of age, 8.9% had PPH with fall in hemoglobin by 1.5-2 gms/dl. Ninety percent of the patients responded to rectal misoprostol. Conclusion: Active management of third stage of labour has a definite role in the preventive of PPH. Rectal administration of misoprostol should be considered for control of PPH in low resource settings like ours as it was found effective in the study. (author)

  11. Acute intracerebral haemorrhage: grounds for optimism in management.

    Science.gov (United States)

    Delcourt, Candice; Anderson, Craig

    2012-12-01

    Spontaneous intracerebral haemorrhage (ICH) is one of the most devastating types of stroke, which has considerable disease burden in "non-white" ethnic groups where the population-attributable risks of elevated blood pressure are very high. Since the treatment of ICH remains largely supportive and expectant, nihilism and the early withdrawal of active therapy influence management decisions in clinical practice. However, approaches to management are now better defined on the basis of evidence that both survival and speed (and degree) of recovery are critically dependent on the location, size, and degree of expansion and extension into the intraventricular system of the haematoma of the ICH. Although no medical treatment has been shown to improve outcome in ICH, several promising avenues have emerged that include haemostatic therapy and intensive control of elevated blood pressure. Conversely, there is continued controversy over the role of evacuation of the haematoma of ICH via open craniotomy. Despite being an established practice for several decades, and having undergone evaluation in multiple randomised trials, there is uncertainty over which patients have the most to gain from an intervention with clear procedural risk. Minimally invasive surgery via local anaesthetic applied drill-puncture of the cranium and infusion of a thrombolytic agent is an attractive option for patients requiring critical management of the haematoma, not just in low resource settings but arguably also in specialist centres of western countries. With several ongoing clinical trials nearing completion, these treatments could enter routine practice within the next few years, further justifying the urgency of "time is brain" and that active management within well-organized, comprehensive acute stroke care units includes patients with ICH. Copyright © 2012 Elsevier Ltd. All rights reserved.

  12. Ataxia-telangiectasia cells are not uniformly deficient in poly(ADP-ribose) synthesis following X-irradiation

    International Nuclear Information System (INIS)

    Zwelling, L.A.; Kerrigan, D.; Mattern, M.R.

    1983-01-01

    The synthesis of poly(adenosine diphosphoribose [poly(ADP-R)] follows the DNA strand breakage produced by a number of physical and chemical agents, including X-radiation, and may be important for repair of several types of DNA damage. The reduction or abolition of its synthesis following X-irradiation might explain the enhanced sensitivity of ataxia-telangiectasia (A-T) cells to X-ray. We have examined 8 lines of human fibroblasts (including 4 A-T lines) for stimulation of the synthesis of poly(ADP-R) by X-irradiation. Similar amounts of X-ray-stimulated synthesis of poly(ADP-R) were detected in 4 lines of A-T fibroblasts, and in fibrolasts from a xeroderma pigmentosum (XP) patient, a Fanconi's anemia (FA) patient and 2 normal patients. 6 lines of human lymphoblastoid lines were also examined for X-ray-stimulated poly(ADP-R) synthesis. 4 A-T lines displayed an unusually high synthesis of poly(ADP-R) in unirradiated cells compared with 2 normal lines. (orig./AJ)

  13. Ataxia-telangiectasia cells are not uniformly deficient in poly(ADP-ribose) synthesis following X-irradiation

    Energy Technology Data Exchange (ETDEWEB)

    Zwelling, L.A.; Kerrigan, D. (National Cancer Inst., Bethesda, MD (USA). Lab. of Molecular Pharmacology); Mattern, M.R. (National Cancer Inst., Bethesda, MD (USA). Lab. of Molecular Carcinogenesis)

    1983-04-01

    The synthesis of poly(adenosine diphosphoribose (poly(ADP-R)) follows the DNA strand breakage produced by a number of physical and chemical agents, including X-radiation, and may be important for repair of several types of DNA damage. The reduction or abolition of its synthesis following X-irradiation might explain the enhanced sensitivity of ataxia-telangiectasia (A-T) cells to X-ray. We have examined 8 lines of human fibroblasts (including 4 A-T lines) for stimulation of the synthesis of poly(ADP-R) by X-irradiation. Similar amounts of X-ray-stimulated synthesis of poly(ADP-R) were detected in 4 lines of A-T fibroblasts, and in fibrolasts from a xeroderma pigmentosum (XP) patient, a Fanconi's anemia (FA) patient and 2 normal patients. 6 lines of human lymphoblastoid lines were also examined for X-ray-stimulated poly(ADP-R) synthesis. 4 A-T lines displayed an unusually high synthesis of poly(ADP-R) in unirradiated cells compared with 2 normal lines.

  14. Targeted disruption of Ataxia-telangiectasia mutated gene in miniature pigs by somatic cell nuclear transfer

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Young June; Ahn, Kwang Sung; Kim, Minjeong; Kim, Min Ju; Park, Sang-Min; Ryu, Junghyun; Ahn, Jin Seop; Heo, Soon Young; Kang, Jee Hyun; Choi, You Jung [Department of Nanobiomedical Science and BK21 PLUS NBM Global Research Center for Regenerative Medicine, Dankook University, Cheonan (Korea, Republic of); Choi, Seong-Jun [Institute of Tissue Regeneration Engineering, Dankook University, Cheonan (Korea, Republic of); Shim, Hosup, E-mail: shim@dku.edu [Department of Nanobiomedical Science and BK21 PLUS NBM Global Research Center for Regenerative Medicine, Dankook University, Cheonan (Korea, Republic of); Institute of Tissue Regeneration Engineering, Dankook University, Cheonan (Korea, Republic of); Department of Physiology, Dankook University School of Medicine, Cheonan (Korea, Republic of)

    2014-10-03

    Highlights: • ATM gene-targeted pigs were produced by somatic cell nuclear transfer. • A novel large animal model for ataxia telangiectasia was developed. • The new model may provide an alternative to the mouse model. - Abstract: Ataxia telangiectasia (A-T) is a recessive autosomal disorder associated with pleiotropic phenotypes, including progressive cerebellar degeneration, gonad atrophy, and growth retardation. Even though A-T is known to be caused by the mutations in the Ataxia telangiectasia mutated (ATM) gene, the correlation between abnormal cellular physiology caused by ATM mutations and the multiple symptoms of A-T disease has not been clearly determined. None of the existing ATM mouse models properly reflects the extent to which neurological degeneration occurs in human. In an attempt to provide a large animal model for A-T, we produced gene-targeted pigs with mutations in the ATM gene by somatic cell nuclear transfer. The disrupted allele in the ATM gene of cloned piglets was confirmed via PCR and Southern blot analysis. The ATM gene-targeted pigs generated in the present study may provide an alternative to the current mouse model for the study of mechanisms underlying A-T disorder and for the development of new therapies.

  15. Targeted disruption of Ataxia-telangiectasia mutated gene in miniature pigs by somatic cell nuclear transfer

    International Nuclear Information System (INIS)

    Kim, Young June; Ahn, Kwang Sung; Kim, Minjeong; Kim, Min Ju; Park, Sang-Min; Ryu, Junghyun; Ahn, Jin Seop; Heo, Soon Young; Kang, Jee Hyun; Choi, You Jung; Choi, Seong-Jun; Shim, Hosup

    2014-01-01

    Highlights: • ATM gene-targeted pigs were produced by somatic cell nuclear transfer. • A novel large animal model for ataxia telangiectasia was developed. • The new model may provide an alternative to the mouse model. - Abstract: Ataxia telangiectasia (A-T) is a recessive autosomal disorder associated with pleiotropic phenotypes, including progressive cerebellar degeneration, gonad atrophy, and growth retardation. Even though A-T is known to be caused by the mutations in the Ataxia telangiectasia mutated (ATM) gene, the correlation between abnormal cellular physiology caused by ATM mutations and the multiple symptoms of A-T disease has not been clearly determined. None of the existing ATM mouse models properly reflects the extent to which neurological degeneration occurs in human. In an attempt to provide a large animal model for A-T, we produced gene-targeted pigs with mutations in the ATM gene by somatic cell nuclear transfer. The disrupted allele in the ATM gene of cloned piglets was confirmed via PCR and Southern blot analysis. The ATM gene-targeted pigs generated in the present study may provide an alternative to the current mouse model for the study of mechanisms underlying A-T disorder and for the development of new therapies

  16. Where does ergometrine stand in prevention of postpartum haemorrhage in caesarean section

    Energy Technology Data Exchange (ETDEWEB)

    Mahmud, G.; Javaid, K.; Tasnim, N.; Tabassum, A.; Bangash, K. T. [Pakistan Inst. of Medical Sciences (PIMS), Islamabad (Pakistan). Maternal and Child Health Centre Unit-I

    2014-08-15

    Objective: To compare the safety and efficacy of 10 units of intravenous syntocinon alone with 10 units intravenous syntocinon and 0.25 mg intramuscular ergometrine in the prevention of atonic uterine haemorrhage during caesarean section. Method: The quasi-experimental study was conducted at the Maternal and Child Health Centre, Unit I, Pakistan Institute of Medical Sciences, Islamabad, from November 1, 2010 to February 28, 2011. All women undergoing caesarean section were included in the study. Patients were given intravenous 10 units syntocinon alone intra-operatively from November 1 to December 31, 2010, while 0.25 mg ergometrine intramuscular was added to 10 units intravenous syntocinon from January 1 to February 28, 2011. Frequency of postpartum haemorrhage, adverse effects of drugs and maternal morbidity and mortality were assessed by using chi square test. P <0.05 was taken as statistically significant. Results: Of the total number of 701 subjects, 378 (54%) women were given 10 units syntocinon and 323 (46%) were given 0.25 mg ergometrine in addition to 10 units syntocinon. The mean age in the syntocinon group was 28+-3.5 yrs with gestational age of 37.5+-2 weeks, while that in syntocinon-ergometrine group was 29+-3.4 years and 38+-2 weeks respectively. Postpartum haemorrhage in the syntocinon group was found in 38 (10%) women versus 05 (1.5%) women) in the other group (p<0.001). Adverse effects like nausea, vomiting and raised blood pressure were slightly more with syntocinon-ergometrine than syntocinon alone (n=56; 15.3% vs n=35; 9.2%), but it was not statistically significant. Post partum haemorrhage was responsible for 40% of maternal mortality during the study period and that was in the syntocinon group. Conclusion: Prophylactic ergometrine in addition to syntocinon is superior to syntocinon alone in decreasing frequency of postpartum haemorrhage in caesarean section and associated maternal morbidity and mortality. Regarding safety profile, the two

  17. Where does ergometrine stand in prevention of postpartum haemorrhage in caesarean section

    International Nuclear Information System (INIS)

    Mahmud, G.; Javaid, K.; Tasnim, N.; Tabassum, A.; Bangash, K. T.

    2014-01-01

    Objective: To compare the safety and efficacy of 10 units of intravenous syntocinon alone with 10 units intravenous syntocinon and 0.25 mg intramuscular ergometrine in the prevention of atonic uterine haemorrhage during caesarean section. Method: The quasi-experimental study was conducted at the Maternal and Child Health Centre, Unit I, Pakistan Institute of Medical Sciences, Islamabad, from November 1, 2010 to February 28, 2011. All women undergoing caesarean section were included in the study. Patients were given intravenous 10 units syntocinon alone intra-operatively from November 1 to December 31, 2010, while 0.25 mg ergometrine intramuscular was added to 10 units intravenous syntocinon from January 1 to February 28, 2011. Frequency of postpartum haemorrhage, adverse effects of drugs and maternal morbidity and mortality were assessed by using chi square test. P <0.05 was taken as statistically significant. Results: Of the total number of 701 subjects, 378 (54%) women were given 10 units syntocinon and 323 (46%) were given 0.25 mg ergometrine in addition to 10 units syntocinon. The mean age in the syntocinon group was 28+-3.5 yrs with gestational age of 37.5+-2 weeks, while that in syntocinon-ergometrine group was 29+-3.4 years and 38+-2 weeks respectively. Postpartum haemorrhage in the syntocinon group was found in 38 (10%) women versus 05 (1.5%) women) in the other group (p<0.001). Adverse effects like nausea, vomiting and raised blood pressure were slightly more with syntocinon-ergometrine than syntocinon alone (n=56; 15.3% vs n=35; 9.2%), but it was not statistically significant. Post partum haemorrhage was responsible for 40% of maternal mortality during the study period and that was in the syntocinon group. Conclusion: Prophylactic ergometrine in addition to syntocinon is superior to syntocinon alone in decreasing frequency of postpartum haemorrhage in caesarean section and associated maternal morbidity and mortality. Regarding safety profile, the two

  18. Sucralfate paste enema: a new method of topical treatment for haemorrhagic radiation proctitis.

    Science.gov (United States)

    McElvanna, K; Wilson, A; Irwin, T

    2014-04-01

    Rectal sucralfate has been reported to deliver clinical improvement in haemorrhagic radiation proctitis, but patients with active proctitis find the enema suspension difficult to retain thus reducing compliance and effectiveness. We describe a novel method of rectal administration via a low-volume sucralfate paste and report its results in a series of 23 patients. Patients with rectal bleeding occurring more than 6 months after radiotherapy were shown how to prepare and self-administer sucralfate paste enemas (SPEs) twice daily for 6 weeks. The SPE was prepared using two sucralfate 1-g tablets mixed with 4.5 ml of water in an enema applicator producing a low-volume paste. The clinical response was evaluated by comparison of pre- and posttreatment clinical proctitis scores (Radiation Therapy Oncology Group/European Organisation for Research and Treatment of Cancer). Eighteen male (postprostatic radiotherapy) and five female (postcervical radiotherapy) patients with a median age of 67 (32-75) years were included. The median interval between pelvic irradiation and SPE treatment was 24 (7-69) months. Twenty-two patients had full clinical scoring, of whom 16 (73%) demonstrated clinical improvement. Six (27%) had neither clinical improvement nor deterioration. Seven (32%) had resolution of all symptoms. Most patients demonstrated clinical improvement. This initial experience of the sucralfate paste enema may provide the basis for a prospective study of its effectiveness in the treatment of haemorrhagic radiation proctitis. Colorectal Disease © 2013 The Association of Coloproctology of Great Britain and Ireland.

