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Sample records for hypertrophic olivary degeneration

  1. Magnetic resonance imaging of hypertrophic olivary degeneration.

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    Blanco Ulla, M; López Carballeira, A; Pumar Cebreiro, J M

    2015-01-01

    To review the pathophysiologic mechanisms involved in hypertrophic olivary degeneration, with attention to epidemiologic and clinical aspects and especially to imaging findings. We reviewed 5 patients diagnosed with hypertrophic olivary degeneration at our center from 2010 through 2013, analyzing relevant clinical, epidemiologic, and radiologic findings. In all cases, a hyperintensity was seen in the inferior olivary nuclei in FLAIR and T2-weighted sequences. No signal alterations were seen on T1-weighted sequences, and no enhancement was seen after intravenous injection of contrast material. In the cases studied by diffusion-weighted imaging, no significant alterations were seen in these sequences. Olivary hypertrophy was seen in all patients except in one, in whom presumably not enough time had elapsed for hypertrophy to occur. The alterations were bilateral in two of the five cases. Only one case exhibited the typical clinical manifestations. Given that patients may not present clinical manifestations that can be attributed to hypertrophic olivary degeneration, it is important to recognize the characteristic radiologic signs of this entity. Copyright © 2014 SERAM. Published by Elsevier España, S.L.U. All rights reserved.

  2. MR imaging findings of hypertrophic olivary degeneration

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    Kim, Do Joong; Jeon, Pyung; Kim, Dong Ik [Yonsei Univ. College of Medicine, Seoul (Korea, Republic of)

    1997-06-01

    To describe the magnetic resonance (MR) imaging findings of hypertrophic olivary degeneration (HOD) MR images of seven patients with HOD were retrospectively reviewed. Two were women and five were men, and they were aged between 48 and 65 (mean 58) years. Imaging examinations were performed with a 1.5-T unit, and the findings were used to evaluate the size and signal intensity of olivary lesions. The time interval from hemorrhagic ictus to MR imaging was between two and 30 months. Follow-up examinations were performed in two patients. All four patients with hemorrhages involving the central tegmental tract in the pons or midbrain showed ipsilateral HOD. Among these four, bilateral HOD was seen in one patient with hemorrhage involving the bilateral central tegmental tract, and in another with tegmental hemorrhage extending to the ipsilateral superior cerebellar peduncle. One patient with cerebellar hemorrhage involving the dentate nucleus had contralateral HOD. Two patients with multiple hemorrhages involving both the pons and cerebellum showed bilateral HOD. Axial MR images showed mild enlargement of the involved olivary mucleus, with high signal intensity on both proton density and T2 weighted images. There was no apparent enhancement on postcontrast T1-weighted images. MR imaging can clearly distinguish secondary olivary degeneration from underlying pathology involving the central tegmental tract in the pons or midbrain and cerebellum. These olivary abnormalities should not, however, be mistaken for primary medullary lesions.

  3. Hypertrophic olivary degeneration and cerebrovascular disease: movement in a triangle.

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    Santos, Ana Filipa; Rocha, Sofia; Varanda, Sara; Pinho, João; Rodrigues, Margarida; Ramalho Fontes, João; Soares-Fernandes, João; Ferreira, Carla

    2015-02-01

    Hypertrophic olivary degeneration is a rare kind of trans-synaptic degeneration that occurs after lesions of the dentatorubro-olivary pathway. The lesions, commonly unilateral, may result from hemorrhage due to vascular malformation, trauma, surgical intervention or hypertension, tumor, or ischemia. Bilateral cases are extremely rare. This condition is classically associated with development of palatal tremor, but clinical manifestations can include other involuntary movements. We describe 2 cases: unilateral hypertrophic olivary degeneration in a 60-year-old man with contralateral athetosis and neurologic worsening developing several years after a pontine hemorrhage and bilateral hypertrophic olivary degeneration in a 77-year-old woman with development of palatal tremor, probably secondary to pontine ischemic lesions (small vessel disease). Copyright © 2015 National Stroke Association. Published by Elsevier Inc. All rights reserved.

  4. Hypertrophic Olivary Degeneration and Palatal or Oculopalatal Tremor

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    Caroline Tilikete

    2017-06-01

    Full Text Available Hypertrophic degeneration of the inferior olive is mainly observed in patients developing palatal tremor (PT or oculopalatal tremor (OPT. This syndrome manifests as a synchronous tremor of the palate (PT and/or eyes (OPT that may also involve other muscles from the branchial arches. It is associated with hypertrophic inferior olivary degeneration that is characterized by enlarged and vacuolated neurons, increased number and size of astrocytes, severe fibrillary gliosis, and demyelination. It appears on MRI as an increased T2/FLAIR signal intensity and enlargement of the inferior olive. There are two main conditions in which hypertrophic degeneration of the inferior olive occurs. The most frequent, studied, and reported condition is the development of PT/OPT and hypertrophic degeneration of the inferior olive in the weeks or months following a structural brainstem or cerebellar lesion. This “symptomatic” condition requires a destructive lesion in the Guillain–Mollaret pathway, which spans from the contralateral dentate nucleus via the brachium conjunctivum and the ipsilateral central tegmental tract innervating the inferior olive. The most frequent etiologies of destructive lesion are stroke (hemorrhagic more often than ischemic, brain trauma, brainstem tumors, and surgical or gamma knife treatment of brainstem cavernoma. The most accepted explanation for this symptomatic PT/OPT is that denervated olivary neurons released from inhibitory inputs enlarge and develop sustained synchronized oscillations. The cerebellum then modulates/accentuates this signal resulting in abnormal motor output in the branchial arches. In a second condition, PT/OPT and progressive cerebellar ataxia occurs in patients without structural brainstem or cerebellar lesion, other than cerebellar atrophy. This syndrome of progressive ataxia and palatal tremor may be sporadic or familial. In the familial form, where hypertrophic degeneration of the inferior olive may not

  5. Hypertrophic olivary degeneration: A clinico-radiologic study.

    Science.gov (United States)

    Konno, Takuya; Broderick, Daniel F; Tacik, Pawel; Caviness, John N; Wszolek, Zbigniew K

    2016-07-01

    The frequency and causes of hypertrophic olivary degeneration (HOD) are unknown. We compared the clinical and radiological characteristics of unilateral HOD and bilateral HOD. We performed a search of a radiologic report database for patients who were radiologically diagnosed as having HOD. This database includes the patients examined at the Mayo Clinic in Florida and Arizona. We used the search terms "hypertrophic olivary degeneration", "HOD", and "olivary" in the reports recorded from 1995 to 2015. Pertinent medical records and magnetic resonance imaging (MRI) scans of the brain for those with HOD were reviewed retrospectively. We identified 142 MRI studies on 95 cases who had radiologically proven HOD, 39 cases had unilateral HOD and 56 with bilateral HOD. In symptomatic cases, the most common symptom was ataxia. Palatal tremor was observed in almost half of all HOD cases. While cerebrovascular diseases were the most frequent etiology in both types of HOD (n = 24, 62% in unilateral; n = 17, 30% in bilateral), more than half of bilateral HOD cases had an unknown etiology (52%, n = 29), whereas only 13% (n = 5) of the unilateral cases had an unknown etiology (χ(2) test, P < 0.001). The lesions of unilateral HOD had a tendency to improve radiologically over time, whereas those associated with bilateral HOD were likely to worsen (χ(2) test, P < 0.05). Our study showed that bilateral HOD is more common than unilateral HOD. Half of bilateral HOD cases had no obvious cause and some worsened over time. This may implicate a possible primary neurodegenerative process. Copyright © 2016 Elsevier Ltd. All rights reserved.

  6. Bilateral Hypertrophic Olivary Degeneration and Holmes Tremor without Palatal Tremor: An Unusual Association

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    Cosentino, Carlos; Velez, Miriam; Nuñez, Yesenia; Palomino, Henry; Quispe, Darko; Flores, Martha; Torres, Luis

    2016-01-01

    Background Lesions in the Guillain–Mollaret triangle or dentate-rubro-olivary pathway may lead to hypertrophic olivary degeneration (HOD), a secondary trans-synaptic degeneration of the inferior olivary nucleus. HOD is usually associated with palatal tremor and rarely with Holmes tremor. Bilateral HOD is a very unusual condition and very few cases are reported. Case Report We report here two cases of bilateral HOD after two different vascular lesions located at the decussation of superior cerebellar peduncles, thus impairing both central tegmental tracts and interrupting bilaterally the dentate-rubral-olivary pathway. Interestingly, both developed bilateral Holmes tremor but not palatal tremor. Discussion Lesions in some of the components in the Guillain–Mollaret triangle may develop Holmes tremor with HOD and without palatal tremor. Magnetic resonance imaging is an invaluable tool in these cases. Better understanding of the pathways in this loop is needed. PMID:27536461

  7. Bilateral Hypertrophic Olivary Degeneration and Holmes Tremor without Palatal Tremor: An Unusual Association

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    Carlos Cosentino

    2016-07-01

    Full Text Available Background: Lesions in the Guillain–Mollaret triangle or dentate-rubro-olivary pathway may lead to hypertrophic olivary degeneration (HOD, a secondary trans-synaptic degeneration of the inferior olivary nucleus. HOD is usually associated with palatal tremor and rarely with Holmes tremor. Bilateral HOD is a very unusual condition and very few cases are reported. Case Report: We report here two cases of bilateral HOD after two different vascular lesions located at the decussation of superior cerebellar peduncles, thus impairing both central tegmental tracts and interrupting bilaterally the dentate-rubral-olivary pathway. Interestingly, both developed bilateral Holmes tremor but not palatal tremor. Discussion: Lesions in some of the components in the Guillain–Mollaret triangle may develop Holmes tremor with HOD and without palatal tremor. Magnetic resonance imaging is an invaluable tool in these cases. Better understanding of the pathways in this loop is needed.

  8. Unilateral Symptomatic Hypertrophic Olivary Degeneration Secondary to Midline Brainstem Cavernous Angioma: A Case Report and Review of the Literature.

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    Rosenblum, Jared; Nazari, Matt; Al-Khalili, Yasir; Potigailo, Valeria; Veznedaroglu, Erol

    2017-10-20

    Hypertrophic olivary degeneration (HOD) is a rare phenomenon in the dento-rubro-olivary pathway due to lesion or disruption of the fibers of the Guillain-Mollaret Triangle. Hemorrhage of pontine and midbrain cavernous angiomas rarely can lead to hypertrophic olivary degeneration (HOD) portending neurological deterioration and possible concomitant life-threatening complications; for this reason, it may define a poignant consideration in planning intervention. 57-year-old female with known midbrain-pontine cavernous angioma. For several years, the lesion was stable, as demonstrated by imaging follow-up until 10 months prior to presentation with falls, dysarthria and headache. Imaging demonstrated some decrease in size as well as blood product around the cavernous angioma suggesting interim period hemorrhage and interval development of unilateral hypertrophic olivary degeneration. Herein, the literature regarding imaging recommendations for stable cavernous angioma in the midbrain-pontine junction is reviewed. The implication of hypertrophic olivary degeneration for patient outcome is discussed and a comment is made on how the development of HOD may impact management of the cavernous angioma. Copyright © 2017. Published by Elsevier Inc.

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

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    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.)

  10. Olivary degeneration after intracranial haemorrhage or trauma: follow-up MRI

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    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.

  11. MRI and neurological presentation of hypertrophic olivary degeneration

    National Research Council Canada - National Science Library

    Elnekiedy, Abdelaziz; Naguib, Nagy; Hamed, Waseem; Mekky, Jaidaa; Mamdouh Hassan, Hebatallah Hassan

    2016-01-01

    .... Our purpose was to describe those MRI features linking them to their corresponding neurological manifestations and possible prior location of brain insults based on our own experience compared...

  12. The Dentato-Rubro-Olivary Tract: Clinical Dimension of This Anatomical Pathway

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    Fadil Khoyratty

    2013-01-01

    Full Text Available Symptomatic palatal tremor is potentially the result of a lesion in the triangle of Guillain-Mollaret (1931 and is associated with hypertrophic olivary degeneration (HOD which has characteristic MR findings. The triangle is defined by dentate efferents ascending through the superior cerebellar peduncle and crossing in the decussation of the brachium conjunctivum inferior to the red nucleus, to finaliy reach the inferior olivary nucleus (ION via the central tegmental tract. The triangle is completed by ION decussating efferents terminating on the original dentate nucleus via the inferior cerebellar peduncle. We can demonstrate the anatomy of this anatomical triangle using a clinical case of palatal tremor presenting with bilateral subjective pulsatile tinnitus along with the pathognomonic MR findings previously described. The hyperintense T2 signal in these patients may be permanent, but the hypertrophied olive normally regresses after 4 years. The temporal relationship between the evolution of the histopathology and the development of the palatal tremor remains unknown as does the natural history of the tremor. Botox injection at the level of tensor and levator veli palatini insertion have been used to treat patients with disabling tremor synchronous tinnitus. A lesion involving the triangle can have a quite varied clinical expression.

  13. Hypertrophic cardiomyopathy.

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    Santos Mateo, Juan José; Sabater Molina, María; Gimeno Blanes, Juan Ramón

    2017-11-14

    Hypertrophic cardiomyopathy is the most common inherited cardiovascular disease. It is characterized by increased ventricular wall thickness and is highly complex due to its heterogeneous clinical presentation, several phenotypes, large number of associated causal mutations and broad spectrum of complications. It is caused by mutations in sarcomeric proteins, which are identified in up to 60% of cases of the disease. Clinical manifestations of Hypertrophic Cardiomyopathy include shortness of breath, chest pain, palpitations and syncope, which are related to the onset of diastolic dysfunction, left ventricular outflow tract obstruction, ischemia, atrial fibrillation and abnormal vascular responses. It is associated with an increased risk of sudden cardiac death, heart failure and thromboembolic events. In this article, we discuss the diagnostic and therapeutic aspects of this disease. Copyright © 2017 Elsevier España, S.L.U. All rights reserved.

  14. Hypertrophic cardiomyopathy.

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    Subasic, Kim

    2013-12-01

    Hypertrophic cardiomyopathy (HCM) is an autosomal dominant, cardiovascular disorder that carries the risk of sudden cardiac death. The prevalence of HCM is 1:500 persons. The purpose of this article is to provide an overview of the pathophysiology, symptoms, complications, diagnostic testing, and treatment. The silent presentation of HCM presents unique diagnostic challenges and complicates prompt identification. Diagnostic testing and management strategies for the care of a person with HCM are discussed. HCM has individualized presentation and therefore requires individualized therapy. Copyright © 2013 Elsevier Inc. All rights reserved.

  15. Hypertrophic Cardiomyopathy Association

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    ... organization improving the lives of those with hypertrophic cardiomyopathy, HCM, preventing untimely deaths and advancing global understanding. ... item purchased, 10% will be donated to Hypertrophic Cardiomyopathy Association. iGive.com - Online Shopping Joing iGive.com ...

  16. Rupture of mitral valve chordae in hypertrophic cardiomyopathy.

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    Boissier, Florence; Achkouty, Guy; Bruneval, Patrick; Fabiani, Jean-Noël; Nguyen, Anh Tuan; Riant, Elisabeth; Desnos, Michel; Hagège, Albert

    2015-04-01

    While occasional reports of mitral valve chordal rupture have been described in hypertrophic cardiomyopathy, the exact prevalence and characteristics of this event in a large medical cohort have not been reported. To assess the prevalence of mitral valve chordal rupture in hypertrophic cardiomyopathy and the clinical, echocardiographic, surgical and histological profiles of those patients. We searched for patients with mitral valve chordal rupture diagnosed by echocardiography among all electronic files of patients admitted to our centre for hypertrophic cardiomyopathy between 2000 and 2010. Among 580 patients admitted for hypertrophic cardiomyopathy, six patients (1%, 5 men, age 68-71 years) presented with mitral valve chordal rupture, symptomatic in five cases, always involving the posterior mitral leaflet. In all cases, echocardiography before rupture showed mitral valve systolic anterior motion, with anterior (and not posterior) leaflet elongation compared with a random sample of patients with non-obstructive hypertrophic cardiomyopathy (P=0.006) (and similar to that observed in obstructive hypertrophic cardiomyopathy). Significant resting left ventricular outflow tract obstruction was always present before rupture and disappeared after rupture in the five cases requiring mitral valve surgery for severe mitral regurgitation. Histological findings were consistent with extensive myxomatous degeneration in all cases. Mitral valve chordal rupture is: infrequent in hypertrophic cardiomyopathy; occurs in aged patients with obstructive disease; involves, essentially, the posterior mitral leaflet; and causes, in general, severe mitral regurgitation requiring surgery. Myxomatous degeneration may be the substrate for rupture in these patients. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

  17. Characterization of the superior olivary complex of Canis lupus domesticus.

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    Fech, Tatiana; Calderón-Garcidueñas, Lilian; Kulesza, Randy J

    2017-08-01

    The superior olivary complex (SOC) is a collection of brainstem auditory nuclei which play essential roles in the localization of sound sources, temporal coding of vocalizations and descending modulation of the cochlea. Notwithstanding, the SOC nuclei vary considerably between species in accordance with the auditory needs of the animal. The canine SOC was subjected to anatomical and physiological examination nearly 50 years ago and was then virtually forgotten. Herein, we aimed to characterize the nuclei of the canine SOC using quantitative morphometrics, estimation of neuronal number, histochemistry for perineuronal nets and immunofluorescence for the calcium binding proteins calbindin and calretinin. We found the principal nuclei to be extremely well developed: the lateral superior olive (LSO) contained over 20,000 neurons and the medial superior olive (MSO) contained over 15,000 neurons. In nearly all non-chiropterian terrestrial mammals, the MSO exists as a thin, vertical column of neurons. The canine MSO was folded into a U-shaped contour and had associated with the ventromedial tip a small, round collection of neurons we termed the tail nucleus of the MSO. Further, we found evidence within the LSO, MSO and medial nucleus of the trapezoid body (MNTB) for significant morphological variations along the mediolateral or rostrocaudal axes. Finally, the majority of MNTB neurons were calbindin-immunopositive and associated with calretinin-immunopositive calyceal terminals. Together, these observations suggest the canine SOC complies with the basic plan of the mammalian SOC but possesses a number of unique anatomical features. Copyright © 2017 Elsevier B.V. All rights reserved.

  18. Metabolic Maturation of Auditory Neurones in the Superior Olivary Complex.

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    Barbara Trattner

    Full Text Available Neuronal activity is energetically costly, but despite its importance, energy production and consumption have been studied in only a few neurone types. Neuroenergetics is of special importance in auditory brainstem nuclei, where neurones exhibit various biophysical adaptations for extraordinary temporal precision and show particularly high firing rates. We have studied the development of energy metabolism in three principal nuclei of the superior olivary complex (SOC involved in precise binaural processing in the Mongolian gerbil (Meriones unguiculatus. We used immunohistochemistry to quantify metabolic markers for energy consumption (Na(+/K(+-ATPase and production (mitochondria, cytochrome c oxidase activity and glucose transporter 3 (GLUT3. In addition, we calculated neuronal ATP consumption for different postnatal ages (P0-90 based upon published electrophysiological and morphological data. Our calculations relate neuronal processes to the regeneration of Na(+ gradients perturbed by neuronal firing, and thus to ATP consumption by Na(+/K(+-ATPase. The developmental changes of calculated energy consumption closely resemble those of metabolic markers. Both increase before and after hearing onset occurring at P12-13 and reach a plateau thereafter. The increase in Na(+/K(+-ATPase and mitochondria precedes the rise in GLUT3 levels and is already substantial before hearing onset, whilst GLUT3 levels are scarcely detectable at this age. Based on these findings we assume that auditory inputs crucially contribute to metabolic maturation. In one nucleus, the medial nucleus of the trapezoid body (MNTB, the initial rise in marker levels and calculated ATP consumption occurs distinctly earlier than in the other nuclei investigated, and is almost completed by hearing onset. Our study shows that the mathematical model used is applicable to brainstem neurones. Energy consumption varies markedly between SOC nuclei with their different neuronal properties

  19. Climbing Fiber Burst Size and Olivary Sub-threshold Oscillations in a Network Setting

    NARCIS (Netherlands)

    J.R. de Gruijl (Jornt); P. Bazzigaluppi (Paolo); M.T.G. de Jeu (Marcel); C.I. de Zeeuw (Chris)

    2012-01-01

    textabstractThe inferior olivary nucleus provides one of the two main inputs to the cerebellum: the so-called climbing fibers. Activation of climbing fibers is generally believed to be related to timing of motor commands and/or motor learning. Climbing fiber spikes lead to large all-or-none action

  20. The Interior Olivary Complex of Guinea Pig: Cytoarchitecture and Cellular Morphology

    Science.gov (United States)

    1986-01-01

    Sotelo , C.. R. Lliniis and R. Baker. Structural study of inferior mammalian central neurone" inl iro’. In: Moieeint Ii.sor- olivary nucleus of the cat...Macmillan. 1984. pp. 165-182. 47. Stewart, W. W. Functional connections hetween cells as re- 24. IHina/s. R.. R. Baker and C. Sotelo . Flectrotonic

  1. Climbing fiber burst size and olivary sub-threshold oscillations in a network setting.

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    Jornt R De Gruijl

    Full Text Available The inferior olivary nucleus provides one of the two main inputs to the cerebellum: the so-called climbing fibers. Activation of climbing fibers is generally believed to be related to timing of motor commands and/or motor learning. Climbing fiber spikes lead to large all-or-none action potentials in cerebellar Purkinje cells, overriding any other ongoing activity and silencing these cells for a brief period of time afterwards. Empirical evidence shows that the climbing fiber can transmit a short burst of spikes as a result of an olivary cell somatic spike, potentially increasing the information being transferred to the cerebellum per climbing fiber activation. Previously reported results from in vitro studies suggested that the information encoded in the climbing fiber burst is related to the occurrence of the spike relative to the ongoing sub-threshold membrane potential oscillation of the olivary cell, i.e. that the phase of the oscillation is reflected in the size of the climbing fiber burst. We used a detailed three-compartmental model of an inferior olivary cell to further investigate the possible factors determining the size of the climbing fiber burst. Our findings suggest that the phase-dependency of the burst size is present but limited and that charge flow between soma and dendrite is a major determinant of the climbing fiber burst. From our findings it follows that phenomena such as cell ensemble synchrony can have a big effect on the climbing fiber burst size through dendrodendritic gap-junctional coupling between olivary cells.

  2. HYPERTROPHIC CARDIOMYOPATHY IN MULTIMORBIDITY

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    A. I. Lakhonina

    2016-06-01

    Full Text Available Aspects of diagnosis, difficulties in the diagnosis and optimal therapeutic strategies in patient with hypertrophic cardiomyopathy and comorbid conditions such as arterial hypertension, ischemic heart disease, dyslipidemia, diabetes mellitus type 2, stenosis of the left renal artery, obesity are reviewed on the example of clinical case. Hypertrophic cardiomyopathy combined with multimorbidity conditions requires a high-quality medical management, where the main goal is to improve the quality and duration of patient's life. This goal is being achieved by optimizing patient's lifestyle and assigning only the minimum amount of medications. Necessity of careful diagnosis of hypertrophic cardiomyopathy, evaluation of the risk of sudden death and search of optimal treatment in patients with multimorbidity pathology are demonstrated in clinical case.

  3. Hypertrophic pachymeningitis: case report

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    Deus-Silva Leonardo de

    2003-01-01

    Full Text Available Hypertrophic pachymeningits is an unusual cause of neurological symptoms and is often secondary to infections, carcinomatosis or inflammatory diseases. It may also be idiopathic. We report a case of pachymeningitis which was manifested primarily by psychosis and visual loss with optic atrophy and destruction of nasal septum. The patient, a 45 year old woman was submitted to extensive investigation without evidence of any underlying disease. A meningeal biopsy was performed and showed a mostly unspecific inflammatory process with extensive fibrosis of the dura and few early stage granulomas. These findings suggest either neurosarcoidosis or idiopathic hypertrophic pachymeningitis.

  4. Infantile hypertrophic pyloric stenosis

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    Pedersen, Rikke Neess; Garne, Ester; Loane, Maria

    2008-01-01

    OBJECTIVE: The objective of this study was to present epidemiologic data on infantile hypertrophic pyloric stenosis (IHPS) from seven well-defined European regions, and to compare incidence and changes in incidence over time between these regions. METHODS: This was a population-based study using...

  5. The Olivary Hypothesis of Essential Tremor: Time to Lay this Model to Rest?

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    Louis, Elan D.; Lenka, Abhishek

    2017-01-01

    Background Although essential tremor (ET) is the most common tremor disorder, its pathogenesis is not fully understood. The traditional model of ET, proposed in the early 1970s, posited that the inferior olivary nucleus (ION) was the prime generator of tremor in ET and that ET is a disorder of electrophysiological derangement, much like epilepsy. This article comprehensively reviews the origin and basis of this model, its merits and problems, and discusses whether it is time to lay this model to rest. Methods A PubMed search was performed in March 2017 to identify articles for this review. Results The olivary model gains support from the recognition of neurons with pacemaker property in the ION and the harmaline-induced tremor models (as the ION is the prime target of harmaline). However, the olivary model is problematic, as neurons with pacemaker property are not specific to the ION and the harmaline model does not completely represent the human disease ET. In addition, a large number of neuroimaging studies in ET have not detected structural or functional changes in the ION; rather, abnormalities have been reported in structures related to the cerebello-thalamo-cortical network. Moreover, a post-mortem study of microscopic changes in the ION did not detect any differences between ET cases and controls. Discussion The olivary model largely remains a physiological construct. Numerous observations have cast considerable doubt as to the validity of this model in ET. Given the limitations of the model, we conclude that it is time now to lay this model to rest. PMID:28966877

  6. The Olivary Hypothesis of Essential Tremor: Time to Lay this Model to Rest?

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    Elan D. Louis

    2017-07-01

    Full Text Available Background: Although essential tremor (ET is the most common tremor disorder, its pathogenesis is not fully understood. The traditional model of ET, proposed in the early 1970s, posited that the inferior olivary nucleus (ION was the prime generator of tremor in ET and that ET is a disorder of electrophysiological derangement, much like epilepsy. This article comprehensively reviews the origin and basis of this model, its merits and problems, and discusses whether it is time to lay this model to rest. Methods: A PubMed search was performed in March 2017 to identify articles for this review. Results: The olivary model gains support from the recognition of neurons with pacemaker property in the ION and the harmaline-induced tremor models (as the ION is the prime target of harmaline. However, the olivary model is problematic, as neurons with pacemaker property are not specific to the ION and the harmaline model does not completely represent the human disease ET. In addition, a large number of neuroimaging studies in ET have not detected structural or functional changes in the ION; rather, abnormalities have been reported in structures related to the cerebello-thalamo-cortical network. Moreover, a post-mortem study of microscopic changes in the ION did not detect any differences between ET cases and controls. Discussion: The olivary model largely remains a physiological construct. Numerous observations have cast considerable doubt as to the validity of this model in ET. Given the limitations of the model, we conclude that it is time now to lay this model to rest.

  7. Genetics Home Reference: familial hypertrophic cardiomyopathy

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    ... Home Health Conditions Familial hypertrophic cardiomyopathy Familial hypertrophic cardiomyopathy Printable PDF Open All Close All Enable Javascript ... view the expand/collapse boxes. Description Familial hypertrophic cardiomyopathy is a heart condition characterized by thickening (hypertrophy) ...

  8. Hypertrophic Cardiomyopathy: A Review

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    Houston, Brian A; Stevens, Gerin R

    2014-01-01

    Hypertrophic cardiomyopathy (HCM) is a global disease with cases reported in all continents, affecting people of both genders and of various racial and ethnic origins. Widely accepted as a monogenic disease caused by a mutation in 1 of 13 or more sarcomeric genes, HCM can present catastrophically with sudden cardiac death (SCD) or ventricular arrhythmias or insidiously with symptoms of heart failure. Given the velocity of progress in both the fields of heart failure and HCM, we present a review of the approach to patients with HCM, with particular attention to those with HCM and the clinical syndrome of heart failure. PMID:25657602

  9. Neuroanatomical dysmorphology of the medial superior olivary nucleus in sudden fetal and infant death

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    Lavezzi, Anna M.; Matturri, Luigi

    2012-01-01

    This study expands our understanding of the organization of the human caudal pons, providing a morphologic characterization of the medial superior olivary nucleus (MSO), component of the superior olivary complex (SOC) that plays an important role in the processing of acoustic information. We examined victims of sudden unexplained fetal and infant death and controls (n = 75), from 25 gestational weeks to 8 months of postnatal age, by complete autopsy and in-depth autonomic nervous system histological examination, particularly of the MSO nucleus, the focus of this study. Peculiar cytoarchitectural features of the MSO nucleus were found in sudden death cases, such as hypoplasia/agenesis and immature hypercellularity, frequently related to dysgenesis of contiguous structures involved in respiratory rhythm-generating circuit, in particular to hypoplasia of the retrotrapezoid and the facial nuclei. We propose the involvement of this nucleus in more important functions than those related to hearing, as breathing and, more extensively, all the vital activities. Besides, we highlight the fundamental role of the maternal smoking in pregnancy as etiological factor in the dysmorphic neuroanatomical development of the MSO nucleus. PMID:23205011

  10. Morphometric analysis of the neuronal numbers and densities of the inferior olivary complex in the donkey (Equus asinus).

    Science.gov (United States)

    Alkafafy, Mohamed; Rashed, Reda; Attia, Hossam

    2011-07-01

    The morphometric interrelations between the compartments of the inferior olivary complex (IOC) in the donkey (Equus asinus) were ascertained by examining serial sections throughout the entire length of the IOC for both sides. Nissl-stained celloidin sections of four brainstems of donkeys were used. The IOC consisted of three major nuclei and four small cell groups. The total neuronal count in both sides of the IOC was 202,040±8480 cells. The medial accessory olivary nucleus (MAO) had the largest relative area (46%) and the highest number of neurons (90,800±7600). The dorsal accessory olivary nucleus (DAO) had the second largest relative area (33%), while the principal olivary nucleus (PO) had the lowest relative area (21%). However, the total neuron count in the PO was larger (60,840±1840) than DAO (50,360±4040). The average neuronal density was 2700±400 cells/mm(3). The numerical values of the current study of the IOC in the donkey were similar to those of other mammals. Copyright © 2010 Elsevier GmbH. All rights reserved.

  11. Cerebellar Degeneration

    Science.gov (United States)

    ... Division of Neuroscience Director, NIH BRAIN Initiative® Health Scientist Administrator Channels Synapses Circuits Cluster Scientific Director, Division of Intramural Research Featured Director's Message menu search Enter Search Term Submit Search Cerebellar Degeneration Information ...

  12. Misconceptions and Facts About Hypertrophic Cardiomyopathy.

    Science.gov (United States)

    Argulian, Edgar; Sherrid, Mark V; Messerli, Franz H

    2016-02-01

    Hypertrophic cardiomyopathy is the most common genetic heart disease. Once considered relentless, untreatable, and deadly, it has become a highly treatable disease with contemporary management. Hypertrophic cardiomyopathy is one of cardiology's "great masqueraders." Mistakes and delays in diagnosis abound. Hypertrophic cardiomyopathy commonly "masquerades" as asthma, anxiety, mitral prolapse, and coronary artery disease. However, once properly diagnosed, patients with hypertrophic cardiomyopathy can be effectively managed to improve both symptoms and survival. This review highlights some of the misconceptions about hypertrophic cardiomyopathy. Providers at all levels should have awareness of hypertrophic cardiomyopathy to promptly diagnose and properly manage these individuals. Copyright © 2016 Elsevier Inc. All rights reserved.

  13. Mechanisms contributing to cluster formation in the inferior olivary nucleus in brainstem slices from postnatal mice

    DEFF Research Database (Denmark)

    Kølvraa, Mathias; Müller, Felix C; Jahnsen, Henrik

    2014-01-01

    The inferior olivary nucleus (IO) in in vitro slices from postnatal mice (P5.5-P15.5) spontaneously generates clusters of neurons with synchronous calcium transients, and intracellular recordings from IO neurons suggest that electrical coupling between neighbouring IO neurons may serve......-5-methyl-4-isoxazolepropionic acid receptors. Spikelets and a small transient depolarizing response were observed during glutamate-induced cluster formation. Calcium transients spread with decreasing velocity during cluster formation, and somatic action potentials and cluster formation...... are accompanied by large dendritic calcium transients. In conclusion, cluster formation depends on gap junctions, sodium action potentials and spontaneous clusters occur randomly throughout the IO. The relative slow signal spread during cluster formation, combined with a strong dendritic influx of calcium, may...

  14. Spontaneous cluster activity in the inferior olivary nucleus in brainstem slices from postnatal mice

    DEFF Research Database (Denmark)

    Rekling, Jens C; Reveles Jensen, Kristian; Jahnsen, Henrik

    2012-01-01

    A distinctive property of the cerebellar system is olivocerebellar modules, where synchronized electrical activity in neurons in the inferior olivary nucleus (IO) evokes organized activity in the cerebellar cortex. However, the exact function of these modules, and how they are developed, is still...... calcium transients. The spatiotemporal activity pattern was occasionally organized in clusters of co-active neighbouring neurons, with regular (16/min) and irregular (2-3/min) repeating cluster activity in the dmcc and PO, respectively. IO clusters had a diameter of 100-170 µm, lasted ~1 s, and increased......-active with separate clusters at different times. The coherence between calcium transients in IO neurons decreased with Euclidean distance between the cells reaching low values at 100-200 µm distances. Intracellular recordings from IO neurons during cluster formation revealed the presence of spikelet-like potentials...

  15. HYPERTROPHIC OBSTRUCTIVE CARDIOMYOPATHY

    Directory of Open Access Journals (Sweden)

    A. G. Osiev

    2015-01-01

    Full Text Available Hypertrophic cardiomyopathy (HCMP is a relatively common disease with genetic predisposition, that is widely spread irrespective of gender, race or ethnicity. The cause of this pathology are mutations of genes encoding synthesis of contracting proteins. Degree and type of mutations define clinical manifestation of the disease and its prognosis. HCMP is classified according to four main criteria: depending on morphology, presence of left ventricular outlet obstruction, pressure gradient and hemodynamic parameters. Its prevalence amounts to 1:500, and in the recent years mortality has decreased significantly to 1%. Main symptoms of HCMP include dyspnoea, dizziness, syncope, angina, and heart arrhythmias. HCMP does not manifest obligatorily with all above mentioned signs and symptoms. Presence and severity of any symptoms depend on morphological particulars of the disease. Particular attention should be paid to arrhythmias, with atrial fibrillation among them, that may cause hazardous and occasionally lethal complications. Electrocardiography and echocardiography are recognized as the “golden standard” of HCMP diagnostics, while in the recent years, magnetic resonance imaging has become a highly informative diagnostic tool. Radionuclide diagnostics is used less frequently, while physical examination and assessments have been moving backwards. At present, main strategies in HCMP include medical treatment with β-blockers, calcium antagonists, angiotensin-converting enzyme inhibitors and anti-arrhythmics. There are two techniques for surgical treatment, i.e. myectomy by Morrow and alcohol septal ablation that is becoming increasingly popular. The article reviews literature on state-of-the-art diagnostics and treatment of HCMP patients.

  16. DIFFERENTIAL DIAGNOSIS OF HYPERTROPHIC CARDIOMYOPATHY

    Directory of Open Access Journals (Sweden)

    I. V. Leontyeva

    2017-01-01

    Full Text Available Hypertrophic cardiomyopathy is the most common form of cardiomyopathy, occurring in childhood, occurring when a gene is mutated that encodes proteins of sarcomeric and non-sarcomeric complexes. The diagnosis of the disease is based on the data of echocardiography, revealing structural changes in the heart muscle according to the type of hypertrophy, while the genesis of these changes remains unclear. The causes of hypertrophic cardiomyopathy in childhood are diverse. Of great importance is the early diagnosis of metabolic forms of hypertrophic cardiomyopathy, so in some cases regress of hypertrophy is possible against the background of enzyme-substitution or other drug therapy. The article presents a clinical (cardiac and extracardiac symptoms and laboratory markers of hypertrophic cardiomyopathy with mutations of genes of proteins of the sarcomeric complex, congenital metabolic disorders (glycogenoses, lysosomal pathology, fatty acid metabolism disorders, and mitochondrial diseases, genetic syndromes (Noonan, LEOPARD, Costello, cardio-fascial-cutaneous, neuromuscular diseases. The criteria for differential diagnosis of genetic forms of hypertrophic cardiomyopathy and myocardial hypertrophy in athletes are presented. 

  17. Living with hypertrophic cardiomyopathy.

    Science.gov (United States)

    Subasic, Kim

    2013-12-01

    The purpose of this study is to provide an insider's account of what it is like to live with hypertrophic cardiomyopathy (HCM), a genetic cardiovascular illness that carries the risk for sudden cardiac death. This study aims to reveal how HCM impacts the family and guides the decision whether or not to pursue genetic testing, how the physical limitations associated with HCM alter being-in-the-world, and how HCM alters social relationships. Fifteen adults with HCM were recruited for a longitudinal, phenomenological, qualitative study through purposive sampling and word of mouth. A total of 45 interviews were conducted by the researcher at a time and place designated by the participant between August 2011 and January 2012. The first interview with each participant was conducted in person. While efforts were made to conduct all interviews in person, a total of three interviews were conducted by telephone as requested by three participants due to scheduling conflicts. Through methods of interpretive phenomenology, three audio-recorded, semistructured interviews occurred over the course of 3 months. Detailed narratives were solicited and transcribed verbatim. Methodological and analytical documentation was supported with the identification of key phrases, similar experiences, themes, and documentation of the rationale for decisions throughout the research process. Participation in genetic testing carries a multitude of personal, familial, financial, and emotional implications. The results of a genetic test elicited an emotional response regardless of whether the results were negative, positive, or inconclusive. Living with a potentially life-threatening illness altered identity, disrupted social relationships, and generated chronic fear and uncertainty. A new normal was re-ordered or transformed by the demands and limitations posed by HCM, and by the person's concerns, priorities, and the meaning of the illness. Results from this study underscore the need for healthcare

  18. Update on hypertrophic scar treatment

    Science.gov (United States)

    Rabello, Felipe Bettini; Souza, Cleyton Dias; Júnior, Jayme Adriano Farina

    2014-01-01

    Scar formation is a consequence of the wound healing process that occurs when body tissues are damaged by a physical injury. Hypertrophic scar/span>s and keloids are pathological scar/span>s resulting from abnormal responses to trauma and can be itchy and painful, causing serious functional and cosmetic disability. The current review will focus on the definition of hypertrophic scar/span>s, distinguishing them from keloids and on the various methods for treating hypertrophic scarring that have been described in the literature, including treatments with clearly proven efficiency and therapies with doubtful benefits. Numerous methods have been described for the treatment of abnormal scars, but to date, the optimal treatment method has not been established. This review will explore the differences between different types of nonsurgical management of hypertrophic scar/span>s, focusing on the indications, uses, mechanisms of action, associations and efficacies of the following therapies: silicone, pressure garments, onion extract, intralesional corticoid injections and bleomycin. PMID:25141117

  19. Familial idiopathic hypertrophic subaortic stenosis

    Directory of Open Access Journals (Sweden)

    Shah Lilam

    1979-01-01

    Full Text Available Echocardiographic features of idiopathic hypertrophic subaortic stenosis (IHSS are described. Systolic anterior motion (SAM of anterior mitral leaflet and asymetric septal hypertrophy (ASH are considered as the diagnostic criteria of IHSS. Effects of amyl nitrite and propranolol-a beta blocker are studied. Echocardio-graphic screening of family members revealed this as a case of fami-lial IHSS.

  20. Light-induced responses of slow oscillatory neurons of the rat olivary pretectal nucleus.

    Science.gov (United States)

    Szkudlarek, Hanna J; Orlowska, Patrycja; Lewandowski, Marian H

    2012-01-01

    The olivary pretectal nucleus (OPN) is a small midbrain structure responsible for pupil constriction in response to eye illumination. Previous electrophysiological studies have shown that OPN neurons code light intensity levels and therefore are called luminance detectors. Recently, we described an additional population of OPN neurons, characterized by a slow rhythmic pattern of action potentials in light-on conditions. Rhythmic patterns generated by these cells last for a period of approximately 2 minutes. To answer whether oscillatory OPN cells are light responsive and whether oscillatory activity depends on retinal afferents, we performed in vivo electrophysiology experiments on urethane anaesthetized Wistar rats. Extracellular recordings were combined with changes in light conditions (light-dark-light transitions), brief light stimulations of the contralateral eye (diverse illuminances) or intraocular injections of tetrodotoxin (TTX). We found that oscillatory neurons were able to fire rhythmically in darkness and were responsive to eye illumination in a manner resembling that of luminance detectors. Their firing rate increased together with the strength of the light stimulation. In addition, during the train of light pulses, we observed two profiles of responses: oscillation-preserving and oscillation-disrupting, which occurred during low- and high-illuminance stimuli presentation respectively. Moreover, we have shown that contralateral retina inactivation eliminated oscillation and significantly reduced the firing rate of oscillatory cells. These results suggest that contralateral retinal innervation is crucial for the generation of an oscillatory pattern in addition to its role in driving responses to visual stimuli.

  1. Malformation of the superior olivary complex in an animal model of autism.

    Science.gov (United States)

    Lukose, Richard; Schmidt, Elise; Wolski, Thomas P; Murawski, Nathen J; Kulesza, Randy J

    2011-06-29

    Autism is a neurodevelopmental disorder characterized by social difficulties, impaired communication skills and repetitive behavioral patterns. Additionally, there is evidence that auditory deficits are a common feature of the autism spectrum disorders. Despite the prevalence of autism, the neurobiology of this disorder is poorly understood. However, abnormalities in neuronal morphology, cell number and connectivity have been described throughout the autistic brain. Indeed, we have demonstrated significant dysmorphology in the superior olivary complex (SOC), a collection of auditory brainstem nuclei, in the autistic brain. Prenatal exposure to valproic acid (VPA) in humans has been associated with autism and in rodents prenatal VPA exposure produces many neuroanatomical and behavioral deficits associated with autism. Thus, in an effort to devise an animal model of the autistic auditory brainstem, we have investigated neuronal number and morphology in animals prenatally exposed to valproic acid (VPA). In VPA exposed rats, we find significantly fewer neurons and significant alterations in neuronal morphology. Thus, prenatal VPA exposure in rats appears to produce similar dysmorphology as we have reported in the autistic human brain. Copyright © 2011 Elsevier B.V. All rights reserved.

  2. Morphological study on the inferior olivary nuclear complex of the donkey (Equus asinus).

    Science.gov (United States)

    Rashed, Reda; Imagawa, Tomohiro; Uehara, Masato

    2006-12-01

    This study provides basic data on the normal structure of the inferior olivary complex (IOC) of the donkey, Equus asinus, at the light microscopic level. In common with that of other mammals, the donkey IOC consisted of three major nuclei and four minor groups of cells. The former was comprised of the medial and dorsal accessory olives (MAO and DAO, respectively) and the principal olive (PO), and the latter was comprised of the dorsal cap, nucleus beta, ventrolateral outgrowth and dorsomedial cell column. The MAO had the longest rostral to caudal representation and formed the caudal pole of IOC. The DAO was located dorsally to the MAO in the caudal half of the IOC. In the rostral half, the DAO bended ventrally and merged with the dorsal lamella of PO. More rostrally, the DAO lost its connection with the dorsal lamella and then conversely connected with the ventral lamella of PO. The DAO formed the rostral pole of the IOC. The PO extended through the rostral half of the IOC. The dorsal cap was a small group of cells. Overall, the donkey IOC is similar to that of other mammals.

  3. Macular degeneration (image)

    Science.gov (United States)

    Macular degeneration is a disease of the retina that affects the macula in the back of the eye. ... see fine details. There are two types of macular degeneration, dry and wet. Dry macular degeneration is more ...

  4. Hypertrophic osteoarthropathy in primary liver rhabdomyosarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Geary, T.R.; Maclennan, A.C.; Irwin, G.J. [Radiology Dept., Royal Hospital for Sick Children, Yorkhill NHS Trust, Glasgow (United Kingdom)

    2004-03-01

    Hypertrophic osteoarthropathy is a well-documented paraneoplastic phenomenon in adults. It is a rare, but important finding in children with malignant disease as it can indicate prognosis. We present a case of hypertrophic osteoarthropathy associated with primary liver rhabdomyosarcoma in a 14-year-old boy. (orig.)

  5. Idiopathic hypertrophic pachymeningitis presenting with occipital ...

    African Journals Online (AJOL)

    Abstract: Background: Although occipital neuralgia is usually caused by degenerative arthropathy, nearly 20 other aetiologies may lead to this condition. Methods: We present the first case report of hypertrophic pachymeningitis revealed by isolated occipital neuralgia. Results and conclusions: Idiopathic hypertrophic ...

  6. Idiopathic hypertrophic pachymeningitis presenting with occipital ...

    African Journals Online (AJOL)

    Background: Although occipital neuralgia is usually caused by degenerative arthropathy, nearly 20 other aetiologies may lead to this condition. Methods: We present the first case report of hypertrophic pachymeningitis revealed by isolated occipital neuralgia. Results and conclusions: Idiopathic hypertrophic ...

  7. Subendocardial ischemia in hypertrophic cardiomyopathy.

    Science.gov (United States)

    Kawasaki, Tatsuya; Sugihara, Hiroki

    2014-02-01

    Hypertrophic cardiomyopathy (HCM) patients often develop subendocardial ischemia in the left ventricle without atherosclerotic coronary stenosis. Myocardial ischemia plays an important role in the pathophysiology of HCM, but diagnostic techniques for the detection of subendocardial ischemia have not been widely available. We developed specific techniques to quantify subendocardial ischemia on stress scintigraphy, and have compared the results with various clinical features in patients with HCM. This article reviews our understanding of subendocardial ischemia in HCM based on more than 20 years of experience. Copyright © 2013 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.

  8. Hypertrophic Nonunion Humerus Mimicking an Enchondroma

    Directory of Open Access Journals (Sweden)

    N. K. Magu

    2014-01-01

    Full Text Available Introduction. Although fractures of humeral shaft show excellent results with conservative management, nonunion does occur. Case Report. We bring forth the case of a young male with a 1.5-year-old hypertrophic nonunion of the humerus mimicking an enchondroma. The initial X-ray images of the patient appeared to be an enchondroma, which only on further evaluation and histopathological analysis was diagnosed conclusively to be a hypertrophic nonunion. Discussion. Enchondromas are often incidentally diagnosed benign tumours. It is however not common to misdiagnose a hypertrophic nonunion to be an enchondroma. We present this case to highlight the unique diagnostic dilemma the treating team had to face.

  9. Hypertrophic lupus vulgaris: an unusual presentation.

    Science.gov (United States)

    Jain, Vijay K; Aggarwal, Kamal; Jain, Sarika; Singh, Sunita

    2009-07-01

    Lupus vulgaris is the most common form of cutaneous tuberculosis occurring in previously sensitized individuals with a high degree of tuberculin sensitivity. Various forms including plaque, ulcerative, hypertrophic, vegetative, papular, and nodular forms have been described. A 30-year-old male patient presented with a very large hypertrophic lupus vulgaris lesion over left side of chest since 22 years. Histopathological examination showed granulomatous infiltration without caseation necrosis. The Mantoux reaction was strongly positive. Hypertrophic lupus vulgaris of such a giant size and that too at an unusual site is extremely rare and hence is being reported.

  10. Hypertrophic cardiomyopathy screening in young athletes

    Energy Technology Data Exchange (ETDEWEB)

    Rappoport, W.J. [Arizona Heart Inst., Phoenix, AZ (United States); Steingard, P.M. [Phoenix Suns, Phoenix, AZ (United States)

    2006-07-01

    Hypertrophic cardiomyopathy is the leading cause of sudden death during vigorous exercise. Early identification of this abnormality by ECG screening of high-school athletes before they participate in competitive sports helps save lives. (orig.)

  11. Verrucous (Hypertrophic) Cutaneous Lupus Erythematosus: A Case ...

    African Journals Online (AJOL)

    Abstract. Verrucous (Hypertrophic) Lupus Erythematosus (LE) represents a rare but distinct, variant of chronic discoid lupus erythematosus. We report a case of LE with verrucous lesions for its rarity and peculiar location posing a diagnostic dilemma.

  12. Hypoxia Inhibits Hypertrophic Differentiation and Endochondral Ossification in Explanted Tibiae

    NARCIS (Netherlands)

    Leijten, Jeroen Christianus Hermanus; Moreira Teixeira, Liliana; Landman, Ellie; van Blitterswijk, Clemens; Karperien, Hermanus Bernardus Johannes

    2012-01-01

    Purpose: Hypertrophic differentiation of growth plate chondrocytes induces angiogenesis which alleviates hypoxia normally present in cartilage. In the current study, we aim to determine whether alleviation of hypoxia is merely a downstream effect of hypertrophic differentiation as previously

  13. Prevention and curative management of hypertrophic scar formation

    NARCIS (Netherlands)

    Bloemen, M.C.; Veer, van der W.M.; Ulrich, M.; Zuijlen, van P.P.; Niessen, F.B.; Middelkoop, E.

    2009-01-01

    Although hypertrophic scarring commonly occurs following burns, many aspects such as incidence of and optimal treatment for scar hypertrophy remain unclear. This review will focus on hypertrophic scar formation after burn in particular, exploring multiple treatment options and describing their

  14. Cerebellar nuclei neurons show only small excitatory responses to optogenetic olivary stimulation in transgenic mice: in vivo and in vitro studies

    Directory of Open Access Journals (Sweden)

    Huo eLu

    2016-03-01

    Full Text Available To study the olivary input to the cerebellar nuclei (CN we used optogenetic stimulation in transgenic mice expressing channelrhodopsin-2 (ChR2 in olivary neurons. We obtained in vivo extracellular Purkinje cell (PC and CN recordings in anesthetized mice while stimulating the contralateral inferior olive (IO with a blue laser (single pulse, 10 - 50 ms duration. Peri-stimulus histograms were constructed to show the spike rate changes after optical stimulation. Among 29 CN neurons recorded, 15 showed a decrease in spike rate of variable strength and duration, and only 1 showed a transient spiking response. These results suggest that direct olivary input to CN neurons is usually overridden by stronger Purkinje cell inhibition triggered by climbing fiber responses. To further investigate the direct input from the climbing fiber collaterals we also conducted whole cell recordings in brain slices, where we used local stimulation with blue light. Due to the expression of ChR2 in Purkinje cell axons as well as the IO in our transgenic line, strong inhibitory responses could be readily triggered with optical stimulation (13 of 15 neurons. After blocking this inhibition with GABAzine, only in 5 of 13 CN neurons weak excitatory responses were revealed. Therefore our in vitro results support the in vivo findings that the excitatory input to CN neurons from climbing fiber collaterals in adult mice is masked by the inhibition under normal conditions.

  15. Apical Hypertrophic Cardiomyopathy in Association with PulmonaryArtery Hypertension

    Directory of Open Access Journals (Sweden)

    Mehdi Peighambari

    2012-09-01

    Full Text Available Apical Hypertrophic Cardiomyopathy is an uncommon condition constituting 1% -2% of the cases with Hypertrophic Cardiomyopathy (HCM diagnosis. We interestingly report two patients with apical hypertrophic cardiomyopathy in association with significant pulmonary artery hypertension without any other underlying reason for pulmonary hypertension. The patients were assessed by echocardiography, cardiac catheterization and pulmonary function parameters study.

  16. Apical Hypertrophic Cardiomyopathy in Association with Pulmonary Artery Hypertension

    OpenAIRE

    Peighambari, Mehdi; Parsaei, Mozghan; Sadeghpour, Anita; Alizadehasl, Azin

    2012-01-01

    Apical Hypertrophic Cardiomyopathy is an uncommon condition constituting 1% -2% of the cases with Hypertrophic Cardiomyopathy (HCM) diagnosis. We interestingly report two patients with apical hypertrophic cardiomyopathy in association with significant pulmonary artery hypertension without any other underlying reason for pulmonary hypertension. The patients were assessed by echocardiography, cardiac catheterization and pulmonary function parameters study.

  17. Multimodality imaging in apical hypertrophic cardiomyopathy

    Science.gov (United States)

    Parisi, Rosario; Mirabella, Francesca; Secco, Gioel Gabrio; Fattori, Rossella

    2014-01-01

    Apical hypertrophic cardiomyopathy (AHCM) is a relatively rare morphologic variant of HCM in which the hypertrophy of myocardium is localized to the left ventricular apex. Symptoms of AHCM might vary from none to others mimic coronary artery disease including acute coronary syndrome, thus resulting in inappropriate hospitalization. Transthoracic echocardiography is the first-line imaging technique for the diagnosis of hypertrophic cardiomyopathies. However, when the hypertrophy of the myocardium is localized in the ventricular apex might results in missed diagnosis. Aim of this paper is to review the different imaging techniques used for the diagnosis of AHCM and their role in the detection and comprehension of this uncommon disease. PMID:25276293

  18. On the surgical treatment of hypertrophic scars: a comprehensive guideline for the surgical treatment of hypertrophic scars

    NARCIS (Netherlands)

    Stekelenburg, C.M.; van der Wal, M.B.A.; Middelkoop, E.; Niessen, F.B.; van Zuijlen, P.P.M.

    2012-01-01

    Background: Treating the hypertrophic scar remains a challenging problem. It often leaves the clinician with the questions whether, when and how to treat a hypertrophic scar. Methods: This review will focus on the surgical treatment of hypertrophic scars considering the latest research in this

  19. Neuropathology of the Guillain-Mollaret Triangle (Dentato-Rubro-Olivary Network) in Sudden Unexplained Perinatal Death and SIDS

    Science.gov (United States)

    Lavezzi, Anna Maria; Corna, Melissa; Matturri, Luigi; Santoro, Franco

    2009-01-01

    The present study was undertaken to evaluate the possible alterations of the triangle of Guillain and Mollaret (G-Mt), a neuronal brainstem/cerebellum network (from the dentate nucleus to red nucleus and inferior olivary nucleus) already known for its involvement in the pathogenesis of the palatal myoclonus, in sudden unexplained perinatal and infant death. In 44 cases of perinatal and infant death victims, aged from 26 gestational weeks to 10 months of life, we investigated, besides the histological morphology of the three nuclei, the c-fos and apoptotic expression, as well as the possible effects elicited by maternal cigarette smoking. A significant increase of lesions (hypoplasia and/or increased c-fos and apoptotic neuronal immunopositivity) of the three nuclei was found in unexplained death victims, compared with age-matched controls. These alterations were related to maternal cigarette smoking habit. We postulated that the G-Mt is an important network involved in the pathogenesis of a wide spectrum of pathological manifestations and that maternal smoking during pregnancy can exert an adverse influence on this complex, even leading to sudden death in vulnerable periods of perinatal or infant development. PMID:19597559

  20. Contractile Dysfunction in Sarcomeric Hypertrophic Cardiomyopathy.

    Science.gov (United States)

    MacIver, David H; Clark, Andrew L

    2016-09-01

    The pathophysiological mechanisms underlying the clinical phenotype of sarcomeric hypertrophic cardiomyopathy are controversial. The development of cardiac hypertrophy in hypertension and aortic stenosis is usually described as a compensatory mechanism that normalizes wall stress. We suggest that an important abnormality in hypertrophic cardiomyopathy is reduced contractile stress (the force per unit area) generated by myocardial tissue secondary to abnormalities such as cardiomyocyte disarray. In turn, a progressive deterioration in contractile stress provokes worsening hypertrophy and disarray. A maintained or even exaggerated ejection fraction is explained by the increased end-diastolic wall thickness producing augmented thickening. We propose that the nature of the hemodynamic load in an individual with hypertrophic cardiomyopathy could determine its phenotype. Hypertensive patients with hypertrophic cardiomyopathy are more likely to develop exaggerated concentric hypertrophy; athletic individuals an asymmetric pattern; and inactive individuals a more apical hypertrophy. The development of a left ventricular outflow tract gradient and mitral regurgitation may be explained by differential regional strain resulting in mitral annular rotation. Copyright © 2016. Published by Elsevier Inc.

  1. Functional effects of losartan in hypertrophic cardiomyopathy

    DEFF Research Database (Denmark)

    Axelsson, Anna Karin Irene; Iversen, Kasper; Vejlstrup, Niels G.

    2016-01-01

    OBJECTIVE: There is a lack of disease-modifying treatments in hypertrophic cardiomyopathy (HCM). The aim of this randomised, placebo-controlled study was to assess if losartan could improve or ameliorate deterioration of cardiac function and exercise capacity. METHODS: Echocardiography, exercise...

  2. Fabry Disease in Families With Hypertrophic Cardiomyopathy

    DEFF Research Database (Denmark)

    Adalsteinsdottir, Berglind; Palsson, Runolfur; Desnick, Robert J

    2017-01-01

    BACKGROUND: The screening of Icelandic patients clinically diagnosed with hypertrophic cardiomyopathy resulted in identification of 8 individuals from 2 families with X-linked Fabry disease (FD) caused by GLA(α-galactosidase A gene) mutations encoding p.D322E (family A) or p.I232T (family B...

  3. Pregnancy in women with hypertrophic cardiomyopathy

    NARCIS (Netherlands)

    Pieper, P. G.; Walker, F.

    Hypertrophic cardiomyopathy (HCM) is increasingly being diagnosed in pregnant women. Women with HCM generally tolerate pregnancy well. The risk is however higher in women who are symptomatic before pregnancy or in those with severe left ventricular outflow tract obstruction. The incidence of

  4. Alcohol septal ablation for obstructive hypertrophic cardiomyopathy

    NARCIS (Netherlands)

    Steggerda, Robbert

    2015-01-01

    Hypertrophic cardiomyopathy is an inhered heart disease characterised by a thickened heart muscle, common in 1:500 persons. Obstruction of blood flow in the heart due the thickened heart muscle can occur and cause dyspnoea. A heart surgeon can cut away part of the thickened muscle (myectomy). With

  5. Hypertrophic Cardiomyopathy: Pathophysiology, Genetics and Invasive Treatment

    NARCIS (Netherlands)

    M. Michels (Michelle)

    2011-01-01

    textabstractHypertrophic cardiomyopathy (HCM) is the most common inheritable cardiac disorder with a phenotypic prevalence of 1:500. It is defined by the presence of left ventricular hypertrophy (LVH) in the absence of loading conditions (hypertension, valve disease) sufficient to cause the observed

  6. Symptomatic palatal tremor is not limited to the activity of guillain-mollaret triangle: A functional magnetic resonance imaging case study

    OpenAIRE

    Balaban, Hatice; Kayım Yıldız, Özlem; Öztoprak, İbrahim; Bolayır, Ertuğrul; Topaktaş, Suat

    2012-01-01

    Abstract A 57-year-old man was admitted to our clinic because of speech and swallowing disorder for six months. Palatal tremor and left upper extremity myorhythmia appeared on examination. Magnetic resonance imaging showed hypertrophic olivary degeneration, dentate pathology, Wallerian degeneration, and left parietal cystic encephalomalacia. Functional magnetic resonance imaging study of the brain showed prominent activation on the bilateral globus pallidus and thalamus. We suggest that invol...

  7. Degenerate Gauss hypergeometric functions

    OpenAIRE

    Vidunas, Raimundas

    2007-01-01

    This is a study of terminating and ill-defined Gauss hypergeometric functions. Corresponding hypergeometric equations have a degenerate set of of 24 Kummer's solutions. We describe those solutions and relations between them.

  8. Evaluation of left ventricular torsion in children with hypertrophic cardiomyopathy.

    Science.gov (United States)

    Prinz, Christian; Faber, Lothar; Horstkotte, Dieter; Körperich, Hermann; Moysich, Axel; Haas, Nikolaus; Kececioglu, Deniz; Thorsten Laser, Kai

    2014-04-01

    To evaluate the role of torsion in hypertrophic cardiomyopathy in children. A total of 88 children with idiopathic hypertrophic cardiomyopathy (n = 24) and concentric hypertrophy (n = 20) were investigated with speckle-tracking echocardiography and compared with age- and gender-matched healthy controls (n = 44). In hypertrophic cardiomyopathy, we found increased torsion (2.8 ± 1.6 versus 1.9 ± 1.0°/cm [controls], p Hypertrophic cardiomyopathy patients demonstrated a negative correlation between left ventricular muscle mass and torsion (r = -0.7, p hypertrophic cardiomyopathy is characterised by predominantly enhanced systolic basal clockwise rotation. Diastolic untwisting is delayed in both groups. Torsion may be an interesting marker to guide patients with hypertrophic cardiomyopathy.

  9. Extensive hypertrophic lupus erythematosus: Atypical presentation

    Directory of Open Access Journals (Sweden)

    Tarun Narang

    2012-01-01

    Full Text Available Lupus erythematosus (LE is a disease with a wide spectrum of cutaneous and systemic manifestations. Clinical features of patients with LE show a great variation, and for this reason it is difficult to develop a unifying concept of this disease. Our objective is to present a case of hypertrophic LE with atypical morphology and extensive involvement, who responded favorably to isotretinoin. Diagnosis of hypertrophic lupus erythematosus (HLE was confirmed by characteristic histopathological findings. Combination therapy with isotretinoin and hydroxychloroquine resulted in flattening and repression of previously refractory skin lesions. Sometimes, HLE lesions may present a diagnostic and therapeutic dilemma. In long standing lesions, squamous cell carcinoma may arise. Therefore, HLE requires adequate therapy with clinical and histopathological follow up.

  10. Hypertrophic cardiomyopathy: Part 1 - Introduction, pathology and pathophysiology

    OpenAIRE

    Praveen Kerala Varma; Praveen Kumar Neema

    2014-01-01

    Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiovascular disease with many genotype and phenotype variations. Earlier terminologies, hypertrophic obstructive cardiomyopathy and idiopathic hypertrophic sub-aortic stenosis are no longer used to describe this entity. Patients present with or without left ventricular outflow tract (LVOT) obstruction. Resting or provocative LVOT obstruction occurs in 70% of patients and is the most common cause of heart failure. The pathology an...

  11. Primary hypertrophic osteoarthropathy: ultrasound and MRI findings

    Energy Technology Data Exchange (ETDEWEB)

    Adams, Brook; Kraft, Jeannette K. [Leeds Children' s Hospital at The Leeds General Infirmary, Clarendon Wing Radiology Department, Leeds, West Yorkshire (United Kingdom); Amin, Tania; Leone, Valentina; Wood, Mark [Leeds Children' s Hospital at The Leeds General Infirmary, Department of Paediatric Rheumatology, Leeds (United Kingdom)

    2016-05-15

    Primary hypertrophic osteoarthropathy is a rare genetic disorder related to failures in prostaglandin metabolism. Patients present with joint pain, limb enlargement, skin thickening and finger clubbing. Radiographs show characteristic periosteal reaction and thickening along the long bones. We present MRI and US findings in a child with the condition. Ultrasound showed echogenic tissue surrounding the long bones, presumably reflecting oedema and inflammatory tissue. Doppler sonograms demonstrated increased vascularity on the surface of some superficial bony structures. (orig.)

  12. The embryological basis of subclinical hypertrophic cardiomyopathy.

    Science.gov (United States)

    Captur, Gabriella; Ho, Carolyn Y; Schlossarek, Saskia; Kerwin, Janet; Mirabel, Mariana; Wilson, Robert; Rosmini, Stefania; Obianyo, Chinwe; Reant, Patricia; Bassett, Paul; Cook, Andrew C; Lindsay, Susan; McKenna, William J; Mills, Kevin; Elliott, Perry M; Mohun, Timothy J; Carrier, Lucie; Moon, James C

    2016-06-21

    Hypertrophic cardiomyopathy (HCM) is caused by mutations in sarcomeric proteins, the commonest being MYBPC3 encoding myosin-binding protein C. It is characterised by left ventricular hypertrophy but there is an important pre-hypertrophic phenotype with features including crypts, abnormal mitral leaflets and trabeculae. We investigated these during mouse cardiac development using high-resolution episcopic microscopy. In embryonic hearts from wildtype, homozygous (HO) and heterozygous (HET) Mybpc3-targeted knock-out (KO) mice we show that crypts (one or two) are a normal part of wildtype development but they almost all resolve by birth. By contrast, HO and HET embryos had increased crypt presence, abnormal mitral valve formation and alterations in the compaction process. In scarce normal human embryos, crypts were sometimes present. This study shows that features of the human pre-hypertrophic HCM phenotype occur in the mouse. In an animal model we demonstrate that there is an embryological HCM phenotype. Crypts are a normal part of cardiac development but, along with the mitral valve and trabeculae, their developmental trajectory is altered by the presence of HCM truncating Mybpc3 gene mutation.

