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Sample records for pyloric stenosis hypertrophic

  1. Infantile hypertrophic pyloric stenosis

    DEFF Research Database (Denmark)

    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...

  2. Hypertrophic pyloric stenosis

    DEFF Research Database (Denmark)

    Lund Kofoed, P E; Høst, A; Elle, B

    1988-01-01

    To evaluate the usefulness of ultrasound in hypertrophic pyloric stenosis (HPS) and to analyse the correlation between the dimensions of the pyloric muscle and the age and the weight of the child, 34 children with suspected HPS and 34 controls were examined. An overlap between the dimensions...... of the pyloric muscle in the HPS group and in the controls stresses the need to assess the muscle length, the muscle diameter, and the muscle wall thickness in establishing the sonographic diagnosis of HPS. We found the following criteria useful: muscle length greater than or equal to 19 mm, muscle diameter...... greater than or equal to 10 mm, and muscle wall thickness greater than or equal to 4 mm. The results did not confirm previous reports of increasing dimensions of the pyloric muscle with age and weight....

  3. 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...

  4. Postoperative ad lib feeding for hypertrophic pyloric stenosis.

    Science.gov (United States)

    Carpenter, R O; Schaffer, R L; Maeso, C E; Sasan, F; Nuchtern, J G; Jaksic, T; Harberg, F J; Wesson, D E; Brandt, M L

    1999-06-01

    The aim of this study was to compare three methods of postoperative feeding after pyloromyotomy for hypertrophic pyloric stenosis (HPS). The authors reviewed retrospectively the charts of 308 patients who underwent pyloromyotomy for HPS from 1984 to 1997. Nineteen patients had prolonged hospitalization for other reasons and were excluded from the study, leaving 289 patients for analysis. All procedures were performed by a single group of pediatric surgeons. The individual preferences of these surgeons resulted in three different feeding schedules: R, strictly regimented (>12 hours nothing by mouth, then incremental feeding over > or =24 hours), I, intermediate (>8 hours nothing by mouth, then incremental feeding over lib (lib feedings). Of the 289 patients, 248 (80.5%) were boys. The average age of the patients was 5.64 weeks (range, 1 to 21 weeks). A total of 265 of 289 (92%) were full term. Thirty-nine of 289 (13.5%) had a family history positive for pyloric stenosis. A total of 104 of 289 (36%) were first-born infants, 89 of 289 (31%) were second born. The diagnosis of pyloric stenosis was made by a combination of physical examination findings and diagnostic image for most patients. An "olive" was palpated in 60.6% of the patients. Sixty percent (60.4%) of patients had an upper gastrointestinal series performed, and 42.5% were examined by ultrasonography. Overall, 53% of the patients had postoperative emesis. Only 3.5% had emesis that persisted greater than 48 hours after surgery. Patients fed ad lib after pyloromyotomy had slightly more emesis (2.2 A v. 1.2 R, and 0.7 I episodes, P = .002), but tolerated full feedings sooner than patients fed with a regimented or intermediate schedule. No patient required additional therapy or readmission after tolerating two consecutive full feedings, suggesting that this might be a suitable discharge criterion for most patients with HPS.

  5. Antral web associated with distal antral hypertrophy and prepyloric stenosis mimicking hypertrophic pyloric stenosis

    Institute of Scientific and Technical Information of China (English)

    Mao-Ming Tiao; Sheung-Fat Ko; Chie-Song Hsieh; Shu-Hang Ng; Chi-Di Liang; Shy Ming Sheen-Chen; Jiin-Haur Chuang; Hsuan-Ying Huang

    2005-01-01

    A 3-year-old boy presented with postprandial vomiting and epigastric pain for 3 wk. Barium meal study suggested hypertrophic pyloric stenosis. Ultrasound of the stomach after water loading revealed an echogenic antral web with an eccentric aperture and distal antral hypertrophy.Subsequent endoscopy confirmed the ultrasound findings.Web resection and antropyloroplasty resulted in excellent recovery. To our knowledge, the barium meal and ultrasound findings of an antral web-associated distal antral hypertrophy and prepyloric stenosis has not previously been described.

  6. 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...

  7. First Report of A Unique Presentation of Hypertrophic Pyloric Stenosis Following Type I Esophageal Atresia; A Case Report

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    Amrollah Salimi

    2017-06-01

    Full Text Available Combination of congenital esophageal atresia and subsequent hypertrophic pyloric stenosis is a rare condition which occurs in early infancy. The underlying etiology and pathophysiology of this association still remains unclear. In this paper we report a unique case of hypertrophic pyloric stenosis, for the first time, which occurred in an infant who underwent surgery for type I esophageal atresia. Therefore, we intend to highlight the role of poor nutrition intake in the incidence of this condition.

  8. [Population prevalence of congenital hypertrophic pyloric stenosis in the Donetsk region of Ukraine].

    Science.gov (United States)

    Mukhin, V N; Moskalenko, V Z; Grona, V N; Sopov, G A; Linchevskiĭ, G L

    2001-01-01

    The population prevalence of congenital hypertrophic pyloric stenosis in Donetsk region in the course of 11 years (1989-1999) has been descriptively studied. Its frequency is 0.73:1000 or 1:1370 newborn infants annual with range of 0.54 to 1.01:1000. It is somewhat lower than that of more wide spread congenital diseases: labial cleft and hemochromatoses. Pyloric stenosis is detected in 42 of 43 populations of Donetsk region. There is a population nonuniformity of the case rate: from 0.19 to 1.9:1000 newborn infants, that the authors are inclined to explain by dissimilarity of a gene pool in the populations due to random migration of the population.

  9. Contemporary management of pyloric stenosis.

    Science.gov (United States)

    Jobson, Matthew; Hall, Nigel J

    2016-08-01

    Hypertrophic pyloric stenosis is a common surgical cause of vomiting in infants. Following appropriate fluid resuscitation, the mainstay of treatment is pyloromyotomy. This article reviews the aetiology and pathophysiology of hypertrophic pyloric stenosis, its clinical presentation, the role of imaging, the preoperative and postoperative management, current surgical approaches and non-surgical treatment options. Contemporary postoperative feeding regimens, outcomes and complications are also discussed. Copyright © 2016 Elsevier Inc. All rights reserved.

  10. [Ultrasound-guided paravertebral block for pyloromyotomy in 3 neonates with congenital hypertrophic pyloric stenosis].

    Science.gov (United States)

    Mata-Gómez, Javier; Guerrero-Domínguez, Rosana; García-Santigosa, Marta; Ontanilla, Antonio

    2015-01-01

    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. 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. 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. Copyright © 2014 Sociedade Brasileira de Anestesiologia. Publicado por Elsevier Editora Ltda. All rights reserved.

  11. Ultrasound-guided paravertebral block for pyloromyotomy in 3 neonates with congenital hypertrophic pyloric stenosis.

    Science.gov (United States)

    Mata-Gómez, Javier; Guerrero-Domínguez, Rosana; García-Santigosa, Marta; Ontanilla, Antonio

    2015-01-01

    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. 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. 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. Copyright © 2014 Sociedade Brasileira de Anestesiologia. Published by Elsevier Editora Ltda. All rights reserved.

  12. Ultrasound-guided paravertebral block for pyloromyotomy in 3 neonates with congenital hypertrophic pyloric stenosis

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    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.

  13. Peculiarities of surgical treatment of acute form of congenital hypertrophic pyloric stenosis

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    Олег Владимирович Спахи

    2015-07-01

    Full Text Available Aim of research: to study an efficiency of the double pyloromyotomy at congenital pyloric stenosis in children and its impact on regress of hypertrophy of pylorus in postoperative period. Material and methods of research: there was carried out an analysis of 157 patients with an acute form of congenital hypertrophic pyloric stenosis (CHPS that characterizes with a sudden beginning of disease, predominance of “fountain” vomiting over regurgitation, fast growth of homeostasis dysfunctions and decrease of the body weight. All children were separated in 2 groups statistically homogenous on sex, age and prescription of disease.  The first group of examination (children who underwent the double pyloromyotomy included 65 boys. The second (control group included 92 male children who underwent the standard pyloromyotomy according to Frede-Weber-Ramstedt.  The ultrasound examination of pylorus in dynamics with detection of its volume was carried out in both groups.Results. The results of intraoperative assessment of the state of injured pylorus indicate an existence of two macroscopic variants of it: “rigid pyloric olive” that was registered in 82 % of children of the first group and the “soft pyloric olive” – in 79% of patients of the control group. “Rigid olive” characterized with hard “cartilaginoid” consistency of fusiform thickened pylorus of the whitish color. “Soft olive” had the dense elastic consistency and the pink-pale color.   The regress of “pyloric olive” in all children took place on average during 67,92,2 (Mm days. But in children of the second group medium terms of involution of hypertrophy of pylorus were 75,74,1 (Mm days. In patients who underwent the double pyloromyotomy these terms were reliably (р0,05 lower and varied within 41,71,5 (Mm days.Conclusions. Involution of hypertrophy of pyloric end after the surgical treatment of CHPS occurs gradually and depends on adequacy of regeneration of the

  14. Sonographic Layers in Infantile Hypertrophic Pyloric Stenosis seen on High Resolution Sonography

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    Kim, Dae Soon; Yoo, Shi Joo; Yoo, Yong Ho; Kim, In Koo; Kim, Kyung Mo; Choe, Ghee Young [Asan Medical Center, University of Ulsan College of Medicine, Seoul (Korea, Republic of)

    1996-12-15

    Sonography is a conclusive method in the diagnosis of infantile hypertrophic pyloric stenosis(IHPS). However, previous reports incompletely described the sonographic layers. This study was performed to define the layers seen on high resolution sonography. We performed high resolution sonography with 10-5 MHz linear transducer in 8 patients with IHPS. The layers seen on sonography were correlated with histopathologic findings by obtaining thin section of thickened gastric wall during surgery. All cases showed typical sonographic findings that were previously described. The thickened pyloric wall consisted of 5 sonographic layers : extremely echogenic mucosal interface, hypoechoic mucosa, hyperechoic submucosa, mixed echogenic circular muscle layer, and hypoechoic longitudinal muscle layer. On longitudinal scan of pylorus,circular muscle fiber directions were identified as alternating bands of hyper-and hypoechogenicity with the appearance of zebra skin. Clear delineation of the layers of gastric wall and the muscle fiber directions of hypertrophied circular muscle in IHPS showed by high resolution sonography. Our results are contradictory to the previous reports that defined the sonolucent double tracks as collapsed lumen and the echogenic lines as mucosa. We believe that the multiple hypoechogenic bands seen in longitudinal scan represent the folded mucosal layers and that the echogenic lines are from the mucosal interface

  15. Estenosis hipertrófica del píloro Hypertrophic pyloric stenosis

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    Ronald Armando Noguera-Valverde

    2009-09-01

    Full Text Available La estenosis hipertrófica del píloro es la causa más frecuente de cirugía en los lactantes menores de 6 meses, superada solamente por las hernioplastias. La causa exacta de la estenosis del píloro no se ha determinado todavía, pero se han desarrollado algunas teorías relacionadas con un desequilibrio neurohormonal, o de mediadores neuroendocrinos, en el control del tono del esfínter pilórico, en la producción de gastrina y la motilidad del estómago. Aunque en la actualidad su tratamiento final es quirúrgico, algunos investigadores están retomando el abordaje farmacológico para el tratamiento de este problema y otros están optando por el abordaje laparoscópico. El diagnóstico clínico está todavía en las manos del médico de atención primaria y del pediatra, quienes reciben en primera instancia a los pacientes. Se deben tener en cuenta los diagnósticos diferenciales y el abordaje inicial para la estabilización del paciente.Hypertrophic pyloric stenosis is the more frequent cause of surgery in infants under 6 months only surmountable by the hernioplasties. Still the exact cause of stenosis has been not determined, but some theories have been developed related to neurohormonal unbalance or from neuroendocrine mediators in control of pyloric sphincter tone, in gastrine production, and stomach motility. Although at present time its final treatment is surgical, some researchers are reconsidering the pharmacologic approach for treatment of this problem, and other choosing by laparoscopic approach. Clinical diagnosis is still a primary care physician criterion or the pediatrician who as a first resort receive the patients. Differential diagnoses and initial approach for patient stabilization, must to be into account.

  16. Role of bottle feeding in the etiology of hypertrophic pyloric stenosis.

    Science.gov (United States)

    McAteer, Jarod P; Ledbetter, Daniel J; Goldin, Adam B

    2013-12-01

    IMPORTANCE Bottle feeding has been implicated in the etiology of hypertrophic pyloric stenosis (HPS). Further data are needed to define the nature of this relationship and the clinical variables that influence it. OBJECTIVE To determine if bottle feeding after birth is associated with the development of HPS in infants. We hypothesized that bottle feeding is associated with an increased risk of HPS and that this risk is modified by other risk factors. DESIGN, SETTING, AND PARTICIPANTS Population-based case-control study of births from January 1, 2003, to December 31, 2009, using Washington State birth certificates linked to hospital discharge data. Cases included all singleton infants born within the study period and subsequently admitted with both a diagnostic code for HPS and a procedure code for pyloromyotomy (n = 714). Controls were randomly chosen among singleton infants who did not develop HPS and were frequency matched to cases by birth year. EXPOSURE Feeding status (breast vs bottle) was coded on the birth certificate as the type of feeding the infant was receiving at birth discharge. MAIN OUTCOME AND MEASURE Diagnosis of HPS. RESULTS Hypertrophic pyloric stenosis incidence decreased over time, from 14 per 10,000 births in 2003 to 9 per 10,000 in 2009. Simultaneously, breastfeeding prevalence increased from 80% in 2003 to 94% in 2009. Compared with controls, cases were more likely to be bottle feeding after birth (19.5% vs 9.1%). After adjustment, bottle feeding was associated with an increased risk of HPS (odds ratio [OR], 2.31; 95% CI, 1.81-2.95). This association did not differ according to sex or maternal smoking status but was significantly modified by maternal age (Bottle feeding is associated with an increased risk of HPS, and this effect seems to be most important in older and multiparous women. These data suggest that bottle feeding may play a role in HPS etiology, and further investigations may help to elucidate the mechanisms underlying the

  17. Changing trends in the management of infantile hypertrophic pyloric stenosis--an audit over 11 years.

    LENUS (Irish Health Repository)

    Doyle, D

    2012-02-03

    BACKGROUND: This article is a follow-up to an audit performed by the Department of Surgery and published in the Irish Journal of Medical Science in 1996. This audit reviewed all cases of Infantile Hypertrophic Pyloric Stenosis (IHPS) operated on over 22 years up to 1991. AIMS: We aim to demonstrate that radiologic investigations, namely barium meal and ultrasound, have been increasingly employed in the diagnosis of IHPS. In addition, ultrasound is now the investigation of choice. METHODS: We have reviewed all cases of IHPS, at the same institution, over the subsequent 11 years, with reference to any radiological investigations performed. In the previous study, the diagnosis of IHPS was made clinically in 92.6% with the remainder diagnosed radiologically. RESULTS: Over 11 years, 157 patients were diagnosed with IHPS. Male to female ratio was 4.06:1. Median age was four weeks (range 1-18 weeks).Twenty-four per cent had a barium meal, 36% had an ultrasound and 13% had both performed. CONCLUSION: We conclude a change in practice in the management of IHPS with radiology, particularly ultrasound, playing an increasing role.

  18. The clinical features of infantile hypertrophic pyloric stenosis in Chinese Han population: analysis from 1998 to 2010.

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    Zhiqiang Feng

    Full Text Available OBJECTIVE: To investigate clinical features of infantile hypertrophic pyloric stenosis (IHPS in Chinese Han population. METHODS: Three hundred and sixteen hospitalized patients with IHPS from January 1998 to February 2010 were retrospectively reviewed, and data including patient's sex, onset age, other coexisting congenital defects, pyloric circular muscle thickness evaluated by ultrasonograph, serum electrolytes concentration, and results of arterial blood gas analysis on admission were collected. The patients were divided into two groups: the duration between first onset and admission less than or equal to 10 days (early onset group, and more than 10 days (late onset group. The results of arterial blood gas and serum electrolyte concentration were compared between the two groups. RESULTS: There were 271 males and 45 females in 316 patients; the onset age ranged between 1 and 351 (26.5±26.6 days. The birth weight ranged between 1.6 and 4.5 (3.23±0.44 kilograms; coexisting congenital defects were found in 65 cases (20.6%. Pyloric circular muscle thickness was 4-8 (5.4±1.0 millimetres (mm. For the early onset group, the rates of hypokalemia, hypochloraemia and hypercapnia were significantly lower than those in the late onset group (18.67% VS 50%, P<0.0001; 46.03% VS 71.01%, P = 0.003; 56.58% VS 83.44%, P = 2.17×10(-5; respectively. CONCLUSIONS: The symptom duration in Chinese Han population was longer than that in other populations. And as the prolongation of symptom duration, the incidence of acid-base imbalance increased significantly. Infants with persistent vomiting at the age of 3∼5 weeks after birth should be considered IHPS, and go to hospital as soon as possible in order to reduce the incidence of hypokalemia, hypochloraemia and hypercapnia, and avoid deterioration.

  19. Adult hypertrophic pyloric stenosis due to peptic ulcer disease: a rare presentation

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    Shameer Deen

    2016-05-01

    Full Text Available Primary adult hypertrophic stenosis is uncommon with an uncertain etiopathogenesis and associated gastric outlet obstruction mimics gastric carcinoma. We present a case of AHPS as sequel of peptic ulcer disease in a 72 year old male. With the advent of proton pump inhibitors as a mainstay of medical therapy, complication into gastric outlet obstruction is a rare disease today. Upper GI endoscopy revealed a distended stomach, residual food and a hyperemic bulky pylorus not accommodating the endoscope. Barium meal follow-through revealed a dilated stomach and minimal barium passing through the pylorus. Histological analysis revealed mild dysplasia at the focus with dense inflammatory infiltrates composed of lymphocytes and eosinophils in the lamina propria. No evidence of malignancy was noted, favouring chronic gastritis. The condition mimics other forms of proliferative disorders like carcinoma, gastrointestinal stromal tumors. We present the clinical findings, imaging analysis and discuss etiopathogenesis and management. [Int J Res Med Sci 2016; 4(5.000: 1730-1732

  20. B型超声波诊断先天性肥厚性幽门狭窄%Diagnosis of Congenital Hypertrophic Pyloric Stenosis by Real Time Sonography

    Institute of Scientific and Technical Information of China (English)

    陈博渊; 韩湘珍; 唐伟椿; 孙建芳; 孟晓敏

    1988-01-01

    本文报道B超诊断先天性肥厚性幽门狭窄的方法,其影像特点为:胃内容呈涡流和滞留现象,肥厚幽门为椭园形暗区,大小为≥19×13mm,幽门壁厚5mm.作者认为B超优于钡餐X线检查,可以作为新生儿吐奶的筛选检查方法.%Real time sonography was performed for identification of congenital hypertrophic pyloric stenosis(CHPS)in 21 patients(19 males and 2 females)from June 1985 to July 1987.Their diagnosis was proved at operation in all cases.50-100 ml of sugar water was fed during sonographic examination.The characteristic image of CHPS includes:1.gastric reflux from the antrum backward and the possible simultaneous clinical vomiting;2.stasis of gastric content lasting over 30 minutes;3.hypertrophic pyloric mass which can be seen and measured about 19×13 mm;4.a thin thread-like shaddow passing through the center of the pylorus demonstrating its hypertrophied muscular wall which can be seen and measured around 5 mm in thickness.It is also discussed in this article that real time sonography can be utilized to differentiate stomach volvulus,pyloric web,pyloric spasm,and other functional vomiting.

  1. Idiopathic Hypertrophic Pyloric Stenosis in an Adult, a Potential Mimic of Gastric Carcinoma

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    Alireza Zarineh

    2010-01-01

    Full Text Available Primary or idiopathic hypertrophy of the pyloric muscle (IHPM is a rare entity with uncertain pathogenesis which both clinically and pathologically mimics gastric cancer. We present a rare late-occurring case of IHPM in a 71-year-old Caucasian man with no apparent predisposing factor. Imaging studies demonstrated gastric distension with air fluid levels and no evidence of extrinsic compression. At upper endoscopy, massive gastric distension and no evidence of any ulcer or other mucosal defects were observed. Microscopically, marked hypertrophy of muscularis mucosa with smooth muscle cells arranged in whorls and fascicles was present which gradually transitioned to normal areas. The muscle fibers stained with smooth muscle actin and trichrome stain highlighted fibrosis between the muscle fibers. Although uncommon, IHPM can clinically and histologically mimic other proliferations in the gastric wall, such as gastrointestinal stromal tumor or a spindle cell neoplasm. The recent advances in understanding the pathogenesis of IHPM are discussed.

  2. 肥厚性幽门狭窄肽能神经的免疫组化研究%Immunohistochemical Study of Peptidergic Narve in Infantile Hypertrophic Pyloric Stenosis

    Institute of Scientific and Technical Information of China (English)

    沈振新; 佘亚雄; 王玲华; 王万超

    1989-01-01

    It is gradually noted that peptidergic nerves may play an important role in infantile hypertrophic pyloric stenosis.The specimens of pyloric muscles from 20 patients with infantile hypertrophic pyloric (sterosis) were examined by immunohistochemistry in regard to the distribution of ganglion cells and fibers containing enkephalin (ENK),substance P (SP) and vasoactive intestinal polypeptide (VIP) in muscles and myenteric plexus.The results showed that three subpopulations of peptide-containing nerve cells and fibers in the circular muscles of control group were remarkable in number,but in patients with pylorie stenosis the number of the nerve fibers was markedly reduced and even missed,especially ENK and VIP fibers.In the myenteric plexus there was no such a reduction of the nerve cells or fibers in either patients or controls.This finding suggests that the abnormality of peptida-containing nerve fibers or an impaired neuronal funetion might result in hypertrophic pyloric stenosis.The mechanism of HPS is discussed briefly in the article.%本文应用免疫组织化学技术,对20例幽门狭窄患儿的脑啡肽、P物质和血管活性肠肽三种肽能神经在幽门组织中的数量和分布进行了观察.发现三种肽能神经纤维在患儿环肌层中有改变,提示肽能神经数量和分布异常与发病有关.本文亦讨论了本病的发病机理.

  3. Familial aggregation and heritability of pyloric stenosis

    DEFF Research Database (Denmark)

    Krogh, Camilla; Fischer, Thea K; Skotte, Line;

    2010-01-01

    .1 for twins. The rate ratios of pyloric stenosis were 182 (95% confidence interval [CI], 70.7-467) for monozygotic twins, 29.4 (95% CI, 9.45-91.5) for dizygotic twins, 18.5 (95% CI, 13.7-25.1) for siblings, 4.99 (95% CI, 2.59-9.65) for half-siblings, 3.06 (95% CI, 2.10-4.44) for cousins, and 1.60 (95% CI, 0.......51-4.99) for half-cousins. We found no difference in rate ratios for maternal and paternal relatives of children with pyloric stenosis and no difference according to sex of cohort member or sex of relative. The heritability of pyloric stenosis was 87%. CONCLUSION: Pyloric stenosis in Danish children shows strong...

  4. Epidemiological features of infantile hypertrophic pyloric stenosis in Taiwanese children: a Nation-Wide Analysis of Cases during 1997-2007.

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    Mee-Mee Leong

    Full Text Available OBJECTIVE: To describe the epidemiological characteristics of infantile hypertrophic pyloric stenosis (IHPS in ethnic Chinese children. MATERIALS AND METHODS: We reviewed the National Health Insurance claims database and analyzed data from children less than one year of age who had been diagnosed with IHPS (ICD-9-CM 750.5 and had undergone pyloromyotomy (ICD-9-CM 43.3. We analyzed the incidence, gender, age at diagnosis, length of hospital stay, seasonal variation and cost of IHPS from data collected between January 1997 and December 2007. RESULTS: A total of 1,077 infants met inclusion criteria, including 889 boys and 188 girls. The annual incidence of IHPS ranged from 0.30 to 0.47 per 1,000 live births with a mean incidence of 0.39 per 1,000 live births. Between 2002 and 2007, the incidence showed a declining trend (P = 0.025 with coincidentally increasing trends for both exclusive breastfeeding (P = 0.014 and breastfeeding plus bottle feeding (P = 0.004. The male-to-female rate ratio was dynamic and increased from 3.03 during the first two weeks of life to 8.94 during the 8(th through 10th weeks of life. The overall male-to-female rate ratio was 4.30. The mean age at diagnosis was 43.1 ± 2.4 days. After analyzing the months of birth and hospital admission, no seasonal variation associated with IHPS was detected. The mean length of hospital stay was 8.28 ± 7.10 days. CONCLUSIONS: The incidence of IHPS in Taiwan, a country with a majority ethnic Chinese population, was lower than observed incidences in Caucasian populations living in Western countries. Breastfeeding campaigns and low maternal smoking rates may contribute to the lower incidence of IHPS in Taiwan. However, additional studies with longer follow-up periods are needed.

  5. 婴幼儿呕吐及先天性肥厚性幽门狭窄诊断分析%Analysis of diagnosis of infantile vomiting and congenital hypertrophic pyloric stenosis

    Institute of Scientific and Technical Information of China (English)

    王丽静; 赵海; 赵宏然; 宫术娟

    2015-01-01

    Objective:To analyze the causes of infant vomiting, congenital hypertrophic pyloric stenosis diagnosis. Methods:analyzed the clinical data of 2009. 1-2014. 2 in our hospital 185 cases of vomiting in infants, the retrospective analysis of diagnosis of congenital hypertrophic pyloric stenosis in children, and summarize the diagnosis anddifferential diagnosis. Results:among the 149 cases of vomiting caused bygastrointestinal and respiratory diseases;18 cases of intussusception;congenital hypertrophic pyloric stenosis in 12 cases;in oth-er 6 cases. Conclusion:The causes of infant vomiting is complicated, through the analysis of disease by imaging examination, could reduce or avoid misdiagnosis, missed diagnosis.%目的::分析婴幼儿呕吐原因,探讨先天性肥厚性幽门狭窄诊断要点。方法:分析2009年1月~2014年2月在我院就诊185例呕吐婴幼儿的临床资料,对确诊先天性肥厚性幽门狭窄的患儿回顾性分析,总结其诊断及鉴别诊断要点。结果:其中胃肠炎及呼吸道疾病所致呕吐149例;肠套叠18例;先天性肥厚性幽门狭窄12例;其它6例。结论:婴幼儿呕吐原因繁杂,通过分析病情借助影像学检查,可以降低或避免误诊、漏诊。

  6. Case reports 1964, medicine: infantile hypertrophic pyloric stenosis in four siblings. Retinopathy and keratopathy due to chloroquine. The first instance of hemoglobin E in a Japanese family

    Energy Technology Data Exchange (ETDEWEB)

    Burmeister, R.E.; Hamilton, H.B.; Hinds, M.J.A.; Slavin, R.E.; Kamata, Nanao; Shibata, Susumu; Miller, R.J.; Phair, J.P.; Kasahara, Masayuki; Shibata, Susumu

    1964-06-18

    This document contains 3 reports. In the first report four siblings are presented who had infantile pyloric stenosis unequivocally demonstrated when pyloromyotomy was performed in the early neonatal period. Their father had symptoms of stenosis as an infant but he was treated medically and it cannot be stated with certainty that he had the disease. Blood groups, determined for the four children and their parents, were not unusual. Chromosome karyotypes, obtained from peripheral blood cultures, were apparently normal. In the second report, a case study of a patient exhibiting side effects due to chloroquine used in the treatment of lupus vulgaris is presented. In the third report, in a survey for hemoglobinopathies in Nagasaki, Japan four members in two generations of a Japanese family were found to have an abnormal hemoglobin, which on detailed chemical analyses was demonstrated to be hemoglobin E. The question of prior introduction of the gene into Japan from Southeast Asia versus independent mutation is briefly discussed. 70 references, 4 figures, 5 tables.

  7. Negative exploration for pyloric stenosis – Is it preventable?

    Directory of Open Access Journals (Sweden)

    Kenny Simon E

    2008-09-01

    Full Text Available Abstract Background The diagnosis of infantile hypertrophic pyloric stenosis (IHPS, although traditionally clinical, is now increasingly dependent on radiological corroboration. The rate of negative exploration in IHPS has been reported as 4%. The purpose of our study was to look at elements of supportive clinical evidence leading to positive diagnosis, and to review these with respect to misdiagnosed cases undergoing negative exploration. Methods All infants undergoing surgical exploration for IHPS between January 2000 and December 2004 were retrospectively analysed with regard to clinical symptoms, examination findings, investigations and operative findings. Results During the study period, 343 explorations were performed with a presumptive diagnosis of IHPS. Of these, 205 infants (60% had a positive test feed, 269 (78% had a positive ultrasound scan and 175 (55% were alkalotic (pH ≥7.45 and/or base excess ≥2.5. The positive predictive value for an ultrasound (US diagnosis was 99.1% for canal length ≥14 mm, and 98.7% for muscle thickness ≥4 mm. Four infants (1.1% underwent a negative surgical exploration; Ultrasound was positive in 3, and negative in 1(who underwent surgery on the basis of a positive upper GI contrast. One US reported as positive had a muscle thickness Conclusion A 1% rate of negative exploration in IHPS compares favourably with other studies. However potential causes of error were identified in all 4 cases. Confident diagnosis comprises a combination of positive test feed and an 'in house US' in an alkalotic infant. UGI contrast study should not be used in isolation to diagnose IHPS. If the test feed is negative, strict diagnostic measurements should be observed on US and the pyloric 'tumour' palpated on table under anaesthetic before exploration.

  8. Early pyloric stenosis: a case control study.

    Science.gov (United States)

    Demian, Marie; Nguyen, Son; Emil, Sherif

    2009-12-01

    Pyloric stenosis (PS) is rare in the first 2 weeks of life, often leading to delays in diagnosis and treatment. We conducted a case control study to delineate the characteristics of patients with early PS (EPS). In addition, we tested the hypothesis that patients with EPS present with a smaller pylorus than older patients. A database of all patients presenting with PS to a children's hospital over a 5-year period (2002-2006) was obtained. Each patient admitted during the first 2 weeks of life (subject) was matched to a patient admitted after 4 weeks of age (control), with the same gender, electrolyte status, and treating surgeon. A single pediatric radiologist, blinded to patient age, reviewed all available ultrasounds retrospectively. Demographic, clinical, diagnostic, therapeutic, and outcome data were compared. During the study period, 278 pyloromyotomies were performed for PS. Sixteen patients (5.8%) presented with EPS between 2 and 14 days of life. EPS patients had a higher prevalence of positive family history (31 vs. 0%, P = 0.043), and breast milk feeding (75 vs. 31%, P = 0.045). Sonographic measurements showed a pylorus that was of significantly less length (17.1 +/- 0.6 vs. 20.5 +/- 0.9 mm, P = 0.006) and muscle thickness (3.5 +/- 0.2 vs. 4.9 +/- 0.2 mm, P < 0.001) in patients with EPS. Hospital stay was significantly longer for EPS patients (4.3 +/- 0.9 vs. 2.0 +/- 0.1 days, P = 0.19). Babies presenting with EPS are more likely to be breast fed and to have a positive family history. EPS is associated with a longer hospital stay. Use of sonographic diagnostic measurements specific to this age group may prevent delays in diagnosis and treatment, and improve outcomes.

  9. Bottle-feeding and the Risk of Pyloric Stenosis

    DEFF Research Database (Denmark)

    Krogh, Camilla; Biggar, Robert J; Fischer, Thea Kølsen

    2012-01-01

    Bottle-feeding has been suggested to increase the risk of pyloric stenosis (PS). However, large population-based studies are needed. We examined the effect of bottle-feeding during the first 4 months after birth, by using detailed data about the timing of first exposure to bottle...

  10. [Pyloric stenosis complicated by Wernicke-Korsakoff syndrome].

    Science.gov (United States)

    Bataller, R; Salmerón, J M; Muñoz, J E; Obach, V; Elizalde, J I; Mas, A; Tolosa, E; Terés, J

    1997-03-01

    The Wernicke-Korsakoff syndrome (WKS) is a picture of oculomotor alterations, ataxia and confusion presented in chronic alcoholics. It has more rarely been described in non alcoholic patients with malnutrition. The case of a patient with ulcerous peptic disease of long evolution who consulted for a picture compatible with WKS following clinical manifestations of repeated vomiting secondary to complete pyloric stenosis is presented. The peculiarity of the picture and the convenience of prevention in malnourished patients receiving intravenous glucose sera is discussed.

  11. A novel mutation of WT1 exon 9 in a patient with Denys-Drash syndrome and pyloric stenosis.

    Science.gov (United States)

    Hu, Min; Craig, Jonathon; Howard, Neville; Kan, Alex; Chaitow, Jeffrey; Little, Dianne; Alexander, Stephen I

    2004-10-01

    We report a novel mutation in WT1 exon 9 (1214 A>G) resulting in an amino acid change from H to R at codon 405 in a 46 XY female patient who had congenital hypertrophic pyloric stenosis, pseudohermaphroditism masculinus, renal failure, and Wilms tumor, and died at the age of 22 months. The patient demonstrated the difficulty in diagnosing a patient with intersex before conclusive genetic characterization.

  12. Estenose de piloro em eqüino adulto Pyloric stenosis in a mature horse

    Directory of Open Access Journals (Sweden)

    Ana Lúcia Miluzzi Yamada

    2009-02-01

    Full Text Available Neste trabalho, é descrito um caso de estenose hipertrófica de piloro diagnosticado por gastroscopia em um eqüino Quarto de Milha, de quatro anos de idade, que apresentava inapetência, emagrecimento progressivo, cólica, bruxismo e sialorréia. A gastroduodenite ulcerativa crônica e os espasmos prolongados foram as supostas causas da hipertrofia da musculatura circular do piloro, causando a estenose. O tratamento com substâncias antiulcerativas é paliativo e o acesso cirúrgico ao piloro no animal adulto é complexo, sendo o prognóstico duvidoso.A case of hypertrophic pyloric stenosis diagnosed by gastroscopy was reported in a 4-years-old Quarter Horse gelding, with signs of inappetence, chronic weight loss, colic, bruxism and sialorrhea. Gastroduodenal ulceration and spasms were supposed as causes of pyloric muscular hypertrophy and stenosis. Antiulcerative medications are merely palliative and the complex surgical approach to the site of stenosis makes the prognostic to be doubtful.

  13. Hypocalcemia and Hypomagnesemia in Perioperative Period of Congenital Hypertrophic Pyloric Stenosis%先天性肥厚性幽门狭窄围手术期低钙低镁血症

    Institute of Scientific and Technical Information of China (English)

    傅红茹; 连贵新; 李新房

    1994-01-01

    32例肥厚性幽门狭窄患儿,因长期大量呕吐、摄入不足及吸收障碍等原因,均有不同程度的低钙、低镁血症,其中低钙24例,低镁18例,钙、镁均低者16例.临床表现为易惊、睡眠不安、夜哭、四肢震颤、头向后仰、背肌紧张、面部抽搐,严重者出现喉痉挛.经使用钙、镁制剂后得到控制.%Because of persistent vomiting,inadequate intake and malabsorption,32 cases of pyloric stenosis had vari ous degrees of hypocalcemia and hypomagnesemia.Of them,hypocalcemia was in 24,hypomagnesemia was in 18,and hypocalcemia and hypomagnesemia were in 16.The presentation was to be frightened easily,dyssomnia,night crying,tremor,hypsokinesis and facial of glottic spasm.With the application of pharmaceutical calcium and magnesium,the symptom has been under control.

  14. Pyloric stenosis associated Crohn’s disease responding to adalimumab therapy

    Institute of Scientific and Technical Information of China (English)

    Sameer; Gaggar; John; Scott; Nicholas; Thompson

    2012-01-01

    Gastroduodenal Crohn’s disease (CD) is rare and the response to standard medical therapy is often poor. Anti-tumor necrosis factor therapy has revolutionised the treatment of CD. We present a patient with pyloric stenosis associated with CD which improved with Adalimumab therapy. We recommend considering antitumor necrosis factor therapy in symptomatic gastroduodenal CD.

  15. 奥美拉唑快速纠正先天性肥厚性幽门狭窄代谢性碱中毒的临床研究%The clinical study of omeprazole in rapid correction of metabolic alkalosis in congenital hypertrophic pyloric stenosis

    Institute of Scientific and Technical Information of China (English)

    孙邡; 刘斌; 张宏伟; 禚保彪; 刘丰丽; 方允

    2014-01-01

    Objetive To explore the function of omeprazole in rapid correction of metabolic alkalosis in congenital hypertrophic pyloric stenosis(CHPS). Methods 80 infants with CHPS were randomly divided into two groups,treatment group 40 cases,control group 40 cases.According to the pH on admission ,those patients were divided into pH>7.5 group (treatment group 21 cases,control group 22 cases),pH≤7.5 group (treat-ment group19 cases,control group18 cases ).The control group was given conventional normal saline,equilibri-um liquid to correct electrolyte and acid-base balance disorders,the treatment group in addition to routine thera-py was given omeprazole 0.7 mg/(kg.d)qd intravenous injection.The arterial blood gas analysis were detec-ted in every 12 h after admission,The SPSS13.0 software of statistics analyzed the primary data. Results pH>7.5 group:12 h after admission treatment group with 8 cases (8/21,38.1%)pH returned to normal,while control group only with 2 cases (2/22,9.1%),(P0.05).Conclusion Intravenous omeprazole administration can rapidly normalize severe meta-bolic alkalosis in CHPS patients,particularly for moderately severe metabolic alkalosis.As a result,pyloromyoto-my can be performed sooner reducing both hospital stay and costs.%目的:探讨奥美拉唑在快速纠正先天性肥厚性幽门狭窄代谢性碱中毒中的作用。方法将本院80例诊断为先天性肥厚性幽门狭窄的患儿随机分成两组,治疗组40例,对照组40例。根据入院时pH值又分为pH>7.5组(治疗组21例,对照组22),pH≤7.5组(治疗组19例,对照组18例)。对照组采用补充生理盐水、平衡液等纠正电解质紊乱及酸碱失衡,治疗组除采用上述治疗外加用奥美拉唑0.7 mg·kg-1·d-1静脉滴注,每日1次。入院后每12 h行动脉血气分析,数据通过SPSS13.0软件包进行统计学处理。结果 pH>7.5组:入院后12h治疗组有8例(8/21,38.1%)pH

  16. A prospective study of comparison between Open Gastrojejunostomy and Laparoscopic Assisted Gastrojejunostomy in patients of post corrosive ingestion pyloric stenosis.

    Directory of Open Access Journals (Sweden)

    Samir shah

    2016-03-01

    Results: We observed that benign etiology was more common for GOO (58% compared to malignant cause (42% and post corrosive ingestion pyloric stenosis was most common benign cause(42% of GOO, Pancreatic cancer was most common malignant cause(18.5% of GOO. Corrosive ingestion was more common in younger age group (66% in 15 -30 yr age and female gender(63.34% and mostly as a suicidal attempt(86.66% and most common corrosive agent was sanitary cleansing agent(hydrochloric acid (70%.Postprandial nonbillious vomiting and weight loss were consistent symptom and appeared after 6-8 week of corrosive ingestion and 50% of patient of post corrosive ingestion pyloric stenosis had concomitant esophageal stricture. In present study those patient operated with Lap Assisted Gastrojejunostomy had smaller size of incision, reduce intra operative need of blood transfusion, less post-operative pain and less chance of wound infection, early drain and suture removal and early discharged from hospital with minimal post-operative morbidity and without significant increase in total duration and cost of operation. Conclusion : As compared to Open Gastrojejunostomy, Lap Assisted Gastrojejunostomy is better alternative operative method for pyloric stenosis. [Natl J Med Res 2016; 6(1.000: 48-50

  17. Early gastric cancer and its complications: bleeding, perforation and pyloric stenosis.

    Directory of Open Access Journals (Sweden)

    Itano,Satoshi

    1983-10-01

    Full Text Available Some cases of early gastric cancer are accompanied with complications of the upper gastro-intestinal tract. The characteristics of these complications were investigated, and the problems of diagnosis and treatment were discussed. Out of 297 cases of early gastric cancer, 18 cases were accompanied with complications of the upper gastro-intestinal tract, including 11 cases of bleeding, a case of perforation and 6 cases of pyloric stenosis. All 18 cases were of the macroscopically depressed type, and about 85 percent of the 297 early gastric cancer cases were of the depressed type. The depressed lesions were often accompanied by ulceration which was an important factor causing the complications, and the mechanism of which appeared to be the same as that of a benign ulcer. There are some cases of early gastric cancer which are discovered by their complications, and it would be more difficult to find an early gastric cancer lesion if there were a benign lesion at the same time. Therefore, it is necessary to take much care when diagnosing and treating cases which have such complications. An endoscopic examination before the operation is especially important, and a biopsy is indispensable.

  18. Fixed subaortic stenosis associated with hypertrophic cardiomyopathy: report of a rare familial occurrence.

    Science.gov (United States)

    Conte, M R; Bongioanni, S; Dall'Orto, G; Nicastro, C; Bonfiglio, G; Morello, M; Mangiardi, L; Brusca, A

    1998-01-01

    Fixed subaortic stenosis is considered to be an acquired condition. It is often associated with congenital heart disease, creating a turbulence in the left ventricle outflow tract. Familial forms of fixed subaortic stenosis are very unusual. We report a remarkable familial cluster in which fixed subaortic stenosis is associated with hypertrophic cardiomyopathy. Fourteen relatives of a patient affected with hypertrophic cardiomyopathy and fixed subaortic stenosis underwent cardiological examination, electrocardiogram and echo-doppler study. Two of the proband's sisters showed an association between asymmetrical hypertrophic cardiomyopathy and fixed subaortic stenosis. The brother presented a subaortic ridge and concentric left ventricular hypertrophy. The other members of the family (another brother and the third-generation relatives) were unaffected. While the association between fixed subaortic stenosis and hypertrophic cardiomyopathy has commonly been reported, there is little in the literature to suggest the family-related nature of this association. The familial occurrence of this association reveals genetic transmission, with a recessive autosomal pattern of inheritance. This finding goes against the usual autosomal dominant pattern of inheritance in hypertrophic cardiomyopathy. Familial studies of FSS are needed in order to gain a better understanding of the genetic background of these patients.

  19. Physiological hypertrophic subaortic stenosis and subendocardial infarction in a patient with a pheochromocytoma.

    Science.gov (United States)

    Levister, E C; Taylor, J B

    1981-04-01

    This report describes the occurrence of a pheochromocytoma in a middle-aged, black female with a 12-year history of hypertension, and a strong family history of hypertension. In this case, the pheochromocytoma was associated with a subendocardial myocardial infarction and congestive heart failure which occurred in the presence of large, dilated coronary arteries without intraluminal obstructions. The patient also had the murmur and echocardiographic and ventriculographic signs typical of idiopathic hypertrophic subaortic stenosis, which resolved following removal of the tumor and return of the blood pressure to normal. The authors believe this to represent a form of transient physiological hypertrophic subaortic stenosis secondary to a hypercatecholamine state.

  20. 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.

  1. [Clinical and therapeutical considerations regarding a rare case of pre-pyloric stenosis].

    Science.gov (United States)

    Sabetay, C; Zavate, A; Ciuca, M; Ciobanu, O; Malos, A

    2009-01-01

    The neonatal obstacle caused by a pre-pyloric diaphragm represent a rare cause of high oclusion in new-born. We present the case of a 6 days old new-born admitted in our department for nonbilious vomiting and feeding intolerance in which the clinical exam and the imagistic explorations (plain and contrast abdominal X-Rays, and ultrasound) could not reveal an evident cause for the oclusion. The surgical intervention imposed by the simptoms revealed the existece of a complete diaphragm in the prepyloric region. The initial excision of the diaphragm was not sufficient, the patient undergoing a second surgical intervention of gastrojejunal anastromosis with favorable evolution this time. The authors are presenting diagnosis and theraputical management peculiarities of this rare condition.

  2. Pyloric Stenosis (For Parents)

    Science.gov (United States)

    ... for the messier aspects of child rearing: poopy diapers, food stains, and of course, spit up. But ... more than 4 to 6 hours between wet diapers. After feeds, increased stomach contractions may make noticeable ...

  3. 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...

  4. Solitary plexiform neurofibroma determining pyloric obstruction: a case report

    Directory of Open Access Journals (Sweden)

    Eduardo Cambruzzi

    2014-06-01

    Full Text Available Solitary gastric plexiform neurofibroma (PN is a very rare tumor that originates from the peripheral nerves. PN is a rare cause of pyloric obstruction. A 58 year-old man, reported epigastric discomfort, nausea, and vomiting for two months. Upper digestive endoscopy showed a moderate/accentuated pyloric stenosis. Computed tomography (CT and echoendoscopy revealed a pyloric nodule. The patient underwent to distal gastrectomy. Macroscopically, a gray nodule measuring 1.1 × 1.0 × 1.0 cm was identified. Using microscopy, a benign tumor composed of enlarged tortuous nerve fascicles showing a neurofibromatous proliferation with mild atypia and myxoid matrix was found. The lesion showed positive immunoexpression for S100, Leu7, and epithelial membrane antigen (EMA, and was negative for CD117, DOG-1, desmin, and smooth muscle actin. The diagnosis of PN was then determined.

  5. Post-pyloric feeding

    Institute of Scientific and Technical Information of China (English)

    Eva Niv; Zvi Fireman; Nachum Vaisman

    2009-01-01

    Postpyloric feeding is an important and promising alternative to parenteral nutrition. The indications for this kind of feeding are increasing and include a variety of clinical conditions, such as gastroparesis, acute pancreatitis, gastric outlet stenosis, hyperemesis (including gravida), recurrent aspiration, tracheoesophageal fistula and stenosis in gastroenterostomy. This review discusses the differences between pre- and postpyloric feeding, indications and contraindications, advantages and disadvantages, and provides an overview of the techniques of placement of various postpyloric devices.

  6. HYPERTROPHIC CARDIOMYOPATHY IN MULTIMORBIDITY

    Directory of Open Access Journals (Sweden)

    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.

  7. Pyloric atresia associated with epidermolysis bullosa

    Energy Technology Data Exchange (ETDEWEB)

    Garcia Hernandez, J.B.; Orense, M.; Celorio, C.; Canga, C.

    1987-07-01

    Since the first reported case of pyloric atresia and epidermolysis bullosa in 1977 by Korber and Glasson, this association has been firmly established. We present a new case giving particular emphasis to the theory that pyloric atresia is secondary to the primary disease epidermolysis bullosa.

  8. Torus hyperplasia of the pyloric antrum.

    Science.gov (United States)

    Kim, Chi-Hun; Han, Hye Seung; Lee, Sun-Young; Kim, Byung Kook; Sung, In-Kyung; Seong, Moo Kyung; Lee, Kyung Yung

    2010-01-01

    Primary or idiopathic hypertrophy of the pyloric muscle in adult, so called torus hyperplasia, is an infrequent but an established entity. It is caused by a circular muscle hypertrophy affecting the lesser curvature near the pylorus. Since most of the lesions are difficult to differentiate from tumor, distal gastrectomy is usually preformed to rule out most causes of pyloric lesions including neoplastic ones through a pathological study. A 56-yr-old man with a family history of gastric cancer presented with abdominal discomfort of 1 month duration. Upper gastrointestinal endoscopy showed a 1.0 cm sized irregular submucosal lesion proximal to the pylorus to the distal antrum on the lesser curvature. On colonoscopy examination, a 1.5 cm sized protruding mass was noticed on the appendiceal orifice. Gastrectomy and cecectomy were done, and histological section revealed marked hypertrophy of the distal circular pyloric musculature and an appendiceal mucocele. To the best of our knowledge, this is the first case of torus hyperplasia with appendiceal mucocele which is found incidentally.

  9. Spinal Stenosis

    Science.gov (United States)

    ... Pharyngitis, Adenitis Syndrome (Juvenile) Polymyalgia Rheumatica Psoriatic Arthritis Raynaud's Phenomenon Reactive Arthritis Rheumatoid Arthritis Scleroderma Sjogren's Syndrome Spinal Stenosis Spondyloarthritis Systemic Lupus Erythematosus (Juvenile) Takayasu's ...

  10. 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.

  11. Glottic stenosis.

    Science.gov (United States)

    Stephenson, Kate A; Wyatt, Michelle E

    2016-06-01

    Glottic stenosis is a fixed, focal narrowing at the level of the laryngeal inlet, the true vocal cords. It may be either congenital or acquired and be related to a wide range of etiologies. The stenosis may be either anterior, posterior, or in rare cases, complete. Isolated glottic stenosis is rare; lesions often involve adjacent regions, namely the subglottis. A diagnosis is made from careful history and examination, including evaluation by microlaryngoscopy and bronchoscopy. The management of glottic stenosis is challenging and should be tailored to each individual case. A secure and adequate airway is the treatment priority alongside optimization of voice and laryngeal competence. Endoscopic and open techniques in either single or multiple stages have been described. Copyright © 2016. Published by Elsevier Inc.

  12. Aortic stenosis

    Science.gov (United States)

    ... Images Aortic stenosis Heart valves References Carabello BA. Valvular heart disease. In: Goldman L, Schafer AI, eds. Goldman's Cecil ... ACC guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/ ...

  13. Spinal Stenosis

    Science.gov (United States)

    ... risk. Diseases such as arthritis and scoliosis can cause spinal stenosis, too. Symptoms might appear gradually or not at all. They include Pain in your neck or back Numbness, weakness, cramping, or pain in ...

  14. 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.

  15. Pyloric atresia epidermolysis bullosa aplasia cutis syndrome: a case ...

    African Journals Online (AJOL)

    Pyloric atresia epidermolysis bullosa aplasia cutis syndrome: a case report and literature ... skin over the right leg from the knee joint up to the middle of the right foot. ... Examination indicated no signs of child abuse; the parents refused an ...

  16. Pyloric obstruction secondary to epicardial pacemaker implantation: a case report.

    Science.gov (United States)

    Bedoya Nader, G; Kellihan, H B; Bjorling, D E; McAnulty, J

    2017-02-01

    A 10-year old Lhasa Apso dog was presented for an acute history of exercise intolerance and hind limb weakness. High grade second degree atrioventricular block with an atrial rate of 200 beats per minute, ventricular rate of 40 beats per minute and an intermittent ventricular escape rhythm, was diagnosed on electrocardiograph. A transdiaphragmatic, unipolar, epicardial pacemaker was implanted without immediate surgical complications. Severe vomiting was noted 12 h post-operatively. Abdominal ultrasound and a barium study supported a diagnosis of pyloric outflow obstruction and exploratory abdominal surgery was performed. The pyloric outflow tract appeared normal and no other causes of an outflow obstruction were identified. The epicardial generator was repositioned from the right to the left abdominal wall. Pyloric cell pacing was presumed to be the cause for the pyloric obstruction and severe vomiting, and this was thought to be due to close proximity of the pacemaker generator to the pylorus situated in the right abdominal wall. Repositioning of the pulse generator to the left abdominal wall resulted in resolution of vomiting. Copyright © 2016 Elsevier B.V. All rights reserved.

  17. New perspectives in Hypertrophic Cardiomyopathy

    NARCIS (Netherlands)

    M.J.M. Kofflard (Marcel)

    1998-01-01

    textabstractHypertrophic cardiomyopathy is a primary cardiac disorder with a heterogeneous expression. Although relatively uncommon, the disease has been studied extensively as appears from the numerous studies that have explored specific facets of hypertrophic cardiomyopathy. This review will focus

  18. The subaortic tendon as a mimic of hypertrophic cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Ker James

    2009-07-01

    Full Text Available Abstract Originally described by Brock and Teare, today hypertrophic cardiomyopathy is clinically defined as left (or right ventricular hypertrophy without a known cardiac or systemic cause, such as systemic hypertension, Fabry's disease or aortic stenosis. Also appreciated today is the enormous genotypic and phenotypic heterogeneity of this disease with more than 300 mutations over more than 24 genes, encoding various sarcomeric, mitochondrial and calcium-handling proteins, all as genetic causes for hypertrophic cardiomyopathy. Phenotypically, the disease can vary from negligible to extreme hypertrophy, affecting either the left and/or right ventricle in an apical, midventricular or subaortic location. Left ventricular false tendons are thin, fibrous or fibromuscular structures that traverse the left ventricular cavity. Recently, a case report was presented where it was shown that such a false tendon, originating from a subaortic location, was responsible for striking ST-segment elevation on the surface electrocardiogram. In this case report, a case is presented where such a subaortic tendon led to the classic echocardiographic appearance of hypertrophic cardiomyopathy, thus in the assessment of hypertrophic cardiomyopathy, this entity needs to be excluded in order to prevent a false positive diagnosis of hypertrophic cardiomyopathy.