  19. Vesical Artery Embolization in Haemorrhagic Cystitis in Children

    Energy Technology Data Exchange (ETDEWEB)

    García-Gámez, Andrés, E-mail: agargamez@gmail.com; Bermúdez Bencerrey, Patricia, E-mail: PBERMUDE@clinic.ub.es [Hospital Clinic (Spain); Brio-Sanagustin, Sonia, E-mail: sbrio@santpau.cat [Hospital de la Santa Creu y Sant Pau (Spain); Guerrero Vara, Rubén, E-mail: rguerrerov@santpau.cat [Hospital Clinic (Spain); Sisinni, Luisa, E-mail: lsisinni@santpau.cat [Hospital de la Santa Creu y Sant Pau (Spain); Stuart, Sam, E-mail: sam.stuart@gosh.nhs.uk; Roebuck, Derek, E-mail: Derek.Roebuck@gosh.nhs.uk [Great Ormond Street Hospital for Children (United Kingdom); Gómez Muñoz, Fernando, E-mail: FEGOMEZ@clinic.ub.es [Hospital Clinic (Spain)

    2016-07-15

    Haemorrhagic cystitis is an uncommon and, in its severe form, potentially life-threatening complication of haematopoietic stem cell transplantation or cancer therapy in children. The severe form involves macroscopic haematuria with blood clots, urinary obstruction and/or renal impairment. There are many therapeutic options to treat acute haemorrhage, but only recombinant factor VII has a high level of clinical evidence in children. Supraselective vesical artery embolization (SVAE) is an increasingly used therapeutic procedure for controlling haemorrhage in adults, but is less commonly used in children. This might be due to several factors, such as the invasive nature of the procedure, lack of appropriate medical experience and possible long-term side effects. We present three cases of children successfully treated by means of effective SVAE.

  20. Neurosurgical management of L-asparaginase induced haemorrhagic stroke.

    LENUS (Irish Health Repository)

    Ogbodo, Elisha

    2012-01-01

    The authors describe a case of L-asparaginase induced intracranial thrombosis and subsequent haemorrhage in a newly diagnosed 30-year-old man with acute lymphoblastic leukaemia who was successfully managed by surgical intervention. At presentation, he had a Glasgow Coma Score of 7\\/15, was aphasic and had dense right hemiplegia. Neuroimaging revealed an acute anterior left middle cerebral artery infarct with parenchymal haemorrhagic conversion, mass effect and subfalcine herniation. He subsequently underwent left frontal craniotomy and evacuation of large frontal haematoma and decompressive craniectomy for cerebral oedema. Six months postoperatively he underwent titanium cranioplasty. He had made good clinical recovery and is currently mobilising independently with mild occasional episodes of expressive dysphasia, difficulty with fine motor movement on the right side, and has remained seizure free. This is the first documented case of L-asparaginase induced haemorrhagic stroke managed by neurosurgical intervention. The authors emphasise the possible role of surgery in managing chemotherapy induced intracranial complications.

  1. International prospective observational study of upper gastrointestinal haemorrhage: Does weekend admission affect outcome?

    DEFF Research Database (Denmark)

    Murray, Iain A.; Dalton, Harry R.; Stanley, Adrian J.

    2017-01-01

    Introduction Out of hours admissions have higher mortality for many conditions but upper gastrointestinal haemorrhage studies have produced variable outcomes. Methods Prospective study of 12 months consecutive admissions of upper gastrointestinal haemorrhage from four international high volume...

  2. Dual-energy CT of the brain: Comparison between DECT angiography-derived virtual unenhanced images and true unenhanced images in the detection of intracranial haemorrhage

    International Nuclear Information System (INIS)

    Bonatti, Matteo; Pernter, Patrizia; Bonatti, Giampietro; Lombardo, Fabio; Zamboni, Giulia A.; Pozzi Mucelli, Roberto

    2017-01-01

    To evaluate the diagnostic performance of virtual non-contrast (VNC) images in detecting intracranial haemorrhages (ICHs). Sixty-seven consecutive patients with and 67 without ICH who underwent unenhanced brain CT and DECT angiography were included. Two radiologists independently evaluated VNC and true non-contrast (TNC) images for ICH presence and type. Inter-observer agreement for VNC and TNC image evaluation was calculated. Sensitivity and specificity of VNC images for ICH detection were calculated using Fisher's exact test. VNC and TNC images were compared for ICH extent (qualitatively and quantitatively) and conspicuity assessment. On TNC images 116 different haemorrhages were detected in 67 patients. Inter-observer agreement ranged from 0.98-1.00 for TNC images and from 0.86-1.00 for VNC images. VNC sensitivity ranged from 0.90-1, according to the different ICH types, and specificity from 0.97-1. Qualitatively, ICH extent was underestimated on VNC images in 11.9% of cases. Haemorrhage volume did not show statistically significant differences between VNC and TNC images. Mean haemorrhage conspicuity was significantly lower on VNC images than on TNC images for both readers (p < 0.001). VNC images are accurate for ICH detection. Haemorrhages are less conspicuous on VNC images and their extent may be underestimated. (orig.)

  3. Dual-energy CT of the brain: Comparison between DECT angiography-derived virtual unenhanced images and true unenhanced images in the detection of intracranial haemorrhage

    Energy Technology Data Exchange (ETDEWEB)

    Bonatti, Matteo; Pernter, Patrizia; Bonatti, Giampietro [Bolzano Central Hospital, Department of Radiology, Bolzano (Italy); Lombardo, Fabio; Zamboni, Giulia A.; Pozzi Mucelli, Roberto [University of Verona, Department of Radiology, Verona (Italy)

    2017-07-15

    To evaluate the diagnostic performance of virtual non-contrast (VNC) images in detecting intracranial haemorrhages (ICHs). Sixty-seven consecutive patients with and 67 without ICH who underwent unenhanced brain CT and DECT angiography were included. Two radiologists independently evaluated VNC and true non-contrast (TNC) images for ICH presence and type. Inter-observer agreement for VNC and TNC image evaluation was calculated. Sensitivity and specificity of VNC images for ICH detection were calculated using Fisher's exact test. VNC and TNC images were compared for ICH extent (qualitatively and quantitatively) and conspicuity assessment. On TNC images 116 different haemorrhages were detected in 67 patients. Inter-observer agreement ranged from 0.98-1.00 for TNC images and from 0.86-1.00 for VNC images. VNC sensitivity ranged from 0.90-1, according to the different ICH types, and specificity from 0.97-1. Qualitatively, ICH extent was underestimated on VNC images in 11.9% of cases. Haemorrhage volume did not show statistically significant differences between VNC and TNC images. Mean haemorrhage conspicuity was significantly lower on VNC images than on TNC images for both readers (p < 0.001). VNC images are accurate for ICH detection. Haemorrhages are less conspicuous on VNC images and their extent may be underestimated. (orig.)

  4. Perforated ileal duplication cyst with haemorrhagic pseudocyst formation

    International Nuclear Information System (INIS)

    Hwang, Im Kyung; Kim, Bong Soo; Kim, Heung Chul; Lee, In Sun; Hwang, Woo Chul; Namkung, Sook

    2003-01-01

    Duplication cysts of the gastrointestinal tract are rare congenital abnormalities. Ectopic gastric mucosa, which can be found in duplications, may cause peptic ulceration, gastrointestinal bleeding or perforation. We report a 1-year-old boy with a perforated ileal duplication cyst with haemorrhagic pseudocyst formation caused by peptic ulceration of the duplication cyst. It presented a snowman-like appearance consisting of a small, thick-walled, true enteric cyst and a large, thin-walled haemorrhagic pseudocyst on US and CT. It is an unusual manifestation of a duplication cyst, which has not been reported in the English language literature. (orig.)

  5. Bilateral adrenal haemorrhage secondary to intra-abdominal sepsis: a case report.

    LENUS (Irish Health Repository)

    Egan, Aoife M

    2012-01-31

    INTRODUCTION: Bilateral adrenal haemorrhage is a rare cause of adrenal failure. Clinical features are non-specific and therefore a high index of suspicion must be maintained in patients at risk. Predisposing factors include infection, malignancy and the post-operative state. CASE PRESENTATION: We report the case of a patient who underwent a left hemicolectomy with primary anastomosis and formation of a defunctioning loop ileostomy for an obstructing colon carcinoma at the splenic flexure. En-bloc splenectomy was performed to ensure an oncologic resection. The patient developed a purulent abdominal collection post-operatively and became septic with hypotension and pyrexia. This precipitated acute bilateral adrenal haemorrhage with consequent adrenal insufficiency. Clinical suspicion was confirmed by radiological findings and a co-syntropin test. Following drainage of the collection, antibiotic therapy and corticosteroid replacement, the patient made an excellent recovery. CONCLUSION: This case highlights the importance of prompt diagnosis and treatment of adrenal failure. In their absence, this condition can rapidly lead to death of the patient.

  6. The rate of DNA synthesis in normal human and ataxia telangiectasia cells after exposure to X-irradiation

    International Nuclear Information System (INIS)

    Wit, J. de; Bootsma, D.; Jaspers, N.G.J.; Rijksverdedigingsorganisatie TNO, Rijswijk

    1981-01-01

    The rate of DNA synthesis was studied in normal cell strains and in strains from patients suffering from the inherited disorder ataxia telangiectasia (AT). After exposure to relatively low doses of oxic X-rays (0- 4 krad) DNA synthesis was depressed in AT cell strains to a significantly lesser extent than in normal cells. This response was observed in both an excision-deficient and an excision-proficient strain. In contrast, there was no difference in DNA-synthesis inhibition between AT and normal cells after UV exposure. After X-irradiation of cells from patients with xeroderma pigmentosum, both complementation group A and XP variants, the observed rate of DNA synthesis was equal to that in normal cells. An exception was the strain XP3BR which has been shown to be X-ray-sensitive. This strain exhibited diminished DNA synthesis inhibition after X-ray doses below 1 krad. These data suggest a relationship between hypersensitivity to X-rays and diminished depression of DNA synthesis. (orig.)

  7. An evaluation of the severity and progression of epistaxis in hereditary hemorrhagic telangiectasia (HHT) 1 vs. HHT 2

    Science.gov (United States)

    Hunter, Benjamin N.; Timmins, Benjamin H.; McDonald, Jamie; Whitehead, Kevin J.; Ward, P. Daniel; Wilson, Kevin F.

    2015-01-01

    Objective Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular dysplasia whose hallmark symptom is spontaneous recurrent epistaxis. Two major genetic sub-types of this syndrome are HHT1 and HHT2. Severity of epistaxis ranges from occasional low-volume bleeding to frequent large-volume hemorrhage. This study evaluated the severity and progression of epistaxis in HHT1 vs. HHT2. Study Design Retrospective cohort study Methods Retrospective chart review was performed for 183 genotyped HHT patients seen at our Center from 2010-2013. Data collected included epistaxis severity score (ESS), age of epistaxis onset, number and type of treatments, age at which treatments were sought, complete blood count values, ferritin, number of telangiectases, blood transfusions, iron therapy history, and patient demographics. Results 115 subjects with HHT2 were compared to 68 with HHT1. Subjects with HHT2 had a higher ESS compared to HHT1 (p=0.043) and a later age of onset of epistaxis (p=0.005). HHT2 subjects were more likely to use oral iron (p=0.032) and were more likely to seek interventions to control their epistaxis (p= 0.029). Conclusion HHT2 is associated with more severe epistaxis and a subsequent higher rate of interventions, requiring more aggressive therapy as compared to HHT1. PMID:26372311

  8. Hemorrhage-Adjusted Iron Requirements, Hematinics and Hepcidin Define Hereditary Hemorrhagic Telangiectasia as a Model of Hemorrhagic Iron Deficiency

    Science.gov (United States)

    Finnamore, Helen; Le Couteur, James; Hickson, Mary; Busbridge, Mark; Whelan, Kevin; Shovlin, Claire L.