  13. Outcomes of acute myocardial infarction in patients with hypertrophic cardiomyopathy.

    Science.gov (United States)

    Gupta, Tanush; Harikrishnan, Prakash; Kolte, Dhaval; Khera, Sahil; Aronow, Wilbert S; Mujib, Marjan; Palaniswamy, Chandrasekar; Sule, Sachin; Jain, Diwakar; Ahmed, Ali; Lanier, Gregg M; Cooper, Howard A; Frishman, William H; Fonarow, Gregg C; Panza, Julio A

    2015-08-01

    Acute myocardial infarction is a recognized complication in patients with hypertrophic cardiomyopathy. However, limited data are available on outcomes of patients with hypertrophic cardiomyopathy and acute myocardial infarction. We analyzed the 2003-2011 Nationwide Inpatient Sample databases to identify all patients aged ≥18 years with a principal diagnosis of acute myocardial infarction. Patients with a concomitant diagnosis of hypertrophic cardiomyopathy were then identified and analyzed as a separate cohort. Multivariate logistic regression was used to compare outcomes in patients with acute myocardial infarction with and without hypertrophic cardiomyopathy. Of 5,901,827 patients with acute myocardial infarction, 5688 (0.1%) had a diagnosis of hypertrophic cardiomyopathy. Patients with hypertrophic cardiomyopathy were older, more likely to be female, and less likely to have traditional cardiovascular risk factors. Compared with patients without hypertrophic cardiomyopathy, patients with hypertrophic cardiomyopathy were less likely to present with ST-elevation myocardial infarction and more likely to present with non-ST-elevation myocardial infarction. Patients with hypertrophic cardiomyopathy with ST-elevation myocardial infarction or non-ST-elevation myocardial infarction were less likely to receive revascularization. In the overall population with acute myocardial infarction, there was no difference in risk-adjusted in-hospital mortality between patients with and without hypertrophic cardiomyopathy (odds ratio [OR], 0.96; 95% confidence interval [CI], 0.84-1.11; P = .59). In the population with ST-elevation myocardial infarction, patients with hypertrophic cardiomyopathy had lower risk-adjusted in-hospital mortality than those without hypertrophic cardiomyopathy (OR, 0.75; 95% CI, 0.63-0.91; P = .003), whereas in the population with non-ST-elevation myocardial infarction, there was no difference in risk-adjusted in-hospital mortality between patients with

  14. Currently known risk factors for hypertrophic skin scarring : A review

    NARCIS (Netherlands)

    Butzelaar, L.; Ulrich, M. M W; Mink Van Der Molen, A. B.; Niessen, F. B.; Beelen, R. H J

    2016-01-01

    SummaryObjective The study aims to provide an overview of risk factors for hypertrophic scarring. Background Hypertrophic skin scarring remains a major concern in medicine and causes considerable morbidity. Despite extensive research on this topic, the precise mechanism of excessive scarring is

  15. Topical modalities for treatment and prevention of postsurgical hypertrophic scars.

    Science.gov (United States)

    Foo, Chong Wee; Tristani-Firouzi, Payam

    2011-08-01

    There is no universally accepted treatment regimen and no evidence-based literature to guide management of hypertrophic scars. This article summarizes the existing literature regarding topical treatments such as silicone gel sheeting and ointment, onion extract, vitamin E, pressure garment therapy, massage therapy, and topical imiquimod 5% cream in the management of hypertrophic scars. 2011 Elsevier Inc. All rights reserved.

  16. The KCNE genes in hypertrophic cardiomyopathy: a candidate gene study

    DEFF Research Database (Denmark)

    Hedley, Paula L; Haundrup, Ole; Andersen, Paal S

    2011-01-01

    as well as the T-tubules of the sarcolemma. It has been suggested that minK forms part of an "electro-mechanical feed-back" which links cardiomyocyte stretching to changes in ion channel function. We examined whether mutations in KCNE genes were associated with hypertrophic cardiomyopathy (HCM), a genetic...... disease associated with an improper hypertrophic response....

  17. Septal alcoholization in hypertrophic cardiomyopathy: about 11 cases

    African Journals Online (AJOL)

    Outcomes of septal alcoholization in hypertrophic obstructive cardiomyopathy are not enough studied in all centers. The purpose of this study was to determine the outcomes of septal alcoholization in hypertrophic obstructive cardiomyopathy in our hospital. A retrospective and prospective descriptive study focused on all ...

  18. Inhibited early immunologic response is associated with hypertrophic scarring

    NARCIS (Netherlands)

    Butzelaar, Liselotte; Schooneman, Dennis P. M.; Soykan, Ezgi A.; Talhout, Wendy; Ulrich, Magda M. W.; van den Broek, Lenie J.; Gibbs, Susan; Beelen, Robert H. J.; van der Molen, Aebele B. Mink; Niessen, Frank B.

    2016-01-01

    This study aimed to examine changes in the inflammatory response in early hypertrophic compared to normal wound healing. The immune system is thought to be involved in hypertrophic scar formation. However, the exact mechanism and time of onset of the derailment remain unknown. In a prospective

  19. Psychosocial impact of specialized cardiac genetic clinics for hypertrophic cardiomyopathy.

    Science.gov (United States)

    Ingles, Jodie; Lind, Joanne M; Phongsavan, Philayrath; Semsarian, Christopher

    2008-02-01

    The diagnosis of hypertrophic cardiomyopathy, an autosomal dominant chronic heart disease, can have significant implications, including increased risk of sudden death, exercise limitations, and risk of transmission to offspring. This study sought to describe the psychosocial factors associated with attending a specialty cardiac genetic clinic, and to determine whether these may be predictors of comorbid anxiety and depression in this population. Questionnaires were sent to 184 individuals attending the Royal Prince Alfred Hospital Hypertrophic Cardiomyopathy Clinic. Questionnaires were anonymous and comprised demographics, the Hospital Anxiety and Depression Scale, Patient Experience Scales, and Patient Satisfaction Scales. Completed questionnaires were returned by 109 participants (59.2% response rate), of which 76.9% had a diagnosis of hypertrophic cardiomyopathy, while 23.1% were at-risk relatives attending for clinical screening. Patient satisfaction scores were generally high to very high across all groups, though only 24% of HCM patients showed good adjustment to hypertrophic cardiomyopathy and 10% had low worry about hypertrophic cardiomyopathy scores. Within the disease group, logistic regression analysis adjusting for age, gender, and education revealed adjustment to hypertrophic cardiomyopathy and worry about hypertrophic cardiomyopathy scores to be significantly associated with anxiety, while adjustment scores and location of patient follow-up (i.e., Hypertrophic Cardiomyopathy clinic or another cardiologist) to be significantly associated with depression scores. HCM patients who attend specialized cardiac genetic clinics are better adjusted and worry less, than those who do not attend. An integrated approach, including a genetic counselor, is important in the management of HCM families.

  20. The Role of Echocardiography in Hypertrophic Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Jing Ping Sun

    2017-02-01

    Full Text Available Hypertrophic cardiomyopathy (HCM is a common genetic cardiovascular disease and appears in all ethnic groups. HCM is diagnosed on the basis of left ventricular hypertrophy. Echocardiography is a key technique in the diagnosis of HCM, the prognosis of patients with HCM, the management strategy for HCM, and the follow-up of patients with HCM. This review briefly describes and discusses the practical use of established echocardiography techniques and the current and emerging echocardiographic methods that can help physicians in the correct diagnostic and pathophysiological assessment of patients with HCM.

  1. Hypertrophic Cardiomyopathy—Past, Present and Future

    Directory of Open Access Journals (Sweden)

    Alphonsus C. Liew

    2017-12-01

    Full Text Available Hypertrophic cardiomyopathy (HCM is the most common genetic cardiomyopathy with a prevalence of 1 in 500 in the general population. Since the first pathological case series at post mortem in 1957, we have come a long way in its understanding, diagnosis and management. Here, we will describe the history of our understanding of HCM including the initial disease findings, diagnostic methods and treatment options. We will review the current guidelines for the diagnosis and management of HCM, current gaps in the evidence base and discuss the new and promising developments in this field.

  2. Blepharoptosis and hypertrophic osteoarthropathy: A case report

    Directory of Open Access Journals (Sweden)

    Aysun Sanal Dogan

    2016-01-01

    Full Text Available A 52-year-old male patient presented to our hospital with a history of secondary hypertrophic osteoarthropathy (HOA associated with an abdominal neoplasia and blepharoptosis. He had finger clubbing, hyperhidrosis, and hypertrichosis. He also had a recent history of extensive abdominal surgery with a pathology report of myelolipoma. Routine blood work was unremarkable. Upper eyelid reconstruction with blepharoplasty, upper eyelid wedge resection, and brow suspension was performed to address his eyelid concerns. By this case report, we would like to attract notice that the eyelid involvement may be a part of HOA and to emphasize the importance of systemic and pathologic evaluation in failed blepharoptosis surgery.

  3. Hypertrophic Cardiomyopathy—Past, Present and Future

    Science.gov (United States)

    Liew, Alphonsus C.; Vassiliou, Vassilios S.; Cooper, Robert; Raphael, Claire E.

    2017-01-01

    Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiomyopathy with a prevalence of 1 in 500 in the general population. Since the first pathological case series at post mortem in 1957, we have come a long way in its understanding, diagnosis and management. Here, we will describe the history of our understanding of HCM including the initial disease findings, diagnostic methods and treatment options. We will review the current guidelines for the diagnosis and management of HCM, current gaps in the evidence base and discuss the new and promising developments in this field. PMID:29231893

  4. Kraepelin and degeneration theory.

    Science.gov (United States)

    Hoff, Paul

    2008-06-01

    Emil Kraepelin's contribution to the clinical and scientific field of psychiatry is recognized world-wide. In recent years, however, there have been a number of critical remarks on his acceptance of degeneration theory in particular and on his political opinion in general, which was said to have carried "overtones of proto-fascism" by Michael Shepherd [28]. The present paper discusses the theoretical cornerstones of Kraepelinian psychiatry with regard to their relevance for Kraepelin's attitude towards degeneration theory. This theory had gained wide influence not only in scientific, but also in philosophical and political circles in the last decades of the nineteenth century. There is no doubt that Kraepelin, on the one hand, accepted and implemented degeneration theory into the debate on etiology and pathogenesis of mental disorders. On the other hand, it is not appropriate to draw a simple and direct line from early versions of degeneration theory to the crimes of psychiatrists and politicians during the rule of national socialism. What we need, is a differentiated view, since this will be the only scientific one. Much research needs to be done here in the future, and such research will surely have a significant impact not only on the historical field, but also on the continuous debate about psychiatry, neuroscience and neurophilosophy.

  5. American Macular Degeneration Foundation

    Science.gov (United States)

    ... Macular Degeneration How The Eye Works as a Camera Anatomy of a Normal Human Eye ADA and ... Low Vision Depression and AMD Service Providers Research & Cases AMD Research Genetics of AMD Complement Factor H (CFH) Study on Lutein and AMD Study on Zeaxanthin and ...

  6. Hypoxia inhibits hypertrophic differentiation and endochondral ossification in explanted tibiae.

    Directory of Open Access Journals (Sweden)

    Jeroen C H Leijten

    Full Text Available Hypertrophic differentiation of growth plate chondrocytes induces angiogenesis which alleviates hypoxia normally present in cartilage. In the current study, we aim to determine whether alleviation of hypoxia is merely a downstream effect of hypertrophic differentiation as previously described or whether alleviation of hypoxia and consequent changes in oxygen tension mediated signaling events also plays an active role in regulating the hypertrophic differentiation process itself.Fetal mouse tibiae (E17.5 explants were cultured up to 21 days under normoxic or hypoxic conditions (21% and 2.5% oxygen respectively. Tibiae were analyzed on growth kinetics, histology, gene expression and protein secretion.The oxygen level had a strong influence on the development of explanted fetal tibiae. Compared to hypoxia, normoxia increased the length of the tibiae, length of the hypertrophic zone, calcification of the cartilage and mRNA levels of hypertrophic differentiation-related genes e.g. MMP9, MMP13, RUNX2, COL10A1 and ALPL. Compared to normoxia, hypoxia increased the size of the cartilaginous epiphysis, length of the resting zone, calcification of the bone and mRNA levels of hyaline cartilage-related genes e.g. ACAN, COL2A1 and SOX9. Additionally, hypoxia enhanced the mRNA and protein expression of the secreted articular cartilage markers GREM1, FRZB and DKK1, which are able to inhibit hypertrophic differentiation.Collectively our data suggests that oxygen levels play an active role in the regulation of hypertrophic differentiation of hyaline chondrocytes. Normoxia stimulates hypertrophic differentiation evidenced by the expression of hypertrophic differentiation related genes. In contrast, hypoxia suppresses hypertrophic differentiation of chondrocytes, which might be at least partially explained by the induction of GREM1, FRZB and DKK1 expression.

  7. The 50-Year History, Controversy, and Clinical Implications of Left Ventricular Outflow Tract Obstruction in Hypertrophic Cardiomyopathy: From Idiopathic Hypertrophic Subaortic Stenosis to Hypertrophic Cardiomyopathy

    National Research Council Canada - National Science Library

    Barry J Maron; Martin S Maron; E Douglas Wigle; Eugene Braunwald

    2009-01-01

    ...) clinical descriptions of hypertrophic cardiomyopathy (HCM) and has proved to be a complex phenomenon unique in many respects, as well as arguably the most visible and well-known pathophysiologic component of this heterogeneous disease...

  8. Apical hypertrophic cardiomyopathy: clinical and metabolic studies.

    Science.gov (United States)

    Bertrand, M E; Tilmant, P Y; Lablanche, J M; Thieuleux, F A

    1983-11-01

    Among the hypertrophic non-obstructive cardiomyopathies, a particular group of patients with concentric apical hypertrophy can be described. We studied seven patients (five men and two women) who underwent heart catheterization because they had giant negative T waves in the precordial leads. M-mode and two-dimensional echocardiograms revealed no obstruction within the outflow tract of the ventricle. Coronary angiography was normal in all cases. None of these patients demonstrated any significant peak systolic pressure gradient in the outflow tract. A characteristic spade-like configuration (concentric apical hypertrophy) was observed in the right anterior oblique ventriculogram at end diastole. The apical thickness reached 17.2 +/- 0.85 mm and was significantly greater than mid-anterior wall thickness (9.8 +/- 2.14 mm). In five cases, atrial pacing with coronary arterial and venous lactate sampling revealed abnormalities in myocardial metabolism. With a mean follow up of 43 months, three patients remain asymptomatic and one had heart failure. ECG abnormalities were unchanged and echocardiograms showed an increase of the septal and posterior wall thickness, suggesting a transformation in concentric diffuse hypertrophic cardiomyopathy.

  9. Clinical predictors of genetic testing outcomes in hypertrophic cardiomyopathy.

    Science.gov (United States)

    Ingles, Jodie; Sarina, Tanya; Yeates, Laura; Hunt, Lauren; Macciocca, Ivan; McCormack, Louise; Winship, Ingrid; McGaughran, Julie; Atherton, John; Semsarian, Christopher

    2013-12-01

    Genetic testing for hypertrophic cardiomyopathy has been commercially available for almost a decade; however, low mutation detection rate and cost have hindered uptake. This study sought to identify clinical variables that can predict probands with hypertrophic cardiomyopathy in whom a pathogenic mutation will be identified. Probands attending specialized cardiac genetic clinics across Australia over a 10-year period (2002-2011), who met clinical diagnostic criteria for hypertrophic cardiomyopathy and who underwent genetic testing for hypertrophic cardiomyopathy were included. Clinical, family history, and genotype information were collected. A total of 265 unrelated individuals with hypertrophic cardiomyopathy were included, with 138 (52%) having at least one mutation identified. The mutation detection rate was significantly higher in the probands with hypertrophic cardiomyopathy with an established family history of disease (72 vs. 29%, P < 0.0001), and a positive family history of sudden cardiac death further increased the detection rate (89 vs. 59%, P < 0.0001). Multivariate analysis identified female gender, increased left-ventricular wall thickness, family history of hypertrophic cardiomyopathy, and family history of sudden cardiac death as being associated with greatest chance of identifying a gene mutation. Multiple mutation carriers (n = 16, 6%) were more likely to have suffered an out-of-hospital cardiac arrest or sudden cardiac death (31 vs. 7%, P = 0.012). Family history is a key clinical predictor of a positive genetic diagnosis and has direct clinical relevance, particularly in the pretest genetic counseling setting.

  10. Laenderyggens degeneration og radiologi

    DEFF Research Database (Denmark)

    Jacobsen, Steffen; Gosvig, Kasper Kjaerulf; Sonne-Holm, Stig

    2006-01-01

    Low back pain (LBP) is one of the most common conditions, and at the same time one of the most complex nosological entities. The lifetime prevalence is approximately 80%, and radiological features of lumbar degeneration are almost universal in adults. The individual risk factors for LBP and signi......Low back pain (LBP) is one of the most common conditions, and at the same time one of the most complex nosological entities. The lifetime prevalence is approximately 80%, and radiological features of lumbar degeneration are almost universal in adults. The individual risk factors for LBP...... and significant relationships between radiological findings and subjective symptoms have both been notoriously difficult to identify. The lack of consensus on clinical criteria and radiological definitions has hampered the undertaking of properly executed epidemiological studies. The natural history of LBP...

  11. Quantum degenerate systems

    Energy Technology Data Exchange (ETDEWEB)

    Micheli, Fiorenza de [Centro de Estudios Cientificos, Arturo Prat 514, Valdivia (Chile); Instituto de Fisica, Pontificia Universidad Catolica de Valparaiso, Casilla 4059, Valparaiso (Chile); Zanelli, Jorge [Centro de Estudios Cientificos, Arturo Prat 514, Valdivia (Chile); Universidad Andres Bello, Av. Republica 440, Santiago (Chile)

    2012-10-15

    A degenerate dynamical system is characterized by a symplectic structure whose rank is not constant throughout phase space. Its phase space is divided into causally disconnected, nonoverlapping regions in each of which the rank of the symplectic matrix is constant, and there are no classical orbits connecting two different regions. Here the question of whether this classical disconnectedness survives quantization is addressed. Our conclusion is that in irreducible degenerate systems-in which the degeneracy cannot be eliminated by redefining variables in the action-the disconnectedness is maintained in the quantum theory: there is no quantum tunnelling across degeneracy surfaces. This shows that the degeneracy surfaces are boundaries separating distinct physical systems, not only classically, but in the quantum realm as well. The relevance of this feature for gravitation and Chern-Simons theories in higher dimensions cannot be overstated.

  12. Cardiovascular magnetic resonance in hypertrophic cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Shiozaki, Afonso Akio; Parga, Jose Rodrigues; Arteaga, Edmundo; Rochitte, Carlos Eduardo [Sao Paulo Univ. (USP), SP (Brazil). Instituto do Coracao. Setor de Tomografia Computarizada e Ressonancia Magnetica Cardiovascular]. E-mail: rochitte@incor.usp.br; Kim, Raymond J. [Duke Cardiovascular Magnetic Resonance Center, Durham, NC (United States); Tassi, Eduardo Marinho [Diagnosticos da America S.A., Rio de Janeiro, RJ (Brazil). Sector of Cardiovascular Magnetic Resonance and Computed Tomography

    2007-03-15

    Hypertrophic cardiomyopathy (HCM) is the most frequent genetic cardiac disease that causes sudden death in young people, with an incidence of 1:500 adults. The routinely used criteria for worst prognosis have limited sensitivity and specificity. Thus, the estimated risk of evolving to dilated cardiomyopathy or sudden death is somewhat inaccurate, leading to management uncertainty of HCM patients. Therefore, an accurate noninvasive method for the diagnosis of HCM with prognostic value is of great importance. In the last years, Cardiovascular Magnetic Resonance (CMR) emerged not only as a diagnostic tool, but also as a study with prognostic values, by characterizing myocardial fibrosis with great accuracy in HCM patients. Additionally, CMR identifies the types of hypertrophy, analyses the ventricular function, estimates the intraventricular gradient and allows the determination of differential diagnosis. Moreover, CMR can uniquely access myocardial fibrosis in HCM. (author)

  13. Electrodiagnostic evaluation in feline hypertrophic muscular dystrophy.

    Science.gov (United States)

    Howard, J; Jaggy, A; Busato, A; Gaschen, F

    2004-07-01

    Standard needle electromyography (EMG) of 56 muscles and nerve conduction velocities (NCV) of the ulnar and common peroneal nerves were investigated in each of six cats affected with hypertrophic feline muscular dystrophy, 10 related heterozygote carriers and 10 normal cats. The EMG findings were considered normal in carrier and control cats, and consisted of 33% normal readings, 22% myotonic discharges, 18% fibrillation potentials, 11% prolonged insertional potentials, 10% complex repetitive discharges and 6% positive sharp waves in affected cats. Muscles of the proximal limbs were most frequently affected. No differences in NCV were found between the three cat groups. It was concluded that dystrophin-deficient dystrophic cats have widespread and frequent EMG changes, predominantly myotonic discharges and fibrillation potentials, which are most pronounced in the proximal appendicular muscles.

  14. MT-CYB mutations in hypertrophic cardiomyopathy

    DEFF Research Database (Denmark)

    Hagen, Christian M; Aidt, Frederik H; Havndrup, Ole

    2013-01-01

    Mitochondrial dysfunction is a characteristic of heart failure. Mutations in mitochondrial DNA, particularly in MT-CYB coding for cytochrome B in complex III (CIII), have been associated with isolated hypertrophic cardiomyopathy (HCM). We hypothesized that MT-CYB mutations might play an important...... and m.15482T>C; p.S246P were identified. Modeling showed that the p.C93Y mutation leads to disruption of the tertiary structure of Cytb by helix displacement, interfering with protein-heme interaction. The p.S246P mutation induces a diproline structure, which alters local secondary structure and induces...... of HCM patients. We propose that further patients with HCM should be examined for mutations in MT-CYB in order to clarify the role of these variants....

  15. Hypertrophic Obesity and Subcutaneous Adipose Tissue Dysfunction

    Directory of Open Access Journals (Sweden)

    Anna Meiliana

    2014-08-01

    Full Text Available BACKGROUND: Over the past 50 years, scientists have recognized that not all adipose tissue is alike, and that health risk is associated with the location as well as the amount of body fat. Different depots are sufficiently distinct with respect to fatty-acid storage and release as to probably play unique roles in human physiology. Whether fat redistribution causes metabolic disease or whether it is a marker of underlying processes that are primarily responsible is an open question. CONTENT: The limited expandability of the subcutaneous adipose tissue leads to inappropriate adipose cell expansion (hypertrophic obesity with local inflammation and a dysregulated and insulin-resistant adipose tissue. The inability to store excess fat in the subcutaneous adipose tissue is a likely key mechanism for promoting ectopic fat accumulation in tissues and areas where fat can be stored, including the intra-abdominal and visceral areas, in the liver, epi/pericardial area, around vessels, in the myocardium, and in the skeletal muscles. Many studies have implicated ectopic fat accumulation and the associated lipotoxicity as the major determinant of the metabolic complications of obesity driving systemic insulin resistance, inflammation, hepatic glucose production, and dyslipidemia. SUMMARY: In summary, hypertrophic obesity is due to an impaired ability to recruit and differentiate available adipose precursor cells in the subcutaneous adipose tissue. Thus, the subcutaneous adipose tissue may be particular in its limited ability in certain individuals to undergo adipogenesis during weight increase. Inability to promote subcutaneous adipogenesis under periods of affluence would favor lipid overlow and ectopic fat accumulation with negative metabolic consequences. KEYWORDS: obesity, adipogenesis, subcutaneous adipose tissue, visceral adipose tissue, adipocyte dysfunction.

  16. Current Therapeutic Approach to Hypertrophic Scars

    Directory of Open Access Journals (Sweden)

    Zrinka Bukvić Mokos

    2017-06-01

    Full Text Available Abnormal scarring and its accompanying esthetic, functional, and psychological sequelae still pose significant challe nges. To date, there is no satisfactory prevention or treatment option for hypertrophic scars (HSs, which is mostly due to not completely comprehending the mechanisms underlying their formation. That is why the apprehension of regular and controlled physiological processes of scar formation is of utmost importance when facing hypertrophic scarring, its pathophysiology, prevention, and therapeutic approach. When treating HSs and choosing the best treatment and prevention modality, physicians can choose from a plethora of therapeutic options and many commercially available products, among which currently there is no efficient option that can successfully overcome impaired skin healing. This article reviews current therapeutic approach and emerging therapeutic strategies for the management of HSs, which should be individualized, based on an evaluation of the scar itself, patients’ expectations, and practical, evidence-based guidelines. Clinicians are encouraged to combine various prevention and treatment modalities where combination therapy that includes steroid injections, 5-fluorouracil, and pulsed-dye laser seems to be the most effective. On the other hand, the current therapeutic options are usually empirical and their results are unreliable and unpredictable. Therefore, there is an unmet need for an effective, targeted therapy and prevention, which would be based on an action or a modulation of a particular factor with clarified mechanism of action that has a beneficial effect on wound healing. As the extracellular matrix has a crucial role in cellular and extracellular events that lead to pathological scarring, targeting its components mostly by regulating bone morphogenetic proteins may throw up new therapeutic approach for reduction or prevention of HSs with functionally and cosmetically acceptable outcome.

  17. Hypertrophic Synovitis of the Facet Joint Causing Root Pain

    Directory of Open Access Journals (Sweden)

    Koichi Iwatsuki M.D.

    2008-01-01

    Full Text Available Osteoarthritic changes in the facet joints are common in the presence of degenerative disc disease. Changes in the joint capsule accompany changes in the articular surfaces. Intraspinal synovial cysts that cause radicular pain, cauda equina syndrome, and myelopathy have been reported; however, there have been few reports in orthopedic or neurosurgical literature regarding hypertrophic synovitis of the facet joint presenting as an incidental para-articular mass. Here, we report a case of hypertrophic synovitis causing root pain. We describe the case of a 65-year-old man suffering from right sciatica and right leg pain in the L5 nerve-root dermatome for 1 year; magnetic resonance imaging (MRI revealed an enhanced mass around the L4–5 facet joint. We investigated this mass pathologically. After right medial facetectomy, the symptoms resolved. Pathological investigation revealed this mass was hypertrophic synovitis. Hypertrophic synovitis of the facet joint might cause root pain.

  18. Regional ischemia in hypertrophic Langendorff-perfused rat hearts

    NARCIS (Netherlands)

    J.F. Ashruf; C. Ince (Can); H.A. Bruining (Hajo)

    1999-01-01

    textabstractMyocardial hypertrophy decreases the muscle mass-to-vascularization ratio, thereby changing myocardial perfusion. The effect of these changes on myocardial oxygenation in hypertrophic Langendorff-perfused rat hearts was measured using epimyocardial NADH

  19. Hypertrophic Pulmonary Osteoarthropathy Associated with Primary Adenocarcinoma of the Lung

    OpenAIRE

    Ayabe, Hiroyoshi; Hisano, Hiroshi; Oka, Tadayuki; Tsuji, Hiroharu; Hara, Shinsuke; Tagawa, Yutaka; Kawahara, Katsunobu; Tomita, Masaotomita

    1991-01-01

    Hypertrophic pulmonary osteoarthropathy (HPOA) associated with primary lung cancer is reported in a fifty-four-year-old man. Symptoms of HPOA were makedly improved following lung resection for adenocarcinoma of the lung.

  20. Cyanobacteria-phytoplankton dynamics of a hypertrophic African lake

    CSIR Research Space (South Africa)

    Zohary, T

    1995-01-01

    Full Text Available Phytoplankton species composition and abundance were recorded weekly or biweekly for nearly 7 years in a hypertrophic lake ( Hartbeespoort Dam, South Africa), together with a range of physical and chemical parameters. A total of 73 species were...

  1. On the surgical treatment of hypertrophic scars: a comprehensive guideline for the surgical treatment of hypertrophic scars

    NARCIS (Netherlands)

    Stekelenburg, C.M.; van der Wal, M.B.A.; Middelkoop, E.; Niessen, F.B.; van Zuijlen, P.P.M.

    2012-01-01

    Background: Treating the hypertrophic scar remains a challenging problem. It often leaves the clinician with the questions whether, when and how to treat a hypertrophic scar. Methods: This review will focus on the surgical treatment of hypertrophic scars considering the latest research in this

  2. A novel instrument aimed at measuring hypertrophic scar formation

    Energy Technology Data Exchange (ETDEWEB)

    Ramella-Roman, J C; Nguyen, T A; Lemaillet, P [Department of Biomedical Engineering, Catholic University of America (United States); Pavlovich, A R; Jordan, M H; Shupp, J W, E-mail: ramella@cua.edu [Burn Center, Department of Surgery, Washington Hospital Center-MedStar Health Research Institute, Washington, DC (United States)

    2011-01-01

    Hypertrophic scaring is a condition that can occur after thermal trauma and can lead to loss of function and psyco-social complications. Assessment of the development of a hypertrophic scar currently relies on visual inspection and grading. In this paper we propose a system based on polarized illumination and collection capable of capturing images of scars and quantify their roughness. Models of rough surface roughness are ultimately used to quantify the level of roughness of scarred skin compared to normal skin.

  3. Hypertrophic cardiomyopathy with mid-ventricular obstruction and apical aneurysm

    Directory of Open Access Journals (Sweden)

    N.D. Oryshchyn

    2016-11-01

    Full Text Available A case report of apical left ventricular aneurysm in patient with hypertrophic cardiomyopathy with mid-ventricular obstruction (diagnosis and surgical treatment is presented. We revealed apical aneurysm and mid-ventricular obstruction during echocardiography and specified anatomical characteristics of aneurysm during computer tomography. There was no evidence of obstructive coronary artery disease during coronary angiography. Taking into consideration multiple cerebral infarcts, aneurysm resection and left ventricular plastics was performed. Electronic microscopy of myocardium confirmed the diagnosis of hypertrophic cardiomyopathy.

  4. Comparison among patients with hypertrophic cardiomyopathy, hypertrophic cardiomyopathy with hypertension and hypertensive heart disease by {sup 123}I-BMIPP myocardial scintigraphy

    Energy Technology Data Exchange (ETDEWEB)

    Yoneyama, Satoshi; Sugihara, Hiroki; Ito, Kazuki [Kyoto Prefectural Univ. of Medicine (Japan)] [and others

    1997-12-01

    The usefulness of {sup 123}I-BMIPP myocardial SPECT in discriminating hypertrophic cardiomyopathy (46 patients), hypertrophic cardiomyopathy with hypertension (23 patients), and hypertensive hypertrophic heart (20 patients) was studied. SPECT image was divided into 17 domains, and dimension of decreased accumulation was decided visually at each domain as four classes called defect score (DS). Summation of DS (TDS) of each group was used to compare frequency and dimension of decreased accumulation, and characteristic of each site. Frequency of decreased accumulation and TDS in hypertrophic cardiomyopathy were similar in dimension with those in hypertrophic cardiomyopathy with hypertension, and those data in hypertensive hypertrophic heart were lower than those in above-mentioned 2 groups. In the cases of hypertrophic cardiomyopathy and hypertrophic cardiomyopathy with hypertension, decreased accumulation site was similar and was anterior wall-septum junction, septum-posterior wall junction and apex of heart. In the case of hypertensive hypertrophic heart, decreased accumulation site was only the posterior wall. Frequency, dimension and site of decreased accumulation in hypertrophic cardiomyopathy were different from those in hypertensive hypertrophic heart, and BMIPP was thought to be useful in discriminating these diseases. (K.H.)

  5. [Hypertrophic cardiomyopathy with left ventricular dilatation].

    Science.gov (United States)

    Iwami, G; Miyazaki, Y; Matsuyama, K; Shida, M; Ooga, M; Furuta, Y; Ikeda, H; Toshima, H; Chiba, M; Koga, Y

    1988-06-01

    There is increasing interest in the notion that some patients with hypertrophic cardiomyopathy (HCM) progress to morphological and functional manifestations similar to those of dilated cardiomyopathy (DCM). From 165 consecutive patients with HCM, 20 patients with left ventricular dilatation (left ventricular end-diastolic diameter greater than or equal to 50 mm) were selected and designated as dilated HCM. The diagnosis of HCM was established in these patients either by detection of the classical form of HCM in family members, with 2-dimensional echocardiographic evidence of asymmetric septal hypertrophy (ASH; septal thickness greater than or equal to 15 mm and a ratio of septal to posterior wall thickness greater than or equal to 1.3); or by demonstrating myocardial fiber disarray in autopsy or biopsy samples. The clinical manifestations of these patients with dilated HCM were then compared with those of other forms of HCM without left ventricular dilatation; 1) 40 patients with hypertrophic obstructive cardiomyopathy (HOCM) who had resting intraventricular pressure gradients of 20 mmHg or more, 2) 80 patients with non-obstructive HCM, each of whom had ASH of the entire ventricular septum (typical ASH), and 3) 25 non-obstructive patients whose hypertrophy was localized to the apical region of the ventricular septum (apical ASH). Patients having apical hypertrophy with a spade-like configuration on the left ventriculogram were excluded from the study. Compared with HOCM and typical ASH groups, the patients with dilated HCM had family histories of significantly more frequent HCM and less frequent hypertension. The patients with dilated HCM also had significantly less fractional shortening (FS), decreased interventricular septal thickness, greater left ventricular end-diastolic pressure (LVEDP), and left ventricular dilatation. During the follow-up period (average: 3.5 years), seven patients (35%) with dilated HCM died; five from congestive heart failure (CHF), one

  6. Keloids and Hypertrophic Scars: Pathophysiology, Classification, and Treatment.

    Science.gov (United States)

    Berman, Brian; Maderal, Andrea; Raphael, Brian

    2017-01-01

    Keloid and hypertrophic scars represent an aberrant response to the wound healing process. These scars are characterized by dysregulated growth with excessive collagen formation, and can be cosmetically and functionally disruptive to patients. Objectives are to describe the pathophysiology of keloid and hypertrophic scar, and to compare differences with the normal wound healing process. The classification of keloids and hypertrophic scars are then discussed. Finally, various treatment options including prevention, conventional therapies, surgical therapies, and adjuvant therapies are described in detail. Literature review was performed identifying relevant publications pertaining to the pathophysiology, classification, and treatment of keloid and hypertrophic scars. Though the pathophysiology of keloid and hypertrophic scars is not completely known, various cytokines have been implicated, including interleukin (IL)-6, IL-8, and IL-10, as well as various growth factors including transforming growth factor-beta and platelet-derived growth factor. Numerous treatments have been studied for keloid and hypertrophic scars,which include conventional therapies such as occlusive dressings, compression therapy, and steroids; surgical therapies such as excision and cryosurgery; and adjuvant and emerging therapies including radiation therapy, interferon, 5-fluorouracil, imiquimod, tacrolimus, sirolimus, bleomycin, doxorubicin, transforming growth factor-beta, epidermal growth factor, verapamil, retinoic acid, tamoxifen, botulinum toxin A, onion extract, silicone-based camouflage, hydrogel scaffold, and skin tension offloading device. Keloid and hypertrophic scars remain a challenging condition, with potential cosmetic and functional consequences to patients. Several therapies exist which function through different mechanisms. Better understanding into the pathogenesis will allow for development of newer and more targeted therapies in the future.

  7. Intervertebral disc degeneration in dogs

    NARCIS (Netherlands)

    Bergknut, N.

    2011-01-01

    Back pain is common in both dogs and humans, and is often associated with intervertebral disc (IVD) degeneration. The IVDs are essential structures of the spine and degeneration can ultimately result in diseases such as IVD herniation or spinal instability. In order to design new treatments halting

  8. Intervertebral disc degeneration in dogs

    NARCIS (Netherlands)

    Bergknut, Niklas

    Back pain is common in both dogs and humans, and is often associated with intervertebral disc (IVD) degeneration. The IVDs are essential structures of the spine and degeneration can ultimately result in diseases such as IVD herniation or spinal instability. In order to design new treatments halting

  9. Hypertrophic osteoarthropathy associated with metastatic melanoma.

    Science.gov (United States)

    Thompson, Michael A; Warner, Noranna B; Hwu, Wen-Jen

    2005-12-01

    Hypertrophic osteoarthropathy (HOA) is one of the paraneoplastic syndromes most commonly associated with non-small-cell lung cancer. Although pulmonary metastasis is the second most common initial site of melanoma metastasis, HOA is rarely detected in patients with metastatic melanoma in the lung. We report a case of a 45-year-old woman with advanced melanoma who developed HOA after her disease had progressed through first-line systemic therapy. The patient's diagnosis of HOA was made on the basis of digital clubbing, arthralgia, pain, joint effusion and periosteal bone formation on X-ray with negative rheumatologic laboratory studies. Only six cases of HOA in metastatic melanoma have been reported previously. This diagnosis should be considered with lung metastases and the presentation of polyarthralgia with appropriate laboratory and imaging findings. Interestingly, the patient responded to bisphosphonates and second-line chemotherapy with carboplatin and paclitaxel, which is commonly used for lung cancer, not advanced melanoma. As with many paraneoplastic syndromes, successful treatment of the underlying disease was associated with a rapid resolution of the symptoms.

  10. Right ventricular involvement in feline hypertrophic cardiomyopathy.

    Science.gov (United States)

    Schober, K E; Savino, S I; Yildiz, V

    2016-12-01

    To evaluate right ventricular (RV) wall thickness and chamber dimensions in cats with hypertrophic cardiomyopathy (HCM). One hundred fifty-one healthy control cats and 200 cats with HCM. Retrospective, observational, clinical cohort study. Two-dimensional echocardiograms from all cats were analyzed. Right atrial diameter, RV free wall thickness, and RV chamber diameter were quantified using multiple imaging views. Conventional (mean ± 2 standard deviations) and allometrically scaled (Y = a × M b ) reference values were determined in normal cats and compared to values found in cats with HCM. Linear and logistic regression, multivariate regression, and mixed model analysis were performed to identify associations between RV wall thickness and severity of left ventricular (LV) hypertrophy, clinical severity of HCM, and presence of pleural effusion. Mean RV wall thickness was increased in HCM (p0.05) in control cats. Increased RV wall thickness is common in cats with HCM and relates to severity of LV hypertrophy and clinical status. Copyright © 2016 Elsevier B.V. All rights reserved.

  11. A One Health Approach to Hypertrophic Cardiomyopathy

    Science.gov (United States)

    Ueda, Yu; Stern, Joshua A.

    2017-01-01

    Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiac disease in humans and results in significant morbidity and mortality. Research over the past 25 years has contributed enormous insight into this inherited disease particularly in the areas of genetics, molecular mechanisms, and pathophysiology. Our understanding continues to be limited by the heterogeneity of clinical presentations with various genetic mutations associated with HCM. Transgenic mouse models have been utilized especially studying the genotypic and phenotypic interactions. However, mice possess intrinsic cardiac and hemodynamic differences compared to humans and have limitations preventing their direct translation. Other animal models of HCM have been studied or generated in part to overcome these limitations. HCM in cats shows strikingly similar molecular, histopathological, and genetic similarities to human HCM, and offers an important translational opportunity for the study of this disease. Recently, inherited left ventricular hypertrophy in rhesus macaques was identified and collaborative investigations have been conducted to begin to develop a non-human primate HCM model. These naturally-occurring large-animal models may aid in advancing our understanding of HCM and developing novel therapeutic approaches to this disease. This review will highlight the features of HCM in humans and the relevant available and developing animal models of this condition. PMID:28955182

  12. Omentin functions to attenuate cardiac hypertrophic response.

    Science.gov (United States)

    Matsuo, Kazuhiro; Shibata, Rei; Ohashi, Koji; Kambara, Takahiro; Uemura, Yusuke; Hiramatsu-Ito, Mizuho; Enomoto, Takashi; Yuasa, Daisuke; Joki, Yusuke; Ito, Masanori; Hayakawa, Satoko; Ogawa, Hayato; Kihara, Shinji; Murohara, Toyoaki; Ouchi, Noriyuki

    2015-02-01

    Cardiac hypertrophy occurs in many obesity-related conditions. Omentin is an adipose-derived plasma protein that is downregulated under obese conditions. Here, we investigated whether omentin modulates cardiac hypertrophic responses in vivo and in vitro. Systemic administration of an adenoviral vector expressing human omentin (Ad-OMT) to wild-type (WT) mice led to the attenuation of cardiac hypertrophy, fibrosis and ERK phosphorylation induced by transverse aortic constriction (TAC) or angiotensin II infusion. In cultured cardiomyocytes, stimulation with phenylephrine (PE) led to an increase in myocyte size, which was prevented by pretreatment with human omentin protein. Pretreatment of cardiomyocytes with omentin protein also reduced ERK phosphorylation in response to PE stimulation. Ad-OMT enhanced phosphorylation of AMP-activated protein kinase (AMPK) in the heart of WT mice after TAC operation. Blockade of AMPK activation by transduction with dominant-negative mutant forms of AMPK reversed the inhibitory effect of omentin on myocyte hypertrophy and ERK phosphorylation following PE stimulation. Moreover, fat-specific transgenic mice expressing human omentin showed reduced cardiac hypertrophy and ERK phosphorylation following TAC surgery compared to littermate controls. These data suggest that omentin functions to attenuate the pathological process of myocardial hypertrophy via the activation of AMPK in the heart, suggesting that omentin may represent a target molecule for the treatment of cardiac hypertrophy. Copyright © 2014 Elsevier Ltd. All rights reserved.

  13. Hypertrophic cardiomyopathy in adults: an overview.

    Science.gov (United States)

    Jacobs, Cynthia

    2014-09-01

    To present an overview of clinical issues related to adults with hypertrophic cardiomyopathy (HCM), their presenting symptoms, diagnosis, physical examination findings, treatment, and follow-up care. A comprehensive search of Medline (PubMed) and CINAHL was conducted using the key terms HCM, treatment, diagnosis, sudden cardiac death (SCD), and complications. This search yielded 21 articles used for this article. There were three reference books used for background, diagnosis, and treatment information as well. Although HCM is the most prevalent genetic disorder affecting the heart, it often goes undiagnosed until midlife after patients show symptoms of myocardial remodeling. Adults with cardiomyopathy suffer SCD or adverse events such as stroke and heart failure from HCM. Early diagnosis will prevent SCD, improve quality of life, and slow patient's progression to heart failure. Early recognition of HCM in adults by their primary care providers will improve patients' quality of life and reduce incidence of SCD, heart failure, and stroke. ©2014 American Association of Nurse Practitioners.

  14. Genetics Home Reference: Stargardt macular degeneration

    Science.gov (United States)

    ... Twitter Home Health Conditions Stargardt macular degeneration Stargardt macular degeneration Printable PDF Open All Close All Enable Javascript to view the expand/collapse boxes. Description Stargardt macular degeneration is a genetic eye disorder that causes progressive ...

  15. Prevalence of hypertrophic cardiomyopathy in highly trained athletes: relevance to pre-participation screening

    National Research Council Canada - National Science Library

    Basavarajaiah, Sandeep; Wilson, Matthew; Whyte, Gregory; Shah, Ajay; McKenna, William; Sharma, Sanjay

    2008-01-01

    This study sought to determine the prevalence of hypertrophic cardiomyopathy (HCM) in elite athletes. Hypertrophic cardiomyopathy is considered to be the most common cause of exercise-related sudden death in young athletes...

  16. HYPERTROPHIC OBSTRUCTIVE CARDIOMYOPATHY AS A SIDE-EFFECT OF DEXAMETHASONE TREATMENT FOR BRONCHOPULMONARY DYSPLASIA

    NARCIS (Netherlands)

    BRAND, PLP; VANLINGEN, RA; BRUS, F; TALSMA, MD; ELZENGA, NJ

    1993-01-01

    We report three infants who developed hypertrophic obstructive cardiomyopathy during dexamethasone treatment for bronchopulmonary dysplasia. In all three infants, echocardiography had ruled out cardiac abnormalities prior to the dexamethasone course. The hypertrophic obstructive cardiomyopathy

  17. Pharmacological and non-pharmacological treatment of obstructive hypertrophic cardiomyopathy.

    Science.gov (United States)

    Hidalgo, Luis F; Naidu, Srihari S; Aronow, Wilbert S

    2018-01-01

    Hypertrophic obstructive cardiomyopathy has been rising in prevalence, due to increased awareness and advanced imaging. For the symptomatic patient, pharmacological management remains an effective approach to the majority of patients with obstructive hypertrophic cardiomyopathy. However, a significant subset fails to improve sufficiently with medical therapy initially, or progressively becomes more symptomatic despite augmented medications over time. Most of the advances in the treatment of obstructive hypertrophic cardiomyopathy have therefore been made in the area of non-pharmacologic management, particularly septal reduction therapy. Both surgical myectomy and alcohol septal ablation have undergone iterative modifications that improve outcomes. Current guidelines support these therapies based on large observational studies, with choice of therapy based on a variety of factors but again based primarily on expert consensus opinion. Areas covered: This article reviews both pharmacological and non-pharmacological interventions to improve outflow tract obstruction and symptoms, and provides an algorithm for addressing the symptomatic obstructive patient. Expert commentary: Current options for hypertrophic cardiomyopathy allow the majority of patients to live their lives with no more than NYHA Class 2 heart failure symptoms. Treatment algorithms can add in identification of patients who may benefit from advanced therapies, and should be instituted routinely to improve care for the majority of patients with symptomatic hypertrophic cardiomyopathy.

  18. Investigating the role of P311 in the hypertrophic scar.

    Directory of Open Access Journals (Sweden)

    Jianglin Tan

    Full Text Available The mechanisms of hypertrophic scar formation are not fully understood. We previously screened the differentially expressed genes of human hypertrophic scar tissue and identified P311 gene as upregulated. As the activities of P311 in human fibroblast function are unknown, we examined the distribution of it and the effects of forced expression or silencing of expression of P311. P311 expression was detected in fibroblast-like cells from the hypertrophic scar of burn injury patients but not in peripheral blood mononuclear cells, bone marrow mesenchymal stem cells, epidermal cells or normal skin dermal cells. Transfection of fibroblasts with P311 gene stimulated the expression of alpha-smooth muscle actin (alpha-SMA, TGF-beta1 and alpha1(I collagen (COL1A1, and enhanced the contraction of fibroblast populated collagen lattices (FPCL. In contrast, interference of fibroblast P311 gene expression decreased the TGF-beta1 mRNA expression and reduced the contraction of fibroblasts in FPCL. These results suggest that P311 may be involved in the pathogenesis of hypertrophic scar via induction of a myofibroblastic phenotype and of functions such as TGF-beta1 expression. P311 could be a novel target for the control of hypertrophic scar development.

  19. Feeding ecology and energetic relationships with habitat of blue catfish, Ictalurus furcatus, and flathead catfish, Pylodictis olivaris, in the lower Mississippi River, U.S.A.

    Science.gov (United States)

    Eggleton, M.A.; Schramm, H.L.

    2004-01-01

    We examined feeding of blue catfish, Ictalurus furcatus, and flathead catfish, Pylodictis olivaris, collected from floodplain lake, secondary (side) river channel, and main river channel habitats in the lower Mississippi River (LMR), U.S.A. We described the feeding ecology of two large river catfish species within the context of whether off-channel habitats in the LMR (i.e., floodplain lakes and secondary channels) potentially provided energetic benefits to these fishes as purported in contemporary theory on the ecology of large rivers. We used diet composition and associated caloric densities of prey consumed as indicators of energetic benefit to catfishes. Differences in diet among habitats were strong for blue catfish, but weak for flathead catfish; consumed foods generally differed among habitats in caloric (energy) content. Caloric densities of consumed foods were generally greatest in floodplain lakes, least in the main river channel, and intermediate in secondary river channels. Strong between-year variation in diet was observed, but only for blue catfish. Blue catfish fed disproportionately on lower-energy zebra mussels in the main river channel during 1997, and higher-energy chironomids and oligochaetes in floodplain lakes during 1998. Results suggested that although off-channel habitats potentially provided greater energetic return to catfishes in terms of foods consumed, patterns of feeding and subsequent energy intake may vary annually. Energetic benefits associated with off-channel habitats as purported under contemporary theory (e.g., the 'flood-pulse concept') may not be accrued by catfishes every year in the LMR.

  20. Process of Hypertrophic Scar Formation: Expression of Eukaryotic Initiation Factor 6

    OpenAIRE

    Qing-Qing Yang; Si-Si Yang; Jiang-Lin Tan; Gao-Xing Luo; Wei-Feng He; Jun Wu

    2015-01-01

    Background: Hypertrophic scar is one of the most common complications and often causes the disfigurement or deformity in burn or trauma patients. Therapeutic methods on hypertrophic scar treatment have limitations due to the poor understanding of mechanisms of hypertrophic scar formation. To throw light on the molecular mechanism of hypertrophic scar formation will definitely improve the outcome of the treatment. This study aimed to illustrate the negative role of eukaryotic initiation factor...

  1. Suppressed inflammatory gene expression during human hypertrophic scar compared to normotrophic scar formation

    NARCIS (Netherlands)

    van den Broek, L.J.; van der Veer, W.M.; de Jong, E.H.; Gibbs, S.; Niessen, F.B.

    2015-01-01

    Hypertrophic scar formation is a result of adverse cutaneous wound healing. The pathogenesis of hypertrophic scar formation is still poorly understood. A problem next to the lack of suitable animal models is that often normal skin is compared to hypertrophic scar (HTscar) and not to normotrophic

  2. An unusual ST-segment elevation: apical hypertrophic cardiomyopathy shows the ace up its sleeve.

    Science.gov (United States)

    de Santis, Francesco; Pergolini, Amedeo; Zampi, Giordano; Pero, Gaetano; Pino, Paolo Giuseppe; Minardi, Giovanni

    2013-01-01

    Apical hypertrophic cardiomyopathy is part of the broad clinical and morphologic spectrum of hypertrophic cardiomyopathy. We report a patient with electrocardiographic abnormalities in whom acute coronary syndrome was excluded and apical hypertrophic cardiomyopathy was demonstrated by careful differential diagnosis. Copyright © 2012 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.

  3. Hypertrophic scarring: the greatest unmet challenge following burn injury

    Science.gov (United States)

    Finnerty, Celeste C; Jeschke, Marc G; Branski, Ludwik K; Barret, Juan P.; Dziewulski, Peter; Herndon, David N

    2017-01-01

    Summary Improvements in acute burn care have enabled patients to survive massive burns which would have once been fatal. Now up to 70% of patients develop hypertrophic scars following burns. The functional and psychosocial sequelae remain a major rehabilitative challenge, decreasing quality of life and delaying reintegration into society. The current approach is to optimise the healing potential of the burn wound using targeted wound care and surgery in order to minimise the development of hypertrophic scarring. This approach often fails, and modulation of established scar is continued although the optimal indication, timing, and combination of therapies have yet to be established. The need for novel treatments is paramount, and future efforts to improve outcomes and quality of life should include optimisation of wound healing to attenuate or prevent hypertrophic scarring, well-designed trials to confirm treatment efficacy, and further elucidation of molecular mechanisms to allow development of new preventative and therapeutic strategies. PMID:27707499

  4. Hypertrophic cardiomyopathy: Part 1 - Introduction, pathology and pathophysiology

    Directory of Open Access Journals (Sweden)

    Praveen Kerala Varma

    2014-01-01

    Full Text Available Hypertrophic cardiomyopathy (HCM is the most common genetic cardiovascular disease with many genotype and phenotype variations. Earlier terminologies, hypertrophic obstructive cardiomyopathy and idiopathic hypertrophic sub-aortic stenosis are no longer used to describe this entity. Patients present with or without left ventricular outflow tract (LVOT obstruction. Resting or provocative LVOT obstruction occurs in 70% of patients and is the most common cause of heart failure. The pathology and pathophysiology of HCM includes hypertrophy of the left ventricle with or without right ventricular hypertrophy, systolic anterior motion of mitral valve, dynamic and mechanical LVOT obstruction, mitral regurgitation, diastolic dysfunction, myocardial ischemia, and fibrosis. Thorough understanding of pathology and pathophysiology is important for anesthetic and surgical management.

  5. Scar massage for hypertrophic burns scarring-A systematic review.

    Science.gov (United States)

    Ault, P; Plaza, A; Paratz, J

    2017-06-29

    Scar massage is used in burn units globally to improve functional and cosmetic outcomes of hypertrophic scarring following a burn, however, the evidence to support this therapy is unknown. To review the literature and assess the efficacy of scar massage in hypertrophic burn scars. MEDLINE, PubMed, Embase, CINAHL and the Cochrane Library were searched using the key words "burn", "burn injury", "thermal injury" and "scar", "hypertrophic scar" and "massage", "manipulation", "soft tissue mobilisation", "soft tissue manipulation". The articles were scored by the assessors using the Physiotherapy Evidence Database (PEDro) scale and outcome measures on range of motion (ROM), cosmesis (vascularity, pliability, height), pain scores, pruritus, and psychological measures of depression and anxiety were extracted. Eight publications were included in the review with 258 human participants and 15 animal subjects who received scar massage following a thermal injury resulting in hypertrophic scarring. Outcome measures that demonstrated that scar massage was effective included scar thickness as measured with ultrasonography (p=0.001; g=-0.512); depression (Centre for Epidemiologic Studies - Depression [CES-D]) (p=0.031; g=-0.555); pain as measured with Visual Analogue Scale (VAS) (p=0.000; g=-1.133) and scar characteristics including vascularity (p=0.000; g=-1.837), pliability (p=0.000; g=-1.270) and scar height (p=0.000; g=-2.054). Outcome measures that trended towards significance included a decrease in pruritus (p=0.095; g=-1.157). It appears that there is preliminary evidence to suggest that scar massage may be effective to decrease scar height, vascularity, pliability, pain, pruritus and depression in hypertrophic burns scaring. This review reflects the poor quality of evidence and lack of consistent and valid scar assessment tools. Controlled, clinical trials are needed to develop evidence-based guidelines for scar massage in hypertrophic burns scarring. Copyright © 2017

  6. Process of Hypertrophic Scar Formation: Expression of Eukaryotic Initiation Factor 6

    Science.gov (United States)

    Yang, Qing-Qing; Yang, Si-Si; Tan, Jiang-Lin; Luo, Gao-Xing; He, Wei-Feng; Wu, Jun

    2015-01-01

    Background: Hypertrophic scar is one of the most common complications and often causes the disfigurement or deformity in burn or trauma patients. Therapeutic methods on hypertrophic scar treatment have limitations due to the poor understanding of mechanisms of hypertrophic scar formation. To throw light on the molecular mechanism of hypertrophic scar formation will definitely improve the outcome of the treatment. This study aimed to illustrate the negative role of eukaryotic initiation factor 6 (eIF6) in the process of human hypertrophic scar formation, and provide a possible indicator of hypertrophic scar treatment and a potential target molecule for hypertrophic scar. Methods: In the present study, we investigated the protein expression of eIF6 in the human hypertrophic scar of different periods by immunohistochemistry and Western blot analysis. Results: In the hypertrophic scar tissue, eIF6 expression was significantly decreased and absent in the basal layer of epidermis in the early period, and increased slowly and began to appear in the basal layer of epidermis by the scar formation time. Conclusions: This study confirmed that eIF6 expression was significantly related to the development of hypertrophic scar, and the eIF6 may be a target molecule for hypertrophic scar control or could be an indicator of the outcomes for other treatment modalities. PMID:26481747

  7. The Corticofugal Effects of Auditory Cortex Microstimulation on Auditory Nerve and Superior Olivary Complex Responses Are Mediated via Alpha-9 Nicotinic Receptor Subunit.

    Directory of Open Access Journals (Sweden)

    Cristian Aedo

    Full Text Available The auditory efferent system is a complex network of descending pathways, which mainly originate in the primary auditory cortex and are directed to several auditory subcortical nuclei. These descending pathways are connected to olivocochlear neurons, which in turn make synapses with auditory nerve neurons and outer hair cells (OHC of the cochlea. The olivocochlear function can be studied using contralateral acoustic stimulation, which suppresses auditory nerve and cochlear responses. In the present work, we tested the proposal that the corticofugal effects that modulate the strength of the olivocochlear reflex on auditory nerve responses are produced through cholinergic synapses between medial olivocochlear (MOC neurons and OHCs via alpha-9/10 nicotinic receptors.We used wild type (WT and alpha-9 nicotinic receptor knock-out (KO mice, which lack cholinergic transmission between MOC neurons and OHC, to record auditory cortex evoked potentials and to evaluate the consequences of auditory cortex electrical microstimulation in the effects produced by contralateral acoustic stimulation on auditory brainstem responses (ABR.Auditory cortex evoked potentials at 15 kHz were similar in WT and KO mice. We found that auditory cortex microstimulation produces an enhancement of contralateral noise suppression of ABR waves I and III in WT mice but not in KO mice. On the other hand, corticofugal modulations of wave V amplitudes were significant in both genotypes.These findings show that the corticofugal modulation of contralateral acoustic suppressions of auditory nerve (ABR wave I and superior olivary complex (ABR wave III responses are mediated through MOC synapses.

  8. Diel vertical migration of zooplankton in a hypertrophic shallow ...

    African Journals Online (AJOL)

    Zooplankton as important links in the food web of aquatic ecosystems have been studied extensively. In current literature their diel vertical migration (DVM) is a highly discussed issue. In this investigation DVM by zooplankton is studied in a hypertrophic shallow lake in Germany. The objectives of the study were to see if ...

  9. diel vertical migration of zooplankton in a hypertrophic shallow

    African Journals Online (AJOL)

    Preferred Customer

    ABSTRACT: Zooplankton as important links in the food web of aquatic ecosystems have been studied extensively. In current literature their diel vertical migration (DVM) is a highly discussed issue. In this investigation DVM by zooplankton is studied in a hypertrophic shallow lake in Germany. The objectives of the study were ...

  10. Prognosis in hypertrophic cardiomyopathy observed in a large clinic population

    NARCIS (Netherlands)

    M.J.M. Kofflard (Marcel); D.J. Waldstein; J. Vos (Jeroen); F.J. ten Cate (Folkert)

    1993-01-01

    textabstractOverall annual cardiac mortality in hypertrophic cardiomyopathy (HC) has been reported to be between 2 and 4%, although these numbers are primarily from retrospective studies of patients referred to large research institutions. A clinic population of 113 patients with HC was

  11. Hypertrophic cardiomyopathy in South African Blacks | Lewis | South ...

    African Journals Online (AJOL)

    Hypertrophic cardiomyopathy (HCM) has been considered rare among the Black population of southern Africa. We report 7 patients with the disease who presented during a 14-month period. Current concepts in the approach to the diagnosis and treatment of HCM are discussed. It is possible that with greater awareness of ...

  12. Potential cellular and molecular causes of hypertrophic scar formation

    NARCIS (Netherlands)

    Veer, van der W.M.; Bloemen, M.C.; Ulrich, M.; Molema, G.; Zuijlen, van P.P.; Middelkoop, E.; Niessen, F.B.

    2009-01-01

    A scar is an expected result of wound healing. However, in some individuals, and particularly in burn victims, the wound healing processes may lead to a fibrotic hypertrophic scar, which is raised, red, inflexible and responsible for serious functional and cosmetic problems. It seems that a wide

  13. Potential cellular and molecular causes of hypertrophic scar formation

    NARCIS (Netherlands)

    van der Veer, Willem M.; Bloemen, Monica C. T.; Ulrich, Magda M. W.; Molema, Grietje; van Zuijlen, Paul P.; Middelkoop, Esther; Niessen, Frank B.

    A scar is an expected result of wound healing. However, in some individuals, and particularly in burn victims, the wound healing processes may lead to a fibrotic hypertrophic scar, which is raised, red, inflexible and responsible serious functional and cosmetic problems. It seems that a wide array

  14. A case of idiopathc hypertrophic pachtymeningitis | Minne | SA ...

    African Journals Online (AJOL)

    SA Journal of Radiology. Journal Home · ABOUT · Advanced Search · Current Issue · Archives · Journal Home > Vol 8, No 4 (2004) >. Log in or Register to get access to full text downloads. Username, Password, Remember me, or Register. A case of idiopathc hypertrophic pachtymeningitis. C Minne, N Khan. Abstract.

  15. Infantile hypertrophic pyloric stenosis: A case report | Okafor ...

    African Journals Online (AJOL)

    Background: The frequency of infantile hypertrophic stenosis is not known in Nigeria. The first case coming to our attention is described. Method: Descriptive case report. Results and conclusion: The infant was otherwise well with low birth weight.. A suggestive abdominal ultrasound was confirmed by barium contrast study ...

  16. Bilious Vomiting in Infantile Hypertrophic Pyloric Stenosis | Tan ...

    African Journals Online (AJOL)

    Infantile Hypertrophic Pyloric Stenosis (IHPS) is the most common cause of intestinal obstruction requiring surgery in the newborn. The exact aetiology is unknown. IHPS classically presents with non-bilious vomiting. Bilious vomiting is a rare presentation of IHPS that could lead to confusion in diagnosis. In this report we ...

  17. Gastric emptying in adults treated for infantile hypertrophic pyloric stenosis

    DEFF Research Database (Denmark)

    Rasmussen, L; Oster-Jörgensen, E; Hansen, L P

    1989-01-01

    The gastric emptying rate was scintigraphically determined in 6 women and 26 men who had undergone medical or surgical treatment for infantile hypertrophic pyloric stenosis a median of 29 years previously. Dyspeptic complaints were reported by four of the seven medically treated and nine of the 25...

  18. Orchidectomy Ameliorates the Vascular Hypertrophic Effect of A ...

    African Journals Online (AJOL)

    Dr Olaleye Samuel

    Orchidectomy Ameliorates the Vascular Hypertrophic Effect of A. High Salt Diet in Sprague-Dawley Rats. Oloyo, AK1, Vineetha VP 2 , Anigbogu CN1 and Sofola, OA1. 1Department of Physiology, College of Medicine, University of Lagos. 2Laboratory for Experimental Pathology, Biomedical Technology (BMT) Wing, Sree ...

  19. Hypertrophic Cardiomyopathy in a Middle Aged Man - A Case Report

    African Journals Online (AJOL)

    Recognition of the condition in our environment is often difficult because of dearth of facilities and expertise for echocardiograghy in most of our hospitals. Objective: To draw attention to the existence of hypertrophic cardiomyopathy in our environment and need to use echocardiography in evaluating patients with cardiac ...

  20. Penetrance of Hypertrophic Cardiomyopathy in Children and Adolescents

    DEFF Research Database (Denmark)

    Jensen, Morten K; Havndrup, Ole; Christiansen, Michael

    2013-01-01

    The penetrance of hypertrophic cardiomyopathy (HCM) during childhood and adolescence has been only sparsely described. We studied the penetrance of HCM and the short- and long-term outcomes of clinical screening and predictive genetic testing of child relatives of patients with HCM....

  1. Private mitochondrial DNA variants in danish patients with hypertrophic cardiomyopathy

    DEFF Research Database (Denmark)

    Hagen, Christian M; Aidt, Frederik H; Havndrup, Ole

    2015-01-01

    Hypertrophic cardiomyopathy (HCM) is a genetic cardiac disease primarily caused by mutations in genes coding for sarcomeric proteins. A molecular-genetic etiology can be established in ~60% of cases. Evolutionarily conserved mitochondrial DNA (mtDNA) haplogroups are susceptibility factors for HCM...

  2. Founder mutations in hypertrophic cardiomyopathy patients in the Netherlands

    NARCIS (Netherlands)

    Christiaans, I.; Nannenberg, E. A.; Dooijes, D.; Jongbloed, R. J. E.; Michels, M.; Postema, P. G.; Majoor-Krakauer, D.; van den Wijngaard, A.; Mannens, M. M. A. M.; van Tintelen, J. P.; van Langen, I. M.; Wilde, A. A. M.

    In this part of a series on cardiogenetic founder mutations in the Netherlands, we review the Dutch founder mutations in hypertrophic cardiomyopathy (HCM) patients. HCM is a common autosomal dominant genetic disease affecting at least one in 500 persons in the general population. Worldwide, most

  3. Infantile Hypertrophic pyloric stenosis: A retrospective study from a ...

    African Journals Online (AJOL)

    Back ground: Infantile hypertrophic pyloric stenosis(IHPS) is a common infantile disorder characterized by enlarged pyloric musculature and gastric outlet obstruction(1). IHPS typically presents with progressive projectile non-bilious vomiting this usually commences between second and eighth week of age. To date there is ...

  4. Symmetric Dimethylarginine in Cats with Hypertrophic Cardiomyopathy and Diabetes Mellitus

    DEFF Research Database (Denmark)

    Langhorn, R.; Kieler, I. N.; Koch, J.

    2018-01-01

    Background: Symmetric dimethylarginine (SDMA) has been increasingly used as a marker of early chronic kidney disease (CKD) in cats, but little is known about the influence of comorbidities on SDMA in this species. Hypothesis: Hypertrophic cardiomyopathy (HCM) and diabetes mellitus (DM), independe...

  5. Peripheral vascular structure and function in hypertrophic cardiomyopathy

    NARCIS (Netherlands)

    Rowley, N.J.; Green, D.J.; George, K.; Thijssen, D.H.J.; Oxborough, D.; Sharma, S.; Somauroo, J.D.; Jones, J.; Sheikh, N.; Whyte, G.

    2012-01-01

    BACKGROUND: Hypertrophic cardiomyopathy (HCM) is characterised by idiopathic cardiac enlargement and represents the most frequent cause of sudden cardiac death in athletes under the age of 35 years. Differentiation between physiological (ie, exercise-related) and pathological (ie, HCM-related)

  6. Combination of MEK-ERK inhibitor and hyaluronic acid has a synergistic effect on anti-hypertrophic and pro-chondrogenic activities in osteoarthritis treatment.

