Search Results
Results 351 - 400 of 1813
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Metry Denise - - 2009
OBJECTIVES: A subgroup of patients with infantile hemangiomas have associated structural anomalies of the brain, cerebral vasculature, eyes, sternum, and/or aorta in the neurocutaneous disorder known as PHACE syndrome. The diagnosis has been broadly inclusive by using a case definition of a facial hemangioma plus >or=1 extracutaneous features, leading to ...
Chapireau David - - 2010
The aim of this study was to review all the paraneoplastic syndromes of primary tumours of the oral cavity. Metastatic tumours of the mouth and primary tumours of the oropharynx (including tonsils), and major salivary glands were excluded. The primary search was conducted on PubMed, Scopus and EMBASE, and included ...
Moss Heather E - - 2010
A 60-year-old man presented with vitritis and optic neuropathy in the setting of headaches and behavioral changes. MRI brain revealed bilateral temporal lobe inflammation consistent with limbic encephalitis. He was subsequently diagnosed with small cell lung cancer with a paraneoplastic syndrome characterized by CRMP5 IgG as a cause of his ...
Soni Sachin S - - 2009
All the vital organs of the body share information by virtue of various biological mediators. Primary pathology of a major organ can lead to dysfunction of the other. Cardiorenal syndrome is an important example of such organ crosstalk. Primary dysfunction of the heart or kidney can lead to injury of ...
Umeda Naoki - - 2009
The pulmonary complications of end-stage liver disease include hepatopulmonary syndrome and portopulmonary hypertension. The etio-pathogenesis of these conditions is as yet unclear. Hepatopulmonary syndrome is a gas exchange abnormality and usually manifests as hypoxemia secondary to intra-pulmonary vascular shunts. These shunts can be demonstrated by echocardiography using agitated saline injections, ...
Ablin Jacob N - - 2009
Fibromyalgia is a common pain syndrome characterized by widespread pain, tenderness, and a number of other somatic symptoms and syndromes. Although there was original skepticism that any objective abnormalities would be identified in these individuals, at present there are many that have been reproducibly identified, and most point to dysregulation ...
Vora Neeta - - 2009
Large (>90%) for gestational age (LGA) fetuses are usually identified incidentally. Detection of the LGA fetus should first prompt the provider to rule out incorrect dates and maternal diabetes. Once this is done, consideration should be given to certain overgrowth syndromes, especially if anomalies are present. The overgrowth syndromes have ...
Neukirchen Judith - - 2009
Cytogenetic findings in myelodysplastic syndromes play an important role in diagnosis, prognostication and clinical decision making. Therefore, they became an important aspect in scoring systems such as the International Prognostic Scoring System (IPSS) and the WHO-adapted Prognostic Scoring System (WPSS). Ongoing efforts to refine the categorization of karyotypes with regard ...
Simadibrata Marcellus - - 2009
Dyspepsia is a syndrome characterized by symptoms and signs of upper gastrointestinal tract and the adjacent organs. It is estimated that 25% of the community have symptoms of dyspepsia syndrome. One-third of patients who visit general physician practices are patients with dyspepsia syndrome; and half of patients who visit gastroenterologists ...
Kotzot Dieter - - 2009
Chest wall deformities such as pectus excavatum, pectus carinatum, and cleft sternum can be isolated malformations or dysmorphic features of genetic associations, monogenic disorders, and various numeric and structural chromosomal aberrations. In contrast to the most important syndromes such as Marfan syndrome or Noonan syndrome that can be associated with ...
Juneja M - - 2009
Macrophage activation syndrome is a rare and potentially life threatening complication of childhood rheumatic disorders. It is described most commonly with systemic onset juvenile idiopathic arthritis (soJIA). The major clinical manifestations are non-remitting fever, hepatosplenomegaly, lymphadenopathy, bleeding diathesis, altered mental status and rash and may mimic a fl are of ...
Hobert Judith A - - 2009
PTEN hamartoma tumor syndrome (PHTS) encompasses four major clinically distinct syndromes associated with germline mutations in the tumor suppressor PTEN. These allelic disorders, Cowden syndrome, Bannayan-Riley-Ruvalcaba syndrome, Proteus syndrome, and Proteus-like syndrome are associated with unregulated cellular proliferation leading to the formation of hamartomas. Thus far, an increased risk of ...
Marks Seth D - - 2009
Neuroendocrine changes in the hypothalamic-pituitary-thyroid axis during critical illness result in nonthyroidal illness syndrome (NTIS) characterized by abnormal thyrotropin (TSH) and thyroid hormone levels. Studies looking at the natural history of neuroendocrine changes during critical illness have revealed the presence of NTIS. NTIS has been described in a variety of ...
