PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled Tracheopathia osteoplastica. (Ann Otolaryngol Chir Cervicofac. 2008 Jul 7). Authors are Liétin B Vellin JF Bivahagumye L et al., from the Service d’ORL et de chirurgie de la face et du cou, CHU de Clermont-Ferrand, BP 69, 63003 Clermont-Ferrand cedex 1, France. Report a case of tracheopathia osteoplastica and describe from a literature analysis the main clinical, radiological, and therapeutic features of this rare disease based on literature review.Tracheopathia osteoplastica is a rare tumor, with unknown etiology and physiopathology. The discovery is most often incidental. Progession is slow and it does not compromise the vital prognosis. The treatment is symptomatic. Few surgical tracheal operations are described in the literature. To access the full abstract of the article, click here.
Publications
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled Retroperitoneal fibrosis, M. Ormond, periaoartitis? ( Ther Umsch. 2008 May;65(5):265-8). Authors are Vogt B Meier P Burnier M. et al., from the Service de Néphrologie et d’Hypertension, Centre Médical Universitaire Vaudois (CHUV)-Université de Lausanne, Rue du Bugnon 17, Lausanne. Retroperitoneal fibrosis or Morbus Ormond is a rare disease characterized by inflammatory fibrosis of the retroperitoneal space and the abdominal aorta often including the common iliac arteries. The abdominal aorta can be enlarged leading to a classification of retroperitoneal fibrosis with or without aneurysm of the abdominal aorta. The classic form of retroperitoneal fibrosis usually presents without aneurysm of the abdominal aorta.Drug therapy is limited to steroids alone or in combination with immunosuppressive drugs such as azathioprine or even cyclophosphamide. The response to medical therapy is variable To access the full abstract of the article, click here.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled Tracheopathia osteoplastica. (Ann Otolaryngol Chir Cervicofac. 2008 Jul 7). Authors are Liétin B Vellin JF Bivahagumye L et al., from the Service d’ORL et de chirurgie de la face et du cou, CHU de Clermont-Ferrand, BP 69, 63003 Clermont-Ferrand cedex 1, France. Report a case of tracheopathia osteoplastica and describe from a literature analysis the main clinical, radiological, and therapeutic features of this rare disease based on literature review.Tracheopathia osteoplastica is a rare tumor, with unknown etiology and physiopathology. The discovery is most often incidental. Progession is slow and it does not compromise the vital prognosis. The treatment is symptomatic. Few surgical tracheal operations are described in the literature. To access the full abstract of the article, click here.
Progressive mitral valve thickening and progressive muscle cramps as manifestations of glycogenosis VII
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PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled Progressive mitral valve thickening and progressive muscle cramps as manifestations of glycogenosis VII (Tarui’s Disease) (Cardiology. 2008;110(4):238-40). Authors are Finsterer J Stöllberger C from the Second Medical Department, and Krankenanstalt Rudolfstiftung, Vienna, Austria. Progressive heart valve thickening and shrinkage, and progressive muscle cramps have not been reported as manifestations of glycogenosis type VII (Tarui’s disease). In a 72-year-old female, Tarui’s disease was diagnosed in 1997, initially manifesting as simple partial seizures since 1977, anginal chest pain since 1982 and muscle cramps since 1983.Progression of Tarui’s disease may manifest as progressive thickening of the heart valves due to glycogen storage. Valve thickening may consecutively lead to valve insufficiency, enlargement of the atrium and atrial fibrillation. Progression of neurological manifestations may manifest as worsening muscle cramps. To access the full abstract of the article, click here.