    Science.gov (United States)

    Prasadam, Indira; Mao, Xinzhan; Shi, Wei; Crawford, Ross; Xiao, Yin

    2013-03-01

    We hypothesised that a potentially disease-modifying osteoarthritis (OA) drug such as hyaluronic acid (HA) given in combination with anti-inflammatory signalling agents such as mitogen-activated protein kinase kinase-extracellular signal-regulated kinase (MEK-ERK) signalling inhibitor (U0126) could result in additive or synergistic effects on preventing the degeneration of articular cartilage. Chondrocyte differentiation and hypertrophy were evaluated using human OA primary cells treated with either HA or U0126, or the combination of HA + U0126. Cartilage degeneration in menisectomy (MSX) induced rat OA model was investigated by intra-articular delivery of either HA or U0126, or the combination of HA + U0126. Histology, immunostaining, RT-qPCR, Western blotting and zymography were performed to assess the expression of cartilage matrix proteins and hypertrophic markers. Phosphorylated ERK (pERK)1/2-positive chondrocytes were significantly higher in OA samples compared with those in healthy control suggesting the pathological role of that pathway in OA. It was noted that HA + U0126 significantly reduced the levels of pERK, chondrocyte hypertrophic markers (COL10 and RUNX2) and degenerative markers (ADAMTs5 and MMP-13), however, increased the levels of chondrogenic markers (COL2) compared to untreated or the application of HA or U0126 alone. In agreement with the results in vitro, intra-articular delivery of HA + U0126 showed significant therapeutic improvement of cartilage in rat MSX OA model compared with untreated or the application of HA or U0126 alone. Our study suggests that the combination of HA and MEK-ERK inhibition has a synergistic effect on preventing cartilage degeneration.

  7. Sporadic fatal insomnia with spongiform degeneration in the thalamus and widespread PrPSc deposits in the brain.

    Science.gov (United States)

    Piao, Yue-Shan; Kakita, Akiyoshi; Watanabe, Hiroyuki; Kitamoto, Tetsuyuki; Takahashi, Hitoshi

    2005-06-01

    We report a case of human prion disease of 29 months duration in a 74-year-old Japanese man. The disease started with progressive sleeplessness and dementia. MRI showed gradually progressive cerebral atrophy. Neuronal loss, spongiform change and gliosis were evident in the thalamus and cerebral cortex, as well as in the striatum and amygdaloid nucleus. In the cerebellar cortex, mild-to-moderate depletion of Pukinje cells and spongiform change were observed. Mild neuronal loss in the inferior olivary nucleus was also seen. Immunohistochemistry revealed widespread perivacuolar deposits of abnormal prion protein (PrPsc) in the cerebral cortex, thalamus, basal ganglia, and brainstem, and minimal plaque-like deposits of PrPSc in the cerebellar cortex. In the cerebellar plaque-like deposits, the presence of amyloid fibrils was confirmed ultrastructurally. The entire pathology appeared to lie halfway between those of CJD and fatal insomnia, and further demonstrated the relationship between spongiform degeneration and PrPSc deposits, especially in the diseased thalamus. By immunoblotting, the thalamus was shown to contain the lowest amount of PrPSc among the brain regions examined. The PrPSc of type 2, in which the ratio of the three glycoforms was compatible with that of sporadic fatal insomnia (MM2-thalamic variant) reported previously, was also demonstrated. Analysis of the prion protein gene (PRNP) showed no mutation, and homozygosity for methionine at codon 129. In conclusion, we considered that this patient had been suffering from sporadic, pathologically atypical fatal insomnia.

  8. Age-Related Macular Degeneration.

    Science.gov (United States)

    Mehta, Sonia

    2015-09-01

    Age-related macular degeneration (AMD) is the leading cause of vision loss in the elderly. AMD is diagnosed based on characteristic retinal findings in individuals older than 50. Early detection and treatment are critical in increasing the likelihood of retaining good and functional vision. Copyright © 2015 Elsevier Inc. All rights reserved.

  9. Macular Degeneration Prevention and Risk Factors

    Science.gov (United States)

    ... Support macular degeneration research to help find a cure. Give Now About Research Programs Leadership Partners Accountability Careers Research Research We Fund Alzheimer’s Disease Research Program Macular Degeneration Research Program National ...

  10. Degenerated differential pair with controllable transconductance

    NARCIS (Netherlands)

    Mensink, Clemens; Mensink, Clemens H.J.; Nauta, Bram

    1998-01-01

    A differential pair with input transistors and provided with a variable degeneration resistor. The degeneration resistor comprises a series arrangement of two branches of coupled resistors which are shunted in mutually corresponding points by respective control transistors whose gates are

  11. Myocardial ischemia in hypertrophic cardiomyopathy; Isquemia miocardica na cardiomiopatia hipertrofica

    Energy Technology Data Exchange (ETDEWEB)

    Lima Filho, Moyses de Oliveira; Figueiredo, Geraldo L.; Simoes, Marcus V.; Pyntia, Antonio O.; Marin Neto, Jose Antonio [Sao Paulo Univ., Ribeirao Preto, SP (Brazil). Faculdade de Medicina. Div. de Cardiologia

    2000-08-01

    Myocardial ischemia in hypertrophic cardiomyopathy is multifactorial and explains the occurrence of angina, in about 50% of patients. The pathophysiology of myocardial ischemia may be explained by the increase of the ventricular mass and relative paucity of the coronary microcirculation; the elevated ventricular filling pressures and myocardial stiffness causing a compression of the coronary microvessels; the impaired coronary vasodilator flow reserve caused by anatomic and functional abnormalities; and the systolic compression of epicardial vessel (myocardial bridges). Myocardial ischemia must be investigated by perfusion scintigraphic methods since its presence influences the prognosis and has relevant clinical implications for management of patients. Patients with hypertrophic cardiomyopathy and documented myocardial ischemia usually need to undergo invasive coronary angiography to exclude the presence of concomitant atherosclerotic coronary disease. (author)

  12. Atrial myxoma in a patient with hypertrophic cardiomyopathy.

    Science.gov (United States)

    Abdou, Mahmoud; Hayek, Salim; Williams, Byron R

    2013-01-01

    Atrial myxoma is the most common primary cardiac tumor. Patients with atrial myxoma typically present with obstructive, embolic, or systemic symptoms; asymptomatic presentation is very rare. To our knowledge, isolated association of atrial myxoma with hypertrophic cardiomyopathy has been reported only once in the English-language medical literature. We report the case of an asymptomatic 71-year-old woman with known hypertrophic cardiomyopathy in whom a left atrial mass was incidentally identified on cardiac magnetic resonance images. After surgical excision of the mass and partial excision of the left atrial septum, histopathologic analysis confirmed the diagnosis of atrial myxoma. The patient was placed on preventive implantable cardioverter-defibrillator therapy and remained asymptomatic. The management of asymptomatic cardiac myxoma is a topic of debate, because no reports definitively favor either conservative or surgical measures.

  13. Prosthodontist contribution in treating post-burn hypertrophic facial scars

    Directory of Open Access Journals (Sweden)

    Padmanabhan T

    2010-01-01

    Full Text Available The formation of hypertrophic scars is common following healing of the burn wound, particularly in children. The face is one of the areas of the body most frequently affected by burns. Scar formation as a result of burn wounds leads to contraction of the formed granulation tissue, which causes both aesthetic and functional impairment for the patient. Scarring has major psychological and physical repercussions. Scarring on the face and visible regions of the body can be very distressing for the patient. Prevention of scars involves early and continuous use of a compressive orthesis. However, their efficacy is often limited to the facial region because of the contours of this area of body. This paper describes a clinical case of post-burn hypertrophic scars treated with silicone gel sheeting applied with pressure under custom made auto-polymerizing resin stent.

  14. Clinicopathological profiles of progressive heart failure in hypertrophic cardiomyopathy

    OpenAIRE

    Melacini, Paola; Basso, Cristina; Angelini, Annalisa; Calore, Chiara; Bobbo, Fabiana; Tokajuk, Barbara; Bellini, Nicoletta; Smaniotto, Gessica; Zucchetto, Mauro; Iliceto, Sabino; Thiene, Gaetano; Maron, Barry J.

    2010-01-01

    Aims Hypertrophic cardiomyopathy (HCM) is an important cause of heart failure-related disability over a wide range of ages. Profiles of severe progressive heart failure symptoms and death, or heart transplantation deserve more complete definition within large patient cohorts. Methods and results Clinical and morphological features of heart failure were assessed in 293 consecutive HCM patients over a median follow-up of 6 (inter-quartile range 2?11) years. Gross and histopathological features ...

  15. Hypertrophic neuropathy in Noonan syndrome with multiple lentigines.

    Science.gov (United States)

    Maridet, Claire; Sole, Guilhem; Morice-Picard, Fanny; Taieb, Alain

    2016-06-01

    RASopathies comprise several genetic syndromes with mainly cardio-facial-cutaneous manifestations. We report a patient with Noonan syndrome with multiple lentigines (NSML) due to a PTPN11 (p.Thr468Met) mutation associated with hypertrophic neuropathy of lumbar plexus in an adult woman, initially referred for neuropathic pain. Differential diagnosis of neurofibromatosis type 1 (NF1) and other RASopathies is difficult without molecular testing. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  16. Cardiac troponin T and familial hypertrophic cardiomyopathy: an energetic affair

    Science.gov (United States)

    Schwartz, Ketty; Mercadier, Jean-Jacques

    2003-01-01

    It has long been noted that while patients with familial hypertrophic cardiomyopathy due to cardiac troponin T (cTnT) mutations often suffer sudden cardiac death, they do not develop significant ventricular hypertrophy, suggesting that a distinct cellular mechanism apart from alterations in myocardial contractility is responsible. A new study has revealed that a single missense mutation in cTnT causes a striking disruption to energy metabolism, leading to cardiomyopathy. PMID:12952912

  17. Management of Keloid and Hypertrophic Scar/span>s

    Science.gov (United States)

    Edriss, A.S.; Mesták, J.

    2005-01-01

    Summary Scar management for the prevention of excessive scar formation has always been important but never so important as it is today. Optimal management continues to be an enigma for surgeons, and the best modality of treatment has been debated for many years. However, most studies have unfortunately been either retrospective or case report descriptions. Advances in scar management have been hampered by confusing or ambiguous terminology. There is no consensus on what amount of post-traumatic skin scar formation is "normal" and what should be considered "hypertrophic". In the World Health Organization's ICD-9, there is no diagnostic code for hypertrophic scar - only keloid is listed. Yet the medical and scientific literature distinguishes them as different conditions. This confusion results in inappropriate management of scar formation, and occasionally contributes to decision making related to elective or cosmetic surgery. Our experience suggests that there is no single treatment for scars that is adequate and that clinical judgement is very important when considering treatment and balancing the potential benefits of the various treatments available. The goal of treating scars is to restore functionality, provide relief of symptoms, enhance cosmetics, and prevent recurrence. This article is based on our scientific and clinical experiences and focuses on over-the-counter options to manage keloid and hypertrophic scar/span>s. PMID:21991008

  18. Two cases of apical ballooning syndrome masking apical hypertrophic cardiomyopathy.

    Science.gov (United States)

    Roy, Ranjini Raina; Hakim, Fayaz A; Hurst, R Todd; Simper, David; Appleton, Christopher P

    2014-04-01

    Apical akinesis and dilation in the absence of obstructive coronary artery disease is a typical feature of stress-induced (takotsubo) cardiomyopathy, whereas apical hypertrophy is seen in apical-variant hypertrophic cardiomyopathy. We report the cases of 2 patients who presented with takotsubo cardiomyopathy and were subsequently found to have apical-variant hypertrophic cardiomyopathy, after the apical ballooning from the takotsubo cardiomyopathy had resolved. The first patient, a 43-year-old woman with a history of alcohol abuse, presented with shortness of breath, electrocardiographic and echocardiographic features consistent with takotsubo cardiomyopathy, and no significant coronary artery disease. An echocardiogram 2 weeks later revealed a normal left ventricular ejection fraction and newly apparent apical hypertrophy. The 2nd patient, a 70-year-old woman with pancreatitis, presented with chest pain, apical akinesis, and a left ventricular ejection fraction of 0.39, consistent with takotsubo cardiomyopathy. One month later, her left ventricular ejection fraction was normal; however, hypertrophy of the left ventricular apex was newly noted. To our knowledge, these are the first reported cases in which apical-variant hypertrophic cardiomyopathy was masked by apical ballooning from stress-induced cardiomyopathy.

  19. What Is Age-Related Macular Degeneration?

    Science.gov (United States)

    ... Stories Español Eye Health / Eye Health A-Z Age-Related Macular Degeneration Sections What Is Macular Degeneration? Who is at ... Low Vision: Making the Most of Low Vision Age-Related Macular Degeneration Vision Simulator AMD Pictures and Videos: What Does ...

  20. Spheroidal Degeneration of the Cornea

    Directory of Open Access Journals (Sweden)

    Erdem Dinç

    2011-08-01

    Full Text Available A thirty-one-year-old male patient presented with bilateral epiphora and stinging sensation in the cornea. Detailed history revealed that a bilateral corneal scraping had been made regarding the initial diagnosis of fungal keratitis. His bestcorrected visual acuities were 20/20 and 20/30 in right and left eyes, respectively. Biomicroscopy showed bilateral amber colored spherules in the anterior stroma of the central cornea. The diagnosis of spheroidal corneal degeneration was established and symptomatic therapy with artificial tear drops was prescribed. Ultraviolet light is widely accepted to be the main etiological factor in the pathogenesis of spheroidal degeneration. Because of difficulties in the early stages of the diagnostic process of the disease, incorrect diagnoses can be made with inappropriate interventions. (Turk J Ophthalmol 2011; 41: 264-6

  1. Spheroidal Degeneration of the Cornea

    OpenAIRE

    Erdem Dinç; Ufuk Ad›güzel; Bahri Ayd›n; İdil Göksel; Özlem Y›ld›r›m

    2011-01-01

    A thirty-one-year-old male patient presented with bilateral epiphora and stinging sensation in the cornea. Detailed history revealed that a bilateral corneal scraping had been made regarding the initial diagnosis of fungal keratitis. His bestcorrected visual acuities were 20/20 and 20/30 in right and left eyes, respectively. Biomicroscopy showed bilateral amber colored spherules in the anterior stroma of the central cornea. The diagnosis of spheroidal corneal degeneration was esta...

  2. Topical Calcipotriol for Preventive Treatment of Hypertrophic Scars A Randomized, Double-blind, Placebo-Controlled Trial

    NARCIS (Netherlands)

    van der Veer, Willem M.; Jacobs, Xander E.; Waardenburg, Ingmar E.; Ulrich, Magda M.; Niessen, Frank B.

    2009-01-01

    Objectives: To evaluate the efficacy of topical application of calcipotriol to healing wounds in preventing or reducing hypertrophic scar formation and to investigate the biochemical properties of the epidermis associated with hypertrophic scar formation. Design: Randomized, double-blind,

  3. Topical Calcipotriol for Preventive Treatment of Hypertrophic Scars A Randomized, Double-blind, Placebo-Controlled Trial

    NARCIS (Netherlands)

    van der Veer, Willem M.; Jacobs, Xander E.; Waardenburg, Ingmar E.; Ulrich, Magda M.; Niessen, Frank B.

    2009-01-01

    Objectives: To evaluate the efficacy of topical application of calcipotriol to healing wounds in preventing or reducing hypertrophic scar formation and to investigate the biochemical properties of the epidermis associated with hypertrophic scar formation. Design: Randomized, double-blind,

  4. Hypertrophic scars after therapy with CO2 laser for treatment of multiple cutaneous neurofibromas

    NARCIS (Netherlands)

    J.U. Ostertag (Judith); C.C.W. Theunissen (Chantal); H.A.M. Neumann (Martino)

    2002-01-01

    textabstractBACKGROUND. CO2 laser surgery is a treatment modality for cutaneous neurofibromas. OBJECTIVE. Hypertrophic and atrophic scars can result from treatment with CO2 laser surgery. We present a case of cutaneous neurofibromatosis that developed hypertrophic scars postoperatively. METHODS.

  5. Survival and sudden cardiac death after septal ablation for hypertrophic obstructive cardiomyopathy

    DEFF Research Database (Denmark)

    Jensen, Morten Kvistholm; Havndrup, Ole; Hassager, Christian

    2011-01-01

    Reports of long-term survival and the risk of sudden cardiac death (SCD) after percutaneous transluminal septal myocardial ablation (PTSMA) in patients with hypertrophic obstructive cardiomyopathy (HOCM) are sparse.......Reports of long-term survival and the risk of sudden cardiac death (SCD) after percutaneous transluminal septal myocardial ablation (PTSMA) in patients with hypertrophic obstructive cardiomyopathy (HOCM) are sparse....

  6. Influence of Septal Thickness on the Clinical Outcome After Alcohol Septal Alation in Hypertrophic Cardiomyopathy

    DEFF Research Database (Denmark)

    Jensen, Morten K; Jacobsson, Linda; Almaas, Vibeke Marie

    2016-01-01

    BACKGROUND: We assessed the influence of interventricular septal thickness (IVSd) on the clinical outcome and survival after alcohol septal ablation (ASA) in patient with hypertrophic cardiomyopathy. METHODS AND RESULTS: We analyzed 531 patients with hypertrophic cardiomyopathy (age: 56±14 years...

  7. HYPERTROPHIC CARDIOMYOPATHY AS A PART OF INHERITED MALFORMATION SYNDROMES IN INFANTS

    Directory of Open Access Journals (Sweden)

    M.V. Tural'chuk

    2011-01-01

    Full Text Available The data of clinical and instrumental examination of two infantile patients with obstructive hypertrophic cardiomyopathy in association with marked multisystem involvement as a picture of inherited malformation syndromes are given.Key words: infants, hypertrophic cardiomyopathy, LEOPARD syndrome, Noonan syndrome.(Voprosy sovremennoi pediatrii — Current Pediatrics. 2011; 10 (3: 166–169

  8. Outcome of Alcohol Septal Ablation in Mildly Symptomatic Patients With Hypertrophic Obstructive Cardiomyopathy

    DEFF Research Database (Denmark)

    Veselka, Josef; Faber, Lothar; Liebregts, Max

    2017-01-01

    BACKGROUND: The long-term efficacy and safety of alcohol septal ablation (ASA) in patients with highly symptomatic hypertrophic obstructive cardiomyopathy has been demonstrated. The aim of this study was to evaluate the long-term outcomes of mildly symptomatic patients with hypertrophic obstructive...

  9. Compression therapy affects collagen type balance in hypertrophic scar.

    Science.gov (United States)

    Tejiram, Shawn; Zhang, Jenny; Travis, Taryn E; Carney, Bonnie C; Alkhalil, Abdulnaser; Moffatt, Lauren T; Johnson, Laura S; Shupp, Jeffrey W

    2016-04-01

    The effects of pressure on hypertrophic scar are poorly understood. Decreased extracellular matrix deposition is hypothesized to contribute to changes observed after pressure therapy. To examine this further, collagen composition was analyzed in a model of pressure therapy in hypertrophic scar. Hypertrophic scars created on red Duroc swine (n = 8) received pressure treatment (pressure device mounting and delivery at 30 mm Hg), sham treatment (device mounting and no delivery), or no treatment for 2 wk. Scars were assessed weekly and biopsied for histology, hydroxyproline quantification, and gene expression analysis. Transcription levels of collagen precursors COL1A2 and COL3A1 were quantified using reverse transcription-polymerase chain reaction. Masson trichrome was used for general collagen quantification, whereas immunofluorescence was used for collagen types I and III specific quantification. Total collagen quantification using hydroxyproline assay showed a 51.9% decrease after pressure initiation. Masson trichrome staining showed less collagen after 1 (P < 0.03) and 2 wk (P < 0.002) of pressure application compared with sham and untreated scars. Collagen 1A2 and 3A1 transcript decreased by 41.9- and 42.3-fold, respectively, compared with uninjured skin after pressure treatment, whereas a 2.3- and 1.3-fold increase was seen in untreated scars. This decrease was seen in immunofluorescence staining for collagen types I (P < 0.001) and III (P < 0.04) compared with pretreated levels. Pressure-treated scars also had lower levels of collagen I and III after pressure treatment (P < 0.05) compared with sham and untreated scars. These results demonstrate the modulation of collagen after pressure therapy and further characterize its role in scar formation and therapy. Copyright © 2016 Elsevier Inc. All rights reserved.

  10. Chronic hypertrophic cranial pachymeningitis associated with HTLV-I infection.

    Science.gov (United States)

    Kawano, Y; Kira, J

    1995-10-01

    Two patients presenting with recurrent multiple cranial neuropathy showed diffuse thickening and gadolinium enhancement of the dura mater on brain MRI. Both had anti-HTLV-I antibodies in serum. A quantitative polymerase chain reaction study of the peripheral blood disclosed that the HTLV-I proviral DNA loads increased considerably in one case and moderately in the other. Both showed a spontaneous proliferation of peripheral blood lymphocytes as well as an increase in helper/inducer T cells. Neither had any other underlying infections or autoimmune diseases. Thus it is possible that hypertrophic pachymeningitis developed as a result of multiorgan involvement of HTLV-I infection in these patients.

  11. Collecting, Analyzing, and Publishing Massive Data about the Hypertrophic Cardiomyopathy

    Science.gov (United States)

    Montserrat, Lorenzo; Cotelo-Lema, Jose Antonio; Luaces, Miguel R.; Seco, Diego

    We present in this paper the architecture and some implementation details of a Document Management System and Workflow to help in the diagnosis of the hypertrophic cardiomyopathy, one of the most frequent genetic cardiovascular diseases. The system allows a gradual and collaborative creation of a knowledge base about the mutations associated with this disease. The system manages both the original documents of the scientific papers and the data extracted from these papers by the experts. Furthermore, a semiautomatic report generation module exploits this knowledge base to create high quality reports about the studied mutations.

  12. Histologic Sequelae of Apical Hypertrophic Cardiomyopathy: Dystrophic Calcification

    Directory of Open Access Journals (Sweden)

    Shahryar G Saba

    2017-05-01

    Full Text Available We present cardiac computed tomography (CT findings demonstrating apical hypertrophic cardiomyopathy with dystrophic calcification of the left ventricular apex. The absence of significant epicardial coronary artery disease demonstrated by coronary CT angiography suggests that increased wall tension and decreased microvascular perfusion over time account for the dyskinetic apical myocardium, rather than myocardial infarction secondary to atherosclerotic plaque rupture. These observations support CT as the imaging modality of choice to visualize the deposition of calcium in injured myocardial tissue, a recognized occurrence in chronically infarcted myocardium.

  13. [Hypertrophic pyloric stenosis. Review of 314 cases (author's transl)].

    Science.gov (United States)

    De Jaureguizar, E; Cabrera, R; Herrero, E; Monereo, J

    1978-04-01

    Three hundred fourteen patients with hypertrophic pyloric stenosis have been operated from 1969 to 1976. The different etiologies are discussed. Clinical and analytical data are evaluated. Radiology is employed in 99 per 100 of the cases with no complications. Medical treatment used is exposed clearly as well as the surgical procedure (Monereo's modification of the classical Fredet-Ramstedt technique). Postoperative results and complications such as vomiting, wound infection (4.4 per 100), eventration (0.6 per 100) and fatal outcome (0.6 per 100) are thouroughly detailed as well as the associated pathology.

  14. Lung carcinoma with hypertrophic osteoarthropathy in a teenager

    Directory of Open Access Journals (Sweden)

    Jeremy Whelan

    2011-03-01

    Full Text Available Hypertrophic osteoarthropathy (HOA characterised by arthralgia, clubbing and periosteal proliferation of long bones, is rarely encountered in children and adolescents. Whereas in adults over 80% of cases are associated with malignancy, in children the majority of cases are due to non-neoplastic causes such as cystic fibrosis, bilary atresia and congenital heart disease. Up to 5% of adults with lung cancer demonstrate signs of HOA. However, lung cancer is extremely uncommon in children and young people. Here we report a case of lung adenocarcinoma in an 18 year old male associated with HOA present both at diagnosis and at subsequent disease progression.

  15. FACTORS INFLUENCING THE OCCURRENCE OF HYPERTROPHIC SCARS AMONG POSTOPERATIVE PATIENTS IN GARUT, INDONESIA

    Directory of Open Access Journals (Sweden)

    Andri Nugraha

    2017-02-01

    Full Text Available Background: Hypertrophic scar causes physical and psychological problems. Thus understanding the factors related to the occurrence of hypertrophic scar tissue is needed. Little is known about its influencing factors in Indonesia, especially in Garut. Objective: This study aims to examine the relationships between hypertrophic scar and its influencing factors, and identify the most dominant factor of the occurrence of hypertrophic scars. Methods: This was an observational case control study using retrospective approach in Polyclinic of Surgery of Regional Public Hospital of dr. Slamet of Garut Regency. There were 40 samples recruited in this study by purposive sampling, which was divided to be case group (20 patients and control group (20 patients. Data were collected using Stony Brook Scar Evaluation Scale by observation and documentation of the medical records of patients. Data were analyzed using logistic regression analysis Results: Findings indicated that there were significant relationships between the surgical wound infection (p = 0.02, family history (p = 0.026, and type of suture (p = 0.043 with the occurrence of hypertrophic scars. The most dominant factor on the occurrence of hypertrophic scars was type of suture, acid polyglactin 910. The variables that had no significant relationships with the occurrence of hypertrophic scar tissue were age (p = 0.34, area of surgical wound (p = 0.177, and smoking habit (p = 0.479. Conclusion: There were significant relationships between infection of surgical wound, genetic history, the type of suture, and the occurrence of hypertrophic scar tissue. The most dominant factor that influenced the occurrence of hypertrophic scar tissue was the type of suture. Therefore, it is suggested to health professionals to modify the using of acid polyglactin 910 sutures, and nurses particularly need to provide the information regarding the family history and genetic-related hypertrophic scar, and prevent the

  16. Scintigraphic evaluation of regional myocardial sympathetic activity in patients with hypertrophic cardiomyopathy. Comparison between asymmetrical hypertrophic cardiomyopathy and apical hypertrophy

    Energy Technology Data Exchange (ETDEWEB)

    Eno, Shin; Takeo, Eiichiro; Sasaki, Satoshi; Matsuda, Keiji; Fujii, Hideaki; Kanazawa, Ikuo [Chugoku Rosai General Hospital, Kure, Hiroshima (Japan)

    1998-02-01

    Using {sup 123}I-MIBG (metaiodobenzylguanidine) and {sup 201}Tl imagings, an examination concerning the relation between the hypertrophic region and its sympathetic nervous function was done. Subjects were 12 normal adults (4 males and 8 females, mean age 61.3 yr), 13 patients with asymmetrical hypertrophic cardiomyopathy (10 males and 3 females, 63.9 yr) and 13 patients with apical hypertrophy (9 males and 4 females, 67.2 yr). The SPECT apparatus was Toshiba two-gated gamma camera GCA 7200A. At 20 min and 3 hr after intravenous injection of 111 MBq of {sup 123}I-MIBG, myocardial SPECT and planar images were obtained with collimator LEHR under following conditions: photoelectric peak 159 KeV, window width 20%, matrix size 64 x 64 (256 x 256 for the planar image), step angle 6deg, 40 sec/step and 180deg for 1 camera. In another day, {sup 201}Tl SPECT and planar imagings were performed 10 min after intravenous injection of 111 MBq of {sup 201}Tl for the photoelectric peak 72 KeV under similar conditions to above. SPECT images were reconstructed using Butterworth filter and Shepp and Logan filter. Images were examined for the defect score, myocardium/mediastinum ratio, whole heart washout rate and regional washout rate. In the asymmetrical hypertrophic myopathy, abnormal sympathetic nerve function was recognized on the regions regardless of their disease severity while in the apical hypertrophy, abnormality was restricted on the apical region. Therefore, the two diseases were found different from each other from the aspect of sympathetic nerve functions. (K.H.)

  17. PHOTODYNAMIC THERAPY OF MACULAR DEGENERATION

    Directory of Open Access Journals (Sweden)

    Davorin Sevšek

    2004-05-01

    Full Text Available Background. University Eye Clinic in Ljubljana began to perform a photodynamic therapy (PDT in July 2002. Up to September 2003 there were 51 PDT interventions on 36 eyes. Almost half of the eyes had one or more reinterventions. PDT was used on 29 eyes with age related macular degeneration (AMD. Just before the treatment all of them had visual acuity (VA 6/60 or better and there were predominantly classical or classical choroidal neovascular (CNV membranes. Most VA did not changed significantly in follow-up visits but diameter of CNV membranes was obviously reduced. In macular degeneration due to pathologic myopia (MMD, photodynamic therapy was performed on 7 eyes with mainly occult CNV and VA was 12/60 or better. VA was mostly better in follow-up visits than before treatment with PDT.Conclusions. There were no serious complications during and after PDT interventions. Two patients had back pain during the infusion of Verteporfin but there was no need to stop the intervention.

  18. Subaortic and midventricular obstructive hypertrophic cardiomyopathy with extreme segmental hypertrophy

    Directory of Open Access Journals (Sweden)

    Karoulas Takis

    2007-03-01

    Full Text Available Abstract Background Subaortic and midventricular hypertrophic cardiomyopathy in a patient with extreme segmental hypertrophy exceeding the usual maximum wall thickness reported in the literature is a rare phenomenon. Case Presentation A 19-year-old man with recently diagnosed hypertrophic cardiomyopathy (HCM was referred for sudden death risk assessment. The patient had mild exertional dyspnea (New York Heart Association functional class II, but without syncope or chest pain. There was no family history of HCM or sudden death. A two dimensional echocardiogram revealed an asymmetric type of LV hypertrophy; anterior ventricular septum = 49 mm; posterior ventricular septum = 20 mm; anterolateral free wall = 12 mm; and posterior free wall = 6 mm. The patient had 2 types of obstruction; a LV outflow obstruction due to systolic anterior motion of both mitral leaflets (Doppler-estimated 38 mm Hg gradient at rest; and a midventricular obstruction (Doppler-estimated 43 mm Hg gradient, but without apical aneurysm or dyskinesia. The patient had a normal blood pressure response on exercise test and no episodes of non-sustained ventricular tachycardia in 24-h ECG recording. Cardiac MRI showed a gross late enhancement at the hypertrophied septum. Based on the extreme degree of LV hypertrophy and the myocardial hyperenhancement, an implantation of a cardioverter-defibrillator was recommended prophylactically for primary prevention of sudden death. Conclusion Midventricular HCM is an infrequent phenotype, but may be associated with an apical aneurysm and progression to systolic dysfunction (end-stage HCM.

  19. Family communication in a population at risk for hypertrophic cardiomyopathy.

    Science.gov (United States)

    Batte, Brittany; Sheldon, Jane P; Arscott, Patricia; Huismann, Darcy J; Salberg, Lisa; Day, Sharlene M; Yashar, Beverly M

    2015-04-01

    Encouraging family communication is an integral component of genetic counseling; therefore, we sought to identify factors impacting communication to family members at risk for Hypertrophic Cardiomyopathy (HCM). Participants (N = 383) completed an online survey assessing: 1) demographics (gender, genetic test results, HCM family history, and disease severity); 2) illness representations; 3) family functioning and cohesiveness; 4) coping styles; 5) comprehension of HCM autosomal dominant inheritance; and 6) communication of HCM risk information to at-risk relatives. Participants were a national sample of individuals with HCM, recruited through the Hypertrophic Cardiomyopathy Association. Data from 183 participants were analyzed using a logistic regression analysis, with family communication as a dichotomous dependent variable. We found that female gender and higher comprehension of autosomal dominant inheritance were significant predictors of participants' communication of HCM risk information to all their siblings and children. Our results suggest that utilizing interventions that promote patient comprehension (e.g., a teaching-focused model of genetic counseling) are important and may positively impact family communication within families with HCM.

  20. CT findings of intrathoricic neoplasm associated with hypertrophic osteoarthropathy

    Energy Technology Data Exchange (ETDEWEB)

    Hwang, Hee Sung; Choe, Kyu Ok; Chung, Jin Il; Oh, Sei Chung [College of Medicine Yonsei University, Seoul (Korea, Republic of)

    1994-02-15

    Hypertrophic osteoarthropathy(HOA) is a clinical syndrome consisting of clubbing, periostitis and synovitis. Most frequent causes of hypertrophic osteoarthropathy are intrathoracic neoplasms, among which the bronchogenic carcinoma ranks the highest. But computed tomographic evaluation of intrathoracic neoplasm associated with HOA has been seldom reported. The purpose of this study is to evaluate CT findings of intrathoracic neoplasm associated with HOA, and to infer possible mechanism. Seven cases of intrathoracic neoplasm associated with HOA were included in our study. Diagnoses of HOA were made by Tc99m bone scintigraphy or plain radiography. The findings of chest CT scans were reviewed retrospectively, with main interests on their size, location and internal characteristics, ect. Seven cases of intrathoracic neoplasm consisted of five bronchogenic carcinomas and two thymic tumors. The size of intrathoracic tumors were relatively large ranging from 6cm to 13cm(average 8.0cm). All thoracic neoplasms showed wide pleural contact, and one of them invaded thoracic wall. The range of length of pleural contact was 5-18cm(average 9.9cm). All of seven patients had internal necrosis, and one of them showed cavitation in thoracic mass. Intrathoracic neoplasms associated with HOA had a tendency to be large, to contain internal necrosis, and to widely abut the thoracic pleura.

  1. Planktonic ciliates in a hypertrophic pond: functional role and importance.

    Science.gov (United States)

    Sanchez Rodriguez, Ma del Rosario; Lugo Vazquez, Alfonso; Oliva Martinez, Ma Guadalupe; Verver y Vargas, Jaqueline Garcia; Rodriguez Rocha, Armando; Peralta Soriano, Laura

    2011-07-01

    Taxonomical composition and abundance of the planktonic ciliate assemblage in a Lake Tezozomoc, a hypertrophic pond located into an urban park in Mexico City, was investigated along the rainy season (May to October). The aims of the study were to know the main trophic roles and ecological significance of ciliates in a highly productive environment. A low number of taxa (27) and a wide abundance fluctuation (104-387 cil ml(-1)) were found. The most abundant species (up to 162 cil ml(-1)) was Halteria grandinella, an oligotrich ciliate that graze on bacteria and picoplankton, but also several big body sized species that feed on pico and nanoplankton were abundant. Sudden temporal changes in species dominance occurred. Ciliate biomass was very high and fluctuated widely (1.6-88 10(6) microm(3) ml(-1)) being dominated by the >50 microm size fraction that mainly included the pico and nanoplankton feeders. Ciliates are a very important component in the plankton of hypertrophic lakes and their main control factor seems to be the grazing by big-body size Daphnia species.

  2. Upregulation of TRPC1 contributes to contractile function in isoproterenol-induced hypertrophic myocardium of rat.

    Science.gov (United States)

    Chen, Mo-Si; Xiao, Jun-Hua; Wang, Yong; Xu, Bo-Ming; Gao, Lu; Wang, Jia-Ling

    2013-01-01

    The transient receptor potential canonical channel 1 (TRPC1) is a crucial component of the stretch-activated ion channels (SACs). The objective of this research was to demonstrate the contribution of TRPC1 in maintaining cardiac contractile function in the hypertrophic myocardium. Hypertrophic rat hearts were induced by injecting isoproterenol intraperitoneally, and the expressions of TRPC1/3/6 and Na(+)/Ca(2+) exchanger 1 (NCX1) proteins were analyzed by Western blot. The intracellular calcium images, the action potential of myocardium, the length-dependent contractile force of ventricle muscle and the cardiac output of isolated heart were investigated. The expression of TRPC1 was increased in the hypertrophic myocardium. After being stretched, the ascendant amplitude of the increase in the intracellular calcium ion concentration ([Ca(2+)]i) in the hypertrophic myocardium was higher than that in the normal myocardium. The increase of the APD50 and the amplitude of the membrane potential depolarization were more significant in the hypertrophic myocardium after the activation of SACs. When the heart preparations were perfused with Tyrode's solution, there was no difference in the cardiac systolic function between the cardiac hypertrophy group and the control group. Gadolinium, a SACs blocker, reduced the length-dependent contractile force and suppressed the ascending limb of the Frank-Starling curves in the hypertrophic heart. The upregulation of TRPC1 contributes to the contractile function in the hypertrophic myocardium by increasing [Ca(2+)]i through the SACs. © 2013 S. Karger AG, Basel

  3. Squamous Cell Carcinoma Arising in Hypertrophic Lichen Planus: A Review and Analysis of 38 Cases.

    Science.gov (United States)

    Knackstedt, Thomas J; Collins, Lindsey K; Li, Zhongze; Yan, Shaofeng; Samie, Faramarz H

    2015-12-01

    Hypertrophic lichen planus is a chronic variant of lichen planus with controversial malignant association. To describe and analyze the relationship of squamous cell carcinoma (SCC) and hypertrophic lichen planus. A retrospective chart review of patients with hypertrophic lichen planus and SCC was performed at the authors' institution. Thereafter, scientific databases were searched for articles reporting cases of SCC arising in hypertrophic lichen planus. Patient demographics, immune status, lichen planus features, and SCC data points were extracted for each patient and evaluated. Thirty-eight cases of SCC in hypertrophic lichen planus occurred in 16 women, average age: 61.4, and 22 men, average age: 51.3, after a lag time of 88 days to 40 years. Squamous cell carcinoma was uniformly located on the lower extremity. Men had larger SCC than women (p = .027) and a significantly longer lag time to SCC development (p = .002). Long lag time was associated with a smaller SCC size (p = .032). In the past, hypertrophic lichen planus and SCC have been considered isolated diseases. Based on an increasing number of cases, the association between hypertrophic lichen planus and keratinocyte malignancies warrants surveillance.

  4. Changes in gadolinium-DTPA enhanced magnetic resonance signal intensity ratio in hypertrophic cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Tsukihashi, Hironori; Ishibashi, Yutaka; Shimada, Toshio; Hatano, Jun; Tanabe, Kazuaki; Ooyake, Nobuyuki; Morioka, Shigefumi; Moriyama, Katsutoshi (Shimane Medical Univ., Izumo (Japan))

    Serial gadolinium-diethylene-triamine-pentaacetic acid (Gd-DTPA) enhanced magnetic resonance (MR) signal intensity ratios were measured in 6 normal subjects and 20 hypertrophic cardiomyopathy (HCM) patients to try to differentiate normal from disorganized myocardial tissue. Images were obtained at 10-minute intervals 5-60 minutes after Gd-DTPA (0.1 mmol/kg) injection. The signal intensity ratio (myocardial signal intensity/skeletal muscle signal intensity) was measured at both hypertrophic and non-hypertrophic regions in each image at the apex and mid-ventricular levels. The signal intensity ratio was standardized to compare each case. Hypertrophic myocardium was classified into two types. Type I in 11 of 20 patients was visualized as a homogeneous image, while type II in the other 9 patients was revealed as a mixed isointensity and high intensity area. The peak value of the standardized signal intensity ratio at the apex level was 1.28[+-]0.09 in HCM patients and 1.23[+-]0.06 in normal subjects, and at the mid ventricular level was 1.26[+-]0.07 in hypertrophic regions, 1.17[+-]0.12 in non-hypertrophic regions, and 1.16[+-]0.07 in normal subjects. Thirty minutes after Gd injection, the standardized signal intensity ratio at the apex level was 1.21[+-]0.08 in HCM patients and 1.07[+-]0.08 in normal subjects, and those at the mid ventricular level was 1.20[+-]0.09 in hypertrophic regions, 1.11[+-]0.11 in non-hypertrophic regions, and 1.04[+-]0.06 in normal subjects. The delayed decay of the signal intensity ratio and high signal intensity ratio in Gd-DTPA enhanced MR images are useful in myocardial tissue characterization in hypertrophic cardiomyopathy. (author).

  5. Genetic association studies in lumbar disc degeneration

    DEFF Research Database (Denmark)

    Eskola, Pasi J; Lemmelä, Susanna; Kjaer, Per

    2012-01-01

    Low back pain is associated with lumbar disc degeneration, which is mainly due to genetic predisposition. The objective of this study was to perform a systematic review to evaluate genetic association studies in lumbar disc degeneration as defined on magnetic resonance imaging (MRI) in humans....

  6. Inflammatory profiles in canine intervertebral disc degeneration

    NARCIS (Netherlands)

    Willems, Nicole; Tellegen, Anna R.; Bergknut, Niklas; Creemers, Laura B.; Wolfswinkel, Jeannette; Freudigmann, Christian; Benz, Karin; Grinwis, Guy C M; Tryfonidou, Marianna A.; Meij, Björn P.

    2016-01-01

    BACKGROUND: Intervertebral disc (IVD) disease is a common spinal disorder in dogs and degeneration and inflammation are significant components of the pathological cascade. Only limited studies have studied the cytokine and chemokine profiles in IVD degeneration in dogs, and mainly focused on gene

  7. Cardiac sarcoid: a chameleon masquerading as hypertrophic cardiomyopathy and dilated cardiomyopathy in the same patient.

    Science.gov (United States)

    Agarwal, Anushree; Sulemanjee, Nasir Z; Cheema, Omar; Downey, Francis X; Tajik, A Jamil

    2014-05-01

    Sarcoidosis is a multisystem, granulomatous disease of unknown etiology often seen in young adults, with cardiac involvement in more than one-quarter of sarcoid patients. The clinical presentation of cardiac sarcoid depends upon the location and extent of myocardium involved. Although cardiac sarcoid may produce asymmetrical septal hypertrophy, it is most commonly considered in the differential diagnosis of dilated cardiomyopathy. The hypertrophic stage of cardiac sarcoid is rarely seen. We describe a case of cardiac sarcoid in a young patient wherein a distinctive appearance of the cardiac sarcoid spectrum from "hypertrophic" stage to thinned/scarred stage, masquerading as hypertrophic cardiomyopathy followed by dilated cardiomyopathy, is demonstrated. © 2014, Wiley Periodicals, Inc.

  8. Down Syndrome with Complete Atrioventricular Septal Defect, Hypertrophic Cardiomyopathy, and Pulmonary Vein Stenosis.

    Science.gov (United States)

    Mahadevaiah, Guruprasad; Gupta, Manoj; Ashwath, Ravi

    2015-10-01

    The prevalence of congenital heart disease in infants with Down syndrome is 40%, compared with 0.3% in children who have normal chromosomes. Atrioventricular and ventricular septal defects are often associated with chromosomal aberrations, such as in trisomy 21, whereas hypertrophic cardiomyopathy is chiefly thought to be secondary to specific gene mutations. We found only one reported case of congenital hypertrophic cardiomyopathy and atrioventricular septal defect in an infant with Down syndrome. Here, we report atrioventricular septal defect, hypertrophic cardiomyopathy, and pulmonary vein stenosis in a neonate with Down syndrome-an apparently unique combination. In addition, we discuss the relevant medical literature.

  9. Perioperative anesthetic management of patients with hypertrophic cardiomyopathy for noncardiac surgery: A case series

    Directory of Open Access Journals (Sweden)

    Sahoo Rajendra

    2010-01-01

    Full Text Available Hypertrophic cardiomyopathy with or without left ventricular outflow tract obstruction is characterized by asymmetric hypertrophy of the interventricular septum causing intermittent obstruction of the left ventricular outflow tract. Because Hypertrophic cardiomyopathy is the most common genetic cardiovascular disease, it may present to the anesthesiologist more often than anticipated, sometimes in undiagnosed form during routine preoperative visit. Surgery and anesthesia often complicate the perioperative outcome if adequate monitoring and proper care are not taken. Therefore, a complete understanding of the pathophysiology, hemodynamic changes and anesthetic implications is needed for successful perioperative outcome. We hereby describe the perioperative management of three patients with Hypertrophic cardiomyopathy for different surgical procedures.

  10. Laser therapy and macular degeneration

    Science.gov (United States)

    Menchini, Ugo; Virgili, Gianni; Giansanti, Fabrizio; Giacomelli, Giovanni; Cappelli, Stefania

    2001-10-01

    Among macular diseases, choroidal neovascularization (CNV) is one of the most common causes of visual loss, especially in the form associated with age-related macular degeneration and pathologic myopia. Research on these diseases has recently evaluated new treatment modalities that use laser light differently; among these, photodynamic therapy (PDT) has been introduced in the clinical practice, allowing us to expand the possibility of reducing visual loss in patients affected by CNV. With PDT, a photosensitizer (verteporfin, VisudyneTM) is injected intravenously, and it selectively binds to new vessels; low-power laser light exposure then activates the drug, leading to oxidative damage of the endothelium and new vessels thrombosis. Yet, other therapies, such as transpupillary termotherapy, or the use of photocoagulation to cause feeder-vessel occlusion, could proof effective, but they need further investigation.

  11. Age-related macular degeneration

    DEFF Research Database (Denmark)

    la Cour, Morten; Kiilgaard, Jens Folke; Nissen, Mogens Holst

    2002-01-01

    Age-related macular degeneration (AMD) is a common macular disease affecting elderly people in the Western world. It is characterised by the appearance of drusen in the macula, accompanied by choroidal neovascularisation (CNV) or geographic atrophy. The disease is more common in Caucasian...... individuals than in pigmented races. In predominantly Caucasian populations, the age-standardised prevalence of AMD in at least one eye is 7760 cases per million. The age-standardised cumulated 1-year incidence of AMD in at least one eye is 1051 cases per million individuals. AMD is the most important single...... cause of blindness among Caucasian individuals in developed countries. Blindness resulting from AMD rarely occurs before age 70, and most cases occur after age 80. The age-standardised 1-year incidence of legal blindness resulting from AMD is 212 cases per million. Two-thirds of AMD cases have CNV...

  12. Ultrastructural myocardial changes in seven cats with spontaneous hypertrophic cardiomyopathy

    DEFF Research Database (Denmark)

    Christiansen, Liselotte Bruun; Prats Gavalda, Clara; Hyttel, Poul

    2015-01-01

    OBJECTIVES: Hypertrophic cardiomyopathy (HCM) is the most common heart disease in cats and shares clinical and pathological characteristics with human HCM. Little is known about the pathogenic mechanisms underlying development of spontaneous feline HCM. ANIMALS: The study population consisted...... of seven cats diagnosed with HCM and eight age-matched cats with no evidence of cardiac disease. METHODS: Fresh myocardial biopsies taken from the middle of the left ventricular posterior free wall were obtained and examined with transmission electron microscopy. RESULTS: Electron microscopic examination...... showed ultrastructural aberrations of the myocardial cytoarchitecture and of the interstitium in the seven cats with HCM. In the most severely affected cats the myofibrils were disorganized and subsarcolemmal mitochondria were depleted. In control cats, contraction band artifacts were commonly seen...

  13. Hypertrophic osteoarthropathy manifested with isolated calcaneal periostitis in bone scintigraphy.

    Science.gov (United States)

    Moralidis, Efstratios; Gerasimou, Georgios; Theodoridou, Athina; Hilidis, Ilias; Mylonaki, Efrosyni; Gotzamani-Psarrakou, Anna

    2010-05-01

    Hypertrophic osteoarthropathy (HOA) is an incompletely understood syndrome characterized by digital clubbing and periosteal proliferation of long bones and it is commonly associated with primary lung tumors. Bone scintigraphy is a sensitive method in detecting HOA and characteristic findings have been reported. We present the case of a man with newly diagnosed non-small cell lung cancer, unremarkable clinical examination and blood tests and no digital clubbing. During disease staging, however, bone scintigraphy showed intense calcaneal cortical proliferation bilaterally without involvement of other parts of the skeleton. Cortical reaction of both calcanei resolved significantly after chemotherapy. This case indicates that HOA may manifest with isolated calcaneal periostitis bilaterally, which is a new addition to the literature.

  14. Cardiac symptoms before sudden cardiac death caused by hypertrophic cardiomyopathy

    DEFF Research Database (Denmark)

    Lynge, Thomas Hadberg; Risgaard, Bjarke; Jabbari, Reza

    2016-01-01

    AIMS: Hypertrophic cardiomyopathy (HCM) is a frequent cause of sudden cardiac death (SCD) among the young (SCDY). The aim of this study was to characterize symptoms before SCDY due to HCM. METHODS AND RESULTS: Through review of all death certificates, we identified all SCDs in Danes aged 1-35 years...... in 2000-2009. Nationwide we included all deaths (n = 8756) and identified 431 autopsied SCDYs. All available records from hospitals and general practitioners were retrieved. To compare symptoms, we included a control groups consisting of traffic accident victims (n = 74). In the 10-year study period, 431...... autopsied SCDY cases were reviewed and 38 cases (9%) were included, of which 22 (58%) had morphologic findings diagnostic of HCM and 16 (42%) had findings suggestive, but not diagnostic, of HCM ('possible HCM'). Cardiac symptoms >1 h prior to death were reported in 21 (55%) of cases, and 16 (42%) sought...

  15. Cubital tunnel syndrome caused by hypertrophic burn scarring: Sonographic envisage

    Directory of Open Access Journals (Sweden)

    Alparslan Bayram Carli

    2015-08-01

    Full Text Available In nerve entrapment syndromes, an electrodiagnostic study during physical examination would usually suffice to assess localization of injury. However, in daily clinical practice, sometimes it may be necessary to depict the insight; in other words to use an imaging tool. From this point of view, with its manifold advantages, ultrasound (US is superior to other imaging technologies such as magnetic resonance imaging (MRI. According to a study, US increased the sensitivity of electrodiagnostic studies from 78% to 98%. By presenting a patient with cubital tunnel syndrome caused by hypertrophic scarring, we wanted to highlight the complementary role of US in nerve entrapment syndromes in confirming the entrapment, as well as the usefulness of it in the follow-up period of burn patients. [Hand Microsurg 2015; 4(2.000: 44-46

  16. Serious arrhythmias in patients with apical hypertrophic cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Okishige, Kaoru; Sasano, Tetsuo; Yano, Kei; Azegami, Kouji; Suzuki, Kou; Itoh, Kuniyasu [Yokohama Red Cross Hospital (Japan)

    2001-05-01

    We report cases of serious arrhythmias associated with apical hypertrophic cardiomyopathy (AHCM). Thirty-one patients were referred to our institute to undergo further assessment of their AHCM from 1988 to 1999. Three patients with nonsustained ventricular tachycardia demonstrated an {sup 123}I-MIBG regional reduction in the tracer uptake. In two patients with ventricular fibrillation (VF), the findings from {sup 123}I-MIBG imaging revealed regional sympathetic denervation in the inferior and lateral regions. Electrophysiologic study demonstrated reproducible induction of VF in aborted sudden death and presyncopal patients, resulting in the need for an implantable defibrillator device and amiodarone in each patient. Patients with refractory atrial fibrillation with a rapid ventricular response suffered from serious congestive heart failure. A prudent assessment and strategy in patients with this disease would be indispensable in avoiding a disastrous outcome. (author)

  17. Hypertrophic osteopathy associated with pulmonary adenosquamous carcinoma in a dog.

    Science.gov (United States)

    Lee, Jung-Ha; Lee, Jong-Hoon; Yoon, Hun-Young; Kim, Na-Hyun; Sur, Jung-Hyang; Jeong, Soon-Wuk

    2012-05-01

    A six-year-old intact female Maltese dog weighing 3.8 kg presented with a history of mild lameness and swelling on both forelimbs. Radiographic and computed tomographic views revealed an extensive periosteal reaction in all four limbs and a large round mass on the right middle lung lobe. A total lobectomy was performed and pulmonary adenosquamous carcinoma was histologically confirmed. A diagnosis of hypertrophic osteopathy (HO) secondary to a lung tumor was made. Periosteal proliferation decreased significantly after surgery; however, there was evidence of dyspnea, mass recurrence, and periosteal reaction three months post-operatively. This is the first case report of pulmonary adenosquamous carcinoma with HO in a dog in which we describe clinical, imaging, surgical, and histological findings.

  18. The prevention of sudden death in hypertrophic cardiomyopathy.

    Science.gov (United States)

    Sachdev, Bhavesh; Hamid, M Shoaib; Elliott, Perry M

    2002-05-01

    Hypertrophic cardiomyopathy (HCM) is a familial myocardial disease caused by mutations in cardiac sarcomeric proteins. HCM is characterised by myocyte disarray and myocardial fibrosis. Most patients are largely asymptomatic but some are prone to a number of disease-related complications, the most problematic of which is sudden cardiac death. Diagnosing patients who are at risk has not been easy because of the clinical heterogeneity of the disease, the frequent absence of symptoms prior to sudden cardiac death and the relatively low disease prevalence and annual mortality rates. To date, both low-dose amiodarone and internal cardioverter/defibrillator implantation have been advocated in high-risk individuals. Further improvements in clinical understanding and risk stratification are necessary to identify HCM patients who are at high risk of sudden death.

  19. Hypertrophic Cardiomyopathy: How do Mutations Lead to Disease?

    Energy Technology Data Exchange (ETDEWEB)

    Marsiglia, Júlia Daher Carneiro, E-mail: julia.marsiglia@usp.br; Pereira, Alexandre Costa [Instituto do Coração, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP (Brazil)

    2014-03-15

    Hypertrophic cardiomyopathy (HCM) is the most common monogenic genetic cardiac disease, with an estimated prevalence of 1:500 in the general population. Clinically, HCM is characterized by hypertrophy of the left ventricle (LV) walls, especially the septum, usually asymmetric, in the absence of any cardiac or systemic disease that leads to a secondary hypertrophy. The clinical course of the disease has a large inter- and intrafamilial heterogeneity, ranging from mild symptoms of heart failure late in life to the onset of sudden cardiac death at a young age and is caused by a mutation in one of the genes that encode a protein from the sarcomere, Z-disc or intracellular calcium modulators. Although many genes and mutations are already known to cause HCM, the molecular pathways that lead to the phenotype are still unclear. This review focus on the molecular mechanisms of HCM, the pathways from mutation to clinical phenotype and how the disease's genotype correlates with phenotype.

  20. Hypertrophic cardiomyopathy secondary to hepatitis C virus-related vasculitis.

    Science.gov (United States)

    Cavalli, Giulio; Berti, Alvise; Fragasso, Gabriele; De Cobelli, Francesco

    2016-12-01

    : Almost invariably associated with chronic HCV infection, cryoglobulinemic vasculitis is a small-vessel vasculitis commonly affecting the skin, kidneys, and peripheral nervous system. Cardiac involvement, possibly due to cardiac microcirculation involvement, is an utterly rare and severe complication. We describe a case of hypertrophic cardiomyopathy secondary to cryoglobulinemic vasculitis. Evaluation with transthoracic cardiac ultrasound and cardiac MRI evidenced severe left ventricular hypertrophy and diffuse hypokinesia, a marked decrease in left ventricular ejection fraction, and a subtle late enhancement of inferior and lateral left ventricular walls. Upon clinical stabilization, the patient received treatment with anti-CD20 monoclonal antibody rituximab. Clinical and radiological follow-up with cardiac ultrasound and cardiac MRI documented a dramatic and sustained clinical improvement, with marked reduction of left ventricular hypertrophy, resolution of late enhancement, recovery of left ventricular contractility and function.

  1. Idiopathic hypertrophic pachymeningitis mimicking hemicrania continua: An unusual clinical case.

    Science.gov (United States)

    Russo, Antonio; Silvestro, Marcello; Cirillo, Mario; Tessitore, Alessandro; Tedeschi, Gioacchino

    2017-01-01

    Background Hemicrania continua (HC) is a primary headache syndrome characterized by a unilateral, moderate, continuous headache with exacerbations marked by migrainous and cranial autonomic symptoms. However, clinical phenotypes similar to primary HC may be subtended by several disorders. Case report We report the case of a 62-year-old man experiencing, over the previous year, a headache completely consistent with HC and its absolute responsiveness to indomethacin therapy. Later, the patient developed diplopia caused by sixth cranial nerve palsy ipsilateral to headache. In this frame, clinical, laboratory and neuroimaging characteristics supported the diagnosis of idiopathic hypertrophic pachymeningitis (IHP). Conclusions IHP is a rare fibrosing inflammatory disorder leading to a localized or diffuse dura mater thickening. IHP clinical manifestations are a progressively worsening headache and signs related to cranial nerves involvement and venous sinus thrombosis. Here, we report, for the first time, a HC phenotype subtended by IHP.

  2. Hypertrophic cardiomyopathy and ultra-endurance running - two incompatible entities?

    Directory of Open Access Journals (Sweden)

    Wilson Mathew G

    2011-11-01

    Full Text Available Abstract Regular and prolonged exercise is associated with increased left ventricular wall thickness that can overlap with hypertrophic cardiomyopathy (HCM. Differentiating physiological from pathological hypertrophy has important implications, since HCM is the commonest cause of exercise-related sudden cardiac death in young individuals. Most deaths have been reported in intermittent 'start-stop' sports such as football (soccer and basketball. The theory is that individuals with HCM are unable to augment stroke volume sufficiently to meet the demands of endurance sports and are accordingly 'selected-out' of participation in such events. We report the case of an ultra-endurance athlete with 25 years of > 50 km competitive running experience, with genetically confirmed HCM; thereby demonstrating that these can be two compatible entities.

  3. Hypertrophic cardiomyopathy and ultra-endurance running - two incompatible entities?

    Science.gov (United States)

    Wilson, Mathew G; Chandra, Navin; Papadakis, Michael; O'Hanlon, Rory; Prasad, Sanjay K; Sharma, Sanjay

    2011-11-29

    Regular and prolonged exercise is associated with increased left ventricular wall thickness that can overlap with hypertrophic cardiomyopathy (HCM). Differentiating physiological from pathological hypertrophy has important implications, since HCM is the commonest cause of exercise-related sudden cardiac death in young individuals. Most deaths have been reported in intermittent 'start-stop' sports such as football (soccer) and basketball. The theory is that individuals with HCM are unable to augment stroke volume sufficiently to meet the demands of endurance sports and are accordingly 'selected-out' of participation in such events. We report the case of an ultra-endurance athlete with 25 years of > 50 km competitive running experience, with genetically confirmed HCM; thereby demonstrating that these can be two compatible entities.

  4. Determination of multidirectional myocardial deformations in cats with hypertrophic cardiomyopathy by using two-dimensional speckle-tracking echocardiography.

    Science.gov (United States)

    Suzuki, Ryohei; Mochizuki, Yohei; Yoshimatsu, Hiroki; Teshima, Takahiro; Matsumoto, Hirotaka; Koyama, Hidekazu

    2017-12-01

    Objectives Hypertrophic cardiomyopathy, a primary disorder of the myocardium, is the most common cardiac disease in cats. However, determination of myocardial deformation with two-dimensional speckle-tracking echocardiography in cats with various stages of hypertrophic cardiomyopathy has not yet been reported. This study was designed to measure quantitatively multidirectional myocardial deformations of cats with hypertrophic cardiomyopathy. Methods Thirty-two client-owned cats with hypertrophic cardiomyopathy and 14 healthy cats serving as controls were enrolled and underwent assessment of myocardial deformation (peak systolic strain and strain rate) in the longitudinal, radial and circumferential directions. Results Longitudinal and radial deformations were reduced in cats with hypertrophic cardiomyopathy, despite normal systolic function determined by conventional echocardiography. Cats with severely symptomatic hypertrophic cardiomyopathy also had lower peak systolic circumferential strain, in addition to longitudinal and radial strain. Conclusions and relevance Longitudinal and radial deformation may be helpful in the diagnosis of hypertrophic cardiomyopathy. Additionally, the lower circumferential deformation in cats with severe hypertrophic cardiomyopathy may contribute to clinical findings of decompensation, and seems to be related to severe cardiac clinical signs. Indices of multidirectional myocardial deformations by two-dimensional speckle-tracking echocardiography may be useful markers and help to distinguish between cats with hypertrophic cardiomyopathy and healthy cats. Additionally, they may provide more detailed assessment of contractile function in cats with hypertrophic cardiomyopathy.

  5. Current Therapeutic Approach to Hypertrophic Scar/span>s

    Science.gov (United States)

    Mokos, Zrinka Bukvić; Jović, Anamaria; Grgurević, Lovorka; Dumić-Čule, Ivo; Kostović, Krešimir; Čeović, Romana; Marinović, Branka

    2017-01-01

    Abnormal scarring and its accompanying esthetic, functional, and psychological sequelae still pose significant challe nges. To date, there is no satisfactory prevention or treatment option for hypertrophic scar/span>s (HSs), which is mostly due to not completely comprehending the mechanisms underlying their formation. That is why the apprehension of regular and controlled physiological processes of scar formation is of utmost importance when facing hypertrophic scarring, its pathophysiology, prevention, and therapeutic approach. When treating HSs and choosing the best treatment and prevention modality, physicians can choose from a plethora of therapeutic options and many commercially available products, among which currently there is no efficient option that can successfully overcome impaired skin healing. This article reviews current therapeutic approach and emerging therapeutic strategies for the management of HSs, which should be individualized, based on an evaluation of the scar itself, patients’ expectations, and practical, evidence-based guidelines. Clinicians are encouraged to combine various prevention and treatment modalities where combination therapy that includes steroid injections, 5-fluorouracil, and pulsed-dye laser seems to be the most effective. On the other hand, the current therapeutic options are usually empirical and their results are unreliable and unpredictable. Therefore, there is an unmet need for an effective, targeted therapy and prevention, which would be based on an action or a modulation of a particular factor with clarified mechanism of action that has a beneficial effect on wound healing. As the extracellular matrix has a crucial role in cellular and extracellular events that lead to pathological scarring, targeting its components mostly by regulating bone morphogenetic proteins may throw up new therapeutic approach for reduction or prevention of HSs with functionally and cosmetically acceptable outcome. PMID:28676850

  6. Decreased coronary flow reserve in hypertrophic cardiomyopathy is related to remodeling of the coronary microcirculation

    NARCIS (Netherlands)

    M.J.M. Kofflard (Marcel); D.J.G.M. Duncker (Dirk); C. von Birgelen (Clemens); S.G. Carlier (Stephan); M. Kliffen (Mike); P.W.J.C. Serruys (Patrick); R. Krams (Rob); F.J. ten Cate (Folkert)

    1998-01-01

    textabstractBACKGROUND: Ischemia occurs frequently in hypertrophic cardiomyopathy (HCM) without evidence of epicardial stenosis. This study evaluates the hypothesis that the occurrence of ischemia in HCM is related to remodeling of the coronary microcirculation. METHODS AND

  7. Keloids and Hypertrophic Scars: Update and Future Directions

    National Research Council Canada - National Science Library

    Huang, Chenyu; Murphy, George F; Akaishi, Satoshi; Ogawa, Rei

    2013-01-01

    The development of cutaneous pathological scars, namely, hypertrophic scars (HSs) and keloids, involves complex pathways, and the exact mechanisms by which they are initiated, evolved, and regulated remain to be fully elucidated...

  8. Nd:YAG Laser Treatment of Keloids and Hypertrophic Scars

    National Research Council Canada - National Science Library

    Akaishi, Satoshi; Koike, Sachiko; Dohi, Teruyuki; Kobe, Kyoko; Hyakusoku, Hiko; Ogawa, Rei

    2012-01-01

    .... Our group has been using long-pulsed, 1064-nm Nd:YAG laser in noncontact mode with low fluence and a submillisecond pulse duration to treat keloids and hypertrophic scars since 2006 with satisfactory results...

  9. Medicinal Plants for the Treatment of Hypertrophic Scars

    National Research Council Canada - National Science Library

    Ye, Qi; Wang, Su-Juan; Chen, Jian-Yu; Rahman, Khalid; Xin, Hai-Liang; Zhang, Hong

    2015-01-01

    .... Although a large amount of scientific research has been reported on the use of medicinal plants as a natural source of treatment for hypertrophic scarring, it is currently scattered across a wide range of publications...

  10. Burden of Recurrent and Ancestral Mutations in Families With Hypertrophic Cardiomyopathy

    NARCIS (Netherlands)

    Ross, Samantha Barratt; Bagnall, Richard D.; Ingles, Jodie; van Tintelen, J. Peter; Semsarian, Christopher

    2017-01-01

    Hypertrophic cardiomyopathy is a genetically heterogeneous myocardial disease with >1000 causal variants identified. Nonunique variants account for disease in many families. We sought to characterize nonunique variants in Australian families and determine whether they arise from common ancestral

  11. Investigating the potential of Shikonin as a novel hypertrophic scar treatment

    National Research Council Canada - National Science Library

    Fan, Chen; Xie, Yan; Dong, Ying; Su, Yonghua; Upton, Zee

    2015-01-01

    Hypertrophic scarring is a highly prevalent condition clinically and results from a decreased number of apoptotic fibroblasts and over-abundant production of collagen during scar formation following wound healing...

  12. Hypertrophic cardiomyopathy family with double-heterozygous mutations; does disease severity suggest doubleheterozygosity?

    OpenAIRE

    van Rijsingen, I.A.W.; Hermans-van Ast, J.F.; Arens, Y.H.J.M.; Schalla, S.M.; de Die-Smulders, C.E.M.; van den Wijngaard, A.; Pinto, Y.M.

    2009-01-01

    Background. With the improvement in genetic testing over time, double-heterozygous mutations are more often found by coincidence in families with hypertrophic cardiomyopathy (HCM). Double heterozygosity can be a cause of the wellknown clinical diversity within HCM families.

  13. Granulomatosis With Polyangiitis-Associated Hypertrophic Pachymeningitis Mimicking Spontaneous Intracranial Hypotension: A Case Report.