  19. Hypertrophic pachymeningitis: case report

    Directory of Open Access Journals (Sweden)

    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.

  20. Hypertrophic discoid lupus erythematosus.

    Science.gov (United States)

    Farley-Loftus, Rachel; Elmariah, Sarina B; Ralston, Jonathan; Kamino, Hideko; Franks, Andrew G

    2010-11-15

    Hypertrophic discoid lupus erythematosus is a distinct form of chronic cutaneous (discoid) lupus, which is characterized by hyperkeratotic plaques that typically are observed over the face, arms, and upper trunk. We present the case of a 43-year-old man with verrucous plaques that were distributed symmetrically over the face, who initially was treated with oral antibiotics and topical glucocorticoids for acne vulgaris. A biopsy specimen confirmed the diagnosis of hypertrophic discoid lupus erythematosus. The clinical and histopathologic features of this clinical variant are reviewed.

  1. Aortic Stenosis.

    Science.gov (United States)

    Bakaeen, Faisal G; Rosengart, Todd K; Carabello, Blase A

    2017-01-03

    This issue provides a clinical overview of aortic stenosis, focusing on screening, diagnosis, treatment, and practice improvement. The content of In the Clinic is drawn from the clinical information and education resources of the American College of Physicians (ACP), including MKSAP (Medical Knowledge and Self-Assessment Program). Annals of Internal Medicine editors develop In the Clinic in collaboration with the ACP's Medical Education and Publishing divisions and with the assistance of additional science writers and physician writers.

  2. Subglottic stenosis.

    Science.gov (United States)

    Jefferson, Niall D; Cohen, Aliza P; Rutter, Michael J

    2016-06-01

    Subglottic stenosis (SGS) is a congenital or acquired condition characterized by a narrowing of the upper airway extending from just below the vocal folds to the lower border of the cricoid cartilage. With the introduction of prolonged intubation in neonates (mid 1960s), acquired SGS became the most frequent cause of laryngeal stenosis; unlike congenital SGS, it does not improve with time. Laryngeal reconstruction surgery evolved as a consequence of the need to manage these otherwise healthy but tracheotomized children. Ongoing innovations in neonatal care have gradually led to the salvage of premature and medically fragile infants in whom laryngeal pathology is often more severe, and in whom stenosis often involves not only the subglottis, but also the supraglottis or glottis-causing significant morbidity and mortality. The primary objective of intervention in these children is decannulation or preventing the need for tracheotomy. The aim of this article is to present a more detailed description of both congenital and acquired SGS, highlighting the essentials of diagnostic assessment and familiarizing the reader with contemporary management approaches. Copyright © 2016 Elsevier Inc. All rights reserved.

  3. Hypertrophic Cardiomyopathy Association

    Science.gov (United States)

    ... be donated to Hypertrophic Cardiomyopathy Association. iGive.com - Online Shopping Joing iGive.com to earn money for the ... it works, check out the iGive website . AmazonSmile - Online Shopping Amazon donates 0.5% of the purchase price ...

  4. Gastric pseudo-ulcers: membrana angularis and pyloric torus defects.

    Science.gov (United States)

    Peavy, P W; Clements, J L; Weens, H S

    1975-03-01

    The membrana angularis and pyloric torus defects are two physiologic bulges which can simulate ulcerations along the lesser curvature of the stomach. The muscular anatomy of the stomach and the mechanism which produces these pseudo-ulcers are discussed. Both pseudoniches can be seen transiently in normal individuals but occasionally are such prominence as to become diagnostic pitfalls. The features and significance of each pseudo-ulcer are reviewed in an attempt to facilitate recognition on the upper gastrointestinal barium examination.

  5. Mathematical Modeling of Subthreshold Resonant Properties in Pyloric Dilator Neurons

    OpenAIRE

    Vazifehkhah Ghaffari, Babak; Kouhnavard, Mojgan; Aihara, Takeshi; Kitajima, Tatsuo

    2015-01-01

    Various types of neurons exhibit subthreshold resonance oscillation (preferred frequency response) to fluctuating sinusoidal input currents. This phenomenon is well known to influence the synaptic plasticity and frequency of neural network oscillation. This study evaluates the resonant properties of pacemaker pyloric dilator (PD) neurons in the central pattern generator network through mathematical modeling. From the pharmacological point of view, calcium currents cannot be blocked in PD neur...

  6. Educational outcome in adolescence following pyloric stenosis repair before 3 months of age

    DEFF Research Database (Denmark)

    Hansen, Tom G; Pedersen, Jacob K; Henneberg, Steen W

    2013-01-01

    Immature animals exposed to anesthetics display apoptotic neurodegeneration with subsequent long-term cognitive dysfunctions. Young age at anesthetic exposure is believed to be critical, but human studies are scarce. This study investigated the association between exposure to surgery and anesthesia...

  7. Genetics Home Reference: familial hypertrophic cardiomyopathy

    Science.gov (United States)

    ... 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. Metabotropic glutamate receptor agonists modify the pyloric output of the crustacean stomatogastric ganglion.

    Science.gov (United States)

    Pérez-Acevedo, Nivia L; Krenz, Wulf D

    2005-11-16

    We have studied the effects of groups I, II, and III metabotropic glutamate receptor (mGluR) agonists and antagonists on pyloric activity in the stomatogastric ganglion (STG) of the Caribbean spiny lobster Panulirus argus. We have found that agonists for all three groups of mGluRs modify the pyloric output. The group I agonist, l-quisqualic acid (l-QA), activated the pyloric central pattern generator (CPG). When the pyloric rhythm was partially suppressed by sucrose-block of input fibers in the stomatogastric nerve (stn), l-QA accelerated the rhythmic activity. In addition, the number of spike discharges was increased in pyloric motoneurons: pyloric (PY), and lateral pyloric (LP). In completely blocked preparations, a slow pyloric rhythm was initiated by l-QA. Groups II and III agonists exerted an inhibitory effect on pyloric activity. The group II agonist, (2S,1'S,2'S)-2-(Carboxycyclopropyl)glycine (L-CCG-I), decreased both the frequency of the pyloric rhythm and the number of spike discharges in the motoneurons: ventricular dilator (VD), PY, and LP. The effects of L-CCG-I were dose-dependent. The group III agonist, l-(+)-2-Amino-4-phosphonobutyric acid (l-AP4), slightly decreased the frequency of the pyloric rhythm and suppressed spike discharges in the VD neuron. All effects of mGluR agonists were reversible. The effect of l-QA was blocked by the broad spectrum mGluR antagonist (S)-Methyl-4-carboxyphenylglycine (MCPG). The inhibitory effect of L-CCG-I was prevented by MCPG and by the group II/III mGluR antagonist (RS)-alpha-Methyl-4-phosphonophenylglycine (MPPG), and was partially blocked by the group II mGluR antagonist (RS)-1-amino-5-phosphonoindan-1-carboxylic acid (APICA). The inhibitory effect of l-AP4 was blocked by MPPG and partially blocked by APICA.

  9. PYLORIC ATRESIA IN ASSOCIATION WITH MULTIPLE COLONIC ATRESIAS IN A NEONATE: AN UNREPORTED ASSOCIATION

    Directory of Open Access Journals (Sweden)

    Vijay C Pujar

    2012-01-01

    Full Text Available Pyloric atresia is rare cause of gastrointestinal obstruction in neonates and usually occurs as an isolated anomaly. They have been associated with multiple small bowel and colonic atresias but not reported in association with isolated multiple colonic atresias. A case of pyloric atresia oc-curring in association with multiple colonic atresias is being reported here.

  10. Pulmonary Valve Stenosis

    Science.gov (United States)

    ... growths called carcinoid tumors in the digestive system. Rheumatic fever. This complication of an infection caused by streptococcus ... valve stenosis later in life, including: Carcinoid syndrome Rheumatic fever Noonan's syndrome Mild to moderate pulmonary valve stenosis ...

  11. Hypertrophic cardiomyopathy South African Blacks

    African Journals Online (AJOL)

    1983-02-19

    Feb 19, 1983 ... Hypertrophic cardiomyopathy (HCM) is an important cardiac lesion to .... showed vigorous myocardial contraction with a markedly increased ejection ... There may be an inherited malformation of muscle in different parts of the ...

  12. Update on hypertrophic scar treatment

    Directory of Open Access Journals (Sweden)

    Felipe Bettini Rabello

    2014-08-01

    Full Text Available Scar formation is a consequence of the wound healing process that occurs when body tissues are damaged by a physical injury. Hypertrophic scars and keloids are pathological scars 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 scars, 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 scars, 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.

  13. Analog electronic model of the lobster pyloric central pattern generator

    Energy Technology Data Exchange (ETDEWEB)

    Volkovskii, A [Institute for Nonlinear Science, University of California San Diego, CA (United States); Brugioni, S [Institute for Nonlinear Science, University of California San Diego, CA (United States); Istituto Nazionale di Ottica Applicata Largo E. Fermi 6 50125 Florence (Italy); Levi, R [Institute for Nonlinear Science, University of California San Diego, CA (United States); Rabinovich, M [Institute for Nonlinear Science, University of California San Diego, CA (United States); Selverston, A [Institute for Nonlinear Science, University of California San Diego, CA (United States); Abarbane, H D I [Institute for Nonlinear Science, University of California San Diego, CA (United States)

    2005-01-01

    An electronic circuit intended to simulate the nonlinear dynamics of a simplified 3-cell model of the pyloric central pattern generator in California spiny lobster stomato gastric ganglion is presented. The model employs the synaptic phase locked loop (SPLL) concept where the frequency of oscillations of a postsynaptic cell is mainly controlled by the synaptic current which depends on the phase shift between the oscillations. The theoretical study showed that the system has a stable steady state with correct phase shifts between the oscillations and that this regime is stable when the frequency of the pacemaker cell is varied over a wide range. The main bifurcations in the system were studied analytically, in computer simulations, and in experiments with the electronic circuit. The experimental measurements are in good agreement with the expectations of the theoretical model.

  14. Acquired lumbar spinal stenosis.

    Science.gov (United States)

    Deasy, JoAnn

    2015-04-01

    Lumbar spinal stenosis is the most frequent reason for spinal surgery in patients over age 65 years. In this condition, narrowing of the lumbar spinal canal and nerve root canals leads to painful, debilitating compression of spinal nerves and blood vessels. As the population ages, an increasing number of patients will be diagnosed and treated for lumbar spinal stenosis by primary care providers. This article reviews the pathophysiology, diagnosis, and management of lumbar spinal stenosis in adults over age 50 years.

  15. Genetic biomarkers in hypertrophic cardiomyopathy.

    Science.gov (United States)

    Coats, Caroline J; Elliott, Perry M

    2013-08-01

    Hypertrophic cardiomyopathy is a common inherited heart muscle disorder associated with sudden cardiac death, arrhythmias and heart failure. Genetic mutations can be identified in approximately 60% of patients; these are commonest in genes that encode proteins of the cardiac sarcomere. Similar to other Mendelian diseases these mutations are characterized by incomplete penetrance and variable clinical expression. Our knowledge of this genetic diversity is rapidly evolving as high-throughput DNA sequencing technology is now used to characterize an individual patient's disease. In addition, the genomic basis of several multisystem diseases associated with a hypertrophic cardiomyopathy phenotype has been elucidated. Genetic biomarkers can be helpful in making an accurate diagnosis and in identifying relatives at risk of developing the condition. In the clinical setting, genetic testing and genetic screening should be used pragmatically with appropriate counseling. Here we review the current role of genetic biomarkers in hypertrophic cardiomyopathy, highlight recent progress in the field and discuss future challenges.

  16. Burns, hypertrophic scar and galactorrhea.

    Science.gov (United States)

    Karimi, Hamid; Nourizad, Samad; Momeni, Mahnoush; Rahbar, Hosein; Momeni, Mazdak; Farhadi, Khosro

    2013-07-01

    An 18-year-old woman was admitted to Motahari Burn Center suffering from 30% burns. Treatment modalities were carried out for the patient and she was discharged after 20 days. Three to four months later she developed hypertrophic scar on her chest and upper limbs. At the same time she developed galactorrhea in both breasts and had a disturbed menstrual cycle four months post-burn. On investigation, we found hyperprolactinemia and no other reasons for the high level of prolactin were detected.She received treatment for both the hypertrophic scar and the severe itching she was experiencing. After seven months, her prolactin level had decreased but had not returned to the normal level. It seems that refractory hypertrophic scar is related to the high level of prolactin in burns patients.

  17. Burns, hypertrophic scar and galactorrhea

    Directory of Open Access Journals (Sweden)

    Hamid Karimi

    2013-07-01

    Full Text Available An 18-year old woman was admitted to Motahari Burn Center suffering from 30% burns. Treatment modalities were carried out for the patient and she was discharged after 20 days. Three to four months later she developed hypertrophic scar on her chest and upper limbs .At the same time she developed galactorrhea in both breasts and had a disturbed menstrual cycle four months post-burn. On investigation, we found hyperprolactinemia and no other reasons for the high level of prolactin were detected. She received treatment for both the hypertrophic scar and the severe itching she was experiencing. After seven months, her prolactin level had decreased but had not returned to the normal level. It seems that refractory hypertrophic scar is related to the high level of prolactin in burns patients.

  18. Mathematical Modeling of Subthreshold Resonant Properties in Pyloric Dilator Neurons

    Directory of Open Access Journals (Sweden)

    Babak Vazifehkhah Ghaffari

    2015-01-01

    Full Text Available Various types of neurons exhibit subthreshold resonance oscillation (preferred frequency response to fluctuating sinusoidal input currents. This phenomenon is well known to influence the synaptic plasticity and frequency of neural network oscillation. This study evaluates the resonant properties of pacemaker pyloric dilator (PD neurons in the central pattern generator network through mathematical modeling. From the pharmacological point of view, calcium currents cannot be blocked in PD neurons without removing the calcium-dependent potassium current. Thus, the effects of calcium ICa and calcium-dependent potassium IKCa currents on resonant properties remain unclear. By taking advantage of Hodgkin-Huxley-type model of neuron and its equivalent RLC circuit, we examine the effects of changing resting membrane potential and those ionic currents on the resonance. Results show that changing the resting membrane potential influences the amplitude and frequency of resonance so that the strength of resonance (Q-value increases by both depolarization and hyperpolarization of the resting membrane potential. Moreover, hyperpolarization-activated inward current Ih and ICa (in association with IKCa are dominant factors on resonant properties at hyperpolarized and depolarized potentials, respectively. Through mathematical analysis, results indicate that Ih and IKCa affect the resonant properties of PD neurons. However, ICa only has an amplifying effect on the resonance amplitude of these neurons.

  19. Mathematical modeling of subthreshold resonant properties in pyloric dilator neurons.

    Science.gov (United States)

    Vazifehkhah Ghaffari, Babak; Kouhnavard, Mojgan; Aihara, Takeshi; Kitajima, Tatsuo

    2015-01-01

    Various types of neurons exhibit subthreshold resonance oscillation (preferred frequency response) to fluctuating sinusoidal input currents. This phenomenon is well known to influence the synaptic plasticity and frequency of neural network oscillation. This study evaluates the resonant properties of pacemaker pyloric dilator (PD) neurons in the central pattern generator network through mathematical modeling. From the pharmacological point of view, calcium currents cannot be blocked in PD neurons without removing the calcium-dependent potassium current. Thus, the effects of calcium (I(Ca)) and calcium-dependent potassium (I(KCa)) currents on resonant properties remain unclear. By taking advantage of Hodgkin-Huxley-type model of neuron and its equivalent RLC circuit, we examine the effects of changing resting membrane potential and those ionic currents on the resonance. Results show that changing the resting membrane potential influences the amplitude and frequency of resonance so that the strength of resonance (Q-value) increases by both depolarization and hyperpolarization of the resting membrane potential. Moreover, hyperpolarization-activated inward current (I(h)) and I(Ca) (in association with I(KCa)) are dominant factors on resonant properties at hyperpolarized and depolarized potentials, respectively. Through mathematical analysis, results indicate that I h and I(KCa) affect the resonant properties of PD neurons. However, I(Ca) only has an amplifying effect on the resonance amplitude of these neurons.

  20. Genetic basis of hypertrophic cardiomyopathy

    NARCIS (Netherlands)

    Bos, J.M.

    2010-01-01

    The understanding of hypertrophic cardiomyopathy (HCM) has matured from its cornerstone as a disease of the sarcomere to a compendium of diseases with various clinical, genetic and morphologic substrates. Research has provided us more insights into i) the pathogenetic development of HCM, ii) the pos

  1. Improving Outcomes in Hypertrophic Cardiomyopathy

    NARCIS (Netherlands)

    P.A. Vriesendorp (Pieter)

    2016-01-01

    markdownabstractImproving outcomes in hypertrophic cardiomyopathy (HCM) is focused on the improvement of the therapeutic strategies for patients with HCM. First it demonstrates that individual patient selection in patients with obstructive and symptomatic HCM can lead to near normal life-expectancy;

  2. Radiotherapy of hypertrophic connective tissue

    Energy Technology Data Exchange (ETDEWEB)

    Haase, W.

    1982-10-11

    Peyronie's disease, Dupuytren's contractures and keloids produce similar pathologic-anatomic alterations of hypertrophic tissue. Different therapeutic modalities are presented in detail. The radiation therapy is one of the most effective treatments. Radiation technique, dose and fractionation are discussed. The results of the different modalities are reviewed.

  3. Anatomical and histological investigation of the pyloric caeca in beluga (Huso huso

    Directory of Open Access Journals (Sweden)

    Hooman Rahmati-holasoo

    2011-07-01

    Full Text Available The objective of this paper was to study anatomy and histology of pyloric caeca in 2 years old beluga (Huso huso (Linnaeus, 1758. In this study 12, 2-year old male and female H. huso were included. Anatomical position of pyloric caeca was studied through dissection. Histological specimens were fixed in 10% formalin buffer phosphate for 72 h. The specimens were processed through paraffin embedding and 7 micrometer sections were cut and stained by Hematoxylin and eosin and observed under light microscope. It was shown that pyloric caeca in H. huso were attached and formed a large mass between stomach and duodenum. The capsule was a smooth muscle that had dispatched trabeculae into the organ and formed lobules. There were numerous villi with simple columnar epithelium in different sizes in each lobule. It was demonstrated that pyloric caeca in H. huso like other sturgeon fish were attached to each other and formed an organ called pyloric caeca with a long leaf-shaped villi.

  4. Das gastral differenzierte Adenom (pyloric gland adenoma. Fact or Fiction?

    Directory of Open Access Journals (Sweden)

    Vieth M

    2005-01-01

    Full Text Available Das gastral differenzierte Adenom des Magens wird in der englischen Literatur als "pyloric gland adenoma" bezeichnet. Der erste Bericht zu dieser Entität erschien 1976 als Buchbeitrag, allerdings wurde die Läsion damals als eine Adenom-artige Hyperplasie mukoider Drüsen beschrieben. Erst ab 1990 wurden Pylorusdrüsen-Adenome korrekt als gastral differenzierte Adenome identifiziert und diagnostische Kriterien entwickelt. Die Bezeichnung Pylorusdrüsen-Adenom spiegelt die Entstehung aus den tiefen gastralen Drüsenverbänden wieder. Sicher sind die Pylorusdrüsen-Adenome bereits im Routine-HE-Präparat zu diagnostizieren und durch die Immunhistochemie zu bestätigen. Typischerweise exprimieren die Pylorusdrüsen-Adenome Mucin 6, das sonst nur im tiefen Drüsenkörper gefunden werden kann, über die gesamte Breite der Läsion, oft bis zur Oberfläche. Das oberflächliche Mucin 5AC ist oft nur auf eine schmale oberflächliche Zone beschränkt; die Expression kann jedoch stark variieren. Eine Kombination mit Anteilen gewöhnlicher tubulärer Adenome (intestinale Differenzierung kann beobachtet werden. Überproportional häufig ist die Magen-Corpusschleimhaut älterer Patientinnen mit Autoimmungastritis betroffen. Die Häufigkeit von Pylorusdrüsen-Adenomen wird mit 2,7 % aller Magenpolypen angegeben und ist damit nicht so selten wie vermutet. Die wenigen vorhandenen Publikationen zu diesem Thema deuten darauf hin, daß diese Entität meist fehlinterpretiert wird. Pylorusdrüsen-Adenome können im gesamten Gastrointestinaltrakt entstehen. Die klinische Bedeutung liegt in der hohen Rate (30 % der malignen Entartung. Allerdings handelt es sich hierbei vorwiegend um hochdifferenzierte Frühkarzinome mit entsprechend guter Prognose nach endoskopischer Abtragung.

  5. Comparative morphology of the pyloric armature of adult mosquitoes (Diptera: Culicidae).

    Science.gov (United States)

    Tuten, H C; Bridges, W C; Adler, P H

    2012-09-01

    The structure of the pyloric armature, hypothesized to aid in blood-meal digestion or parasite resistance, was compared quantitatively among the following 8 species in 5 genera of adult mosquitoes from the southeastern United States: Aedes albopictus, Aedes japonicus, Aedes triseriatus, Anopheles punctipennis, Culex pipiens s.l., Culex restuans, Orthopodomyia signifera, and Toxorhynchites rutilus. Females differed significantly among species in the structure of spines composing the armature, with Aedes spp. forming one general group, Culex spp. another, and An. punctipennis and Or. signifera a third. Relationships of species based on structural characters of the armature were consistent with recent culicid phylogenies. Although pyloric armature has been noted in mosquitoes and other insects, this is the first quantitative investigation of the mosquito pyloric armature.

  6. Congenital Pyloric Atresia with Distal Duodenal Atresia- Role of CT Scan

    Directory of Open Access Journals (Sweden)

    Yogender Singh Kadian

    2014-07-01

    Full Text Available The mainstay of diagnosis of congenital pyloric atresia is by plain X-ray of the abdomen showing a large gas bubble with no gas distally. But very rarely it can be associated with distal duodenal atresia when the baby may present as lump abdomen. In such a situation apart from the X-ray, another radiological investigation is needed to delineate the exact nature of the lump. Since the role of ultrasonography is limited in intestinal pathologies and contrast studies are not informative in atresias, the CT scan is the ideal choice. We had managed a case of pyloric atresia with similar presentation with preoperative CT scan.

  7. Aortic Valve Stenosis

    Science.gov (United States)

    ... evaluation of aortic stenosis in adults. http://www.uptodate.com/home. Accessed April 29, 2014. Mohty D, ... Valvular heart disease in elderly adults. http://www.uptodate.com/home. Accessed May 2, 2014. Bonow RO, ...

  8. Mitral Valve Stenosis

    Science.gov (United States)

    ... up around the ring around the mitral valve (annulus), which can occasionally cause mitral valve stenosis. Other ... the condition of your lungs. Transesophageal echocardiogram. A small transducer attached to the end of a tube ...

  9. Severe Gastrointestinal Bleeding in a Patient With Subvalvular Aortic Stenosis Treated With Thalidomide and Octreotide

    DEFF Research Database (Denmark)

    Hvid-Jensen, Helene S; Poulsen, Steen H; Agnholt, Jørgen S

    2015-01-01

    Gastrointestinal bleeding (GB) due to angiodysplasias can cause severe, recurrent bleeding, especially in elderly patients. Angiodysplastic bleedings in the gastrointestinal tract have been associated with aortic stenosis and, more recently, hypertrophic obstructive cardiomyopathy, caused...... to resolve bleeding, especially in patients with large numbers of angiodysplasias. In patients with aortic stenosis and GB, the main treatment is aortic valve replacement but the patients may be unfit to undergo surgery due to the complicating anemia. In this case story, we present a patient with severe, GB...

  10. [The thickness/radius ratio of the left ventricle in aortic stenosis. Prognostic and therapeutic implications].

    Science.gov (United States)

    Guadalajara, J F; Martínez, C; Huerta, D

    1990-01-01

    Using two-D echocardiography and cardiac catheterization we studied the performance of left ventricle in severe aortic stenosis with normal ventricular function (10 patients), and with heart failure (11 patients). With appropriate hypertrophy increased ventricular function, is found resulting in systolic wall stress normalization. When hypertrophic mechanism is unable to normalize the systolic wall stress; afterload increases with ensuing heart failure (inadequate hypertrophy). Surgical treatment in those cases reduces the afterload and increases de ventricular function. Normalization of systolic wall stress in patients with severe aortic stenosis and heart failure means irreversible myocardial damage.

  11. Fluoroscopic insertion of post-pyloric feeding tubes: success rates and complications

    Energy Technology Data Exchange (ETDEWEB)

    Thurley, P.D.; Hopper, M.A. [Department of Clinical Radiology, Nottingham University Hospitals NHS Trust, Nottingham (United Kingdom); Jobling, J.C. [Department of Clinical Radiology, Nottingham University Hospitals NHS Trust, Nottingham (United Kingdom)], E-mail: craig.jobling@nuh.nhs.uk; Teahon, K. [Department of Clinical Radiology, Nottingham University Hospitals NHS Trust, Nottingham (United Kingdom)

    2008-05-15

    Aim: To examine the success and complication rates of radiological placement of post-pyloric feeding tubes, including those inserted with the assistance of a guide-wire. Materials and Methods: Two hundred referrals (156 patients), between the dates of 5 April 2002 and 10 September 2004, were identified retrospectively from computerized records. Subsequently, the radiology reports and patients' notes were reviewed to evaluate the indications for post-pyloric feeding, success of placement, use of a guide-wire, and any complications. Results: A post-pyloric tube was placed in the distal duodenum/jejunum in 183 (91.5%) patients and in the proximal duodenum or distal stomach in six (3%). A tube could not be inserted in 11 (5.5%) patients, and 51 (25.5%) of the insertions required the use of a guide-wire. Immediate complications were recorded in seven patients (3.5%): vomiting (n = 5); hypotension and apnoea requiring naloxone (n = 1) and hypoxia requiring endotracheal intubation (n = 1). Conclusion: Radiological placement of post-pyloric feeding tubes has a success rate comparable with endoscopically placed tubes, and it rarely involves significant technique-related complications.

  12. Purification and characterization of elastase from the pyloric caeca of rainbow trout (Oncorhynchus mykiss)

    DEFF Research Database (Denmark)

    Bassompierre, Marc; Nielsen, Henrik Hauch; Børresen, Torger

    1993-01-01

    1. An elastase-like enzyme was purified from the pyloric caeca of rainbow trout by hydrophobic interaction, cation exchange and gel-filtration chromatography. 2. The approximate molecular weight of the elastase was 27 kDa and the isoelectric point was remarkably basic. 3. The pH optimum of this e...

  13. 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.

  14. An autopsy report of acute myocardial infarction with hypertrophic obstructive cardiomyopathy-like heart.

    Science.gov (United States)

    Ushikoshi, Hiroaki; Okada, Hideshi; Morishita, Kentaro; Imai, Hajime; Tomita, Hiroyuki; Nawa, Takahide; Suzuki, Kodai; Ikeshoji, Haruka; Kato, Hisaaki; Yoshida, Takahiro; Yoshida, Shozo; Shirai, Kunihiro; Toyoda, Izumi; Hara, Akira; Ogura, Shinji

    2015-01-01

    An 84-year-old woman, who was followed up as hypertrophic obstructive cardiomyopathy (HOCM) in a local hospital, was transferred to our center because of anterior chest pain and diagnosed with acute myocardial infarction (MI). Coronary angiography showed total occlusion of the mid-left anterior descending, and flow was restored after endovascular thrombectomy. An autopsy was performed after she died on hospital day 6. At autopsy, there was no significant stenosis in this vessel and the absence of plaque rupture was confirmed. Likewise, it was unclear asymmetric hypertrophy at autopsy, it could not deny that a sigmoid deformity of the basal septum occurs in elderly patients and can mimic the asymmetric septal hypertrophy of hypertrophic cardiomyopathy. MI was thought to be caused by coronary spasm or squeezing in HOCM-like heart. Therefore, it may be necessary antithrombosis therapy in HOCM-like patients with no history of paroxysmal atrial fibrillation.

  15. Characterization of phospholipase A2 from the pyloric ceca of two species of starfish, Coscinasterias acutispina and Plazaster borealis

    OpenAIRE

    Kishimura, Hideki; Hayashi, Kenji

    2005-01-01

    Phospholipase A (PLA) activities in the pyloric ceca and viscera from seven species of marine invertebrates (four starfish, one sea urchin, and two shellfish) were determined. Relatively high PLA specific activities were found in the pyloric ceca of two species of starfish (Coscinasterias acutispina and Plazaster borealis). Phospholipase A2s (PLA2s) were partially purified from the pyloric ceca of the starfish, C. acutispina PLA2 (C-PLA2) and P. borealis PLA2 (P-PLA2). The C-PLA2 and P-PLA2 m...

  16. Congenital tracheobronchial stenosis.

    Science.gov (United States)

    Hewitt, Richard J; Butler, Colin R; Maughan, Elizabeth F; Elliott, Martin J

    2016-06-01

    Congenital tracheobronchial stenosis is a rare disease characterized by complete tracheal rings that can affect variable lengths of the tracheobronchial tree. It causes high levels of morbidity and mortality both due to the stenosis itself and to the high incidence of other associated congenital malformations. Successful management of this complex condition requires a highly individualized approach delivered by an experienced multidisciplinary team, which is best delivered within centralized units with the necessary diverse expertise. In such settings, surgical correction by slide tracheoplasty has become increasingly successful over the past 2 decades such that long-term survival now exceeds 88%, with normalization of quality of life scores for patients with non-syndrome-associated congenital tracheal stenosis. Careful assessment and planning of treatment strategies is of paramount importance for both successful management and the provision of patients and carers with accurate and realistic treatment counseling. Copyright © 2016 Elsevier Inc. All rights reserved.

  17. Lumbar stenosis: clinical case

    Directory of Open Access Journals (Sweden)

    Pedro Sá

    2014-08-01

    Full Text Available Lumbar stenosis is an increasingly common pathological condition that is becoming more frequent with increasing mean life expectancy, with high costs for society. It has many causes, among which degenerative, neoplastic and traumatic causes stand out. Most of the patients respond well to conservative therapy. Surgical treatment is reserved for patients who present symptoms after implementation of conservative measures. Here, a case of severe stenosis of the lumbar spine at several levels, in a female patient with pathological and surgical antecedents in the lumbar spine, is presented. The patient underwent two different decompression techniques within the same operation.

  18. Severe iatrogenic nostril stenosis

    Directory of Open Access Journals (Sweden)

    Ali Ebrahimi

    2015-01-01

    Full Text Available Nostril stenosis (narrowing of the nasal inlet is an uncommon deformity which results in aesthetic and breathing discomfort in patients. The literature review shows that trauma, infection, iatrogenic insults and congenital lesions are major causes of stenosis. Nowadays, rhinoplasty is one of most popular aesthetic surgeries which may have complications such as bleeding, swelling, bruising, asymmetry, obstruction of nasal airways. We present a 30-year-old female patient, who complained about breathing and aesthetic difficulties due to external nasal valve obstruction and nasal deformity. Past medical history showed that the patient had undergone three unsuccessful rhinoplasty surgeries with aesthetic goals.

  19. Stenosis after stapler anastomosis.

    Science.gov (United States)

    Elliott, T E; Albertazzi, V J; Danto, L A

    1977-06-01

    Two cases of anastomotic stenosis after the use of the GIA Auto Suture Stapler are presented as examples of the potential problem that does exist in using this instrument. Possible causes and a suggestion for eliminating this complication have been outlined.

  20. [Congenital aortic stenosis].

    Science.gov (United States)

    Yamaguchi, M

    2001-08-01

    Recent advances in and controversies concerning the management of children with congenital valvular aortic stenosis are discussed. In neonates with critical aortic stenosis, improved survival has recently been reported after surgical open valvotomy and balloon valvuloplasty, although it is difficult at this point to compare the results of the two procedures and determine their differential indications. Good results have also been achieved after extended aortic valvuloplasty for recurrent aortic stenosis and/or insufficiency, but the length of follow-up in these patients is still short. The technique first reported in 1991 for bilateral enlargement fo a small annulus permits the insertion of an aortic valve 3-4 sizes larger than the native annulus. It entails no risk of distorting the mitral valve, damaging the conduction system or important branches of the coronary arteries, or resulting in left ventricular dysfunction. The Ross procedure is now widely applied in the West, with reports of early mortality rates of less than 5% and event-free survival rates of 80-90% during follow-up of 4-8 years. Longer follow-up and continued careful evaluation are required to resolve the issue of possible dilatation and subsequent neoaortic valve dysfunction and pulmonary stenosis due to allograft degeneration after pulmonary autograft root replacement in children.

  1. 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.

  2. 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.

  3. Partial characterization of pyloric-duodenal lipase of gilthead seabream (Sparus aurata).

    Science.gov (United States)

    Nolasco, Héctor; Moyano-López, Francisco; Vega-Villasante, Fernando

    2011-03-01

    In the present study, we report the isolation and characterization of seabream Sparus aurata pyloric caeca-duodenal lipase. Optimum activity was found at pH 8.5 and salinity of 50 mM NaCl. Lipase activity was sensitive to divalent ions, and extreme pH values (4, 5, and 12), being more stable at alkaline than acid pH. Optimum temperature was found at 50°C, but lipase was stable at temperatures below 40°C. Lipase has a bile salt sodium taurocholate requirement for increased activity. Gradient PAGE electrophoresis revealed the presence of four isoforms with apparent molecular masses of 34, 50, 68, and 84 KDa, respectively. Pyloric-duodenal lipase was able to hydrolyze emulsified alimentary oils. Results confirm the presence of true lipases in Sparus aurata digestive tract.

  4. The use of pyloric exclusion for treating duodenal trauma: case series

    Directory of Open Access Journals (Sweden)

    Gustavo Pereira Fraga

    Full Text Available CONTEXT AND OBJECTIVES: Significant controversy exists regarding the best surgical treatment for complex duodenal injuries. The aims of this study were to report on a series of eight cases of duodenal repairs using pyloric exclusion and to describe reported complications or improvements in clinical outcomes among patients with complex duodenal trauma. DESIGN AND SETTING: Cross-sectional study followed by a case series in a university hospital. METHODS: Data on eight patients with duodenal trauma who underwent pyloric exclusion over a 17.5 year period were collected and analyzed. RESULTS: The causes of the injuries included penetrating gunshot wounds (GSW in five patients and motor vehicle accidents (blunt trauma in three patients. The time elapsed until surgery was longer in the blunt trauma group, while in one patient, the gunshot injury was initially missed and thus the procedure was carried out 36 hours after the original injury. The injuries were grade III (50% or IV (50% and the morbidity rate was 87.5%. Four patients (50% died during the postoperative period from complications, including hypovolemic shock (one patient, sepsis (peritonitis following the missed injury and pancreatitis with an anastomotic fistula (two patients. CONCLUSIONS: Pyloric exclusion was associated with multiple complications and a high mortality rate. This surgical technique is indicated for rare cases of complex injury to the duodenum and the surgeon should be aware that treatment with a minimalistic approach, with only primary repair, may be ideal.

  5. 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.)

  6. Congenital pyriform aperture stenosis

    Energy Technology Data Exchange (ETDEWEB)

    Osovsky, Micky [Schneider Pediatric Hospital, Department of Neonatology, Petach Tikvah (Israel); Rabin Medical Center, Department of Neonatology, Schneider Children' s Medical Center of Israel, Beilinson Campus, Petah Tikvah (Israel); Aizer-Danon, Anat; Horev, Gadi [Schneider Pediatric Hospital, Department of Pediatric Radiology, Petach Tikvah (Israel); Sirota, Lea [Schneider Pediatric Hospital, Department of Neonatology, Petach Tikvah (Israel)

    2007-01-15

    Nasal airway obstruction is a potentially life-threatening condition in the newborn. Neonates are obligatory nasal breathers. The pyriform aperture is the narrowest, most anterior bony portion of the nasal airway, and a decrease in its cross-sectional area will significantly increase nasal airway resistance. Congenital nasal pyriform aperture stenosis (CNPAS) is a rare, unusual form of nasal obstruction. It should be considered in the differential diagnosis of any neonate or infant with signs and symptoms of upper airway compromise. It is important to differentiate this level of obstruction from the more common posterior choanal stenosis or atresia. CNPAS presents with symptoms of nasal airway obstruction, which are often characterized by episodic apnea and cyclical cyanosis. (orig.)

  7. Relationship between coronary atherosclerotic stenosis and cerebral atherosclerotic stenosis

    Institute of Scientific and Technical Information of China (English)

    Jiaping Wei; Shenmao Li; Zhiyong Zhang; Feng Ling; Kang Li; Hong Zhao; Jifang He; Liqing Xu; Jing Wen; Chunyan Zhou; Xiaoguang Wu; Jiarui Wang

    2008-01-01

    To investigate the relationship between severity of cerebrovascular atherosclerosis stenosis and that of coronary atherosclerosis stenosis.Methods Cerebral angiography and coronary angiography were performed in 34 patients who had coronary disease with cerebral ischemia.Patients were divided into 3 subgroups according to the degree ofstenosis on angiography,concomitant diseases,risk factors and biochemical data.Results The follow-up study showed that the incidence of cardiac and cerebrovascular death increased significantly in patients with moderate to severe stenosis of coronary and cerebral arteries;the severity of stenosis in the coronary artery parallels that in the solitary carotid artery,or dual carotid and vertebral arteries.Conclusions Patients with coronary and cerebral artery stenosis,especially those with multi-risk factors,such as hypertension,diabetes and cigarette smoking,should receive intensive treatment to reduce cardiac and cerebrovascular events.(J Geriatr Cardiol 2008;5:227-229)

  8. [Idiopathic progressive subglottic stenosis].

    Science.gov (United States)

    Sittel, C

    2014-07-01

    Idiopathic subglottic stenosis is causing a narrowing of the central airway at the laryngotracheal junction. Etiology is remaining unclear at large. There is a marked preponderance for women in the fertile age, an association to estrogene or progesterone metabolism remains doubtful. Suggested treatment varies from repeated endoscopic interventions to primary open resection. Therapy selection in this heterogeneous condition should be based on the individual patient situation as well as surgeon's expertise. This complex entity is prone to complications and should preferably be managed in a referral center.

  9. Subglottic tracheal stenosis

    Science.gov (United States)

    Venuta, Federico; Rendina, Erino Angelo

    2016-01-01

    Benign subglottic stenosis represents a major therapeutic challenge. Interventional bronchoscopic treatment has a limited role in this setting due to anatomical and technical reasons. The benefit with these techniques is generally temporary, due to frequent recurrences, need for repeated procedures and risk of extending the area of damage. Laryngotracheal resection is at present the curative treatment of choice. Literature data show that surgical treatment may allow very high success rates at long term with low perioperative morbidity and mortality. Technical aspects and results are reported and discussed. PMID:26981264

  10. 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

  11. Clinical significance of pyloric aperture in the aetiology of peptic ulcer disease: a prospective study.

    Science.gov (United States)

    Saha, Sisir Kumar

    2009-04-01

    Despite so much contributions reported in the literature, the aetiology of the duodenal ulcer remains an enigmatic subject to the medical profession. Findings of Helicobacter pylori seem to have overshadowed the real issue, in that, how a small area of the duodenal mucosa could be inflicted with the acid-pepsin injury has not been questioned? One hundred and sixty-eight consecutive patients, presented with epigastric pain were included in the endoscopic study. The aim of the study was to find out the prevalence and its clinical importance on the sizes of the pyloric aperture in the aetiology of peptic ulcer disease. Demographic data on the sizes of the pyloric aperture were divided into two groups, in that, those up to 3 mm in diameter were included in one and those over the size of 3 mm in another. Among the 168 cases, the gastric ulcer was found in 12 and duodenal ulcer in 27 patients. The sex ratio of men to women was 1.4:1 found in the former and 8:1 in the latter. Among other findings, a knuckle of duodenal mucoa was noticed prolapsing through the large pyloric aperture. It could be postulated that a knuckle of the mucosa that keeps peeping through the pylorus acts as a mucosal plug in empty stomach, like a cork in the acid bottle. The main physiological function is to protect the mucosa from being damaged by the acid-pepsin injury or by the reflux of bile, but the tip of the plug seems to be subjected to such injury. Furthermore, the surface epithelial cells could also be subjected to ischaemic change while prolapsing through the pylorus. This may lead to reduced production of the mucosal gel and bicarbonate secretion, thus exposing the damaged mucosa to acid bath. This supports the concept, how a small area of the stomach or duodenum could be inflicted with ulceration.

  12. Toxaemia secondary to pyloric foreign body obstruction in two African lion(Panther leo) cubs

    Institute of Scientific and Technical Information of China (English)

    David; Squarre; John; Yabe; Chisoni; Mumba; Maxwel; Mwase; Katendi; Changula; Wizaso; Mwasinga; Musso; Munyeme

    2015-01-01

    A case of toxaemia secondary to pyloric foreign body obstruction in two four-month-old African lion cubs were presented in this article. The lion cubs were presented to the school of veterinary medicine with a complaint of weight loss and stunted growth despite having a normal appetite and seizures. Defi nitive diagnosis was made based on gross pathology after attempting various symptomatic treatments. This article therefore is meant to discourage the use of blankets as bedding in holding enclosures for warmth and comfort post-weaning in captive lion cubs and indeed wild cats in general as they tend to eat bedding that has been soiled with food.

  13. Toxaemia secondary to pyloric foreign body obstruction in two African lion (Panthera leo) cubs

    Institute of Scientific and Technical Information of China (English)

    David Squarre; John Yabe; Chisoni Mumba; Maxwel Mwase; Katendi Changula; Wizaso Mwasinga; Musso Munyeme

    2015-01-01

    A case of toxaemia secondary to pyloric foreign body obstruction in two four-month-old African lion cubs were presented in this article. The lion cubs were presented to the school of veterinary medicine with a complaint of weight loss and stunted growth despite having a normal appetite and seizures. Definitive diagnosis was made based on gross pathology after attempting various symptomatic treatments. This article therefore is meant to discourage the use of blankets as bedding in holding enclosures for warmth and comfort post-weaning in captive lion cubs and indeed wild cats in general as they tend to eat bedding that has been soiled with food.

  14. Spinal canal stenosis; Spinalkanalstenose

    Energy Technology Data Exchange (ETDEWEB)

    Papanagiotou, P.; Boutchakova, M. [Klinikum Bremen-Mitte/Bremen-Ost, Klinik fuer Diagnostische und Interventionelle Neuroradiologie, Bremen (Germany)

    2014-11-15

    Spinal stenosis is a narrowing of the spinal canal by a combination of bone and soft tissues, which can lead to mechanical compression of spinal nerve roots or the dural sac. The lumbal spinal compression of these nerve roots can be symptomatic, resulting in weakness, reflex alterations, gait disturbances, bowel or bladder dysfunction, motor and sensory changes, radicular pain or atypical leg pain and neurogenic claudication. The anatomical presence of spinal canal stenosis is confirmed radiologically with computerized tomography, myelography or magnetic resonance imaging and play a decisive role in optimal patient-oriented therapy decision-making. (orig.) [German] Die Spinalkanalstenose ist eine umschriebene, knoechern-ligamentaer bedingte Einengung des Spinalkanals, die zur Kompression der Nervenwurzeln oder des Duralsacks fuehren kann. Die lumbale Spinalkanalstenose manifestiert sich klinisch als Komplex aus Rueckenschmerzen sowie sensiblen und motorischen neurologischen Ausfaellen, die in der Regel belastungsabhaengig sind (Claudicatio spinalis). Die bildgebende Diagnostik mittels Magnetresonanztomographie, Computertomographie und Myelographie spielt eine entscheidende Rolle bei der optimalen patientenbezogenen Therapieentscheidung. (orig.)

  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. Hypertrophic pachymeningitis accompanying neuromyelitis optica spectrum disorder: A case report.

    Science.gov (United States)

    Kon, Tomoya; Nishijima, Haruo; Haga, Rie; Funamizu, Yukihisa; Ueno, Tatsuya; Arai, Akira; Suzuki, Chieko; Nunomura, Jin-ichi; Baba, Masayuki; Takahashi, Toshiyuki; Tomiyama, Masahiko

    2015-10-15

    We report a case of idiopathic cerebral hypertrophic pachymeningitis accompanying neuromyelitis optica spectrum disorder. No other identifiable cause of pachymeningitis was detected. Corticosteroid therapy was effective for both diseases. Hypertrophic pachymeningitis is closely related to autoimmune inflammatory disease of the central nervous system. This case supports the hypothesis that hypertrophic pachymeningitis can be a rare comorbidity of neuromyelitis optica spectrum disorder.

  17. Multimodality imaging in apical hypertrophic cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

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

    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 firstline 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.

  18. Gastric inverted hyperplasic polyp composed only of pyloric glands: a rare case report and review of the literature.

    Science.gov (United States)

    Jung, Minsun; Min, Kyueng-Whan; Ryu, Young-Joon

    2015-06-01

    Inverted hyperplastic polyp (IHP) in stomach is a rare benign gastric polypoid lesion, characterized by downward growth of hyperplastic mucosal glands into the submucosal layer. In most previous reported cases, gastric IHP showed mixtures of fundic-type gland, pyloric-type gland, and foveolar-type epithelium. Also, a case of IHP composed of only one type of gland is extremely rare. This report describes a case of a 70-year-old man with gastric IHP, composed only of pyloric-type gland. It was removed completely by endoscopic submucosal dissection, and patient showed no recurrence over 2 years after treatment.

  19. Gastroduodenal ulcer treated by pylorus and pyloric vagus-preser-ving gastrectomy

    Institute of Scientific and Technical Information of China (English)

    1999-01-01

    AIM To evaluate the curative effect of pylorus and pyloric vagus-preserving gastrectomy (PPVPG) on peptic ulcer.METHODS Treating 132 cases of GU and DU with PPVPG, and comparative studies made with 24 cases treated with Billroth Ⅰ (BⅠ) and 20 cases with Billroth Ⅱ (BⅡ); advantages and shortcomings evaluated.RESULTS Not a single death after PPVPG. No recurrence of the disorder in the subsequent follow-up for an average of 6.5 years. Curative effect (visik Ⅰ & Ⅱ) 97.7%. Acidity reduction similar to that found in BⅠ and BⅡ, but 97.7% of the BⅠ and all BⅡ cases having more than second degree intestinal fluid reflux, in contrast to 7.1% in PPVPG cases. Dumping syndrome occurred in the BⅠ and BⅡ cases, none in PPVPG cases. With regard to gastric emptying, food digestion, absorption, body weight and life quality, PPVPG proved to be superior to Billroth procedure.CONCLUSION PPVPG has the advantages of conventional Billroth gastrectomy in reducing acid, removing ulcer focus, and at the same time preserves the pylorus and pyloric vagus for maintaining the normal gastric physiological function. Dumping syndrome, intestinal fluid reflux and other complications of conventional gastrectomy may be avoided.

  20. Histological description of the midgut and the pyloric valve of Tropidacris collaris (Stoll, 1813 (Orthopetera: Romaleidae

    Directory of Open Access Journals (Sweden)

    V. Wanderley-Teixeira

    Full Text Available The present research describes the histology of the midgut, gastric caeca, and pyloric valve of Tropidacris collaris (Stoll, 1813, (Orthopetera: Romaleidae. We used light microscopy, staining (Gomori's trichrome and periodic acid-Schiff (PAS, and a routine histological analysis method (hematoxilin-eosin. The insects were obtained from, and also bred in, the Laboratory of Entomology, Department of Biology, of the Rural Federal University of Pernambuco (UFRPE. The collected material was fixed in alcoholic Boüin and embedded in paraplast. The results demonstrated that the midgut wall is composed of an inner epithelial layer and two outer layers of striate muscles: one internal (circular and the other external (longitudinal, with connective tissue between the muscle fibers. The epithelium is single-layered, with two cell types: regenerative and elongated columnar. The gastric caeca presents muscle layers similar to those of the midgut. Simple columnar epithelium lines the gastric caeca, which presents villi and projects towards the lumen. The pyloric valve is of striate muscle tissue, covered by a single epithelial-cell layer.

  1. SCREENING FOR ANTI ULCER ACTIVITY OF CONVOLVULUS PLURICAULIS USING PYLORIC LIGATION METHOD IN WISTER RATS.

    Directory of Open Access Journals (Sweden)

    Sharath S S

    2015-01-01

    Full Text Available Peptic ulcer disease is a chronic problem of the gastrointestinal tract characterized by mucosal damage secondary to pepsin and gastric acid secretion. Herbal medicines are generally used in cases when drugs are to be used for chronic periods. The study was conducted to evaluate antiulcerogenic activity of convolvulus pluricaulis in wistar rats. The antiulcer activity was carried out employing - Pyloric ligation method. The rats were randomly divided into 4 groups of 6 animals each. First group was given 1ml of 0.1% of vehicle (Tween 80; the second group was treated with ranitidine 50 mg/kg in 1 ml Tween 80. The remaining groups receive 200 mg/kg and 400 mg/kg of Convolvulus pluricaulis alcoholic extract (CPAE in 1 ml Tween 80 respectively. All the drugs were administered orally for five days duration daily in two divided doses. Evaluation of antiulcer activity was done by Ulcer score/ulcer index. Volume of gastric juice secreted, gastric free acidity, total acidity, total protein, pepsin mucin and pH were also estimated. CPAE at a dose of 200 mg/kg inhibited ulcer index by 40.87% and at a dose of 400 mg/kg inhibited ulcer index by 26.64 %. Alcoholic extracts of Convolvulus pluricaulis have shown a significant protection against gastric ulcers in pyloric ligation model.

  2. Aortic biomechanics in hypertrophic cardiomyopathy

    Science.gov (United States)

    Badran, Hala Mahfouz; Soltan, Ghada; Faheem, Nagla; Elnoamany, Mohamed Fahmy; Tawfik, Mohamed; Yacoub, Magdi

    2015-01-01

    Background: Ventricular-vascular coupling is an important phenomenon in many cardiovascular diseases. The association between aortic mechanical dysfunction and left ventricular (LV) dysfunction is well characterized in many disease entities, but no data are available on how these changes are related in hypertrophic cardiomyopathy (HCM). Aim of the work: This study examined whether HCM alone is associated with an impaired aortic mechanical function in patients without cardiovascular risk factors and the relation of these changes, if any, to LV deformation and cardiac phenotype. Methods: 141 patients with HCM were recruited and compared to 66 age- and sex-matched healthy subjects as control group. Pulse pressure, aortic strain, stiffness and distensibility were calculated from the aortic diameters measured by M-mode echocardiography and blood pressure obtained by sphygmomanometer. Aortic wall systolic and diastolic velocities were measured using pulsed wave Doppler tissue imaging (DTI). Cardiac assessment included geometric parameters and myocardial deformation (strain and strain rate) and mechanical dyssynchrony. Results: The pulsatile change in the aortic diameter, distensibility and aortic wall systolic velocity (AWS') were significantly decreased and aortic stiffness index was increased in HCM compared to control (P < .001) In HCM AWS' was inversely correlated to age(r = − .32, P < .0001), MWT (r = − .22, P < .008), LVMI (r = − .20, P < .02), E/Ea (r = − .16, P < .03) LVOT gradient (r = − 19, P < .02) and severity of mitral regurg (r = − .18, P < .03) but not to the concealed LV deformation abnormalities or mechanical dyssynchrony. On multivariate analysis, the key determinant of aortic stiffness was LV mass index and LVOT obstruction while the role LV dysfunction in aortic stiffness is not evident in this population. Conclusion: HCM is associated with abnormal aortic mechanical properties. The severity of cardiac

  3. "Vanishing" pulmonary valve stenosis

    Directory of Open Access Journals (Sweden)

    Nofil I Arain

    2012-01-01

    Full Text Available Objective: Both spontaneous resolution and progression of mild pulmonary valve stenosis (PS have been reported. We reviewed characteristics of the pulmonary valve (PV to determine factors that could influence resolution of mild PS. Methods: Fifteen asymptomatic pediatric patients with spontaneous resolution of isolated mild PS were retrospectively reviewed. Results: There was no correlation between the PV gradient, clinical presentation, age at diagnosis, or PV morphology. The PV annulus was small at initial presentation, which normalized at follow up. When corrected for the body surface area (z-score, the PV annulus was normal in all patients, including at initial evaluation. Conclusions: Based on our observation, neither age at diagnosis, nor PV-morphology-influenced resolution of mild PS. The variable clinical presentation makes it difficult to categorize and observe mild PS by auscultation alone. The PV annulus z-score could be a useful adjunct to determine the course and serial observation of mild PS.