    2013-01-01

    Background Iron deficiency anemia remains a major global health problem. Higher iron demands provide the potential for a targeted preventative approach before anemia develops. The primary study objective was to develop and validate a metric that stratifies recommended dietary iron intake to compensate for patient-specific non-menstrual hemorrhagic losses. The secondary objective was to examine whether iron deficiency can be attributed to under-replacement of epistaxis (nosebleed) hemorrhagic iron losses in hereditary hemorrhagic telangiectasia (HHT). Methodology/Principal Findings The hemorrhage adjusted iron requirement (HAIR) sums the recommended dietary allowance, and iron required to replace additional quantified hemorrhagic losses, based on the pre-menopausal increment to compensate for menstrual losses (formula provided). In a study population of 50 HHT patients completing concurrent dietary and nosebleed questionnaires, 43/50 (86%) met their recommended dietary allowance, but only 10/50 (20%) met their HAIR. Higher HAIR was a powerful predictor of lower hemoglobin (p = 0.009), lower mean corpuscular hemoglobin content (pstopped. Conclusions/significance HAIR values, providing an indication of individuals’ iron requirements, may be a useful tool in prevention, assessment and management of iron deficiency. Iron deficiency in HHT can be explained by under-replacement of nosebleed hemorrhagic iron losses. PMID:24146883

  9. Bilateral haemorrhagic infarction of the globus pallidus after cocaine and alcohol intoxication.

    Science.gov (United States)

    Renard, Dimitri; Brunel, Hervé; Gaillard, Nicolas

    2009-06-01

    Cocaine is a risk factor for both ischemic and haemorrhagic stroke. We present the case of a 31-year-old man with bilateral ischemia of the globus pallidus after excessive alcohol and intranasal cocaine use. Drug-related globus pallidus infarctions are most often associated with heroin. Bilateral basal ganglia infarcts after the use of cocaine, without concurrent heroin use, have never been reported. In our patient, transient cardiac arrhythmia or respiratory dysfunction related to cocaine and/or ethanol use were the most likely causes of cerebral hypoperfusion.

  10. [Haemorrhagic proctocolitis as primary manifestation of lymphogranuloma venereum in an HIV-positive male].

    Science.gov (United States)

    Gormsen, Andreas Brandt; Fraes Diernæs, Jon Erik; Jensen, Jørgen Skov; Koppelhus, Uffe

    2018-05-14

    This is a case report of lymphogranuloma venereum (LGV) manifesting as haemorrhagic proctocolitis in a homosexual HIV-positive male. The primary symptom was a rectal abscess, which was initially surgically treated and subsequently insufficiently treated with single-dose tablet azithromycin. The patient's symptoms were successfully treated after a 21-day doxycycline regime. LGV is a se xually transmitted infection with a rising incidence among persons with risk behaviour. This case report underlines the importance, that all positive rectal screenings for Chlamydia trachomatis should be routinely serotyped.

  11. Treatment of severe refractory epistaxis in hereditary hemorrhagic telangiectasia using a two-flap nasal closure method

    Science.gov (United States)

    Timmins, Benjamin H.; Hunter, Benjamin N.; Wilson, Kevin F.; Ward, P. Daniel

    2016-01-01

    Background Nasal closure has been shown to effectively manage severe epistaxis refractory to other treatments in patients with hereditary hemorrhagic telangiectasia (HHT). The nasal closure procedure may be underutilized due to its surgical complexity and flap breakdown. Methods Retrospective review of thirteen HHT patients treated for severe epistaxis with nasal closure between 2005 and 2013. Operating room (OR) time, need for revision surgery, pre- and post-procedure epistaxis severity score (ESS), complete blood count values, and Glasgow Benefit Inventory (GBI) questionnaire results were collected for each patient. The technique is described. We characterize a typical nasal closure patient and compare outcomes based on our experience with the traditional three-flap closure and a simplified two-flap nasal closure procedure. Results The average candidate for nasal closure in this series had an ESS of 7.88, Hgb of 8.3 g/dL, and received multiple transfusions, iron therapy, and cautery/coagulation procedures. Average ESS subsequent to nasal closure using the two flap method is 0.92 and mean GBI score is 56.3. Comparison of five patients who underwent the traditional three-flap nasal closure procedure and eight patients receiving the two flap nasal closure showed no significant difference in post-op ESS or GBI metrics. Mean operating room times of the traditional and simplified methods were 3.12 hours and 1.44 hours (p=0.0001). Mean time to first revision for eight nasal closure patients is 21.5 months. Conclusion In short-term follow-up, the two-flap procedure has shown comparable effectiveness with significantly reduced complexity and operative time compared to the traditional nasal closure method. PMID:26751606

  12. MRI in acute subarachnoid haemorrhage; findings with a standardised stroke protocol

    International Nuclear Information System (INIS)

    Fiebach, J.B.; Wilde, P.; Meyer, M.; Sartor, K.; Schellinger, P.D.; Hacke, W.; Geletneky, K.

    2004-01-01

    There is doubt as to whether acute haemorrhage is visible on MRI. We carried out MRI within 6 h of symptom onset on five patients with minor (low Hunt and Hess grades 1 or 2) subarachnoid haemorrhage (SAH) diagnosed by CT to search for any specific pattern. We used our standard stroke MRI protocol, including multiecho proton density (PD)- and T2-weighted images, echoplanar (EPI) diffusion- (DWI) and perfusion- (PWI) weighted imaging, and MRA. In all cases SAH was clearly visible on PD-weighted images with a short TE. In four patients it caused a low-signal rim on the T2*-weighted source images of PWI, and DWI revealed high signal in SAH. In the fifth patient SAH was perimesencephalic; susceptibility effects from the skull base made it impossible to detect SAH on EPI DWI and T2*-weighted images. Perfusion maps were normal in all cases. MRA and conventional angiography revealed an aneurysm in only one patient. Stroke MRI within 6 h of SAH thus shows a characteristic pattern. (orig.)

  13. Infrastructure and clinical practice for the detection and management of trauma-associated haemorrhage and coagulopathy.

    Science.gov (United States)

    Driessen, A; Schäfer, N; Albrecht, V; Schenk, M; Fröhlich, M; Stürmer, E K; Maegele, M

    2015-08-01

    Early detection and management of post-traumatic haemorrhage and coagulopathy have been associated with improved outcomes, but local infrastructures, logistics and clinical strategies may differ. To assess local differences in infrastructure, logistics and clinical management of trauma-associated haemorrhage and coagulopathy, we have conducted a web-based survey amongst the delegates to the 15th European Congress of Trauma and Emergency Surgery (ECTES) and the 2nd World Trauma (WT) Congress held in Frankfurt, Germany, 25-27 May 2014. 446/1,540 delegates completed the questionnaire yielding a response rate of 29%. The majority specified to work as consultants/senior physicians (47.3%) in general (36.1%) or trauma/orthopaedic surgery (44.5%) of level I (70%) or level II (19%) trauma centres. Clinical assessment (>80%) and standard coagulation assays (74.6%) are the most frequently used strategies for early detection and monitoring of bleeding trauma patients with coagulopathy. Only 30% of the respondents declared to use extended coagulation assays to better characterise the bleeding and coagulopathy prompted by more individualised treatment concepts. Most trauma centres (69%) have implemented local protocols based on international and national guidelines using conventional blood products, e.g. packed red blood cell concentrates (93.3%), fresh frozen plasma concentrates (93.3%) and platelet concentrates (83%), and antifibrinolytics (100%). 89% considered the continuous intake of anticoagulants including "new oral anticoagulants" and platelet inhibitors as an increasing threat to bleeding trauma patients. This study confirms differences in infrastructure, logistics and clinical practice for the detection and management of trauma-haemorrhage and trauma-associated coagulopathy amongst international centres. Ongoing work will focus on geographical differences.

  14. Lifetime risks for aneurysmal subarachnoid haemorrhage: multivariable risk stratification.

    Science.gov (United States)

    Vlak, Monique H M; Rinkel, Gabriel J E; Greebe, Paut; Greving, Jacoba P; Algra, Ale

    2013-06-01

    The overall incidence of aneurysmal subarachnoid haemorrhage (aSAH) in western populations is around 9 per 100 000 person-years, which confers to a lifetime risk of around half per cent. Risk factors for aSAH are usually expressed as relative risks and suggest that absolute risks vary considerably according to risk factor profiles, but such estimates are lacking. We aimed to estimate incidence and lifetime risks of aSAH according to risk factor profiles. We used data from 250 patients admitted with aSAH and 574 sex-matched and age-matched controls, who were randomly retrieved from general practitioners files. We determined independent prognostic factors with multivariable logistic regression analyses and assessed discriminatory performance using the area under the receiver operating characteristic curve. Based on the prognostic model we predicted incidences and lifetime risks of aSAH for different risk factor profiles. The four strongest independent predictors for aSAH, namely current smoking (OR 6.0; 95% CI 4.1 to 8.6), a positive family history for aSAH (4.0; 95% CI 2.3 to 7.0), hypertension (2.4; 95% CI 1.5 to 3.8) and hypercholesterolaemia (0.2; 95% CI 0.1 to 0.4), were used in the final prediction model. This model had an area under the receiver operating characteristic curve of 0.73 (95% CI 0.69 to 0.76). Depending on sex, age and the four predictors, the incidence of aSAH ranged from 0.4/100 000 to 298/100 000 person-years and lifetime risk between 0.02% and 7.2%. The incidence and lifetime risk of aSAH in the general population varies widely according to risk factor profiles. Whether persons with high risks benefit from screening should be assessed in cost-effectiveness studies.

  15. Regulatory T cells ameliorate tissue plasminogen activator-induced brain haemorrhage after stroke.

    Science.gov (United States)

    Mao, Leilei; Li, Peiying; Zhu, Wen; Cai, Wei; Liu, Zongjian; Wang, Yanling; Luo, Wenli; Stetler, Ruth A; Leak, Rehana K; Yu, Weifeng; Gao, Yanqin; Chen, Jun; Chen, Gang; Hu, Xiaoming

    2017-07-01

    Delayed thrombolytic treatment with recombinant tissue plasminogen activator (tPA) may exacerbate blood-brain barrier breakdown after ischaemic stroke and lead to lethal haemorrhagic transformation. The immune system is a dynamic modulator of stroke response, and excessive immune cell accumulation in the cerebral vasculature is associated with compromised integrity of the blood-brain barrier. We previously reported that regulatory T cells, which function to suppress excessive immune responses, ameliorated blood-brain barrier damage after cerebral ischaemia. This study assessed the impact of regulatory T cells in the context of tPA-induced brain haemorrhage and investigated the underlying mechanisms of action. The number of circulating regulatory T cells in stroke patients was dramatically reduced soon after stroke onset (84 acute ischaemic stroke patients with or without intravenous tPA treatment, compared to 115 age and gender-matched healthy controls). Although stroke patients without tPA treatment gradually repopulated the numbers of circulating regulatory T cells within the first 7 days after stroke, post-ischaemic tPA treatment led to sustained suppression of regulatory T cells in the blood. We then used the murine suture and embolic middle cerebral artery occlusion models of stroke to investigate the therapeutic potential of adoptive regulatory T cell transfer against tPA-induced haemorrhagic transformation. Delayed administration of tPA (10 mg/kg) resulted in haemorrhagic transformation in the ischaemic territory 1 day after ischaemia. When regulatory T cells (2 × 106/mouse) were intravenously administered immediately after delayed tPA treatment in ischaemic mice, haemorrhagic transformation was significantly decreased, and this was associated with improved sensorimotor functions. Blood-brain barrier disruption and tight junction damages were observed in the presence of delayed tPA after stroke, but were mitigated by regulatory T cell transfer. Mechanistic

  16. Intra-arterial nimodipine for cerebral vasospasm after subarachnoid haemorrhage

    DEFF Research Database (Denmark)

    Bashir, Asma; Andresen, Morten; Bartek, Jiri

    2016-01-01

    Intra-arterial nimodipine (IAN) has shown a promising effect on cerebral vasospasm (CV) after aneurysmal subarachnoid haemorrhage. At our institution, Rigshospitalet, IAN treatment has been used since 2009, but the short- and long-term clinical efficacy of IAN has not yet been assessed. The purpo...

  17. Review of Primary Postpartum Haemorrhage in Sagamu, Nigeria ...

    African Journals Online (AJOL)

    Out of these deliveries, 76 had primary postpartum haemorrhage (PPH), giving a prevalence of 3.1%. Uterine atony and genital tract trauma were the main causes of the primary PPH. Associated factors were prolonged second and third stages of labour, induction and augmentation of labour with oxytocin and instrumental ...

  18. New trends in the management of postpartum haemorrhage | Dyer ...

    African Journals Online (AJOL)

    New trends in the management of postpartum haemorrhage. ... Africa is poor access to basic obstetric care, blood products and basic commodities, such as electricity, for the refrigeration of blood and drugs such as oxytocin.1 Nevertheless, there are many areas where management, and hence outcomes, could be improved.

  19. Expression of VP60 gene from rabbit haemorrhagic disease virus ...

    African Journals Online (AJOL)

    The VP60 gene from rabbit haemorrhagic disease virus (RHDV) YL strain in Northeast of China, under control of the ats1A promoter from Rubisco small subunit genes of Arabidopsis thaliana, was introduced into the transfer deoxyribonucleic acid (T-DNA) region of plant transfer vector pCAMBIA1300 and transferred to ...

  20. Haemorrhagic Vaginal Discharge Following Ovariectomy in a Three ...

    African Journals Online (AJOL)

    Haemorrhagic Vaginal Discharge Following Ovariectomy in a Three Year Old Domestic Short-haired Cat. RA Ajadi, OO Adebayo, TA Ajadi. Abstract. Nigerian Veterinary Journal, VOL:33 (1) 403-406. Full Text: EMAIL FREE FULL TEXT EMAIL FREE FULL TEXT · DOWNLOAD FULL TEXT DOWNLOAD FULL TEXT.