    Science.gov (United States)

    Jung, Young Hee; Lee, Mi Ji; Lee, Chungbin; Cha, Jihoon; Chung, Chin-Sang

    2017-03-01

    Dural enhancement is a characteristic finding in both spontaneous intracranial hypotension and hypertrophic pachymeningitis. Positional headache is the most important feature that distinguishes the two diseases. We report a patient with granulomatosis with polyangiitis (formerly Wegener's granulomatosis) who initially manifested like spontaneous intracranial hypotension. We report here the case of a 63-year old man who presented with severe positional headache. The patient had typical symptoms, symmetric dural enhancement, and a recent history of nontraumatic subdural hygroma which led to the diagnosis of spontaneous intracranial hypotension, but was finally diagnosed as granulomatosis with polyangiitis-associated secondary hypertrophic pachymeningitis. Cyclophosphamide therapy was effective for the maintenance of remission. Hypertrophic pachymeningitis associated with granulomatosis with polyangiitis can present with positional headache and subdural hygroma, mimicking spontaneous intracranial hypotension. Granulomatosis with polyangiitis should be suspected when patients with spontaneous intracranial hypotension or hypertrophic pachymeningitis show atypical features. © 2016 American Headache Society.

  14. Echocardiographic evaluation of pre-diagnostic development in young relatives genetically predisposed to hypertrophic cardiomyopathy

    DEFF Research Database (Denmark)

    Jensen, Morten K; Havndrup, Ole; Christiansen, Michael

    2015-01-01

    Identification of the first echocardiographic manifestations of hypertrophic cardiomyopathy may be important for clinical management and our understanding of the pathogenesis. We studied the development of pre-diagnostic echocardiographic changes in young relatives to HCM patients during long...

  15. Angiotensin II type 2 receptors and cardiac hypertrophy in women with hypertrophic cardiomyopathy

    NARCIS (Netherlands)

    J. Deinum (Jacob); J.M. van Gool (Jeanette); M.J.M. Kofflard (Marcel); A.H.J. Danser (Jan); F.J. ten Cate (Folkert)

    2001-01-01

    textabstractThe development of left ventricular hypertrophy in subjects with hypertrophic cardiomyopathy (HCM) is variable, suggesting a role for modifying factors such as angiotensin II. Angiotensin II mediates both trophic and antitrophic effects, via angiotensin II type 1

  16. The role of autologous bone graft in surgical treatment of hypertrophic nonunion of midshaft clavicle fractures

    Directory of Open Access Journals (Sweden)

    Hui-Kuang Huang

    2012-05-01

    Conclusion: LC-DCP fixation is an effective method for treating hypertrophic nonunion of mid-shaft clavicle fracture. Local bone graft is sufficient to achieve necessary union, and autologous bone graft from other sites of the body appears unnecessary.

  17. Extent of hypertrophy in hypertrophic cardiomyopathy: two-dimensional echocardiographic and angiographic correlation

    NARCIS (Netherlands)

    S. Domenicucci; S.K. Das (Swadesh); P.W.J.C. Serruys (Patrick); W.B. Vletter (Wim); J.R.T.C. Roelandt (Jos); F.J. ten Cate (Folkert)

    1983-01-01

    textabstractSince the first anatomical and functional descriptions of hypertrophic cardiomyopathy (HCM) there have been convincing attempts at better understanding and definition of the controversial aspects of this complex disease.

  18. Impaired cardiac mitochondrial oxidative phosphorylation and enhanced mitochondrial oxidative stress in feline hypertrophic cardiomyopathy

    DEFF Research Database (Denmark)

    Christiansen, Liselotte Bruun; Dela, Flemming; Koch, Jørgen

    2015-01-01

    Mitochondrial dysfunction and oxidative stress are important players in the development of various cardiovascular diseases, but their roles in hypertrophic cardiomyopathy (HCM) remain unknown. We examined whether mitochondrial oxidative phosphorylation (OXPHOS) capacity was impaired with enhanced...

  19. ACE I/D polymorphism in Indian patients with hypertrophic cardiomyopathy and dilated cardiomyopathy

    DEFF Research Database (Denmark)

    Rai, Taranjit Singh; Dhandapany, Perundurai Subramaniam; Ahluwalia, Tarun Veer Singh

    2008-01-01

    The study was carried to determine the association of angiotensin converting enzyme (ACE) insertion/deletion (I/D) polymorphism with the risk of hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), and restrictive cardiomyopathy (RCM).......The study was carried to determine the association of angiotensin converting enzyme (ACE) insertion/deletion (I/D) polymorphism with the risk of hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), and restrictive cardiomyopathy (RCM)....

  20. Leopard syndrome and hypertrophic cardiomyopathy: an association related to sudden death.

    Science.gov (United States)

    Antunes, Murillo de Oliveira; Arteaga, Edmundo; Matsumoto, Afonso Yoshikiro; Ianni, Barbara Maria

    2009-06-01

    We describe an uncommon association between Leopard syndrome and hypertrophic cardiomyopathy in a 27-year-old woman, who was little symptomatic and came for sudden death risk stratification and prevention. She has a rare syndrome, whose symptoms are maculae over the body and abnormalities in eyes, genital organs, heart and in growth. Association of hypertrophic cardiomyopathy with sudden death risk factors determined the implantation of cardioverter-defibrillator (ICD) for primary prevention.

  1. Comparison Between Clinical and Echocardiographic Findings in Infants and Children Diagnosed with Hypertrophic Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Cristina Blesneac

    2015-06-01

    Full Text Available Background: Hypertrophic cardiomyopathy is a rather common hereditary disease with an autozomal dominant character, caused by mutations of genes that code for proteins of the cardiac sarcomere. The observed prevalence of this disease is much lower in pediatric patients compared to adults, because it’s late gene expression. Hypertrophic cardiomyopathy presenting in infancy has been shown to have a very high mortality.

  2. The Comparision of Left Atrial Functions Between Hypertrophic Cardiomyopathy and Hypertension

    Directory of Open Access Journals (Sweden)

    Ahmet Akdi

    2016-12-01

    Full Text Available INTRODUCTION: The presence of left ventricule hypertrophy (LVH due to arterial hypertension may impair atrial function. Also, hypertrophic cardiomyopathy (HCM represents a generalized myopathic process affecting both ventricular and atrial myocardium. In this study we aimed to evaluate left atrial volumes, phasic functions in hypertrophic cardiomyopathy and hypertensive heart disease. METHODS: The study consisted of 42 patients (25 men, 17 women; mean age 51.2 years with hypertrophic cardiomyopathy (n = 20 or hypertensive heart disease (n = 22. Two-dimensional echocardiographic left atrial volumes (maximal, minimal and pre-contraction volumes were obtained and left atrial phasic functions (reservoir, conduit, and pumping functions were calculated. The findings were compared with those of age- and sex-matched 20 controls (9 men, 11 women; mean age 44.2 years without structural heart disease. RESULTS: Left atrial volumes were found to be significantly increased in patients with hypertrophic cardiomyopathy and hypertensive heart disease compared with control groups. Left atrial reservoir and conduit functions were significantly lower in patients with hypertrophic cardiomyopathy than in those with hypertensive heart disease and control groups (p < 0.01 and p < 0.01, respectively. There was no significant difference of left atrial pumping functions between study groups (p = 0.2. DISCUSSION AND CONCLUSION: This study showed that left atrial minimal, maximal and pre-contraction volume indexes are increased in hypertensive and hypertrophic cardiomyopathy groups compared with control group, but there was no significant difference between hypertrophic cardiomyopathy and hypertensive patients. In hypertrophic cardiomyopathy group left atrial phasic functions, including conduit, reservoir and pump, were decreased significantly more than hypertensive and control group. In hypertensive patients left atrial phasic functions were decreased but compared with

  3. SFRP2 and Slug Contribute to Cellular Resistance to Apoptosis in Hypertrophic Scars

    OpenAIRE

    Liang Chen; Zhenxiang Wang; Shirong Li; Guangjian Zhao; Maosheng Tian; Zhicheng Sun

    2012-01-01

    Hypertrophic scars (HS) are skin disorders which occur after wounding and thermal injury. Our previous studies have suggested that secreted frizzled-related protein 2 (SFRP2) is involved in HS formation and that the suppression of SFRP2 promotes apoptosis of hypertrophic scar fibroblasts (HSFBs). However, the mechanisms have not been clarified. Previous studies revealed that Slug expression inhibits cell apoptosis, in vitro and in vivo, and SFRP2 regulates the expression of Slug in cervical c...

  4. Frontotemporal lobar degeneration: current perspectives

    Directory of Open Access Journals (Sweden)

    Riedl L

    2014-02-01

    Full Text Available Lina Riedl,1 Ian R Mackenzie,2 Hans Förstl,1 Alexander Kurz,1 Janine Diehl-Schmid1 1Center for Cognitive Disorders, Department of Psychiatry and Psychotherapy, Klinikum rechts der Isar, Technische Universität München, Munich, Germany; 2Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, Canada Abstract: The term frontotemporal lobar degeneration (FTLD refers to a group of progressive brain diseases, which preferentially involve the frontal and temporal lobes. Depending on the primary site of atrophy, the clinical manifestation is dominated by behavior alterations or impairment of language. The onset of symptoms usually occurs before the age of 60 years, and the mean survival from diagnosis varies between 3 and 10 years. The prevalence is estimated at 15 per 100,000 in the population aged between 45 and 65 years, which is similar to the prevalence of Alzheimer's disease in this age group. There are two major clinical subtypes, behavioral-variant frontotemporal dementia and primary progressive aphasia. The neuropathology underlying the clinical syndromes is also heterogeneous. A common feature is the accumulation of certain neuronal proteins. Of these, the microtubule-associated protein tau (MAPT, the transactive response DNA-binding protein, and the fused in sarcoma protein are most important. Approximately 10% to 30% of FTLD shows an autosomal dominant pattern of inheritance, with mutations in the genes for MAPT, progranulin (GRN, and in the chromosome 9 open reading frame 72 (C9orf72 accounting for more than 80% of familial cases. Although significant advances have been made in recent years regarding diagnostic criteria, clinical assessment instruments, neuropsychological tests, cerebrospinal fluid biomarkers, and brain imaging techniques, the clinical diagnosis remains a challenge. To date, there is no specific pharmacological treatment for FTLD. Some evidence has been provided for serotonin reuptake

  5. Particles and Strings in Degenerate Metric Spaces

    OpenAIRE

    Cabral, Luis A.; Rivelles, Victor O.

    1999-01-01

    We consider relativistic and non-relativistic particles and strings in spaces (or space-times) with a degenerate metric. We show that the resulting dynamics is described by a rich structure of constraints. All constraints are classified and the dynamics depends strongly on the parity of the difference between the dimension of the space (or space-time) and the rank of the degenerate metric. For a particular class of degenerate metrics we can identify the null eigenvectors of the metric with it...

  6. A Monte Carlo algorithm for degenerate plasmas

    Energy Technology Data Exchange (ETDEWEB)

    Turrell, A.E., E-mail: a.turrell09@imperial.ac.uk; Sherlock, M.; Rose, S.J.

    2013-09-15

    A procedure for performing Monte Carlo calculations of plasmas with an arbitrary level of degeneracy is outlined. It has possible applications in inertial confinement fusion and astrophysics. Degenerate particles are initialised according to the Fermi–Dirac distribution function, and scattering is via a Pauli blocked binary collision approximation. The algorithm is tested against degenerate electron–ion equilibration, and the degenerate resistivity transport coefficient from unmagnetised first order transport theory. The code is applied to the cold fuel shell and alpha particle equilibration problem of inertial confinement fusion.

  7. Characterizing degenerate Sturm-Liouville problems

    Directory of Open Access Journals (Sweden)

    Angelo B. Mingarelli

    2004-11-01

    Full Text Available Consider the Dirichlet eigenvalue problem associated with the real two-term weighted Sturm-Liouville equation $$-(p(xy'' = lambda r(xy$$ on the finite interval [a,b]. This eigenvalue problem will be called degenerate provided its spectrum fills the whole complex plane. Generally, in degenerate cases the coefficients $p(x, r(x$ must each be sign indefinite on [a,b]. Indeed, except in some special cases, the quadratic forms induced by them on appropriate spaces must also be indefinite. In this note we present a necessary and sufficient condition for this boundary problem to be degenerate. Some extensions are noted.

  8. [Gene mutation and clinical phenotype analysis of patients with Noonan syndrome and hypertrophic cardiomyopathy].

    Science.gov (United States)

    Liu, X H; Ding, W W; Han, L; Liu, X R; Xiao, Y Y; Yang, J; Mo, Y

    2017-10-02

    Objective: To analyze the gene mutations and clinical features of patients with Noonan syndrome and hypertrophic cardiomyopathy. Method: Determined the mutation domain in five cases diagnosed with Noonan syndrome and hypertrophic cardiomyopathy and identified the relationship between the mutant domain and hypertrophic cardiomyopathy by searching relevant articles in pubmed database. Result: Three mutant genes (PTPN11 gene in chromosome 12, RIT1 gene in chromosome 1 and RAF1 gene in chromosome 3) in five cases all had been reported to be related to hypertrophic cardiomyopathy. The reported hypertrophic cardiomyopathy relevant genes MYPN, MYH6 and MYBP3 had also been found in case 1 and 2. Patients with same gene mutation had different clinical manifestations. Both case 4 and 5 had RAF1 mutation (c.770C>T). However, case 4 had special face, low IQ, mild pulmonary artery stenosis, and only mild ventricular hypertrophy. Conclusion: Noonan syndrome is a genetic heterogeneity disease. Our study identified specific gene mutations that could result in Noonan syndrome with hypertrophic cardiomyopathy through molecular biology methods. The results emphasize the importance of gene detection in the management of Noonan syndrome.

  9. Hypertrophic cardiomyopathy: prognostic factors and survival analysis in 128 Egyptian patients.

    Science.gov (United States)

    El-Saiedi, Sonia A; Seliem, Zeinab S; Esmail, Reem I

    2014-08-01

    Hypertrophic cardiomyopathy is an important cause of disability and death in patients of all ages. Egyptian children may differ from Western and Asian patients in the pattern of hypertrophy distribution, clinical manifestations, and risk factors. The aim of our study was to report the clinical characteristics and outcomes of Egyptian children with hypertrophic cardiomyopathy studied over a 7-year duration and to determine whether the reported adult risk factors for sudden cardiac death are predictive of the outcome in these affected children. This retrospective study included 128 hypertrophic cardiomyopathy children. The data included personal history, family history, physical examination, baseline laboratory measurements, electrocardiogram, and Holter and echocardiographic results. Logistic regression analysis was used for the detection of risk factors of death. Fifty-one out of 128 patients died during the period of the study. Of the 51 deaths, 36 (70.5%) occurred in patients presenting before 1 year of age. Only eight patients had surgical intervention. Extreme left ventricular hypertrophy, that is, interventricular septal wall thickness or posterior wall thickness Z-score >6, sinus tachycardia, and supraventricular tachycardia were found to be independent risk factors for prediction of death in patients with hypertrophic cardiomyopathy. At our Egyptian tertiary care centre, hypertrophic cardiomyopathy has a relatively worse prognosis when compared with reports from Western and Asian series. Infants have a worse outcome than children presenting after the age of 1 year. A poorer prognosis in childhood hypertrophic cardiomyopathy is predicted by an extreme left ventricular hypertrophy, the presence of sinus tachycardia, and supraventricular tachycardia.

  10. Degenerate coding in neural systems.

    Science.gov (United States)

    Leonardo, Anthony

    2005-11-01

    When the dimensionality of a neural circuit is substantially larger than the dimensionality of the variable it encodes, many different degenerate network states can produce the same output. In this review I will discuss three different neural systems that are linked by this theme. The pyloric network of the lobster, the song control system of the zebra finch, and the odor encoding system of the locust, while different in design, all contain degeneracies between their internal parameters and the outputs they encode. Indeed, although the dynamics of song generation and odor identification are quite different, computationally, odor recognition can be thought of as running the song generation circuitry backwards. In both of these systems, degeneracy plays a vital role in mapping a sparse neural representation devoid of correlations onto external stimuli (odors or song structure) that are strongly correlated. I argue that degeneracy between input and output states is an inherent feature of many neural systems, which can be exploited as a fault-tolerant method of reliably learning, generating, and discriminating closely related patterns.

  11. Very Degenerate Higgsino Dark Matter

    Energy Technology Data Exchange (ETDEWEB)

    Chun, Eung Jin [Korea Institute for Advanced Study,Seoul 130-722 (Korea, Republic of); Jung, Sunghoon [SLAC National Accelerator Laboratory,Menlo Park, CA 94025 (United States); Kavli Institute for Theoretical Physics,Santa Barbara, CA 93106 (United States); Park, Jong-Chul [Department of Physics, Chungnam National University,Daejeon 34134 (Korea, Republic of)

    2017-01-03

    We present a study of the Very Degenerate Higgsino Dark Matter (DM), whose mass splitting between the lightest neutral and charged components is O(1) MeV, much smaller than radiative splitting of 355 MeV. The scenario is realized in the minimal supersymmetric standard model by small gaugino mixings. In contrast to the pure Higgsino DM with the radiative splitting only, various observable signatures with distinct features are induced. First of all, the very small mass splitting makes (a) sizable Sommerfeld enhancement and Ramsauer-Townsend (RT) suppression relevant to ∼1 TeV Higgsino DM, and (b) Sommerfeld-Ramsauer-Townsend effect saturate at lower velocities v/c≲10{sup −3}. As a result, annihilation signals can be large enough to be observed from the galactic center and/or dwarf galaxies, while the relative signal sizes can vary depending on the locations of Sommerfeld peaks and RT dips. In addition, at collider experiments, stable chargino signatures can be searched for to probe the model in the future. DM direct detection signals, however, depend on the Wino mass; even no detectable signals can be induced if the Wino is heavier than about 10 TeV.

  12. Age-related macular degeneration

    DEFF Research Database (Denmark)

    la Cour, Morten; Kiilgaard, Jens Folke; Nissen, Mogens Holst

    2002-01-01

    Age-related macular degeneration (AMD) is a common macular disease affecting elderly people in the Western world. It is characterised by the appearance of drusen in the macula, accompanied by choroidal neovascularisation (CNV) or geographic atrophy. The disease is more common in Caucasian individ......, photodynamic therapy with verteporfin is the treatment of choice. Photodynamic therapy is also effective in eyes with pure occult CNV and evidence of recent disease progression. For new subfoveal CNV with poor vision and recurrent CNV, laser photocoagulation can be considered....... individuals than in pigmented races. In predominantly Caucasian populations, the age-standardised prevalence of AMD in at least one eye is 7760 cases per million. The age-standardised cumulated 1-year incidence of AMD in at least one eye is 1051 cases per million individuals. AMD is the most important single...... (exudative cases); the remainder has only geographic atrophy. In cross-sectional population-based studies about 45% of eyes with AMD have visual acuity reduced to 20/200 or worse. This is true both for exudative AMD and pure geographic atrophy. Age and genetic predisposition are known risk factors for AMD...

  13. Magnetic resonance imaging of intervertebral disc degeneration

    Energy Technology Data Exchange (ETDEWEB)

    Maeda, Hiroshi; Noguchi, Masao (Kitakyushu City Yahata Hospital, Fukuoka (Japan)); Kira, Hideaki; Fujiki, Hiroshi; Shimokawa, Isao; Hinoue, Kaichi

    1993-02-01

    The aim of this study was to correlate the degree of lumbar intervertebral disc degeneration with findings of magnetic resonance imaging (MRI). Seventeen autopsied (from 7 patients) and 21 surgical (from 20 patients) intervertebral discs were used as specimens for histopathological examination. In addition, 21 intervertebral discs were examined on T2-weighted images. Histopathological findings from both autopsied and surgical specimens were well correlated with MRI findings. In particular, T2-weighted images reflected increased collagen fibers and rupture within the fibrous ring accurately. However, when severely degenerated intervertebral discs and hernia protruding the posterior longitudinal ligament existed, histological findings were not concordant well with T2-weighted images. Morphological appearances of autopsy specimens, divided into four on T2-weighted images, were well consistent with histological degeneration. This morphological classification, as shown on T2-weighted images, could also be used in the evaluation of intervertebral disc degeneration. (N.K.).

  14. Splitting deformations of degenerations of complex curves towards the classification of atoms of degenerations

    CERN Document Server

    2006-01-01

    The author develops a deformation theory for degenerations of complex curves; specifically, he treats deformations which induce splittings of the singular fiber of a degeneration. He constructs a deformation of the degeneration in such a way that a subdivisor is "barked" (peeled) off from the singular fiber. These "barking deformations" are related to deformations of surface singularities (in particular, cyclic quotient singularities) as well as the mapping class groups of Riemann surfaces (complex curves) via monodromies. Important applications, such as the classification of atomic degenerations, are also explained.

  15. Neural remodeling in retinal degeneration.

    Science.gov (United States)

    Marc, Robert E; Jones, Bryan W; Watt, Carl B; Strettoi, Enrica

    2003-09-01

    Mammalian retinal degenerations initiated by gene defects in rods, cones or the retinal pigmented epithelium (RPE) often trigger loss of the sensory retina, effectively leaving the neural retina deafferented. The neural retina responds to this challenge by remodeling, first by subtle changes in neuronal structure and later by large-scale reorganization. Retinal degenerations in the mammalian retina generally progress through three phases. Phase 1 initiates with expression of a primary insult, followed by phase 2 photoreceptor death that ablates the sensory retina via initial photoreceptor stress, phenotype deconstruction, irreversible stress and cell death, including bystander effects or loss of trophic support. The loss of cones heralds phase 3: a protracted period of global remodeling of the remnant neural retina. Remodeling resembles the responses of many CNS assemblies to deafferentation or trauma, and includes neuronal cell death, neuronal and glial migration, elaboration of new neurites and synapses, rewiring of retinal circuits, glial hypertrophy and the evolution of a fibrotic glial seal that isolates the remnant neural retina from the surviving RPE and choroid. In early phase 2, stressed photoreceptors sprout anomalous neurites that often reach the inner plexiform and ganglion cell layers. As death of rods and cones progresses, bipolar and horizontal cells are deafferented and retract most of their dendrites. Horizontal cells develop anomalous axonal processes and dendritic stalks that enter the inner plexiform layer. Dendrite truncation in rod bipolar cells is accompanied by revision of their macromolecular phenotype, including the loss of functioning mGluR6 transduction. After ablation of the sensory retina, Müller cells increase intermediate filament synthesis, forming a dense fibrotic layer in the remnant subretinal space. This layer invests the remnant retina and seals it from access via the choroidal route. Evidence of bipolar cell death begins in

  16. MicroRNA-21 regulates hTERT via PTEN in hypertrophic scar fibroblasts.

    Directory of Open Access Journals (Sweden)

    Hua-Yu Zhu

    Full Text Available As an important oncogenic miRNA, microRNA-21 (miR-21 is associated with various malignant diseases. However, the precise biological function of miR-21 and its molecular mechanism in hypertrophic scar fibroblast cells has not been fully elucidated.Quantitative Real-Time PCR (qRT-PCR analysis revealed significant upregulation of miR-21 in hypertrophic scar fibroblast cells compared with that in normal skin fibroblast cells. The effects of miR-21 were then assessed in MTT and apoptosis assays through in vitro transfection with a miR-21 mimic or inhibitor. Next, PTEN (phosphatase and tensin homologue deleted on chromosome ten was identified as a target gene of miR-21 in hypertrophic scar fibroblast cells. Furthermore, Western-blot and qRT-PCR analyses revealed that miR-21 increased the expression of human telomerase reverse transcriptase (hTERT via the PTEN/PI3K/AKT pathway. Introduction of PTEN cDNA led to a remarkable depletion of hTERT and PI3K/AKT at the protein level as well as inhibition of miR-21-induced proliferation. In addition, Western-blot and qRT-PCR analyses confirmed that hTERT was the downstream target of PTEN. Finally, miR-21 and PTEN RNA expression levels in hypertrophic scar tissue samples were examined. Immunohistochemistry assays revealed an inverse correlation between PTEN and hTERT levels in high miR-21 RNA expressing-hypertrophic scar tissues.These data indicate that miR-21 regulates hTERT expression via the PTEN/PI3K/AKT signaling pathway by directly targeting PTEN, therefore controlling hypertrophic scar fibroblast cell growth. MiR-21 may be a potential novel molecular target for the treatment of hypertrophic scarring.

  17. Hypertrophic Osteodystrophy in Two Red Wolf (Canis rufus Pups

    Directory of Open Access Journals (Sweden)

    Jenessa L. Gjeltema

    2015-01-01

    Full Text Available A 6-month-old red wolf (Canis rufus pup presented for evaluation of progressive thoracic and pelvic limb lameness, joint swelling, and decreased body condition. Radiographic evaluation revealed medullary sclerosis centered at the metaphyses of multiple long bones, well-defined irregular periosteal proliferation, and ill-defined lucent zones paralleling the physes, consistent with hypertrophic osteodystrophy (HOD. Biopsies of affected bone revealed medullary fibrosis and new bone formation. The pup improved following treatment with nonsteroidal anti-inflammatories, opioids, and supportive care over the course of 4 weeks. Metaphyseal periosteal bone proliferation persisted until the animal was humanely euthanized several years later for poor quality of life associated with bilateral cranial cruciate ligament rupture. A second red wolf pup of 4.5 months of age presented for evaluation of lethargy, kyphotic posture, and swollen carpal and tarsal joints. Radiographs revealed bilateral medullary sclerosis and smooth periosteal reaction affecting multiple long bones, suggestive of HOD. Further diagnostics were not pursued in this case to confirm the diagnosis, and the clinical signs persisted for 4 weeks. In light of these two case reports, HOD should be recognized as a developmental orthopedic disease in growing red wolves.

  18. The Signaling Pathways Involved in Chondrocyte Differentiation and Hypertrophic Differentiation

    Directory of Open Access Journals (Sweden)

    Jianmei Li

    2016-01-01

    Full Text Available Chondrocytes communicate with each other mainly via diffusible signals rather than direct cell-to-cell contact. The chondrogenic differentiation of mesenchymal stem cells (MSCs is well regulated by the interactions of varieties of growth factors, cytokines, and signaling molecules. A number of critical signaling molecules have been identified to regulate the differentiation of chondrocyte from mesenchymal progenitor cells to their terminal maturation of hypertrophic chondrocytes, including bone morphogenetic proteins (BMPs, SRY-related high-mobility group-box gene 9 (Sox9, parathyroid hormone-related peptide (PTHrP, Indian hedgehog (Ihh, fibroblast growth factor receptor 3 (FGFR3, and β-catenin. Except for these molecules, other factors such as adenosine, O2 tension, and reactive oxygen species (ROS also have a vital role in cartilage formation and chondrocyte maturation. Here, we outlined the complex transcriptional network and the function of key factors in this network that determine and regulate the genetic program of chondrogenesis and chondrocyte differentiation.

  19. MRI diagnosis of hypertrophic osteoarthropathy from a remote childhood malignancy

    Energy Technology Data Exchange (ETDEWEB)

    Sainani, Nisha I.; Lawande, Malini A.; Pungavkar, Sona A.; Patkar, Deepak P.; Sase, Kirti S. [Dr. Balabhai Nanavati Hospital and Research Centre, Department of MRI, Mammography and BMD, Mumbai (India); Parikh, Vipul P. [Dr. Balabhai Nanavati Hospital and Research Centre, Department of CT and USG, Mumbai (India)

    2007-06-15

    Hypertrophic osteoarthropathy (HOA) is a clinico-radiological syndrome characterized by digital clubbing, periosteal proliferation, bone pain, synovitis and arthralgia, all of these being commonly symmetrical. It is occasionally associated with nasopharyngeal lymphoepitheliomas and may develop before or after development of lung metastases in these patients. We report a case of a healthy 22-year-old female who presented to our institution with pain and swelling in the thighs and legs. She had a history of childhood nasopharyngeal lymphoepithelioma. Radiographs of the knees were negative. Magnetic resonance imaging (MRI) showed features suggestive of bilateral periostitis. Because of the propensity of the rare childhood nasopharyngeal lymphoepithelioma to present with HOA, this entity was included in the differential diagnosis. A subsequent chest radiograph and CT demonstrated a lung and mediastinal mass that were histologically confirmed to be metastatic. To the best of our knowledge, HOA and metastases from nasopharyngeal lymphoepithelioma occurring after such a long time interval have not been previously reported. Early demonstration and consideration of HOA on the basis of MRI, lead to expeditious and appropriate subsequent investigation. (orig.)

  20. Keloids and Hypertrophic Scars: Update and Future Directions

    Directory of Open Access Journals (Sweden)

    Chenyu Huang, MD, PhD

    2013-07-01

    Full Text Available Summary: The development of cutaneous pathological scars, namely, hypertrophic scars (HSs and keloids, involves complex pathways, and the exact mechanisms by which they are initiated, evolved, and regulated remain to be fully elucidated. The generally held concepts that keloids and HSs represent “aberrant wound healing” or that they are “characterized by hyalinized collagen bundles” have done little to promote their accurate clinicopathological classification or to stimulate research into the specific causes of these scars and effective preventative therapies. To overcome this barrier, we review here the most recent findings regarding the pathology and pathogenesis of keloids and HSs. The aberrations of HSs and keloids in terms of the inflammation, proliferation, and remodeling phases of the wound healing process are described. In particular, the significant roles that the extracellular matrix and the epidermal and dermal layers of skin play in scar pathogenesis are examined. Finally, the current hypotheses of pathological scar etiology that should be tested by basic and clinical investigators are detailed. Therapies that have been found to be effective are described, including several that evolved directly from the aforementioned etiology hypotheses. A better understanding of pathological scar etiology and manifestations will improve the clinical and histopathological classification and treatment of these important lesions.

  1. Surgeon as educator: bedside ultrasound in hypertrophic pyloric stenosis.

    Science.gov (United States)

    Wyrick, Deidre L; Smith, Samuel D; Dassinger, Melvin S

    2014-01-01

    Our institution has demonstrated the diagnostic accuracy of surgeon-performed ultrasound (US) in the diagnosis of hypertrophic pyloric stenosis (HPS). Moreover, we have also shown this modality to be accurate and reproducible through surgeon-to-surgeon instruction. The purpose of this study was to determine whether a surgical resident with experience in diagnosing HPS can teach pediatric emergency medicine (PEM) fellows, with little experience in sonography, to accurately measure the pyloric channel with bedside US. A surgical resident with experience in diagnosing HPS with US-proctored 4 emergency medicine fellows for 5 bedside US examinations each. A PEM fellow, who was blinded to the results from the radiology department US, then performed bedside US and measured the pyloric channel in patients presenting to the emergency department with HPS. Results between the radiology department and the fellows were compared using the Student t test. In total, 18 USs were performed on 17 patients. There were no false-negative or false-positive results. There was no statistical difference between the radiology department and fellow measurement when evaluating muscle width (p = 0.21, mean deviation = 0.2 mm) or channel length (p = 0.47, mean deviation = 0.6 mm). Bedside-performed US technique for measuring the pylorus length and width in patients with HPS is reproducible and accurate when taught to PEM providers. The learning curve for this technique is short. Copyright © 2014 Association of Program Directors in Surgery. Published by Elsevier Inc. All rights reserved.

  2. Determinants of myocardial energetics and efficiency in symptomatic hypertrophic cardiomyopathy.

    Science.gov (United States)

    Timmer, Stefan A J; Germans, Tjeerd; Götte, Marco J W; Rüssel, Iris K; Dijkmans, Pieter A; Lubberink, Mark; ten Berg, Jurrien M; ten Cate, Folkert J; Lammertsma, Adriaan A; Knaapen, Paul; van Rossum, Albert C

    2010-04-01

    Next to hypertrophy, hypertrophic cardiomyopathy (HCM) is characterized by alterations in myocardial energetics. A small number of studies have shown that myocardial external efficiency (MEE), defined by external work (EW) in relation to myocardial oxidative metabolism (MVO(2)), is reduced. The present study was conducted to identify determinants of MEE in patients with HCM by use of dynamic positron emission tomography (PET) and cardiovascular magnetic resonance imaging (CMR). Twenty patients with HCM (12 men, mean age: 55.2 + or - 13.9 years) and 11 healthy controls (7 men, mean age: 48.1 + or - 10 years) were studied with [(11)C]acetate PET to assess MVO(2). CMR was performed to determine left ventricular (LV) volumes and mass (LVM). Univariate and multivariate analyses were employed to determine independent predictors of myocardial efficiency. Between study groups, MVO(2) (controls: 0.12 + or - 0.04 ml x min(-1) x g(-1), HCM: 0.13 + or - 0.05 ml x min(-1) x g(-1), p = 0.64) and EW (controls: 9,139 + or - 2,484 mmHg x ml, HCM: 9,368 + or - 2,907 mmHg x ml, p = 0.83) were comparable, whereas LVM was significantly higher (controls: 99 + or - 21 g, HCM: 200 + or - 76 g, p efficiency. Mechanical external efficiency could independently be predicted by SV and LVM.

  3. Diastolic filling in a physical model of obstructive hypertrophic cardiomyopathy

    Science.gov (United States)

    Schovanec, Joseph; Samaee, Milad; Lai, Hong Kuan; Santhanakrishnan, Arvind

    2015-11-01

    Hypertrophic Cardiomyopathy (HCM) is an inherited heart disease that affects as much as one in 500 individuals, and is the most common cause of sudden death in young athletes. The myocardium becomes abnormally thick in HCM and deforms the internal geometry of the left ventricle (LV). Previous studies have shown that a vortex is formed during diastolic filling, and further that the dilated LV morphology seen in systolic heart failure results in altering the filling vortex from elliptical to spherical shape. We have also previously shown that increasing LV wall stiffness decreases the filling vortex circulation. However, alterations to intraventricular filling fluid dynamics due to an obstructive LV morphology and locally elevated wall stiffness (in the hypertrophied region) have not been previously examined from a mechanistic standpoint. We conducted an experimental study using an idealized HCM physical model and compared the intraventricular flow fields obtained from 2D PIV to a baseline LV physical model with lower wall stiffness and anatomical geometry. The obstruction in the HCM model leads to earlier breakdown of the filling vortex as compared to the anatomical LV. Intraventricular filling in both models under increased heart rates will be discussed.

  4. The diagnosis of hypertrophic cardiomyopathy by cardiovascular magnetic resonance

    Science.gov (United States)

    2012-01-01

    Hypertrophic cardiomyopathy (HCM) is the most common genetic disease of the heart. HCM is characterized by a wide range of clinical expression, ranging from asymptomatic mutation carriers to sudden cardiac death as the first manifestation of the disease. Over 1000 mutations have been identified, classically in genes encoding sarcomeric proteins. Noninvasive imaging is central to the diagnosis of HCM and cardiovascular magnetic resonance (CMR) is increasingly used to characterize morphologic, functional and tissue abnormalities associated with HCM. The purpose of this review is to provide an overview of the clinical, pathological and imaging features relevant to understanding the diagnosis of HCM. The early and overt phenotypic expression of disease that may be identified by CMR is reviewed. Diastolic dysfunction may be an early marker of the disease, present in mutation carriers prior to the development of left ventricular hypertrophy (LVH). Late gadolinium enhancement by CMR is present in approximately 60% of HCM patients with LVH and may provide novel information regarding risk stratification in HCM. It is likely that integrating genetic advances with enhanced phenotypic characterization of HCM with novel CMR techniques will importantly improve our understanding of this complex disease. PMID:22348519

  5. Flow-Induced Mitral Leaflet Motion in Hypertrophic Cardiomyopathy

    Science.gov (United States)

    Meschini, Valentina; Mittal, Rajat; Verzicco, Roberto

    2017-11-01

    Hypertrophic cardiomyopathy (HCM) is considered the cause of sudden cardiac death in developed countries. Clinically it is found to be related to the thickening of the intra-ventricular septum combined with elongated mitral leaflets. During systole the low pressure, induced by the abnormal velocities in the narrowed aortic channel, can attract one or both the mitral leaflets causing the aortic obstruction and sometimes instantaneous death. In this paper a fluid structure interaction model for the flow in the left ventricle with a native mitral valve is employed to investigate the physio-pathology of HCM. The problem is studied using direct numerical simulations of the Navier-Stokes equations with a two-way coupled structural solver based on interaction potential approach for the structure dynamics. Simulations are performed for two different degrees of hypertrophy, and two values of pumping efficiency. The leaflets dynamics and the ventricle deformation resulting from the echocardiography of patients affected by HCM are well captured by the simulations. Moreover, the procedures of leaflets plication and septum myectomy are simulated in order to get insights into the efficiency and reliability of such surgery.

  6. Hypertrophic Obstructive Cardiomyopathy: Surgical Myectomy and Septal Ablation.

    Science.gov (United States)

    Nishimura, Rick A; Seggewiss, Hubert; Schaff, Hartzell V

    2017-09-15

    Hypertrophic cardiomyopathy is a genetic disorder characterized by marked hypertrophy of the myocardium. It is frequently accompanied by dynamic left ventricular outflow tract obstruction and symptoms of dyspnea, angina, and syncope. The initial therapy for symptomatic patients with obstruction is medical therapy with β-blockers and calcium antagonists. However, there remain a subset of patients who have continued severe symptoms, which are unresponsive to medical therapy. These patients can be treated with septal reduction therapy, either surgical septal myectomy or alcohol septal ablation. When performed by experienced operators working in high-volume centers, septal myectomy is highly effective with a >90% relief of obstruction and improvement in symptoms. The perioperative mortality rate for isolated septal myectomy in most centers is <1%. Alcohol septal ablation is a less invasive treatment. In many patients, the hemodynamic and clinical results are comparable to that of septal myectomy. However, the results of alcohol septal ablation are dependent on the septal perforator artery supplying the area of the contact between the hypertrophied septum and the anterior leaflet of the mitral valve. There are some patients, particularly younger patients with severe hypertrophy, who do not uniformly experience complete relief of obstruction and symptoms. Both techniques of septal reduction therapy are highly operator dependent. The final decision as to which approach should be selected in any given patient is dependent up patient preference and the availability and experience of the operator and institution at which the patient is being treated. © 2017 American Heart Association, Inc.

  7. Hypertrophic cardiomyopathy in 2013: Current speculations and future perspectives

    Science.gov (United States)

    Efthimiadis, Georgios K; Pagourelias, Efstathios D; Gossios, Thomas; Zegkos, Thomas

    2014-01-01

    Hypertrophic cardiomyopathy (HCM), the most variable cardiac disease in terms of phenotypic presentation and clinical outcome, represents the most common inherited cardiomyopathic process with an autosomal dominant trait of inheritance. To date, more than 1400 mutations of myofilament proteins associated with the disease have been identified, most of them “private” ones. This striking allelic and locus heterogeneity of the disease certainly complicates the establishment of phenotype-genotype correlations. Additionally, topics pertaining to patients’ everyday lives, such as sudden cardiac death (SCD) risk stratification and prevention, along with disease prognosis, are grossly related to the genetic variation of HCM. This review incorporates contemporary research findings and addresses major aspects of HCM, including preclinical diagnosis, genetic analysis, left ventricular outflow tract obstruction and SCD. More specifically, the spectrum of genetic analysis, the selection of the best method for obstruction alleviation and the need for a unique and accurate factor for SCD risk stratification are only some of the controversial HCM issues discussed. Additionally, future perspectives concerning HCM and myocardial ischemia, as well as atrial fibrillation, are discussed. Rather than enumerating clinical studies and guidelines, challenging problems concerning the disease are critically appraised by this review, highlighting current speculations and recommending future directions. PMID:24575171

  8. Hypertrophic cardiomyopathy: an autopsy analysis of 14 cases.

    Directory of Open Access Journals (Sweden)

    Phadke R

    2001-07-01

    Full Text Available BACKGROUND: Hypertrophic cardiomyopathy (HCM is one of the less common forms of primary cardiomyopathies. There is little data available on HCM in Indian literature. AIMS: To assess the incidence and analyse the clinicopathological features of HCM. SETTINGS: Analysis of data of 15 years from a tertiary care centre. METHODS AND MATERIAL: The clinical and pathological data in fourteen cases of HCM with respect to their gross and microscopic features and clinical presentation were reviewed. RESULTS: Incidence of HCM amongst the autopsied primary cardiomyopathies (N = 101 was 13.9% (n=14. Males were affected more. Common presenting symptoms were exertional dyspnoea, angina and palpitations. Concentric and asymmetric hypertrophy was equally seen. Obliterative small vessel disease was noted in 50% of the cases. Although significant myofibre disarray (>5% was seen in all fourteen cases, it could be demonstrated in only 40- 50% of an average of twenty sections studied. Type IA myofibre disarray was the commonest. Six of the fourteen patients died suddenly. Cardiac failure was the commonest cause of death. CONCLUSIONS: Myofibre disarray is a highly sensitive and specific marker for HCM only when considered in a quantitative rather than a qualitative fashion. In this context, the rationale for performing endomyocardial biopsy is to rule out mimics of HCM.

  9. p21WAF1/CIP1 and 14-3-3 sigma gene expression in degenerated aortic valves: a link between cell cycle checkpoints and calcification.

    Science.gov (United States)

    Golubnitschaja, O; Yeghiazaryan, K; Skowasch, D; Schild, H; Bauriedel, G

    2006-10-01

    The mechanisms underlying aortic valve degeneration are largely unknown. Cardiac tissue responds to a variety of stimuli by hypertrophic growth. Molecular mechanisms resulting in the hypertrophic response indicate similarity and overlap with those involved in both cell growth and death. We hypothesized cell cycle control to be the key event in progression regulation of heart valve degeneration followed by tissue mineralization. Human post-operative tissue samples of native non-rheumatic stenosed aortic valves were categorized according to absence (group 1) or presence of calcification (group 2). The samples were ex vivo examined for cell density and presence of macrophage (CD68), as well as expression of two checkpoint genes, p21WAF1/CIP1 and 14-3-3 sigma, arresting the G1 and G2 cell cycle phases, respectively. Expression rates were measured by "Real-Time"-PCR on transcriptional level. Target protein expression was measured and their co-localization in different kinds of valvular cells was tested using immunohistochemical analysis. Whereas macrophages were localized predominantly in sub-endothelial layer of valvular fibrosis, p21WAF1/CIP1 and 14-3-3 sigma expression was observed also in the valvular spongiosa co-localized with alpha-actin positive cells. Significantly higher cell density and inflammation grade were observed in group 2 versus group 1. Accordingly, p21WAF1/CIP1 and 14-3-3 sigma expression was several fold higher in group 1 versus group 2 on both transcription and translation levels. The present findings on degenerated aortic valves show that increased cell density accompanied with consequent calcification might be attributed to the down-regulation of both G1 and G2 checkpoint genes.

  10. Determining pathogenicity of genetic variants in hypertrophic cardiomyopathy: importance of periodic reassessment.

    Science.gov (United States)

    Das K, Jipin; Ingles, Jodie; Bagnall, Richard D; Semsarian, Christopher

    2014-04-01

    Major advances have been made in our understanding and clinical application of genetic testing in hypertrophic cardiomyopathy. Determining pathogenicity of a single-nucleotide variant remains a major clinical challenge. This study sought to reassess single-nucleotide variant classification in hypertrophic cardiomyopathy probands. Consecutive probands with hypertrophic cardiomyopathy with a reported pathogenic mutation or variation of uncertain significance were included. Family and medical history were obtained. Each single-nucleotide variant was reassessed by a panel of four reviewers for pathogenicity based on established criteria together with updated cosegregation data and current population-based allele frequencies. From 2000 to 2012, a total of 136 unrelated hypertrophic cardiomyopathy probands had genetic testing, of which 63 (46%) carried at least one pathogenic mutation. MYBPC3 (n = 34; 47%) and MYH7 (n = 23; 32%) gene variants together accounted for 79%. Five variants in six probands (10%) were reclassified: two variation of uncertain significance were upgraded to pathogenic, one variation of uncertain significance and one pathogenic variant were downgraded to benign, and one pathogenic variant (found in two families) was downgraded to variation of uncertain significance. None of the reclassifications had any adverse clinical consequences. Given the rapid growth of genetic information available in both disease and normal populations, periodic reassessment of single-nucleotide variant data is essential in hypertrophic cardiomyopathy.

  11. Effect of hematoporphyrin monomethyl ether-sonodynamic therapy (HMME-SDT on hypertrophic scarring.

    Directory of Open Access Journals (Sweden)

    Hanjun Zhang

    Full Text Available OBJECTIVE: The aim of the present study was to explore the potential for hematoporphyrin monomethyl ether-Sonodynamic Therapy (HMME-SDT treatment of hypertrophic scars within rabbit ears. METHODS: 60 white rabbits were randomly divided into five groups: (1 untreated controls, (2 lesioned, (3 lesioned + HMME, (4 lesioned + US (Ultrasound, and (5 lesioned +HMME-SDT. After induction of a lesion upon the ears of the rabbits, hypertrophic scars were assessed at 14, 28, 42 and 56 days post-lesion +/- treatment. Assessments consisted of visual inspection in the change of the skin, scar formation pathological morphology by hematoxylin and eosin (HE staining technique with optical microscopy, calculation of a hypertrophic index, fibroblastic density measures, and observation of collagen changes in the scar tissue by Van Gieson's (VGStain along with calculation of collagen area density. RESULTS: With continued HMME-SDT treatment there was a gradual improvement in all parameters over the duration of the experiment. The lesion-induced scars of rabbits receiving HMME-SDT treatment were soft, the size was reduced, hyperplasia was flat and the color pale. The fibroblasts and collagens were reduced and the collagens were light red, sparse and orderly. The hypertrophic index was reduced, since the fibroblastic density was lowered and collagen area density was decreased. CONCLUSION: HMME is an effective sonosensitizer and the combination of HMME-SDT treatment can exert significant benefits in reducing the formation of hypertrophic scars.

  12. Cerebellar degeneration in Old English Sheepdogs.

    Science.gov (United States)

    Steinberg, H S; Van Winkle, T; Bell, J S; de Lahunta, A

    2000-10-15

    To evaluate related and unrelated Old English Sheepdogs (OESD) by clinical examination, histologic evaluation, and pedigree analysis to determine whether cerebellar degeneration develops in this breed and whether there are genetic implications. Case study and pedigree analysis. 24 clinically normal or affected OESD; brain tissue specimens from 25 unaffected or affected OESD. Twenty-four OESD that were chosen because of a family history of gait abnormalities were given physical and neurologic examinations to determine whether they had clinical signs of cerebellar degeneration. Tissue specimens from 25 brains of OESD were examined histologically. Nine OESD that were determined to have cerebellar degeneration histologically as well as 2 clinically affected littermates of the histologically confirmed affected OESD were included in the pedigree analysis. Standard statistical evaluation of pedigrees for hereditary conclusions was used. Twelve of the 24 OESD evaluated by neurologic examination had a progressive gait abnormality. Clinical signs of cerebellar degeneration typically started later in life in OESD, compared with description for other dog breeds, and progressed ore slowly. Results of pedigree analysis revealed that 11 of 49 dogs were affected in 9 litters, providing an affected-to-total ratio of 22.49%. Results of our study indicate that a slowly progressing late-onset form of cerebellar degeneration develops in OESD, and the mode of inheritance is by an autosomal recessive gene.

  13. Optic pathway degeneration in Japanese black cattle

    Science.gov (United States)

    CHIBA, Shiori; FUNATO, Shingo; HORIUCHI, Noriyuki; MATSUMOTO, Kotaro; INOKUMA, Hisashi; FURUOKA, Hidefumi; KOBAYASHI, Yoshiyasu

    2014-01-01

    Degeneration of the optic pathway has been reported in various animal species including cattle. We experienced a case of bilateral optic tract degeneration characterized by severe gliosis in a Japanese black cattle without any obvious visual defects. To evaluate the significance, pathological nature and pathogenesis of the lesions, we examined the optic pathway in 60 cattle (41 Japanese black, 13 Holstein and 6 crossbreed) with or without ocular abnormalities. None of these animals had optic canal stenosis. Degenerative changes with severe gliosis in the optic pathway, which includes the optic nerve, optic chiasm and optic tract, were only observed in 8 Japanese black cattle with or without ocular abnormalities. Furthermore, strong immunoreactivity of glial fibrillary acidic protein was observed in the retinal stratum opticum and ganglion cell layer in all 5 cattle in which the optic pathway lesions could be examined. As etiological research, we also examined whether the concentrations of vitamin A and vitamin B12 or bovine viral diarrhea virus (BVDV) infection was associated with optic pathway degeneration. However, our results suggested that the observed optic pathway degeneration was probably not caused by these factors. These facts indicate the presence of optic pathway degeneration characterized by severe gliosis that has never been reported in cattle without bilateral compressive lesions in the optic pathway or bilateral severe retinal atrophy. PMID:25421501

  14. XIAP Regulates Caspase Activity in Degenerating Axons

    Directory of Open Access Journals (Sweden)

    Nicolas Unsain

    2013-08-01

    Full Text Available Our knowledge of the destructive events that regulate axonal degeneration is rudimentary. Here, we examine the role of caspases and their endogenous inhibitor, the X-linked inhibitor of apoptosis protein (XIAP, in axonal degeneration of dorsal root ganglion (DRG axons. We show that caspase-3, caspase-6, and caspase-9 are present in axons and are cleaved upon nerve growth factor (NGF withdrawal. We observed that caspase-3 activity is high in NGF-withdrawn axons and that CASP3−/− axons are protected from degeneration. XIAP−/− DRG sensory neurons degenerate more rapidly and contain more active caspase-3 than their wild-type counterparts, indicating that axonal caspases are normally regulated by XIAP. Importantly, axonal XIAP levels drop sharply after NGF withdrawal; if XIAP levels are maintained by overexpression, axonal caspase-3 activation and axonal degeneration are suppressed. Finally, we show that XIAP−/− embryos have stunted dermal innervation. We propose that XIAP-mediated caspase inhibition plays an important role in regulating morphogenic events that shape the nervous system during development.

  15. Bimodal spectroscopy for in vivo characterization of hypertrophic skin tissue: pre-clinical experimentation, spectral data selection and classification

    Science.gov (United States)

    Liu, Honghui; Gisquet, Héloïse; Guillemin, F.; Blondel, Walter C. P. M.

    2011-07-01

    Objective: The objective of this study was two folds: firstly, we would like to investigate the efficiency of bimodal spectroscopic technique in characterization of hypertrophic scarring tissue deliberately created on a preclinical model (rabbit's ear); on the other hand, we evaluate the inhibition effect of an anti-inflammatory medication (tacrolimus) on hypertrophic formation in scar by using our bimodal spectroscopic system. Study design: This study was conducted on 20 New Zealand Rabbits receiving hypertrophic scarring treatment on their ears. Fluorescence and Diffuse Reflectance spectra were collected from each scar, amongst which some had received tacrolimus treatment. Features were extracted from corrected spectral data and analyzed to classify the scarring tissues into hypertrophic or non-hypertrophic. Diagnostic algorithms were developed with the use of k-NN classifier and validated by comparing to histological classification result with Leave-one- out cross validation. Results and discussion: The accuracy of our bimodal spectroscopy method for detecting hypertrophic scarring scar tissue was good (sensibility: 90.84%, specificity: 94.44%). The features used for classification were mainly extracted from the spectra exited at 360, 410 and 420 nm. This indicates that the difference between the spectra acquired from hypertrophic and non-hypertrophic tissue may be due to the different intensity distribution of several fluorophores (collagen,elastin and NADH) excited in this range, or to the change in proportion of tissue layers (epidermis and dermis) explored by the CEFS in use.

  16. Laser and intense pulsed light therapy for the treatment of hypertrophic scars: a systematic review

    NARCIS (Netherlands)

    Vrijman, C.; van Drooge, A. M.; Limpens, J.; Bos, J. D.; van der Veen, J. P. W.; Spuls, P. I.; Wolkerstorfer, A.

    2011-01-01

    Hypertrophic scars are difficult to improve and remain a therapeutic challenge. Several lasers and light sources have been evaluated in the past decades and have been shown to improve hypertrophic scars. However, a systematic review is not available. To assess current evidence of efficacy of all

  17. Hypertrophic scars after therapy with CO2 laser for treatment of multiple cutaneous neurofibromas

    NARCIS (Netherlands)

    J.U. Ostertag (Judith); C.C.W. Theunissen (Chantal); H.A.M. Neumann (Martino)

    2002-01-01

    textabstractBACKGROUND. CO2 laser surgery is a treatment modality for cutaneous neurofibromas. OBJECTIVE. Hypertrophic and atrophic scars can result from treatment with CO2 laser surgery. We present a case of cutaneous neurofibromatosis that developed hypertrophic scars postoperatively. METHODS.

  18. Long-term outcome of percutaneous transluminal septal myocardial ablation in hypertrophic obstructive cardiomyopathy: a Scandinavian multicenter study

    DEFF Research Database (Denmark)

    Jensen, Morten Kvistholm; Almaas, Vibeke Marie; Jacobsson, Linda

    2011-01-01

    Single-center reports on percutaneous transluminal septal myocardial ablation (PTSMA) in patients with hypertrophic obstructive cardiomyopathy have shown considerable differences in outcome.......Single-center reports on percutaneous transluminal septal myocardial ablation (PTSMA) in patients with hypertrophic obstructive cardiomyopathy have shown considerable differences in outcome....

  19. Families and degenerations of conformal field theories

    Energy Technology Data Exchange (ETDEWEB)

    Roggenkamp, D.

    2004-09-01

    In this work, moduli spaces of conformal field theories are investigated. In the first part, moduli spaces corresponding to current-current deformation of conformal field theories are constructed explicitly. For WZW models, they are described in detail, and sigma model realizations of the deformed WZW models are presented. The second part is devoted to the study of boundaries of moduli spaces of conformal field theories. For this purpose a notion of convergence of families of conformal field theories is introduced, which admits certain degenerated conformal field theories to occur as limits. To such a degeneration of conformal field theories, a degeneration of metric spaces together with additional geometric structures can be associated, which give rise to a geometric interpretation. Boundaries of moduli spaces of toroidal conformal field theories, orbifolds thereof and WZW models are analyzed. Furthermore, also the limit of the discrete family of Virasoro minimal models is investigated. (orig.)

  20. Phase space methods for degenerate quantum gases

    CERN Document Server

    Dalton, Bryan J; Barnett, Stephen M

    2015-01-01

    Recent experimental progress has enabled cold atomic gases to be studied at nano-kelvin temperatures, creating new states of matter where quantum degeneracy occurs - Bose-Einstein condensates and degenerate Fermi gases. Such quantum states are of macroscopic dimensions. This book presents the phase space theory approach for treating the physics of degenerate quantum gases, an approach already widely used in quantum optics. However, degenerate quantum gases involve massive bosonic and fermionic atoms, not massless photons. The book begins with a review of Fock states for systems of identical atoms, where large numbers of atoms occupy the various single particle states or modes. First, separate modes are considered, and here the quantum density operator is represented by a phase space distribution function of phase space variables which replace mode annihilation, creation operators, the dynamical equation for the density operator determines a Fokker-Planck equation for the distribution function, and measurable...

  1. Successful management of hypertrophic cardiomyopathy in a Matschie's tree kangaroo (Dendrolagus matschiei).

    Science.gov (United States)

    Fredholm, Daniel V; Jones, Ashley E; Hall, Natalie H; Russell, Kathleen; Heard, Darryl J

    2015-03-01

    A 3-yr-old, intact male Matschie's tree kangaroo (Dendrolagus matschiei) was examined for a 1-wk history of intermittent lethargy and tachypnea. An echocardiogram revealed concentric hypertrophy of the left ventricular free wall and interventricular septum. These findings were compared to measurements from healthy Matschie's tree kangaroos, supporting a diagnosis of hypertrophic cardiomyopathy. At the time of publication, the patient has been managed for over 11.5 yr, using a combination of enalapril, furosemide, diltiazem, and diet modifications. Hypertrophic cardiomyopathy should be considered as a differential diagnosis in tree kangaroos exhibiting signs of cardiovascular or respiratory distress. This case represents the first report of antemortem diagnosis and successful management of hypertrophic cardiomyopathy in a Matschie's tree kangaroo.

  2. Outcome of alcohol septal ablation in mildly symptomatic patients with hypertrophic obstructive cardiomyopathy

    DEFF Research Database (Denmark)

    Veselka, Josef; Faber, Lothar; Liebregts, Max

    2017-01-01

    Background- The long-term efficacy and safety of alcohol septal ablation (ASA) in patients with highly symptomatic hypertrophic obstructive cardiomyopathy has been demonstrated. The aim of this study was to evaluate the long-term outcomes of mildly symptomatic patients with hypertrophic obstructive......, and III at the last clinical checkup, respectively. Conclusions- Mildly symptomatic hypertrophic obstructive cardiomyopathy patients treated with ASA had sustained symptomatic and hemodynamic relief with a low risk of developing severe heart failure. Their survival is comparable to the general population....... cardiomyopathy treated with ASA. Methods and Results- We retrospectively evaluated consecutive patients enrolled in the Euro-ASA registry (1427 patients) and identified 161 patients (53±13 years; 27% women) who were mildly symptomatic (New York Heart Association [NYHA] class II) pre-ASA. The median...

  3. Two stream instabilities in degenerate quantum plasmas

    CERN Document Server

    Son, S

    2013-01-01

    The quantum mechanical effect on the plasma two-stream instability is studied based on the dielectric function approach. The analysis suggests that the degenerate plasma relevant to the inertial confinement fusion behaves differently from classical plasmas when the electron drift velocity is comparable to the Fermi velocity. For high wave vector comparable to the Fermi wave vector, the degenerate quantum plasma has larger regime of the two-stream instabilities than the classical plasma. A regime, where the plasma waves with the frequency larger than 1.5 times of the Langmuir wave frequency become unstable to the two-stream instabilities, is identified.

  4. [New aspects in age related macular degeneration].

    Science.gov (United States)

    Turlea, C

    2012-01-01

    Being the leading cause of blindness in modern world Age Related Macular Degeneration has beneficiated in the last decade of important progress in diagnosis, classification and the discovery of diverse factors who contribute to the etiology of this disease. Treatments have arised who can postpone the irreversible evolution of the disease and thus preserve vision. Recent findings have identified predisposing genetic factors and also inflamatory and imunological parameters that can be modified trough a good and adequate prevention and therapy This articole reviews new aspects of patology of Age Related Macular Degeneration like the role of complement in maintaining inflamation and the role of oxidative stress on different structures of the retina.

  5. Histone Deacetylase: Therapeutic Targets in Retinal Degeneration.

    Science.gov (United States)

    Daly, Conor; Yin, Jun; Kennedy, Breandán N

    2016-01-01

    Previous studies report that retinitis pigmentosa (RP) patients treated with the histone deacetylase inhibitor (HDACi) valproic acid (VPA) present with improved visual fields and delayed vision loss. However, other studies report poor efficacy and safety of HDACi in other cohorts of retinal degeneration patients. Furthermore, the molecular mechanisms by which HDACi can improve visual function is unknown, albeit HDACi can attenuate pro-apoptotic stimuli and induce expression of neuroprotective factors. Thus, further analysis of HDACi is warranted in pre-clinical models of retinal degeneration including zebrafish. Analysis of HDAC expression in developing zebrafish reveals diverse temporal expression patterns during development and maturation of visual function.

  6. Nonfamilial Hypertrophic Cardiomyopathy: Prevalence, Natural History, and Clinical Implications.

    Science.gov (United States)

    Ingles, Jodie; Burns, Charlotte; Bagnall, Richard D; Lam, Lien; Yeates, Laura; Sarina, Tanya; Puranik, Rajesh; Briffa, Tom; Atherton, John J; Driscoll, Tim; Semsarian, Christopher

    2017-04-01

    Yield of causative variants in hypertrophic cardiomyopathy (HCM) is increased in some probands, suggesting different clinical subgroups of disease occur. We hypothesized that a negative family history and no sarcomere mutations represent a nonfamilial subgroup of HCM. We sought to determine the prevalence, natural history, and potential clinical implications of this nonfamilial subgroup of HCM. Four hundred and thirteen unrelated probands with HCM seen in a specialized HCM center between 2002 and 2015 and genetic testing performed were included in this retrospective cohort study. There were 251 (61%) probands with no reported family history of HCM, including 166 (40% of total) probands with no sarcomere mutation, that is, nonfamilial HCM. Quantified family pedigree data revealed no difference in mean number of first-degree relatives screened between nonfamilial and sarcomere-positive groups. Adjusted predictors of nonfamilial status were older age (odds ratio, 1.04; 95% confidence interval, 1.02-1.06; P =0.0001), male sex (odds ratio, 1.96; 95% confidence interval, 1.11-3.45; P =0.02), hypertension (odds ratio, 2.80; 95% confidence interval, 1.57-5.00; P =0.0005), and nonasymmetric septal morphology (odds ratio, 3.41; 95% confidence interval, 1.64-7.08; P =0.001). They had a less severe clinical course with greater event-free survival from major cardiac events ( P =0.04) compared with sarcomere-positive HCM probands. Genotype prediction scores showed good performance in identifying genotype-positive patients (area under the curve, 0.71-0.75) and, in combination with pedigree characteristics, were further improved. Approximately 40% of HCM probands have a nonfamilial subtype, with later onset and less severe clinical course. We propose a revised clinical pathway for management, highlighting the role of genetic testing, a detailed pedigree, and refined clinical surveillance recommendations for family members. © 2017 American Heart Association, Inc.

  7. Social determinants of health in the setting of hypertrophic cardiomyopathy.

    Science.gov (United States)

    Ingles, Jodie; Johnson, Renee; Sarina, Tanya; Yeates, Laura; Burns, Charlotte; Gray, Belinda; Ball, Kylie; Semsarian, Christopher

    2015-04-01

    Social determinants of health play an important role in explaining poor health outcomes across many chronic disease states. The impact of the social gradient in the setting of an inherited heart disease, hypertrophic cardiomyopathy (HCM), has not been investigated. This study sought to profile the socioeconomic status of patients attending a specialized multidisciplinary clinic and to determine the impact on clinical factors, psychosocial wellbeing and adherence to medical advice. Patients with HCM and at-risk relatives attending a specialized multidisciplinary clinic in Sydney Australia between 2011 and 2013 were included. Clinical, socioeconomic, geographic remoteness and adherence data were available. A broader clinic and registry-based group completed a survey including psychological wellbeing, health-related quality of life, Morisky Medication Adherence Scale and individual-level socioeconomic information. Over a 3-year period, 486 patients were seen in the specialized clinic. There was an over-representation of patients from socioeconomically advantaged and the least geographically remote areas. Socioeconomic disadvantage was associated with comorbidities, poor psychological wellbeing and health-related quality of life, lower understanding of HCM and more complex clinical management issues such as NYHA class, atrial fibrillation and left ventricular outflow tract obstruction. Approximately 10% of patients were non-adherent to medical advice, and poor medication adherence was seen in 30% of HCM patients with associated factors being younger age, minority ethnicity, anxiety and poor mental quality of life. Of all the patients attending a specialized cardiac genetic clinic, there is an overrepresentation of patients from very advantaged and major metropolitan areas and suggests that those most in need of a multidisciplinary approach to care are not accessing it. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  8. Clinical Characteristics and Prognosis of End-stage Hypertrophic Cardiomyopathy

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    Yan Xiao

    2015-01-01

    Full Text Available Background: End-stage hypertrophic cardiomyopathy (HCM is complicated by substantial adverse events. However, few studies have focused on electrocardiographic features and their prognostic values in HCM. This study aimed to evaluate the clinical manifestations and prognostic value of electrocardiography in patients with end-stage HCM. Methods: End-stage HCM patients were enrolled from a total of 1844 consecutive HCM patients from April 2002 to November 2013 at Fuwai Hospital. Clinical data, including medical history, electrocardiography, and echocardiography, were analyzed. Cox hazards regression analysis was used to assess the risk factors for cardiovascular mortality. Results: End-stage HCM was identified in 99 (5.4% patients, averaged at 52 ± 16 years old at entry. Atrial fibrillation was observed in 53 patients and mural thrombus in 19 patients. During 3.9 ± 3.0 years of follow-up, embolic stroke, refractory heart failure, and death or transplantation were observed in 20, 39, and 51 patients, respectively. The incidence of annual mortality was 13.2%. Multivariate Cox hazards regression analysis identified New York Heart Association Class (NYHA III/IV at entry (hazard ratio [HR]: 1.99; 95% confidence interval [CI]: 1.05-3.80; P = 0.036, left bundle branch block (LBBB (HR: 2.80; 95% CI: 1.47-5.31; P = 0.002, and an abnormal Q wave (HR: 2.21; 95% CI: 1.16-4.23; P = 0.016 as independent predictors of cardiovascular death, in accordance with all-cause death and heart failure-related death. Conclusions: LBBB and an abnormal Q wave are risk factors of cardiovascular mortality in end-stage HCM and provide new evidence for early intervention. Susceptibility of end-stage HCM patients to mural thrombus and embolic events warrants further attention.