  4. Transcriptional regulation of cardiac genes balance pro- and anti-hypertrophic mechanisms in hypertrophic cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Nina Gennebäck

    2012-06-01

    Full Text Available Hypertrophic cardiomyopathy (HCM is characterized by unexplained left ventricular hypertrophy. HCM is often hereditary, but our knowledge of the mechanisms leading from mutation to phenotype is incomplete. The transcriptional expression patterns in the myocar - dium of HCM patients may contribute to understanding the mechanisms that drive and stabilize the hypertrophy. Cardiac myectomies/biopsies from 8 patients with hypertrophic obstructive cardiomyopathy (HOCM and 5 controls were studied with whole genome Illumina microarray gene expression (detecting 18 189 mRNA. When comparing HOCM myocardium to controls, there was significant transcriptional down-regulation of the MYH6, EGR1, APOB and FOS genes, and significant transcriptional up-regulation of the ACE2, JAK2, NPPA (ANP, APOA1 and HDAC5 genes. The transcriptional regulation revealed both pro- and anti-hypertrophic mechanisms. The pro-hypertrophic response was explained by the transcriptional down-regulation of MYH6, indicating that the switch to the fetal gene program is maintained, and the transcriptional up-regulation of JAK2 in the JAK-STAT pathway. The anti-hypertrophic response was seen as a transcriptional down-regulation of the immediate early genes (IEGs, FOS and EGR1, and a transcriptional up-regulation of ACE2 and HDAC5. This can be interpreted as a transcriptional endogenous protection system in the heart of the HOCM patients, neither growing nor suppressing the already hypertrophic myocardium.

  5. 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...

  6. 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...

  7. 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

  8. Prevalence of hypertrophic cardiomyopathy in China

    Institute of Scientific and Technical Information of China (English)

    Tsung O Cheng

    2004-01-01

    @@ To the Editor: I read with interest the recent article on cardiac troponin T (TNNT2) mutations in Chinese patients with hypertrophic cardiomyopathy (HCM) by Wu et al.1 The authors cited a prevalence of HCM of 0.2% in general population, but did not indicate whether it referred to the general population in China or some other countries.

  9. Purification and characterization of two chymotrypsin-like proteases from the pyloric caeca of rainbow trout oncorhynchus-mykiss

    DEFF Research Database (Denmark)

    Kristjansson, Magnus M.; Nielsen, Henrik Hauch

    1992-01-01

    Two chymotrypsins, called chymotrypsin I and II, were purified from the pyloric caeca of rainbow trout, by (NH4)2SO4 fractionation, hydrophobic interaction chromatography (phenyl-Sepharose) and ion-exchange chromatography (DEAE-Sepharose). The approximate molecular weights of chymotrypsin I and I...

  10. Amino acids and carbohydrates absorption by Na+-dependent transporters in the pyloric ceca of Hoplias malabaricus (Erythrinidae

    Directory of Open Access Journals (Sweden)

    Vieira Vania Lucia Pimentel

    2001-01-01

    Full Text Available Information about amino acids and carbohydrate absorption in fish is important to formulate an adequate diet to obtain optimal growth. Therefore, the objective of this study was to investigate if Na+-dependent transporters are involved on the absorption of glycine, L-glutamine, L-leucine, L-lysine, L-proline, L-alanine, and the carbohydrates fructose and glucose in the pyloric ceca of Hoplias malabaricus. The pyloric ceca were mounted in a system of continuous perfusion "in vitro". Amino acids and carbohydrates were placed on the mucosal side at concentrations of 10, 20, and 40mM. The serosal side of the pyloric ceca was positive in relation to the mucosal side. The addition of glycine, L-glutamine, L-leucine, L-lysine, L-proline (all tested concentrations, and glucose (at concentrations of 20 and 40mM increased the positivity of the serosal side, indicating the presence of Na+-dependent transporters in the absorption of these substances. L-alanine and fructose did not change the positivity of the serosal side. The pyloric ceca seem to be the main site of nutrient absorption in the digestive tract of H. malabaricus.

  11. Degenerative lumbosacral stenosis in dogs

    NARCIS (Netherlands)

    Suwankong, N.

    2007-01-01

    Degenerative lumbosacral stenosis (DLS) is now recognized as a significant cause of caudal lumbar pain and pelvic limb lameness in dogs. The condition includes lumbosacral intervertebral disc degeneration and protrusion, spondylosis deformans, sclerosis of the vertebral end plates, osteoarthrosis of

  12. Interstitial cells of Cajal in human gut and gastrointestinal disease

    DEFF Research Database (Denmark)

    Vanderwinden, J M; Rumessen, J J

    1999-01-01

    of their functional significance. Alterations of ICC reported in achalasia of cardia, infantile hypertrophic pyloric stenosis, chronic intestinal pseudoobstruction, Hirschsprung's disease, inflammatory bowel diseases, slow transit constipation, and some other disorders of GI motility as well as in gastrointestinal...... stromal tumors are reviewed, with emphasis on the place of ICC in the pathophysiology of disease....

  13. Disturbed development of the enteric nervous system after in utero exposure of selective serotonin re-uptake inhibitors and tricyclic antidepressants. Part 1 : Literature review

    NARCIS (Netherlands)

    Nijenhuis, C.M.; Ter Horst, P.G.; de Jong-van den Berg, L.T.; Wilffert, B.

    2012-01-01

    The increase in selective serotonin re-uptake inhibitor (SSRI) use during pregnancy, questions concerning abnormal development of the enteric nervous system (ENS), increase in laxative use in children and the association of fluoxetine with infantile hypertrophic pyloric stenosis (IHPS) gave rise to

  14. Cervical stenosis following electrosurgical conization

    Directory of Open Access Journals (Sweden)

    Aparecida Cristina Sampaio Monteiro

    Full Text Available CONTEXT AND OBJECTIVE: Cervical stenosis is a postoperative complication of procedures for treating preinvasive lesions of the cervix and takes on particular importance due to the clinical repercussions associated with it. Furthermore, it causes limitations in relation to cytological and colposcopic follow-up. The aim here was to assess the incidence of cervical stenosis among a cohort of patients who underwent electrosurgical conization and to identify possible prognostic factors associated with its occurrence. DESIGN AND SETTING: Retrospective study at Gynecology and Obstetrics Department, Instituto Fernandes Figueira, Rio de Janeiro. METHODS:This was an observational study among a cohort of patients who underwent electrosurgical conization of the uterine cervix. The possible predictive variables were analyzed as bivariate means between the groups with and without stenosis. We also calculated the incidence density rate ratio for cervical stenosis in relation to each possible predictive variable and the respective confidence intervals (95%. Levels of 5% were considered significant. RESULTS: 274 patients who underwent electrosurgical conization of the uterine cervix with a minimum follow-up period of six months were included. The crude incidence of cervical stenosis was 7.66% and the incidence density was 3.3/1,000 patients-month. CONCLUSIONS: We did not find associations between the variables for stenosis. However, we observed borderline significance levels relating to hemorrhagic complications before and after the operation (p = 0.089.

  15. Fluoxetine and infantile hypertrophic pylorus stenosis : a signal from a birth defects drug exposure surveillance study

    NARCIS (Netherlands)

    Bakker, M.K.; de Walle, H.E.K.; Wilffert, B.; de Jong-van den Berg, L.T.W.

    2010-01-01

    Purpose We report an association found in a surveillance study which systematically evaluated combinations of specific birth defects and drugs used in the first trimester of pregnancy. Method The database of a population-based birth defects registry (birth years 1997-2007) was systematically screene

  16. Gastric serosal tear due to congenital pyloric atresia: A rare anomaly, a rare complication

    Directory of Open Access Journals (Sweden)

    Mithat Gunaydin

    2011-01-01

    Full Text Available Congenital pyloric atresia (CPA is a very rare malformation with unknown aetiology. It has has numerous complications including gastric perforation, aspiration pneumonia. Gastric perforations in newborns occur by three mechanisms: trauma, ischaemia, or spontaneous. Here, we report a newborn with CPA presenting with gastric serosal tearing without full-cut gastric perforation. The diagnosis was confirmed with the help of plain abdominal radiograph, ultrasound, contrast-study, and at operation. Treatment of CPA is surgery irrespective of the type of atresia. We performed serosa repair and then the solid, cord-like atretic pylorus was excised with accompanying gastroduodenostomy. Our patient had an uneventful course and was discharged at the end of the second postoperative week.

  17. Hypoxia-driven glycolytic and fructolytic metabolic programs: Pivotal to hypertrophic heart disease.

    Science.gov (United States)

    Mirtschink, Peter; Krek, Wilhelm

    2016-07-01

    Pathologic cardiac growth is an adaptive response of the myocardium to various forms of systemic (e.g. pressure overload) or genetically-based (e. g. mutations in genes encoding sarcomeric proteins) stress. It represents a key aspect of different types of heart disease including aortic stenosis (AS) and hypertrophic cardiomyopathy (HCM). While many of the pathophysiological and hemodynamical aspects of pathologic cardiac hypertrophy have been uncovered during the last decades, its underlying metabolic determinants are only beginning to come into focus. Here, we review the epidemiological evidence and pathological features of hypertrophic heart disease in AS and HCM and consider in this context the development of microenvironmental tissue hypoxia as a key component of the heart's growth response to pathologic stress. We particularly reflect on recent evidence illustrating how activation of hypoxia-inducible factor (HIF) drives glycolytic and fructolytic metabolic programs to maintain ATP generation and support anabolic growth of the pathologically-stressed heart. Finally we discuss how this metabolic programs, when protracted, deprive the heart of energy leading ultimately to heart failure. This article is part of a Special Issue entitled: Cardiomyocyte Biology: Integration of Developmental and Environmental Cues in the Heart edited by Marcus Schaub and Hughes Abriel.

  18. Left ventricular remodeling and hypertrophy in patients with aortic stenosis: insights from cardiovascular magnetic resonance

    Directory of Open Access Journals (Sweden)

    Dweck Marc R

    2012-07-01

    Full Text Available Abstract Background Cardiovascular magnetic resonance (CMR is the gold standard non-invasive method for determining left ventricular (LV mass and volume but has not been used previously to characterise the LV remodeling response in aortic stenosis. We sought to investigate the degree and patterns of hypertrophy in aortic stenosis using CMR. Methods Patients with moderate or severe aortic stenosis, normal coronary arteries and no other significant valve lesions or cardiomyopathy were scanned by CMR with valve severity assessed by planimetry and velocity mapping. The extent and patterns of hypertrophy were investigated using measurements of the LV mass index, indexed LV volumes and the LV mass/volume ratio. Asymmetric forms of remodeling and hypertrophy were defined by a regional wall thickening ≥13 mm and >1.5-fold the thickness of the opposing myocardial segment. Results Ninety-one patients (61±21 years; 57 male with aortic stenosis (aortic valve area 0.93±0.32cm2 were recruited. The severity of aortic stenosis was unrelated to the degree (r2=0.012, P=0.43 and pattern (P=0.22 of hypertrophy. By univariate analysis, only male sex demonstrated an association with LV mass index (P=0.02. Six patterns of LV adaption were observed: normal ventricular geometry (n=11, concentric remodeling (n=11, asymmetric remodeling (n=11, concentric hypertrophy (n=34, asymmetric hypertrophy (n=14 and LV decompensation (n=10. Asymmetric patterns displayed considerable overlap in appearances (wall thickness 17±2mm with hypertrophic cardiomyopathy. Conclusions We have demonstrated that in patients with moderate and severe aortic stenosis, the pattern of LV adaption and degree of hypertrophy do not closely correlate with the severity of valve narrowing and that asymmetric patterns of wall thickening are common. Trial registration ClinicalTrials.gov Reference Number: NCT00930735

  19. Idiopathic chronic hypertrophic craniocervical pachymeningitis: case report.

    Science.gov (United States)

    Botella, C; Orozco, M; Navarro, J; Riesgo, P

    1994-12-01

    A 55-year-old woman with a unique form of chronic hypertrophic pachymeningitis involving the posterior fossa and upper cervical spine is reported. Unlike other cases previously described, the clinical picture was dominated by signs of increased intracranial pressure, lower cranial nerve disorders, and a progressive cervical radiculomyelopathy. The diagnosis was made by means of a contrast-enhanced magnetic resonance imaging scan and confirmed by histological examination of the excised dura. Surgical treatment with removal of the hypertrophic dura provided temporary relief, although the natural history of the disease was not modified. Exhaustive bacteriological and histopathological studies failed to identify a specific cause for this diffuse hypertrophy of the cranial and cervical dura. The literature is reviewed, and other histologically documented cases are discussed.

  20. 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.)

  1. [Subglottic stenosis and gastroesophageal reflux].

    Science.gov (United States)

    Fligny, I; François, M; Aigrain, Y; Polonovski, J M; Contencin, P; Narcy, P

    1989-01-01

    The authors report the cases of ten children treated for sub-glottic laryngeal stenosis, in the Department of Pr. Narcy in Hospital Robert-Debre. Medical treatment of the laryngeal stenosis had failed in these cases. Treatment, most often surgical, of the gastro-oesophageal reflux present in these ten cases enabled these children to be cured. A review of the literature stresses the role and responsibility of gastro-oesophageal reflux in laryngeal pathology. Based on their experience, the authors suggest: systematic investigation for gastro-oesophageal reflux during management of laryngeal stenosis, especially when laryngeal inflammation is encountered; the adoption of an interventionist attitude vis-a-vis gastro-oesophageal reflux which would seem to have an important pathogenic role in certain laryngeal stenoses.

  2. Hypertrophic Cardiomyopathy Registry: The rationale and design of an international, observational study of hypertrophic cardiomyopathy.

    Science.gov (United States)

    Kramer, Christopher M; Appelbaum, Evan; Desai, Milind Y; Desvigne-Nickens, Patrice; DiMarco, John P; Friedrich, Matthias G; Geller, Nancy; Heckler, Sarahfaye; Ho, Carolyn Y; Jerosch-Herold, Michael; Ivey, Elizabeth A; Keleti, Julianna; Kim, Dong-Yun; Kolm, Paul; Kwong, Raymond Y; Maron, Martin S; Schulz-Menger, Jeanette; Piechnik, Stefan; Watkins, Hugh; Weintraub, William S; Wu, Pan; Neubauer, Stefan

    2015-08-01

    Hypertrophic cardiomyopathy (HCM) is the most common monogenic heart disease with a frequency as high as 1 in 200. In many cases, HCM is caused by mutations in genes encoding the different components of the sarcomere apparatus. Hypertrophic cardiomyopathy is characterized by unexplained left ventricular hypertrophy, myofibrillar disarray, and myocardial fibrosis. The phenotypic expression is quite variable. Although most patients with HCM are asymptomatic, serious consequences are experienced in a subset of affected individuals who present initially with sudden cardiac death or progress to refractory heart failure. The Hypertrophic Cardiomyopathy Registry study is a National Heart, Lung, and Blood Institute-sponsored 2,750-patient, 44-site, international registry and natural history study designed to address limitations in extant evidence to improve prognostication in HCM (NCT01915615). In addition to the collection of standard demographic, clinical, and echocardiographic variables, patients will undergo state-of-the-art cardiac magnetic resonance for assessment of left ventricular mass and volumes as well as replacement scarring and interstitial fibrosis. In addition, genetic and biomarker analyses will be performed. The Hypertrophic Cardiomyopathy Registry has the potential to change the paradigm of risk stratification in HCM, using novel markers to identify those at higher risk.

  3. G-cell hyperplasia of the stomach induces ECL-cell proliferation in the pyloric glands in a paracrinal manner.

    Science.gov (United States)

    Kasajima, Atsuko; Fujishima, Fumiyoshi; Morikawa, Takanori; Kawasaki, Shuhei; Konosu-Fukaya, Sachiko; Shibahara, Yukiko; Nakamura, Tadaho; Yoshikawa, Takeo; Iijima, Katsunori; Koike, Tomoyuki; Watanabe, Mika; Shibata, Chikashi; Sasano, Hironobu

    2015-05-01

    An inhibitory mechanism toward gastrin hypersecretion is significantly different between G-cell hyperplasia and gastrinoma despite the common clinical manifestations; hypergastrinemia and its related persistent gastric ulcers. We recenlty studied the G-cell, d-cell and ECL-cell density in a case of G-cell hyperplasia. The 70-year-old patient has been treated for persistent gastric ulcers with a markedly increased plasma gastrin (5600 pg/mL). The stomach was surgically resected because of the obstruction associated with ulcer scars. The number of G-cells in the pyloric glands was quantified on the surgical specimens and G-cell hyperplasia was histolopathologically identified. Immunostainig of histidine decarboxylate revealed the presence of ECL-cell hyperplasia in the pyloric glands and its density was significantly and positively correlated with G-cell density. Somatostatin immunoreactive cells (D-cells) increased in their number in the oxyntic glands. These results all indicated that hypersecretion of gastrin in G-cell hyperplasia could induce ECL-cell proliferation in a paracrinal manner. In addition, relatively non-prominent endocrinological features in the G-cell hyperplasia compared to gastrinoma could be also related to the paracrinal somatostatin inhibitory effects upon ECL-cells in the pyloric glands.

  4. Post-pyloric versus gastric tube feeding for preventing pneumonia and improving nutritional outcomes in critically ill adults.

    Science.gov (United States)

    Alkhawaja, Sana; Martin, Claudio; Butler, Ronald J; Gwadry-Sridhar, Femida

    2015-08-04

    Nutritional support is an essential component of critical care. Malnutrition has been associated with poor outcomes among patients in intensive care units (ICUs). Evidence suggests that in patients with a functional gut, nutrition should be administered through the enteral route. One of the main concerns regarding use of the enteral route is the reduction in gastric motility that is often responsible for limited caloric intake. This increases the risk of aspiration pneumonia as well. Post-pyloric feeding, in which the feed is delivered directly into the duodenum or the jejunum, could solve these issues and provide additional benefits over routine gastric administration of the feed. To evaluate the effectiveness and safety of post-pyloric feeding versus gastric feeding for critically ill adults who require enteral tube feeding. We searched the following databases: Cochrane Central Register of Controlled Trials (CENTRAL;2013 Issue 10), MEDLINE (Ovid) (1950 to October 2013), EMBASE (Ovid) (1980 to October 2013) and the Cumulative Index to Nursing and Allied Health Literature (CINAHL) via EBSCO host (1982 to October 2013). We reran the search on 4 February 2015 and will deal with the one study of interest when we update the review. Randomized or quasi-randomized controlled trials comparing post-pyloric versus gastric tube feeding in critically ill adults. We extracted data using the standard methods of the Cochrane Anaesthesia, Critical and Emergency Care Group and separately evaluated trial quality and data extraction as performed by each review author. We contacted trials authors to request missing data. We pooled data from 14 trials of 1109 participants in a meta-analysis. Moderate quality evidence suggests that post-pyloric feeding is associated with low rates of pneumonia compared with gastric tube feeding (risk ratio (RR) 0.65, 95% confidence interval (CI) 0.51 to 0.84). Low-quality evidence shows an increase in the percentage of total nutrient delivered to the

  5. Screening for Carotid Artery Stenosis

    Science.gov (United States)

    ... including stroke, heart attack or death. In addition, screening all adults will lead to many false-positive results because ... Force reviewed. 1 The Task Force recommends against screening for asymptomatic carotid artery stenosis in the general adult population . Grade D Notes 1 screening Conducting an ...

  6. 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.

  7. Periostin induces fibroblast proliferation and myofibroblast persistence in hypertrophic scarring.

    Science.gov (United States)

    Crawford, Justin; Nygard, Karen; Gan, Bing Siang; O'Gorman, David Brian

    2015-02-01

    Hypertrophic scarring is characterized by the excessive development and persistence of myofibroblasts. These cells contract the surrounding extracellular matrix resulting in the increased tissue density characteristic of scar tissue. Periostin is a matricellular protein that is abnormally abundant in fibrotic dermis, however, its roles in hypertrophic scarring are largely unknown. In this report, we assessed the ability of matrix-associated periostin to promote the proliferation and myofibroblast differentiation of dermal fibroblasts isolated from the dermis of hypertrophic scars or healthy skin. Supplementation of a thin type-I collagen cell culture substrate with recombinant periostin induced a significant increase in the proliferation of hypertrophic scar fibroblasts but not normal dermal fibroblasts. Periostin induced significant increases in supermature focal adhesion formation, α smooth muscle actin levels and collagen contraction in fibroblasts cultured from hypertrophic scars under conditions of increased matrix tension in three-dimensional type-I collagen lattices. Inhibition of Rho-associated protein kinase activity significantly attenuated the effects of matrix-associated periostin on hypertrophic scar fibroblasts and myofibroblasts. Depletion of endogenous periostin expression in hypertrophic scar myofibroblasts resulted in a sustained decrease in α smooth muscle actin levels under conditions of reducing matrix tension, while matrix-associated periostin levels caused the cells to retain high levels of a smooth muscle actin under these conditions. These findings indicate that periostin promotes Rho-associated protein kinase-dependent proliferation and myofibroblast persistence of hypertrophic scar fibroblasts and implicate periostin as a potential therapeutic target to enhance the resolution of scars.

  8. 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 prope

  9. Twice malignant transformation of hypertrophic lichen planus.

    Science.gov (United States)

    Krasowska, Dorota; Kozłowicz, Katarzyna; Kowal, Małgorzata; Kurylcio, Andrzej; Budzyńska-Włodarczyk, Jolanta; Polkowski, Wojciech; Chodorowska, Grażyna

    2012-01-01

    Lichen planus is a chronic mucocutaneous T-cell-mediated disease, the cause of which remains unknown. The first case of lichen planus that transformed into squamous cell carcinoma was reported in 1903. The presented study concerns the case of a 62-year-old woman in whom twice malignant transformation of hypertrophic lichen planus in the dorsal part of the left foot developed. Several studies have pointed out the malignant transformation potential of lichen planus. Epidemiological studies from the last 20 years have revealed a malignant transformation rate of 0.27% per year, emphasizing the importance of the clinical follow-up of lichen planus patients.

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

    Directory of Open Access Journals (Sweden)

    Jeroen C H Leijten

    Full Text Available 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 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. MATERIALS AND METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

  11. Mechanisms underlying burst generation of the pyloric muscle in the mantis shrimp, Squilla oratoria.

    Science.gov (United States)

    Tazaki, K; Chiba, C

    1991-12-01

    The pyloric constrictor muscles of the stomach in Squilla can generate spikes by synaptic activation via the motor nerve from the stomatogastric ganglion. Spikes are followed by slow depolarizing afterpotentials (DAPs) which lead to sustained depolarization during a burst of spikes. 1. The frequency of rhythmic bursts induced by continuous depolarization is membrane voltage-dependent. A brief depolarizing or hyperpolarizing pulse can trigger or terminate bursts, respectively, in a threshold-dependent manner. 2. The conductance increases during the DAP response. The amplitude of DAP decreases by imposed depolarization, whereas it increases by hyperpolarization. DAPs from successive spikes sum to produce a sustained depolarizing potential capable of firing a burst. 3. The spike and DAP are reduced in amplitude by decreasing [Ca]o, enhanced by Sr2+ or Ba2+ substituted for Ca2+, and blocked by Co2+ or Mn2+. DAPs are selectively blocked by Ni2+, and the spike is followed by a hyperpolarizing afterpotential. 4. The spike and DAP are prolonged by intracellular injection of the Ca2+ chelator EGTA. A hyperpolarizing afterpotential is abolished by EGTA and enhanced by increasing [Ca]o. The DAP is diminished in Na(+)-free saline and reduced by tetrodotoxin. 5. It is concluded that the muscle fiber is endowed with endogenous oscillatory properties and that the oscillatory membrane events result from changes of a voltage- and time-dependent conductance to Ca2+ and Na+ and a Ca2+ activated conductance to K+.

  12. 小儿幽门管溃疡36例%Pyloric Canal Ulcer in 36 Children

    Institute of Scientific and Technical Information of China (English)

    陈昌斌; 游洁玉; 陈志勇; 欧阳文献; 罗艳红; 赵红梅; 段柏萍

    2011-01-01

    目的 探讨小儿幽门管溃疡的临床特点、诊断及治疗方法.方法 回顾性分析36例(男22例,女14例;年龄6个月~12岁)幽门管溃疡的的临床表现、胃镜和钡餐检查特点及其治疗和预后.结果 以呕吐为首发表现的患儿33例(占91.7%),伴食欲减退30例,腹痛20例,黑便、消瘦各12例,面色苍黄、腹上区包块各10例;合并不同程度贫血29例,代谢性碱中毒27例,低钠、低钾、低氯血症各18例.胃镜首次检查能通过幽门并发现幽门管溃疡9例,并幽门梗阻且胃镜前端不能通过者27例;对胃镜不能通过者行稀钡餐检查,结果显示幽门管溃疡6例,幽门不完全性梗阻21例.23例(63.9%)经内科保守治疗治愈,13例(36.1%)经手术治疗治愈.结论 小儿幽门管溃疡的首发表现为呕吐,胃镜和钡餐检查是确诊幽门管溃疡的主要手段.治疗方面以内科保守治疗为主,幽门管溃疡由于其解剖位置特异,其抑酸治疗的疗程适当延长,主张以4~6周为1个疗程抑酸治疗,患儿不易复发;对少数难以控制的大出血者、幽门完全性梗阻经保守治疗72 h无效者及易复发的多发性幽门管溃疡者应选择手术治疗.%Objective To explore the clinical features,diagnosis and treatment of children with pyloric canal ulcer.Methods Medical records of the clinical performance, the characteristics of electronic endoscopy and barium meal examination of 36 hospitalized cases ( with the pyloric canal ulcer) including 22 male and 14 female,aged 6 months to 12 years old and its treatment and prognosis were analyzed retrospectively.Results Vomiting was the first symptom in 33 cases of the 36 children (91.7%) ,30 cases with loss of appetite,abdominal pain in 20 cases,melena in 12 cases,weight loss in 12 cases looking greenish yellow on the face in 10 cases;upper abdominal mass in 10 cases;different degrees of anemia in 29 cases, metabolic alkalosis in 27 cases,low sodium and low potassium and low

  13. Hypertrophic cardiomyopathy in owl monkeys (Aotus spp.).

    Science.gov (United States)

    Knowlen, Grant G; Weller, Richard E; Perry, Ruby L; Baer, Janet F; Gozalo, Alfonso S

    2013-06-01

    Cardiac hypertrophy is a common postmortem finding in owl monkeys. In most cases the animals do not exhibit clinical signs until the disease is advanced, making antemortem diagnosis of subclinical disease difficult and treatment unrewarding. We obtained echocardiograms, electrocardiograms, and thoracic radiographs from members of a colony of owl monkeys that previously was identified as showing a 40% incidence of gross myocardial hypertrophy at necropsy, to assess the usefulness of these modalities for antemortem diagnosis. No single modality was sufficiently sensitive and specific to detect all monkeys with cardiac hypertrophy. Electrocardiography was the least sensitive method for detecting owl monkeys with hypertrophic cardiomyopathy. Thoracic radiographs were more sensitive than was electrocardiography in this context but cannot detect animals with concentric hypertrophy without an enlarged cardiac silhouette. Echocardiography was the most sensitive method for identifying cardiac hypertrophy in owl monkeys. The most useful parameters suggestive of left ventricular hypertrophy in our owl monkeys were an increased average left ventricular wall thickness to chamber radius ratio and an increased calculated left ventricular myocardial mass. Parameters suggestive of dilative cardiomyopathy were an increased average left ventricular myocardial mass and a decreased average ratio of left ventricular free wall thickness to left ventricular chamber radius. When all 4 noninvasive diagnostic modalities (physical examination, echocardiography, electrocardiography, and thoracic radiography) were used concurrently, the probability of detecting hypertrophic cardiomyopathy in owl monkeys was increased greatly.

  14. Statins for aortic valve stenosis

    Directory of Open Access Journals (Sweden)

    Luciana Thiago

    Full Text Available ABSTRACT BACKGROUND: Aortic valve stenosis is the most common type of valvular heart disease in the USA and Europe. Aortic valve stenosis is considered similar to atherosclerotic disease. Some studies have evaluated statins for aortic valve stenosis. OBJECTIVES: To evaluate the effectiveness and safety of statins in aortic valve stenosis. METHODS: Search methods: We searched the Cochrane Central Register of Controlled Trials (CENTRAL, MEDLINE, Embase, LILACS - IBECS, Web of Science and CINAHL Plus. These databases were searched from their inception to 24 November 2015. We also searched trials in registers for ongoing trials. We used no language restrictions. Selection criteria: Randomized controlled clinical trials (RCTs comparing statins alone or in association with other systemic drugs to reduce cholesterol levels versus placebo or usual care. Data collection and analysis: Primary outcomes were severity of aortic valve stenosis (evaluated by echocardiographic criteria: mean pressure gradient, valve area and aortic jet velocity, freedom from valve replacement and death from cardiovascular cause. Secondary outcomes were hospitalization for any reason, overall mortality, adverse events and patient quality of life. Two review authors independently selected trials for inclusion, extracted data and assessed the risk of bias. The GRADE methodology was employed to assess the quality of result findings and the GRADE profiler (GRADEPRO was used to import data from Review Manager 5.3 to create a 'Summary of findings' table. MAIN RESULTS: We included four RCTs with 2360 participants comparing statins (1185 participants with placebo (1175 participants. We found low-quality evidence for our primary outcome of severity of aortic valve stenosis, evaluated by mean pressure gradient (mean difference (MD -0.54, 95% confidence interval (CI -1.88 to 0.80; participants = 1935; studies = 2, valve area (MD -0.07, 95% CI -0.28 to 0.14; participants = 127; studies = 2

  15. What is the prevalence of hypertrophic scarring following burns?

    Science.gov (United States)

    Bombaro, Kristine M; Engrav, Loren H; Carrougher, Gretchen J; Wiechman, Shelly A; Faucher, Lee; Costa, Beth A; Heimbach, David M; Rivara, Frederick P; Honari, Shari

    2003-06-01

    Hypertrophic scarring after burns remains a major problem and is considered to be "common". Pressure garments are commonly used as treatment even though there is little sound data that they reduce the prevalence or magnitude of the scarring. In 1999 we began a study of the efficacy of pressure garments on forearm burns. After studying 30 patients, mainly white adults, we found no hypertrophic scar in either those treated with pressure or without. This prompted us to review the literature on the prevalence of hypertrophic scarring after burns and found only four articles with a relatively small number of patients and only three geographical locations. It became clear that the prevalence of hypertrophic scarring is really unknown. We then did a retrospective study of 110 burn survivors and counted all hypertrophic scars of all sizes and locations in all races and found the prevalence hypertrophic scarring to be 67% which conflicts with the published reports and our prospective study and suggests that further research is necessary. We concluded that a worldwide, prospective survey is necessary to establish the prevalence of hypertrophic scarring after burns. In this article we are calling for and offering to organize this survey.

  16. 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.

  17. Posterolateral hypertrophic cardiomyopathy: a rare, but clinically significant variant of hypertrophic cardiomyopathy.

    Science.gov (United States)

    Seki, Atsuko; Perens, Gregory; Fishbein, Michael C

    2014-01-01

    Posterolateral hypertrophic cardiomyopathy (HCM) is a rare variant of HCM. Segmental HCM is seen in 12% of cases of HCM. Among the patterns of segmental HCM, posterolateral HCM is the least common type. Our case of an 18-year old male documents this unusual type of cardiomyopathy. In this form of HCM, left ventricular thickness and the extent of hypertrophy might be underestimated by 2-dimensional echocardiography. This case illustrates the echocardiographic and pathologic features of posterolateral HCM.

  18. Differential gene expression in the oxyntic and pyloric mucosa of the young pig.

    Science.gov (United States)

    Colombo, Michela; Priori, Davide; Trevisi, Paolo; Bosi, Paolo

    2014-01-01

    The stomach is often considered a single compartment, although morphological differences among specific areas are well known. Oxyntic mucosa (OXY) and pyloric mucosa (PYL, in other species called antral mucosa) are primarily equipped for acid secretion and gastrin production, respectively, while it is not yet clear how the remainder of genes expressed differs in these areas. Here, the differential gene expression between OXY and PYL mucosa was assessed in seven starter pigs. Total RNA expression was analyzed by whole genome Affymetrix Porcine Gene 1.1_ST array strips. Exploratory functional analysis of gene expression values was done by Gene Set Enrichment Analysis, comparing OXY and PYL. Normalized enrichment scores (NESs) were calculated for each gene (statistical significance defined when False Discovery Rate % <25 and P-values of NES<0.05). Expression values were selected for a set of 44 genes and the effect of point of gastric sample was tested by analysis of variance with the procedure for repeated measures. In OXY, HYDROGEN ION TRANSMEMBRANE TRANSPORTER ACTIVITY gene set was the most enriched set compared to PYL, including the two genes for H+/K+-ATPase. Pathways related to mitochondrial activity and feeding behavior were also enriched (primarily cholecystokinin receptors and ghrelin). Aquaporin 4 was the top-ranking gene. In PYL, two gene sets were enriched compared with OXY: LYMPHOCYTE ACTIVATION and LIPID RAFT, a gene set involved in cholesterol-rich microdomains of the plasma membrane. The single most differentially expressed genes were gastrin and secretoglobin 1A, member 1, presumably located in the epithelial line, to inactivate inflammatory mediators. Several genes related to mucosal integrity, immune response, detoxification and epithelium renewal were also enriched in PYL (P<0.05). The data indicate that there is significant differential gene expression between OXY of the young pig and PYL and further functional studies are needed to confirm their

  19. Differential gene expression in the oxyntic and pyloric mucosa of the young pig.

    Directory of Open Access Journals (Sweden)

    Michela Colombo

    Full Text Available The stomach is often considered a single compartment, although morphological differences among specific areas are well known. Oxyntic mucosa (OXY and pyloric mucosa (PYL, in other species called antral mucosa are primarily equipped for acid secretion and gastrin production, respectively, while it is not yet clear how the remainder of genes expressed differs in these areas. Here, the differential gene expression between OXY and PYL mucosa was assessed in seven starter pigs. Total RNA expression was analyzed by whole genome Affymetrix Porcine Gene 1.1_ST array strips. Exploratory functional analysis of gene expression values was done by Gene Set Enrichment Analysis, comparing OXY and PYL. Normalized enrichment scores (NESs were calculated for each gene (statistical significance defined when False Discovery Rate % <25 and P-values of NES<0.05. Expression values were selected for a set of 44 genes and the effect of point of gastric sample was tested by analysis of variance with the procedure for repeated measures. In OXY, HYDROGEN ION TRANSMEMBRANE TRANSPORTER ACTIVITY gene set was the most enriched set compared to PYL, including the two genes for H+/K+-ATPase. Pathways related to mitochondrial activity and feeding behavior were also enriched (primarily cholecystokinin receptors and ghrelin. Aquaporin 4 was the top-ranking gene. In PYL, two gene sets were enriched compared with OXY: LYMPHOCYTE ACTIVATION and LIPID RAFT, a gene set involved in cholesterol-rich microdomains of the plasma membrane. The single most differentially expressed genes were gastrin and secretoglobin 1A, member 1, presumably located in the epithelial line, to inactivate inflammatory mediators. Several genes related to mucosal integrity, immune response, detoxification and epithelium renewal were also enriched in PYL (P<0.05. The data indicate that there is significant differential gene expression between OXY of the young pig and PYL and further functional studies are needed to

  20. 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...... test and MRI or CT were performed at baseline and after 12 months in 133 patients (52±13 years, 35% female) randomly allocated to losartan (100 mg/day) or placebo. RESULTS: Losartan had no effect on systolic function compared with placebo (mean difference for left ventricular ejection fraction (LVEF) 0......%, (95% CI -3% to -1%), p=0.037) and 4% of patients had end-stage HCM with a LVEF of less than 50% at the end of the study. CONCLUSION: Treatment with losartan had no effect on cardiac function or exercise capacity compared with placebo. Losartan fail to improve myocardial performance and failed to alter...

  1. 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)

  2. 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.

  3. Central vein stenosis masquerading as venous thrombosis

    National Research Council Canada - National Science Library

    Nagapriya Vellalacheruvu; Naresh Monigari; Tom Devasia; Hashir Kareem

    2014-01-01

    .... 3 Learning points Central venous stenosis (CVS), although rare, is a known complication in patients requiring maintenance haemodialysis but symptomatic CVS requiring intervention is not common...

  4. Idiopathic subglottic stenosis: a familial predisposition.

    Science.gov (United States)

    Dumoulin, Elaine; Stather, David R; Gelfand, Gary; Maranda, Bruno; Maceachern, Paul; Tremblay, Alain

    2013-03-01

    Idiopathic subglottic stenosis is a narrowing of the trachea at the level of the cricoid cartilage of unknown etiology. It is a rare condition for which the real incidence has never been established owing to the difficulty of making the diagnosis. Although there is a female preponderance, no familial cases have been reported in the literature. We describe two pairs of sisters as well as a mother and daughter presenting with idiopathic subglottic stenosis. All known causes of tracheal stenosis were excluded, including prolonged intubation, surgery, autoimmune and inflammatory disorders, infection and gastroesophageal reflux disease. These are the first cases reported in the literature that suggest a genetic predisposition for idiopathic subglottic stenosis.

  5. Purification and characterisation of trypsin-like enzyme from the pyloric caeca of cod (Gadus morhua) II

    OpenAIRE

    Luiz Henrique Beirão; Ian Mckintoch Mackie; Evanilda Teixeira; César Damian

    2001-01-01

    A trypsin -like enzyme from the pyloric caeca of cod (Gadus morhua) was purified by affinity chromatography on CHOM Sepharose 4B. Some characteristics were established by its catalytic activity on T.A.M.E., typical enzyme substrate, and serine protease inhibitors. The enzyme had an isoelectric point of 5.30 and 5.89 and was very similar in amino acid composition to bovine trypsin, but differed in having a higher relative amount of acidic amino acids and a lower amount of basic amino acids. Th...

  6. Septal Myectomy Surgery to Treat Obstructive Hypertrophic Cardiomyopathy (HCM)

    Medline Plus

    Full Text Available Septal Myectomy Surgery to Treat Obstructive Hypertrophic Cardiomyopathy (HCM) Click Here to view the BroadcastMed, Inc. Privacy Policy and Legal Notice © 2017 BroadcastMed, Inc. All rights reserved.

  7. 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...

  8. [Hypertrophic obstructive cardiomyopathy in a patient with Turner syndrome].

    Science.gov (United States)

    Conte, M R; Bonfiglio, G; Orzan, F; Mangiardi, L; Camaschella, C; Alfarano, A; Brusca, A

    1995-12-01

    A case of hypertrophic obstructive cardiomyopathy in a patient with Turner syndrome is reported. The most frequently associated cardiac anomalies are coarctation of the aorta and bicuspid aortic valve. Hypertrophic cardiomyopathy has never been reported in this syndrome but is frequent in Noonan syndrome. In these two conditions the phenotype may be indistinguishable but the cariotype is different: normal in Noonan and 45X in Turner syndrome. Our patient had the typical somatic features, and the cariotype was 45X in all examined cells. A familial form of hypertrophic cardiomyopathy was excluded by the normal clinical examination of other members of the family. The presence of hypertrophic cardiomyopathy also in Turner syndrome and the recent localization on the long arm of the chromosome 12 of the gene for Noonan syndrome might postulate a common pathogenesis of the two syndromes.

  9. diel vertical migration of zooplankton in a hypertrophic shallow ...

    African Journals Online (AJOL)

    Preferred Customer

    In this investigation DVM by zooplankton is studied in a hypertrophic shallow lake in Germany. The ... they have intimately evolved through time. This ..... seemed to show similar patterns as the daphnids at ..... Estuaries 25(2):296–307. 8.

  10. Septal Myectomy Surgery to Treat Obstructive Hypertrophic Cardiomyopathy (HCM)

    Science.gov (United States)

    Septal Myectomy Surgery to Treat Obstructive Hypertrophic Cardiomyopathy (HCM) Click Here to view the BroadcastMed, Inc. Privacy Policy and Legal Notice © 2017 BroadcastMed, Inc. All rights reserved.

  11. Hypertrophic cardiomyopathy in a neonate associated with nemaline myopathy.

    Science.gov (United States)

    Mir, Arshid; Lemler, Matthew; Ramaciotti, Claudio; Blalock, Shannon; Ikemba, Catherine

    2012-01-01

    Nemaline myopathy is a congenital nonprogressive skeletal muscle disorder with a characteristic rod body formation in the skeletal muscle fibers. Cardiac involvement in nemaline myopathy is rare, although both dilated and hypertrophic cardiomyopathy have been reported. We describe an infant diagnosed with hypertrophic cardiomyopathy and hypotonia on the first day of life. Muscle biopsy confirmed nemaline myopathy at 3 weeks of age. The diagnosis of nemaline myopathy precluded consideration of heart transplantation, thus shifting the focus to comfort care. This is the earliest presentation of hypertrophic cardiomyopathy reported in the literature in the setting of nemaline myopathy. The approach to determining an etiology for hypertrophic cardiomyopathy in an infant is reviewed. © 2011 Wiley Periodicals, Inc.

  12. Inflammation and impaired adipogenesis in hypertrophic obesity in man

    National Research Council Canada - National Science Library

    Birgit Gustafson; Silvia Gogg; Shahram Hedjazifar; Lachmi Jenndahl; Ann Hammarstedt; Ulf Smith

    2009-01-01

    .... Here, we review recent findings linking hypertrophic obesity with inflammation and a dysregulated adipose tissue, including local cellular insulin resistance with reduced IRS-1 and GLUT4 protein content...

  13. Characterization of signalling pathways in cardiac hypertrophic response

    OpenAIRE

    2011-01-01

    Abstract Intracellular signalling cascades regulate cardiomyocyte hypertrophic response. Initially hypertrophy of individual myocytes occurs as an adaptive response to increased demands for cardiac work, e.g. during hypertension or after myocardial infarction, but a prolonged hypertrophic response, accompanied by accelerated fibrosis and apoptosis, predisposes the heart to impaired performance and the syndrome of heart failure. The goal of this work was to elucidate some of the main sig...

  14. 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.

  15. Recurred Post-intubation Tracheal Stenosis Treated with Bronchoscopic Cryotherapy.

    Science.gov (United States)

    Jung, Ye-Ryung; Taek Jeong, Joon; Kyu Lee, Myoung; Kim, Sang-Ha; Joong Yong, Suk; Jeong Lee, Seok; Lee, Won-Yeon

    Post-intubation tracheal stenosis accounts for the greatest proportion of whole-cause tracheal stenosis. Treatment of post-intubation tracheal stenosis requires a multidisciplinary approach. Surgery or an endoscopic procedure can be used, depending on the type of stenosis. However, the efficacy of cryotherapy in post-intubation tracheal stenosis has not been validated. Here, we report a case of recurring post-intubation tracheal stenosis successfully treated with bronchoscopic cryotherapy that had previously been treated with surgery. In this case, cryotherapy was effective in treating web-like fibrous stenosis, without requiring more surgery. Cryotherapy can be considered as an alternative or primary treatment for post-intubation tracheal stenosis.

  16. Systolic time intervals in congenital aortic stenosis.

    NARCIS (Netherlands)

    Moene, Rudolf Johannens

    1974-01-01

    Obstruction to left ventricular outflow may occur at the valvular, subvalvular and Supravalvular level. The most common congenital forms are valvular aortic stenosis and membranous subaortic stenosis, representing about 75 and l0 percent of all cases respectively. ... Zie: Chapter 1

  17. Severe aortic stenosis: diagnosis, treatment and prognosis

    NARCIS (Netherlands)

    M.W.A. van Geldorp (Martijn)

    2013-01-01

    textabstractDegenerative aortic stenosis is the most common valvular heart disease in developed countries. The prevalence of severe aortic stenosis increases with age from 1% in people below 65 years of age to nearly 6% in people over the age of 85. Since the population life expectancy continues to

  18. Angiographic analysis of congenital mitral stenosis

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Kyung Soo; Yeon, Kyung Mo; Han, Man Chung [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1984-09-15

    Congenital mitral stenosis may be defined as a development abnormality of the mitral valve leaflets, commissures, interchordal spaces, papillary muscles, annulus or immediate supravalvular area producing obstruction to left ventricular filling. Authors had experience of nine case of congenital mitral stenosis confirmed by two dimensional echocardiography, angiocardiography and surgery in recent 5 years since 1979, and analyzed them with emphasis on the angiographic findings. The results are as follows: 1. Among 9 cases, 6 patients were male and 3 were female. Age distribution was from 4 month to 11 years. 2. The types of congenital mitral stenosis were 1 typical congenital mitral stenosis, 5 cases of parachute mitral valve and 3 cases of supramitral ring. 3. Angiographically typical congenital mitral stenosis showed narrowing of mitral valvular opening, parachute mitral valve displayed single large papillary muscle with narrowing valvular opening and supramitral ring disclosed semilunar shaped filling defect between left atrium and ventricle. 4. Associated cardiac and extracardiac anomalies of congenital mitral stenosis, as frequency wise, were ventricular septal defect, patent ductus arteriosus, coarctation of aorta, supra and subvalvular aortic stenosis, mitral regurgitation and double outlet right ventricle. 5. Cardiac angiography is essential to diagnose congenital mitral stenosis, but the need of two dimensional echocardiography cannot be ignored.

  19. Lutembacher Syndrome and Rheumatic Pulmonary Stenosis

    Institute of Scientific and Technical Information of China (English)

    Jufang Chi; Hangyuan Guo; Biao Yang

    2008-01-01

    We discdbed a case of a 55-yr-old woman diagnosed with Lutembacher syndrome and rheumatic pulmonary stenosis.Congenital atrial septal defect was found in age 7 and rheumatic fever in age 34.As the patient developed pulmonary hypertension with calcified mitral valve leaflet and pulmonary stenosis so surgery was not indicated.So the patient was managed by medical therapy alone.

  20. Diagnosis and treatment of renal artery stenosis.

    NARCIS (Netherlands)

    Plouin, P.F.; Bax, L.

    2010-01-01

    A reduction in the diameter of the renal arteries can lead to hypertension, renal dysfunction and/or pulmonary edema. About 90% of patients with renal artery stenosis have atherosclerosis, and 10% have fibromuscular dysplasia. Atherosclerotic renal artery stenosis is a common condition that typicall

  1. Acquired subglottic stenosis : an experimental study

    NARCIS (Netherlands)

    J.K. Bean (Jim)

    1995-01-01

    textabstractSubglottic (endolaryngeal) injury can cause a subglottic stenosis. Chronic subglottic stenosis is defined as a partial narrowing (to complete obliteration) of the airway bounded by the inferior margin of the cricoid at the caudal side and cranially by the insertion of the fibres of the c

  2. 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.)

  3. MR imaging findings of hypertrophic olivary degeneration

    Energy Technology Data Exchange (ETDEWEB)

    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.

  4. Diagnostic performance of computed tomography for detection of concomitant coronary disease in hypertrophic cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Zhao, Lei; Ma, Xiaohai; Zhang, Chen; Wang, Zhanhong; Fan, Zhanming [Capital Medical University, Department of Radiology, Beijing Anzhen Hospital, Beijing (China); Ge, Hailong [Capital Medical University, Department of Cardiology, Beijing Anzhen Hospital, Beijing (China); Teraoka, Kunihiko [Tokyo Medical University, Department of Cardiology, Tokyo (Japan)

    2014-10-31

    To evaluate the diagnostic performance of computed tomography (CT) in patients with hypertrophic cardiomyopathy (HCM) and suspected coexistent coronary artery diseases (CADs). Sixty patients were enrolled in this study. Cardiac CT examination included CT coronary angiography (CTCA) and delayed enhancement CT. CT performance in evaluation of the coronary artery was assessed and compared with that of catheter-based coronary angiography (CA). The left ventricle (LV) wall thickness, functional indices and myocardial delayed enhancement (MDE) were measured via cardiac magnetic resonance (CMR) and CT images. Compared with catheter-based CA, CTCA produced a 100 % (24/24) sensitivity, a 94.4 % (34/36) specificity, a 92.3 % (24/26) positive predictive value and a 100 % (34/34) negative predictive value. CT-measured LV wall thickness and functional indices were correlated with those measured via CMR (P < 0.01), though the CT-measured values were smaller than the CMR-measured values. Bland-Altman analysis showed the volume of the focal MDE determined via CT was slightly smaller than that determined using CMR (mean difference: 0.3 cm{sup 3}). For patients with HCM and suspected coexistent CAD, this comprehensive cardiac CT protocol can be helpful in ruling out coronary stenosis and can provide information regarding morphology, function and tissue characterization of the LV myocardium. (orig.)

  5. Ectopic drainage of the common bile duct into the lesser curvature of the gastric antrum in a newborn with pyloric atresia, annular pancreas and congenital short bowel syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Scheida, Nicola; Amaral, Joao G. [Hospital for Sick Children, Department of Diagnostic Imaging, Division of Image Guided Therapy, Toronto (Canada); Wales, Paul W. [Hospital for Sick Children, Department of Surgery, Toronto (Canada); Krishnamurthy, Ganesh [Hospital for Sick Children, Department of Diagnostic Imaging, Division of Image Guided Therapy, Toronto (Canada); Children' s Hospital of Philadelphia, Department of Diagnostic Imaging, Philadelphia, PA (United States); Chait, Peter G. [Ellesmere X-Ray and Associates, Scarborough (Canada)

    2009-01-15

    We report a newborn with bilious vomiting and the rare combination of pyloric atresia, annular pancreas and ectopic drainage of the common bile duct into the lesser curvature of the gastric antrum. Radiologic, sonographic and percutaneous transhepatic transcholecystic cholangiographic (PTTC) findings, with surgical correlation, are presented. (orig.)