  1. Risk of transmission of viral haemorrhagic fevers and the insecticide ...

    African Journals Online (AJOL)

    ... of transmission of viral haemorrhagic fevers and the insecticide susceptibility status of Ae. aegypti in some sites in Accra, Ghana. Design: Larval surveys were carried to inspect containers within households and estimate larval indices and adult Aedes mosquitoes were collected using human landing collection technique.

  2. Prenatal MR imaging features of isolated cerebellar haemorrhagic lesions

    International Nuclear Information System (INIS)

    Martino, Francesca; Malova, Mariya; Ramenghi, Luca A.; Cesaretti, Claudia; Parazzini, Cecilia; Doneda, Chiara; Righini, Andrea; Rossi, Andrea

    2016-01-01

    Prenatal features of isolated cerebellar haemorrhagic lesions have not been sufficiently characterised. We aimed to better define their MR imaging characteristics, documenting the location, extension, evolution stage and anatomic sequelae, and to better understand cerebellar haemorrhage pathophysiology. We screened our foetal MR imaging database (3200 cases) for reports of haemorrhagic lesions affecting only the cerebellum (without any supratentorial bleeding or other clastic lesions), defined as one of the following: T2-weighted hypointense or mixed hypo-/hyperintense signal; rim of T2-weighted hypointense signal covering the surface of volume-reduced parenchyma; T1-weighted hyperintense signal; increased DWI signal. Seventeen cases corresponded to the selection criteria. All lesions occurred before the 26th week of gestation, with prevalent origin from the peripheral-caudal portion of the hemispheres and equal frequency of unilateral/bilateral involvement. The caudal vermis appeared affected in 2/3 of cases, not in all cases confirmed postnatally. Lesions evolved towards malformed cerebellar foliation. The aetiology and pathophysiology were unknown, although in a subset of cases intra- and extracranial venous engorgement seemed to play a key role. Onset from the peripheral and caudal portion of the hemispheres seems characteristic of prenatal cerebellar haemorrhagic lesions. Elective involvement of the peripheral germinal matrix is hypothesised. (orig.)

  3. Periventricular-intraventricular haemorrhage in low-birth-weight ...

    African Journals Online (AJOL)

    The prevalence of periventricular-intraventricular haemorrhage (PV-IVH) aInong very-low-birthweight infants at Baragwanath Hospital has not been well docwnented. In this prospective study, a total of 282 live-born infants with birth weights of 1 000 - 1 749 g were studied over a 41/2-month period. Every infant had at least ...

  4. Considerable delay in diagnosis and acute management of subarachnoid haemorrhage

    DEFF Research Database (Denmark)

    Larsen, Carl Christian; Eskesen, Vagn; Hauerberg, John

    2010-01-01

    Rebleeding from subarachnoid haemorrhage (SAH) usually occurs within the first six hours after the initial bleeding. Rebleeding can be prevented effectively with tranexamic acid (TXA). Although a broad consensus has evolved that SAH should be treated as an emergency, it is likely that delays do...

  5. Marburg haemorrhagic fever: A rare but fatal disease

    African Journals Online (AJOL)

    The causative virus is the Marburgvirus of the Filoviridae family. The disease is clinically indistinguishable from Ebola haemorrhagic fever though the latter's causative agent is unrelated. Transmission of the Marburgvirus is via close contact with blood or other body fluids (faeces, vomitus, urine and respiratory secretions) ...

  6. Post-tonsillectomy haemorrhage following traditional uvulectomy in ...

    African Journals Online (AJOL)

    routinely performed in children due to the traditional belief that an elongated uvula is responsible for all throat problems, including suffocation during sleep in the neonatal period. Occasionally, it is done during ethnic identification ritual practices.2 The commonest complications following this procedure include haemorrhage,.

  7. Life threatening vaginal haemorrhage from coital laceration in a post ...

    African Journals Online (AJOL)

    Case report: A 55-year-old woman who presented as an emergency with haemorrhagic shock following vaginal bleeding from consensual coitus is presented. She was resuscitated with intravenous fluids and blood transfusions. The vaginal laceration of about 5 cm on the right side of the posterior fornix was sutured ...

  8. Haemorrhagic pseudocyst of the pancreatic tail causing acute ...

    African Journals Online (AJOL)

    Haemorrhagic pseudocyst of the pancreatic tail causing acute abdominal pain in a 12-year-old girl. Rolf P. Dahmen a,c. , Gerhard Stuhldreier b. , Hartmut Bindewald c and Malte Weinrich a,c. Pancreatic disorders are a relatively uncommon event in children, particularly the development of pancreatic pseudocysts. The most ...

  9. The role of interventional radiology in reducing haemorrhage and hysterectomy following caesarean section for morbidly adherent placenta

    International Nuclear Information System (INIS)

    Teixidor Viñas, M.; Chandraharan, E.; Moneta, M.V.; Belli, A.M.

    2014-01-01

    Aim: To report experience of prophylactic occlusion balloon catheters (POBCs) in both internal iliac arteries before caesarean section, with or without embolization, to preserve the uterus and reduce haemorrhage. Methods and materials: Twenty-seven women diagnosed with morbidly adherent placenta (MAP) and with suspected placenta percreta underwent POBC placement before caesarean section. The balloons were inflated immediately after delivery of the baby. The patients' case notes were reviewed retrospectively for histological grading of MAP, blood loss, transfusion, requirement of uterine artery embolization (UAE), or hysterectomy, radiation dose, and infant or maternal morbidity and mortality. Results: MAP was confirmed histologically as percreta in 17, accreta in eight, and increta in two women. Mean blood loss was 1.92 l (range 0.5–12 l). Postpartum haemorrhage (PPH) occurred in nine patients. Eight were referred for UAE, which was successful in six. Immediate peri-partum hysterectomy was performed in one patient. Three women in total required hysterectomy, two after recurrent haemorrhage after UAE. No foetal morbidity or mortality occurred. No maternal mortality occurred. There was one case of iliac artery thrombosis, which resolved with conservative therapy. Conclusion: POBC, with or without UAE, contributes to reduction of blood loss and preservation of the uterus in women with MAP. - Highlights: • Management of morbidly adherent placenta requires a multidisciplinary team approach. • Prophylactic occlusion balloon catheters reduce blood loss and help avoid hysterectomy. • Protocols ensure correct management of placenta percreta patients and minimise risk

  10. Ionizing radiation-induced phosphorylation of RPA p34 is deficient in ataxia telangiectasia and reduced in aged normal fibroblasts

    International Nuclear Information System (INIS)

    Xinbo Cheng; Nge Cheong; Ya Wang; Iliakis, George

    1996-01-01

    Replication protein A (RPA, also called human single stranded DNA binding protein, hSSB) is a trimeric, multifunctional protein complex involved in DNA replication, DNA repair and recombination. Phosphorylation of RPA p34 subunit is observed after exposure of cells to radiation and other DNA damaging agents, which implicates the protein not only in repair but also in the regulation of replication on damaged DNA template. Here, we show that the phosphorylation observed in RPA p34 after exposure to ionizing radiation, X- or γ-rays, is reduced and occurs later in primary fibroblasts from patients suffering from ataxia telangiectasia (AT), as compared to normal fibroblasts. We also show that in primary normal human fibroblasts, radiation-induced phosphorylation of RPA p34 is 'age'-dependent and decreases significantly as cultures senesce. Radiation-induced phosphorylation of RPA p34 is nearly absent in non-cycling cells, while the expression of p21 cip1/waf1/sdi1 remains inducible. The results demonstrate a growth-stage and culture-age dependency in radiation-induced RPA p34 phosphorylation, and suggest the operation of a signal transduction pathway that is inactivated in senescing or quiescent fibroblasts and defective in AT cells

  11. Abnormal levels of UV-induced unscheduled DNA synthesis in ataxia telangiectasia cells after exposure to ionizing radiation

    International Nuclear Information System (INIS)

    Jaspers, N.G.J.; Nederlandse Centrale Organisatie voor Toegepast Natuurwetenschappelijk Onderzoek, Rijswijk. Medical Biological Lab.); Bootsma, D.

    1982-01-01

    In cultured cells from normal individuals and from patients having ataxia telangiectasia (AT) the rate of unscheduled DNA synthesis (UDS) induced by UV light was investigated by autoradiography. The number of grains in 6 different AT cell strains was similar to that observed in normal cells. Exposure of normal cells to doses of X-rays up to 20 krad had no influence on the rate of UV-induced UDS. In contrast, the UV-induced UDS was significantly modified in AT cells by treatment with X-rays. In AT cell strains that were reported to have reduced levels of γ-ray-induced repair DNA synthesis ('excision-deficient' AT cells) the effect of X-rays on UV-induced UDS was inhibitory, whereas UV-induced UDS was stimulated by X-ray exposure in 'excision-proficient' AT cell strains. Different UV and X-ray dose-response relationships were seen in the two categories of AT cell strains. (orig./AJ)

  12. The effect of caffeine on X-ray-induced mitotic delay in normal human and ataxia-telangiectasia fibroblasts

    International Nuclear Information System (INIS)

    Zampetti-Bosseler, F.; Scott, D.

    1985-01-01

    The authors previously showed that radiation-sensitive fibroblasts from ataxia-telangiectasia (A-T) patients sustain less G 2 delay after X-irradiation than normal fibroblasts. Caffeine is known to reduce the amount of X-ray-induced delay in various mammalian cell types. It is proposed that A-T cells have an altered chromatin structure, similar to that of caffeine-treated normal cells and that this results in a failure of A-T cells to delay their progression through the cell cycle to allow time for DNA repair. The authors now show that caffeine treatment after X-irradiation reduces G 2 delay in both A-T and normal cells. The authors confirm the results previously obtained on lymphocytes that caffeine potentiates the chromosome-damaging effects of X-rays in both A-T and normal fibroblasts. These and other data suggest that the radiation responses of A-T cells and of caffeine-treated normal cells are caused by different mechanisms. (Auth.)

  13. Treatment of Non-neovascular Idiopathic Macular Telangiectasia Type 2 with Intravitreal Ranibizumab: Results of a Phase II Clinical Trial

    Science.gov (United States)

    Toy, Brian C.; Koo, Euna; Cukras, Catherine; Meyerle, Catherine B.; Chew, Emily Y.; Wong, Wai T.

    2015-01-01

    Purpose To evaluate the safety and preliminary efficacy of intravitreal ranibizumab for non-neovascular idiopathic macular telangiectasia, type 2 (IMT2). Methods Single-center, open-label phase II clinical trial enrolling 5 participants with bilateral non-neovascular IMT2. Intravitreal ranibizumab (0.5mg) was administered every 4 weeks in the study eye for 12 months with the contralateral eye observed. Outcome measures included changes in: best corrected visual acuity (BCVA), area of late-phase leakage on fluorescein angiography (FA), and retinal thickness on optical coherence tomography (OCT). Results The study treatment was well-tolerated and associated with few adverse events. Change in BCVA at 12 months was not significantly different between treated study eyes (0.0±7.5 letters) and control fellow eyes (+2.2±1.9 letters). However, decreases in the area of late-phase FA leakage (−33±20% for study eyes, +1±8% for fellow eyes) and in OCT central subfield retinal thickness (−11.7±7.0% for study eyes and −2.9±3.5% for fellow eyes) were greater in study eyes compared to fellow eyes. Conclusions Despite significant anatomical responses to treatment, functional improvement in visual acuity was not detected. Intravitreal ranibizumab administered monthly over a time course of 12 months is unlikely to provide a general and significant benefit to patients with non-neovascular IMT2. PMID:22266930

  14. Non-fatal case of Crimean-Congo haemorrhagic fever imported into the United Kingdom (ex Bulgaria), June 2014.

    Science.gov (United States)

    Lumley, S; Atkinson, B; Dowall, Sd; Pitman, Jk; Staplehurst, S; Busuttil, J; Simpson, Aj; Aarons, Ej; Petridou, C; Nijjar, M; Glover, S; Brooks, Tj; Hewson, R

    2014-07-31

    Crimean-Congo haemorrhagic fever (CCHF) was diagnosed in a United Kingdom traveller who returned from Bulgaria in June 2014. The patient developed a moderately severe disease including fever, headaches and petechial rash. CCHF was diagnosed following identification of CCHF virus (CCHFV) RNA in a serum sample taken five days after symptom onset. Sequence analysis of the CCHFV genome showed that the virus clusters within the Europe 1 clade, which includes viruses from eastern Europe.