  9. Ventricular arrhythmias and left ventricular hypertrophy in hypertrophic cardiomyopathy.

    Science.gov (United States)

    Piva e Mattos, Beatriz; Torres, Marco Antonio Rodrigues; Freitas, Valéria Centeno de; Scolari, Fernando Luís; Loreto, Melina Silva de

    2013-05-01

    In hypertrophic cardiomyopathy (HCM), the degree of left ventricular hypertrophy (LVH) could influence the development of ventricular arrhythmias. In HCM, analyze the association between the occurrence of ventricular arrhythmias determined by Holter electrocardiogram (ECG-Holter) and the degree of LVH determined by maximum wall thickness (MWT) in echocardiography and body mass index (BMI). Fifty-four consecutive patients with HCM underwent 24-hour ECG-Holter and echocardiography for assessment of level of LVH through MWT and BMI. Two levels were established for the occurrence of Ventricular Arrhythmias: I - alone or paired extrasystoles and II - Non- Sustained Ventricular Tachycardia (NSVT). In 13 patients (24%) with NSVT (level II), there was a higher frequency of MWT of the left ventricle (LV) > 21 mm (n = 10, 77%, 25 ± 4 mm) and LLLV = 144 g/m² (n = 10, 77%, 200 ± 30 g/m²), in comparison with those presenting with extrasystole arrhythmias (level I) (n = 41, 76%), in which these measures were identified in, respectively, 37 % (n= 15, 23 ± 1 mm), p = 0.023, and 39% (n = 16, 192 ± 53 g / m²) of the cases (p = 0.026). The cut-off values mentioned were determined by the ROC curve with a confidence interval of 95%. NSVT was more common in patients with MWTLV > 21 mm and LLLV > 144 g/m² (8 of 13, 62%) than in those with (4 of 13, 31%) or without (1 of 13; 8%) echocardiographic variables above cut-off values (p = 0.04). In HCM, occurrence of ventricular arrhythmias by Holter was associated with the degree of LVH assessed by echocardiography through MWT and BMI.

  10. Sudden death during empiric amiodarone therapy in symptomatic hypertrophic cardiomyopathy.

    Science.gov (United States)

    Fananapazir, L; Leon, M B; Bonow, R O; Tracy, C M; Cannon, R O; Epstein, S E

    1991-01-15

    Amiodarone is reported to improve symptoms and to prevent sudden death in patients with hypertrophic cardiomyopathy (HC). Amiodarone treatment (loading dose 30 g given over 6 weeks; maintenance dose 400 mg/day) was prospectively evaluated in 50 patients with HC in whom the drug was initiated because of symptoms refractory to conventional drug therapy (calcium antagonists and beta blockers). Twenty-one (42%) patients had ventricular tachycardia (VT) during Holter monitoring. Amiodarone significantly and often markedly improved the patients' New York Heart Association functional class status (from 3.3 to 2.7 at 2 months, p less than 0.001) and treadmill exercise duration (p less than 0.001). Eight patients, however, died (7 suddenly) during a mean follow-up period of 2.2 +/- 1.8 years. Of the 7 sudden deaths, 6 occurred within 5 months of initiation of treatment. The 6-month and 1- and 2-year survival rates were 87, 85 and 80%, respectively. The survival rate of patients with VT was significantly worse than that of patients without VT (61 vs 97% at 2 years; p less than 0.01). Sudden death occurred despite abolition of VT on Holter monitoring. Amiodarone increased left ventricular peak filling rate by radionuclide angiography in 20 of 33 patients (61%) (p less than 0.01). Decrease in peak left ventricular filling rate within 10 days of amiodarone therapy (8 of 33 patients) was associated with subsequent sudden death (p less than 0.04).(ABSTRACT TRUNCATED AT 250 WORDS)

  11. A Murine Hypertrophic Cardiomyopathy Model: The DBA/2J Strain.

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    Wenyuan Zhao

    Full Text Available Familial hypertrophic cardiomyopathy (HCM is attributed to mutations in genes that encode for the sarcomere proteins, especially Mybpc3 and Myh7. Genotype-phenotype correlation studies show significant variability in HCM phenotypes among affected individuals with identical causal mutations. Morphological changes and clinical expression of HCM are the result of interactions with modifier genes. With the exceptions of angiotensin converting enzyme, these modifiers have not been identified. Although mouse models have been used to investigate the genetics of many complex diseases, natural murine models for HCM are still lacking. In this study we show that the DBA/2J (D2 strain of mouse has sequence variants in Mybpc3 and Myh7, relative to widely used C57BL/6J (B6 reference strain and the key features of human HCM. Four-month-old of male D2 mice exhibit hallmarks of HCM including increased heart weight and cardiomyocyte size relative to B6 mice, as well as elevated markers for cardiac hypertrophy including β-myosin heavy chain (MHC, atrial natriuretic peptide (ANP, brain natriuretic peptide (BNP, and skeletal muscle alpha actin (α1-actin. Furthermore, cardiac interstitial fibrosis, another feature of HCM, is also evident in the D2 strain, and is accompanied by up-regulation of type I collagen and α-smooth muscle actin (SMA-markers of fibrosis. Of great interest, blood pressure and cardiac function are within the normal range in the D2 strain, demonstrating that cardiac hypertrophy and fibrosis are not secondary to hypertension, myocardial infarction, or heart failure. Because D2 and B6 strains have been used to generate a large family of recombinant inbred strains, the BXD cohort, the D2 model can be effectively exploited for in-depth genetic analysis of HCM susceptibility and modifier screens.

  12. Biventricular / Left Ventricular Pacing in Hypertrophic Obstructive Cardiomyopathy: An Overview

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    Radu Vatasescu, MD

    2012-05-01

    Full Text Available Hypertrophic cardiomyopathy (HCM is an autosomal dominant inherited genetic disease characterized by compensatory pathological left ventricle (LV hypertrophy due to sarcomere dysfunction. In an important proportion of patients with HCM, the site and extent of cardiac hypertrophy results in severe obstruction to LV outflow tract (LVOT, contributing to disabling symptoms and increasing the risk of sudden cardiac death (SCD. In patients with progressive and/or refractory symptoms despite optimal pharmacological treatment, invasive therapies that diminish or abolish LVOT obstruction relieve heart failure-related symptoms, improve quality of life and could be associated with long-term survival similar to that observed in the general population. The gold standard in this respect is surgical septal myectomy, which might be supplementary associated with a reduction in SCD. Percutaneous techniques, particularly alcohol septal ablation (ASA and more recently radiofrequency (RF septal ablation, can achieve LVOT gradient reduction and symptomatic benefit in a large proportion of HOCM patients at the cost of a supposedly limited septal myocardial necrosis and a 10-20% risk of chronic atrioventricular block. After an initial period of enthusiasm, standard DDD pacing failed to show in randomized trials significant LVOT gradient reductions and objective improvement in exercise capacity. However, case reports and recent small pilot studies suggested that atrial synchronous LV or biventricular (biV pacing significantly reduce LVOT obstruction and improve symptoms (acutely as well as long-term in a large proportion of severely symptomatic HOCM patients not suitable to other gradient reduction therapies. Moreover, biV/LV pacing in HOCM seems to be associated with significant LV reverse remodelling.

  13. Clinicopathological profiles of progressive heart failure in hypertrophic cardiomyopathy.

    Science.gov (United States)

    Melacini, Paola; Basso, Cristina; Angelini, Annalisa; Calore, Chiara; Bobbo, Fabiana; Tokajuk, Barbara; Bellini, Nicoletta; Smaniotto, Gessica; Zucchetto, Mauro; Iliceto, Sabino; Thiene, Gaetano; Maron, Barry J

    2010-09-01

    Hypertrophic cardiomyopathy (HCM) is an important cause of heart failure-related disability over a wide range of ages. Profiles of severe progressive heart failure symptoms and death, or heart transplantation deserve more complete definition within large patient cohorts. Clinical and morphological features of heart failure were assessed in 293 consecutive HCM patients over a median follow-up of 6 (inter-quartile range 2-11) years. Gross and histopathological features were analysed in 12 patients for whom the heart was available for inspection. Of the 293 patients, 50 (17%) developed severe progressive heart failure, including 18 who died or were transplanted. Three profiles of heart failure were identified predominantly associated with: (i) end-stage systolic dysfunction (ejection fraction heart failure in 32 patients (64%) among the three profiles. Compared with other patients, those non-obstructive with preserved systolic function had earlier onset of heart failure symptoms mainly due to diastolic dysfunction, and the most accelerated progression to advanced heart failure and adverse outcome (P = 0.04). Thrombi were identified in the left atrial appendage of five gross heart specimens all belonging to patients with AF, including three of which were unrecognized clinically and had previously embolized. Extensive myocardial scarring with LV remodelling was evident in all end-stage patients; no or only focal scars were present in other patients. Profiles of advanced heart failure in HCM are due to diverse pathophysiological mechanisms, including LV outflow obstruction and diastolic or global systolic ventricular dysfunction. Atrial fibrillation proved to be the most common disease variable associated with progressive heart failure. Recognition of the heterogeneous pathophysiology of heart failure in HCM is relevant, given the targeted management strategies necessary in this disease.

  14. Hypertrophic cardiomyopathy with aortic dilation: a novel observation.

    Science.gov (United States)

    Yousefzai, Rayan; Agarwal, Anushree; Fuad Jan, M; Cho, Chi; Anigbogu, Michael; Shetabi, Kambiz; Singh, Maharaj; Bush, Michelle; Treiber, Shannon; Port, Steven; Ammar, Khawaja Afzal; Paterick, Timothy E; Jain, Renuka; Khandheria, Bijoy K; Jamil Tajik, A

    2017-12-01

    Our goal was to identify the prevalence of aortic dilation in patients with hypertrophic cardiomyopathy (HCM), the most prevalent (0.2%) heritable, genetic cardiovascular disease. Aortic dilation also represents a spectrum of familial inheritance. However, data regarding the prevalence of aortic dilation in HCM patients is lacking. This is an observational retrospective study of all patients referred to our HCM centre. Aortic dilation was defined based on recent American Society of Echocardiography and European Association of Cardiovascular Imaging published guidelines. Of the 201 HCM patients seen between Jan. 1, 2011 and March 31, 2014, 18 (9.0%) met the definition of aortic dilation. Mean age was 56.3 ± 9.3 years, 77.8% were male, mean ascending aorta diameter was 4.0 ± 0.4 cm in males and 3.8 ± 0.2 cm in females, mean sinuses of Valsalva diameter was 4.2 ± 0.2 cm in males and 3.8 ± 0.4 cm in females, and 13 (72.2%) had left ventricular outflow tract obstruction. HCM patients with dilated aorta were more likely males, less likely hypertensive and had larger left ventricle diameter and more aortic valve regurgitation; remaining characteristics were similar. We report a novel observation with 9.0% prevalance of dilated aorta in HCM patients. Further studies are needed to help define the genetic and pathophysiologic basis as well as the clinical implications of this association in a larger group of HCM patients.

  15. Hypertrophic Osteoarthropathy and Follicular Thyroid Cancer: A Rare Paraneoplastic Syndrome.

    Science.gov (United States)

    Tavarelli, Martina; Sarfati, Julie; De Gennes, Christian; Haroche, Julien; Buffet, Camille; Ghander, Cécile; Simon, Jean Marc; Ménégaux, Fabrice; Leenhardt, Laurence

    2015-12-01

    Hypertrophic osteoarthropathy (HOA) is a rare condition characterized by bone and joint pain and digital clubbing usually associated with bronchopulmonary diseases. Primary HOA is rare and the pathogenesis remains unclear. Cases of HOA as a paraneoplastic syndrome associated with thyroid carcinoma are very rare - only 2 cases have been described in the literature. We present the first case of a 40-year-old patient affected by HOA associated with invasive differentiated follicular thyroid carcinoma operated in 2 stages. Both operations were followed by radioiodine ablation, and then a rapid unresectable local recurrence developed requiring cervical radiotherapy (70 Gy). A second treatment with 100 mCi of (131)I confirmed it was a refractory thyroid cancer. Further surgery confirmed a poorly differentiated follicular cancer and 12 cycles of chemotherapy by gemcitabine and oxaliplatin followed. During the 8 years of follow-up, cervical recurrence was stable, but severe episodes of hemoptysis occurred requiring iterative embolization of the bronchial and tracheal arteries. Other lung diseases were excluded. Digital clubbing appeared, which was associated with arthritis, bone pain and inflammatory syndrome. X-rays and magnetic resonance imaging found periosteal apposition in the long bones; bone scintigraphy confirmed the HOA diagnosis. Other causes of arthritis were eliminated. She was treated with colchicine, corticosteroids and nonsteroidal anti-inflammatory drugs, but only the combination of methotrexate and hydroxychloroquine reduced the morphine requirements. HOA is exceptionally associated with thyroid cancer and we raised the hypothesis of the secretion of a circulating factor in a patient with invasive and recurrent follicular thyroid cancer, refractory to radioiodine.

  16. Myocardial glucose metabolism in patients with hypertrophic cardiomyopathy. Assessment by F-18-FDG PET study

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    Uehara, Toshiisa [Osaka Univ., Suita (Japan). Medical School; Ishida, Yoshio; Hayashida, Kohei [and others

    1998-04-01

    In an investigation of myocardial metabolic abnormalities in hypertrophic myocardium, the myocardial glucose metabolism was evaluated with F-18-fluorodeoxyglucose (FDG) positron emission tomography (PET) in 32 patients with hypertrophic cardiomyopathy, and the results were compared with those in 9 patients with hypertensive heart disease. F-18-FDG PET study was performed in the fasting and glucose-loading states. The myocardial regional %dose uptake was calculated quantitatively. The average regional %dose uptake in the fasting state in the patients with asymmetric septal hypertrophy and dilated-phase hypertrophic cardiomyopathy was significantly higher than that in the patients with hypertensive heart disease (0.75{+-}0.34%, 0.65{+-}0.25%, and 0.43{+-}0.22%/100 g myocardium, respectively). In contrast, the average %dose uptake in the glucose-loading state in the patients with asymmetric septal hypertrophy and dilated-phase hypertrophic cardiomyopathy was not significantly different from that in patients with hypertensive heart disease (1.17{+-}0.49%, 0.80{+-}0.44% and 0.99{+-}0.45%, respectively). The patients with apical hypertrophy had also low %dose uptake in the fasting state (0.38{+-}0.21%) as in the hypertensive heart disease patients, so that the characteristics of asymmetric septal hypertrophy and dilated-phase hypertrophic cardiomyopathy are considered to be high FDG uptake throughout the myocardium in the fasting state. Patients with apical hypertrophy are considered to belong to other disease categories metabolically. F-18-FDG PET study is useful in the evaluation of the pathophysiologic diagnosis of patients with hypertrophic cardiomyopathy. (author)

  17. [Experimental research of correlation between anatomy structure of rabbit ear and creating hypertrophic scar animal model].

    Science.gov (United States)

    Zhu, Gui-Ying; Xu, Bin; Cai, Jing-Long

    2008-05-01

    To observe the anatomy structure of rabbit ear and the effect of different operation methods and post-operative treatments on the formation of hypertrophic scar. The experimental animals were 25 New Zealand white rabbits. 6 pieces of full skin specimens were obtained from each of the ears in 5 rabbits for histological examination. 6 full-thickness skin wounds (d = 8 mm) were made on different sites of ventral side of each ear in the other 20 rabbits. The total number of the wounds was 240. 120 wounds in 10 rabbits were divided into 4 groups randomly to receive different treatments on day 7 postoperatively. No treatment was performed in the other 120 wounds. The wounds healing and the scar formation were observed for six months. The scars were harvested 4 weeks and 8 weeks after operation for pathologic examination and measurement of scar elevation index (SEI). Histological analysis showed that the anatomy structure was different in different sites of the rabbit ear. The best sites for creating hypertrophic scar model were on the medial margin of the middle- and inferior part of ear. The depth of the wound should reach the cartilage membrane of the ear to facilitate the formation of hypertrophic scar. The second strip crust on day 7 postoperatively enhanced the wounds healing and minimized the scar proliferation and hypertrophy. There is a close correlation between the anatomy structure of the ear and the creation of hypertrophic scar animal model. The wound site, the depth of wound and the post-operative treatment will affect the formation of hypertrophic scar. The study can help to improve the successful rate of creating hypertrophic scar animal model.

  18. Paclitaxel reduces formation of hypertrophic scars in the rabbit ear model

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    Huang LP

    2015-07-01

    Full Text Available Li-ping Huang,1* Guo-qi Wang,2* Zi-shan Jia,1 Jing-wen Chen,1 Gang Wang,1 Xing-lin Wang1   1Department of Physical Therapy, 2Department of Orthopedics, Chinese PLA General Hospital, Beijing, People’s Republic of China *These authors contributed equally to this work Background and objective: The onset and progression of pathological scarring involves multiple cytokines and complex mechanisms. However, hyperplasia of fibroblasts and neovascularization plays important roles, which can be inhibited by paclitaxel. The aim of this study was to investigate the efficacy of paclitaxel in the treatment of hypertrophic scars on rabbit ears. Methods: Rabbit ear models of hypertrophic scars were established to observe the therapeutic effects of paclitaxel at different concentrations (12 mg/L, 24 mg/L, 48 mg/L, 96 mg/L, 18 mg/L, 54 mg/L, 162 mg/L, 486 mg/L, 30 mg/L, 150 mg/L, 750 mg/L, 3,750 mg/L. The outcome measures included hypertrophic index (HI, density of fibroblasts, density of collagenous fibers, and microvessel density. Results: In comparison with the control group, the concentrations of 96 mg/L, 150 mg/L, and 162 mg/L significantly reduce the formation of hypertrophic scars in the rabbit ear models. However, local necrosis was found in the rabbit ear models treated with paclitaxel solution >400 mg/L. Conclusion: Paclitaxel has strong inhibitory effects on the hyperplasia of fibroblasts, deposition of collagen, and microangiogenesis in hypertrophic scars on rabbit ears within the concentration range from 48 mg/L to 162 mg/L, without causing local necrosis. Keywords: hypertrophic scar, paclitaxel, rabbit ear model

  19. Pathophysiology and meaning of washout rate in hypertrophic heart. Comparison between hypertensive cardiac hypertrophy and hypertrophic cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Nitta, Yutaka (Kanazawa Univ. (Japan). School of Medicine)

    1989-02-01

    The present study was attempted to clarify clinically the pathogenesis of hypertensive cardiac hypertrophy (HT) and hypertrophic cardiomyopathy (HCM). The exercise thallium-201 (Tl-201) myocardial scintigraphy by bicycle ergometer was performed in three groups: control, HT and HCM. The scintigrams were evaluated by circumferential profile analysis. Furthermore, the change of Tl-201 dynamics of exercise Tl-201 scintigraphy with verapamil injection was compared with the change of coronary sinus flow after verapamil injection at cardiac catheterization. The analysis of exercise Tl-201 scintigraphy without verapamil injection showed that initial uptake was not different among the three groups, but washout rate at three hours after Tl-201 injection (WR{sub 3}) was different among the three groups. Although WR{sub 3} of HT was not different from that of control, WR{sub 3} of HCM was lower than that of control. Comparison of WR{sub 3} with and without verapamil was performed. Although WR{sub 3} with verapamil injection was equal to that without verapamil injection in control and HT, WR{sub 3} with verapamil injection decreased compared to that without verapamil injection in HCM. As an index at the time that circulation changes rapidly and on a large scale, washout rate at one hour after Tl-201 injection (WR{sub 1}) was calculated. WR{sub 1} without verapamil injection was not different in the three groups and did not differ from that with verapamil injection in each group. By intravenous administration of verapamil, coronary sinus flow (CSF) increased to the same extent in the three groups. And the increment of CSF was not different in the three groups. (J.P.N.).

  20. MR findings of degenerating parenchymal neurocysticercosis

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    Lee, Yul; Chung, Eun A; Yang, Ik; Park, Hae Jung; Chung, Soo Young [Hallym Univ. Kangnam Sungshim Hospital, Seoul (Korea, Republic of)

    1996-06-01

    To evaluate MR imaging findings of degenerating parenchymal neurocysticercosis and to determine the characteristics which distinguish it from other brain diseases. MR imagings of 19 patients (56 lesions) of degenerating parenchymal neurocysticercosis were retrospectively evaluated, focusing on the size and location of lesions signal intensity patterns of cyst fluid and wall, the extent of the surrounding edema and features of contrast enhancement. Degenerating parenchymal neurocysticercosis was located in gray or subcortical while matter in 89.3% of 56 lesions (50/56) ; most of these (98.2%) were smaller than 2 cm in diameter. Cyst fluid signal was hyperintense relative to CSF on T1 and proton density weighted images (92.9%). A hypointense signal rim of the cyst wall was noted in the lesions on proton density (92.9%) and T2 weighted (98.2%) images, Surrounding edema was mostly mild. Peripheral rim enhancement was noted in all lesions, and this was frequently irregular and lobulated (67.9%) with a focal defect in the enhancing rim(41.1%). Findings which could be helpful in distinguishing degenerating parencymal neurocysticercosis from other brain diseases are as follows : small, superficial lesions ; hyperintense signal of the cyst fluid on T1 and proton density weighted images ; hypointense signal of the cyst wall on proton density and T2 weighted images ; relatively mild extent of surrounding edema, and peripheral rim enhancement which is frequently irregular and lobulated with a focal defect in the enhancing rim.

  1. Motor axon excitability during Wallerian degeneration

    DEFF Research Database (Denmark)

    Moldovan, Mihai; Alvarez, Susana; Krarup, Christian

    2008-01-01

    , action potential propagation and structural integrity of the distal segment are maintained. The aim of this study was to investigate in vivo the changes in membrane function of motor axons during the 'latent' phase of Wallerian degeneration. Multiple indices of axonal excitability of the tibial nerve...

  2. Degenerated Cystic Uterine Myoma Mimicking Postpartum Haemoperitonium

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    Servet Gençdal

    2017-10-01

    Full Text Available Haemoperitoneum due to spontaneous haemorrhage of intraleiomyoma is a very rare complication of uterine myoma. Acute complications of uterine myoma are seldom seen, but it may be fatal. We hereby report case of a female, who presented with abdominal pain and distension, postpratum, found to have degenerated cystic uterine myoma, mimicking postpartum haemoperitonium.

  3. Depression in Age-Related Macular Degeneration

    Science.gov (United States)

    Casten, Robin; Rovner, Barry

    2008-01-01

    Age-related macular degeneration (AMD) is a major cause of disability in the elderly, substantially degrades the quality of their lives, and is a risk factor for depression. Rates of depression in AMD are substantially greater than those found in the general population of older people, and are on par with those of other chronic and disabling…

  4. Driving and Age-Related Macular Degeneration

    Science.gov (United States)

    Owsley, Cynthia; McGwin, Gerald, Jr.

    2008-01-01

    This article reviews the research literature on driving and age-related macular degeneration, which is motivated by the link between driving and the quality of life of older adults and their increased collision rate. It addresses the risk of crashes, driving performance, driving difficulty, self-regulation, and interventions to enhance, safety,…

  5. Cell therapy for the degenerating intervertebral disc.

    Science.gov (United States)

    Tong, Wei; Lu, Zhouyu; Qin, Ling; Mauck, Robert L; Smith, Harvey E; Smith, Lachlan J; Malhotra, Neil R; Heyworth, Martin F; Caldera, Franklin; Enomoto-Iwamoto, Motomi; Zhang, Yejia

    2017-03-01

    Spinal conditions related to intervertebral disc (IVD) degeneration cost billions of dollars in the US annually. Despite the prevalence and soaring cost, there is no specific treatment that restores the physiological function of the diseased IVD. Thus, it is vital to develop new treatment strategies to repair the degenerating IVD. Persons with IVD degeneration without back pain or radicular leg pain often do not require any intervention. Only patients with severe back pain related to the IVD degeneration or biomechanical instability are likely candidates for cell therapy. The IVD progressively degenerates with age in humans, and strategies to repair the IVD depend on the stage of degeneration. Cell therapy and cell-based gene therapy aim to address moderate disc degeneration; advanced stage disease may require surgery. Studies involving autologous, allogeneic, and xenogeneic cells have all shown good survival of these cells in the IVD, confirming that the disc niche is an immunologically privileged site, permitting long-term survival of transplanted cells. All of the animal studies reviewed here reported some improvement in disc structure, and 2 studies showed attenuation of local inflammation. Among the 50 studies reviewed, 25 used some type of scaffold, and cell leakage is a consistently noted problem, though some studies showed reduced cell leakage. Hydrogel scaffolds may prevent cell leakage and provide biomechanical support until cells can become established matrix producers. However, these gels need to be optimized to prevent this leakage. Many animal models have been leveraged in this research space. Rabbit is the most frequently used model (28 of 50), followed by rat, pig, and dog. Sheep and goat IVDs resemble those of humans in size and in the absence of notochordal cells. Despite this advantage, there were only 2 sheep and 1 goat studies of 50 studies in this cohort. It is also unclear if a study in large animals is needed before clinical trials since

  6. Quasiconformal mappings and degenerate elliptic and parabolic equations

    Directory of Open Access Journals (Sweden)

    Filippo Chiarenza

    1987-11-01

    Full Text Available In this paper two Harnak inequalities are proved concerning a degenerate elliptic and a degenerate parabolic equation. In both cases the weight giving the degeneracy is a power of the jacobian of a quasiconformal mapping.

  7. Left Atrial Mechanical Function and Global Strain in Hypertrophic Cardiomyopathy.

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    Kyung-Jin Kim

    Full Text Available Atrial fibrillation is the most common arrhythmia and is associated with adverse outcomes in hypertrophic cardiomyopathy (HCM. Although left atrial (LA remodeling and dysfunction are known to associate with the development of atrial fibrillation in HCM, the changes of the LA in HCM patients remain unclear. This study aimed to evaluate the changes in LA size and mechanical function in HCM patients compared to control subjects and to determine the characteristics of HCM associated with LA remodeling and dysfunction.Seventy-nine HCM patients (mean age, 54 ± 11 years; 76% were men were compared to 79 age- and sex-matched controls (mean age, 54 ± 11 years; 76% were men and 20 young healthy controls (mean age, 33 ± 5 years; 45% were men. The LA diameter, volume, and mechanical function, including global strain (ε, were evaluated by 2D-speckle tracking echocardiography. The phenotype of HCM, maximal left ventricular (LV wall thickness, LV mass, and presence and extent of late gadolinium enhancement (LGE were evaluated with cardiac magnetic resonance imaging.HCM patients showed increased LA volume index, impaired reservoir function, and decreased LA ε compared to the control subjects. When we divided the HCM group according to a maximal LA volume index (LAVImax of 38.7 ml/m2 or LA ε of 21%, no significant differences in the HCM phenotype and maximal LV wall thickness were observed for patients with LAVImax >38.7 ml/m2 or LA ε ≤21%. Conversely, the LV mass index was significantly higher both in patients with maximal LA volume index >38.7 ml/m2 and with LA ε ≤21% and was independently associated with LAVImax and LA ε. Although the LGE extent was increased in patients with LA ε ≤21%, it was not independently associated with either LAVImax or LA ε.HCM patients showed progressed LA remodeling and dysfunction; the determinant of LA remodeling and dysfunction was LV mass index rather than LV myocardial fibrosis by LGE-magnetic resonance

  8. Is abandonment of nonoperative management of hypertrophic pyloric stenosis warranted?

    Science.gov (United States)

    Lukac, Marija; Antunovic, Sanja Sindjic; Vujovic, Dragana; Pavicevic, Polina; Jesic, Milos; Krstajic, Tamara; Petronic, Ivana; Nikolic, Dejan

    2013-02-01

    Evaluation of the effectiveness of oral atropine versus surgical therapy for hypertrophic pyloric stenosis (HPS). A total of 66 consecutive patients with HPS were treated at the University Children's Hospital between January 2006 and December 2011. The diagnosis was initially based on medical history and confirmed by ultrasonography (US). The patients were divided into two groups according to the treatment preferred by their parents. The conservatively treated group, consisting of 33 boys and 7 girls, mean age 22.25 days, was given water-soluble atropine sulfate therapy at an initial dose of 0.05 mg/kg/day divided into 8 single doses, and administered after stomach decompression, 20 minutes prior to feeding. If vomiting persisted, the daily dose was progressively increased up to 0.18 mg/kg. If vomiting did not stop and full oral feeding was not reestablished in a week, surgery was done. The second group of 26 patients, mean age 20.86 days, underwent an operative procedure, Ramstedt extramucosal pyloromyotomy after the initial resuscitation. US evaluation was performed on days 7, 14, and 21. The outcome of the treatment was tested by Yates modification of the χ2 test. In the group of patients treated with atropine sulfate, 10 (25%) failed to respond to therapy, therefore, 8 boys and 2 girls underwent surgical treatment between the fifth and seventh day following institution of therapy. The remaining patients who received atropine sulfate (75%) were discharged when vomiting ceased, between the sixth and eighth day. They continued to take oral medication for 4 to 6 weeks, and were followed up by an ultrasound examination. The operated patients were discharged between the third and fifth day after surgery. There was a significant statistical difference between the groups regarding the outcome at a significance level of p < 0.05 (Yates χ2 = 5.839), with no complications regardless of the treatment option. However, at the significance level of p < 0.01 (Yates χ2 = 7

  9. Clinical Utility of Cardiovascular Magnetic Resonance in Hypertrophic Cardiomyopathy

    Science.gov (United States)

    2012-01-01

    Hypertrophic cardiomyopathy (HCM) is characterized by substantial genetic and phenotypic heterogeneity, leading to considerable diversity in clinical course including the most common cause of sudden death in young people and a determinant of heart failure symptoms in patients of any age. Traditionally, two-dimensional echocardiography has been the most reliable method for establishing a clinical diagnosis of HCM. However, cardiovascular magnetic resonance (CMR), with its high spatial resolution and tomographic imaging capability, has emerged as a technique particularly well suited to characterize the diverse phenotypic expression of this complex disease. For example, CMR is often superior to echocardiography for HCM diagnosis, by identifying areas of segmental hypertrophy (ie., anterolateral wall or apex) not reliably visualized by echocardiography (or underestimated in terms of extent). High-risk HCM patient subgroups identified with CMR include those with thin-walled scarred LV apical aneurysms (which prior to CMR imaging in HCM remained largely undetected), end-stage systolic dysfunction, and massive LV hypertrophy. CMR observations also suggest that the cardiomyopathic process in HCM is more diffuse than previously regarded, extending beyond the LV myocardium to include thickening of the right ventricular wall as well as substantial morphologic diversity with regard to papillary muscles and mitral valve. These findings have implications for management strategies in patients undergoing invasive septal reduction therapy. Among HCM family members, CMR has identified unique phenotypic markers of affected genetic status in the absence of LV hypertrophy including: myocardial crypts, elongated mitral valve leaflets and late gadolinium enhancement. The unique capability of contrast-enhanced CMR with late gadolinium enhancement to identify myocardial fibrosis has raised the expectation that this may represent a novel marker, which may enhance risk stratification. At

  10. Contemporary Natural History and Management of Nonobstructive Hypertrophic Cardiomyopathy.

    Science.gov (United States)

    Maron, Martin S; Rowin, Ethan J; Olivotto, Iacopo; Casey, Susan A; Arretini, Anna; Tomberli, Benedetta; Garberich, Ross F; Link, Mark S; Chan, Raymond H M; Lesser, John R; Maron, Barry J

    2016-03-29

    Left ventricular outflow tract gradients are absent in an important proportion of patients with hypertrophic cardiomyopathy (HCM). However, the natural course of this important patient subgroup remains largely unresolved. The authors systematically employed exercise (stress) echocardiography to define those patients without obstruction to left ventricular outflow at rest and/or under physiological exercise and to examine their natural history and clinical course to create a more robust understanding of this complex disease. We prospectively studied 573 consecutive HCM patients in 3 centers (44 ± 17 years; 66% male) with New York Heart Association functional class I/II symptoms at study entry, including 249 in whom left ventricular outflow tract obstruction was absent both at rest and following physiological exercise (<30 mm Hg; nonobstructive HCM) and retrospectively assembled clinical follow-up data. Over a median follow-up of 6.5 years, 225 of 249 nonobstructive patients (90%) remained in classes I/II, whereas 24 (10%) developed progressive heart failure to New York Heart Association functional classes III/IV. Nonobstructive HCM patients were less likely to experience advanced limiting class III/IV symptoms than the 324 patients with outflow obstruction (1.6%/year vs. 7.4%/year rest obstruction vs. 3.2%/year provocable obstruction; p < 0.001). However, 7 nonobstructive patients (2.8%) did require heart transplantation for progression to end stage versus none of the obstructive patients. HCM-related mortality among nonobstructive patients was low (n = 8; 0.5%/year), with 5- and 10-year survival rates of 99% and 97%, respectively, which is not different from expected all-cause mortality in an age- and sex-matched U.S. population (p = 0.15). HCM patients with nonobstructive disease appear to experience a relatively benign clinical course, associated with a low risk for advanced heart failure symptoms, other disease complications, and HCM-related mortality, and

  11. Determinants of myocardial energetics and efficiency in symptomatic hypertrophic cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Timmer, Stefan A.J.; Germans, Tjeerd; Goette, Marco J.W.; Ruessel, Iris K.; Dijkmans, Pieter A.; Knaapen, Paul; Rossum, Albert C. van [VU University Medical Center, Department of Cardiology, 5F, Amsterdam (Netherlands); Lubberink, Mark; Lammertsma, Adriaan A. [VU University Medical Center, Department of Nuclear Medicine and PET Research, Amsterdam (Netherlands); Berg, Jurrien M. ten [St. Antonius Hospital, Department of Cardiology, Nieuwegein (Netherlands); Cate, Folkert J. ten [Thoraxcenter Erasmus Medical Center, Department of Cardiology, Rotterdam (Netherlands)

    2010-04-15

    Next to hypertrophy, hypertrophic cardiomyopathy (HCM) is characterized by alterations in myocardial energetics. A small number of studies have shown that myocardial external efficiency (MEE), defined by external work (EW) in relation to myocardial oxidative metabolism (MVO{sub 2}), is reduced. The present study was conducted to identify determinants of MEE in patients with HCM by use of dynamic positron emission tomography (PET) and cardiovascular magnetic resonance imaging (CMR). Twenty patients with HCM (12 men, mean age: 55.2 {+-} 13.9 years) and 11 healthy controls (7 men, mean age: 48.1 {+-} 10 years) were studied with [{sup 11}C]acetate PET to assess MVO{sub 2}. CMR was performed to determine left ventricular (LV) volumes and mass (LVM). Univariate and multivariate analyses were employed to determine independent predictors of myocardial efficiency. Between study groups, MVO{sub 2} (controls: 0.12 {+-} 0.04 ml.min{sup -1}.g{sup -1}, HCM: 0.13 {+-} 0.05 ml.min{sup -1}.g{sup -1}, p = 0.64) and EW (controls: 9,139 {+-} 2,484 mmHg.ml, HCM: 9,368 {+-} 2,907 mmHg.ml, p = 0.83) were comparable, whereas LVM was significantly higher (controls: 99 {+-} 21 g, HCM: 200 {+-} 76 g, p < 0.001) and MEE was decreased in HCM patients (controls: 35 {+-} 8%, HCM: 21 {+-} 10%, p < 0.001). MEE was related to stroke volume (SV), LV outflow tract gradient, NH{sub 2}-terminal pro-brain natriuretic peptide (NT-proBNP) and serum free fatty acid levels (all p < 0.05). Multivariate analysis revealed that SV (ss = 0.74, p < 0.001) and LVM (ss = -0.43, p = 0.013) were independently related to MEE. HCM is characterized by unaltered MVO{sub 2}, impaired EW generation per gram of myocardial tissue and subsequent deteriorated myocardial efficiency. Mechanical external efficiency could independently be predicted by SV and LVM. (orig.)

  12. Mucoid degeneration of the anterior cruciate Ligament: a case report ...

    African Journals Online (AJOL)

    We report a case of mucoid degeneration of the anterior cruciate ligament (ACL). Mucoid degeneration of the ACL is a very rare cause of knee pain. There have been only some reported cases of mucoid degeneration of the ACL in the English literature. We reviewed previous reports and summarized clinical features and ...

  13. On the Raman instability in degenerate relativistic plasmas

    Science.gov (United States)

    Chanturia, G. T.; Berezhiani, V. I.; Mahajan, S. M.

    2017-07-01

    The stimulated Raman scattering instability in a fully degenerate electron plasma is studied applying relativistic hydrodynamic and Maxwell equations. We demonstrated that the instability develops for weakly and strongly relativistic degenerate plasmas. It is shown that in the field of strong radiation, a degenerate relativistic plasma effectively responses as in the case of weak degeneracy.

  14. Psychosocial adjustment and quality of life in children undergoing screening in a specialist paediatric hypertrophic cardiomyopathy clinic.

    Science.gov (United States)

    Spanaki, Adriani; O'Curry, Sara; Winter-Beatty, Jasmine; Mead-Regan, Sarah; Hawkins, Kate; English, Jennifer; Head, Catherine; Ridout, Deborah; Tome-Esteban, Maria T; Elliott, Perry; Kaski, Juan P

    2016-06-01

    This study aimed to assess the psychological well-being and quality of life in children with hypertrophic cardiomyopathy and the potential psychosocial impact of screening. A total of 152 children (aged 3-18 years) attending a specialist paediatric hypertrophic cardiomyopathy clinic, and their parents completed the Generic Core Scales and Cardiac Module of the Paediatric Quality of Life Inventory (PedsQL) questionnaire as well as the Strengths and Difficulties Questionnaire; 21 patients (14%) had hypertrophic cardiomyopathy (group A); 23 children (15%) harboured hypertrophic cardiomyopathy-causing sarcomeric mutations with normal echocardiograms (group G); and 108 children (71%) had a family history of hypertrophic cardiomyopathy with normal investigations and attended for clinical cardiological screening (group S). In group A, mean PedsQLTM total scores reported by children and parents were lower than those reported by unaffected children (pchildren and parents were lower in children with hypertrophic cardiomyopathy compared with unaffected patients [mean child-reported total score 86.4 in group S versus 72.3 in group A (pChildren with hypertrophic cardiomyopathy have a significantly reduced quality of life. Importantly, Quality-of-Life scores among unaffected children attending for screening were not different compared with scores from a normative UK population.

  15. MicroRNA 181b regulates decorin production by dermal fibroblasts and may be a potential therapy for hypertrophic scar.

    Directory of Open Access Journals (Sweden)

    Peter Kwan

    Full Text Available Hypertrophic scarring is a frequent fibroproliferative complication following deep dermal burns leading to impaired function and lifelong disfigurement. Decorin reduces fibrosis and induces regeneration in many tissues, and is significantly downregulated in hypertrophic scar and normal deep dermal fibroblasts. It was hypothesized that microRNAs in these fibroblasts downregulate decorin and blocking them would increase decorin and may prevent hypertrophic scarring. Lower decorin levels were found in hypertrophic scar as compared to normal skin, and in deep as compared to superficial dermis. A decorin 3' un-translated region reporter assay demonstrated microRNA decreased decorin in deep dermal fibroblasts, and microRNA screening predicted miR- 24, 181b, 421, 526b, or 543 as candidates. After finding increased levels of mir-181b in deep dermal fibroblasts, it was demonstrated that TGF-β1 stimulation decreased miR-24 but increased miR-181b and that hypertrophic scar and deep dermis contained increased levels of miR-181b. By blocking miR-181b with an antagomiR, it was possible to increase decorin protein expression in dermal fibroblasts. This suggests miR-181b is involved in the differential expression of decorin in skin and wound healing. Furthermore, blocking miR-181b reversed TGF-β1 induced decorin downregulation and myofibroblast differentiation in hypertrophic scar fibroblasts, suggesting a potential therapy for hypertrophic scar.

  16. Effects of Autologous Fat and ASCs on Swine Hypertrophic Burn Scars: A Multimodal Quantitative Analysis

    Directory of Open Access Journals (Sweden)

    Scott J. Rapp, MD

    2017-11-01

    Conclusion:. Early results suggest that autologous fat and/or ASCs may improve healing of hypertrophic scarring by altering the cellular and structural components during wound remodeling up to 20 weeks after injury. This may have beneficial applications in early treatment of large or cosmetically sensitive immature burn scars.

  17. Altering the architecture of tissue engineered hypertrophic cartilaginous grafts facilitates vascularisation and accelerates mineralisation.

    Directory of Open Access Journals (Sweden)

    Eamon J Sheehy

    Full Text Available Cartilaginous tissues engineered using mesenchymal stem cells (MSCs can be leveraged to generate bone in vivo by executing an endochondral program, leading to increased interest in the use of such hypertrophic grafts for the regeneration of osseous defects. During normal skeletogenesis, canals within the developing hypertrophic cartilage play a key role in facilitating endochondral ossification. Inspired by this developmental feature, the objective of this study was to promote endochondral ossification of an engineered cartilaginous construct through modification of scaffold architecture. Our hypothesis was that the introduction of channels into MSC-seeded hydrogels would firstly facilitate the in vitro development of scaled-up hypertrophic cartilaginous tissues, and secondly would accelerate vascularisation and mineralisation of the graft in vivo. MSCs were encapsulated into hydrogels containing either an array of micro-channels, or into non-channelled 'solid' controls, and maintained in culture conditions known to promote a hypertrophic cartilaginous phenotype. Solid constructs accumulated significantly more sGAG and collagen in vitro, while channelled constructs accumulated significantly more calcium. In vivo, the channels acted as conduits for vascularisation and accelerated mineralisation of the engineered graft. Cartilaginous tissue within the channels underwent endochondral ossification, producing lamellar bone surrounding a hematopoietic marrow component. This study highlights the potential of utilising engineering methodologies, inspired by developmental skeletal processes, in order to enhance endochondral bone regeneration strategies.

  18. Enhanced in Vivo Delivery of 5-Fluorouracil by Ethosomal Gels in Rabbit Ear Hypertrophic Scar Model

    Directory of Open Access Journals (Sweden)

    Yan Wo

    2014-12-01

    Full Text Available Applying Ethosomal Gels (EGs in transdermal drug delivery systems has evoked considerable interest because of their good water-solubility and biocompatibility. However, there has not been an explicit description of applying EGs as a vehicle for hypertrophic scars treatment. Here, a novel transdermal EGs loaded with 5-fluorouracil (5-FU EGs was successfully prepared and characterized. The stability assay in vitro revealed that 5-FU EGs stored for a period of 30 days at 4 ± 1 °C had a better size stability than that at 25 ± 1 °C. Furthermore, using confocal laser scanning microscopy, EGs labeled with Rhodamine 6 G penetrated into the deep dermis of the hypertrophic scar within 24 h in the rabbit ear hypertrophic model suggested that the EGs were an optional delivery carrier through scar tissues. In addition, the value of the Scar Elevation Index (SEI of 5-FU EGs group in the rabbit ear scar model was lower than that of 5-FU Phosphate Buffered Saline gel and Control groups. To conclude, these results suggest that EGs delivery system loaded 5-fluorouracil is a perfect candidate drug for hypertrophic scars therapy in future.

  19. Echocardiographic strain imaging to assess early and late consequences of sarcomere mutations in hypertrophic cardiomyopathy

    DEFF Research Database (Denmark)

    Ho, Carolyn Y; Carlsen, Christian; Thune, Jens Jakob

    2009-01-01

    BACKGROUND: Genetic testing identifies sarcomere mutation carriers (G+) before clinical diagnosis of hypertrophic cardiomyopathy (HCM), allowing characterization of initial disease manifestations. Previous studies demonstrated that impaired relaxation develops before left ventricular hypertrophy...... to reshape the clinical paradigm for treatment, based on early diagnosis and disease prevention....

  20. Hypertrophic scar formation is associated with an increased number of epidermal Langerhans cells.

    NARCIS (Netherlands)

    Niessen, F.B.; Schalkwijk, J.; Vos, H.; Timens, W.

    2004-01-01

    The exact pathogenesis of hypertrophic scar and keloid formation is still unknown and a good therapy to prevent or treat these scars is lacking. Because immunological processes seem to be important in excessive scar formation, immunological cells and parameters were studied in a standardized breast

  1. Hypertrophic scar formation is associated with an increased number of epidermal Langerhans cells

    NARCIS (Netherlands)

    Niessen, FB; Schalkwijk, J; Vos, H; Timens, W

    The exact pathogenesis of hypertrophic scar and keloid formation is still unknown and a good therapy to prevent or treat these scars is lacking. Because immunological processes seem to be important in excessive scar formation, immunological cells and parameters were studied in a standardized breast

  2. Rising CO2 levels will intensify phytoplankton blooms in eutrophic and hypertrophic lakes

    NARCIS (Netherlands)

    Verspagen, J.M.H.; van de Waal, D.B.; Finke, J.F.; Visser, P.M.; van Donk, E.; Huisman, J.

    2014-01-01

    Harmful algal blooms threaten the water quality of many eutrophic and hypertrophic lakes and cause severe ecological and economic damage worldwide. Dense blooms often deplete the dissolved CO2 concentration and raise pH. Yet, quantitative prediction of the feedbacks between phytoplankton growth, CO2

  3. Morphological and Tissue Alterations in one Papillary Muscle: an Early Sign of Hypertrophic Cardiomyopathy?

    Directory of Open Access Journals (Sweden)

    Alberto Cresti

    2016-12-01

    Full Text Available Isolated Papillary Muscle (PM hypertrophy has been supposed to be a phenotypic variant of hypertrophic cardiomyopathy. Whether this finding may explain an electrocardiographic pattern of left ventricular hypertrophy has to be demonstrated. A cardiac magnetic resonance imaging may add additional crucial information. Our case was a 26-year-old asymptomatic male cyclist who underwent routine sport medicine screening. His cousin had suddenly died during a bicycle race at 40 years of age, and autopsy had revealed a hypertrophic cardiomyopathy. Screening revealed an electrocardiographic pattern of left ventricular hypertrophy. A multimodal imaging examination was also performed and the only abnormal finding was a hypertrophic anterolateral PM and cardiac magnetic resonance imaging showed fibrotic substitution of its head. An otherwise unexplained electrocardiographic pattern of left ventricular hypertrophy can be justified by an isolated PM hypertrophy. Cardiac magnetic resonance imaging is crucial for precise ventricular wall and papillary thickness measurement. In the presence of an isolated PM hypertrophy, postgadolinium T1 mapping can demonstrate the presence of abnormal tissue and probably fibrosis of the papillary head, which can confirm the presence of a strictly localized form of hypertrophic cardiomyopathy.

  4. High T2-weighted signal intensity is associated with elevated troponin T in hypertrophic cardiomyopathy

    NARCIS (Netherlands)

    Gommans, D.H.F.; Cramer, G.E.; Bakker, J.; Michels, M; Dieker, H.J.; Timmermans, J.; Fouraux, M.A.; Marcelis, C.L.M.; Verheugt, F.W.A.; Brouwer, M.A.; Kofflard, M.J.M.

    2017-01-01

    OBJECTIVE: Areas of high signal intensity (HighT2) on T2-weighted cardiovascular magnetic resonance (CMR) imaging have been demonstrated in hypertrophic cardiomyopathy (HCM). It has been hypothesised that HighT2 may indicate active tissue injury in HCM. In this context, we studied HighT2 in relation

  5. The effect of burn rehabilitation massage therapy on hypertrophic scar after burn: a randomized controlled trial.

    Science.gov (United States)

    Cho, Yoon Soo; Jeon, Jong Hyun; Hong, Aram; Yang, Hyeong Tae; Yim, Haejun; Cho, Yong Suk; Kim, Do-Hern; Hur, Jun; Kim, Jong Hyun; Chun, Wook; Lee, Boung Chul; Seo, Cheong Hoon

    2014-12-01

    To evaluate the effect of burn rehabilitation massage therapy on hypertrophic scar after burn. One hundred and forty-six burn patients with hypertrophic scar(s) were randomly divided into an experimental group and a control group. All patients received standard rehabilitation therapy for hypertrophic scars and 76 patients (massage group) additionally received burn scar rehabilitation massage therapy. Both before and after the treatment, we determined the scores of visual analog scale (VAS) and itching scale and assessed the scar characteristics of thickness, melanin, erythema, transepidermal water loss (TEWL), sebum, and elasticity by using ultrasonography, Mexameter(®), Tewameter(®), Sebumeter(®), and Cutometer(®), respectively. The scores of both VAS and itching scale decreased significantly in both groups, indicating a significant intragroup difference. With regard to the scar characteristics, the massage group showed a significant decrease after treatment in scar thickness, melanin, erythema, TEWL and a significant intergroup difference. In terms of scar elasticity, a significant intergroup difference was noted in immediate distension and gross skin elasticity, while the massage group significant improvement in skin distensibility, immediate distension, immediate retraction, and delayed distension. Our results suggest that burn rehabilitation massage therapy is effective in improving pain, pruritus, and scar characteristics in hypertrophic scars after burn. Copyright © 2014 Elsevier Ltd and ISBI. All rights reserved.

  6. Successful treatment of hypertrophic lichen planus with betamethasone under occlusion and TCA-peelings.

    Science.gov (United States)

    Theodosiou, Grigorios; Papageorgiou, Marina; Vakirlis, Efstratios; Mandekou-Lefaki, Ioanna

    2016-09-01

    Hypertrophic lichen planus (HLP) is a variant of lichen planus characterized by marked epidermal hyperplasia and severe pruritus. We present a case of a female patient with HLP and concomitant primary biliary cirrhosis, which responded to topical therapy with betamethasone under occlusion and TCA-peelings. © 2016 Wiley Periodicals, Inc.

  7. Human hypertrophic and keloid scar models: principles, limitations and future challenges from a tissue engineering perspective

    NARCIS (Netherlands)

    van den Broek, L.J.; Limandjaja, G.C.; Niessen, F.B.; Gibbs, S.

    2014-01-01

    Most cutaneous wounds heal with scar formation. Ideally, an inconspicuous normotrophic scar is formed, but an abnormal scar (hypertrophic scar or keloid) can also develop. A major challenge to scientists and physicians is to prevent adverse scar formation after severe trauma (e.g. burn injury) and

  8. Assessment of quantitative hypertrophy scores in hypertrophic cardiomyopathy : Magnetic resonance imaging versus echocardiography

    NARCIS (Netherlands)

    Posma, JL; Blanksma, PK; vanderWall, EE; Hamer, HPM; Mooyaart, EL; Lie, KI

    1996-01-01

    To compare the diagnostic value of spin-echo magnetic resonance (MR) imaging and transthoracic echocardiography in quantitative assessment of the extent of hypertrophy in patients with hypertrophic cardiomyopathy (HCM), we examined 52 consecutive patients with HCM. The Spirito-Maron and Wigle

  9. Hypertrophic cardiomyopathy family with double-heterozygous mutations; does disease severity suggest doubleheterozygosity?

    NARCIS (Netherlands)

    van Rijsingen, I. A. W.; Hermans-van Ast, J. F.; Arens, Y. H. J. M.; Schalla, S. M.; de Die-Smulders, C. E. M.; van den Wijngaard, A.; Pinto, Y. M.

    2009-01-01

    Background. With the improvement in genetic testing over time, double-heterozygous mutations are more often found by coincidence in families with hypertrophic cardiomyopathy (HCM). Double heterozygosity can be a cause of the wellknown clinical diversity within HCM families.Methods and results. We

  10. Comparison of intralesional verapamil with intralesional triamcinolone in the treatment of hypertrophic scars and keloids

    Directory of Open Access Journals (Sweden)

    Margaret Shanthi F

    2008-01-01

    Full Text Available Background : The calcium channel blocker, verapamil stimulates procollagenase synthesis in keloids and hypertrophic scars. Aim : To study the effect of verapamil in the treatment of hypertrophic scars and keloids and to evaluate the effect of verapamil on the rate of reduction of hypertrophic scars and keloids in comparison with triamcinolone. Methods : The study was a randomized, single blind, parallel group study in which 54 patients were allocated to to receive either verapamil or triamcinolone. Drugs were administered intralesionally in both groups. Improvement of the scar was measured using modified Vancouver scale and by using a centimeter scale serially till the scar flattened. Results : There was a reduction in vascularity, pliability, height and width of the scar with both the drugs after 3 weeks of treatment. These changes were present at one year of follow-up after stopping treatment. Scar pigmentation was not changed desirably by either drug. Length of the scars was also not altered significantly by either drug. The rate of reduction in vascularity, pliability, height and width of the scar with triamcinolone was faster than with verapamil. Adverse drug reactions were more with triamcinolone than with verapamil. Conclusion : Intralesional verapamil may be a suitable alternative to triamcinolone in the treatment of hypertrophic scars and keloids.

  11. Hypertrophic phenotype in cardiac cell assemblies solely by structural cues and ensuing self-organization.

    Science.gov (United States)

    Chung, Chiung-yin; Bien, Harold; Sobie, Eric A; Dasari, Vikram; McKinnon, David; Rosati, Barbara; Entcheva, Emilia

    2011-03-01

    In vitro models of cardiac hypertrophy focus exclusively on applying "external" dynamic signals (electrical, mechanical, and chemical) to achieve a hypertrophic state. In contrast, here we set out to demonstrate the role of "self-organized" cellular architecture and activity in reprogramming cardiac cell/tissue function toward a hypertrophic phenotype. We report that in neonatal rat cardiomyocyte culture, subtle out-of-plane microtopographic cues alter cell attachment, increase biomechanical stresses, and induce not only structural remodeling, but also yield essential molecular and electrophysiological signatures of hypertrophy. Increased cell size and cell binucleation, molecular up-regulation of released atrial natriuretic peptide, altered expression of classic hypertrophy markers, ion channel remodeling, and corresponding changes in electrophysiological function indicate a state of hypertrophy on par with other in vitro and in vivo models. Clinically used antihypertrophic pharmacological treatments partially reversed hypertrophic behavior in this in vitro model. Partial least-squares regression analysis, combining gene expression and functional data, yielded clear separation of phenotypes (control: cells grown on flat surfaces; hypertrophic: cells grown on quasi-3-dimensional surfaces and treated). In summary, structural surface features can guide cardiac cell attachment, and the subsequent syncytial behavior can facilitate trophic signals, unexpectedly on par with externally applied mechanical, electrical, and chemical stimulation.

  12. Heterogeneity of myocardial fluoro-18 2-deoxyglucose uptake in patients with apical hypertrophic cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Shiba, Nobuyuki; Kagaya, Yutaka; Ishide, Nobumasa [Tohoku Univ., Sendai (Japan). School of Medicine] [and others

    1997-03-01

    We have shown that myocardial glucose metabolism is heterogeneous in patients with hypertrophic cardiomyopathy. It is not known, however, whether glucose metabolism is impaired in patients with apical hypertrophic cardiomyopathy, which is fairly common in Japan. We studied 7 patients with apical hypertrophic cardiomyopathy and 5 normal subjects using fluoro-18 2-deoxyglucose (FDG) and positron emission tomography (PET). We calculated regional FDG fractional uptake and the inter-regional coefficient of variation (CV) of FDG fractional uptake in the interventricular septal, anteroapical, and posterolateral regions. The regional FDG fractional uptake was similar in the 2 groups and among the 3 different segments within each group. However, the inter-regional CV of FDG fractional uptake was increased in the anteroapical wall segment of the patient group compared with the control group and also with the other 2 regions in the patient group. The results did not differ when we studied another 5 patients and 6 normal control subjects with a PET scanner with higher spatial resolution. These data suggest that myocardial glucose metabolism may be impaired in the anteroapical wall segment of patients with apical hypertrophic cardiomyopathy. (author)

  13. Regression of outflow tract obstruction subsequent to treatment with verapamil in hypertrophic obstructive cardiomyopathy

    DEFF Research Database (Denmark)

    Frandsen, F; Mickley, H

    1990-01-01

    A 29-year-old man suffering from hypertrophic obstructive cardiomyopathy was treated with verapamil 240 mg daily for 4.5 years. During this period the symptoms were reduced, and an intraventricular gradient diminished from 80 to 20 mm Hg, possibly due to a decrease in left ventricular ejection fr...

  14. Systolic anterior motion of the tricuspid valve in a patient with hypertrophic obstructive cardiomyopathy

    NARCIS (Netherlands)

    Farag, Emile S.; Planken, R. Nils; Boekholdt, S. Matthijs; Kluin, Jolanda

    2017-01-01

    Hypertrophic cardiomyopathy is a heterogeneous myocardial disease and is characterized by increased left ventricular wall thickness. Left ventricular outflow tract obstruction occurs in up to 70% of patients and is often caused by systolic anterior motion of the mitral valve, a paradoxical

  15. Uptake of genetic counselling and predictive DNA testing in hypertrophic cardiomyopathy

    NARCIS (Netherlands)

    Christiaans, Imke; Birnie, Erwin; Bonsel, Gouke J.; Wilde, Arthur A.M.; van Langen, Irene M.

    2008-01-01

    Hypertrophic cardiomyopathy is a common autosomal dominant disease, associated with heart failure and arrhythmias predisposing to sudden cardiac death. After the detection of the causal mutation in the proband predictive DNA testing of relatives is possible (cascade screening). Prevention of sudden

  16. Long-term clinical outcome after alcohol septal ablation for obstructive hypertrophic cardiomyopathy

    DEFF Research Database (Denmark)

    Veselka, Josef; Jensen, Morten Kvistholm; Liebregts, Max

    2016-01-01

    AIMS: The first cases of alcohol septal ablation (ASA) for obstructive hypertrophic cardiomyopathy (HCM) were published two decades ago. Although the outcomes of single-centre and national ASA registries have been published, the long-term survival and clinical outcome of the procedure are still...

  17. [Sigmoid septum: A variant of the ventricular hypertrophy or of the hypertrophic cardiomyopathy?].

    Science.gov (United States)

    Gentille-Lorente, Delicia; Salvadó-Usach, Teresa

    2016-01-01

    Sigmoid septum and hypertrophic cardiomyopathy presenting with left ventricular hypertrophy and, although they appear to be different entities, often involve problems in the differential diagnosis. This study was carried out to assess the prevalence and characteristics of the echocardiographic sigmoid septum and its differential findings regarding hypertrophic cardiomyopathy. Descriptive, observational and prospective study. A total of 1,770 patients were studied by echocardiography. Sigmoid septum (focal and isolated hypertrophy of the basal interventricular septum≥13mm in men and ≥12mm in women, exceeding ≥50% of the median septum thickness) was classified as «Type 1» (≤14mm) and «Type 2» (≥15mm). There were 59 cases of sigmoid septum (prevalence of 3.3%): 26 (1.5%) patients with type 1 (50% male) and 33 (1.9%) patients with type 2 (72.7% male); there were 25 (1.4%) cases of hypertrophic cardiomyopathy (76% male). The group with type 2 sigmoid septum differed from hypertrophic cardiomyopathy in: was older (73±10.5years; Phypertrophic cardiomyopathy, patients with type 2 sigmoid septum are older and generally hypertensive; otherwise, often they have no clear differences in their clinical, electrocardiographic or echocardiographic characteristics. Therefore, cardiac resonance is helpful in the differential diagnosis. Copyright © 2016 Instituto Nacional de Cardiología Ignacio Chávez. Published by Masson Doyma México S.A. All rights reserved.

  18. Cardiac troponin I degradation in serum of patients with hypertrophic obstructive cardiomyopathy undergoing percutaneous septal ablation

    DEFF Research Database (Denmark)

    Madsen, Lene H; Lund, Terje; Grieg, Zanina

    2009-01-01

    : percutaneous transluminal septal myocardial ablation (PTSMA) of hypertrophic obstructive cardiomyopathy (HOCM). Here the iatrogenic induction of myocardial necrosis occurs in vivo, allowing us to investigate degradation of cTnI by the second. METHODS: Blood samples were obtained from 8 patients with HOCM just...

  19. Electrocardiographic features of sarcomere mutation carriers with and without clinically overt hypertrophic cardiomyopathy

    DEFF Research Database (Denmark)

    Lakdawala, Neal K; Thune, Jens Jakob; Maron, Barry J

    2011-01-01

    In hypertrophic cardiomyopathy (HC), electrocardiographic (ECG) changes have been postulated to be an early marker of disease, detectable in sarcomere mutation carriers when left ventricular (LV) wall thickness is still normal. However, the ECG features of mutation carriers have not been fully...

  20. Familial hypertrophic cardiomyopathy: Functional effects of myosin mutation R723G in cardiomyocytes

    NARCIS (Netherlands)

    Kraft, T.; Witjas-Paalberends, E.R.; Boontje, N.; Tripathi, S.; Brandis, A.; Montag, J.; Hodgkinson, J.L.; Francino, A.; Navarro-Lopez, F.; Brenner, B.; Stienen, G.J.M.; van der Velden, J.

    2013-01-01

    Familial Hypertrophic Cardiomyopathy (FHC) is frequently caused by mutations in the β-cardiac myosin heavy chain (β-MyHC). To identify changes in sarcomeric function triggered by such mutations, distinguishing mutation effects from other functional alterations of the myocardium is essential. We

  1. Enhanced in Vivo Delivery of 5-Fluorouracil by Ethosomal Gels in Rabbit Ear Hypertrophic Scar Model

    Science.gov (United States)

    Wo, Yan; Zhang, Zheng; Zhang, Yixin; Zhang, Zhen; Wang, Kan; Mao, Xiaohui; Su, Weijie; Li, Ke; Cui, Daxiang; Chen, Jun

    2014-01-01

    Applying Ethosomal Gels (EGs) in transdermal drug delivery systems has evoked considerable interest because of their good water-solubility and biocompatibility. However, there has not been an explicit description of applying EGs as a vehicle for hypertrophic scars treatment. Here, a novel transdermal EGs loaded with 5-fluorouracil (5-FU EGs) was successfully prepared and characterized. The stability assay in vitro revealed that 5-FU EGs stored for a period of 30 days at 4 ± 1 °C had a better size stability than that at 25 ± 1 °C. Furthermore, using confocal laser scanning microscopy, EGs labeled with Rhodamine 6 G penetrated into the deep dermis of the hypertrophic scar within 24 h in the rabbit ear hypertrophic model suggested that the EGs were an optional delivery carrier through scar tissues. In addition, the value of the Scar Elevation Index (SEI) of 5-FU EGs group in the rabbit ear scar model was lower than that of 5-FU Phosphate Buffered Saline gel and Control groups. To conclude, these results suggest that EGs delivery system loaded 5-fluorouracil is a perfect candidate drug for hypertrophic scars therapy in future. PMID:25501333

  2. Obtaining insurance after DNA diagnostics : A survey among hypertrophic cardiomyopathy mutation carriers

    NARCIS (Netherlands)

    Christiaans, Imke; Kok, Tjitske M.; Van Langen, Irene M.; Birnie, Erwin; Bonsel, Gouke J.; Wilde, Arthur A. M.; Smets, Ellen M. A.

    2010-01-01

    Hypertrophic cardiomyopathy (HCM) is a common hereditary heart disease associated with increased mortality. Disclosure of DNA test results may have social implications such as low access to insurance. In the Netherlands, insurance companies are restricted in the use of genetic information of their

  3. Obtaining insurance after DNA diagnostics: a survey among hypertrophic cardiomyopathy mutation carriers

    NARCIS (Netherlands)

    Christiaans, Imke; Kok, Tjitske M.; van Langen, Irene M.; Birnie, Erwin; Bonsel, Gouke J.; Wilde, Arthur A. M.; Smets, Ellen M. A.

    2010-01-01

    Hypertrophic cardiomyopathy (HCM) is a common hereditary heart disease associated with increased mortality. Disclosure of DNA test results may have social implications such as low access to insurance. In the Netherlands, insurance companies are restricted in the use of genetic information of their

  4. Genetic counseling and cardiac care in predictively tested hypertrophic cardiomyopathy mutation carriers: The patients' perspective

    NARCIS (Netherlands)

    Christiaans, Imke; Van Langen, Irene M.; Birnie, Erwin; Bonsel, Gouke J.; Wilde, Arthur A. M.; Smets, Ellen M. A.

    2009-01-01

    Hypertrophic cardiomyopathy (HCM) is a common hereditary heart disease associated with sudden cardiac death. Predictive genetic counseling and testing are performed using adapted Huntington guidelines, that is, psychosocial care and time for reflection are not obligatory and the test result can be

  5. Hypertrophic nerve roots in a case of Roussy-Levy syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Haubrich, C.; Senderek, J.; Noth, J.; Toepper, R. [Neurologische Klinik, Aachen University, Pauwelsstrasse 30, 52074 Aachen (Germany); Krings, T. [Klinik fuer Neuroradiologische Diagnostik, Aachen University, Pauwelsstrasse 30, 52074 Aachen (Germany); Zuechner, S.; Schroeder, J.M. [Institut fuer Neuropathologie, Aachen University, Pauwelsstrasse 30, 52074 Aachen (Germany)

    2002-11-01

    Hypertrophic radiculopathy is a rare feature of neuropathies. Single cases of enlarged nerve roots have been described in hereditary motor sensory neuropathies (HMSN) and chronic inflammatory demyelinating diseases (CIDP). This is the first description of hypertrophied nerve roots in a patient with Roussy-Levy syndrome. MRI did not show contrast enhancement of the enlarged nerve roots or nodular lesions. (orig.)

  6. Risk stratification for sudden cardiac death in hypertrophic cardiomyopathy : Systematic review of clinical risk markers

    NARCIS (Netherlands)

    Christiaans, Imke; Van Engelen, Klaartje; Van Langen, Irene M.; Birnie, Erwin; Bonsel, Gouke J.; Elliott, Perry M.; Wilde, Arthur A.M.

    2010-01-01

    We performed a systematic literature review of recommended 'major' and 'possible' clinical risk markers for sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM). We searched the Medline, Embase and Cochrane databases for articles published between 1971 and 2007. We included English

  7. Risk stratification for sudden cardiac death in hypertrophic cardiomyopathy: systematic review of clinical risk markers

    NARCIS (Netherlands)

    Christiaans, Imke; van Engelen, Klaartje; van Langen, Irene M.; Birnie, Erwin; Bonsel, Gouke J.; Elliott, Perry M.; Wilde, Arthur A. M.