  6. Hypertrophic pachymeningitis: significance of myeloperoxidase anti-neutrophil cytoplasmic antibody.

    Science.gov (United States)

    Yokoseki, Akiko; Saji, Etsuji; Arakawa, Musashi; Kosaka, Takayuki; Hokari, Mariko; Toyoshima, Yasuko; Okamoto, Kouichirou; Takeda, Shigeki; Sanpei, Kazuhiro; Kikuchi, Hirotoshi; Hirohata, Shunsei; Akazawa, Kouhei; Kakita, Akiyoshi; Takahashi, Hitoshi; Nishizawa, Masatoyo; Kawachi, Izumi

    2014-02-01

    The aim of this study was to elucidate the characteristics, pathogenesis and treatment strategy of hypertrophic pachymeningitis that is associated with myeloperoxidase anti-neutrophil cytoplasmic antibody (ANCA). We retrospectively investigated clinical, radiological, immunological and pathological profiles of 36 patients with immune-mediated or idiopathic hypertrophic pachymeningitis, including 17 patients with myeloperoxidase-ANCA, four patients with proteinase 3-ANCA, six patients with other immune-mediated disorders, and nine patients with 'idiopathic' variety. Myeloperoxidase-ANCA-positive hypertrophic pachymeningitis was characterized by: (i) an elderly female predominance; (ii) 82% of patients diagnosed with granulomatosis with polyangiitis (previously known as Wegener's granulomatosis) according to Watts' algorithm; (iii) a high frequency of patients with lesions limited to the dura mater and upper airways, developing headaches, chronic sinusitis, otitis media or mastoiditis; (iv) a low frequency of patients with the 'classical or generalized form' of granulomatosis with polyangiitis involving the entire upper and lower airways and kidney, or progressing to generalized disease, in contrast to proteinase 3-ANCA-positive hypertrophic pachymeningitis; (v) less severe neurological damage according to the modified Rankin Scale and low disease activity according to the Birmingham Vasculitis Activity Score compared with proteinase 3-ANCA-positive hypertrophic pachymeningitis; (vi) increased levels of CXCL10, CXCL8 and interleukin 6 in cerebrospinal fluids, and increased numbers of T cells, neutrophils, eosinophils, plasma cells and monocytes/macrophages in autopsied or biopsied dura mater with pachymeningitis, suggesting TH1-predominant granulomatous lesions in hypertrophic pachymeningitis, as previously reported in pulmonary or renal lesions of granulomatosis with polyangiitis; and (vii) greater efficacy of combination therapy with prednisolone and

  7. Anesthesia for subglottic stenosis in pediatrics

    Directory of Open Access Journals (Sweden)

    Eid Essam

    2009-01-01

    Full Text Available Any site in the upper airway can get obstructed and cause noisy breathing as well as dyspnea. These include nasal causes such as choanal atresia or nasal stenosis; pharyngeal causes including lingual thyroid; laryngeal causes such as laryngomalacia; tracheobronchial causes such as tracheal stenosis; and subglottic stenosis. Lesions in the oropharynx may cause stertor, while lesions in the laryngotracheal tree will cause stridor. Subglottic stenosis is the third leading cause of congenital stridors in the neonate. Subglottic Stenosis presents challenges to the anesthesiologist. Therefore, It is imperative to perform a detailed history, physical examination, and characterization of the extent and severity of stenosis. Rigid endoscopy is essential for the preoperative planning of any of the surgical procedures that can be used for correction. Choice of operation is dependent on the surgeon′s comfort, postoperative capabilities, and severity of disease. For high-grade stenosis, single-stage laryngotracheal resection or cricotracheal resection are the best options. It has to be borne in mind that the goal of surgery is to allow for an adequate airway for normal activity without the need for tracheostomy. Anesthesia for airway surgery could be conducted safely with either sevofl uraneor propofol-based total intravenous anesthesia.

  8. Anesthesia for subglottic stenosis in pediatrics.

    Science.gov (United States)

    Eid, Essam A

    2009-07-01

    Any site in the upper airway can get obstructed and cause noisy breathing as well as dyspnea. These include nasal causes such as choanal atresia or nasal stenosis; pharyngeal causes including lingual thyroid; laryngeal causes such as laryngomalacia; tracheobronchial causes such as tracheal stenosis; and subglottic stenosis. Lesions in the oropharynx may cause stertor, while lesions in the laryngotracheal tree will cause stridor. Subglottic stenosis is the third leading cause of congenital stridors in the neonate. Subglottic Stenosis presents challenges to the anesthesiologist. Therefore, It is imperative to perform a detailed history, physical examination, and characterization of the extent and severity of stenosis. Rigid endoscopy is essential for the preoperative planning of any of the surgical procedures that can be used for correction. Choice of operation is dependent on the surgeon's comfort, postoperative capabilities, and severity of disease. For high-grade stenosis, single-stage laryngotracheal resection or cricotracheal resection are the best options. It has to be borne in mind that the goal of surgery is to allow for an adequate airway for normal activity without the need for tracheostomy. Anesthesia for airway surgery could be conducted safely with either sevofluraneor propofol-based total intravenous anesthesia.

  9. The Influence of Antral Ulcers on Intramural Gastric Nerve Projections Supplying the Pyloric Sphincter in the Pig (Sus scrofa domestica-Neuronal Tracing Studies.

    Directory of Open Access Journals (Sweden)

    Michal Zalecki

    Full Text Available Gastric ulcerations in the region of antrum pylori represent a serious medical problem in humans and animals. Such localization of ulcers can influence the intrinsic descending nerve supply to the pyloric sphincter. The pyloric function is precisely regulated by intrinsic and extrinsic nerves. Impaired neural regulation could result in pyloric sphincter dysfunction and gastric emptying malfunction. The aim of the study was to determine the effect of gastric antral ulcerations on the density and distribution of intramural gastric descending neurons supplying the pyloric sphincter in pigs.The experiment was performed on 2 groups of pigs: healthy gilts (n=6 and gilts with experimentally induced peptic ulcers in the region of antrum pylori (n=6. Gastric neurons supplying pyloric sphincter were labeled using the retrograde neuronal tracing technique (20μl of Fast Blue tracer injected into the pyloric sphincter muscle. After a week survival period the animals were sacrificed and the stomachs were collected. Then, the stomach wall was cross-cut into 0.5cm thick sections taken in specified intervals (section I - 1.5cm; section II - 3.5cm; section III - 5.5cm; section IV - 7.5cm starting from the sphincter. Consecutive microscopic slices prepared from each section were analyzed under fluorescent microscope to count traced neurons. Obtained data were statistically analyzed. The total number of FB-positive perikarya observed within all studied sections significantly decreased from 903.3 ± 130.7 in control to 243.8 ± 67.3 in experimental animals. In healthy pigs 76.1 ± 6.7% of labeled neurons were observed within the section I, 23.53 ± 6.5% in section II and only occasional cells in section III. In experimental animals, as many as 93.8 ± 2.1% of labeled cells were observed within the section I and only 6.2 ± 2.2% in section II, while section III was devoid of such neurons. There were no traced perikarya in section IV observed in both groups of pigs

  10. Low-gradient aortic stenosis.

    Science.gov (United States)

    Clavel, Marie-Annick; Magne, Julien; Pibarot, Philippe

    2016-09-07

    An important proportion of patients with aortic stenosis (AS) have a 'low-gradient' AS, i.e. a small aortic valve area (AVA <1.0 cm(2)) consistent with severe AS but a low mean transvalvular gradient (<40 mmHg) consistent with non-severe AS. The management of this subset of patients is particularly challenging because the AVA-gradient discrepancy raises uncertainty about the actual stenosis severity and thus about the indication for aortic valve replacement (AVR) if the patient has symptoms and/or left ventricular (LV) systolic dysfunction. The most frequent cause of low-gradient (LG) AS is the presence of a low LV outflow state, which may occur with reduced left ventricular ejection fraction (LVEF), i.e. classical low-flow, low-gradient (LF-LG), or preserved LVEF, i.e. paradoxical LF-LG. Furthermore, a substantial proportion of patients with AS may have a normal-flow, low-gradient (NF-LG) AS: i.e. a small AVA-low-gradient combination but with a normal flow. One of the most important clinical challenges in these three categories of patients with LG AS (classical LF-LG, paradoxical LF-LG, and NF-LG) is to differentiate a true-severe AS that generally benefits from AVR vs. a pseudo-severe AS that should be managed conservatively. A low-dose dobutamine stress echocardiography may be used for this purpose in patients with classical LF-LG AS, whereas aortic valve calcium scoring by multi-detector computed tomography is the preferred modality in those with paradoxical LF-LG or NF-LG AS. Although patients with LF-LG severe AS have worse outcomes than those with high-gradient AS following AVR, they nonetheless display an important survival benefit with this intervention. Some studies suggest that transcatheter AVR may be superior to surgical AVR in patients with LF-LG AS.

  11. Potential applications for transesophageal echocardiography in hypertrophic cardiomyopathies.

    Science.gov (United States)

    Widimsky, P; Ten Cate, F J; Vletter, W; van Herwerden, L

    1992-01-01

    The purpose of the present study was to evaluate the potential advantages of transesophageal echocardiography (TEE) in comparison with transthoracic echocardiography (TTE) in selected patients with hypertrophic cardiomyopathy. Ten patients with previously established or suspected diagnosis of hypertrophic cardiomyopathy were examined by TEE to solve specific clinical questions. TEE was well tolerated by all patients; no arrhythmias were seen during the procedure. The comparison of TTE and TEE showed the following: Advantages of TTE--better assessment of the left ventricle, myocardial thickness measurements available in all regions and sufficient for the diagnosis of hypertrophic cardiomyopathy in nine out of 10 patients; advantages of TEE--precise assessment of mitral valve morphology and regurgitant jets, detailed evaluation of systolic anterior motion, and subaortic membrane (not seen by TTE) recognized in one patient. Clinically, in three patients TEE influenced the management (mitral leaflet perforation, subaortic membrane, and residual mitral regurgitation after valvuloplasty). Thus TEE enables more precise diagnosis in some patients with hypertrophic cardiomyopathy and has the potential to influence their surgical management. However, for medical treatment of hypertrophic cardiomyopathy, TTE is sufficient.

  12. Medicinal Plants for the Treatment of Hypertrophic Scars

    Directory of Open Access Journals (Sweden)

    Qi Ye

    2015-01-01

    Full Text Available Hypertrophic scar is a complication of wound healing and has a high recurrence rate which can lead to significant abnormity in aesthetics and functions. To date, no ideal treatment method has been established. Meanwhile, the underlying mechanism of hypertrophic scarring has not been clearly defined. 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. Therefore, a systematic summary and knowledge for future prospects are necessary to facilitate further medicinal plant research for their potential use as antihypertrophic scar agents. A bibliographic investigation was accomplished by focusing on medicinal plants which have been scientifically tested in vitro and/or in vivo and proved as potential agents for the treatment of hypertrophic scars. Although the chemical components and mechanisms of action of medicinal plants with antihypertrophic scarring potential have been investigated, many others remain unknown. More investigations and clinical trials are necessary to make use of these medical plants reasonably and phytotherapy is a promising therapeutic approach against hypertrophic scars.

  13. 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 appea

  14. 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

  15. Keratinocyte-derived growth factors play a role in the formation of hypertrophic scars

    NARCIS (Netherlands)

    Niessen, FB; Andriessen, MP; Schalkwijk, J; Visser, L; Timens, W

    2001-01-01

    In predisposed individuals, wound healing can lead to hypertrophic scar or keloid formation, characterized by an overabundant extracellular matrix. It has recently been shown that hypertrophic scars are accompanied by abnormal keratinocyte differentiation and proliferation, and significantly increas

  16. Carotid artery stenosis after neck radiotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Shimamura, Munehisa; Hashimoto, Yoichiro; Kasuya, Junji; Terasaki, Tadashi [Kumamoto City Hospital (Japan); Uchino, Makoto

    2000-02-01

    Carotid artery stenosis sometimes occurs after cervical radiotherapy. We report a 70-year-old woman with a history of radiotherapy for thyroid cancer at the age of 28 years. She had no signs and symptoms except the skin lesion at the irradiation site. Duplex ultrasonography revealed heterogeneous plaques showing 50% stenosis of bilateral common carotid arteries. Those lesions were observed within segment of irradiation, where atheromatous plaque usually seldom occurs. These indicated that the carotid stenosis was induced by radiotherapy. Although the efficacy of antiplatelet therapy for radiation-induced plaque is not clear, the plaques remained unchanged for 4 years in spite of aspirin administration. (author)

  17. Genetic risk factors for hypertrophic scar development.

    Science.gov (United States)

    Thompson, Callie M; Hocking, Anne M; Honari, Shari; Muffley, Lara A; Ga, Maricar; Gibran, Nicole S

    2013-01-01

    Hypertrophic scars (HTSs) occur in 30 to 72% patients after thermal injury. Risk factors include skin color, female sex, young age, burn site, and burn severity. Recent correlations between genetic variations and clinical conditions suggest that single-nucleotide polymorphisms (SNPs) may be associated with HTS formation. The authors hypothesized that an SNP in the p27 gene (rs36228499) previously associated with decreased restenosis after coronary stenting would be associated with lower Vancouver Scar Scale (VSS) measurements and decreased itching. Patient and injury characteristics were collected from adults with thermal burns. VSS scores were calculated at 4 to 9 months after injury. Genotyping was performed using real-time polymerase chain reaction. Logistic regression was used to determine risk factors for HTS as measured by a VSS score >7. Three hundred subjects had a median age of 39 years (range, 18-91); 69% were male and median burn size was 7% TBSA (range, 0.25-80). Consistent with literature, the p27 variant SNP had an allele frequency of 40%, but was not associated with reduced HTS formation or lower itch scores in any genetic model. HTS formation was associated with American Indian/Alaskan Native race (odds ratio [OR], 12.2; P = .02), facial burns (OR, 9.4; P = .04), and burn size ≥20% TBSA (OR, 1.99; P = .03). Although the p27 SNP may protect against vascular fibroproliferation, the effect cannot be generalized to cutaneous scars. This study suggests that American Indian/Alaskan Native race, facial burns, and higher %TBSA are independent risk factors for HTS. The American Indian/Alaskan Native association suggests that there are potentially yet-to-be-identified genetic variants.

  18. RISK OF PATIENTS WITH HYPERTROPHIC CARDIOMYOPATHY UNDERGOING NONCARDIAC SURGERY

    Institute of Scientific and Technical Information of China (English)

    Tian-ming Xuan; Yong Zeng; Wen-ling Zhu

    2007-01-01

    To determine the risk of noncardiac surgery in patients with hypertrophic cardiomyopathy.Methods We reviewed the medical records of all patients who were diagnosed as hypertrophic cardiomyopathy at Peking Union Medical College Hospital from January 1998 to August 2006 and identified 24 patients who subsequently underwent noncardiac surgery.Results There were no intraoperative cardiac events. Postoperative cardiac events were identified in 3 patients including 1 death due to acute myocardial infarction and 2 episodes of transient hypotension.Conclusions The risk of anesthesia and noncardiac surgery is low in patients with hypertrophic cardiomyopathy.During the perioperative period, beta-blockers and/or calcium channel blockers should be given; vasodilator and inotropic agents should be avoided due to the side effects on hemodynamics.

  19. 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

  20. 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 sca

  1. Left coronary artery stenosis causing left ventricular dysfunction in two children with supravalvular aortic stenosis.

    Science.gov (United States)

    Yildiz, Okan; Altin, Firat H; Kaya, Mehmet; Ozyılmaz, Isa; Guzeltas, Alper; Erek, Ersin

    2015-04-01

    Congenital supravalvar aortic stenosis (SVAS) is an arteriopathy associated with Williams-Beuren syndrome (WBS) and other isolated elastin gene deletions. Cardiovascular manifestations associated with WBS are characterized by obstructive arterial lesions such as SVAS and pulmonary artery stenosis in addition to bicuspid aortic valve and mitral valve prolapse. However, coronary artery ostial stenosis may be associated with SVAS, and it increases the risk of sudden death and may complicate surgical management. In this report, we present our experience with two patients having SVAS and left coronary artery ostial stenosis with associated left ventricular dysfunction. © The Author(s) 2014.

  2. Assessment of myocardial fibrosis and coronary arteries in hypertrophic cardiomyopathy using combined arterial and delayed enhanced CT: comparison with MR and coronary angiography

    Energy Technology Data Exchange (ETDEWEB)

    Zhao, Lei; Ma, Xiaohai; Zhang, Chen; Liu, Yi; Zhang, Zhaoqi [Capital Medical University, Department of Radiology, Beijing Anzhen Hospital, Beijing (China); DeLano, Mark C. [Michigan State University, Division of Radiology and Biomedical Imaging, College of Human Medicine, Lancing, MI (United States); Jiang, Tengyong [Capital Medical University, Department of Cardiology, Beijing Anzhen Hospital, Beijing (China)

    2013-04-15

    We sought to determine the feasibility and accuracy of dual-source computed tomography (DSCT) in assessing coronary artery disease and myocardial fibrosis of hypertrophic cardiomyopathy (HCM) compared with cardiac magnetic resonance (CMR) imaging and coronary angiography (CA). Forty-seven consecutive patients with HCM were prospectively enrolled. DSCT images were acquired in the arterial and late phases following intravenous contrast medium. The CMR and CA were performed within 7 days. Independent blinded readers read each study. Patients were classified according to myocardial delayed enhanced (MDE) CMR, coronary artery stenosis by CA, and arterial and MDE-DSCT. The diagnostic accuracy of DSCT in detecting coronary stenosis and MDE was analysed. Wall thickness determined by DSCT was strongly correlated with MR results (r = 0.91). DSCT and CMR MDE showed substantial agreement for the detection of myocardial fibrosis on per-patient and per-segment levels. The CT classification of patients by arterial stenosis and delayed enhancement had excellent agreement with MR and CA methods. The comprehensive cardiac CT examination provides reliable coronary artery and myocardial assessments. MDE-DSCT is a robust alternative method to MDE-CMR in assessing myocardial fibrosis in HCM particularly in patients with pacemakers or other contraindications to CMR. (orig.)

  3. Subclavian steal syndrome without subclavian stenosis

    Directory of Open Access Journals (Sweden)

    Matt Cwinn, MD

    2017-09-01

    Full Text Available Subclavian steal syndrome (SSS has been well described in the setting of subclavian stenosis. We describe an unusual case of SSS caused by a high-flow arteriovenous dialysis fistula in the absence of subclavian stenosis, provide a review of the literature, and propose that arteriovenous fistula-induced SSS is an underdiagnosed cause of syncope in this population of patients.

  4. MRI Findings in Spinal Canal Stenosis

    OpenAIRE

    Maryam Barzin

    2010-01-01

    Spinal canal stenosis results from progressive narrowing of the central spinal canal and the lateral recesses. Primary (congenital) lumbar spinal stenosis is associated with achondroplastic dwarfism. The spinal canal may become narrowed by bulging or protrusion of the intervertebral disc annulus, herniation of the nucleus pulposus posteriorly, thickening of the posterior longitudinal ligament, hypertrophy of the facet joints, hypertrophy of the ligamentum flavum, epidural fat deposition, spon...

  5. Suture-induced right coronary artery stenosis.

    Science.gov (United States)

    Seltmann, Martin; Achenbach, Stephan; Muschiol, Gerd; Feyrer, Richard

    2010-01-01

    An 82-year-old patient developed right heart failure in the days after surgical aortic valve replacement. Coronary CT angiography showed a high-grade stenosis of the mid-right coronary artery. Adjacent suture material seen on noncontrast CT suggested that the lesion was related to surgical closure of the right atrial cannulation site. Invasive angiography confirmed the stenosis, and percutaneous intervention was successfully performed.

  6. Epidemiology and genetics of hypertrophic cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Tanjore Reena

    2006-01-01

    Full Text Available Background: Hypertrophic cardiomyopathy (HCM is a heart muscle disorder and is known to be inherited as an autosomal dominant trait. Mutations in several sarcomeric, cytoskeletal and mitochondrial genes have been reported in HCM. Though many cases of HCM are being identified, there is limited data regarding the epidemiology and genetics of HCM in India. Aim: Therefore the present study is envisaged at identifying the epidemiological variables in HCM and fitting a probability model assuming dominant mode of inheritance in HCM, which may in turn shed light on the heterogeneity of this complex disorder. Materials AND Methods: The 127 HCM cases were divided into subtypes based on pattern of hypertrophy. Chi square analysis, odds ratio, probability, relative frequency, penetrance and heritability estimates were calculated apart from epidemiological variables. Results: The HCM subtypes revealed the heterogeneous nature of the condition suggesting that the genes/mutations involved in their pathogenesis are different and this is supported by distinctive differences observed in their probability, heritability and penetrance estimates apart from epidemiological variables. An increased male preponderance was observed with the sex ratio being 3.7:1. The age at onset was found to be more than a decade early in familial cases (30 ± 10 yrs compared to non familial cases (44 ± 14 yrs. Chi square analysis revealed obstructive HCM to be following autosomal dominant mode of inheritance where as non-obstructive HCM was significantly deviating. The level of deviation was significantly high for the middle onset group compared to early and late onset groups, therefore this group may be considered as an admixture wherein genes/gene modifiers and environmental variables may be contributing to the heterogeneity and this is further supported by odds ratio. Conclusions: The study thus brings out the complexity of HCM and suggests that modes of inheritance other than

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

    Institute of Scientific and Technical Information of China (English)

    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 bum 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 ofeukaryotic 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.

  8. 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....

  9. Hypertrophic cardiomyopathy with apical left ventricular aneurysm: a case report

    Institute of Scientific and Technical Information of China (English)

    江腾勇; 韩智红; 王京; 吕强; 吴学思

    2002-01-01

    @@ Morphological diversity is one characteristic of hypertrophic cardi omyopathy (HCM), but it is not common that HCM is associated with apical left ve ntricular aneurysm (LVA) without evidence of a coronary artery lesion. We repor t on such a case and review the pathogenesis, manifestations and diagnostic meth ods by collecting the few available papers published on this topic.

  10. 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.

    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 serious functional and cosmetic problems. It seems that a wide array o

  11. 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 arr

  12. 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

  13. 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

  14. Hypertrophic osteopathy associated with a renal adenoma in a cat.

    Science.gov (United States)

    Johnson, Robert L; Lenz, Stephen D

    2011-01-01

    Hypertrophic osteopathy is a hyperostotic syndrome of the appendicular skeleton that is most commonly associated with intrathoracic neoplasia or inflammation. The condition is rarely associated with intra-abdominal lesions. The majority of cases have occurred in dogs and human beings, with fewer cases reported in cats, horses, and other species. A 15-year-old male neutered Domestic Shorthair cat presented for swollen limbs and difficulty in ambulation. Radiographs and gross postmortem revealed severe periosteal hyperostosis of the diaphysis and metaphysis of all 4 limbs, including the humerus, radius, ulna, carpi, metacarpi, femur, tibia, tarsi, metatarsi, and phalanges. The axial skeleton was spared. Hyperostotic lesions were characterized microscopically by lamellar bony trabeculae separated by adipocytes and scant hematopoietic tissue. In several areas, fibrovascular connective tissue, woven bone, and islands of cartilage were also present. A 2.5 cm × 2.5 cm perirenal neoplasm compressed the left kidney and adrenal gland. This mass consisted of well-differentiated tubules of cuboidal epithelial cells and was most consistent with a renal tubular adenoma, because mitotic figures were rare, and no distant metastases were found. Thoracic pathology was absent. Hyperostosis was consistent with hypertrophic osteopathy secondary to the renal adenoma. The pathogenesis of hypertrophic osteopathy is uncertain, but predominant theories point to increased peripheral circulation and angiogenesis as a key initiating event. Recent literature highlights the potential role of vascular endothelial growth factor and platelet-derived growth factor in the human condition. The mechanism by which this renal adenoma caused hypertrophic osteopathy is unknown.

  15. MRI Findings in Spinal Canal Stenosis

    Directory of Open Access Journals (Sweden)

    Maryam Barzin

    2010-05-01

    Full Text Available Spinal canal stenosis results from progressive narrowing of the central spinal canal and the lateral recesses. Primary (congenital lumbar spinal stenosis is associated with achondroplastic dwarfism. The spinal canal may become narrowed by bulging or protrusion of the intervertebral disc annulus, herniation of the nucleus pulposus posteriorly, thickening of the posterior longitudinal ligament, hypertrophy of the facet joints, hypertrophy of the ligamentum flavum, epidural fat deposition, spondylosis of the intervertebral disc margins and uncovertebral joint hypertrophy in the neck. The central canal and the neurorecess may be compromised by tumor infiltration, such as metastatic disease, or by infectious spondylitis."nAP diameter of the normal adult cervical canal has a mean value of 17-18 mm at vertebral levels C3-5. The lower cervical canal measures 12-14 mm. Cervical stenosis is associated with an AP diameter of less than 10 mm. The thoracic spinal canal varies from 12 to 14 mm in diameter in the adult. The diameter of the normal lumbar spinal canal varies from 15 to 27 mm. Lumbar stenosis results from a spinal canal diameter of less than 12 mm in some patients; a diameter of 10 mm is definitely stenotic."nSpinal MRI is the most suitable technique for the diagnosis of spinal stenosis. The examination should be performed using thin sections (3 mm and high resolution, including the axial and sagittal planes using T1-weighted, proton-density, and T2-weighted techniques. The bony and osteophytic components are seen best using a T2-weighted gradient-echo technique."nOn MRI, findings of spinal stenosis have a variable presentation depending on the specific disease. The goal of spinal imaging is to localize the site and level of disease and to help differentiate between conditions in which patients require surgery or conservative treatment."nIn this presentation, different kinds of spinal canal stenosis and their MRI findings would be discussed.

  16. Quantitative coronary CT angiography: absolute lumen sizing rather than %stenosis predicts hemodynamically relevant stenosis

    Energy Technology Data Exchange (ETDEWEB)

    Plank, Fabian [Innsbruck Medical University, Department of Radiology, Innsbruck (Austria); Innsbruck Medical University, Department of Internal Medicine III - Cardiology, Innsbruck (Austria); Burghard, Philipp; Mayr, Agnes; Klauser, Andrea; Feuchtner, Gudrun [Innsbruck Medical University, Department of Radiology, Innsbruck (Austria); Friedrich, Guy; Dichtl, Wolfgang [Innsbruck Medical University, Department of Internal Medicine III - Cardiology, Innsbruck (Austria); Wolf, Florian [Vienna Medical University, Department of Cardiovascular and Interventional Radiology, Vienna (Austria)

    2016-11-15

    To identify the most accurate quantitative coronary stenosis parameter by CTA for prediction of functional significant coronary stenosis resulting in coronary revascularization. 160 consecutive patients were prospectively examined with CTA. Proximal coronary stenosis was quantified by minimal lumen area (MLA) and minimal lumen diameter (MLD), %area and %diameter stenosis. Lesion length (LL) was measured. The reference standard was invasive coronary angiography (ICA) (>70 % stenosis, FFR <0.8). 210 coronary segments were included (59 % positive). MLA of ≤1.8 mm{sup 2} was identified as the optimal cut-off (c = 0.97, p < 0.001; 95 % CI 0.94-0.99) (sensitivity 90.9 %, specificity 89.3 %) for prediction of functional-relevant stenosis (for MLA >2.1 mm{sup 2} sensitivity was 100 %). The optimal cut-off for MLD was 1.2 mm (c = 0.92; p < 0.001; 95 % CI 0.88-95) (sensitivity 90.9, specificity 85.2) while %area and %diameter stenosis were less accurate (c = 0.89; 95 % CI 0.84-93, c = 0.87; 95 % CI 0.82-92, respectively, with thresholds at 73 % and 61 % stenosis). Accuracy for LL was c = 0.74 (95 % CI 0.67-81), and for LL/MLA and LL/MLD ratio c = 0.90 and c = 0.84. MLA ≤1.8 mm{sup 2} and MLD ≤1.2 mm are the most accurate cut-offs for prediction of haemodynamically significant stenosis by ICA, with a higher accuracy than relative % stenosis. (orig.)

  17. Therapeutic value of endoscopic metal pyloric stent implantation for gastric outlet obstruction%经内镜放置幽门金属支架对胃出口梗阻的治疗价值

    Institute of Scientific and Technical Information of China (English)

    朱静; 张杰; 陈婧; 郎海波; 于剑锋; 郝建宇

    2012-01-01

    目的 探讨经内镜放置幽门金属支架对胃出口梗阻的治疗价值.方法 对2008年1月至2012年1月因多种良恶性疾病引起胃出口梗阻而接受内镜下幽门金属支架植入术的21例患者的病例资料进行回顾性分析.结果 21例成功进行了幽门十二指肠支架置入术,14例24小时内症状明显缓解,1例于48小时后行CRE球囊扩张后缓解,总缓解率为71.4%.21例中有3例十二指肠壶腹部受累伴梗阻性黄疸的病例成功进行了双支架的置入治疗.3例出现术中出血,对症治疗后停止.1例良性狭窄病例使用覆膜支架发生移位,4例出现支架术后再堵塞,其中2例行二次支架置入.恶性肿瘤引起的狭窄术后生存时间平均为2.5个月.结论 对由于各种原因导致胃出口梗阻且无法手术或拒绝外科治疗的患者,幽门支架的置入可有效解除梗阻,改善患者生活质量.%Objective To evaluate the therapeutic value of endoscopic implantation of metal stent in gastric outlet obstruction. Methods Data of 21 patients who developed benign or malignant gastric outlet obstruction from January 2008 to January 2011 were retrospectively reviewed. Results Endoscopic metal pyloric stents were successfully placed in 21 patients. Symptoms were subsided in 14 patients. 1 patient subsided after CRE ballon in 48 hours. Response rate was 71.4%. 3 duodenal patients ampulla involvement with cases of obstructive jaundice successfully treated two-stent implantation. Bleeding occurred in 3 patients, 1 case of benign stenosis using stent occurred displacement , Obstruction occurred in 4 patients, and 2 of them received the second metal pyloric stent. The average survival time of obstruction patient with malignant was 2.5 months. Conclusion No chance of surgical treatment or refuse surgery patients because of some causes gastric outlet obstruction can effectively remove the obstruction and improve patients quality of life.

  18. Value of electrocardiogram in the differentiation of hypertensive heart disease, hypertrophic cardiomyopathy, aortic stenosis, amyloidosis, and Fabry disease.

    NARCIS (Netherlands)

    Namdar, M.; Steffel, J.; Jetzer, S.; Schmied, C.; Hurlimann, D.; Camici, G.G.; Bayrak, F.; Ricciardi, D.; Rao, J.Y.; Asmundis, C. de; Chierchia, G.B.; Sarkozy, A.; Luscher, T.F.; Jenni, R.; Duru, F.; Brugada, P.

    2012-01-01

    Left ventricular hypertrophy is 1 of the most frequent cardiac manifestations associated with an unfavorable prognosis. However, many different causes of left ventricular hypertrophy exist. The aim of the present study was to assess the diagnostic value of common electrocardiographic (ECG) parameter

  19. Echocardiographic Assessment of Mantle Radiation Mitral Stenosis.

    Science.gov (United States)

    Bastiaenen, Rachel; Sneddon, James; Sharma, Rajan

    2016-02-01

    The long-term sequelae of mantle radiotherapy include lung disease and cardiac disorders. Dyspnea on exertion is a common complaint and can be due to one or more pathologies. We describe a case of mantle radiotherapy-induced mitral stenosis, characterized by aorto-mitral continuity calcification and absent commissural fusion which precludes balloon valvotomy. The latency period is long, and this patient presented 42 years after radiotherapy. Importantly, as previously described with radiation-induced valve disease, significant mitral stenosis developed 10 years after surgery for significant aortic stenosis. Two-dimensional and three-dimensional transthoracic and transesophageal echocardiography should be considered during assessment of symptomatic survivors of Hodgkin's disease where the index of suspicion for valvular stenosis increases over time. Given the natural history of mantle radiation valvular disease, a lower threshold for surgical intervention in radiation-induced mitral stenosis may need to be considered if cardiac surgery is planned for other reasons in order to avoid repeated sternotomy in patients with prior irradiation.

  20. Haemoptysis due to pulmonary venous stenosis

    Directory of Open Access Journals (Sweden)

    Silke Braun

    2014-06-01

    Full Text Available Haemoptysis is a potentially life-threatening condition with the need for prompt diagnosis. In about 10–20% of all cases the bleeding source remains unexplained with the standard diagnostic approach. The aim of this article is to show the necessity of widening the diagnostic approach to haemoptysis with consideration of pulmonary venous stenosis as a possible cause of even severe haemoptysis and haemoptoe. A review of the literature was performed using the Medline/PubMed database with the terms: “pulmonary venous stenosis”, “pulmonary venous infarction” and “haemoptysis”. Further references from the case reports were considered. 58 case reports and case collections about patients with haemoptysis due to pulmonary venous stenosis were detected. This review gives an overview about the case reports and discusses the underlying pathophysiology and the pros and cons of different imaging techniques for the detection of pulmonary venous stenosis. Several conditions predispose to the obstruction of the mediastinal pulmonary veins. Clinical findings are unspecific and may be misleading. Pulmonary venous stenosis can be detected using several imaging techniques, yet three-dimensional magnetic resonance-angiography and three-dimensional contrast-enhanced computed tomography are the most appropriate. Pulmonary venous stenosis should be considered in patients with haemoptysis.

  1. Stenosis of calcified carotid artery detected on Panoramic Radiography

    Energy Technology Data Exchange (ETDEWEB)

    Cho, So Yang; Oh, Won Mann; Yoon, Suk Ja; Yoon, Woong; Lee, Jae Seo; Kang, Byung Cheol [School of Dentistry, Chonnam National University, Seoul (Korea, Republic of); Palomo, Juan M. [Department of Orthodontics, School of Dental Medicine, Case Western Reserve University, Cleveland (United States)

    2009-09-15

    This study aimed to investigate the luminal stenosis of the internal carotid artery with calcification detected on panoramic radiographs. This study used fifty carotid arteries of 36 dental patients whose panoramic radiograph and computed tomography angiography (CTA) revealed the presence of carotid artery calcification. A neuroradiologist interpreted CTA to determine the degree of stenosis of the internal carotid arteries. The degree of stenosis was stratified in four stages; normal (no stenosis), mild stenosis (1-49%), moderate stenosis (50-69%) and severe stenosis (70-99%). Among the fifty carotid arteries with calcification detected on both panoramic radiography and CTA, 20 carotid arteries (40%) were normal, 29 carotid arteries (18%) had mild stenosis, 1 carotid artery (2%) had moderate stenosis, and there was none with severe stenosis. Sixty percent of the carotid arteries with calcification detected on both panoramic radiography and CTA had internal luminal stenosis, and two percent had moderate stenosis. When carotid atheroma is detected on panoramic radiograph, it is possible that the dental patient has luminal stenosis of the internal carotid artery.

  2. A Pyloric Gland-Phenotype Ovarian Mucinous Tumor Resembling Lobular Endocervical Glandular Hyperplasia in a Patient with Peutz-Jeghers Syndrome.

    Science.gov (United States)

    Kim, Eun Na; Kim, Gu-Hwan; Kim, Jiyoon; Park, In Ah; Shin, Jin Ho; Chai, Yun; Kim, Kyu-Rae

    2017-03-01

    We describe an ovarian mucinous neoplasm that histologically resembles lobular endocervical glandular hyperplasia (LEGH) containing pyloric gland type mucin in a patient with Peutz-Jeghers syndrome (PJS). Although ovarian mucinous tumors rarely occur in PJS patients, their pyloric gland phenotype has not been clearly determined. The histopathologic features of the ovarian mucinous tumor were reminiscent of LEGH. The cytoplasmic mucin was stained with periodic acid-Schiff reaction after diastase treatment but was negative for Alcian blue pH 2.5, suggesting the presence of neutral mucin. Immunohistochemically, the epithelium expressed various gastric markers, including MUC6, HIK1083, and carbonic anhydrase-IX. Multiple ligation-dependent probe amplification detected a germline heterozygous deletion mutation at exons 1-7 of the STK11 gene (c.1-?_920+?del) in peripheral blood leukocytes and mosaic loss of heterozygosity in ovarian tumor tissue. Considering that LEGH and/or gastric-type cervical adenocarcinoma can be found in patients with PJS carrying germline and/or somatic STK11 mutations, our case indicates that STK11 mutations have an important role in the proliferation of pyloric-phenotype mucinous epithelium at various anatomical locations.

  3. Global Strain in Severe Aortic Valve Stenosis

    DEFF Research Database (Denmark)

    Dahl, Jordi S; Videbæk, Lars; Poulsen, Mikael K

    2012-01-01

    BACKGROUND: -Global longitudinal systolic strain (GLS) is often reduced in aortic stenosis despite normal ejection fraction. The importance of reduced preoperative GLS on long-term outcome after aortic valve replacement (AVR) is unknown. METHODS AND RESULTS: -A total of 125 patients with severe...... aortic stenosis and ejection fraction >40% scheduled for AVR were evaluated preoperatively and divided into 4 groups according to GLS quartiles. Patients were followed up for 4 years. The primary endpoint was major adverse cardiac events (MACE) defined as cardiovascular mortality and cardiac......Score, history with ischemic heart disease and ejection fraction. CONCLUSIONS: -In patients with symptomatic severe aortic stenosis undergoing AVR reduced GLS provides important prognostic information beyond standard risk factors. Clinical Trial Registration-URL: http://www.clinicaltrial.gov. Unique identifier...

  4. Oriental Medical Treatment of Lumbar Spinal Stenosis

    Directory of Open Access Journals (Sweden)

    Hae-Yeon Lee

    2003-12-01

    Full Text Available Lumbar spinal stenosis results from the progressive combined narrowing of the central spinal canal, the neurorecesses, and the neuroforaminal canals. In the absence of prior surgery, tumor, or infection, the spinal canal may become narrowed by bulging or protrusion of the intervertebral disc annulus, herniation of the nucleus pulposis posteriorly, thickening of the posterior longitudinal ligament, hypertrophy of the ligamentum flavum, epidural fat deposition, spondylosis of the intervertebral disc margins, or a combination of two or more of the above factors. Patients with spinal stenosis become symptomatic when pain, motor weakness, paresthesia, or other neurologic compromise causes distress. In one case, we administrated oriental medical treatment with acupuncture treatment and herb-medicine. Oriental medical treatment showed desirable effect on lumbar spinal stenosis.

  5. Percutaneous Valvuloplasty for Bioprosthetic Tricuspid Valve Stenosis

    Science.gov (United States)

    Malhotra, Rohit; Sharma, Anjali; Kakouros, Nikolaos

    2017-01-01

    Percutaneous transcatheter tricuspid balloon valvuloplasty (PTTBV) is an accepted treatment option for symptomatic severe native tricuspid valve stenosis, although surgical tricuspid valve replacement remains the treatment of choice. There have been few reports of successful PTTBV for bioprosthetic tricuspid valve stenosis. We present case reports of 3 patients from our hospital experience. Two of the 3 cases were successful, with lasting clinical improvement, whereas the 3rd patient failed to show a reduction in valve gradient. We describe the standard technique used for PTTBV. We present results from a literature review that identified 16 previously reported cases of PTTBV for bioprosthetic severe tricuspid stenosis, with overall favorable results. We conclude that PTTBV should perhaps be considered for a select patient population in which symptomatic improvement and hemodynamic stability are desired immediately, and particularly for patients who are inoperable or at high surgical risk.

  6. Asymptomatic internal carotid artery stenosis and cerebrovascular risk stratification

    DEFF Research Database (Denmark)

    Nicolaides, Andrew N; Kakkos, Stavros K; Kyriacou, Efthyvoulos

    2010-01-01

    The purpose of this study was to determine the cerebrovascular risk stratification potential of baseline degree of stenosis, clinical features, and ultrasonic plaque characteristics in patients with asymptomatic internal carotid artery (ICA) stenosis....

  7. Questions and Answers about Treating Arterial Stenosis and Preventing Stroke

    Science.gov (United States)

    ... and Answers About Treating Arterial Stenosis and Preventing Stroke A stroke is any sudden event affecting the ... and high cholesterol. How does stenosis contribute to stroke? Atherosclerosis can activate cells involved in blood clotting. ...

  8. Recurred Post-intubation Tracheal Stenosis Treated with Bronchoscopic Cryotherapy

    Science.gov (United States)

    Jung, Ye-Ryung; Taek Jeong, Joon; Kyu Lee, Myoung; Kim, Sang-Ha; Joong Yong, Suk; Jeong Lee, Seok; Lee, Won-Yeon

    2016-01-01

    Post-intubation tracheal stenosis accounts for the greatest proportion of whole-cause tracheal stenosis. Treatment of post-intubation tracheal stenosis requires a multidisciplinary approach. Surgery or an endoscopic procedure can be used, depending on the type of stenosis. However, the efficacy of cryotherapy in post-intubation tracheal stenosis has not been validated. Here, we report a case of recurring post-intubation tracheal stenosis successfully treated with bronchoscopic cryotherapy that had previously been treated with surgery. In this case, cryotherapy was effective in treating web-like fibrous stenosis, without requiring more surgery. Cryotherapy can be considered as an alternative or primary treatment for post-intubation tracheal stenosis. PMID:27853078

  9. Supravalvular aortic stenosis with sudden cardiac death

    Directory of Open Access Journals (Sweden)

    Pradeep Vaideeswar

    2015-01-01

    Full Text Available Sudden cardiac death (SCD most commonly results from previously undiagnosed congenital, acquired, or hereditary cardiac diseases. Congenital aortic valvular, subvalvular, and supravalvular disease with left ventricular outflow tract obstruction is an important preventable cause of sudden death. This report documents sudden death presumably due to acute myocardial ischemia in a young male with an undiagnosed supravalvular aortic stenosis (SVAS due to a rare association of isolation of coronary sinuses of Valsalva. Congenital supravalvular pulmonary stenosis and mitral valvular dysplasia were also present.

  10. Minimal Invasive Decompression for Lumbar Spinal Stenosis

    Directory of Open Access Journals (Sweden)

    Victor Popov

    2012-01-01

    Full Text Available Lumbar spinal stenosis is a common condition in elderly patients and may lead to progressive back and leg pain, muscular weakness, sensory disturbance, and/or problems with ambulation. Multiple studies suggest that surgical decompression is an effective therapy for patients with symptomatic lumbar stenosis. Although traditional lumbar decompression is a time-honored procedure, minimally invasive procedures are now available which can achieve the goals of decompression with less bleeding, smaller incisions, and quicker patient recovery. This paper will review the technique of performing ipsilateral and bilateral decompressions using a tubular retractor system and microscope.

  11. Asymptomatic internal carotid artery stenosis and cerebrovascular risk stratification

    DEFF Research Database (Denmark)

    Nicolaides, Andrew N; Kakkos, Stavros K; Kyriacou, Efthyvoulos;

    2010-01-01

    The purpose of this study was to determine the cerebrovascular risk stratification potential of baseline degree of stenosis, clinical features, and ultrasonic plaque characteristics in patients with asymptomatic internal carotid artery (ICA) stenosis.......The purpose of this study was to determine the cerebrovascular risk stratification potential of baseline degree of stenosis, clinical features, and ultrasonic plaque characteristics in patients with asymptomatic internal carotid artery (ICA) stenosis....

  12. Studies on diagnosis and treatment of renal artery stenosis

    NARCIS (Netherlands)

    P. Krijnen (Pieta)

    2004-01-01

    textabstractThis thesis describes studies on ~onosis and treatment of renal artery stenosis in patients with drug-resistant hypertension. In Chapter 1, the clinical problem of renal artery stenosis is discussed. Renal artery stenosis, a narrowing of the renal artery, is a potential cause of

  13. A Tension-Based Model Distinguishes Hypertrophic versus Dilated Cardiomyopathy.

    Science.gov (United States)

    Davis, Jennifer; Davis, L Craig; Correll, Robert N; Makarewich, Catherine A; Schwanekamp, Jennifer A; Moussavi-Harami, Farid; Wang, Dan; York, Allen J; Wu, Haodi; Houser, Steven R; Seidman, Christine E; Seidman, Jonathan G; Regnier, Michael; Metzger, Joseph M; Wu, Joseph C; Molkentin, Jeffery D

    2016-05-19

    The heart either hypertrophies or dilates in response to familial mutations in genes encoding sarcomeric proteins, which are responsible for contraction and pumping. These mutations typically alter calcium-dependent tension generation within the sarcomeres, but how this translates into the spectrum of hypertrophic versus dilated cardiomyopathy is unknown. By generating a series of cardiac-specific mouse models that permit the systematic tuning of sarcomeric tension generation and calcium fluxing, we identify a significant relationship between the magnitude of tension developed over time and heart growth. When formulated into a computational model, the integral of myofilament tension development predicts hypertrophic and dilated cardiomyopathies in mice associated with essentially any sarcomeric gene mutations, but also accurately predicts human cardiac phenotypes from data generated in induced-pluripotent-stem-cell-derived myocytes from familial cardiomyopathy patients. This tension-based model also has the potential to inform pharmacologic treatment options in cardiomyopathy patients.

  14. 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.

  15. 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. PMID:24082380

  16. Hypertrophic Obstructive Cardiomyopathy and Takotsubo Syndrome: Could They Coexist?

    Directory of Open Access Journals (Sweden)

    Egea-Serrano

    2015-11-01

    Full Text Available Introduction Takotsubo syndrome (TKS is generally caused by a stressful condition, and it usually has a good prognosis after the recovery of left ventricular function. About 70% of the cases of hypertrophic cardiomyopathy may develop obstruction in the left ventricular outflow tract (LVOT, which is responsible for heart failure. Case Presentation We present a unique case where TKS occurred in a middle-aged male patient with hypertrophic obstructive cardiomyopathy (HOCM without a clearly identifiable initial stress trigger. Conclusions In the setting of acute left ventricular function depression in HOCM, a comprehensive differential diagnosis should be established. Treatment should be based on hemodynamic changes. After recovery, the prognosis is related to HOCM.

  17. Hypertrophic Neuropathy Secondary to Hansen's Disease. A Case Report

    Directory of Open Access Journals (Sweden)

    María Octavina Rodríguez Roque

    2016-08-01

    Full Text Available We present the case 49-year-old unmarried woman treated at the hospital of Cienfuegos. The patient complained of weakness in the lower limbs and "numbness" (paresthesia of all four extremities, which improved slightly after treatment. A year later, the muscle weakness and paresthesia worsened, particularly in the upper limbs. She also developed hypopigmented skin lesions on the chest and abdomen. The investigation was initiated due to suspicion of hypertrophic polyneuropathy. A biopsy of the hypochromic lesions was performed, which led to the conclusion that the patient suffered from a hypertrophic polyneuropathy secondary to tuberculoid Hansen's disease. We decided to present this case since a neuropathy is usually slow, insidious and has a long course in these patients; however, leprosy reactions can lead to acute nerve damage, resulting in disabilities and deformities. Consequently, it is important to act quickly in order to avoid them.

  18. 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)

  19. Cardiac troponin T mutations in Chinese patients with hypertrophic cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    吴恒芳; 杨笛; 万文辉; 卞智萍; 徐晋丹; 马文珠; 张寄南

    2004-01-01

    @@ Hypertrophic cardiomyopathy (HCM) is a myocardial disorder characterized by unexplained ventricular hypertrophy and myofibrillar disarray, with a prevalence of about 0.2% in general population. HCM is associated with gene abnormalities. Nearly 200 mutations have been described in ten genes in patients with HCM.1 Cardiac troponin T (cTnT) is an essential component of the troponin complex and plays a central role in the calcium regulation of contractions in cardiac myocytes

  20. Hypertrophic Cardiomyopathy Complicated by Pulmonary Edema in the Postpartum Period

    Directory of Open Access Journals (Sweden)

    Kate Hanneman

    2013-01-01

    Full Text Available We report the case of a 42-year-old patient with hypertrophic cardiomyopathy (HCM who presented to the emergency department with severe shortness of breath one week following uneventful cesarean delivery. Thoracic CT ruled out pulmonary embolus and confirmed pulmonary edema. Asymmetric interventricular septal thickening was clearly identified, demonstrating that the heart may be evaluated even on a non-ECG gated study. Acute pulmonary edema in the postpartum period is an unusual clinical presentation of HCM.

  1. Esophageal duplication and congenital esophageal stenosis.

    Science.gov (United States)

    Trappey, A Francois; Hirose, Shinjiro

    2017-04-01

    Esophageal duplication and congenital esophageal stenosis (CES) may represent diseases with common embryologic etiologies, namely, faulty tracheoesophageal separation and differentiation. Here, we will re-enforce definitions for these diseases as well as review their embryology, diagnosis, and treatment. Copyright © 2017. Published by Elsevier Inc.

  2. Severe aortic valve stenosis and nosebleed

    NARCIS (Netherlands)

    Schoedel, Johannes; Obergfell, Achim; Maass, Alexander H.; Schodel, Johannes

    2007-01-01

    Aortic valve stenosis is known to be associated with loss of high molecular von Willebrand multimers. This can lead to gastrointestinal bleeding in patients with gastrointestinal angiodysplasia, the Heyde syndrome. Here we present a case of anaemia and severe epistaxis associated with acquired von W

  3. Duplex ultrasound for identifying renal artery stenosis

    DEFF Research Database (Denmark)

    Zachrisson, Karin; Herlitz, Hans; Lönn, Lars

    2017-01-01

    Background Renal artery duplex ultrasound (RADUS) is an established method for diagnosis of renal artery stenosis (RAS), but there is no consensus regarding optimal RADUS criteria. Purpose To define optimal cutoff values for RADUS parameters when screening for RAS using intra-arterial trans...

  4. Developmental spinal canal stenosis and somatotype.

    OpenAIRE

    Nightingale, S.

    1989-01-01

    The hypothesis that somatotype and cervical spine developmental canal stenosis may be associated has been investigated by anthropometry and measurement of lateral projection cervical spine radiographs. A significant association of canal size with somatotype has been found such that those with developmentally narrow canals are more likely to have relatively shorter long-bones, particularly in the upper arm, and longer trunks.

  5. Infundibulopelvic stenosis - evaluation of diagnostic imaging

    Energy Technology Data Exchange (ETDEWEB)

    Schneider, K.; Martin, W.; Fendel, H.; Helmig, F.J.

    1988-08-01

    Infundibulopelvic stenosis is a very rare kidney disease. This congenital disorder must be differentiated from multicystic dysplastic kidney, polycystic kidney disease, simple renal cysts and mega-(poly)-calicosis. Associated abnormalities of the ureter are rare. Ureteric obstruction was associated in our two patients, and in one case agenesis of the bladder and a duplicated genital tract were also present.

  6. Stent treatment of symptomatic intracranial arterial stenosis

    Directory of Open Access Journals (Sweden)

    DONG Feng-ju

    2012-08-01

    Full Text Available Objective To investigate the safety, feasibility and efficacy of the Wingspan stent in treatment of symptomatic intracranial arterial stenosis. Methods Wingspan stenting was applied in 90 cases with symptomatic intracranial arterial stenosis. The surgical success rate and periprocedural neurological complications were observed. Digital subtraction angiography was performed at 6 months after stenting to detect the occurrence of restenosis. Results The technical success rate was 98.92% (92/93. Pre-treatment stenosis (83.42 ± 9.53% was improved to (21.82 ± 9.86% after stent placement. The difference was statistically significant (t = 3.280, P = 0.002. There were 5 patients (5.56% occurred major periprocedural neurological complications, 3 of them died within 30 days after the procedure. The restenosis rate at 6 months after operation was 19.10% (17/89. Conclusion Symptomatic intracranial arterial stenosis can be treated by Wingspan stenting with high technical success rate. The occurrence of complication is low and short-term efficacy is good. However, further study is needed to investigate long-term effect.

  7. Noncardiac surgery in patients with aortic stenosis

    DEFF Research Database (Denmark)

    Andersson, Charlotte; Jørgensen, Mads Emil; Martinsson, Andreas

    2014-01-01

    BACKGROUND: Past research has identified aortic stenosis (AS) as a major risk factor for adverse outcomes in noncardiac surgery; however, more contemporary studies have questioned the grave prognosis. To further our understanding of this, the risks of a 30-day major adverse cardiovascular event (...

  8. 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.

  9. Hypertrophic cranial pachymeningitis in a patient with aplastic anemia.

    Science.gov (United States)

    Asano, T; Hayashida, M; Ogawa, K; Adachi, K; Teramoto, A; Yamamoto, M

    1998-12-01

    We report on a 13-year old girl with severe aplastic anemia and hypertrophic cranial pachymeningitis. She was admitted to our hospital with severe headache and vomiting. A computerized tomographic (CT) scan of the brain on the third day of symptoms showed a hyperdense area in the tentorial region. Magnetic resonance imaging (MRI) showed iso-intensity in the same tentorial region in T1- and T2-weighted images, and gadolinium enhancement of this region suggested a thickened dura mater. Initially, a diagnosis of subdural or subarachnoid hemorrhage was made. Since her platelet count was low (3000/microl) making the patient a poor-risk candidate for surgery, and the area was limited to the dura mater, conservative therapy, including glycerol administration and platelet transfusion, was carried out. Despite clinical improvement 10 days after admission without specific therapy, the iso-intense region on the left side of the tentorial region remained unchanged on MRI. On the other hand, the iso-intense area on the right side of the tentorial region became hyperdense on T1-weighted MRI images and was also enhanced by gadolinium. Cerebrospinal fluid findings were normal except for slightly elevated protein at 62 mg/dl. A diagnosis of hypertrophic cranial pachymeningitis of the tentorial dura mater with hemorrhage on the right side was made. Although hypertrophic cranial pachymeningitis is a rare disease, it must be considered in the differential diagnosis of severe headache in a case of aplastic anemia.