  15. Uterine massage for preventing postpartum haemorrhage.

    Science.gov (United States)

    Hofmeyr, G Justus; Abdel-Aleem, Hany; Abdel-Aleem, Mahmoud A

    2013-07-01

    Postpartum haemorrhage (PPH) (bleeding from the genital tract after childbirth) is a major cause of maternal mortality and disability, particularly in under-resourced areas. In these settings, uterotonics are often not accessible. There is a need for simple, inexpensive techniques which can be applied in low-resourced settings to prevent and treat PPH. Uterine massage is recommended as part of the routine active management of the third stage of labour. However, it is not known whether it is effective. If shown to be effective, uterine massage would represent a simple intervention with the potential to have a major effect on PPH and maternal mortality in under-resourced settings. To determine the effectiveness of uterine massage after birth and before or after delivery of the placenta, or both, to reduce postpartum blood loss and associated morbidity and mortality. We searched the Cochrane Pregnancy and Childbirth Group's Trials Register (30 April 2013). All published, unpublished and ongoing randomised controlled trials comparing uterine massage alone or in addition to uterotonics before or after delivery of the placenta, or both, with non-massage. Two researchers independently considered trials for eligibility, assessed risk of bias and extracted the data using the agreed form. Data were checked for accuracy. The effect of uterine massage commenced before or after placental delivery were first assessed separately, and then the combined for an overall result. This review included two randomised controlled trials. The first trial included 200 women who were randomised to receive uterine massage or no massage following delivery of the placenta, after active management of the third stage of labour including use of oxytocin. The numbers of women with blood loss more than 500 mL was small, with no statistically significant difference (risk ratio (RR) 0.52, 95% confidence interval (CI) 0.16 to 1.67). There were no cases of retained placenta in either group. The mean

  16. Severe gastric variceal haemorrhage due to splenic artery thrombosis and consecutive arterial bypass

    Directory of Open Access Journals (Sweden)

    Wasmuth Hermann E

    2011-06-01

    Full Text Available Abstract Background Upper gastrointestinal haemorrhage is mainly caused by ulcers. Gastric varicosis due to portal hypertension can also be held responsible for upper gastrointestinal bleeding. Portal hypertension causes the development of a collateral circulation from the portal to the caval venous system resulting in development of oesophageal and gastric fundus varices. Those may also be held responsible for upper gastrointestinal haemorrhage. Case presentation In this study, we describe the case of a 69-year-old male with recurrent severe upper gastrointestinal bleeding caused by arterial submucosal collaterals due to idiopathic splenic artery thrombosis. The diagnosis was secured using endoscopic duplex ultrasound and angiography. The patient was successfully treated with a laparoscopic splenectomy and complete dissection of the short gastric arteries, resulting in the collapse of the submucosal arteries in the gastric wall. Follow-up gastroscopy was performed on the 12th postoperative week and showed no signs of bleeding and a significant reduction in the arterial blood flow within the gastric wall. Subsequent follow-up after 6 months also showed no further gastrointestinal bleeding as well as subjective good quality of life for the patient. Conclusion Submucosal arterial collaterals must be excluded by endosonography via endoscopy in case of recurrent upper gastrointestinal bleeding. Laparoscopic splenectomy provides adequate treatment in preventing any recurrent bleeding, if gastric arterial collaterals are caused by splenic artery thrombosis.

  17. T1-weighted MRI for the detection of coronary artery plaque haemorrhage

    International Nuclear Information System (INIS)

    Oei, May Lin; Ozgun, Murat; Seifarth, Harald; Bunck, Alexander; Fischbach, Roman; Heindel, Walter; Maintz, David; Orwat, Stefan; Botnar, Rene

    2010-01-01

    Hyperintense areas in atherosclerotic plaques on pre-contrast T1-weighted MRI have been shown to correlate with intraplaque haemorrhage. We evaluated the presence of T1 hyperintensity in coronary artery plaques in coronary artery disease (CAD) patients and correlated results with multi-detector computed tomography (MDCT) findings. Fifteen patients with CAD were included. Plaques detected by MDCT were categorised based on their Hounsfield number. T1-weighted inversion recovery (IR) MRI prepared coronary MRI for the detection of plaque and steady-state free-precession coronary MR-angiography for anatomical correlation was performed. After registration of MDCT and MRI, regions of interest were defined on MDCT-visible plaques and in corresponding vessel segments acquired with MRI. MDCT density and MR signal measurement were performed in each plaque. Forty-three plaques were identified with MDCT. With IR-MRI 5/43 (12%) plaques were hyperintense, 2 of which were non-calcified and 3 mixed. Average signal-to-noise and contrast-to-noise ratios of hyperintense plaques were 15.7 and 9.1, compared with 5.6 and 1.2 for hypointense plaques. Hyperintense plaques exhibited a significantly lower CT density than hypointense plaques (63.6 vs. 140.8). There was no correlation of plaque signal intensity with degree of stenosis. T1-weighted IR-MRI may be useful for non-invasive detection and characterisation of intraplaque haemorrhage in coronary artery plaques. (orig.)

  18. Acute Primary Haemorrhagic Omental Torsion Mimicking Perforated Appendicitis: An Unorthodox Surgical Paradox

    International Nuclear Information System (INIS)

    Rehman, A.

    2014-01-01

    Acute primary haemorrhagic omental torsion is an atypical and deceptive cause of acute abdomen that could closely mimic a myriad of intra-abdominal catastrophes, especially perforated appendicitis. The author reports a 30 years man who had presented with gradually worsening right-sided abdominal pain of 2 days duration. Laboratory work-up and abdominal radiographs were inconclusive. Abdominal sonography detected presence of free fluid in the pelvic cul-de-sac. Based on clinical and sonographic findings, presumptive diagnosis of perforated appendicitis was made and the patient was explored through extended Rockey-Davis incision. About 500 - 700 ml of dark-coloured blood (haemoperitoneum) was present in the peritoneal cavity and the pelvis secondary to acute haemorrhagic omental torsion. The appendix was grossly normal. Omentectomy and prophylactic appendicectomy resulted in uneventful recovery of the patient. Acute primary omental torsion is an uncommon pathology that must be kept in mind during differential diagnosis of acute abdomen, especially acute or perforated appendicitis. (author)

  19. Dose-time relationships for post-irradiation cutaneous telangiectasia

    International Nuclear Information System (INIS)

    Cohen, L.; Ubaldi, S.E.

    1977-01-01

    Seventy-five patients who had received electron beam radiation a year or more previously were studied. The irradiated skin portals were photographed and late reactions graded in terms of the number and severity of telangiectatic lesions observed. The skin dose, number of fractions, overall treatment time and irradiated volume were recorded in each case. A Strandqvist-type iso-effect line was derived for this response. A multi-probit search program also was used to derive best-fitting cell population kinetic parameters for the same data. From these parameters a comprehensive iso-effect table could be computed for a wide range of treatment schedules including daily treatment as well as fractionation at shorter and longer intervals; this provided a useful set of normal tissue tolerance limits for late effects

  20. DNA-mediated gene transfer into human diploid fibroblasts derived from normal and ataxia-telangiectasia donors: parameters for DNA transfer and properties of DNA transformants

    International Nuclear Information System (INIS)

    Debenham, P.G.; Webb, M.B.T.; Masson, W.K.; Cox, R.

    1984-01-01

    An investigation was made of the feasibility of DNA-mediated gene transfer into human diploid fibroblasts derived from patients with the radiation sensitive syndrome ataxia-telangiectasia (A-T) and from a normal donor. Although they are markedly different in their growth characteristics, both normal and A-T strains give similar frequencies for DNA transfer in a model system using the recombinant plasmid pSV2-gpt. pSV2-gpt DNA transformants arise with a frequency between 10 -5 and 10 -4 per viable cell. Analysis of such transformants, although possible, is severely handicapped by the limited clonal life span of diploid human cells. Despite these problems it may be concluded that diploid human fibroblasts are competent recipients for DNA-mediated gene transfer and the putative repair deficiency of A-T does not markedly effect the efficiency of this process. (author)

  1. Excision repair in ataxia telangiectasia, Fanconi's anemia, Cockayne syndrome, and Bloom's syndrome after treatment with ultraviolet radiation and N-acetoxy-2-acetylaminofluorene

    International Nuclear Information System (INIS)

    Ahmed, F.E.; Setlow, R.B.

    1978-01-01

    Excision repair of damage due to ultraviolet radiation, N-acetoxy-2-acetylaminofluorene and a combination of both agents was studied in normal human fibroblasts and various cells from cancer prone patients (ataxia telangiectasia, Fanconi's anemia, Cockayne syndrome and Bloom's syndrome). Three methods giving similar results were used: unscheduled DNA synthesis by radioautography, photolysis of bromodeoxyuridine incorporated into parental DNA during repair, and loss of sites sensitive to an ultraviolet endonuclease. All cell lines were proficient in repair of ultraviolet and acetoxy acetylaminofluorene damage and at saturation doses of both agents repair was additive. We interpret these data as indicating that the rate limiting step in excision repair of ultraviolet and acetoxy acetylaminofluorene is different and that there are different enzyme(s) working on incision of both types of damages. (Auth.)

  2. Major obstetric haemorrhage of 2000 ml or greater: a clinical audit.

    Science.gov (United States)

    O'Sullivan, J; Mansfield, R; Talbot, R; Cairns, A E

    2018-05-04

    transfusion), and raises questions about what constitutes optimum PPH management. What the implications are of these findings for clinical practice and/or further research? The primary and secondary transfusion data raised new questions to investigate in the future: does the involvement of consultants and the escalation of care via the instigation of major haemorrhage protocols improve decision-making and patient outcomes? Does the necessity for a secondary transfusion indicate a suboptimal acute care?

  3. Diagnostic yield and accuracy of CT angiography, MR angiography, and digital subtraction angiography for detection of macrovascular causes of intracerebral haemorrhage: Prospective, multicentre cohort study

    NARCIS (Netherlands)

    C.J.J. Van Asch (Charlotte J.J.); B.K. Velthuis (Birgitta K.); G.J.E. Rinkel (Gabriël J.E.); A. Algra (Ale); G.A.P. de Kort (G. A P); T.D. Witkamp (Theo); J.C.M. De Ridder (Johanna C.M.); K.M. Van Nieuwenhuizen (Koen M.); F.-E. De Leeuw (Frank-Erik); W.J. Schonewille (Wouter); P.L.M. de Kort (Paul); D.W.J. Dippel (Diederik); T.W.M. Raaymakers (Theodora W.M.); J. Hofmeijer; M.J.H. Wermer (Marieke); H. Kerkhoff (Henk); K. Jellema (Korné); I.M. Bronner (Irene M.); M.J.M. Remmers (Michel ); H.P. Bienfait (Henri); R.J.G.M. Witjes (Ron J.G.M.); J.P. Greving (Jacoba); C.J.M. Klijn (Catharina J.M.); H.F. de Leeuw (Frank); H.B. Boogaarts; E.J. van Dijk (Ewoud); W.J. Schonewille; W.M.J. Pellikaan; C. Puppels-De Waard; P.L.M. De Kort; J.P. Peluso; J. van Tuijl (Jordie); J. Hofmeijer; F.B.M. Joosten (Frank); D.W.J. Dippel (Diederik); L. Khajeh (Ladbon); T.W.M. Raaijmakers; M.J. Wermer; M.A.A. van Walderveen (Marianne); H. Kerkhoff; E. Zock; K. Jellema (Korné); G.J. Lycklama à Nijeholt (Geert); I.M. Bronner; M.J.M. Remmers; R.J.G.M. Witjes; H.P. Bienfait; K.E. Droogh-Greve; R. Donders (Rogier); V.I.H. Kwa; T.H.C.M.L. Schreuder (Tobien H. C. M. L.); C.L. Franke (Cees); J.S. Straver; C. Jansen; S.L.M. Bakker (Stef); C.C. Pleiter (C.); M.C. Visser; C.J.J. Van Asch; B.K. Velthuis (Birgitta); G.J.E. Rinkel (Gabriel); K.M. Van Nieuwenhuizen; C.J.M. Klijn (Catharina J.M.)

    2015-01-01

    textabstractStudy question What are the diagnostic yield and accuracy of early computed tomography (CT) angiography followed by magnetic resonance imaging/angiography (MRI/MRA) and digital subtraction angiography (DSA) in patients with non-traumatic intracerebral haemorrhage? Methods This

  4. A prospective cohort study evaluating the cost-effectiveness of carbetocin for prevention of postpartum haemorrhage in caesarean sections.

    Science.gov (United States)

    Luni, Yasmin; Borakati, Aditya; Matah, Arti; Skeats, Katie; Eedarapalli, Padma

    2017-07-01

    Postpartum haemorrhage (PPH) is the leading cause of maternal mortality worldwide. Prophylaxis with oxytocic medication is recommended by the WHO to prevent its occurrence. Carbetocin is a newer oxytocic, with potential to lower PPH rates, reduce the total use of oxytocic drugs and lead to financial savings. Meta-analyses have confirmed a reduction in the use of additional oxytocic medication with the use of carbetocin compared to oxytocin. However, there are few studies evaluating the costs of carbetocin prophylaxis. We carried out a prospective cohort study evaluating the financial impact of carbetocin, following its introduction at our centre for caesarean section. We collected data for 400 patients in total, making this, to our knowledge, the largest study conducted on this topic. We found a significant reduction in PPH rates and the use of additional oxytocics with projected overall financial savings of £68.93 per patient with the use of carbetocin. Impact statement It is well established that carbetocin reduces the use of secondary oxytocics compared to oxytocin alone in the active management of the third stage of labour. Evidence for reduction of post-partum haemorrhage and its cost effectiveness are more equivocal. Our study demonstrates that carbetocin also reduces post-partum haemorrhage, use of blood and blood products and midwifery recovery time in the setting of caesarean section. We have also demonstrated that despite the increased index cost of carbetocin it delivers an overall substantial cost benefit. The implications of these findings are of reduced morbidity, faster recovery and cost savings in these times of austerity in the UK. It allows more efficient labour distribution of midwives, particularly in the setting of staff shortages across the NHS. A randomised control trial in this area needs to be conducted to determine the cost benefit of carbetocin and with this and post-partum haemorrhage rates as the primary outcome measures.