    2010-01-01

    We performed a systematic literature review of recommended 'major' and 'possible' clinical risk markers for sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM). We searched the Medline, Embase and Cochrane databases for articles published between 1971 and 2007. We included English

  8. Electrocardiographic features of sarcomere mutation carriers with and without clinically overt hypertrophic cardiomyopathy

    DEFF Research Database (Denmark)

    Lakdawala, Neal K; Thune, Jens Jakob; Maron, Barry J

    2011-01-01

    In hypertrophic cardiomyopathy (HC), electrocardiographic (ECG) changes have been postulated to be an early marker of disease, detectable in sarcomere mutation carriers when left ventricular (LV) wall thickness is still normal. However, the ECG features of mutation carriers have not been fully ch...

  9. Three patients presenting with severe macrosomia and congenital hypertrophic cardiomyopathy: a case series.

    Science.gov (United States)

    Vincent, Marie; Benbrik, Nadir; Romefort, Bénédicte; Colombel, Agnès; Bézieau, Stéphane; Isidor, Bertrand

    2017-03-24

    Macrosomia and hypertrophic cardiomyopathy are two features often associated in neonates of diabetic mothers. We report the cases of three patients with severe macrosomia and critical hypertrophic cardiomyopathy without severely unbalanced maternal diabetes. Only three patients with those two features and no uncontrolled maternal diabetes have been previously reported. The first patient was a 39-week-old girl, the second patient was a 39-week-old girl, and the third patient was a 41-week-old boy. The two French girls and the French boy had severe macrosomia and hypertrophic cardiomyopathy, leading to the death of the boy. The outcome of the two girls was favorable, with a standardization of growth curves and ventricular hypertrophy. Their mothers presented with high body mass index but no severe documented maternal diabetes; glycemic imbalance was only suspected on postnatal analyses. There was no hydramnios during pregnancy and no other environmental factor, especially toxic exposure. Their parents are from Mayotte, Guadeloupe, and Guinea-Conakry. The usual genetics causes, Beckwith-Wiedemann syndrome, and chromosomal copy number variation, were also excluded. This report suggests the implication of other factors in addition to glycemic disorders, including genetic factors, in the occurrence of macrosomia and severe hypertrophic cardiomyopathy in neonates. These three original observations indicate that gynecologists and neonatologists should pay attention to neonates from mothers with a high body mass index and when maternal diabetes is not documented.

  10. Observational retrospective study evaluating the effects of oral isotretinoin in keloids and hypertrophic scars.

    Science.gov (United States)

    Guadanhim, Lilia R S; Gonçalves, Rubens G; Bagatin, Ediléia

    2016-11-01

    Acne vulgaris is a chronic inflammatory disease characterized by non-inflammatory and inflammatory lesions that can cause scarring. Oral isotretinoin is the current recommended treatment for moderate and severe cases; however, there are reports of possible influences on the healing process of the skin, leading to an increase in the risk for hypertrophic scars and keloids. This hypothesis, although unproven, represents a contraindication to the treatment of acne scars during the 6-12 months after the cessation of isotretinoin. The aim of this study was to investigate the prevalences of hypertrophic scars and keloids in acne patients treated with oral isotretinoin. Three data collection strategies were used: (i) clinical examination of patients with acne vulgaris, exposed or unexposed to oral isotretinoin, focusing on the occurrence of hypertrophic scars and/or keloids; (ii) telephone interviews of patients using oral isotretinoin to treat acne vulgaris on the occurrence or worsening of keloids; and (iii) clinical examination of patients with previous use of oral isotretinoin followed at a specific keloid treatment clinic. The resulting data showed no differences in wound healing. These findings may indicate that the occurrence of hypertrophic scars or keloids in patients using oral isotretinoin is an undesirable event arising from an individual response and may be related to inflammatory acne evolution. © 2016 The International Society of Dermatology.

  11. Establishing a Reproducible Hypertrophic Scar following Thermal Injury: A Porcine Model

    Directory of Open Access Journals (Sweden)

    Scott J. Rapp, MD

    2015-02-01

    Conclusions: Deep partial-thickness thermal injury to the back of domestic swine produces an immature hypertrophic scar by 10 weeks following burn with thickness appearing to coincide with the location along the dorsal axis. With minimal pig to pig variation, we describe our technique to provide a testable immature scar model.

  12. Altering the architecture of tissue engineered hypertrophic cartilaginous grafts facilitates vascularisation and accelerates mineralisation.

    Science.gov (United States)

    Sheehy, Eamon J; Vinardell, Tatiana; Toner, Mary E; Buckley, Conor T; Kelly, Daniel J

    2014-01-01

    Cartilaginous tissues engineered using mesenchymal stem cells (MSCs) can be leveraged to generate bone in vivo by executing an endochondral program, leading to increased interest in the use of such hypertrophic grafts for the regeneration of osseous defects. During normal skeletogenesis, canals within the developing hypertrophic cartilage play a key role in facilitating endochondral ossification. Inspired by this developmental feature, the objective of this study was to promote endochondral ossification of an engineered cartilaginous construct through modification of scaffold architecture. Our hypothesis was that the introduction of channels into MSC-seeded hydrogels would firstly facilitate the in vitro development of scaled-up hypertrophic cartilaginous tissues, and secondly would accelerate vascularisation and mineralisation of the graft in vivo. MSCs were encapsulated into hydrogels containing either an array of micro-channels, or into non-channelled 'solid' controls, and maintained in culture conditions known to promote a hypertrophic cartilaginous phenotype. Solid constructs accumulated significantly more sGAG and collagen in vitro, while channelled constructs accumulated significantly more calcium. In vivo, the channels acted as conduits for vascularisation and accelerated mineralisation of the engineered graft. Cartilaginous tissue within the channels underwent endochondral ossification, producing lamellar bone surrounding a hematopoietic marrow component. This study highlights the potential of utilising engineering methodologies, inspired by developmental skeletal processes, in order to enhance endochondral bone regeneration strategies.

  13. Hypertrophic cardiomyopathy in pompe disease is not limited to the classic infantile-onset phenotype.

    Science.gov (United States)

    Lee, Dong-Hwan; Qiu, Wen-Juan; Lee, Jeongho; Chien, Yin-Hsiu; Hwu, Wuh-Liang

    2014-01-01

    Pompe disease is a genetic disorder caused by a deficiency of acid α-glucosidase (GAA). Patients with classic infantile-onset Pompe disease usually present with hypertrophic cardiomyopathy and die before 1 year of age, if not treated with enzyme replacement therapy (ERT). In comparison, patients with late-onset Pompe disease typically do not have hypertrophic cardiomyopathy. However, here we describe five patients who presented with hypertrophic cardiomyopathy but did not fit the criteria of classic infantile-onset Pompe disease. Their ages at diagnosis of cardiomyopathy were 1 month in two patients following detection of an audible cardiac murmur and 2-3 years in the three remaining patients. All patients survived for 5-8 years without ERT. Three patients died before the advent of ERT from causes other than congestive heart failure. One patient had a good response to ERT starting at 5 years of age. The sibling of one patient, who did not receive ERT and died at age seven, was diagnosed prenatally. At 3 months of age, the sibling had hypertrophic cardiomyopathy, and a muscle biopsy at that time revealed glycogen accumulation.This case series demonstrates that Pompe disease is a continuum of disease, and the development of cardiomyopathy is not limited to classic infantile-onset Pompe disease. These patients do not fit into the discrete phenotypes of infantile- or late-onset Pompe disease, which may suggest reconsidering the nomenclature of Pompe disease.

  14. Canine hypertrophic osteopathy associated with extra-thoracic lesions Osteopatia hipertrófica canina associada com lesões extratorácicas

    Directory of Open Access Journals (Sweden)

    Selwyn Arlington Headley

    2005-08-01

    Full Text Available Canine hypertrophic osteopathy is described in a dog that presented extra-thoracic lesions, mainly in the liver. Hepatic lesions were characterized by necrosis, hemorrhage, severe hydropic degeneration of centrolobular hepatocytes, proliferation of epithelial cells of bile ducts, and mild biliary stasis. The disease syndrome was diagnosed based on clinical signs, radiological evaluation, and inspection of macerated bones.Osteopatia hipertrófica canina é descrita em um cão que apresentou lesões extratorácicas, principalmente no fígado. As alterações hepáticas foram caracterizadas por necrose, hemorragia, degeneração hepatocelular severa, proliferação das células epiteliais dos ductos biliares e estase biliar. O diagnóstico desta síndrome foi baseado nos sinais clínicos, a avaliação radiológica e a inspeção dos ossos macerados.

  15. Silicone gel sheeting for preventing and treating hypertrophic and keloid scars.

    Science.gov (United States)

    O'Brien, Lisa; Jones, Daniel J

    2013-09-12

    Keloid and hypertrophic scars are common and are caused by a proliferation of dermal tissue following skin injury. They cause functional and psychological problems for patients, and their management can be difficult. The use of silicone gel sheeting to prevent and treat hypertrophic scarring is still relatively new and started in 1981 with treatment of burn scars. To determine the effectiveness of silicone gel sheeting for:(1) prevention of hypertrophic or keloid scarring in people with newly healed wounds (e.g. post surgery);(2) treatment of established scarring in people with existing keloid or hypertrophic scars. In May 2013 we searched the Cochrane Wounds Group Specialised Register; the Cochrane Central Register of Controlled Trials (CENTRAL); Ovid MEDLINE; Ovid MEDLINE (In-Process & Other Non-Indexed Citations); Ovid EMBASE; and EBSCO CINAHL for this second update. Any randomised or quasi-randomised controlled trials, or controlled clinical trials, comparing silicone gel sheeting for prevention or treatment of hypertrophic or keloid scars with any other non surgical treatment, no treatment or placebo. We assessed all relevant trials for methodological quality. Three review authors extracted data independently using a standardised form and cross-checked the results. We assessed all trials meeting the selection criteria for methodological quality. We included 20 trials involving 873 people, ranging in age from 1.5 to 81 years. The trials compared adhesive silicone gel sheeting with no treatment; non silicone dressing; other silicone products; laser therapy; triamcinolone acetonide injection; topical onion extract and pressure therapy. In the prevention studies, when compared with a no treatment option, whilst silicone gel sheeting reduced the incidence of hypertrophic scarring in people prone to scarring (risk ratio (RR) 0.46, 95% confidence interval (CI) 0.21 to 0.98) these studies were highly susceptible to bias. In treatment studies, silicone gel sheeting

  16. Keloid and Hypertrophic Scars Are the Result of Chronic Inflammation in the Reticular Dermis

    Directory of Open Access Journals (Sweden)

    Rei Ogawa

    2017-03-01

    Full Text Available Keloids and hypertrophic scars are caused by cutaneous injury and irritation, including trauma, insect bite, burn, surgery, vaccination, skin piercing, acne, folliculitis, chicken pox, and herpes zoster infection. Notably, superficial injuries that do not reach the reticular dermis never cause keloidal and hypertrophic scarring. This suggests that these pathological scars are due to injury to this skin layer and the subsequent aberrant wound healing therein. The latter is characterized by continuous and histologically localized inflammation. As a result, the reticular layer of keloids and hypertrophic scars contains inflammatory cells, increased numbers of fibroblasts, newly formed blood vessels, and collagen deposits. Moreover, proinflammatory factors, such as interleukin (IL-1α, IL-1β, IL-6, and tumor necrosis factor-α are upregulated in keloid tissues, which suggests that, in patients with keloids, proinflammatory genes in the skin are sensitive to trauma. This may promote chronic inflammation, which in turn may cause the invasive growth of keloids. In addition, the upregulation of proinflammatory factors in pathological scars suggests that, rather than being skin tumors, keloids and hypertrophic scars are inflammatory disorders of skin, specifically inflammatory disorders of the reticular dermis. Various external and internal post-wounding stimuli may promote reticular inflammation. The nature of these stimuli most likely shapes the characteristics, quantity, and course of keloids and hypertrophic scars. Specifically, it is likely that the intensity, frequency, and duration of these stimuli determine how quickly the scars appear, the direction and speed of growth, and the intensity of symptoms. These proinflammatory stimuli include a variety of local, systemic, and genetic factors. These observations together suggest that the clinical differences between keloids and hypertrophic scars merely reflect differences in the intensity, frequency

  17. A new 4D trajectory-based approach unveils abnormal LV revolution dynamics in hypertrophic cardiomyopathy.

    Directory of Open Access Journals (Sweden)

    Andrea Madeo

    Full Text Available The assessment of left ventricular shape changes during cardiac revolution may be a new step in clinical cardiology to ease early diagnosis and treatment. To quantify these changes, only point registration was adopted and neither Generalized Procrustes Analysis nor Principal Component Analysis were applied as we did previously to study a group of healthy subjects. Here, we extend to patients affected by hypertrophic cardiomyopathy the original approach and preliminarily include genotype positive/phenotype negative individuals to explore the potential that incumbent pathology might also be detected. Using 3D Speckle Tracking Echocardiography, we recorded left ventricular shape of 48 healthy subjects, 24 patients affected by hypertrophic cardiomyopathy and 3 genotype positive/phenotype negative individuals. We then applied Generalized Procrustes Analysis and Principal Component Analysis and inter-individual differences were cleaned by Parallel Transport performed on the tangent space, along the horizontal geodesic, between the per-subject consensuses and the grand mean. Endocardial and epicardial layers were evaluated separately, different from many ecocardiographic applications. Under a common Principal Component Analysis, we then evaluated left ventricle morphological changes (at both layers explained by first Principal Component scores. Trajectories' shape and orientation were investigated and contrasted. Logistic regression and Receiver Operating Characteristic curves were used to compare these morphometric indicators with traditional 3D Speckle Tracking Echocardiography global parameters. Geometric morphometrics indicators performed better than 3D Speckle Tracking Echocardiography global parameters in recognizing pathology both in systole and diastole. Genotype positive/phenotype negative individuals clustered with patients affected by hypertrophic cardiomyopathy during diastole, suggesting that incumbent pathology may indeed be foreseen by

  18. Comparative effect and safety of verapamil in keloid and hypertrophic scar treatment: a meta-analysis

    Science.gov (United States)

    Li, Zhouna; Jin, Zhehu

    2016-01-01

    Background Keloids and hypertrophic scar/span>s are the most common types of pathological scarring. Traditionally, keloids have been considered as a result of aberrant wound healing, involving excessive fibroblast participation that is characterized by hyalinized collagen bundles. However, the usefulness of this characterization has been questioned. In recent years, studies have reported the appropriate use of verapamil for keloids and hypertrophic scar/span>s. Methods Searches were conducted on the databases Medline, Embase, Cochrane, PubMed, and China National Knowledge Infrastructure from 2006 to July 2016. State12.0 was used for literature review, data extraction, and meta-analysis. Treatment groups were divided into verapamil and nonverapamil group. Nonverapamil group includes steroids and intense pulsed light (IPL) therapy. Total effective rates include cure rate and effective rate. Cure: skin lesions were completely flattened, became soft and symptoms disappeared. Efficacy: skin lesions subsided, patient significantly reduced symptoms. Inefficient definition of skin was progression free or became worse. Random-effects model was used for the meta-analysis. Results Six studies that included 331 patients with keloids and hypertrophic scar/span>s were analyzed. Analysis of the total effective rate of skin healing was performed. The total effective rates in the two groups were 54.07% (verapamil) and 53.18% (nonverapamil), respectively. The meta-analysis showed that there was no difference between the two groups. We also compared the adverse reactions between the verapamil treatment group and the steroids treatment group in two studies, and the result indicated that the verapamil group showed less adverse reactions. Conclusion There were no differences between the application of verapamil and nonverapamil group in keloids and hypertrophic scar/span>s treatment. Verapamil could act as an effective alternative modality in the prevention and treatment of keloid and

  19. Wallerian degeneration of brain: MRI and CT findings

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Woo Suk; Ryu, Kyung Nam [Kyung Hee University Hsopital, Seoul (Korea, Republic of)

    1992-11-15

    Wallerian degeneration is well known as the anterograde degeneration of axon and their accompanying myelin sheath from injury to the proximal portion of the axon or its cell body. The most common cause of wallerian degeneration is cerebral infarction. Authors experienced three patients with old hemispheric infarct with typical wallerian degeneration in the brain stem, which was demonstrated by magnetic resonance imaging (MRI) in two cases and CT in one case. This report demonstrates the wallerian degeneration in the corticospinal tract in on the MRI and CT with the brief review of literatures.

  20. Ultracompact quantum splitter of degenerate photon pairs

    CERN Document Server

    He, Jiakun; Casas-Bedoya, Alvaro; Zhang, Yanbing; Xiong, Chunle; Eggleton, Benjamin J

    2015-01-01

    Integrated sources of indistinguishable photons have attracted a lot of attention because of their applications in quantum communication and optical quantum computing. Here, we demonstrate an ultra-compact quantum splitter for degenerate single photons based on a monolithic chip incorporating Sagnac loop and a micro-ring resonator with a footprint of 0.011 mm2, generating and deterministically splitting indistinguishable photon pairs using time-reversed Hong-Ou-Mandel interference. The ring resonator provides enhanced photon generation rate, and the Sagnac loop ensures the photons travel through equal path lengths and interfere with the correct phase to enable the reversed HOM effect to take place. In the experiment, we observed a HOM dip visibility of 94.5 +- 3.3 %, indicating the photons generated by the degenerate single photon source are in a suitable state for further integration with other components for quantum applications, such as controlled-NOT gates.

  1. Mesenteric paraganglioma with cystic degeneration: case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Dae Bong; Cho, June Sik; Shin, Kyung Sook; Shin, Byung Seok; Noh, Seung Moo; Kang, Dae Young [Chungnam National University College of Medicine, Taejon (Korea, Republic of)

    2003-09-01

    Extra-adrenal abdominal paragangliomas are rare. Most arise from the organs of Zuckerkandl, involve large concentrations of paraganglionic tissue, and are located in the para-aortic space along the sympathetic chain. Published reports have, however, described normal paraganglionic tissue at the root of the mesentery which serves as the superior limit of the organs of Zuckerkandl, and mesenteric paraganglioma is very rare. We report a case of paraganglioma with cystic degeneration arising from the mesentery.

  2. Degenerate RFID Channel Modeling for Positioning Applications

    Directory of Open Access Journals (Sweden)

    A. Povalac

    2012-12-01

    Full Text Available This paper introduces the theory of channel modeling for positioning applications in UHF RFID. It explains basic parameters for channel characterization from both the narrowband and wideband point of view. More details are given about ranging and direction finding. Finally, several positioning scenarios are analyzed with developed channel models. All the described models use a degenerate channel, i.e. combined signal propagation from the transmitter to the tag and from the tag to the receiver.

  3. Muscle weakness causes joint degeneration in rabbits.

    Science.gov (United States)

    Rehan Youssef, A; Longino, D; Seerattan, R; Leonard, T; Herzog, W

    2009-09-01

    The objective of this study was to investigate the effects of botulinum toxin type-A (BTX-A) induced quadriceps weakness on micro-structural changes in knee cartilage of New Zealand White (NZW) rabbits. Fifteen rabbits were divided randomly into an experimental and a sham control group. Each group received a unilateral single quadriceps muscle injection either with saline (sham control; n=4) or BTX-A (experimental; n=11). BTX-A injection produced significant quadriceps muscle weakness (Pmuscle mass (Pknee cartilage, assessed with the Mankin grading system, were the same for the injected and non-injected hind limbs of the experimental group animals. Sham injection had no effect on joint degeneration but all control animals showed some degenerative changes in the knee. Degenerative changes of the retro-patellar cartilage were more severe in the experimental compared to sham control group rabbits (P0.05). Quadriceps muscle weakness caused increased degeneration in the retro-patellar cartilage of NZW rabbits, providing evidence that muscle weakness might be a risk factor for the onset and progression of osteoarthritis (OA). Future work needs to delineate whether muscle weakness directly affects joint degeneration, or if changes in function and movement execution associated with muscle weakness are responsible for the increased rate of OA onset and progression observed here.

  4. The trapping effect on degenerate horizons

    CERN Document Server

    Angelopoulos, Yannis; Gajic, Dejan

    2015-01-01

    We show that degenerate horizons exhibit a new trapping effect. Specifically, we obtain a non-degenerate Morawetz estimate for the wave equation in the domain of outer communications of extremal Reissner-Nordstrom up to and including the future event horizon. We show that such an estimate requires 1) a higher degree of regularity for the initial data, reminiscent of the regularity loss in the high-frequency trapping estimates on the photon sphere, and 2) the vanishing of an explicit quantity that depends on the restriction of the initial data on the horizon. The latter condition demonstrates that degenerate horizons exhibit a global trapping effect (in the sense that this effect is not due to individual underlying null geodesics as in the case of the photon sphere). We moreover uncover a new stable higher-order trapping effect; we show that higher-order estimates do not hold regardless of the degree of regularity and the support of the initial data. We connect our findings to the spectrum of the stability ope...

  5. Keloid and Hypertrophic Scars Are the Result of Chronic Inflammation in the Reticular Dermis

    National Research Council Canada - National Science Library

    Rei Ogawa

    2017-01-01

    Keloids and hypertrophic scars are caused by cutaneous injury and irritation, including trauma, insect bite, burn, surgery, vaccination, skin piercing, acne, folliculitis, chicken pox, and herpes zoster infection...

  6. Comparison of Valsalva manoeuvre and exercise in echocardiographic evaluation of left ventricular outflow tract obstruction in hypertrophic cardiomyopathy

    DEFF Research Database (Denmark)

    Jensen, Morten Krogh; Havndrup, Ole; Pecini, Redi

    2010-01-01

    Several methods are used to induce latent left ventricular outflow tract (LVOT) gradients in patients with hypertrophic cardiomyopathy (HCM). We compared LVOT gradients induced by Valsalva manoeuvre (VM) and exercise echocardiography (EE) in patients with HCM treated with percutaneous translumina...

  7. Comparison of Valsalva manoeuvre and exercise in echocardiographic evaluation of left ventricular outflow tract obstruction in hypertrophic cardiomyopathy

    DEFF Research Database (Denmark)

    Jensen, Morten Krogh; Havndrup, Ole; Pecini, Redi

    2010-01-01

    Several methods are used to induce latent left ventricular outflow tract (LVOT) gradients in patients with hypertrophic cardiomyopathy (HCM). We compared LVOT gradients induced by Valsalva manoeuvre (VM) and exercise echocardiography (EE) in patients with HCM treated with percutaneous transluminal...

  8. In vivo cardiovascular magnetic resonance diffusion tensor imaging shows evidence of abnormal myocardial laminar orientations and mobility in hypertrophic cardiomyopathy

    National Research Council Canada - National Science Library

    Ferreira, Pedro F; Kilner, Philip J; McGill, Laura-Ann; Nielles-Vallespin, Sonia; Scott, Andrew D; Ho, Siew Y; McCarthy, Karen P; Haba, Margarita M; Ismail, Tevfik F; Gatehouse, Peter D; de Silva, Ranil; Lyon, Alexander R; Prasad, Sanjay K; Firmin, David N; Pennell, Dudley J

    2014-01-01

    ... orientation and mobility in hypertrophic cardiomyopathy (HCM). We performed cDTI in vivo at 3 Tesla at end-systole and late diastole in 11 healthy controls and 11 patients with HCM, as well as late gadolinium enhancement (LGE...

  9. Unravelling the grey zone : Cardiac MRI volume to wall mass ratio to differentiate hypertrophic cardiomyopathy and the athlete's heart

    NARCIS (Netherlands)

    Luijkx, Tim; Cramer, Maarten J.; Buckens, Stan; Zaidi, Abbas; Rienks, R; Mosterd, Arend; Prakken, Niek H J; Dijkman, Barbara; Mali, W.P.; Velthuis, BK

    2015-01-01

    Background Differentiating physiological left ventricular hypertrophy (LVH) in athletes from pathological hypertrophic cardiomyopathy (HCM) can be challenging. This study assesses the ability of cardiac MRI (CMR) to distinguish between physiological LVH (so-called athlete's heart) and HCM. Methods

  10. The structure of winter phytoplankton in Lake Nero, Russia, a hypertrophic lake dominated by Planktothrix-like Cyanobacteria

    OpenAIRE

    Babanazarova, Olga; Sidelev, Sergey; Schischeleva, Svetlana

    2013-01-01

    Background The permanent dominance of Planktothrix-like ?yanobacteria has been often reported for shallow eutrophic\\hypertrophic lakes in central Europe in summer\\autumn. However studies on phytoplankton growth under ice cover in nutrient-rich lakes are very scarce. Lake Nero provides a good example of the contrasting seasonal extremes in environmental conditions. Moreover, the ecosystem underwent a catastrophic transition from eutrophic to hypertrophic 2003?05, with dominance of filamentous ...

  11. Biological and Chemical Removal of Primary Cilia Affects Mechanical Activation of Chondrogenesis Markers in Chondroprogenitors and Hypertrophic Chondrocytes

    Directory of Open Access Journals (Sweden)

    Matthew E. Deren

    2016-02-01

    Full Text Available Chondroprogenitors and hypertrophic chondrocytes, which are the first and last stages of the chondrocyte differentiation process, respectively, are sensitive to mechanical signals. We hypothesize that the mechanical sensitivity of these cells depends on the cell surface primary cilia. To test this hypothesis, we removed the primary cilia by biological means with transfection with intraflagellar transport protein 88 (IFT88 siRNA or by chemical means with chloral hydrate treatment. Transfection of IFT88 siRNA significantly reduced the percentage of ciliated cells in both chondroprogenitor ATDC5 cells as well as primary hypertrophic chondrocytes. Cyclic loading (1 Hz, 10% matrix deformation of ATDC5 cells in three-dimensional (3D culture stimulates the mRNA levels of chondrogenesis marker Type II collagen (Col II, hypertrophic chondrocyte marker Type X collagen (Col X, and a molecular regulator of chondrogenesis and chondrocyte hypertrophy bone morphogenetic protein 2 (BMP-2. The reduction of ciliated chondroprogenitors abolishes mechanical stimulation of Col II, Col X, and BMP-2. In contrast, cyclic loading stimulates Col X mRNA levels in hypertrophic chondrocytes, but not those of Col II and BMP-2. Both biological and chemical reduction of ciliated hypertrophic chondrocytes reduced but failed to abolish mechanical stimulation of Col X mRNA levels. Thus, primary cilia play a major role in mechanical stimulation of chondrogenesis and chondrocyte hypertrophy in chondroprogenitor cells and at least a partial role in hypertrophic chondrocytes.

  12. Q wave T wave vector discordance in hypertrophic cardiomyopathy: septal hypertrophy and strain pattern.

    Science.gov (United States)

    Goldberger, A L

    1979-08-01

    Hypertrophic cardiomyopathy is a common cause of prominent non-infarctional Q waves. A retrospective analysis of previously published cases confirmed a characteristic Q wave T wave vector discordance in hypertrophic cardiomyopathy. In 41 of 44 cases with predominant Q waves (as part of QS or Qr complexes where Q wave amplitude exceeded R wave height), the T wave was positive, and in all cases with QS type complexes the T wave was positive. This characteristic electrocardiographic sign probably represents a pattern of septal hypertrophy and strain (Q waves with positive T waves and ST segment elevation) which is the inverse of the classical pattern of left ventricular hypertrophy and strain (tall R waves with inverted T waves and ST segment depression).

  13. Apical hypertrophic cardiomyopathy with hemodynamically unstable ventricular arrhythmia – Atypical presentation

    Directory of Open Access Journals (Sweden)

    Hemant Chaturvedi

    2016-09-01

    Full Text Available We present a patient with asymptomatic apical hypertrophic cardiomyopathy (AHCM who recently developed cardiac arrhythmias, and shortly discuss the diagnostic modalities, differential diagnosis, and treatment strategy for this condition. AHCM is a rare form of hypertrophic cardiomyopathy, which usually involves the apex of the left ventricle. AHCM can occur with varied presentations such as chest pain, palpitations, dyspnea, syncope, atrial fibrillation, myocardial infarction, embolic events, ventricular fibrillation, and congestive heart failure. The most peculiar electrocardiogram findings are giant T-waves inversion in the precordial leads with left ventricular (LV hypertrophy. A transthoracic echocardiogram is the initial diagnostic modality in the evaluation of AHCM and shows hypertrophy of the LV apex. Other diagnostic modalities, including left ventriculography, multislice spiral computed tomography, and cardiac magnetic resonance imagings, are also valuable tools. Medications used to manage include verapamil, beta-blockers, and antiarrhythmic agents. An implantable cardioverter defibrillator (ICD is recommended for high-risk patients.

  14. Hypertrophic Scarring and Keloids: Pathomechanisms and Current and Emerging Treatment Strategies

    Science.gov (United States)

    Gauglitz, Gerd G; Korting, Hans C; Pavicic, Tatiana; Ruzicka, Thomas; Jeschke, Marc G

    2011-01-01

    Excessive scars form as a result of aberrations of physiologic wound healing and may arise following any insult to the deep dermis. By causing pain, pruritus and contractures, excessive scarring significantly affects the patient’s quality of life, both physically and psychologically. Multiple studies on hypertrophic scar and keloid formation have been conducted for decades and have led to a plethora of therapeutic strategies to prevent or attenuate excessive scar formation. However, most therapeutic approaches remain clinically unsatisfactory, most likely owing to poor understanding of the complex mechanisms underlying the processes of scarring and wound contraction. In this review we summarize the current understanding of the pathophysiology underlying keloid and hypertrophic scar formation and discuss established treatments and novel therapeutic strategies. PMID:20927486

  15. Transcriptional Profiling of Rapamycin-Treated Fibroblasts From Hypertrophic and Keloid Scars

    Science.gov (United States)

    Wong, Victor W.; You, Fanglei; Januszyk, Michael; Gurtner, Geoffrey C.; Kuang, Anna A.

    2016-01-01

    Excess scar formation after cutaneous injury can result in hypertrophic scar (HTS) or keloid formation. Modern strategies to treat pathologic scarring represent nontargeted approaches that produce suboptimal results. Mammalian target of rapamycin (mTOR), a central mediator of inflammation, has been proposed as a novel target to block fibroproliferation. To examine its mechanism of action, we performed genomewide microarray on human fibroblasts (from normal skin, HTS, and keloid scars) treated with the mTOR inhibitor, rapamycin. Hypertrophic scar and keloid fibroblasts demonstrated overexpression of collagen I and III that was effectively abrogated with rapamycin. Blockade of mTOR specifically impaired fibroblast expression of the collagen biosynthesis genes PLOD, PCOLCE, and P4HA, targets significantly overexpressed in HTS and keloid scars. These data suggest that pathologic scarring can be abrogated via modulation of mTOR pathways in procollagen and collagen processing. PMID:24835866

  16. Multimodality treatment of hypertrophic scars using long-wave X-radiation

    Energy Technology Data Exchange (ETDEWEB)

    Protsenko, T.V. (Donetskij Meditsinskij Inst. (Ukrainian SSR))

    1984-02-01

    The paper is concerned with a therapeutic method for hypertrophic scars including Bucky rays, pyrogenal, vitamins A, E, B/sub 12/, sodium salicylate and dimexide ointment. Multimodality treatment of hypertrophic scars is effective, well tolerated and can be widely used in out-patient clinics. It makes it possible to reduce the period of therapy by 2-3 mos., and the summary dose by 20 000-3000 rad (18.6-27.9 Gy) as compared to common therapy with Bucky rays only. Changes in the connective structure of scars in the process of multimodality therapy are nonspecific and account for some mechanisms of scar regression and reaffirm the efficacy of this therapeutic method.

  17. Hypertrophic lichen planus as a presenting feature of human immunodeficiency virus infection

    Directory of Open Access Journals (Sweden)

    Kumari Rashmi

    2009-01-01

    Full Text Available Lichen planus (LP is a chronic papulosquamous dermatosis in which both skin and mucous membranes may be involved. To date, there have been only five reports of human immunodeficiency virus (HIV-positive patients with hypertrophic LP. In the present report, we describe a 37-year-old female who presented with widely distributed, hyperpigmented, pruritic scaly lesions involving the face, trunk, and upper and lower extremities for one month. She also had swelling of both lower legs with low grade fever for past one week. She was diagnosed to be a HIV-positive patient who had severe, widespread hypertrophic LP lesions along with acute eruptive lesions of LP. These LP lesions were a presenting feature of HIV infection in our case.

  18. Hypertrophic Cardiomyopathy Mimicking Acute Anterior Myocardial Infarction Associated with Sudden Cardiac Death

    Directory of Open Access Journals (Sweden)

    Y. Daralammouri

    2012-01-01

    Full Text Available Hypertrophic cardiomyopathy is the most common genetic disease of the heart. We report a rare case of hypertrophic obstructive cardiomyopathy mimicking an acute anterior myocardial infarction associated with sudden cardiac death. The patient presented with acute ST elevation myocardial infarction and significant elevation of cardiac enzymes. Cardiac catheterization showed some atherosclerotic coronary artery disease, without significant stenosis. Echocardiography showed left ventricular hypertrophy with a left ventricular outflow tract obstruction; the pressure gradient at rest was 20 mmHg and became severe with the Valsalva maneuver (100 mmHg. There was no family history of sudden cardiac death. Six days later, the patient suffered a syncope on his way to magnetic resonance imaging. He was successfully resuscitated by ventricular fibrillation.

  19. Hypertrophic Cardiomyopathy (HCM): How Flow Analysis May Drive Medical Management and Surgical Approach

    Science.gov (United States)

    Abraham, Theodore P.

    2011-11-01

    Hypertrophic Cardiomyopathy (HCM) is the most common inherited heart disease and occurs in 1 in 500 persons worldwide regardless of race, age and gender. It is the most common cause of sudden death in the young and also causes heart failure and cardiac arrhythmias. The primary anatomic abnormality is thickening of certain walls, or sometimes global thickening of the left or right ventricle. The patterns of thickening along with increased ventricular stiffness lead to suboptimal ventricular filling and inefficient ejection of blood from the ventricle. Treatment for HCM can be medical or surgical. The choice of therapy is driven by the presence and severity of outflow obstruction. Flow analysis could provide sophisticated information about outflow and inflow ventricular dynamics. These flow dynamics features may enable better medical choices and provide information that would allow superior surgical planning. Associate Professor of Medicine & Director, Hypertrophic Cardiomyopathy Clinic

  20. Squamous Cell Carcinoma of the Penis with Pulmonary Metastasis and Paraneoplastic Hypertrophic Osteopathy in a Dog.

    Science.gov (United States)

    Jenkins, Victoria; Henrique de Mello Souza, Carlos; de Lorimier, Louis-Phillippe; de Toledo-Piza, Evandro

    Squamous cell carcinoma of the penis was diagnosed by incisional biopsy of a penile mass in a 12 yr old intact male beagle dog presenting with hemorrhagic discharge from the prepuce. Penile amputation, orchiectomy with scrotal ablation, and scrotal urethrostomy were performed. Hypertrophic osteopathy secondary to pulmonary metastatic disease occurred 10 mo after the surgery. Palliative treatment with piroxicam was administered and led to complete resolution of the clinical signs of the pain. Sixteen months following surgery, the dog presented with significant dyspnea and anorexia and was euthanized due to poor prognosis. This case report describes a rare penile tumor, squamous cell carcinoma. Consequent paraneoplastic hypertrophic osteopathy and its palliative treatment are also reviewed.

  1. Hypertrophic non-union of a pathological forearm fracture secondary to multiple myeloma: a case report

    Directory of Open Access Journals (Sweden)

    Okoro Tosan

    2010-04-01

    Full Text Available Abstract Skeletal lesions in multiple myeloma are predominantly lytic and when non-union of pathological fractures occur it is typically atrophic. We report a lady of 61 years of age with myeloma who presented with a pathological fracture through an ulnar myeloma deposit. The fracture was immobilised initially then irradiated. Nine months later she re-presented with marked forearm pain particularly on rotation. Radiographs demonstrated a hypertrophic non-union of a pathological fracture with a typical elephant's hoof appearance. The fracture was immobilised using an ulnar nail. Whilst non-unions in metastatic malignancy are typically atrophic, just occasionally hypertrophic non-unions can occur. Management principles remain the same with stabilisation of the entire bone and early mobilisation being appropriate.

  2. Efficacy and safety of the angiotensin II receptor blocker losartan for hypertrophic cardiomyopathy

    DEFF Research Database (Denmark)

    Axelsson, Anna; Iversen, Kasper; Vejlstrup, Niels

    2015-01-01

    . Treatment was well tolerated by patients with left ventricular outflow obstruction at baseline. INTERPRETATION: Our findings challenge the generally held view that angiotensin II receptor blockers reduce cardiac hypertrophy. Treatment with losartan was safe, suggesting that it can be used for other......BACKGROUND: No medical treatment has been reliably shown to halt or reverse disease progression in hypertrophic cardiomyopathy, but the results of several pilot studies have suggested beneficial effects of angiotensin II receptor blockers on left ventricular hypertrophy and fibrosis, which...... are predictive of an adverse outcome. We aimed to assess the effect of the angiotensin II receptor blocker losartan on left ventricular hypertrophy and fibrosis in patients with hypertrophic cardiomyopathy. METHODS: In this single-centre, randomised, double-blind, placebo-controlled trial, adult patients (aged...

  3. Cheating the CHA2DS2-VASc Score: Thromboembolism in Apical Hypertrophic Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Robin A. P. Weir

    2014-01-01

    Full Text Available Atrial fibrillation increases the risk of systemic thromboembolism in general and stroke in particular. Not all patients who develop atrial fibrillation are at significantly heightened risk of thromboembolic complications, however, with the development of risk scoring systems aiding clinicians in determining whether formal anticoagulation is mandated. The most commonly used contemporary scoring systems—CHADS2 and CHA2DS2-VASc—provide a reliable means of assessing stroke risk, but certain cardiac conditions are associated with an increased incidence of thromboembolism without impacting on these risk scores. Hypertrophic cardiomyopathy, with its apical variant, is such a condition. We present a case of a patient with apical hypertrophic cardiomyopathy and atrial fibrillation who suffered dire thromboembolic consequences despite a reassuringly low CHA2DS2-VASc score and suggest that this scoring system is modified to incorporate the thromboembolic risk inherent to certain cardiomyopathies irrespective of impairment of left ventricular systolic dysfunction or clinical heart failure.

  4. Effect of Institutional Experience on Outcomes of Alcohol Septal Ablation for Hypertrophic Obstructive Cardiomyopathy

    DEFF Research Database (Denmark)

    Veselka, Josef; Faber, Lothar; Jensen, Morten Kvistholm

    2018-01-01

    BACKGROUND: The current American College of Cardiology Foundation/American Heart Association guidelines on hypertrophic cardiomyopathy state that institutional experience is a key determinant of successful outcomes and lower complication rates of alcohol septal ablation (ASA). The aim of this study...... was to evaluate the safety and efficacy of ASA according to institutional experience with the procedure. METHODS: We retrospectively evaluated 1310 patients with symptomatic obstructive hypertrophic cardiomyopathy who underwent ASA and were divided into 2 groups. The first-50 group consisted of the first......), and a probability of repeated septal reduction therapy (P = 0.03). CONCLUSIONS: An institutional experience of > 50 ASA procedures was associated with a lower occurrence of ASA complications, better cardiovascular survival, better hemodynamic and clinical effect, and less need for repeated septal reduction therapy....

  5. Progressive Hypertrophic Genital Herpes in an HIV-Infected Woman despite Immune Recovery on Antiretroviral Therapy

    Directory of Open Access Journals (Sweden)

    Mark H. Yudin

    2008-01-01

    Full Text Available Most HIV-infected individuals are coinfected by Herpes simplex virus type 2 (HSV-2. HSV-2 reactivates more frequently in HIV-coinfected individuals with advanced immunosuppression, and may have very unusual clinical presentations, including hypertrophic genital lesions. We report the case of a progressive, hypertrophic HSV-2 lesion in an HIV-coinfected woman, despite near-complete immune restoration on antiretroviral therapy for up to three years. In this case, there was prompt response to topical imiquimod. The immunopathogenesis and clinical presentation of HSV-2 disease in HIV-coinfected individuals are reviewed, with a focus on potential mechanisms for persistent disease despite apparent immune reconstitution. HIV-infected individuals and their care providers should be aware that HSV-2 may cause atypical disease even in the context of near-comlpete immune reconstitution on HAART.

  6. Video capsule endoscopy and CT enterography in diagnosing adult hypertrophic pyloric stenosis.

    Science.gov (United States)

    Gurvits, Grigoriy E; Tan, Amy; Volkov, Dmitri

    2013-10-07

    Primary adult hypertrophic pyloric stenosis is a rare but important cause of gastric outlet obstruction that may be misdiagnosed as idiopathic gastroparesis. Clinically, patients present with early satiety, abdominal fullness, nausea, epigastric discomfort and eructation. Permanent gastric retention of a video capsule endoscope is diagnostic in differentiating between the two diseases, in the absence of an organic gastric outlet obstruction. This case presents the longest video capsule retention in the medical literature to date. It is also the first case report of adult hypertrophic pyloric stenosis diagnosed with video capsule endoscopy or a computed tomography scan. Finally, an unusual "plugging" of the gastric outlet with free floating capsule has an augmented effect on disease physiology and on patient's symptoms.

  7. Hypertrophic pachymeningitis: Current criteria for diagnosis and differentiation (Clinical case and review of literature

    Directory of Open Access Journals (Sweden)

    E. G. Mendelevich

    2015-01-01

    Full Text Available The paper describes a 44-year-old male patient with an about 6-year history of hypertrophic pachymeningitis. The major clinical symptoms were characterized by headache, exophthalmos, and blindness in one eye. The data for differential diagnosis of the disease are given. The current literature on the clinical manifestations of hypertrophic pachymeningitis, its differential diagnosis, and the results of magnetic resonance imaging (MRI is reviewed. Diagnostic difficulties at the stage of a clinical observation are due to the nonspecificity of neurological manifestations and the need for a comprehensive examination to detect a somatic disease. MRI can diagnose the disease-specific phenomenon of damage to the meninges, which calls for further careful differentiation. Clinicians must be familiar with alternative differential diagnosis, as a rapid specific therapeutic approach will help avoid long-term or irreversible neurological complications.

  8. Development of detonations in degenerate stars

    Science.gov (United States)

    Blinnikov, S. I.; Khokhlov, A. M.

    1986-04-01

    It is now widely believed that thermal instability at the center of a carbon-oxygen white dwarf will produce a deflagration wave. In this paper, numerical analysis of the preoutburst conditions near the center of a C-O white dwarf as well as of the three stages of the thermonuclear explosion of the star (i.e., the stages of spontaneous burning front, shock formation, and postshock burning and shock amplification) is presented. It is demonstrated that when a degenerate C-O star explodes as a supernova, thermal instability may cause the thermonuclear burning front to develop into a detonation (rather than a deflagration) regime.

  9. Hypertrophic Effects of Concentric vs. Eccentric Muscle Actions: A Systematic Review and Meta-analysis.

    Science.gov (United States)

    Schoenfeld, Brad J; Ogborn, Dan I; Vigotsky, Andrew D; Franchi, Martino V; Krieger, James W

    2017-09-01

    Schoenfeld, BJ, Ogborn, DI, Vigotsky, AD, Franchi, MV, and Krieger, JW. Hypertrophic effects of concentric vs. eccentric muscle actions: A systematic review and meta-analysis. J Strength Cond Res 31(9): 2599-2608, 2017-Controversy exists as to whether different dynamic muscle actions produce divergent hypertrophic responses. The purpose of this paper was to conduct a systematic review and meta-analysis of randomized controlled trials comparing the hypertrophic effects of concentric vs. eccentric training in healthy adults after regimented resistance training (RT). Studies were deemed eligible for inclusion if they met the following criteria: (a) were an experimental trial published in an English-language refereed journal; (b) directly compared concentric and eccentric actions without the use of external implements (i.e., blood pressure cuffs) and all other RT variables equivalent; (c) measured morphologic changes using biopsy, imaging (magnetic resonance imaging, computerized tomography, or ultrasound), bioelectrical impedance, and/or densitometry; (d) had a minimum duration of 6 weeks; and (e) used human participants without musculoskeletal injury or any health condition that could directly, or through the medications associated with the management of said condition, be expected to impact the hypertrophic response to resistance exercise. A systematic literature search determined that 15 studies met inclusion criteria. Results showed that eccentric muscle actions resulted in a greater effect size (ES) compared with concentric actions, but results did not reach statistical significance (ES difference = 0.25 ± 0.13; 95% confidence interval: -0.03 to 0.52; p = 0.076). The mean percent change in muscle growth across studies favored eccentric compared with concentric actions (10.0% vs. 6.8, respectively). The findings indicate the importance of including eccentric and concentric actions in a hypertrophy-oriented RT program, as both have shown to be effective in

  10. A Systematic Review on the Effect of Mechanical Stretch on Hypertrophic Scars after Burn Injuries

    Directory of Open Access Journals (Sweden)

    Yu-ting Zhang

    2017-06-01

    Conclusion: From extensive literature search, there was no strong evidence indicating the positive effect of mechanical stretch using stretching exercise, massage, or splinting on hypertrophic scars. A firm conclusion cannot be drawn for the discrepancy of outcome measures and varied effectiveness. Most of the included studies lacked objective evaluation or control group for comparison. Further high quality studies with larger sample size and using standardized measurements are needed.

  11. Nd:YAG Laser Treatment of Keloids and Hypertrophic Scar/span>s

    Science.gov (United States)

    Akaishi, Satoshi; Koike, Sachiko; Dohi, Teruyuki; Kobe, Kyoko; Hyakusoku, Hiko; Ogawa, Rei

    2012-01-01

    Pathological cutaneous scars such as keloids and hypertrophic scar/span>s (HSs) are characterized by a diffuse redness that is caused by the overgrowth of capillary vessels due to chronic inflammation. Our group has been using long-pulsed, 1064-nm Nd:YAG laser in noncontact mode with low fluence and a submillisecond pulse duration to treat keloids and hypertrophic scar/span>s since 2006 with satisfactory results. The present study examined the efficacy of this approach in 22 Japanese patients with keloids (n = 16) or hypertrophic scar/span>s (n = 6) who were treated every 3 to 4 weeks. Treatment settings were as follows: 5 mm spot size diameter; 14 J/cm2 energy density; 300 μs exposure time per pulse; and 10 Hz repetition rate. The responses of the pathological scar/span>s to the treatment were assessed by measuring their erythema, hypertrophy, hardness, itching, and pain or tenderness. Moreover, skin samples from 3 volunteer patients were subjected to histological evaluation and 5 patients underwent thermography during therapy. The average total scar assessment score dropped from 9.86 to 6.34. Hematoxylin and eosin staining and Elastica Masson-Goldner staining showed that laser treatment structurally changed the tissue collagen. This influence reached a depth of 0.5 to 1 mm. Electron microscopy revealed plasma protein leakage, proteoglycan particles, and a change in the collagen fiber fascicles. Further analyses revealed that noncontact mode Nd:YAG laser treatment is highly effective for keloids and hypertrophic scar/span>s regardless of patient age, the origin and multiplicity of scarring, the location of the scar(s), or the tension on the scar. PMID:22259645

  12. Multiple Gene Variants in Hypertrophic Cardiomyopathy in the Era of Next-Generation Sequencing.

    Science.gov (United States)

    Burns, Charlotte; Bagnall, Richard D; Lam, Lien; Semsarian, Christopher; Ingles, Jodie

    2017-08-01

    Multiple likely pathogenic/pathogenic (LP/P; ≥2) variants in patients with hypertrophic cardiomyopathy were described 10 years ago with a prevalence of 5%. We sought to re-examine the significance of multiple rare variants in patients with hypertrophic cardiomyopathy in the setting of comprehensive and targeted panels. Of 758 hypertrophic cardiomyopathy probands, we included 382 with ≥45 cardiomyopathy genes screened. There were 224 (59%) with ≥1 rare variant (allele frequency ≤0.02%). Variants were analyzed using varying sized gene panels to represent comprehensive or targeted testing. Based on a 45-gene panel, 127 (33%) had a LP/P variant, 139 (36%) had variants of uncertain significance, and 66 (17%) had multiple rare variants. A targeted 8-gene panel yielded 125 (32%) LP/P variants, 52 (14%) variants of uncertain significance, and 14 (4%) had multiple rare variants. No proband had 2 LP/P variants. Including affected family members (total n=412), cluster-adjusted analyses identified a phenotype effect, with younger age (odds ratio, 0.95; 95% confidence interval, 0.92-0.98; P =0.004) and family history of sudden cardiac death (odds ratio, 3.5; 95% confidence interval, 1.3-9.9; P =0.02) significantly more likely in multiple versus single variant patients when considering an 8-gene panel but not larger panels. Those with multiple variants had worse event-free survival from all-cause death, cardiac transplantation, and cardiac arrest (log-rank P =0.008). No proband had multiple LP/P variants in contrast to previous reports. However, multiple rare variants regardless of classification were seen in 4% and contributed to earlier disease onset and cardiac events. Our findings support a cumulative variant hypothesis in hypertrophic cardiomyopathy. © 2017 American Heart Association, Inc.

  13. [Utility of cardiovascular magnetic resonance in hypertrophic cardiomyopathy: when is it superior to echocardiography?].

    Science.gov (United States)

    Kammoun, I; Marrakchi, S; Zidi, A; Ibn ElHaj, Z; Naccache, S; Ben Amara, W; Jebri, F; Bennour, E; Kachboura, S

    2015-02-01

    The diagnosis of hypertrophic cardiomyopathy is usually established by echocardiography. Recently, there has been greatly increased use of cardiac magnetic resonance (CMR) because of its precise determination of myocardial anatomy and the depiction of myocardial fibrosis. In this review, we describe the role of echocardiography and magnetic resonance in the assessment of this complex disease. In conclusion, there is a complementarity between cardiovascular magnetic resonance imaging and echocardiography for the diagnosis and the management of HCM. Copyright © 2014. Published by Elsevier SAS.

  14. Lack of reliable clinical predictors to identify obstructive sleep apnea in patients with hypertrophic cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Flávia B. Nerbass

    2013-07-01

    Full Text Available OBJECTIVE: Obstructive sleep apnea is common among patients with hypertrophic cardiomyopathy and may contribute to poor cardiovascular outcomes. However, obstructive sleep apnea is largely unrecognized in this population. We sought to identify the clinical predictors of obstructive sleep apnea among patients with hypertrophic cardiomyopathy. METHODS: Consecutive patients with hypertrophic cardiomyopathy were recruited from a tertiary University Hospital and were evaluated using validated sleep questionnaires (Berlin and Epworth and overnight portable monitoring. Ninety patients (males, 51%; age, 46±15 years; body mass index, 26.6±4.9 kg/m2 were included, and obstructive sleep apnea (respiratory disturbance index ≥15 events/h was present in 37 patients (41%. RESULTS: Compared with the patients without obstructive sleep apnea, patients with obstructive sleep apnea were older and had higher body mass index, larger waist circumference, larger neck circumference, and higher prevalence of atrial fibrillation. Excessive daytime sleepiness (Epworth scale was low and similar in the patients with and without obstructive sleep apnea, respectively. The only predictors of obstructive sleep apnea (using a logistic regression analysis were age ≥45 years (odds ratio [OR], 4.46; 95% confidence interval [CI 95%], 1.47-13.54; p = 0.008 and the presence of atrial fibrillation [OR, 5.37; CI 95%, 1.43-20.12; p = 0.013]. CONCLUSION: Consistent clinical predictors of obstructive sleep apnea are lacking for patients with hypertrophic cardiomyopathy, which suggests that objective sleep evaluations should be considered in this population, particularly among elderly patients with atrial fibrillation.

  15. Pattern and degree of left ventricular remodeling following a tailored surgical approach for hypertrophic obstructive cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Ismail El-Hamamsy

    2012-07-01

    Full Text Available Background The role of a tailored surgical approach for hypertrophic cardiomyopathy (HCM on regional ventricular remodelling remains unknown. The aims of this study were to evaluate the pattern, extent and functional impact of regional ventricular remodelling after a tailored surgical approach. Conclusion Following a tailored surgical relief of outflow obstruction for HCM, there is a marked regional reverse LV remodelling. These changes could have a significant impact on overall ventricular dynamics and function.

  16. Papillary thyroid carcinoma treated with radiofrequency ablation in a patient with hypertrophic cardiomyopathy: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Sun, Jian Yi; Liu, Xiao Sun; Zhang, Qing; Hong, Yan Yun; Song, Bin; Teng, Xiao Dong; Yu, Ji Ren [The First Affiliated Hospital, Medical College, Zhejiang University, Hangzhou (China)

    2016-07-15

    Standard therapy has not been established for thyroid cancer when a thyroidectomy is contraindicated due to systemic disease. Herein, we reported a patient who had hypertrophic cardiomyopathy and papillary thyroid carcinoma treated by radiofrequency ablation because of inability to tolerate a thyroidectomy. Radiofrequency ablation can be used to treat thyroid cancer when surgery is not feasible, although the long-term outcome needs further observation.

  17. Asymmetric left ventricular hypertrophy associated with morbid obesity mimicking familial hypertrophic cardiomyopathy.

    Science.gov (United States)

    Wong, Raymond Ching-Chiew; Tan, Kong Bing

    2014-12-01

    Asymmetric septal hypertrophy with systolic anterior motion of the mitral valve is frequently a phenotypic, but not pathognomonic, expression of genetic hypertrophic cardiomyopathy (HCM) with or without obstruction. It can, however, be associated nonspecifically with other forms of increased left ventricular (LV) afterload. We herein report the case of a young man with obesity cardiomyopathy and heart failure who presented with asymmetric septal hypertrophy and marked LV hypertrophy, and endomyocardial biopsy ruled out genetic HCM.

  18. Lack of reliable clinical predictors to identify obstructive sleep apnea in patients with hypertrophic cardiomyopathy

    Science.gov (United States)

    Nerbass, Flávia B.; Pedrosa, Rodrigo P.; Genta, Pedro R.; Antunes, Murillo O.; Arteaga-Fernández, Edmundo; Drager, Luciano F.; Lorenzi-Filho, Geraldo

    2013-01-01

    OBJECTIVE: Obstructive sleep apnea is common among patients with hypertrophic cardiomyopathy and may contribute to poor cardiovascular outcomes. However, obstructive sleep apnea is largely unrecognized in this population. We sought to identify the clinical predictors of obstructive sleep apnea among patients with hypertrophic cardiomyopathy. METHODS: Consecutive patients with hypertrophic cardiomyopathy were recruited from a tertiary University Hospital and were evaluated using validated sleep questionnaires (Berlin and Epworth) and overnight portable monitoring. Ninety patients (males, 51%; age, 46±15 years; body mass index, 26.6±4.9 kg/m2) were included, and obstructive sleep apnea (respiratory disturbance index ≥15 events/h) was present in 37 patients (41%). RESULTS: Compared with the patients without obstructive sleep apnea, patients with obstructive sleep apnea were older and had higher body mass index, larger waist circumference, larger neck circumference, and higher prevalence of atrial fibrillation. Excessive daytime sleepiness (Epworth scale) was low and similar in the patients with and without obstructive sleep apnea, respectively. The only predictors of obstructive sleep apnea (using a logistic regression analysis) were age ≥45 years (odds ratio [OR], 4.46; 95% confidence interval [CI 95%], 1.47–13.54; p = 0.008) and the presence of atrial fibrillation [OR, 5.37; CI 95%, 1.43–20.12; p = 0.013]. CONCLUSION: Consistent clinical predictors of obstructive sleep apnea are lacking for patients with hypertrophic cardiomyopathy, which suggests that objective sleep evaluations should be considered in this population, particularly among elderly patients with atrial fibrillation. PMID:23917665

  19. Papillary Thyroid Carcinoma Treated with Radiofrequency Ablation in a Patient with Hypertrophic Cardiomyopathy: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Sun, Jianyi; Liu, Xiaosun; Zhang, Qing; Hong, Yanyun; Song, Bin [Department of Gastrointestinal and Thyroid Surgery, The First Affiliated Hospital, Medical College, Zhejiang University, Hangzhou 310003 (China); Teng, Xiaodong [Department of Pathology, The First Affiliated Hospital, Medical College, Zhejiang University, Hangzhou 310003 (China); Yu, Jiren [Department of Gastrointestinal and Thyroid Surgery, The First Affiliated Hospital, Medical College, Zhejiang University, Hangzhou 310003 (China)

    2016-11-01

    Standard therapy has not been established for thyroid cancer when a thyroidectomy is contraindicated due to systemic disease. Herein, we reported a patient who had hypertrophic cardiomyopathy and papillary thyroid carcinoma treated by radiofrequency ablation because of inability to tolerate a thyroidectomy. Radiofrequency ablation can be used to treat thyroid cancer when surgery is not feasible, although the long-term outcome needs further observation.

  20. Undifferentiated pulmonary adenocarcinoma of clear cells associated to hypertrophic osteopathy in a dog

    OpenAIRE

    Rossetto, Victor José Vieira [UNESP; Rahal, Sheila Canevese [UNESP; Pardini, Luciana Moura Campos [UNESP; Fabris, Viciany Erique [UNESP; Mamprim, Maria Jaqueline [UNESP; Ribeiro, Sergio Marrone [UNESP

    2015-01-01

    Background: Most of the primary pulmonary tumors in dogs are malignant and from epithelial origin, being bronchioalveolar tumors more prevalent. Adenocarcinoma of clear cells, however, is a very rare pulmonary tumor and its origin is still unknown. It is related to several clinical abnormalities, including hypertrophic osteopathy, an unusual paraneoplastic syndrome characterized by a periosteal reaction along the shaft of long bones. Because of the unusual presentation of the pulmonary adenoc...

  1. Intrauterine Treatment of a Fetus with Familial Hypertrophic Cardiomyopathy Secondary to MYH7 Mutation.

    Science.gov (United States)

    Hill, Meghan G; Sekhon, Mehtab K; Reed, Kathryn L; Anderson, Caroline F; Borjon, Nydia D; Tardiff, Jil C; Barber, Brent J

    2015-12-01

    There is no clear consensus on optimal management of fetuses affected by familial hypertrophic cardiomyopathy (HCM). Intrauterine treatment of the condition has not been attempted in any standardized fashion. We report the case of a fetus treated by maternal propranolol during the third trimester after septal hypertrophy and diastolic dysfunction was diagnosed on fetal echocardiogram. The pregnancy went successfully to term, and fetal septal hypertrophy was noted to improve prior to delivery.

  2. A Case Report of Percutaneous Transluminal Septal Myocardial Ablation in Patients with Hypertrophic Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    M.H. Namazi

    2004-10-01

    Full Text Available A number of patients with severe obstruction due to hypertrophic cardiomyopathy have derived benefit at least over the short-term from inventional infarction of a portion of the interventricular septum by the infusion of alcohol into a selectively catheterized septal artery , with reduction of the outflow gradient and improvement in symptoms .This paper contains successful TASH on a symptomatic patient with high LVOT gradient and methods and complications.

  3. Burden of Recurrent and Ancestral Mutations in Families With Hypertrophic Cardiomyopathy.

    Science.gov (United States)

    Ross, Samantha Barratt; Bagnall, Richard D; Ingles, Jodie; Van Tintelen, J Peter; Semsarian, Christopher

    2017-06-01

    Hypertrophic cardiomyopathy is a genetically heterogeneous myocardial disease with >1000 causal variants identified. Nonunique variants account for disease in many families. We sought to characterize nonunique variants in Australian families and determine whether they arise from common ancestral mutations or recurrent mutation events. Genetic test results of 467 index patients from apparently unrelated families with hypertrophic cardiomyopathy were evaluated. Causal variants were found in 185 of 467 (40%) families. Nonunique variants accounted for 122 of 185 (66%) families. The most common single genetic cause of hypertrophic cardiomyopathy is the recurrent MYBPC3 (myosin-binding protein-C) variant c.1504C>T, p.Arg502Trp, which was found in 13 of 185 (7%) families with a causal variant identified. Thirteen variants in MYBPC3 and MYH7 (myosin heavy chain 7) were each identified >3 times and accounted for 78 of 185 (42%) hypertrophic cardiomyopathy families with a causal variant. Haplotype analysis of these 13 variants was performed on 126 individuals from 70 Australian families, and 11 variants arose through recurrent mutation events. Two variants, MYBPC3 c.1928-2A>G and MYH7 c.2681A>G, p.Glu894Gly, were found on 1 haplotype in 6 families each, supportive of a single mutation event inherited from a common ancestor. The majority of families with a causal variant identified have a nonunique variant. Discovery of the genetic origins of human disease forms a fundamental basis for improved understanding of disease pathogenesis and phenotype development. © 2017 American Heart Association, Inc.

  4. Improved survival with amiodarone in patients with hypertrophic cardiomyopathy and ventricular tachycardia.

    OpenAIRE

    McKenna, W. J.; Oakley, C M; Krikler, D. M.; Goodwin, J. F.

    1985-01-01

    The effect of amiodarone on survival was assessed in patients with hypertrophic cardiomyopathy and ventricular tachycardia in a drug trial with historical controls. During 1976 and 1977, 24 hour (seven) or 48 hour (79) electrocardiographic monitoring was performed in 86 consecutive patients; 24 had ventricular tachycardia and received conventional antiarrhythmic agents. Nineteen clinical, echocardiographic, and haemodynamic features were assessed. Seven patients died suddenly during follow up...

  5. Bilateral brachial plexus blocks in a patient of hypertrophic obstructive cardiomyopathy with hypertensive crisis

    OpenAIRE

    Bhat Pai, Rohini V; Hegde, Harihar V.; MCB, Santhosh; S Roopa; Shrinivas S Deshpande; P Raghavendra Rao

    2013-01-01

    Hypertrophic obstructive cardiomyopathy (HOCM) is a challenge to anesthesiologists due to the complex pathophysiology involved and various perioperative complications associated with it. We present a 50-year-old man, a known case of HOCM, who successfully underwent emergency haemostasis, and debridement of the traumatically amputated right upper limb and the contused lacerated wound on the left forearm under bilateral brachial plexus blocks. His co-morbidities included hypertension (in hypert...

  6. Family-Specific Degenerate Primer Design: A Tool to Design Consensus Degenerated Oligonucleotides

    Science.gov (United States)

    Goñi, Sandra Elizabeth; Lozano, Mario Enrique

    2013-01-01

    Designing degenerate PCR primers for templates of unknown nucleotide sequence may be a very difficult task. In this paper, we present a new method to design degenerate primers, implemented in family-specific degenerate primer design (FAS-DPD) computer software, for which the starting point is a multiple alignment of related amino acids or nucleotide sequences. To assess their efficiency, four different genome collections were used, covering a wide range of genomic lengths: Arenavirus (10 × 104 nucleotides), Baculovirus (0.9 × 105 to 1.8 × 105 bp), Lactobacillus sp. (1 × 106 to 2 × 106 bp), and Pseudomonas sp. (4 × 106 to 7 × 106 bp). In each case, FAS-DPD designed primers were tested computationally to measure specificity. Designed primers for Arenavirus and Baculovirus were tested experimentally. The method presented here is useful for designing degenerate primers on collections of related protein sequences, allowing detection of new family members. PMID:23533783

  7. The PI3K pathway regulates endochondral bone growth through control of hypertrophic chondrocyte differentiation

    Directory of Open Access Journals (Sweden)

    Beier Frank

    2008-04-01

    Full Text Available Abstract Background The majority of our bones develop through the process of endochondral ossification that involves chondrocyte proliferation and hypertrophic differentiation in the cartilage growth plate. A large number of growth factors and hormones have been implicated in the regulation of growth plate biology, however, less is known about the intracellular signaling pathways involved. PI3K/Akt has been identified as a major regulator of cellular proliferation, differentiation and death in multiple cell types. Results and Discussion Employing an organ culture system of embryonic mouse tibiae and LY294002, a pharmacological inhibitor of PI3K, we show that inhibition of the pathway results in significant growth reduction, demonstrating that PI3K is required for normal endochondral bone growth in vitro. PI3K inhibition reduces the length of the proliferating and particularly of the hypertrophic zone. Studies with organ cultures and primary chondrocytes in micromass culture show delayed hypertrophic differentiation of chondrocytes and increased apoptosis in the presence of LY294002. Surprisingly, PI3K inhibition had no strong effect on IGF1-induced bone growth, but partially blocked the anabolic effects of C-type natriuretic peptide. Conclusion Our data demonstrate an essential role of PI3K signaling in chondrocyte differentiation and as a consequence of this, in the endochondral bone growth process.