  10. Hypertrophic osteopathy associated with systemic granulomatous disease in a horse

    Directory of Open Access Journals (Sweden)

    Liomara Andressa do Amaral Kwirant

    2016-04-01

    Full Text Available A 4-year-old Criollo stallion was presented at the equine clinic of veterinary hospital of the Federal University of Santa Maria, RS, with a 30-day history of progressive weight loss, anemia and swelling of the forelimbs and face. Physical examination revealed that the swelling was firm and had a bone-like consistency, also radiographs showed extensive periosteal proliferation on the forelimb long bones that suggested hypertrophic osteopathy (Marie´s disease. Physical examinations identified no respiratory findings. However, during ultrasound examination, superficial lung disease was identified. The animal was treated with antibiotics and nonsteroidal anti-inflammatory drugs for 12 days. Due to a complete lack of response to this treatment, the horse was euthanized. At necropsy several granulomatous lesions were identified in the thorax, abdomen and testicular tunics. Bony proliferation was evident on many bones of the appendicular skeleton and face. Based on these findings the diagnosis of hypertrophic osteopathy associated with sarcoidosis was established. It is important to perform a thorough clinical examination and include hypertrophic osteopathy in the differential diagnosis of diseases that are accompanied by swelling of the face and limbs as edema from various causes, fibrous osteodystrophy, for example. Key words: Pulmonary. Periosteal. Sarcoidosis. Pathology

  11. Surgical outcomes and strategy of hypertrophic obstructive cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    ZHU Ya-bin; RAJAN S.; KURIAN V.M.; LIU Zhi-yong

    2006-01-01

    Objective: To evaluate the surgical clinical results of hypertrophic obstructive cardiomyopathy. Methods: We retrospectively collected data on 24 patients who underwent surgical management in the past ten years in two hospitals in China and Madras Medical Mission in India. Myomectomy was carried out on all patients. Among them 3 patients underwent mitral valve replacement; 2 patients underwent mitral valve repair (anterior mitral leaflet plication); 2 patients underwent aortic valve replacement; 1 patient underwent aortic valve repair; 2 patients underwent aortic root replacement; 1 patient underwent Bentall's procedure and 1 patient underwent coronary artery bypass grafting because of a breached muscle bridge. Results: One patient died of post-operative heart failure. The mean follow-up time was 4.3 years. There was significant improvement in the symptomatic status. Sixteen patients were asymptomatic with good effort tolerance and only four patients had New York heart association (NYHA) Classes Ⅰ~Ⅱ due to associated valvular lesions. Conclusion: Our experience proved that symptomatic hypertrophic obstructive cardiomyopathy or non-symptomatic hypertrophic obstructive cardiomyopathy with combined heart disease is indication for surgery as surgical intervention could get better clinical results in this kind of patients compared with other non-surgical method because it beneficially reduces the systolic anterior motion (SAM) of the mitral valve leaflet, which could not be avoided by other non-surgical treatment.

  12. Age-dependent heterogeneity of familiar hypertrophic cardiomyopathy phenotype: a role of cardiovascular magnetic resonance.

    Science.gov (United States)

    Glaveckaitė, Sigita; Rudys, Alfredas; Mikštienė, Violeta; Valevičienė, Nomeda; Palionis, Darius; Laucevičius, Aleksandras

    2013-01-01

    In this case report, we present familiar hypertrophic cardiomyopathy with age-dependent heterogeneity of the disease phenotype among the members of one family who carry the same mutation of the myosin-binding protein C gene. Phenotypic heterogeneity is common in patients with familial forms of hypertrophic cardiomyopathy, both in clinical expression and outcome. Compared with other noninvasive cardiac imaging modalities, cardiovascular magnetic resonance provides an opportunity to more accurately characterize the varying phenotypic presentations of hypertrophic cardiomyopathy.

  13. Balloon dilation of congenital supravalvular pulmonic stenosis in a dog

    Science.gov (United States)

    Treseder, Julia R.

    2017-01-01

    Percutaneous balloon valvuloplasty is considered the standard of care for treatment of valvular pulmonic stenosis, a common congenital defect in dogs. Supravalvular pulmonic stenosis is a rare form of pulmonic stenosis in dogs and standard treatment has not been established. Although, there have been reports of successful treatment of supravalvular pulmonic stenosis with surgical and stenting techniques, there have been no reports of balloon dilation to treat dogs with this condition. Here, a case of supravalvular pulmonic stenosis diagnosed echocardiographically and angiographically in which a significant reduction in pressure gradient was achieved with balloon dilation alone is presented. PMID:27297421

  14. Aortic stenosis: From diagnosis to optimal treatment

    Directory of Open Access Journals (Sweden)

    Tavčiovski Dragan

    2008-01-01

    Full Text Available Aortic stenosis is the most frequent valvular heart disease. Aortic sclerosis is the first characteristic lesion of the cusps, which is considered today as the process similar to atherosclerosis. Progression of the disease is an active process leading to forming of bone matrix and heavily calcified stiff cusps by inflammatory cells and osteopontin. It is a chronic, progressive disease which can remain asymptomatic for a long time even in the presence of severe aortic stenosis. Proper physical examination remains an essential diagnostic tool in aortic stenosis. Recognition of characteristic systolic murmur draws attention and guides further diagnosis in the right direction. Doppler echocardiography is an ideal tool to confirm diagnosis. It is well known that exercise tests help in stratification risk of asymptomatic aortic stenosis. Serial measurements of brain natriuretic peptide during a follow-up period may help to identify the optimal time for surgery. Heart catheterization is mostly restricted to preoperative evaluation of coronary arteries rather than to evaluation of the valve lesion itself. Currently, there is no ideal medical treatment for slowing down the disease progression. The first results about the effect of ACE inhibitors and statins in aortic sclerosis and stenosis are encouraging, but there is still not enough evidence. Onset symptoms based on current ACC/AHA/ESC recommendations are I class indication for aortic valve replacement. Aortic valve can be replaced with a biological or prosthetic valve. There is a possibility of percutaneous aortic valve implantation and transapical operation for patients that are contraindicated for standard cardiac surgery.

  15. Valvular Aortic Stenosis: A Proteomic Insight

    Directory of Open Access Journals (Sweden)

    Fernando Vivanco

    2010-02-01

    Full Text Available Calcified aortic valve disease is a slowly progressive disorder that ranges from mild valve thickening with no obstruction of blood flow, known as aortic sclerosis, to severe calcification with impaired leaflet motion or aortic stenosis. In the present work we describe a rapid, reproducible and effective method to carry out proteomic analysis of stenotic human valves by conventional 2-DE and 2D-DIGE, minimizing the interference due to high calcium concentrations. Furthermore, the protocol permits the aortic stenosis proteome to be analysed, advancing our knowledge in this area. Summary: Until recently, aortic stenosis (AS was considered a passive process secondary to calcium deposition in the aortic valves. However, it has recently been highlighted that the risk factors associated with the development of calcified AS in the elderly are similar to those of coronary artery disease. Furthermore, degenerative AS shares histological characteristics with atherosclerotic plaques, leading to the suggestion that calcified aortic valve disease is a chronic inflammatory process similar to atherosclerosis. Nevertheless, certain data does not fit with this theory making it necessary to further study this pathology. The aim of this study is to develop an effective protein extraction protocol for aortic stenosis valves such that proteomic analyses can be performed on these structures. In the present work we have defined a rapid, reproducible and effective method to extract proteins and that is compatible with 2-DE, 2D-DIGE and MS techniques. Defining the protein profile of this tissue is an important and challenging task that will help to understand the mechanisms of physiological/pathological processes in aortic stenosis valves.

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

    National Research Council Canada - National Science Library

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

    2008-01-01

    .... In 97 hypertrophic cardiomyopathy families with a sarcomere gene mutation we retrospectively determined uptake of genetic counselling and predictive DNA testing in relatives within 1 year after...

  17. Hypertrophic Olivary Degeneration and Palatal or Oculopalatal Tremor

    Directory of Open Access Journals (Sweden)

    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

  18. Stroke in Patients With Aortic Stenosis

    DEFF Research Database (Denmark)

    Greve, Anders Møller; Dalsgaard, Morten; Bang, Casper N

    2014-01-01

    BACKGROUND AND PURPOSE: There are limited data on risk stratification of stroke in aortic stenosis. This study examined predictors of stroke in aortic stenosis, the prognostic implications of stroke, and how aortic valve replacement (AVR) with or without concomitant coronary artery bypass grafting......, and poststroke survival a secondary outcome. Cox models treating AVR as a time-varying covariate were adjusted for atrial fibrillation and congestive heart failure, hypertension, age≥75 years, diabetes mellitus, stroke/transient ischemic attack, vascular disease, age 65-74 years and female sex (CHA2DS2-VASc......) scores. RESULTS: One thousand five hundred nine patients were followed for 4.3±0.8 years (6529 patient-years). Rates of stroke were 5.6 versus 21.8 per 1000 patient-years pre- and post-AVR; 429 (28%) underwent AVR and 139 (9%) died. Atrial fibrillation (hazard ratio [HR], 2.7; 95% confidence interval [CI...

  19. Punctal stenosis: definition, diagnosis, and treatment

    Directory of Open Access Journals (Sweden)

    Soiberman U

    2012-07-01

    Full Text Available Uri Soiberman,1 Hirohiko Kakizaki,2 Dinesh Selva,3 Igal Leibovitch11Division of Oculoplastic and Orbital Surgery, Department of Ophthalmology, Tel-Aviv Medical Center, Tel-Aviv University, Tel-Aviv, Israel; 2Department of Ophthalmology, Aichi Medical University, Nagakute, Japan; 3South Australian Institute of Ophthalmology and Discipline of Ophthalmology and Visual Sciences, University of Adelaide, South Australia, AustraliaAbstract: Acquired punctal stenosis is a condition in which the external opening of the lacrimal canaliculus is narrowed or occluded. This condition is a rare cause of symptomatic epiphora, but its incidence may be higher in patients with chronic blepharitis, in those treated with various topical medications, including antihypertensive agents, and especially in patients treated with taxanes for cancer. The purpose of this review is to cover the medical literature, focusing in particular on definition, incidence, risk factors, etiology and treatment options.Keywords: acquired punctal stenosis, definition, epiphora, etiology, treatment

  20. 无X线监视内镜下置入幽门支架治疗胃出口恶性梗阻36例%Treatment of malignant gastric outlet obstruction by endoscopically implanting pyloric stents without fluoroscopic guidance: an analysis of 36 cases

    Institute of Scientific and Technical Information of China (English)

    吴齐; 李士杰; 曹长琦; 张集昌

    2011-01-01

    AIM: To evaluate the feasibility, efficacy, and complications of endoscopic placement of pyloric stents without fluoroscopic guidance in patients with malignant gastric outlet obstruction.METHODS: The clinical data for 36 patients with malignant gastric outlet obstruction who underwent endoscopic placement of pyloric stents between January 2007 and December 2009 were analyzed retrospectively.RESULTS: Thirty-six patients received a total of 39 stents, one stent each in 33 patients and two stents each in three patients.In seven patients with high-grade stenosis, endoscopic balloon dilatation was performed before stent insertion.Successful stent implantation was achieved in all patients.The gastric outlet obstruction score system (GOOSS) score was significantly increased within 1 and 4 weeks after stent placement (P = 0.000).The mean duration was increased 101.3 days (7-380 days).The mean survival time was 123.3 days (10-380 days).Device-related adverse events included stent migration (n = 2, day 20 and day 65), stent occlusion caused by tumor infiltration (n = 2), and acute cholangitis (n = 1,day 7).No hemorrhage or perforation occurred.CONCLUSION: Endoscopic implantation of pyloric stents without fluoroscopic guidance is a simple, safe and effective method for malignant gastric outlet obstruction.%目的:评价无X线监视内镜下置入幽门支架治疗胃出口恶性梗阻的操作技术、临床疗效及并发症.方法:对2007-01/2009-12接受无X线监视内镜下幽门支架置入治疗的36例胃出口恶性梗阻患者的临床资料进行回顾性分析.结果:36例患者共置入39枚支架,其中3例患者为双支架.7例患者因病变狭窄程度高,先行内镜下球囊扩张,再行支架置入.支架置入成功率100%.患者术后1 wk及4 wk的GOOSS评分高于术前,且差异具有统计学意义(P=0.000).患者GOOSS评分高于术前时间平均为101.3(7-380)d.患者平均生存期123.3(10-380)d.2例患者发生术后支架移位(20 d及65 d).2

  1. Ultrasonographic and surgical findings of a gastric hyperplastic polyp resulting in pyloric obstruction in an 11-week-old French Bulldog.

    Science.gov (United States)

    Kuan, S; Hoffmann, K; Tisdall, P

    2009-06-01

    An 11-week-old male entire French Bulldog was presented with a 3-week history of projectile vomiting after eating that was unresponsive to medical therapy. Ultrasonographic examination revealed a 1 x 2 cm pedunculated polypoid mass in the pyloric antrum. Histopathology showed this to be comprised of a pedunculated hyperplastic proliferation of gastric mucosal epithelium and submucosa, with dilatation of the glandular pits and a moderate degree of submucosal fibrosis and small amount of smooth muscle hyperplasia in the submucosa. The tunica muscularis was not involved in the polyp and appeared to be of normal thickness. Surgical treatment by pylorectomy and end-to-end gastroduodenostomy (Billroth I) was successful.

  2. An Unusual Cause of Rectal Stenosis

    Directory of Open Access Journals (Sweden)

    Maja Gruber

    2016-08-01

    Full Text Available Solitary rectal ulcer syndrome (SRUS is a benign disease that is often misdiagnosed. It is characterized by a combination of symptoms, endoscopic findings and histology. Patients present with constipation, rectal bleeding, mucous discharge, pain and a sensation of incomplete defecation. There are many different manifestations of this disease, with or without rectal prolapse. We report an unusual presentation of SRUS as a circular stenosis in a middle-aged male.

  3. Punctal stenosis: definition, diagnosis, and treatment

    OpenAIRE

    Soiberman U; Kakizaki H; Selva D; Leibovitch I

    2012-01-01

    Uri Soiberman,1 Hirohiko Kakizaki,2 Dinesh Selva,3 Igal Leibovitch11Division of Oculoplastic and Orbital Surgery, Department of Ophthalmology, Tel-Aviv Medical Center, Tel-Aviv University, Tel-Aviv, Israel; 2Department of Ophthalmology, Aichi Medical University, Nagakute, Japan; 3South Australian Institute of Ophthalmology and Discipline of Ophthalmology and Visual Sciences, University of Adelaide, South Australia, AustraliaAbstract: Acquired punctal stenosis is a condition in which the exter...

  4. Punctal stenosis: definition, diagnosis, and treatment

    Science.gov (United States)

    Soiberman, Uri; Kakizaki, Hirohiko; Selva, Dinesh; Leibovitch, Igal

    2012-01-01

    Acquired punctal stenosis is a condition in which the external opening of the lacrimal canaliculus is narrowed or occluded. This condition is a rare cause of symptomatic epiphora, but its incidence may be higher in patients with chronic blepharitis, in those treated with various topical medications, including antihypertensive agents, and especially in patients treated with taxanes for cancer. The purpose of this review is to cover the medical literature, focusing in particular on definition, incidence, risk factors, etiology and treatment options. PMID:22848141

  5. Post intubation tracheal stenosis in children

    Directory of Open Access Journals (Sweden)

    Marco Caruselli

    2014-12-01

    Full Text Available Many authors have reported that tracheal stenosis is a complication that can follow tracheal intubation in both adults and children. The symptoms, when they do appear, can be confused with asthma, with subsequent treatment providing only mild and inconsistent relief. We report here the case of an 8 year old girl admitted to our hospital for whooping cough that was not responding to therapy.

  6. Male sex aggravates the phenotype in mouse models of hypertrophic cardiomyopathy

    NARCIS (Netherlands)

    Maass, AH; Maier, SKG

    2005-01-01

    The authors have created transgenic mouse models of hypertrophic cardiomyopathy with mutations in either cardiac troponin T or myosin heavy chain. Mice mutant in myosin heavy chain develop significant cardiac hypertrophy at young adult age. Female mice keep that hypertrophic state, whereas male mice

  7. Histochemical evidences on the chronological alterations of the hypertrophic zone of mandibular condylar cartilage.

    Science.gov (United States)

    Hossain, Kazi Sazzad; Amizuka, Norio; Ikeda, Nobuyki; Nozawa-Inoue, Kayoko; Suzuki, Akiko; Li, Minqi; Takeuchi, Kiichi; Aita, Megumi; Kawano, Yoshiro; Hoshino, Masaaki; Oda, Kimimitsu; Takagi, Ritsuo; Maeda, Takeyasu

    2005-08-15

    The hypertrophic chondrocytes lack the ability to proliferate, thus permitting matrix mineralization as well as vascular invasion from the bone in both the mandibular condyle and the epiphyseal cartilage. This study attempted to verify whether the histological appearance of the hypertrophic chondrocytes is in a steady state during postnatal development of the mouse mandibular condyle. Type X collagen immunohistochemistry apparently distinguished the fibrous layer described previously as the "articular zone," "articular layer," and "resting zone" from the hypertrophic zone. Interestingly, the ratio of the type X collagen-positive hypertrophic zone in the entire condyle seemed higher in the early stages but decreased in the later stages. Some apparently compacted cells in the hypertrophic zone showed proliferating cell nuclear antigen (PCNA) immunoreaction, indicating the potential for cell proliferation at the early stages. As the mice matured, in contrast, they further enlarged and assumed typical features of hypertrophic chondrocytes. Apoptotic cells were also discernible in the hypertrophic zone at the early but not later stages. Consistent with morphological configurations of hypertrophic chondrocytes, immunoreactions for alkaline phosphatase, osteopontin, and type I collagen were prominent at the later stage, but not the early stage. Cartilaginous matrices demonstrated scattered patches of mineralization at the early stage, but increased in their volume and connectivity at the later stage. Thus, the spatial and temporal occurrence of these immunoreactions as well as apoptosis likely reflect the prematurity of hypertrophying cells at the early stage, and imply a physiological relevance during the early development of the mandibular condyles.

  8. 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;

    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...

  9. Male sex aggravates the phenotype in mouse models of hypertrophic cardiomyopathy

    NARCIS (Netherlands)

    Maass, AH; Maier, SKG

    2005-01-01

    The authors have created transgenic mouse models of hypertrophic cardiomyopathy with mutations in either cardiac troponin T or myosin heavy chain. Mice mutant in myosin heavy chain develop significant cardiac hypertrophy at young adult age. Female mice keep that hypertrophic state, whereas male mice

  10. Pressure therapy upregulates matrix metalloproteinase expression and downregulates collagen expression in hypertrophic scar tissue

    Institute of Scientific and Technical Information of China (English)

    HUANG Dong; SHEN Kuan-hong; WANG Hong-gang

    2013-01-01

    Background Pressure therapy improves hypertrophic scar healing,but the mechanisms for this process are not well understood.We sought to investigate the differential expression of matrix metalloproteinases (Mmps) and collagen in posttraumatic hypertrophic scar tissue with mechanical pressure and delineate the molecular mechanisms of pressure therapy for hypertrophic scars.Methods Fibroblast lines of normal skin and scar tissue were established and a mechanical pressure system was devised to simulate pressure therapy.Reverse transcription-polymerase chain reaction (RT-PCR) and Western blotting assays were used to compare differences in the mRNA and protein expression of Mmps and collagen in scar fibroblasts before and after pressure therapy.Results The expression differed between the hypertrophic scar cell line and the normal cell line.RT-PCR assays showed that Collagen I,highly expressed in the hypertrophic scar cell line,decreased significantly after pressure therapy.Mmp2,Mmp9,and Mmp12 expression in the hypertrophic scar tissue increased significantly after pressure therapy (P <0.05).Western blotting assays further revealed that Mmp9 and Mmp12 expression increased significantly in the hypertrophic scar tissue after pressure therapy (P <0.05) but not Mmp2 expression (P >0.05).Conclusion Mechanical pressure induces degradation of Collagen Ⅰ in hypertrophic scar tissue by affecting the expression of Mmp9 and Mmp12.

  11. [Idiopathic Progressive Subglottic Stenosis: Surgical Techniques].

    Science.gov (United States)

    Hoetzenecker, K; Schweiger, T; Klepetko, W

    2016-09-01

    Idiopathic subglottic stenosis is a disease characterized by slow, progressive scarring and constriction of the subglottic airway. It almost always occurs in females between the 3rd and 5th decade of life. Symptoms are frequently misinterpreted as asthma and patients are referred for endoscopic evaluation only when asthma medications fail to alleviate their symptoms. Treatment options can be divided into endoscopic and open surgical techniques. Microlaryngoscopic scar reduction by laser followed by balloon dilation usually delivers good short-term results. However, the majority of patients will experience restenosis within a short period of time. Open surgical correction techniques are based on a complete removal of the affected airway segment. This must be combined with various extended resection techniques in patients with advanced stenosis. Depending on the extent and severity of the stenosis the following surgical techniques are required: standard cricotracheal resection (Grillo's technique), cricoplasty with dorsal and lateral mucosaplasty, or a combination of resection and enlargement techniques using rib cartilage grafts. In experienced centres, success rates of over 95 % are reported with good functional outcome of voice and deglutition.

  12. [A clinical study of hypertrophic obstructive cardiomyopathy in the elderly].

    Science.gov (United States)

    Chida, K; Ohkawa, S; Maeda, S; Kuboki, K; Imai, T; Sakai, M; Watanabe, C; Matsushita, S; Ueda, K; Kuramoto, K

    1990-09-01

    Seven elderly patients with hypertrophic obstructive cardiomyopathy (HOCM), who had the three following characteristics on echocardiograms 1) extremely thickened septum, 2) systolic anterior motion of the mitral valve, 3) mid systolic semi-closure of the aortic valve, were clinically evaluated. Ages ranged from 73 to 86 years old (average 78.9% yr.) and all were women. None had not a family history of hypertrophic cardiomyopathy but they had mild hypertension. Six patients showed a significant high voltage on the ST-segment and T-wave abnormalities ("strain" pattern). The left ventricular posterior wall as well as the septum was thickened in 5 and the remaining 2 showed asymmetrical septal hypertrophy (ASH) on echocardiograms. The left ventricular cavity was narrowed due to left ventricular hypertrophy and the shape of the left ventricular cavity was ovoid in all patients. The aorto-septal angles in these 7 patients were 80 degrees to 120 degrees. In addition, proximal septal bulge in all and anterior displacement of the mitral posterior leaflet due to the mitral ring calcification (MRC) in some patients contributed to the narrowing of the left ventricular outflow tract, and the mitral valve was pulled up toward the septum because of the good left ventricular systolic function (ejection fraction: 70 to 94% by echocardiography) and blood was ejected at a high velocity through a narrowed outflow tract (Venturi effect). Pressure gradients in the left ventricular outflow tract was 38 to 146 mmHg in 5 examined by cardiac catheterization. Biopsy specimens were obtained from 2 patients, showing hypertrophic myocytes (diameter: 20 to 30 micron) in 2 and mild disarray in 1.(ABSTRACT TRUNCATED AT 250 WORDS)

  13. Recent progress in end-stage hypertrophic cardiomyopathy.

    Science.gov (United States)

    Xiao, Yan; Yang, Kun-Qi; Jiang, Yong; Zhou, Xian-Liang

    2015-05-01

    Within the diverse spectrum of hypertrophic cardiomyopathy (HCM), a unique subgroup characterized by left ventricular enlargement and systolic dysfunction has emerged (defined as end-stage HCM [ES-HCM]). This underestimated entity provides challenging treatment strategies for extremely high risk of refractory heart failure and sudden cardiac death. Over the last 2 decades, the clinical features of ES-HCM have expanded and the underlying mechanisms gradually elucidated. Moreover, there is increasing evidence for early recognition of ES-HCM. New insights into early prevention and management will improve the clinical outcomes of this entity.

  14. Radiotherapy for extreme hypertrophic pulmonary osteoarthropathy associated with malignancy

    Energy Technology Data Exchange (ETDEWEB)

    Yeo, W.; Leung, S.F.; Chan, A.T.C.; Chiu, K.W. [Prince of Wales Hospital, Shatin (Hong Kong)

    1996-11-01

    A patient with florid hypertrophic pulmonary osteoarthropathy (HPOA) associated with metastatic nasopharyngeal carcinoma is presented. Despite the presence of metastatic disease in the thorax and in bone, the patient`s main symptom was severe pain from the HPOA, which was temporarily relieved by chemotherapy. Her disease subsequently progressed during chemotherapy and the pain became resistant to conventional treatment, including high dose morphine, non-steroidal anti-inflammatory agents and steroids. It was only with local radiation to the involved joints that the pain could be controlled. Our patient demonstrates that local radiotherapy is an option for the palliation of extreme HPOA. (author).

  15. 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.

  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

    The gene family KCNE1-5, which encode modulating β-subunits of several repolarising K+-ion channels, has been associated with genetic cardiac diseases such as long QT syndrome, atrial fibrillation and Brugada syndrome. The minK peptide, encoded by KCNE1, is attached to the Z-disc of the sarcomere...... 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...

  17. Effective Response of Methotrexate for Recurrent Idiopathic Hypertrophic Spinal Pachymeningitis

    Science.gov (United States)

    Park, Tae Joon; Seo, Won Deok; Kim, Sang Young; Cho, Jae Hoon; Kim, Dae Hyun

    2016-01-01

    Idiopathic hypertrophic spinal pachymeningitis (IHSP) is a chronic progressive and diffuse inflammatory fibrosis of the spinal dura mater. Though treatment of IHSP is surgical decompression with steroid therapy, treatment for recurrent IHSP is controversial. Our patient was diagnosed with IHSP based on magnetic resonance imaging (MRI) and underwent laminectomy for decompression following steroid pulse therapy. Despite maintenance of steroid therapy, the patient experienced 3 recurrences. As an alternative immunosuppressant medication, methotrexate was introduced with low-dose steroid. Fortunately, the symptom was resolved, and a decrease of dura thickening was revealed on MRI. We present the case and suggest that methotrexate might be an effective treatment modality for recurrent IHSP.

  18. 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.

  19. Intracranial cerebral artery stenosis with associated coronary artery and extracranial carotid artery stenosis in Turkish patients

    Energy Technology Data Exchange (ETDEWEB)

    Alkan, Ozlem [Department of Radiology, Baskent University, Faculty of Medicine, Ankara (Turkey)], E-mail: yalinozlem@hotmail.com; Kizilkilic, Osman; Yildirim, Tulin [Department of Radiology, Baskent University, Faculty of Medicine, Ankara (Turkey); Atalay, Hakan [Department of Cardiovascular Surgery, Baskent University, Faculty of Medicine, Ankara (Turkey)

    2009-09-15

    Purpose: Although it has been demonstrated that there is a high prevalence of extracranial carotid artery stenosis (ECAS) in patients with severe coronary artery disease, intracranial cerebral artery stenosis (ICAS) is rarely mentioned. We evaluated the prevalence of ICAS in patients with ECAS having elective coronary artery bypass grafting (CABG) surgery to determine the relations between ICAS, ECAS and atherosclerotic risk factors. Methods: We retrospectively reviewed the digital subtraction angiography findings of 183 patients with ECAS {>=} 50% preparing for CABG surgery. The analyses focused on the intracranial or extracranial location and degree of the stenosis. The degree of extracranial stenoses were categorized as normal, <50%, 50-69%, 70-89%, and 90-99% stenosis and occluded. The degree of intracranial stenosis was classified as normal or {<=}25%, 25-49%, and {>=}50% stenosis and occluded. Traditional atherosclerotic risk factors were recorded. Results: ECAS < 70% in 42 patients and ECAS {>=} 70% in 141 patients. ICAS was found in 51 patients and ICAS {>=} 50% in 30 patients. Regarding risk factors, we found hypertension in 135 patients, diabetes mellitus in 91 patients, hyperlipidemia in 84 patients, and smoking in 81 patients. No risk factor was significant predictors of intracranial atherosclerosis. The severity of ICAS was not significantly associated with that of the ECAS. Conclusions: We found ICAS in 27.8% of the patients with ECAS > 50% on digital subtraction angiography preparing for CABG. Therefore a complete evaluation of the neck vessels with magnetic resonance or catheter angiography seems to be indicated as well as intracranial circulation for the risk assessment of CABG.

  20. 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

  1. 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.)

  2. Model Validation for a Noninvasive Arterial Stenosis Detection Problem

    Science.gov (United States)

    2013-06-09

    Diastolic murmur caused by coronary artery stenosis , Ann. Int. Med, 72 (1970), 543. [33] T. Deffieux, G. Montaldo, M. Tanter, Shear wave spectroscopy for...Circulation, 103 (2001), 604–616. [45] N. Owsley and A. Hull, Beamformed nearfield imaging of a simulated coronary artery containing a stenosis , IEEE...murmur of coronary artery stenosis , Brit. Heart J., 35 (1973), 840. [53] J. Semmlow and K. Rahalkar, Acoustic detection of coronary artery disease, Annu

  3. Semilobar Holoprosencephaly with Congenital Oropharyngeal Stenosis in a Term Neonate

    OpenAIRE

    Kenji Hishikawa; Hideshi Fujinaga; Chie Nagata; Masataka Higuchi; Yushi Ito

    2015-01-01

    Background - Holoprosencephaly (HPE) is often accompanied by a deficit in midline facial development; however, congenital oropharyngeal stenosis in neonates with HPE has not been reported before. We describe a case of a neonate with prenatally diagnosed semilobar HPE accompanied by congenital oropharyngeal stenosis. Case Report - The patient was born at 39 weeks of gestation and developed dyspnea shortly after. Laryngoscopic test revealed oropharyngeal stenosis. Nasal continuous positive ...

  4. Hypertrophic Cardiomyopathy as a Manifestation of Multiple Myeloma

    Institute of Scientific and Technical Information of China (English)

    Haojian Dong; Yingling Zhou

    2008-01-01

    Multiple myeloma (MM) is a malignancy of the plasma cell characterized by migration and localization to the bone marrow where cells then disseminate and facilitate the formation of bone lesions.It is associated with a constellation of disease manifestations,apart from osteolytic lesions,anemia and immuno-suppression due to loss of normal hematopoieric stem cell function,and cardiac amyloidosis due to monoclonal immunoglobulin secretion as well[1].Amyloid infiltration of the heart may frequently masquerade as hypertrophic cardiomyopathy (HCM).HCM,of which underlying cause and pathogenesis are largely unknown,is characterized by left and/or right ventricular hypertrophy,with predominant involvement of the interventricular septum in the absence of other causes of hypertrophy,such as hypertension or valvular heart diseases[2].While excessive hypertrophy of the myocardium is most commonly associated with myocyte hypertrophy,infiltration with amyloid always needs to be considered.In this report we presented two cases of multiple myeloma that mimicked hypertrophic cardiomyopathy so closely that it required bone marrow or endomyocardial biopsy to establish the diagnosis.

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

    Directory of Open Access Journals (Sweden)

    Moolman-Smook Johanna C

    2011-10-01

    Full Text Available Abstract Background The gene family KCNE1-5, which encode modulating β-subunits of several repolarising K+-ion channels, has been associated with genetic cardiac diseases such as long QT syndrome, atrial fibrillation and Brugada syndrome. The minK peptide, encoded by KCNE1, is attached to the Z-disc of the sarcomere 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. Results The coding regions of KCNE1, KCNE2, KCNE3, KCNE4, and KCNE5 were examined, by direct DNA sequencing, in a cohort of 93 unrelated HCM probands and 188 blood donor controls. Fifteen genetic variants, four previously unknown, were identified in the HCM probands. Eight variants were non-synonymous and one was located in the 3'UTR-region of KCNE4. No disease-causing mutations were found and no significant difference in the frequency of genetic variants was found between HCM probands and controls. Two variants of likely functional significance were found in controls only. Conclusions Mutations in KCNE genes are not a common cause of HCM and polymorphisms in these genes do not seem to be associated with a propensity to develop arrhythmia

  6. 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.

  7. Reversible transition from a hypertrophic to a dilated cardiomyopathy

    Science.gov (United States)

    Spillmann, Frank; Kühl, Uwe; Van Linthout, Sophie; Dominguez, Fernando; Escher, Felicitas; Schultheiss, Heinz‐Peter; Pieske, Burkert

    2015-01-01

    Abstract We report the case of a 17‐year‐old female patient with known hypertrophic cardiomyopathy and a Wolff‐Parkinson‐White syndrome. She came to our department for further evaluation of a new diagnosed dilated cardiomyopathy characterized by an enlargement of the left ventricle and a fall in ejection fraction. Clinically, she complained about atypical chest pain, arrhythmic episodes with presyncopal events, and dyspnea (NYHA III) during the last 6 months. Non‐invasive and invasive examinations including magnetic resonance imaging, electrophysiological examinations, and angiography did not lead to a conclusive diagnosis. Therefore, endomyocardial biopsies (EMBs) were taken to investigate whether a specific myocardial disease caused the impairment of the left ventricular function. EMB analysis resulted in the diagnosis of a virus‐negative, active myocarditis. Based on this diagnosis, an immunosuppressive treatment with prednisolone and azathioprine was started, which led to an improvement of cardiac function and symptoms within 3 months after initiating therapy. In conclusion, we show that external stress triggered by myocarditis can induce a reversible transition from a hypertrophic cardiomyopathy to a dilated cardiomyopathy phenotype. This case strongly underlines the need for a thorough and invasive examination of heart failure of unknown causes, including EMB investigations as recommend by the actual ESC position statement. PMID:27774273

  8. 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.

  9. NADPH-, NADH- and cumene hydroperoxide-dependent metabolism of benzo[a]pyrene by pyloric caeca microsomes of the sea star Asterias rubens L. (Echinodermata: Asteroidea).

    Science.gov (United States)

    den Besten, P J; Lemaire, P; Livingstone, D R

    1994-10-01

    1. Benzo[a]pyrene (BaP) metabolism was studied in microsomes of the pyloric caeca (main digestive tissue and site of P450) of the echinoderm sea star (starfish) Asterias rubens. 2. NADPH-dependent metabolism of BaP produced phenols (36% of total metabolism), quinones (19%), dihydrodiols (25%) and putative protein adducts (20%). 3. NADH-dependent rates of BaP metabolism were approximately twice those found for NADPH-dependent metabolism, and metabolite formation was shifted towards dihydrodiols and quinones. 4. Cumene hydroperoxide (CHP)-dependent rates of BaP metabolism were also higher than NADPH-dependent rates by a factor of six for quinone and putative protein adduct production, and by a factor of four for phenol and dihydrodiol production. 5. Microsomal rates of BaP metabolism in BaP-exposed sea stars appeared to be elevated more in the case of NADPH-dependent than for CHP-dependent metabolism (respectively, increases of 130 and 41%), indicating the induction of forms of P450 preferentially catalysing NADPH-dependent metabolism. 6. 1,1,1-Trichloropropene-2,3-oxide (TCPO) inhibited dihydrodiol formation from both NADPH- and CHP-dependent BaP metabolism, indicating the involvement of epoxide hydratase in BaP metabolism. 7. Incubations of pyloric caeca microsomes with BaP and a superoxide anion radical-generating system (xanthine/xanthine oxidase) produced putative protein adducts but no free metabolites.

  10. MANAGEMENT OF LUMBAR SPINAL CANAL STENOSIS

    Directory of Open Access Journals (Sweden)

    Mukhergee G. S

    2016-06-01

    Full Text Available BACKGROUND Spinal stenosis is one of the most common conditions in the elderly. It is defined as a narrowing of the spinal canal. The term stenosis is derived from the Greek word for narrow, which is “Stenos”. The first description of this condition is attributed to Antoine portal in 1803. Verbiest is credited with coining the term spinal stenosis and the associated narrowing of the spinal canal as its potential cause. [1-10] Kirkaldy–Willis subsequently described the degenerative cascade in the lumbar spine as the cause for the altered anatomy and pathophysiology in spinal stenosis. [11-15] If compression does not occur, the canal should be described as narrow but not stenotic. Some studies defined lumbar spinal stenosis as a “narrowing of the osteoligamentous vertebral canal and/or the intervertebral foramina causing compression of the thecal sac and/or the caudal nerve roots; at a single vertebral level, narrowing may affect the whole canal or part of it” (Postacchini 1983. This definition distinguished between disc herniation and stenosis. [16] . The most common type of spinal stenosis is caused by degenerative arthritis of the spine. Hypertrophy and ossification of the posterior longitudinal ligament which usually are confined to the cervical spine, and diffuse idiopathic skeletal hyperostosis (DISH syndrome also may result in an acquired form of spinal stenosis. Congenital forms caused by disorders such as achondroplasia and dysplastic spondylolisthesis are much less common. Congenital spinal stenosis usually is central and is evident or imaging studies. Idiopathic congenital narrowing usually involves the anteroposterior dimension of the canal secondary to short pedicles; the patient otherwise is normal. In contrast, in achondroplasia, the canal is narrowed in the anteroposterior plane owing to shortened pedicles and in lateral dimension because of diminished interpedicular distance. Acquired forms of spinal stenosis usually are

  11. Significant regional heterogeneity of coronary flow reserve in paediatric hypertrophic cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Tadamura, E.; Kudoh, T.; Kubo, S.; Konishi, J. [Kyoto Univ. (Japan). School of Medicine; Yoshibayashi, M.; Yonemura, T. [Dept. of Pediatrics, Kyoto Univ. Graduate School of Medicine, Kyoto (Japan); Motooka, M.; Nohara, R.; Matsumori, A.; Sasayama, S. [Third Div., Dept. of Internal Medicine, Kyoto Univ. Graduate School of Medicine, Kyoto (Japan); Matsuda, T. [Dept. of Medical Informatics, Kyoto Univ. Graduate School of Medicine, Kyoto (Japan); Tamaki, N. [Dept. of Nuclear Medicine, Hokkaido Univ. School of Medicine, Sapporo (Japan)

    2000-09-01

    Previous studies have indicated that cardiac events in young patients with hypertrophic cardiomyopathy (HCM) are related to ischaemia rather than to arrhythmia. We measured coronary flow reserve in paediatric HCM and compared the values with those in adult HCM. We studied 12 patients with HCM including six paediatric (<20 years old; mean 13 years) and six adult patients (>20 years old: mean 62 years), and six healthy young adults (mean 29 years) as controls. Every patient underwent magnetic resonance imaging (MRI) for anatomical assessment. Myocardial blood flow at rest and after dipyridamole infusion was measured with dynamic nitrogen-13 ammonia positron emission tomography (PET). Partial volume effect was corrected for using the anatomical data obtained with MRI. In adult patients with HCM, coronary flow reserve in the hypertrophied septal region was not significantly different from that in the non-hypertrophied lateral wall (1.38{+-}0.29 vs 1.77{+-}0.39, respectively). In the paediatric patients, coronary flow reserve in the hypertrophied septal region was significantly lower than in the non-hypertrophied lateral wall (0.84{+-}0.33 vs 2.74{+-}0.90, respectively, P<0.01). In addition, coronary flow reserve in adult patients was lower than in control subjects both in the septal wall (1.38{+-}0.29 vs 2.94{+-}0.35, respectively, P<0.0001) and in the lateral wall (1.77{+-}0.39 vs 2.85{+-}0.69, respectively, P<0.05). In contrast, coronary flow reserve in paediatric patients was not significantly different from that in control subjects in the lateral wall (2.74{+-}0.90 vs 2.85{+-}0.69, respectively), while absolute reduction of myocardial blood flow was noted after pharmacological vasodilatation in the hypertrophied septal region. In conclusion, significant regional differences of coronary flow reserve were present in the paediatric patients with HCM. These results suggest that paediatric patients with HCM intrinsically have the potential to experience significant

  12. Surgical intervention strategies for congenital tracheal stenosis associated with a tracheal bronchus based on the location of stenosis.

    Science.gov (United States)

    Morita, Keiichi; Yokoi, Akiko; Fukuzawa, Hiroaki; Hisamatsu, Chieko; Endo, Kosuke; Okata, Yuichi; Tamaki, Akihiko; Mishima, Yasuhiko; Oshima, Yoshihiro; Maeda, Kosaku

    2016-09-01

    The aim of this study was to determine the appropriate surgical intervention strategies for congenital tracheal stenosis (CTS) associated with a tracheal bronchus based on the location of stenosis. The medical records of 13 pediatric patients with CTS associated with a tracheal bronchus at a single institution between January 2006 and December 2015 were retrospectively reviewed. Type 1: tracheal stenosis above the right upper lobe bronchus (RULB) (n = 1). One patient underwent slide tracheoplasty and was successfully extubated. Type 2: tracheal stenosis below the RULB (n = 7). Tracheal end-to-end anastomosis was performed before 2014, and one patient failed to extubate. Posterior-anterior slide tracheoplasty was performed since 2014, and all three patients were successfully extubated. Type 3: tracheal stenosis above the RULB to the carina (n = 5). One patient underwent posterior-anterior slide tracheoplasty and was successfully extubated. Two patients with left-right slide tracheoplasty and another two patients with tracheal end-to-end anastomosis for the stenosis below the RULB could not be extubated. Tracheal end-to-end anastomosis or slide tracheoplasty can be selected for tracheal stenosis above the RULB according to the length of stenosis. Posterior-anterior slide tracheoplasty appears feasible for tracheal stenosis below the RULB or above the RULB to the carina.

  13. STUDY ON FUNCTION OF FOCAL ADHENSIVE KINASE AND INTEGRIN α1 IN HYPERTROPHIC SCAR FIBROBLASTS

    Institute of Scientific and Technical Information of China (English)

    FU Min-gang; PING Ping; FAN Zhi-hong

    2008-01-01

    Objective To study the function of focal adhesion kinase (FAK) in the formation of hyper-trophic scar and its interrelationship with integrin α1. Methods Original fibroblasts from human hypertrophic scar and human normal dermis were cultured, and immanocytochemistry was applied to detect localization of expres-sion of FAK and integrin α1 in hypertrophic scar and human normal skin fibroblasts. The expression of integrin α1 was detected before and after FAK antibody blocking hypertrophic scar fibroblasts (HSFB) 48 h later. Meanwhile the collagen synthesis was evaluated by [3H] -proline incorporation and HSFB cell proliferation was measured by MTT method. Results The expression of FAK and integrin α1 of hypertrophic scar fibroblasts was higher than that of the normal skin fibroblasts significantly (P <0. 01). The expression of integrinct, was reduced after FAK be-ing blocked (P<0.01). Meanwhile the collagen synthesis of human scar-derived fibroblasts by [3H] -proline incor-poration was depressed respectively (P<0.01). The cell proliferation was inhibited by using 1:100 and 1:200 FAK antibody with MTT method (P<0.01). Conclusion FAK is the key point of signal transmission pathway medi-ated by integrin α1, which regulates protein synthesis of integrin α1, it may play an important role in the prolifera-tion and constriction of hypertrophic scar. FAK antibody can inhibit the collagen synthesis and cell proliferation of hypertrophic scar fibroblasts.

  14. [Enlargement in managment of lumbar spinal stenosis].

    Science.gov (United States)

    Steib, J P; Averous, C; Brinckert, D; Lang, G

    1996-05-01

    Lumbar stenosis has been well discussed recently, especially at the 64th French Orthopaedic Society (SOFCOT: July 1989). The results of different surgical treatments were considered as good, but the indications for surgical treatment were not clear cut. Laminectomy is not the only treatment of spinal stenosis. Laminectomy is an approach with its own rate of complications (dural tear, fibrosis, instability... ).Eight years ago, J. Sénégas described what he called the "recalibrage" (enlargement). His feeling was that, in the spinal canal, we can find two different AP diameters. The first one is a fixed constitutional AP diameter (FCAPD) at the cephalic part of the lamina. The second one is a mobile constitutional AP diameter (MCAPD) marked by the disc and the ligamentum flavum. This diameter is maximal in flexion, minimal in extension. The nerve root proceeds through the lateral part of the canal: first above, between the disc and the superior articular process, then below, in the lateral recess bordered by the pedicle, the vertebral body and the posterior articulation. With the degenerative change the disc space becomes shorter, the superior articular process is worn out with osteophytes. These degenerative events are complicated by inter vertebral instability increasing the stenosis. The idea of the "recalibrage" is to remove only the upper part of the lamina with the ligamentum flavum and to cut the hypertrophied anterior part of the articular process from inside. If needed the disc and other osteophytes are removed. The surgery is finished with a ligamentoplasty reducing the flexion and preventing the extension by a posterior wedge.Our experience in spine surgery especially in scoliosis surgery, showed us that it was possible to cure a radicular compression without opening the canal. The compression is then lifted by the 3D reduction and restoration of an anatomy as normal as possible. Lumbar stenosis is the consequence of a degenerative process. Indeed, hip

  15. 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.

  16. An Unusual Type of Localized Hypertrophic Cardiomyopathy With Wolf Parkinson White Syndrome Presenting With Pulmonary Edema

    Science.gov (United States)

    Vatan, Mehmet Bulent; Gunduz, Huseyin; Gurel, Safiye; Kocayigit, Ibrahim; Vural, Ahmet; Demirtas, Saadet; Cakar, Mehmet Akif; Gunduz, Yasemin

    2012-01-01

    Hypertrophic cardiomyopathy (HCM) is an autosomal dominant heart disease that is the most common genetic cardiac disorder. The disease is characterized by excessive thickening of the left ventricular myocardium. The anterior portion of the interventricular ventricular septum is often involved. Asymmetric hypertrophy of apical site, left ventricular free wall, and right ventricle are less common in hypertrophic cardiomyopathy that occur in 1% cases. We report a case of a patient with an unusual type of hypertrophic cardiomyopathy and Wolf Parkinson White (WPW) presenting with pulmonary edema.

  17. Successful correction of unroofed coronary sinus with pulmonary vein stenosis.

    Science.gov (United States)

    Li, Yang; An, Qi; Zhang, Eryong

    2012-07-01

    We present a case of an infant with an unroofed coronary sinus associated with a persistent left superior vena cava draining into the left atrium, right superior pulmonary vein stenosis, an atretic left superior pulmonary vein and a double-outlet right ventricle. For pulmonary vein stenosis and atresia, we used a sutureless technique with an autologous pericardial patch to create a neoatrium.

  18. Carotid endarterectomies for relieving severe or moderate carotid stenosis

    Institute of Scientific and Technical Information of China (English)

    L(U) Zhi-qian 吕志前; XIAO Ming-di 萧明第; Montagna Pietro; Farhat Fadi; Olivier Jegaden

    2004-01-01

    @@ Carotid artery stenosis is one of the major causes of ischemic strokes.1 To prevent or reduce the occurrences of ischemic strokes, carotid endarterectomies (CEAs) were performed on 48 patients with carotid artery stenosis from November 2000 to June 2003. Results from this study were analyzed and presented here.

  19. Ectopic cartilage in subglottic stenosis: Hamartoma or reaction to trauma?

    NARCIS (Netherlands)

    F.C.P.M. Adriaansen; L.J. Hoeve (Hans); H.L. Verwoerd-Verhoef (Henriëtte); R.O. van der Heul (R.); C.D.A. Verwoerd (Carel)

    1992-01-01

    textabstractIn an experimental study in growing rabbits an endolaryngeal injury to the subglottis resulted in the development of a stenosis due to the formation of scar tissue containing ectopic cartilage. For comparison, biopsies taken from the subglottic stenosis in 8 children were studied

  20. T-tube insertion for sclerotic subglottic stenosis.

    Science.gov (United States)

    Goto, Taichiro; Kato, Ryoichi

    2014-02-01

    T-tube insertion is effective treatment for subglottic stenosis, but it is generally difficult due to bending of the T-tube. In a 52-year-old woman with relapsing polychondritis, a T-tube was inserted after predilatation using Hegar dilators. We describe the details of our T-tube insertion methods for sclerotic subglottic stenosis.

  1. Percutaneous balloon valvuloplasty in mitral stenosis

    Energy Technology Data Exchange (ETDEWEB)

    Park, Jae Hyung; Oh, Byung Hee; Park, Kyung Ju; Kim, Seung Hyup; Lee, Young Woo; Han, Man Chung [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1989-02-15

    Percutaneous balloon valvuloplasty(PBV) was successfully performed in 8 mitral stenosis patients for recent 3 months. Five patients have aortic insufficiencies also and two patients have mitral regurgitations below grade II/IV. All patients showed sinus rhythm on EKG, and had no mitral valvular calcification on echocardiography and fluoroscopy. PBV resulted in an increase in mitral valve area from 1.22{+-}0.22 to 2.57{+-}0.86 cm{sup 2}, a decrease in mean left atrial pressure from 23.4{+-}9.6 to 7.5{+-}3.4 mmHg and a decrease in mean mitral pressure gradient from 21.3{+-}9.4 to 6.8{+-}3.1 mmHg. There were no significant complications except 2 cases of newly appeared and mildly aggravated mitral regurgitation. We believe that PBV will become a treatment modality of choice replacing surgical commissurotomy or valve replacement in a group of mitral stenosis patients, because of its effectiveness and safety.

  2. Horner syndrome due to vertebral artery stenosis.

    Science.gov (United States)

    Kim, Chul Han

    2013-11-01

    The author reports a rare case of Horner syndrome in a patient who resulted from stenosis of the vertebral artery after blunt trauma. A 31-year-old man was transferred to our department for evaluation of left medial orbital wall and nasal bone fractures. Five days ago, he was hospitalized due to multiple second to fourth rib fractures of the right chest following blunt trauma of the face, neck, and chest. Surgery was performed. Ten days later, he complained of drooping of the right eyelid. Physical examination revealed a discrete miosis and ptosis with normal levator function in the right eye. A workup for Horner syndrome was performed. Magnetic resonance angiography of the head and neck revealed a stenosis of the distal part of the right vertebral artery without the abnormality of carotid artery. He wore a cervical collar and underwent anticoagulation. However, Horner syndrome was not resolved over the next 12 months. Acute traumatic Horner syndrome may be associated with vertebral artery dissection in which the possibility of life-threatening injury can be masked.

  3. Adjusting parameters of aortic valve stenosis severity by body size

    DEFF Research Database (Denmark)

    Minners, Jan; Gohlke-Baerwolf, Christa; Kaufmann, Beat A

    2014-01-01

    BACKGROUND: Adjustment of cardiac dimensions by measures of body size appears intuitively convincing and in patients with aortic stenosis, aortic valve area (AVA) is commonly adjusted by body surface area (BSA). However, there is little evidence to support such an approach. OBJECTIVE: To identify...... the adequate measure of body size for the adjustment of aortic stenosis severity. METHODS: Parameters of aortic stenosis severity (jet velocity, mean pressure gradient (MPG) and AVA) and measures of body size (height, weight, BSA and body mass index (BMI)) were analysed in 2843 consecutive patients with aortic...... stenosis (jet velocity ≥2.5 m/s) and related to outcomes in a second cohort of 1525 patients from the Simvastatin/Ezetimibe in Aortic Stenosis (SEAS) study. RESULTS: Whereas jet velocity and MPG were independent of body size, AVA was significantly correlated with height, weight, BSA and BMI (Pearson...

  4. Experimental study of effect of stenosis geometry on flow parameters

    Directory of Open Access Journals (Sweden)

    Veselý Ondřej

    2015-01-01

    Full Text Available A stenosis is a narrowing in a tubular organ or structure. In medicine, vessel stenosis poses health risks for people. In this work, experimental investigations of pressure loss coefficient for varying stenosis eccentricity and shape were performed. Five models of different geometry were studied; all models were stenosis of 75 % area reduction. The flow conditions approximate physiological flow. The measuring range of Reynolds number was from 130 to 2730, measured values of pressure loss coefficient were from 12 to 20. The steady experimental results indicated that static pressure loss coefficient is affected by the shape of stenosis, but it was affected more significantly by the eccentricity. Visualization experiments have been performed in Polycarbonate models.