  5. Multimodality imaging in macular telangiectasia 2: A clue to its pathogenesis

    Directory of Open Access Journals (Sweden)

    Lihteh Wu

    2015-01-01

    Full Text Available Macular telangiectasia type 2 also known as idiopathic perifoveal telangiectasia and juxtafoveolar retinal telangiectasis type 2A is an acquired bilateral neurodegenerative macular disease that manifests itself during the fifth or sixth decades of life. It is characterized by minimal dilatation of the parafoveal capillaries with graying of the retinal area involved, a lack of lipid exudation, right-angled retinal venules, refractile deposits in the superficial retina, hyperplasia of the retinal pigment epithelium, foveal atrophy, and subretinal neovascularization (SRNV. Our understanding of the disease has paralleled advances in multimodality imaging of the fundus. Optical coherence tomography (OCT images typically demonstrate the presence of intraretinal hyporeflective spaces that are usually not related to retinal thickening or fluorescein leakage. The typical fluorescein angiographic (FA finding is a deep intraretinal hyperfluorescent staining in the temporal parafoveal area. With time, the staining may involve the whole parafoveal area but does not extend to the center of the fovea. Long-term prognosis for central vision is poor, because of the development of SRNV or macular atrophy. Its pathogenesis remains unclear but multimodality imaging with FA, spectral domain OCT, adaptive optics, confocal blue reflectance and short wave fundus autofluorescence implicate Müller cells and macular pigment. Currently, there is no known treatment for this condition.

  6. Neurodegeneration in ataxia-telangiectasia: Multiple roles of ATM kinase in cellular homeostasis.

    Science.gov (United States)

    Choy, Kay Rui; Watters, Dianne J

    2018-01-01

    Ataxia-telangiectasia (A-T) is characterized by neuronal degeneration, cancer, diabetes, immune deficiency, and increased sensitivity to ionizing radiation. A-T is attributed to the deficiency of the protein kinase coded by the ATM (ataxia-telangiectasia mutated) gene. ATM is a sensor of DNA double-strand breaks (DSBs) and signals to cell cycle checkpoints and the DNA repair machinery. ATM phosphorylates numerous substrates and activates many cell-signaling pathways. There has been considerable debate about whether a defective DNA damage response is causative of the neurological aspects of the disease. In proliferating cells, ATM is localized mainly in the nucleus; however, in postmitotic cells such as neurons, ATM is mostly cytoplasmic. Recent studies reveal an increasing number of roles for ATM in the cytoplasm, including activation by oxidative stress. ATM associates with organelles including mitochondria and peroxisomes, both sources of reactive oxygen species (ROS), which have been implicated in neurodegenerative diseases and aging. ATM is also associated with synaptic vesicles and has a role in regulating cellular homeostasis and autophagy. The cytoplasmic roles of ATM provide a new perspective on the neurodegenerative process in A-T. This review will examine the expanding roles of ATM in cellular homeostasis and relate these functions to the complex A-T phenotype. Developmental Dynamics 247:33-46, 2018. © 2017 Wiley Periodicals, Inc. © 2017 Wiley Periodicals, Inc.

  7. The effect of tranexamic acid on the risk of death and hysterectomy in women with post-partum haemorrhage: statistical analysis plan for the WOMAN trial.

    Science.gov (United States)

    Shakur, Haleema; Roberts, Ian; Edwards, Philip; Elbourne, Diana; Alfirevic, Zarko; Ronsmans, Carine

    2016-05-17

    Severe haemorrhage is a leading cause of maternal death worldwide. Most haemorrhage deaths occur soon after childbirth. Severe post-partum bleeding is sometimes managed by the surgical removal of the uterus (hysterectomy). Death and hysterectomy are important health consequences of post-partum haemorrhage, and clinical trials of interventions aimed at preventing these outcomes are needed. The World Maternal Antifibrinolytic trial aims to determine the effect of tranexamic acid on death, hysterectomy and other health outcomes in women with post-partum haemorrhage. It is an international, multicentre, randomised trial. Approximately 20,000 women with post-partum haemorrhage will be randomly allocated to receive an intravenous injection of either tranexamic acid or matching placebo in addition to usual care. The primary outcome measure is a composite of death in hospital or hysterectomy within 42 days of delivery. The cause of death will be described. Secondary outcomes include death, death due to bleeding, hysterectomy, thromboembolic events, blood transfusion, surgical and radiological interventions, complications, adverse events and quality of life. The health status and occurrence of thromboembolic events in breastfed babies will also be reported. We will conduct subgroup analyses for the primary outcome by time to treatment, type of delivery and cause of haemorrhage. We will conduct an analysis of treatment effect adjusted for baseline risk. The World Maternal Antifibrinolytic trial should provide reliable evidence for the efficacy of tranexamic acid in the prevention of death, hysterectomy and other outcomes that are important to patients. We present a protocol update and the statistical analysis plan for the trial. Current Controlled Trials ISRCTN76912190 (Registration date 08 December 2008), Clinicaltrials.gov NCT00872469 (Registration date 30 March 2009) and Pan African Clinical Trials Registry: PACTR201007000192283 (Registration date 02 September 2010).

  8. FETOMATERNAL HAEMORRHAGE – DIFFERENTIAL DIAGNOSIS – CASE REPORT

    Directory of Open Access Journals (Sweden)

    Jadranka Domazet-Fink

    2002-07-01

    Full Text Available Background. There are several different causes for fetomaternal haemorrhage. Sinusoidal pattern, which is relatively characteristic for fetal anaemia, may in its final stages completely disappear. Differential diagnosis of silent trace of cardiotocogram with late decelerations is quite difficult to solve.Case report. A case of unusual injury of a pregnant woman is described. The injury led to fetomaternal haemorrhage. Typical sinusoidal pattern cardiotocogram was not seen and the differential diagnosis was hard to determine. Because of prompt intervention and efficient postnatal therapy the child and mother are both well. The dilemmas in regards to differential diagnosis are being discussed.Conclusions. It is important to be very careful in history taking even if the situation is urgent. If there is no explanation for pathological cardiotocogram, decision must be made according to pregnant woman’s wishes. Kleihauer-Betke test, which is easy to perform and gives much information, is described.

  9. Clinical presentation of late haemorrhagic disease of newborn

    International Nuclear Information System (INIS)

    Majeed, R.; Memon, Y.; Majeed, F.

    2008-01-01

    To observe the clinical presentation of late haemorrhagic disease of the newborn (LHDNB), and clinical improvement after the administration of vitamin K/sub 1/. This is a prospective descriptive study. All the children older than seven days who presented with bleeding were admitted in pediatrics ward of Isra University Hyderabad from April 2006 to April 2007 were included. Data collection was done by means of detailed proforma. Analysis was done on SPSS version 11. Thirty five cases were included. Commonest site of bleeding was subcutaneous followed by oral and injection site. Mean age of late haemorrhagic disease of newborn was 109 days and minimum age of presentation was 28 days. Common clinical presentations were irritability, convulsions, poor reflexes and poor feeding. Mostly recovery was within 24 hours after vit K. Late HDN results in severe hemorrhage especially hemorrhage in the central nervous system. Administration of Vitamin K (1mg, 1M) at birth can present these severe complications. (author)

  10. The role of fibrinogen and haemostatic assessment in postpartum haemorrhage

    DEFF Research Database (Denmark)

    Wikkelsø, Anne Juul

    2015-01-01

    Pregnancy is a state of hypercoagulobility that might be an evolutionary way of protecting parturients from exsanguination following child birth. Observational studies suggest an association between a low level of fibrinogen (coagulation factor I) at the start of postpartum haemorrhage (PPH....... Paper III was based on two national Danish registries evaluating the predictability of postpartum blood transfusion. Prediction was found difficult. However, retained placental parts seemed to be the strongest predictor. Since this diagnosis is made very late and often in association with the onset...... describes the protocol for a RCT of early fibrinogen supplementation in women with severe postpartum haemorrhage. Several practical, ethical and trial management challenges need to be addressed when conducting independent clinical research involving parturients with severe bleeding, placebo...

  11. Showing no spot sign is a strong predictor of independent living after intracerebral haemorrhage

    DEFF Research Database (Denmark)

    Havsteen, Inger; Ovesen, Christian; Christensen, Anders F

    2014-01-01

    BACKGROUND: A spot sign on computed tomography angiography (CTA) is a potentially strong predictor of poor outcome on ultra-early radiological imaging. The aim of this study was to assess the spot sign as a predictor of functional outcome at 3 months as well as long-term mortality, with a focus...... on the ability to identify patients with a spontaneous, acceptable outcome. METHODS: In a prospective, consecutive single-centre registry of acute stroke patients, we investigated patients with spontaneous intracerebral haemorrhage (ICH) admitted within 4.5 h after symptom onset from April 2009 to January 2013....... The standard work-up in our centre included CTA for spot sign status, unless a contraindication was present. Modified Rankin Scale (mRS) scores were assessed at 3 months in the outpatient clinic or by telephone interviews. Long-term mortality was assessed by electronic chart follow-up for up to 1,500 days...

  12. Does ethamsylate reduce haemorrhage in transurethral prostatectomy?

    Science.gov (United States)

    Lyth, D R; Booth, C M

    1990-12-01

    A double-blind, randomised trial of 44 consecutive patients undergoing transurethral prostatectomy demonstrated that ethamsylate (Dicynene) did not reduce blood loss during either the operative or the post- operative periods.

  13. Acute post-stroke blood pressure relative to premorbid levels in intracerebral haemorrhage versus major ischaemic stroke: a population-based study

    Science.gov (United States)

    Fischer, Urs; Cooney, Marie Therese; Bull, Linda M; Silver, Louise E; Chalmers, John; Anderson, Craig S; Mehta, Ziyah; Rothwell, Peter M

    2014-01-01

    Summary Background It is often assumed that blood pressure increases acutely after major stroke, resulting in so-called post-stroke hypertension. In view of evidence that the risks and benefits of blood pressure-lowering treatment in acute stroke might differ between patients with major ischaemic stroke and those with primary intracerebral haemorrhage, we compared acute-phase and premorbid blood pressure levels in these two disorders. Methods In a population-based study in Oxfordshire, UK, we recruited all patients presenting with stroke between April 1, 2002, and March 31, 2012. We compared all acute-phase post-event blood pressure readings with premorbid readings from 10-year primary care records in all patients with acute major ischaemic stroke (National Institutes of Health Stroke Scale >3) versus those with acute intracerebral haemorrhage. Findings Of 653 consecutive eligible patients, premorbid and acute-phase blood pressure readings were available for 636 (97%) individuals. Premorbid blood pressure (total readings 13 244) had been measured on a median of 17 separate occasions per patient (IQR 8–31). In patients with ischaemic stroke, the first acute-phase systolic blood pressure was much lower than after intracerebral haemorrhage (158·5 mm Hg [SD 30·1] vs 189·8 mm Hg [38·5], pblood pressure after intracerebral haemorrhage was substantially higher than premorbid levels (mean increase of 40·7 mm Hg, pblood pressure also increased steeply in the days and weeks before intracerebral haemorrhage (regression pblood pressure reading after primary intracerebral haemorrhage was more likely than after ischaemic stroke to be the highest ever recorded (OR 3·4, 95% CI 2·3–5·2, pblood pressure within 3 h of onset was 50 mm Hg higher, on average, than the maximum premorbid level whereas that after ischaemic stroke was 5·2 mm Hg lower (pblood pressure is substantially raised compared with usual premorbid levels after intracerebral haemorrhage, whereas acute

  14. A Q fever case mimicking crimean-congo haemorrhagic fever

    Directory of Open Access Journals (Sweden)

    O Karabay

    2011-01-01

    Full Text Available Coxiella burnetii is the bacterium that causes Q fever. Human infection is mainly transmitted from cattle, goats and sheep. The disease is usually self-limited. Pneumonia and hepatitis are the most common clinical manifestations. In this study, we present a case of Q fever from the western part of Turkey mimicking Crimean-Congo haemorrhagic fever (CCHF in terms of clinical and laboratory findings.

  15. Ligation of internal iliac arteries for control of pelvic haemorrhage.

    Directory of Open Access Journals (Sweden)

    Nandanwar Y

    1993-10-01

    Full Text Available This is a retrospective review of the internal iliac ligations (IAL done over a period of 3 years. Both gynaecological and obstetric cases were considered. A total of 46 ligations were performed. The additional treatment required was hysterectomy to control haemorrhage. Intra-and post-operative complications were noted. A comparative review of the effectiveness of IAL in different situations is done and alternative modalities of treatment are considered.

  16. Haemorrhagic enteritis seroconversion in turkey breeders: field observations

    Directory of Open Access Journals (Sweden)

    Raffaella Ceruti

    2010-01-01

    Full Text Available Seroconversion to viral haemorrhagic enteritis (HE was studied in seven flocks of turkey breeders (17.974 birds in total, after 20 weeks of the onset of egg production. They showed no clinical signs, and mortality rate was normal. However, the infection caused a drop in egg production lasting about five weeks (-2.32 eggs laid during this period, but had no effect on hatching parameters.

  17. Case definition for Ebola and Marburg haemorrhagic fevers: a complex challenge for epidemiologists and clinicians.

    Science.gov (United States)

    Pittalis, Silvia; Fusco, Francesco Maria; Lanini, Simone; Nisii, Carla; Puro, Vincenzo; Lauria, Francesco Nicola; Ippolito, Giuseppe

    2009-10-01

    Viral haemorrhagic fevers (VHFs) represent a challenge for public health because of their epidemic potential, and their possible use as bioterrorism agents poses particular concern. In 1999 the World Health Organization (WHO) proposed a case definition for VHFs, subsequently adopted by other international institutions with the aim of early detection of initial cases/outbreaks in western countries. We applied this case definition to reports of Ebola and Marburg virus infections to estimate its sensitivity to detect cases of the disease. We analyzed clinical descriptions of 795 reported cases of Ebola haemorrhagic fever: only 58.5% of patients met the proposed case definition. A similar figure was obtained reviewing 169 cases of Marburg diseases, of which only 64.5% were in accordance with the case definition. In conclusion, the WHO case definition for hemorrhagic fevers is too specific and has poor sensitivity both for case finding during Ebola or Marburg outbreaks, and for early detection of suspected cases in western countries. It can lead to a hazardous number of false negatives and its use should be discouraged for early detection of cases.