  8. Topical Silicone Sheet Application in the Treatment of Hypertrophic Scar/span>s and Keloids

    Science.gov (United States)

    Westra, Iris; Pham, Hth

    2016-01-01

    Objective: Since the early 1980s, topical silicone sheets have been used in the treatment of hypertrophic scar/span>s and keloids.This study aimed to determine the optimal duration and application of these sheets. Design: multi-centered therapeutic study. Setting and participants: A total of 224 patients were included in this study; 205 patients with hypertrophic scar/span>s and 19 patients with keloids. Patients received treatment with a topical silicone sheet. Treated scars varied in age, ranging from two weeks to 62 years and treatment time ranged from one month to 16 months. Assessment of the scars was performed by the use of standardized study forms and digital photography. Measurements: Skin therapists objectively assessed the scars on its color, thickness, and elasticity. Patients themselves subjectively assessed their perception of their scar and their experience with the usage of the topical silicone sheet. Results: After applying the topical silicone sheet, all scars, regardless of type of scar and maturity, improved significantly in color, thickness, and elasticity. Conclusion: In this study, treatment with the topical silicone sheet showed significant improvement on both hypertrophic scar/span>s and keloids. Best results were reached when the silicone sheet was applied at least four hours per day. PMID:27847546

  9. Diagnostic Value of Histological and Microbiological Screening in Etiopathogenesis of Recurrent and Hypertrophic Tonsillitis

    Directory of Open Access Journals (Sweden)

    Uldis Viesturs

    2006-12-01

    Full Text Available Interest in the mechanisms and causes of recurrent tonsillitis is considerable. CD4+CD25+ T-lymphocytes have an important role in the maintenance of immunological tolerance. The aim of our research was to compare the diagnostic value of palatine tonsils histological and microbiological screening in patients with hypertrophic and recurrent tonsillitis. 14 patients with hypertrophic and 10 patients with chronic tonsillitis undergoing tonsillectomy were enrolled in the study. Rapid diagnosis of adenovirus, parainfluenza, influenza A and B, and respiratory syncytial virus infection was made before tonsillectomy by viral antigen detection using the immunofluorescence procedure from tonsils. Herpes simplex and cytomegaloviruses DNA were detected by the polymerase chain reaction. Samples for bacteriological studies were collected using a cotton swab. Immunohistochemical methods were used to evaluate S-100 and TGF-beta1 expression. The obtained results showed that patients with recurrent tonsillitis had less S-100 and TGF-beta1 positive cells in parafollicular regions compared to patients with hypertrophic tonsillitis. In both groups, tonsils were colonized predominantly by gram-positive microorganisms and adenovirus (36% of cases. However, in patients with recurrent tonsillitis, associations of gram-positive, gram-negative bacteria and viruses (40% of cases were observed. To conclude, recurrent tonsillitis is characterized by the breakdown of the immunological tolerance to oral microflora.

  10. Conditional deletion of Tgfbr2 in hypertrophic chondrocytes delays terminal chondrocyte differentiation.

    Science.gov (United States)

    Sueyoshi, Tatsuya; Yamamoto, Koji; Akiyama, Haruhiko

    2012-07-01

    Transforming growth factor β (Tgfb) signaling plays an important role in endochondral ossification. Previous studies of mice in which the Tgfb type II receptor gene (Tgfbr2) was deleted in the limb bud mesenchymal cells or differentiated chondrocytes showed defects in the development of the long bones or the axial skeleton, respectively. Here, we generated mouse embryos in which the Tgfbr2 gene was ablated in hypertrophic chondrocytes. These mice exhibited delays in both the hypertrophic conversion of proliferating chondrocytes and the subsequent terminal chondrocyte differentiation. The expression domains of Col10a1, Matrix metalloproteinase 13, and Osteopontin were small, and the expression of Vascular endothelial growth factor and Platelet endothelial cell adhesion molecule was downregulated. The calcification of the bone collar in the mutant mice was markedly delayed and the periosteum was thin, possibly because of the downregulation of Indian hedgehog expression. We conclude that Tgfb signaling in hypertrophic chondrocytes positively regulates terminal chondrocyte differentiation, angiogenesis in calcified cartilage, and osteogenesis in the bone collar, at least partly through Indian hedgehog signaling in vivo. Copyright © 2012 International Society of Matrix Biology. Published by Elsevier B.V. All rights reserved.

  11. Evaluation of hypertrophic obstructive cardiomyopathy by exercise stress echocardiography. New methodology.

    Science.gov (United States)

    Cotrim, Carlos; Loureiro, Maria José; Simões, Otília; Miranda, Rita; Cordeiro, Pedro; Ialá, Mário; Matias, Carla; João, Isabel; Carrageta, Manuel

    2005-11-01

    Exercise echocardiography with image acquisition during treadmill exercise is the most common form of stress testing used in our department. The usual evaluation of patients with hypertrophic obstructive cardiomyopathy consists of serial echocardiographic studies at rest. The purpose of this study was to evaluate intraventricular gradients during exercise echocardiography in patients with a diagnosis of hypertrophic obstructive cardiomyopathy. We studied 13 patients, 8 male, mean age 56 +/- 9 years Intraventricular gradients were measured using continuous wave Doppler; two evaluations were performed at rest: one in left lateral decubitus and the other in orthostatic position after one minute in this position. The patients then underwent a treadmill exercise test using the modified Bruce protocol, during which intraventricular gradients were measured at peak exercise. Finally, a further measurement was taken in the first 90 seconds of the recovery period in left lateral decubitus. The intraventricular gradient in left lateral decubitus was 54 +/- 29 mmHg; in orthostatic position 69 +/- 30 mmHg (p happens during effort or therefore during these patients' daily activities. This type of evaluation can help us to better understand the pathophysiology of patients with a diagnosis of hypertrophic obstructive cardiomyopathy and to optimize treatment.

  12. Aberrant α-Adrenergic Hypertrophic Response in Cardiomyocytes from Human Induced Pluripotent Cells

    Directory of Open Access Journals (Sweden)

    Gabor Földes

    2014-11-01

    Full Text Available Cardiomyocytes from human embryonic stem cells (hESC-CMs and induced pluripotent stem cells (hiPSC-CMs represent new models for drug discovery. Although hypertrophy is a high-priority target, we found that hiPSC-CMs were systematically unresponsive to hypertrophic signals such as the α-adrenoceptor (αAR agonist phenylephrine (PE compared to hESC-CMs. We investigated signaling at multiple levels to understand the underlying mechanism of this differential responsiveness. The expression of the normal α1AR gene, ADRA1A, was reversibly silenced during differentiation, accompanied by ADRA1B upregulation in either cell type. ADRA1B signaling was intact in hESC-CMs, but not in hiPSC-CMs. We observed an increased tonic activity of inhibitory kinase pathways in hiPSC-CMs, and inhibition of antihypertrophic kinases revealed hypertrophic increases. There is tonic suppression of cell growth in hiPSC-CMs, but not hESC-CMs, limiting their use in investigation of hypertrophic signaling. These data raise questions regarding the hiPSC-CM as a valid model for certain aspects of cardiac disease.

  13. Treatment of hypertrophic scars and keloids using intense pulsed light (IPL).

    Science.gov (United States)

    Erol, O Onur; Gurlek, Ali; Agaoglu, Galip; Topcuoglu, Ela; Oz, Hayat

    2008-11-01

    Keloids and hypertrophic scars are extremely disturbing to patients, both physically and psychologically. This study prospectively assessed the safety and efficacy of intense pulsed light (IPL) on scars originating from burns, trauma, surgery, and acne. Hypertrophic scars in 109 patients, originating from surgical incisions (n = 55), traumatic cuts (traffic accidents) (n = 24), acne scars (n = 6), keloids (n = 5), and burns (n = 19), were treated using an IPL Quantum device. Treatment was administered at 2-4-week intervals, and patients received an average of 8 treatments (range = 6-24). Using digital photographs, Changes in scar appearance were assessed by two physicians who were blinded to the study patients and treatments. The photographs were graded on a scale of 0 to 4 (none, minimal, moderate, good, excellent) for improvement in overall clinical appearance and reduction in height, erythema, and hardness. An overall clinical improvement in the appearance of scars and reductions in height, erythema, and hardness were seen in the majority of the patients (92.5%). Improvement was excellent in 31.2% of the patients, good in 25.7%, moderate in 34%, and minimal in 9.1%. Over half the patients had good or excellent improvement. In the preventive IPL treatment group, 65% had good to excellent improvement in clinical appearance. Patient satisfaction was very high. This study suggests that IPL is effective not only in improving the appearance of hypertrophic scars and keloids regardless of their origin, but also in reducing the height, redness, and hardness of scars.

  14. The follow-up of progressive hypertrophic cardiomyopathy using magnetic resonance rotating frame relaxation times.

    Science.gov (United States)

    Khan, Muhammad Arsalan; Laakso, Hanne; Laidinen, Svetlana; Kettunen, Sanna; Heikura, Tommi; Ylä-Herttuala, Seppo; Liimatainen, Timo

    2017-12-15

    Magnetic resonance rotating frame relaxation times are an alternative non-contrast agent choice for the diagnosis of chronic myocardial infarct. Fibrosis typically occurs in progressive hypertrophic cardiomyopathy. Fibrosis has been imaged in myocardial infarcted tissue using rotating frame relaxation times, which provides the possibility to follow up progressive cardiomyopathy without contrast agents. Mild and severe left ventricular hypertrophy were induced in mice by transverse aortic constriction, and the longitudinal rotating frame relaxation times (T1ρ ) and relaxation along the fictitious field (TRAFF2 , TRAFF3 ) were measured at 5, 10, 24, 62 and 89 days after transverse aortic constriction in vivo. Myocardial fibrosis was verified using Masson's trichrome staining. Increases in the relative relaxation time differences of T1ρ , together with TRAFF2 and TRAFF3 , between fibrotic and remote tissues over time were observed. Furthermore, TRAFF2 and TRAFF3 showed higher relaxation times overall in fibrotic tissue than T1ρ . Relaxation time differences were highly correlated with an excess of histologically verified fibrosis. We found that TRAFF2 and TRAFF3 are more sensitive than T1ρ to hypertrophic cardiomyopathy-related tissue changes and can serve as non-invasive diagnostic magnetic resonance imaging markers to follow up the mouse model of progressive hypertrophic cardiomyopathy. Copyright © 2017 John Wiley & Sons, Ltd.

  15. Quantum Degenerate Gases of Atomic Strontium

    Science.gov (United States)

    Killian, T. C.

    2010-03-01

    This talk will describe the production and properties of a Bose-Einstein condensate of ^84Sr and a quantum degenerate mixture of ^87Sr (fermion) and ^88Sr (boson). ^88Sr has a small negative scattering length leading to a maximum condensate size for our trapping conditions of about 10^4 atoms. ^87Sr is used to sympathetically cool ^88Sr, but it is also of interest for study of quantum degenerate Fermi gases because it has a large nuclear spin (I=9/2). Alkaline-earth metal atoms and atoms with similar electronic structure are of interest for quantum computing proposals, cold collision studies, and investigation of quantum fluids. There are a wealth of isotopes that allow mass-tuning of interactions and creation of various quantum mixtures. The two-valence electrons lead to a singlet ground state and narrow intercombination transitions to metastable triplet states, offering the promise of low-loss optical Feshbach resonances for manipulating scattering lengths. Fermions often have large nuclear spin, which is decoupled from electronic degrees of freedom and leads to a large degree of symmetry and degeneracy in the interaction Hamiltonian. Work done in collaboration with Y.N. Martinez de Escobar, P.G. Mickelson, M. Yan, B.J. DeSalvo, and S.B. Nagel, Rice University.

  16. Progranulin in frontotemporal lobar degeneration and neuroinflammation

    Directory of Open Access Journals (Sweden)

    Hutton Michael L

    2007-02-01

    Full Text Available Abstract Progranulin (PGRN is a pleiotropic protein that has gained the attention of the neuroscience community with recent discoveries of mutations in the gene for PGRN that cause frontotemporal lobar degeneration (FTLD. Pathogenic mutations in PGRN result in null alleles, and the disease is likely the result of haploinsufficiency. Little is known about the normal function of PGRN in the central nervous system apart from a role in brain development. It is expressed by microglia and neurons. In the periphery, PGRN is involved in wound repair and inflammation. High PGRN expression has been associated with more aggressive growth of various tumors. The properties of full length PGRN are distinct from those of proteolytically derived peptides, referred to as granulins (GRNs. While PGRN has trophic properties, GRNs are more akin to inflammatory mediators such as cytokines. Loss of the neurotrophic properties of PGRN may play a role in selective neuronal degeneration in FTLD, but neuroinflammation may also be important. Gene expression studies suggest that PGRN is up-regulated in a variety of neuroinflammatory conditions, and increased PGRN expression by microglia may play a pivotal role in the response to brain injury, neuroinflammation and neurodegeneration.

  17. Metabolic anatomy of paraneoplastic cerebellar degeneration

    Energy Technology Data Exchange (ETDEWEB)

    Anderson, N.E.; Posner, J.B.; Sidtis, J.J.; Moeller, J.R.; Strother, S.C.; Dhawan, V.; Rottenberg, D.A.

    1988-06-01

    Eleven patients with acquired cerebellar degeneration (10 of whom had paraneoplastic cerebellar degeneration (PCD)) were evaluated using neuropsychological tests and /sup 18/F-fluorodeoxyglucose/positron emission tomography to (1) quantify motor, cognitive, and metabolic abnormalities; (2) determine if characteristic alterations in the regional cerebral metabolic rate for glucose (rCMRGlc) are associated with PCD; and (3) correlate behavioral and metabolic measures of disease severity. Eighteen volunteer subjects served as normal controls. Although some PCD neuropsychological test scores were abnormal, these results could not, in general, be dissociated from the effects of dysarthria and ataxia. rCMRGlc was reduced in patients with PCD (versus normal control subjects) in all regions except the brainstem. Analysis of patient and control rCMRGlc data using a mathematical model of regional metabolic interactions revealed two metabolic pattern descriptors, SSF1 and SSF2, which distinguished patients with PCD from normal control subjects; SSF2, which described a metabolic coupling between cerebellum, cuneus, and posterior temporal, lateral frontal, and paracentral cortex, correlated with quantitative indices of cerebellar dysfunction. Our inability to document substantial intellectual impairment in 7 of 10 patients with PCD contrasts with the 50% incidence of dementia in PCD reported by previous investigators. Widespread reductions in PCD rCMRGlc may result from the loss of cerebellar efferents to thalamus and forebrain structures, a reverse cerebellar diaschisis.

  18. Concomitant ablation for atrial fibrillation during septal myectomy in patients with hypertrophic obstructive cardiomyopathy.

    Science.gov (United States)

    Bogachev-Prokophiev, Alexander V; Afanasyev, Alexander V; Zheleznev, Sergei I; Pivkin, Alexei N; Fomenko, Michael S; Sharifulin, Ravil M; Karaskov, Alexander M

    2017-09-01

    The appearance of atrial fibrillation is associated with significant clinical deterioration in patients with obstructive hypertrophic cardiomyopathy; therefore, maintenance of sinus rhythm is desirable. Guidelines and most articles have reported the results of catheter ablation and pharmacologic atrial fibrillation treatment; nevertheless, data regarding concomitant procedures during septal myectomy are limited. The aim of this study was to assess the outcomes of concomitant atrial fibrillation treatment in patients with obstructive hypertrophic cardiomyopathy. Between 2010 and 2013 in our clinic, 187 patients with obstructive hypertrophic cardiomyopathy underwent extended myectomy. In 45 cases, concomitant Cox-Maze IV procedure was performed; however, obstructive hypertrophic cardiomyopathy was the primary indication for surgery. Atrial fibrillation was paroxysmal in 26 patients (58%) and nonparoxysmal in 19 patients (42%). The mean age of patients was 52.8 ± 14.2 years (range, 22-74 years). Mean peak gradient was 90.7 ± 24.2 mm Hg, and interventricular septum thickness was 26.1 ± 4.3 mm. Mean atrial fibrillation duration was 17.3 ± 8.5 months. There were no early deaths. No procedure-related complications occurred with regard to ablation procedure. Complete atrioventricular block was achieved in 2 patients (4.0%). Mean crossclamping time was 61 ± 36 minutes. Peak left ventricular outflow tract gradient was 12.6 ± 5.5 mm Hg based on transesophageal echocardiography. The Maze IV procedure was used for ablation in all patients (radiofrequency ablation with bipolar clamp + cryolesion for mitral and tricuspid lines). Because of the atrial wall thickness (5-6 mm), applications were performed 8 to 10 times on each line. There were no cases of pacemaker implantation due to sinus node dysfunction. All patients were discharged in stable sinus rhythm. Mean follow-up was 23.7 ± 1.3 months. The rate of atrial fibrillation freedom was 100% (45

  19. Nd:YAG Laser Treatment for Keloids and Hypertrophic Scar/span>s: An Analysis of 102 Cases

    Science.gov (United States)

    Koike, Sachiko; Akaishi, Satoshi; Nagashima, Yuki; Dohi, Teruyuki; Hyakusoku, Hiko

    2014-01-01

    Background: The present retrospective cohort study was performed to determine the efficacy of contact-mode 1064 nm neodymium-yttrium-aluminum-garnet (Nd:YAG) laser laser for keloids and hypertrophic scar/span>s. The indication and limitations of this modality are discussed. Methods: The cohort consisted of 102 consecutive Japanese patients (23 males and 79 females) with keloids and hypertrophic scar/span>s for more than 1 year. They were treated every 3–4 weeks for 1 year with a long-pulsed 1064 nm Nd:YAG laser (Cutera, Brisbane, Calif.) in contact mode. Thirty-eight patients had hypertrophic scar/span>s and 64 had keloids. The scars were evaluated before the treatment commenced and 1 month after the last session by using the Japan Scar Workshop Scar Scale 2011. Recurrence was assessed at 6 months after the termination of treatment. Results: The average total Japan Scar Workshop score of the keloid and hypertrophic scar region groups dropped significantly after 1 year of treatment compared with before treatment (all P hypertrophic scar/span>s or keloids deteriorated. However, 3 of the 34 anterior chest keloids (8.8%) did not respond. The following recurrence rates were observed 6 months after stopping laser treatment: 1 of the abdomen hypertrophic scar/span>s (4%), 18 of the anterior chest keloids (52.9%), 5 of the upper arm keloids (35.7%), and 4 of the scapula keloids (25%). Conclusions: Hypertrophic scar/span>s responded significantly better to 1064 nm Nd:YAG laser treatment than keloids. However, keloid recurrence occurred when there was remaining redness and induration, even if only a small part of the scar was affected. PMID:25587506

  20. Pattern of degeneration of leiomyoma uteri | Igwegbe | Journal of ...

    African Journals Online (AJOL)

    The aim of this study is to determine the prevalence and pattern of degenerations in leiomyomas. We sought to relate these degenerations with clinical features. It is a retrospective study of histological features of specimens of leiomyomas obtained at the myomectomy, hysterectomy and polypectomy. Results showed that ...

  1. Viscosity solution of linear regulator quadratic for degenerate diffusions

    Directory of Open Access Journals (Sweden)

    2006-01-01

    Full Text Available The paper studied a linear regulator quadratic control problem for degenerate Hamilton-Jacobi-Bellman (HJB equation. We showed the existence of viscosity properties and established a unique viscosity solution of the degenerate HJB equation associated with this problem by the technique of viscosity solutions.

  2. Age-related Macular Degeneration: Current concepts in ...

    African Journals Online (AJOL)

    This paper reviews current concepts in the pathogenesis and management of agerelated macular degeneration as found in Pubmed journals over the past ten years with a view to recommending optimal treatment regimes for African populations. Keywords: age-related macular degeneration, pathogenesis, genetics ...

  3. Prevalence of age-related macular degeneration in elderly Caucasians

    DEFF Research Database (Denmark)

    Erke, Maja G; Bertelsen, Geir; Peto, Tunde

    2012-01-01

    To describe the sex- and age-specific prevalence of drusen, geographic atrophy, and neovascular age-related macular degeneration (AMD).......To describe the sex- and age-specific prevalence of drusen, geographic atrophy, and neovascular age-related macular degeneration (AMD)....

  4. Delivery systems for the treatment of degenerated intervertebral discs

    NARCIS (Netherlands)

    Blanquer, Sebastien; Grijpma, Dirk W.; Poot, Andreas A.

    2015-01-01

    The intervertebral disc (IVD) is the most avascular and acellular tissue in the body and therefore prone to degeneration. During IVD degeneration, the balance between anabolic and catabolic processes in the disc is deregulated, amongst others leading to alteration of extracellular matrix production,

  5. Delivery systems for the treatment of degenerated intervertebral discs

    NARCIS (Netherlands)

    Blanquer, S. B. G.; Grijpma, D. W.; Poot, A. A.

    The intervertebral disc (ND) is the most avascular and acellular tissue in the body and therefore prone to degeneration. During IVD degeneration, the balance between anabolic and catabolic processes in the disc is deregulated, amongst others leading to alteration of extracellular matrix production,

  6. Ion waves driven by shear flow in a relativistic degenerate ...

    Indian Academy of Sciences (India)

    Abstract. We investigate the existence and propagation of low-frequency (in comparison to ion cyclotron frequency) electrostatic ion waves in highly dense inhomogeneous astrophysical mag- netoplasma comprising relativistic degenerate electrons and non-degenerate ions. The dispersion equation is obtained by Fourier ...

  7. Frequency metrology in quantum degenerate helium

    Directory of Open Access Journals (Sweden)

    Vassen Wim

    2013-08-01

    Full Text Available We have measured the absolute frequency of the 1557-nm doubly forbidden transition between the two metastable states of helium, 2 3S1 (lifetime 8000 s and 2 1S0 (lifetime 20 ms, with 1 kHz precision. With an Einstein coefficient of 10−7 s−1 this is one of weakest optical transitions ever measured. The measurement was performed in a Bose-Einstein condensate of 4He* as well as in a Degenerate Fermi Gas of 3He*, trapped in a crossed dipole trap. From the isotope shift we deduced the nuclear charge radius difference between the α-particle and the helion. Our value differs by 4σ with a very recent result obtained on the 2 3S → 2 3P transition.

  8. Bmi-1 absence causes premature brain degeneration.

    Directory of Open Access Journals (Sweden)

    Guangliang Cao

    Full Text Available Bmi-1, a polycomb transcriptional repressor, is implicated in cell cycle regulation and cell senescence. Its absence results in generalized astrogliosis and epilepsy during the postnatal development, but the underlying mechanisms are poorly understood. Here, we demonstrate the occurrence of oxidative stress in the brain of four-week-old Bmi-1 null mice. The mice showed various hallmarks of neurodegeneration including synaptic loss, axonal demyelination, reactive gliosis and brain mitochondrial damage. Moreover, astroglial glutamate transporters and glutamine synthetase decreased in the Bmi-1 null hippocampus, which might contribute to the sporadic epileptic-like seizures in these mice. These results indicate that Bmi-1 is required for maintaining endogenous antioxidant defenses in the brain, and its absence subsequently causes premature brain degeneration.

  9. Conjunctival intraepithelial neoplasia with corneal furrow degeneration

    Directory of Open Access Journals (Sweden)

    Pukhraj Rishi

    2014-01-01

    Full Text Available A 68-year-old man presented with redness of left eye since six months. Examination revealed bilateral corneal furrow degeneration. Left eye lesion was suggestive of conjunctival squamous cell carcinoma, encroaching on to cornea. Anterior segment optical coherence tomography (AS-OCT confirmed peripheral corneal thinning. Fluorescein angiography confirmed intrinsic vascularity of lesion. Patient was managed with "no touch" surgical excision, dry keratectomy without alcohol, cryotherapy, and primary closure. Pathologic examination of removed tissue confirmed clinical diagnosis. Management of this particular case required modification of standard treatment protocol. Unlike the alcohol-assisted technique of tumor dissection described, ethyl alcohol was not used for risk of corneal perforation due to underlying peripheral corneal thinning. Likewise, topical steroids were withheld in the post-operative period. Three weeks post-operatively, left eye was healing well. Hence, per-operative usage of absolute alcohol and post-operative use of topical steroids may be best avoided in such eyes.

  10. Prevention of Age-Related Macular Degeneration.

    Science.gov (United States)

    Singh, Niharika; Srinivasan, Sangeetha; Muralidharan, Vinata; Roy, Rupak; V, Jayprakash; Raman, Rajiv

    2017-01-01

    Age-related macular degeneration (AMD) compromises quality of life. However, the available therapeutic options are limited. This has led to the identification of modifiable risk factors to prevent the development or alter the natural course and prognosis of AMD. The identification and modification of risk factors has the potential for greater public health impact on reducing morbidity from AMD. Likewise, identifying the imaging clues and genetic clues could serve as a guide to recognizing the propensity for progression to severe and end stages of the disease. Several attempts, both successful and unsuccessful, have been made for interventions that could delay the progression of AMD. Of these, pharmacological interventions have shown promising results. The Age-Related Eye Disease Study 1 and 2 have shown the beneficial role of antioxidants in a selected group of patients. Copyright 2017 Asia-Pacific Academy of Ophthalmology.

  11. A Novel Triple Medicine Combination Injection for the Resolution of Keloids and Hypertrophic Scar/span>s

    Science.gov (United States)

    Gold, Michael H.

    2014-01-01

    Keloids and hypertrophic scar/span>s remain one of the more difficult treatment concerns for clinicians. A variety of therapies have been used in the past with moderate success. On occasion, combination therapy has been used to treat these lesion, in an attempt to lessen the symptoms of pain and pruritus that often accompanies keloids and hypertrophic scar/span>s, as well as treating the actual lesions themselves. A novel triple combination injection process is introduced here in an attempt to further reduce the signs and symptoms of these lesions. The combination includes 5-fluoruracil, triamcinolone acetonide, and hyaluronidase. All three work in concert to treat keloids and hypertrophic scar/span>s, and this is the first work at looking at these medicines given together, at the same time, in a series of recalcitrant keloids and hypertrophic scar/span>s. The positive results warrant further investigation and hope for those with keloids and hypertrophic scar/span>s. PMID:25489380

  12. Effect of HMME-PDT with different parameters in rabbit ear model: a possible way for hypertrophic scarring

    Science.gov (United States)

    Cai, Hong; Gu, Ying; Zeng, Jing; Li, Shao-ran; Sun, Qiang; Wang, Ying; Shi, Dong-wen; Zhang, Lu-yong

    2007-11-01

    Background and Objective: Hypertrophic scar is a pathological scar that grows aberrantly by excessive deposition of collagens in the dermis. It is known that photodynamic therapy (PDT) contributes to a variety of diseases, however, the use of inhibiting scar formation has not been fully explored. The purpose of this study is to investigate the effect of HMME-PDT (Photodynamic therapy induced by Hematoporphyrin Monomethyl Ether) with different parameters on hypertrophic scar in rabbit ear. Materials and Methods: After the placement of 7-mm diameter dermal wounds on each ear, the acute model of dermal hypertrophic scar in the New Zealand white rabbits was established. Scar wounds were randomly separated into 2 groups: the experimental group received HMME-PDT with different parameters, and the control group received no special treatment. Specimens were harvested from scar wounds on postoperative day 28. Scar and hypertrophic index (HI) were observed by haematoxylin-eosin staining. Results: Compared with the control group, scar formation was inhibited by HMME-PDT in the experimental group with parameters as follows: photosensitizer dose 10mg/kg, power density 20mw/cm2, fluence 5J/cm2, meanwhile, HI was decreased significantly. Conclusion: HMME-PDT may play a role in inhibiting hypertrophic scarring in rabbit ear. The biological effect is determined by the dose of photosensitizer, interval between the injection of photosensitizer and irradiation, power density and energy fluence.

  13. Ablative fractional laser treatment for hypertrophic scars: comparison between Er:YAG and CO2 fractional lasers.

    Science.gov (United States)

    Choi, Jae Eun; Oh, Ga Na; Kim, Jong Yeob; Seo, Soo Hong; Ahn, Hyo Hyun; Kye, Young Chul

    2014-08-01

    Nonablative fractional photothermolysis has been reported to show early promise in the treatment of hypertrophic scars, but there are few reports on ablative fractional photothermolysis for the treatment of hypertrophic scars. To evaluate and compare the efficacy and safety of Er:YAG fractional laser (EYFL) and CO2 fractional laser (CO2FL) for treatment of hypertrophic scars. Thirteen patients with hypertrophic scars were treated with 2,940 nm EYFL, and ten were treated with 10,600 nm CO2FL. An independent physician evaluator assessed the treatment outcomes using Vancouver scar scale (VSS) and 5-point grading scale (grade 0, no improvement; grade 1, 1-25%; grade 2, 26-50%; grade 3, 51-75%; grade 4, 76-100% improvement). Patients are queried about their subjective satisfaction with the treatment outcomes. After the final treatment, average percentage changes of VSS were 28.2% for EYFL and 49.8% for CO2FL. Improvement was evident in terms of pliability, while insignificant in terms of vascularity and pigmentation. Based on physician's global assessment, mean grade of 1.8 for EYFL and 2.7 for CO2FL was achieved. Patient's subjective satisfaction scores paralleled the physician's objective evaluation. CO2FL is a potentially effective and safe modality for the treatment of hypertrophic scars, particularly in terms of pliability.

  14. Evidence for lysosomal exocytosis and release of aggrecan-degrading hydrolases from hypertrophic chondrocytes, in vitro and in vivo

    Directory of Open Access Journals (Sweden)

    Edward R. Bastow

    2012-02-01

    The abundant proteoglycan, aggrecan, is resorbed from growth plate cartilage during endochondral bone ossification, yet mice with genetically-ablated aggrecan-degrading activity have no defects in bone formation. To account for this apparent anomaly, we propose that lysosomal hydrolases degrade extracellular, hyaluronan-bound aggrecan aggregates in growth plate cartilage, and that lysosomal hydrolases are released from hypertrophic chondrocytes into growth plate cartilage via Ca2+-dependent lysosomal exocytosis. In this study we confirm that hypertrophic chondrocytes release hydrolases via lysosomal exocytosis in vitro and we show in vivo evidence for lysosomal exocytosis in hypertrophic chondrocytes during skeletal development. We show that lysosome-associated membrane protein 1 (LAMP1 is detected at the cell surface following in vitro treatment of epiphyseal chondrocytes with the calcium ionophore, ionomycin. Furthermore, we show that in addition to the lysosomal exocytosis markers, cathepsin D and β-hexosaminidase, ionomycin induces release of aggrecan- and hyaluronan-degrading activity from cultured epiphyseal chondrocytes. We identify VAMP-8 and VAMP7 as v-SNARE proteins with potential roles in lysosomal exocytosis in hypertrophic chondrocytes, based on their colocalisation with LAMP1 at the cell surface in secondary ossification centers in mouse tibiae. We propose that resorbing growth plate cartilage involves release of destructive hydrolases from hypertrophic chondrocytes, via lysosomal exocytosis.

  15. Formation of Degenerate Band Gaps in Layered Systems

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    Alexey P. Vinogradov

    2012-06-01

    Full Text Available In the review, peculiarities of spectra of one-dimensional photonic crystals made of anisotropic and/or magnetooptic materials are considered. The attention is focused on band gaps of a special type—the so called degenerate band gaps which are degenerate with respect to polarization. Mechanisms of formation and properties of these band gaps are analyzed. Peculiarities of spectra of photonic crystals that arise due to the linkage between band gaps are discussed. Particularly, it is shown that formation of a frozen mode is caused by linkage between Brillouin and degenerate band gaps. Also, existence of the optical Borrmann effect at the boundaries of degenerate band gaps and optical Tamm states at the frequencies of degenerate band gaps are analyzed.

  16. Graft Infection Masquerading as Rheumatologic Disease: a Rare Case of Aortobifemoral Graft Infection Presenting as Hypertrophic Osteoarthropathy.

    Science.gov (United States)

    Chapman, Stephanie A; Delgadillo, Daniel; MacGuidwin, Elizabeth; Greenberg, Joshua I; Jameson, Andrew P

    2017-05-01

    Prosthetic vascular graft procedures are a common treatment modality for peripheral vascular disease. A relatively common complication is graft infection, occurring at a rate of 0.5-5%. When they occur, graft infections are associated with significant morbidity and mortality. Vascular graft infections also represent a diagnostic and therapeutic challenge for the physician METHODS: Here, we report a case where the rare finding of secondary hypertrophic osteoarthropathy was an important indication of underlying aortic graft infection that was initially misdiagnosed. A review of the literature revealed 34 cases of vascular graft infection associated with hypertrophic osteoarthropathy. The mean interval from surgery to time of infection was approximately 5 years. Mortality was 35%. When combined with hypertrophic osteoarthropathy, vascular graft infection was complicated by an aortoenteric fistula in 53% of the cases. The complexity of this case highlights the challenges physicians face in order to diagnose and treat this condition. Copyright © 2017 Elsevier Inc. All rights reserved.

  17. Outcomes of Alcohol Septal Ablation in Younger Patients With Obstructive Hypertrophic Cardiomyopathy

    DEFF Research Database (Denmark)

    Liebregts, Max; Faber, Lothar; Jensen, Morten K.

    2017-01-01

    Objectives The aim of this study was to describe the safety and outcomes of alcohol septal ablation (ASA) in younger patients with obstructive hypertrophic cardiomyopathy. Background The American College of Cardiology Foundation/American Heart Association guidelines reserve ASA for older patients...... and patients with serious comorbidities. Data on long-term age-specific outcomes after ASA are scarce. Methods A total of 1,197 patients (mean age 58 ± 14 years) underwent ASA for obstructive hypertrophic cardiomyopathy. Patients were divided into young (≤50 years), middle-age (51 to 64 years), and older (≥65...... patients with obstructive hypertrophic cardiomyopathy was safe and effective for relief of symptoms at long-term follow-up. The authors propose that the indication for ASA can be broadened to younger patients....

  18. Recent advances in early-onset severe retinal degeneration: more than just basic research?

    DEFF Research Database (Denmark)

    Preising, M. N.; Heegaard, S.

    2004-01-01

    ophthalmology, Leber's congenital amaurosis, phenotype, gene therapy, retina, degeneration, rewiew......ophthalmology, Leber's congenital amaurosis, phenotype, gene therapy, retina, degeneration, rewiew...

  19. Transplantation of retinal pigment epithelial cells - a possible future treatment for age-related macular degeneration

    DEFF Research Database (Denmark)

    Wiencke, Anne Katrine

    2001-01-01

    ophthalmology, age-related macular degeneration, transplantation, retinal pigment epithelial cells, treatment......ophthalmology, age-related macular degeneration, transplantation, retinal pigment epithelial cells, treatment...

  20. Transplantation of retinal pigment epithelial cells - a possible future treatment for age-related macular degeneration

    DEFF Research Database (Denmark)

    Wiencke, Anne Katrine

    2001-01-01

    ophthalmology, age-related macular degeneration, retinal pigment epithelial cells, transplantation, treatment......ophthalmology, age-related macular degeneration, retinal pigment epithelial cells, transplantation, treatment...

  1. Logistics of building a laser practice for the treatment of hypertrophic burn scars.

    Science.gov (United States)

    Hultman, Charles Scott; Edkins, Renee E; Cairns, Bruce A; Meyer, Anthony A

    2013-05-01

    Although lasers can improve burn scars, such treatment has not been adopted universally, due to operational challenges starting a practice and the perception that such a program is not financially viable. We report the logistics of building a laser practice for the treatment of hypertrophic burn scars. We analyzed the clinical, operational, and financial components of our laser practice, focusing on treatment of hypertrophic burn scars, using pulsed dye laser, fractional CO2 laser, and intense pulsed light. Cases were performed in an operating room, with anesthesia, after preauthorization. We examined professional charges and collections, case time, variable and indirect expenses, and breakeven volumes. Our practice grew as follows: 2008, 1 case; 2009, 44 cases; 2010, 169 cases; and 2011, 415 cases. Overall collection rate was 32.1%. Expenses incurred by the provider, per 8-hour session, included laser rental/lease ($2375), personnel salaries ($1900), and physician overhead ($808), for a total cost of $5083. Mean charge was $1642 per case; mean collection was $527 per case. Median case time (procedure plus turnover) was 40 minutes. In this model, breakeven volume is 9.7 cases per day; breakeven time is 49.7 minutes. Provider profit margin for 10 cases per day, or 83% capacity utilization, is $187 per day (income - expenses = $5270 - $5083). Despite high costs associated with starting and operating a laser practice for the treatment of hypertrophic burn scars, a sustainable enterprise can be achieved when the provider has accrued enough volume to batch cases over an entire day. Critical to achieving breakeven is preauthorization, controlling overhead, and efficient throughput.

  2. Mechanism of Progressive Heart Failure and Significance of Pulmonary Hypertension in Obstructive Hypertrophic Cardiomyopathy.

    Science.gov (United States)

    Covella, Michele; Rowin, Ethan J; Hill, Nicholas S; Preston, Ioana R; Milan, Alberto; Opotowsky, Alexander R; Maron, Barry J; Maron, Martin S; Maron, Bradley A

    2017-04-01

    There are limited data on the prevalence, pathophysiology, and management implications of pulmonary hypertension in patients with obstructive hypertrophic cardiomyopathy and advanced heart failure. To assess the clinical significance of measured cardiopulmonary hemodynamics in hypertrophic cardiomyopathy patients with heart failure, we retrospectively assessed right heart catheterization data in 162 consecutive patients with outflow tract gradients (median [interquartile range], 90 mm Hg [70-110 mm Hg]), 59±11 years old, and 49% men, predominately New York Heart Association class III/IV status. Pulmonary hypertension (mean pulmonary artery pressure, ≥25 mm Hg) was present in 82 patients (51%), including 29 (18%) regarded as moderate-severe (mean pulmonary artery pressure, ≥35 mm Hg) and 28 (34%) also had increased pulmonary vascular resistance >3.0 WU. The pulmonary artery wedge pressure was ≤15 mm Hg in 54%, indicating that left atrial hypertension was absent in a majority of patients. Notably, 9 patients (11%) met hemodynamic criteria for precapillary pulmonary hypertension (mean pulmonary artery pressure, ≥25 mm Hg; pulmonary vascular resistance, >3.0 WU; pulmonary artery wedge pressure, ≤15 mm Hg). Over a median follow-up of 327 days (90-743 days) after surgical myectomy (or alcohol septal ablation), 92% and 95% of patients with or without preoperative pulmonary hypertension, respectively, were asymptomatic or mildly symptomatic. One postoperative death occurred in a 59-year-old woman with acute respiratory failure and mean pulmonary artery pressure of 65 mm Hg. Pulmonary hypertension was common in obstructive hypertrophic cardiomyopathy patients with advanced heart failure. Although possibly a contributor to preoperative heart failure, pulmonary hypertension did not significantly influence clinical and surgical outcome. Notably, a novel patient subgroup was identified with resting invasive hemodynamics consistent with pulmonary

  3. Suppressed inflammatory gene expression during human hypertrophic scar compared to normotrophic scar formation.

    Science.gov (United States)

    van den Broek, Lenie J; van der Veer, Willem M; de Jong, Etty H; Gibbs, Susan; Niessen, Frank B

    2015-08-01

    Hypertrophic scar formation is a result of adverse cutaneous wound healing. The pathogenesis of hypertrophic scar formation is still poorly understood. A problem next to the lack of suitable animal models is that often normal skin is compared to hypertrophic scar (HTscar) and not to normotrophic scar (NTscar) tissue. Another drawback is that often only one time period after wounding is studied, while scar formation is a dynamic process over a period of several months. In this study, we compared the expression of genes involved in inflammation, angiogenesis and extracellular matrix (ECM) formation and also macrophage infiltration in biopsies obtained before and up to 52 weeks after standard surgery in five patients who developed HTscar and six patients who developed NTscar. It was found that HTscar formation coincided with a prolonged decreased expression of inflammatory genes (TNFα, IL-1α, IL-1RN, CCL2, CCL3, CXCL2, CXCR2, C3 and IL-10) and an extended increased expression of ECM-related genes (PLAU, Col3A1, TGFβ3). This coincided with a delayed but prolonged infiltration of macrophages (type 2) in HTscar tissue compared to NTscar tissue. These findings were supported by immunohistochemical localization of proteins coding for select genes named above. Our study emphasizes that human cutaneous wound healing is a dynamic process that is needed to be studied over a period of time rather than a single point of time. Taken together, our results suggest innate immune stimulatory therapies may be a better option for improving scar quality than the currently used anti-inflammatory scar therapies. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  4. Evaluating hypertrophic thyroidectomy scar outcomes after treatment with triamcinolone injections and copper bromide laser therapy.

    Science.gov (United States)

    On, Hye Rang; Lee, Sang Hee; Lee, Yong Sang; Chang, Hang-Seok; Park, CheongSoo; Roh, Mi Ryung

    2015-08-01

    Postoperative hypertrophic scar following thyroidectomy can be a major concern due to its disfiguring appearance. Recently, copper bromide laser (CBL) and intralesional triamcinolone injection (TA ILI) have been used to treat hypertrophic thyroidectomy scars. Data regarding the number of treatment sessions needed to reach a certain endpoint and the prognostic factors that affect treatment duration are unknown. The aim of this study was to evaluate the number of treatment sessions required to reduce VSS score by 50%, which was regarded as the treatment endpoint, and to investigate the factors that influence treatment duration when using CBL and TA ILI. A total of 67 patients were enrolled in this study. Baseline characteristics of the patients including age, sex, body mass index (BMI), distance of the scar from the sternal notch, time of development of the hypertrophic scar, sternocleidomastoid (SCM) muscle prominence, and date of operation were collected on the first visit. They were treated with CBL and TA. The concentration of triamcinolone used was 2.5 mg/ml or 5 mg/ml according to the pliability score of each scar. The mean number of treatment sessions required to achieve the endpoint was 3.85 ± 1.25. Among the variables assessed, location of the scar near the sternal notch (P = 0.020) and patient BMI (P = 0.001) were associated with the increasing number of treatment sessions. In our study cohort, four treatments were required to reduce the VSS of thyroidectomy scars by 50% when using a combination treatment of CBL and low concentration TA ILI. Also, scar location and patient BMI are factors that affect treatment outcome. © 2015 Wiley Periodicals, Inc.

  5. Desmodium gangeticum root extract attenuates isoproterenol-induced cardiac hypertrophic growth in rats.

    Directory of Open Access Journals (Sweden)

    Divya Hitler

    2014-10-01

    Full Text Available Context: Desmodium gangeticum (L DC (Fabaceae; DG, a medicinal plant that grows in tropical habitats, is widely used to treat various ailments including digestive and inflammatory disorders. Aims: To investigate the possible cardioprotective activity of a DG root extract against isoproterenol (ISO-induced left ventricular cardiac hypertrophy (LVH in adult Wistar rats. Methods: Daily intraperitoneal administration of ISO (10 mg/kg body weight, single injection for 7 days induced LVH in rats. The LVH rats were post-treated orally with DG (100 mg/kg body weight for a period of 30 days. Thereafter, changes in heart weight (HW and body weight (BW, HW/BW ratio, percent of hypertrophy, collagen accumulation, activities of matrix metalloproteinase (MMP -2 and -9, superoxide dismutase (SOD and catalase (CAT enzymes, and the level of an oxidative stress marker, lipid peroxide (LPO, were determined. Results: HW/BW ratio, an indicator of hypertrophic growth, was significantly reduced in DG root post-treated LVH rats as compared with that for the non-treated LVH rats. The altered levels of ventricular LPO, collagen, MMPs-2 and -9, and antioxidant enzymes in the ISO-treated animals reverted back to near normal upon DG treatment. Further, the anti-hypertrophic activity of DG was comparable to that of the standard drug losartan (10 mg/kg. Conclusions: The results of the present study suggest that the aqueous root extract of DG exhibited anti-hypertrophic activity in-vivo by inhibiting ISO-induced ROS generation and MMP activities.

  6. SFRP2 and slug contribute to cellular resistance to apoptosis in hypertrophic scars.

    Directory of Open Access Journals (Sweden)

    Liang Chen

    Full Text Available Hypertrophic scars (HS are skin disorders which occur after wounding and thermal injury. Our previous studies have suggested that secreted frizzled-related protein 2 (SFRP2 is involved in HS formation and that the suppression of SFRP2 promotes apoptosis of hypertrophic scar fibroblasts (HSFBs. However, the mechanisms have not been clarified. Previous studies revealed that Slug expression inhibits cell apoptosis, in vitro and in vivo, and SFRP2 regulates the expression of Slug in cervical cancer cells. In the present study, we quantified differential expression levels of expression of SFRP2 and Slug in HS and normal skin tissues by immunohistochemistry, both of which have important anti-apoptosis roles. Furthermore, a short hairpin RNA approach was adopted to investigate the potential function of SFRP2 and Slug in HSFB apoptosis. Cell apoptosis was detected using fluorescence-activated cell sorting and Caspase-3 activity was assayed by spectrophotometry. This study demonstrates that SFRP2 expression, as well as Slug, is dramatically up-regulated in HS relative to normal skin tissues, and the Slug expression is positively correlated with SFRP2. Slug expression was down-regulated in SFRP2-deficient cells, and the down-regulation of Slug expression increased sensitivity to apoptosis which was induced through a caspase-3-dependent pathway. The infected cells with reduced levels of Slug were tested for the expression of apoptosis-related genes (Bcl-2, Bax and PUMA which were previously identified as Slug targets. Bcl-2 expression was down-regulated in Slug-deficient cells. In conclusion, SFRP2 appears to interact with Slug to affect the apoptosis of hypertrophic scar fibroblasts.

  7. High sensitivity of late gadolinium enhancement for predicting microscopic myocardial scarring in biopsied specimens in hypertrophic cardiomyopathy.

    Directory of Open Access Journals (Sweden)

    Tetsuo Konno

    Full Text Available BACKGROUND: Myocardial scarring can be assessed by cardiac magnetic resonance imaging with late gadolinium enhancement and by endomyocardial biopsy. However, accuracy of late gadolinium enhancement for predicting microscopic myocardial scarring in biopsied specimens remains unknown in hypertrophic cardiomyopathy. We investigated whether late gadolinium enhancement in the whole heart reflects microscopic myocardial scarring in the small biopsied specimens in hypertrophic cardiomyopathy. METHODS AND RESULTS: Twenty-one consecutive patients with hypertrophic cardiomyopathy who were examined both by cardiac magnetic resonance imaging and by endomyocardial biopsy were retrospectively studied. The right interventricular septum was the target site for endomyocardial biopsy in all patients. Late gadolinium enhancement in the ventricular septum had an excellent sensitivity (100% with a low specificity (40% for predicting microscopic myocardial scarring in biopsied specimens. The sensitivity of late gadolinium enhancement in the whole heart remained 100% with a specificity of 27% for predicting microscopic myocardial scarring in biopsied specimens. Quantitative assessments of fibrosis revealed that the extent of late gadolinium enhancement in the whole heart was the only independent variable related to the microscopic collagen fraction in biopsied specimens (β  =  0.59, 95% confident interval: 0.15 - 1.0, p  =  0.012. CONCLUSIONS: Although there was a compromise in the specificity, the sensitivity of late gadolinium enhancement was excellent for prediction of microscopic myocardial scarring in hypertrophic cardiomyopathy. Moreover, the severity of late gadolinium enhancement was independently associated with the quantitative collagen fraction in biopsied specimens in hypertrophic cardiomyopathy. These findings indicate that late gadolinium enhancement can reflect both the presence and the extent of microscopic myocardial scarring in the small

  8. A case of mid-apical obstructive hypertrophic cardiomyopathy treated with a transapical myectomy approach: a case report.

    Science.gov (United States)

    Scudeler, Thiago Luis; Rezende, Paulo Cury; Oikawa, Fernando Teiichi Costa; da Costa, Leandro Menezes Alves; Hueb, Alexandre Ciappina; Hueb, Whady

    2014-11-11

    Hypertrophic cardiomyopathy is a genetic cardiac disease characterized by marked variability in morphological expression and natural history. The hypertrophic myocardium is often confined to the septum or lateral wall of the left ventricle, but it can also be encountered in the middle or apical segments of the myocardium. Treatment is based on medical therapy. Others therapies, such as embolization of the septal artery or ventriculomyectomy, are indicated in special situations. Surgery is the standard treatment, and it is classically done via a transaortic approach; however, in cases in which the hypertrophic myocardium is confined to mid-apical segments, a transapical approach is an option. Only a few cases of mid-apical obstructive hypertrophic cardiomyopathy treated with a myectomy using a transapical approach have been reported in the English-language literature. In this report, we present a case of a patient with mid-apical obstructive hypertrophic cardiomyopathy treated using this new approach. A 63-year-old Caucasian woman presented with a history of chest pain and shortness of breath causing significant limitations on her daily life activities. She had a history of coronary artery disease. Her physical examination was unremarkable. Transthoracic echocardiography revealed normal systolic function and significant concentric left ventricular hypertrophy that was greater in the mid-apical region. Nuclear magnetic resonance imaging confirmed significant hypertrophy of the median segments of the left ventricle. The patient had persistent symptoms despite receiving optimized medical treatment, and a surgical approach was indicated. As a myectomy using transaortic technique was thought to be difficult to perform in her case, a transapical approach was used. No complications occurred, and her symptoms resolved. A transapical myectomy should be taken into consideration for patients with mid-apical obstructive hypertrophic cardiomyopathy that is refractory to medical

  9. Percutaneous septal ablation for left mid-ventricular obstructive hypertrophic cardiomyopathy: a case report

    Directory of Open Access Journals (Sweden)

    Alioglu Emin

    2006-04-01

    Full Text Available Abstract Background Mid-ventricular obstructive hypertrophic cardiomyopathy (MVOHC is a rare type of cardiomyopathy. The diagnosis is based on the hourglass appearance on the left ventriculogram and the presence of pressure gradient between apical and basal chamber of the ventriculum on the hemodynamic assessment. Case presentation The present case represents successful percutaneous treatment with septal ablation to patient with MVOHC associated with systolic anterior motion of the mitral valve and obstruction at both the mid-ventricular and outflow levels. Conclusion Alcohol septal ablation has been proposed as less invasive alternatives to surgery in patients with MVOHC.

  10. Non-Operative Treatment of Hypertrophic Scar/span>s and Keloids After Burns in Children

    Science.gov (United States)

    Argirova, M.; Hadjiski, O.; Victorova, A.

    2006-01-01

    Summary Scars are a consequence of the natural way of wound healing and replacement of the damaged part of the skin. Hypertrophic scar/span>s and keloids are formed as a result of the process of abnormal wound healing, causing aesthetic and functional deformities, discomfort, and disturbance of children's normal growth. The prophylaxis and treatment of these scars, i.e. burns sequelae, are a significant moment in the treatment of children with burns. The objectives of the present study are methods for the prophylaxis and non-operative treatment of hypertrophic scar/span>s and keloids in children with burns. Altogether, 547 children with 485 burns sequelae - fresh scars in the period of healing and old hypertrophic scar/span>s (377) and keloids (108) - were treated in our Clinic of Children's Burns for a period of five years. The age of the patients varied between 0 and 18 years. Non-operative treatment was applied in 276 children. The patients were divided into two groups according to the treatment applied. Compression, intralesional application of triamcinolone acetonide, and silicone sheets were employed in the first group (84.06%), while only silicone sheets were applied to children in the second group (15.94%). Unaffected areas and areas adjacent to the scar were used as control areas. The sequelae monitored in both groups were assessed using the Vancouver Scar Scale. The children were monitored in an out-patient department. The results obtained are regardless of age, anatomical location, and scar aetiology. In old scars, and especially in keloids, the treatment proved to have variable results, according to the assessment parameters. We were satisfied with the improvement of the parameters during the course of treatment and with the good final results, which we consider a therapeutic success. Analysis of the results shows that the parameters improved slowly during the course of treatment, the process being most active during the first few weeks. The use of

  11. Increased transient receptor potential vanilloid type 1 (TRPV1) channel expression in hypertrophic heart

    DEFF Research Database (Denmark)

    Thilo, Florian; Liu, Ying; Schulz, Nico

    2010-01-01

    The aim of this study was to compare the expression of transient receptor potential vanilloid type 1 (TRPV1) channels in hypertrophic hearts from transgenic mice showing overexpression of the catalytic subunit alpha of protein phosphatase 2A alpha (PP2Ac alpha) with wild-type mice and with TRPV1......-/- mice. Transcripts of TRPV1, matrix metalloproteinase 9 (MMP9), discoidin domain receptor family, member 2 (DDR-2), atrial natriuretic peptide (ANP), GATA 4, and regulatory microRNA (miR-21) were analyzed using quantitative real-time PCR. Ventricle-to-body-weight-ratio was significantly higher in PP2Ac...

  12. Periostal hypertrophic osteopathy of bones (long bones) in colitis ulcerosa in adolescents

    Energy Technology Data Exchange (ETDEWEB)

    Bargon, G.; Arlart, I.

    1982-03-01

    The article reports on a 14-year old girl with periostal new formation of bones at the long bones of the lower arms, the femora and the lower legs the individual phalanges and metacarpalia after colitis ulcerosa which had lasted for several years and had progressed stagewise. After a clinically recorded new attack the periostal new formations of bone progressed. Some time after the last attack of colitis the periostal changes in the bones partially receded. The article discusses the hypothetic explanations aiming at interpreting the pathogenesis of hypertrophic osteoarthropathies and periostoses, as given in the literature.

  13. Takotsubo cardiomyopathy in a patient with previously undiagnosed hypertrophic cardiomyopathy with obstruction.

    Science.gov (United States)

    Brabham, William W; Lewis, Geoffrey F; Bonnema, David D; Nielsen, Christopher D; O'Brien, Terrence X

    2011-01-01

    Takotsubo cardiomyopathy (TCM) is usually characterized by left ventricular anteroapical dysfunction in the absence of significant coronary disease commonly precipitated by an emotional or stressful trigger. Hypertrophic cardiomyopathy (HCM) is usually diagnosed on the basis of symptoms, family history, echocardiography, or by the presence of a characteristic murmur. We report a unique case of TCM occurring in a patient with previously undiagnosed HCM with left ventricular outflow tract (LVOT) obstruction who presented with an acute coronary syndrome and ultimately underwent successful alcohol septal ablation. The potential pathophysiologic correlations are discussed. Copyright © 2011 Elsevier Inc. All rights reserved.

  14. Two different cardiomyopathies in a single patient : hypertrophic cardiomyopathy and left ventricular noncompaction.

    Science.gov (United States)

    Sunbul, M; Ozben, B; Mutlu, B

    2013-05-01

    Hypertrophic cardiomyopathy is a complex and relatively common genetic disorder characterized by left ventricular (LV) hypertrophy, usually associated with a nondilated and hyperdynamic chamber with heterogeneous phenotypic expression and clinical course. On the other hand, LV noncompaction is an uncommon cardiomyopathy characterized by the persistence of fetal myocardium with a pattern of prominent trabecular meshwork and deep intertrabecular recesses, systolic dysfunction, and LV dilatation. We report a 29-year-old man with these two different inherent conditions. Our case raises the possibility of a genetic mutation common to these two clinical entities or different gene mutations existing in the same individual.

  15. Hypertrophic Cardiomyopathy After a Single Dose of Dexamethasone in a Preterm Infant

    Directory of Open Access Journals (Sweden)

    Yusuf Kale

    2015-08-01

    Full Text Available Dexamethasone is widely used in preterm infants with severe pulmonary disease. Hypertrophic cardiomyopathy (HCM is a transient side effect observed after multiple doses of dexamethasone. We report a preterm infant with myocardial hypertrophy after a single dose of dexamethasone (0.5 mg/kg used to treat laryngeal edema secondary to prolonged intubation. A benign course was observed without left ventricular outflow tract obstruction and with recovery within 4 weeks. Myocardial effects of dexamethasone may be independent of dose and duration of treatment. The risk/benefit ratio must be carefully considered before using even a single dose of dexamethasone in preterm infants.

  16. Hypertrophic Pachymeningitis as an Early Manifestation of Relapsing Polychondritis: Case Report and Review of the Literature.

    Science.gov (United States)

    Ushiyama, Satoru; Kinoshita, Tomomi; Shimojima, Yasuhiro; Ohashi, Nobuhiko; Kishida, Dai; Miyazaki, Daigo; Nakamura, Katsuya; Sekijima, Yoshiki; Ikeda, Shu-Ichi

    2016-01-01

    Neurological involvement in relapsing polychondritis (RP) is relatively rare. We describe the case of an 80-year-old man who presented with hypertrophic pachymeningitis (HP) together with arthritis as the first manifestation of RP. Auricular chondritis, which subsequently determined the diagnosis of RP, occurred a few weeks after the detection of HP. The neurological symptoms, as well as arthritis, were promptly improved by treatment with corticosteroids. It is generally difficult to diagnose RP in the absence of typical cartilaginous involvement; however, the present case suggests that HP may occur as an early clinical manifestation of RP.

  17. Vagal enhancement linking abnormal blood pressure response and subendocardial ischemia in hypertrophic cardiomyopathy.

    Science.gov (United States)

    Kawasaki, Tatsuya; Sugihara, Hiroki

    2014-01-01

    An abnormal blood pressure response to exercise has been reported to be associated with left ventricular subendocardial ischemia in patients with hypertrophic cardiomyopathy (HCM), but the underlying mechanism remains unclear. We report a case of HCM with an abnormal blood pressure response and subendocardial ischemia, in which the analysis of heart rate variability revealed exercise-induced vagal enhancement. The present case highlights the possible mechanism linking abnormal blood pressure response and left ventricular subendocardial ischemia in patients with HCM. ©2013 Wiley Periodicals, Inc.

  18. Increased left ventricular torsion in hypertrophic cardiomyopathy mutation carriers with normal wall thickness

    Science.gov (United States)

    2011-01-01

    Background Increased left ventricular (LV) torsion has been observed in patients with manifest familial hypertrophic cardiomyopathy (HCM), and is thought to be caused by subendocardial dysfunction. We hypothesize that increased LV torsion is already present in healthy mutation carriers with normal wall thickness. Methods Seventeen carriers with an LV wall thickness subendocardial myocardial dysfunction. As similar abnormalities are observed in patients with manifest HCM, the changes in healthy carriers may be target for clinical intervention to delay or prevent the onset of hypertrophy. PMID:21219655

  19. Hypertrophic Cardiomyopathy due to Mitochondrial Disease: Prenatal Diagnosis, Management, and Outcome

    Directory of Open Access Journals (Sweden)

    Lutgardo García-Díaz

    2013-01-01

    Full Text Available A case of prenatally diagnosed fetal hypertrophic cardiomyopathy is reported. The mother was referred to our department at 37 weeks' gestation because of suspected congenital heart disease. Prenatal echocardiography showed biventricular hypertrophy and pericardial effusion, without additional abnormalities. Postnatal echocardiography confirmed prenatal diagnosis. Neonatal EKG showed biventricular hypertrophy and Wolff-Parkinson-White syndrome. Skeletal muscle biopsy was consistent with mitochondrial oxidative phosphorylation defect involving a combined defect of respiratory complexes I and IV. Echocardiographic followup during the first year of life showed progressive regression of hypertrophy and evolution to left ventricular myocardial noncompaction.

  20. Hypertrophic obstructive cardiomyopathy in rheumatoid arthtritis - coincidence or association? A case report.

    Science.gov (United States)

    Moyssakis, I; Lionakis, N; Votteas, V

    2009-01-01

    A 60-year-old woman with a history of rheumatoid arthritis was admitted to the hospital for investigation of dyspnea on exertion (New York Heart Association class II), polyarthralgias and mild fever. An echocardiogram revealed asymmetric hypertrophy of the interventricular septum with signs of subaortic obstruction.The coexistence of rheumatoid arthritis and hypertrophic cardiomyopathy could be connected with the human lymphocyte antigen DR4, which is common in both conditions. Further studies are necessary to assess whether a true association of the above diseases exists.

  1. Hypertrophic obstructive cardiomyopathy in rheumatoid arthtritis – coincidence or association? A case report

    Science.gov (United States)

    Moyssakis, I; Lionakis, N; Votteas, V

    2009-01-01

    A 60-year-old woman with a history of rheumatoid arthritis was admitted to the hospital for investigation of dyspnea on exertion (New York Heart Association class II), polyarthralgias and mild fever. An echocardiogram revealed asymmetric hypertrophy of the interventricular septum with signs of subaortic obstruction. The coexistence of rheumatoid arthritis and hypertrophic cardiomyopathy could be connected with the human lymphocyte antigen DR4, which is common in both conditions. Further studies are necessary to assess whether a true association of the above diseases exists. PMID:19492031

  2. Progress toward the maintenance and repair of degenerating retinal circuitry.

    Science.gov (United States)

    Vugler, Anthony A

    2010-01-01

    Retinal diseases such as age-related macular degeneration and retinitis pigmentosa remain major causes of severe vision loss in humans. Clinical trials for treatment of retinal degenerations are underway and advancements in our understanding of retinal biology in health/disease have implications for novel therapies. A review of retinal biology is used to inform a discussion of current strategies to maintain/repair neural circuitry in age-related macular degeneration, retinitis pigmentosa, and Type 2 Leber congenital amaurosis. In age-related macular degeneration/retinitis pigmentosa, a progressive loss of rods/cones results in corruption of bipolar cell circuitry, although retinal output neurons/photoreceptive melanopsin cells survive. Visual function can be stabilized/enhanced after treatment in age-related macular degeneration, but in advanced degenerations, reorganization of retinal circuitry may preclude attempts to restore cone function. In Type 2 Leber congenital amaurosis, useful vision can be restored by gene therapy where central cones survive. Remarkable progress has been made in restoring vision to rodents using light-responsive ion channels inserted into bipolar cells/retinal ganglion cells. Advances in genetic, cellular, and prosthetic therapies show varying degrees of promise for treating retinal degenerations. While functional benefits can be obtained after early therapeutic interventions, efforts should be made to minimize circuitry changes as soon as possible after rod/cone loss. Advances in retinal anatomy/physiology and genetic technologies should allow refinement of future reparative strategies.

  3. Age related macular degeneration and visual disability.

    Science.gov (United States)

    Christoforidis, John B; Tecce, Nicola; Dell'Omo, Roberto; Mastropasqua, Rodolfo; Verolino, Marco; Costagliola, Ciro

    2011-02-01

    Age-related macular degeneration (AMD) is the leading cause of central blindness or low vision among the elderly in industrialized countries. AMD is caused by a combination of genetic and environmental factors. Among modifiable environmental risk factors, cigarette smoking has been associated with both the dry and wet forms of AMD and may increase the likelihood of worsening pre-existing AMD. Despite advances, the treatment of AMD has limitations and affected patients are often referred for low vision rehabilitation to help them cope with their remaining eyesight. The characteristic visual impairment for both forms of AMD is loss of central vision (central scotoma). This loss results in severe difficulties with reading that may be only partly compensated by magnifying glasses or screen-projection devices. The loss of central vision associated with the disease has a profound impact on patient quality of life. With progressive central visual loss, patients lose their ability to perform the more complex activities of daily living. Common vision aids include low vision filters, magnifiers, telescopes and electronic aids. Low vision rehabilitation (LVR) is a new subspecialty emerging from the traditional fields of ophthalmology, optometry, occupational therapy, and sociology, with an ever-increasing impact on the usual concepts of research, education, and services for visually impaired patients. Relatively few ophthalmologists practise LVR and fewer still routinely use prismatic image relocation (IR) in AMD patients. IR is a method of stabilizing oculomotor functions with the purpose of promoting better function of preferred retinal loci (PRLs). The aim of vision rehabilitation therapy consists in the achievement of techniques designed to improve PRL usage. The use of PRLs to compensate for diseased foveae has offered hope to these patients in regaining some function. However, in a recently published meta-analysis, prism spectacles were found to be unlikely to be of

  4. Infantile hypertrophic pyloric stenosis and subsequent ulcer dyspepsia. A follow-up study of medically and surgically treated patients

    DEFF Research Database (Denmark)

    Rasmussen, L; Hansen, L P; Qvist, N

    1988-01-01

    Infantile hypertrophic pyloric stenosis was treated in 324 cases in 1950-1966. At follow-up 19-35 years later, 296 of the patients could be traced, and 284 replied to a questionnaire concerning ulcer dyspepsia. Among the 80 patients who had been medically treated for pyloric stenosis, the prevale......Infantile hypertrophic pyloric stenosis was treated in 324 cases in 1950-1966. At follow-up 19-35 years later, 296 of the patients could be traced, and 284 replied to a questionnaire concerning ulcer dyspepsia. Among the 80 patients who had been medically treated for pyloric stenosis...

  5. Animal models of disc degeneration and major genetic strategies.

    Science.gov (United States)

    Sun, Fu; Qu, Ji-Ning; Zhang, Yin-Gang

    2013-01-01

    The establishment of a reliable animal model of lumbar disc degeneration (AMDD) is important for studying pathogenesis and evaluating treatment effectiveness. However, an ideal AMDD for use in laboratory studies has not yet been produced. This retrospective study reviews and compares several common AMDD and discusses their strengths and weaknesses. We also suggest a new method for establishing future AMDD. The identified genes associated with disc degeneration are susceptibility genes, which elevate risk but do not necessarily lead to disease occurrence. We propose to identify families with hereditary disc degeneration, find major casual genes with exome sequencing, and establish transgenic animal models. This approach may help us to build an improved AMDD.

  6. Acute energy restriction triggers Wallerian degeneration in mouse

    DEFF Research Database (Denmark)

    Alvarez, Susana; Moldovan, Mihai; Krarup, Christian

    2008-01-01

    : (i) a transient conduction failure that rapidly normalized within one hour of washout and (ii) subsequent Wallerian degeneration of some axons confirmed at morphological studies. Taken together, these data support the hypothesis that neurotoxicity may be caused by energy restriction. Since......Acute exposure of peripheral axons to the free radical Nitric Oxide (NO) may trigger conduction block and, if prolonged, Wallerian degeneration. It was hypothesized that this neurotoxic effect of NO may be due primarily to energy restriction by inhibition of mitochondrial respiration. We compared...... the pharmacologic effect of NO and DNP was only transient, our data suggest that even a brief period of focal energy restriction can trigger Wallerian degeneration....