  5. Stroke prevention-surgical and interventional approaches to carotid stenosis

    Directory of Open Access Journals (Sweden)

    Kumar Rajamani

    2013-01-01

    Full Text Available Extra cranial carotid artery stenosis is an important cause of stroke, which often needs treatment with carotid revascularization. To prevent stroke recurrence, carotid endarterectomy (CEA has been well-established for several decades for symptomatic high and moderate grade stenosis. Carotid stenting is a less invasive alternative to CEA and several recent trials have compared the efficacy of the 2 procedures in patients with carotid stenosis. Carotid artery stenting has emerged as a potential mode of therapy for high surgical risk patients with symptomatic high-grade stenosis. This review focuses on the current data available that will enable the clinician to decide optimal treatment strategies for patients with carotid stenosis.

  6. Clinical utility of natriuretic peptides and troponins in hypertrophic cardiomyopathy.

    Science.gov (United States)

    Kehl, Devin W; Buttan, Anshu; Siegel, Robert J; Rader, Florian

    2016-09-01

    The diagnosis of hypertrophic cardiomyopathy (HCM) is based on clinical, echocardiographic and in some cases genetic findings. However, prognostication remains limited except in the subset of patients with high-risk indicators for sudden cardiac death. Additional methods are needed for risk stratification and to guide clinical management in HCM. We reviewed the available data regarding natriuretic peptides and troponins in HCM. Plasma levels of natriuretic peptides, and to a lesser extent serum levels of troponins, correlate with established disease markers, including left ventricular thickness, symptom status, and left ventricular hemodynamics by Doppler measurements. As a reflection of left ventricular filling pressure, natriuretic peptides may provide an objective measure of the efficacy of a specific therapy. Both natriuretic peptides and troponins predict clinical risk in HCM independently of established risk factors, and their prognostic power is additive. Routine measurement of biomarker levels therefore may be useful in the clinical evaluation and management of patients with HCM.

  7. [Cause and prevention of surgical site infection and hypertrophic scars].

    Science.gov (United States)

    Ogawa, Rei

    2012-03-01

    Surgical site infection (SSI) occurs at the site of surgery within 1 month of an operation or within 1 year of an operation if a foreign body is implanted as part of the surgery. Most SSIs (about 70%) are superficial infections involving the skin and subcutaneous tissues only. The remaining infections are more serious and can involve tissues under the skin, organs, or implanted material. Hypertrophic scars( HSs) occur frequently on particular sites, including the anterior chest wall. The anterior chest wall is frequently subjected to skin stretching caused by the natural daily movements of the body. Most cases of SSIs and HSs can be prevented by (1) suture technique modification to prevent high stretching tension and ischemia, and (2) appropriate wound care after surgery. It would be useful to avoid subjecting wounded skin to sustained mechanical force, thereby permitting the wound to rest and heal normally.

  8. 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.

  9. 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

  10. 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.

  11. Hypertrophic pachymeningitis : varied manifestations of a single disease entity.

    Directory of Open Access Journals (Sweden)

    Prabhakar S

    2002-01-01

    Full Text Available Hypertrophic pachymeningitis is a unique clinical entity characterised by fibrosis and thickening of the duramater with resulting neurological dysfunction. Three cases of this entity are described. Presenting features were headaches and cranial neuropathies in two patients and predominantly cerebellar dysfunction in the third. One of the patients also had evidence of spinal involvement. Lower cranial nerves were chiefly involved in two patients whereas optic nerve was the predominantly affected nerve in one. Except for the presence of rheumatoid arthritis in one of the patients, we could not document clinical or biochemical evidence of any predisposing infective, inflammatory or infiltrative condition in the other two. All three patients had characteristic changes on imaging suggestive of thickened and enhancing duramater. Although variable steroid responsiveness was seen in all the three patients, tendency towards steroid dependence was evident. The clinical presentations, causes, radiological features, management options and differential diagnosis of this unique clinical syndrome have been discussed.

  12. Distinguishing Hypertrophic Cardiomyopathy-Associated Mutations from Background Genetic Noise

    Science.gov (United States)

    Kapplinger, Jamie D.; Landstrom, Andrew P.; Bos, J. Martijn; Salisbury, Benjamin A.; Callis, Thomas E.; Ackerman, Michael J.

    2014-01-01

    Despite the significant progress that has been made in identifying disease-associated mutations, the utility of the Hypertrophic Cardiomyopathy (HCM) genetic test is limited by a lack of understanding of the background genetic variation inherent to these sarcomeric genes in seemingly healthy subjects. This study represents the first comprehensive analysis of genetic variation in 427 ostensibly healthy individuals for the HCM genetic test using the “Gold Standard” Sanger sequencing method validating the background rate identified in the publically available exomes. While mutations are clearly over-represented in disease, a background rate as high as ~5% among healthy individuals prevents diagnostic certainty. To this end, we have identified a number of estimated predictive value-based associations including gene-specific, topology, and conservation methods generating an algorithm aiding in the probabilistic interpretation of an HCM genetic test. PMID:24510615

  13. [A case of localized hypertrophic neuropathy in the sciatic nerve].

    Science.gov (United States)

    Izumi, T; Kusaka, H; Imai, T

    1995-01-01

    A 26-year-old male patient gradually developed muscular atrophy of the right lower leg over a two-year period. Neurological examination revealed absent Achilles tendon reflex and muscular atrophy of the right lower leg and right hamstring muscles. Conduction velocity of the F waves was delayed in the right posterior tibial nerve. A computerized tomography scan and magnetic resonance imaging revealed a mass lesion along the proximal segment of the right sciatic nerve. Exploration revealed a fusiformly swollen sciatic nerve. Histological examination showed that a swollen segment of the sciatic nerve was filled with onion-bulb formations of perineurial cells, consistent with the diagnosis of localized hypertrophic neuropathy. This condition should be added to several etiologies of monomelic amyotrophy. Electrophysiological studies and neuroimaging techniques were useful in obtaining differential diagnosis.

  14. 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....... Several polymorphic mtDNA variants are associated with a variety of late-onset degenerative diseases and affect mitochondrial function. We examined the role of private, non-haplogroup associated, mitochondrial variants in the etiology of HCM. In 87 Danish HCM patients, full mtDNA sequencing revealed 446......>G, and MT-CYB: m.15024G>A, p.C93Y remained. A detailed analysis of these variants indicated that none of them are likely to cause HCM. In conclusion, private mtDNA mutations are frequent, but they are rarely, if ever, associated with HCM....

  15. Hypertrophic cardiomyopathy: a heart in need of an energy bar?

    Directory of Open Access Journals (Sweden)

    Styliani eVakrou

    2014-08-01

    Full Text Available Hypertrophic cardiomyopathy (HCM has been recently recognized as the most common inherited cardiovascular disorder, affecting 1 in 500 adults worldwide. HCM is characterized by myocyte hypertrophy resulting in thickening of the ventricular wall, myocyte disarray, interstitial and/or replacement fibrosis, decreased ventricular cavity volume and diastolic dysfunction. HCM is also the most common cause of sudden death in the young. A large proportion of patients diagnosed with HCM have mutations in sarcomeric proteins. However, it is unclear how these mutations lead to the cardiac phenotype, which is variable even in patients carrying the same causal mutation. Abnormalities in calcium cycling, oxidative stress, mitochondrial dysfunction and energetic deficiency have been described, constituting the basis of therapies in experimental models of HCM and HCM patients. This review focuses on evidence supporting the role of cellular metabolism and mitochondria in HCM.

  16. Neuromuscular fatigue during dynamic maximal strength and hypertrophic resistance loadings.

    Science.gov (United States)

    Walker, Simon; Davis, Lisa; Avela, Janne; Häkkinen, Keijo

    2012-06-01

    The purpose of this study was to compare the acute neuromuscular fatigue during dynamic maximal strength and hypertrophic loadings, known to cause different adaptations underlying strength gain during training. Thirteen healthy, untrained males performed two leg press loadings, one week apart, consisting of 15 sets of 1 repetition maximum (MAX) and 5 sets of 10 repetition maximums (HYP). Concentric load and muscle activity, electromyography (EMG) amplitude and median frequency, was assessed throughout each set. Additionally, maximal bilateral isometric force and muscle activity was assessed pre-, mid-, and up to 30 min post-loading. Concentric load during MAX was decreased after set 10 (Pmuscle activity during HYP loading. Copyright © 2011 Elsevier Ltd. All rights reserved.

  17. Surgery Management of Rare Hypertrophic Frenum in an Infant

    Directory of Open Access Journals (Sweden)

    Sheila de Carvalho Stroppa

    2014-01-01

    Full Text Available To report a rare case of a lateral frenum hypertrophy in an infant, this paper describes the case of a girl who came to a first dental appointment when she was 4 months old. A hypertrophic lateral frenum in the upper left canine region was detected. A great depression in the gingival rodet separated the anterior maxillary segment from the posterior one and also decreased the lip mobility in this region. A frenectomy was performed when the patient was 11 months old and the clinical follow-up was done up to the age of 30 months. There was normalization in the vestibular insertion of the lateral frenum, lip mobility, physiological development of the maxilla, and eruption of the upper incisors, canines, and first primary molars. Infants should go to a dental examination precociously in order to detect possible congenital and development alterations.

  18. Surgery management of rare hypertrophic frenum in an infant.

    Science.gov (United States)

    Stroppa, Sheila de Carvalho; da Silva, Juliana Yassue Barbosa; Tavares, Maria Cristina Reis; Duda, João Gilberto; Losso, Estela Maris

    2014-01-01

    To report a rare case of a lateral frenum hypertrophy in an infant, this paper describes the case of a girl who came to a first dental appointment when she was 4 months old. A hypertrophic lateral frenum in the upper left canine region was detected. A great depression in the gingival rodet separated the anterior maxillary segment from the posterior one and also decreased the lip mobility in this region. A frenectomy was performed when the patient was 11 months old and the clinical follow-up was done up to the age of 30 months. There was normalization in the vestibular insertion of the lateral frenum, lip mobility, physiological development of the maxilla, and eruption of the upper incisors, canines, and first primary molars. Infants should go to a dental examination precociously in order to detect possible congenital and development alterations.

  19. 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)

  20. 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.

  1. 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...

  2. A new era in clinical genetic testing for hypertrophic cardiomyopathy.

    Science.gov (United States)

    Wheeler, Matthew; Pavlovic, Aleksandra; DeGoma, Emil; Salisbury, Heidi; Brown, Colleen; Ashley, Euan A

    2009-12-01

    Building on seminal studies of the last 20 years, genetic testing for hypertrophic cardiomyopathy (HCM) has become a clinical reality in the form of targeted exonic sequencing of known disease-causing genes. This has been driven primarily by the decreasing cost of sequencing, but the high profile of genome-wide association studies, the launch of direct-to-consumer genetic testing, and new legislative protection have also played important roles. In the clinical management of hypertrophic cardiomyopathy, genetic testing is primarily used for family screening. An increasing role is recognized, however, in diagnostic settings: in the differential diagnosis of HCM; in the differentiation of HCM from hypertensive or athlete's heart; and more rarely in preimplantation genetic diagnosis. Aside from diagnostic clarification and family screening, use of the genetic test for guiding therapy remains controversial, with data currently too limited to derive a reliable mutation risk prediction from within the phenotypic noise of different modifying genomes. Meanwhile, the power of genetic testing derives from the confidence with which a mutation can be called present or absent in a given individual. This confidence contrasts with our more limited ability to judge the significance of mutations for which co-segregation has not been demonstrated. These variants of "unknown" significance represent the greatest challenge to the wider adoption of genetic testing in HCM. Looking forward, next-generation sequencing technologies promise to revolutionize the current approach as whole genome sequencing will soon be available for the cost of today's targeted panel. In summary, our future will be characterized not by lack of genetic information but by our ability to effectively parse it.

  3. Management of venous stenosis in living donor liver transplant recipients

    Institute of Scientific and Technical Information of China (English)

    Jie Yang; Ming-Qing Xu; Lu-Nan Yan; Wu-Sheng Lu; Xiao Li; Zheng-Rong Shi; Bo Li; Tian-Fu Wen; Wen-Tao Wang; Jia-Ying Yang

    2009-01-01

    AIM: To retrospectively evaluate the management and outcome of venous obstruction after living donor liver transplantation (LDLT).METHODS: From February 1999 to May 2009, 1 intraoperative hepatic vein (HV) tension induced HV obstruction and 5 postoperative HV anastomotic stenosis occurred in 6 adult male LDLT recipients. Postoperative portal vein (PV) anastomotic stenosis occurred in 1 pediatric left lobe LDLT. Patients ranged in age from 9 to 56 years (median, 44 years). An air balloon was used to correct the intraoperative HV tension. Emergent surgical reoperation, transjugular HV balloon dilatation with stent placement and transfemoral venous HV balloon dilatation was performed for HV stenosis on days 3, 15, 50, 55, and 270 after LDLT, respectively. Balloon dilatation followed with stent placement via superior mesenteric vein was performed for the pediatric PV stenosis 168 d after LDLT.RESULTS: The intraoperative HV tension was corrected with an air balloon. The recipient who underwent emergent reoperation for hepatic stenosis died of hemorrhagic shock and renal failure 2 d later. HV balloon dilatation via the transjugular and transfemoral venous approach was technically successful in all patients. The patient with early-onset HV stenosis receiving transjugular balloon dilatation and stent placement on the 15th postoperative day left hospital 1 wk later and disappeared, while the patient receiving the same interventional procedures on the 50th postoperative day died of graft failure and renal failure 2 wk later. Two patients with late-onset HV stenosis receiving balloon dilatation have survived for 8 and 4 mo without recurrent stenosis and ascites, respectively. Balloon dilatation and stent placement via the superior mesenteric venous approach was technically successful in the pediatric left lobe LDLT, and this patient has survived for 9 mo without recurrent PV stenosis and ascites.CONCLUSION: Intraoperative balloon placement, emergent reoperation, proper

  4. Does the effectiveness of core stability exercises correlate with the severity of spinal stenosis in patients with lumbar spinal stenosis?

    Science.gov (United States)

    Chen, Chaxiang; Lin, Zhichao; Zhang, Yingjie; Chen, Zemin; Tang, Shujie

    2017-01-01

    To determine whether the effectiveness of core stability exercises correlates with the severity of spinal stenosis in patients with degenerative lumbar spinal stenosis. Forty-two patients with degenerative lumbar spinal stenosis treated in the department of orthopedics of our hospital between May 2013 and January 2016 were included in the study. All the patients performed core stability exercises once daily for six weeks, and the clinical outcomes were evaluated using Japanese Orthopaedic Association (JOA) score and self-reported walking capacity. The anteroposterior osseous spinal canal diameter was measured to evaluate the severity of spinal stenosis. The correlation between the stenosis degree and the differences of Japanese Orthopaedic Association score or self-reported walking capacity at baseline and after treatment were analyzed. The patients were divided into three groups according to the spinal stenosis degree. In the three groups, there was no significant difference in JOA or self-reported walking distance at baseline (p>0.05) and after treatment (p>0.05). The JOA scores and self-reported walking distance were significantly increased after treatment (p0.05) or self-reported walking distance (p>0.05). There was no significantcorrelation between the effectiveness of core stability exercises and the severity of spinal stenosis in patients with degenerative lumbar spinal stenosis.

  5. 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

    2017-02-01

    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.

  6. COMPARATIVE STUDY OF FIBRONECTIN GENE EXPRESSION IN TISSUES FROM HYPERTROPHIC SCARS AND DIABETIC FOOT ULCERS

    Institute of Scientific and Technical Information of China (English)

    付小兵; 杨银辉; 孙同柱; 王亚平; 盛志勇

    2002-01-01

    Objective.To explore the expression characteristic of fibronectin gene in hypertrophic scars and diabetic ulcer tissues.Methods.The biopsies from normal skins,hypertrophic scars and diabetic foot ulcers were taken.The technique of quantitative polymerase chain reaction (PCR) was used to evaluate the gene expression of fibronectin in the above biopsies.Results.Fibronectin gene expression was enhanced in hypertrophic scars and decreased in diabetic foot ulcers compared with that in normal skins.Quantitative comparison showed about 2 fold increase of fibronectin mRNA level in hypertrophic scars and about 3 fold decrease of fibronectin mRNA level in diabetic ulcers as compared with that in normal skins.Conclusions.Fibronectin gene expression is influenced by the tissue environment.Different expression and synthesis of fibronectin may cause different outcomes in wound healing.

  7. Nd: YAG Laser Treatment for Keloids and Hypertrophic Scars: An Analysis of 102 Cases

    Directory of Open Access Journals (Sweden)

    Sachiko Koike, MD

    2014-12-01

    Conclusions: Hypertrophic scars 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.

  8. 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

    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...

  9. Exercise echocardiography in the evaluation of obstructive types of hypertrophic cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    邵春丽

    2013-01-01

    Objective To assess the condition of left ventricular outflow tract obstruction (LVOTO) under resting conditions and physiological exercise in hypertrophic cardiomyopathy (HCM) patients.Methods A total of 60 patients with HCM and left ventricular outflow tract gradient

  10. Production of cytokines by mononuclear cells of hypertrophic adenoids in children with otitis media with effusion.

    Science.gov (United States)

    Zelazowska-Rutkowska, Beata; Ilendo, Elzbieta; Skotnicka, Bozena; Wysocka, Jolanta; Kasprzycka, Edwina

    2012-01-01

    Hypertrophic adenoids with otitis media with effusion is a common infectious disease and present a serious otological problem in children. Cytokines, potent inflammatory mediators, play important role in the initiation of immunological response in otitis media. Adenoids excised due to hypertrophy with or without chronic otitis media with effusion were used to isolate mononuclear cells. Secretion of cytokines by non-stimulated and PHA-stimulated cells was determined by specific ELISAs. We found a significant increase in the production of IL-5 and TNF-α secreted by adenoidal cells of children with otitis media with effusion compared to group with hypertrophic adenoids. No differences were found in the secretion of IL-8, IL-6, and IL-10 between these two groups of patients. Our results suggest a difference between the immunological responses in the course of hypertrophic adenoids with otitis media as compared to hypertrophic adenoids.

  11. Time-course changes in the expression of Na+, K+-ATPase in gills and pyloric caeca of brown trout (Salmo trutta) during acclimation to seawater

    DEFF Research Database (Denmark)

    Seidelin, Michel; Madsen, Steffen; Blenstrup, Henrik;

    2000-01-01

    Changes in protein and mRNA expression of Na+,K+-ATPase in gills and pyloric caeca of brown trout were investigated on a detailed time course after transfer from freshwater to 25 ppt seawater (SW). A transient deflection in plasma osmolality and muscle water content lasting from 4 h until day 3...... posttransfer. The similarity of the response in these two organs suggests that they both play significant physiological roles in restoring hydromineral balance after abrupt increase in salinity. Further, SW transfer induced a slight, though significant, increase in primary gill filament Na+, K......+-ATPase immunoreactive (NKIR) cell abundance. This was paralleled by a marked (50%) decrease in secondary lamellar NKIR cell abundance after less than 1 d in SW. Thus, SW acclimation in brown trout is characterised by a lasting decrease in overall NKIR cell abundance in the gill. We propose that SW transfer stimulates...

  12. 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.

  13. 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)....

  14. Pulmonary vein stenosis: Etiology, diagnosis and management

    Institute of Scientific and Technical Information of China (English)

    Pablo Pazos-López; Cristina García-Rodríguez; Alba Guitián-González; Emilio Paredes-Galán; María ángel; De La Guarda álvarez-Moure; Marta Rodríguez-álvarez; José Antonio Baz-Alonso; Elvis Teijeira-Fernández; Francisco Eugenio Calvo-Iglesias; Andrés í?iguez-Romo

    2016-01-01

    Pulmonary vein stenosis(PVS) is rare condition characterized by a challenging diagnosis and unfavorable prognosis at advance stages. At present, injury from radiofrequency ablation for atrial fibrillation has become the main cause of the disease. PVS is characterized by a progressive lumen size reduction of one or more pulmonary veins that, when hemodynamically significant, may raise lobar capillary pressure leading to signs and symptoms such as shortness of breath, cough, and hemoptysis. Image techniques(transesophageal echocardiography, computed tomography, magnetic resonance and perfusion imaging) are essential to reach a final diagnosis and decide an appropriate therapy. In this regard, series from referral centers have shown that surgical and transcatheter interventions may improve prognosis. The purpose of this article is to review the etiology, assessment and management of PVS.

  15. OPERATIVE TREATMENT FOR DEGENERATIVE LUMBAR SPINAL STENOSIS

    Directory of Open Access Journals (Sweden)

    Samo K. Fokter

    2002-11-01

    Full Text Available Background. Degenerative lumbar spinal stenosis (DLSS is a common cause of low back and leg pain in the elderly. Conservative treatment seldom results in sustained improvement.Methods. Fifty-six patients (33 women, 23 men older than 50 years (mean 67 years, range 51 to 82 years and with no prior low back surgery were treated from 1993 to 1999 for clinical and radiologic evidence of DLSS. The goal of this study was to describe the results of decompressive laminectomy with or without fusion in terms of reoperation, severity of back pain, leg pain and patient satisfaction. Answers to Swiss spinal stenosis questionnaires completed before surgery and one to five years afterwards were evaluated. Seven patients (12.5% with degenerative spondylolisthesis, scoliosis and/or more radical facetectomies received fusion.Results. Of the 56 patients in the original cohort, two were deceased and two had undergone reoperation by follow-up. Forty-eight patients answered questionnaires. Average duration of follow-up was 2.5 years. More than 70 percent of the respondents had no or only mild back or buttock pain at follow-up and more than 60 percent were able to walk more than 500 m. Added fusion reduced the incidence of low back pain and pain frequency, and increased walking distance (ANOVA.Conclusions. Eighty-one percent of patients were satisfied with the results of surgery and 87.5% would choose to have the operation again if they had the choice. Decompressive laminectomy for DLSS yields best results if instrumented fusion is included in the procedure.

  16. Relationship of cerebral arterial stenosis to cognitive and memory disorders

    Institute of Scientific and Technical Information of China (English)

    Jifeng Li; Zhou Wang; Shenggang Sun; Gaomei Cai; Kejin Gu; Yaoqun Li

    2006-01-01

    BACKGROUND: Cerebral arterial stenosis can cause cerebral hypoperfusion, and than result in the decline of cognitive function, whereas the cognitive dysfunction induced by different cerebral arterial stenosis have different manifestations and types.OBJECTIVE: To observe the differences of cognitive and memory dysfunctions in patients with cerebral arterial stenosis of different types.DESIGN: A comparative observation.SETTING: Affiliated Hospital of Jining Medical College.PARTICIPANTS: Forty-two outpatients or inpatients with cerebral arterial stenosis were selected from the Department of Neurology, Affiliated Hospital of Jining Medical College from February 2005 to January 2006,including 25 males and 17 females. There were 18 cases of internal carotid arterial stenosis, 14 cases of vertebrobasilar arterial stenosis and 10 cases of whole cerebral arterial stenosis. The diagnostic standards for cerebral arterial stenosis were identified according to North American Symptomatic Carotid Endarterectomy Trial (NAS CET). Meanwhile, 18 healthy physical examinees were enrolled as the control group, including 10males and 8 females, aged 58-80 years old. All the enrolled subjects were informed and agreed with the detection and evaluation.METHODS: ① The memory function was evaluated using revised Wechsler memory scale for adults, including long-term memory (experience, orientation and counting), short-term memory (visual recognition, picture memory, visual regeneration, association and thigmesthesia) and sensory memory (forward and backward recitation of numbers). The scale scores were turned to memory quotients. The higher the scores, the better the memory function. ② The cognitive function was evaluated using revised Wechsler adult intelligence scale:It consisted of eleven subtests, including six language scales (information, digit span, vocabulary, arithmetics,apprehension, similarity) and five operation scales (picture completion, picture arrangement, block design

  17. Successful angioplasty during pregnancy for renal artery stenosis.

    Science.gov (United States)

    Margueritte, François; Velasco, Stephane; Pourrat, Olivier; Pierre, Fabrice

    2016-03-01

    Renal artery stenosis can be diagnosed during pregnancy and treated at the same time. A 30-year-old woman had a sudden, severe but asymptomatic hypertensive crisis at 21 weeks of gestation. The diagnosis of renal artery stenosis suspected on Doppler ultrasonography was confirmed and treated by renal angioplasty, which reduced her blood pressure. At 27 weeks of gestation, her blood pressure increased again, associated with significant proteinuria, suggesting pre-eclampsia. A cesarean section was performed giving birth to a healthy 940-g child. Renal artery stenosis should be considered when sudden and early-onset hypertension appears during pregnancy.

  18. MicroRNA 181b Regulates Decorin Production by Dermal Fibroblasts and May Be a Potential Therapy for Hypertrophic Scar

    OpenAIRE

    Peter Kwan; Jie Ding; Tredget, Edward E.

    2015-01-01

    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 fo...

  19. Endostatin inhibits hypertrophic scarring in a rabbit ear model

    Institute of Scientific and Technical Information of China (English)

    Hai-tao REN; Hang HU; Yuan LI; Hong-fei JIANG; Xin-lei HU; Chun-mao HAN

    2013-01-01

    Objective:The present study was designed to use an in vivo rabbit ear scar model to investigate the efficacy of systemic administration of endostatin in inhibiting scar formation.Methods:Eight male New Zealand white rabbits were randomly assigned to two groups.Scar model was established by making six full skin defect wounds in each ear.For the intervention group,intraperitoneal injection of endostatin was performed each day after the wound healed (about 15 d post wounding).For the control group,equal volume of saline was injected.Thickness of scars in each group was measured by sliding caliper and the scar microcirculatory perfusion was assessed by laser Doppler flowmetry on Days 15,21,28,and 35 post wounding.Rabbits were euthanatized and their scars were harvested for histological and proteomic analyses on Day 35 post wounding.Results:Macroscopically,scars of the control group were thicker than those of the intervention group.Significant differences between the two groups were observed on Days 21 and 35 (p<0.05).Scar thickness,measured by scar elevation index (SEI) at Day 35 post wounding,was significantly reduced in the intervention group (1.09±0.19) compared with the controls (1.36±0.28).Microvessel density (MVD) observed in the intervention group (1.73±0.94) was significantly lower than that of the control group (5.63±1.78)on Day 35.The distribution of collagen fibers in scars treated with endostatin was relatively regular,while collagen fibers in untreated controls were thicker and showed disordered alignment.Western blot analysis showed that the expressions of type Ⅰ collagen and Bcl-2 were depressed by injection of endostatin.Conclusions:Our results from the rabbit ear hypertrophic scar model indicate that systemic application of endostatin could inhibit local hypertrophic scar formation,possibly through reducing scar vascularization and angiogenesis.Our results indicated that endostatin may promote the apoptosis of endothelial cells and block their

  20. [Nasal respiratory stenosis and maxillary hypoplasia. Changes after orthodontic treatment with rapid palatal expansion].

    Science.gov (United States)

    Piccini, A; Giorgetti, R; Fiorelli, G

    1989-01-01

    The relationship between hypoplasia of the upper maxillary bone and nasal respiratory insufficiency in a group of twenty infants with malocclusion being treated by rapid maxillary expansion (RME) were studied. Prior to treatment all patients presented endognatia with discrepancies of from -4 to -7 mm in the transverse basal skeleton. These were often associated with adenoid hypertrophy (70% of the cases), increased total nasal resistance (70%), oral respiration (80%) and middle ear diseases (30%). RME led to resolution of occlusion alterations in all cases and often also brought about a regression in adenoid hypertrophy (57% of the cases), normalization of the total nasal resistance (70%) and respiration (80%). These effects were achieved alone without association with any other form of medical or surgical E.N.T. treatment. The functional results confirmed by the radiological and clinical findings indicate an increase in the diameters of the nasal fossa and in the distance between the canines, between premolars and between molars as well as reduction in adenoid vegetation and in the diffuse hypertrophic tissues lining the naso-pharyngeal space. Nonetheless, hypoplasia of the upper maxillary bone and nasal respiratory insufficiency remain strictly linked and are bound to a variable, and at times uncertain, cause-effect relationship. Is nasal stenosis the moving force of maxillary-mandibular dysmorphism and gnatological dysfunction or does it result from an overall genetic conditioning of facial skeleton development? During their vast experience in adenoid and metadenoid pathologies in infancy the authors have, at times, observed significant maxillo-facial dysmorphisms. They have likewise found that "facies adenoidea" were not always associated with hypertrophy of the pharyngeal tonsil.(ABSTRACT TRUNCATED AT 250 WORDS)

  1. Intensive lipid lowering with simvastatin and ezetimibe in aortic stenosis

    DEFF Research Database (Denmark)

    Rossebo, A.B.; Pedersen, T.R.; Boman, K.

    2008-01-01

    failure, coronary-artery bypass grafting, percutaneous coronary intervention, and nonhemorrhagic stroke. Secondary outcomes were events related to aortic-valve stenosis and ischemic cardiovascular events. RESULTS: During a median follow-up of 52.2 months, the primary outcome occurred in 333 patients (35...... of patients who underwent coronary-artery bypass grafting. Cancer occurred more frequently in the simvastatin-ezetimibe group (105 vs. 70, P=0.01). CONCLUSIONS: Simvastatin and ezetimibe did not reduce the composite outcome of combined aortic-valve events and ischemic events in patients with aortic stenosis......BACKGROUND: Hyperlipidemia has been suggested as a risk factor for stenosis of the aortic valve, but lipid-lowering studies have had conflicting results. METHODS: We conducted a randomized, double-blind trial involving 1873 patients with mild-to-moderate, asymptomatic aortic stenosis. The patients...

  2. Intermittent priapism as a clinical feature of lumbar spinal stenosis

    National Research Council Canada - National Science Library

    Rojas, J I; Zurrú-Ganen, M C; Romano, M; Patrucco, L; Cristiano, E

    2007-01-01

    .... Patients with lumbar spinal stenosis may present a variety of signs and symptoms. One such syndrome is neurogenic intermittent claudication, characterized by radicular symptoms exacerbated by walking or standing and relieved by rest...

  3. Imaging markers of stroke risk in asymptomatic carotid artery stenosis

    Directory of Open Access Journals (Sweden)

    Shyam Prabhakaran

    2015-01-01

    Full Text Available Carotid stenosis is a major cause of ischemic stroke. While symptomatic carotid stenosis requires prompt revascularization, there is significant debate about the management of asymptomatic carotid stenosis (ACS, especially in light of recent advances in medical therapy. As a result, there is an even greater need for reliable predictors of stroke risk in asymptomatic patients. Besides clinical factors and stenosis grade, plaque morphology and cerebral hemodynamics may be suitable prognostic tools. High-risk features, using Doppler and magnetic resonance imaging (MRI suggest that subpopulations at sufficiently high risk (10% annually can be identified and in whom revascularization would be most beneficial. In this review, imaging tools to aid in stroke risk stratification in patients with ACS are discussed.

  4. Semilobar Holoprosencephaly with Congenital Oropharyngeal Stenosis in a Term Neonate

    Directory of Open Access Journals (Sweden)

    Kenji Hishikawa

    2015-10-01

    Full Text Available Background - Holoprosencephaly (HPE is often accompanied by a deficit in midline facial development; however, congenital oropharyngeal stenosis in neonates with HPE has not been reported before. We describe a case of a neonate with prenatally diagnosed semilobar HPE accompanied by congenital oropharyngeal stenosis. Case Report - The patient was born at 39 weeks of gestation and developed dyspnea shortly after. Laryngoscopic test revealed oropharyngeal stenosis. Nasal continuous positive airway pressure, high-flow nasal cannula, and nasopharyngeal airway did not resolve her dyspnea; tracheostomy was required. Conclusion - Neonates with HPE might be at higher risk of pharyngeal stenosis because of the functional and/or anatomical abnormalities. In the case of dyspnea in neonates with HPE, laryngoscopic evaluation should be considered.

  5. Semilobar Holoprosencephaly with Congenital Oropharyngeal Stenosis in a Term Neonate.

    Science.gov (United States)

    Hishikawa, Kenji; Fujinaga, Hideshi; Nagata, Chie; Higuchi, Masataka; Ito, Yushi

    2015-10-01

    Background Holoprosencephaly (HPE) is often accompanied by a deficit in midline facial development; however, congenital oropharyngeal stenosis in neonates with HPE has not been reported before. We describe a case of a neonate with prenatally diagnosed semilobar HPE accompanied by congenital oropharyngeal stenosis. Case Report The patient was born at 39 weeks of gestation and developed dyspnea shortly after. Laryngoscopic test revealed oropharyngeal stenosis. Nasal continuous positive airway pressure, high-flow nasal cannula, and nasopharyngeal airway did not resolve her dyspnea; tracheostomy was required. Conclusion Neonates with HPE might be at higher risk of pharyngeal stenosis because of the functional and/or anatomical abnormalities. In the case of dyspnea in neonates with HPE, laryngoscopic evaluation should be considered.

  6. Prevalence and risk factors of atherosclerotic renal artery stenosis

    Institute of Scientific and Technical Information of China (English)

    严健华

    2013-01-01

    Objective To explore the prevalence and risk factors of atherosclerotic renal artery stenosis(ARAS) in patients undergoing coronary angiography.Methods A total of 2506 patients with suspected and known coronary

  7. Functional outcome of surgical management of degenerative lumbar canal stenosis

    Directory of Open Access Journals (Sweden)

    Rajendra Nath

    2012-01-01

    Conclusion: Operative treatment in patients of degenerative lumbar canal stenosis yields excellent results as observed on the basis of JOA scoring system. No patient got recurrence of symptoms of nerve compression.

  8. Celiac artery stenosis/occlusion treated by interventional radiology

    Energy Technology Data Exchange (ETDEWEB)

    Ikeda, Osamu [Department of Diagnostic Radiology, Kumamoto University Graduate School of Medical and Pharmaceutical Sciences, 1-1-1, Honjo Kumamoto 860-8505 (Japan)], E-mail: osamu-3643ik@do9.enjoy.ne.jp; Tamura, Yoshitaka; Nakasone, Yutaka; Yamashita, Yasuyuki [Department of Diagnostic Radiology, Kumamoto University Graduate School of Medical and Pharmaceutical Sciences, 1-1-1, Honjo Kumamoto 860-8505 (Japan)

    2009-08-15

    Severe stenosis/occlusion of the proximal celiac trunk due to median arcuate ligament compression (MALC), arteriosclerosis, pancreatitis, tumor invasion, and celiac axis agenesis has been reported. However, clinically significant ischemic bowel disease attributable to celiac axis stenosis/occlusion appears to be rare because the superior mesenteric artery (SMA) provides for rich collateral circulation. In patients with celiac axis stenosis/occlusion, the most important and frequently encountered collateral vessels from the SMA are the pancreaticoduodenal arcades. Patients with celiac artery stenosis/occlusion are treated by interventional radiology (IR) via dilation of the pancreaticoduodenal arcade. In patients with dilation of the pancreaticoduodenal arcade on SMA angiograms, IR through this artery may be successful. Here we provide several tips on surmounting these difficulties in IR including transcatheter arterial chemoembolization for hepatocellular carcinoma, an implantable port system for hepatic arterial infusion chemotherapy to treat metastatic liver tumors, coil embolization of pancreaticoduodenal artery aneurysms, and arterial stimulation test with venous sampling for insulinomas.

  9. Congenital Tracheal Stenosis in a Patient with Cleft Lip | Qureshi ...

    African Journals Online (AJOL)

    Congenital Tracheal Stenosis in a Patient with Cleft Lip. ... of the distal trachea, in addition to a right sided cleft lip without cleft palate. Management of CTS depends on several factors, with surgery being the definitive form of treatment.

  10. Involvement of impaired desmosome-related proteins in hypertrophic scar intraepidermal blister formation.

    Science.gov (United States)

    Tan, Jianglin; He, Weifeng; Luo, Gaoxing; Wu, Jun

    2015-11-01

    Hypertrophic scar is one of the unique fibrotic diseases in human. Intraepidermal blister is a common clinical symptom following the hypertrophic scar formation. However, little is known about the reason of blister creation. In this study, we selected three patients with hypertrophic scar as manifested by raised, erythematous, pruritic, blister and thickened appearance undergoing scar resection. The first scar sample was 6 months after burn from the neck of a 3 years old male patient with 10 score by Vancouver Scar Scale (VSS). The second scar sample was 12 months after burn from the dorsal foot of a 16 years old female patient with 13 score by VSS. The third one was 9 months after burn from the elbow of a 34 years old male patients with 13 score by VSS. In order to understand the molecular mechanism of blister formation, we screened the different protein expression between hypertrophic scar and normal skin tissue by means of isobaric tags for relative and absolute quantitation (iTRAQ) labeling technology and high throughput 2D LC-MS/MS. There were 48 proteins found to be downregulated in hypertrophic scar. Among the downregulated ones, plakophilin1 (PKP1), plakophilin3 (PKP3) and desmoplakin (DSP) were the desmosome-related proteins which were validated by immunohistochemistry and western blotting assay. Transmission electron microscopy further showed the considerably reduced size and intensity of hemidesmosome and desmosome in hypertrophic scar tissue, compared to control normal skin. Our data indicted for the first time that downregulation of DSP, PKP1 and PKP3 in hypertrophic scar might be responsible for intraepidermal blister formation. Copyright © 2015 Elsevier Ltd and ISBI. All rights reserved.

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

    Directory of Open Access Journals (Sweden)

    Hua-Yu Zhu

    Full Text Available BACKGROUND: 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. METHODOLOGY/PRINCIPAL FINDINGS: 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. CONCLUSIONS/SIGNIFICANCE: 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.

  12. Predictors of exercise capacity and symptoms in severe aortic stenosis

    DEFF Research Database (Denmark)

    Dalsgaard, Morten; Kjaergaard, Jesper; Pecini, Redi

    2010-01-01

    This study investigated the association between invasive and non-invasive estimates of left ventricular (LV) filling pressure and exercise capacity, in order to find new potential candidates for risk markers in severe aortic valve stenosis (AS).......This study investigated the association between invasive and non-invasive estimates of left ventricular (LV) filling pressure and exercise capacity, in order to find new potential candidates for risk markers in severe aortic valve stenosis (AS)....

  13. Craniovertebral junction stenosis in Lenz-Majewski syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Mizuguchi, Koichi; Ishigro, Akira [National Center for Child Health and Development, Department of General Pediatrics and Interdisciplinary Medicine, Setagaya-ku, Tokyo (Japan); Miyazaki, Osamu [National Center for Child Health and Development, Department of Radiology, Tokyo (Japan); Nishimura, Gen [Tokyo Metropolitan Children' s Medical Center, Department of Pediatric Imaging, Tokyo (Japan)

    2015-09-15

    We report a girl with Lenz-Majewski syndrome associated with craniovertebral junction stenosis that led to communicating hydrocephalus and cervical myelopathy. The life-threatening complication was related to progressive craniovertebral hyperostosis that rapidly exacerbated during early childhood. Despite initial success of surgical intervention at 2 years of age, she developed apneic spells and died suddenly at age 5 years. Close monitoring for craniovertebral junction stenosis is essential to reduce morbidity and mortality in children with Lenz-Majewski syndrome. (orig.)

  14. Unilateral pulmonary agenesis associated with tracheal stenosis : a case report

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Yong Suk; Yoon, Chong Hyun; Kim, Kyung Sook; Kim, Ki Soo; Pi, Soo Young [Univ. of Ulsan, Ulsan (Korea, Republic of). Colle. of Medicine

    1998-02-01

    Unilateral pulmonary agenesis is a rare congenital anomaly and is frequently associated with other congenital anomalies. We report a case of left pulmonary agenesis associated with congenital tracheal stenosis in a newborn infant. Simple chest radiographs showed an overinflate right lung and mediastinal shifting to the left side. Chest ST and reconstructed three-dimensional images showed left pulmonary agenesis and tracheal stenosis. These anomalies of the tracheobronchial system were confirmed by bronchography. (author). 10 refs., 3 figs.

  15. Predictors of exercise capacity and symptoms in severe aortic stenosis

    DEFF Research Database (Denmark)

    Dalsgaard, Morten; Kjaergaard, Jesper; Pecini, Redi

    2010-01-01

    This study investigated the association between invasive and non-invasive estimates of left ventricular (LV) filling pressure and exercise capacity, in order to find new potential candidates for risk markers in severe aortic valve stenosis (AS).......This study investigated the association between invasive and non-invasive estimates of left ventricular (LV) filling pressure and exercise capacity, in order to find new potential candidates for risk markers in severe aortic valve stenosis (AS)....

  16. Craniovertebral junction stenosis in Lenz-Majewski syndrome.

    Science.gov (United States)

    Mizuguchi, Koichi; Miyazaki, Osamu; Nishimura, Gen; Ishigro, Akira

    2015-09-01

    We report a girl with Lenz-Majewski syndrome associated with craniovertebral junction stenosis that led to communicating hydrocephalus and cervical myelopathy. The life-threatening complication was related to progressive craniovertebral hyperostosis that rapidly exacerbated during early childhood. Despite initial success of surgical intervention at 2 years of age, she developed apneic spells and died suddenly at age 5 years. Close monitoring for craniovertebral junction stenosis is essential to reduce morbidity and mortality in children with Lenz-Majewski syndrome.

  17. Subcoronary versus supracoronary aortic stenosis. an experimental evaluation

    Directory of Open Access Journals (Sweden)

    Hasenkam J Michael

    2011-08-01

    Full Text Available Abstract Background Valvular aortic stenosis is the most common cause of left ventricular hypertrophy due to gradually increasing pressure work. As the stenosis develop the left ventricular hypertrophy may lead to congestive heart failure, increased risk of perioperative complications and also increased risk of sudden death. A functional porcine model imitating the pathophysiological nature of valvular aortic stenosis is very much sought after in order to study the geometrical and pathophysiological changes of the left ventricle, timing of surgery and also pharmacological therapy in this patient group. Earlier we developed a porcine model for aortic stenosis based on supracoronary aortic banding, this model may not completely imitate the pathophysiological changes that occurs when valvular aortic stenosis is present including the coronary blood flow. It would therefore be desirable to optimize this model according to the localization of the stenosis. Methods In 20 kg pigs subcoronary (n = 8, supracoronary aortic banding (n = 8 or sham operation (n = 4 was preformed via a left lateral thoracotomy. The primary endpoint was left ventricular wall thickness; secondary endpoints were heart/body weight ratio and the systolic/diastolic blood flow ratio in the left anterior descending coronary. Statistical evaluation by oneway anova and unpaired t-test. Results Sub- and supracoronary banding induce an equal degree of left ventricular hypertrophy compared with the control group. The coronary blood flow ratio was slightly but not significantly higher in the supracoronary group (ratio = 0.45 compared with the two other groups (subcoronary ratio = 0.36, control ratio = 0.34. Conclusions A human pathophysiologically compatible porcine model for valvular aortic stenosis was developed by performing subcoronary aortic banding. Sub- and supracoronary aortic banding induce an equal degree of left ventricular hypertrophy. This model may be valid for experimental

  18. [Surgical prevention of stroke in patients with carotid stenosis].

    Science.gov (United States)

    Pyshkina, L I; Khatagova, D T; Kabanov, A A; Darvish, N A; Alibekova, Zh M

    2014-01-01

    Objectives. To study the cerebral and central hemodynamics in patients with stenotic lesions of inner carotid arteries (ICA) before and after reconstructive surgery. Material and methods. Fifty-nine patients, aged from 46 to 78 years, with >50% atherosclerotic stenosis of ICA who underwent preventive carotid endarterectomy (CEAE) were examined. The isolate stenosis of ICA was identified in 13.6% of patients, concomitant lesions of brachiocephalic arteries in 86.4%. Atherosclerotic lesions of coronary arteries were found in 91.1% of patients and those of arteries of lower extremities in 45.8%. In 25.5% of patients, the heart surgery was performed before the current hospitalization. Heterogeneous atherosclerotic plaques with dense or hyperdense components were more frequents in symptomatic stenosis (63.3%). The maximal stenosis was identified in heterogeneous hyperechogenic plaques, the minimal ones in homogenous hypoechogenic plaques. Parameters of central hemodynamics were better in patients with 2nd stage of reconstructive surgeries. The emission fraction decreased proportionally to the degree of stenosis. The parameters of cerebral hemodynamics were significantly decreased in ICA stenosis and improved in the early post-surgery period. Results. CEAE promoted the improvement of cognitive functions and the recovery of motor functions. The best positive dynamics was recorded in asymptomatic ICA stenosis. Poor outcome (transitory ischemic attacks, urgent surgery, restenosis) was found in patients with low levels of central and cerebral hemodynamics 12-24 months after the discharge. Moreover, smoking and the degree of stenosis predicted poor outcome. Conclusions. Surgical treatment in combination with the complex pharmacotherapy (hypotensive drugs, antiaggregants and statins) had the maximal effect, including the remote period.

  19. Association between the Gensini Score and Carotid Artery Stenosis

    Science.gov (United States)

    Fidan, Serdar; Tabakçı, Mehmet Mustafa; Toprak, Cuneyt; Alizade, Elnur; Acar, Emrah; Bayam, Emrah; Tellice, Muhammet; Naser, Abdurrahman; Kargın, Ramazan

    2016-01-01

    Background and Objectives The aim of this study was to evaluate the association between the extent of coronary artery disease assessed by the Gensini score and/or the SYNTAX score and the significant carotid stenosis in patients undergoing coronary artery bypass grafting (CABG). Subjects and Methods A total of 225 patients who had carotid doppler ultrasonography prior to CABG were included retrospectively. Significant coronary artery disease was assumed as a lumen diameter stenosis of ≥50% in any of the major epicardial coronary arteries. The severity of carotid stenosis was determined by B-mode and duplex ultrasonography. Clinically significant carotid stenosis was defined as peak systolic velocity greater than 125 cm/s. Results The mean value of SYNTAX score and Gensini score was highest in patients allocated to significant carotid stenosis (22.98±7.32, p<0.001 and 77.40±32.35, p<0.001, respectively). The other risk factors for significant carotid stenosis were found to be male gender (p=0.029), carotid bruit (p<0.001), diabetes (p=0.021), left main disease (p=0.002), 3-vessel disease (p=0.008), chronic total coronary occlusion (p=0.001), and coronary artery calcification (p=0.001) in univariate analysis. However, only the Gensini score (odds ratio[OR]=1.030, p=0.004), carotid bruit (OR=0.068, p<0.001), and male gender (OR=0.190, p=0.003) were the independent predictors. The Gensini score cut off value predicting significant carotid stenosis was 50.5 with 77% sensitivity (p<0.001). Conclusion The Gensini score may be used to identify patients at high risk for significant carotid stenosis prior to CABG. PMID:27721854

  20. Hypertrophic cardiomyopathy--state of the art in 2007.

    Science.gov (United States)

    Monteiro, Silvia; Costa, Susana; Monteiro, Pedro; Gonçalves, Lino; Providência, Luís A

    2008-05-01

    Hypertrophic cardiomyopathy (HCM) is a primary disease of the sarcomere, with considerable genetic heterogeneity and variability in phenotypic expression, whose main complication is sudden cardiac death (SCD). Genetic aspects of HCM, its molecular pathophysiology and genotype-phenotype relationships are the subject of this review, which is aimed at better understanding of practical management in this patient population. As HCM is a genetic disease whose initial manifestation can be sudden death, it is essential to establish the diagnosis at an early stage, to proceed with risk stratification and implementation of SCD prevention strategies, and to promote genetic counseling of patients and screening of their families. Detection of pathological mutations through progressive sequencing of the genes most commonly involved is the most efficient way to diagnose HCM, even in the absence of clinical evidence of the disease. Identification of individuals at high risk of SCD is a major challenge in the management of this population, since SCD can be prevented by use of an implantable cardioverter-defibrillator. The selection of patients for prophylactic implantation of these devices, particularly those who have only one major risk factor, is currently the subject of controversy.

  1. 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.

  2. 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.

  3. Hypertrophic obstructive cardiomyopathy in an infant with an adrenocortical tumor.

    Science.gov (United States)

    Hauser, Jakob; Riedl, Stefan; Michel-Behnke, Ina; Minkov, Milen; Perneczky, Eva; Horcher, Ernst

    2013-08-01

    Nonfamilial cardiomyopathies in childhood have been only sporadically ascribed to endocrine disorders. We report on a 4-month-old male infant presenting with Cushing's syndrome associated with excessive body weight (8.9 kg; >97th percentile) and features of virilization (Tanner stage 2 for pubic hair development). Abdominal sonography showed a large adrenal tumor. Echocardiography revealed myocardial hypertrophy with severe subaortic obstruction. Blood tests showed excessive androgen and cortisol serum levels with absent circadian rhythm as well as suppressed corticotropin. Urine catecholamine levels were within the normal range. Tumor resection with general anesthesia was performed after preparation with antihypertensive and anticongestive drug therapy. Continuous intravenous hydrocortisone substitution was started intraoperatively and subsequently tapered and switched to oral administration after 12 days. A gradual reduction in glucocorticoid substitution and its discontinuation after a total duration of 9 months were well tolerated. Histopathologic workup revealed an adrenocortical tumor of intermediate dignity. Postoperative tumor staging excluded both residual primary tumor and metastases. Both a normalization of body weight and myocardial mass were observed. The present article is, to our knowledge, the first to describe severe hypertrophic obstructive cardiomyopathy caused by an adrenocortical tumor and provides novel detailed data on postoperative glucocorticoid management.

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

    Directory of Open Access Journals (Sweden)

    Christian M Hagen

    Full Text Available 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. Several polymorphic mtDNA variants are associated with a variety of late-onset degenerative diseases and affect mitochondrial function. We examined the role of private, non-haplogroup associated, mitochondrial variants in the etiology of HCM. In 87 Danish HCM patients, full mtDNA sequencing revealed 446 variants. After elimination of 312 (69.9% non-coding and synonymous variants, a further 109 (24.4% with a global prevalence > 0.1%, three (0.7% haplogroup associated and 19 (2.0% variants with a low predicted in silico likelihood of pathogenicity, three variants: MT-TC: m.5772G>A, MT-TF: m.644A>G, and MT-CYB: m.15024G>A, p.C93Y remained. A detailed analysis of these variants indicated that none of them are likely to cause HCM. In conclusion, private mtDNA mutations are frequent, but they are rarely, if ever, associated with HCM.

  5. Standard guidelines of care: Keloids and hypertrophic scars

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    Somesh Gupta

    2011-01-01

    Full Text Available Keloids and hypertrophic scars (HTS are the result of overgrowth of fibrous tissue, following healing of a cutaneous injury, and cause morbidity. There are several treatment modalities which are useful for the management of keloids, though no single modality is completely effective. The most commonly used modalities are pressure, silicone gel sheet, intralesional steroids, 5-fluorouracil (5 FU, cryotherapy, surgical excision, and lasers. They may be used either singly or, as is done more commonly, in combinations. Any qualified dermatologist who has attained postgraduate qualification in dermatology can treat keloids and HTS. Some procedures, such as cryosurgery and surgical excision, may require additional training in dermatologic surgery. Most modalities for keloids, including intralesional injections and mechanical therapies such as pressure and silicone gel based products, can be given/prescribed on OPD basis. Surgical excision requires a minor operation theater with the facility to handle emergencies. It is important to counsel the patient about the nature of the problem. One should realize that keloid will only improve and not disappear completely. Patients should be informed about the high recurrence rates. Different modalities carry risk of adverse effects and complications and the treating physician needs to be aware of these and patients should be informed about them.

  6. Keloids and Hypertrophic Scars: Update and Future Directions

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    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.