Monte R - - 2009
BACKGROUND: The clinical expressions of alcohol withdrawal syndrome (AWS) may vary and the factors determining these variations are not well-known. It would be useful to have a set of clinical tools capable of predicting which patients are likely to develop the more severe forms of the syndrome. AIM: To analyse ...
Finsterer Josef - - 2009
Mitochondrial disorders (MIDs) are an increasingly recognized condition. The second most frequently affected organ in MIDs is the central nervous system. One of the most prevalent clinical CNS manifestations of MIDs is ataxia. Ataxia may be even the dominant manifestation of a MID. This is why certain MIDs should be ...
Boscaro Marco - - 2009
Clinical decision making for patients with suspect hypercortisolism involves a complex diagnostic assessment. Cushing's syndrome remains one of the most challenging endocrine pathologies. Most clinical features overlap with those of common diseases found in the general population, and some patients have an atypical clinical presentation with only isolated symptoms. Recently, ...
Schröder Alexandra - - 2009
Apheresis is a general term that describes removal of abnormal blood constituents by extracorporeal blood purification methods. To date, therapeutic plasma exchange (PE) is the most common apheresis procedure. Here, plasma is separated from corpuscular blood constituents and replaced with a substitution fluid. In contrast to immunoadsorption, PE is a ...
Tack Jan - - 2009
Dumping syndrome is a frequent complication of esophageal, gastric or bariatric surgery. Rapid gastric emptying, with the delivery to the small intestine of a significant proportion of solid food as large particles that are difficult to digest, is a key event in the pathogenesis of this syndrome. This occurrence causes ...
Falk Dean - - 2009
The type specimen (LB1) of Homo floresiensis has been hypothesized to be a pathological human afflicted with Laron Syndrome (LS), a type of primary growth hormone insensitivity (Hershkovitz et al.: Am J Phys Anthropol 134 [2007] 198-208). Comparing measurements, photographs and three-dimensional, computed-tomography reconstructions of LB1 with data and diagnoses ...
Bennani-Baiti N - - 2009
Cancer anorexia-cachexia syndrome (CACS) is a lethal but poorly defined involuntary wasting disorder. Loss of skeletal muscle and fat distinguishes it from starvation. Cachexia has been described as a clinical syndrome since ancient times, and the poor prognosis has long been acknowledged. In this article we have reviewed historical perspectives ...
Hagmann Sebastién - - 2009
Skewfoot is a rare deformity characterized by forefoot adduction and hindfoot valgus. Its etiology and natural history are unknown, although congenital and syndromic forms are observed. Currently, there is no consent about the treatment of skewfoot. Due to its potential resistance to the effects of therapy, it must be differentiated ...
Nilsson Björn M - - 2009
We report successful treatment with electroconvulsive therapy of a comorbid condition including severe obsessive-compulsive symptoms and hypochondriacal delusions in a 38-year-old man with Asperger syndrome. His condition deteriorated into a severely disabled chronic state that was refractory to different pharmacological and psychological treatments but was completely reversed after electroconvulsive therapy. ...
Lazar Meredith A - - 2009
Snapping scapula syndrome arises from either a soft-tissue or a skeletal anomaly within the scapulothoracic space that creates a cracking sound during scapulothoracic motion that patients associate with pain. Nonoperative measures consisting of supervised physical therapy, anti-inflammatory medications, and therapeutic injections are the mainstay of treatment. Open, arthroscopic, and combined ...
Salinas Carlos F - - 2009
There are many ways to classify ectodermal dysplasia syndromes. Clinicians in practice use a list of syndromes from which to choose a potential diagnosis, paging through a volume, such as Freire-Maia and Pinheiro's corpus, matching their patient's findings to listed syndromes. Medical researchers may want a list of syndromes that ...
Shoja Mohammadali M - - 2010
Johan Georg Raeder (1889-1959) was the most eminent Norwegian ophthalmologist in the early decades of the last century. Raeder made significant contributions to our current understanding of glaucoma. He is remembered for a syndrome he described, that of trigeminal nerve neuralgia and/or paresis and incomplete Horner's syndrome (oculopupillary sympathetic paresis). ...
Wong Hilda - - 2010
Functional neuroendocrine tumors are often low-grade malignant neoplasms that can be cured by surgery if detected early, and such detection may in turn be accelerated by the recognition of neuropeptide hypersecretion syndromes. Uniquely, however, relief of peptic symptoms induced by hypergastrinemia is now available from acid-suppressive drugs such as proton-pump ...