  7. Corticobasal degeneration as a cognitive disorder.

    Science.gov (United States)

    Graham, Naida L; Bak, Thomas H; Hodges, John R

    2003-11-01

    The presence of cognitive impairment in corticobasal degeneration (CBD) is now widely recognised. Our review of the literature reveals that, although the pattern and severity of neuropsychological impairments can be highly variable across patients, several general trends can be identified. The most characteristic impairments are limb apraxia (usually ideomotor), constructional and visuospatial difficulties, acalculia, frontal dysfunction, and nonfluent aphasia. The limb apraxia is associated with deficits in drawing, copying, and handwriting, but there is emerging evidence that the problems with handwriting are not due exclusively to the apraxia. The findings with respect to episodic memory are more variable, but when there is impairment in this area, it tends to be milder than that seen in Alzheimer's disease. Semantic memory functioning appears relatively preserved but has been poorly studied. Problems with speech are common, and may be due to dysarthria or buccofacial apraxia. Aphasia, although initially considered rare, is in fact a common accompaniment of CBD, may be the presenting feature, and is typically nonfluent in type. More systematic investigation of the clinical and neuropathological overlap between progressive nonfluent aphasia (generally considered to be a form of frontotemporal dementia) and CBD is needed.

  8. Quantum kinetic theories in degenerate plasmas

    Science.gov (United States)

    Brodin, Gert; Ekman, Robin; Zamanian, Jens

    2017-01-01

    In this review we give an overview of the recent work on quantum kinetic theories of plasmas. We focus, in particular, on the case where the electrons are fully degenerate. For such systems, perturbation methods using the distribution function can be problematic. Instead we present a model that considers the dynamics of the Fermi surface. The advantage of this model is that, even though the value of the distribution function can be greatly perturbed outside the equilibrium Fermi surface, deformation of the Fermi surface is small up to very large amplitudes. Next, we investigate the short-scale dynamics for which the Wigner-Moyal equation replaces the Vlasov equation. In particular, we study wave-particle interaction, and deduce that new types of wave damping can occur due to the simultaneous absorption (or emission) of multiple wave quanta. Finally, we consider exchange effects within a quantum kinetic formalism to find a model that is more accurate than those using exchange potentials from density functional theory. We deduce the exchange corrections to the dispersion relations for Langmuir and ion-acoustic waves. In comparison to results based on exchange potentials deduced from density functional theory we find that the latter models are reasonably accurate for Langmuir waves, but rather inaccurate for ion acoustic waves.

  9. Correlations in a partially degenerate electron plasma

    Energy Technology Data Exchange (ETDEWEB)

    Chihara, Junzo [Japan Atomic Energy Research Inst., Tokai, Ibaraki (Japan). Tokai Research Establishment

    1998-03-01

    The density-functional theory proves that an ion-electron mixture can be treated as a one-component liquid interacting only via a pairwise interaction in the evaluation of the ion-ion radial distribution function (RDF), and provides a set of integral equations: one is an integral equation for the ion-ion RDF and another for an effective ion-ion interaction, which depends on the ion-ion RDF. This formulation gives a set of integral equation to calculate plasma structures with combined use of the electron-electron correlations in a partially degenerate electron plasma. Therefore, it is important for this purpose to determine the electron-electron correlations at a arbitrary temperature. Here, they are calculated by the quantal version of the hypernetted chain (HNC) equation. On the basis of the jellium-vacancy model, the ionic and electronic structures of rubidium are calculated for the range from liquid metal to plasma states by increasing the temperature at the fixed density using the electron-correlation results. (author)

  10. Nerve degeneration in inguinal hernia specimens.

    Science.gov (United States)

    Amato, G; Ober, E; Romano, G; Salamone, G; Agrusa, A; Gulotta, G; Bussani, R

    2011-02-01

    The histological study of the herniated inguinal area is rare in the literature. This report is focused on the detection of structural changes of the nerves within tissues bordering the inguinal hernia of cadavers. Their physiopathological consequences are hypothesized. Primary inguinal hernia was diagnosed in 30 fresh cadavers. Tissue specimens from the inguinal region close to and around the hernia opening were excised for histological examination. A control of the data was achieved through tissue samples excised from equivalent sites of the inguinal region in 15 cadavers without hernia. The detected nerves in the inguinal area demonstrated pathological changes such as fibrotic degeneration, atrophy, and fatty dystrophy of the axons. The thickening of the perineural sheath was constantly seen. These findings were consistently present, independent of the hernia type. The detected nerve alterations lead us to imagine a worsening, or even the cessation, of the nervous impulse to the muscles, leading to atrophy and weakening of the abdominal wall. This could represent one of the multifactorial causes of hernia genesis.

  11. Flavour identification in frontotemporal lobar degeneration.

    Science.gov (United States)

    Omar, Rohani; Mahoney, Colin J; Buckley, Aisling H; Warren, Jason D

    2013-01-01

    Deficits of flavour processing may be clinically important in frontotemporal lobar degeneration (FTLD). To examine flavour processing in FTLD. We studied flavour identification prospectively in 25 patients with FTLD (12 with behavioural variant frontotemporal dementia (bvFTD), eight with semantic variant primary progressive aphasia (svPPA), five with non-fluent variant primary progressive aphasia (nfvPPA)) and 17 healthy control subjects, using a new test based on cross-modal matching of flavours to words and pictures. All subjects completed a general neuropsychological assessment, and odour identification was also assessed using a modified University of Pennsylvania Smell Identification Test. Brain MRI volumes from the patient cohort were analysed using voxel-based morphometry to identify regional grey matter associations of flavour identification. Relative to the healthy control group, the bvFTD and svPPA subgroups showed significant (pidentification and all three FTLD subgroups showed deficits of odour identification. Flavour identification performance did not differ significantly between the FTLD syndromic subgroups. Flavour identification performance in the combined FTLD cohort was significantly (pidentification and this is underpinned by grey matter atrophy in an anteromedial temporal lobe network. These findings may have implications for our understanding of abnormal eating behaviour in these diseases.

  12. Animal models of age related macular degeneration

    Science.gov (United States)

    Pennesi, Mark E.; Neuringer, Martha; Courtney, Robert J.

    2013-01-01

    Age related macular degeneration (AMD) is the leading cause of vision loss of those over the age of 65 in the industrialized world. The prevalence and need to develop effective treatments for AMD has lead to the development of multiple animal models. AMD is a complex and heterogeneous disease that involves the interaction of both genetic and environmental factors with the unique anatomy of the human macula. Models in mice, rats, rabbits, pigs and non-human primates have recreated many of the histological features of AMD and provided much insight into the underlying pathological mechanisms of this disease. In spite of the large number of models developed, no one model yet recapitulates all of the features of human AMD. However, these models have helped reveal the roles of chronic oxidative damage, inflammation and immune dysregulation, and lipid metabolism in the development of AMD. Models for induced choroidal neovascularization have served as the backbone for testing new therapies. This article will review the diversity of animal models that exist for AMD as well as their strengths and limitations. PMID:22705444

  13. Precursors of age-related macular degeneration

    DEFF Research Database (Denmark)

    Munch, Inger Christine; Toft, Ulla; Linneberg, Allan

    2016-01-01

    PURPOSE: To investigate associations of very early age-related macular degeneration (AMD) with daily intake of vitamin A, beta-carotene, vitamin E, vitamin C, zinc and copper and interactions with AMD-associated polymorphisms in complement factor H (CFHY402H) and ARMS2/LOC387715. METHODS: Cross......-sectional study of 848 subjects aged 30-60 years from the Inter99 Eye Study. Daily intake of vitamins and minerals was estimated from a 198-item food frequency questionnaire. Digital fundus photographs were recorded in red-free illumination and graded for macular drusen >63 μm and numerous (>20) small hard...... for recruitment group, age and sex. There was a significant interaction with CFHY402H (p = 0.038). Among 504 participants with CFHY402H, the relative risk of having macular drusen >63 μm was increased in participants in the highest quartile of vitamin A intake (odds ratio = 2.58; CI95 1.16-5.73, p = 0...

  14. Degenerate nucleophilic substitution in phosphonium salts.

    Science.gov (United States)

    Jennings, Elizabeth V; Nikitin, Kirill; Ortin, Yannick; Gilheany, Declan G

    2014-11-19

    Rates and energy barriers of degenerate halide substitution on tetracoordinate halophosphonium cations have been measured by NMR techniques (VT and EXSY) using a novel experimental design whereby a chiral substituent ((s)Bu) lifts the degeneracy of the resultant salts. Concomitantly, a viable computational approach to the system was developed to gain mechanistic insights into the structure and relative stabilities of the species involved. Both approaches strongly suggest a two-step mechanism of formation of a pentacoordinate dihalophosphorane via backside attack followed by dissociation, resulting in inversion of configuration at phosphorus. The experimentally determined barriers range from salts. Calculations determined that this was due to the easier accessibility in solution of pentacoordinate dichlorophosphoranes when compared to analogous dibromophosphoranes. In line with the proposed associative mechanism, bulky substituents slow the reaction in the order Me < Et < (i)Pr < (t)Bu. Bulky substituents affect the shape of the reaction energy profile so that the pentacoordinate intermediate is destabilized eventually becoming a transition state. The magnitude of the steric effects is comparable to that of the same substituents on substitution at primary alkyl halides, which can be rationalized by the relatively longer P-C bonds. The reaction displays first-order kinetics due to the prevalence of tight- or solvent-separated ion pairs in solution. Three-dimensional reaction potential energy profiles (More O'Ferrall-Jencks plots) indicated a relatively shallow potential well corresponding to the trigonal bipyramid intermediate flanked by two transition states.

  15. CERKL knockdown causes retinal degeneration in zebrafish.

    Directory of Open Access Journals (Sweden)

    Marina Riera

    Full Text Available The human CERKL gene is responsible for common and severe forms of retinal dystrophies. Despite intense in vitro studies at the molecular and cellular level and in vivo analyses of the retina of murine knockout models, CERKL function remains unknown. In this study, we aimed to approach the developmental and functional features of cerkl in Danio rerio within an Evo-Devo framework. We show that gene expression increases from early developmental stages until the formation of the retina in the optic cup. Unlike the high mRNA-CERKL isoform multiplicity shown in mammals, the moderate transcriptional complexity in fish facilitates phenotypic studies derived from gene silencing. Moreover, of relevance to pathogenicity, teleost CERKL shares the two main human protein isoforms. Morpholino injection has been used to generate a cerkl knockdown zebrafish model. The morphant phenotype results in abnormal eye development with lamination defects, failure to develop photoreceptor outer segments, increased apoptosis of retinal cells and small eyes. Our data support that zebrafish Cerkl does not interfere with proliferation and neural differentiation during early developmental stages but is relevant for survival and protection of the retinal tissue. Overall, we propose that this zebrafish model is a powerful tool to unveil CERKL contribution to human retinal degeneration.

  16. Alzheimer's disease neurofibrillary degeneration: pivotal and multifactorial.

    Science.gov (United States)

    Iqbal, Khalid; Wang, Xiaochuan; Blanchard, Julie; Liu, Fei; Gong, Cheng-Xin; Grundke-Iqbal, Inge

    2010-08-01

    Independent of the aetiology, AD (Alzheimer's disease) neurofibrillary degeneration of abnormally hyperphosphorylated tau, a hallmark of AD and related tauopathies, is apparently required for the clinical expression of the disease and hence is a major therapeutic target for drug development. However, AD is multifactorial and heterogeneous and probably involves several different aetiopathogenic mechanisms. On the basis of CSF (cerebrospinal fluid) levels of Abeta(1-42) (where Abeta is amyloid beta-peptide), tau and ubiquitin, five different subgroups, each with its own clinical profile, have been identified. A successful development of rational therapeutic disease-modifying drugs for AD will require understanding of the different aetiopathogenic mechanisms involved and stratification of AD patients by different disease subgroups in clinical trials. We have identified a novel aetiopathogenic mechanism of AD which is initiated by the cleavage of SET, also known as inhibitor-2 (I(2)(PP2A)) of PP2A (protein phosphatase 2A) at Asn(175) into N-terminal (I(2NTF)) and C-terminal (I(2CTF)) halves and their translocation from the neuronal nucleus to the cytoplasm. AAV1 (adeno-associated virus 1)-induced expression of I(2CTF) in rat brain induces inhibition of PP2A activity, abnormal hyperphosphorylation of tau, neurodegeneration and cognitive impairment in rats. Restoration of PP2A activity by inhibition of the cleavage of I(2)(PP2A)/SET offers a promising therapeutic opportunity in AD with this aetiopathogenic mechanism.

  17. Regional cerebral glucose metabolic changes in oculopalatal myoclonus: implication for neural pathways, underlying the disorder

    Energy Technology Data Exchange (ETDEWEB)

    Cho, Sang Soo; Moon, So Young; Kim, Ji Soo; Kim, Sang Eun [College of Medicine, Seoul National University, Seoul (Korea, Republic of)

    2004-07-01

    Palatal myoclonus (PM) is characterized by rhythmic involuntary jerky movements of the soft palate of the throat. When associated with eye movements, it is called oculopalatal myoclonus (OPM). Ordinary PM is characterized by hypertrophic olivary degeneration, a trans-synaptic degeneration following loss of neuronal input to the inferior olivary nucleus due to an interruption of the Guillain-Mollaret triangle usually by a hemorrhage. However, the neural pathways underlying the disorder are uncertain. In an attempt to understand the pathologic neural pathways, we examined the metabolic correlates of this tremulous condition. Brain FDG PET scans were acquired in 8 patients with OPM (age, 49.9{+-}4.6 y: all males: 7 with pontine hemorrhage, 1 with diffuse brainstem infarction) and age-matched 50 healthy males (age, 50.7{+-} 9.0) and the regional glucose metabolism compared using SPM99. For group analysis, the hemispheres containing lesions were assigned to the right side of the brain. Patients with OPM had significant hypometabolism in the ipsilateral (to the lesion) brainstem and superior temporal and parahippocampal gyri (P < 0.05 corrected, k = 100). By contrast, there was significant hypermetabolism in the contralateral middle and inferior temporal gyri, thalamus, middle frontal gyrus and precuneus (P < 0.05 corrected, k=l00). Our data demonstrate the distinct metabolic changes between several ipsilateral and contralateral brain regions (hypometabolism vs. hypermetabolism) in patients with OPM. This may provide clues for understanding the neural pathways underlying the disorder.

  18. β-catenin activity in late hypertrophic chondrocytes locally orchestrates osteoblastogenesis and osteoclastogenesis

    Science.gov (United States)

    Houben, Astrid; Weissenböck, Martina; Graf, Julian; Teufel, Stefan; von der Mark, Klaus

    2016-01-01

    Trabecular bone formation is the last step in endochondral ossification. This remodeling process of cartilage into bone involves blood vessel invasion and removal of hypertrophic chondrocytes (HTCs) by chondroclasts and osteoclasts. Periosteal- and chondrocyte-derived osteoprogenitors utilize the leftover mineralized HTC matrix as a scaffold for primary spongiosa formation. Here, we show genetically that β-catenin (encoded by Ctnnb1), a key component of the canonical Wnt pathway, orchestrates this remodeling process at multiple levels. Conditional inactivation or stabilization of β-catenin in HTCs by a Col10a1-Cre line locally modulated osteoclastogenesis by altering the Rankl:Opg ratio in HTCs. Lack of β-catenin resulted in a severe decrease of trabecular bone in the embryonic long bones. Gain of β-catenin activity interfered with removal of late HTCs and bone marrow formation, leading to a continuous mineralized hypertrophic core in the embryo and resulting in an osteopetrotic-like phenotype in adult mice. Furthermore, β-catenin activity in late HTCs is required for chondrocyte-derived osteoblastogenesis at the chondro-osseous junction. The latter contributes to the severe trabecular bone phenotype in mutants lacking β-catenin activity in HTCs. PMID:27621061

  19. Percutaneous Septal Ablation in Hypertrophic Obstructive Cardiomyopathy: From Experiment to Standard of Care

    Directory of Open Access Journals (Sweden)

    Lothar Faber

    2014-01-01

    Full Text Available Hypertrophic cardiomyopathy (HCM is one of the more common hereditary cardiac conditions. According to presence or absence of outflow obstruction at rest or with provocation, a more common (about 60–70% obstructive type of the disease (HOCM has to be distinguished from the less common (30–40% nonobstructive phenotype (HNCM. Symptoms include exercise limitation due to dyspnea, angina pectoris, palpitations, or dizziness; occasionally syncope or sudden cardiac death occurs. Correct diagnosis and risk stratification with respect to prophylactic ICD implantation are essential in HCM patient management. Drug therapy in symptomatic patients can be characterized as treatment of heart failure with preserved ejection fraction (HFpEF in HNCM, while symptoms and the obstructive gradient in HOCM can be addressed with beta-blockers, disopyramide, or verapamil. After a short overview on etiology, natural history, and diagnostics in hypertrophic cardiomyopathy, this paper reviews the current treatment options for HOCM with a special focus on percutaneous septal ablation. Literature data and the own series of about 600 cases are discussed, suggesting a largely comparable outcome with respect to procedural mortality, clinical efficacy, and long-term outcome.

  20. Higher copeptin levels are associated with worse outcome in patients with hypertrophic cardiomyopathy.

    Science.gov (United States)

    Sahin, Irfan; Gungor, Baris; Ozkaynak, Berk; Uzun, Fatih; Küçük, Suat Hayri; Avci, Ilhan Iker; Ozal, Ender; Ayça, Burak; Cetın, Sukru; Okuyan, Ertugrul; Dinckal, Mustafa Hakan

    2017-01-01

    Correlation of increased copeptin levels with various cardiovascular diseases has been described. The clinical use of copeptin levels in patients with hypertrophic cardiomyopathy (HCM) has not been investigated before. In this study, we aimed to investigate the prognostic value of copeptin levels in patients with hypertrophic cardiomyopathy (HCM). HCM was defined as presence of left ventricular wall thickness ≥15 mm in a subject without any concomitant disease that may cause left ventricular hypertrophy. Levels of copeptin and plasma N-terminal probrain natriuretic peptide (NT-proBNP) were evaluated prospectively in 24 obstructive HCM patients, 36 nonobstructive HCM patients, and 36 age- and sex-matched control subjects. Blood samples were collected in the morning between 7 and 9 am after overnight fasting. Patients were followed for 24 months. Hospitalization with diagnosis of heart failure/arrhythmia, implantable cardioverter-defibrillator implantation, and cardiac mortality were accepted as adverse cardiac events. Copeptin and NT-proBNP levels were higher in the HCM group compared with controls (14.1 vs 8.4 pmol/L, P Copeptin and NT-proBNP levels were higher in the obstructive HCM subgroup compared with the nonobstructive HCM subgroup (18.3 vs 13.1 pmol/L, P copeptin and NT-proBNP levels remained as independent predictors of heart failure (P Copeptin and NT-proBNP levels were significantly higher in patients with obstructive HCM, and higher levels were associated with worse outcome. © 2016 Wiley Periodicals, Inc.

  1. Potential mechanisms for a role of metabolic stress in hypertrophic adaptations to resistance training.

    Science.gov (United States)

    Schoenfeld, Brad J

    2013-03-01

    It is well established that regimented resistance training can promote increases in muscle hypertrophy. The prevailing body of research indicates that mechanical stress is the primary impetus for this adaptive response and studies show that mechanical stress alone can initiate anabolic signalling. Given the dominant role of mechanical stress in muscle growth, the question arises as to whether other factors may enhance the post-exercise hypertrophic response. Several researchers have proposed that exercise-induced metabolic stress may in fact confer such an anabolic effect and some have even suggested that metabolite accumulation may be more important than high force development in optimizing muscle growth. Metabolic stress pursuant to traditional resistance training manifests as a result of exercise that relies on anaerobic glycolysis for adenosine triphosphate production. This, in turn, causes the subsequent accumulation of metabolites, particularly lactate and H(+). Acute muscle hypoxia associated with such training methods may further heighten metabolic buildup. Therefore, the purpose of this paper will be to review the emerging body of research suggesting a role for exercise-induced metabolic stress in maximizing muscle development and present insights as to the potential mechanisms by which these hypertrophic adaptations may occur. These mechanisms include increased fibre recruitment, elevated systemic hormonal production, alterations in local myokines, heightened production of reactive oxygen species and cell swelling. Recommendations are provided for potential areas of future research on the subject.

  2. Alcohol Septal Ablation for Obstructive Hypertrophic Cardiomyopathy: A Word of Endorsement.

    Science.gov (United States)

    Liebregts, Max; Vriesendorp, Pieter A; Ten Berg, Jurrien M

    2017-07-25

    Twenty years after the introduction of alcohol septal ablation (ASA) for the treatment of obstructive hypertrophic cardiomyopathy, the arrhythmogenicity of the ablation scar appears to be overemphasized. When systematically reviewing all studies comparing ASA with myectomy with long-term follow-up, (aborted) sudden cardiac death and mortality rates were found to be similarly low. The focus should instead shift toward lowering the rate of reinterventions and pacemaker implantations following ASA because, in this area, ASA still seems inferior to myectomy. Part of the reason for this difference is that ASA is limited by the route of the septal perforators, whereas myectomy is not. Improvement may be achieved by: 1) confining ASA to hypertrophic cardiomyopathy centers of excellence with high operator volumes; 2) improving patient selection using multidisciplinary heart teams; 3) use of (3-dimensional) myocardial contrast echocardiography for selecting the correct septal (sub)branch; and 4) use of appropriate amounts of alcohol for ASA. Copyright © 2017 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  3. Evidence of hypertrophic osteoarthropathy in human skeletal remains from pre-Hispanic Mesoamerica.

    Science.gov (United States)

    Martínez-Lavín, M; Mansilla, J; Pineda, C; Pijoán, C; Ochoa, P

    1994-02-01

    Hypertrophic osteoarthropathy is one of the earliest recognized disease entities in the history of medicine. It has a peculiar periosteal proliferation distinctive from other bone diseases. In its advanced stage, it leaves an indelible mark on the skeleton. It has been recently shown that digital clubbing is accompanied by a bone remodeling process of the underlying phalanges. Thus, theoretically, this entity can be recognized in ancient human skeletal remains. We studied part of the collection of skeletal remains from pre-Hispanic Mesoamerica preserved at the National Museum of Anthropology of Mexico City. We examined 1000 specimens and found 2 skeletons with widespread, bilateral, symmetric periosteal proliferation of the tubular bones in addition to the bone remodeling changes of the distal phalanges. One of the specimens was from the Formative period (2000 B.C. to 100 A.D.). We conclude that hypertrophic osteoarthropathy can be recognized in ancient human skeletal remains and that this disease was present in Mesoamerica near the time of the original description of clubbing by Hippocrates about 2500 years ago.

  4. Virtual Cardiac Surgery Using CFD: Application to Septal Myectomy in Obstructive Hypertrophic Cardiomyopathy

    Science.gov (United States)

    Vedula, Vijay; Mittal, Rajat; Abraham, Theodore

    2011-11-01

    Obstructive hypertrophic cardiomyopathy (HOCM) is characterized by ventricular wall thickening, diastolic dysfunction, and dynamic outflow tract obstruction, all of which strongly influence the vortex dynamics and pressure distribution in the left ventricle (LV). Severe cases of HCM are usually managed through septal myectomy where the surgeon resects the hypertrophic mass. Surgeons currently try to remove as much tissue as possible in order to optimize the post surgical result. However, excessive debulking increases the chance of ventricular septal defects, bundle branch block or complete heart block, and aneurysmal septal thinning. On the other hand, insufficient tissue removal also leads to unsatisfactory outcomes in terms of reduction of outflow tract pressure gradient. Knowing how much muscle to remove and where to remove it from could reduce the likelihood of complications and suboptimal outcomes. In the present study, we employ an immersed boundary solver to model the effect of septal myectomy for ventricles with HOCM and demonstrate the potential of such an approach for surgical planning. Computational resources were provided by the National Institute of Computational Science under Tergrid grant number TG-CTS100002.

  5. Ultrasound-guided paravertebral block for pyloromyotomy in 3 neonates with congenital hypertrophic pyloric stenosis

    Directory of Open Access Journals (Sweden)

    Javier Mata-Gómez

    2015-08-01

    Full Text Available BACKGROUND AND OBJECTIVES: Hypertrophic pyloric stenosis is a relatively common affection of gastrointestinal tract in childhood that results in symptoms, such as projectile vomiting and metabolic disorders that imply a high risk of aspiration during anesthetic induction. In this way, the carrying out of a technique with general anesthesia and intravenous rapid sequence induction, preoxygenation and cricoid pressure are recommended. After the correction of systemic metabolic alkalosis and pH normalization, cerebrospinal fluid can keep a state of metabolic alkalosis. This circumstance, in addition to the residual effect of neuromuscular blocking agents, inhalant anesthetics and opioids could increase the risk of postoperative apnea after a general anesthesia.CASE REPORT: We present the successful management in 3 neonates in those a pyloromyotomy was carried out because they had presented congenital hypertrophic pyloric stenosis. This procedure was done under general anesthesia with orotracheal intubation and rapid sequence induction. Then, ultrasound-guided paravertebral block was performed as analgesic method without the need for administrating opioids within intraoperative period and keeping an appropriate analgesic level.CONCLUSIONS: Local anesthesia has demonstrated to be safe and effective in pediatric practice. We consider the ultrasound-guided paravertebral block with one dose as a possible alternative for other local techniques described, avoiding the use of opioids and neuromuscular blocking agents during general anesthesia, and reducing the risk of central apnea within postoperative period.

  6. Hypertrophic osteopathy associated with mycotic pneumonia in two juvenile elk (Cervus elaphus).

    Science.gov (United States)

    Ferguson, Nicole M; Lévy, Michel; Ramos-Vara, José A; Baird, Debra K; Wu, Ching Ching

    2008-11-01

    Two yearling bull elk (cervus claphus) from the same farm developed anorexia, weight loss, and lameness. On physical examination, both elk were thin and showed diffuse swelling of all lower limbs. Radiographs of the lower limbs showed periosteal thickening of the distal extremities, consistent with hypertrophic osteopathy. Thoracic radiographs indicated the presence of pulmonary nodules. Cytologic evaluations of tracheal washes on both elk were consistent with inflammation. Acid-fast stains on both samples were negative. Because of the poor prognosis, both elk were euthanized. At necropsy, the carpal, metacarpal, tarsal, and metatarsal bones, as well as the radius, ulna, and tibia had thickening of cortical bone. There were multiple encapsulated nodules throughout the lungs, lymph nodes, and kidney, and smaller nodules in the myocardium. On microscopic examination, these nodules contained myriads of hyphae, and immunohistochemistry for Aspergillus sp. was strongly positive. Aspergillus fumigatus was isolated from affected tissue in 1 elk. Necropsy findings in both elk were consistent with disseminated fungal granulomas and periosteal hyperostosis. This case presents the first description of hypertrophic osteopathy in elk. The source of infection was undetermined, but inhalation of spores from contaminated feed or bedding was suspected.

  7. Invasive assessment of coronary microvascular dysfunction in hypertrophic cardiomyopathy: the index of microvascular resistance

    Energy Technology Data Exchange (ETDEWEB)

    Gutiérrez-Barrios, Alejandro, E-mail: aleklos@hotmail.com [Cardiology Department, Jerez Hospital, Jerez (Spain); Camacho-Jurado, Francisco [Cardiology Department, Punta Europa Hospital, Algeciras (Spain); Díaz-Retamino, Enrique; Gamaza-Chulián, Sergio; Agarrado-Luna, Antonio; Oneto-Otero, Jesús; Del Rio-Lechuga, Ana; Benezet-Mazuecos, Javier [Cardiology Department, Jerez Hospital, Jerez (Spain)

    2015-10-15

    Summary: We present a review of microvascular dysfunction in hypertrophic cardiomyopathy (HCM) and an interesting case of a symptomatic familial HCM patient with inducible ischemia by single photon emission computed tomography. Coronary angiography revealed normal epicardial arteries. Pressure wire measurements of fractional flow reserve (FFR), coronary flow reserve (CFR) and index of microvascular resistance (IMR) demonstrated a significant microcirculatory dysfunction. This is the first such case that documents this abnormality invasively using the IMR. The measurement of IMR, a novel marker of microcirculatory dysfunction, provides novel insights into the pathophysiology of this condition. - Highlights: • Microvascular dysfunction is a common feature in hypertrophic cardiomyopathy (HCM) and represents a strong predictor of unfavorable outcome and cardiovascular mortality. • The index of microvascular resistance (IMR) is a new method for invasively assessing the state of the coronary microcirculation using a single pressure-temperature sensor-tipped coronary wire. • However assessment of IMR in HCM has not been previously reported. We report a case in which microvascular dysfunction is assessed by IMR. This index may be useful in future researches of HCM.

  8. Relationship between electrocardiographic features and distribution of hypertrophy in patients with hypertrophic cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Sato, Tetsuya; Nakamura, Kazufumi; Yamanari, Hiroshi; Ohe, Tohru [Okayama Univ. (Japan). School of Medicine; Yoshinouchi, Takeo

    1998-07-01

    To evaluate the relationship between the distribution of hypertrophy and the electrocardiographic findings in patients with hypertrophic cardiomyopathy (HCM), 54 HCM patients were studied using magnetic resonance imaging. The patients were divided into 4 groups according to hypertrophic patterns: hypertrophy only at the apex (group I, n=12); hypertrophy in both the apex and base (group II, n=20); hypertrophy only at the base with asymmetric septal hypertrophy (ASH) (group IIIa, n=17); and hypertrophy only at the base without ASH (group IIIb, n=5). Abnormal Q waves in leads II, III and aV{sub F} were found in 1/12, 3/20, 10/17 and 0/5, respectively, and in leads I and aV{sub L} they were found in 1/12, 8/20, 4/17 and 1/5, respectively. The largest negative T waves (mm) were found in group I (group I vs group II vs group IIIa vs group IIIb: 15.2{+-}5.3, 8.2{+-}6.1, 1.6{+-}2.0, 0.8{+-}1.3, respectively). The largest positive T waves (mm) were identified in group IIIb (3.8{+-}3.0, 6.8{+-}3.2, 5.8{+-}3.6, 9.3{+-}2.1, respectively). The presence of abnormal Q waves reflected regional hypertrophy in HCM patients but the configuration of T waves represented the difference in the localization of hypertrophy between the basal and apical segments. (author)

  9. Hypertrophic Cardiomyopathy from A to Z: Genetics, Pathophysiology, Imaging, and Management.

    Science.gov (United States)

    Baxi, Ameya Jagdish; Restrepo, Carlos S; Vargas, Daniel; Marmol-Velez, Alejandro; Ocazionez, Daniel; Murillo, Horacio

    2016-01-01

    Hypertrophic cardiomyopathy (HCM) is a heterogeneous group of diseases related to sarcomere gene mutations exhibiting heterogeneous phenotypes with an autosomal dominant mendelian pattern of inheritance. The disorder is characterized by diverse phenotypic expressions and variable natural progression, which may range from dyspnea and/or syncope to sudden cardiac death. It is found across all racial groups and is associated with left ventricular hypertrophy in the absence of another systemic or cardiac disease. The management of HCM is based on a thorough understanding of the underlying morphology, pathophysiology, and clinical course. Imaging findings of HCM mirror the variable expressivity and penetrance heterogeneity, with the added advantage of diagnosis even in cases where a specific mutation may not yet be found. The diagnostic information obtained from imaging varies depending on the specific stage of HCM-phenotype manifestation, including the prehypertrophic, hypertrophic, and later stages of adverse remodeling into the burned-out phase of overt heart failure. However, subtle or obvious, these imaging findings become critical components in diagnosis, management, and follow-up of HCM patients. Although diagnosis of HCM traditionally relies on clinical assessment and transthoracic echocardiography, recent studies have demonstrated increased utility of multidetector computed tomography (CT) and particularly cardiac magnetic resonance (MR) imaging in diagnosis, phenotype differentiation, therapeutic planning, and prognostication. In this article, we provide an overview of the genetics, pathophysiology, and clinical manifestations of HCM, with the spectrum of imaging findings at MR imaging and CT and their contribution in diagnosis, risk stratification, and therapy. (©)RSNA, 2016.

  10. Myocardial Fibrosis in Hypertrophic Cardiomyopathy Demonstrated by Integrated Cardiac F-18 FDG PET/MR

    Energy Technology Data Exchange (ETDEWEB)

    Kong, Eunjung; Lee, Sanghee; Cho, Ihnho [Yeungnam Univ., Daegu (Korea, Republic of)

    2013-09-15

    Hypertrophic cardiomyopathy (HCM) is a common condition defined as a diffuse or segmental left ventricular (LV) hypertrophy with a nondilated and hyperdynamic chamber as well as cardiac arrhythmias. Cardiac MR (CMR) imaging is a key modality for evaluation of HCM. In addition to the assessment of LV wall thickness, LV function and aortic flow, CMR is capable of estimation of late gadolinium enhancement (LGE) in affected myocardium which has been shown to have a direct correlation with incidence and severity of arrhythmias in HCM. In patients with HCM, LGE on CMR is presumed to represent intramyocardial fibrosis. Meanwhile, F-18 FDG myocardial PET has been sporadically studied in HCM, mostly for evaluation of the metabolic status of a hypertrophic myocardial segment, especially after interventions or to demonstrate partial myocardial fibrosis. We presented here the case of a 25-year-old male patient referred for simultaneous F-18 FDG cardiac PET/MR for the evaluation of septal hypertrophy. The PET/MR revealed myocardial fibrosis in the septum associated with FDG-defect and LGE.

  11. Overexpression of mitofilin in the mouse heart promotes cardiac hypertrophy in response to hypertrophic stimuli.

    Science.gov (United States)

    Zhang, Yuan; Xu, Jing; Luo, Yu-Xuan; An, Xi-Zhou; Zhang, Ran; Liu, Guang; Li, Hongliang; Chen, Hou-Zao; Liu, De-Pei

    2014-10-20

    Mitofilin was originally described as a heart muscle protein because of its abundance in the heart tissue; however, its function in the heart is still to be elucidated. Thus, this study aims at investigating the role of mitofilin in the heart in response to hypertrophic stimuli. In this study, a significant increase in mitofilin expression was observed in the hearts of patients with hypertrophic cardiomyopathy. Transgenic (TG) mice with cardiomyocyte-specific overexpression of mitofilin were generated, and cardiac hypertrophy was introduced by transverse aortic constriction (TAC) or chronic infusion of isoproterenol (ISO). In TG mice overexpressing mitofilin, the level of cardiac hypertrophy was significantly greater than that in wild-type (WT) mice after TAC and ISO stimulation. A detailed analysis showed that compared with WT mice, the level of reactive oxygen species was increased after TAC and ISO induction and mitochondrial oxidative phosphorylation (OXPHOS) activity in the TG hearts was lower. These alterations may contribute to the aggravated cardiac hypertrophy observed in response to TAC and ISO stimulation. Over-expression of mitofilin promotes cardiac hypertrophy under pathological conditions both in vivo and in vitro. Mitofilin, a mitochondria protein, is shown to be related to cardiac hypertrophy for the first time, which enhances our understanding of the role of mitochondria in cardiac hypertrophy.

  12. Paquidermoperiostosis (osteoartropatía hipertrófica primaria Pachydermoperiostosis (primary hypertrophic osteoarthropathy

    Directory of Open Access Journals (Sweden)

    Javier A. Cavallasca

    2006-04-01

    Full Text Available La paquidermoperiostosis u osteoartropatía hipertrófica primaria es una rara enfermedad caracterizada por compromiso cutáneo y osteoarticular. Comunicamos dos casos que presentaban hipocratismo digital, uñas en vidrio de reloj, agrandamiento óseo, tumefacción articular y diferentes grados de afectación cutánea, sin otros hallazgos clínicos relevantes. Ambos desconocían antecedentes familiares similares. El estudio radiográfico de las zonas comprometidas mostró periostosis. Con resultados de laboratorio y radiografía de tórax normales, y ausencia de evidencia clínica de otra enfermedad subyacente, se realizó diagnóstico de osteoartropatía hipertrófica primaria.Pachydermoperiostosis or primary hypertrophic osteoarthropathy is a rare disease characterized by cutaneous and osteo-arthicular involvement. We describe two patients with finger clubbing, watch crystal nails, bones thickenings, arthritis and different grades of skin affection, without other clinical manifestations. Both did not know of having relatives with the same alterations. Radiological studies of the affected areas showed periostosis. Because of normal laboratory results and chest radiography plus the absence of other underlying causes, diagnosis of primary hypertrophic osteoarthropathy was made.

  13. Exercise Tl-201 myocardial scintigraphy and coronary blood flow velocity in patients with hypertrophic cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Sugihara, Hiroki; Umamoto, Ikuo; Harada, Yoshiaki (Kyoto Prefectural Univ. of Medicine (Japan)) (and others)

    1992-03-01

    In 22 patients with hypertrophic cardiomyopathy not associated with coronary stenosis, findings of exercise Tl-201 myocardial scintigraphy were reviewed in relation to coronary blood flow velocity. Blood flow velocity of the left antero-inferior segment was recorded by using a Doppler catheter. From time velocity integration in systolic and diastolic phases, both % systolic fraction (%SF) and %diastolic 1st third fraction (%d1F) were obtained. A decreased perfusion was visually assessed on exercise Tl-201 myocardial images. In addition, transient dilation of the left ventricular cavity was quantitatively assessed by expressing it as a transient dilation index (TDI) for subendocardial ischemia. Coronary blood flow velocity was characterized by a decrease in both %SF and %d1F and negative systolic flow. Both %SF and %d1F were inversely correlated with TDI. Coronary negative systolic flow was frequently associated with a decreased perfusion. These results suggest factors such as left ventricular relaxation impairment, other than coronary small vessel lesions, may also be involved in the occurrence of myocardial ischemia in hypertrophic cardiomyopathy. (N.K.).

  14. Phytoplankton biodiversity changes in a shallow tropical reservoir during the hypertrophication process

    Directory of Open Access Journals (Sweden)

    LO. Crossetti

    Full Text Available Study aimed at evaluating phytoplankton biodiversity changes in a shallow tropical reservoir during its hypertrophication process. Samplings were carried out monthly during 8 consecutive years (1997-2004 in 5 depths. Conspicuous limnological changes in the reservoir derived from the presence and/or removal of the water hyacinth, characterized 3 different phases. Over the time series, reservoir changed from a typical polymictic eutrophic system to hypertrophic one, leading to a reduction of approximately 70 species (average 37%. Chlorophyceae accounted for the highest species richness (46% among all algal classes and strictly followed total species richness variation. Internal feedback mechanisms intensification over phase III clearly promoted the sharp decrease in biodiversity. Highest decreases, mainly during springs, occurred simultaneously to the highest Cyanobacteria blooms. Increased turbidity due to heavy phytoplankton blooms suppressed all other algal groups, so that at the end of the present study even Cyanobacteria species richness decreased. Total dissolved phosphorous was included in most of the best selected models used to analyze the temporal patterns in species richness loss. Present data show that biodiversity loss following trophic change was not a single dimension of a single factor but, rather, a template of factors (e.g. light, stability co-varying in consequence of the larger levels of biomass supported in the reservoir.

  15. Continuous rhythm monitoring for ventricular arrhythmias after alcohol septal ablation for hypertrophic cardiomyopathy.

    Science.gov (United States)

    Balt, Jippe C; Wijffels, Maurits C E F; Boersma, Lucas V A; Wever, Eric F D; ten Berg, Jurriën M

    2014-12-01

    The purpose of the present study was to determine the incidence of ventricular arrhythmias before and after alcohol septal ablation (ASA). In patients with hypertrophic obstructive cardiomyopathy (HOCM), gradient reduction by ASA is an alternative for surgical myectomy. However, concerns exist about whether the induction of a myocardial scar during ASA may create substrate for ventricular arrhythmias. The study group consisted of 44 patients in whom ASA was performed for symptomatic, drug-refractory hypertrophic cardiomyopathy. Continuous rhythm monitoring was obtained by implantable loop recorder (n=30) or pacemaker (n=14). Occurrence of ventricular and supraventricular arrhythmias before and after ASA was noted, retrospectively. The ASA procedure was considered successful (resting gradient 30 days after ASA. No cardiac deaths occurred during follow-up. In a low-risk cohort of patients who underwent ASA, in which continuous rhythm monitoring was performed, sustained VT or VF within 30 days occurred in 3 patients (7%) while no VT/VF was observed before ASA. During long-term follow-up, no sustained VT or VF was observed >30 days after ASA. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

  16. A study of retrograde degeneration of median nerve forearm ...

    African Journals Online (AJOL)

    Mona Mokhtar El Bardawil

    Alexandria University, Department of Physical Medicine, Rheumatology and Rehabilitation, Egypt. Received 16 April 2013; accepted 24 August 2013. Available online 22 October 2013. KEYWORDS. Carpal tunnel syndrome;. Electrodiagnosis;. Forearm median mixed study;. Retrograde degeneration. Abstract Introduction: ...

  17. Higher order nonlinear degenerate elliptic problems with weak monotonicity

    Directory of Open Access Journals (Sweden)

    Youssef Akdim

    2006-09-01

    Full Text Available We prove the existence of solutions for nonlinear degenerate elliptic boundary-value problems of higher order. Solutions are obtained using pseudo-monotonicity theory in a suitable weighted Sobolev space.

  18. [Research advances in animal models of intervertebral disc degeneration].

    Science.gov (United States)

    Zhang, Wenli; Liu, Hao; Li, Tanzhu

    2007-11-01

    To review the research advances in animal models of human disc degeneration. The relative articles in recent years were extensively reviewed. Studies both at home and abroad were analyzed and classified. The advantages and disadvantages of each method were compared. Studies were classified as either experimentally induced models or spontaneous models. The induced models were subdivided as mechanical (alteration of forces on the normal disc), structural (injury or chemical alteration) and genetically induced models. Spontaneous models included those animals that naturally developed degenerative disc disease. Animal model of intervertebral disc degeneration is an important path for revealing the pathogenesis of human disc degeneration, and play an important role in testing novel interventions. With recent advances in the relevance of animal models and humans, it has a great prospect in study of human disc degeneration.

  19. Broadband degenerate OPO for mid-infrared frequency comb generation

    National Research Council Canada - National Science Library

    Leindecker, Nick; Marandi, Alireza; Byer, Robert L; Vodopyanov, Konstantin L

    2011-01-01

    .... Our source is based on a degenerate optical parametric oscillator (OPO) which rigorously both down-converts and augments the spectrum of a pump frequency comb provided by a commercial mode-locked near-IR laser...

  20. The degenerate-internal-states approximation for cold collisions

    NARCIS (Netherlands)

    Maan, A.C.; Tiesinga, E.; Stoof, H.T.C.; Verhaar, B.J.

    1990-01-01

    The Degenerate-Internal-States approximation as well as its first-order correction are shown to provide a convenient method for calculating elastic and inelastic collision amplitudes for low temperature atomic scattering.

  1. [Depression in Patients with Age-Related Macular Degeneration].

    Science.gov (United States)

    Narváez, Yamile Reveiz; Gómez-Restrepo, Carlos

    2012-09-01

    Age-related macular degeneration is a cause for disability in the elderly since it greatly affects their quality of life and increases depression likelihood. This article discusses the negative effect depression has on patients with age-related macular degeneration and summarizes the interventions available for decreasing their depression index. Copyright © 2012 Asociación Colombiana de Psiquiatría. Publicado por Elsevier España. All rights reserved.

  2. Degenerated uterine leiomyomas mimicking malignant bilateral ovarian surface epithelial tumors

    Energy Technology Data Exchange (ETDEWEB)

    Hwang, Yi Boem Ha; Lee, Hae Kyung; Lee, Min Hee; Choi, Seo Youn; Chung, Soo Ho [Bucheon Hospital, Soonchunhyang University College of Medicine, Bucheon (Korea, Republic of)

    2017-07-15

    Uterine leiomyomas are the most common benign uterine neoplasms. Undegenerated uterine leiomyomas are easily recognizable by the typical imaging findings on radiologic studies. However, degenerated fibroids can have unusual and variable appearances. The atypical appearances due to degenerative changes may cause confusion in diagnosis of leiomyomas. In this article, we report a case of a patient with extensive cystic and myxoid degeneration of uterine leiomyoma, mimicking malignant bilateral ovarian surface epithelial tumors.

  3. Magnetic resonance imaging of hypertrophic cardiomyopathy. Evaluation of diastolic function; MRT-Bildgebung bei hypertropher Kardiomyopathie (HCM). Evaluation der diastolischen Funktion

    Energy Technology Data Exchange (ETDEWEB)

    Schwarz, F.; Reiser, M.F.; Theisen, D. [Klinikum der Ludwig-Maximilians-Universitaet Muenchen, Campus Grosshadern, Institut fuer Klinische Radiologie, Muenchen (Germany); Deutsches Zentrum fuer Herzkreislaufforschung (DZHK), Muenchen (Germany); Schwab, F. [Klinikum der Ludwig-Maximilians-Universitaet Muenchen, Campus Grosshadern, Institut fuer Klinische Radiologie, Muenchen (Germany); Klinikum der Ludwig-Maximilians-Universitaet Muenchen, Josef Lissner Laboratory for Biomedical Imaging, Institut fuer Klinische Radiologie, Muenchen (Germany); Beckmann, B.M.; Schuessler, F.; Kaeaeb, S. [Klinikum der Ludwig-Maximilians-Universitaet Muenchen, Medizinische Klinik und Poliklinik I, Muenchen (Germany); Zinsser, D.; Goelz, T. [Klinikum der Ludwig-Maximilians-Universitaet Muenchen, Campus Grosshadern, Institut fuer Klinische Radiologie, Muenchen (Germany)

    2013-01-15

    Hypertrophic cardiomyopathy (HCM) has a prevalence of approximately 0.2% and is clinically asymptomatic in many patients or presents with unspecific symptoms. This explains the importance of imaging for the diagnosis of HCM as well as for the assessment of the clinical course. The definitive finding in HCM is myocardial hypertrophy with thickening of the ventricular wall {>=} 15 mm. While echocardiography is an excellent screening tool magnetic resonance imaging (MRI) allows a comprehensive analysis of the heart in HCM. This includes a detailed analysis of the distribution and extent of myocardial hypertrophy, a thorough evaluation of systolic and diastolic cardiac function, the assessment of the presence and extent of dynamic outflow tract obstruction as well as the description of the systolic anterior motion (SAM) phenomenon of the mitral valve with secondary mitral insufficiency. When contrast material is administered, additional information about myocardial perfusion as well as the presence and extent of myocardial fibrosis can be obtained. This study compared systolic functional parameters as well as end systolic and end diastolic wall thickness of patients with and without diastolic dysfunction. (orig.) [German] Die hypertrophe Kardiomyopathie (HCM) hat eine Praevalenz von ca. 0,2% und verlaeuft in vielen Faellen zeitlebens klinisch asymptomatisch. Falls es zur Ausbildung von Symptomen kommt, sind diese oft unspezifisch. Dies erklaert den Stellenwert der Bildgebung bei der Erstdiagnose und Verlaufsbeurteilung der HCM. Leitbefund ist eine myokardiale Hypertrophie mit Wanddicken von {>=} 15 mm. Waehrend die Echokardiographie ein hervorragendes Screeningverfahren ist, erlaubt die MRT eine umfassende Feindiagnostik bei der HCM, zu der gezaehlt werden: eine genaue Darstellung des Verteilungsmusters und des Schweregrads der Hypertrophie, eine detaillierte Analyse der linksventrikulaeren systolischen und diastolischen Funktion, eine Beurteilung und Quantifizierung

  4. Asymptotic coincidence of the statistics for degenerate and non-degenerate correlated real Wishart ensembles

    Science.gov (United States)

    Wirtz, Tim; Kieburg, Mario; Guhr, Thomas

    2017-06-01

    The correlated Wishart model provides the standard benchmark when analyzing time series of any kind. Unfortunately, the real case, which is the most relevant one in applications, poses serious challenges for analytical calculations. Often these challenges are due to square root singularities which cannot be handled using common random matrix techniques. We present a new way to tackle this issue. Using supersymmetry, we carry out an anlaytical study which we support by numerical simulations. For large but finite matrix dimensions, we show that statistical properties of the fully correlated real Wishart model generically approach those of a correlated real Wishart model with doubled matrix dimensions and doubly degenerate empirical eigenvalues. This holds for the local and global spectral statistics. With Monte Carlo simulations we show that this is even approximately true for small matrix dimensions. We explicitly investigate the k-point correlation function as well as the distribution of the largest eigenvalue for which we find a surprisingly compact formula in the doubly degenerate case. Moreover we show that on the local scale the k-point correlation function exhibits the sine and the Airy kernel in the bulk and at the soft edges, respectively. We also address the positions and the fluctuations of the possible outliers in the data.

  5. Incomplete cortical reorganization in macular degeneration.

    Science.gov (United States)

    Liu, Tingting; Cheung, Sing-Hang; Schuchard, Ronald A; Glielmi, Christopher B; Hu, Xiaoping; He, Sheng; Legge, Gordon E

    2010-12-01

    Activity in regions of the visual cortex corresponding to central scotomas in subjects with macular degeneration (MD) is considered evidence for functional reorganization in the brain. Three unresolved issues related to cortical activity in subjects with MD were addressed: Is the cortical response to stimuli presented to the preferred retinal locus (PRL) different from other retinal loci at the same eccentricity? What effect does the role of age of onset and etiology of MD have on cortical responses? How do functional responses in an MD subject's visual cortex vary for task and stimulus conditions? Eight MD subjects-four with age-related onset (AMD) and four with juvenile onset (JMD)-and two age-matched normal vision controls, participated in three testing conditions while undergoing functional magnetic resonance imaging (fMRI). First, subjects viewed a small stimulus presented at the PRL compared with a non-PRL control location to investigate the role of the PRL. Second, they viewed a full-field flickering checkerboard compared with a small stimulus in the original fovea to investigate brain activation with passive viewing. Third, they performed a one-back task with scene images to investigate brain activation with active viewing. A small stimulus at the PRL generated more extensive cortical activation than at a non-PRL location, but neither yielded activation in the foveal cortical projection. Both passive and active viewing of full-field stimuli left a silent zone at the posterior pole of the occipital cortex, implying a lack of complete cortical reorganization. The silent zone was smaller in the task requiring active viewing compared with the task requiring passive viewing, especially in JMD subjects. The PRL for MD subjects has more extensive cortical representation than a retinal region with matched eccentricity. There is evidence for incomplete functional reorganization of early visual cortex in both JMD and AMD. Functional reorganization is more prominent

  6. Frontotemporal Lobar Degeneration and microRNAs

    Directory of Open Access Journals (Sweden)

    Paola ePiscopo

    2016-02-01

    Full Text Available Frontotemporal lobar degeneration (FTLD includes a spectrum of disorders characterized by changes of personality and social behaviour and, often, a gradual and progressive language dysfunction. In the last years, several efforts have been fulfilled in identifying both genetic mutations and pathological proteins associated with FTLD. The molecular bases undergoing the onset and progression of the disease remain still unknown. Recent literature prompts an involvement of RNA metabolism in FTLD, particularly miRNAs. Dysregulation of miRNAs in several disorders, including neurodegenerative diseases, and increasing importance of circulating miRNAs in different pathologies has suggested to implement the study of their possible application as biological markers and new therapeutic targets; moreover, miRNA-based therapy is becoming a powerful tool to deepen the function of a gene, the mechanism of a disease, and validate therapeutic targets. Regarding FTLD, different studies showed that miRNAs are playing an important role. For example, several reports have evaluated miRNA regulation of the progranulin gene suggesting that it is under their control, as described for miR-29b, miR-107 and miR-659. More recently, it has been demonstrated that TMEM106B gene, which protein is elevated in FTLD-TDP brains, is repressed by miR-132/212 cluster; this post-transcriptional mechanism increases intracellular levels of progranulin, affecting its pathways. These findings if confirmed could suggest that these microRNAs have a role as potential targets for some related-FTLD genes. In this review, we focus on the emerging roles of the miRNAs in the pathogenesis of FTLD.

  7. Muscle degeneration in inguinal hernia specimens.

    Science.gov (United States)

    Amato, G; Agrusa, A; Romano, G; Salamone, G; Gulotta, G; Silvestri, F; Bussani, R

    2012-06-01

    There are few articles in the literature reporting the histological changes of groin structures affected by inguinal hernia. A deeper knowledge of this matter could represent an important step forward in the identification of the causes of hernia protrusion. This study aimed to recognise the pathological modifications of muscular structures in autopsy specimens excised from tissues surrounding the hernia orifice. Inguinal hernia was identified in 30 autopsied cadavers, which presented different varieties of hernia, including indirect, direct and mixed. Tissue specimens were resected for histological study from structures of the inguinal area surrounding the hernia opening, following a standardised procedure. The histological examination was focussed on the detection of structural changes in the muscle tissues. The results were compared with biopsy specimens resected from corresponding sites of the inguinal region in a control group of 15 fresh cadavers without hernia. Significant modification of the muscular arrangement of the inguinal area was recognized. Pathological alterations such as atrophy, hyaline and fibrotic degeneration, as well as fatty dystrophy of the myocytes were detected. These findings were observed consistently in the context of multistructural damage also involving vessels and nerves. In cadavers with hernia these alterations were always present independent of hernia type. No comparable damage was found in control cadavers without hernia. The high degree of degenerative changes in the muscle fibres in the inguinal area involved in hernia protrusion described in this report seems to be consistent with chronic compressive damage. These alterations could embody one important factor among the multifactorial sources of hernia genesis. Conjectures concerning its impact on the physiology and biodynamics of the inguinal region are made. The relationship between the depicted degenerative injuries and the genesis of inguinal hernia is also a focus of

  8. Evidence for degenerate tetraploidy in bdelloid rotifers

    Science.gov (United States)

    Mark Welch, David B.; Mark Welch, Jessica L.; Meselson, Matthew

    2008-01-01

    Rotifers of class Bdelloidea have evolved for millions of years apparently without sexual reproduction. We have sequenced 45- to 70-kb regions surrounding the four copies of the hsp82 gene of the bdelloid rotifer Philodina roseola, each of which is on a separate chromosome. The four regions comprise two colinear gene-rich pairs with gene content, order, and orientation conserved within each pair. Only a minority of genes are common to both pairs, also in the same orientation and order, but separated by gene-rich segments present in only one or the other pair. The pattern is consistent with degenerate tetraploidy with numerous segmental deletions, some in one pair of colinear chromosomes and some in the other. Divergence in 1,000-bp windows varies along an alignment of a colinear pair, from zero to as much as 20% in a pattern consistent with gene conversion associated with recombinational repair of DNA double-strand breaks. Although pairs of colinear chromosomes are a characteristic of sexually reproducing diploids and polyploids, a quite different explanation for their presence in bdelloids is suggested by the recent finding that bdelloid rotifers can recover and resume reproduction after suffering hundreds of radiation-induced DNA double-strand breaks per oocyte nucleus. Because bdelloid primary oocytes are in G1 and therefore lack sister chromatids, we propose that bdelloid colinear chromosome pairs are maintained as templates for the repair of DNA double-strand breaks caused by the frequent desiccation and rehydration characteristic of bdelloid habitats. PMID:18362354

  9. Treatment of macular degeneration, according to Bangerter.

    Science.gov (United States)

    Teichmann, K D

    1997-10-30

    Age-related macular degeneration (AMD) is a common cause of visual loss among elderly patients. Although some risk factors have been determined, the ultimate cause of the disease is not known. For a long time, therapeutic nihilism has been the rule among ophthalmologists confronted with such patients. Bangerter has not shared this attitude, especially since the time that he incidentally discovered, more than 40 years ago, the beneficial effects of radiotherapy, in discouraging the growth of new vessels at the posterior pole of the eye. A variety of approaches are combined and used by Bangerter in the treatment of the different types of AMD, including retrobulbar injections of either vasodilating medications (in the dry - or atrophic - type) or corticosteroids (in the wet - or exudative - type), general medical measures aimed at improving metabolic and vascular functions such as supplementation with trace elements, antioxidants, and vitamins; ozone therapy; advice to increase physical fitness, improve nutrition, and abstain from smoking; and protection from excessive light exposure. Being convinced of the usefulness of his type of combination treatment, he has always rejected undertaking controlled clinical trials, of only single aspects of the therapy, as unethical and invalid. For this reason, scientific journals have not proven cooperative in several attempts at publishing his results, as collected in retrospective surveys. Recently, however, some of the several approaches combined by Bangerter in treating AMD have been pronounced effective by other investigators. We present here an overview of his treatment approaches, as few people are aware of them, to clear up misconceptions and to set records straight.

  10. Perceptual learning in patients with macular degeneration

    Directory of Open Access Journals (Sweden)

    Tina ePlank

    2014-10-01

    Full Text Available Patients with age-related macular degeneration (AMD or hereditary macular dystrophies (JMD rely on an efficient use of their peripheral visual field. We trained eight AMD and five JMD patients to perform a texture-discrimination task (TDT at their preferred retinal locus (PRL used for fixation. Six training sessions of approximately one hour duration were conducted over a period of approximately 3 weeks. Before, during and after training twelve patients and twelve age-matched controls (the data from two controls had to be discarded later took part in three functional magnetic resonance imaging (fMRI sessions to assess training-related changes in the BOLD response in early visual cortex. Patients benefited from the training measurements as indexed by significant decrease (p=.001 in the stimulus onset asynchrony (SOA between the presentation of the texture target on background and the visual mask, and in a significant location specific effect of the PRL with respect to hit rate (p=.014. The following trends were observed: (i Improvement in Vernier acuity for an eccentric line-bisection task; (ii positive correlation between the development of BOLD signals in early visual cortex and initial fixation stability (r=0.531; (iii positive correlation between the increase in task performance and initial fixation stability (r=0.730. The first two trends were non-significant, whereas the third trend was significant at p=.014, Bonferroni corrected. Consequently, our exploratory study suggests that training on the TDT can enhance eccentric vision in patients with central vision loss. This enhancement is accompanied by a modest alteration in the BOLD response in early visual cortex.

  11. Clinical efficacy of utilizing Ultrapulse CO2 combined with fractional CO2 laser for the treatment of hypertrophic scars in Asians-A prospective clinical evaluation.

    Science.gov (United States)

    Lei, Ying; Li, Shi Feng; Yu, Yi Ling; Tan, Jun; Gold, Michael H

    2017-06-01

    Hypertrophic scarring is seen regularly. Tissue penetration of laser energy into hypertrophic scars using computer defaults from some lasers may be insufficient and penetration not enough. We have developed a treatment with an interrupted laser "drilling" by the Ultrapulse CO2 (Manual Fractional Technology, MFT) and, a second pass, with fractional CO2 . The MFT with fractional CO2 lasers to treat hypertrophic scars is evaluated. A total of 158 patients with hypertrophic scars had three sessions of MFT with fractional CO2 laser at 3-month intervals. Evaluations made before and 6 months after the 3rd treatment: (1) the Vancouver Scar Scale (VSS), (2) the University of North Carolina (UNC) Scar Scale, and (3) a survey of patient satisfaction. All data were analyzed using a t-test before and after treatment. The VSS score decreased from 9.35 to 3.12 (PCO2 laser had profound effects on the hypertrophic scars treated. It works by increasing the penetration depth of the CO2 laser in the scar tissue, exerting more precise effects on the hypertrophic scars. MFT combined with fractional CO2 laser has the potential to be a major advance in the treatment of hypertrophic scars. © 2017 Wiley Periodicals, Inc.

  12. Hypertrophic chondrocytes in the rabbit growth plate can proliferate and differentiate into osteogenic cells when capillary invasion is interposed by a membrane filter.

    Directory of Open Access Journals (Sweden)

    Tetsuya Enishi

    Full Text Available The fate of hypertrophic chondrocytes during endochondral ossification remains controversial. It has long been thought that the calcified cartilage is invaded by blood vessels and that new bone is deposited on the surface of the eroded cartilage by newly arrived cells. The present study was designed to determine whether hypertrophic chondrocytes were destined to die or could survive to participate in new bone formation. In a rabbit experiment, a membrane filter with a pore size of 1 µm was inserted in the middle of the hypertrophic zone of the distal growth plate of ulna. In 33 of 37 animals, vascular invasion was successfully interposed by the membrane filter. During 8 days, the cartilage growth plate was enlarged, making the thickness 3-fold greater than that of the nonoperated control side. Histological examination demonstrated that the hypertrophic zone was exclusively elongated. At the terminal end of the growth plate, hypertrophic chondrocytes extruded from their territorial matrix into the open cavity on the surface of the membrane filter. The progenies of hypertrophic chondrocytes (PHCs were PCNA positive and caspase-3 negative. In situ hybridization studies demonstrated that PHCs did not express cartilage matrix proteins anymore but expressed bone matrix proteins. Immunohistochemical studies also demonstrated that the new matrix produced by PHCs contained type I collagen, osteonectin, and osteocalcin. Based on these results, we concluded that hypertrophic chondrocytes switched into bone-forming cells after vascular invasion was interposed in the normal growth plate.

  13. Prevalence of exercise-induced left ventricular outflow tract obstruction in symptomatic patients with non-obstructive hypertrophic cardiomyopathy.

    LENUS (Irish Health Repository)

    Shah, J S

    2008-10-01

    Resting left ventricular outflow tract obstruction (LVOTO) occurs in 25% of patients with hypertrophic cardiomyopathy (HCM) and is an important cause of symptoms and disease progression. The prevalence and clinical significance of exercise induced LVOTO in patients with symptomatic non-obstructive HCM is uncertain.

  14. Intralesional triamcinolone acetonide injection in hypertrophic skin surrounding the percutaneous titanium implant of a bone-anchored hearing aid.

    NARCIS (Netherlands)

    Rijswijk, JB Van; Mylanus, E.A.M.

    2008-01-01

    OBJECTIVE: We present a patient with persistent hypertrophic skin surrounding the percutaneous implant of a bone-anchored hearing aid system, successfully treated with intralesional applied corticosteroids. METHOD: Case report and review of the world literature concerning bone-anchored hearing aid

  15. Mortality Risk of Untreated Myosin-Binding Protein C-Related Hypertrophic Cardiomyopathy Insight Into the Natural History

    NARCIS (Netherlands)

    Nannenberg, Eline A.; Michels, Michelle; Christiaans, Imke; Majoor-Krakauer, Danielle; Hoedemaekers, Yvonne M.; van Tintelen, J. Peter; Lombardi, M. Paola; ten Cate, Folkert J.; Schinkel, Arend F. L.; Tijssen, Jan G. P.; van Langen, Irene M.; Wilde, Arthur A. M.; Sijbrands, Eric J. G.

    2011-01-01

    Objectives The goal of this study was to assess the mortality of hypertrophic cardiomyopathy (HCM), partly in times when the disease was not elucidated and patients were untreated. Background HCM is feared for the risk of sudden cardiac death (SCD). Insight in the natural history of the disorder is

  16. Differences in collagen architecture between keloid, hypertrophic scar, normotrophic scar, and normal skin: An objective histopathological analysis

    NARCIS (Netherlands)

    Verhaegen, P.D.; Zuijlen, van P.P.; Pennings, N.M.; Marle, van J.; Niessen, F.B.; Horst, C.; Middelkoop, E.

    2009-01-01

    Normotrophic, hypertrophic, and keloidal scars are different types of scar formation, which all need a different approach in treatment. Therefore, it is important to differentiate between these types of scar, not only clinically but also histopathologically. Differences were explored for collagen

  17. Effects of dredging and lanthanum-modified clay on water quality variables in an enclosure study in a hypertrophic pond

    NARCIS (Netherlands)

    Lürling, Miquel; Waajen, Guido; Engels, Bart; Oosterhout, van Frank

    2017-01-01

    An enclosure experiment was conducted between July and September 2009 to compare the effectiveness of a phosphate fixative, the lanthanum-modified bentonite clay Phoslock® (LMB), dredging, and their combination in controlling eutrophication in a hypertrophic urban pond in Heesch, The Netherlands.

  18. Validation of the HCM Risk-SCD model in patients with hypertrophic cardiomyopathy following alcohol septal ablation

    DEFF Research Database (Denmark)

    Liebregts, Max; Faber, Lothar; Jensen, Morten K

    2018-01-01

    Aims: The HCM Risk-SCD model for prediction of sudden cardiac death (SCD) in hypertrophic cardiomyopathy recommended by the 2014 European Society of Cardiology (ESC) guidelines has not been validated after septal reduction therapy. The aim of this study was to validate the HCM Risk-SCD model...

  19. One third of Danish hypertrophic cardiomyopathy patients with MYH7 mutations have mutations [corrected] in MYH7 rod region

    DEFF Research Database (Denmark)

    Hougs, Lotte; Havndrup, Ole; Bundgaard, Henning

    2005-01-01

    Familial hypertrophic cardiomyopathy (FHC) is, in most cases, a disease of the sarcomere, caused by a mutation in one of 10 known sarcomere disease genes. More than 266 mutations have been identified since 1989. The FHC disease gene first characterized MYH7, encodes the cardiac beta-myosin heavy...

  20. Life-long tailoring of management for patients with hypertrophic cardiomyopathy: Awareness and decision-making in changing scenarios

    NARCIS (Netherlands)

    M. Michels (Michelle); I. Olivotto (Iacopo); F.W. Asselbergs (Folkert); J. van der Velden (Jolanda)

    2017-01-01

    textabstractHypertrophic cardiomyopathy (HCM) is the most common genetic heart disease, characterised by complex pathophysiology and extensive genetic and clinical heterogeneity. In most patients, HCM is caused by mutations in cardiac sarcomere protein genes and inherited as an autosomal dominant