  7. Hypertrophic Cardiomyopathy and Connective Tissue Dysplasia Syndrome: Comorbidity Variants

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    Yu. N. Belenkov

    2016-01-01

    Full Text Available Aim. To study the structure of co-morbidities, especially connective tissue undifferentiated dysplasia syndrome (CTDS, in patients with hypertrophic cardiomyopathy (HCM to develop an algorithm of complex examination of patients.Material and methods. Patients with HCM (n=186; 88 men and 78 women were examined. The diagnosis of HCM was based on current guidelines; molecular genetic study was performed in the absence of phenotypic manifestations. Echocardiography and standard examination of cardiac patient were performed in all patients to identify comorbidities. Genotyping of polymorphisms of 12 modifying genes was performed in 61 patients and 61 people in the control group.Results. HCM was most often associated with uterine myoma (52%, cardiac and extracardiac congenital malformations (50%, and thyroid diseases (37%. Combination of HCM with different variants of connective tissue dysplasia was found in 17% of patients (mitral valve prolapse – 6.3%, tricuspid valve prolapse – 2.7%, supplemental chords – 4.5%, bivalve aortic disease – 1.8%, increased left ventricular trabeculation – 3.6%, atrial septal aneurysm – 3.6%, membranous ventricular septal defect – 1.8%.Conclusion. CTDS is one of the most often associated disorders in patients with HCM. The study of the association of CTDS and HCM, the nature of their genetic structure and similarity of pathogenesis require further study.

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

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    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.

  9. Exercise heart rates in patients with hypertrophic cardiomyopathy.

    Science.gov (United States)

    Luo, Hong-Chang; Dimaano, Veronica L; Kembro, Jackelyn M; Hilser, Alex; Hurtado-de-Mendoza, David; Pozios, Iraklis; Tomas, Miguel S; Yalcin, Hulya; Dolores-Cerna, Ketty; Mormontoy, Wilfredo; Aon, Miguel A; Cameron, Duke; Bluemke, David A; Stewart, Kerry J; Russell, Stuart D; Cordova, Jorge G; Abraham, Theodore P; Abraham, M Roselle

    2015-04-15

    The exercise heart rate (HR) profile and its relation to cardiac function and arrhythmias were investigated in patients with hypertrophic cardiomyopathy (HC). Chronotropic response (CR) and heart rate recovery (HRR) were computed during and after treadmill exercise testing in 273 patients with HC and 95 age-matched healthy controls. Patients with HC had higher prevalence of chronotropic incompetence and lower HRR1-5min compared with controls. Exercise capacity, diastolic function (assessed by E/e') and left atrial volume index were associated with HRR1min and CR in HC. Septal myectomy was associated with reduction in chronotropic incompetence but did not affect HRR1min. In conclusion, impaired CR and HRR1min are associated with advanced disease and do not appear to be independent clinical markers indicating high-risk status in HC. Improving CR by titrating doses of negative chronotropic agents, myectomy, and atrial pacing may be useful to increase exercise capacity in patients with HC. Copyright © 2015 Elsevier Inc. All rights reserved.

  10. 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.)

  11. Metabolism of lipids in experimental hypertrophic hearts of rabbits

    Energy Technology Data Exchange (ETDEWEB)

    Revis, N.W.; Cameron, A.J.V.

    1979-06-01

    Cardiac hypertrophy was induced in rabbits by subcutaneous injection of thyroxine or isoprenaline or by surgically constricting the abdominal aorta. Alterations in lipid metabolism were observed in these hypertrophic hearts. Thyroxine or isoprenaline treatment increased the fatty acids in the serum and stimulated a marked increase in total lipids, triglycerides, and fatty acids in the hypertrophied myocardium. Coarctation of the aorta, in contrast, induced a significant increase in these lipids without significantly affecting serum free fatty acids. Histochemical and morphological studies confirmed an increase in neutral lipids. It is suggested that the observed increase in fatty acids in the heart following thyroxine or isoprenaline treatment is related to the increase in serum free fatty acids, which is followed by an increase in the removal of serum fatty acids by the heart. However, the amount of serum fatty acids that is removed exceeds the amount that is oxidized, which leads to an increase in lipid stores. The increase in lipid stores in the heart following coarctation of the aorta probably corresponds to the decrease in myocardial concentrations of carnitine. Serum lipid levels following coarctation were not significantly different from those of controls.

  12. Idiopathic hypertrophic pachymeningitis mimicking prolactinoma with recurrent vision loss.

    Science.gov (United States)

    Lok, Julie Y C; Yip, Nelson K F; Chong, Kelvin K L; Li, C L; Young, Alvin L

    2015-08-01

    Idiopathic hypertrophic pachymeningitis is a rare inflammatory condition with diffuse thickening of the dura mater, which may cause a compressive effect or vascular compromise. We report on a 28-year-old Chinese woman with a history of granulomatous mastitis 7 years previously and oligomenorrhoea, headache, blurred vision, and raised prolactin level 2 years previously, that was diagnosed as prolactinoma and treated conservatively with bromocriptine. However, she had recurrent bilateral vision loss when the bromocriptine was stopped. Her symptoms were resolved by high-dose steroid injection but remained steroid-dependent. Serial magnetic resonance imaging scan showed progressive diffuse thickening of the pachymeningitis with disappearance of pituitary apoplexy. Lumbar puncture showed lymphocytosis with no organisms. Open biopsy of the meninges was performed and histology showed features of inflammatory infiltrates and vasculitis. This is an unusual presentation of a rare condition in this age-group, with co-existing granulomatous mastitis and chronic otitis media, and is a diagnostic challenge mimicking pituitary macroadenoma and meningioma in initial magnetic resonance imaging scans.

  13. Hypertrophic cardiomyopathy: from gene defect to clinical disease

    Institute of Scientific and Technical Information of China (English)

    2003-01-01

    Major advances have been made over the last decade in our understanding of the molecular basis ofseveral cardiac conditions. Hypertrophic cardiomyopathy (HCM) was the first cardiac disorder in whicha genetic basis was identified and as such, has acted as a paradigm for the study of an inherited cardiacdisorder. HCM can result in clinical symptoms ranging from no symptoms to severe heart failure andpremature sudden death. HCM is the commonest cause of sudden death in those aged less than 35 years,including competitive athletes. At least ten genes have now been identified, defects in which cause HCM.All of these genes encode proteins which comprise the basic contractile unit of the heart, i.e. the sarcomere.While much is now known about which genes cause disease and the various clinical presentations, very littleis known about how these gene defects cause disease, and what factors modify the expression of the mutantgenes. Studies in both cell culture and animal models of HCM are now beginning to shed light on thesignalling pathways involved in HCM, and the role of both environmental and genetic modifying factors.Understanding these mechanisms will ultimately improve our knowledge of the basic biology of heart musclefunction, and will therefore provide new avenues for treating cardiovascular disease in man.

  14. Regulation of pyloric rhythm by IA and Ih in crayfish stomatogastric ganglion%IA和Ih对鳌虾口胃神经节幽门节律的调节

    Institute of Scientific and Technical Information of China (English)

    匡国晖; 刘一辉; 任维

    2012-01-01

    甲壳类动物的口胃神经节(stomatogastric ganglion,STG)由30个神经元组成,产生幽门节律,是研究中枢模式发生器(central pattern generator,CPG)的常用模型.幽门节律的维持与调控,不仅与STG神经元网络中单个神经元的特性有关,也与整个网络的连接及其性质相关.已有研究观察到STG网络中不同神经元上存在瞬时外向钾电流(transient potassium current,IA)与超极化激活阳离子电流(hyperpolarization-activated cation current,Ih),但这两种电流对整个STG网络节律的维持与调控作用仍不清楚.本实验采用鳌虾STG离体标本神经纤维记录,通过施加特异性阻断剂来研究IA和Ih对鳌虾STG幽门节律性放电的调节作用.结果显示:施加IA特异性阻断剂4AP(2 mmol/L)后,幽门节律周期变短(P<0.01),PD (pyloric dilator)细胞放电时程占整个三相周期的比率增加(P< 0.01),LP (lateral pyloric)细胞放电时程不变(P>0.05)但相位滞后,PY (pyloric)细胞放电时程占整个三相周期的比率减小(P<0.05)且相位滞后,三相顺序没有改变;施加Ih特异性阻断剂ZD7288 (100 μmol/L)后,幽门节律周期变短(P<0.01),PD细胞放电时程占整个三相周期的比率增加(P<0.01),LP细胞放电时程占整个三相周期的比率增加(P< 0.01)且相位滞后,PY细胞放电时程占整个三相周期的比率减小(P<0.01)且相位滞后,三相顺序没有改变.上述结果表明,IA和Ih对鳌虾STG幽门节律有重要的调节作用,进而影响到幽门活动的生理功能.%The stomatogastric ganglion (STG) of shellfish includes 30 neurons and produces pyloric rhythms. It is the common model to study central pattern generator (CPG). Regulation of pyloric rhythms not only is related to the property of single neurons in STG but also depends on the connections and property of the whole neuronal network. It has been found that transient potassium current (IA) and hyperpolarization-activated cation

  15. The Effects of Fetuin-A Levels on Aortic Stenosis

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    Ahmet Tutuncu

    2014-12-01

    Full Text Available Aim: We aimed to investigate the relation between fetuin-A and calcific aortic stenosis in non diabetic patients whose renal function were normal. Material and Method: 26 patients followed for aortic stenosis by our cardiology clinic for outpatients and 25 voluntary healthy subjects were included in the study. The fetuin%u2013A levels were measured from the venous blood samples of the study population. All patients underwent transthorasic echocardiography, the aortic valvular area and left ventricular parameters of the patients were measured. Results: The average age of the patients in degenerative aortic stenosis group was significantly higher than the control group. The parameters related to aortic valve were naturally higher in patients with dejenerative aortic valve. There was no siginificant difference between two groups about fetuin-A levels. Further more there was no significant relation between fetuin-a levels and aortic stenosis severity. Discussion: In conclusion fetuin-A is a multifunctional glycoprotein that plays important role in systemic calcification inhibition and valvular calcification. Finally aortic stenosis is an active process and larger studies that investigate the relation between fetuin-a and the progression and prognosis of aortic stenosis are needed.

  16. Treatment of vaginal stenosis with fasciocutaneous Singapore flap.

    Science.gov (United States)

    Clifton, Marisa M; Gurunluoglu, Raffi; Pizarro-Berdichevsky, Javier; Baker, Todd; Vasavada, Sandip P

    2017-03-01

    Vaginal stenosis is an unfortunate complication that can occur after pelvic radiation therapy for gynecologic or colorectal malignancies. Treatment is challenging and can require significant reconstructive surgery. The objective of this video is to present a case of vaginal stenosis after radiation and describe vaginal reconstruction with a fasciocutaneous Singapore flap. We describe the case of a 42-year-old woman with a history of stage 3 colorectal cancer who underwent partial colectomy, chemotherapy, and pelvic radiation. She subsequently developed a rectovaginal fistula requiring repair with a right-sided gracilis flap. When her stenosis recurred, she underwent vaginal reconstruction with a medial thigh flap. The Singapore flap is a pudendal thigh flap centered on the labial crural fold with a base at the perineal body. As the cutaneous innervation is spared, this flap is sensate. This technique is one option for patients with complex vaginal stenosis who have failed conservative management. However, it is imperative the patient perform vaginal dilation postoperatively and maintain close follow-up with her surgeon, as vaginal stenosis can recur. Postradiation vaginal stenosis is a complex condition to treat; however, vaginal reconstruction with a thigh flap can provide excellent cosmetic and functional results.

  17. [Clinical practice guideline. Traumatic urethral stenosis in males].

    Science.gov (United States)

    Serrano-Brambila, Eduardo Alonso; Moreno-Alcázar, Othón Martino; Neri-Páez, Edgar; Sánchez-Martínez, Luis Carlos; Hernández-Ordóñez, Octavio Francisco; Morales-Morales, Arturo; Basavilvazo-Rodríguez, M Antonia; Torres-Arreola, Laura del Pilar; Valenzuela-Flores, Adriana Abigail; Hernández-Valencia, Marcelino

    2013-01-01

    The incidence of urethral stenosis in Mexico had not been documented. At the Centro Médico Nacional La Raza, during the year 2010, 629 patients with urethral stenosis were attended as outpatient consultation: 85 % with previous urethral stenosis and 15 % with urethral treatment complication. Urethral stenosis is a chronic illness, with multiple etiological origins and the handling is controversial. It has a great negative impact for the patients and the recurrence reaches 85 %. The treatment consisted of an invasive approach (urethral dilations, endoscopy procedure) and open surgery (urethroplasty). The World Health Organization and World Alliance take the world challenge about the urinary tract infections associated with the attention of patients, focused on urethral stenosis. The objective of the following clinical guide is to offer to the health professional a clinical tool for making decisions in the handling of the hardship or masculine urethral stenosis, based on the best available evidence, carrying out in systematized form with bibliographical research using validated terms of the MeSH: urethral structures, in the databases Trip database, PubMed, Guideline Clearinghouse, Cochrane Library and Ovid.

  18. Middle cerebral artery stenosis associated with moyamoya pattern collateralization

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    Randall Edgell

    2010-11-01

    Full Text Available Background and Purpose: Moyamoya disease is a well described phenomenon presenting with terminal internal carotid artery occlusion and rete pattern of collateralization around the occlusion. The development of moyamoya-like collaterals secondary to isolated middle cerebral artery stenosis or occlusion and the natural history of this entity in Caucasians have not been well described. Methods: Cerebral angiograms and CT angiograms performed between August 2004 and August of 2006 demonstrating moyamoya collateralization at a single US center were retrospectively reviewed. All cases of middle cerebral artery stenosis associated with a rete pattern of collateralization were included in this series. Demographic, clinical, and angiographic data were obtained. Results: There were 3 cases of middle cerebral artery stenosis associated with a moyamoya pattern of collateralization. The average age of the patients was 36 years old, 2 were male, and all were Caucasian. All patients presented with ischemic symptoms. The average degree of stenosis was 91%. No stenosis was seen in the supraclinoid internal carotid arteries or elsewhere in the intracranial vasculature. Conclusion: We describe a moyamoya-like pattern of anastomosis associated with isolated severe middle cerebral artery stenosis or occlusion in Caucasians.

  19. Assessing Optimal Blood Pressure in Patients With Asymptomatic Aortic Valve Stenosis The Simvastatin Ezetimibe in Aortic Stenosis Study (SEAS)

    DEFF Research Database (Denmark)

    Nielsen, O. W.; Sajadieh, A.; Sabbah, M.

    2016-01-01

    BACKGROUND: Evidence for treating hypertension in patients with asymptomatic aortic valve stenosis is scarce. We used data from the SEAS trial (Simvastatin Ezetimibe in Aortic Stenosis) to assess what blood pressure (BP) would be optimal. METHODS: A total of 1767 patients with asymptomatic aortic...... stenosis and no manifest atherosclerotic disease were analyzed. Outcomes were all-cause mortality, cardiovascular death, heart failure, stroke, myocardial infarction, and aortic valve replacement. BP was analyzed in Cox models as the cumulative average of serially measured BP and a time-varying covariate...... to 69 mm Hg taken as reference. Low systolic and diastolic BPs increased risk in patients with moderate aortic stenosis. With a time-varying systolic BP from 130 to 139 mm Hg used as reference, mortality was increased for systolic BP ≥160 mm Hg (HR, 1.7; P=0.033) and BP of 120 to 129 mm Hg (HR, 1.6; P=0...

  20. Management of Renal Artery Stenosis - an Update

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    Alhadad A

    2008-01-01

    Full Text Available The role of the renal vasculature in eliciting renovascular hypertension (RVH was established in 1934, when Goldblatt et al. [1] in a classical experimental study demonstrated that partial obstruction of the renal artery increased mean arterial blood pressure (BP. The pathophysiology of renal artery stenosis (RAS is incompletely understood but has been postulated to be related to increased afterload from neurohormonal activation and cytokine release [2].Atherosclerotic RAS (ARAS is increasingly diagnosed in the expanding elderly population, which also has a high prevalence of arterial hypertension. There is still considerable uncertainty concerning the optimal management of patients with RAS. Many hypertensive patients with RAS have co-existing essential hypertension and furthermore, it is often difficult to determine to what degree the RAS is responsible for the impairment of renal function. There are three possible treatment strategies: medical management, surgery, or percutaneous transluminal renal angioplasty (PTRA with or without stent implantation. The use of stents has improved the technical success rate of PTRA and also led to lower risk of restenosis, in particular for ostial RAS. PTRA with stenting has therefore replaced surgical revascularisation for most patients with RAS and has led to a lower threshold for intervention. The treatment of choice to control hypertension in fibromuscular dysplasia (FMD is generally accepted to be PTRA [3]. In ARAS, on the other hand, the benefits with PTRA are less clear [4] and the challenge to identify which patients are likely to benefit from revascularisation remains unknown.

  1. Neurological manifestations of calcific aortic stenosis

    Directory of Open Access Journals (Sweden)

    I. V. Egorov

    2014-01-01

    Full Text Available Despite being thoroughly studied, senile aortic stenosis (AS remains a disease that is frequently underestimated by Russian clinicians. Meanwhile, its manifestations can not only deteriorate quality of life in patients, but can also be poor prognostic signs. The most common sequels of this disease include heart failure and severe arrhythmias. However, there may be also rare, but no less dangerous complications: enteric bleeding associated with common dysembriogenetic backgrounds, infarctions of various organs, the basis for which is spontaneous calcium embolism, and consciousness loss episodes. The latter are manifestations of cardiocerebral syndrome. Apart from syncope, embolic stroke may develop within this syndrome. There is evidence that after syncope occurs, life expectancy averages 3 years. Global practice is elaborating approaches to the intracardiac calcification prevention based on the rapid development of new pathogenetic ideas on this disease. In particular, it is clear that valvular calcification is extraskeletal leaflet ossification rather than commonplace impregnation with calcium salts, i.e. the case in point is the reverse of osteoporosis. This is the basis for a new concept of drug prevention of both calcification and the latter-induced heart disease. But the view of senile AS remains more than conservative in Russia. The paper describes a clinical case of a rare complication as cerebral calcium embolism and discusses the nature of neurological symptoms of the disease, such as vertigo and syncope.

  2. New polymorphisms in human MEF2C gene as potential modifier of hypertrophic cardiomyopathy.

    Science.gov (United States)

    Alonso-Montes, Cristina; Naves-Diaz, Manuel; Fernandez-Martin, Jose Luis; Rodriguez-Reguero, Julian; Moris, Cesar; Coto, Eliecer; Cannata-Andia, Jorge B; Rodriguez, Isabel

    2012-09-01

    Hypertrophic cardiomyopathy is caused by mutations in genes encoding sarcomeric proteins. Its variable phenotype suggests the existence of modifier genes. Myocyte enhancer factor (MEF) 2C could be important in this process given its role as transcriptional regulator of several cardiac genes. Any variant affecting MEF2C expression and/or function may impact on hypertrophic cardiomyopathy clinical manifestations. In this candidate gene approach, we screened 209 Caucasian hypertrophic cardiomyopathy patients and 313 healthy controls for genetic variants in MEF2C gene by single-strand conformation polymorphism analysis and direct sequencing. Functional analyses were performed with transient transfections of luciferase reporter constructions. Three new variants in non-coding exon 1 were found both in patients and controls with similar frequencies. One-way ANOVA analyses showed a greater left ventricular outflow tract obstruction (p = 0.011) in patients with 10C+10C genotype of the c.-450C(8_10) variant. Moreover, one patient was heterozygous for two rare variants simultaneously. This patient presented thicker left ventricular wall than her relatives carrying the same sarcomeric mutation. In vitro assays additionally showed a slightly increased transcriptional activity for both rare MEF2C alleles. In conclusion, our data suggest that 15 bp-deletion and C-insertion in the 5'UTR region of MEF2C could affect hypertrophic cardiomyopathy, potentially by affecting expression of MEF2C and therefore, the expression of their target cardiac proteins that are implicated in the hypertrophic process.

  3. 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.

  4. MicroRNA profiling in early hypertrophic growth of the left ventricle in rats

    DEFF Research Database (Denmark)

    Busk, Peter K.; Cirera, Susanna

    2010-01-01

    Pressure overload induces hypertrophic growth of the heart and in the long term this condition can lead to cardiomyopathy and heart failure. Several miRNAs are upregulated in heart failure. However, it is not clear, which miRNAs (if any) are induced during the early hypertrophic growth phase...... was highly induced in animals with aortic banding. Out of 13 miRs that have previously been reported to be associated with late-stage pressure-overload-induced hypertrophy and heart failure only four (miR-23a, miR-27b, miR-125b and miR-195) were induced during early hypertrophic growth. These miRs were....... To investigate whether the upregulation of miRNAs is an integrated part of hypertrophic growth or an effect of cardiac disease we investigated miRNA expression in early hypertrophic development. Hypertrophy was induced by banding of the ascending aorta of male rats. After 14 days, the heart left ventricle weight...

  5. Curative Effects of Oleanolic Acid on Formed Hypertrophic Scars in the Rabbit Ear Model

    Directory of Open Access Journals (Sweden)

    Hong Zhang

    2012-01-01

    Full Text Available Hypertrophic scarring is a common proliferative disorder of dermal fibroblasts characterized by collagen overproduction and excessive deposition of extracellular matrix (ECM. There is no consensus about the best therapeutics to produce complete and permanent improvement of scars with few side effects. To investigate the therapeutic effects of oleanolic acid (OA on hypertrophic scars and explore the possible mechanism of action involved, a rabbit ear model with hypertrophic scars was established. OA (2.5%, 5%, and 10% was given once daily to the scars for 28 consecutive days. As a result, OA significantly alleviated formed hypertrophic scars on rabbit ears. The levels of TGF-β1, MMP-1, TIMP-1, and collagens I and III were notably decreased, and the number of apoptosis cells and mRNA expression of MMP-2, caspase-3, and caspase-9 were markedly increased in the scar tissue. The scar elevation index (SEI was also evidently reduced. Histological findings exhibited significant amelioration of the collagen tissue. These results suggest that OA has the favorable curative effects on formed hypertrophic scars in the rabbit ear model, and the possible mechanism of action is that OA decreases HSFs proliferation and increases HSFs apoptosis by reduction of P311 gene expression and TGF-β1 production, inhibition of TIMP-1 secretion, enhancement of MMP-2 activity, and subsequently facilitation of degradation of collagen types I and III.

  6. ASSESSMENT OF DIASTOLIC FUNCTION IN PATIENTS WITH HYPERTROPHIC CARDIOMYOPATHY BY DOPPLER TISSUE IMAGING

    Institute of Scientific and Technical Information of China (English)

    Jing Li; Yan-ling Liu; Hao Wang; Xiu-zhang Lü; Hong-chang Yang; Fu-jian Duan; Zhen-hui Zhu

    2004-01-01

    Objective To determine the clinical application of pulsed Doppler tissue imaging in assessing the left ventricular diastolic function and in discriminating between normal subjects and patients with hypertrophic cardiomyopathy with various stages of diastolic dysfunction.Methods We measured the peak diastolic velocities of mitral annulus in 81 patients with hypertrophic cardiomyopathy with various stages of diastolic dysfunction and 50 normal volunteers by Doppler tissue imaging using the apical window at 2-chamber and long apical views, respectively. The myocardial velocities were determined with use of variance F statistical analysis.Results Early diastolic myocardial velocities ofmitral annulus were higher in normal subjects than in patients with hypertrophic cardiomyopathy with either delayed relaxation, pseudonormal filling, or restrictive filling. However, peak myocardial velocities of mitral annulus during atrial contraction were similar in normal subjects and patients with hypertrophic cardiomyopathy.Conclusion Doppler tissue imaging can directly reflect upon left diastolic ventricular function. Early phase of diastole was the best discriminator between control subjects and patients with hypertrophic cardiomyopathy.

  7. Assessment of Regional Myocardial Function in Patients with Hypertrophic Cardiomyopathy by Tissue Strain Imaging

    Institute of Scientific and Technical Information of China (English)

    XIONG Runqing; XIE Mingxing; WANG Xinfang; L(U) Qing

    2006-01-01

    The value of tissue strain imaging (SI) in regional myocardial systolic anddiastolic function assessment was studied. In 18 patients with nonobstructive hypertrophic cardiomyopathy (HCM) and 20 age-matched healthy subjects, regional myocardial longitudinal peak systolic strain in eject time (represented by εet) was measured at basal, mid and apical segments of septal, lateral and posterior walls of the left ventricle (LV) and compared between groups. εet had no significant difference between segments in control group (P>0.05), which displayed a decreasing trend from basal segments to apical ones. εet in the HCM group was significantly decreased (P<0. 05) as compared with that in the healthy group. In the HCM group, εet in the midseptum was significantly less than at the basal and apical septum, and was also less than at the rest LV walls in the same group (P<0.01). The systolic reversed εet was noticed in 35 % of the hypertrophic segments in HCM group. Significantly negative correlation existed between the absolute value of εet and wall thickness in the midseptum (r=- 0.83). The post-systolic strain(PSS) segment number the and amplitudes in healthy group were significantly less than those in HCM group (P<0.05). Both regional myocardial systolic and diastolic functions were impaired in hypertrophic or non-hypertrophic segments in patients with the HCM, especially in hypertrophic segments. Strain imaging technique is a sensitive and accura tool in myocardial dysfunction assessment.

  8. Effects of highly selective vagotomy plus resection of antral mucosa or highly selective vagotomy alone on motility function of pyloric antrum

    Institute of Scientific and Technical Information of China (English)

    文亚渊; 王代科; 刘宝华

    2004-01-01

    Objective: To study the effects of highly selective vagotomy plus resection of antral mucosa ( HSV + RAM)or highly selective vagotomy (HSV) alone on the motility function of the pyloric antrum. Methods: 48 patients with duodenal ulcer were studied. 18 dogs were employed as experimental animals. 20 patients were operated on with HSV and 28 with HSV + RAM. The frequency of gastric evacuation and the amplitude of electrogastrography were determined 4 to 6 months after operation. 18 dogs were divided into the control group, HSV group and HSV + RAM group. The time of gastric evacuation, antral myoelectric activity and antral pressure were determined in the dogs 4 to 6 months after operation. The preoperative findings of the patients and the control dogs served as the control. Results: After operation, barium meal revealed that the shape of the stomach and duodenum was normal and the gastric peristalsis was clearly visible in human patients and experimental dogs. In the HSV + RAM group of dogs, the initial evacuation time was (5.0 + 0.06) min and the time of complete evacuation was (4.0 + 0.4) h after food-taking, which were similar to those of the control and the HSV group of dogs(P>0.05). The frequency of the antral myoelectric action potential was (3.11 + 0.65) cycles/min in the dog HAS + RAM group and the frequency of electrogastrography was (3.25 + 0.75) cycles/min in the human HSV + RAM group, which were significantly lower than those of the control and the dog and human HSV groups (P < 0.05). Injection of pentagastrin in dogs and food-taking in human beings significantly increased the antral pressure and the amplitude and frequency of electrogastrography. Conclusion: The findings of this study suggest that the motility function of the reconstructed pyloric antrum in the HSV + RAM group of both the experimental dogs and human patients approaches to the normal even though there is a decrease of antral myoelectric frequency. It is suggested that HSV + RAM should

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

    Directory of Open Access Journals (Sweden)

    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.

  10. Smac/DIABLO regulates the apoptosis of hypertrophic scar fibroblasts.

    Science.gov (United States)

    Liu, Bao-Heng; Chen, Liang; Li, Shi-Rong; Wang, Zhen-Xiang; Cheng, Wen-Guang

    2013-09-01

    In abnormal skin wound healing, hypertrophic scars (HS) are characterized by excessive fibroblast hypercellularity and an overproduction of collagen, leading to atypical extracellular matrix (ECM) remodeling. Although the exact mechanisms of HS remain unclear, decreased HS fibroblast (HSFB) apoptosis and increased proliferation are evident in the development of HS. In this study, the contribution of the second mitochondria-derived activator of caspases/direct inhibitor of apoptosis protein (IAP)-binding protein with a low isoelectric point (pI) (Smac/DIABLO), an apoptosis-promoting protein released from the mitochondria, was investigated in human normal skin and HSFB cultures. The expression of Smac/DIABLO is usually decreased in many malignant tumors compared with normal tissues. Immunohistochemical analysis of skin tissues and the western blot analyses of fibroblasts revealed that the expression of Smac/DIABLO was lower in HS tissues compared with normal skin tissues. Of note, adenovirus-mediated Smac/DIABLO overexpression in the cultured HSFBs significantly reduced cell proliferation, as detected by the cell counting kit-8, and increased caspase-3 and -9 activity, as detected by spectrofluorimetry. In addition, it increased apoptosis, as detected by fluorescence-activated cell sorting (FACS). Furthermore, we found that the silencing of Smac with siRNA in the HSFBs induced a noticeable decrease in caspase-3 and -9 activity, leading to a significant reduction in apoptosis. In addition, the mRNA expression of type I and III pro-collagen detected in the HSFBs was significantly increased following the silencing of Smac with siRNA and was inhibited following Smac/DIABLO overexpression, as shown by real-time RT-PCR. In conclusion, Smac/DIABLO decreases the proliferation and increases the apoptosis of HSFBs. To our knowledge, the data from our study suggest for the first time that Smac/DIABLO is a novel therapeutic target for HS.

  11. 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.

  12. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  14. Quantitative measurement of hypertrophic scar: interrater reliability and concurrent validity.

    Science.gov (United States)

    Nedelec, Bernadette; Correa, José A; Rachelska, Grazyna; Armour, Alexis; LaSalle, Léo

    2008-01-01

    Research into the pathophysiology and treatment of hypertrophic scar (HSc) remains limited by the heterogeneity of scar and the imprecision with which its severity is measured. The objective of this study was to test the interrater reliability and concurrent validity of the Cutometer measurement of elasticity, the Mexameter measurement of erythema and pigmentation, and total thickness measure of the DermaScan C relative to the modified Vancouver Scar Scale (mVSS) in patient-matched normal skin, normal scar, and HSc. Three independent investigators evaluated 128 sites (severe HSc, moderate or mild HSc, donor site, and normal skin) on 32 burn survivors using all of the above measurement tools. The intraclass correlation coefficient, which was used to measure interrater reliability, reflects the inherent amount of error in the measure and is considered acceptable when it is >0.75. Interrater reliability of the totals of the height, pliability, and vascularity subscales of the mVSS fell below the acceptable limit ( congruent with0.50). The individual subscales of the mVSS fell well below the acceptable level (0.89) for each study site with the exception of severe scar. Mexameter and DermaScan C reliability measurements were acceptable for all sites (>0.82). Concurrent validity correlations with the mVSS were significant except for the comparison of the mVSS pliability subscale and the Cutometer maximum deformation measure comparison in severe scar. In conclusion, the Mexameter and DermaScan C measurements of scar color and thickness of all sites, as well as the Cutometer measurement of elasticity in all but the most severe scars shows high interrater reliability. Their significant concurrent validity with the mVSS confirms that these tools are measuring the same traits as the mVSS, and in a more objective way.

  15. Clinical Analysis of 42 Patients With Hypertrophic Cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    Objectives To investigate the clinical manifestations of hypertrophic cardiomyopathy (HCM), and to find out the clinical clues to avoid misdiagnosis and provide reference for future clinical diagnosis and treatment. Methods A retrospective analysis of 42 consecutive patients with HCM hospitalized in our hospital between January 1995 and December 2002 was explored. Based on the family history of HCM, clinical manifestations, electrocardiogram, echocardiogram, coronary angiography and left ventriculography, the clinical characteristics between HCM patients with left ventricular outflow tract obstruction (HOCM) and HCM patients without obstruction (HNOCM) were compared. The causes of misdiagnosis and losing diagnosis were analysis.Results 13 patients were in HOCM group and 29 patients were in HNOCM group. More patients with syncope were in HOCM group than in HNOCM group (6/13 vs. 2/29, P < 0.05). Patients with ejective murmur were in HOCM group only (P < 0.01). Left ventricular outflow tract pressure gradient (LVOTPG)only observed in HOCM group (P<0.01). Ventricular tachycardia was seen in both groups. 28 out of 42 patients (66.67%) had misdiagnosis, and 4 out of 42 patients (9.53%) had losing diagnosis. Thus, coronary heart disease (CHD) had the highest rate of misdiagnosis. There were 20 CHD patients (71.43%)among 28 patients with misdiagnosis. Hypertension was in 3, congenital heart in 2, cerebro-embolism in 2, and myocarditis in 1. Conclusions For a patient with family history or sudden death history of HCM,unexplained syncope episodes, chest pain (angina),especially in young, an ejection murmur along the left sternum border, the presence of narrow and deep Q waves, or inversion of giant T waves in V3-V6, atrial fibrillation and /or cerebra-embolism echocardiogram should be given. CAG and LVG are necessary only if the result of echo is negative, and the patients with suspected HCM or CHD.

  16. Characteristics of hypertrophic pachymeningitis in patients with granulomatosis with polyangiitis.

    Science.gov (United States)

    Choi, Hyun Ah; Lee, Mi Ji; Chung, Chin-Sang

    2017-04-01

    Hypertrophic pachymeningitis (HP) is an important neurologic complication of granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis). The aim of this study is to investigate the clinical features, radiological findings, and diagnostic pitfalls of GPA-related HP. A retrospective chart review was performed to screen patients diagnosed with GPA at Samsung Medical Center between 1997 and 2016. Neurologic manifestation, laboratory findings, neuroimaging data, and clinical course were evaluated in all patients. Characteristics of patients with HP were compared to those of patients without HP. Sixty-five patients with GPA were identified. Twenty-five of these patients had central nervous system involvement. HP (N = 9, 36%) was the second most common radiologic finding. Other neurologic findings included stroke (N = 7, 28%) and granulomatous disease (N = 10, 40%). Patients with HP had lower incidences of systemic manifestations (N = 2, 22.2% vs. N = 38, 67.9%, p = 0.013 in the lung and N = 1, 11.1% vs. N = 28, 50.0%, p = 0.030 in the kidney) than those without HP. Six patients with GPA-related HP were MPO-ANCA positive (66.7%) and two had PR3-ANCA (22.2%). Most of the patients with HP presented with headache (N = 8, 88.9%) at a rate that is similar to those of primary headache disorders (migraine, tension-type, and stabbing) and other secondary headache disorders (postural type and meningitis). Patients with HP rarely had neurologic deficits (N = 3, 37.5%). Different clinical or radiologic features may be observed in GPA-related HP. Early recognition and accurate diagnosis of GPA-related HP are needed in addition to neuroimaging findings.

  17. A myomesin mutation associated with hypertrophic cardiomyopathy deteriorates dimerisation properties.

    Science.gov (United States)

    Siegert, Romy; Perrot, Andreas; Keller, Sandro; Behlke, Joachim; Michalewska-Włudarczyk, Aleksandra; Wycisk, Anna; Tendera, Michal; Morano, Ingo; Ozcelik, Cemil

    2011-02-18

    Myomesin plays an important structural and functional role in the M-band of striated muscles. The C-terminal domain 13 of myomesin dimerises and forms antiparallel strands which cross-link neighboring Myosin filaments and titin in the M-line of the sarcomeres. These interactions stabilise the contractile apparatus during striated muscle contraction. Since myomesin is an important component of the M-band we screened the myomesin gene for genetic variants in patients with hypertrophic cardiomyopathy (HCM). We identified the missense mutation V1490I in domain 12 of myomesin in a family with inherited HCM. Analytical ultracentrifugation experiments, circular dichroism spectra, and surface plasmon resonance spectroscopy of myomesin fragments were carried out to investigate the effects of the mutation V1490I on structure and function of myomesin domains 11-13 and 12-13. Both the wild type and mutated myomesin domains My11-13 revealed similar secondary structures and formed stable dimers. Mutated myomesin domains My11-13 and My12-13 dimers revealed a reduced thermal stability and a significantly decreased dimerisation affinity, showing disturbed functional properties of V1490I mutated myomesin. However, monomeric myomesin domains My11-12, i.e. without dimerisation domain 13 showed no difference in thermal stability between wild type and V1490I mutated myomesin. In conclusion, the V1490I mutation associated with HCM lead to myomesin proteins with abnormal functional properties which affect dimerisation properties of myomesin domain 13. These effects may contribute to the pathogenesis of HCM. Copyright © 2011 Elsevier Inc. All rights reserved.

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

    Directory of Open Access Journals (Sweden)

    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.

  19. Clinical analysis of 121 patients with hypertrophic cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    Shilu Zhao; Hengfang Wu; Jizhen Ma; Xiangjian Chen; Junhong Wang; Di Yang; Jinan Zhang

    2008-01-01

    Objective:Several studies have analyzed the clinical profiles of patients diagnosed with hypertrophic cardiomyopathy(HCM). We sought to identify its characteristics in a regional cohort of Nanjing and its adjacent region. Methods:Clinical profiles of 121 referred patients were collected and analyzed retrospectively. Data including family history, clinical symptoms, electrocardiography and recent echocardiography were collected. Results: The mean age of this population was 42±17 years(range from 6 to 76) at diagnosis of HCM. Most patients were male(60%). 48 patients(39.7%) has a family history, 19 had a sudden death in a first degree relative and 96(792,%) were recognized with cardiac symptoms. Left ventricular outflow obstruction (gradient≥30 mmHg at rest) was presented in 26(21.5%) patients. ECG abnormalities comprised of arrhythmia in 54(51.4%) and abnormal T wave in 72(68.6%) patients. FS were higher in female than male(P=0.001). Among younger patients(age ≤ 50 years), LVDd and LVWP were smaller in females than males(P=0.042 & 0.023 respectvely). In older patients(age > 50 years), LVDs was higher in male(P= 0.016) and EF was higher in female (P=0.048). Conclusion:HCM patients in the region are almost diagnosed with the presentation of cardiac symptoms; those without any symptoms could be recognized by ECG and family screening. Most cardiac hypertrophy affects the interventricular septum. LVDd, LVWP, LVDs, FS and EF showed significant differences related to age and gender.

  20. Angiotensin converting enzyme gene polymorphism in familial hypertrophic cardiomyopathy patients

    Energy Technology Data Exchange (ETDEWEB)

    Yu, B; Peric, S.; Ross, D. [Royal Prince Alfred Hospital, Campertown (Australia)] [and others

    1994-09-01

    An insertion/deletion (I/D) polymorphism of the angiotensin I converting enzyme (ACE) gene is a useful predictor of human plasma ACE levels. ACE levels tend to be lowest in subjects with ACE genotype DD and intermediate in subjects with ACE genotype ID. Angiotensin II (Ang II) as a product of ACE is a cardiac growth factor and produces a marked hypertrophy of the chick myocyte in cell culture. Rat experiments also suggest that a small dose of ACE inhibitor that does not affect the afterload results in prevention or regression of cardiac hypertrophy. In order to study the relationship of ACE and the severity of hypertrophy, the ACE genotype has been determined in 28 patients with a clinical diagnosis of familial hypertrophic cardiomyopathy (FHC) and 51 normal subjects. The respective frequencies of I and D alleles were: 0.52 and 0.48 (in FHC patients) and 0.44 and 0.56 (in the normal controls). There was no significant difference in the allele frequencies between FHC and normal subjects ({chi}{sup 2}=0.023, p>0.05). The II, ID, and DD genotypes were present in 7, 15, and 6 FHC patients, respectively. The averages of maximal thickness of the interventricular septum measured by echocardiography or at autopsy were 18 {plus_minus}3, 19{plus_minus}4, and 19{plus_minus}3 mm in II, ID and DD genotypes, respectively. The ACE gene polymorphism did not correlate with the severity of left ventricular hypertrophy in FHC patients (r{sub s}=0.231, p>0.05). These results do not necessarily exclude the possible effect of Ang II on the hypertrophy since the latter may be produced through the action of chymase in the human ventricles. However, ACE gene polymorphism is not a useful predictor of the severity of myocardial hypertrophy in FHC patients.

  1. Spinal stenosis surgery in pediatric patients with achondroplasia.

    Science.gov (United States)

    Sciubba, Daniel M; Noggle, Joseph C; Marupudi, Neena I; Bagley, Carlos A; Bookland, Markus J; Carson, Benjamin S; Ain, Michael C; Jallo, George I

    2007-05-01

    Achondroplasia is a hereditary form of dwarfism caused by a defect in endochondral bone formation, resulting in skeletal abnormalities including short stature, shortened limb bones, macrocephaly, and small vertebral bodies. In the pediatric population, symptomatic spinal stenosis occurs at all spinal levels due to the abnormally narrow bone canal. In this study, clinical outcomes were assessed in children with achondroplasia after spinal canal decompression. A retrospective review was conducted involving pediatric patients with heterozygous achondroplasia and symptomatic stenosis after decompressive procedures at the authors' institution within a 9-year period. Measured outcomes included resolution of symptoms, need for repeated surgery, presence of fusion, development of deformity, and complications. Forty-four pediatric patients underwent a total of 60 decompressive procedures. The average patient age at surgery was 12.7 years (range 5-21 years). Forty-nine operations were performed for initial treatment of stenosis, and 11 were performed as revision surgeries on previously operated levels. A large proportion of patients (> 60%) required additional cervicomedullary decompressions, most often preceding the symptoms of spinal stenosis. Of the initial procedures, decompression locations included 32 thoracolumbar (65%), 10 lumbar (20%), four cervical (8%), two cervicothoracic (4%), and one thoracic (2%). Forty-three of the decompressive procedures (72%) included spinal fusion procedures. Of the 11 revisions, five were fusion procedures for progressive deformity at levels previously decompressed but not fused (all thoracolumbar), five were for decompressions of symptomatic junctional stenosis with extension of fusion, and one was for repeated decompression at the same level due to recurrence of symptomatic stenosis. Decompression of the spinal canal in pediatric patients with achondroplasia can be accomplished safely with significant clinical benefit. Patients with a

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

    Science.gov (United States)

    Liu, H; Gisquet, H; Blondel, W; Guillemin, F

    2012-12-01

    This study aims at investigating the efficiency of bimodal spectroscopy in detection of hypertrophic scar tissue on a preclinical model. Fluorescence and Diffuse Reflectance spectra were collected from 55 scars deliberately created on ears of 20 rabbits, amongst which some received tacrolimus injection to provide non-hypertrophic scar tissue. The spectroscopic data measured on hypertrophic and non-hypertrophic scar tissues were used for developing our classification algorithm. Spectral features were extracted from corrected data and analyzed to classify the scar tissues into hypertrophic or non-hypertrophic. The Algorithm was developed using k-NN classifier and validated by comparing to histological classification result with Leave-One-Out cross validation. Bimodal spectroscopy showed promising results in detecting hypertrophic tissue (sensibility 90.5%, specificity 94.4%). The features used for classification were extracted from the autofluorescence spectra collected at 4 CEFS with excitations at 360, 410, and 420 nm. This indicates the hypertrophic process may involve change in concentration of several fluorophores (collagen, elastin and NADH) excited in this range, or modification in volume of explored tissue layers (epidermis and dermis) due to tissue thickening.

  3. Acute effects of sildenafil and dobutamine in the hypertrophic and failing right heart in vivo

    DEFF Research Database (Denmark)

    Andersen, Asger; Nielsen, Jan M; Rasalingam, Sivagowry

    2013-01-01

    Abstract The purpose of this study was to investigate whether acute intravenous administration of the phosphodiesterase type 5 (PDE-5) inhibitor sildenafil in a single clinically relevant dose improves the in vivo function of the hypertrophic and failing right ventricle (RV). Wistar rats ([Formula...... significant hypertrophy, cardiac fibrosis, and reduction in RV function evaluated by echocardiography (TAPSE) and invasive pressure measurements. Sildenafil did not improve the function of the hypertrophic failing right heart in vivo, measured by TAPSE, RV systolic pressure (RVsP), and dp/dtmax. Dobutamine...... administration of the PDE-5 inhibitor sildenafil in a single clinically relevant dose did not modulate the in vivo function of the hypertrophic failing right heart of the rat measured by echocardiography and invasive hemodynamics. In the same model, dobutamine acutely improved RV function....

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

    Directory of Open Access Journals (Sweden)

    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

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

    Energy Technology Data Exchange (ETDEWEB)

    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)

  6. The evidence for medicine versus surgery for carotid stenosis

    Energy Technology Data Exchange (ETDEWEB)

    Ederle, Joerg [Stroke Research Group, UCL Institute of Neurology, London (United Kingdom); Brown, Martin M. [Stroke Research Group, UCL Institute of Neurology, London (United Kingdom)]. E-mail: m.brown@ion.ucl.ac.uk

    2006-10-15

    Atherosclerotic stenosis of the internal carotid artery is an important cause of stroke. Several large randomised trials have compared best medical management with carotid endarterectomy and provide a strong evidence base for advising and selecting patients for carotid surgery. Best medical management of carotid stenosis includes lowering of blood pressure, treatment with statins and antiplatelet therapy in symptomatic patients. Combined analysis of the symptomatic carotid surgery trials, together with observational data, has shown that patients with recently symptomatic severe carotid stenosis have a very high risk of recurrent stroke in the first few days and weeks after symptoms. Carotid endarterectomy has a risk of causing stroke or death at the time of surgery in symptomatic patients of around 5-7%, but in patients with recently symptomatic stenosis of more than 70%, the benefits of endarterectomy outweigh the risks. In patients with moderate stenosis of between 50 and 69%, the benefits may justify surgery in patients with very recent symptoms, and in patients older than 75 years within a few months of symptoms. Patients with less than 50% stenosis do not benefit from surgery. In asymptomatic patients, or those whose symptoms occurred more than 6 months ago, the benefits of surgery are considerably less. Patients with asymptomatic stenosis treated medically only have a small risk of future stroke when treated medically of about 2% per annum. If carotid endarterectomy can be performed safely with a perioperative stroke and death rate of no more than 3%, then the randomised trials showed a significant benefit of surgery over 5 years follow-up, with an overall reduction in the risk of stroke from about 11% over 5 years down to 6%. However, of 100 patients operated, only 5 will benefit from avoiding a stroke over 5 years. The majority of neurologists have concluded that this does not justify a policy of routine screening and endarterectomy for asymptomatic

  7. The spectrum of soft tissue and skeletal abnormalities of hypertrophic osteoarthropathy.

    Science.gov (United States)

    Pineda, C; Fonseca, C; Martinez-Lavin, M

    1990-05-01

    We review our radiographic experience with hypertrophic osteoarthropathy. Soft tissue abnormalities such as finger clubbing, "elephant feet" and cutis verticis gyrata are well appreciated by plain radiography. Hypertrophic osteoarthropathy is characterized by a bone remodeling process at the tip of the digits. In older patients it is manifested as tuftal hypertrophy whereas in younger individuals acroosteolysis takes place. Radiographic signs of inflammatory arthropathy are conspicuously absent. Periosteal proliferation is an orderly evolving process in 3 dimensions: in the number of affected bones, in the area of involvement of a given bone and in the shape of periostitis.

  8. [The thickness/radius ratio (t/r) in patients with dilated and hypertrophic cardiomyopathy].

    Science.gov (United States)

    Guadalajara, J F; Valenzuela, F; Martínez Sánchez, C; Huerta, D

    1990-01-01

    We studied 17 patients with cardiomyopathy (10 hypertrophic and 7 dilated). With two-dimensional echocardiography, we obtained a short axis view at the level of papillary muscle. We calculated the ratio between thickness (h), of ventricular wall and cavity's radius (r) in diastole and systole (h/r ratio). Hypertrophic cardiomyopathy has a high h/r ratio in diastole (inappropriate hypertrophy), hypercontractility and low and systolic wall stress. Dilated cardiomyopathy has a low diastolic h/r ratio (inadequate hypertrophy) with low contractility and elevated end-systolic, wall stress. We discuss the mechanisms and consequences of different patterns of hypertrophy on the ventricular performance.

  9. Cytomegalovirus-induced transient protein-losing hypertrophic gastropathy in an immunocompetent adult.

    Science.gov (United States)

    Suter, W R; Neuweiler, J; Borovicka, J; Binek, J; Fantin, A C; Meyenberger, C

    2000-01-01

    Transient protein-losing hypertrophic gastropathy with similarity to Ménétrier's disease is described. Acute infection with cytomegalovirus (CMV) could be shown to play a causative role. Immunodeficiency was ruled out. The 34-year-old patient had complete resolution of the disease without antiviral treatment. To our knowledge the present report is the first case of CMV-associated protein-losing hypertrophic gastropathy in an immunocompetent adult. To date, a similar disorder has only been described in children. CMV infection should be considered in patients with acute and symptomatic protein loss of gastrointestinal origin. Copyright 2000 S. Karger AG, Basel

  10. Characterization of the cephalic arch and location of stenosis

    Science.gov (United States)

    Bennett, Shelby; Hammes, Mary S.; Blicharski, Tom; Watson, Sydeaka; Funaki, Brian

    2015-01-01

    Purpose The purpose of this study is to accurately characterize the cephalic arch segments into four domains and to enable more specific evaluation of cephalic arch stenosis (CAS) and determine the frequency of stenosis in each domain. Methods After Institutional Review Board (IRB) approval, a retrospective chart review was done to define a population of patients receiving hemodialysis who developed CAS as apparent on clinically indicated radiologic imaging. A standardized approach was devised to categorize four domains of the cephalic arch. Domain I was defined as the peripheral portion of the arch and Domain IV was the distal portion of the cephalic vein near termination with the axillary vein. The magnitude of stenosis as measured by percentage was determined and compared in the four domains. Results The most frequent location for stenosis was found in domain IV when compared with domains II or I (p<0.01). The magnitude of stenosis differed across all domains (p<0.001) with the least common place for CAS in domain I. Treatment of CAS included angioplasty in all, thrombectomy in eight, and stent placement in five. Conclusions CAS occurs most commonly in the terminal portion of the arch. Four standardized domains have been defined; future work will validate these findings and determine the best intervention for each domain. PMID:25198819

  11. [Two-stage surgical treatment of urethral stenosis].

    Science.gov (United States)

    Aboutaieb, R; Joual, A; El Moussaoui, A; El Mrini, M; Benjelloun, S

    1995-01-01

    We analysed the results of two-operation procedures for cutaneous urethroplasty performed over a 10 year period. From 1983 to 1993, 44 patients had a Leadbetter urethroplasty. The indication in all cases was stenosis of the bulbal or bulbomenbranous urethra complicated by urethro-cutaneous fistula. After the first operation, 10 patients (22.7%) had a stenosis of one or both of the urethrostomy orifices, requiring one or more further procedures. The second operation concerned 18 patients (40.9%) and gave goods results in 10 (55.5%). Poor results were due to recurrence of the stenosis in 3 cases (16.6%), perineal suppuration in 1 (5.5%), failure of the skin plasty in 1 (5.5%) and formation of a stenosis by a tuft of hair in 1 (5.5%). Finally, 2 patients were lost to follow-up. Mean interval between operations was 8 months. It appears, despite the drawbacks and uncertain results, that sequential operations for cutaneous urethroplasty are indicated when stenosis of the urethra is complicated by fistulization or perineal infection.

  12. Restenosis following balloon dilation of benign esophageal stenosis

    Institute of Scientific and Technical Information of China (English)

    Ying-Sheng Cheng; Ming-Hua Li; Ren-Jie Yang; Hui-Zhen Zhang; Zai-Xian Ding; Qi-Xin Zhuang; Zhi-Ming Jiang; Ke-Zhong Shang

    2003-01-01

    AIM: To elucidate the mechanism of restenosis following balloon dilation of benign esophageal stenosis.METHODS: A total of 49 rats with esophageal stenosis were induced in 70 rats using 5 ml of 50 % sodium hydroxide solution and the double-balloon method, and an esophageal restenosis (RS) model was developed by esophageal stenosis using dilation of a percutaneous transluminal coronary angioplasty (PTCA) balloon catheter. These 49 rats were divided into two groups: rats with benign esophageal stricture caused by chemical burn only (control group, n=21) and rats with their esophageal stricture treated with balloon catheter dilation (experimental group, n=28). Imaging analysis and immunohistochemistry were used for both quantitative and qualitative analyses of esophageal stenosis and RS formation in the rats, respectively.RESULTS: Cross-sectional areas and perimeters of the esophageal mucosa layer, muscle layer, and the entire esophageal layers increased significantly in the experimental group compared with the control group. Proliferating cell nuclear antigen (PCNA) was expressed on the 5th day after dilation, and was still present at 1 month. Fibronectin (FN)was expressed on the 1st day after dilation, and was still present at 1 month.CONCLUSION: Expression of PCNA and FN plays an important role in RS after balloon dilation of benign esophageal stenosis.