Reich-Schupke Stefanie - - 2010
The post-thrombotic syndrome (PTS) is a common but not completely understood and rarely studied sequela of an acute deep vein thrombosis. The influence of several risk factors on the incidence or severity of PTS is controversial. The therapeutic options for PTS are still limited. Appropriate medical compression stockings can reduce ...
Guerriero Silvana - - 2009
Aicardi syndrome is characterized by agenesis of the corpus callosum, chorioretinal lacunae, and infantile spasms. The authors describe the case of a girl with chorioretinal lacunae, seizures, and cerebral cyst, but a normal corpus callosum. Incomplete forms of Aicardi syndrome are reported in the literature, but typical choroidal lacunae are ...
Nolan Jerry P - - 2010
AIM OF THE REVIEW: To review the epidemiology, pathophysiology, treatment and prognostication in relation to the post-cardiac arrest syndrome. METHODS: Relevant articles were identified using PubMed, EMBASE and an American Heart Association EndNote master resuscitation reference library, supplemented by hand searches of key papers. Writing groups comprising international experts were ...
Naumov Leonid B - - 2009
Diagnostic interpretation at auscultation of heart murmurs is accompanied by frequent errors. It creates serious clinical, pedagogical, organizational and social problems. The standard nosological principle of a clinical information description from the diagnosis (a disease name) to the description of symptoms/signs contradicts to real clinical practice from revealing of symptoms ...
Nguyen Phuong L - - 2009
Myelodysplastic syndromes (MDS) are a heterogeneous group of bone marrow disorders that affect mostly the elderly and have a variable probability of progression to acute leukemia. The diagnosis of MDS rests largely on a critical morphologic review of blood and bone marrow slides, with careful correlation with other clinical and ...
English W A - - 2009
Accurate prediction of neurological outcome in survivors of cardiac arrest may be difficult. We report the case of a 44-year-old survivor of a hypoxic cardiac arrest who repeatedly developed relentless myoclonic jerks on attempted discontinuation of his propofol infusion. These were initially thought to represent myoclonic status epilepticus before the ...
Rigante Donato - - 2009
Many children experience recurrent fevers with no easily identifiable source and only a careful follow-up helps in the early identification of other presenting symptoms of other defined conditions which require medical intervention. Autoinflammatory syndromes are rare childhood-onset disorders of the innate immunity in which recurrent flares of fever and inflammation ...
Lefebvre Valerie - - 2009
BACKGROUND: There are many conditions affecting the corneal endothelium with similar clinical appearances, though with different prognoses, management approaches, and pathophysiologic development. CASE: A 39-year-old black woman with a previous diagnosis of asymmetric corneal posterior polymorphous dystrophy (PPMD) presented complaining of irritation in the left eye, worsening over the last ...
de Buys Roessingh Anthony S - - 2009
Lambert-Eaton myasthenic syndrome is a paraneoplastic syndrome that may reveal a primitive tumor. Neuroblastoma in children and small cell lung carcinoma in adults are the leading tumors revealed or expressed by paraneoplastic phenomena. The clinical neurologic manifestations of Lambert-Eaton myasthenic syndrome are muscular weakness, sleepiness, absence of reflexes, and dysautonomia. ...
Lankisch Tim O - - 2009
Autoimmune polyglandular syndromes are rare autoimmune endocrinopathies that are associated with nonendocrine autoimmunopathies. Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED), also named autoimmune polyglandular syndrome type 1 (APS-1), is distinguished from autoimmune polyglandular syndrome 2 (APS-2). Major disease components of APECED are adrenal insufficiency, hypoparathyroidism, and candidiasis. The diagnosis is established by the ...
Passos-Bueno Maria Rita - - 2009
Our aim in this review is to discuss currently known mechanisms associated with three important syndromes of the first and second pharyngeal arches: Treacher Collins syndrome (TCS), Oculo-auriculo-vertebral syndrome (AOVS) and Auriculo-Condylar syndrome (ACS) or question mark ear syndrome. TCS and ACS are autosomal dominant diseases, with nearly complete penetrance ...
Kamenisch York - - 2009
Progeroid syndromes are a group of diseases characterized by signs of premature aging. These syndromes comprise diseases such as Werner syndrome, Bloom syndrome, Rothmund-Thomson syndrome, Hutchinson-Gilford syndrome, Fanconi anemia, and ataxia-telangiectasia, as well as xeroderma pigmentosum, trichothiodystrophy, and Cockayne syndrome. Clinical symptoms of premature aging are skin atrophy with loss ...