  18. Depression and post-traumatic stress disorder in individuals with hereditary hemorrhagic telangiectasia: A cross-sectional survey.

    Science.gov (United States)

    Chaturvedi, Shruti; Clancy, Marianne; Schaefer, Nicole; Oluwole, Olalekan; McCrae, Keith R

    2017-05-01

    Hereditary hemorrhagic telangiectasia (HHT) is characterized by frequent severe bleeding, particularly epistaxis, and life-threatening complications including stroke, brain abscess and heart failure. The psychological impact of HHT is not known. We conducted this cross sectional study to determine the prevalence of depression and post-traumatic stress disorder (PTSD) related to HHT. A survey tool comprising demographic and clinical information and two validated self-administered questionnaires, the PTSD checklist for DSM-5 (PCL-5) and Beck Depression Inventory-II (BDI-II), was distributed to individuals with HHT. Associations with clinical and demographic variables with depression and PTSD were evaluated in a logistic regression model. A total of 222 individuals responded to the survey. Of these, 185 completed either the BDI II or PCL-5 and were included in the analysis. Median age was 54years and 142 (76.8%) were female. An existing diagnosis of depression, anxiety disorder and PTSD was present in 81 (43.8%), 59 (31.9%) and 16(8.6%) respondents, respectively. BDI-II scores>13 indicating at least mild depressive symptoms were present in 142 (88.7%) patients and 52 (28.1%) patients had a positive screen for PTSD (PCL-5 score≥38). On multivariable analysis, depression [OR 2.17 (95% CI 1.045-4.489), p=0.038], anxiety disorder [OR 2.232 (95% CI 1.066-4.676), p=0.033], and being unemployed [OR 2.234 (95% CI 1.46-4.714), p=0.019) were associated with PTSD. We report a high prevalence of depressive and PTSD symptoms in individuals with HHT. While selection bias may lead to overestimation of prevalence in this study, our results are concerning and clinicians should remain vigilant for signs of psychological distress and consider screening for these disorders. Copyright © 2017 Elsevier Ltd. All rights reserved.

  19. Oxidative stress, mitochondrial abnormalities and antioxidant defense in Ataxia-telangiectasia, Bloom syndrome and Nijmegen breakage syndrome

    Directory of Open Access Journals (Sweden)

    Mateusz Maciejczyk

    2017-04-01

    Full Text Available Rare pleiotropic genetic disorders, Ataxia-telangiectasia (A-T, Bloom syndrome (BS and Nijmegen breakage syndrome (NBS are characterised by immunodeficiency, extreme radiosensitivity, higher cancer susceptibility, premature aging, neurodegeneration and insulin resistance. Some of these functional abnormalities can be explained by aberrant DNA damage response and chromosomal instability. It has been suggested that one possible common denominator of these conditions could be chronic oxidative stress caused by endogenous ROS overproduction and impairment of mitochondrial homeostasis. Recent studies indicate new, alternative sources of oxidative stress in A-T, BS and NBS cells, including NADPH oxidase 4 (NOX4, oxidised low-density lipoprotein (ox-LDL or Poly (ADP-ribose polymerases (PARP. Mitochondrial abnormalities such as changes in the ultrastructure and function of mitochondria, excess mROS production as well as mitochondrial damage have also been reported in A-T, BS and NBS cells. A-T, BS and NBS cells are inextricably linked to high levels of reactive oxygen species (ROS, and thereby, chronic oxidative stress may be a major phenotypic hallmark in these diseases. Due to the presence of mitochondrial disturbances, A-T, BS and NBS may be considered mitochondrial diseases. Excess activity of antioxidant enzymes and an insufficient amount of low molecular weight antioxidants indicate new pharmacological strategies for patients suffering from the aforementioned diseases. However, at the current stage of research we are unable to ascertain if antioxidants and free radical scavengers can improve the condition or prolong the survival time of A-T, BS and NBS patients. Therefore, it is necessary to conduct experimental studies in a human model.

  20. Transcranial Doppler velocimetry in aneurysmal subarachnoid haemorrhage

    DEFF Research Database (Denmark)

    Staalsø, J M; Edsen, T; Romner, B

    2013-01-01

    -coded transcranial Doppler (TCCD), with the secondary aim of describing prediction of angiographic vasospasm and mortality. METHODS: /st>Sixty patients and 70 healthy controls were each examined in duplicate by alternating operators. A total of 939 measurements divided on 201 examination sets were conducted by four...

  1. Secondary postpartum haemorrhage with uterine artery ...

    African Journals Online (AJOL)

    a left uterine artery PA, which measured 4 cm in diameter, with extravasation of contrast into a pocket that connected to the uterine cavity. After obtaining the patient's consent, selective left uterine artery embolisation was performed with a mixture of Gelfoam and contrast media, followed by one stainless steel coil insertion.

  2. A randomised controlled trial comparing oxytocin and oxytocin + ergometrine for prevention of postpartum haemorrhage at caesarean section.

    Science.gov (United States)

    Koen, Sandy; Snyman, Leon Cornelius; Pattinson, Robert C; Makin, Jennifer A

    2016-03-07

    Globally 166 000 women die annually as a result of obstetric haemorrhage. More than 50% of these deaths occur in sub-Saharan Africa. Uterine atony is the commonest cause of severe postpartum haemorrhage (PPH). Bleeding at or after caesarean section (CS) is responsible for >30% of maternal deaths due to obstetric haemorrhage in South Africa (SA). To compare oxytocin alone with oxytocin + ergometrine in terms of primary prophylaxis for PPH at the time of CS. This was a double-blind randomised controlled interventional study comparing oxytocin with oxytocin + ergometrine administered during CS. Patients were randomised to receive oxytocin alone intravenously as a bolus or oxytocin + ergometrine intramuscularly, with the placebo being an injection of sterile water. The study population consisted of women undergoing CS at Kalafong Provincial Tertiary Hospital in Atteridgeville, Gauteng, SA. Five hundred and forty women were randomised and data for 416 women, of whom 214 received oxytocin and 202 oxytocin + ergometrine, were available for analysis. In the oxytocin group 19 women (8.9%) required blood transfusion, compared with seven (3.5%) in the oxytocin + ergometrine group (p=0.01; relative risk = 2.78; 95% confidence interval 1.21 - 6.4). There were no statistically significant differences in the mean estimated visual and mean calculated blood loss. The overall need for blood transfusion was significantly reduced by about two-thirds in women receiving the oxytocin + ergometrine combination. Consideration should be given to using oxytocin + ergometrine for prophylaxis of PPH at CS.

  3. Small Molecules Targeting Ataxia Telangiectasia and Rad3-Related (ATR) Kinase: An Emerging way to Enhance Existing Cancer Therapy

    Czech Academy of Sciences Publication Activity Database

    Andrs, M.; Korábečný, J.; Nepovimova, E.; Jun, D.; Hodný, Zdeněk; Kuca, K.

    2016-01-01

    Roč. 16, č. 3 (2016), s. 200-208 ISSN 1568-0096 Institutional support: RVO:68378050 Keywords : Ataxia telangiectasia and Rad3-related kinase (ATR) * cancer * chemosensitization * DNA damage response * phosphatidylinositol 3-kinase-related protein kinases (PIKK) * radiosensitization * synthetic lethality Subject RIV: EB - Genetics ; Molecular Biology Impact factor: 2.992, year: 2016

  4. Ataxia Telangiectasia

    Science.gov (United States)

    ... is the measurement of "fetal proteins," or serum alpha-fetoprotein , in the blood. These are proteins that are ... than 95 percent) have elevated levels of serum alpha-fetoprotein. When other causes of elevations of alpha-fetoprotein ...

  5. Ataxia Telangiectasia

    Science.gov (United States)

    ... the hair, difficulty swallowing, and delayed physical and sexual development. Children with A-T usually have normal or ... the hair, difficulty swallowing, and delayed physical and sexual development. Children with A-T usually have normal or ...

  6. Bilateral adrenal cystic neuroblastoma with superior vena cava syndrome and massive intracystic haemorrhage

    International Nuclear Information System (INIS)

    Pinarli, Faruk Guclu; Danaci, Murat; Diren, Baris; Tander, Burak; Rizalar, Riza; Dagdemir, Ayhan; Acar, Sabri

    2004-01-01

    Bilateral cystic adrenal tumours are a rare presentation of neuroblastoma. Intratumoural haemorrhage is a frequent finding in neuroblastoma, but is rarely symptomatic. We present an 11-month-old girl with predominantly cystic bilateral neuroblastomas and distant lymph-node metastasis. Massive intracystic haemorrhage and superior vena cava (SVC) syndrome were ominous prognostic factors, leading to death. Large tumours with intracystic haemorrhage might require a conservative approach. (orig.)

  7. Stable radioresistance in ataxia-telangiectasia cells containing DNA from normal human cells

    International Nuclear Information System (INIS)

    Kapp, L.N.; Painter, R.B.

    1989-01-01

    SV40-transformed ataxia-telangiectasia (AT) cells were transfected with a cosmid containing a normal human DNA library and selectable marker, the neo gene, which endows successfully transformed mammalian cells with resistance to the antibiotic G418. Cells from this line were irradiated with 50 Gy of X-rays and fused with non-transfected AT cells. Among the G418-resistant colonies recovered was one stably resistant to radiation. Resistance to ionizing radiation of both primary transfectant line and its fusion derivative was intermediate between that of AT cells and normal cells, as assayed by colony-forming ability and measurement of radiation-induced G 2 chromatic aberrations; both cell lines retained AT-like radioresistant DNA synthesis. Results suggest that, because radioresistance in transfected cells was not as great as in normal human cells, two hallmarks of AT, radiosensitivity and radioresistant DNA synthesis, may still be the result of a single defective AT gene. (author)

  8. Cell and Molecular Biology of Ataxia Telangiectasia Heterozygous Human Mammary Epithelial Cells Irradiated in Culture

    Science.gov (United States)

    Richmond, Robert C.

    2001-01-01

    Autologous isolates of cell types from obligate heterozygotes with the autosomal disorder ataxia-telangiectasia (A-T)were used to begin a tissue culture model for assessing pathways of radiation-induced cancer formation in this target tissue. This was done by establishing cultures of stromal fibroblasts and long-term growth human mammary epithelial cells (HMEC) in standard 2-dimensional tissue culture in order to establish expression of markers detailing early steps of carcinogenesis. The presumptive breast cancer susceptibility of A-T heterozygotes as a sequel to damage caused by ionizing radiation provided reason to study expression of markers in irradiated HMEC. Findings from our study with HMEC have included determination of differences in specific protein expression amongst growth phase (e.g., log vs stationary) and growth progression (e.g., pass 7 vs pass 9), as well as differences in morphologic markers within populations of irradiated HMEC (e.g., development of multinucleated cells).

  9. Impact of NICE guidance on rates of haemorrhage after tonsillectomy: an evaluation of guidance issued during an ongoing national tonsillectomy audit.

    Science.gov (United States)

    Audit, National Prospective Tonsillectomy

    2008-08-01

    The National Institute for Health and Clinical Excellence (NICE) issued guidance on surgical techniques for tonsillectomy during a national audit of surgical practice and postoperative complications. To assess the impact of the guidance on tonsillectomy practice and outcomes. An interrupted time-series analysis of routinely collected Hospital Episodes Statistics data, and an analysis of longitudinal trends in surgical technique using data from the National Prospective Tonsillectomy Audit. Patients undergoing tonsillectomy in English NHS hospitals between January 2002 and December 2004. Postoperative haemorrhage within 28 days. The rate of haemorrhage increased by 0.5% per year from 2002, reaching 6.4% when the guidance was published. After publication, the rate of haemorrhage fell immediately to 5.7% (difference 0.7%: 95% CI -1.3% to 0.0%) and the rate of increase appeared to have stopped. Data from the National Prospective Tonsillectomy Audit showed that the fall coincided with a shift in surgical techniques, which was consistent with the guidance. NICE guidance influenced surgical tonsillectomy technique and in turn produced an immediate fall in postoperative haemorrhage. The ongoing national audit and strong support from the surgical specialist association may have aided its implementation.