  13. 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.).

  14. Role of 7,8-dimethoxycoumarin in anti-secretary and anti-inflammatory action on pyloric ligation-induced gastritis in rats.

    Science.gov (United States)

    Sood, Shailja; Muthuraman, Arunachalam; Gill, Naresh Singh; Bali, Manoj; Sharma, Pritam Dev

    2010-07-01

    The present study was designed to investigate the effect of 7,8-dimethoxycoumarin (DMC) isolated from ethyl acetate extract of Citrus decumana peels on gastritis in rats. Isolation of 7,8-DMC from ethyl acetate extract of C. decumana peels was done by column and preparative thin layer chromatography using different solvents on polarity basis. Furthermore, effect of 7,8-DMC (50, 75, and 100 mg/kg, i.p.) in pyloric ligation-induced gastritis was studied in rats. The highest dose of 7,8-DMC showed significant decrease in the gastric volume, total acidity, ulcerative index, thiobarbituric acid reactive species levels, and myeloperoxidase activity, whereas there was an increase in the glutathione level. However, the lowest and medium doses did not produce significant results as compared to omeprazole and N-acetyl cysteine-treated groups. Compound 7,8-DMC (100 mg/kg) showed ameliorative effect on gastric inflammation and may be used as a therapeutic agent in the treatment of gastritis.

  15. Prognostic importance of atrial fibrillation in asymptomatic aortic stenosis

    DEFF Research Database (Denmark)

    Greve, Anders; Gerdts, Eva; Boman, Kurt

    2013-01-01

    BACKGROUND: The frequency and prognostic importance of atrial fibrillation (AF) in asymptomatic mild-to-moderate aortic stenosis (AS) has not been well described. METHODS: Clinical examination, electrocardiography and echocardiography were obtained in asymptomatic patients with mild-to-moderate A......BACKGROUND: The frequency and prognostic importance of atrial fibrillation (AF) in asymptomatic mild-to-moderate aortic stenosis (AS) has not been well described. METHODS: Clinical examination, electrocardiography and echocardiography were obtained in asymptomatic patients with mild......-to-moderate AS and preserved left ventricular (LV) systolic function, randomized to simvastatin/ezetimibe combination vs. placebo in the Simvastatin and Ezetimibe in Aortic Stenosis (SEAS) study. At inclusion, AF was categorized as episodic or longstanding. Rhythm change was assessed on annual in-study electrocardiograms...

  16. Effects of non Newtonian spiral blood flow through arterial stenosis

    Science.gov (United States)

    Hasan, Md. Mahmudul; Maruf, Mahbub Alam; Ali, Mohammad

    2016-07-01

    The spiral component of blood flow has both beneficial and detrimental effects in human circulatory system. A numerical investigation is carried out to analyze the effect of spiral blood flow through an axisymmetric three dimensional artery having 75% stenosis at the center. Blood is assumed as a Non-Newtonian fluid. Standard k-ω model is used for the simulation with the Reynolds number of 1000. A parabolic velocity profile with spiral flow is used as inlet boundary condition. The peak values of all velocity components are found just after stenosis. But total pressure gradually decreases at downstream. Spiral flow of blood has significant effects on tangential component of velocity. However, the effect is mild for radial and axial velocity components. The peak value of wall shear stress is at the stenosis zone and decreases rapidly in downstream. The effect of spiral flow is significant for turbulent kinetic energy. Detailed investigation and relevant pathological issues are delineated throughout the paper.

  17. Complete vaginal outlet stenosis in a patient with Sheehan's syndrome.

    Science.gov (United States)

    Choo, Minji; Park, Hana; Yi, Kyong Wook

    2016-11-01

    We present a case of complete vaginal stenosis in a woman diagnosed with Sheehan's syndrome. The patient delivered at full-term 5 months prior, and experienced massive postpartum bleeding at that time. During evaluation of persistent amenorrhea, we found that her vaginal orifice was completely adhesive and obstructed. Prior to corrective surgery, we managed the patient with an oral contraceptive to induce uterine bleeding into the vaginal outflow tract. After three cycles of an oral contraceptive, we could confirm that there was no stenotic lesion in the vaginal cavity as a hematocolpos was created. Adhesiolysis with scar revision for the vaginal stenosis was successfully performed; it was found that the lesion was limited to only the distal part of the vaginal outlet. Complete vaginal stenosis in reproductive age women with hypopituitarism has not been reported. The artificial induction of hematometrocolpos before surgery was useful in determining the extent of the stenotic lesion, and assured safety.

  18. Drug-eluting stents in renal artery stenosis

    Energy Technology Data Exchange (ETDEWEB)

    Zaehringer, M. [Marienhospital Stuttgart, Department of Radiology, Stuttgart (Germany); Pattynama, P.M.T. [Erasmus MC-University Medical Center Rotterdam, Rotterdam (Netherlands); Talen, A. [genae associates nv, Antwerp (Belgium); Sapoval, M. [Hopital Europeen Georges Pompidou, Service de Radiologie Cardio-Vasculaire, Paris (France); Inserm U 780 epidemiologie Cardio Vasculaire, Paris (France)

    2008-04-15

    Because of higher acute and long-term success rates compared with balloon angioplasty alone, percutaneous stent implantation has become an accepted therapy for the treatment of atherosclerotic renal artery stenosis. Restenosis rates after successful renal stent placement vary from 6 up to 40%, depending on the definition of restenosis, the diameter of the treated vessel segment and comorbidities. The safety and efficacy of drug-eluting stents for the treatment of renal-artery stenosis is poorly defined. The recently published GREAT study is the only prospective study, comparing bare-metal and sirolimus-coated low profile stent systems in renal artery stenosis, showing a relative risk reduction of angiographic binary in-stent restenosis by 50%. This is an opinion paper on indications, current treatment options and restenosis rates following renal artery stenting and the potential use of drug-eluting stents for this indication. (orig.)

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

    Directory of Open Access Journals (Sweden)

    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

  20. 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

  1. Clinical Utility of Cardiovascular Magnetic Resonance in Hypertrophic Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Maron Martin S

    2012-02-01

    Full Text Available Abstract 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

  2. 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

  3. 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.)

  4. CT Angiographic Analysis of Carotid Artery Stenosis: Comparison of Manual Assessment, Semiautomatic Vessel Analysis, and Digital Subtraction Angiography

    National Research Council Canada - National Science Library

    Silvennoinen, H.M; Ikonen, S; Soinne, L; Railo, M; Valanne, L

    2007-01-01

    ...) in detection and grading of carotid artery bifurcation stenosis. METHODS: Consecutive patients with sonography evidence of a marked internal carotid artery stenosis underwent both carotid CTA and DSA...

  5. Neonate Aortic Stenosis: Importance of Myocardial Perfusion in Prognosis

    Directory of Open Access Journals (Sweden)

    Marco Aurélio Santos

    2002-09-01

    Full Text Available OBJECTIVE: To analyze our experience with percutaneous aortic balloon valvuloplasty in newborn infants with aortic stenosis, emphasizing the extraordinary importance of myocardial perfusion.METHODS: Over a 10-year-period, 21 neonates underwent percutaneous aortic balloon valvuloplasty. Age ranged from 2 to 27 days, weight ranged from 2.2 to 4.1 kg and 19 were males. All patients presented with congestive heart failure that could not be treated clinically. The onset of symptoms in the first week of life occurred in 9 patients considered as having critical aortic stenosis. Severe aortic stenosis occurred in 12 patients with the onset of symptoms in the second week of life.RESULTS: Mortality reached 100% in the patients with critical aortic stenosis. The procedure was considered effective in the 12 patients with severe aortic stenosis. Vascular complications included the loss of pulse in 12 patients and rupture of the femoral artery in 2 patients. Cardiac complications included acute aortic regurgitation in 2 patients and myocardial perforation in one. In an 8.2±1.3-year follow-up, 5 of the 12 patients died (2 patients due to septicemia and 3 patients due to congestive heart failure. Five of the other 7 patients underwent a new procedure and 2 required surgery.CONCLUSION: Percutaneous aortic valvuloplasty in neonates is not an effective procedure in the 1st week of life, because at this age the common presentation is cardiogenic shock. It is possible that, in those patients with critical aortic stenosis, dilation of the aortic valve during fetal life may change the prognosis of its clinical outcome.

  6. Asymmetric septal hypertrophy - a marker of hypertension in aortic stenosis (a SEAS substudy)

    DEFF Research Database (Denmark)

    Tuseth, Nora; Cramariuc, Dana; Rieck, Ashild E

    2010-01-01

    Some patients with aortic stenosis develop asymmetric septal hypertrophy (ASH) that may influence the surgical approach and is associated with higher perioperative morbidity. The aim of this analysis was to characterize further this subtype of aortic stenosis patients....

  7. MR findings of spondylolisthesis: assessment of associated spinal and neural foraminal stenosis

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jae Seung; Kang, Heung Sik; Yoon, Hye Kyung; Kim, Chu Wan [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1994-02-15

    To assess the spinal canal and neural foraminal stenosis associated with spondylolisthesis on MR imaging. We retrospectively analysed MR findings of 63 cases of spondylolisthesis(degenerative type: 23 cases, isthmic type: 40 cases) regarding the type and grade of spondylolisthesis, presence or absence of associated spinal canal stenosis, and the severity of associated neural foraminal stenosis. Central canal stenosis were more frequent in degenerative type(91%) than isthmic type(33%), and more frequent in grade II spondylolisthesis of degenerative type(100%) and isthmic type(89%) than in grade I spondylolisthesis of degenerative type(45%) and isthmic type(20%). There was positive correlation between the severity of neural foraminal stenosis and the grade of spondylolisthesis, whereas there was no significant difference between degenerative and isthmic types. Degenerative spondylolisthesis were frequently associated with central canal stenosis more than isthmic type. When the grade of spondylolisthesis was higher, it was more frequently associated with central canal stenosis and severe neural foraminal stenosis.

  8. A case of William's syndrome associated peripheral pulmonary arterial stenosis

    Energy Technology Data Exchange (ETDEWEB)

    Jung, Kyung Hwa; Hwang, Mi Soo; Kim, Sun Yong; Chang, Jae Chun; Park, Bok Hwan [College of Medicine, Yeungam University, Daegu (Korea, Republic of)

    1988-06-15

    William's syndrome, in order to more completely delineate the total spectrum of the disorder, indicates that 'infantile hypercalcemia', 'peculiar facies' and 'supravalvular aortic stenosis.' In has other many vascular anomalies, such as peripheral pulmonary arterial stenosis, coronary arterial stenosis, celiac arterial stenosis, and renal aterial stenosis. Only 32% of the patients have evidence of supravalvular aortic stenosis. And it is very rare disease entity that has been reported rarely in Korea. Recently authors experienced a case that was questioned William's syndrome with peripheral pulmonary arterial stenosis, clinically and preliminary radiologically and this case was confirmed by operation. Here we report a case of William's syndrome with peripheral pulmonary arterial stenosis and reviewed literatures.

  9. Tracheal diaphragm: a very unusual form of congenital tracheal stenosis.

    Science.gov (United States)

    Le Bret, Emmanuel; Teissier, Natacha; Menif, Khaled; Bruniaux, Jacqueline; Gharbi, Nourredine; Ben Jaballah, Nejla; Serraf, Alain; Van den Abbeele, Thierry

    2007-01-01

    A 3.2-kg newborn was intubated for neonatal respiratory distress owing to a congenital tracheal stenosis. The preoperative assessment showed a sphincter-like stenosis located 1 cm above the carina. The child was cured by resection-anastomosis through sternotomy under cardiopulmonary bypass. The pathological examination showed hypoplastic cartilage islets embedded in a fibroelastic conjunctive tissue. The posterior membranous region was preserved with the existence of smooth muscle cells. This curious malformation resembled a diaphragm at the junction between trachea and carina.

  10. Indexing aortic valve area by body surface area increases the prevalence of severe aortic stenosis

    DEFF Research Database (Denmark)

    Jander, Nikolaus; Gohlke-Bärwolf, Christa; Bahlmann, Edda

    2014-01-01

    To account for differences in body size in patients with aortic stenosis, aortic valve area (AVA) is divided by body surface area (BSA) to calculate indexed AVA (AVAindex). Cut-off values for severe stenosis are......To account for differences in body size in patients with aortic stenosis, aortic valve area (AVA) is divided by body surface area (BSA) to calculate indexed AVA (AVAindex). Cut-off values for severe stenosis are...

  11. Asymmetric septal hypertrophy - a marker of hypertension in aortic stenosis (a SEAS substudy)

    DEFF Research Database (Denmark)

    Tuseth, Nora; Cramariuc, Dana; Rieck, Ashild E

    2010-01-01

    Some patients with aortic stenosis develop asymmetric septal hypertrophy (ASH) that may influence the surgical approach and is associated with higher perioperative morbidity. The aim of this analysis was to characterize further this subtype of aortic stenosis patients.......Some patients with aortic stenosis develop asymmetric septal hypertrophy (ASH) that may influence the surgical approach and is associated with higher perioperative morbidity. The aim of this analysis was to characterize further this subtype of aortic stenosis patients....

  12. Optimized pacing mode for hypertrophic cardiomyopathy: Impact of ECG fusion during pacing

    NARCIS (Netherlands)

    Berruezo, A.; Penela, D.; Burgos, F.; Evertz, R.; Fernandez-Armenta, J.; Roca, J.; Doltra, A.; Acosta, J.; Francino, A.; Sitges, M.; Alsina, X.; Ordonez, A.; Villuendas, R.; Brugada, R.; Mont, L.; Brugada, J.

    2015-01-01

    BACKGROUND: Electrocardiographic (ECG) fusion with intrinsic QRS could reduce the benefit of atrial synchronous biventricular pacing (AS-BiVP) in patients with hypertrophic obstructive cardiomyopathy (HOCM). OBJECTIVES: The purpose of this study was to assess the benefit of AS-BiVP and the influence

  13. Time course of the angiogenic response during normotrophic and hypertrophic scar formation in humans

    NARCIS (Netherlands)

    van der Veer, Willem M.; Niessen, Frank B.; Ferreira, Jose A.; Zwiers, Peter J.; de Jong, Etty H.; Middelkoop, Esther; Molema, Grietje

    2011-01-01

    Previous research suggests that in hypertrophic scars (HSs), an excess of microvessels is present compared with normotrophic scars (NSs). The aim of our study was to quantify vascular densities in HSs and normotrophic scars and to provide an insight into the kinetics of changes in the expression of

  14. Transforming growth factor alpha controls the transition from hypertrophic cartilage to bone during endochondral bone growth.

    Science.gov (United States)

    Usmani, Shirine E; Pest, Michael A; Kim, Gunwoo; Ohora, Sara N; Qin, Ling; Beier, Frank

    2012-07-01

    We have recently identified transforming growth factor alpha (TGFα) as a novel growth factor involved in the joint disease osteoarthritis. The role of TGFα in normal cartilage and bone physiology however, has not been well defined. The objective of this study was to determine the role of TGFα in bone development through investigation of the Tgfa knockout mouse. The gross skeletons as well as the cartilage growth plates of Tgfa knockout mice and their control littermates were examined during several developmental stages ranging from newborn to ten weeks old. Knockout mice experienced skeletal growth retardation and expansion of the hypertrophic zone of the growth plate. These phenotypes were transient and spontaneously resolved by ten weeks of age. Tgfa knockout growth plates also had fewer osteoclasts along the cartilage/bone interface. Furthermore, knockout mice expressed less RUNX2, RANKL, and MMP13 mRNA in their cartilage growth plates than controls did. Tgfa knockout mice experience a delay in bone development, specifically the conversion of hypertrophic cartilage to true bone. The persistence of the hypertrophic zone of the growth plate appears to be mediated by a decrease in MMP13 and RANKL expression in hypertrophic chondrocytes and a resulting reduction in osteoclast recruitment. Overall, TGFα appears to be an important growth factor regulating the conversion of cartilage to bone during the process of endochondral ossification. Copyright © 2012 Elsevier Inc. All rights reserved.

  15. 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...

  16. 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...

  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. 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 hypert

  19. 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.)

  20. Effects of permanent dual chamber pacing on myocardial perfusion in symptomatic hypertrophic cardiomyopathy

    NARCIS (Netherlands)

    Posma, JL; Blanksma, PK; VanderWall, EE; Vaalburg, W; Crijns, HJGM; Lie, KI

    1996-01-01

    Objective-Angina and the presence of myocardial ischaemia are common in hypertrophic cardiomyopathy. Dual chamber pacing results in clinical improvement in these patients. This study evaluates the effects of permanent dual chamber pacing on absolute regional myocardial perfusion and perfusion

  1. Scar management practice and science: a comprehensive approach to controlling scar tissue and avoiding hypertrophic scarring.

    Science.gov (United States)

    Widgerow, Alan David; Chait, Laurence A

    2011-12-01

    A meta-analysis of the literature forms the basis of a treatment regimen focused on the principles of support, controlled inflammation, hydration, and hastened collagen remodeling. The presented clinical trial avoided hypertrophic scarring in more than 80% of cases, validating the theory of targeting many areas of scar control in 1 approach.

  2. 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 langua

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

    NARCIS (Netherlands)

    I. Christiaans; K. van Engelen; I.M. van Langen; E. Birnie; G.J. Bonsel; P.M. Elliott; A.A.M. Wilde

    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 langua

  4. 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.

  5. 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.

  6. 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

  7. 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.

  8. 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.

  9. Marked left ventricular hypertrophy mimicking hypertrophic cardiomyopathy associated with steroid therapy for congenital adrenal hyperplasia.

    Science.gov (United States)

    Scirè, Giuseppe; D'Anella, Giorgio; Cristofori, Laura; Mazzuca, Valentina; Cianfarani, Stefano

    2007-06-01

    We describe a child treated with high-dose steroid therapy for congenital adrenal hyperplasia who showed marked left ventricular hypertrophy mimicking hypertrophic cardiomyopathy associated with steroid therapy for congenital adrenal hyperplasia. Cardiomyopathy reversed completely when an appropriate steroid therapeutic regimen was established.

  10. Multivariate-Analysis of Phytoplankton and Related Environmental-Factors, in a Shallow Hypertrophic Lake

    NARCIS (Netherlands)

    Romo, S.; Van Tongeren, O.F.R.

    1995-01-01

    Data on some relevant environmental variables and phytoplankton species composition, collected from the hypertrophic shallow lake Albufera of Valencia (Spain) during 1980-88, were examined using Redundancy Analysis (RDA). The hydrological cycle of the lake is manipulated for rice cultivation in the

  11. A novel elastic liposome for skin delivery of papain and its application on hypertrophic scar.

    Science.gov (United States)

    Chen, Yan-Yan; Lu, Ye-Hui; Ma, Chun-Hua; Tao, Wei-Wei; Zhu, Jing-Juan; Zhang, Xu

    2017-03-01

    This study aims to investigate the therapeutic effects of papain elastic liposomes (PEL) on hypertrophic scar through topical application. PEL were prepared using the reverse-phase evaporation method and optimized by response surface methodology. The transdermal absorption of optimized PEL was tested by vertical Franz diffusion cells in vitro. The effects of PEL were investigated in rabbit model of hypertrophic scar in vivo, histological analysis and scar-related proteins were detected to reveal potential scar repair mechanism. The best formulation of PEL had EE (43.8±1.4%), particle size (100.9±2.2nm), PDI (0.037±0.003), zeta potential (-26.3±1.3mV), and DI (21.9±3.1). PEL gave the cumulative amounts and steady state fluxes in the receiver solution of 381.9±32.4μg/cm(2), 11.4±1.5μg/cm(2)/h, and showed drug deposition in skin of 19.1±3.2% after 24h. After topical application, the scar elevation index, microvascular density, and collagen fiber were significantly decreased with regular arrangement. The expressions of TGF-β1, P-Smad-3, P-NF-κB p65, and P-IKBa in hypertrophic scar were significantly down regulated in contrast with those in model group. PEL were proven as an excellent topical preparation for hypertrophic scar treatment.

  12. Multivariate-Analysis of Phytoplankton and Related Environmental-Factors, in a Shallow Hypertrophic Lake

    NARCIS (Netherlands)

    Romo, S.; Van Tongeren, O.F.R.

    1995-01-01

    Data on some relevant environmental variables and phytoplankton species composition, collected from the hypertrophic shallow lake Albufera of Valencia (Spain) during 1980-88, were examined using Redundancy Analysis (RDA). The hydrological cycle of the lake is manipulated for rice cultivation in the

  13. Hair-thread tourniquet syndrome of the hypertrophic clitoris in a 6-year-old girl.

    Science.gov (United States)

    Parlak, Mesut; Karakaya, Ali Erdal

    2015-05-01

    Hair-thread tourniquet syndrome is defined as the ischemic strangulation of hair resulting in edema and severe pain and may cause amputation of organs. Strangulation of the external genitalia (clitoris, labia minora) has rarely been described in girls. Here, we present a case of hypertrophic clitoris injury secondary to hair strangulation in a 6-year-old girl.

  14. 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 d

  15. 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 c

  16. 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

  17. 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

  18. [Right ventricular involvement in hypertrophic cardiomyopathy. A case report and brief review of the literature].

    Science.gov (United States)

    Comella, Alessandro; Magnacca, Massimo; Gistri, Roberto; Lombardi, Massimo; Neglia, Danilo; Poddighe, Rosa; Pesola, Antonio

    2004-02-01

    A clinical case of non-obstructive hypertrophic cardiomyopathy with involvement of the right ventricle is reported. The patient was a 42-year-old male with symptoms suggesting an effort angina of recent onset. The diagnosis was established by echocardiography, which showed asymmetric hypertrophy of the interventricular septum (20 mm), hypertrophy of the right ventricular free wall, and severe hypertrophy of the septal papillary muscle of the tricuspid valve. The patient underwent a complete diagnostic evaluation, including exercise stress test, Holter monitoring, magnetic resonance, myocardial tomoscintigraphy and complete hemodynamic assessment. Medical treatment with atenolol 50 mg day was started; at 1-year follow-up the patient's clinical conditions are good, with decrease of anginal episodes. The literature review elicits the paucity of information about this condition, despite a frequent involvement of both ventricles in hypertrophic obstructive cardiomyopathy. The case reported shows two atypical aspects: a) the involvement of the right ventricle in non-obstructive hypertrophic cardiomyopathy is anecdotal; b) this pattern of hypertrophy (right ventricular free wall/septal papillary muscle) has never been previously reported. Right ventricular involvement in patients with hypertrophic cardiomyopathy must be carefully investigated, because it may be more frequent than conventionally deemed.

  19. Effects of permanent dual chamber pacing on myocardial perfusion in symptomatic hypertrophic cardiomyopathy

    NARCIS (Netherlands)

    Posma, JL; Blanksma, PK; VanderWall, EE; Vaalburg, W; Crijns, HJGM; Lie, KI

    1996-01-01

    Objective-Angina and the presence of myocardial ischaemia are common in hypertrophic cardiomyopathy. Dual chamber pacing results in clinical improvement in these patients. This study evaluates the effects of permanent dual chamber pacing on absolute regional myocardial perfusion and perfusion reserv

  20. Transcoronary ablation of septal hypertrophy compared with surgery in the treatment of hypertrophic obstructive cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    姜腾勇; 吴学思; 吕强; 孟旭; 贾长琪; 张银

    2004-01-01

    @@ Transcoronary ablation of septal hypertrophy (TASH)can lead to sustained improvement in both hemodynamics and symptoms in patients with hypertrophic obstructive cardiomyopathy ( HOCM ) . 1-4 However, there have been few reports about its efficacy and safety compared with traditional surgical procedures. This study sought to compare TASH with surgery in the treatment of HOCM.

  1. 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 hypert

  2. Basal-cell carcinoma resulting from radiation therapy for hypertrophic tonsils

    Energy Technology Data Exchange (ETDEWEB)

    Hanke, C.W.; O' Brian, J.J.; Shidnia, H.; Becker, J.M.

    1985-02-01

    Benign dermatologic conditions are no longer treated with therapeutic radiation because of the late complications that can result. One of the most serious complications that may occur is the development of skin cancer of various types. In this report, a patient received radiation for hypertrophic tonsils and later developed basal-cell carcinomas in the irradiated areas.

  3. Iloprost improves ventricular function in the hypertrophic and functionally impaired right heart by direct stimulation

    DEFF Research Database (Denmark)

    Holmboe, Sarah; Andersen, Asger; Vildbrad, Mads D;

    2013-01-01

    and dysfunctional right ventricle (RV). Rats were randomized to monocrotaline injection (60 mg/kg; [Formula: see text]), pulmonary trunk banding (PTB; [Formula: see text]), or a sham operation ([Formula: see text]). RV function was evaluated with magnetic resonance imaging, echocardiography, and invasive pressure...... that iloprost has inotropic properties directly improving ventricular function in the hypertrophic and dysfunctional right heart of the rat....

  4. 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 u

  5. 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.

  6. Effect of BTXA on Inhibiting Hypertrophic Scar Formation in a Rabbit Ear Model.

    Science.gov (United States)

    Liu, Dong-Qing; Li, Xiao-Jing; Weng, Xiao-Juan

    2017-06-01

    Hypertrophic scar (HS) is a refractory skin disease caused by major physical damage or other inflammation. Some reports found that botulinum toxin type A (BTXA) could be an alternative treatment of the HS. Therefore, the authors studied the effects of BTXA on the treatment of HS and the dose response of BTXA. Hypertrophic scars were harvested from the ears of 18 young adult New Zealand big-eared rabbits and treated with BTXA or triamcinolone acetonide (TAC) in vivo experiment. The hypertrophic index (HI) was measured by histological examination. Collagen fibrils were checked by sirius red straining, and the cell nucleuses of fibroblasts were checked by Ki67. The HI of hypertrophic scars with BTXA treatment was lower than that with phosphate-buffered saline treatment (P Book Reviews, and manuscripts that concern Basic Science, Animal Studies, Cadaver Studies, and Experimental Studies. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

  7. 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.

  8. 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)

  9. Dietary copper supplementation reverses hypertrophic cardiomyopathy induced by chronic pressure overload in mice

    Science.gov (United States)

    Sustained pressure overload causes cardiac hypertrophy and the transition to heart failure. We show here that dietary supplementation with physiologically relevant levels of copper (Cu) reverses pre-established hypertrophic cardiomyopathy in the presence of pressure overload induced by ascending aor...

  10. 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

  11. 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.

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

    Science.gov (United States)

    Kwan, Peter; Ding, Jie; Tredget, Edward E

    2015-01-01

    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.

  13. Cephalic arch stenosis in autogenous brachiocephalic hemodialysis fistulas: results of cutting balloon angioplasty

    DEFF Research Database (Denmark)

    Heerwagen, Søren Thorup; Lönn, Lars; Schroeder, Torben V;

    2010-01-01

    Cephalic arch stenosis is a known cause of hemodialysis access failure in patients with brachiocephalic fistulas (BCFs). Outcomes of endovascular treatment are affected by resistance of the stenosis to balloon dilation, a high vein rupture rate and the development of early restenosis. The purpose...... of this retrospective study was to report outcomes after cutting balloon angioplasty (CBA) of cephalic arch stenosis....

  14. Radiation induced renal arterial stenosis detected by color duplex ultrasonography: case report

    Institute of Scientific and Technical Information of China (English)

    Jing Gao; Byong K Park; Arnold Alday

    2005-01-01

    Renal artery stenosis as a complication from radiation therapy is not common, but it is life threatening and needs to be corrected urgently in order to prevent renal failure even losing kidney. The diagnostic criteria of renal artery stenosis in the adults by color duplex ultrasonography have been established, which may play an important role in screening radiation induced renal artery stenosis.

  15. A case of valvular pulmonic stenosis and an aberrant coronary artery in a Brittany spaniel.

    Science.gov (United States)

    Estey, Chelsie

    2011-05-01

    Valvular pulmonic stenosis and aberrancy of the right coronary artery with subsequent subvalvular stenosis was found on echocardiographic evaluation of a 9-month-old Brittany spaniel. Previous echocardiography at 4 mo of age revealed the pulmonic stenosis; however, the aberrant coronary artery only became apparent during the second evaluation.

  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. Left atrial volume in patients with asymptomatic aortic valve stenosis (the Simvastatin and Ezetimibe in Aortic Stenosis study)

    DEFF Research Database (Denmark)

    Dalsgaard, Morten; Egstrup, K.; Wachtell, K.

    2008-01-01

    Left atrial (LA) size is known to increase with persistently increased left ventricular (LV) filling pressure. We therefore hypothesized that LA volume might reflect the severity of aortic valve stenosis (AS). Transthoracic echocardiography was performed in 1,758 patients with asymptomatic...... AS (transaortic Doppler velocity > or =2.5 and Aortic Stenosis (SEAS) study. LA volume was measured in end-systole in the apical 4-chamber view in 1,503 patients (85%), and aortic valve area (AVA) was estimated by the continuity equation and indexed by body surface...

  18. Prognostic importance of atrial fibrillation in asymptomatic aortic stenosis: The Simvastatin and Ezetimibe in Aortic Stenosis study

    DEFF Research Database (Denmark)

    Greve, Anders M; Gerdts, Eva; Boman, Kurt

    2011-01-01

    BACKGROUND: The frequency and prognostic importance of atrial fibrillation (AF) in asymptomatic mild-to-moderate aortic stenosis (AS) has not been well described. METHODS: Clinical examination, electrocardiography and echocardiography were obtained in asymptomatic patients with mild......-to-moderate AS and preserved left ventricular (LV) systolic function, randomized to simvastatin/ezetimibe combination vs. placebo in the Simvastatin and Ezetimibe in Aortic Stenosis (SEAS) study. At inclusion, AF was categorized as episodic or longstanding. Rhythm change was assessed on annual in-study electrocardiograms...

  19. Quantification of carbonic anhydrase gene expression in ventricle of hypertrophic and failing human heart

    Directory of Open Access Journals (Sweden)

    Alvarez Bernardo V

    2013-01-01

    Full Text Available Abstract Background Carbonic anhydrase enzymes (CA catalyze the reversible hydration of carbon dioxide to bicarbonate in mammalian cells. Trans-membrane transport of CA-produced bicarbonate contributes significantly to cellular pH regulation. A body of evidence implicates pH-regulatory processes in the hypertrophic growth pathway characteristic of hearts as they fail. In particular, Na+/H+ exchange (NHE activation is pro-hypertrophic and CA activity activates NHE. Recently Cardrase (6-ethoxyzolamide, a CA inhibitor, was found to prevent and revert agonist-stimulated cardiac hypertrophy (CH in cultured cardiomyocytes. Our goal thus was to determine whether hypertrophied human hearts have altered expression of CA isoforms. Methods We measured CA expression in hypertrophied human hearts to begin to examine the role of carbonic anhydrase in progression of human heart failure. Ventricular biopsies were obtained from patients undergoing cardiac surgery (CS, n = 14, or heart transplantation (HT, n = 13. CS patients presented mild/moderate concentric left ventricular hypertrophy and normal right ventricles, with preserved ventricular function; ejection fractions were ~60%. Conversely, HT patients with failing hearts presented CH or ventricular dilation accompanied by ventricular dysfunction and EF values of 20%. Non-hypertrophic, non-dilated ventricular samples served as controls. Results Expression of atrial and brain natriuretic peptide (ANP and BNP were markers of CH. Hypertrophic ventricles presented increased expression of CAII, CAIV, ANP, and BNP, mRNA levels, which increased in failing hearts, measured by quantitative real-time PCR. CAII, CAIV, and ANP protein expression also increased approximately two-fold in hypertrophic/dilated ventricles. Conclusions These results, combined with in vitro data that CA inhibition prevents and reverts CH, suggest that increased carbonic anhydrase expression is a prognostic molecular marker of cardiac

  20. 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

  1. Anti-inflammatory cytokine TSG-6 inhibits hypertrophic scar formation in a rabbit ear model.

    Science.gov (United States)

    Wang, Hui; Chen, Zhao; Li, Xiao-Jing; Ma, Li; Tang, Yue-Ling

    2015-03-15

    Hypertrophic scars are characterized by excessive fibrosis and extracellular matrix (ECM) deposition and can be functionally and cosmetically problematic; however, there are few satisfactory treatments for controlling hypertrophic scars. The inflammatory cells and cytokines involved in excessive inflammation during wound healing facilitate fibroblast proliferation and collagen deposition, leading to pathologic scar formation. TSG-6 exhibits anti-inflammatory activity. This study examined the effect of recombinant TSG-6 on inflammation in hypertrophic scars using a rabbit ear model. Six 7-mm, full-thickness, circular wounds were made on the ears of 12 rabbits. TSG-6 and PBS were intradermally injected into the right and left ear wounds, respectively. The methods of TEM and TUNEL were used to detect fibroblast apoptosis. The expressions of inflammatory factors: IL-1β, IL-6 and TNF-α, were detected by immunohistochemistry and real time polymerase chain reaction. Collagen I and III expression detected by immunohistochemistry and Masson׳s trichrome staining and SEI (scar elevation index) was used to evaluate the extent of scarring. TSG-6 injection mitigated the formation of a hypertrophic scar in the rabbit ear. TSG-6-treated wounds exhibited decreased inflammation compared with the control group, as evidenced by the lower levels of IL-1β, IL-6, TNF-α and MPO. The SEI and the synthesis of collagens I and III were significantly decreased in the TSG-6-treated scars compared with control scars. The apoptosis rate was higher in the TSG-6-treated scars. TSG-6 exhibited anti-inflammatory effects during the wound healing process and cicatrization and significantly diminished hypertrophic scar formation in a rabbit ear model. Copyright © 2015 Elsevier B.V. All rights reserved.

  2. Assessment of intralesional injection of botulinum toxin type A injection for hypertrophic scars

    Directory of Open Access Journals (Sweden)

    Alhasan M Elhefnawy

    2016-01-01

    Full Text Available Background: Hypertrophic scars are dermal fibroproliferative disorders that typically develop after a skin injury heals. They can cause physical, psychological, and cosmetic problems. The management of such scars remains a matter of debate due to lack of effective treatment methods and the inability to prevent recurrences. Recent reports have demonstrated that botulinum toxin type A improves wound healing so it may play a role in treating hypertrophic scars. Aims: We assessed the effectiveness of intralesional botulinum toxin type A injection for treating hypertrophic scars. Methods: This prospective clinical study included twenty patients with hypertrophic scars. Intralesional injection of botulinum toxin type A was given once a month for three months with a follow-up period of six months. Each lesion was injected until slight blanching occurred. Therapeutic satisfaction of the patient and physician were recorded. Lesions were assessed for erythema, itching and pliability. Each item was assessed on a 5-point scale. Results: Therapeutic satisfaction was recorded as 'good' in 14 patients and 'excellent' in the remaining six. The mean erythema score decreased from 3.2 to 1.0, the mean pliability score from 3.3 to 0.8 and the mean itching score from 2.7 to 0.7. All of these were statistically significant. Limitations: A larger sample size and longer follow-up period would have given a better evaluation but was not feasible due to the high expenses involved. Conclusion: Botulinum toxin type A is a novel and promising therapy for hypertrophic scars with few side effects.

  3. Duodenal Bulb Mucosa with Hypertrophic Gastric Oxyntic Heterotopia in Patients with Zollinger Ellison Syndrome

    Directory of Open Access Journals (Sweden)

    Emil Kohan

    2009-01-01

    Full Text Available Objectives. Zollinger-Ellison Syndrome (ZES results in hypersecretion of gastric acid (via gastrinoma leading to peptic ulcers, diarrhea, and abdominal pain. We describe the novel discovery of hypertrophic, heterotopic gastric mucosa in the proximal duodenal bulb in patients with ZES, which we hypothesize results in an increased incidence of postbulbar ulcers in patients with ZES (a mechanism previously unreported. We determined the incidence of the novel finding of duodenal gastric oxyntic hypertrophic heterotopia (GOH in patients with ZES. Methods. Seven patients with ZES were enrolled. The diagnosis of ZES was established by hypergastrinemia, gastric acid hypersecretion, and a positive secretin test or based on biopsy specimens (evaluated via tissue staining. Basal acid output (BAO and baseline gastrin secretion were determined by established methods. Endoscopic examinations with methylene blue staining and biopsy of the gastric and duodenal mucosa were conducted in all patients every 3–6 months for an average of 5 years. Results. The duodenal mucosa demonstrated hypertrophic GOH in 5 out of 7 patients with ZES and an intact stomach and duodenum. Biopsies from the bowel mucosa demonstrated patchy replacement of surface epithelium by gastric-type epithelium with hypertrophic oxyntic glands in the lamina propria in 5 patients. Two of the patients had no evidence of GOH in the duodenal bulb. Patients with GOH had an average serum gastrin level of 1245 pg/mL and BAO of 2.92 mEq/hr versus 724 pg/mL and 0.8 mEq/hr in patients without GOH. Conclusions. This study demonstrated the presence of duodenal mucosa with GOH in 5 out of 7 patients with ZES and an intact stomach and duodenum. The presence of hypertrophic and heterotopic gastric mucosa is proposed to result from increased gastrin levels and may contribute to the increased incidence of postbulbar ulcers in these patients.

  4. Changes the state of secretory granules of the superficial epitheliocytes of the pyloric mucous membrane in the stomach of rats under the action of epichlorohydrin

    Directory of Open Access Journals (Sweden)

    A. S. Smirnov

    2016-10-01

    Full Text Available The objective of the research was to examine the patterns of action of epichlorohydrin on the state of the secretory granules of the superficial epitheliocytes of the mucous membrane of pyloric part of stomach of rats. Material and methods of the research. Mature male albino rats were used in the experiment. Rats of Group I were Control animals. Rats of Group II were administered epichlorohydrin. Rats of Group III were given Echinacea purpurea extract. Rats of Group IV were administered thiotriazoline. Rats of Group V were exposed to epichlorohydrin and extract of Echinacea purpurea, and rats of Group VI were exposed to epichlorohydrin and thiotriazoline. Results of the research. The experiment showed that long-term inhalations of epichlorohydrin are accompanied by changes of secretory granules area, which remain after inhalations. The use of the extract of Echinacea purpurea on the background of epichlorohydrin prevents epichlorohydrin induced increase in the area of secretory granules on the thirtieth day of observation. Using thiotriazoline during inhalations of epichlorohydrin prevents the appearance of epichlorohydrin induced effects reducing the area of the secretory granules of the cytoplasm of epitheliocytes surface on the first day of observation and increasing their area on the thirtieth day of observation. Conclusions. Inhalation of epichlorohydrin is accompanied by changes of the area of secretory granules in the cytoplasm of superficial epitheliocytes. Use of the extract of Echinacea purpurea and thiotriazoline on the background of epichlorohydrin prevents the epichlorohydrin induced changes in the area of the secretory granules of the cytoplasm of superficial epitheliocytes.

  5. Cone pathway function in relation to asymmetric carotid artery stenosis

    DEFF Research Database (Denmark)

    Kofoed, Peter Kristian; Munch, Inger Christine; Holfort, Stig K;

    2013-01-01

    in the same patient. Results:  Ophthalmic systolic pressure was 95.8 ± 13.1 mmHg on the side with the highest degree of carotid artery stenosis (mean 94.0%) and 111.7 ± 10.3 mmHg in the fellow eyes on the side with the lesser degree of stenosis (mean 33.9%). Summed mfERG implicit times (N1 and P1) were 3......Purpose:  To examine retinal function in relation to retinal perfusion pressure in patients with carotid artery stenosis. Methods:  Thirteen patients with carotid artery stenosis without clinical eye disease underwent assessment of ophthalmic artery systolic blood pressure (OSP) by ocular...... pneumoplethysmography, carotid artery obstructive disease by ultrasonography, intraocular pressure by applanation tonometry, retinal perfusion by fluorescein angiography and retinal function by multifocal electroretinography (mfERG). Data analysis compared the eye on the most stenotic side with the fellow eye...

  6. Carotid plaque, intima-media thickness, and incident aortic stenosis

    DEFF Research Database (Denmark)

    Martinsson, Andreas; Östling, Gerd; Persson, Margaretha

    2014-01-01

    OBJECTIVE: Aortic stenosis (AS) shares risk factors with atherosclerotic vascular disease. Carotid intima-media thickness (IMT) and plaque may reflect the cumulative damage from exposure to different atherosclerotic risk factors. We examined the relationship of carotid IMT and plaque with incident...

  7. Aortic Stenosis in Adults: natural history, treatment and outcome

    NARCIS (Netherlands)

    H.J. Heuvelman (Helena )

    2015-01-01

    markdownabstract__Abstract__ This thesis concerns aortic stenosis (AS) in contemporary clinical practice. First, an introduction will be given to provide background information on the normal aortic valve, and thereafter on the incidence, disease spectrum, diagnosis, treatment, and prognosis of AS d

  8. Congenital nasal piriform aperture stenosis with vestibular abnormality

    Energy Technology Data Exchange (ETDEWEB)

    Rajaram, Smitha; Raghavan, Ashok [Sheffield Children' s Hospital, Department of Paediatric Radiology, Sheffield (United Kingdom); Bateman, Neil [Sheffield Children' s Hospital, ENT Department, Sheffield (United Kingdom)

    2008-10-15

    We present a neonate with congenital nasal piriform aperture stenosis associated with an abnormal vestibular aperture. Radiological evaluation with CT is essential to confirm the diagnosis and delineate the anatomy for surgical planning. Extension of the scan field of view to include the petrous temporal bone is essential to identify associated abnormalities of the vestibule. (orig.)

  9. Transesophageal echocardiography assessment of severe ostial left main coronary stenosis

    Science.gov (United States)

    Firstenberg, M. S.; Greenberg, N. L.; Lin, S. S.; Garcia, M. J.; Alexander, L. A.; Thomas, J. D.

    2000-01-01

    Doppler echocardiography is commonly used in the assessment of stenotic valvular orifices. We describe the application of transesophageal echocardiography for the detection of a critical ostial left main coronary stenosis. Because preoperative coronary angiography often is not routinely performed in young patients undergoing valve surgery, application of Doppler echocardiography can potentially prevent catastrophic complications, particularly in atypical cases.

  10. Coronary flow reserve : a functional measure of stenosis severity

    NARCIS (Netherlands)

    F. Zijlstra (Felix)

    1988-01-01

    textabstractIn 1959 Sones developed selective coronary cineangiography. To date, this technique has remained the only means available for the visualization of the coronary arterial system with such image contrast and resolution, that the presence and severity of coronary stenosis can be

  11. Modified technique of BMV for severe submitral stenosis.

    Science.gov (United States)

    Nanjappa, Manjunath C; Bhat, Prabhavathi; Panneerselvam, Arunkumar

    2011-09-01

    We present a case where difficulty was encountered during balloon mitral valvotomy (BMV) because of severe submitral stenosis. As the orifice was 0.4 cm² at submitral level the BMV balloon catheter could not enter the left ventricle. We used a modified technique of liberating the submitral apparatus that facilitated successful BMV.

  12. Evaluation of semi-automatic arterial stenosis quantification

    Energy Technology Data Exchange (ETDEWEB)

    Hernandez Hoyos, M. [CREATIS Research Unit, CNRS, INSERM, INSA, Lyon (France); Universite Claude Bernard Lyon 1, 69 - Villeurbanne (France). INSA; Univ. de los Andes, Bogota (Colombia). Grupo de Ingenieria Biomedica; Serfaty, J.M.; Douek, P.C. [CREATIS Research Unit, CNRS, INSERM, INSA, Lyon (France); Universite Claude Bernard Lyon 1, 69 - Villeurbanne (France). INSA; Hopital Cardiovasculaire et Pneumologique L. Pradel, Bron (France). Dept. de Radiologie; Maghiar, A. [Hopital Cardiovasculaire et Pneumologique L. Pradel, Bron (France). Dept. de Radiologie; Mansard, C.; Orkisz, M.; Magnin, I. [CREATIS Research Unit, CNRS, INSERM, INSA, Lyon (France); Universite Claude Bernard Lyon 1, 69 - Villeurbanne (France). INSA

    2006-11-15

    Object: To assess the accuracy and reproducibility of semi-automatic vessel axis extraction and stenosis quantification in 3D contrast-enhanced Magnetic Resonance Angiography (CE-MRA) of the carotid arteries (CA). Materials and methods: A total of 25 MRA datasets was used: 5 phantoms with known stenoses, and 20 patients (40 CAs) drawn from a multicenter trial database. Maracas software extracted vessel centerlines and quantified the stenoses, based on boundary detection in planes perpendicular to the centerline. Centerline accuracy was visually scored. Semi-automatic measurements were compared with: (1) theoretical phantom morphometric values, and (2) stenosis degrees evaluated by two independent radiologists. Results: Exploitable centerlines were obtained in 97% of CA and in all phantoms. In phantoms, the software achieved a better agreement with theoretic stenosis degrees (weighted kappa {kappa}{sub W} = 0.91) than the radiologists ({kappa}{sub W} = 0.69). In patients, agreement between software and radiologists varied from {kappa}{sub W} =0.67 to 0.90. In both, Maracas was substantially more reproducible than the readers. Mean operating time was within 1 min/ CA. Conclusion: Maracas software generates accurate 3D centerlines of vascular segments with minimum user intervention. Semi-automatic quantification of CA stenosis is also accurate, except in very severe stenoses that cannot be segmented. It substantially reduces the inter-observer variability. (orig.)

  13. Coexistence of osteopoikilosis with seronegative spondyloarthritis and spinal stenosis

    Science.gov (United States)

    Demir, Saliha Eroglu; Özaras, Nihal; Poyraz, Emine; Toprak, Hüseyin; Güler, Mustafa

    2015-01-01

    [Purpose] Osteopoikilosis is a rare hereditary bone disease that is usually asymptomatic. It is generally diagnosed incidentally on plain radiography. The coexistence of osteopoikilosis with seronegative spondyloarthritis or spinal stenosis is rarely reported. Here, we report the case of a 27-year-old male patient with osteopoikilosis, seronegative spondyloarthritis, and spinal stenosis. [Subject] A 27-year-old male patient with buttock pain and back pain radiating to the legs. [Methods] A plain anteroposterior radiograph of the pelvis revealed numerous round and oval sclerotic bone areas of varying size. Investigation of the knee joints showed similar findings, and the patient was diagnosed with osteopoikilosis. Lumbar magnetic resonance images showed spinal stenosis and degenerative changes in his lumbar facet joints. Magnetic resonance images of the sacroiliac joints showed bilateral involvement with narrowing of both sacroiliac joints, nodular multiple sclerotic foci, and contrast enhancement in both joint spaces and periarticular areas. HLA B-27 test was negative. [Results] The patient was diagnosed with osteopoikilosis, seronegative spondyloarthritis, and spinal stenosis. Treatment included asemetasin twice daily and exercise therapy. [Conclusion] Symptomatic patients with osteopoikilosis should be investigated for other possible coexisting medical conditions; this will shorten the times to diagnosis and treatment. PMID:26157277

  14. Transnasal endoscopic repair of acquired posterior choanal stenosis and atresia

    Institute of Scientific and Technical Information of China (English)

    WANG Qin-ying; WANG Shen-qing; LIN Shan; CHEN Hai-hong; LU Yu-yu

    2008-01-01

    Background There are conqenital and acquired choanal atresias and many approaches have been used for their repair.We assessed the clinical effect of power instrument.endoscopic repair of acquired choanaI stenosis and atresia.Methods Nineteen patients,aged from 32 to 61 years,with acquired choanal stenosis and atresia (from trauma in 5 cases and from radiotherapy after nasopharyngeal carcinoma in 14:6 bilateral and 13 unilateral cases),underwent transnasal endoscopic repair of choanal stenosis and atresia.No patient had stenting.Antibiotic and local glucocorticoid were administered postoperatively.Results Eiqhteen patients remained free of symptoms for 12-40 months after the surgery,and the diameter of the neochoana was more than 1 cm after the procedure.One patient required revision surgery and recovered completely with no restenosis at 12 months after the second surgery.There were no postoperative complications.Histology of the resected tissue revealed respiratory epithelial-lined stromal tissue with chronic inflammation,edema and fibrosis,but no tumor cells.Conclusions Transnasal endoscopic approach is a useful procedure for the repair of acquired choanal stenosis and atresia:it is highly successful,safe and effective with swift recovery and short time of hospitalizalion.It is very important in postoperative care to remove any granulation or polyps at the site of the neochoana at that time.

  15. PERFORMING TRANSCATHETER AORTIC VALVE IMPLANTATION IN PATIENTS WITH CAROTID STENOSIS

    Directory of Open Access Journals (Sweden)

    Veselin Valkov

    2016-07-01

    Full Text Available The management of carotid artery disease in patients with severe aortic stenosis referred for transcatheter aortic valve implantation is challenging. By reviewing the very limited amount of literature we will try to answer the question should we perform carotid revascularization before or after the TAVI procedure.

  16. [Treatment of pulmonary vein stenosis secondary to radiofrequency ablation].

    Science.gov (United States)

    Ferrero Guadagnoli, Adolfo; Contreras, Alejandro E; Leonardi, Carlos R; Ballarino, Miguel A; Atea, Leonardo; Peirone, Alejandro R

    2014-01-01

    Isolation of the pulmonary veins by applying radiofrequency is an effective treatment for atrial fibrillation. One of the potential complications with higher clinical compromise utilizing this invasive technique is the occurrence of stenosis of one or more pulmonary veins. This complication can be treated by angioplasty with or without stent implantation, with an adequate clinical improvement, but with a high rate of restenosis.

  17. Clinical analysis of 132 patients with atherosclerotic renal artery stenosis

    Institute of Scientific and Technical Information of China (English)

    徐红

    2006-01-01

    Objective To evaluate the prognostic result of renal function on atherosclerotic renal artery stenosis (ARAS) patients after revascularization and medication therapy. Methods The clinical data of 132 AEIAS patients diagnosed by renal angiography were analysed. For comparing the differences of glomenilar filtration rate (GFR) be-

  18. Renal vein oxygen saturation in renal artery stenosis

    DEFF Research Database (Denmark)

    Nielsen, K; Rehling, M; Henriksen, Jens Henrik Sahl

    1992-01-01

    Renal vein oxygen-saturation was measured in 56 patients with arterial hypertension and unilateral stenosis or occlusion of the renal artery. Oxygen-saturation in blood from the ischaemic kidney (84.4%, range 73-93%) was significantly higher than that from the 'normal' contralateral kidney (81...

  19. Subglottic stenosis and cricoid growth : an experimental study

    NARCIS (Netherlands)

    F.C.P.M. Adriaansen

    1987-01-01

    textabstractThe introduction of this thesis (chapter 1) ended in three questions, concerning stenosis of the airway and of the subgtottis in particular. The first and second issue can be summarized as follows: do different types of trauma have different effects on the growth of the subglottis and is

  20. Angioplasty and stent treatment of transplant renal artery stenosis.

    Science.gov (United States)

    Del Pozo, Maitane; Martí, Jordi; Guirado, Lluís; Facundo, Carme; Canal, Cristina; de la Torre, Pablo; Ballarín, José; Díaz, Joan M

    2012-07-17

    Transplant renal artery stenosis is a major complication that requires a therapeutic approach involving surgery or angioplasty. The aim of this study was to analyse the evolution of renal transplant patients with renal allograft artery stenosis treated by angioplasty and stent placement. Thirteen patients were diagnosed with transplant renal artery stenosis. Clinical suspicion was based on deterioration of renal function and/or poorly controlled hypertension with compatible Doppler ultrasound findings. The diagnosis was confirmed by arteriography, performing an angioplasty with stent placement during the same operation. A progressive improvement in renal function was observed during the first 3 months after the angioplasty, and renal function then remained stable over 2 years. In addition, blood pressure improved during the first 2 years, and as a consequence there was no need to increase the average number of anti-hypertensive drugs administered (2.5 drugs per patient). In conclusion, angioplasty with stent placement is a safe and effective procedure for the treatment of transplant renal artery stenosis.