Ecallantide (DX-88), a plasma kallikrein inhibitor for the treatment of hereditary angioedema
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PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled Ecallantide (DX-88), a plasma kallikrein inhibitor for the treatment of hereditary angioedema and the prevention of blood loss in on-pump cardiothoracic surgery (Expert Opin Biol Ther. 2008 Aug;8(8):1187-99). Author is Lehmann A from the Department of Anesthesiology and Intensive Care Medicine, Klinikum der Stadt Ludwigshafen, Germany. Plasma kallikrein plays a major role in the contact (kallikrein-kinin) cascade producing bradykinin. Bradykinin is a vasodilator, which increases vascular permeability, activates inflammation and produces pain. Plasma kallikrein is also crosslinked to the coagulation system and the complement cascade.Ecallantide (DX-88) is a potent and specific inhibitor of plasma kallikrein. Ecallantide is a recombinantly produced and engineered small protein. At present, the drug is being studied for two major indications. First, the results for the treatment of hereditary angioedema are promising. Second, a prospective randomised multi-centre trial for the reduction of blood loss during on-pump cardiothoracic surgery will be terminated in October 2008. To access the full abstract of the article, click here.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled C1 inhibitor: just a serine protease inhibitor? New and old considerations on therapeutic applications of C1 inhibitor (Expert Opin Biol Ther. 2008 Aug;8(8):1225-40). Authors are Wouters D Wagenaar-Bos I van Ham M et al., from the Department of Immunopathology, Sanquin Research at CLB and Landsteiner Laboratory, University of Amsterdam, Academic Medical Center, Plesmanlaan 125, 1066 CX Amsterdam, The Netherlands. C1 inhibitor is a potent anti-inflammatory protein as it is the major inhibitor of proteases of the contact and the complement systems. C1-inhibitor administration is an effective therapy in the treatment of patients with hereditary angioedema (HAE) who are genetically deficient in C1 inhibitor. Owing to its ability to modulate the contact and complement systems and the convincing safety profile, plasma-derived C1 inhibitor is an attractive therapeutic protein to treat inflammatory diseases other than HAE. In the present review we give an overview of the biology of C1 inhibitor and its use in HAE. Furthermore, we discuss C1 inhibitor as an experimental therapy in diseases such as sepsis and myocardial infarction To access the full abstract of the article, click here.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled Pharyngitis of infectious mononucleosis: computed tomography findings (Radiat Med. 2008 May;26(4):248-51). Authors are Kutuya N Kurosaki Y Suzuki K et al., from the Department of Radiology, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, Japan. Two women presented with sore throat and fever. Their symptoms were not alleviated by antibiotics. Cervical computed tomography (CT) with contrast enhancement demonstrated enlargement of predominant posterior cervical lymph nodes and streaky heterogeneous tonsils with interspersed low attenuation. They were diagnosed as having infectious mononucleosis by their laboratory data. Thus, when radiologists encounter these CT findings of pharyngitis that is not alleviated by antibiotic therapy, infectious mononucleosis should be considered in the differential diagnosis. To access the full abstract of the article, click here.
Safety and efficacy of carvedilol therapy for patients with dilated cardiomyopathy secondary to muscular dystrophy
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PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled Safety and efficacy of carvedilol therapy for patients with dilated cardiomyopathy secondary to muscular dystrophy (Pediatr Cardiol. 2008 Mar;29(2):343-51). Authors are Rhodes J Margossian R Darras BT et al., from the Department of Cardiology, Children’s Hospital Boston, 300 Longwood Avenue, Boston, MA 02115, USA. By the age of 20 years, almost all patients with Duchenne’s or Becker’s muscular dystrophy have experienced dilated cardiomyopathy (DCM), a condition that contributes significantly to their morbidity and mortality. Although studies have shown carvedilol to be an effective therapy for patients with other forms of DCM, few data exist concerning its safety and efficacy for patients with muscular dystrophy. Carvedilol therapy appears to be safe for patients with DCM secondary to muscular dystrophy and produces a modest improvement in systolic and diastolic function. To access the full abstract of the article, click here.
Secondary intracranial hypertension with acute intracranial pressure crisis in superficial siderosis
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PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled Secondary intracranial hypertension with acute intracranial pressure crisis in superficial siderosis (J Clin Neurosci. 2008 Jul 22.). Authors are Linder S Nowak DA Rodiek SO et al., from the Department of Neurology and Clinical Neurophysiology, Academic Hospital Bogenhausen, Technical University of Munich, Germany. Superficial siderosis of the central nervous system is a very rare disease related to hemosiderin deposits in the brain, brainstem, cerebellum and spinal cord due to chronic subarachnoid hemorrhage. Chronic increased intracranial pressure develops in about one-third of affected cases. Our case emphasizes that patients with superficial siderosis may present with sudden elevation of intracranial pressure due to chronic intracranial hypertension. In this situation permanent CSF drainage provides a useful therapeutic option. To access the full abstract of the article, click here.
Functional C1-Inhibitor diagnostics in hereditary angioedema: assay evaluation and recommendations
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PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled Functional C1-Inhibitor diagnostics in hereditary angioedema: Assay evaluation and recommendations. (J Immunol Methods. 2008 Jul 22.). Authors are Wagenaar-Bos IG Drouet C Aygören-Pursun E et al., from the Department Immunopathology, Sanquin Research at CLB, and Landsteiner Laboratory, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands. Hereditary angioedema (HAE) is an autosomal dominant disease characterized by recurrent episodes of potentially life-threatening angioedema.In addition to low C4 levels, the most important laboratory parameter for correct diagnosis of HAE or angioedema due to acquired C1-Inh deficiency is reduced C1-Inh function (fC1-Inh).We can recommend the chromogenic assay for the determination of fC1-Inh, while the complex ELISA needs further investigation. To access the full abstract of the article, click here.