  10. New Therapeutic Possibilities of the Post-Irradiation Haemorrhagic Syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Pospisil, J.; Dienstbier, Z. [Institute of Biophysics and Nuclear Medicine, Faculty of General Medicine, Charles University, Prague, Czechoslovak Socialist Republic (Czech Republic); Skala, E. [Central Military Hospital, Prague-Stresovice, Czechoslovak Socialist Republic (Czech Republic)

    1969-10-15

    Haemorrhagic diathesis is one of the dominant symptoms of acute post-irradiation lesion. Haemorrhagic syndrome is caused by the disturbance of haemocoagulation during simultaneous lesion of the vascular system. In our study we have tried to affect the post-irradiation haemocoagulation disturbance. Epsilon- amino-caproic acid (EACA) administered between the 8{sup th} and the 18{sup th} day (0.4 g/kg per day) to whole- body irradiated dogs (600 R) partially regulated the post-irradiation disturbance of haemocoagulation. The favourable effect of EACA was verified by in vitro experiments in which the blood of irradiated dogs was used. A repeated administration of EACA in the dose of 0.4 g/kg per day to whole-body irradiated rats (600 R) did not substantially affect the post-irradiation changes in the number of white blood elements; however, its administration to healthy animals caused lymphocytosis. In whole-body irradiated dogs (600 R) we have found lower levels of EACA in the blood up to the 8 day following irradiation as compared with healthy dogs after oral application of EACA. The whole-body irradiation of mice did not increase the acute toxicity of EACA. The daily administration of 0.4 g EACA/kg to whole-body irradiated mice (600 and 700 R) did not change the mortality induced by irradiation. The authors consider EACA to be a suitable compound for a complex therapy of radiation sickness. The administration of para-amino-methyl-benzoic acid (PAMBA), in spite of a certain improvement of postirradiation haemocoagulation disturbance, is less efficient. Our recent experiments with ellagic acid which significantly affects the post-traumatic haemorrhage in whole-body irradiated rats seem to be very promising. (author)

  11. Profound bilateral visual loss after hysterectomy indicated for severe postpartum haemorrhage

    DEFF Research Database (Denmark)

    Ostri, Christoffer; Zibrandtsen, Nathalie; Larsen, Michael

    2014-01-01

    We present a case of a patient with bilateral posterior ischaemic optic neuropathy in the previously unreported setting of hysterectomy indicated for severe postpartum haemorrhage. The diagnosis was based on clinical and paraclinical examinations, including MRI of the head, electroretinography (ERG....... The diagnosis of perioperative posterior ischaemic optic neuropathy is mostly a clinical diagnosis. However, MRI plays a major role in excluding other causes of visual loss, and VEP, ERG and OCT are valuable supplemental diagnostic tools.......) and visual evoked potentials (VEP) testing. During 1 year of follow-up, repeated optical coherence tomography (OCT) scans demonstrated optic disc atrophy, which was interpreted as a sign of direct retrograde ganglion cell degeneration after ischaemic damage to the retrolaminar part of the optic nerves...

  12. Reducing Haemorrhagic Transformation after Thrombolysis for Stroke: A Strategy Utilising Minocycline

    Directory of Open Access Journals (Sweden)

    David J. Blacker

    2013-01-01

    Full Text Available Haemorrhagic transformation (HT of recently ischaemic brain is a feared complication of thrombolytic therapy that may be caused or compounded by ischaemia-induced activation of matrix metalloproteinases (MMPs. The tetracycline antibiotic minocycline inhibits matrix MMPs and reduces macroscopic HT in rodents with stroke treated with tissue plasminogen activator (tPA. The West Australian Intravenous Minocycline and TPA Stroke Study (WAIMATSS aims to determine the safety and efficacy of adding minocycline to tPA in acute ischaemic stroke. The WAIMATSS is a multicentre, prospective, and randomised pilot study of intravenous minocycline, 200 mg 12 hourly for 5 doses, compared with standard care, in patients with ischaemic stroke treated with intravenous tPA. The primary endpoint is HT diagnosed by brain CT and MRI. Secondary endpoints include clinical outcome measures. Some illustrative cases from the early recruitment phase of this study will be presented, and future perspectives will be discussed.

  13. Crystals seen on CSF microscopy in a case of suspected subarachnoid haemorrhage

    Science.gov (United States)

    Weiand, Daniel; Hanning, Ian; Mouhamadou, Moussa; Wearmouth, Debbie

    2015-01-01

    Although crystals are rarely identified on cerebrospinal fluid (CSF) microscopy, their presence can be of significant diagnostic value. We report a case of oxalate crystals seen on CSF microscopy of a 43-year-old woman. The patient presented with headaches, nausea and vomiting. CT of the head showed a small focus of hyper-density, suspicious of haemorrhage, in the right side of the pontine cistern. CSF cell count was within the normal range. Although no organisms were seen on microscopy, copious oxalate crystals were seen. The same crystals were seen on microscopy of CSF collected in a fluoride oxalate container used for glucose analysis. A follow-up contrast-enhanced CT angiogram did not demonstrate any abnormalities. It transpired that excess CSF had been collected into a fluoride oxalate container. This had subsequently been decanted into a plain container for microbiological analysis. Correct specimen collection should be emphasised when teaching lumbar puncture technique. PMID:26139652

  14. Prediction of delayed neurological deficit after subarachnoid haemorrhage: a CT blood load and Doppler velocity approach

    International Nuclear Information System (INIS)

    Grosset, D.G.; McDonald, I.; Cockburn, M.; Straiton, J.; Bullock, R.R.

    1994-01-01

    The predictive value of cranial computed tomography (CT) blood load and serial transcranial Doppler sonography for the development of delayed ischaemic neurological deficit was assessed in 121 patients following subarachnoid haemorrhage. Of the 121 patients, 81 (67 %) had thick layers of blood or haematoma, including intraventricular bleeding. The proportion of patients who developed delayed deficit was higher with increasing amounts of subarachnoid blood on the admission CT (51 % of 53 cases in Fisher grade 3; 35 % of 33 cases in grade 2; 28 % of 7 cases in grade 1, P < 0.01). Doppler velocities obtained from readings at least every 2 days following admission were higher in patients with delayed neurological deficit (peak velocity for grade 3 patients 176 ± 6 cm/s (mean ± SE), versus grade 2: 164 ± 7 cm/s; grade 4 149 ± 9, both P = 0.04, Mann-Whitney). Peak velocity and maximal 24-h rise tended to be higher within different CT grades in patients with a deficit than in those without; this difference was significant for grade 3 patients (P < 0.01). We conclude that a combined approach with CT and Doppler sonography provides greater predictive value for the development of delayed ischaemic neurological deficit than either test considered independently. The value of Doppler sonography may be greatest for patients with Fisher grade 3 blood, in whom the risk of delayed ischaemia is greatest. (orig.)

  15. The effectiveness of the double B-lynch suture as a modification in the treatment of intractable postpartum haemorrhage.

    Science.gov (United States)

    Şahin, Hanifi; Soylu Karapınar, Oya; Şahin, Eda Adeviye; Dolapçıoğlu, Kenan; Baloğlu, Ali

    2018-03-20

    A broader range of more effective compression techniques are needed in the patients who have an intractable postpartum haemorrhage due to uterine atony despite medical treatment and B-Lynch sutures. The aim of this study was to report the outcome of a series of patients with haemorrhage who were managed by double B-Lynch suture. Fourteen patients who were treated in a tertiary hospital between July 2010 and February 2015 were included in the study. The intractable haemorrhage rate was 0.35% over 5 years (14/4000 births). Bleeding was controlled in all the patients with a double B-Lynch suture. The mean age of the patients was 24 ± 3.4 years. The mean estimated blood loss was 1696 ± 272.075 mL, and the mean transfusion rate was 4.2 ± 2.5 units. Pregnancy was observed in five patients at follow up. The double B-Lynch suture seems to be an effective and reliable solution to an intractable postpartum haemorrhage resulting from uterine atony and has no unfavourable impacts on fertility. It should be considered before the use of any aggressive surgical techniques such as a hypogastric artery ligation or a hysterectomy. This the first study to investigate the effectiveness of the double B-Lynch suture, and we showed that the hysterectomy and/or hypogastric artery ligation rate can be decreased by adding a second B-Lynch suture in cases where the medical treatment or a single B-Lynch has failed. Impact statement What is already known on the subject? Uterine atony is the most common cause of a primary postpartum haemorrhage. When a simple massage of the uterus and medication failed to manage this condition, various surgical solutions have been sought, including uterine compression sutures, uterine artery ligation, devascularisation of the uterus, internal iliac artery ligation and, ultimately, a hysterectomy. The B-Lynch suturing technique is particularly useful because of its simplicity of application, life-saving potential, relative safety and capacity

  16. Haemorrhagic acoustic neuroma with features of a vascular malformation. A case report

    Energy Technology Data Exchange (ETDEWEB)

    Benhaiem-Sigaux, N. [Dept. of Pathology, Hopital Henri Mondor, Creteil (France); Ricolfi, F. [Dept. of Neuroradiology, Henri Mondor Hospital, Creteil (France); Torres-Diaz, A.; Keravel, Y. [Dept. of Neurosurgery, Henri Mondo Hospital, Creteil (France); Poirier, J. [Dept. of Histology, Pitie-Salpetriere Hospital, Paris (France)

    1999-10-01

    A 55-year-old man with hearing loss presented with vertigo and vomiting. CT tomography and MRI demonstrated a cerebellopontine angle mass with foci of haemorrhage. An angiomatous tumour, with large abnormal veins adhering to the capsule, was completely removed. Histologically, the tumour was an acoustic neuroma with abnormal vascularisation and limited intratumoral haemorrhage. (orig.)

  17. A composite neurobehavioral test to evaluate acute functional deficits after cerebellar haemorrhage in rats.

    Science.gov (United States)

    McBride, Devin W; Nowrangi, Derek; Kaur, Harpreet; Wu, Guangyong; Huang, Lei; Lekic, Tim; Tang, Jiping; Zhang, John H

    2018-03-01

    Cerebellar haemorrhage accounts for 5-10% of all intracerebral haemorrhages and leads to severe, long-lasting functional deficits. Currently, there is limited research on this stroke subtype, which may be due to the lack of a suitable composite neuroscoring system specific for cerebellar injury in rodents. The purpose of this study is to develop a comprehensive composite neuroscore test for cerebellar injury using a rat model of cerebellar haemorrhage. Sixty male Sprague-Dawley rats were subjected to either sham surgery or cerebellar haemorrhage. Twenty-four hours post-injury, neurological behaviour was evaluated using 17 cost-effective and easy-to-perform tests, and a composite neuroscore was developed. The composite neuroscore was then used to assess functional recovery over seven days after cerebellar haemorrhage. Differences in the composite neuroscore deficits for the mild and moderate cerebellar haemorrhage models were observed for up to five days post-ictus. Until now, a composite neuroscore for cerebellar injury was not available for rodent studies. Herein, using mild and moderate cerebellar haemorrhage rat models a composite neuroscore for cerebellar injury was developed and used to assess functional deficits after cerebellar haemorrhage. This composite neuroscore may also be useful for other cerebellar injury models.

  18. Haemorrhagic acoustic neuroma with features of a vascular malformation. A case report

    International Nuclear Information System (INIS)

    Benhaiem-Sigaux, N.; Ricolfi, F.; Torres-Diaz, A.; Keravel, Y.; Poirier, J.

    1999-01-01

    A 55-year-old man with hearing loss presented with vertigo and vomiting. CT tomography and MRI demonstrated a cerebellopontine angle mass with foci of haemorrhage. An angiomatous tumour, with large abnormal veins adhering to the capsule, was completely removed. Histologically, the tumour was an acoustic neuroma with abnormal vascularisation and limited intratumoral haemorrhage. (orig.)

  19. Acute haemorrhagic diarrhoea syndrome in dogs: 108 cases.

    Science.gov (United States)

    Mortier, F; Strohmeyer, K; Hartmann, K; Unterer, S

    2015-06-13

    No prospective studies including large numbers of dogs with acute haemorrhagic diarrhoea syndrome (AHDS) are published so far. The aim of this case-control study was to describe signalment, history, clinical signs, laboratory values and course of disease in dogs with AHDS. Dogs (108) with idiopathic acute haemorrhagic diarrhoea (schnauzer and Maltese. The syndrome was more likely to occur during winter. Vomiting preceded the onset of bloody diarrhoea in 80 per cent of dogs and haematemesis was observed in half of those cases. Median AHDS index at presentation was 12 (range 3-17). Haematocrit was generally high (median 57.1 per cent; range 33-76 per cent), but exceeded 60 per cent only in 31.4 per cent of dogs. Haematocrit of 48.1 per cent of dogs was above reference range, as was monocyte (50.0 per cent), segmented (59.6 per cent) and band neutrophil count (45.2 per cent). A rapid clinical improvement occurred during the first 48 hours. British Veterinary Association.

  20. Genetic population structure of marine viral haemorrhagic septicaemia virus (VHSV)

    DEFF Research Database (Denmark)

    Snow, M.; Bain, N.; Black, J.

    2004-01-01

    The nucleotide sequences of a specific region of the nucleoprotein gene were compared in order to investigate the genetic population structure of marine viral haemorrhagic septicaemia virus (VHSV). Analysis of the sequence from 128 isolates of diverse geographic and host origin renders this the m......The nucleotide sequences of a specific region of the nucleoprotein gene were compared in order to investigate the genetic population structure of marine viral haemorrhagic septicaemia virus (VHSV). Analysis of the sequence from 128 isolates of diverse geographic and host origin renders...... this the most comprehensive molecular epidemiological study of marine VHSV conducted to date. Phylogenetic analysis of nucleoprotein gene sequences confirmed the existence of the 4 major genotypes previously identified based on N- and subsequent G-gene based analyses. The range of Genotype I included subgroups...... of isolates associated with rainbow trout aquaculture (Genotype la) and those from the Baltic marine environment (Genotype Ib) to emphasise the relatively close genetic relationship between these isolates. The existence of an additional genotype circulating within the Baltic Sea (Genotype II) was also...