Yuki Nobuhiro - - 2009
Since identification of the autoantibodies in Fisher syndrome, remarkable progress has been made in our understanding of that syndrome and related conditions. Because of the similarities in the clinical presentations of it and Bickerstaff brainstem encephalitis, opinions differ as to whether the two are distinct or related syndromes and whether ...
Malkani Roneil G - - 2010
Waldenstrom's macroglobulinemia (WM) is a chronic lymphoproliferative disorder within the spectrum of lymphoplasmacytic lymphoma characterized by proliferation of plasma cells, small lymphocytes, and plasmacytoid lymphocytes. Central nervous system involvement is very rare (Bing-Neel [BN] syndrome). We present the case of a 62-year-old woman previously diagnosed with WM who presented with ...
Nolan Jerry P - - 2009
AIM OF THE REVIEW: To review the epidemiology, pathophysiology, treatment and prognostication in relation to the post-cardiac arrest syndrome. METHODS: Relevant articles were identified using PubMed, EMBASE and an American Heart Association EndNote master resuscitation reference library, supplemented by hand searches of key papers. Writing groups comprising international experts were ...
Balk Lennart - - 2009
Wild birds of several species are dying in large numbers from an idiopathic paralytic disease in the Baltic Sea area. Here, we demonstrate strong relationships between this disease, breeding failure, and thiamine (vitamin B(1)) deficiency in eggs, pulli, and full-grown individuals. Thiamine is essential for vertebrates, and its diphosphorylated form ...
Dass Rashna - - 2010
Macrophage activation syndrome (MAS) is a clinical syndrome caused by an excessive proliferation of T lymphocytes and well-differentiated macrophages; an entity distinct from malignant histiocytosis. Although rheumatologic conditions are the common cause of MAS, a wide range of infections are also seen to cause MAS. We report an adolescent with ...
Mosca Andrea - - 2009
OBJECTIVES: The increase in hemoglobin (Hb) F level is variably associated to the presence of beta thalassemia trait, and is more typical in presence of deltabeta thalassemia and of hereditary persistence of fetal hemoglobin. In normal healthy subjects variable levels of HbF are related to the presence of the polymorphism ...
Cheptou Pierre-Olivier - - 2009
The existence of a syndrome linking dispersal rate and mating system has long been debated in evolutionary ecology, especially in plants. Some verbal models hypothesize that the ability to self-fertilize may be associated with high dispersal, since completely outcrossing species cannot reproduce when they disperse to an empty destination site. ...
Mehta Shyamal H - - 2009
Neurologic paraneoplastic syndromes (NPSs) result from damage to the nervous system due to the remote effects of cancer not related to metastasis, infection, or metabolic derangements. NPSs are rare, affecting 1 in 10,000 patients with cancer. Pathogenesis is likely related to the immune mechanisms: normal neural tissue is mistakenly attacked ...
Marshall John C - - 2009
BACKGROUND: A complex network of biological mediators underlies the clinical syndrome of sepsis. The nonspecific physiologic criteria of sepsis syndrome or the systemic inflammatory response syndrome do not adequately identify patients who might benefit from either conventional anti-infective therapies or from novel therapies that target specific mediators of sepsis. Validated ...
Yilmaz Mehmet - - 2009
Beckwith-Wiedemann syndrome is a congenital syndrome with some anomaly in overgrowth. Most common manifestations are exomphalos, macroglossia, gigantism, and visceromegaly. Overgrowth in tongue's size caused clinical symptoms such as dysphagia, speech disorder, strong in chewing, upper-airway obstruction, and psychological problems with appearance. Cold surgical techniques are commonly used in treating ...
Cadena Jose - - 2009
Tumor necrosis factor alpha antagonists have proven efficacious for a variety of autoimmune-mediated diseases. However, recent data have highlighted the risk of invasive fungal infections with their use. These agents are typically discontinued when infectious complications occur during therapy; however, the immune reconstitution inflammatory syndrome (IRIS) may be seen after ...
Piagkou Maria M Department of Anatomy, Medical School, University of Athens, Athens, Greece. - - 2009
Eagle's syndrome represents symptoms brought on by compression of regional structures by elongation of the styloid process or ossification of the stylohyoid or stylomandibular ligaments. Watt Eagle described it for the first time in 1937, dividing it into two subtypes: the "classic syndrome" and the "stylo-carotid artery syndrome." Many theories ...
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